Plasma Biomarkers Differentiate Parkinson’s Disease From Atypical Parkinsonism Syndromes

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Chin-Hsien Lin

2018-04-01

Full Text Available Objective: Parkinson’s disease (PD has significant clinical overlaps with atypical parkinsonism syndromes (APS, which have a poorer treatment response and a more aggressive course than PD. We aimed to identify plasma biomarkers to differentiate PD from APS.Methods: Plasma samples (n = 204 were obtained from healthy controls and from patients with PD, dementia with Lewy bodies (DLB, multiple system atrophy, progressive supranuclear palsy (PSP, corticobasal degeneration (CBD, or frontotemporal dementia (FTD with parkinsonism (FTD-P or without parkinsonism. We measured plasma levels of α-synuclein, total tau, p-Tau181, and amyloid beta 42 (Aβ42 by immunomagnetic reduction-based immunoassay.Results: Plasma α-synuclein level was significantly increased in patients with PD and APS when compared with controls and FTD without parkinsonism (p < 0.01. Total tau and p-Tau181 were significantly increased in all disease groups compared to controls, especially in patients with FTD (p < 0.01. A multivariate and receiver operating characteristic curve analysis revealed that a cut-off value for Aβ42 multiplied by p-Tau181 for discriminating patients with FTD from patients with PD and APS was 92.66 (pg/ml2, with an area under the curve (AUC of 0.932. An α-synuclein cut-off of 0.1977 pg/ml could separate FTD-P from FTD without parkinsonism (AUC 0.947. In patients with predominant parkinsonism, an α-synuclein cut-off of 1.388 pg/ml differentiated patients with PD from those with APS (AUC 0.87.Conclusion: Our results suggest that integrated plasma biomarkers improve the differential diagnosis of PD from APS (PSP, CBD, DLB, and FTD-P.

Wolff-Parkinson-White Syndrome and Accessory Pathways

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... the American Heart Association Cardiology Patient Page Wolff-Parkinson-White Syndrome and Accessory Pathways James Kulig , Bruce ... rate, which can be dangerous. What is Wolff-Parkinson-White Syndrome? Wolff-Parkinson-White syndrome (WPW) is ...

Comparison with Parkinson's disease and Parkinsonism in dementia with lewy bodies

International Nuclear Information System (INIS)

Kang, Do Young; Park, Kyung Won; Kim, Jae Woo

2002-01-01

Dementia with lewy bodies (DLB) is recognized as a common cause of dementia in elderly people. It is characterized by Parkinsonism as well as fluctuation cognition and visual hallucination. It is not easy to differentiated DLB with Parkinsonism from Parkinson's disease (PD). We compared clinical features and I-123 IPT SPECT findings between two groups. Ten probable DLB patients and 15 PD patients were included. They were all matched for age (66.9±8.5) vs 65.8±8.5), education period (9.8±4.9 vs 9.1±4.5) and H and Y stage (2.9±0.8). Clinical features were scored. I-123 IPT SPECT were performed. The mean age of disease onset was seen more common in PD, but the severity of rigidity and bradykinesia was not significantly different. We found no significant difference of responsiveness to levodopa measured with decrement of UPDRS scores before and after treatment of levodopa. The mean disease duration reaching same H and Y stage was longer in PD than in DLB. The average K-MMSE score was significantly lower in DLB than in PD. The uptake ratio in the striatum in DLB was comparable to that in PD. In differentiating the two conditions the speed of disease progression and cognitive state were considered to be important. The level of dopamine transporter was not different between two diseases with same H and Y stage

Comparison study of positron emission tomography, X-ray CT and MRI in Parkinsonism with dementia

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Okada, Junichi; Peppard, R.; Calne, D.B.

1989-01-01

Brain atrophy and local cerebral metabolic rate of glucose (LCMR-glc) in Parkinson's disease with dementia and Parkinsonism-dementia complex (PDC) were studied using positron emission tomography (PET) with F-18-2-deoxy-2-fluoro-D-glucose, X-ray CT and magnetic resonance imaging (MRI). The group of Parkinson's disease with dementia (n=7) had a significantly decreased LCMR-glc in all regions when compared with the age-matched normal group. In the group of Parkinson's disease without dementia (n=6), LCMR-glc was also significantly lower than the control group, although it was higher than the group with associated dementia. Some of the normal aged persons had cortical atrophy. There was no correlation between LCMR-glc and cortical atrophy. Six Guamnian patients had PDC associated with amyotrophic lateral sclerosis (ALS), and four patients had it without ALS. LCMR-glc did not differ in the two groups. It was, however, significantly lower than that in 5 Guamanian and 10 Caucasian normal persons. The group of PDC had a noticeable cortical atrophy and ventricular dilatation, regardless of the presence or absence of ALS. There was correlation between decrease of LCMR-glc and cortical atrophy of the frontal, parietal and temporal lobes. Parkinson's disease and PDC were different from Alzheimer's disease in which a decreased LCMR-glc has been reported to be usually confined to the cerebral cortex. Cortical atrophy and ventricular dilatation were depicted on MRI and CT in the PDC group, but did not in the group of Parkinson's disease. PET was useful in the functional examination and both MRI and CT were useful in the anatomical examination of these diseases. (Namekawa, K)

Comparison study of positron emission tomography, X-ray CT and MRI in Parkinsonism with dementia

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Okada, Junichi; Peppard, R; Calne, D B

1989-05-01

Brain atrophy and local cerebral metabolic rate of glucose (LCMR-glc) in Parkinson's disease with dementia and Parkinsonism-dementia complex (PDC) were studied using positron emission tomography (PET) with F-18-2-deoxy-2-fluoro-D-glucose, X-ray CT and magnetic resonance imaging (MRI). The group of Parkinson's disease with dementia (n=7) had a significantly decreased LCMR-glc in all regions when compared with the age-matched normal group. In the group of Parkinson's disease without dementia (n=6), LCMR-glc was also significantly lower than the control group, although it was higher than the group with associated dementia. Some of the normal aged persons had cortical atrophy. There was no correlation between LCMR-glc and cortical atrophy. Six Guamnian patients had PDC associated with amyotrophic lateral sclerosis (ALS), and four patients had it without ALS. LCMR-glc did not differ in the two groups. It was, however, significantly lower than that in 5 Guamanian and 10 Caucasian normal persons. The group of PDC had a noticeable cortical atrophy and ventricular dilatation, regardless of the presence or absence of ALS. There was correlation between decrease of LCMR-glc and cortical atrophy of the frontal, parietal and temporal lobes. Parkinson's disease and PDC were different from Alzheimer's disease in which a decreased LCMR-glc has been reported to be usually confined to the cerebral cortex. Cortical atrophy and ventricular dilatation were depicted on MRI and CT in the PDC group, but did not in the group of Parkinson's disease. PET was useful in the functional examination and both MRI and CT were useful in the anatomical examination of these diseases. (Namekawa, K).

Imaging biomarkers in Parkinson?s disease and Parkinsonian syndromes: current and emerging concepts

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Saeed, Usman; Compagnone, Jordana; Aviv, Richard I.; Strafella, Antonio P.; Black, Sandra E.; Lang, Anthony E.; Masellis, Mario

2017-01-01

Two centuries ago in 1817, James Parkinson provided the first medical description of Parkinson?s disease, later refined by Jean-Martin Charcot in the mid-to-late 19th century to include the atypical parkinsonian variants (also termed, Parkinson-plus syndromes). Today, Parkinson?s disease represents the second most common neurodegenerative disorder with an estimated global prevalence of over 10 million. Conversely, atypical parkinsonian syndromes encompass a group of relatively heterogeneous d...

The different faces of the p. A53T alpha-synuclein mutation: A screening of Greek patients with parkinsonism and/or dementia.

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

2018-04-13

The p. A53T mutation in the alpha-synuclein (SNCA) gene is a rare cause of autosomal dominant Parkinson's disease (PD). Although generally rare, it is particularly common in the Greek population due to a founder effect. A53T-positive PD patients often develop dementia during disease course and may very rarely present with dementia. We screened for the p. A53T SNCA mutation a total of 347 cases of Greek origin with parkinsonism and/or dementia, collected over 15 years at the Neurogenetics Unit, Eginition Hospital, University of Athens. Cases were classified into: "pure parkinsonism", "pure dementia" and "parkinsonism plus dementia". In total, 4 p. A53T SNCA mutation carriers were identified. All had autosomal dominant family history and early onset. Screening of the "pure parkinsonism" category revealed 2 cases with typical PD. The other two mutation carriers were identified in the "parkinsonism plus dementia" category. One had a diagnosis of PD dementia and the other of behavioral variant frontotemporal dementia. Screening of patients with "pure dementia" failed to identify any further A53T-positive cases. Our results confirm that the p. A53T SNCA mutation is relatively common in Greek patients with PD or PD plus dementia, particularly in cases with early onset and/or autosomal dominant family history. Copyright © 2017 Elsevier B.V. All rights reserved.

Comparison study of positron emission tomography, X-ray CT and MRI in Parkinsonism with dementia

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Okada, Junichi; Peppard, R.; Calne, D.B.

1989-05-01

Brain atrophy and local cerebral metabolic rate of glucose (LCMR-glc) in Parkinson's disease with dementia and Parkinsonism-dementia complex (PDC) were studied using positron emission tomography (PET) with F-18-2-deoxy-2-fluoro-D-glucose, X-ray CT and magnetic resonance imaging (MRI). The group of Parkinson's disease with dementia (n=7) had a significantly decreased LCMR-glc in all regions when compared with the age-matched normal group. In the group of Parkinson's disease without dementia (n=6), LCMR-glc was also significantly lower than the control group, although it was higher than the group with associated dementia. Some of the normal aged persons had cortical atrophy. There was no correlation between LCMR-glc and cortical atrophy. Six Guamnian patients had PDC associated with amyotrophic lateral sclerosis (ALS), and four patients had it without ALS. LCMR-glc did not differ in the two groups. It was, however, significantly lower than that in 5 Guamanian and 10 Caucasian normal persons. The group of PDC had a noticeable cortical atrophy and ventricular dilatation, regardless of the presence or absence of ALS. There was correlation between decrease of LCMR-glc and cortical atrophy of the frontal, parietal and temporal lobes. Parkinson's disease and PDC were different from Alzheimer's disease in which a decreased LCMR-glc has been reported to be usually confined to the cerebral cortex. Cortical atrophy and ventricular dilatation were depicted on MRI and CT in the PDC group, but did not in the group of Parkinson's disease. PET was useful in the functional examination and both MRI and CT were useful in the anatomical examination of these diseases. (Namekawa, K).

Brain perfusion SPECT in dementia syndromes

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Libus, P.; Stupalova, J.; Kuzelka, I.; Konrad, J.

2002-01-01

Aim: Brain perfusion SPECT is used in differential diagnostics of dementia syndromes. First of all the aim is to distinguish vascular dementia from degenerative dementia and to differentiate dementia from delirium, psychiatric syndromes, depression and secondary dementia, which is important in relation to therapy. The purpose of our study was to detect significance of BP SPECT and include it into the diagnostic process in dementia syndromes. Materials and methods: 51 women and 63 men aged 55 - 88 were evaluated in the study. The patients correspond to the general criteria of dementia diagnosis. They were sent to the examination by neurological, internal and psychiatric departments and out-patient departments. All patients were examined by 99mTc ECD SPECT using a double head camera PRISM 200 VP with LEHR collimator. The scintigraphic data were evaluated by the visual and semiquantitative analysis. Results: It was established that most patients in our group had vascular dementia, while Alzheimer's disease was second. In other groups we found out dementia at strategic infarct location, e.g. in gyrus angularis in the dominant hemisphere, frontal temporal lobe dementia and alcoholic dementia. Twenty-four patients had a normal diagnosis. Fifteen of them had a somatic reason of the delirious state and were re-classified into pseudodementia. Nine patients were not diagnostically included and the examination will repeated in four months time. Conclusion: We have found out a good applicability of brain perfusion SPECT in dementia syndromes diagnosis in our work. The best diagnosticable and most specific were the findings in multi-infarct dementia, Alzheimer's disease and frontal temporal lobe dementia. When vascular dementia is concerned we can even distinguish dementia at strategic infarction location, e.g. in thalamus, basal frontal telencefalon, in gyrus angularis of the dominant hemisphere, etc

CBF tomograms with [/sup 99m/Tc-HM-PAO in patients with dementia (Alzheimer type and HIV) and Parkinson's disease--initial results

International Nuclear Information System (INIS)

Costa, D.C.; Ell, P.J.; Burns, A.; Philpot, M.; Levy, R.

1988-01-01

We present preliminary data on the utility of functional brain imaging with [99mTc]-d,l-HM-PAO and single photon emission computed tomography (SPECT) in the study of patients with dementia of the Alzheimer type (DAT), HIV-related dementia syndrome, and the on-off syndrome of Parkinson's disease. In comparison with a group of age-matched controls, the DAT patients revealed distinctive bilateral temporal and posterior parietal deficits, which correlate with detailed psychometric evaluation. Patients with amnesia as the main symptom (group A) showed bilateral mesial temporal lobe perfusion deficits (p less than 0.02). More severely affected patients (group B) with significant apraxia, aphasia, or agnosia exhibited patterns compatible with bilateral reduced perfusion in the posterior parietal cortex, as well as reduced perfusion to both temporal lobes, different from the patients of the control group (p less than 0.05). SPECT studies of HIV patients with no evidence of intracraneal space occupying pathology showed marked perfusion deficits. Patients with Parkinson's disease and the on-off syndrome studied during an on phase (under levodopa therapy) and on another occasion after withdrawal of levodopa (off) demonstrated a significant change in the uptake of [99mTc]-d,l-HM-PAO in the caudate nucleus (lower on off) and thalamus (higher on off). These findings justify the present interest in the functional evaluation of the brain of patients with dementia. [99mTc]-d,l-HM-PAO and regional cerebral blood flow (rCBF)/SPECT appear useful and highlight individual disorders of flow in a variety of neuropsychiatric conditions

Cerebral blood flow changes in Parkinson's disease associated with dementia

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Derejko, M.; Lass, P.; Slawek, J.; Nyka, W.M.

2001-01-01

Dementia is one of the main non-motor symptoms of Parkinson's disease (PD) and it is diagnosed in about 30% of cases. Its aetiology remains unclear and contributing factors are controversial. Dementia may be more common in old patients with severe motor symptoms and mild cognitive impairment. Clinico-pathological studies show the association between dementia in PD and the age-related group of dementias, such as AD and VaD. A valuable aid in the assessment of dementia in PD is cerebral blood flow (CBF) brain SPECT scanning. It shows three different patterns of rCBF reduction, including frontal lobe hypoperfusion, iu Alzheimer-likel type of hypoperfusion and multiple, vascular defects. The heterogeneity of rCBF reduction may reflect the multifactorial pathophysiology of dementia in PD. It may result from concomitant AD pathology, cerebrovascular disease, destruction of nigro-striato-frontal projection or may be a distinct disease of different aetiology. (author)

Astrocyte dysfunction following molybdenum-associated purine loading could initiate Parkinson's disease with dementia.

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Bourke, Christopher A

2018-01-01

Sporadic or idiopathic Parkinson's disease is a movement disorder with a worldwide distribution, a long pre-clinical latent period and a frequent association with dementia. The combination of molybdenum deficiency and purine ingestion could explain the movement disorder, the distribution, the latent period and the dementia association. Recent studies in sheep have shown that molybdenum deficiency enables some dietary purines to accumulate in the central nervous system. This causes astrocyte dysfunction, altered neuromodulation and eventually irreversible central nervous system disease. Humans and sheep share the ability to salvage purines and this ability places humans at risk when they ingest xanthosine, inosine, adenosine and guanosine. Adenosine ingestion in molybdenum-deficient humans will lead to adenosine loading and potentially a disturbance to the A2a adenosine receptors in the nigro-striatum. This could result in Parkinson's disease. Guanosine ingestion in molybdenum-deficient humans will lead to guanosine loading and potentially a disturbance to the guanosine receptors in the hippocampus, amygdala and ventral striatum. This could result in dementia. The molybdenum content of the average daily diet in the United States is 0.07 ppm and in the United Kingdom 0.04 ppm. Central nervous system disease occurs in sheep at <0.04 ppm. Consistent with the role proposed for molybdenum deficiency in Parkinson's disease is the observation that affected individuals have elevated sulfur amino acid levels, depressed sulfate levels, and depressed uric acid levels. Likewise the geographical distribution of Parkinson's dementia complex on Guam corresponds with the distribution of molybdenum-deficient soils hence molybdenum-deficient food gardens on that island.

Regional cerebral glucose metabolism in patients with Parkinson's disease with or without dementia

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Sasaki, Masayuki; Ichiya, Yuichi; Hosokawa, Shinichi; Otsuka, Makoto; Kuwabara, Yasuo; Fukumura, Toshimitsu; Kato, Motohiro; Goto, Ikuo; Masuda, Kouji [Kyushu Univ., Fukuoka (Japan). Faculty of Medicine

1992-11-01

By means of positron emission tomography, the cerebral glucose metabolism in 5 patients with Parkinson's disease with dementia was compared with that in 9 patients without dementia, and that in 5 normal volunteers. The metabolic rates for glucose were measured by placing one hundred regions of interest. In the demented patients, cerebral glucose metabolism was diffusely decreased compared with that of the non-demented patients and the normal controls. The most significant decrease in glucose metabolism was observed in the angular gyrus (49.7% of the normal controls). The glucose metabolism in the cingulate, pre- and postcentral, occipital and subcortical regions was relatively spared (62.1 to 85.5% of the normal controls). In the patients without dementia, the glucose metabolism in each region was not significantly different from that in the normal controls. These results suggest that diffuse glucose hypometabolism in the cerebral cortex may correlate with that of patients with Parkinson's disease with dementia. (author).

COTARD SYNDROME IN SEMANTIC DEMENTIA

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Mendez, Mario F.; Ramírez-Bermúdez, Jesús

2011-01-01

Background Semantic dementia is a neurodegenerative disorder characterized by the loss of meaning of words or concepts. semantic dementia can offer potential insights into the mechanisms of content-specific delusions. Objective The authors present a rare case of semantic dementia with Cotard syndrome, a delusion characterized by nihilism or self-negation. Method The semantic deficits and other features of semantic dementia were evaluated in relation to the patient's Cotard syndrome. Results Mrs. A developed the delusional belief that she was wasting and dying. This occurred after she lost knowledge for her somatic discomforts and sensations and for the organs that were the source of these sensations. Her nihilistic beliefs appeared to emerge from her misunderstanding of her somatic sensations. Conclusion This unique patient suggests that a mechanism for Cotard syndrome is difficulty interpreting the nature and source of internal pains and sensations. We propose that loss of semantic knowledge about one's own body may lead to the delusion of nihilism or death. PMID:22054629

Limbic system, the main focus of dementia syndrome

International Nuclear Information System (INIS)

Matsuzawa, Taiju

1990-01-01

Alzheimer disease and multi-infarct dementia are two entirely different diseases producing almost the same abnormalities as dementia syndrome. The statistical studies with MRI to locate the focus of dementia syndrome in the neocortex was an absolute failure. With MRI there is drastic atrophy and destruction of the amygdala and hippocampus suggesting the limbic system as the focus of dementia syndrome. Destruction of the limbic system in particular amygdala and hippocampus produced the functional obstruction brought about by the marked reduction in the glucose utilization with PET in the bilateral temporal, parietal and occipital association cortices. Although this type constitutes only about 1/5 of all dementia patients. It is considered the fundamental type of dementia syndrome. Aside from this, there is a type wherein simultaneous and symmetrical reductions in glucose utilization of the frontal association cortex and the motor association cortex in the anterior part of the neocortex. This is referred to as type II. It constitutes about 4/5 of all dementia patients which is far more than type I. Based on these results, it is thought that limbic system is the main focus of dementia syndrome. (author)

CBF tomograms with (/sup 99m/Tc-HM-PAO in patients with dementia (Alzheimer type and HIV) and Parkinson's disease--initial results

Energy Technology Data Exchange (ETDEWEB)

Costa, D.C.; Ell, P.J.; Burns, A.; Philpot, M.; Levy, R.

1988-12-01

We present preliminary data on the utility of functional brain imaging with (99mTc)-d,l-HM-PAO and single photon emission computed tomography (SPECT) in the study of patients with dementia of the Alzheimer type (DAT), HIV-related dementia syndrome, and the on-off syndrome of Parkinson's disease. In comparison with a group of age-matched controls, the DAT patients revealed distinctive bilateral temporal and posterior parietal deficits, which correlate with detailed psychometric evaluation. Patients with amnesia as the main symptom (group A) showed bilateral mesial temporal lobe perfusion deficits (p less than 0.02). More severely affected patients (group B) with significant apraxia, aphasia, or agnosia exhibited patterns compatible with bilateral reduced perfusion in the posterior parietal cortex, as well as reduced perfusion to both temporal lobes, different from the patients of the control group (p less than 0.05). SPECT studies of HIV patients with no evidence of intracraneal space occupying pathology showed marked perfusion deficits. Patients with Parkinson's disease and the on-off syndrome studied during an on phase (under levodopa therapy) and on another occasion after withdrawal of levodopa (off) demonstrated a significant change in the uptake of (99mTc)-d,l-HM-PAO in the caudate nucleus (lower on off) and thalamus (higher on off). These findings justify the present interest in the functional evaluation of the brain of patients with dementia. (99mTc)-d,l-HM-PAO and regional cerebral blood flow (rCBF)/SPECT appear useful and highlight individual disorders of flow in a variety of neuropsychiatric conditions.

Goal Setting for Cognitive Rehabilitation in Mild to Moderate Parkinson's Disease Dementia and Dementia with Lewy Bodies.

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Watermeyer, Tamlyn J; Hindle, John V; Roberts, Julie; Lawrence, Catherine L; Martyr, Anthony; Lloyd-Williams, Huw; Brand, Andrew; Gutting, Petra; Hoare, Zoe; Edwards, Rhiannon Tudor; Clare, Linda

2016-01-01

Alongside the physical symptoms associated with Parkinson's disease dementia and dementia with Lewy bodies, health services must also address the cognitive impairments that accompany these conditions. There is growing interest in the use of nonpharmacological approaches to managing the consequences of cognitive disorder. Cognitive rehabilitation is a goal-orientated behavioural intervention which aims to enhance functional independence through the use of strategies specific to the individual's needs and abilities. Fundamental to this therapy is a person's capacity to set goals for rehabilitation. To date, no studies have assessed goal setting in early-stage Parkinson's disease dementia or dementia with Lewy bodies. Semistructured interviews were carried out with 29 participants from an ongoing trial of cognitive rehabilitation for people with these conditions. Here, we examined the goal statements provided by these participants using qualitative content analysis, exploring the types and nature of the goals set. Participants' goals reflected their motivations to learn new skills or improve performance in areas such as technology-use, self-management and orientation, medication management, and social and leisure activities. These results suggest that goal setting is achievable for these participants, provide insight into the everyday cognitive difficulties that they experience, and highlight possible domains as targets for intervention. The trial is registered with ISRCTN16584442 (DOI 10.1186/ISRCTN16584442 13/04/2015).

The Brainstem Pathologies of Parkinson's Disease and Dementia with Lewy Bodies

NARCIS (Netherlands)

Seidel, Kay; Mahlke, Josefine; Siswanto, Sonny; Krueger, Reijko; Heinsen, Helmut; Auburger, Georg; Bouzrou, Mohamed; Grinberg, Lea T.; Wicht, Helmut; Korf, Horst-Werner; den Dunnen, Wilfred; Rueb, Udo

Parkinson's disease (PD) and dementia with Lewy bodies (DLB) are among the human synucleinopathies, which show alpha-synuclein immunoreactive neuronal and/or glial aggregations and progressive neuronal loss in selected brain regions (eg, substantia nigra, ventral tegmental area, pedunculopontine

Depression and dementia in Parkinson's disease.

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Sinanović, Osman; Hudić, Josip; Zukić, Sanela; Kapidžić, Almasa; Zonić, Lejla; Vidović, Mirjana

2015-03-01

Parkinson's disease (PD) is a neurodegenerative disorder causing not only motor dysfunction but also cognitive, psychiatric, autonomic and sensory disturbances. Depression is the most common psychiatric disturbance identified in patients with PD and has been shown to be more common in PD than in other chronic and disabling disorders, occurring in approximately 40% of PD patients. However, the prevalence and clinical features associated with depression in PD remain controversial. Dementia is increasingly recognized as a symptom associated with idiopathic PD, and is found in up to 40% of all patients suffering from that condition. The aim of this study was to estimate the prevalence of depressive and dementia symptoms in PD patients. The study included 35 consecutive patients with PD, 13 (37.4%) male and 22 (62.6%) female (mean age 62.9 ± 11.0, range 36-85 years), mean duration of disease 4.7 ± 2.9 (range 1-10) years, hospitalized during one year at Clinical Department of Neurology, Tuzla University Clinical Center, Tuzla, Bosnia and Herzegovina. The Mini Mental State Examination (MMSE) was used for assessment of cognitive deterioration and Beck Depression Inventory (BDI) for depression. Computerized tomography was performed in all patients. According to BDI scale, depressive symptoms were present in all 35 PD patients: minimal in 4 (11.4%), low in 7 (20%), moderate in 8 (22.8%), severe in 9 (25.4%) and extreme in 7 (20%) patients. On MMSE scale, 9 (25.4%) patients were free from cognitive deterioration and 26 (74.6%) patients had moderate to severe deterioration, but 21 (60%) patients (7 (33.33%) male and 14 (66.66%) female) had symptoms of dementia (MMSE score ≤ 23). Using MMSE scale, 8 (22.8%) patients were free from dementia and 27 (77.2%) patients had some cognitive deterioration. Very mild symptoms of dementia were found in 6 (25.9%) and overt features of dementia in 21 (74.1%) PD patients. So, out of 35 PD study patients, 21 (60%) (7 (33.3%) male and 14

Parkinson's: a syndrome rather than a disease?

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Titova, Nataliya; Padmakumar, C; Lewis, Simon J G; Chaudhuri, K Ray

2017-08-01

Emerging concepts suggest that a multitude of pathology ranging from misfolding of alpha-synuclein to neuroinflammation, mitochondrial dysfunction, and neurotransmitter driven alteration of brain neuronal networks lead to a syndrome that is commonly known as Parkinson's disease. The complex underlying pathology which may involve degeneration of non-dopaminergic pathways leads to the expression of a range of non-motor symptoms from the prodromal stage of Parkinson's to the palliative stage. Non-motor clinical subtypes, cognitive and non-cognitive, have now been proposed paving the way for possible subtype specific and non-motor treatments, a key unmet need currently. Natural history of these subtypes remains unclear and need to be defined. In addition to in vivo biomarkers which suggest variable involvement of the cholinergic and noradrenergic patterns of the Parkinson syndrome, abnormal alpha-synuclein accumulation have now been demonstrated in the gut, pancreas, heart, salivary glands, and skin suggesting that Parkinson's is a multi-organ disorder. The Parkinson's phenotype is thus not just a dopaminergic motor syndrome, but a dysfunctional multi-neurotransmitter pathway driven central and peripheral nervous system disorder that possibly ought to be considered a syndrome and not a disease.

Clinical utility of FDG PET in Parkinson's disease and atypical parkinsonism associated with dementia.

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Walker, Zuzana; Gandolfo, Federica; Orini, Stefania; Garibotto, Valentina; Agosta, Federica; Arbizu, Javier; Bouwman, Femke; Drzezga, Alexander; Nestor, Peter; Boccardi, Marina; Altomare, Daniele; Festari, Cristina; Nobili, Flavio

2018-05-19

There are no comprehensive guidelines for the use of FDG PET in the following three clinical scenarios: (1) diagnostic work-up of patients with idiopathic Parkinson's disease (PD) at risk of future cognitive decline, (2) discriminating idiopathic PD from progressive supranuclear palsy, and (3) identifying the underlying neuropathology in corticobasal syndrome. We therefore performed three literature searches and evaluated the selected studies for quality of design, risk of bias, inconsistency, imprecision, indirectness and effect size. Critical outcomes were the sensitivity, specificity, accuracy, positive/negative predictive value, area under the receiving operating characteristic curve, and positive/negative likelihood ratio of FDG PET in detecting the target condition. Using the Delphi method, a panel of seven experts voted for or against the use of FDG PET based on published evidence and expert opinion. Of 91 studies selected from the three literature searches, only four included an adequate quantitative assessment of the performance of FDG PET. The majority of studies lacked robust methodology due to lack of critical outcomes, inadequate gold standard and no head-to-head comparison with an appropriate reference standard. The panel recommended the use of FDG PET for all three clinical scenarios based on nonquantitative evidence of clinical utility. Despite widespread use of FDG PET in clinical practice and extensive research, there is still very limited good quality evidence for the use of FDG PET. However, in the opinion of the majority of the panellists, FDG PET is a clinically useful imaging biomarker for idiopathic PD and atypical parkinsonism associated with dementia.

Neuropsychiatric symptoms and PET imaging characteristics in patients with Parkinson-plus syndromes

Directory of Open Access Journals (Sweden)

Miao ZHANG

2017-01-01

Full Text Available Objective To explore the neuropsychiatric symptoms and 18F-fluoro-2-deoxy-D-glucose (18F-FDG PET imaging features of Parkinson-plus syndromes. Methods There were 8 patients with probable Parkinson-plus syndromes, including one case of multiple system atrophy-cerebellar predominant (MSA-C, 4 cases of progressive supranuclear palsy (PSP, one case of corticobasal ganglionic degeneration (CBD and 2 cases of dementia with Lewy bodies (DLB. Mini-Mental State Examination (MMSE and Montreal Cognitive Assessment (MoCA were used to evaluate cognitive function, Neuropsychiatric Inventory (NPI was used to evaluate neuropsychiatric behaviors, and Hamilton Depression Rating Scale-21 Items (HAMD-21 was used to evaluate the emotional state of patients. Results One MSA-C patient showed only anxiety. Four PSP patients showed different degrees of cognitive disorders, of whom 3 cases also presented obvious depression, anxiety, irritability and sleep disorders. One case of CBD showed dysfunction in executive function, visual spatial ability, verbal function, attention and orientation, as well as depression, anxiety, irritability and sleep disorders. Two cases of DLB were found unable to copy pentagon in MMSE chart or draw a circle in Clock Drawing Test (CDT, and they also presented hallucination, depression and indifference. As for the result of 18F-FDG PET, one MSA-C patient showed cerebellarglucose hypometabolism; 4 PSP patients showed hypometabolism in bilateral symmetrical frontal lobes, anterior cingulate gyrus and parietal lobe, especially in thalamus, basal ganglia region and brain stem; one case of CBD showed hypometabolism in right lateral fronto-temporo-parieto-occipital lobes, left lateral parietal lobe, bilateral cingulate gyri and precuneus; 2 cases of DLB showed hypometabolism in bilateral temporo-occipital lobes. Conclusions Patients with early Parkinson-plus syndromes are easily misdiagnosed as mental illness and delayed treatment, in addition, their

Overset Wolff-Parkinson-White-syndrom

DEFF Research Database (Denmark)

Nielsen, Trine Skov; Dalager, Søren; Larsen, Maiken Kudahl

2010-01-01

An autopsy in a 28-year-old man did not explain the cause of sudden unexpected death. However, a history of episodes with tachycardia and dizziness and a reassessed previous electrocardiogram exhibiting ventricular pre-excitation was consistent with Wolff-Parkinson-White (WPW) syndrome. In this p......An autopsy in a 28-year-old man did not explain the cause of sudden unexpected death. However, a history of episodes with tachycardia and dizziness and a reassessed previous electrocardiogram exhibiting ventricular pre-excitation was consistent with Wolff-Parkinson-White (WPW) syndrome...

Wolff-Parkinson-white syndrome mimics a conduction disease.

Science.gov (United States)

Marrakchi, S; Kammoun, I; Kachboura, S

2014-01-01

Background. It is important to recognise Wolff-Parkinson-White (WPW) syndrome in electrocardiograms (ECG), as it may mimic ischaemic heart disease, ventricular hypertrophy, and bundle branch block. Recognising WPW syndrome allows for risk stratification, the identification of associated conditions, and the institution of appropriate management. Objective. The present case showed that electrophysiological study is indicated in patients with abnormal ECG and syncope. Case Report. A 40-year-old man with Wolff-Parkinson-White syndrome was presented to emergency with syncope. A baseline ECG was a complete right branch block and posterior left hemiblock. He was admitted to the cardiac care unit for pacemaker implantation. The atypical figure of complete right branch block and posterior left hemiblock was thought to be a "false positive" of conduction abnormality. But the long anterograde refractory period of the both accessory pathway and atrioventricular conduction may cause difficulty in diagnosing Wolff-Parkinson-White syndrome, Conclusion. A Wolff-Parkinson-White Syndrome may mimic a conduction disease. No reliable algorithm exists for making an ECG diagnosis of a preexcitation syndrome with conduction disorders. This can lead to diagnostic and therapeutic dilemmas in the context of syncope.

Wolff-Parkinson-White Syndrome Mimics a Conduction Disease

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S. Marrakchi

2014-01-01

Full Text Available Background. It is important to recognise Wolff-Parkinson-White (WPW syndrome in electrocardiograms (ECG, as it may mimic ischaemic heart disease, ventricular hypertrophy, and bundle branch block. Recognising WPW syndrome allows for risk stratification, the identification of associated conditions, and the institution of appropriate management. Objective. The present case showed that electrophysiological study is indicated in patients with abnormal ECG and syncope. Case Report. A 40-year-old man with Wolff-Parkinson-White syndrome was presented to emergency with syncope. A baseline ECG was a complete right branch block and posterior left hemiblock. He was admitted to the cardiac care unit for pacemaker implantation. The atypical figure of complete right branch block and posterior left hemiblock was thought to be a “false positive” of conduction abnormality. But the long anterograde refractory period of the both accessory pathway and atrioventricular conduction may cause difficulty in diagnosing Wolff-Parkinson-White syndrome, Conclusion. A Wolff-Parkinson-White Syndrome may mimic a conduction disease. No reliable algorithm exists for making an ECG diagnosis of a preexcitation syndrome with conduction disorders. This can lead to diagnostic and therapeutic dilemmas in the context of syncope.

New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration

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Hee Kyung Park

2013-04-01

Full Text Available Frontotemporal dementia (FTD is the second most common type of presenile dementia. Three clinical prototypes have been defined; behavioral variant FTD, semantic dementia, and progressive nonfluent aphasia. Progressive supranuclear palsy, corticobasal degeneration, and motor neuron disease may possess clinical and pathological characteristics that overlap with FTD, and it is possible that they may all belong to the same clinicopathological spectrum. Frontotemporal lobar degeneration (FTLD is a clinicopathological syndrome that encompasses a heterogenous group of neurodegenerative disorders. Owing to the advancement in the field of molecular genetics, diagnostic imaging, and pathology, FTLD has been the focus of great interest. Nevertheless, parkinsonism in FTLD has received relatively less attention. Parkinsonism is found in approximately 20–30% of patients in FTLD. Furthermore, parkinsonism can be seen in all FTLD subtypes, and some patients with familial and sporadic FTLD can present with prominent parkinsonism. Therefore, there is a need to understand parkinsonism in FTLD in order to obtain a better understanding of the disease. With regard to the clinical characteristics, the akinetic rigid type of parkinsonism has predominantly been described. Parkinsonism is frequently observed in familial FTD, more specifically, in FTD with parkinsonism linked to chromosome 17q (FTDP-17. The genes associated with parkinsonism are microtubule associated protein tau (MAPT, progranulin (GRN or PGRN, and chromosome 9 open reading frame 72 (C9ORF72 repeat expansion. The neural substrate of parkinsonism remains to be unveiled. Dopamine transporter (DAT imaging revealed decreased uptake of DAT, and imaging findings indicated atrophic changes of the basal ganglia. Parkinsonism can be an important feature in FTLD and, therefore, increased attention is needed on the subject.

Association of restless legs syndrome, pain, and mood disorders in Parkinson's disease.

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Rana, Abdul Qayyum; Qureshi, Abdul Rehman M; Rahman, Labiba; Jesudasan, Ajantha; Hafez, Kevin K; Rana, Mohammad A

2016-01-01

The objectives of the study were to analyze the association between Parkinson's disease and restless legs syndrome, and to explore the relationship between mood disorder comorbidity (anxiety and depression), pain, and restless legs syndrome. This study included 123 Parkinson's disease patients and 123 non-Parkinson's disease patients matched for age and gender, and evaluated for anxiety severity, depression severity, pain severity, pain interference, pain disability, and restless legs syndrome prevalence. This was performed using semi-structured interviews and a neurological examination through the restless legs syndrome diagnostic criteria and the following inventories; Hospital Anxiety and Depression Scale, Brief Pain Inventory, and Pain Disability Index. Parkinson's disease patients had significantly greater anxiety severity, depression severity, pain severity, pain interference, pain disability, and restless legs syndrome prevalence in comparison to controls. In addition, Parkinson's disease patients' comorbid for anxiety and depression had significantly greater pain severity, pain interference, and pain disability, but not RLS prevalence, in comparison to Parkinson's disease only, Parkinson's disease anxiety, and Parkinson's disease depression patients. Pain interference, pain severity, and pain disability is greater among Parkinson's disease patients with anxiety and depression, in comparison to Parkinson's disease patients without anxiety and depression. On the contrary, the prevalence of restless legs syndrome was not found to be relevant.

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 between...

Phenomenology and management of cognitive and behavioral disorders in Parkinson's disease. Rise and logic of dementia in Parkinson's disease

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Papageorgiou Sokratis

2006-08-01

Full Text Available Abstract An overview of studies on the issue of dementia in Parkinson's disease shows that, over time, there has been an evolution in the perception of the magnitude of the problem and of its nature. Dementia seems today to be part of the disease. This change in the understanding of the disease can be accounted for by various methodological problems and by difficulties, on one hand, in the definition of dementia and its differentiation from other conditions, and, on the other hand, in the diagnosis of the disease itself in individual cases. Optimal therapeutic strategies are also examined, either based on cholinesterase inhibitors or antiparkinsonian drugs and symptomatic measures.

Cognitive and motor symptoms in dementia: focus on dementia with Lewy bodies.

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Lingler, Jennifer Hagerty; Kaufer, Daniel I

2002-09-01

To describe the clinical syndrome called dementia with Lewy bodies (DLB) and highlight its common and unique characteristics with respect to diagnosis and management. Review of the scientific literature including psychiatric literature, reports of clinical trials, and clinical practice guidelines. DLB is a clinical and histopathologic disease, which is second only to Alzheimer's disease (AD) as a cause of dementia in older adults. The clinical syndrome of DLB includes cognitive and motor deterioration reminiscent of symptoms associated with AD and Parkinson's disease (PD) respectively. The late life intersection of cognitive and motor symptoms can present significant challenges in the primary care setting. Recognizing key features of common neurodegenerative disorders is essential to accurately diagnosing and appropriately treating the growing population of older adults who suffer from AD, PD, and DLB.

Can loss of the swallow tail sign help distinguish between Parkinson Disease and the Parkinson-Plus syndromes?

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Oustwani, Christopher Sami; Korutz, Alexander William; Lester, Malisa Siri; Kianirad, Yasaman; Simuni, Tanya; Hijaz, Tarek Aref

To determine if loss of the swallow tail sign (STS) can distinguish Parkinson Disease (PD) from the Parkinson-Plus syndromes. Twenty-five patients with PD, 21 with Parkinson-Plus syndromes, and 14 control patients were included. Presence of the STS was assessed. The STS was present in 79% of controls, statistically greater than the PD/Parkinson-Plus patients. There was no difference in the presence of the STS between the PD/Parkinson-Plus subgroups or when scanning at 1.5 T or 3 T. Loss of the STS could not distinguish between PD and Parkinson-Plus patients. The STS can be identified at both 1.5 T and 3 T. Copyright © 2017 Elsevier Inc. All rights reserved.

[The prevalence of Parkinson's disease, associated dementia, and depression in Dresden].

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Riedel, O; Schneider, C; Klotsche, J; Reichmann, H; Storch, A; Wittchen, H-U

2013-02-01

Parkinson's disease (PD) is frequently compounded by dementia and depression. Yet local total estimates on the prevalence of PD with dementia/depression are still lacking. These are socioeconomically important, especially for the eastern federal states in Germany due to the demographic structures. We conducted a two-staged total estimation in the area of Dresden. First, all local office-based neurologists, hospitals and retirement homes were asked to list their patients/residents with PD on a single study day. Then a random sample of patients/home residents was neuropsycholoigcally examined, including the Mini-mental-state exam and the Montgomery-Asberg Depression rating scale. Dementia was diagnosed according to DSM-IV criteria. Overall, 886 PD cases (95 % CI: 809 - 926) were estimated, of which 252 (95 % CI: 226 - 279) suffered from dementia and 216 (95 % CI: 191 - 242) from depression. Dementia rates increased by age with 13.8 % (≤ 65 years) to 40.2 % (≥ 76 years). Depression rates ranged from 23.3 % to 28.0 %. Overall, 20.6 % of all ambulatory treated PD patients and 85.7 % of all home residents with PD had dementia. The prevalence of PD in Dresden dovetails with previous reported estimates. Dementia and depression are frequent complications in outpatients as well as home residents with PD. © Georg Thieme Verlag KG Stuttgart · New York.

The impact of autonomic dysfunction on survival in patients with dementia with Lewy bodies and Parkinson's disease with dementia.

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Kajsa Stubendorff

Full Text Available INTRODUCTION: Autonomic dysfunction is a well-known feature in neurodegenerative dementias, especially common in α-synucleinopathies like dementia with Lewy bodies and Parkinson's disease with dementia. The most common symptoms are orthostatic hypotension, incontinence and constipation, but its relevance in clinical practice is poorly understood. There are no earlier studies addressing the influence of autonomic dysfunction on clinical course and survival. The aim of this study was to investigate the frequency of the three most common features of autonomic dysfunction and analyze how it affects survival. METHODS: Thirty patients with dementia with Lewy bodies and Parkinson's disease with dementia were included in this prospective, longitudinal follow-up study. Presence of incontinence and constipation was recorded at baseline. Blood pressure was measured at baseline, after 3 months and after 6 months according to standardized procedures, with 5 measurements during 10 minutes after rising. Orthostatic hypotension was defined using consensus definitions and persistent orthostatic hypotension was defined as 5 or more measurements with orthostatic hypotension. Difference in survival was analyzed 36 months after baseline. RESULTS: There was a high frequency of persistent orthostatic blood pressure (50%, constipation (30% and incontinence (30%. Patients with persistent orthostatic hypotension had a significantly shorter survival compared to those with no or non-persistent orthostatic hypotension (Log rank x(2 = 4.47, p = 0.034. Patients with constipation and/or urinary incontinence, in addition to persistent orthostatic hypotension, had a poorer prognosis compared to those with isolated persistent orthostatic hypotension or no orthostatic hypotension (Log rank x(2 = 6.370, p = 0.041. DISCUSSION: According to our findings, the identification of autonomic dysfunction seems to be of great importance in clinical practice, not only to

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Full Text Available ... Gene and Cell Therapies Expert Briefings: Dealing with Dementia in PD Expert Briefings: Anxiety in Parkinson's Disease ... Lobo: Música en vivo con Lobo! Dealing with Dementia Hallucinations and Parkinson's with Dr. Friedman CareMAP: Putting ...

Wolff-Parkinson-White syndrome in infants.

Science.gov (United States)

Hermosura, Tisha; Bradshaw, Wanda T

2010-01-01

Wolff-Parkinson-White (WPW) syndrome is a ventricular preexcitation that presents as supraventricular tachycardia. Health care professionals can attain optimal results in caring for infants with WPW syndrome by understanding both its pathophysiology and proper management to prevent and treat complications associated with it. This article reviews the prevalence, pathophysiology, clinical manifestations, diagnostic modalities, assessment, and management of WPW syndrome.

Capgras' syndrome in dementia with Lewy bodies.

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Marantz, Andrew G; Verghese, Joe

2002-01-01

We report the occurrence of Capgras' syndrome, or the delusion of doubles, in a patient with dementia with Lewy bodies. The patient believed that several similar-looking impostors had replaced his wife of over 50 years. Uncharacteristically, he adopted a friendly attitude with these impostors. This unusual convivial reaction to the impostors may result from differential involvement of the dual visual pathways processing facial recognition and emotional responses to faces. The delusion resolved spontaneously, coincident with worsening of the dementia. In a retrospective chart review of 18 autopsy-confirmed cases of dementia with Lewy bodies, delusions were reported in 5 subjects (27.8%), of whom 1 had misidentification delusions much like Capgras' syndrome.

Mini-mental Parkinson (MMP) as a dementia screening test: comparison with the Mini-Mental State Examination (MMSE).

Science.gov (United States)

Larner, Andrew J

2012-07-01

As populations age, screening instruments for cognitive impairment and dementia will become of increasing importance in clinical practice. Mini-Mental Parkinson (MMP), a derivative of the Mini-Mental State Examination (MMSE), was originally described as a cognitive screening instrument for use in Parkinson's disease. Its item content addresses some of the acknowledged shortcomings of the MMSE. Pragmatic use of MMP in general cognitive clinics has not previously been examined. To compare the performance of two scales, Mini-Mental Parkinson (MMP) and the Mini-Mental State Examination (MMSE), as cognitive screening instruments for dementia in a memory clinic population. MMP was administered prospectively to 201 consecutive new patient referrals independent of other tests used to establish dementia diagnosis according to standard diagnostic criteria (DSM-IV). Diagnostic utility of MMP for dementia was measured and compared with MMSE. MMP proved easy to use and acceptable to patients. Optimal test accuracy (0.86) was at MMP cutoff of ≤ 17/32, with sensitivity 0.51, specificity 0.97, positive predictive value 0.83, negative predictive value 0.87, and area under Receiver Operating Characteristic curve 0.89. Using a higher cutoff (≤ 29/32), MMP sensitivity was 1.00 with specificity 0.70. MMP scores correlated with MMSE (r = 0.93) and diagnostic agreement was high (κ = 0.85). MMP is a useful screening instrument in the memory clinic setting, with patients who fall below the designated cutoff requiring further investigation to ascertain a cause for their cognitive impairment.

Brain Connectivity Alterations Are Associated with the Development of Dementia in Parkinson's Disease.

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Bertrand, Josie-Anne; McIntosh, Anthony R; Postuma, Ronald B; Kovacevic, Natasha; Latreille, Véronique; Panisset, Michel; Chouinard, Sylvain; Gagnon, Jean-François

2016-04-01

Dementia affects a high proportion of Parkinson's disease (PD) patients and poses a burden on caregivers and healthcare services. Electroencephalography (EEG) is a common nonevasive and nonexpensive technique that can easily be used in clinical settings to identify brain functional abnormalities. Only few studies had identified EEG abnormalities that can predict PD patients at higher risk for dementia. Brain connectivity EEG measures, such as multiscale entropy (MSE) and phase-locking value (PLV) analyses, may be more informative and sensitive to brain alterations leading to dementia than previously used methods. This study followed 62 dementia-free PD patients for a mean of 3.4 years to identify cerebral alterations that are associated with dementia. Baseline resting state EEG of patients who developed dementia (N = 18) was compared to those of patients who remained dementia-free (N = 44) and of 37 healthy subjects. MSE and PLV analyses were performed. Partial least squares statistical analysis revealed group differences associated with the development of dementia. Patients who developed dementia showed higher signal complexity and lower PLVs in low frequencies (mainly in delta frequency) than patients who remained dementia-free and controls. Conversely, both patient groups showed lower signal variability and higher PLVs in high frequencies (mainly in gamma frequency) compared to controls, with the strongest effect in patients who developed dementia. These findings suggest that specific disruptions of brain communication can be measured before PD patients develop dementia, providing a new potential marker to identify patients at highest risk of developing dementia and who are the best candidates for neuroprotective trials.

Demography, diagnostics, and medication in dementia with Lewy bodies and Parkinson's disease with dementia: data from the Swedish Dementia Quality Registry (SveDem

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Fereshtehnejad SM

2013-06-01

Full Text Available Seyed-Mohammad Fereshtehnejad,1 Dorota Religa,2,3 Eric Westman,1 Dag Aarsland,2,4 Johan Lökk,1,3 Maria Eriksdotter1,3 1Division of Clinical Geriatrics, Department of Neurobiology, Care Sciences, and Society (NVS, Karolinska Institutet, Stockholm, Sweden; 2Alzheimer's Disease Research Center, Department of Neurobiology, Care Sciences and Society (NVS, Karolinska Institutet, Stockholm, Sweden; 3Department of Geriatric Medicine, Karolinska University Hospital, Stockholm, Sweden; 4Centre for Age-Related Diseases, Stavanger University Hospital, Stavanger, Norway Introduction: Whether dementia with Lewy bodies (DLB and Parkinson's disease with dementia (PDD should be considered as one entity or two distinct conditions is a matter of controversy. The aim of this study was to compare the characteristics of DLB and PDD patients using data from the Swedish Dementia Quality Registry (SveDem. Methods: SveDem is a national Web-based quality registry initiated to improve the quality of diagnostic workup, treatment, and care of patients with dementia across Sweden. Patients with newly diagnosed dementia of various types were registered in SveDem during the years 2007–2011. The current cross-sectional report is based on DLB (n = 487 and PDD (n = 297 patients. Demographic characteristics, diagnostic workup, Mini-Mental State Examination (MMSE score, and medications were compared between DLB and PDD groups. Results: No gender differences were observed between the two study groups (P = 0.706. PDD patients were significantly younger than DLB patients at the time of diagnosis (74.8 versus 76.8 years, respectively; P < 0.001. A significantly higher prevalence of patients with MMSE score #24 were found in the PDD group (75.2% versus 67.6%; P = 0.030. The mean number of performed diagnostic modalities was significantly higher in the DLB group (4.9 ± 1.7 than in the PDD group (4.1 ± 1.6; P< 0.001. DLB patients were more likely than PDD patients to be treated with

Overset Wolff-Parkinson-White-syndrom

DEFF Research Database (Denmark)

Nielsen, Trine Skov; Dalager, Søren; Larsen, Maiken Kudahl

2010-01-01

An autopsy in a 28-year-old man did not explain the cause of sudden unexpected death. However, a history of episodes with tachycardia and dizziness and a reassessed previous electrocardiogram exhibiting ventricular pre-excitation was consistent with Wolff-Parkinson-White (WPW) syndrome...

Episodic Memory in Alzheimer Disease, Frontotemporal Dementia, and Dementia With Lewy Bodies/Parkinson Disease Dementia: Disentangling Retrieval From Consolidation.

Science.gov (United States)

Economou, Alexandra; Routsis, Christopher; Papageorgiou, Sokratis G

2016-01-01

Differences in episodic memory performance in patients with Alzheimer disease (AD), frontotemporal dementia (FTD), dementia with Lewy bodies (DLB)/Parkinson disease with dementia (PDD) are inconsistent and task dependent. The inconsistencies may be attributed to the different tasks drawing on different memory processes. Few studies have examined episodic memory impairment in the above groups using memory tests that facilitate encoding, to distinguish memory deficits due to impairment of specific processes. We examined the memory performance of 106 AD patients, 51 FTD patients, 26 DLB/PDD patients, and 37 controls using the Five-Words Test, a 5-item memory test that facilitates encoding. The patient groups did not differ in modified Mini Mental State Examination scores. AD patients scored lowest on the Five-Words Test overall, and showed the greatest reduction from immediate total recall to delayed free recall relative to the other 2 groups, consistent with a predominantly consolidation deficit. DLB/PDD patients showed the largest improvement from delayed free to delayed total recall relative to the other 2 groups, consistent with a predominantly retrieval deficit. Deficits in both consolidation and retrieval underlie the memory impairment of the patients, to different extents, and contribute to the theoretical understanding of the nature of the memory impairment of the patient groups.

Lewy body dementias

DEFF Research Database (Denmark)

Løkkegaard, Annemette; Korbo, Lise

2017-01-01

Dementia with Lewy bodies and Parkinson disease dementia share the same pathophysiology. Together they are called Lewy body dementias and are the second most common type of dementia. Lewy body dementias receive little attention, and patients are often misdiagnosed, leading to less than ideal...

Pilot study of a three-step diagnostic pathway for young and old patients with Parkinson's disease dementia: screen, test and then diagnose.

NARCIS (Netherlands)

Robben, S.H.M.; Sleegers, M.J.; Dautzenberg, P.LJ.; Bergen, F.S. van; Bruggen, J.P.; Olde Rikkert, M.G.M.

2010-01-01

OBJECTIVE: To pilot a three-step diagnostic model for young and old patients with Parkinson's disease dementia (PDD). METHODS: Prospective investigator-blinded study. We developed a screening questionnaire for patients with Parkinson's disease (PD) and their caregivers. Further, patients were

Apathy in patients with Parkinson disease without dementia or depression: a PET study.

Science.gov (United States)

Robert, Gabriel; Le Jeune, Florence; Lozachmeur, Clément; Drapier, Sophie; Dondaine, Thibault; Péron, Julie; Travers, David; Sauleau, Paul; Millet, Bruno; Vérin, Marc; Drapier, Dominique

2012-09-11

We sought to identify apathy metabolic bases in Parkinson disease (PD). A total of 45 patients with PD who were not clinically depressed (Montgomery-Åsberg Depression Rating Scale [MADRS] dementia (Mattis Dementia Rating Scale [MDRS] >130) were assessed with the Apathy Evaluation Scale (AES) and underwent a resting-state F-18 fluorodeoxyglucose PET (FDG-PET) scan. A motor assessment comprising the Unified Parkinson's Disease Rating Scale Part III (UPDRS-III) was conducted and total levodopa equivalent daily dose (LEDD) was calculated. Imaging data were analyzed with statistical parametric mapping. Age, LEDD, and MDRS scores were introduced as covariates. Positive correlations were observed between the AES score and cerebral metabolism in the right inferior frontal gyrus (Brodmann area [BA] 47), right middle frontal gyrus (BA 10), right cuneus (BA 18), and right anterior insula (BA 13). Negative correlations were observed between the AES score and cerebellar metabolism in the semilunar lobules bilaterally, within the posterior lobe. Using an AES score equal to or above 42 to define clinical apathy, prevalence in our patient group was 17.8%. The AES score was negatively correlated with the MDRS score and positively correlated with the "retardation" subscore of the MADRS. It was not correlated with either UPDRS III or LEDD. Results indicate that the frontal, temporal, and cerebellar areas known to be involved in reward, emotion, and cognition are also implicated in apathy in patients with PD without dementia or depression. Their roles in the etiopathology of apathy are discussed.

Depression and synaptic zinc regulation in Alzheimer disease, dementia with lewy bodies, and Parkinson disease dementia.

Science.gov (United States)

Whitfield, David R; Vallortigara, Julie; Alghamdi, Amani; Hortobágyi, Tibor; Ballard, Clive; Thomas, Alan J; O'Brien, John T; Aarsland, Dag; Francis, Paul T

2015-02-01

Depression is a common symptom in dementia with Lewy bodies (DLB), Parkinson disease dementia (PDD), and Alzheimer disease (AD), yet its molecular basis remains unclear and current antidepressants do not appear to be effective. Cerebral zinc has been implicated in depression and synaptic dysfunction. We investigated the relationship between synaptic zinc regulation (for which zinc transporter 3 [ZnT3] is responsible) and depression in a large clinicopathologic study. We examined brains from people with PDD (N = 29), DLB (N = 27), and AD (N = 15) and comparison subjects without depression or dementia (N = 24). Individuals were categorized according to the presence and severity of depression (on a scale of 0-3) based on standardized assessments during life (principally Neuropsychiatric Inventory). Western blotting was used to determine ZnT3 levels in Brodmann area 9 (BA9), and regression analysis was used to determine the relationship between ZnT3 and depression. Reductions in ZnT3 in BA9 were significantly associated with elevated depression scores in the study cohort (β = -0.351, df = 93, t = -3.318 p = 0.0004). This association remained when only individuals with DLB, PDD, and no dementia or depression were examined (β = -0.347, df = 78, t = -3.271, p = 0.002) or only individuals with AD and no dementia or depression were examined (β = -0.433, df = 37, t = -2.924, p = 0.006). Although decreased zinc levels have been implicated in the genesis of depression in animal models and in major depressive disorder in humans, this study provides the first evidence of a role for zinc in depression in people with dementia and highlights zinc metabolism as a therapeutic target. Copyright © 2015 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.

I-123 IMP SPECT in Parkinson's disease

International Nuclear Information System (INIS)

Kawabata, Keita; Tachibana, Kyudai; Sugita, Minoru

1990-01-01

To examine semiquantitatively regional cerebral blood flow, SPECT with N-isopropyl-p-[I-123]iodoamphetamine (I-123 IMP) was undertaken in 17 patients with Parkinson's disease. Seven patients with Alzheimer's disease and 9 senile control subjects were also imaged for comparison. Both the Parkinson's disease group and the Alzheimer's disease group had a decreased uptake of I-123 IMP in the frontal lobe, in comparison with the control group. A remarkably decreased uptake was seen in the lateral and parietal lobes in the group of Parkinson's disease associated with dementia, as well as in the Alzheimer's disease group. A significantly decreased uptake was observed in the frontal lobe, lateral lobe, thalamus, and basal ganglia in the Parkinson's disease group, irrespective of the presence or absence of dementia. For Parkinson's disease associated with dementia, there was much more significant decrease in I-123 IMP uptake. The pattern of regional cerebral blood flow in the Alzheimer's disease group was analogous to that in the Parkinson's disease group associated with dementia. This supports the hypothesis that Alzheimer's disease may be somewhat involved in the occurrence of dementia for Parkinson's disease. (N.K.)

Addenbrooke's Cognitive Examination-Revised is accurate for detecting dementia in Parkinson's disease patients with low educational level.

Science.gov (United States)

Rocha, Maria Sheila Guimarães; Bassetti, Elida Maria; Oliveira, Maira Okada; Kuark, Roberta Gomes Borges; Estevam, Nathercia Marinho; Brucki, Sonia Maria Dozzi

2014-01-01

Diagnosis of Parkinson's disease dementia is a challenge in clinical settings. A comprehensive neuropsychological evaluation is time-consuming and expensive; brief instruments for cognitive evaluation must be easier to administer and provide a reliable classification. To study the validity of the Brazilian version of Addenbrooke's Cognitive Examination-Revised (ACE-R) for the cognitive assessment of Parkinson's disease (PD) patients with heterogeneous educational level. Patients were evaluated according to the diagnostic procedures recommended by the Movement Disorder Society (MDS) as the gold standard for the diagnosis of dementia in PD. We studied 70 idiopathic PD patients, with a mean (SD) age of 64.1 (9.3) years and mean disease duration of 7.7 (5.3) years and educational level of 5.9 years, matched for education and age to controls. Twenty-seven patients fulfilled MDS clinical criteria for PD dementia. Mean scores on the ACE-R were 54.7 (12.8) points for patients with PD dementia, 76 (9.9) for PD patients without dementia and 79.7 (1.8) points for healthy controls. The area under the receiver operating curve, taking the MDS diagnostic procedures as a reference, was 0.93 [95% CI, 0.87-0.98; p<0.001] for ACE-R. The optimal cut-off value for ACE-R was ≤72 points [sensitivity 90%; specificity 85%; Kappa concordance (K) 0.79]. ACE-R appears to be a valid tool for dementia evaluation in PD patients with heterogeneous educational level, displaying good correlation with clinical criteria and diagnostic procedures of the MDS.

Addenbrooke's Cognitive Examination-Revised is accurate for detecting dementia in Parkinson's disease patients with low educational level

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Maria Sheila Guimarães Rocha

Full Text Available ABSTRACT Diagnosis of Parkinson's disease dementia is a challenge in clinical settings. A comprehensive neuropsychological evaluation is time-consuming and expensive; brief instruments for cognitive evaluation must be easier to administer and provide a reliable classification. Objective: To study the validity of the Brazilian version of Addenbrooke's Cognitive Examination-Revised (ACE-R for the cognitive assessment of Parkinson's disease (PD patients with heterogeneous educational level. Methods: Patients were evaluated according to the diagnostic procedures recommended by the Movement Disorder Society (MDS as the gold standard for the diagnosis of dementia in PD. Results: We studied 70 idiopathic PD patients, with a mean (SD age of 64.1 (9.3 years and mean disease duration of 7.7 (5.3 years and educational level of 5.9 years, matched for education and age to controls. Twenty-seven patients fulfilled MDS clinical criteria for PD dementia. Mean scores on the ACE-R were 54.7 (12.8 points for patients with PD dementia, 76 (9.9 for PD patients without dementia and 79.7 (1.8 points for healthy controls. The area under the receiver operating curve, taking the MDS diagnostic procedures as a reference, was 0.93 [95% CI, 0.87-0.98; p<0.001] for ACE-R. The optimal cut-off value for ACE-R was ≤72 points [sensitivity 90%; specificity 85%; Kappa concordance (K 0.79]. Conclusion: ACE-R appears to be a valid tool for dementia evaluation in PD patients with heterogeneous educational level, displaying good correlation with clinical criteria and diagnostic procedures of the MDS.

Italian version of the Parkinson Neuropsychometric Dementia Assessment (PANDA): a useful instrument to detect cognitive impairments in Parkinson's Disease.

Science.gov (United States)

Pignatti, Riccardo; Bertella, Laura; Scarpina, Federica; Mauro, Alessandro; Portolani, Elisa; Calabrese, Pasquale

2014-01-01

Parkinson's disease (PD) is frequently characterized by cognitive and affective dysfunctions. The "Parkinson Neuropsychometric Dementia Assessment" (PANDA) is a screening tool designed for the early detection of mild cognitive impairment as well as dementia in PD. The PANDA is already validated in German and in French. The aim of the present work was to provide normative data for the Italian-speaking population, Swiss regions included; moreover, the effectiveness of the PANDA compared to the Mini Mental State Examination (MMSE) was tested. One-hundred and eleven PD patients with and without cognitive impairment and one-hundred and three matched healthy subjects participated at this study; all patients underwent an extensive neuropsychological evaluation. A PANDA total score of 13 appeared to be the most fitting cut-off with a sensitivity of 96.6% and a specificity of 82.2%; with the MMSE, the same value of sensitivity but with a specificity of 72,4% was reached only by adopting a cut-off of 28. Moreover, a PANDA range of 13-17 appeared to be suggestive for possible cognitive disturbance. The present work provides evidence for the effectiveness of the PANDA in evaluating cognitive deficits also in PD Italian-speaking patients, even when their pathological degree is still initial or very mild.

Pleiotropic Effects of Variants in Dementia Genes in Parkinson Disease

Directory of Open Access Journals (Sweden)

Laura Ibanez

2018-04-01

Full Text Available Background: The prevalence of dementia in Parkinson disease (PD increases dramatically with advancing age, approaching 80% in patients who survive 20 years with the disease. Increasing evidence suggests clinical, pathological and genetic overlap between Alzheimer disease, dementia with Lewy bodies and frontotemporal dementia with PD. However, the contribution of the dementia-causing genes to PD risk, cognitive impairment and dementia in PD is not fully established.Objective: To assess the contribution of coding variants in Mendelian dementia-causing genes on the risk of developing PD and the effect on cognitive performance of PD patients.Methods: We analyzed the coding regions of the amyloid-beta precursor protein (APP, Presenilin 1 and 2 (PSEN1, PSEN2, and Granulin (GRN genes from 1,374 PD cases and 973 controls using pooled-DNA targeted sequence, human exome-chip and whole-exome sequencing (WES data by single variant and gene base (SKAT-O and burden tests analyses. Global cognitive function was assessed using the Mini-Mental State Examination (MMSE or the Montreal Cognitive Assessment (MoCA. The effect of coding variants in dementia-causing genes on cognitive performance was tested by multiple regression analysis adjusting for gender, disease duration, age at dementia assessment, study site and APOE carrier status.Results: Known AD pathogenic mutations in the PSEN1 (p.A79V and PSEN2 (p.V148I genes were found in 0.3% of all PD patients. There was a significant burden of rare, likely damaging variants in the GRN and PSEN1 genes in PD patients when compared with frequencies in the European population from the ExAC database. Multiple regression analysis revealed that PD patients carrying rare variants in the APP, PSEN1, PSEN2, and GRN genes exhibit lower cognitive tests scores than non-carrier PD patients (p = 2.0 × 10−4, independent of age at PD diagnosis, age at evaluation, APOE status or recruitment site.Conclusions: Pathogenic mutations in

Rivastigmine in Alzheimer's disease and Parkinson's disease dementia: an ADAS-cog factor analysis.

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Weintraub, Daniel; Somogyi, Monique; Meng, Xiangyi

2011-09-01

Rivastigmine treatment is associated with significant improvements on the cognitive subscale of the Alzheimer's Disease Assessment Scale (ADAS-cog) in patients with mild-to-moderate Alzheimer's disease (AD) and Parkinson's disease dementia (PDD). Both AD and PDD are purported to have different profiles of cognitive impairment, which may respond differentially to rivastigmine treatment. This was a retrospective analysis of 3 randomized, double-blind, rivastigmine trial databases (Investigation of transDermal Exelon in ALzheimer's disease [IDEAL; AD], EXelon in PaRkinson's disEaSe dementia Study [EXPRESS; PDD], and Alzheimer's Disease with ENA 713 [ADENA; AD]). Factor analyses of the 11 baseline ADAS-cog items derived the same factors in the 2 diseases, that is, "memory" and "language". Rivastigmine-treated AD and PDD patients showed significant improvements (P < .0001 versus placebo) on both factors. For both AD and PDD, rivastigmine had a numerically greater effect on memory than language. Treatment effect sizes were numerically greater in PDD compared with AD. Rivastigmine treatment is associated with improvement in memory and language in AD and PDD. The numerically greater response in PDD is consistent with greater cholinergic deficits in this disease state.

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Full Text Available ... Briefings: Dealing with Dementia in PD Expert Briefings: Anxiety in Parkinson's Disease Expert Briefings: Nutrition and Parkinson's ... and Tomorrow Expert Briefings: A Closer Look at Anxiety and Depression in Parkinson's Disease Expert Briefings: Driving ...

The characteristics of autonomic nervous system disorders in burning mouth syndrome and Parkinson disease.

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Koszewicz, Magdalena; Mendak, Magdalena; Konopka, Tomasz; Koziorowska-Gawron, Ewa; Budrewicz, Sławomir

2012-01-01

To conduct a clinical electrophysiologic evaluation of autonomic nervous system functions in patients with burning mouth syndrome and Parkinson disease and estimate the type and intensity of the autonomic dysfunction. The study involved 83 subjects-33 with burning mouth syndrome, 20 with Parkinson disease, and 30 controls. The BMS group included 27 women and 6 men (median age, 60.0 years), and the Parkinson disease group included 15 women and 5 men (median age, 66.5 years). In the control group, there were 20 women and 10 men (median age, 59.0 years). All patients were subjected to autonomic nervous system testing. In addition to the Low autonomic disorder questionnaire, heart rate variability (HRV), deep breathing (exhalation/inspiration [E/I] ratio), and sympathetic skin response (SSR) tests were performed in all cases. Parametric and nonparametric tests (ANOVA, Kruskal-Wallis, and Scheffe tests) were used in the statistical analysis. The mean values for HRV and E/I ratios were significantly lower in the burning mouth syndrome and Parkinson disease groups. Significant prolongation of SSR latency in the foot was revealed in both burning mouth syndrome and Parkinson disease patients, and lowering of the SSR amplitude occurred in only the Parkinson disease group. The autonomic questionnaire score was significantly higher in burning mouth syndrome and Parkinson disease patients than in the control subjects, with the Parkinson disease group having the highest scores. In patients with burning mouth syndrome, a significant impairment of both the sympathetic and parasympathetic nervous systems was found but sympathetic/parasympathetic balance was preserved. The incidence and intensity of autonomic nervous system dysfunction was similar in patients with burning mouth syndrome and Parkinson disease, which may suggest some similarity in their pathogeneses.

Limbic system, the main focus of dementia syndrome; A study with MRI and PET

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Matsuzawa, Taiju [Morinosato Hospital, Atsugi, Kanagawa (Japan)

1990-12-01

Alzheimer disease and multi-infarct dementia are two entirely different diseases producing almost the same abnormalities as dementia syndrome. The statistical studies with MRI to locate the focus of dementia syndrome in the neocortex was an absolute failure. With MRI there is drastic atrophy and destruction of the amygdala and hippocampus suggesting the limbic system as the focus of dementia syndrome. Destruction of the limbic system in particular amygdala and hippocampus produced the functional obstruction brought about by the marked reduction in the glucose utilization with PET in the bilateral temporal, parietal and occipital association cortices. Although this type constitutes only about 1/5 of all dementia patients. It is considered the fundamental type of dementia syndrome. Aside from this, there is a type wherein simultaneous and symmetrical reductions in glucose utilization of the frontal association cortex and the motor association cortex in the anterior part of the neocortex. This is referred to as type II. It constitutes about 4/5 of all dementia patients which is far more than type I. Based on these results, it is thought that limbic system is the main focus of dementia syndrome. (author).

Goal-orientated cognitive rehabilitation for dementias associated with Parkinson's disease-A pilot randomised controlled trial.

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Hindle, John V; Watermeyer, Tamlyn J; Roberts, Julie; Brand, Andrew; Hoare, Zoe; Martyr, Anthony; Clare, Linda

2018-05-01

To examine the appropriateness and feasibility of cognitive rehabilitation for people with dementias associated with Parkinson's in a pilot randomised controlled study. This was a single-blind pilot randomised controlled trial of goal-oriented cognitive rehabilitation for dementias associated with Parkinson's. After goal setting, participants were randomised to cognitive rehabilitation (n = 10), relaxation therapy (n = 10), or treatment-as-usual (n = 9). Primary outcomes were ratings of goal attainment and satisfaction with goal attainment. Secondary outcomes included quality of life, mood, cognition, health status, everyday functioning, and carers' ratings of goal attainment and their own quality of life and stress levels. Assessments were at 2 and 6 months following randomisation. At 2 months, cognitive rehabilitation was superior to treatment-as-usual and relaxation therapy for the primary outcomes of self-rated goal attainment (d = 1.63 and d = 1.82, respectively) and self-rated satisfaction with goal attainment (d = 2.04 and d = 1.84). At 6 months, cognitive rehabilitation remained superior to treatment-as-usual (d = 1.36) and relaxation therapy (d = 1.77) for self-rated goal attainment. Cognitive rehabilitation was superior to treatment as usual and/or relaxation therapy in a number of secondary outcomes at 2 months (mood, self-efficacy, social domain of quality of life, carers' ratings of participants' goal attainment) and at 6 months (delayed recall, health status, quality of life, carer ratings of participants' goal attainment). Carers receiving cognitive rehabilitation reported better quality of life, health status, and lower stress than those allocated to treatment-as-usual. Cognitive rehabilitation is feasible and potentially effective for dementias associated with Parkinson's disease. Copyright © 2018 John Wiley & Sons, Ltd.

Demência na doença de Parkinson: avaliação crítica da literatura Dementia in Parkinson's disease: a critical review of literature

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Luciana de Abreu e Lima Pamplona

1996-12-01

momento, não reconhece agentes eficazes.In the last 30 years, Parkinson's disease has been object of great progress. The majority of patients reaches a longer life with quality because of the modern therapeutic approach. However, dementia that can occur in the evolutive process, has its neuropathology not completelly defined until now. There are lesions in the basal ganglia, in the ventral area of the mesencephalic tegmentum, in the thalamus, in the substantia nigra and in the frontal cortex. The presence of Lewy bodies in the cortex is associated with dementia, in the same way that the anatomopathological features of Alzheimer's disease, in many cases. Dementia should have a multifactorial basis. Different types of neurotransmitters, like serotonin, acetylcholine and dopamine, or even hormones, like Cortisol, may be altered in a great number of demented parkinsonians. Depression, found in up to 40% of patients, have been related as a risk factor for dementia, present approximatelly in 25% of cases. Studies in this area are still conflicting, with some confirming the relation among depression, cortical atrophy, hypercortisolemia and Parkinson's disease. Neuropsychologic studies show that the dementia in Parkinson's disease is of subcortical type. It is also known that parkinsonians, even those without cognitive deficiencies clinically significant, present deficits if submitted to more detailed neuropsychological tests. It is assumed, so, that cognitive impairments are intrinsic to the disease, varying its expression among patients. Dementia shall be diagnosed based on the criteria established in the diagnostic and statistical manual of mental disorders of the American Psychiatry Association, as well as computed tomography and magnetic resonance. For treatment, parkinsonian dementia does not recognize efficacious agents until now.

The Distinct Cognitive Syndromes of Parkinson's Disease: 5 Year Follow-Up of the CamPaIGN Cohort

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Williams-Gray, Caroline H.; Evans, Jonathan R.; Goris, An; Foltynie, Thomas; Ban, Maria; Robbins, Trevor W.; Brayne, Carol; Kolachana, Bhaskar S.; Weinberger, Daniel R.; Sawcer, Stephen J.; Barker, Roger A.

2009-01-01

Cognitive abnormalities are common in Parkinson's disease, with important social and economic implications. Factors influencing their evolution remain unclear but are crucial to the development of targeted therapeutic strategies. We have investigated the development of cognitive impairment and dementia in Parkinson's disease using a longitudinal…

Malignant vasovagal syndrome in two patients with Wolff-Parkinson-White syndrome

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Gandhi, N M; Bennett, D H

2004-01-01

The presence of Wolff-Parkinson-White (WPW) syndrome in patients presenting with syncope suggests that tachyarrhythmia may be the cause. However, the symptoms require careful evaluation. Two young patients presented with syncope and were found to have WPW syndrome on their ECG. In both patients symptoms were suggestive of vasovagal syncope. During tilt testing, both the patients developed their typical symptoms with a fall in blood pressure and heart rate confirming the diagnosis of malignant vasovagal syndrome. PMID:15020537

Depression and Dementia in Aging Adults with Down Syndrome: A Case Study Approach.

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Sung, Hyunsook; And Others

1997-01-01

A case study of three adults (ages 46-47) with Down syndrome investigated the patterns of symptoms associated with depression and dementia. Characteristics that distinguish between dementia and depression in adults with Down syndrome are described. Periodic comprehensive assessment of adults with Down syndrome to detect functioning changes is…

What can the treatment of Parkinson's disease learn from dementia care; applying a bio-psycho-social approach to Parkinson's disease.

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Gibson, Grant

2017-12-01

Within contemporary medical practice, Parkinson's disease (PD) is treated using a biomedical, neurological approach, which although bringing numerous benefits can struggle to engage with how people with PD experience the disease. A bio-psycho-social approach has not yet been established in PD; however, bio-psycho-social approaches adopted within dementia care practice could bring significant benefit to PD care. This paper summarises existing bio-psycho-social models of dementia care and explores how these models could also usefully be applied to care for PD. Specifically, this paper adapts the bio-psycho-social model for dementia developed by Spector and Orrell (), to suggest a bio-psycho-social model, which could be used to inform routine care in PD. Drawing on the biopsychosocial model of Dementia put forward by Spector and Orrell (), this paper explores the application of a bio-psycho-social model of PD. This model conceptualises PD as a trajectory, in which several interrelated fixed and tractable factors influence both PD's symptomology and the various biological and psychosocial challenges individuals will face as their disease progresses. Using an individual case study, this paper then illustrates how such a model can assist clinicians in identifying suitable interventions for people living with PD. This model concludes by discussing how a bio-psycho-social model could be used as a tool in PD's routine care. The model also encourages the development of a theoretical and practical framework for the future development of the role of the PD specialist nurse within routine practice. A biopsychosocial approach to Parkinson's Disease provides an opportunity to move towards a holistic model of care practice which addresses a wider range of factors affecting people living with PD. The paper puts forward a framework through which PD care practice can move towards a biopsychosocial perspective. PD specialist nurses are particularly well placed to adopt such a model

Prion disease resembling frontotemporal dementia and parkinsonism linked to chromosome 17

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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.

Pareidolia in Parkinson's disease without dementia: A positron emission tomography study.

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Uchiyama, Makoto; Nishio, Yoshiyuki; Yokoi, Kayoko; Hosokai, Yoshiyuki; Takeda, Atsushi; Mori, Etsuro

2015-06-01

Pareidolia, which is a particular type of complex visual illusion, has been reported to be a phenomenon analogous to visual hallucinations in patients with dementia with Lewy bodies. However, whether pareidolia is observed in Parkinson's disease (PD) or whether there are common underlying mechanisms of these two types of visual misperceptions remains to be elucidated. A test to evoke pareidolia, the Pareidolia test, was administered to 53 patients with PD without dementia and 24 healthy controls. The regional cerebral metabolic rate of glucose was measured using 18F-fluorodeoxyglucose positron emission tomography in the PD patients. PD patients without dementia produced a greater number of pareidolic illusions compared with the controls. Pareidolia was observed in all of the patients having visual hallucinations as well as a subset of those without visual hallucinations. The number of pareidolic illusions was correlated with hypometabolism in the bilateral temporal, parietal and occipital cortices. The index of visual hallucinations was correlated with hypometabolism in the left parietal cortex. A region associated with both pareidolia and visual hallucinations was found in the left parietal lobe. Our study suggests that PD patients without dementia experience pareidolia more frequently than healthy controls and that posterior cortical dysfunction could be a common neural mechanism of pareidolia and visual hallucinations. Pareidolia could represent subclinical hallucinations or a predisposition to visual hallucinations in Lewy body disease. Copyright © 2015 Elsevier Ltd. All rights reserved.

Memantine improves attention and episodic memory in Parkinson's disease dementia and dementia with Lewy bodies.

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Wesnes, Keith A; Aarsland, Dag; Ballard, Clive; Londos, Elisabet

2015-01-01

In both dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), attentional dysfunction is a core clinical feature together with disrupted episodic memory. This study evaluated the cognitive effects of memantine in DLB and PDD using automated tests of attention and episodic memory. A randomised double-blind, placebo-controlled, 24-week three centre trial of memantine (20 mg/day) was conducted in which tests of attention (simple and choice reaction time) and word recognition (immediate and delayed) from the CDR System were administered prior to dosing and again at 12 and 24 weeks. Although other results from this study have been published, the data from the CDR System tests were not included and are presented here for the first time. Data were available for 51 patients (21 DLB and 30 PDD). In both populations, memantine produced statistically significant medium to large effect sized improvements to choice reaction time, immediate and delayed word recognition. These are the first substantial improvements on cognitive tests of attention and episodic recognition memory identified with memantine in either DLB or PDD. Copyright © 2014 John Wiley & Sons, Ltd.

[Syndrome of rapid eye movement sleep behavior disorder and nocturia in Parkinson's disease].

Science.gov (United States)

Nodel, M R; Ukraintseva, Yu V; Yakhno, N N

Parasomnia, a syndrome of rapid eye movement sleep behavior disorder (RBD), is a common non-motor impairment in patients with Parkinson's disease (PD). The relationship between RBD with other symptoms of PD affecting night sleep, in particular, nocturia, is understudied. An aim of the study was to determine the symptoms related to night sleep disturbances in PD patients with RBD and assess the dynamics of these disturbances with the disease progression taking into account RBD onset. One hundred and forty patients (72 male and 68 female) with PD without dementia (mean age 61.98±0.79 years, PD stage - 2.35±0.05, duration 5.82±90.65 years) were examined. Motor disorders were assessed with the unified Parkinson's disease rating scale (UPDRS), sleep disturbances and frequent night urinations were evaluated with the Parkinson's Disease Sleep Scale (PDSS). The diagnosis of probable RBD was based on reports of patients or their relatives on the dream-related motor activity and vocalization. Quality-of-life was evaluated with the Parkinson's Disease Questionnaire (PDQ-39). Patients were followed up after 2.5 years. Probable RBD was diagnosed in 46.43% of patients, including 30.77%, who developed the syndrome before the manifestation of motor symptoms, 16.92% patients with simultaneous development of RBD and motor symptoms and 52.31% with RBD development >2 years after motor disorders. Patients with RBD differed from those without parasomnia by the higher severity of nocturia. After 2.5 years of follow-up, the severity of disease was greater in patients with RBD assessed by UPDRS, quality-of-life indices, severity of nocturia and episodes of nocturia. The highest frequency of episodes of nocturia was noted in patients with early onset of RBD before the manifestation of motor symptoms. RBD in patients with PD is associated with the rapid progress of nocturia, higher degree of worsening of daily activities and deterioration of quality of life. The relationship between RBD

Coexistent Brugada Syndrome and Wolff-Parkinson-White Syndrome: What is the Optimal Management?

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Abhishek Jaiswal, MBBS

2013-09-01

Full Text Available Coexistent Brugada syndrome and Wolff-Parkinson-White (WPW syndrome is rare, and as such poses management challenges. The overlap of symptoms attributable to each condition, the timing of ventricular stimulation after accessory pathway ablation and the predictive value of programmed stimulation in Brugada syndrome are controversial. We describe a case of coexistent Brugada syndrome and WPW syndrome in a symptomatic young adult. We discuss our treatment approach and the existing literature along with the challenges in management of such cases.

Cortical serotonin-S2 receptor binding in Lewy body dementia, Alzheimer's and Parkinson's diseases.

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Cheng, A V; Ferrier, I N; Morris, C M; Jabeen, S; Sahgal, A; McKeith, I G; Edwardson, J A; Perry, R H; Perry, E K

1991-11-01

The binding of the selective 5-HT2 antagonist [3H]ketanserin has been investigated in the temporal cortex of patients with Alzheimer's disease (SDAT), Parkinson's disease (PD), senile dementia of Lewy body type (SDLT) and neuropathologically normal subjects (control). 5-HT2 binding was reduced in SDAT, PD with dementia and SDLT. SDAT showed a 5-HT2 receptor deficit across most of the cortical layers. A significant decrease in 5-HT2 binding in the deep cortical layers was found in those SDLT cases without hallucinations. SDLT cases with hallucinations only showed a deficit in one upper layer. There was a significant difference in cortical layers III and V between SDLT without hallucinations and SDLT with hallucinations. The results confirm an abnormality of serotonin binding in various forms of dementia and suggest that preservation of 5-HT2 receptor in the temporal cortex may differentiate hallucinating from non-hallucinating cases of SDLT.

Diffusion tensor imaging differentiates vascular parkinsonism from parkinsonian syndromes of degenerative origin in elderly subjects

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Deverdun, Jérémy [Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Laboratoire Charles Coulomb, CNRS UMR 5221 - Université Montpellier II, Montpellier (France); I2FH, Institut d’Imagerie Fonctionnelle Humaine, Hôpital Gui de Chauliac, CHRU de, Montpellier (France); Menjot de Champfleur, Sophie [Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Clinique du Parc, Castelnau-le-Lez (France); Cabello-Aguilar, Simon [Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); I2FH, Institut d’Imagerie Fonctionnelle Humaine, Hôpital Gui de Chauliac, CHRU de, Montpellier (France); Maury, Florence [Department of Neurology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Molino, François [Laboratoire Charles Coulomb, CNRS UMR 5221 - Université Montpellier II, Montpellier (France); Institut de Génomique Fonctionnelle, UMR 5203 - INSERM U661 - Université Montpellier II - Université, Montpellier I (France); Charif, Mahmoud [Department of Neurology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Leboucq, Nicolas [Department of Neuroradiology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); Ayrignac, Xavier; Labauge, Pierre [Department of Neurology, Montpellier University Hospital Center, Gui de Chauliac Hospital, Montpellier (France); and others

2014-11-15

Background and Purpose: The etiologic diagnosis of parkinsonian syndromes is of particular importance when considering syndromes of vascular or degenerative origin. The purpose of this study is to find differences in the white-matter architecture between those two groups in elderly patients. Materials and Methods: Thirty-five patients were prospectively included (multiple-system atrophy, n = 5; Parkinson's disease, n = 15; progressive supranuclear palsy, n = 9; vascular parkinsonism, n = 6), with a mean age of 76 years. Patients with multiple-system atrophy, progressive supranuclear palsy and Parkinson's disease were grouped as having parkinsonian syndromes of degenerative origin. Brain MRIs included diffusion tensor imaging. Fractional anisotropy and mean-diffusivity maps were spatially normalized, and group analyses between parkinsonian syndromes of degenerative origin and vascular parkinsonism were performed using a voxel-based approach. Results: Statistical parametric-mapping analysis of diffusion tensor imaging data showed decreased fractional anisotropy value in internal capsules bilaterally in patients with vascular parkinsonism compared to parkinsonian syndromes of degenerative origin (p = 0.001) and showed a lower mean diffusivity in the white matter of the left superior parietal lobule (p = 0.01). Fractional anisotropy values were found decreased in the middle cerebellar peduncles in multiple-system atrophy compared to Parkinson's disease and progressive supranuclear palsy. The mean diffusivity was increased in those regions for these subgroups. Conclusion: Clinically defined vascular parkinsonism was associated with decreased fractional anisotropy in the deep white matter (internal capsules) compared to parkinsonian syndromes of degenerative origin. These findings are consistent with previously published neuropathological data.

Diffusion tensor imaging differentiates vascular parkinsonism from parkinsonian syndromes of degenerative origin in elderly subjects

International Nuclear Information System (INIS)

Deverdun, Jérémy; Menjot de Champfleur, Sophie; Cabello-Aguilar, Simon; Maury, Florence; Molino, François; Charif, Mahmoud; Leboucq, Nicolas; Ayrignac, Xavier; Labauge, Pierre

2014-01-01

Background and Purpose: The etiologic diagnosis of parkinsonian syndromes is of particular importance when considering syndromes of vascular or degenerative origin. The purpose of this study is to find differences in the white-matter architecture between those two groups in elderly patients. Materials and Methods: Thirty-five patients were prospectively included (multiple-system atrophy, n = 5; Parkinson's disease, n = 15; progressive supranuclear palsy, n = 9; vascular parkinsonism, n = 6), with a mean age of 76 years. Patients with multiple-system atrophy, progressive supranuclear palsy and Parkinson's disease were grouped as having parkinsonian syndromes of degenerative origin. Brain MRIs included diffusion tensor imaging. Fractional anisotropy and mean-diffusivity maps were spatially normalized, and group analyses between parkinsonian syndromes of degenerative origin and vascular parkinsonism were performed using a voxel-based approach. Results: Statistical parametric-mapping analysis of diffusion tensor imaging data showed decreased fractional anisotropy value in internal capsules bilaterally in patients with vascular parkinsonism compared to parkinsonian syndromes of degenerative origin (p = 0.001) and showed a lower mean diffusivity in the white matter of the left superior parietal lobule (p = 0.01). Fractional anisotropy values were found decreased in the middle cerebellar peduncles in multiple-system atrophy compared to Parkinson's disease and progressive supranuclear palsy. The mean diffusivity was increased in those regions for these subgroups. Conclusion: Clinically defined vascular parkinsonism was associated with decreased fractional anisotropy in the deep white matter (internal capsules) compared to parkinsonian syndromes of degenerative origin. These findings are consistent with previously published neuropathological data

Cognitive impairment in early-stage non-demented Parkinson's disease patients

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Pfeiffer, Helle Cecilie Viekilde; Løkkegaard, A; Zoetmulder, Marielle

2013-01-01

In Parkinson's disease (PD), Parkinson's disease dementia (PDD) and Parkinson's disease-mild cognitive impairment (PD-MCI) are common. PD-MCI is a risk factor for developing PDD. Knowledge of cognition in early-stages PD is essential in understanding and predicting the dementia process....

Cushing's Syndrome and Steroid Dementia.

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Bernini, Giampaolo; Tricò, Domenico

2016-01-01

Cushing's Syndrome (CS) is associated with a specific spectrum of dementia-like symptoms, including psychiatric disorders, such as major depression, anxiety and mania, and neurocognitive alterations, like impairment of memory and concentration. This pattern of clinical complications, which significantly impair the health-related quality of life of CS patients, is sometimes referred to as "steroid dementia syndrome" (SDS). The SDS is the result of anatomical and functional anomalies in brain areas involved in the processing of emotion and cognition, which are only partially restored after the biochemical remission of the disease. Therefore, periodical neuropsychiatric evaluations are recommended in all CS patients, and a long-term follow-up is required after normalization of hypercortisolism. Recent evidences demonstrate that three classes of drugs (glucocorticoid receptor antagonists, steroidogenesis inhibitors, and pituitary tumor-targeted drugs), which are used for medical treatment of CS, can rapidly relief neuropsychiatric symptoms of SDS. Furthermore, several psychoactive medications have demonstrated effectiveness in the treatment of symptoms induced by the acute or chronic glucocosteroid administration. In this paper, a review of the current and future patents for the treatment and prevention of CS and SDS will be presented.

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Wolff-Parkinson-White Syndrome in a Term Infant Presenting With Cardiopulmonary Arrest.

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Hoeffler, Christina D; Krenek, Michele E; Brand, M Colleen

2016-02-01

Wolff-Parkinson-White syndrome is a congenital abnormality of the cardiac conduction system caused by the presence of an abnormal accessory electrical pathway between the atria and the ventricles. This can result in intermittent tachyarrhythmias such as supraventricular tachycardia. In rare occasions, sudden death may occur from atrial fibrillation with rapid ventricular conduction. Supraventricular tachycardia typically has a sudden onset and offset, classified as a paroxysmal arrhythmia. Because of the variable occurrence, Wolff-Parkinson-White syndrome may go undiagnosed in the immediate newborn period. To highlight arrhythmia as a possible cause of sudden decompensation in infants. The clinical presentation of this infant is complex and a number of potential diagnoses were considered. Preexcitation on electrocardiogram resulted in the diagnosis of Wolff-Parkinson-White syndrome. Nurses caring for infants should be alert to tachycardia and irregularities of the heart rate, including those in the prenatal history, and should report them for evaluation. While all parents should be taught to watch for signs of illness, parents of infants with Wolff-Parkinson-White have additional learning needs, including recognizing early signs and symptoms of heart failure.

Dementia and mild cognitive impairment in patients with Parkinson's disease Demência e transtorno cognitivo leve em pacientes com doença de Parkinson

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Gloria Maria Almeida Souza Tedrus

2009-06-01

Full Text Available The objective of this research was to assess the occurrence of cognitive impairment in 32 individuals (average age: 67.2 years old with Parkinson' disease (PD. Procedures: clinical-neurological assessment; modified Hoehn and Yahr staging scale (HYS; standard neuropsychological battery of CERAD (Consortium to Establish a Registry for Alzheimer' Disease; Pfeffer questionnaire; and Clinical Dementia Rating. A comparison was made with a control group (CG, consisting of 26 individuals with similar age and educational level but without cognitive impairment. The PD patients showed an inferior performance in the CERAD battery when compared to the CG. Three PD sub-groups were characterised according to cognition: no cognitive impairment - 15 cases; mild cognitive impairment - 10; dementia - 7 cases. There was a significant association between motor disability (HYS and the occurrence of dementia. Dementia and mild cognitive impairment frequently occur in PD patients and should be investigated in a routine way.O objetivo desta pesquisa foi avaliar a ocorrência de déficits cognitivos em 32 indivíduos (idade média: 67,2 anos com doença de Parkinson (DP. Procedimentos: avaliação clínico-neurológica, escala de Hoehn and Yahr modificada (EHY, bateria neurospicológica do CERAD (Consortium to Establish a Registry for Alzheimer' Disease, questionário de Pfeffer e escore clínico da demência (Clinical Dementia Rating. Foi feita comparação com grupo controle (GC de 26 indivíduos sem declínio cognitivo, com idade e nível educacional similares. Os pacientes com DP tiveram desempenho inferior na bateria CERAD, quando comparados ao do GC. Foram caracterizados 3 subgrupos com PD segundo a cognição: sem déficits cognitivos - 15 casos; transtorno cognitivo leve - 10; demência - 7 casos. Houve associação entre comprometimento motor e ocorrência de demência. Demência e transtorno cognitivo leve são freqüentes em pacientes com DP e devem ser

Sexuality in patients with Parkinson's disease, Alzheimer's disease, and other dementias.

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Bronner, Gila; Aharon-Peretz, Judith; Hassin-Baer, Sharon

2015-01-01

Sexual dysfunction (SD) is common among patients with Parkinson's disease (PD), Alzheimer's disease (AD), and other dementias. Sexual functioning and well-being of patients with PD and their partners are affected by many factors, including motor disabilities, non-motor symptoms (e.g., autonomic dysfunction, sleep disturbances, mood disorders, cognitive abnormalities, pain, and sensory disorders), medication effects, and relationship issues. The common sexual problems are decreased desire, erectile dysfunction, difficulties in reaching orgasm, and sexual dissatisfaction. Hypersexuality is one of a broad range of impulse control disorders reported in PD, attributed to antiparkinsonian therapy, mainly dopamine agonists. Involvement of a multidisciplinary team may enable a significant management of hypersexuality. Data on SD in demented patients are scarce, mainly reporting reduced frequency of sex and erectile dysfunction. Treatment of SD is advised at an early stage. Behavioral problems, including inappropriate sexual behavior (ISB), are distressing for patients and their caregivers and may reflect the prevailing behavior accompanying dementia (disinhibition or apathy associated with hyposexuality). The neurobiologic basis of ISB is still only vaguely understood but assessment and intervention are recommended as soon as ISB is suspected. Management of ISB in dementia demands a thorough evaluation and understanding of the behavior, and can be treated by non-pharmacologic and pharmacologic interventions. © 2015 Elsevier B.V. All rights reserved.

Psychosocial therapy for Parkinson's-related dementia: study protocol for the INVEST randomised controlled trial.

Science.gov (United States)

McCormick, Sheree A; McDonald, Kathryn R; Vatter, Sabina; Orgeta, Vasiliki; Poliakoff, Ellen; Smith, Sarah; Silverdale, Monty A; Fu, Bo; Leroi, Iracema

2017-06-19

Parkinson's disease (PD) with mild cognitive impairment (MCI-PD) or dementia (PDD) and dementia with Lewy bodies (DLB) are characterised by motor and 'non-motor' symptoms which impact on quality of life. Treatment options are generally limited to pharmacological approaches. We developed a psychosocial intervention to improve cognition, quality of life and companion burden for people with MCI-PD, PDD or DLB. Here, we describe the protocol for a single-blind randomised controlled trial to assess feasibility, acceptability and tolerability of the intervention and to evaluate treatment implementation. The interaction among the intervention and selected outcome measures and the efficacy of this intervention in improving cognition for people with MCI-PD, PDD or DLB will also be explored. Dyads will be randomised into two treatment arms to receive either 'treatment as usual' (TAU) or cognitive stimulation therapy specifically adapted for Parkinson's-related dementias (CST-PD), involving 30 min sessions delivered at home by the study companion three times per week over 10 weeks. A mixed-methods approach will be used to collect data on the operational aspects of the trial and treatment implementation. This will involve diary keeping, telephone follow-ups, dyad checklists and researcher ratings. Analysis will include descriptive statistics summarising recruitment, acceptability and tolerance of the intervention, and treatment implementation. To pilot an outcome measure of efficacy, we will undertake an inferential analysis to test our hypothesis that compared with TAU, CST-PD improves cognition. Qualitative approaches using thematic analysis will also be applied. Our findings will inform a larger definitive trial. Ethical opinion was granted (REC reference: 15/YH/0531). Findings will be published in peer-reviewed journals and at conferences. We will prepare reports for dissemination by organisations involved with PD and dementia. ISRCTN (ISRCTN11455062). © Article author

Anaesthetic management of Wolff-Parkinson-White syndrome for hysterectomy

Directory of Open Access Journals (Sweden)

Sandeep Sahu

2011-01-01

Full Text Available Wolff-Parkinson-White syndrome (WPW is an uncommon cardiac disorder having an aberrant pathway between atria and ventricles. We are reporting a known case of WPW syndrome for hysterectomy under combined spinal epidural anaesthesia. Management of the present case is an important pearl to revisit management of WPW syndrome. The perioperative management should be tailored according to the nature of surgery and the clinical presentation of the patient.

[Behavioral impairments in Parkinson's disease].

Science.gov (United States)

Kashihara, Kenichi

2004-09-01

Behavioral impairments in parkinsonian patients include agitation, hypersexuality, stereotypic movement, pathological gambling, abuse of antiparkinsonian drugs, REM sleep behavioral disorder, and restless legs syndrome. Dementia, psychoses, and emotional disorders, such as depression and anxiety/panic disorder, also impair behavior. Symptoms may be produced by dysfunction of the central nervous system, medication, and/or the psychosocial problems associated with Parkinson's disease. Treatment therefore should be based on the cause of the symptoms seen. In some cases, the reduction or change of antiparkinsonian drugs, or both, may be effective. Treatment of the motor symptoms of Parkinson's disease, including motor fluctuations, may reduce the risk of panic attacks being evoked in the 'off' period. Use of antidepressants, sedatives, and neuroleptics may often be effective. Physicians should identify the causes of the symptoms of behavioral impairment and select appropriate treatments.

Dementia in Parkinson's disease: a Brazilian sample

Directory of Open Access Journals (Sweden)

Beatriz Baldivia

2011-10-01

Full Text Available To determine the prevalence of dementia associated with Parkinson's disease (PD-D in a Brazilian sample adopting clinical and diagnostic procedures recommended by the Movement Disorders Society (MDS. Sixty-seven patients were consecutively submitted to neurological, neuropsychological and functional examinations. PD-D was established according to MDS clinical criteria (Level II and clinical procedures for PD-D (Level I and prevalence rate was compared between the levels adopted. Ten patients (14.9% were diagnosed as demented by Level I criteria whereas sixteen (23.8% were diagnosed based on Level II criteria. Level I criteria had low sensitivity in detecting PD-D (31.25%, but greater specificity (90.19. The PD-D group had significantly worse performance on all neuropsychological tests, were older (p<0.001, had an older age of onset ofdisease (p<0.01, had lower educational level (p<0.02 and had higher scores on functional scales. Current age (p=0.046 and Hoehn & Yahr score (p=0.048 were predictors for developing PD-D.

A prospective 20-year longitudinal follow-up of dementia in persons with Down syndrome.

Science.gov (United States)

McCarron, M; McCallion, P; Reilly, E; Dunne, P; Carroll, R; Mulryan, N

2017-09-01

To examine dementia characteristics, age at onset and associated co-morbidities in persons with Down syndrome. A total of 77 people with Down syndrome aged 35 years and older were followed up from 1996 to 2015. The diagnosis of dementia was established using the modified ICD 10 Criteria and a combination of objective and informant-based tests. Cognitive tests included the Test for Severe Impairment and the Down Syndrome Mental Status Examination; adaptive behaviour was measured using the Daily Living Skills Questionnaire, and data from the Dementia Questionnaire for People with Intellectual Disabilities have been available since 2005. Over the 20-year period, 97.4% (75 of 77) persons developed dementia with a mean age of dementia diagnosis of 55 years (SD = 7.1, median = 56 years). Clinical dementia was associated with cognitive and function decline and seizure activity. Risk for dementia increased from 23% in those aged 50 years to 80% in those aged 65 years and above. There were no differences by level of ID. The previously reported high risk levels for dementia among people with Down syndrome were confirmed in this data as was the relationship with late onset epilepsy. The value of the instruments utilised in tracking decline and helping to confirm diagnosis is further highlighted. © 2017 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

Dementia and severity of parkinsonism determines the handicap of patients in late-stage Parkinson's disease: the Barcelona-Lisbon cohort.

Science.gov (United States)

Coelho, M; Marti, M J; Sampaio, C; Ferreira, J J; Valldeoriola, F; Rosa, M M; Tolosa, E

2015-02-01

Handicap has not been explored as a patient-centred outcome measure in Parkinson's disease (PD). The clinical features and medication use in late stages of PD (LS-PD) were reported previously. Handicap, medical conditions, use of healthcare resources and the impact of LS-PD upon caregivers were characterized in a cross-sectional study of LS-PD stages 4 or 5 of Hoehn and Yahr (H&Y). Handicap was measured using the London Handicap Scale (LHS: 0, maximal handicap; 1, no handicap). The mean LHS score in 50 patients was 0.33 (SD ±0.15). The presence of dementia, the Unified Parkinson's Disease Rating Scale part I score and the H&Y stage in 'off' independently predicted the LHS score (adjusted R(2) = 0.62; P = 0.000). Comorbidities and past medical conditions were frequent. Thirty-five patients lived at their house. Forty-five received unpaid care. Mean visits to the family doctor in the preceding 6 months were 2.2 (SD ±3.0) and to a neurologist 1.7 (SD ±1.0). Use of other health resources was low. Unpaid caregivers spent much time with patients and reported a high burden. Handicap could be measured in LS-PD and the LHS was easily completed by patients and caregivers. The high handicap in our cohort was mostly driven by the presence of dementia, behavioural complaints and the severity of non-dopaminergic motor features. Patients visited doctors infrequently and made low use of health resources, whilst unpaid caregivers reported a high burden. © 2014 EAN.

Atrial flutter with 1:1 conduction in undiagnosed Wolff-Parkinson-White syndrome.

Science.gov (United States)

Nelson, Jessie G; Zhu, Dennis W

2014-05-01

Atrial flutter with 1:1 atrioventricular conduction via an accessory pathway is an uncommon presentation of Wolff-Parkinson-White syndrome not previously reported in the emergency medicine literature. Wolff-Parkinson-White syndrome, a form of ventricular preexcitation sometimes initially seen and diagnosed in the emergency department (ED), can present with varied tachydysrhythmias for which certain treatments are contraindicated. For instance, atrial fibrillation with preexcited conduction needs specific consideration of medication choice to avoid potential degeneration into ventricular fibrillation. We describe an adult female presenting with a very rapid, regular wide complex tachycardia successfully cardioverted in the ED followed by a normal electrocardiogram (ECG). Electrophysiology study confirmed atrial flutter with 1:1 conduction and revealed an accessory pathway consistent with Wolff-Parkinson-White syndrome, despite lack of ECG findings of preexcitation during sinus rhythm. Why should an emergency physician be aware of this? Ventricular tachycardia must be the first consideration in patients with regular wide complex tachycardia. However, clinicians should consider atrial flutter with 1:1 conduction related to an accessory pathway when treating patients with the triad of very rapid rate (>250 beats/min), wide QRS complex, and regular rhythm, especially when considering pharmacologic treatment. Emergency physicians also should be aware of electrocardiographically concealed accessory pathways, and that lack of delta waves does not rule out preexcitation syndromes such as Wolff-Parkinson-White syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

Cuba's Strategy for Alzheimer Disease and Dementia Syndromes.

Science.gov (United States)

Bosch-Bayard, Rodolfo I; Llibre-Rodríguez, Juan J; Fernández-Seco, Alberto; Borrego-Calzadilla, Carmen; Carrasco-García, Mayra R; Zayas-Llerena, Tania; Moreno-Carbonell, Carmen R; Reymond-Vasconcelos, Ana G

2016-10-01

Dementia is a great challenge to public health in Cuba due to its impact on society and families. Cuba's National Intervention Strategy for Alzheimer Disease and Dementia Syndromes is designed to address this challenge. The Strategy includes working guidelines for primary and secondary care, education about rights of people with cognitive impairment, professional development, research, and health promotion and dementia prevention. An associated action plan, focused on primary care, includes proposals for creation of memory clinics, day centers and comprehensive rehabilitation services for cognitive stimulation. Short-term measures proposed include increasing early detection; creating a dementia morbidity and mortality registry; promoting professional training; providing support for families; and promoting basic and clinical research on dementia. Medium-term proposals aim to reduce dementia incidence and mortality by controlling risk factors and promoting healthy lifestyles, offering new treatment options and optimizing early detection. A set of indicators has been developed to evaluate strategy implementation. With this strategy, Cuba joins the small number of developing countries that have responded to WHO's call to improve care for patients with dementia and alleviate its impact on society and families. KEYWORDS Dementia, Alzheimer disease, aging, national health programs, social stigma, primary prevention, health promotion, civil rights, Cuba.

Clinical and neuroimage findings of dementia with lewy bodies

International Nuclear Information System (INIS)

Kang, Do Young; Park, Kyung Won; Kim, Jae Woo

2002-01-01

Dementia with lewy bodies (DLB) is the second common degenerative dementia and has several characteristics including fluctuating cognition, visual hallucination and Parkinsonism. We investigated clinical manifestations and neuroimaging findings in DLB patients. Ten probable DLB patients were included in this study. Brain MRI, Tc-99m HMPAO brain perfusion SPECT and I-123 IPT SPECT were performed. All patients were men and mean age of onset was 64.2 years (range from 54 to 80). All had fluctuating cognition and Parkinsonism, and 8 had visual hallucination. Dementia preceded Parkinsonism in 3 patients. Fluctuation of K-MMSE ranges from 3 to 8 points. Rest tremor was seen in 5 patients. Brain MRI showed cortical atrophy in all patients. Tc-99m brain perfusion SPECT showed hypoperfusion in occipital area as well as fronto-temporo-parietal areas. I-123 IPT SPECT revealed reduced uptake comparable to Parkinson's disease in the striatum. DLB should be first considered as one of possible diagnosis in patients showing dementia in the early stage of Parkinsonism. Hypoperfusion in the occipital area was thought to be a characteristic finding in DLB and to be helpful in differentiating DLB from other degenerative dementias

Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia.

Science.gov (United States)

Nagao, Shigeto; Yokota, Osamu; Nanba, Reiko; Takata, Hiroshi; Haraguchi, Takashi; Ishizu, Hideki; Ikeda, Chikako; Takeda, Naoya; Oshima, Etsuko; Sakane, Katsuaki; Terada, Seishi; Ihara, Yuetsu; Uchitomi, Yosuke

2012-12-15

We report an autopsy case of progressive supranuclear palsy (PSP) that clinically showed only slowly progressive and symmetric upper motor neuron syndrome over a disease course of 12 years. A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs. However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. She was clinically diagnosed as having motor neuron disease consistent with so-called primary lateral sclerosis. Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a lesser degree, in the basal ganglia and brain stem nuclei. In addition, severe fibrillary gliosis was noted in the precentral gyrus and corticospinal tract, being consistent with upper motor neuron syndrome observed in this case. No TAR-DNA binding protein 43-positive lesion, FUS pathology, Bunina body, or Lewy body-like hyaline inclusion was noted in the motor cortex or lower motor neurons. These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis. Copyright © 2012 Elsevier B.V. All rights reserved.

Mild Cognitive Impairment in Parkinson's Disease-What Is It?

Science.gov (United States)

Weil, Rimona S; Costantini, Alyssa A; Schrag, Anette E

2018-03-10

Mild cognitive impairment is a common feature of Parkinson's disease, even at the earliest disease stages, but there is variation in the nature and severity of cognitive involvement and in the risk of conversion to Parkinson's disease dementia. This review aims to summarise current understanding of mild cognitive impairment in Parkinson's disease. We consider the presentation, rate of conversion to dementia, underlying pathophysiology and potential biomarkers of mild cognitive impairment in Parkinson's disease. Finally, we discuss challenges and controversies of mild cognitive impairment in Parkinson's disease. Large-scale longitudinal studies have shown that cognitive involvement is important and common in Parkinson's disease and can present early in the disease course. Recent criteria for mild cognitive impairment in Parkinson's provide the basis for further study of cognitive decline and for the progression of different cognitive phenotypes and risk of conversion to dementia. Improved understanding of the underlying pathology and progression of cognitive change are likely to lead to opportunities for early intervention for this important aspect of Parkinson's disease.

Lewy body dementia--clinical, pathological and neurochemical interconnections.

Science.gov (United States)

Perry, R; McKeith, I; Perry, E

1997-01-01

Senile dementia of Lewy body type or Lewy body dementia (SDLT or LBD) is defined as a Lewy body associated disease presenting in the elderly primarily with dementia with variable extrapyramidal disorder. Characteristic clinical symptoms include fluctuating cognitive impairment, psychotic features such as hallucinations and a particular sensitivity to neuroleptic medication. Although apolipoprotein e4 allele is increased 2-3 fold in SDLT (as in Alzheimer's disease) and beta-amyloidosis occurs in most cases, the most robust neurobiological correlate of the dementia so far identified appears to be extensive cholinergic deficits in the neocortex. This is consistent with previously reported correlations between cortical cholinergic activity and dementia in Parkinson's disease (PD) and Alzheimer's disease. There is also a significant interaction between the density of limbic cortical Lewy bodies and dementia in both SDLT and PD, although the cortical neuronal population affected remains to be identified. Cortical Lewy body density is positively correlated with the age of disease onset in PD and SDLT. This may account for the increased incidence of psychiatric syndromes, as opposed to extrapyramidal disorder in Lewy body disease with advancing age as may age-related loss of cholinergic activity in cortical areas such as the hippocampus.

How to maintain the oral health of a child with Wolff-Parkinson-White syndrome: a case report.

Science.gov (United States)

Petroniatis, Tsampikos; Ortu, Eleonora; Marchili, Nicola; Giannoni, Mario; Marzo, Giuseppe; Monaco, Annalisa

2014-09-30

Wolff-Parkinson-White syndrome is one of the most important disorders of the heart conduction system. It is caused by the presence of an abnormal accessory electrical conduction pathway between the atria and the ventricles. In the present report, we describe the correct oral health management of a 12-year-old Caucasian girl with Wolff-Parkinson-White syndrome. We successfully undertook the dental care of a girl with Wolff-Parkinson-White syndrome, which we describe here.

Allgrove syndrome: an Egyptian family with two affected siblings

African Journals Online (AJOL)

Ola H. Gebril

2013-11-15

Nov 15, 2013 ... The neurological symptoms are variable and may affect the central, peripheral and autonomic nervous system [4,5]. Seri- ous reported neurological manifestations include dementia,. Wernicke's encephalopathy [6], optic atrophy, cerebellar atax- ia, and Parkinsonism [7]. The syndrome has been mapped to ...

Lewy body dementias

DEFF Research Database (Denmark)

Løkkegaard, Annemette; Korbo, Lise

2017-01-01

Dementia with Lewy bodies and Parkinson disease dementia share the same pathophysiology. Together they are called Lewy body dementias and are the second most common type of dementia. Lewy body dementias receive little attention, and patients are often misdiagnosed, leading to less than ideal...... management. In this article, diagnostic criteria combined with imaging and other biomarkers as well as current treatment recommendations are summarized, and some of the challenges for the future are outlined. Refinement of diagnosis and clarification of the pathogenesis are required in search for disease...

Subcortical grey matter changes in untreated, early stage Parkinson's disease without dementia.

Science.gov (United States)

Lee, Hye Mi; Kwon, Kyum-Yil; Kim, Min-Jik; Jang, Ji-Wan; Suh, Sang-Il; Koh, Seong-Beom; Kim, Ji Hyun

2014-06-01

Previous MRI studies have investigated cortical or subcortical grey matter changes in patients with Parkinson's disease (PD), yielding inconsistent findings between the studies. We therefore sought to determine whether focal cortical or subcortical grey matter changes may be present from the early disease stage. We recruited 49 untreated, early stage PD patients without dementia and 53 control subjects. Voxel-based morphometry was used to evaluate cortical grey matter changes, and automated volumetry and shape analysis were used to assess volume changes and shape deformation of the subcortical grey matter structures, respectively. Voxel-based morphometry showed neither reductions nor increases in grey matter volume in patients compared to controls. Compared to controls, PD patients had significant reductions in adjusted volumes of putamen, nucleus accumbens, and hippocampus (corrected p grey matter and clinical variables representing disease duration and severity. Our results suggest that untreated, early stage PD without dementia is associated with volume reduction and shape deformation of subcortical grey matter, but not with cortical grey matter reduction. Our findings of structural changes in the posterolateral putamen and ventromedial putamen/nucleus accumbens could provide neuroanatomical basis for the involvement of motor and limbic striatum, further implicating motor and non-motor symptoms in PD, respectively. Early hippocampal involvement might be related to the risk for developing dementia in PD patients. Copyright © 2014 Elsevier Ltd. All rights reserved.

Intraneuronal aluminum accumulation in amyotrophic lateral sclerosis and Parkinsonism-dementia of Guam

International Nuclear Information System (INIS)

Perl, D.P.; Gajdusek, D.C.; Garruto, R.M.; Yanagihara, R.T.; Gibbs, C.J.

1982-01-01

Scanning electron microscopy with energy-dispersive x-ray spectrometry was used to analyze the elemental content of neurofibrillary tangle (NFT)-bearing and NFT-free neurons within the Sommer's sector (H1 region) of the hippocampus in Guamanian Chamorros with amyotrophic lateral sclerosis and parkinsonism-dementia and in neurologically normal controls. Preliminary data indicate prominent accumulation of aluminum within the nuclear region and perikaryal cytoplasm of NFT-bearing hippocampal neurons, regardless of the underlying neurological diagnosis. These findings further extend the association between intraneuronal aluminum and NFT formation and support the hypothesis that environmental factors are related to the neurodegenerative changes seen in the Chamorro population

Characterisation of three-dimensional mapping in Wolff-Parkinson-White syndrome with septal aneurysmal dyskinesis.

Science.gov (United States)

Okada, Seigo; Muneuchi, Jun; Origuchi, Hideki

2018-01-01

A 21-year-old man with Wolff-Parkinson-White syndrome and aneurysmal septal dyskinesis underwent radiofrequency catheter ablation of the accessory pathways. Before radiofrequency catheter ablation, the activation wavefront arose from the aneurysmal septum, whereas the propagation of the left ventricle was normalised after radiofrequency catheter ablation. These findings demonstrate the importance of the electro-mechanical interaction in patients with Wolff-Parkinson-White syndrome and ventricular dysfunction.

Dementia syndrome and the onset of mind

International Nuclear Information System (INIS)

Matsuzawa, Taiju; Meguro, Kenichi; Ueda, Masamichi; Matsui, Hiroshige

1988-01-01

The present report is designed to make clear the mechanism of dementia syndrome and the onset area of the mind. The plan of the statistic studies with X-CT, MRI and PET to find out the focus of dementia in the cortex was an absolute failure. A large number of patients having infarction of varying numbers and sizes in the cortex was neuropsychologically normal. With MRI, quantitative changes of atrophy and destruction were observed in the amygdaloid and hippocampal system bilaterally in both multiinfarct dementia (MID) and Alzheimer disease (AD) patients. With PET, the activity or excitability of the cortices was estimated by measuring the glucose utilization with 18 F-2-fluorodeoxyglucose in response to musical stimulation (a Japanese popular song entitled Sakura, Sakura=cherry blossoms, cherry blossoms) while having the eyes closed, (1) Not only normal volunteers but also with cases of MID and AD, the primary sensory and motor areas were stimulated. (2) In cases of MID and AD, the glucose utilization, was reduced drastically in the bilateral temporal and parietal association cortices. The impulses from all the primary sensory areas drain into the amygdala. Furthermore the impulses from the amygdala drain directly or indirectly into the hippocampus, and the impulses flow into the temporal cortex. Recognition may take place in this temporal cortex. Then, the impulses come to the parietal cortex. Conception may be completed there. Any damage to the amygdaloid and hippocampal system would result in abnormalities in memory, recognition, conception and various emotions. This is a possible mechanism of dementia syndrome. In view of this data the system also can be said to be the onset area of the mind. (author)

Dementia syndrome and the onset of mind

Energy Technology Data Exchange (ETDEWEB)

Matsuzawa, Taiju; Meguro, Kenichi; Ueda, Masamichi; Matsui, Hiroshige

1988-12-01

The present report is designed to make clear the mechanism of dementia syndrome and the onset area of the mind. The plan of the statistic studies with X-CT, MRI and PET to find out the focus of dementia in the cortex was an absolute failure. A large number of patients having infarction of varying numbers and sizes in the cortex was neuropsychologically normal. With MRI, quantitative changes of atrophy and destruction were observed in the amygdaloid and hippocampal system bilaterally in both multiinfarct dementia (MID) and Alzheimer disease (AD) patients. With PET, the activity or excitability of the cortices was estimated by measuring the glucose utilization with /sup 18/F-2-fluorodeoxyglucose in response to musical stimulation (a Japanese popular song entitled Sakura, Sakura=cherry blossoms, cherry blossoms) while having the eyes closed, (1) Not only normal volunteers but also with cases of MID and AD, the primary sensory and motor areas were stimulated. (2) In cases of MID and AD, the glucose utilization, was reduced drastically in the bilateral temporal and parietal association cortices. The impulses from all the primary sensory areas drain into the amygdala. Furthermore the impulses from the amygdala drain directly or indirectly into the hippocampus, and the impulses flow into the temporal cortex. Recognition may take place in this temporal cortex. Then, the impulses come to the parietal cortex. Conception may be completed there. Any damage to the amygdaloid and hippocampal system would result in abnormalities in memory, recognition, conception and various emotions. This is a possible mechanism of dementia syndrome. In view of this data the system also can be said to be the onset area of the mind. (author).

Wolff-Parkinson-White syndrome: Implications for an anaesthesiologist

Directory of Open Access Journals (Sweden)

Vinaya Udaybhaskar

2017-01-01

Full Text Available Wolff-Parkinson-White (WPW syndrome is an electrical conduction abnormality of the heart that can induce potentially fatal arrhythmias at intermittent intervals. The induction and maintenance of general anaesthesia for a patient with WPW syndrome are risky due to the triggering capability of arrhythmias by various drugs and instrumentation. We hereby present the case of a 28-year-old male with previous cardiac illness, admitted for neurosurgical procedure, with drug-controlled WPW syndrome. The pre-operative optimisation, intraoperative scrutiny and vigil, along with readiness of standby medications and defibrillator made the ingress and egress from general anaesthesia uneventful. Thus, the potential dangers of WPW syndrome can be circumvented with watchful preparedness and meticulous monitoring.

Non-motor symptoms of Parkinson's disease

National Research Council Canada - National Science Library

Chaudhuri, K. Ray

2009-01-01

... dysfunction of Parkinson's disease 95 Daisy L. Whitehead and Richard. G. Brown 9 Depression, anxiety and apathy in Parkinson's disease 107 David A. Gallagher and Anette Schrag 10 Dementia in Pa...

A syndrome of epilepsy, dementia, and amelogenesis imperfecta: genetic and clinical features.

OpenAIRE

Christodoulou, J; Hall, R K; Menahem, S; Hopkins, I J; Rogers, J G

1988-01-01

A family is described with six members affected by a syndrome of epilepsy, dementia, and amelogenesis imperfecta (Kohlschütter's syndrome). An autosomal recessive pattern of inheritance is established for this disorder.

Chromosome 13 dementia syndromes as models of neurodegeneration

DEFF Research Database (Denmark)

Ghiso, J.; Revesz, T.; Holton, J.

2001-01-01

Two hereditary conditions, familial British dementia (FBD) and familial Danish dementia (FDD), are associated with amyloid deposition in the central nervous system and neurodegeneration. The two amyloid proteins, ABri and ADan, are degradation products of the same precursor molecule BriPP bearing......-terminus. Neurofibrillary tangles containing the classical paired helical filaments as well as neuritic components in many instances co-localize with the amyloid deposits. In both disorders, the pattern of hyperphosphorylatedtau immunoreactivity is almost indistinguishable from that seen in Alzheimer's disease....... These issues argue for the primary importance of the amyloid deposits in the mechanism(s) of neuronal cell loss. We propose FBD and FDD, the chromosome 13 dementia syndromes, as models to study the molecular basis of neurofibrillary degeneration, cell death and amyloid formation in the brain....

The Swedish BioFINDER 2 Study

Science.gov (United States)

2018-04-16

Dementia; Alzheimer Disease; Parkinson Disease; Lewy Body Disease; Parkinson-Dementia Syndrome; Frontotemporal Degeneration; Semantic Dementia; Progressive Nonfluent Aphasia; Progressive Supranuclear Palsy; Corticobasal Degeneration; Multiple System Atrophy; Mild Cognitive Impairment

Natural history of Wolff-Parkinson-White syndrome diagnosed in childhood.

Science.gov (United States)

Cain, Nicole; Irving, Claire; Webber, Steven; Beerman, Lee; Arora, Gaurav

2013-10-01

Wolff-Parkinson-White (WPW) syndrome carries a risk for symptomatic arrhythmias and sudden death. The aim of this study was to examine the natural history of patients with Wolff-Parkinson-White syndrome diagnosed in childhood followed longitudinally at a single institution. The study population consisted of 446 patients. The median age of diagnosis was 7 years, and 61% were male. Associated heart disease was present in 40 patients (9%). Modes of presentation included supraventricular tachycardia (38%), palpitations (22%), chest pain (5%), syncope (4%), atrial fibrillation (0.4%), sudden death (0.2%), and incidental findings (26%); data were unavailable in 4%. During the study period, a total of 243 patients (54%) had supraventricular tachycardia, and 7 patients (1.6%) had atrial fibrillation. Of patients who presented at ≤3 months of age, 35% had resolution of manifest preexcitation compared with 5.8% who presented at >3 months of age (p Wolff-Parkinson-White syndrome diagnosed in childhood, 64% had symptoms at presentation, and an additional 20% developed symptoms during follow-up. There were 6 sudden deaths (1.3%), with an overall incidence of 1.1 per 1,000 patient-years in patients with structurally normal hearts and 27 per 1,000 patient-years in patients with associated heart disease. Copyright © 2013 Elsevier Inc. All rights reserved.

Dural arteriovenous fistula as a treatable dementia.

Science.gov (United States)

Enofe, Ikponmwosa; Thacker, Ike; Shamim, Sadat

2017-04-01

Dementia is a chronic loss of neurocognitive function that is progressive and irreversible. Although rare, dural arteriovenous fistulas (DAVFs) could present with a rapid decline in neurocognitive function with or without Parkinson-like symptoms. DAVFs represent a potentially treatable and reversible cause of dementia. Here, we report the case of an elderly woman diagnosed with a DAVF after presenting with new-onset seizures, deteriorating neurocognitive function, and Parkinson-like symptoms.

A QUANTITATIVE STUDY OF α-SYNUCLEIN PATHOLOGY IN FIFTEEN CASES OF DEMENTIA ASSOCIATED WITH PARKINSON DISEASE

OpenAIRE

Armstrong, Richard A.; Kotzbauer, Paul T.; Perlmutter, Joel S.; Campbell, Meghan C.; Hurth, Kyle M.; Schmidt, Robert E.; Cairns, Nigel J.

2013-01-01

The α-synuclein-immunoreactive pathology of dementia associated with Parkinson disease (DPD) comprises Lewy bodies (LB), Lewy neurites (LN), and Lewy grains (LG). The densities of LB, LN, LG together with vacuoles, neurons, abnormally enlarged neurons (EN), and glial cell nuclei were measured in fifteen cases of DPD. Densities of LN and LG were up to 19 and 70 times those of LB respectively, depending on region. Densities were significantly greater in amygdala, entorhinal cortex (EC), and sec...

Association of metabolic syndrome and change in Unified Parkinson's Disease Rating Scale scores.

Science.gov (United States)

Leehey, Maureen; Luo, Sheng; Sharma, Saloni; Wills, Anne-Marie A; Bainbridge, Jacquelyn L; Wong, Pei Shieen; Simon, David K; Schneider, Jay; Zhang, Yunxi; Pérez, Adriana; Dhall, Rohit; Christine, Chadwick W; Singer, Carlos; Cambi, Franca; Boyd, James T

2017-10-24

To explore the association between metabolic syndrome and the Unified Parkinson's Disease Rating Scale (UPDRS) scores and, secondarily, the Symbol Digit Modalities Test (SDMT). This is a secondary analysis of data from 1,022 of 1,741 participants of the National Institute of Neurological Disorders and Stroke Exploratory Clinical Trials in Parkinson Disease Long-Term Study 1, a randomized, placebo-controlled trial of creatine. Participants were categorized as having or not having metabolic syndrome on the basis of modified criteria from the National Cholesterol Education Program Adult Treatment Panel III. Those who had the same metabolic syndrome status at consecutive annual visits were included. The change in UPDRS and SDMT scores from randomization to 3 years was compared in participants with and without metabolic syndrome. Participants with metabolic syndrome (n = 396) compared to those without (n = 626) were older (mean [SD] 63.9 [8.1] vs 59.9 [9.4] years; p metabolic syndrome experienced an additional 0.6- (0.2) unit annual increase in total UPDRS ( p = 0.02) and 0.5- (0.2) unit increase in motor UPDRS ( p = 0.01) scores compared with participants without metabolic syndrome. There was no difference in the change in SDMT scores. Persons with Parkinson disease meeting modified criteria for metabolic syndrome experienced a greater increase in total UPDRS scores over time, mainly as a result of increases in motor scores, compared to those who did not. Further studies are needed to confirm this finding. NCT00449865. © 2017 American Academy of Neurology.

Multiple Cardiac Rhabdomyomas, Wolff-Parkinson-White Syndrome, and Tuberous Sclerosis: An Infrequent Combination

Science.gov (United States)

Castilla Cabanes, Elena; Lacambra Blasco, Isaac

2014-01-01

Cardiac rhabdomyomas are benign cardiac tumours and are often associated with tuberous sclerosis. They are often asymptomatic with spontaneus regresion but can cause heart failure, arrhythmias, and obstruction. There have also been a few isolated reports of Wolff-Parkinson-White syndrome occurring in association with tuberous sclerosis and the great majority has been detected in patients with concomitant rhabdomyomas. We report a 12-day-old infant girl with tuberous sclerosis who presented with intraparietal and intracavitary rhabdomyomas with a Wolff-Parkinson-White syndrome (WPW). She represents one of the few published cases of WPW syndrome and tuberous sclerosis and particularly interesting because of intramural rhabdomyomas regression with persistent intracavitary rhabdomyomas after two years of followup. PMID:25328743

Multiple Cardiac Rhabdomyomas, Wolff-Parkinson-White Syndrome, and Tuberous Sclerosis: An Infrequent Combination

Directory of Open Access Journals (Sweden)

Elena Castilla Cabanes

2014-01-01

Full Text Available Cardiac rhabdomyomas are benign cardiac tumours and are often associated with tuberous sclerosis. They are often asymptomatic with spontaneus regresion but can cause heart failure, arrhythmias, and obstruction. There have also been a few isolated reports of Wolff-Parkinson-White syndrome occurring in association with tuberous sclerosis and the great majority has been detected in patients with concomitant rhabdomyomas. We report a 12-day-old infant girl with tuberous sclerosis who presented with intraparietal and intracavitary rhabdomyomas with a Wolff-Parkinson-White syndrome (WPW. She represents one of the few published cases of WPW syndrome and tuberous sclerosis and particularly interesting because of intramural rhabdomyomas regression with persistent intracavitary rhabdomyomas after two years of followup.

Surgical cure ofthe Wolff-Parkinson-White syndrome a comparison ...

African Journals Online (AJOL)

Surgical cure ofthe Wolff-Parkinson-White syndrome a comparison oftwo techniques. u. o. VON OPPELL, R. N. SCOTT MILLAR, D. A. MILNE. TABLE!. Characteristics of WPW patients referred for surgical ablation of their aberrant atrioventricular pathways. Patient population and methods. We' retrospectively reviewed 19 ...

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Quantitative Electroencephalography as a Diagnostic Tool for Alzheimer's Dementia in Adults with Down Syndrome

DEFF Research Database (Denmark)

Salem, Lise Cronberg; Sabers, Anne; Kjaer, Troels W

2015-01-01

be used as a diagnostic marker for dementia. The aim of this study was to examine the value of qEEG in the diagnostic evaluation of dementia in patients with Down syndrome (DS). METHOD: The study included 21 patients with DS and mild-to-moderate dementia due to Alzheimer's disease (DS-AD) and 16 age...

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Full Text Available ... Swallowing: Part 2 What Is the Relationship Between Depression and Parkinson's Disease? What Is Patient-Centered Care? Hallucinations and Delusions Dealing with Dementia CareMAP: Medicamentos ...

[Case of neuroleptic malignant syndrome following open heart surgery for thoracic aortic aneurysm with parkinson's disease].

Science.gov (United States)

Shinoda, Maiko; Sakamoto, Mik; Shindo, Yuki; Ando, Yumi; Tateda, Takeshi

2013-12-01

An 80-year-old woman with Parkinson's disease was scheduled for open heart surgery to repair thoracic aortic aneurysm. Parkinson's symptoms were normally treated using oral levodopa (200 mg), selegiline-hydrochloride (5 mg), bromocriptine-mesilate (2 mg), and amantadine-hydrochloride (200 mg) daily. On the day before surgery, levodopa 50mg was infused intravenously. Another 25 mg of levodopa was infused immediately after surgery. Twenty hours later, the patient developed tremors, heyperventilation, but no obvious muscle rigidity. Two days after surgery, the patient exhibited high fever, hydropoiesis, elevated creatine kinase, and a rise in blood leukocytes. She was diagnosed with neuroleptic malignant syndrome. She was intubated, and received dantrolene sodium. Symptoms of neuroleptic malignant syndrome disappeared on the fourth postoperative day. The stress of open heart surgery, specifically extracorporeal circulation and concomitant dilution of levodopa, triggered neuroleptic malignant syndrome in this patient. Parkinson's patients require higher doses of levodopa prior to surgery to compensate and prevent neuroleptic malignant syndrome after surgery.

The genetics of dementias. Part 1: Molecular basis of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17

Directory of Open Access Journals (Sweden)

Anna Kowalska

2009-06-01

Full Text Available Frontotemporal dementia (FTD, characterized by neurodegeneration mainly in the frontal and temporal lobes, accounts for ca. 10–15�0of all dementias. In 1892 the Czech-German neuropsychiatrist Arnold Pick reported the first case of FTD in a 71-year-old patient suffering from progressive dementia, memory disturbances, and aphasia associated with frontal and temporal lobe atrophy and the presence of neuronal inclusions. Later the inclusions were named Pick bodies. The neuropathological hallmark of FTD is very differentiated. In contrast to Alzheimer’s disease (AD, there are neither senile plaques nor neurofibrillary tangles in the brains of FTD patients. Frontotemporal dementias are tauopathies, a group of disorders caused by aberrant metabolism of tau protein, a family of proteins associated with microtubules (MAPT: macrotubule-associated tau protein. In the nervous system the protein stabilizes microtubules in neuronal axons and is thus responsible for crucial processes in neuron metabolism, such as signal transduction, plasticity, and intracellular transport. In the human brain, six isoforms are produced from the MAPT gene (chromosome 17 q21.2 by alternative mRNA splicing. The isoforms differ in the number of amino acids in the protein chain, the presence of three (3R tau type or four (4R tau type domains responsible for binding to microtubules, and one or two inserts containing from 29 to 58 amino acids. The isoforms are modified posttranslationally by hyperphosphorylation, glycation, or oxidation, which can change the protein’s properties and disturb its normal function. Altered metabolism of tau protein changes its interactions with tubulin, leading to destabilization of the microtubule structure and initiating the generation of toxic tau aggregates. The first mutations in the MAPT gene responsible for frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17 were found in 1998. So far over 40 mutations in the MAPT

Antihypertensive agents and risk of Parkinson's disease, essential tremor and dementia: a population-based prospective study (NEDICES).

Science.gov (United States)

Louis, Elan D; Benito-León, Julián; Bermejo-Pareja, Félix

2009-01-01

Recent interest in antihypertensive agents, especially calcium channel blockers, has been sparked by the notion that these medications may be neuroprotective. A modest literature, with mixed results, has examined whether these medications might lower the odds or risk of Parkinson's disease (PD) or dementia. There are no data for essential tremor (ET). To examine the association between antihypertensive use (defined broadly and by individual subclasses) and ET, PD and dementia. For each disorder, we used cross-sectional data (association with prevalent disease) and prospective data (association with incident disease). Prospective population-based study in Spain enrolling 5,278 participants at baseline. Use of antihypertensive medications (aside from beta-blockers) was similar in prevalent ET cases and controls. Baseline use of antihypertensive agents was not associated with reduced risk of incident ET. Antihypertensive medication use was not associated with prevalent or incident PD. Calcium channel blocker use was marginally reduced in prevalent dementia cases (OR(adjusted) = 0.63, p = 0.06) but was not associated with reduced risk of incident dementia (RR(adjusted) = 1.02, p = 0.95). We did not find evidence of a protective effect of antihypertensive medications in these three neurodegenerative disorders. Copyright 2009 S. Karger AG, Basel.

Cognitive profiling of Parkinson disease patients with mild cognitive impairment and dementia.

Science.gov (United States)

Biundo, Roberta; Weis, Luca; Facchini, Silvia; Formento-Dojot, Patrizia; Vallelunga, Annamaria; Pilleri, Manuela; Antonini, Angelo

2014-04-01

Prevalence of mild cognitive impairment (MCI) and dementia in Parkinson disease (PD) is variable because different classification criteria are applied and there is lack of consensus about neuropsychological tests and cut-off used for cognitive profiling. Given the important therapeutic consequences for patient management, we aimed at identifying suitable diagnostic cognitive tests and respective screening cut-off values for MCI and dementia in PD (PDD). We evaluated 105 PD patients using an extensive neuropsychological battery categorized as PD without cognitive impairment (PD-CNT) (35%), PD-MCI (47%) and PDD (18%) based on established criteria and calculated Receiver Operating Characteristic (ROC) curves. We found different sensitivity and specificity among neuropsychological tests in detecting PD-MCI and PDD. In particular performance in attention/set shifting, verbal memory and language abilities, discriminated both PD-MCI and PDD from PD-CNT. Abilities involved mainly in semantic retrieval mechanisms discriminated PD-CNT from PD-MCI but also PD-MCI from PDD. Finally deficits in executive and visual-spatial abilities were only affected in PDD. Our data point to an independent and different load of each test in defining different PD cognitive statuses. These findings can help selection of appropriate cognitive batteries in longitudinal studies and definition of stage-specific therapeutic targets. Copyright © 2014 Elsevier Ltd. All rights reserved.

Imaging dementias

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Savoiardo, M.; Grisoli, M. [Dept. of Neuroradiology, Istituto Nazionale Neurologico, Milan (Italy)

2001-03-01

Dementia is the progressive loss of intellectual functions due to involvement of cortical or subcortical areas. Specific involvement of certain brain areas in the different diseases leads to impairment of different functions, e. g., memory, language, visuospatial abilities, and behavior. Magnetic resonance imaging and other neuroradiological studies may indicate which structures are mainly or selectively involved in a demented patient, thus allowing clinical-radiological correlations. Clinical presentation and evolution of the disease, supported by imaging studies, may lead to a highly probable diagnosis. The most common disorders, or the most relevant from the neuroradiological point of view, such as Alzheimer's disease, frontotemporal dementia, vascular dementias, dementia associated with parkinsonism, Huntington's disease, Creutzfeldt-Jakob disease, and normal-pressure hydrocephalus, are briefly discussed. (orig.)

Imaging dementias

International Nuclear Information System (INIS)

Savoiardo, M.; Grisoli, M.

2001-01-01

Dementia is the progressive loss of intellectual functions due to involvement of cortical or subcortical areas. Specific involvement of certain brain areas in the different diseases leads to impairment of different functions, e. g., memory, language, visuospatial abilities, and behavior. Magnetic resonance imaging and other neuroradiological studies may indicate which structures are mainly or selectively involved in a demented patient, thus allowing clinical-radiological correlations. Clinical presentation and evolution of the disease, supported by imaging studies, may lead to a highly probable diagnosis. The most common disorders, or the most relevant from the neuroradiological point of view, such as Alzheimer's disease, frontotemporal dementia, vascular dementias, dementia associated with parkinsonism, Huntington's disease, Creutzfeldt-Jakob disease, and normal-pressure hydrocephalus, are briefly discussed. (orig.)

Deep brain stimulation for the treatment of Alzheimer disease and dementias.

Science.gov (United States)

Laxton, Adrian W; Lozano, Andres M

2013-01-01

To review the use of deep brain stimulation (DBS) for treatment of dementia. A PubMed literature search was conducted to identify all studies that have investigated the use of DBS for treatment of dementia. Three studies examined the use of DBS for dementia. One study involved fornix DBS for Alzheimer disease (AD), and two studies involved DBS of the nucleus basalis of Meynert, one to treat AD and one to treat Parkinson disease dementia. Evidence for the use of DBS to treat dementia is preliminary and limited. Fornix and nucleus basalis of Meynert DBS can influence activity in the pathologic neural circuits that underlie AD and Parkinson disease dementia. Further investigation into the potential clinical effects of DBS for dementia is warranted. Copyright © 2013 Elsevier Inc. All rights reserved.

A Case of Morvan Syndrome Mimicking Amyotrophic Lateral Sclerosis With Frontotemporal Dementia.

Science.gov (United States)

Freund, Brin; Maddali, Manoj; Lloyd, Thomas E

2016-06-01

Morvan syndrome is a rare autoimmune/paraneoplastic disorder involving antibodies to the voltage-gated potassium channel complex. It is defined by subacute encephalopathy, neuromuscular hyperexcitability, dysautonomia, and sleep disturbance. It may present a diagnostic dilemma when trying to differentiate from amyotrophic lateral sclerosis with frontotemporal dementia. A 76-year-old man with a history of untreated prostate adenocarcinoma was evaluated for subacute cognitive decline, diffuse muscle cramps, and hyponatremia. MRI demonstrated atrophy most prominent in the frontal and temporal regions. Electromyography (EMG) demonstrated diffuse myokymia/neuromyotonia. Polysomnography lacked REM and N3 sleep. Paraneoplastic panel detected antibodies to voltage-gated potassium channel complex (CASPR2 subtype). It is difficult to differentiate between Morvan syndrome and amyotrophic lateral sclerosis with frontotemporal dementia with examination and neuroimaging alone. There may be a link between Morvan syndrome and prostate adenocarcinoma which could help with screening/diagnosis. The authors found that laboratory and neurophysiological tests are indispensable in diagnosing and treating Morvan syndrome.

Sensitivity and specificity of Addenbrooke's Cognitive Examination, Mattis Dementia Rating Scale, Frontal Assessment Battery and Mini Mental State Examination for diagnosing dementia in Parkinson's disease.

Science.gov (United States)

Kaszás, B; Kovács, N; Balás, I; Kállai, J; Aschermann, Z; Kerekes, Z; Komoly, S; Nagy, F; Janszky, J; Lucza, T; Karádi, K

2012-06-01

Among the non-motor features of Parkinson's disease (PD), cognitive impairment is one of the most troublesome problems. Highly sensitive and specific screening instruments for detecting dementia in PD (PDD) are required in the clinical practice. In our study we evaluated the sensitivity and specificity of different neuropsychological tests (Addenbrooke's Cognitive Examination, ACE; Frontal Assessment Battery, FAB and Mattis Dementia Rating Scale, MDRS) in 73 Parkinson's disease patients without depression. By receiver operating characteristic curve analysis, these screening instruments were tested against the recently established clinical diagnostic criteria of PDD. Best cut-off score for ACE to identify PDD was 80 points (sensitivity = 74.0%, specificity = 78.1%). For FAB the most optimal cut-off value was 12 points (sensitivity = 66.3%, specificity = 72.2%); whereas for MDRS it was 125 points (sensitivity = 89.8%, specificity = 98.3%). Among the examined test batteries, MDRS had the best clinicometric profile for detecting PDD. Although the types of applied screening instruments might differ from movement disorder clinic to clinic within a country, determination of the most specific and sensitive test for the given population remains to be an important task. Our results demonstrated that the specificity and sensitivity of MDRS was better than those of ACE, FAB and MMSE in Hungary. However, further studies with larger sample size and more uniform criteria for participation are required to determine the most suitable screening instrument for cognitive impairment. Copyright © 2012 Elsevier Ltd. All rights reserved.

Sleepiness in Idiopathic REM Sleep Behavior Disorder and Parkinson Disease.

Science.gov (United States)

Arnulf, Isabelle; Neutel, Dulce; Herlin, Bastien; Golmard, Jean-Louis; Leu-Semenescu, Smaranda; Cochen de Cock, Valérie; Vidailhet, Marie

2015-10-01

To determine whether patients with idiopathic and symptomatic RBD were sleepier than controls, and if sleepiness in idiopathic RBD predicted earlier conversion to Parkinson disease. The Epworth Sleepiness Scale (ESS) and its determinants were compared at the time of a video-polysomnography for an RBD diagnosis in patients with idiopathic RBD, in patients with Parkinson disease, and in controls. Whether sleepiness at time of RBD diagnosis predicted an earlier conversion to neurodegenerative diseases was retrospectively analyzed in the followed-up patients. The 75 patients with idiopathic RBD were sleepier (ESS: 7.8 ± 4.6) at the time of RBD diagnosis than 74 age- and sex-matched controls (ESS: 5.0 ± 3.6, P sleep measures. Among the 69 patients with idiopathic RBD who were followed up for a median 3 years (1-15 years), 16 (23.2%) developed parkinsonism (n = 6), dementia (n = 6), dementia plus parkinsonism (n = 2), and multiple system atrophy (n = 2). An ESS greater than 8 at time of RBD diagnosis predicted a shorter time to phenoconversion to parkinsonism and dementia, from RBD onset, and from RBD diagnosis (when adjusted for age and time between RBD onset and diagnosis). Sleepiness is associated with idiopathic REM sleep behavior disorder and predicts more rapid conversion to parkinsonism and dementia, suggesting it is an early marker of neuronal loss in brainstem arousal systems. © 2015 Associated Professional Sleep Societies, LLC.

Detecting cognitive impairment in patients with Parkinson's disease using a brief cognitive screening tool: Addenbrooke's Cognitive Examination (ACE

Directory of Open Access Journals (Sweden)

Anabel Chade

Full Text Available Abstract Detecting cognitive impairment in patients with Parkinson's disease is crucial for good clinical practice given the new therapeutic possibilities available. When full neuropsychological evaluations are not available, screening tools capable of detecting cognitive difficulties become crucial. Objective: The goal of this study was to investigate whether the Spanish version of the Addenbrooke's Cognitive Examination (ACE is capable of detecting cognitive difficulties in patients with Parkinson's disease and discriminating their cognitive profile from patients with dementia. Methods: 77 early dementia patients (53 with Alzheimer's Disease and 24 with Frontotemporal Dementia, 22 patients with Parkinson's disease, and 53 healthy controls were evaluated with the ACE. Results: Parkinson's disease patients significantly differed from both healthy controls and dementia patients on ACE total score. Conclusions: This study shows that the Spanish version of the ACE is capable of detecting patients with cognitive impairment in Parkinson's disease and is able to differentiate them from patients with dementia based on their general cognitive status.

Caring for the Student with Wolff-Parkinson-White Syndrome

Science.gov (United States)

Prenni, Patricia G.

2009-01-01

Wolff-Parkinson-White syndrome is a cardiac condition in which an extra electrical pathway within the heart causes an abnormal increase in heart rate. It affects one to three people of every 1,000 people worldwide, occurring more often in males. Diagnosis usually occurs during young adulthood, so it is important for school nurses to be familiar…

Renal Failure in Dementia with Lewy Bodies Presenting as Catatonia

Science.gov (United States)

Fekete, Robert

2013-01-01

Catatonia, originally described by Karl Kahlbaum in 1874, may be regarded as a set of clinical features found in a subtype of schizophrenia, but the syndrome may also stem from organic causes including vascular parkinsonism, brain masses, globus pallidus lesions, metabolic derangements, and pharmacologic agents, especially first generation antipsychotics. Catatonia may include paratonia, waxy flexibility (cerea flexibilitas), stupor, mutism, echolalia, and catalepsy (abnormal posturing). A case of catatonia as a result of acute renal failure in a patient with dementia with Lewy bodies is described. This patient recovered after intravenous fluid administration and reinstitution of the atypical dopamine receptor blocking agent quetiapine, but benzodiazepines and amantadine are additional possible treatments. Recognition of organic causes of catatonia leads to timely treatment and resolution of the syndrome. PMID:23466522

Lymphocyte concanavalin A capping: a similarity between Down's syndrome and early onset primary degenerative dementia.

OpenAIRE

Duijndam-van den Berge, M; Goekoop, J G

1986-01-01

Lymphocyte capping with concanavalin A was studied in adult patients with Down's syndrome and aged patients with primary degenerative dementia. In both disorders a decreased capping was found as compared with age-matched and clinically relevant control groups. Colchicine had a strong enhancing effect on capping in Down's syndrome. In primary degenerative dementia the enhancing effect of colchicine was restricted to a subgroup of patients with onset of the dementing illness before the age of 8...

Wolff-Parkinson-White syndrome with cleft lip and palate: A rare, previously unreported association

Directory of Open Access Journals (Sweden)

K Kannan

2008-01-01

Full Text Available Wolff-Parkinson-White syndrome, also called Pre Excitation Syndrome, is characterized by an extra pathway that conducts the electrical impulses from the atria to the ventricles without the normal delay. We are reporting a case of WPW syndrome with a cleft lip and palate, which is a rare association and previously unreported in literature.

Detecting cognitive impairment in patients with Parkinson's disease with a brief cognitive screening tool: the Addenbrooke's Cognitive Examination (ACE).

Science.gov (United States)

Chade, Anabel; Roca, María; Torralva, Teresa; Gleichgerrcht, Ezequiel; Fabbro, Nicolás; Arévalo, Gonzalo Gómez; Gershanik, Oscar; Manes, Facundo

2008-01-01

Detecting cognitive impairment in patients with Parkinson's disease is crucial for good clinical practice given the new therapeutic possibilities available. When full neuropsychological evaluations are not available, screening tools capable of detecting cognitive difficulties become crucial. The goal of this study was to investigate whether the Spanish version of the Addenbrooke's Cognitive Examination (ACE) is capable of detecting cognitive difficulties in patients with Parkinson's disease and discriminating their cognitive profile from patients with dementia. 77 early dementia patients (53 with Alzheimer's Disease and 24 with Frontotemporal Dementia), 22 patients with Parkinson's disease, and 53 healthy controls were evaluated with the ACE. Parkinson's disease patients significantly differed from both healthy controls and dementia patients on ACE total score. This study shows that the Spanish version of the ACE is capable of detecting patients with cognitive impairment in Parkinson's disease and is able to differentiate them from patients with dementia based on their general cognitive status.

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Can the CERAD neuropsychological battery be used to assess cognitive impairment in Parkinson's disease?

Directory of Open Access Journals (Sweden)

Carlos Henrique Ferreira Camargo

Full Text Available ABSTRACT The Consortium to Establish a Registry for Alzheimer's Disease (CERAD neuropsychological battery was created to assess cognitive impairment in Alzheimer's disease (AD but it is widely-used for various dementias. The aim of this study was to analyze the efficacy of using the CERAD battery in the assessment of patients with Parkinson's disease. Forty-nine patients with Parkinson's disease were divided into two groups (one with dementia and one without using the Movement Disorder Society criteria for Parkinson's disease dementia. Cognitive deficits were assessed with the Clinical Dementia Rating Scale as the gold standard, and the CERAD. The ROC curve for the CERAD battery had an area under the curve = 0.989 (95% CI = 0.967 – 1, p<0.0001. Among the CERAD subtests, verbal fluency had the worst accuracy, and word list learning had the best accuracy. Despite the limits of this study, the CERAD battery can be efficient for assessment of cognitive deficits in Parkinson's disease patients.

A three dimensional anatomical view of oscillatory resting-state activity and functional connectivity in Parkinson's disease related dementia: An MEG study using atlas-based beamforming

NARCIS (Netherlands)

Ponsen, M.M.; Stam, C.J.; Bosboom, J.L.W.; Berendse, H.W.; Hillebrand, A.

2013-01-01

Parkinson's disease (PD) related dementia (PDD) develops in up to 80% of PD patients. The present study was performed to further unravel the underlying pathophysiological mechanisms by applying a new analysis approach that uses an atlas-based MEG beamformer to provide a detailed anatomical mapping

A placebo-controlled study of memantine (Ebixa) in dementia of Wernicke-Korsakoff syndrome.

Science.gov (United States)

Rustembegović, Avdo; Kundurović, Zlata; Sapcanin, Aida; Sofic, Emin

2003-01-01

We evaluated the responses of 16 patients to preliminarily explore the spectrum of effectiveness and tolerability of the memantine, and NMDA antagonist, in the treatment of dementia in Wernicke-Korsakoff syndrome. In this study, for the first time in dementia of Wernicke-Korsakoff syndrome, the response to memantine was assessed. 16 patients with median age of 64 years and median body weight of 77 kg were treated with memantine 10 mg twice daily for up to 28 weeks. Clinical global impressions (CGI), and Mini Mental Status Examination (MMSE) were performed during the treatment period (after 2, 4, and 28 weeks). Efficacy measures also included the ADCS-Activities of Daily Living scale (ADCS-ADL). At 28 weeks, the ADCS-ADL showed significantly less deterioration in memantine treated patients compared with placebo (-2.3 compared with -4.3: p = 0.005). The results of MMSE demonstrate a significant and clinically relevant benefit for memantine relative to placebo as shown by positive outcomes in cognitive and functional assessments. Memantine (10 mg) was safe and well tolerated. The preliminarily findings of this study with 16 patients suggested that memantine is effective in the treatment of dementia in Wernicke-Korsakoff syndrome.

[Dementia and depression determine care dependency in Parkinson's disease: analysis of 1,449 outpatients receiving nursing care in Germany].

Science.gov (United States)

Riedel, O; Dodel, R; Deuschl, G; Förstl, H; Henn, F; Heuser, I; Oertel, W; Reichmann, H; Riederer, P; Trenkwalder, C; Wittchen, H U

2011-08-01

Parkinson's disease (PD) is frequently accompanied by dementia or depression which can aggravate the clinical picture of the disease and increase the risk of care dependency (CD). Little is known about the associations between PD, these neuropsychiatric comorbidities and CD in outpatients. A nationwide sample of outpatients (n=1,449) was examined by office-based neurologists (n=315) comprising the documentation of the general, neurological status and the degree of CD. The dementia status was clinically rated according to the established DSM-IV criteria. Depression was screened with the Montgomery-Asberg Depression Rating Scale (MADRS). Overall, 18.3% of all patients were care dependent. Even after adjustment for PD severity, patients with depression (OR=2.8; 95% CI 1.8-4.3), dementia (OR=2.7; 95% CI 1.8-4.1) or both (OR=3.9; 95% CI 2.5-60,0) were at higher risk for CD than patients without dementia or depression. Patients aged ≥76 years were fourfold more likely to be care dependent than patients aged ≤65 years (OR=3.5; 95% CI 2.3-5.5). Across all age groups, patients with depression featured the highest increments (from 11.9 to 42.0%). The risk for CD is substantially elevated in outpatients with PD when further neuropsychiatric symptoms are present. The data suggest that depression contributes equally to disability as does dementia.

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The effects of cognitive reserve and lifestyle on cognition and dementia in Parkinson's disease--a longitudinal cohort study.

Science.gov (United States)

Hindle, John V; Hurt, Catherine S; Burn, David J; Brown, Richard G; Samuel, Mike; Wilson, Kenneth C; Clare, Linda

2016-01-01

Cognitive reserve theory seeks to explain the observed mismatch between the degree of brain pathology and clinical manifestations. Early-life education, midlife social and occupational activities and later-life cognitive and social interactions are associated with a more favourable cognitive trajectory in older people. Previous studies of Parkinson's disease (PD) have suggested a possible role for the effects of cognitive reserve, but further research into different proxies for cognitive reserve and longitudinal studies is required. This study examined the effects of cognitive lifestyle on cross-sectional and longitudinal measures of cognition and dementia severity in people with PD. Baseline assessments of cognition, and of clinical, social and demographic information, were completed by 525 participants with PD. Cognitive assessments were completed by 323 participants at 4-year follow-up. Cognition was assessed using the measures of global cognition dementia severity. Cross-sectional and longitudinal serial analyses of covariance for cognition and binomial regression for dementia were performed. Higher educational level, socio-economic status and recent social engagement were associated with better cross-sectional global cognition. In those with normal cognition at baseline, higher educational level was associated with better global cognition after 4 years. Increasing age and low levels of a measure of recent social engagement were associated with an increased risk of dementia. Higher cognitive reserve has a beneficial effect on performance on cognitive tests and a limited effect on cognitive decline and dementia risk in PD. Copyright © 2015 John Wiley & Sons, Ltd.

Vascular Parkinsonism and cognitive impairment: literature review, Brazilian studies and case vignettes

Directory of Open Access Journals (Sweden)

Thiago Cardoso Vale

Full Text Available ABSTRACT Vascular Parkinsonism (VP is a form of secondary Parkinsonism resulting from cerebrovascular disease. Estimates of the frequency of VP vary greatly worldwide; 3% to 6% of all cases of Parkinsonism are found to have a vascular etiology. In a Brazilian community-based study on Parkinsonism, 15.1% of all cases were classified as VP, the third most common form, with a prevalence of 1.1% in an elderly cohort. Another Brazilian survey found a prevalence of 2.3% of VP in the elderly. VP is usually the result of conventional vascular risk factors, particularly hypertension, leading to strategic infarcts of subcortical gray matter nuclei, diffuse white matter ischaemic lesions and less commonly, large vessel infarcts. Patients with VP tend to be older and present with gait difficulties, symmetrical predominant lower-body involvement, poor levodopa responsiveness, postural instability, falls, cognitive impairment and dementia, corticospinal findings, urinary incontinence and pseudobulbar palsy. This article intends to provide physicians with an insight on the practical issues of VP, a disease potentially confounded with vascular dementia, idiopathic Parkinson's disease, dementia with Lewy bodies and other secondary causes of Parkinsonism.

Differentiating Aging among Adults with Down Syndrome and Comorbid Dementia or Psychopathology

OpenAIRE

Esbensen, Anna J.; Johnson, Emily Boshkoff; Amaral, Joseph L.; Tan, Christine M.; Macks, Ryan

2016-01-01

Differences were examined between three groups of adults with Down syndrome in their behavioral presentation, social life/activities, health, and support needs. We compared those with comorbid dementia, with comorbid psychopathology, and with no comorbid conditions. Adults with comorbid dementia were more likely to be older, have lower functional abilities, have worse health and more health conditions, and need more support in self-care. Adults with comorbid psychopathology were more likely t...

A clinical study in elderly patients with Parkinson's disease using MRI and SPECT; Parkinson's disease and the lacunar state

Energy Technology Data Exchange (ETDEWEB)

Umahara, Takahiko; Iwamoto, Toshihiko; Takasaki, Masaru; Katsunuma, Hideyo [Tokyo Medical Coll. (Japan)

1991-11-01

Parkinson's disease (PD) is often associated with dementia in elderly patients, and sometimes PD coexists with senile dementia of the Alzheimer type (SDAT) or cerebrovascular disease (CVD) in the elderly. However, since there are few previous clinical studies on the coincidence of, or relationship between PD and CVD, the authors evaluated these aspects in 34 elderly patients with PD using MRI and SPECT. All the patients were over 70 years old. The diagnosis of PD was based on the presence of three symptons (resting tremor, cogwheel rigidity and bradikinesia) which are characteristic of PD, and the effectiveness of L-DOPA therapy. We therefore believe that patients with vascular Parkinsonism were excluded from our study. In 34 cases, 24 (71%) had MRI evidence of CVD (mainly the lacunar state). In the 10 cases who had no CVD, 2 (20%) had severe dementia and the decrease of regional cerebral blood flow (rCBF) in the temporal and parietal lobes bilaterally correlated with the SPECT findings commonly found in SDAT. A comparison of the rCBF and the results of Hasegawa's dementia score (HDS) (verbal intelligence score) was made between the patients with PD associated with CVD and the patients with PD who had no CVD and no SPECT findings which correlated with SDAT. The rCBF in the frontal lobes and the results of the HDS of the former group were significantly lower than those of the latter. As mentioned above, elderly patients with PD often had CVD, leading to dementia. We also pointed out the difficulty in making a differential diagnosis between PD with CVD and vascular Parkinsonism, and the necessity of whether or not the concept of 'mixed type Parkinsonism' should be considered. (author).

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Alternating Wolff-Parkinson-White syndrome associated with attack of angina

International Nuclear Information System (INIS)

Mangiafico, R.A.; Petralito, A.; Grimaldi, D.R.

1990-01-01

In a patient with Wolff-Parkinson-White syndrome and an inferior-posterior bypass tract, transient restoration of normal conduction occurred during an attack of angina. The ECG pattern of inferior posterior ischemia was present when the conduction was normal. Thallium scintigraphy showed a reversible posterolateral perfusion defect. The possible mechanisms for production of intermittent preexcitation are discussed

Alternating Wolff-Parkinson-White syndrome associated with attack of angina

Energy Technology Data Exchange (ETDEWEB)

Mangiafico, R.A.; Petralito, A.; Grimaldi, D.R. (Univ. of Catania (Italy))

1990-07-01

In a patient with Wolff-Parkinson-White syndrome and an inferior-posterior bypass tract, transient restoration of normal conduction occurred during an attack of angina. The ECG pattern of inferior posterior ischemia was present when the conduction was normal. Thallium scintigraphy showed a reversible posterolateral perfusion defect. The possible mechanisms for production of intermittent preexcitation are discussed.

Renal Failure in Dementia with Lewy Bodies Presenting as Catatonia

Directory of Open Access Journals (Sweden)

Robert Fekete

2013-01-01

Full Text Available Catatonia, originally described by Karl Kahlbaum in 1874, may be regarded as a set of clinical features found in a subtype of schizophrenia, but the syndrome may also stem from organic causes including vascular parkinsonism, brain masses, globus pallidus lesions, metabolic derangements, and pharmacologic agents, especially first generation antipsychotics. Catatonia may include paratonia, waxy flexibility (cerea flexibilitas, stupor, mutism, echolalia, and catalepsy (abnormal posturing. A case of catatonia as a result of acute renal failure in a patient with dementia with Lewy bodies is described. This patient recovered after intravenous fluid administration and reinstitution of the atypical dopamine receptor blocking agent quetiapine, but benzodiazepines and amantadine are additional possible treatments. Recognition of organic causes of catatonia leads to timely treatment and resolution of the syndrome.

Down Syndrome and Dementia: Is Depression a Confounder for Accurate Diagnosis and Treatment?

Science.gov (United States)

Wark, Stuart; Hussain, Rafat; Parmenter, Trevor

2014-01-01

The past century has seen a dramatic improvement in the life expectancy of people with Down syndrome. However, research has shown that individuals with Down syndrome now have an increased likelihood of early onset dementia. They are more likely than their mainstream peers to experience other significant co-morbidities including mental health…

Diagnosing Alzheimer's Dementia in Down Syndrome: Problems and Possible Solutions

Science.gov (United States)

Nieuwenhuis-Mark, Ruth E.

2009-01-01

It is widely accepted that people with Down syndrome are more likely than the general population to develop Alzheimer's dementia as they age. However, the diagnosis can be problematic in this population for a number of reasons. These include: the large intra-individual variability in cognitive functioning, the different diagnostic and…

Is There a Characteristic Clinical Profile for Patients with Dementia and Sundown Syndrome?

Science.gov (United States)

Angulo Sevilla, David; Carreras Rodríguez, María Teresa; Heredia Rodríguez, Patricia; Fernández Sánchez, Marisa; Vivancos Mora, José Aurelio; Gago-Veiga, Ana Beatriz

2018-01-01

Sundown syndrome (SS) is the onset or worsening of behavioral symptoms in the evening in patients with dementia. To identify the differential clinical profile of patients with dementia who present SS. A cross-sectional, case-control observational study was conducted by retrospectively reviewing the medical records of patients with dementia in a specialized Memory Unit. We compared the characteristics of patients with and without SS, including sociodemographic variables, etiology, and severity of the dementia, behavioral symptoms, sleep disorders (considering insomnia and hypersomnia), other diseases and treatments employed. We identified the factors related to SS and conducted a logistic regression analysis to establish a predictive nomogram. Of the 216 study patients with dementia, 41 (19%) had SS. There was a predominance of women (2.4:1), advanced age (p = 0.0001), dependence (p patients with dementia, with a predictive capacity of 80.1%. In our study, age, a higher score on the GDS, and the presence of insomnia or hypersomnia are differential clinical characteristics of patients with SS. We defined a nomogram that helps predicting the occurrence of SS in patients with dementia.

Low LDL cholesterol, PCSK9 and HMGCR genetic variation, and risk of Alzheimer's disease and Parkinson's disease

DEFF Research Database (Denmark)

Benn, Marianne; Nordestgaard, Børge G.; Frikke-Schmidt, Ruth

2017-01-01

 Risk of Alzheimer's disease, vascular dementia, all dementia, and Parkinson's disease.Results In observational analyses, the multifactorially adjusted hazard ratio for Parkinson's disease in participants with an LDL cholesterol level ....79), whereas the corresponding hazard ratios for Alzheimer's disease, vascular dementia, or any dementia did not differ from 1.0. PCSK9 and HMGCR variants combined were associated with a 9.3% lower LDL cholesterol level. In genetic, causal analyses adjusted for age, sex, and year of birth, the risk ratios...... for a lifelong 1 mmol/L lower LDL cholesterol level were 0.57 (0.27 to 1.17) for Alzheimer's disease, 0.81 (0.34 to 1.89) for vascular dementia, 0.66 (0.34 to 1.26) for any dementia, and 1.02 (0.26 to 4.00) for Parkinson's disease. Summary level data from the International Genomics of Alzheimer's Project using...

Exercise for Individuals with Lewy Body Dementia: A Systematic Review.

Directory of Open Access Journals (Sweden)

Michael Inskip

Full Text Available Individuals with Lewy body Dementia (LBD, which encompasses both Parkinson disease dementia (PDD and Dementia with Lewy Bodies (DLB experience functional decline through Parkinsonism and sedentariness exacerbated by motor, psychiatric and cognitive symptoms. Exercise may improve functional outcomes in Parkinson's disease (PD, and Alzheimer's disease (AD. However, the multi-domain nature of the LBD cluster of symptoms (physical, cognitive, psychiatric, autonomic results in vulnerable individuals often being excluded from exercise studies evaluating physical function in PD or cognitive function in dementia to avoid confounding results. This review evaluated existing literature reporting the effects of exercise interventions or physical activity (PA exposure on cluster symptoms in LBD.A high-sensitivity search was executed across 19 databases. Full-length articles of any language and quality, published or unpublished, that analysed effects of isolated exercise/physical activity on indicative Dementia with Lewy Bodies or PD-dementia cohorts were evaluated for outcomes inclusive of physical, cognitive, psychiatric, physiological and quality of life measures. The protocol for this review (Reg. #: CRD42015019002 is accessible at http://www.crd.york.ac.uk/PROSPERO/.111,485 articles were initially retrieved; 288 full articles were reviewed and 89.6% subsequently deemed ineligible due to exclusion of participants with co-existence of dementia and Parkinsonism. Five studies (1 uncontrolled trial, 1 randomized controlled trial and 3 case reports evaluating 16 participants were included. Interventions were diverse and outcome homogeneity was low. Habitual gait speed outcomes were measured in 13 participants and increased (0.18m/s, 95% CI -0.02, 0.38m/s, exceeding moderate important change (0.14m/s for PD cohorts. Other outcomes appeared to improve modestly in most participants.Scarce research investigating exercise in LBD exists. This review confirms

Progression of dopaminergic degeneration in dementia with Lewy bodies and Parkinson's disease with and without dementia assessed using 123I-FP-CIT SPECT

International Nuclear Information System (INIS)

Colloby, Sean J.; McKeith, Ian G.; O'Brien, John T.; Williams, E. David; Burn, David J.; Lloyd, Jim J.

2005-01-01

The objective of this study was to investigate the rate of progression of nigrostriatal dopaminergic loss in subjects with dementia with Lewy bodies (DLB), Parkinson's disease (PD) and PD with dementia (PDD) using serial 123 I-FP-CIT SPECT imaging. We hypothesised that striatal rates of decline in patients would be greater than in controls, and that DLB and PDD would show similar rates, reflecting the similarity in neurobiological mechanisms of dopaminergic loss between the two disorders. We studied 20 patients with DLB, 20 with PD, 15 with PDD and 22 healthy age-matched controls. Semi-automated region of interest (ROI) analysis was performed on both baseline and repeat scans for each subject and mean striatal uptake ratios (caudate, anterior and posterior putamen) were calculated. Rates of decline in striatal binding between groups were assessed using ANCOVA. Significant differences between patients and controls were observed in caudate (DLB, PD, PDD, p≤0.01), anterior putamen (DLB, PDD, p≤0.05; PD, p=0.07) and posterior putamen (DLB, PD, PDD, p<0.006). Rates of decline were similar between DLB, PD and PDD. (orig.)

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Sudden death in a young competitive athlete with Wolff-Parkinson-White syndrome

NARCIS (Netherlands)

Wiedermann, C. J.; Becker, A. E.; Hopferwieser, T.; Mühlberger, V.; Knapp, E.

1987-01-01

The case history is documented of a young competitive athlete known to have the electrocardiographic pattern of the Wolff-Parkinson-White syndrome, but considered asymptomatic. On that basis competitive sport was not proscribed. In retrospect, he had experienced occasional tachycardias which were of

ɑ-Synuclein strains and seeding in Parkinson's disease, incidental Lewy body disease, dementia with Lewy bodies and multiple system atrophy: similarities and differences.

Science.gov (United States)

Peelaerts, W; Bousset, L; Baekelandt, V; Melki, R

2018-04-27

Several age-related neurodegenerative disorders are characterized by the deposition of aberrantly folded endogenous proteins. These proteins have prion-like propagation and amplification properties but so far appear nontransmissible between individuals. Because of the features they share with the prion protein, PrP, the characteristics of pathogenic protein aggregates in several progressive brain disorders, including different types of Lewy body diseases (LBDs), such as Parkinson's disease (PD), multiple system atrophy (MSA) and dementia with Lewy bodies (DLB), have been actively investigated. Even though the pleomorphic nature of these syndromes might suggest different underlying causes, ɑ-synuclein (ɑSyn) appears to play an important role in this heterogeneous group of diseases (the synucleinopathies). An attractive hypothesis is that different types of ɑSyn protein assemblies have a unique and causative role in distinct synucleinopathies. We will discuss the recent research progress on ɑSyn assemblies involved in PD, MSA and DLB; their behavior as strains; current spreading hypotheses; their ability to seed centrally and peripherally; and their implication for disease pathogenesis.

Evaluating rivastigmine in mild-to-moderate Parkinson's disease dementia using ADAS-cog items.

Science.gov (United States)

Schmitt, Frederick A; Aarsland, Dag; Brønnick, Kolbjørn S; Meng, Xiangyi; Tekin, Sibel; Olin, Jason T

2010-08-01

Rivastigmine has been shown to improve cognition in patients with Parkinson's disease dementia (PDD). To further explore the impact of anticholinesterase therapy on PDD, Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog) items were assessed in a retrospective analysis of a 24-week, double-blind, placebo-controlled trial of rivastigmine. Mean changes from baseline at week 24 were calculated for ADAS-cog item scores and for 3 cognitive domain scores. A total of 362 patients were randomized to 3 to 12 mg/d rivastigmine capsules and 179 to placebo. Patients with PDD receiving rivastigmine improved versus placebo on items: word recall, following commands, ideational praxis, remembering test instructions, and comprehension of spoken language (P ADAS-cog is sensitive to broad cognitive changes in PDD. Overall, rivastigmine was associated with improvements on individual cognitive items and general cognitive domains.

FDG PET imaging dementia

Energy Technology Data Exchange (ETDEWEB)

Ahn, Byeong Cheol [Kyungpook National University Medical School and Kyungpook National University Hospital, Daegu (Korea, Republic of)

2007-04-15

Dementia is a major burden for many countries including South Korea, where life expectancy is continuously growing and the proportion of aged people is rapidly growing. Neurodegenerative disorders, such as, Alzheimer disease, dementia with Lewy bodies, frontotemporal dementia. Parkinson disease, progressive supranuclear palsy, corticobasal degeneration, Huntington disease, can cause dementia, and cerebrovascular disease also can cause dementia. Depression or hypothyroidism also can cause cognitive deficits, but they are reversible by management of underlying cause unlike the forementioned dementias. Therefore these are called pseudodementia. We are entering an era of dementia care that will be based upon the identification of potentially modifiable risk factors and early disease markers, and the application of new drugs postpone progression of dementias or target specific proteins that cause dementia. Efficient pharmacologic treatment of dementia needs not only to distinguish underlying causes of dementia but also to be installed as soon as possible. Therefore, differential diagnosis and early diagnosis of dementia are utmost importance. F-18 FDG PET is useful for clarifying dementing diseases and is also useful for early detection of the disease. Purpose of this article is to review the current value of FDG PET for dementing diseases including differential diagnosis of dementia and prediction of evolving dementia.

FDG PET imaging dementia

International Nuclear Information System (INIS)

Ahn, Byeong Cheol

2007-01-01

Dementia is a major burden for many countries including South Korea, where life expectancy is continuously growing and the proportion of aged people is rapidly growing. Neurodegenerative disorders, such as, Alzheimer disease, dementia with Lewy bodies, frontotemporal dementia. Parkinson disease, progressive supranuclear palsy, corticobasal degeneration, Huntington disease, can cause dementia, and cerebrovascular disease also can cause dementia. Depression or hypothyroidism also can cause cognitive deficits, but they are reversible by management of underlying cause unlike the forementioned dementias. Therefore these are called pseudodementia. We are entering an era of dementia care that will be based upon the identification of potentially modifiable risk factors and early disease markers, and the application of new drugs postpone progression of dementias or target specific proteins that cause dementia. Efficient pharmacologic treatment of dementia needs not only to distinguish underlying causes of dementia but also to be installed as soon as possible. Therefore, differential diagnosis and early diagnosis of dementia are utmost importance. F-18 FDG PET is useful for clarifying dementing diseases and is also useful for early detection of the disease. Purpose of this article is to review the current value of FDG PET for dementing diseases including differential diagnosis of dementia and prediction of evolving dementia

Electroconvulsive therapy-induced Wolff-Parkinson-White syndrome: a case report.

Science.gov (United States)

Enomoto, Shingo; Yoshino, Aihide; Takase, Bonpei; Kuwahara, Tatsuro; Tatsuzawa, Yasutaka; Nomura, Soichiro

2013-01-01

Wolff-Parkinson-White (WPW) syndrome is characterized by premature ventricular excitation due to the presence of an abnormal accessory pathway. Electrocardiography (ECG) of patients with WPW syndrome portrays a short PR interval and a wide QRS interval with a delta wave. Herein, we report the case of a patient with schizophrenia who developed a wide QRS interval with a delta wave immediately following electroconvulsive therapy (ECT). Initially, the delta wave disappeared within 2 days after ECT. However, the duration of the delta wave increased exponentially to 4 months when ECT was repeated. Although the patient's cardiocirculatory dynamics remained normal, we continued to monitor her ECG until the delta wave disappeared because WPW syndrome can lead to serious arrhythmia. Copyright © 2013 Elsevier Inc. All rights reserved.

Acute parietal lobe infarction presenting as Gerstmann’s syndrome and cognitive decline mimicking senile dementia

Directory of Open Access Journals (Sweden)

Chen TY

2013-07-01

Full Text Available Tien-Yu Chen,1 Chun-Yen Chen,1,3 Che-Hung Yen,2,3 Shin-Chang Kuo,1,3 Yi-Wei Yeh,1,3 Serena Chang,1 San-Yuan Huang1,31Department of Psychiatry, 2Department of Neurology, Tri-Service General Hospital, School of Medicine, National Defense Medical Center, 3Graduate Institute of Medical Sciences, National Defense Medical Center, Taipei, Taiwan, Republic of ChinaAbstract: Gerstmann’s syndrome encompasses the tetrad of finger agnosia, agraphia, acalculia, and right-left confusion. An elderly man with a history of several cardiovascular diseases was initially brought to the psychiatric outpatient department by his family because of worsening of recent memory, executive function, and mixed anxious-depressive mood. Gerstmann’s syndrome without obvious motor function impairment and dementia-like features could be observed at first. Emergent brain computed tomography scan revealed new left-middle cerebral artery infarction over the left posterior parietal lobe. This case reminds us that acute cerebral infarction involving the parietal lobe may present as Gerstmann’s syndrome accompanied by cognitive decline mimicking dementia. As a result, emergent organic workups should be arranged, especially for elderly patients at high risk for cerebral vascular accident.Keywords: Gerstmann’s syndrome, dementia, parietal lobe infarction

Patient with Wolff-Parkinson-White syndrome with intermittent pre-excitation under subarachnoid block for urological surgery

Science.gov (United States)

Garg, Rakesh; Sinha, Renu; Nishad, PK

2011-01-01

Wolff-Parkinson-White (WPW) syndrome is one of the pre-excitation syndromes in which activation of an accessory atrioventricular (AV) conduction pathway leads to bypass the AV node and cause earlier ventricular activation than the normal pathway. We report a patient with intermittent WPW syndrome who repeatedly manifested pre-excitation after subarachnoid block. PMID:21712875

Quantitative neuropathological study of Alzheimer-type pathology in the hippocampus: comparison of senile dementia of Alzheimer type, senile dementia of Lewy body type, Parkinson's disease and non-demented elderly control patients.

Science.gov (United States)

Ince, P; Irving, D; MacArthur, F; Perry, R H

1991-12-01

A Lewy body dementing syndrome in the elderly has been recently described and designated senile dementia of Lewy body type (SDLT) on the basis of a distinct clinicopathological profile. The pathological changes seen in SDLT include the presence of cortical Lewy bodies (LB) frequently, but not invariably, associated with senile plaque (SP) formation. Whilst neocortical neurofibrillary tangles (NFT) are sparse or absent, a proportion of these cases show involvement of the temporal archicortex by lesions comprising Alzheimer-type pathology (ATP, i.e. NFT, SP and granulovacuolar degeneration [GVD]). Thus the relationship between SDLT and senile dementia of Alzheimer type (SDAT) is complex and controversial. In this study quantitative neuropathology was used to compare the intensity and distribution of ATP in the hippocampus and entorhinal cortex of 53 patients from 3 disease groups (SDLT, SDAT, Parkinson's disease (PD)) and a group of neurologically and mentally normal elderly control patients. For most brain areas examined the extent of ATP between the patient groups followed the trend SDAT greater than SDLT greater than PD greater than control. Statistical comparison of these groups revealed significant differences between the mean densities of NFT, SP and GVD although individual cases showed considerable variability. These results confirm additional pathological differences between SDAT and SDLT regarding the intensity of involvement of the temporal archicortex by ATP. Many patients with Lewy body disorders (LBdis) show a predisposition to develop ATP albeit in a more restricted distribution (e.g. low or absent neocortical NFT) and at lower densities than is found in SDAT. Some cases of SDLT show minimal SP and NFT formation in both neocortex and archicortex supporting previously published data distinguishing this group from Alzheimer's disease.

Comparing Clinical Profiles in Alzheimer's Disease and Parkinson's Disease Dementia

Directory of Open Access Journals (Sweden)

Martin R. Farlow

2013-09-01

Full Text Available Background: Greater understanding of differences in baseline impairment and disease progression in patients with Alzheimer's disease (AD and Parkinson's disease dementia (PDD may improve the interpretation of drug effects and the design of future studies. Methods: This was a retrospective analysis of three randomized, double-blind rivastigmine databases (one in PDD, two in AD. Impairment on the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog, Alzheimer's Disease Cooperative Study-Activities of Daily Living (ADCS-ADL scale, 10-item Neuropsychiatric Inventory (NPI-10 and the ADCS-Clinical Global Impression of Change (CGIC was compared [standardized difference (Cohen's d, similar if Results: Patients with AD or PDD had similar levels of impairment on the ADAS-cog and NPI-10. Scores on the ADCS-ADL scale (standardized difference = 0.47 and the ADAS-cog memory domain (total, 0.33; items, 0.10-0.58 were higher in AD; PDD patients were more impaired in the language (0.23 and praxis (0.34 domains. AD patients receiving placebo showed greater deterioration on the ADAS-cog (0.14 and improvement on the NPI-10 (0.11 compared with patients with PDD. Conclusion: Differing patterns of impairment occur in AD and PDD.

Comparing clinical profiles in Alzheimer's disease and Parkinson's disease dementia.

Science.gov (United States)

Farlow, Martin R; Schmitt, Frederick; Aarsland, Dag; Grossberg, George T; Somogyi, Monique; Meng, Xiangyi

2013-01-01

Greater understanding of differences in baseline impairment and disease progression in patients with Alzheimer's disease (AD) and Parkinson's disease dementia (PDD) may improve the interpretation of drug effects and the design of future studies. This was a retrospective analysis of three randomized, double-blind rivastigmine databases (one in PDD, two in AD). Impairment on the Alzheimer's Disease Assessment Scale-cognitive subscale (ADAS-cog), Alzheimer's Disease Cooperative Study-Activities of Daily Living (ADCS-ADL) scale, 10-item Neuropsychiatric Inventory (NPI-10) and the ADCS-Clinical Global Impression of Change (CGIC) was compared [standardized difference (Cohen's d), similar if <0.1]. Patients with AD or PDD had similar levels of impairment on the ADAS-cog and NPI-10. Scores on the ADCS-ADL scale (standardized difference = 0.47) and the ADAS-cog memory domain (total, 0.33; items, 0.10-0.58) were higher in AD; PDD patients were more impaired in the language (0.23) and praxis (0.34) domains. AD patients receiving placebo showed greater deterioration on the ADAS-cog (0.14) and improvement on the NPI-10 (0.11) compared with patients with PDD. Differing patterns of impairment occur in AD and PDD.

Spinal Anaesthesia is Safe in a Patient with Wolff-Parkinson-White Syndrome Undergoing Evacuation of Molar Pregnancy.

Science.gov (United States)

Deviseti, Pravalika; Pujari, Vinayak S

2016-02-01

Wolff-Parkinson-White (WPW) syndrome is an uncommon cardiac condition where there is an abnormal band of atrial tissue connecting atria and ventricles which can electrically bypass atrioventricular node. The anaesthetic management in these patients is challenging as life threatening complications can occur perioperatively like paroxysmal supraventricular tachycardia and atrial fibrillation. Also, regional anaesthetic technique like subarachnoid block is a safe and cost effective alternative to general anaesthesia as it avoids polypharmacy. We report the successful anaesthetic management of Wolff Parkinson White syndrome in a primi with hydatiform mole posted for suction and evacuation.

Patient with Wolff-Parkinson-White syndrome with intermittent pre-excitation under subarachnoid block for urological surgery

Directory of Open Access Journals (Sweden)

Rakesh Garg

2011-01-01

Full Text Available Wolff-Parkinson-White (WPW syndrome is one of the pre-excitation syndromes in which activation of an accessory atrioventricular (AV conduction pathway leads to bypass the AV node and cause earlier ventricular activation than the normal pathway. We report a patient with intermittent WPW syndrome who repeatedly manifested pre-excitation after subarachnoid block.

Expanding spectrum of contactin-associated protein 2 (CASPR2) autoimmunity-syndrome of parkinsonism and ataxia.

Science.gov (United States)

Kannoth, Sudheeran; Nambiar, Vivek; Gopinath, Siby; Anandakuttan, Anandkumar; Mathai, Annamma; Rajan, Parvathy Kanjiramana

2018-03-01

Contactin-associated protein 2 (CASPR2) antibodies are originally associated with Morvan's syndrome and peripheral nerve hyper excitability. Our objective was to study retrospectively the clinical spectrum of CASPR2 antibody-positive patients in our hospital. This is a retrospective observational study. Patients treated at the Amrita Institute of Medical Sciences from May 2013 to April 2016, who were tested positive for CASPR2 antibodies, were included. A total of 1584 samples were tested in the neuroimmunology laboratory during the study period for voltage-gated potassium channel (VGKC) complex antibodies-leucine-rich glioma-inactivated protein 1 (LGI1) and CASPR2 antibodies. Thirty-four were positive for LGI1, 13 were positive for CASPR2, and 7 were for both (total 54-3.4% positivity). Of these 54 cases, 11 were treated in our hospital. Seven were positive for LGI1, three for CASPR2, and one for both. The patient who had both CASPR2 and LGI1 antibody positive had Morvan's syndrome. One patient with CASPR2 had neuromyotonia. The other patient was admitted with status epilepticus with a syndrome of parkinsonism and ataxia. The third patient had encephalopathy and myoclonus with a syndrome of parkinsonism and ataxia. Two of them underwent siddha treatment for other ailments prior to the onset of the disease for other ailments. Our short series shows the expanding spectrum of CASPR2 autoimmunity. Syndrome of parkinsonism and ataxia is an important manifestation of CASPR2 autoimmunity where we can offer a definitive treatment.

Evolution of diagnostic criteria and assessments for Parkinson's disease mild cognitive impairment.

Science.gov (United States)

Goldman, Jennifer G; Holden, Samantha K; Litvan, Irene; McKeith, Ian; Stebbins, Glenn T; Taylor, John-Paul

2018-04-01

Mild cognitive impairment has gained recognition as a construct and a potential prodromal stage to dementia in both Alzheimer's disease and Parkinson's disease (PD). Although mild cognitive impairment has been recognized in the Alzheimer's disease field, it is a relatively more recent topic of interest in PD. Recent advances include the development of diagnostic criteria for PD mild cognitive impairment to provide more uniform definitions for clinical and research use. Studies reveal that mild cognitive impairment in PD is frequent, but also heterogeneous, with variable clinical presentations, differences in its progression to dementia, and likely differences in underlying pathophysiology. Application of the International Parkinson and Movement Disorder Society PD Mild Cognitive Impairment Task Force diagnostic criteria has provided insights regarding cognitive measures, functional assessments, and other key topics that may require additional refinement. Furthermore, it is important to consider definitions of PD mild cognitive impairment in the landscape of other related Lewy body disorders, such as dementia with Lewy bodies, and in the context of prodromal and early-stage PD. This article examines the evolution of mild cognitive impairment in concept and definition, particularly in PD, but also in related disorders such as Alzheimer's disease and dementia with Lewy bodies; the development and application of International Parkinson and Movement Disorder Society PD Mild Cognitive Impairment diagnostic criteria; and insights and future directions for the field of PD mild cognitive impairment. © 2018 International Parkinson and Movement Disorder Society. © 2018 International Parkinson and Movement Disorder Society.

Regional cerebral blood flow in SPECT pattern in Parkinson's disease

International Nuclear Information System (INIS)

Lenart-Jankowska, D.; Junik, R.; Sowinski, J.; Gembicki, M.; Wender, M.

1997-01-01

The purpose of our work was to compare the regional cerebral blood flow (rCBF) in SPECT examination in Parkinson's disease with (17 cases) and without (7 cases) dementia and in various clinical stages of the disease. The patients underwent SPECT examination 5-40 min after intravenous application of HMPAO (Ceretec, Amersham) with 740 Mbq (20 mCi) pertechnate 99m Tc. SPECT was performed with a Siemens Diacam single-head rotating gamma camera coupled to a high resolution collimator and Icon computer system provided by the manufacturer. The results were defined in relative values of ROI in relation to cerebellum. Patients with Parkinson's disease showed hypoperfusion in cerebral lobes and in deep cerebral structures including the basal ganglia. Regional perfusion deficit in SPECT was seen with and without associated dementia and already in early stage of the disease. Parkinson's disease is provoked by the lesions of dopaminergic neurons of the central nervous system leading to domination of extrapyramidal symptoms. There are many indications that also the neurotransmitters associated with cognitive functions as acetylcholine demonstrate some abnormalities. However, only in some cases of Parkinson's disease dementia is the dominating symptom. Our results of regional cerebral blood flow testify that in Parkinson's disease the dysfunction of the central nervous system is more diffuse than has previously been suggested. (author)

Metabolic topography of Parkinsonism

Energy Technology Data Exchange (ETDEWEB)

Kim, Jae Seung [Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

2007-04-15

Parkinson's disease is one of the most frequent neurodegenerative diseases, which mainly affects the elderly. Parkinson's disease is often difficult to differentiate from atypical parkinson disorder such as progressive supranuclear palsy, multiple system atrophy, dementia with Lewy body, and corticobasal ganglionic degeneration, based on the clinical findings because of the similarity of phenotypes and lack of diagnostic markers. The accurate diagnosis of Parkinson's disease and atypical Parkinson disorders is not only important for deciding on treatment regimens and providing prognosis, but also it is critical for studies designed to investigate etiology and pathogenesis of parkinsonism and to develop new therapeutic strategies. Although degeneration of the nigrostriatal dopamine system results in marked loss of striatal dopamine content in most of the diseases causing parkinsonism, pathologic studies revealed different topographies of the neuronal cell loss in Parkinsonism. Since the regional cerebral glucose metabolism is a marker of integrated local synaptic activity and as such is sensitive to both direct neuronal/synaptic damage and secondary functional disruption at synapses distant from the primary site of pathology, and assessment of the regional cerebral glucose metabolism with F-18 FDG PET is useful in the differential diagnosis of parkinsonism and evaluating the pathophysiology of Parkinsonism.

Metabolic topography of Parkinsonism

International Nuclear Information System (INIS)

Kim, Jae Seung

2007-01-01

Parkinson's disease is one of the most frequent neurodegenerative diseases, which mainly affects the elderly. Parkinson's disease is often difficult to differentiate from atypical parkinson disorder such as progressive supranuclear palsy, multiple system atrophy, dementia with Lewy body, and corticobasal ganglionic degeneration, based on the clinical findings because of the similarity of phenotypes and lack of diagnostic markers. The accurate diagnosis of Parkinson's disease and atypical Parkinson disorders is not only important for deciding on treatment regimens and providing prognosis, but also it is critical for studies designed to investigate etiology and pathogenesis of parkinsonism and to develop new therapeutic strategies. Although degeneration of the nigrostriatal dopamine system results in marked loss of striatal dopamine content in most of the diseases causing parkinsonism, pathologic studies revealed different topographies of the neuronal cell loss in Parkinsonism. Since the regional cerebral glucose metabolism is a marker of integrated local synaptic activity and as such is sensitive to both direct neuronal/synaptic damage and secondary functional disruption at synapses distant from the primary site of pathology, and assessment of the regional cerebral glucose metabolism with F-18 FDG PET is useful in the differential diagnosis of parkinsonism and evaluating the pathophysiology of Parkinsonism

Noninvasive Localization of Accessory Pathways in Wolff-Parkinson-White Syndrome by Three-Dimensional Speckle Tracking Echocardiography.

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Ishizu, Tomoko; Seo, Yoshihiro; Igarashi, Miyako; Sekiguchi, Yukio; Machino-Ohtsuka, Tomoko; Ogawa, Kojiro; Kuroki, Kenji; Yamamoto, Masahiro; Nogami, Akihiko; Kawakami, Yasushi; Aonuma, Kazutaka

2016-06-01

We have developed a noninvasive isochrone activation imaging (AI) system with 3-dimensional (3D) speckle tracking echocardiography (STE), which allows visualization of the wavefront image of mechanical propagation of the accessory pathway (ACP) in Wolff-Parkinson-White syndrome. Patients with manifest Wolff-Parkinson-White syndrome were imaged in 3D-STE AI mode, which quantified the time from QRS onset to regional endocardial deformation. In 2 patients with left- and right-side ACP, we confirmed that intraoperative contact endocardial electric mapping and the 3D-STE AI system showed comparable images pre- and postablation. In normal heart assessment by 3D-echo AI, the earliest activation sites were found at the attachment of the papillary muscles in the left ventricle and midseptum in the right ventricle, and none showed earliest activation at the peri-atrioventricular valve annuli. An analyzer who was unaware of the clinical information assessed 39 ACP locations in 38 Wolff-Parkinson-White syndrome patients using 3D-STE. All showed abnormal perimitral or tricuspid annular activations, and the location of 34 ACP (87%) showed agreement with the successful ablation sites within a 2-o'clock range. Especially for left free wall ACP, 17/18 (94%) showed consistency with the ablation site within a 2 o'clock range. Among 15 ACP at the ventricular septum, 9 (60%) showed early local activation in both right and left sides of the septum. Isochrone AI with 3D-STE may be a promising noninvasive imaging tool to assess cardiac synchronized activation in normal hearts and detect abnormal breakthrough of mechanical activation from both atrioventricular annuli in Wolff-Parkinson-White syndrome. © 2016 American Heart Association, Inc.

The Role of Insulin/IGF-1/PI3K/Akt/GSK3β Signaling in Parkinson's Disease Dementia

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Liying Yang

2018-02-01

Full Text Available Dementia, a condition that frequently afflicts patients in advanced stages of Parkinson's disease (PD, results in decreased quality of life and survival time. Nevertheless, the pathological mechanisms underlying Parkinson's disease dementia (PDD are not completely understood. The symptoms characteristic of PDD may be the result of functional and structural deficiencies. The present study implicates the accumulation of Lewy bodies in the cortex and limbic system as a potent trigger in the development of PDD. In addition, significant Alzheimer-type pathologies, including amyloid-β (Aβ plaques and NFTs, are observed in almost half of PDD patients. Interestingly, links between PDD pathogenesis and the mechanisms underlying the development of insulin resistance have begun to emerge. Furthermore, previous studies have demonstrated that insulin treatment reduces amyloid plaques in Alzheimer's disease (AD, and normalizes the production and functionality of dopamine and ameliorates motor impairments in 6-OHDA-induced rat PD models. GSK3β, a downstream substrate of PI3K/Akt signaling following induction by insulin and IGF-1, exerts an influence on AD and PD physiopathology. The genetic overexpression of GSK3β in cortex and hippocampus results in signs of neurodegeneration and spatial learning deficits in in vivo models (Lucas et al., 2001, whereas its inhibition results in improvements in cognitive impairment in these rodents, including AD and PD. Accordingly, insulin- or IGF-1-activated PI3K/Akt/GSK3β signaling may be involved in PDD pathogenesis, at least in the pathology of PD-type + AD-type.

Parkinsonism in fragile X-associated tremor/ataxia syndrome (FXTAS): revisited.

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Niu, Yu-Qiong; Yang, Jin-Chen; Hall, Deborah A; Leehey, Maureen A; Tassone, Flora; Olichney, John M; Hagerman, Randi J; Zhang, Lin

2014-04-01

Parkinsonian features have been used as a minor diagnostic criterion for fragile X-associated tremor/ataxia syndrome (FXTAS). However, prior studies have examined parkinsonism (defined as having bradykinesia with at least rest tremor or postural instability) mostly in premutation carriers without a diagnosis of FXTAS. The current study was intended to elaborate this important aspect of the FXTAS spectrum, and to quantify the relationships between parkinsonism, FXTAS clinical staging and genetic/molecular measures. Thirty eight (38) FXTAS patients and 10 age-matched normal controls underwent a detailed neurological examination that included all but one item (i.e. rigidity) of the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS). The FXTAS patient group displayed substantially higher prevalence of parkinsonian features including body bradykinesia (57%) and rest tremor (26%), compared to the control group. Furthermore, parkinsonism was identified in 29% of FXTAS patients. Across all patients, body bradykinesia scores significantly correlated with FXTAS clinical stage, FMR1 mRNA level, and ataxic gait of cerebellar origin, while postural instability was associated with intention tremor. Parkinsonian features in FXTAS appear to be characterized as bradykinesia concurrent with cerebellar gait ataxia, postural instability accompanied by intention tremor, and frequent rest tremor, representing distinctive patterns that highlight the need for further clinical studies including genetic testing for the FMR1 premutation. The association between FMR1 mRNA level and bradykinesia implicates pathophysiological mechanisms which may link FMR1 mRNA toxicity, dopamine deficiency and parkinsonism in FXTAS. Copyright © 2014 Elsevier Ltd. All rights reserved.

Correlative study of the brain CT and clinical features of patients with Down's syndrome in three clinical stages of Alzheimer type dementia

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Maruyama, Keiko; Ikeda, Shu-ichi; Yanagisawa, Nobuo.

1995-01-01

Patients with Down's syndrome often develop Alzheimer type neuropathological changes as well as dementia of the Alzheimer type after the age of 40. We studied brain CT findings in relation to three clinical stages of Alzheimer type dementia in 11 patients with Down's syndrome aged from 17 to 55 years. In addition, 123 I-IMP-SPECT was studied in 4 of these patients. Dementia of the Alzheimer type was present in 9 patients; 5 patients were in the early stage, 2 were in the progressive stage, and the other 2 were in the end stage. The earliest CT finding was enlargement of the suprasellar cistern, which indicated atrophy of the medial temporal lobe including the hippocampus and amygdala. This finding was not present in non-demented individuals with Down's syndrome. Moreover, CT scans showed that brain atrophy progressed to the temporal, frontal lobe, and then generalized cerebral cortices, which correlated clinically with the severity of dementia. Studies of 123 I-IMP-SPECT in two patients with mild dementia revealed abnormally decreased isotope uptake in the temporal and posterior parietal regions. We suggest to measure the size of the suprasellar cistern in CT and SPECT scans for early detection and diagnosis of mild dementia of the Alzheimer type in patients with Down's syndrome. (author)

The Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) Scale: Comprehensive Assessment of Psychopathology in Down Syndrome

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Dekker, Alain D.; Sacco, Silvia; Carfi, Angelo; Benejam, Bessy; Vermeiren, Yannick; Beugelsdijk, Gonny; Schippers, Mieke; Hassefras, Lyanne; Eleveld, José; Grefelman, Sharina; Fopma, Roelie; Bomer-Veenboer, Monique; Boti, Mariángeles; Oosterling, G. Danielle E.; Scholten, Esther; Tollenaere, Marleen; Checkley, Laura; Strydom, André; Van Goethem, Gert; Onder, Graziano; Blesa, Rafael; zu Eulenburg, Christine; Coppus, Antonia M.W.; Rebillat, Anne-Sophie; Fortea, Juan; De Deyn, Peter P.

2018-01-01

People with Down syndrome (DS) are prone to develop Alzheimer’s disease (AD). Behavioral and psychological symptoms of dementia (BPSD) are core features, but have not been comprehensively evaluated in DS. In a European multidisciplinary study, the novel Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) scale was developed to identify frequency and severity of behavioral changes taking account of life-long characteristic behavior. 83 behavioral items in 12 clinically defined sections were evaluated. The central aim was to identify items that change in relation to the dementia status, and thus may differentiate between diagnostic groups. Structured interviews were conducted with informants of persons with DS without dementia (DS, n = 149), with questionable dementia (DS+Q, n = 65), and with diagnosed dementia (DS+AD, n = 67). First exploratory data suggest promising interrater, test-retest, and internal consistency reliability measures. Concerning item relevance, group comparisons revealed pronounced increases in frequency and severity in items of anxiety, sleep disturbances, agitation & stereotypical behavior, aggression, apathy, depressive symptoms, and eating/drinking behavior. The proportion of individuals presenting an increase was highest in DS+AD, intermediate in DS+Q, and lowest in DS. Interestingly, among DS+Q individuals, a substantial proportion already presented increased anxiety, sleep disturbances, apathy, and depressive symptoms, suggesting that these changes occur early in the course of AD. Future efforts should optimize the scale based on current results and clinical experiences, and further study applicability, reliability, and validity. Future application of the scale in daily care may aid caregivers to understand changes, and contribute to timely interventions and adaptation of caregiving. PMID:29689719

The Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) Scale: Comprehensive Assessment of Psychopathology in Down Syndrome.

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Dekker, Alain D; Sacco, Silvia; Carfi, Angelo; Benejam, Bessy; Vermeiren, Yannick; Beugelsdijk, Gonny; Schippers, Mieke; Hassefras, Lyanne; Eleveld, José; Grefelman, Sharina; Fopma, Roelie; Bomer-Veenboer, Monique; Boti, Mariángeles; Oosterling, G Danielle E; Scholten, Esther; Tollenaere, Marleen; Checkley, Laura; Strydom, André; Van Goethem, Gert; Onder, Graziano; Blesa, Rafael; Zu Eulenburg, Christine; Coppus, Antonia M W; Rebillat, Anne-Sophie; Fortea, Juan; De Deyn, Peter P

2018-01-01

People with Down syndrome (DS) are prone to develop Alzheimer's disease (AD). Behavioral and psychological symptoms of dementia (BPSD) are core features, but have not been comprehensively evaluated in DS. In a European multidisciplinary study, the novel Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) scale was developed to identify frequency and severity of behavioral changes taking account of life-long characteristic behavior. 83 behavioral items in 12 clinically defined sections were evaluated. The central aim was to identify items that change in relation to the dementia status, and thus may differentiate between diagnostic groups. Structured interviews were conducted with informants of persons with DS without dementia (DS, n = 149), with questionable dementia (DS+Q, n = 65), and with diagnosed dementia (DS+AD, n = 67). First exploratory data suggest promising interrater, test-retest, and internal consistency reliability measures. Concerning item relevance, group comparisons revealed pronounced increases in frequency and severity in items of anxiety, sleep disturbances, agitation & stereotypical behavior, aggression, apathy, depressive symptoms, and eating/drinking behavior. The proportion of individuals presenting an increase was highest in DS+AD, intermediate in DS+Q, and lowest in DS. Interestingly, among DS+Q individuals, a substantial proportion already presented increased anxiety, sleep disturbances, apathy, and depressive symptoms, suggesting that these changes occur early in the course of AD. Future efforts should optimize the scale based on current results and clinical experiences, and further study applicability, reliability, and validity. Future application of the scale in daily care may aid caregivers to understand changes, and contribute to timely interventions and adaptation of caregiving.

There is no Parkinson disease.

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Weiner, William J

2008-06-01

The term Parkinson disease defines a specific clinical condition characterized by a typical history and characteristic signs. This review examines the historical evolution of the concept of Parkinson disease and how the misunderstanding of Parkinson disease may be hindering clinical research trials. It is proposed that this syndrome be called Parkinson diseases or parkinsonism type 1 through infinity.

Functional Neuroimaging in Dementia

NARCIS (Netherlands)

J.M. Papma (Janne)

2012-01-01

textabstractDementia refers to a clinical syndrome of cognitive deterioration and difficulty in the performance of activities of daily living. The most common cause of dementia is Alzheimer’s disease (AD), followed by vascular dementia (VaD) at old age and frontotemporal dementia (FTD) at young

Molecular Pathogenesis of Familial Wolff-Parkinson-White Syndrome.

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Licht, Miyamotoa

2018-01-01

Familial Wolff-Parkinson-White (WPW) syndrome is an autosomal dominant inherited disease and consists of a small percentage of WPW syndrome which exhibits ventricular pre-excitation by development of accessory atrioventricular pathway. A series of mutations in PRKAG2 gene encoding gamma2 subunit of 5'AMP-activated protein kinase (AMPK) has been identified as the cause of familial WPW syndrome. AMPK is one of the most important metabolic regulators of carbohydrates and lipids in many types of tissues including cardiac and skeletal muscles. Patients and animals with the mutation in PRKAG2 gene exhibit aberrant atrioventricular conduction associated with cardiac glycogen overload. Recent studies have revealed "novel" significance of canonical pathways leading to glycogen synthesis and provided us profound insights into molecular mechanism of the regulation of glycogen metabolism by AMPK. This review focuses on the molecular basis of the pathogenesis of cardiac abnormality due to PRKAG2 mutation and will provide current overviews of the mechanism of glycogen regulation by AMPK. J. Med. Invest. 65:1-8, February, 2018.

Imaging biomarkers in Parkinson's disease and Parkinsonian syndromes: current and emerging concepts.

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Saeed, Usman; Compagnone, Jordana; Aviv, Richard I; Strafella, Antonio P; Black, Sandra E; Lang, Anthony E; Masellis, Mario

2017-01-01

Two centuries ago in 1817, James Parkinson provided the first medical description of Parkinson's disease, later refined by Jean-Martin Charcot in the mid-to-late 19th century to include the atypical parkinsonian variants (also termed, Parkinson-plus syndromes). Today, Parkinson's disease represents the second most common neurodegenerative disorder with an estimated global prevalence of over 10 million. Conversely, atypical parkinsonian syndromes encompass a group of relatively heterogeneous disorders that may share some clinical features with Parkinson's disease, but are uncommon distinct clinicopathological diseases. Decades of scientific advancements have vastly improved our understanding of these disorders, including improvements in in vivo imaging for biomarker identification. Multimodal imaging for the visualization of structural and functional brain changes is especially important, as it allows a 'window' into the underlying pathophysiological abnormalities. In this article, we first present an overview of the cardinal clinical and neuropathological features of, 1) synucleinopathies: Parkinson's disease and other Lewy body spectrum disorders, as well as multiple system atrophy, and 2) tauopathies: progressive supranuclear palsy, and corticobasal degeneration. A comprehensive presentation of well-established and emerging imaging biomarkers for each disorder are then discussed. Biomarkers for the following imaging modalities are reviewed: 1) structural magnetic resonance imaging (MRI) using T1, T2, and susceptibility-weighted sequences for volumetric and voxel-based morphometric analyses, as well as MRI derived visual signatures, 2) diffusion tensor MRI for the assessment of white matter tract injury and microstructural integrity, 3) proton magnetic resonance spectroscopy for quantifying proton-containing brain metabolites, 4) single photon emission computed tomography for the evaluation of nigrostriatal integrity (as assessed by presynaptic dopamine

Is Apolipoprotein E4 an Important Risk Factor for Dementia in Persons with Down Syndrome?

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Rohn, Troy T; McCarty, Katie L; Love, Julia E; Head, Elizabeth

2014-12-08

Down syndrome is one of the most common genetic causes of intellectual disability and is characterized by a number of behavioral as well as cognitive symptoms. Triplication of all or part of human chromosome 21 has been considered as the main cause of Down syndrome. Due to the location of the amyloid precursor protein on chromosome 21, many of the neuropathological features of early-onset Alzheimer's disease including senile plaques and neurofibrillary tangles are also present in Down syndrome patients who are either demented or nondemented. Significant advances in medical treatment have increased longevity in people with Down syndrome resulting in an increased population that may be subjected to many of the same risk factors as those with Alzheimer's disease. It is well established that harboring one or both apolipoprotein E4 alleles greatly increases the risk for Alzheimer's disease. However, whether apolipoprotein E4 contributes to an earlier onset of dementia or increased mortality in Down syndrome patients is still a matter of debate. The purpose of this mini review is to provide an updated assessment on apolipoprotein E4 status and risk potential of developing dementia and mortality associated with Down syndrome.

A rivastigmine patch for the treatment of Alzheimer's disease and Parkinson's disease dementia.

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Cummings, Jeffrey; Winblad, Bengt

2007-11-01

Rivastigmine patch is the first transdermal treatment to be approved for Alzheimer's disease (AD) and Parkinson's disease dementia in the USA and for AD in Europe. It provides smooth, continuous drug delivery, and has the potential to maintain rivastigmine concentrations within an optimal therapeutic window while avoiding the peaks and troughs associated with oral drug delivery. The target dose, rivastigmine 9.5 mg/24 h patch (a 10 cm(2) patch), is given once daily and requires a simple one-step dose titration to the therapeutic dose. In a 24-week study in 1195 AD patients, the rivastigmine 9.5 mg/24 h patch provided similar efficacy to the highest dose range of capsules, with approximately three-times fewer reports of nausea and vomiting. Patients in the 9.5 mg/24 h patch and 12 mg/day capsule groups evidenced significant improvements versus placebo on both primary outcome measures: the Alzheimer's Disease Assessment Scale-Cognitive subscale; and Alzheimer's Disease Cooperative Study-Clinical Global Impression of Change; in addition to the following secondary outcome measures: Alzheimer's Disease Cooperative Study-Activities of Daily Living scale; Mini-Mental State Examination; and Trail Making Test Part A for assessment of attention, visual tracking and motor processing speed. Treatment differences on the Neuropsychiatric Inventory and Ten Point Clock-drawing Test did not reach statistical significance in this study. The patch may be the optimal way to treat dementia patients with rivastigmine.

Efficacy of rivastigmine for cognitive symptoms in Parkinson disease with dementia.

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Almaraz, Amy C; Driver-Dunckley, Erika D; Woodruff, Bryan K; Wellik, Kay E; Caselli, Richard J; Demaerschalk, Bart M; Adler, Charles H; Caviness, John N; Wingerchuk, Dean M

2009-07-01

Impairment of multiple neurotransmitter networks, including acetylcholine, may contribute to the cognitive impairment in patients with Parkinson disease with dementia (PDD). Therefore, cholinesterase inhibitors might improve cognitive function in PDD. On the other hand, enhancing cholinergic function could plausibly worsen features of parkinsonism. To determine if oral cholinesterase inhibitors improve measures of cognitive outcome and are tolerated by people with PDD. We addressed the question through the development of a critically appraised topic. Participants included consultant and resident neurologists, clinical epidemiologists, a medical librarian, and behavioral neurology and movement disorder specialists. Participants began with a structured clinical question, devised search strategies, compiled the best evidence, performed a critical appraisal, summarized the evidence, provided commentary, and declared bottom-line conclusions. A randomized controlled trial (n = 541) showed that, compared with placebo, rivastigmine (mean, 8.6 mg/d) significantly improved scores on 2 coprimary cognitive outcome scales in PDD, including the Alzheimer disease Cooperative Study-Clinician's Global Impression of Change. When dichotomized to evaluate clinically significant benefit (moderate or marked improvement), this outcome was not significant (risk difference = 5.3%; 95% confidence interval (CI) = -1.6 to 12.1). The number needed to treat (NNT) to avoid clinically significant worsening of cognition was 10 (95% CI = 6-28). The NNT for the combined outcome of either achieving clinically significant benefit or avoiding significant worsening was 7. The numbers needed to harm for cholinergic side effects were 9 (95% CI = 5-24) for parkinsonian symptoms and 11 (95% CI = 6-32) for rivastigmine discontinuation due to any side effect. Rivastigmine therapy for PDD is associated with significant tradeoffs in efficacy and adverse effects. Carefully monitored trials of rivastigmine may

Ethnopharmacological Approaches for Dementia Therapy and Significance of Natural Products and Herbal Drugs

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Devesh Tewari

2018-02-01

Full Text Available Dementia is a clinical syndrome wherein gradual decline of mental and cognitive capabilities of an afflicted person takes place. Dementia is associated with various risk factors and conditions such as insufficient cerebral blood supply, toxin exposure, mitochondrial dysfunction, oxidative damage, and often coexisting with some neurodegenerative disorders such as Alzheimer's disease (AD, Huntington's disease (HD, and Parkinson's disease (PD. Although there are well-established (semi-synthetic drugs currently used for the management of AD and AD-associated dementia, most of them have several adverse effects. Thus, traditional medicine provides various plant-derived lead molecules that may be useful for further medical research. Herein we review the worldwide use of ethnomedicinal plants in dementia treatment. We have explored a number of recognized databases by using keywords and phrases such as “dementia”, “Alzheimer's,” “traditional medicine,” “ethnopharmacology,” “ethnobotany,” “herbs,” “medicinal plants” or other relevant terms, and summarized 90 medicinal plants that are traditionally used to treat dementia. Moreover, we highlight five medicinal plants or plant genera of prime importance and discuss the physiological effects, as well as the mechanism of action of their major bioactive compounds. Furthermore, the link between mitochondrial dysfunction and dementia is also discussed. We conclude that several drugs of plant origin may serve as promising therapeutics for the treatment of dementia, however, pivotal evidence for their therapeutic efficacy in advanced clinical studies is still lacking.

Mutation of Mitochondrial DNA G13513A Presenting with Leigh Syndrome, Wolff-Parkinson-White Syndrome and Cardiomyopathy

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Shi-Bing Wang

2008-08-01

Full Text Available Mutation of mitochondrial DNA (mtDNA G13513A, encoding the ND5 subunit of respiratory chain complex I, can cause mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS and Leigh syndrome. Wolff-Parkinson-White (WPW syndrome and optic atrophy were reported in a high proportion of patients with this mutation. We report an 18-month-old girl, with an 11-month history of psychomotor regression who was diagnosed with WPW syndrome and hypertrophic cardiomyopathy, in association with Leigh syndrome. Supplementation with coenzyme Q10, thiamine and carnitine prevented further regression in gross motor function but the patient's heart function deteriorated and dilated cardiomyopathy developed 11 months later. She was found to have a mutation of mtDNA G13513A. We suggest that mtDNA G13513A mutation is an important factor in patients with Leigh syndrome associated with WPW syndrome and/or optic atrophy, and serial heart function monitoring by echocardiography is recommended in this group of patients.

Rett syndrome: an overlooked diagnosis in women with stereotypic hand movements, psychomotor retardation, Parkinsonism, and dystonia?

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Roze, Emmanuel; Cochen, Valérie; Sangla, Sophie; Bienvenu, Thierry; Roubergue, Anne; Leu-Semenescu, Smaranda; Vidaihet, Marie

2007-02-15

Rett syndrome is an X-linked neurodevelopmental disorder resulting in profound psychomotor retardation. It is usually diagnosed by a pediatrician or pediatric neurologist. Adult neurologists may, therefore, overlook the possibility of Rett syndrome in women with psychomotor retardation of unknown etiology. We report the case of a woman diagnosed with Rett syndrome at age 49 years. This report emphasizes the diagnostic value of movement disorders, including hand stereotypies, Parkinsonism, and dystonia, in adults with Rett syndrome.

Syndrome and Phenomenon of Wolff — Parkinson — White in Children (Literature Review

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N.V. Nagornaya

2013-03-01

Full Text Available The article deals with the data on the history of the discovery and study of the phenomenon and syndrome Wolff — Parkinson — White (WPW. The features of the anatomy and electrophysiology of additional ways, mechanisms of attacks of atrioventricular reentrant tachycardia in WPW syndrome are considered. The role of the standard ECG, methods for topical analysis of additional atrioventricular connections and non-invasive electrophysiological studies in the diagnosis of this disease is shown.

Anaesthetic management of a case of Wolff-Parkinson-White syndrome

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Savitri D Kabade

2011-01-01

Full Text Available We report a case of fibroid uterus with Wolff-Parkinson-White (WPW syndrome in a 48-year-old female, posted for elective hysterectomy. Patient gave history of short recurrent episodes of palpitation and electrocardiograph confirmed the diagnosis of WPW syndrome. The anaesthetic management of these patients is challenging as they are known to develop life threatening tachyarrhythmia like paroxysmal supra-ventricular tachycardia (PSVT and atrial fibrillation (AF. Epidural anaesthesia is preferred compared to general anaesthesia to avoid polypharmacy, noxious stimuli of laryngoscopy and intubation. To deal with perioperative complications like PSVT and AF, anti-arrhythmic drugs like adenosine, beta blockers and defibrillator should be kept ready. Perioperative monitoring is essential as patients can develop complications.

[CADASIL with clinical manifestations of baldness, lumbago and Parkinson's symptoms].

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Ren, Zhixia; Chen, Shuai; Shi, Yingying; Zhang, Yuanxing; Wang, Wan; Chen, Zuzhi; Xia, Mingrong; Shi, Xiaohong; Zhang, Jiewen

2017-12-10

To investigate a cerebral autosomal dominant arteriopathy with the subcortical infarcts and leukoencephalopathy (CADASIL) case with clinical manifestations of baldness, lumbago and Parkinson's symptoms. Clinical and imaging data of the patient were analyzed. The patient and his family members were also subjected to genetic testing. The symptoms of the patient included recurrent stroke, dementia, and mood disturbance, in addition with lumbago, baldness and Parkinson's symptoms but no migraine. Cranial MRI of the patient showed bilateral symmetric leukoencephalopathy and multiple small subcortical lacunar infarcts. A point mutation in exon 11 of the NOTCH3 gene (R558C) was discovered in the proband and four asymptomatic relatives. CADASIL is characterized by recurrent subcortical ischemic stroke, dementia, pseudobulbar palsy, and mood disturbance. Baldness, lumbago and Parkinson's symptoms may also be seen in such patients.

Cognitive rehabiliation for Parkinson's disease demantia: a study protocol for a pilot randomised controlled trial.

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Hindle, John V; Watermeyer, Tamlyn J; Roberts, Julie; Martyr, Anthony; Lloyd-Williams, Huw; Brand, Andrew; Gutting, Petra; Hoare, Zoe; Edwards, Rhiannon Tudor; Clare, Linda

2016-03-22

There is growing interest in developing non-pharmacological treatments to address the cognitive deficits apparent in Parkinson's disease dementia and dementia with Lewy bodies. Cognitive rehabilitation is a goal-oriented behavioural intervention which focuses on improving everyday functioning through management of cognitive difficulties; it has been shown to be effective in Alzheimer's disease. To date, no studies have assessed its potential efficacy for addressing the impact of cognitive impairment in people with Parkinson's disease or dementia with Lewy bodies. Participants (n = 45) will be recruited from movement disorders, care for the elderly and memory clinics. Inclusion criteria include: a diagnosis of Parkinson's disease, Parkinson's disease dementia or dementia with Lewy bodies according to consensus criteria and an Addenbrooke's Cognitive Examination - III score of ≤ 82. Exclusion criteria include: a diagnosis of any other significant neurological condition; major psychiatric disorder, including depression, which is not related to the patient's Parkinson's disease and unstable medication use for their physical or cognitive symptoms. A single-blind pilot randomised controlled trial, with concurrent economic evaluation, will compare the relative efficacy of cognitive rehabilitation with that of two control conditions. Following a goal-setting interview, the participants will be randomised to one of the three study arms: cognitive rehabilitation (eight weekly sessions), relaxation therapy (eight weekly sessions) or treatment as usual. Randomisation and treatment group allocation will be carried out by a clinical trials unit using a dynamic adaptive sequential randomisation algorithm. The primary outcomes are patients' perceived goal attainment at a 2-months post-intervention assessment and a 6-months follow-up. Secondary outcomes include patients' objective cognitive performance (on tests of memory and executive function) and satisfaction with goal

Dementia in Fragile X-associated Tremor/Ataxia Syndrome

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Ricardo Nitrini

Full Text Available Abstract Fragile X-associated tremor/ataxia syndrome (FXTAS is a cause of movement disorders and cognitive decline which has probably been underdiagnosed, especially if its prevalence proves similar to those of progressive supranuclear palsy and amyotrophic lateral sclerosis. We report a case of a 74-year-old man who presented with action tremor, gait ataxia and forgetfulness. There was a family history of tremor and dementia, and one of the patient's grandsons was mentally deficient. Neuropsychological evaluation disclosed a frontal network syndrome. MRI showed hyperintensity of both middle cerebellar peduncles, a major diagnostic hallmark of FXTAS. Genetic testing revealed premutation of the FMR1 gene with an expanded (CGG90 repeat. The diagnosis of FXTAS is important for genetic counseling because the daughters of the affected individuals are at high risk of having offspring with fragile X syndrome. Tremors and cognitive decline should raise the diagnostic hypothesis of FXTAS, which MRI may subsequently reinforce, while the detection of the FMR1 premutation can confirm the condition.

Threatening auditory hallucinations and Cotard syndrome in Parkinson disease.

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Factor, Stewart A; Molho, Eric S

2004-01-01

Psychotic symptoms are commonly reported in patients with Parkinson disease (PD). In particular, patients experience nonthreatening visual hallucinations that can occur with insight (so called hallucinosis) or without. Auditory hallucinations are uncommon, and schizophrenialike symptoms such as pejorative and threatening auditory hallucinations and delusions that are persecutory, referential, somatic, religious, or grandiose have rarely been reported. The authors present 2 PD patients who experienced threatening auditory hallucinations, without visual hallucinations, and schizophrenialike delusions with detailed description of the clinical phenomenology including 1 patient with Cotard syndrome.

Motor function and incident dementia: a systematic review and meta-analysis.

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Kueper, Jacqueline Kathleen; Speechley, Mark; Lingum, Navena Rebecca; Montero-Odasso, Manuel

2017-09-01

cognitive and mobility decline are interrelated processes, whereby mobility decline coincides or precedes the onset of cognitive decline. to assess whether there is an association between performance on motor function tests and incident dementia. electronic database, grey literature and hand searching identified studies testing for associations between baseline motor function and incident dementia in older adults. of 2,540 potentially relevant documents, 37 met the final inclusion criteria and were reviewed qualitatively. Three meta-analyses were conducted using data from 10 studies. Three main motor domains-upper limb motor function, parkinsonism and lower limb motor function-emerged as associated with increased risk of incident dementia. Studies including older adults without neurological overt disease found a higher risk of incident dementia associated with poorer performance on composite motor function scores, balance and gait velocity (meta-analysis pooled HR = 1.94, 95% CI: 1.41, 2.65). Mixed results were found across different study samples for upper limb motor function, overall parkinsonism (meta-analysis pooled OR = 3.05, 95% CI: 1.31, 7.08), bradykinesia and rigidity. Studies restricted to older adults with Parkinson's Disease found weak or no association with incident dementia even for motor domains highly associated in less restrictive samples. Tremor was not associated with an increased risk of dementia in any population (meta-analysis pooled HR = 0.80, 95% CI 0.31, 2.03). lower limb motor function was associated with increased risk of developing dementia, while tremor and hand grip strength were not. Our results support future research investigating the inclusion of quantitative motor assessment, specifically gait velocity tests, for clinical dementia risk evaluation. © The Author 2017. Published by Oxford University Press on behalf of the British Geriatrics Society.All rights reserved. For permissions, please email: journals.permissions@oup.com

Embodying Emotional Disorders: New Hypotheses about Possible Emotional Consequences of Motor Disorders in Parkinson's Disease and Tourette's Syndrome.

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Mermillod, Martial; Vermeulen, Nicolas; Droit-Volet, Sylvie; Jalenques, Isabelle; Durif, Franck; Niedenthal, Paula

2011-01-01

Parkinson's disease (PD) and Tourette's syndrome (TS) lead to important motor disorders among patients such as possible facial amimia in PD and tics in Tourette's syndrome. Under the grounded cognition framework that shows the importance of motor embodiment in emotional feeling (Niedenthal, 2007), both types of pathology with motor symptoms should be sufficient to induce potential impairments for these patients when recognizing emotional facial expressions (EFE). In this opinion paper, we describe a theoretical framework that assumes potential emotional disorders in Parkinson's disease and Tourette's syndrome based on motor disorders characterizing these two pathologies. We also review different methodological barriers in previous experimental designs that could enable the identification of emotional facial expressions despite emotional disorders in PD and TS.

Music perception in dementia

Science.gov (United States)

Nicholas, Jennifer M; Cohen, Miriam H; Slattery, Catherine F; Paterson, Ross W; Foulkes, Alexander J M; Schott, Jonathan M; Mummery, Catherine J; Crutch, Sebastian J; Warren, Jason D

2017-01-01

Despite much recent interest in music and dementia, music perception has not been widely studied across dementia syndromes using an information processing approach. Here we addressed this issue in a cohort of 30 patients representing major dementia syndromes of typical Alzheimer’s disease (AD, n=16), logopenic aphasia (LPA, an Alzheimer variant syndrome; n=5) and progressive nonfluent aphasia (PNFA; n=9) in relation to 19 healthy age-matched individuals. We designed a novel neuropsychological battery to assess perception of musical patterns in the dimensions of pitch and temporal information (requiring detection of notes that deviated from the established pattern based on local or global sequence features) and musical scene analysis (requiring detection of a familiar tune within polyphonic harmony). Performance on these tests was referenced to generic auditory (timbral) deviance detection and recognition of familiar tunes and adjusted for general auditory working memory performance. Relative to healthy controls, patients with AD and LPA had group-level deficits of global pitch (melody contour) processing while patients with PNFA as a group had deficits of local (interval) as well as global pitch processing. There was substantial individual variation within syndromic groups. No specific deficits of musical temporal processing, timbre processing, musical scene analysis or tune recognition were identified. The findings suggest that particular aspects of music perception such as pitch pattern analysis may open a window on the processing of information streams in major dementia syndromes. The potential selectivity of musical deficits for particular dementia syndromes and particular dimensions of processing warrants further systematic investigation. PMID:27802226

Music Perception in Dementia.

Science.gov (United States)

Golden, Hannah L; Clark, Camilla N; Nicholas, Jennifer M; Cohen, Miriam H; Slattery, Catherine F; Paterson, Ross W; Foulkes, Alexander J M; Schott, Jonathan M; Mummery, Catherine J; Crutch, Sebastian J; Warren, Jason D

2017-01-01

Despite much recent interest in music and dementia, music perception has not been widely studied across dementia syndromes using an information processing approach. Here we addressed this issue in a cohort of 30 patients representing major dementia syndromes of typical Alzheimer's disease (AD, n = 16), logopenic aphasia (LPA, an Alzheimer variant syndrome; n = 5), and progressive nonfluent aphasia (PNFA; n = 9) in relation to 19 healthy age-matched individuals. We designed a novel neuropsychological battery to assess perception of musical patterns in the dimensions of pitch and temporal information (requiring detection of notes that deviated from the established pattern based on local or global sequence features) and musical scene analysis (requiring detection of a familiar tune within polyphonic harmony). Performance on these tests was referenced to generic auditory (timbral) deviance detection and recognition of familiar tunes and adjusted for general auditory working memory performance. Relative to healthy controls, patients with AD and LPA had group-level deficits of global pitch (melody contour) processing while patients with PNFA as a group had deficits of local (interval) as well as global pitch processing. There was substantial individual variation within syndromic groups. Taking working memory performance into account, no specific deficits of musical temporal processing, timbre processing, musical scene analysis, or tune recognition were identified. The findings suggest that particular aspects of music perception such as pitch pattern analysis may open a window on the processing of information streams in major dementia syndromes. The potential selectivity of musical deficits for particular dementia syndromes and particular dimensions of processing warrants further systematic investigation.

Capgras syndrome in Dementia with Lewy Bodies.

Science.gov (United States)

Thaipisuttikul, Papan; Lobach, Iryna; Zweig, Yael; Gurnani, Ashita; Galvin, James E

2013-05-01

Capgras syndrome is characterized by the recurrent, transient belief that a person has been replaced by an identical imposter. We reviewed clinical characteristics of Dementia with Lewy Bodies (DLB) patients with Capgras syndrome compared to those without Capgras. We identified 55 consecutive DLB patients (11 cases with Capgras syndrome (DLB-C) and 44 cases without evidence of Capgras (DLB). Semi-structured interviews with the patient and an informant, neurological exams, and neuropsychological testing were performed. Caregivers were assessed for caregiver burden and depression. Primary comparisons were made between DLB-C and DLB. Exploratory analyses using stepwise logistic regression and bootstrap analyses were performed to determine clinical features associated with Capgras. DLB-C patients experienced more visual hallucinations and self-reported anxiety, had higher scores on the Neuropsychiatric Inventory, and were less likely to be treated with cholinesterase inhibitors at time of initial evaluation. Extrapyramidal symptoms and depression were not associated with Capgras. Caregivers of DLB-C patients had higher caregiver burden. DLB-C was associated with self-reported anxiety (OR = 10.9; 95% CI = 2.6-47.6). In a bootstrap analysis, clinical findings that were predictors of Capgras included visual hallucinations (log(OR) = 18.3; 95% CI = 17.9-19.3) and anxiety (log(OR) = 2.9; 95% CI = 0.31-20.2). Our study suggests that Capgras syndrome is common in DLB and usually occurs in the presence of anxiety and visual hallucinations, suggesting related etiopathogenesis. Early appreciation of Capgras syndrome may afford the opportunity to alleviate caregiver burden and improve patient and caregiver outcomes.

Parkinson's disease mild cognitive impairment: application and validation of the criteria

NARCIS (Netherlands)

Geurtsen, Gert J.; Hoogland, Jeroen; Goldman, Jennifer G.; Schmand, Ben A.; Tröster, Alexander I.; Burn, David J.; Litvan, Irene; Filoteo, J. Vincent; Hurtig, Howard; Chen-Plotkin, Alice; Adler, Charles H.; Jacobson, Sandra A.; Leverenz, Jim; Zabetian, Cyrus; Huang, Xuemei; Eslinger, Paul J.; Marras, Connie; Duff-Canning, Sarah; Dalrymple-Alford, John C.; Anderson, Tim J.; Naismith, Sharon L.; Lewis, Simon J. G.; Wu, Ruey-Meei; Yu, Rwei-Ling; Bhattacharyya, Kalyan B.; Williams-Gray, Caroline H.; Breen, David P.; Barker, Roger A.; Yarnall, Alison J.; de Bie, Rob M. A.; Koene, Ted; Klein, Martin; Trautmann, Ellen; Mollenhauer, Brit; Dodel, Richard; Kulisevsky, Jaime; Pagonabaragga, Javier; Rodriguez-Oroz, Maria C.; Gasca-Salas, Carmen; Junque, Carme; Segura, Barbara; Sportiello, Marco Timpano; Cammisuli, Davide M.; Barone, Paolo; Pedersen, Kenn Freddy; Alves, Guido

2014-01-01

Dementia in Parkinson's disease (PD) is a serious health issue and a major concern for many patients. In most cases mild cognitive impairment (MCI) is considered a transitional stage between normal cognitive functioning and dementia which is of potential importance in the early identification of

Accelerated Age-Dependent Hippocampal Volume Loss in Parkinson Disease With Mild Cognitive Impairment.

Science.gov (United States)

Schneider, Christine B; Donix, Markus; Linse, Katharina; Werner, Annett; Fauser, Mareike; Klingelhoefer, Lisa; Löhle, Matthias; von Kummer, Rüdiger; Reichmann, Heinz; Storch, Alexander

2017-09-01

Patients with Parkinson disease are at high risk of developing dementia. During the course of the disease, a substantial number of patients will experience a cognitive decline, indicating the dynamics of the underlying neuropathology. Magnetic resonance imaging (MRI) has become increasingly useful for identifying structural characteristics in radiological brain anatomy existing prior to clinical symptoms. Whether these changes reflect pathology, whether they are aging related, or both often remains unclear. We hypothesized that aging-associated brain structural changes would be more pronounced in the hippocampal region among patients with Parkinson disease having mild cognitive deficits relative to cognitively unimpaired patients. Using MRI, we investigated 30 cognitively healthy patients with Parkinson disease and 33 patients with nondemented Parkinson disease having mild cognitive impairment. All participants underwent structural MRI scanning and extensive clinical and neuropsychological assessments. Irrespective of the study participants' cognitive status, older age was associated with reduced cortical thickness in various neocortical regions. Having mild cognitive impairment was not associated with an increased rate of cortical thinning or volume loss in these regions, except in the hippocampus bilaterally. Patients with Parkinson disease having mild cognitive impairment show an accelerated age-dependent hippocampal volume loss when compared with cognitively healthy patients with Parkinson disease. This may indicate pathological processes in a key region for memory functioning in patients with Parkinson disease at risk of developing dementia. Structural MRI of the hippocampal region could potentially contribute to identifying patients who should receive early treatment aimed at delaying the clinical onset of dementia.

Glucocerebrosidase mutations and neuropsychiatric phenotypes in Parkinson's disease and Lewy body dementias: Review and meta-analyses.

Science.gov (United States)

Creese, Byron; Bell, Emily; Johar, Iskandar; Francis, Paul; Ballard, Clive; Aarsland, Dag

2018-03-01

Heterozygous mutations in glucocerebrosidase gene (GBA) are a major genetic risk factor for Parkinson's disease (PD) and dementia with Lewy bodies (DLB). Recently, there has been a considerable focus on the relationship between GBA mutations and emergence of cognitive impairment and neuropsychiatric symptoms in these diseases. Here, we review the literature in this area, with a particular focus, including meta-analysis, on the key neuropsychiatric symptoms of cognitive impairment, psychosis, and depression in Parkinson's disease. Our meta-analysis demonstrated that GBA mutations are associated with a 2.4-fold increased risk of cognitive impairment. In addition, our novel meta-analyses of psychosis and depression showed a 1.8- and 2.2-fold increased risk respectively associated with GBA mutations, although due to possible bias and heterogeneity the depression findings should be interpreted with caution. While the precise mechanisms which increase susceptibility to neurodegeneration in GBA carriers are not known, evidence of greater cortical Lewy body pathology, reduced patterns of cortical activation, and hippocampal pathology in animal models are all consistent with a direct effect of GBA mutations on these symptoms. Extension of this work in DLB and individuals without neurodegeneration will be important in further characterizing how GBA mutations increase risk for PD and DLB and influence disease course. © 2017 Wiley Periodicals, Inc.

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...

[Dementias: impact on the family and prevention of caregivers' syndrome].

Science.gov (United States)

Sabater Mateu, M P; López Cortacans, G

1998-11-01

This article deals with the impact dementia can cause in a family in our modern society, an impact which often leads to a crisis or a rupture in a family. Due to the evolution in family structure in Spain over the last few decades, as evidenced by separations, divorces, reconstructed families, a descent in birth rate, and especially by women joining the work force, the possibilities for traditional family care of the elderly have become complicated and have been reduced, which leads to a tremendous physical and psychological wear on the person who takes on the largest part of this care, the so-called main caretaker, usually a woman. In consequence to this situation, dysfunctional behaviors may develop in this caretaker which, if not treated, may early or later on develop into the caretaker syndrome. The effects of this syndrome include subjective ones such as emotional suffering and objective ones such as a loss of health. This article presents a proposal for action by nurses acting out of primary health care centers which leads to a series of measures providing containing methods and formal support structures, in other words prevention, for the family and especially for the main caretaker of patients suffering from dementia.

Neocortical concentrations of neuropeptides in senile dementia of the Alzheimer and Lewy body type: comparison with Parkinson's disease and severity correlations.

Science.gov (United States)

Leake, A; Perry, E K; Perry, R H; Jabeen, S; Fairbairn, A F; McKeith, I G; Ferrier, I N

1991-02-15

Corticotropin releasing hormone (CRH), somatostatin (SRIF), and arginine vasopressin (AVP) concentrations were estimated using radioimmunoassay in the temporal and occipital cortices in postmortem brain from patients clinically and neuropathologically diagnosed as senile dementia of the Lewy body type (SDLT), senile dementia of the Alzheimer type (SDAT), and Parkinson's disease (PD) and from neurologically normal controls. The concentration of temporal and occipital neocortical CRH was diminished in both SDAT and SDLT compared to control values, whereas SRIF was reduced only in temporal cortex in both these conditions. In contrast, the concentrations of both CRH and SRIF were unaltered in PD. The concentrations of AVP in SDLT, SDAT, and PD were similar to those found in the control groups. The decrement in SRIF, but not CRH, was found to be correlated with some indices of severity of illness in SDAT; a similar but nonsignificant trend for SRIF was observed in SDLT.

Increased aluminum content in the spinal cord of amyotrophic lateral sclerosis and Parkinsonism-dementia of Guam and in the Kii Peninsula of Japan

Energy Technology Data Exchange (ETDEWEB)

Wakayama, Ikuro; Yoshida, Sohei [Wakayama Medical Coll. (Japan); Sasajima, Kazuhisa; Takada, Jitsuya; Yoshida, Koichi

1994-07-01

Aluminum content in the lumbar spinal cord of patients with amyotrophic lateral sclerosis(ALS) and Parkinsonism-dementia(PD) in the Kii Peninsula of Japan and in the island of Guam was measured using a particle induced X-ray emission analysis. We demonstrated that aluminum content was increased in the spinal cord of patients with ALS in two foci of the western Pacific, indicating aluminum to be a important factor in the process of spinal motor neuron degeneration. (author).

Increased aluminum content in the spinal cord of amyotrophic lateral sclerosis and Parkinsonism-dementia of Guam and in the Kii Peninsula of Japan

International Nuclear Information System (INIS)

Wakayama, Ikuro; Yoshida, Sohei; Sasajima, Kazuhisa; Takada, Jitsuya; Yoshida, Koichi.

1994-01-01

Aluminum content in the lumbar spinal cord of patients with amyotrophic lateral sclerosis(ALS) and Parkinsonism-dementia(PD) in the Kii Peninsula of Japan and in the island of Guam was measured using a particle induced X-ray emission analysis. We demonstrated that aluminum content was increased in the spinal cord of patients with ALS in two foci of the western Pacific, indicating aluminum to be a important factor in the process of spinal motor neuron degeneration. (author)

Comparative study of CT scan findings and intellectual function between Parkinson's disease and vascular Parkinsonism

International Nuclear Information System (INIS)

Indo, Toshikatsu

1986-01-01

Comparative study of CT scan findings and intellectual function between 64 cases with Parkinson's disease and 25 cases with vascular Parkinsonism was carried out. The rate of abnormality of CT scan findings, either ventricular dilatation or widening of sulci, in vascular Parkinsonism was strikingly high compared with Parkinson's disease. Patients could be divided into three groups according to the degree of overall abnormalities of CT scan findings (group A: markedly abnormal, group B: mildly abnormal, group C: normal). Incidences of group A were 9.4 % in Parkinson's disease and 52 % in vascular Parkinsonism, whereas those of group C were 56 % in the former and 28 % in the latter. All patients of group A were over 65 years of age in Parkinson's disease, but one-third of patients in group A were under 59 years of age in vascular Parkinsonism. Moreover, in vascular Parkinsonism, the level of disability was directly proportional to the abnormality of CT scan findings. The rate of predementia and dementia classified by Hasegawa's intelligence scale was 12.5 % in Parkinson's disease and 48 % in vascular Parkinsonism. No difference was found between the mean values of intelligence scale and background factors in Parkinson's disease. On the other hand, the mean value was significantly low in proportion to the poverty of L-dopa effect in vascular Parkinsonism. From these results, the abnormality of CT scan findings and intellectual impairment were probably related to the cerebral pathological process in vascular Parkinsonism, but these relationship was absent in Parkinson's disease. (author)

Comparative study of CT scan findings and intellectual function between Parkinson's disease and vascular Parkinsonism

Energy Technology Data Exchange (ETDEWEB)

Indo, Toshikatsu

1986-01-01

Comparative study of CT scan findings and intellectual function between 64 cases with Parkinson's disease and 25 cases with vascular Parkinsonism was carried out. The rate of abnormality of CT scan findings, either ventricular dilatation or widening of sulci, in vascular Parkinsonism was strikingly high compared with Parkinson's disease. Patients could be divided into three groups according to the degree of overall abnormalities of CT scan findings (group A: markedly abnormal, group B: mildly abnormal, group C: normal). Incidences of group A were 9.4 % in Parkinson's disease and 52 % in vascular Parkinsonism, whereas those of group C were 56 % in the former and 28 % in the latter. All patients of group A were over 65 years of age in Parkinson's disease, but one-third of patients in group A were under 59 years of age in vascular Parkinsonism. Moreover, in vascular Parkinsonism, the level of disability was directly proportional to the abnormality of CT scan findings. The rate of predementia and dementia classified by Hasegawa's intelligence scale was 12.5 % in Parkinson's disease and 48 % in vascular Parkinsonism. No difference was found between the mean values of intelligence scale and background factors in Parkinson's disease. On the other hand, the mean value was significantly low in proportion to the poverty of L-dopa effect in vascular Parkinsonism. From these results, the abnormality of CT scan findings and intellectual impairment were probably related to the cerebral pathological process in vascular Parkinsonism, but these relationship was absent in Parkinson's disease.

Sugammadex Use in a Patient with Wolff-Parkinson-White (WPW) Syndrome.

Science.gov (United States)

Şahin, Sevtap Hekimoğlu; Öztekin, İlhan; Kuzucuoğlu, Aytuna; Aslanoğlu, Ayça

2015-07-01

Wolff-Parkinson-White (WPW) syndrome is a disease associated with episodes of supraventricular tachycardia and ventricular pre-excitation or atrial fibrillation. WPW is characterized by an aberrant electrical conduction pathway between atria and ventricles. The major anesthetic problem connected with WPW syndrome is the risk of tachyarrhythmias due to accessory pathway. Therefore, it has been proposed that the aim of anesthetic management should be the avoidance of tachyarrhythmia and sympathetic stimulation. Sugammadex was administered as a neuromuscular reversal agent in this case. To our knowledge, this is the first case report of sugammadex use in a patient with WPW. This report presents a case of general anesthesia management in a patient with WPW syndrome. We think that it is appropriate to use sugammadex to reverse rocuronium for the prevention of sudden hemodynamic changes in patients with WPW who underwent general anesthesia.

Neuroimaging and functional assessment in dementia

Energy Technology Data Exchange (ETDEWEB)

Terashi, Akiro; Kitamura, Shin; Ujike, Takashi [Nippon Medical School, Tokyo (Japan)

1990-05-01

Recent progress in diagnostic imaging techniques has greatly contributed to the elucidation of pathophysiology, as well as differential diagnosis in dementia. In particular, X-ray computed tomography (CT) offers the ability to detect morphological changes of the brain, whereby making it possible to differentiate between cerebrovascular and degenerative dementias. Magnetic resonance imaging (MRI) allows three-dimensional visualization of gyrus atrophy, providing the ability to depict subcortical minor infarcts and white matter lesions. The advent of positron emission tomography (PET) and single photon emission computed tomography (SPECT) has led to a dramatic progression in the search for pathophysiology of dementia. The purpose of this paper is to outline the recent findings of diagnostic imaging modalities, such as X-ray CT, MRI, PET and SPECT, focusing on those in commonly observed cerebrovascular dementia and in degenerative dementias that are projected to increase. Degenerative dementias cover Alzheimer's disease, Pick's disease, Huntington's disease, progressive supranuclear palsy, Parkinson's disease, and normal pressure hydrocephalus. (N.K.) 90 refs.

The Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) Scale : Comprehensive Assessment of Psychopathology in Down Syndrome

NARCIS (Netherlands)

Dekker, Alain D; Sacco, Silvia; Carfi, Angelo; Benejam, Bessy; Vermeiren, Yannick; Beugelsdijk, Gonny; Schippers, Mieke; Hassefras, Lyanne; Eleveld, José; Grefelman, Sharina; Fopma, Roelie; Bomer-Veenboer, Monique; Boti, Mariángeles; Oosterling, G Danielle E; Scholten, Esther; Tollenaere, Marleen; Checkley, Laura; Strydom, André; Van Goethem, Gert; Onder, Graziano; Blesa, Rafael; Zu Eulenburg, Christine; Coppus, Antonia M W; Rebillat, Anne-Sophie; Fortea, Juan; De Deyn, Peter P

2018-01-01

People with Down syndrome (DS) are prone to develop Alzheimer's disease (AD). Behavioral and psychological symptoms of dementia (BPSD) are core features, but have not been comprehensively evaluated in DS. In a European multidisciplinary study, the novel Behavioral and Psychological Symptoms of

Wolff-Parkinson-White Syndrome: Electrocardiogram

Directory of Open Access Journals (Sweden)

Brianna Miner

2016-09-01

Full Text Available History of present illness: A 26-year-old male with no significant past medical history presented to the emergency department with palpitations. The patient experienced these symptoms five times before in his life, but they had self-resolved with squatting or raising his arms. He denied chest pain, shortness of breath, dizziness, syncope, or pre-syncope symptoms. He denied any recent illnesses, cough, chest pain, drug use, or infections. Significant findings: The initial EKG showed wide complex, irregular tachycardia > 200 bpm (EKG 1. Given the possibility of Wolff-Parkinson-White (WPW, procainamide was given to the patient. The patient’s heart rate responded and decreased to 120-140 bpm with narrowing of the QRS complex. A repeat EKG showed narrow complex tachycardia without P waves approximately 120 bpm (EKG 2. Once the procainamide infusion was complete, the patient had converted to sinus rhythm with a delta wave now apparent, consistent with WPW (EKG 3. Discussion: Wolff-Parkinson-White syndrome is a combination of the presence of a certain congenital accessory pathway (Bundle of Kent and episodes of tachyarrhythmia.1 The incidence is less than one percent of the general population,2 but it is associated with the risk of sudden cardiac death, making the diagnosis imperative. When examining a wide-based tachycardia, it is important to consider WPW when choosing medications for rate control or chemical cardioversion. The first EKG shows an example of atrial fibrillation with WPW. While procainamide blocks the accessory pathway, other drugs such as beta blockers and calcium channel blockers can inhibit the AV node resulting in increased use of the accessory pathway. This can worsen the tachyarrhythmia and potentially lead to ventricular fibrillation, ventricular tachycardia, or asystole.

[Artistic creativity and dementia].

Science.gov (United States)

Sellal, François; Musacchio, Mariano

2008-03-01

Artistic creativity can be defined as the ability to produce both innovative and esthetic works. Though most dementias result in a loss of instrumental functions and a deterioration in artistic production, for some established artists, dementia, most often Alzheimer's disease, changed their style and technique but preserved their creativity and prolific artistic drive. Moreover, in some cases, mainly frontotemporal dementia, Parkinson's disease, and very occasionally strokes, the disease may favour the emergence of de novo artistic talent. This phenomenon has been conceptualized as a paradoxical facilitation, a disinhibition of brain areas devoted to visuospatial processing, greater freedom in a patient who becomes less bound by social conventions, enhancement of motivation and pleasure, etc. These neurological cases provide an opportunity to shed some light on the roots of artistic creation.

Behavioural Excesses and Deficits Associated with Dementia in Adults Who Have Down Syndrome

Science.gov (United States)

Oliver, Chris; Kalsy, Sunny; McQuillan, Sharna; Hall, Scott

2011-01-01

Background: Informant-based assessment of behavioural change and difference in dementia in Down syndrome can aid diagnosis and inform service delivery. To date few studies have examined the impact of different types of behavioural change. Methods: The Assessment for Adults with Developmental Disabilities (AADS), developed for this study, assesses…

Cerebral perfusion scintigraphy and the exploration of dementia syndromes: An illustration with five clinical cases

International Nuclear Information System (INIS)

Farid, K.; Perdrisot, R.; Habert, M.O.

2007-01-01

The epidemiological evidence suggests that individuals with higher education level have a reduced risk of developing dementia. Because cognitive reserve and its compensation mechanisms may modulate the clinical expression in neuro-degenerative pathology, it is important to study subjects who present mild cognitive disturbance with functional imaging. The cerebral SPECT has been used to determine regional uptake of radiotracer into the brain of patients with cognitive impairment. These abnormalities of blood flow were correlated with cognitive impairment. The cerebral SPECT is also useful to investigate preclinical dementia and to predict the evolution of cognitive disturbance. This article, reports some technical and semeiological notions and illustrate with five clinical cases the scintigraphic aspect of some dementia syndrome. (authors)

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.

Dementia in Qatar

International Nuclear Information System (INIS)

Hamad, Ahmad I.; Ibrahim, Mohammed A.; Sulaiti, Essa M.

2004-01-01

Dementia is the major public health problem among the elderly in developed countries and a growing problem in the underdeveloped countries. There are no published data on dementia in any of the Arab countries. The aim of this study was to determine the different subtypes of dementia among Qataris. A retrospective and prospective ongoing hospital based study in which all medical records of the patients with diagnosis of dementia seen at the Hamad General Hospital, Doha, Qatar, between June 1997 and June 2003, whether inpatient and outpatient were reviewed. Dementia was defined according to diagnostic and statistical manual (DSM) IV criteria. Those who had dementia were evaluated by a psychologist, psychiatrist, neurologist and a geriatrician. All had brain computerized tomography, magnetic resonance imaging or both and routine blood test. Finally, they were classified into sub-types according to the cause of dementia. One of 300 patients, 134 fulfilled the inclusion criteria, most of them were illiterate, married and non-smokers. Among those dementia sub-types were: Alzheimer disease (AD) 39 (29%), vascular dementia (VaD) 30 (22%), mixed AD and VaD 20 (15%) and Parkinson's disease with dementia due to other medical conditions. Our stidy showed that AD is more prevalent than VaD. It also showed that patients and their families seek medical help late due to to the general belief among the public that forgetfulness and other associated cognitive impairment are part of normal aging process. The emergence of new drugs and advancement in prevention of cerebrovascular diseases make early diagnosis of dementia sub-type important. A community based study to show the real prevalence and incidence of sub-types of dementia is highly indicated. These data are necessory for planning and setting up community services and health care programs for demented patients. (author)

Wolff-Parkinson-White syndrome: a single exercise stress test might be misleading.

Science.gov (United States)

Salavitabar, Arash; Silver, Eric S; Liberman, Leonardo

2017-05-01

Risk stratification of patients with Wolff-Parkinson-White syndrome for sudden death is a complex process, particularly in understanding the utility of the repeat exercise stress test. We report a case of an 18-year-old patient who was found to have a high-risk pathway by both invasive and exercise stress testing after an initial exercise stress test showing beat-to-beat loss of pre-excitation.

Capgras delusion for animals and inanimate objects in Parkinson?s Disease: a case report

OpenAIRE

Islam, Lucrezia; Piacentini, Sylvie; Soliveri, Paola; Scarone, Silvio; Gambini, Orsola

2015-01-01

Background Capgras delusion is a delusional misidentification syndrome, in which the patient is convinced that someone that is well known to them, usually a close relative, has been replaced by an impostor or double. Although it has been frequently described in psychotic syndromes, including paranoid schizophrenia, over a third of the documented cases of Capgras delusion are observed in patients with organic brain lesions or neurodegenerative disease, including Parkinson?s Disease. Variants o...

Magnetisation transfer measurements of the subcortical grey and white matter in Parkinson's disease with and without dementia and in progressive supranuclear palsy

International Nuclear Information System (INIS)

Hanyu, H.; Asano, T.; Sakurai, H.; Takasaki, M.; Shindo, H.; Abe, K.

2001-01-01

We measured the magnetisation transfer ratio (MTR) in the subcortical grey and white matter of 11 patients with idiopathic Parkinson's disease (PD) without dementia, six with PD with dementia (PDD), six with progressive supranuclear palsy (PSP), and 12 elderly control subjects to assess regional differences in structural brain damage. There were no significant differences in MTR in any region between PD and controls. However, patients with PDD had significantly lower MTR in the subcortical white matter, including the frontal white matter and the genu of the corpus callosum than the controls, whereas PSP had significantly lower MTR in the subcortical grey matter, including the putamen, globus pallidus and thalamus, in addition to the subcortical white matter. This suggests that regional patterns of structural brain damage can be detected using the magnetisation transfer technique. Measurement of MTR in the subcortical grey and white matter may be useful in differential diagnosis. (orig.)

Fluctuating Cotard syndrome in a patient with advanced Parkinson disease.

Science.gov (United States)

Solla, Paolo; Cannas, Antonino; Orofino, Gianni; Marrosu, Francesco

2015-02-01

Nonmotor fluctuations of psychiatric symptoms in patients suffering from Parkinson disease (PD) represent a very disabling condition, which may seriously interfere with the quality of life of patients and caregivers. In this regard, these disturbances are present with a higher frequency in advanced PD patients with associated motor complications and can appear both in "on" and in "off" period. Here we report on a case of fluctuating Cotard syndrome clearly related to "wearing-off" deterioration and responsive to levodopa treatment in a patient affected by advanced PD. A 76-year-old woman presented with a 13-year history of PD. Her caregivers reported that, in the last 2 months, she has developed a sudden onset of nihilistic delusion (Cotard syndrome), mainly during the "wearing-off" condition and associated with end of dose dyskinesias and akathisia.As Cotard syndrome clearly improved with the administration of levodopa, the patient was successfully treated changing the levodopa schedule with the shortening of intervals between levodopa intakes in small doses. Both the appearance of the Cotard syndrome in this patient during the "off" state and the subsequent improvement of psychotic symptoms after levodopa administration strongly suggest an important correlation with the dopaminergic dysregulation.This finding suggests that dopaminergic deficit might play a key factor in the development of Cotard syndrome.

Electroversion in treatment of arrhythmia in a patient with Wolff-Parkinson-White syndrome and cervical spinal cord injury

Directory of Open Access Journals (Sweden)

SHEN Peng

2013-06-01

Full Text Available 【Abstract】We report electroversion in treatment of atrial fibrillation (AF and atrioventricular nodal reentry ta-chycardia (AVNRT in a patient with Wolff-Parkinson-White syndrome and cervical spinal cord injury. At first, the pa-tient sustained respiratory failure and weak cough reflex, thereafter repeated bronchoscopy was used to aspirate the sputum as well as control the pneumonia, which resulted in arrhythmia (AF and AVNRT. Two doses of intravenous amiodarone failed to correct the arrhythmia. After restora-tion of sinus rhythm by electroversion, he was successfully weaned from mechanical ventilation and discharged from the intensive care unit without recurrent arrhythmia. Key words: Arrhythmia, cardiac; Atrial fibrillation; Electric countershock; Wolff-Parkinson-White syndrome; Spinal cord injuries

Combined Analysis of CSF Tau, Aβ42, Aβ1–42% and Aβ1–40ox% in Alzheimer's Disease, Dementia with Lewy Bodies and Parkinson's Disease Dementia

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Mirko Bibl

2010-01-01

Full Text Available We studied the diagnostic value of CSF Aβ42/tau versus low Aβ1–42% and high Aβ1–40ox% levels for differential diagnosis of Alzheimer's disease (AD and dementia with Lewy bodies (DLB, respectively. CSF of 45 patients with AD, 15 with DLB, 21 with Parkinson's disease dementia (PDD, and 40 nondemented disease controls (NDC was analyzed by Aβ-SDS-PAGE/immunoblot and ELISAs (Aβ42 and tau. Aβ42/tau lacked specificity in discriminating AD from DLB and PDD. Best discriminating biomarkers were Aβ1–42% and Aβ1–40ox% for AD and DLB, respectively. AD and DLB could be differentiated by both Aβ1–42% and Aβ1–40ox% with an accuracy of 80% at minimum. Thus, we consider Aβ1–42% and Aβ1–40ox% to be useful biomarkers for AD and DLB, respectively. We propose further studies on the integration of Aβ1–42% and Aβ1–40ox% into conventional assay formats. Moreover, future studies should investigate the combination of Aβ1–40ox% and CSF alpha-synuclein for the diagnosis of DLB.

Sugammadex Use in a Patient with Wolff-Parkinson-White (WPW Syndrome

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Sevtap Hekimoğlu Şahin

2015-09-01

Full Text Available Background: Wolff-Parkinson-White (WPW syndrome is a disease associated with episodes of supraventricular tachycardia and ventricular pre-excitation or atrial fibrillation. WPW is characterized by an aberrant electrical conduction pathway between atria and ventricles. Case Report: The major anesthetic problem connected with WPW syndrome is the risk of tachyarrhythmias due to accessory pathway. Therefore, it has been proposed that the aim of anesthetic management should be the avoidance of tachyarrhythmia and sympathetic stimulation. Sugammadex was administered as a neuromuscular reversal agent in this case. To our knowledge, this is the first case report of sugammadex use in a patient with WPW. This report presents a case of general anesthesia management in a patient with WPW syndrome. Conclusion: We think that it is appropriate to use sugammadex to reverse rocuronium for the prevention of sudden hemodynamic changes in patients with WPW who underwent general anesthesia.

Cross-sectional and longitudinal associations of motor fluctuations and non-motor predominance with cerebrospinal τ and Aβ as well as dementia-risk in Parkinson's disease.

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Modreanu, Raluca; Cerquera, Sonia Catalina; Martí, María José; Ríos, José; Sánchez-Gómez, Almudena; Cámara, Ana; Fernández, Manel; Compta, Yaroslau

2017-02-15

Experimental, neuropathological and cerebrospinal fluid (CSF) studies support τ and amyloid-β (Aβ) relevance in Parkinson's disease (PD) related dementia. Lesser motor fluctuations (MFs) and non-motor features have also been related to PD-dementia. Yet, little is known about the association of MFs and non-motor symptoms with CSF τ and Aβ in PD. We hypothesized that lesser MFs and non-motor predominance are related to these CSF markers and dementia-risk in PD. We studied 58 PD patients (dementia at baseline, n=21; dementia at 18-months, n=35) in whom CSF Aβ and τ had been determined with ELISA techniques. MFs and a number of non-motor symptoms (apathy, anxiety, irritability, depression, visual hallucinations, spatial disorientation, memory complaints) over disease course were dichotomized as absent-mild vs. moderate-severe by retrospective clinical chart review blind to CSF findings. Non-motor predominance was defined as ≥3 non-motor symptoms (after the cohort-median of non-motor symptoms per patient) with ≥2 being moderate-severe and ≥1 having been present from onset, with all these being more disabling overall than motor features. Cross-sectionally, CSF biomarkers were non-parametrically compared according to dichotomized MFs and non-motor predominance. Longitudinally, dementia was the outcome (dependent variable), CSF markers, MFs and non-motor predominance were the predictors (independent variables), and potential modifiers as age, sex, and memory complaints were the covariates in binary regression models. Absent-mild MFs were associated with higher CSF τ markers and shorter time-to-dementia, while non-motor predominance and decreasing CSF Aβ independently increased longitudinal dementia-risk. In summary, absent-mild MFs, non-motor predominance and CSF τ and Aβ might define endophenotypes related to the timing or risk of dementia in PD. Copyright © 2016 Elsevier B.V. All rights reserved.

Sudden Death: An Uncommon Occurrence in Dementia with Lewy Bodies.

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Molenaar, Joery P; Wilbers, Joyce; Aerts, Marjolein B; Leijten, Quinten H; van Dijk, Jan G; Esselink, Rianne A; Bloem, Bastiaan R

2016-01-01

We present a 75-year-old woman with dementia and parkinsonism who developed severe orthostatic hypotension and eventually died. Autopsy revealed extensive Lewy body formation in the midbrain, limbic system, intermediate spinal cord, and medulla oblongata. Furthermore, a vast amount of Lewy bodies was seen in the paravertebral sympathetic ganglia which likely explained the severe autonomic failure. We speculate that this autonomic failure caused sudden death through dysregulation of respiration or heart rhythm, reminiscent of sudden death in multiple system atrophy (MSA). Clinicians should be aware of this complication in patients presenting with parkinsonism and autonomic dysfunction, and that sudden death may occur in dementia with Lewy bodies (DLB) as it does in MSA.

Diagnostic criteria for vascular dementia

NARCIS (Netherlands)

Scheltens, P.; Hijdra, A. H.

1998-01-01

The term vascular dementia implies the presence of a clinical syndrome (dementia) caused by, or at least assumed to be caused by, a specific disorder (cerebrovascular disease). In this review, the various sets of criteria used to define vascular dementia are outlined. The various sets of criteria

Extracorporeal life support for cardiac arrest in a 13-year-old girl caused by Wolff-Parkinson-White syndrome.

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Song, Kyoung Hwan; Lee, Byung Kook; Jeung, Kyung Woon; Lee, Dong Hun

2015-10-01

Generally, Wolff-Parkinson-White (WPW) syndrome presents good prognosis. However, several case reports demonstrated malignant arrhythmia or sudden cardiac death as WPW syndrome's first presentation. Cardiopulmonary resuscitation using extracorporeal life support is a therapeutic option in refractory cardiac arrest. We present a WPW syndrome patient who had sudden cardiac arrest as the first presentation of the disease and treated it using extracorporeal life support with good neurologic outcome.

Cerebral correlates of psychotic syndromes in neurodegenerative diseases

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Jellinger, Kurt A

2012-01-01

Abstract Psychosis has been recognized as a common feature in neurodegenerative diseases and a core feature of dementia that worsens most clinical courses. It includes hallucinations, delusions including paranoia, aggressive behaviour, apathy and other psychotic phenomena that occur in a wide range of degenerative disorders including Alzheimer?s disease, synucleinopathies (Parkinson?s disease, dementia with Lewy bodies), Huntington?s disease, frontotemporal degenerations, motoneuron and prion...

Low LDL cholesterol, PCSK9 and HMGCR genetic variation, and risk of Alzheimer's disease and Parkinson's disease: Mendelian randomisation study.

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Benn, Marianne; Nordestgaard, Børge G; Frikke-Schmidt, Ruth; Tybjærg-Hansen, Anne

2017-04-24

Objective  To test the hypothesis that low density lipoprotein (LDL) cholesterol due to genetic variation in the genes responsible for LDL cholesterol metabolism and biosynthesis( PCSK9 and 3-hydroxy-3-methylglutaryl-CoA reductase ( HMGCR ), respectively) is associated with a high risk of Alzheimer's disease, vascular dementia, any dementia, and Parkinson's disease in the general population. Design  Mendelian randomisation study. Setting  Copenhagen General Population Study and Copenhagen City Heart Study. Participants  111 194 individuals from the Danish general population. Main outcome measures  Risk of Alzheimer's disease, vascular dementia, all dementia, and Parkinson's disease. Results  In observational analyses, the multifactorially adjusted hazard ratio for Parkinson's disease in participants with an LDL cholesterol level LDL cholesterol level. In genetic, causal analyses adjusted for age, sex, and year of birth, the risk ratios for a lifelong 1 mmol/L lower LDL cholesterol level were 0.57 (0.27 to 1.17) for Alzheimer's disease, 0.81 (0.34 to 1.89) for vascular dementia, 0.66 (0.34 to 1.26) for any dementia, and 1.02 (0.26 to 4.00) for Parkinson's disease. Summary level data from the International Genomics of Alzheimer's Project using Egger Mendelian randomisation analysis gave a risk ratio for Alzheimer's disease of 0.24 (0.02 to 2.79) for 26 PCSK9 and HMGCR variants, and of 0.64 (0.52 to 0.79) for 380 variants of LDL cholesterol level lowering. Conclusion  Low LDL cholesterol levels due to PCSK9 and HMGCR variants had no causal effect on high risk of Alzheimer's disease, vascular dementia, any dementia, or Parkinson's disease; however, low LDL cholesterol levels may have a causal effect in reducing the risk of Alzheimer's disease. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Differentiating fasciculoventricular pathway from Wolff-Parkinson-White syndrome by electrocardiography.

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Suzuki, Tsugutoshi; Nakamura, Yoshihide; Yoshida, Shuichiro; Yoshida, Yoko; Shintaku, Haruo

2014-04-01

In school-based cardiovascular screening programs in Japan, Wolff-Parkinson-White (WPW) syndrome is diagnosed based on the presence of an electrocardiographic (ECG) delta wave without differentiation from the fasciculoventricular pathway (FVP), although the risk of sudden death is associated only with the former. The purpose of this study was to differentiate FVP patients among children diagnosed with WPW syndrome by ECG. Children who were diagnosed with WPW syndrome through school screening between April 2006 and March 2008 and had QRS width ≤120 ms were included. Patients with asthma and/or coronary heart disease were excluded. FVP and WPW syndrome were differentiated based on ECG responses to adenosine triphosphate (ATP) injection. Age, PR interval, QRS width, and Rosenbaum classification were compared among patients. Thirty patients (median age 12.7 years, range 6.5-15.7 years) participated in the study. FVP was diagnosed in 23 patients (76.7%), and WPW syndrome in 7 (23.3%). In Rosenbaum type A patients, all six patients had WPW syndrome, whereas FVP was diagnosed in 23 of 24 and WPW syndrome was diagnosed in 1 of 24 of type B patients. Age, PR interval, and QRS width were not significantly different between the two conditions. ATP stress test was reliable in differentiating FVP from WPW syndrome. Although FVP is considered rare, the results of our study indicate that many WPW syndrome patients with QRS width ≤120 ms may actually have FVP. Patients categorized as type B are more likely to have FVP, whereas type A patients are most likely to have WPW syndrome. © 2013 Heart Rhythm Society Published by Heart Rhythm Society All rights reserved.

Distinct brain metabolic patterns separately associated with cognition, motor function, and aging in Parkinson's disease dementia.

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Ko, Ji Hyun; Katako, Audrey; Aljuaid, Maram; Goertzen, Andrew L; Borys, Andrew; Hobson, Douglas E; Kim, Seok Min; Lee, Chong Sik

2017-12-01

We explored whether patients with Parkinson's disease dementia (PDD) show a distinct spatial metabolic pattern that characterizes cognitive deficits in addition to motor dysfunction. Eighteen patients with PDD underwent 3 separate positron emission tomography sessions with [ 18 F]fluorodeoxyglucose (for glucose metabolism), fluorinated N-3-fluoropropyl-2-beta-carboxymethoxy-3-beta-(4-iodophenyl) nortropane (for dopamine transporter density) and Pittsburgh compound-B (for beta-amyloid load). We confirmed in PDD versus normal controls, overall hypometabolism in the posterior and prefrontal brain regions accompanied with hypermetabolism in subcortical structures and the cerebellar vermis. A multivariate network analysis then revealed 3 metabolic patterns that are separately associated with cognitive performance (p = 0.042), age (p = 0.042), and motor symptom severity (p = 0.039). The age-related pattern's association with aging was replicated in healthy controls (p = 0.047) and patients with Alzheimer's disease (p = 0.002). The cognition-related pattern's association with cognitive performance was observed, with a trend-level of correlation, in patients with dementia with Lewy bodies (p = 0.084) but not in patients with Alzheimer's disease (p = 0.974). We found no association with fluorinated N-3-fluoropropyl-2-beta-carboxymethoxy-3-beta-(4-iodophenyl) nortropane and Pittsburgh compound-B positron emission tomography with patients' cognitive performance. Copyright © 2017 Elsevier Inc. All rights reserved.

A clinical study in elderly patients with Parkinson's disease using MRI and SPECT

International Nuclear Information System (INIS)

Umahara, Takahiko; Iwamoto, Toshihiko; Takasaki, Masaru; Katsunuma, Hideyo

1991-01-01

Parkinson's disease (PD) is often associated with dementia in elderly patients, and sometimes PD coexists with senile dementia of the Alzheimer type (SDAT) or cerebrovascular disease (CVD) in the elderly. However, since there are few previous clinical studies on the coincidence of, or relationship between PD and CVD, the authors evaluated these aspects in 34 elderly patients with PD using MRI and SPECT. All the patients were over 70 years old. The diagnosis of PD was based on the presence of three symptons (resting tremor, cogwheel rigidity and bradikinesia) which are characteristic of PD, and the effectiveness of L-DOPA therapy. We therefore believe that patients with vascular Parkinsonism were excluded from our study. In 34 cases, 24 (71%) had MRI evidence of CVD (mainly the lacunar state). In the 10 cases who had no CVD, 2 (20%) had severe dementia and the decrease of regional cerebral blood flow (rCBF) in the temporal and parietal lobes bilaterally correlated with the SPECT findings commonly found in SDAT. A comparison of the rCBF and the results of Hasegawa's dementia score (HDS) (verbal intelligence score) was made between the patients with PD associated with CVD and the patients with PD who had no CVD and no SPECT findings which correlated with SDAT. The rCBF in the frontal lobes and the results of the HDS of the former group were significantly lower than those of the latter. As mentioned above, elderly patients with PD often had CVD, leading to dementia. We also pointed out the difficulty in making a differential diagnosis between PD with CVD and vascular Parkinsonism, and the necessity of whether or not the concept of 'mixed type Parkinsonism' should be considered. (author)

18F-FDG PET in Parkinsonism: Differential Diagnosis and Evaluation of Cognitive Impairment.

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Meyer, Philipp T; Frings, Lars; Rücker, Gerta; Hellwig, Sabine

2017-12-01

Accurate differential diagnosis of parkinsonism is of paramount therapeutic and prognostic importance. In addition, with the development of invasive therapies and novel disease-specific therapies, strategies for patient enrichment in trial populations are of growing importance. Imaging disease-specific patterns of regional glucose metabolism with PET and 18 F-FDG allows for a highly accurate distinction between Parkinson disease (PD) and atypical parkinsonian syndromes, including multiple-system atrophy, progressive supranuclear palsy, and corticobasal degeneration. On the basis of a preliminary metaanalysis of currently available studies with inclusion of multiple disease groups, we estimated that the diagnostic sensitivity and specificity for visual PET readings supported by voxel-based statistical analyses for diagnosis of atypical parkinsonian syndromes are 91.4% and 90.6%, respectively. The diagnostic specificity of 18 F-FDG PET for diagnosing multiple-system atrophy, progressive supranuclear palsy, and corticobasal degeneration was consistently shown to be high (>90%), whereas sensitivity was more variable (>75%). It is increasingly acknowledged that cognitive impairment represents a major challenge in PD, with mild cognitive impairment being a prodromal stage of PD with dementia (PDD). In line with clinical and neuropsychologic studies, recent PET studies demonstrated that posterior cortical dysfunction in nondemented PD patients precedes cognitive decline and the development of PDD by several years. Taken together, the current literature underscores the utility of 18 F-FDG PET for diagnostic evaluation of parkinsonism and the promising role of 18 F-FDG PET for assessment and risk stratification of cognitive impairment in PD. © 2017 by the Society of Nuclear Medicine and Molecular Imaging.

Role of atrial fibrillation and atrioventricular conduction (including Wolff-Parkinson-White Syndrome) in sudden death

NARCIS (Netherlands)

Meijler, F.L.; Tweel, I. van der; Herbschleb, J.N.; Hauer, R.N.W.; Robles de Medina, E.O.

A short refractory period of the accessory pathway is considered a major threat for sudden death in patients with Wolff-Parkinson-White syndrome and atrial fibrillation. RR interval and QRS signal analysis together with signal analysis of a bipolar high right atrial electrogram were obtained in six

Cognitive impairment of dementias

OpenAIRE

Medina, L. D.; Rodríguez-Agudelo, Yaneth

2012-01-01

Dementia is a clinical syndrome characterized by a loss of cognitive and emotional abilities of sufficient severity to infer with social or occupational functioning, or both. Although the causes of dementia and characteristics are not always fully understood, it is understood that it is not a natural part of aging. Definitive diagnosis of dementia is made only through the autopsy and although the diagnosis of probable or possible dementia is complex is achieved by the intervention of several ...

Electroencephalography Is a Good Complement to Currently Established Dementia Biomarkers

DEFF Research Database (Denmark)

Ferreira, Daniel; Jelic, Vesna; Cavallin, Lena

2016-01-01

, 135 Alzheimer's disease (AD), 15 dementia with Lewy bodies/Parkinson's disease with dementia (DLB/PDD), 32 other dementias]. The EEG data were recorded in a standardized way. Structural imaging data were visually rated using scales of atrophy in the medial temporal, frontal, and posterior cortex......BACKGROUND/AIMS: Dementia biomarkers that are accessible and easily applicable in nonspecialized clinical settings are urgently needed. Quantitative electroencephalography (qEEG) is a good candidate, and the statistical pattern recognition (SPR) method has recently provided promising results. We......EEG to the diagnostic workup substantially increases the detection of AD pathology even in pre-dementia stages and improves differential diagnosis. EEG could serve as a good complement to currently established dementia biomarkers since it is cheap, noninvasive, and extensively applied outside academic centers....

SAFETY OF AMIODARONE USAGE IN PATIENTS WITH WOLFF-PARKINSON-WHITE SYNDROME AND ATRIAL FIBRILLATION

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D. A. Kuzhel

2010-01-01

Full Text Available Amiodarone is one of the basic antiarrhytmic drugs for atrial fibrillation treatment. However application of amiodarone in patients with atrial fibrillation and Wolff-Parkinson-White syndrome can induce ventricular fibrillation. Amiodarone usage in these patients should be accompanied by readiness for performance of resuscitation. This is confirmed by clinical case presentation.

Stereotypic behaviors in degenerative dementias.

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Prioni, S; Fetoni, V; Barocco, F; Redaelli, V; Falcone, C; Soliveri, P; Tagliavini, F; Scaglioni, A; Caffarra, P; Concari, L; Gardini, S; Girotti, F

2012-11-01

Stereotypies are simple or complex involuntary/unvoluntary behaviors, common in fronto-temporal dementia (FTD), but not studied in other types of degenerative dementias. The aim was to investigate stereotypy frequency and type in patients with FTD, Alzheimer's disease (AD), progressive supranuclear palsy (PSP) and Parkinson's disease with dementia (PDD) in a multicenter observational study; and to investigate the relation of stereotypies to cognitive, behavioral and motor impairment. One hundred fifty-five consecutive outpatients (45 AD, 40 FTD, 35 PSP and 35 PDD) were studied in four hospitals in northern Italy. Stereotypies were examined by the five-domain Stereotypy Rating Inventory. Cognition was examined by the Mini Mental State and Frontal Assessment Battery, neuropsychiatric symptoms by the Neuropsychiatric Inventory, and motor impairment and invalidity by the Unified Parkinson's Disease Rating Scale part III, and activities of daily living. Stereotypies were present in all groups. FTD and PDD had the greatest frequency of one-domain stereotypies; FTD also had the greatest frequency of two-or-more domain stereotypies; movement stereotypies were the most common stereotypies in all groups. AD patients had fewer stereotypies than the other groups. Stereotypies are not exclusive to FTD, but are also fairly common in PSP and PDD, though less so in AD. Stereotypies may be underpinned by dysfunctional striato-frontal circuits, known to be damaged in PSP and PDD, as well as FTD.

Nonmotor Features in Atypical Parkinsonism.

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Bhatia, Kailash P; Stamelou, Maria

2017-01-01

Atypical parkinsonism (AP) comprises mainly multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD), which are distinct pathological entities, presenting with a wide phenotypic spectrum. The classic syndromes are now called MSA-parkinsonism (MSA-P), MSA-cerebellar type (MSA-C), Richardson's syndrome, and corticobasal syndrome. Nonmotor features in AP have been recognized almost since the initial description of these disorders; however, research has been limited. Autonomic dysfunction is the most prominent nonmotor feature of MSA, but also gastrointestinal symptoms, sleep dysfunction, and pain, can be a feature. In PSP and CBD, the most prominent nonmotor symptoms comprise those deriving from the cognitive/neuropsychiatric domain. Apart from assisting the clinician in the differential diagnosis with Parkinson's disease, nonmotor features in AP have a big impact on quality of life and prognosis of AP and their treatment poses a major challenge for clinicians. © 2017 Elsevier Inc. All rights reserved.

Percutenous Catheter Ablation of the Accessory Pathway in a Patient with Wolff-Parkinson-White Syndrome Associated with Familial Atrial Fibrillation

Science.gov (United States)

Cay, Serkan; Topaloglu, Serkan; Aras, Dursun

2008-01-01

Percutenous catheter ablation of the accessory pathway in Wolff-Parkinson-White syndrome is a highly successful mode of therapy. Sudden cardiac arrest survivors associated with WPW syndrome should undergo radiofrequency catheter ablation. WPW syndrome associated with familial atrial fibrillation is a very rare condition. Herein, we describe a case who presented with sudden cardiac arrest secondary to WPW syndrome and familial atrial fibrillation and treated via radiofrequency catheter ablation. PMID:18379660

Clinical characteristics with an impact on ADL functions of PD patients with cognitive impairment indicative of dementia.

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Inga Liepelt-Scarfone

Full Text Available BACKGROUND: Dementia in Parkinson's disease (PD is defined as cognitive decline severe enough to affect activities of daily living function (ADL. The aim of our exploratory study was to compare two groups of PD patients. Both groups had cognitive deficits severe enough to justify diagnosis of dementia, but they differed according to caregivers' rating on ADL dysfunction. Parameters which differed between the two groups were interpreted to affect the caregivers' perception of ADL dysfunction in PD patients with cognitive impairment indicative of Parkinson's disease dementia. METHODOLOGY/PRINCIPAL FINDINGS: Thirty of 131 Parkinson's disease patients fulfilled the Movement Disorders Society Task Force - recommended, cognitive Level-I-criteria for dementia. According to standardized caregiver ratings, volunteers were grouped into 18 patients with (ADL- and 12 without instrumental activities of daily living dysfunction (ADL+. Caregiver activities of daily living function ratings closely correlated with self-estimates of patients and those of physician (p<0.001. ADL- patients performed worse on tests assessing visual-construction (p<0.05 and attention (p=0.03 than ADL+ patients. Moreover, the postural instability and gait disorder subtype was more frequent in ADL- patients (p=0.009. ADL- patients tended to have more communication problems (p=0.05, more anxiety (p=0.05 and showed a tendency to be treated more often with neuroleptics (p=0.049 than ADL+. CONCLUSIONS/SIGNIFICANCE: Results indicate that worse attention, visual-construction abilities, the postural instability and gait disorder subtype, communication problems, medication and presence of anxiety are related to activities of daily living dysfunctions in Parkinson's disease patients with cognitive decline indicative of dementia. Our data suggests that not only cognitive factors but also non-cognitive factors seem to be linked to the diagnosis of Parkinson's disease dementia associated with

Dementias show differential physiological responses to salient sounds.

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Fletcher, Phillip D; Nicholas, Jennifer M; Shakespeare, Timothy J; Downey, Laura E; Golden, Hannah L; Agustus, Jennifer L; Clark, Camilla N; Mummery, Catherine J; Schott, Jonathan M; Crutch, Sebastian J; Warren, Jason D

2015-01-01

Abnormal responsiveness to salient sensory signals is often a prominent feature of dementia diseases, particularly the frontotemporal lobar degenerations, but has been little studied. Here we assessed processing of one important class of salient signals, looming sounds, in canonical dementia syndromes. We manipulated tones using intensity cues to create percepts of salient approaching ("looming") or less salient withdrawing sounds. Pupil dilatation responses and behavioral rating responses to these stimuli were compared in patients fulfilling consensus criteria for dementia syndromes (semantic dementia, n = 10; behavioral variant frontotemporal dementia, n = 16, progressive nonfluent aphasia, n = 12; amnestic Alzheimer's disease, n = 10) and a cohort of 26 healthy age-matched individuals. Approaching sounds were rated as more salient than withdrawing sounds by healthy older individuals but this behavioral response to salience did not differentiate healthy individuals from patients with dementia syndromes. Pupil responses to approaching sounds were greater than responses to withdrawing sounds in healthy older individuals and in patients with semantic dementia: this differential pupil response was reduced in patients with progressive nonfluent aphasia and Alzheimer's disease relative both to the healthy control and semantic dementia groups, and did not correlate with nonverbal auditory semantic function. Autonomic responses to auditory salience are differentially affected by dementias and may constitute a novel biomarker of these diseases.

Percutenous Catheter Ablation of the Accessory Pathway in a Patient with Wolff-Parkinson-White Syndrome Associated with Familial Atrial Fibrillation

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Serkan Cay

2008-04-01

Full Text Available Percutenous catheter ablation of the accessory pathway in Wolff-Parkinson-White syndrome is a highly successful mode of therapy. Sudden cardiac arrest survivors associated with WPW syndrome should undergo radiofrequency catheter ablation. WPW syndrome associated with familial atrial fibrillation is a very rare condition. Herein, we describe a case who presented with sudden cardiac arrest secondary to WPW syndrome and familial atrial fibrillation and treated via radiofrequency catheter ablation.

Opening up the DNA methylome of dementia.

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Delgado-Morales, R; Esteller, M

2017-04-01

Dementia is a complex clinical condition characterized by several cognitive impairments that interfere with patient independence in executing everyday tasks. Various neurodegenerative disorders have dementia in common among their clinical manifestations. In addition, these diseases, such as Alzheimer's disease, Parkinson's disease, dementia with Lewy bodies and frontotemporal dementia, share molecular alterations at the neuropathological level. In recent years, the field of neuroepigenetics has expanded massively and it is now clear that epigenetic processes, such as DNA methylation, are mechanisms involved in both normal and pathological brain function. Despite the persistent methodological and conceptual caveats, it has been reported that several genes fundamental to the development of neurodegenerative disorders are deregulated by aberrant methylation patterns of their promoters, and even common epigenetic signatures for some dementia-associated pathologies have been identified. Therefore, understanding the epigenetic mechanisms that are altered in dementia, especially those associated with the initial phases, will allow us not only to understand the etiopathology of dementia and its progression but also to design effective therapies to reduce this global public health problem. This review provides an in-depth summary of our current knowledge about DNA methylation in dementia, focusing exclusively on the analyses performed in human brain.

Reduced sympathetic activity in idiopathic rapid-eye-movement sleep behavior disorder and Parkinson's disease

DEFF Research Database (Denmark)

Sorensen, Gertrud Laura; Mehlsen, Jesper; Jennum, Poul

2013-01-01

More than 50% of patients with idiopathic REM sleep behavior disorder (iRBD) will develop Parkinson's disease or Lewy body dementia. In a previous study, we found attenuated heart rate responses in iRBD and Parkinson's disease patients during sleep. The current study aimed to evaluate heart rate...... variability further in order to identify possible changes in these components during wakefulness and sleep in patients with iRBD and Parkinson's disease....

Visual Hallucinations in PD and Lewy Body Dementias: Old and New Hypotheses

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M. Onofrj

2013-01-01

Full Text Available Visual Hallucinations (VH are a common non-motor symptom of Parkinson’s Disease (PD and the Lewy body dementias (LBD of Parkinson's disease with dementia (PDD and Dementia with Lewy Bodies (DLB. The origin of VH in PD and LBD is debated: earlier studies considered a number of different possible mechanisms underlying VH including visual disorders, Rapid Eye Movement (REM Sleep Intrusions, dysfunctions of top down or bottom up visual pathways, and neurotransmitter imbalance.

Low frequency of bipolar disorder, dopamine dysregulation syndrome, and punding in Brazilian patients with Parkinson's disease Baixa frequência de transtorno bipolar, síndrome de desregulação dopaminérgica e punding em pacientes brasileiros com doença de Parkinson

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Arthur Kummer

2010-03-01

Full Text Available OBJECTIVE: To investigate the frequency of bipolar disorder, dopamine dysregulation syndrome and punding in Parkinson's disease patients from a Brazilian movement disorders clinic. METHOD: One hundred patients underwent a comprehensive psychiatric examination composed of MINI-plus and specific questionnaires to investigate dopamine dysregulation syndrome and punding. RESULTS: We identified, respectively, one and five Parkinson's disease patients with bipolar disorder type I and type II. All manic/hypomanic episodes occurred before Parkinson's disease onset. No patient was identified with dopamine dysregulation syndrome or punding. CONCLUSION: The frequency of manic/hypomanic episodes seems to decrease with Parkinson's disease onset, and local environmental factors (e.g. drug availability may be responsible for the low frequency of dopamine dysregulation syndrome and punding in Brazilian Parkinson's disease patients.OBJETIVO: Investigar a frequência de transtorno bipolar, síndrome de desregulação dopaminérgica e punding em pacientes com doença de Parkinson de uma clínica de movimentos anormais no Brasil. MÉTODO: Cem pacientes foram submetidos à avaliação psiquiátrica composta pelo MINI-Plus e questionários específicos para investigar síndrome de desregulação dopaminérgica e punding. RESULTADOS: Identificamos, respectivamente, um e cinco pacientes com transtorno bipolar tipo I e tipo II. Todos os episódios maníacos/hipomaníacos ocorreram antes do início da doença de Parkinson. Nenhum paciente foi identificado com síndrome de desregulação dopaminérgica ou punding. CONCLUSÃO: A frequência de episódios maníacos/ hipomaníacos parece declinar com o início da doença de Parkinson. Fatores ambientais locais (p.ex.: disponibilidade de drogas podem ser responsáveis pela baixa frequência de síndrome de desregulação dopaminérgica e punding em pacientes brasileiros com doença de Parkinson.

Phase analysis in patients with Wolff-Parkinson-White syndrome

International Nuclear Information System (INIS)

Nakajima, Kenichi; Bunko, Hisashi; Tada, Akira; Taki, Junichi; Tonami, Norihisa

1983-01-01

Twenty-five patients with Wolff-Parkinson-White (WPW) syndrome who underwent surgical division of the accessory conduction pathway (ACP) were studied by gated blood pool studies and phase analyses. All of 11 patients with right cardiac type (R-type) had abnormal initial phase in the right ventricle (RV), while 10 out of 14 patients with left cardiac type (L-type) had initial phase in the left ventricle (LV). However, in 4 L-type patients, there were no significant differences in the initiation of both ventricular contractions. In 10 patients who had radionuclide studies before and after surgical division of the ACP, the ventricular contraction patterns were apparently changed and the abnormal wall motions induced by the presence of ACPs disappeared. These observations indicate that the abnormal initial contraction is associated with pre-excitation of WPW syndrome. Sensitivities to identify the side of preexcitation were 100% (11/11) for R-type and 71% (10/14) for L-type. However, regarding the detection of the precise site of ACP, the agreement was 48% (12/25). Therefore, as a method of preoperative study, it seemed difficult to identify the precise localization of the ACP by phase analysis alone. Phase analysis provided interesting informations and was useful for evaluating patients with WPW syndrome before and after surgery. (author)

Wolff-Parkinson-White syndrome concomitant with idiopathic ventricular fibrillation associated with inferior early repolarization.

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Takahashi, Naohiko; Shinohara, Tetsuji; Hara, Masahide; Saikawa, Tetsunori

2012-01-01

We encountered a 39-year-old man with documented ventricular fibrillation (VF). His ECGs showed intermittent Wolff-Parkinson-White (WPW) syndrome pattern. During electrophysiological study, no ventricular preexcitation was observed. An accessory pathway located at the posterior mitral annulus was identified, and successfully eliminated by radiofrequency catheter ablation. VF was not induced. His ECGs in the absence of delta waves demonstrated early repolarization in the inferior leads. This case raises the possibility that patients with manifest WPW syndrome may have an arrhythmogenic substrate associated with early repolarization, and the characteristic J waves can be masked by the presence of ventricular preexcitation.

Internet-based screening for dementia risk.

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Brandt, Jason; Sullivan, Campbell; Burrell, Larry E; Rogerson, Mark; Anderson, Allan

2013-01-01

The Dementia Risk Assessment (DRA) is an online tool consisting of questions about known risk factors for dementia, a novel verbal memory test, and an informant report of cognitive decline. Its primary goal is to educate the public about dementia risk factors and encourage clinical evaluation where appropriate. In Study 1, more than 3,000 anonymous persons over age 50 completed the DRA about themselves; 1,000 people also completed proxy reports about another person. Advanced age, lower education, male sex, complaints of severe memory impairment, and histories of cerebrovascular disease, Parkinson's disease, and brain tumor all contributed significantly to poor memory performance. A high correlation was obtained between proxy-reported decline and actual memory test performance. In Study 2, 52 persons seeking first-time evaluation at dementia clinics completed the DRA prior to their visits. Their responses (and those of their proxy informants) were compared to the results of independent evaluation by geriatric neuropsychiatrists. The 30 patients found to meet criteria for probable Alzheimer's disease, vascular dementia, or frontotemporal dementia differed on the DRA from the 22 patients without dementia (most other neuropsychiatric conditions). Scoring below criterion on the DRA's memory test had moderately high predictive validity for clinically diagnosed dementia. Although additional studies of larger clinical samples are needed, the DRA holds promise for wide-scale screening for dementia risk.

[123I]FP-CIT (DaTSCAN) and SPET in the diagnostics of Parkinson's disease and Parkinsonian syndromes

International Nuclear Information System (INIS)

Chmielowski, K.; Szalus, B.; Pietrzykowski, J.; Brodacki, B.; Kotowicz, J.; Skrobowska, E.

2003-01-01

The aim of study was to verify the diagnostic value of the radiopharmaceutic [ 123I ]FP-CIT (DaTSCAN) in functional imaging of the presynaptical dopaminergic system in patients with Parkison's disease and parkinsonian syndromes: multiple system atrophy, orthostatic hypotonia Shy-Drager, essential tremor. That pilot study group consisted of 8 patients in which either preliminary diagnosis or suspicion of Parkinson's disease, parkinsonian syndrome or multiple system atrophy was set. Imaging of the brain with SPET (dual head detector Varicam Elscint) and MRI were performed. The radiopharmaceutic [ 123I ] FP-CIT (DaTSCAN) was administered intravenously in the dose 145 -148 MBq. SPET images were reconstructed by filtered backprojection with the use of Butterworth filter. The images were inspected visually. Images from SPET and MRI were superimposed by means of the workstation Hermes (Nucklear Diagnostic) with designatad regions interest (ROI) in the striatum and occipital cortex in order to assess semiquantitatively the binding of dopamine transporter. In the group of 8 patients evaluated with the use of [ 123I ]FP-CIT DaTSCAN four had normal results, and four - abnormal. The preliminary diagnosis was sustained in 3/8 of patients (including Parkinson's disease in two patients and multiple system atrophy in one patient). In the remainig 5 patients the preliminary diagnosis was changed, namely: in 2 cases the essential tremor was diagnosed, in 1 case - Parkinson's disease, in 1 case - orthostaic Sky-Drager, and in 1 case - despite the tremor of the upper limbs - results were normal. In all 8 patients the tracer proved to be useful in the confirmation of clinical diagnosis, especially in the differentiation between the essential tremor and Parkinson's disease. In the case of multiple system atrophy the imaging revealed significant loss of nigrostriatal dopaminergic neurons. Such loss was observed also in the cases of Parkinson's disease affecting the posterior parts of the

Cognitive impairment in Parkinson's disease

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Jing YUAN

2017-07-01

Full Text Available Parkinson's disease cognitive impairment (PD-CI is one of the major non-motor symtoms (NMS of PD, including Parkinson's disease with mild cognitive impairment (PD - MCI and Parkinson's disease dementia (PDD. Executive dysfunction is relatively prominent, but other cognitive domains as visuospatial ability, memory and language can also be affected. Main risk factors for PD-CI include male gender, advanced age, low education, severe motor symptoms, low baseline cognitive function and excessive daytime sleepiness (EDS. Lewy bodies are main pathological changes, and Alzheimer's disease (AD related pathological changes can also be seen. The application value of decreased α?synuclein (α-Syn and β-amyloid 1-42 (Aβ1-42 levels in cerebrospinal fluid (CSF as biomarkers remains controversial. There are few related research and no defined pathogenic genes currently. Both dopaminergic pathway and acetylcholinergic pathway are involved in the occurrence of PD - CI as demonstrated in PET studies. Cortical and subcortical atrophy are associated with PD - CI as observed in MRI studies. Olfactory dysfunction may be one of the predictors of cognitive impairment. PDD and dementia with Lewy bodies (DLB share common biological characteristics, therefore the differential diagnosis sometimes is difficult. Cholinesterase inhibitors (ChEIs and memantine help to improve clinical symptoms, but treatment decision should be made with individualization. Cognitive behavioral treatment (CBT has potential clinical value and should be investigated by more studies. DOI: 10.3969/j.issn.1672-6731.2017.06.004

Normalization of reverse redistribution of thallium-201 with procainamide pretreatment in Wolff-Parkinson-White syndrome

International Nuclear Information System (INIS)

Nii, T.; Nakashima, Y.; Nomoto, J.; Hiroki, T.; Ohshima, F.; Arakawa, K.

1991-01-01

Stress thallium-201 myocardial perfusion imaging was performed in a patient with Wolff-Parkinson-White syndrome. Reverse redistribution phenomenon was observed in the absence of coronary artery disease. This seems to be the first report of normalization of this phenomenon in association with reversion of accessory pathway to normal atrioventricular conduction after pretreatment with procainamide

Normalization of reverse redistribution of thallium-201 with procainamide pretreatment in Wolff-Parkinson-White syndrome

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Nii, T.; Nakashima, Y.; Nomoto, J.; Hiroki, T.; Ohshima, F.; Arakawa, K. (Fukuoka Univ. School of Medicine (Japan))

1991-03-01

Stress thallium-201 myocardial perfusion imaging was performed in a patient with Wolff-Parkinson-White syndrome. Reverse redistribution phenomenon was observed in the absence of coronary artery disease. This seems to be the first report of normalization of this phenomenon in association with reversion of accessory pathway to normal atrioventricular conduction after pretreatment with procainamide.

Dementias show differential physiological responses to salient sounds

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Phillip David Fletcher

2015-03-01

Full Text Available Abnormal responsiveness to salient sensory signals is often a prominent feature of dementia diseases, particularly the frontotemporal lobar degenerations, but has been little studied. Here we assessed processing of one important class of salient signals, looming sounds, in canonical dementia syndromes. We manipulated tones using intensity cues to create percepts of salient approaching (‘looming’ or less salient withdrawing sounds. Pupil dilatation responses and behavioural rating responses to these stimuli were compared in patients fulfilling consensus criteria for dementia syndromes (semantic dementia, n=10; behavioural variant frontotemporal dementia, n=16, progressive non-fluent aphasia, n=12; amnestic Alzheimer’s disease, n=10 and a cohort of 26 healthy age-matched individuals. Approaching sounds were rated as more salient than withdrawing sounds by healthy older individuals but this behavioural response to salience did not differentiate healthy individuals from patients with dementia syndromes. Pupil responses to approaching sounds were greater than responses to withdrawing sounds in healthy older individuals and in patients with semantic dementia: this differential pupil response was reduced in patients with progressive nonfluent aphasia and Alzheimer’s disease relative both to the healthy control and semantic dementia groups, and did not correlate with nonverbal auditory semantic function. Autonomic responses to auditory salience are differentially affected by dementias and may constitute a novel biomarker of these diseases.

Dementias show differential physiological responses to salient sounds

Science.gov (United States)

Fletcher, Phillip D.; Nicholas, Jennifer M.; Shakespeare, Timothy J.; Downey, Laura E.; Golden, Hannah L.; Agustus, Jennifer L.; Clark, Camilla N.; Mummery, Catherine J.; Schott, Jonathan M.; Crutch, Sebastian J.; Warren, Jason D.

2015-01-01

Abnormal responsiveness to salient sensory signals is often a prominent feature of dementia diseases, particularly the frontotemporal lobar degenerations, but has been little studied. Here we assessed processing of one important class of salient signals, looming sounds, in canonical dementia syndromes. We manipulated tones using intensity cues to create percepts of salient approaching (“looming”) or less salient withdrawing sounds. Pupil dilatation responses and behavioral rating responses to these stimuli were compared in patients fulfilling consensus criteria for dementia syndromes (semantic dementia, n = 10; behavioral variant frontotemporal dementia, n = 16, progressive nonfluent aphasia, n = 12; amnestic Alzheimer's disease, n = 10) and a cohort of 26 healthy age-matched individuals. Approaching sounds were rated as more salient than withdrawing sounds by healthy older individuals but this behavioral response to salience did not differentiate healthy individuals from patients with dementia syndromes. Pupil responses to approaching sounds were greater than responses to withdrawing sounds in healthy older individuals and in patients with semantic dementia: this differential pupil response was reduced in patients with progressive nonfluent aphasia and Alzheimer's disease relative both to the healthy control and semantic dementia groups, and did not correlate with nonverbal auditory semantic function. Autonomic responses to auditory salience are differentially affected by dementias and may constitute a novel biomarker of these diseases. PMID:25859194

Language and Dementia: Neuropsychological Aspects

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Kempler, Daniel; Goral, Mira

2008-01-01

This article reviews recent evidence for the relationship between extralinguistic cognitive and language abilities in dementia. A survey of data from investigations of three dementia syndromes (Alzheimer's disease, semantic dementia and progressive nonfluent aphasia) reveals that, more often than not, deterioration of conceptual organization appears associated with lexical impairments, whereas impairments in executive function are associated with sentence- and discourse-level deficits. These ...

Evaluation of Parkinson's disease using magnetic resonance imaging

International Nuclear Information System (INIS)

Vedolin, Leonardo; Marchiori, Edson; Rieder, Carlos

2004-01-01

The objective of this study was to evaluate the magnetic resonance imaging findings in patients with Parkinson's disease. In the period from October 1999 to October 2002, 42 patients with parkinsonism were investigated using a 1.5 T MR equipment. Patients were divided into two groups: patients with Parkinson's disease (n = 26) and patients with atypical Parkinsonian syndrome (n = 16). The results were compared with a control group (n = 18). The following variables were evaluated: thickness of the mesencephalon compact pars, hypointense signal in the putamen, degree of brain atrophy, lesions in the mesencephalon, lesions in the white matter, and the presence of lesions in the posterior-lateral edge of the putamen. Statistical data analysis was carried out using the SPSS program. Results: The mean age was 58.2 years for the Parkinson's disease and control groups, and 60.5 years for the atypical Parkinsonian syndrome group. Patients with Parkinson's disease and atypical Parkinsonian syndromes presented decreased thickness of the compact pars and a higher degree of signal hypointensity in the putamen. Cerebral atrophy was more prominent in the patients with atypical Parkinsonian syndrome. Lesions in mesencephalon and white matter were similar in both groups. The frequency of hyperintense signal in the posterior-lateral edge of the putamen was low within the studied population, although that could suggest multiple-system atrophy. Magnetic resonance imaging allows the detection of brain morphological changes that may help in the diagnosis of Parkinsonian syndromes. (author)

Parkinson disease: α-synuclein mutational screening and new clinical insight into the p.E46K mutation.

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Pimentel, Márcia M G; Rodrigues, Fabíola C; Leite, Marco Antônio A; Campos Júnior, Mário; Rosso, Ana Lucia; Nicaretta, Denise H; Pereira, João S; Silva, Delson José; Della Coletta, Marcus V; Vasconcellos, Luiz Felipe R; Abreu, Gabriella M; Dos Santos, Jussara M; Santos-Rebouças, Cíntia B

2015-06-01

Amongst Parkinson's disease-causing genetic factors, missense mutations and genomic multiplications in the gene encoding α-synuclein are well established causes of the disease, although genetic data in populations with a high degree of admixture, such as the Brazilian one, are still scarce. In this study, we conducted a molecular screening of α-synuclein point mutations and copy number variation in the largest cohort of Brazilian patients with Parkinson's disease (n = 549) and also in twelve Portuguese and one Bolivian immigrants. Genomic DNA was isolated from peripheral blood leukocytes or saliva, and the mutational screening was performed by quantitative and qualitative real-time PCR. The only alteration identified was the p.E46K mutation in a 60-year-old man, born in Bolivia, with a familial history of autosomal dominant Parkinson's disease. This is the second family ever reported, in which this rare pathogenic mutation is segregating. The same mutation was firstly described ten years ago in a Spanish family with a neurodegenerative syndrome combining parkinsonism, dementia and visual hallucinations. The clinical condition of our proband reveals a less aggressive phenotype than previously described and reinforces that marked phenotypic heterogeneity is common among patients with Parkinson's disease, even among those carriers sharing the same mutation. Our findings add new insight into the preexisting information about α-synuclein p.E46K, improving our understanding about the endophenotypes associated to this mutation and corroborate that missense alterations and multiplications in α-synuclein are uncommon among Brazilian patients with Parkinson's disease. Copyright © 2015 Elsevier Ltd. All rights reserved.

Probable association of tachyarrhythmia with nebulized albuterol in a child with previously subclinical wolff Parkinson white syndrome

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Kroesen, M.; Maseland, M.; Smal, J.; Reimer, A.; van Setten, P.

2012-01-01

We present the case of a 2-year-old asthmatic boy with atrioventricular (AV)-reentry tachycardia following albuterol inhalation, who was later diagnosed with Wolff-Parkinson-White (WPW) syndrome. The Naranjo adverse drug reaction probability scale score for this adverse event was 7, indicating that

[Neurological syndromes linked with the intake of plants and fungi containing a toxic component (I). Neurotoxic syndromes caused by the ingestion of plants, seeds and fruits].

Science.gov (United States)

Carod-Artal, F J

A wide range of plants, seeds and fruits used for nutritional and medicinal purposes can give rise to neurotoxic symptoms. We review the neurological pathology associated with the acute or chronic consumption of plants, seeds and fruits in human beings and in animals. Of the plants that can trigger acute neurotoxic syndromes in humans, some of the most notable include Mandragora officinalis, Datura stramonium, Conium maculatum (hemlock), Coriaria myrtifolia (redoul), Ricinus communis, Gloriosa superba, Catharanthus roseus, Karwinskia humboldtiana and Podophyllum pelatum. We also survey different neurological syndromes linked with the ingestion of vegetable foodstuffs that are rich in cyanogenic glycosides, Jamaican vomiting sickness caused by Blighia sapida, Parkinson dementia ALS of Guam island and exposition to Cycas circinalis, Guadeloupean parkinsonism and exposition to Annonaceae, konzo caused by ingestion of wild manioc and neurolathyrism from ingestion of Lathyrus sativus, the last two being models of motor neurone disease. Locoism is a chronic disease that develops in livestock feeding on plants belonging to Astragalus and Oxytropis sp., Sida carpinifolia and Ipomea carnea, which are rich in swainsonine, a toxin that inhibits the enzyme alpha mannosidase and induces a cerebellar syndrome. The ingestion of neurotoxic seeds, fruits and plants included in the diet and acute poisoning by certain plants can give rise to different neurological syndromes, some of which are irreversible.

"It's All Changed:" Carers' Experiences of Caring for Adults Who Have Down's Syndrome and Dementia

Science.gov (United States)

McLaughlin, Katrina; Jones, Aled

2011-01-01

A qualitative interview study was undertaken to determine the information and support needs of carers of adults who have Down's syndrome and dementia. The data were analysed thematically. Carers' information and support needs were seen to change at pre-diagnosis, diagnosis and post-diagnosis. Helping carers to manage the changing nature of the…

Digital 3D reconstruction of two parahissian accessory bundles in a case of Wolff-Parkinson White syndrome

NARCIS (Netherlands)

Steffen, C.; Schaepman, M.E.; Hardmeier, T.; Schweitzer, W.

2004-01-01

Three-dimensional reconstruction of digitized histological serial sections of the cardiac conduction system yielded two accessory pathways in a case of a 24-day-old male infant who died after a short period of illness with ECG findings of Wolff-Parkinson-White syndrome. In infants, the differential

Differential Classification of Dementia

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E. Mohr

1995-01-01

Full Text Available In the absence of biological markers, dementia classification remains complex both in terms of characterization as well as early detection of the presence or absence of dementing symptoms, particularly in diseases with possible secondary dementia. An empirical, statistical approach using neuropsychological measures was therefore developed to distinguish demented from non-demented patients and to identify differential patterns of cognitive dysfunction in neurodegenerative disease. Age-scaled neurobehavioral test results (Wechsler Adult Intelligence Scale—Revised and Wechsler Memory Scale from Alzheimer's (AD and Huntington's (HD patients, matched for intellectual disability, as well as normal controls were used to derive a classification formula. Stepwise discriminant analysis accurately (99% correct distinguished controls from demented patients, and separated the two patient groups (79% correct. Variables discriminating between HD and AD patient groups consisted of complex psychomotor tasks, visuospatial function, attention and memory. The reliability of the classification formula was demonstrated with a new, independent sample of AD and HD patients which yielded virtually identical results (classification accuracy for dementia: 96%; AD versus HD: 78%. To validate the formula, the discriminant function was applied to Parkinson's (PD patients, 38% of whom were classified as demented. The validity of the classification was demonstrated by significant PD subgroup differences on measures of dementia not included in the discriminant function. Moreover, a majority of demented PD patients (65% were classified as having an HD-like pattern of cognitive deficits, in line with previous reports of the subcortical nature of PD dementia. This approach may thus be useful in classifying presence or absence of dementia and in discriminating between dementia subtypes in cases of secondary or coincidental dementia.

Death certificate data and causes of death in patients with parkinsonism.

Science.gov (United States)

Moscovich, Mariana; Boschetti, Gabriela; Moro, Adriana; Teive, Helio A G; Hassan, Anhar; Munhoz, Renato P

2017-08-01

Assessment of variables related to mortality in Parkinson disease (PD) and other parkinsonian syndromes relies, among other sources, on accurate death certificate (DC) documentation. We assessed the documentation of the degenerative disorder on DCs and evaluated comorbidities and causes of death among parkinsonian patients. Demographic and clinical data were systematically and prospectively collected on deceased patients followed at a tertiary movement disorder clinic. DCs data included the documentation of parkinsonism, causes, and place of death. Among 138 cases, 84 (60.9%) male, mean age 77.9 years, mean age of onset 66.7, and mean disease duration 10.9 years. Clinical diagnoses included PD (73.9%), progressive supranuclear palsy (10.9%), multiple system atrophy (7.2%), Lewy body dementia (7.2%) and corticobasal degeneration (0.7%). Psychosis occurred in 60.1% cases, dementia in 48.5%. Most PD patients died due to heterogeneous causes before reaching advanced stages. Non-PD parkinsonian patients died earlier due to causes linked to the advanced neurodegenerative process. PD was documented in 38.4% of DCs with different forms of inconsistencies. That improved, but remained significant when it was signed by a specialist. More than half of PD cases died while still ambulatory and independent, after a longer disease course and due to causes commonly seen in that age group. Deaths among advanced PD patients occurred due to causes similar to what we found in non-PD cases. These findings can be useful for clinical, prognostic and counseling purposes. Underlying parkinsonian disorders are poorly documented in DCs, undermining its' use as sources of data collection. Copyright © 2017 Elsevier Ltd. All rights reserved.

Risk Factors for Dementia in People with Down Syndrome: Issues in Assessment and Diagnosis

Science.gov (United States)

Bush, Alick; Beail, Nigel

2004-01-01

It has been clearly established that there is an increased incidence of early onset dementia of the Alzheimer type (DAT) in people who have Down syndrome. There are variations in the age of onset of the clinical signs of DAT, which may be accounted for by different risk factors. In this review we examined the evidence that different biological and…

Vascular risk factors, cognitve decline, and dementia

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E Duron

2008-04-01

Full Text Available E Duron, Olivier HanonBroca Hospital, Paris, FranceAbstract: Dementia is one of the most important neurological disorders in the elderly. Aging is associated with a large increase in the prevalence and incidence of degenerative (Alzheimer’s disease and vascular dementia, leading to a devastating loss of autonomy. In view of the increasing longevity of populations worldwide, prevention of dementia has turned into a major public health challenge. In the past decade, several vascular risk factors have been found to be associated with vascular dementia but also Alzheimer’s disease. Some longitudinal studies, have found significant associations between hypertension, diabetus mellitus, and metabolic syndrome, assessed at middle age, and dementia. Studies assessing the link between hypercholesterolemia, atrial fibrillation, smoking, and dementia have given more conflicting results. Furthermore, some studies have highlighted the possible protective effect of antihypertensive therapy on cognition and some trials are evaluating the effects of statins and treatments for insulin resistance. Vascular risk factors and their treatments are a promising avenue of research for prevention of dementia, and further long-term, placebo-controlled, randomized studies, need to be performed.Keywords: dementia, hypertension, diabetus mellitus, hypercholesterolemia, metabolic syndrome

rCBF SPECT in Parkinson's disease patients with mental dysfunction

International Nuclear Information System (INIS)

Bissessur, S.; Tissingh, G.; Wolters, E.C.; Scheltens, P.

1997-01-01

Functional imaging of the brain using SPECT provides information correlative to the alterations of regional blood flow. In this paper we review the literature pertaining to SPECT in Parkinson's disease with and without dementia and depression. Parkinson's disease itself is not associated with a consistent pattern of cerebral blood flow alterations in the basal ganglia, but reduced parietal blood flow is more often reported. The heterogeneity of blood flow changes possibly reflects the multifactorial pathophysiology of the disease. In demented Parkinson's disease patients frontal hypoperfusion is often found or bilateral temporoparietal deficits, probably indicative of concomitant Alzheimer's disease. The SPECT studies undertaken in depressed patients with and without Parkinson's disease show highly conflicting and inconsistent results, probably due to methodological and diagnostic flaws (especially the inclusion of demented Parkinson patients). Several lines of reasoning point to a prefrontal dysfunction and future SPECT studies are planned to study this region in non-demented Parkinson's disease patients with and without major depression. (author)

Cardiac memory in patients with Wolff-Parkinson-White syndrome: noninvasive imaging of activation and repolarization before and after catheter ablation.

Science.gov (United States)

Ghosh, Subham; Rhee, Edward K; Avari, Jennifer N; Woodard, Pamela K; Rudy, Yoram

2008-08-26

Cardiac memory refers to a change in ventricular repolarization induced by and persisting for minutes to months after cessation of a period of altered ventricular activation (eg, resulting from pacing or preexcitation in patients with Wolff-Parkinson-White syndrome). ECG imaging (ECGI) is a novel imaging modality for noninvasive electroanatomic mapping of epicardial activation and repolarization. Fourteen pediatric patients with Wolff-Parkinson-White syndrome and no other congenital disease, were imaged with ECGI a day before and 45 minutes, 1 week, and 1 month after successful catheter ablation. ECGI determined that preexcitation sites were consistent with sites of successful ablation in all cases to within a 1-hour arc of each atrioventricular annulus. In the preexcited rhythm, activation-recovery interval (ARI) was the longest (349+/-6 ms) in the area of preexcitation leading to high average base-to-apex ARI dispersion of 95+/-9 ms (normal is approximately 40 ms). The ARI dispersion remained the same 45 minutes after ablation, although the activation sequence was restored to normal. ARI dispersion was still high (79+/-9 ms) 1 week later and returned to normal (45+/-6 ms) 1 month after ablation. The study demonstrates that ECGI can noninvasively localize ventricular insertion sites of accessory pathways to guide ablation and evaluate its outcome in pediatric patients with Wolff-Parkinson-White syndrome. Wolff-Parkinson-White is associated with high ARI dispersion in the preexcited rhythm that persists after ablation and gradually returns to normal over a period of 1 month, demonstrating the presence of cardiac memory. The 1-month time course is consistent with transcriptional reprogramming and remodeling of ion channels.

Embodying Emotional Disorders: New Hypotheses about Possible Emotional Consequences of Motor Disorders in Parkinson's Disease and Tourette's Syndrome

OpenAIRE

Mermillod, Martial; Vermeulen, Nicolas; Droit-Volet, Sylvie; Jalenques, Isabelle; Durif, Franck; Niedenthal, Paula

2011-01-01

Parkinson's disease (PD) and Tourette's syndrome (TS) lead to important motor disorders among patients such as possible facial amimia in PD and tics in Tourette's syndrome. Under the grounded cognition framework that shows the importance of motor embodiment in emotional feeling (Niedenthal, 2007), both types of pathology with motor symptoms should be sufficient to induce potential impairments for these patients when recognizing emotional facial expressions (EFE). In this opinion paper, we des...

Addenbrooke's Cognitive Examination validation in Parkinson's disease.

Science.gov (United States)

Reyes, M A; Perez-Lloret, S; Lloret, S P; Roldan Gerschcovich, E; Gerscovich, E R; Martin, M E; Leiguarda, R; Merello, M

2009-01-01

There is a clear need for brief, sensitive and specific cognitive screening instruments in Parkinson's disease (PD). To study Addenbrooke's Cognitive Examination (ACE) validity for cognitive assessment of PD patient's using the Mattis Dementia Rating Scale (MDRS) as reference method. A specific scale for cognitive evaluation in PD, in this instance the Scales for Outcomes of Parkinson's disease-Cognition (SCOPA-COG), as well as a general use scale the Mini-mental state examination (MMSE) were also studied for further correlation. Forty-four PD patients were studied, of these 27 were males (61%), with a mean (SD) age of 69.5 (11.8) years, mean (SD) disease duration of 7.6 (6.4) years (range 1-25), mean (SD) total Unified Parkinson's Disease Rating Scale (UPDRS) score 37 (24) points, UPDRS III 16.5 (11.3) points. MDRS, ACE and SCOPA-COG scales were administered in random order. All patients remained in on-state during the study. Addenbrooke's Cognitive Examination correlated with SCOPA-COG (r = 0.93, P Addenbrooke's Cognitive Examination appears to be a valid tool for dementia evaluation in PD, with a cut-off point which should probably be set at 83 points, displaying good correlation with both the scale specifically designed for cognitive deficits in PD namely SCOPA-COG, as well as with less specific tests such as MMSE.

Wolff-Parkinson-White Syndrome and Rheumatic Mitral Stenosis: an Uncommon Coincidence that can Cause Severe Hemodynamic Disturbance

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Ahmet Taha Alper

2008-11-01

Full Text Available The combination of rheumatic mitral stenosis and Wolff-Parkinson-White syndrome is a rare situation. In this case, we are reporting an 72-year-old man presenting with multi-organ failure due to the this combination and successfully treated with radiofrequency ablation during preexcitated atrial fibrillation.

Wolff-Parkinson-White Syndrome and Rheumatic Mitral Stenosis: an Uncommon Coincidence that can Cause Severe Hemodynamic Disturbance

Science.gov (United States)

Alper, Ahmet Taha; Hasdemir, Hakan; Akyol, Ahmet

2008-01-01

The combination of rheumatic mitral stenosis and Wolff-Parkinson-White syndrome is a rare situation. In this case, we are reporting an 72-year-old man presenting with multi-organ failure due to the this combination and successfully treated with radiofrequency ablation during preexcitated atrial fibrillation. PMID:18982140

Cognitive decline in Parkinson disease

Science.gov (United States)

Aarsland, Dag; Creese, Byron; Politis, Marios; Chaudhuri, K. Ray; ffytche, Dominic H.; Weintraub, Daniel; Ballard, Clive

2017-01-01

Dementia is a frequent problem encountered in advanced stages of Parkinson disease (PD). In recent years, research has focused on the pre-dementia stages of cognitive impairment in PD, including mild cognitive impairment (MCI). Several longitudinal studies have shown that MCI is a harbinger of dementia in PD, although the course is variable, and stabilization of cognition — or even reversal to normal cognition — is not uncommon. In addition to limbic and cortical spread of Lewy pathology, several other mechanisms are likely to contribute to cognitive decline in PD, and a variety of biomarker studies, some using novel structural and functional imaging techniques, have documented in vivo brain changes associated with cognitive impairment. The evidence consistently suggests that low cerebrospinal fluid levels of amyloid-β42, a marker of comorbid Alzheimer disease (AD), predict future cognitive decline and dementia in PD. Emerging genetic evidence indicates that in addition to the APOE*ε4 allele (an established risk factor for AD), GBA mutations and SCNA mutations and triplications are associated with cognitive decline in PD, whereas the findings are mixed for MAPT polymorphisms. Cognitive enhancing medications have some effect in PD dementia, but no convincing evidence that progression from MCI to dementia can be delayed or prevented is available, although cognitive training has shown promising results. PMID:28257128

Asymptomatic Wolff-Parkinson-White Syndrome: Incidental EKG

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Samer Assaf

2017-07-01

Full Text Available History of present illness: A 38-year-old male presents to the emergency department for a minor trauma evaluation after falling off a bicycle at a moderate rate of speed. The patient was not wearing a helmet when he hit his head with unknown loss of consciousness. Focused assessment with sonography for trauma (FAST exam and head computed tomography (CT were negative. Routine electrocardiogram (ECG showed sinus rhythm with pre-excitation indicative of Wolff-Parkinson-White Syndrome (WPW. The patient confirmed a previous diagnosis of WPW, but had not previously followed up with a cardiologist. Significant findings: The ECG shows slurred up-stroking of the QRS complexes characteristic of a delta wave. The PR interval is normal; however, the QT interval is greater than 110ms. Discussion: Wolff-Parkinson-White Syndrome (WPW is a frequently encountered macro-reentrant arrhythmia characterized by a shortened PR interval less than 120ms, prolonged QRS greater than 120ms with an up-stroking QRS complex (delta wave, and occasional ST abnormalities.1 The incidence is reported to be 0.9%-3% of the general population and most diagnoses are made on routine EKGs.2,3 WPW is thought to be caused by abnormalities of conduction through the accessory pathway, also known as the Bundle of Kent, causing premature excitation of the ventricles. The complications from WPW are supraventricular tachycardia, atrial arrhythmias, and ventricular fibrillation leading to sudden cardiac death.3 Approximately 40-50% of patients who die from sudden cardiac arrest associated with WPW were previously asymptomatic.4 Unfortunately, it is agreed that approximately 50% of patients with WPW are asymptomatic and unaware of their diagnosis.5 The definitive treatment for WPW is radiofrequency catheter ablation (RFCA. However, it comes with a low risk of complications including arrhythmias and death.6 For asymptomatic WPW, children are at the highest risk for ventricular arrhythmias while

Duplication of 20p12.3 associated with familial Wolff-Parkinson-White syndrome.

Science.gov (United States)

Mills, Kimberly I; Anderson, Jacqueline; Levy, Philip T; Cole, F Sessions; Silva, Jennifer N A; Kulkarni, Shashikant; Shinawi, Marwan

2013-01-01

Wolff-Parkinson-White (WPW) syndrome is caused by preexcitation of the ventricular myocardium via an accessory pathway which increases the risk for paroxysmal supraventricular tachycardia. The condition is often sporadic and of unknown etiology in the majority of cases. Autosomal dominant inheritance and association with congenital heart defects or ventricular hypertrophy were described. Microdeletions of 20p12.3 have been associated with WPW syndrome with either cognitive dysfunction or Alagille syndrome. Here, we describe the association of 20p12.3 duplication with WPW syndrome in a patient who presented with non-immune hydrops. Her paternal uncle carries the duplication and has attention-deficit hyperactivity disorder and electrocardiographic findings consistent with WPW. The 769 kb duplication was detected by the Affymetrix Whole Genome-Human SNP Array 6.0 and encompasses two genes and the first two exons of a third gene. We discuss the potential role of the genes in the duplicated region in the pathogenesis of WPW and possible neurobehavioral abnormalities. Our data provide additional support for a significant role of 20p12.3 chromosomal rearrangements in the etiology of WPW syndrome. Copyright © 2012 Wiley Periodicals, Inc.

A case with Parkinsonism secondary to bilateral subdural hematoma

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Adalet Arıkanoğlu

2011-03-01

Full Text Available Subdural hematoma is a rare cause of secondary Parkinsonism. In this study, we presented a case of Parkinsonian syndrome caused by a bilateral subdural hematoma. The patient’s Parkinsonism completely healed following successful surgical removal of the hematomas without any anti-parkinson drug.

Accuracy of algorithms to predict accessory pathway location in children with Wolff-Parkinson-White syndrome.

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Wren, Christopher; Vogel, Melanie; Lord, Stephen; Abrams, Dominic; Bourke, John; Rees, Philip; Rosenthal, Eric

2012-02-01

The aim of this study was to examine the accuracy in predicting pathway location in children with Wolff-Parkinson-White syndrome for each of seven published algorithms. ECGs from 100 consecutive children with Wolff-Parkinson-White syndrome undergoing electrophysiological study were analysed by six investigators using seven published algorithms, six of which had been developed in adult patients. Accuracy and concordance of predictions were adjusted for the number of pathway locations. Accessory pathways were left-sided in 49, septal in 20 and right-sided in 31 children. Overall accuracy of prediction was 30-49% for the exact location and 61-68% including adjacent locations. Concordance between investigators varied between 41% and 86%. No algorithm was better at predicting septal pathways (accuracy 5-35%, improving to 40-78% including adjacent locations), but one was significantly worse. Predictive accuracy was 24-53% for the exact location of right-sided pathways (50-71% including adjacent locations) and 32-55% for the exact location of left-sided pathways (58-73% including adjacent locations). All algorithms were less accurate in our hands than in other authors' own assessment. None performed well in identifying midseptal or right anteroseptal accessory pathway locations.

Abnormally phosphorylated tau protein in senile dementia of Lewy body type and Alzheimer disease: evidence that the disorders are distinct.

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Strong, C; Anderton, B H; Perry, R H; Perry, E K; Ince, P G; Lovestone, S

1995-01-01

The relationship between Alzheimer disease (AD) and dementia with Lewy bodies (senile dementia Lewy body type, or SDLT) and dementia in Parkinson's disease is unclear. AD pathology is characterised by both amyloid deposition and abnormal phosphorylation of tau in paired helical filaments (PHF-tau). In AD, abnormally phosphorylated PHF-tau is present in neurofibrillary tangles, in neuritic processes of senile plaques, and also in neuropil threads dispersed throughout the cerebral cortex. Cortical homogenates from 12 cases each of AD and SDLT, 13 cases of Parkinson's disease, and 11 normal controls were examined by Western blot analysis with antibodies that detect PHF-tau. No PHF-tau was found in Parkinson's disease or control cortex. No PHF-tau was found in SDLT cases without histological evidence of tangles. PHF-tau was detectable in SDLT cases with a low density of tangles, and large amounts of PHF-tau were present in AD cases. This study demonstrates that abnormally phosphorylated PHF-tau is only present where tangles are found and not in SDLT cases without tangles or with only occasional tangles. It is concluded that Lewy body dementias are distinct at a molecular level from AD.

Phase analysis of regional and global ventricular contraction patterns in Wolff-Parkinson-White syndrome

International Nuclear Information System (INIS)

Konishi, Tokuji; Koyama, Takao; Ichikawa, Takehiko

1989-01-01

Multigated blood pool scintigraphy was performed in 20 normal subjects and 39 patients with various intraventricular conduction abnormalities, including 25 patients with Wolff-Parkinson-White (WPW) syndrome. Cardiac imaging was performed in the modified left anterior oblique, right anterior oblique, and left lateral projections. In WPW syndrome, early contraction sites which were not seen in normal subjects were detected at the ventricular base in phase images. These anomalous early contraction sites disappeared after successful suppression of conduction through an accessory pathway by intravenous procainamide. These sites are believed to correspond to the location of the bundle of Kent and were consistent with the electrocardiographic findings. Phase mapping is a suitable noninvasive method to locate the position of the bundle of Kent and evaluate the ventricular contraction pattern in WPW syndrome and other intraventricular conduction abnormalities. (author)

The development of the dementia concept in 19th century

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Leonardo Caixeta

2014-07-01

Full Text Available The dementia concept has been reformulated through its history and the 19th century was remarkable in the construction of this concept as we understand it today. Like other syndromes, much of the history of the dementia concept comes from the attempt to separate it from other nosological conditions, giving it a unique identity. The fundamental elements for the arising of the dementia modern concept were: a correlation of the observed syndrome with organic-cerebral lesions; b understanding of the irreversibility of the dementia evolution; c its relation with human ageing; and d the choice of the cognitive dysfunction as a clinical marker of the dementia concept.

Advanced MR Neuroimaging in Early Stage Presenile Dementia

NARCIS (Netherlands)

R.M.E. Steketee (Rebecca)

2016-01-01

markdownabstractDementia is a syndrome affecting cognitive functions and behavior, with an overwhelming impact on both patients and caregivers. An estimated number of 35.6 million patients suffers from dementia, with a subset affected before the age of 65 years, i.e. presenile dementia. Establishing

The use of sugammadex in a pregnant patient with Wolff-Parkinson-White syndrome.

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Sengul, Turker; Saracoglu, Ayten; Sener, Sibel; Bezen, Olgac

2016-09-01

Wolff-Parkinson-White (WPW) syndrome is a rare pre-excitation syndrome which develops when atrioventricular conduction occurs through a pathologic accessory pathway known as the bundle of Kent instead of atrioventricular node, hence resulting in tachycardia. Patients with WPW syndrome may experience various symptoms arising from mild-to-moderate chest disease, palpitations, hypotension, and severe cardiopulmonary dysfunction. These patients are most often symptomatic because of cardiac arrhythmias. In this case report, we present an uneventful anesthetic management of a pregnant patient with WPW syndrome undergoing cesarean delivery. A 23-year-old American Society of Anesthesiologists class 2 pregnant patient was diagnosed with WPW syndrome. Her preoperative 12-lead electrocardiogram showed a sinus rhythm at 82 beats per minute, a delta wave, and a short PR interval. After an uneventful surgery, sugammadex 2mg/kg was administered as a reversal agent instead of neostigmine. Then she was discharged to her obstetrics service. Serious hemodynamic disorders may occur in patients with WPW syndrome due to development of fatal arrhythmias. Neostigmine used as a reversal agent in general anesthesia can trigger such fatal arrhythmias by leading changes in cardiac conduction. We believe that sugammadex, which is safely used in many areas in the scope of clinical practice, can be also used for patients diagnosed with WPW syndrome. Copyright © 2016 Elsevier Inc. All rights reserved.

The use of echocardiography in Wolff-Parkinson-White syndrome.

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Cai, Qiangjun; Shuraih, Mossaab; Nagueh, Sherif F

2012-04-01

Endocardial mapping and radiofrequency catheter ablation are well established modalities for the diagnosis and treatment of patients with Wolff-Parkinson-White (WPW) syndrome associated with tachyarrhythmias. However, the electrophysiologic techniques are invasive, require radiation exposure, and lack spatial resolution of cardiac structures. A variety of echocardiographic techniques have been investigated as a non-invasive alternative for accessory pathway localization. Conventional M-mode echocardiography can detect the fine premature wall motion abnormalities associated with WPW syndrome. However, it is unable to identify the exact site of accessory pathway with sufficient accuracy. 2D, 2D-guided M-mode, and 2D phase analysis techniques are limited by image quality and endocardial border definition. Various modalities of tissue Doppler echocardiography significantly increase the accuracy of left-sided accessory pathway localization to 80-90% even in patients with poor acoustic window. However, right-sided pathways remain a diagnostic challenge. Strain echocardiography by speckle tracking has recently been evaluated and appears promising. Different cardiac abnormalities have been detected by echocardiography in WPW patients. Patients with WPW syndrome and tachyarrhythmias have impaired systolic and diastolic function which improves after radiofrequency ablation. Echocardiography is useful in identifying patient with accessory pathway-associated left ventricular dyssynchrony and dysfunction who may benefit from ablation therapy. Transesophageal and intracardiac echocardiography have been used to guide ablation procedure. Ablation-related complications detected by routine echocardiography are infrequent, rarely clinically relevant, and of limited value.

Clinical and imaging correlates of amyloid deposition in dementia with Lewy bodies.

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Donaghy, Paul C; Firbank, Michael J; Thomas, Alan J; Lloyd, Jim; Petrides, George; Barnett, Nicola; Olsen, Kirsty; O'Brien, John T

2018-04-19

Amyloid deposition is common in dementia with Lewy bodies, but its pathophysiological significance is unclear. The objective of this study was to investigate the relationship between amyloid deposition and clinical profile, gray matter volume, and brain perfusion in dementia with Lewy bodies. Dementia with Lewy bodies (n = 37), Alzheimer's disease (n = 20), and controls (n = 20) underwent a thorough clinical assessment, 3T MRI, and early- and late-phase 18 F-Florbetapir PET-CT to assess cortical perfusion and amyloid deposition, respectively. Amyloid scans were visually categorized as positive or negative. Image analysis was carried out using statistical parametric mapping (SPM) 8. There were no significant differences between amyloid-positive and amyloid-negative dementia with Lewy bodies cases in age (P = .78), overall cognitive impairment (P = .83), level of functional impairment (P = .80), or any other clinical or cognitive scale. There were also no significant differences in hippocampal or gray matter volumes. However, amyloid-positive dementia with Lewy bodies cases had lower medial temporal lobe perfusion (P = .03) than amyloid-negative cases, although a combination of medial temporal lobe perfusion, hippocampal volume, and cognitive measures was unable to accurately predict amyloid status in dementia with Lewy bodies. Amyloid deposition was not associated with differences in clinical or neuropsychological profiles in dementia with Lewy bodies, but was associated with imaging evidence of medial temporal lobe dysfunction. The presence of amyloid in dementia with Lewy bodies cannot be identified on the basis of clinical and other imaging features and will require direct assessment via PET imaging or CSF. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf

Conversion between mini-mental state examination, montreal cognitive assessment, and dementia rating scale-2 scores in Parkinson's disease.

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van Steenoven, Inger; Aarsland, Dag; Hurtig, Howard; Chen-Plotkin, Alice; Duda, John E; Rick, Jacqueline; Chahine, Lama M; Dahodwala, Nabila; Trojanowski, John Q; Roalf, David R; Moberg, Paul J; Weintraub, Daniel

2014-12-01

Cognitive impairment is one of the earliest, most common, and most disabling non-motor symptoms in Parkinson's disease (PD). Thus, routine screening of global cognitive abilities is important for the optimal management of PD patients. Few global cognitive screening instruments have been developed for or validated in PD patients. The Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), and Dementia Rating Scale-2 (DRS-2) have been used extensively for cognitive screening in both clinical and research settings. Determining how to convert the scores between instruments would facilitate the longitudinal assessment of cognition in clinical settings and the comparison and synthesis of cognitive data in multicenter and longitudinal cohort studies. The primary aim of this study was to apply a simple and reliable algorithm for the conversion of MoCA to MMSE scores in PD patients. A secondary aim was to apply this algorithm for the conversion of DRS-2 to both MMSE and MoCA scores. The cognitive performance of a convenience sample of 360 patients with idiopathic PD was assessed by at least two of these cognitive screening instruments. We then developed conversion scores between the MMSE, MoCA, and DRS-2 using equipercentile equating and log-linear smoothing. The conversion score tables reported here enable direct and easy comparison of three routinely used cognitive screening assessments in PD patients. © 2014 International Parkinson and Movement Disorder Society.

Language and Dementia: Neuropsychological Aspects.

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Kempler, Daniel; Goral, Mira

2008-01-01

This article reviews recent evidence for the relationship between extralinguistic cognitive and language abilities in dementia. A survey of data from investigations of three dementia syndromes (Alzheimer's disease, semantic dementia and progressive nonfluent aphasia) reveals that, more often than not, deterioration of conceptual organization appears associated with lexical impairments, whereas impairments in executive function are associated with sentence- and discourse-level deficits. These connections between extralinguistic functions and language ability also emerge from the literature on cognitive reserve and bilingualism that investigates factors that delay the onset and possibly the progression of neuropsychological manifestation of dementia.

Effect of Radiofrequency Catheter Ablation on Quality of Life in Patients with Wolff-Parkinson-White Syndrome

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Shokhrukh Erkaboev

2015-09-01

Full Text Available Wolff-Parkinson-White (WPW syndrome is one of several disorders of the conduction system of the heart that are commonly referred to as pre-excitation syndromes. As the syndrome significantly reduces the patients’ quality of life (QoL, the purpose of the current study was to compare QoL scores in patients with WPW syndrome before and after a radiofrequency catheter ablation (RFA procedure. To assess the patients’ QoL, the MOS 36-Item Short-Form Health Survey was used. Immediate and long-term outcomes of radiofrequency catheter ablation were analyzed in 60 patients diagnosed with WPW syndrome, 41(68.3% men and 19(31.7% women. As compared with the controls (28 apparently healthy persons, patients with WPW syndrome before RFA experienced significant reduction in both physical and mental health components. RFA was found effective in 93.3% of patients with WPW syndrome. At 3 months after RFA, patients showed significant improvement in both physical (13.5% and mental (17.2% health components; at 12 months, QoL parameters reached those of the controls.

Rasagiline in the pharmacotherapy of Parkinson's disease--a review.

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Rascol, Olivier

2005-10-01

Despite the current efficacious symptomatic approaches, the search is on for new therapies for Parkinson's disease that can control the cardinal symptoms of the disease (tremor, rigidity and bradykinesia), control/prevent motor complications induced by long-term levodopa, act on non-motor disease symptoms (dementia, dysautonomia, pain, insomnia, falls) and halt disease progression. Rasagiline is a monoamine oxidase-B inhibitor that has demonstrated efficacy against the cardinal symptoms of Parkinson's disease when used as monotherapy in early Parkinson's disease, and as an adjunct to levodopa in advanced disease stages. It reduces the duration and severity of poor symptom response episodes in fluctuating patients. Preliminary results allow discussion of putative effects of rasagiline on some non-motor signs and disease progression. This article outlines the evidence surrounding the efficacy and safety of rasagiline, and discusses its potential to address some of the currently unmet needs of Parkinson's disease therapy.

Noninvasive risk stratification for sudden death in asymptomatic patients with Wolff-Parkinson-White syndrome.

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Novella, John; DeBiasi, Ralph M; Coplan, Neil L; Suri, Ranji; Keller, Seth

2014-01-01

Sudden cardiac death (SCD) as the first clinical manifestation of Wolff-Parkinson-White (WPW) syndrome is a well-documented, although rare occurrence. The incidence of SCD in patients with WPW ranges from 0% to 0.39% annually. Controversy exists regarding risk stratification for patients with preexcitation on surface electrocardiogram (ECG), particularly in those who are asymptomatic. This article focuses on the role of risk stratification using exercise and pharmacologic testing in patients with WPW pattern on ECG.

Insecticide Exposure in Parkinsonism

National Research Council Canada - National Science Library

Bloomquist, Jeffrey

2002-01-01

Behavioral, neurochemical, and immunocytochemical studies characterized the possible role of insecticide exposure in the etiology of Parkinson's disease as it may relate to Gulf War Syndrome. Chlorpyrifos (CP) and permethrin (PM...

Insecticide Exposure in Parkinsonism

National Research Council Canada - National Science Library

Bloomquist, Jeffrey

2001-01-01

Behavioral, neurochemical, and immunocytochemical studies characterized the possible role of insecticide exposure in the etiology of Parkinson's disease as it may relate to Gulf War Syndrome. Chlorpyrifos (CP) and permethrin (PM...

Variability in the clinical expression of Parkinson's disease

NARCIS (Netherlands)

Wolters, E.C.

2008-01-01

Parkinsonism is a clinical syndrome characterized by bradykinesia, hypo-/akinesia, muscular rigidity, and resting tremor, mainly caused by Parkinson's disease (PD). Symptoms of PD are due to a progressive loss of nigral neurons causing striatal dopaminergic denervation. However, nigral degeneration

Síndrome de Wolff-Parkinson-White associada a comunicação interatrial tipo seio venoso Wolff-Parkinson-White syndrome and the sinus venosus atrial septal defect association

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Patrícia Lopes Moraes

2005-02-01

Full Text Available A associação de comunicação interatrial (CIA tipo seio venoso com síndrome de Wolff Parkinson White (WPW é muito rara e ainda não descrita na literatura médica especializada. Descreve-se o caso de uma jovem portadora dessa associação de patologias tratada com ablação da via acessória por radiofreqüência, seguida de correção cirúrgica do defeito do septo interatrial.The Wolff-Parkinson-White syndrome (WPW and sinus venosus atrial septal defect (ASD association is very rare and not yet reported in the literature. It is the main basis for this case report.

Characterization of Burning Mouth Syndrome in Patients with Parkinson's Disease.

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Bonenfant, David; Rompré, Pierre H; Rei, Nathalie; Jodoin, Nicolas; Soland, Valerie Lynn; Rey, Veronica; Brefel-Courbon, Christine; Ory-Magne, Fabienne; Rascol, Olivier; Blanchet, Pierre J

2016-01-01

To determine the prevalence and characteristics of burning mouth syndrome (BMS) in a Parkinson's disease (PD) population through a self-administered, custom-made survey. A total of 218 surveys were collected during regular outpatient visits at two Movement Disorders Clinics in Montreal (Canada) and Toulouse (France) to gather information about pain experience, PD-related symptoms, and oral and general health. A neurologist confirmed the diagnosis of PD, drug treatment, Hoehn-Yahr stage, and Schwab & England Activity of Daily Living score. Data between groups were compared using the independent samples Mann-Whitney U test and two-sided exact Fisher test. Data from 203 surveys were analyzed. BMS was reported by eight subjects (seven females and one male), resulting in a prevalence of 4.0% (95% confidence interval [CI] = 2.1-7.8). Five participants with chronic nonburning oral pain were excluded. PD severity and levodopa equivalent daily dose did not differ between non-BMS and BMS participants. Mean poor oral health index was higher in BMS compared to non-BMS subjects (49.0 vs 32.2 points, P syndrome. This survey yielded a low prevalence of BMS in PD patients, indicating no strong link between the two conditions. An augmenting effect such as that resulting from drug treatment in restless legs syndrome or sensory neuropathy cannot be excluded.

Alaska Native Parkinson’s Disease Registry

Science.gov (United States)

2008-11-01

Anti- Tremor drugs beta blockers systemic ocular primidone carbonic anhydrase inhibitors gabapentin Drugs causing...parkinsonism or tremor DA receptor blockers metoclopramide other Seroquel DA depletors reserpine tetrabenazine methyldopa beta ...Principal Investigator 4 A. Introduction Parkinsonism (PS) is a syndrome characterized by tremor , rigidity, slowness of movement, and problems

Depression associated with dementia.

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Gutzmann, H; Qazi, A

2015-06-01

Depression and cognitive disorders, including dementia and mild cognitive impairment, are common disorders in old age. Depression is frequent in dementia, causing distress, reducing the quality of life, exacerbating cognitive and functional impairment and increasing caregiver stress. Even mild levels of depression can significantly add to the functional impairment of dementia patients and the severity of psychopathological and neurological impairments increases with increasing severity of depression. Depressive symptoms may be both a risk factor for, as well as a prodrome of dementia. Major depressive syndrome of Alzheimer's disease may be among the most common mood disorders of older adults. Treating depression is therefore a key clinical priority to improve the quality of life both of people with dementia as well as their carergivers. Nonpharmacological approaches and watchful waiting should be attempted first in patients who present with mild to moderate depression and dementia. In cases of severe depression or depression not able to be managed through nonpharmacological means, antidepressant therapy should be considered.

[Metronome therapy in patients with Parkinson disease].

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Enzensberger, W; Oberländer, U; Stecker, K

1997-12-01

We studied 10 patients with Parkinson's disease and 12 patients with Parkinson-plus-syndrome, trying to improve patients' gait by application of various external rhythmic stimuli, including metronome stimulation (96 beats per minute = middle andante). The test course of the patients was 4 x 10 meters and 3 U-turns. The patients' gait quality under stimulation was compared with their free walk (velocity, number of steps, number of freezing episodes). Metronome stimulation significantly reduced the time and number of steps needed for the test course and also diminished the number of freezing episodes. March music stimulation was less effective and tactile stimulation (rhythmically tapping on the patient's shoulder) even produced negative results. The positive effect of metronome stimulation was also found, when the tests were not performed inside the hospital building, but outside in the hospital parc. Metronome stimulation was comparably effective in both patient sub-groups examined in this study (M. Parkinson, Parkinson-plus-syndrome) and seems to be an important additional help in the treatment of these patients. Electronical metronomes are not expensive, easy in handling, and portable. A theoretical explanation of metronome stimulation effectivity in patients with Parkinson's disease still needs to be elucidated.

Causes of Death According to Death Certificates in Individuals with Dementia: A Cohort from the Swedish Dementia Registry.

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Garcia-Ptacek, Sara; Kåreholt, Ingemar; Cermakova, Pavla; Rizzuto, Debora; Religa, Dorota; Eriksdotter, Maria

2016-11-01

The causes of death in dementia are not established, particularly in rarer dementias. The aim of this study is to calculate risk of death from specific causes for a broader spectrum of dementia diagnoses. Cohort study. Swedish Dementia Registry (SveDem), 2007-2012. Individuals with incident dementia registered in SveDem (N = 28,609); median follow-up 741 days. Observed deaths were 5,368 (19%). Information on number of deaths and causes of mortality was obtained from death certificates. Odds ratios for the presence of dementia on death certificates were calculated. Hazard ratios (HRs) and 95% confidence intervals (CIs) were calculated using Cox hazards regression for cause-specific mortality, using Alzheimer's dementia (AD) as reference. Hazard ratios for death for each specific cause of death were compared with hazard ratios of death from all causes (P-values from t-tests). The most frequent underlying cause of death in this cohort was cardiovascular (37%), followed by dementia (30%). Dementia and cardiovascular causes appeared as main or contributory causes on 63% of certificates, followed by respiratory (26%). Dementia was mentioned less in vascular dementia (VaD; 57%). Compared to AD, cardiovascular mortality was higher in individuals with VaD than in those with AD (HR = 1.82, 95% CI = 1.64-2.02). Respiratory death was higher in individuals with Lewy body dementia (LBD, including Parkinson's disease dementia and dementia with Lewy bodies, HR = 2.16, 95% CI = 1.71-2.71), and the risk of respiratory death was higher than expected from the risk for all-cause mortality. Participants with frontotemporal dementia were more likely to die from external causes of death than those with AD (HR = 2.86, 95% CI = 1.53-5.32). Dementia is underreported on death certificates as main and contributory causes. Individuals with LBD had a higher risk of respiratory death than those with AD. © 2016 The Authors. The Journal of the American Geriatrics Society published by Wiley

Acid rain may cause senile dementia

Energy Technology Data Exchange (ETDEWEB)

Pearce, F

1985-04-25

Aluminium, released from the soil by acid rain, may be a cause of several forms of senile dementia including Parkinson's disease and Alzheimer's disease. Many upland reservoirs, fed by acid rain, supply homes with water laced with significant amounts of aluminium. Studies in the Pacific have shown that communities living on soils that are extremely rich in bauxite, the rock containing aluminium, have a very high incidence of Alzheimer's disease.

Insecticide Exposure in Parkinsonism

National Research Council Canada - National Science Library

Bloomquist, Jeffrey

2003-01-01

Behavioral, neurochemical, and immunocytochemical studies are characterizing the possible role of insecticide exposure in the etiology of Parkinson's disease as it may relate to Gulf War Syndrome. Chlorpyrifos (CP) and/or permethrin (PM...

Dementia--epidemiological considerations, nomenclature, and a tacit consensus definition.

Science.gov (United States)

Breitner, John C S

2006-09-01

Epidemiologic inquiry requires the definition of a "case." Dementia may be defined clinically or alternatively by inference of irreversible brain pathology. Several iterations of the Diagnostic and Statistical Manual of Mental Disorders and International Classification of Diseases have skirted this issue by using criteria that are at once syndromic and neuropathological. The limitations of this compromise are revealed by large discrepancies in case identification when the various published criteria are strictly applied. Despite this problem, neuroepidemiologists have produced convergent estimates of the prevalence and incidence of dementia and its association with risk factors. This progress has reflected the tacit reliance of investigators on a simple definition of dementia as the syndrome of substantial global cognitive decline not attributable to alteration in level of consciousness. Beyond this description, our knowledge of pathology and, ultimately, the etiology of individual cases is extremely variable. Whatever its antecedents, syndromically defined dementia presents a looming public health crisis.

Advanced Parkinson's or "complex phase" Parkinson's disease? Re-evaluation is needed.

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Titova, Nataliya; Martinez-Martin, Pablo; Katunina, Elena; Chaudhuri, K Ray

2017-12-01

Holistic management of Parkinson's disease, now recognised as a combined motor and nonmotor disorder, remains a key unmet need. Such management needs relatively accurate definition of the various stages of Parkinson's from early untreated to late palliative as each stage calls for personalised therapies. Management also needs to have a robust knowledge of the progression pattern and clinical heterogeneity of the presentation of Parkinson's which may manifest in a motor dominant or nonmotor dominant manner. The "advanced" stages of Parkinson's disease qualify for advanced treatments such as with continuous infusion or stereotactic surgery yet the concept of "advanced Parkinson's disease" (APD) remains controversial in spite of growing knowledge of the natural history of the motor syndrome of PD. Advanced PD is currently largely defined on the basis of consensus opinion and thus with several caveats. Nonmotor aspects of PD may also reflect advancing course of the disorder, so far not reflected in usual scale based assessments which are largely focussed on motor symptoms. In this paper, we discuss the problems with current definitions of "advanced" PD and also propose the term "complex phase" Parkinson's disease as an alternative which takes into account a multimodal symptoms and biomarker based approach in addition to patient preference.

Computed tomography brain changes in Parkinsonian dementia

International Nuclear Information System (INIS)

Inzelberg, R.; Teeves, T.; Reider, I.; Gerlenter, I.; Korczyn, A.D.; Tel Aviv Univ.

1987-01-01

In order to evaluate the relationship between brain atrophy and the motor and cognitive function in Parkinson's disease, we have evaluated CT changes in 132 consecutive patients and compared them to measures of physical and mental decline, using intercorrelations and variance analysis. The result demonstrated age as a most important factor relating to brain atrophy. After correction for this determinant, it became clear that the motor and cognitive parameters were interdependent but they affected similar CT parameters. The effect of motor decline was the stronger of the two and it was the only one which correlated with cortical atrophy. The results support the notion of subcortical changes underlying the dementia of Parkinson's disease. (orig.)

Computed tomography brain changes in Parkinsonian dementia

Energy Technology Data Exchange (ETDEWEB)

Inzelberg, R; Teeves, T; Reider, I; Gerlenter, I; Korczyn, A D

1987-11-01

In order to evaluate the relationship between brain atrophy and the motor and cognitive function in Parkinson's disease, we have evaluated CT changes in 132 consecutive patients and compared them to measures of physical and mental decline, using intercorrelations and variance analysis. The result demonstrated age as a most important factor relating to brain atrophy. After correction for this determinant, it became clear that the motor and cognitive parameters were interdependent but they affected similar CT parameters. The effect of motor decline was the stronger of the two and it was the only one which correlated with cortical atrophy. The results support the notion of subcortical changes underlying the dementia of Parkinson's disease.

A Dementia Case Presenting with Psychotic Symptoms

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Osman Ozdemir

2013-06-01

Full Text Available Dementia is a progressive clinical syndrome in which affected areas of brain function may be affected, such as memory, language, abstract thinking, problem solving and attention. Psychotic symptoms include auditory and visual hallucinations and delusions, which usually occur in the dementia. In this paper, a dementia case presenting with psychotic symptoms is presented. [Cukurova Med J 2013; 38(3.000: 482-486

Exercise testing in children with Wolff-Parkinson-White syndrome: what is its value?

Science.gov (United States)

Dalili, M; Vahidshahi, K; Aarabi-Moghaddam, M Y; Rao, J Y; Brugada, P

2014-10-01

This study was conducted to evaluate the accuracy of exercise testing for predicting accessory pathway characteristics in children with Wolff-Parkinson-White (WPW) syndrome. The study enrolled 37 children with WPW syndrome and candidates for invasive electrophysiologic study (EPS). Exercise testing was performed for all the study participants before the invasive study. Data from the invasive EPS were compared with findings from the exercise testing. The sudden disappearance of the delta (Δ) wave was seen in 10 cases (27 %). No significant correlation was found between the Δ wave disappearance and the antegrade effective refractory period of the accessory pathway (AERP-AP) or the shortest pre-excited RR interval (SPERRI). The sensitivity, specificity, and positive and negative predictive values of Δ wave disappearance, based on AERP-AP as gold standard, were respectively 29.4, 80, 71.4, and 40 %. The corresponding values with SPERRI as the gold standard were respectively 23.8, 71.4, 71.4 and 23.8 %. Exercise testing has a medium to low rate of accuracy in detecting low-risk WPW syndrome patients in the pediatric age group.

Assessing the dysexecutive syndrome in dementia.

Science.gov (United States)

Gansler, David A; Huey, Edward D; Pan, Jessica J; Wasserman, Eric; Grafman, Jordan H

2017-03-01

We compared performance on tests of dysexecutive behaviour (DB) and executive function (EF) in patients with behavioural variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA) and corticobasal syndrome (CBS). Patients diagnosed with bvFTD (n=124), PPA (n=34) and CBS (n=85) were recruited. EF was measured with the Delis-Kaplan Executive Function System (DKEFS: performance based), and DB was measured with the Frontal Systems Behavior Scale (FrSBe: caregiver-report based). Confirmatory factor analysis characterised the relationship between EF and DB, binary logistic regression evaluated the incremental diagnostic utility of the measures and neuroimaging data from 110 patients identified neural correlates. EF was lowest and DB was highest in bvFTD participants. EF and DB were distinct but related (r=-0.48). Measures correctly classified 89% of bvFTD from CBS patients and 93% of bvFTD from PPA patients-30% and 13% above base rates (59%, 80%), respectively. All modalities were useful in identifying CBS and PPA, whereas DB alone was useful for identifying bvFTD. EF was uniquely associated with caudal left dorsolateral prefrontal and lateral temporo-parietal cortices. DB was uniquely associated with the cingulate (R>L), right subcallosal and right anterior frontal cortex. EF and DB were associated with the rostral dorsolateral prefrontal cortex bilaterally. EF and DB measures displayed criterion and construct validity, had incremental utility at low DB levels (CBS and PPA) and were associated with overlapping and distinct neural correlates. EF and DB procedures can conjointly provide useful diagnostic and descriptive information in identifying and ruling out the dysexecutive syndrome. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Spontaneous Transition of Double Tachycardias with Atrial Fusion in a Patient with Wolff-Parkinson-White Syndrome.

Science.gov (United States)

Kim, Dongmin; Lee, Myung-Yong

2016-07-01

Among patients with Wolff-Parkinson-White syndrome, atrioventricular reciprocating tachycardia (AVRT) and atrioventricular nodal reentrant tachycardia (AVNRT) can coexist in a single patient. Direct transition of both tachycardias is rare; however, it can occur after premature atrial or ventricular activity if the cycle lengths of the two tachycardias are similar. Furthermore, persistent atrial activation by an accessory pathway (AP) located outside of the AV node during ongoing AVNRT is also rare. This article describes a case of uncommon atrial activation by an AP during AVNRT and gradual transition of the two supraventricular tachycardias without any preceding atrial or ventricular activity in a patient with preexcitation syndrome.

Etiologies and risk factors for dementia

Directory of Open Access Journals (Sweden)

Sandeep Grover

2016-01-01

Full Text Available Dementia is understood as a clinical syndrome characterized by impairment in memory impairment along with cognitive deficits in other domains. Over the years, understanding about the causes of dementias has improved. Broadly, dementias can be classified as irreversible degenerative dementias and reversible dementias. Alzheimer′s disease is the prototype of degenerative dementias and is characterized by the accumulation of beta-amyloid protein (called as amyloid plaques outside the neurons and accumulation of tau protein (called tau tangles inside the neurons. Vascular dementias are characterized by cerebrovascular insults which lead to pathological brain changes that impair cognition. The pathological hallmark of Lewy body dementia is the presence of α-synuclein neuronal inclusions, also known as Lewy bodies, accompanied by neuronal loss. Frontotemporal dementias are characterized by abnormal deposits of the microtubule-associated protein tau, the trans-activation response TAR DNA-binding protein with molecular weight 43 kDa (TDP-43, and the fused in sarcoma protein. Reversible dementias are characterized by the primary illness and may not present with characteristic brain deposits as seen with many degenerative dementias.

Association of plasma homovanillic acid with behavioral symptoms in patients diagnosed with dementia: a preliminary report.

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Sweet, R A; Pollock, B G; Mulsant, B H; Rosen, J; Lo, K H; Yao, J K; Henteleff, R A; Mazumdar, S

1997-12-01

Neuroleptic treatment of psychotic symptoms or agitated behavior in elderly patients diagnosed with dementia is associated with reduced efficacy and increased rates of neuroleptic-induced parkinsonism in comparison to younger patients with schizophrenia. We report the first study to examine the relationship between an in vivo measure of dopaminergic function, plasma homovanillic acid (pHVA), and ratings of psychosis, agitation, and parkinsonism before and after neuroleptic treatment in dementia patients. Pretreatment pHVA was significantly correlated with parkinsonian rigidity, with a trend observed with agitation and hostility. Though mean pHVA did not change during perphenazine treatment, intraindividual change in pHVA at day 15 was correlated with improvement in hostility, with a similar trend for improvement in agitation. These preliminary findings are consistent with reports associating dopaminergic function with agitated, but not psychotic, symptoms in patients diagnosed with dementia, and with a reduced responsivity of dopaminergic systems to neuroleptic treatment in these patients.

Woman with x-linked recessive dystonia-parkinsonism: clue to the epidemiology of parkinsonism in Filipino women?

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Domingo, Aloysius; Lee, Lillian V; Brüggemann, Norbert; Freimann, Karen; Kaiser, Frank J; Jamora, Roland D G; Rosales, Raymond L; Klein, Christine; Westenberger, Ana

2014-09-01

Despite recessive inheritance, X-linked dystonia-parkinsonism (Lubag disease) has also been described in women presenting with a late-onset isolated parkinsonian syndrome. Interestingly, unlike in other populations, there is a slight female predominance in the prevalence of parkinsonism in the Philippines. In a Filipino woman with suspected Parkinson disease, we confirmed the presence of all changes specific for X-linked dystonia-parkinsonism in genomic DNA. Subsequently, we analyzed complementary DNA and evaluated the methylation status of the androgen receptor gene. Owing to extremely skewed (98%:2%) X-chromosome inactivation, the patient expressed almost solely the mutated allele in a disease-specific change, rendering her molecularly comparable with a hemizygously affected man. Skewed X-chromosome inactivation is the likely cause of parkinsonism in this heterozygous mutation carrier. Because women carriers of the genetic changes specific for X-linked dystonia-parkinsonism are common in the Philippines, the epigenetic factor of nonrandom X-chromosome inactivation may contribute to the skewing of the sex prevalence of parkinsonism toward women in this country, warranting further investigation.

Primary caregivers' awareness and perception of early-onset dementia conditions in adolescents and young and middle-aged adults with Down syndrome.

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Lin, Jin-Ding; Chen, Wen-Xiu; Hsu, Shang-Wei; Lin, Lan-Ping; Lin, Fu-Gong; Tang, Chi-Chieh; Wu, Jia-Ling; Chu, Cordia; Chou, Yu-Ching

2014-09-01

The present study aims to investigate the onset of dementia conditions using the Dementia Screening Questionnaire for Individuals with Intellectual Disabilities (DSQIID) scale and to identify the possible factors associated with DSQIID scores in people with Down syndrome (DS). The study population was recruited from the voluntary registry members of the Republic of China Foundation for Persons with Down syndrome; primary caregivers provided DSQIID information on 196 adolescents and adults with DS (aged 15-48 years) who were entered into the database and analyzed using SPSS 20.0 software. The results described the distribution of early-onset dementia conditions in 53 adolescents and adults with DS, and 2.6% of the subjects with DS had possible dementia (DSQIID score ≧ 20). Univariate analyses found that older age (p=0.001) and comorbid conditions (p=0.003) were significantly associated with DSQIID scores. Older subjects were more likely to have higher DSQIID scores than were younger age groups after ANOVA and Scheffe's tests. Lastly, a multiple linear regression analysis revealed that age (p<0.01), severe disability level (p<0.05) and comorbid condition (p<0.01) significantly explained 13% of the variation in DSQIID scores after adjusting for the factors of gender, education level and multiple disabilities in adolescents and adults with DS. The study highlights that future research should focus on the occurrence of dementia in people with DS and on identifying its influencing factors based on sound measurements, to initiate appropriate healthy aging policies for this group of people. Copyright © 2014 Elsevier Ltd. All rights reserved.

Electrophysiological evaluation of Wolff-Parkinson-White Syndrome

Science.gov (United States)

Brembilla-Perrot, Beatrice

2002-01-01

Sudden death might complicate the follow-up of symptomatic patients with the Wolff-Parkinson-White syndrome (WPW) and might be the first event in patients with asymptomatic WPW. The risk of sudden death is increased in some clinical situations. Generally, the noninvasive studies are unable to predict the risk of sudden death correctly . The electrophysiological study is the best means to detect the risk of sudden death and to evaluate the nature of symptoms. Methods used to define the prognosis of WPW are well-defined. At first the maximal rate of conduction through the accessory pathway is evaluated; programmed atrial stimulation using 1 and 2 extrastimuli delivered at different cycle lengths is then used to determine the accessory pathway refractory period and to induce a supraventricular tachycardia. These methods should be performed in the control state and repeated in adrenergic situations either during exercise test or more simply during a perfusion of small doses of isoproterenol. The induction of an atrial fibrillation with rapid conduction through the accessory pathway (> 240/min in control state, > 300/min after isoproterenol) is the sign of a form of WPW at risk of sudden death. PMID:16951730

Electrophysiological evaluation of Wolff-Parkinson-White Syndrome

Directory of Open Access Journals (Sweden)

Béatrice Brembilla-Perrot

2002-10-01

Full Text Available Sudden death might complicate the follow-up of symptomatic patients with the Wolff-Parkinson-White syndrome (WPW and might be the first event in patients with asymptomatic WPW. The risk of sudden death is increased in some clinical situations. Generally, the noninvasive studies are unable to predict the risk of sudden death correctly . The electrophysiological study is the best means to detect the risk of sudden death and to evaluate the nature of symptoms. Methods used to define the prognosis of WPW are well-defined. At first the maximal rate of conduction through the accessory pathway is evaluated; programmed atrial stimulation using 1 and 2 extrastimuli delivered at different cycle lengths is then used to determine the accessory pathway refractory period and to induce a supraventricular tachycardia. These methods should be performed in the control state and repeated in adrenergic situations either during exercise test or more simply during a perfusion of small doses of isoproterenol. The induction of an atrial fibrillation with rapid conduction through the accessory pathway (> 240/min in control state, > 300/min after isoproterenol is the sign of a form of WPW at risk of sudden death.

A Rare Case of Parkinson's Disease with Severe Neck Pain Owing to Crowned Dens Syndrome

OpenAIRE

Teruyuki Takahashi; Masato Tamura; Keiichi Osabe; Takashi Tamiya; Kenji Miki; Mai Yamaguchi; Kanno Akira; Satoshi Kamei; Toshiaki Takasu

2014-01-01

Background: Pain is regarded as one of the most common nonmotor symptoms in Parkinson's disease (PD). In particular, musculoskeletal pain has been reported as the most common type of PD-associated pain. Crowned dens syndrome (CDS), related to microcrystalline deposition in the periodontoid process, is the main cause of acute or chronic cervical pain. Case Presentation: This report describes the case of an 87-year-old woman who had severe bradykinesia, muscle rigidity, gait disturbance and nec...

Usefulness of severe cardiac sympathetic dysfunction to predict the occurrence of rapid atrial fibrillation in patients with Wolff-Parkinson-White syndrome.

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Akutsu, Yasushi; Kaneko, Kyouichi; Kodama, Yusuke; Li, Hui-Ling; Asano, Taku; Suyama, Jumpei; Tanno, Kaoru; Namiki, Atsuo; Shinozuka, Akira; Gokan, Takehiko; Kobayashi, Youichi

2013-09-01

Atrial fibrillation (AF) can be a potentially life-threatening arrhythmia when it conducts rapidly through the accessory pathway, which was not predicted by the noninvasive method. We evaluated the cardiac sympathetic activity for predicting the occurrence of AF in patients with Wolff-Parkinson-White (WPW) syndrome. Iodine-123 metaiodobenzylguanidine scintigraphy was performed under stable sinus rhythm conditions at rest syndrome than in the normal control group, and in the 15 patients with AF induced during EPS than in the 30 patients without AF (p syndrome. Copyright © 2013 Elsevier Inc. All rights reserved.

Factors associated with a depressive disorder in Alzheimer's disease are different from those found for other dementia disorders.

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Barca, Maria Lage; Engedal, Knut; Laks, Jerson; Selbaek, Geir

2012-01-01

This study explores factors associated with depression in Alzheimer's disease (AD) compared with mild cognitive impairment (MCI) and other dementia disorders. In a prospective study we included 195 patients: 31 with MCI, 112 with AD and 52 with other dementias. According to the ICD-10 and the DSM-IV criteria, 88 (44.1%) and 59 (30.3%), respectively, had a depressive disorder. An adjusted multiple regression analysis showed that previous depression (p depression in AD patients. Severity of dementia (p depressive disorder in a group of patients with frontotemporal dementia, vascular dementia, or dementia due to Lewy Body disease or Parkinson's disease. We found different factors associated with a depressive disorder in AD compared to those found for other dementia disorders.

Automatic extraction of the cingulum bundle in diffusion tensor tract-specific analysis. Feasibility study in Parkinson's disease with and without dementia

International Nuclear Information System (INIS)

Ito, Kenji; Masutani, Yoshitaka; Suzuki, Yuichi; Ino, Kenji; Kunimatsu, Akira; Ohtomo, Kuni; Kamagata, Koji; Yasmin, Hasina; Aoki, Shigeki

2013-01-01

Tract-specific analysis (TSA) measures diffusion parameters along a specific fiber that has been extracted by fiber tracking using manual regions of interest (ROIs), but TSA is limited by its requirement for manual operation, poor reproducibility, and high time consumption. We aimed to develop a fully automated extraction method for the cingulum bundle (CB) and to apply the method to TSA in neurobehavioral disorders such as Parkinson's disease (PD). We introduce the voxel classification (VC) and auto diffusion tensor fiber-tracking (AFT) methods of extraction. The VC method directly extracts the CB, skipping the fiber-tracking step, whereas the AFT method uses fiber tracking from automatically selected ROIs. We compared the results of VC and AFT to those obtained by manual diffusion tensor fiber tracking (MFT) performed by 3 operators. We quantified the Jaccard similarity index among the 3 methods in data from 20 subjects (10 normal controls [NC] and 10 patients with Parkinson's disease dementia [PDD]). We used all 3 extraction methods (VC, AFT, and MFT) to calculate the fractional anisotropy (FA) values of the anterior and posterior CB for 15 NC subjects, 15 with PD, and 15 with PDD. The Jaccard index between results of AFT and MFT, 0.72, was similar to the inter-operator Jaccard index of MFT. However, the Jaccard indices between VC and MFT and between VC and AFT were lower. Consequently, the VC method classified among 3 different groups (NC, PD, and PDD), whereas the others classified only 2 different groups (NC, PD or PDD). For TSA in Parkinson's disease, the VC method can be more useful than the AFT and MFT methods for extracting the CB. In addition, the results of patient data analysis suggest that a reduction of FA in the posterior CB may represent a useful biological index for monitoring PD and PDD. (author)

Cognitive Profiles of Patients with Mild Cognitive Impairment or Dementia in Alzheimer's or Parkinson's Disease

Directory of Open Access Journals (Sweden)

Helmut Hildebrandt

2013-04-01

Full Text Available Background: Alzheimer's disease (AD and Parkinson's disease (PD are associated with severe cognitive decline, but it is still unclear to what extent they become functionally more similar over time. Methods: We compared amnestic mild cognitively impaired (aMCI; n = 29 patients to mild cognitively impaired (MCI PD patients (n = 25, and patients with AD (n = 34 to patients with PD dementia (PDD; n = 15 with respect to cognitive functioning and mood. Results: aMCI patients were impaired in episodic memory, while MCI PD patients showed deficits in visuoconstruction and attention. AD and PDD patients showed comparable deficits on tests for language, attention and visuoconstruction. However, unlike PDD patients but similar to aMCI patients, AD patients showed a characteristic memory impairment, especially commission errors on recognition tasks, whereas PDD patients scored higher on the depressive mood questionnaire. Conclusions: In advanced stages of both diseases, the pattern of functional deficits associated with parietal and temporal lobe functions (attention, visuoconstruction and language is similar. However, specific differences, already present in the early stage (recognition errors in AD, associated with mediobasal temporal lobe functioning, and depressed mood in PDD, associated with non-motor basal ganglia loops, are also observed in the late stage.

The Everyday Cognition Scale in Parkinson's disease

OpenAIRE

Cooper, Rachel A.; Benge, Jared; Lantrip, Crystal; Soileau, Michael J.

2017-01-01

This brief report describes caregiver ratings on the Everyday Cognition (ECog) scale, a psychometrically robust measure of cognitively driven daily activities that was initially designed for other neurodegenerative conditions, in individuals with Parkinson's disease (PD). In 49 individuals with PD, those with suspected PD dementia had more difficulties across ECog domains than those with normal cognition or mild cognitive impairment. Results from multiple regression analyses revealed that act...

Validation of the MDS research criteria for prodromal Parkinson's disease: Longitudinal assessment in a REM sleep behavior disorder (RBD) cohort.

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Fereshtehnejad, Seyed-Mohammad; Montplaisir, Jacques Y; Pelletier, Amelie; Gagnon, Jean-François; Berg, Daniela; Postuma, Ronald B

2017-06-01

Recently, the International Parkinson and Movement Disorder Society introduced the prodromal criteria for PD. Objectives Our study aimed to examine diagnostic accuracy of the criteria as well as the independence of prodromal markers to predict conversion to PD or dementia with Lewy bodies. This prospective cohort study was performed on 121 individuals with rapid eye movement sleep behavior disorder who were followed annually for 1 to 12 years. Using data from a comprehensive panel of prodromal markers, likelihood ratio and post-test probability of the criteria were calculated at baseline and during each follow-up visit. Forty-eight (39.7%) individuals with rapid eye movement sleep behavior disorder converted to PD/dementia with Lewy bodies. The prodromal criteria had 81.3% sensitivity and 67.9% specificity for conversion to PD/dementia with Lewy bodies at 4-year follow-up. One year before conversion, sensitivity was 100%. The criteria predicted dementia with Lewy bodies with even higher accuracy than PD without dementia at onset. Those who met the threshold of prodromal criteria at baseline had significantly more rapid conversion into a neurodegenerative state (4.8 vs. 9.1 years; P conversion time in a rapid eye movement sleep behavior disorder cohort, with high sensitivity and high specificity with long follow-up. Prodromal markers influence the overall likelihood ratio independently, allowing them to be reliably multiplied. Defining additional markers with high likelihood ratio, further studies with longitudinal assessment and testing thresholds in different target populations will improve the criteria. © 2017 International Parkinson and Movement Disorder Society. © 2017 International Parkinson and Movement Disorder Society.

Early repolarization in Wolff-Parkinson-White syndrome: prevalence and clinical significance.

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Mizumaki, Koichi; Nishida, Kunihiro; Iwamoto, Jotaro; Nakatani, Yosuke; Yamaguchi, Yoshiaki; Sakamoto, Tamotsu; Tsuneda, Takayuki; Inoue, Hiroshi; Sakabe, Masao; Fujiki, Akira

2011-08-01

Idiopathic ventricular fibrillation (IVF) with early repolarization (ER) has recently been reported; however, ER is a common finding in healthy subjects and is also found sporadically in patients with Wolff-Parkinson-White (WPW) syndrome. The present study was designed to evaluate the prevalence and clinical significance of ER in patients with WPW syndrome. One hundred and eleven patients with WPW syndrome were studied retrospectively. Early repolarization was defined as QRS slurring or notching with J-point elevation ≥ 1 mm. The prevalence of ER was determined before and after successful catheter ablation. Before ablation, ER was found in 35 of 75 patients with a left free wall, 6 of 23 with a right free wall, and 7 of 13 with a septal accessory pathway (48 of 111, 43% as a whole). Early repolarization was always observed in leads with positive deflection of the initial part of the delta wave. After successful ablation of accessory pathways, ER was preserved in 28 (25%), disappeared in 20 (18%), and newly developed in 8 (7%) patients. In the remaining 55 (50%) patients, ER was not observed either before or after ablation. In patients with persistent ER, the amplitude and width of ER were significantly decreased 3-7 days after the ablation (1.7 ± 0.7 vs. 1.4 ± 0.6 mm, P syndrome, ER could be partly related to early depolarization through the accessory pathway. However, persistent ER and new ER appearing after the ablation were frequently found. Therefore, in these patients, mechanisms other than early depolarization may be involved in the genesis of ER.

Autobiographical memory in Parkinson's disease: a retrieval deficit.

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Souchay, Celine; Smith, Sarah Jane

2013-09-01

This study examined the effects of providing cues to facilitate autobiographical memory retrieval in Parkinson's disease. Previous findings have shown that individuals with Parkinson's disease retrieve fewer specific autobiographical memories than older adult controls. These findings are clinically significant since the quality of autobiographical memory is linked to identity and sense of self. In the current study, 16 older adults with Parkinson's disease without dementia and 16 matched older adult controls were given 3 min in which to recall autobiographical memories associated with five different time periods and to give each memory a short title. Participants were later asked to retrieve the memories in three phases: firstly in a free recall phase; secondly in response to general cues (time periods) and finally in response to specific cues (the short titles previously given). The number of memories and the quality of the memory (general or specific) was recorded in each condition. Compared with matched older adult controls, the Parkinson's disease group was impaired in retrieving the memories that they had previously given in the free recall phase and in response to general cues. The performance of the group with Parkinson's disease was only equivalent to the older adults when they retrieved memories in response to self-generated cues. The findings are discussed in relation to theories of autobiographical memory and the neuropsychology of Parkinson's disease. © 2013 The British Psychological Society.

Probable Association of Tachyarrhythmia With Nebulized Albuterol in a Child With Previously Subclinical Wolff Parkinson White Syndrome

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Kroesen, Michiel; Maseland, Machiel; Smal, Jaime; Reimer, Annet; van Setten, Petra

2012-01-01

We present the case of a 2-year-old asthmatic boy with atrioventricular (AV)-reentry tachycardia following albuterol inhalation, who was later diagnosed with Wolff-Parkinson-White (WPW) syndrome. The Naranjo adverse drug reaction probability scale score for this adverse event was 7, indicating that the association between his AV-reentry tachycardia and inhalation of albuterol is probable. To our knowledge, this is the first case report that shows the potential arrhythmogenic effects of albuterol in a child with WPW syndrome. We urge clinicians to be aware of this potentially life-threatening adverse effect and to closely monitor these patients when they need beta-adrenergic drugs in case of emergency. Furthermore, this report highlights the dilemma regarding the safe treatment of pediatric patients with both asthma and WPW syndrome. PMID:23118663

VGKC positive autoimmune encephalopathy mimicking dementia.

LENUS (Irish Health Repository)

Molloy, Anna

2011-01-01

Voltage gated potassium channel antibodies (VGKC Abs) are known to cause three rare neurological syndromes- neuromyotonia, Morvan\\'s syndrome and limbic encephalitis although an increasing array of other associated neurological symptoms are becoming recognised. The authors describe the case of a 60-year-old female who presented to the neurology clinic with an apparent early onset dementing process. She was noted to have both extrapyramidal and frontal release signs on examination and was admitted for further evaluation. Her dementia investigation including a neoplastic screen was negative except for VGKC antibody positivity. Her symptoms dramatically improved with commencement of immunosuppression. A non-paraneoplastic VGKC antibody associated dementia-like syndrome has rarely been described. The authors add to the few existing reports of what represents an important reversible cause of cognitive impairment.

VGKC positive autoimmune encephalopathy mimicking dementia.

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Molloy, Anna; Cassidy, Eugene; Ryan, Aisling; O' Toole, Orna

2011-12-01

Voltage gated potassium channel antibodies (VGKC Abs) are known to cause three rare neurological syndromes- neuromyotonia, Morvan's syndrome and limbic encephalitis although an increasing array of other associated neurological symptoms are becoming recognised. The authors describe the case of a 60-year-old female who presented to the neurology clinic with an apparent early onset dementing process. She was noted to have both extrapyramidal and frontal release signs on examination and was admitted for further evaluation. Her dementia investigation including a neoplastic screen was negative except for VGKC antibody positivity. Her symptoms dramatically improved with commencement of immunosuppression. A non-paraneoplastic VGKC antibody associated dementia-like syndrome has rarely been described. The authors add to the few existing reports of what represents an important reversible cause of cognitive impairment.

Accessory pathway location affects brain natriuretic peptide level in patients with Wolff-Parkinson-White syndrome.

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Nakatani, Yosuke; Kumagai, Koji; Naito, Shigeto; Nakamura, Kohki; Minami, Kentaro; Nakano, Masahiro; Sasaki, Takehito; Kinugawa, Koichiro; Oshima, Shigeru

2017-01-01

The purpose of this study was to investigate the relationship between the accessory pathway location and brain natriuretic peptide (BNP) level in patients with Wolff-Parkinson-White (WPW) syndrome. We divided 102 WPW syndrome patients with normal left ventricular systolic function into four groups: those with manifest right (MR, n = 14), manifest septal (MS, n = 11), manifest left (ML, n = 30), and concealed (C, n = 47) accessory pathways. BNP level and electrophysiological properties, including difference in timing of the ventricular electrogram between the His bundle area and the distal coronary sinus area (His-CS delay), which indicate intraventricular dyssynchrony, were compared. BNP levels (pg/dl) were higher in the MR and MS groups than in the ML and C groups (MR, 64 ± 58; MS, 55 ± 45; ML, 17 ± 15; C, 25 ± 21; P syndrome patients with normal cardiac function.

Dementia: role of MRI

International Nuclear Information System (INIS)

Georgieva-Kozarova, G.

2012-01-01

Full text: This presentation will focus on the role of MRI in the diagnosis of dementia and related diseases. We will discuss the following subjects: 1. Systematic assessment of MR in dementia 2. MR protocol for dementia 3. Typical findings in the most common dementia syndrome Alzheimer's disease (AD), Vascular Dementia (VaD), Frontotemporal lobe dementia (FTLD) 4. Short overview of neurodegenerative disorders which may be associated with dementia. The role of neuroimaging in dementia nowadays extends to support the diagnosis of specific neurodegenerative disorders. It is a challenge to the early diagnosis of neurodegenerative diseases such as Alzheimer's disease. Early diagnosis includes recognition of predementia conditions, such as mild cognitive impairment (MCI). Neuroimaging may also be used to assess disease progression and is adopted in current trials investigating MCI and AD. An MR-study of a patient suspected of having dementia must be assessed in a standardized way. First of all, treatable diseases like subdural hematomas, tumors and hydrocephalus need to be excluded. Next we should look for signs of specific dementias such as: Alzheimer's disease (AD): medial temporal lobe atrophy (MTA) and parietal atrophy. Frontotemporal Lobar Degeneration (FTLD): (asymmetric) frontal lobe atrophy and atrophy of the temporal pole. Vascular Dementia (VaD): global atrophy, diffuse white matter lesions, lacunas and 'strategic infarcts' (infarcts in regions that are involved in cognitive function). Dementia with Lewy bodies (DLB): in contrast to other forms of dementia usually no specific abnormalities. So when we study the MR images we should score in a systematic way for global atrophy, focal atrophy and for vascular disease (i.e. infarcts, white matter lesions, lacunas)

Endophenotypes of Dementia Associated with Traumatic Brain Injury in Retired Military Personnel

Science.gov (United States)

2014-10-01

depression , anxiety, and  Parkinsonism compared to veterans with cognitive impairment/ dementia  who have not  experienced a TBI, and 2) veterans with TBI...Award Number:  W81XWH‐12‐1‐0581  TITLE:   Endophenotypes of  Dementia  Associated with Traumatic Brain Injury in Retired Military  Personnel  PRINCIPAL...REPORT TYPE Annual 3. DATES COVERED 30 Sept 2013– 29 Sept 2014 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER Endophenotypes of  Dementia  Associated with

Anosognosia for cognitive and behavioral symptoms in Parkinson's disease with mild dementia and mild cognitive impairment: Frequency and neuropsychological/neuropsychiatric correlates.

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Orfei, Maria Donata; Assogna, Francesca; Pellicano, Clelia; Pontieri, Francesco Ernesto; Caltagirone, Carlo; Pierantozzi, Mariangela; Stefani, Alessandro; Spalletta, Gianfranco

2018-04-17

Anosognosia is a multidimensional phenomenon with detrimental effects on patients' illness course, therapy compliance and quality of life. We aimed at investigating anosognosia for cognitive and behavioral symptoms in Parkinson's Disease (PD) with dementia (PDD) and, for the first time, in PD with Mild Cognitive Impairment (MCI-PD). Community dwelling subjects (47 mild PDD, 136 multidomain MCI-PD (mdMCI-PD), 5 single domain MCI-PD (sdMCI-PD), and 197 PD without cognitive impairment (noCI-PD) were enrolled in a cross-sectional design study. All the subjects were administered the Anosognosia Questionnaire for Dementia, the Mental Deterioration Battery and a number of neuropsychiatric inventories. A diagnosis of anosognosia was made in 36% of patients with mild PDD and 16% with mdMCI-PD, whether it was negligible in sdMCI-PD and noCI-PD. Higher severity of anosognosia for cognitive impairment was also found in PDD and in mdMCI-PD. SdMCI-PD had the lower severity of anosognosia for cognitive impairment. Higher anosognosia for cognitive impairment was associated to lower depression in noCI-PD (r = -0.227, p = 0.0013) and mdMCI-PD (r = -0.266, p = 0.0016), and to reduced hedonic tone in noCI-PD (r = -0.191, p = 0.0071). Greater anosognosia was associated to lower executive performances in PDD (r = 0.424, p = 0.0074). Anosognosia for non-motor symptoms is frequent in PD patients with mild dementia or mdMCI. Results confirm the role of neuropsychiatric characteristics in anosognosia also in PD, the high prevalence of anosognosia in neurodegenerative illnesses and suggest a common pathogenic path for anosognosia in different neurodegenerative and psychiatric disorders. Copyright © 2018 Elsevier Ltd. All rights reserved.

The correlation of characters of cognitive impairment preceding Alzheimer s dementia in individuals with questionable dementia to Chinese medical syndrome%可疑痴呆人群中阿尔茨海默氏病临床前的认识损害特征及其与中医证候的关系

Institute of Scientific and Technical Information of China (English)

田金洲; 杨承芝; 盛彤; 刘桓; JudyHawortb; RomolaBucks; GordonWilcock

1999-01-01

To look for predictive markers preceding Alzheimer s dementia. Methods: This study involved multitudinal assessment of individuals in whom there was cognitive impairment that was insufficient to warrant a diagnosis of dementia, which were followed up for mean 11 months. Results: A diagnosis of probable AD was made in 58% of cases, VD in 21%, FLD in 5% and dementia of uncertain aetiology in the remainder. 42% subjects with memory loss are as result of ageassociated cognitive decline, 25% subjects are due to cerebralvascular accident, 1.8% subjects due to menopausal symptoms. 30.4% are of memory loss of unknown aetiology. Cognitive markers preceding dementia found within this study actually have discriminant function of the earliest changes of AD from age-associated cognitive decline. The group that developed dementia on follow-up does not differ significantly from the memory loss group on the WAIS-R total scores or on the digit forward, digit backward and picture completion. However, the subtest score for similarities on the WAIS-R is significantly lower in patients who became demented comparing with the rest of sample. Verbal memory such as story recall immediate and story recall delayed, verbal learning discriminant index, verbal fluency raw score showed significant lower in patients who became dementia. Moreover digit copy transformedscore on the SSRS also is very significantly lower in patients who became demented than those who were stable memory loss. The kidney deficiency syndrome of Chinese medicine was most common in QD, and the phlegm syndrome and the blood stasis syndrome were more common. The scores of the picture completion subtest and the story recall immediate subtest were significantly correlated to the score of the phlegm syndrome respectively. The scores of the picture completion subtest and the similarities subtest were significantly correlated with the score of the kidney deficiency syndrome respectively. Suggestion: Often a significantly long

Depression masquerading as chest pain in a patient with Wolff Parkinson White syndrome

Science.gov (United States)

Madabushi, Rajashree; Agarwal, Anil; Gautam, Sujeet K S; Khuba, Sandeep

2016-01-01

Wolff Parkinson White (WPW) syndrome is a condition in which there is an aberrant conduction pathway between the atria and ventricles, resulting in tachycardia. A 42-year-old patient, who was treated for WPW syndrome previously, presented with chronic somatic pain. With her cardiac condition in mind, she was thoroughly worked up for a recurrence of disease. As part of routine screening of all patients at our pain clinic, she was found to have severe depression as per the Patient Health Questionnaire–9 (PHQ–9) criteria. After ruling out sinister causes, she was treated for depression using oral Duloxetine and counselling. This led to resolution of symptoms, and improved her mood and functional capability. This case highlights the use of psychological screening tools and diligent examination in scenarios as confusing as the one presented here. Addressing the psychological aspects of pain and adopting a holistic approach are as important as treatment of the primary pathology. PMID:27738505

JPH3 Repeat Expansions Cause a Progressive Akinetic-Rigid Syndrome with Severe Dementia and Putaminal Rim in a Five-Generation African-American Family

Science.gov (United States)

Schneider, Susanne A.; Marshall, Kate E.; Xiao, Jianfeng; LeDoux, Mark S.

2012-01-01

We report the clinical, neuropsychological, genetic and radiological features of a large five-generation African-American kindred from the southern United States presenting with a progressive akinetic-rigid syndrome and severe dementia, but clinically insignificant chorea, due to mutations in JPH3. Overt disease onset was in the mid-twenties to late thirties with cognitive decline, REM sleep disturbance or psychiatric features, followed by development of a levodopa-unresponsive akinetic-rigid motor syndrome. Dystonia and myoclonus were present in some subjects. A bedridden, non-verbal severely akinetic-rigid state developed within 10 to 15 years after onset. CTG repeat expansions ranged from 47 to 53. Imaging revealed generalized cerebral atrophy with severe striatal involvement and putaminal rim hyperintensity. Analysis of our kindred indicates that JPH3 mutations should be considered in the differential diagnosis of early-onset dementia and hypokinetic-rigid syndromes in individuals of African descent. Moreover, chorea may not be overtly manifest at presentation or during significant parts of the disease course. PMID:22447335

CT scan of Parkinson's disease

Energy Technology Data Exchange (ETDEWEB)

Konishi, T; Noguchi, S; Nishitani, H [National Sanatorium of Utano, Kyoto (Japan); Kitano, H; Ikegami, Y

1981-04-01

In forty-eight patients with Parkinson's disease, we examined the ventricular size and the degree of cortical atrophy which were measured by the photos of CT scan and compared them with their clinical symptoms and side effects of anti-parkinsonian drugs. The ventricular size was expressed as the ventricular ratio which is the percentage of superimposed lateral ventricular area to the white and gray matter area at the slice number 2B of CT scan photos. The degree of the cortical atrophy was expressed as the sulcal numbers which were clearly visualized at the slice number 3B or 4A of CT scan photos. We used the CT scan photos of age-matched other patients which did not show definit central nervous system abnormalities. Our findings were as follows: (1) The ventricular enlargement was observed in the parkinsonian patients who showed dementia and/or Yahr's classification grades IV or V. (2) There was no correlation between the duration of this disease and the L--dopa treatments with the ventricular size and sulcal numbers. (3) The side effects of drugs such as visual hallucination were tended to be observed in the patients who showed the ventricular enlargement. (4) There was no definite correlation between the degree of cortical atrophy with clinical symptoms and side effects of various drugs. These findings suggested that the ventricular enlargement in Parkinson's disease was an important sign of dementia and the tendency of appearance of side effects of various drugs.

CT scan of Parkinson's disease

International Nuclear Information System (INIS)

Konishi, Tetsuro; Noguchi, Sadako; Nishitani, Hiroshi; Kitano, Haruo; Ikegami, Yoshinori.

1981-01-01

In forty-eight patients with Parkinson's disease, we examined the ventricular size and the degree of cortical atrophy which were measured by the photos of CT scan and compared them with their clinical symptoms and side effects of anti-parkinsonian drugs. The ventricular size was expressed as the ventricular ratio which is the percentage of superimposed lateral ventricular area to the white and gray matter area at the slice number 2B of CT scan photos. The degree of the cortical atrophy was expressed as the sulcal numbers which were clearly visualized at the slice number 3B or 4A of CT scan photos. We used the CT scan photos of age-matched other patients which did not show definit central nervous system abnormalities. Our findings were as follows: (1) The ventricular enlargement was observed in the parkinsonian patients who showed dementia and/or Yahr's classification grades IV or V. (2) There was no correlation between the duration of this disease and the L--dopa treatments with the ventricular size and sulcal numbers. (3) The side effects of drugs such as visual hallucination were tended to be observed in the patients who showed the ventricular enlargement. (4) There was no definite correlation between the degree of cortical atrophy with clinical symptoms and side effects of various drugs. These findings suggested that the ventricular enlargement in Parkinson's disease was an important sign of dementia and the tendency of appearance of side effects of various drugs. (author)

Clinical course and prognosis in patients with Gaucher disease and parkinsonism

Science.gov (United States)

Lopez, Grisel; Kim, Jenny; Wiggs, Edythe; Cintron, Dahima; Groden, Catherine; Tayebi, Nahid; Mistry, Pramod K.; Pastores, Gregory M.; Zimran, Ari; Goker-Alpan, Ozlem

2016-01-01

Objective: The goal of this study was to characterize the parkinsonian phenotype in patients with Gaucher disease (GD) who developed parkinsonism in order to evaluate clinical course and prognosis. Methods: This is a retrospective observational study conducted at the Clinical Center of the NIH, Bethesda, MD, over a period of 10 years. The study included 19 patients with GD and parkinsonism. The severity of Gaucher and parkinsonian symptoms was determined from clinical data including physical, neurologic, pathologic, and neurocognitive evaluations, family histories, imaging studies, olfactory testing, and validated questionnaires. Results: We found an earlier age at onset of parkinsonism and evidence of mild cognitive dysfunction in our cohort. Although the clinical course in some patients was similar to that of idiopathic Parkinson disease with a favorable levodopa response, others exhibited features characteristic of dementia with Lewy bodies. When we examined the patients as a group, we did not observe a uniformly aggressive form of parkinsonism after the initial onset of symptoms, contrary to other published reports. Conclusions: Appreciable clinical variation was seen in this cohort with GD and parkinsonism. Although some patients had early onset and prominent cognitive changes, others had a later, slower course, indicating that GBA1 mutations may not be a reliable prognostic indicator in Parkinson disease in clinical settings. PMID:27123476

Computertomographic studies of dementia

International Nuclear Information System (INIS)

Kohlmeyer, K.

1983-01-01

It seems to be very complicated even for the experienced neurologist and psychiatrist to correlate the clinical syndrome of dementia to different causing cerebral processes such as a primarily degenerative brain atrophy, a chronic cerebrovascular insufficiency, or other rarer occurring brain diseases unless neurological signs and symptoms do indicate a focal brain lesion. Since computed tomography is able to show both focal and general changes of the brain tissue each patient presenting with a dementia clinically should be undergone such a neuroradiological investigation at least once, and if being negative even repeatedly. Computed tomography is able not only to detect unexpected treatable brain lesions as a cause of dementia for instance tumors, subdural hematomas, and communicating hydrocephalus to expect in about 6% of cases with the clinical diagnosis of dementia, but also it is able to help to make the differentialdiagnosis of the dementia of Alzheimer's and the multi-infarct-type in a high percentage. Nevertheless despite the use of computed tomography the pathogenesis of dementia even though being undoubtful clinically remains obscure in 15% of our material of 367 demented patients studied by computed tomography but presenting with a normal finding. (orig.) [de

Influence of Different Cut-Off Values on the Diagnosis of Mild Cognitive Impairment in Parkinson's Disease

Directory of Open Access Journals (Sweden)

Inga Liepelt-Scarfone

2011-01-01

Full Text Available Comparable to Alzheimer's disease, mild cognitive impairment in Parkinson's disease (PD-MCI is associated with an increased risk for dementia. However different definitions of PD-MCI may have varying predictive accuracy for dementia. In a cohort of 101 nondemented Parkinson patients who underwent neuropsychological testing, the frequency of PD-MCI subjects and PD-MCI subtypes (i.e., amnestic/nonamnestic was determined by use of varying healthy population-based cut-off values. We also investigated the association between defined PD-MCI groups and ADL scales. Varying cut-off values for the definition of PD-MCI were found to affect frequency of PD-MCI subjects (9.9%–92.1% and, maybe more important, lead to a “shift” of proportion of detected PD-MCI subtypes especially within the amnestic single-domain subtype. Models using a strict cut-off value were significantly associated with lower ADL scores. Thus, the use of defined cut-off values for the definition of PD-MCI is highly relevant for comparison purposes. Strict cut-off values may have a higher predictive value for dementia.

White matter alterations in neurodegenerative and vascular dementia

International Nuclear Information System (INIS)

Supprian, T.; Kessler, H.; Falkai, P.; Retz, W.; Roesler, M.; Grunwald, I.; Reith, W.

2003-01-01

Due to a significant overlap of the two syndromes, differentiation of degenerative dementia of the Alzheimer-type from vascular dementia may be difficult even when imaging studies are available. White matter changes occur in many patients suffering from Alzheimer's disease. Little is known about the impact of white matter changes on the course and clinical presentation of Alzheimer's disease. High sensitivity of MRI in the detection of white matter alterations may account for over-diagnosing vascular dementia. The clinical significance of white matter alterations in dementia is still a matter of debate. The article reviews current concepts about the role of white matter alterations in dementia. (orig.) [de

Wolff-Parkinson-White Syndrome with Ventricular Hypertrophy in a Brazilian Family.

Science.gov (United States)

van der Steld, Lenises de Paula; Campuzano, Oscar; Pérez-Serra, Alexandra; Moura de Barros Zamorano, Mabel; Sousa Matos, Selma; Brugada, Ramon

2017-07-10

BACKGROUND PRKAG2 syndrome diagnosis is already well-defined as Wolff-Parkinson-White syndrome (WPW), ventricular hypertrophy (VH) due to glycogen accumulation, and conduction system disease (CSD). Because of its rarity, there is a lack of literature focused on the treatment. The present study aimed to describe appropriate strategies for the treatment of affected family members with PRKAG2 syndrome with a long follow-up period. CASE REPORT We studied 60 selected individuals from 84 family members (32 males, 53.3%) (mean age 27±16 years). Patients with WPW and/or VH were placed in a group of 18 individuals, in which 11 (61.1%) had VH and WPW, 6 (33.3%) had isolated WPW, and 1 (5.6%) had isolated VH. Palpitations occurred in 16 patients (88.9%), chest pain in 11 (61.1%), dizziness in 13 (72.2%), syncope in 15 (83.3%), and dyspnea in 13 (72%). Sudden cardiac death (SCD) occurred in 2 (11.1%), and 2 patients with cardiac arrest (CA) had asystole and pre-excited atrial flutter-fibrillation (AFL and AF) as the documented mechanism. Transient ischemic attack (TIA) and learning/language disabilities with delayed development were observed. Genetic analysis identified a new missense pathogenic variant (p.K290I) in the PRKAG2 gene. Cardiac histopathology demonstrated the predominance of vacuoles containing glycogen derivative and fibrosis. The treatment was based on hypertension and diabetes mellitus (DM) control, antiarrhythmic drugs (AD), anticoagulation, and radiofrequency catheter ablation (RCA). Six patients (33.3%) underwent pacemaker implantation (PM). CONCLUSIONS The present study describes the clinical treatment for a rare cardiac syndrome caused by a PRKAG2 mutation.

Neuropsychiatric Manifestations of Parkinson`s Disease

Directory of Open Access Journals (Sweden)

Ana Peixinho

2014-10-01

Full Text Available Parkinson’s disease affects about 1% of the world population older than 65 years. It’s most frequently considered a movement disorder, but the neuropsychiatric manifestations associated with the disease and/or its treatment may be of equal or greater significance in some patients. We will discuss briefly the epidemiology, physiopathology and diagnosis of Parkinson’s disease, highlighting the neuropsychiatric manifestations: depression, anxiety, psychosis, dementia, sleep disorders, dopamine dysregulation syndrome.

The utility of neuroimaging in the management of dementia

OpenAIRE

Uduak E Williams; Ekanem E Philip Ephraim; Sidney K Oparah

2015-01-01

Dementia is a syndrome of progressive dysfunction of two or more cognitive domains associated with impairment of activities of daily living. An understanding of the pathophysiology of dementia and its early diagnosis is important in the pursuit of possible disease modifying therapy for dementia. Neuroimaging has greatly transformed this field of research as its function has changed from a mere tool for diagnosing treatable causes of demen...

Depressive symptoms predict slow cognitive decline in mild dementia.

NARCIS (Netherlands)

Janzing, J.G.E.; Naarding, P.; Eling, P.A.T.M.

2005-01-01

Depression may be a prognostic marker of subsequent cognitive decline in patients with dementia. Earlier investigations did not find support for this hypothesis, but these considered mainly syndromal depression. In this prospective study, 32 subjects with mild dementia were followed up for 12

Parkinson disease and positron tomography

International Nuclear Information System (INIS)

Baron, J.C.

1984-10-01

Physiopathologic investigations of Parkinson disease and parkinsonian syndrome using positron tomography are briefly reviewed: study of cerebral blood flow and metabolism; effects of L-DOPA; study of dopaminergic receptors and of 18 F-Fluoro-L-DOPA incorporation [fr

Impact of Alzheimer's-Type Dementia and Information Source on the Assessment of Depression.

Science.gov (United States)

Gilley, David W.; And Others

1995-01-01

The level of agreement between the patient and a collateral source with regard to depressive symptomatology was compared for 185 patients with Alzheimer's-type dementia (AD), 57 patients with Parkinson's disease, and 54 nondemented geriatric referrals. Findings highlight the potential insensitivity of patient report in AD. (SLD)

IBZM- and CIT-SPECT of the dopaminergic system in Parkinsonism

International Nuclear Information System (INIS)

Tissingh, G.; Winogradzka, A.; Wolters, E.C.; Booij, J.; Royen, E.A. van

1997-01-01

Parkinsonism is most of the time caused by idiopathic Parkinson's disease (IPD). Considering the differences in therapeutic response and prognosis. in viva discrimination between IPD and 'Parkinsonism-plus' syndromes is important. Recently, ligands have become available for imaging the pre- and postsynaptic dopaminergic system by Single Photon Emission Computed Tomography (SPECT). Visualization of postsynaptic D 2 dopamine receptors using 123 I-iodobenzamide ( 123 I-IBZM) may contribute to the differential diagnosis between IPD and 'Parkinsonism-plus' syndromes as IPD is a pure presynaptic disease. Imaging of the presynaptic dopamine transporters using [ 123 I]β-CIT (2β-carbomethoxy-3β-(4-iodophenyl)tropane) may be used as a diagnostic technique. Early disease detection in subjects suspected to be at risk for developing IPD has become possible using [ 123 I]β-CIT or other ligands for the dopamine transporter. Furthermore, with SPECT one is probably able to monitor in an objective way the efficacy of new pharmacological therapies. (author)

Cerebral perfusion scintigraphy and the exploration of dementia syndromes: An illustration with five clinical cases; Scintigraphie cerebrale de perfusion et exploration des syndromes dementiels: illustration a l'aide de cinq cas cliniques

Energy Technology Data Exchange (ETDEWEB)

Farid, K.; Perdrisot, R. [Centre Hospitalier Universitaire de Poitiers, Service de Medecine Nucleaire, 86 - Poitiers (France); Habert, M.O. [Centre Hospitalier Universitaire de Pitie-Salpetriere, Service de Medecine Nucleaire, 75 - Paris (France)

2007-02-15

The epidemiological evidence suggests that individuals with higher education level have a reduced risk of developing dementia. Because cognitive reserve and its compensation mechanisms may modulate the clinical expression in neuro-degenerative pathology, it is important to study subjects who present mild cognitive disturbance with functional imaging. The cerebral SPECT has been used to determine regional uptake of radiotracer into the brain of patients with cognitive impairment. These abnormalities of blood flow were correlated with cognitive impairment. The cerebral SPECT is also useful to investigate preclinical dementia and to predict the evolution of cognitive disturbance. This article, reports some technical and semeiological notions and illustrate with five clinical cases the scintigraphic aspect of some dementia syndrome. (authors)

Utility of Exercise Testing and Adenosine Response for Risk Assessment in Children with Wolff-Parkinson-White Syndrome.

Science.gov (United States)

Ergul, Yakup; Ozturk, Erkut; Ozyilmaz, Isa; Unsal, Serkan; Carus, Hayat; Tola, Hasan Tahsin; Tanidir, Ibrahim Cansaran; Guzeltas, Alper

2015-01-01

We aimed to determine the correlation between noninvasive testing (exercise stress testing [EST] and adenosine responsiveness of accessory pathway [AP] ) and invasive electrophysiology study (EPS) for assessment antegrade conduction of the AP in Wolff-Parkinson-White syndrome. This prospective, observational study enrolled 40 children (58% male children, median age of 13 years, and median weight of 47.5 kg) with Wolff-Parkinson-White syndrome. Conduction through the AP to a cycle length of ≤250 ms was considered rapid or high-risk; otherwise, patients were nonrapid or low-risk. The sudden disappearance of the delta-wave was seen in 10 cases (25%) during EST. Accessory pathway was found to be high-risk in 13 cases (13/40, 32.5%) while the accessory path was identified as low-risk in 27 cases; however, six patients (15%) had blocked AP conduction with adenosine during EPS. Low-risk classification by EST alone to identify patients with nonrapid conduction in baseline EPS had a specificity of 93% and a positive predictive value of 90% (accuracy 54%). Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 93% and a positive predictive value of 84%. Finally, AP was adenosine nonresponsive in the majority of patients (28/30, 93%) with persistent delta-waves, 40% of those who had a sudden disappearance of delta-waves had an adenosine-responsive AP (P value: .028). Abrupt loss of preexcitation during EST and blocked AP conduction with adenosine had high specificity and positive predictive value for nonrapid and low-risk antegrade conduction during baseline invasive EPS. Successful risk stratification of pediatric patients with Wolff-Parkinson-White is possible through the use of EST and the adenosine responsiveness of AP. © 2015 Wiley Periodicals, Inc.

PET studies in dementia

International Nuclear Information System (INIS)

Herholz, K.

2003-01-01

Measurement of local cerebral glucose metabolism (lCMRGlc) by positron emission tomography (PET) and 18 F-2-fluoro-2-deoxy-D-glucose (FDG) has become a standard technique during the past 20 years and is now available at many university hospitals in all highly developed countries. Many studies have documented a close relation between lCMRGlc and localized cognitive functions, such as language and visuoconstructive abilities. Alzheimer's disease (AD) is characterized by regional impairment of cerebral glucose metabolism in neocortical association areas (posterior cingulate, temporoparietal and frontal multimodal association cortex), whereas primary visual and sensorimotor cortex, basal ganglia, and cerebellum are relatively well preserved. In a multicenter study comprising 10 PET centers (Network for Efficiency and Standardization of Dementia Diagnosis, NEST-DD) that employed an automated voxel-based analysis of FDG PET images, the distinction between controls and AD patients was 93% sensitive and 93% specific, and even in very mild dementia (at Mini Mental Status Examination (MMSE) 24 or higher) sensitivity was still 84% at 93% specificity. Significantly abnormal metabolism in mild cognitive deficit (MCI) indicates a high risk to develop dementia within the next two years. Reduced neocortical glucose metabolism can probably be detected with FDG PET in AD on average one year before onset of subjective cognitive impairment. In addition to glucose metabolism, specific tracers for dopamine synthesis ( 18 F-F-DOPA) and for ( 11 C-MP4A) are of interest for differentiation among dementia subtypes. Cortical acetylcholine esterase activity (AChE) activity is significantly lower in patients with AD or with dementia with Lewy bodies (DLB) than in age-matched normal controls. In LBD there is also impairment of dopamine synthesis, similar to Parkinson disease. (author) 115 refs

PET studies in dementia

Energy Technology Data Exchange (ETDEWEB)

Herholz, K. [Neurologische Universitaetsklinik and Max-Planck-Inst. fuer neurologische Forschung, Koeln (Germany)

2003-04-01

Measurement of local cerebral glucose metabolism (lCMRGlc) by positron emission tomography (PET) and {sup 18}F-2-fluoro-2-deoxy-D-glucose (FDG) has become a standard technique during the past 20 years and is now available at many university hospitals in all highly developed countries. Many studies have documented a close relation between lCMRGlc and localized cognitive functions, such as language and visuoconstructive abilities. Alzheimer's disease (AD) is characterized by regional impairment of cerebral glucose metabolism in neocortical association areas (posterior cingulate, temporoparietal and frontal multimodal association cortex), whereas primary visual and sensorimotor cortex, basal ganglia, and cerebellum are relatively well preserved. In a multicenter study comprising 10 PET centers (Network for Efficiency and Standardization of Dementia Diagnosis, NEST-DD) that employed an automated voxel-based analysis of FDG PET images, the distinction between controls and AD patients was 93% sensitive and 93% specific, and even in very mild dementia (at Mini Mental Status Examination (MMSE) 24 or higher) sensitivity was still 84% at 93% specificity. Significantly abnormal metabolism in mild cognitive deficit (MCI) indicates a high risk to develop dementia within the next two years. Reduced neocortical glucose metabolism can probably be detected with FDG PET in AD on average one year before onset of subjective cognitive impairment. In addition to glucose metabolism, specific tracers for dopamine synthesis ({sup 18}F-F-DOPA) and for ({sup 11}C-MP4A) are of interest for differentiation among dementia subtypes. Cortical acetylcholine esterase activity (AChE) activity is significantly lower in patients with AD or with dementia with Lewy bodies (DLB) than in age-matched normal controls. In LBD there is also impairment of dopamine synthesis, similar to Parkinson disease. (author) 115 refs.

CSF/serum albumin ratio in dementias: a cross-sectional study on 1861 patients.

Science.gov (United States)

Skillbäck, Tobias; Delsing, Louise; Synnergren, Jane; Mattsson, Niklas; Janelidze, Shorena; Nägga, Katarina; Kilander, Lena; Hicks, Ryan; Wimo, Anders; Winblad, Bengt; Hansson, Oskar; Blennow, Kaj; Eriksdotter, Maria; Zetterberg, Henrik

2017-11-01

A connection between dementias and blood-brain barrier (BBB) dysfunction has been suggested, but previous studies have yielded conflicting results. We examined cerebrospinal fluid (CSF)/serum albumin ratio in a large cohort of patients diagnosed with Alzheimer's disease (AD, early onset [EAD, n = 130], late onset AD [LAD, n = 666]), vascular dementia (VaD, n = 255), mixed AD and VaD (MIX, n = 362), Lewy body dementia (DLB, n = 50), frontotemporal dementia (FTD, n = 56), Parkinson's disease dementia (PDD, n = 23), other dementias (other, n = 48), and dementia not otherwise specified (NOS, n = 271). We compared CSF/serum albumin ratio to 2 healthy control groups (n = 292, n = 20), between dementia diagnoses, and tested biomarker associations. Patients in DLB, LAD, VaD, MIX, other, and NOS groups had higher CSF/serum albumin ratio than controls. CSF/serum albumin ratio correlated with CSF neurofilament light in LAD, MIX, VaD, and other groups but not with AD biomarkers. Our data show that BBB leakage is common in dementias. The lack of association between CSF/serum albumin ratio and AD biomarkers suggests that BBB dysfunction is not inherent to AD but might represent concomitant cerebrovascular pathology. Copyright © 2017 Elsevier Inc. All rights reserved.

Music-based therapeutic interventions for people with dementia

NARCIS (Netherlands)

Steen, J.T. van der; Soest-Poortvliet, M.C. van; Wouden, J.C. van der; Bruinsma, M.S.; Scholten, R.J.; Vink, A.C.

2017-01-01

BACKGROUND: Dementia is a clinical syndrome with a number of different causes which is characterised by deterioration in cognitive, behavioural, social and emotional functions. Pharmacological interventions are available but have limited effect to treat many of the syndrome's features. Less research

Medical image of the week: Wolff-Parkinson-White syndrome

Directory of Open Access Journals (Sweden)

Van Hook C

2017-04-01

Full Text Available No abstract available. Article truncated at 150 words. A 38-year-old man developed sustained rapid heart rate while rock climbing. The patient reported that he had experienced rare bouts of self-limited palpitations in the past. Blood pressure on arrival to the emergency department was 112/ 65 mm Hg. The patient’s initial EKG demonstrated a regular, narrow complex supraventricular tachycardia, with a rate of 232 (Figure 1. Intravenous adenosine was administered with no change in his rate or rhythm. The patient then received amiodarone by intravenous bolus, with subsequent conversion to sinus rhythm (Figure 2. Wolff-Parkinson-White (WPW syndrome is a congenital cardiac condition present in approximately 0.15% of the general population. WPW is characterized by the abnormal presence of conduction tissue that creates an accessory atrioventricular pathway and thus potentiates reentrant tachycardia (1. The classic resting EKG findings in WPW are: a shortened PR interval (less than 0.12 seconds, an indistinct initial upslope of the QRS complex (known as the …

REM Sleep Behavior Disorder in Parkinson's Disease and Other Synucleinopathies.

Science.gov (United States)

St Louis, Erik K; Boeve, Angelica R; Boeve, Bradley F

2017-05-01

Rapid eye movement sleep behavior disorder is characterized by dream enactment and complex motor behaviors during rapid eye movement sleep and rapid eye movement sleep atonia loss (rapid eye movement sleep without atonia) during polysomnography. Rapid eye movement sleep behavior disorder may be idiopathic or symptomatic and in both settings is highly associated with synucleinopathy neurodegeneration, especially Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure. Rapid eye movement sleep behavior disorder frequently manifests years to decades prior to overt motor, cognitive, or autonomic impairments as the presenting manifestation of synucleinopathy, along with other subtler prodromal "soft" signs of hyposmia, constipation, and orthostatic hypotension. Between 35% and 91.9% of patients initially diagnosed with idiopathic rapid eye movement sleep behavior disorder at a sleep center later develop a defined neurodegenerative disease. Less is known about the long-term prognosis of community-dwelling younger patients, especially women, and rapid eye movement sleep behavior disorder associated with antidepressant medications. Patients with rapid eye movement sleep behavior disorder are frequently prone to sleep-related injuries and should be treated to prevent injury with either melatonin 3-12 mg or clonazepam 0.5-2.0 mg to limit injury potential. Further evidence-based studies about rapid eye movement sleep behavior disorder are greatly needed, both to enable accurate prognostic prediction of end synucleinopathy phenotypes for individual patients and to support the application of symptomatic and neuroprotective therapies. Rapid eye movement sleep behavior disorder as a prodromal synucleinopathy represents a defined time point at which neuroprotective therapies could potentially be applied for the prevention of Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure. © 2017

An Intron 7 Polymorphism in APP Affects the Age of Onset of Dementia in Down Syndrome

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Emma L. Jones

2011-01-01

Full Text Available People with Down syndrome (DS develop Alzheimer's disease (AD with an early age of onset. A tetranucleotide repeat, attt5−8, in intron 7 of the amyloid precursor protein has been associated with the age of onset of AD in DS in a preliminary study. The authors examine the impact of this polymorphism in a larger cohort of individuals with DS. Adults with DS were genotyped for attt5−8 and APOE. The results were analysed with respect to the age of onset of dementia. The presence of three copies of the six-repeat allele resulted in onset of dementia seven years earlier than in the presence of other genotypes. Further study is essential to elucidate the mechanism by which this polymorphism functions, with an exciting opportunity to identify novel treatment targets relevant for people with DS and AD.

Thyroid storm in a patient with Wolff-Parkinson-White syndrome.

Science.gov (United States)

Naqvi, Syed Yaseen; Luebbert, Jeffrey J; Rosen, Stephen G

2015-12-15

A 44-year-old woman with no medical history presented to the emergency department with a 2 h history of sudden onset chest pressure, palpitations, diaphoresis and shortness of breath. She reported a 90-pound unintentional weight loss, increased appetite, irritability, night sweats and palpitations for 2 months. Physical examination revealed a heart rate (HR) of 269 bpm and a blood pressure of 116/94 mm Hg. Her ECG revealed a wide-complex tachycardia with right bundle branch morphology and an HR of 265 bpm. Intravenous adenosine was administered with resolution of the arrhythmia and symptoms. Her subsequent ECG revealed sinus tachycardia with δ waves, which was consistent with Wolff-Parkinson-White (WPW) syndrome. Laboratory findings confirmed thyroid storm and treatment began with intravenous hydrocortisone, methimazole, metoprolol, amiodarone and iodine drops. Graves' disease was confirmed based on the presence of serum thyroid-stimulating hormone receptor antibody. The patient underwent successful WPW accessory tract ablation 6 weeks after initial presentation. 2015 BMJ Publishing Group Ltd.

Wolff-Parkinson-White syndrome in the era of catheter ablation: insights from a registry study of 2169 patients.

Science.gov (United States)

Pappone, Carlo; Vicedomini, Gabriele; Manguso, Francesco; Saviano, Massimo; Baldi, Mario; Pappone, Alessia; Ciaccio, Cristiano; Giannelli, Luigi; Ionescu, Bogdan; Petretta, Andrea; Vitale, Raffaele; Cuko, Amarild; Calovic, Zarko; Fundaliotis, Angelica; Moscatiello, Mario; Tavazzi, Luigi; Santinelli, Vincenzo

2014-09-02

The management of Wolff-Parkinson-White is based on the distinction between asymptomatic and symptomatic presentations, but evidence is limited in the asymptomatic population. The Wolff-Parkinson-White registry was an 8-year prospective study of either symptomatic or asymptomatic Wolff-Parkinson-White patients referred to our Arrhythmology Department for evaluation or ablation. Inclusion criteria were a baseline electrophysiological testing with or without radiofrequency catheter ablation (RFA). Primary end points were the percentage of patients who experienced ventricular fibrillation (VF) or potentially malignant arrhythmias and risk factors. Among 2169 enrolled patients, 1001 (550 asymptomatic) did not undergo RFA (no-RFA group) and 1168 (206 asymptomatic) underwent ablation (RFA group). There were no differences in clinical and electrophysiological characteristics between the 2 groups except for symptoms. In the no-RFA group, VF occurred in 1.5% of patients, virtually exclusively (13 of 15) in children (median age, 11 years), and was associated with a short accessory pathway antegrade refractory period (PParkinson-White syndrome essentially depends on intrinsic electrophysiological properties of AP rather than on symptoms. RFA performed during the same procedure after electrophysiological testing is of benefit in improving the long-term outcomes. © 2014 American Heart Association, Inc.

Validity of dementia diagnoses in the danish hospital registers

DEFF Research Database (Denmark)

Phung, T.K.T.; Andersen, B.B.; Phung, T.K.T.

2007-01-01

.24-0.48). Conclusion: The validity of dementia syndrome in the Danish hospital registers was high and allows for epidemiological studies about dementia. Alzheimer's disease, although underregistered, also had a good validity once the diagnosis was registered. In general, other ICD-10 dementia subtypes in the registers......Background:The validity of dementia diagnoses in the Danish nationwide hospital registers was evaluated to determine the value of these registers in epidemiological research about dementia. Methods: Two hundred patients were randomly selected from 4,682 patients registered for the first time...... with a dementia diagnosis in the last 6 months of 2003. The patients' medical journals were reviewed to evaluate if they fulfilled ICD-10 and/or DSM-IV criteria for dementia and specific dementia subtypes. The patients who were still alive in 2006 were invited to an interview. Results: One hundred and ninety...

Parkinsonian syndroms: Clinical phenotype, differential diagnosis and disease progression

International Nuclear Information System (INIS)

Storch, A.

2002-01-01

Parkinsonian syndromes include idiopathic Parkinson's disease (IPD), other neurodegenerative diseases with parkinsonism, the so-called atypical parkinsonian syndromes, and symptomatic parkinsonian syndromes, such as Wilson's disease. IPD is the most frequent disease with parkinsonism as the main clinical feature and is responsible for approx. 80% of all parkinsonian syndromes. Atypical parkinsonian syndromes are the most important differential diagnoses of IPD. The two most frequent types are multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). For clinical diagnosis it is essential to take a careful medical history and to examine the patients physically in regular intervals. However, various clinico-pathological studies have shown that approx. 25% of patients with clinical diagnosis of IPD may have other causes of parkinsonism. Selected technical investigations, in particular functional imaging of the central dopaminergic system using PET or SPECT, may help to make clinical diagnosis more secure. This paper reviews the clinical features and diagnostic findings in diseases with parkinsonism and summarises the difficulties in establishing early and differential diagnoses. (orig.) [de

Phase analysis of regional and global ventricular contraction patterns in Wolff-Parkinson-White syndrome. Assessment by radionuclide ventriculography

Energy Technology Data Exchange (ETDEWEB)

Konishi, Tokuji; Koyama, Takao; Ichikawa, Takehiko and others

1989-04-01

Multigated blood pool scintigraphy was performed in 20 normal subjects and 39 patients with various intraventricular conduction abnormalities, including 25 patients with Wolff-Parkinson-White (WPW) syndrome. Cardiac imaging was performed in the modified left anterior oblique, right anterior oblique, and left lateral projections. In WPW syndrome, early contraction sites which were not seen in normal subjects were detected at the ventricular base in phase images. These anomalous early contraction sites disappeared after successful suppression of conduction through an accessory pathway by intravenous procainamide. These sites are believed to correspond to the location of the bundle of Kent and were consistent with the electrocardiographic findings. Phase mapping is a suitable noninvasive method to locate the position of the bundle of Kent and evaluate the ventricular contraction pattern in WPW syndrome and other intraventricular conduction abnormalities. (author).

[Cardioversion for paroxysmal supraventricular tachycardia during lung surgery in a patient with concealed Wolff-Parkinson-White syndrome].

Science.gov (United States)

Sato, Yoshiharu; Nagata, Hirofumi; Inoda, Ayako; Miura, Hiroko; Watanabe, Yoko; Suzuki, Kenji

2014-10-01

We report a case of paroxysmal supraventricular tachycardia (PSVT) that occurred during video-assisted thoracoscopic (VATS) lobectomy in a patient with concealed Wolff-Parkinson-White (WPW) syndrome. A 59-year-old man with lung cancer was scheduled for VATS lobectomy under general anesthesia. After inserting a thoracic epidural catheter, general anesthesia was induced with intravenous administration of propofol. Anesthesia was maintained with inhalation of desfurane in an air/oxygen mixture and intravenous infusion of remifentanil. Recurrent PSVT occurred three times, and the last episode of PSVT continued for 50 minutes regardless of administration of antiarrhythmic drugs. Synchronized electric shock via adhesive electrode pads on the patient's chest successfully converted PSVT back to normal sinus rhythm. The remaining course and postoperative period were uneventful. An electrophysiological study performed after hospital discharge detected concealed WPW syndrome, which had contributed to the development of atrioventricular reciprocating tachycardia. Concealed WPW syndrome is a rare, but critical complication that could possibly cause lethal atrial tachyarrhythmias during the perioperative period. In the present case, cardioversion using adhesive electrode pads briefly terminated PSVT in a patient with concealed WPW syndrome.

Neuroleptic malignant-like syndrome with a slight elevation of creatine-kinase levels and respiratory failure in a patient with Parkinson's disease

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Wei L

2014-02-01

Full Text Available Li Wei,1,2 Yinghui Chen1,2 1Department of Neurology, Jinshan Hospital, 2Department of Neurology, Shanghai Medical College, Fudan University, Shanghai, People's Republic of China Abstract: Neuroleptic malignant-like syndrome (NMLS is a rare but catastrophic complication of drug treatment for Parkinson's disease (PD. Sudden withdrawal and abrupt reduction of antiparkinsonian drugs are major risk factors. Just as its name suggests, the clinical features of NMLS are similar to neuroleptic malignant syndrome, which is a dangerous adverse response to antipsychotic drugs. Both of these conditions can present with hyperthermia, marked muscle rigidity, altered consciousness, autonomic dysfunction, and elevated serum creatine-kinase (CK levels. However, we describe a special NMLS case with a slight elevation of CK levels and respiratory failure in the full course of her treatment. The patient, a 68-year-old woman with a 4-years history of Parkinson's disease, presented with hyperthermia and severe muscular rigidity. During the course of her treatment, her maximum temperature was extremely high (above 41°C. At the beginning, the diagnosis of NMLS secondary to dopamine decrease was difficult to make, because her initial blood examination revealed that her serum CK levels were mildly elevated and decreased to normal range rapidly. Although antiparkinsonian drugs and supportive treatment were applied, the patient developed an acute respiratory failure in the early course of treatment. This case report highlights that when confronted with Parkinson's patients with high body temperature and muscle rigidity, NMLS should be taken into consideration even if there is no CK elevation. Likewise, the need for supportive care is essential, because its complications are severe, even such as respiratory failure. Keywords: antiparkinsonian drugs, creatine kinase, parkinsonism–hyperpyrexia syndrome, respiratory failure

Mental and physical performance of dementia patients in long-term residential care

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Zbigniew Śliwiński

2013-10-01

Full Text Available Introduction: Dementia syndromes are an increasing medical and social problem in today’s world. Preservation of the best possible quality of life in dementia patients relies on prolonging their independence in daily life for as long as possible. Dementia patients require increasing support as the disease progresses and will ultimately become dependent on the help of others. Aim of the research: To assess the level of mental and physical performance and nutritional status in patients with dementia syndromes in long-term residential care. Material and methods : The study group comprised 62 patients with dementia syndromes resident in a Medical and Nursing Care Facility in Pustków. Selected aspects of quality of life were investigated with the Barthel scale, GDS scale according to Reissberg, Abbreviated Mental Test Score (AMTS and Mini-Nutritional Assessment (MNA scale. Results: In our study men performed better than women on the Barthel scale, 58% of all patients were rated moderately severe on that scale, 36% were severe and 7% were mild. Assessment of the current severity of dementia on the GDS scale showed that 28% of the patients had very severe dementia, 27% had mild deficits, 27% had moderate deficits, 11% had moderately severe dementia and 6% had borderline dementia. In a mental state assessment according to the AMTS scale, men scored higher than women. This difference indicates less memory deficit and better psychological and physical status among men. With regard to nutritional status, our study revealed a risk of malnutrition in 65% of the patient and actual malnutrition in 7%. Conclusions : The Barthel scale, rating the performance of dementia patients with regard to activities of daily life, classified more than half of the patients as „moderately severe”. Women had lower mean scores than men in the Barthel scale, AMTS scale and GDS scale, indicating that dementia is more prevalent among women than among men. The findings of the

Diagnosis and management of dementia

African Journals Online (AJOL)

2007-09-19

Sep 19, 2007 ... Dementia is an acquired syndrome of memory decline with at least one other cognitive .... functions and memory retrieval are the .... behavioural problems.5 Cost-effectiveness of ... (For words not recalled, prompt with a cue.).

Wolff-Parkinson-White syndrome in young people, from childhood to young adulthood: relationships between age and clinical and electrophysiological findings

Science.gov (United States)

Jung, Hae Jung; Ju, Hwang Young; Hyun, Myung Chul; Lee, Sang Bum

2011-01-01

Purpose The aim of the present study was to evaluate the characteristics of electrophysiologic studies (EPS) and radiofrequency ablation (RFA) performed in subjects aged less than 30 years with Wolff-Parkinson-White (WPW) syndrome, particularly pediatric patients under 18 years of age, based on our experience. Methods Two hundred and one consecutive patients with WPW syndrome were recruited and divided to 3 groups according to age: group 1, 6 to 17 years; group 2, 18 to 29 years; and group 3, 30 to 60 years. The clinical, electrophysiological, and therapeutic data for these patients were evaluated by a retrospective medical record review. Results A total of 73 (36%) of these patients were syndrome. Therefore, when EPS and RFA are performed in children and adolescence with WPW syndrome, we recommend that these characteristics be considered. PMID:22323907

Sex differences in progression to mild cognitive impairment and dementia in Parkinson's disease.

Science.gov (United States)

Cholerton, Brenna; Johnson, Catherine O; Fish, Brian; Quinn, Joseph F; Chung, Kathryn A; Peterson-Hiller, Amie L; Rosenthal, Liana S; Dawson, Ted M; Albert, Marilyn S; Hu, Shu-Ching; Mata, Ignacio F; Leverenz, James B; Poston, Kathleen L; Montine, Thomas J; Zabetian, Cyrus P; Edwards, Karen L

2018-05-01

Identification of factors associated with progression of cognitive symptoms in Parkinson's disease (PD) is important for treatment planning, clinical care, and design of future clinical trials. The current study sought to identify whether prediction of cognitive progression is aided by examining baseline cognitive features, and whether this differs according to stage of cognitive disease. Participants with PD in the Pacific Udall Center Clinical Consortium who had longitudinal data available and were nondemented at baseline were included in the study (n = 418). Logistic and Cox regression models were utilized to examine the relationship between cognitive, demographic, and clinical variables with risk and time to progression from no cognitive impairment to mild cognitive impairment (PD-MCI) or dementia (PDD), and from PD-MCI to PDD. Processing speed (OR = 1.05, p = 0.009) and working memory (OR = 1.01, p = 0.03) were associated with conversion to PDD among those with PD-MCI at baseline, over and above demographic variables. Conversely, the primary predictive factor in the transition from no cognitive impairment to PD-MCI or PDD was male sex (OR = 4.47, p = 0.004), and males progressed more rapidly than females (p = 0.01). Further, among females with shorter disease duration, progression was slower than for their male counterparts, and poor baseline performance on semantic verbal fluency was associated with shorter time to cognitive impairment in females but not in males. This study provides evidence for sex differences in the progression to cognitive impairment in PD, while specific cognitive features become more important indicators of progression with impending conversion to PDD. Copyright © 2018 Elsevier Ltd. All rights reserved.

Late onset myoclonic epilepsy in Down syndrome and dementia

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Annapia Verri

2012-09-01

Full Text Available Specific forms of epilepsy may be found at various ages in Down Syndrome (DS and a sharp increase in the incidence of epilepsy with age has been documented. A specific type of myoclonic epilepsy associated with cognitive decline has been reported as “senile myoclonic epilepsy” or “late onset myoclonic epilepsy in DS” (LOMEDS. We report a new case of LOMEDS, documented by clinical and neurophysiological evaluation and psychometric assessment (DSDS and DMR. MF, male, affected by DS, was referred in 2004 at 40 years of age; he had no personal or familial history of epilepsy. Since one year, the patient presented cognitive deterioration, characterized by regression of language abilities, loss of memory, and loss of sphincters control. A brain TC showed mild brainstem and sub-cortical atrophy. In 2006, myoclonic jerks involving upper limbs occurred mainly after awakening. EEG showed a low voltage 8 Hz background activity with diffuse slow activity, intermingled with spikes or polyspikes, persisting during NREM sleep. MF was initially treated with clonazepam and after with topiramate, resulting in partial seizures control. MRI (2008 demonstrated diffuse brain atrophy, associated with marked ventricular enlargement. At the psychometric evaluation, onset of dementia was evident late in 2004, with transition to the middle stage in 2006. Last assessment (2009 showed the clinical signs of a late stage of deterioration, with loss of verbal abilities and autonomous ambulation. Using levetiracetam till 2,000 mg/die, myoclonic jerks decreased but are still present every day after awakening. On the EEG slow and poorly organized background activity with bilateral polyspike-wave discharges was recorded. Therefore, we documented a parallel progression of dementia and myoclonic epilepsy in a DS subject.

Current strategy for treatment of patients with Wolff-Parkinson-White syndrome and asymptomatic preexcitation in Europe

DEFF Research Database (Denmark)

Svendsen, Jesper Hastrup; Dagres, Nikolaos; Dobreanu, Dan

2013-01-01

network, covering 20 countries answered the survey questions. All centres were high-volume ablation centres. A younger person with asymptomatic Wolff-Parkinson-White (WPW) pattern has a higher likelihood of being risk-stratified or receiving ablation therapy compared with an older subject. Two...... of responding centres (61-69%) report that their country lack national guidelines dealing with clinical strategies related to WPW. There is a need for national guidelines dealing with clinical strategy in patients with WPW syndrome. Older individuals with asymptomatic WPW pattern have a higher risk...

Cholinergic imaging in dementia spectrum disorders

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Roy, Roman; Niccolini, Flavia; Pagano, Gennaro; Politis, Marios [Institute of Psychiatry, Psychology and Neuroscience, King' s College London, Neurodegeneration Imaging Group, Department of Basic and Clinical Neuroscience, London (United Kingdom)

2016-07-15

The multifaceted nature of the pathology of dementia spectrum disorders has complicated their management and the development of effective treatments. This is despite the fact that they are far from uncommon, with Alzheimer's disease (AD) alone affecting 35 million people worldwide. The cholinergic system has been found to be crucially involved in cognitive function, with cholinergic dysfunction playing a pivotal role in the pathophysiology of dementia. The use of molecular imaging such as SPECT and PET for tagging targets within the cholinergic system has shown promise for elucidating key aspects of underlying pathology in dementia spectrum disorders, including AD or parkinsonian dementias. SPECT and PET studies using selective radioligands for cholinergic markers, such as [{sup 11}C]MP4A and [{sup 11}C]PMP PET for acetylcholinesterase (AChE), [{sup 123}I]5IA SPECT for the α{sub 4}β{sub 2} nicotinic acetylcholine receptor and [{sup 123}I]IBVM SPECT for the vesicular acetylcholine transporter, have been developed in an attempt to clarify those aspects of the diseases that remain unclear. This has led to a variety of findings, such as cortical AChE being significantly reduced in Parkinson's disease (PD), PD with dementia (PDD) and AD, as well as correlating with certain aspects of cognitive function such as attention and working memory. Thalamic AChE is significantly reduced in progressive supranuclear palsy (PSP) and multiple system atrophy, whilst it is not affected in PD. Some of these findings have brought about suggestions for the improvement of clinical practice, such as the use of a thalamic/cortical AChE ratio to differentiate between PD and PSP, two diseases that could overlap in terms of initial clinical presentation. Here, we review the findings from molecular imaging studies that have investigated the role of the cholinergic system in dementia spectrum disorders. (orig.)

Cholinergic imaging in dementia spectrum disorders

International Nuclear Information System (INIS)

Roy, Roman; Niccolini, Flavia; Pagano, Gennaro; Politis, Marios

2016-01-01

The multifaceted nature of the pathology of dementia spectrum disorders has complicated their management and the development of effective treatments. This is despite the fact that they are far from uncommon, with Alzheimer's disease (AD) alone affecting 35 million people worldwide. The cholinergic system has been found to be crucially involved in cognitive function, with cholinergic dysfunction playing a pivotal role in the pathophysiology of dementia. The use of molecular imaging such as SPECT and PET for tagging targets within the cholinergic system has shown promise for elucidating key aspects of underlying pathology in dementia spectrum disorders, including AD or parkinsonian dementias. SPECT and PET studies using selective radioligands for cholinergic markers, such as [ 11 C]MP4A and [ 11 C]PMP PET for acetylcholinesterase (AChE), [ 123 I]5IA SPECT for the α 4 β 2 nicotinic acetylcholine receptor and [ 123 I]IBVM SPECT for the vesicular acetylcholine transporter, have been developed in an attempt to clarify those aspects of the diseases that remain unclear. This has led to a variety of findings, such as cortical AChE being significantly reduced in Parkinson's disease (PD), PD with dementia (PDD) and AD, as well as correlating with certain aspects of cognitive function such as attention and working memory. Thalamic AChE is significantly reduced in progressive supranuclear palsy (PSP) and multiple system atrophy, whilst it is not affected in PD. Some of these findings have brought about suggestions for the improvement of clinical practice, such as the use of a thalamic/cortical AChE ratio to differentiate between PD and PSP, two diseases that could overlap in terms of initial clinical presentation. Here, we review the findings from molecular imaging studies that have investigated the role of the cholinergic system in dementia spectrum disorders. (orig.)

Toxic adenoma of the thyroid gland and Wolff-Parkinson-White syndrome

Science.gov (United States)

Naço, M; Çeliku, E; Llukaçaj, A; Shehaj, J; Kameniku, R

2009-01-01

We report the case of a 17-year-old girl with toxic adenoma scheduled for surgery right lobectomy and isthmectomy of thyroid gland. During the examination before surgery, patient was diagnosed for the first time as having with Wolff – Parkinson – White (WPW) syndrome. In the operating room, after the induction of anesthesia, the electrocardiogram showed wide QRS complex tachycardia with a rate of 180 beats/min, which was diagnosed as paroxysmal supraventricular tachycardia. The patient was treated immediately with antiarrhythmic drugs: adenosine iv three times (at doses of 6 mg, 12mg, 12mg bolus) and esmolol iv twice (at doses 28.5 mg). This approach resulted in disappearance of the delta wave and tachycardia for the whole surgery period. In this case report we discuss the role of induction of anesthesia and presence of toxic adenoma in a patient with WPW. PMID:19561784

Restless legs syndrome in Parkinson's disease: clinical characteristics and biochemical correlations

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Tiago Machado Guerreiro

2010-12-01

Full Text Available Restless legs syndrome (RLS is a neurological disorder that responds to dopaminergic drugs, indicating a common pathophysiology with Parkinson's disease (PD. The prevalence of RLS was estimated in a group of PD patients and its clinical and biochemical characteristics were analysed. Forty-eight patients with PD were evaluated into two groups, with and without RLS. Clinical characteristics assessed in both groups were age, gender, duration of PD, Hoehn and Yahr, and Schwab and England scales. Laboratory variables such as hemoglobin, s-iron, s-ferritin and creatinine were obtained. The prevalence of RLS was 18.75%. No significant differences regarding clinical variables and biochemical parameters were observed. The high prevalence of RLS found in PD patients suggests the concept of a common etiological link and it seems that secondary causes did not play a central role in the pathophysiology of RLS in this group of parkinsonian patients.

New possibility of traditional Chinese and Japanese medicine as treatment for behavioral and psychiatric symptoms in dementia

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Kung FC

2012-10-01

Full Text Available Fan-Chin Kung,1 Ryouhei Ishii,2 Hsing-Cheng Liu,3 Masatoshi Takeda21Yuli Hospital, DOH, Hualien, Taiwan; 2Department of Psychiatry, Osaka University Graduate School of Medicine, Osaka, Japan; 3Department of General Psychiatry, Taipei City Psychiatric Center, Taipei, TaiwanAbstract: Yokukansan, one of the Kampo prescriptions, is composed of seven herbaceous plants and was developed in China in the 16th century as a cure for restlessness and agitation in children. Yokukansan has also become a popular drug combination in Japan, especially for the behavioral and psychiatric symptoms of dementia (BPSD. Recent studies have shown that yokukansan might also be quite effective against BPSD occurring in association with other types of dementia, such as Alzheimer's disease, Lewy body disease, Parkinson's disease with dementia, frontotemporal dementia, and vascular dementia. Researchers have intensively investigated yokukansan, focusing on the pharmacological mechanisms against glutamate-mediated excitotoxicity. This traditional Chinese and Japanese medicine holds potential promise for improving BPSD in elderly patients suffering from dementia.Keywords: yokukansan, dementia, Alzheimer's disease, behavioral and psychological symptoms of dementia, Kampo, glutamate

Correlation between pathology and neuromelanin MR imaging in Parkinson's disease and dementia with Lewy bodies

International Nuclear Information System (INIS)

Kitao, Shinichiro; Fujii, Shinya; Miyoshi, Fuminori; Kaminou, Toshio; Ogawa, Toshihide; Matsusue, Eiji; Kato, Shinsuke; Ito, Hisao

2013-01-01

Direct correlation between neuropathological findings and postmortem neuromelanin MR imaging (NmMRI) was performed in the substantia nigra pars compacta (SNc) to clarify the pathological background of the signal changes in normal, Parkinson's disease (PD), and dementia with Lewy bodies (DLB) cases. NmMRI of 10 % formalin-fixed autopsied midbrains was performed in three cases (normal control, DLB, and PD) with a 3T imaging system, using a 3D gradient echo T1-weighted sequence with a magnetization transfer contrast pulse. Neuropathological examinations of the midbrains were performed, and the density of neuromelanin-positive neurons (number per square millimeter) was determined. The extent of iron deposition in the midbrain was also evaluated using ferritin immunohistochemistry. Furthermore, we directly correlated the contrast signal ratio in the SNc and the density of neuromelanin-containing neurons. Diffuse hyperintense areas in the SNc reflected well-preserved neuromelanin-containing neurons in the normal control case, whereas an iso-intense area in the SNc showed severe loss of neuromelanin-containing neurons in the DLB and PD cases. Increased signal intensity in the SNc was apparently not influenced by iron deposition. Furthermore, a significant positive correlation between signal intensity and the density of neuromelanin-containing neurons was seen in the SNc. Based on the direct correlation between postportem NmMRI and neuropathological findings, signal intensity in the SNc is closely related to the quantity of neuromelanin-containing neurons but is not influenced by iron deposition. (orig.)

Wolf-Parkinson-White syndrome in young men presenting with palpitation: the pattern of delta waves in predicting location of accessory pathway

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Miryanti Cahyaningtias

2011-11-01

Full Text Available Palpitation is a common presenting symptom in the emergency department. Wolf-Parkinson White (WPW syndrome is a cardiac conduction disorder that may present with palpitation and lead to sudden cardiac death. WPW could be detected by  electrocardiogram (ECG. In this case report, we present two young male patients with WPW syndrome admitted to our hospital with history of repeated and progressive palpitation. ECG of the first patient revealed supraventricular tachycardia which converted to sinus rhythm after propanolol treatment. ECG showed sinus rhythm with delta wave in lead II,III,aVF, V1 suggesting the presence of accessory pathway (AP in left lateral wall. Electrophysiology study confirmed the presence of AP and radio frequency catheter ablation was successfully done resulted in disappearance of delta on outpatient clinic ECG. Patient has no symptom and he do not have to take medication. ECG of the second patient revealed supraventricular tachycardia with abberancy. After amiodarone infusion, ECG showed sinus rhythm with delta wave in lead I,II,aVL suggesting the presence of accessory pathway in anteroseptal wall. Electrophysiology study and catheter ablation did not perform for this patient because of financial problem, however amidarone has to be taken regularly to prevent the recurrence of supraventricular tachycardia. (Med J Indones 2011; 20:298-301Keywords: ECG, palpitation, supraventricular tachycardia, Wolf- Parkinson White syndrome

Imaging insights into basal ganglia function, Parkinson's disease, and dystonia.

Science.gov (United States)

Stoessl, A Jon; Lehericy, Stephane; Strafella, Antonio P

2014-08-09

Recent advances in structural and functional imaging have greatly improved our ability to assess normal functions of the basal ganglia, diagnose parkinsonian syndromes, understand the pathophysiology of parkinsonism and other movement disorders, and detect and monitor disease progression. Radionuclide imaging is the best way to detect and monitor dopamine deficiency, and will probably continue to be the best biomarker for assessment of the effects of disease-modifying therapies. However, advances in magnetic resonance enable the separation of patients with Parkinson's disease from healthy controls, and show great promise for differentiation between Parkinson's disease and other akinetic-rigid syndromes. Radionuclide imaging is useful to show the dopaminergic basis for both motor and behavioural complications of Parkinson's disease and its treatment, and alterations in non-dopaminergic systems. Both PET and MRI can be used to study patterns of functional connectivity in the brain, which is disrupted in Parkinson's disease and in association with its complications, and in other basal-ganglia disorders such as dystonia, in which an anatomical substrate is not otherwise apparent. Functional imaging is increasingly used to assess underlying pathological processes such as neuroinflammation and abnormal protein deposition. This imaging is another promising approach to assess the effects of treatments designed to slow disease progression. Copyright © 2014 Elsevier Ltd. All rights reserved.

Risk of malignant arrhythmias in initially symptomatic patients with Wolff-Parkinson-White syndrome: results of a prospective long-term electrophysiological follow-up study.

Science.gov (United States)

Pappone, Carlo; Vicedomini, Gabriele; Manguso, Francesco; Baldi, Mario; Pappone, Alessia; Petretta, Andrea; Vitale, Raffaele; Saviano, Massimo; Ciaccio, Cristiano; Giannelli, Luigi; Calovic, Zarko; Tavazzi, Luigi; Santinelli, Vincenzo

2012-02-07

The available amount of detailed long-term data in patients with Wolff-Parkinson-White syndrome is limited, and no prospective electrophysiological studies looking at predictors of malignant arrhythmia are available. Among 8575 symptomatic Wolff-Parkinson-White patients with atrioventricular reentrant tachycardia referred for electrophysiological test, 369 (mean age, 23±12.5 years) declined catheter ablation and were followed up. The primary end point of the study was to evaluate over a 5-year follow-up the predictors and characteristics of patients who develop malignant arrhythmias. After a mean follow-up of 42.1±10 months, malignant arrhythmias developed in 29 patients (mean age, 13.9±5.6 years; 26 male), resulting in presyncope/syncope (25 patients), hemodynamic collapse (3 patients), or cardiac arrest caused by ventricular fibrillation (1 patient). Of the remaining 340 patients, 168 (mean age, 34.2±9.0 years) remained asymptomatic up to 5 years, and 172 (mean age, 13.6±5.1 years) had benign recurrence, including sustained atrioventricular reentrant tachycardia (132 patients) or atrial fibrillation (40 patients). Compared with the group with no malignant arrhythmias, the group with malignant arrhythmias showed shorter accessory-pathway effective refractory period (PWolff-Parkinson-White syndrome generally have a good outcome, and predictors of malignant arrhythmias are similar to those reported for asymptomatic patients with ventricular pre-excitation.

Treatment of autonomic dysfunction in Parkinson disease and other synucleinopathies.

Science.gov (United States)

Palma, Jose-Alberto; Kaufmann, Horacio

2018-03-01

Dysfunction of the autonomic nervous system afflicts most patients with Parkinson disease and other synucleinopathies such as dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure, reducing quality of life and increasing mortality. For example, gastrointestinal dysfunction can lead to impaired drug pharmacodynamics causing a worsening in motor symptoms, and neurogenic orthostatic hypotension can cause syncope, falls, and fractures. When recognized, autonomic problems can be treated, sometimes successfully. Discontinuation of potentially causative/aggravating drugs, patient education, and nonpharmacological approaches are useful and should be tried first. Pathophysiology-based pharmacological treatments that have shown efficacy in controlled trials of patients with synucleinopathies have been approved in many countries and are key to an effective management. Here, we review the treatment of autonomic dysfunction in patients with Parkinson disease and other synucleinopathies, summarize the nonpharmacological and current pharmacological therapeutic strategies including recently approved drugs, and provide practical advice and management algorithms for clinicians, with focus on neurogenic orthostatic hypotension, supine hypertension, dysphagia, sialorrhea, gastroparesis, constipation, neurogenic overactive bladder, underactive bladder, and sexual dysfunction. © 2018 International Parkinson and Movement Disorder Society. © 2018 International Parkinson and Movement Disorder Society.

Clinical Subtypes of Dementia with Lewy Bodies Based on the Initial Clinical Presentation.

Science.gov (United States)

Morenas-Rodríguez, Estrella; Sala, Isabel; Subirana, Andrea; Pascual-Goñi, Elba; Sánchez-Saudinós, MaBelén; Alcolea, Daniel; Illán-Gala, Ignacio; Carmona-Iragui, María; Ribosa-Nogué, Roser; Camacho, Valle; Blesa, Rafael; Fortea, Juan; Lleó, Alberto

2018-06-04

Dementia with Lewy bodies (DLB) is a heterogeneous disease in which clinical presentation, symptoms, and evolution widely varies between patients. To investigate the existence of clinical subtypes in DLB based on the initial clinical presentation. 81 patients with a clinical diagnosis of probable DLB were consecutively included. All patients underwent a neurological evaluation including a structured questionnaire about neuropsychiatric symptoms and sleep, an assessment of motor impairment (Unified Parkinson Disease Rating Scale subscale III), and a formal neuropsychological evaluation. Onset of core symptoms (hallucinations, parkinsonism, and fluctuations) and dementia were systematically reviewed from medical records. We applied a K-means clustering method based on the initial clinical presentation. Cluster analysis yielded three different groups. Patients in cluster I (cognitive-predominant, n = 46) presented more frequently with cognitive symptoms (95.7%, n = 44, p presented more frequently with psychotic symptoms (77.3%, n = 17), and had a shorter duration until the onset of hallucinations (p clinical DLB can be defined when considering the differential initial presentations. The proposed subtypes have distinct clinical profiles and progression patterns.

Dysphagia in Lewy body dementia - a clinical observational study of swallowing function by videofluoroscopic examination.

Science.gov (United States)

Londos, Elisabet; Hanxsson, Oskar; Alm Hirsch, Ingrid; Janneskog, Anna; Bülow, Margareta; Palmqvist, Sebastian

2013-10-07

Dysphagia, which can result in aspiration pneumonia and death, is a well-known problem in patients with dementia and Parkinson's disease. There are few studies on dysphagia in patients with dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), especially studies objectively documenting the type of swallowing dysfunction. The aim of this study was therefore to investigate the prevalence, and define the actual swallowing dysfunction according to a videofluoroscopic swallowing examination (VFSE) in patients with DLB and PDD. Eighty-two consecutive patients with DLB or PDD in a clinical follow-up program were asked about symptoms of dysphagia. Those experiencing dysphagia were examined with VFSE. Prevalence and type of swallowing dysfunction was recorded. Twenty-six patients (32%) reported symptoms of dysphagia such as swallowing difficulties or coughing. Twenty-four (92%) of these had a documented swallowing dysfunction on VFSE. Eighty-eight percent suffered from pharyngeal dysfunction. Almost all DLB or PDD patients with subjective signs of dysphagia had pathologic results on VFSE, the majority of pharyngeal type. This type of dysphagia has not been reported in DLB before. The results have clinical implications and highlight the importance of asking for and examining swallowing function to prevent complications such as aspiration.

[Neuroepigenetics: Desoxyribonucleic acid methylation in Alzheimer's disease and other dementias].

Science.gov (United States)

Mendioroz Iriarte, Maite; Pulido Fontes, Laura; Méndez-López, Iván

2015-05-21

DNA methylation is an epigenetic mechanism that controls gene expression. In Alzheimer's disease (AD), global DNA hypomethylation of neurons has been described in the human cerebral cortex. Moreover, several variants in the methylation pattern of candidate genes have been identified in brain tissue when comparing AD patients and controls. Specifically, DNA methylation changes have been observed in PSEN1 and APOE, both genes previously being involved in the pathophysiology of AD. In other degenerative dementias, methylation variants have also been described in key genes, such as hypomethylation of the SNCA gene in Parkinson's disease and dementia with Lewy bodies or hypermethylation of the GRN gene promoter in frontotemporal dementia. The finding of aberrant DNA methylation patterns shared by brain tissue and peripheral blood opens the door to use those variants as epigenetic biomarkers in the diagnosis of neurodegenerative diseases. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

Effect of atrial pacing on phase analysis in patients with the Wolff-Parkinson-White syndrome

International Nuclear Information System (INIS)

Magosaki, Nobuhisa; Hiroe, Michiaki; Kasanuki, Hiroshi; Ohnishi, Satoshi; Tanaka, Etsuko; Horie, Toshinobu; Kusakabe, Kiyoko; Kondo, Mizuka; Hirosawa, Koshichiro

1984-01-01

Phase analysis of ECG-gated radionuclide ventriculography was performed during sinus rhythm and atrial pacing for 11 patients with the Wolff-Parkinson-White (WPW) syndrome. During sinus rhythm, phase analysis demonstrated abnormal early-emptying segments reflecting preexcitation in six of the 11 patients. In the remaining five patients, the precise site of abnormal early-emptying could not be detected. Atrial pacing increased the degree of pre-exicitation, and abnormal early-emptying segments became clear in all patients. Our study demonstrated the utility of atrial pacing in performing phase analysis for patients with the WPW syndrome. Seven patients had abnormal early-emptying segments; four of them with ECG type A, in the left ventricle, and three of them with ECG type B, in the right ventricle. These results were consistent with results of the body surface and electrophysiologic mapping. In patients with posterior septal accessory pathways, phase analysis suggested probable laterality of the accessory pathway. (author)

Phase analysis in the Wolff-Parkinson-White syndrome with surgically proven accessory conduction pathways: concise communication

International Nuclear Information System (INIS)

Nakajima, K.; Bunko, H.; Tada, A.; Taki, J.; Tonami, N.; Hisada, K.; Misaki, T.; Iwa, T.

1984-01-01

Twenty-one patients with the Wolff-Parkinson-White (WPW) syndrome who underwent surgical division of the accessory conduction pathway (ACP) were studied by gated blood-pool scintigraphy. In each case, a functional image of the phase was generated, based on the fundamental frequency of the Fourier transform. The location of the ACP was confirmed by electrophysiologic study, epicardial mapping, and surgery. Phase analysis identified the side of preexcitation correctly in 16 out of 20 patients with WPW syndrome with a delta wave. All patients with right-cardiac type (N=9) had initial contraction in the right ventricle (RV). In patients with left-cardiac type (N=10), six had initial movement in the left ventricle (LV); but in the other four the ACPs in the anterior or lateral wall of the left ventricle (LV) could not be detected. In patients with multiple ACPs (N=2), one right-cardiac type had initial contraction in the RV, while in the other (with an intermittent WPW syndrome) the ACP was not detected. These observations indicate that abnormal wall motion is associated with the conduction anomalies of the WPW syndrome. We conclude that phase analysis can correctly identify the side of initial contraction in the WPW syndrome before and after surgery. However, as a method of preoperative study, it seems difficult to determine the precise site of the ACP by phase analysis alone

Phase analysis in the Wolff-Parkinson-White syndrome with surgically proven accessory conduction pathways: concise communication

Energy Technology Data Exchange (ETDEWEB)

Nakajima, K.; Bunko, H.; Tada, A.; Taki, J.; Tonami, N.; Hisada, K.; Misaki, T.; Iwa, T.

1984-01-01

Twenty-one patients with the Wolff-Parkinson-White (WPW) syndrome who underwent surgical division of the accessory conduction pathway (ACP) were studied by gated blood-pool scintigraphy. In each case, a functional image of the phase was generated, based on the fundamental frequency of the Fourier transform. The location of the ACP was confirmed by electrophysiologic study, epicardial mapping, and surgery. Phase analysis identified the side of preexcitation correctly in 16 out of 20 patients with WPW syndrome with a delta wave. All patients with right-cardiac type (N=9) had initial contraction in the right ventricle (RV). In patients with left-cardiac type (N=10), six had initial movement in the left ventricle (LV); but in the other four the ACPs in the anterior or lateral wall of the left ventricle (LV) could not be detected. In patients with multiple ACPs (N=2), one right-cardiac type had initial contraction in the RV, while in the other (with an intermittent WPW syndrome) the ACP was not detected. These observations indicate that abnormal wall motion is associated with the conduction anomalies of the WPW syndrome. We conclude that phase analysis can correctly identify the side of initial contraction in the WPW syndrome before and after surgery. However, as a method of preoperative study, it seems difficult to determine the precise site of the ACP by phase analysis alone.

[Sleep disturbances in Parkinson's disease: characteristics, evaluation and therapeutic approaches].

Science.gov (United States)

Faludi, Béla; Janszky, József; Komoly, Sámuel; Kovács, Norbert

2015-07-05

Parkinson's disease is a well known representent of the movement disorder group of neurological disorders. The diagnosis of Parkinson's disease is based on specific symptoms and signs of movement abnormalities. In addition to classic motor symptoms, Parkinson's disease has characteristic non-motor features, and some of these emerges the classic signs. The authors discuss characteristics and therapeutic interventions in Parkinson's disease related sleep disturbances. The authors reviewed and summarised literature data on sleep disorders in Parkinson's disease published in the PubMed database up to January 2015. Sleep problems are important non-motor complains (insomnia, hypersomnia, REM behaviour disorder, sleep apnea and restless legs syndrome). The neurodegenerative process of the brain-stem, the effect of symptoms of Parkinson's disease on sleep and concomitant sleep disorders constitute the background of the patient's complains. Appropriate diagnosis and therapy of the consequential or concomitant sleep disorders in Parkinson's disease will help to improve the patient's quality of life.

Comparison of the accuracy of three algorithms in predicting accessory pathways among adult Wolff-Parkinson-White syndrome patients.

Science.gov (United States)

Maden, Orhan; Balci, Kevser Gülcihan; Selcuk, Mehmet Timur; Balci, Mustafa Mücahit; Açar, Burak; Unal, Sefa; Kara, Meryem; Selcuk, Hatice

2015-12-01

The aim of this study was to investigate the accuracy of three algorithms in predicting accessory pathway locations in adult patients with Wolff-Parkinson-White syndrome in Turkish population. A total of 207 adult patients with Wolff-Parkinson-White syndrome were retrospectively analyzed. The most preexcited 12-lead electrocardiogram in sinus rhythm was used for analysis. Two investigators blinded to the patient data used three algorithms for prediction of accessory pathway location. Among all locations, 48.5% were left-sided, 44% were right-sided, and 7.5% were located in the midseptum or anteroseptum. When only exact locations were accepted as match, predictive accuracy for Chiang was 71.5%, 72.4% for d'Avila, and 71.5% for Arruda. The percentage of predictive accuracy of all algorithms did not differ between the algorithms (p = 1.000; p = 0.875; p = 0.885, respectively). The best algorithm for prediction of right-sided, left-sided, and anteroseptal and midseptal accessory pathways was Arruda (p algorithms were similar in predicting accessory pathway location and the predicted accuracy was lower than previously reported by their authors. However, according to the accessory pathway site, the algorithm designed by Arruda et al. showed better predictions than the other algorithms and using this algorithm may provide advantages before a planned ablation.

Mild Cognitive Impairment as a Risk Factor for Parkinson's Disease Dementia

NARCIS (Netherlands)

Hoogland, Jeroen; Boel, Judith A.; de Bie, Rob M. A.; Geskus, Ronald B.; Schmand, Ben A.; Dalrymple-Alford, John C.; Marras, Connie; Adler, Charles H.; Goldman, Jennifer G.; Tröster, Alexander I.; Burn, David J.; Litvan, Irene; Geurtsen, Gert J.

2017-01-01

Background: The International Parkinson and Movement Disorder Society criteria for mild cognitive impairment in PD were recently formulated. Objectives: The aim of this international study was to evaluate the predictive validity of the comprehensive (level II) version of these criteria by assessment

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.

Incidence of Dementia in Older Adults with Intellectual Disabilities

Science.gov (United States)

Strydom, Andre; Chan, Trevor; King, Michael; Hassiotis, Angela; Livingston, Gill

2013-01-01

Dementia may be more common in older adults with intellectual disability (ID) than in the general population. The increased risk for Alzheimer's disease in people with Down syndrome (DS) is well established, but much less is known about dementia in adults with ID who do not have DS. We estimated incidence rates from a longitudinal study of…

Best practice in caring for adults with dementia and learning disabilities.

Science.gov (United States)

Strydom, André; Al-Janabi, Tamara; Houston, Marie; Ridley, James

2016-10-05

People with learning disabilities, particularly Down's syndrome, are at increased risk of dementia. At present, services and care tailored to people with both dementia and a learning disability are unsatisfactory. This article reviews the literature specific to dementia in people with learning disabilities, including: comprehensive screening, diagnosis, management, environmental considerations, end of life care and training issues for nursing staff. Recommendations for best practice and service improvement are made to improve the quality of life for individuals with dementia and learning disabilities, pre and post-diagnosis.

The effects of anticholinergic drugs on regional cerebral blood flow, and oxygen metabolism in previously untreated patients with Parkinson`s disease

Energy Technology Data Exchange (ETDEWEB)

Obara, Satoko; Takahashi, Satoshi; Yonezawa, Hisashi; Sato, Yoshitomo [Iwate Medical Univ., Morioka (Japan). School of Medicine

1998-12-01

Regional cerebral blood flow (rCBF) and oxygen metabolism (rCMRO{sub 2}) were measured using the steady-state {sup 15}O technique and positron emission tomography (PET) in six previously untreated patients with Parkinson`s disease before and after trihexyphenidyl (THP) treatment. The patients comprised of 4 men and 2 women with Hoehn-Yahr stage II-III. Their ages at the onset of the study ranged from 46 to 57 years (mean{+-}SD, 51.8{+-}3.7) and the duration of the illness ranged from 10 to 48 months (mean{+-}SD, 28.8{+-}15.5). The PET study, assessments of the disability and cognitive function were undergone twice. The first time assessments were done was when the patients were not receiving any drugs, and the second time was one to three months after administration of 6 mg THP. All patients showed clinical improvement after THP treatment. The mean disability score of Unified Parkinson`s Disease Rating Scale decreased from 35.1 (SD{+-}11.3) to 25.7 (SD{+-}11.6). The cognitive function assessed by Hasegawa`s dementia rating scale-revised, Mini-Mental State Examination, Wechsler Adult Intelligence Scale-Revised, and Wechsler Memory Scale-Revised, were not significantly different before and after the THP treatment. After the THP treatment, rCBF and rCMRO{sub 2} decreased significantly in the striatum (about 15%) and all cerebral cortices (about 10%) on both sides contralateral and ipsilateral to the predominantly symptomatic limbs. We conclude that an anticholinergic THP decreases the rCBF and rCMRO{sub 2} significantly in the cerebral cortices without cognitive impairment in early untreated patients with Parkinson`s disease. (author)

Amiodarone and Catheter Ablation as Cardiac Resynchronization Therapy for Children with Dilated Cardiomyopathy and Wolff-Parkinson-White Syndrome

Science.gov (United States)

Kim, Sung Hoon; Jeong, Soo In; Kang, I-Seok; Lee, Heung Jae

2013-01-01

Preexcitation by accessory pathways (APs) is known to cause dyssynchrony of the ventricle, related to ventricular dysfunction. Correction of ventricular dyssynchrony can improve heart failure in cases of dilated cardiomyopathy (DCMP) with preexcitation. Here, we report the first case of a child with DCMP and Wolff-Parkinson-White (WPW) syndrome treated with amiodarone and radiofrequency catheter ablation (RFCA) in Korea. A 7-year-old boy, who suffered from DCMP and WPW syndrome, showed improved left ventricular function and clinical functional class after treatment with amiodarone to eliminate preexcitation. QRS duration and left ventricular ejection fraction (LVEF) were inversely correlated with amiodarone dosage. After confirming the reduction of preexcitation effects in DCMP, successful RFCA of the right anterior AP resulted in LVEF improvement, along with the disappearance of preexcitation. Our findings suggest that ventricular dyssynchrony, caused by preexcitation in DCMP with WPW syndrome, can worsen ventricular function and amiodarone, as well as RFCA, which should be considered as a treatment option, even in young children. PMID:23407697

The usefulness of SPECT and MRI in the diagnosis of atypical parkinsonian syndromes

International Nuclear Information System (INIS)

Skogseid, I.M.; Gerdts, R.; Nyberg-Hansen, R.; Rootwelt, K.; Bakke, S.J.

2001-01-01

Clinico-pathological studies have shown that only three out of four patients with parkinsonism have idiopathic Parkinson's disease. In patients with so-called Parkinson plus syndrome, the degeneration in the brain is more widespread and the variety of neurological signs greater than in Parkinson's disease. The differentiation of these syndromes from Parkinson's disease can be difficult. Single photon emission computed tomography (SPECT) and magnetic resonance imaging (MRl) can be of value in the differential diagnosis of parkinsonism. We present three patients with atypical parkinsonism in whom MRI and SPECT with β-CIT and epidepride was performed in addition to the clinical evaluation. The three patients all had a rapidly developing symmetric akinetic-rigid syndrome that responded poorly to levodopa. MRI showed findings regarded as typical for multiple system atrophy in two patients, but only nonspecific findings in the third patient. SPECT with β-CIT showed a pronounced bilateral and relatively symmetric reduction in the striatal dopaminergic activity in all patients. SPECT with epidepride showed a clearly reduced striatal D 2 -receptor binding bilaterally in only one of the patients. In patients with atypical parkinsonism, MRI and SPECT with β-CIT and epidepride can give valuable support to the clinical diagnosis of a Parkinson plus syndrome

Delaying cognitive and physical decline through multidomain interventions for residents with mild-to-moderate dementia in dementia care units in Taiwan: A prospective cohort study.

Science.gov (United States)

Liang, Chih-Kuang; Chou, Ming-Yueh; Chen, Liang-Yu; Wang, Kuei-Yu; Lin, Shih-Yi; Chen, Liang-Kung; Lin, Yu-Te; Liu, Tsung-Yun; Loh, Ching-Hui

2017-04-01

To develop experimental multi-domain interventions for older people with mild-to-moderate dementia, and to evaluate the effect of delaying cognitive and physical decline, and improvement or prevention of geriatric syndromes during 1-year follow up. Participants aged 65 years and older with mild-to-moderate dementia (clinical dementia rating [CDR] 1 or 2) were grouped as intervention in Jia-Li Veterans Home and usual care model in the community (Memory clinic). All residents in Jia-Li Veterans Home received comprehensive intervention, including Multi-disciplinary team consultation and intervention, Multi-component non-pharmacological management, geriatric syndromes survey and intervention by CGA, and a dementia friendly medical Green channel Approach (2MCGA). The decline of cognitive and physical function are determined by the change of Mini-Mental State Examination score, CDR and the sum of CDR box, as well as activities of daily living based on the Barthel Index. We also screened geriatric syndromes at baseline and 1 year later. Participants in the intervention group were older and had a lower educational level, lower body mass index, poor baseline activities of daily living function, lower visual impairment, and higher rates of hearing impairment, polypharmacy and risk of malnutrition. The residents receiving 2MCGA had lower baseline Mini-Mental State Examination scores, and higher CDR. For residents in Jia-Li Veterans Home, all cognitive measurements except Mini-Mental State Examination were significantly associated with delaying the decline of cognition after analyzing by multiple linear regression, and multivariate logistic regression also showed that patients living in the community was independently associated with a higher odds ratio for activities of daily living decline (3.180, 95% CI 1.384-7.308, P = 0.006). There are also more improvement in their baseline geriatric syndromes and suffered less from new geriatric syndromes, including falls, urinary

Magnetic resonance spectroscopic study of parkinsonism related to boxing.

OpenAIRE

Davie, C A; Pirtosek, Z; Barker, G J; Kingsley, D P; Miller, P H; Lees, A J

1995-01-01

Proton magnetic resonance spectroscopy, localised to the lentiform nucleus, was carried out in three ex-professional boxers who developed a parkinsonian syndrome, six patients with idiopathic Parkinson's disease, and six age matched controls. The three ex-boxers all showed a pronounced reduction in the absolute concentration of N-acetylaspartate compared with the patients with idiopathic Parkinson's disease and the control group. This reduction is likely to reflect neuronal loss occurring in ...

Rational pharmacological approaches for cognitive dysfunction and depression in Parkinson's disease.

Science.gov (United States)

Sandoval-Rincón, Maritza; Sáenz-Farret, Michel; Miguel-Puga, Adán; Micheli, Federico; Arias-Carrión, Oscar

2015-01-01

Parkinson's disease (PD) is not a single entity but rather a heterogeneous neurodegenerative disorder. The present study aims to conduct a critical systematic review of the literature to describe the main pharmacological strategies to treat cognitive dysfunction and major depressive disorder in PD patients. We performed a search of articles cited in PubMed from 2004 to 2014 using the following MeSH terms (Medical subject headings) "Parkinson disease"; "Delirium," "Dementia," "Amnestic," "Cognitive disorders," and "Parkinson disease"; "depression," "major depressive disorder," "drug therapy." We found a total of 71 studies related to pharmacological treatment in cognitive dysfunction and 279 studies for pharmacological treatment in major depressive disorder. After fulfillment of all the inclusion and exclusion criteria, 13 articles remained for cognitive dysfunction and 11 for major depressive disorder, which are presented and discussed in this study. Further research into non-motor symptoms of PD may provide insights into mechanisms of neurodegeneration, and provide better quality of life by using rational drugs.

PRKAG3 polymorphisms associated with sporadic Wolff-Parkinson-White syndrome among a Taiwanese population.

Science.gov (United States)

Weng, Ken-Pen; Yuh, Yeong-Seng; Huang, Shih-Hui; Hsiao, Hsiang-Chiang; Wu, Huang-Wei; Chien, Jen-Hung; Chen, Bo-Hau; Huang, Shih-Ming; Chien, Kuang-Jen; Ger, Luo-Ping

2016-12-01

The aim of this study was to investigate whether mutation in AMP-activated protein kinase (AMPK) subunit genes (PRKAG3-230) is associated with sporadic, isolated Wolff-Parkinson-White (WPW) syndrome. This study consisted of 87 patients with symptomatic WPW syndrome and 93 healthy controls. PRKAG3-230 genotypes were determined using real-time polymerase chain reaction assay. Genotype and allele frequencies of PRKAG3-230 between patients with WPW syndrome and healthy controls were ascertained using chi-square test or Fisher exact test when appropriate. PRKAG3-230 were genotyped in 87 patients (53 men and 34 women; age=24.4±18.0 years) with WPW syndrome and 93 healthy controls (57 men and 36 women; age=16.8±4.2 years). There were no significant differences between the two groups in terms of age and sex. The patients with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype [odds ratio (OR)=1.99, 95% confidence interval (CI)=1.01-3.89, p=0.045; OR=1.99, 95% CI=1.04-3.78, p=0.037, respectively]. The allelic types were not associated with the risk of WPW syndrome. The patients with manifest type with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype (OR=2.86, 95% CI=1.16-7.05, p=0.022; OR=2.84, 95% CI=1.19-6.80, p=0.019, respectively). The patients with right-side accessory pathways with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype (OR=3.07, 95% CI=1.25-7.51, p=0.014; OR=2.84, 95% CI=1.19-6.80, p=0.019, respectively). The allelic types were not associated with the risk of WPW types and locations. This study shows that PRKAG3-230 may be associated with sporadic WPW syndrome among a Taiwanese population. Further studies are warranted to elucidate the role of mutations in AMPK subunit genes other than PRKAG3-230 in sporadic WPW syndrome. Copyright © 2016. Published by Elsevier Taiwan LLC.

Prevalence of major depressive disorder and dementia in psychogeriatric outpatients.

Science.gov (United States)

Chinello, A; Grumelli, B; Perrone, C; Annoni, G

2007-01-01

The relationship between depression and dementia in the elderly has been widely investigated, but the real interplay between these variables is still not clear. This observational study highlights the influence of some basic variables, such as sex and age, in the development of dementia and major depression. It shows (i) the importance of sex in the age of onset of depression and dementia, (ii) the presence of two types of depressive syndrome, the first linked to the development of dementia, the second as reactive depression; (iii) the need for more attention to depressive symptoms in young-elderly men.

The prevalence of early repolarization in Wolff-Parkinson-White syndrome with a special reference to J waves and the effects of catheter ablation.

Science.gov (United States)

Yagihara, Nobue; Sato, Akinori; Iijima, Kenichi; Izumi, Daisuke; Furushima, Hiroshi; Watanabe, Hiroshi; Irie, Tadanobu; Kaneko, Yoshiaki; Kurabayashi, Masahiko; Chinushi, Masaomi; Satou, Masahito; Aizawa, Yoshifusa

2012-01-01

We determined the prevalence of J waves in the electrocardiograms (ECG) of 120 patients with Wolff-Parkinson-White syndrome in comparison with J-wave prevalence in a control group of 1936 men and women with comparable demographic and ECG characteristics and with normal atrioventricular conduction. J waves were present only during manifest preexcitation in 22 of 120 patients (18.3%), disappearing after catheter ablation and suggesting that J waves were associated with the presence of preexcitation. J waves were present in 19 (15.8%) of 120 patients only after ablation, apparently having been masked by early depolarization of the preexcited myocardial region, and in 22 patients (18.3%), J waves were not altered significantly by preexcitation. Thus, the overall J-wave prevalence was 52.5% (63/120) and, excluding those apparently due to preexcitation, 34.8% (41/120), both substantially higher than the prevalence (11.5%) in the control group (P Wolff-Parkinson-White syndrome and is influenced by manifest preexcitation. Copyright © 2012 Elsevier Inc. All rights reserved.

Parkinson's Disease: Leucine-Rich Repeat Kinase 2 and Autophagy, Intimate Enemies

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José M. Bravo-San Pedro

2012-01-01

Full Text Available Parkinson's disease is the second common neurodegenerative disorder, after Alzheimer's disease. It is a clinical syndrome characterized by loss of dopamine-generating cells in the substancia nigra, a region of the midbrain. The etiology of Parkinson's disease has long been through to involve both genetic and environmental factors. Mutations in the leucine-rich repeat kinase 2 gene cause late-onset Parkinson's disease with a clinical appearance indistinguishable from Parkinson's disease idiopathic. Autophagy is an intracellular catabolic mechanism whereby a cell recycles or degrades damage proteins and cytoplasmic organelles. This degradative process has been associated with cellular dysfunction in neurodegenerative processes including Parkinson's disease. We discuss the role of leucine-rich repeat kinase 2 in autophagy, and how the deregulations of this degradative mechanism in cells can be implicated in the Parkinson's disease etiology.

Rapid recovery from congestive heart failure following successful radiofrequency catheter ablation in a patient with late onset of Wolff-Parkinson-White syndrome.

Science.gov (United States)

Yodogawa, Kenji; Ono, Norihiko; Seino, Yoshihiko

2012-01-01

A 56-year-old man was admitted because of palpitations and dyspnea. A 12-lead electrocardiogram showed irregular wide QRS complex tachycardia with a slur at the initial portion of the QRS complex. He had preexisting long-standing persistent atrial fibrillation, but early excitation syndrome had never been noted. Chest X-ray showed heart enlargement and pulmonary congestion. He was diagnosed with late onset of Wolff-Parkinson-White syndrome, and congestive heart failure was probably caused by rapid ventricular response of atrial fibrillation through the accessory pathway. Emergency catheter ablation for the accessory pathway was undertaken, and heart failure was dramatically improved.

Post-cueing deficits with maintained cueing benefits in patients with Parkinson's disease dementia

Directory of Open Access Journals (Sweden)

Susanne eGräber

2014-11-01

Full Text Available In Parkinson’s disease (PD internal cueing mechanisms are impaired leading to symptoms such as like hypokinesia. However external cues can improve movement execution by using cortical resources. These cortical processes can be affected by cognitive decline in dementia.It is still unclear how dementia in PD influences external cueing. We investigated a group of 25 PD patients with dementia (PDD and 25 non-demented PD patients (PDnD matched by age, sex and disease duration in a simple reaction time (SRT task using an additional acoustic cue. PDD patients benefited from the additional cue in similar magnitude as did PDnD patients. However, withdrawal of the cue led to a significantly increased reaction time in the PDD group compared to the PDnD patients. Our results indicate that even PDD patients can benefit from strategies using external cue presentation but the process of cognitive worsening can reduce the effect when cues are withdrawn.

FTLD-TDP with motor neuron disease, visuospatial impairment and a progressive supranuclear palsy-like syndrome: broadening the clinical phenotype of TDP-43 proteinopathies. A report of three cases

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Holmerová Iva

2011-05-01

Full Text Available Abstract Background Frontotemporal lobar degeneration with ubiquitin and TDP-43 positive neuronal inclusions represents a novel entity (FTLD-TDP that may be associated with motor neuron disease (FTLD-MND; involvement of extrapyramidal and other systems has also been reported. Case presentation We present three cases with similar clinical symptoms, including Parkinsonism, supranuclear gaze palsy, visuospatial impairment and a behavioral variant of frontotemporal dementia, associated with either clinically possible or definite MND. Neuropathological examination revealed hallmarks of FTLD-TDP with major involvement of subcortical and, in particular, mesencephalic structures. These cases differed in onset and progression of clinical manifestations as well as distribution of histopathological changes in the brain and spinal cord. Two cases were sporadic, whereas the third case had a pathological variation in the progranulin gene 102 delC. Conclusions Association of a "progressive supranuclear palsy-like" syndrome with marked visuospatial impairment, motor neuron disease and early behavioral disturbances may represent a clinically distinct phenotype of FTLD-TDP. Our observations further support the concept that TDP-43 proteinopathies represent a spectrum of disorders, where preferential localization of pathogenetic inclusions and neuronal cell loss defines clinical phenotypes ranging from frontotemporal dementia with or without motor neuron disease, to corticobasal syndrome and to a progressive supranuclear palsy-like syndrome.

[Evaluation of the radiofrequency ablation effectiveness in patients with the Wolff-Parkinson-White syndrome].

Science.gov (United States)

Ushakov, I B; Ardashev, A V; Ardashev, V N; Voronkov, Iu I; Sharoĭko, M V; Akimova, O S

2012-01-01

A one-year prospective study involved 22 patients with the Wolff-Parkinson-White syndrome (WPW) and 20 healthy people. Means age of patients was 34.3 +/- 16.3 years. All 22 patients were successfully treated with radiofrequency ablation (RFA) of additional pathways. RFA effectiveness was evaluated with the help of clinical questionnaire, data of ECG, EchoCG, heart rate variability (HRV), frequency response and nonlinear dynamics. Cardiac rhythm disturbances were verified using Holter monitoring applied to all patients. Positive clinical effect was achieved in all the WPW patients, as RFA arrested cardiac arrhythmias completely. Holter monitoring did not register cardiac disturbances which points to high RFA effectiveness in WPW patients. HRV, frequency response and nonlinear dynamics reassumed their normal patterns.

Exome analysis of a family with Wolff-Parkinson-White syndrome identifies a novel disease locus.

Science.gov (United States)

Bowles, Neil E; Jou, Chuanchau J; Arrington, Cammon B; Kennedy, Brett J; Earl, Aubree; Matsunami, Norisada; Meyers, Lindsay L; Etheridge, Susan P; Saarel, Elizabeth V; Bleyl, Steven B; Yost, H Joseph; Yandell, Mark; Leppert, Mark F; Tristani-Firouzi, Martin; Gruber, Peter J

2015-12-01

Wolff-Parkinson-White (WPW) syndrome is a common cause of supraventricular tachycardia that carries a risk of sudden cardiac death. To date, mutations in only one gene, PRKAG2, which encodes the 5'-AMP-activated protein kinase subunit γ-2, have been identified as causative for WPW. DNA samples from five members of a family with WPW were analyzed by exome sequencing. We applied recently designed prioritization strategies (VAAST/pedigree VAAST) coupled with an ontology-based algorithm (Phevor) that reduced the number of potentially damaging variants to 10: a variant in KCNE2 previously associated with Long QT syndrome was also identified. Of these 11 variants, only MYH6 p.E1885K segregated with the WPW phenotype in all affected individuals and was absent in 10 unaffected family members. This variant was predicted to be damaging by in silico methods and is not present in the 1,000 genome and NHLBI exome sequencing project databases. Screening of a replication cohort of 47 unrelated WPW patients did not identify other likely causative variants in PRKAG2 or MYH6. MYH6 variants have been identified in patients with atrial septal defects, cardiomyopathies, and sick sinus syndrome. Our data highlight the pleiotropic nature of phenotypes associated with defects in this gene. © 2015 Wiley Periodicals, Inc.

Genetics Home Reference: Wolff-Parkinson-White syndrome

Science.gov (United States)

... Davis AM, Drago F, Janousek J, Klein GJ, Law IH, Morady FJ, Paul T, Perry JC, Sanatani S, Tanel RE. PACES/HRS expert consensus statement on the management of the asymptomatic young patient with a Wolff-Parkinson-White (WPW, ventricular preexcitation) electrocardiographic pattern: developed in ...

Three-dimensional surface display of brain perfusion with 123I-IMP in Parkinson's disease

International Nuclear Information System (INIS)

Tachibana, H.; Kawabata, K.; Tomino, Y.; Sugita, M.; Fukuchi, M.

1994-01-01

We reconstructed three-dimensional (3D) surface images from single-photon emission computed tomography (SPECT) data using N-isopropyl-p[ 123 I]-iodoamphetamine ( 123 I-IMP) in 27 patients with Parkinson's disease and 11 normal control subjects. The 3D reconstruction was performed using distance-shaded methods at threshold levels with an interval of 5% from 45-80%. Any area of decreased perfusion at each threshold level was visualised as a defect area by the algorithm. In nondemented patients with Parkinson's disease, perfusion defects were frequently found in the parietal cortex at a threshold value of 65%. In demented patients, perfusion defects were frequently seen at thresholds of 45-65%, and were more marked in the temporal and parietal cortex bilaterally. This suggests that dementia in Parkinson's disease is related to a reduction of perfusion in the temporoparietal cortex. (orig.)

Parametric imaging of experimentally simulated Wolff-Parkinson-White syndrome conduction abnormalities in dogs: a concise communication

Energy Technology Data Exchange (ETDEWEB)

Weismueller, P.H.; Henze, E.; Adam, W.E.; Roth, J.; Bitter, F.; Stauch, M.

1986-01-01

In order to test the diagnostic potential of phase analysis of radionuclide ventriculography (RNV) for localizing accessory bundles in Wolff-Parkinson-White (WPW) syndrome, 24 experimental runs were performed in three open chest instrumented dogs. After a baseline study, WPW syndrome was simulated by stimulation at seven different sites around the base of the ventricles, and RNV's were obtained. Subsequent data processing including Fourier transformation allowed the localization of the site of the first inward motion of the ventricles by an isophasic wave display. In sinus rhythm, the septum contracted first. During ectopic premature ventricular stimulation by triggering the atrial signal, the phase scan was altered only when the stimulus was applied earlier than 20 ms before the expected QRS complex during sinus rhythm. During stimulation with fixed frequency, only the left lateral positions of the premature stimulation were detected by phase analysis with a sensitivity of 86%. Neither the antero- or posteroseptal nor the right ventricular premature contraction pattern could be exactly localized.

Parametric imaging of experimentally simulated Wolff-Parkinson-White syndrome conduction abnormalities in dogs: a concise communication

International Nuclear Information System (INIS)

Weismueller, P.H.; Henze, E.; Adam, W.E.; Roth, J.; Bitter, F.; Stauch, M.

1986-01-01

In order to test the diagnostic potential of phase analysis of radionuclide ventriculography (RNV) for localizing accessory bundles in Wolff-Parkinson-White (WPW) syndrome, 24 experimental runs were performed in three open chest instrumented dogs. After a baseline study, WPW syndrome was simulated by stimulation at seven different sites around the base of the ventricles, and RNV's were obtained. Subsequent data processing including Fourier transformation allowed the localization of the site of the first inward motion of the ventricles by an isophasic wave display. In sinus rhythm, the septum contracted first. During ectopic premature ventricular stimulation by triggering the atrial signal, the phase scan was altered only when the stimulus was applied earlier than 20 ms before the expected QRS complex during sinus rhythm. During stimulation with fixed frequency, only the left lateral positions of the premature stimulation were detected by phase analysis with a sensitivity of 86%. Neither the antero- or posteroseptal nor the right ventricular premature contraction pattern could be exactly localized

[Care-Dependency in Parkinson's Disease: More Frequent than Assumed?].

Science.gov (United States)

Riedel, O

2015-06-01

Parkinson's disease (PD) increases the risk of care-dependency (CDP). While motor functions worsen continuously, the assignment of patients to CDP occurs categorically. It is unknown how many patients are already sufficiently severely impaired to be categorised as CDP yet do not have an officially acknowledged level of CDP. A random sample of 1,449 PD outpatients was clinically characterised by office-based neurologists, including impairments of activities of daily living (ADL with the Unified Parkinson's Disease Rating scale (UPDRS subscale II) as well as regarding the presence of dementia according to DSM-IV criteria and the Mini-Mental State Exam (MMSE). Depression was screened for with the Montgomery-Asberg Depression Rating Scale (MADRS). For each patient the officially acknowledged level of CDP was documented; for patients without official CDP level, the clinician appraised whether the patient was care-dependent anyhow. 266 patients (18.3%) were officially acknowledged as care-dependent, while n=121 patients (8.5%) were not, yet were appraised to be care-dependent according to the clinician. Compared to non-CDP patients, they differed on every measure considered. Compared to patients with an official CDP, their PD duration was significantly shorter (6.0 vs. 8.0 years, p<0.01) and they were less severely impaired in ADL (13.3 vs. 15.5, p<0.01). They did not differ regarding the rates of dementia (52.9 vs. 44.9%, p=0.203) or depression according to the MADRS (13.1 vs. 13.1, p=0.989). ADL impairments are the most important predictor for CDP while dementia and depression are not considered despite the impairments that are additionally caused by them. © Georg Thieme Verlag KG Stuttgart · New York.

Diagnostic evaluation of dementia in the secondary health care sector

DEFF Research Database (Denmark)

Phung, Thien Kieu Thi; Andersen, Birgitte Bo; Kessing, Lars Vedel

2009-01-01

BACKGROUND: We conducted a nationwide registry-based study of the quality of diagnostic evaluation for dementia in the secondary health care sector. METHOD: Two hundred patients were randomly selected from the patient population (4,682 patients) registered for the first time with a dementia...... diagnosis in the nationwide hospital registries during the last 6 months of 2003. Through medical record review, we evaluated the completeness of the work-up on which the dementia diagnosis was based, using evidence-based dementia guidelines as reference standards. RESULTS: Satisfactory or acceptable...... completion of the basic dementia work-up was documented in 51.3% of the patients. Only 11.5% of those with unsatisfactory work-up were referred to follow-up investigations. Dementia syndrome was confirmed in 88.5% of the cases, but correct subtypes were diagnosed in only 35.1%. CONCLUSION: The adherence...

Familial aggregation of Alzheimer's disease and related disorders: A collaborative re-analysis of case-control studies

NARCIS (Netherlands)

C.M. van Duijn (Cornelia); D.G. Clayton (David); V. Chandra; L. Fratiglioni (Laura); A.B. Graves; A. Heyman; A.F. Jorm; E. Kokmen (Emre); K. Kondo; J.A. Mortimer; W.A. Rocca; S.L. Shalat; H. Soininen; A. Hofman (Albert)

1991-01-01

textabstractCase-control studies of Alzheimer's disease were re-analysed to examine the association of Alzheimer's disease with family history in first degree relatives of dementia, Down's syndrome and Parkinson's disease. Overall, the relative risk of Alzheimer's disease for those with at least one

POLYMORPHISMS OF ENDOTHELIAL NITRIC OXIDE SYNTHASE GENE AS PREDICTORS OF WOLFF-PARKINSON-WHITE SYNDROME

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G. V. Matyushin

2017-01-01

Full Text Available Background. The discovery of new genetic predictors of cardiovascular diseases can be used in predicting and diagnosing latent forms of the disease. Wolff-Parkinson-White syndrome (WPW occurs in all age groups  and detected in 1-30 people per 10000, it manifests mainly in young age (on average 20 years, and the risk of sudden cardiac death is higher than in general population.Aim. To study the relationship of WPW syndrome with the polymorphism of endothelial nitric synthase gene (NOS3, and to identify genetic predictors of this syndrome.Material and methods. The study included 51 people with ECG proven WPW syndrome and 153 people with no cardiovascular disease. The patients were divided into subgroups according to sex: 21 women, 30 men. All patients underwent a standard cardiac examination (anamnesis, electrocardiography, echocardiography, bicycle ergometry, transesophageal electrical stimulation of the atria, Holter monitoring and blood was taken for molecular genetic testing of DNA.Results. The results showed a statistically significant prevalence of rare genotype 4b\\4b NOS3 gene in the control group of women (16.3%; р<0.05 compared with women from the main group, who did not have this genotype, while there was significant prevalence of genotype 4a\\4a in the main group of women (81.0%; р<0.05 compared with women from the control group.  In men this prevalence was not found.Conclusion. The presence of genotype 4b\\4b NOS3 gene reduces the likelihood of WPW syndrome and its symptoms in females. In men,  this prevalence is not found, presumably, in connection with some mechanisms of hormonal regulation. The results can be used in the genetic prediction of the course of the disease.

Successful ablation of a right atrium-axillary ventricular accessory pathway associated with Wolff-Parkinson-White syndrome.

Science.gov (United States)

Yuan, Yuan; Long, Deyong; Dong, Jianzeng; Tao, Ling; Ma, Changsheng

2017-12-01

We report a case of a patient with right axillary ventricular. Similar congenital anomaly of the right atrium was reported as "right appendage diverticulum or right atrial diverticulum." However, this independent chamber has its own annulus, synchronizes with the right ventricular, and generates large ventricular potential. Under the guidance of the CARTO mapping system (Biosense Webster, Diamond Bar, CA, USA), a right atrioventricular accessory pathway associated with type B Wolff-Parkinson-White syndrome was ablated successfully. This pathway was close to the annulus of the axillary ventricular. The patient remained free of arrhythmia at 1-year follow-up. © 2017 Wiley Periodicals, Inc.

Influence of apolipoprotein E genotype on senile dementia of the Alzheimer and Lewy body types. Significance for etiological theories of Alzheimer's disease.

OpenAIRE

Harrington, C. R.; Louwagie, J.; Rossau, R.; Vanmechelen, E.; Perry, R. H.; Perry, E. K.; Xuereb, J. H.; Roth, M.; Wischik, C. M.

1994-01-01

Alzheimer's disease (AD) is associated with an increased frequency of the apolipoprotein E type epsilon 4 allele. To address both the disease and the allele specificity of this association, we have examined the apolipoprotein E allele distribution in 255 elderly persons including those with autopsy-confirmed AD, senile dementia of the Lewy body type (SDLT), vascular dementia, Parkinson's disease (PD) or Huntington's disease and in nondemented controls either with or without coronary complicat...

Successful Radiofrequency Catheter Ablation for Wolff-Parkinson-White Syndrome Within the Neck of a Coronary Sinus Diverticulum

Science.gov (United States)

Jang, Sung-Won; Kim, Dong-Bin; Kwon, Bum-Jun; Cho, Eun-Joo; Shin, Woo-Seung; Kim, Ji-Hoon; Jin, Seung-Won; Oh, Yong-Seog; Lee, Man-Young; Kim, Jae-Hyung

2009-01-01

Posteroseptal accessory pathways are often associated with coronary sinus diverticula. These diverticula contain myocardial coats which serve as a bypass tract. We report a 54-year-old woman who underwent radiofrequency (RF) catheter ablation for Wolff-Parkinson-White (WPW) syndrome. The surface electrocardiography (ECG) demonstrated pre-excitation, indicating a posteroseptal accessory pathway. A catheter ablation via a transaortic approach failed to ablate the accessory pathway. Coronary sinus venography revealed the presence of a diverticulum near the ostium. An electrogram in the neck of the diverticulum showed the coronary sinus myocardial extension potential, which was successfully ablated by delivery of RF energy. PMID:19949625

The utility of neuroimaging in the management of dementia

Directory of Open Access Journals (Sweden)

Uduak E Williams

2015-01-01

Full Text Available Dementia is a syndrome of progressive dysfunction of two or more cognitive domains associated with impairment of activities of daily living. An understanding of the pathophysiology of dementia and its early diagnosis is important in the pursuit of possible disease modifying therapy for dementia. Neuroimaging has greatly transformed this field of research as its function has changed from a mere tool for diagnosing treatable causes of dementia to an instrument for pre-symptomatic diagnosis of dementia. This review focuses on the diagnostic utility of neuroimaging in the management of progressive dementias. Structural imaging techniques like computerized tomography scan and magnetic resonance imaging highlights the anatomical, structural and volumetric details of the brain; while functional imaging techniques such as positron emission tomography, arterial spin labeling, single photon emission computerized tomography and blood oxygen level-dependent functional magnetic resonance imaging focuses on chemistry, circulatory status and physiology of the different brain structures and regions.

Dementia in a retired world boxing champion: case report and literature review.

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Nowak, L A; Smith, G G; Reyes, P F

2009-01-01

Dementia in retired boxers, also referred to as "dementia pugilistica" (DP), is usually attributed to repeated concussive and subconcussive blows to the head. We report the case of a former world boxing champion whose progressive cognitive decline could be ascribed to DP, cerebral infarcts and Wernicke-Korsakoff syndrome. This case demonstrates that dementia in retired boxers may be caused and/or exacerbated by etiologic factors other than DP. We correlated the clinical features with the histochemical and immunohistochemical changes observed on autopsy brain material from a retired boxer, reviewed the literature on boxing-related dementia, and compared our findings with previous reports on DP. Neuropathologic examination revealed numerous neurofibrillary tangles (NFTs), rare neuritic plaques (NPs), multiple cerebral infarcts, fenestrated septum pellucidum, atrophic and gliotic mamillary bodies, and pale substantia nigra and locus ceruleus. Our neuropathologic data confirmed the notion that dementia in retired boxers could be due to several factors such as DP, multiple cerebral infarcts and Wernicke-Korsakoff syndrome. Our findings illustrate the need to comprehensively examine former boxers with dementia as well as carefully evaluate the neuropathologic changes that may cause or contribute to the patient's cognitive and behavioral symptoms. Such an approach is crucial in order to provide prompt and more definitive therapies.