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Sample records for left frontotemporal area

  1. Perfusion SPECT studies with mapping of Brodmann areas in differentiating Alzheimer's disease from frontotemporal degeneration syndromes.

    Science.gov (United States)

    Valotassiou, Varvara; Papatriantafyllou, John; Sifakis, Nikolaos; Tzavara, Chara; Tsougos, Ioannis; Kapsalaki, Eftychia; Hadjigeorgiou, George; Georgoulias, Panagiotis

    2012-12-01

    The aim of this study was to evaluate the contribution of brain perfusion single-photon emission computed tomography (SPECT) studies with mapping of Brodmann areas (BAs) in the differential diagnosis between Alzheimer's disease (AD) and frontotemporal degeneration (FTLD) syndromes. Thirty-nine patients with AD and 73 patients with FTLD syndromes [behavioural variant FTLD (bvFTLD); language variant FTLD (lvFTLD), including semantic dementia (SD) and progressive nonfluent aphasia (PNFA); and corticobasal degeneration (CBD)/progressive supranuclear palsy (PSP) syndromes] underwent brain perfusion SPECT. The NeuroGam software was used for the semiquantitative evaluation of perfusion in BAs of the left (L) and right (R) hemispheres. Compared with those in AD patients, BAs with statistically significant hypoperfusion were found in the prefrontal, orbitofrontal and cingulated cortices and Broca's areas of FTLD and bvFTLD patients; in the temporal and prefrontal cortices and Broca's areas of lvFTLD patients; in the left temporal gyrus of SD patients; in premotor and supplementary motor, prefrontal, orbitofrontal, temporal and anterior cingulated cortices and Broca's areas of PNFA patients; and in the prefrontal, temporal, posterior cingulated and primary and secondary visual cortices of CBD/PSP patients. BA 46R could differentiate AD patients from FTLD and bvFTLD patients; 21L and 25L were found to be independent predictors for lvFTLD in comparison with AD, and 25R, 21L and 23R could differentiate AD patients from PNFA, SD and CBD/PSP patients, respectively. Brain perfusion SPECT with BA mapping in AD and FTLD patients could improve the definition of brain areas that are specifically implicated in these disorders, resulting in a more accurate differential diagnosis.

  2. Left fronto-temporal dynamics during agreement processing: evidence for feature-specific computations.

    Science.gov (United States)

    Molinaro, Nicola; Barber, Horacio A; Pérez, Alejandro; Parkkonen, Lauri; Carreiras, Manuel

    2013-09-01

    Grammatical agreement is a widespread language phenomenon that indicates formal syntactic relations between words; however, it also conveys basic lexical (e.g. grammatical gender) or semantic (e.g. numerosity) information about a discourse referent. In this study, we focus on the reading of Spanish noun phrases, violating either number or gender determiner-noun agreement compared to grammatical controls. Magnetoencephalographic activity time-locked to the onset of the noun in both types of violation revealed a left-lateralized brain network involving anterior temporal regions (~220 ms) and, later in time, ventro-lateral prefrontal regions (>300 ms). These activations coexist with dependency-specific effects: in an initial step (~170 ms), occipito-temporal regions are employed for fine-grained analysis of the number marking (in Spanish, presence or absence of the suffix '-s'), while anterior temporal regions show increased activation for gender mismatches compared to grammatical controls. The semantic relevance of number agreement dependencies was mainly reflected by left superior temporal increased activity around 340 ms. These findings offer a detailed perspective on the multi-level analyses involved in the initial computation of agreement dependencies, and theoretically support a derivational approach to agreement computation. Copyright © 2013 Elsevier Inc. All rights reserved.

  3. Brain perfusion SPECT with Brodmann areas analysis in differentiating frontotemporal dementia subtypes.

    Science.gov (United States)

    Valotassiou, Varvara; Papatriantafyllou, John; Sifakis, Nikolaos; Tzavara, Chara; Tsougos, Ioannis; Psimadas, Dimitrios; Kapsalaki, Eftychia; Fezoulidis, Ioannis; Hadjigeorgiou, George; Georgoulias, Panagiotis

    2014-01-01

    Despite the known validity of clinical diagnostic criteria, significant overlap of clinical symptoms between Frontotemporal dementia (FTD) subtypes exists in several cases, resulting in great uncertainty of the diagnostic boundaries. We evaluated the perfusion between FTD subtypes using brain perfusion (99m)Tc-HMPAO SPECT with Brodmann areas (BA) mapping. NeuroGam software was applied on single photon emission computed tomographic (SPECT) studies for the semi-quantitative evaluation of perfusion in BA and the comparison with the software's normal database. We studied 91 consecutive FTD patients: 21 with behavioural variants (bvFTD), 39 with language variants (lvFTD) [12 with progressive non-fluent aphasia (PNFA), 27 with semantic dementia (SD)], and 31 patients with progressive supranuclear palsy (PSP)/corticobasal degeneration (CBD). Stepwise logistic regression analyses showed that the BA 28L and 32R could independently differentiate bvFTD from lvFTD, while the BA 8R and 25R could discriminate bvFTD from SD and PNFA, respectively. Additionally, BA 7R and 32R were found to discriminate bvFTD from CBD/PSP. The only BA that could differentiate SD from PNFA was 6L. BA 6R and 20L were found to independently differentiate CBD/PSP from lvFTD. Moreover, BA 20L and 22R could discriminate CBD/PSP from PNFA, while BA 6R, 20L and 45R were found to independently discriminate CBD/PSP from SD. Brain perfusion SPECT with BA mapping can be a useful additional tool in differentiating FTD variants by improving the definition of brain areas that are specifically implicated, resulting in a more accurate differential diagnosis in atypical or uncertain forms of FTD.

  4. Modality-Dependent or Modality-Independent Processing in Mental Arithmetic: Evidence From Unimpaired Auditory Multiplication for a Patient With Left Frontotemporal Stroke.

    Science.gov (United States)

    Cheng, Dazhi; Wu, Haiyan; Yuan, Li; Xu, Rui; Chen, Qian; Zhou, Xinlin

    2017-09-01

    Mental arithmetic is essential to daily life. Researchers have explored the mechanisms that underlie mental arithmetic. Whether mental arithmetic fact retrieval is dependent on surface modality or knowledge format is still highly debated. Chinese individuals typically use a procedure strategy for addition; and they typically use a rote verbal strategy for multiplication. This provides a way to examine the effect of surface modality on different arithmetic operations. We used a series of neuropsychological tests (i.e., general cognitive, language processing, numerical processing, addition, and multiplication in visual and auditory conditions) for a patient who had experienced a left frontotemporal stroke. The patient had language production impairment; but preserved verbal processing concerning basic numerical abilities. Moreover, the patient had preserved multiplication in the auditory presentation rather than in the visual presentation. The patient suffered from impairments in an addition task, regardless of visual or auditory presentation. The findings suggest that mental multiplication could be characterized as a form of modality-dependent processing, which was accessed through auditory input. The learning strategy of multiplication table recitation could shape the verbal memory of multiplication leading to persistence of the auditory module. (JINS, 2017, 23, 692-699).

  5. Evaluation of brain perfusion in specific Brodmann areas in Frontotemporal dementia and Alzheimer disease using automated 3-D voxel based analysis

    Energy Technology Data Exchange (ETDEWEB)

    Valotassiou, V; Tsougos, I; Tzavara, C; Georgoulias, P [Nuclear Medicine Dpt, University Hospital of Larissa, Larissa (Greece); Papatriantafyllou, J; Karageorgiou, C [Neurology Dpt, General Hospital ' G. Gennimatas' , Athens (Greece); Sifakis, N; Zerva, C [Nuclear Medicine Dpt, ' Alexandra' University Hospital, Athens (Greece)], E-mail: vanvalot@yahoo.gr

    2009-05-15

    Introduction. Brain perfusion studies with single-photon emission computed tomography (SPECT) have been applied in demented patients to provide better discrimination between frontotemporal dementia (FTD) and Alzheimer's disease (AD). Aim. To assess the perfusion of specific Brodmann (Br) areas of the brain cortex in FTD and AD patients, using NeuroGam processing program to provide 3D voxel-by-voxel cerebral SPECT analysis. Material and methods. We studied 34 consecutive patients. We used the established criteria for the diagnosis of dementia and the specific established criteria for the diagnosis of FTD and AD. All the patients had a neuropsychological evaluation with a battery of tests including the mini-mental state examination (MMSE).Twenty-six patients (16 males, 10 females, mean age 68.76{+-}6.51 years, education 11.81{+-}4.25 years, MMSE 16.69{+-}9.89) received the diagnosis of FTD and 8 patients (all females, mean age 71.25{+-}10.48 years, education 10{+-}4.6 years, MMSE 12.5{+-}3.89) the diagnosis of AD. All the patients underwent a brain SPECT. We applied the NeuroGam Software for the evaluation of brain perfusion in specific Br areas in the left (L) and right (R) hemispheres. Results. Statistically significant hypoperfusion in FTD compared to AD patients, was found in the following Br areas: 11L (p<0.0001), 11R, 20L, 20R, 32L, 38L, 38R, 44L (p<0.001), 32R, 36L, 36R, 45L, 45R, 47R (p<0.01), 9L, 21L, 39R, 44R, 46R, 47L (p<0.05). On the contrary, AD patients presented significant (p<0.05) hypoperfusion in 7R and 39R Br areas. Conclusion. NeuroGam processing program of brain perfusion SPECT could result in enhanced accuracy for the differential diagnosis between AD and FTD patients.

  6. Evaluation of brain perfusion in specific Brodmann areas in Frontotemporal dementia and Alzheimer disease using automated 3-D voxel based analysis

    Science.gov (United States)

    Valotassiou, V.; Papatriantafyllou, J.; Sifakis, N.; Karageorgiou, C.; Tsougos, I.; Tzavara, C.; Zerva, C.; Georgoulias, P.

    2009-05-01

    Introduction. Brain perfusion studies with single-photon emission computed tomography (SPECT) have been applied in demented patients to provide better discrimination between frontotemporal dementia (FTD) and Alzheimer's disease (AD). Aim. To assess the perfusion of specific Brodmann (Br) areas of the brain cortex in FTD and AD patients, using NeuroGam processing program to provide 3D voxel-by-voxel cerebral SPECT analysis. Material and methods. We studied 34 consecutive patients. We used the established criteria for the diagnosis of dementia and the specific established criteria for the diagnosis of FTD and AD. All the patients had a neuropsychological evaluation with a battery of tests including the mini-mental state examination (MMSE).Twenty-six patients (16 males, 10 females, mean age 68.76±6.51 years, education 11.81±4.25 years, MMSE 16.69±9.89) received the diagnosis of FTD and 8 patients (all females, mean age 71.25±10.48 years, education 10±4.6 years, MMSE 12.5±3.89) the diagnosis of AD. All the patients underwent a brain SPECT. We applied the NeuroGam Software for the evaluation of brain perfusion in specific Br areas in the left (L) and right (R) hemispheres. Results. Statistically significant hypoperfusion in FTD compared to AD patients, was found in the following Br areas: 11L (pareas. Conclusion. NeuroGam processing program of brain perfusion SPECT could result in enhanced accuracy for the differential diagnosis between AD and FTD patients.

  7. Regional cerebral glucose metabolism in frontotemporal lobar degeneration

    International Nuclear Information System (INIS)

    Park, J.M.; Cho, S.S.; Lee, K.-H.; Choi, Y.; Choe, Y.S.; Kim, B.-T.; Kim, S.E.; Kwon, J.C.; Na, D.L.

    2002-01-01

    Purpose: Frontotemporal lobar degeneration (FTLD) is the third most common cause of dementia, following Alzheimer's disease and Lewy body disease. Four prototypic neuro behavioral syndromes can be produced by FTLD: frontotemporal dementia (FTD), frontotemporal dementia with motor neuron disease (MND), semantic dementia (SD), and progressive aphasia (PA). We investigated patterns of metabolic impairment in patients with FTLD presented with four different clinical syndromes. Methods: We analyzed glucose metabolic patterns on FDG PET images obtained from 34 patients with a clinical diagnosis of FTLD (19 FTD, 6 MND, 6 SD, and 3 PA, according to a consensus criteria for clinical syndromes associated with FTLD) and 7 age-matched healthy controls using SPM99. Results: Patients with FTD had metabolic deficit in the left frontal cortex and bilateral anterior temporal cortex. Hypometabolism in the bilateral pre-motor area was shown in patients with MND. Patients with SD had metabolic deficit in the left posterior temporal cortex including Wernicke's area, while hypometabolism in the bilateral inferior frontal gyrus including Broca's area and left angular gyrus was seen in patients with PA. These metabolic patterns were well correlated with clinical and neuropsychological features of FTLD syndromes. Conclusion: These data provide a biochemical basis of clinical classification of FTLD. FDG PET may help evaluate and classify patients with FTLD

  8. Evaluation of brain perfusion in specific Brodmann areas in Frontotemporal dementia and Alzheimer disease using automated 3-D voxel based analysis

    International Nuclear Information System (INIS)

    Valotassiou, V; Tsougos, I; Tzavara, C; Georgoulias, P; Papatriantafyllou, J; Karageorgiou, C; Sifakis, N; Zerva, C

    2009-01-01

    Introduction. Brain perfusion studies with single-photon emission computed tomography (SPECT) have been applied in demented patients to provide better discrimination between frontotemporal dementia (FTD) and Alzheimer's disease (AD). Aim. To assess the perfusion of specific Brodmann (Br) areas of the brain cortex in FTD and AD patients, using NeuroGam processing program to provide 3D voxel-by-voxel cerebral SPECT analysis. Material and methods. We studied 34 consecutive patients. We used the established criteria for the diagnosis of dementia and the specific established criteria for the diagnosis of FTD and AD. All the patients had a neuropsychological evaluation with a battery of tests including the mini-mental state examination (MMSE).Twenty-six patients (16 males, 10 females, mean age 68.76±6.51 years, education 11.81±4.25 years, MMSE 16.69±9.89) received the diagnosis of FTD and 8 patients (all females, mean age 71.25±10.48 years, education 10±4.6 years, MMSE 12.5±3.89) the diagnosis of AD. All the patients underwent a brain SPECT. We applied the NeuroGam Software for the evaluation of brain perfusion in specific Br areas in the left (L) and right (R) hemispheres. Results. Statistically significant hypoperfusion in FTD compared to AD patients, was found in the following Br areas: 11L (p<0.0001), 11R, 20L, 20R, 32L, 38L, 38R, 44L (p<0.001), 32R, 36L, 36R, 45L, 45R, 47R (p<0.01), 9L, 21L, 39R, 44R, 46R, 47L (p<0.05). On the contrary, AD patients presented significant (p<0.05) hypoperfusion in 7R and 39R Br areas. Conclusion. NeuroGam processing program of brain perfusion SPECT could result in enhanced accuracy for the differential diagnosis between AD and FTD patients.

  9. Moral processing deficit in behavioral variant frontotemporal dementia is associated with facial emotion recognition and brain changes in default mode and salience network areas.

    Science.gov (United States)

    Van den Stock, Jan; Stam, Daphne; De Winter, François-Laurent; Mantini, Dante; Szmrecsanyi, Benedikt; Van Laere, Koen; Vandenberghe, Rik; Vandenbulcke, Mathieu

    2017-12-01

    Behavioral variant frontotemporal dementia (bvFTD) is associated with abnormal emotion recognition and moral processing. We assessed emotion detection, discrimination, matching, selection, and categorization as well as judgments of nonmoral, moral impersonal, moral personal low- and high-conflict scenarios. bvFTD patients gave more utilitarian responses on low-conflict personal moral dilemmas. There was a significant correlation between a facial emotion processing measure derived through principal component analysis and utilitarian responses on low-conflict personal scenarios in the bvFTD group (controlling for MMSE-score and syntactic abilities). Voxel-based morphometric multiple regression analysis in the bvFTD group revealed a significant association between the proportion of utilitarian responses on personal low-conflict dilemmas and gray matter volume in ventromedial prefrontal areas ( p height  importance of emotions in moral cognition and suggest a common basis for deficits in both abilities, possibly related to reduced experience of emotional sensations. At the neural level abnormal moral cognition in bvFTD is related to structural integrity of the medial prefrontal cortex and functional characteristics of the anterior insula. The present findings provide a common basis for emotion recognition and moral reasoning and link them with areas in the default mode and salience network.

  10. The frontotemporal (pterional) approach: an historical perspective.

    Science.gov (United States)

    Altay, Tamer; Couldwell, William T

    2012-08-01

    The frontotemporal, so-called pterional, approach has evolved with the contribution of many neurosurgeons over the past century. It has stood the test of time and has been the most commonly used transcranial approach in neurosurgery. In its current form, drilling the sphenoid wing as far down as the superior orbital fissure with or without the removal of the anterior clinoid, thinning the orbital roof, and opening the Sylvian fissure and basal cisterns are the hallmarks of this approach. Tumoral and vascular lesions involving the sellar/parasellar area, anterior and anterolateral circle of Willis, middle cerebral artery, anterior brainstem, upper basilar artery, insula, basal ganglia, mesial temporal region, anterior cranial fossa, orbit, and optic nerve are within the reach of the frontotemporal approach. In this article, we review the origins, evolution, and modifications of the frontotemporal approach and update the discussion of some of the related derivative procedures.

  11. Semi-quantitative analysis of perfusion of Brodmann areas in the differential diagnosis of cognitive impairment in Alzheimer's disease, fronto-temporal dementia and mild cognitive impairment.

    Science.gov (United States)

    Tranfaglia, Cristina; Palumbo, Barbara; Siepi, Donatella; Sinzinger, Helmut; Parnetti, Lucilla

    2009-01-01

    Different perfusion defects reflect neurological damage characteristics of different kinds of dementia. Our aim was to investigate the role of brain single photon emission tomography (SPET) with semiquantitative analysis of Brodmann areas in dementia, by technetium-99m - hexamethyl-propylenamine- oxime ((99m)Tc-HMPAO) brain SPET with semiquantitative analysis of Brodmann areas in patients with Alzheimer's disease (AD), frontotemporal dementia (FTD) and mild cognitive impairment (MCI). We studied 75 patients, 25 with AD (NiNCDS ADRDA criteria), 25 with FTD (Lund and Manchester criteria), 25 with MCI (EADC criteria). After i.v. injection of 740MBq of (99m)Tc-HMPAO, each patient underwent brain SPET. A software application was used able to map the SPET brain image to a stereotaxic atlas (Talairach), providing an affine co-registration by blocks of data defined in the Talairach space. A normal database calculating voxel by voxel the mean and the standard deviation of the measured values was built. Functional SPET data of 3D regions of interest (ROI) of predefined Brodmann's area templates were compared with those of a database of healthy subjects of the same age and gender. Mean values obtained in the Brodmann area ROI in the different groups of patients studied were evaluated. Our results showed that different Brodmann areas were significantly impaired in the different categories of dementia subjects. Both areas 37 (temporal gyrus) and 39 (angular gyrus) of AD patients (mean+/-SD: 37L= -1.6+/-1.0; 37R= -1.5+/-1.1; 39L= -2.3+/-1.3; 39R= -1.9+/-1.2) showed significant hypoperfusion (Pareas 40 (supramarginal gyrus) (40L= -2.6+/-1.0; 40R= -2.3+/-1.1) with respect to MCI patients (40L= -1.8+/-0.9; 40R= -1.7+/-1.2). Finally, FTD patients showed significant hypoperfusion (Pareas 47 (frontal association cortex) (47L= -1.8+/-0.8; 47R= -1.1+/-0.8) in comparison with MCI subjects (47L= -1.2+/-0.9; 47R= -0.9+/-0.9). In conclusion, our results suggest that semiquantitative

  12. Differential regional cerebral glucose metabolism in clinical syndromes of frontotemporal lobar degeneration: a study with FDG PET

    Energy Technology Data Exchange (ETDEWEB)

    Park, J. M.; Cho, S. S.; Na, D. L.; Lee, K. H.; Choi, Y.; Choe, Y. S.; Kim, B. T.; Kim, S. E. [College of Medicine, Sungkyunkwan Univ., Seoul (Korea, Republic of)

    2001-07-01

    Frontotemporal lobar degeneration( FTLD) is the third most common dementia, following Alzheimer's disease and Lewy body disease. Four prototypic neurobehavioral syndromes can be produced by FTLD: frontotemporal dementia (FTD), frontotemporal dementia with motor neuron disease (MND), semantic dementia (SD), and progressive aphasia (PA). We investigated patterns of metabolic impairment in patient with FTLD presented with four different clinical syndromes. We analysed glucose metabolic patterns on FDG PET images obtained from 34 patients with a clinical diagnosis of FTLD (19 FTD, 6 MND, 6 SD, and 3 PA, according to a consensus criteria for clinical syndromes associated with FTLD) and 7 age-matched healthy controls using SPM99. Patients with FTD had metabolic deficit in the left frontal cortex and bilateral anterior temporal cortex. Hypometabolism in the bilateral premotor are was shown in patients with MND. Patients with SD had metabolic deficit in the left posterior temporal cortex including Wernicke's area, while hypometabolism in the bilateral inferior frontal gyrus including Broca's area and left angular gyrus was seen in patients with PA. These metabolic patterns were well correlated with clinical features of FTLD syndromes. These data provide a biochemical basis of clinical classification of FTLD. FDG PET may help evaluate and classify patients with FTLD.

  13. [Neuropathology of frontotemporal dementia].

    Science.gov (United States)

    Murayama, Shigeo

    2008-11-01

    Frontotemporal dementia (FTD) is a clinical phenotype of dementia, characterized by complex of clinical symptoms, including disinhibition, character change, increased appetite, sexual misconduct and language problems. Frontotemporal lobar degeneration (FTLD) is a pathological classification of neurodegenerative disorder and its core consists of Pick's disease (PiD). Historically, PiD was morphologically subclassified into three types, but recent immunocytochemical investigations defined type I as PiD with Pick bodies (three repeat tauopathy), type II as corticobasal degeneration (CBD, four repeat tauopathy) and type III as FTLD with ubiquitinated inclusions (FTLD-U). The recent progress provided an evidence that the majority of FTLD-U represented primary TDP 43 proteionopathy. Three major clinical phenotypes of FTLD consist of FTD, semantic dementia (SD) and progressive non-fluent aphasia (PNFA). Clinical and pathological correlative studies demonstrated that majority of the background pathology of FTD is PiD with Pick bodies, that of SD is FTLD-U and that of PNFA is CBD, although there are too many exceptions. Although FTD is one of the major clinical manifestations of FTLD, the most frequent pathological background of FTD is Alzheimer disease (AD). The degenerative processes causing FTD symptoms include dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) and argyrophilic grain disease. Moreover, vascular process such as Binswanger disease and inflammatory process such as neurosyphilis could also present with FTD symptoms. Since FTD requires special clinical care distinct from AD, clinical diagnosis of FTD is quite important. But for the fundamental treatment based on background pathological processes, surrogate biomarkers, including structural and functional neuroimages and findings of cerebrospinal fluid, blood and urine, should be pursued for future progress in FTD research.

  14. Frontotemporal dementia: An updated overview

    OpenAIRE

    Mohandas, E.; Rajmohan, V.

    2009-01-01

    Frontotemporal dementia (FTD) is a progressive neurodegenerative syndrome occurring between 45 and 65 years. The syndrome is also called frontotemporal lobar degeneration (FTLD). However, FTLD refers to a larger group of disorders FTD being one of its subgroups. The other subgroups of FTLD are progressive nonfluent aphasia (PFNA), and semantic dementia (SD). FTLD is characterized by atrophy of prefrontal and anterior temporal cortices. FTD occurs in 5-15% of patients with dementia and it is t...

  15. Auditory conflict and congruence in frontotemporal dementia.

    Science.gov (United States)

    Clark, Camilla N; Nicholas, Jennifer M; Agustus, Jennifer L; Hardy, Christopher J D; Russell, Lucy L; Brotherhood, Emilie V; Dick, Katrina M; Marshall, Charles R; Mummery, Catherine J; Rohrer, Jonathan D; Warren, Jason D

    2017-09-01

    Impaired analysis of signal conflict and congruence may contribute to diverse socio-emotional symptoms in frontotemporal dementias, however the underlying mechanisms have not been defined. Here we addressed this issue in patients with behavioural variant frontotemporal dementia (bvFTD; n = 19) and semantic dementia (SD; n = 10) relative to healthy older individuals (n = 20). We created auditory scenes in which semantic and emotional congruity of constituent sounds were independently probed; associated tasks controlled for auditory perceptual similarity, scene parsing and semantic competence. Neuroanatomical correlates of auditory congruity processing were assessed using voxel-based morphometry. Relative to healthy controls, both the bvFTD and SD groups had impaired semantic and emotional congruity processing (after taking auditory control task performance into account) and reduced affective integration of sounds into scenes. Grey matter correlates of auditory semantic congruity processing were identified in distributed regions encompassing prefrontal, parieto-temporal and insular areas and correlates of auditory emotional congruity in partly overlapping temporal, insular and striatal regions. Our findings suggest that decoding of auditory signal relatedness may probe a generic cognitive mechanism and neural architecture underpinning frontotemporal dementia syndromes. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  16. Borders of left gastric lymph node area in 124 patients with esophageal and gastric cardia carcinoma

    International Nuclear Information System (INIS)

    Qian Pudong; Guo Yesong; Li Jianzhong; Wang Yufen; Feng Chunwei; Lv Hong; Fei Wenlong

    2006-01-01

    Objective: To measure and define the distribution of left gastric lymph nodes. Methods: From Jan. 2004 to Apr. 2005, silver clips were set around the root of the left gastric artery in 124 patients with esophageal and gastric cardia carcinoma, X-ray films at 0 degree and 90 degree simulator gantry in the radio- therapeutic position were taken. Then, the data of the superior, lower, left, right, anterior and posterior bor- der in each patient was recorded. With SAS 8.02 software, data of minimum area which covered the left gastric lymph node in different incidences were obtained. Results: According to the analysis of Shapiro-Wilk, Kolmogorov-Smimov, Cramervon Mises and Anderson-Darling tests, each border' was of normal distribution, with equal frequency in the male and female, despite the actual results in different genders. Pearson Correlation Coefficients analysis did not suggest a significant relationship between the border and height, weight and size of vertebrae, which formed the minimum area covering the left gastric area at frequency of 100%, 95%, 90% and 85%, which were drawn out through the calculation. Conclusions: Aiming at completely identifying the normal distribution of the left gastric lymph node, more patients are required to be in the pool. For the time being, location in the left gastric area can be obtained from details of the results in the present study. (authors)

  17. Individual structural differences in left inferior parietal area are associated with schoolchildrens’ arithmetic scores

    Directory of Open Access Journals (Sweden)

    Yongxin eLi

    2013-12-01

    Full Text Available Arithmetic skill is of critical importance for academic achievement, professional success and everyday life, and childhood is the key period to acquire this skill. Neuroimaging studies have identified that left parietal regions are a key neural substrate for representing arithmetic skill. Although the relationship between functional brain activity in left parietal regions and arithmetic skill has been studied in detail, it remains unclear about the relationship between arithmetic achievement and structural properties in left inferior parietal area in schoolchildren. The current study employed a combination of voxel-based morphometry (VBM for high-resolution T1-weighted images and fiber tracking on diffusion tensor imaging (DTI to examine the relationship between structural properties in the inferior parietal area and arithmetic achievement in 10-year-old schoolchildren. VBM of the T1-weighted images revealed that individual differences in arithmetic scores were significantly and positively correlated with the grey matter (GM volume in the left intraparietal sulcus (IPS. Fiber tracking analysis revealed that the forceps major, left superior longitudinal fasciculus (SLF, bilateral inferior longitudinal fasciculus (ILF and inferior fronto-occipital fasciculus (IFOF were the primary pathways connecting the left IPS with other brain areas. Furthermore, the regression analysis of the probabilistic pathways revealed a significant and positive correlation between the fractional anisotropy (FA values in the left SLF, ILF and bilateral IFOF and arithmetic scores. The brain structure-behavior correlation analyses indicated that the GM volumes in the left IPS and the FA values in the tract pathways connecting left IPS were both related to children’s arithmetic achievement. The present findings provide evidence that individual structural differences in the left IPS are associated with arithmetic scores in schoolchildren.

  18. Bradycardia in frontotemporal dementia.

    Science.gov (United States)

    Robles Bayón, A; Gude Sampedro, F; Torregrosa Quesada, J M

    2014-03-01

    Numerous regions of the brain, such as the medial frontal cortex, orbitofrontal cortex, insula, and amygdala, participate in the autonomic control of cardiovascular functions such as heart rate. The degenerative process in frontotemporal dementia (FTD) involves the listed anatomical structures and may therefore produce dysautonomic cardiovascular symptoms. To observe whether or not non-cardiogenic bradycardia was more frequent in a group of patients with FTD than in subjects with mild cognitive impairment or dementia of a different aetiology. Once patients with primary cardiac arrhythmia were excluded, we registered the heart rates of 258 patients with cognitive symptoms (36 with FTD, 22 with Alzheimer disease, 23 with vascular dementia, 10 with other dementias, and 167 with non-dementia cognitive impairment). Bradycardia (<60 beats/minute) was significantly more frequent in patients with FTD. This difference remained significant after excluding subjects undergoing treatment with a potentially bradycardic effect. Bradycardia was more prevalent in behavioural FTD cases than in cases of the aphasic variant, and we detected a trend toward higher frequency among patients with more pronounced right hemisphere atrophy. Moreover, mean systolic blood pressure in FTD patients was lower than in other participants, and systolic hypotension (<120 and <100mm Hg) was more prevalent. Bradycardia was more frequent in the FTD sample than in other patients with cognitive symptoms. Further investigations will be necessary before we may consider bradycardia to be a sign supporting diagnosis of FTD or its behavioural variant. Copyright © 2012 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  19. The role of the left Brodmann's areas 44 and 45 in reading words and pseudowords

    OpenAIRE

    Heim, S.; Alter, K.; Ischebeck, A.; Amunts, K.; Eickhoff, S.; Mohlberg, H.; Zilles, K.; von Cramon, D.; Friederici, A.

    2005-01-01

    In this functional magnetic resonance imaging (fMRI) study, we investigated the influence of two task (lexical decision, LDT; phonological decision, PDT) on activation in Broca's region (left Brodmann's areas [BA] 44 and 45) during the processing of visually presented words and pseudowords. Reaction times were longer for pseudowords than words in LDT but did not differ in PDT. By combining the fMRI data with cytoarchitectonic anatomical probability maps, we demonstrated that the left BA 44 an...

  20. The brain basis of musicophilia: evidence from frontotemporal lobar degeneration

    Directory of Open Access Journals (Sweden)

    Phillip David Fletcher

    2013-06-01

    Full Text Available Musicophilia, or abnormal craving for music, is a poorly understood phenomenon that has been associated in particular with focal degeneration of the temporal lobes. Here we addressed the brain basis of musicophilia using voxel-based morphometry (VBM on MR volumetric brain images in a retrospectively ascertained cohort of patients meeting clinical consensus criteria for frontotemporal lobar degeneration: of 37 cases ascertained, 12 had musicophilia and 25 did not exhibit the phenomenon. The syndrome of semantic dementia was relatively over-represented among the musicophilic subgroup. A VBM analysis revealed significantly increased regional grey matter volume in left posterior hippocampus in the musicophilic subgroup relative to the non-musicophilic group (p<0.05 corrected for regional comparisons; at a relaxed significance threshold (P<0.001 uncorrected across the brain volume musicophilia was associated with additional relative sparing of regional grey matter in other temporal lobe and prefrontal areas and atrophy of grey matter in posterior parietal and orbitofrontal areas. The present findings suggest a candidate brain substrate for musicophilia as a signature of distributed network damage that may reflect a shift of hedonic processing toward more abstract (non-social stimuli, with some specificity for particular neurodegenerative pathologies.

  1. Frontotemporal dementia and its subtypes

    DEFF Research Database (Denmark)

    Ferrari, Raffaele; Hernandez, Dena G; Nalls, Michael A

    2014-01-01

    BACKGROUND: Frontotemporal dementia (FTD) is a complex disorder characterised by a broad range of clinical manifestations, differential pathological signatures, and genetic variability. Mutations in three genes-MAPT, GRN, and C9orf72-have been associated with FTD. We sought to identify novel gene...

  2. Apathy and disinhibition in frontotemporal dementia

    Science.gov (United States)

    Zamboni, G; Huey, E D.; Krueger, F; Nichelli, P F.; Grafman, J

    2008-01-01

    Background: Aberrant social behavior is a defining symptom of frontotemporal dementia (FTD) and may eventually occur in all syndromes composing the FTD spectrum. Two main behavioral abnormalities have been described: apathy and disinhibition, but their neuroanatomical correlates remain underspecified. Methods: Sixty-two patients with a clinical diagnosis of FTD participated in the study. Voxel-based morphometry of MRI data was performed to explore the association between gray matter loss and severity of the two behavioral profiles as measured by the Apathy and Disinhibition subscales of the Frontal Systems Behavior Scale. Results: Compared with a group of controls, the FTD group showed extensive bilateral atrophy predominantly involving frontal and temporal lobes. Within the FTD group, the severity of apathy correlated with atrophy in the right dorsolateral prefrontal cortex. The severity of disinhibition correlated with atrophy in the right nucleus accumbens, right superior temporal sulcus, and right mediotemporal limbic structures. Conclusions: Prefrontal and temporal regions are differentially associated with apathy and disinhibition. Our results support the view that successful execution of complex social behaviors relies on the integration of social knowledge and executive functions, represented in the prefrontal cortex, and reward attribution and emotional processing, represented in mesolimbic structures. GLOSSARY ACC = anterior cingulate cortex; BA = Brodmann area; DLPFC = dorsolateral prefrontal cortex; FrSBe = Frontal Systems Behavior Scale; FTD = frontotemporal dementia; FWE = family-wise error; Mattis-DRS = Mattis Dementia Rating Scale; NPI = Neuropsychiatric Inventory; NS = not significant; OFC = orbitofrontal cortex; VBM = voxel-based morphometry. PMID:18765649

  3. Genetics Home Reference: GRN-related frontotemporal dementia

    Science.gov (United States)

    ... Conditions GRN-related frontotemporal dementia GRN-related frontotemporal dementia Printable PDF Open All Close All Enable Javascript ... the expand/collapse boxes. Description GRN -related frontotemporal dementia is a progressive brain disorder that can affect ...

  4. To leave an area after disaster: how evacuees from the WTC buildings left the WTC area following the attacks.

    Science.gov (United States)

    Zimmerman, Rae; Sherman, Martin F

    2011-05-01

    How people leave a devastated area after a disaster is critical to understanding their ability to cope with risks they face while evacuating. Knowledge of their needs for communications about these risks is particularly crucial in planning for emergency responses. A convenience sample of 1,444 persons who survived the World Trade Center (WTC) attacks on September 11, 2001 were surveyed to ascertain their initial and ultimate destinations once they had left the buildings, how they arrived there, the role of types of obstacles they encountered, and the need for information and the seeking of other people as potential factors in influencing the process of leaving immediately. This survey was part of a larger, original survey. Results showed differences in how people traveled by mode to initial and ultimate destinations, how immediately they left the area, and factors associated with when they left. How they traveled and when they left were associated with where people lived, their tendency in times of stress to seek out other people including who they knew in the immediate area (e.g., co-workers or friends), the physical conditions surrounding them, and the importance to some of waiting for more information. Many people indicated they did not leave immediately because they had no information about where to go or what services would be available to them. Perceptions and communications about risks they were facing were reflected in the choices they considered in how and when to leave the area. These findings have numerous ramifications for understanding and guiding personal behavior in catastrophic situations. © 2010 Society for Risk Analysis.

  5. Repeatability and reproducibility of measurements obtained via two-dimensional speckle tracking echocardiography of the left atrium and time-left atrial area curve analysis in healthy dogs.

    Science.gov (United States)

    Osuga, Tatsuyuki; Nakamura, Kensuke; Lim, Sue Yee; Tamura, Yu; Kumara, Wickramasekara Rajapakshage Bandula; Murakami, Masahiro; Sasaki, Noboru; Morishita, Keitaro; Ohta, Hiroshi; Yamasaki, Masahiro; Takiguchi, Mitsuyoshi

    2013-06-01

    To evaluate left atrial phasic function in healthy dogs by means of 2-D speckle tracking echocardiography with time-left atrial area curve analysis and to assess repeatability and reproducibility of obtained measurements. 6 healthy Beagles. Each dog underwent echocardiography twice on different days (3 nonconsecutive examinations/d). Images were analyzed with offline software; area of the left atrium was automatically calculated in each frame throughout the cardiac cycle to derive time-left atrial area curves. Variables used to assess left atrial phasic function (total, passive, and active emptying area and emptying fractions and mean active and total emptying rates) were calculated. Agreement between variables measured via speckle tracking echocardiography and a manual tracing method was assessed with modified Bland-Altman analysis. Within-day and between-day coefficients of variation were determined. Mean ± SD total, passive, and active emptying fractions of the left atrium were 49.8 ± 3.5%, 277 ± 4.0%, and 30.5 ± 4.3%, respectively. Mean ± SD total and active emptying rates were 16.0 ± 2.5 cm(2)/s and 25.1 ± 4.9 cm(2)/s, respectively. Within-day and between-day coefficients of variation were canine patients.

  6. Functional MRI evaluation of supplementary motor area language dominance in right- and left-handed subjects.

    Science.gov (United States)

    Dalacorte, Amauri; Portuguez, Mirna Wetters; Maurer das Neves, Carlos Magno; Anes, Maurício; Dacosta, Jaderson Costa

    2012-01-01

    Functional magnetic resonance imaging (fMRI) is a non-invasive brain imaging technique widely used in the evaluation of the brain function that provides images with high temporal and spatial resolution. Investigation of the supplementary motor area (SMA) function is critical in the pre-surgical evaluation of neurological patients, since marked individual differences and complex overlapping with adjacent cortical areas exist, and it is important to spare the SMA from lesions when adjacent cortical tissue is surgically removed. We used fMRI to assess the activity of SMA in six right-handed and six left-handed healthy volunteers when a task requiring silent repetition of a series of words was given. Brain activation areas in each of the subjects were localized according to the standard Talairach coordinate space, and the individual voxels for each map were compared after 3D sagittal images were created and SMA was delimited. Quantitative analysis of hemispheric and bilateral SMA activation was described as mean ± standard deviation of hot points/total points. The results show that the language task induced bilateral SMA activation. Left SMA activation was significantly higher than right SMA activation in both right-handed and left-handed subjects.

  7. The role of left supplementary motor area in grip force scaling.

    Directory of Open Access Journals (Sweden)

    Olivier White

    Full Text Available Skilled tool use and object manipulation critically relies on the ability to scale anticipatorily the grip force (GF in relation to object dynamics. This predictive behaviour entails that the nervous system is able to store, and then select, the appropriate internal representation of common object dynamics, allowing GF to be applied in parallel with the arm motor commands. Although psychophysical studies have provided strong evidence supporting the existence of internal representations of object dynamics, known as "internal models", their neural correlates are still debated. Because functional neuroimaging studies have repeatedly designated the supplementary motor area (SMA as a possible candidate involved in internal model implementation, we used repetitive transcranial magnetic stimulation (rTMS to interfere with the normal functioning of left or right SMA in healthy participants performing a grip-lift task with either hand. TMS applied over the left, but not right, SMA yielded an increase in both GF and GF rate, irrespective of the hand used to perform the task, and only when TMS was delivered 130-180 ms before the fingers contacted the object. We also found that both left and right SMA rTMS led to a decrease in preload phase durations for contralateral hand movements. The present study suggests that left SMA is a crucial node in the network processing the internal representation of object dynamics although further experiments are required to rule out that TMS does not affect the GF gain. The present finding also further substantiates the left hemisphere dominance in scaling GF.

  8. Left posterior-dorsal area 44 couples with parietal areas to promote speech fluency, while right area 44 activity promotes the stopping of motor responses.

    Science.gov (United States)

    Neef, Nicole E; Bütfering, Christoph; Anwander, Alfred; Friederici, Angela D; Paulus, Walter; Sommer, Martin

    2016-11-15

    Area 44 is a cytoarchitectonically distinct portion of Broca's region. Parallel and overlapping large-scale networks couple with this region thereby orchestrating heterogeneous language, cognitive, and motor functions. In the context of stuttering, area 44 frequently comes into focus because structural and physiological irregularities affect developmental trajectories, stuttering severity, persistency, and etiology. A remarkable phenomenon accompanying stuttering is the preserved ability to sing. Speaking and singing are connatural behaviours recruiting largely overlapping brain networks including left and right area 44. Analysing which potential subregions of area 44 are malfunctioning in adults who stutter, and what effectively suppresses stuttering during singing, may provide a better understanding of the coordination and reorganization of large-scale brain networks dedicated to speaking and singing in general. We used fMRI to investigate functionally distinct subregions of area 44 during imagery of speaking and imaginary of humming a melody in 15 dextral males who stutter and 17 matched control participants. Our results are fourfold. First, stuttering was specifically linked to a reduced activation of left posterior-dorsal area 44, a subregion that is involved in speech production, including phonological word processing, pitch processing, working memory processes, sequencing, motor planning, pseudoword learning, and action inhibition. Second, functional coupling between left posterior area 44 and left inferior parietal lobule was deficient in stuttering. Third, despite the preserved ability to sing, males who stutter showed bilaterally a reduced activation of area 44 when imagine humming a melody, suggesting that this fluency-enhancing condition seems to bypass posterior-dorsal area 44 to achieve fluency. Fourth, time courses of the posterior subregions in area 44 showed delayed peak activations in the right hemisphere in both groups, possibly signaling the

  9. Perturbation of the left inferior frontal gyrus triggers adaptive plasticity in the right homologous area during speech production

    DEFF Research Database (Denmark)

    Hartwigsen, Gesa; Saur, Dorothee; Price, Cathy J

    2013-01-01

    The role of the right hemisphere in aphasia recovery after left hemisphere damage remains unclear. Increased activation of the right hemisphere has been observed after left hemisphere damage. This may simply reflect a release from transcallosal inhibition that does not contribute to language...... hemisphere lesion. Our findings lend further support to the notion that increased activation of homologous right hemisphere areas supports aphasia recovery after left hemisphere damage....

  10. Research progress of behavioral variant frontotemporal dementia

    Directory of Open Access Journals (Sweden)

    Xiao-hua GU

    2015-07-01

    Full Text Available There is no epidemiological data of frontotemporal dementia (FTD in China. The application of updated diagnostic criteria, publishing of frontotemporal lobar degeneration (FTLD consensus in China, development of multimodal imaging and biomarkers promote the clinical understanding on behavioral variant frontotemporal dementia (bvFTD. There is still no drugs treating FTD approved by U.S. Food and Drug Administration (FDA. Multidisciplinary intervention may delay the progression of bvFTD. DOI: 10.3969/j.issn.1672-6731.2015.07.006

  11. Vestibular Impairment in Frontotemporal Dementia Syndrome

    OpenAIRE

    Nakamagoe, Kiyotaka; Kadono, Kotarou; Koganezawa, Tadachika; Takiguchi, Mao; Terada, Makoto; Yamamoto, Fumiko; Moriyama, Tetsuya; Yanagiha, Kumi; Nohara, Seitaro; Tozaka, Naoki; Miyake, Zenshi; Aizawa, Satoshi; Furusho, Kentaro; Tamaoka, Akira

    2016-01-01

    Background: No studies to date have attempted to evaluate frontotemporal lobar degeneration from the perspective of the vestibular system. Objective: The present study examined vestibular function in patients with frontotemporal dementia (FTD) clinical syndrome and evaluated whether vestibular disorders are involved in the clinical symptoms due to FTD. Methods: Fourteen patients with FTD syndrome, as well as healthy elderly controls without dementia, were included in the present study. All su...

  12. Left atrial area index predicts adverse cardiovascular events in patients with unstable angina pectoris.

    Science.gov (United States)

    Li, Yi-Fan; Li, Wei-Hong; Li, Zhao-Ping; Feng, Xin-Heng; Xu, Wei-Xian; Chen, Shao-Min; Gao, Wei

    2016-08-01

    The left atrial size has been considered as a useful marker of adverse cardiovascular outcomes. However, it is not well known whether left atrial area index (LAAI) has predictive value for prognosis in patients with unstable angina pectoris (UAP). This study was aimed to assess the association between LAAI and outcomes in UAP patients. We enrolled a total of 391 in-hospital patients diagnosed as UAP. Clinical and echocardiographic data at baseline were collected. The patients were followed for the development of adverse cardiovascular (CV) events, including hospital readmission for angina pectoris, acute myocardial infarction (AMI), congestive heart failure (CHF), stroke and all-cause mortality. During a mean follow-up time of 26.3 ± 8.6 months, 98 adverse CV events occurred (84 hospital readmission for angina pectoris, four AMI, four CHF, one stroke and five all-cause mortality). In a multivariate Cox model, LAAI [OR: 1.140, 95% CI: 1.016-1.279, P = 0.026], diastolic blood pressure (OR: 0.976, 95% CI: 0.956-0.996, P = 0.020) and pulse pressure (OR: 1.020, 95% CI: 1.007-1.034, P = 0.004) were independent predictors for adverse CV events in UAP patients. LAAI is a predictor of adverse CV events independent of clinical and other echocardiographic parameters in UAP patients.

  13. Cosmetic Frontotemporal Craniotomy Using an Osteotome: Technical Note

    Science.gov (United States)

    Sakata, Yoshinori; Hadeishi, Hiromu; Moroi, Junta

    2013-01-01

    A frontotemporal craniotomy is usually performed using a “keyhole,” made at the union of the zygomatic arch and frontal bone. Consequently, skull depression may occur postoperatively, leading to temporal area deformities and poor cosmetic results. To prevent these complications, we describe our technique for frontotemporal craniotomy using an osteotome to prevent cosmetic deformities. After the temporal muscle is dissected and reflected with the scalp flap, a total of 3 burr holes are made in the frontal and temporal bones. In the lateral greater wing of the sphenoid, where a keyhole is usually made, a bone incision is made anteriorly-posteriorly with an osteotome. The bone flap is lifted upward, and the osteotome is inserted from behind to continue the incision. At craniotomy closure, the bone flap is fixed using a cranial bone flap fixation clamp. This procedure involves almost no removal of frontal or inferior temporal bone, resulting in virtually no bone defect. The absence of skull depression or deformity in the temples postoperatively leads to excellent cosmetic results. Our technique for frontotemporal craniotomy using an osteotome does not create bone defects, and use of titanium clamps for bone flap fixation provides normal skull bone alignment. This procedure provides excellent postoperative cosmetic results. PMID:24067778

  14. Greater pre-stimulus effective connectivity from the left inferior frontal area to other areas is associated with better phonological decoding in dyslexic readers

    Directory of Open Access Journals (Sweden)

    Richard E Frye

    2010-12-01

    Full Text Available Functional neuroimaging studies suggest that neural networks that subserve reading are organized differently in dyslexic readers (DRs and typical readers (TRs, yet the hierarchical structure of these networks has not been well studied. We used Granger Causality (GC to examine the effective connectivity of the preparatory network that occurs prior to viewing a non-word stimulus that requires phonological decoding in 7 DRs and 10 TRs who were young adults. The neuromagnetic activity that occurred 500 ms prior to each rhyme trial was analyzed from sensors overlying the left and right inferior frontal areas (IFA, temporoparietal areas (TPA, and ventral occipitotemporal areas (VOTA within the low, medium, and high beta and gamma sub-bands. A mixed-model analysis determined whether connectivity to or from the left and right IFAs differed across connectivity direction (into vs. out of the IFAs, brain areas, reading group, and/or performance. Results indicated that greater connectivity in the low beta sub-band from the left IFA to other cortical areas was significantly related to better non-word rhyme discrimination in DRs but not TRs. This suggests that the left IFA is an important cortical area involved in compensating for poor phonological function in DRs. We suggest that the left IFA activates a wider-than usual network prior to each trial in the service of supporting otherwise effortful phonological decoding in DRs. The fact that the left IFA provides top-down activation to both posterior left hemispheres areas used by typical readers for phonological decoding and homologous right hemisphere areas is discussed. In contrast, within the high gamma sub-band, better performance was associated with decreased connectivity between the left IFA and other brain areas, in both reading groups. Overly strong gamma connectivity during the pre-stimulus period may interfere with subsequent transient activation and deactivation of sub-networks once the non

  15. Electroencephalographic (eeg coherence between visual and motor areas of the left and the right brain hemisphere while performing visuomotor task with the right and the left hand

    Directory of Open Access Journals (Sweden)

    Simon Brežan

    2007-09-01

    Full Text Available Background: Unilateral limb movements are based on the activation of contralateral primary motor cortex and the bilateral activation of premotor cortices. Performance of a visuomotor task requires a visuomotor integration between motor and visual cortical areas. The functional integration (»binding« of different brain areas, is probably mediated by the synchronous neuronal oscillatory activity, which can be determined by electroencephalographic (EEG coherence analysis. We introduced a new method of coherence analysis and compared coherence and power spectra in the left and right hemisphere for the right vs. left hand visuomotor task, hypothesizing that the increase in coherence and decrease in power spectra while performing the task would be greater in the contralateral hemisphere.Methods: We analyzed 6 healthy subjects and recorded their electroencephalogram during visuomotor task with the right or the left hand. For data analysis, a special Matlab computer programme was designed. The results were statistically analysed by a two-way analysis of variance, one-way analysis of variance and post-hoc t-tests with Bonferroni correction.Results: We demonstrated a significant increase in coherence (p < 0.05 for the visuomotor task compared to control tasks in alpha (8–13 Hz in beta 1 (13–20 Hz frequency bands between visual and motor electrodes. There were no significant differences in coherence nor power spectra depending on the hand used. The changes of coherence and power spectra between both hemispheres were symmetrical.Conclusions: In previous studies, a specific increase of coherence and decrease of power spectra for the visuomotor task was found, but we found no conclusive asymmetries when performing the task with right vs. left hand. This could be explained in a way that increases in coherence and decreases of power spectra reflect symmetrical activation and cooperation between more complex visual and motor brain areas.

  16. Structural changes in left fusiform areas and associated fiber connections in children with abacus training: Evidence from morphometry and tractography

    Directory of Open Access Journals (Sweden)

    Yongxin eLi

    2013-07-01

    Full Text Available Evidence supports the notion that the fusiform gyrus (FG, as an integral part of the ventral occipitotemporal junction, is involved widely in cognitive processes as perceiving faces, objects, places or words, and this region also might represent the visual form of an abacus in the abacus-based mental calculation process. The current study uses a combined voxel-based morphometry (VBM and diffusion tensor imaging (DTI analysis to test whether long-term abacus training could induce structural changes in the left FG and in the white matter (WM tracts distribution connecting with this region in school children. We found that, abacus-trained children exhibited significant smaller grey matter (GM volume than controls in the left FG. And the connectivity mapping identified left forceps major as a key pathway connecting left FG with other brain areas in the trained group, but not in the controls. Furthermore, mean fractional anisotropy (FA values within left forceps major were significantly increased in the trained group. Interestingly, a significant negative correlation was found in the trained group between the GM volume in left FG and the mean FA value in left forceps major, suggesting an inverse effect of the reported GM and WM structural changes. In the control group, a positive correlation between left FG GM volume and tract FA was found as well. This analysis visualized the group level differences in GM volume, FA and fiber tract between the abacus-trained children and the controls, and provided the first evidence that GM volume change in the left FG is intimately linked with the micro-structural properties of the left forceps major tracts. The present results demonstrate the structural changes in the left FG from the intracortical GM to the subcortical WM regions and provide insights into the neural mechanism of structural plasticity induced by abacus training.

  17. Structural changes in left fusiform areas and associated fiber connections in children with abacus training: evidence from morphometry and tractography.

    Science.gov (United States)

    Li, Yongxin; Wang, Yunqi; Hu, Yuzheng; Liang, Yurong; Chen, Feiyan

    2013-01-01

    Evidence supports the notion that the fusiform gyrus (FG), as an integral part of the ventral occipitotemporal junction, is involved widely in cognitive processes as perceiving faces, objects, places or words, and this region also might represent the visual form of an abacus in the abacus-based mental calculation process. The current study uses a combined voxel-based morphometry (VBM) and diffusion tensor imaging (DTI) analysis to test whether long-term abacus training could induce structural changes in the left FG and in the white matter (WM) tracts distribution connecting with this region in school children. We found that, abacus-trained children exhibited significant smaller gray matter (GM) volume than controls in the left FG. And the connectivity mapping identified left forceps major as a key pathway connecting left FG with other brain areas in the trained group, but not in the controls. Furthermore, mean fractional anisotropy (FA) values within left forceps major were significantly increased in the trained group. Interestingly, a significant negative correlation was found in the trained group between the GM volume in left FG and the mean FA value in left forceps major, suggesting an inverse effect of the reported GM and WM structural changes. In the control group, a positive correlation between left FG GM volume and tract FA was found as well. This analysis visualized the group level differences in GM volume, FA and fiber tract between the abacus-trained children and the controls, and provided the first evidence that GM volume change in the left FG is intimately linked with the micro-structural properties of the left forceps major tracts. The present results demonstrate the structural changes in the left FG from the intracortical GM to the subcortical WM regions and provide insights into the neural mechanism of structural plasticity induced by abacus training.

  18. Apathy and impulsivity in frontotemporal lobar degeneration syndromes.

    Science.gov (United States)

    Lansdall, Claire J; Coyle-Gilchrist, Ian T S; Jones, P Simon; Vázquez Rodríguez, Patricia; Wilcox, Alicia; Wehmann, Eileen; Dick, Katrina M; Robbins, Trevor W; Rowe, James B

    2017-06-01

    Apathy and impulsivity are common and disabling consequences of frontotemporal lobar degeneration. They cause substantial carer distress, but their aetiology remains elusive. There are critical limitations to previous studies in this area including (i) the assessment of either apathy or impulsivity alone, despite their frequent co-existence; (ii) the assessment of behavioural changes within single diagnostic groups; and (iii) the use of limited sets of tasks or questions that relate to just one aspect of these multifactorial constructs. We proposed an alternative, dimensional approach that spans behavioural and language variants of frontotemporal dementia, progressive supranuclear palsy and corticobasal syndrome. This accommodates the commonalities of apathy and impulsivity across disorders and reveals their cognitive and anatomical bases. The ability to measure the components of apathy and impulsivity and their associated neural correlates across diagnostic groups would provide better novel targets for pharmacological manipulations, and facilitate new treatment strategies and strengthen translational models. We therefore sought to determine the neurocognitive components of apathy and impulsivity in frontotemporal lobar degeneration syndromes. The frequency and characteristics of apathy and impulsivity were determined by neuropsychological and behavioural assessments in 149 patients and 50 controls from the PIck's disease and Progressive supranuclear palsy Prevalence and INcidence study (PiPPIN). We derived dimensions of apathy and impulsivity using principal component analysis and employed these in volumetric analyses of grey and white matter in a subset of 70 patients (progressive supranuclear palsy, n = 22; corticobasal syndrome, n = 13; behavioural variant, n = 14; primary progressive aphasias, n = 21) and 27 control subjects. Apathy and impulsivity were present across diagnostic groups, despite being criteria for behavioural variant frontotemporal dementia

  19. Genetics Home Reference: CHMP2B-related frontotemporal dementia

    Science.gov (United States)

    ... Conditions CHMP2B-related frontotemporal dementia CHMP2B-related frontotemporal dementia Printable PDF Open All Close All Enable Javascript ... the expand/collapse boxes. Description CHMP2B -related frontotemporal dementia is a progressive brain disorder that affects personality, ...

  20. Mentalising music in frontotemporal dementia.

    Science.gov (United States)

    Downey, Laura E; Blezat, Alice; Nicholas, Jennifer; Omar, Rohani; Golden, Hannah L; Mahoney, Colin J; Crutch, Sebastian J; Warren, Jason D

    2013-01-01

    Despite considerable recent interest, the biological basis and clinical diagnosis of behavioural variant frontotemporal dementia (bvFTD) pose unresolved problems. Mentalising (the cognitive capacity to interpret the behaviour of oneself and others in terms of mental states) is impaired as a prominent feature of bvFTD, consistent with involvement of brain regions including ventro-medial prefrontal cortex (PFC), orbitofrontal cortex and anterior temporal lobes. Here, we investigated mentalising ability in a cohort of patients with bvFTD using a novel modality: music. We constructed a novel neuropsychological battery requiring attribution of affective mental or non-mental associations to musical stimuli. Mentalising performance of patients with bvFTD (n = 20) was assessed in relation to matched healthy control subjects (n = 20); patients also had a comprehensive assessment of behaviour and general neuropsychological functions. Neuroanatomical correlates of performance on the experimental tasks were investigated using voxel-based morphometry of patients' brain magnetic resonance imaging (MRI) scans. Compared to healthy control subjects, patients showed impaired ability to attribute mental states but not non-mental characteristics to music, and this deficit correlated with performance on a standard test of social inference and with carer ratings of patients' empathic capacity, but not with other potentially relevant measures of general neuropsychological function. Mentalising performance in the bvFTD group was associated with grey matter changes in anterior temporal lobe and ventro-medial PFC. These findings suggest that music can represent surrogate mental states and the ability to construct such mental representations is impaired in bvFTD, with potential implications for our understanding of the biology of bvFTD and human social cognition more broadly. Copyright © 2012 Elsevier Ltd. All rights reserved.

  1. Occupation attributes relate to location of atrophy in frontotemporal lobar degeneration

    OpenAIRE

    Spreng, R. Nathan; Rosen, Howard J.; Strother, Stephen; Chow, Tiffany W.; Diehl-Schmid, Janine; Freedman, Morris; Graff-Radford, Neill R.; Hodges, John R.; Lipton, Anne M.; Mendez, Mario F.; Morelli, Sylvia A.; Black, Sandra E.; Miller, Bruce L.; Levine, Brian

    2010-01-01

    Frontotemporal lobar degeneration (FTLD) often presents with asymmetric atrophy. We assessed whether premorbid occupations in FTLD patients were associated with these hemispheric asymmetries. In a multi-center chart review of 588 patients, occupation information was related to location of tissue loss or dysfunction. Patients with atrophy lateralized to the right had professions more dependent on verbal abilities than patients with left-lateralized or symmetrical atrophy. In a subgroup of 96 w...

  2. Pre-surgical evaluation of the cerebral tumor in the left language related areas by functional MRI

    International Nuclear Information System (INIS)

    Zou Zhitong; Ma Lin; Weng Xuchu

    2010-01-01

    Objective: To evaluate the application of combination of BOLD-fMRI and diffusion tensor tractography (DTT) in pre-operative evaluation of cerebral tumors located at the left language related areas. Methods: A non-vocal button pressing semantic judging paradigm was developed and validated in 10 right-handed volunteers at 3 T. After validation, this protocol combined with DTI were applied to 15 patients with left cerebral tumor prior to surgical resection, and 3 of them had aphasia. fMRI data analysis was on subject-specific basis by one-sampled t-test. The distance from the tumor to Broca area and pre-central 'hand-knot' area were measured separately. Functional language laterality index (LI) was calculated by taking out Broca area and Wernicke area. Three dimensional architecture of frontal lobe white matter fibers, especially arcuate fasciculus, were visualized using diffusion tensor tractography on Volume-one software. The images demonstrating relationship among tumor, language activation areas and white matter fibers were reviewed by neurosurgeons as part of pre-operative planning. One year after the operation, patients were followed up with MRI and language function test. Results: The non-vocal semantic judging paradigm successfully detect Broca area, Wernicke area and pre-central 'hand-knot' area. In 12 of 15 patients, the relationship of Broca area and pre-central motor area to the left brain tumor in language related areas was identified, which make the pre-operative neurosurgical plan applicable to minimize the disruption of language and motor. 8 patients had the left language dominant hemisphere, 3 patients with the right language dominant hemisphere and 1 patient with bilateral dominance. The other 3 patients' fMRI data were corrupted by patients' motion. Diffusion tensor images were corrupted by motion in 1 patient but demonstrated the impact of tumor on left accouter fasciculus in 14 patients. Diffusion tensor tractography showed disruption of left

  3. Comparison of neurodegeneration between right and left hippocampus area in rats

    Directory of Open Access Journals (Sweden)

    Arezo Nahavandi

    2015-02-01

    Conclusion: Our study showed different manifestations of depression after UCMS. It showed that UCMS could lead to mental depression. This study showed that the right hippocampus was more sensitive to stress than the left hippocampus. In fact, UCMS resulted in depression. The study showed that the right hippocampus was more sensitive to stress than the left hippocampus. Therefore, the main function of the right hemisphere, which is adaptation to the new environment, is disturbed more.

  4. Frontotemporal dementia and primary progressive aphasia, a review

    Directory of Open Access Journals (Sweden)

    Kirshner HS

    2014-06-01

    Full Text Available Howard S KirshnerDepartment of Neurology, Vanderbilt University Medical Center, Nashville, TN, USAAbstract: Frontotemporal dementias are neurodegenerative diseases in which symptoms of frontal and/or temporal lobe disease are the first signs of the illness, and as the diseases progress, they resemble a focal left hemisphere process such as stroke or traumatic brain injury, even more than a neurodegenerative disease. Over time, some patients develop a more generalized dementia. Four clinical subtypes characterize the predominant presentations of this illness: behavioral or frontal variant FTD, progressive nonfluent aphasia, semantic dementia, and logopenic primary progressive aphasia. These clinical variants correlate with regional patterns of atrophy on brain imaging studies such as MRI and PET scanning, as well as with biochemical and molecular genetic variants of the disorder. The treatment is as yet only symptomatic, but advances in molecular genetics promise new therapies.Keywords: FTD, behavior variant or frontal variant FTD, pick's disease, PPA, progressive nonfluent aphasia

  5. Burdening Care: A Study on Informal Caregivers of Frontotemporal Dementia Patients

    NARCIS (Netherlands)

    S.R. Riedijk (Samantha)

    2009-01-01

    textabstractFrontotemporal dementia (FTD) is a degenerative disease of the brain. The frontal areas of the brain that are affected by degeneration of neurones and accumulation of tau and other inclusion material control personality, social conduct, speech and language, organization and reasoning.

  6. My belief or yours? Differential theory of mind deficits in frontotemporal dementia and Alzheimer's disease.

    Science.gov (United States)

    Le Bouc, Raphaël; Lenfant, Pierre; Delbeuck, Xavier; Ravasi, Laura; Lebert, Florence; Semah, Franck; Pasquier, Florence

    2012-10-01

    reasoning; patients with Alzheimer's disease had a predominant deficit in inferring someone else's belief, whereas patients with behavioural variant frontotemporal dementia were selectively impaired in inhibiting their own mental perspective. Moreover, inhibiting one's own perspective was strongly correlated with inhibition in a Stroop task but not with other subprocesses of executive functions. This finding suggests that self-perspective inhibition may depend on cognitive processes that are not specific to the social domain. Last, the severity of the deficit in inferring someone else's beliefs correlated significantly over all subjects with hypometabolism in the left temporoparietal junction, whereas the severity of the deficit in self-perspective inhibition correlated significantly with hypometabolism in the right lateral prefrontal cortex. In conclusion, our findings provided clinical and imaging evidence to support differential deficits in two components of theory of mind reasoning (subserved by distinct brain regions) in patients with Alzheimer's disease and patients with behavioural variant frontotemporal dementia.

  7. Longitudinal Alterations of Frontoparietal and Frontotemporal Networks Predict Future Creative Cognitive Ability.

    Science.gov (United States)

    Chen, Qunlin; Beaty, Roger E; Wei, Dongtao; Yang, Junyi; Sun, Jiangzhou; Liu, Wei; Yang, Wenjing; Zhang, Qinglin; Qiu, Jiang

    2018-01-01

    Creative cognition is important to academic performance and career success during late adolescence and adulthood. However, there is a lack of longitudinal data on whether brain structural development could predict improvements in creative thinking, and how such changes interact with other cognitive abilities to support creative performance. Here we examined longitudinal alterations of brain structure and their relation to creative cognitive ability in a sample of 159 healthy young adults who were scanned using magnetic resonance imaging 2-3 times over the course of 3 years. The most robust predictor of future creative ability was the right dorsolateral prefrontal cortex (DLPFC), which in conjunction with baseline creative capacity showed a 31% prediction rate. Longitudinal analysis revealed that slower decreases in gray matter density within left frontoparietal and right frontotemporal clusters predicted enhanced creative ability. Moreoever, the relationship between longitudinal alterations within frontal-related clusters and improved creative ability was moderated by the right DLPFC and working memory ability. We conclude that continuous goal-directed planning and accumulated knowledge are implemented in the right DLPFC and temporal areas, respectively, which in turn support longitudinal gains in creative cognitive ability. © The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

  8. [Pathological gambling and epilepsy in patients with frontotemporal dementia: Two case reports].

    Science.gov (United States)

    Castro-Suarez, Sheila; Martinez, Peggy; Meza, María

    2016-01-01

    Frontotemporal dementia is a neurodegenerative disorder of which the behavioral variant is most common. This condition is currently considered the most common cause of dementia in people younger than 60 years. Here, we present two unrelated cases in which the typical symptoms were cognitive and behavioral progressive deterioration and psychiatric disorders such as disinhibition, impulsive acts, apathy, lack of empathy, stereotypies, and changes in eating habits. The first case exhibited pathological gambling as the initial symptom and resided in a psychiatric facility for a year. Notably, this was the second such case in Latin America and one of only a few such cases reported worldwide. The second case presented with epileptic seizures during evolution. In both cases, brain magnetic resonance revealed left-predominant frontotemporal atrophy, and alterations in executive function were evident during neuropsychological assessments.

  9. Neuroimaging Correlates of Frontotemporal Dementia Associated with SQSTM1 Mutations.

    Science.gov (United States)

    Luis, Elkin; Ortiz, Alexandra; Eudave, Luis; Ortega-Cubero, Sara; Borroni, Barbara; van der Zee, Julie; Gazzina, Stefano; Caroppo, Paola; Rubino, Elisa; D'Agata, Federico; Le Ber, Isabelle; Santana, Isabel; Cunha, Gil; Almeida, Maria R; Boutoleau-Bretonnière, Claire; Hannequin, Didier; Wallon, David; Rainero, Innocenzo; Galimberti, Daniela; Van Broeckhoven, Christine; Pastor, Maria A; Pastor, Pau

    2016-05-07

    Frontotemporal lobar degeneration (FTLD) is a progressive dementia characterized by focal atrophy of frontal and/or temporal lobes caused by mutations in the gene coding for sequestosome 1 (SQSTM1), among other genes. Rare SQSTM1 gene mutations have been associated with Paget's disease of bone, amyotrophic lateral sclerosis, and, more recently, frontotemporal lobar degeneration (FTLD). The aim of the study was to determine whether a characteristic pattern of grey and white matter loss is associated with SQSTM1 dysfunction. We performed a voxel-based morphometry (VBM) study in FTD subjects carrying SQSTM1 pathogenic variants (FTD/SQSTM1 mutation carriers; n = 10), compared with FTD subjects not carrying SQSTM1 mutations (Sporadic FTD; n = 20) and healthy controls with no SQSTM1 mutations (HC/SQSTM1 noncarriers; n = 20). The groups were matched according to current age, disease duration, and gender. After comparing FTD/SQSTM1 carriers with Sporadic FTD, a predominantly right cortical atrophy pattern was localized in the inferior frontal, medial orbitofrontal, precentral gyri, and the anterior insula. White matter atrophy was found in both medial and inferior frontal gyri, pallidum, and putamen. FTD/SQSTM1 carriers compared with HC/SQSTM1 noncarriers showed atrophy at frontal, temporal, and parietal lobes of both hemispheres whereas the MRI pattern found in Sporadic FTD compared with controls was frontal and left temporal lobe atrophy, extending toward parietal and occipital lobes of both hemispheres. These results suggest that fronto-orbito-insular regions including corticospinal projections as described in ALS are probably more susceptible to the damaging effect of SQSTM1 mutations delineatinga specific gene-linked atrophy pattern.

  10. [Frontotemporal dementia non familial and generalized epilepsy].

    Science.gov (United States)

    Fuentes, Patricio; Slachevsky, Andrea; Reyes, Pablo; Cartier, Luis

    2005-12-01

    A 62 year-old patient is presented, without family antecedents of dementia who begins with 45 years of age with sudden crisis of dyspnea, blurred vision, fall to the floor and repetitive jerks of arms. Tried by epilepsy with phenytoin and valproate repeats similar crisis sporadically. Four years ago their relatives began to notice changes of personality, irritability and obsessive behaviors. Later on, are added episodes of disorientation of days of duration, some with auditory hallucinations and also convulsive manifestations. Finally appear polymorphic crisis, some with continue alteration of consciousness, catatonic states and sphincteric incontinence. Physical and neurological examination without abnormalities. Neuropsychological evaluation evidenced consistent defects in frontal functions. EEG showed widespread slowness and sporadic irritative activity in frontotemporal regions. Anterior cortical atrophy in CT scan and bilateral frontotemporal hypoperfusion in SPECT. Labs exams and CSF were normal. The association of FTD and epilepsy, in non-family form, suggests a different neurodegenerative cortical syndrome.

  11. Frontotemporal dementia and neurocysticercosis: a case report

    Directory of Open Access Journals (Sweden)

    Corina Satler

    Full Text Available ABSTRACT We report a case of a 67-year-old woman with frontotemporal dementia (FTD and a history of neurocysticercosis. After her retirement she showed progressive behavioral changes and neuropsychiatric symptoms with relative preservation of cognitive functioning. During the next three years, the patient manifested progressive deterioration of verbal communication gradually evolving to mutism, a hallmark of cases of progressive nonfluent aphasia.

  12. Importance of the left auditory areas in chord discrimination in music experts as demonstrated by MEG.

    Science.gov (United States)

    Tervaniemi, Mari; Sannemann, Christian; Noyranen, Maiju; Salonen, Johanna; Pihko, Elina

    2011-08-01

    The brain basis behind musical competence in its various forms is not yet known. To determine the pattern of hemispheric lateralization during sound-change discrimination, we recorded the magnetic counterpart of the electrical mismatch negativity (MMNm) responses in professional musicians, musical participants (with high scores in the musicality tests but without professional training in music) and non-musicians. While watching a silenced video, they were presented with short sounds with frequency and duration deviants and C major chords with C minor chords as deviants. MMNm to chord deviants was stronger in both musicians and musical participants than in non-musicians, particularly in their left hemisphere. No group differences were obtained in the MMNm strength in the right hemisphere in any of the conditions or in the left hemisphere in the case of frequency or duration deviants. Thus, in addition to professional training in music, musical aptitude (combined with lower-level musical training) is also reflected in brain functioning related to sound discrimination. The present magnetoencephalographic evidence therefore indicates that the sound discrimination abilities may be differentially distributed in the brain in musically competent and naïve participants, especially in a musical context established by chord stimuli: the higher forms of musical competence engage both auditory cortices in an integrative manner. © 2011 The Authors. European Journal of Neuroscience © 2011 Federation of European Neuroscience Societies and Blackwell Publishing Ltd.

  13. Cognitive Reserve Hypothesis in Frontotemporal Dementia: Evidence from a Brain SPECT Study in a Series of Greek Frontotemporal Dementia Patients.

    Science.gov (United States)

    Maiovis, Pantelis; Ioannidis, Panagiotis; Gerasimou, Georgios; Gotzamani-Psarrakou, Anna; Karacostas, Dimitrios

    2018-03-07

    Cognitive reserve (CR) mediates the clinical expression of brain pathology in Alzheimer's disease, while there are much less relevant data in frontotemporal dementia (FTD). In the present study we examined whether CR, measured using the Cognitive Reserve Index (CRI), correlated with regional cerebral blood flow (rCBF) in Greek FTD patients. Eighty FTD patients, i.e., 47 with behavioral variant FTD (bvFTD) and 33 with primary progressive aphasia (PPA), were enrolled into this study. CR was assessed using the CRI questionnaire, which provides a total score (CRI) and 3 subscores, i.e., CRI-education, CRI-working activity, and CRI-leisure time. The FTD-Clinical Dementia Rating Scale was used to assess the severity of dementia and a brain SPECT study was performed to measure rCBF. Finally, multiple regression analyses were conducted to explore correlations between CR indices and frontotemporal rCBF. In both the bvFTD and the PPA groups, higher scores in the CRI, CRI-education, and CRI-leisure time correlated with lower rCBF in the bilateral frontal and left temporal cortex, respectively, controlling for age, sex, time since symptom onset, and disease severity. In the present study, lifetime participation in leisure time activities was found to mitigate the burden of disease in bvFTD and PPA patients. Moreover, FTD patients with a higher educational attainment were able to cope better with greater brain damage. Determination of the most suitable activities to build an adequate level of CR is crucial for dementia prevention. © 2018 S. Karger AG, Basel.

  14. Influence of hypertensive left ventricular hypertrophy on detection of ischemic area with exercise thallium-201 myocardial scintigraphy

    International Nuclear Information System (INIS)

    Toyama, Takuji; Nishimura, Tsunehiko; Uehara, Toshiisa

    1992-01-01

    Sixty-four patients with single left anterior descending artery disease having effort angina (group A: 40 patients with hypertrophic hypertension, group B: 10 patients with hypertrophic hypertension, group C: 14 patients with non-hypertrophic hypertension) were assessed to determine the influence of hypertensive left ventricular (LV) hypertrophy on detection of ischemic area. The criterion of hypertrophy by two-dimensional echocardiography was >12 mm in the wall thickness of interventricular septal or posterior wall. Population in Group B might show low detectability in ischemic area by 201 Tl myocardial scintigraphy (positive thallium rate 60%, defect score 2.7±3.6), and high lung thallium uptake and high frequence of ECG positive among three groups. In semiquantitative analysis, the washout rate of the posterolateral wall and %RD (delayed %uptake-initial %uptake) of the septal wall in patients with Group B were lowest among three groups. However, the washout rate in the septal wall against the posterior wall, and the initial %uptake and the delayed %uptake of the septal wall were not significantly different among three groups. We could conclude that the decreased washout rate in nonischemic area with hypertensive LV hypertrophy might make the ischemic area masked. (author)

  15. Frontotemporal dementia caused by CHMP2B mutations

    DEFF Research Database (Denmark)

    Isaacs, A M; Johannsen, P; Holm, I

    2011-01-01

    CHMP2B mutations are a rare cause of autosomal dominant frontotemporal dementia (FTD). The best studied example is frontotemporal dementia linked to chromosome 3 (FTD-3) which occurs in a large Danish family, with a further CHMP2B mutation identified in an unrelated Belgian familial FTD patient...

  16. Areas of Left Perisylvian Cortex Mediate Auditory-Verbal Short-Term Memory

    Science.gov (United States)

    Koenigs, Michael; Acheson, Daniel J.; Barbey, Aron K.; Solomon, Jeffrey; Postle, Bradley R.; Grafman, Jordan

    2011-01-01

    A contentious issue in memory research is whether verbal short-term memory (STM) depends on a neural system specifically dedicated to the temporary maintenance of information, or instead relies on the same brain areas subserving the comprehension and production of language. In this study, we examined a large sample of adults with acquired brain…

  17. The frontotemporal dementia-motor neuron disease continuum.

    Science.gov (United States)

    Burrell, James R; Halliday, Glenda M; Kril, Jillian J; Ittner, Lars M; Götz, Jürgen; Kiernan, Matthew C; Hodges, John R

    2016-08-27

    Early reports of cognitive and behavioural deficits in motor neuron disease might have been overlooked initially, but the concept of a frontotemporal dementia-motor neuron disease continuum has emerged during the past decade. Frontotemporal dementia-motor neuron disease is now recognised as an important dementia syndrome, which presents substantial challenges for diagnosis and management. Frontotemporal dementia, motor neuron disease, and frontotemporal dementia-motor neuron disease are characterised by overlapping patterns of TAR DNA binding protein (TDP-43) pathology, while the chromosome 9 open reading frame 72 (C9orf72) repeat expansion is common across the disease spectrum. Indeed, the C9orf72 repeat expansion provides important clues to disease pathogenesis and suggests potential therapeutic targets. Variable diagnostic criteria identify motor, cognitive, and behavioural deficits, but further refinement is needed to define the clinical syndromes encountered in frontotemporal dementia-motor neuron disease. Copyright © 2016 Elsevier Ltd. All rights reserved.

  18. T-wave area predicts response to cardiac resynchronization therapy in patients with left bundle branch block.

    Science.gov (United States)

    Engels, Elien B; Végh, Eszter M; Van Deursen, Caroline J M; Vernooy, Kevin; Singh, Jagmeet P; Prinzen, Frits W

    2015-02-01

    Chronic heart failure patients with a left ventricular (LV) conduction delay, mostly due to left bundle branch block (LBBB), generally derive benefit from cardiac resynchronization therapy (CRT). However, 30-50% of patients do not show a clear response to CRT. We investigated whether T-wave analysis of the ECG can improve patient selection. The study population comprised 244 CRT recipients with baseline 12-lead electrocardiogram recordings. Echocardiographic response after 6-month CRT was defined as a ≥5% increase in LV ejection fraction (LVEF). Vectorcardiograms (VCGs) were constructed from the measured 12-lead ECGs using an adapted Kors algorithm on digitized ECGs. Logistic regression models indicated repolarization variables as good predictors of CRT response. The VCG-derived T-wave area predicted CRT response (odds ratio [OR] per 10 μVs increase 1.172 [P < 0.001]) even better than QRS-wave area (OR = 1.116 [P = 0.001]). T-wave area had especially predictive value in the LBBB patient group (OR = 2.77 in LBBB vs. 1.09 in non-LBBB). This predictive value persisted after adjustment of multiple covariates, such as gender, ischemia, age, hypertension, coronary artery bypass graft, and the usage of diuretics and β-blockers. In LBBB patients, the increase in LVEF was 6.1 ± 9.7% and 11.3 ± 9.1% in patients with T-wave area below and above the median value, respectively (P < 0.01). In patients with LBBB morphology of the QRS complex, a larger baseline T-wave area is an important independent predictor of LVEF increase following CRT. © 2014 Wiley Periodicals, Inc.

  19. High-Frequency Electroencephalographic Activity in Left Temporal Area Is Associated with Pleasant Emotion Induced by Video Clips

    Directory of Open Access Journals (Sweden)

    Jukka Kortelainen

    2015-01-01

    Full Text Available Recent findings suggest that specific neural correlates for the key elements of basic emotions do exist and can be identified by neuroimaging techniques. In this paper, electroencephalogram (EEG is used to explore the markers for video-induced emotions. The problem is approached from a classifier perspective: the features that perform best in classifying person’s valence and arousal while watching video clips with audiovisual emotional content are searched from a large feature set constructed from the EEG spectral powers of single channels as well as power differences between specific channel pairs. The feature selection is carried out using a sequential forward floating search method and is done separately for the classification of valence and arousal, both derived from the emotional keyword that the subject had chosen after seeing the clips. The proposed classifier-based approach reveals a clear association between the increased high-frequency (15–32 Hz activity in the left temporal area and the clips described as “pleasant” in the valence and “medium arousal” in the arousal scale. These clips represent the emotional keywords amusement and joy/happiness. The finding suggests the occurrence of a specific neural activation during video-induced pleasant emotion and the possibility to detect this from the left temporal area using EEG.

  20. Apathy: a neurocircuitry model based on frontotemporal dementia.

    Science.gov (United States)

    Ducharme, Simon; Price, Bruce H; Dickerson, Bradford C

    2018-04-01

    Apathy is a symptom shared among many neurological and psychiatric disorders. However, the underlying neurocircuitry remains incompletely understood. Apathy is one of the core features of behavioural variant frontotemporal dementia (bvFTD), a neurodegenerative disease presenting with heterogeneous combinations of socioaffective symptoms and executive dysfunction. We reviewed all neuroimaging studies of apathy in frontotemporal dementia (FTD) attempting to refine a neurocircuitry model and inform clinical definitions. Levels of apathy have been consistently shown to correlate with the severity of executive dysfunctions across a wide range of diseases, including FTD. Some authors view 'energisation'-the loss of which is central in apathy-as a core executive function. Apathy in FTD is most robustly associated with atrophy, hypometabolism and/or hypoperfusion in the dorsolateral prefrontal cortex, the anterior and middle cingulate cortex, the orbitofrontal cortex and the medial and ventromedial superior frontal gyri. Data also suggest that abnormalities in connecting white matter pathways and functionally connected more posterior cortical areas could contribute to apathy. There is a lack of consistency across studies due to small samples, lenient statistical thresholds, variable measurement scales and the focus on apathy as a unitary concept. Integrating findings across studies, we revise a neurocircuitry model of apathy divided along three subcomponents (cognition/planning, initiation, emotional-affective/motivation) with specific neuroanatomical and cognitive substrates. To increase consistency in clinical practice, a recommendation is made to modify the bvFTD diagnostic criteria of apathy/inertia. More generally, we argue that bvFTD constitutes a disease model to study the neurocircuitry of complex behaviours as a 'lesion-based' approach to neuropsychiatric symptoms observed across diagnostic categories. © Article author(s) (or their employer(s) unless otherwise

  1. Vestibular Impairment in Frontotemporal Dementia Syndrome

    Directory of Open Access Journals (Sweden)

    Kiyotaka Nakamagoe

    2016-05-01

    Full Text Available Background: No studies to date have attempted to evaluate frontotemporal lobar degeneration from the perspective of the vestibular system. Objective: The present study examined vestibular function in patients with frontotemporal dementia (FTD clinical syndrome and evaluated whether vestibular disorders are involved in the clinical symptoms due to FTD. Methods: Fourteen patients with FTD syndrome, as well as healthy elderly controls without dementia, were included in the present study. All subjects underwent vestibular function tests using electronystagmography, such as caloric tests and visual suppression (VS tests, in which the induced caloric nystagmus was suppressed by visual stimuli. The association between clinical symptoms and vestibular function in the FTD syndrome group was further examined. Results: In the FTD syndrome group, caloric nystagmus was not necessarily suppressed during VS tests. Furthermore, VS was observed to be significantly impaired in FTD syndrome patients with gait disturbance as compared to those without such disturbance. Conclusion: The present study revealed that impairment of VS in patients with FTD results in an inability to regulate vestibular function by means of visual perception, regardless of multiple presumed neuropathological backgrounds. This could also be associated with gait disturbance in patients with FTD syndrome.

  2. Vestibular Impairment in Frontotemporal Dementia Syndrome.

    Science.gov (United States)

    Nakamagoe, Kiyotaka; Kadono, Kotarou; Koganezawa, Tadachika; Takiguchi, Mao; Terada, Makoto; Yamamoto, Fumiko; Moriyama, Tetsuya; Yanagiha, Kumi; Nohara, Seitaro; Tozaka, Naoki; Miyake, Zenshi; Aizawa, Satoshi; Furusho, Kentaro; Tamaoka, Akira

    2016-01-01

    No studies to date have attempted to evaluate frontotemporal lobar degeneration from the perspective of the vestibular system. The present study examined vestibular function in patients with frontotemporal dementia (FTD) clinical syndrome and evaluated whether vestibular disorders are involved in the clinical symptoms due to FTD. Fourteen patients with FTD syndrome, as well as healthy elderly controls without dementia, were included in the present study. All subjects underwent vestibular function tests using electronystagmography, such as caloric tests and visual suppression (VS) tests, in which the induced caloric nystagmus was suppressed by visual stimuli. The association between clinical symptoms and vestibular function in the FTD syndrome group was further examined. In the FTD syndrome group, caloric nystagmus was not necessarily suppressed during VS tests. Furthermore, VS was observed to be significantly impaired in FTD syndrome patients with gait disturbance as compared to those without such disturbance. The present study revealed that impairment of VS in patients with FTD results in an inability to regulate vestibular function by means of visual perception, regardless of multiple presumed neuropathological backgrounds. This could also be associated with gait disturbance in patients with FTD syndrome.

  3. Presymptomatic studies in genetic frontotemporal dementia.

    Science.gov (United States)

    Rohrer, J D; Warren, J D; Fox, N C; Rossor, M N

    2013-10-01

    Approximately 20% of patients with the neurodegenerative disorder frontotemporal dementia (FTD) have an autosomal dominant pattern of inheritance. Genetic FTD is caused by mutations in three genes in most cases (progranulin, microtubule-associated protein tau and chromosome 9 open reading frame 72) although a number of other genes are rare causes. Studies of other neurodegenerative diseases have shown imaging and biomarker evidence of disease onset many years prior to the development of symptoms. Similar studies in genetic FTD are now revealing evidence of a series of presymptomatic changes, initially in plasma biomarkers followed by MR imaging abnormalities of functional and structural connectivity and then grey matter atrophy. Lastly, neuropsychometric tests become abnormal in proximity to the onset of symptoms. Such studies have been relatively small until now but research centres with an expertise in genetic FTD are now forming consortia such as the Genetic Frontotemporal Dementia Initiative (GenFI) to create larger cohorts that can form the basis of future clinical trials. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  4. Apathy in Frontotemporal Dementia: Behavioral and Neuroimaging Correlates

    Directory of Open Access Journals (Sweden)

    Paul J. Eslinger

    2012-01-01

    Full Text Available We investigated the occurrence of goal-directed motivational change in the form of apathy in patients with frontotemporal dementia (FTD, particularly those with behavioral variant social and executive deficits (bvFTD. Standardized behavioral inventory was employed to survey and compare apathy ratings from patients and caregivers. In cases of bvFTD, apathy ratings were further related to measures of social cognition, executive function, and atrophy on brain MRI. Results indicated that caregivers rated bvFTD patients as having significantly elevated apathy scores though patient self-ratings were normal. Caregiver and self-ratings of FTD samples with progressive nonfluent aphasia and semantic dementia did not differ from healthy controls and their informants. In the bvFTD sample, caregiver apathy scores were not correlated with general cognitive screening or depression scores, but were significantly correlated with social cognition and executive function measures. Voxel-based morphometry revealed that apathy ratings in bvFTD were related to prominent atrophy in the right caudate (including the ventral striatum, the right temporo-parietal junction, right posterior inferior and middle temporal gyri, and left frontal operculum- anterior insula region. Findings suggest that bvFTD is associated with a significant breakdown in goal-directed motivated behavior involving disruption of cortical-basal ganglia circuits that is also related to social and executive function deficits.

  5. Comparison of left and right ventricular volume measurement using the Simpson's method and the area length method

    International Nuclear Information System (INIS)

    Hergan, Klaus; Schuster, Antonius; Fruehwald, Julia; Mair, Michael; Burger, Ralph; Toepker, Michael

    2008-01-01

    Purpose: To compare ventricular volume measurement using a volumetric approach in the three standard cardiac planes and ventricular volume estimation by a geometrical model, the Area-Length method (ALM). Materials and methods: Fifty-six healthy volunteers were examined (27 males, 29 females) on a 1.5 T MR-unit with ECG-triggered steady state free precision (SSFP) Cine-MR sequences and parallel image acquisition. Multiple slices in standardized planes including the short-axis view (sa), 4-chamber view (4ch), left and right 2-chamber views (2ch) were used to cover the whole heart. End-systolic and end-diastolic ventricular volumes (EDV, ESV), stroke volume (SV), and ejection fraction (EF) were calculated with Simpson's rule in all planes and with ALM in the 2ch and 4ch planes. Global function parameters measured in the sa plane were compared with those obtained in the other imaging planes. Results: A very good correlation is observed when comparing functional parameters calculated with Simpson's rule in all imaging planes: for instance, the mean EDV/ESV of the left and right ventricle of the female population group measured in sa, 4ch, and 2ch: left ventricle EDV/ESV 114.3/44.4, 120.9/46.5, and 117.7/45.3 ml; right ventricle EDV/ESV 106.6/46.0, 101.2/41.1, and 103.5/43.0 ml. Functional parameters of the left ventricle calculated with ALM in 2ch and 4ch correlate to parameters obtained in sa with Simpson's rule in the range of 5-10%: for instance, the EDV/ESV of the left ventricle of the male population group measured in the sa, 4ch, and 2ch: 160.3/63.5, 163.1/59.0, and 167.0/65.7 ml. Functional parameters of the right ventricle measured with ALM in 4ch are 40-50% lower and calculated in 2ch almost double as high as compared with the parameters obtained in sa with Simpson's rule: for instance, male right ventricular EDV/ESV measured in sa, 4ch, and 2ch: 153.4/68.1, 97.5/34.5, and 280.2/123.2 ml. The EF correlates for all imaging planes measured with the Simpson's rule

  6. Comparison of left and right ventricular volume measurement using the Simpson's method and the area length method

    Energy Technology Data Exchange (ETDEWEB)

    Hergan, Klaus [Department of Radiology, Paracelsus Medical University Salzburg (Austria)], E-mail: k.hergan@salk.at; Schuster, Antonius [Department of Radiology, LKH Feldkirch (Austria); Fruehwald, Julia [Department of Radiology, Medical University Vienna (Austria); Mair, Michael; Burger, Ralph; Toepker, Michael [Department of Radiology, LKH Feldkirch (Austria)

    2008-02-15

    Purpose: To compare ventricular volume measurement using a volumetric approach in the three standard cardiac planes and ventricular volume estimation by a geometrical model, the Area-Length method (ALM). Materials and methods: Fifty-six healthy volunteers were examined (27 males, 29 females) on a 1.5 T MR-unit with ECG-triggered steady state free precision (SSFP) Cine-MR sequences and parallel image acquisition. Multiple slices in standardized planes including the short-axis view (sa), 4-chamber view (4ch), left and right 2-chamber views (2ch) were used to cover the whole heart. End-systolic and end-diastolic ventricular volumes (EDV, ESV), stroke volume (SV), and ejection fraction (EF) were calculated with Simpson's rule in all planes and with ALM in the 2ch and 4ch planes. Global function parameters measured in the sa plane were compared with those obtained in the other imaging planes. Results: A very good correlation is observed when comparing functional parameters calculated with Simpson's rule in all imaging planes: for instance, the mean EDV/ESV of the left and right ventricle of the female population group measured in sa, 4ch, and 2ch: left ventricle EDV/ESV 114.3/44.4, 120.9/46.5, and 117.7/45.3 ml; right ventricle EDV/ESV 106.6/46.0, 101.2/41.1, and 103.5/43.0 ml. Functional parameters of the left ventricle calculated with ALM in 2ch and 4ch correlate to parameters obtained in sa with Simpson's rule in the range of 5-10%: for instance, the EDV/ESV of the left ventricle of the male population group measured in the sa, 4ch, and 2ch: 160.3/63.5, 163.1/59.0, and 167.0/65.7 ml. Functional parameters of the right ventricle measured with ALM in 4ch are 40-50% lower and calculated in 2ch almost double as high as compared with the parameters obtained in sa with Simpson's rule: for instance, male right ventricular EDV/ESV measured in sa, 4ch, and 2ch: 153.4/68.1, 97.5/34.5, and 280.2/123.2 ml. The EF correlates for all imaging planes measured

  7. A Single-Centre, Randomized, Double-Blind, Placebo-Controlled Clinical Trial to Investigate the Efficacy and Safety of Minoxidil Topical Foam in Frontotemporal and Vertex Androgenetic Alopecia in Men.

    Science.gov (United States)

    Hillmann, Kathrin; Garcia Bartels, Natalie; Kottner, Jan; Stroux, Andrea; Canfield, Douglas; Blume-Peytavi, Ulrike

    2015-01-01

    5% minoxidil formulations twice daily are effective in treating vertex male androgenetic alopecia (AGA); however, efficacy and safety data in frontotemporal regions are lacking. To assess the efficacy of 5% minoxidil topical foam (5% MTF) in the frontotemporal region of male AGA patients after 24 weeks of treatment compared to placebo treatment and to the vertex region. Seventy males with moderate AGA applied 5% MTF or placebo foam (plaTF) twice daily for 24 weeks in frontotemporal and vertex regions. Target area non-vellus hair count (TAHC) was the primary end point. Frontotemporal and vertex TAHC and target area cumulative non-vellus hair width (TAHW) showed similar responses to 5% MTF with significant increases up to week 16 compared to baseline (p < 0.001). After 24 weeks of treatment, frontotemporal TAHW increased significantly in the 5% MTF group compared to the plaTF group (p = 0.017), while TAHC showed a similar non-significant increase from baseline in both regions. At 24 weeks, 5% MTF users rated a significant improvement in scalp coverage for the frontotemporal (p = 0.016) and vertex areas (p = 0.027). 5% MTF twice a day promotes hair density and width in both frontotemporal and vertex regions in men with moderate stages of AGA. © 2015 S. Karger AG, Basel.

  8. Gastric bare area and left adrenal gland involvement on abdominal computed tomography and their prognostic value in acute pancreatitis

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Zaiyi [Guangdong Provincial People' s Hospital, Department of Radiology, Guangzhou, Guangdong Province (China); Sichuan University, Department of Radiology, West China Hospital, Chengdu, Sichuan Province (China); Yan, Zhihan [Wenzhou Medical College, Department of Radiology, Second Affiliated Hospital, Wenzhou, Zhejiang Province (China); Min, Pengqiu [Sichuan University, Department of Radiology, West China Hospital, Chengdu, Sichuan Province (China); Liang, Changhong [Guangdong Provincial People' s Hospital, Department of Radiology, Guangzhou, Guangdong Province (China); Wang, Ying [Sun Yatsen University, Department of Medical Ultrasonics, First Affiliated Hospital, Guangzhou, Guangdong Province (China)

    2008-08-15

    To demonstrate the CT manifestations of gastric bare area involvement (GBAI) and left adrenal gland involvement (LAGI) in acute pancreatitis (AP) and evaluate their prognostic value. From January 2003 to December 2006, CT examinations of 116 patients with AP were retrospectively reviewed. There were 34 (29.3%) patients with GBAI showing haziness and streaky density or fluid collection in the gastric bare area, and 18 (15.5%) with LAGI showing deformity and hypoattenuation of left adrenal gland. The mean duration of hospital stay in patients with GBAI and LAGI was longer than that of patients without (P<0.001). The sensitivity and specificity of GBAI for predicting complications were 43.3% (0.31, 0.55) and 89.8% (0.81, 0.98), respectively; and 83.3% (0.36, 1.00) and 73.6% (0.65, 0.82) for predicting mortality. A patient with GBAI was 6.7 (2.4, 19.1) and 14.0 (1.6, 124.6) times more likely to have complications and die than was a patient without. The sensitivity and specificity of LAGI for predicting complications were 23.9% (0.14, 0.34) and 95.9% (0.86, 0.99), respectively, and 66.7% (0.22, 0.96) and 87.3% (0.81, 0.94) for predicting mortality. A patient with LAGI was 7.4 (1.6, 33.8) and 13.7 (2.3, 81.9) times more likely to have complications and die than was a patient without. Our results showed that GBAI and LAGI were characteristic CT findings in AP and could serve as useful prognostic indicators for this disease. (orig.)

  9. Controversies over the mechanisms underlying the crucial role of the left fronto-parietal areas in the representation of tools

    Directory of Open Access Journals (Sweden)

    Guido eGainotti

    2013-10-01

    Full Text Available Anatomo-clinical and neuroimaging data show that the left fronto-parietal areas play an important role in representing tools. As manipulation is an important source of knowledge about tools, it has been assumed that motor activity explains the link between tool knowledge and the left fronto-parietal areas. However, controversies exist over the exact mechanisms underlying this relationship. According to a strong version of the ‘embodied cognition theory’, activation of a tool concept necessarily involves re-enactment of the corresponding kind of action. Impairment of the ability to use tools should, therefore, lead to impairment of tool knowledge. Both the ‘domains of knowledge hypothesis’ and the ‘sensory-motor model of conceptual knowledge’ refute the strong version of the ‘embodied cognition hypothesis’ but acknowledge that manipulation and other action schemata play an important role in our knowledge of tools. The basic difference between these two models is that the former is based on an innatist model and the latter holds that the brain’s organization of categories is experience dependent. Data supporting and arguing against each of these models are briefly reviewed. In particular, the following lines of research, which argue against the innate nature of the brain’s categorical organization, are discussed: (1 the observation that in patients with category-specific disorders the semantic impairment does not respect the boundaries between biological entities and artefact items; (2 data showing that experience-driven neuroplasticity in musicians is not confined to alterations of perceptual and motor maps but also leads to the establishment of higher-level semantic representations for musical instruments; (3 results of experiments using previously unfamiliar materials showing that the history of our sensory-motor experience with an object significantly affects its neural representation.

  10. Genetics Home Reference: frontotemporal dementia with parkinsonism-17

    Science.gov (United States)

    ... Practice Guideline for the Treatment of Patients with Alzheimer's Disease and Other Dementias (PDF) Genetic Testing (1 link) Genetic Testing Registry: Frontotemporal dementia Other Diagnosis and Management Resources (1 link) GeneReview: MAPT-Related ...

  11. Episodic memory in frontotemporal dementia: a critical review.

    Science.gov (United States)

    Hornberger, Michael; Piguet, Olivier

    2012-03-01

    This review offers a critical appraisal of the literature on episodic memory performance in frontotemporal dementia. Historically, description of patients diagnosed with what was then known as Pick's disease included the presence of memory deficits and an underlying amnestic syndrome was noted in some of these patients. Over the last 20 years, however, the clinical view has been that episodic memory processing is relatively intact in the frontotemporal dementia syndrome. In particular, patients with the subtypes of behavioural variant frontotemporal dementia and progressive non-fluent aphasia are reported to perform within normal limits on standard memory tests. In the third clinical presentation of frontotemporal dementia, semantic dementia, relatively intact episodic memory against a significantly impaired semantic memory was regarded as the hallmark. This position was instrumental in the development of clinical diagnostic criteria for frontotemporal dementia in which amnesia was explicitly listed as an exclusion criterion for the disease. The relative intactness of episodic memory, therefore, appeared to be a useful diagnostic marker to distinguish early frontotemporal dementia from Alzheimer's disease, in which early episodic memory disturbance remains the most common clinical feature. We argue that recent evidence questions the validity of preserved episodic memory in frontotemporal dementia, particularly in behavioural variant frontotemporal dementia. In semantic dementia, a complex picture emerges with preservation of some components of episodic memory, notably recognition-based visual memory and recall of recent autobiographical events. We propose a critical synthesis of recent neuropsychological evidence on retrograde and anterograde memory in light of neuroimaging and neuropathological findings, demonstrating involvement of medial temporal structures in frontotemporal dementia, structures known to be critical for episodic memory processing. We further

  12. White matter change with apathy and impulsivity in frontotemporal lobar degeneration syndromes.

    Science.gov (United States)

    Lansdall, Claire J; Coyle-Gilchrist, Ian T S; Jones, P Simon; Vázquez Rodríguez, Patricia; Wilcox, Alicia; Wehmann, Eileen; Dick, Katrina M; Robbins, Trevor W; Rowe, James B

    2018-03-20

    To identify the white matter correlates of apathy and impulsivity in the major syndromes associated with frontotemporal lobar degeneration, using diffusion-weighted imaging and data from the PiPPIN (Pick's Disease and Progressive Supranuclear Palsy: Prevalence and Incidence) study. We included behavioral and language variants of frontotemporal dementia, corticobasal syndrome, and progressive supranuclear palsy. Seventy patients and 30 controls underwent diffusion tensor imaging at 3-tesla after detailed assessment of apathy and impulsivity. We used tract-based spatial statistics of fractional anisotropy and mean diffusivity, correlating with 8 orthogonal dimensions of apathy and impulsivity derived from a principal component analysis of neuropsychological, behavioral, and questionnaire measures. Three components were associated with significant white matter tract abnormalities. Carer-rated change in everyday skills, self-care, and motivation correlated with widespread changes in dorsal frontoparietal and corticospinal tracts, while carer observations of impulsive-apathetic and challenging behaviors revealed disruption in ventral frontotemporal tracts. Objective neuropsychological tests of cognitive control, reflection impulsivity, and reward responsiveness were associated with focal changes in the right frontal lobe and presupplementary motor area. These changes were observed across clinical diagnostic groups, and were not restricted to the disorders for which diagnostic criteria include apathy and impulsivity. The current study provides evidence of distinct structural network changes in white matter associated with different neurobehavioral components of apathy and impulsivity across the diverse spectrum of syndromes and pathologies associated with frontotemporal lobar degeneration. Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

  13. Changes in regional cerebral blood flow in the right cortex homologous to left language areas are directly affected by left hemispheric damage in aphasic stroke patients: evaluation by Tc-ECD SPECT and novel analytic software.

    Science.gov (United States)

    Uruma, G; Kakuda, W; Abo, M

    2010-03-01

    The objective of this study was to clarify the influence of regional cerebral blood flow (rCBF) changes in language-relevant areas of the dominant hemisphere on rCBF in each region in the non-dominant hemisphere in post-stroke aphasic patients. The study subjects were 27 aphasic patients who suffered their first symptomatic stroke in the left hemisphere. In each subject, we measured rCBF by means of 99mTc-ethylcysteinate dimmer single photon emission computed tomography (SPECT). The SPECT images were analyzed by the statistical imaging analysis programs easy Z-score Imaging System (eZIS) and voxel-based stereotactic extraction estimation (vbSEE). Segmented into Brodmann Area (BA) levels, Regions of Interest (ROIs) were set in language-relevant areas bilaterally, and changes in the relative rCBF as average negative and positive Z-values were computed fully automatically. To assess the relationship between rCBF changes of each ROIs in the left and right hemispheres, the Spearman ranked correlation analysis and stepwise multiple regression analysis were applied. Globally, a negative and asymmetric influence of rCBF changes in the language-relevant areas of the dominant hemisphere on the right hemisphere was found. The rCBF decrease in left BA22 significantly influenced the rCBF increase in right BA39, BA40, BA44 and BA45. The results suggested that the chronic increase in rCBF in the right language-relevant areas is due at least in part to reduction in the trancallosal inhibitory activity of the language-dominant left hemisphere caused by the stroke lesion itself and that these relationships are not always symmetric.

  14. Psychosis in behavioral variant frontotemporal dementia

    Directory of Open Access Journals (Sweden)

    Gossink FT

    2017-04-01

    Full Text Available Flora T Gossink,1,2 Everard GB Vijverberg,2,3 Welmoed Krudop,2 Philip Scheltens,2 Max L Stek,1 Yolande AL Pijnenburg,1,2 Annemiek Dols1,2 1Department of Old Age Psychiatry, GGZinGeest, 2Alzheimer Center & Department of Neurology, VU University Medical Center, Amsterdam, 3Department of Neurology, HagaZiekenhuis, The Hague, the Netherlands Background: Dementia is generally characterized by cognitive impairment that can be accompanied by psychotic symptoms; for example, visual hallucinations are a core feature of dementia with Lewy bodies, and delusions are often seen in Alzheimer’s disease. However, for behavioral variant of frontotemporal dementia (bvFTD, studies on the broad spectrum of psychotic symptoms are still lacking. The aim of this study was to systematically and prospectively subtype the wide spectrum of psychotic symptoms in probable and definite bvFTD.Methods: In this study, a commonly used and validated clinical scale that quantifies the broad spectrum of psychotic symptoms (Positive and Negative Symptom Scale was used in patients with probable and definite bvFTD (n=22 and with a primary psychiatric disorder (n=35 in a late-onset frontal lobe cohort. Median symptom duration was 2.8 years, and the patients were prospectively followed for 2 years.Results: In total, 22.7% of bvFTD patients suffered from delusions, hallucinatory behavior, and suspiciousness, although the majority of the patients exhibited negative psychotic symptoms such as social and emotional withdrawal and blunted affect (95.5% and formal thought disorders (81.8%. “Difficulty in abstract thinking” and “stereotypical thinking” (formal thought disorders differentiated bvFTD from psychiatric disorders. The combined predictors difficulty in abstract thinking, stereotypical thinking, “anxiety”, “guilt feelings,” and “tension” explained 75.4% of variance in the diagnosis of bvFTD versus psychiatric diagnoses (P<0.001.Conclusion: Delusions

  15. [Left ventricular hypertrophy in black African subjects with artery hypertension: Results of a cross-sectional survey conducted in semi-rural area in Senegal].

    Science.gov (United States)

    Mbaye, A; Dodo, B; Ngaïde, A A; Sy, N F; Babaka, K; Mingou, J S; Faye, M; Niang, K; Sarr, S A; Dioum, M; Bodian, M; Ndiaye, M B; Kane, A D; Ndour-Mbaye, M; Diao, M; Diack, B; Kane, M; Diagne-Sow, D; Thiaw, I; Kane, A

    2017-09-01

    To assess the prevalence of left ventricular hypertrophy according to electrocardiographic and echocardiographic criteria among hypertensive patients living in semi-rural Senegalese area. According to the World Health Organization STEPSwise approach, we conducted, in November 2012, a cross-sectional and exhaustive study in the population aged at least 35 years old and living for at least six months in the semi-rural area of Guéoul. We researched electrocardiographic and echocardiographic left ventricular hypertrophy in hypertensive subjects. Data were analyzed with SPSS 18.0 software version. The significance level was agreed for a value of P<0.05. We examined 1411 subjects aged on average of 48.5±12.7 years. In total, 654 subjects were hypertensive and screening of left ventricular hypertrophy (LVH) was effective in 515 of them. According to Sokolow-Lyon index, 86 subjects (16.7%) presented electrocardiographic LVH, more frequently in men (P=0.002). According to Cornell index and Cornell product, LVH was founded respectively in 66 (12.8%) and 52 subjects (10.1%), more frequently in female (P=0.0001; P=0.004). It was more common in grade 3 of hypertension however criteria. In echocardiography, prevalence of LVH was 2.2% (13 cases) according to the left ventricular mass, 9.3% (48 cases) according to the left ventricular mass indexed to body surface area and 8.2% (42 cases) according to the left ventricular mass indexed to height 2.7 . LVH was significantly correlated with the electrocardiographic LVH according to Sokolow-Lyon index (P<0.0001) and the grade 3 of hypertension (P=0.003). Although rare in hypertensive Senegalese living in semi-rural area, left ventricular hypertrophy is correlated with severity of grade of hypertension. Screening by electrocardiogram will allow better follow-up of these hypertensive subjects. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  16. Behavioural-variant frontotemporal dementia: An update

    Directory of Open Access Journals (Sweden)

    Olivier Piguet

    Full Text Available ABSTRACT Behavioural-variant frontotemporal dementia (bvFTD is characterised by insidious changes in personality and interpersonal conduct that reflect progressive disintegration of the neural circuits involved in social cognition, emotion regulation, motivation and decision making. The underlying pathology is heterogeneous and classified according to the presence of intraneuronal inclusions of tau, TDP-43 or occasionally FUS. Biomarkers to detect these histopathological changes in life are increasingly important with the development of disease-modifying drugs. Gene mutations have been found which collectively account for around 10-20% of cases including a novel hexanucleotide repeat on chromosome 9 (C9orf72. The recently reviewed International Consensus Criteria for bvFTD propose three levels of diagnostic certainly: possible, probable and definite. Detailed history taking from family members to elicit behavioural features underpins the diagnostic process with support from neuropsychological testing designed to detect impairment in decision-making, emotion processing and social cognition. Brain imaging is important for increasing the level of diagnosis certainty. Carer education and support remain of paramount importance.

  17. Agomelatine Improves Apathy in Frontotemporal Dementia.

    Science.gov (United States)

    Callegari, Ilaria; Mattei, Chiara; Benassi, Francesca; Krueger, Frank; Grafman, Jordan; Yaldizli, Özgür; Sassos, Davide; Massucco, Davide; Scialò, Carlo; Nobili, Flavio; Serrati, Carlo; Amore, Mario; Cocito, Leonardo; Emberti Gialloreti, Leonardo; Pardini, Matteo

    2016-01-01

    Apathy is the most common initial symptom of frontotemporal dementia (FTD) and has been linked to frontal-subcortical dopaminergic system dysfunction. No pharmacological therapy has been approved for the treatment of apathy, but, on the basis of its physiopathological mechanism, we suspected that increasing prefrontal dopaminergic innervation could improve this disabling symptom. We evaluated a group of 24 nondepressed patients with a diagnosis of the behavioral variant of FTD, in order to determine the effectiveness on apathy of agomelatine, an antidepressant with MT1 and MT2 receptor agonism and 5-HT2C receptor antagonism; the latter leads to an increase in prefrontal dopaminergic and noradrenergic tone. To try to tease out the effects of 5-HT2C antagonism on apathy, patients were randomized, using a cross-over design, to receive either agomelatine 50 mg/day or sustained release melatonin 10 mg/day for 10 weeks in a double-blind procedure. At the end of the follow-up period, subjects receiving melatonin switched to agomelatine for the following 10 weeks. Agomelatine, but not melatonin, was associated with a significant reduction of apathy in FTD subjects and of caregiver distress due to patients' apathy. The switch from melatonin to agomelatine was associated with a reduction in apathetic behavior. Agomelatine was well-tolerated by all enrolled subjects. Our data, albeit preliminary, suggest that agomelatine could represent a novel useful approach to the treatment of apathy in FTD patients. © 2016 S. Karger AG, Basel.

  18. Progranulin in frontotemporal lobar degeneration and neuroinflammation

    Directory of Open Access Journals (Sweden)

    Hutton Michael L

    2007-02-01

    Full Text Available Abstract Progranulin (PGRN is a pleiotropic protein that has gained the attention of the neuroscience community with recent discoveries of mutations in the gene for PGRN that cause frontotemporal lobar degeneration (FTLD. Pathogenic mutations in PGRN result in null alleles, and the disease is likely the result of haploinsufficiency. Little is known about the normal function of PGRN in the central nervous system apart from a role in brain development. It is expressed by microglia and neurons. In the periphery, PGRN is involved in wound repair and inflammation. High PGRN expression has been associated with more aggressive growth of various tumors. The properties of full length PGRN are distinct from those of proteolytically derived peptides, referred to as granulins (GRNs. While PGRN has trophic properties, GRNs are more akin to inflammatory mediators such as cytokines. Loss of the neurotrophic properties of PGRN may play a role in selective neuronal degeneration in FTLD, but neuroinflammation may also be important. Gene expression studies suggest that PGRN is up-regulated in a variety of neuroinflammatory conditions, and increased PGRN expression by microglia may play a pivotal role in the response to brain injury, neuroinflammation and neurodegeneration.

  19. Neural correlates of the DemTect in Alzheimer's disease and frontotemporal lobar degeneration - A combined MRI & FDG-PET study.

    Science.gov (United States)

    Woost, Timo B; Dukart, Juergen; Frisch, Stefan; Barthel, Henryk; Sabri, Osama; Mueller, Karsten; Schroeter, Matthias L

    2013-01-01

    Valid screening devices are critical for an early diagnosis of dementia. The DemTect is such an internationally accepted tool. We aimed to characterize the neural networks associated with performance on the DemTect's subtests in two frequent dementia syndromes: early Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD). Voxel-based group comparisons of cerebral glucose utilization (as measured by F-18-fluorodeoxyglucose positron emission tomography) and gray matter atrophy (as measured by structural magnetic resonance imaging) were performed on data from 48 subjects with AD (n = 21), FTLD (n = 14) or subjective cognitive impairment (n = 13) as a control group. We performed group comparisons and correlation analyses between multimodal imaging data and performance on the DemTect's subtests. Group comparisons showed regional patterns consistent with previous findings for AD and FTLD. Interestingly, atrophy dominated in FTLD, whereas hypometabolism in AD. Across diagnostic groups performance on the "wordlist" subtest was positively correlated with glucose metabolism in the left temporal lobe. The "number transcoding" subtest was significantly associated with glucose metabolism in both a predominantly left lateralized frontotemporal network and a parietooccipital network including parts of the basal ganglia. Moreover, this subtest was associated with gray matter density in an extensive network including frontal, temporal, parietal and occipital areas. No significant correlates were observed for the "supermarket task" subtest. Scores on the "digit span reverse" subtest correlated with glucose metabolism in the left frontal cortex, the bilateral putamen, the head of caudate nucleus and the anterior insula. Disease-specific correlation analyses could partly verify or extend the correlates shown in the analyses across diagnostic groups. Correlates of gray matter density were found in FTLD for the "number transcoding" subtest and the "digit span

  20. Neural correlates of the DemTect in Alzheimer's disease and frontotemporal lobar degeneration – A combined MRI & FDG-PET study☆

    Science.gov (United States)

    Woost, Timo B.; Dukart, Juergen; Frisch, Stefan; Barthel, Henryk; Sabri, Osama; Mueller, Karsten; Schroeter, Matthias L.

    2013-01-01

    Valid screening devices are critical for an early diagnosis of dementia. The DemTect is such an internationally accepted tool. We aimed to characterize the neural networks associated with performance on the DemTect's subtests in two frequent dementia syndromes: early Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD). Voxel-based group comparisons of cerebral glucose utilization (as measured by F-18-fluorodeoxyglucose positron emission tomography) and gray matter atrophy (as measured by structural magnetic resonance imaging) were performed on data from 48 subjects with AD (n = 21), FTLD (n = 14) or subjective cognitive impairment (n = 13) as a control group. We performed group comparisons and correlation analyses between multimodal imaging data and performance on the DemTect's subtests. Group comparisons showed regional patterns consistent with previous findings for AD and FTLD. Interestingly, atrophy dominated in FTLD, whereas hypometabolism in AD. Across diagnostic groups performance on the “wordlist” subtest was positively correlated with glucose metabolism in the left temporal lobe. The “number transcoding” subtest was significantly associated with glucose metabolism in both a predominantly left lateralized frontotemporal network and a parietooccipital network including parts of the basal ganglia. Moreover, this subtest was associated with gray matter density in an extensive network including frontal, temporal, parietal and occipital areas. No significant correlates were observed for the “supermarket task” subtest. Scores on the “digit span reverse” subtest correlated with glucose metabolism in the left frontal cortex, the bilateral putamen, the head of caudate nucleus and the anterior insula. Disease-specific correlation analyses could partly verify or extend the correlates shown in the analyses across diagnostic groups. Correlates of gray matter density were found in FTLD for the “number transcoding” subtest and

  1. Alzheimer's disease and frontotemporal dementia are differentiated by discriminant analysis applied to 99mTc HmPAO SPECT data

    Science.gov (United States)

    Charpentier, P; Lavenu, I; Defebvre, L; Duhamel, A; Lecouffe, P; Pasquier, F; Steinling, M

    2000-01-01

    OBJECTIVE—Alzheimer's disease (AD) and frontotemporal dementia (FTD) are the most frequent neurodegenerative cognitive disorders. However, FTD remains poorly recognised clinically. The use of 99mHmPAO-single photon emission computed tomography (SPECT) has been demonstrated in the differentiation of AD and FTD. Nethertheless, there are very few comparative studies designed to assess its precise value in this differential diagnosis. The aim was to determine a simple decision rule, deduced from statistical analysis, which, if applied to regions of interest (ROIs) and mini mental state examination (MMSE), could improve the predictive value of SPECT in differential diagnosis between AD and FTD.
METHODS—Forty patients, 20 with probable AD and 20 with probable FTD were included. All patients underwent brain SPECT imaging, after an intravenous injection of 99mTc HmPAO-(555mBq). For each patient, 20 ROIs were determined on the Fleishig's slice and their activity was normalised to the mean cerebellar activity. Bivariate analysis (Wilcoxon rank tests) and multivariate analysis (stepwise discriminant analysis) were performed to determine the subgroup of variables able to give the highest predictive value for this differential diagnosis. A simple decision rule was built from a predictive score derived by factorial discriminant analysis.
RESULTS—As previously described, the fixation defect was found in frontal regions of interest (ROIs) in FTD and in the left temporoparietal-occipital ROIs in AD. Among the 21 variables, five were finally selected: right median frontal, left lateral frontal, left tempoparietal, left temporoparietal-occipital areas, and MMSE. One hundred per cent of patients with FTD were correctly classified by the decision rule (20/20 patients) and 90% of patients with AD (18/20).
CONCLUSION—AD and FTD are differentiated by SPECT. Automatic classification based on a decision rule deduced from factorial discriminant analysis could enhance its

  2. Alzheimer's disease and frontotemporal dementia are differentiated by discriminant analysis applied to (99m)Tc HmPAO SPECT data.

    Science.gov (United States)

    Charpentier, P; Lavenu, I; Defebvre, L; Duhamel, A; Lecouffe, P; Pasquier, F; Steinling, M

    2000-11-01

    Alzheimer's disease (AD) and frontotemporal dementia (FTD) are the most frequent neurodegenerative cognitive disorders. However, FTD remains poorly recognised clinically. The use of (99m)HmPAO-single photon emission computed tomography (SPECT) has been demonstrated in the differentiation of AD and FTD. Nethertheless, there are very few comparative studies designed to assess its precise value in this differential diagnosis. The aim was to determine a simple decision rule, deduced from statistical analysis, which, if applied to regions of interest (ROIs) and mini mental state examination (MMSE), could improve the predictive value of SPECT in differential diagnosis between AD and FTD. Forty patients, 20 with probable AD and 20 with probable FTD were included. All patients underwent brain SPECT imaging, after an intravenous injection of (99m)Tc HmPAO-(555mBq). For each patient, 20 ROIs were determined on the Fleishig's slice and their activity was normalised to the mean cerebellar activity. Bivariate analysis (Wilcoxon rank tests) and multivariate analysis (stepwise discriminant analysis) were performed to determine the subgroup of variables able to give the highest predictive value for this differential diagnosis. A simple decision rule was built from a predictive score derived by factorial discriminant analysis. As previously described, the fixation defect was found in frontal regions of interest (ROIs) in FTD and in the left temporoparietal-occipital ROIs in AD. Among the 21 variables, five were finally selected: right median frontal, left lateral frontal, left tempoparietal, left temporoparietal-occipital areas, and MMSE. One hundred per cent of patients with FTD were correctly classified by the decision rule (20/20 patients) and 90% of patients with AD (18/20). AD and FTD are differentiated by SPECT. Automatic classification based on a decision rule deduced from factorial discriminant analysis could enhance its performance.

  3. Primary empathy deficits in frontotemporal dementia

    Science.gov (United States)

    Baez, Sandra; Manes, Facundo; Huepe, David; Torralva, Teresa; Fiorentino, Natalia; Richter, Fabian; Huepe-Artigas, Daniela; Ferrari, Jesica; Montañes, Patricia; Reyes, Pablo; Matallana, Diana; Vigliecca, Nora S.; Decety, Jean; Ibanez, Agustin

    2014-01-01

    Loss of empathy is an early central symptom and diagnostic criterion of the behavioral variant frontotemporal dementia (bvFTD). Although changes in empathy are evident and strongly affect the social functioning of bvFTD patients, few studies have directly investigated this issue by means of experimental paradigms. The current study assessed multiple components of empathy (affective, cognitive and moral) in bvFTD patients. We also explored whether the loss of empathy constitutes a primary deficit of bvFTD or whether it is explained by impairments in executive functions (EF) or other social cognition domains. Thirty-seven bvFTD patients with early/mild stages of the disease and 30 healthy control participants were assessed with a task that involves the perception of intentional and accidental harm. Participants were also evaluated on emotion recognition, theory of mind (ToM), social norms knowledge and several EF domains. BvFTD patients presented deficits in affective, cognitive and moral aspects of empathy. However, empathic concern was the only aspect primarily affected in bvFTD that was neither related nor explained by deficits in EF or other social cognition domains. Deficits in the cognitive and moral aspects of empathy seem to depend on EF, emotion recognition and ToM. Our findings highlight the importance of using tasks depicting real-life social scenarios because of their greater sensitivity in the assessment of bvFTD. Moreover, our results contribute to the understanding of primary and intrinsic empathy deficits of bvFTD and have important theoretical and clinical implications. PMID:25346685

  4. Frontotemporal Lobar Degeneration and microRNAs

    Directory of Open Access Journals (Sweden)

    Paola ePiscopo

    2016-02-01

    Full Text Available Frontotemporal lobar degeneration (FTLD includes a spectrum of disorders characterized by changes of personality and social behaviour and, often, a gradual and progressive language dysfunction. In the last years, several efforts have been fulfilled in identifying both genetic mutations and pathological proteins associated with FTLD. The molecular bases undergoing the onset and progression of the disease remain still unknown. Recent literature prompts an involvement of RNA metabolism in FTLD, particularly miRNAs. Dysregulation of miRNAs in several disorders, including neurodegenerative diseases, and increasing importance of circulating miRNAs in different pathologies has suggested to implement the study of their possible application as biological markers and new therapeutic targets; moreover, miRNA-based therapy is becoming a powerful tool to deepen the function of a gene, the mechanism of a disease, and validate therapeutic targets. Regarding FTLD, different studies showed that miRNAs are playing an important role. For example, several reports have evaluated miRNA regulation of the progranulin gene suggesting that it is under their control, as described for miR-29b, miR-107 and miR-659. More recently, it has been demonstrated that TMEM106B gene, which protein is elevated in FTLD-TDP brains, is repressed by miR-132/212 cluster; this post-transcriptional mechanism increases intracellular levels of progranulin, affecting its pathways. These findings if confirmed could suggest that these microRNAs have a role as potential targets for some related-FTLD genes. In this review, we focus on the emerging roles of the miRNAs in the pathogenesis of FTLD.

  5. Demencia frontotemporal no familiar y epilepsia generalizada Frontotemporal dementia non familial and generalized epilepsy

    Directory of Open Access Journals (Sweden)

    Patricio Fuentes

    2005-12-01

    Full Text Available Se presenta un paciente de 62 años, sin antecedentes familiares de demencia, que a los 45 años debuta con crisis súbitas de disnea, visión borrosa, caída al suelo y movimientos repetitivos de brazos. Tratado por epilepsia con fenitoína y ácido valproico, repite esporádicamente crisis semejantes. Hace 4 años sus familiares notan cambios de personalidad, irritabilidad y conductas obsesivas. Hace 2 años aparecen episodios de desorientación de días de duración, algunos con alucinaciones auditivas y también fenómenos convulsivos. Ultimamente presenta crisis polimorfas, algunas con prolongada alteración de conciencia, estados catatoniformes y relajación esfinteriana. Examen físico y neurológico sin anormalidades. Evaluación neuropsicológica evidenció consistentes defectos en funciones frontales. EEG mostraron lentitud generalizada y actividad irritativa esporádica en regiones frontotemporales. Atrofia cortical de predominio anterior en CT scan e hipoperfusión fronto-temporal bilateral en SPECT. Exámenes de laboratorio y LCR normales. CONCLUSIÓN: La asociación de DFT con epilepsia, en forma no familiar, sugiere un síndrome neurodegenerativo cortical diferente.A 62 year-old patient is presented, without family antecedents of dementia who begins with 45 years of age with sudden crisis of dyspnea, blurred vision, fall to the floor and repetitive jerks of arms. Tried by epilepsy with phenytoin and valproate repeats similar crisis sporadically. Four years ago their relatives began to notice changes of personality, irritability and obsessive behaviors. Later on, are added episodes of disorientation of days of duration, some with auditory hallucinations and also convulsive manifestations. Finally appear polymorphic crisis, some with continue alteration of consciousness, catatonic states and sphincteric incontinence. Physical and neurological examination without abnormalities. Neuropsychological evaluation evidenced consistent

  6. Frontotemporal dementia caused by CHMP2B mutations

    DEFF Research Database (Denmark)

    Isaacs, A M; Johannsen, P; Holm, I

    2011-01-01

    CHMP2B mutations are a rare cause of autosomal dominant frontotemporal dementia (FTD). The best studied example is frontotemporal dementia linked to chromosome 3 (FTD-3) which occurs in a large Danish family, with a further CHMP2B mutation identified in an unrelated Belgian familial FTD patient. ...... features of FTD caused by CHMP2B truncation mutations as well as new brain imaging and neuropathological findings. Finally, we collate the current data on CHMP2B missense mutations, which have been reported in FTD and motor neuron disease....

  7. Frontotemporal dementia with trans-activation response DNA-binding protein 43 presenting with catatonic syndrome.

    Science.gov (United States)

    Watanabe, Ryohei; Kawakami, Ito; Onaya, Mitsumoto; Higashi, Shinji; Arai, Nobutaka; Akiyama, Haruhiko; Hasegawa, Masato; Arai, Tetsuaki

    2017-11-07

    Catatonia is a clinical syndrome characterized by symptoms such as immobility, mutism, stupor, stereotypy, echophenomena, catalepsy, automatic obedience, posturing, negativism, gegenhalten and ambitendency. This syndrome occurs mostly in mood disorder and schizophrenic patients, and is related to neuronal dysfunction involving the frontal lobe. Some cases of frontotemporal dementia (FTD) with catatonia have been reported, but these cases were not examined by autopsy. Here, we report on a FTD case which showed catatonia after the first episode of brief psychotic disorder. At the age of 58, the patient had a sudden onset of disorganized behavior and meaningless speech. Psychotropic drugs were effective for catatonic symptoms. However, after remission apathy, hyperorality, socially inappropriate behavior, hoarding, and an instinctive grasp reaction appeared and persisted. Brain MRI showed significant atrophy of the bilateral fronto-temporal lobes. A neuropathological examination revealed extensive trans-activation response DNA-binding protein 43 (TDP-43) positive neurocytoplasmic inclusions and dystrophic neurites in the brain, including the cerebral cortex, basal ganglia, and brainstem. Pathological diagnosis was frontotemporal lobar degeneration (FTLD) with TDP-43 (FTLD-TDP) type C, which was also confirmed by the band pattern of C-terminal fragments of TDP-43 on western blotting of sarkosyl-insoluble fractions extracted from the frozen brain. Dysfunction of the thalamus, globus pallidus, supplementary motor area, amygdala and cingulate cortex have been said to be related to the catatonic syndrome. In this case, these areas were affected, showing abnormal TDP-43-positive structures. Further studies are expected to confirm further clinical - pathological correlations to FTLD. © 2017 Japanese Society of Neuropathology.

  8. [A case of combined sensation disturbance and clumsiness of the left hand caused by an infarction localized to brodmann areas 1 and 2].

    Science.gov (United States)

    Kutoku, Yumiko; Hagiwara, Hiroki; Ichikawa, Yaeko; Takeda, Katsuhiko; Sunada, Yoshihide

    2007-04-01

    A 70-year-old woman was admitted to our hospital with a complaint of numbness and clumsiness of the left hand. On physical examination 23 days after the onset of cerebral infarction, she showed no apparent muscle weakness. Although her elementary somatosensory function was mostly intact with a minimal joint position sensation disturbance, she showed disturbances in tactile recognition, two-point discrimination, and weight perception. She also had difficulty in discrete finger movement of her left hand, especially when her eyes were closed. Brain MRI disclosed a small infarction localized to Brodmann areas 1 and 2 in the right postcentral gyrus. In the left median nerve short-latency somatosensory evoked potentials (s-SEPs), the N20 potential was normally evoked. This finding also indicated that the area 3b was preserved. The sensory symptoms observed in this patient were compatible with the hierarchical somatosensory processing model in the postcentral gyrus proposed by Iwamura et al, in which the elementary sensation recognized in area 3 is transferred to areas 1 and 2, and then processed to discriminative sensation. The disturbed discrete finger movement in this patient probably resulted from impaired tactile recognition which could be compensated for by visual information.

  9. Cortical function in Alzheimer’s disease and frontotemporal dementia

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    Wang Pan

    2016-01-01

    Full Text Available Alzheimer’s disease (AD and the behavioral variant of frontotemporal dementia (bvFTD are the most common causes of dementia; however, their overlapping clinical syndromes and involved brain regions make a differential diagnosis difficult. We aimed to identify the differences in the cognition and motor cortex excitability between AD and bvFTD patients.

  10. Referral Patterns for Syndromes Associated With Frontotemporal Lobar Degeneration

    OpenAIRE

    Chow, Tiffany W.; Hodges, John R.; Dawson, Kate E.; Miller, Bruce L.; Smith, Virginia; Mendez, Mario F.; Lipton, Anne M.

    2005-01-01

    We compared demographics of subjects diagnosed with frontotemporal degeneration (FTD) at a group of 5 clinics specializing in this non-Alzheimer dementia against those subjects diagnosed at standard Alzheimer disease centers, to determine any differences in referral patterns between such clinics.

  11. Four-dimensional echocardiography area strain combined with exercise stress echocardiography to evaluate left ventricular regional systolic function in patients with mild single vessel coronary artery stenosis.

    Science.gov (United States)

    Deng, Yan; Peng, Long; Liu, Yuan-Yuan; Yin, Li-Xue; Li, Chun-Mei; Wang, Yi; Rao, Li

    2017-09-01

    The aim of this prospective study was to assess the diagnosis value of four-dimensional echocardiography area strain (AS) combined with exercise stress echocardiography to evaluate left ventricular regional systolic function in patients with mild single vessel coronary artery stenosis. Based on treadmill exercise load status, two-dimensional conventional echocardiography and four-dimensional echocardiography area strain were performed on patients suspected coronary artery disease before coronary angiogram. Thirty patients (case group) with mild left anterior descending coronary artery stenosis (stenosis coronary artery stenosis according to the coronary angiogram results were prospectively enrolled. All the patients had no left ventricular regional wall motion abnormality in two-dimensional echocardiography at rest and exercise stress. There was no significant difference in the 16 segmental systolic peak AS at rest between two groups. After exercise stress, the peak systolic AS rest-stress at mid anterior wall (-7.00%±10.90% vs 2.80%±23.69%) and mid anterolateral wall (-4.40%±18.81% vs 8.80%±19.16%) were decreased, while increased at basal inferolateral wall (14.00%±19.27% vs -5.60%±15.94%) in case group compared with control group (Pcoronary artery stenosis, the area strain was decreased at involved segments, while compensatory increased at noninvolved segments after exercise stress. Four-dimensional echocardiography area strain combined with exercise stress echocardiography could sensitively find left ventricular regional systolic function abnormality in patients with mild single vessel coronary artery stenosis, and locate stenosis coronary artery accordingly. © 2017, Wiley Periodicals, Inc.

  12. Asymmetric oculomotor apraxia, optic ataxia, and simultanagnosia with right hemispatial neglect from a predominantly left-sided lesion of the parieto-occipital area.

    Science.gov (United States)

    Sakurai, Yasuhisa; Fujimoto, Masanori; Hamada, Kensuke; Sugimoto, Izumi

    2018-01-01

    Bálint's syndrome involves bilateral damage to the parieto-occipital area. The extent of the effect of unilateral damage on the Bálint's triad (oculomotor apraxia, optic ataxia, and simultanagnosia) remains unknown. We examined a 63-year-old, right-handed woman who developed right hemianopia, oculomotor apraxia, optic ataxia, simultanagnosia, and hemispatial neglect (HSN) for the right after a cerebral infarction, with detailed neuropsychological tests, magnetic resonance imaging, and single photon emission computed tomography (SPECT). Neuropsychological examination showed that oculomotor apraxia, optic ataxia, and simultanagnosia were more pronounced in the right hemi-space, probably due to the limited eye movement in the right visual field, whereas HSN was restricted to the right hemi-space. Diffusion-weighted MR images revealed hyperintensity in the left parieto-temporo-occipital region, and several spotty areas of the bilateral frontal and parietal subcortical regions. SPECT revealed hypoperfusion in the left parieto-occipital region and frontal operculum and small areas of the right superior parietal lobule. The case suggests that asymmetric (more pronounced in the right hemi-space) oculomotor apraxia, optic ataxia, and simultanagnosia occur in an extensive lesion of the left parieto-occipital cortices. Although HSN is not a prerequisite for simultanagnosia, the coexistence of HSN aggravates simultanagnosia in the hemi-space opposite the lesion.

  13. Quantification of aortic valve area and left ventricular muscle mass in healthy subjects and patients with symptomatic aortic valve stenosis by MRI.

    Science.gov (United States)

    Haimerl, J; Freitag-Krikovic, A; Rauch, A; Sauer, E

    2005-03-01

    MRI allows visualization and planimetry of the aortic valve orifice and accurate determination of left ventricular muscle mass, which are important parameters in aortic stenosis. In contrast to invasive methods, MRI planimetry of the aortic valve area (AVA) is flow independent. AVA is usually indexed to body surface area. Left ventricular muscle mass is dependent on weight and height in healthy individuals. We studied AVA, left ventricular muscle mass (LMM) and ejection fraction (EF) in 100 healthy individuals and in patients with symptomatic aortic valve stenosis (AS). All were examined by MRI (1.5 Tesla Siemens Sonate) and the AVA was visualized in segmented 2D flash sequences and planimetry of the performed AVA was manually. The aortic valve area in healthy individuals was 3.9+/-0.7 cm(2), and the LMM was 99+/-27 g. In a correlation analysis, the strongest correlation of AVA was to height (r=0.75, pvalve stenosis, AVA was 1.0+/-0.35 cm(2), in correlation to cath lab r=0.72, and LMM was 172+/-56 g. We compared the AS patients results with the data of the healthy subjects, where the reduction of the AVA was 28+/-10% of the expected normal value, while LMM was 42% higher in patients with AS. There was no correlation to height, weight or BSA in patients with AS. With cardiac MRI, planimetry of AVA for normal subjects and patients with AS offered a simple, fast and non-invasive method to quantify AVA. In addition LMM and EF could be determined. The strong correlation between height and AVA documented in normal subjects offered the opportunity to integrate this relation between expected valve area and definitive orifice in determining the disease of the aortic valve for the individual patient. With diagnostic MRI in patients with AS, invasive measurements of the systolic transvalvular gradient does not seem to be necessary.

  14. Behavioural variant frontotemporal dementia: clinical and therapeutic approaches.

    Science.gov (United States)

    Fernández-Matarrubia, M; Matías-Guiu, J A; Moreno-Ramos, T; Matías-Guiu, J

    2014-10-01

    Behavioural variant frontotemporal dementia (bvFTD) is the most frequent presentation in the clinical spectrum of frontotemporal dementia (FTD) and it is characterised by progressive changes in personality and conduct. Major breakthroughs in molecular biology and genetics made during the last two decades have lent us a better understanding of this syndrome, which may be the first manifestation in many different neurodegenerative diseases. We reviewed the main epidemiological, clinical, diagnostic and therapeutic aspects of bvFTD. Most cases manifest sporadically and the average age of onset is 58 years. Current criteria for bvFTD propose three levels of diagnostic certainty: possible, probable, and definite. Clinical diagnosis is based on a detailed medical history provided by family members and caregivers, in conjunction with neuropsychological testing. Treatments which have been used in bvFDT to date are all symptomatic and their effectiveness is debatable. New drugs designed for specific molecular targets that are implicated in frontotemporal lobar degeneration are being developed. BvFDT is a frequent cause of dementia. It is a non-specific syndrome associated with heterogeneous histopathological and biomolecular findings. The definition of clinical subtypes complemented by biomarker identification may help predict the underlying pathology. This knowledge, along with the development of drugs designed for molecular targets, will offer new treatment possibilities. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  15. Functional MRI of music emotion processing in frontotemporal dementia.

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    Agustus, Jennifer L; Mahoney, Colin J; Downey, Laura E; Omar, Rohani; Cohen, Miriam; White, Mark J; Scott, Sophie K; Mancini, Laura; Warren, Jason D

    2015-03-01

    Frontotemporal dementia is an important neurodegenerative disorder of younger life led by profound emotional and social dysfunction. Here we used fMRI to assess brain mechanisms of music emotion processing in a cohort of patients with frontotemporal dementia (n = 15) in relation to healthy age-matched individuals (n = 11). In a passive-listening paradigm, we manipulated levels of emotion processing in simple arpeggio chords (mode versus dissonance) and emotion modality (music versus human emotional vocalizations). A complex profile of disease-associated functional alterations was identified with separable signatures of musical mode, emotion level, and emotion modality within a common, distributed brain network, including posterior and anterior superior temporal and inferior frontal cortices and dorsal brainstem effector nuclei. Separable functional signatures were identified post-hoc in patients with and without abnormal craving for music (musicophilia): a model for specific abnormal emotional behaviors in frontotemporal dementia. Our findings indicate the potential of music to delineate neural mechanisms of altered emotion processing in dementias, with implications for future disease tracking and therapeutic strategies. © 2014 The Authors. Annals of the New York Academy of Sciences published by Wiley Periodicals Inc. on behalf of The New York Academy of Sciences.

  16. Thoracic aortic stent-graft placement combined with left subclavian artery 'chimney operation': therapeutic analysis of 15 cases with insufficient proximal anchor area

    International Nuclear Information System (INIS)

    Liu Jiayi; Huang Lianjun; Fan Zhanming; Zhang Zhaoqi

    2012-01-01

    Objective: To discuss the strategies for the management of insufficient proximal anchoring area during the performance of transluminal stent-graft placement (TSGP), and to evaluate the feasibility of intentional coverage of the left subclavian artery (LSA) together with left subclavian artery stent-graft placement by using 'chimney operation' technique. Methods: A total of 15 patients with thoracic aortic diseases complicated by insufficient proximal anchoring area, who were encountered in authors' hospital during the period from Dec. 2009 to April 2011, were enrolled in this study. The clinical data were retrospectively analyzed. The thoracic aortic diseases included aortic dissection (n=6), aortic pseudoaneurysm (n=1), aortic aneurysm (n=4) and penetrating ulcer (n=4). Of the 15 patients, the distance between the lesion and LSA anchoring site 15 mm in 2. TSGP was carried out. The ostium of LSA was intentionally and completely covered by thoracic aortic stent-graft and left subclavian artery stent-graft placement was subsequently performed. The patients were kept under observation for symptoms of cerebral and upper limb ischemia. The postoperative complications such as endoleak and the patency of LSA were assessed with angiography. Results: Thoracic aortic stent-graft placement was successfully carried out in all 15 patients. In addition, one 'chimney' stent was properly implanted in LSA in each patient. After the procedure, no complications of nervous system or severe ischemia of upper extremity occurred. Follow-up examinations performed between 5 days to 3 months after the treatment revealed that the aortic stent-graft remained in stable condition and no type Ⅰ endoleak occurred, meanwhile the blood flow in 'chimney' stent was unobstructed. Conclusion: Intentional LSA coverage with 'chimney operation' can expand the applicability of TSGP with high tolerability. It is especially useful for patients with left vertebral artery blood supply dominance or with

  17. Augmented Input Reveals Word Deafness in a Man with Frontotemporal Dementia

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    Chris Gibbons

    2012-01-01

    Full Text Available We describe a 57 year old, right handed, English speaking man initially diagnosed with progressive aphasia. Language assessment revealed inconsistent performance in key areas. Expressive language was reduced to a few short, perseverative phrases. Speech was severely apraxic. Primary modes of communication included gesture, pointing, gaze, physical touch and leading. Responses were 100% accurate when he was provided with written words, with random or inaccurate responses for strictly auditory/verbal input. When instructions to subsequent neuropsychological tests were written instead of spoken, performance improved markedly. A comprehensive audiology assessment revealed no hearing impairment. Neuroimaging was unremarkable. Neurobehavioral evaluation utilizing written input led to diagnoses of word deafness and frontotemporal dementia, resulting in very different management. We highlight the need for alternative modes of language input for assessment and treatment of patients with language comprehension symptoms.

  18. The Left Superior Longitudinal Fasciculus within the Primary Sensory Area of Inferior Parietal Lobe Plays a Role in Dysgraphia of Kana Omission within Sentences

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    Nobusada Shinoura

    2012-01-01

    Full Text Available Functional neurological changes after surgery combined with diffusion tensor imaging (DTI tractography can directly provide evidence of anatomical localization of brain function. Using these techniques, a patient with dysgraphia before surgery was analyzed at our hospital in 2011. The patient showed omission of kana within sentences before surgery, which improved after surgery. The brain tumor was relatively small and was located within the primary sensory area (S1 of the inferior parietal lobe (IPL. DTI tractography before surgery revealed compression of the branch of the superior longitudinal fasciculus (SLF by the brain tumor. These results suggest that the left SLF within the S1 of IPL plays a role in the development of dysgraphia of kana omission within sentences.

  19. Right lower limb apraxia in a patient with left supplementary motor area infarction: intactness of the corticospinal tract confirmed by transcranial magnetic stimulation

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    Min Cheol Chang

    2015-01-01

    Full Text Available We reported a 50-year-old female patient with left supplementary motor area infarction who presented right lower limb apraxia and investigated the possible causes using transcranial magnetic stimulation. The patient was able to walk and climb stairs spontaneously without any assistance at 3 weeks after onset. However, she was unable to intentionally move her right lower limb although she understood what she supposed to do. The motor evoked potential evoked by transcranial magnetic stimulation from the right lower limb was within the normal range, indicating that the corticospinal tract innervating the right lower limb was uninjured. Thus, we thought that her motor dysfunction was not induced by motor weakness, and confirmed her symptoms as apraxia. In addition, these results also suggest that transcranial magnetic stimulation is helpful for diagnosing apraxia.

  20. Epidemiological Survey of Frontotemporal Lobar Degeneration in Tottori Prefecture, Japan

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    Kenji Wada-Isoe

    2012-09-01

    Full Text Available Background: The prevalence of frontotemporal lobar degeneration (FTLD in Japan is unknown. An epidemiological survey study of FTLD was undertaken in Tottori Prefecture, a district in the western region of Japan. Methods: Hospitals in Tottori Prefecture were surveyed by a two-step questionnaire in 2010, and the prevalence of FTLD per 100,000 inhabitants was calculated using the actual number of patients and inhabitants in Tottori Prefecture on the prevalence day of October 1, 2010. Results: In this survey, 66 patients were diagnosed with FTLD. The subtypes of FTLD were as follows: 62 cases of frontotemporal dementia (FTD, 3 cases of progressive nonfluent aphasia, and 1 case of semantic dementia. Among the FTD cases, 5 cases were FTD with motor neuron disease and 1 case was FTD with parkinsonism linked to chromosome 17. The prevalence of FTD in the total population of Tottori Prefecture was 11.2 per 100,000 inhabitants. Based on these results, the prevalence of FTLD in Japan in 2008 was estimated to be 9.5 per 100,000 individuals. Conclusions: Our epidemiological survey results suggest that there are at least 12,000 FTLD patients in Japan, indicating that FTLD is not a rare disease.

  1. Biomarkers: a new approach to behavioural variant frontotemporal dementia.

    Science.gov (United States)

    Fernández-Matarrubia, M; Matías-Guiu, J A; Moreno-Ramos, T; Matías-Guiu, J

    2015-01-01

    Lobar frontotemporal degeneration (FTLD) encompasses a group of molecular disease defined by the deposition of an abnormal protein in the central nervous system. Behavioural variant frontotemporal dementia (bvFTD) is the most frequent clinical presentation of FTLD. The past two decades of research have contributed to a better understanding of this entity, which may be the first manifestation in many different neurodegenerative disorders. We reviewed correlations between clinical, pathological, and genetic findings and the main disease biomarkers of FTLD, with particular interest in bvFTD. Anatomical pathology findings in FTLD are heterogeneous and the syndrome is not associated with any one specific histopathological type. Promising available biomarkers include structural and functional neuroimaging techniques and biochemical and genetic biomarkers. Disease-modifying drugs designed for specific molecular targets that are implicated in FTLD pathogenesis are being developed. BvFTD is a frequent cause of dementia. Of all the clinical variants of FTLD, behavioural variant is the one in which establishing a correlation between clinical and pathological signs is the most problematic. A biomarker evaluation may help predict the underlying pathology; this approach, in conjunction with the development of disease-modifying drugs, offers new therapeutic possibilities. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  2. A Case of Frontotemporal Lobar Degeneration with Progressive Dysarthria

    Science.gov (United States)

    Ihori, Nami; Araki, Shigeo; Ishihara, Kenji; Kawamura, Mitsuru

    2006-01-01

    We investigated the evolution of the neurological and neuropsychological characteristics in a right-handed woman who was 53-years-old at the onset and who showed personality changes and behavioral disorders accompanied by progressive dysarthria. She had hypernasality and a slow rate of speech with distorted consonants and vowels, which progressed as motor disturbances affecting her speech apparatus increased; finally, she became mute two years post onset. Her dysarthria due to bilateral voluntary facio-velo-linguo-pharyngeal paralysis accompanied with automatic-voluntary dissociation fit the description of anterior opercular syndrome. She showed personality changes and behavioral abnormalities from the initial stage of the disease, as is generally observed in frontotemporal degeneration (FTD), and her magnetic resonance image showed progressive atrophy in the frontotemporal lobes; thus, she was clinically diagnosed with FTLD. This patient’s symptoms suggest that FTLD, including bilateral anterior operculum degeneration, causes progressive pseudobulbar paretic dysarthria accompanied by clinical symptoms of FTD, which raises the possibility of a new clinical subtype in the FTLD spectrum. PMID:16873920

  3. Latent profile analysis in frontotemporal lobar degeneration and related disorders: clinical presentation and SPECT functional correlates

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    Di Luca Monica

    2007-05-01

    Full Text Available Abstract Background Frontotemporal Lobar Degeneration (FTLD thus recently renamed, refers to a spectrum of heterogeneous conditions. This same heterogeneity of presentation represents the major methodological limit for the correct evaluation of clinical designation and brain functional correlates. At present, no study has investigated clinical clusters due to specific cognitive and behavioural disturbances beyond current clinical criteria. The aim of this study was to identify clinical FTLD presentation, based on cognitive and behavioural profile, and to define their SPECT functional correlations. Methods Ninety-seven FTLD patients entered the study. A clinical evaluation and standardised assessment were preformed, as well as a brain SPECT perfusion imaging study. Latent Profile Analysis on clinical, neuropsychological, and behavioural data was performed. Voxel-basis analysis of SPECT data was computed. Results Three specific clusters were identified and named "pseudomanic behaviour" (LC1, "cognitive" (LC2, and "pseudodepressed behaviour" (LC3 endophenotypes. These endophenotypes showed a comparable hypoperfusion in left temporal lobe, but a specific pattern involving: medial and orbitobasal frontal cortex in LC1, subcortical brain region in LC2, and right dorsolateral frontal cortex and insula in LC3. Conclusion These findings provide evidence that specific functional-cluster symptom relationship can be delineated in FTLD patients by a standardised assessment. The understanding of the different functional correlates of clinical presentations will hopefully lead to the possibility of individuating diagnostic and treatment algorithms.

  4. Common and unique gray matter correlates of episodic memory dysfunction in frontotemporal dementia and Alzheimer's disease.

    Science.gov (United States)

    Irish, Muireann; Piguet, Olivier; Hodges, John R; Hornberger, Michael

    2014-04-01

    Conflicting evidence exists regarding the integrity of episodic memory in the behavioral variant of frontotemporal dementia (bvFTD). Recent converging evidence suggests that episodic memory in progressive cases of bvFTD is compromised to the same extent as in Alzheimer's disease (AD). The underlying neural substrates of these episodic memory deficits, however, likely differ contingent on dementia type. In this study we sought to elucidate the neural substrates of episodic memory performance, across recall and recognition tasks, in both patient groups using voxel-based morphometry (VBM) analyses. We predicted that episodic memory dysfunction would be apparent in both patient groups but would relate to divergent patterns of neural atrophy specific to each dementia type. We assessed episodic memory, across verbal and visual domains, in 19 bvFTD, 18 AD patients, and 19 age- and education-matched controls. Behaviorally, patient groups were indistinguishable for immediate and delayed recall, across verbal and visual domains. Whole-brain VBM analyses revealed regions commonly implicated in episodic retrieval across groups, namely the right temporal pole, right frontal lobe, left paracingulate gyrus, and right anterior hippocampus. Divergent neural networks specific to each group were also identified. Whereas a widespread network including posterior regions such as the posterior cingulate cortex, parietal and occipital cortices was exclusively implicated in AD, the frontal and anterior temporal lobes underpinned the episodic memory deficits in bvFTD. Our results point to distinct neural changes underlying episodic memory decline specific to each dementia syndrome. Copyright © 2013 Wiley Periodicals, Inc.

  5. Effect of minoxidil topical foam on frontotemporal and vertex androgenetic alopecia in men: a 104-week open-label clinical trial.

    Science.gov (United States)

    Kanti, V; Hillmann, K; Kottner, J; Stroux, A; Canfield, D; Blume-Peytavi, U

    2016-07-01

    Topical minoxidil formulations have been shown to be effective in treating androgenetic alopecia (AGA) for 12 months. Efficacy and safety in both frontotemporal and vertex regions over longer application periods have not been studied so far. To evaluate the effect of 5% minoxidil topical foam (5% MTF) in the frontotemporal and vertex areas in patients with moderate AGA over 104 weeks. An 80-week, open-label extension phase was performed, following a 24-week randomized, double-blind, placebo-controlled study in men with AGA grade IIIvertex to VI. Group 1 (n = 22) received ongoing 5% MTF for 104 weeks, Group 2 (n = 23) received placebo topical foam (plaTF) until week 24, followed by 5% MTF until week 104 during the extension phase. Frontotemporal and vertex target area non-vellus hair counts (f-TAHC, v-TAHC) and cumulative hair width (f-TAHW, v-TAHW) were assessed at baseline and at weeks 24, 52, 76 and 104. In Group 1, f-TAHW and f-TAHC showed a statistically significant increase from baseline to week 52 and week 76, respectively, returning to values comparable to baseline at week 104. No significant differences were found between baseline and week 104 in v-TAHC in Group 1 as well as f-TAHC, v-TAHC, f-TAHW and v-TAHW values in Group 2. 5% MTF is effective in stabilizing hair density, hair width and scalp coverage in both frontotemporal and vertex areas over an application period of 104 weeks, while showing a good safety and tolerability profile with a low rate of irritant contact dermatitis. © 2015 European Academy of Dermatology and Venereology.

  6. Fronto-parietal and fronto-temporal theta phase synchronization for visual and auditory-verbal working memory

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    Masahiro eKawasaki

    2014-03-01

    Full Text Available In humans, theta phase (4–8 Hz synchronization observed on electroencephalography (EEG plays an important role in the manipulation of mental representations during working memory (WM tasks; fronto-temporal synchronization is involved in auditory-verbal WM tasks and fronto-parietal synchronization is involved in visual WM tasks. However, whether or not theta phase synchronization is able to select the to-be-manipulated modalities is uncertain. To address the issue, we recorded EEG data from subjects who were performing auditory-verbal and visual WM tasks; we compared the theta synchronizations when subjects performed either auditory-verbal or visual manipulations in separate WM tasks, or performed both two manipulations in the same WM task. The auditory-verbal WM task required subjects to calculate numbers presented by an auditory-verbal stimulus, whereas the visual WM task required subjects to move a spatial location in a mental representation in response to a visual stimulus. The dual WM task required subjects to manipulate auditory-verbal, visual, or both auditory-verbal and visual representations while maintaining auditory-verbal and visual representations. Our time-frequency EEG analyses revealed significant fronto-temporal theta phase synchronization during auditory-verbal manipulation in both auditory-verbal and auditory-verbal/visual WM tasks, but not during visual manipulation tasks. Similarly, we observed significant fronto-parietal theta phase synchronization during visual manipulation tasks, but not during auditory-verbal manipulation tasks. Moreover, we observed significant synchronization in both the fronto-temporal and fronto-parietal theta signals during simultaneous auditory-verbal/visual manipulations. These findings suggest that theta synchronization seems to flexibly connect the brain areas that manipulate WM.

  7. Fronto-parietal and fronto-temporal theta phase synchronization for visual and auditory-verbal working memory.

    Science.gov (United States)

    Kawasaki, Masahiro; Kitajo, Keiichi; Yamaguchi, Yoko

    2014-01-01

    In humans, theta phase (4-8 Hz) synchronization observed on electroencephalography (EEG) plays an important role in the manipulation of mental representations during working memory (WM) tasks; fronto-temporal synchronization is involved in auditory-verbal WM tasks and fronto-parietal synchronization is involved in visual WM tasks. However, whether or not theta phase synchronization is able to select the to-be-manipulated modalities is uncertain. To address the issue, we recorded EEG data from subjects who were performing auditory-verbal and visual WM tasks; we compared the theta synchronizations when subjects performed either auditory-verbal or visual manipulations in separate WM tasks, or performed both two manipulations in the same WM task. The auditory-verbal WM task required subjects to calculate numbers presented by an auditory-verbal stimulus, whereas the visual WM task required subjects to move a spatial location in a mental representation in response to a visual stimulus. The dual WM task required subjects to manipulate auditory-verbal, visual, or both auditory-verbal and visual representations while maintaining auditory-verbal and visual representations. Our time-frequency EEG analyses revealed significant fronto-temporal theta phase synchronization during auditory-verbal manipulation in both auditory-verbal and auditory-verbal/visual WM tasks, but not during visual manipulation tasks. Similarly, we observed significant fronto-parietal theta phase synchronization during visual manipulation tasks, but not during auditory-verbal manipulation tasks. Moreover, we observed significant synchronization in both the fronto-temporal and fronto-parietal theta signals during simultaneous auditory-verbal/visual manipulations. These findings suggest that theta synchronization seems to flexibly connect the brain areas that manipulate WM.

  8. Bupropion improved apathy in behavioral variant frontotemporal dementia: a case report.

    Science.gov (United States)

    Lin, Chia-Pei; Chu, Chih-Pang; Liu, Hsing-Cheng

    2016-10-01

    Apathy is a common neurobehavioral sign in cases of behavioral variant frontotemporal dementia. However, there is still no established sustained effective treatment. We present the case of a 65-year-old man with behavioral variant frontotemporal dementia who suffered from severe apathy, but his apathy improved after a 10-month period of bupropion treatment. His single photon emission computed tomography report also showed slight improvement. To the best of our knowledge, such a case with imaging evidence has never been reported. Further studies to correlate the effects of bupropion on apathy in behavioral variant frontotemporal dementia patients are clearly needed.

  9. Demência fronto-temporal: aspectos clínicos e terapêuticos Demencia frontotemporal: aspectos clínicos y terapéuticos Frontotemporal dementia: clinical and therapeutic features

    Directory of Open Access Journals (Sweden)

    Antônio Lúcio Teixeira-Jr

    2006-04-01

    Full Text Available A demência fronto-temporal é uma importante causa de demência no período pré-senil. Caracteriza-se por significativas modificações do comportamento e da personalidade, enquanto o funcionamento cognitivo avaliado por testes psicométricos tradicionais encontra-se relativamente preservado. Muitos pacientes buscam o psiquiatra em virtude dos sintomas comportamentais proeminentes, como apatia, desinibição e comportamentos perseverativos ou estereotipados. O tratamento racional da demência fronto-temporal é atualmente limitado. Os sintomas comportamentais são controlados principalmente por inibidores seletivos da recaptação de serotonina.La demencia frontotemporal es una importante causa de demencia en el período presenil de la vida. Se caracteriza por alteraciones significativas en el comportamiento y en la personalidad, mientras la función cognitiva evaluada por pruebas psicométricas convencionales resulta relativamente preservada. Muchos pacientes recurren al psiquiatra en función de síntomas comportamentales sobresalientes como apatía, desinhibición y comportamientos perseverantes o estereotipados. El tratamiento racional de la demencia frontotemporal aún se encuentra bastante limitado. Los síntomas comportamentales se controlan principalmente con inhibidores selectivos de la recaptación de serotonina.Frontotemporal dementia is a major cause of dementia in the presenium. It is characterized by significant changes in behavior and personality, while cognitive functioning as assessed by traditional psychometric tests is relatively preserved. Thus, many patients present to the psychiatrist because of the prominence of behavioral symptoms, such as apathy, disinhibition, perseverative or stereotyped behaviors. Rational treatment for frontotemporal dementia is currently limited. The behavioral symptoms are controlled mainly with selective serotonin reuptake inhibitors.

  10. Molecular Pathways Bridging Frontotemporal Lobar Degeneration and Psychiatric Disorders

    Directory of Open Access Journals (Sweden)

    Roberta eZanardini

    2016-02-01

    Full Text Available The overlap of symptoms between neurodegenerative and psychiatric diseases has been reported. Neuropsychiatric alterations are commonly observed in dementia, especially in the behavioral variant of frontotemporal dementia (bvFTD, which is the most common clinical FTD subtype. At the same time, psychiatric disorders, like schizophrenia, can display symptoms of dementia, including features of frontal dysfunction with relative sparing of memory. In the present review we discuss common molecular features in these pathologies with a special focus on FTD. Molecules like Brain Derived Neurotrophic Factor (BDNF and progranulin are linked to the pathophysiology of both neurodegenerative and psychiatric diseases. In these brain-associated illnesses, the presence of disease-associated variants in BDNF and progranulin (GRN genes cause a reduction of circulating proteins levels, through alterations in proteins expression or secretion. For these reasons, we believe that prevention and therapy of psychiatric and neurological disorders could be achieved enhancing both BDNF and progranulin levels thanks to drug discovery efforts.

  11. Behavioral variant of frontotemporal dementia mimicking Huntington's disease

    DEFF Research Database (Denmark)

    Nielsen, T Rune; Bruhn, Peter; Nielsen, Jørgen E

    2010-01-01

    Behavioral changes and cognitive decline are the core clinical manifestations in the behavioral variant of frontotemporal dementia (bv-FTD). The behavioral changes may include characteristic stereotypic movements. These movements, although without clear purpose, are not involuntary. Involuntary m...... and Huntington's disease-like (HDL) phenotypes. Although a definite diagnosis will require neuropathological confirmation, we conclude that a HDL phenotype may be part of the clinical spectrum of the bv-FTD phenotype....... movements are usually not seen in FTD.Two patients with involuntary choreoathetoid movements but otherwise presenting a bv-FTD-phenotype were referred and Huntington's disease (HD) was suspected. The diagnoses of bv-FTD were made after comprehensive assessment and exclusion of other diagnoses, including HD...

  12. Excessive TV watching in patients with frontotemporal dementia.

    Science.gov (United States)

    Shin, Ji Soo; Kim, Michael Sunmin; Kim, Nicholas Sunyong; Kim, Geon Ha; Seo, Sang Won; Kim, Eun-Joo; Heilman, Kenneth M; Na, Duk L

    2013-01-01

    The "environmental dependency syndrome" refers to a loss of personal autonomy such that a person's environment almost entirely controls their actions. The goal of this study is to learn if patients with frontotemporal degeneration (FTD) exhibit prolonged TV watching, a behavior which may be a symptom of environmental dependency. We recruited 40 patients with FTD and 48 patients with Alzheimer's disease (AD), and asked these participants' caregivers about TV watching behaviors including total viewing time and channel/show preference, along with other behaviors indicative of environmental dependency. Compared to AD patients, FTD patients watched TV for a longer time. In addition, the patients who watched more TV showed more signs of environmental dependency. Increased TV watching may be a sign of environmental dependency, however further research is needed to explore other hypotheses.

  13. The unique predisposition to criminal violations in frontotemporal dementia.

    Science.gov (United States)

    Mendez, Mario F

    2010-01-01

    Brain disorders can lead to criminal violations. Patients with frontotemporal dementia (FTD) are particularly prone to sociopathic behavior while retaining knowledge of their acts and of moral and conventional rules. This report describes four FTD patients who committed criminal violations in the presence of clear consciousness and sufficiently intact cognition. They understood the nature of their acts and the potential consequences, but did not feel sufficiently concerned to be deterred. FTD involves a unique pathologic combination affecting the ventromedial prefrontal cortex, with altered moral feelings, right anterior temporal loss of emotional empathy, and orbitofrontal changes with disinhibited, compulsive behavior. These case histories and the literature indicate that those with right temporal FTD retain the capacity to tell right from wrong but have the slow and insidious loss of the capacity for moral rationality. Patients with early FTD present a challenge to the criminal justice system to consider alterations in moral cognition before ascribing criminal responsibility.

  14. Processing emotion from abstract art in frontotemporal lobar degeneration

    Science.gov (United States)

    Cohen, Miriam H.; Carton, Amelia M.; Hardy, Christopher J.; Golden, Hannah L.; Clark, Camilla N.; Fletcher, Phillip D.; Jaisin, Kankamol; Marshall, Charles R.; Henley, Susie M.D.; Rohrer, Jonathan D.; Crutch, Sebastian J.; Warren, Jason D.

    2016-01-01

    Abstract art may signal emotions independently of a biological or social carrier: it might therefore constitute a test case for defining brain mechanisms of generic emotion decoding and the impact of disease states on those mechanisms. This is potentially of particular relevance to diseases in the frontotemporal lobar degeneration (FTLD) spectrum. These diseases are often led by emotional impairment despite retained or enhanced artistic interest in at least some patients. However, the processing of emotion from art has not been studied systematically in FTLD. Here we addressed this issue using a novel emotional valence matching task on abstract paintings in patients representing major syndromes of FTLD (behavioural variant frontotemporal dementia, n=11; sematic variant primary progressive aphasia (svPPA), n=7; nonfluent variant primary progressive aphasia (nfvPPA), n=6) relative to healthy older individuals (n=39). Performance on art emotion valence matching was compared between groups taking account of perceptual matching performance and assessed in relation to facial emotion matching using customised control tasks. Neuroanatomical correlates of art emotion processing were assessed using voxel-based morphometry of patients' brain MR images. All patient groups had a deficit of art emotion processing relative to healthy controls; there were no significant interactions between syndromic group and emotion modality. Poorer art emotion valence matching performance was associated with reduced grey matter volume in right lateral occopitotemporal cortex in proximity to regions previously implicated in the processing of dynamic visual signals. Our findings suggest that abstract art may be a useful model system for investigating mechanisms of generic emotion decoding and aesthetic processing in neurodegenerative diseases. PMID:26748236

  15. Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype.

    Science.gov (United States)

    Mann, David M A; Snowden, Julie S

    2017-11-01

    Frontotemporal Lobar Degeneration (FTLD) is a clinically, pathologically and genetically heterogeneous group of disorders that affect principally the frontal and temporal lobes of the brain. There are three major associated clinical syndromes, behavioral variant frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA); three principal histologies, involving tau, TDP-43 and FUS proteins; and mutations in three major genes, MAPT, GRN and C9orf72, along with several other less common gene mutations. All three clinical syndromes can exist separately or in combination with Amyotrophic Lateral Sclerosis (ALS). SD is exclusively a TDP-43 proteinopathy, and PNFA may be so, with both showing tight clinical, histological and genetic inter-relationships. bvFTD is more of a challenge with overlapping histological and genetic features, involvement of any of the three aggregating proteins, and changes in any of the three major genes. However, when ALS is present, all cases show a clear histological phenotype with TDP-43 aggregated proteins, and familial forms are associated with expansions in C9orf72. TDP-43 and FUS are nuclear carrier proteins involved in the regulation of RNA metabolism, whereas tau protein - the product of MAPT - is responsible for the assembly/disassembly of microtubules, which are vital for intracellular transport. Mutations in TDP-43 and FUS genes are linked to clinical ALS rather than FTLD (with or without ALS), suggesting that clinical ALS may be a disorder of RNA metabolism. Conversely, the protein products of GRN and C9orf72, along with those of the other minor genes, appear to form part of the cellular protein degradation machinery. It is possible therefore that FTLD is a reflection of dysfunction within lysosomal/proteasomal systems resulting in failure to remove potentially neurotoxic (TDP-43 and tau) aggregates, which ultimately overwhelm capacity to function. Spread of aggregates along distinct pathways may

  16. Abnormal network connectivity in frontotemporal dementia: evidence for prefrontal isolation.

    Science.gov (United States)

    Farb, Norman A S; Grady, Cheryl L; Strother, Stephen; Tang-Wai, David F; Masellis, Mario; Black, Sandra; Freedman, Morris; Pollock, Bruce G; Campbell, Karen L; Hasher, Lynn; Chow, Tiffany W

    2013-01-01

    Degraded social function, disinhibition, and stereotypy are defining characteristics of frontotemporal dementia (FTD), manifesting in both the behavioral variant of frontotemporal dementia (bvFTD) and semantic dementia (SD) subtypes. Recent neuroimaging research also associates FTD with alterations in the brain's intrinsic connectivity networks. The present study explored the relationship between neural network connectivity and specific behavioral symptoms in FTD. Resting-state functional magnetic resonance imaging was employed to investigate neural network changes in bvFTD and SD. We used independent components analysis (ICA) to examine changes in frontolimbic network connectivity, as well as several metrics of local network strength, such as the fractional amplitude of low-frequency fluctuations, regional homogeneity, and seed-based functional connectivity. For each analysis, we compared each FTD subgroup to healthy controls, characterizing general and subtype-unique network changes. The relationship between abnormal connectivity in FTD and behavior disturbances was explored. Across multiple analytic approaches, both bvFTD and SD were associated with disrupted frontolimbic connectivity and elevated local connectivity within the prefrontal cortex. Even after controlling for structural atrophy, prefrontal hyperconnectivity was robustly associated with apathy scores. Frontolimbic disconnection was associated with lower disinhibition scores, suggesting that abnormal frontolimbic connectivity contributes to positive symptoms in dementia. Unique to bvFTD, stereotypy was associated with elevated default network connectivity in the right angular gyrus. The behavioral variant was also associated with marginally higher apathy scores and a more diffuse pattern of prefrontal hyperconnectivity than SD. The present findings support a theory of FTD as a disorder of frontolimbic disconnection leading to unconstrained prefrontal connectivity. Prefrontal hyperconnectivity may

  17. Genetic architecture of sporadic frontotemporal dementia and overlap with Alzheimer's and Parkinson's diseases

    DEFF Research Database (Denmark)

    Ferrari, Raffaele; Wang, Yunpeng; Vandrovcova, Jana

    2017-01-01

    BACKGROUND: Clinical, pathological and genetic overlap between sporadic frontotemporal dementia (FTD), Alzheimer's disease (AD) and Parkinson's disease (PD) has been suggested; however, the relationship between these disorders is still not well understood. Here we evaluated genetic overlap betwee...

  18. Familial frontotemporal lobar degeneration : Phenotypical characterization of presymptomatic and clinical disease stages

    NARCIS (Netherlands)

    E.G.P. Dopper (Elise)

    2015-01-01

    markdownabstractAbstract Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia, and is characterized by neurodegeneration of the frontal and temporal lobes. The disease typically presents with behavioral disturbances and language difficulties that occur

  19. The structural neuroanatomy of music emotion recognition: evidence from frontotemporal lobar degeneration

    NARCIS (Netherlands)

    Omar, R.; Henley, S.M.D.; Bartlett, J.W.; Hailstone, J.C.; Gordon, E.; Sauter, D.A.; Frost, C.; Scott, S.K.; Warren, J.D.

    2011-01-01

    Despite growing clinical and neurobiological interest in the brain mechanisms that process emotion in music, these mechanisms remain incompletely understood. Patients with frontotemporal lobar degeneration (FTLD) frequently exhibit clinical syndromes that illustrate the effects of breakdown in

  20. Comparison of left ventricular outflow geometry and aortic valve area in patients with aortic stenosis by 2-dimensional versus 3-dimensional echocardiography.

    Science.gov (United States)

    Saitoh, Takeji; Shiota, Maiko; Izumo, Masaki; Gurudevan, Swaminatha V; Tolstrup, Kirsten; Siegel, Robert J; Shiota, Takahiro

    2012-06-01

    The present study sought to elucidate the geometry of the left ventricular outflow tract (LVOT) in patients with aortic stenosis and its effect on the accuracy of the continuity equation-based aortic valve area (AVA) estimation. Real-time 3-dimensional transesophageal echocardiography (RT3D-TEE) provides high-resolution images of LVOT in patients with aortic stenosis. Thus, AVA is derived reliably with the continuity equation. Forty patients with aortic stenosis who underwent 2-dimensional transthoracic echocardiography (2D-TTE), 2-dimensional transesophageal echocardiography (2D-TEE), and RT3D-TEE were studied. In 2D-TTE and 2D-TEE, the LVOT areas were calculated as π × (LVOT dimension/2)(2). In RT3D-TEE, the LVOT areas and ellipticity ([diameter of the anteroposterior axis]/[diameter of the medial-lateral axis]) were evaluated by planimetry. The AVA is then determined using planimetry and the continuity equation method. LVOT shape was found to be elliptical (ellipticity of 0.80 ± 0.08). Accordingly, the LVOT areas measured by 2D-TTE (median 3.7 cm(2), interquartile range 3.1 to 4.1) and 2D-TEE (median 3.7 cm(2), interquartile range 3.1 to 4.0) were smaller than those by 3D-TEE (median 4.6 cm(2), interquartile range 3.9 to 5.3; p interquartile range 0.79 to 1.3, p interquartile range 0.64 to 0.94) and 2D-TEE (median 0.76 cm(2), interquartile range 0.62 to 0.95). Additionally, the continuity equation-based AVA by RT3D-TEE was consistent with the planimetry method. In conclusion, RT3D-TEE might allow more accurate evaluation of the elliptical LVOT geometry and continuity equation-based AVA in patients with aortic stenosis than 2D-TTE and 2D-TEE. Copyright © 2012 Elsevier Inc. All rights reserved.

  1. Study on the Status of Health Service Utilization among Caregivers of Left-Behind Children in Poor Rural Areas of Hunan Province: A Baseline Survey.

    Science.gov (United States)

    Ji, Meimei; Zhang, Yefu; Zou, Jiaojiao; Yuan, Tong; Tang, Amber; Deng, Jing; Yang, Lina; Li, Mingzhi; Chen, Jihua; Qin, Hong; Lin, Qian

    2017-08-12

    The caregivers of left-behind children (CLBC) in China's poor, rural areas are mostly elderly and women. Their health status and access to health services have not been previously characterized. This study aims to explore the status of CLBC in terms of their health service utilization and to provide a scientific basis for guiding effective implementation of health policy in rural Hunan. Random cluster sampling was used to survey CLBC in two rural counties. Face-to-face interviews and questionnaires were used to collect data, including socioeconomic status and health service utilization. The two-week prevalence rate was used to reflect health service needs, while the two-week visiting rate, annual hospitalization rate and participation in basic public health services were used to evaluate health service utilization. Of the 518 respondents in the study, 95.9% were farmers and 88.4% were over 40 years old. The two-week prevalence rate was 36.1%. Furthermore, 40.1% of ill caregivers' activities were partly restricted by illness and 3.7% needed to be on bed rest. The two-week visiting rate was 21.0%. The main reasons for not seeing a doctor were "self-medication" (39.1%) or "financial difficulties" (32.6%). The annual hospitalization rate of the CLBC was 22.6% and the non-hospitalization rate of those who needed hospitalization was 41.5%. "Lack of time" (22.3%) and "financial difficulties" (50.5%) were the major factors affecting the utilization of hospitalization services. In terms of participation in basic public health services, only 35.1% CLBC clearly knew that township hospitals have established health records for them. Only 50.6% of caregivers received free health examinations in village clinics or township hospitals and 81.3% of the caregivers did not participate in health education or lectures organized by local health institutions in 2014. The utilization rate of health services was extremely low, which may affect the quality of care for left-behind children

  2. An Open Label Study of Memantine Treatment in Three Subtypes of Frontotemporal Lobar Degeneration

    OpenAIRE

    Boxer, Adam L.; Lipton, Anne M.; Womack, Kyle; Merrilees, Jennifer; Neuhaus, John; Pavlic, Danijela; Gandhi, Anisha; Red, Dana; Kristen-Martin-Cook; Svetlik, Doris; Miller, Bruce L.

    2009-01-01

    There are currently no FDA-approved treatments for frontotemporal lobar degeneration (FTLD). The objectives of this study were to explore the tolerability of memantine treatment in FTLD and to monitor for possible effects on behavior, cognition and function. 43 individuals who met clinical criteria for FTLD (21 with frontotemporal dementia [FTD], 13 with semantic dementia [SD] and 9 with progressive nonfluent aphasia [PA]) received 26 weeks of open label treatment with memantine at a target d...

  3. Frontotemporal Dementia Complicated by Comorbid Borderline Personality Disorder: A Case Report

    OpenAIRE

    Salzbrenner, LCDR Stephen; Brown, Jaime; Hart, Gavin; Dettmer, Ens Jonathan; Williams, LT Raquel; Ormeno, LT Monica; O’Neal, LCDR Ethel; Shippy, LT Jennifer

    2009-01-01

    Frontotemporal dementia is the fourth most common cause of dementia in the United States and characteristically presents with an early decline in social conduct, impaired regulation of interpersonal conduct, emotional blunting, and general loss of insight, with relative preservation of memory. This a case of frontotemporal dementia in a 46-year-old woman who presented with existing diagnoses of borderline personality disorder and major depressive disorder. She had been repeatedly evaluated fo...

  4. Left ventricular mass regression is independent of gradient drop and effective orifice area after aortic valve replacement with a porcine bioprosthesis.

    Science.gov (United States)

    Sádaba, Justo Rafael; Herregods, Marie-Christine; Bogaert, Jan; Harringer, Wolfgang; Gerosa, Gino

    2012-11-01

    The question of whether left ventricular mass (LVM) regression following aortic valve replacement (AVR) is affected by the prosthesis indexed effective orifice area (IEOA) and transprosthetic gradient has not been fully elucidated. Data from a prospective, core-laboratory-reviewed echocardiography and magnetic resonance imaging (MRI) study was used to determine if the degree of LVM regression following AVR with two types of porcine bioprosthesis in patients suffering from predominant aortic valve stenosis (AS) was related to the prosthesis IEOA and transprosthetic gradient. Over a two-year period, 149 patients enrolled at eight centers received either an Epic or an Epic Supra aortic bioprosthesis (St. Jude Medical, MN, USA). Preoperative valve dysfunction was pure AS in 54 patients (36%) and mixed valve disease (primarily stenosis) in 95 patients (64%). LVM was determined preoperatively and at six months postoperatively, using MRI. The prosthesis IEOA and transprosthetic gradient were calculated at six months by means of echocardiography. Data were available for 111 patients at both enrolment and six months postoperatively. The LVM at enrolment and at follow up was 154.96 +/- 42.50 g and 114.83 +/- 29.20 g, respectively (p regression methods, showed LVM regression to be independent of the mean systolic pressure gradient, peak systolic pressure and prosthesis IEOA at six months (p = 0.53, 0.43, and 0.15, respectively). At six months after AVR with a porcine bioprosthesis to treat AS, there was a significant LVM regression that was independent of the prosthesis IEOA and the mean systolic pressure gradient and peak systolic pressure.

  5. Different apathy clinical profile and neural correlates in behavioral variant frontotemporal dementia and Alzheimer's disease.

    Science.gov (United States)

    Fernández-Matarrubia, Marta; Matías-Guiu, Jordi A; Cabrera-Martín, María Nieves; Moreno-Ramos, Teresa; Valles-Salgado, María; Carreras, José Luis; Matías-Guiu, Jorge

    2018-01-01

    Apathy is one of the most common and disabling syndromes of dementia. Clinical apathy expression and neuroanatomical basis of apathy seem to differ between behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD), although evidence is scarce and poorly understood. Our main purposes were to compare the clinical apathy profile from patients with bvFTD and AD and analyze the relationship between apathy and brain metabolism measured using positron emission tomography imaging with 18 F fluorodeoxyglucose (FDG-PET). Forty-two bvFTD, 42 AD, and 30 healthy volunteers without cognitive or behavioral complaints were included. Apathy was defined using Robert's 2009 diagnostic criteria, and specific apathy characteristics were assessed with the Lille Apathy Rating Scale. All participants underwent FDG-PET brain scan to provide data for voxel-based morphometric analysis. Multivariate analysis showed that subjects affected by bvFTD displayed greater impairment of emotional apathy and self-awareness in comparison with AD sample. Additionally, FDG-PET imaging analyses revealed that apathy was associated with different neuroanatomical substrates in each dementia group: left lateral prefrontal, medial frontal/anterior cingulate, lateral orbitofrontal and anterior insular cortices in bvFTD, and right anterior cingulate in AD. These results support that apathy is a complex syndrome, with different clinical expressions across different pathological conditions. Those differences in qualitative aspects of apathy seem to be associated with differences in the damage sites, as shown by our FDG-PET imaging analysis. Our findings provide a better knowledge about pathophysiology of apathy in dementia, which could have practical implications for therapeutic management. Copyright © 2017 John Wiley & Sons, Ltd. Copyright © 2017 John Wiley & Sons, Ltd.

  6. Value of 18F-FDG PET in differentiating Alzheimer's disease with frontotemporal dementia

    Directory of Open Access Journals (Sweden)

    Rui-xue CUI

    2014-03-01

    Full Text Available Objective To delineate the pattern of reduction of cerebral glucose metabolism in patients with Alzheimer's disease (AD and frontotemporal dementia (FTD and investigate the value of 18F-FDG PET in the differential diagnosis. Methods Twenty patients with FTD (behavioral variant and 20 AD patients underwent 18F-FDG PET scanning. All the images were compared with that from 20 healthy age-matched control subjects on a voxel-based analysis (VBA using SPM5. Visual analyses of 18F-FDG PET were performed by 2 independent nuclear medicine specialists who were blinded to the clinical background. Results 1 The PET scans of all the patients in 2 groups presented impairment of cortical metabolism. 2 Subjects with AD showed hypometabolism in the bilateral temporoparietal association cortex and posterior cingulate cortex, and hypometabolim in part of bilateral frontal lobes was observed in patients with progression. The metabolic activity was relatively kept in the primary motor-sensor cortex, occipital lobes and subcortical structures (basal ganglia and thalamus. The asymmetric hemispheric hypometabolic involvement was rare and observed in only 2 of 20 cases. 3 Subjects with FTD showed a significant hypometabolism of the frontal lobes and anterior temporal lobes, accompanied by mild to moderate reductions in glucose metabolism in parietal cortices and subcortical structures. The asymmetric hemispheric hypometabolic involvement was commonly observed in 16 of 20 cases with right-dominant type in 4 of 16 cases and left-dominant type in 12 cases. Conclusions 18F-FDG PET is a reliable diagnostic test in distinguishing FTD from AD due to the sharp contrast pattern of cerebral glucose hypometabolism.

  7. Frontotemporal Dysfunction in Amyotrophic Lateral Sclerosis: A Discriminant Function Analysis.

    Science.gov (United States)

    Nidos, Andreas; Kasselimis, Dimitrios S; Simos, Panagiotis G; Rentzos, Michael; Alexakis, Theodoros; Zalonis, Ioannis; Zouvelou, Vassiliki; Potagas, Constantin; Evdokimidis, Ioannis; Woolley, Susan C

    2016-01-01

    There is growing evidence for extramotor dysfunction (EMd) in amyotrophic lateral sclerosis (ALS), with a reported prevalence of up to 52%. In the present study, we explore the clinical utility of a brief neuropsychological battery for the investigation of cognitive, behavioral, and language deficits in patients with ALS. Thirty-four consecutive ALS patients aged 44-89 years were tested with a brief neuropsychological battery, including executive, behavioral, and language measures. Patients were initially classified as EMd or non-EMd based on their scores on the frontal assessment battery (FAB). Between-group comparisons revealed significant differences in all measures (p < 0.01). Discriminant analysis resulted in a single canonical function, with all tests serving as significant predictors. This function agreed with the FAB in 13 of 17 patients screened as EMd and identified extramotor deficits in 2 additional patients. Overall sensitivity and specificity estimates against FAB were 88.2%. We stress the importance of discriminant function analysis in clinical neuropsychological assessment and argue that the proposed neuropsychological battery may be of clinical value, especially when the option of extensive and comprehensive neuropsychological testing is limited. The psychometric validity of an ALS-frontotemporal dementia diagnosis using neuropsychological tests is also discussed. © 2015 S. Karger AG, Basel.

  8. Hippocampal sclerosis dementia: An amnesic variant of frontotemporal degeneration

    Directory of Open Access Journals (Sweden)

    Chiadi U. Onyike

    Full Text Available ABSTRACT Objective: To describe characteristics of hippocampal sclerosis dementia. Methods: Convenience sample of Hippocampal sclerosis dementia (HSD recruited from the Johns Hopkins University Brain Resource Center. Twenty-four cases with post-mortem pathological diagnosis of hippocampal sclerosis dementia were reviewed for clinical characterization. Results: The cases showed atrophy and neuronal loss localized to the hippocampus, amygdala and entorrhinal cortex. The majority (79.2% had amnesia at illness onset, and many (54.2% showed abnormal conduct and psychiatric disorder. Nearly 42% presented with an amnesic state, and 37.5% presented with amnesia plus abnormal conduct and psychiatric disorder. All eventually developed a behavioral or psychiatric disorder. Disorientation, executive dysfunction, aphasia, agnosia and apraxia were uncommon at onset. Alzheimer disease (AD was the initial clinical diagnosis in 89% and the final clinical diagnosis in 75%. Diagnosis of frontotemporal dementia (FTD was uncommon (seen in 8%. Conclusion: HSD shows pathological characteristics of FTD and clinical features that mimic AD and overlap with FTD. The findings, placed in the context of earlier work, support the proposition that HSD belongs to the FTD family, where it may be identified as an amnesic variant.

  9. Individual Music Therapy with Persons with Frontotemporal Dementia

    DEFF Research Database (Denmark)

    Ridder, Hanne Mette Ochsner; Aldridge, David

    2005-01-01

    It is possible to slow down the progression of Alzheimer’s disease with pharmacological treatment. When this treatment is given to people with types of dementia that affect the frontal and temporal lobes (Frontotemporal Dementia) the results are discouraging. It is observed that the patients show...... is an integration of a relational music therapy approach and a more physiologically based arousal model, and is here illustrated in a case study research that integrated both qualitative and quantitative data in a flexible research design....... pronounced restlessness and mania. In this article we describe a non-pharmacological psychosocial approach, music therapy, and how it is possible to work with this method when constitutional, regulative, dialogical, and integrative aspects are included. The focus is on therapeutic singing where well known...... songs are applied in order to build up structure and stability and/or as means of arousal regulation. Songs with personal meaning make it possible to acknowledge the person’s emotions, breaking the social isolation, and meeting the music therapy participant’s psychosocial needs. The clinical approach...

  10. Urinary incontinence and its functional anatomy in frontotemporal lobar degenerations

    Energy Technology Data Exchange (ETDEWEB)

    Perneczky, Robert [Technical University Munich Medical School, Department of Psychiatry and Psychotherapy, Munich (Germany); Technische Universitaet Muenchen, Klinik und Poliklinik fuer Psychiatrie und Psychotherapie, Muenchen (Germany); Diehl-Schmid, Janine; Foerstl, Hans; Kurz, Alexander [Technical University Munich Medical School, Department of Psychiatry and Psychotherapy, Munich (Germany); Drzezga, Alexander [Technical University Munich Medical School, Department of Nuclear Medicine, Munich (Germany); May, Florian [Technical University Munich Medical School, Department of Urology, Munich (Germany)

    2008-03-15

    The frontal lobes play a crucial role in micturition control. However, no reports exist on the functional role of distinct frontal brain regions in urinary incontinence (UIC) in patients with a neurodegenerative damage of the frontal lobe. The aim of the present study was therefore to explore if functional brain lesions were associated with UIC in patients suffering from frontotemporal lobar degenerations (FTLD). Forty-four patients, including eight incontinent subjects, underwent cranial positron emission tomography scanning with {sup 18}F-fluoro-2-deoxy-glucose ({sup 18}F-FDG PET) to assess the relative metabolic rate of glucose (rCMRglc). Group comparisons of rCMRglc were conducted in SPM2 to identify brain regions where the group of incontinent patients (FTLD+UIC) had significant alterations compared with the group without UIC (FTLD-UIC). At the stringent statistical threshold of p < 0.05, corrected for multiple comparisons according to the family-wise error rate, the statistical analysis revealed two significant right-hemispheric hypometabolic clusters located in the premotor/anterior cingulate cortex and the putamen/claustrum/insula. No hypermetabolic regions were found. The present study is the first to provide evidence for brain functional alterations involved in the occurrence of UIC in FTLD. These results provide an important piece of evidence to the understanding of a particularly distressing autonomic nervous system symptom of dementia. (orig.)

  11. Diagnosing, monitoring and managing behavioural variant frontotemporal dementia.

    Science.gov (United States)

    Piguet, Olivier; Kumfor, Fiona; Hodges, John

    2017-09-02

    Behavioural variant frontotemporal dementia is characterised by insidious changes in personality and interpersonal conduct that reflect progressive disintegration of the neural circuits involved in social cognition, emotion regulation, motivation and decision making. The underlying pathology is heterogeneous and classified according to the presence of intraneuronal inclusions of tau, TDP-43 or, occasionally, fused in sarcoma proteins. Biomarkers to detect these histopathological changes in life are increasingly important with the development of disease-modifying drugs. A number of gene abnormalities have been identified, the most common being an expansion in the C9orf72 gene, which together account for most familial cases. The 2011 international consensus criteria propose three levels of diagnostic certainty: possible, probable and definite. Detailed history taking from family members to elicit behavioural features underpins the diagnostic process, with support from neuropsychological testing designed to detect impairment in decision making, emotion processing and social cognition. Brain imaging is important for increasing the level of diagnosis certainty over time. Carer education and support remain of paramount importance.

  12. Pain perception and tolerance in patients with frontotemporal dementia.

    Science.gov (United States)

    Carlino, Elisa; Benedetti, Fabrizio; Rainero, Innocenzo; Asteggiano, Giovanni; Cappa, Giorgia; Tarenzi, Luisella; Vighetti, Sergio; Pollo, Antonella

    2010-12-01

    Pain management in elderly people with cognitive impairment poses special challenges, due to difficulties in pain assessment and specific neurodegenerative changes along pain pathways. Most studies have concentrated on Alzheimer's disease (AD) patients, in whom some contrasting findings have been found. For example, while psychophysical data suggest a selective blunting of the affective dimension of pain, pain-related fMRI signal increases have also been described. Few data have been reported in patients with frontotemporal dementia (FTD). By electrical stimulation, we have measured pain threshold and pain tolerance in clinically diagnosed FTD patients with SPECT cerebral hypoperfusion. We performed our analysis on two separate and overlapping subgroups selected on the basis of (1) neuropsychological scores below cut-off values (2) a strictly localized frontal and/or temporal hypoperfusion. We observed increased pain threshold in the first group and increased pain threshold and pain tolerance in the second group. Our results suggest differences in pain processing changes in distinct types of dementia, while at the same time caution that pain perception assessment may depend on the criteria adopted for diagnosis. Copyright © 2010 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.

  13. ARE LEFT HANDED SURGEONS LEFT OUT?

    OpenAIRE

    SriKamkshi Kothandaraman; Balasubramanian Thiagarajan

    2012-01-01

    Being a left-handed surgeon, more specifically a left-handed ENT surgeon, presents a unique pattern of difficulties.This article is an overview of left-handedness and a personal account of the specific difficulties a left-handed ENT surgeon faces.

  14. Presymptomatic generalized brain atrophy in frontotemporal dementia caused by CHMP2B mutation

    DEFF Research Database (Denmark)

    Rohrer, Jonathan D; Ahsan, R Laila; Isaacs, Adrian M

    2009-01-01

    BACKGROUND/AIMS: CHMP2B mutations are a rare cause of familial frontotemporal dementia (FTD). The clinical syndrome is dominated by personality change and behavioural symptoms, but language, memory, calculation and praxis impairments are also seen early in the course of the disease. There are no ......BACKGROUND/AIMS: CHMP2B mutations are a rare cause of familial frontotemporal dementia (FTD). The clinical syndrome is dominated by personality change and behavioural symptoms, but language, memory, calculation and praxis impairments are also seen early in the course of the disease...... gene mutation carriers. CONCLUSIONS: This finding suggests that mutations in CHMP2B have widespread effects throughout the brain, leading to a neuro-anatomical signature distinct from other diseases in the frontotemporal lobar degeneration spectrum....

  15. Frontotemporal dementia with severe thalamic involvement : a clinical and neuropathological study

    Directory of Open Access Journals (Sweden)

    Radanovic Márcia

    2003-01-01

    Full Text Available Frontotemporal dementia (FTD is the third-leading cause of cortical dementia after Alzheimer's disease and Lewy body dementia, and is characterized by a dementia where behavioral disturbances are prominent and appear early in the course of the disease. We report the case of a 58 year-old man affected by dementia with behavioral disturbances, in addition to rigid-hypokinetic and a lower motor neuron syndrome that were present at later stages of the illness. Neuroimaging studies showed frontotemporal atrophy. Neuropathological studies revealed intense thalamic neuronal loss and astrocytic gliosis, as well as moderate frontotemporal neuronal loss, astrocytosis and spongiform degeneration. Thalamic degeneration has previously been described among the wide group of neuropathological features of FTD. The aim of the present study is to show the clinical and neuropathological aspects of thalamic degeneration in FTD, along with its role in behavioral disturbances, a common finding in this condition.

  16. Dural based primary osteosarcoma in right fronto-temporal region with review of literature.

    Science.gov (United States)

    Ghosal, Nandita; Dadlani, Ravi; Furtado, Sunil V; Bagdi, Naman; Hegde, A S

    2010-01-01

    We report a case of primary dural based osteosarcoma in the right fronto-temporal convexity in a 43-year-old female who presented with a short history of seizure and headache. Radiologic evaluation revealed a well defined brightly enhancing extra-axial lesion in the right fronto-temporal region with a dural tail around the sylvian fissure. The overlying bone was uninvolved. Paraffin section of the tumor showed plump cells with moderate nuclear and cellular pleomorphism with eosinophilic extracellular material (osteiod) between the cells. At a few places, lace like osteiod was seen encasing individual cells signifying osteiod being formed by tumor cells. Immunohistochemistry for EMA was focally positive and negative for S-100 protein and GFAP. A final histopathological diagnosis of dural based primary osteosarcoma of the right fronto-temporal region was rendered. To the best of our knowledge this will be the eighth such case in literature.

  17. Analysis on early clinical features of behavioral variant frontotemporal dementia

    Directory of Open Access Journals (Sweden)

    Guan-jun LI

    2017-11-01

    Full Text Available Background Although the early behavioral symptoms of behavioral variant frontotemporal dementia (bvFTD are prominent, early diagnosis for bvFTD is difficult due to confusion with other mental disorders, and lack of sensitivity and specificity of diagnostic criteria, etc. In this paper, we summarized the important reviews in recent years and analyzed the clinical characteristics of bvFTD patients to improve the detection of early symptoms in bvFTD. Methods Twenty-three possible or probable bvFTD patients were diagnosed according to International Behavioral Variant Frontotemporal Dementia Criteria Consortium (FTDC. Self-designed questionnaires designed by Shanghai Mental Health Center were used to collect sociodemographic data and general information of patients. Their clinical characteristics were summarized, including abnormal behaviors, cognitive impairment, psychotic symptoms and other symptoms. Mini-Mental State Examination (MMSE, Activities of Daily Living (ADL and Clinical Dementia Rating Scale (CDR were used to make neuropsychological tests and compare with similar overseas studies (control group, N = 66. Results Eleven male patients and 12 female patients were included in our study. Compared with control group, the average age of onset [(50.83 ± 11.55 years vs. (57.00 ± 10.00 years; t = 3.863, P = 0.000] and average age of diagnosis [(53.22 ± 11.55 years vs. (61.00 ± 9.00 years; t = 13.423, P = 0.000] of bvFTD patients were smaller. The study showed that bvFTD patients had more apathy or indolence [95.65% (22/23 vs. 65.15% (43/66; χ2 = 8.057, P = 0.005], loss of sympathy or empathy [95.65% (22/23 vs. 33.33% (22/66; χ2 = 26.499, P = 0.000], while patients in control group showed more derepression behavior [98.48% (65/66 vs. 52.17% (12/23; χ2 = 27.514, P = 0.000] and continuous, stiff, obsessive and/or ritualized behavior [95.45% (63/66 vs. 30.43% (7/23; adjusted χ2 = 39.159, P = 0.000]. For cognitive impairment, bvFTD patients

  18. Optical coherence tomography identifies outer retina thinning in frontotemporal degeneration.

    Science.gov (United States)

    Kim, Benjamin J; Irwin, David J; Song, Delu; Daniel, Ebenezer; Leveque, Jennifer D; Raquib, Aaishah R; Pan, Wei; Ying, Gui-Shuang; Aleman, Tomas S; Dunaief, Joshua L; Grossman, Murray

    2017-10-10

    Whereas Alzheimer disease (AD) is associated with inner retina thinning visualized by spectral-domain optical coherence tomography (SD-OCT), we sought to determine if the retina has a distinguishing biomarker for frontotemporal degeneration (FTD). Using a cross-sectional design, we examined retinal structure in 38 consecutively enrolled patients with FTD and 44 controls using a standard SD-OCT protocol. Retinal layers were segmented with the Iowa Reference Algorithm. Subgroups of highly predictive molecular pathology (tauopathy, TAR DNA-binding protein 43, unknown) were determined by clinical criteria, genetic markers, and a CSF biomarker (total tau: β-amyloid) to exclude presumed AD. We excluded eyes with poor image quality or confounding diseases. SD-OCT measures of patients (n = 46 eyes) and controls (n = 69 eyes) were compared using a generalized linear model accounting for intereye correlation, and correlations between retinal layer thicknesses and Mini-Mental State Examination (MMSE) were evaluated. Adjusting for age, sex, and race, patients with FTD had a thinner outer retina than controls (132 vs 142 μm , p = 0.004). Patients with FTD also had a thinner outer nuclear layer (ONL) (88.5 vs 97.9 μm, p = 0.003) and ellipsoid zone (EZ) (14.5 vs 15.1 μm, p = 0.009) than controls, but had similar thicknesses for inner retinal layers. The outer retina thickness of patients correlated with MMSE (Spearman r = 0.44, p = 0.03). The highly predictive tauopathy subgroup (n = 31 eyes) also had a thinner ONL (88.7 vs 97.4 μm, p = 0.01) and EZ (14.4 vs 15.1 μm, p = 0.01) than controls. FTD is associated with outer retina thinning, and this thinning correlates with disease severity. © 2017 American Academy of Neurology.

  19. Beyond words: Pragmatic inference in behavioral variant of frontotemporal degeneration.

    Science.gov (United States)

    Spotorno, Nicola; McMillan, Corey T; Rascovsky, Katya; Irwin, David J; Clark, Robin; Grossman, Murray

    2015-08-01

    When the message of a speaker goes beyond the literal or logical meaning of the sentences used, a pragmatic inference is required to understand the complete meaning of an utterance. Here we study one example of pragmatic inference, called scalar implicature. Such an inference is required when a weaker term "some" is used in a sentence like "Some of the students passed the exam" because the speaker presumably had a reason not to use a stronger term like "all". We investigated the comprehension of scalar implicatures in a group of 17 non-aphasic patients with behavioral variant frontotemporal degeneration (bvFTD) in order to test the contribution of non-linguistic decision-making ability and the role of prefrontal cortex in supporting the computation of pragmatic inferences. The results of two experiments point to a deficit in producing alternative interpretations beyond a logical reading. bvFTD patients thus prefer the narrowly literal or logical interpretation of a scalar term when they must generate a possible alternative interpretation by themselves, but patients prefer a pragmatic reading when offered a choice between the logical and the pragmatic interpretation of the same sentence. An imaging analysis links bvFTD patients' spontaneous tendency toward a narrowly logical interpretation with atrophy in ventromedial prefrontal cortex. Our findings are consistent with the pragmatic tolerance hypothesis, which proposes that difficulty generating alternative interpretations of an utterance, rather than a frank inability to compute an inference, affects the comprehension of a scalar term. Copyright © 2015 Elsevier Ltd. All rights reserved.

  20. Neuropsychological differences between frontotemporal lobar degeneration and Alzheimer's disease

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    Claudia Sellitto Porto

    Full Text Available Abstract Memory impairment is the main clinical feature in Alzheimer disease (AD, whereas in frontotemporal lobar degeneration (FTLD behavioral and language disorders predominate. Objectives: To investigate possible differences between the neuropsychological performance in FTLD and AD. Methods: Fifty-six AD patients (mean age=72.98±7.43; mean schooling=9.62±4.68; 35 women and 21 men, 17 FTLD patients (mean age=67.64±7.93; mean schooling=12.12±4.77; 9 women and 8 men, and 60 controls (mean age=68.90±7.48; mean schooling=10.72±4.74; 42 women and 18 men were submitted to a Dementia Rating Scale (DRS and a comprehensive neuropsychological evaluation composed of tasks assessing attention, visuoperceptual abilities, constructive abilities, executive functions, memory and language. Results: DRS total score and subscales were not able to differentiate FTLD from AD patients. However, FTLD and AD patients showed statistically significant differences in performance in tests of verbal (Logical Memory, Rey Auditory Verbal Learning Test and visual (Visual Reproduction, recall of the Rey Complex Figure episodic memory, verbal immediate memory (Logical Memory, attention with interference (Trail Making Test - Part B, verbal fluency (semantic and phonemic and concept formation (WCST. Conclusion: Contrary to expectations, only a few tasks executive function tasks (Trail Making Test - Part B, F.A.S. and WCST and two memory tests (verbal and visual episodic memory tests were able to differentiate between FTLD and AD patients.

  1. Functional communication ability in frontotemporal lobar degeneration and Alzheimer's disease

    Directory of Open Access Journals (Sweden)

    Isabel Albuquerque M. de Carvalho

    Full Text Available Abstract Functional communication is crucial for independent and efficient communicative behavior in response to every day activities. In the course of dementia progression, cognitive losses may impair these abilities. For this reason, functional communication assessment should be part of a formal assessment to quantify and qualify the impact of deficiency on patients' lives. Objective: To compare functional communication abilities in fronto-temporal lobar degeneration (FLTD and Alzheimer's disease (AD. Methods: Six AD patients (mean age: 82.50±2.66 years; mean education: 5.67±3.61 years, and eight FTLD patients (mean age: 57.13±9.63 years; mean education: 10.86±6.91 years had their close relatives answer the Functional Assessment of Communication Skills for Adults (Asha-facs . Statistical analyses correlated the performance on each of the Asha-facs domains (social communication, communication of basic needs; reading, writing, number concept and daily planning between both groups. Results: Analyses showed that functional communication was similar for AD and FTLD patients. Only two items had statistical difference, namely 'Comprehension of inference' (AD 6.7±1.33; FTLD 2.43±2.30, p=0.017 and 'capacity to make basic money transactions' (AD 2.17±2.04; FTLD 4.00±0.90, p=0.044. Comparison among the four domains' mean scores revealed no significant difference. Conclusion: The Asha-facs is a useful instrument to characterize functional communication abilities in both FTLD and AD. Nevertheless, the analysis presented for this sample showed that the Asha-facs could not discriminate which aspects of the FTLD and AD differed.

  2. Weighted Protein Interaction Network Analysis of Frontotemporal Dementia.

    Science.gov (United States)

    Ferrari, Raffaele; Lovering, Ruth C; Hardy, John; Lewis, Patrick A; Manzoni, Claudia

    2017-02-03

    The genetic analysis of complex disorders has undoubtedly led to the identification of a wealth of associations between genes and specific traits. However, moving from genetics to biochemistry one gene at a time has, to date, rather proved inefficient and under-powered to comprehensively explain the molecular basis of phenotypes. Here we present a novel approach, weighted protein-protein interaction network analysis (W-PPI-NA), to highlight key functional players within relevant biological processes associated with a given trait. This is exemplified in the current study by applying W-PPI-NA to frontotemporal dementia (FTD): We first built the state of the art FTD protein network (FTD-PN) and then analyzed both its topological and functional features. The FTD-PN resulted from the sum of the individual interactomes built around FTD-spectrum genes, leading to a total of 4198 nodes. Twenty nine of 4198 nodes, called inter-interactome hubs (IIHs), represented those interactors able to bridge over 60% of the individual interactomes. Functional annotation analysis not only reiterated and reinforced previous findings from single genes and gene-coexpression analyses but also indicated a number of novel potential disease related mechanisms, including DNA damage response, gene expression regulation, and cell waste disposal and potential biomarkers or therapeutic targets including EP300. These processes and targets likely represent the functional core impacted in FTD, reflecting the underlying genetic architecture contributing to disease. The approach presented in this study can be applied to other complex traits for which risk-causative genes are known as it provides a promising tool for setting the foundations for collating genomics and wet laboratory data in a bidirectional manner. This is and will be critical to accelerate molecular target prioritization and drug discovery.

  3. Left atrial appendage occlusion

    Directory of Open Access Journals (Sweden)

    Ahmad Mirdamadi

    2013-01-01

    Full Text Available Left atrial appendage (LAA occlusion is a treatment strategy to prevent blood clot formation in atrial appendage. Although, LAA occlusion usually was done by catheter-based techniques, especially percutaneous trans-luminal mitral commissurotomy (PTMC, it can be done during closed and open mitral valve commissurotomy (CMVC, OMVC and mitral valve replacement (MVR too. Nowadays, PTMC is performed as an optimal management of severe mitral stenosis (MS and many patients currently are treated by PTMC instead of previous surgical methods. One of the most important contraindications of PTMC is presence of clot in LAA. So, each patient who suffers of severe MS is evaluated by Trans-Esophageal Echocardiogram to rule out thrombus in LAA before PTMC. At open heart surgery, replacement of the mitral valve was performed for 49-year-old woman. Also, left atrial appendage occlusion was done during surgery. Immediately after surgery, echocardiography demonstrates an echo imitated the presence of a thrombus in left atrial appendage area, although there was not any evidence of thrombus in pre-pump TEE. We can conclude from this case report that when we suspect of thrombus of left atrial, we should obtain exact history of previous surgery of mitral valve to avoid misdiagnosis clotted LAA, instead of obliterated LAA. Consequently, it can prevent additional evaluations and treatments such as oral anticoagulation and exclusion or postponing surgeries including PTMC.

  4. Functional mapping of left parietal areas involved in simple addition and multiplication. A single-case study of qualitative analysis of errors.

    Science.gov (United States)

    Della Puppa, Alessandro; De Pellegrin, Serena; Salillas, Elena; Grego, Alberto; Lazzarini, Anna; Vallesi, Antonino; Saladini, Marina; Semenza, Carlo

    2015-09-01

    All electrostimulation studies on arithmetic have so far solely reported general errors. Nonetheless, a classification of the errors during stimulation can inform us about underlying arithmetic processes. The present electrostimulation study was performed in a case of left parietal glioma. The patient's erroneous responses suggested that calculation was mainly applied for addition and a combination of retrieval and calculation was mainly applied for multiplication. The findings of the present single-case study encourage follow up with further data collection with the same paradigm. © 2014 The British Psychological Society.

  5. South-east frontier of the Russian Empire and the processes on the division of the Ural River left bank area between Kirghiz-Kaysaks and Ural Cossacks in the second half of the XIX century

    Directory of Open Access Journals (Sweden)

    Alexey I. Kortunov

    2015-03-01

    Full Text Available The article highlights the issue related to the peculiarities of formation of the south-east frontier of the Russian Empire and with the process on separation of the border areas of the Orenburg line (in particular of the Ural River left bank area between Kirghiz-Kaysaks and Ural Cossacks in the second half of XIX century. The author pays particular attention to the problem of the resolution of disputes between the Ural Cossacks and Kirghiz-Kaysaks by local and central authorities.

  6. Criminal behavior in frontotemporal dementia and Alzheimer disease.

    Science.gov (United States)

    Liljegren, Madeleine; Naasan, Georges; Temlett, Julia; Perry, David C; Rankin, Katherine P; Merrilees, Jennifer; Grinberg, Lea T; Seeley, William W; Englund, Elisabet; Miller, Bruce L

    2015-03-01

    Neurodegenerative diseases can cause dysfunction of neural structures involved in judgment, executive function, emotional processing, sexual behavior, violence, and self-awareness. Such dysfunctions can lead to antisocial and criminal behavior that appears for the first time in the adult or middle-aged individual or even later in life. To investigate the frequency and type of criminal behavior among patients with a diagnosed dementing disorder. We conducted a retrospective medical record review of 2397 patients who were seen at the University of California, San Francisco, Memory and Aging Center between 1999 and 2012, including 545 patients with Alzheimer disease (AD), 171 patients with behavioral variant of frontotemporal dementia (bvFTD), 89 patients with semantic variant of primary progressive aphasia, and 30 patients with Huntington disease. Patient notes containing specific keywords denoting criminal behavior were reviewed. Data were stratified by criminal behavior type and diagnostic groups. Frequencies of criminal behavior and χ² statistics were calculated. Of the 2397 patients studied, 204 (8.5%) had a history of criminal behavior that emerged during their illness. Of the major diagnostic groups, 42 of 545 patients (7.7%) with AD, 64 of 171 patients (37.4%) with bvFTD, 24 of 89 patients (27.0%) with semantic variant of primary progressive aphasia, and 6 of 30 patients (20%) with Huntington disease exhibited criminal behavior. A total of 14% of patients with bvFTD were statistically significantly more likely to present with criminal behavior compared with 2% of patients with AD (P criminal behavior in the bvFTD group included theft, traffic violations, sexual advances, trespassing, and public urination in contrast with those in the AD group, who commonly committed traffic violations, often related to cognitive impairment. Criminal behavior is more common in patients with bvFTD and semantic variant of primary progressive aphasia than in those with AD and

  7. [Reconciliating neurology and psychiatry: The prototypical case of frontotemporal dementia].

    Science.gov (United States)

    Lagarde, J; Sarazin, M

    2017-10-01

    Frontotemporal degeneration (FTD) in its behavioral variant (bvFTD) is probably one of the conditions that best illustrates the links between psychiatry and neurology. It is indeed admitted that between a third and half of patients with this condition, especially in early-onset forms, receive an initial diagnosis of psychiatric disorder (depression, schizophrenia, bipolar disorder) and are then referred to a psychiatric ward. BvFTD can thus be considered a neurological disorder with a psychiatric presentation. Among psychiatric symptoms reported in this disease, psychotic symptoms (hallucinations, delusions, especially of persecution), which have long been underestimated in bvFTD and are not part of the current diagnostic criteria, are present in about 20% of cases and may be inaugural. They are particularly common in the genetic forms related to a mutation in the C9orf72 gene (up to 50%), and to a lesser extent in the GRN gene (up to 25%). C9orf72 gene mutation is often associated with a family history of dementia or motor neuron disease but also of psychiatric disorders. It has also been described in sporadic presentation forms. Sometimes, the moderate degree of brain atrophy on MRI described in patients carrying this mutation may complicate the differential diagnosis with late-onset psychiatric diseases. In the present article, we underline the importance of considering that psychiatric - especially psychotic - symptoms are not rare in bvFTD, which should lead to a revision of the diagnostic criteria of this disease by taking greater account of this fact. We also propose a diagnostic chart, based on concerted evaluation by neurologists and psychiatrists for cases of atypical psychiatric symptoms (late-onset or pharmacoresistant troubles) leading to consider the possibility of a neurological disorder, in order to shed a new light on these difficult clinical situations. In the field of research, bvFTD may constitute a model to explore the neural basis of certain

  8. Inferior parietal transcranial direct current stimulation with training improves cognition in anomic Alzheimer's disease and frontotemporal dementia.

    Science.gov (United States)

    Roncero, Carlos; Kniefel, Heike; Service, Erik; Thiel, Alexander; Probst, Stephan; Chertkow, Howard

    2017-06-01

    We evaluated whether transcranial direct current stimulation (tDCS) can improve picture-naming abilities in subjects with anomic Alzheimer or frontotemporal dementias. Using a double-blind crossover design, 10 participants were trained on picture naming over a series of 10 sessions with either 30 minutes of anodal (2 mA) tDCS stimulation to the left inferior parieto-temporal region (P3) or sham stimulation. We evaluated performance on a trained picture-naming list, an equivalent untrained list, and additional neuropsychological tasks. Participants improved significantly more receiving real stimulation rather than sham stimulation (40% vs. 19%, P  < .01), lasting at least 2 weeks after stimulation. Furthermore, these participants showed a small increase for untrained picture-naming items and digit span when they received real stimulation but a decrease when sham stimulation was received. tDCS stimulation has promise as a treatment for anomia in demented individuals and the effect can generalize to unstudied items as well as other cognitive abilities.

  9. The fronto-temporal component in mild and moderately severe head injury

    NARCIS (Netherlands)

    Minderhoud, JM; vanZomeren, AH; vanderNaalt, J

    The history of the identification of the so-called (fronto-)temporal lobe contusion is reviewed. Treatment of minor head injuries actually starts with the right diagnosis. Injuries of the temporal lobe, characterized by a comparatively long period of post-traumatic amnesia should be distinguished

  10. Frontotemporal dementia caused by CHMP2B mutation is characterised by neuronal lysosomal storage pathology

    DEFF Research Database (Denmark)

    Clayton, Emma L.; Mizielinska, Sarah; Edgar, James R.

    2015-01-01

    Mutations in the charged multivesicular body protein 2B (CHMP2B) cause frontotemporal dementia (FTD). We report that mice which express FTD-causative mutant CHMP2B at physiological levels develop a novel lysosomal storage pathology characterised by large neuronal autofluorescent aggregates. The a...

  11. Frontotemporal dementia and its subtypes: A genome-wide association study

    NARCIS (Netherlands)

    R. Ferrari (Roberto); D.G. Hernandez (Dena); M.A. Nalls (Michael); J.D. Rohrer (Jonathan Daniel); A. Ramasamy (Adaikalavan); J.B.J. Kwok (John); C. Dobson-Stone (Carol); Brooks Brooks William S. (W.S.); C.J. Schofield (Christopher); G.M. Halliday (Glenda Margaret); J. Hodges (John); O. Piguet (Olivier); L. Bartley (Lauren); E. Thompson (Elizabeth); E. Haan (Eric); I. Hernández (Isabel); A. Ruiz (Agustin); M. Boada (Mercè); B. Borroni (Barbara); A. Padovani (Alessandro); C. Cruchaga (Carlos); N.J. Cairns (Nigel); L. Benussi (Luisa); G. Binetti (Giuliano); R. Ghidoni (Roberta); G. Forloni (Gianluigi); D. Galimberti (Daniela); C. Fenoglio (Chiara); M. Serpente (Maria); E. Scarpini (Elio); J. Clarimon (Jordi); A. Lleo (Alberto); R. Blesa (Rafael); M.L. Waldö (Maria Landqvist); K. Nilsson (Karin); C. Nilsson (Christer); I.R.A. Mackenzie (Ian); G.Y.R. Hsiung (Ging-Yuek); D.M.A. Mann (David); J. Grafman (Jordan); C.M. Morris (Chris); J. Attems (Johannes); T.D. Griffiths (Timothy); I.G. McKeith (Ian); A.W. Thomas (Alan); P. Pietrini (P.); E.D. Huey (Edward); E.M. Wassermann (Eric); A. Baborie (Atik); J.A.J. Jaros (Julian); M.C. Tierney (Michael); P. Pastor (Pau); C. Razquin (Cristina); S. Ortega-Cubero (Sara); E. Alonso (Elena); R. Perneczky (Robert); J. Diehl-Schmid (Janine); E.C. Alexopoulos (Evangelos); A. Kurz; I. Rainero (Innocenzo); M. Rubino (Maurizio); L. Pinessi (Lorenzo); E. Rogaeva (Ekaterina); P.H. St George-Hyslop (Peter); G. de Rossi (Giulio); F. Tagliavini (Fabrizio); G. Giaccone (Giuseppe); J.B. Rowe (James); J.C.M. Schlachetzki (Johannes C.); J. Uphill (James); J. Collinge (John); S. Mead (Simon); A. Danek (Adrian); V.M. Deerlin (Vivianna); M. Grossman (Murray); J.Q. Trojanowski (John); J. van der Zee (Jill); J. Deschamps (Jacqueline); T. van Langenhove (Tim); M. Cruts (Marc); C. van Broeckhoven (Christine); S.F. Cappa (Stefano); I. Le Ber (Isabelle); D. Hannequin (Didier); V. Golfier (Véronique); M. Vercelletto (Martine); A. Brice; B. Nacmias (Benedetta); S. Sorbi (Sandro); S. Bagnoli (Silvia); I. Piaceri (Irene); J.E. Nielsen (Jorgen); L.E. Hjermind (Lena); M. Riemenschneider (Matthias); M. Mayhaus (Manuel); B. Ibach (Bernd); G. Gasparoni (Gilles); I. Pichler (Irene); W. Gu (Wei); M. Rossor (Martin); N.C. Fox (Nick); J.D. Warren (Jason); M.G. Spillantini; H. Morris (Huw); P. Rizzu (Patrizia); P. Heutink (Peter); J. Snowden (Julie); S. Rollinson (Sara); A. Richardson (Anna); A. Gerhard (Alex); A.C. Bruni (Amalia); R. Maletta (Raffaele); F. Frangipane (Francesca); C. Cupidi (Chiara); L. Bernardi (Livia); M. Anfossi (Maria); V. Gallo (Valentina); A. Conidi (Andrea); N. Smirne (Nicoletta); S. Rademakers (Suzanne); M.C. Baker (Matthew); D.W. Dickson (Dennis); N.R. Graff-Radford (Neill); R.C. Petersen (Ronald); D.S. Knopman (David); K.A. Josephs (Keith); B.F. Boeve (Bradley); J.E. Parisi (Joseph); W. Seeley (William); B.L. Miller (Bruce Lars); A. Karydas (Anna); H. Rosen (Howard); J.C. van Swieten (John); E.G.P. Dopper (Elise); H. Seelaar (Harro); Y. Pijnenburg (Yolande); P. Scheltens (Philip); G. Logroscino (Giancarlo); R. Capozzo (Rosa); V. Novelli (Valeria); A.A. Puca (Annibale); C. Franceschi (Claudio); A. Postiglione (Alfredo); D.J. Milan (David); D. Sorrentino (Dario); M. Kristiansen (Mark); Y.T. Chiang; M.J. Graff (Maud J.L.); F. Pasquier (Florence); P.E. Rollin (Pierre); V. Deramecourt (Vincent); F. Lebert (Florence); D. Kapogiannis (Dimitrios); L. Ferrucci (Luigi); S. Pickering-Brown (Stuart); A. Singleton (Andrew); J. Hardy (John); M. Momeni (Mona)

    2014-01-01

    textabstractBackground: Frontotemporal dementia (FTD) is a complex disorder characterised by a broad range of clinical manifestations, differential pathological signatures, and genetic variability. Mutations in three genes-MAPT, GRN, and C9orf72-have been associated with FTD. We sought to identify

  12. Medical and environmental risk factors for sporadic frontotemporal dementia: a retrospective case-control study

    NARCIS (Netherlands)

    S.M. Rosso (Sonia); E.J. Landweer; M. Houterman; C.M. van Duijn (Cornelia); J.C. van Swieten (John); L. Donker Kaat (Laura)

    2003-01-01

    textabstractA retrospective case-control study was carried out on 80 patients with sporadic frontotemporal dementia and 124 age, sex, and surrogate informant matched controls with respect to various medical and environmental risk factors. Head trauma was associated with an odds ratio of 3.3 (95%

  13. Motor Speech Phenotypes of Frontotemporal Dementia, Primary Progressive Aphasia, and Progressive Apraxia of Speech

    Science.gov (United States)

    Poole, Matthew L.; Brodtmann, Amy; Darby, David; Vogel, Adam P.

    2017-01-01

    Purpose: Our purpose was to create a comprehensive review of speech impairment in frontotemporal dementia (FTD), primary progressive aphasia (PPA), and progressive apraxia of speech in order to identify the most effective measures for diagnosis and monitoring, and to elucidate associations between speech and neuroimaging. Method: Speech and…

  14. Disruption of endocytic trafficking in frontotemporal dementia with CHMP2B mutations

    DEFF Research Database (Denmark)

    Urwin, Hazel; Authier, Astrid; Nielsen, Jørgen Erik

    2010-01-01

    Mutations in CHMP2B cause frontotemporal dementia (FTD) in a large Danish pedigree, which is termed FTD linked to chromosome 3 (FTD-3), and also in an unrelated familial FTD patient. CHMP2B is a component of the ESCRT-III complex, which is required for function of the multivesicular body (MVB), a...

  15. A case series of PLS patients with frontotemporal dementia and overview of the literature

    NARCIS (Netherlands)

    de Vries, Bálint S.; Rustemeijer, Laura M.M.; van der Kooi, Anneke J.; Raaphorst, Joost; Schröder, Carin D.; Nijboer, Tanja C.W.|info:eu-repo/dai/nl/304832421; Hendrikse, Jeroen; Veldink, Jan H.; van den Berg, Leonard H.; van Es, M.A.

    2017-01-01

    Objective: Primary lateral sclerosis (PLS) is a rare form of motor neuron disease characterised by UMN degeneration leading to slowly progressive spasticity. Whether it is a separate disease or a subtype of ALS has been debated. In ALS comorbid frontotemporal dementia (FTD) is frequently seen

  16. A case series of PLS patients with frontotemporal dementia and overview of the literature

    NARCIS (Netherlands)

    de Vries, Bálint S|info:eu-repo/dai/nl/413986209; Rustemeijer, Laura M M; van der Kooi, Anneke J.; Raaphorst, Joost; Schröder, Carin D|info:eu-repo/dai/nl/413971449; Nijboer, Tanja C W|info:eu-repo/dai/nl/304832421; Hendrikse, Jeroen|info:eu-repo/dai/nl/266590268; Veldink, Jan H|info:eu-repo/dai/nl/266575722; van den Berg, Leonard H|info:eu-repo/dai/nl/288255216; van Es, Michael A

    2017-01-01

    OBJECTIVE: Primary lateral sclerosis (PLS) is a rare form of motor neuron disease characterised by UMN degeneration leading to slowly progressive spasticity. Whether it is a separate disease or a subtype of ALS has been debated. In ALS comorbid frontotemporal dementia (FTD) is frequently seen

  17. A case series of PLS patients with frontotemporal dementia and overview of the literature

    NARCIS (Netherlands)

    de Vries, Bálint S.; Rustemeijer, Laura M. M.; van der Kooi, Anneke J.; Raaphorst, Joost; Schröder, Carin D.; Nijboer, Tanja C. W.; Hendrikse, Jeroen; Veldink, Jan H.; van den Berg, Leonard H.; van Es, Michael A.

    2017-01-01

    Primary lateral sclerosis (PLS) is a rare form of motor neuron disease characterised by UMN degeneration leading to slowly progressive spasticity. Whether it is a separate disease or a subtype of ALS has been debated. In ALS comorbid frontotemporal dementia (FTD) is frequently seen (±15%). However,

  18. Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP43 inclusions

    NARCIS (Netherlands)

    Van Deerlin, V.M.; Sleiman, P.M.A.; Martinez-Lage, M.; Chen-Plotkin, A.; Wang, L.S.; Graff-Radford, N.R.; Dickson, D.W.; Rademakers, R.; Boeve, B.F.; Grossman, M.; Arnold, S.E.; Mann, D.M.A.; Pickering-Brown, S.M.; Seelaar, H.; Heutink, P.; van Swieten, J.C.; Murrell, J.R.; Ghetti, B.; Spina, S.; Grafman, J.; Hodges, J.; Spillantini, M.G.; Gilman, S.; Lieberman, A.P.; Kaye, J.A.; Woltjer, R.L.; Bigio, E.H.; Mesulam, M.; Al-Sarraj, S.; Troakes, C.; Rosenberg, R.N.; White, C.L.; Ferrer, I.; Llado, A.; Neumann, M.; Kretzschmar, H.A.; Hulette, C.M.; Welsh-Bohmer, K.A.; Miller, B.L.; Alzualde, A.; de Munain, A.L.; Mckee, A.C.; Gearing, M.; Levey, A.I.; Lah, J.J.; Hardy, J.; Rohrer, J.D.; Lashley, T.; Mackenzie, I.R.A.; Feldman, H.H.; Hamilton, R.L.; Dekosky, S.T.; van der Zee, J.; Kumar-Singh, S.; Van Broeckhoven, C.; Mayeux, R.; Vonsattel, J.P.G.; Troncoso, J.C.; Kril, J.J.; Kwok, J.B.J.; Halliday, G.M.; Bird, T.D.; Ince, P.G.; Shaw, P.J.; Cairns, N.J.; Morris, J.C.; McLean, C.A.; deCarli, C.; Ellis, W.G.; Freeman, S.H.; Frosch, M.P.; Growdon, J.H.; Perl, D.P.; Sano, M.; Bennett, D.A.; Schneider, J.A.; Beach, T.G.; Reiman, E.M.; Woodruff, B.K.; Cummings, J.; Vinters, H.V.; Miller, C.A.; Chui, H.C.; Alafuzoff, I.; Hartikainen, P.; Seilhean, D.; Galasko, D.; Masliah, E.; Cotman, C.W.; Tunon, M.T.; Martinez, M.C.C.; Munoz, D.G.; Carroll, S.L.; Marson, D.; Riederer, P.F.; Bogdanovic, N.; Schellenberg, G.D.; Hakonarson, H.; Trojanowski, J.Q. and others

    2010-01-01

    Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia. The predominant neuropathology is FTLD with TAR DNA-binding protein (TDP-43) inclusions (FTLD-TDP). FTLD-TDP is frequently familial, resulting from mutations in GRN (which encodes progranulin). We

  19. Anatomical correlates of reward-seeking behaviours in behavioural variant frontotemporal dementia

    Science.gov (United States)

    Sturm, Virginia E.; Seeley, William W.; Miller, Bruce L.; Kramer, Joel H.; Rosen, Howard J.

    2014-01-01

    Behavioural variant frontotemporal dementia is characterized by abnormal responses to primary reward stimuli such as food, sex and intoxicants, suggesting abnormal functioning of brain circuitry mediating reward processing. The goal of this analysis was to determine whether abnormalities in reward-seeking behaviour in behavioural variant frontotemporal dementia are correlated with atrophy in regions known to mediate reward processing. Review of case histories in 103 patients with behavioural variant frontotemporal dementia identified overeating or increased sweet food preference in 80 (78%), new or increased alcohol or drug use in 27 (26%), and hypersexuality in 17 (17%). For each patient, a primary reward-seeking score of 0–3 was created with 1 point given for each target behaviour (increased seeking of food, drugs, or sex). Voxel-based morphometry performed in 91 patients with available imaging revealed that right ventral putamen and pallidum atrophy correlated with higher reward-seeking scores. Each of the reward-related behaviours involved partially overlapping right hemisphere reward circuit regions including putamen, globus pallidus, insula and thalamus. These findings indicate that in some patients with behavioural variant frontotemporal dementia, low volume of subcortical reward-related structures is associated with increased pursuit of primary rewards, which may be a product of increased thalamocortical feedback. PMID:24740987

  20. Reference cluster normalization improves detection of frontotemporal lobar degeneration by means of FDG-PET.

    Directory of Open Access Journals (Sweden)

    Juergen Dukart

    Full Text Available Positron emission tomography with [18F] fluorodeoxyglucose (FDG-PET plays a well-established role in assisting early detection of frontotemporal lobar degeneration (FTLD. Here, we examined the impact of intensity normalization to different reference areas on accuracy of FDG-PET to discriminate between patients with mild FTLD and healthy elderly subjects. FDG-PET was conducted at two centers using different acquisition protocols: 41 FTLD patients and 42 controls were studied at center 1, 11 FTLD patients and 13 controls were studied at center 2. All PET images were intensity normalized to the cerebellum, primary sensorimotor cortex (SMC, cerebral global mean (CGM, and a reference cluster with most preserved FDG uptake in the aforementioned patients group of center 1. Metabolic deficits in the patient group at center 1 appeared 1.5, 3.6, and 4.6 times greater in spatial extent, when tracer uptake was normalized to the reference cluster rather than to the cerebellum, SMC, and CGM, respectively. Logistic regression analyses based on normalized values from FTLD-typical regions showed that at center 1, cerebellar, SMC, CGM, and cluster normalizations differentiated patients from controls with accuracies of 86%, 76%, 75% and 90%, respectively. A similar order of effects was found at center 2. Cluster normalization leads to a significant increase of statistical power in detecting early FTLD-associated metabolic deficits. The established FTLD-specific cluster can be used to improve detection of FTLD on a single case basis at independent centers - a decisive step towards early diagnosis and prediction of FTLD syndromes enabling specific therapies in the future.

  1. Aphasia following left thalamic hemorrhage

    International Nuclear Information System (INIS)

    Makishita, Hideo; Miyasaka, Motomaro; Tanizaki, Yoshio; Yanagisawa, Nobuo; Sugishita, Morihiro.

    1984-01-01

    We reported 7 patients with left thalamic hemorrhage in the chronic stage (from 1.5 months to 4.5 months), and described language disorders examined by Western Aphasia Battery (WAB) and measured cerebral blood flow by single photon emission CT. Examination of language by WAB revealed 4 aphasics out of 7 cases, and 3 patients had no language deficit. The patient with Wernicke's aphasia showed low density area only in the left posterior thalamus in X-ray CT, and revealed severe low blood flow area extending to left temporal lobe in emission CT. In the case with transcortical sensory aphasia, although X-ray CT showed no obvious low density area, emission CT revealed moderate low flow area in watershed area that involved the territory between posterior cerebral and middle cerebral arteries in the left temporooccipital region in addition to low blood flow at the left thalamus. In one of the two patients classified as anomic aphasia, whose score of repetition (8.4) was higher than that of comprehension (7.4), emission CT showed slight low flow area at the temporo-occipital region similarly as the case with transcortical sensory aphasia. In another case with anomic aphasia, scored 9 on both fluensy and comprehension subtests and 10 on repetition, there was wide low density area all over the left thalamus and midline shift to the right in X-ray CT, and emission CT showed severe low blood flow in the same region spreading widely toward the cerebral surface. On the other hand, in all of the 3 patients without aphasia, emission CT showed low flow region restricted to the left thalamus. (J.P.N.)

  2. An impact of tectonic structures on the groundwater circulation and losses from surface accumulation in the area of the left bank of Lazići dam on the Tara MTS

    Directory of Open Access Journals (Sweden)

    Jemcov Igor R.

    2017-01-01

    Full Text Available The process of water leakage determination in the area of water accumulation and left bank of the Lazići dam requires extensive application of different methods: very detailed monitoring data, tracing, structural and tectonic investigations, etc. Obtained results of water losses from the reservoir indicate a tendency for further increases. According to the applied analyses, two zones at the narrow zone of the grout curtain, characterized by different hydraulic mechanisms were detected. Additionally, the general direction of water leakage from the reservoir was detected. An important conclusion is that in such hydraulically intense conditions leakage is unstoppable, but can be partially controlled or slowed down.

  3. Brain Serotonergic and Noradrenergic Deficiencies in Behavioral Variant Frontotemporal Dementia Compared to Early-Onset Alzheimer's Disease

    NARCIS (Netherlands)

    Vermeiren, Yannick; Janssens, Jana; Aerts, Tony; Martin, Jean-Jacques; Sieben, Anne; Van Dam, Debby; De Deyn, Peter P.

    2016-01-01

    Routinely prescribed psychoactive drugs in behavioral variant frontotemporal dementia (FTD) for improvement of (non) cognitive symptoms are primarily based on monoamine replacement or augmentation strategies. These were, however, initially intended to symptomatically treat other degenerative,

  4. Striatal Atrophy in the Behavioural Variant of Frontotemporal Dementia: Correlation with Diagnosis, Negative Symptoms and Disease Severity.

    Directory of Open Access Journals (Sweden)

    Matthew D Macfarlane

    Full Text Available Behavioural variant frontotemporal dementia (bvFTD is associated with changes in dorsal striatal parts of the basal ganglia (caudate nucleus and putamen, related to dysfunction in the cortico-striato-thalamic circuits which help mediate executive and motor functions. We aimed to determine whether the size and shape of striatal structures correlated with diagnosis of bvFTD, and measures of clinical severity, behaviour and cognition.Magnetic resonance imaging scans from 28 patients with bvFTD and 26 healthy controls were manually traced using image analysis software (ITK-SNAP. The resulting 3-D objects underwent volumetric analysis and shape analysis, through spherical harmonic description with point distribution models (SPHARM-PDM. Correlations with size and shape were sought with clinical measures in the bvTFD group, including Frontal Behavioural Inventory, Clinical Dementia Rating for bvFTD, Color Word Interference, Hayling part B and Brixton tests, and Trail-Making Test.Caudate nuclei and putamina were significantly smaller in the bvFTD group compared to controls (left caudate 16% smaller, partial eta squared 0.173, p=0.003; right caudate 11% smaller, partial eta squared 0.103, p=0.023; left putamen 18% smaller, partial eta squared 0.179, p=0.002; right putamen 12% smaller, partial eta squared 0.081, p=0.045, with global shape deflation in the caudate bilaterally but no localised shape change in putamen. In the bvFTD group, shape deflations on the left, corresponding to afferent connections from dorsolateral prefrontal mediofrontal/anterior cingulate and orbitofrontal cortex, correlated with worsening disease severity. Global shape deflation in the putamen correlated with Frontal Behavioural Inventory scores-higher scoring on negative symptoms was associated with the left putamen, while positive symptoms were associated with the right. Other cognitive tests had poor completion rates.Behavioural symptoms and severity of bvFTD are correlated

  5. Late onset bipolar disorder and frontotemporal dementia with mutation in progranulin gene: a case report.

    Science.gov (United States)

    Rubino, Elisa; Vacca, Alessandro; Gallone, Salvatore; Govone, Flora; Zucca, Milena; Gai, Annalisa; Ferrero, Patrizia; Fenoglio, Pierpaola; Giordana, Maria Teresa; Rainero, Innocenzo

    2017-11-01

    Bipolar disorder is a chronic psychiatric illness characterised by fluctuation in mood state, with a relapsing and remitting course. Frontotemporal dementia (FTD) is a clinically and genetically heterogeneous syndrome, with the most frequent phenotype being behavioural variant frontotemporal dementia (bvFTD). Here, we report the case of an Italian male presenting with late-onset bipolar disorder that developed into bvFTD over time, carrying a mutation in the GRN gene. Interestingly, the patient carried the c.1639 C > T variant in the GRN gene, resulting in a R547C substitution. Our case report further corroborates the notion that, in addition to FTD, progranulin may be involved in the neurobiology of bipolar disorder type 1, and suggests to screen patients with late-onset bipolar disorder for GRN mutations.

  6. Reversal of pathology in CHMP2B-mediated frontotemporal dementia patient cells using RNA interference

    DEFF Research Database (Denmark)

    Nielsen, Troels Tolstrup; Mizielinska, Sarah; Hasholt, Lis

    2012-01-01

    role in the pathogenesis of the disease. METHODS: In the present study, we used lentiviral vectors to efficiently knockdown CHMP2B by delivering microRNA embedded small hairpin RNAs. RESULTS: We show that CHMP2B can be efficiently knocked down in patient fibroblasts using an RNA interference approach......BACKGROUND: Frontotemporal dementia is the second most common form of young-onset dementia after Alzheimer's disease, and several genetic forms of frontotemporal dementia are known. A rare genetic variant is caused by a point mutation in the CHMP2B gene. CHMP2B is a component of the ESCRT......-III complex, which is involved in endosomal trafficking of proteins targeted for degradation in lysosomes. Mutations in CHMP2B result in abnormal endosomal structures in patient fibroblasts and patient brains, probably through a gain-of-function mechanism, suggesting that the endosomal pathway plays a central...

  7. Predicting behavioral variant frontotemporal dementia with pattern classification in multi-center structural MRI data.

    Science.gov (United States)

    Meyer, Sebastian; Mueller, Karsten; Stuke, Katharina; Bisenius, Sandrine; Diehl-Schmid, Janine; Jessen, Frank; Kassubek, Jan; Kornhuber, Johannes; Ludolph, Albert C; Prudlo, Johannes; Schneider, Anja; Schuemberg, Katharina; Yakushev, Igor; Otto, Markus; Schroeter, Matthias L

    2017-01-01

    Frontotemporal lobar degeneration (FTLD) is a common cause of early onset dementia. Behavioral variant frontotemporal dementia (bvFTD), its most common subtype, is characterized by deep alterations in behavior and personality. In 2011, new diagnostic criteria were suggested that incorporate imaging criteria into diagnostic algorithms. The study aimed at validating the potential of imaging criteria to individually predict diagnosis with machine learning algorithms. Brain atrophy was measured with structural magnetic resonance imaging (MRI) at 3 Tesla in a multi-centric cohort of 52 bvFTD patients and 52 healthy control subjects from the German FTLD Consortium's Study. Beside group comparisons, diagnosis bvFTD vs. controls was individually predicted in each subject with support vector machine classification in MRI data across the whole brain or in frontotemporal, insular regions, and basal ganglia known to be mainly affected based on recent meta-analyses. Multi-center effects were controlled for with a new method, "leave one center out" conjunction analyses, i.e. repeatedly excluding subjects from each center from the analysis. Group comparisons revealed atrophy in, most consistently, the frontal lobe in bvFTD beside alterations in the insula, basal ganglia and temporal lobe. Most remarkably, support vector machine classification enabled predicting diagnosis in single patients with a high accuracy of up to 84.6%, where accuracy was highest in a region-of-interest approach focusing on frontotemporal, insular regions, and basal ganglia in comparison with the whole brain approach. Our study demonstrates that MRI, a widespread imaging technology, can individually identify bvFTD with high accuracy in multi-center imaging data, paving the road to personalized diagnostic approaches in the future.

  8. Semantic memory: Nouns and action verbs in cognitively unimpaired individuals and frontotemporal lobar degeneration

    Directory of Open Access Journals (Sweden)

    Leticia Lessa Mansur

    Full Text Available ABSTRACT Among the instruments to evaluate semantic memory, the Pyramids and Palm Trees (PPT and the Kissing and Dancing (KDT tests are widely used but none have a performance referential for cognitively normal and impaired Brazilian populations. Objective: [A] To study these two tests in a sample of young healthy Brazilian individuals living in São Paulo; [B] To apply the results to the evaluation of two cases diagnosed with frontotemporal lobar degeneration. Methods: We evaluated 50 normal participants (41 females and 9 males aged between 20-63 years, with schooling level of 14-20 years. In addition, two individuals diagnosed with frontotemporal lobar degeneration were examined, one with behavioral-variant frontotemporal dementia and the other with semantic dementia. Results: On the two tests, no effects of age, gender and schooling on the performance of normal individuals were observed. According to the performance of the sample of controls, scores below 46 points on the PPT and below 47 on the KDT are suggestive of deficits in semantic memory. The analyses of both cases indicated double dissociation in establishing associations between nouns and action verbs. Although the two patients had low scores on both tests, the patient with behavioral-variant frontotemporal dementia performed better on the PPT compared to the KDT, while the patient with semantic dementia showed the reverse, performing better on the KDT. Conclusion: The PPT and KDT are suitable tests for use in the Brazilian population, with minimal need for adjustments. They are applicable tools both for cognitive assessment and research in semantic memory. In the present study, we obtained representative values of performance for cognitively unimpaired individuals and demonstrated the utility of these instruments for cognitive assessment of patients with FTLD.

  9. Semantic memory: nouns and action verbs in cognitively unimpaired individuals and frontotemporal lobar degeneration.

    Science.gov (United States)

    Mansur, Leticia Lessa; Carthery-Goulart, Maria Teresa; Bahia, Valéria Santoro; Bak, Thomas H; Nitrini, Ricardo

    2013-01-01

    Among the instruments to evaluate semantic memory, the Pyramids and Palm Trees (PPT) and the Kissing and Dancing (KDT) tests are widely used but none have a performance referential for cognitively normal and impaired Brazilian populations. [A] To study these two tests in a sample of young healthy Brazilian individuals living in São Paulo; [B] To apply the results to the evaluation of two cases diagnosed with frontotemporal lobar degeneration. We evaluated 50 normal participants (41 females and 9 males) aged between 20-63 years, with schooling level of 14-20 years. In addition, two individuals diagnosed with frontotemporal lobar degeneration were examined, one with behavioral-variant frontotemporal dementia and the other with semantic dementia. On the two tests, no effects of age, gender and schooling on the performance of normal individuals were observed. According to the performance of the sample of controls, scores below 46 points on the PPT and below 47 on the KDT are suggestive of deficits in semantic memory. The analyses of both cases indicated double dissociation in establishing associations between nouns and action verbs. Although the two patients had low scores on both tests, the patient with behavioral-variant frontotemporal dementia performed better on the PPT compared to the KDT, while the patient with semantic dementia showed the reverse, performing better on the KDT. The PPT and KDT are suitable tests for use in the Brazilian population, with minimal need for adjustments. They are applicable tools both for cognitive assessment and research in semantic memory. In the present study, we obtained representative values of performance for cognitively unimpaired individuals and demonstrated the utility of these instruments for cognitive assessment of patients with FTLD.

  10. Factors Underpinning Caregiver Burden in Frontotemporal Dementia Differ in Spouses and their Children

    Science.gov (United States)

    Kaizik, Cassandra; Caga, Jashelle; Camino, Julieta; O’Connor, Claire M.; McKinnon, Colleen; Oyebode, Jan R.; Piguet, Olivier; Hodges, John R.; Mioshi, Eneida

    2017-01-01

    The objectives of this observational study were to (1) compare spousal and child caregiver burden; (2) compare co-resident and live-out child caregiver burden; and (3) investigate factors influencing spousal and child caregiver burden. Data was collected from 90 caregivers of people with frontotemporal degeneration (FTD) recruited from the Frontotemporal Dementia Research Group (Frontier) at Neuroscience Research, Australia. Of this caregiver group, 43 were spousal caregivers and 47 were child caregivers. Caregiver burden and emotional state were evaluated using the short Zarit Burden Interview and the short version of the Depression, Anxiety and Stress Scale-21. The Social Network Index was applied to ascertain the social network of the caregiver, while the Intimate Bond Measure was used to evaluate the current quality of the relationship between the caregiver and the person with dementia. The Frontotemporal Dementia Rating Scale was used to assess severity of dementia. Spousal and child caregivers experienced similar levels of burden, depression, anxiety, and stress, regardless of disease severity. Co-resident child caregivers had smaller social networks and greater burden than live-out caregivers. Dementia severity was key in spousal caregiver burden, whereas caregiver depression was most important in child caregiver burden. Child and spousal caregivers of individuals with FTD share similar levels of burden, influenced by different factors. Future interventions need to account for these differences. PMID:28106550

  11. Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria.

    Science.gov (United States)

    Strong, Michael J; Abrahams, Sharon; Goldstein, Laura H; Woolley, Susan; Mclaughlin, Paula; Snowden, Julie; Mioshi, Eneida; Roberts-South, Angie; Benatar, Michael; HortobáGyi, Tibor; Rosenfeld, Jeffrey; Silani, Vincenzo; Ince, Paul G; Turner, Martin R

    2017-05-01

    This article presents the revised consensus criteria for the diagnosis of frontotemporal dysfunction in amyotrophic lateral sclerosis (ALS) based on an international research workshop on frontotemporal dementia (FTD) and ALS held in London, Canada in June 2015. Since the publication of the Strong criteria, there have been considerable advances in the understanding of the neuropsychological profile of patients with ALS. Not only is the breadth and depth of neuropsychological findings broader than previously recognised - - including deficits in social cognition and language - but mixed deficits may also occur. Evidence now shows that the neuropsychological deficits in ALS are extremely heterogeneous, affecting over 50% of persons with ALS. When present, these deficits significantly and adversely impact patient survival. It is the recognition of this clinical heterogeneity in association with neuroimaging, genetic and neuropathological advances that has led to the current re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum. These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD).

  12. Manic behavior and asymmetric right frontotemporal dementia from a novel progranulin mutation

    Directory of Open Access Journals (Sweden)

    Mendez MF

    2018-02-01

    Full Text Available Mario F Mendez1–3 1Department of Neurology, 2Department of Psychiatry & Biobehavioral Sciences, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA, USA; 3Neurology Service, VA Greater Los Angeles Healthcare System, Los Angeles, CA, USA Abstract: Studies suggest a relationship of manic behavior and bipolar disorder (BD with behavioral variant frontotemporal dementia (bvFTD. The nature of this relationship is unclear. This report presents a patient with initial manic behavior as the main manifestation of familial bvFTD from a novel progranulin (GRN mutation. In contrast, there are other reports of a long background of BD preceding a diagnosis of bvFTD. A review of the literature and this patient suggest that manic symptoms result from damage to right frontotemporal neural structures from longstanding BD, as well as from bvFTD and other focal neurological disorders. In addition, there is a subgroup of patients with a probable genetic predisposition to both BD and bvFTD. Keywords: frontotemporal dementia, mania, bipolar disorder, progranulin mutation

  13. Shared neural correlates of limb apraxia in early stages of Alzheimer's dementia and behavioural variant frontotemporal dementia.

    Science.gov (United States)

    Johnen, Andreas; Brandstetter, Lisa; Kärgel, Christian; Wiendl, Heinz; Lohmann, Hubertus; Duning, Thomas

    2016-11-01

    Limb apraxia denotes a cognitive impairment of gesture production. Lesion studies in patients with stroke point towards distinct neural processing streams for limb imitation and object-pantomime within left parietal and temporal cortex, respectively. Despite its frequent occurrence as an early symptom in both, Alzheimer's dementia (AD) and behavioural variant frontotemporal dementia (bvFTD), neural correlates of limb apraxia within these patient groups remain unexplored. Using voxel-based morphometry and multiple regression models, associations between limb apraxia and gray matter (GM) volume were investigated in 36 dementia patients (18 AD, 18 bvFTD) in early disease stages. Both dementia subtypes showed a comparable degree of limb apraxia. Although the patient groups showed distinct atrophy patterns with significantly more severe frontal GM loss in bvFTD, we found similar neural correlates of limb apraxia within posterior brain regions for both dementia subtypes: limb-imitation was associated with bilateral atrophy of superior, inferior and medial parietal cortex. Object-pantomime showed associations with GM volume in right middle temporal and angular gyrus. Our results argue for shared neural correlates of limb apraxia in AD and bvFTD and validate the syndrome as an important neuropsychological feature across different etiologies. Moreover, our results are compatible with neural models derived from patients with stroke, suggesting partly distinct neural representations of imitation and pantomime. Compared to patients with stroke however, AD and bvFTD showed more bilateral or even right lateralized neural representations of limb apraxia, proposing a greater influence of visuospatial impairments and spatial body representation deficits on praxis performance. Copyright © 2016 Elsevier Ltd. All rights reserved.

  14. Functional dissociation between anterior temporal lobe and inferior frontal gyrus in the processing of dynamic body expressions: Insights from behavioral variant frontotemporal dementia.

    Science.gov (United States)

    Jastorff, Jan; De Winter, Francois-Laurent; Van den Stock, Jan; Vandenberghe, Rik; Giese, Martin A; Vandenbulcke, Mathieu

    2016-12-01

    Several brain regions are involved in the processing of emotional stimuli, however, the contribution of specific regions to emotion perception is still under debate. To investigate this issue, we combined behavioral testing, structural and resting state imaging in patients diagnosed with behavioral variant frontotemporal dementia (bvFTD) and age matched controls, with task-based functional imaging in young, healthy volunteers. As expected, bvFTD patients were impaired in emotion detection as well as emotion categorization tasks, testing dynamic emotional body expressions as stimuli. Interestingly, their performance in the two tasks correlated with gray matter volume in two distinct brain regions, the left anterior temporal lobe for emotion detection and the left inferior frontal gyrus (IFG) for emotion categorization. Confirming this observation, multivoxel pattern analysis in healthy volunteers demonstrated that both ROIs contained information for emotion detection, but that emotion categorization was only possible from the pattern in the IFG. Furthermore, functional connectivity analysis showed reduced connectivity between the two regions in bvFTD patients. Our results illustrate that the mentalizing network and the action observation network perform distinct tasks during emotion processing. In bvFTD, communication between the networks is reduced, indicating one possible cause underlying the behavioral symptoms. Hum Brain Mapp 37:4472-4486, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  15. Frontotemporal correlates of impulsivity and machine learning in retired professional athletes with a history of multiple concussions.

    Science.gov (United States)

    Goswami, R; Dufort, P; Tartaglia, M C; Green, R E; Crawley, A; Tator, C H; Wennberg, R; Mikulis, D J; Keightley, M; Davis, Karen D

    2016-05-01

    The frontotemporal cortical network is associated with behaviours such as impulsivity and aggression. The health of the uncinate fasciculus (UF) that connects the orbitofrontal cortex (OFC) with the anterior temporal lobe (ATL) may be a crucial determinant of behavioural regulation. Behavioural changes can emerge after repeated concussion and thus we used MRI to examine the UF and connected gray matter as it relates to impulsivity and aggression in retired professional football players who had sustained multiple concussions. Behaviourally, athletes had faster reaction times and an increased error rate on a go/no-go task, and increased aggression and mania compared to controls. MRI revealed that the athletes had (1) cortical thinning of the ATL, (2) negative correlations of OFC thickness with aggression and task errors, indicative of impulsivity, (3) negative correlations of UF axial diffusivity with error rates and aggression, and (4) elevated resting-state functional connectivity between the ATL and OFC. Using machine learning, we found that UF diffusion imaging differentiates athletes from healthy controls with significant classifiers based on UF mean and radial diffusivity showing 79-84 % sensitivity and specificity, and 0.8 areas under the ROC curves. The spatial pattern of classifier weights revealed hot spots at the orbitofrontal and temporal ends of the UF. These data implicate the UF system in the pathological outcomes of repeated concussion as they relate to impulsive behaviour. Furthermore, a support vector machine has potential utility in the general assessment and diagnosis of brain abnormalities following concussion.

  16. Multiparametric computer-aided differential diagnosis of Alzheimer's disease and frontotemporal dementia using structural and advanced MRI

    Energy Technology Data Exchange (ETDEWEB)

    Bron, Esther E.; Klein, Stefan [Erasmus MC, Biomedical Imaging Group Rotterdam, Departments of Medical Informatics and Radiology and Nuclear Medicine, Office Na2502, P.O. Box 2040, Rotterdam (Netherlands); Smits, Marion; Steketee, Rebecca M.E.; Meijboom, Rozanna [Erasmus MC, Department of Radiology and Nuclear Medicine, Rotterdam (Netherlands); Papma, Janne M.; Swieten, John C. van [Erasmus MC, Department of Neurology, Rotterdam (Netherlands); Groot, Marius de [Erasmus MC, Biomedical Imaging Group Rotterdam, Departments of Medical Informatics and Radiology and Nuclear Medicine, Office Na2502, P.O. Box 2040, Rotterdam (Netherlands); Erasmus MC, Department of Epidemiology, Rotterdam (Netherlands); Niessen, Wiro J. [Erasmus MC, Biomedical Imaging Group Rotterdam, Departments of Medical Informatics and Radiology and Nuclear Medicine, Office Na2502, P.O. Box 2040, Rotterdam (Netherlands); Delft University of Technology, Imaging Physics, Applied Sciences, Delft (Netherlands)

    2017-08-15

    To investigate the added diagnostic value of arterial spin labelling (ASL) and diffusion tensor imaging (DTI) to structural MRI for computer-aided classification of Alzheimer's disease (AD), frontotemporal dementia (FTD), and controls. This retrospective study used MRI data from 24 early-onset AD and 33 early-onset FTD patients and 34 controls (CN). Classification was based on voxel-wise feature maps derived from structural MRI, ASL, and DTI. Support vector machines (SVMs) were trained to classify AD versus CN (AD-CN), FTD-CN, AD-FTD, and AD-FTD-CN (multi-class). Classification performance was assessed by the area under the receiver-operating-characteristic curve (AUC) and accuracy. Using SVM significance maps, we analysed contributions of brain regions. Combining ASL and DTI with structural MRI resulted in higher classification performance for differential diagnosis of AD and FTD (AUC = 84%; p = 0.05) than using structural MRI by itself (AUC = 72%). The performance of ASL and DTI themselves did not improve over structural MRI. The classifications were driven by different brain regions for ASL and DTI than for structural MRI, suggesting complementary information. ASL and DTI are promising additions to structural MRI for classification of early-onset AD, early-onset FTD, and controls, and may improve the computer-aided differential diagnosis on a single-subject level. (orig.)

  17. Effects of 17-allylamino-17-demethoxygeldanamycin (17-AAG) in transgenic mouse models of frontotemporal lobar degeneration and Alzheimer's disease.

    Science.gov (United States)

    Ho, Shuk Wai; Tsui, Yuk Tung Chanel; Wong, Ting Ting; Cheung, Stanley Kwok-Kuen; Goggins, William B; Yi, Lau Ming; Cheng, Kwok Kin; Baum, Larry

    2013-12-17

    Alzheimer's disease (AD), the most common dementia, is characterized by potentially neurotoxic aggregation of Aβ peptide and tau protein, and their deposition as amyloid plaques and neurofibrillary tangles (NFTs). Tau aggregation also occurs in other common neurodegenerative diseases. Frontotemporal dementia (FTD) can be caused by tau mutations that increase the susceptibility of tau to hyperphosphorylation and aggregation, which may cause neuronal dysfunction and deposition of NFTs. 17-allylamino-17-demethoxygeldanamycin (17-AAG) is a potent inhibitor of heat shock protein 90 (Hsp90), a cytosolic chaperone implicated in the proper folding and functions of a repertoire of client proteins. 17-AAG binds to Hsp90 and enhances degradation of Hsp90 client protein. We sought to determine whether 17-AAG can reduce Aβ and tau pathology in the brains of AD and FTD model mice expressing Aβ or P301L mutant tau, respectively. Mice were randomized to receive 25, 5, or 0 mg/kg 17-AAG thrice weekly from age eight to 11 months. Analysis was performed by rotarod test on motor function, on the area occupied by plaques in hippocampus or NFTs in medulla tissue sections, and on mortality. A high dose of 17-AAG tended to decrease NFTs in male mice (p = 0.08). Further studies are required to confirm the effect of 17-AAG in diseases of tau aggregation.

  18. Perceived Conventionality in Co-speech Gestures Involves the Fronto-Temporal Language Network

    Directory of Open Access Journals (Sweden)

    Dhana Wolf

    2017-11-01

    Full Text Available Face-to-face communication is multimodal; it encompasses spoken words, facial expressions, gaze, and co-speech gestures. In contrast to linguistic symbols (e.g., spoken words or signs in sign language relying on mostly explicit conventions, gestures vary in their degree of conventionality. Bodily signs may have a general accepted or conventionalized meaning (e.g., a head shake or less so (e.g., self-grooming. We hypothesized that subjective perception of conventionality in co-speech gestures relies on the classical language network, i.e., the left hemispheric inferior frontal gyrus (IFG, Broca's area and the posterior superior temporal gyrus (pSTG, Wernicke's area and studied 36 subjects watching video-recorded story retellings during a behavioral and an functional magnetic resonance imaging (fMRI experiment. It is well documented that neural correlates of such naturalistic videos emerge as intersubject covariance (ISC in fMRI even without involving a stimulus (model-free analysis. The subjects attended either to perceived conventionality or to a control condition (any hand movements or gesture-speech relations. Such tasks modulate ISC in contributing neural structures and thus we studied ISC changes to task demands in language networks. Indeed, the conventionality task significantly increased covariance of the button press time series and neuronal synchronization in the left IFG over the comparison with other tasks. In the left IFG, synchronous activity was observed during the conventionality task only. In contrast, the left pSTG exhibited correlated activation patterns during all conditions with an increase in the conventionality task at the trend level only. Conceivably, the left IFG can be considered a core region for the processing of perceived conventionality in co-speech gestures similar to spoken language. In general, the interpretation of conventionalized signs may rely on neural mechanisms that engage during language comprehension.

  19. Perceived Conventionality in Co-speech Gestures Involves the Fronto-Temporal Language Network

    Science.gov (United States)

    Wolf, Dhana; Rekittke, Linn-Marlen; Mittelberg, Irene; Klasen, Martin; Mathiak, Klaus

    2017-01-01

    Face-to-face communication is multimodal; it encompasses spoken words, facial expressions, gaze, and co-speech gestures. In contrast to linguistic symbols (e.g., spoken words or signs in sign language) relying on mostly explicit conventions, gestures vary in their degree of conventionality. Bodily signs may have a general accepted or conventionalized meaning (e.g., a head shake) or less so (e.g., self-grooming). We hypothesized that subjective perception of conventionality in co-speech gestures relies on the classical language network, i.e., the left hemispheric inferior frontal gyrus (IFG, Broca's area) and the posterior superior temporal gyrus (pSTG, Wernicke's area) and studied 36 subjects watching video-recorded story retellings during a behavioral and an functional magnetic resonance imaging (fMRI) experiment. It is well documented that neural correlates of such naturalistic videos emerge as intersubject covariance (ISC) in fMRI even without involving a stimulus (model-free analysis). The subjects attended either to perceived conventionality or to a control condition (any hand movements or gesture-speech relations). Such tasks modulate ISC in contributing neural structures and thus we studied ISC changes to task demands in language networks. Indeed, the conventionality task significantly increased covariance of the button press time series and neuronal synchronization in the left IFG over the comparison with other tasks. In the left IFG, synchronous activity was observed during the conventionality task only. In contrast, the left pSTG exhibited correlated activation patterns during all conditions with an increase in the conventionality task at the trend level only. Conceivably, the left IFG can be considered a core region for the processing of perceived conventionality in co-speech gestures similar to spoken language. In general, the interpretation of conventionalized signs may rely on neural mechanisms that engage during language comprehension. PMID:29249945

  20. Fronto-Temporal Connectivity Predicts ECT Outcome in Major Depression

    Directory of Open Access Journals (Sweden)

    Amber M. Leaver

    2018-03-01

    Full Text Available BackgroundElectroconvulsive therapy (ECT is arguably the most effective available treatment for severe depression. Recent studies have used MRI data to predict clinical outcome to ECT and other antidepressant therapies. One challenge facing such studies is selecting from among the many available metrics, which characterize complementary and sometimes non-overlapping aspects of brain function and connectomics. Here, we assessed the ability of aggregated, functional MRI metrics of basal brain activity and connectivity to predict antidepressant response to ECT using machine learning.MethodsA radial support vector machine was trained using arterial spin labeling (ASL and blood-oxygen-level-dependent (BOLD functional magnetic resonance imaging (fMRI metrics from n = 46 (26 female, mean age 42 depressed patients prior to ECT (majority right-unilateral stimulation. Image preprocessing was applied using standard procedures, and metrics included cerebral blood flow in ASL, and regional homogeneity, fractional amplitude of low-frequency modulations, and graph theory metrics (strength, local efficiency, and clustering in BOLD data. A 5-repeated 5-fold cross-validation procedure with nested feature-selection validated model performance. Linear regressions were applied post hoc to aid interpretation of discriminative features.ResultsThe range of balanced accuracy in models performing statistically above chance was 58–68%. Here, prediction of non-responders was slightly higher than for responders (maximum performance 74 and 64%, respectively. Several features were consistently selected across cross-validation folds, mostly within frontal and temporal regions. Among these were connectivity strength among: a fronto-parietal network [including left dorsolateral prefrontal cortex (DLPFC], motor and temporal networks (near ECT electrodes, and/or subgenual anterior cingulate cortex (sgACC.ConclusionOur data indicate that pattern classification of multimodal f

  1. Right Frontotemporal Cortex Mediates the Relationship between Cognitive Insight and Subjective Quality of Life in Patients with Schizophrenia.

    Science.gov (United States)

    Pu, Shenghong; Nakagome, Kazuyuki; Itakura, Masashi; Ohtachi, Hiroaki; Iwata, Masaaki; Nagata, Izumi; Kaneko, Koichi

    2018-01-01

    Although prior studies identified a relationship between cognitive insight and subjective quality of life (QOL) in patients with schizophrenia, the brain regions mediating this relationship remain unknown. Recent studies have shown that the ventrolateral prefrontal cortex may be particularly important for cognitive insight in individuals with schizophrenia. Here, we examined whether frontotemporal function mediates the relationship between cognitive insight and QOL in 64 participants, including 32 patients with schizophrenia and 32 healthy controls. Cognitive insight was measured using the Beck Cognitive Insight Scale (BCIS), while participants' subjective QOL was assessed using the Medical Outcomes Study 36-item Short-form Health Survey. Frontotemporal function was evaluated during a verbal fluency task using multichannel near-infrared spectroscopy. Consistent with previous findings, we found that frontotemporal function was impaired in patients with schizophrenia. Interestingly, our data also revealed that the right ventrolateral PFC and the right anterior part of the temporal cortex significantly mediated the relationship between the self-reflectiveness (SR) subscale of the BCIS and subjective QOL. These findings suggest that cognitive insight, particularly SR, is associated with subjective QOL in patients with schizophrenia via right frontotemporal function. The findings of this study provide important insight into a QOL model of schizophrenia, which may guide the development of cost-effective interventions that target frontotemporal function in patients with schizophrenia.

  2. [Research on child neglect situation and influential factors of left-behind children and living-with-parents children aged 6-17 year-old in rural areas of two provinces, western China].

    Science.gov (United States)

    Zhong, Yin; Zhong, Zhaohui; Pan, Jianping; Li, Qunying; Zhong, Yun; Sun, Haoling

    2015-10-01

    To investigate the situation and the influential factors of child neglect between left-behind children and living-with-parents children aged 6-17 years in the rural areas in western China. Students were randomly selected according to the principle of multi-stage stratified cluster sampling and they were from three cities in Shanxi and four districts in Chongqing. Among the 4,131 children, there were 1,874 students in the 6-11 years group and the left-behind children accounted for 50.21% (941/1,874) in this group. There were 2,257 students in the 12-17 years old group and the left-behind children accounted for 53.35% (1,204/2,257) in this group. The questionnaire named "Evaluation on Neglect for Elementary and Secondary School Students Aged 6-17 Years in Rural Areas in China" was used in the field investigation. The students' neglect frequency was described by neglect rate and the factors affecting students' neglect were analyzed by means of binary logistic regression. In the 6-11 years old group, the neglect rates of left-behind children and living-with-parents children were 63.03% (474/752) and 43.87% (347/791), respectively (χ2=58.86, Pchildren and living-with-parents children were 60.64% (627/1 034) and 53.57% (495/924), respectively (χ2=9.96, Pchildren, compared to the factors about boys, younger mother (≤40 years old), presence of parents' income reduction within the last year and nuclear family, the factors about girls, elder mother (41-49 years), absence of parents' income reduction within the last year and three-generation family or single-parent family were associated with lower neglect risk, and OR values were 0.67, 0.68, 0.70, 0.73, and 0.43 (Pchildren, the factors about other nationalities, non-only child, no separate room at home, and non-resident children were associated with high neglect risk, and OR values were 1.85, 1.34, 1.46, and 1.32 (Pchildren and parents as well as good relationship between parents, fair or poor relationship was associated

  3. Health allowance for improving the nutritional status and development of 3-5-year-old left-behind children in poor rural areas of China: study protocol for a cluster randomised trial.

    Science.gov (United States)

    Lin, Qian; Adab, Peymané; Hemming, Karla; Yang, Lina; Qin, Hong; Li, Mingzhi; Deng, Jing; Shi, Jingcheng; Chen, Jihua

    2015-08-18

    Left-behind children (LBC) are recognised as a new social group in China. LBC are young children who are abandoned in rural villages whilst their parents travel to distant urban centres for employment (a new generation of migrant workers). Following the rapid growth in the number of migrant workers, the LBC population is also rapidly increasing. These children are usually left to be raised by elderly grandparents, a single parent, or sometimes distant relatives or neighbours who have limited resources, tend to have a poor education and sometimes are in frail health. Over 40 % of the 61 million LBC in China who are under 5 years old are undernourished, which affects their long-term health and abilities. An intervention that combines a conditional cash transfer (CCT) with nutrition education offers a potential solution. A cluster randomised controlled trial design will be used to allocate 40 villages to the intervention arm (20 villages) or control arm (20 villages). The caregivers and all of the 3-5-year-old LBC will be the target population. Caregivers in the intervention arm will receive a cash allowance conditional on attending nutrition education sessions, ensuring that the LBC will use basic public health services over a 12-month period. At the baseline, midterm (month 6) and end (month 12) of the intervention period, evaluations will be conducted in all 40 villages. Multilevel generalised linear models will be used to analyse the impact of the intervention on nutrition status and other outcomes, adjusting for baseline levels using an analysis of covariance approach. The cost of the intervention will also be estimated. If found to be cost-effective, the findings will inform the development of a sustainable model to improve nutrition status among LBC in rural areas of China. Chinese Trial Register (ChiCTR) identifier: CTXY-140003-2 . Registered on 19 Aug 2014.

  4. Areas of Active Tectonic Uplift Are Sensitive to Small Changes in Fold Orientations within a Broad Zone of Left-lateral Transpression and Shearing, Dominican Republic and Haiti (Hispaniola)

    Science.gov (United States)

    Ambrosius, I.; Mann, P.

    2014-12-01

    Previous GPS studies have shown that the island of Hispaniola is a 250 km-wide zone of active, east-west, left-lateral shearing along two major strike-slip zones: the Septentrional-Oriente fault zone through the northern part of the island and the Enriquillo-Plantain Garden fault zone (EPGFZ) through the southern part of the island. The total interplate rate distributed on both faults is 21 mm/yr. Using a high-resolution DEM, we constructed fluvial channel profiles across transpression-related folds of late Miocene to recent age in the area of central and southern Dominican Republic and Haiti to determine controls of areas of relatively high, moderate, and slow uplift inferred from fluvial channel profiles. Fold axes in this area extend for 50-150 km and exhibit two different trends: 1) folds that occupy the area of the Sierra de Neiba-Chaine des Matheux north of the Enriquillo-Cul-de-Sac Valley and EPGFZ and folds that occupy the area of the Sierra de Bahoruco-Massif de la Selle all exhibit more east-west fold axes trending 110; 2) folds that occupy the area northwest of the EPGFZ in the western Chaine des Matheux and Sierra de Neiba all exhibit fold axes with more northwest trends of 125. River channel profiles show that the second group of more northwesterly-trending fold axes show relatively higher rates of tectonic uplift based on their convex-upward river profiles. Our interpretation for regional variations in river profiles and inferred uplift is that uplift is more pronounced on fold axes trending 15 degrees more to the northwest because their axes are more oblique to the interplate direction of east-west shearing. Longterm uplift rates previously measured from a stairstep of late Quaternary coral terraces at the plunging nose of the westernmost Chaine des Matheux have been previously shown to be occurring at a rate of 0.19 mm/yr. Onland exposures of Holocene corals are found only on one locality within the southern area of folds 30 km west of the epicenter

  5. White matter tract signatures of impaired social cognition in frontotemporal lobar degeneration

    Directory of Open Access Journals (Sweden)

    Laura E. Downey

    2015-01-01

    Full Text Available Impairments of social cognition are often leading features in frontotemporal lobar degeneration (FTLD and likely to reflect large-scale brain network disintegration. However, the neuroanatomical basis of impaired social cognition in FTLD and the role of white matter connections have not been defined. Here we assessed social cognition in a cohort of patients representing two core syndromes of FTLD, behavioural variant frontotemporal dementia (bvFTD; n = 29 and semantic variant primary progressive aphasia (svPPA; n = 15, relative to healthy older individuals (n = 37 using two components of the Awareness of Social Inference Test, canonical emotion identification and sarcasm identification. Diffusion tensor imaging (DTI was used to derive white matter tract correlates of social cognition performance and compared with the distribution of grey matter atrophy on voxel-based morphometry. The bvFTD and svPPA groups showed comparably severe deficits for identification of canonical emotions and sarcasm, and these deficits were correlated with distributed and overlapping white matter tract alterations particularly affecting frontotemporal connections in the right cerebral hemisphere. The most robust DTI associations were identified in white matter tracts linking cognitive and evaluative processing with emotional responses: anterior thalamic radiation, fornix (emotion identification and uncinate fasciculus (sarcasm identification. DTI associations of impaired social cognition were more consistent than corresponding grey matter associations. These findings delineate a brain network substrate for the social impairment that characterises FTLD syndromes. The findings further suggest that DTI can generate sensitive and functionally relevant indexes of white matter damage in FTLD, with potential to transcend conventional syndrome boundaries.

  6. The tailored activity program (TAP) to address behavioral disturbances in frontotemporal dementia: a feasibility and pilot study.

    Science.gov (United States)

    O'Connor, Claire M; Clemson, Lindy; Brodaty, Henry; Low, Lee-Fay; Jeon, Yun-Hee; Gitlin, Laura N; Piguet, Olivier; Mioshi, Eneida

    2017-10-15

    To explore the feasibility of implementing the Tailored Activity Program with a cohort of people with frontotemporal dementia and their carers (dyads). The Tailored Activity Program is an occupational therapy based intervention that involves working collaboratively with family carers and prescribes personalized activities for behavioral management in people with dementia. Twenty dyads randomized into the study (Tailored Activity Program: n = 9; Control: n = 11) were assessed at baseline and 4-months. Qualitative analyzes evaluated feasibility and acceptability of the program for the frontotemporal dementia cohort, and quantitative analyzes (linear mixed model analyzes, Spearman's rho correlations) measured the impact of the program on the dyads. The Tailored Activity Program was an acceptable intervention for the frontotemporal dementia dyads. Qualitative analyses identified five themes: "carer perceived benefits", "carer readiness to change", "strategies used by carer to engage person with dementia", "barriers to the Tailored Activity Program uptake/implementation", and "person with dementia engagement". Quantitative outcomes showed an overall reduction of behavioral symptoms (F 18.34  = 8.073, p = 0.011) and maintenance of functional performance in the person with dementia (F 18.03  = 0.375, p = 0.548). This study demonstrates the potential for using an activity-based intervention such as the Tailored Activity Program in frontotemporal dementia. Service providers should recognize that while people with frontotemporal dementia present with challenging issues, tailored therapies may support their function and reduce their behavioral symptoms. Implications for rehabilitation The Tailored Activity Program is an occupational therapy based intervention that involves prescribing personalized activities for behavioral management in dementia. The Tailored Activity Program is an acceptable and feasible intervention approach to address some of the

  7. C9ORF72 Repeat Expansion in Australian and Spanish Frontotemporal Dementia Patients

    OpenAIRE

    Dobson-Stone, Carol; Hallupp, Marianne; Loy, Clement T.; Thompson, Elizabeth M.; Haan, Eric; Sue, Carolyn M.; Panegyres, Peter K.; Razquin, Cristina; Seijo-Mart?nez, Manuel; Rene, Ramon; Gascon, Jordi; Campdelacreu, Jaume; Schmoll, Birgit; Volk, Alexander E.; Brooks, William S.

    2013-01-01

    A hexanucleotide repeat expansion in C9ORF72 has been established as a common cause of frontotemporal dementia (FTD). However, the minimum repeat number necessary for disease pathogenesis is not known. The aims of our study were to determine the frequency of the C9ORF72 repeat expansion in two FTD patient collections (one Australian and one Spanish, combined n = 190), to examine C9ORF72 expansion allele length in a subset of FTD patients, and to examine C9ORF72 allele length in 'non-expansion...

  8. The study of fMRI in Alzheimer's disease, frontotemporal dementia and Lewy bodies dementia

    International Nuclear Information System (INIS)

    Zhang Bing; Xu Jun; Xu Yun; Zhu Bin

    2010-01-01

    Alzheimer's disease (AD), dementia with Lewy bodies (DLB) and frontotemporal dementia (FTD) are the main type of neuro degenerative diseases, but the FTD and DLB are always confused with AD. Structural MRI, diffusion-weighted imaging and proton magnetic resonance spectroscopy have the potential to support the diagnosis of AD and the relative disease. Brain atrophy pattern, apparent diffusion coefficient and fractional anisotropy pattern, the distribution mode of N-acetylaspartate and myo-inositol in temporal lobe, hippocampus, parietal lobe, frontal lobe could help to differentiate AD from FTD, DLB and those patterns are in accordance with the pathological changes. (authors)

  9. Left atrial volume index

    DEFF Research Database (Denmark)

    Poulsen, Mikael K; Dahl, Jordi S; Henriksen, Jan Erik

    2013-01-01

    To determine the prognostic importance of left atrial (LA) dilatation in patients with type 2 diabetes (T2DM) and no history of cardiovascular disease.......To determine the prognostic importance of left atrial (LA) dilatation in patients with type 2 diabetes (T2DM) and no history of cardiovascular disease....

  10. Telephone based cognitive-behavioral screening for frontotemporal changes in patients with amyotrophic lateral sclerosis (ALS).

    Science.gov (United States)

    Christodoulou, Georgia; Gennings, Chris; Hupf, Jonathan; Factor-Litvak, Pam; Murphy, Jennifer; Goetz, Raymond R; Mitsumoto, Hiroshi

    Our objective was to establish a valid and reliable battery of measures to evaluate frontotemporal dementia (FTD) in patients with ALS over the telephone. Thirty-one subjects were administered either in-person or by telephone-based screening followed by the opposite mode of testing two weeks later, using a modified version of the UCSF Cognitive Screening Battery. Equivalence testing was performed for in-person and telephone based tests. The standard ALS Cognitive Behavioral Screen (ALS-CBS) showed statistical equivalence at the 5% significance level compared to a revised phone version of the ALS-CBS. In addition, the Controlled Oral Word Association Test (COWAT) and Center for Neurologic Study-Lability Scale (CNS-LS) were also found to be equivalent at the 5% and 10% significance level, respectively. Similarly, the Mini-Mental State Examination (MMSE) and the well-established Telephone Interview for Cognitive Status (TICS) were also statistically equivalent. Equivalence could not be claimed for the ALS-Frontal Behavioral Inventory (ALS-FBI) caregiver interview and the Written Verbal Fluency Index (WVFI). In conclusion, our study suggests that telephone-based versions of the ALS-CBS, COWAT, and CNS-LS may offer clinicians valid tools to detect frontotemporal changes in the ALS population. Development of telephone based cognitive testing for ALS could become an integral resource for population based research in the future.

  11. Co-Occurrence of Language and Behavioural Change in Frontotemporal Lobar Degeneration

    Directory of Open Access Journals (Sweden)

    Jennifer M. Harris

    2016-06-01

    Full Text Available Background/Objectives: We aimed to evaluate the co-occurrence of language and behavioural impairment in patients with frontotemporal lobar degeneration (FTLD spectrum pathology. Methods: Eighty-one dementia patients with pathological confirmation of FTLD were identified. Anonymized clinical records from patients' first assessment were rated for language and behavioural features from frontotemporal dementia consensus criteria, primary progressive aphasia (PPA criteria and 1998 FTLD criteria. Results: Over 90% of patients with FTLD pathology exhibited a combination of at least one behavioural and one language feature. Changes in language, in particular, were commonly accompanied by behavioural change. Notably, the majority of patients who displayed language features characteristic of semantic variant PPA exhibited ‘early perseverative, stereotyped or compulsive/ritualistic behaviour'. Moreover, ‘executive/generation deficits with relative sparing of memory and visuospatial functions' occurred in most patients with core features of non-fluent variant PPA. Conclusion: Behavioural and language symptoms frequently co-occur in patients with FTLD pathology. Current classifications, which separate behavioural and language syndromes, do not reflect this co-occurrence.

  12. PROGRANULIN MUTATIONS AFFECTS BRAIN OSCILLATORY ACTIVITY IN FRONTO-TEMPORAL DEMENTIA

    Directory of Open Access Journals (Sweden)

    Davide Vito Moretti

    2016-02-01

    Full Text Available Background: mild cognitive impairment (MCI is a clinical stage indicating a prodromal phase of dementia. This practical concept could be used also for fronto-temporal dementia (FTD. Progranulin (PGRN has been recently recognized as a useful diagnostic biomarker for fronto-temporal lobe degeneration (FTLD due to GRN null mutations. Electroencephalography (EEG is a reliable tool in detecting brain networks changes. The working hypothesis of the present study is that EEG oscillations could detect different modifications among FTLD stages (FTD-MCI versus overt FTD as well as differences between GRN mutation carriers versus non carriers in patients with overt FTD. Methods: EEG in all patients and PGRN dosage in patients with a clear FTD were detected. The cognitive state has been investigated through mini mental state examination (MMSE. Results: MCI-FTD showed a significant lower spectral power in both alpha and theta oscillations as compared to overt FTD. GRN mutations carriers affected by FTLD show an increase in high alpha and decrease in theta oscillations as compared to non-carriers.Conclusion: EEG frequency rhythms are sensible to different stage of FTD and could detect changes in brain oscillatory activity affected by GRN mutations

  13. The impact of dementia severity on caregiver burden in frontotemporal dementia and Alzheimer disease.

    Science.gov (United States)

    Mioshi, Eneida; Foxe, David; Leslie, Felicity; Savage, Sharon; Hsieh, Sharpley; Miller, Laurie; Hodges, John R; Piguet, Olivier

    2013-01-01

    Caregiver burden is greater in frontotemporal dementia (FTD) than in Alzheimer disease (AD). However, little is known of the impact of the 3 main clinical variants of FTD- behavioral-variant frontotemporal dementia (bvFTD), semantic dementia (SemDem), and progressive nonfluent aphasia (PNFA)-or the role of disease severity in caregiver burden. The Zarit Burden Inventory was used to measure caregiver burden of bvFTD (n=17), SemDem (n=20), PNFA (n=20), and AD (n=19) patients. Symptom duration, caregiver age, and relationship type were matched across groups. Moreover, a number of caregiver (mood, social network) and patient variables (functional disability, behavioral changes, relationship with caregiver, and dementia stage) were addressed to investigate their impact on caregiver burden. Caregivers of bvFTD patients reported the highest burden, whereas SemDem and PNFA caregivers reported burden similar to AD. A regression analysis revealed that caregiver burden in FTD, regardless of subtype, was explained by a model combining disease staging, relationship changes, and caregiver depression. Burden increased with disease severity in FTD. This study is the first to show that caregivers of SemDem, PNFA, and AD patients show similar burden, while confirming that bvFTD caregivers show higher burden than AD caregivers. More importantly, this study demonstrates that burden worsens with disease progression in FTD.

  14. Comparing moral judgments of patients with frontotemporal dementia and frontal stroke.

    Science.gov (United States)

    Baez, Sandra; Couto, Blas; Torralva, Teresa; Sposato, Luciano A; Huepe, David; Montañes, Patricia; Reyes, Pablo; Matallana, Diana; Vigliecca, Nora S; Slachevsky, Andrea; Manes, Facundo; Ibanez, Agustin

    2014-09-01

    Several clinical reports have stated that patients with prefrontal lesions or patients with the behavioral variant of frontotemporal dementia share social cognition impairments. Moral reasoning is impaired in both conditions but there have been few investigations that directly compare this domain in the 2 groups. This work compared the moral judgments of these patient groups using a task designed to disentangle the contributions of intentions and outcomes in moral judgment. For both disorders, patients judged scenarios where the protagonists believed that they would cause harm but did not as being more permissible than the control group. Moreover, patients with frontotemporal dementia judged harmful outcomes in the absence of harmful intentions as less permissible than the control participants. There were no differences between the 2 conditions. Both disorders involved impairments in integrating intention and outcome information for moral judgment. This study was the first, to our knowledge, to directly compare a social cognition domain in 2 frontal pathologies with different etiology. Our results highlighted the importance of comparing patients with vascular lesions and patients with neurodegenerative diseases.

  15. The Relationship between Subclinical Asperger’s Syndrome and Frontotemporal Lobar Degeneration

    Directory of Open Access Journals (Sweden)

    Akira Midorikawa

    2012-04-01

    Full Text Available Background/Aims: The existence of the behavioral variant of frontotemporal dementia (bv-FTD, including senile Asperger’s syndrome (AS, has been proposed. However, there are no empirical case reports to support the proposal. In this report, we present 3 patients who showed symptoms of bv-FTD and demonstrated signs of autistic spectrum disorder, especially AS. Methods: We evaluated 3 subjects using the diagnostic criteria for bv-FTD, and their caregivers retrospectively provided data to calculate the Autism-Spectrum Quotient, Japanese version [Wakabayashi et al.: Shinrigaku Kenkyu 2004;75:78–84]. We also compared these data with those obtained from 3 individuals with Alzheimer’s disease. Results: All 3 patients met the criteria for bv-FTD and had a higher Autism-Spectrum Quotient score than did comparable Alzheimer’s disease subjects. Conclusion: It is possible that some senile persons with frontotemporal lobar degeneration-like maladaptive behavior may suffer from subclinical AS.

  16. Ludopatía y epilepsia en demencia frontotemporal: Reporte de dos casos

    Directory of Open Access Journals (Sweden)

    Sheila Castro-Suarez

    Full Text Available RESUMEN La demencia frontotemporal es un trastorno neurodegenerativo cuyo subtipo más común es la variante conductual. Es considerada la causa de demencia más frecuente en menores de 60 años. Se presentan dos casos no emparentados cuyos síntomas típicos fueron: deterioro progresivo de la cognición, del comportamiento, alteraciones psiquiátricas como desinhibición, actos impulsivos, apatía, falta de empatía, estereotipias y cambios en los hábitos de alimentación. La resonancia magnética cerebral, en ambos casos, mostró atrofia frontotemporal a predominio izquierdo; en la evaluación neuropsicológica se evidenció alteraciones de las funciones ejecutivas. El primer caso cursó con ludopatía como síntoma inicial, motivo por el cual permaneció por un año en un centro psiquiátrico, siendo el segundo en Latinoamérica y uno de los pocos casos reportados en el mundo. El segundo caso presentó crisis epilépticas en su evolución.

  17. The structural neuroanatomy of music emotion recognition: evidence from frontotemporal lobar degeneration.

    Science.gov (United States)

    Omar, Rohani; Henley, Susie M D; Bartlett, Jonathan W; Hailstone, Julia C; Gordon, Elizabeth; Sauter, Disa A; Frost, Chris; Scott, Sophie K; Warren, Jason D

    2011-06-01

    Despite growing clinical and neurobiological interest in the brain mechanisms that process emotion in music, these mechanisms remain incompletely understood. Patients with frontotemporal lobar degeneration (FTLD) frequently exhibit clinical syndromes that illustrate the effects of breakdown in emotional and social functioning. Here we investigated the neuroanatomical substrate for recognition of musical emotion in a cohort of 26 patients with FTLD (16 with behavioural variant frontotemporal dementia, bvFTD, 10 with semantic dementia, SemD) using voxel-based morphometry. On neuropsychological evaluation, patients with FTLD showed deficient recognition of canonical emotions (happiness, sadness, anger and fear) from music as well as faces and voices compared with healthy control subjects. Impaired recognition of emotions from music was specifically associated with grey matter loss in a distributed cerebral network including insula, orbitofrontal cortex, anterior cingulate and medial prefrontal cortex, anterior temporal and more posterior temporal and parietal cortices, amygdala and the subcortical mesolimbic system. This network constitutes an essential brain substrate for recognition of musical emotion that overlaps with brain regions previously implicated in coding emotional value, behavioural context, conceptual knowledge and theory of mind. Musical emotion recognition may probe the interface of these processes, delineating a profile of brain damage that is essential for the abstraction of complex social emotions. Copyright © 2011 Elsevier Inc. All rights reserved.

  18. A case series of PLS patients with frontotemporal dementia and overview of the literature.

    Science.gov (United States)

    de Vries, Bálint S; Rustemeijer, Laura M M; van der Kooi, Anneke J; Raaphorst, Joost; Schröder, Carin D; Nijboer, Tanja C W; Hendrikse, Jeroen; Veldink, Jan H; van den Berg, Leonard H; van Es, Michael A

    2017-11-01

    Primary lateral sclerosis (PLS) is a rare form of motor neuron disease characterised by UMN degeneration leading to slowly progressive spasticity. Whether it is a separate disease or a subtype of ALS has been debated. In ALS comorbid frontotemporal dementia (FTD) is frequently seen (±15%). However, cognitive and behavioural changes are generally not considered to be a part of PLS. To report the clinical findings and frequency of PLS patients that developed FTD in a referral-based cohort and provide an overview of the literature. In our cohort six out of 181 (3.3%) PLS patients developed FTD. In the literature a few cases of PLS with FTD have been reported and only a limited number of small studies have investigated cognition in PLS. However, when these studies are summarised a pattern emerges with FTD diagnoses in ±2% and frontotemporal impairment in 22% of patients. These findings suggest that PLS is part of the FTD-MND continuum and would favour viewing it as a subtype of ALS. It is, however, not a restricted (isolated UMN involvement) phenotype.

  19. History, Present, and Progress of Frontotemporal Dementia in China: A Systematic Review

    Directory of Open Access Journals (Sweden)

    Ru-Jing Ren

    2012-01-01

    Full Text Available We aim to provide an overview of clinical and demographical features and neuropathological research on frontotemporal dementia (FTD from China over the past decade. We reviewed the demographic features, clinical presentations, and neuropathology of the FTD-spectrum disorders from the 49 cases in China published since 1998. On the basis of these findings, we retrospect the history and speculate on future progress in terms of FTD in China. We found that most published papers comprise case reports with a few retrospective studies with small sample sizes. Behavior variant FTD (bvFTD was the most common diagnostic subtype, of which 35% were associated with amyotrophic lateral sclerosis or Parkinsonian syndrome. More than 47% patients with FTD had age onset before 65. There were no differences in age of onset and sex distribution between diagnostic subtypes. The spectrum of neuropathological diagnosis of bvFTD was frontotemporal lobe degeneration (FTLD with tau protein or ubiquitin-immunopositive inclusions, and FTLD without intracellular inclusions. Median survival in bvFTD was 14 years. This paper provides an overview of the current status and pointers for future research directions of FTD in China.

  20. Right Fronto-Temporal EEG can Differentiate the Affective Responses to Award-Winning Advertisements.

    Science.gov (United States)

    Wang, Regina W Y; Huarng, Shy-Peih; Chuang, Shang-Wen

    2018-04-01

    Affective engineering aims to improve service/product design by translating the customer's psychological feelings. Award-winning advertisements (AAs) were selected on the basis of the professional standards that consider creativity as a prerequisite. However, it is unknown if AA is related to satisfactory advertising performance among customers or only to the experts' viewpoints towards the advertisements. This issue in the field of affective engineering and design merits in-depth evaluation. We recruited 30 subjects and performed an electroencephalography (EEG) experiment while watching AAs and non-AAs (NAAs). The event-related potential (ERP) data showed that AAs evoked larger positive potentials 250-1400 [Formula: see text]ms after stimulus onset, particularly in the right fronto-temporal regions. The behavioral results were consistent with the professional recognition given to AAs by experts. The perceived levels of creativity and "product-like" quality were higher for the AAs than for the NAAs. Event-related spectral perturbation (ERSP) analysis further revealed statistically significant differences in the theta, alpha, beta, and gamma band activity in the right fronto-temporal regions between the AAs and NAAs. Our results confirm that EEG features from the time/frequency domains can differentiate affective responses to AAs at a neural circuit level, and provide scientific evidence to support the identification of AAs.

  1. Reduction in left supplementary motor area grey matter in adult female fibromyalgia sufferers with marked fatigue and without affective disorder: a pilot controlled 3-T magnetic resonance imaging voxel-based morphometry study.

    Science.gov (United States)

    Puri, B K; Agour, M; Gunatilake, K Dr; Fernando, K Ac; Gurusinghe, A I; Treasaden, I H

    2010-01-01

    This study aimed to test the hypothesis that structural grey matter brain changes might occur in the chronic intractable pain disorder fibromyalgia when this is associated with marked fatigue in the absence of a DSM-IV-TR (Diagnostic and Statistical Manual of Mental Disorders, 4th edition, text revision) diagnosis of affective disorder. High-resolution 3-T cerebral magnetic resonance imaging scans were acquired in 10 female, right-handed, non-smoking, white Caucasian subjects: five patients with fibromyalgia associated with marked fatigue and five age-matched healthy women. Voxel-wise generalized linear modelling of the processed neuroanatomical data using permutation-based non-parametric testing, forming clusters at t > 2.3 and testing clusters for significance at P fibromyalgia and marked fatigue in the left supplementary motor area. This brain region plays an important role in cognitive or executive control and in the translation of painful cognition; these functions are impaired in fibromyalgia associated with marked fatigue.

  2. What is left behind when the lights go off? Comparing the abundance and composition of litter in urban areas with different intensity of nightlife use in Mar del Plata, Argentina.

    Science.gov (United States)

    Becherucci, Maria Eugenia; Seco Pon, Juan Pablo

    2014-08-01

    Nightlife activities represents an important source of urban litter; the latter often being left behind or abandoned in public places and streets. Mar del Plata is a very important city on the Atlantic coast of Argentina and is the main tourism destination in the South Atlantic region of South America. However, few studies on urban litter related to nightlife activities have been conducted in the area. Here we assessed (i) the abundance and composition of litter, and (ii) the spatial and temporal variations of its abundance, diversity, richness and evenness in urbanized areas with different intensity of nightlife activities from April 2008 to March 2009. An overall of 13,503 items were counted. Around 92% of the total litter was comprised by cigarette butts, papers and plastics. We found significant spatial differences in the abundance of litter between sampling sites, with the greatest amounts of litter at the Alem site followed by the Hipólito site (both with an intensive nightlife activity) compared with the Chauvin site (a quiet high-income neighborhood). The composition of litter of the Alem and the Hipólito sites was relatively similar and both sites differ with respect to the Chauvin site. Cigarette butts, papers, and plastics were the items that contributed most to the dissimilarity between sampling sites. The diversity of litter was the single community parameter that significantly differed from the other seasons. We discussed the potential effect of nightlife activities on the amounts and quality of urban litter in the city of Mar del Plata. Copyright © 2014 Elsevier Ltd. All rights reserved.

  3. Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation

    DEFF Research Database (Denmark)

    Clayton, Emma L.; Mancuso, Renzo; Nielsen, Troels Tolstrup

    2017-01-01

    Frontotemporal dementia (FTD)-causing mutations in the CHMP2B gene lead to the generation of mutant C-terminally truncated CHMP2B. We report that transgenic mice expressing endogenous levels of mutant CHMP2B developed late-onset brain volume loss associated with frank neuronal loss and FTD-like c...

  4. TMEM106B and ApoE polymorphisms in CHMP2B-mediated frontotemporal dementia (FTD-3)

    DEFF Research Database (Denmark)

    Rostgaard, Nina; Roos, Peter; Budtz-Jørgensen, Esben

    2017-01-01

    Single-nucleotide polymorphisms in the TMEM106B gene have been identified as a risk factor in frontotemporal dementia (FTD). The major allele of SNP rs3173615 is a risk factor in sporadic FTD, whereas the minor allele seems protective in GRN- and C9orf72-mediated FTD. The role of apolipoprotein E...

  5. TMEM106B is a genetic modifier of frontotemporal lobar degeneration with C9orf72 hexanucleotide repeat expansions

    NARCIS (Netherlands)

    Gallagher, M.D.; Suh, E.R.; Grossman, M.; Elman, L.; McCluskey, L.; van Swieten, J.C.; Al-Sarraj, S.; Neumann, M.; Gelpi, E.; Ghetti, B.; Rohrer, J.D.; Halliday, G.; Van Broeckhoven, C.; Seilhean, D.; Shaw, P.J.; Frosch, M.P.; Alafuzoff, I.; Antonell, A.; Bogdanovic, N.; Brooks, W.; Cairns, N.J.; Cooper-Knock, J.; Cotman, C.; Cras, P.; Cruts, M.; de Deyn, P.P.; deCarli, C.; Dobson-Stone, C.; Engelborghs, S.; Fox, N.; Galasko, D.; Gearing, M.; Gijselinck, I.; Grafman, J.; Hartikainen, P.; Hatanpaa, K.J.; Highley, J.R.; Hodges, J.; Hulette, C.; Ince, P.G.; Jin, L.W.; Kirby, J.; Kofler, J.; Kril, J.; Kwok, J.B.J.; Levey, A.; Lieberman, A.; Llado, A.; Martin, J.J.; Masliah, E.; McDermott, C.J.; McKee, A.; McLean, C.; Mead, S.; Miller, C.A.; Miller, J.; Munoz, D.G.; Murrell, J.; Paulson, H.; Piguet, O.; Rossor, M.; Sanchez-Valle, R.; Sano, M.; Schneider, J.; Silbert, L.C.; Spina, S.; van der Zee, J.; Van Langenhove, T.; Warren, J.; Wharton, S.B.; White, C.L.; Woltjer, R.L.; Trojanowski, J.Q.; Lee, V.M.Y.; Van Deerlin, V.; Chen-Plotkin, A.S.

    2014-01-01

    Hexanucleotide repeat expansions in chromosome 9 open reading frame 72 (C9orf72) have recently been linked to frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis, and may be the most common genetic cause of both neurodegenerative diseases. Genetic variants at TMEM106B

  6. Characterization of Movement Disorder Phenomenology in Genetically Proven, Familial Frontotemporal Lobar Degeneration: A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Gasca-Salas, Carmen; Masellis, Mario; Khoo, Edwin; Shah, Binit B; Fisman, David; Lang, Anthony E; Kleiner-Fisman, Galit

    2016-01-01

    Mutations in granulin (PGRN) and tau (MAPT), and hexanucleotide repeat expansions near the C9orf72 genes are the most prevalent genetic causes of frontotemporal lobar degeneration. Although behavior, language and movement presentations are common, the relationship between genetic subgroup and movement disorder phenomenology is unclear. We conducted a systematic review and meta-analysis of the literature characterizing the spectrum and prevalence of movement disorders in genetic frontotemporal lobar degeneration. Electronic databases were searched using terms related to frontotemporal lobar degeneration and movement disorders. Articles were included when cases had a proven genetic cause. Study-specific prevalence estimates for clinical features were transformed using Freeman-Tukey arcsine transformation, allowing for pooled estimates of prevalence to be generated using random-effects models. The mean age at onset was earlier in those with MAPT mutations compared to PGRN (pphenomenology in genetic frontotemporal lobar degeneration. Standardized prospective collection of clinical information in conjunction with genetic characterization will be crucial for accurate clinico-genetic correlation.

  7. A decade of genetic counseling in frontotemporal dementia affected families: Few counseling requests and much familial opposition to testing

    NARCIS (Netherlands)

    S.R. Riedijk (Samantha); M.F. Niermeijer (Martinus); D. Dooijes (Dennis); A. Tibben (Arend)

    2009-01-01

    textabstractA decade of genetic counseling of frontotemporal dementia (FTD) affected families has generated two important observations. First, the uptake rate for presymptomatic testing for FTD is low in our department of Clinical Genetics at the Erasmus Medical Center in the Netherlands. Second,

  8. Frontotemporal dementia: Change of familial caregiver burden and partner relation in a Dutch cohort of 63 patients

    NARCIS (Netherlands)

    S.R. Riedijk (Samantha); H.J. Duivenvoorden (Hugo); S.M. Rosso (Sonia); J.C. van Swieten (John); M.F. Niermeijer (Martinus); A. Tibben (Arend)

    2008-01-01

    textabstractBackground/Aims: The current study examined the change of caregiver burden and the development of the quality of the partner relation in frontotemporal dementia (FTD). Methods: During a 2-year period, deterioration, behavioural problems, caregiver burden, general psychopathology, quality

  9. Frontotemporal dementia: change of familial caregiver burden and partner relation in a Dutch cohort of 63 patients.

    NARCIS (Netherlands)

    Riedijk, S.R.; Duivenvoorden, H.J.; Rosso, S.; Swieten, J. van; Niermeijer, M.F.; Tibben, A.

    2008-01-01

    BACKGROUND/AIMS: The current study examined the change of caregiver burden and the development of the quality of the partner relation in frontotemporal dementia (FTD). METHODS: During a 2-year period, deterioration, behavioural problems, caregiver burden, general psychopathology, quality of life,

  10. Clinical Utility of Short Social Cognitive Tests in Early Differentiation of Behavioral Variant Frontotemporal Dementia from Alzheimer’s Disease

    DEFF Research Database (Denmark)

    Buhl, Christian; Stokholm, Jette; Gade, Anders

    2013-01-01

    Traditional cognitive tests used in clinical practice may not be sensitive enough for the early differentiation of behavioral variant frontotemporal dementia (bvFTD) from Alzheimer's disease (AD). A growing body of literature has shown that deficits in various aspects of social cognition can...

  11. A Modified Reading the Mind in the Eyes Test Predicts Behavioral Variant Frontotemporal Dementia Better Than Executive Function Tests

    Directory of Open Access Journals (Sweden)

    Matthias L. Schroeter

    2018-01-01

    Full Text Available Behavioral variant frontotemporal dementia (bvFTD is characterized by deep alterations in behavior and personality. Although revised diagnostic criteria agree for executive dysfunction as most characteristic, impairments in social cognition are also suggested. The study aimed at identifying those neuropsychological and behavioral parameters best discriminating between bvFTD and healthy controls. Eighty six patients were diagnosed with possible or probable bvFTD according to Rascovsky et al. (2011 and compared with 43 healthy age-matched controls. Neuropsychological performance was assessed with a modified Reading the Mind in the Eyes Test (RMET, Stroop task, Trail Making Test (TMT, Hamasch-Five-Point Test (H5PT, and semantic and phonemic verbal fluency tasks. Behavior was assessed with the Apathy Evaluation Scale, Frontal Systems Behavioral Scale, and Bayer Activities of Daily Living Scale. Each test’s discriminatory power was investigated by Receiver Operating Characteristic curves calculating the area under the curve (AUC. bvFTD patients performed significantly worse than healthy controls in all neuropsychological tests. Discriminatory power (AUC was highest in behavioral questionnaires, high in verbal fluency tasks and the RMET, and lower in executive function tests such as the Stroop task, TMT and H5PT. As fluency tasks depend on several cognitive functions, not only executive functions, results suggest that the RMET discriminated better between bvFTD and control subjects than other executive tests. Social cognition should be incorporated into diagnostic criteria for bvFTD in the future, such as in the International Classification of Diseases (ICD-11, as already suggested in the Diagnostic and Statistical Manual for Mental Disorders (DSM-5.

  12. Left heart catheterization

    Science.gov (United States)

    Catheterization - left heart ... to help guide the catheters up into your heart and arteries. Dye (sometimes called "contrast") will be ... in the blood vessels that lead to your heart. The catheter is then moved through the aortic ...

  13. Assessment of left and right ventricular parameters in healthy Korean volunteers using cardiac magnetic resonance imaging: change in ventricular volume and function based on age, gender and body surface area.

    Science.gov (United States)

    Chang, Sung-A; Choe, Yeon Hyeon; Jang, Shin Yi; Kim, Sung Mok; Lee, Sang-Chol; Oh, Jae K

    2012-12-01

    The clinical utility of cardiac magnetic resonance imaging (CMR) is growing and is being used predominantly as a means of measuring ventricular function. The normal reference range of ventricular function may vary based on age, sex and ethnicity. At present, most CMR reference values for healthy individuals have been reported from Western countries. The intent of this study was to investigate the normal CMR reference range for left ventricular (LV) and right ventricular (RV) parameters in healthy Koreans. Healthy volunteers between the ages of 20-70 years without any history of cardiovascular disease or associated risk factors were prospectively recruited to be a part of this study. A total of 124 patients were recruited for this study. Steady-state free precession pulse sequences were used to obtain the cine images for LV and RV volume analysis. All parameters were analyzed based on age and gender, and normalized to body surface area (BSA). LV volume, mass and cardiac output were significantly greater in males than in females. However, all of these parameters which are associated with BSA and gender differences disappeared when corrected for BSA. RV volume was less in females even after the data was normalized for BSA. LV and RV volumes normalized for BSA gradually decreased with greater age, whereas the ejection fraction increased with age, thus maintaining the stroke volume index and cardiac index. LV and RV volumes, mass and function values for a healthy population largely depend on BSA and should be evaluated after normalization by BSA. LV parameters show no difference based on gender, but RV volume is less in the female. Greater age is associated with less ventricular volume, suggesting the possibility of volume sensitivity in the elderly.

  14. Right to Left Ventricular Diameter Ratio ≥0.42 is the Warning Flag for Suspecting Atrial Septal Defect in Preschool Children: Age- and Body Surface Area-Related Reference Values Determined by M-Mode Echocardiography.

    Science.gov (United States)

    Hashimoto, Ikuo; Watanabe, Kazuhiro; Ichida, Fukiko

    2016-04-01

    It is not always easy to observe and screen atrial septal defects (ASD) using echocardiography. In addition, there are no established echocardiographic reference indices for screening patients with ASDs. We retrospectively reviewed our database and recruited 151 isolated ASD patients and 2769 healthy subjects. In total, 307 echocardiographic studies were performed for ASD patients. Surgical repairs were done in 75 of the ASD patients. The ratio of right to left ventricular end-diastolic dimensions (RVD/LVD), which was determined by M-mode echocardiography, was used as an index of RV dilatation. After obtaining age- and body surface area (BSA)-related RVD/LVD nomograms in healthy subjects, we calculated the z-scores of RVD/LVD for all subjects and obtained the optimal cut-off values to differentiate patients with ASD from healthy subjects. The optimal cut-off values were high in neonates and gradually decreased with an increase in the age and BSA, but were almost constant in children aged >4 years or whose BSA was >0.65 m(2). The cut-off values of RVD/LVD for suspected ASD were ≥0.42 in children aged >4 years or those whose BSA was >0.65 m(2). Those for an ASD operation were ≥0.46 in those whose BSA > 0.65 m(2). The RVD/LVD determined by M-mode echocardiography is a useful index to evaluate RV dilatation in patients with ASDs. The RVD/LVD ≥ 0.42 is the warning flag for suspecting ASD in preschool children and that ≥0.46 may be a clinical important sign to determine ASD operation.

  15. Left-Handed Children--Are They Losing Out?

    Science.gov (United States)

    Milsom, Lauren

    1995-01-01

    Discusses difficulties faced by left-handed children in everyday schoolwork. Highlights include right-handed bias of toys, clothing, and tools; the need for guidance in handwriting; problem areas including domestic science, arts and crafts, and metal and woodwork; left-hand advantages in sports and creative arts; and the European Left-Handers Club…

  16. Cerebrospinal Fluid Biomarkers in Familial Forms of Alzheimer's Disease and Frontotemporal Dementia

    DEFF Research Database (Denmark)

    Rostgaard, Nina; Waldemar, Gunhild; Nielsen, Jørgen Erik

    2015-01-01

    , the biomarkers have proved insufficient predictors of dementias with a different pathology, e.g. frontotemporal dementia (FTD); furthermore, the biomarkers are not useful for early AD diagnosis. Familial dementias with a known disease-causing mutation can be extremely valuable to study; yet the biomarker...... profiles in patients with familial dementias are not clear. This review summarizes CSF biomarker findings from studies on symptomatic and presymptomatic individuals carrying a mutation in one of the genes known to cause early-onset familial AD or FTD. In conclusion, the biomarker profile of inherited AD......As dementia is a fast-growing health care problem, it is becoming an increasingly urgent need to provide an early diagnosis in order to offer patients the best medical treatment and care. Validated biomarkers which reflect the pathology and disease progression are essential for diagnosis...

  17. Language in Frontotemporal Dementia: an analysis in light of Enunciative-Discursive Neurolinguistics.

    Science.gov (United States)

    Ucedo, Daniel de Martino; Santos, Karoline Pimentel Dos; Santana, Ana Paula de Oliveira

    2017-08-17

    The aim of this case study was to perform a cross-sectional analysis of spontaneous speech of a patient with Frontotemporal Dementia (FTD). For this purpose, four speech and language therapy episodes, from 2012 to 2014, were selected, transcribed and analyzed in light of Enunciative-Discursive Neurolinguistics. The analysis showed, as the patient's FTD status progressed, that he used different semiotic strategies, e.g., use of repetition and gesture during speech production. It also highlighted the importance of the interlocutor's role of prompting the patient to express verbal meaning. Thus, it can be concluded that the recognition of the strategies used by the patient in favor of his role as a speaker, during interactions, is what enables and legitimates his role.

  18. A Pilot Study on the effects of Music Therapy on Frontotemporal Dementia - developing a research protocol

    DEFF Research Database (Denmark)

    Ridder, Hanne Mette Ochsner; Wigram, Tony; Ottesen, Anne Marie

    2009-01-01

    . As an example of a non-pharmacologic treatment procedure music therapy was investigated. With the focus to develop a research protocol for a future larger population study a pilot study was carried out. In two case studies a combination of data collection methods were examined with the overall goal to document...... of Life (ADRQL), the Cohen-Mansfield Agitation Inventory (CMAI), and the Neuro-Psychiatric Inventory (NPI), and related to case descriptions and video analyses. Results: Recommendations for a mixed method research protocol focused on measuring the effect of music therapy with persons with frontotemporal......, and pharmacological treatment of the psychiatric symptoms is difficult, requiring specialist proficiency in the field. Pilot study: As there is not yet sufficient research that examines the effects of non-pharmacologic treatment with this group there is a need to develop valid and reliable research protocols...

  19. Examining Factors Affecting Caregiver Burden: A Comparison of Frontotemporal Dementia and Alzheimer's Disease.

    Science.gov (United States)

    Küçükgüçlü, Özlem; Söylemez, Burcu Akpınar; Yener, Görsev; Barutcu, Canan Demir; Akyol, Merve Aliye

    2017-06-01

    This study was conducted to compare the caregiver burden with regard to Alzheimer's disease (AD) and frontotemporal dementia (FTD) and to determine the factors affecting the caregiver burden of patients with AD and FTD. A comparative descriptive study design was used. The sample consisted of 90 patients with AD and 44 patients with FTD and their caregivers. Sociodemographic questionnaire, Mini-Mental State Examination (MMSE), the Caregiver Burden Inventory (CBI), Neuropsychiatric Inventory (NPI), and Functional Activities Questionnaire (FAQ) were used. Descriptive statistics, t tests, χ 2 test, and multinominal regression were used for analysis. No difference was found with subscales and the total score of the CBI, except time dependency scale. The total NPI and FAQ scores correlated significantly, and the MMSE score did not show a significant correlation with the AD and FTD caregiver burden. The presence and frequency of neuropsychological symptoms differ among patients with FTD and AD, and different symptoms affect the caregiver burdens in both groups.

  20. Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia.

    Science.gov (United States)

    Lall, Deepti; Baloh, Robert H

    2017-09-01

    Amyotrophic lateral sclerosis (ALS) is a degenerative disorder that is characterized by loss of motor neurons and shows clinical, pathological, and genetic overlap with frontotemporal dementia (FTD). Activated microglia are a universal feature of ALS/FTD pathology; however, their role in disease pathogenesis remains incompletely understood. The recent discovery that ORF 72 on chromosome 9 (C9orf72), the gene most commonly mutated in ALS/FTD, has an important role in myeloid cells opened the possibility that altered microglial function plays an active role in disease. This Review highlights the contribution of microglia to ALS/FTD pathogenesis, discusses the connection between autoimmunity and ALS/FTD, and explores the possibility that C9orf72 and other ALS/FTD genes may have a "dual effect" on both neuronal and myeloid cell function that could explain a shared propensity for altered systemic immunity and neurodegeneration.

  1. Friedrich Nietzsche's mental illness--general paralysis of the insane vs. frontotemporal dementia.

    Science.gov (United States)

    Orth, M; Trimble, M R

    2006-12-01

    For a long time it was thought that Nietzsche suffered from general paralysis of the insane (GPI). However, this diagnosis has been questioned recently, and alternative diagnoses have been proposed. We have charted Friedrich Nietzsche's final fatal illness, and viewed the differential diagnosis in the light of recent neurological understandings of dementia syndromes. It is unclear that Nietzsche ever had syphilis. He lacked progressive motor and other neurological features of a progressive syphilitic central nervous system (CNS) infection and lived at least 12 years following the onset of his CNS signs, which would be extremely rare for patients with untreated GPI. Finally, his flourish of productivity in 1888 would be quite uncharacteristic of GPI, but in keeping with reports of burgeoning creativity at some point in the progression of frontotemporal dementia (FTD). We suggest that Nietzsche did not have GPI, but died from a chronic dementia, namely FTD.

  2. The experience of caregiving: differences between behavioral variant of frontotemporal dementia and Alzheimer disease.

    Science.gov (United States)

    Wong, Cindy; Merrilees, Jennifer; Ketelle, Robin; Barton, Cynthia; Wallhagen, Margaret; Miller, Bruce

    2012-08-01

    To examine caregiver strain, depression, perceived sense of control, and distress from patient neuropsychiatric symptoms in family caregivers of persons with Alzheimer disease (AD) and behavioral variant of frontotemporal dementia (bvFTD) and determine whether group differences exist. Family caregivers were recruited from the Memory and Aging Center in San Francisco, California. Analyses of cross-sectional data on 53 family caregivers (AD = 31, bvFTD = 22) were performed. The Mann-Whitney U test was used to contrast groups. There were statistically significant differences between the AD and bvFTD caregivers in strain, distress, and perceived control but not in depression. On average, bvFTD caregivers experienced greater strain and distress, more depressive symptoms, and lower perceived control. Findings support that experiences of AD and bvFTD caregivers may differ. Further study is needed to identify possible explanatory factors for these group differences.

  3. Relationship satisfaction and emotional language in frontotemporal dementia and Alzheimer disease patients and spousal caregivers.

    Science.gov (United States)

    Ascher, Elizabeth A; Sturm, Virginia E; Seider, Benjamin H; Holley, Sarah R; Miller, Bruce L; Levenson, Robert W

    2010-01-01

    We studied the impact of 2 types of dementia on marital satisfaction and on the emotional language that spouses use during conflictive marital interactions. Fifteen frontotemporal dementia (FTD) and 16 Alzheimer disease (AD) patient-caregiver couples, and 21 control couples, discussed a relationship problem in a laboratory setting. Marital satisfaction was assessed through questionnaire, and emotion language was quantified using text analysis. FTD couples reported lower marital satisfaction than AD and control couples. During the interactions, FTD and AD caregivers used significantly more negative emotion words than their patient spouses (no spousal differences were found in control couples). FTD caregivers also used more negative words than AD caregivers and controls. We interpret these findings as reflecting challenges that the behavioral changes in FTD create for maintaining a healthy marital bond.

  4. Recognition of Facial Expressions of Different Emotional Intensities in Patients with Frontotemporal Lobar Degeneration

    Directory of Open Access Journals (Sweden)

    Roy P. C. Kessels

    2007-01-01

    Full Text Available Behavioural problems are a key feature of frontotemporal lobar degeneration (FTLD. Also, FTLD patients show impairments in emotion processing. Specifically, the perception of negative emotional facial expressions is affected. Generally, however, negative emotional expressions are regarded as more difficult to recognize than positive ones, which thus may have been a confounding factor in previous studies. Also, ceiling effects are often present on emotion recognition tasks using full-blown emotional facial expressions. In the present study with FTLD patients, we examined the perception of sadness, anger, fear, happiness, surprise and disgust at different emotional intensities on morphed facial expressions to take task difficulty into account. Results showed that our FTLD patients were specifically impaired at the recognition of the emotion anger. Also, the patients performed worse than the controls on recognition of surprise, but performed at control levels on disgust, happiness, sadness and fear. These findings corroborate and extend previous results showing deficits in emotion perception in FTLD.

  5. A neurocomputational model of analogical reasoning and its breakdown in frontotemporal lobar degeneration.

    Science.gov (United States)

    Morrison, Robert G; Krawczyk, Daniel C; Holyoak, Keith J; Hummel, John E; Chow, Tiffany W; Miller, Bruce L; Knowlton, Barbara J

    2004-03-01

    Analogy is important for learning and discovery and is considered a core component of intelligence. We present a computational account of analogical reasoning that is compatible with data we have collected from patients with cortical degeneration of either their frontal or anterior temporal cortices due to frontotemporal lobar degeneration (FTLD). These two patient groups showed different deficits in picture and verbal analogies: frontal lobe FTLD patients tended to make errors due to impairments in working memory and inhibitory abilities, whereas temporal lobe FTLD patients tended to make errors due to semantic memory loss. Using the "Learning and Inference with Schemas and Analogies" model, we provide a specific account of how such deficits may arise within neural networks supporting analogical problem solving.

  6. Dendritic Homeostasis Disruption in a Novel Frontotemporal Dementia Mouse Model Expressing Cytoplasmic Fused in Sarcoma

    Directory of Open Access Journals (Sweden)

    Gen Shiihashi

    2017-10-01

    Full Text Available Cytoplasmic aggregation of fused in sarcoma (FUS is detected in brain regions affected by amyotrophic lateral sclerosis (ALS and frontotemporal dementia (FTD, which compose the disease spectrum, FUS proteinopathy. To understand the pathomechanism of ALS-FTD-associated FUS, we examined the behavior and cellular properties of an ALS mouse model overexpressing FUS with nuclear localization signal deletion. Mutant FUS transgenic mice showed hyperactivity, social interactional deficits, and impaired fear memory retrieval, all of which are compatible with FTD phenotypes. Histological analyses showed decreased dendritic spine and synaptic density in the frontal cortex before neuronal loss. Examination of cultured cells confirmed that mutant but not wild-type FUS was associated with decreased dendritic growth, mRNA levels, and protein synthesis in dendrites. These data suggest that cytoplasmic FUS aggregates impair dendritic mRNA trafficking and translation, in turn leading to dendritic homeostasis disruption and the development of FTD phenotypes.

  7. Predictive genetic testing for amyotrophic lateral sclerosis and frontotemporal dementia: genetic counselling considerations.

    Science.gov (United States)

    Crook, Ashley; Williams, Kelly; Adams, Lorel; Blair, Ian; Rowe, Dominic B

    2017-11-01

    Once a gene mutation that is causal of amyotrophic lateral sclerosis (ALS) and/or frontotemporal dementia (FTD) is identified in a family, relatives may decide to undergo predictive genetic testing to determine whether they are at risk of developing disease. Recent advances in gene discovery have led to a pressing need to better understand the implications of predictive genetic testing. Here we review the uptake of genetic counselling, predictive and reproductive testing, and the factors that impact the decision to undergo testing, for consideration in clinical practice. The literature suggests that the factors impacting the decision to undergo testing are complex due to the nature of these diseases, absence of available preventative medical treatment and variable age of onset in mutation carriers. Gaining further insight into the decision-making process and the impact of testing is critical as we seek to develop best-practice guidelines for predictive testing for familial ALS and FTD.

  8. Detailed volumetric analysis of the hypothalamus in behavioral variant frontotemporal dementia.

    Science.gov (United States)

    Bocchetta, Martina; Gordon, Elizabeth; Manning, Emily; Barnes, Josephine; Cash, David M; Espak, Miklos; Thomas, David L; Modat, Marc; Rossor, Martin N; Warren, Jason D; Ourselin, Sebastien; Frisoni, Giovanni B; Rohrer, Jonathan D

    2015-12-01

    Abnormal eating behaviors are frequently reported in behavioral variant frontotemporal dementia (bvFTD). The hypothalamus is the regulatory center for feeding and satiety but its involvement in bvFTD has not been fully clarified, partly due to its difficult identification on MR images. We measured hypothalamic volume in 18 patients with bvFTD (including 9 MAPT and 6 C9orf72 mutation carriers) and 18 cognitively normal controls using a novel optimized multimodal segmentation protocol, combining 3D T1 and T2-weighted 3T MRIs (intrarater intraclass correlation coefficients ≥0.93). The whole hypothalamus was subsequently segmented into five subunits: the anterior (superior and inferior), tuberal (superior and inferior), and posterior regions. The presence of abnormal eating behavior was assessed with the revised version of the Cambridge Behavioural Inventory (CBI-R). The bvFTD group showed a 17% lower hypothalamic volume compared with controls (p hypothalamus.

  9. Exploring frontotemporal dementia through a case report: An emerging public health concern in disguise

    Directory of Open Access Journals (Sweden)

    Nitin Khadilkar

    2015-01-01

    Full Text Available Dementia has been declared by the World Health Organization as a significant public health problem around the world. Frontotemporal dementia (FTD is a lesser known, yet the second most common type of dementia among older adults under the age of 65 years. Age of onset in FTD is around late fifties, which is not typical for a diagnosis of dementia. In dementia, it is common to see psychiatric symptoms such as hallucinations or delusions as initial presentations. However, FTD may mimic mood disorders. Unfortunately, there are no definitive treatments or ways to prevent FTD. Additionally, challenges such as an earlier age of onset, delay in diagnosis, and difficulties with placement in nursing homes may be encountered while treating FTD patients. Here, we explore FTD through the case of a 61-year-old Caucasian female who initially presented with suicidal ideations.

  10. Genetic and Clinical Features of Progranulin-Associated Frontotemporal Lobar Degeneration

    Science.gov (United States)

    Chen-Plotkin, Alice S.; Martinez-Lage, Maria; Sleiman, Patrick M. A.; Hu, William; Greene, Robert; Wood, Elisabeth McCarty; Bing, Shaoxu; Grossman, Murray; Schellenberg, Gerard D.; Hatanpaa, Kimmo J.; Weiner, Myron F.; White, Charles L.; Brooks, William S.; Halliday, Glenda M.; Kril, Jillian J.; Gearing, Marla; Beach, Thomas G.; Graff-Radford, Neill R.; Dickson, Dennis W.; Rademakers, Rosa; Boeve, Bradley F.; Pickering-Brown, Stuart M.; Snowden, Julie; van Swieten, John C.; Heutink, Peter; Seelaar, Harro; Murrell, Jill R.; Ghetti, Bernardino; Spina, Salvatore; Grafman, Jordan; Kaye, Jeffrey A.; Woltjer, Randall L.; Mesulam, Marsel; Bigio, Eileen; Lladó, Albert; Miller, Bruce L.; Alzualde, Ainhoa; Moreno, Fermin; Rohrer, Jonathan D.; Mackenzie, Ian R. A.; Feldman, Howard H.; Hamilton, Ronald L.; Cruts, Marc; Engelborghs, Sebastiaan; De Deyn, Peter P.; Van Broeckhoven, Christine; Bird, Thomas D.; Cairns, Nigel J.; Goate, Allison; Frosch, Matthew P.; Riederer, Peter F.; Bogdanovic, Nenad; Lee, Virginia M. Y.; Trojanowski, John Q.; Van Deerlin, Vivianna M.

    2011-01-01

    Objective To assess the relative frequency of unique mutations and their associated characteristics in 97 individuals with mutations in progranulin (GRN), an important cause of frontotemporal lobar degeneration (FTLD). Participants and Design A 46-site International Frontotemporal Lobar Degeneration Collaboration was formed to collect cases of FTLD with TAR DNA-binding protein of 43-kDa (TDP-43)–positive inclusions (FTLD-TDP). We identified 97 individuals with FTLD-TDP with pathogenic GRN mutations (GRN+ FTLD-TDP), assessed their genetic and clinical characteristics, and compared them with 453 patients with FTLD-TDP in which GRN mutations were excluded (GRN− FTLD-TDP). No patients were known to be related. Neuropathologic characteristics were confirmed as FTLD-TDP in 79 of the 97 GRN+ FTLDTDP cases and all of the GRN− FTLD-TDP cases. Results Age at onset of FTLD was younger in patients with GRN+ FTLD-TDP vs GRN− FTLD-TDP (median, 58.0 vs 61.0 years; PA (p.C126X). The 2 most common GRN mutations were c.1477C>T (p.R493X, found in 18 patients, representing 18.6% of GRN cases) and c.26C>A (p.A9D, found in 6 patients, representing 6.2% of cases). Patients with the c.1477C>T mutation shared a haplotype on chromosome 17; clinically, they resembled patients with other GRN mutations. Patients with the c.26C>A mutation appeared to have a younger age at onset of FTLD and at death and more parkinsonian features than those with other GRN mutations. Conclusion GRN+ FTLD-TDP differs in key features from GRN− FTLD-TDP. PMID:21482928

  11. Bilingualism delays the onset of behavioral but not aphasic forms of frontotemporal dementia.

    Science.gov (United States)

    Alladi, Suvarna; Bak, Thomas H; Shailaja, Mekala; Gollahalli, Divyaraj; Rajan, Amulya; Surampudi, Bapiraju; Hornberger, Michael; Duggirala, Vasanta; Chaudhuri, Jaydip Ray; Kaul, Subhash

    2017-05-01

    Bilingualism has been found to delay onset of dementia and this has been attributed to an advantage in executive control in bilinguals. However, the relationship between bilingualism and cognition is complex, with costs as well as benefits to language functions. To further explore the cognitive consequences of bilingualism, the study used Frontotemporal dementia (FTD) syndromes, to examine whether bilingualism modifies the age at onset of behavioral and language variants of Frontotemporal dementia (FTD) differently. Case records of 193 patients presenting with FTD (121 of them bilingual) were examined and the age at onset of the first symptoms were compared between monolinguals and bilinguals. A significant effect of bilingualism delaying the age at onset of dementia was found in behavioral variant FTD (5.7 years) but not in progressive nonfluent aphasia (0.7 years), semantic dementia (0.5 years), corticobasal syndrome (0.4 years), progressive supranuclear palsy (4.3 years) and FTD-motor neuron disease (3 years). On dividing all patients predominantly behavioral and predominantly aphasic groups, age at onset in the bilingual behavioral group (62.6) was over 6 years higher than in the monolingual patients (56.5, p=0.006), while there was no difference in the aphasic FTD group (60.9 vs. 60.6 years, p=0.851). The bilingual effect on age of bvFTD onset was shown independently of other potential confounding factors such as education, gender, occupation, and urban vs rural dwelling of subjects. To conclude, bilingualism delays the age at onset in the behavioral but not in the aphasic variants of FTD. The results are in line with similar findings based on research in stroke and with the current views of the interaction between bilingualism and cognition, pointing to advantages in executive functions and disadvantages in lexical tasks. Copyright © 2017 Elsevier Ltd. All rights reserved.

  12. No mutations in hnRNPA1 and hnRNPA2B1 in Dutch patients with amyotrophic lateral sclerosis, frontotemporal dementia, and inclusion body myopathy

    NARCIS (Netherlands)

    Seelen, Meinie; Visser, Anne E.; Overste, Daniel J.; Kim, Hong J.; Palud, A.; Wong, Tsz H.; van Swieten, John C.; Scheltens, Philip; Voermans, Nicol C.; Baas, Frank; de Jong, J. M. B. V.; van der Kooi, Anneke J.; de Visser, Marianne; Veldink, Jan H.; Taylor, J. Paul; van Es, Michael A.; van den Berg, Leonard H.

    2014-01-01

    Inclusion body myopathy (IBM) associated with Paget disease of the bone, frontotemporal dementia (FTD), and amyotrophic lateral sclerosis (ALS), sometimes called IBMPFD/ALS or multi system proteinopathy, is a rare, autosomal dominant disorder characterized by progressive degeneration of muscle,

  13. [Recurrent left atrial myxoma].

    Science.gov (United States)

    Moreno Martínez, Francisco L; Lagomasino Hidalgo, Alvaro; Mirabal Rodríguez, Roger; López Bermúdez, Félix H; López Bernal, Omaida J

    2003-01-01

    Primary cardiac tumors are rare. Mixomas are the most common among them; 75% are located in the left atrium, 20% in the right atrium, and the rest in the ventricles. The seldom appear in atrio-ventricular valves. Recidivant mixoma are also rare, appearing in 1-5% of all patients that have undergone surgical treatment of a mixoma. In this paper we present our experience with a female patient, who 8 years after having been operated of a left atrial mixoma, began with symptoms of mild heart failure. Transthoracic echocardiography revealed recurrence of the tumor, and was therefore subjected to a second open-heart surgery from which she recovered without complications.

  14. Caregiver burden, sleep quality, depression, and anxiety in dementia caregivers: a comparison of frontotemporal lobar degeneration, dementia with Lewy bodies, and Alzheimer's disease.

    Science.gov (United States)

    Liu, Shuai; Liu, Jing; Wang, Xiao-Dan; Shi, Zhihong; Zhou, Yuying; Li, Jing; Yu, Tao; Ji, Yong

    2017-12-10

    Very few recent studies are available that compare caregiver burden, sleep quality, and stress in caregivers of different types of dementia. We aimed to investigate caregiver burden, sleep quality, and stress in caregivers of patients with frontotemporal lobar degeneration and dementia with Lewy bodies, as compared with caregivers of patients with Alzheimer's disease. This study was carried out from March 2011 to January 2014. In total, 492 dyads of patient and caregiver (frontotemporal lobar degeneration, n = 131; dementia with Lewy bodies, n = 36; Alzheimer's disease, n = 325) participated in this study. We compared patients with respect to the Neuropsychiatric Inventory and caregivers with respect to the Zarit Caregiver Burden Interview, Pittsburgh Sleep Quality Index, Patient Health Questionnaire-9, and Generalized Anxiety Disorder scale. Frontotemporal lobar degeneration and dementia with Lewy bodies patients presented significantly more neuropsychiatric symptoms compared to Alzheimer's disease patients. Caregivers of frontotemporal lobar degeneration and dementia with Lewy bodies patients experienced significantly more burden compared to Alzheimer's disease caregivers. Furthermore, among caregivers of both frontotemporal lobar degeneration and dementia with Lewy bodies patients burden was predicted by the neuropsychiatric symptoms, PHQ-9 scores, and GAD-7 scores. The frequency and severity of behavioral disturbances in patient and caregiver stress accounted for the increased caregiver burden, which suggests that frontotemporal lobar degeneration and dementia with Lewy bodies caregivers should receive more support than is currently available.

  15. Hypoplastic left heart syndrome

    Directory of Open Access Journals (Sweden)

    Thiagarajan Ravi

    2007-05-01

    Full Text Available Abstract Hypoplastic left heart syndrome(HLHS refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch. Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. The treatment chosen is dependent on the preference of the institution, its experience, and also preference. Although survival following initial surgical intervention has improved significantly over the last 20 years, significant mortality and morbidity are present for both surgical strategies. As a result pediatric cardiologists continue to be challenged by discussions with families regarding initial decision

  16. Fronto-parietal and fronto-temporal theta phase synchronization for visual and auditory-verbal working memory

    OpenAIRE

    Masahiro eKawasaki; Masahiro eKawasaki; Masahiro eKawasaki; Keiichi eKitajo; Keiichi eKitajo; Yoko eYamaguchi

    2014-01-01

    In humans, theta phase (4–8 Hz) synchronization observed on electroencephalography (EEG) plays an important role in the manipulation of mental representations during working memory (WM) tasks; fronto-temporal synchronization is involved in auditory-verbal WM tasks and fronto-parietal synchronization is involved in visual WM tasks. However, whether or not theta phase synchronization is able to select the to-be-manipulated modalities is uncertain. To address the issue, we recorded EEG data from...

  17. A 43-kDa TDP-43 species is present in aggregates associated with frontotemporal lobar degeneration.

    Directory of Open Access Journals (Sweden)

    Patrick J Bosque

    Full Text Available The transactive response DNA-binding protein (TDP-43 is a major component of the abnormal intracellular inclusions that occur in two common neurodegenerative diseases of humans: (1 a subtype of frontotemporal lobar degeneration and (2 amyotrophic lateral sclerosis. Genetics, experiments in cultured cells and animals, and analogy with other neurodegenerative diseases indicate that the process of TDP-43 aggregation is fundamental to the pathogenesis of these 2 diseases, but the process by which this aggregation occurs is not understood. Biochemical fractionation has revealed truncated, phosphorylated and ubiquitinated forms of TDP-43 in a detergent-insoluble fraction from diseased CNS tissue, while these forms are absent from controls. However, a large amount of the normally predominant 43-kDa form of TDP-43 is present in the detergent-insoluble fraction even from control brains, so it has not been possible to determine if this form of TDP-43 is part of pathological aggregates in frontotemporal lobe degeneration. We used semi-denaturing detergent-agarose gel electrophoresis to isolate high molecular weight aggregates containing TDP-43 that are present in the cerebral cortex of individuals with frontotemporal lobar degeneration but not that of controls. These aggregates include the same covalently modified forms of TDP-43 seen in detergent-insoluble extracts. In addition, aggregates include a 43-kDa TDP-43 species. This aggregated 43-kDa form of TDP-43 is absent or present only at low levels in controls. The presence of 43-kDa TDP-43 in aggregates raises the possibility that covalent modification is not a primary step in the pathogenic aggregation of TDP-43 associated with frontotemporal lobar degeneration and amyotrophic lateral sclerosis.

  18. Left Ventricular Assist Devices

    Directory of Open Access Journals (Sweden)

    Khuansiri Narajeenron

    2017-04-01

    Full Text Available Audience: The audience for this classic team-based learning (cTBL session is emergency medicine residents, faculty, and students; although this topic is applicable to internal medicine and family medicine residents. Introduction: A left ventricular assist device (LVAD is a mechanical circulatory support device that can be placed in critically-ill patients who have poor left ventricular function. After LVAD implantation, patients have improved quality of life.1 The number of LVAD patients worldwide continues to rise. Left-ventricular assist device patients may present to the emergency department (ED with severe, life-threatening conditions. It is essential that emergency physicians have a good understanding of LVADs and their complications. Objectives: Upon completion of this cTBL module, the learner will be able to: 1 Properly assess LVAD patients’ circulatory status; 2 appropriately resuscitate LVAD patients; 3 identify common LVAD complications; 4 evaluate and appropriately manage patients with LVAD malfunctions. Method: The method for this didactic session is cTBL.

  19. The 5-HTTLPR variant in the serotonin transporter gene modifies degeneration of brain regions important for emotion in behavioral variant frontotemporal dementia

    Directory of Open Access Journals (Sweden)

    Jennifer S. Yokoyama

    2015-01-01

    Full Text Available The serotonin transporter length polymorphism (5-HTTLPR short allele (5-HTTLPR-s has been associated with differential susceptibility for anxiety and depression in multiple psychiatric disorders. 5-HTTLPR-s modifies the serotonergic systems that support emotion and behavioral regulation by reducing gene expression, which slows the reuptake of serotonin, and is associated with distinct morphological and functional effects. Serotonergic systems are also shown to be dysfunctional in behavioral variant frontotemporal dementia (bvFTD, a disease characterized by marked socioemotional dysfunction. However, studies of 5-HTTLPR-s effects in bvFTD have been inconsistent. Our objective was to investigate the patterns of gray matter volume by 5-HTTLPR-s genotype in both healthy older controls and bvFTD patients. We performed voxel-based morphometry of 179 cognitively normal older adults and 24 bvFTD cases to determine brain changes associated with dose (0/1/2 of 5-HTTLPR-s allele. 5-HTTLPR-s frequency did not differ between controls and bvFTD. We found a significant interaction effect whereby carrying more 5-HTTLPR-s alleles in bvFTD was associated with smaller volume in left inferior frontal gyrus (T = 4.86, PFWE = 0.03 and larger volume in right temporal lobe (T = 5.01, PFWE = 0.01. These results suggest that the 5-HTTLPR-s allele differentially influences brain morphology in bvFTD. We propose that patients with bvFTD and 5-HTTLPR-s have altered volumes in regions that support socioemotional behavior, which may be a developmental or disease-related compensation for altered serotonergic activity.

  20. Social and cognitive control skills in long-life occupation activities modulate the brain reserve in the behavioural variant of frontotemporal dementia.

    Science.gov (United States)

    Dodich, Alessandra; Carli, Giulia; Cerami, Chiara; Iannaccone, Sandro; Magnani, Giuseppe; Perani, Daniela

    2018-02-01

    Cognitive reserve may delay disease onset and mitigate symptoms presentation in neurodegenerative dementias. Although high occupation levels can be associated with higher cognitive reserve in the behavioural variant of frontotemporal dementia (bvFTD), it was never addressed how specific occupation profiles involving social interaction, executive and attention abilities can modulate neural reserve in bvFTD. We retrospectively included thirty-seven bvFTD patients with clinical-neuropsychological and FDG-PET brain metabolic data. We considered occupation levels according to 1) a 5-point scale and 2) the specific cognitive dimensions from the O*Net network database. We used the Principal Component Analysis (PCA) with the O*Net variables most representative of "worker" and "occupation" socio-cognitive skills to merge the best components describing such occupation profiles. We then performed regression analyses with brain metabolism using either 5-level occupation scale or the PCA specific profiles as independent variables, controlling for education and disease severity. According to the brain reserve hypothesis, higher occupation levels were associated with a more severe hypometabolism in the dorsolateral prefrontal cortex. In addition, among the identified PCA profiles, social skills were associated with severe hypometabolism in medial and dorsolateral prefrontal regions, and cognitive control in the left fronto-insular cortex. This study contributes to define the role of specific occupation profiles as proxy of cognitive reserve in bvFTD, providing the first evidence for social interaction and cognitive control skills in life-occupation activities as influencing factors of neural reserve against neurodegeneration in bvFTD. Jobs placing high demand on such abilities seem to act as protective factors in bvFTD. Copyright © 2017 Elsevier Ltd. All rights reserved.

  1. Effects of 17-allylamino-17-demethoxygeldanamycin (17-AAG) in transgenic mouse models of frontotemporal lobar degeneration and Alzheimer’s disease

    Science.gov (United States)

    2013-01-01

    Alzheimer’s disease (AD), the most common dementia, is characterized by potentially neurotoxic aggregation of Aβ peptide and tau protein, and their deposition as amyloid plaques and neurofibrillary tangles (NFTs). Tau aggregation also occurs in other common neurodegenerative diseases. Frontotemporal dementia (FTD) can be caused by tau mutations that increase the susceptibility of tau to hyperphosphorylation and aggregation, which may cause neuronal dysfunction and deposition of NFTs. 17-allylamino-17-demethoxygeldanamycin (17-AAG) is a potent inhibitor of heat shock protein 90 (Hsp90), a cytosolic chaperone implicated in the proper folding and functions of a repertoire of client proteins. 17-AAG binds to Hsp90 and enhances degradation of Hsp90 client protein. We sought to determine whether 17-AAG can reduce Aβ and tau pathology in the brains of AD and FTD model mice expressing Aβ or P301L mutant tau, respectively. Mice were randomized to receive 25, 5, or 0 mg/kg 17-AAG thrice weekly from age eight to 11 months. Analysis was performed by rotarod test on motor function, on the area occupied by plaques in hippocampus or NFTs in medulla tissue sections, and on mortality. A high dose of 17-AAG tended to decrease NFTs in male mice (p = 0.08). Further studies are required to confirm the effect of 17-AAG in diseases of tau aggregation. PMID:24344631

  2. Left Ventricular Pseudoaneurysm Perceived as a Left Lung Mass

    Directory of Open Access Journals (Sweden)

    Ugur Gocen

    2013-02-01

    Full Text Available Left ventricular pseudo-aneurysm is a rare complication of aneurysmectomy. We present a case of surgically-treated left ventricular pseudo-aneurysm which was diagnosed three years after coronary artery bypass grafting and left ventricular aneurysmectomy. The presenting symptoms, diagnostic evaluation and surgical repair are described. [Cukurova Med J 2013; 38(1.000: 123-125

  3. The established and emerging roles of astrocytes and microglia in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.

    Directory of Open Access Journals (Sweden)

    Rowan Andrew Warren Radford

    2015-10-01

    Full Text Available Amyotrophic lateral sclerosis (ALS and Frontotemporal Dementia (FTD are two progressive, fatal neurodegenerative syndromes with considerable clinical, genetic and pathological overlap. Clinical symptoms of FTD can be seen in ALS patients and vice versa, recent genetic discoveries conclusive link the two diseases, and several common molecular players have been identified (TDP-43, FUS, C9ORF72.The definitive aetiologies of ALS and FTD are currently unknown and both disorders lack a cure. Glia, specifically astrocytes and microglia are heavily implicated in the onset and progression of neurodegeneration witnessed in ALS and FTD. In this review, we summarise the current understanding of the role of microglia and astrocytes involved in ALS and FTD, highlighting their recent implications in neuroinflammation, alterations in waste clearance involving phagocytosis and the newly described glymphatic system, and vascular abnormalities. Elucidating the precise mechanisms of how astrocytes and microglia are involved in ALS and FTD will be crucial in characterising these two disorders and may represent more effective interventions for disease progression and treatment options in the future.

  4. Distinct perfusion patterns in Alzheimer's disease, frontotemporal dementia and dementia with Lewy bodies

    International Nuclear Information System (INIS)

    Binnewijzend, Maja A.A.; Wattjes, Mike P.; Berckel, Bart N.M. van; Barkhof, Frederik; Kuijer, Joost P.A.; Flier, Wiesje M. van der; Benedictus, Marije R.; Moeller, Christiane M.; Pijnenburg, Yolande A.L.; Lemstra, Afina W.; Prins, Niels D.; Scheltens, Philip

    2014-01-01

    To compare pseudo-continuous arterial spin-labelled (PCASL) magnetic resonance imaging (MRI) measured quantitative cerebral blood flow (CBF) of patients with frontotemporal dementia (FTD), dementia with Lewy Bodies (DLB), Alzheimer's disease (AD) and controls, in a region of interest (ROI) and voxel-wise fashion. We analysed whole-brain 3D fast-spin-echo PCASL images of 20 FTD patients, 14 DLB patients, 48 AD patients and 50 controls from the Amsterdam Dementia Cohort. Regional CBF patterns were compared using analyses of variance for repeated measures. Permutation tests were used for voxel-wise comparisons. Analyses were performed using uncorrected and partial volume corrected (PVC) maps. All analyses were corrected for age and sex. There was an interaction between diagnosis and region (p < 0.001), implying differences in regional CBF changes between diagnostic groups. In AD patients, CBF was decreased in all supratentorial regions, most prominently so in the posterior regions. DLB patients showed lowest CBF values throughout the brain, but temporal CBF was preserved. Supratentorial PVC cortical CBF values were lowest in the frontal lobes in FTD patients, and in the temporal lobes in AD patients. Patients with AD, FTD and DLB display distinct patterns of quantitative regional CBF changes. 3D-PCASL may provide additional value in the workup of dementia patients. (orig.)

  5. Mutation screening of the MAPT and STH genes in Polish patients with clinically diagnosed frontotemporal dementia.

    Science.gov (United States)

    Zekanowski, Cezary; Pepłońska, Beata; Styczyńska, Maria; Gustaw, Katarzyna; Kuźnicki, Jacek; Barcikowska, Maria

    2003-01-01

    Frontotemporal dementia (FTD) is a common neurodegenerative disorder and is connected with about 10% of all dementias. In approximately half of all FTD cases, a positive family history has been reported. To date, several mutations at the tau protein gene (MAPT) were identified causing familial and sporadic FTD. Extensive polymorphic variability at the MAPT gene has also been shown to be a risk factor in progressive supranuclear palsy (PSP). The recently described gene Saitohin (STH), located in the intron 9 of MAPT gene, was also reported to be polymorphic. In the present study 23 unrelated Polish patients with clinically defined sporadic and familial FTD were screened for mutations at the MAPT gene. No pathogenic mutations were found in the group. Several novel silent intronic and exonic mutations were identified, most of them associated with two common haplotypes. In the reported group no correlation between extended MAPT haplotype and APOE genotype was determined. There was also no observed relation between age of onset and APOE status. At the STH gene only a common polymorphic change was found. It is postulated that MAPT mutations are not connected with most of the FTD cases in the Polish population. Copyright 2003 S. Karger AG, Basel

  6. EEG microstates associated with salience and frontoparietal networks in frontotemporal dementia, schizophrenia and Alzheimer's disease.

    Science.gov (United States)

    Nishida, Keiichiro; Morishima, Yosuke; Yoshimura, Masafumi; Isotani, Toshiaki; Irisawa, Satoshi; Jann, Kay; Dierks, Thomas; Strik, Werner; Kinoshita, Toshihiko; Koenig, Thomas

    2013-06-01

    There are relevant links between resting-state fMRI networks, EEG microstate classes and psychopathological alterations in mental disorders associated with frontal lobe dysfunction. We hypothesized that a certain microstate class, labeled C and correlated with the salience network, was impaired early in frontotemporal dementia (FTD), and that microstate class D, correlated with the frontoparietal network, was impaired in schizophrenia. We measured resting EEG microstate parameters in patients with mild FTD (n = 18), schizophrenia (n = 20), mild Alzheimer's disease (AD; n = 19) and age-matched controls (old n = 19, young n = 18) to investigate neuronal dynamics at the whole-brain level. The duration of class C was significantly shorter in FTD than in controls and AD, and the duration of class D was significantly shorter in schizophrenia than in controls, FTD and AD. Transition analysis showed a reversed sequence of activation of classes C and D in FTD and schizophrenia patients compared with that in controls, with controls preferring transitions from C to D, and patients preferring D to C. The duration and sequence of EEG microstates reflect specific aberrations of frontal lobe functions in FTD and schizophrenia. This study highlights the importance of subsecond brain dynamics for understanding of psychiatric disorders. Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  7. Prion disease resembling frontotemporal dementia and parkinsonism linked to chromosome 17

    Directory of Open Access Journals (Sweden)

    Nitrini Ricardo

    2001-01-01

    Full Text Available OBJECTIVE: To compare the clinical features of a familial prion disease with those of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17. BACKGROUND: Prion diseases are not usually considered in the differential diagnosis of FTDP-17, since familial Creutzfeldt-Jakob disease (CJD, the most common inherited prion disease, often manifests as a rapidly progressive dementia. Conversely, FTDP-17 usually has an insidious onset in the fifth decade, with abnormal behavior and parkinsonian features. METHOD: We present the clinical features of 12 patients from a family with CJD associated with a point mutation at codon 183 of the prion protein gene. RESULTS: The mean age at onset was 44.0 ± 3.7; the duration of the symptoms until death ranged from two to nine years. Behavioral disturbances were the predominant presenting symptoms. Nine patients were first seen by psychiatrists. Eight patients manifested parkinsonian signs. CONCLUSION: These clinical features bear a considerable resemblance to those described in FTDP-17.

  8. Functional connectivity and microstructural white matter changes in phenocopy frontotemporal dementia

    Energy Technology Data Exchange (ETDEWEB)

    Meijboom, R.; Steketee, R.M.E.; Lugt, A. van der; Smits, M. [Erasmus MC - University Medical Centre, Radiology and Nuclear Medicine, Rotterdam (Netherlands); Koning, I. de [Erasmus MC - University Medical Centre, Neuropsychology, Rotterdam (Netherlands); Osse, R.J. [Erasmus MC - University Medical Centre, Psychiatry, Rotterdam (Netherlands); Jiskoot, L.C. [Erasmus MC - University Medical Centre, Neuropsychology, Rotterdam (Netherlands); Erasmus MC - University Medical Centre, Neurology, Rotterdam (Netherlands); Jong, F.J. de; Swieten, J.C. van [Erasmus MC - University Medical Centre, Neurology, Rotterdam (Netherlands)

    2017-04-15

    Phenocopy frontotemporal dementia (phFTD) is a rare and poorly understood clinical syndrome. PhFTD shows core behavioural variant FTD (bvFTD) symptoms without associated cognitive deficits and brain abnormalities on conventional MRI and without progression. In contrast to phFTD, functional connectivity and white matter (WM) microstructural abnormalities have been observed in bvFTD. We hypothesise that phFTD belongs to the same disease spectrum as bvFTD and investigated whether functional connectivity and microstructural WM changes similar to bvFTD are present in phFTD. Seven phFTD patients without progression or alternative psychiatric diagnosis, 12 bvFTD patients and 17 controls underwent resting state functional MRI (rs-fMRI) and diffusion tensor imaging (DTI). Default mode network (DMN) connectivity and WM measures were compared between groups. PhFTD showed subtly increased DMN connectivity and subtle microstructural changes in frontal WM tracts. BvFTD showed abnormalities in similar regions as phFTD, but had lower increased DMN connectivity and more extensive microstructural WM changes. Our findings can be interpreted as neuropathological changes in phFTD and are in support of the hypothesis that phFTD and bvFTD may belong to the same disease spectrum. Advanced MRI techniques, objectively identifying brain abnormalities, would therefore be potentially suited to improve the diagnosis of phFTD. (orig.)

  9. Neuropsychiatric Symptoms, Caregiver Burden and Distress in Behavioral-Variant Frontotemporal Dementia and Alzheimer's Disease.

    Science.gov (United States)

    Lima-Silva, Thais Bento; Bahia, Valéria Santoro; Carvalho, Viviane Amaral; Guimarães, Henrique Cerqueira; Caramelli, Paulo; Balthazar, Márcio Luiz; Damasceno, Benito; Bottino, Cássio Machado; Brucki, Sônia Maria; Nitrini, Ricardo; Yassuda, Mônica Sanches

    2015-01-01

    We aimed to compare caregiver burden and distress in behavioral-variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD) and to investigate which factors contribute to caregivers' burden and distress. Fifty patients and their caregivers were invited to participate. Among the patients, 20 had a diagnosis of bvFTD and 30 had AD. Caregivers and patients were statistically equivalent for age, sex, education and dementia severity according to Clinical Dementia Rating. The protocol included the Short Zarit Burden Inventory, the Neuropsychiatric Inventory (NPI), Disability Assessment for Dementia (DAD), the Cornell Scale for Depression in Dementia (CSDD), Addenbrooke's Cognitive Examination-Revised, the Executive Interview with 25 Items, Direct Assessment of Functional Status and the Geriatric Anxiety Inventory (GAI). In the NPI, caregivers of bvFTD patients reported a higher presence and severity of neuropsychiatric symptoms and caregiver distress compared to caregivers of AD patients. There was no significant difference in the perceived burden. In bvFTD, DAD and GAI scores were significantly correlated with burden, whereas in AD, burden was correlated with CSDD and NPI scores. Psychiatric symptoms were associated with distress in both groups. Caregivers of bvFTD patients experienced higher levels of distress than caregivers of AD patients. Patients' functional limitations were associated with burden of caregivers of bvFTD patients, whereas neuropsychiatric symptoms were associated with caregiver strain in both groups. © 2015 S. Karger AG, Basel.

  10. Screening for the C9ORF72 repeat expansion in a greek frontotemporal dementia cohort.

    Science.gov (United States)

    Kartanou, Chrisoula; Karadima, Georgia; Koutsis, Georgios; Breza, Marianthi; Papageorgiou, Sokratis G; Paraskevas, George P; Kapaki, Elisabeth; Panas, Marios

    2018-02-01

    The C9orf72 repeat expansion is a common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) in European populations. A previous study has reported a high frequency of the expansion in Greek ALS. However, no data have been reported on the frequency of the expansion in Greek FTD. Currently, we investigated the frequency of the C9orfF72 expansion in a well-characterized cohort of 64 Greek FTD patients. We detected the C9orf72 repeat expansion in 9.3% of cases. Overall, 27.7% of familial and 2.2% of sporadic cases were expansion-positive. Five out of 6 cases had a diagnosis of behavioral variant FTD. All expansion-positive cases had fairly typical FTD presentations. Clinical features included motor neuron disease, Parkinsonism and hallucinations. We conclude that the overall frequency of C9orf72-positive cases in Greek FTD is high, comparable to Greek ALS, similar to some Western European, but significantly higher than some Mediterranean FTD populations.

  11. ADNP: In search for molecular mechanisms and innovative therapeutic strategies for frontotemporal degeneration

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    Illana eGozes

    2015-10-01

    Full Text Available Activity-dependent neuroprotective protein (ADNP is deregulated in Alzheimer’s disease (AD and in schizophrenia and mutated in autism. In mice, ADNP is essential for brain formation and ADNP haploinsufficiency is associated with cognitive and social deficits and tauopathy. Tauopathy, a major pathology in AD, is also found in ~45% of frontotemporal dementias (FTDs. Tau transcript, a product of a single gene, undergoes alternative splicing. Tau splicing seems to be altered in FTD brain. In transgenic mice overexpressing a mutated tau in the cerebral cortex, significant increases in ADNP transcript expression were observed in the cerebral cortex of young transgenic mice (~disease onset and a marked decrease with aging as compared to control littermates. ADNP is a member of the SWItch/Sucrose NonFermentable (SWI/SNF chromatin remodeling complex also associated with alternative splicing, including tau transcript splicing. Further cellular interactions of ADNP include association with microtubules, with tau being a microtubule – associated protein. NAP (davundetide, a novel drug candidate derived from ADNP, increases ADNP-microtubule association and protects against tauopathy and cognitive deficiencies in mice. Although, NAP did not provide protection in progressive supranuclear palsy (PSP, a pure tauopathy, it increased cognitive scores in amnestic mild cognitively impaired patients and protected functional activity in schizophrenia patients. This mini-review focuses on ADNP in the context of FTD and tau/microtubules and proposes NAP as a novel drug target for future clinical evaluations.

  12. FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration.

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    Cao Huang

    2011-03-01

    Full Text Available Fused in Sarcoma (FUS proteinopathy is a feature of frontotemporal lobar dementia (FTLD, and mutation of the fus gene segregates with FTLD and amyotrophic lateral sclerosis (ALS. To study the consequences of mutation in the fus gene, we created transgenic rats expressing the human fus gene with or without mutation. Overexpression of a mutant (R521C substitution, but not normal, human FUS induced progressive paralysis resembling ALS. Mutant FUS transgenic rats developed progressive paralysis secondary to degeneration of motor axons and displayed a substantial loss of neurons in the cortex and hippocampus. This neuronal loss was accompanied by ubiquitin aggregation and glial reaction. While transgenic rats that overexpressed the wild-type human FUS were asymptomatic at young ages, they showed a deficit in spatial learning and memory and a significant loss of cortical and hippocampal neurons at advanced ages. These results suggest that mutant FUS is more toxic to neurons than normal FUS and that increased expression of normal FUS is sufficient to induce neuron death. Our FUS transgenic rats reproduced some phenotypes of ALS and FTLD and will provide a useful model for mechanistic studies of FUS-related diseases.

  13. Cognitive reserve in granulin-related frontotemporal dementia: from preclinical to clinical stages.

    Science.gov (United States)

    Premi, Enrico; Gazzina, Stefano; Bozzali, Marco; Archetti, Silvana; Alberici, Antonella; Cercignani, Mara; Bianchetti, Angelo; Gasparotti, Roberto; Turla, Marinella; Caltagirone, Carlo; Padovani, Alessandro; Borroni, Barbara

    2013-01-01

    Consistent with the cognitive reserve hypothesis, higher education and occupation attainments may help persons with neurodegenerative dementias to better withstand neuropathology before developing cognitive impairment. We tested here the cognitive reserve hypothesis in patients with frontotemporal dementia (FTD), with or without pathogenetic granulin mutations (GRN+ and GRN-), and in presymptomatic GRN mutation carriers (aGRN+). Education and occupation attainments were assessed and combined to define Reserve Index (RI) in 32 FTD patients, i.e. 12 GRN+ and 20 GRN-, and in 17 aGRN+. Changes in functional connectivity were estimated by resting state fMRI, focusing on the salience network (SN), executive network (EN) and bilateral frontoparietal networks (FPNs). Cognitive status was measured by FTD-modified Clinical Dementia Rating Scale. In FTD patients higher level of premorbid cognitive reserve was associated with reduced connectivity within the SN and the EN. EN was more involved in FTD patients without GRN mutations, while SN was more affected in GRN pathology. In aGRN+, cognitive reserve was associated with reduced SN. This study suggests that cognitive reserve modulates functional connectivity in patients with FTD, even in monogenic disease. In GRN inherited FTD, cognitive reserve mechanisms operate even in presymptomatic to clinical stages.

  14. FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration

    DEFF Research Database (Denmark)

    Urwin, Hazel; Josephs, Keith A; Rohrer, Jonathan D

    2010-01-01

    % (34/37) had fused in sarcoma (FUS) protein pathology, indicating that FTLD-FUS is an important FTLD subtype. This FTLD-FUS collection specifically focussed on aFTLD-U cases, one of three recently defined subtypes of FTLD-FUS. The aFTLD-U subtype of FTLD-FUS is characterised clinically by behavioural...... variant frontotemporal dementia (bvFTD) and has a particularly young age of onset with a mean of 41 years. Further, this subtype had a high prevalence of psychotic symptoms (36% of cases) and low prevalence of motor symptoms (3% of cases). We did not find FUS mutations in any aFTLD-U case. To date......, the only subtype of cases reported to have ubiquitin-positive but tau-, TDP-43- and FUS-negative pathology, termed FTLD-UPS, is the result of charged multivesicular body protein 2B gene (CHMP2B) mutation. We identified three FTLD-UPS cases, which are negative for CHMP2B mutation, suggesting that the full...

  15. Faulty Suppression of Irrelevant Material in Patients with Thought Disorder Linked to Attenuated Frontotemporal Activation

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    S. M. Arcuri

    2012-01-01

    Full Text Available Formal thought disorder is a feature schizophrenia that manifests as disorganized, incoherent speech, and is associated with a poor clinical outcome. The neurocognitive basis of this symptom is unclear but it is thought to involve an impairment in semantic processing classically described as a loosening of meaningful associations. Using a paradigm derived from the n400 event-related, potential, we examined the extent to which regional activation during semantic processing is altered in schizophrenic patients with formal thought disorder. Ten healthy control and 18 schizophrenic participants (9 with and 9 without formal thought disorder performed a semantic decision sentence task during an event-related functional magnetic resonance imaging experiment. We employed analysis of variance to estimate the main effects of semantic congruency and groups on activation and specific effects of formal thought disorder were addressed using post-hoc comparisons. We found that the frontotemporal network, normally engaged by a semantic decision task, was underactivated in schizophrenia, particularly in patients with FTD. This network is implicated in the inhibition of automatically primed stimuli and impairment of its function interferes with language processing and contributes to the production of incoherent speech.

  16. Fronto-striatal atrophy correlates of neuropsychiatric dysfunction in frontotemporal dementia (FTD and Alzheimer's disease (AD

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    Dong Seok Yi

    Full Text Available ABSTRACT Behavioural disturbances in frontotemporal dementia (FTD are thought to reflect mainly atrophy of cortical regions. Recent studies suggest that subcortical brain regions, in particular the striatum, are also significantly affected and this pathology might play a role in the generation of behavioural symptoms. Objective: To investigate prefrontal cortical and striatal atrophy contributions to behavioural symptoms in FTD. Methods: One hundred and eighty-two participants (87 FTD patients, 39 AD patients and 56 controls were included. Behavioural profiles were established using the Cambridge Behavioural Inventory Revised (CBI-R and Frontal System Behaviour Scale (FrSBe. Atrophy in prefrontal (VMPFC, DLPFC and striatal (caudate, putamen regions was established via a 5-point visual rating scale of the MRI scans. Behavioural scores were correlated with atrophy rating scores. Results: Behavioural and atrophy ratings demonstrated that patients were significantly impaired compared to controls, with bvFTD being most severely affected. Behavioural-anatomical correlations revealed that VMPFC atrophy was closely related to abnormal behaviour and motivation disturbances. Stereotypical behaviours were associated with both VMPFC and striatal atrophy. By contrast, disturbance of eating was found to be related to striatal atrophy only. Conclusion: Frontal and striatal atrophy contributed to the behavioural disturbances seen in FTD, with some behaviours related to frontal, striatal or combined fronto-striatal pathology. Consideration of striatal contributions to the generation of behavioural disturbances should be taken into account when assessing patients with potential FTD.

  17. Automatic MRI Quantifying Methods in Behavioral-Variant Frontotemporal Dementia Diagnosis

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    Antti Cajanus

    2018-02-01

    Full Text Available Aims: We assessed the value of automated MRI quantification methods in the differential diagnosis of behavioral-variant frontotemporal dementia (bvFTD from Alzheimer disease (AD, Lewy body dementia (LBD, and subjective memory complaints (SMC. We also examined the role of the C9ORF72-related genetic status in the differentiation sensitivity. Methods: The MRI scans of 50 patients with bvFTD (17 C9ORF72 expansion carriers were analyzed using 6 quantification methods as follows: voxel-based morphometry (VBM, tensor-based morphometry, volumetry (VOL, manifold learning, grading, and white-matter hyperintensities. Each patient was then individually compared to an independent reference group in order to attain diagnostic suggestions. Results: Only VBM and VOL showed utility in correctly identifying bvFTD from our set of data. The overall classification sensitivity of bvFTD with VOL + VBM achieved a total sensitivity of 60%. Using VOL + VBM, 32% were misclassified as having LBD. There was a trend of higher values for classification sensitivity of the C9ORF72 expansion carriers than noncarriers. Conclusion: VOL, VBM, and their combination are effective in differential diagnostics between bvFTD and AD or SMC. However, MRI atrophy profiles for bvFTD and LBD are too similar for a reliable differentiation with the quantification methods tested in this study.

  18. Olfactory deficits in frontotemporal dementia as measured by the Alberta Smell Test.

    Science.gov (United States)

    Heyanka, Daniel J; Golden, Charles J; McCue, Robert B; Scarisbrick, David M; Linck, John F; Zlatkin, Nancy I

    2014-01-01

    The study of olfaction in neurodegeneration has primarily focused on Alzheimer's disease. Research of olfaction in frontotemporal dementia (FTD) has generally not been empirically studied. The current study compared olfaction in FTD to major depressive disorder (MDD) using the Alberta Smell Test (AST). Independent-samples t test results suggested olfaction in FTD was impaired when compared with participants diagnosed with MDD. The AST Total score (out of 20 trials) significantly predicted the diagnostic group and accounted for 40% of the variance in diagnostic group status with an odds ratio of 20.08. Results suggested that a cutoff of ≤2/20 differentiated FTD from MDD with 94% accuracy (91% sensitivity, 97% specificity) and a cutoff of ≤1/20 differentiated the groups with a 95.5% hit rate (91% sensitivity, 100% specificity). Results confirmed olfactory identification deficits in FTD and suggested that the AST is an effective tool for the demarcation of FTD from MDD. This is especially important due to the potential for significant overlap in the behavioral/emotional phenotype and cognitive deficits between the two disorders when presented with early stages of FTD.

  19. Differentiating Subtypes of Apathy to Improve Person-Centered Care in Frontotemporal Degeneration

    Science.gov (United States)

    Massimo, Lauren; Evans, Lois K.; Grossman, Murray

    2014-01-01

    Apathy, a reduction in goal-directed behavior (GDB), affects 90% of individuals with behavioral variant frontotemporal degeneration, which is a common cause of early onset neurodegenerative disease. The cognitive and neural impairments associated with apathy make it difficult to initiate, plan, and self-motivate activities toward a specific goal, such as dressing or bathing. These impairments are associated with significant decline in functional ability, caregiver burden, and increased cost of care due to early institutionalization. The current article reviews the evidence suggesting that apathy arises from the interruption of one or any combination of three GDB processes: initiation, planning, and motivation. From this perspective, three subtypes of apathy related to dysfunction at the level of GDB and the corresponding neuroanatomy are explored. Further research is required to confirm and measure these subtypes of apathy for use in clinical and research settings. A more precise classification of apathy by subtype will allow implementation of the most appropriate person-centered, individualized therapy. PMID:25199154

  20. Different Apathy Profile in Behavioral Variant of Frontotemporal Dementia and Alzheimer's Disease: A Preliminary Investigation

    Directory of Open Access Journals (Sweden)

    Davide Quaranta

    2012-01-01

    Full Text Available Apathy is one of the most common behavioral symptoms of dementia; it is one of the salient features of behavioral variant of frontotemporal dementia (bvFTD but is also very frequent in Alzheimer's disease. This preliminary investigation was aimed at assessing the type of apathy-related symptoms in a population of bvFTD and AD subjects showing comparable apathy severity. Each patient underwent a comprehensive neuropsychological assessment; behavioral changes were investigated by the neuropsychiatric inventory (NPI, using the NPI-apathy subscale to detect apathetic symptoms. At univariate analysis, bvFTD subjects showed lack of initiation (χ2=4.602, p=0.032, reduced emotional output (χ2=6.493, p=0.008, and reduced interest toward friends and family members (χ2=4.898, p=0.027, more frequently than AD subjects. BvFTD displayed higher scores than AD on NPI total score (p=0.005 and on subscales assessing agitation (p=0.004, disinhibition (p=0.007 and sleep disturbances (p=0.025; conversely, AD subjects were more impaired on memory, constructional abilities, and attention. On multivariate logistic regression, reduced emotional output was highly predictive of bvFTD (OR=18.266; p=0.008. Our preliminary findings support the hypothesis that apathy is a complex phenomenon, whose clinical expression is conditioned by the site of anatomical damage. Furthermore, apathy profile may help in differentiating bvFTD from AD.

  1. Early cranioplasty benefits patients with obvious bilateral frontotemporal bone window collapse after decompressive craniectomy.

    Science.gov (United States)

    Zhu, Haifeng; Ji, Chengfu; Shen, Zhouming; Luo, Zhengxiang; Shi, Lei

    2018-02-23

    Obvious skin flap collapse is often accompanied by reduced neurological recovery after decompressive craniectomy. This study explored the feasibility of early cranioplasty (EC) in patients with obvious bilateral frontotemporal bone window (BFBW) collapse after decompressive craniectomy. Patients with obvious BFBW collapse who underwent EC or traditional cranioplasty (TC) were divided into three groups according to their preoperative Glasgow Coma Scale (GCS) scores. The indexes including postoperative incision healing, salivation symptoms, postoperative infection, and 6-month postoperative follow-up after EC or TC were compared in each group. Two of 32 patients with GCS scores of 3-8 points exhibited poor healing of the scalp incision following EC, whereas no TC patients had poor healing. Incision healing significantly differed between these two groups (P>0.05), and long-term prognoses based on GOS scores were the same after a 6-month postoperative follow-up (P>0.05). In patients with GCS scores of 9-12 points, salivation improved by 84.2% and 17.6% in the EC and TC groups, respectively (P0.05). However, salivation improved by 86.7% in the EC group but by only by 12.5% in the TC group (P<0.05). We therefore recommend EC for patients with obvious BFBW collapse and GCS scores ≥9. Copyright © 2018 Elsevier Inc. All rights reserved.

  2. Methylphenidate ('Ritalin') can ameliorate abnormal risk-taking behavior in the frontal variant of frontotemporal dementia.

    Science.gov (United States)

    Rahman, Shibley; Robbins, Trevor W; Hodges, John R; Mehta, Mitul A; Nestor, Peter J; Clark, Luke; Sahakian, Barbara J

    2006-03-01

    The frontal variant of frontotemporal dementia is a significant neurological condition worldwide. There exist few treatments available for the cognitive and behavioural sequelae of fvFTD. Previous research has shown that these patients display risky decision-making, and numerous studies have now demonstrated pathology affecting the orbitofrontal cortex. The present study uses a within-subjects, double-blind, placebo-controlled procedure to investigate the effects of a single dose of methylphenidate (40 mg) upon a range of different cognitive processes including those assessing prefrontal cortex integrity. Methylphenidate was effective in 'normalizing' the decision-making behavior of patients, such that they became less risk taking on medication, although there were no significant effects on other aspects of cognitive function, including working memory, attentional set shifting, and reversal learning. Moreover, there was an absence of the normal subjective and autonomic responses to methylphenidate seen in elderly subjects. The results are discussed in terms of the 'somatic marker' hypothesis of impaired decision-making following orbitofrontal dysfunction.

  3. Longitudinal grey and white matter changes in frontotemporal dementia and Alzheimer's disease.

    Directory of Open Access Journals (Sweden)

    Lars Frings

    Full Text Available Behavioural variant frontotemporal dementia (bvFTD and Alzheimer's disease (AD dementia are characterised by progressive brain atrophy. Longitudinal MRI volumetry may help to characterise ongoing structural degeneration and support the differential diagnosis of dementia subtypes. Automated, observer-independent atlas-based MRI volumetry was applied to analyse 102 MRI data sets from 15 bvFTD, 14 AD, and 10 healthy elderly control participants with consecutive scans over at least 12 months. Anatomically defined targets were chosen a priori as brain structures of interest. Groups were compared regarding volumes at clinic presentation and annual change rates. Baseline volumes, especially of grey matter compartments, were significantly reduced in bvFTD and AD patients. Grey matter volumes of the caudate and the gyrus rectus were significantly smaller in bvFTD than AD. The bvFTD group could be separated from AD on the basis of caudate volume with high accuracy (79% cases correct. Annual volume decline was markedly larger in bvFTD and AD than controls, predominantly in white matter of temporal structures. Decline in grey matter volume of the lateral orbitofrontal gyrus separated bvFTD from AD and controls. Automated longitudinal MRI volumetry discriminates bvFTD from AD. In particular, greater reduction of orbitofrontal grey matter and temporal white matter structures after 12 months is indicative of bvFTD.

  4. Agreement among neuropsychological and behavioral data and PiB findings in diagnosing Frontotemporal Dementia.

    Science.gov (United States)

    Ryan, Kelly A; Hammers, Dustin; DeLeon, Angeline; Bilen, Hande; Frey, Kirk; Burke, James; Albin, Roger; Barbas, Nancy; Heidebrink, Judith; Giordani, Bruno

    2017-10-01

    Diagnostic inaccuracies have been reported in Alzheimer's disease (AD) and Frontotemporal Dementia (FTD) using clinical data alone. The [ 11 C]-PiB PET scan offers a new method of identifying AD based on the detection of amyloid deposits. Our study investigated whether there was an agreement between neuropsychological and behavioral data and PiB findings in the diagnosis of FTD. Participants were 32 patients diagnosed with suspected FTD by clinical consensus. All participants underwent neuropsychological testing and PiB imaging. In addition, caregivers completed behavioral ratings of participants' memory, frontal behaviors, and mood. Seventeen participants were classified as PiB positive (+). Results of MANOVA and subsequent ANOVA analyses showed a significant difference on memory performance between the PiB- and PiB+groups, with the PiB- group performing better than the PiB+group. There were no significant differences between the groups on cognitive or behavioral measures of executive/frontal impairment, mood. Both groups showed similar severity of dementia. These findings provide evidence for the utility of the [ 11 C]-PiB PET scan in distinguishing between AD and FTD, with evaluation of memory at clinical diagnosis serving as a valuable indicator of the absence of FTD and consideration for an AD diagnosis. Our results would support the concern that patients who may present with primary behavioral or executive dysfunction may not necessarily have FTD, particularly if memory deficits are evident. Copyright © 2017 Elsevier Ltd. All rights reserved.

  5. Performance of patients with frontotemporal lobar degeneration on artistic tasks: A pilot study

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    Maria Cristina Anauate

    Full Text Available ABSTRACT Several studies have addressed visuospatial and executive skills in artistic activities in Frontotemporal Lobar Degeneration (FTLD and Alzheimer's disease (AD. Objective: To investigate the performance of FTLD patients compared to controls on two artistic tasks. Methods: Four FTLD patients with mean age of 57 (8.7 years and schooling of 12.2 (4.5 years plus 10 controls with mean age of 62.9 (8.6 years and schooling of 12.3 (4.6 years, were assessed using the Lowenstein Occupational Therapy Cognitive Assessment (LOTCA and by a three-stage artistic protocol including visual observation, copying and collage, based on a Sisley painting. Results: FTLD patients had lower scores than controls on Visuospatial Perception, Copy, Collage, Examiner's Observation, and Total, showing distinct patterns of performance according to FTLD sub-type: semantic PPA, nonfluent PPA and bvFTD. Conclusion: FTLD patients presented impairment in the visuospatial and executive skills required to perform artistic tasks. We demonstrated that the application of the instrument as a complimentary method for assessing cognitive skills in this group of patients is possible. Further studies addressing larger and more homogeneous samples of FTLD patients as well as other dementias are warranted.

  6. Evaluation of patients with behavioral and cognitive complaints: Misdiagnosis in frontotemporal dementia and Alzheimer's disease

    Directory of Open Access Journals (Sweden)

    Bárbara Costa Beber

    Full Text Available ABSTRACT Background: Frontotemporal dementia (FTD is a heterogeneous clinicopathological syndrome whose early diagnosis is critical for developing management strategies. Objective: To analyze the variables associated with misdiagnosis in a group of patients with FTD, Alzheimer's disease (AD, and without neurodegenerative disorders (WND, all of whom were evaluated for behavioral and cognitive complaints. Methods: A case-control study with FTD (n=10, probable AD (n=10 and WND (n=10 patients was carried out. The studied variables were disease duration, reason for referral, former diagnosis, behavioral and cognitive symptoms at evaluation, MMSE at the specialist evaluation, and follow-up outcome. The data were analyzed by ANOVA with Bonferroni post-hoc and by Pearson's Chi-Square tests. Results: FTD patients and WND patients showed longer disease duration than AD patients; the main reasons for referral in the FTD group were behavioral, memory and memory plus language problems while all AD and 90% of the WND group were referred for memory. The FTD group had the highest rate of misdiagnosis and worst outcomes after the 12-month follow-up. The majority of AD and WND patients had memory symptoms, while FTD patients presented language (30%, memory and/or language (40% problems on the evaluation. Conclusion: Difficulty in recognizing the main features of FTD and psychiatric disorders with memory impairment was observed. Clinicians tended to generalize memory complaints toward a single diagnosis, identifying almost all these patients as AD or leaving them undiagnosed.

  7. Life enhancing activities for family caregivers of people with frontotemporal dementia.

    Science.gov (United States)

    Dowling, Glenna A; Merrilees, Jennifer; Mastick, Judy; Chang, Vickie Y; Hubbard, Erin; Moskowitz, Judith Tedlie

    2014-01-01

    Aberrant psychological and behavioral symptoms are common in patients with dementia. These symptoms have negative consequences for family caregivers, causing stress and burden. Frontotemporal dementia (FTD) symptoms cause more pronounced stress and burden on caregivers than those associated with Alzheimer dementia. In this randomized, attention control pilot study, we delivered 5-weekly, one-on-one, positive affect intervention sessions to family caregivers of people with FTD. The program, Life Enhancing Activities for Family Caregivers: LEAF was conducted in-person or by videoconference with caregivers across the United States. Measures of affect, caregiver mood, stress, distress, and caregiver burden were assessed at baseline, end of sessions, and 1 month after completion. Twenty-four caregivers (12 intervention and 12 attention control) participated. At the end of the intervention, scores on positive affect, negative affect, burden, and stress all improved in the intervention compared with the control group. These scores continued to show improvement at the assessment done 1 month after intervention. Subjects were receptive to the skills and the delivery methods. The positive emotion skill-building intervention proved feasible especially in the internet videoconference delivery format. The intervention promoted positive affect and improved psychological outcomes for family caregivers of people with FTD.

  8. Functional connectivity and microstructural white matter changes in phenocopy frontotemporal dementia

    International Nuclear Information System (INIS)

    Meijboom, R.; Steketee, R.M.E.; Lugt, A. van der; Smits, M.; Koning, I. de; Osse, R.J.; Jiskoot, L.C.; Jong, F.J. de; Swieten, J.C. van

    2017-01-01

    Phenocopy frontotemporal dementia (phFTD) is a rare and poorly understood clinical syndrome. PhFTD shows core behavioural variant FTD (bvFTD) symptoms without associated cognitive deficits and brain abnormalities on conventional MRI and without progression. In contrast to phFTD, functional connectivity and white matter (WM) microstructural abnormalities have been observed in bvFTD. We hypothesise that phFTD belongs to the same disease spectrum as bvFTD and investigated whether functional connectivity and microstructural WM changes similar to bvFTD are present in phFTD. Seven phFTD patients without progression or alternative psychiatric diagnosis, 12 bvFTD patients and 17 controls underwent resting state functional MRI (rs-fMRI) and diffusion tensor imaging (DTI). Default mode network (DMN) connectivity and WM measures were compared between groups. PhFTD showed subtly increased DMN connectivity and subtle microstructural changes in frontal WM tracts. BvFTD showed abnormalities in similar regions as phFTD, but had lower increased DMN connectivity and more extensive microstructural WM changes. Our findings can be interpreted as neuropathological changes in phFTD and are in support of the hypothesis that phFTD and bvFTD may belong to the same disease spectrum. Advanced MRI techniques, objectively identifying brain abnormalities, would therefore be potentially suited to improve the diagnosis of phFTD. (orig.)

  9. Distinct perfusion patterns in Alzheimer's disease, frontotemporal dementia and dementia with Lewy bodies

    Energy Technology Data Exchange (ETDEWEB)

    Binnewijzend, Maja A.A.; Wattjes, Mike P.; Berckel, Bart N.M. van; Barkhof, Frederik [VU University Medical Center and Neuroscience Campus Amsterdam, Department of Radiology and Nuclear Medicine, Amsterdam (Netherlands); Kuijer, Joost P.A. [VU University Medical Center and Neuroscience Campus Amsterdam, Department of Physics and Medical Technology, Amsterdam (Netherlands); Flier, Wiesje M. van der [VU University Medical Center and Neuroscience Campus Amsterdam, Alzheimercenter and Department of Neurology, Amsterdam (Netherlands); VU University Medical Center and Neuroscience Campus Amsterdam, Department of Epidemiology and Biostatistics, Amsterdam (Netherlands); Benedictus, Marije R.; Moeller, Christiane M.; Pijnenburg, Yolande A.L.; Lemstra, Afina W.; Prins, Niels D.; Scheltens, Philip [VU University Medical Center and Neuroscience Campus Amsterdam, Alzheimercenter and Department of Neurology, Amsterdam (Netherlands)

    2014-09-15

    To compare pseudo-continuous arterial spin-labelled (PCASL) magnetic resonance imaging (MRI) measured quantitative cerebral blood flow (CBF) of patients with frontotemporal dementia (FTD), dementia with Lewy Bodies (DLB), Alzheimer's disease (AD) and controls, in a region of interest (ROI) and voxel-wise fashion. We analysed whole-brain 3D fast-spin-echo PCASL images of 20 FTD patients, 14 DLB patients, 48 AD patients and 50 controls from the Amsterdam Dementia Cohort. Regional CBF patterns were compared using analyses of variance for repeated measures. Permutation tests were used for voxel-wise comparisons. Analyses were performed using uncorrected and partial volume corrected (PVC) maps. All analyses were corrected for age and sex. There was an interaction between diagnosis and region (p < 0.001), implying differences in regional CBF changes between diagnostic groups. In AD patients, CBF was decreased in all supratentorial regions, most prominently so in the posterior regions. DLB patients showed lowest CBF values throughout the brain, but temporal CBF was preserved. Supratentorial PVC cortical CBF values were lowest in the frontal lobes in FTD patients, and in the temporal lobes in AD patients. Patients with AD, FTD and DLB display distinct patterns of quantitative regional CBF changes. 3D-PCASL may provide additional value in the workup of dementia patients. (orig.)

  10. Induced Pluripotent Stem Cell Models of Progranulin-Deficient Frontotemporal Dementia Uncover Specific Reversible Neuronal Defects

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    Sandra Almeida

    2012-10-01

    Full Text Available The pathogenic mechanisms of frontotemporal dementia (FTD remain poorly understood. Here we generated multiple induced pluripotent stem cell lines from a control subject, a patient with sporadic FTD, and an FTD patient with a novel heterozygous GRN mutation (progranulin [PGRN] S116X. In neurons and microglia differentiated from PGRN S116X induced pluripotent stem cells, the levels of intracellular and secreted PGRN were reduced, establishing patient-specific cellular models of PGRN haploinsufficiency. Through a systematic screen of inducers of cellular stress, we found that PGRN S116X neurons, but not sporadic FTD neurons, exhibited increased sensitivity to staurosporine and other kinase inhibitors. Moreover, the serine/threonine kinase S6K2, a component of the phosphatidylinositol 3-kinase and mitogen-activated protein kinase pathways, was specifically downregulated in PGRN S116X neurons. Both increased sensitivity to kinase inhibitors and reduced S6K2 were rescued by PGRN expression. Our findings identify cell-autonomous, reversible defects in patient neurons with PGRN deficiency, and provide a compelling model for studying PGRN-dependent pathogenic mechanisms and testing potential therapies.

  11. TDP-43 pathology in primary progressive aphasia and frontotemporal dementia with pathologic Alzheimer disease

    Science.gov (United States)

    Mishra, Manjari; Hatanpaa, Kimmo J.; White, Charles L.; Johnson, Nancy; Rademaker, Alfred; Weitner, Bing Bing; Deng, Han-Xiang; Dubner, Steven D.; Weintraub, Sandra; Mesulam, Marsel

    2010-01-01

    The clinical syndrome of primary progressive aphasia (PPA) can be associated with a variety of neuropathologic diagnoses at autopsy. Thirty percent of cases have Alzheimer disease (AD) pathology, most often in the usual distribution, which defies principles of brain–behavior organization, in that aphasia is not symptomatic of limbic disease. The present study investigated whether concomitant TDP-43 pathology could resolve the lack of clinicoanatomic concordance. In this paper, 16 cases of clinical PPA and 10 cases of primarily non-aphasic frontotemporal dementia (FTD), all with AD pathology, were investigated to determine whether their atypical clinical phenotypes reflected the presence of additional TDP-43 pathology. A comparison group consisted of 27 cases of pathologic AD with the typical amnestic clinical phenotype of probable AD. Concomitant TDP-43 pathology was discovered in only three of the FTD and PPA but in more than half of the typical amnestic clinical phenotypes. Hippocampal sclerosis (HS) was closely associated with TDP-43 pathology when all groups were combined for analysis. Therefore, the clinical phenotypes of PPA and FTD in cases with pathologic AD are only rarely associated with TDP-43 proteinopathy. Furthermore, medial temporal TDP-43 pathology is more tightly linked to HS than to clinical phenotype. These findings challenge the current notions about clinicopathologic correlation, especially about the role of multiple pathologies. PMID:20361198

  12. Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum

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    Hamideh Shahheydari

    2017-05-01

    Full Text Available Protein homeostasis, or proteostasis, has an important regulatory role in cellular function. Protein quality control mechanisms, including protein folding and protein degradation processes, have a crucial function in post-mitotic neurons. Cellular protein quality control relies on multiple strategies, including molecular chaperones, autophagy, the ubiquitin proteasome system, endoplasmic reticulum (ER-associated degradation (ERAD and the formation of stress granules (SGs, to regulate proteostasis. Neurodegenerative diseases are characterized by the presence of misfolded protein aggregates, implying that protein quality control mechanisms are dysfunctional in these conditions. Amyotrophic lateral sclerosis (ALS and frontotemporal dementia (FTD are neurodegenerative diseases that are now recognized to overlap clinically and pathologically, forming a continuous disease spectrum. In this review article, we detail the evidence for dysregulation of protein quality control mechanisms across the whole ALS-FTD continuum, by discussing the major proteins implicated in ALS and/or FTD. We also discuss possible ways in which protein quality mechanisms could be targeted therapeutically in these disorders and highlight promising protein quality control-based therapeutics for clinical trials.

  13. Behavioral variant frontotemporal dementia patients do not succumb to the Allais paradox

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    Maxime eBertoux

    2014-09-01

    Full Text Available The Allais Paradox represents on of the earliest empirical challenges to normative models of decision-making, and suggests that choices in one part of a gamble may depend on the possible outcome in another, independent, part of the gamble—a violation of the so-called independence axiom. To account for Allaisian behavior, one well-known class of models propose that individuals’ choices are influenced not only by possible outcomes resulting from one’s choices, but also the anticipation of regret for foregone options. Here we test the regret hypothesis using a population of patients with behavioral variant frontotemporal dementia (bvFTD, a clinical population known to present ventromedial prefrontal cortex dysfunctions and associated with impaired regret processing in previous studies of decision-making. Compared to behavior of matched controls and Alzheimer (AD patients that has no ventromedial prefrontal atrophy, we found a striking diminution of Allaisian behavior among bvFTD patients. These results are consistent with the regret hypothesis and furthermore suggest a crucial role for prefrontal regions in choices that typically stands in contradiction with a basic axiom of rational decision-making.

  14. Reduced left precentral regional responses in patients with major depressive disorder and history of suicide attempts.

    Science.gov (United States)

    Tsujii, Noa; Mikawa, Wakako; Tsujimoto, Emi; Adachi, Toru; Niwa, Atsushi; Ono, Hisae; Shirakawa, Osamu

    2017-01-01

    Previous neuroimaging studies have revealed frontal and temporal functional abnormalities in patients with major depressive disorder (MDD) and a history of suicidal behavior. However, it is unknown whether multi-channel near-infrared spectroscopy (NIRS) signal changes among individuals with MDD are associated with a history of suicide attempts and a diathesis for suicidal behavior (impulsivity, hopelessness, and aggression). Therefore, we aimed to explore frontotemporal hemodynamic responses in depressed patients with a history of suicide attempts using 52-channel NIRS. We recruited 30 patients with MDD and a history of suicidal behavior (suicide attempters; SAs), 38 patient controls without suicidal behavior (non-attempters; NAs), and 40 healthy controls (HCs) matched by age, gender ratio, and estimated IQ. Regional hemodynamic responses during a verbal fluency task (VFT) were monitored using NIRS. Our results showed that severities of depression, impulsivity, aggression, and hopelessness were similar between SAs and NAs. Both patient groups had significantly reduced activation compared with HCs in the bilateral frontotemporal regions. Post hoc analyses revealed that SAs exhibited a smaller hemodynamic response in the left precentral gyrus than NAs and HCs. Furthermore, the reduced response in the left inferior frontal gyrus was negatively correlated with impulsivity level and hemodynamic responses in the right middle frontal gyrus were negatively associated with hopelessness and aggression in SAs but not in NAs and HCs. Our findings suggest that MDD patients with a history of suicide attempts demonstrate patterns of VFT-induced NIRS signal changes different from those demonstrated by individuals without a history of suicidal behaviors, even in cases where clinical symptoms are similar. NIRS has a relatively high time resolution, which may help visually differentiate SAs from NAs.

  15. Why Dora Left

    DEFF Research Database (Denmark)

    Gammelgård, Judy

    2017-01-01

    The question of why Dora left her treatment before it was brought to a satisfactory end and the equally important question of why Freud chose to publish this problematic and fragmentary story have both been dealt with at great length by Freud’s successors. Dora has been read by analysts, literary...... critics, and not least by feminists. The aim of this paper is to point out the position Freud took toward his patient. Dora stands out as the one case among Freud’s 5 great case stories that has a female protagonist, and reading the case it becomes clear that Freud stumbled because of an unresolved...... problem toward femininity, both Dora’s and his own. In Dora, it is argued, Freud took a new stance toward the object of his investigation, speaking from the position of the master. Freud presents himself as the one who knows, in great contrast to the position he takes when unraveling the dream. Here he...

  16. Patterns of Microglial Cell Activation in Alzheimer Disease and Frontotemporal Lobar Degeneration.

    Science.gov (United States)

    Taipa, Ricardo; Brochado, Paulo; Robinson, Andrew; Reis, Inês; Costa, Patrício; Mann, David M; Melo Pires, Manuel; Sousa, Nuno

    2017-01-01

    Microglia-driven neuroinflammation can play an important role in the pathophysiology of neurodegenerative disorders. In this study, we sought to characterize the distribution of microglial cell activation in 2 neurodegenerative dementias with distinct protein signatures, Alzheimer disease (AD) and frontotemporal lobar degeneration (FTLD) of the TDP subtype, and to determine if there was an anatomical correlation with the phenotypes most commonly associated with these conditions. The distribution and extent of microglial cell activation was assessed semiquantitatively in the hippocampal formation, cortical gray matter, and subcortical white matter of CD68-immunostained sections of the frontal, temporal, parietal, and occipital cortices from 15 pathologically confirmed cases of AD, 13 cases of FTLD, and 18 controls. Significantly higher levels of microglial cell activation occurred in the subiculum in AD and FTLD than in controls. Additionally, AD had higher microglial activation in the CA1 and FTLD in the hippocampal white matter than the controls. Microglial activation was greater in the dentate gyrus molecular layer in AD than in FTLD. In the cortical regions, the 2 pathological groups differed only in frontal white matter, with the FTLD group showing higher microglial scores. FTLD showed higher microglial activation in the white matter compared to the respective gray matter in the entorhinal, temporal, and frontal regions. Our work expands the knowledge of the distribution and magnitude of microglial activation in these disorders. Additionally, we found some microglial circuit-specific patterns that could help to explain some of the clinical overlap between AD and FTLD-TDP, namely in memory deficits. © 2017 S. Karger AG, Basel.

  17. Profiling Speech and Pausing in Amyotrophic Lateral Sclerosis (ALS and Frontotemporal Dementia (FTD.

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    Yana Yunusova

    Full Text Available This study examines reading aloud in patients with amyotrophic lateral sclerosis (ALS and those with frontotemporal dementia (FTD in order to determine whether differences in patterns of speaking and pausing exist between patients with primary motor vs. primary cognitive-linguistic deficits, and in contrast to healthy controls.136 participants were included in the study: 33 controls, 85 patients with ALS, and 18 patients with either the behavioural variant of FTD (FTD-BV or progressive nonfluent aphasia (FTD-PNFA. Participants with ALS were further divided into 4 non-overlapping subgroups--mild, respiratory, bulbar (with oral-motor deficit and bulbar-respiratory--based on the presence and severity of motor bulbar or respiratory signs. All participants read a passage aloud. Custom-made software was used to perform speech and pause analyses, and this provided measures of speaking and articulatory rates, duration of speech, and number and duration of pauses. These measures were statistically compared in different subgroups of patients.The results revealed clear differences between patient groups and healthy controls on the passage reading task. A speech-based motor function measure (i.e., articulatory rate was able to distinguish patients with bulbar ALS or FTD-PNFA from those with respiratory ALS or FTD-BV. Distinguishing the disordered groups proved challenging based on the pausing measures.This study demonstrated the use of speech measures in the identification of those with an oral-motor deficit, and showed the usefulness of performing a relatively simple reading test to assess speech versus pause behaviors across the ALS-FTD disease continuum. The findings also suggest that motor speech assessment should be performed as part of the diagnostic workup for patients with FTD.

  18. Amygdala TDP-43 Pathology in Frontotemporal Lobar Degeneration and Motor Neuron Disease.

    Science.gov (United States)

    Takeda, Takahiro; Seilhean, Danielle; Le Ber, Isabelle; Millecamps, Stéphanie; Sazdovitch, Véronique; Kitagawa, Kazuo; Uchihara, Toshiki; Duyckaerts, Charles

    2017-09-01

    TDP-43-positive inclusions are present in the amygdala in frontotemporal lobar degeneration (FTLD) and motor neuron disease (MND) including amyotrophic lateral sclerosis. Behavioral abnormalities, one of the chief symptoms of FTLD, could be, at least partly, related to amygdala pathology. We examined TDP-43 inclusions in the amygdala of patients with sporadic FTLD/MND (sFTLD/MND), FTLD/MND with mutation of the C9ORF72 (FTLD/MND-C9) and FTLD with mutation of the progranulin (FTLD-GRN). TDP-43 inclusions were common in each one of these subtypes, which can otherwise be distinguished on topographical and genetic grounds. Conventional and immunological stainings were performed and we quantified the numerical density of inclusions on a regional basis. TDP-43 inclusions in amygdala could be seen in 10 out of 26 sFTLD/MND cases, 5 out of 9 FTLD/MND-C9 cases, and all 4 FTLD-GRN cases. Their numerical density was lower in FTLD/MND-C9 than in sFTLD/MND and FTLD-GRN. TDP-43 inclusions were more numerous in the ventral region of the basolateral nucleus group in all subtypes. This contrast was apparent in sporadic and C9-mutated FTLD/MND, while it was less evident in FTLD-GRN. Such differences in subregional involvement of amygdala may be related to the region-specific neuronal connections that are differentially affected in FTLD/MND and FTLD-GRN. © 2017 American Association of Neuropathologists, Inc. All rights reserved.

  19. Caregiver burden in atypical dementias: comparing frontotemporal dementia, Creutzfeldt-Jakob disease, and Alzheimer's disease.

    Science.gov (United States)

    Uflacker, Alice; Edmondson, Mary C; Onyike, Chiadi U; Appleby, Brian S

    2016-02-01

    Caregiver burden is a significant issue in the treatment of dementia and a known contributor to institutionalization of patients with dementia. Published data have documented increased caregiver burden in behavioral variant frontotemporal dementia (bvFTD) compared to Alzheimer's disease (AD). Another atypical dementia with high-perceived caregiver burden is sporadic Creutzfeldt-Jakob disease (sCJD), but no formal studies have assessed this perception. The aim of this study was to compare caregiver burden across atypical dementia etiologies. 76 adults with atypical dementia (young-onset AD [YOAD], bvFTD, language variant FTD [lvFTD], and sCJD) were administered an abbreviated version of the Zarit Burden Interview (ZBI), Neuropsychiatric Inventory (NPI-Q), and other assessment instruments during a five-year time period at Johns Hopkins Hospital (JHH). A Cox regression model examined differences between disease categories that impact mean ZBI scores. Mean ZBI scores were significantly different between dementia etiologies, with bvFTD and sCJD having the highest caregiver burden (p = 0.026). Mean NPI-Q caregiver distress scores were highest in bvFTD and sCJD (p = 0.002), with sCJD and bvFTD also having the highest number of endorsed symptom domains (p = 0.012). On regression analyses, an interactive variable combining final diagnosis category and NPI-Q total severity score demonstrated statistically significant differences in mean ZBI scores for sCJD and bvFTD. This study demonstrates that bvFTD and sCJD have increased levels of caregiver burden, NPI-Q caregiver distress, total severity scores, and number of endorsed symptom domains. These results suggest that higher caregiver burden in bvFTD and sCJD are disease specific and possibly related to neuropsychiatric symptoms.

  20. The Role of Coping Strategies in Psychological Outcomes for Frontotemporal Dementia Caregivers.

    Science.gov (United States)

    Roche DClinPsy, Lauren; Croot, Karen; MacCann, Carolyn; Cramer, Barbara; Diehl-Schmid, Janine

    2015-09-01

    Caregiving for a person with frontotemporal dementia (FTD) is related to poor caregiver outcomes. Coping strategies adopted by caregivers are known to influence psychological outcomes in other dementia caregiver populations, however, their influence on psychological outcomes in FTD caregivers is poorly understood at present. Questionnaire data for 94 German primary caregivers (mean [M] 59.11 years, 68 females) of FTD care-recipients living in the community (M 63.94, 30 females) were investigated. Standardized measures completed by the caregiver included the Caregiver Strain Index (CSI), Beck Depression Inventory II (BDI-II), Quality of Life-Alzheimer's Disease (QoL-AD), and the Brief Coping Orientations to Problems Experienced (COPE). Care-recipients' nursing care level was collected as a measure of the intensity of their care needs. Mediation analyses showed that the effect of the intensity of care-recipients' care needs on caregiver well-being depended on caregivers' experience of strain. High levels of caregiver strain did not predict depression (-0.22, 95% confidence interval CI: [0.16 to 2.04]) but predicted reduced QoL (-0.44, CI: [-1.15 to -.16]). Moreover, caregivers' experience of strain was exacerbated by their use of dysfunctional coping (β = .21; p = .04), care-recipients' intensity of care needs (β = .25; p = .01), and fewer financial resources (β = .23; p = .02). In turn, caregivers' use of dysfunctional coping as a response to their strain increased the levels of depression (0.46, CI: [0.19-0.82]). By contrast, use of problem-focused coping strategies increased caregivers' QoL (0.10, CI: [0.00 to 0.31]). This study identifies variables amenable to clinical interventions that can improve caregivers' well-being: specifically, caregiver strain and coping strategies. For a disease without cure yet increasing prevalence and cost, ameliorating the caregiver experience through targeted interventions is essential. © The Author(s) 2015.

  1. Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum.

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    Patricia Lillo

    Full Text Available There is increasing evidence that amyotrophic lateral sclerosis (ALS and frontotemporal dementia (FTD lie on a clinical, pathological and genetic continuum with patients of one disease exhibiting features of the other. Nevertheless, to date, the underlying grey matter and white matter changes across the ALS-FTD disease continuum have not been explored. In this study fifty-three participants with ALS (n = 10, ALS-FTD (n = 10 and behavioural variant FTD (bvFTD; n = 15 as well as controls (n = 18, underwent detailed clinical assessment plus structural imaging using voxel-based morphometry (VBM and diffusion tensor imaging (DTI analysis of magnetic resonance brain imaging to examine grey and white matter differences and commonalities across the continuum. Importantly, patient groups were matched for age, education, gender and disease duration. VBM and DTI results showed that changes in the ALS group were confined mainly to the motor cortex and anterior cingulate as well as their underlying white matter tracts. ALS-FTD and bvFTD showed widespread grey matter and white matter changes involving frontal and temporal lobes. Extensive prefrontal cortex changes emerged as a marker for bvFTD compared to other subtypes, while ALS-FTD could be distinguished from ALS by additional temporal lobe grey and white matter changes. Finally, ALS could be mainly distinguished from the other two groups by corticospinal tract degeneration. The present study shows for the first time that FTD and ALS overlap in anterior cingulate, motor cortex and related white matter tract changes across the whole continuum. Nevertheless, frontal and temporal atrophy as well as corticospinal tract degeneration emerged as marker for subtype classification, which will inform future diagnosis and target disease management across the continuum.

  2. Emotion Recognition in Frontotemporal Dementia and Alzheimer's Disease: A New Film-Based Assessment

    Science.gov (United States)

    Goodkind, Madeleine S.; Sturm, Virginia E.; Ascher, Elizabeth A.; Shdo, Suzanne M.; Miller, Bruce L.; Rankin, Katherine P.; Levenson, Robert W.

    2015-01-01

    Deficits in recognizing others' emotions are reported in many psychiatric and neurological disorders, including autism, schizophrenia, behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD). Most previous emotion recognition studies have required participants to identify emotional expressions in photographs. This type of assessment differs from real-world emotion recognition in important ways: Images are static rather than dynamic, include only 1 modality of emotional information (i.e., visual information), and are presented absent a social context. Additionally, existing emotion recognition batteries typically include multiple negative emotions, but only 1 positive emotion (i.e., happiness) and no self-conscious emotions (e.g., embarrassment). We present initial results using a new task for assessing emotion recognition that was developed to address these limitations. In this task, respondents view a series of short film clips and are asked to identify the main characters' emotions. The task assesses multiple negative, positive, and self-conscious emotions based on information that is multimodal, dynamic, and socially embedded. We evaluate this approach in a sample of patients with bvFTD, AD, and normal controls. Results indicate that patients with bvFTD have emotion recognition deficits in all 3 categories of emotion compared to the other groups. These deficits were especially pronounced for negative and self-conscious emotions. Emotion recognition in this sample of patients with AD was indistinguishable from controls. These findings underscore the utility of this approach to assessing emotion recognition and suggest that previous findings that recognition of positive emotion was preserved in dementia patients may have resulted from the limited sampling of positive emotion in traditional tests. PMID:26010574

  3. Social Cognition Differentiates Behavioral Variant Frontotemporal Dementia From Other Neurodegenerative Diseases and Psychiatric Disorders.

    Science.gov (United States)

    Gossink, Flora; Schouws, Sigfried; Krudop, Welmoed; Scheltens, Philip; Stek, Max; Pijnenburg, Yolande; Dols, Annemiek

    2018-02-01

    Although deficits in social cognition are established as core features in behavioral variant frontotemporal dementia (bvFTD), it remains unresolved if impaired social cognition distinguishes bvFTD from the broad differential diagnoses in clinical practice. Our aim was to study whether social cognition discriminates bvFTD from other neurodegenerative diseases and psychiatric disorders in patients presenting with late-onset frontal symptoms. Next, we studied the association of social cognition with frontal symptoms and cognitive functioning. In this longitudinal multicenter study, besides clinical rating scales for frontal symptoms, social cognition was determined by Ekman 60 Faces test and Faux Pas in addition to neuropsychological tests for other cognitive domains in patients with probable and definite bvFTD (N = 22), other neurodegenerative diseases (N = 24), and psychiatric disorders (N = 33). Median symptom duration was 2.8 years, and patients were prospectively followed over 2 years. Total scores from Ekman 60 Faces test were significantly lower in bvFTD than in other neurodegenerative diseases and psychiatric disorders. Ekman 60 Faces test explained 91.2% of the variance of psychiatric disorders and other neurodegenerative diseases versus bvFTD (χ 2  = 11.02, df = 1, p = 0.001) and was associated with all other cognitive domains. Faux Pas and the other cognitive domains did not differ between these diagnostic groups. In this clinical sample Ekman 60 Faces test distinguished bvFTD successfully from other neurodegenerative diseases and psychiatric disorders. Although associated with social cognition, other cognitive domains were not discriminative. This study provides arguments to add the Ekman 60 Faces test to the neuropsychological examination in the diagnostic procedure of bvFTD. Copyright © 2018 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.

  4. White Matter Changes Associated with Resting Sympathetic Tone in Frontotemporal Dementia vs. Alzheimer's Disease.

    Directory of Open Access Journals (Sweden)

    Mario F Mendez

    Full Text Available Resting sympathetic tone, a measure of physiological arousal, is decreased in patients with apathy and inertia, such as those with behavioral variant frontotemporal dementia (bvFTD and other frontally-predominant disorders.To identify the neuroanatomical correlates of skin conductance levels (SCLs, an index of resting sympathetic tone and apathy, among patients with bvFTD, where SCLs is decreased, compared to those with Alzheimer's disease (AD, where it is not.This study analyzed bvFTD (n = 14 patients and a comparison group with early-onset AD (n = 19. We compared their resting SCLs with gray matter and white matter regions of interest and white matter measures of fiber integrity on magnetic resonance imaging and diffusion tensor imaging.As expected, bvFTD patients, compared to AD patients, had lower SCLs, which correlated with an apathy measure, and more gray matter loss and abnormalities of fiber integrity (fractional anisotropy and mean diffusivity in frontal-anterior temporal regions. After controlling for group membership, the SCLs were significantly correlated with white matter volumes in the cingulum and inferior parietal region in the right hemisphere.Among dementia patients, SCLs, and resting sympathetic tone, may correlate with quantity of white matter, rather than with gray matter or with white matter fiber integrity. Loss of white matter volumes, especially involving a right frontoparietal network, may reflect chronic loss of cortical axons that mediate frontal control of resting sympathetic tone, changes that could contribute to the apathy and inertia of bvFTD and related disorders.

  5. Apathy in Frontotemporal Degeneration: Neuroanatomical Evidence of Impaired Goal-Directed Behavior

    Directory of Open Access Journals (Sweden)

    Lauren eMassimo

    2015-11-01

    Full Text Available Background: Apathy, the major manifestation of impaired goal-directed behavior (GDB, is the most common neuropsychiatric syndrome associated with behavioral variant frontotemporal degeneration (bvFTD. The behavioral and biological mechanisms of apathy, however, are not well understood. We hypothesized that GDB has multiple components – including at least initiation, planning and motivation – and that GDB is supported by a network of multiple frontal brain regions. In this study, we examined this hypothesis by evaluating the selective breakdown of GDB in bvFTD, and relating these deficits to grey matter (GM atrophy and white matter (WM integrity. Methods: Eighteen apathetic bvFTD participants and 17 healthy controls completed the Philadelphia Apathy Computerized Test (PACT. This test quantifies each of three components of GDB hypothesized to contribute to apathy. We then used regression analyses to relate PACT scores to GM atrophy and reduced white matter (WM fractional anisotropy (FA in bvFTD. Results: Compared to controls, bvFTD participants demonstrated significant impairments in each of the three hypothesized components of GDB that contribute to apathy. Regression analyses related each component to disease in specific GM structures and associated WM tracts. Poor initiation thus was related to GM atrophy in anterior cingulate and reduced FA in the cingulum. Planning impairment was related to GM atrophy in dorsolateral prefrontal cortex and reduced FA in superior longitudinal fasciculus. Poor motivation was related to GM atrophy in orbitofrontal cortex and reduced FA in uncinate fasciculus. Conclusions: bvFTD patients have difficulty with initiation, planning and motivation components of GDB. These findings are consistent with the hypotheses that GDB encompasses at least three processes, that these are supported by a large-scale neural network within specific portions of the frontal lobe, and that degradation of any one of these prefrontal

  6. Motor speech signature of behavioral variant frontotemporal dementia: Refining the phenotype.

    Science.gov (United States)

    Vogel, Adam P; Poole, Matthew L; Pemberton, Hugh; Caverlé, Marja W J; Boonstra, Frederique M C; Low, Essie; Darby, David; Brodtmann, Amy

    2017-08-22

    To provide a comprehensive description of motor speech function in behavioral variant frontotemporal dementia (bvFTD). Forty-eight individuals (24 bvFTD and 24 age- and sex-matched healthy controls) provided speech samples. These varied in complexity and thus cognitive demand. Their language was assessed using the Progressive Aphasia Language Scale and verbal fluency tasks. Speech was analyzed perceptually to describe the nature of deficits and acoustically to quantify differences between patients with bvFTD and healthy controls. Cortical thickness and subcortical volume derived from MRI scans were correlated with speech outcomes in patients with bvFTD. Speech of affected individuals was significantly different from that of healthy controls. The speech signature of patients with bvFTD is characterized by a reduced rate (75%) and accuracy (65%) on alternating syllable production tasks, and prosodic deficits including reduced speech rate (45%), prolonged intervals (54%), and use of short phrases (41%). Groups differed on acoustic measures derived from the reading, unprepared monologue, and diadochokinetic tasks but not the days of the week or sustained vowel tasks. Variability of silence length was associated with cortical thickness of the inferior frontal gyrus and insula and speech rate with the precentral gyrus. One in 8 patients presented with moderate speech timing deficits with a further two-thirds rated as mild or subclinical. Subtle but measurable deficits in prosody are common in bvFTD and should be considered during disease management. Language function correlated with speech timing measures derived from the unprepared monologue only. © 2017 American Academy of Neurology.

  7. Profiling Speech and Pausing in Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD)

    Science.gov (United States)

    Yunusova, Yana; Graham, Naida L.; Shellikeri, Sanjana; Phuong, Kent; Kulkarni, Madhura; Rochon, Elizabeth; Tang-Wai, David F.; Chow, Tiffany W.; Black, Sandra E.; Zinman, Lorne H.; Green, Jordan R.

    2016-01-01

    Objective This study examines reading aloud in patients with amyotrophic lateral sclerosis (ALS) and those with frontotemporal dementia (FTD) in order to determine whether differences in patterns of speaking and pausing exist between patients with primary motor vs. primary cognitive-linguistic deficits, and in contrast to healthy controls. Design 136 participants were included in the study: 33 controls, 85 patients with ALS, and 18 patients with either the behavioural variant of FTD (FTD-BV) or progressive nonfluent aphasia (FTD-PNFA). Participants with ALS were further divided into 4 non-overlapping subgroups—mild, respiratory, bulbar (with oral-motor deficit) and bulbar-respiratory—based on the presence and severity of motor bulbar or respiratory signs. All participants read a passage aloud. Custom-made software was used to perform speech and pause analyses, and this provided measures of speaking and articulatory rates, duration of speech, and number and duration of pauses. These measures were statistically compared in different subgroups of patients. Results The results revealed clear differences between patient groups and healthy controls on the passage reading task. A speech-based motor function measure (i.e., articulatory rate) was able to distinguish patients with bulbar ALS or FTD-PNFA from those with respiratory ALS or FTD-BV. Distinguishing the disordered groups proved challenging based on the pausing measures. Conclusions and Relevance This study demonstrated the use of speech measures in the identification of those with an oral-motor deficit, and showed the usefulness of performing a relatively simple reading test to assess speech versus pause behaviors across the ALS—FTD disease continuum. The findings also suggest that motor speech assessment should be performed as part of the diagnostic workup for patients with FTD. PMID:26789001

  8. Emotion recognition in frontotemporal dementia and Alzheimer's disease: A new film-based assessment.

    Science.gov (United States)

    Goodkind, Madeleine S; Sturm, Virginia E; Ascher, Elizabeth A; Shdo, Suzanne M; Miller, Bruce L; Rankin, Katherine P; Levenson, Robert W

    2015-08-01

    Deficits in recognizing others' emotions are reported in many psychiatric and neurological disorders, including autism, schizophrenia, behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD). Most previous emotion recognition studies have required participants to identify emotional expressions in photographs. This type of assessment differs from real-world emotion recognition in important ways: Images are static rather than dynamic, include only 1 modality of emotional information (i.e., visual information), and are presented absent a social context. Additionally, existing emotion recognition batteries typically include multiple negative emotions, but only 1 positive emotion (i.e., happiness) and no self-conscious emotions (e.g., embarrassment). We present initial results using a new task for assessing emotion recognition that was developed to address these limitations. In this task, respondents view a series of short film clips and are asked to identify the main characters' emotions. The task assesses multiple negative, positive, and self-conscious emotions based on information that is multimodal, dynamic, and socially embedded. We evaluate this approach in a sample of patients with bvFTD, AD, and normal controls. Results indicate that patients with bvFTD have emotion recognition deficits in all 3 categories of emotion compared to the other groups. These deficits were especially pronounced for negative and self-conscious emotions. Emotion recognition in this sample of patients with AD was indistinguishable from controls. These findings underscore the utility of this approach to assessing emotion recognition and suggest that previous findings that recognition of positive emotion was preserved in dementia patients may have resulted from the limited sampling of positive emotion in traditional tests. (c) 2015 APA, all rights reserved).

  9. Analysis of a case series of behavioral variant frontotemporal dementia: Emphasis on diagnostic delay

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    Henrique Cerqueira Guimarães

    Full Text Available ABSTRACT Introduction: Despite many advances in the characterization of the behavioral variant of frontotemporal dementia (bvFTD, the diagnosis of this syndrome poses a significant challenge, while delays or diagnostic mistakes may impact the proper clinical management of these patients. Objective: To describe the clinical profile at first evaluation of a sample of patients with bvFTD from a specialized outpatient neurological unit, with emphasis on the analysis of the delay between the onset of symptoms and diagnosis. Methods: We selected 31 patients that fulfilled international consensus criteria for possible or probable bvFTD. Patients' medical admission sheets were thoroughly reviewed. Results: Patients' mean age was 67.9±8.2 years; 16 (51.6% were men. Mean number of years of formal education was 7.7±4.0 years. Mean age at onset was 62.2±7.7 years, indicating a mean of 5.8 years of diagnostic delay. Thirteen patients (41.9% presented with initial behavioral complaints only, eleven patients (35.5% had mixed behavioral and memory complaints, five patients (16.1% presented with memory complaints only, and two patient (6.4% had behavioral and speech problems. Nine patients (29% were admitted with alternative diagnoses. Mean and standard deviation scores for the mini-mental state examination, animal category fluency and memory test for drawings (five-minute delayed recall were 19.3±6.3, 8.3±4.1 and 3.7±2.7, respectively. Conclusion: Most patients from this sample were evaluated almost six years after the onset of symptoms and performed poorly on both cognitive screening tests and functional evaluation measures.

  10. Episodic future thinking is impaired in the behavioural variant of frontotemporal dementia.

    Science.gov (United States)

    Irish, Muireann; Hodges, John R; Piguet, Olivier

    2013-10-01

    Remembering the past and imagining the future are complex endeavours proposed to rely on a core neurobiological brain system. In the behavioural variant of frontotemporal dementia (bvFTD), regional patterns of brain atrophy affect medial prefrontal and temporal cortices of this core network. While autobiographical memory impairments have been documented in bvFTD, it remains unknown whether the ability to imagine future events is also compromised. Here, we investigated episodic future thinking in 10 bvFTD patients and contrasted their performance with Alzheimer's disease (AD, n = 10) and healthy matched Control (n = 10) participants. Participants were asked to remember 3 events from the previous year and to envisage 3 possible events that could occur in the next year. Both patient groups showed equivalent episodic detail performance for the retrieval of past events and the simulation of possible future episodes. Patients with bvFTD, however, showed additional impairments for the generation of external (non-episodic) details irrespective of condition. Voxel-based morphometry analyses revealed divergent neural correlates of episodic past and future thinking performance specific to each patient group. Atrophy in the posterior cingulate cortex was implicated in the disruption of past and future thinking in AD. In contrast, in bvFTD, disruption of past retrieval correlated with atrophy in medial prefrontal regions, whereas future thinking deficits were associated with atrophy of frontopolar, medial temporal regions including the right hippocampus, and lateral temporal and occipital cortices. Our results point to the involvement of multiple brain regions in facilitating retrieval of past, and simulation of future, events. Damage to any of these key regions thus adversely affects the ability to engage in personally relevant mental time travel. Copyright © 2013 Elsevier Ltd. All rights reserved.

  11. Motor neuron disease and frontotemporal dementia: One, two, or three diseases?

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    Bak Thomas

    2010-10-01

    Full Text Available The relationship between motor neurone disease (MND and frontotemporal dementia (FTD has been a topic of scientific exploration for over hundred years. A connection between both diseases was first postulated in 1932 and has been strengthened by a steady stream of case reports since then. By the late 20th century, the link between both diseases was firmly established, with the resulting condition often referred to as MND/FTD. Several strands of evidence support the notion of an MND/FTD overlap. First, a small but well-documented group of patients present with a full-blown FTD, associated with MND. Second, subtle but characteristic changes in frontal-executive functions and social cognition have been described in non-demented MND patients, often in association with frontal atrophy/hypoactivity on neuroimaging. Third, amyotrophic features have been documented in patients primarily diagnosed with FTD. Moreover, the same genetic defect can lead to FTD and MND phenotypes in different members of the same family. However, as the current research is moving toward a more fine-grained evaluation, an increasingly complex picture begins to emerge. Some features, such as psychotic symptoms or severe language deficits (particularly in comprehension and verb processing, seem to occur more often in MND/dementia than in the classical FTD. On the basis of the review of 100 years of literature as well as 10 years of clinical experience of longitudinal follow-up of MND/dementia patients, this review argues in favor of MND/dementia (or, more precisely, MND/dementia/aphasia as a separate clinical entity, not sufficiently explained by a combination of MND and FTD.

  12. So Close Yet So Far: Executive Contribution to Memory Processing in Behavioral Variant Frontotemporal Dementia.

    Science.gov (United States)

    Bertoux, Maxime; Ramanan, Siddharth; Slachevsky, Andrea; Wong, Stephanie; Henriquez, Fernando; Musa, Gada; Delgado, Carolina; Flanagan, Emma; Bottlaender, Michel; Sarazin, Marie; Hornberger, Michael; Dubois, Bruno

    2016-10-04

    Memory impairment in behavioral variant frontotemporal dementia (bvFTD) is traditionally considered to be mild and attributed to prefrontal cortex dysfunction. Recent studies, however, indicated that some patients can present with a memory impairment of the hippocampal type, showing storage and consolidation deficits in addition to the more executive/prefrontal related encoding and strategic difficulties. This study aimed to study the relationship between executive functions (EF) and memory processes in bvFTD via a data-driven approach. Participants consisted of 71 bvFTD (among which 60.6% had a lumbar puncture showing non-Alzheimer biomarker profile) and 60 controls (among which 45% had amyloid imaging showing a normal profile). EF were assessed by the Frontal Assessment Battery, semantic/lexical verbal fluency tests, and forward/backward digit spans. Patients were split into amnestic (n = 33) and non-amnestic (n = 38) subgroups based on normative data (total recall score) from the Free and Cued Selective Reminding Test (FCSRT). Relationships between FCSRT subscores and EF measures were explored through hierarchical clustering analysis, partial correlation analysis with an EF component, and automated linear modeling. Convergent findings across the statistical approaches show that, overall, memory performance was independent from EF in bvFTD whereas the relationship was stronger in controls. Indeed, in bvFTD, memory performance did not cluster with EF, was not correlated with the EF component, and was only partially (4% - 12.7%) predicted by EF. These findings show that executive dysfunctions cannot solely explain the memory deficits occurring in bvFTD. Indeed, some patients present with a genuine amnesia affecting storage and consolidation abilities, which are independent from executive dysfunctions. On the clinical level, this study highlights the importance of revising the neuropsychological diagnosis criteria for bvFTD.

  13. Strategic value-directed learning and memory in Alzheimer's disease and behavioural-variant frontotemporal dementia.

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    Wong, Stephanie; Irish, Muireann; Savage, Greg; Hodges, John R; Piguet, Olivier; Hornberger, Michael

    2018-02-12

    In healthy adults, the ability to prioritize learning of highly valued information is supported by executive functions and enhances subsequent memory retrieval for this information. In Alzheimer's disease (AD) and behavioural-variant frontotemporal dementia (bvFTD), marked deficits are evident in learning and memory, presenting in the context of executive dysfunction. It is unclear whether these patients show a typical memory bias for higher valued stimuli. We administered a value-directed word-list learning task to AD (n = 10) and bvFTD (n = 21) patients and age-matched healthy controls (n = 22). Each word was assigned a low, medium or high point value, and participants were instructed to maximize the number of points earned across three learning trials. Participants' memory for the words was assessed on a delayed recall trial, followed by a recognition test for the words and corresponding point values. Relative to controls, both patient groups showed poorer overall learning, delayed recall and recognition. Despite these impairments, patients with AD preferentially recalled high-value words on learning trials and showed significant value-directed enhancement of recognition memory for the words and points. Conversely, bvFTD patients did not prioritize recall of high-value words during learning trials, and this reduced selectivity was related to inhibitory dysfunction. Nonetheless, bvFTD patients showed value-directed enhancement of recognition memory for the point values, suggesting a mismatch between memory of high-value information and the ability to apply this in a motivationally salient context. Our findings demonstrate that value-directed enhancement of memory may persist to some degree in patients with dementia, despite pronounced deficits in learning and memory. © 2018 The British Psychological Society.

  14. Hypersexual Behavior in Frontotemporal Dementia: A Comparison with Early-Onset Alzheimer’s Disease

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    Mendez, Mario F.; Shapira, Jill S.

    2013-01-01

    The basis of hypersexual behavior among patients with dementia is not entirely clear. Hypersexual behavior may be a particular feature of behavioral variant frontotemporal dementia (bvFTD), which affects ventromedial frontal and adjacent anterior temporal regions specialized in interpersonal behavior. Recent efforts to define Hypersexual Disorder indicate an increasing awareness of heightened sexual activity as a source of personal distress and functional impairment, and clarification of hypersexuality in bvFTD could contribute to understanding the neurobiology of this behavior. This study reviewed 47 patients with bvFTD compared to 58 patients with Alzheimer’s disease (AD) for the presence of heightened sexual activity to the point of distress to caregivers and others. Hypersexual behavior occurred in 6 (13%) bvFTD patients compared to none of the AD patients. Caregivers judged all six bvFTD patients with hypersexual behavior as having a dramatic increase in sexual frequency from premorbid levels. All had general disinhibition, poor impulse control, and actively sought sexual stimulation. They had widened sexual interests and experienced sexual arousal from previously unexciting stimuli. One patient, with early and predominant right anterior temporal involvement, was easily aroused by slight stimuli, such as touching her palms. Although previously considered to be predominantly disinhibited sexual behavior as part of generalized disinhibition, these patients with dementia illustrate varying degrees of increased sexual desire. We conclude that bvFTD is uniquely associated with hypersexuality; it is more than just cognitive impairment with frontal disinhibition but also involves alterations in sexual drive, possibly from right anterior temporal-limbic involvement in this disease. PMID:23297146

  15. A tale of two hemispheres: Contrasting socioemotional dysfunction in right- versus left-lateralised semantic dementia

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    Muireann Irish

    Full Text Available ABSTRACT Objective: Semantic dementia, a subtype of frontotemporal lobar degeneration, is characterised by cross-modal loss of conceptual knowledge attributable to progressive degeneration of the left anterior temporal lobe. Much less is known regarding the clinical presentation of SD patients with predominantly right-lateralised atrophy. Recent reports emphasise marked socioemotional and behavioural disturbances in such cases. Given the importance of the right anterior temporal lobes in social cognition, we hypothesised that socioemotional functioning would be disproportionately affected in right versus left-lateralised SD cases. Methods: We assessed well-characterised cases of predominantly right (n=10 and left (n=12 SD and 20 matched healthy controls on tests of emotion processing and interpersonal functioning. Results: Right SD cases showed disproportionate difficulties in the recognition of positive and negative facial emotions, specifically happiness and anger, compared with left SD cases. Deficits in anger recognition persisted in right SD despite covarying for facial and semantic processing. On a contextually rich task of emotion recognition using multimodal videos, no subgroup differences were evident. Finally, empathic concern was rated as significantly lower by caregivers of right versus left SD cases. Overall, the extent of socioemotional disturbance was associated with the degree of behavioural changes in SD. Conclusion: Our results reveal considerable overlap in the extent to which socioemotional processes are disrupted in left and right-lateralised cases of SD. Notably, however, right SD cases show disproportionate deficits for recognition of facial emotions and the capacity for empathic concern, supporting a specialised role for the right anterior temporal lobes in mediating these cognitive functions.

  16. Non-compact left ventricle/hypertrabeculated left ventricle

    International Nuclear Information System (INIS)

    Restrepo, Gustavo; Castano, Rafael; Marmol, Alejandro

    2005-01-01

    Non-compact left ventricle/hypertrabeculated left ventricle is a myocardiopatie produced by an arrest of the normal left ventricular compaction process during the early embryogenesis. It is associated to cardiac anomalies (congenital cardiopaties) as well as to extracardial conditions (neurological, facial, hematologic, cutaneous, skeletal and endocrinological anomalies). This entity is frequently unnoticed, being diagnosed only in centers with great experience in the diagnosis and treatment of myocardiopathies. Many cases of non-compact left ventricle have been initially misdiagnosed as hypertrophic myocardiopatie, endocardial fibroelastosis, dilated cardiomyopatie, restrictive cardiomyopathy and endocardial fibrosis. It is reported the case of a 74 years old man with a history of chronic arterial hypertension and diabetes mellitus, prechordial chest pain and mild dyspnoea. An echocardiogram showed signs of non-compact left ventricle with prominent trabeculations and deep inter-trabecular recesses involving left ventricular apical segment and extending to the lateral and inferior walls. Literature on this topic is reviewed

  17. Presymptomatic cerebral blood flow changes in CHMP2B mutation carriers of familial frontotemporal dementia (FTD-3), measured with MRI

    DEFF Research Database (Denmark)

    Lunau, Line Andersen; Mouridsen, Kim; Rodell, Anders

    2012-01-01

    OBJECTIVES: To assess functional changes measured by cerebral blood flow (CBF) in the presymptomatic stage of frontotemporal dementia linked to chromosome 3 (FTD-3) caused by a truncating mutation in CHMP2B. DESIGN: Case-control study. SETTING: A memory clinic and tertiary referrals centre...... changes in brain tissue perfusion were measured as CBF with two different MR techniques, gradient echo (GRE) and spin echo (SE), focusing on CBF in all cerebral vessels (GRE) and cerebral capillaries (SE), respectively. As planned, data analysis included co-registration of perfusion images to structural T...... indicate that FTD-3 vascular pathology might primarily affect brain capillaries....

  18. Cognitive reserve and TMEM106B genotype modulate brain damage in presymptomatic frontotemporal dementia: a GENFI study.

    Science.gov (United States)

    Premi, Enrico; Grassi, Mario; van Swieten, John; Galimberti, Daniela; Graff, Caroline; Masellis, Mario; Tartaglia, Carmela; Tagliavini, Fabrizio; Rowe, James B; Laforce, Robert; Finger, Elizabeth; Frisoni, Giovanni B; de Mendonça, Alexandre; Sorbi, Sandro; Gazzina, Stefano; Cosseddu, Maura; Archetti, Silvana; Gasparotti, Roberto; Manes, Marta; Alberici, Antonella; Cardoso, Manuel J; Bocchetta, Martina; Cash, David M; Ourselin, Sebastian; Padovani, Alessandro; Rohrer, Jonathan D; Borroni, Barbara

    2017-06-01

    Frontotemporal dementia is a heterogeneous neurodegenerative disorder with around a third of cases having autosomal dominant inheritance. There is wide variability in phenotype even within affected families, raising questions about the determinants of the progression of disease and age at onset. It has been recently demonstrated that cognitive reserve, as measured by years of formal schooling, can counteract the ongoing pathological process. The TMEM106B genotype has also been found to be a modifier of the age at disease onset in frontotemporal dementia patients with TDP-43 pathology. This study therefore aimed to elucidate the modulating effect of environment (i.e. cognitive reserve as measured by educational attainment) and genetic background (i.e. TMEM106B polymorphism, rs1990622 T/C) on grey matter volume in a large cohort of presymptomatic subjects bearing frontotemporal dementia-related pathogenic mutations. Two hundred and thirty-one participants from the GENFI study were included: 108 presymptomatic MAPT, GRN, and C9orf72 mutation carriers and 123 non-carriers. For each subject, cortical and subcortical grey matter volumes were generated using a parcellation of the volumetric T1-weighted magnetic resonance imaging brain scan. TMEM106B genotyping was carried out, and years of education recorded. First, we obtained a composite measure of grey matter volume by graph-Laplacian principal component analysis, and then fitted a linear mixed-effect interaction model, considering the role of (i) genetic status; (ii) educational attainment; and (iii) TMEM106B genotype on grey matter volume. The presence of a mutation was associated with a lower grey matter volume (P = 0.002), even in presymptomatic subjects. Education directly affected grey matter volume in all the samples (P = 0.02) with lower education attainment being associated with lower volumes. TMEM106B genotype did not influence grey matter volume directly on its own but in mutation carriers it modulated the

  19. Patient iPSC-derived neurons for disease modeling of frontotemporal dementia with mutation in CHMP2B

    DEFF Research Database (Denmark)

    Zhang, Yu; Schmid, Benjamin; Nikolaisen, Nanett Kvist

    2017-01-01

    The truncated mutant form of the charged multivesicular body protein 2B (CHMP2B) is causative for frontotemporal dementia linked to chromosome 3 (FTD3). CHMP2B is a constituent of the endosomal sorting complex required for transport (ESCRT) and, when mutated, disrupts endosome-to-lysosome traffic...... in neurodegenerative diseases. All phenotypes observed in FTD3 neurons were rescued in CRISPR/Cas9-edited isogenic controls. These findings illustrate the relevance of our patient-specific in vitro models and open up possibilities for drug target development....

  20. Mechanical discordance between left atrium and left atrial appendage

    Directory of Open Access Journals (Sweden)

    Arash Khamooshian

    2018-01-01

    Full Text Available During standard transesophageal echocardiographic examinations in sinus rhythm (SR patients, the left atrial appendage (LAA is not routinely assessed with Doppler. Despite having a SR, it is still possible to have irregular activity in the LAA. This situation is even more important for SR patients where assessment of the left atrium is often foregone. We describe a case where we encountered this situation and briefly review how to assess the left atrium and its appendage in such a case scenario.

  1. [Left-handedness and health].

    Science.gov (United States)

    Milenković, Sanja; Belojević, Goran; Kocijancić, Radojka

    2010-01-01

    Hand dominance is defined as a proneness to use one hand rather than another in performing the majority of activities and this is the most obvious example of cerebral lateralization and an exclusive human characteristic. Left-handed people comprise 6-14% of the total population, while in Serbia, this percentage is 5-10%, moving from undeveloped to developed environments, where a socio-cultural pressure is less present. There is no agreement between investigators who in fact may be considered a left-handed person, about the percentage of left-handers in the population and about the etiology of left-handedness. In the scientific literature left-handedness has been related to health disorders (spine deformities, immunological disorders, migraine, neurosis, depressive psychosis, schizophrenia, insomnia, homosexuality, diabetes mellitus, arterial hypertension, sleep apnea, enuresis nocturna and Down Syndrome), developmental disorders (autism, dislexia and sttutering) and traumatism. The most reliable scientific evidences have been published about the relationship between left-handedness and spinal deformities in school children in puberty and with traumatism in general population. The controversy of other results in up-to-now investigations of health aspects of left-handedness may partly be explained by a scientific disagreement whether writing with the left hand is a sufficient criterium for left-handedness, or is it necessary to investigate other parameters for laterality assessment. Explanation of health aspects of left-handedness is dominantly based on Geschwind-Galaburda model about "anomalous" cerebral domination, as a consequence of hormonal disbalance.

  2. Hits to the left, flops to the right: different emotions during listening to music are reflected in cortical lateralisation patterns.

    Science.gov (United States)

    Altenmüller, Eckart; Schürmann, Kristian; Lim, Vanessa K; Parlitz, Dietrich

    2002-01-01

    In order to investigate the neurobiological mechanisms accompanying emotional valence judgements during listening to complex auditory stimuli, cortical direct current (dc)-electroencephalography (EEG) activation patterns were recorded from 16 right-handed students. Students listened to 160 short sequences taken from the repertoires of jazz, rock-pop, classical music and environmental sounds (each n=40). Emotional valence of the perceived stimuli were rated on a 5-step scale after each sequence. Brain activation patterns during listening revealed widespread bilateral fronto-temporal activation, but a highly significant lateralisation effect: positive emotional attributions were accompanied by an increase in left temporal activation, negative by a more bilateral pattern with preponderance of the right fronto-temporal cortex. Female participants demonstrated greater valence-related differences than males. No differences related to the four stimulus categories could be detected, suggesting that the actual auditory brain activation patterns were more determined by their affective emotional valence than by differences in acoustical "fine" structure. The results are consistent with a model of hemispheric specialisation concerning perceived positive or negative emotions proposed by Heilman [Journal of Neuropsychiatry and Clinical Neuroscience 9 (1997) 439].

  3. [Emotion and Brodmann's areas: special reference on area 12].

    Science.gov (United States)

    Kawamura, Mitsuru

    2010-11-01

    Brodmann's brain maps, assembled in 1909, are still in use, but understanding of their animal-human homology is uncertain. Furthermore, in 1909, Brodmann did not identify human Area 12 (BA12); a location now important to understanding of frontotemporal lobar degeneration (FTLD) and emotional function. We found Brodmann did identify human BA12 in later maps (1910 and 1914), not in the 1909 monograph. Because of its current link with FTLD, BA 12's translation from animal (1909) to human (1910 and 1914) is not only an historical puzzle. It impacts how Brodmann's areas, based on comparative animal-human cyto-architecture, are widely used in current research as functional loci in human brain. If Brodmann's maps are of current value, then an accurate rather than a generic Brodmann number is in order.

  4. Telephone-Based Cognitive-Behavioral Screening for Frontotemporal Changes in Patients with Amyotrophic Lateral Sclerosis (ALS)

    Science.gov (United States)

    Christodoulou, Georgia; Gennings, Chris; Hupf, Jonathan; Factor-Litvak, Pam; Murphy, Jennifer; Goetz, Raymond R.; Mitsumoto, Hiroshi

    2017-01-01

    Objective To establish a valid and reliable battery of measures to evaluate frontotemporal dementia (FTD) in patients with ALS over the phone. Methods Thirty-one subjects were administered either in-person or telephone-based screening followed by the opposite mode of testing two weeks later, using a modified version of the UCSF Cognitive Screening Battery. Results Equivalence testing was performed for in-person and telephone-based tests. The standard ALS Cognitive Behavioral Screen (ALS-CBS) showed statistical equivalence at the 5% significance level when compared to a revised phone-version of the ALS-CBS. In addition, the Controlled Oral Word Association Test (COWAT) and Center for Neurologic Study-Lability Scale (CNS-LS) were also found to be equivalent at the 5% and 10% significance level respectively. Similarly, the Mini-Mental State Examination (MMSE) and the well-established Telephone Interview for Cognitive Status (TICS) were also statistically equivalent. Equivalence could not be claimed for the ALS-Frontal Behavioral Inventory (ALS-FBI) caregiver interview and the Written Verbal Fluency Index (WVFI). Conclusions Our study suggests that telephone-based versions of the ALS-CBS, COWAT, and CNS-LS may offer clinicians valid tools to detect frontotemporal changes in the ALS population. Development of telephone-based cognitive testing for ALS could become an integral resource for population-based research in the future. PMID:27121545

  5. Globular Glial Mixed Four Repeat Tau and TDP-43 Proteinopathy with Motor Neuron Disease and Frontotemporal Dementia.

    Science.gov (United States)

    Takeuchi, Ryoko; Toyoshima, Yasuko; Tada, Mari; Tanaka, Hidetomo; Shimizu, Hiroshi; Shiga, Atsushi; Miura, Takeshi; Aoki, Kenju; Aikawa, Akane; Ishizawa, Shin; Ikeuchi, Takeshi; Nishizawa, Masatoyo; Kakita, Akiyoshi; Takahashi, Hitoshi

    2016-01-01

    Amyotrophic lateral sclerosis (ALS) may be accompanied by frontotemporal dementia (FTD). We report a case of glial mixed tau and TDP-43 proteinopathies in a Japanese patient diagnosed clinically as having ALS-D. Autopsy revealed loss of lower motor neurons and degeneration of the pyramidal tracts in the spinal cord and brain stem. The brain showed frontotemporal lobar degeneration (FTLD), the most severe neuronal loss and gliosis being evident in the precentral gyrus. Although less severe, such changes were also observed in other brain regions, including the basal ganglia and substantia nigra. AT8 immunostaining revealed that predominant occurrence of astrocytic tau lesions termed globular astrocytic inclusions (GAIs) was a feature of the affected regions. These GAIs were Gallyas-Braak negative. Neuronal and oligodendrocytic tau lesions were comparatively scarce. pS409/410 immunostaining also revealed similar neuronal and glial TDP-43 lesions. Interestingly, occasional co-localization of tau and TDP-43 was evident in the GAIs. Immunoblot analyses revealed band patterns characteristic of a 4-repeat (4R) tauopathy, corticobasal degeneration and a TDP-43 proteinopathy, ALS/FTLD-TDP Type B. No mutations were found in the MAPT or TDP-43 genes. We consider that this patient harbored a distinct, sporadic globular glial mixed 4R tau and TDP-43 proteinopathy associated with motor neuron disease and FTD. © 2015 International Society of Neuropathology.

  6. Adaptation and validation of a Spanish-language version of the Frontotemporal Dementia Rating Scale (FTD-FRS).

    Science.gov (United States)

    Turró-Garriga, O; Hermoso Contreras, C; Olives Cladera, J; Mioshi, E; Pelegrín Valero, C; Olivera Pueyo, J; Garre-Olmo, J; Sánchez-Valle, R

    2017-06-01

    The Frontotemporal Dementia Rating Scale (FTD-FRS) is a tool designed to aid with clinical staging and assessment of the progression of frontotemporal dementia (FTD-FRS). Present a multicentre adaptation and validation study of a Spanish version of the FRS. The adapted version was created using 2 translation-back translation processes (English to Spanish, Spanish to English) and verified by the scale's original authors. We validated the adapted version in a sample of consecutive patients diagnosed with FTD. The procedure included evaluating internal consistency, testing unidimensionality with the Rasch model, analysing construct validity and discriminant validity, and calculating the degree of agreement between the Clinical Dementia Rating scale (CDR) and FTD-FRS for FTD cases. The study included 60 patients with DFT. The mean score on the FRS was 12.1 points (SD=6.5; range, 2-25) with inter-group differences (F=120.3; df=3; Pde Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  7. Familial frontotemporal dementia with neuronal intranuclear inclusions is not a polyglutamine expansion disease

    Directory of Open Access Journals (Sweden)

    Neal Scott J

    2006-08-01

    Full Text Available Abstract Background Many cases of frontotemporal dementia (FTD are familial, often with an autosomal dominant pattern of inheritance. Some are due to a mutation in the tau- encoding gene, on chromosome 17, and show an accumulation of abnormal tau in brain tissue (FTDP-17T. Most of the remaining familial cases do not exhibit tau pathology, but display neuropathology similar to patients with dementia and motor neuron disease, characterized by the presence of ubiquitin-immunoreactive (ub-ir, dystrophic neurites and neuronal cytoplasmic inclusions in the neocortex and hippocampus (FTLD-U. Recently, we described a subset of patients with familial FTD with autopsy-proven FTLD-U pathology and with the additional finding of ub-ir neuronal intranuclear inclusions (NII. NII are a characteristic feature of several other neurodegenerative conditions for which the genetic basis is abnormal expansion of a polyglutamine-encoding trinucleotide repeat region. The genetic basis of familial FTLD-U is currently not known, however the presence of NII suggests that a subset of cases may represent a polyglutamine expansion disease. Methods We studied DNA and post mortem brain tissue from 5 affected members of 4 different families with NII and one affected individual with familial FTLD-U without NII. Patient DNA was screened for CAA/CAG trinucleotide expansion in a set of candidate genes identified using a genome-wide computational approach. Genes containing CAA/CAG trinucleotide repeats encoding at least five glutamines were examined (n = 63, including the nine genes currently known to be associated with human disease. CAA/CAG tract sizes were compared with published normal values (where available and with those of healthy controls (n = 94. High-resolution agarose gel electrophoresis was used to measure allele size (number of CAA/CAG repeats. For any alleles estimated to be equal to or larger than the maximum measured in the control population, the CAA/CAG tract

  8. Frontotemporal dementia and its subtypes: a genome-wide association study

    Science.gov (United States)

    Ferrari, Raffaele; Hernandez, Dena G; Nalls, Michael A; Rohrer, Jonathan D; Ramasamy, Adaikalavan; Kwok, John B J; Dobson-Stone, Carol; Brooks, William S; Schofield, Peter R; Halliday, Glenda M; Hodges, John R; Piguet, Olivier; Bartley, Lauren; Thompson, Elizabeth; Haan, Eric; Hernández, Isabel; Ruiz, Agustín; Boada, Mercè; Borroni, Barbara; Padovani, Alessandro; Cruchaga, Carlos; Cairns, Nigel J; Benussi, Luisa; Binetti, Giuliano; Ghidoni, Roberta; Forloni, Gianluigi; Galimberti, Daniela; Fenoglio, Chiara; Serpente, Maria; Scarpini, Elio; Clarimón, Jordi; Lleó, Alberto; Blesa, Rafael; Waldö, Maria Landqvist; Nilsson, Karin; Nilsson, Christer; Mackenzie, Ian R A; Hsiung, Ging-Yuek R; Mann, David M A; Grafman, Jordan; Morris, Christopher M; Attems, Johannes; Griffiths, Timothy D; McKeith, Ian G; Thomas, Alan J; Pietrini, P; Huey, Edward D; Wassermann, Eric M; Baborie, Atik; Jaros, Evelyn; Tierney, Michael C; Pastor, Pau; Razquin, Cristina; Ortega-Cubero, Sara; Alonso, Elena; Perneczky, Robert; Diehl-Schmid, Janine; Alexopoulos, Panagiotis; Kurz, Alexander; Rainero, Innocenzo; Rubino, Elisa; Pinessi, Lorenzo; Rogaeva, Ekaterina; George-Hyslop, Peter St; Rossi, Giacomina; Tagliavini, Fabrizio; Giaccone, Giorgio; Rowe, James B; Schlachetzki, J C M; Uphill, James; Collinge, John; Mead, S; Danek, Adrian; Van Deerlin, Vivianna M; Grossman, Murray; Trojanowsk, John Q; van der Zee, Julie; Deschamps, William; Van Langenhove, Tim; Cruts, Marc; Van Broeckhoven, Christine; Cappa, Stefano F; Le Ber, Isabelle; Hannequin, Didier; Golfier, Véronique; Vercelletto, Martine; Brice, Alexis; Nacmias, Benedetta; Sorbi, Sandro; Bagnoli, Silvia; Piaceri, Irene; Nielsen, Jørgen E; Hjermind, Lena E; Riemenschneider, Matthias; Mayhaus, Manuel; Ibach, Bernd; Gasparoni, Gilles; Pichler, Sabrina; Gu, Wei; Rossor, Martin N; Fox, Nick C; Warren, Jason D; Spillantini, Maria Grazia; Morris, Huw R; Rizzu, Patrizia; Heutink, Peter; Snowden, Julie S; Rollinson, Sara; Richardson, Anna; Gerhard, Alexander; Bruni, Amalia C; Maletta, Raffaele; Frangipane, Francesca; Cupidi, Chiara; Bernardi, Livia; Anfossi, Maria; Gallo, Maura; Conidi, Maria Elena; Smirne, Nicoletta; Rademakers, Rosa; Baker, Matt; Dickson, Dennis W; Graff-Radford, Neill R; Petersen, Ronald C; Knopman, David; Josephs, Keith A; Boeve, Bradley F; Parisi, Joseph E; Seeley, William W; Miller, Bruce L; Karydas, Anna M; Rosen, Howard; van Swieten, John C; Dopper, Elise G P; Seelaar, Harro; Pijnenburg, Yolande AL; Scheltens, Philip; Logroscino, Giancarlo; Capozzo, Rosa; Novelli, Valeria; Puca, Annibale A; Franceschi, M; Postiglione, Alfredo; Milan, Graziella; Sorrentino, Paolo; Kristiansen, Mark; Chiang, Huei-Hsin; Graff, Caroline; Pasquier, Florence; Rollin, Adeline; Deramecourt, Vincent; Lebert, Florence; Kapogiannis, Dimitrios; Ferrucci, Luigi; Pickering-Brown, Stuart; Singleton, Andrew B; Hardy, John; Momeni, Parastoo

    2014-01-01

    Summary Background Frontotemporal dementia (FTD) is a complex disorder characterised by a broad range of clinical manifestations, differential pathological signatures, and genetic variability. Mutations in three genes—MAPT, GRN, and C9orf72—have been associated with FTD. We sought to identify novel genetic risk loci associated with the disorder. Methods We did a two-stage genome-wide association study on clinical FTD, analysing samples from 3526 patients with FTD and 9402 healthy controls. All participants had European ancestry. In the discovery phase (samples from 2154 patients with FTD and 4308 controls), we did separate association analyses for each FTD subtype (behavioural variant FTD, semantic dementia, progressive non-fluent aphasia, and FTD overlapping with motor neuron disease [FTD-MND]), followed by a meta-analysis of the entire dataset. We carried forward replication of the novel suggestive loci in an independent sample series (samples from 1372 patients and 5094 controls) and then did joint phase and brain expression and methylation quantitative trait loci analyses for the associated (p<5 × 10−8) and suggestive single-nucleotide polymorphisms. Findings We identified novel associations exceeding the genome-wide significance threshold (p<5 × 10−8) that encompassed the HLA locus at 6p21.3 in the entire cohort. We also identified a potential novel locus at 11q14, encompassing RAB38/CTSC, for the behavioural FTD subtype. Analysis of expression and methylation quantitative trait loci data suggested that these loci might affect expression and methylation incis. Interpretation Our findings suggest that immune system processes (link to 6p21.3) and possibly lysosomal and autophagy pathways (link to 11q14) are potentially involved in FTD. Our findings need to be replicated to better define the association of the newly identified loci with disease and possibly to shed light on the pathomechanisms contributing to FTD. Funding The National Institute of

  9. Myxoma of the Left Ventricle

    Science.gov (United States)

    Novoa, José; Delgado, Antonio; Alonso, Ana

    2014-01-01

    This report concerns a 69-year-old woman who presented with an asymptomatic myxoma in the left ventricle. The tumor was successfully excised. We provide a very brief review of 72 other published cases of surgically treated left ventricular myxoma. PMID:25120392

  10. Left ventricular hypertrophy in athletes.

    Science.gov (United States)

    Douglas, P S; O'Toole, M L; Katz, S E; Ginsburg, G S; Hiller, W D; Laird, R H

    1997-11-15

    Left ventricular wall thickness >1.3 cm, septal-to-posterior wall ratios > 1.5, diastolic left ventricular size >6.0 cm, and eccentric or concentric remodeling are rare in athletes. Values outside of these cutoffs in an athlete of any age probably represent a pathologic state.

  11. The Left-Handed Writer.

    Science.gov (United States)

    Bloodsworth, James Gaston

    Contrary to the beliefs of many, right-handedness is not a single factor existing in almost all people, with a few exceptions termed left-handed: neither extreme exists independently of the other. During the first 4 years of life there is a period of fluctuation between right and left-handed dominance. Statistics and findings vary in determining…

  12. Two Lefts in Latin America?

    DEFF Research Database (Denmark)

    Christensen, Steen Fryba

    In this working paper I list five researchers' categorizations of the Latin American left in power (april 2006) in a schematic form. The most important criteria for the categorizations are given.......In this working paper I list five researchers' categorizations of the Latin American left in power (april 2006) in a schematic form. The most important criteria for the categorizations are given....

  13. A Giant Left Atrial Myxoma

    Directory of Open Access Journals (Sweden)

    Medhat F. Zaher

    2014-01-01

    Full Text Available Atrial myxomas are the most common primary cardiac tumors. Patients with left atrial myxomas generally present with mechanical obstruction of blood flow, systemic embolization, and constitutional symptoms. We present a case of an unusually large left atrial myxoma discovered incidentally in a patient with longstanding dyspnea being managed as bronchial asthma.

  14. Induced pluripotent stem cells (iPSCs) derived from a patient with frontotemporal dementia caused by a R406W mutation in microtubule-associated protein tau (MAPT)

    DEFF Research Database (Denmark)

    Rasmussen, Mikkel A.; Hjermind, Lena E.; Hasholt, Lis F.

    2016-01-01

    Skin fibroblasts were obtained from a 59-year-old woman diagnosed with frontotemporal dementia. The disease is caused by a R406W mutation in microtubule-associated protein tau (MAPT). Induced pluripotent stem cells (iPSCs) were established by electroporation with episomal plasmids containing hOCT4...

  15. The role of TREM2 R47H as a risk factor for Alzheimer's disease, frontotemporal lobar degeneration, amyotrophic lateral sclerosis, and Parkinson's disease

    DEFF Research Database (Denmark)

    Lill, Christina M; Rengmark, Aina; Pihlstrøm, Lasse

    2015-01-01

    A rare variant in TREM2 (p.R47H, rs75932628) was recently reported to increase the risk of Alzheimer's disease (AD) and, subsequently, other neurodegenerative diseases, i.e. frontotemporal lobar degeneration (FTLD), amyotrophic lateral sclerosis (ALS), and Parkinson's disease (PD). Here we...

  16. Caregiver burden, health-related quality of life and coping in dementia caregivers: a comparison of frontotemporal dementia and Alzheimer's disease.

    NARCIS (Netherlands)

    Riedijk, S.R.; Vugt, M.E. de; Duivenvoorden, H.J.; Niermeijer, M.F.; Swieten, J. van; Verhey, F.R.J.; Tibben, A.

    2006-01-01

    Frontotemporal dementia (FTD) is the second most prevalent dementia after Alzheimer's disease (AD). We compared 29 FTD and 90 AD caregivers with respect to burden, health-related quality of life (HQoL) and coping. FTD caregivers were more burdened than AD caregivers, and caregivers of patients who

  17. Head to head comparison of [18F] AV-1451 and [18F] THK5351 for tau imaging in Alzheimer's disease and frontotemporal dementia.

    Science.gov (United States)

    Jang, Young Kyoung; Lyoo, Chul Hyoung; Park, Seongbeom; Oh, Seung Jun; Cho, Hanna; Oh, Minyoung; Ryu, Young Hoon; Choi, Jae Yong; Rabinovici, Gil D; Kim, Hee Jin; Moon, Seung Hwan; Jang, Hyemin; Lee, Jin San; Jagust, William J; Na, Duk L; Kim, Jae Seung; Seo, Sang Won

    2018-03-01

    Tau accumulation is a core pathologic change in various neurodegenerative diseases including Alzheimer's disease and frontotemporal lobar degeneration-tau. Recently, tau positron emission tomography tracers such as [ 18 F] AV-1451 and [ 18 F] THK5351 have been developed to detect tau deposition in vivo. In the present study, we performed a head to head comparison of these two tracers in Alzheimer's disease and frontotemporal dementia cases and aimed to investigate which tracers are better suited to image tau in these disorders. A cross-sectional study was conducted using a hospital-based sample at a tertiary referral center. We recruited eight participants (two Alzheimer's disease, four frontotemporal dementia and two normal controls) who underwent magnetic resonance image, amyloid positron emission tomography with [ 18 F]-Florbetaben and tau positron emission tomography with both THK5351 and AV-1451. To measure regional AV1451 and THK5351 uptakes, we used the standardized uptake value ratios by dividing mean activity in target volume of interest by mean activity in the cerebellar hemispheric gray matter. Although THK5351 and AV-1451 uptakes were highly correlated, cortical uptake of AV-1451 was more striking in Alzheimer's disease, while cortical uptake of THK5351 was more prominent in frontotemporal dementia. THK5351 showed higher off-target binding than AV-1451 in the white matter, midbrain, thalamus, and basal ganglia. AV-1451 is more sensitive and specific to Alzheimer's disease type tau and shows lower off-target binding, while THK5351 may mirror non-specific neurodegeneration.

  18. Frontotemporal dementia linked to chromosome 3 (FTD-3)--current concepts and the detection of a previously unknown branch of the Danish FTD-3 family

    DEFF Research Database (Denmark)

    Lindquist, S.G.; Braedgaard, H.; Svenstrup, K.

    2008-01-01

    BACKGROUND: Among patients with onset of dementia below the age of 65 years, frontotemporal dementia (FTD) is the second most prevalent cause, secondary only to Alzheimer's disease. Recent advances in understanding the heterogeneous genetic background for different clinical and neuropathological...... discuss recent advances and current concepts in the understanding of CHMP2B-related dementia Udgivelsesdato: 2008/7...

  19. Left-handedness and health

    Directory of Open Access Journals (Sweden)

    Milenković Sanja

    2010-01-01

    Full Text Available Hand dominance is defined as a proneness to use one hand rather than another in performing the majority of activities and this is the most obvious example of cerebral lateralization and an exclusive human characteristic. Left-handed people comprise 6-14% of the total population, while in Serbia, this percentage is 5-10%, moving from undeveloped to developed environments, where a socio-cultural pressure is less present. There is no agreement between investigators who in fact may be considered a left-handed person, about the percentage of left-handers in the population and about the etiology of left-handedness. In the scientific literature left-handedness has been related to health disorders (spine deformities, immunological disorders, migraine, neurosis, depressive psychosis, schizophrenia, insomnia, homosexuality, diabetes mellitus, arterial hypertension, sleep apnea, enuresis nocturna and Down Syndrome, developmental disorders (autism, dislexia and sttutering and traumatism. The most reliable scientific evidences have been published about the relationship between left-handedness and spinal deformities in school children in puberty and with traumatism in general population. The controversy of other results in up-to-now investigations of health aspects of left-handedness may partly be explained by a scientific disagreement whether writing with the left hand is a sufficient criterium for left-handedness, or is it necessary to investigate other parameters for laterality assessment. Explanation of health aspects of left-handedness is dominantly based on Geschwind-Galaburda model about 'anomalous' cerebral domination, as a consequence of hormonal disbalance. .

  20. Left ventricular wall stress compendium.

    Science.gov (United States)

    Zhong, L; Ghista, D N; Tan, R S

    2012-01-01

    Left ventricular (LV) wall stress has intrigued scientists and cardiologists since the time of Lame and Laplace in 1800s. The left ventricle is an intriguing organ structure, whose intrinsic design enables it to fill and contract. The development of wall stress is intriguing to cardiologists and biomedical engineers. The role of left ventricle wall stress in cardiac perfusion and pumping as well as in cardiac pathophysiology is a relatively unexplored phenomenon. But even for us to assess this role, we first need accurate determination of in vivo wall stress. However, at this point, 150 years after Lame estimated left ventricle wall stress using the elasticity theory, we are still in the exploratory stage of (i) developing left ventricle models that properly represent left ventricle anatomy and physiology and (ii) obtaining data on left ventricle dynamics. In this paper, we are responding to the need for a comprehensive survey of left ventricle wall stress models, their mechanics, stress computation and results. We have provided herein a compendium of major type of wall stress models: thin-wall models based on the Laplace law, thick-wall shell models, elasticity theory model, thick-wall large deformation models and finite element models. We have compared the mean stress values of these models as well as the variation of stress across the wall. All of the thin-wall and thick-wall shell models are based on idealised ellipsoidal and spherical geometries. However, the elasticity model's shape can vary through the cycle, to simulate the more ellipsoidal shape of the left ventricle in the systolic phase. The finite element models have more representative geometries, but are generally based on animal data, which limits their medical relevance. This paper can enable readers to obtain a comprehensive perspective of left ventricle wall stress models, of how to employ them to determine wall stresses, and be cognizant of the assumptions involved in the use of specific models.

  1. The Left Atrio-Vertebral Ratio: a new simple means for assessing left atrial enlargement on Computed Tomography.

    Science.gov (United States)

    Montillet, Marie; Baqué-Juston, Marie; Tasu, Jean-Pierre; Bertrand, Sandra; Berthier, Frédéric; Zarqane, Naïma; Brunner, Philippe

    2018-03-01

    The purpose of this study is to describe a new method to quickly estimate left atrial enlargement (LAE) on Computed Tomography. Left atrial (LA) volume was assessed with a 3D-threshold Hounsfield unit detection technique, including left atrial appendage and excluding pulmonary venous confluence, in 201 patients with ECG-gated 128-slice dual-source CT and indexed to body surface area. LA and vertebral axial diameter and area were measured at the bottom level of the right inferior pulmonary vein ostium. Ratio of LA diameter and surface on vertebra (LAVD and LAVA) were compared to LA volume. In accordance with the literature, a cutoff value of 78 ml/m 2 was chosen for maximal normal LA volume. 18% of LA was enlarged. The best cutoff values for LAE assessment were 2.5 for LAVD (AUC: 0.65; 95% CI: 0.58-0.73; sensitivity: 57%; specificity: 71%), and 3 for LAVA (AUC: 0.78; 95% CI: 0.72-0.84; sensitivity: 67%; specificity: 79%), with higher accuracy for LAVA (P=0.015). Inter-observer and intra-observer variability were either good or excellent for LAVD and LAVA (respective intraclass coefficients: 0.792 and 0.910; 0.912 and 0.937). A left atrium area superior to three times the vertebral area indicates LAE with high specificity. • Left atrial enlargement is a frequent condition associated with poor cardiac outcome. • Left atrial enlargement is highly time-consuming to diagnose on CT. • The left atrio-vertebral ratio quickly assesses left atrial enlargement. • A left atrial area > three times vertebral area is highly specific.

  2. [The Role of Brodmann Area 12: Taste, Social Cognition, and Mental Time].

    Science.gov (United States)

    Kawamura, Mitsuru

    2017-04-01

    Broadman area 12, together with area 11, is located in the orbitofrontal area. A voxel-based morphometric (VBM) study revealed the association between bilateral brodmann areas 12/47 and taste disturbance in individuals with frontotemporal lobar degeneration (FTLD). In our VBM study in patients with Parkinson's disease, decision-making impairments were associated with atrophy of the bilateral Brodmann area 12, indicating that this area may play an important role in social cognitive function. Our recent study also demonstrated that this area may serve as time order judgement or mental time travel.

  3. Left main percutaneous coronary intervention.

    Science.gov (United States)

    Teirstein, Paul S; Price, Matthew J

    2012-10-23

    The introduction of drug-eluting stents and advances in catheter techniques have led to increasing acceptance of percutaneous coronary intervention (PCI) as a viable alternative to coronary artery bypass graft (CABG) for unprotected left main disease. Current guidelines state that it is reasonable to consider unprotected left main PCI in patients with low to intermediate anatomic complexity who are at increased surgical risk. Data from randomized trials involving patients who are candidates for either treatment strategy provide novel insight into the relative safety and efficacy of PCI for this lesion subset. Herein, we review the current data comparing PCI with CABG for left main disease, summarize recent guideline recommendations, and provide an update on technical considerations that may optimize clinical outcomes in left main PCI. Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  4. Left bundle-branch block

    DEFF Research Database (Denmark)

    Risum, Niels; Strauss, David; Sogaard, Peter

    2013-01-01

    The relationship between myocardial electrical activation by electrocardiogram (ECG) and mechanical contraction by echocardiography in left bundle-branch block (LBBB) has never been clearly demonstrated. New strict criteria for LBBB based on a fundamental understanding of physiology have recently...

  5. Dabigatran for left ventricular thrombus

    Directory of Open Access Journals (Sweden)

    Satishkumar Kolekar

    2015-09-01

    Dabigatran is a reversible direct thrombin inhibitor and currently approved for the prevention of thromboembolic episodes in non-valvar atrial fibrillation. This case demonstrates possible thrombolytic properties of dabigatran in resolution of left ventricular thrombus.

  6. Left ventricular noncompaction in a patient presenting with a left ventricular failure

    Directory of Open Access Journals (Sweden)

    Ristić-Anđelkov Anđelka

    2018-01-01

    Full Text Available Introduction. Left ventricular noncompaction (LVNC is a congenital disorder characterised by prominent trabeculations in the left ventricular myocardium. This heart condition very often goes completely undetected, or is mistaken for hypertrophic cardiomyopathy or coronary disease. Case report. A middle-aged female with a positive family history of coronary disease was admitted with chest pain, electrocardiography (ECG changes in the area of the inferolateral wall and elevation in cardiac specific enzymes. Initially, she was suspected of having acute coronary syndrome. However, in the left ventricular apex, especially alongside the lateral and inferior walls, cardiac ultrasound visualised hypertrabeculation with multiple trabeculae projecting inside the left ventricular cavity. A short-axis view of the heart above the papillary muscles revealed the presence of two layers of the myocardium: a compacted homogeneous layer adjacent to the epicardium and a spongy layer with trabeculae and sinusoids under the endocardium. The thickness ratio between the two layers was 2.2:1. The same abnormalities were corroborated by multislice computed tomography (MSCT of the heart. Conclusion. Left ventricular noncompaction is a rare, usually hereditary cardiomyopathy, which should be considered as a possibility in patients with myocardial hypertrophy. It is very often mistaken for coronary disease owing to ECG changes and elevated cardiac specific enzymes associated with myocardial hypertrophy and heart failure.

  7. Immune-related genetic enrichment in frontotemporal dementia: An analysis of genome-wide association studies.

    Science.gov (United States)

    Broce, Iris; Karch, Celeste M; Wen, Natalie; Fan, Chun C; Wang, Yunpeng; Tan, Chin Hong; Kouri, Naomi; Ross, Owen A; Höglinger, Günter U; Muller, Ulrich; Hardy, John; Momeni, Parastoo; Hess, Christopher P; Dillon, William P; Miller, Zachary A; Bonham, Luke W; Rabinovici, Gil D; Rosen, Howard J; Schellenberg, Gerard D; Franke, Andre; Karlsen, Tom H; Veldink, Jan H; Ferrari, Raffaele; Yokoyama, Jennifer S; Miller, Bruce L; Andreassen, Ole A; Dale, Anders M; Desikan, Rahul S; Sugrue, Leo P

    2018-01-01

    Converging evidence suggests that immune-mediated dysfunction plays an important role in the pathogenesis of frontotemporal dementia (FTD). Although genetic studies have shown that immune-associated loci are associated with increased FTD risk, a systematic investigation of genetic overlap between immune-mediated diseases and the spectrum of FTD-related disorders has not been performed. Using large genome-wide association studies (GWASs) (total n = 192,886 cases and controls) and recently developed tools to quantify genetic overlap/pleiotropy, we systematically identified single nucleotide polymorphisms (SNPs) jointly associated with FTD-related disorders-namely, FTD, corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), and amyotrophic lateral sclerosis (ALS)-and 1 or more immune-mediated diseases including Crohn disease, ulcerative colitis (UC), rheumatoid arthritis (RA), type 1 diabetes (T1D), celiac disease (CeD), and psoriasis. We found up to 270-fold genetic enrichment between FTD and RA, up to 160-fold genetic enrichment between FTD and UC, up to 180-fold genetic enrichment between FTD and T1D, and up to 175-fold genetic enrichment between FTD and CeD. In contrast, for CBD and PSP, only 1 of the 6 immune-mediated diseases produced genetic enrichment comparable to that seen for FTD, with up to 150-fold genetic enrichment between CBD and CeD and up to 180-fold enrichment between PSP and RA. Further, we found minimal enrichment between ALS and the immune-mediated diseases tested, with the highest levels of enrichment between ALS and RA (up to 20-fold). For FTD, at a conjunction false discovery rate < 0.05 and after excluding SNPs in linkage disequilibrium, we found that 8 of the 15 identified loci mapped to the human leukocyte antigen (HLA) region on Chromosome (Chr) 6. We also found novel candidate FTD susceptibility loci within LRRK2 (leucine rich repeat kinase 2), TBKBP1 (TBK1 binding protein 1), and PGBD5 (piggyBac transposable element

  8. Apraxia in left-handers.

    Science.gov (United States)

    Goldenberg, Georg

    2013-08-01

    In typical right-handed patients both apraxia and aphasia are caused by damage to the left hemisphere, which also controls the dominant right hand. In left-handed subjects the lateralities of language and of control of the dominant hand can dissociate. This permits disentangling the association of apraxia with aphasia from that with handedness. Pantomime of tool use, actual tool use and imitation of meaningless hand and finger postures were examined in 50 consecutive left-handed subjects with unilateral hemisphere lesions. There were three aphasic patients with pervasive apraxia caused by left-sided lesions. As the dominant hand is controlled by the right hemisphere, they constitute dissociations of apraxia from handedness. Conversely there were also three patients with pervasive apraxia caused by right brain lesions without aphasia. They constitute dissociations of apraxia from aphasia. Across the whole group of patients dissociations from handedness and from aphasia were observed for all manifestations of apraxia, but their frequency depended on the type of apraxia. Defective pantomime and defective tool use occurred rarely without aphasia, whereas defective imitation of hand, but not finger, postures was more frequent after right than left brain damage. The higher incidence of defective imitation of hand postures in right brain damage was mainly due to patients who had also hemi-neglect. This interaction alerts to the possibility that the association of right hemisphere damage with apraxia has to do with spatial aptitudes of the right hemisphere rather than with its control of the dominant left hand. Comparison with data from right-handed patients showed no differences between the severity of apraxia for imitation of hand or finger postures, but impairment on pantomime of tool use was milder in apraxic left-handers than in apraxic right-handers. This alleviation of the severity of apraxia corresponded with a similar alleviation of the severity of aphasia as

  9. Left ventricular apical ballooning syndrome

    International Nuclear Information System (INIS)

    Rahman, N.; Tai, J.; Soofi, A.

    2007-01-01

    The transient left ventricular apical ballooning syndrome, also known as Takotsubo cardiomyopathy, is characterized by transient left ventricular dysfunction in the absence of obstructive epicardial coronary disease. Although the syndrome has been reported in Japan since 1990, it is rare in other regions. Rapid recognition of the syndrome can modify the diagnostic and therapeutic attitude i.e. avoiding thrombolysis and performing catheterization in the acute phase. (author)

  10. Left Main Coronary Artery Aneurysm

    Directory of Open Access Journals (Sweden)

    Hossein Doustkami

    2016-07-01

    Full Text Available Aneurysms of the left main coronary artery are exceedingly rare clinical entities, encountered incidentally in approximately 0.1% of patients who undergo routine angiography. The most common cause of coronary artery aneurysms is atherosclerosis. Angiography is the gold standard for diagnosis and treatment. Depending on the severity of the coexisting coronary stenosis, patients with left main coronary artery aneurysms can be effectively managed either surgically or pharmacologically. We herein report a case of left main coronary artery aneurysm in a 72-year-old man with a prior history of hypertension presenting to our hospital because of unstable angina. The electrocardiogram showed ST-segment depression and T-wave inversion in the precordial leads. All the data of blood chemistry were normal. Echocardiography showed akinetic anterior wall, septum, and apex, mild mitral regurgitation and ejection fraction of 45%. Coronary angiography revealed a saccular aneurysm of the left main coronary artery with significant stenosis in the left anterior descending, left circumflex, and right coronary artery. The patient immediately underwent coronary artery bypass grafting and ligation of the aneurysm. At six months’ follow-up, he remained asymptomatic.

  11. Right colon cancer: Left behind.

    Science.gov (United States)

    Gervaz, P; Usel, M; Rapiti, E; Chappuis, P; Neyroud-Kaspar, I; Bouchardy, C

    2016-09-01

    Prognosis of colon cancer (CC) has steadily improved during the past three decades. This trend, however, may vary according to proximal (right) or distal (left) tumor location. We studied if improvement in survival was greater for left than for right CC. We included all CC recorded at the Geneva population-based registry between 1980 and 2006. We compared patients, tumor and treatment characteristics between left and right CC by logistic regression and compared CC specific survival by Cox models taking into account putative confounders. We also compared changes in survival between CC location in early and late years of observation. Among the 3396 CC patients, 1334 (39%) had right-sided and 2062 (61%) left-sided tumors. In the early 1980s, 5-year specific survival was identical for right and left CCs (49% vs. 48%). During the study period, a dramatic improvement in survival was observed for patients with left-sided cancers (Hazard ratio [HR]: 0.42, 95% confidence interval [CI]: 0.29-0.62, p colon cancer patients, those with right-sided lesions have by far the worse prognosis. Change of strategic management in this subgroup is warranted. Copyright © 2016 Elsevier Ltd. All rights reserved.

  12. Definite behavioral variant of frontotemporal dementia with C9ORF72 expansions despite positive Alzheimer's disease cerebrospinal fluid biomarkers.

    Science.gov (United States)

    Wallon, David; Rovelet-Lecrux, Anne; Deramecourt, Vincent; Pariente, Jeremie; Auriacombe, Sophie; Le Ber, Isabelle; Schraen, Suzanna; Pasquier, Florence; Campion, Dominique; Hannequin, Didier

    2012-01-01

    Hexanucleotide expansion repeats in the C9ORF72 gene are a major cause of familial and, to a lesser extent, sporadic frontotemporal lobar degeneration (FTLD), amyotrophic lateral sclerosis (ALS), and FTLD-ALS. To examine whether C9ORF72 expansions could be involved in early-onset Alzheimer's disease (EOAD), we genotyped the hexanucleotide repeat region in a large cohort of 114 EOAD patients who all had positive AD cerebrospinal fluid (CSF) biomarkers. We found hexanucleotide expansion repeats of the C9ORF72 gene in 3 out of 114 patients (2.6%). We raise several hypotheses to explain our results and discuss the current status of AD CSF biomarkers in the dementia diagnostic algorithm.

  13. The Role of the Innate Immune System in Alzheimer’s Disease and Frontotemporal Lobar Degeneration: An Eye on Microglia

    Directory of Open Access Journals (Sweden)

    Elisa Ridolfi

    2013-01-01

    Full Text Available In the last few years, genetic and biomolecular mechanisms at the basis of Alzheimer’s disease (AD and frontotemporal lobar degeneration (FTLD have been unraveled. A key role is played by microglia, which represent the immune effector cells in the central nervous system (CNS. They are extremely sensitive to the environmental changes in the brain and are activated in response to several pathologic events within the CNS, including altered neuronal function, infection, injury, and inflammation. While short-term microglial activity has generally a neuroprotective role, chronic activation has been implicated in the pathogenesis of neurodegenerative disorders, including AD and FTLD. In this framework, the purpose of this review is to give an overview of clinical features, genetics, and novel discoveries on biomolecular pathogenic mechanisms at the basis of these two neurodegenerative diseases and to outline current evidence regarding the role played by activated microglia in their pathogenesis.

  14. The role of the innate immune system in Alzheimer's disease and frontotemporal lobar degeneration: an eye on microglia.

    Science.gov (United States)

    Ridolfi, Elisa; Barone, Cinzia; Scarpini, Elio; Galimberti, Daniela

    2013-01-01

    In the last few years, genetic and biomolecular mechanisms at the basis of Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD) have been unraveled. A key role is played by microglia, which represent the immune effector cells in the central nervous system (CNS). They are extremely sensitive to the environmental changes in the brain and are activated in response to several pathologic events within the CNS, including altered neuronal function, infection, injury, and inflammation. While short-term microglial activity has generally a neuroprotective role, chronic activation has been implicated in the pathogenesis of neurodegenerative disorders, including AD and FTLD. In this framework, the purpose of this review is to give an overview of clinical features, genetics, and novel discoveries on biomolecular pathogenic mechanisms at the basis of these two neurodegenerative diseases and to outline current evidence regarding the role played by activated microglia in their pathogenesis.

  15. The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine

    Directory of Open Access Journals (Sweden)

    Erin C. Hedges

    2016-01-01

    Full Text Available In recent years several genes have linked amyotrophic lateral sclerosis (ALS and frontotemporal dementia (FTD as a spectrum disease; however little is known about what triggers their onset. With the ability to generate patient specific stem cell lines from somatic cells, it is possible to model disease without the need to transfect cells with exogenous DNA. These pluripotent stem cells have opened new avenues for identification of disease phenotypes and their relation to specific molecular pathways. Thus, as never before, compounds with potential applications for regenerative medicine can be specifically tailored in patient derived cultures. In this review, we discuss how patient specific induced pluripotent stem cells (iPSCs have been used to model ALS and FTD and the most recent drug screening targets for these diseases. We also discuss how an iPSC bank would improve the quality of the available cell lines and how it would increase knowledge about the ALS/FTD disease spectrum.

  16. Memory Test Performance on Analogous Verbal and Nonverbal Memory Tests in Patients with Frontotemporal Dementia and Alzheimer's Disease.

    Science.gov (United States)

    Baldock, Deanna; Miller, Justin B; Leger, Gabriel C; Banks, Sarah Jane

    2016-01-01

    Patients with frontotemporal dementia (FTD) typically have initial deficits in language or changes in personality, while the defining characteristic of Alzheimer's disease (AD) is memory impairment. Neuropsychological findings in the two diseases tend to differ, but can be confounded by verbal impairment in FTD impacting performance on memory tests in these patients. Twenty-seven patients with FTD and 102 patients with AD underwent a neuropsychological assessment before diagnosis. By utilizing analogous versions of a verbal and nonverbal memory test, we demonstrated differences in these two modalities between AD and FTD. Better differentiation between AD and FTD is found in a nonverbal memory test, possibly because it eliminates the confounding variable of language deficits found in patients with FTD. These results highlight the importance of nonverbal learning tests with multiple learning trials in diagnostic testing.

  17. Kraepelin’s description of chronic mania: a clinical picture that meets the behavioral variant frontotemporal dementia phenotype

    Directory of Open Access Journals (Sweden)

    Leandro Boson Gambogi

    Full Text Available ABSTRACT Chronic mania is an under-investigated condition and few reports have associated this disorder with an organic background. The present work examines Kraepelin’s reliable description of chronic mania from a current behavioral neurology viewpoint. Kraepelin had described a cluster of symptoms that are now recognized as core manifestations of the behavioral variant frontotemporal dementia (bvFTD clinical phenotype. We also carried out additional reviews of original manuscripts from Kraepelin’s peers, in order to find any case reports that might fulfill the current diagnostic proposal for bvFTD. Even though we failed to find an ideal case, we found some scholars who seemed to agree that chronic mania should be considered a special form of dementia. The present work highlights, through historical data, the possible overlapping features between primary psychiatric disorders and neuropsychiatric symptoms secondary to neurodegenerative conditions.

  18. Effectiveness of Electroconvulsive Therapy for Depression and Cotard’s Syndrome in a Patient with Frontotemporal Lobe Dementia

    Directory of Open Access Journals (Sweden)

    Toshiyuki Kobayashi

    2012-01-01

    Full Text Available In the field of psychogeriatrics, the differential diagnosis of depression and dementia, as well as the treatment of depression and comorbid dementia, is an important issue. In this paper, the authors present the case of a 72-year-old woman with Cotard’s syndrome and frontotemporal dementia (FTD who was admitted to a psychiatric hospital with delusions of negation accompanied by depressive symptoms. Pharmacotherapy over a 2-year hospitalization was unsuccessful, and she was subsequently transferred to our university hospital. A total of 18 sessions of electroconvulsive therapy released her from psychomotor inhibition, appetite loss, and Cotard’s delusions. The indication for electroconvulsive therapy in patients with dementia is discussed.

  19. Family caregivers of individuals with frontotemporal dementia: examining the relationship between coping and caregiver physical and mental health.

    Science.gov (United States)

    Wong, Cindy C; Wallhagen, Margaret I

    2014-01-01

    To identify strategies to assist family caregivers of individuals with frontotemporal dementia (FTD) in dealing with their caregiving demands, nurses must understand these family members' unique needs and how they currently deal with their demands. The purpose of this study was to examine the relationship between coping and caregiver physical and mental health among FTD family caregivers. Participants were primary caregivers of individuals with FTD (with behavioral symptoms) living at home (N = 61). A small positive association was noted between problem-focused coping and caregiver physical health (r = 0.29, p coping and caregiver mental health (r = 0.21, p = 0.10). However, multiple regression analysis showed that emotion-focused coping (β = 0.46, p coping strategies when dealing with behavioral symptoms manifested by individuals with FTD. Copyright 2014, SLACK Incorporated.

  20. Invited review: Frontotemporal dementia caused by microtubule-associated protein tau gene (MAPT) mutations: a chameleon for neuropathology and neuroimaging.

    Science.gov (United States)

    Ghetti, B; Oblak, A L; Boeve, B F; Johnson, K A; Dickerson, B C; Goedert, M

    2015-02-01

    Hereditary frontotemporal dementia associated with mutations in the microtubule-associated protein tau gene (MAPT) is a protean disorder. Three neuropathologic subtypes can be recognized, based on the presence of inclusions made of tau isoforms with three and four repeats, predominantly three repeats and mostly four repeats. This is relevant for establishing a correlation between structural magnetic resonance imaging and positron emission tomography using tracers specific for aggregated tau. Longitudinal studies will be essential to determine the evolution of anatomical alterations from the asymptomatic stage to the various phases of disease following the onset of symptoms. © 2014 The Authors. Neuropathology and Applied Neurobiology published by John Wiley & Sons Ltd on behalf of British Neuropathological Society.

  1. Memory Test Performance on Analogous Verbal and Nonverbal Memory Tests in Patients with Frontotemporal Dementia and Alzheimer's Disease

    Directory of Open Access Journals (Sweden)

    Deanna Baldock

    2016-01-01

    Full Text Available Background: Patients with frontotemporal dementia (FTD typically have initial deficits in language or changes in personality, while the defining characteristic of Alzheimer's disease (AD is memory impairment. Neuropsychological findings in the two diseases tend to differ, but can be confounded by verbal impairment in FTD impacting performance on memory tests in these patients. Methods: Twenty-seven patients with FTD and 102 patients with AD underwent a neuropsychological assessment before diagnosis. By utilizing analogous versions of a verbal and nonverbal memory test, we demonstrated differences in these two modalities between AD and FTD. Discussion: Better differentiation between AD and FTD is found in a nonverbal memory test, possibly because it eliminates the confounding variable of language deficits found in patients with FTD. These results highlight the importance of nonverbal learning tests with multiple learning trials in diagnostic testing.

  2. Right Limbic FDG-PET Hypometabolism Correlates with Emotion Recognition and Attribution in Probable Behavioral Variant of Frontotemporal Dementia Patients.

    Directory of Open Access Journals (Sweden)

    Chiara Cerami

    Full Text Available The behavioural variant of frontotemporal dementia (bvFTD is a rare disease mainly affecting the social brain. FDG-PET fronto-temporal hypometabolism is a supportive feature for the diagnosis. It may also provide specific functional metabolic signatures for altered socio-emotional processing. In this study, we evaluated the emotion recognition and attribution deficits and FDG-PET cerebral metabolic patterns at the group and individual levels in a sample of sporadic bvFTD patients, exploring the cognitive-functional correlations. Seventeen probable mild bvFTD patients (10 male and 7 female; age 67.8±9.9 were administered standardized and validated version of social cognition tasks assessing the recognition of basic emotions and the attribution of emotions and intentions (i.e., Ekman 60-Faces test-Ek60F and Story-based Empathy task-SET. FDG-PET was analysed using an optimized voxel-based SPM method at the single-subject and group levels. Severe deficits of emotion recognition and processing characterized the bvFTD condition. At the group level, metabolic dysfunction in the right amygdala, temporal pole, and middle cingulate cortex was highly correlated to the emotional recognition and attribution performances. At the single-subject level, however, heterogeneous impairments of social cognition tasks emerged, and different metabolic patterns, involving limbic structures and prefrontal cortices, were also observed. The derangement of a right limbic network is associated with altered socio-emotional processing in bvFTD patients, but different hypometabolic FDG-PET patterns and heterogeneous performances on social tasks at an individual level exist.

  3. Structural connectivity of right frontal hyperactive areas scales with stuttering severity.

    Science.gov (United States)

    Neef, Nicole E; Anwander, Alfred; Bütfering, Christoph; Schmidt-Samoa, Carsten; Friederici, Angela D; Paulus, Walter; Sommer, Martin

    2018-01-01

    A neuronal sign of persistent developmental stuttering is the magnified coactivation of right frontal brain regions during speech production. Whether and how stuttering severity relates to the connection strength of these hyperactive right frontal areas to other brain areas is an open question. Scrutinizing such brain-behaviour and structure-function relationships aims at disentangling suspected underlying neuronal mechanisms of stuttering. Here, we acquired diffusion-weighted and functional images from 31 adults who stutter and 34 matched control participants. Using a newly developed structural connectivity measure, we calculated voxel-wise correlations between connection strength and stuttering severity within tract volumes that originated from functionally hyperactive right frontal regions. Correlation analyses revealed that with increasing speech motor deficits the connection strength increased in the right frontal aslant tract, the right anterior thalamic radiation, and in U-shaped projections underneath the right precentral sulcus. In contrast, with decreasing speech motor deficits connection strength increased in the right uncinate fasciculus. Additional group comparisons of whole-brain white matter skeletons replicated the previously reported reduction of fractional anisotropy in the left and right superior longitudinal fasciculus as well as at the junction of right frontal aslant tract and right superior longitudinal fasciculus in adults who stutter compared to control participants. Overall, our investigation suggests that right fronto-temporal networks play a compensatory role as a fluency enhancing mechanism. In contrast, the increased connection strength within subcortical-cortical pathways may be implied in an overly active global response suppression mechanism in stuttering. Altogether, this combined functional MRI-diffusion tensor imaging study disentangles different networks involved in the neuronal underpinnings of the speech motor deficit in

  4. Non-invasive quick diagnosis of cardiovascular problems from visible and invisible abnormal changes with increased cardiac troponin I appearing on cardiovascular representation areas of the eyebrows, left upper lip, etc. of the face & hands: beneficial manual stimulation of hands for acute anginal chest pain, and important factors in safe, effective treatment.

    Science.gov (United States)

    Omura, Yoshiaki; Jones, Marilyn K; Duvvi, Harsha; Shimotsuura, Yasuhiro; Ohki, Motomu; Rodriques, Aaron

    2014-01-01

    Our previous study indicated that there are at least 7 cardiovascular representation areas on the face, including the "Eyebrows", both sides of the "Nose", "Lelt Upper Lip" and the "Outside of the corner of both sides of the mouth," in addition to 2 areas in each hand. When there are cardiovascular problems, some of the heart representation areas of these areas often show the following changes: 1) Most distinctive visible changes such as the initial whitening with or without long white hair, then hair loss and complete disappearance of the hairs of the heart representation area of "Eyebrows" 2) Invisible biochemical changes that happen in heart representation areas at the "Left Upper Lips", 3) "Nose" below eye level as well as 4) "3rd segment of Middle Finger of Hands." Most distinctive visible & invisible changes are found in heart representation areas on the "Eyebrow", located nearest to the midline of face, where the color of the hairs becomes white compared with the rest of the Eyebrow. Then the cardiovascular problem advances, and hair starts disappearing. When there are no hairs at the heart representation areas of the Eyebrow, usually Cardiac Troponin I is increased to a very serious, abnormal high value. Most of the cardiovascular representation areas of the face show, regardless of presence or absence of visible change. When there is a cardiovascular problem, not only simple Bi-Digital O-Ring Test can detect without using any instrument in several minutes but also, corresponding biochemical changes of abnormally increased Cardiac Troponin I level can often be detected non-invasively from these Organ Representation Areas of Face & Hands, although changes in Eyebrows, L-Upper Lip & 3rd segment of middle fingers are clinically the most reliable changes & easy to identify the locations. Manual Stimulation of Hand's heart representation areas often eliminated acute anginal chest pain before medical help became available. Important factors for safe, effective

  5. Primary left ventricular hydatid cyst in a child: case report

    Energy Technology Data Exchange (ETDEWEB)

    Turkvatan, A. [Turkiye Yuksek Ihtisas Hospital, Dept. of Radiology, Ankara (Turkey); Yelgec, N.S. [Turkiye Yuksek Ihtisas Hospital, Dept. of Cardiology, Ankara (Turkey); Calikoglu, U.; Olcer, T. [Turkiye Yuksek Ihtisas Hospital, Dept. of Radiology, Ankara (Turkey)

    2000-12-01

    The most common cause of echinococcosis in humans is Echinococcus granulosus. Although hydatid cyst is most frequently localized in liver (more than 65% of cases) and lung (25%) by means of portal and systemic circulation, it may involve other tissues and organs. Cardiac hydatid cysts account for only 0.5%-2% of all hydatid cysts, even in endemic areas. Of all cardiac hydatid cysts, the left ventricle accounts for 60%, right ventricle 10%, pericardium 7%, pulmonary artery 6%, left atrial appendage 6%, and interventricular septum 4%. We report the case of a myocardial hydatid cyst of the left ventricle in a 9-year-old boy. (author)

  6. Longitudinal strain predicts left ventricular mass regression after aortic valve replacement for severe aortic stenosis and preserved left ventricular function.

    Science.gov (United States)

    Gelsomino, Sandro; Lucà, Fabiana; Parise, Orlando; Lorusso, Roberto; Rao, Carmelo Massimiliano; Vizzardi, Enrico; Gensini, Gian Franco; Maessen, Jos G

    2013-11-01

    We explored the influence of global longitudinal strain (GLS) measured with two-dimensional speckle-tracking echocardiography on left ventricular mass regression (LVMR) in patients with pure aortic stenosis (AS) and normal left ventricular function undergoing aortic valve replacement (AVR). The study population included 83 patients with severe AS (aortic valve area regression (all P regression in patients with pure AS undergoing AVR. Our findings must be confirmed by further larger studies.

  7. Clinical, neuropathological, and genetic characteristics of the novel IVS9+1delG GRN mutation in a patient with frontotemporal dementia.

    Science.gov (United States)

    Taipa, Ricardo; Tuna, Assunção; Damásio, Joana; Pinto, Pedro S; Cavaco, Sara; Pereira, Sonia; Milterberger-Miltenyi, Gabriel; Galimberti, Daniela; Melo-Pires, Manuel

    2012-01-01

    Frontotemporal lobar degeneration (FTLD) refers to a clinically, pathologically, and genetically heterogeneous group of dementias that arises from the degeneration of the frontal and temporal lobes. Mutations in the progranulin gene (GRN) are a major cause of FTLD with TDP-43 inclusions. Herein, we describe the clinical, neuropathological, and genetic findings in a case of autosomal dominant behavioral variant of frontotemporal dementia (bvFTD) with asymmetrical parkinsonism and prominent visuospatial deficits that carries a novel GRN mutation. This case highlights important clinical characteristics that seem to be common in FTLD GRN-associated patients, such as asymmetrical parkinsonism and parietal symptoms, and that are correlated to the pathological involvement of striatum (rather than substantia nigra in our case) and parietal lobe. We also emphasize that plasma progranulin level can be useful to infer about the pathogenicity of new GRN mutations.

  8. Alzheimer's disease and frontotemporal dementia are differentiated by discriminant analysis applied to 99mTc HmPAO SPECT data

    OpenAIRE

    Charpentier, P; Lavenu, I; Defebvre, L; Duhamel, A; Lecouffe, P; Pasquier, F; Steinling, M

    2000-01-01

    OBJECTIVE—Alzheimer's disease (AD) and frontotemporal dementia (FTD) are the most frequent neurodegenerative cognitive disorders. However, FTD remains poorly recognised clinically. The use of 99mHmPAO-single photon emission computed tomography (SPECT) has been demonstrated in the differentiation of AD and FTD. Nethertheless, there are very few comparative studies designed to assess its precise value in this differential diagnosis. The aim was to determine a simple decisio...

  9. Left Activism, Succour and Selfhood

    DEFF Research Database (Denmark)

    Hughes, Celia Penelope

    2014-01-01

    an interchange of motherhood, domesticity, far-left politics, and close female friendship. The article will show how the women's epistolary friendship offers intimate insight into female self-fashioning at a breakthrough social and political moment in 1970s Britain. As they reflected on some of the key political...

  10. Behavioral and Neuroimaging Evidence for Facial Emotion Recognition in Elderly Korean Adults with Mild Cognitive Impairment, Alzheimer’s Disease, and Frontotemporal Dementia

    Directory of Open Access Journals (Sweden)

    Soowon Park

    2017-11-01

    Full Text Available Background: Facial emotion recognition (FER is impaired in individuals with frontotemporal dementia (FTD and Alzheimer’s disease (AD when compared to healthy older adults. Since deficits in emotion recognition are closely related to caregiver burden or social interactions, researchers have fundamental interest in FER performance in patients with dementia.Purpose: The purpose of this study was to identify the performance profiles of six facial emotions (i.e., fear, anger, disgust, sadness, surprise, and happiness and neutral faces measured among Korean healthy control (HCs, and those with mild cognitive impairment (MCI, AD, and FTD. Additionally, the neuroanatomical correlates of facial emotions were investigated.Methods: A total of 110 (33 HC, 32 MCI, 32 AD, 13 FTD older adult participants were recruited from two different medical centers in metropolitan areas of South Korea. These individuals underwent an FER test that was used to assess the recognition of emotions or absence of emotion (neutral in 35 facial stimuli. Repeated measures two-way analyses of variance were used to examine the distinct profiles of emotional recognition among the four groups. We also performed brain imaging and voxel-based morphometry (VBM on the participants to examine the associations between FER scores and gray matter volume.Results: The mean score of negative emotion recognition (i.e., fear, anger, disgust, and sadness clearly discriminated FTD participants from individuals with MCI and AD and HC [F(3,106 = 10.829, p < 0.001, η2 = 0.235], whereas the mean score of positive emotion recognition (i.e., surprise and happiness did not. A VBM analysis showed negative emotions were correlated with gray matter volume of anterior temporal regions, whereas positive emotions were related to gray matter volume of fronto-parietal regions.Conclusion: Impairment of negative FER in patients with FTD is cross-cultural. The discrete neural correlates of FER indicate that

  11. Behavioral and Neuroimaging Evidence for Facial Emotion Recognition in Elderly Korean Adults with Mild Cognitive Impairment, Alzheimer's Disease, and Frontotemporal Dementia.

    Science.gov (United States)

    Park, Soowon; Kim, Taehoon; Shin, Seong A; Kim, Yu Kyeong; Sohn, Bo Kyung; Park, Hyeon-Ju; Youn, Jung-Hae; Lee, Jun-Young

    2017-01-01

    Background: Facial emotion recognition (FER) is impaired in individuals with frontotemporal dementia (FTD) and Alzheimer's disease (AD) when compared to healthy older adults. Since deficits in emotion recognition are closely related to caregiver burden or social interactions, researchers have fundamental interest in FER performance in patients with dementia. Purpose: The purpose of this study was to identify the performance profiles of six facial emotions (i.e., fear, anger, disgust, sadness, surprise, and happiness) and neutral faces measured among Korean healthy control (HCs), and those with mild cognitive impairment (MCI), AD, and FTD. Additionally, the neuroanatomical correlates of facial emotions were investigated. Methods: A total of 110 (33 HC, 32 MCI, 32 AD, 13 FTD) older adult participants were recruited from two different medical centers in metropolitan areas of South Korea. These individuals underwent an FER test that was used to assess the recognition of emotions or absence of emotion (neutral) in 35 facial stimuli. Repeated measures two-way analyses of variance were used to examine the distinct profiles of emotional recognition among the four groups. We also performed brain imaging and voxel-based morphometry (VBM) on the participants to examine the associations between FER scores and gray matter volume. Results: The mean score of negative emotion recognition (i.e., fear, anger, disgust, and sadness) clearly discriminated FTD participants from individuals with MCI and AD and HC [ F (3,106) = 10.829, p < 0.001, η 2 = 0.235], whereas the mean score of positive emotion recognition (i.e., surprise and happiness) did not. A VBM analysis showed negative emotions were correlated with gray matter volume of anterior temporal regions, whereas positive emotions were related to gray matter volume of fronto-parietal regions. Conclusion: Impairment of negative FER in patients with FTD is cross-cultural. The discrete neural correlates of FER indicate that emotional

  12. Behavioral and Neuroimaging Evidence for Facial Emotion Recognition in Elderly Korean Adults with Mild Cognitive Impairment, Alzheimer’s Disease, and Frontotemporal Dementia

    Science.gov (United States)

    Park, Soowon; Kim, Taehoon; Shin, Seong A; Kim, Yu Kyeong; Sohn, Bo Kyung; Park, Hyeon-Ju; Youn, Jung-Hae; Lee, Jun-Young

    2017-01-01

    Background: Facial emotion recognition (FER) is impaired in individuals with frontotemporal dementia (FTD) and Alzheimer’s disease (AD) when compared to healthy older adults. Since deficits in emotion recognition are closely related to caregiver burden or social interactions, researchers have fundamental interest in FER performance in patients with dementia. Purpose: The purpose of this study was to identify the performance profiles of six facial emotions (i.e., fear, anger, disgust, sadness, surprise, and happiness) and neutral faces measured among Korean healthy control (HCs), and those with mild cognitive impairment (MCI), AD, and FTD. Additionally, the neuroanatomical correlates of facial emotions were investigated. Methods: A total of 110 (33 HC, 32 MCI, 32 AD, 13 FTD) older adult participants were recruited from two different medical centers in metropolitan areas of South Korea. These individuals underwent an FER test that was used to assess the recognition of emotions or absence of emotion (neutral) in 35 facial stimuli. Repeated measures two-way analyses of variance were used to examine the distinct profiles of emotional recognition among the four groups. We also performed brain imaging and voxel-based morphometry (VBM) on the participants to examine the associations between FER scores and gray matter volume. Results: The mean score of negative emotion recognition (i.e., fear, anger, disgust, and sadness) clearly discriminated FTD participants from individuals with MCI and AD and HC [F(3,106) = 10.829, p < 0.001, η2 = 0.235], whereas the mean score of positive emotion recognition (i.e., surprise and happiness) did not. A VBM analysis showed negative emotions were correlated with gray matter volume of anterior temporal regions, whereas positive emotions were related to gray matter volume of fronto-parietal regions. Conclusion: Impairment of negative FER in patients with FTD is cross-cultural. The discrete neural correlates of FER indicate that emotional

  13. Left ventricular diastolic performance of left ventricular hypertrophy

    Energy Technology Data Exchange (ETDEWEB)

    Ikezono, Tohru; Ozaki, Masaharu; Yamagishi, Takashi; Shimizu, Tatsuro; Furutani, Yuji; Kusukawa, Reizo

    1987-02-01

    To study left ventricular diastolic performance in different forms of left ventricular hypertrophy, ECG gated cardiac blood pool scan was performed in 11 patients with hypertrophic nonobstructive cardiomyopathy (HCM) and in 19 patients with hypertension (HT), and left ventricular volume curve (LVVC) was analyzed and compared with those of 13 normal subjects (N). Ejection fraction (EF) and early filling volume ratio (the ratio of volume increment of 100 msec later than the zero point in the first derivative of LVVC to the end diastolic volume) (%EFV) were computed from LVVC. Peak ejection rate (PER) and peak filling rate (PFR) were obtained from the first derivative of LVVC. Peak ejection acceleration (PEA) and peak filling acceleration (PFA) were calculated from the second derivative of LVVC. EF, PER and PEA did not show any difference between these 3 groups. PFR was lower in HT (2.6 +- 0.5) compared with those in HCM (3.0 +- 0.5) (p < 0.05) and in N (3.4 +- 0.5) (p < 0.001), but the %EFV in HCM (4.9 +- 1.8) was lower than those in HT (6.9 +- 1.9) (p < 0.01) and in N (11.4 +- 1.4) (p < 0.001). Moreover, PFA in HCM (27.9 +- 7.2) was increased than those in HT (20.2 +- 5.4) (p < 0.01) with no differences between HCM and N (29.4 +- 8.1). Significant correlation was observed between PFR and PFA (Y = 0.06X + 1.4. r = 0.856. p < 0.001). These result indicate that, in HCM, reduced increase in early left ventricular volume is compensated by a greater filling acceleration. In contrast, there is no compensation by filling acceleration in HT.

  14. Detailed anatomy of a left accessory aberrant colic artery.

    Science.gov (United States)

    Rusu, M C; Vlad, M; Voinea, L M; Curcă, G C; Sişu, A M

    2008-10-01

    In an aged human female cadaver a left accessory aberrant colic artery (LAACA) was observed and studied. It originated from the superior mesenteric artery at 3 cm proximal to the middle colic artery, at the inferior border of pancreas, passing over Treitz's muscle and continued covered by the superior duodenal fold where it crossed the inferior mesenteric vein. Further, it continued with a satellite vein anterior to the left renal vein and the anterior branch of the renal artery. The LAACA divided into an ascending branch and a descending one, anastomosed with the middle colic and proper left colic arteries; between its two primary branches and the splenic flexure of colon, a hypovascular area was observed. The surgical relevance of the LAACA detailed anatomy mainly relates to specific procedures performed in left colectomies and nephrectomies.

  15. Producing The New Regressive Left

    DEFF Research Database (Denmark)

    Crone, Christine

    This thesis is the first comprehensive research work conducted on the Beirut based TV station, an important representative of the post-2011 generation of Arab satellite news media. The launch of al-Mayadeen in June 2012 was closely linked to the political developments across the Arab world...... members, this thesis investigates a growing political trend and ideological discourse in the Arab world that I have called The New Regressive Left. On the premise that a media outlet can function as a forum for ideology production, the thesis argues that an analysis of this material can help to trace...... the contexture of The New Regressive Left. If the first part of the thesis lays out the theoretical approach and draws the contextual framework, through an exploration of the surrounding Arab media-and ideoscapes, the second part is an analytical investigation of the discourse that permeates the programmes aired...

  16. Left Ventricular Hypertrophy Evaluation in Obese Hypertensive Patients: Effect of Left Ventricular Mass Index Criteria

    Directory of Open Access Journals (Sweden)

    Eduardo Cantoni Rosa

    2002-04-01

    Full Text Available PURPOSE: To evaluate left ventricular mass (LVM index in hypertensive and normotensive obese individuals. METHODS: Using M mode echocardiography, 544 essential hypertensive and 106 normotensive patients were evaluated, and LVM was indexed for body surface area (LVM/BSA and for height² (LVM/h². The 2 indexes were then compared in both populations, in subgroups stratified according to body mass index (BMI: or = 30kg/m². RESULTS: The BSA index does not allow identification of significant differences between BMI subgroups. Indexing by height² provides significantly increased values for high BMI subgroups in normotensive and hypertensive populations. CONCLUSION: Left ventricular hypertrophy (LVH has been underestimated in the obese with the use of LVM/BSA because this index considers obesity as a physiological variable. Indexing by height² allows differences between BMI subgroups to become apparent and seems to be more appropriate for detecting LVH in obese populations.

  17. From the Left to the Right: How the Brain Compensates Progressive Loss of Language Function

    Science.gov (United States)

    Thiel, Alexander; Habedank, Birgit; Herholz, Karl; Kessler, Josef; Winhuisen, Lutz; Haupt, Walter F.; Heiss, Wolf-Dieter

    2006-01-01

    In normal right-handed subjects language production usually is a function of the left brain hemisphere. Patients with aphasia following brain damage to the left hemisphere have a considerable potential to compensate for the loss of this function. Sometimes, but not always, areas of the right hemisphere which are homologous to language areas of the…

  18. Structural neuroimaging of social cognition in progressive non-fluent aphasia and behavioral variant of frontotemporal dementia

    Science.gov (United States)

    Couto, Blas; Manes, Facundo; Montañés, Patricia; Matallana, Diana; Reyes, Pablo; Velasquez, Marcela; Yoris, Adrián; Baez, Sandra; Ibáñez, Agustin

    2013-01-01

    Social cognition impairments are pervasive in the frontotemporal dementias (FTD). These deficits would be triggered by (a) basic emotion and face recognition processes as well as by (b) higher level social cognition (e.g., theory of mind, ToM). Both emotional processing and social cognition impairments have been previously reported in the behavioral variant of FTD (bvFTD) and also in other versions of FTDs, including primary progressive aphasia. However, no neuroanatomic comparison between different FTD variants has been performed. We report selective behavioral impairments of face recognition, emotion recognition, and ToM in patients with bvFTD and progressive non-fluent aphasia (PNFA) when compared to controls. Voxel-based morphometry (VBM) shows a classical impairment of mainly orbitofrontal (OFC), anterior cingulate (ACC), insula and lateral temporal cortices in patients. Comparative analysis of regional gray matter related to social cognition deficits (VBM) reveals a differential pattern of fronto-insulo-temporal atrophy in bvFTD and an insulo-temporal involvement in PNFA group. Results suggest that in spite of similar social cognition impairments reported in bvFTD and PNFA, the former represents an inherent ToM affectation whereas in the PNFA these deficits could be related to more basic processes of face and emotion recognition. These results are interpreted in the frame of the fronto-insulo-temporal social context network model (SCNM). PMID:23966929

  19. Constructional apraxia in frontotemporal dementia associated with the C9orf72 mutation: broadening the clinical and neuropsychological phenotype.

    Science.gov (United States)

    Floris, Gianluca; Borghero, Giuseppe; Cannas, Antonino; Di Stefano, Francesca; Ruiu, Elisa; Murru, Maria R; Corongiu, Daniela; Cuccu, Stefania; Tranquilli, Stefania; Sardu, Claudia; Marrosu, Maria G; Chiò, Adriano; Marrosu, Francesco

    2015-03-01

    In our study we analysed clinical and neuropsychological data in a cohort of 57 Sardinian patients with FTD (55 apparently unrelated and two belonging to the same family), who underwent genetic screening for the C9orf72 mutation. Eight out of 56 patients were found positive for the C9orf72 mutation representing 14% of the entire cohort and 31.6% of the familial cases (6/19). C9orf72 mutated patients differed from the other FTD cases of the cohort for a younger age of onset, higher frequency of familial history for FTD and higher prevalence of delusional psychotic symptoms and hallucinations. In the neuropsychological assessment, C9orf72 mutated patients differed from non-mutated for the high frequency of visuospatial dysfunction regarding constructional apraxia (p = 0.02). In conclusion, our study confirms that Sardinian FTD patients have peculiar genetic characteristics and that C9orf72 mutated patients have a distinctive clinical and neuropsychological profile that could help differentiate them from other FTD patients. In our cohort we found that constructional apraxia, rarely reported in FTD, can properly discriminate between C9orf72 mutated and non-mutated patients and contribute to broaden the neuropsychological profile in frontotemporal dementia associated with this mutation.

  20. Early-stage differentiation between presenile Alzheimer's disease and frontotemporal dementia using arterial spin labeling MRI

    Energy Technology Data Exchange (ETDEWEB)

    Steketee, Rebecca M.E.; Meijboom, Rozanna; Lugt, Aad van der; Smits, Marion [Erasmus MC - University Medical Center, Department of Radiology, PO Box 2040, Rotterdam (Netherlands); Bron, Esther E.; Klein, Stefan [Erasmus MC - University Medical Center, Biomedical Imaging Group Rotterdam, Departments of Medical Informatics and Radiology, PO Box 2040, Rotterdam (Netherlands); Houston, Gavin C. [GE Healthcare, Hatfield (United Kingdom); Mutsaerts, Henri J.M.M. [Academic Medical Center, Department of Radiology, PO Box 22660, Amsterdam (Netherlands); Mendez Orellana, Carolina P. [Erasmus MC - University Medical Center, Department of Radiology, PO Box 2040, Rotterdam (Netherlands); Erasmus MC - University Medical Center, Department of Neurology, PO Box 2040, Rotterdam (Netherlands); Jong, Frank Jan de; Swieten, John C. van [Erasmus MC - University Medical Center, Department of Neurology, PO Box 2040, Rotterdam (Netherlands)

    2016-01-15

    To investigate arterial spin labeling (ASL)-MRI for the early diagnosis of and differentiation between the two most common types of presenile dementia: Alzheimer's disease (AD) and frontotemporal dementia (FTD), and for distinguishing age-related from pathological perfusion changes. Thirteen AD and 19 FTD patients, and 25 age-matched older and 22 younger controls underwent 3D pseudo-continuous ASL-MRI at 3 T. Gray matter (GM) volume and cerebral blood flow (CBF), corrected for partial volume effects, were quantified in the entire supratentorial cortex and in 10 GM regions. Sensitivity, specificity and diagnostic performance were evaluated in regions showing significant CBF differences between patient groups or between patients and older controls. AD compared with FTD patients had hypoperfusion in the posterior cingulate cortex, differentiating these with a diagnostic performance of 74 %. Compared to older controls, FTD patients showed hypoperfusion in the anterior cingulate cortex, whereas AD patients showed a more widespread regional hypoperfusion as well as atrophy. Regional atrophy was not different between AD and FTD. Diagnostic performance of ASL to differentiate AD or FTD from controls was good (78-85 %). Older controls showed global hypoperfusion compared to young controls. ASL-MRI contributes to early diagnosis of and differentiation between presenile AD and FTD. (orig.)

  1. Estimating severity of illness and disability in Frontotemporal Dementia: Preliminary analysis of the Dementia Disability Rating (DDR).

    Science.gov (United States)

    Onyike, Chiadi U; Sloane, Kelly L; Smyth, Shawn F; Appleby, Brian S; Blass, David M; Rabins, Peter V

    2011-01-01

    Current measures of severity and disability do not stage or track the progression of disability in frontotemporal dementia (FTD) well. We investigated the reliability of the newly developed Dementia Disability Rating (DDR) in the measurement and staging of illness severity in FTD and dementia of the Alzheimer type (DAT). We studied 48 consecutive patients of the Johns Hopkins FTD and Young-Onset Dementias Clinic, with diagnoses of DAT, FTD, vascular dementia and "other" cognitive disorder (CDNOS). Cases were scored on the CDR and DDR by three trained raters, based on neuropsychiatric examinations performed at first visit and other assessments performed within the preceding year. Consensus ratings were assigned in conference. Inter-rater correlations of DDR sum of ranks scores for DAT ranged from 0.88 to 0.91, for FTD 0.89-0.96 and for CDNOS 0.85-0.97. Similar correlations were observed of the CDR sum of rank scores for DAT and FTD. Correlations of DDR summary scores for DAT were 0.67-0.91 and for FTD 0.79-0.91, as compared to CDR data: 0.87-0.92 (pDDR summary scores was higher than in FTD patients, whereas correlations based on sum of ranks scores were high in both groups. These preliminary data indicate the DDR measures disability in DAT and FTD, with reliability comparable to the CDR. Convergent validity was demonstrated for the DDR.

  2. Accuracy of neuropsychological tests and the Neuropsychiatric Inventory in differential diagnosis between Frontotemporal dementia and Alzheimer's disease

    Directory of Open Access Journals (Sweden)

    Valéria Santoro Bahia

    Full Text Available Abstract The differential diagnosis between frontotemporal dementia (FTD and Alzheimer's disease (AD is sometimes difficult. Objectives: To verify the accuracy of neuropsychological tests and a behavioral disorders scale in the differential diagnosis between FTLD and AD. Methods: Retrospective data on 12 FTD patients and 12 probable AD patients were analyzed. The scores on neuropsychological tests (MMSE score, reverse digit span, delayed recall for drawings, semantic fluency of animals and the Neuropsychiatric Inventory (NPI in both groups were compared. Results: Both groups had similar performance on neuropsychological tests. All FTD patients and 50% of AD patients had neuropsychiatric abnormalities. The NPI score was 58.0±19.3 for the FTD patients, and 3.6±4.7 for the AD patients (p<0.01. Using a NPI cut-off score of 13, the sensitivity and specificity were 100% in this sample. The four most common neuropsychiatric disturbances in FTD patients were: apathy, aberrant motor behavior, disinhibition and eating abnormalities. Apathy and dysphoria/depression were the most common behavioral symptoms among the AD patients. Conclusions: In this study, NPI was found to be a useful tool for the differential diagnosis between FTD and AD. The neuropsychological tests commonly used in the medical office were unable to distinguish between the two groups.

  3. Brain Regions Involved in Arousal and Reward Processing are Associated with Apathy in Alzheimer's Disease and Frontotemporal Dementia.

    Science.gov (United States)

    Huey, Edward D; Lee, Seonjoo; Cheran, Gayathri; Grafman, Jordan; Devanand, Davangere P

    2017-01-01

    Apathy is a common and problematic symptom of several neurodegenerative illnesses, but its neuroanatomical bases are not understood. To determine the regions associated with apathy in subjects with mild Alzheimer's disease (AD) using a method that accounts for the significant co-linearity of regional atrophy and neuropsychiatric symptoms. We identified 57 subjects with mild AD (CDR = 1) and neuropsychiatric symptoms in the Alzheimer's Disease Neuroimaging Initiative (ADNI) database. We performed a multivariate multiple regression with LASSO regularization on all symptom subscales of the Neuropsychiatric Inventory and the whole-brain ROI volumes calculated from their baseline MRIs with FreeSurfer. We compared our results to those from a previous study using the same method in patients with frontotemporal dementia (FTD) and corticobasal syndrome (CBS). Of neuropsychiatric symptoms, apathy showed the most robust neuroanatomical associations in the AD subjects. Atrophy of the following regions were independently associated with apathy: the ventromedial prefrontal cortex; ventrolateral prefrontal cortex; posterior cingulate cortex and adjacent lateral cortex; and the bank of the superior temporal sulcus. These results replicate previous studies using FTD and CBS patients, mostly agree with the previous literature on apathy in AD, and correspond to the Medial and Orbital Prefrontal Cortex networks identified in non-human primates. The current study, previous studies from our laboratory, and the previous literature suggest that impairment of the same brain networks involved in arousal, threat response, and reward processing are associated with apathy in AD and FTD.

  4. Patient iPSC-Derived Neurons for Disease Modeling of Frontotemporal Dementia with Mutation in CHMP2B

    Directory of Open Access Journals (Sweden)

    Yu Zhang

    2017-03-01

    Full Text Available The truncated mutant form of the charged multivesicular body protein 2B (CHMP2B is causative for frontotemporal dementia linked to chromosome 3 (FTD3. CHMP2B is a constituent of the endosomal sorting complex required for transport (ESCRT and, when mutated, disrupts endosome-to-lysosome trafficking and substrate degradation. To understand the underlying molecular pathology, FTD3 patient induced pluripotent stem cells (iPSCs were differentiated into forebrain-type cortical neurons. FTD3 neurons exhibited abnormal endosomes, as previously shown in patients. Moreover, mitochondria of FTD3 neurons displayed defective cristae formation, accompanied by deficiencies in mitochondrial respiration and increased levels of reactive oxygen. In addition, we provide evidence for perturbed iron homeostasis, presenting an in vitro patient-specific model to study the effects of iron accumulation in neurodegenerative diseases. All phenotypes observed in FTD3 neurons were rescued in CRISPR/Cas9-edited isogenic controls. These findings illustrate the relevance of our patient-specific in vitro models and open up possibilities for drug target development.

  5. [A nursing experience using the props-integrated communicative approach to ameliorate aggression in a frontotemporal dementia patient].

    Science.gov (United States)

    Shih, Ying-Jyun; Wang, Ya-Hui; Yang, Yung-Jen

    2014-12-01

    This report introduces the nursing caring experience with a male patient with frontotemporal dementia (FTD) who was hospitalized in an acute psychiatric ward from March 5th to April 30th, 2012 due to the clinical manifestations of verbally expressive impairment, aggression, and subsequent caregiver burden. The patient was assessed according to the guidelines of clinical competencies for mental health nursing assessments developed by the Psychiatric Mental Health Nurses Association. Three clinical diagnoses were identified after this assessment, including (1) impaired verbal communication, (2) chronic confusion, and (3) caregiver role strain. The current report focuses only on the clinical issue of impaired verbal communication. We adopted a props-integrated communicative approach by integrating props and physical motions with communicative strategies. This approach enabled us to formulate a patient-centered communicating model and prompt for active expression and adequate communication, which ultimately resolved the patient's aggression problem. In addition, we provided psychoeducation to the family members in order to teach them the relevant knowledge, skills, and approaches that caregivers may use to enhance their caring capabilities and reduce the burden of caregiving. This successful experience may be used as a reference in caring for FTD patients with communicative impairments. Our proposed approach integrates props with simple language and develops an appropriate communicating model to provide high quality care for patients.

  6. Early-stage differentiation between presenile Alzheimer's disease and frontotemporal dementia using arterial spin labeling MRI

    International Nuclear Information System (INIS)

    Steketee, Rebecca M.E.; Meijboom, Rozanna; Lugt, Aad van der; Smits, Marion; Bron, Esther E.; Klein, Stefan; Houston, Gavin C.; Mutsaerts, Henri J.M.M.; Mendez Orellana, Carolina P.; Jong, Frank Jan de; Swieten, John C. van

    2016-01-01

    To investigate arterial spin labeling (ASL)-MRI for the early diagnosis of and differentiation between the two most common types of presenile dementia: Alzheimer's disease (AD) and frontotemporal dementia (FTD), and for distinguishing age-related from pathological perfusion changes. Thirteen AD and 19 FTD patients, and 25 age-matched older and 22 younger controls underwent 3D pseudo-continuous ASL-MRI at 3 T. Gray matter (GM) volume and cerebral blood flow (CBF), corrected for partial volume effects, were quantified in the entire supratentorial cortex and in 10 GM regions. Sensitivity, specificity and diagnostic performance were evaluated in regions showing significant CBF differences between patient groups or between patients and older controls. AD compared with FTD patients had hypoperfusion in the posterior cingulate cortex, differentiating these with a diagnostic performance of 74 %. Compared to older controls, FTD patients showed hypoperfusion in the anterior cingulate cortex, whereas AD patients showed a more widespread regional hypoperfusion as well as atrophy. Regional atrophy was not different between AD and FTD. Diagnostic performance of ASL to differentiate AD or FTD from controls was good (78-85 %). Older controls showed global hypoperfusion compared to young controls. ASL-MRI contributes to early diagnosis of and differentiation between presenile AD and FTD. (orig.)

  7. Restoration of Progranulin Expression Rescues Cortical Neuron Generation in an Induced Pluripotent Stem Cell Model of Frontotemporal Dementia

    Directory of Open Access Journals (Sweden)

    Susanna Raitano

    2015-01-01

    Full Text Available To understand how haploinsufficiency of progranulin (PGRN causes frontotemporal dementia (FTD, we created induced pluripotent stem cells (iPSCs from patients carrying the GRNIVS1+5G > C mutation (FTD-iPSCs. FTD-iPSCs were fated to cortical neurons, the cells most affected in FTD. Although generation of neuroprogenitors was unaffected, their further differentiation into CTIP2-, FOXP2-, or TBR1-TUJ1 double-positive cortical neurons, but not motorneurons, was significantly decreased in FTD-neural progeny. Zinc finger nuclease-mediated introduction of GRN cDNA into the AAVS1 locus corrected defects in cortical neurogenesis, demonstrating that PGRN haploinsufficiency causes inefficient cortical neuron generation. RNA sequencing analysis confirmed reversal of the altered gene expression profile following genetic correction. We identified the Wnt signaling pathway as one of the top defective pathways in FTD-iPSC-derived neurons, which was reversed following genetic correction. Differentiation of FTD-iPSCs in the presence of a WNT inhibitor mitigated defective corticogenesis. Therefore, we demonstrate that PGRN haploinsufficiency hampers corticogenesis in vitro.

  8. Psychological Impact of Predictive Genetic Testing in VCP Inclusion Body Myopathy, Paget Disease of Bone and Frontotemporal Dementia.

    Science.gov (United States)

    Surampalli, Abhilasha; Khare, Manaswitha; Kubrussi, Georgette; Wencel, Marie; Tanaja, Jasmin; Donkervoort, Sandra; Osann, Kathryn; Simon, Mariella; Wallace, Douglas; Smith, Charles; M McInerney-Leo, Aideen; Kimonis, Virginia

    2015-10-01

    Inclusion Body Myopathy associated with Paget's disease of bone and Fronto-temporal Dementia, also known as multisystem proteinopathy is an autosomal dominant, late onset neurodegenerative disorder caused by mutations in Valosin containing protein (VCP) gene. This study aimed to assess uptake and decision making for predictive genetic testing and the impact on psychological well-being. Individuals who had participated in the gene discovery study with a 50 % a priori risk of inheriting VCP disease were sent a letter of invitation offering genetic counseling and testing and were also invited to participate in this psychosocial study. A total of 102 individuals received an invitation and 33 individuals participated in genetic counseling and testing (32.3 %) with 29 completing baseline questionnaires. Twenty completed the follow-up post-test Hospital Anxiety and Depression Scale questionnaire including 13 of the 18 who had tested positive. Mean risk perception at baseline was 50.1 %. Reasons for testing included planning for the future, relieving uncertainty, informing children and satisfying curiosity. At baseline, one quarter of the participants had high levels of anxiety. However, scores were normal one year following testing. In this small cohort, one third of individuals at 50 % risk chose pre-symptomatic testing. Although one quarter of those choosing testing had high anxiety at baseline, this was not evident at follow-up.

  9. Brodmann area 12: an historical puzzle relevant to FTLD.

    Science.gov (United States)

    Kawamura, M; Miller, M W; Ichikawa, H; Ishihara, K; Sugimoto, A

    2011-05-03

    Brodmann brain maps, assembled in 1909, are still in use, but understanding of their animal-human homology is uncertain. Furthermore, in 1909, Brodmann did not identify human area 12 (BA12), a location now important to understanding of frontotemporal lobar degeneration (FTLD). We re-examined Brodmann's areas, both animal and human, in his 1909 monograph and other literature, both historical and contemporary, and projected BA12 onto the medial surface of a fixed human brain to show its location. We found Brodmann did identify human BA12 in later maps (1910 and 1914), but that his brain areas, contrary to his own aims as a comparative anatomist, are now used as physiologic loci in human brain. Because of its current link with frontotemporal dementia, BA12's transition from animal (1909) to human (1910 and 1914) is not only an historical puzzle. It impacts how Brodmann's areas, based on comparative animal-human cytoarchitecture, are widely used in current research as functional loci in human brain.

  10. Systolic left ventricular function according to left ventricular concentricity and dilatation in hypertensive patients

    DEFF Research Database (Denmark)

    Bang, Casper; Gerdts, Eva; Aurigemma, Gerard P

    2013-01-01

    Left ventricular hypertrophy [LVH, high left ventricular mass (LVM)] is traditionally classified as concentric or eccentric based on left ventricular relative wall thickness. We evaluated left ventricular systolic function in a new four-group LVH classification based on left ventricular dilatation...

  11. A case study of an emerging visual artist with frontotemporal lobar degeneration and amyotrophic lateral sclerosis.

    Science.gov (United States)

    Liu, Anli; Werner, Kelly; Roy, Subhojit; Trojanowski, John Q; Morgan-Kane, Ursula; Miller, Bruce L; Rankin, Katherine P

    2009-06-01

    Patients presenting with left-sided FTLD syndromes sometimes develop a new preoccupation with art, greater attention to visual stimuli, and increased visual creativity. We describe the case of a 53-year-old, right-handed man with a history of bipolar disorder who presented with language and behavior impairments characteristic of FTLD, then developed motor symptoms consistent with a second diagnosis of amyotrophic lateral sclerosis. Though the patient had never created visual art before, he developed a compulsion for painting beginning at the earliest stages of his disease, and continued producing art daily until he could no longer lift a paintbrush because of his motor deficits. Upon autopsy, he was found to have ubiquitin and TDP43-positive inclusions with MND pathology. This case study details the patient's longitudinal neuropsychological, emotional, behavioral, and motor symptoms, along with structural imaging, neurologic, and neuropathologic findings. Multiple examples of the patient's art are depicted throughout all stages of his illness, and the possible cognitive, behavioral, and neurologic correlates of his new-onset visual artistry are discussed.

  12. Nonlinear left-handed transmission line metamaterials

    International Nuclear Information System (INIS)

    Kozyrev, A B; Weide, D W van der

    2008-01-01

    Metamaterials, exhibiting simultaneously negative permittivity ε and permeability μ, more commonly referred to as left-handed metamaterials (LHMs) and also known as negative-index materials, have received substantial attention in the scientific and engineering communities [1]. Most studies of LHMs (and electromagnetic metamaterials in general) have been in the linear regime of wave propagation and have already inspired new types of microwave circuits and devices. The results of these studies have already been the subject of numerous reviews and books. This review covers a less explored but rapidly developing area of investigation involving media that combine nonlinearity (dependence of the permittivity and permeability on the magnitude of the propagating field) with the anomalous dispersion exhibited by LHM. The nonlinear phenomena in such media will be considered on the example of a model system: the nonlinear left-handed transmission line. These nonlinear phenomena include parametric generation and amplification, harmonic and subharmonic generation as well as modulational instabilities and envelope solitons. (topical review)

  13. Rebuilding the US Health Left

    Directory of Open Access Journals (Sweden)

    Victor W. Sidel, MD

    2010-02-01

    Full Text Available With this issue Social Medicine begins a series of invited papers on the topic: “Rebuilding the US Health Left.” In this editorial we will outline our vision for this series. We undertake this project aware that our good friend and mentor, Dr. Walter Lear, one of the leading health activists of the 20th century, lies critically ill. Walter was the creator and custodian of the US Health Left Archives, a collection that is now with the University of Pennsylvania library. The collection reminds us of the important role left health care workers played in US history throughout the 20th century. They advocated for a national health program (Committee on the Costs of Medical Care, Physicians Forum, Medical Care Section/APHA, HealthPAC, Physicians for a National Health Program, National Physicians Alliance, provided international solidarity (American Soviet Medical Society, international brigades during the Spanish Civil War, Central American Solidarity Movement, Committee to Help Chilean Health Workers, Doctors for Global Health, traced the connections between disease and social class (Sigerist Circle, Spirit of 1848, APHA, fought for workers’ health (Councils for Occupational Safety and Health; Occupational Health and Safety Section, APHA participated in anti-war movements (Medical Committee for Human Rights, Physicians for Social Responsibility, International Physicians for the Prevention of Nuclear War, created new models of health care delivery (Health Cooperatives, Prepaid Health Maintenance Organizations, Community Health Centers, National Health Service Corps, Free Clinics, were central to the struggle for women’s rights (Planned Parenthood, Physicians for Reproductive Choice and Health, supported the civil rights movement both in medicine and in the broader society (National Medical Association, Medical Committee for Human Rights, played key roles in the movement for gay rights (ACT-UP, Gay & Lesbian Medical Association, Lesbian, Gay

  14. Blood pressure control and left ventricular hypertrophy in ...

    African Journals Online (AJOL)

    surface area (BSA). When LVM was indexed to height, left ventricular hypertrophy was found in none of the subjects of the normotensive group, while it was found present in 43 (22.4%) and 14 (24.1%) subjects of the uncontrolled and controlled hypertensive groups, respectively. Significant difference in the prevalence of ...

  15. The role of left posterior inferior temporal cortex in spelling.

    Science.gov (United States)

    Rapcsak, Steven Z; Beeson, Pélagie M

    2004-06-22

    To determine whether damage to left posterior inferior temporal cortex (PITC) is associated with agraphia and to characterize the nature of the spelling impairment. Left angular gyrus may play a critical role in spelling. However, this traditional view is challenged by reports of agraphia after left temporo-occipital lesions and by functional imaging studies demonstrating activation of left PITC during writing in normal individuals. Patients with focal damage to the left temporo-occipital cortex and normal control subjects were administered a comprehensive spelling battery that included regular words, irregular words, and nonwords as stimuli. Although patients performed worse than control subjects in all experimental conditions, the spelling deficit was particularly severe for irregular words, whereas regular word and nonword spelling were less impaired. Additional analyses indicated that orthographic regularity and word frequency had a much more pronounced effect on spelling accuracy in patients compared with control subjects. Most errors on irregular words were phonologically plausible, consistent with reliance on a sublexical phonologic spelling strategy (i.e., phoneme-grapheme conversion). Overall, the spelling impairment of the patients showed the characteristic profile of lexical agraphia. Lesion analyses indicated that the damage in the majority of patients encompassed an area within the left PITC (BA 37/20) where the authors previously obtained evidence of activation in a functional imaging study of writing in normal participants. The behavioral and neuroanatomic observations in the patients are consistent with functional imaging studies of writing in neurologically intact individuals and provide converging evidence for the role of left PITC in spelling. Together, these findings implicate left PITC as a possible neural substrate of the putative orthographic lexicon that contains stored memory representations for the written forms of familiar words.

  16. ODEs: is there anything left to do

    Energy Technology Data Exchange (ETDEWEB)

    Gear, C.W.

    1979-10-01

    Two decades ago many people thought that there was very little left to occupy the researcher in the numerical initial-value problem for ordinary differential equations, but there has since been an enormous increase in the effort devoted to this problem and related software. The question is whether everything is finally done so that more difficult areas can be studied. The development of numerical solution techniques is examined from the identification of a problem to the never-final preparation of automatic codes for the solution of classes of similar problems. A number of ODE problems that are only a part of the way along this path of development are discussed. These problems include ones with highly oscillatory solutions, ones with frequent discontinuities, and ones in which very little accuracy is needed (and which should be integrated at low cost). 4 figures.

  17. Semantic dementia and the left and right temporal lobes.

    Science.gov (United States)

    Snowden, Julie S; Harris, Jennifer M; Thompson, Jennifer C; Kobylecki, Christopher; Jones, Matthew; Richardson, Anna M; Neary, David

    2017-08-31

    Semantic dementia, a circumscribed disorder of semantic knowledge, provides a unique model for understanding the neural basis for semantic representation. The study addressed areas of contention: the relative roles of the left and right temporal lobe, the contribution of anterior versus posterior temporal cortex and the status of the anterior temporal lobes as amodal hub. Naming and word comprehension was examined in 41 semantic dementia patients, 31 with left-predominant and 10 right-predominant atrophy. In keeping with expectation, naming and comprehension were significantly poorer in left-predominant patients. Structural magnetic resonance image analysis, using a visual rating scale, showed strong inverse correlations between naming scores and severity of both left anterior and posterior temporal lobe atrophy. By contrast, comprehension performance was more strongly correlated with left posterior temporal atrophy. Analysis of naming errors revealed a correlation between anterior temporal atrophy and associative/functional descriptive responses, implying availability of semantic information. By contrast, 'don't know' responses, indicative of loss of semantic knowledge, were linked to left posterior temporal lobe atrophy. Semantic errors, the hallmark of semantic dementia, were linked to right hemisphere atrophy, especially the right posterior temporal lobe. Matched visual-verbal tasks (famous face and name identification, Pyramids and Palm trees pictures and words, animal knowledge from 3-D models and animal names) administered to nine patients elicited variable correspondence between performance on nonverbal and verbal versions of the task. Marked performance dissociations were demonstrated in some patients: poorer understanding of names/words in left-predominant patients and of faces/pictures/models in right-predominant cases. The findings are compatible with the notion of the anterior temporal lobes as areas of convergence, but are less easily accommodated

  18. Phase-Amplitude Coupling and Long-Range Phase Synchronization Reveal Frontotemporal Interactions during Visual Working Memory.

    Science.gov (United States)

    Daume, Jonathan; Gruber, Thomas; Engel, Andreas K; Friese, Uwe

    2017-01-11

    It has been suggested that cross-frequency phase-amplitude coupling (PAC), particularly in temporal brain structures, serves as a neural mechanism for coordinated working memory storage. In this magnetoencephalography study, we show that during visual working memory maintenance, temporal cortex regions, which exhibit enhanced PAC, interact with prefrontal cortex via enhanced low-frequency phase synchronization. Healthy human participants were engaged in a visual delayed match-to-sample task with pictures of natural objects. During the delay period, we observed increased spectral power of beta (20-28 Hz) and gamma (40-94 Hz) bands as well as decreased power of theta/alpha band (7-9 Hz) oscillations in visual sensory areas. Enhanced PAC between the phases of theta/alpha and the amplitudes of beta oscillations was found in the left inferior temporal cortex (IT), an area known to be involved in visual object memory. Furthermore, the IT was functionally connected to the prefrontal cortex by increased low-frequency phase synchronization within the theta/alpha band. Together, these results point to a mechanism in which the combination of PAC and long-range phase synchronization subserves enhanced large-scale brain communication. They suggest that distant brain regions might coordinate their activity in the low-frequency range to engage local stimulus-related processing in higher frequencies via the combination of long-range, within-frequency phase synchronization and local cross-frequency PAC. Working memory maintenance, like other cognitive functions, requires the coordinated engagement of brain areas in local and large-scale networks. However, the mechanisms by which spatially distributed brain regions share and combine information remain primarily unknown. We show that the combination of long-range, low-frequency phase synchronization and local cross-frequency phase-amplitude coupling might serve as a mechanism to coordinate memory processes across distant brain areas

  19. Echocardiographic assessment of inappropriate left ventricular mass and left ventricular hypertrophy in patients with diastolic dysfunction

    Directory of Open Access Journals (Sweden)

    Hasan Shemirani

    2012-01-01

    Full Text Available Background: early diagnosis of left ventricular mass (LVM inappropriateness and left ventricular hypertrophy (LVH can result in preventing diastolic left ventricular dysfunction and its related morbidity and mortality. This study was performed to determine if diastolic dysfunction is associated with LVH and inappropriate LVM. Materials and Methods: one hundred and twenty five uncomplicated hypertension from Isfahan Healthy Heart Program underwent two-dimensional echocardiography. Inappropriate LVM was defined as an LVM index greater than 88 g/m2 of body-surface area in women and greater than 102 g/m2 in men. LVH-defined septal and posterior wall thickness greater than 0/9 cm in women and greater than 1 cm in men, respectively. Echocardiographic parameters, including early diastolic peak velocity (E/late diastolic peak velocity (A, deceleration time (DT, and E/early mitral annulus velocity (E′ were measured. Results: the mean systolic and diastolic blood pressure at the patients′ admission day were 142.87 ± 18.12 and 88.45 ± 9.18 mmHg, respectively. Totally, 21.7% of subjects had inappropriate LV mass that moderate and severe abnormal LV mass was revealed in 5.6% and 5.6%, respectively. The mean of age and BMI was significantly higher in patients with moderate left ventricular hypertrophy (P 0.05. Spearman′s Rank test was used to test the correlation between diastolic dysfunction and LV mass (P = 0.025. Conclusion: LVH is correlated with the severity of diastolic dysfunction manifested by the E/A value and deceleration time, but inappropriate LVM can slightly predict diastolic dysfunction severity in uncomplicated hypertension.

  20. Left-handed Children in Singapore.

    Science.gov (United States)

    Gan, Linda

    1998-01-01

    Used teacher questionnaires to examine incidence of left-handedness in nearly 2,800 Singaporean children, racial differences in this left-handed population, and educational provisions in preschool and primary school. Findings indicated that 7.5% of preschoolers and 6.3% of primary children were left-handed, with a higher proportion being Chinese…

  1. The Left-Handed: "Their Sinister" History.

    Science.gov (United States)

    Costas, Elaine Fowler

    The history of left-handedness can provide teachers and parents a better understanding of left-handed children and give those children more pride in their difference. No child should be made to feel that he or she is abnormal because of using the left hand, although some specific instruction for these students is necessary in handwriting. Many…

  2. Effect of ferulic acid and Angelica archangelica extract on behavioral and psychological symptoms of dementia in frontotemporal lobar degeneration and dementia with Lewy bodies.

    Science.gov (United States)

    Kimura, Takemi; Hayashida, Hideki; Murata, Masako; Takamatsu, Junichi

    2011-07-01

    The behavioral and psychological symptoms of dementia place a heavy burden on caregivers. Antipsychotic drugs, though used to reduce the symptoms, frequently decrease patients' activities of daily living and reduce their quality of life. Recently, it was suggested that ferulic acid is an effective treatment for behavioral and psychological symptoms. We have also reported several patients with dementia with Lewy bodies showing good responses to ferulic acid and Angelica archangelica extract (Feru-guard). The present study investigated the efficacy of Feru-guard in the treatment of behavioral and psychological symptoms in frontotemporal lobar degeneration and dementia with Lewy bodies. We designed a prospective, open-label trial of daily Feru-guard (3.0 g/day) lasting 4 weeks in 20 patients with frontotemporal lobar degeneration or dementia with Lewy bodies. Behavioral and psychological symptoms of dementia were assessed at baseline and 4 weeks after the start of treatment, using the Neuropsychiatric Inventory. The Neuropsychiatric Inventory scores were analyzed using the Wilcoxon rank sum test. Treatment with Feru-guard led to decreased scores on the Neuropsychiatric Inventory in 19 of 20 patients and significantly decreased the score overall. The treatment also led to significantly reduced subscale scores on the Neuropsychiatric Inventory ("delusions", "hallucinations", "agitation/aggression", "anxiety", "apathy/indifference", "irritability/lability" and "aberrant behavior"). There were no adverse effects or significant changes in physical findings or laboratory data. Feru-guard may be effective and valuable for treating the behavioral and psychological symptoms of dementia in frontotemporal lobar degeneration and dementia with Lewy bodies. © 2011 Japan Geriatrics Society.

  3. Social Cognition Deficits: The Key to Discriminate Behavioral Variant Frontotemporal Dementia from Alzheimer's Disease Regardless of Amnesia?

    Science.gov (United States)

    Bertoux, Maxime; de Souza, Leonardo Cruz; O'Callaghan, Claire; Greve, Andrea; Sarazin, Marie; Dubois, Bruno; Hornberger, Michael

    2016-01-01

    Relative sparing of episodic memory is a diagnostic criterion of behavioral variant frontotemporal dementia (bvFTD). However, increasing evidence suggests that bvFTD patients can show episodic memory deficits at a similar level as Alzheimer's disease (AD). Social cognition tasks have been proposed to distinguish bvFTD, but no study to date has explored the utility of such tasks for the diagnosis of amnestic bvFTD. Here, we contrasted social cognition performance of amnestic and non-amnestic bvFTD from AD, with a subgroup having confirmed in vivo pathology markers. Ninety-six participants (38 bvFTD and 28 AD patients as well as 30 controls) performed the short Social-cognition and Emotional Assessment (mini-SEA). BvFTD patients were divided into amnestic versus non-amnestic presentation using the validated Free and Cued Selective Reminding Test (FCSRT) assessing episodic memory. As expected, the accuracy of the FCSRT to distinguish the overall bvFTD group from AD was low (69.7% ) with ∼50% of bvFTD patients being amnestic. By contrast, the diagnostic accuracy of the mini-SEA was high (87.9% ). When bvFTD patients were split on the level of amnesia, mini-SEA diagnostic accuracy remained high (85.1% ) for amnestic bvFTD versus AD and increased to very high (93.9% ) for non-amnestic bvFTD versus AD. Social cognition deficits can distinguish bvFTD and AD regardless of amnesia to a high degree and provide a simple way to distinguish both diseases at presentation. These findings have clear implications for the diagnostic criteria of bvFTD. They suggest that the emphasis should be on social cognition deficits with episodic memory deficits not being a helpful diagnostic criterion in bvFTD.

  4. Dementia Apraxia Test (DATE): A Brief Tool to Differentiate Behavioral Variant Frontotemporal Dementia from Alzheimer's Dementia Based on Apraxia Profiles.

    Science.gov (United States)

    Johnen, Andreas; Frommeyer, Jana; Modes, Fenja; Wiendl, Heinz; Duning, Thomas; Lohmann, Hubertus

    2016-01-01

    Standardized praxis assessments with modern, empirically validated screening tests have substantially improved clinical evaluation of apraxia in patients with stroke. Although apraxia may contribute to early differential diagnosis of Alzheimer's dementia (AD) and behavioral variant frontotemporal dementia (bvFTD), no comparable test is readily available to clinicians for this purpose to date. To design a clinically useful apraxia test for the differentiation of AD and bvFTD. 84 test items pertaining to twelve praxis subdomains were evaluated for their efficacy to discriminate between patients with bvFTD (n = 24), AD (n = 28), and elderly healthy controls (HC; n = 35). Items were then selected based on discriminative value and psychometric properties. Items indicative of mild AD comprised spatially complex imitation of hand and finger postures and to a lesser degree, pantomime of common object-use. Buccofacial apraxia including imitation of face postures, emblematic face postures, and repetition of multisyllabic pseudowords differentiated bvFTD from HC and AD. The final test version consisting of 20 items proved highly efficient for the discrimination of biologically confirmed dementia patients from HC (sensitivity 91% , specificity 71%) but also for differential diagnosis of bvFTD and AD (sensitivity 74% , specificity 93%). Assessment of praxis profiles effectively contributes to diagnosis and differential diagnosis of AD and bvFTD. The Dementia Apraxia Test (DATE) is a brief and easy to administer cognitive tool for dementia assessment, has a high inter-rater reliability (Cohen's κ= 0.885) and demonstrates content validity.

  5. Frontal and temporal lobe contributions to emotional enhancement of memory in behavioural-variant frontotemporal dementia and Alzheimer's disease

    Directory of Open Access Journals (Sweden)

    Fiona eKumfor

    2014-06-01

    Full Text Available Emotional events gain special priority in how they are remembered, with emotionally arousing events typically recalled more vividly and with greater confidence than non-emotional events. In dementia, memory and emotion processing are affected to varying degrees, however, whether emotional enhancement of memory for complex ecologically valid events is differentially affected across dementia syndromes remains unclear, with previous studies examining effects of emotion on simple visual recognition only. Here, we examined memory for an emotionally arousing short story and a closely matched, emotionally neutral story in behavioural-variant frontotemporal dementia (bvFTD (n = 13 and Alzheimer’s disease (AD (n = 14, and contrasted their performance with healthy controls (n = 12. Multiple-choice recognition memory for specific details of the story was assessed after a 1-hour delay. While AD and control groups showed enhanced memory for the emotional story, the bvFTD group recalled a similar number of details from the emotional and neutral stories. Voxel-based morphometry analyses revealed emotional enhancement of memory correlated with distinct brain regions in each patient group. In AD, emotional enhancement was associated with integrity of the bilateral hippocampus, parahippocampal gyri, temporal fusiform gyrus and frontal pole, regions implicated in memory processes. In contrast in bvFTD, integrity of emotion processing regions, including the orbitofrontal cortex, right amygdala and right insula, correlated with the extent emotion enhanced memory. Our results reveal that integrity of frontal and temporal regions determine the quality and nature of emotional memories. While emotional enhancement of memory is present in mild AD, in bvFTD emotion does not facilitate memory retrieval for complex realistic events. This attenuation of emotional enhancement is due to degradation of emotion processing regions, which may be important for modulating levels

  6. The clinical, neuroanatomical, and neuropathologic phenotype ofTBK1-associated frontotemporal dementia: A longitudinal case report.

    Science.gov (United States)

    Koriath, Carolin A M; Bocchetta, Martina; Brotherhood, Emilie; Woollacott, Ione O C; Norsworthy, Penny; Simón-Sánchez, Javier; Blauwendraat, Cornelis; Dick, Katrina M; Gordon, Elizabeth; Harding, Sophie R; Fox, Nick C; Crutch, Sebastian; Warren, Jason D; Revesz, Tamas; Lashley, Tammaryn; Mead, Simon; Rohrer, Jonathan D

    2017-01-01

    Mutations in the TANK-binding kinase 1 ( TBK1 ) gene have recently been shown to cause frontotemporal dementia (FTD). However, the phenotype of TBK1 -associated FTD is currently unclear. We performed a single case longitudinal study of a patient who was subsequently found to have a novel A705fs mutation in the TBK1 gene. He was assessed annually over a 7-year period with a series of clinical, cognitive, and magnetic resonance imaging assessments. His brain underwent pathological examination at postmortem. The patient presented at the age of 64 years with an 18-month history of personality change including increased rigidity and obsessiveness, apathy, loss of empathy, and development of a sweet tooth. His mother had developed progressive behavioral and cognitive impairment from the age of 57 years. Neuropsychometry revealed intact cognition at first assessment. Magnetic resonance imaging showed focal right temporal lobe atrophy. Over the next few years his behavioral problems progressed and he developed cognitive impairment, initially with anomia and prosopagnosia. Neurological examination remained normal throughout without any features of motor neurone disease. He died at the age of 72 years and postmortem showed TDP-43 type A pathology but with an unusual novel feature of numerous TAR DNA-binding protein 43 (TDP-43)-positive neuritic structures at the cerebral cortex/subcortical white matter junction. There was also associated argyrophilic grain disease not previously reported in other TBK1 mutation cases. TBK1 -associated FTD can be associated with right temporal variant FTD with progressive behavioral change and relatively intact cognition initially. The case further highlights the benefits of next-generation sequencing technologies in the diagnosis of neurodegenerative disorders and the importance of detailed neuropathologic analysis.

  7. Optimization of a 3D Dynamic Culturing System for In Vitro Modeling of Frontotemporal Neurodegeneration-Relevant Pathologic Features.

    Science.gov (United States)

    Tunesi, Marta; Fusco, Federica; Fiordaliso, Fabio; Corbelli, Alessandro; Biella, Gloria; Raimondi, Manuela T

    2016-01-01

    Frontotemporal lobar degeneration (FTLD) is a severe neurodegenerative disorder that is diagnosed with increasing frequency in clinical setting. Currently, no therapy is available and in addition the molecular basis of the disease are far from being elucidated. Consequently, it is of pivotal importance to develop reliable and cost-effective in vitro models for basic research purposes and drug screening. To this respect, recent results in the field of Alzheimer's disease have suggested that a tridimensional (3D) environment is an added value to better model key pathologic features of the disease. Here, we have tried to add complexity to the 3D cell culturing concept by using a microfluidic bioreactor, where cells are cultured under a continuous flow of medium, thus mimicking the interstitial fluid movement that actually perfuses the body tissues, including the brain. We have implemented this model using a neuronal-like cell line (SH-SY5Y), a widely exploited cell model for neurodegenerative disorders that shows some basic features relevant for FTLD modeling, such as the release of the FTLD-related protein progranulin (PRGN) in specific vesicles (exosomes). We have efficiently seeded the cells on 3D scaffolds, optimized a disease-relevant oxidative stress experiment (by targeting mitochondrial function that is one of the possible FTLD-involved pathological mechanisms) and evaluated cell metabolic activity in dynamic culture in comparison to static conditions, finding that SH-SY5Y cells cultured in 3D scaffold are susceptible to the oxidative damage triggered by a mitochondrial-targeting toxin (6-OHDA) and that the same cells cultured in dynamic conditions kept their basic capacity to secrete PRGN in exosomes once recovered from the bioreactor and plated in standard 2D conditions. We think that a further improvement of our microfluidic system may help in providing a full device where assessing basic FTLD-related features (including PRGN dynamic secretion) that may be

  8. Brain reserve capacity in frontotemporal dementia: a voxel-based {sup 18}F-FDG PET study

    Energy Technology Data Exchange (ETDEWEB)

    Perneczky, Robert; Diehl-Schmid, Janine; Kurz, Alexander [Technische Universitaet Muenchen, Klinik und Poliklinik fuer Psychiatrie und Psychotherapie, Muenchen (Germany); Drzezga, Alexander [Technische Universitaet Muenchen, Nuklearmedizinische Klinik, Muenchen (Germany)

    2007-07-15

    The association of the regional cerebral metabolic rate of glucose utilisation (rCMRglc) and years of schooling has been extensively studied in Alzheimer's disease (AD). The results suggest that brain reserve capacity (BRC) allows patients with more years of schooling to cope better with AD pathology. The objective of this study was to provide initial evidence for BRC in frontotemporal dementia (FTD). Twenty-nine patients with FTD and 16 healthy age- and education-matched controls underwent PET imaging of the brain with {sup 18}F-fluoro-2-deoxy-glucose. A group comparison of rCMRglc was conducted between patients and controls and the output was saved as region of interest (ROI). A linear regression analysis with education as the independent and rCMRglc as the dependent variable, adjusted for age, gender and total score on the CERAD neuropsychological battery, was conducted in SPM2 over the pre-assigned ROI. Patients showed a reduced rCMRglc in almost the entire prefrontal cortex and the anterior cingulate cortex as compared with controls (p < 0.05 corrected for multiple comparisons). The regression analysis revealed a significant negative association between years of schooling and rCMRglc in the bilateral inferior frontal cortex (p < 0.001, uncorrected for multiple comparisons), which was independent of demographic variables and cognitive performance level. There was a strong negative correlation of rCMRglc and education (r = -0.45). The study provides initial evidence for BRC in FTD. The findings suggest that interindividual differences in educational level affect BRC by partially mediating the relationship between neurodegeneration and the clinical manifestation of FTD. (orig.)

  9. Dissociating Memory Networks in Early Alzheimer’s Disease and Frontotemporal Lobar Degeneration - A Combined Study of Hypometabolism and Atrophy

    Science.gov (United States)

    Frisch, Stefan; Dukart, Juergen; Vogt, Barbara; Horstmann, Annette; Becker, Georg; Villringer, Arno; Barthel, Henryk; Sabri, Osama; Müller, Karsten; Schroeter, Matthias L.

    2013-01-01

    Introduction We aimed at dissociating the neural correlates of memory disorders in Alzheimer’s disease (AD) and frontotemporal lobar degeneration (FTLD). Methods We included patients with AD (n = 19, 11 female, mean age 61 years) and FTLD (n = 11, 5 female, mean age 61 years) in early stages of their diseases. Memory performance was assessed by means of verbal and visual memory subtests from the Wechsler Memory Scale (WMS-R), including forgetting rates. Brain glucose utilization was measured by [18F]fluorodeoxyglucose positron emission tomography (FDG-PET) and brain atrophy by voxel-based morphometry (VBM) of T1-weighted magnetic resonance imaging (MRI) scans. Using a whole brain approach, correlations between test performance and imaging data were computed separately in each dementia group, including a group of control subjects (n = 13, 6 female, mean age 54 years) in both analyses. The three groups did not differ with respect to education and gender. Results Patients in both dementia groups generally performed worse than controls, but AD and FTLD patients did not differ from each other in any of the test parameters. However, memory performance was associated with different brain regions in the patient groups, with respect to both hypometabolism and atrophy: Whereas in AD patients test performance was mainly correlated with changes in the parieto-mesial cortex, performance in FTLD patients was correlated with changes in frontal cortical as well as subcortical regions. There were practically no overlapping regions associated with memory disorders in AD and FTLD as revealed by a conjunction analysis. Conclusion Memory test performance may not distinguish between both dementia syndromes. In clinical practice, this may lead to misdiagnosis of FTLD patients with poor memory performance. Nevertheless, memory problems are associated with almost completely different neural correlates in both dementia syndromes. Obviously, memory functions are carried out by

  10. Episodic Memory Dysfunction in Behavioral Variant Frontotemporal Dementia: A Clinical And FDG-PET Study.

    Science.gov (United States)

    Fernández-Matarrubia, Marta; Matías-Guiu, Jordi A; Cabrera-Martín, María Nieves; Moreno-Ramos, Teresa; Valles-Salgado, María; Carreras, José Luis; Matías-Guiu, Jorge

    2017-01-01

    Episodic memory disturbance is still considered as an exclusion criterion for behavioral variant frontotemporal dementia (bvFTD), but growing evidence suggests that memory can be impaired. Our main purposes were to assess episodic memory in a group of bvFTD patients comparatively with Alzheimer's disease (AD) patients, and analyze the relationship between episodic memory and brain metabolism measured using positron emission tomography imaging with 18F-fluorodeoxyglucose (FDG-PET). Twenty-six bvFTD, 29 AD, and 24 healthy controls were included. Episodic memory was assessed by the Free and Cued Selective Reminding Test (FCSRT), which controls for effective encoding and measures memory consolidation processing. All participants underwent FDG-PET brain scans to provide data for voxel-based brain mapping analysis. Half of bvFTD patients had a deficit of total, free delayed, and total free delayed recall as severe as AD patients (amnestic-FTD). The other half had FCSRT scores similar to controls (non-amnestic-FTD). Imaging analyses revealed that amnestic-FTD showed bilateral lower metabolism than non-amnestic-FTD in anterior parahippocampal and inferior temporal gyri. Additionally, FCSRT total and total delayed scores were inversely correlated with parahippocampal metabolism in both bvFTD and AD. Besides, bvFTD showed an inverse association among FCSRT and inferior temporal metabolism. Our findings support that bvFTD could present a genuine amnesia affecting storage and consolidation abilities, which involves structures implicated in the Papez circuit, as occurs in AD, and also inferior temporal regions. These results contribute to understanding the mechanisms underpinning memory dysfunction in bvFTD, and may be relevant to further revisions of the current diagnostic criteria.

  11. Cerebral perfusion and glucose metabolism in Alzheimer's disease and frontotemporal dementia: two sides of the same coin?

    Energy Technology Data Exchange (ETDEWEB)

    Verfaillie, Sander C.J.; Adriaanse, Sofie M.; Binnewijzend, Maja A.A.; Benedictus, Marije R.; Ossenkoppele, Rik [VU University Medical Centre, Department of Radiology and Nuclear Medicine, Amsterdam (Netherlands); VU University Medical Centre, Alzheimer Centre and Department of Neurology, P.O. Box 7057, Amsterdam (Netherlands); Wattjes, Mike P.; Lammertsma, Adriaan A.; Boellaard, Ronald; Berckel, Bart N.M. van; Barkhof, Frederik [VU University Medical Centre, Department of Radiology and Nuclear Medicine, Amsterdam (Netherlands); Pijnenburg, Yolande A.L.; Scheltens, Philip [VU University Medical Centre, Alzheimer Centre and Department of Neurology, P.O. Box 7057, Amsterdam (Netherlands); Flier, Wiesje M. van der [VU University Medical Centre, Alzheimer Centre and Department of Neurology, P.O. Box 7057, Amsterdam (Netherlands); VU University Medical Centre, Department of Epidemiology and Biostatistics, Amsterdam (Netherlands); Kuijer, Joost P.A. [VU University Medical Centre, Department of Physics and Medical Technology, Amsterdam (Netherlands)

    2015-10-15

    Alzheimer's disease (AD) and frontotemporal (FTD) dementia can be differentiated using [{sup 18}F]-2-deoxy-2-fluoro-D-glucose (FDG)-PET. Since cerebral blood flow (CBF) is related to glucose metabolism, our aim was to investigate the extent of overlap of abnormalities between AD and FTD. Normalized FDG-PET and arterial spin labelling (ASL-MRI)-derived CBF was measured in 18 AD patients (age, 64 ± 8), 12 FTD patients (age, 61 ± 8), and 10 controls (age, 56 ± 10). Voxel-wise comparisons, region-of-interest (ROI), correlation, and ROC curve analyses were performed. Voxel-wise comparisons showed decreased CBF and FDG uptake in AD compared with controls and FTD in both precuneus and inferior parietal lobule (IPL). Compared with controls and AD, FTD patients showed both hypometabolism and hypoperfusion in medial prefrontal cortex (mPFC). ASL and FDG were related in precuneus (r = 0.62, p < 0.001), IPL (r = 0.61, p < 0.001), and mPFC across groups (r = 0.74, p < 001). ROC analyses indicated comparable performance of perfusion and metabolism in the precuneus (AUC, 0.72 and 0.74), IPL (0.85 and 0.94) for AD relative to FTD, and in the mPFC in FTD relative to AD (both 0.68). Similar patterns of hypoperfusion and hypometabolism were observed in regions typically associated with AD and FTD, suggesting that ASL-MRI provides information comparable to FDG-PET. (orig.)

  12. Perfis Diferenciais de Perda de Memória entre a Demência Frontotemporal e a do Tipo Alzheimer

    Directory of Open Access Journals (Sweden)

    Allegri Ricardo F.

    2001-01-01

    Full Text Available Os estados iniciais da demência tipo Alzheimer (DTA caracterizam-se classicamente por deterioração da memória enquanto que as mudanças de conduta e de personalidade aparecem nas etapas iniciais da demência frontotemporal (DFT. Entretanto, na prática clínica, o diagnóstico diferencial é difícil. O objetivo do presente trabalho foi estudar o rendimento da memória de pacientes com DTA (n= 20 e com DFT (n= 20 comparando-o com um grupo de controles (n = 20. Os pacientes, emparelhados por idade e escolaridade, foram avaliados com uma bateria neuropsicológica exaustiva. Para a avaliação da memória, examinou-se a "queixa subjetiva" de perda de memória (memória subjetiva, a aprendizagem de uma lista de palavras (memória episódica e o desempenho no teste de denominação de Boston (memória semântica. As pontuações de ambos os grupos de pacientes, na grande maioria das provas, foram significativamente inferiores às dos controles. Os pacientes com DTA mostraram uma deterioração global da memória episódica (tipo amnésia e semântica com um alto nível de queixa subjetiva. Os sujeitos com DFT, por outro lado, apresentaram um déficit de memória importante na recuperação da informação, mas com melhores capacidades de registro da informação, apesar das dificuldades de reconhecimento do seu distúrbio.

  13. Comparison of arterial spin labeling registration strategies in the multi-center GENetic frontotemporal dementia initiative (GENFI).

    Science.gov (United States)

    Mutsaerts, Henri J M M; Petr, Jan; Thomas, David L; De Vita, Enrico; Cash, David M; van Osch, Matthias J P; Golay, Xavier; Groot, Paul F C; Ourselin, Sebastien; van Swieten, John; Laforce, Robert; Tagliavini, Fabrizio; Borroni, Barbara; Galimberti, Daniela; Rowe, James B; Graff, Caroline; Pizzini, Francesca B; Finger, Elizabeth; Sorbi, Sandro; Castelo Branco, Miguel; Rohrer, Jonathan D; Masellis, Mario; MacIntosh, Bradley J

    2018-01-01

    To compare registration strategies to align arterial spin labeling (ASL) with 3D T1-weighted (T1w) images, with the goal of reducing the between-subject variability of cerebral blood flow (CBF) images. Multi-center 3T ASL data were collected at eight sites with four different sequences in the multi-center GENetic Frontotemporal dementia Initiative (GENFI) study. In a total of 48 healthy controls, we compared the following image registration options: (I) which images to use for registration (perfusion-weighted images [PWI] to the segmented gray matter (GM) probability map (pGM) (CBF-pGM) or M0 to T1w (M0-T1w); (II) which transformation to use (rigid-body or non-rigid); and (III) whether to mask or not (no masking, M0-based FMRIB software library Brain Extraction Tool [BET] masking). In addition to visual comparison, we quantified image similarity using the Pearson correlation coefficient (CC), and used the Mann-Whitney U rank sum test. CBF-pGM outperformed M0-T1w (CC improvement 47.2% ± 22.0%; P Masking only improved the M0-T1w rigid-body registration (14.5% ± 15.5%; P = 0.007). The choice of image registration strategy impacts ASL group analyses. The non-rigid transformation is promising but requires validation. CBF-pGM rigid-body registration without masking can be used as a default strategy. In patients with expansive perfusion deficits, M0-T1w may outperform CBF-pGM in sequences with high effective spatial resolution. BET-masking only improves M0-T1w registration when the M0 image has sufficient contrast. 1 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2018;47:131-140. © 2017 International Society for Magnetic Resonance in Medicine.

  14. Spatio-temporal and kinematic gait analysis in patients with Frontotemporal dementia and Alzheimer's disease through 3D motion capture.

    Science.gov (United States)

    Rucco, Rosaria; Agosti, Valeria; Jacini, Francesca; Sorrentino, Pierpaolo; Varriale, Pasquale; De Stefano, Manuela; Milan, Graziella; Montella, Patrizia; Sorrentino, Giuseppe

    2017-02-01

    Alzheimer's disease (AD) and behavioral variant of Frontotemporal Dementia (bvFTD) are characterized respectively by atrophy in the medial temporal lobe with memory loss and prefrontal and anterior temporal degeneration with dysexecutive syndrome. In this study, we hypothesized that specific gait patterns are induced by either frontal or temporal degeneration. To test this hypothesis, we studied the gait pattern in bvFTD (23) and AD (22) patients in single and dual task ("motor" and "cognitive") conditions. To detect subtle alterations, we performed motion analysis estimating both spatio-temporal parameters and joint excursions. In the single task condition, the bvFTD group was more unstable and slower compared to healthy subjects, while only two stability parameters were compromised in the AD group. During the motor dual task, both velocity and stability parameters worsened further in the bvFTD group. In the same experimental conditions, AD patients showed a significantly lower speed and stride length than healthy subjects. During the cognitive dual task, a further impairment of velocity and stability parameters was observed in the bvFTD group. Interestingly, during the cognitive dual task, the gait performance of the AD group markedly deteriorated, as documented by the impairment of more indices of velocity and stability. Finally, the kinematic data of thigh, knee, and ankle were more helpful in revealing gait impairment than the spatio-temporal parameters alone. In conclusion, our data showed that the dysexecutive syndrome induces specific gait alterations. Furthermore, our results suggest that the gait worsens in the AD patients when the cognitive resources are stressed. Copyright © 2016 Elsevier B.V. All rights reserved.

  15. Agraphia in patients with frontotemporal dementia and parkinsonism linked to chromosome 17 with P301L MAPT mutation: dysexecutive, aphasic, apraxic or spatial phenomenon?

    Science.gov (United States)

    Sitek, Emilia J; Narozanska, Ewa; Barczak, Anna; Jasinska-Myga, Barbara; Harciarek, Michał; Chodakowska-Zebrowska, Małgorzata; Kubiak, Małgorzata; Wieczorek, Dariusz; Konieczna, Seweryna; Rademakers, Rosa; Baker, Matt; Berdynski, Mariusz; Brockhuis, Bogna; Barcikowska, Maria; Zekanowski, Cezary; Heilman, Kenneth M; Wszolek, Zbigniew K; Slawek, Jarosław

    2014-01-01

    Patients with frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) may be agraphic. The study aimed at characterizing agraphia in individuals with a P301L MAPT mutation. Two pairs of siblings with FTDP-17 were longitudinally examined for agraphia in relation to language and cognitive deficits. All patients presented with dysexecutive agraphia. In addition, in the first pair of siblings one sibling demonstrated spatial agraphia with less pronounced allographic agraphia and the other sibling had aphasic agraphia. Aphasic agraphia was also present in one sibling from the second pair. Agraphia associated with FTDP-17 is very heterogeneous.

  16. Is blue light, cryptochrome in the eye, and magnetite in the brain involved in the development of frontotemporal dementia and other diseases?

    Science.gov (United States)

    Størmer, Fredrik C

    2015-04-01

    When cryptochrome in the retina is exposed to blue light, it undergo series of complicated chemical reactions. One of these intermediates has magnetic properties. It could be a link between the magnetic stage of cryptochrome in the retina and magnetite in the brain. A disturbance in this system could be involved in the development of frontotemporal dementia and other mental disturbances like Alzheimer's disease. There could also be a link between circadian rhythms and memory dysfunction connected to schizophrenia, type 2 diabetes, and blue light. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Flavoured Dark Matter moving left

    Science.gov (United States)

    Blanke, Monika; Das, Satrajit; Kast, Simon

    2018-02-01

    We investigate the phenomenology of a simplified model of flavoured Dark Matter (DM), with a dark fermionic flavour triplet coupling to the left-handed SU(2) L quark doublets via a scalar mediator. The DM-quark coupling matrix is assumed to constitute the only new source of flavour and CP violation, following the hypothesis of Dark Minimal Flavour Violation. We analyse the constraints from LHC searches, from meson mixing data in the K, D, and B d,s meson systems, from thermal DM freeze-out, and from direct detection experiments. Our combined analysis shows that while the experimental constraints are similar to the DMFV models with DM coupling to right-handed quarks, the multitude of couplings between DM and the SM quark sector resulting from the SU(2) L structure implies a richer phenomenology and significantly alters the resulting impact on the viable parameter space.

  18. Reorganization of the Cerebro-Cerebellar Network of Language Production in Patients with Congenital Left-Hemispheric Brain Lesions

    Science.gov (United States)

    Lidzba, K.; Wilke, M.; Staudt, M.; Krageloh-Mann, I.; Grodd, W.

    2008-01-01

    Patients with congenital lesions of the left cerebral hemisphere may reorganize language functions into the right hemisphere. In these patients, language production is represented homotopically to the left-hemispheric language areas. We studied cerebellar activation in five patients with congenital lesions of the left cerebral hemisphere to assess…

  19. Utilidad de la batería ejecutiva y cognición social de INECO en la evaluación de funciones ejecutivas de una serie de pacientes peruanos con demencia fronto-temporal, variante conductual.

    OpenAIRE

    Custodio, Nilton; Herrera-Pérez, Eder; Lira, David; Montesinos, Rosa; Alva-Diaz, Carlos; Cortijo, Patricia; Cuenca-Alfaro, José; Valeriano-Lorenzo, Elizabeth

    2015-01-01

    Los síntomas característicos de la demencia fronto-temporal, variante conductual reflejan disfunción del cortex prefrontal ventromedial, y no existe consenso respecto a las pruebas neuropsicológicas más sensibles y específicos para evaluarlos. Objetivos: El objetivo de este estudio fue detectar alteraciones específicas de funciones ejecutivas y de cognición social en pacientes con demencia fronto-temporal variante conductual. Material y Métodos: Evaluamos la batería ejecutiva y cognición soci...

  20. A visual MRI atrophy rating scale for the amyotrophic lateral sclerosis-frontotemporal dementia continuum.

    Science.gov (United States)

    Ambikairajah, Ananthan; Devenney, Emma; Flanagan, Emma; Yew, Belinda; Mioshi, Eneida; Kiernan, Matthew C; Hodges, John R; Hornberger, Michael

    2014-06-01

    Our objective was to distinguish ALS, ALS-FTD and bvFTD via a novel visual MRI cortical atrophy scale that can be employed in a clinical setting. MRI images of 100 participants (33 ALS, 11 ALS-FTD, 22 bvFTD and 34 controls) were rated in four brain areas: orbitofrontal cortex, anterior temporal pole, anterior cingulate, and motor cortex. Areas were rated on a 5- point Likert scale by two raters blinded to the diagnosis. Results demonstrated that bvFTD patients showed the highest levels of atrophy across all regions, while ALS patients had the lowest atrophy scores. ALS-FTD patients have higher atrophy ratings compared to ALS patients for the motor cortex, anterior cingulate and anterior temporal lobe, with a statistical trend for the orbitofrontal cortex. ALS-FTD patients were not significantly different from bvFTD for any of the brain regions. These findings were confirmed in a post hoc VBM analysis of the same participants. Our study demonstrates that a simple visual MRI rating scale can reliably distinguish ALS, ALS-FTD and bvFTD atrophy patterns in a clinical setting. Motor cortex, anterior cingulate and anterior temporal atrophy emerged as good diagnostic markers for ALS-FTD. Employment of this MRI rating scale can complement clinical diagnostics of patients in the ALS-FTD continuum.

  1. Value of the radiological study of the thorax for diagnosing left ventricular dysfunction in Chagas' disease

    Directory of Open Access Journals (Sweden)

    Perez Amanda Arantes

    2003-01-01

    Full Text Available OBJECTIVE: To determine the value of the radiological study of the thorax for diagnosing left ventricular dilation and left ventricular systolic dysfunction in patients with Chagas' disease. METHODS: A cross-sectional study of 166 consecutive patients with Chagas' disease and no other associated diseases. The patients underwent cardiac assessment with chest radiography and Doppler echocardiography. Sensitivity, specificity, and positive and negative predictive values of chest radiography were calculated to detect left ventricular dysfunction and the accuracy of the cardiothoracic ratio in the diagnosis of left ventricular dysfunction with the area below the ROC curve. The cardiothoracic ratio was correlated with the left ventricular ejection fraction and the left ventricular diastolic diameter. RESULTS: The abnormal chest radiogram had a sensitivity of 50%, specificity of 80.5%, and positive and negative predictive values of 51.2% and 79.8%, respectively, in the diagnosis of left ventricular dysfunction. The cardiothoracic ratio showed a weak correlation with left ventricular ejection fraction (r=-0.23 and left ventricular diastolic diameter (r=0.30. The area calculated under the ROC curve was 0.734. CONCLUSION: The radiological study of the thorax is not an accurate indicator of left ventricular dysfunction; its use as a screening method to initially approach the patient with Chagas' disease should be reevaluated.

  2. Right ventricular dysfunction affects survival after surgical left ventricular restoration.

    Science.gov (United States)

    Couperus, Lotte E; Delgado, Victoria; Palmen, Meindert; van Vessem, Marieke E; Braun, Jerry; Fiocco, Marta; Tops, Laurens F; Verwey, Harriëtte F; Klautz, Robert J M; Schalij, Martin J; Beeres, Saskia L M A

    2017-04-01

    Several clinical and left ventricular parameters have been associated with prognosis after surgical left ventricular restoration in patients with ischemic heart failure. The aim of this study was to determine the prognostic value of right ventricular function. A total of 139 patients with ischemic heart failure (62 ± 10 years; 79% were male; left ventricular ejection fraction 27% ± 7%) underwent surgical left ventricular restoration. Biventricular function was assessed with echocardiography before surgery. The independent association between all-cause mortality and right ventricular fractional area change, tricuspid annular plane systolic excursion, and right ventricular longitudinal peak systolic strain was assessed. The additive effect of multiple impaired right ventricular parameters on mortality also was assessed. Baseline right ventricular fractional area change was 42% ± 9%, tricuspid annular plane systolic excursion was 18 ± 3 mm, and right ventricular longitudinal peak systolic strain was -24% ± 7%. Within 30 days after surgery, 15 patients died. Right ventricular fractional area change (hazard ratio, 0.93; 95% confidence interval, 0.88-0.98; P right ventricular longitudinal peak systolic strain (hazard ratio, 1.15; 95% confidence interval, 1.05-1.26; P Right ventricular function was impaired in 21%, 20%, and 27% of patients on the basis of right ventricular fractional area change, tricuspid annular plane systolic excursion, and right ventricular longitudinal peak systolic strain, respectively. Any echocardiographic parameter of right ventricular dysfunction was present in 39% of patients. The coexistence of several impaired right ventricular parameters per patient was independently associated with increased 30-day mortality (hazard ratio, 2.83; 95% confidence interval, 1.64-4.87, P right ventricular systolic dysfunction is independently associated with increased mortality in patients with ischemic heart failure undergoing surgical left

  3. Aphasia following left thalamic hemorrhage. A study by Western Aphasia Battery and single photon emission CT

    Energy Technology Data Exchange (ETDEWEB)

    Makishita, Hideo; Miyasaka, Motomaro; Tanizaki, Yoshio; Yanagisawa, Nobuo; Sugishita, Morihiro

    1984-07-01

    A report is given of 7 patients with left thalamic hemorrhage in the chronic stage (from 1.5 months to 4.5 months) in which language disorders were examined by Western Aphasia Battery (WAB) and cerebral blood flow was measured by single photon emission CT. Examination of language by WAB revealed 4 aphasics out of 7 cases, and 3 patients had no language deficit. The patient with Wernicke's aphasia showed low density area only in the left posterior thalamus in X-ray CT, and revealed severe low blood flow area extending to left temporal lobe in emission CT. In the case with transcortical sensory aphasia, although X-ray CT showed no obvious low density area, emission CT revealed moderate low flow area in the left temporooccipital region and low blood flow at the left thalamus. In one of the two patients classified as anomic aphasia, emission CT showed slight low flow area at the temporo-occipital region similar to the case with transcortical sensory aphasia. In another case with anomic aphasia there was a wide low density area all over the left thalamus and midline shift to the right in X-ray CT, and emission CT showed severe low blood flow in the same region spreading widely toward the cerebral surface. In all of the 3 patients without aphasia, emission CT showed low flow region restricted to the left thalamus.

  4. Problems of Face Recognition in Patients with Behavioral Variant Frontotemporal Dementia.

    Science.gov (United States)

    Chandra, Sadanandavalli Retnaswami; Patwardhan, Ketaki; Pai, Anupama Ramakanth

    2017-01-01

    Faces are very special as they are most essential for social cognition in humans. It is partly understood that face processing in its abstractness involves several extra striate areas. One of the most important causes for caregiver suffering in patients with anterior dementia is lack of empathy. This apart from being a behavioral disorder could be also due to failure to categorize the emotions of the people around them. Inlusion criteria: DSM IV for Bv FTD Tested for prosopagnosia - familiar faces, famous face, smiling face, crying face and reflected face using a simple picture card (figure 1). Advanced illness and mixed causes. 46 patients (15 females, 31 males) 24 had defective face recognition. (mean age 51.5),10/15 females (70%) and 14/31males(47. Familiar face recognition defect was found in 6/10 females and 6/14 males. Total- 40%(6/15) females and 19.35%(6/31)males with FTD had familiar face recognition. Famous Face: 9/10 females and 7/14 males. Total- 60% (9/15) females with FTD had famous face recognition defect as against 22.6%(7/31) males with FTD Smiling face defects in 8/10 female and no males. Total- 53.33% (8/15) females. Crying face recognition defect in 3/10 female and 2 /14 males. Total- 20%(3/15) females and 6.5%(2/31) males. Reflected face recognition defect in 4 females. Famous face recognition and positive emotion recognition defect in 80%, only 20% comprehend positive emotions, Face recognition defects are found in only 45% of males and more common in females. Face recognition is more affected in females with FTD There is differential involvement of different aspects of the face recognition could be one of the important factor underlying decline in the emotional and social behavior of these patients. Understanding these pathological processes will give more insight regarding patient behavior.

  5. Compression syndrome of the left renal vein

    Energy Technology Data Exchange (ETDEWEB)

    Justich, E.

    1982-04-01

    Severe compression of the left renal vein produces a pressure gradient between it and the inferior vena cava and results in changes in haemodynamics. The cause of the narrowing is usually the aorta, less commonly the superior mesenteric artery. Compression of the left renal vein may be responsible for a number of abnormalities such as primary varicoceles, primary varices of the ovarian, renal, pelvic and ureteric veins on the left, the more frequent occurrence of unilateral renal vein thrombosis on the left and the development of renovascular hypertension. One hundred and twenty-three selective phlebograms of the left renal vein and CT examinations of this structure in a further 87 patients acting as a control group were carried out. The significance of compression of the left renal vein as an aetiological factor in the development of the above mentioned abnormalities is discussed.

  6. Mistakes, Too Few to Mention? Impaired Self-conscious Emotional Processing of Errors in the Behavioral Variant of Frontotemporal Dementia

    Directory of Open Access Journals (Sweden)

    Carole S. Scherling

    2017-10-01

    Full Text Available Anosognosia, or lack of awareness of one's deficits, is a core feature of the behavioral variant of frontotemporal dementia (bvFTD. We hypothesized that this deficit has its origins in failed emotional processing of errors. We studied autonomic and facial emotional reactivity to errors in patients with bvFTD (n = 17, Alzheimer's disease (AD, n = 20, and healthy controls (HC, n = 35 during performance of a timed two-alternative-choice button press task. Performance-related behavioral responses to errors were quantified using rates of error correction and post-error slowing of reaction times. Facial emotional responses were measured by monitoring facial reactivity via video and subsequently coding the type, duration and intensity of all emotional reactions. Skin conductance response (SCR was measured via noninvasive sensors. SCR and total score for each facial emotion expression were quantified for each trial. Facial emotions were grouped into self-conscious (amusement, embarrassment and negative (fear, sadness, anger, disgust, contempt emotions. HCs corrected 99.4% of their errors. BvFTD patients corrected 94% (not statistically different compared with HC and AD corrected 74.8% of their errors (p < 0.05 compared with HC and bvFTD. All groups showed similar post-error slowing. Errors in HCs were associated with greater facial reactivity and SCRs compared with non-error trials, including both negative and self-conscious emotions. BvFTD patients failed to produce self-conscious emotions or an increase in SCR for errors, although they did produce negative emotional responses to a similar degree as HCs. AD showed no deficit in facial reactivity to errors. Although, SCR was generally reduced in AD during error trials, they showed a preserved increase in SCR for errors relative to correct trials. These results demonstrate a specific deficit in emotional responses to errors in bvFTD, encompassing both physiological response and a specific deficit in self

  7. The Relationship between Left Atrial Mechanical Function and Functional Capacity in Mitral Stenosis

    Directory of Open Access Journals (Sweden)

    Mücahit Yetim

    2013-11-01

    Full Text Available Aim: In this study, left atrial functions of patients with rheumatic mitral stenosis and sinus rhythm, which was determined by transthorasic echocardiography, was compared with those of healhty subjects and the association of left atrial functions with functional capacity was investigated in subgroup analyses.   Material and methods: 32 patients with isolated rheumatic mitral stenosis (median age was 39.1±11  (group 1 and 20 patients in the control group ( median age was 37±8,2 (group 2 were enrolled to study. The average mitral valve area of patients was 1.1±0,3 cm2. When patients were divided according to New York Heart Association (NYHA classification ; 16 patients were NYHA 2 (Grup A and 16 patients were NYHA 3 (Grup B. There were not any asymptomatic patients and no patients were NYHA 4. Left atrium diameters, left atrium volume, left atrium fractional area change and left atrium ejection fractions  of patients in these groups were calculated.   Results: The demographic characteristics of patients is shown in table 1. Left atrium ejection fraction (LAEF and left atrium fractional area change (LAFAC that were determined echocardiographycally were significantly lower in patients with mitral stenosis (32 ± 5, 44 ± 3; p<0.001- 25 ± 11, 32 ± 6; p< 0.02.  When patients were divided according to New York Heart Association (NYHA classification ; 16 patients were NYHA 2 (Grup A and 16 patients were NYHA 3 (Grup B. There were not any asymptomatic patients and no patients were NYHA 4. The clinical and echocardiographic data of patients are shown in table 2. Despite of similar mitral valve area and average mitral gradient ,systolic pulmonary artery pressure was found to be higher in symptomatic group. But there was no difference between left atrial functions of the two groups.   Discussion: In this study we have shown that left atrial functions determined echocardiographically  can decline in patients with mitral stenosis but the

  8. Independence of intrapericardial right and left ventricular performance in septic pulmonary hypertension

    International Nuclear Information System (INIS)

    Boeck, J.C.; Eichstaedt, H.; Barker, B.C.; Lewis, F.R.; Lim, A.D.; Pollycove, M.

    1990-01-01

    To study the effect of septic pulmonary hypertension on right/left ventricular intrapericardial interactions thirteen trauma patients, seven septic and six nonseptic controls, were compared. Ventricular volumes were derived from firstpass or gated equilibrium radionuclide angiocardiography, and related to body surface area. Systemic and pulmonary pressures were measured invasively. Pulmonary arterial pressure was significantly increased in the sepsis group. Although right ventricular end-diastolic volumes were higher in sepsis, left ventricular end-diastolic volumes were not decreased. In terms of intrapericardial right/left ventricular interactions these results indicate that the right and left ventricles operate independently in septic pulmonary hypertension. (orig.) [de

  9. Multiplicativity of left centralizers forcing additivity

    Directory of Open Access Journals (Sweden)

    Mohammad Sayed Tammam El-Sayiad

    2014-01-01

    Full Text Available A multiplicative left centralizer for an associative ring R is a map satisfying T(xy = T\\(xy for all x,y in R. T is not assumed to be additive. In this paper we deal with the additivity of the multiplicative left centralizers in a ring which contains an idempotent element. Specially, we study additivity for multiplicative left centralizers in prime and semiprime rings which contain an idempotent element.

  10. Anarchy, socialism and a Darwinian left.

    Science.gov (United States)

    Clarke, Ellen

    2006-03-01

    In A Darwinian left Peter Singer aims to reconcile Darwinian theory with left wing politics, using evolutionary game theory and in particular a model proposed by Robert Axelrod, which shows that cooperation can be an evolutionarily successful strategy. In this paper I will show that whilst Axelrod's model can give support to a kind of left wing politics, it is not the kind that Singer himself envisages. In fact, it is shown that there are insurmountable problems for the idea of increasing Axelrodian cooperation within a welfare state. My surprising conclusion will be that a Darwinian left worthy of the name would be anarchistic.

  11. What is Beyond Right/Left?

    DEFF Research Database (Denmark)

    Dyrberg, Torben Bech

    2009-01-01

    The article looks at New Labour's move beyond right/left in the mid/late 1990s, which is an occasion to spell out the nature of right/left and what it means for democracy. In contrast to both defenders and critics of this move I argue in the first part that right/left is not an empty label bound up...... right/left. I argue that the new hegemonic orientation is that of front/back, which designs political renewal as a response to the social changes cutting across the outdated lines of contestation of partisan politics. The democratic problem of this move lies in squeezing politics between technocratic...

  12. Induced pluripotent stem cells (iPSCs) derived from a symptomatic carrier of a S305I mutation in the microtubule-associated protein tau (MAPT)-gene causing frontotemporal dementia

    DEFF Research Database (Denmark)

    Nimsanor, Natakarn; Jørring, Ida; Rasmussen, Mikkel A.

    2016-01-01

    Frontotemporal dementia with parkinsonism linked to chromosome 17q21.2 (FTDP-17) is an autosomal-dominant neurodegenerative disorder. Mutations in the gene coding the microtubule-associated protein tau (MAPT) can cause FTDP-17 but the underlying mechanisms of the disease are still unknown. Induced...

  13. Induced pluripotent stem cells (iPSCs) derived from af pre-symptomatic carrier of a R406W mutation in microtubule-associated protein tau (MAPT) causing frontotemporal dementia

    DEFF Research Database (Denmark)

    Rasmussen, Mikkel A.; Hjermind, Lena Elisabeth; Hasholt, Lis Frydenreich

    2016-01-01

    Skin fibroblasts were obtained from a 28-year-old pre-symptomatic woman carrying a R406W mutation in microtubule-associated protein tau (MAPT), known to cause frontotemporal dementia. Induced pluripotent stem cell (iPSCs) were established by electroporation with episomal plasmids containing hOCT4...

  14. A functional MRI study of language networks in left medial temporal lobe epilepsy

    International Nuclear Information System (INIS)

    Yu Aihong; Wang Xiaoyi; Xu Guoqing; Li Yongjie; Qin Wen; Li Kuncheng; Wang, Yuping

    2011-01-01

    Purpose: The purpose of this study was to investigate the abnormality of language networks in left medial temporal lobe epilepsy (MTLE) using fMRI. Materials and methods: Eight patients with left MTLE and 15 healthy subjects were evaluated. An auditory semantic judgment (AJ) paradigm was used. The fMRI data were collected on a 3T MR system and analyzed by AFNI (analysis of functional neuroimages) to generate the activation map. Results: Behavioral data showed that the reaction time of the left MTLE patients was significantly longer than that of controls on the AJ task (t = -3.396, P < 0.05). The left MTLE patients also exhibited diffusively decreased activation in the AJ task. Right hemisphere dominance of Broca's and Wernicke's areas was demonstrated in left MTLE patients. Conclusions: Long-term activation of spikes in left MTLE patients results in language impairment, which is associated with an abnormality of the brain neural network.

  15. Left atrial systolic force in hypertensive patients with left ventricular hypertrophy: the LIFE study

    DEFF Research Database (Denmark)

    Chinali, M.; Simone, G. de; Wachtell, K.

    2008-01-01

    In hypertensive patients without prevalent cardiovascular disease, enhanced left atrial systolic force is associated with left ventricular hypertrophy and increased preload. It also predicts cardiovascular events in a population with high prevalence of obesity. Relations between left atrial...... with larger left ventricular diameter and higher left ventricular mass index (both P hypertrophy was greater (84 vs. 64%; P ..., transmitral peak E velocities and peak A velocities; and lower E/A ratio (all P hypertrophy, but normal left ventricular chamber systolic function with increased...

  16. Left atrial leiomyosarcoma as cause of heart failure

    International Nuclear Information System (INIS)

    Valdes Martin, Alexander

    2012-01-01

    The case of a 26 year-old patient with diagnosis of heart failure and presence of a mobile echogenic mass without calcification attached to the posterolateral area of the left atrium was reported. She underwent surgery and tissue examination evidenced a high-grade heart leiomyosarcoma. Postoperative course was satisfactory and her clinical state was rigorously monitored by oncology and heart specialists in the institution

  17. Left Paraduodenal Hernia: An Autopsy Case

    DEFF Research Database (Denmark)

    Omland, Silje Haukali; Hougen, Hans Petter

    2011-01-01

    We present a case of a left paraduodenal hernia diagnosed at autopsy. A left paraduodenal hernia is an internal hernia of congenital origin due to the abnormal rotation of the midgut during embryonic development. Internal hernias are a rare cause of intestinal obstruction, with the paraduodenal...

  18. Malignant phyllodes tumor of the left atrium

    Directory of Open Access Journals (Sweden)

    Anupam Bhambhani

    2014-03-01

    Full Text Available Metastatic tumors to the heart usually involve right sided chambers. We report a rare case of malignant phyllodes tumor of breast with metastatic involvement of left atrium occurring through direct invasion from mediastinal micro-metastasis and presenting as a left atrial mass causing arrhythmia.

  19. Left ventricular hypertrophy, geometric patterns and clinical ...

    African Journals Online (AJOL)

    Background: Left ventricular hypertrophy can be due to various reasons including hypertension. It constitutes an increased cardiovascular risk. Various left ventricular geometric patterns occur in hypertension and may affect the cardiovascular risk profile of hypertensive subjects. Methods: One hundred and eighty eight ...

  20. Leptogenesis with left-right domain walls

    Indian Academy of Sciences (India)

    the scale of left-right symmetry breaking. Keywords. Leptogenesis; baryogenesis; domain walls; left-right symmetry. PACS Nos 12.10.Dm; 98.80.Cq; 98.80.Ft. Explaining the observed baryon asymmetry of the Universe within the framework of gauge theories and the standard Big Bang cosmology remains an open problem.

  1. On establishing coreference in Left Dislocation constructions ...

    African Journals Online (AJOL)

    The phenomenon of left dislocation (LD) has received relatively little attention in the generative literature. In Government & Binding theory and early versions of Minimalist Syntax, the left-dislocated expression is conventionally taken to be base-generated in its sentence-initial surface position and the resumptive pronoun in ...

  2. A new clinical sign probably associated to left hemiplegia with left hemineglect syndrome: the crossed legs.

    Science.gov (United States)

    Bazan, Rodrigo; Fernandes, Thiago; Braga, Gabriel; Luvizutto, Gustavo; Resende, Luiz

    2014-06-01

    To describe a new clinical sign associated with left unilateral neglect syndrome (UNS) in patients with ischemic stroke. Head computed tomography (CT) and National Institute of Health Stroke Scale were obtained in 150 patients with ischemic stroke. Those with right cerebral vascular lesions, left hemiplegia and right leg persistently crossed over the left were submitted to specific tests for UNS. The tests were also applied to 30 patients with right cerebral vascular lesions, left hemiplegia but without crossed legs. From 9 patients with persistent tendency to cross the right leg over the left, UNS was detected in 8. One patient died before the clinical tests were applied. Of the 30 patients without the crossed legs, 20 had normal clinical tests for UNS and 10 had minimal alterations, not sufficient for the diagnosis of UNS. The right leg crossed over the left may represent a new neurological semiotic sign associated with left hemiplegia and left UNS.

  3. VARIATION IN THE OPENINGS (OSTIA OF LEFT PULMONARY VEINS INTO THE LEFT ATRIUM: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Sesi

    2015-03-01

    Full Text Available During early embryonic development, absorption of pulmonary venous network by the left primitive atrial chamber results in opening of four pulmonary veins which drain independently into its chamber. The extent of absorption and hence, the number of pulmon ary veins which open into the left atrium, may vary. Here we report a variation in the opening of the Left upper (superior pulmonary vein into the Left atrium. A total of six openings observed

  4. When right is all that's left: plasticity of right-hemisphere tracts in a young aphasic patient

    Science.gov (United States)

    Zipse, Lauryn; Norton, Andrea; Marchina, Sarah; Schlaug, Gottfried

    2012-01-01

    Using an adapted version of melodic intonation therapy (MIT), we treated an adolescent girl with a very large left-hemisphere lesion and severe nonfluent aphasia secondary to an ischemic stroke. At the time of her initial assessment 1.25 years after her stroke, she had reached a plateau in her recovery despite intense and long-term traditional speech-language therapy (approximately five times per week for more than one year). Following an intensive course of treatment with our adapted form of MIT, her performance improved on both trained and untrained phrases, as well as on speech and language tasks. These behavioral improvements were accompanied by functional MRI changes in the right frontal lobe as well as by an increased volume of white matter pathways in the right hemisphere. No increase in white matter volume was seen in her healthy twin sister, who was scanned twice over the same time period. This case study not only provides further evidence for MIT's effectiveness, but also indicates that intensive treatment can induce functional and structural changes in a right hemisphere fronto-temporal network. PMID:22524365

  5. When right is all that is left: plasticity of right-hemisphere tracts in a young aphasic patient.

    Science.gov (United States)

    Zipse, Lauryn; Norton, Andrea; Marchina, Sarah; Schlaug, Gottfried

    2012-04-01

    Using an adapted version of Melodic Intonation Therapy (MIT), we treated an adolescent girl with a very large left-hemisphere lesion and severe nonfluent aphasia secondary to an ischemic stroke. At the time of her initial assessment 15 months after her stroke, she had reached a plateau in her recovery despite intense and long-term traditional speech-language therapy (approximately five times per week for more than one year). Following an intensive course of treatment with our adapted form of MIT, her performance improved on both trained and untrained phrases, as well as on speech and language tasks. These behavioral improvements were accompanied by functional MRI changes in the right frontal lobe as well as by an increased volume of white matter pathways in the right hemisphere. No increase in white matter volume was seen in her healthy twin sister, who was scanned twice over the same time period. This case study not only provides further evidence for MIT's effectiveness, but also indicates that intensive treatment can induce functional and structural changes in a right-hemisphere fronto-temporal network. © 2012 New York Academy of Sciences.

  6. Famous People Knowledge and the Right and Left Temporal Lobes

    Science.gov (United States)

    Snowden, Julie S.; Thompson, Jennifer C.; Neary, David

    2012-01-01

    It is generally accepted that the anterior temporal lobes support knowledge of famous people. The specific roles of the right and left temporal lobe remain a subject of debate, with some studies suggesting differential roles based on modality (visual versus verbal information) and others category (person knowledge versus general semantics). The present study re-examined performance of semantic dementia patients with predominantly right and predominantly left temporal lobe atrophy on famous face, famous name and general semantic tasks, with the specific aim of testing the hypothesis that the right temporal lobe has a privileged role for person knowledge and the left temporal lobe for general semantic knowledge. Comparisons of performance rankings across tasks showed no evidence to support this hypothesis. By contrast, there was robust evidence from naming, identification and familiarity measures for modality effects: right-sided atrophy being associated with relatively greater impairment for faces and visual tasks and left-sided atrophy for names and verbal tasks. A double dissociation in test scores in two patients reinforced these findings. The data present a challenge for the influential ‘semantic hub’ model, which views the anterior temporal lobes as an area of convergence in which semantic information is represented in amodal form. PMID:22207421

  7. Frontotemporal Dementias: Diagnosis

    Science.gov (United States)

    ... Behavioral Variant FTD (bvFTD) Corticobasal Syndrome FTD with Motor Neuron Disease (FTD/MND) Pick’s Disease Nonfluent / Agrammatic variant Primary ... This test is important in the diagnosis of motor neuron diseases such as ALS (Lou Gehrig’s disease). Lumbar puncture ( ...

  8. Frontotemporal Dementia (Pick's Disease)

    Science.gov (United States)

    ... been shown to slow the progression of FTD. Behavior modification may help control unacceptable or dangerous behaviors. Aggressive, ... been shown to slow the progression of FTD. Behavior modification may help control unacceptable or dangerous behaviors. Aggressive, ...

  9. Regional cerebral blood flow single photon emission computed tomography for detection of Frontotemporal dementia in people with suspected dementia.

    Science.gov (United States)

    Archer, Hilary A; Smailagic, Nadja; John, Christeena; Holmes, Robin B; Takwoingi, Yemisi; Coulthard, Elizabeth J; Cullum, Sarah

    2015-06-23

    In the UK, dementia affects 5% of the population aged over 65 years and 25% of those over 85 years. Frontotemporal dementia (FTD) represents one subtype and is thought to account for up to 16% of all degenerative dementias. Although the core of the diagnostic process in dementia rests firmly on clinical and cognitive assessments, a wide range of investigations are available to aid diagnosis.Regional cerebral blood flow (rCBF) single-photon emission computed tomography (SPECT) is an established clinical tool that uses an intravenously injected radiolabelled tracer to map blood flow in the brain. In FTD the characteristic pattern seen is hypoperfusion of the frontal and anterior temporal lobes. This pattern of blood flow is different to patterns seen in other subtypes of dementia and so can be used to differentiate FTD.It has been proposed that a diagnosis of FTD, (particularly early stage), should be made not only on the basis of clinical criteria but using a combination of other diagnostic findings, including rCBF SPECT. However, more extensive testing comes at a financial cost, and with a potential risk to patient safety and comfort. To determine the diagnostic accuracy of rCBF SPECT for diagnosing FTD in populations with suspected dementia in secondary/tertiary healthcare settings and in the differential diagnosis of FTD from other dementia subtypes. Our search strategy used two concepts: (a) the index test and (b) the condition of interest. We searched citation databases, including MEDLINE (Ovid SP), EMBASE (Ovid SP), BIOSIS (Ovid SP), Web of Science Core Collection (ISI Web of Science), PsycINFO (Ovid SP), CINAHL (EBSCOhost) and LILACS (Bireme), using structured search strategies appropriate for each database. In addition we searched specialised sources of diagnostic test accuracy studies and reviews including: MEDION (Universities of Maastricht and Leuven), DARE (Database of Abstracts of Reviews of Effects) and HTA (Health Technology Assessment) database

  10. Social aspects of left-handedness

    Directory of Open Access Journals (Sweden)

    Belojević Goran

    2010-01-01

    Full Text Available Throughout human history left-handedness has been considered as sinful. It has been associated with the devil, weakness, female gender, unhealthiness, evil, something that has to be turned to a “good” - right side by force. Left-handedness is being more and more acceptable at rational level, but in everyday life it is still considered to be unusual if someone writes with the left hand. Lessening of the number of lefthanders is associated with ageing. There are about 13% lefthanders among people in twenties and less than 1% lefthanders among those in eighties. This finding may be explaned with more pronounced socio-cultural pressure on left-handed people in the past, compared to nowadays. On the other hand, this may also support the hypothesis about a reduced life span of lefthanded people. With cross-exercising of left-handedness, certain typical characteristics and behavioral patterns appear in these people. This was a sort of provoked behavior and an attack on the integrity of an emotional attitude toward oneself. Stuttering may also appear as a consequence of unsuccessful cross-exercising of left-handedness. The hypothesis about left-handedness as an advantage is supported with the reports about relatively more lefthanders in some specific groups such as: mathematicians, sculptors, architects, painters, musicians, actors, tennis players, as well as famous army commanders and rulers.

  11. Left ventricular performance during triggered left ventricular pacing in patients with cardiac resynchronization therapy and left bundle branch block

    DEFF Research Database (Denmark)

    Witt, Christoffer Tobias; Kronborg, Mads Brix; Nohr, Ellen Aagaard

    2016-01-01

    PURPOSE: To assess the acute effect of triggered left ventricular pacing (tLVp) on left ventricular performance and contraction pattern in patients with heart failure, left bundle branch block (LBBB), and cardiac resynchronization therapy (CRT). METHODS: Twenty-three patients with pre-implant QRS...... complex >150 ms, QRS complex narrowing under CRT, and sinus rhythm were included ≥3 months after CRT implantation. Echocardiographic assessment of left ventricular ejection fraction (LVEF), global peak systolic longitudinal strain (GLS), and contraction pattern by 2D strain was performed during intrinsic......V pacing. CONCLUSIONS: The acute effect of tLVp on LV systolic function and contraction pattern is significantly lower than the effect of BiV pacing and not different from intrinsic conduction in patients with LBBB and CRT....

  12. hnRNPA2B1 and hnRNPA1 mutations are rare in patients with "multisystem proteinopathy" and frontotemporal lobar degeneration phenotypes.

    Science.gov (United States)

    Le Ber, Isabelle; Van Bortel, Inge; Nicolas, Gael; Bouya-Ahmed, Kawtar; Camuzat, Agnès; Wallon, David; De Septenville, Anne; Latouche, Morwena; Lattante, Serena; Kabashi, Edor; Jornea, Ludmila; Hannequin, Didier; Brice, Alexis

    2014-04-01

    hnRNPA2B1 and hnRNPA1 mutations have been recently identified by exome sequencing in three families presenting with multisystem proteinopathy (MSP), a rare complex phenotype associating frontotemporal lobar degeneration (FTLD), Paget disease of bone (PDB), inclusion body myopathy (IBM), and amyotrophic lateral sclerosis (ALS). No study has evaluated the exact frequency of these genes in cohorts of MSP or FTD patients so far. We sequenced both genes in 17 patients with MSP phenotypes, and in 60 patients with FTLD and FTLD-ALS to test whether mutations could be implicated in the pathogenesis of these disorders. No disease-causing mutation was identified. We conclude that hnRNPA2B1 and hnRNPA1 mutations are rare in MSP and FTLD spectrum of diseases, although further investigations in larger populations are needed. Copyright © 2014 Elsevier Inc. All rights reserved.

  13. Neurodegenerative disease phenotypes in carriers of MAPT p.A152T, a risk factor for frontotemporal dementia spectrum disorders and Alzheimer's disease

    Science.gov (United States)

    Lee, Suzee E.; Tartaglia, Maria Carmela; Yener, Görsev; Genç, Sermin; Seeley, William W.; Sanchez-Juan, Pascual; Moreno, Fermin; Mendez, Mario F.; Klein, Eric; Rademakers, Rosa; de Munain, Adolfo López; Combarros, Onofre; Kramer, Joel H.; Kenet, Robert O.; Boxer, Adam L.; Geschwind, Michael D.; Gorno-Tempini, Maria-Luisa; Karydas, Anna M.; Rabinovici, Gil D.; Coppola, Giovanni; Geschwind, Daniel; Miller, Bruce L.

    2013-01-01

    Recently, Coppola and colleagues demonstrated that a rare MAPT sequence variant, c.454G>A (p.A152T), significantly increases the risk of frontotemporal dementia (FTD) spectrum disorders and Alzheimer's disease (AD) in a screen of 15,369 subjects1. We describe clinical features of 9 patients with neurodegenerative disease (4 women) harboring p.A152T, aged 51 to 79 years at symptom onset. Seven developed FTD spectrum clinical syndromes, including progressive supranuclear palsy syndrome (PSP, n=2), behavioral variant FTD (bvFTD, n=1), nonfluent variant primary progressive aphasia (nfvPPA, n=2), and corticobasal syndrome (CBS, n=2); two patients were diagnosed with clinical AD. Thus, MAPT p.A152T is associated with a variety of FTD spectrum clinical presentations, although patients with clinical AD are also identified. These data warrant larger studies with clinicopathological correlation to elucidate the influence of this genetic variant on neurodegenerative disease. PMID:23518664

  14. Genetic analysis of matrin 3 gene in French amyotrophic lateral sclerosis patients and frontotemporal lobar degeneration with amyotrophic lateral sclerosis patients.

    Science.gov (United States)

    Millecamps, Stéphanie; De Septenville, Anne; Teyssou, Elisa; Daniau, Mailys; Camuzat, Agnès; Albert, Mélanie; LeGuern, Eric; Galimberti, Daniela; Brice, Alexis; Marie, Yannick; Le Ber, Isabelle

    2014-12-01

    Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are adult-onset neurodegenerative diseases with overlapping clinical characteristics. They share common genetic causes and pathologic hallmarks such as TDP-43 neuronal accumulations. Recently, exome analysis identified mutations in matrin 3 (MATR3) gene in patients with familial ALS, suggesting a role for this gene in the pathogenesis of the disease. MATR3 is a nuclear matrix protein with DNA and RNA binding domains that interacts with TDP-43. To confirm the contribution of MATR3 to ALS, we studied a French cohort of 153 familial ALS or ALS/FTLD patients, without finding any variant. We conclude that mutations in MATR3 are rare in French familial ALS and ALS with FTLD patients. Copyright © 2014 Elsevier Inc. All rights reserved.

  15. [Vectorcardiographic manifestations of left intraventricular conduction disorders].

    Science.gov (United States)

    de Micheli, A; Medrano, G A

    1979-01-01

    Both, the vectorcardiographic changes produced by the various degrees of left bundle branch block and these observed with the different types of left distal block, are described. When a "wave jumping" phenomenon exists, the vectorcardiographic changes are more characteristic in the horizontal plane than in the frontal plane and can be interpreted satisfactorily in basis of the ventricular activation sequence. The normal counterclockwise rotation of the horizontal vectorcardiogram persists in the presence of left bundle branch block of slight and moderate degrees, since the electromotive forces of the free left ventricular wall are still predominant. In the majority of intermediate degree blocks, the middle portion of the RH loop develops with a clockwise rotation and general aspect with a clockwise rotation and the general aspect of the ventricular loop resembles an eight figure. This is due to the electromotive forces originated by the delayed depolarization of the left septal mass that starts to predominate. With advanced degrees of block, the largest portion of the RH loop shows a clockwise rotation, as well as marked notchings and slurrings. The initial anterior portion of the horizontal vectorcardiogram does not disappear, but is situated to the left of the anterior-posterior axis with a counterclockwise rotation (first right septal vector). Otherwise, the direct electrical sign of left distal block emphasized: evidence of delayed activation in a limited zone of the homolateral ventricle. This local delay gives rise to an asynchronism of the activation phenomenon between the upper and lower regions of the ventricle. The diagnosis of left bifascicular block is based essentially on the evidence of unequal delay of the activation sequence in the basal regions and in the inferior ones of the homolateral ventricle and also on the frequent persistence of the first left septal vector.

  16. Ventricular Energetics in Pediatric Left Ventricular Assist Device Patients: A Retrospective Clinical Study.

    Science.gov (United States)

    Di Molfetta, Arianna; Ferrari, Gianfranco; Iacobelli, Roberta; Filippelli, Sergio; Di Chiara, Luca; Guccione, Paolo; Amodeo, Antonio

    The aim of this study is to estimate the trend of right and left energetic parameters in left ventricular assist device (LVAD) pediatric patients. Echocardiographic data were retrospectively collected at the baseline, in the acute phase after and at the monthly follow-ups till the LVAD explantation to estimate left and right ventricular energetic parameters. A significant relationship between the left and right ventricular energetic parameter trends was found along all the study period. Left ventricular end-systolic pressure-volume relationship improved till the follow-up of 2 months and then progressively decreases. Left arteroventricular coupling decreases after the LVAD, and right arteroventricular coupling decreases at the short-term follow-up. Left ventricular external work, potential energy, and pressure-volume area decrease at the short-term follow-up and then increase progressively. Right ventricular external work, potential energy, and pressure-volume area increase after the LVAD implantation. Left (right) cardiac mechanical efficiency is improved (worsened) by the LVAD. Energetic variables show that the LVAD benefits could decrease over time. A continuous and patient tailored LVAD setting could contribute to prolong LVAD benefits. The introduction of energetic parameters could lead to a more complete evaluation of LVAD patients' outcome which is a multiparametric process.

  17. Are there excitability changes in the hand motor cortex during speech in left-handed subjects?

    Science.gov (United States)

    Tokimura, Hiroshi; Tokimura, Yoshika; Arita, Kazunori

    2012-01-01

    Hemispheric dominance was investigated in left-handed subjects using single transcranial magnetic stimulation to assess the possible effect of forced change in the dominant hand. Single transcranial magnetic stimuli were delivered randomly over the hand area of the left or right motor cortex of 8 Japanese self-declared left-handed adult volunteers. Electromyographic responses were recorded in the relaxed first dorsal interosseous muscle while the subjects read aloud. Laterality quotient calculated by the Edinburgh Inventory ranged from -100 to -5.26 and laterality index calculated from motor evoked potentials ranged from -86.2 to 38.8. There was no significant correlation between laterality quotient and laterality index. Mean data values across all 8 subjects indicated significant increases only in the left hand. Our ratio analysis of facilitation of the hand motor potentials showed that 2 each of the 8 self-declared left-handers were right- and left-hand dominant and the other 4 were bilateral-hand dominant. Speech dominancy was localized primarily in the right cerebral hemisphere in left-handed subjects, but some individuals exhibited bilateral or left dominance, possibly attributable to the forced change of hand preference for writing in childhood. Our findings suggest changes in the connections between the speech and hand motor areas.

  18. The effects of left and right monocular viewing on hemispheric activation.

    Science.gov (United States)

    Wang, Chao; Burtis, D Brandon; Ding, Mingzhou; Mo, Jue; Williamson, John B; Heilman, Kenneth M

    2018-03-01

    Prior research has revealed that whereas activation of the left hemisphere primarily increases the activity of the parasympathetic division of the autonomic nervous system, right-hemisphere activation increases the activity of the sympathetic division. In addition, each hemisphere primarily receives retinocollicular projections from the contralateral eye. A prior study reported that pupillary dilation was greater with left- than with right-eye monocular viewing. The goal of this study was to test the alternative hypotheses that this asymmetric pupil dilation with left-eye viewing was induced by activation of the right-hemispheric-mediated sympathetic activity, versus a reduction of left-hemisphere-mediated parasympathetic activity. Thus, this study was designed to learn whether there are changes in hemispheric activation, as measured by alteration of spontaneous alpha activity, during right versus left monocular viewing. High-density electroencephalography (EEG) was recorded from healthy participants viewing a crosshair with their right, left, or both eyes. There was a significantly less alpha power over the right hemisphere's parietal-occipital area with left and binocular viewing than with right-eye monocular viewing. The greater relative reduction of right-hemisphere alpha activity during left than during right monocular viewing provides further evidence that left-eye viewing induces greater increase in right-hemisphere activation than does right-eye viewing.

  19. Impairment of language is related to left parieto-temporal glucose metabolism in aphasic stroke patients.

    Science.gov (United States)

    Karbe, H; Szelies, B; Herholz, K; Heiss, W D

    1990-02-01

    Twenty-six aphasic patients who had an ischaemic infarct in the territory of the left middle cerebral artery (MCA) were investigated. Cranial computed tomography (CT) showed various lesion sites: infarcts restricted to cortical structures in 12 patients, combined cortical and subcortical infarcts in 7 and isolated subcortical infarcts sparing the left cortex in another 7 cases. 18F-2-fluoro-2-deoxyglucose positron emission tomography revealed remote hypometabolism of the left convexity cortex and of the left basal ganglia, which was extended further than the morphological infarct zone in all cases. Types and degrees of aphasia were classified using the Aachener Aphasie Test (AAT): 10 patients had global aphasia, 2 Broca's, 5 Wernicke's, and 5 amnesic aphasia. Four patients suffered from minimal or residual aphasic symptoms. The AAT results were compared with the regional cerebral metabolic rates of glucose of the left hemisphere. Irrespective of the infarct location all five AAT subtests (Token test, repetition, written language, confrontation naming, auditory and reading comprehension) were closely correlated among each other and with left parieto-temporal metabolic rates, whereas left frontal and left basal ganglia metabolism showed no significant correlation. The close relation between left temporo-parietal functional activity and all five AAT subtests suggests that the different aspects of aphasia tested by AAT can be related to a common disorder of language processing in those areas.

  20. Body-specific representations of action verbs: neural evidence from right- and left-handers.

    Science.gov (United States)

    Willems, Roel M; Hagoort, Peter; Casasanto, Daniel

    2010-01-01

    According to theories of embodied cognition, understanding a verb like throw involves unconsciously simulating the action of throwing, using areas of the brain that support motor planning. If understanding action words involves mentally simulating one's own actions, then the neurocognitive representation of word meanings should differ for people with different kinds of bodies, who perform actions in systematically different ways. In a test of the body-specificity hypothesis, we used functional magnetic resonance imaging to compare premotor activity correlated with action verb understanding in right- and left-handers. Right-handers preferentially activated the left premotor cortex during lexical decisions on manual-action verbs (compared with nonmanual-action verbs), whereas left-handers preferentially activated right premotor areas. This finding helps refine theories of embodied semantics, suggesting that implicit mental simulation during language processing is body specific: Right- and left-handers, who perform actions differently, use correspondingly different areas of the brain for representing action verb meanings.

  1. The effects of behavioral and psychological symptoms on caregiver burden in frontotemporal dementia, Lewy body dementia, and Alzheimer's disease: clinical experience in China.

    Science.gov (United States)

    Liu, Shuling; Jin, Yi; Shi, Zhihong; Huo, Ya Ruth; Guan, Yalin; Liu, Mengyuan; Liu, Shuai; Ji, Yong

    2017-06-01

    Caregivers of individuals with neurodegenerative diseases, including frontotemporal dementia (FTD), Lewy body dementia (DLB), and Alzheimer's disease (AD), experience high levels of psychological and physical stress, likely due to behavioral and psychological symptoms of dementia (BPSD). This study is the first to simultaneously evaluate the effects of BPSD on caregiver burden in these three types of dementia. A total of 214 dementia patients, including probable FTD (n = 82), DLB (n = 22), and AD (n = 110), as well as their primary caregivers, were assessed using psychological inventories and cognitive evaluation. The FTD group was further divided into the three established clinical variants: behavioral variant frontotemporal dementia (bvFTD, n = 51), non-fluent variant primary progressive aphasia (nfvPPA, n = 15), and semantic variant primary progressive aphasia (svPPA, n = 16). Cognitive impairment and neuropsychiatric symptoms were assessed using the Mini Mental State Examination, Montreal Cognitive Assessment, Clock Drawing Test, and Neuropsychiatric Inventory (NPI), respectively. Caregiver burden was assessed using the Zarit Burden Inventory (ZBI). FTD patients had higher NPI and ZBI scores than DLB and AD patients, whose scores were similar. Logistic regression analysis revealed that the factors influencing caregiver burden for each group were: FTD: total NPI scores, agitation, and aberrant motor behavior; bvFTD: total NPI scores; DLB: total NPI scores; and AD: total NPI scores, onset age, apathy, and ADL. Caregivers of bvFTD patients had the highest levels of burden, which were significantly greater than for caregivers of nfvPPA, svPPA, DLB, and AD patients. BPSD was highly correlated with emotional burden in caregivers of FTD, DLB, and AD patients. The highest burden was observed in bvFTD caregivers.

  2. Pedigree with frontotemporal lobar degeneration – motor neuron disease and Tar DNA binding protein-43 positive neuropathology: genetic linkage to chromosome 9

    Directory of Open Access Journals (Sweden)

    Loy Clement T

    2008-08-01

    Full Text Available Abstract Background Frontotemporal lobar degeneration (FTLD represents a clinically, pathologically and genetically heterogenous neurodegenerative disorder, often complicated by neurological signs such as motor neuron-related limb weakness, spasticity and paralysis, parkinsonism and gait disturbances. Linkage to chromosome 9p had been reported for pedigrees with the neurodegenerative disorder, frontotemporal lobar degeneration (FTLD and motor neuron disease (MND. The objective in this study is to identify the genetic locus in a multi-generational Australian family with FTLD-MND. Methods Clinical review and standard neuropathological analysis of brain sections from affected pedigree members. Genome-wide scan using microsatellite markers and single nucleotide polymorphism fine mapping. Examination of candidate genes by direct DNA sequencing. Results Neuropathological examination revealed cytoplasmic deposition of the TDP-43 protein in three affected individuals. Moreover, we identify a family member with clinical Alzheimer's disease, and FTLD-Ubiquitin neuropathology. Genetic linkage and haplotype analyses, defined a critical region between markers D9S169 and D9S1845 on chromosome 9p21. Screening of all candidate genes within this region did not reveal any novel genetic alterations that co-segregate with disease haplotype, suggesting that one individual carrying a meiotic recombination may represent a phenocopy. Re-analysis of linkage data using the new affection status revealed a maximal two-point LOD score of 3.24 and a multipoint LOD score of 3.41 at marker D9S1817. This provides the highest reported LOD scores from a single FTLD-MND pedigree. Conclusion Our reported increase in the minimal disease region should inform other researchers that the chromosome 9 locus may be more telomeric than predicted by published recombination boundaries. Moreover, the existence of a family member with clinical Alzheimer's disease, and who shares the disease

  3. Unclassified cases of behavioral variant of major frontotemporal neurocognitive disorder in the Diagnostic and Statistical Manual of Mental Disorders, 5th edition.

    Science.gov (United States)

    Fukuda, Koji; Hattori, Hideyuki

    2014-04-01

    In the Diagnostic and Statistical Manual of Mental Disorders, 5th edition (DSM-5), the behavioral variant of major frontotemporal neurocognitive disorder (bvFT-NCD) is subclassified into "probable bvFT-NCD" or "possible bvFT-NCD." When genetic evidence is unavailable, cases without clinical neuroimaging are subclassified into "possible bvFT-NCD," whereas cases whose clinical images show the typical characteristics are subclassified into "probable bvFT-NCD." Thus, the cases that meet the diagnostic criteria of bvFT-NCD based on their symptoms, but lack the neuroimaging characteristics, fall between the two categories of probable and possible bvFT-NCD. These cases herein are defined as "unclassified bvFT-NCD," and the present study aims at considering an appropriate diagnostic approach to such cases, that is, whether unclassified bvFT-NCD should be included in bvFT-NCD as a third subcategory, or whether it should be classified into diseases other than bvFT-NCD. All patients who presented at the Department of Psychiatry of the National Center for Geriatrics and Gerontology with suspicion of the behavioral variant of frontotemporal dementia between 1 May 2011 and 30 April 2013 were retrospectively rediagnosed based on the DSM-5 criteria. A total of 16 cases met the criteria of bvFT-NCD, and among them, eight cases corresponded to unclassified bvFT-NCD. From a cross-sectional and clinical perspective, all eight cases of unclassified bvFT-NCD fulfilled the symptomatic criteria for bvFT-NCD, although the possibilities of Alzheimer's disease and other mental disorders could not be ruled out completely. To establish clinical diagnostic criteria for unclassified bvFT-NCD, accumulation of cases and evidence will be required along with longitudinal observation using various diagnostic technologies and post-mortem examination. © 2014 Japan Geriatrics Society.

  4. Anatomic relationship between left coronary artery and left atrium in patients undergoing atrial fibrillation ablation.

    Science.gov (United States)

    Anselmino, Matteo; Torri, Federica; Ferraris, Federico; Calò, Leonardo; Castagno, Davide; Gili, Sebastiano; Rovera, Chiara; Giustetto, Carla; Gaita, Fiorenzo

    2017-07-01

    Atrial fibrillation transcatheter ablation (TCA) is, within available atrial fibrillation rhythm control strategies, one of the most effective. To potentially improve ablation outcome in case of recurrent atrial fibrillation after a first procedure or in presence of structural myocardial disease, isolation of the pulmonary veins may be associated with extensive lesions within the left atrium. To avoid rare, but potentially life-threatening, complications, thorough knowledge and assessment of left atrium anatomy and its relation to structures in close proximity are, therefore, mandatory. Aim of the present study is to describe, by cardiac computed tomography, the anatomic relationship between aortic root, left coronary artery and left atrium in patients undergoing atrial fibrillation TCA. The cardiac computed tomography scan of 21 patients affected by atrial fibrillation was elaborated to segment left atrium, aortic root and left coronary artery from the surrounding structures and the following distances measured: left atrium and aortic root; left atrium roof and aortic root; left main coronary artery and left atrium; circumflex artery and left atrium appendage; and circumflex artery and mitral valve annulus. Above all, the median distance between left atrium and aortic root (1.9, 1.5-2.1 mm), and between circumflex artery and left atrium appendage ostium (3.0, 2.1-3.4 mm) were minimal (≤3 mm). None of measured distances significantly varied between patients presenting paroxysmal versus persistent atrial fibrillation. The anatomic relationship between left atrium and coronary arteries is extremely relevant when performing atrial fibrillation TCA by extensive lesions. Therefore, at least in the latter case, preablation imaging should be recommended to avoid rare, but potentially life-threatening, complications with the aim of an as well tolerated as possible procedure.

  5. The assessment of left atrial function in hypertrophic cardiomyopathy using an ultrafast computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Karikomi, Masahito (Chiba Univ. (Japan). School of Medicine)

    1994-06-01

    Ultrafast computed tomography was performed in 22 patients with hypertrophic cardiomyopathy (HCM) and 8 normal subjects to evaluate left atrial function. The area-time curve of the left atrium was obtained from the long axial view and analyzed. The diminishing fraction of the left atrial area from passive atrial emptying to atrial diastasis in HCM was significantly less than that in normal subjects (17.4[+-]6.3% vs 23.0[+-]6.8%, p<0.05). The maximum area, filling fraction, time to peak diminishing rate, peak diminishing rate, time to 50% of peak diminishing rate, and diminishing fraction at the time of peak diminishing rate in HCM did not differ significantly from those in normal subjects. No indices differed between symptomatic patients with HCM and asymptomatic patients with HCM. In conclusion, the contraction of the left atrium is increased and a compensatory mechanism is at work in response to the impairment of left ventricular early diastolic filling, which does not affect the conduit function of the left atrium. It is suggested that the left atrial function in HCM may well be altered before symptom is present. (author).

  6. Beware the left-sided gallbladder

    African Journals Online (AJOL)

    %, and is defined as a gallbladder located to the left of the ligamentum teres and the falciform ligament.1 LSGB was first described by Hochstetter in 1886, and its identification is important because of the numerous and potentially hazardous.

  7. Introduction to left-right symmetric models

    International Nuclear Information System (INIS)

    Grimus, W.

    1993-01-01

    We motivate left-right symmetric models by the possibility of spontaneous parity breaking. Then we describe the multiplets and the Lagrangian of such models. Finally we discuss lower bounds on the right-handed scale. (author)

  8. Electrocardiographic features suggestive of a left. ventricular ...

    African Journals Online (AJOL)

    Abstract. Electrocardiographic features suggestive of a transmural anterior myocardial infarction with resultant left ventricular aneurysm formation were found in a 22-year-old man who had sustained a ballistic missile injury to his chest.

  9. No Child Left Inside Week: Pilot Program

    OpenAIRE

    Clark, Jamie C.

    2013-01-01

    This program evaluation assessed the feasibility and effectiveness of a free No Child Left Inside (NCLI) week-long outdoor program to coincide with the Utah state-designated No Child Left Inside Week. The pilot program was implemented at the community level in Cache Valley, Utah, in 2012. Families attended eleven activities throughout the week that included hands-on experience and participation. A community BioBlitz was also planned as a conclusion to the week. Survey results demonstrate incr...

  10. Drilling simulated temporal bones with left-handed tools: a left-hander's right?

    Science.gov (United States)

    Torgerson, Cory S; Brydges, Ryan; Chen, Joseph M; Dubrowski, Adam

    2007-11-01

    Left-handed trainees can be at a disadvantage in the surgical environment because of a right-handed bias. The effectiveness of teaching left-handed trainees to use an otologic drill designed for their dominant hand versus the conventional right-handed drill was examined. Novice medical students were recruited from the university community. Twenty-four subjects were left-handed, and 12 were right-handed. Eight left-handed surgeons also participated. A randomized controlled trial was conducted to compare the performance of left-handed trainees using novel left-handed drills to that of left-handed trainees using right-handed tools and to that of right-handed trainees using right-handed tools. The evaluation consisted of 3 phases: pretest, skill acquisition, and 2 post-tests. The measurement tools included expert assessment of performance, and subjective and objective final product analyses. An initial construct validity phase was conducted in which validity of the assessment tools was ensured. Both the left-handers using left-handed tools and the right-handers using right-handed tools significantly outperformed the left-handers using right-handed tools at pretest, immediate posttest, and delayed posttest. All participants improved their performance as a function of practice. The left-handed trainees learned bone drilling better with tools designed for the left hand. These tools may be incorporated into residency training programs for the development of surgical technical skills. Future studies should assess skill transfer between the left-handed and right-handed drills.

  11. Low-frequency transcranial magnetic stimulation over left dorsal premotor cortex improves the dynamic control of visuospatially cued actions

    DEFF Research Database (Denmark)

    Ward, Nick S; Bestmann, Sven; Hartwigsen, Gesa

    2010-01-01

    Left rostral dorsal premotor cortex (rPMd) and supramarginal gyrus (SMG) have been implicated in the dynamic control of actions. In 12 right-handed healthy individuals, we applied 30 min of low-frequency (1 Hz) repetitive transcranial magnetic stimulation (rTMS) over left rPMd to investigate...... the involvement of left rPMd and SMG in the rapid adjustment of actions guided by visuospatial cues. After rTMS, subjects underwent functional magnetic resonance imaging while making spatially congruent button presses with the right or left index finger in response to a left- or right-sided target. Subjects were...... responses in invalidly cued trials. After real rTMS, task-related activity of the stimulated left rPMd showed increased task-related coupling with activity in ipsilateral SMG and the adjacent anterior intraparietal area (AIP). Individuals who showed a stronger increase in left-hemispheric premotor...

  12. Transmural distribution of myocardial infarction: difference between the right and left ventricles in a canine model

    International Nuclear Information System (INIS)

    Ohzono, K.; Koyanagi, S.; Urabe, Y.; Harasawa, Y.; Tomoike, H.; Nakamura, M.

    1986-01-01

    The evolution of myocardial infarction 24 hours after ligating both the right coronary artery and the obtuse marginal branch of the left circumflex coronary artery was examined in 33 anesthetized dogs. Postmortem coronary angiography and a tracer microsphere technique were used to determine risk areas and their collateral blood flows, respectively. The mean weight of the risk areas was 11.3 +/- 0.5 g (mean +/- SEM) in the right ventricle and 10.5 +/- 0.9 g in the left ventricle (NS). The weight of infarcted tissue was 5.7 +/- 0.7 g in the right ventricle and 5.2 +/- 0.9 g in the left ventricle (NS). In both ventricles, infarct weight was linearly related to risk area size, and the percent of risk area necrosis was inversely correlated with the extent of collateral flow at 24 hours of coronary ligation, defined as the mean myocardial blood flow inside the central risk area. Ratios of infarct to risk area between the subendocardial and subepicardial layers were 0.76 +/- 0.06 and 0.28 +/- 0.05 in the right and left ventricles, respectively (p less than 0.01, between ventricles, n = 31), which coincided well with subendocardial-to-subepicardial-flow ratios at 24 hours, ie, 0.86 +/- 0.04 in the right ventricle and 0.32 +/- 0.06 in the left ventricle (p less than 0.01). The regional distribution of myocardial infarction correlated well with flow distribution inside the risk area; the slope of these relations was similar between the subendocardium and subepicardium in the right ventricle, whereas in the left ventricle it was larger in the subendocardium than in the subepicardium. Thus, in the dog, the inherent change in the regional distribution of coronary collateral blood flow is an important modifier in the evolution of myocardial infarction, especially in the left ventricle

  13. Left Vocal Cord Paralysis Detected by PET/CT in a Case of Lung Cancer

    Directory of Open Access Journals (Sweden)

    Ali Ozan Oner

    2015-01-01

    Full Text Available We report a patient with lung cancer. The first PET/CT imaging revealed hypermetabolic mass in the left aortopulmonary region and hypermetabolic nodule in the anterior segment of the upper lobe of the left lung. After completing chemotherapy and radiotherapy against the primary mass in the left lung, the patient underwent a second PET/CT examination for evaluation of treatment response. This test demonstrated, compared with the first PET/CT, an increase in the size and metabolic activity of the primary mass in the left lung in addition to multiple, pathologic-sized, hypermetabolic metastatic lymph nodes as well as multiple metastatic sclerotic areas in bones. These findings were interpreted as progressive disease. In addition, an asymmetrical FDG uptake was noticed at the level of right vocal cord. During follow-up, a laryngoscopy was performed, which demonstrated left vocal cord paralysis with no apparent mass. Thus, we attributed the paralytic appearance of the left vocal cord to infiltration of the left recurrent laryngeal nerve by the primary mass located in the apical region of the left lung. In conclusion, the knowledge of this pitfall is important to avoid false-positive PET results.

  14. Multiple cavities in myocardium of left ventricle after irradiation therapy for breast cancer. A case report

    Energy Technology Data Exchange (ETDEWEB)

    Miyake, Shinobu; Kato, Hiroshi; Koizumi, Katsumi [Nishi-Kobe Medical Center (Japan)] (and others)

    1999-09-01

    A 68-year-old woman was admitted to our hospital with congestive heart failure. She had been diagnosed with hypertrophic cardiomyopathy 12 years ago in another hospital. She had received irradiation therapy for left breast cancer 33 years ago after resection of her left breast. Echocardiography revealed left ventricular hypertrophy and wall motion hypokinesis, and multiple cavities in the myocardium of the left ventricle, interventricular septum, and anterior wall. Some cavities were observed to connect to the left ventricular cavity and Doppler echocardiography showed slow velocity flows in them different from that of the coronary artery. The pathologic diagnosis was severe sclerosis of the left coronary artery, especially the left descending artery and its branch, which was the area with irradiation. Histopathology revealed sclerotic changes of the coronary artery causing acute and chronic myocardial infarction, and incomplete regeneration and hypertrophy of cardiac cells. There was no sign of hypertrophic cardiomyopathy. Myocardial degeneration and deciduation were present next to the cavities connected to left ventricle-like fistulas. (author)

  15. Involvement of the Left Supramarginal Gyrus in Manipulation Judgment Tasks: Contributions to Theories of Tool Use.

    Science.gov (United States)

    Lesourd, Mathieu; Osiurak, François; Navarro, Jordan; Reynaud, Emanuelle

    2017-09-01

    Two theories of tool use, namely the gesture engram and the technical reasoning theories, make distinct predictions about the involvement of the left inferior parietal lobe (IPL) in manipulation judgement tasks. The objective here is to test these alternative predictions based on previous studies on manipulation judgment tasks using transcranial magnetic stimulations (TMS) targeting the left supramarginal gyrus (SMG). We review recent TMS studies on manipulation judgement tasks and confront these data with predictions made by both tool use theories. The left SMG is a highly intertwined region, organized following several functionally distinct areas and TMS may have disrupted a cortical network involved in the ability to use tools rather than only one functional area supporting manipulation knowledge. Moreover, manipulation judgement tasks may be impaired following virtual lesions outside the IPL. These data are more in line with the technical reasoning hypothesis, which assumes that the left IPL does not store manipulation knowledge per se. (JINS, 2017, 23, 685-691).

  16. Simultaneous occurrence of a large asymptomatic prolapsing left atrial myxoma with a cutaneous squamous cell carcinoma.

    Science.gov (United States)

    Iltumur, Kenan; Demir, Tolga; Ariturk, Zuhal; Toprak, Nizamettin; Oto, Oztekin

    2015-02-27

    Synchronous myxoma of the heart and other malignancies are extremely rare. We report a case of a 64-year-old man who had a large left atrial myxoma that obstructed the mitral valve, as well as an unrelated, coexistent cutaneous squamous cell carcinoma in the sacral area. During the preoperative evaluation for non-cardiac surgery, the tumor was diagnosed coincidentally by echocardiographic examination. Echocardiography findings were consistent with a large left atrial myxoma originating from the posterior wall and prolapsing into the left ventricular cavity through the mitral valve, causing mitral stenosis. The mass was successfully completely excised. Histologic examination of the mass confirmed the diagnosis of cardiac myxoma. We report a casual echocardiographic finding of a left atrial myxoma that obstructed the mitral valve outflow tract, and an unrelated, synchronous cutaneous squamous cell carcinoma in the sacral area.

  17. Left Right Patterning, Evolution and Cardiac Development

    Directory of Open Access Journals (Sweden)

    Iain M. Dykes

    2014-04-01

    Full Text Available Many aspects of heart development are determined by the left right axis and as a result several congenital diseases have their origins in aberrant left-right patterning. Establishment of this axis occurs early in embryogenesis before formation of the linear heart tube yet impacts upon much later morphogenetic events. In this review I discuss the differing mechanisms by which left-right polarity is achieved in the mouse and chick embryos and comment on the evolution of this system. I then discus three major classes of cardiovascular defect associated with aberrant left-right patterning seen in mouse mutants and human disease. I describe phenotypes associated with the determination of atrial identity and venous connections, looping morphogenesis of the heart tube and finally the asymmetric remodelling of the embryonic branchial arch arterial system to form the leftward looped arch of aorta and associated great arteries. Where appropriate, I consider left right patterning defects from an evolutionary perspective, demonstrating how developmental processes have been modified in species over time and illustrating how comparative embryology can aide in our understanding of congenital heart disease.

  18. Left behind radiological investigations: An inherent problem

    Directory of Open Access Journals (Sweden)

    Giridhar A Govindswamy

    2011-01-01

    Full Text Available Objectives: To assess the number of investigations left behind by patients in radiology department, their cost, and the possible methods of reducing the problem. Materials and Methods: A total of 1424 radiographs, 160 computed tomography (CT scans, 300 ultrasonography (USG reports, and 46 Doppler reports were left behind by patients in one financial year. The total cost of these left behind investigations was calculated and the reports were categorized into normal and abnormal for each modality. Results: Of the radiographs left behind 658 were abnormal, with 211 among these being radiographs of postoperative patients. Thirty-seven percent of CT scans had positive findings. Sixty-eight percent of USG reports had positive findings while 46% of Doppler reports were abnormal. Conclusion: We believe that the cost and number of these left behind investigations over a period of time would definitely be significant for the health care system in a developing country. It is time to think of the possible reasons and methods for containing this problem.

  19. Left atrial laceration with epicardial ligation device.

    Science.gov (United States)

    Keating, Vincent P; Kolibash, Christopher P; Khandheria, Bijoy K; Bajwa, Tanvir; Sra, Jasbir; Kress, David C

    2014-01-01

    Many new devices and techniques are being developed to attempt a reduction in embolic stroke risk for patients with atrial fibrillation who are either unable or unwilling to maintain long-term anticoagulation. One of these new devices (LARIAT®, SentreHEART Inc., Redwood City, California, USA) employs delivery of an epicardial suture to ligate the left atrial appendage after percutaneous pericardial and transseptal access. This series presents three clinical cases that demonstrate a serious and recurrent complication of left atrial laceration and cardiac tamponade shortly following delivery of an epicardial suture ligation to the left atrial appendage. Three clinical cases are described in detail with pre- and postprocedure angiography and echocardiography as well as illustrations reflecting the surgeon's findings on direct visualization of the left atrial lacerations postligation. Potential hypotheses of each injury are examined in light of the case timelines and findings at sternotomy. There was no suggestion that tamponade was related to pericardial or transseptal access, but rather a complication with device delivery. These three patients quickly progressed to clinical cardiac tamponade despite attempted drainage, stressing the importance of cardiovascular surgery backup, including a cardiopulmonary bypass pump, when delivering novel, percutaneous ligation devices for the left atrial appendage.

  20. Generation of an isogenic, gene-corrected iPSC line from a symptomatic 59-year-old female patient with frontotemporal dementia caused by an R406W mutation in the microtubule associated protein tau (MAPT) gene

    DEFF Research Database (Denmark)

    Nimsanor, Natakarn; Poulsen, Ulla; Rasmussen, Mikkel A.

    2016-01-01

    Frontotemporal dementia with parkinsonism linked to chromosome 17q21.2 (FTDP-17) is an autosomal-dominant neurodegenerative disorder. Mutations in the MAPT (microtubule-associated protein tau) gene can cause FTDP-17, but the underlying pathomechanisms of the disease are still unknown. Induced plu...... of genetically corrected iPSCs from a 59-year-old female FTD-17 patient carrying an R406W mutation in the MAPT-gene....

  1. Descriptive anatomy of Heschl?s gyri in 430 healthy volunteers, including 198 left-handers

    OpenAIRE

    Marie, D.; Jobard, G.; Crivello, F.; Perchey, G.; Petit, L.; Mellet, E.; Joliot, M.; Zago, L.; Mazoyer, B.; Tzourio-Mazoyer, N.

    2013-01-01

    This study describes the gyrification patterns and surface areas of Heschl?s gyrus (HG) in 430 healthy volunteers mapped with magnetic resonance imaging. Among the 232 right-handers, we found a large occurrence of duplication (64?%), especially on the right (49 vs. 37?% on the left). Partial duplication was twice more frequent on the left than complete duplication. On the opposite, in the right hemisphere, complete duplication was 10?% more frequent than partial duplication. The most frequent...

  2. [Surface ECG characteristics of right and left atrial flutter].

    Science.gov (United States)

    Rostock, Thomas; Konrad, Torsten; Sonnenschein, Sebastian; Mollnau, Hanke; Ocete, Blanca Quesada; Bock, Karsten; Spittler, Raphael; Huber, Carola; Theis, Cathrin

    2015-09-01

    Atrial tachycardia in virtually all areas of both atria has become more important in the clinical management of patients with previous complex atrial fibrillation ablation. Accurate interpretation of surface electrocardiogram (ECG) characteristics is of paramount importance to localize the origin of atrial tachycardia, particularly for planning interventional treatment. This article highlights the ECG features of different types of right and left atrial tachycardia. Typical right atrial flutter through the cavotricuspid isthmus conducts septally in a cranial direction and demonstrates sawtooth-like flutter waves which start negative in II, III and aVF and then show a steep slope upwards to the isoelectric line. The flutter rate typically ranges between 240-250 beats/min. In contrast, right atrial flutter in a clockwise rotation, flutter around the vena cava inferior or superior and around a scar (e.g. after cardiac surgery) show positive or biphasic flutter waves (lower or upper loop reentry). Left atrial flutter waves (e.g. around the mitral valve or around the pulmonary veins) are very heterogeneous and are typically positive in V1 as the left atrium is located in the posterior mediastinum. Specific knowledge of flutter wave morphology in surface ECG facilitates planning and performance of the ablation strategy.

  3. Left-Wing Extremism: The Current Threat

    Energy Technology Data Exchange (ETDEWEB)

    Karl A. Seger

    2001-04-30

    Left-wing extremism is ''alive and well'' both in the US and internationally. Although the current domestic terrorist threat within the U. S. is focused on right-wing extremists, left-wing extremists are also active and have several objectives. Leftist extremists also pose an espionage threat to U.S. interests. While the threat to the U.S. government from leftist extremists has decreased in the past decade, it has not disappeared. There are individuals and organizations within the U.S. who maintain the same ideology that resulted in the growth of left-wing terrorism in this country in the 1970s and 1980s. Some of the leaders from that era are still communicating from Cuba with their followers in the U.S., and new leaders and groups are emerging.

  4. Left ventricular performance during psychological stress

    International Nuclear Information System (INIS)

    Young, D.Z.; Massachusetts General Hospital, Boston; Dimsdale, J.E.; Moore, R.H.; Barlai-Kovach, M.; Newell, J.B.; McKusick, K.A.; Boucher, C.A.; Fifer, M.A.; Strauss, H.W.

    1989-01-01

    Left ventricular ejection fraction, systolic blood pressure and plasma norepinephrine were measured in six normotensive and six mildly hypertensive subjects during rest and psychological stress. Compared with rest, 8 of the 12 subjects developed significant changes in ejection fraction (increase in 6, decrease in 2); 10 of 12 subjects developed significant elevations of plasma norepinephrine; and all developed significant increases in systolic blood pressure. When the stress effects were examined for the total group, as opposed to within subjects, there were significant increases in plasma norepinephrine and systolic blood pressure but, interestingly, mean ejection fraction and stroke volume remained unchanged, implying stress led to increased left ventricular contractility. (orig.)

  5. Echocardiographic study of left atrial myxoma

    Directory of Open Access Journals (Sweden)

    Dalal J

    1979-01-01

    Full Text Available Four cases of left atrial myxoma were diagnosed pre-operatively by echocardiography. All cases showed characteristic echocardio-graphic features of variegated shadows behind the mitral valve in diastole and within the left atrium in systole. In two cases the my-xomas were surgically removed and confirmed on histology. In one case the post-operative echocardiogram showed complete dis-appearance of the abnormal shadows. Echocardiography is the most reliable method today for the diagnosis of a myxoma.

  6. “Getting the Left Right”

    DEFF Research Database (Denmark)

    Klinke, Marianne E.; Zahavi, Dan; Hjaltason, Haukur

    2015-01-01

    experience was captured in the overarching theme, “getting the left right,” which encompasses the two subthemes of (a) surreal awareness of the left and (b) emergence of a different world. Patients had unclear perceptions of their own body and surroundings, their attention was brittle, and they encountered...... bewildering reactions from other people. They simultaneously pursued the ineffable neglected space and searched for coherence. The vulnerability, loss, and conflicting perceptions that patients with neglect face should be acknowledged and alleviation sought. Facilitating methods should provide additional...

  7. Clustered survival data with left-truncation

    DEFF Research Database (Denmark)

    Eriksson, Frank; Martinussen, Torben; Scheike, Thomas H.

    2015-01-01

    Left-truncation occurs frequently in survival studies, and it is well known how to deal with this for univariate survival times. However, there are few results on how to estimate dependence parameters and regression effects in semiparametric models for clustered survival data with delayed entry....... Surprisingly, existing methods only deal with special cases. In this paper, we clarify different kinds of left-truncation and suggest estimators for semiparametric survival models under specific truncation schemes. The large-sample properties of the estimators are established. Small-sample properties...

  8. Left paraduodenal hernias; Hernias paraduodenales izquierdas

    Energy Technology Data Exchange (ETDEWEB)

    Lopez-Negrete, L.; Garcia-Lozano, J.; Sanchez, J. L.; Tejeiro, A.; Sala, J. [Hospital Valle del Nalon. Riano-Sama. Asturias (Spain)

    2002-07-01

    We report two cases of left paraduodenal internal hernias located in the fossa of Landzert that were diagnosed by CT. Internal hernias are an infrequent cause of acute abdomen, due to the intestinal sub occlusion they produce. Left paraduodenal hernias are responsible for about 50% of internal hernias. CT makes it possible to demonstrate the group of herniated loops between the stomach, fourth segment of the duodenum, descending colon, and tail of the pancreas. The identification of the loops in an atypical position together with displaced blood vessels (mesenteric vessels) and colon gives concerns on them a typical radiological semiology that makes them easily identifiable. (Author) 9 refs.

  9. Left ventricular assist device implantation via left thoracotomy: alternative to repeat sternotomy.

    Science.gov (United States)

    Pierson, Richard N; Howser, Renee; Donaldson, Terri; Merrill, Walter H; Dignan, Rebecca J; Drinkwater, Davis C; Christian, Karla G; Butler, Javed; Chomsky, Don; Wilson, John R; Clark, Rick; Davis, Stacy F

    2002-03-01

    Repeat sternotomy for left ventricular assist device insertion may result in injury to the right heart or patent coronary grafts, complicating intraoperative and postoperative management. In 4 critically ill patients, left thoracotomy was used as an alternative to repeat sternotomy. Anastomosis of the outflow conduit to the descending thoracic aorta provided satisfactory hemodynamic support.

  10. Transient Beneficial Effects of Excitatory Theta Burst Stimulation in a Patient with Phonological Agraphia after Left Supramarginal Gyrus Infarction

    Science.gov (United States)

    Nardone, Raffaele; De Blasi, Pierpaolo; Zuccoli, Giulio; Tezzon, Frediano; Golaszewski, Stefan; Trinka, Eugen

    2012-01-01

    We report a patient showing isolated phonological agraphia after an ischemic stroke involving the left supramarginal gyrus (SMG). In this patient, we investigated the effects of focal repetitive transcranial magnetic stimulation (rTMS) given as theta burst stimulation (TBS) over the left SMG, corresponding to the Brodmann area (BA) 40. The patient…

  11. Impact of aortic prosthesis-patient mismatch on left ventricular mass regression.

    Science.gov (United States)

    Alassal, Mohamed A; Ibrahim, Bedir M; Elsadeck, Nabil

    2014-06-01

    Prostheses used for aortic valve replacement may be small in relation to body size, causing prosthesis-patient mismatch and delaying left ventricular mass regression. This study examined the effect of prosthesis-patient mismatch on regression of left ventricular mass after aortic valve replacement. We prospectively studied 96 patients undergoing aortic valve replacement between 2007 and 2012. Mean and peak gradients and indexed effective orifice area were measured by transthoracic echocardiography at 3 and 6 months postoperatively. Patient-prosthesis mismatch was defined as indexed effective orifice area ≤0.85 cm(2)·m(-2). Moderate prosthesis-patient mismatch was present in 25% of patients. There were no significant differences in demographic and operative data between patients with and without prosthesis-patient mismatch. Left ventricular dimensions, posterior wall thickness, transvalvular gradients, and left ventricular mass decreased significantly after aortic valve replacement in both groups. The interventricular septal diameter and left ventricular mass index regression, and left ventricular ejection fraction were better in patients without prosthesis-patient mismatch. There was a significant positive correlation between the postoperative indexed effective orifice area of each valve prosthesis and the rate of left ventricular mass regression. Prosthesis-patient mismatch leads to higher transprosthetic gradients and impaired left ventricular mass regression. A small-sized valve prosthesis does not necessarily result in prosthesis-patient mismatch, and may be perfectly adequate in patient with small body size. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  12. Magnetization of left-handed metamaterials

    International Nuclear Information System (INIS)

    Kourakis, I; Shukla, P K

    2006-01-01

    We propose a possible mechanism for the generation of magnetic fields in negative refraction index composite metamaterials. Considering the propagation of a high-frequency modulated amplitude electric field in a left-handed material (LHM), we show that the ponderomotive interaction between the field and low-frequency potential distributions leads to spontaneous generation of magnetic fields, whose form and properties are discussed

  13. Left ventricular mass: Myxoma or thrombus?

    Directory of Open Access Journals (Sweden)

    Monish S Raut

    2015-01-01

    Full Text Available Patient with embolic episode should always be evaluated for cardiac mass. Mass in left ventricular can be a myxoma or thrombus even in a normal functioning heart . In either case, mobile mass with embolic potential should be surgically resected.

  14. Left atrial myxoma with biventricular dysfunction

    Directory of Open Access Journals (Sweden)

    Monish S. Raut

    2016-09-01

    Full Text Available Occurrence of left atrial myxoma with severe ventricular dysfunction without any obstructive coronary artery disease, as presented in our case, is very rare. It may be due to undiagnosed concomitant dilated cardiomyopathy or unknown cardiodepressant effect of myxoma which warrants further research.

  15. Left atrial myxoma with biventricular dysfunction.

    Science.gov (United States)

    Raut, Monish S; Shad, Sujay; Maheshwari, Arun

    2016-09-01

    Occurrence of left atrial myxoma with severe ventricular dysfunction without any obstructive coronary artery disease, as presented in our case, is very rare. It may be due to undiagnosed concomitant dilated cardiomyopathy or unknown cardiodepressant effect of myxoma which warrants further research. Copyright © 2016 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  16. Vergisson 4: a left-handed Neandertal.

    Science.gov (United States)

    Condemi, Silvana; Monge, Janet; Quertelet, Sylvain; Frayer, David W; Combier, Jean

    2017-01-01

    Handedness is an important marker for lateralization of humans in the modern and fossil record. For the most part, Neandertals and their ancestors are strongly right-handed. We describe a single tooth from a Neandertal level at Vergisson 4 (Vg 4-83). This left upper central incisor shows all the features typical of Neandertal incisors. It also exhibits a predominance of left-handed striations. Striations on the incisor's labial surface were mapped at 20x magnification using Photoshop. Angulations of the striations were determined from their deviation from the maximum mesio-distal line and were analyzed using NIH's freeware, Image J. Of the 60 labial surface striations, Vg 4-83 shows a strong predominance of left-handed striations (46; 76.7%), which are statistically significantly different (p handed striations. The identification of another left-handed Neandertal adds to our understanding about handedness variation in this fossil hominin. Given the high frequency of right-handed Neandertals, the 90: 10 modern ratio is still preserved in this group. © 2016 Wiley Periodicals, Inc.

  17. Electrocardiographic features suggestive of a left. ventricular ...

    African Journals Online (AJOL)

    1983-01-01

    Jan 1, 1983 ... sniper with an Armourlite assault rifle in Ireland. He had received a single bullet injury to the left ... His history negated any cardiovascular impairment, angina or limitation in effort tolerance. He plays ... In view of the patient's age, history and the cardiovascular findings, we would like to postulate that the ...

  18. Radiative left-right Dirac neutrino mass

    Science.gov (United States)

    Ma, Ernest; Sarkar, Utpal

    2018-01-01

    We consider the conventional left-right gauge extension of the standard model of quarks and leptons without a scalar bidoublet. We study systematically how one-loop radiative Dirac neutrino masses may be obtained. In addition to two well-known cases from almost 30 years ago, we find two new scenarios with verifiable predictions.

  19. Giant melanoma of the left thumb

    NARCIS (Netherlands)

    Zeebregts, CJAM; Schraffordt Koops, H.

    A 74-year-old female patient is described with a giant melanoma of the left thenar and concomitant bilateral pulmonary metastases. Palliative treatment consisted of a two-staged procedure in order to save the limb from amputation. Firstly, perfusion with gamma-interferon, tumour necrosis

  20. Submitral Left Ventricular Aneurysm Associated with Thrombus

    African Journals Online (AJOL)

    2018-01-01

    Jan 1, 2018 ... She was given drugs for management of heart failure and ... treatment abroad. KEYWORDS: Ethiopia, heart failure, submitral aneurysm, thrombus. INTRODUCTION. Submitral left ventricle aneurysm is a rare cardiovascular disorder worldwide, but ... grade 2 pulmonary edema, and bilateral pleural effusion.

  1. Left-Right Symmetry at LHC

    CERN Document Server

    Maiezza, Alessio; Nesti, Fabrizio; Senjanovic, Goran

    2010-01-01

    We revisit the issue of the limit on the scale of Left-Right symmetry breaking. We focus on the minimal SU(2)_L x SU(2)_R x U(1)_B-L gauge theory with the seesaw mechanism and discuss the two possibilities of defining Left-Right symmetry as parity or charge conjugation. In the commonly adopted case of parity, we perform a complete numerical study of the quark mass matrices and the associated left and right mixing matrices without any assumptions usually made in the literature about the ratio of vacuum expectation values. We find that the usual lower limit on the mass of the right-handed gauge boson from the K mass difference, M_WR>2.5TeV, is subject to a possible small reduction due to the difference between right and left Cabibbo angles. In the case of charge conjugation the limit on M_WR is somewhat more robust. However, the more severe bounds from CP-violating observables are absent in this case. In fact, the free phases can also resolve the present mild discrepancy between the Standard Model and CP-violat...

  2. Left Dislocation: a typological overview | Westbury | Stellenbosch ...

    African Journals Online (AJOL)

    The Left Dislocation construction is a typologically universal phenomenon that has received detailed analysis, from both formal and functional perspectives, in a number of genetically and areally diverse languages. The present paper aims to provide a general overview of this cross-linguistic research with a concentration ...

  3. Left-forbidding cooperating distributed grammar systems

    Czech Academy of Sciences Publication Activity Database

    Goldefus, F.; Masopust, Tomáš; Meduna, A.

    2010-01-01

    Roč. 411, 40-42 (2010), s. 3661-3667 ISSN 0304-3975 Institutional research plan: CEZ:AV0Z10190503 Keywords : cooperating distributed grammar system * cooperating derivation mode * left-forbidding grammar * generative power * descriptional complexity Subject RIV: BA - General Mathematics Impact factor: 0.838, year: 2010 http://www.sciencedirect.com/science/article/pii/S0304397510003440

  4. Left Ventricular Thrombus among patients undergoing Transthoracic ...

    African Journals Online (AJOL)

    Objective: Left Ventricular Thrombus (LVT) is a well recognized complication of various cardiac conditions, particularly following an acute anterior myocardial infarction and in those with systolic congestive heart failure. Transthoracic echocardiography (TTE) remains the most common imaging modality to make the diagnosis ...

  5. Modern Democratic Left and Economic Crisis

    Directory of Open Access Journals (Sweden)

    Dubravko Radošević

    2011-01-01

    Full Text Available Political left, notably social democracy, implies acceptance of market economy and parliamentary democracy, as a political framework within which the interests of large sections of the population could be preserved. Does the modern political left have a chance to revive, after the global economic crisis? We believe it does. The core idea of social democracy is the idea of equality. It is the growing inequality that could help the return of social democracy. In Croatia, during transition, there was predominantly the process of neoliberalization, and the democratic left accepted a neoliberal economic agenda. Currently Croatia has a structural economic problem and deep recession. Due to the global crisis, the democratic left in Croatia should abandon economic neoliberalism. Macroeconomic policy decision-makers in Croatia should reject orthodox policies, and it is necessary to implement heterodox economic policies. There is an urgent need to achieve a new (postKeynesian consensus, a new combination of post-Keynesian economics and social democracy

  6. Left-sided portal hypertension revisited

    Directory of Open Access Journals (Sweden)

    Antonio Manenti

    2016-12-01

    Conclusions: In every case of left-sided portal hypertension, upper digestive endoscopy and close follow-up are recommended. Besides, computed tomography can demonstrate particular conditions directly favoring gastroesophageal varices, and aid in selection of the appropriate therapeutic decisions. [Arch Clin Exp Surg 2016; 5(4.000: 211-215

  7. Reorganization of syntactic processing following left-hemisphere brain damage: does right-hemisphere activity preserve function?

    Science.gov (United States)

    Tyler, Lorraine K; Wright, Paul; Randall, Billi; Marslen-Wilson, William D; Stamatakis, Emmanuel A

    2010-11-01

    The extent to which the human brain shows evidence of functional plasticity across the lifespan has been addressed in the context of pathological brain changes and, more recently, of the changes that take place during healthy ageing. Here we examine the potential for plasticity by asking whether a strongly left-lateralized system can successfully reorganize to the right-hemisphere following left-hemisphere brain damage. To do this, we focus on syntax, a key linguistic function considered to be strongly left-lateralized, combining measures of tissue integrity, neural activation and behavioural performance. In a functional neuroimaging study participants heard spoken sentences that differentially loaded on syntactic and semantic information. While healthy controls activated a left-hemisphere network of correlated activity including Brodmann areas 45/47 and posterior middle temporal gyrus during syntactic processing, patients activated Brodmann areas 45/47 bilaterally and right middle temporal gyrus. However, voxel-based morphometry analyses showed that only tissue integrity in left Brodmann areas 45/47 was correlated with activity and performance; poor tissue integrity in left Brodmann area 45 was associated with reduced functional activity and increased syntactic deficits. Activity in the right-hemisphere was not correlated with damage in the left-hemisphere or with performance. Reduced neural integrity in the left-hemisphere through brain damage or healthy ageing results in increased right-hemisphere activation in homologous regions to those left-hemisphere regions typically involved in the young. However, these regions do not support the same linguistic functions as those in the left-hemisphere and only indirectly contribute to preserved syntactic capacity. This establishes the unique role of the left hemisphere in syntax, a core component in human language.

  8. Sympathetic stimulation alters left ventricular relaxation and chamber size.

    Science.gov (United States)

    Burwash, I G; Morgan, D E; Koilpillai, C J; Blackmore, G L; Johnstone, D E; Armour, J A

    1993-01-01

    Alterations in left ventricular (LV) contractility, relaxation, and chamber dimensions induced by efferent sympathetic nerve stimulation were investigated in nine anesthetized open-chest dogs in sinus rhythm. Supramaximal stimulation of acutely decentralized left stellate ganglia augmented heart rate, LV systolic pressure, and rate of LV pressure rise (maximum +dP/dt, 1,809 +/- 191 to 6,304 +/- 725 mmHg/s) and fall (maximum -dP/dt, -2,392 +/- 230 to -4,458 +/- 482 mmHg/s). It also reduced the time constant of isovolumic relaxation, tau (36.5 +/- 4.8 to 14.9 +/- 1.1 ms). Simultaneous two-dimensional echocardiography recorded reductions in end-diastolic and end-systolic LV cross-sectional chamber areas (23 and 31%, respectively), an increase in area ejection fraction (32%), and increases in end-diastolic and end-systolic wall thicknesses (14 and 13%, respectively). End-systolic and end-diastolic wall stresses were unchanged by stellate ganglion stimulation (98 +/- 12 to 95 +/- 9 dyn x 10(3)/cm2; 6.4 +/- 2.4 to 2.4 +/- 0.3 dyn x 10(3)/cm2, respectively). Atrial pacing to similar heart rates did not alter monitored indexes of contractility. Dobutamine and isoproterenol induced changes similar to those resulting from sympathetic neuronal stimulation. These data indicate that when the efferent sympathetic nervous system increases left ventricular contractility and relaxation, concomitant reductions in systolic and diastolic dimensions of that chamber occur that are associated with increasing wall thickness such that LV wall stress changes are minimized.

  9. Left ventricular function in chronic aortic regurgitation

    International Nuclear Information System (INIS)

    Iskandrian, A.S.; Hakki, A.H.; Manno, B.; Amenta, A.; Kane, S.A.

    1983-01-01

    Left ventricular performance was determined in 42 patients with moderate or severe aortic regurgitation during upright exercise by measuring left ventricular ejection fraction and volume with radionuclide ventriculography. Classification of the patients according to exercise tolerance showed that patients with normal exercise tolerance (greater than or equal to 7.0 minutes) had a significantly higher ejection fraction at rest (probability [p] . 0.02) and during exercise (p . 0.0002), higher cardiac index at exercise (p . 0.0008) and lower exercise end-systolic volume (p . 0.01) than did patients with limited exercise tolerance. Similar significant differences were noted in younger patients compared with older patients in ejection fraction at rest and exercise (both p . 0.001) and cardiac index at rest (p . 0.03) and exercise (p . 0.0005). The end-diastolic volume decreased during exercise in 60% of the patients. The patients with a decrease in volume were significantly younger and had better exercise tolerance and a larger end-diastolic volume at rest than did patients who showed an increase in volume. The mean corrected left ventricular end-diastolic radius/wall thickness ratio was significantly greater in patients with abnormal than in those with normal exercise reserve (mean +/- standard deviation 476 +/- 146 versus 377 +/- 92 mm Hg, p less than 0.05). Thus, in patients with chronic aortic regurgitation: 1) left ventricular systolic function during exercise was related to age, exercise tolerance and corrected left ventricular end-diastolic radius/wall thickness ratio, and 2) the end-diastolic volume decreased during exercise, especially in younger patients and patients with normal exercise tolerance or a large volume at rest

  10. Assessment of stiffness of the hypertrophied left ventricle of bicyclists using left ventricular inflow Doppler velocimetry.

    Science.gov (United States)

    Fagard, R; Van den Broeke, C; Bielen, E; Vanhees, L; Amery, A

    1987-06-01

    Sixteen male bicyclists and 16 control subjects were studied to assess whether the left ventricular hypertrophy of athletes is associated with changes in diastolic left ventricular function. The cyclists had a larger left ventricular internal diameter on echocardiography (55.2 versus 47.9 mm; p less than 0.001) and a disproportionate increase in wall thickness relative to the internal diameter (0.48 versus 0.41; p less than 0.01), indicating a mixed eccentric-concentric type of hypertrophy. Left ventricular inflow Doppler velocimetry showed similar results in athletes and control subjects for peak flow velocities in the atrial contraction phase (30 versus 32 cm/s; p = NS) and in the early diastolic rapid filling phase (71 versus 67 cm/s; p = NS). The similar ratio of both velocities, that is, 0.43 in the cyclists and 0.49 in the control subjects, suggests that left ventricular distensibility is unaltered in cyclists. It is concluded that the left ventricular hypertrophy observed in cyclists is not associated with changes in ventricular stiffness, as estimated from left ventricular inflow Doppler velocimetry.