WorldWideScience

Sample records for basal ganglia diseases

  1. Basal ganglia circuits changes in Parkinson's disease patients.

    Science.gov (United States)

    Wu, Tao; Wang, Jue; Wang, Chaodong; Hallett, Mark; Zang, Yufeng; Wu, Xiaoli; Chan, Piu

    2012-08-22

    Functional changes in basal ganglia circuitry are responsible for the major clinical features of Parkinson's disease (PD). Current models of basal ganglia circuitry can only partially explain the cardinal symptoms in PD. We used functional MRI to investigate the causal connectivity of basal ganglia networks from the substantia nigra pars compacta (SNc) in PD in the movement and resting state. In controls, SNc activity predicted increased activity in the supplementary motor area, the default mode network, and dorsolateral prefrontal cortex, but, in patients, activity predicted decreases in the same structures. The SNc had decreased connectivity with the striatum, globus pallidus, subthalamic nucleus, thalamus, supplementary motor area, dorsolateral prefrontal cortex, insula, default mode network, temporal lobe, cerebellum, and pons in patients compared to controls. Levodopa administration partially normalized the pattern of connectivity. Our findings show how the dopaminergic system exerts influences on widespread brain networks, including motor and cognitive networks. The pattern of basal ganglia network connectivity is abnormal in PD secondary to dopamine depletion, and is more deviant in more severe disease. Use of functional MRI with network analysis appears to be a useful method to demonstrate basal ganglia pathways in vivo in human subjects. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  2. Basal Ganglia Circuits as Targets for Neuromodulation in Parkinson Disease.

    Science.gov (United States)

    DeLong, Mahlon R; Wichmann, Thomas

    2015-11-01

    The revival of stereotactic surgery for Parkinson disease (PD) in the 1990s, with pallidotomy and then with high-frequency deep brain stimulation (DBS), has led to a renaissance in functional surgery for movement and other neuropsychiatric disorders. To examine the scientific foundations and rationale for the use of ablation and DBS for treatment of neurologic and psychiatric diseases, using PD as the primary example. A summary of the large body of relevant literature is presented on anatomy, physiology, pathophysiology, and functional surgery for PD and other basal ganglia disorders. The signs and symptoms of movement disorders appear to result largely from signature abnormalities in one of several parallel and largely segregated basal ganglia thalamocortical circuits (ie, the motor circuit). The available evidence suggests that the varied movement disorders resulting from dysfunction of this circuit result from propagated disruption of downstream network activity in the thalamus, cortex, and brainstem. Ablation and DBS act to free downstream networks to function more normally. The basal ganglia thalamocortical circuit may play a key role in the expression of disordered movement, and the basal ganglia-brainstem projections may play roles in akinesia and disturbances of gait. Efforts are under way to target circuit dysfunction in brain areas outside of the traditionally implicated basal ganglia thalamocortical system, in particular, the pedunculopontine nucleus, to address gait disorders that respond poorly to levodopa and conventional DBS targets. Deep brain stimulation is now the treatment of choice for many patients with advanced PD and other movement disorders. The success of DBS and other forms of neuromodulation for neuropsychiatric disorders is the result of the ability to modulate circuit activity in discrete functional domains within the basal ganglia circuitry with highly focused interventions, which spare uninvolved areas that are often disrupted with

  3. Neuroradiology of basal ganglia diseases in children and adolescents

    International Nuclear Information System (INIS)

    Savoiardo, M.; Passerini, A.; D'Incerti, L.

    1987-01-01

    Computerized tomography and NMR imaging findings observed in the diseases affecting the basal ganglia in childhood and adolescence are discussed. First the dystonic syndromes associated with hereditary neurologic disorders of probable metabolic degenerative origin are considered; then the non-hereditary dystonias caused by various intoxications or acute insults are briefly discussed. 26 refs.; 4 figs

  4. 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.

  5. Relationship between obsessive-compulsive disorders and diseases affecting primarily the basal ganglia

    Directory of Open Access Journals (Sweden)

    Maia Alex S. S. Freire

    1999-01-01

    Full Text Available Obsessive-compulsive disorder (OCD has been reported in association with some neurological diseases that affect the basal ganglia such as Tourette's syndrome, Sydenham's chorea, Parkinson's disease, and Huntington's disease. Furthermore, studies such as neuroimaging, suggest a role of the basal ganglia in the pathophysiology of OCD. The aim of this paper is to describe the association of OCD and several neurologic disorders affecting the basal ganglia, report the existing evidences of the role of the basal ganglia in the pathophysiology of OCD, and analyze the mechanisms probably involved in this pathophysiology.

  6. Levodopa Effect on Basal Ganglia Motor Circuit in Parkinson's Disease.

    Science.gov (United States)

    Gao, Lin-Lin; Zhang, Jia-Rong; Chan, Piu; Wu, Tao

    2017-01-01

    To investigate the effects of levodopa on the basal ganglia motor circuit (BGMC) in Parkinson's disease (PD). Thirty PD patients with asymmetrical bradykinesia and 30 control subjects were scanned using resting-state functional MRI. Functional connectivity of the BGMC was measured and compared before and after levodopa administration in patients with PD. The correlation between improvements in bradykinesia and changes in BGMC connectivity was examined. In the PD-off state (before medication), the posterior putamen and internal globus pallidus (GPi) had decreased connectivity while the subthalamic nucleus (STN) had enhanced connectivity within the BGMC relative to control subjects. Levodopa administration increased the connectivity of posterior putamen- and GPi-related networks but decreased the connectivity of STN-related networks. Improvements in bradykinesia were correlated with enhanced connectivity of the posterior putamen-cortical motor pathway and with decreased connectivity of the STN-thalamo-cortical motor pathway. In PD patients with asymmetrical bradykinesia, levodopa can partially normalize the connectivity of the BGMC with a larger effect on the more severely affected side. Moreover, the beneficial effect of levodopa on bradykinesia is associated with normalization of the striato-thalamo-cortical motor and STN-cortical motor pathways. Our findings inform the neural mechanism of levodopa treatment in PD. © 2016 John Wiley & Sons Ltd.

  7. Multiple Frequencies in the Basal Ganglia in Parkinson's Disease

    Directory of Open Access Journals (Sweden)

    Clare M. Davidson

    2015-01-01

    Full Text Available In recent years, the authors have developed what appears to be a very successful phenomenological model for analyzing the role of deep brain stimulation (DBS in alleviating the symptoms of Parkinson's disease. In this paper, we extend the scope of the model by using it to predict the generation of new frequencies from networks tuned to a specific frequency, or indeed not self-oscillatory at all. We have discussed two principal cases: firstly where the constituent systems are coupled in an excitatory-excitatory fashion, which we designate by ``+/+''; and secondly where the constituent systems are coupled in an excitatory-inhibitory fashion, which we designate ``+/-''. The model predicts that from a basic system tuned to tremor frequency we can generate an unlimited range of frequencies. We illustrate in particular, starting from systems which are initially non-oscillatory, that when the coupling coefficient exceeds a certain value, the system begins to oscillate at an amplitude which increases with the coupling strength. Another very interesting feature, which has been shown by colleagues of ours to arise through the coupling of complicated networks based on the physiology of the basal ganglia, can be illustrated by the root locus method which shows that increasing and decreasing frequencies of oscillation, existing simultaneously, have the property that their geometric mean remains substantially constant as the coupling strength is varied. We feel that with the present approach, we have provided another tool for understanding the existence and interaction of pathological oscillations which underlie, not only Parkinson's disease, but other conditions such as Tourette's syndrome, depression and epilepsy.

  8. The role of basal ganglia in language production: evidence from Parkinson's disease.

    Science.gov (United States)

    Macoir, Joël; Fossard, Marion; Mérette, Chantal; Langlois, Mélanie; Chantal, Sophie; Auclair-Ouellet, Noémie

    2013-01-01

    According to the dominant view in the literature, basal ganglia do not play a direct role in language but are involved in cognitive control required by linguistic and non-linguistic processing. In Parkinson's disease, basal ganglia impairment leads to motor symptoms and language deficits; those affecting the production of verbs have been frequently explored. According to a controversial theory, basal ganglia play a specific role in the conjugation of regular verbs as compared to irregular verbs. We report the results of 15 patients with Parkinson's disease in experimental conjugation tasks. They performed below healthy controls but their performance did not differ for regular and irregular verbs. These results confirm that basal ganglia are involved in language processing but do not play a specific role in verb production.

  9. Aberrant functional connectivity within the basal ganglia of patients with Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Michal Rolinski

    2015-01-01

    Full Text Available Resting state functional MRI (rs-fMRI has been previously shown to be a promising tool for the assessment of early Parkinson's disease (PD. In order to assess whether changes within the basal ganglia network (BGN are disease specific or relate to neurodegeneration generally, BGN connectivity was assessed in 32 patients with early PD, 19 healthy controls and 31 patients with Alzheimer's disease (AD. Voxel-wise comparisons demonstrated decreased connectivity within the basal ganglia of patients with PD, when compared to patients with AD and healthy controls. No significant changes within the BGN were seen in AD, when compared to healthy controls. Moreover, measures of functional connectivity extracted from regions within the basal ganglia were significantly lower in the PD group. Consistent with previous radiotracer studies, the greatest change when compared to the healthy control group was seen in the posterior putamen of PD subjects. When combined into a single component score, this method differentiated PD from AD and healthy control subjects, with a diagnostic accuracy of 81%. Rs-fMRI can be used to demonstrate the aberrant functional connectivity within the basal ganglia of patients with early PD. These changes are likely to be representative of patho-physiological basal ganglia dysfunction and are not associated with generalised neurodegeneration seen in AD. Further studies are necessary to ascertain whether this method is sensitive enough to detect basal ganglia dysfunction in prodromal PD, and its utility as a potential diagnostic biomarker for premotor and early motoric disease.

  10. Aberrant functional connectivity within the basal ganglia of patients with Parkinson's disease.

    Science.gov (United States)

    Rolinski, Michal; Griffanti, Ludovica; Szewczyk-Krolikowski, Konrad; Menke, Ricarda A L; Wilcock, Gordon K; Filippini, Nicola; Zamboni, Giovanna; Hu, Michele T M; Mackay, Clare E

    2015-01-01

    Resting state functional MRI (rs-fMRI) has been previously shown to be a promising tool for the assessment of early Parkinson's disease (PD). In order to assess whether changes within the basal ganglia network (BGN) are disease specific or relate to neurodegeneration generally, BGN connectivity was assessed in 32 patients with early PD, 19 healthy controls and 31 patients with Alzheimer's disease (AD). Voxel-wise comparisons demonstrated decreased connectivity within the basal ganglia of patients with PD, when compared to patients with AD and healthy controls. No significant changes within the BGN were seen in AD, when compared to healthy controls. Moreover, measures of functional connectivity extracted from regions within the basal ganglia were significantly lower in the PD group. Consistent with previous radiotracer studies, the greatest change when compared to the healthy control group was seen in the posterior putamen of PD subjects. When combined into a single component score, this method differentiated PD from AD and healthy control subjects, with a diagnostic accuracy of 81%. Rs-fMRI can be used to demonstrate the aberrant functional connectivity within the basal ganglia of patients with early PD. These changes are likely to be representative of patho-physiological basal ganglia dysfunction and are not associated with generalised neurodegeneration seen in AD. Further studies are necessary to ascertain whether this method is sensitive enough to detect basal ganglia dysfunction in prodromal PD, and its utility as a potential diagnostic biomarker for premotor and early motoric disease.

  11. Motor phenotype and magnetic resonance measures of basal ganglia iron levels in Parkinson's disease.

    Science.gov (United States)

    Bunzeck, Nico; Singh-Curry, Victoria; Eckart, Cindy; Weiskopf, Nikolaus; Perry, Richard J; Bain, Peter G; Düzel, Emrah; Husain, Masud

    2013-12-01

    In Parkinson's disease the degree of motor impairment can be classified with respect to tremor dominant and akinetic rigid features. While tremor dominance and akinetic rigidity might represent two ends of a continuum rather than discrete entities, it would be important to have non-invasive markers of any biological differences between them in vivo, to assess disease trajectories and response to treatment, as well as providing insights into the underlying mechanisms contributing to heterogeneity within the Parkinson's disease population. Here, we used magnetic resonance imaging to examine whether Parkinson's disease patients exhibit structural changes within the basal ganglia that might relate to motor phenotype. Specifically, we examined volumes of basal ganglia regions, as well as transverse relaxation rate (a putative marker of iron load) and magnetization transfer saturation (considered to index structural integrity) within these regions in 40 individuals. We found decreased volume and reduced magnetization transfer within the substantia nigra in Parkinson's disease patients compared to healthy controls. Importantly, there was a positive correlation between tremulous motor phenotype and transverse relaxation rate (reflecting iron load) within the putamen, caudate and thalamus. Our findings suggest that akinetic rigid and tremor dominant symptoms of Parkinson's disease might be differentiated on the basis of the transverse relaxation rate within specific basal ganglia structures. Moreover, they suggest that iron load within the basal ganglia makes an important contribution to motor phenotype, a key prognostic indicator of disease progression in Parkinson's disease. Copyright © 2013 The Authors. Published by Elsevier Ltd.. All rights reserved.

  12. Positron emission tomography and basal ganglia functions

    International Nuclear Information System (INIS)

    Kato, Motohiro; Otsuka, Makoto; Taniwaki, Koukyo; Hosokawa, Shinichi; Kuwabara, Yasuo; Ichiya, Yuichi

    1990-01-01

    With the advent of positron emission tomography (PET), studies on the human brain function and pathophysiology of brain damage have been extremely progressed. It is well-known that the basal ganglia plays an important role as one of the central nervous system involved in exercise regulation. More recently, the potential involvement of the basal ganglia in psychological processes, such as cognitive function, has been pointed out, receiving much attention. In spite of such a lot of studies, however, basal ganglia function remains unclear. This paper describes the relationships between PET findings and basal ganglia function. PET findings are discussed in relation to brain energy metabolism and striatal dopamine function. Pathophysiology of the basal ganglia are described in terms of the following diseases: Parkinson's disease, Parkinson's syndrome, progressive supranuclear palsy, Huntington's disease, and dystonia. Physiological backgrounds of the basal ganglia for PET images are also referred to. (N.K.) 75 refs

  13. Basal ganglia dysfunction in idiopathic REM sleep behaviour disorder parallels that in early Parkinson's disease.

    Science.gov (United States)

    Rolinski, Michal; Griffanti, Ludovica; Piccini, Paola; Roussakis, Andreas A; Szewczyk-Krolikowski, Konrad; Menke, Ricarda A; Quinnell, Timothy; Zaiwalla, Zenobia; Klein, Johannes C; Mackay, Clare E; Hu, Michele T M

    2016-08-01

    SEE POSTUMA DOI101093/AWW131 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Resting state functional magnetic resonance imaging dysfunction within the basal ganglia network is a feature of early Parkinson's disease and may be a diagnostic biomarker of basal ganglia dysfunction. Currently, it is unclear whether these changes are present in so-called idiopathic rapid eye movement sleep behaviour disorder, a condition associated with a high rate of future conversion to Parkinson's disease. In this study, we explore the utility of resting state functional magnetic resonance imaging to detect basal ganglia network dysfunction in rapid eye movement sleep behaviour disorder. We compare these data to a set of healthy control subjects, and to a set of patients with established early Parkinson's disease. Furthermore, we explore the relationship between resting state functional magnetic resonance imaging basal ganglia network dysfunction and loss of dopaminergic neurons assessed with dopamine transporter single photon emission computerized tomography, and perform morphometric analyses to assess grey matter loss. Twenty-six patients with polysomnographically-established rapid eye movement sleep behaviour disorder, 48 patients with Parkinson's disease and 23 healthy control subjects were included in this study. Resting state networks were isolated from task-free functional magnetic resonance imaging data using dual regression with a template derived from a separate cohort of 80 elderly healthy control participants. Resting state functional magnetic resonance imaging parameter estimates were extracted from the study subjects in the basal ganglia network. In addition, eight patients with rapid eye movement sleep behaviour disorder, 10 with Parkinson's disease and 10 control subjects received (123)I-ioflupane single photon emission computerized tomography. We tested for reduction of basal ganglia network connectivity, and for loss of tracer uptake in rapid eye movement sleep

  14. Interaction of synchronized dynamics in cortex and basal ganglia in Parkinson's disease.

    Science.gov (United States)

    Ahn, Sungwoo; Zauber, S Elizabeth; Worth, Robert M; Witt, Thomas; Rubchinsky, Leonid L

    2015-09-01

    Parkinson's disease pathophysiology is marked by increased oscillatory and synchronous activity in the beta frequency band in cortical and basal ganglia circuits. This study explores the functional connections between synchronized dynamics of cortical areas and synchronized dynamics of subcortical areas in Parkinson's disease. We simultaneously recorded neuronal units (spikes) and local field potentials (LFP) from subthalamic nucleus (STN) and electroencephalograms (EEGs) from the scalp in parkinsonian patients, and analysed the correlation between the time courses of the spike-LFP synchronization and inter-electrode EEG synchronization. We found the (non-invasively obtained) time course of the synchrony strength between EEG electrodes and the (invasively obtained) time course of the synchrony between spiking units and LFP in STN to be weakly, but significantly, correlated with each other. This correlation is largest for the bilateral motor EEG synchronization, followed by bilateral frontal EEG synchronization. Our observations suggest that there may be multiple functional modes by which the cortical and basal ganglia circuits interact with each other in Parkinson's disease: not only may synchronization be observed between some areas in cortex and the basal ganglia, but also synchronization within cortex and within basal ganglia may be related, suggesting potentially a more global functional interaction. More coherent dynamics in one brain region may modulate or activate the dynamics of another brain region in a more powerful way, causing correlations between changes in synchrony strength in the two regions. © 2015 Federation of European Neuroscience Societies and John Wiley & Sons Ltd.

  15. Activity of the basal ganglia in Parkinson's disease estimated by PET

    International Nuclear Information System (INIS)

    Ohye, Chihiro

    1995-01-01

    Positron emission tomographic (PET) studies on the local cerebral blood flow, oxygen metabolic rate, glucose metabolic rate in the basal ganglia of Parkinson's disease are reviewed. PET has demonstrated that blood flow was decreased in the cerebral cortex, especially the frontal region, of Parkinson's disease and that specific change in blood flow or metabolic rate in the basal ganglia was detected only in patients with hemi-parkinsonism. In authors' study on PET using 18 FDG in patients with tremor type and rigid type Parkinson's disease, changes in blood flow and metabolic rate were minimal at the basal ganglia level in tremor type patients, but cortical blood flow was decreased and metabolic rate was more elevated in the basal ganglia in rigid type patients. These findings were correlated with depth micro-recordings obtained by stereotactic pallidotomy. PET studies have also revealed that activity in the nerve terminal was decreased with decreasing dopamine and that dopamine (mainly D 2 ) activity was remarkably increased. PET studies with specific tracers are promising in providing more accurate information about functional state of living human brain with minimal invasion to patients. (N.K.)

  16. The role of inhibition in generating and controlling Parkinson's disease oscillations in the basal ganglia

    Directory of Open Access Journals (Sweden)

    Arvind eKumar

    2011-10-01

    Full Text Available Movement disorders in Parkinson's disease (PD are commonly associated with slow oscillations and increased synchrony of neuronal activity in the basal ganglia. The neural mechanisms underlying this dynamic network dysfunction, however, are only poorly understood. Here, we show that the strength of inhibitory inputs from striatum to globus pallidus external (GPe is a key parameter controlling oscillations in the basal ganglia. Specifically, the increase in striatal activity observed in PD is sufficient to unleash the oscillations in the basal ganglia. This finding allows us to propose a unified explanation for different phenomena: absence of oscillation in the healthy state of the basal ganglia, oscillations in dopamine-depleted state and quenching of oscillations under deep brain stimulation (DBS. These novel insights help us to better understand and optimize the function of DBS protocols. Furthermore, studying the model behaviour under transient increase of activity of the striatal neurons projecting to the indirect pathway, we are able to account for both motor impairment in PD patients and for reduced response inhibition in DBS implanted patients.

  17. Imaging insights into basal ganglia function, Parkinson’s disease, and dystonia

    OpenAIRE

    Stoessl, A. Jon; Lehericy, Stephane; Strafella, Antonio P.

    2014-01-01

    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, adva...

  18. Oscillatory activity in the human basal ganglia: more than just beta, more than just Parkinson's disease.

    Science.gov (United States)

    Alegre, Manuel; Valencia, Miguel

    2013-10-01

    The implantation of deep brain stimulators in different structures of the basal ganglia to treat neurological and psychiatric diseases has allowed the recording of local field potential activity in these structures. The analysis of these signals has helped our understanding of basal ganglia physiology in health and disease. However, there remain some major challenges and questions for the future. In a recent work, Tan et al. (Tan, H., Pogosyan, A., Anam, A., Foltynie, T., Limousin, P., Zrinzo, L., et al. 2013. Frequency specific activity in subthalamic nucleus correlates with hand bradykinesia in Parkinson's disease. Exp. Neurol. 240,122-129) take profit of these recordings to study the changes in subthalamic oscillatory activity during the hold and release phases of a grasping paradigm, and correlate the changes in different frequency bands with performance parameters. They found that beta activity was related to the release phase, while force maintenance related most to theta and gamma/HFO activity. There was no significant effect of the motor state of the patient on this latter association. These findings suggest that the alterations in the oscillatory activity of the basal ganglia in Parkinson's disease are not limited to the beta band, and they involve aspects different from movement preparation and initiation. Additionally, these results highlight the usefulness of the combination of well-designed paradigms with recordings in off and on motor states (in Parkinson's disease), or in different pathologies, in order to understand not only the pathophysiology of the diseases affecting the patients, but also the normal physiology of the basal ganglia. Copyright © 2013 Elsevier Inc. All rights reserved.

  19. Basal ganglia modulation of thalamocortical relay in Parkinson's disease and dystonia.

    Science.gov (United States)

    Guo, Yixin; Park, Choongseok; Worth, Robert M; Rubchinsky, Leonid L

    2013-01-01

    Basal ganglia dysfunction has being implied in both Parkinson's disease and dystonia. While these disorders probably involve different cellular and circuit pathologies within and beyond basal ganglia, there may be some shared neurophysiological pathways. For example, pallidotomy and pallidal Deep Brain Stimulation (DBS) are used in symptomatic treatment of both disorders. Both conditions are marked by alterations of rhythmicity of neural activity throughout basal ganglia-thalamocortical circuits. Increased synchronized oscillatory activity in beta band is characteristic of Parkinson's disease, while different frequency bands, theta and alpha, are involved in dystonia. We compare the effect of the activity of GPi, the output nuclei of the basal ganglia, on information processing in the downstream neural circuits of thalamus in Parkinson's disease and dystonia. We use a data-driven computational approach, a computational model of the thalamocortical (TC) cell modulated by experimentally recorded data, to study the differences and similarities of thalamic dynamics in dystonia and Parkinson's disease. Our analysis shows no substantial differences in TC relay between the two conditions. Our results suggest that, similar to Parkinson's disease, a disruption of thalamic processing could also be involved in dystonia. Moreover, the degree to which TC relay fidelity is impaired is approximately the same in both conditions. While Parkinson's disease and dystonia may have different pathologies and differ in the oscillatory content of neural discharge, our results suggest that the effect of patterning of pallidal discharge is similar in both conditions. Furthermore, these results suggest that the mechanisms of GPi DBS in dystonia may involve improvement of TC relay fidelity.

  20. PET activation in basal ganglia disorders: Parkinson's disease and dystonia

    International Nuclear Information System (INIS)

    Ceballos-Baumann, A.O.; Boecker, H.; Conrad, B.

    1997-01-01

    This article reviews PET activation studies with performance of different motor paradigms (joy-stick movements, imagination of movement, writing) in patients with movement disorders. The focus will be on Parkinson's disease (PD) and dystonia. PET findings will be related to clinical and electrophysiological observations. PET activation studies before and after therapeutic interventions such as pallidotomy in Parkinson's disease and botulinum toxin in writer's cramp are described. The contribution of PET activation studies to the understanding of the pathophysiology of dystonia and PD is discussed. (orig.) [de

  1. Crossed cerebellar and uncrossed basal ganglia and thalamic diaschisis in Alzheimer's disease

    International Nuclear Information System (INIS)

    Akiyama, H.; Harrop, R.; McGeer, P.L.; Peppard, R.; McGeer, E.G.

    1989-01-01

    We detected crossed cerebellar as well as uncrossed basal ganglia and thalamic diaschisis in Alzheimer's disease by positron emission tomography (PET) using 18 F-fluorodeoxyglucose. We studied a series of 26 consecutive, clinically diagnosed Alzheimer cases, including 6 proven by later autopsy, and compared them with 9 age-matched controls. We calculated asymmetry indices (AIs) of cerebral metabolic rate for matched left-right regions of interest (ROIs) and determined the extent of diaschisis by correlative analyses. For the Alzheimer group, we found cerebellar AIs correlated negatively, and thalamic AIs positively, with those of the cerebral hemisphere and frontal, temporal, parietal, and angular cortices, while basal ganglia AIs correlated positively with frontal cortical AIs. The only significant correlation of AIs for normal subjects was between the thalamus and cerebral hemisphere. These data indicate that PET is a sensitive technique for detecting diaschisis

  2. Dopamine-transporter SPECT and Dopamine-D2-receptor SPECT in basal ganglia diseases

    International Nuclear Information System (INIS)

    Hesse, S.; Barthel, H.; Seese, A.; Sabri, O.

    2007-01-01

    The basal ganglia comprise a group of subcortical nuclei, which are essential for motor control. Dysfunction of these areas, especially in dopaminergic transmission, results in disordered movement and neurological diseases such as Parkinson's disease, Wilson's disease, or Huntington disease. Positron emission tomography and single photon emission computed tomography (SPECT) have enhanced the understanding of the underlying pathophysiology, but they much more contribute to the early differential diagnosis of patients suffering from Parkinsonian syndrome in routine care. The present article provides dopamine transporter and D 2 receptor SPECT findings in selected movement disorders. (orig.)

  3. A neural mass model of basal ganglia nuclei simulates pathological beta rhythm in Parkinson's disease

    Science.gov (United States)

    Liu, Fei; Wang, Jiang; Liu, Chen; Li, Huiyan; Deng, Bin; Fietkiewicz, Chris; Loparo, Kenneth A.

    2016-12-01

    An increase in beta oscillations within the basal ganglia nuclei has been shown to be associated with movement disorder, such as Parkinson's disease. The motor cortex and an excitatory-inhibitory neuronal network composed of the subthalamic nucleus (STN) and the external globus pallidus (GPe) are thought to play an important role in the generation of these oscillations. In this paper, we propose a neuron mass model of the basal ganglia on the population level that reproduces the Parkinsonian oscillations in a reciprocal excitatory-inhibitory network. Moreover, it is shown that the generation and frequency of these pathological beta oscillations are varied by the coupling strength and the intrinsic characteristics of the basal ganglia. Simulation results reveal that increase of the coupling strength induces the generation of the beta oscillation, as well as enhances the oscillation frequency. However, for the intrinsic properties of each nucleus in the excitatory-inhibitory network, the STN primarily influences the generation of the beta oscillation while the GPe mainly determines its frequency. Interestingly, describing function analysis applied on this model theoretically explains the mechanism of pathological beta oscillations.

  4. The role of the basal ganglia in learning and memory: Insight from Parkinson's disease

    Science.gov (United States)

    2013-01-01

    It has long been known that memory is not a single process. Rather, there are different kinds of memory that are supported by distinct neural systems. This idea stemmed from early findings of dissociable patterns of memory impairments in patients with selective damage to different brain regions. These studies highlighted the role of the basal ganglia in non-declarative memory, such as procedural or habit learning, contrasting it with the known role of the medial temporal lobes in declarative memory. In recent years, major advances across multiple areas of neuroscience have revealed an important role for the basal ganglia in motivation and decision making. These findings have led to new discoveries about the role of the basal ganglia in learning and highlighted the essential role of dopamine in specific forms of learning. Here we review these recent advances with an emphasis on novel discoveries from studies of learning in patients with Parkinson's disease. We discuss how these findings promote the development of current theories away from accounts that emphasize the verbalizability of the contents of memory and towards a focus on the specific computations carried out by distinct brain regions. Finally, we discuss new challenges that arise in the face of accumulating evidence for dynamic and interconnected memory systems that jointly contribute to learning. PMID:21945835

  5. Imaging insights into basal ganglia function, Parkinson’s disease, and dystonia

    Science.gov (United States)

    Stoessl, A. Jon; Lehericy, Stephane; Strafella, Antonio P.

    2015-01-01

    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. PMID:24954673

  6. Balancing the Basal Ganglia Circuitry: A Possible New Role for Dopamine D2 Receptors in Health and Disease

    OpenAIRE

    Cazorla, Maxime; Kang, Un Jung; Kellendonk, Christoph

    2015-01-01

    Current therapies for treating movement disorders such as Parkinson’s disease are effective but limited by undesirable and intractable side effects. Developing more effective therapies will require better understanding of what causes basal ganglia dys-regulation and why medication-induced side effects develop. Although basal ganglia have been extensively studied in the last decades, its circuit anatomy is very complex, and significant controversy exists as to how the interplay of different ba...

  7. Microstructural Changes within the Basal Ganglia Differ between Parkinson Disease Subtypes.

    Science.gov (United States)

    Nagae, Lidia M; Honce, Justin M; Tanabe, Jody; Shelton, Erika; Sillau, Stefan H; Berman, Brian D

    2016-01-01

    Diffusion tensor imaging (DTI) of the substantia nigra has shown promise in detecting and quantifying neurodegeneration in Parkinson disease (PD). It remains unknown, however, whether differences in microstructural changes within the basal ganglia underlie PD motor subtypes. We investigated microstructural changes within the basal ganglia of mild to moderately affected PD patients using DTI and sought to determine if microstructural changes differ between the tremor dominant (TD) and postural instability/gait difficulty (PIGD) subtypes. Fractional anisotropy, mean diffusivity, radial, and axial diffusivity were obtained from bilateral caudate, putamen, globus pallidus, and substantia nigra of 21 PD patients (12 TD and 9 PIGD) and 20 age-matched healthy controls. T-tests and ANOVA methods were used to compare PD patients, subtypes, and controls, and Spearman correlations tested for relationships between DTI and clinical measures. We found our cohort of PD patients had reduced fractional anisotropy within the substantia nigra and increased mean and radial diffusivity within the substantia nigra and globus pallidus compared to controls, and that changes within those structures were largely driven by the PIGD subtype. Across all PD patients fractional anisotropy within the substantia nigra correlated with disease stage, while in PIGD patients increased diffusivity within the globus pallidus correlated with disease stage and motor severity. We conclude that PIGD patients have more severely affected microstructural changes within the substantia nigra compared to TD, and that microstructural changes within the globus pallidus may be particularly relevant for the manifestation of the PIGD subtype.

  8. The basal ganglia cholinergic neurochemistry of progressive supranuclear palsy and other neurodegenerative diseases.

    Science.gov (United States)

    Warren, N M; Piggott, M A; Lees, A J; Burn, D J

    2007-06-01

    Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disorder involving motor and cognitive dysfunction. Currently, there is no effective treatment either for symptomatic relief or disease modification. This relates, in part, to a lack of knowledge of the underlying neurochemical abnormalities, including cholinergic receptor status in the basal ganglia. To measure muscarinic M2 and M4 receptors in the basal ganglia in PSP. The muscarinic M2 (presynaptic) and M4 (postsynaptic) receptors in the striatum, pallidum and adjacent insular cortex were autoradiographically measured in pathologically confirmed cases of PSP (n = 18), and compared with cases of Lewy body dementias (LBDs; n = 45), Alzheimer's disease (AD; n = 39) and controls (n = 50). In cases of PSP, there was a reduction in M2 and M4 receptors in the posterior caudate and putamen compared to controls, but no significant changes in the pallidum. Cases with AD showed lower M2 receptors in the posterior striatum. Groups with LBD and AD showed higher M2 binding in the insular cortex compared with controls. The results suggest loss of posterior striatal cholinergic interneurones in PSP, and reduction in medium spiny projection neurones bearing M4 receptors. These results should be taken in the context of more widespread pathology in PSP, but may have implications for future trials of cholinergic treatments.

  9. Basal ganglia neuronal activity during scanning eye movements in Parkinson's disease.

    Directory of Open Access Journals (Sweden)

    Tomáš Sieger

    Full Text Available The oculomotor role of the basal ganglia has been supported by extensive evidence, although their role in scanning eye movements is poorly understood. Nineteen Parkinsońs disease patients, which underwent implantation of deep brain stimulation electrodes, were investigated with simultaneous intraoperative microelectrode recordings and single channel electrooculography in a scanning eye movement task by viewing a series of colored pictures selected from the International Affective Picture System. Four patients additionally underwent a visually guided saccade task. Microelectrode recordings were analyzed selectively from the subthalamic nucleus, substantia nigra pars reticulata and from the globus pallidus by the WaveClus program which allowed for detection and sorting of individual neurons. The relationship between neuronal firing rate and eye movements was studied by crosscorrelation analysis. Out of 183 neurons that were detected, 130 were found in the subthalamic nucleus, 30 in the substantia nigra and 23 in the globus pallidus. Twenty percent of the neurons in each of these structures showed eye movement-related activity. Neurons related to scanning eye movements were mostly unrelated to the visually guided saccades. We conclude that a relatively large number of basal ganglia neurons are involved in eye motion control. Surprisingly, neurons related to scanning eye movements differed from neurons activated during saccades suggesting functional specialization and segregation of both systems for eye movement control.

  10. Functional imaging of the cerebellum and basal ganglia during predictive motor timing in early Parkinson's disease.

    Science.gov (United States)

    Husárová, Ivica; Lungu, Ovidiu V; Mareček, Radek; Mikl, Michal; Gescheidt, Tomáš; Krupa, Petr; Bareš, Martin

    2014-01-01

    The basal ganglia and the cerebellum have both emerged as important structures involved in the processing of temporal information. We examined the roles of the cerebellum and striatum in predictive motor timing during a target interception task in healthy individuals (HC group; n = 21) and in patients with early Parkinson's disease (early stage PD group; n = 20) using functional magnetic resonance imaging. Despite having similar hit ratios, the PD failed more often than the HC to postpone their actions until the right moment and to adapt their behavior from one trial to the next. We found more activation in the right cerebellar lobule VI in HC than in early stage PD during successful trials. Successful trial-by-trial adjustments were associated with higher activity in the right putamen and lobule VI of the cerebellum in HC. We conclude that both the cerebellum and striatum are involved in predictive motor timing tasks. The cerebellar activity is associated exclusively with the postponement of action until the right moment, whereas both the cerebellum and striatum are needed for successful adaptation of motor actions from one trial to the next. We found a general ''hypoactivation'' of basal ganglia and cerebellum in early stage PD relative to HC, indicating that even in early stages of the PD there could be functional perturbations in the motor system beyond striatum. Copyright © 2011 by the American Society of Neuroimaging.

  11. Basal ganglia neuronal activity during scanning eye movements in Parkinson's disease.

    Science.gov (United States)

    Sieger, Tomáš; Bonnet, Cecilia; Serranová, Tereza; Wild, Jiří; Novák, Daniel; Růžička, Filip; Urgošík, Dušan; Růžička, Evžen; Gaymard, Bertrand; Jech, Robert

    2013-01-01

    The oculomotor role of the basal ganglia has been supported by extensive evidence, although their role in scanning eye movements is poorly understood. Nineteen Parkinsońs disease patients, which underwent implantation of deep brain stimulation electrodes, were investigated with simultaneous intraoperative microelectrode recordings and single channel electrooculography in a scanning eye movement task by viewing a series of colored pictures selected from the International Affective Picture System. Four patients additionally underwent a visually guided saccade task. Microelectrode recordings were analyzed selectively from the subthalamic nucleus, substantia nigra pars reticulata and from the globus pallidus by the WaveClus program which allowed for detection and sorting of individual neurons. The relationship between neuronal firing rate and eye movements was studied by crosscorrelation analysis. Out of 183 neurons that were detected, 130 were found in the subthalamic nucleus, 30 in the substantia nigra and 23 in the globus pallidus. Twenty percent of the neurons in each of these structures showed eye movement-related activity. Neurons related to scanning eye movements were mostly unrelated to the visually guided saccades. We conclude that a relatively large number of basal ganglia neurons are involved in eye motion control. Surprisingly, neurons related to scanning eye movements differed from neurons activated during saccades suggesting functional specialization and segregation of both systems for eye movement control.

  12. Structural findings in the basal ganglia in genetically determined and idiopathic Parkinson's disease

    DEFF Research Database (Denmark)

    Reetz, Kathrin; Gaser, Christian; Klein, Christine

    2009-01-01

    A bilateral compensatory increase of basal ganglia (BG) gray matter value (GMV) was recently demonstrated in asymptomatic Parkin mutation carriers, who likely have an increased risk to develop Parkinson's disease (PD). We hypothesized BG morphological changes in symptomatic Parkin mutation carriers...... (sPARKIN-MC) and idiopathic PD patients (iPD) after the occurrence of PD symptoms, reflecting the breakdown of compensatory mechanisms. Nine sPARKIN-MC, 14 iPD, and 24 controls were studied clinically and with voxel-based morphometry. Analysis of variance revealed mainly BG decrease of GMV in s...... manifest. Simple regression analyses with the UPDRS-III and disease duration score revealed a distinct more bilateral linear decrease of BG GMV in sPARKIN-MC than in iPD that may correspond to previous findings showing a symmetric reduction in putaminal (18)F-DOPA-uptake and bilateral manifestation...

  13. Bilateral calcifications in the basal ganglia, and frontal and parietal lobes of a patient with coeliac disease

    Energy Technology Data Exchange (ETDEWEB)

    Yildirim, Selman Vefa; Tiker, Filiz; Cengiz, Nurcan [Adana Hospital, Department of Paediatrics, Baskent University Medical Faculty, Seyhan-Adana (Turkey); Barutcu, Ozlem [Fatih University Medical Faculty, Radiology Department, Ankara (Turkey)

    2005-07-01

    Previous authors have described a specific syndrome of coeliac disease, bilateral cerebral calcifications and epileptic seizures. We report a 4-year-old boy with coeliac disease who had bilateral calcifications in the basal ganglia and frontal and parietal lobes, but did not exhibit epileptic seizures. (orig.)

  14. Basal ganglia dysfunction in idiopathic REM sleep behaviour disorder parallels that in early Parkinson’s disease

    Science.gov (United States)

    Rolinski, Michal; Griffanti, Ludovica; Piccini, Paola; Roussakis, Andreas A.; Szewczyk-Krolikowski, Konrad; Menke, Ricarda A.; Quinnell, Timothy; Zaiwalla, Zenobia; Klein, Johannes C.; Mackay, Clare E.

    2016-01-01

    Abstract See Postuma (doi:10.1093/aww131) for a scientific commentary on this article. Resting state functional magnetic resonance imaging dysfunction within the basal ganglia network is a feature of early Parkinson’s disease and may be a diagnostic biomarker of basal ganglia dysfunction. Currently, it is unclear whether these changes are present in so-called idiopathic rapid eye movement sleep behaviour disorder, a condition associated with a high rate of future conversion to Parkinson’s disease. In this study, we explore the utility of resting state functional magnetic resonance imaging to detect basal ganglia network dysfunction in rapid eye movement sleep behaviour disorder. We compare these data to a set of healthy control subjects, and to a set of patients with established early Parkinson’s disease. Furthermore, we explore the relationship between resting state functional magnetic resonance imaging basal ganglia network dysfunction and loss of dopaminergic neurons assessed with dopamine transporter single photon emission computerized tomography, and perform morphometric analyses to assess grey matter loss. Twenty-six patients with polysomnographically-established rapid eye movement sleep behaviour disorder, 48 patients with Parkinson’s disease and 23 healthy control subjects were included in this study. Resting state networks were isolated from task-free functional magnetic resonance imaging data using dual regression with a template derived from a separate cohort of 80 elderly healthy control participants. Resting state functional magnetic resonance imaging parameter estimates were extracted from the study subjects in the basal ganglia network. In addition, eight patients with rapid eye movement sleep behaviour disorder, 10 with Parkinson’s disease and 10 control subjects received 123I-ioflupane single photon emission computerized tomography. We tested for reduction of basal ganglia network connectivity, and for loss of tracer uptake in rapid eye

  15. Basal ganglia dysfunction

    Science.gov (United States)

    ... degenerate) Multiple system atrophy (widespread nervous system disorder) Parkinson disease Progressive supranuclear palsy (movement disorder from damage to certain nerve cells in the brain) Wilson disease (disorder causing too much copper in the body's tissues)

  16. Task-Rest Modulation of Basal Ganglia Connectivity in Mild to Moderate Parkinson’s Disease

    Science.gov (United States)

    Müller-Oehring, Eva M.; Sullivan, Edith V.; Pfefferbaum, Adolf; Huang, Neng C.; Poston, Kathleen L.; Bronte-Stewart, Helen M.; Schulte, Tilman

    2014-01-01

    Parkinson’s disease (PD) is associated with abnormal synchronization in basal ganglia-thalamo-cortical loops. We tested whether early PD patients without demonstrable cognitive impairment exhibit abnormal modulation of functional connectivity at rest, while engaged in a task, or both. PD and healthy controls underwent two functional MRI scans: a resting-state scan and a Stroop Match-to-Sample task scan. Rest-task modulation of basal ganglia (BG) connectivity was tested using seed-to-voxel connectivity analysis with task and rest time series as conditions. Despite substantial overlap of BG–cortical connectivity patterns in both groups, connectivity differences between groups had clinical and behavioral correlates. During rest, stronger putamen–medial parietal and pallidum–occipital connectivity in PD than controls was associated with worse task performance and more severe PD symptoms suggesting that abnormalities in resting-state connectivity denote neural network dedifferentiation. During the executive task, PD patients showed weaker BG-cortical connectivity than controls, i.e., between caudate–supramarginal gyrus and pallidum–inferior prefrontal regions, that was related to more severe PD symptoms and worse task performance. Yet, task processing also evoked stronger striatal–cortical connectivity, specifically between caudate–prefrontal, caudate–precuneus, and putamen–motor/premotor regions in PD relative to controls, which was related to less severe PD symptoms and better performance on the Stroop task. Thus, stronger task-evoked striatal connectivity in PD demonstrated compensatory neural network enhancement to meet task demands and improve performance levels. fMRI-based network analysis revealed that despite resting-state BG network compromise in PD, BG connectivity to prefrontal, premotor, and precuneus regions can be adequately invoked during executive control demands enabling near normal task performance. PMID:25280970

  17. Task-rest modulation of basal ganglia connectivity in mild to moderate Parkinson's disease.

    Science.gov (United States)

    Müller-Oehring, Eva M; Sullivan, Edith V; Pfefferbaum, Adolf; Huang, Neng C; Poston, Kathleen L; Bronte-Stewart, Helen M; Schulte, Tilman

    2015-09-01

    Parkinson's disease (PD) is associated with abnormal synchronization in basal ganglia-thalamo-cortical loops. We tested whether early PD patients without demonstrable cognitive impairment exhibit abnormal modulation of functional connectivity at rest, while engaged in a task, or both. PD and healthy controls underwent two functional MRI scans: a resting-state scan and a Stroop Match-to-Sample task scan. Rest-task modulation of basal ganglia (BG) connectivity was tested using seed-to-voxel connectivity analysis with task and rest time series as conditions. Despite substantial overlap of BG-cortical connectivity patterns in both groups, connectivity differences between groups had clinical and behavioral correlates. During rest, stronger putamen-medial parietal and pallidum-occipital connectivity in PD than controls was associated with worse task performance and more severe PD symptoms suggesting that abnormalities in resting-state connectivity denote neural network dedifferentiation. During the executive task, PD patients showed weaker BG-cortical connectivity than controls, i.e., between caudate-supramarginal gyrus and pallidum-inferior prefrontal regions, that was related to more severe PD symptoms and worse task performance. Yet, task processing also evoked stronger striatal-cortical connectivity, specifically between caudate-prefrontal, caudate-precuneus, and putamen-motor/premotor regions in PD relative to controls, which was related to less severe PD symptoms and better performance on the Stroop task. Thus, stronger task-evoked striatal connectivity in PD demonstrated compensatory neural network enhancement to meet task demands and improve performance levels. fMRI-based network analysis revealed that despite resting-state BG network compromise in PD, BG connectivity to prefrontal, premotor, and precuneus regions can be adequately invoked during executive control demands enabling near normal task performance.

  18. The Role of the Basal Ganglia in Implicit Contextual Learning: A Study of Parkinson's Disease

    Science.gov (United States)

    van Asselen, Marieke; Almeida, Ines; Andre, Rui; Januario, Cristina; Goncalves, Antonio Freire; Castelo-Branco, Miguel

    2009-01-01

    Implicit contextual learning refers to the ability to memorize contextual information from our environment. This contextual information can then be used to guide our attention to a specific location. Although the medial temporal lobe is important for this type of learning, the basal ganglia might also be involved considering its role in many…

  19. Basal Ganglia, Dopamine and Temporal Processing: Performance on Three Timing Tasks on and off Medication in Parkinson's Disease

    Science.gov (United States)

    Jones, Catherine R. G.; Malone, Tim J. L.; Dirnberger, Georg; Edwards, Mark; Jahanshahi, Marjan

    2008-01-01

    A pervasive hypothesis in the timing literature is that temporal processing in the milliseconds and seconds range engages the basal ganglia and is modulated by dopamine. This hypothesis was investigated by testing 12 patients with Parkinson's disease (PD), both "on" and "off" dopaminergic medication, and 20 healthy controls on three timing tasks.…

  20. Review: electrophysiology of basal ganglia and cortex in models of Parkinson disease.

    Science.gov (United States)

    Ellens, Damien J; Leventhal, Daniel K

    2013-01-01

    Incomplete understanding of the systems-level pathophysiology of Parkinson Disease (PD) remains a significant barrier to improving its treatment. Substantial progress has been made, however, due to the availability of neurotoxins that selectively target monoaminergic (in particular, dopaminergic) neurons. This review discusses the in vivo electrophysiology of basal ganglia (BG), thalamic, and cortical regions after dopamine-depleting lesions. These include firing rate changes, neuronal burst-firing, neuronal oscillations, and neuronal synchrony that result from a combination of local microanatomic changes and network-level interactions. While much is known of the clinical and electrophysiological phenomenology of dopamine loss, a critical gap in our conception of PD pathophysiology is the link between them. We discuss potential mechanisms by which these systems-level electrophysiological changes may emerge, as well as how they may relate to clinical parkinsonism. Proposals for an updated understanding of BG function are reviewed, with an emphasis on how emerging frameworks will guide future research into the pathophysiology and treatment of PD.

  1. Subclinical visuospatial impairment in Parkinson's disease: the role of basal ganglia and limbic system

    Directory of Open Access Journals (Sweden)

    Stefano eCaproni

    2014-08-01

    Full Text Available BACKGROUND: Visual perception deficits are a recurrent manifestation in Parkinson's disease (PD. Recently, structural abnormalities of fronto-parietal areas and subcortical regions, implicated in visual stimuli analysis, have been observed in PD patients with cognitive decline and visual hallucinations. The aim of the present study was to investigate the salient aspects of visual perception in cognitively unimpaired PD patients. METHODS: Eleven right-handed non-demented right-sided onset PD patients without visuospatial impairment or hallucinations and eleven healthy controls were studied with fMRI while performing a specific visuoperceptual/visuospatial paradigm that allowed to highlight the specific process underlying visuospatial judgment. RESULTS: Significant changes in both cortical areas and subcortical regions involved in visual stimuli processing were observed. In particular, PD patients showed a reduced activation for the right insula, left putamen, bilateral caudate and right hippocampus, as well as an over-activation of the right dorso-lateral prefrontal and of the posterior parietal cortices, particularly in the right hemisphere. CONCLUSIONS: We found that both loss of efficiency and compensatory mechanisms occur in PD patients, providing further insight into the pathophysiological role of the functional alterations of basal ganglia and limbic structures in the impairment of visuoperceptual and visuospatial functions observed in PD.

  2. Subclinical Visuospatial Impairment in Parkinson’s Disease: The Role of Basal Ganglia and Limbic System

    Science.gov (United States)

    Caproni, Stefano; Muti, Marco; Di Renzo, Antonio; Principi, Massimo; Caputo, Nevia; Calabresi, Paolo; Tambasco, Nicola

    2014-01-01

    Background: Visual perception deficits are a recurrent manifestation in Parkinson’s disease (PD). Recently, structural abnormalities of fronto-parietal areas and subcortical regions, implicated in visual stimuli analysis, have been observed in PD patients with cognitive decline and visual hallucinations. The aim of the present study was to investigate the salient aspects of visual perception in cognitively unimpaired PD patients. Methods: Eleven right-handed non-demented right-sided onset PD patients without visuospatial impairment or hallucinations and 11 healthy controls were studied with functional magnetic resonance imaging while performing a specific visuoperceptual/visuospatial paradigm that allowed to highlight the specific process underlying visuospatial judgment. Results: Significant changes in both cortical areas and subcortical regions involved in visual stimuli processing were observed. In particular, PD patients showed a reduced activation for the right insula, left putamen, bilateral caudate, and right hippocampus, as well as an over-activation of the right dorso-lateral prefrontal and of the posterior parietal cortices, particularly in the right hemisphere. Conclusions: We found that both loss of efficiency and compensatory mechanisms occur in PD patients, providing further insight into the pathophysiological role of the functional alterations of basal ganglia and limbic structures in the impairment of visuoperceptual and visuospatial functions observed in PD. PMID:25157239

  3. Survey of 37 cases with basal ganglia calcification. Relationship to CT-scan findings, underlying diseases, and epilepsy

    Energy Technology Data Exchange (ETDEWEB)

    Ogata, Akira; Ishida, Shiro; Wada, Toyoji (National Epilepsy Center, Shizuoka Higashi Hospital (Japan))

    1984-11-01

    Of 5,987 patients (5,196 epileptic patients and 791 non-epileptic patients) undergoing CT-scan, calcification of the bilateral basal ganglia was detected in 28 epileptic patients and 9 non-epileptic patients. Relationship among CT-scan findings, underlying diseases and epilepsy was studied. CT-scan findings could be classified into the localized type in which calcification was limited to the globus pallidus in 33 patients and diffuse type in which it extended to the putamen in 4 patients. Localized type increased with age. It was about 70 times higher in patients above the age of 50 than in patients below the age of 10. The female to male ratio was 2:1. Underlying diseases were idiopathic or familial in cases of localized type, and hypoparathyroidism in cases of diffuse type. Seventy-five percent of the epileptic patients had partial epilepsy. There seems not to be direct correlation between basal ganglia calcification and epilepsy.

  4. The relative phases of basal ganglia activities dynamically shape effective connectivity in Parkinson's disease.

    Science.gov (United States)

    Cagnan, Hayriye; Duff, Eugene Paul; Brown, Peter

    2015-06-01

    Optimal phase alignment between oscillatory neural circuits is hypothesized to optimize information flow and enhance system performance. This theory is known as communication-through-coherence. The basal ganglia motor circuit exhibits exaggerated oscillatory and coherent activity patterns in Parkinson's disease. Such activity patterns are linked to compromised motor system performance as evinced by bradykinesia, rigidity and tremor, suggesting that network function might actually deteriorate once a certain level of net synchrony is exceeded in the motor circuit. Here, we characterize the processes underscoring excessive synchronization and its termination. To this end, we analysed local field potential recordings from the subthalamic nucleus and globus pallidus of five patients with Parkinson's disease (four male and one female, aged 37-64 years). We observed that certain phase alignments between subthalamic nucleus and globus pallidus amplified local neural synchrony in the beta frequency band while others either suppressed it or did not induce any significant change with respect to surrogates. The increase in local beta synchrony directly correlated with how long the two nuclei locked to beta-amplifying phase alignments. Crucially, administration of the dopamine prodrug, levodopa, reduced the frequency and duration of periods during which subthalamic and pallidal populations were phase-locked to beta-amplifying alignments. Conversely ON dopamine, the total duration over which subthalamic and pallidal populations were aligned to phases that left beta-amplitude unchanged with respect to surrogates increased. Thus dopaminergic input shifted circuit dynamics from persistent periods of locking to amplifying phase alignments, associated with compromised motoric function, to more dynamic phase alignment and improved motoric function. This effect of dopamine on local circuit resonance suggests means by which novel electrical interventions might prevent resonance

  5. The Effects of Cues on Neurons in the Basal Ganglia in Parkinson’s Disease

    Directory of Open Access Journals (Sweden)

    Sridevi V. Sarma

    2012-07-01

    Full Text Available Visual cues open a unique window to the understanding of Parkinson’s disease (PD. These cues can temporarily but dramatically improve PD motor symptoms. Although details are unclear, cues are believed to suppress pathological basal ganglia (BG activity through activation of corticostriatal pathways. In this study, we investigated human BG neurophysiology under different cued conditions. We evaluated bursting, 10-30Hz oscillations (OSCs, and directional tuning (DT dynamics in the subthalamic nucleus activity while 7 patients executed a two-step motor task. In the first step (predicted +cue, the patient moved to a target when prompted by a visual go cue that appeared 100% of the time. Here, the timing of the cue is predictable and the cue serves an external trigger to execute a motor plan. In the second step, the cue appeared randomly 50% of the time, and the patient had to move to the same target as in the first step. When it appeared (unpredicted +cue, the motor plan was to be triggered by the cue, but its timing was not predictable. When the cue failed to appear (unpredicted -cue, the motor plan was triggered by the absence of the visual cue. We found that during predicted +cue and unpredicted -cue trials, OSCs significantly decreased and DT significantly increased above baseline, though these modulations occurred an average of 640 milliseconds later in unpredicted -cue trials. Movement and reaction times were comparable in these trials. During unpredicted +cue trials, OSCs and DT failed to modulate though bursting significantly decreased after movement. Correspondingly, movement performance deteriorated. These findings suggest that during motor planning either a predictably timed external cue or an internally generated cue (generated by the absence of a cue trigger the execution of a motor plan in premotor cortex, whose increased activation then suppresses pathological activity in STN through direct pathways, leading to motor facilitation in

  6. Vascular Risk Factors and Diseases Modulate Deficits of Reward-Based Reversal Learning in Acute Basal Ganglia Stroke.

    Directory of Open Access Journals (Sweden)

    Ulla K Seidel

    Full Text Available Besides motor function, the basal ganglia have been implicated in feedback learning. In patients with chronic basal ganglia infarcts, deficits in reward-based reversal learning have previously been described.We re-examined the acquisition and reversal of stimulus-stimulus-reward associations and acquired equivalence in eleven patients with acute basal ganglia stroke (8 men, 3 women; 57.8±13.3 years, whose performance was compared eleven healthy subjects of comparable age, sex distribution and education, who were recruited outside the hospital. Eleven hospitalized patients with a similar vascular risk profile as the stroke patients but without stroke history served as clinical control group.In a neuropsychological assessment 7±3 days post-stroke, verbal and spatial short-term and working memory and inhibition control did not differ between groups. Compared with healthy subjects, control patients with vascular risk factors exhibited significantly reduced performance in the reversal phase (F[2,30] = 3.47; p = 0.044; post-hoc comparison between risk factor controls and healthy controls: p = 0.030, but not the acquisition phase (F[2,30] = 1.01; p = 0.376 and the acquired equivalence (F[2,30] = 1.04; p = 0.367 tasks. In all tasks, the performance of vascular risk factor patients closely resembled that of basal ganglia stroke patients. Correlation studies revealed a significant association of the number of vascular risk factors with reversal learning (r = -0.33, p = 0.012, but not acquisition learning (r = -0.20, p = 0.121 or acquired equivalence (r = -0.22, p = 0.096.The previously reported impairment of reward-based learning may be attributed to vascular risk factors and associated diseases, which are enriched in stroke patients. This study emphasizes the necessity of appropriate control subjects in cognition studies.

  7. Electrocorticography reveals beta desynchronization in the basal ganglia-cortical loop during rest tremor in Parkinson's disease.

    Science.gov (United States)

    Qasim, Salman E; de Hemptinne, Coralie; Swann, Nicole C; Miocinovic, Svjetlana; Ostrem, Jill L; Starr, Philip A

    2016-02-01

    The pathophysiology of rest tremor in Parkinson's disease (PD) is not well understood, and its severity does not correlate with the severity of other cardinal signs of PD. We hypothesized that tremor-related oscillatory activity in the basal-ganglia-thalamocortical loop might serve as a compensatory mechanism for the excessive beta band synchronization associated with the parkinsonian state. We recorded electrocorticography (ECoG) from the sensorimotor cortex and local field potentials (LFP) from the subthalamic nucleus (STN) in patients undergoing lead implantation for deep brain stimulation (DBS). We analyzed differences in measures of network synchronization during epochs of spontaneous rest tremor, versus epochs without rest tremor, occurring in the same subjects. The presence of tremor was associated with reduced beta power in the cortex and STN. Cortico-cortical coherence and phase-amplitude coupling (PAC) decreased during rest tremor, as did basal ganglia-cortical coherence in the same frequency band. Cortical broadband gamma power was not increased by tremor onset, in contrast to the movement-related gamma increase typically observed at the onset of voluntary movement. These findings suggest that the cortical representation of rest tremor is distinct from that of voluntary movement, and support a model in which tremor acts to decrease beta band synchronization within the basal ganglia-cortical loop. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. Identifying the Basal Ganglia network model markers for medication-induced impulsivity in Parkinson's disease patients.

    Science.gov (United States)

    Balasubramani, Pragathi Priyadharsini; Chakravarthy, V Srinivasa; Ali, Manal; Ravindran, Balaraman; Moustafa, Ahmed A

    2015-01-01

    Impulsivity, i.e. irresistibility in the execution of actions, may be prominent in Parkinson's disease (PD) patients who are treated with dopamine precursors or dopamine receptor agonists. In this study, we combine clinical investigations with computational modeling to explore whether impulsivity in PD patients on medication may arise as a result of abnormalities in risk, reward and punishment learning. In order to empirically assess learning outcomes involving risk, reward and punishment, four subject groups were examined: healthy controls, ON medication PD patients with impulse control disorder (PD-ON ICD) or without ICD (PD-ON non-ICD), and OFF medication PD patients (PD-OFF). A neural network model of the Basal Ganglia (BG) that has the capacity to predict the dysfunction of both the dopaminergic (DA) and the serotonergic (5HT) neuromodulator systems was developed and used to facilitate the interpretation of experimental results. In the model, the BG action selection dynamics were mimicked using a utility function based decision making framework, with DA controlling reward prediction and 5HT controlling punishment and risk predictions. The striatal model included three pools of Medium Spiny Neurons (MSNs), with D1 receptor (R) alone, D2R alone and co-expressing D1R-D2R. Empirical studies showed that reward optimality was increased in PD-ON ICD patients while punishment optimality was increased in PD-OFF patients. Empirical studies also revealed that PD-ON ICD subjects had lower reaction times (RT) compared to that of the PD-ON non-ICD patients. Computational modeling suggested that PD-OFF patients have higher punishment sensitivity, while healthy controls showed comparatively higher risk sensitivity. A significant decrease in sensitivity to punishment and risk was crucial for explaining behavioral changes observed in PD-ON ICD patients. Our results highlight the power of computational modelling for identifying neuronal circuitry implicated in learning, and its

  9. Basal ganglia lesions in children and adults

    International Nuclear Information System (INIS)

    Bekiesinska-Figatowska, Monika; Mierzewska, Hanna; Jurkiewicz, Elżbieta

    2013-01-01

    The term “basal ganglia” refers to caudate and lentiform nuclei, the latter composed of putamen and globus pallidus, substantia nigra and subthalamic nuclei and these deep gray matter structures belong to the extrapyramidal system. Many diseases may present as basal ganglia abnormalities. Magnetic resonance imaging (MRI) and computed tomography (CT) – to a lesser degree – allow for detection of basal ganglia injury. In many cases, MRI alone does not usually allow to establish diagnosis but together with the knowledge of age and circumstances of onset and clinical course of the disease is a powerful tool of differential diagnosis. The lesions may be unilateral: in Rassmussen encephalitis, diabetes with hemichorea/hemiballism and infarction or – more frequently – bilateral in many pathologic conditions. Restricted diffusion is attributable to infarction, acute hypoxic–ischemic injury, hypoglycemia, Leigh disease, encephalitis and CJD. Contrast enhancement may be seen in cases of infarction and encephalitis. T1-hyperintensity of the lesions is uncommon and may be observed unilaterally in case of hemichorea/hemiballism and bilaterally in acute asphyxia in term newborns, in hypoglycemia, NF1, Fahr disease and manganese intoxication. Decreased signal intensity on GRE/T2*-weighted images and/or SWI indicating iron, calcium or hemosiderin depositions is observed in panthotenate kinase-associated neurodegeneration, Parkinson variant of multiple system atrophy, Fahr disease (and other calcifications) as well as with the advancing age. There are a few papers in the literature reviewing basal ganglia lesions. The authors present a more detailed review with rich iconography from the own archive

  10. Basal ganglia lesions in children and adults

    Energy Technology Data Exchange (ETDEWEB)

    Bekiesinska-Figatowska, Monika, E-mail: m.figatowska@mp.pl [Department of Diagnostic Imaging, Institute of Mother and Child, ul. Kasprzaka 17a, 01-211 Warsaw (Poland); Mierzewska, Hanna, E-mail: h.mierzewska@gmail.com [Department of Neurology of Children and Adolescents, Institute of Mother and Child, ul. Kasprzaka 17a, 01-211 Warsaw (Poland); Jurkiewicz, Elżbieta, E-mail: e-jurkiewicz@o2.pl [Department of Diagnostic Imaging, Children' s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw (Poland)

    2013-05-15

    The term “basal ganglia” refers to caudate and lentiform nuclei, the latter composed of putamen and globus pallidus, substantia nigra and subthalamic nuclei and these deep gray matter structures belong to the extrapyramidal system. Many diseases may present as basal ganglia abnormalities. Magnetic resonance imaging (MRI) and computed tomography (CT) – to a lesser degree – allow for detection of basal ganglia injury. In many cases, MRI alone does not usually allow to establish diagnosis but together with the knowledge of age and circumstances of onset and clinical course of the disease is a powerful tool of differential diagnosis. The lesions may be unilateral: in Rassmussen encephalitis, diabetes with hemichorea/hemiballism and infarction or – more frequently – bilateral in many pathologic conditions. Restricted diffusion is attributable to infarction, acute hypoxic–ischemic injury, hypoglycemia, Leigh disease, encephalitis and CJD. Contrast enhancement may be seen in cases of infarction and encephalitis. T1-hyperintensity of the lesions is uncommon and may be observed unilaterally in case of hemichorea/hemiballism and bilaterally in acute asphyxia in term newborns, in hypoglycemia, NF1, Fahr disease and manganese intoxication. Decreased signal intensity on GRE/T2*-weighted images and/or SWI indicating iron, calcium or hemosiderin depositions is observed in panthotenate kinase-associated neurodegeneration, Parkinson variant of multiple system atrophy, Fahr disease (and other calcifications) as well as with the advancing age. There are a few papers in the literature reviewing basal ganglia lesions. The authors present a more detailed review with rich iconography from the own archive.

  11. Bilateral functional connectivity of the basal ganglia in patients with Parkinson's disease and its modulation by dopaminergic treatment.

    Science.gov (United States)

    Little, Simon; Tan, Huiling; Anzak, Anam; Pogosyan, Alek; Kühn, Andrea; Brown, Peter

    2013-01-01

    Parkinson's disease is characterised by excessive subcortical beta oscillations. However, little is known about the functional connectivity of the two basal ganglia across hemispheres and specifically the role beta plays in this. We recorded local field potentials from the subthalamic nucleus bilaterally in 23 subjects with Parkinson's disease at rest, on and off medication. We found suppression of low beta power in response to levodopa (t22 = -4.4, pbasal ganglia networks may have to be approached separately with independent sensing and stimulation during adaptive deep brain stimulation. In addition, our findings highlight the functional distinction between the lower and upper beta frequency ranges and between amplitude co-modulation and phase synchronization across subthalamic nuclei.

  12. The Basal Ganglia and Adaptive Motor Control

    Science.gov (United States)

    Graybiel, Ann M.; Aosaki, Toshihiko; Flaherty, Alice W.; Kimura, Minoru

    1994-09-01

    The basal ganglia are neural structures within the motor and cognitive control circuits in the mammalian forebrain and are interconnected with the neocortex by multiple loops. Dysfunction in these parallel loops caused by damage to the striatum results in major defects in voluntary movement, exemplified in Parkinson's disease and Huntington's disease. These parallel loops have a distributed modular architecture resembling local expert architectures of computational learning models. During sensorimotor learning, such distributed networks may be coordinated by widely spaced striatal interneurons that acquire response properties on the basis of experienced reward.

  13. Involvement of dopamine loss in extrastriatal basal ganglia nuclei in the pathophysiology of Parkinson’s disease

    Directory of Open Access Journals (Sweden)

    Abdelhamid eBenazzouz

    2014-05-01

    Full Text Available Parkinson’s disease is a neurological disorder characterized by the manifestation of motor symptoms, such as akinesia, muscle rigidity and tremor at rest. These symptoms are classically attributed to the degeneration of dopamine neurons in the pars compacta of substantia nigra (SNc, which results in a marked dopamine depletion in the striatum. It is well established that dopamine neurons in the SNc innervate not only the striatum, which is the main target, but also other basal ganglia nuclei including the two segments of globus pallidus and the subthalamic nucleus. The role of dopamine and its depletion in the striatum is well known, however, the role of dopamine depletion in the pallidal complex and the subthalamic nucleus in the genesis of their abnormal neuronal activity and in parkinsonian motor deficits is still not clearly determined. Based on recent experimental data from animal models of Parkinson's disease in rodents and non-human primates and also from parkinsonian patients, this review summarizes current knowledge on the role of dopamine in the modulation of basal ganglia neuronal activity and also the role of dopamine depletion in these nuclei in the pathophysiology of Parkinson's disease.

  14. Nitric oxide modulation of the basal ganglia circuitry: therapeutic implication for Parkinson's disease and other motor disorders.

    Science.gov (United States)

    Pierucci, Massimo; Galati, Salvatore; Valentino, Mario; Di Matteo, Vincenzo; Benigno, Arcangelo; Pitruzzella, Alessandro; Muscat, Richard; Di Giovanni, Giuseppe

    2011-11-01

    Several recent studies have emphasized a crucial role for the nitrergic system in movement control and the pathophysiology of the basal ganglia (BG). These observations are supported by anatomical evidence demonstrating the presence of nitric oxide synthase (NOS) in all the basal ganglia nuclei. In fact, nitrergic terminals have been reported to make synaptic contacts with both substantia nigra dopamine-containing neurons and their terminal areas such as the striatum, the globus pallidus and the subthalamus. These brain areas contain a high expression of nitric oxide (NO)-producing neurons, with the striatum having the greatest number, together with important NO afferent input. In this paper, the distribution of NO in the BG nuclei will be described. Furthermore, evidence demonstrating the nitrergic control of BG activity will be reviewed. The new avenues that the increasing knowledge of NO in motor control has opened for exploring the pathophysiology and pharmacology of Parkinson's disease and other movement disorders will be discussed. For example, inhibition of striatal NO/guanosine monophosphate signal pathway by phosphodiesterases seems to be effective in levodopa-induced dyskinesia. However, the results of experimental studies have to be interpreted with caution given the complexities of nitrergic signalling and the limitations of animal models. Nevertheless, the NO system represents a promising pharmacological intervention for treating Parkinson's disease and related disorders.

  15. Associations of olfactory bulb and depth of olfactory sulcus with basal ganglia and hippocampus in patients with Parkinson's disease.

    Science.gov (United States)

    Tanik, Nermin; Serin, Halil Ibrahim; Celikbilek, Asuman; Inan, Levent Ertugrul; Gundogdu, Fatma

    2016-05-04

    Parkinson's disease (PD) is a neurodegenerative disorder characterized by hyposmia in the preclinical stages. We investigated the relationships of olfactory bulb (OB) volume and olfactory sulcus (OS) depth with basal ganglia and hippocampal volumes. The study included 25 patients with PD and 40 age- and sex-matched control subjects. Idiopathic PD was diagnosed according to published diagnostic criteria. The Hoehn and Yahr (HY) scale, the motor subscale of the Unified Parkinson's Disease Rating Scale (UPDRS III), and the Mini-Mental State Examination (MMSE) were administered to participants. Volumetric measurements of olfactory structures, the basal ganglia, and hippocampus were performed using magnetic resonance imaging (MRI). OB volume and OS depth were significantly reduced in PD patients compared to healthy control subjects (p<0.001 and p<0.001, respectively). The OB and left putamen volumes were significantly correlated (p=0.048), and the depth of the right OS was significantly correlated with right hippocampal volume (p=0.018). We found significant correlations between OB and putamen volumes and OS depth and hippocampal volume. Our study is the first to demonstrate associations of olfactory structures with the putamen and hippocampus using MRI volumetric measurements. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  16. Dopaminergic innervation of human basal ganglia.

    Science.gov (United States)

    Prensa, L; Cossette, M; Parent, A

    2000-12-01

    This paper summarises the results of some of our recent tyrosine hydroxylase (TH) immunohistochemical studies of the dopaminergic innervation of the human basal ganglia. It also reports new findings on the presence of TH-immunoreactive (ir) neurons in the striatum. Our data show the existence of nigrostriatal TH-ir axons that provide collaterals arborizing in the globus pallidus and subthalamic nucleus. These thin and varicose collaterals emerge from thick and smooth axons that course along the main output pathways of the basal ganglia, including the ansa lenticularis, the lenticular fasciculus and Wilson's pencils. We postulate that this extrastriatal innervation, which allows nigral dopaminergic neurons to directly affect the pallidum and subthalamic nucleus, plays a critical role in the functional organisation of human basal ganglia. The TH-ir fibres that reach the striatum arborize according to a highly heterogeneous pattern. At rostral striatal levels, numerous small TH-poor zones embedded in a TH-rich matrix correspond to calbindin-poor striosomes and calbindin-rich extrastriosomal matrix, respectively. At caudal striatal levels, in contrast, striosomes display a TH immunostaining that is more intense than that of the matrix. A significant number of small, oval, aspiny TH-ir neurons scattered throughout the rostrocaudal extent of the caudate nucleus and putamen, together with a few larger, multipolar, spiny TH-ir neurons lying principally within the ventral portion of the putamen, were disclosed in human. This potential source of intrinsic striatal dopamine might play an important role in the functional organisation of the human striatum, particularly in case of Parkinson's disease.

  17. Computed tomography of calcification of the basal ganglia

    International Nuclear Information System (INIS)

    Park, Churl Min; Suh, Soo Jhi; Kim, Soon Yong

    1981-01-01

    Calcifications of the basal ganglia are rarely found at routine autopsies and in skull radiographs. CT is superior to the plain skull radiographs in detecting intracranial attenuation differences and may be stated to be the method of choice in the diagnosis of intracranial calcifications. Of 5985 brain CT scans performed in Kyung Hee University Hospital during past 3 years, 36 cases were found to have high attenuation lesions suggesting calcifications within basal ganglia. 1. The incidence of basal ganglia calcification on CT scan was about 0.6%. 2. Of these 36 cases, 34 cases were bilateral and the remainder was unilateral. 3. The plain skull films of 23 cases showed visible calcification of basal ganglia in 3 cases (13%). 4. No specific metabolic disease was noted in the cases

  18. Oscillatory activity in the basal ganglia.

    Science.gov (United States)

    Eusebio, Alexandre; Brown, Peter

    2007-01-01

    The exact mechanisms underlying the dysfunction of the basal ganglia (BG) that leads to movement disorders such as Parkinson's disease (PD) and dystonia still remain unclear. The classic model, based on two distinct pathways and described nearly 20 years ago by Albin and Delong, fails to explain why lesion or stimulation of the globus pallidus interna improves dyskinesias and why lesion or stimulation of the thalamus does not cause prominent bradykinesia. These paradoxes, initially highlighted out by Marsden and Obeso, led to the proposition that the pattern of neuronal discharge determines pathological function. Accordingly, over the past decade, attention has switched from considerations of discharge rate to the characterisation of synchronised activity within BG networks. Here we would like to briefly review current knowledge about synchronised oscillatory activity in the BG and focus on its relationship to abnormal motor function. In particular, we hypothesise that the frequency of synchronisation helps determine the nature of any motor deficit, perhaps as a consequence of the different tuning properties of basal ganglia-cortical sub-circuits.

  19. Freezing of gait in Parkinson's disease is associated with functional decoupling between the cognitive control network and the basal ganglia.

    Science.gov (United States)

    Shine, James M; Matar, Elie; Ward, Philip B; Frank, Michael J; Moustafa, Ahmed A; Pearson, Mark; Naismith, Sharon L; Lewis, Simon J G

    2013-12-01

    Recent neuroimaging evidence has led to the proposal that freezing of gait in Parkinson's disease is due to dysfunctional interactions between frontoparietal cortical regions and subcortical structures, such as the striatum. However, to date, no study has employed task-based functional connectivity analyses to explore this hypothesis. In this study, we used a data-driven multivariate approach to explore the impaired communication between distributed neuronal networks in 10 patients with Parkinson's disease and freezing of gait, and 10 matched patients with no clinical history of freezing behaviour. Patients performed a virtual reality gait task on two separate occasions (once ON and once OFF their regular dopaminergic medication) while functional magnetic resonance imaging data were collected. Group-level independent component analysis was used to extract the subject-specific time courses associated with five well-known neuronal networks: the motor network, the right- and left cognitive control networks, the ventral attention network and the basal ganglia network. We subsequently analysed both the activation and connectivity of these neuronal networks between the two groups with respect to dopaminergic state and cognitive load while performing the virtual reality gait task. During task performance, all patients used the left cognitive control network and the ventral attention network and in addition, showed increased connectivity between the bilateral cognitive control networks. However, patients with freezing demonstrated functional decoupling between the basal ganglia network and the cognitive control network in each hemisphere. This decoupling was also associated with paroxysmal motor arrests. These results support the hypothesis that freezing behaviour in Parkinson's disease is because of impaired communication between complimentary yet competing neural networks.

  20. Behavioural effects of basal ganglia rho-kinase inhibition in the unilateral 6-hydroxydopamine rat model of Parkinson's disease.

    Science.gov (United States)

    Inan, Salim Yalcin; Soner, Burak Cem; Sahin, Ayse Saide

    2016-08-01

    Parkinson's disease (PD) is one of the most common neurodegenerative disorders, which affects more than six million people in the world. While current available pharmacological therapies for PD in the early stages of the disease usually improve motor symptoms, they cause side effects, such as fluctuations and dyskinesias in the later stages. In this later stage, high frequency deep brain stimulation of the subthalamic nucleus (STN-DBS) is a treatment option which is most successful to treat drug resistant advanced PD. It has previously been demonstrated that activation of Rho/Rho-kinase pathway is involved in the dopaminergic cell degeneration which is one of the main characteristics of PD pathology. In addition, the involvement of this pathway has been suggested in diverse cellular events in the central nervous system; such as epilepsy, anxiety-related behaviors, regulation of dendritic and axonal morphology, antinociception, subarachnoid haemorrhage, spinal cord injury and amyotrophic lateral sclerosis. However, up to date, to our knowledge there are no previous reports showing the beneficial effects of the potent Rho-kinase inhibitor Y-27632 in the 6-hydroxydopamine (6-OHDA) rat model of PD. Therefore, in the present study, we investigated the behavioural effects of basal ganglia Y-27632 microinjections in this PD model. Our results indicated that basal ganglia Y-27632 microinjections significantly decreased the number of contralateral rotations-induced by apomorphine, significantly increased line crossings in the open-field test, contralateral forelimb use in the limb-use asymmetry test and contralateral tape playing time in the somatosensory asymmetry test, which may suggest that Y-27632 could be a potentially active antiparkinsonian agent.

  1. The connectome of the basal ganglia.

    Science.gov (United States)

    Schmitt, Oliver; Eipert, Peter; Kettlitz, Richard; Leßmann, Felix; Wree, Andreas

    2016-03-01

    The basal ganglia of the laboratory rat consist of a few core regions that are specifically interconnected by efferents and afferents of the central nervous system. In nearly 800 reports of tract-tracing investigations the connectivity of the basal ganglia is documented. The readout of connectivity data and the collation of all the connections of these reports in a database allows to generate a connectome. The collation, curation and analysis of such a huge amount of connectivity data is a great challenge and has not been performed before (Bohland et al. PloS One 4:e7200, 2009) in large connectomics projects based on meta-analysis of tract-tracing studies. Here, the basal ganglia connectome of the rat has been generated and analyzed using the consistent cross-platform and generic framework neuroVIISAS. Several advances of this connectome meta-study have been made: the collation of laterality data, the network-analysis of connectivity strengths and the assignment of regions to a hierarchically organized terminology. The basal ganglia connectome offers differences in contralateral connectivity of motoric regions in contrast to other regions. A modularity analysis of the weighted and directed connectome produced a specific grouping of regions. This result indicates a correlation of structural and functional subsystems. As a new finding, significant reciprocal connections of specific network motifs in this connectome were detected. All three principal basal ganglia pathways (direct, indirect, hyperdirect) could be determined in the connectome. By identifying these pathways it was found that there exist many further equivalent pathways possessing the same length and mean connectivity weight as the principal pathways. Based on the connectome data it is unknown why an excitation pattern may prefer principal rather than other equivalent pathways. In addition to these new findings the local graph-theoretical features of regions of the connectome have been determined. By

  2. A three-dimensional histological atlas of the human basal ganglia. II. Atlas deformation strategy and evaluation in deep brain stimulation for Parkinson disease.

    Science.gov (United States)

    Bardinet, Eric; Bhattacharjee, Manik; Dormont, Didier; Pidoux, Bernard; Malandain, Grégoire; Schüpbach, Michael; Ayache, Nicholas; Cornu, Philippe; Agid, Yves; Yelnik, Jérôme

    2009-02-01

    The localization of any given target in the brain has become a challenging issue because of the increased use of deep brain stimulation to treat Parkinson disease, dystonia, and nonmotor diseases (for example, Tourette syndrome, obsessive compulsive disorders, and depression). The aim of this study was to develop an automated method of adapting an atlas of the human basal ganglia to the brains of individual patients. Magnetic resonance images of the brain specimen were obtained before extraction from the skull and histological processing. Adaptation of the atlas to individual patient anatomy was performed by reshaping the atlas MR images to the images obtained in the individual patient using a hierarchical registration applied to a region of interest centered on the basal ganglia, and then applying the reshaping matrix to the atlas surfaces. Results were evaluated by direct visual inspection of the structures visible on MR images and atlas anatomy, by comparison with electrophysiological intraoperative data, and with previous atlas studies in patients with Parkinson disease. The method was both robust and accurate, never failing to provide an anatomically reliable atlas to patient registration. The registration obtained did not exceed a 1-mm mismatch with the electrophysiological signatures in the region of the subthalamic nucleus. This registration method applied to the basal ganglia atlas forms a powerful and reliable method for determining deep brain stimulation targets within the basal ganglia of individual patients.

  3. Basal Ganglia Perfusion in Fibromyalgia is Related to Pain Disability and Disease Impact: An Arterial Spin Labeling Study.

    Science.gov (United States)

    Shokouhi, Mahsa; Davis, Karen D; Moulin, Dwight E; Morley-Forster, Pat; Nielson, Warren R; Bureau, Yves; St Lawrence, Keith

    2016-06-01

    Pain disability is a major impediment to fibromyalgia (FM) patients' quality of life. Neuroimaging studies have demonstrated abnormal pain processing in FM. However, it is not known whether there are brain abnormalities linked to pain disability. Understanding neural correlates of pain disability in FM, independent from pain intensity, could provide a framework to guide future more efficient therapy strategies to improve patients' functional ability. We used arterial spin labeling to image cerebral blood flow (CBF) in 23 FM patients and 16 controls. Functional connectivity was also estimated using blood oxygenation level-dependent imaging to further investigate the possible underpinnings of the observed CBF changes. Among patients, CBF in the basal ganglia correlated negatively with pain disability index and positively with the overall impact of FM (Fibromyalgia Impact Questionnaire) but did not correlate with pain intensity. Whole-brain analysis revealed no CBF differences between the 2 groups; however, post hoc analysis in the basal ganglia showed CBF reductions mainly in the right putamen and right lateral globus pallidus in patients, likely reflecting the negative correlation with the pain disability index. However, the connectivity of the corresponding corticobasal ganglia-thalamus loop, that is, motor network (the connection between supplementary motor area, putamen, and thalamus) remained intact. Basal ganglia perfusion reflects long-term symptoms, including somatic and psychological components of FM rather than pain intensity. These CBF findings may reflect differences in behavioral and psychological responses between patients.

  4. [Emotion and basal ganglia (II): what can we learn from subthalamic nucleus deep brain stimulation in Parkinson's disease?].

    Science.gov (United States)

    Péron, J; Dondaine, T

    2012-01-01

    The subthalamic nucleus deep-brain stimulation Parkinson's disease patient model seems to represent a unique opportunity for studying the functional role of the basal ganglia and notably the subthalamic nucleus in human emotional processing. Indeed, in addition to constituting a therapeutic advance for severely disabled Parkinson's disease patients, deep brain stimulation is a technique, which selectively modulates the activity of focal structures targeted by surgery. There is growing evidence of a link between emotional impairments and deep-brain stimulation of the subthalamic nucleus. In this context, according to the definition of emotional processing exposed in the companion paper available in this issue, the aim of the present review will consist in providing a synopsis of the studies that investigated the emotional disturbances observed in subthalamic nucleus deep brain stimulation Parkinson's disease patients. This review leads to the conclusion that several emotional components would be disrupted after subthalamic nucleus deep brain stimulation in Parkinson's disease: subjective feeling, neurophysiological activation, and motor expression. Finally, after a description of the limitations of this study model, we discuss the functional role of the subthalamic nucleus (and the striato-thalamo-cortical circuits in which it is involved) in emotional processing. It seems reasonable to conclude that the striato-thalamo-cortical circuits are indeed involved in emotional processing and that the subthalamic nucleus plays a central in role the human emotional architecture. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  5. Interaction between the 5-HT system and the basal ganglia: Functional implication and therapeutic perspective in Parkinson’s disease

    Directory of Open Access Journals (Sweden)

    Cristina eMiguelez

    2014-03-01

    Full Text Available The neurotransmitter serotonin (5-HT has a multifaceted function in the modulation of information processing through the activation of multiple receptor families, including G-protein-coupled receptor subtypes (5-HT1, 5-HT2, 5-HT4-7 and ligand-gated ion channels (5-HT3. The largest population of serotonergic neurons is located in the midbrain, specifically in the raphe nuclei. Although the medial and dorsal raphe nucleus (DRN share common projecting areas, in the basal ganglia (BG nuclei serotonergic innervations come mainly from the DRN. The BG are a highly organized network of subcortical nuclei composed of the striatum (caudate and putamen, subthalamic nucleus (STN, internal and external globus pallidus (or entopeduncular nucleus in rodents, GPi/EP and GPe and substantia nigra (pars compacta, SNc, and pars reticulata, SNr. The BG are part of the cortico-BG-thalamic circuits, which play a role in many functions like motor control, emotion, and cognition and are critically involved in diseases such as Parkinson’s disease. This review provides an overview of serotonergic modulation of the BG at the functional level and a discussion of how this interaction may be relevant to treating Parkinson’s disease and the motor complications induced by chronic treatment with L-DOPA.

  6. Interaction between the 5-HT system and the basal ganglia: functional implication and therapeutic perspective in Parkinson's disease.

    Science.gov (United States)

    Miguelez, Cristina; Morera-Herreras, Teresa; Torrecilla, Maria; Ruiz-Ortega, Jose A; Ugedo, Luisa

    2014-01-01

    The neurotransmitter serotonin (5-HT) has a multifaceted function in the modulation of information processing through the activation of multiple receptor families, including G-protein-coupled receptor subtypes (5-HT1, 5-HT2, 5-HT4-7) and ligand-gated ion channels (5-HT3). The largest population of serotonergic neurons is located in the midbrain, specifically in the raphe nuclei. Although the medial and dorsal raphe nucleus (DRN) share common projecting areas, in the basal ganglia (BG) nuclei serotonergic innervations come mainly from the DRN. The BG are a highly organized network of subcortical nuclei composed of the striatum (caudate and putamen), subthalamic nucleus (STN), internal and external globus pallidus (or entopeduncular nucleus in rodents, GPi/EP and GPe) and substantia nigra (pars compacta, SNc, and pars reticulata, SNr). The BG are part of the cortico-BG-thalamic circuits, which play a role in many functions like motor control, emotion, and cognition and are critically involved in diseases such as Parkinson's disease (PD). This review provides an overview of serotonergic modulation of the BG at the functional level and a discussion of how this interaction may be relevant to treating PD and the motor complications induced by chronic treatment with L-DOPA.

  7. Reduced topological efficiency in cortical-basal Ganglia motor network of Parkinson's disease: a resting state fMRI study.

    Science.gov (United States)

    Wei, Luqing; Zhang, Jiuquan; Long, Zhiliang; Wu, Guo-Rong; Hu, Xiaofei; Zhang, Yanling; Wang, Jian

    2014-01-01

    Parkinson's disease (PD) is mainly characterized by dopamine depletion of the cortico-basal ganglia (CBG) motor circuit. Given that dopamine dysfunction could affect functional brain network efficiency, the present study utilized resting-state fMRI (rs-fMRI) and graph theoretical approach to investigate the topological efficiency changes of the CBG motor network in patients with PD during a relatively hypodopaminergic state (12 hours after a last dose of dopamimetic treatment). We found that PD compared with controls had remarkable decreased efficiency in the CBG motor network, with the most pronounced changes observed in rostral supplementary motor area (pre-SMA), caudal SMA (SMA-proper), primary motor cortex (M1), primary somatosensory cortex (S1), thalamus (THA), globus pallidus (GP), and putamen (PUT). Furthermore, reduced efficiency in pre-SMA, M1, THA and GP was significantly correlated with Unified Parkinson's Disease Rating Scale (UPDRS) motor scores in PD patients. Together, our results demonstrate that individuals with PD appear to be less effective at information transfer within the CBG motor pathway, which provides a novel perspective on neurobiological explanation for the motor symptoms in patients. These findings are in line with the pathophysiology of PD, suggesting that network efficiency metrics may be used to identify and track the pathology of PD.

  8. Restoring the basal ganglia in Parkinson's disease to normal via multi-input phase-shifted deep brain stimulation.

    Science.gov (United States)

    Agarwal, Rahul; Sarma, Sridevi V

    2010-01-01

    Deep brain stimulation (DBS) injects a high frequency current that effectively disables the diseased basal ganglia (BG) circuit in Parkinson's disease (PD) patients, leading to a reversal of motor symptoms. Though therapeutic, high frequency stimulation consumes significant power forcing frequent surgical battery replacements and causing widespread influence into other brain areas which may lead to adverse side effects. In this paper, we conducted a rigorous study to assess whether low frequency signals can restore behavior in PD patients by restoring neural activity in the BG to the normal state. We used a biophysical-based model of BG nuclei and motor thalamus whose parameters can be set to simulate the normal state and the PD state with and without DBS. We administered pulse train DBS waveforms to the subthalamic nucleus (STN) with frequencies ranging from 1-150Hz. For each DBS frequency, we computed statistics on the simulated neural activity to assess whether it is restored to the normal state. In particular, we searched for DBS waveforms that suppress pathological bursting, oscillations, correlations and synchronization prevalent in the PD state and that enable thalamic cells to relay cortical inputs reliably. We found that none of the tested waveforms restores neural activity to the normal state. However, our simulations led us to construct a novel DBS strategy involving low frequency multi-input phaseshifted DBS to be administered into the STN. This strategy successfully suppressed all pathological symptoms in the BG in addition to enabling thalamic cells to relay cortical inputs reliably.

  9. Time representation in reinforcement learning models of the basal ganglia

    Directory of Open Access Journals (Sweden)

    Samuel Joseph Gershman

    2014-01-01

    Full Text Available Reinforcement learning models have been influential in understanding many aspects of basal ganglia function, from reward prediction to action selection. Time plays an important role in these models, but there is still no theoretical consensus about what kind of time representation is used by the basal ganglia. We review several theoretical accounts and their supporting evidence. We then discuss the relationship between reinforcement learning models and the timing mechanisms that have been attributed to the basal ganglia. We hypothesize that a single computational system may underlie both reinforcement learning and interval timing—the perception of duration in the range of seconds to hours. This hypothesis, which extends earlier models by incorporating a time-sensitive action selection mechanism, may have important implications for understanding disorders like Parkinson's disease in which both decision making and timing are impaired.

  10. Traumatic bilateral basal ganglia hematoma: A report of two cases

    OpenAIRE

    Bhargava, Pranshu; Grewal, Sarvpreet Singh; Gupta, Bharat; Jain, Vikas; Sobti, Harman

    2012-01-01

    Traumatic Basal ganglia hemorrhage is relatively uncommon. Bilateral basal ganglia hematoma after trauma is extremely rare and is limited to case reports. We report two cases of traumatic bilateral basal ganglia hemorrhage, and review the literature in brief. Both cases were managed conservatively.

  11. Learning Reward Uncertainty in the Basal Ganglia.

    Directory of Open Access Journals (Sweden)

    John G Mikhael

    2016-09-01

    Full Text Available Learning the reliability of different sources of rewards is critical for making optimal choices. However, despite the existence of detailed theory describing how the expected reward is learned in the basal ganglia, it is not known how reward uncertainty is estimated in these circuits. This paper presents a class of models that encode both the mean reward and the spread of the rewards, the former in the difference between the synaptic weights of D1 and D2 neurons, and the latter in their sum. In the models, the tendency to seek (or avoid options with variable reward can be controlled by increasing (or decreasing the tonic level of dopamine. The models are consistent with the physiology of and synaptic plasticity in the basal ganglia, they explain the effects of dopaminergic manipulations on choices involving risks, and they make multiple experimental predictions.

  12. Do gap junctions regulate synchrony in the parkinsonian basal ganglia?

    NARCIS (Netherlands)

    Schwab, B.C.

    2016-01-01

    Patients with Parkinson’s disease (PD) typically suffer severely from different types of symptoms. Motor symptoms, restricting the patients’ ability to perform controlled movements in daily life, are of special clinical interest and have been related to neural activity in the basal ganglia.

  13. [Emotion and basal ganglia (I): what can we learn from Parkinson's disease?].

    Science.gov (United States)

    Dondaine, T; Péron, J

    2012-01-01

    Parkinson's disease provides a useful model for studying the neural substrates of emotional processing. The striato-thalamo-cortical circuits, like the mesolimbic dopamine system that modulates their function, are thought to be involved in emotional processing. As Parkinson's disease is histopathologically characterized by the selective, progressive and chronic degeneration of the nigrostriatal and mesocorticolimbic dopamine systems, it can therefore serve as a model for assessing the functional role of these circuits in humans. In the present review, after a definition of emotional processing from a multicomponential perspective, a synopsis of the emotional disturbances observed in Parkinson's disease is proposed. Note that the studies on the affective consequences of subthalamic nucleus deep brain stimulation in Parkinson's disease were excluded from this review because the subject of a companion paper in this issue. This review leads to the conclusion that several emotional components would be disrupted in Parkinson's disease: subjective feeling, neurophysiological activation, and motor expression. We then discuss the functional roles of the striato-thalamo-cortical and mesolimbic circuits, ending with the conclusion that both these pathways are indeed involved in emotional processing. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  14. Bilateral hyperintense basal ganglia on T1-weighted image

    International Nuclear Information System (INIS)

    Baik, Seung Kug; Ahn, Woo Hyun; Choi, Han Yong; Kim, Bong Gi

    1994-01-01

    Bilateral high signal intensity in basal ganglia on T1-weighted images is unusual, the purpose of this study is to describe the pattern of high signal intensity and underlying disease. During the last three years, 8 patients showed bilateral high signal intensity in basal ganglia on T1-weighted image, as compared with cerebral white matter. Authors analyzed the images and underlying causes retrospectively. Of 8 patients, 5 were male and 3 were female. The age ranged from 15 days to 79 years. All patient were examined by a 0.5T superconductive MRI. Images were obtained by spin echo multislice technique. Underlying causes were 4 cases of hepatopathy, 2 cases of calcium metabolism disorder, and one case each of neurofibromatosis and hypoxic brain injury. These process were bilateral in all cases and usually symmetric. In all cases the hyperintense areas were generally homogenous without mass effect or edema, although somewhat nodular appearance was seen in neurofibromatosis. Lesions were located in the globus pallidus and internal capsule in hepatopathy and neurofibromatosis, head of the caudate nucleus in disorder of calcum metabolism, and the globus pallidus in hypoxic brain injury. Although this study is limited by its patient population, bilateral hyperintense basal ganglia is associated with various disease entities. On analysis of hyperintense basal ganglia lesion, the knowledge of clinical information improved diagnostic accuracy

  15. Gait variability and basal ganglia disorders: stride-to-stride variations of gait cycle timing in Parkinson's disease and Huntington's disease

    Science.gov (United States)

    Hausdorff, J. M.; Cudkowicz, M. E.; Firtion, R.; Wei, J. Y.; Goldberger, A. L.

    1998-01-01

    The basal ganglia are thought to play an important role in regulating motor programs involved in gait and in the fluidity and sequencing of movement. We postulated that the ability to maintain a steady gait, with low stride-to-stride variability of gait cycle timing and its subphases, would be diminished with both Parkinson's disease (PD) and Huntington's disease (HD). To test this hypothesis, we obtained quantitative measures of stride-to-stride variability of gait cycle timing in subjects with PD (n = 15), HD (n = 20), and disease-free controls (n = 16). All measures of gait variability were significantly increased in PD and HD. In subjects with PD and HD, gait variability measures were two and three times that observed in control subjects, respectively. The degree of gait variability correlated with disease severity. In contrast, gait speed was significantly lower in PD, but not in HD, and average gait cycle duration and the time spent in many subphases of the gait cycle were similar in control subjects, HD subjects, and PD subjects. These findings are consistent with a differential control of gait variability, speed, and average gait cycle timing that may have implications for understanding the role of the basal ganglia in locomotor control and for quantitatively assessing gait in clinical settings.

  16. Effect of Dexmedetomidine and Propofol on Basal Ganglia Activity in Parkinson Disease: A Controlled Clinical Trial.

    Science.gov (United States)

    Martinez-Simon, Antonio; Alegre, Manuel; Honorato-Cia, Cristina; Nuñez-Cordoba, Jorge M; Cacho-Asenjo, Elena; Trocóniz, Iñaki F; Carmona-Abellán, Mar; Valencia, Miguel; Guridi, Jorge

    2017-06-01

    Deep brain stimulation electrodes can record oscillatory activity from deep brain structures, known as local field potentials. The authors' objective was to evaluate and quantify the effects of dexmedetomidine (0.2 μg·kg·h) on local field potentials in patients with Parkinson disease undergoing deep brain stimulation surgery compared with control recording (primary outcome), as well as the effect of propofol at different estimated peak effect site concentrations (0.5, 1.0, 1.5, 2.0, and 2.5 μg/ml) from control recording. A nonrandomized, nonblinded controlled clinical trial was carried out to assess the change in local field potentials activity over time in 10 patients with Parkinson disease who underwent deep brain stimulation placement surgery (18 subthalamic nuclei). The relationship was assessed between the activity in nuclei in the same patient at a given time and repeated measures from the same nucleus over time. No significant difference was observed between the relative beta power of local field potentials in dexmedetomidine and control recordings (-7.7; 95% CI, -18.9 to 7.6). By contrast, there was a significant decline of 12.7% (95% CI, -21.3 to -4.7) in the relative beta power of the local field potentials for each increment in the estimated peak propofol concentrations at the effect site relative to the control recordings. Dexmedetomidine (0.2 μg·kg·h) did not show effect on local field potentials compared with control recording. A significant deep brain activity decline from control recording was observed with incremental doses of propofol.

  17. Use of intraoperative local field potential spectral analysis to differentiate basal ganglia structures in Parkinson's disease patients.

    Science.gov (United States)

    Kolb, Rachel; Abosch, Aviva; Felsen, Gidon; Thompson, John A

    2017-06-01

    Identification of brain structures traversed during implantation of deep brain-stimulating (DBS) electrodes into the subthalamic nucleus (STN-DBS) for the treatment of Parkinson's disease (PD) frequently relies on subjective correspondence between kinesthetic response and multiunit activity. However, recent work suggests that local field potentials (LFP) could be used as a more robust signal to objectively differentiate subcortical structures. The goal of this study was to analyze the spectral properties of LFP collected during STN-DBS in order to objectively identify commonly traversed brain regions and improve our understanding of aberrant oscillations in the PD-related pathophysiological cortico-basal ganglia network. In 21 PD patients, LFP were collected and analyzed during STN-DBS implantation surgery. Spectral power for delta-, theta-, alpha-, low-beta-, and high-beta-frequency bands was assessed at multiple depths throughout the subcortical structures traversed on the trajectory to the ventral border of STN. Similar to previous findings, beta-band oscillations had an increased magnitude within the borders of the motor-related area of STN, however, across several subjects, we also observed increased high-beta magnitude within the borders of thalamus. Comparing across all patients using relative power, we observed a gradual increase in the magnitude of both low- and high-beta-frequency bands as the electrode descended from striatum to STN. These results were also compared with frequency bands below beta, and similar trends were observed. Our results suggest that LFP signals recorded during the implantation of a DBS electrode evince distinct oscillatory signatures that distinguish subcortical structures. © 2017 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society.

  18. Bee Venom Alleviates Motor Deficits and Modulates the Transfer of Cortical Information through the Basal Ganglia in Rat Models of Parkinson's Disease.

    Science.gov (United States)

    Maurice, Nicolas; Deltheil, Thierry; Melon, Christophe; Degos, Bertrand; Mourre, Christiane; Amalric, Marianne; Kerkerian-Le Goff, Lydia

    2015-01-01

    Recent evidence points to a neuroprotective action of bee venom on nigral dopamine neurons in animal models of Parkinson's disease (PD). Here we examined whether bee venom also displays a symptomatic action by acting on the pathological functioning of the basal ganglia in rat PD models. Bee venom effects were assessed by combining motor behavior analyses and in vivo electrophysiological recordings in the substantia nigra pars reticulata (SNr, basal ganglia output structure) in pharmacological (neuroleptic treatment) and lesional (unilateral intranigral 6-hydroxydopamine injection) PD models. In the hemi-parkinsonian 6-hydroxydopamine lesion model, subchronic bee venom treatment significantly alleviates contralateral forelimb akinesia and apomorphine-induced rotations. Moreover, a single injection of bee venom reverses haloperidol-induced catalepsy, a pharmacological model reminiscent of parkinsonian akinetic deficit. This effect is mimicked by apamin, a blocker of small conductance Ca2+-activated K+ (SK) channels, and blocked by CyPPA, a positive modulator of these channels, suggesting the involvement of SK channels in the bee venom antiparkinsonian action. In vivo electrophysiological recordings in the substantia nigra pars reticulata (basal ganglia output structure) showed no significant effect of BV on the mean neuronal discharge frequency or pathological bursting activity. In contrast, analyses of the neuronal responses evoked by motor cortex stimulation show that bee venom reverses the 6-OHDA- and neuroleptic-induced biases in the influence exerted by the direct inhibitory and indirect excitatory striatonigral circuits. These data provide the first evidence for a beneficial action of bee venom on the pathological functioning of the cortico-basal ganglia circuits underlying motor PD symptoms with potential relevance to the symptomatic treatment of this disease.

  19. Impact of surgery targeting the caudal intralaminar thalamic nuclei on the pathophysiological functioning of basal ganglia in a rat model of Parkinson's disease.

    Science.gov (United States)

    Kerkerian-Le Goff, Lydia; Bacci, Jean-Jacques; Jouve, Loreline; Melon, Christophe; Salin, Pascal

    2009-02-16

    There is accumulating evidence that the centre median-parafascicular (CM/Pf) complex of the thalamus is implicated in basal ganglia-related movement disorders and notably in Parkinson's disease. However, the impact of the changes affecting CM/Pf on the pathophysiological functioning of basal ganglia in parkinsonian state remains poorly understood. To address this issue, we have examined the effects of excitotoxic lesion of CM/Pf and of 6-hydroxydopamine-induced lesion of nigral dopamine neurons, separately or in association, on gene expression of markers of neuronal activity in the rat basal ganglia (striatal neuropeptide precursors, GAD67, cytochrome oxidase subunit I) by quantitative in situ hybridization histochemistry. CM/Pf lesion prevented the changes produced by the dopamine denervation in the components of the indirect pathway connecting the striatum to the output structures (striatopallidal neurons, globus pallidus, subthalamic nucleus), and among the output structures, in the entopeduncular nucleus. Preliminary data on the effects of deep brain stimulation of CM/Pf in rats with nigral dopamine lesion show that this surgical approach produces efficient anti-akinetic effect associated with partial reversal of the dopamine lesion-induced increase in striatal preproenkephalin A mRNA levels, a marker of the striatopallidal neurons. These data, which provide substrates for the potential of CM/Pf surgery in the treatment of movement disorders, are discussed in comparison with the effects of lesion or deep brain stimulation of the subthalamic nucleus, the currently preferred target for the surgical treatment of PD.

  20. Paradoxes of functional neurosurgery: clues from basal ganglia recordings.

    Science.gov (United States)

    Brown, Peter; Eusebio, Alexandre

    2008-01-01

    Deep brain stimulation (DBS) can be remarkably effective in treating movement disorders such as Parkinson's disease, dystonia, and essential tremor. Yet these effects remain essentially unexplained, even paradoxical. Equally challenging is the fact that DBS of motor targets in the basal ganglia appears to reverse abnormalities of movement without any obvious deleterious effects on remaining aspects of movement. Here, we explore the extent to which the noisy signal hypothesis might help solve some of these apparent paradoxes. Essentially the hypothesis, first tentatively advanced by Marsden and Obeso (1994), suggests that disease leads to a pattern of basal ganglia activity that disrupts local and distant function and that surgery acts to suppress or override this noisy signal. Critical to the success this theory is that different disease phenotypes are associated with different patterns of noisy signal, and we survey the evidence to support this contention, with specific emphasis on different types of pathological synchronization. However, just as DBS may suppress or override noisy signals in the basal ganglia, it must equally antagonize any remaining physiological functioning in these key motor structures. We argue that the latter effect of DBS becomes manifest when baseline motor performance is relatively preserved, i.e., when pathological activity is limited. Under these circumstances, the deleterious effects of DBS are no longer obscured by its therapeutic actions in suppressing noisy signals. Whether true, oversimplified or simply incorrect, the noisy signal hypothesis has served to focus attention on the detailed character of basal ganglia discharge and its variation with disease and therapy. 2007 Movement Disorder Society

  1. Centrality of striatal cholinergic transmission in basal ganglia function

    Directory of Open Access Journals (Sweden)

    Paola eBonsi

    2011-02-01

    Full Text Available Work over the past two decades revealed a previously unexpected role for striatal cholinergic interneurons in the context of basal ganglia function. The recognition that these interneurons are essential in synaptic plasticity and motor learning represents a significant step ahead in deciphering how the striatum processes cortical inputs, and why pathological circumstances cause motor dysfunction.Loss of the reciprocal modulation between dopaminergic inputs and the intrinsic cholinergic innervation within the striatum appears to be the trigger for pathophysiological changes occurring in basal ganglia disorders. Accordingly, there is now compelling evidence showing profound changes in cholinergic markers in these disorders, in particular Parkinson’s disease and dystonia.Based on converging experimental and clinical evidence, we provide an overview of the role of striatal cholinergic transmission in physiological and pathological conditions, in the context of the pathogenesis of movement disorders.

  2. Treatment of biotin-responsive basal ganglia disease: Open comparative study between the combination of biotin plus thiamine versus thiamine alone.

    Science.gov (United States)

    Tabarki, Brahim; Alfadhel, Majid; AlShahwan, Saad; Hundallah, Khaled; AlShafi, Shatha; AlHashem, Amel

    2015-09-01

    To compare the combination of biotin plus thiamine to thiamine alone in treating patients with biotin-responsive basal ganglia disease in an open-label prospective, comparative study. twenty patients with genetically proven biotin-responsive basal ganglia disease were enrolled, and received for at least 30 months a combination of biotin plus thiamine or thiamine alone. The outcome measures included duration of the crisis, number of recurrence/admissions, the last neurological examination, the severity of dystonia using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), and the brain MRI findings during the crisis and after 30 months of follow-up. Ten children with a mean age of 6 years(1/2) were recruited in the biotin plus thiamine group (group 1) and ten children (6 females and 4 males) with a mean age of 6 years and 2 months were recruited in the thiamine group (group 2). After 2 years of follow-up treatment, 6 of 20 children achieved complete remission, 10 had minimal sequelae in the form of mild dystonia and dysarthria (improvement of the BFMDRS, mean: 80%), and 4 had severe neurologic sequelae. All these 4 patients had delayed diagnosis and management. Regarding outcome measures, both groups have a similar outcome regarding the number of recurrences, the neurologic sequelae (mean BFMDS score between the groups, p = 0.84), and the brain MRI findings. The only difference was the duration of the acute crisis: group 1 had faster recovery (2 days), versus 3 days in group 2 (p = 0.005). Our study suggests that over 30 months of treatment, the combination of biotin plus thiamine is not superior to thiamine alone in the treatment of biotin-responsive basal ganglia disease. Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  3. Basal ganglia mechanisms underlying precision grip force control.

    Science.gov (United States)

    Prodoehl, Janey; Corcos, Daniel M; Vaillancourt, David E

    2009-06-01

    The classic grasping network has been well studied but thus far the focus has been on cortical regions in the control of grasping. Sub-cortically, specific nuclei of the basal ganglia have been shown to be important in different aspects of precision grip force control but these findings have not been well integrated. In this review, we outline the evidence to support the hypothesis that key basal ganglia nuclei are involved in parameterizing specific properties of precision grip force. We review literature from different areas of human and animal work that converges to build a case for basal ganglia involvement in the control of precision gripping. Following on from literature showing anatomical connectivity between the basal ganglia nuclei and key nodes in the cortical grasping network, we suggest a conceptual framework for how the basal ganglia could function within the grasping network, particularly as it relates to the control of precision grip force.

  4. A biophysical model of the cortex-basal ganglia-thalamus network in the 6-OHDA lesioned rat model of Parkinson's disease.

    Science.gov (United States)

    Kumaravelu, Karthik; Brocker, David T; Grill, Warren M

    2016-04-01

    Electrical stimulation of sub-cortical brain regions (the basal ganglia), known as deep brain stimulation (DBS), is an effective treatment for Parkinson's disease (PD). Chronic high frequency (HF) DBS in the subthalamic nucleus (STN) or globus pallidus interna (GPi) reduces motor symptoms including bradykinesia and tremor in patients with PD, but the therapeutic mechanisms of DBS are not fully understood. We developed a biophysical network model comprising of the closed loop cortical-basal ganglia-thalamus circuit representing the healthy and parkinsonian rat brain. The network properties of the model were validated by comparing responses evoked in basal ganglia (BG) nuclei by cortical (CTX) stimulation to published experimental results. A key emergent property of the model was generation of low-frequency network oscillations. Consistent with their putative pathological role, low-frequency oscillations in model BG neurons were exaggerated in the parkinsonian state compared to the healthy condition. We used the model to quantify the effectiveness of STN DBS at different frequencies in suppressing low-frequency oscillatory activity in GPi. Frequencies less than 40 Hz were ineffective, low-frequency oscillatory power decreased gradually for frequencies between 50 Hz and 130 Hz, and saturated at frequencies higher than 150 Hz. HF STN DBS suppressed pathological oscillations in GPe/GPi both by exciting and inhibiting the firing in GPe/GPi neurons, and the number of GPe/GPi neurons influenced was greater for HF stimulation than low-frequency stimulation. Similar to the frequency dependent suppression of pathological oscillations, STN DBS also normalized the abnormal GPi spiking activity evoked by CTX stimulation in a frequency dependent fashion with HF being the most effective. Therefore, therapeutic HF STN DBS effectively suppresses pathological activity by influencing the activity of a greater proportion of neurons in the output nucleus of the BG.

  5. Pharmacologic MRI (phMRI) as a tool to differentiate Parkinson's disease-related from age-related changes in basal ganglia function.

    Science.gov (United States)

    Andersen, Anders H; Hardy, Peter A; Forman, Eric; Gerhardt, Greg A; Gash, Don M; Grondin, Richard C; Zhang, Zhiming

    2015-02-01

    The prevalence of both parkinsonian signs and Parkinson's disease (PD) per se increases with age. Although the pathophysiology of PD has been studied extensively, less is known about the functional changes taking place in the basal ganglia circuitry with age. To specifically address this issue, 3 groups of rhesus macaques were studied: normal middle-aged animals (used as controls), middle-aged animals with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced parkinsonism, and aged animals (>20 years old) with declines in motor function. All animals underwent the same behavioral and pharmacologic magnetic resonance imaging (phMRI) procedures to measure changes in basal ganglia function in response to dopaminergic drug challenges consisting of apomorphine administration followed by either a D1 (SCH23390) or a D2 (raclopride) receptor antagonist. Significant functional changes were predominantly seen in the external segment of the globus pallidus (GPe) in aged animals and in the striatum (caudate nucleus and putamen) in MPTP-lesioned animals. Despite significant differences seen in the putamen and GPe between MPTP-lesioned versus aged animals, a similar response profile to dopaminergic stimulations was found between these 2 groups in the internal segment of the GP. In contrast, the pharmacologic responses seen in the control animals were much milder compared with the other 2 groups in all the examined areas. Our phMRI findings in MPTP-lesioned parkinsonian and aged animals suggest that changes in basal ganglia function in the elderly may differ from those seen in parkinsonian patients and that phMRI could be used to distinguish PD from other age-associated functional alterations in the brain. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Basal ganglia - thalamus and the crowning enigma

    Directory of Open Access Journals (Sweden)

    Marianela eGarcia-Munoz

    2015-11-01

    Full Text Available When Hubel (1982 referred to layer 1 of primary visual cortex as …a ‘crowning mystery’ to keep area-17 physiologists busy for years to come... he could have been talking about any cortical area. In the 80’s and 90’s there were no methods to examine this neuropile on the surface of the cortex: a tangled web of axons and dendrites from a variety of different places with unknown specificities and doubtful connections to the cortical output neurons some hundreds of microns below. Recently, three changes have made the crowning enigma less of an impossible mission: the clear presence of neurons in layer 1 (L1, the active conduction of voltage along apical dendrites and optogenetic methods that might allow us to look at one source of input at a time. For all of those reasons alone, it seems it is time to take seriously the function of L1. The functional properties of this layer will need to wait for more experiments but already L1 cells are GAD67 positive, i.e., inhibitory! They could reverse the sign of the thalamic glutamate (GLU input for the entire cortex. It is at least possible that in the near future normal activity of individual sources of L1 could be detected using genetic tools. We are at the outset of important times in the exploration of thalamic functions and perhaps the solution to the crowning enigma is within sight. Our review looks forward to that solution from the solid basis of the anatomy of the basal ganglia output to motor thalamus. We will focus on L1, its afferents, intrinsic neurons and its influence on responses of pyramidal neurons in layers 2/3 and 5. Since L1 is present in the whole cortex we will provide a general overview considering evidence mainly from the somatosensory cortex before focusing on motor cortex.

  7. Basal ganglia disorders studied by positron emission tomography

    International Nuclear Information System (INIS)

    Shinotoh, Hitoshi

    1994-01-01

    Recent development of positron emitting radioligands has made it possible to investigate the alterations of neurotransmitter systems associated with basal ganglia disorders in vivo. The functional integrity of nigro-striatal dopaminergic terminals may be studied with [ 18 F]6-fluoro-L-dopa ([ 18 F]dopa), and striatal dopamine receptor density with suitable PET ligands. [ 18 F]dopa uptake in the striatum (putamen) is markedly reduced in patients with Parkinson's disease (PD). [ 18 F]dopa-PET is capable of detecting sub-clinical nigral dysfunction in asymptomatic patients with familial PD and those who become Parkinsonian on conventional doses of dopamine receptor antagonists. While putamen [ 18 F]dopa uptake is reduced to a similar level in patients with multiple system atrophy (MSA) and PD, caudate [ 18 F] dopa uptake is lower in MSA than PD. However, [ 18 F]dopa PET cannot consistently distinguish MSA from PD because individual ranges of caudate [ 18 F]dopa uptake overlap. D 1 and D 2 receptor binding is markedly reduced in the striatum (posterior putamen) of MSA patients. Therefore, dopamine receptor imaging is useful for the differential diagnosis of MSA and PD. Similar marked reductions in putamen and caudate [ 18 F]dopa uptake have been observed in patients with progressive supranuclear palsy (PSP). Moderate reductions in D 2 receptor binding have been reported in the striatum of PSP patients. The reduction in D 2 receptor binding is more prominent in the caudate than putamen. Striatal [ 18 F]dopa uptake is normal or only mildly reduced in patients with dopa responsive dystonia (DRD). D 2 receptor binding is markedly reduced in patients with Huntington's disease, while striatal [ 18 F]dopa uptake is normal or mildly reduced. In summary, PET can demonstrate characteristic patterns of disruption of dopaminergic systems associated with basal ganglia disorders. These PET findings are useful in the differential diagnosis of basal ganglia disorders. (J.P.N.) 55 refs

  8. Information processing, dimensionality reduction and reinforcement learning in the basal ganglia.

    Science.gov (United States)

    Bar-Gad, Izhar; Morris, Genela; Bergman, Hagai

    2003-12-01

    Modeling of the basal ganglia has played a major role in our understanding of this elusive group of nuclei. Models of the basal ganglia have undergone evolutionary and revolutionary changes over the last 20 years, as new research in the fields of anatomy, physiology and biochemistry of these nuclei has yielded new information. Early models dealt with a single pathway through the nuclei and focused on the nature of the processing performed within it, convergence of information versus parallel processing of information. Later, the Albin-DeLong "box-and-arrow" model characterized the inter-nuclei interaction as multiple pathways while maintaining a simplistic scalar representation of the nuclei themselves. This model made a breakthrough by providing key insights into the behavior of these nuclei in hypo- and hyper-kinetic movement disorders. The next generation of models elaborated the intra-nuclei interactions and focused on the role of the basal ganglia in action selection and sequence generation which form the most current consensus regarding basal ganglia function in both normal and pathological conditions. However, new findings challenge these models and point to a different neural network approach to information processing in the basal ganglia. Here, we take an in-depth look at the reinforcement driven dimensionality reduction (RDDR) model which postulates that the basal ganglia compress cortical information according to a reinforcement signal using optimal extraction methods. The model provides new insights and experimental predictions on the computational capacity of the basal ganglia and their role in health and disease.

  9. Basal ganglia calcification on computed tomography in systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Nagaoka, Shohei; Tani, Kenji; Ishigatsubo, Yoshiaki

    1988-01-01

    The development of basal ganglia calcification was studied in 85 patients with systemic lupus erythematosus (SLE) by computed tomography (CT). Bilateral calcification of the basal ganglia was found to occur in 5 patients (5.9 %) with SLE, but was not seen in patients with rheumatoid arthritis and progressive systemic sclerosis. All were female with a mean age of 42 years (range 29 - 49). The patients with calcification of the basal ganglia had neurological symptoms, such as psychiatric problems (3 cases), grand mal seizures (1 case), CSF abnormalities (2 cases), and EEG changes (4 cases). There were significantly higher incidences of alopecia, cutaneous vasculitis, leukopenia, and thrombocytopenia in the group with calcifications than those in the group with normal CT findings. Circulating immune complexes were detected and LE tests were positive in 2 patients. Endocrinological examination showed no abnormality in any. We suggest that basal ganglia calcification in SLE might be related to cerebral vasculitis. (author)

  10. Basal ganglia calcification on computed tomography in systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Nagaoka, Shohei; Tani, Kenji; Ishigatsubo, Yoshiaki and others

    1988-09-01

    The development of basal ganglia calcification was studied in 85 patients with systemic lupus erythematosus (SLE) by computed tomography (CT). Bilateral calcification of the basal ganglia was found to occur in 5 patients (5.9 %) with SLE, but was not seen in patients with rheumatoid arthritis and progressive systemic sclerosis. All were female with a mean age of 42 years (range 29 - 49). The patients with calcification of the basal ganglia had neurological symptoms, such as psychiatric problems (3 cases), grand mal seizures (1 case), CSF abnormalities (2 cases), and EEG changes (4 cases). There were significantly higher incidences of alopecia, cutaneous vasculitis, leukopenia, and thrombocytopenia in the group with calcifications than those in the group with normal CT findings. Circulating immune complexes were detected and LE tests were positive in 2 patients. Endocrinological examination showed no abnormality in any. We suggest that basal ganglia calcification in SLE might be related to cerebral vasculitis.

  11. Bilateral basal ganglia calcifications visualised on CT scan.

    OpenAIRE

    Brannan, T S; Burger, A A; Chaudhary, M Y

    1980-01-01

    Thirty-eight cases of basal ganglia calcification imaged on computed axial tomography were reviewed. Most cases were felt to represent senescent calcification. The possibility of a vascular aetiology in this group is discussed. A less common group of patients was identified with calcification secondary to abnormalities in calcium metabolism or radiation therapy. Three cases of basal ganglia calcifications were detected in juvenile epileptic patients receiving chronic anticonvulsants. These ca...

  12. Basal Ganglia Mechanisms Underlying Precision Grip Force Control

    OpenAIRE

    Prodoehl, Janey; Corcos, Daniel M.; Vaillancourt, David E.

    2009-01-01

    The classic grasping network has been well studied but thus far the focus has been on cortical regions in the control of grasping. Sub-cortically, specific nuclei of the basal ganglia have been shown to be important in different aspects of precision grip force control but these findings have not been well integrated. In this review we outline the evidence to support the hypothesis that key basal ganglia nuclei are involved in parameterizing specific properties of precision grip force. We revi...

  13. CT and MRI diagnosis of traumatic basal ganglia hemorrhage

    International Nuclear Information System (INIS)

    Wu Shike; Zhang Yalin; Xu Derong; Zou Gaowei; Chen Dan; He Sujun; Zhou Lichao

    2009-01-01

    Objective: To analyze CT and MRI features of traumatic basal ganglia hemorrhage and investigate the diagnostic value. Methods: 21 cases with traumatic basal ganglia hemorrhage diagnosed by clinic, CT and MRI in our hospital were collected in this study Plain CT scan were immediately performed in 21 cases after injury, plain MR scan were performed in 1 to 3 days. 12 cases of them underwent diffusion weighted imagine (DWI). The CT and MRI findings were retrospectively summarized. Results: 8 cases were found with simple traumatic basal ganglia hemorrhage. Complexity of basal ganglia hemorrhage occurred in 13 cases, 6 cases combined with subdural hemorrhage, 3 cases with epidural hematoma, 2 cases with subarachnoid hemorrhage, 6 cases with brain contusion and laceration in other locations, 4 cases with skull fracture. 26 lesions of basal ganglia hematoma were showed in 21 cases, 14 lesions of pallidum hemorrhage in 11 cases confirmed by MR could not be distinguished from calcification at the fast CT scan. 5 more lesions of brain contusion and laceration and 4 more lesions of brain white matter laceration were found by MR. Conclusion: CT in combination with MRI can diagnose traumatic basal ganglia hemorrhage and its complications early, comprehensively and accurately, which plays an important role in the clinical therapy selection and prognosis evaluation. (authors)

  14. Adult-onset Alexander disease with typical "tadpole" brainstem atrophy and unusual bilateral basal ganglia involvement: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Sakoe Kumi

    2010-04-01

    Full Text Available Abstract Background Alexander disease (ALX is a rare neurological disorder characterized by white matter degeneration and cytoplasmic inclusions in astrocytes called Rosenthal fibers, labeled by antibodies against glial fibrillary acidic protein (GFAP. Three subtypes are distinguished according to age at onset: infantile (under age 2, juvenile (age 2 to 12 and adult (over age 12. Following the identification of heterozygous mutations in GFAP that cause this disease, cases of adult-onset ALX have been increasingly reported. Case Presentation We present a 60-year-old Japanese man with an unremarkable past and no family history of ALX. After head trauma in a traffic accident at the age of 46, his character changed, and dementia and dysarthria developed, but he remained independent. Spastic paresis and dysphagia were observed at age 57 and 59, respectively, and worsened progressively. Neurological examination at the age of 60 revealed dementia, pseudobulbar palsy, left-side predominant spastic tetraparesis, axial rigidity, bradykinesia and gaze-evoked nystagmus. Brain MRI showed tadpole-like atrophy of the brainstem, caused by marked atrophy of the medulla oblongata, cervical spinal cord and midbrain tegmentum, with an intact pontine base. Analysis of the GFAP gene revealed a heterozygous missense mutation, c.827G>T, p.R276L, which was already shown to be pathogenic in a case of pathologically proven hereditary adult-onset ALX. Conclusion The typical tadpole-like appearance of the brainstem is strongly suggestive of adult-onset ALX, and should lead to a genetic investigation of the GFAP gene. The unusual feature of this patient is the symmetrical involvement of the basal ganglia, which is rarely observed in the adult form of the disease. More patients must be examined to confirm, clinically and neuroradiologically, extrapyramidal involvement of the basal ganglia in adult-onset ALX.

  15. Survey of 37 cases with basal ganglia calcification

    International Nuclear Information System (INIS)

    Ogata, Akira; Ishida, Shiro; Wada, Toyoji

    1984-01-01

    Of 5,987 patients (5,196 epileptic patients and 791 non-epileptic patients) undergoing CT-scan, calcification of the bilateral basal ganglia was detected in 28 epileptic patients and 9 non-epileptic patients. Relationship among CT-scan findings, underlying diseases and epilepsy was studied. CT-scan findings could be classified into the localized type in which calcification was limited to the globus pallidus in 33 patients and diffuse type in which it extended to the putamen in 4 patients. Localized type increased with age. It was about 70 times higher in patients above the age of 50 than in patients below the age of 10. The female to male ratio was 2:1. Underlying diseases were idiopathic or familial in cases of localized type, and hypoparathyroidism in cases of diffuse type. Seventy-five percent of the epileptic patients had partial epilepsy. There seems not to be direct correlation between basal ganglia calcification and epilepsy. (Namekawa, K)

  16. Fos expression following activation of the ventral pallidum in normal rats and in a model of Parkinson's Disease: implications for limbic system and basal ganglia interactions.

    Science.gov (United States)

    Turner, Michael S; Gray, Thackery S; Mickiewicz, Amanda L; Napier, T Celeste

    2008-09-01

    The circuit-related consequences of activating the ventral pallidum (VP) are not well known, and lacking in particular is how these effects are altered in various neuropathological states. To help to address these paucities, this study investigated the brain regions affected by VP activation by quantifying neurons that stain for Fos-like immunoreactivity (ir). Fos-ir was assessed after intra-pallidal injections of the excitatory amino acid agonist, NMDA, or the GABA(A) antagonist, bicuculline in normal rats and in those rendered Parkinsonian-like by lesioning dopaminergic neurons with the neurotoxin, 6-OHDA. We hypothesized that activation of the VP will alter the activity state of brain regions associated with both the basal ganglia and limbic system, and that this influence would be modified in the Parkinsonian state. Blocking tonically activated GABA(A) receptors with bicuculline (50 ng/0.5 microl) elevated Fos-ir in the VP to 423% above the contralateral, vehicle-injected side. Likewise, intra-VP NMDA (0.23 microg or 0.45 microg/0.5 microl), dose-dependently increased the number of pallidal neurons expressing Fos-ir by 224 and 526%, respectively. At higher NMDA doses, the density of Fos-ir neurons was not elevated above control levels. This inverted U-shaped profile was mirrored by a VP output structure, the medial subthalamic nucleus (mSTN). The mSTN showed a 289% increase in Fos-ir neurons with intra-VP injections of 0.45 microg NMDA, and this response was halved following intra-VP injections of 0.9 microg NMDA. Of the 12 other brain regions measured, three showed VP NMDA-induced enhancements in Fos-ir: the frontal cortex, entopeduncular nucleus and substantia nigra pars reticulata, all regions associated with the basal ganglia. In a second study, we evaluated the NMDA activation profile in a rat model of Parkinson's Disease (PD) which was created by a unilateral injection of 6-OHDA into the rostral substantia nigra pars compacta. Comparisons of responses to

  17. Altered neuronal firing pattern of the basal ganglia nucleus plays a role in levodopa-induced dyskinesia in patients with Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Xiaoyu eLi

    2015-11-01

    Full Text Available Background: Levodopa therapy alleviates the symptoms of Parkinson's disease (PD, but long-term treatment often leads to motor complications such as levodopa-induced dyskinesia (LID. Aim: To explore the neuronal activity in the basal ganglia nuclei in patients with PD and LID. Methods: Thirty patients with idiopathic PD (age, 55.1±11.0 years; disease duration, 8.7±5.6 years were enrolled between August 2006 and August 2013 at the Xuanwu Hospital, Capital Medical University, China. Their Hoehn and Yahr scores ranged from 2 to 4 and their UPDRS III scores were 28.5±5.2. Fifteen of them had severe LID (UPDRS IV scores of 6.7±1.6. Microelectrode recording was performed in the globus pallidus internus (GPi and subthalamic nucleus (STN during pallidotomy (n=12 or STN deep brain stimulation (DBS; bilateral, n=12; unilateral, n=6. The firing patterns and frequencies of various cell types were analyzed by assessing single cell interspike intervals (ISIs and the corresponding coefficient of variation (CV. Results: A total of 295 neurons were identified from the GPi (n=12 and STN (n=18. These included 26 (8.8% highly grouped discharge, 30 (10.2% low frequency firing, 78 (26.4% rapid tonic discharge, 103 (34.9% irregular activity, and 58 (19.7% tremor-related activity. There were significant differences between the two groups (P<0.05 for neurons with irregular firing, highly irregular cluster-like firing, and low-frequency firing. Conclusion: Altered neuronal activity was observed in the basal ganglia nucleus of GPi and STN, and may play important roles in the pathophysiology of PD and LID.

  18. Influence of basal ganglia on upper limb locomotor synergies. Evidence from deep brain stimulation and L-DOPA treatment in Parkinson's disease.

    Science.gov (United States)

    Crenna, P; Carpinella, I; Lopiano, L; Marzegan, A; Rabuffetti, M; Rizzone, M; Lanotte, M; Ferrarin, M

    2008-12-01

    Clinical evidence of impaired arm swing while walking in patients with Parkinson's disease suggests that basal ganglia and related systems play an important part in the control of upper limb locomotor automatism. To gain more information on this supraspinal influence, we measured arm and thigh kinematics during walking in 10 Parkinson's disease patients, under four conditions: (i) baseline (no treatment), (ii) therapeutic stimulation of the subthalamic nucleus (STN), (iii)L-DOPA medication and (iv) combined STN stimulation and L-DOPA. Ten age-matched controls provided reference data. Under baseline conditions the range of patients' arm motion was severely restricted, with no correlation with the excursion of the thigh. In addition, the arm swing was abnormally coupled in time with oscillation of the ipsilateral thigh. STN stimulation significantly increased the gait speed and improved the spatio-temporal parameters of arm and thigh motion. The kinematic changes as a function of gait speed changes, however, were significantly smaller for the upper than the lower limb, in contrast to healthy controls. Arm motion was also less responsive after L-DOPA. Simultaneous deep brain stimulation and L-DOPA had additive effects on thigh motion, but not on arm motion and arm-thigh coupling. The evidence that locomotor automatisms of the upper and lower limbs display uncorrelated impairment upon dysfunction of the basal ganglia, as well as different susceptibility to electrophysiological and pharmacological interventions, points to the presence of heterogeneously distributed, possibly partially independent, supraspinal control channels, whereby STN and dopaminergic systems have relatively weaker influence on the executive structures involved in the arm swing and preferential action on those for lower limb movements. These findings might be considered in the light of phylogenetic changes in supraspinal control of limb motion related to primate bipedalism.

  19. Basal ganglia, movement disorders and deep brain stimulation: advances made through non-human primate research.

    Science.gov (United States)

    Wichmann, Thomas; Bergman, Hagai; DeLong, Mahlon R

    2018-03-01

    Studies in non-human primates (NHPs) have led to major advances in our understanding of the function of the basal ganglia and of the pathophysiologic mechanisms of hypokinetic movement disorders such as Parkinson's disease and hyperkinetic disorders such as chorea and dystonia. Since the brains of NHPs are anatomically very close to those of humans, disease states and the effects of medical and surgical approaches, such as deep brain stimulation (DBS), can be more faithfully modeled in NHPs than in other species. According to the current model of the basal ganglia circuitry, which was strongly influenced by studies in NHPs, the basal ganglia are viewed as components of segregated networks that emanate from specific cortical areas, traverse the basal ganglia, and ventral thalamus, and return to the frontal cortex. Based on the presumed functional domains of the different cortical areas involved, these networks are designated as 'motor', 'oculomotor', 'associative' and 'limbic' circuits. The functions of these networks are strongly modulated by the release of dopamine in the striatum. Striatal dopamine release alters the activity of striatal projection neurons which, in turn, influences the (inhibitory) basal ganglia output. In parkinsonism, the loss of striatal dopamine results in the emergence of oscillatory burst patterns of firing of basal ganglia output neurons, increased synchrony of the discharge of neighboring basal ganglia neurons, and an overall increase in basal ganglia output. The relevance of these findings is supported by the demonstration, in NHP models of parkinsonism, of the antiparkinsonian effects of inactivation of the motor circuit at the level of the subthalamic nucleus, one of the major components of the basal ganglia. This finding also contributed strongly to the revival of the use of surgical interventions to treat patients with Parkinson's disease. While ablative procedures were first used for this purpose, they have now been largely

  20. Past, present and future of the pathophysiological model of the basal ganglia

    Directory of Open Access Journals (Sweden)

    Jose A Obeso

    2011-07-01

    Full Text Available The current model of basal ganglia was introduced two decades ago and has settled most of our current understanding of basal ganglia function and dysfunction. Extensive research efforts have been carried out in recent years leading to further refinement and understanding of the normal and diseased basal ganglia. Several questions, however, are yet to be resolved. This short review provides a synopsis of the evolution of thought regarding the pathophysiological model of the BG and summarizes the main recent findings and additions to this field of research. We have also tried to identify major challenges that need to be addressed and resolved in the near future. Detailed accounts and state-of-the-art developments concerning research on the basal ganglia are provided in the articles that make up this Special Issue.

  1. Bilateral symmetrical basal ganglia and thalamic lesions in children: an update (2015)

    Energy Technology Data Exchange (ETDEWEB)

    Zuccoli, Giulio [Children' s Hospital of Pittsburgh of UPMC, Section of Neuroradiology, Pittsburgh, PA (United States); Yannes, Michael Paul [University of Pittsburgh School of Medicine, Department of Radiology, Pittsburgh, PA (United States); Nardone, Raffaele [Paracelsus Medical University, Department of Neurology, Christian Doppler Klinik, Salzburg (Austria); Bailey, Ariel [West Virginia University, Department of Radiology, Morgantown, WV (United States); Goldstein, Amy [Children' s Hospital of Pittsburgh of UPMC, Department of Neurology, Section of Metabolic Disorders and Neurogenetics, Pittsburgh, PA (United States)

    2015-10-15

    In children, many inherited or acquired neurological disorders may cause bilateral symmetrical signal intensity alterations in the basal ganglia and thalami. A literature review was aimed at assisting neuroradiologists, neurologists, infectious diseases specialists, and pediatricians to provide further understanding into the clinical and neuroimaging features in pediatric patients presenting with bilateral symmetrical basal ganglia and thalamic lesions on magnetic resonance imaging (MRI). We discuss hypoxic-ischemic, toxic, infectious, immune-mediated, mitochondrial, metabolic, and neurodegenerative disorders affecting the basal ganglia and thalami. Recognition and correct evaluation of basal ganglia abnormalities, together with a proper neurological examination and laboratory findings, may enable the identification of each of these clinical entities and lead to earlier diagnosis. (orig.)

  2. Bilateral symmetrical basal ganglia and thalamic lesions in children: an update (2015)

    International Nuclear Information System (INIS)

    Zuccoli, Giulio; Yannes, Michael Paul; Nardone, Raffaele; Bailey, Ariel; Goldstein, Amy

    2015-01-01

    In children, many inherited or acquired neurological disorders may cause bilateral symmetrical signal intensity alterations in the basal ganglia and thalami. A literature review was aimed at assisting neuroradiologists, neurologists, infectious diseases specialists, and pediatricians to provide further understanding into the clinical and neuroimaging features in pediatric patients presenting with bilateral symmetrical basal ganglia and thalamic lesions on magnetic resonance imaging (MRI). We discuss hypoxic-ischemic, toxic, infectious, immune-mediated, mitochondrial, metabolic, and neurodegenerative disorders affecting the basal ganglia and thalami. Recognition and correct evaluation of basal ganglia abnormalities, together with a proper neurological examination and laboratory findings, may enable the identification of each of these clinical entities and lead to earlier diagnosis. (orig.)

  3. Fiber tractography of the axonal pathways linking the basal ganglia and cerebellum in Parkinson disease: implications for targeting in deep brain stimulation.

    Science.gov (United States)

    Sweet, Jennifer A; Walter, Benjamin L; Gunalan, Kabilar; Chaturvedi, Ashutosh; McIntyre, Cameron C; Miller, Jonathan P

    2014-04-01

    Stimulation of white matter pathways near targeted structures may contribute to therapeutic effects of deep brain stimulation (DBS) for patients with Parkinson disease (PD). Two tracts linking the basal ganglia and cerebellum have been described in primates: the subthalamopontocerebellar tract (SPCT) and the dentatothalamic tract (DTT). The authors used fiber tractography to evaluate white matter tracts that connect the cerebellum to the region of the basal ganglia in patients with PD who were candidates for DBS. Fourteen patients with advanced PD underwent 3-T MRI, including 30-directional diffusion-weighted imaging sequences. Diffusion tensor tractography was performed using 2 regions of interest: ipsilateral subthalamic and red nuclei, and contralateral cerebellar hemisphere. Nine patients underwent subthalamic DBS, and the course of each tract was observed relative to the location of the most effective stimulation contact and the volume of tissue activated. In all patients 2 distinct tracts were identified that corresponded closely to the described anatomical features of the SPCT and DTT, respectively. The mean overall distance from the active contact to the DTT was 2.18 ± 0.35 mm, and the mean proportional distance relative to the volume of tissue activated was 1.35 ± 0.48. There was a nonsignificant trend toward better postoperative tremor control in patients with electrodes closer to the DTT. The SPCT and the DTT may be related to the expression of symptoms in PD, and this may have implications for DBS targeting. The use of tractography to identify the DTT might assist with DBS targeting in the future.

  4. The pedunculopontine tegmental nucleus as a motor and cognitive interface between the cerebellum and basal ganglia

    Directory of Open Access Journals (Sweden)

    Fumika Mori

    2016-11-01

    Full Text Available As an important component of ascending activating systems, brainstem cholinergic neurons in the pedunculopontine tegmental nucleus (PPTg are involved in the regulation of motor control (locomotion, posture and gaze and cognitive processes (attention, learning, and memory. The PPTg is highly interconnected with several regions of the basal ganglia, and one of its key functions is to regulate and relay activity from the basal ganglia. Together, they have been implicated in the motor control system (such as voluntary movement initiation or inhibition, and modulate aspects of executive function (such as motivation. In addition to its intimate connection with the basal ganglia, projections from the PPTg to the cerebellum have been recently reported to synaptically activate the deep cerebellar nuclei. Classically, the cerebellum and basal ganglia were regarded as forming separated anatomical loops that play a distinct functional role in motor and cognitive behavioral control. Here, we suggest that the PPTg may also act as an interface device between the basal ganglia and cerebellum. As such, part of the therapeutic effect of PPTg deep brain stimulation to relieve gait freezing and postural instability in advanced Parkinson’s disease patients might also involve modulation of the cerebellum. We review the anatomical position and role of the PPTg in the pathway of basal ganglia and cerebellum in relation to motor control, cognitive function, and Parkinson’s disease.

  5. Electrophysiological Evidences of Organization of Cortical Motor Information in the Basal Ganglia

    Directory of Open Access Journals (Sweden)

    Hirokazu Iwamuro

    2011-05-01

    Full Text Available During the last two decades, the many developments in the treatment of movement disorders such as Parkinson disease and dystonia have enhanced our understanding on organization of the basal ganglia, and this knowledge has led to other advances in the field. According to many electrophysiological and anatomical findings, it is considered that motor information from different cortical areas is processed through several cortico-basal ganglia loops principally in a parallel fashion and somatotopy from each cortical area is also well preserved in each loop. Moreover, recent studies suggest that not only the parallel processing but also some convergence of information occur through the basal ganglia. Information from cortical areas whose functions are close to each other tends to converge in the basal ganglia. The cortico-basal ganglia loops should be comprehended more as a network rather than as separated subdivisions. However, the functions of this convergence still remain unknown. It is important even for clinical doctors to be well informed about this kind of current knowledge because some symptoms of movement disorders may be explained by disorganization of the information network in the basal ganglia.

  6. Deep Brain Stimulation for Movement Disorders of Basal Ganglia Origin: Restoring Function or Functionality?

    Science.gov (United States)

    Wichmann, Thomas; DeLong, Mahlon R

    2016-04-01

    Deep brain stimulation (DBS) is highly effective for both hypo- and hyperkinetic movement disorders of basal ganglia origin. The clinical use of DBS is, in part, empiric, based on the experience with prior surgical ablative therapies for these disorders, and, in part, driven by scientific discoveries made decades ago. In this review, we consider anatomical and functional concepts of the basal ganglia relevant to our understanding of DBS mechanisms, as well as our current understanding of the pathophysiology of two of the most commonly DBS-treated conditions, Parkinson's disease and dystonia. Finally, we discuss the proposed mechanism(s) of action of DBS in restoring function in patients with movement disorders. The signs and symptoms of the various disorders appear to result from signature disordered activity in the basal ganglia output, which disrupts the activity in thalamocortical and brainstem networks. The available evidence suggests that the effects of DBS are strongly dependent on targeting sensorimotor portions of specific nodes of the basal ganglia-thalamocortical motor circuit, that is, the subthalamic nucleus and the internal segment of the globus pallidus. There is little evidence to suggest that DBS in patients with movement disorders restores normal basal ganglia functions (e.g., their role in movement or reinforcement learning). Instead, it appears that high-frequency DBS replaces the abnormal basal ganglia output with a more tolerable pattern, which helps to restore the functionality of downstream networks.

  7. Homologous Basal Ganglia Network Models in Physiological and Parkinsonian Conditions

    Directory of Open Access Journals (Sweden)

    Jyotika Bahuguna

    2017-08-01

    diversity in basal ganglia networks. We propose that our approach of generating and analyzing an ensemble of multiple solutions to an underdetermined network model provides greater confidence in its predictions than those derived from a unique solution, and that projecting such homologous networks on a lower dimensional space of sensibly chosen dynamical features gives a better chance than a purely structural analysis at understanding complex pathologies such as Parkinson's disease.

  8. Hyperkinetic mutism: bilateral ballism and basal ganglia calcification.

    Science.gov (United States)

    Inbody, S; Jankovic, J

    1986-06-01

    We studied a 70-year-old woman with a unique combination of hyperkinesia and mutism. These findings differed from akinetic mutism because there was continuous bilateral ballism and dystonia--hence the term "hyperkinetic mutism." CT demonstrated bilateral calcifications in the basal ganglia, and MRI indicated bilateral watershed infarcts. Different dopaminergic mechanisms may underlie the hyperkinesia and mutism.

  9. Substrates for normal gait and pathophysiology of gait disturbances with respect to the basal ganglia dysfunction.

    Science.gov (United States)

    Takakusaki, Kaoru; Tomita, Nozomi; Yano, Masafumi

    2008-08-01

    In this review, we have tried to elucidate substrates for the execution of normal gait and to understand pathophysiological mechanisms of gait failure in basal ganglia dysfunctions. In Parkinson's disease, volitional and emotional expressions of movement processes are seriously affected in addition to the disturbance of automatic movement processes, such as adjustment of postural muscle tone before gait initiation and rhythmic limb movements during walking. These patients also suffer from muscle tone rigidity and postural instability, which may also cause reduced walking capabilities in adapting to various environments. Neurophysiological and clinical studies have suggested the importance of basal ganglia connections with the cerebral cortex and limbic system in the expression of volitional and emotional behaviors. Here we hypothesize a crucial role played by the basal ganglia-brainstem system in the integrative control of muscle tone and locomotion. The hypothetical model may provide a rational explanation for the role of the basal ganglia in the control of volitional and automatic aspects of movements. Moreover, it might also be beneficial for understanding pathophysiological mechanisms of basal ganglia movement disorders. A part of this hypothesis has been supported by studies utilizing a constructive simulation engineering technique that clearly shows that an appropriate level of postural muscle tone and proper acquisition and utilization of sensory information are essential to maintain adaptable bodily functions for the full execution of bipedal gait. In conclusion, we suggest that the major substrates for supporting bipedal posture and executing bipedal gait are 1) fine neural networks such as the cortico-basal ganglia loop and basal ganglia-brainstem system, 2) fine musculoskeletal structures with adequately developed (postural) muscle tone, and 3) proper sensory processing. It follows that any dysfunction of the above sensorimotor integration processes

  10. Neuropathological characteristics of the brain in two patients with SLC19A3 mutations related to the biotin-thiamine-responsive basal ganglia disease

    Directory of Open Access Journals (Sweden)

    Maciej Pronicki

    2017-06-01

    Full Text Available Biotin-thiamine-responsive basal ganglia disease is a severe form of a rare neurogenetic disorder caused by pathogenic molecular variants in the thiamine transporter gene. Nowadays, a potentially effective treatment is known, therefore the early diagnosis is mandatory. The aim of the paper was to assess the contribution of neuropathological and magnetic resonance imaging (MRI studies to a proper diagnosis. We present the brain study of two Polish patients with SLC19A3 mutations, including (1 an infant with an intriguing “walnut” appearance of the brain autopsied many years before the discovery of the SLC19A3 defect, and (2 a one-year-old patient with clinical features of Leigh syndrome. In patient 2, biotin/thiamine responsiveness was not tested at the time of diagnosis and causal treatment started with one-year delay. The central nervous system lesions found in the patients displayed almost clearly a specific pattern for SLC19A3 defect, as previously proposed in diagnostic criteria. Our study presents a detailed description of neuropathological and MRI findings of both patients. We confirm that the autopsy and/or MRI of the brain is sufficient to qualify a patient with an unknown neuropathological disorder directly for SLC19A3 mutations testing and a prompt trial of specific treatment.

  11. Single-photon-emission-computed-tomography (SPECT) in basal ganglia disorders

    International Nuclear Information System (INIS)

    Tatsch, K.

    1997-01-01

    In the past, SPECT investigations of regional cerebral blood flow have played a minor role in the diagnostic work-up of patients with basal ganglia disorders. More recently, however, interest in nuclear medicine procedures has dramatically increased since with the development of selective receptor ligands diagnostic tools have been provided which address the pathology in basal ganglia disorders more specifically than other diagnostic modalities. Evaluations of the pre- and postsynaptic aspects of the dopaminergic system, for example, deliver not only interesting data from the scientific point of view but also for the daily routine work. This paper summarizes some of the experience reported in the literature on SPECT investigations in basal ganglia disorders, such as Parkinson's disease, parkinsonian syndromes of other etiology, Wilson's and Huntington's disease, focal dystonias, and schizophrenia under treatment with neuroleptics. (orig.) [de

  12. Correlation of dopaminergic terminal dysfunction and microstructural abnormalities of the basal ganglia and the olfactory tract in Parkinson's disease.

    Science.gov (United States)

    Scherfler, Christoph; Esterhammer, Regina; Nocker, Michael; Mahlknecht, Philipp; Stockner, Heike; Warwitz, Boris; Spielberger, Sabine; Pinter, Bernadette; Donnemiller, Eveline; Decristoforo, Clemens; Virgolini, Irene; Schocke, Michael; Poewe, Werner; Seppi, Klaus

    2013-10-01

    Signal abnormalities of the substantia nigra and the olfactory tract detected either by diffusion tensor imaging, including measurements of mean diffusivity, a parameter of brain tissue integrity, and fractional anisotropy, a parameter of neuronal fibre integrity, or transcranial sonography, were recently reported in the early stages of Parkinson's disease. In this study, changes in the nigral and olfactory diffusion tensor signal, as well as nigral echogenicity, were correlated with clinical scales of motor disability, odour function and putaminal dopamine storage capacity measured with 6-[(18)F] fluorolevodopa positron emission tomography in early and advanced stages of Parkinson's disease. Diffusion tensor imaging, transcranial sonography and positron emission tomography were performed on 16 patients with Parkinson's disease (mean disease duration 3.7 ± 3.7 years, Hoehn and Yahr stage 1 to 4) and 14 age-matched healthy control subjects. Odour function was measured by the standardized Sniffin' Sticks Test. Mean putaminal 6-[(18)F] fluorolevodopa influx constant, mean nigral echogenicity, mean diffusivity and fractional anisotropy values of the substantia nigra and the olfactory tract were identified by region of interest analysis. When compared with the healthy control group, the Parkinson's disease group showed significant signal changes in the caudate and putamen by 6-[(18)F] fluorolevodopa positron emission tomography, in the substantia nigra by transcranial sonography, mean diffusivity and fractional anisotropy (P Parkinson's Disease Rating Scale motor score (r = -0.48, P Parkinson's Disease Rating Scale motor score (r = -0.53, P Parkinson's disease. Since increases in nigral mean diffusivity signal also correlated with motor dysfunction, diffusion tensor imaging may serve as a surrogate marker for disease progression in future studies of putative disease modifying therapies.

  13. Do basal Ganglia amplify willed action by stochastic resonance? A model.

    Directory of Open Access Journals (Sweden)

    V Srinivasa Chakravarthy

    Full Text Available Basal ganglia are usually attributed a role in facilitating willed action, which is found to be impaired in Parkinson's disease, a pathology of basal ganglia. We hypothesize that basal ganglia possess the machinery to amplify will signals, presumably weak, by stochastic resonance. Recently we proposed a computational model of Parkinsonian reaching, in which the contributions from basal ganglia aid the motor cortex in learning to reach. The model was cast in reinforcement learning framework. We now show that the above basal ganglia computational model has all the ingredients of stochastic resonance process. In the proposed computational model, we consider the problem of moving an arm from a rest position to a target position: the two positions correspond to two extrema of the value function. A single kick (a half-wave of sinusoid, of sufficiently low amplitude given to the system in resting position, succeeds in taking the system to the target position, with high probability, only at a critical noise level. But for suboptimal noise levels, the model arm's movements resemble Parkinsonian movement symptoms like akinetic rigidity (low noise and dyskinesias (high noise.

  14. Sensitivity analysis of discharge patterns of subthalamic nucleus in the model of basal ganglia in Parkinson disease.

    Science.gov (United States)

    Singh, Jyotsna; Singh, Phool; Malik, Vikas

    2017-01-01

    Parkinson disease alters the information patterns in movement related pathways in brain. Experimental results performed on rats show that the activity patterns changes from single spike activity to mixed burst mode in Parkinson disease. However the cause of this change in activity pattern is not yet completely understood. Subthalamic nucleus is one of the main nuclei involved in the origin of motor dysfunction in Parkinson disease. In this paper, a single compartment conductance based model is considered which focuses on subthalamic nucleus and synaptic input from globus pallidus (external). This model shows highly nonlinear behavior with respect to various intrinsic parameters. Behavior of model has been presented with the help of activity patterns generated in healthy and Parkinson condition. These patterns have been compared by calculating their correlation coefficient for different values of intrinsic parameters. Results display that the activity patterns are very sensitive to various intrinsic parameters and calcium shows some promising results which provide insights into the motor dysfunction.

  15. Infant who had chorea-athetotic movement and psychomotor deterioration associated with the low density area in the bilateral cerebral basal ganglia on CT. Comparison with analogous diseases

    Energy Technology Data Exchange (ETDEWEB)

    Tojo, Megumu; Matsui, Akira; Sakuragawa, Norio; Hirayama, Yoshito; Arima, Masataka

    1984-10-01

    A 6-year-old girl with convulsive tetraplegia and chorea-athetotic movement was reported. Since the age of one year, psychomotor retardation had begun to occur and CT showed a low density area in the putamen. At the age of 3 years and 6 months, psychomotor deterioration occurred subsequently to varicella. An abnormality in carbohydrate metabolism was suspected because of a slightly increased lactic acid and pyruvic acid. Because CT showed a low density area in the cerebral basal ganglia, juvenile Lee's encephalopathy and striatal necrosis remained to be ruled out. (Namekawa, K.).

  16. Neural activity in the rat basal ganglia

    NARCIS (Netherlands)

    Zhao, Yan; Stegenga, J.; Heida, Tjitske; van Wezel, Richard Jack Anton

    2013-01-01

    Objectives: Pathological oscillations in the beta frequencies (8-30Hz) have been found in the local field potentials of Parkinson's disease (PD) patients and non-human primate models of PD1. In particular, these synchronizations appear in the subthalamic nucleus (STN), a common target for deep brain

  17. High-frequency stimulation of the subthalamic nucleus modifies the expression of vesicular glutamate transporters in basal ganglia in a rat model of Parkinson's disease.

    Science.gov (United States)

    Favier, Mathieu; Carcenac, Carole; Drui, Guillaume; Boulet, Sabrina; El Mestikawy, Salah; Savasta, Marc

    2013-12-05

    It has been suggested that glutamatergic system hyperactivity may be related to the pathogenesis of Parkinson's disease (PD). Vesicular glutamate transporters (VGLUT1-3) import glutamate into synaptic vesicles and are key anatomical and functional markers of glutamatergic excitatory transmission. Both VGLUT1 and VGLUT2 have been identified as definitive markers of glutamatergic neurons, but VGLUT 3 is also expressed by non glutamatergic neurons. VGLUT1 and VGLUT2 are thought to be expressed in a complementary manner in the cortex and the thalamus (VL/VM), in glutamatergic neurons involved in different physiological functions. Chronic high-frequency stimulation (HFS) of the subthalamic nucleus (STN) is the neurosurgical therapy of choice for the management of motor deficits in patients with advanced PD. STN-HFS is highly effective, but its mechanisms of action remain unclear. This study examines the effect of STN-HFS on VGLUT1-3 expression in different brain nuclei involved in motor circuits, namely the basal ganglia (BG) network, in normal and 6-hydroxydopamine (6-OHDA) lesioned rats. Here we report that: 1) Dopamine(DA)-depletion did not affect VGLUT1 and VGLUT3 expression but significantly decreased that of VGLUT2 in almost all BG structures studied; 2) STN-HFS did not change VGLUT1-3 expression in the different brain areas of normal rats while, on the contrary, it systematically induced a significant increase of their expression in DA-depleted rats and 3) STN-HFS reversed the decrease in VGLUT2 expression induced by the DA-depletion. These results show for the first time a comparative analysis of changes of expression for the three VGLUTs induced by STN-HFS in the BG network of normal and hemiparkinsonian rats. They provide evidence for the involvement of VGLUT2 in the modulation of BG cicuits and in particular that of thalamostriatal and thalamocortical pathways suggesting their key role in its therapeutic effects for alleviating PD motor symptoms.

  18. Basal ganglia function, stuttering, sequencing, and repair in adult songbirds

    Science.gov (United States)

    Kubikova, Lubica; Bosikova, Eva; Cvikova, Martina; Lukacova, Kristina; Scharff, Constance; Jarvis, Erich D.

    2014-01-01

    A pallial-basal-ganglia-thalamic-pallial loop in songbirds is involved in vocal motor learning. Damage to its basal ganglia part, Area X, in adult zebra finches has been noted to have no strong effects on song and its function is unclear. Here we report that neurotoxic damage to adult Area X induced changes in singing tempo and global syllable sequencing in all animals, and considerably increased syllable repetition in birds whose song motifs ended with minor repetitions before lesioning. This stuttering-like behavior started at one month, and improved over six months. Unexpectedly, the lesioned region showed considerable recovery, including immigration of newly generated or repaired neurons that became active during singing. The timing of the recovery and stuttering suggest that immature recovering activity of the circuit might be associated with stuttering. These findings indicate that even after juvenile learning is complete, the adult striatum plays a role in higher level organization of learned vocalizations. PMID:25307086

  19. Impact of L-DOPA treatment on regional cerebral blood flow and metabolism in the basal ganglia in a rat model of Parkinson's disease.

    Science.gov (United States)

    Ohlin, K Elisabet; Sebastianutto, Irene; Adkins, Chris E; Lundblad, Cornelia; Lockman, Paul R; Cenci, M Angela

    2012-05-15

    Large increases in regional cerebral blood flow (rCBF) have been measured in patients with Parkinson's disease (PD) following the administration of L-DOPA, but the underlying mechanisms have remained unknown. In this study, rats with unilateral 6-hydroxydopamine (6-OHDA) lesions were used to compare patterns of rCBF and regional cerebral glucose utilisation (rCGU) in chronically L-DOPA-treated subjects following a final injection of L-DOPA or saline. The same animal model was used to the leakage of a blood-brain barrier (BBB) tracer molecule at 60 min vs. 24h following the last L-DOPA injection of a chronic treatment. All the parameters under investigation were examined with brain autoradiography following intravenous injections of specific radiotracers in awake animals ([14C]-iodoantipyrine for rCBF, [14C]-2-deoxyglucose for rCGU, and [14C]-α-aminoisobutyric acid for BBB leakage). Significant changes in rCBF and rCGU on the side ipsilateral to the 6-OHDA lesion relative to the non-lesioned side were seen at 60 min ("ON") but not 24h ("OFF") following L-DOPA administration. These changes were not seen in sham-operated rats. In the output nuclei of the basal ganglia (the entopeduncular nucleus and the substantia nigra pars reticulata) both rCBF and rCGU were elevated both in acutely L-DOPA-treated rats and chronically L-DOPA-treated rats displaying dyskinesia, but did not change significantly in chronically L-DOPA-treated non-dyskinetic cases. Acutely and chronically L-DOPA-treated rats with dyskinesia exhibited increases in rCBF "ON L-DOPA" also in the motor cortex, the striatum, and the globus pallidus, but the corresponding changes in rCGU did not show the same direction, magnitude, and/or relative group differences. The uptake of a BBB tracer (studied in the striatum and the substantia nigra reticulata in chronically L-DOPA treated rats) was significantly higher ON vs. OFF L-DOPA. The present results are the first to show that the administration of L-DOPA is

  20. Hemodynamics in the cerebral cortex and basal ganglia

    International Nuclear Information System (INIS)

    Yamaguchi, Shinya; Fukuyama, Hidenao; Yamauchi, Hiroshi; Kimura, Jun

    1991-01-01

    We examined ten healthy volunteers using positron emission tomography (PET) in order to elucidate regional changes and correlations in the cerebral circulation and oxygen metabolism. We also studied eight lacunar stroke patients so as to disclose the influences of vascular risk factors and aging on the cerebral blood flow and metabolism. We can conclude from our result as follows: (1) Cerebral blood volume (CBV) was minimum in the basal ganglia and cerebral blood flow (CBF)/CBV ratio was higher than that of cerebral cortex in healthy volunteers; (2) CBF of gray matter in healthy volunteers correlated with CBV and cerebral metabolic rate of oxygen where oxygen extraction fraction inversely correlated with CBF, CBV, and CBF/CBV; and (3) the basal ganglia CBF/CBV ratio in lacunar stroke patients was lower than that of healthy volunteers. These findings suggested that the perfusion pressure in the basal ganglia was so high in the normal condition than the angionecrosis or occlusion in the perforating arteries would be induced, especially in the aged and hypertensive patients. (author)

  1. Effects of Focal Basal Ganglia Lesions on Timing and Force Control

    Science.gov (United States)

    Aparicio, P.; Diedrichsen, J.; Ivry, R.B.

    2005-01-01

    Studies of basal ganglia dysfunction in humans have generally involved patients with degenerative disorders, notably Parkinson's disease. In many instances, the performance of these patients is compared to that of patients with focal lesions of other brain structures such as the cerebellum. In the present report, we studied the performance of…

  2. Acute movement disorder with bilateral basal ganglia lesions in diabetic uremia

    Directory of Open Access Journals (Sweden)

    Gurusidheshwar M Wali

    2011-01-01

    Full Text Available Acute movement disorder associated with symmetrical basal ganglia lesions occurring in the background of diabetic end stage renal disease is a recently described condition. It has distinct clinico-radiological features and is commonly described in Asian patients. We report the first Indian case report of this potentially reversible condition and discuss its various clinico-radiological aspects.

  3. Multiplexed coding in the human basal ganglia

    Science.gov (United States)

    Andres, D. S.; Cerquetti, D.; Merello, M.

    2016-04-01

    A classic controversy in neuroscience is whether information carried by spike trains is encoded by a time averaged measure (e.g. a rate code), or by complex time patterns (i.e. a time code). Here we apply a tool to quantitatively analyze the neural code. We make use of an algorithm based on the calculation of the temporal structure function, which permits to distinguish what scales of a signal are dominated by a complex temporal organization or a randomly generated process. In terms of the neural code, this kind of analysis makes it possible to detect temporal scales at which a time patterns coding scheme or alternatively a rate code are present. Additionally, finding the temporal scale at which the correlation between interspike intervals fades, the length of the basic information unit of the code can be established, and hence the word length of the code can be found. We apply this algorithm to neuronal recordings obtained from the Globus Pallidus pars interna from a human patient with Parkinson’s disease, and show that a time pattern coding and a rate coding scheme co-exist at different temporal scales, offering a new example of multiplexed neuronal coding.

  4. Alterations in neuronal activity in basal ganglia-thalamocortical circuits in the parkinsonian state

    Science.gov (United States)

    Galvan, Adriana; Devergnas, Annaelle; Wichmann, Thomas

    2015-01-01

    In patients with Parkinson’s disease and in animal models of this disorder, neurons in the basal ganglia and related regions in thalamus and cortex show changes that can be recorded by using electrophysiologic single-cell recording techniques, including altered firing rates and patterns, pathologic oscillatory activity and increased inter-neuronal synchronization. In addition, changes in synaptic potentials or in the joint spiking activities of populations of neurons can be monitored as alterations in local field potentials (LFPs), electroencephalograms (EEGs) or electrocorticograms (ECoGs). Most of the mentioned electrophysiologic changes are probably related to the degeneration of diencephalic dopaminergic neurons, leading to dopamine loss in the striatum and other basal ganglia nuclei, although degeneration of non-dopaminergic cell groups may also have a role. The altered electrical activity of the basal ganglia and associated nuclei may contribute to some of the motor signs of the disease. We here review the current knowledge of the electrophysiologic changes at the single cell level, the level of local populations of neural elements, and the level of the entire basal ganglia-thalamocortical network in parkinsonism, and discuss the possible use of this information to optimize treatment approaches to Parkinson’s disease, such as deep brain stimulation (DBS) therapy. PMID:25698937

  5. Alterations in Neuronal Activity in Basal Ganglia-Thalamocortical Circuits in the Parkinsonian State

    Directory of Open Access Journals (Sweden)

    Adriana eGalvan

    2015-02-01

    Full Text Available In patients with Parkinson’s disease and in animal models of this disorder, neurons in the basal ganglia and related regions in thalamus and cortex show changes that can be recorded by using electrophysiologic single-cell recording techniques, including altered firing rates and patterns, pathologic oscillatory activity and increased inter-neuronal synchronization. In addition, changes in synaptic potentials or in the joint spiking activities of populations of neurons can be monitored as alterations in local field potentials, electroencephalograms or electrocorticograms. Most of the mentioned electrophysiologic changes are probably related to the degeneration of diencephalic dopaminergic neurons, leading to dopamine loss in the striatum and other basal ganglia nuclei, although degeneration of non-dopaminergic cell groups may also have a role. The altered electrical activity of the basal ganglia and associated nuclei may contribute to some of the motor signs of the disease. We here review the current knowledge of the electrophysiologic changes at the single cell level, the level of local populations of neural elements, and the level of the entire basal ganglia-thalamocortical network in parkinsonism, and discuss the possible use of this information to optimize treatment approaches to Parkinson’s disease, such as deep brain stimulation therapy.

  6. Phenotypic spectrum of probable and genetically-confirmed idiopathic basal ganglia calcification.

    Science.gov (United States)

    Nicolas, Gaël; Pottier, Cyril; Charbonnier, Camille; Guyant-Maréchal, Lucie; Le Ber, Isabelle; Pariente, Jérémie; Labauge, Pierre; Ayrignac, Xavier; Defebvre, Luc; Maltête, David; Martinaud, Olivier; Lefaucheur, Romain; Guillin, Olivier; Wallon, David; Chaumette, Boris; Rondepierre, Philippe; Derache, Nathalie; Fromager, Guillaume; Schaeffer, Stéphane; Krystkowiak, Pierre; Verny, Christophe; Jurici, Snejana; Sauvée, Mathilde; Vérin, Marc; Lebouvier, Thibaud; Rouaud, Olivier; Thauvin-Robinet, Christel; Rousseau, Stéphane; Rovelet-Lecrux, Anne; Frebourg, Thierry; Campion, Dominique; Hannequin, Didier

    2013-11-01

    Idiopathic basal ganglia calcification is characterized by mineral deposits in the brain, an autosomal dominant pattern of inheritance in most cases and genetic heterogeneity. The first causal genes, SLC20A2 and PDGFRB, have recently been reported. Diagnosing idiopathic basal ganglia calcification necessitates the exclusion of other causes, including calcification related to normal ageing, for which no normative data exist. Our objectives were to diagnose accurately and then describe the clinical and radiological characteristics of idiopathic basal ganglia calcification. First, calcifications were evaluated using a visual rating scale on the computerized tomography scans of 600 consecutively hospitalized unselected controls. We determined an age-specific threshold in these control computerized tomography scans as the value of the 99th percentile of the total calcification score within three age categories: 60 years. To study the phenotype of the disease, patients with basal ganglia calcification were recruited from several medical centres. Calcifications that rated below the age-specific threshold using the same scale were excluded, as were patients with differential diagnoses of idiopathic basal ganglia calcification, after an extensive aetiological assessment. Sanger sequencing of SLC20A2 and PDGFRB was performed. In total, 72 patients were diagnosed with idiopathic basal ganglia calcification, 25 of whom bore a mutation in either SLC20A2 (two families, four sporadic cases) or PDGFRB (one family, two sporadic cases). Five mutations were novel. Seventy-one per cent of the patients with idiopathic basal ganglia calcification were symptomatic (mean age of clinical onset: 39 ± 20 years; mean age at last evaluation: 55 ± 19 years). Among them, the most frequent signs were: cognitive impairment (58.8%), psychiatric symptoms (56.9%) and movement disorders (54.9%). Few clinical differences appeared between SLC20A2 and PDGFRB mutation carriers. Radiological analysis

  7. Rehabilitation program based on sensorimotor recovery improves the static and dynamic balance and modifies the basal ganglia neurochemistry: A pilot 1H-MRS study on Parkinson's disease patients.

    Science.gov (United States)

    Delli Pizzi, Stefano; Bellomo, Rosa Grazia; Carmignano, Simona Maria; Ancona, Emilio; Franciotti, Raffaella; Supplizi, Marco; Barassi, Giovanni; Onofrj, Marco; Bonanni, Laura; Saggini, Raoul

    2017-12-01

    Rehabilitation interventions represent an alternative strategy to pharmacological treatment in order to slow or reverse some functional aspects of disability in Parkinson's disease (PD). To date, the neurophysiological mechanisms underlying rehabilitation-mediated improvement in PD patients are still poorly understood. Interestingly, growing evidence has highlighted a key role of the glutamate in neurogenesis and brain plasticity. The brain levels of glutamate, and of its precursor glutamine, can be detected in vivo and noninvasively as "Glx" by means of proton magnetic resonance spectroscopy (H-MRS). In the present pilot study, 7 PD patients with frequent falls and axial dystonia underwent 8-week rehabilitative protocol focused on sensorimotor improvement. Clinical evaluation and Glx quantification were performed before and after rehabilitation. The Glx assessment was focused on the basal ganglia in agreement with their key role in the motor functions. We found that the rehabilitation program improves the static and dynamic balance in PD patients, promoting a better global motor performance. Moreover, we observed that the levels of Glx within the left basal ganglia were higher after rehabilitation as compared with baseline. Thus, we posit that our sensorimotor rehabilitative protocol could stimulate the glutamate metabolism in basal ganglia and, in turn, neuroplasticity processes. We also hypothesize that these mechanisms could prepare the ground to restore the functional interaction among brain areas deputed to motor controls, which are affected in PD. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  8. Similar circuits but different connectivity patterns between the cerebellum, basal ganglia, and supplementary motor area in early Parkinson's disease patients and controls during predictive motor timing.

    Science.gov (United States)

    Husárová, Ivica; Mikl, Michal; Lungu, Ovidiu V; Mareček, Radek; Vaníček, Jiří; Bareš, Martin

    2013-10-01

    The cerebellum, basal ganglia (BG), and other cortical regions, such as supplementary motor area (SMA) have emerged as important structures dealing with various aspects of timing, yet the modulation of functional connectivity between them during motor timing tasks remains unexplored. We used dynamic causal modeling to investigate the differences in effective connectivity (EC) between these regions and its modulation by behavioral outcome during a motor timing prediction task in a group of 16 patients with early Parkinson's disease (PD) and 17 healthy controls. Behavioral events (hits and errors) constituted the driving input connected to the cerebellum, and the modulation in connectivity was assessed relative to the hit condition (successful interception of target). The driving input elicited response in the target area, while modulatory input changed the specific connection strength. The neuroimaging data revealed similar structure of intrinsic connectivity in both groups with unidirectional connections from cerebellum to both sides of the BG, from BG to the SMA, and then from SMA to the cerebellum. However, the type of intrinsic connection was different between two groups. In the PD group, the connection between the SMA and cerebellum was inhibitory in comparison to the HC group, where the connection was activated. Furthermore, the modulation of connectivity by the performance in the task was different between the two groups, with decreased connectivity between the cerebellum and left BG and SMA and a more pronounced symmetry of these connections in controls. In the same time, there was an increased EC between the cerebellum and both sides of BG with more pronounced asymmetry (stronger connection with left BG) in patients. In addition, in the PD group the modulatory input strengthened inhibitory connectivity between the SMA and the cerebellum, while in the HC group the excitatory connection was slightly strengthened. Our findings indicate that although early PD

  9. Potential mechanisms for imperfect synchronization in parkinsonian basal ganglia.

    Directory of Open Access Journals (Sweden)

    Choongseok Park

    Full Text Available Neural activity in the brain of parkinsonian patients is characterized by the intermittently synchronized oscillatory dynamics. This imperfect synchronization, observed in the beta frequency band, is believed to be related to the hypokinetic motor symptoms of the disorder. Our study explores potential mechanisms behind this intermittent synchrony. We study the response of a bursting pallidal neuron to different patterns of synaptic input from subthalamic nucleus (STN neuron. We show how external globus pallidus (GPe neuron is sensitive to the phase of the input from the STN cell and can exhibit intermittent phase-locking with the input in the beta band. The temporal properties of this intermittent phase-locking show similarities to the intermittent synchronization observed in experiments. We also study the synchronization of GPe cells to synaptic input from the STN cell with dependence on the dopamine-modulated parameters. Earlier studies showed how the strengthening of dopamine-modulated coupling may lead to transitions from non-synchronized to partially synchronized dynamics, typical in Parkinson's disease. However, dopamine also affects the cellular properties of neurons. We show how the changes in firing patterns of STN neuron due to the lack of dopamine may lead to transition from a lower to a higher coherent state, roughly matching the synchrony levels observed in basal ganglia in normal and parkinsonian states. The intermittent nature of the neural beta band synchrony in Parkinson's disease is achieved in the model due to the interplay of the timing of STN input to pallidum and pallidal neuronal dynamics, resulting in sensitivity of pallidal output to the phase of the arriving STN input. Thus the mechanism considered here (the change in firing pattern of subthalamic neurons through the dopamine-induced change of membrane properties may be one of the potential mechanisms responsible for the generation of the intermittent synchronization

  10. Network effects of subthalamic deep brain stimulation drive a unique mixture of responses in basal ganglia output

    OpenAIRE

    Humphries, Mark D.; Gurney, Kevin

    2012-01-01

    Deep brain stimulation (DBS) is a remarkably successful treatment for the motor symptoms of Parkinson's disease. High-frequency stimulation of the subthalamic nucleus (STN) within the basal ganglia is a main clinical target, but the physiological mechanisms of therapeutic STN DBS at the cellular and network level are unclear. We set out to begin to address the hypothesis that a mixture of responses in the basal ganglia output nuclei, combining regularized firing and inhibition, is a key contr...

  11. Why we can talk, debate, and change our minds: neural circuits, basal ganglia operations, and transcriptional factors.

    Science.gov (United States)

    Lieberman, Philip

    2014-12-01

    Ackermann et al. disregard attested knowledge concerning aphasia, Parkinson disease, cortical-to-striatal circuits, basal ganglia, laryngeal phonation, and other matters. Their dual-pathway model cannot account for "what is special about the human brain." Their human cortical-to-laryngeal neural circuit does not exist. Basal ganglia operations, enhanced by mutations on FOXP2, confer human motor-control, linguistic, and cognitive capabilities.

  12. Neuroanatomical Correlates of Intelligence in Healthy Young Adults: The Role of Basal Ganglia Volume

    Science.gov (United States)

    Rhein, Cosima; Mühle, Christiane; Richter-Schmidinger, Tanja; Alexopoulos, Panagiotis; Doerfler, Arnd; Kornhuber, Johannes

    2014-01-01

    Background In neuropsychiatric diseases with basal ganglia involvement, higher cognitive functions are often impaired. In this exploratory study, we examined healthy young adults to gain detailed insight into the relationship between basal ganglia volume and cognitive abilities under non-pathological conditions. Methodology/Principal Findings We investigated 137 healthy adults that were between the ages of 21 and 35 years with similar educational backgrounds. Magnetic resonance imaging (MRI) was performed, and volumes of basal ganglia nuclei in both hemispheres were calculated using FreeSurfer software. The cognitive assessment consisted of verbal, numeric and figural aspects of intelligence for either the fluid or the crystallised intelligence factor using the intelligence test Intelligenz-Struktur-Test (I-S-T 2000 R). Our data revealed significant correlations of the caudate nucleus and pallidum volumes with figural and numeric aspects of intelligence, but not with verbal intelligence. Interestingly, figural intelligence associations were dependent on sex and intelligence factor; in females, the pallidum volumes were correlated with crystallised figural intelligence (r = 0.372, p = 0.01), whereas in males, the caudate volumes were correlated with fluid figural intelligence (r = 0.507, p = 0.01). Numeric intelligence was correlated with right-lateralised caudate nucleus volumes for both females and males, but only for crystallised intelligence (r = 0.306, p = 0.04 and r = 0.459, p = 0.04, respectively). The associations were not mediated by prefrontal cortical subfield volumes when controlling with partial correlation analyses. Conclusions/Significance The findings of our exploratory analysis indicate that figural and numeric intelligence aspects, but not verbal aspects, are strongly associated with basal ganglia volumes. Unlike numeric intelligence, the type of figural intelligence appears to be related to distinct basal ganglia

  13. Neuroanatomical correlates of intelligence in healthy young adults: the role of basal ganglia volume.

    Science.gov (United States)

    Rhein, Cosima; Mühle, Christiane; Richter-Schmidinger, Tanja; Alexopoulos, Panagiotis; Doerfler, Arnd; Kornhuber, Johannes

    2014-01-01

    In neuropsychiatric diseases with basal ganglia involvement, higher cognitive functions are often impaired. In this exploratory study, we examined healthy young adults to gain detailed insight into the relationship between basal ganglia volume and cognitive abilities under non-pathological conditions. We investigated 137 healthy adults that were between the ages of 21 and 35 years with similar educational backgrounds. Magnetic resonance imaging (MRI) was performed, and volumes of basal ganglia nuclei in both hemispheres were calculated using FreeSurfer software. The cognitive assessment consisted of verbal, numeric and figural aspects of intelligence for either the fluid or the crystallised intelligence factor using the intelligence test Intelligenz-Struktur-Test (I-S-T 2000 R). Our data revealed significant correlations of the caudate nucleus and pallidum volumes with figural and numeric aspects of intelligence, but not with verbal intelligence. Interestingly, figural intelligence associations were dependent on sex and intelligence factor; in females, the pallidum volumes were correlated with crystallised figural intelligence (r = 0.372, p = 0.01), whereas in males, the caudate volumes were correlated with fluid figural intelligence (r = 0.507, p = 0.01). Numeric intelligence was correlated with right-lateralised caudate nucleus volumes for both females and males, but only for crystallised intelligence (r = 0.306, p = 0.04 and r = 0.459, p = 0.04, respectively). The associations were not mediated by prefrontal cortical subfield volumes when controlling with partial correlation analyses. The findings of our exploratory analysis indicate that figural and numeric intelligence aspects, but not verbal aspects, are strongly associated with basal ganglia volumes. Unlike numeric intelligence, the type of figural intelligence appears to be related to distinct basal ganglia nuclei in a sex-specific manner. Subcortical brain structures thus may contribute substantially to

  14. Toward sophisiticated basal ganglia neuromodulation: review on basal gaglia deep brain stimulation

    Science.gov (United States)

    Da Cunha, Claudio; Boschen, Suelen L.; Gómez-A, Alexander; Ross, Erika K.; Gibson, William S. J.; Min, Hoon-Ki; Lee, Kendall H.; Blaha, Charles D.

    2015-01-01

    This review presents state-of-the-art knowledge about the roles of the basal ganglia (BG) in action-selection, cognition, and motivation, and how this knowledge has been used to improve deep brain stimulation (DBS) treatment of neurological and psychiatric disorders. Such pathological conditions include Parkinson’s disease, Huntington’s disease, Tourette syndrome, depression, and obsessive-compulsive disorder. The first section presents evidence supporting current hypotheses of how the cortico-BG circuitry works to select motor and emotional actions, and how defects in this circuitry can cause symptoms of the BG diseases. Emphasis is given to the role of striatal dopamine on motor performance, motivated behaviors and learning of procedural memories. Next, the use of cutting-edge electrochemical techniques in animal and human studies of BG functioning under normal and disease conditions is discussed. Finally, functional neuroimaging studies are reviewed; these works have shown the relationship between cortico-BG structures activated during DBS and improvement of disease symptoms. PMID:25684727

  15. Synergy as a new and sensitive marker of basal ganglia dysfunction: A study of asymptomatic welders.

    Science.gov (United States)

    Lewis, Mechelle M; Lee, Eun-Young; Jo, Hang Jin; Du, Guangwei; Park, Jaebum; Flynn, Michael R; Kong, Lan; Latash, Mark L; Huang, Xuemei

    2016-09-01

    Multi-digit synergies, a recently developed, theory-based method to quantify stability of motor action, are shown to reflect basal ganglia dysfunction associated with parkinsonian syndromes. In this study, we tested the hypothesis that multi-digit synergies may capture early and subclinical basal ganglia dysfunction. We chose asymptomatic welders to test the hypothesis because the basal ganglia are known to be most susceptible to neurotoxicity caused by welding-related metal accumulation (such as manganese and iron). Twenty right-handed welders and 13 matched controls were invited to perform single- and multi-finger pressing tasks using the fingers of the right or left hand. Unified Parkinson's Disease Rating Scale and Grooved Pegboard scores were used to gauge gross and fine motor dysfunction, respectively. High-resolution (3T) T1-weighted, T2-weighted, T1 mapping, susceptibility, and diffusion tensor MRIs were obtained to reflect manganese, iron accumulation, and microstructural changes in basal ganglia. The synergy index stabilizing total force and anticipatory synergy adjustments were computed, compared between groups, and correlated with estimates of basal ganglia manganese [the pallidal index, R1 (1/T1)], iron [R2* (1/T2*)], and microstructural changes [fractional anisotropy and mean diffusivity]. There were no significant differences in Unified Parkinson's Disease Rating Scale (total or motor subscale) or Grooved Pegboard test scores between welders and controls. The synergy index during steady-state accurate force production was decreased significantly in the left hand of welders compared to controls (p=0.004) but did not reach statistical significance in the right hand (p=0.16). Anticipatory synergy adjustments, however, were not significantly different between groups. Among welders, higher synergy indices in the left hand were associated significantly with higher fractional anisotropy values in the left globus pallidus (R=0.731, psynergy metrics may serve

  16. Bilateral symmetrical low density areas in the basal ganglia

    International Nuclear Information System (INIS)

    Ugawa, Yoshikazu; Ihara, Yasuo

    1984-01-01

    We reported a case with dysarthria and gait disturbance, in which CT revealed symmetrical well-demarcated low density areas in the basal ganglia. The patient was a 43-year-old woman. Her family history and past history were not contributory. She had a little difficulty in speaking at the age of 17. Gait disturbance and micrographia appeared later. Although her expressionless face resembles to that seen in Parkinsonism, rigidity, akinesia and small-stepped gait were not present. The unclassified types of dysarthria and gait disturbance, which characterize the present case, were considered to be a kind of extrapyramidal symptoms, which were distinct from those of Parkinsonism. CT showed well demarcated low density areas predominantly in bilateral putamen. Metrizamide CT failed to show any communication between low density areas and subarachnoid spaces. To date, six cases, which presented similar clinical features and almost same CT findings as our case, were reported. (author)

  17. Disconnection syndromes of basal ganglia, thalamus, and cerebrocerebellar systems.

    Science.gov (United States)

    Schmahmann, Jeremy D; Pandya, Deepak N

    2008-09-01

    Disconnection syndromes were originally conceptualized as a disruption of communication between different cerebral cortical areas. Two developments mandate a re-evaluation of this notion. First, we present a synopsis of our anatomical studies in monkey elucidating principles of organization of cerebral cortex. Efferent fibers emanate from every cortical area, and are directed with topographic precision via association fibers to ipsilateral cortical areas, commissural fibers to contralateral cerebral regions, striatal fibers to basal ganglia, and projection subcortical bundles to thalamus, brainstem and/or pontocerebellar system. We note that cortical areas can be defined by their patterns of subcortical and cortical connections. Second, we consider motor, cognitive and neuropsychiatric disorders in patients with lesions restricted to basal ganglia, thalamus, or cerebellum, and recognize that these lesions mimic deficits resulting from cortical lesions, with qualitative differences between the manifestations of lesions in functionally related areas of cortical and subcortical nodes. We consider these findings on the basis of anatomical observations from tract tracing studies in monkey, viewing them as disconnection syndromes reflecting loss of the contribution of subcortical nodes to the distributed neural circuits. We introduce a new theoretical framework for the distributed neural circuits, based on general, and specific, principles of anatomical organization, and on the architecture of the nodes that comprise these systems. We propose that neural architecture determines function, i.e., each architectonically distinct cortical and subcortical area contributes a unique transform, or computation, to information processing; anatomically precise and segregated connections between nodes define behavior; and association fiber tracts that link cerebral cortical areas with each other enable the cross-modal integration required for evolved complex behaviors. This model

  18. Role of basal ganglia in sleep-wake regulation: neural circuitry and clinical significance

    Directory of Open Access Journals (Sweden)

    Ramalingam Vetrivelan

    2010-11-01

    Full Text Available Researchers over the last decade have made substantial progress towards understanding the roles of dopamine and the basal ganglia in the control of sleep-wake behavior. In this review, we outline recent advancements regarding dopaminergic modulation of sleep through the basal ganglia (BG and extra-BG sites. Our main hypothesis is that dopamine promotes sleep by its action on the D2 receptors in the BG and promotes wakefulness by its action on D1 and D2 receptors in the extra-BG sites. This hypothesis implicates dopamine depletion in the BG (such as in Parkinson’s disease in causing frequent nighttime arousal and overall insomnia. Furthermore, the arousal effects of psychostimulants (methamphetamine, cocaine and modafinil may be linked to the ventral periaquductal grey (vPAG dopaminergic circuitry targeting the extra-BG sleep-wake network.

  19. Extrahepatic portal vein obstruction with parkinsonism and symmetric hyperintense basal ganglia on T1 weighted MRI

    Directory of Open Access Journals (Sweden)

    Jayalakshmi Sita

    2006-01-01

    Full Text Available Abnormal high signal in the globus pallidus on T1 weighted magnetic resonance imaging (MRI of the brain has been well described in patients with chronic liver disease. It may be related to liver dysfunction or portal-systemic shunting. We report a case of extra hepatic portal vein obstruction with portal hypertension and esophageal varices that presented with extra pyramidal features. T1 weighted MRI brain scans showed increased symmetrical signal intensities in the basal ganglia. Normal hepatic function in this patient emphasizes the role of portal- systemic communications in the development of these hyperintensities, which may be due to deposition of paramagnetic substances like manganese in the basal ganglia.

  20. Cognitive consequences of thalamic, basal ganglia, and deep white matter lacunes in brain aging and dementia.

    Science.gov (United States)

    Gold, Gabriel; Kövari, Enikö; Herrmann, François R; Canuto, Alessandra; Hof, Patrick R; Michel, Jean-Pierre; Bouras, Constantin; Giannakopoulos, Panteleimon

    2005-06-01

    Most previous studies addressed the cognitive impact of lacunar infarcts using radiologic correlations that are known to correlate poorly with neuropathological data. Moreover, absence of systematic bilateral assessment of vascular lesions and masking effects of Alzheimer disease pathology and macrovascular lesions may explain discrepancies among previous reports. To define the relative contribution of silent lacunes to cognitive decline, we performed a detailed analysis of lacunar and microvascular pathology in both cortical and subcortical areas of 72 elderly individuals without significant neurofibrillary tangle pathology or macrovascular lesions. Cognitive status was assessed prospectively using the Clinical Dementia Rating (CDR) scale; neuropathological evaluation included Abeta-protein deposition staging and bilateral assessment of microvascular ischemic pathology and lacunes; statistical analysis included multivariate models controlling for age, amyloid deposits, and microvascular pathology. Thalamic and basal ganglia lacunes were negatively associated with CDR scores; cortical microinfarcts, periventricular and diffuse white matter demyelination also significantly affected cognition. In a multivariate model, cortical microinfarcts and thalamic and basal ganglia lacunes explained 22% of CDR variability; amyloid deposits and microvascular pathology explained 12%, and the assessment of thalamic and basal ganglia lacunes added an extra 17%. Deep white matter lacunes were not related to cognitive status in univariate and multivariate models. In agreement with the recently proposed concept of subcortical ischemic vascular dementia, our autopsy series provides important evidence that gray matter lacunes are independent predictors of cognitive decline in elderly individuals without concomitant dementing processes such as Alzheimer disease.

  1. Ketamine-induced oscillations in the motor circuit of the rat basal ganglia.

    Directory of Open Access Journals (Sweden)

    María Jesús Nicolás

    Full Text Available Oscillatory activity can be widely recorded in the cortex and basal ganglia. This activity may play a role not only in the physiology of movement, perception and cognition, but also in the pathophysiology of psychiatric and neurological diseases like schizophrenia or Parkinson's disease. Ketamine administration has been shown to cause an increase in gamma activity in cortical and subcortical structures, and an increase in 150 Hz oscillations in the nucleus accumbens in healthy rats, together with hyperlocomotion.We recorded local field potentials from motor cortex, caudate-putamen (CPU, substantia nigra pars reticulata (SNr and subthalamic nucleus (STN in 20 awake rats before and after the administration of ketamine at three different subanesthetic doses (10, 25 and 50 mg/Kg, and saline as control condition. Motor behavior was semiautomatically quantified by custom-made software specifically developed for this setting.Ketamine induced coherent oscillations in low gamma (~ 50 Hz, high gamma (~ 80 Hz and high frequency (HFO, ~ 150 Hz bands, with different behavior in the four structures studied. While oscillatory activity at these three peaks was widespread across all structures, interactions showed a different pattern for each frequency band. Imaginary coherence at 150 Hz was maximum between motor cortex and the different basal ganglia nuclei, while low gamma coherence connected motor cortex with CPU and high gamma coherence was more constrained to the basal ganglia nuclei. Power at three bands correlated with the motor activity of the animal, but only coherence values in the HFO and high gamma range correlated with movement. Interactions in the low gamma band did not show a direct relationship to movement.These results suggest that the motor effects of ketamine administration may be primarily mediated by the induction of coherent widespread high-frequency activity in the motor circuit of the basal ganglia, together with a frequency

  2. Comprehensive in vivo mapping of the human basal ganglia and thalamic connectome in individuals using 7T MRI.

    Directory of Open Access Journals (Sweden)

    Christophe Lenglet

    Full Text Available Basal ganglia circuits are affected in neurological disorders such as Parkinson's disease (PD, essential tremor, dystonia and Tourette syndrome. Understanding the structural and functional connectivity of these circuits is critical for elucidating the mechanisms of the movement and neuropsychiatric disorders, and is vital for developing new therapeutic strategies such as deep brain stimulation (DBS. Knowledge about the connectivity of the human basal ganglia and thalamus has rapidly evolved over recent years through non-invasive imaging techniques, but has remained incomplete because of insufficient resolution and sensitivity of these techniques. Here, we present an imaging and computational protocol designed to generate a comprehensive in vivo and subject-specific, three-dimensional model of the structure and connections of the human basal ganglia. High-resolution structural and functional magnetic resonance images were acquired with a 7-Tesla magnet. Capitalizing on the enhanced signal-to-noise ratio (SNR and enriched contrast obtained at high-field MRI, detailed structural and connectivity representations of the human basal ganglia and thalamus were achieved. This unique combination of multiple imaging modalities enabled the in-vivo visualization of the individual human basal ganglia and thalamic nuclei, the reconstruction of seven white-matter pathways and their connectivity probability that, to date, have only been reported in animal studies, histologically, or group-averaged MRI population studies. Also described are subject-specific parcellations of the basal ganglia and thalamus into sub-territories based on their distinct connectivity patterns. These anatomical connectivity findings are supported by functional connectivity data derived from resting-state functional MRI (R-fMRI. This work demonstrates new capabilities for studying basal ganglia circuitry, and opens new avenues of investigation into the movement and neuropsychiatric

  3. Off-Target18F-AV-1451 Binding in the Basal Ganglia Correlates with Age-Related Iron Accumulation.

    Science.gov (United States)

    Choi, Jae Yong; Cho, Hanna; Ahn, Sung Jun; Lee, Jae Hoon; Ryu, Young Hoon; Lee, Myung Sik; Lyoo, Chul Hyoung

    2018-01-01

    Off-target binding in the basal ganglia is commonly observed in the 18 F-AV-1451 PET studies of the elderly. We sought to investigate the relationship between this phenomenon in the basal ganglia and iron accumulation using iron-sensitive R 2 * MRI. Methods: Fifty-nine healthy controls and 61 patients with Alzheimer disease and mild cognitive impairment underwent 18 F-AV-1451 PET and R 2 * MRI studies. A correlation analysis was performed for age, 18 F-AV-1451 binding, and R 2 * values. Results: There was an age-related increase in both 18 F-AV-1451 binding in the basal ganglia and R 2 * values in the putamen in both the controls and the Alzheimer disease/mild cognitive impairment patients. 18 F-AV-1451 binding in the basal ganglia increased with R 2 * values. Conclusion: Off-target 18 F-AV-1451 binding in the basal ganglia is associated with the age-related increases in iron accumulation. Postmortem studies are required to further investigate the nature of this association. © 2018 by the Society of Nuclear Medicine and Molecular Imaging.

  4. The role of the basal ganglia in the control of seizure.

    Science.gov (United States)

    Vuong, J; Devergnas, Annaelle

    2018-03-01

    Epilepsy is a network disorder and each type of seizure involves distinct cortical and subcortical network, differently implicated in the control and propagation of the ictal activity. The role of the basal ganglia has been revealed in several cases of focal and generalized seizures. Here, we review the data that show the implication of the basal ganglia in absence, temporal lobe, and neocortical seizures in animal models (rodent, cat, and non-human primate) and in human. Based on these results and the advancement of deep brain stimulation for Parkinson's disease, basal ganglia neuromodulation has been tested with some success that can be equally seen as promising or disappointing. The effect of deep brain stimulation can be considered promising with a 76% in seizure reduction in temporal lobe epilepsy patients, but also disappointing, since only few patients have become seizure free and the antiepileptic effects have been highly variable among patients. This variability could probably be explained by the heterogeneity among the patients included in these clinical studies. To illustrate the importance of specific network identification, electrophysiological activity of the putamen and caudate nucleus has been recorded during penicillin-induced pre-frontal and motor seizures in one monkey. While an increase of the firing rate was found in putamen and caudate nucleus during pre-frontal seizures, only the activity of the putamen cells was increased during motor seizures. These preliminary results demonstrate the implication of the basal ganglia in two types of neocortical seizures and the necessity of studying the network to identify the important nodes implicated in the propagation and control of each type of seizure.

  5. Frequency and function in the basal ganglia: the origins of beta and gamma band activity.

    Science.gov (United States)

    Blenkinsop, Alexander; Anderson, Sean; Gurney, Kevin

    2017-07-01

    Neuronal oscillations in the basal ganglia have been observed to correlate with behaviours, although the causal mechanisms and functional significance of these oscillations remain unknown. We present a novel computational model of the healthy basal ganglia, constrained by single unit recordings from non-human primates. When the model is run using inputs that might be expected during performance of a motor task, the network shows emergent phenomena: it functions as a selection mechanism and shows spectral properties that match those seen in vivo. Beta frequency oscillations are shown to require pallido-striatal feedback, and occur with behaviourally relevant cortical input. Gamma oscillations arise in the subthalamic-globus pallidus feedback loop, and occur during movement. The model provides a coherent framework for the study of spectral, temporal and functional analyses of the basal ganglia and lays the foundation for an integrated approach to study basal ganglia pathologies such as Parkinson's disease in silico. Neural oscillations in the basal ganglia (BG) are well studied yet remain poorly understood. Behavioural correlates of spectral activity are well described, yet a quantitative hypothesis linking time domain dynamics and spectral properties to BG function has been lacking. We show, for the first time, that a unified description is possible by interpreting previously ignored structure in data describing globus pallidus interna responses to cortical stimulation. These data were used to expose a pair of distinctive neuronal responses to the stimulation. This observation formed the basis for a new mathematical model of the BG, quantitatively fitted to the data, which describes the dynamics in the data, and is validated against other stimulus protocol experiments. A key new result is that when the model is run using inputs hypothesised to occur during the performance of a motor task, beta and gamma frequency oscillations emerge naturally during static-force and

  6. 1H MR spectroscopy of the basal ganglia in childhood: a semiquantitative analysis

    International Nuclear Information System (INIS)

    Lam, W.W.M.; Zhao, H.; Berry, G.T.; Kaplan, P.; Gibson, J.; Kaplan, B.S.

    1998-01-01

    Proton MR spectra of the basal ganglia were obtained from 28 patients, 24 male and 14 female, median age 16.3 months (5 weeks to 31 years). They included 17 patients with normal MRI of the basal ganglia without metabolic disturbance (control group) and 11 patients with various metabolic diseases: one case each of high serum sodium and high serum osmolarity, cobalamin C deficiency, Leigh disease, Galloway-Mowat syndrome, Pelizaeus-Merzbacher disease, hemolytic-uremic syndrome and Wilson disease and two cases of Alagille syndrome and methylmalonic acidemia with abnormal MRI of the basal ganglia or blood or urine analysis (abnormal group). The MR spectrum was measured by using STEAM. The MR-visible water content of the region of interest was obtained. Levels of myoinositol, choline, creatine and N -acetylaspartate were measured using a semiquantitative approach, with absolute reference calibration. In the control group, there was a gradual drop of water content over the first year of life; N -acetylaspartate, creatine and myoinositol levels showed no significant change with age, in contrast to the occipital, parietal and cerebellar regions. Choline showed a gradual decrease for the first 2 years of life and then remained fairly constant. In the abnormal group the water content was not significantly different. N -Acetylaspartate was decreased in patients with high serum sodium and high serum osmolarity, cobalamin C deficiency, Leigh disease and one case of methylmalonic acidemia. Decreased creatine was also found in Leigh disease, and decreased choline in Galloway-Mowat syndrome and Wilson disease. Myoinositol was elevated in the patient with abnormally high serum sodium, and decreased in the hemolytic-uremic syndrome. (orig.)

  7. Computational Stimulation of the Basal Ganglia Neurons with Cost Effective Delayed Gaussian Waveforms

    Directory of Open Access Journals (Sweden)

    Mohammad Daneshzand

    2017-08-01

    Full Text Available Deep brain stimulation (DBS has compelling results in the desynchronization of the basal ganglia neuronal activities and thus, is used in treating the motor symptoms of Parkinson's disease (PD. Accurate definition of DBS waveform parameters could avert tissue or electrode damage, increase the neuronal activity and reduce energy cost which will prolong the battery life, hence avoiding device replacement surgeries. This study considers the use of a charge balanced Gaussian waveform pattern as a method to disrupt the firing patterns of neuronal cell activity. A computational model was created to simulate ganglia cells and their interactions with thalamic neurons. From the model, we investigated the effects of modified DBS pulse shapes and proposed a delay period between the cathodic and anodic parts of the charge balanced Gaussian waveform to desynchronize the firing patterns of the GPe and GPi cells. The results of the proposed Gaussian waveform with delay outperformed that of rectangular DBS waveforms used in in-vivo experiments. The Gaussian Delay Gaussian (GDG waveforms achieved lower number of misses in eliciting action potential while having a lower amplitude and shorter length of delay compared to numerous different pulse shapes. The amount of energy consumed in the basal ganglia network due to GDG waveforms was dropped by 22% in comparison with charge balanced Gaussian waveforms without any delay between the cathodic and anodic parts and was also 60% lower than a rectangular charged balanced pulse with a delay between the cathodic and anodic parts of the waveform. Furthermore, by defining a Synchronization Level metric, we observed that the GDG waveform was able to reduce the synchronization of GPi neurons more effectively than any other waveform. The promising results of GDG waveforms in terms of eliciting action potential, desynchronization of the basal ganglia neurons and reduction of energy consumption can potentially enhance the

  8. Quantitation of the human basal ganglia with Positron Emission Tomography

    International Nuclear Information System (INIS)

    Bendriem, B.; Dewey, S.L.; Schlyer, D.J.; Wolf, A.P.; Volkow, N.D.

    1990-01-01

    The accurate measurement of the concentration of a radioisotope in small structures with PET requires a correction for quantitation loss due to the partial volume effect and the effect of scattered radiation. To evaluate errors associated with measures in the human basal ganglia (BG) we have built a unilateral model of the BG that we have inserted in a 20 cm cylinder. The recovery coefficient (RC = measured activity/true activity) for our BG phantom has been measured on a CTI tomograph (model 931-08/12) with different background concentrations (contrast) and at different axial locations in the gantry. The BG was visualized on 4 or 5 slices depending on its position in the gantry and on the contrast used. The RC was 0.75 with no background (contrast equal to 1.0). Increasing the relative radioactivity concentration in the background increased the RC from 0.75 to 2.00 when the contrast was -0.7 (BG 2 ). These results show that accurate RC correction depends not only on the volume of the structure but also on its contrast with its surroundings as well as on the selection of the ROI. They also demonstrate that the higher the contrast the more sensitive to axial positioning PET measurements in the BG are. These data provide us with some information about the variability of PET measurements in small structure like the BG and we have proposed some strategies to improve the reproducibility. 18 refs., 3 figs., 5 tabs

  9. Effect of an 8-week practice of externally triggered speech on basal ganglia activity of stuttering and fluent speakers.

    Science.gov (United States)

    Toyomura, Akira; Fujii, Tetsunoshin; Kuriki, Shinya

    2015-04-01

    The neural mechanisms underlying stuttering are not well understood. It is known that stuttering appears when persons who stutter speak in a self-paced manner, but speech fluency is temporarily increased when they speak in unison with external trigger such as a metronome. This phenomenon is very similar to the behavioral improvement by external pacing in patients with Parkinson's disease. Recent imaging studies have also suggested that the basal ganglia are involved in the etiology of stuttering. In addition, previous studies have shown that the basal ganglia are involved in self-paced movement. Then, the present study focused on the basal ganglia and explored whether long-term speech-practice using external triggers can induce modification of the basal ganglia activity of stuttering speakers. Our study of functional magnetic resonance imaging revealed that stuttering speakers possessed significantly lower activity in the basal ganglia than fluent speakers before practice, especially when their speech was self-paced. After an 8-week speech practice of externally triggered speech using a metronome, the significant difference in activity between the two groups disappeared. The cerebellar vermis of stuttering speakers showed significantly decreased activity during the self-paced speech in the second compared to the first experiment. The speech fluency and naturalness of the stuttering speakers were also improved. These results suggest that stuttering is associated with defective motor control during self-paced speech, and that the basal ganglia and the cerebellum are involved in an improvement of speech fluency of stuttering by the use of external trigger. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Automatic evaluation of speech rhythm instability and acceleration in dysarthrias associated with basal ganglia dysfunction

    Directory of Open Access Journals (Sweden)

    Jan eRusz

    2015-07-01

    Full Text Available Speech rhythm abnormalities are commonly present in patients with different neurodegenerative disorders. These alterations are hypothesized to be a consequence of disruption to the basal ganglia circuitry involving dysfunction of motor planning, programming and execution, which can be detected by a syllable repetition paradigm. Therefore, the aim of the present study was to design a robust signal processing technique that allows the automatic detection of spectrally-distinctive nuclei of syllable vocalizations and to determine speech features that represent rhythm instability and acceleration. A further aim was to elucidate specific patterns of dysrhythmia across various neurodegenerative disorders that share disruption of basal ganglia function. Speech samples based on repetition of the syllable /pa/ at a self-determined steady pace were acquired from 109 subjects, including 22 with Parkinson's disease (PD, 11 progressive supranuclear palsy (PSP, 9 multiple system atrophy (MSA, 24 ephedrone-induced parkinsonism (EP, 20 Huntington's disease (HD, and 23 healthy controls. Subsequently, an algorithm for the automatic detection of syllables as well as features representing rhythm instability and rhythm acceleration were designed. The proposed detection algorithm was able to correctly identify syllables and remove erroneous detections due to excessive inspiration and nonspeech sounds with a very high accuracy of 99.6%. Instability of vocal pace performance was observed in PSP, MSA, EP and HD groups. Significantly increased pace acceleration was observed only in the PD group. Although not significant, a tendency for pace acceleration was observed also in the PSP and MSA groups. Our findings underline the crucial role of the basal ganglia in the execution and maintenance of automatic speech motor sequences. We envisage the current approach to become the first step towards the development of acoustic technologies allowing automated assessment of rhythm

  11. Dopamine-dependent changes in the functional connectivity between basal ganglia and cerebral cortex in humans

    NARCIS (Netherlands)

    Williams, D; Tijssen, M; van Bruggen, G; Bosch, A; Insola, A; Di Lazzaro, V; Mazzone, P; Oliviero, A; Quartarone, A; Speelman, H; Brown, P

    2002-01-01

    We test the hypothesis that interaction between the human basal ganglia and cerebral cortex involves activity in multiple functional circuits characterized by their frequency of oscillation, phase characteristics, dopamine dependency and topography. To this end we took recordings from

  12. Mutations in SLC20A2 are a major cause of familial idiopathic basal ganglia calcification

    Science.gov (United States)

    Hsu, Sandy Chan; Sears, Renee L.; Lemos, Roberta R.; Quintáns, Beatriz; Huang, Alden; Spiteri, Elizabeth; Nevarez, Lisette; Mamah, Catherine; Zatz, Mayana; Pierce, Kerrie D.; Fullerton, Janice M.; Adair, John C.; Berner, Jon E.; Bower, Matthew; Brodaty, Henry; Carmona, Olga; Dobricić, Valerija; Fogel, Brent L.; García-Estevez, Daniel; Goldman, Jill; Goudreau, John L.; Hopfer, Suellen; Janković, Milena; Jaumà, Serge; Jen, Joanna C.; Kirdlarp, Suppachok; Klepper, Joerg; Kostić, Vladimir; Lang, Anthony E.; Linglart, Agnès; Maisenbacher, Melissa K.; Manyam, Bala V.; Mazzoni, Pietro; Miedzybrodzka, Zofia; Mitarnun, Witoon; Mitchell, Philip B.; Mueller, Jennifer; Novaković, Ivana; Paucar, Martin; Paulson, Henry; Simpson, Sheila A.; Svenningsson, Per; Tuite, Paul; Vitek, Jerrold; Wetchaphanphesat, Suppachok; Williams, Charles; Yang, Michele; Schofield, Peter R.; de Oliveira, João R. M.; Sobrido, María-Jesús

    2014-01-01

    Familial idiopathic basal ganglia calcification (IBGC) or Fahr’s disease is a rare neurodegenerative disorder characterized by calcium deposits in the basal ganglia and other brain regions, which is associated with neuropsychiatric and motor symptoms. Familial IBGC is genetically heterogeneous and typically transmitted in an autosomal dominant fashion. We performed a mutational analysis of SLC20A2, the first gene found to cause IBGC, to assess its genetic contribution to familial IBGC. We recruited 218 subjects from 29 IBGC-affected families of varied ancestry and collected medical history, neurological exam, and head CT scans to characterize each patient’s disease status. We screened our patient cohort for mutations in SLC20A2. Twelve novel (nonsense, deletions, missense, and splice site) potentially pathogenic variants, one synonymous variant, and one previously reported mutation were identified in 13 families. Variants predicted to be deleterious cosegregated with disease in five families. Three families showed nonsegregation with clinical disease of such variants, but retrospective review of clinical and neuroimaging data strongly suggested previous misclassification. Overall, mutations in SLC20A2 account for as many as 41 % of our familial IBGC cases. Our screen in a large series expands the catalog of SLC20A2 mutations identified to date and demonstrates that mutations in SLC20A2 are a major cause of familial IBGC. Non-perfect segregation patterns of predicted deleterious variants highlight the challenges of phenotypic assessment in this condition with highly variable clinical presentation. PMID:23334463

  13. Basal ganglia calcification on CT-scanning in children with acute lymphocytic leukemia

    International Nuclear Information System (INIS)

    Smith, D.; Bloch, S.; Al-Rashid, R.A.

    1980-01-01

    Calcification occurring in the basal ganglia in children with acute hympocytic leukemia following therapy is uncommon and to the best of our knowledge has not been reported prior to therapy. Eleven cases of bilateral symmetrical calcification in the basal ganglia were noted in 2350 CT scans, two being in children with acute lymphocytic leukemia. In one of the two cases, calcification was present prior to therapy. (orig.)

  14. Creation of computerized 3D MRI-integrated atlases of the human basal ganglia and thalamus

    Directory of Open Access Journals (Sweden)

    Abbas F. Sadikot

    2011-09-01

    Full Text Available Functional brain imaging and neurosurgery in subcortical areas often requires visualization of brain nuclei beyond the resolution of current Magnetic Resonance Imaging (MRI methods. We present techniques used to create: 1 a lower resolution 3D atlas, based on the Schaltenbrand and Wahren print atlas, which was integrated into a stereotactic neurosurgery planning and visualization platform (VIPER; and 2 a higher resolution 3D atlas derived from a single set of manually segmented histological slices containing nuclei of the basal ganglia, thalamus, basal forebrain and medial temporal lobe. Both atlases were integrated to a canonical MRI (Colin27 from a young male participant by manually identifying homologous landmarks. The lower resolution atlas was then warped to fit the MRI based on the identified landmarks. A pseudo-MRI representation of the high-resolution atlas was created, and a nonlinear transformation was calculated in order to match the atlas to the template MRI. The atlas can then be warped to match the anatomy of Parkinson’s disease surgical candidates by using 3D automated nonlinear deformation methods. By way of functional validation of the atlas, the location of the sensory thalamus was correlated with stereotactic intraoperative physiological data. The position of subthalamic electrode positions in patients with Parkinson’s disease was also evaluated in the atlas-integrated MRI space. Finally, probabilistic maps of subthalamic stimulation electrodes were developed, in order to allow group analysis of the location of contacts associated with the best motor outcomes. We have therefore developed, and are continuing to validate, a high-resolution computerized MRI-integrated 3D histological atlas, which is useful in functional neurosurgery, and for functional and anatomical studies of the human basal ganglia, thalamus and basal forebrain.

  15. Dysfunctions of the basal ganglia-cerebellar-thalamo-cortical system produce motor tics in Tourette syndrome.

    Directory of Open Access Journals (Sweden)

    Daniele Caligiore

    2017-03-01

    Full Text Available Motor tics are a cardinal feature of Tourette syndrome and are traditionally associated with an excess of striatal dopamine in the basal ganglia. Recent evidence increasingly supports a more articulated view where cerebellum and cortex, working closely in concert with basal ganglia, are also involved in tic production. Building on such evidence, this article proposes a computational model of the basal ganglia-cerebellar-thalamo-cortical system to study how motor tics are generated in Tourette syndrome. In particular, the model: (i reproduces the main results of recent experiments about the involvement of the basal ganglia-cerebellar-thalamo-cortical system in tic generation; (ii suggests an explanation of the system-level mechanisms underlying motor tic production: in this respect, the model predicts that the interplay between dopaminergic signal and cortical activity contributes to triggering the tic event and that the recently discovered basal ganglia-cerebellar anatomical pathway may support the involvement of the cerebellum in tic production; (iii furnishes predictions on the amount of tics generated when striatal dopamine increases and when the cortex is externally stimulated. These predictions could be important in identifying new brain target areas for future therapies. Finally, the model represents the first computational attempt to study the role of the recently discovered basal ganglia-cerebellar anatomical links. Studying this non-cortex-mediated basal ganglia-cerebellar interaction could radically change our perspective about how these areas interact with each other and with the cortex. Overall, the model also shows the utility of casting Tourette syndrome within a system-level perspective rather than viewing it as related to the dysfunction of a single brain area.

  16. Dysfunctions of the basal ganglia-cerebellar-thalamo-cortical system produce motor tics in Tourette syndrome.

    Science.gov (United States)

    Caligiore, Daniele; Mannella, Francesco; Arbib, Michael A; Baldassarre, Gianluca

    2017-03-01

    Motor tics are a cardinal feature of Tourette syndrome and are traditionally associated with an excess of striatal dopamine in the basal ganglia. Recent evidence increasingly supports a more articulated view where cerebellum and cortex, working closely in concert with basal ganglia, are also involved in tic production. Building on such evidence, this article proposes a computational model of the basal ganglia-cerebellar-thalamo-cortical system to study how motor tics are generated in Tourette syndrome. In particular, the model: (i) reproduces the main results of recent experiments about the involvement of the basal ganglia-cerebellar-thalamo-cortical system in tic generation; (ii) suggests an explanation of the system-level mechanisms underlying motor tic production: in this respect, the model predicts that the interplay between dopaminergic signal and cortical activity contributes to triggering the tic event and that the recently discovered basal ganglia-cerebellar anatomical pathway may support the involvement of the cerebellum in tic production; (iii) furnishes predictions on the amount of tics generated when striatal dopamine increases and when the cortex is externally stimulated. These predictions could be important in identifying new brain target areas for future therapies. Finally, the model represents the first computational attempt to study the role of the recently discovered basal ganglia-cerebellar anatomical links. Studying this non-cortex-mediated basal ganglia-cerebellar interaction could radically change our perspective about how these areas interact with each other and with the cortex. Overall, the model also shows the utility of casting Tourette syndrome within a system-level perspective rather than viewing it as related to the dysfunction of a single brain area.

  17. What basal ganglia changes underlie the parkinsonian state? The significance of neuronal oscillatory activity

    Science.gov (United States)

    Quiroga-Varela, A.; Walters, J.R.; Brazhnik, E.; Marin, C.; Obeso, J.A.

    2014-01-01

    One well accepted functional feature of the parkinsonian state is the recording of enhanced beta oscillatory activity in the basal ganglia. This has been demonstrated in patients with Parkinson's disease (PD) and in animal models such as the rat with 6-hydroxydopamine (6-OHDA)-induced lesion and 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-treated monkeys, all of which are associated with severe striatal dopamine depletion. Neuronal hyper-synchronization in the beta (or any other) band is not present despite the presence of bradykinetic features in the rat and monkey models, suggesting that increased beta band power may arise when nigro-striatal lesion is advanced and that it is not an essential feature of the early parkinsonian state. Similar observations and conclusions have been previously made for increased neuronal firing rate in the subthalamic and globus pallidus pars interna nuclei. Accordingly, it is suggested that early parkinsonism may be associated with dynamic changes in basal ganglia output activity leading to reduced movement facilitation that may be an earlier feature of the parkinsonian state. PMID:23727447

  18. Acute bilateral basal ganglia lesions in diabetic uraemia: diffusion-weighted MRI

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eun Ja; Park, Chan Sup [Kwandong University, College of Medicine, Department of Radiology, Myongji Hospital, Koyang-City, Gyunggi-Do (Korea); Park, Jong-Ho [Myongji Hospital, Kwandong University, College of Medicine, Department of Neurology, Koyang (Korea); Ihn, Yon kwon; Kim, Young Joo [The Catholic University of Korea, Department of Radiology, Seoul (Korea); Lee, Seon Kyu [University of Toronto, Department of Medical Imaging, Toronto Western Hospital, Toronto (Canada)

    2007-12-15

    We studied four patients with diabetes mellitus and chronic renal failure who developed sudden choreic movement disorders. The clinical manifestations, laboratory findings, MR imaging findings, and clinical outcome in each patient were evaluated. All four patients had long-term diabetes mellitus and severe azotaemia. Brain MR findings consisted of bilateral symmetric basal ganglia lesions, with decreased signal intensity on T1-weighted images and increased signal intensity on T2-weighted images. All three patients who underwent diffusion-weighted MR imaging (DWI) showed signal intensities similar to those of the surroundings in regions corresponding to increased signal intensity on T2-weighted images, with slightly increased apparent diffusion coefficient (ADC) values. Two of the patients showed small focal areas of restricted diffusion within the basal ganglia lesions. After haemodialysis, follow-up MR imaging in all patients demonstrated that the basal ganglia lesions had regressed markedly, with some residual changes. The movement disorders also improved in all patients. A syndrome associated with acute bilateral basal ganglia lesions in diabetic uraemic patients is rare, with reversible changes demonstrated by clinical and imaging findings. DWI showed that the bilateral basal ganglia lesions in this syndrome were primarily vasogenic in origin, although there were small foci of cytotoxic oedema within the lesions. (orig.)

  19. The basal ganglia and the cerebellum: nodes in an integrated network.

    Science.gov (United States)

    Bostan, Andreea C; Strick, Peter L

    2018-04-11

    The basal ganglia and the cerebellum are considered to be distinct subcortical systems that perform unique functional operations. The outputs of the basal ganglia and the cerebellum influence many of the same cortical areas but do so by projecting to distinct thalamic nuclei. As a consequence, the two subcortical systems were thought to be independent and to communicate only at the level of the cerebral cortex. Here, we review recent data showing that the basal ganglia and the cerebellum are interconnected at the subcortical level. The subthalamic nucleus in the basal ganglia is the source of a dense disynaptic projection to the cerebellar cortex. Similarly, the dentate nucleus in the cerebellum is the source of a dense disynaptic projection to the striatum. These observations lead to a new functional perspective that the basal ganglia, the cerebellum and the cerebral cortex form an integrated network. This network is topographically organized so that the motor, cognitive and affective territories of each node in the network are interconnected. This perspective explains how synaptic modifications or abnormal activity at one node can have network-wide effects. A future challenge is to define how the unique learning mechanisms at each network node interact to improve performance.

  20. Functional neuroanatomy of the basal ganglia as studied by dual-probe microdialysis

    International Nuclear Information System (INIS)

    O'Connor, William T.

    1998-01-01

    Dual probe microdialysis was employed in intact rat brain to investigate the effect of intrastriatal perfusion with selective dopamine D 1 and D 2 receptor agonists and with c-fos antisense oligonucleotide on (a) local GABA release in the striatum; (b) the internal segment of the globus pallidus and the substantia nigra pars reticulata, which is the output site of the strionigral GABA pathway; and (c) the external segment of the globus pallidus, which is the output site of the striopallidal GABA pathway. The data provide functional in vivo evidence for a selective dopamine D 1 receptor-mediated activation of the direct strionigral GABA pathway and a selective dopamine D 2 receptor inhibition of the indirect striopallidal GABA pathway and provides a neuronal substrate for parallel processing in the basal ganglia regulation of motor function. Taken together, these findings offer new therapeutic strategies for the treatment of dopamine-linked disorders such as Parkinson's disease, Huntington's disease, and schizophrenia

  1. Hypofractionated Stereotactic Radiosurgery in a Large Bilateral Thalamic and Basal Ganglia Arteriovenous Malformation

    Directory of Open Access Journals (Sweden)

    Janet Lee

    2013-01-01

    Full Text Available Purpose. Arteriovenous malformations (AVMs in the basal ganglia and thalamus have a more aggressive natural history with a higher morbidity and mortality than AVMs in other locations. Optimal treatment—complete obliteration without new neurological deficits—is often challenging. We present a patient with a large bilateral basal ganglia and thalamic AVM successfully treated with hypofractionated stereotactic radiosurgery (HFSRS with intensity modulated radiotherapy (IMRT. Methods. The patient was treated with hypofractionated stereotactic radiosurgery to 30 Gy at margin in 5 fractions of 9 static fields with a minimultileaf collimator and intensity modulated radiotherapy. Results. At 10 months following treatment, digital subtraction angiography showed complete obliteration of the AVM. Conclusions. Large bilateral thalamic and basal ganglia AVMs can be successfully treated with complete obliteration by HFSRS with IMRT with relatively limited toxicity. Appropriate caution is recommended.

  2. Chronological changes in nonhaemorrhagic brain infarcts with short T1 in the cerebellum and basal ganglia

    Energy Technology Data Exchange (ETDEWEB)

    Komiyama, M.; Nakajima, H.; Nishikawa, M.; Yasui, T. [Dept. of Neurosurgery, Osaka City General Hospital, Miyakojima-Hondouri, Miyakojima, Osaka (Japan)

    2000-07-01

    Our purpose was to investigate nonhaemorrhagic infarcts with a short T1 in the cerebellum and basal ganglia. We carried out repeat MRI on 12 patients with infarcts in the cerebellum or basal ganglia with a short T1. Cerebellar cortical lesions showed high signal on T1-weighted spin-echo images beginning at 2 weeks, which became prominent from 3 weeks to 2 months, and persisted for as long as 14 months after the ictus. The basal ganglia lesions demonstrated slightly high signal from a week after the ictus, which became more intense thereafter. Signal intensity began to fade gradually after 2 months. High signal could be seen at the periphery until 5 months, and then disappeared, while low or isointense signal, seen in the central portion from day 20, persisted thereafter. (orig.)

  3. Prevalences of CT detected calcification in the basal ganglia in idiopathic hypoparathyroidism and pseudohypoparathyroidism

    International Nuclear Information System (INIS)

    Illum, F.; Dupont, E.; Aarhus Univ.; Aarhus Univ.

    1985-01-01

    Sixteen patients with idiopathic hypoparathyroidism (IHP) and eight patients with pseudohypoparathyroidism (PHP) were examined by CT scan of the brain. Calcification in the basal ganglia was observed in 11 patients with IHP (69%) and in all eight patients with PHP. Of the 19 patients with basal ganglia calcification, nine had calcifications in the cerebral cortex (47%), and four had calcifications in the cerebellum (21%). Observation of basal ganglia calcification on CT gave rise to suspicion of IHP or PHP in three patients (12%). The remaining patients were examined at varying time after diagnosis. Since arrest in growth of calcifications after institution of treatment has never been proven, the reported prevalences of calcifications may not be valid to the situation at the time of diagnosis. (orig.)

  4. Prevalences of CT detected calcification in the basal ganglia in idiopathic hypoparathyroidism and pseudohypoparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Illum, F.; Dupont, E.

    1985-01-01

    Sixteen patients with idiopathic hypoparathyroidism (IHP) and eight patients with pseudohypoparathyroidism (PHP) were examined by CT scan of the brain. Calcification in the basal ganglia was observed in 11 patients with IHP (69%) and in all eight patients with PHP. Of the 19 patients with basal ganglia calcification, nine had calcifications in the cerebral cortex (47%), and four had calcifications in the cerebellum (21%). Observation of basal ganglia calcification on CT gave rise to suspicion of IHP or PHP in three patients (12%). The remaining patients were examined at varying time after diagnosis. Since arrest in growth of calcifications after institution of treatment has never been proven, the reported prevalences of calcifications may not be valid to the situation at the time of diagnosis.

  5. Attenuated frontal and sensory inputs to the basal ganglia in cannabis users.

    Science.gov (United States)

    Blanco-Hinojo, Laura; Pujol, Jesus; Harrison, Ben J; Macià, Dídac; Batalla, Albert; Nogué, Santiago; Torrens, Marta; Farré, Magí; Deus, Joan; Martín-Santos, Rocío

    2017-07-01

    Heavy cannabis use is associated with reduced motivation. The basal ganglia, central in the motivation system, have the brain's highest cannabinoid receptor density. The frontal lobe is functionally coupled to the basal ganglia via segregated frontal-subcortical circuits conveying information from internal, self-generated activity. The basal ganglia, however, receive additional influence from the sensory system to further modulate purposeful behaviors according to the context. We postulated that cannabis use would impact functional connectivity between the basal ganglia and both internal (frontal cortex) and external (sensory cortices) sources of influence. Resting-state functional connectivity was measured in 28 chronic cannabis users and 29 controls. Selected behavioral tests included reaction time, verbal fluency and exposition to affective pictures. Assessments were repeated after one month of abstinence. Cannabis exposure was associated with (1) attenuation of the positive correlation between the striatum and areas pertaining to the 'limbic' frontal-basal ganglia circuit, and (2) attenuation of the negative correlation between the striatum and the fusiform gyrus, which is critical in recognizing significant visual features. Connectivity alterations were associated with lower arousal in response to affective pictures. Functional connectivity changes had a tendency to normalize after abstinence. The results overall indicate that frontal and sensory inputs to the basal ganglia are attenuated after chronic exposure to cannabis. This effect is consistent with the common behavioral consequences of chronic cannabis use concerning diminished responsiveness to both internal and external motivation signals. Such an impairment of the fine-tuning in the motivation system notably reverts after abstinence. © 2016 Society for the Study of Addiction.

  6. Global dysrhythmia of cerebro-basal ganglia-cerebellar networks underlies motor tics following striatal disinhibition.

    Science.gov (United States)

    McCairn, Kevin W; Iriki, Atsushi; Isoda, Masaki

    2013-01-09

    Motor tics, a cardinal symptom of Tourette syndrome (TS), are hypothesized to arise from abnormalities within cerebro-basal ganglia circuits. Yet noninvasive neuroimaging of TS has previously identified robust activation in the cerebellum. To date, electrophysiological properties of cerebellar activation and its role in basal ganglia-mediated tic expression remain unknown. We performed multisite, multielectrode recordings of single-unit activity and local field potentials from the cerebellum, basal ganglia, and primary motor cortex using a pharmacologic monkey model of motor tics/TS. Following microinjections of bicuculline into the sensorimotor putamen, periodic tics occurred predominantly in the orofacial region, and a sizable number of cerebellar neurons showed phasic changes in activity associated with tic episodes. Specifically, 64% of the recorded cerebellar cortex neurons exhibited increases in activity, and 85% of the dentate nucleus neurons displayed excitatory, inhibitory, or multiphasic responses. Critically, abnormal discharges of cerebellar cortex neurons and excitatory-type dentate neurons mostly preceded behavioral tic onset, indicating their central origins. Latencies of pathological activity in the cerebellum and primary motor cortex substantially overlapped, suggesting that aberrant signals may be traveling along divergent pathways to these structures from the basal ganglia. Furthermore, the occurrence of tic movement was most closely associated with local field potential spikes in the cerebellum and primary motor cortex, implying that these structures may function as a gate to release overt tic movements. These findings indicate that tic-generating networks in basal ganglia mediated tic disorders extend beyond classical cerebro-basal ganglia circuits, leading to global network dysrhythmia including cerebellar circuits.

  7. Clinical studies of the calcification of the basal ganglia as disclosed by computed tomography

    International Nuclear Information System (INIS)

    Node, Yoji; Nakazawa, Shozo

    1983-01-01

    One hundred and twenty-nine of the 12,645 patients (1.0%) were found to have attenuating changes suggesting calcification of the basal ganglia. Thirty-seven of those patients were male and 92 were female. The calcification was bilateral and grossly symmetric in 108 of these patients (83.7%), while it was unilateral in 21 (16.3%). In the unilaterally located cases, 15 were on the left side and 6 were on the right side. In 128 of these patients (99.2%), calcification was located in the globus pallidus. Only one patient, whose diagnosis was hypoparathyroidism, had calcification in both the globus pallidus and the head of the caudate nucleus. The patients' ages ranged from 10 to 85 years (mean, 58), but 88.4% of the patients were more than 40 years old at the time of the CT scanning. The attenuation values of the lesions varied from 35 to 375 EMI units (mean, 55.7). Skull radiographs were performed in 120 of the 129 patients. Calcification was detected in only one patient, a 76-year-old woman, whose diagnosis was myasthenia gravis. The clinical diagnoses of the 129 patients were as follows: 37, headache; 22, cerebrovascular diseases (19, occlusive cerebrovascular diseases); 20, vertigo and/or tinnitus; 12, psychiatric disorders; 5, Parkinson's Syndrome; 2, hypopara thyroidism; 2, Fahr's disease; 2, familial basal ganglia calcification; 2, epilepsy, and 25, miscellaneous (including carcinoma, brain tumor, and trauma). Nervous system abnormalities were observed in 41 of the 129 patients (31.2%). Mental signs, such as disturbance of recent memory, mental retardation, and dementia, were noted in 14 patients. Movement disorders were noted in 13 patients. Other nervous-system abnormalities were sensory disturbances (5 patients) and seizures (4 patients). Abnormal EEG activities were noted in 9 patients; three patients showed epileptic activity, and six had a pathologically slow rhythm. (J.P.N.)

  8. Clinical studies of the calcification of the basal ganglia as disclosed by computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Node, Yoji; Nakazawa, Shozo (Nippon Medical School, Tokyo)

    1983-04-01

    One hundred and twenty-nine of the 12,645 patients (1.0%) were found to have attenuating changes suggesting calcification of the basal ganglia. Thirty-seven of those patients were male and 92 were female. The calcification was bilateral and grossly symmetric in 108 of these patients (83.7%), while it was unilateral in 21 (16.3%). In the unilaterally located cases, 15 were on the left side and 6 were on the right side. In 128 of these patients (99.2%), calcification was located in the globus pallidus. Only one patient, whose diagnosis was hypoparathyroidism, had calcification in both the globus pallidus and the head of the caudate nucleus. The patients' ages ranged from 10 to 85 years (mean, 58), but 88.4% of the patients were more than 40 years old at the time of the CT scanning. The attenuation values of the lesions varied from 35 to 375 EMI units (mean, 55.7). Skull radiographs were performed in 120 of the 129 patients. Calcification was detected in only one patient, a 76-year-old woman, whose diagnosis was myasthenia gravis. The clinical diagnoses of the 129 patients were as follows: 37, headache; 22, cerebrovascular diseases (19, occlusive cerebrovascular diseases); 20, vertigo and/or tinnitus; 12, psychiatric disorders; 5, Parkinson's Syndrome; 2, hypopara thyroidism; 2, Fahr's disease; 2, familial basal ganglia calcification; 2, epilepsy, and 25, miscellaneous (including carcinoma, brain tumor, and trauma). Nervous system abnormalities were observed in 41 of the 129 patients (31.2%). Mental signs, such as disturbance of recent memory, mental retardation, and dementia, were noted in 14 patients. Movement disorders were noted in 13 patients. Other nervous-system abnormalities were sensory disturbances (5 patients) and seizures (4 patients). Abnormal EEG activities were noted in 9 patients; three patients showed epileptic activity, and six had a pathologically slow rhythm.

  9. Cavitary Cryptogenic Organizing Pneumonia and abnormalities of the Basal Ganglia Case presentation

    International Nuclear Information System (INIS)

    Prieto, Enrique; Mora, Alfonso Sergio

    2007-01-01

    Cryptogenic Organizing Pneumonia (COP) is a pulmonary disorder with a wide spectrum of radiological features. A case of a young patient of 16 years old is shown with CAT appearance of multiple cavitary nodules in both lungs that responded with a complete resolution after corticosteroid therapy. This patient also reveals abnormalities of the basal ganglia as the result of hypoxic ischemic encephalopathy associated with the acute presentation of this disorder. We justify the inclusion of COP in the differential diagnosis of multiple cavitary nodules, and it is discussed the differential diagnosis of her abnormalities of the basal ganglia

  10. Computed tomography of basal ganglia calcifications in pseudo- and idiopathic hypoparathyroidism

    International Nuclear Information System (INIS)

    Fukunaga, Masao; Otsuka, Nobuaki; Ono, Shimato; Kajihara, Yasumasa; Nishishita, Soichi; Morita, Rikushi; Nakano, Yoshihisa; Yamamoto, Itsuo; Torizuka, Kanji.

    1987-01-01

    It is well known that patients with pseudo (PHP)- and idiopathic (IHP) hypoparathyroidism are frequently associated with intracranial calcifications. The relative sensitivity of computed tomography (CT) and conventional skull radiography in detecting basal ganglia calcifications was studied in two patients with PHP and six with IHP. CT was more sensitive: the detection rate was 71 % (5/7) for CT and 14 % (1/7) for skull radiography. Furthermore, patients with more prolonged hypocalcemia showed a higher incidence of calcifications. Thus, CT was useful as a diagnostic technique in the early detection of calcified basal ganglia. (author)

  11. Computed tomography of basal ganglia calcifications in pseudo- and idiopathic hypoparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Fukunaga, Masao; Otsuka, Nobuaki; Ono, Shimato; Kajihara, Yasumasa; Nishishita, Soichi; Morita, Rikushi; Nakano, Yoshihisa; Yamamoto, Itsuo; Torizuka, Kanji.

    1987-12-01

    It is well known that patients with pseudo (PHP)- and idiopathic (IHP) hypoparathyroidism are frequently associated with intracranial calcifications. The relative sensitivity of computed tomography (CT) and conventional skull radiography in detecting basal ganglia calcifications was studied in two patients with PHP and six with IHP. CT was more sensitive: the detection rate was 71 % (5/7) for CT and 14 % (1/7) for skull radiography. Furthermore, patients with more prolonged hypocalcemia showed a higher incidence of calcifications. Thus, CT was useful as a diagnostic technique in the early detection of calcified basal ganglia.

  12. Primary choriocarcinoma of the bilateral basal ganglia presenting in a teenaged male

    Directory of Open Access Journals (Sweden)

    Brandon C. Perry, MD

    2017-03-01

    Full Text Available Primary intracranial choriocarcinoma (PICCC, a type of germ-cell tumor, is a very rare primary tumor of the central nervous system that generally arises in the pineal or suprasellar region. We present a case of a teenage boy with PICCC of the bilateral basal ganglia, an anatomic site for which we were unable to find the previous reports. We offer discussion of the differential diagnosis, imaging characteristics, and prognosis of PICCC and germ-cell tumors of the basal ganglia, in the hope that it will increase awareness and allow for early detection.

  13. Basal ganglia calcification on CT in adult patients with Down's syndrome

    International Nuclear Information System (INIS)

    Ono, Yoshiro; Yoshida, Hironobu; Yoshimasu, Fumio; Higashi, Yuji.

    1987-01-01

    Fourteen adult cases with Down's syndrome were examined on cranial CT scan, and 5 of them (35.7 %) showed basal ganglia calcification (BGC). The incidence of BGC in the present cases was very high in comparison with the one in general population (0.3 ∼ 1.5 %). Abnormalities of calcium metabolism or dysfunctions of the basal ganglia were absent in each case with BGC. Calcifications were exclusively located in globus pallidus. It is considered that BGC found in the present cases may be due to the premature aging process in Down's syndrome. (author)

  14. Clinical observation of hemocoagulase combined with aminomethylbenzoic acid in the treatment of basal ganglia hemorrhage

    Directory of Open Access Journals (Sweden)

    Min SU

    2014-07-01

    Full Text Available Patients with cerebral hemorrhage in basal ganglia were treated with hemocoagulase combined with aminomethylbenzoic acid from May 2010 to April 2013 in our hospital, and hematoma volume and neurological impairment were compared with the control group before and after treatment. This study confirmed that hemocoagulase combined with aninomethylbenzoic acid is a safe and effective method for cerebral hemorrhage in basal ganglia. It can effectively prevent the hematoma enlargement and improve neurological function and prognosis. doi: 10.3969/j.issn.1672-6731.2014.07.014

  15. Analysis of delay-induced basal ganglia oscillations: the role of external excitatory nuclei

    Science.gov (United States)

    Haidar, Ihab; Pasillas-Lépine, William; Panteley, Elena; Chaillet, Antoine; Palfi, Stéphane; Senova, Suhan

    2014-09-01

    Basal ganglia are interconnected deep brain structures involved in movement generation. Their persistent beta-band oscillations (13-30 Hz) are known to be linked to Parkinson's disease motor symptoms. In this paper, we provide conditions under which these oscillations may occur, by explicitly considering the role of the pedunculopontine nucleus (PPN). We analyse the existence of equilibria in the associated firing-rate dynamics and study their stability by relying on a delayed multiple-input/multiple-output (MIMO) frequency analysis. Our analysis suggests that the PPN has an influence on the generation of pathological beta-band oscillations. These results are illustrated by simulations that confirm numerically the analytic predictions of our two main theorems.

  16. Quetiapine responsive catatonia in an autistic patient with comorbid bipolar disorder and idiopathic basal ganglia calcification.

    Science.gov (United States)

    Ishitobi, Makoto; Kawatani, Masao; Asano, Mizuki; Kosaka, Hirotaka; Goto, Takashi; Hiratani, Michio; Wada, Yuji

    2014-10-01

    Bipolar disorder (BD) has been linked with the manifestation of catatonia in subjects with autism spectrum disorders (ASD). Idiopathic basal ganglia calcification (IBGC) is characterized by movement disorders and various neuropsychiatric disturbances including mood disorder. We present a patient with ASD and IBGC who developed catatonia presenting with prominent dystonic feature caused by comorbid BD, which was treated effectively with quetiapine. In addition to considering the possibility of neurodegenerative disease, careful psychiatric interventions are important to avoid overlooking treatable catatonia associated with BD in cases of ASD presenting with both prominent dystonic features and apparent fluctuation of the mood state. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  17. Basal ganglia-dependent processes in recalling learned visual-motor adaptations.

    Science.gov (United States)

    Bédard, Patrick; Sanes, Jerome N

    2011-03-01

    Humans learn and remember motor skills to permit adaptation to a changing environment. During adaptation, the brain develops new sensory-motor relationships that become stored in an internal model (IM) that may be retained for extended periods. How the brain learns new IMs and transforms them into long-term memory remains incompletely understood since prior work has mostly focused on the learning process. A current model suggests that basal ganglia, cerebellum, and their neocortical targets actively participate in forming new IMs but that a cerebellar cortical network would mediate automatization. However, a recent study (Marinelli et al. 2009) reported that patients with Parkinson's disease (PD), who have basal ganglia dysfunction, had similar adaptation rates as controls but demonstrated no savings at recall tests (24 and 48 h). Here, we assessed whether a longer training session, a feature known to increase long-term retention of IM in healthy individuals, could allow PD patients to demonstrate savings. We recruited PD patients and age-matched healthy adults and used a visual-motor adaptation paradigm similar to the study by Marinelli et al. (2009), doubling the number of training trials and assessed recall after a short and a 24-h delay. We hypothesized that a longer training session would allow PD patients to develop an enhanced representation of the IM as demonstrated by savings at the recall tests. Our results showed that PD patients had similar adaptation rates as controls but did not demonstrate savings at both recall tests. We interpret these results as evidence that fronto-striatal networks have involvement in the early to late phase of motor memory formation, but not during initial learning.

  18. How preparation changes the need for top-down control of the basal ganglia when inhibiting premature actions

    NARCIS (Netherlands)

    Jahfari, S.; Verbruggen, F.; Frank, M.J.; Waldorp, L.J.; Colzato, L.; Ridderinkhof, K.R.; Forstmann, B.U.

    2012-01-01

    Goal-oriented signals from the prefrontal cortex gate the selection of appropriate actions in the basal ganglia. Key nodes within this fronto-basal ganglia action regulation network are increasingly engaged when one anticipates the need to inhibit and override planned actions. Here, we ask how the

  19. Disentangling the Role of Cortico-Basal Ganglia Loops in Top-Down and Bottom-Up Visual Attention: An Investigation of Attention Deficits in Parkinson Disease.

    Science.gov (United States)

    Tommasi, Giorgio; Fiorio, Mirta; Yelnik, Jérôme; Krack, Paul; Sala, Francesca; Schmitt, Emmanuelle; Fraix, Valérie; Bertolasi, Laura; Le Bas, Jean-François; Ricciardi, Giuseppe Kenneth; Fiaschi, Antonio; Theeuwes, Jan; Pollak, Pierre; Chelazzi, Leonardo

    2015-06-01

    It is solidly established that top-down (goal-driven) and bottom-up (stimulus-driven) attention mechanisms depend on distributed cortical networks, including prefrontal and frontoparietal regions. On the other hand, it is less clear whether the BG also contribute to one or the other of these mechanisms, or to both. The current study was principally undertaken to clarify this issue. Parkinson disease (PD), a neurodegenerative disorder primarily affecting the BG, has proven to be an effective model for investigating the contribution of the BG to different brain functions; therefore, we set out to investigate deficits of top-down and bottom-up attention in a selected cohort of PD patients. With this objective in mind, we compared the performance on three computerized tasks of two groups of 12 parkinsonian patients (assessed without any treatment), one otherwise pharmacologically treated and the other also surgically treated, with that of a group of controls. The main behavioral tool for our study was an attentional capture task, which enabled us to tap the competition between top-down and bottom-up mechanisms of visual attention. This task was suitably combined with a choice RT and a simple RT task to isolate any specific deficit of attention from deficits in motor response selection and initiation. In the two groups of patients, we found an equivalent increase of attentional capture but also comparable delays in target selection in the absence of any salient distractor (reflecting impaired top-down mechanisms) and movement initiation compared with controls. In contrast, motor response selection processes appeared to be prolonged only in the operated patients. Our results confirm that the BG are involved in both motor and cognitive domains. Specifically, damage to the BG, as it occurs in PD, leads to a distinct deficit of top-down control of visual attention, and this can account, albeit indirectly, for the enhancement of attentional capture, reflecting weakened

  20. Ictal and peri-ictal oscillations in the human basal ganglia in temporal lobe epilepsy

    Czech Academy of Sciences Publication Activity Database

    Rektor, I.; Kuba, R.; Brázdil, M.; Halámek, Josef; Jurák, Pavel

    2011-01-01

    Roč. 20, č. 3 (2011), s. 512-517 ISSN 1525-5050 Institutional research plan: CEZ:AV0Z20650511 Keywords : basal ganglia * oscillations * epilepsy * ictal Subject RIV: FH - Neurology Impact factor: 2.335, year: 2011

  1. Stroke After Minor Head Trauma in Infants and Young Children With Basal Ganglia Calcification: A Lenticulostriate Vasculopathy?

    Science.gov (United States)

    Goraya, Jatinder Singh; Berry, Shivankshi; Saggar, Kavita; Ahluwalia, Archana

    2018-02-01

    The authors retrospectively reviewed charts of the children with basal ganglia stroke who either had preceding minor head injury or showed basal ganglia calcification on computed tomography (CT) scan. Twenty children, 14 boys and 6 girls were identified. Eighteen were aged between 7 months to 17 months. Presentation was with hemiparesis in 17 and seizures in 3. Preceding minor head trauma was noted in 18. Family history was positive in 1 case. Bilateral basal ganglia calcification on CT scan was noted in 18. Brain magnetic resonance imaging done in 18 infants showed acute or chronic infarcts in basal ganglia. Results of other laboratory and radiological investigations were normal. Four infants were lost to follow-up, 9 achieved complete or nearly completely recovery, and 7 had persistent neurological deficits. Basal ganglia calcification likely represents mineralized lenticulostriate arteries, a marker of lenticulostriate vasculopathy. Abnormal lenticulostriate vessels are vulnerable to injury and thrombosis after minor head trauma resulting in stroke.

  2. Decreased basal ganglia activation in subjects with chronic fatigue syndrome: association with symptoms of fatigue.

    Directory of Open Access Journals (Sweden)

    Andrew H Miller

    Full Text Available Reduced basal ganglia function has been associated with fatigue in neurologic disorders, as well as in patients exposed to chronic immune stimulation. Patients with chronic fatigue syndrome (CFS have been shown to exhibit symptoms suggestive of decreased basal ganglia function including psychomotor slowing, which in turn was correlated with fatigue. In addition, CFS patients have been found to exhibit increased markers of immune activation. In order to directly test the hypothesis of decreased basal ganglia function in CFS, we used functional magnetic resonance imaging to examine neural activation in the basal ganglia to a reward-processing (monetary gambling task in a community sample of 59 male and female subjects, including 18 patients diagnosed with CFS according to 1994 CDC criteria and 41 non-fatigued healthy controls. For each subject, the average effect of winning vs. losing during the gambling task in regions of interest (ROI corresponding to the caudate nucleus, putamen, and globus pallidus was extracted for group comparisons and correlational analyses. Compared to non-fatigued controls, patients with CFS exhibited significantly decreased activation in the right caudate (p = 0.01 and right globus pallidus (p = 0.02. Decreased activation in the right globus pallidus was significantly correlated with increased mental fatigue (r2 = 0.49, p = 0.001, general fatigue (r2 = 0.34, p = 0.01 and reduced activity (r2 = 0.29, p = 0.02 as measured by the Multidimensional Fatigue Inventory. No such relationships were found in control subjects. These data suggest that symptoms of fatigue in CFS subjects were associated with reduced responsivity of the basal ganglia, possibly involving the disruption of projections from the globus pallidus to thalamic and cortical networks.

  3. Localization of Basal Ganglia and Thalamic Damage in Dyskinetic Cerebral Palsy.

    Science.gov (United States)

    Aravamuthan, Bhooma R; Waugh, Jeff L

    2016-01-01

    Dyskinetic cerebral palsy affects 15%-20% of patients with cerebral palsy. Basal ganglia injury is associated with dyskinetic cerebral palsy, but the patterns of injury within the basal ganglia predisposing to dyskinetic cerebral palsy are unknown, making treatment difficult. For example, deep brain stimulation of the globus pallidus interna improves dystonia in only 40% of patients with dyskinetic cerebral palsy. Basal ganglia injury heterogeneity may explain this variability. To investigate this, we conducted a qualitative systematic review of basal ganglia and thalamic damage in dyskinetic cerebral palsy. Reviews and articles primarily addressing genetic or toxic causes of cerebral palsy were excluded yielding 22 studies (304 subjects). Thirteen studies specified the involved basal ganglia nuclei (subthalamic nucleus, caudate, putamen, globus pallidus, or lentiform nuclei, comprised by the putamen and globus pallidus). Studies investigating the lentiform nuclei (without distinguishing between the putamen and globus pallidus) showed that all subjects (19 of 19) had lentiform nuclei damage. Studies simultaneously but independently investigating the putamen and globus pallidus also showed that all subjects (35 of 35) had lentiform nuclei damage (i.e., putamen or globus pallidus damage); this was followed in frequency by damage to the putamen alone (70 of 101, 69%), the subthalamic nucleus (17 of 25, 68%), the thalamus (88 of 142, 62%), the globus pallidus (7/35, 20%), and the caudate (6 of 47, 13%). Globus pallidus damage was almost always coincident with putaminal damage. Noting consistent involvement of the lentiform nuclei in dyskinetic cerebral palsy, these results could suggest two groups of patients with dyskinetic cerebral palsy: those with putamen-predominant damage and those with panlenticular damage involving both the putamen and the globus pallidus. Differentiating between these groups could help predict response to therapies such as deep brain

  4. Basal ganglia germinoma in children with associated ipsilateral cerebral and brain stem hemiatrophy

    Energy Technology Data Exchange (ETDEWEB)

    Ozelame, Rodrigo V.; Shroff, Manohar; Wood, Bradley; Bouffet, Eric; Bartels, Ute; Drake, James M.; Hawkins, Cynthia; Blaser, Susan [Hospital for Sick Children, Department of Diagnostic Imaging, Toronto, Ontario (Canada)

    2006-04-15

    Germinoma is the most common and least-malignant intracranial germ cell tumor, usually found in the midline. Germinoma that arises in the basal ganglia, called ectopic germinoma, is a rare and well-documented entity representing 5% to 10% of all intracranial germinomas. The association of cerebral and/or brain stem atrophy with basal ganglia germinoma on CT and MRI is found in 33% of the cases. To review the literature and describe the CT and MRI findings of basal ganglia germinoma in children, known as ectopic germinoma, with associated ipsilateral cerebral and brain stem hemiatrophy. Three brain CT and six brain MRI studies performed in four children at two institutions were retrospectively reviewed. All patients were male (case 1, 14 years; case 2, 13 years; case 3, 9 years; case 4, 13 years), with pathologically proved germinoma arising in the basal ganglia, and associated ipsilateral cerebral and/or brain stem hemiatrophy on the first imaging study. It is important to note that three of these children presented with cognitive decline, psychosis and slowly progressive hemiparesis as their indication for imaging. Imaging results on initial scans were varied. In all patients, the initial study showed ipsilateral cerebral and/or brain stem hemiatrophy, representing Wallerian degeneration. All patients who underwent CT imaging presented with a hyperdense or calcified lesion in the basal ganglia on unenhanced scans. Only one of these lesions had a mass effect on the surrounding structures. In one of these patients a large, complex, heterogeneous mass appeared 15 months later. Initial MR showed focal or diffusely increased T2 signal in two cases and heterogeneous signal in the other two. (orig.)

  5. Basal ganglia germinoma in children with associated ipsilateral cerebral and brain stem hemiatrophy

    International Nuclear Information System (INIS)

    Ozelame, Rodrigo V.; Shroff, Manohar; Wood, Bradley; Bouffet, Eric; Bartels, Ute; Drake, James M.; Hawkins, Cynthia; Blaser, Susan

    2006-01-01

    Germinoma is the most common and least-malignant intracranial germ cell tumor, usually found in the midline. Germinoma that arises in the basal ganglia, called ectopic germinoma, is a rare and well-documented entity representing 5% to 10% of all intracranial germinomas. The association of cerebral and/or brain stem atrophy with basal ganglia germinoma on CT and MRI is found in 33% of the cases. To review the literature and describe the CT and MRI findings of basal ganglia germinoma in children, known as ectopic germinoma, with associated ipsilateral cerebral and brain stem hemiatrophy. Three brain CT and six brain MRI studies performed in four children at two institutions were retrospectively reviewed. All patients were male (case 1, 14 years; case 2, 13 years; case 3, 9 years; case 4, 13 years), with pathologically proved germinoma arising in the basal ganglia, and associated ipsilateral cerebral and/or brain stem hemiatrophy on the first imaging study. It is important to note that three of these children presented with cognitive decline, psychosis and slowly progressive hemiparesis as their indication for imaging. Imaging results on initial scans were varied. In all patients, the initial study showed ipsilateral cerebral and/or brain stem hemiatrophy, representing Wallerian degeneration. All patients who underwent CT imaging presented with a hyperdense or calcified lesion in the basal ganglia on unenhanced scans. Only one of these lesions had a mass effect on the surrounding structures. In one of these patients a large, complex, heterogeneous mass appeared 15 months later. Initial MR showed focal or diffusely increased T2 signal in two cases and heterogeneous signal in the other two. (orig.)

  6. Basal ganglia impairments in autism spectrum disorder are related to abnormal signal gating to prefrontal cortex.

    Science.gov (United States)

    Prat, Chantel S; Stocco, Andrea; Neuhaus, Emily; Kleinhans, Natalia M

    2016-10-01

    Research on the biological basis of autism spectrum disorder has yielded a list of brain abnormalities that are arguably as diverse as the set of behavioral symptoms that characterize the disorder. Among these are patterns of abnormal cortical connectivity and abnormal basal ganglia development. In attempts to integrate the existing literature, the current paper tests the hypothesis that impairments in the basal ganglia's function to flexibly select and route task-relevant neural signals to the prefrontal cortex underpins patterns of abnormal synchronization between the prefrontal cortex and other cortical processing centers observed in individuals with autism spectrum disorder (ASD). We tested this hypothesis using a Dynamic Causal Modeling analysis of neuroimaging data collected from 16 individuals with ASD (mean age=25.3 years; 6 female) and 17 age- and IQ-matched neurotypical controls (mean age=25.6, 6 female), who performed a Go/No-Go test of executive functioning. Consistent with the hypothesis tested, a random-effects Bayesian model selection procedure determined that a model of network connectivity in which basal ganglia activation modulated connectivity between the prefrontal cortex and other key cortical processing centers best fit the data of both neurotypicals and individuals with ASD. Follow-up analyses suggested that the largest group differences were observed for modulation of connectivity between prefrontal cortex and the sensory input region in the occipital lobe [t(31)=2.03, p=0.025]. Specifically, basal ganglia activation was associated with a small decrease in synchronization between the occipital region and prefrontal cortical regions in controls; however, in individuals with ASD, basal ganglia activation resulted in increased synchronization between the occipital region and the prefrontal cortex. We propose that this increased synchronization may reflect a failure in basal ganglia signal gating mechanisms, resulting in a non-selective copying

  7. Endoscopic Evacuation of Basal Ganglia Hemorrhage via Keyhole Approach Using an Adjustable Cannula in Comparison with Craniotomy

    Science.gov (United States)

    Zhang, Heng-Zhu; Li, Yu-Ping; Yan, Zheng-cun; Wang, Xing-dong; She, Lei; Wang, Xiao-dong; Dong, Lun

    2014-01-01

    Neuroendoscopic (NE) surgery as a minimal invasive treatment for basal ganglia hemorrhage is a promising approach. The present study aims to evaluate the efficacy and safety of NE approach using an adjustable cannula to treat basal ganglia hemorrhage. In this study, we analysed the clinical and radiographic outcomes between NE group (21 cases) and craniotomy group (30 cases). The results indicated that NE surgery might be an effective and safe approach for basal ganglia haemorrhage, and it is also suggested that NE approach may improve good functional recovery. However, NE approach only suits the selected patient, and the usefulness of NE approach needs further randomized controlled trials (RCTs) to evaluate. PMID:24949476

  8. Acute Psychosis Associated with Subcortical Stroke: Comparison between Basal Ganglia and Mid-Brain Lesions

    Directory of Open Access Journals (Sweden)

    Aaron McMurtray

    2014-01-01

    Full Text Available Acute onset of psychosis in an older or elderly individual without history of previous psychiatric disorders should prompt a thorough workup for neurologic causes of psychiatric symptoms. This report compares and contrasts clinical features of new onset of psychotic symptoms between two patients, one with an acute basal ganglia hemorrhagic stroke and another with an acute mid-brain ischemic stroke. Delusions and hallucinations due to basal ganglia lesions are theorized to develop as a result of frontal lobe dysfunction causing impairment of reality checking pathways in the brain, while visual hallucinations due to mid-brain lesions are theorized to develop due to dysregulation of inhibitory control of the ponto-geniculate-occipital system. Psychotic symptoms occurring due to stroke demonstrate varied clinical characteristics that depend on the location of the stroke within the brain. Treatment with antipsychotic medications may provide symptomatic relief.

  9. Asymptomatic moyamoya syndrome, atlantoaxial subluxation and basal ganglia calcification in a child with Down syndrome

    Science.gov (United States)

    Lee, Kun-Soo; Weon, Young Cheol

    2013-01-01

    Down syndrome, the most common chromosomal abnormality, may be associated with various neurologic complications such as moyamoya syndrome, cervical spinal cord compression due to atlantoaxial subluxation, and basal ganglia damage, as well as epileptic seizures and stroke. Many cases of Down syndrome accompanied by isolated neurologic manifestations have been reported in children; however, Down syndrome with multiple neurologic conditions is rare. Here, we have reported a case of Down syndrome in a 10-year-old girl who presented with asymptomatic moyamoya syndrome, atlantoaxial subluxation with spinal cord compression, and basal ganglia calcification. To the best of our knowledge, this is the first report of Down syndrome, in a child, which was accompanied by these 3 neurologic complications simultaneously. As seen in this case, patients with Down syndrome may have neurologic conditions without any obvious neurologic symptoms; hence, patients with Down syndrome should be carefully examined for the presence of neurologic conditions. PMID:24416050

  10. Minimizing Human Intervention in the Development of Basal Ganglia-Inspired Robot Control

    Directory of Open Access Journals (Sweden)

    F. Montes-Gonzalez

    2007-01-01

    Full Text Available A biologically inspired mechanism for robot action selection, based on the vertebrate basal ganglia, has been previously presented (Prescott et al. 2006, Montes Gonzalez et al. 2000. In this model the task confronting the robot is decomposed into distinct behavioural modules that integrate information from multiple sensors and internal state to form ‘salience’ signals. These signals are provided as inputs to a computational model of the basal ganglia whose intrinsic processes cause the selection by disinhibition of a winning behaviour. This winner is then allowed access to the motor plant whilst losing behaviours are suppressed. In previous research we have focused on the development of this biomimetic selection architecture, and have therefore used behavioural modules that were hand-coded as algorithmic procedures. In the current article, we demonstrate the use of genetic algorithms and gradient–descent learning to automatically generate/tune some of the modules that generate the model behaviour.

  11. Functional properties of the basal ganglia's re-entrant loop architecture: selection and reinforcement.

    Science.gov (United States)

    Redgrave, P; Vautrelle, N; Reynolds, J N J

    2011-12-15

    Multifunctional agents with limited motor resources must decide what actions will best ensure their survival. Moreover, given that in an unpredictable world things don't always work out, considerable advantage is to be gained by learning from experience - instrumental behaviour that maximises reward and minimises punishment. In this review we will argue that the re-entrant looped architecture of the basal ganglia represents biological solutions to these fundamental behavioural problems of selection and reinforcement. A potential solution to the selection problem is provided for by selective disinhibition within the parallel loop architecture that connects the basal ganglia with external neural structures. The relay points within these loops permit the signals of a particular channel to be modified by external influences. In part, these influences have the capacity to modify overall selections so that the probability of re-selecting reinforced behaviours in the future is altered. This is the basic process of instrumental learning, which we suggest decomposes into two sub-problems for the agent: (i) learning which external events it causes to happen and learning precisely what it is doing that is causal; and (ii) having determined agency and discovered novel action-outcome routines, how best to exploit this knowledge to maximise future reward acquisitions. Considerations of connectional architecture and signal timing suggest that the short-latency, sensory-evoked dopamine response, which can modulate the re-entrant loop structure within the basal ganglia, is ideally suited to reinforce the determination of agency and the discovery of novel actions. Alternatively, recent studies showing that presence or absence of reward can selectively modulate the magnitude of signals in structures providing input signals to the basal ganglia, offer an alternative mechanism for biasing selection within the re-entrant loop architecture. We suggest that this mechanism may be better

  12. Neuronal degeneration induced by status epilepticus in basal ganglia of immature rats

    Czech Academy of Sciences Publication Activity Database

    Druga, Rastislav; Kubová, Hana; Mareš, Pavel

    2005-01-01

    Roč. 46, č. S8 (2005), s. 98-99 ISSN 0013-9580. [Joint Annual Meeting of the American Epilepsy Society and American Clinical Neurophysiology Society. 02.12.2005-06.12.2005, Washington, DC] R&D Projects: GA ČR(CZ) GA304/04/0464 Institutional research plan: CEZ:AV0Z50110509 Keywords : status epilepticus * neurodegeneration * basal ganglia Subject RIV: ED - Physiology

  13. United in Diversity : A Physiological and Molecular Characterization of Subpopulations in the Basal Ganglia Circuitry

    OpenAIRE

    Viereckel, Thomas

    2017-01-01

    The Basal Ganglia consist of a number of different nuclei that form a diverse circuitry of GABAergic, dopaminergic and glutamatergic neurons. This complex network is further organized in subcircuits that govern limbic and motor functions in humans and other vertebrates. Due to the interconnection of the individual structures, dysfunction in one area or cell population can affect the entire network, leading to synaptic and molecular alterations in the circuitry as a whole. The studies in this ...

  14. Dopamine transporter density of the basal ganglia assessed with I-123 IPT SPECT in methamphetamine abusers

    International Nuclear Information System (INIS)

    Lee, Joo Ryung; Ahn, Byeong Cheol; Kewm, Do Hun

    2005-01-01

    Functional imaging of dopamine transporter (DAT) defines integrity of the dopaminergic system, and DAT is the target site of drugs of abuse such as cocaine and methamphetamine. Functional imaging the DAT may be a sensitive and selective indicator of neurotoxic change by the drug. The aim of the present study is to evaluate the clinical implications of qualitative/quantitative analyses of dopamine transporter imaging in methamphetamine abusers. Six detoxified methamphetamine abusers (abuser group) and 4 volunteers (control group) were enrolled in this study. Brain MRI was performed in all of abuser group. Abuser group underwent psychiatric and depression assessment using brief psychiatric rating scale (BPRS) and Hamilton depression rating scale (HAMD), respectively. All of the subjects underwent I-123 IPT SPECT (IPT SPECT). IPT SPECT image was analysed with visual qualitative method and quantitative method using basal ganglia dopamine transporter (DAT) specific/non-specific binding ratio (SBR). Comparison of DAT SBR between abuser and control groups was performed. We also performed correlation tests between psychiatric and depression assessment results and DAT SBR in abuser group. All of abuser group showed normal MRI finding, but had residual psychiatric and depressive symptoms, and psychiatric and depressive symptom scores were exactly correlated (r=1.0, ρ =0.005) each other. Five of them showed abnormal finding on qualitative visual I-123 IPT SPECT. Abuser group had lower basal ganglia DAT SBR than that of control (2.38 ± 0.20 vs 3.04 ± 0.27, ρ =0.000). Psychiatric and depressive symptoms were negatively well correlated with basal ganglia DAT SBR (r=-0.908, ρ =0.012, r=-0.924, ρ =0.009) This results suggest that dopamine transporter imaging using I-123 IPT SPECT may be used to evaluate dopaminergic system of the basal ganglia and the clinical status in methamphetamine abusers

  15. Dopamine transporter density of the basal ganglia assessed with I-123 IPT SPECT in methamphetamine abusers

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Joo Ryung; Ahn, Byeong Cheol [Kyungpook National University Medical School, Daegu (Korea, Republic of); Kewm, Do Hun [National Bugok Mental Hospital, Changryung (Korea, Republic of)] (and others)

    2005-10-15

    Functional imaging of dopamine transporter (DAT) defines integrity of the dopaminergic system, and DAT is the target site of drugs of abuse such as cocaine and methamphetamine. Functional imaging the DAT may be a sensitive and selective indicator of neurotoxic change by the drug. The aim of the present study is to evaluate the clinical implications of qualitative/quantitative analyses of dopamine transporter imaging in methamphetamine abusers. Six detoxified methamphetamine abusers (abuser group) and 4 volunteers (control group) were enrolled in this study. Brain MRI was performed in all of abuser group. Abuser group underwent psychiatric and depression assessment using brief psychiatric rating scale (BPRS) and Hamilton depression rating scale (HAMD), respectively. All of the subjects underwent I-123 IPT SPECT (IPT SPECT). IPT SPECT image was analysed with visual qualitative method and quantitative method using basal ganglia dopamine transporter (DAT) specific/non-specific binding ratio (SBR). Comparison of DAT SBR between abuser and control groups was performed. We also performed correlation tests between psychiatric and depression assessment results and DAT SBR in abuser group. All of abuser group showed normal MRI finding, but had residual psychiatric and depressive symptoms, and psychiatric and depressive symptom scores were exactly correlated (r=1.0, {rho} =0.005) each other. Five of them showed abnormal finding on qualitative visual I-123 IPT SPECT. Abuser group had lower basal ganglia DAT SBR than that of control (2.38 {+-} 0.20 vs 3.04 {+-} 0.27, {rho} =0.000). Psychiatric and depressive symptoms were negatively well correlated with basal ganglia DAT SBR (r=-0.908, {rho} =0.012, r=-0.924, {rho} =0.009) This results suggest that dopamine transporter imaging using I-123 IPT SPECT may be used to evaluate dopaminergic system of the basal ganglia and the clinical status in methamphetamine abusers.

  16. Hereditary haemochromatosis: a case of iron accumulation in the basal ganglia associated with a parkinsonian syndrome

    DEFF Research Database (Denmark)

    Nielsen, J.E.; Jensen, L. Neerup; Krabbe, K.

    1995-01-01

    . A patient is reported with hereditary haemochromatosis and a syndrome of dementia, dysarthria, a slowly progressive gait disturbance, imbalance, muscle weakness, rigidity, bradykinesia, tremor, ataxia, and dyssynergia. The findings on MRI of a large signal decrease in the basal ganglia, consistent...... with excessive iron accumulation, indicate a causal relation to the symptoms. Although the neurological symptoms did not improve in our patient, hereditary haemochromatosis should be considered in the differential diagnosis of parkinsonian syndromes, because complications of iron induced organ injury may...

  17. Long-term depression at distinct glutamatergic synapses in the basal ganglia.

    Science.gov (United States)

    Dupuis, Julien P; Bioulac, Bernard H; Baufreton, Jérôme

    2014-01-01

    Long-term adaptations of synaptic transmission are believed to be the cellular basis of information storage in the brain. In particular, long-term depression of excitatory neurotransmission has been under intense investigation since convergent lines of evidence support a crucial role for this process in learning and memory. Within the basal ganglia, a network of subcortical nuclei forming a key part of the extrapyramidal motor system, plasticity at excitatory synapses is essential to the regulation of motor, cognitive, and reward functions. The striatum, the main gateway of the basal ganglia, receives convergent excitatory inputs from cortical areas and transmits information to the network output structures and is a major site of activity-dependent plasticity. Indeed, long-term depression at cortico-striatal synapses modulates the transfer of information to basal ganglia output structures and affects voluntary movement execution. Cortico-striatal plasticity is thus considered as a cellular substrate for adaptive motor control. Downstream in this network, the subthalamic nucleus and substantia nigra nuclei also receive glutamatergic innervation from the cortex and the subthalamic nucleus, respectively. Although these connections have been less investigated, recent studies have started to unravel the molecular mechanisms that contribute to adjustments in the strength of cortico-subthalamic and subthalamo-nigral transmissions, revealing that adaptations at these synapses governing the output of the network could also contribute to motor planning and execution. Here, we review our current understanding of long-term depression mechanisms at basal ganglia glutamatergic synapses and emphasize the common and unique plastic features observed at successive levels of the network in healthy and pathological conditions.

  18. MRI and MR spectroscopy study on basal ganglia alterations in patients with liver cirrhosis

    International Nuclear Information System (INIS)

    Wu Haibo; Ma Lin; Cai Youquan; Li Tao; Li Dejun; Liang Li

    2007-01-01

    Objective: To study the signal changes and metabolic alterations in the basal ganglia (BG) by using magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (MRS) in patients with hepatic encephalopathy with and without parkinsonism. Methods: MRI and MRS in the basal ganglia were performed in 27 patients (22 males, 5 females, age ranging from 29 to 62 years) with liver cirrhosis and hepatic encephalopathy. 14 of the 27 patients were classified as having parkinsonian signs evaluated by Unified Parkinson's Disease Rating Scale (UPDRS) test. 18 age-matched healthy volunteers (13 males, 5 females, age ranging from 24 to 51 years) underwent MRI and MRS as a control group. Results: NAA/Cr levels (average numbers are 1.40±0.03, 1.35±0.03 respectively) showed no statistical difference between cirrhotic patients with hepatic encephalopathy and the control group (t=1.16, t=0.87, P>0.05). Values of signal hyperintensities (average numbers are 1.03±0.002, 1.04± 0.003 respectively) in globus pallidus and ratios of mI/Cr(average numbers are 0.63±0.01, 0.61± 0.02 respectively) and Cho/Cr (average numbers are 0.82±0.03, 0.80±0.02 respectively) showed no statistically significant differences between the control group and the 13 patients without parkinsonism (t=0.63, t=-0.52, t=-0.54, P>0.05), whereas values of signal hyperintensities (average numbers are 1.18±0.001, 1.04±0.003 respectively) in globus pallidus and ratios of mI/Cr (average numbers are 0.39±0.02, 0.63±0.01 respectively) and Cho/Cr(average numbers are 0.68±0.01, 0.82±0.03 respectively) shows statistically significant difference in patients without and with parkinsonism (t=-5.16, t=7.61, t=4.12, P<0.05). In patients with cirrhosis, the values of signal hyperintensities in globus pallidus were inversely correlated with the ratio for mI/Cr(r=-0.764, P<0.05) and Cho/Cr (r=-0.553, P<0.05), respectively. Conclusion: MRI and MRS may be useful tools in the evaluation of extrapyramidal

  19. Integration of reinforcement learning and optimal decision-making theories of the basal ganglia.

    Science.gov (United States)

    Bogacz, Rafal; Larsen, Tobias

    2011-04-01

    This article seeks to integrate two sets of theories describing action selection in the basal ganglia: reinforcement learning theories describing learning which actions to select to maximize reward and decision-making theories proposing that the basal ganglia selects actions on the basis of sensory evidence accumulated in the cortex. In particular, we present a model that integrates the actor-critic model of reinforcement learning and a model assuming that the cortico-basal-ganglia circuit implements a statistically optimal decision-making procedure. The values of cortico-striatal weights required for optimal decision making in our model differ from those provided by standard reinforcement learning models. Nevertheless, we show that an actor-critic model converges to the weights required for optimal decision making when biologically realistic limits on synaptic weights are introduced. We also describe the model's predictions concerning reaction times and neural responses during learning, and we discuss directions required for further integration of reinforcement learning and optimal decision-making theories.

  20. Early imaging findings in germ cell tumors arising from the basal ganglia

    Energy Technology Data Exchange (ETDEWEB)

    Lee, So Mi [Seoul National University College of Medicine, Department of Radiology, 101 Daehak-ro, Jongno-gu, Seoul (Korea, Republic of); Kyungpook National University Medical Center, Department of Radiology, Daegu (Korea, Republic of); Kim, In-One; Choi, Young Hun; Cheon, Jung-Eun; Kim, Woo Sun [Seoul National University College of Medicine, Department of Radiology and Institute of Radiation Medicine, 101 Daehak-ro, Jongno-gu, Seoul (Korea, Republic of); Cho, Hyun-Hae [Seoul National University College of Medicine, Department of Radiology, 101 Daehak-ro, Jongno-gu, Seoul (Korea, Republic of); Ewha Woman' s University Mokdong Hospital, Department of Radiology, Seoul (Korea, Republic of); You, Sun Kyoung [Seoul National University College of Medicine, Department of Radiology, 101 Daehak-ro, Jongno-gu, Seoul (Korea, Republic of); Chungnam National University Hospital, Department of Radiology, Daejeon (Korea, Republic of)

    2016-05-15

    It is difficult to diagnosis early stage germ cell tumors originating in the basal ganglia, but early recognition is important for better outcome. To evaluate serial MR images of basal ganglia germ cell tumors, with emphasis on the features of early stage tumors. We retrospectively reviewed serial MR images of 15 tumors in 14 children and young adults. We categorized MR images of the tumors as follows: type I, ill-defined patchy lesions (<3 cm) without cyst; type II, small mass lesions (<3 cm) with cyst; and type III, large lesions (≥3 cm) with cyst. We also assessed temporal changes of the MR images. On the initial images, 8 of 11 (73%) type I tumors progressed to types II or III, and 3 of 4 (75%) type II tumors progressed to type III. The remaining 4 tumors did not change in type. All type II tumors (5/5, 100%) that changed from type I had a few tiny cysts. Intratumoral hemorrhage was observed even in the type I tumor. Ipsilateral hemiatrophy was observed in most of the tumors (13/15, 87%) on initial MR images. As tumors grew, cystic changes, intratumoral hemorrhage, and ipsilateral hemiatrophy became more apparent. Early stage basal ganglia germ cell tumors appear as ill-defined small patchy hyperintense lesions without cysts on T2-weighted images, are frequently associated with ipsilateral hemiatrophy, and sometimes show microhemorrhage. Tumors develop tiny cysts at a relatively early stage. (orig.)

  1. Meige`s syndrome associated with basal ganglia and thalamic functional disorders

    Energy Technology Data Exchange (ETDEWEB)

    Sakai, Tsutomu; Shikishima, Keigo; Kawai, Kazushige; Kitahara, Kenji [Jikei Univ., Tokyo (Japan). School of Medicine

    1998-11-01

    Magnetic resonance imaging (MRI) or single positron emission computed tomography (SPECT) or both were performed and the responses of surface electromyography (EMG) were examined in seven cases of Meige`s syndrome. MRI or SPECT or both demonstrated lesions of the basal ganglia, the thalamus, or both in five of the cases. Surface EMG revealed abnormal burst discharges in the orbicularis oculi and a failure of reciprocal muscular activity between the frontalis and orbicularis oculi in all the cases. These findings suggest that voluntary motor control and reciprocal activity in the basal ganglia-thalamocortical circuits are impaired in Meige`s syndrome. In addition, good responses were seen to clonazepam, tiapride and trihexyphenidyl in these cases. Therefore, we conclude that dopaminergic, cholinergic, and {gamma}-aminobutyric acid (GABA) ergic imbalances in the disorders of the basal ganglia and thalamus in Meige`s syndrome cause control in the excitatory and inhibitory pathways to be lost, resulting in the failure of integration in reciprocal muscular activity and voluntary motor control. This failure subsequently causes the symptoms of Meige`s syndrome. (author)

  2. Early imaging findings in germ cell tumors arising from the basal ganglia

    International Nuclear Information System (INIS)

    Lee, So Mi; Kim, In-One; Choi, Young Hun; Cheon, Jung-Eun; Kim, Woo Sun; Cho, Hyun-Hae; You, Sun Kyoung

    2016-01-01

    It is difficult to diagnosis early stage germ cell tumors originating in the basal ganglia, but early recognition is important for better outcome. To evaluate serial MR images of basal ganglia germ cell tumors, with emphasis on the features of early stage tumors. We retrospectively reviewed serial MR images of 15 tumors in 14 children and young adults. We categorized MR images of the tumors as follows: type I, ill-defined patchy lesions (<3 cm) without cyst; type II, small mass lesions (<3 cm) with cyst; and type III, large lesions (≥3 cm) with cyst. We also assessed temporal changes of the MR images. On the initial images, 8 of 11 (73%) type I tumors progressed to types II or III, and 3 of 4 (75%) type II tumors progressed to type III. The remaining 4 tumors did not change in type. All type II tumors (5/5, 100%) that changed from type I had a few tiny cysts. Intratumoral hemorrhage was observed even in the type I tumor. Ipsilateral hemiatrophy was observed in most of the tumors (13/15, 87%) on initial MR images. As tumors grew, cystic changes, intratumoral hemorrhage, and ipsilateral hemiatrophy became more apparent. Early stage basal ganglia germ cell tumors appear as ill-defined small patchy hyperintense lesions without cysts on T2-weighted images, are frequently associated with ipsilateral hemiatrophy, and sometimes show microhemorrhage. Tumors develop tiny cysts at a relatively early stage. (orig.)

  3. Evidence for altered basal ganglia-brainstem connections in cervical dystonia.

    Directory of Open Access Journals (Sweden)

    Anne J Blood

    Full Text Available There has been increasing interest in the interaction of the basal ganglia with the cerebellum and the brainstem in motor control and movement disorders. In addition, it has been suggested that these subcortical connections with the basal ganglia may help to coordinate a network of regions involved in mediating posture and stabilization. While studies in animal models support a role for this circuitry in the pathophysiology of the movement disorder dystonia, thus far, there is only indirect evidence for this in humans with dystonia.In the current study we investigated probabilistic diffusion tractography in DYT1-negative patients with cervical dystonia and matched healthy control subjects, with the goal of showing that patients exhibit altered microstructure in the connectivity between the pallidum and brainstem. The brainstem regions investigated included nuclei that are known to exhibit strong connections with the cerebellum. We observed large clusters of tractography differences in patients relative to healthy controls, between the pallidum and the brainstem. Tractography was decreased in the left hemisphere and increased in the right hemisphere in patients, suggesting a potential basis for the left/right white matter asymmetry we previously observed in focal dystonia patients.These findings support the hypothesis that connections between the basal ganglia and brainstem play a role in the pathophysiology of dystonia.

  4. iPhone-Assisted Augmented Reality Localization of Basal Ganglia Hypertensive Hematoma.

    Science.gov (United States)

    Hou, YuanZheng; Ma, LiChao; Zhu, RuYuan; Chen, XiaoLei

    2016-10-01

    A low-cost, time-efficient technique that could localize hypertensive hematomas in the basal ganglia would be beneficial for minimally invasive hematoma evacuation surgery. We used an iPhone to achieve this goal and evaluated its accuracy and feasibility. We located basal ganglia hematomas in 26 patients and depicted the boundaries of the hematomas on the skin. To verify the accuracy of the drawn boundaries, computed tomography (CT) markers surrounding the depicted boundaries were attached to 10 patients. The deviation between the CT markers and the actual hematoma boundaries was then measured. In the other 16 patients, minimally invasive endoscopic hematoma evacuation surgery was performed according to the depicted hematoma boundary. The deflection angle of the actual trajectory and deviation in the hematoma center were measured according to the preoperative and postoperative CT data. There were 40 CT markers placed on 10 patients. The mean deviation of these markers was 3.1 mm ± 2.4. In the 16 patients who received surgery, the deflection angle of the actual trajectory was 4.3° ± 2.1. The deviation in the hematoma center was 5.2 mm ± 2.6. This new method can locate basal ganglia hematomas with a sufficient level of accuracy and is helpful for minimally invasive endoscopic hematoma evacuation surgery. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Reinforcement-driven dimensionality reduction--a model for information processing in the basal ganglia.

    Science.gov (United States)

    Bar-Gad, I; Havazelet-Heimer, G; Goldberg, J A; Ruppin, E; Bergman, H

    2000-01-01

    Although anatomical studies of the basal ganglia show the existence of extensive convergence and lateral inhibitory connections, physiological studies failed to show correlated neural activity or lateral interaction in these nuclei. These seemingly contradictory results could be explained with a model in which the basal ganglia reduce the dimensionality of cortical information using optimal extraction methods. Simulations of this model predict a transient change in the efficacy of the feed-forward and lateral synapses following changes in reinforcement signal, causing an increase in correlated firing rates. This process ultimately restores the steady-state situation with diminished efficacy of lateral inhibition and no correlation of firing. Our experimental results confirm the model's predictions: rate correlations show a drastic decrease between the input stage (cortex) and output stage (pallidum). Moreover, preliminary analysis revealed that pallidal correlations show a transient increase following discrepancies between the animal's predictions and reality. We therefore propose that by using a reinforcement-driven dimensionality reduction process the basal ganglia achieve efficient extraction of cortical salient information that may then be used by the frontal cortex for execution and planning of forthcoming actions.

  6. Basal ganglia-premotor dysfunction during movement imagination in writer's cramp.

    Science.gov (United States)

    Castrop, Florian; Dresel, Christian; Hennenlotter, Andreas; Zimmer, Claus; Haslinger, Bernhard

    2012-09-15

    The pathophysiology of idiopathic focal hand dystonia (writer's cramp) is characterized by deficient inhibitory basal ganglia function and altered cortical sensorimotor processing. To explore if this is already a primary finding in dystonia for internal movement simulation independent of dystonic motor output or abnormal sensory input, we investigated the neural correlates of movement imagination and observation in patients with writer's cramp. Event-related fMRI was applied during kinesthetic motor imagery of drawing simple geometric figures (imagination task) and passively observing videos of hands drawing identical figures (observation task). Compared with healthy controls, patients with writer's cramp showed deficient activation of the left primary sensorimotor cortex, mesial and left dorsal premotor cortex, bilateral putamen, and bilateral thalamus during motor imagery. No significant signal differences between both groups were found during the observation task. We conclude that internal movement simulation and planning as tested during imagination of hand movements appear to be dysfunctional in patients with writer's cramp, whereas visual signal processing and observation-induced activation are unaffected. Deficient basal ganglia-premotor activation could be a correlate of impaired basal ganglia inhibition and focusing during the selection of motor programs in dystonia. This finding seems to be an intrinsic deficit, as it is found during motor imagery in the absence of dystonic symptoms. Copyright © 2012 Movement Disorder Society.

  7. A morphometric CT study of Down's syndrome showing small posterior fossa and calcification of basal ganglia

    International Nuclear Information System (INIS)

    Ieshima, A.; Yoshino, K.; Takashima, S.; Takeshita, K.; Kisa, T.

    1984-01-01

    We report characteristic and morphometric changes of cranial computed tomography (CT) with increasing age in 56 patients with Down's syndrome aged from 0 month to 37 years. Patients were compared with 142 normal controls aged 0 to 59 years. Width of ventricles, Sylvian fissures, posterior fossa, pons and cisterna magna were measured on CT. The incidences of the cavum septi pellucidi, cavum vergae and cavum veli interpositi and high density in the basal ganglia were examined. There was high incidence (10.7%) of bilateral calcification of basal ganglia in Down's syndrome, although that of pineal body and choroid plexus calcification was similar in Down's syndrome and controls. Basal ganglia calcification is more frequently seen in young Down's syndrome and may be related to the premature aging characteristic of Down's syndrome. The CT in Down's syndrome showed relatively small posterior fossa, small cerebellum, small brain stem and relatively large Sylvian fissures in those under one year of age. There was a high frequency of midline cava and large cisterna magna. There were no significant atrophic changes on CT except after the fifth decade comparing with controls. (orig.)

  8. Viral vector-based tools advance knowledge of basal ganglia anatomy and physiology.

    Science.gov (United States)

    Sizemore, Rachel J; Seeger-Armbruster, Sonja; Hughes, Stephanie M; Parr-Brownlie, Louise C

    2016-04-01

    Viral vectors were originally developed to deliver genes into host cells for therapeutic potential. However, viral vector use in neuroscience research has increased because they enhance interpretation of the anatomy and physiology of brain circuits compared with conventional tract tracing or electrical stimulation techniques. Viral vectors enable neuronal or glial subpopulations to be labeled or stimulated, which can be spatially restricted to a single target nucleus or pathway. Here we review the use of viral vectors to examine the structure and function of motor and limbic basal ganglia (BG) networks in normal and pathological states. We outline the use of viral vectors, particularly lentivirus and adeno-associated virus, in circuit tracing, optogenetic stimulation, and designer drug stimulation experiments. Key studies that have used viral vectors to trace and image pathways and connectivity at gross or ultrastructural levels are reviewed. We explain how optogenetic stimulation and designer drugs used to modulate a distinct pathway and neuronal subpopulation have enhanced our mechanistic understanding of BG function in health and pathophysiology in disease. Finally, we outline how viral vector technology may be applied to neurological and psychiatric conditions to offer new treatments with enhanced outcomes for patients. Copyright © 2016 the American Physiological Society.

  9. Serial dynamic CT scan in patients with acute basal ganglia infarctions

    International Nuclear Information System (INIS)

    Node, Yoji; Nakazawa, Shozo; Tsuji, Yukihide.

    1987-01-01

    Dynamic computed tomography (CT) was performed on 15 patients (37 to 93 years of age) with acute basal ganglia infarctions, and the perfusion patterns of the infarcted regions on CT were evaluated. The initial dynamic CT was performed within 12 hours after onset, while the serial studies of the dynamic CT were performed on the 3rd and 7th days. The left-over-right ratio in the peak value in the basal ganglia in 15 normal subjects was 1.01 ± 0.03 (mean ± SD), so there were no differences in the peak values of the bilateral basal ganglia. We also examined the left-over-right ratio in the peak value and in the rapid-washout ratio in the basal ganglia in the 15 normal subjects. There was no difference in the peak values of the bilateral basal ganglia. The mean rapid-washout ratio was 0.62 ± 0.11 (mean ± SD). The prognoses of these patients three months after onset were as follows: 8 showed a good recovery, 5 had a moderate disability, and 2 had a severe disability. The perfusions on admission were as follows. 10 were hypoperfusions, 3 were hypo + late perfusions, one was a normoperfusion, and one was a late perfusion. There was a tendency for the rapid-washout ratio decrease more in the hypo + late perfusion group than in the other groups. Twelve patients showed an iso-density, while 3 showed a low density, on admission. The ''low-density'' group showed a decrease in the A/N ratio of the peak value. We performed serial dynamic CT in 11 cases. The group with severe disabilities (2 cases) showed a hypo + late perfusion in the initial CT, one case kept a hypo + late perfusion, and another case changed to a hypoperfusion; also, there was a tendency for there to be a poor improvement in the A/N ratio of the peak value in these two ''severe-disability'' patients. (J.P.N.)

  10. Novel signal-dependent filter bank method for identification of multiple basal ganglia nuclei in Parkinsonian patients

    Science.gov (United States)

    Pinzon-Morales, R. D.; Orozco-Gutierrez, A. A.; Castellanos-Dominguez, G.

    2011-06-01

    Microelectrode recordings are a valuable tool for assisting localization targets during deep brain stimulation procedures in Parkinson's disease neurosurgery. Attempts to automate and standardize this process have been limited by variability in patient neurophysiology and strong dynamics of microelectrode recordings. In this paper, a methodology for the identification of basal ganglia nuclei is presented that is based on a signal-dependent filter bank method using microelectrode recordings. The method is a customized realization of the discrete wavelet transform via the lifting scheme that is optimally tuned by genetic algorithms. Using this method, unique mother wavelet functions that exhibit an adaptable spectrum to the microelectrode recording dynamic are generated. Additionally, by extracting morphological features from the space-transformed microelectrode recording, it is possible to integrate them into three-dimensional (3D) feature spaces with maximum class separability. Finally, high discriminant feature spaces are fed into basic classifiers to recognize up to four basal nuclei. Comparison with several existing wavelets highlights the characteristics of new mother wavelets. Additionally, classification results show that identification of addressed nuclei in the basal ganglia can be performed with 95% confidence.

  11. Altered basal ganglia-cortical functional connections in frontal lobe epilepsy: A resting-state fMRI study.

    Science.gov (United States)

    Dong, Li; Wang, Pu; Peng, Rui; Jiang, Sisi; Klugah-Brown, Benjamin; Luo, Cheng; Yao, Dezhong

    2016-12-01

    The purpose of this study was to investigate alterations of basal ganglia-cortical functional connections in patients with frontal lobe epilepsy (FLE). Resting-state functional magnetic resonance imaging (fMRI) data were gathered from 19 FLE patients and 19 age- and gender-matched healthy controls. Functional connectivity (FC) analysis was used to assess the functional connections between basal ganglia and cerebral cortex. Regions of interest, including the left/right caudate, putamen, pallidum and thalamus, were selected as the seeds. Two sample t-test was used to determine the difference between patients and controls, while controlling the age, gender and head motions. Compared with controls, FLE patients demonstrated increased FCs between basal ganglia and regions including the right fusiform gyrus, the bilateral cingulate gyrus, the precuneus and anterior cingulate gyrus. Reduced FCs were mainly located in a range of brain regions including the bilateral middle occipital gyrus, the ventral frontal lobe, the right putamen, the left fusiform gyrus and right rolandic operculum. In addition, the relationships between basal ganglia-cingulate connections and durations of epilepsy were also found. The alterations of functional integrity within the basal ganglia, as well as its connections to limbic and ventral frontal areas, indicate the important roles of the basal ganglia-cortical functional connections in FLE, and provide new insights in the pathophysiological mechanism of FLE. Copyright © 2016 Elsevier B.V. All rights reserved.

  12. Basal ganglia disorders associated with imbalances in the striatal striosome and matrix compartments

    Directory of Open Access Journals (Sweden)

    Jill R. Crittenden

    2011-09-01

    Full Text Available The striatum is composed principally of GABAergic, medium spiny projection neurons (MSNs that can be categorized based on their gene expression, electrophysiological profiles and input-output circuits. Major subdivisions of MSN populations include 1 those in ventromedial and dorsolateral striatal regions, 2 those giving rise to the direct and indirect pathways, and 3 those that lie in the striosome and matrix compartments. The first two classificatory schemes have enabled advances in understanding of how basal ganglia circuits contribute to disease. However, despite the large number of molecules that are differentially expressed in the striosomes or the extra-striosomal matrix, and the evidence that these compartments have different input-output connections, our understanding of how this compartmentalization contributes to striatal function is still not clear. A broad view is that the matrix contains the direct and indirect pathway MSNs that form parts of sensorimotor and associative circuits, whereas striosomes contain MSNs that receive input from parts of limbic cortex and project directly or indirectly to the dopamine-containing neurons of the substantia nigra, pars compacta. Striosomes are widely distributed within the striatum and are thought to exert global, as well as local, influences on striatal processing by exchanging information with the surrounding matrix, including through interneurons that send processes into both compartments. It has been suggested that striosomes exert and maintain limbic control over behaviors driven by surrounding sensorimotor and associative parts of the striatal matrix. Consistent with this possibility, imbalances between striosome and matrix functions have been reported in relation to neurological disorders, including Huntington’s disease, L-DOPA-induced dyskinesias, dystonia and drug addiction. Here, we consider how signaling imbalances between the striosomes and matrix might relate to symptomatology in

  13. Minimal invasive puncture and drainage versus endoscopic surgery for spontaneous intracerebral hemorrhage in basal ganglia.

    Science.gov (United States)

    Li, Zhihong; Li, Yuqian; Xu, Feifei; Zhang, Xi; Tian, Qiang; Li, Lihong

    2017-01-01

    Two prevalent therapies for the treatment of spontaneous intracerebral hemorrhage (ICH) in basal ganglia are, minimally invasive puncture and drainage (MIPD), and endoscopic surgery (ES). Because both surgical techniques are of a minimally invasive nature, they have attracted greater attention in recent years. However, evidence comparing the curative effect of MIPD and ES has been uncertain. The indication for MIPD or ES has been uncertain till now. In the present study, 112 patients with spontaneous ICH in basal ganglia who received MIPD or ES were reviewed retrospectively. Baseline parameters prior to the operation, evacuation rate (ER), perihematoma edema, postoperative complications, and rebleeding incidences were collected. Moreover, 1-year postictus, the long-term functional outcomes of patients with regard to hematoma volume (HV) or Glasgow Coma Scale (GCS) score were judged, respectively, by the case fatality, Glasgow Outcome Scale (GOS), Barthel Index (BI), and modified Rankin Scale (mRS). The ES group had a higher ER than the MIPD group on postoperative day 1. The MIPD group had fewer adverse outcomes, which included less perihematoma edema, anesthetic time, and blood loss, than the ES group. The functional outcomes represented by GOS, BI, and mRS were better in the MIPD group than in the ES group for patients with HV 30-60 mL or GCS score 9-14. These results indicate that ES is more effective in evacuating hematoma in basal ganglia, while MIPD is less invasive than ES. Patients with HV 30-60 mL or GCS score 9-14 may benefit more from the MIPD procedure than from ES.

  14. White matter integrity between left basal ganglia and left prefrontal cortex is compromised in gambling disorder.

    Science.gov (United States)

    van Timmeren, Tim; Jansen, Jochem M; Caan, Matthan W A; Goudriaan, Anna E; van Holst, Ruth J

    2017-11-01

    Pathological gambling (PG) is a behavioral addiction characterized by an inability to stop gambling despite the negative consequences, which may be mediated by cognitive flexibility deficits. Indeed, impaired cognitive flexibility has previously been linked to PG and also to reduced integrity of white matter connections between the basal ganglia and the prefrontal cortex. It remains unclear, however, how white matter integrity problems relate to cognitive inflexibility seen in PG. We used a cognitive switch paradigm during functional magnetic resonance imaging in pathological gamblers (PGs; n = 26) and healthy controls (HCs; n = 26). Cognitive flexibility performance was measured behaviorally by accuracy and reaction time on the switch task, while brain activity was measured in terms of blood oxygen level-dependent responses. We also used diffusion tensor imaging on a subset of data (PGs = 21; HCs = 21) in combination with tract-based spatial statistics and probabilistic fiber tracking to assess white matter integrity between the basal ganglia and the dorsolateral prefrontal cortex. Although there were no significant group differences in either task performance, related neural activity or tract-based spatial statistics, PGs did show decreased white matter integrity between the left basal ganglia and prefrontal cortex. Our results complement and expand similar findings from a previous study in alcohol-dependent patients. Although we found no association between white matter integrity and task performance here, decreased white matter connections may contribute to a diminished ability to recruit prefrontal networks needed for regulating behavior in PG. Hence, our findings could resonate an underlying risk factor for PG, and we speculate that these findings may extend to addiction in general. © 2016 Society for the Study of Addiction.

  15. Effect of basal ganglia calcification on its glucose metabolism and dopaminergic function in idiopathic hypoparathyroidism.

    Science.gov (United States)

    Modi, Sagar; Arora, Geetanjali; Bal, Chandra Shekhar; Sreenivas, Vishnubhatla; Kailash, Suparna; Sagar, Rajesh; Goswami, Ravinder

    2015-10-01

    The functional significance of basal ganglia calcification (BGC) in idiopathic hypoparathyroidism (IH) is not clear. To assess the effect of BGC on glucose metabolism and dopaminergic function in IH. (18) F-FDG and (99m) Tc-TRODAT-1 nuclear imaging were performed in 35 IH patients with (n = 26) and without (n = 9) BGC. Controls were subjects without hypoparathyroidism or BGC (nine for (18) F-FDG and 12 for (99m) Tc-TRODAT-1). Relationship of the glucose metabolism and dopaminergic function was assessed with the neuropsychological and biochemical abnormalities. (18) F-FDG uptake in IH patients with calcification at caudate and striatum was less than that of IH patients without calcification (1·06 ± 0·13 vs 1·24 ± 0·09, P = basal ganglia was comparable between IH with and without BGC and between IH without BGC and controls. Serum calcium-phosphorus ratio maintained by the patients correlated with (18) F-FDG uptake at striatum (r = 0·57, P = 0·001). For every 0·1 unit reduction in calcium-phosphorus ratio, (18) F-FDG uptake decreased by 2·5 ± 0·68% (P = 0·001). BGC was associated with modest reduction (15%) in (18) F-FDG uptake at basal ganglia in IH but did not affect dopaminergic function. (18) F-FDG uptake did not correlate with neuropsychological dysfunctions. Interestingly, chronic hypocalcaemia-hyperphosphataemia also contributed to reduction in (18) F-FDG uptake which was independent of BGC. © 2014 John Wiley & Sons Ltd.

  16. Exercise Mode Moderates the Relationship Between Mobility and Basal Ganglia Volume in Healthy Older Adults.

    Science.gov (United States)

    Nagamatsu, Lindsay S; Weinstein, Andrea M; Erickson, Kirk I; Fanning, Jason; Awick, Elizabeth A; Kramer, Arthur F; McAuley, Edward

    2016-01-01

    To examine whether 12 months of aerobic training (AT) moderated the relationship between change in mobility and change in basal ganglia volume than balance and toning (BAT) exercises in older adults. Secondary analysis of a randomized controlled trial. Champaign-Urbana, Illinois. Community-dwelling older adults (N=101; mean age 66.4). Twelve-month exercise trial with two groups: AT and BAT. Mobility was assessed using the Timed Up and Go test. Basal ganglia (putamen, caudate nucleus, pallidum) was segmented from T1-weighted magnetic resonance images using the Oxford Centre for Functional Magnetic Resonance Imaging of the Brain Software Library Integrated Registration and Segmentation Tool. Measurements were obtained at baseline and trial completion. Hierarchical multiple regression was conducted to examine whether exercise mode moderates the relationship between change in mobility and change in basal ganglia volume over 12 months. Age, sex, and education were included as covariates. Exercise significantly moderated the relationship between change in mobility and change in left putamen volume. Specifically, for the AT group, volume of the left putamen did not change, regardless of change in mobility. Similarly, in the BAT group, those who improved their mobility most over 12 months had no change in left putamen volume, although left putamen volume of those who declined in mobility levels decreased significantly. The primary finding that older adults who engaged in 12 months of BAT training and improved mobility exhibited maintenance of brain volume in an important region responsible for motor control provides compelling evidence that such exercises can contribute to the promotion of functional independence and healthy aging. © 2016, Copyright the Authors Journal compilation © 2016, The American Geriatrics Society.

  17. Massive calcification in basal ganglia, thalamus and cerebellum caused by postoperative hypoparathyroidism

    International Nuclear Information System (INIS)

    Toneva, T.; Mlachkova, D.; Kaitazki, L.; Boneva, J.; Yordanova, S.

    2015-01-01

    The depicted case is of a 65 year old woman, who was admitted to hospital with complaints of excess sweating, dizziness and loss of consciousness. Symptomatic epilepsy was established after examination from a neurologist. A CT scan showed hyperdense symmetrical striation of the hemisphere of the small brain (parasagittal); symmetrical double-sided calcifications in the caudate nucleus, globus pallidus, thalamus and medial to the capsula interna; snake-like calcifications of the sulcus (occipital, parasagittai). Paraclinical tests have found hypocalcemia and hypoparathyroidism. Past illnesses: resection of the thyroid due to a nodose struma 20 years before. Key words: Calcifications in Basal Ganglia. Calcifications in the Cerebrum. Hypoparathyroidism

  18. Minimal invasive puncture and drainage versus endoscopic surgery for spontaneous intracerebral hemorrhage in basal ganglia

    Directory of Open Access Journals (Sweden)

    Li Z

    2017-01-01

    Full Text Available Zhihong Li,1,* Yuqian Li,1,* Feifei Xu,2,* Xi Zhang,3 Qiang Tian,4 Lihong Li1 1Department of Neurosurgery, Tangdu Hospital, 2Department of Foreign Languages, 3Department of Biomedical Engineering, 4Department of Radiology, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi Province, People’s Republic of China *These authors contributed equally to this work Abstract: Two prevalent therapies for the treatment of spontaneous intracerebral hemorrhage (ICH in basal ganglia are, minimally invasive puncture and drainage (MIPD, and endoscopic surgery (ES. Because both surgical techniques are of a minimally invasive nature, they have attracted greater attention in recent years. However, evidence comparing the curative effect of MIPD and ES has been uncertain. The indication for MIPD or ES has been uncertain till now. In the present study, 112 patients with spontaneous ICH in basal ganglia who received MIPD or ES were reviewed retrospectively. Baseline parameters prior to the operation, evacuation rate (ER, perihematoma edema, postoperative complications, and rebleeding incidences were collected. Moreover, 1-year postictus, the long-term functional outcomes of patients with regard to hematoma volume (HV or Glasgow Coma Scale (GCS score were judged, respectively, by the case fatality, Glasgow Outcome Scale (GOS, Barthel Index (BI, and modified Rankin Scale (mRS. The ES group had a higher ER than the MIPD group on postoperative day 1. The MIPD group had fewer adverse outcomes, which included less perihematoma edema, anesthetic time, and blood loss, than the ES group. The functional outcomes represented by GOS, BI, and mRS were better in the MIPD group than in the ES group for patients with HV 30–60 mL or GCS score 9–14. These results indicate that ES is more effective in evacuating hematoma in basal ganglia, while MIPD is less invasive than ES. Patients with HV 30–60 mL or GCS score 9–14 may benefit more from the MIPD

  19. Region of Interest Template for the Human Basal Ganglia: Comparing EPI and Standardized Space Approaches

    OpenAIRE

    Janey Prodoehl, PT; Yu, Hong; Little, Deborah M.; Abraham, Ivy; Vaillancourt, David E.

    2007-01-01

    Identifying task related activation in the basal ganglia (BG) is an important area of interest in normal motor systems and cognitive neuroscience. The purpose of this study was to compare changes in brain activation in the BG using results obtained from two different masking methods: a mask drawn in standardized space from a T1 weighted anatomical image and individual region of interest (ROI) masks drawn from each subject’s echo-planar (EPI) image from different tasks with reference to the hi...

  20. Dopamine transporter density of basal ganglia assessed with [123I]IPT SPET in obsessive-compulsive disorder

    International Nuclear Information System (INIS)

    Kim, Chan-Hyung; Cheon, Keun-Ah; Lee, Hong-Shick; Koo, Min-Seong; Ryu, Young-Hoon; Lee, Jong-Doo

    2003-01-01

    It has been suggested that dopamine, as well as serotonin, is associated with the pathophysiology of obsessive-compulsive disorder (OCD). Thus, many studies have been performed on brain regions associated with dopamine in patients with OCD. In the present study, we investigated the DAT density of the basal ganglia using iodine-123 labelled N-(3-iodopropen-2-yl)-2β-carbomethoxy-3β-(4-chlorophenyl) tropane ([ 123 I]IPT) single-photon emission tomography (SPET) and evaluated the activity of the presynaptic dopamine function in patients with OCD. Fifteen patients with OCD and 19 normal control adults were included in the study. We performed brain SPET 2 h after the intravenous administration of [ 123 I]IPT and carried out both quantitative and qualitative analyses using the obtained SPET data, which were reconstructed for the assessment of the specific/non-specific dopamine transporter (DAT) binding ratio in the basal ganglia. We then investigated the correlation between the severity scores of OCD symptoms assessed with the Yale-Brown Obsessive-Compulsive Scale (Y-BOCS) and the specific/non-specific DAT binding ratio of the basal ganglia. Compared with normal control adults, patients with OCD showed a significantly increased specific/non-specific DAT binding ratio in the right basal ganglia and a tendency towards an increased specific/non-specific DAT binding ratio in the left basal ganglia. No significant correlation was found between the total scores on the Y-BOCS and the specific/non-specific DAT binding ratio of the basal ganglia. These findings suggest that the dopaminergic neurotransmitter system of the basal ganglia in patients with OCD could be involved in the pathophysiology of OCD. (orig.)

  1. Dopamine Transporter Density of the Basal Ganglia Assessed with I-123 IPT SPECT in Patients with Obsessive-Compulsive Disorder

    International Nuclear Information System (INIS)

    Ryu, W. K.; Ryu, Y. H.; Yoon, M. J.; Kim, C. H.; Chun, K. A.; Lee, J. D.; Jee, D. Y.; Choi, T. H.

    2003-01-01

    It has been suggested that dopamine as well as serotonin is associated with the pathophysiology of obsessive-compulsive disorder (OCD). Thus, many studies about brain regions associated with dopamine in OCD have been performed. In the present study, we investigated the DAT density of the basal ganglia using iodine-123 labelled N-(3-iodopropen-2-yl) - 2beta - carbomethoxy - 3beta - (4 - chloropheny1) tropane (I-123 IPT) single-photon emission tomography (SPECT) in patients with OCD and evaluated the activity of the presynaptic dopamine function in patients with OCD. Fifteen patients with OCD and nineteen normal control adults were included in the study. We performed brain SPET 2 hours after the intravenous administration of I-123 IPT and carried out both quantitative and qualitative analyses using the obtained SPET data, which were reconstructed for the assessment of the specific/non-specific DAT binding ratio in the basal ganglia. We then investigated the correlation between the severity scores of OCD symptoms assessed with the Yale-Brown Obsessive-Compulsive Scale (Y-BOCS) and the specific/non-specific DAT binding ratio of the basal ganglia. Patients with OCD showed a significantly increased specific/non-specific DAT binding ratio in right basal ganglia compared with normal control adults and an increased tendency in the specific/non-specific DAT binding ratio in left basal ganglia. No significant correlation was found between the total scores of the Y-BOCS and the specific/non-specific DAT binding ratio of the basal ganglia. Our findings suggest that the dopaminergic neurotransmitter system of the basal ganglia in patients with OCD plays an important role in fronto-subcortical circuit well-known as the pathophysiological mechanism of OCD

  2. Functional Relevance of Different Basal Ganglia Pathways Investigated in a Spiking Model with Reward Dependent Plasticity

    Directory of Open Access Journals (Sweden)

    Pierre Berthet

    2016-07-01

    Full Text Available The brain enables animals to behaviourally adapt in order to survive in a complex and dynamic environment, but how reward-oriented behaviours are achieved and computed by its underlying neural circuitry is an open question. To address this concern, we have developed a spiking model of the basal ganglia (BG that learns to dis-inhibit the action leading to a reward despite ongoing changes in the reward schedule. The architecture of the network features the two pathways commonly described in BG, the direct (denoted D1 and the indirect (denoted D2 pathway, as well as a loop involving striatum and the dopaminergic system. The activity of these dopaminergic neurons conveys the reward prediction error (RPE, which determines the magnitude of synaptic plasticity within the different pathways. All plastic connections implement a versatile four-factor learning rule derived from Bayesian inference that depends upon pre- and postsynaptic activity, receptor type and dopamine level. Synaptic weight updates occur in the D1 or D2 pathways depending on the sign of the RPE, and an efference copy informs upstream nuclei about the action selected. We demonstrate successful performance of the system in a multiple-choice learning task with a transiently changing reward schedule. We simulate lesioning of the various pathways and show that a condition without the D2 pathway fares worse than one without D1. Additionally, we simulate the degeneration observed in Parkinson’s disease (PD by decreasing the number of dopaminergic neurons during learning. The results suggest that the D1 pathway impairment in PD might have been overlooked. Furthermore, an analysis of the alterations in the synaptic weights shows that using the absolute reward value instead of the RPE leads to a larger change in D1.

  3. The basal ganglia matching tools package for striatal uptake semi-quantification: description and validation

    International Nuclear Information System (INIS)

    Calvini, Piero; Rodriguez, Guido; Nobili, Flavio; Inguglia, Fabrizio; Mignone, Alessandro; Guerra, Ugo P.

    2007-01-01

    To design a novel algorithm (BasGan) for automatic segmentation of striatal 123 I-FP-CIT SPECT. The BasGan algorithm is based on a high-definition, three-dimensional (3D) striatal template, derived from Talairach's atlas. A blurred template, obtained by convolving the former with a 3D Gaussian kernel (FWHM = 10 mm), approximates striatal activity distribution. The algorithm performs translations and scale transformation on the bicommissural aligned image to set the striatal templates with standard size in an appropriate initial position. An optimization protocol automatically performs fine adjustments in the positioning of blurred templates to best match the radioactive counts, and locates an occipital ROI for background evaluation. Partial volume effect correction is included in the process of uptake computation of caudate, putamen and background. Experimental validation was carried out by means of six acquisitions of an anthropomorphic striatal phantom. The BasGan software was applied to a first set of patients with Parkinson's disease (PD) versus patients affected by essential tremor. A highly significant correlation was achieved between true binding potential and measured 123 I activity from the phantom. 123 I-FP-CIT uptake was significantly lower in all basal ganglia in the PD group versus controls with both BasGan and a conventional ROI method used for comparison, but particularly with the former. Correlations with the motor UPDRS score were far more significant with the BasGan. The novel BasGan algorithm automatically performs the 3D segmentation of striata. Because co-registered MRI is not needed, it can be used by all nuclear medicine departments, since it is freely available on the Web. (orig.)

  4. Functional connectivity in the basal ganglia network differentiates PD patients from controls

    Science.gov (United States)

    Szewczyk-Krolikowski, Konrad; Menke, Ricarda A.L.; Rolinski, Michal; Duff, Eugene; Salimi-Khorshidi, Gholamreza; Filippini, Nicola; Zamboni, Giovanna; Hu, Michele T.M.

    2014-01-01

    Objective: To examine functional connectivity within the basal ganglia network (BGN) in a group of cognitively normal patients with early Parkinson disease (PD) on and off medication compared to age- and sex-matched healthy controls (HC), and to validate the findings in a separate cohort of participants with PD. Methods: Participants were scanned with resting-state fMRI (RS-fMRI) at 3T field strength. Resting-state networks were isolated using independent component analysis. A BGN template was derived from 80 elderly HC participants. BGN maps were compared between 19 patients with PD on and off medication in the discovery group and 19 age- and sex-matched controls to identify a threshold for optimal group separation. The threshold was applied to 13 patients with PD (including 5 drug-naive) in the validation group to establish reproducibility of findings. Results: Participants with PD showed reduced functional connectivity with the BGN in a wide range of areas. Administration of medication significantly improved connectivity. Average BGN connectivity differentiated participants with PD from controls with 100% sensitivity and 89.5% specificity. The connectivity threshold was tested on the validation cohort and achieved 85% accuracy. Conclusions: We demonstrate that resting functional connectivity, measured with MRI using an observer-independent method, is reproducibly reduced in the BGN in cognitively intact patients with PD, and increases upon administration of dopaminergic medication. Our results hold promise for RS-fMRI connectivity as a biomarker in early PD. Classification of evidence: This study provides Class III evidence that average connectivity in the BGN as measured by RS-fMRI distinguishes patients with PD from age- and sex-matched controls. PMID:24920856

  5. Neuronal activity (c-Fos delineating interactions of the cerebral cortex and basal ganglia

    Directory of Open Access Journals (Sweden)

    Mei-Hong eQiu

    2014-03-01

    Full Text Available The cerebral cortex and basal ganglia (BG form a neural circuit that is disrupted in disorders such as Parkinson’s disease. We found that neuronal activity (c-Fos in the BG followed cortical activity, i.e., high in arousal state and low in sleep state. To determine if cortical activity is necessary for BG activity, we administered atropine to rats to induce a dissociative state resulting in slow-wave EEG but hyperactive motor behaviors. Atropine blocked c-Fos expression in the cortex and BG, despite high c-Fos expression in the sub-cortical arousal neuronal groups and thalamus, indicating that cortical activity is required for BG activation. To identify which glutamate receptors in the BG that mediate cortical inputs, we injected ketamine (NMDA receptor antagonist and 6-cyano-nitroquinoxaline-2, 3-dione (CNQX, a non-NMDA receptor antagonist. Systemic ketamine and CNQX administration revealed that NMDA receptors mediated subthalamic nucleus (STN input to internal globus pallidus (GPi and substantia nigra pars reticulata (SNr, while non-NMDA receptor mediated cortical input to the STN. Both types of glutamate receptors were involved in mediating cortical input to the striatum. Dorsal striatal (caudoputamen, CPu dopamine depletion by 6-hydroxydopamine resulted in reduced activity of the CPu, globus pallidus externa (GPe, and STN but increased activity of the GPi, SNr and putative layer V neurons in the motor cortex. Our results reveal that the cortical activity is necessary for BG activity and clarifies the pathways and properties of the BG-cortical network and their putative role in the pathophysiology of BG disorders.

  6. The effects of DBS patterns on basal ganglia activity and thalamic relay : a computational study.

    Science.gov (United States)

    Agarwal, Rahul; Sarma, Sridevi V

    2012-08-01

    Thalamic neurons receive inputs from cortex and their responses are modulated by the basal ganglia (BG). This modulation is necessary to properly relay cortical inputs back to cortex and downstream to the brain stem when movements are planned. In Parkinson's disease (PD), the BG input to thalamus becomes pathological and relay of motor-related cortical inputs is compromised, thereby impairing movements. However, high frequency (HF) deep brain stimulation (DBS) may be used to restore relay reliability, thereby restoring movements in PD patients. Although therapeutic, HF stimulation consumes significant power forcing surgical battery replacements, and may cause adverse side effects. Here, we used a biophysical-based model of the BG-Thalamus motor loop in both healthy and PD conditions to assess whether low frequency stimulation can suppress pathological activity in PD and enable the thalamus to reliably relay movement-related cortical inputs. We administered periodic pulse train DBS waveforms to the sub-thalamic nucleus (STN) with frequencies ranging from 0-140 Hz, and computed statistics that quantified pathological bursting, oscillations, and synchronization in the BG as well as thalamic relay of cortical inputs. We found that none of the frequencies suppressed all pathological activity in BG, though the HF waveforms recovered thalamic reliability. Our rigorous study, however, led us to a novel DBS strategy involving low frequency multi-input phase-shifted DBS, which successfully suppressed pathological symptoms in all BG nuclei and enabled reliable thalamic relay. The neural restoration remained robust to changes in the model parameters characterizing early to late PD stages.

  7. Neurobrucellosis with transient ischemic attack, vasculopathic changes, intracerebral granulomas and basal ganglia infarction: a case report

    Directory of Open Access Journals (Sweden)

    Ozyurek Seyfi C

    2010-10-01

    Full Text Available Abstract Introduction Central nervous system involvement is a rare but serious manifestation of brucellosis. We present an unusual case of neurobrucellosis with transient ischemic attack, intracerebral vasculopathy granulomas, seizures, and paralysis of sixth and seventh cranial nerves. Case presentation A 17-year-old Caucasian man presented with nausea and vomiting, headache, double vision and he gave a history of weakness in the left arm, speech disturbance and imbalance. Physical examination revealed fever, doubtful neck stiffness and left abducens nerve paralysis. An analysis of his cerebrospinal fluid showed a pleocytosis (lymphocytes, 90%, high protein and low glucose levels. He developed generalized tonic-clonic seizures, facial paralysis and left hemiparesis. Cranial magnetic resonance imaging demonstrated intracerebral vasculitis, basal ganglia infarction and granulomas, mimicking the central nervous system involvement of tuberculosis. On the 31st day of his admission, neurobrucellosis was diagnosed with immunoglobulin M and immunoglobulin G positivity by standard tube agglutination test and enzyme-linked immunosorbent assay in both serum and cerebrospinal fluid samples (the tests had been negative until that day. He was treated successfully with trimethoprim and sulfamethoxazole, doxycyline and rifampicin for six months. Conclusions Our patient illustrates the importance of suspecting brucellosis as a cause of meningoencephalitis, even if cultures and serological tests are negative at the beginning of the disease. As a result, in patients who have a history of residence or travel to endemic areas, neurobrucellosis should be considered in the differential diagnosis of any neurologic symptoms. If initial tests fail, repetition of these tests at appropriate intervals along with complementary investigations are indicated.

  8. Temporal changes of CB1 cannabinoid receptor in the basal ganglia as a possible structure-specific plasticity process in 6-OHDA lesioned rats.

    Directory of Open Access Journals (Sweden)

    Gabriela P Chaves-Kirsten

    Full Text Available The endocannabinoid system has been implicated in several neurobiological processes, including neurodegeneration, neuroprotection and neuronal plasticity. The CB1 cannabinoid receptors are abundantly expressed in the basal ganglia, the circuitry that is mostly affected in Parkinson's Disease (PD. Some studies show variation of CB1 expression in basal ganglia in different animal models of PD, however the results are quite controversial, due to the differences in the procedures employed to induce the parkinsonism and the periods analyzed after the lesion. The present study evaluated the CB1 expression in four basal ganglia structures, namely striatum, external globus pallidus (EGP, internal globus pallidus (IGP and substantia nigra pars reticulata (SNpr of rats 1, 5, 10, 20, and 60 days after unilateral intrastriatal 6-hydroxydopamine injections, that causes retrograde dopaminergic degeneration. We also investigated tyrosine hydroxylase (TH, parvalbumin, calbindin and glutamic acid decarboxylase (GAD expression to verify the status of dopaminergic and GABAergic systems. We observed a structure-specific modulation of CB1 expression at different periods after lesions. In general, there were no changes in the striatum, decreased CB1 in IGP and SNpr and increased CB1 in EGP, but this increase was not sustained over time. No changes in GAD and parvalbumin expression were observed in basal ganglia, whereas TH levels were decreased and the calbindin increased in striatum in short periods after lesion. We believe that the structure-specific variation of CB1 in basal ganglia in the 6-hydroxydopamine PD model could be related to a compensatory process involving the GABAergic transmission, which is impaired due to the lack of dopamine. Our data, therefore, suggest that the changes of CB1 and calbindin expression may represent a plasticity process in this PD model.

  9. Dopamine transporter density of the basal ganglia in children with attention deficit hyperactivity disorder assessed with I-123 IPT SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Ryu, Won Gee; Kim, Tae Hoon; Ryu, Young Hoon; Yun, Mi Jin; Lee, Jong Doo; Cheon, Keun Ah [College of Medicine, Yonsei Univ., Seoul (Korea, Republic of); Chi, Dae Yoon [College of Medicine, Inha Univ., Incheon (Korea, Republic of); Kim, Jong Ho; Choi, Tae Hyun [School of Medicine, Gachon Univ., Gachon (Korea, Republic of)

    2003-08-01

    Attention deficit hyperactivity disorder (ADHD) has been known as psychiatric disorder in childhood associated with dopamine dysregulation. In present study, we investigated changes in dopamine transporter (DAT) density of the basal ganglias using I-123 N-(3-iodopropen-2-yl) -2-carbomethoxy-3beta-(4-chlorphenyl) tropane (I-123 IPT) SPECT in children with ADHD before and after methylphenidate treatment. Nine drug-naive children with ADHD and seven normal children were included in the study. We performed brain SPECT two hours after the intravenous administration of I-123 IPT and made both quantitative and qualitative analyses using the obtained SPECT data, which were reconstructed for the assessment of specific/nonspecific DAT binding ratios in the basal ganglia. All children with ADHD reperformed (123I)IPT SPECT after treatment with methylphenidate (0.7mg/kg/d) during about 8 weeks. SPECT data reconstructed for the assessment of specific/nonspecific DAT binding ratio of the basal ganglia were compared between before and after treatment methyphenidate. We investigated correlation between the change of ADHD symptom severity assessed with ADHD rating scale-IV and specific/nonspecific DAT binding ratio of basal ganglia. Children with ADHD had a significantly greater specific/nonspecific DAT binding ratio of the basal ganglia comparing to normal children (Right : z = 2.057, p = 0.041 ; Left : z = 2.096, p = 0.032). Under treatment with methylphenidate in all children with ADHD, specific/nonspecific DAT binding ratio of both ganglia decreased significantly greater than before treatment with methylphenidate (Right : t = 3.239, p = 0.018 ; Left : t = 3.133, p 0.020). However, no significant correlation between the change of ADHD symptom severity scores and specific/nonspecific DAT binding ratio of the basal ganglia were found. These findings support the complex dysregulation of the dopaminergic neurotransmitter system in children with ADHD.

  10. Dopamine transporter density of the basal ganglia in children with attention deficit hyperactivity disorder assessed with I-123 IPT SPECT

    International Nuclear Information System (INIS)

    Ryu, Won Gee; Kim, Tae Hoon; Ryu, Young Hoon; Yun, Mi Jin; Lee, Jong Doo; Cheon, Keun Ah; Chi, Dae Yoon; Kim, Jong Ho; Choi, Tae Hyun

    2003-01-01

    Attention deficit hyperactivity disorder (ADHD) has been known as psychiatric disorder in childhood associated with dopamine dysregulation. In present study, we investigated changes in dopamine transporter (DAT) density of the basal ganglias using I-123 N-(3-iodopropen-2-yl) -2-carbomethoxy-3beta-(4-chlorphenyl) tropane (I-123 IPT) SPECT in children with ADHD before and after methylphenidate treatment. Nine drug-naive children with ADHD and seven normal children were included in the study. We performed brain SPECT two hours after the intravenous administration of I-123 IPT and made both quantitative and qualitative analyses using the obtained SPECT data, which were reconstructed for the assessment of specific/nonspecific DAT binding ratios in the basal ganglia. All children with ADHD reperformed (123I)IPT SPECT after treatment with methylphenidate (0.7mg/kg/d) during about 8 weeks. SPECT data reconstructed for the assessment of specific/nonspecific DAT binding ratio of the basal ganglia were compared between before and after treatment methyphenidate. We investigated correlation between the change of ADHD symptom severity assessed with ADHD rating scale-IV and specific/nonspecific DAT binding ratio of basal ganglia. Children with ADHD had a significantly greater specific/nonspecific DAT binding ratio of the basal ganglia comparing to normal children (Right : z = 2.057, p = 0.041 ; Left : z = 2.096, p = 0.032). Under treatment with methylphenidate in all children with ADHD, specific/nonspecific DAT binding ratio of both ganglia decreased significantly greater than before treatment with methylphenidate (Right : t = 3.239, p = 0.018 ; Left : t = 3.133, p 0.020). However, no significant correlation between the change of ADHD symptom severity scores and specific/nonspecific DAT binding ratio of the basal ganglia were found. These findings support the complex dysregulation of the dopaminergic neurotransmitter system in children with ADHD

  11. MRI pattern of infarcts in basal ganglia region in patients with tuberculous meningitis

    Energy Technology Data Exchange (ETDEWEB)

    Nair, P.P.; Kalita, J.; Misra, U.K. [Sanjay Gandhi Postgraduate Institute of Medical Sciences, Department of Neurology, Lucknow (India); Kumar, S. [Sanjay Gandhi Postgraduate Institute of Medical sciences, Department of Radiology, Lucknow (India)

    2009-04-15

    This study aimed to evaluate the pattern of infarct in basal ganglia region in tuberculous meningitis (TBM) and ischemic strokes and its sensitivity and specificity in the diagnosis of these disorders. Patients with TBM and ischemic strokes in basal ganglia region were retrospectively evaluated from our tuberculous meningitis and ischemic stroke registry. Magnetic resonance imaging findings were grouped into anterior (caudate, genu, anterior limb of internal capsule, anteromedial thalamus) and posterior (lentiform nuclei, posterior limb of internal capsule, posterolateral thalamus). The sensitivity and specificity of these patterns in diagnosing TBM and ischemic stroke were evaluated. There were 24 patients in each group. Infarct in TBM was purely anterior in eight patients and in ischemic stroke purely posterior in 18 patients. The frequency of caudate infarct was significantly higher in TBM compared to ischemic stroke (37.5% vs 8.3%). In TBM patients, purely posterior infarcts were present in seven patients; three had associated risk factors of ischemic stroke. The sensitivity of pure anterior infarct in the diagnosis of TBM was 33%, specificity 91.66%. For ischemic stroke, the sensitivity of posterior infarct was 75% and specificity 70.83%. TBM patients having infarcts in posterior region should be looked for associated risk factors of ischemic stroke. (orig.)

  12. Basal Ganglia Activity Mirrors a Benefit of Action and Reward on Long-Lasting Event Memory.

    Science.gov (United States)

    Koster, Raphael; Guitart-Masip, Marc; Dolan, Raymond J; Düzel, Emrah

    2015-12-01

    The expectation of reward is known to enhance a consolidation of long-term memory for events. We tested whether this effect is driven by positive valence or action requirements tied to expected reward. Using a functional magnetic resonance imaging (fMRI) paradigm in young adults, novel images predicted gain or loss outcomes, which in turn were either obtained or avoided by action or inaction. After 24 h, memory for these images reflected a benefit of action as well as a congruence of action requirements and valence, namely, action for reward and inaction for avoidance. fMRI responses in the hippocampus, a region known to be critical for long-term memory function, reflected the anticipation of inaction. In contrast, activity in the putamen mirrored the congruence of action requirement and valence, whereas other basal ganglia regions mirrored overall action benefits on long-lasting memory. The findings indicate a novel type of functional division between the hippocampus and the basal ganglia in the motivational regulation of long-term memory consolidation, which favors remembering events that are worth acting for. © The Author 2015. Published by Oxford University Press.

  13. CT differential diagnosis between hypertensive putaminal hemorrhage and hemorrhagic infarction localized in basal ganglia

    International Nuclear Information System (INIS)

    Tazawa, Toshiaki; Mizukami, Masahiro; Kawase, Takeshi.

    1984-01-01

    The symptoms of hypertensive putaminal hemorrhage and of middle cerebral artery occlusion are sometimes similar to each other. Hemorrhage sometimes occurs following cerebral infarction. We experienced 7 patients with hemorrhages localized in the basal ganglia following cerebral infarction. The CT findings of 55 patients with putaminal hemorrhage and 7 patients with hemorrhagic infarction localized at the basal ganglia were investigated retrospectively in order to discuss their characteristics. The high-density area (HD) of a putaminal hemorrhage was homogeneous on a plain CT within a week of the onset. There was a close correlation between the size of the HD and the timing of its disappearance. The HD with a maximum diameter of A cm generally disappeared A weeks after. On the other hand, the HD of a hemorrhagic infarction was lower in density than that of the putaminal hemorrhage. The HD of a hemorrhagic infarction generally disappeared earlier than that of a putaminal hemorrhage. Ring enhancement was visualized on contrast-enhanced CT (CECT) from 2 or 3 weeks after the onset in patients with putaminal hemorrhages except in the case of small hemorrhages (less than 1 cm diameter). Ring enhancement was also visualized in 6 out of 7 patients with hemorrhagic infarction; one of them was recognized within a week of the onset. Contrast enhancement of the cortex in the territory of the middle cerebral artery was visualized in 4 out of 7 patients with hemorrhagic infarction. This finding seems to indicate one characteristic of hemorrhagic infarction. (author)

  14. MRI Findings of Syndrome of Acute Bilateral Symmetrical Basal Ganglia Lesions in Diabetic Uremia: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Xin Cao

    2016-01-01

    Full Text Available The syndrome of acute bilateral basal ganglia lesions is an uncommon clinical occurrence exhibiting acute onset of movement abnormalities, which can be seen almost exclusively among patients with chronic renal failure, especially in the setting of concurrent diabetes mellitus. Symmetrical lesions located in basal ganglia demonstrated in MRI are typical manifestation of this syndrome. Our study includes routine MRI examination, MRS, 3D-ASL, and SWI findings, which have been rarely reported and will contribute to diagnosing more cases about this syndrome.

  15. Impaired Frontal-Basal Ganglia Connectivity in Male Adolescents with Conduct Disorder.

    Directory of Open Access Journals (Sweden)

    Jibiao Zhang

    Full Text Available Alack of inhibition control has been found in subjects with conduct disorder (CD, but the underlying neuropathophysiology remains poorly understood. The current study investigated the different mechanism of inhibition control in adolescent-onset CD males (n = 29 and well-matched healthy controls (HCs (n = 40 when performing a GoStop task by functional magnetic resonance images. Effective connectivity (EC within the inhibition control network was analyzed using a stochastic dynamic causality model. We found that EC within the inhibition control network was significantly different in the CD group when compared to the HCs. Exploratory relationship analysis revealed significant negative associations between EC between the IFG and striatum and behavioral scale scores in the CD group. These results suggest for the first time that the failure of inhibition control in subjects with CD might be associated with aberrant connectivity of the frontal-basal ganglia pathways, especially between the IFG and striatum.

  16. Primary hypoparathyroidism presenting as basal ganglia calcification secondary to extreme hypocalcemia

    Directory of Open Access Journals (Sweden)

    Edite Marques Mendes

    2018-01-01

    Full Text Available Hypoparathyroidism is a rare endocrine disorder characterized by low serum calcium and parathyroid hormone levels. The most common cause is parathyroid iatrogenic surgical removal. However, innumerous and rarer conditions can cause hypoparathyroidism. The authors describe a 27-year-old man that presented in emergency department with confusion, amnesia and decreased attention span. A cerebral computed tomography revealed bilateral extensive calcification in the basal ganglia. A complete work-up revealed low serum calcium, high serum phosphorus and low parathyroid hormone, leading to the diagnosis of idiopathic primary hypoparathyroidism. Initial intravenous therapy with calcium gluconate and calcitriol was administered, with clinical and analytical improvement. The authors describe a rare condition, with an exuberant cerebral presentation and extreme hypocalcemia, which did not directly correlate to the severity of symptoms. Not only this is a treatable disorder that may have catastrophic results if overlooked but also its symptoms may be completely reversed with prompt treatment.

  17. Bilateral symmetrical low density areas in the basal ganglia. A case with dysarthria and gait disturbance

    Energy Technology Data Exchange (ETDEWEB)

    Ugawa, Yoshikazu; Ihara, Yasuo (Tokyo Univ. (Japan). Faculty of Medicine)

    1984-04-01

    We reported a case with dysarthria and gait disturbance, in which CT revealed symmetrical well-demarcated low density areas in the basal ganglia. The patient was a 43-year-old woman. Her family history and past history were not contributory. She had a little difficulty in speaking at the age of 17. Gait disturbance and micrographia appeared later. Although her expressionless face resembles to that seen in Parkinsonism, rigidity, akinesia and small-stepped gait were not present. The unclassified types of dysarthria and gait disturbance, which characterize the present case, were considered to be a kind of extrapyramidal symptoms, which were distinct from those of Parkinsonism. CT showed well demarcated low density areas predominantly in bilateral putamen. Metrizamide CT failed to show any communication between low density areas and subarachnoid spaces. To date, six cases, which presented similar clinical features and almost same CT findings as our case, were reported.

  18. Increased functional connectivity in the resting-state basal ganglia network after acute heroin substitution

    Science.gov (United States)

    Schmidt, A; Denier, N; Magon, S; Radue, E-W; Huber, C G; Riecher-Rossler, A; Wiesbeck, G A; Lang, U E; Borgwardt, S; Walter, M

    2015-01-01

    Reinforcement signals in the striatum are known to be crucial for mediating the subjective rewarding effects of acute drug intake. It is proposed that these effects may be more involved in early phases of drug addiction, whereas negative reinforcement effects may occur more in later stages of the illness. This study used resting-state functional magnetic resonance imaging to explore whether acute heroin substitution also induced positive reinforcement effects in striatal brain regions of protracted heroin-maintained patients. Using independent component analysis and a dual regression approach, we compared resting-state functional connectivity (rsFC) strengths within the basal ganglia/limbic network across a group of heroin-dependent patients receiving both an acute infusion of heroin and placebo and 20 healthy subjects who received placebo only. Subsequent correlation analyses were performed to test whether the rsFC strength under heroin exposure correlated with the subjective rewarding effect and with plasma concentrations of heroin and its main metabolites morphine. Relative to the placebo treatment in patients, heroin significantly increased rsFC of the left putamen within the basal ganglia/limbic network, the extent of which correlated positively with patients' feelings of rush and with the plasma level of morphine. Furthermore, healthy controls revealed increased rsFC of the posterior cingulate cortex/precuneus in this network relative to the placebo treatment in patients. Our results indicate that acute heroin substitution induces a subjective rewarding effect via increased striatal connectivity in heroin-dependent patients, suggesting that positive reinforcement effects in the striatum still occur after protracted maintenance therapy. PMID:25803496

  19. Creative cognition and the brain: dissociations between frontal, parietal-temporal and basal ganglia groups.

    Science.gov (United States)

    Abraham, Anna; Beudt, Susan; Ott, Derek V M; Yves von Cramon, D

    2012-10-30

    The objective of the study was to investigate creativity in relation to brain function by assessing creative thinking in various neurological populations. Several measures were employed to assess different facets of creative thinking in clinical groups with frontal lobe, basal ganglia or parietal-temporal lesions relative to matched healthy control participants. The frontal group was subdivided into frontolateral, frontopolar and frontal-extensive groups. Hierarchical regression analyses were employed to assess the significance levels associated with the effects after accounting for IQ differences between the groups. Findings were only considered noteworthy if they at least suggested the presence of a strong trend and were accompanied by medium to large effect sizes. The parietal-temporal and frontolateral groups revealed poorer overall performance with the former demonstrating problems with fluency related measures, whereas the latter were also less proficient at producing original responses. In contrast, the basal ganglia and frontopolar groups demonstrated superior performance in the ability to overcome the constraints imposed by salient semantic distractors when generating creative responses. In summary, the dissociations in the findings reveal the selective involvement of different brain regions in diverse aspects of creativity. Lesion location posed selective limitations on the ability to generate original responses in different contexts, but not on the ability to generate relevant responses, which was compromised in most patient groups. The noteworthy findings from this exploratory study of enhanced performance in specific aspects of creative cognition following brain damage are discussed with reference to the generic idea that superior creative ability can result from altered brain function. Copyright © 2012 Elsevier B.V. All rights reserved.

  20. Default Mode Network, Motor Network, Dorsal and Ventral Basal Ganglia Networks in the Rat Brain: Comparison to Human Networks Using Resting State-fMRI

    Science.gov (United States)

    Sierakowiak, Adam; Monnot, Cyril; Aski, Sahar Nikkhou; Uppman, Martin; Li, Tie-Qiang; Damberg, Peter; Brené, Stefan

    2015-01-01

    Rodent models are developed to enhance understanding of the underlying biology of different brain disorders. However, before interpreting findings from animal models in a translational aspect to understand human disease, a fundamental step is to first have knowledge of similarities and differences of the biological systems studied. In this study, we analyzed and verified four known networks termed: default mode network, motor network, dorsal basal ganglia network, and ventral basal ganglia network using resting state functional MRI (rsfMRI) in humans and rats. Our work supports the notion that humans and rats have common robust resting state brain networks and that rsfMRI can be used as a translational tool when validating animal models of brain disorders. In the future, rsfMRI may be used, in addition to short-term interventions, to characterize longitudinal effects on functional brain networks after long-term intervention in humans and rats. PMID:25789862

  1. Dynamic stereotypic responses of basal ganglia neurons to subthalamic nucleus high frequency stimulation in the parkinsonian primate

    Directory of Open Access Journals (Sweden)

    Anan eMoran

    2011-04-01

    Full Text Available Deep brain stimulation in the subthalamic nucleus (STN is a well-established therapy for patients with severe Parkinson‟s disease (PD; however, its mechanism of action is still unclear. In this study we explored static and dynamic activation patterns in the basal ganglia during high frequency macro-stimulation of the STN. Extracellular multi-electrode recordings were performed in primates rendered parkinsonian using 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Recordings were preformed simultaneously in the STN and the globus pallidus externus and internus. Single units were recorded preceding and during the stimulation. During the stimulation, STN mean firing rate dropped significantly, while pallidal mean firing rates did not change significantly. The vast majority of neurons across all three nuclei displayed stimulation driven modulations, which were stereotypic within each nucleus but differed across nuclei. The predominant response pattern of STN neurons was somatic inhibition. However, most pallidal neurons demonstrated synaptic activation patterns. A minority of neurons across all nuclei displayed axonal activation. Temporal dynamics were observed in the response to stimulation over the first 10 seconds in the STN and over the first 30 seconds in the pallidum. In both pallidal segments, the synaptic activation response patterns underwent delay and decay of the magnitude of the peak response due to short term synaptic depression. We suggest that during STN macro stimulation the STN goes through a functional ablation as its upper bound on information transmission drops significantly. This notion is further supported by the evident dissociation between the stimulation driven pre-synaptic STN somatic inhibition and the post-synaptic axonal activation of its downstream targets. Thus, basal ganglia output maintains its firing rate while losing the deleterious effect of the STN. This may be a part of the mechanism leading to the beneficial

  2. Changes in total cell numbers of the basal ganglia in patients with multiple system atrophy - A stereological study

    DEFF Research Database (Denmark)

    Salvesen, Lisette; Ullerup, Birgitte H; Sunay, Fatma B

    2014-01-01

    Total numbers of neurons, oligodendrocytes, astrocytes, and microglia in the basal ganglia and red nucleus were estimated in brains from 11 patients with multiple system atrophy (MSA) and 11 age- and gender-matched control subjects with unbiased stereological methods. Compared to the control...

  3. Consensus Paper: Towards a Systems-Level View of Cerebellar Function: the Interplay Between Cerebellum, Basal Ganglia, and Cortex

    NARCIS (Netherlands)

    Caligiore, D.; Pezzulo, G.; Baldassarre, G.; Bostan, A.C.; Strick, P.L.; Doya, K.; Helmich, R.C.G.; Dirkx, M.F.M.; Houk, J.; Jorntell, H.; Lago-Rodriguez, A.; Galea, J.M.; Miall, R.C.; Popa, T.; Kishore, A.; Verschure, P.F.; Zucca, R.; Herreros, I.

    2017-01-01

    Despite increasing evidence suggesting the cerebellum works in concert with the cortex and basal ganglia, the nature of the reciprocal interactions between these three brain regions remains unclear. This consensus paper gathers diverse recent views on a variety of important roles played by the

  4. Structural differences in basal ganglia of elite running versus martial arts athletes: a diffusion tensor imaging study.

    Science.gov (United States)

    Chang, Yu-Kai; Tsai, Jack Han-Chao; Wang, Chun-Chih; Chang, Erik Chihhung

    2015-07-01

    The aim of this study was to use diffusion tensor imaging (DTI) to characterize and compare microscopic differences in white matter integrity in the basal ganglia between elite professional athletes specializing in running and martial arts. Thirty-three young adults with sport-related skills as elite professional runners (n = 11) or elite professional martial artists (n = 11) were recruited and compared with non-athletic and healthy controls (n = 11). All participants underwent health- and skill-related physical fitness assessments. Fractional anisotropy (FA) and mean diffusivity (MD), the primary indices derived from DTI, were computed for five regions of interest in the bilateral basal ganglia, including the caudate nucleus, putamen, globus pallidus internal segment (GPi), globus pallidus external segment (GPe), and subthalamic nucleus. Results revealed that both athletic groups demonstrated better physical fitness indices compared with their control counterparts, with the running group exhibiting the highest cardiovascular fitness and the martial arts group exhibiting the highest muscular endurance and flexibility. With respect to the basal ganglia, both athletic groups showed significantly lower FA and marginally higher MD values in the GPi compared with the healthy control group. These findings suggest that professional sport or motor skill training is associated with changes in white matter integrity in specific regions of the basal ganglia, although these positive changes did not appear to depend on the type of sport-related motor skill being practiced.

  5. Basal Ganglia Structures Differentially Contribute to Verbal Fluency: Evidence from Human Immunodeficiency Virus (HIV)-Infected Adults

    Science.gov (United States)

    Thames, April D.; Foley, Jessica M.; Wright, Matthew J.; Panos, Stella E.; Ettenhofer, Mark; Ramezani, Amir; Streiff, Vanessa; El-Saden, Suzie; Goodwin, Scott; Bookheimer, Susan Y.; Hinkin, Charles H.

    2012-01-01

    Background: The basal ganglia (BG) are involved in executive language functions (i.e., verbal fluency) through their connections with cortical structures. The caudate and putamen receive separate inputs from prefrontal and premotor cortices, and may differentially contribute to verbal fluency performance. We examined BG integrity in relation to…

  6. Radiological imaging features of the basal ganglia that may predict progression to hemicraniectomy in large territory middle cerebral artery infarct

    Energy Technology Data Exchange (ETDEWEB)

    Mian, Asim Z.; Edasery, David; Sakai, Osamu; Mustafa Qureshi, M. [Boston University School of Medicine, Department of Radiology, Boston Medical Center, Boston, MA 02118 (United States); Holsapple, James [Boston University School of Medicine, Department of Neurosurgery, Boston Medical Center, Boston, MA (United States); Nguyen, Thanh [Boston University School of Medicine, Department of Neurology, Boston Medical Center, Boston, MA (United States)

    2017-05-15

    Predicting which patients are at risk for hemicraniectomy can be helpful for triage and can help preserve neurologic function if detected early. We evaluated basal ganglia imaging predictors for early hemicraniectomy in patients with large territory anterior circulation infarct. This retrospective study evaluated patients with ischemic infarct admitted from January 2005 to July 2011. Patients with malignant cerebral edema refractory to medical therapy or with herniating signs such as depressed level of consciousness, anisocoria, and contralateral leg weakness were triaged to hemicraniectomy. Admission images were reviewed for presence of caudate, lentiform nucleus (putamen and globus pallidus), or basal ganglia (caudate + lentiform nucleus) infarction. Thirty-one patients with large territory MCA infarct, 10 (32%), underwent hemicraniectomy. Infarction of the caudate nucleus (9/10 vs 6/21, p = 0.002) or basal ganglia (5/10 vs 2/21, p = 0.02) predicted progression to hemicraniectomy. Infarction of the lentiform nucleus only did not predict progression to hemicraniectomy. Sensitivity for patients who did and did not have hemicraniectomy were 50% (5/10) and 90.5% (19/21). For caudate nucleus and caudate plus lentiform nucleus infarcts, the crude- and age-adjusted odds of progression to hemicraniectomy were 9.5 (1.4-64.3) and 6.6 (0.78-55.4), respectively. Infarction of the caudate nucleus or basal ganglia correlated with patients progressing to hemicraniectomy. Infarction of the lentiform nucleus alone did not. (orig.)

  7. How preparation changes the need for top-down control of the basal ganglia when inhibiting premature actions.

    Science.gov (United States)

    Jahfari, Sara; Verbruggen, Frederick; Frank, Michael J; Waldorp, Lourens J; Colzato, Lorenza; Ridderinkhof, K Richard; Forstmann, Birte U

    2012-08-08

    Goal-oriented signals from the prefrontal cortex gate the selection of appropriate actions in the basal ganglia. Key nodes within this fronto-basal ganglia action regulation network are increasingly engaged when one anticipates the need to inhibit and override planned actions. Here, we ask how the advance preparation of action plans modulates the need for fronto-subcortical control when a planned action needs to be withdrawn. Functional magnetic resonance imaging data were collected while human participants performed a stop task with cues indicating the likelihood of a stop signal being sounded. Mathematical modeling of go trial responses suggested that participants attained a more cautious response strategy when the probability of a stop signal increased. Effective connectivity analysis indicated that, even in the absence of stop signals, the proactive engagement of the full control network is tailored to the likelihood of stop trial occurrence. Importantly, during actual stop trials, the strength of fronto-subcortical projections was stronger when stopping had to be engaged reactively compared with when it was proactively prepared in advance. These findings suggest that fronto-basal ganglia control is strongest in an unpredictable environment, where the prefrontal cortex plays an important role in the optimization of reactive control. Importantly, these results further indicate that the advance preparation of action plans reduces the need for reactive fronto-basal ganglia communication to gate voluntary actions.

  8. Basal ganglia and thalamic input from neurons located within the ventral tier cell cluster region of the substantia nigra pars compacta in the rat.

    Science.gov (United States)

    Cebrián, Carolina; Prensa, Lucía

    2010-04-15

    The most caudally located dopaminergic (DA) ventral tier neurons of the substantia nigra pars compacta (SNc) form typical cell clusters that are deeply embedded in the substantia nigra pars reticulata (SNr). Here we examine the efferent projections of 35 neurons located in the SNr region where these SNc cell clusters reside. The neuronal cell body was injected with biotinylated dextran amine so as to trace each complete axon in the sagittal or the coronal plane. Electrophysiological guidance guaranteed that the tracer was ejected among neurons displaying a typical SNc discharge pattern. Furthermore, double immunofluorescence and immunohistochemical labeling ensured that the tracer deposits were placed within the DA cell clusters. Three types of projection neurons occurred in the SNc ventral tier cell cluster region: type I neurons, projecting to basal ganglia; type II neurons, targeting both the basal ganglia and thalamus; and type III neurons, projecting only to the thalamus. The striatum was targeted by most of the type I and II neurons and the innervation reached both the striosome/subcallosal streak and matrix compartments. Many nigrostriatal fibers provided collaterals to the globus pallidus and, less frequently, to the subthalamic nucleus. At a thalamic level, type II and III neurons preferentially targeted the reticular, ventral posterolateral, and ventral medial nuclei. Our results reveal that the SNr region where DA ventral tier cell clusters reside harbors neurons projecting to the basal ganglia and/or the thalamus, thus suggesting that neurodegeneration of nigral neurons in Parkinson's disease might affect various extrastriatal basal ganglia structures and multiple thalamic nuclei. (c) 2009 Wiley-Liss, Inc.

  9. Endoscopic surgery versus conservative treatment for the moderate-volume hematoma in spontaneous basal ganglia hemorrhage (ECMOH: study protocol for a randomized controlled trial

    Directory of Open Access Journals (Sweden)

    Zan Xin

    2012-06-01

    Full Text Available Abstract Background Spontaneous intracerebral hemorrhage is a disease with high morbidity, high disability rate, high mortality, and high economic burden. Whether patients can benefit from surgical evacuation of hematomas is still controversial, especially for those with moderate-volume hematomas in the basal ganglia. This study is designed to compare the efficacy of endoscopic surgery and conservative treatment for the moderate-volume hematoma in spontaneous basal ganglia hemorrhage. Methods Patients meet the criteria will be randomized into the endoscopic surgery group (endoscopic surgery for hematoma evacuation and the best medical treatment or the conservative treatment group (the best medical treatment. Patients will be followed up at 1, 3, and 6 months after initial treatment. The primary outcomes include the Extended Glasgow Outcome Scale and the Modified Rankin Scale. The secondary outcomes consist of the National Institutes of Health Stroke Scale and the mortality. The Barthel Index(BI will also be evaluated. The sample size is 100 patients. Discussion The ECMOH trial is a randomized controlled trial designed to evaluate if endoscopic surgery is better than conservative treatment for patients with moderate-volume hematomas in the basal ganglia. Trial registration Chinese Clinical Trial Registry: ChiCTR-TRC-11001614 (http://www.chictr.org/en/proj/show.aspx?proj=1618

  10. Analysis of Grey Matter in Thalamus and Basal Ganglia Based on EEG α3/α2 Frequency Ratio Reveals Specific Changes in Subjects with Mild Cognitive Impairment

    Directory of Open Access Journals (Sweden)

    Davide V Moretti

    2012-11-01

    Full Text Available GM (grey matter changes of thalamus and basal ganglia have been demonstrated to be involved in AD (Alzheimer's disease. Moreover, the increase of a specific EEG (electroencephalogram marker, α3/α2, have been associated with AD-converters subjects with MCI (mild cognitive impairment. To study the association of prognostic EEG markers with specific GM changes of thalamus and basal ganglia in subjects with MCI to detect biomarkers (morpho-physiological early predictive of AD and non-AD dementia. Seventy-four adult subjects with MCI underwent EEG recording and high-resolution 3D MRI (three-dimensional magnetic resonance imaging. The α3/α2 ratio was computed for each subject. Three groups were obtained according to increasing tertile values of α3/α2 ratio. GM density differences between groups were investigated using a VBM (voxel-based morphometry technique. Subjects with higher α3/α2 ratios when compared with subjects with lower and middle α3/α2 ratios showed minor atrophy in the ventral stream of basal ganglia (head of caudate nuclei and accumbens nuclei bilaterally and of the pulvinar nuclei in the thalamus; The integrated analysis of EEG and morpho-structural markers could be useful in the comprehension of anatomo-physiological underpinning of the MCI entity.

  11. Chronic alcohol exposure disrupts top-down control over basal ganglia action selection to produce habits.

    Science.gov (United States)

    Renteria, Rafael; Baltz, Emily T; Gremel, Christina M

    2018-01-15

    Addiction involves a predominance of habitual control mediated through action selection processes in dorsal striatum. Research has largely focused on neural mechanisms mediating a proposed progression from ventral to dorsal lateral striatal control in addiction. However, over reliance on habit striatal processes may also arise from reduced cortical input to striatum, thereby disrupting executive control over action selection. Here, we identify novel mechanisms through which chronic intermittent ethanol exposure and withdrawal (CIE) disrupts top-down control over goal-directed action selection processes to produce habits. We find CIE results in decreased excitability of orbital frontal cortex (OFC) excitatory circuits supporting goal-directed control, and, strikingly, selectively reduces OFC output to the direct output pathway in dorsal medial striatum. Increasing the activity of OFC circuits restores goal-directed control in CIE-exposed mice. Our findings show habitual control in alcohol dependence can arise through disrupted communication between top-down, goal-directed processes onto basal ganglia pathways controlling action selection.

  12. A de novo nonsense PDGFB mutation causing idiopathic basal ganglia calcification with laryngeal dystonia.

    Science.gov (United States)

    Nicolas, Gaël; Jacquin, Agnès; Thauvin-Robinet, Christel; Rovelet-Lecrux, Anne; Rouaud, Olivier; Pottier, Cyril; Aubriot-Lorton, Marie-Hélène; Rousseau, Stéphane; Wallon, David; Duvillard, Christian; Béjot, Yannick; Frébourg, Thierry; Giroud, Maurice; Campion, Dominique; Hannequin, Didier

    2014-10-01

    Idiopathic basal ganglia calcification (IBGC) is characterized by brain calcification and a wide variety of neurologic and psychiatric symptoms. In families with autosomal dominant inheritance, three causative genes have been identified: SLC20A2, PDGFRB, and, very recently, PDGFB. Whereas in clinical practice sporadic presentation of IBGC is frequent, well-documented reports of true sporadic occurrence are rare. We report the case of a 20-year-old woman who presented laryngeal dystonia revealing IBGC. Her healthy parents' CT scans were both normal. We identified in the proband a new nonsense mutation in exon 4 of PDGFB, c.439C>T (p.Gln147*), which was absent from the parents' DNA. This mutation may result in a loss-of-function of PDGF-B, which has been shown to cause IBGC in humans and to disrupt the blood-brain barrier in mice, resulting in brain calcification. The c.439C>T mutation is located between two previously reported nonsense mutations, c.433C>T (p.Gln145*) and c.445C>T (p.Arg149*), on a region that could be a hot spot for de novo mutations. We present the first full demonstration of the de novo occurrence of an IBGC-causative mutation in a sporadic case.

  13. Technical integration of hippocampus, Basal Ganglia and physical models for spatial navigation.

    Science.gov (United States)

    Fox, Charles; Humphries, Mark; Mitchinson, Ben; Kiss, Tamas; Somogyvari, Zoltan; Prescott, Tony

    2009-01-01

    Computational neuroscience is increasingly moving beyond modeling individual neurons or neural systems to consider the integration of multiple models, often constructed by different research groups. We report on our preliminary technical integration of recent hippocampal formation, basal ganglia and physical environment models, together with visualisation tools, as a case study in the use of Python across the modelling tool-chain. We do not present new modeling results here. The architecture incorporates leaky-integrator and rate-coded neurons, a 3D environment with collision detection and tactile sensors, 3D graphics and 2D plots. We found Python to be a flexible platform, offering a significant reduction in development time, without a corresponding significant increase in execution time. We illustrate this by implementing a part of the model in various alternative languages and coding styles, and comparing their execution times. For very large-scale system integration, communication with other languages and parallel execution may be required, which we demonstrate using the BRAHMS framework's Python bindings.

  14. Technical integration of hippocampus, basal ganglia and physical models for spatial navigation

    Directory of Open Access Journals (Sweden)

    Charles W Fox

    2009-03-01

    Full Text Available Computational neuroscience is increasingly moving beyond modeling individual neurons or neural systems to consider the integration of multiple models, often constructed by different research groups. We report on our preliminary technical integration of recent hippocampal formation, basal ganglia and physical environment models, together with visualisation tools, as a case study in the use of Python across the modelling tool-chain. We do not present new modeling results here. The architecture incorporates leaky-integrator and rate-coded neurons, a 3D environment with collision detection and tactile sensors, 3D graphics and 2D plots. We found Python to be a flexible platform, offering a significant reduction in development time, without a corresponding significant increase in execution time. We illustrate this by implementing a part of the model in various alternative languages and coding styles, and comparing their execution times. For very large scale system integration, communication with other languages and parallel execution may be required, which we demonstrate using the BRAHMS framework's Python bindings.

  15. Identifying enhanced cortico-basal ganglia loops associated with prolonged dance training.

    Science.gov (United States)

    Li, Gujing; He, Hui; Huang, Mengting; Zhang, Xingxing; Lu, Jing; Lai, Yongxiu; Luo, Cheng; Yao, Dezhong

    2015-06-02

    Studies have revealed that prolonged, specialized training combined with higher cognitive conditioning induces enhanced brain alternation. In particular, dancers with long-term dance experience exhibit superior motor control and integration with their sensorimotor networks. However, little is known about the functional connectivity patterns of spontaneous intrinsic activities in the sensorimotor network of dancers. Our study examined the functional connectivity density (FCD) of dancers with a mean period of over 10 years of dance training in contrast with a matched non-dancer group without formal dance training using resting-state fMRI scans. FCD was mapped and analyzed, and the functional connectivity (FC) analyses were then performed based on the difference of FCD. Compared to the non-dancers, the dancers exhibited significantly increased FCD in the precentral gyri, postcentral gyri and bilateral putamen. Furthermore, the results of the FC analysis revealed enhanced connections between the middle cingulate cortex and the bilateral putamen and between the precentral and the postcentral gyri. All findings indicated an enhanced functional integration in the cortico-basal ganglia loops that govern motor control and integration in dancers. These findings might reflect improved sensorimotor function for the dancers consequent to long-term dance training.

  16. A reverse engineering algorithm for neural networks, applied to the subthalamopallidal network of basal ganglia.

    Science.gov (United States)

    Floares, Alexandru George

    2008-01-01

    Modeling neural networks with ordinary differential equations systems is a sensible approach, but also very difficult. This paper describes a new algorithm based on linear genetic programming which can be used to reverse engineer neural networks. The RODES algorithm automatically discovers the structure of the network, including neural connections, their signs and strengths, estimates its parameters, and can even be used to identify the biophysical mechanisms involved. The algorithm is tested on simulated time series data, generated using a realistic model of the subthalamopallidal network of basal ganglia. The resulting ODE system is highly accurate, and results are obtained in a matter of minutes. This is because the problem of reverse engineering a system of coupled differential equations is reduced to one of reverse engineering individual algebraic equations. The algorithm allows the incorporation of common domain knowledge to restrict the solution space. To our knowledge, this is the first time a realistic reverse engineering algorithm based on linear genetic programming has been applied to neural networks.

  17. Radiation Absorbed Dose to the Basal Ganglia from Dopamine Transporter Radioligand 18F-FPCIT

    Directory of Open Access Journals (Sweden)

    William Robeson

    2014-01-01

    Full Text Available Our previous dosimetry studies have demonstrated that for dopaminergic radiotracers, 18F-FDOPA and 18F-FPCIT, the urinary bladder is the critical organ. As these tracers accumulate in the basal ganglia (BG with high affinity and long residence times, radiation dose to the BG may become significant, especially in normal control subjects. We have performed dynamic PET measurements using 18F-FPCIT in 16 normal adult subjects to determine if in fact the BG, although not a whole organ, but a well-defined substructure, receives the highest dose. Regions of interest were drawn over left and right BG structures. Resultant time-activity curves were generated and used to determine residence times for dosimetry calculations. S-factors were computed using the MIRDOSE3 nodule model for each caudate and putamen. For 18F-FPCIT, BG dose ranged from 0.029 to 0.069 mGy/MBq. In half of all subjects, BG dose exceeded 85% of the published critical organ (bladder dose, and in three of those, the BG dose exceeded that for the bladder. The BG can become the dose-limiting organ in studies using dopamine transporter ligands. For some normal subjects studied with F-18 or long half-life radionuclide, the BG may exceed bladder dose and become the critical structure.

  18. Decision making under uncertainty in a spiking neural network model of the basal ganglia.

    Science.gov (United States)

    Héricé, Charlotte; Khalil, Radwa; Moftah, Marie; Boraud, Thomas; Guthrie, Martin; Garenne, André

    2016-12-01

    The mechanisms of decision-making and action selection are generally thought to be under the control of parallel cortico-subcortical loops connecting back to distinct areas of cortex through the basal ganglia and processing motor, cognitive and limbic modalities of decision-making. We have used these properties to develop and extend a connectionist model at a spiking neuron level based on a previous rate model approach. This model is demonstrated on decision-making tasks that have been studied in primates and the electrophysiology interpreted to show that the decision is made in two steps. To model this, we have used two parallel loops, each of which performs decision-making based on interactions between positive and negative feedback pathways. This model is able to perform two-level decision-making as in primates. We show here that, before learning, synaptic noise is sufficient to drive the decision-making process and that, after learning, the decision is based on the choice that has proven most likely to be rewarded. The model is then submitted to lesion tests, reversal learning and extinction protocols. We show that, under these conditions, it behaves in a consistent manner and provides predictions in accordance with observed experimental data.

  19. Identifying enhanced cortico-basal ganglia loops associated with prolonged dance training

    Science.gov (United States)

    Li, Gujing; He, Hui; Huang, Mengting; Zhang, Xingxing; Lu, Jing; Lai, Yongxiu; Luo, Cheng; Yao, Dezhong

    2015-01-01

    Studies have revealed that prolonged, specialized training combined with higher cognitive conditioning induces enhanced brain alternation. In particular, dancers with long-term dance experience exhibit superior motor control and integration with their sensorimotor networks. However, little is known about the functional connectivity patterns of spontaneous intrinsic activities in the sensorimotor network of dancers. Our study examined the functional connectivity density (FCD) of dancers with a mean period of over 10 years of dance training in contrast with a matched non-dancer group without formal dance training using resting-state fMRI scans. FCD was mapped and analyzed, and the functional connectivity (FC) analyses were then performed based on the difference of FCD. Compared to the non-dancers, the dancers exhibited significantly increased FCD in the precentral gyri, postcentral gyri and bilateral putamen. Furthermore, the results of the FC analysis revealed enhanced connections between the middle cingulate cortex and the bilateral putamen and between the precentral and the postcentral gyri. All findings indicated an enhanced functional integration in the cortico-basal ganglia loops that govern motor control and integration in dancers. These findings might reflect improved sensorimotor function for the dancers consequent to long-term dance training. PMID:26035693

  20. Unusual progression of herpes simplex encephalitis with basal ganglia and extensive white matter involvement

    Directory of Open Access Journals (Sweden)

    Yasuhiro Manabe

    2009-08-01

    Full Text Available We report a 51-year old male with herpes simplex encephalitis (HSE showing unusual progression and magnetic resonance (MR findings. The initial neurological manifestation of intractable focal seizure with low-grade fever persisted for three days, and rapidly coma, myoclonic status, and respiratory failure with high-grade fever emerged thereafter. The polymerase chain reaction (PCR result of cerebrospinal fluid (CSF was positive for HSV-1 DNA. In the early stage, MR images (MRI were normal. On subsequent MR diffusion-weighted (DW and fluid-attenuated inversion recovery (FLAIR images, high-intensity areas first appeared in the left frontal cortex, which was purely extra-temporal involvement, and extended into the basal ganglia, then the white matter, which are relatively spared in HSE. Antiviral therapy and immunosuppressive therapy did not suppress the progression of HSE, and finally severe cerebral edema developed into cerebral herniation, which required emergency decompressive craniectomy. Histological examination of a biopsy specimen of the white matter detected perivascular infiltration and destruction of basic structure, which confirmed non specific inflammatory change without obvious edema or demyelination. The present case shows both MR and pathological findings in the white matter in the acute stage of HSE.

  1. The pulvinar nucleus is associated with the presence of dysarthria in patients with basal ganglia hemorrhage.

    Science.gov (United States)

    Kim, Dae Hyun; Kyeong, Sunghyon; Ahn, Sung Jun; Park, Yoon Ghil

    2017-08-10

    Dysarthria is a frequent symptom in patients with stroke. The anatomical structures responsible for dysarthria have been reported in patients with lacunar infarcts, but the related lesions in patients with basal ganglia hemorrhage (BGH) have not been investigated. The aim of this study was to identify associations between the lesion location and the presence/absence of dysarthria in patients with BGH using voxel-based lesion symptom mapping (VLSM) analyses. A retrospective analysis was conducted on 26 patients with acute BGH (mean age, 54.0 years; men:women, 14:12) who underwent conservative management. The patients were classified into groups based on the presence or absence of dysarthria at the time of admission, which was determined by reviewing the patients' medical records. Brain lesions were traced on magnetic resonance images that were acquired within the first 3 weeks after BGH onset, and then separate high-resolution region-of-interest images were generated. Associations between dysarthria and the lesion location were determined with the VLSM analyses. The average volume of the delimited lesions was 7.38±5.75cm 3 . The VLSM analyses identified several voxel clusters, mainly in the pulvinar nucleus of the left thalamus, that were significantly related to the presence of dysarthria at admission. These findings suggest that patients with BGH extending into the left pulvinar nucleus should be monitored for dysarthria. Copyright © 2017 Elsevier B.V. All rights reserved.

  2. Reduced basal ganglia function when elderly switch between coordinated movement patterns.

    Science.gov (United States)

    Coxon, James P; Goble, Daniel J; Van Impe, Annouchka; De Vos, Jeroen; Wenderoth, Nicole; Swinnen, Stephan P

    2010-10-01

    Structural and neurochemical changes in frontostriatal circuits are thought to underlie age-related behavioral deficits on cognitive tasks. Here, we test the hypothesis that age-related motor switching deficits are associated with reduced basal ganglia (BG) function. Right-handed volunteers (15 Old, and 15 Young) made spatially and temporally coupled bimanual circular motions during event-related FMRI. A visual cue signaled the right hand to Switch or Continue its circling direction. Switching from mirror symmetric to asymmetric (SW»ASYMM) took longer and resulted in more contralateral (left-) hand disruptions than vice versa. These effects were more pronounced in the elderly, showing that the ability to suppress and flexibly adapt motor behavior (agility) declines with age. For both groups, switching activated the BG and a typical network for task-set implementation, including dorsal anterior cingulate cortex/supplementary motor area (pre-SMA, SMA-proper) and anterior insula/inferior frontal gyrus. A region of interest analysis revealed significantly reduced SW»ASYMM activation in bilateral subthalamic nucleus and right globus pallidus, only in the elderly. Age-related behavioral deficits may be related to inefficient recruitment of cortico-BG loops to suppress undesired movements. The elderly may use an alternative strategy to select the required movement pattern as indicated by increased activation of prefrontal cortex.

  3. [Pathomorphologic changes of the solar plexus ganglia in burn disease].

    Science.gov (United States)

    Isaev, I M; Muzykant, L I; Tumanov, V P; Gasanov, R P

    1989-01-01

    Solar ganglia taken from 64 subjects who died in different periods of burn disease: (shock, toxemia septico toxemia, and burn exhaustion) were pathomorphologically examined. The specific time course of changes in solar ganglia was established in various periods of the disease. Dystrophic changes, necrosis of individual neuron groups, and varices of myelinated fibers were detected during burn shock, hypertrophic neurons with thick processes interwoven in between as "felt" predominanted in toxemia whereas in septicotoxemia and burn exhaustion, necrotic changes in neurons and degeneration of nerve fibrous bundle.

  4. The Relationship of Hematoma Size and Mortality in Non-Traumatic Intra-Cerebral Hemorrhages in Basal Ganglia

    Directory of Open Access Journals (Sweden)

    P. Ahmadi

    2006-04-01

    Full Text Available Introduction & Objective: Among all of the neurologic diseases in adult life, the cerebrovascular disease (CVD is the most common and important ones. Intracerebral hemorrhage (ICH in basal ganglia (BG is one of the common and major types of CVD. The relations between clot size and mortality rate, in different parts of the brain, has been addressed by several researchers. It is unclear whether such a relationship is in BG. Therefore this study was designed to find a formula that predicts outcome of hemorrhage based on clot size in BG.Materials & Methods: This descriptive-comparative study that was carried out prospectively, conducted on all 63 patients who admitted to the hospital during one year, with definite diagnosis of ICH in BG. After urgent CT scanning, the size of hematoma was determined by scan images. Routine treatment was uniform for all patients. Focal signs and consciousness state were assessed in the first and last days of admission. The data were analyzed using descriptive statistics, frequency tables and chi-square and T- test. Results: 33% of patients died. Hematoma size in 70% of them was larger than 5cm and in other 30% smaller. None of the hematoma with less than 4cm size was fatal. In patients with clots of 5cm or larger, the mortality was 100%. Conclusion: The results indicated that, there was meaningful relationship between hematoma size and mortality, in BG hemorrhages. So the clot size can be used as a factor in predicting hemorrhage outcome in BG.

  5. Novel SLC19A3 Promoter Deletion and Allelic Silencing in Biotin-Thiamine-Responsive Basal Ganglia Encephalopathy.

    Directory of Open Access Journals (Sweden)

    Irene Flønes

    Full Text Available Biotin-thiamine responsive basal ganglia disease is a severe, but potentially treatable disorder caused by mutations in the SLC19A3 gene. Although the disease is inherited in an autosomal recessive manner, patients with typical phenotypes carrying single heterozygous mutations have been reported. This makes the diagnosis uncertain and may delay treatment.In two siblings with early-onset encephalopathy dystonia and epilepsy, whole-exome sequencing revealed a novel single heterozygous SLC19A3 mutation (c.337T>C. Although Sanger-sequencing and copy-number analysis revealed no other aberrations, RNA-sequencing in brain tissue suggested the second allele was silenced. Whole-genome sequencing resolved the genetic defect by revealing a novel 45,049 bp deletion in the 5'-UTR region of the gene abolishing the promoter. High dose thiamine and biotin therapy was started in the surviving sibling who remains stable. In another patient two novel compound heterozygous SLC19A3 mutations were found. He improved substantially on thiamine and biotin therapy.We show that large genomic deletions occur in the regulatory region of SLC19A3 and should be considered in genetic testing. Moreover, our study highlights the power of whole-genome sequencing as a diagnostic tool for rare genetic disorders across a wide spectrum of mutations including non-coding large genomic rearrangements.

  6. Application of Deep Learning in Neuroradiology: Automated Detection of Basal Ganglia Hemorrhage using 2D-Convolutional Neural Networks

    OpenAIRE

    Desai, Vishal; Flanders, Adam E.; Lakhani, Paras

    2017-01-01

    Background: Deep learning techniques have achieved high accuracy in image classification tasks, and there is interest in applicability to neuroimaging critical findings. This study evaluates the efficacy of 2D deep convolutional neural networks (DCNNs) for detecting basal ganglia (BG) hemorrhage on noncontrast head CT. Materials and Methods: 170 unique de-identified HIPAA-compliant noncontrast head CTs were obtained, those with and without BG hemorrhage. 110 cases were held-out for test, and ...

  7. A spiking neuron model of the cortico-basal ganglia circuits for goal-directed and habitual action learning.

    Science.gov (United States)

    Chersi, Fabian; Mirolli, Marco; Pezzulo, Giovanni; Baldassarre, Gianluca

    2013-05-01

    Dual-system theories postulate that actions are supported either by a goal-directed or by a habit-driven response system. Neuroimaging and anatomo-functional studies have provided evidence that the prefrontal cortex plays a fundamental role in the first type of action control, while internal brain areas such as the basal ganglia are more active during habitual and overtrained responses. Additionally, it has been shown that areas of the cortex and the basal ganglia are connected through multiple parallel "channels", which are thought to function as an action selection mechanism resolving competitions between alternative options available in a given context. In this paper we propose a multi-layer network of spiking neurons that implements in detail the thalamo-cortical circuits that are believed to be involved in action learning and execution. A key feature of this model is that neurons are organized in small pools in the motor cortex and form independent loops with specific pools of the basal ganglia where inhibitory circuits implement a multistep selection mechanism. The described model has been validated utilizing it to control the actions of a virtual monkey that has to learn to turn on briefly flashing lights by pressing corresponding buttons on a board. When the animal is able to fluently execute the task the button-light associations are remapped so that it has to suppress its habitual behavior in order to execute goal-directed actions. The model nicely shows how sensory-motor associations for action sequences are formed at the cortico-basal ganglia level and how goal-directed decisions may override automatic motor responses. Copyright © 2012 Elsevier Ltd. All rights reserved.

  8. Model-based analysis and control of a network of basal ganglia spiking neurons in the normal and Parkinsonian states

    Science.gov (United States)

    Liu, Jianbo; Khalil, Hassan K.; Oweiss, Karim G.

    2011-08-01

    Controlling the spatiotemporal firing pattern of an intricately connected network of neurons through microstimulation is highly desirable in many applications. We investigated in this paper the feasibility of using a model-based approach to the analysis and control of a basal ganglia (BG) network model of Hodgkin-Huxley (HH) spiking neurons through microstimulation. Detailed analysis of this network model suggests that it can reproduce the experimentally observed characteristics of BG neurons under a normal and a pathological Parkinsonian state. A simplified neuronal firing rate model, identified from the detailed HH network model, is shown to capture the essential network dynamics. Mathematical analysis of the simplified model reveals the presence of a systematic relationship between the network's structure and its dynamic response to spatiotemporally patterned microstimulation. We show that both the network synaptic organization and the local mechanism of microstimulation can impose tight constraints on the possible spatiotemporal firing patterns that can be generated by the microstimulated network, which may hinder the effectiveness of microstimulation to achieve a desired objective under certain conditions. Finally, we demonstrate that the feedback control design aided by the mathematical analysis of the simplified model is indeed effective in driving the BG network in the normal and Parskinsonian states to follow a prescribed spatiotemporal firing pattern. We further show that the rhythmic/oscillatory patterns that characterize a dopamine-depleted BG network can be suppressed as a direct consequence of controlling the spatiotemporal pattern of a subpopulation of the output Globus Pallidus internalis (GPi) neurons in the network. This work may provide plausible explanations for the mechanisms underlying the therapeutic effects of deep brain stimulation (DBS) in Parkinson's disease and pave the way towards a model-based, network level analysis and closed

  9. Significant Risk Factors for Postoperative Enlargement of Basal Ganglia Hematoma after Frameless Stereotactic Aspiration: Antiplatelet Medication and Concomitant IVH.

    Science.gov (United States)

    Son, Wonsoo; Park, Jaechan

    2017-09-01

    Frameless stereotactic aspiration of a hematoma can be the one of the treatment options for spontaneous intracerebral hemorrhage in the basal ganglia. Postoperative hematoma enlargement, however, can be a serious complication of intracranial surgery that frequently results in severe neurological deficit and even death. Therefore, it is important to identify the risk factors of postoperative hematoma growth. During a 13-year period, 101 patients underwent minimally invasive frameless stereotactic aspiration for basal ganglia hematoma. Patients were classified into two groups according to whether or not they had postoperative hematoma enlargement in a computed tomography scan. Baseline demographic data and several risk factors, such as hypertension, preoperative hematoma growth, antiplatelet medication, presence of concomitant intraventricular hemorrhage (IVH), were analysed via a univariate statistical study. Nine of 101 patients (8.9%) showed hematoma enlargement after frameless stereotactic aspiration. Among the various risk factors, concomitant IVH and antiplatelet medication were found to be significantly associated with postoperative enlargement of hematomas. In conclusion, our study revealed that aspirin use and concomitant IVH are factors associated with hematoma enlargement subsequent to frameless stereotactic aspiration for basal ganglia hematoma.

  10. Evidence for the importance of basal ganglia output nuclei in semantic event sequencing: an fMRI study.

    Science.gov (United States)

    Tinaz, Sule; Schendan, Haline E; Schon, Karin; Stern, Chantal E

    2006-01-05

    Semantic event sequencing is the ability to plan ahead and order meaningful events chronologically. To investigate the neural systems supporting this ability, an fMRI picture sequencing task was developed. Participants sequenced a series of four pictures presented in random order based on the temporal relationship among them. A control object discrimination task was designed to be comparable to the sequencing task regarding semantic, visuospatial, and motor processing requirements but without sequencing demands. fMRI revealed significant activation in the dorsolateral prefrontal cortex and globus pallidus internal part in the picture sequencing task compared with the control task. The findings suggest that circuits involving the frontal lobe and basal ganglia output nuclei are important for picture sequencing and more generally for the sequential ordering of events. This is consistent with the idea that the basal ganglia output nuclei are critical not only for motor but also for high-level cognitive function, including behaviors involving meaningful information. We suggest that the interaction between the frontal lobes and basal ganglia output nuclei in semantic event sequencing can be generalized to include the sequential ordering of behaviors in which the selective updating of neural representations is the key computation.

  11. The Arbitration–Extension Hypothesis: A Hierarchical Interpretation of the Functional Organization of the Basal Ganglia

    Science.gov (United States)

    Kamali Sarvestani, Iman; Lindahl, Mikael; Hellgren-Kotaleski, Jeanette; Ekeberg, Örjan

    2011-01-01

    Based on known anatomy and physiology, we present a hypothesis where the basal ganglia motor loop is hierarchically organized in two main subsystems: the arbitration system and the extension system. The arbitration system, comprised of the subthalamic nucleus, globus pallidus, and pedunculopontine nucleus, serves the role of selecting one out of several candidate actions as they are ascending from various brain stem motor regions and aggregated in the centromedian thalamus or descending from the extension system or from the cerebral cortex. This system is an action-input/action-output system whose winner-take-all mechanism finds the strongest response among several candidates to execute. This decision is communicated back to the brain stem by facilitating the desired action via cholinergic/glutamatergic projections and suppressing conflicting alternatives via GABAergic connections. The extension system, comprised of the striatum and, again, globus pallidus, can extend the repertoire of responses by learning to associate novel complex states to certain actions. This system is a state-input/action-output system, whose organization enables it to encode arbitrarily complex Boolean logic rules using striatal neurons that only fire given specific constellations of inputs (Boolean AND) and pallidal neurons that are silenced by any striatal input (Boolean OR). We demonstrate the capabilities of this hierarchical system by a computational model where a simulated generic “animal” interacts with an environment by selecting direction of movement based on combinations of sensory stimuli, some being appetitive, others aversive or neutral. While the arbitration system can autonomously handle conflicting actions proposed by brain stem motor nuclei, the extension system is required to execute learned actions not suggested by external motor centers. Being precise in the functional role of each component of the system, this hypothesis generates several readily testable

  12. Stimulation sites in the subthalamic nucleus projected onto a mean 3-D atlas of the thalamus and basal ganglia.

    Science.gov (United States)

    Sarnthein, Johannes; Péus, Dominik; Baumann-Vogel, Heide; Baumann, Christian R; Sürücü, Oguzkan

    2013-09-01

    In patients with severe forms of Parkinson's disease (PD), deep brain stimulation (DBS) commonly targets the subthalamic nucleus (STN). Recently, the mean 3-D Morel-Atlas of the basal ganglia and the thalamus was introduced. It combines information contained in histological data from ten post-mortem brains. We were interested whether the Morel-Atlas is applicable for the visualization of stimulation sites. In a consecutive PD patient series, we documented preoperative MRI planning, intraoperative target adjustment based on electrophysiological and neurological testing, and perioperative CT target reconstruction. The localization of the DBS electrodes and the optimal stimulation sites were projected onto the Morel-Atlas. We included 20 patients (median age 62 years). The active contact had mean coordinates Xlat = ±12.1 mm, Yap = -1.8 mm, Zvert = -3.2 mm. There was a significant difference between the initially planned site and the coordinates of the postoperative active contact site (median 2.2 mm). The stimulation site was, on average, more anterior and more dorsal. The electrode contact used for optimal stimulation was found within the STN of the atlas in 38/40 (95 %) of implantations. The cluster of stimulation sites in individual patients-as deduced from preoperative MR, intraoperative electrophysiology and neurological testing-showed a high degree of congruence with the atlas. The mean 3D Morel Atlas is thus a useful tool for postoperative target visualization. This represents the first clinical evaluation of the recently created atlas.

  13. Loss of function of Slc20a2 associated with familial idiopathic Basal Ganglia calcification in humans causes brain calcifications in mice

    DEFF Research Database (Denmark)

    Jensen, N.; Schroder, H. D.; Hejbol, E. K.

    2013-01-01

    Familial idiopathic basal ganglia calcification (FIBGC) is a neurodegenerative disorder with neuropsychiatric and motor symptoms. Deleterious mutations in SLC20A2, encoding the type III sodium-dependent phosphate transporter 2 (PiT2), were recently linked to FIBGC in almost 50% of the families...... reported worldwide. Here, we show that knockout of Slc20a2 in mice causes calcifications in the thalamus, basal ganglia, and cortex, demonstrating that reduced PiT2 expression alone can cause brain calcifications....

  14. Correlation of iron deposition and change of gliocyte metabolism in the basal ganglia region evaluated using magnetic resonance imaging techniques: an in vivo study.

    Science.gov (United States)

    Liu, Haodi; Wang, Xiaoming

    2016-02-01

    We assessed the correlation between iron deposition and the change of gliocyte metabolism in healthy subjects' basal ganglia region, by using 3D-enhanced susceptibility weighted angiography (ESWAN) and proton magnetic resonance spectroscopy ((1)H-MRS). Seventy-seven healthy volunteers (39 female and 38 male subjects; age range: 24-82 years old) were enrolled in the experiment including ESWAN and proton MRS sequences, consent for which was provided by themselves or their guardians. For each subject, the mean phase value gained by ESWAN was used to evaluate the iron deposition; choline/creatine (Cho/Cr) and mI/Cr ratios gained by (1)H-MRS were used to evaluate gliocyte metabolism in the basal ganglia region of both sides. The paired t test was used to test the difference between the two sides of the basal ganglia region. Linear regression was performed to evaluate the relation between mean phase value and age. Pearson's correlation coefficient was calculated to analyze the relationship between the result of ESWAN and (1)H-MRS. There was no difference between the two sides of the basal ganglia region in the mean phase value and Cho/Cr. But in mI/Cr the mean phase value of each nucleus in bilateral basal ganglia decreased with increasing age. There are 16 r-values between the mean phase value and Cho/Cr and mI/Cr in bilateral basal ganglia region. And each of all p-values is less than 0.001 (p basal ganglia is associated with the change of gliocyte metabolism with increasing age. Iron deposition in each nucleus of the basal ganglia region changes with age.

  15. The Effects of Medium Spiny Neuron Morphologcial Changes on Basal Ganglia Network under External Electric Field: A Computational Modeling Study

    Directory of Open Access Journals (Sweden)

    Xiaohan Zhang

    2017-10-01

    Full Text Available The damage of dopaminergic neurons that innervate the striatum has been considered to be the proximate cause of Parkinson's disease (PD. In the dopamine-denervated state, the loss of dendritic spines and the decrease of dendritic length may prevent medium spiny neuron (MSN from receiving too much excitatory stimuli from the cortex, thereby reducing the symptom of Parkinson's disease. However, the reduction in dendritic spine density obtained by different experiments is significantly different. We developed a biological-based network computational model to quantify the effect of dendritic spine loss and dendrites tree degeneration on basal ganglia (BG signal regulation. Through the introduction of error index (EI, which was used to measure the attenuation of the signal, we explored the amount of dendritic spine loss and dendritic trees degradation required to restore the normal regulatory function of the network, and found that there were two ranges of dendritic spine loss that could reduce EI to normal levels in the case of dopamine at a certain level, this was also true for dendritic trees. However, although these effects were the same, the mechanisms of these two cases were significant difference. Using the method of phase diagram analysis, we gained insight into the mechanism of signal degradation. Furthermore, we explored the role of cortex in MSN morphology changes dopamine depletion-induced and found that proper adjustments to cortical activity do stop the loss in dendritic spines induced by dopamine depleted. These results suggested that modifying cortical drive onto MSN might provide a new idea on clinical therapeutic strategies for Parkinson's disease.

  16. No change of dopamine transporter density in basal ganglia after risperidone treatment in drug-naive children with Tourette's disorder

    International Nuclear Information System (INIS)

    Ryu, W. K.; Ryu, Y. H.; Yoon, M. J.; Chun, K. A.; Lee, J. D.; Zee, D. Y.; Choi, T. H.

    2003-01-01

    Tourette's disorder (TD), which is characterized by multiple waxing and waning motor tics and one or more vocal tics, is known to be associated with abnormalities in the dopaminergic system. To testify our hypothesis that risperidone would improve tic symptoms of TD patients through the change of the dopaminergic system, we measured the DAT densities between drug-naive children with TD and normal children investigated the DAT density before and after treatment with risperidone in drug-naive children with TD, using lodine-123 labelled N-(3-iodopropen-2-yl)-2beta-carbomethoxy-3beta-(4-chlorophenyl) tropane(I-123 IPT) single photon emission computed tomography (SPECT). I-123 IPT SPECT imaging and Yale Global Tic Severity Scale-Korean version (YGTSS-K) for assessing the tic symptom severity were carried out before and after treatment with risperidone for 8 weeks in eight drug-naive children with TD. Eight normal children also underwent SPECT imaging 2 hours after an intravenous administration of I-123 IPT and carried out both quantitative and qualitative analyses using the obtained SPECT data, which were reconstructed for the assessment of the specific/non-specific DAT binding ratio in the basal ganglia. The drug-naive children with TD had a significantly greater increase in the specific/nonspecific DAT binding ratio of both basal ganglia compared with the normal children. However, no significant difference in the specific/nonspecific DAT binding ratio of the basal ganglia before and after treatment with riperidone in children with TD was not found, although tic symptoms were significantly improved with risperidone. These findings suggest that DAT densities are directly associated with the pathophysiology of TD, however, that the effect of risperidone on tic symptoms in children with TD is not attributed to the change of dopaminergic system

  17. Basal ganglia perfusion using dynamic color Doppler sonography in infants with hypoxic ischemic encephalopathy receiving therapeutic hypothermia: a pilot study.

    Science.gov (United States)

    Faingold, Ricardo; Cassia, Guilherme; Morneault, Linda; Saint-Martin, Christine; Sant'Anna, Guilherme

    2016-10-01

    The objective of this study was to evaluate the cerebral perfusion of the basal ganglia in infants with hypoxic-ischemic encephalopathy (HIE) receiving hypothermia using dynamic color Doppler sonography (CDS) and investigate for any correlation between these measurements and survival. Head ultrasound (HUS) was performed with a 9S4 MHz sector transducer in HIE infants submitted to hypothermia as part of their routine care. Measurements of cerebral perfusion intensity (CPI) with an 11LW4 MHz linear array transducer were performed to obtain static images and DICOM color Doppler videos of the blood flow in the basal ganglia area. Clinical and radiological data were evaluated retrospectively. The video images were analyzed by two radiologists using dedicated software, which allows automatic quantification of color Doppler data from a region of interest (ROI) by dynamically assessing color pixels and flow velocity during the heart cycle. CPI is expressed in cm/sec and is calculated by multiplying the mean velocity of all pixels divided by the area of the ROI. Three videos of 3 seconds each were obtained of the ROI, in the coronal plane, and used to calculate the CPI. Data are presented as mean ± SEM or median (quartiles). A total of 28 infants were included in this study: 16 male, 12 female. HUS was performed within the first 48 hours of therapeutic hypothermia treatment. CPI values were significantly higher in the seven non-survivors when compared to survivors (0.226±0.221 vs . 0.111±0.082 cm/sec; P=0.02). Increased perfusion intensity of the basal ganglia area within the first 48 of therapeutic hypothermia treatment was associated with poor outcome in neonates with HIE.

  18. Urokinase vs Tissue-Type Plasminogen Activator for Thrombolytic Evacuation of Spontaneous Intracerebral Hemorrhage in Basal Ganglia

    Directory of Open Access Journals (Sweden)

    Yuqian Li

    2017-08-01

    Full Text Available Spontaneous intracerebral hemorrhage (ICH is a devastating form of stroke, which leads to a high rate of mortality and poor neurological outcomes worldwide. Thrombolytic evacuation with urokinase-type plasminogen activator (uPA or tissue-type plasminogen activator (tPA has been showed to be a hopeful treatment for ICH. However, to the best of our knowledge, no clinical trials were reported to compare the efficacy and safety of these two fibrinolytics administrated following minimally invasive stereotactic puncture (MISP in patients with spontaneous basal ganglia ICH. Therefore, the authors intended here to evaluate the differential impact of uPA and tPA in a retrospective study. In the present study, a total of 86 patients with spontaneous ICH in basal ganglia using MISP received either uPA (uPA group, n = 45 or tPA (tPA group, n = 41, respectively. The clinical baseline characteristics prior to the operation were collected. In addition, therapeutic responses were assessed by the short-term outcomes within 30 days postoperation, as well as long-term outcomes at 1 year postoperation. Our findings showed that, in comparison with tPA, uPA was able to better promote hematoma evacuation and ameliorate perihematomal edema, but the differences were not statistically significant. Moreover, the long-term functional outcomes of both groups were similar, with no statistical difference. In conclusion, these results provide evidence supporting that uPA and tPA are similar in the efficacy and safety for thrombolytic evacuation in combination with MISP in patients with spontaneous basal ganglia ICH.

  19. Proton MR spectroscopic imaging of basal ganglia and thalamus in neurofibromatosis type 1: correlation with T2 hyperintensities

    Energy Technology Data Exchange (ETDEWEB)

    Barbier, Charlotte; Barantin, Laurent [CHRU and Tours University, Department of Neuroradiology, Tours (France); Chabernaud, Camille [CHRU and Tours University et INSERM U930, Department of Pediatric Neurology, Tours (France); Bertrand, Philippe [CHRU and Tours University, Department of Radiology, Tours (France); Sembely, Catherine; Sirinelli, Dominique [CHRU and Tours University, Department of Pediatric Radiology, Tours (France); Castelnau, Pierre [CHRU and Tours University et INSERM U930, Department of Pediatric Neurology, Tours (France); CHRU and Tours University et INSERM U930, Tours (France); Neurologie Pediatrique and INSERM U930, Hopital d' Enfants Gatien de Clocheville, Tours cedex 09 (France); Cottier, Jean-Philippe [CHRU and Tours University, Department of Neuroradiology, Tours (France); CHRU and Tours University et INSERM U930, Tours (France)

    2011-02-15

    Neurofibromatosis type 1 (NF1) is frequently associated with hyperintense lesions on T2-weighted images called ''unidentified bright objects'' (UBO). To better characterize the functional significance of UBO, we investigate the basal ganglia and thalamus using spectroscopic imaging in children with NF1 and compare the results to anomalies observed on T2-weighted images. Magnetic resonance (MR) data of 25 children with NF1 were analyzed. On the basis of T2-weighted images analysis, two groups were identified: one with normal MR imaging (UBO- group; n = 10) and one with UBO (UBO+ group; n = 15). Within the UBO+ group, a subpopulation of patients (n = 5) only had lesions of the basal ganglia. We analyzed herein seven regions of interest (ROIs) for each side: caudate nucleus, capsulo-lenticular region, lateral and posterior thalamus, thalamus (lateral and posterior voxels combined), putamen, and striatum. For each ROI, a spectrum of the metabolites and their ratio was obtained. Patients with abnormalities on T2-weighted images had significantly lower NAA/Cr, NAA/Cho, and NAA/mI ratios in the lateral right thalamus compared with patients with normal T2. These abnormal spectroscopic findings were not observed in capsulo-lenticular regions that had UBO but in the thalamus region that was devoid of UBO. Multivoxel spectroscopic imaging using short-time echo showed spectroscopic abnormalities in the right thalamus of NF1 patients harboring UBO, which were mainly located in the basal ganglia. This finding could reflect the anatomical and functional interactions of these regions. (orig.)

  20. OCD candidate gene SLC1A1/EAAT3 impacts basal ganglia-mediated activity and stereotypic behavior.

    Science.gov (United States)

    Zike, Isaac D; Chohan, Muhammad O; Kopelman, Jared M; Krasnow, Emily N; Flicker, Daniel; Nautiyal, Katherine M; Bubser, Michael; Kellendonk, Christoph; Jones, Carrie K; Stanwood, Gregg; Tanaka, Kenji Fransis; Moore, Holly; Ahmari, Susanne E; Veenstra-VanderWeele, Jeremy

    2017-05-30

    Obsessive-compulsive disorder (OCD) is a chronic, disabling condition with inadequate treatment options that leave most patients with substantial residual symptoms. Structural, neurochemical, and behavioral findings point to a significant role for basal ganglia circuits and for the glutamate system in OCD. Genetic linkage and association studies in OCD point to SLC1A1 , which encodes the neuronal glutamate/aspartate/cysteine transporter excitatory amino acid transporter 3 (EAAT3)/excitatory amino acid transporter 1 (EAAC1). However, no previous studies have investigated EAAT3 in basal ganglia circuits or in relation to OCD-related behavior. Here, we report a model of Slc1a1 loss based on an excisable STOP cassette that yields successful ablation of EAAT3 expression and function. Using amphetamine as a probe, we found that EAAT3 loss prevents expected increases in ( i ) locomotor activity, ( ii ) stereotypy, and ( iii ) immediate early gene induction in the dorsal striatum following amphetamine administration. Further, Slc1a1 -STOP mice showed diminished grooming in an SKF-38393 challenge experiment, a pharmacologic model of OCD-like grooming behavior. This reduced grooming is accompanied by reduced dopamine D 1 receptor binding in the dorsal striatum of Slc1a1 -STOP mice. Slc1a1 -STOP mice also exhibit reduced extracellular dopamine concentrations in the dorsal striatum both at baseline and following amphetamine challenge. Viral-mediated restoration of Slc1a1 /EAAT3 expression in the midbrain but not in the striatum results in partial rescue of amphetamine-induced locomotion and stereotypy in Slc1a1 -STOP mice, consistent with an impact of EAAT3 loss on presynaptic dopaminergic function. Collectively, these findings indicate that the most consistently associated OCD candidate gene impacts basal ganglia-dependent repetitive behaviors.

  1. Interregional correlations of glucose metabolism between the basal ganglia and different cortical areas: an ultra-high resolution PET/MRI fusion study using 18F-FDG.

    Science.gov (United States)

    Kim, J H; Son, Y D; Kim, J M; Kim, H K; Kim, Y B; Lee, C; Oh, C H

    2017-11-13

    Basal ganglia have complex functional connections with the cerebral cortex and are involved in motor control, executive functions of the forebrain, such as the planning of movement, and cognitive behaviors based on their connections. The aim of this study was to provide detailed functional correlation patterns between the basal ganglia and cerebral cortex by conducting an interregional correlation analysis of the 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) data based on precise structural information. Fifteen participants were scanned with 7-Tesla magnetic resonance imaging (MRI) and high resolution research tomography (HRRT)-PET fusion system using 18F-FDG. For detailed interregional correlation analysis, 24 subregions of the basal ganglia including pre-commissural dorsal caudate, post-commissural caudate, pre-commissural dorsal putamen, post-commissural putamen, internal globus pallidus, and external globus pallidus and 80 cerebral regions were selected as regions of interest on the MRI image and their glucose metabolism were calculated from the PET images. Pearson's product-moment correlation analysis was conducted for the interregional correlation analysis of the basal ganglia. Functional correlation patterns between the basal ganglia and cerebral cortex were not only consistent with the findings of previous studies, but also showed new functional correlation between the dorsal striatum (i.e., caudate nucleus and putamen) and insula. In this study, we established the detailed basal ganglia subregional functional correlation patterns using 18F-FDG PET/MRI fusion imaging. Our methods and results could potentially be an important resource for investigating basal ganglia dysfunction as well as for conducting functional studies in the context of movement and psychiatric disorders.

  2. Adaptive autoregressive identification with spectral power decomposition for studying movement-related activity in scalp EEG signals and basal ganglia local field potentials

    Science.gov (United States)

    Foffani, Guglielmo; Bianchi, Anna M.; Priori, Alberto; Baselli, Giuseppe

    2004-09-01

    We propose a method that combines adaptive autoregressive (AAR) identification and spectral power decomposition for the study of movement-related spectral changes in scalp EEG signals and basal ganglia local field potentials (LFPs). This approach introduces the concept of movement-related poles, allowing one to study not only the classical event-related desynchronizations (ERD) and synchronizations (ERS), which correspond to modulations of power, but also event-related modulations of frequency. We applied the method to analyze movement-related EEG signals and LFPs contemporarily recorded from the sensorimotor cortex, the globus pallidus internus (GPi) and the subthalamic nucleus (STN) in a patient with Parkinson's disease who underwent stereotactic neurosurgery for the implant of deep brain stimulation (DBS) electrodes. In the AAR identification we compared the whale and the exponential forgetting factors, showing that the whale forgetting provides a better disturbance rejection and it is therefore more suitable to investigate movement-related brain activity. Movement-related power modulations were consistent with previous studies. In addition, movement-related frequency modulations were observed from both scalp EEG signals and basal ganglia LFPs. The method therefore represents an effective approach to the study of movement-related brain activity.

  3. Acupuncture inhibits Notch1 and Hes1 protein expression in the basal ganglia of rats with cerebral hemorrhage

    Directory of Open Access Journals (Sweden)

    Wei Zou

    2015-01-01

    Full Text Available Notch pathway activation maintains neural stem cells in a proliferating state and increases nerve repair capacity. To date, studies have rarely focused on changes or damage to signal transduction pathways during cerebral hemorrhage. Here, we examined the effect of acupuncture in a rat model of cerebral hemorrhage. We examined four groups: in the control group, rats received no treatment. In the model group, cerebral hemorrhage models were established by infusing non-heparinized blood into the brain. In the acupuncture group, modeled rats had Baihui (DU20 and Qubin (GB7 acupoints treated once a day for 30 minutes. In the DAPT group, modeled rats had 0.15 μg/mL DAPT solution (10 mL infused into the brain. Immunohistochemistry and western blot results showed that acupuncture effectively inhibits Notch1 and Hes1 protein expression in rat basal ganglia. These inhibitory effects were identical to DAPT, a Notch signaling pathway inhibitor. Our results suggest that acupuncture has a neuroprotective effect on cerebral hemorrhage by inhibiting Notch-Hes signaling pathway transduction in rat basal ganglia after cerebral hemorrhage.

  4. T1-weighted hyperintensity in basal ganglia at brain magnetic resonance imaging: are different pathologies sharing a common mechanism?

    Science.gov (United States)

    Herrero Hernandez, Elena; Valentini, Maria Consuelo; Discalzi, Gianluigi

    2002-12-01

    Basal ganglia bilateral symmetric hyperintensity in T1-weighted sequences at magnetic resonance imaging (MRI) is recognized to be due to the presence of manganese deposits. This abnormal finding has been reported in occupational exposures, liver cirrhosis and total parenteral nutrition with unbalanced solutions. However, the same imaging is often observed "by chance" in brain MRIs of patients not belonging to these groups. In order to better understand which are the clinical conditions coexisting with such findings, we decided to study systematically patients which showed this kind of imaging, focusing on their manganese and iron status, as it is known that these two metals have similar properties and that iron-deficiency can competitively increase manganese absorption. The 20 patients studied underwent clinical evaluation and the following laboratory tests: whole blood iron and manganese, hemoglobin, plasma iron, transferrin and ferritin. The neuroradiologic evaluation was integrated by pallidal index calculation, in order to provide a semi-quantitative esteem of the hyperintensity. The patients could be classified into four subgroups: Parkinsonism, anemia, cirrhosis, central nervous system tumors. In 18 out of 20 cases, we found abnormalities in iron and/or manganese-related values. Particularly, iron-deficiency seems to be frequent among patients showing brain MRI abnormalities compatible with manganese deposits in basal ganglia. This observation suggests that iron-deficiency could be an important risk factor for manganese-induced neurotoxicity and should, therefore, be accurately considered and treated.

  5. Prefrontal Activity and Connectivity with the Basal Ganglia during Performance of Complex Cognitive Tasks Is Associated with Apathy in Healthy Subjects.

    Science.gov (United States)

    Fazio, Leonardo; Logroscino, Giancarlo; Taurisano, Paolo; Amico, Graziella; Quarto, Tiziana; Antonucci, Linda Antonella; Barulli, Maria Rosaria; Mancini, Marina; Gelao, Barbara; Ferranti, Laura; Popolizio, Teresa; Bertolino, Alessandro; Blasi, Giuseppe

    2016-01-01

    Convergent evidence indicates that apathy affects cognitive behavior in different neurological and psychiatric conditions. Studies of clinical populations have also suggested the primary involvement of the prefrontal cortex and the basal ganglia in apathy. These brain regions are interconnected at both the structural and functional levels and are deeply involved in cognitive processes, such as working memory and attention. However, it is unclear how apathy modulates brain processing during cognition and whether such a modulation occurs in healthy young subjects. To address this issue, we investigated the link between apathy and prefrontal and basal ganglia function in healthy young individuals. We hypothesized that apathy may be related to sub-optimal activity and connectivity in these brain regions. Three hundred eleven healthy subjects completed an apathy assessment using the Starkstein's Apathy Scale and underwent fMRI during working memory and attentional performance tasks. Using an ROI approach, we investigated the association of apathy with activity and connectivity in the DLPFC and the basal ganglia. Apathy scores correlated positively with prefrontal activity and negatively with prefrontal-basal ganglia connectivity during both working memory and attention tasks. Furthermore, prefrontal activity was inversely related to attentional behavior. These results suggest that in healthy young subjects, apathy is a trait associated with inefficient cognitive-related prefrontal activity, i.e., it increases the need for prefrontal resources to process cognitive stimuli. Furthermore, apathy may alter the functional relationship between the prefrontal cortex and the basal ganglia during cognition.

  6. Prefrontal Activity and Connectivity with the Basal Ganglia during Performance of Complex Cognitive Tasks Is Associated with Apathy in Healthy Subjects.

    Directory of Open Access Journals (Sweden)

    Leonardo Fazio

    Full Text Available Convergent evidence indicates that apathy affects cognitive behavior in different neurological and psychiatric conditions. Studies of clinical populations have also suggested the primary involvement of the prefrontal cortex and the basal ganglia in apathy. These brain regions are interconnected at both the structural and functional levels and are deeply involved in cognitive processes, such as working memory and attention. However, it is unclear how apathy modulates brain processing during cognition and whether such a modulation occurs in healthy young subjects. To address this issue, we investigated the link between apathy and prefrontal and basal ganglia function in healthy young individuals. We hypothesized that apathy may be related to sub-optimal activity and connectivity in these brain regions.Three hundred eleven healthy subjects completed an apathy assessment using the Starkstein's Apathy Scale and underwent fMRI during working memory and attentional performance tasks. Using an ROI approach, we investigated the association of apathy with activity and connectivity in the DLPFC and the basal ganglia.Apathy scores correlated positively with prefrontal activity and negatively with prefrontal-basal ganglia connectivity during both working memory and attention tasks. Furthermore, prefrontal activity was inversely related to attentional behavior.These results suggest that in healthy young subjects, apathy is a trait associated with inefficient cognitive-related prefrontal activity, i.e., it increases the need for prefrontal resources to process cognitive stimuli. Furthermore, apathy may alter the functional relationship between the prefrontal cortex and the basal ganglia during cognition.

  7. Dopamine transporter density in the basal ganglia assessed with [123I]IPT SPET in children with attention deficit hyperactivity disorder

    International Nuclear Information System (INIS)

    Cheon, Keun-Ah; Kim, Young-Kee; Namkoong, Kee; Kim, Chan-Hyung; Ryu, Young Hoon; Lee, Jong Doo

    2003-01-01

    Attention deficit hyperactivity disorder (ADHD) is a psychiatric disorder in childhood that is known to be associated with dopamine dysregulation. In this study, we investigated dopamine transporter (DAT) density in children with ADHD using iodine-123 labelled N-(3-iodopropen-2-yl)-2β-carbomethoxy-3β-(4-chlorophenyl) tropane ([ 123 I]IPT) single-photon emission tomography (SPET) and postulated that an alteration in DAT density in the basal ganglia is responsible for dopaminergic dysfunction in children with ADHD. Nine drug-naive children with ADHD and six normal children were included in the study. We performed brain SPET 2 h after the intravenous administration of [ 123 I]IPT and carried out both quantitative and qualitative analyses using the obtained SPET data, which were reconstructed for the assessment of the specific/non-specific DAT binding ratio in the basal ganglia. We then investigated the correlation between the severity scores of ADHD symptoms in children with ADHD assessed with ADHD rating scale-IV and the specific/non-specific DAT binding ratio in the basal ganglia. Drug-naive children with ADHD showed a significantly increased specific/non-specific DAT binding ratio in the basal ganglia compared with normal children. However, no significant correlation was found between the severity scores of ADHD symptoms in children with ADHD and the specific/non-specific DAT binding ratio in the basal ganglia. Our findings support the complex dysregulation of the dopaminergic neurotransmitter system in children with ADHD. (orig.)

  8. Dopamine transporter density in the basal ganglia assessed with [{sup 123}I]IPT SPET in children with attention deficit hyperactivity disorder

    Energy Technology Data Exchange (ETDEWEB)

    Cheon, Keun-Ah; Kim, Young-Kee; Namkoong, Kee; Kim, Chan-Hyung [Department of Psychiatry, College of Medicine, Yonsei University, Seoul (Korea); Ryu, Young Hoon; Lee, Jong Doo [Division of Nuclear Medicine, Department of Radiology, College of Medicine, Yonsei University, 146-92 Dogokdong, Gangnam-Gu, Seoul, 135-720 (Korea)

    2003-02-01

    Attention deficit hyperactivity disorder (ADHD) is a psychiatric disorder in childhood that is known to be associated with dopamine dysregulation. In this study, we investigated dopamine transporter (DAT) density in children with ADHD using iodine-123 labelled N-(3-iodopropen-2-yl)-2β-carbomethoxy-3β-(4-chlorophenyl) tropane ([{sup 123}I]IPT) single-photon emission tomography (SPET) and postulated that an alteration in DAT density in the basal ganglia is responsible for dopaminergic dysfunction in children with ADHD. Nine drug-naive children with ADHD and six normal children were included in the study. We performed brain SPET 2 h after the intravenous administration of [{sup 123}I]IPT and carried out both quantitative and qualitative analyses using the obtained SPET data, which were reconstructed for the assessment of the specific/non-specific DAT binding ratio in the basal ganglia. We then investigated the correlation between the severity scores of ADHD symptoms in children with ADHD assessed with ADHD rating scale-IV and the specific/non-specific DAT binding ratio in the basal ganglia. Drug-naive children with ADHD showed a significantly increased specific/non-specific DAT binding ratio in the basal ganglia compared with normal children. However, no significant correlation was found between the severity scores of ADHD symptoms in children with ADHD and the specific/non-specific DAT binding ratio in the basal ganglia. Our findings support the complex dysregulation of the dopaminergic neurotransmitter system in children with ADHD. (orig.)

  9. Parkinsonism and vigilance: alteration in neural oscillatory activity and phase-amplitude coupling in the basal ganglia and motor cortex.

    Science.gov (United States)

    Escobar Sanabria, David; Johnson, Luke A; Nebeck, Shane D; Zhang, Jianyu; Johnson, Matthew D; Baker, Kenneth B; Molnar, Gregory F; Vitek, Jerrold L

    2017-11-01

    Oscillatory neural activity in different frequency bands and phase-amplitude coupling (PAC) are hypothesized to be biomarkers of Parkinson's disease (PD) that could explain dysfunction in the motor circuit and be used for closed-loop deep brain stimulation (DBS). How these putative biomarkers change from the normal to the parkinsonian state across nodes in the motor circuit and within the same subject, however, remains unknown. In this study, we characterized how parkinsonism and vigilance altered oscillatory activity and PAC within the primary motor cortex (M1), subthalamic nucleus (STN), and globus pallidus (GP) in two nonhuman primates. Static and dynamic analyses of local field potential (LFP) recordings indicate that 1 ) after induction of parkinsonism using the neurotoxin MPTP, low-frequency power (8-30 Hz) increased in the STN and GP in both subjects, but increased in M1 in only one subject; 2 ) high-frequency power (~330 Hz) was present in the STN in both normal subjects but absent in the parkinsonian condition; 3 ) elevated PAC measurements emerged in the parkinsonian condition in both animals, but in different sites in each animal (M1 in one subject and GPe in the other); and 4 ) the state of vigilance significantly impacted how oscillatory activity and PAC were expressed in the motor circuit. These results support the hypothesis that changes in low- and high-frequency oscillatory activity and PAC are features of parkinsonian pathophysiology and provide evidence that closed-loop DBS systems based on these biomarkers may require subject-specific configurations as well as adaptation to changes in vigilance. NEW & NOTEWORTHY Chronically implanted electrodes were used to record neural activity across multiple nodes in the basal ganglia-thalamocortical circuit simultaneously in a nonhuman primate model of Parkinson's disease, enabling within-subject comparisons of electrophysiological biomarkers between normal and parkinsonian conditions and different

  10. Genetics Home Reference: biotin-thiamine-responsive basal ganglia disease

    Science.gov (United States)

    ... eye muscles (external ophthalmoplegia), difficulty chewing or swallowing (dysphagia), and slurred speech. Affected individuals may also experience ... Drug Therapy Genetic Counseling Palliative Care Surgery and Rehabilitation Related Information How are genetic conditions diagnosed? How ...

  11. Functional Connectivity of Insula, Basal Ganglia, and Prefrontal Executive Control Networks during Hypoglycemia in Type 1 Diabetes.

    Science.gov (United States)

    Bolo, Nicolas R; Musen, Gail; Simonson, Donald C; Nickerson, Lisa D; Flores, Veronica L; Siracusa, Tamar; Hager, Brandon; Lyoo, In Kyoon; Renshaw, Perry F; Jacobson, Alan M

    2015-08-05

    Human brain networks mediating interoceptive, behavioral, and cognitive aspects of glycemic control are not well studied. Using group independent component analysis with dual-regression approach of functional magnetic resonance imaging data, we examined the functional connectivity changes of large-scale resting state networks during sequential euglycemic-hypoglycemic clamp studies in patients with type 1 diabetes and nondiabetic controls and how these changes during hypoglycemia were related to symptoms of hypoglycemia awareness and to concurrent glycosylated hemoglobin (HbA1c) levels. During hypoglycemia, diabetic patients showed increased functional connectivity of the right anterior insula and the prefrontal cortex within the executive control network, which was associated with higher HbA1c. Controls showed decreased functional connectivity of the right anterior insula with the cerebellum/basal ganglia network and of temporal regions within the temporal pole network and increased functional connectivity in the default mode and sensorimotor networks. Functional connectivity reductions in the right basal ganglia were correlated with increases of self-reported hypoglycemic symptoms in controls but not in patients. Resting state networks that showed different group functional connectivity during hypoglycemia may be most sensitive to glycemic environment, and their connectivity patterns may have adapted to repeated glycemic excursions present in type 1 diabetes. Our results suggest that basal ganglia and insula mediation of interoceptive awareness during hypoglycemia is altered in type 1 diabetes. These changes could be neuroplastic adaptations to frequent hypoglycemic experiences. Functional connectivity changes in the insula and prefrontal cognitive networks could also reflect an adaptation to changes in brain metabolic pathways associated with chronic hyperglycemia. The major factor limiting improved glucose control in type 1 diabetes is the significant increase

  12. Resolving basal ganglia calcification in hereditary hypomagnesemia with secondary hypocalcemia due to a novel TRMP6 gene mutation

    Directory of Open Access Journals (Sweden)

    Abdelhadi M Habeb

    2012-01-01

    Full Text Available Hereditary hypomagnesemia with secondary hypocalcemia (HSH is a rare condi-tion caused by mutations in the Transient Receptor Potential Melastatin 6 (TRMP6 gene. Patients usually present during early infancy with symptomatic hypocalcemia; however, intracranial calcification has not been previously reported in HSH. We report on a three-month-old Saudi girl who presented with hypocalcemic convulsions and was initially treated as nutritional rickets. However, further biochemical analysis of blood and urine were suggestive of HSH. This diagnosis was confirmed by mutation analysis, which identified a novel homozygous frame shift mutation (ins 2999T of the TRMP6 gene. A computed tomography brain scan, done around the time of diagnosis, identified bilateral basal ganglia calcification (BGC. Her serum calcium and the BGC improved with magnesium replacement. BGC can be added as a new feature of HSH and the case highlights the importance of measuring serum Mg in patients with hypocalcemic convulsions, particularly in children of consanguineous parents.

  13. Alterations of the cerebellum and basal ganglia in bipolar disorder mood states detected by quantitative T1ρ mapping.

    Science.gov (United States)

    Johnson, Casey P; Christensen, Gary E; Fiedorowicz, Jess G; Mani, Merry; Shaffer, Joseph J; Magnotta, Vincent A; Wemmie, John A

    2018-01-07

    Quantitative mapping of T1 relaxation in the rotating frame (T1ρ) is a magnetic resonance imaging technique sensitive to pH and other cellular and microstructural factors, and is a potentially valuable tool for identifying brain alterations in bipolar disorder. Recently, this technique identified differences in the cerebellum and cerebral white matter of euthymic patients vs healthy controls that were consistent with reduced pH in these regions, suggesting an underlying metabolic abnormality. The current study built upon this prior work to investigate brain T1ρ differences across euthymic, depressed, and manic mood states of bipolar disorder. Forty participants with bipolar I disorder and 29 healthy control participants matched for age and gender were enrolled. Participants with bipolar disorder were imaged in one or more mood states, yielding 27, 12, and 13 imaging sessions in euthymic, depressed, and manic mood states, respectively. Three-dimensional, whole-brain anatomical images and T1ρ maps were acquired for all participants, enabling voxel-wise evaluation of T1ρ differences between bipolar mood state and healthy control groups. All three mood state groups had increased T1ρ relaxation times in the cerebellum compared to the healthy control group. Additionally, the depressed and manic groups had reduced T1ρ relaxation times in and around the basal ganglia compared to the control and euthymic groups. The study implicated the cerebellum and basal ganglia in the pathophysiology of bipolar disorder and its mood states, the roles of which are relatively unexplored. These findings motivate further investigation of the underlying cause of the abnormalities, and the potential role of altered metabolic activity in these regions. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  14. A patient with Moyamoya-like vessels after radiation therapy for a tumor in the basal ganglia

    Energy Technology Data Exchange (ETDEWEB)

    Ishiyama, Koichi; Tomura, Noriaki; Kato, Koki; Takahashi, Satoshi; Watarai, Jiro; Sasajima, Toshio; Mizoi, Kazuo [Akita Univ. (Japan). School of Medicine

    2001-10-01

    A patient with Moyamoya-like vessels after radiation therapy for treatment of a tumor in the basal ganglia is reported. He was diagnosed as Down syndrome at birth. He had a tumor in the left basal ganglionic region at 12 years of the age. The tumor increased in size at age 14. He underwent cerebral angiography, which did not show a stenosis nor occlusion of the internal carotid artery, anterior cerebral artery, nor the middle cerebral artery. He received radiation therapy with a total dose of 56 Gy. He presented a dressing apraxia at age 19. MRI showed cerebral infarction in the left temporo-occipital region. Right internal carotid angiography revealed a severe stenosis of the internal carotid artery and anterior cerebral artery as well as a severe stenosis of the middle cerebral artery on the right side. Moyamoya-like vessels were seen in the basal ganglionic region. Left internal carotid angiography also showed a stenosis of the internal carotid artery and anterior cerebral artery as well as a severe stenosis of the middle cerebral artery on the left side. Moyamoya-like vessels were seen in the basal ganglionic region. Leptomeningeal anastomose and transdural anastomose were bilaterally seen. These arterial occlusion and stenotic phenomenon corresponded to a previous radiation field. These Moyamoya-like vessels with arterial stenosis and occlusion were thought to be due to radiation-induced vasculopathy, because a previous cerebral angiography showed a normal caliber of cerebral arteries. This patient showed that patients with radiation therapy in their early childhood should be carefully observed considering the possibility of the phenomenon. (author)

  15. Differential effects of levodopa and apomorphine on neuronal population oscillations in the cortico-basal ganglia loop circuit in vivo in experimental parkinsonism.

    Science.gov (United States)

    Kühn, Johanna; Haumesser, Jens K; Beck, Maximilian H; Altschüler, Jennifer; Kühn, Andrea A; Nikulin, Vadim V; van Riesen, Christoph

    2017-12-01

    The current pharmacotherapy of Parkinson's disease (PD) is primarily based on two classes of drugs: dopamine precursors, namely levodopa, and dopamine receptor agonists, such as apomorphine. Although both types of agents exert their beneficial clinical effects on motor and non-motor symptoms in PD via dopamine receptors, clinical efficiency and side effects differ substantially between levodopa and apomorphine. Levodopa can provide a greater symptomatic relief than dopamine receptor agonists. However, because long-term levodopa use is associated with early debilitating motor fluctuations, dopamine receptor agonists are often recommended in younger patients. The pharmacodynamic basis of these profound differences is incompletely understood. It has been hypothesized that levodopa and dopamine receptor agonists may have diverging effects on beta and gamma oscillations that have been shown to be of importance for the pathophysiology of PD. Here, we used electrophysiological recordings in anesthetized dopamine-intact and dopamine-depleted rats to systemically compare the impact of levodopa or apomorphine on neuronal population oscillations in three nodes of the cortico-basal ganglia loop circuit. Our results showed that levodopa had a higher potency than apomorphine to suppress the abnormal beta oscillations often associated with bradykinesia while simultaneously enhancing the gamma oscillations often associated with increased movement. Our data suggests that the higher clinical efficacy of levodopa as well as some of its side effects, as e.g. dyskinesias may be based on its characteristic ability to modulate beta-/gamma-oscillation dynamics in the cortico-basal ganglia loop circuit. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Sodium Para-aminosalicylic Acid Protected Primary Cultured Basal Ganglia Neurons of Rat from Manganese-Induced Oxidative Impairment and Changes of Amino Acid Neurotransmitters.

    Science.gov (United States)

    Li, Shao-Jun; Li, Yong; Chen, Jing-Wen; Yuan, Zong-Xiang; Mo, Yu-Huan; Lu, Guo-Dong; Jiang, Yue-Ming; Ou, Chao-Yan; Wang, Fang; Huang, Xiao-Wei; Luo, Yi-Ni; Ou, Shi-Yan; Huang, Yan-Ni

    2016-04-01

    Manganese (Mn), an essential trace metal for protein synthesis and particularly neurotransmitter metabolism, preferentially accumulates in basal ganglia. However, excessive Mn accumulation may cause neurotoxicity referred to as manganism. Sodium para-aminosalicylic acid (PAS-Na) has been used to treat manganism with unclear molecular mechanisms. Thus, we aim to explore whether PAS-Na can inhibit Mn-induced neuronal injury in basal ganglia in vitro. We exposed basal ganglia neurons with 50 μM manganese chloride (MnCl2) for 24 h and then replaced with 50, 150, and 450 μM PAS-Na treatment for another 24 h. MnCl2 significantly decreased cell viability but increased leakage rate of lactate dehydrogenase and DNA damage (as shown by increasing percentage of DNA tail and Olive tail moment). Mechanically, Mn reduced glutathione peroxidase and catalase activity and interrupted amino acid neurotransmitter balance. However, PAS-Na treatment reversed the aforementioned Mn-induced toxic effects. Taken together, these results showed that PAS-Na could protect basal ganglia neurons from Mn-induced neurotoxicity.

  17. How does environmental enrichment reduce repetitive motor behaviors? Neuronal activation and dendritic morphology in the indirect basal ganglia pathway of a mouse model.

    Science.gov (United States)

    Bechard, Allison R; Cacodcar, Nadia; King, Michael A; Lewis, Mark H

    2016-02-15

    Repetitive motor behaviors are observed in many neurodevelopmental and neurological disorders (e.g., autism spectrum disorders, Tourette syndrome, fronto-temporal dementia). Despite their clinical importance, the neurobiology underlying these highly stereotyped, apparently functionless behaviors is poorly understood. Identification of mechanisms that mediate the development of repetitive behaviors will aid in the discovery of new therapeutic targets and treatment development. Using a deer mouse model, we have shown that decreased indirect basal ganglia pathway activity is associated with high levels of repetitive behavior. Environmental enrichment (EE) markedly attenuates the development of such aberrant behaviors in mice, although mechanisms driving this effect are unknown. We hypothesized that EE would reduce repetitive motor behaviors by increasing indirect basal ganglia pathway function. We assessed neuronal activation and dendritic spine density in basal ganglia of adult deer mice reared in EE and standard housing. Significant increases in neuronal activation and dendritic spine densities were observed only in the subthalamic nucleus (STN) and globus pallidus (GP), and only for those mice that exhibited an EE-induced decrease in repetitive motor behavior. As the STN and GP lie within the indirect pathway, these data suggest that EE-induced attenuation of repetitive motor behaviors is associated with increased functional activation of the indirect basal ganglia pathway. These results are consistent with our other findings highlighting the importance of the indirect pathway in mediating repetitive motor behaviors. Copyright © 2015 Elsevier B.V. All rights reserved.

  18. Singing can improve speech function in aphasics associated with intact right basal ganglia and preserve right temporal glucose metabolism: Implications for singing therapy indication.

    Science.gov (United States)

    Akanuma, Kyoko; Meguro, Kenichi; Satoh, Masayuki; Tashiro, Manabu; Itoh, Masatoshi

    2016-01-01

    Clinically, we know that some aphasic patients can sing well despite their speech disturbances. Herein, we report 10 patients with non-fluent aphasia, of which half of the patients improved their speech function after singing training. We studied ten patients with non-fluent aphasia complaining of difficulty finding words. All had lesions in the left basal ganglia or temporal lobe. They selected the melodies they knew well, but which they could not sing. We made a new lyric with a familiar melody using words they could not name. The singing training using these new lyrics was performed for 30 minutes once a week for 10 weeks. Before and after the training, their speech functions were assessed by language tests. At baseline, 6 of them received positron emission tomography to evaluate glucose metabolism. Five patients exhibited improvements after intervention; all but one exhibited intact right basal ganglia and left temporal lobes, but all exhibited left basal ganglia lesions. Among them, three subjects exhibited preserved glucose metabolism in the right temporal lobe. We considered that patients who exhibit intact right basal ganglia and left temporal lobes, together with preserved right hemispheric glucose metabolism, might be an indication of the effectiveness of singing therapy.

  19. Endothelial Proliferation and Increased Blood - Brain Barrier Permeability in the Basal Ganglia in a Rat Model of 3,4-Dihydrozyphenyl-L-Alanine-Induced Dyskinesia

    DEFF Research Database (Denmark)

    Westin, Jenny E.; Lindgren, Hanna S.; Gardi, Jonathan Eyal

    2006-01-01

    3,4-Dihydroxyphenyl-L-alanine (L-DOPA)-induced dyskinesia is associated with molecular and synaptic plasticity in the basal ganglia, but the occurrence of structural remodeling through cell genesis has not been explored. In this study, rats with 6-hydroxydopamine lesions received injections...

  20. Altered effective connectivity network of the basal ganglia in low-grade hepatic encephalopathy: a resting-state fMRI study with Granger causality analysis.

    Directory of Open Access Journals (Sweden)

    Rongfeng Qi

    Full Text Available BACKGROUND: The basal ganglia often show abnormal metabolism and intracranial hemodynamics in cirrhotic patients with hepatic encephalopathy (HE. Little is known about how the basal ganglia affect other brain system and is affected by other brain regions in HE. The purpose of this study was to investigate whether the effective connectivity network associated with the basal ganglia is disturbed in HE patients by using resting-state functional magnetic resonance imaging (rs-fMRI. METHODOLOGY/PRINCIPAL FINDINGS: Thirty five low-grade HE patients and thirty five age- and gender- matched healthy controls participated in the rs-fMRI scans. The effective connectivity networks associated with the globus pallidus, the primarily affected region within basal ganglia in HE, were characterized by using the Granger causality analysis and compared between HE patients and healthy controls. Pearson correlation analysis was performed between the abnormal effective connectivity and venous blood ammonia levels and neuropsychological performances of all HE patients. Compared with the healthy controls, patients with low-grade HE demonstrated mutually decreased influence between the globus pallidus and the anterior cingulate cortex (ACC, cuneus, bi-directionally increased influence between the globus pallidus and the precuneus, and either decreased or increased influence from and to the globus pallidus in many other frontal, temporal, parietal gyri, and cerebellum. Pearson correlation analyses revealed that the blood ammonia levels in HE patients negatively correlated with effective connectivity from the globus pallidus to ACC, and positively correlated with that from the globus pallidus to precuneus; and the number connectivity test scores in patients negatively correlated with the effective connectivity from the globus pallidus to ACC, and from superior frontal gyrus to globus pallidus. CONCLUSIONS/SIGNIFICANCE: Low-grade HE patients had disrupted effective

  1. Raclopride or high-frequency stimulation of the subthalamic nucleus stops cocaine-induced motor stereotypy and restores related alterations in prefrontal basal ganglia circuits.

    Science.gov (United States)

    Aliane, Verena; Pérez, Sylvie; Deniau, Jean-Michel; Kemel, Marie-Louise

    2012-11-01

    Motor stereotypy is a key symptom of various neurological or neuropsychiatric disorders. Neuroleptics or the promising treatment using deep brain stimulation stops stereotypies but the mechanisms underlying their actions are unclear. In rat, motor stereotypies are linked to an imbalance between prefrontal and sensorimotor cortico-basal ganglia circuits. Indeed, cortico-nigral transmission was reduced in the prefrontal but not sensorimotor basal ganglia circuits and dopamine and acetylcholine release was altered in the prefrontal but not sensorimotor territory of the dorsal striatum. Furthermore, cholinergic transmission in the prefrontal territory of the dorsal striatum plays a crucial role in the arrest of motor stereotypy. Here we found that, as previously observed for raclopride, high-frequency stimulation of the subthalamic nucleus (HFS STN) rapidly stopped cocaine-induced motor stereotypies in rat. Importantly, raclopride and HFS STN exerted a strong effect on cocaine-induced alterations in prefrontal basal ganglia circuits. Raclopride restored the cholinergic transmission in the prefrontal territory of the dorsal striatum and the cortico-nigral information transmissions in the prefrontal basal ganglia circuits. HFS STN also restored the N-methyl-d-aspartic-acid-evoked release of acetylcholine and dopamine in the prefrontal territory of the dorsal striatum. However, in contrast to raclopride, HFS STN did not restore the cortico-substantia nigra pars reticulata transmissions but exerted strong inhibitory and excitatory effects on neuronal activity in the prefrontal subdivision of the substantia nigra pars reticulata. Thus, both raclopride and HFS STN stop cocaine-induced motor stereotypy, but exert different effects on the related alterations in the prefrontal basal ganglia circuits. © 2012 The Authors. European Journal of Neuroscience © 2012 Federation of European Neuroscience Societies and Blackwell Publishing Ltd.

  2. Transtorno obsessivo-compulsivo e os gânglios da base Obsessive-compulsive disorder and the basal ganglia

    Directory of Open Access Journals (Sweden)

    Eurípedes Constantino Miguel Filho

    1995-12-01

    Full Text Available O transtorno obsessivo compulsivo (TOC, caracterizado por obsessões e compulsões, foi descrito com frequência aumentada em várias doenças que acometem primariamente of gânglios da base sugerindo que estas estruturas também estivessem acometidas no TOC. Os gânglios da base, que no passado se acreditava estarem implicados apenas no comportamento motor, são, na verdade, importantes em inúmeras outras funções psíquicas como o processamento de vivências cognitivas. Estudos recentes utilizando imagem de ressonância magnética mostraram tendência a diminuição do núcleo caudado em pacientes com TOC. De forma coerente, estudos com neuroimagem funcional, sugerem a implicação de um circuito cerebral envolvendo o córtex órbito-frontal, o giro cíngulo, o núcleo caudado e o tálamo na patofisiologia do TOC. Entre as diversas hipóteses formuladas a partir desses achados, especula-se que um déficit no funcionamento do núcleo caudado levaria a uma filtragem inadequada de preocupações que então estimulariam o córtex órbito-frontal a desencadear ações adaptativas: as compulsões.Obsessive-compulsive disorder (OCD, characterized by obsessions and compulsions, was described as more frequent in patients with primary lesions of the basal ganglia suggesting that these brain structures may be also altered in OCD. The basal ganglia, that were considered important only for the motor control, are known now as crucial for many other mental functions as processing of cognitive experience. Recent studies using magnetic resonance image have found a tendency for smaller caudate nucleus in patients with OCD. Consistently, studies using functional neuroimaging suggest implication of a neurocircuit that includes the orbitalfrontal cortex, the cingulate cortex, caudate nucleus and thalamus in the pathophysiology of OCD. Among several hypotheses formulated to explain these findings, some authors speculated that a deficit of the caudate nucleus

  3. Expression Changes in Lactate and Glucose Metabolism and Associated Transporters in Basal Ganglia following Hypoxic-Ischemic Reperfusion Injury in Piglets.

    Science.gov (United States)

    Zheng, Y; Wang, X-M

    2018-01-11

    The neonatal brain has active energy metabolism, and glucose oxidation is the major energy source of brain tissue. Lactate is produced by astrocytes and released to neurons. In the central nervous system, lactate is transported between neurons and astrocytes via the astrocyte-neuron lactate shuttle. The aim of this study was to investigate the regulatory mechanisms of energy metabolism in neurons and astrocytes in the basal ganglia of a neonatal hypoxic-ischemic brain injury piglet model. A total of 35 healthy piglets (3-5 days of age; 1.0-1.5 kg) were assigned to a control group ( n = 5) or a hypoxic-ischemic model group ( n = 30). The hypoxic-ischemic model group was further divided into 6 groups according to the 1 H-MR spectroscopy and PET/CT scan times after hypoxia-ischemia (0-2, 2-6, 6-12, 12-24, 24-48, and 48-72 hours; n = 5/group). 1 H-MR spectroscopy data were processed with LCModel software. Maximum standard uptake values refer to the maximum standard uptake values for glucose (or FDG). The maximum standard uptake values of the basal ganglia-to-occipital cortex ratio were analyzed. The expression levels of glucose transporters and monocarboxylate transporters were detected by immunohistochemical analysis. Lactate levels decreased after an initial increase, with the maximal level occurring around 2-6 hours following hypoxia-ischemia. After hypoxia-ischemia, the maximum standard uptake values of the basal ganglia and basal ganglia/occipital cortex initially increased then decreased, with the maximum occurring at approximately 6-12 hours. The lactate and glucose uptake (basal ganglia/occipital cortex maximum standard uptake values) levels were positively correlated. The expression levels of glucose transporter-1 and glucose transporter-3 were positively correlated with the basal ganglia/occipital cortex. The expression levels of monocarboxylic acid transporter-2 and monocarboxylic acid transporter-4 were positively correlated with lactate content. The

  4. Aberrant white matter networks mediate cognitive impairment in patients with silent lacunar infarcts in basal ganglia territory.

    Science.gov (United States)

    Tang, Jinfu; Zhong, Suyu; Chen, Yaojing; Chen, Kewei; Zhang, Junying; Gong, Gaolang; Fleisher, Adam S; He, Yong; Zhang, Zhanjun

    2015-09-01

    Silent lacunar infarcts, which are present in over 20% of healthy elderly individuals, are associated with subtle deficits in cognitive functions. However, it remains largely unclear how these silent brain infarcts lead to cognitive deficits and even dementia. Here, we used diffusion tensor imaging tractography and graph theory to examine the topological organization of white matter networks in 27 patients with silent lacunar infarcts in the basal ganglia territory and 30 healthy controls. A whole-brain white matter network was constructed for each subject, where the graph nodes represented brain regions and the edges represented interregional white matter tracts. Compared with the controls, the patients exhibited a significant reduction in local efficiency and global efficiency. In addition, a total of eighteen brain regions showed significantly reduced nodal efficiency in patients. Intriguingly, nodal efficiency-behavior associations were significantly different between the two groups. The present findings provide new aspects into our understanding of silent infarcts that even small lesions in subcortical brain regions may affect large-scale cortical white matter network, as such may be the link between subcortical silent infarcts and the associated cognitive impairments. Our findings highlight the need for network-level neuroimaging assessment and more medical care for individuals with silent subcortical infarcts.

  5. Age-related decreases in the concentration of Met- and Leu-enkephalin and neurotensin in the basal ganglia or rats

    International Nuclear Information System (INIS)

    Ceballos, M.L. de; Boyce, S.; Taylor, M.; Jenner, P.; Marsden, C.D.

    1987-01-01

    Previous studies using radioimmunoassay procedures have failed to show age-related changes in the concentration of Met-and Leu-enkephalin or neurotensin in rat basal ganglia. In contrast, using a combined high-pressure liquid chromatography (HLPC)- radioimmunoassay (RIA) technique we now report considerable decreases in the levels of these neuropeptides in areas of basal ganglia of 22 months-old compared to 3 months-old male Wistar rats. The concentration of Met-enkephalin was greatly reduced in the striatum and nucleus accumbens, but not in substantia nigra, of old compared to young animals. There was a similarly large decrease in Leu-enkephalin content in striatum of old rats with less marked decreases occurring in both the nucleus accumbens and substantia nigra. Neurotensin levels in the striatum and substantia nigra were greatly reduced in old rats, with a less marked decrease in the nucleus accumbens

  6. Effect of teriflunomide on cortex-basal ganglia-thalamus (CxBGTh circuit glutamatergic dysregulation in the Theiler's Murine Encephalomyelitis Virus mouse model of multiple sclerosis.

    Directory of Open Access Journals (Sweden)

    Claire M Modica

    Full Text Available Pathology of gray matter is associated with development of physical and cognitive disability in patients with multiple sclerosis. In particular, glutamatergic dysregulation in the cortex-basal ganglia-thalamus (CxBGTh circuit could be associated with decline in these behaviors.To investigate the effect of an immunomodulatory therapy (teriflunomide, Aubagio® on changes of the CxBGTh loop in the Theiler's Murine Encephalomyelitis Virus, (TMEV mouse model of MS.Forty-eight (48 mice were infected with TMEV, treated with teriflunomide (24 or control vehicle (24 and followed for 39 weeks. Mice were examined with MRS and volumetric MRI scans (0, 8, 26, and 39 weeks in the cortex, basal ganglia and thalamus, using a 9.4T scanner, and with behavioral tests (0, 4, 8, 12, 17, 26, and 39 weeks. Within conditions, MRI measures were compared between two time points by paired samples t-test and across multiple time points by repeated measures ANOVA (rmANOVA, and between conditions by independent samples t-test and rmANOVA, respectively. Data were considered as significant at the p<0.01 level and as a trend at p<0.05 level.In the thalamus, the teriflunomide arm exhibited trends toward decreased glutamate levels at 8 and 26 weeks compared to the control arm (p = 0.039 and p = 0.026, while the control arm exhibited a trend toward increased glutamate between 0 to 8 weeks (p = 0.045. In the basal ganglia, the teriflunomide arm exhibited a trend toward decreased glutamate earlier than the control arm, from 0 to 8 weeks (p = 0.011, resulting in decreased glutamate compared to the control arm at 8 weeks (p = 0.016.Teriflunomide may reduce possible excitotoxicity in the thalamus and basal ganglia by lowering glutamate levels.

  7. Correlation of iron deposition and change of gliocyte metabolism in the basal ganglia region evaluated using magnetic resonance imaging techniques: an in vivo study

    OpenAIRE

    Liu, Haodi; Wang, Xiaoming

    2016-01-01

    Introduction We assessed the correlation between iron deposition and the change of gliocyte metabolism in healthy subjects? basal ganglia region, by using 3D-enhanced susceptibility weighted angiography (ESWAN) and proton magnetic resonance spectroscopy (1H-MRS). Material and methods Seventy-seven healthy volunteers (39 female and 38 male subjects; age range: 24?82 years old) were enrolled in the experiment including ESWAN and proton MRS sequences, consent for which was provided by themselves...

  8. Neuromodulatory Adaptive Combination of Correlation-based Learning in Cerebellum and Reward-based Learning in Basal Ganglia for Goal-directed Behavior Control

    Directory of Open Access Journals (Sweden)

    Sakyasingha eDasgupta

    2014-10-01

    Full Text Available Goal-directed decision making in biological systems is broadly based on associations between conditional and unconditional stimuli. This can be further classified as classical conditioning (correlation-based learning and operant conditioning (reward-based learning. A number of computational and experimental studies have well established the role of the basal ganglia in reward-based learning, where as the cerebellum plays an important role in developing specific conditioned responses. Although viewed as distinct learning systems, recent animal experiments point towards their complementary role in behavioral learning, and also show the existence of substantial two-way communication between these two brain structures. Based on this notion of co-operative learning, in this paper we hypothesize that the basal ganglia and cerebellar learning systems work in parallel and interact with each other. We envision that such an interaction is influenced by reward modulated heterosynaptic plasticity (RMHP rule at the thalamus, guiding the overall goal directed behavior. Using a recurrent neural network actor-critic model of the basal ganglia and a feed-forward correlation-based learning model of the cerebellum, we demonstrate that the RMHP rule can effectively balance the outcomes of the two learning systems. This is tested using simulated environments of increasing complexity with a four-wheeled robot in a foraging task in both static and dynamic configurations. Although modeled with a simplified level of biological abstraction, we clearly demonstrate that such a RMHP induced combinatorial learning mechanism, leads to stabler and faster learning of goal-directed behaviors, in comparison to the individual systems. Thus in this paper we provide a computational model for adaptive combination of the basal ganglia and cerebellum learning systems by way of neuromodulated plasticity for goal-directed decision making in biological and bio-mimetic organisms.

  9. Neuromodulatory adaptive combination of correlation-based learning in cerebellum and reward-based learning in basal ganglia for goal-directed behavior control.

    Science.gov (United States)

    Dasgupta, Sakyasingha; Wörgötter, Florentin; Manoonpong, Poramate

    2014-01-01

    Goal-directed decision making in biological systems is broadly based on associations between conditional and unconditional stimuli. This can be further classified as classical conditioning (correlation-based learning) and operant conditioning (reward-based learning). A number of computational and experimental studies have well established the role of the basal ganglia in reward-based learning, where as the cerebellum plays an important role in developing specific conditioned responses. Although viewed as distinct learning systems, recent animal experiments point toward their complementary role in behavioral learning, and also show the existence of substantial two-way communication between these two brain structures. Based on this notion of co-operative learning, in this paper we hypothesize that the basal ganglia and cerebellar learning systems work in parallel and interact with each other. We envision that such an interaction is influenced by reward modulated heterosynaptic plasticity (RMHP) rule at the thalamus, guiding the overall goal directed behavior. Using a recurrent neural network actor-critic model of the basal ganglia and a feed-forward correlation-based learning model of the cerebellum, we demonstrate that the RMHP rule can effectively balance the outcomes of the two learning systems. This is tested using simulated environments of increasing complexity with a four-wheeled robot in a foraging task in both static and dynamic configurations. Although modeled with a simplified level of biological abstraction, we clearly demonstrate that such a RMHP induced combinatorial learning mechanism, leads to stabler and faster learning of goal-directed behaviors, in comparison to the individual systems. Thus, in this paper we provide a computational model for adaptive combination of the basal ganglia and cerebellum learning systems by way of neuromodulated plasticity for goal-directed decision making in biological and bio-mimetic organisms.

  10. Common features of neural activity during singing and sleep periods in a basal ganglia nucleus critical for vocal learning in a juvenile songbird.

    Science.gov (United States)

    Yanagihara, Shin; Hessler, Neal A

    2011-01-01

    Reactivations of waking experiences during sleep have been considered fundamental neural processes for memory consolidation. In songbirds, evidence suggests the importance of sleep-related neuronal activity in song system motor pathway nuclei for both juvenile vocal learning and maintenance of adult song. Like those in singing motor nuclei, neurons in the basal ganglia nucleus Area X, part of the basal ganglia-thalamocortical circuit essential for vocal plasticity, exhibit singing-related activity. It is unclear, however, whether Area X neurons show any distinctive spiking activity during sleep similar to that during singing. Here we demonstrate that, during sleep, Area X pallidal neurons exhibit phasic spiking activity, which shares some firing properties with activity during singing. Shorter interspike intervals that almost exclusively occurred during singing in awake periods were also observed during sleep. The level of firing variability was consistently higher during singing and sleep than during awake non-singing states. Moreover, deceleration of firing rate, which is considered to be an important firing property for transmitting signals from Area X to the thalamic nucleus DLM, was observed mainly during sleep as well as during singing. These results suggest that songbird basal ganglia circuitry may be involved in the off-line processing potentially critical for vocal learning during sensorimotor learning phase.

  11. Common features of neural activity during singing and sleep periods in a basal ganglia nucleus critical for vocal learning in a juvenile songbird.

    Directory of Open Access Journals (Sweden)

    Shin Yanagihara

    Full Text Available Reactivations of waking experiences during sleep have been considered fundamental neural processes for memory consolidation. In songbirds, evidence suggests the importance of sleep-related neuronal activity in song system motor pathway nuclei for both juvenile vocal learning and maintenance of adult song. Like those in singing motor nuclei, neurons in the basal ganglia nucleus Area X, part of the basal ganglia-thalamocortical circuit essential for vocal plasticity, exhibit singing-related activity. It is unclear, however, whether Area X neurons show any distinctive spiking activity during sleep similar to that during singing. Here we demonstrate that, during sleep, Area X pallidal neurons exhibit phasic spiking activity, which shares some firing properties with activity during singing. Shorter interspike intervals that almost exclusively occurred during singing in awake periods were also observed during sleep. The level of firing variability was consistently higher during singing and sleep than during awake non-singing states. Moreover, deceleration of firing rate, which is considered to be an important firing property for transmitting signals from Area X to the thalamic nucleus DLM, was observed mainly during sleep as well as during singing. These results suggest that songbird basal ganglia circuitry may be involved in the off-line processing potentially critical for vocal learning during sensorimotor learning phase.

  12. Impaired causal awareness and associated cortical-basal ganglia structural changes in youth psychiatric disorders.

    Science.gov (United States)

    Griffiths, Kristi R; Lagopoulos, Jim; Hermens, Daniel F; Lee, Rico S C; Guastella, Adam J; Hickie, Ian B; Balleine, Bernard W

    2016-01-01

    Cognitive impairments contribute significantly to disease burden in young individuals presenting with major psychiatric disorders. The capacity to encode the consequences of one's actions may be of particular importance for real-world functioning due to its fundamental role in goal-directed behavior. Here, we investigated a dimensional measure of causal awareness during a probabilistic learning task in 92 young individuals with an admixture of major mood and psychotic illnesses, at early and more established stages. Using automated gray matter segmentation of T1-weighted images, we estimated the volume and shapes of major subcortical structures and investigated their association with causal awareness. The low causal awareness (LCA) group (n = 35) reported increased social disability (p = .004) and reduced right pallidal size, specifically within the dorsolateral surfaces (p = .02), relative to the unimpaired high causal awareness (HCA) patients (n = 57). In early-stage illness, LCA had a smaller right thalamus (p = .002) relative to HCA. Exploratory investigations suggested that in developed psychotic syndromes, causal awareness was correlated with left hippocampal size (p = .006) whereas, in more persistent affective disorders, causal awareness was correlated with left amygdala size (p = .013), specifically within the anterior aspect. Low causal awareness occurs across diagnoses and stages of illness and is associated with poor functional outcomes. Our results suggest that there may be shared neural underpinnings of its dysfunction in the early course of mood and psychotic disorders, however in more established illness, there is greater neurobiological divergence in causal awareness correlates between diagnoses.

  13. Optogenetic stimulation in a computational model of the basal ganglia biases action selection and reward prediction error.

    Directory of Open Access Journals (Sweden)

    Pierre Berthet

    Full Text Available Optogenetic stimulation of specific types of medium spiny neurons (MSNs in the striatum has been shown to bias the selection of mice in a two choices task. This shift is dependent on the localisation and on the intensity of the stimulation but also on the recent reward history. We have implemented a way to simulate this increased activity produced by the optical flash in our computational model of the basal ganglia (BG. This abstract model features the direct and indirect pathways commonly described in biology, and a reward prediction pathway (RP. The framework is similar to Actor-Critic methods and to the ventral/dorsal distinction in the striatum. We thus investigated the impact on the selection caused by an added stimulation in each of the three pathways. We were able to reproduce in our model the bias in action selection observed in mice. Our results also showed that biasing the reward prediction is sufficient to create a modification in the action selection. However, we had to increase the percentage of trials with stimulation relative to that in experiments in order to impact the selection. We found that increasing only the reward prediction had a different effect if the stimulation in RP was action dependent (only for a specific action or not. We further looked at the evolution of the change in the weights depending on the stage of learning within a block. A bias in RP impacts the plasticity differently depending on that stage but also on the outcome. It remains to experimentally test how the dopaminergic neurons are affected by specific stimulations of neurons in the striatum and to relate data to predictions of our model.

  14. Emergent structured transition from variation to repetition in a biologically-plausible model of learning in basal ganglia.

    Directory of Open Access Journals (Sweden)

    Ashvin eShah

    2014-02-01

    Full Text Available Often, when animals encounter an unexpected sensory event, they transition from executing a variety of movements to repeating the movement(s that may have caused the event. According to a recent theory of action discovery (Redgrave and Gurney 2006, repetition allows the animal to represent those movements, and the outcome, as an action for later recruitment. The transition from variation to repetition often follows a non-random, structured, pattern. While the structure of the pattern can be explained by sophisticated cognitive mechanisms, simpler mechanisms based on dopaminergic modulation of basal ganglia (BG activity are thought to underlie action discovery (Redgrave and Gurney 2006. In this paper we ask the question: can simple BG-mediated mechanisms account for a structured transition from variation to repetition, or are more sophisticated cognitive mechanisms always necessary?To address this question, we present a computational model of BG-mediated biasing of behavior. In our model, unlike most other models of BG function, the BG biases behaviour through modulation of cortical response to excitation; many possible movements are represented by the cortical area; and excitation to the cortical area is topographically-organized. We subject the model to simple reaching tasks, inspired by behavioral studies, in which a location to which to reach must be selected. Locations within a target area elicit a reinforcement signal. A structured transition from variation to repetition emerges from simple BG-mediated biasing of cortical response to excitation. We show how the structured pattern influences behavior in simple and complicated tasks. We also present analyses that describe the structured transition from variation to repetition due to BG-mediated biasing and from biasing that would be expected from a type of cognitive biasing, allowing us to compare behaviour resulting from these types of biasing and make connections with future behavioural

  15. Thalamic haemorrhage vs internal capsule-basal ganglia haemorrhage: clinical profile and predictors of in-hospital mortality

    Directory of Open Access Journals (Sweden)

    García-Eroles Luis

    2007-10-01

    Full Text Available Abstract Background There is a paucity of clinical studies focused specifically on intracerebral haemorrhages of subcortical topography, a subject matter of interest to clinicians involved in stroke management. This single centre, retrospective study was conducted with the following objectives: a to describe the aetiological, clinical and prognostic characteristics of patients with thalamic haemorrhage as compared with that of patients with internal capsule-basal ganglia haemorrhage, and b to identify predictors of in-hospital mortality in patients with thalamic haemorrhage. Methods Forty-seven patients with thalamic haemorrhage were included in the "Sagrat Cor Hospital of Barcelona Stroke Registry" during a period of 17 years. Data from stroke patients are entered in the stroke registry following a standardized protocol with 161 items regarding demographics, risk factors, clinical features, laboratory and neuroimaging data, complications and outcome. The region of the intracranial haemorrhage was identified on computerized tomographic (CT scans and/or magnetic resonance imaging (MRI of the brain. Results Thalamic haemorrhage accounted for 1.4% of all cases of stroke (n = 3420 and 13% of intracerebral haemorrhage (n = 364. Hypertension (53.2%, vascular malformations (6.4%, haematological conditions (4.3% and anticoagulation (2.1% were the main causes of thalamic haemorrhage. In-hospital mortality was 19% (n = 9. Sensory deficit, speech disturbances and lacunar syndrome were significantly associated with thalamic haemorrhage, whereas altered consciousness (odds ratio [OR] = 39.56, intraventricular involvement (OR = 24.74 and age (OR = 1.23, were independent predictors of in-hospital mortality. Conclusion One in 8 patients with acute intracerebral haemorrhage had a thalamic hematoma. Altered consciousness, intraventricular extension of the hematoma and advanced age were determinants of a poor early outcome.

  16. Striatal dopamine ramping may indicate flexible reinforcement learning with forgetting in the cortico-basal ganglia circuits.

    Science.gov (United States)

    Morita, Kenji; Kato, Ayaka

    2014-01-01

    It has been suggested that the midbrain dopamine (DA) neurons, receiving inputs from the cortico-basal ganglia (CBG) circuits and the brainstem, compute reward prediction error (RPE), the difference between reward obtained or expected to be obtained and reward that had been expected to be obtained. These reward expectations are suggested to be stored in the CBG synapses and updated according to RPE through synaptic plasticity, which is induced by released DA. These together constitute the "DA=RPE" hypothesis, which describes the mutual interaction between DA and the CBG circuits and serves as the primary working hypothesis in studying reward learning and value-based decision-making. However, recent work has revealed a new type of DA signal that appears not to represent RPE. Specifically, it has been found in a reward-associated maze task that striatal DA concentration primarily shows a gradual increase toward the goal. We explored whether such ramping DA could be explained by extending the "DA=RPE" hypothesis by taking into account biological properties of the CBG circuits. In particular, we examined effects of possible time-dependent decay of DA-dependent plastic changes of synaptic strengths by incorporating decay of learned values into the RPE-based reinforcement learning model and simulating reward learning tasks. We then found that incorporation of such a decay dramatically changes the model's behavior, causing gradual ramping of RPE. Moreover, we further incorporated magnitude-dependence of the rate of decay, which could potentially be in accord with some past observations, and found that near-sigmoidal ramping of RPE, resembling the observed DA ramping, could then occur. Given that synaptic decay can be useful for flexibly reversing and updating the learned reward associations, especially in case the baseline DA is low and encoding of negative RPE by DA is limited, the observed DA ramping would be indicative of the operation of such flexible reward learning.

  17. Selected Gray Matter Volumes and Gender but Not Basal Ganglia nor Cerebellum Gyri Discriminate Left Versus Right Cerebral Hemispheres: Multivariate Analyses in human Brains at 3T.

    Science.gov (United States)

    Roldan-Valadez, Ernesto; Suarez-May, Marcela A; Favila, Rafael; Aguilar-Castañeda, Erika; Rios, Camilo

    2015-07-01

    Interest in the lateralization of the human brain is evident through a multidisciplinary number of scientific studies. Understanding volumetric brain asymmetries allows the distinction between normal development stages and behavior, as well as brain diseases. We aimed to evaluate volumetric asymmetries in order to select the best gyri able to classify right- versus left cerebral hemispheres. A cross-sectional study performed in 47 right-handed young-adults healthy volunteers. SPM-based software performed brain segmentation, automatic labeling and volumetric analyses for 54 regions involving the cerebral lobes, basal ganglia and cerebellum from each cerebral hemisphere. Multivariate discriminant analysis (DA) allowed the assembling of a predictive model. DA revealed one discriminant function that significantly differentiated left vs. right cerebral hemispheres: Wilks' λ = 0.008, χ(2) (9) = 238.837, P vision and language; our findings favored the concept that lateralization has been evolutionary favored by mental processes increasing cognitive efficiency and brain capacity. © 2015 Wiley Periodicals, Inc.

  18. MR spectroscopy-based brain metabolite profiling in propionic acidaemia: metabolic changes in the basal ganglia during acute decompensation and effect of liver transplantation

    Directory of Open Access Journals (Sweden)

    McKiernan Patrick J

    2011-05-01

    Full Text Available Abstract Background Propionic acidaemia (PA results from deficiency of Propionyl CoA carboxylase, the commonest form presenting in the neonatal period. Despite best current management, PA is associated with severe neurological sequelae, in particular movement disorders resulting from basal ganglia infarction, although the pathogenesis remains poorly understood. The role of liver transplantation remains controversial but may confer some neuro-protection. The present study utilises quantitative magnetic resonance spectroscopy (MRS to investigate brain metabolite alterations in propionic acidaemia during metabolic stability and acute encephalopathic episodes. Methods Quantitative MRS was used to evaluate brain metabolites in eight children with neonatal onset propionic acidaemia, with six elective studies acquired during metabolic stability and five studies during acute encephalopathic episodes. MRS studies were acquired concurrently with clinically indicated MR imaging studies at 1.5 Tesla. LCModel software was used to provide metabolite quantification. Comparison was made with a dataset of MRS metabolite concentrations from a cohort of children with normal appearing MR imaging. Results MRI findings confirm the vulnerability of basal ganglia to infarction during acute encephalopathy. We identified statistically significant decreases in basal ganglia glutamate+glutamine and N-Acetylaspartate, and increase in lactate, during encephalopathic episodes. In white matter lactate was significantly elevated but other metabolites not significantly altered. Metabolite data from two children who had received liver transplantation were not significantly different from the comparator group. Conclusions The metabolite alterations seen in propionic acidaemia in the basal ganglia during acute encephalopathy reflect loss of viable neurons, and a switch to anaerobic respiration. The decrease in glutamine + glutamate supports the hypothesis that they are consumed to

  19. Safety and Efficacy Study of VY-AADC01 for Advanced Parkinson's Disease

    Science.gov (United States)

    2018-02-27

    Idiopathic Parkinson's Disease; Parkinson's Disease; Basal Ganglia Disease; Brain Diseases; Central Nervous System Diseases; Movement Disorders; Nervous System Diseases; Neurodegenerative Diseases; Parkinsonian Disorders

  20. No change of dopamine transporter density in basal ganglia after risperidone treatment in drug-naive children with Tourette's disorder

    Energy Technology Data Exchange (ETDEWEB)

    Ryu, W. K.; Ryu, Y. H.; Yoon, M. J.; Chun, K. A.; Lee, J. D. [College of Medicine, Univ. of Yonsei, Seoul (Korea, Republic of); Zee, D. Y. [Univ. of Inhwa, Incheon (Korea, Republic of); Choi, T. H. [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

    2003-07-01

    Tourette's disorder (TD), which is characterized by multiple waxing and waning motor tics and one or more vocal tics, is known to be associated with abnormalities in the dopaminergic system. To testify our hypothesis that risperidone would improve tic symptoms of TD patients through the change of the dopaminergic system, we measured the DAT densities between drug-naive children with TD and normal children investigated the DAT density before and after treatment with risperidone in drug-naive children with TD, using lodine-123 labelled N-(3-iodopropen-2-yl)-2beta-carbomethoxy-3beta-(4-chlorophenyl) tropane(I-123 IPT) single photon emission computed tomography (SPECT). I-123 IPT SPECT imaging and Yale Global Tic Severity Scale-Korean version (YGTSS-K) for assessing the tic symptom severity were carried out before and after treatment with risperidone for 8 weeks in eight drug-naive children with TD. Eight normal children also underwent SPECT imaging 2 hours after an intravenous administration of I-123 IPT and carried out both quantitative and qualitative analyses using the obtained SPECT data, which were reconstructed for the assessment of the specific/non-specific DAT binding ratio in the basal ganglia. The drug-naive children with TD had a significantly greater increase in the specific/nonspecific DAT binding ratio of both basal ganglia compared with the normal children. However, no significant difference in the specific/nonspecific DAT binding ratio of the basal ganglia before and after treatment with riperidone in children with TD was not found, although tic symptoms were significantly improved with risperidone. These findings suggest that DAT densities are directly associated with the pathophysiology of TD, however, that the effect of risperidone on tic symptoms in children with TD is not attributed to the change of dopaminergic system.

  1. An infant who had chorea-athetotic movement and psychomotor deterioration associated with the low density area in the bilateral cerebral basal ganglia on CT

    International Nuclear Information System (INIS)

    Tojo, Megumu; Matsui, Akira; Sakuragawa, Norio; Hirayama, Yoshito; Arima, Masataka

    1984-01-01

    A 6-year-old girl with convulsive tetraplegia and chorea-athetotic movement was reported. Since the age of one year, psychomotor retardation had begun to occur and CT showed a low density area in the putamen. At the age of 3 years and 6 months, psychomotor deterioration occurred subsequently to varicella. An abnormality in carbohydrate metabolism was suspected because of a slightly increased lactic acid and pyruvic acid. Because CT showed a low density area in the cerebral basal ganglia, juvenile Lee's encephalopathy and striatal necrosis remained to be ruled out. (Namekawa, K.)

  2. Deep frontal and periventricular age related white matter changes but not basal ganglia and infratentorial hyperintensities are associated with falls: cross sectional results from the LADIS study

    DEFF Research Database (Denmark)

    Blahak, C; Baezner, H; Pantoni, L

    2009-01-01

    BACKGROUND: Global age related white matter changes (ARWMC) are associated with progressive gait disturbances and falls, hypothesised to result from interruptions of cortico-subcortical circuits controlling balance, posture and locomotion. METHODS: The location of ARWMC in a large cohort of elderly...... scale, in multivariate analysis, periventricular (p = 0.006) and frontal deep (p = 0.033) ARWMC were independently associated with falls. Furthermore, logistic regression identified frontal deep (p = 0.003) ARWMC, but not basal ganglia and infratentorial hyperintensities, as significantly associated...

  3. Inter regional correlations of glucose metabolism between the basal ganglia and different cortical areas: an ultra-high resolution PET/MRI fusion study using 18F-FDG

    International Nuclear Information System (INIS)

    Kim, J.H.; Son, Y.D.; Kim, H.K.; Oh, C.H.; Kim, J.M.; Kim, Y.B.; Lee, C.

    2018-01-01

    Basal ganglia have complex functional connections with the cerebral cortex and are involved in motor control, executive functions of the forebrain, such as the planning of movement, and cognitive behaviors based on their connections. The aim of this study was to provide detailed functional correlation patterns between the basal ganglia and cerebral cortex by conducting an inter regional correlation analysis of the 18 F-fluorodeoxyglucose ( 18 F-FDG) positron emission tomography (PET) data based on precise structural information. Fifteen participants were scanned with 7-Tesla magnetic resonance imaging (MRI) and high resolution research tomography (HRRT)-PET fusion system using 18 F-FDG. For detailed inter regional correlation analysis, 24 subregions of the basal ganglia including pre-commissural dorsal caudate, post-commissural caudate, pre-commissural dorsal putamen, post-commissural putamen, internal globus pallidus, and external globus pallidus and 80 cerebral regions were selected as regions of interest on the MRI image and their glucose metabolism were calculated from the PET images. Pearson's product-moment correlation analysis was conducted for the inter regional correlation analysis of the basal ganglia. Functional correlation patterns between the basal ganglia and cerebral cortex were not only consistent with the findings of previous studies, but also showed new functional correlation between the dorsal striatum (i.e., caudate nucleus and putamen) and insula. In this study, we established the detailed basal ganglia subregional functional correlation patterns using 18 F-FDG PET/MRI fusion imaging. Our methods and results could potentially be an important resource for investigating basal ganglia dysfunction as well as for conducting functional studies in the context of movement and psychiatric disorders. (author)

  4. Inter regional correlations of glucose metabolism between the basal ganglia and different cortical areas: an ultra-high resolution PET/MRI fusion study using {sup 18}F-FDG

    Energy Technology Data Exchange (ETDEWEB)

    Kim, J.H. [Research Institute for Advanced Industrial Technology, Korea University, Sejong (Korea, Republic of); Son, Y.D.; Kim, H.K.; Oh, C.H., E-mail: ohch@korea.ac.kr [College of Health Science, Gachon University, Incheon, (Korea, Republic of); Kim, J.M. [College of Science and Technology, Korea University, Sejong (Korea, Republic of); Kim, Y.B. [Gachon University School of Medicine, Incheon (Korea, Republic of); Lee, C. [Bioimaging Research Team, Korea Basic Science Institute, Cheongju (Korea, Republic of)

    2018-02-01

    Basal ganglia have complex functional connections with the cerebral cortex and are involved in motor control, executive functions of the forebrain, such as the planning of movement, and cognitive behaviors based on their connections. The aim of this study was to provide detailed functional correlation patterns between the basal ganglia and cerebral cortex by conducting an inter regional correlation analysis of the {sup 18}F-fluorodeoxyglucose ({sup 18}F-FDG) positron emission tomography (PET) data based on precise structural information. Fifteen participants were scanned with 7-Tesla magnetic resonance imaging (MRI) and high resolution research tomography (HRRT)-PET fusion system using {sup 18}F-FDG. For detailed inter regional correlation analysis, 24 subregions of the basal ganglia including pre-commissural dorsal caudate, post-commissural caudate, pre-commissural dorsal putamen, post-commissural putamen, internal globus pallidus, and external globus pallidus and 80 cerebral regions were selected as regions of interest on the MRI image and their glucose metabolism were calculated from the PET images. Pearson's product-moment correlation analysis was conducted for the inter regional correlation analysis of the basal ganglia. Functional correlation patterns between the basal ganglia and cerebral cortex were not only consistent with the findings of previous studies, but also showed new functional correlation between the dorsal striatum (i.e., caudate nucleus and putamen) and insula. In this study, we established the detailed basal ganglia subregional functional correlation patterns using {sup 18}F-FDG PET/MRI fusion imaging. Our methods and results could potentially be an important resource for investigating basal ganglia dysfunction as well as for conducting functional studies in the context of movement and psychiatric disorders. (author)

  5. Behavioral Abnormalities and Circuit Defects in the Basal Ganglia of a Mouse Model of 16p11.2 Deletion Syndrome

    Directory of Open Access Journals (Sweden)

    Thomas Portmann

    2014-05-01

    Full Text Available A deletion on human chromosome 16p11.2 is associated with autism spectrum disorders. We deleted the syntenic region on mouse chromosome 7F3. MRI and high-throughput single-cell transcriptomics revealed anatomical and cellular abnormalities, particularly in cortex and striatum of juvenile mutant mice (16p11+/−. We found elevated numbers of striatal medium spiny neurons (MSNs expressing the dopamine D2 receptor (Drd2+ and fewer dopamine-sensitive (Drd1+ neurons in deep layers of cortex. Electrophysiological recordings of Drd2+ MSN revealed synaptic defects, suggesting abnormal basal ganglia circuitry function in 16p11+/− mice. This is further supported by behavioral experiments showing hyperactivity, circling, and deficits in movement control. Strikingly, 16p11+/− mice showed a complete lack of habituation reminiscent of what is observed in some autistic individuals. Our findings unveil a fundamental role of genes affected by the 16p11.2 deletion in establishing the basal ganglia circuitry and provide insights in the pathophysiology of autism.

  6. Neural Dynamics of Autistic Repetitive Behaviors and Fragile X Syndrome: Basal Ganglia Movement Gating and mGluR-Modulated Adaptively Timed Learning

    Directory of Open Access Journals (Sweden)

    Stephen Grossberg

    2018-03-01

    Full Text Available This article develops the iSTART neural model that proposes how specific imbalances in cognitive, emotional, timing, and motor processes that involve brain regions like prefrontal cortex, temporal cortex, amygdala, hypothalamus, hippocampus, and cerebellum may interact together to cause behavioral symptoms of autism. These imbalances include underaroused emotional depression in the amygdala/hypothalamus, learning of hyperspecific recognition categories that help to cause narrowly focused attention in temporal and prefrontal cortices, and breakdowns of adaptively timed motivated attention and motor circuits in the hippocampus and cerebellum. The article expands the model’s explanatory range by, first, explaining recent data about Fragile X syndrome (FXS, mGluR, and trace conditioning; and, second, by explaining distinct causes of stereotyped behaviors in individuals with autism. Some of these stereotyped behaviors, such as an insistence on sameness and circumscribed interests, may result from imbalances in the cognitive and emotional circuits that iSTART models. These behaviors may be ameliorated by operant conditioning methods. Other stereotyped behaviors, such as repetitive motor behaviors, may result from imbalances in how the direct and indirect pathways of the basal ganglia open or close movement gates, respectively. These repetitive behaviors may be ameliorated by drugs that augment D2 dopamine receptor responses or reduce D1 dopamine receptor responses. The article also notes the ubiquitous role of gating by basal ganglia loops in regulating all the functions that iSTART models.

  7. Infiltration of the basal ganglia by brain tumors is associated with the development of co-dominant language function on fMRI.

    Science.gov (United States)

    Shaw, Katharina; Brennan, Nicole; Woo, Kaitlin; Zhang, Zhigang; Young, Robert; Peck, Kyung K; Holodny, Andrei

    2016-01-01

    Studies have shown that some patients with left-hemispheric brain tumors have an increased propensity for developing right-sided language support. However, the precise trigger for establishing co-dominant language function in brain tumor patients remains unknown. We analyzed the MR scans of patients with left-hemispheric tumors and either co-dominant (n=35) or left-hemisphere dominant (n=35) language function on fMRI to investigate anatomical factors influencing hemispheric language dominance. Of eleven neuroanatomical areas evaluated for tumor involvement, the basal ganglia was significantly correlated with co-dominant language function (planguage co-dominance performed significantly better on the Boston Naming Test, a clinical measure of aphasia, compared to their left-lateralized counterparts (56.5 versus 36.5, p=0.025). While further studies are needed to elucidate the role of the basal ganglia in establishing co-dominance, our results suggest that reactive co-dominance may afford a behavioral advantage to patients with left-hemispheric tumors. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Neural Dynamics of Autistic Repetitive Behaviors and Fragile X Syndrome: Basal Ganglia Movement Gating and mGluR-Modulated Adaptively Timed Learning

    Science.gov (United States)

    Grossberg, Stephen; Kishnan, Devika

    2018-01-01

    This article develops the iSTART neural model that proposes how specific imbalances in cognitive, emotional, timing, and motor processes that involve brain regions like prefrontal cortex, temporal cortex, amygdala, hypothalamus, hippocampus, and cerebellum may interact together to cause behavioral symptoms of autism. These imbalances include underaroused emotional depression in the amygdala/hypothalamus, learning of hyperspecific recognition categories that help to cause narrowly focused attention in temporal and prefrontal cortices, and breakdowns of adaptively timed motivated attention and motor circuits in the hippocampus and cerebellum. The article expands the model’s explanatory range by, first, explaining recent data about Fragile X syndrome (FXS), mGluR, and trace conditioning; and, second, by explaining distinct causes of stereotyped behaviors in individuals with autism. Some of these stereotyped behaviors, such as an insistence on sameness and circumscribed interests, may result from imbalances in the cognitive and emotional circuits that iSTART models. These behaviors may be ameliorated by operant conditioning methods. Other stereotyped behaviors, such as repetitive motor behaviors, may result from imbalances in how the direct and indirect pathways of the basal ganglia open or close movement gates, respectively. These repetitive behaviors may be ameliorated by drugs that augment D2 dopamine receptor responses or reduce D1 dopamine receptor responses. The article also notes the ubiquitous role of gating by basal ganglia loops in regulating all the functions that iSTART models. PMID:29593596

  9. Early Changes in Glutamate Metabolism and Perfusion in Basal Ganglia following Hypoxia-Ischemia in Neonatal Piglets: A Multi-Sequence 3.0T MR Study

    Directory of Open Access Journals (Sweden)

    Xiao-ming Wang

    2017-04-01

    Full Text Available The excitotoxicity of glutamate metabolism as well as hemodynamic disorders of the brain are both risk factors for neonatal hypoxic–ischemic brain damage (HIBD. In the present study, changes in glutamate metabolism in the basal ganglia were detected by proton magnetic resonance spectroscopy (1H-MRS at 0–6, 8–12, 24–30, and 48–60 h after the induction of hypoxia-ischemia (HI in newborn piglets. Meanwhile, correlation analysis was performed by combining the microcirculatory perfusion informations acquired by intravoxel incoherent motion (IVIM scan to explore their possible interaction mechanism. The results suggested that Glu level in the basal ganglia underwent a “two-phase” change after HI; perfusion fraction f, an IVIM-derived perfusion parameter, was clearly decreased in the early stage after HI, then demonstrated a transient and slight recovery process, and thereafter continued to decrease. The changes in f and Glu level were in a significant negative correlation (r = −0.643, P = 0.001. Our study results revealed that Glu level is closely associated with the microcirculatory perfusion changes in the acute stage of HIBD.

  10. Large germinoma in basal ganglia treated by intraarterial chemotherapy with ACNU following osmotic blood-brain barrier disruption and radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Miyagami, Mitsusuke; Tsubokawa, Takashi; Kobayashi, Makio.

    1988-10-01

    A rare case of large germinoma in the basal ganglia is reported which was effectively treated by intracarotid chemotherapy with ACNU following osmotic blood-brain barrier disruption using 20 % mannitol and radiation therapy. A 19-year-old man displayed slowly progressive right hemiparesis, motor aphasia and predementia on admission. Plain CT demonstrated a tumor which had a slightly high density with intratumoral calcification and a small cyst, and slight to moderate enhancement was observed following intravenous injection of contrast medium, but there was no unilateral ventricular enlargement. Cerebral angiography revealed hypervascular tumor staining with early draining veins. After biopsy, and as a result of intracarotid chemotherapy with ACNU following osmotic blood-brain barrier disruption and radiation therapy, the tumor decreased rapidly to about 20 % of its original mass. After discharge, tumor progression was observed. However, the enlarged tumor mass almost disappeared (except for calcification) on CT with clinical improvement in response to intracarotid chemotherapy with ACNU following 20 % mannitol.

  11. Computational modeling of stuttering caused by impairments in a basal ganglia thalamo-cortical circuit involved in syllable selection and initiation

    Science.gov (United States)

    Civier, Oren; Bullock, Daniel; Max, Ludo; Guenther, Frank H.

    2013-01-01

    A typical white-matter integrity and elevated dopamine levels have been reported for individuals who stutter. We investigated how such abnormalities may lead to speech dysfluencies due to their effects on a syllable-sequencing circuit that consists of basal ganglia (BG), thalamus, and left ventral premotor cortex (vPMC). “Neurally impaired” versions of the neurocomputational speech production model GODIVA were utilized to test two hypotheses: (1) that white-matter abnormalities disturb the circuit via corticostriatal projections carrying copies of executed motor commands, and (2) that dopaminergic abnormalities disturb the circuit via the striatum. Simulation results support both hypotheses: in both scenarios, the neural abnormalities delay readout of the next syllable’s motor program, leading to dysfluency. The results also account for brain imaging findings during dysfluent speech. It is concluded that each of the two abnormality types can cause stuttering moments, probably by affecting the same BG-thalamus-vPMC circuit. PMID:23872286

  12. Anti-basal ganglia antibodies and Tourette's syndrome: a voxel-based morphometry and diffusion tensor imaging study in an adult population.

    Science.gov (United States)

    Martino, D; Draganski, B; Cavanna, A; Church, A; Defazio, G; Robertson, M M; Frackowiak, R S J; Giovannoni, G; Critchley, H D

    2008-07-01

    Anti-basal ganglia antibodies (ABGAs) have been suggested to be a hallmark of autoimmunity in Gilles de la Tourette's syndrome (GTS), possibly related to prior exposure to streptococcal infection. In order to detect whether the presence of ABGAs was associated with subtle structural changes in GTS, whole-brain analysis using independent sets of T(1) and diffusion tensor imaging MRI-based methods were performed on 22 adults with GTS with (n = 9) and without (n = 13) detectable ABGAs in the serum. Voxel-based morphometry analysis failed to detect any significant difference in grey matter density between ABGA-positive and ABGA-negative groups in caudate nuclei, putamina, thalami and frontal lobes. These results suggest that ABGA synthesis is not related to structural changes in grey and white matter (detectable with these methods) within frontostriatal circuits.

  13. The development of repetitive motor behaviors in deer mice: Effects of environmental enrichment, repeated testing, and differential mediation by indirect basal ganglia pathway activation.

    Science.gov (United States)

    Bechard, Allison R; Bliznyuk, Nikolay; Lewis, Mark H

    2017-04-01

    Little is known about the mechanisms mediating the development of repetitive behaviors in human or animals. Deer mice reared with environmental enrichment (EE) exhibit fewer repetitive behaviors and greater indirect basal ganglia pathway activation as adults than those reared in standard cages. The developmental progression of these behavioral and neural circuitry changes has not been characterized. We assessed the development of repetitive behavior in deer mice using both a longitudinal and cohort design. Repeated testing negated the expected effect of EE, but cohort analyses showed that progression of repetitive behavior was arrested after 1 week of EE and differed significantly from controls after 3 weeks. Moreover, EE reductions in repetitive behavior were associated with increasing activation of indirect pathway nuclei in males across adolescence, but not females. These findings provide the first assessment of developmental trajectories within EE and support indirect pathway mediation of repetitive behavior in male deer mice. © 2017 Wiley Periodicals, Inc.

  14. Believer-Skeptic meets Actor-Critic: Rethinking the role of basal ganglia pathways during decision-making and reinforcement learning

    Directory of Open Access Journals (Sweden)

    Kyle eDunovan

    2016-03-01

    Full Text Available The flexibility of behavioral control is a testament to the brain’s capacity for dynamically resolving uncertainty during goal-directed actions. This ability to select actions and learn from immediate feedback is driven by the dynamics of basal ganglia (BG pathways. A growing body of empirical evidence conflicts with the traditional view that these pathways act as independent levers for facilitating (i.e., direct pathway or suppressing (i.e., indirect pathway motor output, suggesting instead that they engage in a dynamic competition during action decisions that computationally captures action uncertainty. Here we discuss the utility of encoding action uncertainty as a dynamic competition between opposing control pathways and provide evidence that this simple mechanism may have powerful implications for bridging neurocomputational theories of decision making and reinforcement learning.

  15. Comparing the neural correlates of affective and cognitive theory of mind using fMRI: Involvement of the basal ganglia in affective theory of mind.

    Science.gov (United States)

    Bodden, Maren E; Kübler, Dorothee; Knake, Susanne; Menzler, Katja; Heverhagen, Johannes T; Sommer, Jens; Kalbe, Elke; Krach, Sören; Dodel, Richard

    2013-01-01

    Theory of Mind (ToM) is the ability to infer other people's mental states like intentions or desires. ToM can be differentiated into affective (i.e., recognizing the feelings of another person) and cognitive (i.e., inferring the mental state of the counterpart) subcomponents. Recently, subcortical structures such as the basal ganglia (BG) have also been ascribed to the multifaceted concept ToM and most BG disorders have been reported to elicit ToM deficits. In order to assess both the correlates of affective and cognitive ToM as well as involvement of the basal ganglia, 30 healthy participants underwent event-related fMRI scanning, neuropsychological testing, and filled in questionnaires concerning different aspects of ToM and empathy. Directly contrasting affective (aff) as well as cognitive (cog) ToM to the control (phy) condition, activation was found in classical ToM regions, namely parts of the temporal lobe including the superior temporal sulcus, the supplementary motor area, and parietal structures in the right hemisphere. The contrast aff > phy yielded additional activation in the orbitofrontal cortex on the right and the cingulate cortex, the precentral and inferior frontal gyrus and the cerebellum on the left. The right BG were recruited in this contrast as well. The direct contrast aff > cog showed activation in the temporoparietal junction and the cingulate cortex on the right as well as in the left supplementary motor area. The reverse contrast cog > aff however did not yield any significant clusters. In summary, affective and cognitive ToM partly share neural correlates but can also be differentiated anatomically. Furthermore, the BG are involved in affective ToM and thus their contribution is discussed as possibly providing a motor component of simulation processes, particularly in affective ToM.

  16. Using multi-echo simultaneous multi-slice (SMS) EPI to improve functional MRI of the subcortical nuclei of the basal ganglia at ultra-high field (7T).

    Science.gov (United States)

    Puckett, Alexander M; Bollmann, Saskia; Poser, Benedikt A; Palmer, Jake; Barth, Markus; Cunnington, Ross

    2017-12-05

    The nuclei of the basal ganglia pose a special problem for functional MRI, especially at ultra-high field, because T2* variations between different regions result in suboptimal BOLD sensitivity when using gradient-echo echo-planar imaging (EPI). Specifically, the iron-rich lentiform nucleus of the basal ganglia, including the putamen and globus pallidus, suffers from substantial signal loss when imaging is performed using conventional single-echo EPI with echo times optimized for the cortex. Multi-echo EPI acquires several echoes at different echo times for every imaging slice, allowing images to be reconstructed with a weighting of echo times that is optimized individually for each voxel according to the underlying tissue or T2* properties. Here we show that multi-echo simultaneous multi-slice (SMS) EPI can improve functional activation of iron-rich subcortical regions while maintaining sensitivity within cortical areas. Functional imaging during a motor task known to elicit strong activations in the cortex and the subcortex (basal ganglia) was performed to compare the performance of multi-echo SMS EPI to single-echo SMS EPI. Notably within both the caudate nucleus and putamen of the basal ganglia, multi-echo SMS EPI yielded higher tSNR (an average 84% increase) and CNR (an average 58% increase), an approximate 3-fold increase in supra-threshold voxels, and higher t-values (an average 39% increase). The degree of improvement in the group level t-statistics was negatively correlated to the underlying T2* of the voxels, such that the shorter the T2*, as in the iron-rich nuclei of the basal ganglia, the higher the improvement of t-values in the activated region. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Rare case of reversible acute symmetrical lesions of the bilateral Basal Ganglia associated with diabetic nephropathy and chronic renal failure.

    Science.gov (United States)

    Mahajan, Parag Suresh; El Esnawi, Mohamed Amin; Hussein, Sheik Akbar; Al Maslamani, Nasser Jassim

    2014-01-01

    Reversible acute symmetrical basal ganglial lesion on magnetic resonance imaging and/or computed tomography in cases of diabetic nephropathy and chronic renal failure exhibiting acute onset of movement abnormalities like chorea is a very rare entity. It has characteristic clinical and imaging features. Only 29 cases are described in the literature, including the current one. These cases are predominantly Asian patients from the Far East and only one Asian Indian patient has been described. We report the second Asian Indian case of this condition and describe its various clinical and imaging features. Our aim is to educate the clinicians and radiologists about this condition, so that more such cases can be detected.

  18. Expression of Osteogenic Molecules in the Caudate Nucleus and Gray Matter and Their Potential Relevance for Basal Ganglia Calcification in Hypoparathyroidism

    Science.gov (United States)

    Millo, Tabin; Mishra, Shruti; Das, Madhuchhanda; Kapoor, Mansi; Tomar, Neeraj; Saha, Soma; Roy, Tara Shankar; Sreenivas, Vishnubhatla

    2014-01-01

    Background: Basal ganglia calcification (BGC) is an interesting example of ectopic calcification in patients with hypoparathyroidism. Its pathogenesis and reasons for predilection of calcification at basal ganglia are not clear. Objective: To assess the expression of osteogenesis-related molecules in the caudate nucleus and surface gray matter (an area spared from calcification) and discuss potential relevance of the results in context of BGC in idiopathic hypoparathyroidism. Methods: Caudate nucleus and gray matter were obtained from 14 autopsies performed in accidental deaths. The mRNA expression of bone transcription factors (RUNX2/osterix), bone morphogenetic proteins (BMPs) 2 and 4, osteonectin, osteopontin, osteocalcin, vitamin D receptor, calcium sensing-receptor, Na phosphate transporters (PiTs) 1 and 2, N-methyl-D-aspartate receptor 2B (NMDAR2B), carbonic anhydrase II (CA-II), PTH1 receptor (PTH1R), PTH2R, and PTHrP were assessed by RT-PCR. Western blot, spot densitometry, and immunohistochemistry were performed to assess protein expression of molecules showing differences in mRNA expression between caudate and gray tissues. Results: The mean mRNA expression of PiT1 (11.0 ± 10.39 vs 32.9 ± 20.98, P = .003) and PTH2R (1.6 ± 1.47 vs 13.7 ± 6.11, P = .001) were significantly lower in the caudate nucleus than the gray matter. The expression of osteonectin, osteopontin, and CA-II were significantly higher in the caudate nucleus than the gray matter (P = .01, .001, and .04, respectively). The mRNA expression of other molecules was comparable in the 2 tissues. The protein expression of both CA-II and osteonectin was 24% higher and PiT1 17% lower in caudate than the gray matter. The differences in the PTH2R and osteopontin protein expression were not appreciable. Conclusions: The presence of several osteogenic molecules in caudate nucleus indicates that BGC would probably be the outcome of an active process. The differences in expression of these molecules in

  19. An extended reinforcement learning model of basal ganglia to understand the contributions of serotonin and dopamine in risk-based decision making, reward prediction, and punishment learning.

    Science.gov (United States)

    Balasubramani, Pragathi P; Chakravarthy, V Srinivasa; Ravindran, Balaraman; Moustafa, Ahmed A

    2014-01-01

    Although empirical and neural studies show that serotonin (5HT) plays many functional roles in the brain, prior computational models mostly focus on its role in behavioral inhibition. In this study, we present a model of risk based decision making in a modified Reinforcement Learning (RL)-framework. The model depicts the roles of dopamine (DA) and serotonin (5HT) in Basal Ganglia (BG). In this model, the DA signal is represented by the temporal difference error (δ), while the 5HT signal is represented by a parameter (α) that controls risk prediction error. This formulation that accommodates both 5HT and DA reconciles some of the diverse roles of 5HT particularly in connection with the BG system. We apply the model to different experimental paradigms used to study the role of 5HT: (1) Risk-sensitive decision making, where 5HT controls risk assessment, (2) Temporal reward prediction, where 5HT controls time-scale of reward prediction, and (3) Reward/Punishment sensitivity, in which the punishment prediction error depends on 5HT levels. Thus the proposed integrated RL model reconciles several existing theories of 5HT and DA in the BG.

  20. Dopamine transporter density in the basal ganglia assessed with {sup 123}I-IPT SPECT in children with attention deficit hyperactivity disorder

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Y. H.; Cheon, K. A.; Yoon, M. J.; Kim, C. H.; Lee, J. D. [Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, H. H.; Choi, T. H. [Gachon Medical School, Incheon (Korea, Republic of)

    2002-07-01

    Attention deficit hyperactivity disorder (ADHD) is known as a psychiatric disorder in childhood associated with dopamine dysregulation. We investigated dopamine transporter (DAT) density in children with ADHD in the present study using {sup 123}I-IPT SPECT and postulated that an alteration in DAT density in the basal ganglia (BG) is responsible for dopaminergic dysfunction in children with ADHD. 9 durg-naive children with ADHD and 6 normal children were included in the study. We performed brain SPECT 2 hours after administration of {sup 123}I-IPT and made both quantitative and qualitative analyses for assessment of specific/nonspecific DAT binding ratio in the BG. We investigated the correlation between the severity scores of ADHD symptoms in children with ADHD assessed with ADHD rating scale and specific/nonspecific DAT binding ratio in the BG. Drug-naive children with ADHD showed a significantly incresed specific/nonspecific DAT binding ratio in the BG compared with normal children. Whereas, no significant correlation was found between severity scores of symptoms in children with ADHD and specific/nonspecific DAT binding ratio n the BG. Our findings support complex dysregulation of the dopaminergic neurotransmitter system in children with ADHD.

  1. Dopamine transporter density in the basal ganglia assessed with 123I-IPT SPECT in children with attention deficit hyperactivity disorder

    International Nuclear Information System (INIS)

    Yoo, Y. H.; Cheon, K. A.; Yoon, M. J.; Kim, C. H.; Lee, J. D.; Kim, H. H.; Choi, T. H.

    2002-01-01

    Attention deficit hyperactivity disorder (ADHD) is known as a psychiatric disorder in childhood associated with dopamine dysregulation. We investigated dopamine transporter (DAT) density in children with ADHD in the present study using 123 I-IPT SPECT and postulated that an alteration in DAT density in the basal ganglia (BG) is responsible for dopaminergic dysfunction in children with ADHD. 9 durg-naive children with ADHD and 6 normal children were included in the study. We performed brain SPECT 2 hours after administration of 123 I-IPT and made both quantitative and qualitative analyses for assessment of specific/nonspecific DAT binding ratio in the BG. We investigated the correlation between the severity scores of ADHD symptoms in children with ADHD assessed with ADHD rating scale and specific/nonspecific DAT binding ratio in the BG. Drug-naive children with ADHD showed a significantly incresed specific/nonspecific DAT binding ratio in the BG compared with normal children. Whereas, no significant correlation was found between severity scores of symptoms in children with ADHD and specific/nonspecific DAT binding ratio n the BG. Our findings support complex dysregulation of the dopaminergic neurotransmitter system in children with ADHD

  2. Brain MR imaging in patients with hepatic cirrhosis: relationship between high intensity signal in basal ganglia on T1-weighted images and elemental concentrations in brain

    International Nuclear Information System (INIS)

    Maeda, H.; Sato, M.; Yoshikawa, A.; Kimura, M.; Sonomura, T.; Terada, M.; Kishi, K.

    1997-01-01

    In patients with hepatic cirrhosis, the globus pallidus and putamen show high intensity on T1-weighted MRI. While the causes of this high signal have been thought to include paramagnetic substances, especially manganese, no evidence for this has been presented. Autopsy in four cases of hepatic cirrhosis permitted measurement of metal concentrations in brain and histopathological examination. In three cases the globus pallidus showed high intensity on T1-weighted images. Mean manganese concentrations in globus pallidus, putamen and frontal white matter were 3.03 ± 0.38, 2.12 ± 0.37, and 1.38 ± 0.24 (μg/g wet weight), respectively, being approximately four- to almost ten-fold the normal values. Copper concentrations in globus pallidus and putamen were also high, 50 % more than normal. Calcium, iron, zinc and magnesium concentrations were all normal. The fourth case showed no abnormal intensity in the basal ganglia and brain metal concentrations were all normal. Histopathologically, cases with showing high signal remarkable atrophy, necrosis, and deciduation of nerve cells and proliferation of glial cells and microglia in globus pallidus. These findings were similar to those in chronic manganese poisoning. On T1-weighted images, copper deposition shows no abnormal intensity. It is therefore inferred that deposition of highly concentrations of manganese may caused high signal on T1-weighted images and nerve cell death in the globus pallidus. (orig.). With 2 figs., 2 tabs

  3. AN EXTENDED REINFORCEMENT LEARNING MODEL OF BASAL GANGLIA TO UNDERSTAND THE CONTRIBUTIONS OF SEROTONIN AND DOPAMINE IN RISK-BASED DECISION MAKING, REWARD PREDICTION, AND PUNISHMENT LEARNING

    Directory of Open Access Journals (Sweden)

    Pragathi Priyadharsini Balasubramani

    2014-04-01

    Full Text Available Although empirical and neural studies show that serotonin (5HT plays many functional roles in the brain, prior computational models mostly focus on its role in behavioral inhibition. In this study, we present a model of risk based decision making in a modified Reinforcement Learning (RL-framework. The model depicts the roles of dopamine (DA and serotonin (5HT in Basal Ganglia (BG. In this model, the DA signal is represented by the temporal difference error (δ, while the 5HT signal is represented by a parameter (α that controls risk prediction error. This formulation that accommodates both 5HT and DA reconciles some of the diverse roles of 5HT particularly in connection with the BG system. We apply the model to different experimental paradigms used to study the role of 5HT: 1 Risk-sensitive decision making, where 5HT controls risk assessment, 2 Temporal reward prediction, where 5HT controls time-scale of reward prediction, and 3 Reward/Punishment sensitivity, in which the punishment prediction error depends on 5HT levels. Thus the proposed integrated RL model reconciles several existing theories of 5HT and DA in the BG.

  4. Regulation of neuropeptide mRNA expression in the basal ganglia by intrastriatal and intranigral transplants in the rat Parkinson model.

    Science.gov (United States)

    Winkler, C; Bentlage, C; Cenci, M A; Nikkhah, G; Björklund, A

    2003-01-01

    Previous studies have shown that intrastriatal transplants of dopamine (DA)-rich fetal ventral mesencephalic (VM) tissue can correct denervation-induced changes in the cellular expression of neuropeptide and receptor mRNAs in the rat Parkinson model. However, with the standard transplantation approach normalization of all cellular parameters has not been obtained. This may be due either to the incomplete striatal reinnervation achieved by these transplants, or to the ectopic placement of the grafts. In the present study we have used a microtransplantation approach to obtain a more complete reinnervation of the denervated striatum (20 micrograft deposits spread over the entire structure). Neurons were also implanted directly into the substantia nigra. In rats with multiple intrastriatal VM transplants the lesion-induced upregulation of mRNAs encoding for preproenkephalin (PPE), the D(2)-type DA-receptor, and the GABA-synthesizing enzyme glutamic acid decarboxylase (GAD(67)) was normalized throughout the striatum, whereas the lesion-induced downregulation of preprotachykinin mRNA was unaffected. Intranigral grafts of either fetal DA-rich VM tissue or GABA-rich striatal tissue did not induce any changes in striatal neuropeptide and D(2)-receptor mRNA expression despite significant behavioral improvement. Comparison of the behavioral data with levels of neuropeptide expression showed that in rats with intrastriatal VM transplants a complete normalization of striatal PPE and GAD(67) mRNA expression did not translate into a complete recovery of spontaneous motor behaviors. The results show that extensive DA reinnervation of the host striatum by multiple VM microtransplants is insufficient to obtain full recovery of all lesion-induced changes at both the cellular and the behavioral level. A full reconstruction of the nigrostriatal pathway or, alternatively, modulation of basal ganglia function by grafting in non-striatal regions may be required to further improve the

  5. c-Fos immunoreactivity in prefrontal, basal ganglia and limbic areas of the rat brain after central and peripheral administration of ethanol and its metabolite acetaldehyde.

    Directory of Open Access Journals (Sweden)

    Kristen N. Segovia

    2013-05-01

    Full Text Available Considerable evidence indicates that the metabolite of ethanol (EtOH, acetaldehyde, is biologically active. Acetaldehyde can be formed from EtOH peripherally mainly by alcohol dehydrogenase, and also centrally by catalase. EtOH and acetaldehyde show differences in their behavioral effects depending upon the route of administration. In terms of their effects on motor activity and motivated behaviors, when administered peripherally acetaldehyde tends to be more potent than EtOH but shows very similar potency administered centrally. Since dopamine (DA rich areas have an important role in regulating both motor activity and motivation, the present studies were undertaken to compare the effects of central (intraventricular, ICV and peripheral (intraperitoneal, IP administration of EtOH and acetaldehyde on a cellular marker of brain activity, c-Fos immunoreactivity, in DA innervated areas. Male Sprague-Dawley rats received an IP injection of vehicle, EtOH (0.5 or 2.5 g/kg or acetaldehyde (0.1 or 0.5 g/kg or an ICV injection of vehicle, EtOH or acetaldehyde (2.8 or 14.0 µmoles. IP administration of EtOH minimally induced c-Fos in some regions of the prefrontal cortex and basal ganglia, mainly at the low dose (0.5 g/kg, while IP acetaldehyde induced c-Fos in virtually all the structures studied at both doses. Acetaldehyde administered centrally increased c-Fos in all areas studied, a pattern that was very similar to EtOH. Thus, IP administered acetaldehyde was more efficacious than EtOH at inducing c-Fos expression. However, the general pattern of c-Fos induction promoted by ICV EtOH and acetaldehyde was similar. These results are consistent with the pattern observed in behavioral studies in which both substances produced the same magnitude of effect when injected centrally, and produced differences in potency after peripheral administration.

  6. A network model of basal ganglia for understanding the roles of dopamine and serotonin in reward-punishment-risk based decision making

    Directory of Open Access Journals (Sweden)

    Pragathi Priyadharsini Balasubramani

    2015-06-01

    Full Text Available There is significant evidence that in addition to reward-punishment based decision making, the Basal Ganglia (BG contributes to risk-based decision making as well. Despite this evidence, little is known about the computational principles and neural correlates of risk computation in this subcortical system. We have previously proposed a reinforcement learning based model of the BG that simulates the interactions between dopamine (DA and serotonin (5HT in a diverse set of experimental effects including reward, punishment and risk based decision making. Starting with the idea that the activity of DA represents reward prediction error, the model posits that serotoninergic activity in the striatum controls risk-prediction error. Our prior model of the BG was an abstract model that did not incorporate anatomical and cellular-level data. In this work, we expand the earlier model into a detailed network model of the BG and demonstrate the joint contributions of DA-5HT in risk and reward-punishment sensitivity. At the core of the proposed network model is the following insight regarding cellular correlates of value and risk computation. Just as DA D1 receptor (D1R expressing medium spiny neurons (MSNs of the striatum were thought to be neural substrates for value computation, we propose that DA D1R and D2R co-expressing MSNs, reported to occupy a significant proportion of the striatum and are implicated in disorders like schizophrenia and drug addiction, are capable of computing risk. Ours is the first-of-its-kind model that accounts for the significant computational possibilities of these co-expressing D1R-D2R MSNs, and describes how DA-5HT mediated activity in these classes of neurons (D1R-, D2R-, D1R-D2R- MSNs contribute to the BG dynamics. We also apply the model to capture the behaviour of PD patients in a probabilistic learning paradigm. The study observes that optimizing 5HT levels along with DA medication could be essential to improving the

  7. Influence of deep brain stimulation on postural stability in patients with Parkinson disease

    OpenAIRE

    Zelenková, Jana

    2012-01-01

    Parkinson's disease is a neurodegenerative disease of the basal ganglia. Its main symptoms are rigidity, tremor, bradykinesia, hypokinesia and postural instability. One possible way how to infuence diseases is neurosurgical treatment - deep brain stimulation. The principle is the implantation of electrodes in the basal ganglia and modulation of activity of the basal ganglia circuits due to electrical stimulation. Stimulation affects the motor symptoms of Parkinson's disease. This thesis deals...

  8. Do spotty high intensity regions found in basal ganglia on MRI T2-weighted brain images of elderly subjects indicate gliosis? Comparison of brain MRI T2-weighted images of elderly subjects and necropsy brain

    International Nuclear Information System (INIS)

    Murai, Hiroshi; Hattori, Hideyuki; Matsumoto, Masayuki

    2001-01-01

    Spotty high intensity regions are frequently found on the MRI T2-weighted brain images (T2WI) of elderly people. High intensity regions with a diameter of 3 mm or less have been considered as expanded perivascular space with no pathological implications on radiological diagnosis. However, its morphometrical basis is not clear. We examined the character of the spotty regions using brain MRI of brain screening subjects, and studied morphometrically arteriolosclerosis and perivascular tissue damage using necropsy brains of subjects aged 65 years and over. The size, number and location of the spotty high intensity regions were examined using the brain MRI of 109 T2WI which is used for brain screening at Kanazawa Medical University Hospital. The frontal lobe, temporal lobe, parietal lobe, hippocampus, midbrain and basal ganglia were sampled from 15 subjects aged 65 years and over, and the tissue sections were processed for HE stain, Elastica van Gieson stain and immunostaining with GFAP. We took photographs of brain arterioli and surrounding parenchyma with a digital telescope camera and the degree of arterioscleosis and tissue damage were assessed by measurements with an image analyzer. Spotty high intensity regions on T2WI with a diameter of 3 mm or less were observed in 95.5% subjects aged 65 years and over. 69.4% spotty region was observed in basal ganglia. There was a significant correlation between age and size. In morphometrical examination, at the basal ganglia, the density of GFAP-positive astrocytes in the perivascular tissue had a significant positive correlation with the proportional thickness of the adventitia, which is an index of arteriosclerosis, and a significant negative correlation with the size of the perivascular space. The results suggested that the spotty regions in the brain MRI of elderly people do not represent dilatations of the perivascular space, but is mild brain damage caused by arteriosclerosis. (author)

  9. Sodium P-Aminosalicylic Acid Improved Manganese-Induced Learning and Memory Dysfunction via Restoring the Ultrastructural Alterations and γ-Aminobutyric Acid Metabolism Imbalance in the Basal Ganglia.

    Science.gov (United States)

    Ou, Chao-Yan; Luo, Yi-Ni; He, Sheng-Nan; Deng, Xiang-Fa; Luo, Hai-Lan; Yuan, Zong-Xiang; Meng, Hao-Yang; Mo, Yu-Huan; Li, Shao-Jun; Jiang, Yue-Ming

    2017-03-01

    Excessive intake of manganese (Mn) may cause neurotoxicity. Sodium para-aminosalicylic acid (PAS-Na) has been used successfully in the treatment of Mn-induced neurotoxicity. The γ-aminobutyric acid (GABA) is related with learning and memory abilities. However, the mechanism of PAS-Na on improving Mn-induced behavioral deficits is unclear. The current study was aimed to investigate the effects of PAS-Na on Mn-induced behavioral deficits and the involvement of ultrastructural alterations and γ-aminobutyric acid (GABA) metabolism in the basal ganglia of rats. Sprague-Dawley rats received daily intraperitoneally injections of 15 mg/kg MnCl 2 .4H 2 O, 5d/week for 4 weeks, followed by a daily back subcutaneously (sc.) dose of PAS-Na (100 and 200 mg/kg), 5 days/week for another 3 or 6 weeks. Mn exposure for 4 weeks and then ceased Mn exposure for 3 or 6 weeks impaired spatial learning and memory abilities, and these effects were long-lasting. Moreover, Mn exposure caused ultrastructural alterations in the basal ganglia expressed as swollen neuronal with increasing the electron density in the protrusions structure and fuzzed the interval of neuropil, together with swollen, focal hyperplasia, and hypertrophy of astrocytes. Additionally, the results also indicated that Mn exposure increased Glu/GABA values as by feedback loops controlling GAT-1, GABA A mRNA and GABA A protein expression through decreasing GABA transporter 1(GAT-1) and GABA A receptor (GABA A ) mRNA expression, and increasing GABA A protein expression in the basal ganglia. But Mn exposure had no effects on GAT-1 protein expression. PAS-Na treatment for 3 or 6 weeks effectively restored the above-mentioned adverse effects induced by Mn. In conclusion, these findings suggest the involvement of GABA metabolism and ultrastructural alterations of basal ganglia in PAS-Na's protective effects on the spatial learning and memory abilities.

  10. Hypomyelination with atrophy of the basal ganglia and cerebellum: case report Hipomielinização com atrofia dos núcleos da base e do cerebelo: relato de caso

    Directory of Open Access Journals (Sweden)

    André Palma da Cunha Matta

    2007-03-01

    Full Text Available Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC is a rare disease that has been recently described. It must be remembered as a possible etiology of leukoencephalopathies in children. We describe a typical case of H-ABC in a 11-month-old boy. He presents with global development delay, oral dyskinesia, and global dystonia and spasticity. Magnetic resonance imaging disclosed typical features of H-ABC and clinical laboratory tests were all negative. A slow neurological deterioration has been detected with worsening of involuntary movements.A hipomielinização com atrofia dos núcleos da base e do cerebelo (H-ABC é uma rara afecção que deve ser lembrada como possível diagnóstico das leucoencefalopatias de difícil definição etiológica. Descrevemos um típico caso de H-ABC em um menino de 11 meses, sem antecedentes de risco para lesão cerebral, que evoluiu com atraso psicomotor acompanhado de discinesia perioral, distonia e espasticidade generalizadas. A ressonância magnética do encéfalo sugere fortemente o diagnóstico de H-ABC e os exames complementares para pesquisar possíveis diagnósticos diferenciais são negativos. O curso clínico tem sido lentamente progressivo com ausência de ganhos motores e piora dos movimentos involuntários.

  11. Neurobiologia do parkinsonismo: I. substratos neurais e neuroquímica dos gânglios basais Neurobiology of parkinsonism: I. neuroanatomical substrates and neurochemistry of the basal ganglia

    Directory of Open Access Journals (Sweden)

    Noberto Garcia-Cairasco

    1995-09-01

    Full Text Available As desordens do movimento caracterizam-se pelo rompimento da harmonia existente entre os múltiplos sistemas cerebrais e musculares responsáveis pela integração do indivíduo com o seu meio e espécie. Dentre os sistemas cerebrais, os gânglios basais são a sede das manifestações motoras presentes tanto nas hipercinesias quanto nas hipocinesias. A busca de agentes etiológicos para o parkinsonismo em muito tem contribuído para o conhecimento da organização neuroanatômica e neuroquímica dos gânglios basais, sugerindo a existência de múltiplos sub-sistemas dentro do circuito córtex-gânglios da base-tálamo-córtex. Nesta revisão apresentamos os principais sistemas e sub-sistemas envolvidos no parkinsonismo.Movement disorders, in general, are characterized by a breakdown in the integrated coordination of posture and motion by multiple brain and muscular systems. In the expression of parkinsonism, in particular, critical and altered structures such as substantia nigra, appear to be related to the cortex-basal ganglia and thalamus-basal ganglia sub-circuits.

  12. Basal Cell Carcinoma in Type 2 Segmental Darier's Disease

    Directory of Open Access Journals (Sweden)

    Lynne Robertson

    2012-01-01

    Full Text Available Background. Darier's disease (DD, also known as Keratosis Follicularis or Darier-White disease, is a rare disorder of keratinization. DD can present as a generalized autosomal dominant condition as well as a localized or segmental postzygotic condition (Vázquez et al., 2002. Clinical features of DD include greasy, warty papules and plaques on seborrheic areas, dystrophic nails, palmo-plantar pits, and papules on the dorsum of the hands and feet. Objective. We report a case of basal cell carcinoma developing in a patient with type 2 segmental DD. Conclusion. According to the current literature, Type 2 segmental disease is a rare presentation of Darier's disease with only 8 previous cases reported to date. In addition, nonmelanoma skin cancer (NMSC arising from DD is rarely reported; however, there may be an association between DD and risk of carcinogenesis.

  13. Transcranial Magnetic Stimulation (TMS) as a Tool for Early Diagnosis and Prognostication in Cortico-Basal Ganglia Degeneration (CBD) Syndromes: Review of Literature and Case Report.

    Science.gov (United States)

    Issac, Thomas Gregor; Chandra, Sadanandavalli Retnaswami; Nagaraju, B C

    2016-01-01

    Cortico basal degeneration (CBD) of the brain is a rare progressive neurodegenerative disease which encompasses unique neuropsychiatric manifestations. Early diagnosis is essential for initiating proper treatment and favorable outcome. Transcranial Magnetic Stimulation (TMS), a well-known technique for assessment of cortical excitatory and inhibitory properties. It was suggested that in a degenerative disease like CBD which involves the cortex as well as the subcortical structures, comparing both hemispheres, a differential pattern in TMS can be obtained which would help in early identification, prognostication and early therapeutic intervention. We describe a case of CBD with corroborative clinical and imaging picture wherein single pulse TMS was used over both the hemispheres measuring the following parameters of interest which included: Motor Threshold (MT), Central Motor Conduction Time (CMCT) and Silent Period (SP). Differential patterns of MT, CMCT and SP was obtained by stimulating over both the hemispheres with the affected hemisphere showing significantly reduced MT and prolonged CMCT implying early impairment of cortical and subcortical structures thereby revealing the potential application of TMS being utilized in a novel way for early detection and prognostication in CBD syndromes.

  14. Neurobiological correlates of impulsivity in healthy adults: Lower prefrontal gray matter volume and spontaneous eye-blink rate but greater resting-state functional connectivity in basal ganglia-thalamo-cortical circuitry.

    Science.gov (United States)

    Korponay, Cole; Dentico, Daniela; Kral, Tammi; Ly, Martina; Kruis, Ayla; Goldman, Robin; Lutz, Antoine; Davidson, Richard J

    2017-08-15

    Studies consistently implicate aberrance of the brain's reward-processing and decision-making networks in disorders featuring high levels of impulsivity, such as attention-deficit hyperactivity disorder, substance use disorder, and psychopathy. However, less is known about the neurobiological determinants of individual differences in impulsivity in the general population. In this study of 105 healthy adults, we examined relationships between impulsivity and three neurobiological metrics - gray matter volume, resting-state functional connectivity, and spontaneous eye-blink rate, a physiological indicator of central dopaminergic activity. Impulsivity was measured both by performance on a task of behavioral inhibition (go/no-go task) and by self-ratings of attentional, motor, and non-planning impulsivity using the Barratt Impulsiveness Scale (BIS-11). Overall, we found that less gray matter in medial orbitofrontal cortex and paracingulate gyrus, greater resting-state functional connectivity between nodes of the basal ganglia-thalamo-cortical network, and lower spontaneous eye-blink rate were associated with greater impulsivity. Specifically, less prefrontal gray matter was associated with higher BIS-11 motor and non-planning impulsivity scores, but was not related to task performance; greater correlated resting-state functional connectivity between the basal ganglia and thalamus, motor cortices, and prefrontal cortex was associated with worse no-go trial accuracy on the task and with higher BIS-11 motor impulsivity scores; lower spontaneous eye-blink rate was associated with worse no-go trial accuracy and with higher BIS-11 motor impulsivity scores. These data provide evidence that individual differences in impulsivity in the general population are related to variability in multiple neurobiological metrics in the brain's reward-processing and decision-making networks. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Subthalamic nucleus stimulation does not influence basal glucose metabolism or insulin sensitivity in patients with Parkinson's disease

    NARCIS (Netherlands)

    Lammers, Nicolette M.; Sondermeijer, Brigitte M.; Twickler, Th B. Marcel; de Bie, Rob M.; Ackermans, Mariëtte T.; Fliers, Eric; Schuurman, P. Richard; la Fleur, Susanne E.; Serlie, Mireille J.

    2014-01-01

    Animal studies have shown that central dopamine signaling influences glucose metabolism. As a first step to show this association in an experimental setting in humans, we studied whether deep brain stimulation (DBS) of the subthalamic nucleus (STN), which modulates the basal ganglia circuitry,

  16. Subregional Basal Forebrain Atrophy in Alzheimer's Disease: A Multicenter Study

    Science.gov (United States)

    Kilimann, Ingo; Grothe, Michel; Heinsen, Helmut; Alho, Eduardo Joaquim Lopez; Grinberg, Lea; Amaro, Edson; dos Santos, Gláucia Aparecida Bento; da Silva, Rafael Emídio; Mitchell, Alex J.; Frisoni, Giovanni B.; Bokde, Arun L.W.; Fellgiebel, Andreas; Filippi, Massimo; Hampel, Harald; Klöppel, Stefan; Teipel, Stefan J.

    2014-01-01

    Histopathological studies in Alzheimer's disease (AD) suggest severe and region-specific neurodegeneration of the basal forebrain cholinergic system (BFCS). Here, we studied the between-center reliability and diagnostic accuracy of MRI-based BFCS volumetry in a large multicenter data set, including participants with prodromal (n = 41) or clinically manifest AD (n = 134) and 148 cognitively healthy controls. Atrophy was determined using voxel-based and region-of-interest based analyses of high-dimensionally normalized MRI scans using a newly created map of the BFCS based on postmortem in cranio MRI and histology. The AD group showed significant volume reductions of all subregions of the BFCS, which were most pronounced in the posterior nucleus basalis Meynert (NbM). The mild cognitive impairment-AD group showed pronounced volume reductions in the posterior NbM, but preserved volumes of anterior-medial regions. Diagnostic accuracy of posterior NbM volume was superior to hippocampus volume in both groups, despite higher multicenter variability of the BFCS measurements. The data of our study suggest that BFCS morphometry may provide an emerging biomarker in AD. PMID:24503619

  17. Pallidal gap junctions-triggers of synchrony in Parkinson's disease?

    NARCIS (Netherlands)

    Schwab, B.C.; Heida, T.; Zhao, Y.; Gils, S.A. van; Wezel, R.J.A. van

    2014-01-01

    Although increased synchrony of the neural activity in the basal ganglia may underlie the motor deficiencies exhibited in Parkinson's disease (PD), how this synchrony arises, propagates through the basal ganglia, and changes under dopamine replacement remains unknown. Gap junctions could play a

  18. Pallidal gap junctions - Triggers of synchrony in Parkinson's disease?

    NARCIS (Netherlands)

    Schwab, B.C.; Heida, Tjitske; Zhao, Yan; van Gils, Stephanus A.; van Wezel, Richard Jack Anton

    2014-01-01

    Although increased synchrony of the neural activity in the basal ganglia may underlie the motor deficiencies exhibited in Parkinson's disease (PD), how this synchrony arises, propagates through the basal ganglia, and changes under dopamine replacement remains unknown. Gap junctions could play a

  19. Parkinson's disease as a system-level disorder

    NARCIS (Netherlands)

    Caligiore, D.; Helmich, R.C.G.; Hallett, M.; Moustafa, A.A.; Timmermann, L.; Toni, I.; Baldassarre, G.

    2016-01-01

    Traditionally, the basal ganglia have been considered the main brain region implicated in Parkinson's disease. This single area perspective gives a restricted clinical picture and limits therapeutic approaches because it ignores the influence of altered interactions between the basal ganglia and

  20. Pathogenesis of Parkinson's disease

    OpenAIRE

    Riederer, Peter; Lange, Klaus W.

    1992-01-01

    The importance of genetic aspects, ageing, environmental factors, head trauma, defective mitochondrial respiration, altered iron metabolism, oxidative stress and glutamatergic overactivity of the basal ganglia in the pathogenesis of Parkinson's disease (PD) are considered in this review.

  1. Neuromodulatory Adaptive Combination of Correlation-based Learning in Cerebellum and Reward-based Learning in Basal Ganglia for Goal-directed Behavior Control

    DEFF Research Database (Denmark)

    Dasgupta, Sakyasingha; Wörgötter, Florentin; Manoonpong, Poramate

    2014-01-01

    of biological abstraction, we clearly demonstrate that such a RMHP induced combinatorial learning mechanism, leads to stabler and faster learning of goal-directed behaviors, in comparison to the individual systems. Thus, in this paper we provide a computational model for adaptive combination of the basal......Goal-directed decision making in biological systems is broadly based on associations between conditional and unconditional stimuli. This can be further classified as classical conditioning (correlation-based learning) and operant conditioning (reward-based learning). A number of computational...... of the cerebellum, we demonstrate that the RMHP rule can effectively balance the outcomes of the two learning systems. This is tested using simulated environments of increasing complexity with a four-wheeled robot in a foraging task in both static and dynamic configurations. Although modeled with a simplified level...

  2. Light-Induced Alterations in Basil Ganglia Kynurenic Acid Levels

    Science.gov (United States)

    Sroufe, Angela E.; Whittaker, J. A.; Patrickson, J. W.; Orr, M. C.

    1997-01-01

    The metabolic synthesis, release and breakdown of several known CNS neurotransmitters have been shown to follow a circadian pattern entrained to the environmental light/dark cycle. The levels of excitatory amino acid (EAA) transmitters such as glutamate, have been shown to vary with environmental lighting conditions. Kynurenic Acid (KA), an endogenous tryptophan metabolite and glutamate receptor antagonist, has been reported to have neuroprotective effects against EAA-induced excitotoxic cell damage. Changes in KA's activity within the mammalian basal ganglia has been proposed as being contributory to neurotoxicity in Huntington's Disease. It is not known whether CNS KA levels follow a circadian pattern or exhibit light-induced fluctuations. However, because the symptoms of certain degenerative motor disorders seem to fluctuate with daily 24 hour rhythm, we initiated studies to determine if basal ganglia KA were influenced by the daily light/dark cycle and could influence motor function. Therefore in this study, HPLC-EC was utilized to determine if basal ganglia KA levels in tissue extracts from adult male Long-Evans rats (200-250g) entrained to 24 and 48 hours constant light and dark conditions, respectively. Samples were taken one hour before the onset of the subjective day and one hour prior to the onset of the subjective night in order to detect possible phase differences in KA levels and to allow for accumulation of factors expressed in association with the light or dark phase. Data analysis revealed that KA levels in the basal ganglia vary with environmental lighting conditions; being elevated generally during the dark. Circadian phase differences in KA levels were also evident during the subjective night and subjective day, respectively. Results from these studies are discussed with respect to potential cyclic changes in neuronal susceptibility to excitotoxic damage during the daily 24 hour cycle and its possible relevance to future therapeutic approaches in

  3. A pilot study of basal ganglia and thalamus structure by high dimensional mapping in children with Tourette syndrome [v1; ref status: indexed, http://f1000r.es/1yu

    Directory of Open Access Journals (Sweden)

    Alton C. Williams

    2013-10-01

    Full Text Available Background: Prior brain imaging and autopsy studies have suggested that structural abnormalities of the basal ganglia (BG nuclei may be present in Tourette Syndrome (TS. These studies have focused mainly on the volume differences of the BG structures and not their anatomical shapes.  Shape differences of various brain structures have been demonstrated in other neuropsychiatric disorders using large-deformation, high dimensional brain mapping (HDBM-LD.  A previous study of a small sample of adult TS patients demonstrated the validity of the method, but did not find significant differences compared to controls. Since TS usually begins in childhood and adult studies may show structure differences due to adaptations, we hypothesized that differences in BG and thalamus structure geometry and volume due to etiological changes in TS might be better characterized in children. Objective: Pilot the HDBM-LD method in children and estimate effect sizes. Methods: In this pilot study, T1-weighted MRIs were collected in 13 children with TS and 16 healthy, tic-free, control children. The groups were well matched for age.  The primary outcome measures were the first 10 eigenvectors which are derived using HDBM-LD methods and represent the majority of the geometric shape of each structure, and the volumes of each structure adjusted for whole brain volume. We also compared hemispheric right/left asymmetry and estimated effect sizes for both volume and shape differences between groups. Results: We found no statistically significant differences between the TS subjects and controls in volume, shape, or right/left asymmetry.  Effect sizes were greater for shape analysis than for volume. Conclusion: This study represents one of the first efforts to study the shape as opposed to the volume of the BG in TS, but power was limited by sample size. Shape analysis by the HDBM-LD method may prove more sensitive to group differences.

  4. Understanding the Functional Plasticity in Neural Networks of the Basal Ganglia in Cocaine Use Disorder: A Role for Allosteric Receptor-Receptor Interactions in A2A-D2 Heteroreceptor Complexes

    Directory of Open Access Journals (Sweden)

    Dasiel O. Borroto-Escuela

    2016-01-01

    Full Text Available Our hypothesis is that allosteric receptor-receptor interactions in homo- and heteroreceptor complexes may form the molecular basis of learning and memory. This principle is illustrated by showing how cocaine abuse can alter the adenosine A2AR-dopamine D2R heterocomplexes and their receptor-receptor interactions and hereby induce neural plasticity in the basal ganglia. Studies with A2AR ligands using cocaine self-administration procedures indicate that antagonistic allosteric A2AR-D2R heterocomplexes of the ventral striatopallidal GABA antireward pathway play a significant role in reducing cocaine induced reward, motivation, and cocaine seeking. Anticocaine actions of A2AR agonists can also be produced at A2AR homocomplexes in these antireward neurons, actions in which are independent of D2R signaling. At the A2AR-D2R heterocomplex, they are dependent on the strength of the antagonistic allosteric A2AR-D2R interaction and the number of A2AR-D2R and A2AR-D2R-sigma1R heterocomplexes present in the ventral striatopallidal GABA neurons. It involves a differential cocaine-induced increase in sigma1Rs in the ventral versus the dorsal striatum. In contrast, the allosteric brake on the D2R protomer signaling in the A2AR-D2R heterocomplex of the dorsal striatopallidal GABA neurons is lost upon cocaine self-administration. This is potentially due to differences in composition and allosteric plasticity of these complexes versus those in the ventral striatopallidal neurons.

  5. Iodine-123 IMP SPECT before and after bypass surgery in a patient with occlusion of left anterior and middle cerebral arteries with basal abnormal telangiectasis (unilateral Moyamoya disease)

    International Nuclear Information System (INIS)

    Honda, Norinari; Machida, Kikuo; Takishima, Teruo; Kaizu, Hiroyuki; Sugimoto, Eiichi

    1987-01-01

    A case of left anterior and middle cerebral arterial occlusion with angiographic features similar to Moyamoya disease was reported. IMP SPECT of the patient revealed the success of bypass surgery clearly. The patient complained of transient right hemiparesis with aphasia 4 times. The cerebral arteriography disclosed occlusions of left anterior and middle cerebral arteries at their proximal portions. Right internal carotid and its branches were normal. I-123 IMP SPECT study showed hypoperfusion in left temporal lobe, basal ganglia with incomplete reperfusion on the delayed (4 hours after injection) SPECT images. After the superficial temporal-middle cerebral artery anastomosis, I-123 IMP SPECT showed improvement of the brain blood flow. I-123 IMP SPECT was very useful in detecting the ischemic areas and evaluating the revascularizing surgery in this case. (author)

  6. Basal ganglia contributions to adaptive navigation.

    Science.gov (United States)

    Mizumori, Sheri J Y; Puryear, Corey B; Martig, Adria K

    2009-04-12

    The striatum has long been considered to be selectively important for nondeclarative, procedural types of memory. This stands in contrast with spatial context processing that is typically attributed to hippocampus. Neurophysiological evidence from studies of the neural mechanisms of adaptive navigation reveals that distinct neural systems such as the striatum and hippocampus continuously process task relevant information regardless of the current cognitive strategy. For example, both striatal and hippocampal neural representations reflect spatial location, directional heading, reward, and egocentric movement features of a test situation in an experience-dependent way, and independent of task demands. Thus, continual parallel processing across memory systems may be the norm rather than the exception. It is suggested that neuromodulators, such as dopamine, may serve to differentially regulate learning-induced neural plasticity mechanisms within these memory systems such that the most successful form of neural processing exerts the strongest control over response selection functions. In this way, dopamine may serve to optimize behavioral choices in the face of changing environmental demands during navigation.

  7. Increased basal glucose production in type 1 Gaucher's disease

    NARCIS (Netherlands)

    Corssmit, E. P.; Hollak, C. E.; Endert, E.; van Oers, M. H.; Sauerwein, H. P.; Romijn, J. A.

    1995-01-01

    To evaluate the metabolic effects of Gaucher's disease, glucose metabolism and parameters of fat metabolism were studied by indirect calorimetry and primed continuous infusion of [3-3H]glucose in seven clinically stable untreated patients with type 1 Gaucher's disease and in seven healthy matched

  8. Enteric Neural Cells From Hirschsprung Disease Patients Form Ganglia in Autologous Aneuronal ColonSummary

    Directory of Open Access Journals (Sweden)

    Benjamin N. Rollo

    2016-01-01

    Full Text Available Background & Aims: Hirschsprung disease (HSCR is caused by failure of cells derived from the neural crest (NC to colonize the distal bowel in early embryogenesis, resulting in absence of the enteric nervous system (ENS and failure of intestinal transit postnatally. Treatment is by distal bowel resection, but neural cell replacement may be an alternative. We tested whether aneuronal (aganglionic colon tissue from patients may be colonized by autologous ENS-derived cells. Methods: Cells were obtained and cryopreserved from 31 HSCR patients from the proximal resection margin of colon, and ENS cells were isolated using flow cytometry for the NC marker p75 (nine patients. Aneuronal colon tissue was obtained from the distal resection margin (23 patients. ENS cells were assessed for NC markers immunohistologically and by quantitative reverse-transcription polymerase chain reaction, and mitosis was detected by ethynyl-2′-deoxyuridine labeling. The ability of human HSCR postnatal ENS-derived cells to colonize the embryonic intestine was demonstrated by organ coculture with avian embryo gut, and the ability of human postnatal HSCR aneuronal colon muscle to support ENS formation was tested by organ coculture with embryonic mouse ENS cells. Finally, the ability of HSCR patient ENS cells to colonize autologous aneuronal colon muscle tissue was assessed. Results: ENS-derived p75-sorted cells from patients expressed multiple NC progenitor and differentiation markers and proliferated in culture under conditions simulating Wnt signaling. In organ culture, patient ENS cells migrated appropriately in aneural quail embryo gut, and mouse embryo ENS cells rapidly spread, differentiated, and extended axons in patient aneuronal colon muscle tissue. Postnatal ENS cells derived from HSCR patients colonized autologous aneuronal colon tissue in cocultures, proliferating and differentiating as neurons and glia. Conclusions: NC-lineage cells can be obtained from HSCR

  9. Glucose metabolism in small subcortical structures in Parkinson's disease

    DEFF Research Database (Denmark)

    Borghammer, Per; Hansen, Søren B; Eggers, Carsten

    2012-01-01

    Evidence from experimental animal models of Parkinson's disease (PD) suggests a characteristic pattern of metabolic perturbation in discrete, very small basal ganglia structures. These structures are generally too small to allow valid investigation by conventional positron emission tomography (PET...

  10. Kinesiotherapy of Parkinson`s disease and Parkinson`s syndrom

    OpenAIRE

    Zechovská, Lenka

    2013-01-01

    Author: Lenka Zechovská Institution: Rehabilitation Clinic, Faculty of Medicine in Hradec Králové Title: Kinesiotherapy of Parkinson's disease and Parkinson's syndrome Supervisor: Mgr. Ivana Vondráková Number of pages: 115 Number of attachments: 8 Year of defence: 2013 Keywords: Parkinson's disease, basal ganglia, tremor, rigidity, hypokinesia Bachelor thesis deals with the problems of Parkinson's disease and Parkinson's syndrome. The theoretical part includes the basal ganglia pathophysiolog...

  11. Darier′s disease with warty dyskeratoma and basal cell epithelioma

    Directory of Open Access Journals (Sweden)

    Prabhakara V

    1996-01-01

    Full Text Available A 48-year old man with warty dirty papules over the seborrhoeic areas, also had a nodule with a central keratotic crater over the right cheek. Biopsy of this nodule revealed features of Darier′s disease, warty dyskeratoma and basal cell epithelioma. Even though Darier′s disease and warty dyskeratoma are considered as a distinct entities, the presence in the same lesion emphasizes the need for further studies on this association.

  12. Basal and Adrenocorticotropic Hormone Stimulated Plasma Cortisol Levels Among Egyptian Autistic Children: Relation to Disease Severity

    Directory of Open Access Journals (Sweden)

    Hewedi Doaa H

    2010-10-01

    Full Text Available Abstract Background Autism is a disorder of early childhood characterized by social impairment, communication abnormalities and stereotyped behaviors. The hypothalamic-pituitary-adrenocortical (HPA axis deserves special attention, since it is the basis for emotions and social interactions that are affected in autism. Aim To assess basal and stimulated plasma cortisol, and adrenocorticotropic hormone (ACTH levels in autistic children and their relationship to disease characteristics. Methods Fifty autistic children were studied in comparison to 50 healthy age-, sex- and pubertal stage- matched children. All subjects were subjected to clinical evaluation and measurement of plasma cortisol (basal and stimulated and ACTH. In addition, electroencephalography (EEG and intelligence quotient (IQ assessment were done for all autistic children. Results Sixteen% of autistic patients had high ACTH, 10% had low basal cortisol and 10% did not show adequate cortisol response to ACTH stimulation. Autistic patients had lower basal (p = 0.032 and stimulated cortisol (p = 0.04 and higher ACTH (p = 0.01 than controls. Childhood Autism Rating Scale (CARS score correlated positively with ACTH (r = 0.71, p = 0.02 and negatively with each of basal (r = -0.64, p = 0.04 and stimulated cortisol (r = -0.88, p Conclusions The observed hormonal changes may be due to a dysfunction in the HPA axis in autistic individuals. Further studies are warranted regarding the role of HPA axis dysfunction in the pathogenesis of autism.

  13. Anticipatory guidance in type 2 diabetes to improve disease management; next steps after basal insulin.

    Science.gov (United States)

    Johnson, Eric L; Frias, Juan P; Trujillo, Jennifer M

    2018-03-23

    The alarming rise in the number of people living with type 2 diabetes (T2D) presents primary care physicians with increasing challenges associated with long-term chronic disease care. Studies have shown that the majority of patients are not achieving or maintaining glycemic goals, putting them at risk of a wide range of diabetes-related complications. Disease- and self-management programs have been shown to help patients improve their glycemic control, and are likely to be of particular benefit for patients with diabetes dealing with these issues. Anticipatory guidance is an individualized, proactive approach to patient education and counseling by a health-care professional to support patients in better coping with problems before they arise. It has been shown to improve disease outcomes in a variety of chronic conditions, including diabetes. While important at all stages, anticipatory guidance may be of particular importance during changes in treatment regimens, and especially during transition to, and escalation of, insulin-based regimens. The aim of this article is to provide advice to physicians on anticipatory guidance for basal-insulin dosing, focusing on appropriate basal-insulin-dose increase and prevention of potentially deleterious basal-insulin doses, so called overbasalization. It also provides an overview of new treatment options for patients with T2D who are not well controlled on basal-insulin therapy, fixed-ratio combinations of basal insulin and glucagon-like peptide-1 receptor agonists, and advice on the type of anticipatory guidance needed to ensure safe and appropriate switching to these therapies.

  14. Fronto-striatal grey matter contributions to discrimination learning in Parkinson's disease

    NARCIS (Netherlands)

    O'Callaghan, C.; Moustafa, A.A.; de Wit, S.; Shine, J.M.; Robbins, T.W.; Lewis, S.J.G.; Hornberger, M.

    2013-01-01

    Discrimination learning deficits in Parkinson's disease (PD) have been well-established. Using both behavioral patient studies and computational approaches, these deficits have typically been attributed to dopamine imbalance across the basal ganglia. However, this explanation of impaired learning in

  15. Comparative histochemical study of Bowen’s disease and actinic keratosis: preserved normal basal cells in Bowen’s disease

    Directory of Open Access Journals (Sweden)

    H Ishida

    2009-12-01

    Full Text Available The degree of DNA-instability as revealed by immunohistochemical staining with anti-cytidine antibody after acid hydrolysis (DNA-instability test has been recently used as a marker of malignancy. This technique was applied to examine 17 skin tissue samples of Bowen’s disease, 47 of actinic keratosis, 15 of squamous cell carcinoma, 5 of seborrheic keratosis, and 10 of normal skin. All benign neoplastic cells of seborrheic keratosis and normal epidermal cells were negative. On the other hand, all cancer cells were positive with the DNA-instability test, indicating their malignancy, but all basal cells in Bowen’s disease were completely negative. Compatible with this result, the basal cells in Bowen’s disease were characteristically normal as evident in other histochemical examinations. Thus, they were negative with p53 immunohistochemistry, with normal signals of chromosome 17 in situ hybridisation and argyrophilic nucleolar organiser region, and showed slightly enhanced proliferative activity as revealed by proliferating cell nuclear antigen immunohistochemistry. Immunohistochemical staining with 34 ß E12 (monoclonal antibody against cytokeratins 1, 5, 10, and 14, which stains all normal epidermal keratinocytes including basal cells, showed that only the basal cells of Bowen’s disease stained strongly and homogeneously, while all cancer cells in the upper layers of Bowen’s disease and all layers of actinic keratosis were only sporadically or weakly stained. Staining with 34 ß B4 (monoclonal antibody against cytokeratin 1, which recognises the whole epidermis except for the basal layer in the normal epidermis, showed that the basal cells in the Bowen’s disease were completely negative, and lower layer cells in the actinic keratosis and upper layer cells in Bowen’s disease were only sporadically stained positive, although the superficial layer cells in actinic keratosis stained strongly and homogeneously. Our findings clearly

  16. Knockdown of GAD67 protein levels normalizes neuronal activity in a rat model of Parkinson's disease

    DEFF Research Database (Denmark)

    Horvath, Lazlo; van Marion, Ingrid; Taï, Khalid

    2011-01-01

    Dopamine depletion of the striatum is one of the hallmarks of Parkinson's disease. The loss of dopamine upregulates GAD67 expression in the striatal projection neurons and causes other changes in the activity of the basal ganglia circuit.......Dopamine depletion of the striatum is one of the hallmarks of Parkinson's disease. The loss of dopamine upregulates GAD67 expression in the striatal projection neurons and causes other changes in the activity of the basal ganglia circuit....

  17. Survey of Basal Stem Rot Disease on Oil Palms (Elaeis guineensis Jacq.) in Kebun Bukit Kijang,North Sumatera, Indonesia

    Science.gov (United States)

    Lisnawita; Hanum, H.; Tantawi, A. R.

    2016-08-01

    Basal stem rot disease caused by Ganoderma sp. is a significant disease on oil palm plantations in Indonesia, especially in North Sumatera. Currently, the pathogen does not only attack the plants that have produced (old plants) but also attacks the plants that have not produced in the first generation yet. A survey of the distribution of the basal stem rot disease in the plantation of the community has been completed in order to illustrate the distribution and the incidence of the basal stem rot disease in 5 locations of the oil palm plantation of the community in Desa Bukit Kijang, Region of Asahan, North Sumatera, Indonesia. From the research, it is revealed that the basal stem rot disease has spread to all of the observed locations with the level of disease incidence between 0.71% in Kebun Bukit Kijang 3 to 50% in the 17 years old oil palm in Kebun Bukit Kijang 4 and Bukit Kijang 5. The observable symptoms of the basal stem rot disease are chlorotic leaves, the appearance of fruiting body, collapsed plants, and the existence of holes on the basal stem. The incidence of basal stem rot disease is higher on land due to a high sand content (>50%).

  18. CT brain demonstration of basal ganglion calcification in adult HIV ...

    African Journals Online (AJOL)

    brain barrier has been postulated. Calcification of the basal ganglia in encephalopathic HIV/AIDS children has been relatively well documented. Only two adult HIV cases with basal ganglion calcification (BGC) have been reported in the literature.

  19. Parkinson's Disease

    Science.gov (United States)

    ... a long and relatively healthy life. What Causes Parkinson's Disease? In the very deep parts of the brain, there is a collection of nerve cells that help control movement, known as the basal ganglia (say: BAY-sul GAN-glee-ah). In a ...

  20. [Expression of promyelocytic leukaemia protein in Bowen's disease, skin squamous cell carcinoma and basal cell carcinoma].

    Science.gov (United States)

    Wang, Qiongyu; Ma, Huiqun; Wang, Shijie; Ma, Yunyun; Zou, Xingwei; Li, Ruilian

    2013-07-01

    To investigate the expression of promyelocytic leukaemia (PML) protein of PML protein in Bowen's disease (BD), skin squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) and explore the role of PML in the pathogenesis of these diseases. PML protein in normal skin tissues and lesions of Bowen's disease, SCC and BCC were detected with immunohistochemistry. Normal skin tissues did not express PML protein. In BCC, PML showed rather low expressions in the skin lesions (8.69% in cell nuclei and 4.35% in cytoplasm). The lesions in BD and SCC (grade I and II) showed obvious overexpression of PML protein in the cell nuclei and cytoplasm, and its expression in the cell nuclei of these lesions was significantly higher than that in grade III-IV SCC. PML protein may play an important role in the early stage of SCC, and its overexpression may contribute to the carcinogenesis and metastasis of SCC.

  1. Spatial statistical analysis of basal stem root disease under natural field epidemic of oil palm

    Science.gov (United States)

    Kamu, Assis; Phin, Chong Khim; Seman, Idris Abu; Wan, Hoong Hak; Mun, Ho Chong

    2015-02-01

    Oil palm or scientifically known as Elaeis guineensis Jacq. is the most important commodity crop in Malaysia and has greatly contributed to the economy growth of the country. As far as disease is concerned in the industry, Basal Stem Rot (BSR) caused by Ganoderma boninence remains the most important disease. BSR disease is the most widely studied with information available for oil palm disease in Malaysia. However, there is still limited study on the spatial as well as temporal pattern or distribution of the disease especially under natural field epidemic condition in oil palm plantation. The objective of this study is to spatially identify the pattern of BSR disease under natural field epidemic using two geospatial analytical techniques, which are quadrat analysis for the first order properties of partial pattern analysis and nearest-neighbor analysis (NNA) for the second order properties of partial pattern analysis. Two study sites were selected with different age of tree. Both sites are located in Tawau, Sabah and managed by the same company. The results showed that at least one of the point pattern analysis used which is NNA (i.e. the second order properties of partial pattern analysis) has confirmed the disease is complete spatial randomness. This suggests the spread of the disease is not from tree to tree and the age of palm does not play a significance role in determining the spatial pattern of the disease. From the spatial pattern of the disease, it would help in the disease management program and for the industry in the future. The statistical modelling is expected to help in identifying the right model to estimate the yield loss of oil palm due to BSR disease in the future.

  2. Basal Root Rot, a new Disease of Teak (Tectona grandis in Malaysia caused by Phellinus noxius

    Directory of Open Access Journals (Sweden)

    Mohd Farid, A.

    2005-01-01

    Full Text Available Basal root rot of teak was first reported from Sabak Bernam, Selangor making this the first report of the disease on teak in Peninsular Malaysia. The fungus found associated with the disease was Phellinus noxious. The disease aggressively killed its host irrespective of the host health status. Bark depression at the root collar which was visible from a distance was the characteristic symptom and the main indicator in identifying the disease in the plantation since above ground symptoms of the canopy could not be differentiated from crowns of healthy trees. However, although above ground symptoms were not easily discernible, the disease was already advanced and the trees mostly beyond treatment; 3.4 % of the trees in the plantation were affected and the disease occurred both on solitary trees and in patches. Below ground, infected trees had rotted root systems, mainly below and around the collar region with brown discolored wood and irregular golden-brown honeycomb-like pockets of fungal hyphae in the wood. Pathogenicity tests showed that the fungus produced symptoms similar to those observed in the plantation and killed two year-old teak plants. The disease killed all the inoculated hosts within three months, irrespective of wounded or unwounded treatments.

  3. Neural correlates underlying micrographia in Parkinson’s disease

    Science.gov (United States)

    Zhang, Jiarong; Hallett, Mark; Feng, Tao; Hou, Yanan; Chan, Piu

    2016-01-01

    Micrographia is a common symptom in Parkinson’s disease, which manifests as either a consistent or progressive reduction in the size of handwriting or both. Neural correlates underlying micrographia remain unclear. We used functional magnetic resonance imaging to investigate micrographia-related neural activity and connectivity modulations. In addition, the effect of attention and dopaminergic administration on micrographia was examined. We found that consistent micrographia was associated with decreased activity and connectivity in the basal ganglia motor circuit; while progressive micrographia was related to the dysfunction of basal ganglia motor circuit together with disconnections between the rostral supplementary motor area, rostral cingulate motor area and cerebellum. Attention significantly improved both consistent and progressive micrographia, accompanied by recruitment of anterior putamen and dorsolateral prefrontal cortex. Levodopa improved consistent micrographia accompanied by increased activity and connectivity in the basal ganglia motor circuit, but had no effect on progressive micrographia. Our findings suggest that consistent micrographia is related to dysfunction of the basal ganglia motor circuit; while dysfunction of the basal ganglia motor circuit and disconnection between the rostral supplementary motor area, rostral cingulate motor area and cerebellum likely contributes to progressive micrographia. Attention improves both types of micrographia by recruiting additional brain networks. Levodopa improves consistent micrographia by restoring the function of the basal ganglia motor circuit, but does not improve progressive micrographia, probably because of failure to repair the disconnected networks. PMID:26525918

  4. Celiac ganglia block

    Energy Technology Data Exchange (ETDEWEB)

    Akinci, Devrim [Department of Radiology, Hacettepe University School of Medicine, Sihhiye, 06100 Ankara (Turkey); Akhan, Okan [Department of Radiology, Hacettepe University School of Medicine, Sihhiye, 06100 Ankara (Turkey)]. E-mail: oakhan@hacettepe.edu.tr

    2005-09-01

    Pain occurs frequently in patients with advanced cancers. Tumors originating from upper abdominal viscera such as pancreas, stomach, duodenum, proximal small bowel, liver and biliary tract and from compressing enlarged lymph nodes can cause severe abdominal pain, which do not respond satisfactorily to medical treatment or radiotherapy. Percutaneous celiac ganglia block (CGB) can be performed with high success and low complication rates under imaging guidance to obtain pain relief in patients with upper abdominal malignancies. A significant relationship between pain relief and degree of tumoral celiac ganglia invasion according to CT features was described in the literature. Performing the procedure in the early grades of celiac ganglia invasion on CT can increase the effectiveness of the CGB, which is contrary to World Health Organization criteria stating that CGB must be performed in patients with advanced stage cancer. CGB may also be effectively performed in patients with chronic pancreatitis for pain palliation.

  5. Celiac ganglia block

    International Nuclear Information System (INIS)

    Akinci, Devrim; Akhan, Okan

    2005-01-01

    Pain occurs frequently in patients with advanced cancers. Tumors originating from upper abdominal viscera such as pancreas, stomach, duodenum, proximal small bowel, liver and biliary tract and from compressing enlarged lymph nodes can cause severe abdominal pain, which do not respond satisfactorily to medical treatment or radiotherapy. Percutaneous celiac ganglia block (CGB) can be performed with high success and low complication rates under imaging guidance to obtain pain relief in patients with upper abdominal malignancies. A significant relationship between pain relief and degree of tumoral celiac ganglia invasion according to CT features was described in the literature. Performing the procedure in the early grades of celiac ganglia invasion on CT can increase the effectiveness of the CGB, which is contrary to World Health Organization criteria stating that CGB must be performed in patients with advanced stage cancer. CGB may also be effectively performed in patients with chronic pancreatitis for pain palliation

  6. Bilateral lunate intraosseous ganglia

    International Nuclear Information System (INIS)

    Pablos, J.M.; Valdes, J.C.; Gavilan, F.

    1998-01-01

    An intraosseous ganglion is a relatively uncommon, benign, cyst-like lesion that occurs in young and middle-aged adults. Most commonly seen adjacent to the hip, ankle, knee, or wrist, they are histologically identical to their soft tissue counterparts. A review of the literature revealed only two previously reported examples of bilateral symmetrical ganglia of the lunate bones. (orig.)

  7. Basal metabolic rate in children with chronic kidney disease and healthy control children.

    Science.gov (United States)

    Anderson, Caroline E; Gilbert, Rodney D; Elia, Marinos

    2015-11-01

    Meeting energy requirements of children with chronic kidney disease (CKD) is paramount to optimising growth and clinical outcome, but little information on this subject has been published. In this study, we examined basal metabolic rate (BMR; a component of energy expenditure) with the aim to determine whether it is related to kidney function independently of weight, height and lean body mass (LBM). Twenty children with CKD and 20 healthy age- and gender-matched control children were studied on one occasion. BMR was measured by indirect open circuit calorimetry and predicted by the Schofield equation. Estimated glomerular filtration rate (eGFR) was related to BMR and adjusted for weight, height, age and LBM measured by skinfold thickness. The adjusted BMR of children with CKD did not differ significantly from that of healthy subjects (1296 ± 318 vs.1325 ± 178 kcal/day; p = 0.720). Percentage of predicted BMR also did not differ between the two groups (102 ± 12% vs. 99 ± 14%; p = 0.570). Within the CKD group, eGFR (mean 33.7 ± 20.5 mL/min/m(2)) was significantly related to BMR (β 0.3, r = 0.517, p = 0.019) independently of nutritional status and LBM. It seems reasonable to use estimated average requirement as the basis of energy prescriptions for children with CKD (mean CKD stage 3 disease). However, those who were sicker had significantly lower metabolic rates.

  8. Functional Connectivity of the Corticobasal Ganglia-Thalamocortical Network in Parkinson Disease: A Systematic Review and Meta-Analysis with Cross-Validation.

    Science.gov (United States)

    Ji, Gong-Jun; Hu, Panpan; Liu, Ting-Ting; Li, Ying; Chen, Xingui; Zhu, Chunyan; Tian, Yanghua; Chen, Xianwen; Wang, Kai

    2018-03-07

    Purpose To quantitatively summarize the functional connectivity (FC) feature of the corticobasal ganglia-thalamocortical (CBTC) network in patients with Parkinson disease (PD) by means of a meta-analysis with cross-validation. Materials and Methods For this prospective study, a systematic literature search in the PubMed and EMBASE databases was performed for resting-state functional magnetic resonance (MR) imaging studies of PD published between January 2000 and May 2017. Then, a coordinate-based meta-analysis was conducted by Effect Size-Signed Differential Mapping. A cross-validation analysis was performed by using an independent resting-state functional MR imaging data set that contained 25 patients with PD and 19 age-, sex-, and education-matched healthy control participants. Two-sample t test was performed on FC maps between PD and control groups. Results Thirty studies with 854 patients with PD and 831 control participants were included in this meta-analysis. The main meta-analysis found increased FC in the left pre- and postcentral gyrus in patients with PD compared with healthy control participants (z = 2.6; P meta-analyses on medication-naive (n = 25; z = 2.2; P meta-analysis emphasizes the left postcentral gyrus as a critical region in PD, which may become a potential target for clinical intervention. © RSNA, 2018 Online supplemental material is available for this article.

  9. Association Between Smoking and Cholinergic Basal Forebrain Volume in Healthy Aging and Prodromal and Dementia Stages of Alzheimer's Disease.

    Science.gov (United States)

    Teipel, Stefan; Grothe, Michel J

    2016-04-12

    Smoking has been found associated with decreased cerebral volumes in healthy adults and in various neuropsychiatric disorders. We aimed to determine whether chronic nicotine exposure through smoking is associated with reduced volume of cortically projecting cholinergic basal forebrain nuclei in healthy aging, mild cognitive impairment (MCI), and dementia stages of Alzheimer's disease (AD). We retrieved cross-sectional data from the Alzheimer's Disease Neuroimaging Initiative (ADNI) database including 179 cognitively normal elderly subjects, 270 subjects with early stage MCI, 136 subjects in later, more advanced, stage of MCI, and 86 subjects in dementia stages of AD. We determined the association between past or current smoking versus lifetime non-smoker status on the volumes of the basal forebrain determined from volumetric MRI scans. Hippocampus volume was used as a control region. Significant effects were controlled for mediating or moderating effects of respiratory and cardiovascular morbidity. In cognitively healthy individuals and early MCI, past or current smoking was significantly associated with smaller basal forebrain volume. This effect was independent from age, sex, or cardiovascular or respiratory morbidity. Hippocampus volume was not associated with smoking. In late MCI and AD dementia, smoking was not associated with basal forebrain or hippocampus volumes. Our findings suggest that chronic nicotine exposure through smoking may lead to atrophy of cholinergic input areas of the basal forebrain. This effect may account for an increased risk of AD dementia onset with smoking by exhausting the cholinergic system reserve capacity.

  10. Basal ganglia hemorrhagic ablation associated with temporary suppression of obsessive-compulsive symptoms Ablação hemorrágica dos gânglios da base associada temporalmente à supressão dos sintomas obsessivos-compulsivos

    Directory of Open Access Journals (Sweden)

    José A Yaryura-Tobias

    2003-03-01

    Full Text Available Currently, basal ganglia (BG are considered regulators of motor and emotional activity. It's operationality encompass Obsessive Compulsive Disorder (OCD. The case of a patient suffering with severe OCD is described of note, his symptoms disappeared following a hemorrhage of the left BG. However, once the hemorrhage was reabsorbed his symptoms returned. It is possible that lesions affecting cerebral OCD association circuits may influence the evolution of obsessive-compulsive symptoms.Atualmente, os gânglios da base (GB são considerados reguladores da atividade motora e emocional e seu funcionamento está envolvido com o Transtorno Compulsivo Obsessivo (TOC. Este relato descreve um caso de um paciente com diagnóstico de TOC, cujos sintomas remitiram após hemorragia nos GB à esquerda. No entanto, após a reabsorção do processo hemorrágico, os sintomas reapareceram. É possível que lesões que afetem os circuitos cerebrais associados ao TOC possam influenciar na evolução dos sintomas obsessivos compulsivos.

  11. Limitations of Ber-EP4 for distinction of Bowen disease from basal cell carcinoma.

    Science.gov (United States)

    Kogut, Marta; Toberer, Ferdinand; Enk, Alexander H; Hassel, Jessica C

    2016-04-01

    Diagnostic differentiation between Bowen disease a variant of squamous cell carcinoma in situ (SCCIS) and basal cell carcinoma (BCC) can be difficult on the basis of hematoxylin and eosin (routine) staining in small or fragmented biopsy samples. Ber-EP4 staining is diagnostically reliable for differentiation between BCC and cutaneous squamous cell carcinoma [Dasgeb et al. Biomark Cancer, 5: 7, (2013); Tellechea et al. Am J Dermatopathol, 15: 452 (1993)]. The objective of this study was to determine the usefulness of Ber-EP4 staining for differentiation of SCCIS from BCC. Here, we performed immunohistochemistry with a Ber-EP4 antibody in 76 cases of Bowen type of SCCIS. As positive controls we selected 16 cases of BCC. A positive reaction was obtained for Ber-EP4 in the secretory portion of eccrine and apocrine glands and in follicular germinative cells at the lower end of catagen hairs of normal skin tissue. All BCC samples tested were positive. Of the Bowen type SCCIS samples, 26.3% reacted positively (at least 5% positive staining of neoplastic cells). In 3/76 cases (3.9%) more than 50% of the tumor cells expressed Ber-EP4. We conclude that Ber-EP4 expression is not always helpful to distinguish between Bowen type of SCCIS and BCC, as Ber-EP4 expression in Bowen type SCCIS is not infrequent. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Proton MRS in Behcet's disease with and without neurological findings

    International Nuclear Information System (INIS)

    Baysal, T.; Sarac, K.; Dusak, A.; Ozisik, H.I.; Ozcan, C.; Karlidag, R.; Baysal, O.; Hazneci, E.

    2003-01-01

    Our aim was to investigate whether neurological impairment in Behcet's disease (BD) can be assessed by means of proton MRS and whether it can assist in prognosis. We used single-voxel MRS to measure metabolites in regions of normal-appearing pons, basal ganglia and periventricular white matter (PWM) in 32 patients with chronic BD patients with and without neurological deficits and 29 control subjects. Patients had significantly higher N-acetylaspartate (NAA)/creatine (Cr) and choline (Cho)/Cr ratios in the basal ganglia than the controls. The Cho/Cr ratio in the PWM was also significantly higher in the patients. MRS enabled clear discrimination of patients and controls and also revealed spectral differences between non-neuro-Behcet's disease and neuro-Behcet's disease in the basal ganglia. MRS can be used to assess brain involvement in BD even if structural changes are absent. (orig.)

  13. CT identification of coeliac ganglia

    Energy Technology Data Exchange (ETDEWEB)

    Dal Pozzo, G.; Bozza, A.; Fargnoli, R.; Brizzi, E.

    1985-02-01

    The authors achieved the ''in vivo'' identification of the coeliac ganglia (C.G), using computerised tomography (CT). This result was confirmed by autopsies and by CT scans of an anatomical specimen in which the coeliac ganglia had been previously marked. CT allows and exact location of the coeliac ganglia and can be very useful for a precise alcoholic neurolysis of the coeliac plexus.

  14. Time estimation in Parkinson's disease and degenerative cerebellar disease

    NARCIS (Netherlands)

    Beudel, Martijin; Galama, Sjoukje; Leenders, Klaus L.; de Jong, Bauke M.

    2008-01-01

    With functional MRI, we recently identified fronto-cerebellar activations in predicting time to reach a target and basal ganglia activation in velocity estimation, that is, small interval assessment. We now tested these functions in patients with Parkinson's disease (PD) and degenerative cerebellar

  15. Cortical restricted diffusion as the predominant MRI finding in sporadic Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Talbott, Sabrina D.; Sattenberg, Ronald J.; Heidenreich, Jens O. (Dept. of Radiology, Univ. of Louisville, Louisville (United States)), e-mail: sdtalb02@gwise.louisville.edu; Plato, Brian M (Dept. of Neurology, Univ. of Louisville, Louisville (United States)); Parker, John (Dept. of Pathology and Laboratory Medicine, Univ. of Louisville, Louisville (United States))

    2011-04-15

    Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder with MR findings predominantly limited to the grey matter of the cortex and the basal ganglia. Sporadic Creutzfeldt-Jakob disease can produce a spectrum of MR imaging findings of the brain, most notably on DWI and FLAIR sequences. Involvement of the basal ganglia and neocortex is the most common finding, but isolated involvement of the cortex can also be seen. We describe the clinical history and MRI findings of three patients with sporadic Creutzfeldt-Jakob disease confirmed by brain biopsy or autopsy and review the literature of imaging manifestations of this disease

  16. Cortical restricted diffusion as the predominant MRI finding in sporadic Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Talbott, Sabrina D.; Sattenberg, Ronald J.; Heidenreich, Jens O.; Plato, Brian M; Parker, John

    2011-01-01

    Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder with MR findings predominantly limited to the grey matter of the cortex and the basal ganglia. Sporadic Creutzfeldt-Jakob disease can produce a spectrum of MR imaging findings of the brain, most notably on DWI and FLAIR sequences. Involvement of the basal ganglia and neocortex is the most common finding, but isolated involvement of the cortex can also be seen. We describe the clinical history and MRI findings of three patients with sporadic Creutzfeldt-Jakob disease confirmed by brain biopsy or autopsy and review the literature of imaging manifestations of this disease

  17. Primary brain lymphoma presenting as Parkinson's disease

    International Nuclear Information System (INIS)

    Sanchez-Guerra, M.; Leno, C.; Berciano, J.; Cerezal, L.; Diez, C.; Figols, J.

    2001-01-01

    Neoplasm is an uncommon cause of a parkinsonian syndrome. We report a woman with primary brain B-cell lymphoma presenting as Parkinson's disease. After 1 year of the illness, CT and MRI showed lesions without mass effect in the basal ganglia and corpus callosum. The patient did not respond to levodopa and right cerebellar and brain-stem signs appeared, which prompted further neuroimaging, showing an increase in size of the lesions and a right cerebellar and pontine mass. Stereotactic biopsy of the basal ganglia showed high-grade B-cell lymphoma. Despite the basal ganglia frequently being involved in lymphoma of the brain, presentation with typical or atypical parkinsonism is exceptional. (orig.)

  18. Gaucher disease type I: assessment of basal metabolic rate in patients from southern Brazil.

    Science.gov (United States)

    Doneda, Divair; Lopes, André L; Oliveira, Alvaro R; Netto, Cristina B; Moulin, Cileide C; Schwartz, Ida V D

    2011-01-15

    Gaucher disease (GD) is characterized by clinical heterogeneity and is associated with metabolic abnormalities such as increased resting energy expenditure. To assess the basal metabolic rate (BMR) of patients with GD type I followed at the Gaucher Disease Reference Center of Rio Grande do Sul, Brazil. Fourteen patients (male=6) and 14 healthy controls matched by gender, age and body mass index (BMI) were included in the study. The nutritional status of patients was assessed by BMI. The BMR was measured by indirect calorimetry. In two patients, it was possible to perform BMR in the pre- and the post-treatment periods. Mean age and BMI of patients and controls were, respectively, 32.8 ± 17.6 and 32.1 ± 16.6 years and 23.3 ± 3.1 and 22.4 ± 3.1 kg/m(2). Twelve patients were receiving enzyme replacement therapy (ERT) with imiglucerase (mean duration of treatment=5.2 ± 4.3 years; mean dosage of imiglucerase=24.2 ± 7.3 UI/kg/inf). Five patients (36%) were overweight, and nine (64%) were normal weight. Mean BMR of patients on ERT was 27.1% higher than that of controls (p=0.007). There was no difference between the BMR of patients on ERT and not on ERT (n=4) (p=0.92). Comparing the BMR of patients on ERT and their controls with the BMR estimated by the Harris-Benedict equation, the BMR of patients was 6.3% higher than the estimated (p = 0.1), while the BMR of their controls was 17.0% lower than the estimated (p = 0.001). Most treated GD type I patients were normal weight. The patients including those on ERT showed higher BMR when compared to controls. Imiglucerase is probably unable to normalize the hypermetabolism presented by GD type I patients. Additional studies should be performed to confirm our findings. Copyright © 2010 Elsevier Inc. All rights reserved.

  19. Cerebral glucose metabolism in Parkinson's disease

    International Nuclear Information System (INIS)

    Martin, W.R.W.; Beckman, J.H.; Calne, D.B.; Adam, M.J.; Harrop, R.; Rogers, J.G.; Ruth, T.J.; Sayre, C.I.; Pate, B.D.

    1984-01-01

    Local cerebral glucose utilization was measured in patients with predominantly unilateral Parkinson's disease using sup(18)F-2-fluoro-deoxyglucose and positron emission tomography. Preliminary results indicate the presence of asymmetric metabolic rates in the inferior basal ganglia. The structure comprising the largest portion of basal ganglia at this level is globus pallidus. These findings are consistent with metabolic studies on animals with unilateral nigrostriatal lesions in which pallidal hypermetabolism on the lesioned side has been demonstrated. Increased pallidal activity is likely secondary to a loss of inhibitory dopaminergic input to the striatum from substantia nigra

  20. Comparison on different strategies for treatments of hypertensive hemorrhage in the basal ganglia region with a volume of 25 to 35ml Comparação de diferentes estratégias no tratamento da hemorragia hipertensiva da região dos gânglios da base com volume de 25 a 35ml

    Directory of Open Access Journals (Sweden)

    Wei Zheng

    2012-10-01

    Full Text Available PURPOSE: To compare curative effect of different treatments for hypertensive cerebral hemorrhage of 25 to 35ml. METHODS: In this study, 595 cases were enrolled and grouped regarding treatments including conservative treatment, evacuation with microinvasive craniopuncture technique within 6h and 6-48h after the attack. RESULTS: After follow up for three months after the attack, the assessment based on the Activity of Daily Living (ADL indicated no significant difference among conservative treatment and surgical interventions (p>0.05. However, surgical interventions showed advantages of shorter hospitalization, quick removal of hematoma and obvious reduction of cost. CONCLUSION: The microinvasive craniopuncture technique to drain the hematoma within 6-48h may be a good way in treating hypertensive hemorrhage of basal ganglia region.OBJETIVO: Comparar o efeito curativo de diferentes tratamentos da hemorragia hipertensiva cerebral de 25 a 35ml. MÉTODOS: Foram analisados 595 casos agrupados segundo tratamento conservador e evacuação com técnica de punção transcraniana dentro de 6h ou de 6 às 48h do início do quadro clínico. RESULTADOS: O seguimento após três meses e avaliado pelo Escore de Atividade de Vida Diário, indicou que não houve diferenças significantes entre os tratamentos conservador e cirúrgico (p>0.05 O tratamento cirúrgico mostrou vantagem com hospitalização mais curta e redução de custos. CONCLUSÃO: A técnica de punção transcraniana para drenagem de hematoma dos núcleos da base pode ser uma boa alternativa de tratamento.

  1. modelling gait syndrome in huntington's disease: the genetic ...

    African Journals Online (AJOL)

    HOD

    Huntington's disease (HD) which usually affects the patients at middle age results from malfunctioning of the basal ganglia. It is characterized by cognitive impairment, involuntary movements, neuropsychiatric and psychological disturbances. Early motor signs of Huntington's disease typically include the gradual onset of ...

  2. Non-motor extranigral signs and symptoms in Parkinson's disease

    NARCIS (Netherlands)

    Wolters, E.C.

    2009-01-01

    Clinical symptoms in Parkinson's disease (PD) comprise both motor and non-motor symptoms. In this disease, synucleinopathic-induced, nigral dopamine deficiency-related dysfunction of the basal ganglia is held responsible for the characteristic levodopa-responsive motor signs and symptoms

  3. Proton MRS in Behcet's disease with and without neurological findings

    Energy Technology Data Exchange (ETDEWEB)

    Baysal, T.; Sarac, K.; Dusak, A. [Department of Radiology, Inonu University School of Medicine, 44069, Malatya (Turkey); Ozisik, H.I.; Ozcan, C. [Department of Neurology, Inonu University School of Medicine, 44069, Malatya (Turkey); Karlidag, R. [Department of Psychiatry, Inonu University School of Medicine, 44069, Malatya (Turkey); Baysal, O. [Department of Physical Therapy and Rehabilitation, Inonu University School of Medicine, 44069, Malayta (Turkey); Hazneci, E. [Department of Dermatology, Inonu University School of Medicine, 44069, Malatya (Turkey)

    2003-12-01

    Our aim was to investigate whether neurological impairment in Behcet's disease (BD) can be assessed by means of proton MRS and whether it can assist in prognosis. We used single-voxel MRS to measure metabolites in regions of normal-appearing pons, basal ganglia and periventricular white matter (PWM) in 32 patients with chronic BD patients with and without neurological deficits and 29 control subjects. Patients had significantly higher N-acetylaspartate (NAA)/creatine (Cr) and choline (Cho)/Cr ratios in the basal ganglia than the controls. The Cho/Cr ratio in the PWM was also significantly higher in the patients. MRS enabled clear discrimination of patients and controls and also revealed spectral differences between non-neuro-Behcet's disease and neuro-Behcet's disease in the basal ganglia. MRS can be used to assess brain involvement in BD even if structural changes are absent. (orig.)

  4. Role of Basal Ganglia in Swallowing Process: A Systematic Review

    Directory of Open Access Journals (Sweden)

    Hamideh Ghaemi

    2016-12-01

    Discussion: Swallowing is a multifaceted performance that needs contribution of the tongue, larynx, pharynx, and esophagus as well as the neurological structures such as neocortex and subcortical regions - BG and brainstem.

  5. Genetics Home Reference: familial idiopathic basal ganglia calcification

    Science.gov (United States)

    ... O, Defebvre L, Krystkowiak P, Pariente J, Clanet M, Labauge P, Ayrignac X, Lefaucheur R, Le Ber I, Frébourg T, Hannequin D, Campion D. Mutation of ... DB, Stoessl AJ, Hutton M, Manyam BV, Boller F, Baquero M, Geschwind DH. Genetic heterogeneity in ... M, Saiki S, Sakai K, Matsunari I, Higashi K, Murata KY, Hattori N, Hirose G. ...

  6. computed tomography features of basal ganglia and periventricular ...

    African Journals Online (AJOL)

    CWelman. E Kader. Departmmt of Paediatric Radiology. University of Cape Town and. Institute of Chz1d Health. Red Cross War Memorial Children's Hospital. Cape Town. I. Osborne AG. DiDgrwstic Neuroradiology. Sl Louis Mosby· Year Book. I 994, 93-98, 673-l;S(), 743-. 745. 2. Barkovich A). Pt!diatric N

  7. Volumetric changes in the Basal Ganglia after antipsychotic monotherapy

    DEFF Research Database (Denmark)

    Ebdrup, B H; Nørbak, H; Borgwardt, S

    2013-01-01

    studies. Results: We identified 13 studies published in the period from 1996 to 2011. Overall six compounds (two classified as FGAs and four as SGAs) have been investigated: haloperidol, zuclophentixol, risperidone, olanzapine, clozapine, and quetiapine. The follow-up period ranged from 3-24 months...

  8. Neural correlates underlying micrographia in Parkinson's disease.

    Science.gov (United States)

    Wu, Tao; Zhang, Jiarong; Hallett, Mark; Feng, Tao; Hou, Yanan; Chan, Piu

    2016-01-01

    Micrographia is a common symptom in Parkinson's disease, which manifests as either a consistent or progressive reduction in the size of handwriting or both. Neural correlates underlying micrographia remain unclear. We used functional magnetic resonance imaging to investigate micrographia-related neural activity and connectivity modulations. In addition, the effect of attention and dopaminergic administration on micrographia was examined. We found that consistent micrographia was associated with decreased activity and connectivity in the basal ganglia motor circuit; while progressive micrographia was related to the dysfunction of basal ganglia motor circuit together with disconnections between the rostral supplementary motor area, rostral cingulate motor area and cerebellum. Attention significantly improved both consistent and progressive micrographia, accompanied by recruitment of anterior putamen and dorsolateral prefrontal cortex. Levodopa improved consistent micrographia accompanied by increased activity and connectivity in the basal ganglia motor circuit, but had no effect on progressive micrographia. Our findings suggest that consistent micrographia is related to dysfunction of the basal ganglia motor circuit; while dysfunction of the basal ganglia motor circuit and disconnection between the rostral supplementary motor area, rostral cingulate motor area and cerebellum likely contributes to progressive micrographia. Attention improves both types of micrographia by recruiting additional brain networks. Levodopa improves consistent micrographia by restoring the function of the basal ganglia motor circuit, but does not improve progressive micrographia, probably because of failure to repair the disconnected networks. © The Author (2015). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  9. Emotion and Object Processing in Parkinson's Disease

    Science.gov (United States)

    Cohen, Henri; Gagne, Marie-Helene; Hess, Ursula; Pourcher, Emmanuelle

    2010-01-01

    The neuropsychological literature on the processing of emotions in Parkinson's disease (PD) reveals conflicting evidence about the role of the basal ganglia in the recognition of facial emotions. Hence, the present study had two objectives. One was to determine the extent to which the visual processing of emotions and objects differs in PD. The…

  10. Primary brain lymphoma presenting as Parkinson's disease

    Energy Technology Data Exchange (ETDEWEB)

    Sanchez-Guerra, M.; Leno, C.; Berciano, J. [Servicio de Neurologia, Hospital Univ., Santander (Spain); Cerezal, L. [Servicio de Radiologia, Mompia Hospital, Santander (Spain); Diez, C. [Servicio de Neuroradiologia, Hospital Univ., Santander (Spain); Figols, J. [Servicio de Neuropathologia, Hospital Univ., Santander (Spain)

    2001-01-01

    Neoplasm is an uncommon cause of a parkinsonian syndrome. We report a woman with primary brain B-cell lymphoma presenting as Parkinson's disease. After 1 year of the illness, CT and MRI showed lesions without mass effect in the basal ganglia and corpus callosum. The patient did not respond to levodopa and right cerebellar and brain-stem signs appeared, which prompted further neuroimaging, showing an increase in size of the lesions and a right cerebellar and pontine mass. Stereotactic biopsy of the basal ganglia showed high-grade B-cell lymphoma. Despite the basal ganglia frequently being involved in lymphoma of the brain, presentation with typical or atypical parkinsonism is exceptional. (orig.)

  11. Hippocampal Sclerosis but Not Normal Aging or Alzheimer Disease Is Associated With TDP-43 Pathology in the Basal Forebrain of Aged Persons.

    Science.gov (United States)

    Cykowski, Matthew D; Takei, Hidehiro; Van Eldik, Linda J; Schmitt, Frederick A; Jicha, Gregory A; Powell, Suzanne Z; Nelson, Peter T

    2016-05-01

    Transactivating responsive sequence (TAR) DNA-binding protein 43-kDa (TDP-43) pathology has been described in various brain diseases, but the full anatomical distribution and clinical and biological implications of that pathology are incompletely characterized. Here, we describe TDP-43 neuropathology in the basal forebrain, hypothalamus, and adjacent nuclei in 98 individuals (mean age, 86 years; median final mini-mental state examination score, 27). On examination blinded to clinical and pathologic diagnoses, we identified TDP-43 pathology that most frequently involved the ventromedial basal forebrain in 19 individuals (19.4%). As expected, many of these brains had comorbid pathologies including those of Alzheimer disease (AD), Lewy body disease (LBD), and/or hippocampal sclerosis of aging (HS-Aging). The basal forebrain TDP-43 pathology was strongly associated with comorbid HS-Aging (odds ratio = 6.8, p = 0.001), whereas there was no significant association between basal forebrain TDP-43 pathology and either AD or LBD neuropathology. In this sample, there were some cases with apparent preclinical TDP-43 pathology in the basal forebrain that may indicate that this is an early affected area in HS-Aging. We conclude that TDP-43 pathology in the basal forebrain is strongly associated with HS-Aging. These results raise questions about a specific pathogenetic relationship between basal forebrain TDP-43 and non-HS-Aging comorbid diseases (AD and LBD). © 2016 American Association of Neuropathologists, Inc. All rights reserved.

  12. Hippocampal Sclerosis but Not Normal Aging or Alzheimer Disease Is Associated With TDP-43 Pathology in the Basal Forebrain of Aged Persons

    Science.gov (United States)

    Takei, Hidehiro; Van Eldik, Linda J.; Schmitt, Frederick A.; Jicha, Gregory A.; Powell, Suzanne Z.; Nelson, Peter T.

    2016-01-01

    Transactivating responsive sequence (TAR) DNA-binding protein 43-kDa (TDP-43) pathology has been described in various brain diseases, but the full anatomical distribution and clinical and biological implications of that pathology are incompletely characterized. Here, we describe TDP-43 neuropathology in the basal forebrain, hypothalamus, and adjacent nuclei in 98 individuals (mean age, 86 years; median final mini-mental state examination score, 27). On examination blinded to clinical and pathologic diagnoses, we identified TDP-43 pathology that most frequently involved the ventromedial basal forebrain in 19 individuals (19.4%). As expected, many of these brains had comorbid pathologies including those of Alzheimer disease (AD), Lewy body disease (LBD), and/or hippocampal sclerosis of aging (HS-Aging). The basal forebrain TDP-43 pathology was strongly associated with comorbid HS-Aging (odds ratio = 6.8, p = 0.001), whereas there was no significant association between basal forebrain TDP-43 pathology and either AD or LBD neuropathology. In this sample, there were some cases with apparent preclinical TDP-43 pathology in the basal forebrain that may indicate that this is an early affected area in HS-Aging. We conclude that TDP-43 pathology in the basal forebrain is strongly associated with HS-Aging. These results raise questions about a specific pathogenetic relationship between basal forebrain TDP-43 and non-HS-Aging comorbid diseases (AD and LBD). PMID:26971127

  13. Formulaic Language in Parkinson's Disease and Alzheimer's Disease: Complementary Effects of Subcortical and Cortical Dysfunction

    Science.gov (United States)

    Van Lancker Sidtis, Diana; Choi, JiHee; Alken, Amy; Sidtis, John J.

    2015-01-01

    Purpose: The production of formulaic expressions (conversational speech formulas, pause fillers, idioms, and other fixed expressions) is excessive in the left hemisphere and deficient in the right hemisphere and in subcortical stroke. Speakers with Alzheimer's disease (AD), having functional basal ganglia, reveal abnormally high proportions of…

  14. A case of squamous cell carchinoma, basal cell epithelioma and Bowen`s disease arising from chronic radiodermatitis

    Energy Technology Data Exchange (ETDEWEB)

    Matsushima, Hironori; Yamasaki, Kenshi; Hatamochi, Atsushi; Shinkai, Hiroshi [Chiba Univ. (Japan). School of Medicine

    1997-10-01

    We report a case of multiple cancers developing in chronic radiodermatitis. A 43-year-old Japanese male presented with chronic radiodermatitis on both dorsal surfaces of his fingers, following a long-term irradiation by Grenz rays for the treatment of psoriasis vulgaris. At the same site, he developed squamous cell carcinoma and Bowen`s disease 7 years after the last irradiation. Three years later, he noticed a black papule on the dorsal surface of his right middle finger. After a histological examination, this papule diagnosed to be basal cell epithelioma. (author)

  15. Effect of Estradiol on Neurotrophin Receptors in Basal Forebrain Cholinergic Neurons: Relevance for Alzheimer’s Disease

    Directory of Open Access Journals (Sweden)

    Andrea Kwakowsky

    2016-12-01

    Full Text Available The basal forebrain is home to the largest population of cholinergic neurons in the brain. These neurons are involved in a number of cognitive functions including attention, learning and memory. Basal forebrain cholinergic neurons (BFCNs are particularly vulnerable in a number of neurological diseases with the most notable being Alzheimer’s disease, with evidence for a link between decreasing cholinergic markers and the degree of cognitive impairment. The neurotrophin growth factor system is present on these BFCNs and has been shown to promote survival and differentiation on these neurons. Clinical and animal model studies have demonstrated the neuroprotective effects of 17β-estradiol (E2 on neurodegeneration in BFCNs. It is believed that E2 interacts with neurotrophin signaling on cholinergic neurons to mediate these beneficial effects. Evidence presented in our recent study confirms that altering the levels of circulating E2 levels via ovariectomy and E2 replacement significantly affects the expression of the neurotrophin receptors on BFCN. However, we also showed that E2 differentially regulates neurotrophin receptor expression on BFCNs with effects depending on neurotrophin receptor type and neuroanatomical location. In this review, we aim to survey the current literature to understand the influence of E2 on the neurotrophin system, and the receptors and signaling pathways it mediates on BFCN. In addition, we summarize the physiological and pathophysiological significance of E2 actions on the neurotrophin system in BFCN, especially focusing on changes related to Alzheimer’s disease.

  16. Clinical significance of the position of dorsal root ganglia in degenerative lumbar diseases. Correlation between anatomic study and imaging study with MRI

    Energy Technology Data Exchange (ETDEWEB)

    Seki, Masahiro; Kikuchi, Tomiichi [Fukushima Medical Coll., Matsuoka (Japan)

    1995-06-01

    In order to estimate the ralationship between the position of dorsal root ganglia (DRG) and radicular symptoms, anatomical study was done on 81 cadavers, and a clinical study with MRI was done on 20 cases of lumbar disc herniation and 20 of lumbar spondylosis with L{sub 5} radiculopathy. The position of DRG is not related to the occurrence of radicular symptoms in disc herniation, while in lumbar spondylosis proximally placed DRG are related to both of unilateral and bilateral occurrence of redicular symptoms. Unilateral occurrence of radicular symptoms is influenced by surrounding tissues of the nerve root, rather than the position of DRG. (author).

  17. Surgical insights into Parkinson's disease

    OpenAIRE

    Pereira, Erlick A C; Aziz, Tipu Z

    2006-01-01

    Surgery for Parkinson's disease was popularized in the midtwentieth century before the advent of effective medical therapies. Early lesioning treatments contributed to our understanding of the functional anatomy of Parkinson's disease. Observations of the limitations and long-term complications of established pharmacological therapies for Parkinson's disease, together with major contributions from animal research to elucidate the roles of the basal ganglia in movement disorders, inspired a re...

  18. Cerebral blood flow SPECT scanning in cortico-basal degeneration

    International Nuclear Information System (INIS)

    Slawek, J.; Walczak, A.; Krupa-Olchawa, J.; Lass, P.; Dubaniewicz, M.

    1999-01-01

    Idiopathic Parkinson's disease accounts for ca. 75% of all cases of Parkinsonism. Corticobasal degeneration is a relatively rare example of the so-called ''Parkinson-plus'' syndrome. The authors present the case of a 56-year-old woman with rigidity and atypical tremor of upper extremity followed by gait apraxia, dysarthria, bilateral pyramidal signs and myoclonus. There was no improvement after treatment with L-dopa. The disease has progressed, but the patient is still alive. On the basis of clinical data a diagnosis of corticobasal degeneration has been established. Cerebral blood flow SPECT scanning revealed diffuse hypoperfusion of left frontal lobe, antero-inferior part of the left temporal lobe and left basal ganglia. The case illustrates the usefulness of brain SPECT in atypical forma of Parkinson's disease. (author)

  19. Basal Cell Carcinoma: From the Molecular Understanding of the Pathogenesis to Targeted Therapy of Progressive Disease

    Directory of Open Access Journals (Sweden)

    Daniela Göppner

    2011-01-01

    Full Text Available Due to intensified research over the past decade, the Hedgehog (HH pathway has been identified as a pivotal defect implicated in roughly 25% of all cancers. As one of the most frequent cancer worldwide, the development of Basal cell carcinoma (BCC due to activation of the HH pathway has been convincingly demonstrated. Thus the discovery of this central tumor-promoting signalling pathway has not only revolutionized the understanding of BCC carcinogenesis but has also enabled the development of a completely novel therapeutic approach. Targeting just a few of several potential mutations, HH inhibitors such as GDC-0449 achieved already the first promising results in metastatic or locally advanced BCC. This paper summarizes the current understanding of BCC carcinogenesis and describes the current “mechanism-based” therapeutic strategies.

  20. Control of Basal Stem Rot Disease in Oil Palm by Supplementation of Calcium, Copper, and Salicylic Acid

    Science.gov (United States)

    Bivi, M. Shahul Hamid Rahamah; Paiko, Adamu Saidu; Khairulmazmi, Ahmad; Akhtar, M. S.; Idris, Abu Seman

    2016-01-01

    Continuous supplementation of mineral nutrients and salicylic acid (SA) as foliar application could improve efficacy in controlling basal stem rot (BSR) disease in oil palm seedling. It is revealed from the results that the highest disease severity index (58.3%) was recorded in T8 treatments at 9 months after inoculation. The best disease control was achieved by T7 treatments (calcium/copper/SA [Ca/Cu/SA]) (5.0%) followed by T1 (5.5%), T5 (5.8%), T3 (8.3%), T6 (8.3%), T4 (13.3%), and T2 (15.8%) treatments. Continuous supplementation of Ca/Cu/SA was found to be the most effective in controlling the disease and the high performance liquid chromatography results showed the detection of ergosterol at very low concentration in the treated samples. Moreover, the transmission electron microscopy analysis results clearly indicated that T7 treatment was also enhancing lignification, which was responsible for the thickness of the secondary cell walls and middle lamella compared to untreated samples. It was therefore, concluded that continuous supplementation of minerals nutrients and SA could effectively suppress disease severity by reducing ergosterol activity and also improve the process of lignification in the treated plants. Furthermore, this treatment also managed to delay the onset of BSR symptoms and promote the growth of the seedlings and eventually suppress the BSR disease. PMID:27721689

  1. Sequential MRI in a case of Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Tribl, G.G.; Zeitlhofer, J.; Asenbaum, S.; Wessely, P. [Universitaetsklinik fuer Neurologie, Allgemeines Krankenhaus Wien (Austria); Strasser, G.; Prayer, D. [Universitaetsklinik fuer Radiodiagnostik, Allgemeines Krankenhaus Wien (Austria); Jarius, C. [Klinisches Institut fuer Neurologie, Universitaet Wien (Austria)

    2002-03-01

    A 48-year-old man suddenly developed clinically and electroencephalographically nonspecific dementia. On MRI sequences, only diffusion-weighted images (DWI) of the cortex were unequivocally pathological. Obvious atrophy and basal ganglia signal changes appeared only 9 months after the onset. Brain biopsy confirmed Creutzfeldt-Jakob disease (CJD). In rapidly progressive dementia, we recommend DWI for early diagnosis of CJD. (orig.)

  2. Sequential MRI in a case of Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Tribl, G.G.; Zeitlhofer, J.; Asenbaum, S.; Wessely, P.; Strasser, G.; Prayer, D.; Jarius, C.

    2002-01-01

    A 48-year-old man suddenly developed clinically and electroencephalographically nonspecific dementia. On MRI sequences, only diffusion-weighted images (DWI) of the cortex were unequivocally pathological. Obvious atrophy and basal ganglia signal changes appeared only 9 months after the onset. Brain biopsy confirmed Creutzfeldt-Jakob disease (CJD). In rapidly progressive dementia, we recommend DWI for early diagnosis of CJD. (orig.)

  3. Neuroprotection in Parkinson's disease: modafinil and Δ9-tetrahydrocannabinol

    NARCIS (Netherlands)

    Vliet, S.A.M. van

    2007-01-01

    In Parkinson's disease (PD) is the output of the basal ganglia irreversibly affected due to degeneration of the neuromelanin-containing dopaminergic neurons in the substantia nigra pars compacta. This results in manifestation of symptoms including akinesia, postural instability, rigidity and resting

  4. Possible roles of neural gap junctions in Parkinson's disease pathology

    NARCIS (Netherlands)

    Schwab, B.C.; van Wezel, Richard Jack Anton; Heida, Tjitske; van Gils, Stephanus A.

    2013-01-01

    The pathology of Parkinson's disease (PD) is characterized by modified behavior of neuronal networks in the basal ganglia after depletion of dopamine. PD states show bursting neural activity and high synchronization among neurons as well as altered oscillations in local field potentials. These

  5. One-stop-shop treatment for basal cell carcinoma, part of a new disease management strategy.

    Science.gov (United States)

    van der Geer, S; Frunt, M; Romero, H L; Dellaert, N P; Jansen-Vullers, M H; Demeyere, T B J; Neumann, H A M; Krekels, G A M

    2012-09-01

    The number of skin cancer patients, especially patients with basal cell carcinoma (BCC), is rapidly increasing. Resources available at dermato-oncology units have not increased proportionally, which affects the throughput time of patients. To assess the feasibility and safety of implementation of the one-stop-shop concept for the treatment of patients with BCC at a dermato-oncology unit. A pilot study on a one-stop-shop concept for BCC was performed to investigate procedure safety and patient satisfaction. Fresh frozen sections were used to diagnose the tumours, and subsequently treatment with photodynamic therapy or excision was performed on the same day. Time spent in the hospital was measured and questionnaires were used to evaluate patient satisfaction. Sixteen patients, who together had 19 tumours, were included. Diagnoses were made within a mean time of 100 min (range 27-160 min). The mean throughput time was 4 hours and 7 min (range 60-420 min). No complications were observed, and patient satisfaction was high. The one-stop-shop concept for the treatment of skin cancer patients is feasible and efficient for both patients and dermato-oncology units. Further research is necessary to investigate cost-effectiveness when larger patient groups are involved. © 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology.

  6. Chronic Kidney Disease, Basal Insulin Glargine, and Health Outcomes in People with Dysglycemia: The ORIGIN Study.

    Science.gov (United States)

    Papademetriou, Vasilios; Nylen, Eric S; Doumas, Michael; Probstfield, Jeff; Mann, Johannes F E; Gilbert, Richard E; Gerstein, Hertzel C

    2017-12-01

    Early stages of chronic kidney disease are associated with an increased cardiovascular risk in patients with established type 2 diabetes and macrovascular disease. The role of early stages of chronic kidney disease on macrovascular outcomes in prediabetes and early type 2 diabetes mellitus is not known. In the Outcome Reduction with an Initial Glargine Intervention (ORIGIN) trial, the introduction of insulin had no effect on cardiovascular outcomes compared with standard therapy. In this post hoc analysis of ORIGIN, we compared cardiovascular outcomes in subjects without to those with mild (Stages 1-2) or moderate chronic kidney disease (Stage 3). Τwo co-primary composite cardiovascular outcomes were assessed. The first was the composite end point of nonfatal myocardial infarction, nonfatal stroke, or death from cardiovascular causes; and the second was a composite of any of these events plus a revascularization procedure, or hospitalization for heart failure. Several secondary outcomes were prespecified, including microvascular outcomes, incident diabetes, hypoglycemia, weight, and cancers. Complete renal function data were available in 12,174 of 12,537 ORIGIN participants. A total of 8114 (67%) had no chronic kidney disease, while 4060 (33%) had chronic kidney disease stage 1-3. When compared with nonchronic kidney disease participants, the risk of developing the composite primary outcome (nonfatal myocardial infarction, nonfatal stroke, or cardiovascular death) in those with mild to moderate chronic kidney disease was 87% higher; hazard ratio (HR) 1.87; 95% confidence interval (CI), 1.71-2.04 (P chronic kidney disease 1-3 was also associated with a greater than twofold higher risk for both all-cause mortality (HR 2.17; 95% CI, 1.98-2.38; P chronic kidney disease had significantly higher risk for nonfatal myocardial infarction (50%), nonfatal stroke (68%), any stroke (84%), the above composite primary end point plus revascularization or heart failure requiring

  7. Effect of memantine on CBF and CMRO2 in patients with early Parkinson's disease

    DEFF Research Database (Denmark)

    Borghammer, P; Vafaee, M; Ostergaard, K

    2008-01-01

    Objectives –  Parkinson’s disease (PD) may be associated with increased energy metabolism in overactive regions of the basal ganglia. Therefore, we hypothesized that treatment with the N-methyl-d-aspartate receptor (NMDAR) antagonist memantine would decrease regional cerebral blood flow (r......CBF) and oxygen metabolism in the basal ganglia of patients with early-stage PD. Methods –  Quantitative positron emission tomography (PET) recordings were obtained with [15O]water and [15O]oxygen in 10 patients, scanned first in a baseline condition, and again 6 weeks after treatment with a daily dose of 20 mg...... memantine. Dynamic PET data were analyzed using volume of interest and voxel-based approaches. Results –  The treatment evoked rCBF decreases in basal ganglia, and in several frontal cortical areas. The regional cerebral metabolic rate of oxygen (rCMRO2) did not decrease in any of the a priori defined...

  8. Functional characterization and expression of thalamic GABA(B) receptors in a rodent model of Parkinson's disease

    NARCIS (Netherlands)

    de Groote, C; Wullner, U; Loschmann, PA; Luiten, PGM; Klockgether, T

    1999-01-01

    Increased GABAergic neurotransmission of the basal ganglia output nuclei projecting to the motor thalamus is thought to contribute to the pathophysiology of Parkinson's disease. We investigated the functional role of thalamic GABA(B) receptors in a rodent model of Parkinson's disease. First, we

  9. Supervisory and Routine Processes in Noun and Verb Generation in Nondemented Patients with Parkinson's Disease

    Science.gov (United States)

    Crescentini, Cristiano; Mondolo, Federica; Biasutti, Emanuele; Shallice, Tim

    2008-01-01

    Despite the increased comprehension of the role of the basal ganglia in cognitive functions such as learning, attention, and executive functions, the exact implication of these structures in language remains unclear. A specific role of basal ganglia in language has been proposed. Nonetheless, a recent hypothesis gives the basal ganglia a…

  10. Predictive genetic model for levodopa-induced dyskinesia in patients with Parkinson's disease

    NARCIS (Netherlands)

    Ivanova, S.A.; Alifirova, V.M.; Freidin, M.B.; Pozhidaev, I.V.; Fedorenko, O.Y.; Bokhan, N.A.; Zhukova, I.A.; Zhukova, N.G.; Wilffert, B.; Loonen, A.J.M.

    2017-01-01

    Parkinson's disease (PD), a common neurodegenerative disorder caused by the loss of the dopaminergic input to the basal ganglia, is commonly treated with levodopa (L-DOPA). The use of this drug, however, is severely limited by adverse effects. Levodopa-induced dyskinesia (LID) is one of these and

  11. Stimulation of the subthalamic region facilitates the selection and inhibition of motor responses in Parkinson's disease

    NARCIS (Netherlands)

    van den Wildenberg, Wery P. M.; van Boxtel, Geert J. M.; van der Molen, Maurits W.; Bosch, D. Andries; Speelman, Johannes D.; Brunia, Cornelis H. M.

    2006-01-01

    The aim of the present study was to specify the involvement of the basal ganglia in motor response selection and response inhibition. Two samples were studied. The first sample consisted of patients diagnosed with Parkinson's disease (PD) who received deep-brain stimulation (DBS) of the subthalamic

  12. Pallidotomy suppresses beta power in the subthalamic nucleus of Parkinson's disease patients

    NARCIS (Netherlands)

    Contarino, Maria Fiorella; Bour, Lo J.; Bot, Maarten; van den Munckhof, Pepijn; Speelman, Johannes D.; Schuurman, P. Richard; de Bie, Rob M. A.

    2011-01-01

    Parkinsonian patients, who have had a unilateral pallidotomy, may require bilateral deep brain stimulation of the subthalamic nucleus (STN), due to disease progression. The current model of the basal ganglia circuitry does not predict a direct effect of pallidotomy on the neuronal activity of the

  13. Effects of rhythmic stimulus presentation on oscillatory brain activity: the physiology of cueing in Parkinson's disease

    NARCIS (Netherlands)

    Woerd, E.S. te; Oostenveld, R.; Bloem, B.R.; Lange, F.P. de; Praamstra, P.

    2015-01-01

    The basal ganglia play an important role in beat perception and patients with Parkinson's disease (PD) are impaired in perception of beat-based rhythms. Rhythmic cues are nonetheless beneficial in gait rehabilitation, raising the question how rhythm improves movement in PD. We addressed this

  14. A Translational Approach to Vocalization Deficits and Neural Recovery after Behavioral Treatment in Parkinson Disease

    Science.gov (United States)

    Ciucci, Michelle R.; Vinney, Lisa; Wahoske, Emerald J.; Connor, Nadine P.

    2010-01-01

    Parkinson disease is characterized by a complex neuropathological profile that primarily affects dopaminergic neural pathways in the basal ganglia, including pathways that modulate cranial sensorimotor functions such as swallowing, voice and speech. Prior work from our lab has shown that the rat model of unilateral 6-hydroxydopamine infusion to…

  15. Type 2 transglutaminase in Huntington's disease: A double-edged sword with clinical potential

    NARCIS (Netherlands)

    P.G. Mastroberardino (Pier); M. Piacentini

    2010-01-01

    textabstractHuntington's disease (HD) is a dominant genetic neurodegenerative disorder. The pathology affects principally neurons in the basal ganglia circuits and terminates invariably in death. There is compelling necessity for safe and effective therapeutic strategies to arrest, or even retard

  16. Cloning and characterization of a laccase gene from Ganoderma spp. causing basal stem rot disease in coconut

    Directory of Open Access Journals (Sweden)

    Lingan Rajendran

    2008-10-01

    Full Text Available Basal stem rot disease in coconut is caused by the white-rot fungus Ganoderma lucidum, which is soilborne in nature. Its degree of virulence is governed by the activity of the laccase enzyme. Of twenty-five isolates belonging to the genus Ganoderma obtained from different host species, the isolate from Silent Valley (SV showed the greatest laccase activity in vitro, followed by the isolate from Veppankulam (CRS-1. These two isolates also reacted positively in the laccase assay in vitro. The laccase-positive SV and CRS-1 isolates were further amplified by polymerase chain reaction (PCR using degenerate primers for the partial sequence, which showed the fragment size of 200 bp. The highly virulent SV isolate was cloned in a plasmid vector and sequenced. It was confirmed as a partial-length laccase gene and submitted to the GenBank database. The nucleotide sequence of the DNA of this isolate showed high homology with those of the laccase genes of other basidiomycetes.

  17. Frequency-selectivity of a thalamocortical relay neuron during Parkinson's disease and deep brain stimulation: a computational study

    NARCIS (Netherlands)

    Cagnan, Hayriye; Cagnan, H.; Meijer, Hil Gaétan Ellart; van Gils, Stephanus A.; Krupa, M.; Heida, Tjitske; Rudolph, Michelle; Wadman, Wyse J.; Martens, Hubert C.F.

    2009-01-01

    In this computational study, we investigated (i) the functional importance of correlated basal ganglia (BG) activity associated with Parkinson's disease (PD) motor symptoms by analysing the effects of globus pallidus internum (GPi) bursting frequency and synchrony on a thalamocortical (TC) relay

  18. Effects of rhythmic stimulus presentation on oscillatory brain activity: the physiology of cueing in Parkinson’s disease

    NARCIS (Netherlands)

    Woerd, E.S. te; Oostenveld, R.; Bloem, B.R.; Lange, F.P. de; Praamstra, P.

    2015-01-01

    The basal ganglia play an important role in beat perception and patients with Parkinson’s disease (PD) are impaired in perception of beat-based rhythms. Rhythmic cues are nonetheless beneficial in gait rehabilitation, raising the question how rhythm improves movement in PD. We addressed this

  19. Effects of Internal Clock and Memory Disorders on Duration Reproductions and Duration Productions in Patients with Parkinson's Disease

    Science.gov (United States)

    Perbal, S.; Deweer, B.; Pillon, B.; Vidailhet, M.; Dubois, B.; Pouthas, V.

    2005-01-01

    Patients with Parkinson's disease (PD) exhibit deficits in perceptual and motor timing as well as impairments in memory and attentional processes that are related to dysfunction of dopaminergic systems in the basal ganglia. The aim of the present study was to assess the relationships existing between impaired duration judgments and defective…

  20. Quantitative assessment of rest and action tremor and the effect of cueing in Parkinson’s disease patients treated with deep brain stimulation

    NARCIS (Netherlands)

    Heida, Tjitske; Wentink, E.C.; Marani, Enrico

    2011-01-01

    Introduction: In Parkinson’s disease rest and action tremor may occur. High frequency deep brain stimulation in basal ganglia nuclei has proved to be effective in the suppression of tremor. In addition, rhythmic auditory cues have shown to result in improved performance of repetitive movements,

  1. Effects of STN DBS and auditory cueing on the performance of sequential movements and the occurrence of action tremor in Parkinson’s disease

    NARCIS (Netherlands)

    Heida, Tjitske; Wentink, E.C.; Zhao, Yan; Marani, Enrico

    2014-01-01

    Background: Parkinson’s disease (PD) patients show a higher ability to perform repetitive movements when they are cued by external stimuli, suggesting that rhythmic synchronization with an auditory timekeeper can be achieved in the absence of intact basal ganglia function. Deep brain stimulation

  2. Basal Cell Carcinoma

    Science.gov (United States)

    ... Kids’ zone Video library Find a dermatologist Basal cell carcinoma Overview Basal cell carcinoma: This skin cancer ... that has received years of sun exposure. Basal cell carcinoma: Overview Basal cell carcinoma (BCC) is the ...

  3. Evaluation of Basal Serum Adrenocorticotropic Hormone and Cortisol Levels and Their Relationship with Nonalcoholic Fatty Liver Disease in Male Patients with Idiopathic Hypogonadotropic Hypogonadism.

    Science.gov (United States)

    Wang, Wen-Bo; She, Fei; Xie, Li-Fang; Yan, Wen-Hua; Ouyang, Jin-Zhi; Wang, Bao-An; Ma, Hang-Yun; Zang, Li; Mu, Yi-Ming

    2016-05-20

    Prolonged gonadal hormone deficiency in patients with idiopathic hypogonadotropic hypogonadism (IHH) may produce adverse effects on the endocrine homeostasis and metabolism. This study aimed to compare basal serum adrenocorticotropic hormone (ACTH) and cortisol levels between male IHH patients and healthy controls. Moreover, this study compared the basal hypothalamic-pituitary-adrenal (HPA) axis in patients with and without nonalcoholic fatty liver disease (NAFLD), and also evaluated the relationship between basal HPA axis and NAFLD in male IHH patients. This was a retrospective case-control study involving 75 Chinese male IHH patients (mean age 21.4 ± 3.8 years, range 17-30 years) and 135 healthy controls after matching for gender and age. All subjects underwent physical examination and blood testing for serum testosterone, luteinizing hormone, follicle-stimulating hormone, ACTH, and cortisol and biochemical tests. Higher basal serum ACTH levels (8.25 ± 3.78 pmol/L vs. 6.97 ± 2.81 pmol/L) and lower cortisol levels (366.70 ± 142.48 nmol/L vs. 452.82 ± 141.53 nmol/L) were observed in male IHH patients than healthy subjects (all p<0.05). IHH patients also showed higher metabolism parameters and higher prevalence rate of NAFLD (34.9% vs. 4.4%) than the controls (all P < 0.05). Basal serum ACTH (9.91 ± 4.98 pmol/L vs. 7.60 ± 2.96 pmol/L) and dehydroepiandrosterone sulfate (2123.7 ± 925.8 μg/L vs. 1417.1 ± 498.4 μg/L) levels were significantly higher in IHH patients with NAFLD than those without NAFLD (all P < 0.05). We also found that basal serum ACTH levels were positively correlated with NAFLD (r = 0.289,p<0.05) and triglyceride levels (r = 0.268, P< 0.05) in male IHH patients. Furthermore, NAFLD was independently associated with ACTH levels in male IHH patients by multiple linear regression analysis. The male IHH patients showed higher basal serum ACTH levels and lower cortisol levels than matched healthy controls. NAFLD was an independent associated

  4. Neural code alterations and abnormal time patterns in Parkinson’s disease

    Science.gov (United States)

    Andres, Daniela Sabrina; Cerquetti, Daniel; Merello, Marcelo

    2015-04-01

    Objective. The neural code used by the basal ganglia is a current question in neuroscience, relevant for the understanding of the pathophysiology of Parkinson’s disease. While a rate code is known to participate in the communication between the basal ganglia and the motor thalamus/cortex, different lines of evidence have also favored the presence of complex time patterns in the discharge of the basal ganglia. To gain insight into the way the basal ganglia code information, we studied the activity of the globus pallidus pars interna (GPi), an output node of the circuit. Approach. We implemented the 6-hydroxydopamine model of Parkinsonism in Sprague-Dawley rats, and recorded the spontaneous discharge of single GPi neurons, in head-restrained conditions at full alertness. Analyzing the temporal structure function, we looked for characteristic scales in the neuronal discharge of the GPi. Main results. At a low-scale, we observed the presence of dynamic processes, which allow the transmission of time patterns. Conversely, at a middle-scale, stochastic processes force the use of a rate code. Regarding the time patterns transmitted, we measured the word length and found that it is increased in Parkinson’s disease. Furthermore, it showed a positive correlation with the frequency of discharge, indicating that an exacerbation of this abnormal time pattern length can be expected, as the dopamine depletion progresses. Significance. We conclude that a rate code and a time pattern code can co-exist in the basal ganglia at different temporal scales. However, their normal balance is progressively altered and replaced by pathological time patterns in Parkinson’s disease.

  5. Hypoattenuation of the basal nuclei as a sign of propionic acidemia

    International Nuclear Information System (INIS)

    Asenjo, M.; Moron, A.; Marti, M.; Dominguez, F.

    1999-01-01

    The hypoattenuation of the basal ganglia is an uncommon radiological finding that suggests a metabolic or hypoxic disorder. We report a case of propionic acidemia in a five-year-old boy, presenting as a symmetric hypoattenuation of the basal neclei. We discuss this and other causes of this radiological finding, as well as the possible mechanism and underlying pathology. (Author) 17 refs

  6. Momentum-based morphometric analysis with application to Parkinson's disease

    Science.gov (United States)

    Chen, Jingyun; Khan, Ali R.; McKeown, Martin J.; Beg, Mirza F.

    2011-03-01

    We apply the initial momentum shape representation of diffeomorphic metric mapping from a template region of interest (ROI) to a given ROI as a morphometic marker in Parkinson's disease. We used a three-step segmentation-registrationmomentum process to derive feature vectors from ROIs in a group of 42 subjects consisting of 19 Parkinson's Disease (PD) subjects and 23 normal control (NC) subjects. Significant group differences between PD and NC subjects were detected in four basal ganglia structures including the caudate, putamen, thalamus and globus pallidus. The magnitude of regionally significant between-group differences detected ranged between 34-75%. Visualization of the different structural deformation pattern between-groups revealed that some parts of basal ganglia structure actually hypertrophy, presumably as a compensatory response to more widespread atrophy. Our results of both hypertrophy and atrophy in the same structures further demonstrate the importance of morphological measures as opposed to overall volume in the assessment of neurodegenerative disease.

  7. Is there an association between Fahr′s disease and cardiac conduction system disease?: A case report

    Directory of Open Access Journals (Sweden)

    Prashanth Panduranga

    2012-01-01

    Full Text Available Background: Fahr′s disease is a rare neurodegenerative disorder of unknown cause characterized by idiopathic basal ganglia calcification that is associated with neuropsychiatric and cognitive impairment. No case of Fahr′s disease with associated cardiac conduction disease has been described in the literature to date. The objective of this case report was to describe a young female with various cardiac conduction system abnormalities and bilateral basal ganglia calcifica-tion suggestive of Fahr′s disease. Case Report: A 19-year-old female was transferred to our hospital for a pacemaker insertion. Her past medical history included cognitive impairment and asymptomatic congenital complete heart block since birth. Her manifestations in-cluded cognitive impairment, tremors, rigidity, ataxia, bilateral basal ganglia calcification without clinical manifesta-tions of mitochondrial cytopathy. She also had right bundle branch block, left anterior fascicular block, intermittent complete heart block, atrial arrhythmias with advanced atrioventricular blocks and ventricular asystole manifested by Stokes-Adams seizures, which was diagnosed as epilepsy. Conclusions: According to our knowledge, this was the first case report of a su spected association between Fahr′s disease and isolated cardiac conduction system disease. In addition, this case illustrated that in patients with heart blocks and seizures, a diagnosis of epilepsy needs to be made with caution and such patients need further evaluations by a cardiologist or electrophysiologist to consider pacing and prevent future catastrophic events.

  8. New strategies for the treatment of Parkinson's disease hold considerable promise for future management of neurodegenerative disorders

    DEFF Research Database (Denmark)

    Bjarkam, Carsten Reidies; Sørensen, Jens Christian; Sunde, Niels Å

    2001-01-01

    or more. Parkinson's disease ischaracterized by a massive loss of dopaminergicneurons in the substantia nigra, leading tosevere functional disturbance of the neuronalcircuitry in the basal ganglia. A thoroughdescription of basal ganglia circuitry inhealth and disease is presented. We describehow...... the functional disturbances seen inParkinson's disease may be corrected atspecific sites in this circuitry by medicaltreatment or, in advanced stages of Parkinson'sdisease, by neurosurgical methods. The latterinclude lesional surgery, neuraltransplantation and deep brain stimulation,together with future......Neurodegenerative diseases are often consideredincurable with no efficient therapies to modifyor halt the progress of disease, and ultimatelylead to reduced quality of life and to death.Our knowledge of the nervous system in healthand disease has, however, increasedconsiderably during the last...

  9. Thoracoscopic sympathectomy ganglia ablation in the management ...

    African Journals Online (AJOL)

    Thoracoscopic sympathectomy ganglia ablation in the management of palmer hyperhidrosis: A decade experience in a single institution. D Kravarusic, E Freud. Abstract. Background: Hyperhidrosis can cause significant professional and social handicaps. Surgery is the preferred treatment modality for hyperhidrosis.

  10. Basal cell carcinoma

    International Nuclear Information System (INIS)

    Baruah, J.D.

    1981-01-01

    Seven cases of basal cell carcinoma are reported in this paper. The incidence of this disease is two percent of all malignancies seen at the Miraj Medical Centre, Miraj, Maharashtra. There were five male and two female patients in this series. The youngest patient was 40 years old and the oldest 70 years. The average age of the patients was 57.3 years. All the cases in the series had lesions confined to the head and neck region. Radiation therapy was given to all the seven cases which was the primary form of treatment in five cases. In two cases surgical excision had been done and the growth in both the cases had recurred. Radiation therapy is considered more ideal and suitable in the treatment of basal cell carcinomas. (auth.)

  11. Diffusion-Weighted MRI in Creutzfeldt-Jakob Disease: Focus on the Cerebral Cortex and Chronologic Change

    International Nuclear Information System (INIS)

    Lee, Jeong Eun; Song, Chang Joon; Lee, In Ho; Yu, In Kyu; Choi, See Sung

    2010-01-01

    To evaluate high cortical signal intensity and chronologic changes for diffusion-weighted MR imaging (DWI) in sporadic Creutzfeldt-Jakob disease. We retrospectively analyzed the DWI results of 16 patients with probable CJD (according to WHO criteria) and evaluated the distribution, extent and bilaterality of the lesions in the cortex, basal ganglia and thalamus. We also reviewed the chronologic changes of the lesions by evaluating the followup MR examination results in 8 of 16 patients. Cortical abnormalities were present in 15 (94%) of 16 patients. Isolated cortical involvement was present in 6 patients (40%), while the combined involvement of the cortex and basal ganglia was present in 9 patients (60%). The distribution of the lesions was bilateral in 12 patients and predominantly on the right side in 8 patients. Upon follow-up MR imaging, the cortical lesions showed progress in terms of extent and signal intensity. Basal ganglia abnormalities were present in 9 of 15 patients. Moreover, 4 of 6 patients who had no abnormal signal intensity in the basal ganglia on the initial MR imaging results, showed abnormally high signal intensity upon follow-up MR imaging. The characteristically high cortical signal intensities on DWI in an elderly patient with rapidly progressive dementia should point to the diagnosis of early phase CJD and might be useful for the differential diagnosis

  12. Diffusion-Weighted MRI in Creutzfeldt-Jakob Disease: Focus on the Cerebral Cortex and Chronologic Change

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jeong Eun; Song, Chang Joon; Lee, In Ho [Chungnam National University, Daejeon (Korea, Republic of); Yu, In Kyu [Eulji University Hospital, Seoul (Korea, Republic of); Choi, See Sung [Wonkwang University Hospital, Iksan (Korea, Republic of)

    2010-08-15

    To evaluate high cortical signal intensity and chronologic changes for diffusion-weighted MR imaging (DWI) in sporadic Creutzfeldt-Jakob disease. We retrospectively analyzed the DWI results of 16 patients with probable CJD (according to WHO criteria) and evaluated the distribution, extent and bilaterality of the lesions in the cortex, basal ganglia and thalamus. We also reviewed the chronologic changes of the lesions by evaluating the followup MR examination results in 8 of 16 patients. Cortical abnormalities were present in 15 (94%) of 16 patients. Isolated cortical involvement was present in 6 patients (40%), while the combined involvement of the cortex and basal ganglia was present in 9 patients (60%). The distribution of the lesions was bilateral in 12 patients and predominantly on the right side in 8 patients. Upon follow-up MR imaging, the cortical lesions showed progress in terms of extent and signal intensity. Basal ganglia abnormalities were present in 9 of 15 patients. Moreover, 4 of 6 patients who had no abnormal signal intensity in the basal ganglia on the initial MR imaging results, showed abnormally high signal intensity upon follow-up MR imaging. The characteristically high cortical signal intensities on DWI in an elderly patient with rapidly progressive dementia should point to the diagnosis of early phase CJD and might be useful for the differential diagnosis.

  13. GENETICS AND NEUROPATHOLOGY OF HUNTINGTON’S DISEASE

    Science.gov (United States)

    Reiner, Anton; Dragatsis, Ioannis; Dietrich, Paula

    2015-01-01

    Huntington’s disease (HD) is an autosomal dominant progressive neurodegenerative disorder that prominently affects the basal ganglia, leading to affective, cognitive, behavioral and motor decline. The basis of HD is a CAG repeat expansion to >35 CAG in a gene that codes for a ubiquitous protein known as huntingtin, resulting in an expanded N-terminal polyglutamine tract. The size of the expansion is correlated with disease severity, with increasing CAG accelerating the age of onset. A variety of possibilities have been proposed as to the mechanism by which the mutation causes preferential injury to the basal ganglia. The present chapter provides a basic overview of the genetics and pathology of HD. PMID:21907094

  14. Combined Wnt/β-Catenin, Met, and CXCL12/CXCR4 Signals Characterize Basal Breast Cancer and Predict Disease Outcome

    Directory of Open Access Journals (Sweden)

    Jane D. Holland

    2013-12-01

    Full Text Available Prognosis for patients with estrogen-receptor (ER-negative basal breast cancer is poor, and chemotherapy is currently the best therapeutic option. We have generated a compound-mutant mouse model combining the activation of β-catenin and HGF (Wnt-Met signaling, which produced rapidly growing basal mammary gland tumors. We identified the chemokine system CXCL12/CXCR4 as a crucial driver of Wnt-Met tumors, given that compound-mutant mice also deficient in the CXCR4 gene were tumor resistant. Wnt-Met activation rapidly expanded a population of cancer-propagating cells, in which the two signaling systems control different functions, self-renewal and differentiation. Molecular therapy targeting Wnt, Met, and CXCR4 in mice significantly delayed tumor development. The expression of a Wnt-Met 322 gene signature was found to be predictive of poor survival of human patients with ER-negative breast cancers. Thus, targeting CXCR4 and its upstream activators, Wnt and Met, might provide an efficient strategy for breast cancer treatment.

  15. Automatic activation of motor programs by object affordances in patients with Parkinson's disease

    OpenAIRE

    Oguro, Hiroaki; Ward, Robert; Bracewel, Martyn; Hindle, John; Rafal, Robert

    2009-01-01

    Clinical observations of kinesia paradoxica and freezing in patients with Parkinson's disease suggest that the automatic activation of motor programmes by visual stimuli may not require intact basal ganglia function, and that an increased sensitivity to such object affordances may contribute to some symptoms of the disease. Employing a paradigm that measures the degree of interference from object affordances on voluntary actions, we confirm that activation of object affordances are preserved ...

  16. Fahr′s disease Presenting with Aneurysmal Subarachnoid Hemorrhage

    Directory of Open Access Journals (Sweden)

    Hosam Al-Jehani

    2012-01-01

    Full Text Available Fahr′s disease is a rare disorder of slowly progressive cognitive, psychiatric, and motor decline associated with idiopathic basal ganglia calcification (IBGC and widespread calcification in the brain and cerebellum. Acute presentation of IBGC is most often as a seizure disorder; however, we present a case of an acute IBCG presentation in which the cause of the deterioration was an aneurysmal subarachnoid hemorrhage.

  17. Whole transcriptome expression of trigeminal ganglia compared to dorsal root ganglia in Rattus Norvegicus

    DEFF Research Database (Denmark)

    Kogelman, Lisette Johanna Antonia; Christensen, Rikke Elgaard; Pedersen, Sara Hougaard

    2017-01-01

    The trigeminal ganglia (TG) subserving the head and the dorsal root ganglia (DRG) subserving the rest of the body are homologous handling sensory neurons. Differences exist, as a number of signaling substances cause headache but no pain in the rest of the body. To date, very few genes involved in...

  18. Cardiovascular effects of basal insulins

    Directory of Open Access Journals (Sweden)

    Mannucci E

    2015-07-01

    Full Text Available Edoardo Mannucci,1 Stefano Giannini,2 Ilaria Dicembrini1 1Diabetes Agency, Careggi Teaching Hospital, Florence, 2Section of Endocrinology, Department of Biomedical Clinical and Experimental Sciences, University of Florence and Careggi University Hospital, Florence, Italy Abstract: Basal insulin is an important component of treatment for both type 1 and type 2 diabetes. One of the principal aims of treatment in patients with diabetes is the prevention of diabetic complications, including cardiovascular disease. There is some evidence, although controversial, that attainment of good glycemic control reduces long-term cardiovascular risk in both type 1 and type 2 diabetes. The aim of this review is to provide an overview of the potential cardiovascular safety of the different available preparations of basal insulin. Current basal insulin (neutral protamine Hagedorn [NPH], or isophane and basal insulin analogs (glargine, detemir, and the more recent degludec differ essentially by various measures of pharmacokinetic and pharmacodynamic effects in the bloodstream, presence and persistence of peak action, and within-subject variability in the glucose-lowering response. The currently available data show that basal insulin analogs have a lower risk of hypoglycemia than NPH human insulin, in both type 1 and type 2 diabetes, then excluding additional harmful effects on the cardiovascular system mediated by activation of the adrenergic system. Given that no biological rationale for a possible difference in cardiovascular effect of basal insulins has been proposed so far, available meta-analyses of publicly disclosed randomized controlled trials do not show any signal of increased risk of major cardiovascular events between the different basal insulin analogs. However, the number of available cardiovascular events in these trials is very small, preventing any clear-cut conclusion. The results of an ongoing clinical trial comparing glargine and degludec with

  19. STN-stimulation in Parkinson's disease restores striatal inhibition of thalamocortical projection

    DEFF Research Database (Denmark)

    Geday, Jacob; Østergaard, Karen; Johnsen, Erik

    2009-01-01

    To test the hypothesis that deep brain stimulation of the subthalamic nucleus (STN) restores the inhibitory output to the striatothalamocortical loop in Parkinson's disease, we obtained functional brain images of blood flow in 10 STN-stimulated patients with Parkinson's disease. Patients were...... in the STN and in the left nucleus lentiformis. Conversely, flow declined in the left supplementary motor area (BA 6), ventrolateral nucleus of the left thalamus, and right cerebellum. Activation of the basal ganglia and deactivation of supplementary motor area and thalamus were both correlated...... with the improvement of motor function. The result is consistent with the explanation that stimulation in resting patients raises output from the STN with activation of the inhibitory basal ganglia output nuclei and subsequent deactivation of the thalamic anteroventral and ventrolateral nuclei and the supplementary...

  20. Tay-Sachs disease: progression of changes on neuroimaging in four cases

    Energy Technology Data Exchange (ETDEWEB)

    Fukumizu, M.; Yoshikawa, H.; Kurokawa, T. (National Center Hospital for Mental, Nervous, and Muscular Disorders, Tokyo (Japan). Div. of Child Neurology); Takashima, S. (National Inst. of Neuroscience, National Center of Neurology and Psychiatry, Tokyo (Japan). Div. of Mental Retardation and Birth Defect Research); Sakuragawa, N. (National Inst. of Neuroscience, National Center of Neurology and Psychiatry, Tokyo (Japan). Div. of Inherited Metabolic Disease)

    1992-11-01

    The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter. (orig.).

  1. l-Dopa responsiveness is associated with distinctive connectivity patterns in advanced Parkinson's disease.

    Science.gov (United States)

    Akram, Harith; Wu, Chengyuan; Hyam, Jonathan; Foltynie, Thomas; Limousin, Patricia; De Vita, Enrico; Yousry, Tarek; Jahanshahi, Marjan; Hariz, Marwan; Behrens, Timothy; Ashburner, John; Zrinzo, Ludvic

    2017-06-01

    Neuronal loss and dopamine depletion alter motor signal processing between cortical motor areas, basal ganglia, and the thalamus, resulting in the motor manifestations of Parkinson's disease. Dopamine replacement therapy can reverse these manifestations with varying degrees of improvement. To evaluate functional connectivity in patients with advanced Parkinson's disease and changes in functional connectivity in relation to the degree of response to l-dopa, 19 patients with advanced Parkinson's disease underwent resting-state functional magnetic resonance imaging in the on-medication state. Scans were obtained on a 3-Tesla scanner in 3 × 3 × 2.5 mm 3 voxels. Seed-based bivariate regression analyses were carried out with atlas-defined basal ganglia regions as seeds, to explore relationships between functio