WorldWideScience

Sample records for basal ganglia diseases

  1. Cortico-Basal Ganglia Circuit Function in Psychiatric Disease.

    Science.gov (United States)

    Gunaydin, Lisa A; Kreitzer, Anatol C

    2016-01-01

    Circuit dysfunction models of psychiatric disease posit that pathological behavior results from abnormal patterns of electrical activity in specific cells and circuits in the brain. Many psychiatric disorders are associated with abnormal activity in the prefrontal cortex and in the basal ganglia, a set of subcortical nuclei implicated in cognitive and motor control. Here we discuss the role of the basal ganglia and connected prefrontal regions in the etiology and treatment of obsessive-compulsive disorder, anxiety, and depression, emphasizing mechanistic work in rodent behavioral models to dissect causal cortico-basal ganglia circuits underlying discrete behavioral symptom domains relevant to these complex disorders. PMID:26667072

  2. Synchronizing activity of basal ganglia and pathophysiology of Parkinson's disease.

    Science.gov (United States)

    Heimer, G; Rivlin, M; Israel, Z; Bergman, H

    2006-01-01

    Early physiological studies emphasized changes in the discharge rate of basal ganglia in the pathophysiology of Parkinson's disease (PD), whereas recent studies stressed the role of the abnormal oscillatory activity and neuronal synchronization of pallidal cells. However, human observations cast doubt on the synchronization hypothesis since increased synchronization may be an epi-phenomenon of the tremor or of independent oscillators with similar frequency. Here, we show that modern actor/ critic models of the basal ganglia predict the emergence of synchronized activity in PD and that significant non-oscillatory and oscillatory correlations are found in MPTP primates. We conclude that the normal fluctuation of basal ganglia dopamine levels combined with local cortico-striatal learning rules lead to noncorrelated activity in the pallidum. Dopamine depletion, as in PD, results in correlated pallidal activity, and reduced information capacity. We therefore suggest that future deep brain stimulation (DBS) algorithms may be improved by desynchronizing pallidal activity. PMID:17017503

  3. Familial idiopathic basal ganglia calcification (Fahr’s disease)

    Science.gov (United States)

    Mufaddel, Amir A.; Al-Hassani, Ghanem A.

    2014-01-01

    Familial idiopathic basal ganglia calcification (Fahr’s disease) is a rare neurodegenerative disorder characterized by symmetrical and bilateral calcification of the basal ganglia. Calcifications may also occur in other brain regions such as dentate nucleus, thalamus, and cerebral cortex. Both familial and non-familial cases of Fahr’s disease have been reported, predominantly with autosomal-dominant fashion. The disease has a wide range of clinical presentations, predominantly with neuropsychiatric features and movement disorders. Psychiatric features reported in the literature include: cognitive impairment, depression, hallucinations, delusions, manic symptoms, anxiety, schizophrenia-like psychosis, and personality change. Other clinical features include: Parkinsonism, ataxia, headache, seizures, vertigo, stroke-like events, orthostatic hypotension, tremor, dysarthria, and paresis. Fahr’s disease should be considered in the differential diagnosis of psychiatric symptoms, particularly when associated with movement disorder. The disease should be differentiated from other conditions that can cause intracranial calcification. No specific treatment is currently available. Further research is needed to bridge the gap existing in our current knowledge of the prevalence, etiology, symptoms, and treatment of Fahr’s disease. PMID:24983277

  4. Familial idiopathic basal ganglia calcification (Fahr`s disease).

    Science.gov (United States)

    Mufaddel, Amir A; Al-Hassani, Ghanem A

    2014-07-01

    Familial idiopathic basal ganglia calcification (Fahr`s disease) is a rare neurodegenerative disorder characterized by symmetrical and bilateral calcification of the basal ganglia. Calcifications may also occur in other brain regions such as dentate nucleus, thalamus, and cerebral cortex. Both familial and non-familial cases of Fahr`s disease have been reported, predominantly with autosomal-dominant fashion. The disease has a wide range of clinical presentations, predominantly with neuropsychiatric features and movement disorders. Psychiatric features reported in the literature include: cognitive impairment, depression, hallucinations, delusions, manic symptoms, anxiety, schizophrenia-like psychosis, and personality change. Other clinical features include: Parkinsonism, ataxia, headache, seizures, vertigo, stroke-like events, orthostatic hypotension, tremor, dysarthria, and paresis. Fahr`s disease should be considered in the differential diagnosis of psychiatric symptoms, particularly when associated with movement disorder. The disease should be differentiated from other conditions that can cause intracranial calcification. No specific treatment is currently available. Further research is needed to bridge the gap existing in our current knowledge of the prevalence, etiology, symptoms, and treatment of Fahr`s disease.

  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. Correlation transfer from basal ganglia to thalamus in Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Pamela eReitsma

    2011-12-01

    Full Text Available Spike trains from neurons in the basal ganglia of parkinsonian primatesshow increased pairwise correlations, oscillatory activity, and burstrate compared to those from neurons recorded during normal brainactivity. However, it is not known how these changes affect the behaviorof downstream thalamic neurons. To understand how patterns ofbasal ganglia population activity may affect thalamic spike statistics,we study pairs of model thalamocortical (TC relay neurons receivingcorrelated inhibitory input from the internal segment of the globus pallidus(GPi, a primary output nucleus of the basal ganglia. We observethat the strength of correlations of TC neuron spike trains increaseswith the GPi correlation level, and bursty firing patterns such as thoseseen in the parkinsonian GPi allow for stronger transfer of correlationsthan do firing patterns found under normal conditions. We also showthat the T-current in the TC neurons does not significantly affect correlationtransfer, despite its pronounced effects on spiking. Oscillatoryfiring patterns in GPi are shown to affect the timescale at which correlationsare best transferred through the system. To explain this lastresult, we analytically compute the spike count correlation coefficientfor oscillatory cases in a reduced point process model. Our analysisindicates that the dependence of the timescale of correlation transfer isrobust to different levels of input spike and rate correlations and arisesdue to differences in instantaneous spike correlations, even when thelong timescale rhythmic modulations of neurons are identical. Overall,these results show that parkinsonian firing patterns in GPi do affectthe transfer of correlations to the thalamus.

  7. 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

  8. Positron emission tomography and basal ganglia functions

    International Nuclear Information System (INIS)

    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

  9. Basal ganglia modulation of thalamocortical relay in Parkinson’s disease and dystonia

    Directory of Open Access Journals (Sweden)

    Yixin eGuo

    2013-09-01

    Full Text Available 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 the 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 maybe involve improvement of TC relay fidelity.

  10. 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.

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

    International Nuclear Information System (INIS)

    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.)

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

    International Nuclear Information System (INIS)

    We detected crossed cerebellar as well as uncrossed basal ganglia and thalamic diaschisis in Alzheimer's disease by positron emission tomography (PET) using 18F-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

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

    International Nuclear Information System (INIS)

    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 D2 receptor SPECT findings in selected movement disorders. (orig.)

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

    Science.gov (United States)

    Foerde, Karin; Shohamy, Daphna

    2011-11-01

    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

  15. 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 sPARKIN......-MC and to a lesser extent in iPD. However, a slight increase in GMV was also found in the right globus pallidus externus in sPARKIN-MC and in the right putamen in iPD. This may reflect a structural correlate of functional compensation that can only partially be maintained when nigrostriatal neurodegeneration becomes...

  16. 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

    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 movement

  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

    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

  18. Somatotopic organization of the primate basal ganglia

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    Atsushi eNambu

    2011-04-01

    Full Text Available Somatotopic organization is a fundamental and key concept to understand how the cortico-basal ganglia loop works. It is also indispensable knowledge to perform stereotaxic surgery for movement disorders. Here I would like to describe the somatotopic organization of the basal ganglia, which consist of the striatum, subthalamic nucleus, globus pallidus and substantia nigra. Projections from motor cortical regions representing different body parts terminate in different regions of these nuclei. Basal ganglia neurons respond not only to the stimulation of the corresponding regions of the motor cortices, but also to active and passive movements of the corresponding body parts. On the basis of these anatomical and physiological findings, somatotopic organization can be identified in the motor territories of these nuclei in the basal ganglia. In addition, projections from functionally interrelated cortical areas partially converge through the cortico-basal ganglia loop, but nevertheless the somatotopy is still preserved. Disorganized somatotopy may explain, at least in part, the pathophysiology of movement disorders, such as Parkinson’s disease and dystonia.

  19. Basal ganglia dysfunction

    Science.gov (United States)

    ... overdose Head injury Infection Liver disease Metabolic problems Multiple sclerosis Poisoning with copper, manganese, or other heavy metals Side effects of certain medications Stroke Tumors Many brain disorders are associated with ...

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

    OpenAIRE

    Foerde, Karin; Shohamy, Daphna

    2011-01-01

    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 ...

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

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    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

  2. Basal ganglia lesions in children and adults.

    Science.gov (United States)

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

    2013-05-01

    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. PMID:23313708

  3. Striatal plasticity and basal ganglia circuit function

    OpenAIRE

    Kreitzer, Anatol C.; Malenka, Robert C.

    2008-01-01

    The dorsal striatum, which consists of the caudate and putamen, is the gateway to the basal ganglia. It receives convergent excitatory afferents from cortex and thalamus and forms the origin of the direct and indirect pathways—distinct basal ganglia circuits involved in motor control. It is also a major site of activity-dependent synaptic plasticity. Striatal plasticity alters the transfer of information throughout basal ganglia circuits and may represent a key neural substrate for adaptive m...

  4. Striatal plasticity and basal ganglia circuit function.

    Science.gov (United States)

    Kreitzer, Anatol C; Malenka, Robert C

    2008-11-26

    The dorsal striatum, which consists of the caudate and putamen, is the gateway to the basal ganglia. It receives convergent excitatory afferents from cortex and thalamus and forms the origin of the direct and indirect pathways, which are distinct basal ganglia circuits involved in motor control. It is also a major site of activity-dependent synaptic plasticity. Striatal plasticity alters the transfer of information throughout basal ganglia circuits and may represent a key neural substrate for adaptive motor control and procedural memory. Here, we review current understanding of synaptic plasticity in the striatum and its role in the physiology and pathophysiology of basal ganglia function. PMID:19038213

  5. Whole exome sequencing reveals compound heterozygous mutations in SLC19A3 causing biotin-thiamine responsive basal ganglia disease

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    L.J. Sremba

    2014-01-01

    Full Text Available Biotin-thiamine responsive basal ganglia disease (BTBGD is a rare metabolic condition caused by mutations in the SLC19A3 gene. BTBGD presents with encephalopathy and significant disease progression when not treated with biotin and/or thiamine. We present a patient of Mexican and European ancestry diagnosed with BTBGD found to have compound heterozygous frameshift mutations, one novel. Our report adds to the genotype-phenotype correlation, highlighting the clinical importance of considering SLC19A3 gene defects as part of the differential diagnosis for Leigh syndrome.

  6. The basal ganglia communicate with the cerebellum.

    Science.gov (United States)

    Bostan, Andreea C; Dum, Richard P; Strick, Peter L

    2010-05-01

    The basal ganglia and cerebellum are major subcortical structures that influence not only movement, but putatively also cognition and affect. Both structures receive input from and send output to the cerebral cortex. Thus, the basal ganglia and cerebellum form multisynaptic loops with the cerebral cortex. Basal ganglia and cerebellar loops have been assumed to be anatomically separate and to perform distinct functional operations. We investigated whether there is any direct route for basal ganglia output to influence cerebellar function that is independent of the cerebral cortex. We injected rabies virus (RV) into selected regions of the cerebellar cortex in cebus monkeys and used retrograde transneuronal transport of the virus to determine the origin of multisynaptic inputs to the injection sites. We found that the subthalamic nucleus of the basal ganglia has a substantial disynaptic projection to the cerebellar cortex. This pathway provides a means for both normal and abnormal signals from the basal ganglia to influence cerebellar function. We previously showed that the dentate nucleus of the cerebellum has a disynaptic projection to an input stage of basal ganglia processing, the striatum. Taken together these results provide the anatomical substrate for substantial two-way communication between the basal ganglia and cerebellum. Thus, the two subcortical structures may be linked together to form an integrated functional network. PMID:20404184

  7. Basal ganglia lesions in children and adults

    International Nuclear Information System (INIS)

    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

  8. 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.

  9. Functional Neuroanatomy of the Basal Ganglia

    OpenAIRE

    Lanciego, José L.; Luquin, Natasha; Obeso, José A.

    2012-01-01

    The “basal ganglia” refers to a group of subcortical nuclei responsible primarily for motor control, as well as other roles such as motor learning, executive functions and behaviors, and emotions. Proposed more than two decades ago, the classical basal ganglia model shows how information flows through the basal ganglia back to the cortex through two pathways with opposing effects for the proper execution of movement. Although much of the model has remained, the model has been modified and amp...

  10. 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.

  11. Hypernasality associated with basal ganglia dysfunction: evidence from Parkinson’s disease and Huntington’s disease

    Science.gov (United States)

    Novotný, Michal; Čmejla, Roman; Růžičková, Hana; Klempíř, Jiří; Růžička, Evžen

    2016-01-01

    Background Although increased nasality can originate from basal ganglia dysfunction, data regarding hypernasality in Parkinson’s disease (PD) and Huntington’s disease (HD) are very sparse. The aim of the current study was to analyze acoustic and perceptual correlates of velopharyngeal seal closure in 37 PD and 37 HD participants in comparison to 37 healthy control speakers. Methods Acoustical analysis was based on sustained phonation of the vowel /i/ and perceptual analysis was based on monologue. Perceptual analysis was performed by 10 raters using The Great Ormond Street Speech Assessment ’98. Acoustic parameters related to changes in a 1/3-octave band centered on 1 kHz were proposed to reflect nasality level and behavior through utterance. Results Perceptual analysis showed the occurrence of mild to moderate hypernasality in 65% of PD, 89% of HD and 22% of control speakers. Based on acoustic analyses, 27% of PD, 54% of HD and 19% of control speakers showed an increased occurrence of hypernasality. In addition, 78% of HD patients demonstrated a high occurrence of intermittent hypernasality. Further results indicated relationships between the acoustic parameter representing fluctuation of nasality and perceptual assessment (r = 0.51, p Huntington Disease Rating Scale chorea composite subscore (r = 0.42, p = 0.01). Conclusions In conclusion the acoustic assessment showed that abnormal nasality was not a common feature of PD, whereas patients with HD manifested intermittent hypernasality associated with chorea. PMID:27703866

  12. 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. PMID:26996632

  13. 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

  14. 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.

  15. Basal ganglia orient eyes to reward.

    Science.gov (United States)

    Hikosaka, Okihide; Nakamura, Kae; Nakahara, Hiroyuki

    2006-02-01

    Expectation of reward motivates our behaviors and influences our decisions. Indeed, neuronal activity in many brain areas is modulated by expected reward. However, it is still unclear where and how the reward-dependent modulation of neuronal activity occurs and how the reward-modulated signal is transformed into motor outputs. Recent studies suggest an important role of the basal ganglia. Sensorimotor/cognitive activities of neurons in the basal ganglia are strongly modulated by expected reward. Through their abundant outputs to the brain stem motor areas and the thalamocortical circuits, the basal ganglia appear capable of producing body movements based on expected reward. A good behavioral measure to test this hypothesis is saccadic eye movement because its brain stem mechanism has been extensively studied. Studies from our laboratory suggest that the basal ganglia play a key role in guiding the gaze to the location where reward is available. Neurons in the caudate nucleus and the substantia nigra pars reticulata are extremely sensitive to the positional difference in expected reward, which leads to a bias in excitability between the superior colliculi such that the saccade to the to-be-rewarded position occurs more quickly. It is suggested that the reward modulation occurs in the caudate where cortical inputs carrying spatial signals and dopaminergic inputs carrying reward-related signals are integrated. These data support a specific form of reinforcement learning theories, but also suggest further refinement of the theory.

  16. Reward functions of the basal ganglia.

    Science.gov (United States)

    Schultz, Wolfram

    2016-07-01

    Besides their fundamental movement function evidenced by Parkinsonian deficits, the basal ganglia are involved in processing closely linked non-motor, cognitive and reward information. This review describes the reward functions of three brain structures that are major components of the basal ganglia or are closely associated with the basal ganglia, namely midbrain dopamine neurons, pedunculopontine nucleus, and striatum (caudate nucleus, putamen, nucleus accumbens). Rewards are involved in learning (positive reinforcement), approach behavior, economic choices and positive emotions. The response of dopamine neurons to rewards consists of an early detection component and a subsequent reward component that reflects a prediction error in economic utility, but is unrelated to movement. Dopamine activations to non-rewarded or aversive stimuli reflect physical impact, but not punishment. Neurons in pedunculopontine nucleus project their axons to dopamine neurons and process sensory stimuli, movements and rewards and reward-predicting stimuli without coding outright reward prediction errors. Neurons in striatum, besides their pronounced movement relationships, process rewards irrespective of sensory and motor aspects, integrate reward information into movement activity, code the reward value of individual actions, change their reward-related activity during learning, and code own reward in social situations depending on whose action produces the reward. These data demonstrate a variety of well-characterized reward processes in specific basal ganglia nuclei consistent with an important function in non-motor aspects of motivated behavior. PMID:26838982

  17. Dopamine-glutamate interactions in the basal ganglia.

    Science.gov (United States)

    Schmidt, W J

    1998-01-01

    In an attempt to formulate a working hypothesis of basal-ganglia functions, arguments are considered suggesting that the basal ganglia are involved in a process of response selection i.e. in the facilitation of "wanted" and in the suppression of "unwanted" behaviour. The meso-accumbal dopamine-system is considered to mediate natural and drug-induced reward and sensitization. The meso-striatal dopamine-system seems to fulfill similar functions: It may mediate reinforcement which strengthens a given behaviour when elicited subsequently, but which is not experienced as reward or hedonia. Glutamate as the transmitter of the corticofugal projections to the basal ganglia nuclei and of the subthalamic neurons is critically involved in basal ganglia functions and dysfunctions; for example Parkinson's disease can be considered to be a secondary hyperglutamatergic disease. Additionally, glutamate is an essential factor in the plasticity response of the basal-ganglia. However, opposite to previous suggestions, the NMDA-receptor blocker MK-801 does not prevent psychostimulant- nor morphine-induced day to day increase (sensitization) of locomotion. Also the day to day increase of haloperidol-induced catalepsy was not prevented by MK-801. PMID:9871434

  18. 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.

  19. The role of exercise in facilitating basal ganglia function in Parkinson’s disease

    OpenAIRE

    Petzinger, Giselle M.; Fisher, Beth E.; Akopian, Garnik; Holschneider, Daniel P.; Wood, Ruth; Walsh, John P.; Lund, Brett; Meshul, Charles; Vuckovic, Marta; Jakowec, Michael W.

    2011-01-01

    Epidemiological and clinical studies have suggested that exercise is beneficial for patients with Parkinson’s disease (PD). Through research in normal (noninjured) animals, neuroscientists have begun to understand the mechanisms in the brain by which behavioral training and exercise facilitates improvement in motor behavior through modulation of neuronal function and structure, called experience-dependent neuroplasticity. Recent studies are beginning to reveal molecules and downstream signali...

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

    Directory of Open Access Journals (Sweden)

    Nicolas Maurice

    Full Text Available 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.

  1. Mössbauer spectroscopy of Basal Ganglia

    Energy Technology Data Exchange (ETDEWEB)

    Miglierini, Marcel, E-mail: marcel.miglierini@stuba.sk [Institute of Nuclear and Physical Engineering, Faculty of Electrical Engineering and Information Technology, Slovak University of Technology, Ilkovičova 3, 812 19 Bratislava, Slovakia and Regional Centre of Advanced Technologies and Materials (Czech Republic); Lančok, Adriana [Institute of Inorganic Chemistry AS CR, v. v. i., 250 68 Husinec-Řež 1001 (Czech Republic); Kopáni, Martin [Institute of Medical Physics, Biophysics, Informatics and Telemedicine, Faculty of Medicine, Comenius University, Sasinkova 2, 811 08 Bratislava (Slovakia); Boča, Roman [Department of Chemistry, Faculty of Natural Sciences, University of SS. Cyril and Methodius, 917 01 Trnava (Slovakia)

    2014-10-27

    Chemical states, structural arrangement, and magnetic features of iron deposits in biological tissue of Basal Ganglia are characterized. The methods of SQUID magnetometry and electron microscopy are employed. {sup 57}Fe Mössbauer spectroscopy is used as a principal method of investigation. Though electron microscopy has unveiled robust crystals (1-3 μm in size) of iron oxides, they are not manifested in the corresponding {sup 57}Fe Mössbauer spectra. The latter were acquired at 300 K and 4.2 K and resemble ferritin-like behavior.

  2. Sequence learning in a model of the basal ganglia

    OpenAIRE

    Søiland, Stian

    2006-01-01

    This thesis presents a computational model of the basal ganglia that is able to learn sequences and perform action selection. The basal ganglia is a set of structures in the human brain involved in everything from action selection to reinforcement learning, inspiring research in psychology, neuroscience and computer science. Two temporal difference models of the basal ganglia based on previous work have been reimplemented. Several experiments and analyses help understand and describe the or...

  3. Covert skill learning in a cortical-basal ganglia circuit

    OpenAIRE

    Charlesworth, JD; Warren, TL; Brainard, MS

    2012-01-01

    We learn complex skills such as speech and dance through a gradual process of trial and error. Cortical-basal ganglia circuits have an important yet unresolved function in this trial-and-error skill learning; influential ' actor-models propose that basal ganglia circuits generate a variety of behaviours during training and learn to implement the successful behaviours in their repertoire. Here we show that the anterior forebrain pathway (AFP), a cortical-basal ganglia circuit, contributes to s...

  4. Mineralizing angiopathy with basal ganglia stroke in an infant

    Directory of Open Access Journals (Sweden)

    Puneet Jain

    2015-01-01

    Full Text Available Basal ganglia stroke is known following trivial head trauma. Recently a distinct clinic-radiological entity termed ′mineralizing angiopathy′ was described. We report an infant who developed basal ganglia stroke following trivial fall. His clinic-radiological features are described.

  5. Youth hypertension cerebral hemorrhage in basal ganglia surgery operation analysis

    Institute of Scientific and Technical Information of China (English)

    Qi-Hua Wang; Da-Shuang Lu; Jie Cui; Bo-Lin Qiao; Jing-Chun Wang

    2016-01-01

    Objective:Discuss surgical treatment of youth hypertension cerebral hemorrhage in basal ganglia.Methods:Retrospective analysis from January 2012 to April 2015 were adopted to bone flap craniotomy decompression for removal of hematoma and drainage drilling two kinds of surgical treatment of 46 cases of young patients with hypertension cerebral hemorrhage in basal ganglia.Results:Surgical operation, 28 patients postoperative review head CT, no further hemorrhage cases, residual hematoma volume 2-6 mL. Drilling drainage in the treatment of 18 patients, 1 case was bleeding again given surgical operation to remove the hematoma and the rest of the 17 cases without bleeding again, after 3 d, 17 cases of patients of postoperative hematoma drainage thoroughly. After 6 months, 46 cases of patients with postoperative review, GOS score light disability 9 cases, moderate disability 33 cases, 4 cases were severely disabled, curative effect is satisfied.Conclusions:Two kinds of operative methods each have advantages and disadvantages, young patients with hypertension cerebral hemorrhage in basal ganglia should according to patients' disease progression after speed, on admission patient's state of consciousness and head CT measured on admission hematoma volume, respectively.

  6. Proactive selective response suppression is implemented via the basal ganglia.

    Science.gov (United States)

    Majid, D S Adnan; Cai, Weidong; Corey-Bloom, Jody; Aron, Adam R

    2013-08-14

    In the welter of everyday life, people can stop particular response tendencies without affecting others. A key requirement for such selective suppression is that subjects know in advance which responses need stopping. We hypothesized that proactively setting up and implementing selective suppression relies on the basal ganglia and, specifically, regions consistent with the inhibitory indirect pathway for which there is scant functional evidence in humans. Consistent with this hypothesis, we show, first, that the degree of proactive motor suppression when preparing to stop selectively (indexed by transcranial magnetic stimulation) corresponds to striatal, pallidal, and frontal activation (indexed by functional MRI). Second, we demonstrate that greater striatal activation at the time of selective stopping correlates with greater behavioral selectivity. Third, we show that people with striatal and pallidal volume reductions (those with premanifest Huntington's disease) have both absent proactive motor suppression and impaired behavioral selectivity when stopping. Thus, stopping goals are used to proactively set up specific basal ganglia channels that may then be triggered to implement selective suppression. By linking this suppression to the striatum and pallidum, these results provide compelling functional evidence in humans of the basal ganglia's inhibitory indirect pathway.

  7. Understanding Parkinsonian handwriting through a computational model of basal ganglia.

    Science.gov (United States)

    Gangadhar, Garipelli; Joseph, Denny; Chakravarthy, V Srinivasa

    2008-10-01

    Handwriting in Parkinson's disease (PD) is typically characterized by micrographia, jagged line contour, and unusual fluctuations in pen tip velocity. Although PD handwriting features have been used for diagnostics, they are not based on a signaling model of basal ganglia (BG). In this letter, we present a computational model of handwriting generation that highlights the role of BG. When PD conditions like reduced dopamine and altered dynamics of the subthalamic nucleus and globus pallidus externa subsystems are simulated, the handwriting produced by the model manifested characteristic PD handwriting distortions like micrographia and velocity fluctuations. Our approach to PD modeling is in tune with the perspective that PD is a dynamic disease. PMID:18386983

  8. A Mathematical Model of Levodopa Medication Effect on Basal Ganglia in Parkinson's Disease: An Application to the Alternate Finger Tapping Task.

    Science.gov (United States)

    Baston, Chiara; Contin, Manuela; Calandra Buonaura, Giovanna; Cortelli, Pietro; Ursino, Mauro

    2016-01-01

    Malfunctions in the neural circuitry of the basal ganglia (BG), induced by alterations in the dopaminergic system, are responsible for an array of motor disorders and milder cognitive issues in Parkinson's disease (PD). Recently Baston and Ursino (2015a) presented a new neuroscience mathematical model aimed at exploring the role of basal ganglia in action selection. The model is biologically inspired and reproduces the main BG structures and pathways, modeling explicitly both the dopaminergic and the cholinergic system. The present work aims at interfacing this neurocomputational model with a compartmental model of levodopa, to propose a general model of medicated Parkinson's disease. Levodopa effect on the striatum was simulated with a two-compartment model of pharmacokinetics in plasma joined with a motor effect compartment. The latter is characterized by the levodopa removal rate and by a sigmoidal relationship (Hill law) between concentration and effect. The main parameters of this relationship are saturation, steepness, and the half-maximum concentration. The effect of levodopa is then summed to a term representing the endogenous dopamine effect, and is used as an external input for the neurocomputation model; this allows both the temporal aspects of medication and the individual patient characteristics to be simulated. The frequency of alternate tapping is then used as the outcome of the whole model, to simulate effective clinical scores. Pharmacokinetic-pharmacodynamic modeling was preliminary performed on data of six patients with Parkinson's disease (both "stable" and "wearing-off" responders) after levodopa standardized oral dosing over 4 h. Results show that the model is able to reproduce the temporal profiles of levodopa in plasma and the finger tapping frequency in all patients, discriminating between different patterns of levodopa motor response. The more influential parameters are the Hill coefficient, related with the slope of the effect sigmoidal

  9. 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.

  10. Basal ganglia disorders studied by positron emission tomography

    International Nuclear Information System (INIS)

    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 [18F]6-fluoro-L-dopa ([18F]dopa), and striatal dopamine receptor density with suitable PET ligands. [18F]dopa uptake in the striatum (putamen) is markedly reduced in patients with Parkinson's disease (PD). [18F]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 [18F]dopa uptake is reduced to a similar level in patients with multiple system atrophy (MSA) and PD, caudate [18F] dopa uptake is lower in MSA than PD. However, [18F]dopa PET cannot consistently distinguish MSA from PD because individual ranges of caudate [18F]dopa uptake overlap. D1 and D2 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 [18F]dopa uptake have been observed in patients with progressive supranuclear palsy (PSP). Moderate reductions in D2 receptor binding have been reported in the striatum of PSP patients. The reduction in D2 receptor binding is more prominent in the caudate than putamen. Striatal [18F]dopa uptake is normal or only mildly reduced in patients with dopa responsive dystonia (DRD). D2 receptor binding is markedly reduced in patients with Huntington's disease, while striatal [18F]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

  11. Basal ganglia - thalamus and the crowning enigma

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    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.

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

    International Nuclear Information System (INIS)

    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)

  13. 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.

  14. Short latency cerebellar modulation of the basal ganglia.

    Science.gov (United States)

    Chen, Christopher H; Fremont, Rachel; Arteaga-Bracho, Eduardo E; Khodakhah, Kamran

    2014-12-01

    The graceful, purposeful motion of our body is an engineering feat that remains unparalleled in robotic devices using advanced artificial intelligence. Much of the information required for complex movements is generated by the cerebellum and the basal ganglia in conjunction with the cortex. Cerebellum and basal ganglia have been thought to communicate with each other only through slow, multi-synaptic cortical loops, begging the question as to how they coordinate their outputs in real time. We found that the cerebellum rapidly modulates the activity of the striatum via a disynaptic pathway in mice. Under physiological conditions, this short latency pathway was capable of facilitating optimal motor control by allowing the basal ganglia to incorporate time-sensitive cerebellar information and by guiding the sign of cortico-striatal plasticity. Conversely, under pathological condition, this pathway relayed aberrant cerebellar activity to the basal ganglia to cause dystonia. PMID:25402853

  15. Idiopathic Basal Ganglia Calcification Presented with Impulse Control Disorder

    Science.gov (United States)

    Sahin, Cem; Levent, Mustafa; Akbaba, Gulhan; Kara, Bilge; Yeniceri, Emine Nese; Inanc, Betul Battaloglu

    2015-01-01

    Primary familial brain calcification (PFBC), also referred to as Idiopathic Basal Ganglia Calcification (IBGC) or “Fahr's disease,” is a clinical condition characterized by symmetric and bilateral calcification of globus pallidus and also basal ganglions, cerebellar nuclei, and other deep cortical structures. It could be accompanied by parathyroid disorder and other metabolic disturbances. The clinical features are dysfunction of the calcified anatomic localization. IBGC most commonly presents with mental damage, convulsion, parkinson-like clinical picture, and neuropsychiatric behavior disorders; however, presentation with impulse control disorder is not a frequent presentation. In the current report, a 43-year-old male patient who has been admitted to psychiatry policlinic with the complaints of aggressive behavior episodes and who has been diagnosed with impulse control disorder and IBGC was evaluated in the light of the literature. PMID:26246920

  16. 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.)

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

    International Nuclear Information System (INIS)

    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.)

  18. Genetics Home Reference: familial idiopathic basal ganglia calcification

    Science.gov (United States)

    ... Wang QK, Liu JY. Identification of a novel genetic locus on chromosome 8p21.1-q11.23 for idiopathic ... DH. Analysis of candidate genes at the IBGC1 locus associated with idiopathic basal ... DH. Genetic heterogeneity in familial idiopathic basal ganglia calcification (Fahr ...

  19. 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.

  20. 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. PMID:26956115

  1. Covert skill learning in a cortical-basal ganglia circuit.

    Science.gov (United States)

    Charlesworth, Jonathan D; Warren, Timothy L; Brainard, Michael S

    2012-06-14

    We learn complex skills such as speech and dance through a gradual process of trial and error. Cortical-basal ganglia circuits have an important yet unresolved function in this trial-and-error skill learning; influential 'actor-critic' models propose that basal ganglia circuits generate a variety of behaviours during training and learn to implement the successful behaviours in their repertoire. Here we show that the anterior forebrain pathway (AFP), a cortical-basal ganglia circuit, contributes to skill learning even when it does not contribute to such 'exploratory' variation in behavioural performance during training. Blocking the output of the AFP while training Bengalese finches to modify their songs prevented the gradual improvement that normally occurs in this complex skill during training. However, unblocking the output of the AFP after training caused an immediate transition from naive performance to excellent performance, indicating that the AFP covertly gained the ability to implement learned skill performance without contributing to skill practice. In contrast, inactivating the output nucleus of the AFP during training completely prevented learning, indicating that learning requires activity within the AFP during training. Our results suggest a revised model of skill learning: basal ganglia circuits can monitor the consequences of behavioural variation produced by other brain regions and then direct those brain regions to implement more successful behaviours. The ability of the AFP to identify successful performances generated by other brain regions indicates that basal ganglia circuits receive a detailed efference copy of premotor activity in those regions. The capacity of the AFP to implement successful performances that were initially produced by other brain regions indicates precise functional connections between basal ganglia circuits and the motor regions that directly control performance. PMID:22699618

  2. Alteration of basal ganglia and right frontoparietal network in early drug-naïve Parkinson’s disease during heat pain stimuli and resting state

    Directory of Open Access Journals (Sweden)

    Ying eTan

    2015-08-01

    Full Text Available Background: The symptoms and pathogenesis of Parkinson’s disease (PD are complicated and accurate diagnosis is difficult, particularly in early-stage. Functional magnetic resonance imaging is noninvasive and characterized by the integration of different brain areas at functional connectivity (FC. Considering pain process in PD, we hypothesized that pain is one of the earliest symptoms and investigated whether FC of the pain network was disrupted in PD without pain.Methods: Fourteen early drug-naïve PD without pain and 17 age- and sex-matched healthy controls (HC participated in our test. We investigate abnormalities in FC and in functional network connectivity in PD compared with HC during the task (51 °C heat pain stimuli and at rest.Results: Compared with HC, PD showed decreased FC in basal ganglia network (BGN, salience network (SN and sensorimotor network in two states respectively. FNC between the BGN and the SN are reduced during both states in PD compared with HC. In addition, the FNC associated with right frontoparietal network (RFPN was also significantly disturbed during the task.Conclusion: These findings suggest that BGN plays a role in the pathological mechanisms of pain underlying PD, and RFPN likely contributes greatly to harmonization between intrinsic brain activity and external stimuli.

  3. Mephedrone alters basal ganglia and limbic neurotensin systems.

    Science.gov (United States)

    German, Christopher L; Hoonakker, Amanda H; Fleckenstein, Annette E; Hanson, Glen R

    2014-08-01

    Mephedrone (4-methylmethcathinone) is a synthetic cathinone designer drug that alters pre-synaptic dopamine (DA) activity like many psychostimulants. However, little is known about the post-synaptic dopaminergic impacts of mephedrone. The neuropeptide neurotensin (NT) provides inhibitory feedback for basal ganglia and limbic DA pathways, and post-synaptic D1 -like and D2 -like receptor activity affects NT tissue levels. This study evaluated how mephedrone alters basal ganglia and limbic system NT content and the role of NT receptor activation in drug consumption behavior. Four 25 mg/kg injections of mephedrone increased NT content in basal ganglia (striatum, substantia nigra and globus pallidus) and the limbic regions (nucleus accumbens core), while a lower dosage (5 mg/kg/injection) only increased striatal NT content. Mephedrone-induced increases in basal ganglia NT levels were mediated by D1 -like receptors in the striatum and the substantia nigra by both D1 -like and D2 -like receptors in the globus pallidus. Mephedrone increased substance P content, another neuropeptide, in the globus pallidus, but not in the dorsal striatum or substantia nigra. Finally, the NT receptor agonist PD149163 blocked mephedrone self-administration, suggesting reduced NT release, as indicated by increased tissue levels, likely contributing to patterns of mephedrone consumption.

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

    International Nuclear Information System (INIS)

    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.)

  5. Unilateral germinomas involving the basal ganglia and thalamus.

    Science.gov (United States)

    Kobayashi, T; Kageyama, N; Kida, Y; Yoshida, J; Shibuya, N; Okamura, K

    1981-07-01

    Clinical characteristics of six cases of germinoma involving a unilateral basal ganglion and thalamus are summarized. The incidence was estimated as 10% of all intracranial germinomas. The average age at the onset was 10.5 years. The sex incidence showed a male dominance. The clinical course was slowly progressive, and the average duration between onset and diagnosis was 2 years 5 months. Common symptoms and signs were hemiparesis in all cases, fever of unknown origin and eye symptoms in most, mental deterioration and psychiatric signs in three, and convulsions, pubertas praecox, and diabetes insipidus in two. Signs of increased intracranial pressure were found in only two cases in the later state of the disease. Early diagnosis is difficult because of nonspecific symptomatology and slow progression. Carotid angiography and pneumoencephalography showed abnormal findings compatible with basal ganglia and thalamic tumors, but not specific to germinoma. Ipsilateral cortical atrophy and ventricular dilatation might be significant findings. Radioisotope scanning was useful. Computerized tomography scans were the best method of detecting the location and nature of this tumor, and repeat scans showed response to radiation therapy. PMID:7241216

  6. Functional Neuroanatomy and Behavioural Correlates of the Basal Ganglia: Evidence from Lesion Studies

    Directory of Open Access Journals (Sweden)

    Peter Ward

    2013-01-01

    Full Text Available Introduction: The basal ganglia are interconnected with cortical areas involved in behavioural, cognitive and emotional processes, in addition to movement regulation. Little is known about which of these functions are associated with individual basal ganglia substructures.

  7. Cognition and the basal ganglia: a possible substrate for procedural knowledge.

    Science.gov (United States)

    Phillips, A G; Carr, G D

    1987-08-01

    Disruption of neural activity within the basal ganglia of experimental animals causes selective learning deficits in tasks requiring switching between response strategies. These data along with reports of both general and specific intellectual impairment in patients with neurodegenerative disorders such as Parkinson's disease, appear to support the theory of cognitive functions of the basal ganglia. Recent studies have failed to confirm general cognitive or memory deficits in parkinsonian patients, but have identified deficiencies in devising and executing certain cognitive strategies. Following the lead of theorists such as Squire and Mishkin, this brief review emphasizes the distinction between procedural and declarative knowledge and examines the possible role of the basal ganglia in the acquisition and retention of procedural knowledge. PMID:3315145

  8. The role of the basal ganglia in beat perception: neuroimaging and neuropsychological investigations.

    Science.gov (United States)

    Grahn, Jessica A

    2009-07-01

    Perception of musical rhythms is culturally universal. Despite this special status, relatively little is known about the neurobiology of rhythm perception, particularly with respect to beat processing. Findings are presented here from a series of studies that have specifically examined the neural basis of beat perception, using functional magnetic resonance imaging (fMRI) and studying patients with Parkinson's disease. fMRI data indicate that novel beat-based sequences robustly activate the basal ganglia when compared to irregular, nonbeat sequences. Furthermore, although most healthy participants find it much easier to discriminate changes in beat-based sequences compared to irregular sequences, Parkinson's disease patients fail to show the same degree of benefit. Taken together, these data suggest that the basal ganglia are performing a crucial function in beat processing. The results of an additional fMRI study indicate that the role of the basal ganglia is strongly linked to internal generation of the beat. Basal ganglia activity is greater when participants listen to rhythms in which internal generation of the beat is required, as opposed to rhythms with strongly externally cued beats. Functional connectivity between part of the basal ganglia (the putamen) and cortical motor areas (premotor and supplementary motor areas) is also higher during perception of beat rhythms compared to nonbeat rhythms. Increased connectivity between cortical motor and auditory areas is found in those with musical training. The findings from these converging methods strongly implicate the basal ganglia in processing a regular beat, particularly when internal generation of the beat is required. PMID:19673753

  9. Modeling basal ganglia for understanding Parkinsonian reaching movements.

    Science.gov (United States)

    Magdoom, K N; Subramanian, D; Chakravarthy, V S; Ravindran, B; Amari, Shun-Ichi; Meenakshisundaram, N

    2011-02-01

    We present a computational model that highlights the role of basal ganglia (BG) in generating simple reaching movements. The model is cast within the reinforcement learning (RL) framework with correspondence between RL components and neuroanatomy as follows: dopamine signal of substantia nigra pars compacta as the temporal difference error, striatum as the substrate for the critic, and the motor cortex as the actor. A key feature of this neurobiological interpretation is our hypothesis that the indirect pathway is the explorer. Chaotic activity, originating from the indirect pathway part of the model, drives the wandering, exploratory movements of the arm. Thus, the direct pathway subserves exploitation, while the indirect pathway subserves exploration. The motor cortex becomes more and more independent of the corrective influence of BG as training progresses. Reaching trajectories show diminishing variability with training. Reaching movements associated with Parkinson's disease (PD) are simulated by reducing dopamine and degrading the complexity of indirect pathway dynamics by switching it from chaotic to periodic behavior. Under the simulated PD conditions, the arm exhibits PD motor symptoms like tremor, bradykinesia and undershooting. The model echoes the notion that PD is a dynamical disease. PMID:21105828

  10. Neural activity in the rat basal ganglia

    NARCIS (Netherlands)

    Zhao, Y.; Stegenga, J.; Heida, T.; Wezel, van R.J.A.

    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

  11. Morphological elucidation of basal ganglia circuits contributing reward prediction.

    Science.gov (United States)

    Fujiyama, Fumino; Takahashi, Susumu; Karube, Fuyuki

    2015-01-01

    Electrophysiological studies in monkeys have shown that dopaminergic neurons respond to the reward prediction error. In addition, striatal neurons alter their responsiveness to cortical or thalamic inputs in response to the dopamine signal, via the mechanism of dopamine-regulated synaptic plasticity. These findings have led to the hypothesis that the striatum exhibits synaptic plasticity under the influence of the reward prediction error and conduct reinforcement learning throughout the basal ganglia circuits. The reinforcement learning model is useful; however, the mechanism by which such a process emerges in the basal ganglia needs to be anatomically explained. The actor-critic model has been previously proposed and extended by the existence of role sharing within the striatum, focusing on the striosome/matrix compartments. However, this hypothesis has been difficult to confirm morphologically, partly because of the complex structure of the striosome/matrix compartments. Here, we review recent morphological studies that elucidate the input/output organization of the striatal compartments. PMID:25698913

  12. [Morphological Re-evaluation of the Basal Ganglia Network].

    Science.gov (United States)

    Fujiyama, Fumino

    2016-07-01

    Electrophysiological studies in monkeys have shown that dopaminergic neurons respond to the reward prediction error. In addition, striatal neurons alter their responsiveness to cortical or thalamic inputs in response to dopamine signals, via dopamine-regulated synaptic plasticity. These findings have led to the hypothesis that the striatum exhibits synaptic plasticity under the influence of reward prediction error and conducts reinforcement learning throughout the basal ganglia circuits. The reinforcement learning model is useful; however, the mechanism by which such a process emerges in the basal ganglia needs to be anatomically explained. The actor-critic model has been previously proposed and extended by the existence of role sharing within the striatum, with particular focus on the striosome and matrix compartments. However, this hypothesis has been difficult to confirm morphologically, partly because of the complex structure of the striosome and matrix compartments. Here, we review recent morphological studies that elucidate the input/output organization of the striatal compartments. PMID:27395470

  13. Is Broca's area part of a basal ganglia thalamocortical circuit?

    Science.gov (United States)

    Ullman, Michael T

    2006-05-01

    The cortex constituting Broca's area does not exist in isolation. Rather, like other cortical regions, Broca's area is connected to other brain structures, which likely play closely related functional roles. This paper focuses on the basal ganglia, a set of subcortical structures that project through topographically organized "channels" via the thalamus to different frontal regions. It is hypothesized that the basal ganglia project to Broca's area. This circuitry is further posited to encompass at least two channels. One channel can be characterized as subserving procedural memory, while the other underlies the retrieval of knowledge from declarative memory. These hypotheses are supported by both anatomical and functional evidence. Implications and issues for further investigation are discussed. PMID:16881254

  14. Cerebellar networks with the cerebral cortex and basal ganglia.

    Science.gov (United States)

    Bostan, Andreea C; Dum, Richard P; Strick, Peter L

    2013-05-01

    The dominant view of cerebellar function has been that it is exclusively concerned with motor control and coordination. Recent findings from neuroanatomical, behavioral, and imaging studies have profoundly changed this view. Neuroanatomical studies using virus transneuronal tracers have demonstrated that cerebellar output reaches vast areas of the neocortex, including regions of prefrontal and posterior parietal cortex. Furthermore, it has recently become clear that the cerebellum is reciprocally connected with the basal ganglia, which suggests that the two subcortical structures are part of a densely interconnected network. Taken together, these findings elucidate the neuroanatomical substrate for cerebellar involvement in non-motor functions mediated by the prefrontal and posterior parietal cortex, as well as in processes traditionally associated with the basal ganglia. PMID:23579055

  15. 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.

  16. Subsystems of the basal ganglia and motor infrastructure

    OpenAIRE

    Kamali Sarvestani, Iman

    2013-01-01

    The motor nervous system is one of the main systems of the body and is our principle means ofbehavior. Some of the most debilitating and wide spread disorders are motor systempathologies. In particular the basal ganglia are complex networks of the brain that control someaspects of movement in all vertebrates. Although these networks have been extensively studied,lack of proper methods to study them on a system level has hindered the process ofunderstanding what they do and how they do it. In ...

  17. Meige's syndrome associated with basal ganglia and thalamic functional disorders

    International Nuclear Information System (INIS)

    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 γ-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)

  18. 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.

  19. Basal ganglia cholinergic and dopaminergic function in progressive supranuclear palsy.

    Science.gov (United States)

    Warren, Naomi M; Piggott, Margaret A; Greally, Elizabeth; Lake, Michelle; Lees, Andrew J; Burn, David J

    2007-08-15

    Progressive Supranuclear Palsy (PSP) is a progressive neurodegenerative disorder. In contrast to Parkinson's disease (PD) and dementia with Lewy bodies (DLB), replacement therapy with dopaminergic and cholinergic agents in PSP has been disappointing. The neurochemical basis for this is unclear. Our objective was to measure dopaminergic and cholinergic receptors in the basal ganglia of PSP and control brains. We measured, autoradiographically, dopaminergic (dopamine transporter, 125I PE2I and dopamine D2 receptors, 125I epidepride) and cholinergic (nicotinic alpha4beta2 receptors, 125I 5IA85380 and muscarinic M1 receptors, 3H pirenzepine) parameters in the striatum and pallidum of pathologically confirmed PSP cases (n=15) and controls (n=32). In PSP, there was a marked loss of dopamine transporter and nicotinic alpha4beta2 binding in the striatum and pallidum, consistent with loss of nigrostriatal neurones. Striatal D2 receptors were increased in the caudate and muscarinic M1 receptors were unchanged compared with controls. These results do not account for the poor response to dopaminergic and cholinergic replacement therapies in PSP, and suggest relative preservation of postsynaptic striatal projection neurones bearing D2/M1 receptors. PMID:17534953

  20. 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.

  1. Saccade learning with concurrent cortical and subcortical basal ganglia loops

    Directory of Open Access Journals (Sweden)

    Steve eN'guyen

    2014-04-01

    Full Text Available The Basal Ganglia is a central structure involved in multiple cortical and subcortical loops. Some of these loops are believed to be responsible for saccade target selection. We study here how the very specific structural relationships of these saccadic loops can affect the ability of learning spatial and feature-based tasks.We propose a model of saccade generation with reinforcement learning capabilities based onour previous basal ganglia and superior colliculus models. It is structured around the interactions of two parallel cortico-basal loops and one tecto-basal loop. The two cortical loops separately deal with spatial and non-spatial information to select targets in a concurrent way. The subcortical loop is used to make the final target selection leading to the production of thesaccade. These different loops may work in concert or disturb each other regarding reward maximization. Interactions between these loops and their learning capabilities are tested on different saccade tasks.The results show the ability of this model to correctly learn basic target selection based on different criteria (spatial or not. Moreover the model reproduces and explains training dependent express saccades toward targets based on a spatial criterion. Finally, the model predicts that in absence of prefrontal control, the spatial loop should dominate.

  2. 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

  3. Focal expression of mutant huntingtin in the songbird basal ganglia disrupts cortico-basal ganglia networks and vocal sequences.

    Science.gov (United States)

    Tanaka, Masashi; Singh Alvarado, Jonnathan; Murugan, Malavika; Mooney, Richard

    2016-03-22

    The basal ganglia (BG) promote complex sequential movements by helping to select elementary motor gestures appropriate to a given behavioral context. Indeed, Huntington's disease (HD), which causes striatal atrophy in the BG, is characterized by hyperkinesia and chorea. How striatal cell loss alters activity in the BG and downstream motor cortical regions to cause these disorganized movements remains unknown. Here, we show that expressing the genetic mutation that causes HD in a song-related region of the songbird BG destabilizes syllable sequences and increases overall vocal activity, but leave the structure of individual syllables intact. These behavioral changes are paralleled by the selective loss of striatal neurons and reduction of inhibitory synapses on pallidal neurons that serve as the BG output. Chronic recordings in singing birds revealed disrupted temporal patterns of activity in pallidal neurons and downstream cortical neurons. Moreover, reversible inactivation of the cortical neurons rescued the disorganized vocal sequences in transfected birds. These findings shed light on a key role of temporal patterns of cortico-BG activity in the regulation of complex motor sequences and show how a genetic mutation alters cortico-BG networks to cause disorganized movements. PMID:26951661

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

    Directory of Open Access Journals (Sweden)

    Cosima Rhein

    Full Text Available 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 nuclei in a sex

  5. 基底节性失语%Basal Ganglia Aphasia

    Institute of Scientific and Technical Information of China (English)

    隆昱洲; 柳华; 艾青龙

    2008-01-01

    基底节病变常导致语言功能障碍,其表现彤式复杂,既可出现口语语言障碍,也可出现书面语语言障碍,几乎包括所有失语类型.文章就基底节解剖、基底节失语的定义、特点、机制以及病变部位对语言的影响做了综述.%Basal ganglion lesions often result in language impairment. Its patterns of manifestation are complicated. Patients may either have oral language disorders or written language disorders, which almost includes all types of aphasia, The article reviews the anatomy, definition, feature and mechanisms of basal ganglia aphasia as well as the effect of lesion sites on language.

  6. Novelty encoding by the output neurons of the basal ganglia

    Directory of Open Access Journals (Sweden)

    Mati Joshua

    2010-01-01

    Full Text Available Reinforcement learning models of the basal ganglia have focused on the resemblance of the dopamine signal to the temporal difference error. However the role of the network as a whole is still elusive, in particular whether the output of the basal ganglia encodes only the behavior (actions or it is part of the valuation process. We trained a monkey extensively on a probabilistic conditional task with seven fractal cues predicting rewarding or aversive outcomes (familiar cues. Then in each recording session we added a cue that the monkey had never seen before (new cue and recorded from single units in the Substantia Nigra pars reticulata (SNpr while the monkey was engaged in a task with new cues intermingled within the familiar ones. The monkey learned the association between the new cue and outcome and modified its licking and blinking behavior which became similar to responses to the familiar cues with the same outcome. However, the responses of many SNpr neurons to the new cue exceeded their response to familiar cues even after behavioral learning was completed. This dissociation between behavior and neural activity suggests that the BG output code goes beyond instruction or gating of behavior to encoding of novel cues. Thus, BG output can enable learning at the levels of its target neural networks.

  7. Morphological elucidation of basal ganglia circuits contributing reward prediction

    Directory of Open Access Journals (Sweden)

    Fumino eFujiyama

    2015-02-01

    Full Text Available Electrophysiological studies in monkeys have shown that dopaminergic neurons respond to the reward prediction error. In addition, striatal neurons alter their responsiveness to cortical or thalamic inputs in response to the dopamine signal, via the mechanism of dopamine-regulated synaptic plasticity. These findings have led to the hypothesis that the striatum exhibits synaptic plasticity under the influence of the reward prediction error and conduct reinforcement learning throughout the basal ganglia circuits.The reinforcement learning model is useful; however, the mechanism by which such a process emerges in the basal ganglia needs to be anatomically explained. The actor–critic model has been previously proposed and extended by the existence of role sharing within the striatum, focusing on the striosome/matrix compartments. However, this hypothesis has been difficult to confirm morphologically, partly because of the complex structure of the striosome/matrix compartments. Here, we review recent morphological studies that elucidate the input/output organization of the striatal compartments.

  8. Mephedrone alters basal ganglia and limbic dynorphin systems.

    Science.gov (United States)

    German, Christopher L; Alburges, Mario E; Hoonakker, Amanda J; Fleckenstein, Annette E; Hanson, Glen R

    2014-08-25

    Mephedrone (4-methymethcathinone) is a synthetic cathinone designer drug that disrupts central nervous system (CNS) dopamine (DA) signaling. Numerous central neuropeptide systems reciprocally interact with dopaminergic neurons to provide regulatory counterbalance, and are altered by aberrant DA activity associated with stimulant exposure. Endogenous opioid neuropeptides are highly concentrated within dopaminergic CNS regions and facilitate many rewarding and aversive properties associated with drug use. Dynorphin, an opioid neuropeptide and kappa receptor agonist, causes dysphoria and aversion to drug consumption through signaling within the basal ganglia and limbic systems, which is affected by stimulants. This study evaluated how mephedrone alters basal ganglia and limbic system dynorphin content, and the role of DA signaling in these changes. Repeated mephedrone administrations (4 × 25 mg/kg/injection, 2-h intervals) selectively increased dynorphin content throughout the dorsal striatum and globus pallidus, decreased dynorphin content within the frontal cortex, and did not alter dynorphin content within most limbic system structures. Pretreatment with D1 -like (SCH-23380) or D2 -like (eticlopride) antagonists blocked mephedrone-induced changes in dynorphin content in most regions examined, indicating altered dynorphin activity is a consequence of excessive DA signaling. Synapse, 2014. © 2014 Wiley Periodicals, Inc.

  9. Chorea due to basal ganglia involvement in a uremic diabetic patient

    Directory of Open Access Journals (Sweden)

    Faik Ilik

    2014-04-01

    Full Text Available Syndromes associated with acute bilateral lesions of the basal ganglia in diabetic uremic patients are uncommon. Uremic encephalopathy is typical of patients showing cortical involvement, with symptoms including confusion, seizures, tremors, or myoclonus. Whenever basal ganglia are anatomically involved, movement disorders arise, including chorea. In this article we present a case with basal ganglia involvement in a uremic diabetic patient causes chorea because of rare presentation. [Cukurova Med J 2014; 39(2.000: 353-356

  10. Traumatic bilateral basal ganglia bleed: A report of rare two cases and review of the literature

    Science.gov (United States)

    Kankane, Vivek Kumar; Gupta, Tarun Kumar; Jaiswal, Gaurav

    2016-01-01

    Traumatic basal ganglia hemorrhage (TBGH) 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. The general incidence of TBGH is reported between 2.4% and 3% of closed head injury. However, the incidence is higher in postmortem studies (9.8%). Bilateral traumatic basal ganglia hematoma is extremely rare. Descriptions are limited to case reports.

  11. Role of movement in long-term basal ganglia changes: implications for abnormal motor responses

    OpenAIRE

    Nicola eSimola; Micaela eMorelli; Giuseppe eFrazzitta; Lucia eFrau

    2013-01-01

    Abnormal involuntary movements and dyskinesias elicited by drugs that stimulate dopamine receptors in the basal ganglia are a major issue in the management of Parkinson’s disease (PD). Preclinical studies in dopamine-denervated animals have contributed to the modeling of these abnormal movements, but the precise neurochemical and functional mechanisms underlying these untoward effects are still elusive. It has recently been suggested that the performance of movement may itself promote the lat...

  12. Role of movement in long-term basal ganglia changes: implications for abnormal motor responses

    OpenAIRE

    Simola, Nicola; Morelli, Micaela; Frazzitta, Giuseppe; Frau, Lucia

    2013-01-01

    Abnormal involuntary movements (AIMs) and dyskinesias elicited by drugs that stimulate dopamine receptors in the basal ganglia are a major issue in the management of Parkinson’s disease (PD). Preclinical studies in dopamine-denervated animals have contributed to the modeling of these abnormal movements, but the precise neurochemical and functional mechanisms underlying these untoward effects are still elusive. It has recently been suggested that the performance of movement may itself promote ...

  13. Using a hybrid neuron in physiologically inspired models of the basal ganglia

    Directory of Open Access Journals (Sweden)

    Corey Michael Thibeault

    2013-07-01

    Full Text Available Our current understanding of the basal ganglia has facilitated the creation of computational models that have contributed novel theories, explored new functional anatomy and demonstrated results complementing physiological experiments. However, the utility of these models extends beyond these applications. Particularly in neuromorphic engineering, where the basal ganglia's role in computation is important for applications such as power efficient autonomous agents and model-based control strategies. The neurons used in existing computational models of the basal ganglia however, are not amenable for many low-power hardware implementations. Motivated by a need for more hardware accessible networks, we replicate four published models of the basal ganglia, spanning single neuron and small networks, replacing the more computationally expensive neuron models with an Izhikevich hybrid neuron. This begins with a network modeling action-selection, where the basal activity levels and the ability to appropriately select the most salient input is reproduced. A Parkinson's disease model is then explored under normal conditions, Parkinsonian conditions and during subthalamic nucleus deep brain stimulation. The resulting network is capable of replicating the loss of thalamic relay capabilities in the Parkinsonian state and its return under deep brain stimulation. This is also demonstrated using a network capable of action-selection. Finally, a study of correlation transfer under different patterns of Parkinsonian activity is presented. These networks successfully captured the significant results of the originals studies. This not only creates a foundation for neuromorphic hardware implementations but may also support the development of large-scale biophysical models. The former potentially providing a way of improving the efficacy of deep brain stimulation and the latter allowing for the efficient simulation of larger more comprehensive networks.

  14. 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.

  15. 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.

  16. Striatal Cholinergic Interneurons Control Motor Behavior and Basal Ganglia Function in Experimental Parkinsonism.

    Science.gov (United States)

    Maurice, Nicolas; Liberge, Martine; Jaouen, Florence; Ztaou, Samira; Hanini, Marwa; Camon, Jeremy; Deisseroth, Karl; Amalric, Marianne; Kerkerian-Le Goff, Lydia; Beurrier, Corinne

    2015-10-27

    Despite evidence showing that anticholinergic drugs are of clinical relevance in Parkinson's disease (PD), the causal role of striatal cholinergic interneurons (CINs) in PD pathophysiology remains elusive. Here, we show that optogenetic inhibition of CINs alleviates motor deficits in PD mouse models, providing direct demonstration for their implication in parkinsonian motor dysfunctions. As neural correlates, CIN inhibition in parkinsonian mice differentially impacts the excitability of striatal D1 and D2 medium spiny neurons, normalizes pathological bursting activity in the main basal ganglia output structure, and increases the functional weight of the direct striatonigral pathway in cortical information processing. By contrast, CIN inhibition in non-lesioned mice does not affect locomotor activity, equally modulates medium spiny neuron excitability, and does not modify spontaneous or cortically driven activity in the basal ganglia output, suggesting that the role of these interneurons in motor function is highly dependent on dopamine tone. PMID:26489458

  17. Striatal Cholinergic Interneurons Control Motor Behavior and Basal Ganglia Function in Experimental Parkinsonism

    Directory of Open Access Journals (Sweden)

    Nicolas Maurice

    2015-10-01

    Full Text Available Despite evidence showing that anticholinergic drugs are of clinical relevance in Parkinson’s disease (PD, the causal role of striatal cholinergic interneurons (CINs in PD pathophysiology remains elusive. Here, we show that optogenetic inhibition of CINs alleviates motor deficits in PD mouse models, providing direct demonstration for their implication in parkinsonian motor dysfunctions. As neural correlates, CIN inhibition in parkinsonian mice differentially impacts the excitability of striatal D1 and D2 medium spiny neurons, normalizes pathological bursting activity in the main basal ganglia output structure, and increases the functional weight of the direct striatonigral pathway in cortical information processing. By contrast, CIN inhibition in non-lesioned mice does not affect locomotor activity, equally modulates medium spiny neuron excitability, and does not modify spontaneous or cortically driven activity in the basal ganglia output, suggesting that the role of these interneurons in motor function is highly dependent on dopamine tone.

  18. Bilateral basal ganglia lesions in patients with end-stage diabetic nephropathy.

    Science.gov (United States)

    Li, Jordan Y Z; Yong, Tuck Y; Sebben, Ruben; Khoo, Eewin; Disney, Alex P S

    2008-02-01

    Acute movement disorder associated with reversible bilateral basal ganglia lesions is an increasingly recognized syndrome in patients with end-stage renal disease, especially in the setting of concurrent diabetes mellitus. We report an elderly man with end-stage diabetic nephropathy treated by daily automated peritoneal dialysis who developed subacute symptoms of gait disturbance, dysarthria, dysphagia and lethargy. Computed tomography and magnetic resonance imaging of the head revealed bilateral symmetrical basal ganglia lesions. Repeat imaging 3 weeks later showed that these lesions had regressed spontaneously. However, his neurological symptoms improved slowly. These findings were similar to 23 other cases in the literature. Review of these cases shows that clinical features were predominantly bradykinesia, gait disturbance and concurrent metabolic acidosis (observed in 90% of cases). The pathogenesis of this condition has not been clearly defined, but uraemia may be an aggravating factor in predisposed patients, particularly in the presence of diabetic microvascular disease. There is no specific treatment for this condition; supportive measures are the mainstay of management. In the majority of patients, neurological improvement lags behind regression of basal ganglia lesions seen with neuroimaging, and the long-term outcome is variable.

  19. Exploring the cognitive and motor functions of the basal ganglia: an integrative review of computational cognitive neuroscience models

    OpenAIRE

    Sebastien Helie; Srinivasa Chakravarthy; Moustafa, Ahmed A.

    2013-01-01

    Many computational models of the basal ganglia have been proposed over the past twenty-five years. While computational neuroscience models have focused on closely matching the neurobiology of the basal ganglia, computational cognitive neuroscience models have focused on how the basal ganglia can be used to implement cognitive and motor functions. This review article focuses on computational cognitive neuroscience models of the basal ganglia and how they use the neuroanatomy of the basal gangl...

  20. Mean-field modeling of the basal ganglia-thalamocortical system. II Dynamics of parkinsonian oscillations.

    Science.gov (United States)

    van Albada, S J; Gray, R T; Drysdale, P M; Robinson, P A

    2009-04-21

    Neuronal correlates of Parkinson's disease (PD) include a shift to lower frequencies in the electroencephalogram (EEG) and enhanced synchronized oscillations at 3-7 and 7-30 Hz in the basal ganglia, thalamus, and cortex. This study describes the dynamics of a recent physiologically based mean-field model of the basal ganglia-thalamocortical system, and shows how it accounts for many key electrophysiological correlates of PD. Its detailed functional connectivity comprises partially segregated direct and indirect pathways through two populations of striatal neurons, a hyperdirect pathway involving a corticosubthalamic projection, thalamostriatal feedback, and local inhibition in striatum and external pallidum (GPe). In a companion paper, realistic steady-state firing rates were obtained for the healthy state, and after dopamine loss modeled by weaker direct and stronger indirect pathways, reduced intrapallidal inhibition, lower firing thresholds of the GPe and subthalamic nucleus (STN), a stronger projection from striatum to GPe, and weaker cortical interactions. Here it is shown that oscillations around 5 and 20 Hz can arise with a strong indirect pathway, which also causes increased synchronization throughout the basal ganglia. Furthermore, increased theta power with progressive nigrostriatal degeneration is correlated with reduced alpha power and peak frequency, in agreement with empirical results. Unlike the hyperdirect pathway, the indirect pathway sustains oscillations with phase relationships that coincide with those found experimentally. Alterations in the responses of basal ganglia to transient stimuli accord with experimental observations. Reduced cortical gains due to both nigrostriatal and mesocortical dopamine loss lead to slower changes in cortical activity and may be related to bradykinesia. Finally, increased EEG power found in some studies may be partly explained by a lower effective GPe firing threshold, reduced GPe-GPe inhibition, and/or weaker

  1. Characteristics of basal ganglia aphasia after stroke and the rehabilitative interventions

    Institute of Scientific and Technical Information of China (English)

    Yating Kong; Xifeng Pan; Qimei Zhang

    2006-01-01

    OBJECTIVE: To introduce the characteristics of basal ganglia aphasia after stroke and the rehabilitative interventions.DATA SOURCES: Articles related to stroke, subcortical aphasia, basal ganglia aphasia and language rehabilitation published in Chinese from January 1988 to December 2005 were searched in Chinese journal full-text database (CJFD) using the keywords of"stroke, basal ganglia aphasia, language rehabilitation" in Chinese. Meanwhile, English articles about aphasia published from January 1982 to December 2005 were searched in and Pubmed database. Besides, several books associated with the contents were looked through manually.STUDY SELECTION: The data were checked primarily, the articles about the pathomechanism and neurolinguistic characteristics of basal ganglia aphasia, diagnostic methods of aphasia and language rehabilitation were selected, and those had no obvious relation with the above contents were excluded.Inclusive criteria: literatures explain the clinical characteristics of basal ganglia aphasia, neurolinguistic pathogenesis and methods of rehabilitation therapy in details. The repetitive studies were excluded.DATA EXTRACTION: Totally 95 literatures about basal ganglia aphasia were collected, including 31 about the clinical characteristics of basal ganglia aphasia, 45 about its neurolinguistic pathogenesis, 5 about the evaluation and classification of aphasia, and 14 about its rehabilitation therapy. Thirty accorded with the inclusive criteria were used for review, and the other 65 were excluded.DATA SYNTHESIS: Concisely introduced the definition, past investigation of basal ganglia aphasia after stroke, then dwelled on the multiplicity neurolinguistics characteristics. Aphasia evaluation was dependent upon clinical aphasic symptoms. The relationship between symptom and focus of infection was explored, and the mechanism of pathosis language behavior on basal ganglia aphasia patients was understood to provide consequence data that could

  2. Basal ganglia contributions to motor control: a vigorous tutor.

    Science.gov (United States)

    Turner, Robert S; Desmurget, Michel

    2010-12-01

    The roles of the basal ganglia (BG) in motor control are much debated. Many influential hypotheses have grown from studies in which output signals of the BG were not blocked, but pathologically disturbed. A weakness of that approach is that the resulting behavioral impairments reflect degraded function of the BG per se mixed together with secondary dysfunctions of BG-recipient brain areas. To overcome that limitation, several studies have focused on the main skeletomotor output region of the BG, the globus pallidus internus (GPi). Using single-cell recording and inactivation protocols these studies provide consistent support for two hypotheses: the BG modulates movement performance ('vigor') according to motivational factors (i.e. context-specific cost/reward functions) and the BG contributes to motor learning. Results from these studies also add to the problems that confront theories positing that the BG selects movement, inhibits unwanted motor responses, corrects errors on-line, or stores and produces well-learned motor skills. PMID:20850966

  3. Basal ganglia outputs map instantaneous position coordinates during behavior.

    Science.gov (United States)

    Barter, Joseph W; Li, Suellen; Sukharnikova, Tatyana; Rossi, Mark A; Bartholomew, Ryan A; Yin, Henry H

    2015-02-11

    The basal ganglia (BG) are implicated in many movement disorders, yet how they contribute to movement remains unclear. Using wireless in vivo recording, we measured BG output from the substantia nigra pars reticulata (SNr) in mice while monitoring their movements with video tracking. The firing rate of most nigral neurons reflected Cartesian coordinates (either x- or y-coordinates) of the animal's head position during movement. The firing rates of SNr neurons are either positively or negatively correlated with the coordinates. Using an egocentric reference frame, four types of neurons can be classified: each type increases firing during movement in a particular direction (left, right, up, down), and decreases firing during movement in the opposite direction. Given the high correlation between the firing rate and the x and y components of the position vector, the movement trajectory can be reconstructed from neural activity. Our results therefore demonstrate a quantitative and continuous relationship between BG output and behavior. Thus, a steady BG output signal from the SNr (i.e., constant firing rate) is associated with the lack of overt movement, when a stable posture is maintained by structures downstream of the BG. Any change in SNr firing rate is associated with a change in position (i.e., movement). We hypothesize that the SNr output quantitatively determines the direction, velocity, and amplitude of voluntary movements. By changing the reference signals to downstream position control systems, the BG can produce transitions in body configurations and initiate actions.

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

    International Nuclear Information System (INIS)

    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

  5. Parallel basal ganglia circuits for voluntary and automatic behaviour to reach rewards.

    Science.gov (United States)

    Kim, Hyoung F; Hikosaka, Okihide

    2015-07-01

    The basal ganglia control body movements, value processing and decision-making. Many studies have shown that the inputs and outputs of each basal ganglia structure are topographically organized, which suggests that the basal ganglia consist of separate circuits that serve distinct functions. A notable example is the circuits that originate from the rostral (head) and caudal (tail) regions of the caudate nucleus, both of which target the superior colliculus. These two caudate regions encode the reward values of visual objects differently: flexible (short-term) values by the caudate head and stable (long-term) values by the caudate tail. These value signals in the caudate guide the orienting of gaze differently: voluntary saccades by the caudate head circuit and automatic saccades by the caudate tail circuit. Moreover, separate groups of dopamine neurons innervate the caudate head and tail and may selectively guide the flexible and stable learning/memory in the caudate regions. Studies focusing on manual handling of objects also suggest that rostrocaudally separated circuits in the basal ganglia control the action differently. These results suggest that the basal ganglia contain parallel circuits for two steps of goal-directed behaviour: finding valuable objects and manipulating the valuable objects. These parallel circuits may underlie voluntary behaviour and automatic skills, enabling animals (including humans) to adapt to both volatile and stable environments. This understanding of the functions and mechanisms of the basal ganglia parallel circuits may inform the differential diagnosis and treatment of basal ganglia disorders. PMID:25981958

  6. A neuromotor model of handwriting generation highlighting the role of basal ganglia

    OpenAIRE

    Garipelli, Gangadhar

    2006-01-01

    Handwriting (HW), unlike reaching or walking, is a high-level motor activity, engaging large parts of cortical and sub-cortical regions that include supplementary motor area(SMA), premotor area(PM), primary motor area(M1), basal ganglia(BG), cerebellum, spinal cord etc. Since each of these regions contributes to HW output in its own unique fashion, pathology of any of these regions is manifest as characteristic features in HW. For example, in Parkinson's disease, a disorder of BG, HW is marke...

  7. 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

  8. Re-evaluation of the functional anatomy of the basal ganglia in normal and Parkinsonian states.

    Science.gov (United States)

    Levy, R; Hazrati, L N; Herrero, M T; Vila, M; Hassani, O K; Mouroux, M; Ruberg, M; Asensi, H; Agid, Y; Féger, J; Obeso, J A; Parent, A; Hirsch, E C

    1997-01-01

    In the late 1980s, a functional and anatomical model of basal ganglia organization was proposed in order to explain the clinical syndrome of Parkinson's disease. According to this model, the pathological overactivity observed in the subthalamic nucleus and the output station of the basal ganglia plays a crucial role in the pathophysiology of the motor signs of Parkinson's disease. The hyperactivity of subthalamic neurons in Parkinsonism is viewed as a direct consequence of a pathological hypoactivity of the external segment of the pallidum. This article reviews recent data from different experimental approaches that challenge the established model of basal ganglia organization by reinterpreting the functional interaction between the external segment of the pallidum and the subthalamic nucleus in both the normal and pathological state. Indeed, recent neurobiochemical studies have rather unexpectedly shown that the GABAergic and metabolic activities of the external pallidum are not decreased in human and non-human primates with Parkinsonism. This absence of any decrease in activity might be explained by the functionally antagonistic influences of the striatal and subthalamic afferences within the external pallidum, as suggested by several anatomical studies. In addition, there are clues from electrophysiological studies to suggest that the hyperactivity found in the subthalamic neurons in Parkinsonism may not depend solely on the level of activity in the external pallidum. In such a framework, the hyperactivity of the subthalamic neurons would have to be explained, at least in part, by other sources of excitation or disinhibition. However, any explanation for the origin of the subthalamic overactivity in Parkinsonism remains speculative.

  9. Refractory epilepsy and basal ganglia: the role of seizure frequency

    Energy Technology Data Exchange (ETDEWEB)

    Bouilleret, V.; Trebossen, R.; Mantzerides, M.; Semah, F.; Ribeiro, M.J. [Service Hospitalier Frederic Joliot, I2BM/DSV, CEA, 91 - Orsay (France); Bouilleret, V. [CHU Bicetre, Unite de Neurophysiologie et d' Epileptologie, AP-HP, 75 - Paris (France); Chassoux, F. [Hopital Saint Anne, Service de Neurochirurgie, 75 - Paris (France); Biraben, A. [CHU, Service de Neurologie, Hopital Pontchaillou, 35 - Rennes (France)

    2008-02-15

    Objectives. - A decrease of [{sup 18}F]Fluoro-L-DOPA uptake in basal ganglia (B.G.) was recently reported in medically refractory epilepsy. The purpose of this study was to assess the involvement of dopaminergic neurotransmission in refractory Temporal Lobe Epilepsy (T.L.E.) and its relationship to glucose metabolism and morphological changes. Methods. - Twelve T.L.E. patients were studied using [{sup 18}F]FDG PET, [{sup 18}F]Fluoro-L-DOPA PET and MRI and compared with healthy control volunteers. Morphological cerebral changes were assessed using Voxel-Based Morphometry (V.B.M.). Student t test statistical maps of functional and morphological differences between patients and controls were obtained using a general linear model. Results. - In T.L.E. patients, [{sup 18}F]Fluoro-L-DOPA uptake was reduced to the same extent in caudate and putamen in both cerebral hemispheres as well as in the substantia nigra (S.N.). These dopaminergic functional alterations occurred without any glucose metabolism changes in these areas. The only mild morphological abnormality was found in striatal regions without any changes in the S.N.. Conclusion. - The present study provides support for dopaminergic neurotransmission involvement in T.L.E.. The discrepancies between G.M.V. atrophy and the pattern of [{sup 18}F]Fluoro-L-DOPA suggest that B.G. involvement is not related to structural subcortical abnormalities. A functional decrease can be ruled out as there was no change of the glycolytic pathway metabolism in these areas. (authors)

  10. Endoscopic considerations treating hydrocephalus caused by basal ganglia and large thalamic tumors

    Directory of Open Access Journals (Sweden)

    Jonathan Roth

    2015-01-01

    Conclusions: Endoscopic surgery may potentially play a significant role in the initial management of patients with large basal ganglia and large thalamic tumors causing obstructive hydrocephalus. Technical nuances and individualized goals are crucial for optimal outcomes.

  11. Actor-critic models of the basal ganglia: new anatomical and computational perspectives.

    Science.gov (United States)

    Joel, Daphna; Niv, Yael; Ruppin, Eytan

    2002-01-01

    A large number of computational models of information processing in the basal ganglia have been developed in recent years. Prominent in these are actor-critic models of basal ganglia functioning, which build on the strong resemblance between dopamine neuron activity and the temporal difference prediction error signal in the critic, and between dopamine-dependent long-term synaptic plasticity in the striatum and learning guided by a prediction error signal in the actor. We selectively review several actor-critic models of the basal ganglia with an emphasis on two important aspects: the way in which models of the critic reproduce the temporal dynamics of dopamine firing, and the extent to which models of the actor take into account known basal ganglia anatomy and physiology. To complement the efforts to relate basal ganglia mechanisms to reinforcement learning (RL), we introduce an alternative approach to modeling a critic network, which uses Evolutionary Computation techniques to 'evolve' an optimal RL mechanism, and relate the evolved mechanism to the basic model of the critic. We conclude our discussion of models of the critic by a critical discussion of the anatomical plausibility of implementations of a critic in basal ganglia circuitry, and conclude that such implementations build on assumptions that are inconsistent with the known anatomy of the basal ganglia. We return to the actor component of the actor-critic model, which is usually modeled at the striatal level with very little detail. We describe an alternative model of the basal ganglia which takes into account several important, and previously neglected, anatomical and physiological characteristics of basal ganglia-thalamocortical connectivity and suggests that the basal ganglia performs reinforcement-biased dimensionality reduction of cortical inputs. We further suggest that since such selective encoding may bias the representation at the level of the frontal cortex towards the selection of rewarded

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

    OpenAIRE

    Aaron McMurtray; Ben Tseng; Natalie Diaz; Julia Chung; Bijal Mehta; Erin Saito

    2014-01-01

    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 ...

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

    OpenAIRE

    F. Montes-Gonzalez; Prescott, T.J; Negrete-Martinez, J.

    2007-01-01

    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 selec...

  14. Single-voxel proton MR spectroscopy of the basal ganglia in patients with neurofibromatosis type 1

    International Nuclear Information System (INIS)

    To demonstrate the proton MR spectroscopic characteristics of non-neoplastic focal basal ganglia lesions with high signal intensity on long TR MR images in patients with neurofibromatosis type 1(NF-1), and to compare them with those of normal-appearing basal ganglia in patients without focal lesions. Materials and Methods: Single-voxel proton MR spectroscopy was performed in six patients with NF-1 from two families(three with and three without non-neoplastic focal brain lesions). All six individual spectra were obtained from basal ganglia with voxel sizes of about 1 x 1 x 1 cm, three from focal pallidal lesions in patients with focal lesions and three from normal-appearing basal ganglia in patients without focal lesions. Spectra were acquired using a 1.5T clinical MR imager and stimulated echo acquisition mode sequence, with the following parameters: 30 ms of echo time, 13.7ms of mixing time, and 2560 ms of repetition time. Zero and first-order phase correction was performed. Results :N-acetyl aspartate(NAA)/creatine(Cr) ratios were similar between focal basal ganglia lesions and normal-appearing basal ganglia, though the former showed slightly lower choline(Cho)/Cr ratios and slightly higher NAA/Cho ratios than the latter. Relatively enhanced resonances around 3.75 ppm, assigned as glutamate/glutamine, were observed in the spectra of three focal lesions. Lipid resonances around slightly different positions were observed in all six patients, regardless of the presence or absence of focal lesions. Conclusion : Slightly decreased Cho levels and relatively enhanced glutamate/glutamine resonances are thought to characterize the focal basal ganglia lesions of NF-1. Different mobile lipids appear to be present in the basal ganglia of NF-1 patients, regardless of the presence of focal lesions

  15. Acute Chorea Characterized by Bilateral Basal Ganglia Lesions in a Patient with Diabetic Nephropathy

    OpenAIRE

    İbrahim DOĞAN; Serdar KAHVECİOĞLU; Kurtoğlu, Ünal; YILDIZ, DEMET; Abdulmecit YILDIZ

    2015-01-01

    The syndrome of acute bilateral basal ganglia lesions associated with uremia presents with parkinsonism, altered mental status, and chorea in association with specific imaging findings in the basal ganglia. It is an uncommon syndrome seen generally in patients with diabetes mellitus and renal failure. We report a male patient with diabetes mellitus who received hemodialysis treatment 3 days a week for 5 years and suffered from choreic movements developed suddenly and associated with bilateral...

  16. MR imaging study of tumors originating in the basal ganglia and thalamus in children

    International Nuclear Information System (INIS)

    Objective: To study and compare the clinical and MR imaging characteristics of tumors originating in the basal ganglia and thalamus in children. Analysis was focussed on the relationship of the sex, location and the signal characteristics of the tumors. Methods: MR imaging studies of 36 children (23 boys and 13 girls; ranging in age from 3-15 years; mean age, 10.7 years) with the tumors arising from the basal ganglia and thalamus were reviewed retrospectively. The tumors included 15 astrocytomas, 8 glioblastomas, 9 germinoma, 2 malignant teratomas and 2 gangliocytomas. All had surgery, with pathologic confirmation. Results: In 23 gliomas, 1 was located in basal ganglia and 22 in thalamus. There was a slight male predilection. All germinomas and malignant teratomas were male in this group. In germinoma and malignant teratoma group, 2 germinomas and one malignant teratoma were in thalamus, the others were in basal ganglia. Two gangliocytomas were female and located in thalamus. Conclusion: The tumors originating in the basal ganglia and thalamus in children have sex, located in thalamus. Conclusion: The tumors originating in the basal ganglia and thalamus in children have sex, location and imaging characteristics. Therefore, it is possible to make preoperative diagnosis using CT or MR

  17. The basal ganglia and rule-governed language use: evidence from vascular and degenerative conditions.

    Science.gov (United States)

    Longworth, C E; Keenan, S E; Barker, R A; Marslen-Wilson, W D; Tyler, L K

    2005-03-01

    The Declarative/Procedural Model of Pinker, Ullman and colleagues claims that the basal ganglia are part of a fronto-striatal procedural memory system which applies grammatical rules to combine morphemes (the smallest meaningful units in language) into complex words (e.g. talk-ed, talk-ing). We tested this claim by investigating whether striatal damage or loss of its dopaminergic innervation is reliably associated with selective regular past tense deficits in patients with subcortical cerebrovascular damage, Parkinson's disease or Huntington's disease. We focused on past tense morphology since this allows us to contrast the regular past tense (jump-jumped), which is rule-based, with the irregular past tense (sleep-slept), which is not. We used elicitation and priming tasks to test patients' ability to comprehend and produce inflected forms. We found no evidence of a consistent association between striatal dysfunction and selective impairment of regular past tense morphology, suggesting that the basal ganglia are not essential for processing the regular past tense as a sequence of morphemes, either in comprehension or production, in contrast to the claims of the Declarative/Procedural Model. All patient groups showed normal activation of semantic and morphological representations in comprehension, despite difficulties suppressing semantically appropriate alternatives when trying to inflect novel verbs. This is consistent with previous reports that striatal dysfunction spares automatic activation of linguistic information, but disrupts later language processes that require inhibition of competing alternatives. PMID:15659423

  18. 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.

  19. Role of movement in long-term basal ganglia changes: implications for abnormal motor responses

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    Nicola eSimola

    2013-10-01

    Full Text Available Abnormal involuntary movements and dyskinesias elicited by drugs that stimulate dopamine receptors in the basal ganglia are a major issue in the management of Parkinson’s disease (PD. Preclinical studies in dopamine-denervated animals have contributed to the modeling of these abnormal movements, but the precise neurochemical and functional mechanisms underlying these untoward effects are still elusive. It has recently been suggested that the performance of movement may itself promote the later emergence of drug-induced motor complications, by favoring the generation of aberrant motor memories in the dopamine-denervated basal ganglia. Our recent results from hemiparkinsonian rats subjected to the priming model of dopaminergic stimulation are in agreement with this and may constitute a useful model to study the early neurochemical events underling dyskinesia. These results demonstrate that early performance of movement is crucial for the manifestation of sensitized rotational behavior, indicative of an abnormal motor response, and neurochemical modifications in selected striatal neurons following a dopaminergic challenge. Building on this evidence, this paper discusses the possible role of movement performance in drug-induced motor complications, with a look at the implications for PD management.

  20. Neurotensin receptor binding levels in basal ganglia are not altered in Huntington's chorea or schizophrenia

    International Nuclear Information System (INIS)

    Autoradiographic techniques were used to examine the distribution and levels of neurotensin receptor binding sites in the basal ganglia and related regions of the human brain. Monoiodo (125I-Tyr3)neurotensin was used as a ligand. High amounts of neurotensin receptor binding sites were found in the substantia nigra pars compacta. Lower but significant quantities of neurotensin receptor binding sites characterized the caudate, putamen, and nucleus accumbens, while very low quantities were seen in both medial and lateral segments of the globus pallidus. In Huntington's chorea, the levels of neurotensin receptor binding sites were found to be comparable to those of control cases. Only slight but not statistically significant decreases in amounts of receptor binding sites were detected in the dorsal part of the head and in the body of caudate nucleus. No alterations in the levels of neurotensin receptor binding sites were observed in the substantia nigra pars compacta and reticulata. These results suggest that a large proportion of neurotensin receptor binding sites in the basal ganglia are located on intrinsic neurons and on extrinsic afferent fibers that do not degenerate in Huntington's disease

  1. Dopaminergic Control of the Exploration-Exploitation Trade-Off via the Basal Ganglia

    Science.gov (United States)

    Humphries, Mark D.; Khamassi, Mehdi; Gurney, Kevin

    2012-01-01

    We continuously face the dilemma of choosing between actions that gather new information or actions that exploit existing knowledge. This “exploration-exploitation” trade-off depends on the environment: stability favors exploiting knowledge to maximize gains; volatility favors exploring new options and discovering new outcomes. Here we set out to reconcile recent evidence for dopamine’s involvement in the exploration-exploitation trade-off with the existing evidence for basal ganglia control of action selection, by testing the hypothesis that tonic dopamine in the striatum, the basal ganglia’s input nucleus, sets the current exploration-exploitation trade-off. We first advance the idea of interpreting the basal ganglia output as a probability distribution function for action selection. Using computational models of the full basal ganglia circuit, we showed that, under this interpretation, the actions of dopamine within the striatum change the basal ganglia’s output to favor the level of exploration or exploitation encoded in the probability distribution. We also found that our models predict striatal dopamine controls the exploration-exploitation trade-off if we instead read-out the probability distribution from the target nuclei of the basal ganglia, where their inhibitory input shapes the cortical input to these nuclei. Finally, by integrating the basal ganglia within a reinforcement learning model, we showed how dopamine’s effect on the exploration-exploitation trade-off could be measurable in a forced two-choice task. These simulations also showed how tonic dopamine can appear to affect learning while only directly altering the trade-off. Thus, our models support the hypothesis that changes in tonic dopamine within the striatum can alter the exploration-exploitation trade-off by modulating the output of the basal ganglia. PMID:22347155

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

    International Nuclear Information System (INIS)

    Dual probe microdialysis was employed in intact rat brain to investigate the effect of intrastriatal perfusion with selective dopamine D1 and D2 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 D1 receptor-mediated activation of the direct strionigral GABA pathway and a selective dopamine D2 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

  3. Selection of cortical dynamics for motor behaviour by the basal ganglia.

    Science.gov (United States)

    Mannella, Francesco; Baldassarre, Gianluca

    2015-12-01

    The basal ganglia and cortex are strongly implicated in the control of motor preparation and execution. Re-entrant loops between these two brain areas are thought to determine the selection of motor repertoires for instrumental action. The nature of neural encoding and processing in the motor cortex as well as the way in which selection by the basal ganglia acts on them is currently debated. The classic view of the motor cortex implementing a direct mapping of information from perception to muscular responses is challenged by proposals viewing it as a set of dynamical systems controlling muscles. Consequently, the common idea that a competition between relatively segregated cortico-striato-nigro-thalamo-cortical channels selects patterns of activity in the motor cortex is no more sufficient to explain how action selection works. Here, we contribute to develop the dynamical view of the basal ganglia-cortical system by proposing a computational model in which a thalamo-cortical dynamical neural reservoir is modulated by disinhibitory selection of the basal ganglia guided by top-down information, so that it responds with different dynamics to the same bottom-up input. The model shows how different motor trajectories can so be produced by controlling the same set of joint actuators. Furthermore, the model shows how the basal ganglia might modulate cortical dynamics by preserving coarse-grained spatiotemporal information throughout cortico-cortical pathways.

  4. Exercise-induced changes in basal ganglia volume and cognition in older adults.

    Science.gov (United States)

    Niemann, C; Godde, B; Staudinger, U M; Voelcker-Rehage, C

    2014-12-01

    Physical activity has been demonstrated to diminish age-related brain volume shrinkage in several brain regions accompanied by a reduction of age-related decline in cognitive functions. Most studies investigated the impact of cardiovascular fitness or training. Other types of fitness or training are less well investigated. In addition, little is known about exercise effects on volume of the basal ganglia, which, however, are involved in motor activities and cognitive functioning. In the current study (1) we examined the relationships of individual cardiovascular and motor fitness levels with the volume of the basal ganglia (namely caudate, putamen, and globus pallidus) and selected cognitive functions (executive control, perceptual speed). (2) We investigated the effect of 12-month training interventions (cardiovascular and coordination training, control group stretching and relaxation) on the volume of the respective basal ganglia nuclei. Results revealed that motor fitness but not cardiovascular fitness was positively related with the volume of the putamen and the globus pallidus. Additionally, a moderating effect of the volume of the basal ganglia (as a whole, but also separately for putamen and globus pallidus) on the relationship between motor fitness and executive function was revealed. Coordination training increased caudate and globus pallidus volume. We provide evidence that coordinative exercise seems to be a favorable leisure activity for older adults that has the potential to improve volume of the basal ganglia. PMID:25255932

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

    International Nuclear Information System (INIS)

    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.)

  6. 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.)

  7. Exploring the cognitive and motor functions of the basal ganglia: An integrative review of computational cognitive neuroscience models

    Directory of Open Access Journals (Sweden)

    Sebastien eHelie

    2013-12-01

    Full Text Available Many computational models of the basal ganglia have been proposed over the past twenty-five years. While computational neuroscience models have focused on closely matching the neurobiology of the basal ganglia, computational cognitive neuroscience models have focused on how the basal ganglia can be used to implement cognitive and motor functions. This review article focuses on computational cognitive neuroscience models of the basal ganglia and how they use the neuroanatomy of the basal ganglia to account for cognitive and motor functions such as categorization, instrumental conditioning, probabilistic learning, working memory, sequence learning, automaticity, reaching, handwriting, and eye saccades. A total of 19 basal ganglia models accounting for one or more of these functions are reviewed and compared. The review concludes with a discussion of the limitations of existing computational cognitive neuroscience models of the basal ganglia and prescriptions for future modeling, including the need for computational models of the basal ganglia that can simultaneously account for cognitive and motor functions, and the need for a more complete specification of the role of the basal ganglia in behavioral functions.

  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. 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.

  10. MR-DTI and PET multimodal imaging of dopamine release within subdivisions of basal ganglia

    Science.gov (United States)

    Tziortzi, A.; Searle, G.; Tsoumpas, C.; Long, C.; Shotbolt, P.; Rabiner, E.; Jenkinson, M.; Gunn, R. N.

    2011-09-01

    The basal ganglia is a group of anatomical nuclei, functionally organised into limbic, associative and sensorimotor regions, which plays a central role in dopamine related neurological and psychiatric disorders. In this study, we combine two imaging modalities to enable the measurement of dopamine release in functionally related subdivisions of the basal ganglia. [11C]-(+)-PHNO Positron Emission Tomography (PET) measurements in the living human brain pre- and post-administration of amphetamine allow for the estimation of regional dopamine release. Combined Magnetic Resonance Diffusion Tensor Imaging (MR-DTI) data allows for the definition of functional territories of the basal ganglia from connectivity information. The results suggest that there is a difference in dopamine release among the connectivity derived functional subdivisions. Dopamine release is highest in the limbic area followed by the sensorimotor and then the associative area with this pattern reflected in both striatum and pallidum.

  11. 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

    2013-01-01

    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.

  12. Developmental Venous Anomaly With Asymmetrical Basal Ganglia Calcification: Two Case Reports and Review of the Literature

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    Sarp

    2015-07-01

    Full Text Available Developmental venous anomaly (DVA is a common lesion formerly known as venous angioma. DVAs drain normal brain parenchyma; however, parenchymal abnormalities surrounding DVAs have been reported. Unilateral putamen and caudate calcification in the drainage territory of DVAs has so far been reported in 7 cases, all with deep venous drainage. We present two additional cases of DVAs, one with superficial and the other one with deep venous drainage, associated with basal ganglia calcifications. We emphasize that DVAs should be in the differential diagnosis of unilateral basal ganglia calcifications.

  13. Late-Onset Mania in a Patient with Movement Disorder and Basal Ganglia Calcifications: A Challenge for Diagnosis and Treatment

    Science.gov (United States)

    Roiter, Beatrice; Pigato, Giorgio; Perugi, Giulio

    2016-01-01

    Age of onset can have a significant impact on clinical course and pathophysiological mechanism of bipolar disorder. Late-onset bipolar episodes are more likely linked to medical illnesses and so are frequently classified as “secondary” forms of mood disorder. We discuss the case of a patient who at the age of 58 presented his first delusional-manic episode. He also had mild frontal and occipital cortical atrophy, white matter posterior ischemic lesions, and small basal ganglia calcifications. Seven years later, he presented a second manic episode with new emergent hyperkinetic choreiform symptoms. Taking into account movement disturbances, the presence of basal ganglia calcification, and worsening of cortical atrophy, we performed a differential diagnosis between Fahr disease, Fahr's syndrome, calcifications due to ageing, supersensitivity psychosis, and dementia. Valproate, quetiapine, and tetrabenazine were sequentially administered and yielded a good therapeutic response as regards manic and movement symptoms. Relationship between medications and course of specific symptoms was observed. PMID:27213069

  14. Coupling in the cortico-basal ganglia circuit is aberrant in the ketamine model of schizophrenia.

    Science.gov (United States)

    Cordon, Ivan; Nicolás, María Jesús; Arrieta, Sandra; Lopetegui, Eneko; López-Azcárate, Jon; Alegre, Manuel; Artieda, Julio; Valencia, Miguel

    2015-08-01

    Recent studies have suggested the implication of the basal ganglia in the pathogenesis of schizophrenia. To investigate this hypothesis, here we have used the ketamine model of schizophrenia to determine the oscillatory abnormalities induced in the rat motor circuit of the basal ganglia. The activity of free moving rats was recorded in different structures of the cortico-basal ganglia circuit before and after an injection of a subanesthesic dose of ketamine (10mg/kg). Spectral estimates of the oscillatory activity, phase-amplitude cross-frequency coupling interactions (CFC) and imaginary event-related coherence together with animals׳ behavior were analyzed. Oscillatory patterns in the cortico-basal ganglia circuit were highly altered by the effect of ketamine. CFC between the phases of low-frequency activities (delta, 1-4; theta 4-8Hz) and the amplitude of high-gamma (~80Hz) and high-frequency oscillations (HFO) (~150Hz) increased dramatically and correlated with the movement increment shown by the animals. Between-structure analyses revealed that ketamine had also a massive effect in the low-frequency mediated synchronization of the HFO's across the whole circuit. Our findings suggest that ketamine administration results in an aberrant hypersynchronization of the whole cortico-basal circuit where the tandem theta/HFO seems to act as the main actor in the hyperlocomotion shown by the animals. Here we stress the importance of the basal ganglia circuitry in the ketamine model of schizophrenia and leave the door open to further investigations devoted to elucidate to what extent these abnormalities also reflect the prominent neurophysiological deficits observed in schizophrenic patients.

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

    NARCIS (Netherlands)

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

    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 a

  16. Functions of the cortico-basal ganglia circuits for spoken language may extend beyond emotional-affective modulation in adults.

    Science.gov (United States)

    Hanakawa, Takashi; Hosoda, Chihiro

    2014-12-01

    We support Ackermann et al.'s proposal that the cortico-basal ganglia circuits may play essential roles in the evolution of spoken language. Here we discuss further evidence indicating that the cortico-basal ganglia circuits may contribute to various aspects of spoken language including planning, learning, and controlling of speech in adulthood.

  17. Conditional Routing of Information to the Cortex: A Model of the Basal Ganglia's Role in Cognitive Coordination

    Science.gov (United States)

    Stocco, Andrea; Lebiere, Christian; Anderson, John R.

    2010-01-01

    The basal ganglia play a central role in cognition and are involved in such general functions as action selection and reinforcement learning. Here, we present a model exploring the hypothesis that the basal ganglia implement a conditional information-routing system. The system directs the transmission of cortical signals between pairs of regions…

  18. The Differential Effects of Thalamus and Basal Ganglia on Facial Emotion Recognition

    Science.gov (United States)

    Cheung, Crystal C. Y.; Lee, Tatia M. C.; Yip, James T. H.; King, Kristin E.; Li, Leonard S. W.

    2006-01-01

    This study examined if subcortical stroke was associated with impaired facial emotion recognition. Furthermore, the lateralization of the impairment and the differential profiles of facial emotion recognition deficits with localized thalamic or basal ganglia damage were also studied. Thirty-eight patients with subcortical strokes and 19 matched…

  19. Opponent and bidirectional control of movement velocity in the basal ganglia.

    Science.gov (United States)

    Yttri, Eric A; Dudman, Joshua T

    2016-05-19

    For goal-directed behaviour it is critical that we can both select the appropriate action and learn to modify the underlying movements (for example, the pitch of a note or velocity of a reach) to improve outcomes. The basal ganglia are a critical nexus where circuits necessary for the production of behaviour, such as the neocortex and thalamus, are integrated with reward signalling to reinforce successful, purposive actions. The dorsal striatum, a major input structure of basal ganglia, is composed of two opponent pathways, direct and indirect, thought to select actions that elicit positive outcomes and suppress actions that do not, respectively. Activity-dependent plasticity modulated by reward is thought to be sufficient for selecting actions in the striatum. Although perturbations of basal ganglia function produce profound changes in movement, it remains unknown whether activity-dependent plasticity is sufficient to produce learned changes in movement kinematics, such as velocity. Here we use cell-type-specific stimulation in mice delivered in closed loop during movement to demonstrate that activity in either the direct or indirect pathway is sufficient to produce specific and sustained increases or decreases in velocity, without affecting action selection or motivation. These behavioural changes were a form of learning that accumulated over trials, persisted after the cessation of stimulation, and were abolished in the presence of dopamine antagonists. Our results reveal that the direct and indirect pathways can each bidirectionally control movement velocity, demonstrating unprecedented specificity and flexibility in the control of volition by the basal ganglia.

  20. Bidirectional Plasticity in Striatonigral Synapses: A Switch to Balance Direct and Indirect Basal Ganglia Pathways

    Science.gov (United States)

    Aceves, Jose J.; Rueda-Orozco, Pavel E.; Hernandez-Martinez, Ricardo; Galarraga, Elvira; Bargas, Jose

    2011-01-01

    There is no hypothesis to explain how direct and indirect basal ganglia (BG) pathways interact to reach a balance during the learning of motor procedures. Both pathways converge in the substantia nigra pars reticulata (SNr) carrying the result of striatal processing. Unfortunately, the mechanisms that regulate synaptic plasticity in striatonigral…

  1. 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.N.; 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...

  2. Association Between Invisible Basal Ganglia and ZNF335 Mutations: A Case Report.

    Science.gov (United States)

    Sato, Rieko; Takanashi, Jun-Ichi; Tsuyusaki, Yu; Kato, Mitsuhiro; Saitsu, Hirotomo; Matsumoto, Naomichi; Takahashi, Takao

    2016-09-01

    ZNF335 was first reported in 2012 as a causative gene for microcephaly. Because only 1 consanguineous pedigree has ever been reported, the key clinical features associated with ZNF335 mutations remain unknown. In this article, we describe another family harboring ZNF335 mutations. The female proband was the first child of nonconsanguineous Japanese parents. At birth, microcephaly was absent; her head circumference was 32.0 cm (-0.6 SD). At 3 months, microcephaly was noted, (head circumference, 34.0 cm [-4.6 SD]). Brain MRI showed invisible basal ganglia, cerebral atrophy, brainstem hypoplasia, and cerebellar atrophy. At 33 months, (head circumference, 41.0 cm [-5.1 SD]), she had severe psychomotor retardation. After obtaining informed consent from her parents, we performed exome sequencing in the proband and identified 1 novel and 1 known mutation in ZNF335, namely, c.1399T>C (p.C467R) and c.1505A>G (p.Y502C), respectively. The mutations were individually transmitted by her parents, indicating that the proband was compound heterozygous for the mutations. Her brain imaging findings, including invisible basal ganglia, were similar to those observed in the previous case with ZNF335 mutations. We speculate that invisible basal ganglia may be the key feature of ZNF335 mutations. For infants presenting with both microcephaly and invisible basal ganglia, ZNF335 mutations should be considered as a differential diagnosis. PMID:27540107

  3. Opponent and bidirectional control of movement velocity in the basal ganglia.

    Science.gov (United States)

    Yttri, Eric A; Dudman, Joshua T

    2016-05-02

    For goal-directed behaviour it is critical that we can both select the appropriate action and learn to modify the underlying movements (for example, the pitch of a note or velocity of a reach) to improve outcomes. The basal ganglia are a critical nexus where circuits necessary for the production of behaviour, such as the neocortex and thalamus, are integrated with reward signalling to reinforce successful, purposive actions. The dorsal striatum, a major input structure of basal ganglia, is composed of two opponent pathways, direct and indirect, thought to select actions that elicit positive outcomes and suppress actions that do not, respectively. Activity-dependent plasticity modulated by reward is thought to be sufficient for selecting actions in the striatum. Although perturbations of basal ganglia function produce profound changes in movement, it remains unknown whether activity-dependent plasticity is sufficient to produce learned changes in movement kinematics, such as velocity. Here we use cell-type-specific stimulation in mice delivered in closed loop during movement to demonstrate that activity in either the direct or indirect pathway is sufficient to produce specific and sustained increases or decreases in velocity, without affecting action selection or motivation. These behavioural changes were a form of learning that accumulated over trials, persisted after the cessation of stimulation, and were abolished in the presence of dopamine antagonists. Our results reveal that the direct and indirect pathways can each bidirectionally control movement velocity, demonstrating unprecedented specificity and flexibility in the control of volition by the basal ganglia.

  4. A resting state network in the motor control circuit of the basal ganglia

    Directory of Open Access Journals (Sweden)

    Bruzzone Lorenzo

    2009-11-01

    Full Text Available Abstract Background In the absence of overt stimuli, the brain shows correlated fluctuations in functionally related brain regions. Approximately ten largely independent resting state networks (RSNs showing this behaviour have been documented to date. Recent studies have reported the existence of an RSN in the basal ganglia - albeit inconsistently and without the means to interpret its function. Using two large study groups with different resting state conditions and MR protocols, the reproducibility of the network across subjects, behavioural conditions and acquisition parameters is assessed. Independent Component Analysis (ICA, combined with novel analyses of temporal features, is applied to establish the basis of signal fluctuations in the network and its relation to other RSNs. Reference to prior probabilistic diffusion tractography work is used to identify the basal ganglia circuit to which these fluctuations correspond. Results An RSN is identified in the basal ganglia and thalamus, comprising the pallidum, putamen, subthalamic nucleus and substantia nigra, with a projection also to the supplementary motor area. Participating nuclei and thalamo-cortical connection probabilities allow this network to be identified as the motor control circuit of the basal ganglia. The network was reproducibly identified across subjects, behavioural conditions (fixation, eyes closed, field strength and echo-planar imaging parameters. It shows a frequency peak at 0.025 ± 0.007 Hz and is most similar in spectral composition to the Default Mode (DM, a network of regions that is more active at rest than during task processing. Frequency features allow the network to be classified as an RSN rather than a physiological artefact. Fluctuations in this RSN are correlated with those in the task-positive fronto-parietal network and anticorrelated with those in the DM, whose hemodynamic response it anticipates. Conclusion Although the basal ganglia RSN has not been

  5. Familial idiopathic basal ganglia calcification: Histopathologic features of an autopsied patient with an SLC20A2 mutation.

    Science.gov (United States)

    Kimura, Tadashi; Miura, Takeshi; Aoki, Kenju; Saito, Shoji; Hondo, Hiroaki; Konno, Takuya; Uchiyama, Akio; Ikeuchi, Takeshi; Takahashi, Hitoshi; Kakita, Akiyoshi

    2016-08-01

    Idiopathic basal ganglia calcification (IBGC), or Fahr's disease, is a neurological disorder characterized by widespread calcification in the brain. Recently, several causative genes have been identified, but the histopathologic features of the brain lesions and expression of the gene products remain unclear. Here, we report the clinical and autopsy features of a 62-year-old Japanese man with familial IBGC, in whom an SLC20A2 mutation was identified. The patient developed mild cognitive impairment and parkinsonism. A brain CT scan demonstrated abnormal calcification in the bilateral basal ganglia, thalami and cerebellum. An MRI study at this point revealed glioblastoma, and the patient died 6 months later. At autopsy, symmetric calcification in the basal ganglia, thalami, cerebellar white matter and deeper layers of the cerebral cortex was evident. The calcification was observed in the tunica media of small arteries, arterioles and capillaries, but not in veins. Immunohistochemistry using an antibody against type III sodium-dependent phosphate transporter 2 (PiT-2), the SLC20A2 product, demonstrated that astrocytic processes were labeled in several regions in control brains, whereas in the patient, reactivity in astrocytes was apparently weak. Immunoblotting demonstrated a marked decrease of PiT-2 in the patient. There are few autopsy reports of IBGC patients with confirmation of the genetic background. The autopsy features seem informative for better understanding the histogenesis of IBGC lesions. PMID:26635128

  6. Evidence for a glutamatergic projection from the zona incerta to the basal ganglia of rats.

    Science.gov (United States)

    Heise, Claire E; Mitrofanis, John

    2004-01-19

    This study explores the organisation and neurochemical nature of the projections from the zona incerta (ZI) to the basal ganglia. Sprague-Dawley rats were anaesthetised with ketamine (100 mg/kg) and Rompun (10 mg/kg), and injections of cholera toxin subunit B were made into each of the following nuclei: the ZI, the substantia nigra (SN), the pedunculopontine tegmental nucleus (PpT), and the entopeduncular nucleus (Ep). Brains were aldehyde fixed, sectioned, and processed using standard methods. Tracer-labelled sections were then doubly labelled with antibodies to glutamate (Glu), nitric oxide synthase (NOS), parvalbumin (Pv), or glutamic acid decarboxylase (GAD; the latter two are markers for GABAergic cells); these neurochemicals characterise most types of ZI cells. After ZI injections, labelling was nonuniform across the different basal ganglia nuclei. The bulk of labelling, both anterograde and retrograde, was seen in the SN and PpT and, to a lesser extent, within the other nuclei of the basal ganglia (e.g., caudate-putamen, globus pallidus, subthalamus, Ep). In the SN, labelling was found in both major parts of the nucleus, the pars compacta and pars reticulata. Within the PpT, however, the bulk of labelling was limited to only one of the two sectors of the nucleus, namely, the pars dissipata (PpTd). The pars compacta of the PpT (PpTc) remained largely free of labelled profiles. After CTb injections into three basal ganglia nuclei (SN, PpT, Ep), most labelled cells in the ZI were glutamate+ and very few were NOS+ or gamma-aminobutyric acidergic. Overall, the results indicate that the ZI is in a position to influence preferentially the activity of the SN and PpTd of the basal ganglia via an excitatory, glutamatergic input. PMID:14689481

  7. 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.)

  8. Acute Chorea Characterized by Bilateral Basal Ganglia Lesions in a Patient with Diabetic Nephropathy

    Directory of Open Access Journals (Sweden)

    İbrahim DOĞAN

    2015-09-01

    Full Text Available The syndrome of acute bilateral basal ganglia lesions associated with uremia presents with parkinsonism, altered mental status, and chorea in association with specific imaging findings in the basal ganglia. It is an uncommon syndrome seen generally in patients with diabetes mellitus and renal failure. We report a male patient with diabetes mellitus who received hemodialysis treatment 3 days a week for 5 years and suffered from choreic movements developed suddenly and associated with bilateral basal ganglia lesions. In the brain magnetic resonance (MR imaging, isointense was detected in sequence T1 in the bilateral basal ganglions and hyperintense lesion was determined in T2 and FLAIR sequences. The patient was administered daily hemodialysis and neuroleptic treatment. After intensified hemodialysis, his symptoms and follow-up brain MR imaging showed marked improvement. The underlying mechanism of such lesions may be associated with metabolic, as well as vascular factors. Acute choreic movements may be seen in patients with diabetic nephropathy and intensification of hemodialysis treatment along with blood glucose regulation may provide improvement in this syndrome.

  9. Eyes on MEGDEL: distinctive basal ganglia involvement in dystonia deafness syndrome.

    Science.gov (United States)

    Wortmann, Saskia B; van Hasselt, Peter M; Barić, Ivo; Burlina, Alberto; Darin, Niklas; Hörster, Friederike; Coker, Mahmut; Ucar, Sema Kalkan; Krumina, Zita; Naess, Karin; Ngu, Lock H; Pronicka, Ewa; Riordan, Gilian; Santer, Rene; Wassmer, Evangeline; Zschocke, Johannes; Schiff, Manuel; de Meirleir, Linda; Alowain, Mohammed A; Smeitink, Jan A M; Morava, Eva; Kozicz, Tamas; Wevers, Ron A; Wolf, Nicole I; Willemsen, Michel A

    2015-04-01

    Pediatric movement disorders are still a diagnostic challenge, as many patients remain without a (genetic) diagnosis. Magnetic resonance imaging (MRI) pattern recognition can lead to the diagnosis. MEGDEL syndrome (3-MethylGlutaconic aciduria, Deafness, Encephalopathy, Leigh-like syndrome MIM #614739) is a clinically and biochemically highly distinctive dystonia deafness syndrome accompanied by 3-methylglutaconic aciduria, severe developmental delay, and progressive spasticity. Mutations are found in SERAC1, encoding a phosphatidylglycerol remodeling enzyme essential for both mitochondrial function and intracellular cholesterol trafficking. Based on the homogenous phenotype, we hypothesized an accordingly characteristic MRI pattern. A total of 43 complete MRI studies of 30 patients were systematically reevaluated. All patients presented a distinctive brain MRI pattern with five characteristic disease stages affecting the basal ganglia, especially the putamen. In stage 1, T2 signal changes of the pallidum are present. In stage 2, swelling of the putamen and caudate nucleus is seen. The dorsal putamen contains an "eye" that shows no signal alteration and (thus) seems to be spared during this stage of the disease. It later increases, reflecting progressive putaminal involvement. This "eye" was found in all patients with MEGDEL syndrome during a specific age range, and has not been reported in other disorders, making it pathognomonic for MEDGEL and allowing diagnosis based on MRI findings.

  10. Surprise disrupts cognition via a fronto-basal ganglia suppressive mechanism.

    Science.gov (United States)

    Wessel, Jan R; Jenkinson, Ned; Brittain, John-Stuart; Voets, Sarah H E M; Aziz, Tipu Z; Aron, Adam R

    2016-04-18

    Surprising events markedly affect behaviour and cognition, yet the underlying mechanism is unclear. Surprise recruits a brain mechanism that globally suppresses motor activity, ostensibly via the subthalamic nucleus (STN) of the basal ganglia. Here, we tested whether this suppressive mechanism extends beyond skeletomotor suppression and also affects cognition (here, verbal working memory, WM). We recorded scalp-EEG (electrophysiology) in healthy participants and STN local field potentials in Parkinson's patients during a task in which surprise disrupted WM. For scalp-EEG, surprising events engage the same independent neural signal component that indexes action stopping in a stop-signal task. Importantly, the degree of this recruitment mediates surprise-related WM decrements. Intracranially, STN activity is also increased post surprise, especially when WM is interrupted. These results suggest that surprise interrupts cognition via the same fronto-basal ganglia mechanism that interrupts action. This motivates a new neural theory of how cognition is interrupted, and how distraction arises after surprising events.

  11. Computational models of basal-ganglia pathway functions: Focus on functional neuroanatomy

    Directory of Open Access Journals (Sweden)

    Henning eSchroll

    2013-12-01

    Full Text Available Over the past 15 years, computational models have had a considerable impact on basal-ganglia research. Most of these models implement multiple distinct basal ganglia pathways and assume them to fulfill different functions. As there is now a multitude of different models, it has become complex to keep track of their various, sometimes just marginally different assumptions on pathway functions. Moreover, it has become a challenge to oversee to what extent individual assumptions are corroborated or challenged by empirical data. Focusing on computational, but also considering non-computational models, we review influential concepts of pathway functions and show to what extent they are compatible with or contradict each other. Moreover, we outline how empirical evidence favors or challenges specific assumptions and propose experiments that allow testing assumptions against each other.

  12. 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.

  13. Two Case Reports on Thalamic and Basal Ganglia Involvement in Children with Dengue Fever

    Science.gov (United States)

    Adhikari, Lihini; Wijesekera, Saraji; Wijayawardena, Maheshaka; Chandrasiri, Suchithra

    2016-01-01

    There have been increasing numbers of case reports of dengue infection with unusual manifestations. Such unusual manifestations including acute liver failure and encephalopathy could be manifested even in the absence of significant plasma leakage. Further, severe organ involvement including nervous system involvement indicates severe dengue infection. However, neurological manifestations of dengue fever are rare. This is the first case report of dengue infection with thalamic and basal ganglia involvement in Sri Lanka.

  14. Two Case Reports on Thalamic and Basal Ganglia Involvement in Children with Dengue Fever

    Science.gov (United States)

    Adhikari, Lihini; Wijesekera, Saraji; Wijayawardena, Maheshaka; Chandrasiri, Suchithra

    2016-01-01

    There have been increasing numbers of case reports of dengue infection with unusual manifestations. Such unusual manifestations including acute liver failure and encephalopathy could be manifested even in the absence of significant plasma leakage. Further, severe organ involvement including nervous system involvement indicates severe dengue infection. However, neurological manifestations of dengue fever are rare. This is the first case report of dengue infection with thalamic and basal ganglia involvement in Sri Lanka. PMID:27478661

  15. FROM REINFORCEMENT LEARNING MODELS OF THE BASAL GANGLIA TO THE PATHOPHYSIOLOGY OF PSYCHIATRIC AND NEUROLOGICAL DISORDERS

    OpenAIRE

    Maia, Tiago V.; Frank, Michael J.

    2011-01-01

    Over the last decade and a half, reinforcement learning models have fostered an increasingly sophisticated understanding of the functions of dopamine and cortico-basal ganglia-thalamo-cortical (CBGTC) circuits. More recently, these models, and the insights that they afford, have started to be used to understand key aspects of several psychiatric and neurological disorders that involve disturbances of the dopaminergic system and CBGTC circuits. We review this approach and its existing and pote...

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

    International Nuclear Information System (INIS)

    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

  17. Secondary attention deficit/hyperactivity disorder due to right basal ganglia injury: A case report

    OpenAIRE

    Ceylan, Mehmet Fatih; AKCA, Ömer Faruk

    2013-01-01

    Attention deficit/hyperactivity disorder (ADHD) is a frequent and commonly studied neuropsychiatric disorder in children and adolescents. The symptoms of ADHD include inattention and/or hyperactivity and impulsivity. Diagnosis of ADHD requires a persistent pattern of symptoms beginning before the age of 7 except for secondary ADHD. Secondary ADHD may occur as a consequence of childhood traumatic brain injury. A patient with secondary ADHD as a result of right basal ganglia injury is presented...

  18. 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.

  19. Actor-critic models of reinforcement learning in the basal ganglia: From natural to artificial rats

    OpenAIRE

    Khamassi, Mehdi; Lachèze, Loïc; Girard, Benoît; Berthoz, Alain; Guillot, Agnès

    2005-01-01

    International audience Since 1995, numerous Actor–Critic architectures for reinforcement learning have been proposed as models of dopamine-like reinforcement learning mechanisms in the rat's basal ganglia. However, these models were usually tested in different tasks, and it is then difficult to compare their efficiency for an autonomous animat. We present here the comparison of four architectures in an animat as it per forms the same reward-seeking task. This will illustrate the consequenc...

  20. Neural circuits and topographic organization of the basal ganglia and related regions.

    Science.gov (United States)

    Nakano, K

    2000-09-01

    The present review was attempted to analyze the multiple channels of basal ganglia-thalamocortical connections, and the connections of their related nuclei. The prefrontal and motor areas consist of a number of modules, which seem to provide multiple subloops of the basal ganglia-thalamocortical connections in subhuman primates. There may be a great degree of convergence of the limbic, associative and motor loops at the level of the striatum, substantia nigra, pallidum, and the subthalamic nucleus as well as the pedunculopontine nucleus. Nigral dopaminergic neurons receive limbic input directly as well as indirectly through the striosomes in the striatum. Dopamine contributes to behavioral learning by signaling motivation and reinforcement. The pedunculopontine nucleus might be involved in behavioral state control, learning and reinforcement processes, locomotion and autonomic functions. Each subdivision of the motor areas receives a mixed and weighted transthalamic input from both the cerebellum and basal ganglia. In particular, based on the author's data, the hand/arm motor area and adjacent premotor area receive strong superficial basal ganglia-thalamocortical projections as well as the deep cerebello-thalamocortical projections. These areas, have very dense corticocotrical connections with other cortical areas, receive polymodal afferents from the parietal and temporal cortices, and integrated information, via multiple routes, from the prefrontal cortex. The author suggests that the ventrolateral part of the caudal medial pallidal segment (GPi) and the ventromedial part of the GPi are linked directly to these areas by ways of the oral part of ventral lateral nucleus (VLo) and the ventral part of the parvicellular part of ventral anterior nucleus (VApc), respectively. These connections are thought to be involved in the acquisition and coordination of motor sequences. PMID:10984656

  1. Cardiorespiratory fitness and its association with thalamic, hippocampal, and basal ganglia volumes in multiple sclerosis

    OpenAIRE

    Motl, Robert W.; Pilutti, Lara A.; Hubbard, Elizabeth A.; Wetter, Nathan C.; Sosnoff, Jacob J.; Sutton, Bradley P.

    2015-01-01

    Background: There is little known about cardiorespiratory fitness and its association with volumes of the thalamus, hippocampus, and basal ganglia in multiple sclerosis (MS). Such inquiry is important for identifying a possible behavioral approach (e.g., aerobic exercise training) that might change volumes of deep gray matter (DGM) structures associated with cognitive and motor functions in MS. Purpose: This study examined the association between cardiorespiratory fitness and volumes of th...

  2. 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

  3. Two Case Reports on Thalamic and Basal Ganglia Involvement in Children with Dengue Fever.

    Science.gov (United States)

    Liyanage, Guwani; Adhikari, Lihini; Wijesekera, Saraji; Wijayawardena, Maheshaka; Chandrasiri, Suchithra

    2016-01-01

    There have been increasing numbers of case reports of dengue infection with unusual manifestations. Such unusual manifestations including acute liver failure and encephalopathy could be manifested even in the absence of significant plasma leakage. Further, severe organ involvement including nervous system involvement indicates severe dengue infection. However, neurological manifestations of dengue fever are rare. This is the first case report of dengue infection with thalamic and basal ganglia involvement in Sri Lanka. PMID:27478661

  4. 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.)

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

    International Nuclear Information System (INIS)

    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.)

  6. 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. PMID:21222528

  7. 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)

  8. [Cortico-basal ganglia circuits--parallel closed loops and convergent/divergent connections].

    Science.gov (United States)

    Miyachi, Shigehiro

    2009-04-01

    The basal ganglia play important roles not only in motor control but also in higher cognitive functions such as reinforcement learning and procedural memory. Anatomical studies on the neuronal connections between the basal ganglia, cerebral cortex, and thalamus have demonstrated that these nuclei and cortical areas are interconnected via independent parallel loop circuits. The association, motor, and limbic cortices project to specific domains in the striatum, which, in turn, project back to the corresponding cortical areas via the substantia nigra/globus pallidus and the thalamus. Likewise, subregions in the motor cortex representing different body parts project to specific regions in the putamen, which project back to the original motor cortical regions. These parallel loops have been thought to be the basic anatomical structures involved in the basal ganglia functions. Furthermore, neuronal projections communicating between different loops (or functional domains) have also been discovered. A considerable number of corticostriatal projections from functionally interrelated cortical areas (e. g., hand representations of the motor cortex and somatosensory cortex) converge at the striatum. It has also been suggested that the location of the substantia nigra is in such that it can transmit information from the 'limbic loop' to the 'association loop', and from the 'association loop' to the 'motor loop'. Furthermore, a recent transsynaptic neuronal tracing study conducted at our laboratory demonstrated that the ventral (limbic) striatum sends divergent outputs to multiple regions in the frontal cortex. These 'inter-loop' connections would be important for the integration of information to achieve goal-directed behaviors. PMID:19378804

  9. Supplementary motor area and presupplementary motor area: targets of basal ganglia and cerebellar output.

    Science.gov (United States)

    Akkal, Dalila; Dum, Richard P; Strick, Peter L

    2007-10-01

    We used retrograde transneuronal transport of neurotropic viruses in Cebus monkeys to examine the organization of basal ganglia and cerebellar projections to two cortical areas on the medial wall of the hemisphere, the supplementary motor area (SMA) and the pre-SMA. We found that both of these cortical areas are the targets of disynaptic projections from the dentate nucleus of the cerebellum and from the internal segment of the globus pallidus (GPi). On average, the number of pallidal neurons that project to the SMA and pre-SMA is approximately three to four times greater than the number of dentate neurons that project to these cortical areas. GPi neurons that project to the pre-SMA are located in a rostral, "associative" territory of the nucleus, whereas GPi neurons that project to the SMA are located in a more caudal and ventral "sensorimotor" territory. Similarly, dentate neurons that project to the pre-SMA are located in a ventral, "nonmotor" domain of the nucleus, whereas dentate neurons that project to the SMA are located in a more dorsal, "motor" domain. The differential origin of subcortical projections to the SMA and pre-SMA suggests that these cortical areas are nodes in distinct neural systems. Although both systems are the target of outputs from the basal ganglia and the cerebellum, these two cortical areas seem to be dominated by basal ganglia input. PMID:17913900

  10. 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.

  11. Time-course of coherence in the human basal ganglia during voluntary movements

    Science.gov (United States)

    Talakoub, Omid; Neagu, Bogdan; Udupa, Kaviraja; Tsang, Eric; Chen, Robert; Popovic, Milos R.; Wong, Willy

    2016-01-01

    We are interested in characterizing how brain networks interact and communicate with each other during voluntary movements. We recorded electrical activities from the globus pallidus pars interna (GPi), subthalamic nucleus (STN) and the motor cortex during voluntary wrist movements. Seven patients with dystonia and six patients with Parkinson’s disease underwent bilateral deep brain stimulation (DBS) electrode placement. Local field potentials from the DBS electrodes and scalp EEG from the electrodes placed over the motor cortices were recorded while the patients performed externally triggered and self-initiated movements. The coherence calculated between the motor cortex and STN or GPi was found to be coupled to its power in both the beta and the gamma bands. The association of coherence with power suggests that a coupling in neural activity between the basal ganglia and the motor cortex is required for the execution of voluntary movements. Finally, we propose a mathematical model involving coupled neural oscillators which provides a possible explanation for how inter-regional coupling takes place. PMID:27725721

  12. Changing pattern in the basal ganglia: motor switching under reduced dopaminergic drive.

    Science.gov (United States)

    Fiore, Vincenzo G; Rigoli, Francesco; Stenner, Max-Philipp; Zaehle, Tino; Hirth, Frank; Heinze, Hans-Jochen; Dolan, Raymond J

    2016-01-01

    Action selection in the basal ganglia is often described within the framework of a standard model, associating low dopaminergic drive with motor suppression. Whilst powerful, this model does not explain several clinical and experimental data, including varying therapeutic efficacy across movement disorders. We tested the predictions of this model in patients with Parkinson's disease, on and off subthalamic deep brain stimulation (DBS), focussing on adaptive sensory-motor responses to a changing environment and maintenance of an action until it is no longer suitable. Surprisingly, we observed prolonged perseverance under on-stimulation, and high inter-individual variability in terms of the motor selections performed when comparing the two conditions. To account for these data, we revised the standard model exploring its space of parameters and associated motor functions and found that, depending on effective connectivity between external and internal parts of the globus pallidus and saliency of the sensory input, a low dopaminergic drive can result in increased, dysfunctional, motor switching, besides motor suppression. This new framework provides insight into the biophysical mechanisms underlying DBS, allowing a description in terms of alteration of the signal-to-baseline ratio in the indirect pathway, which better account of known electrophysiological data in comparison with the standard model. PMID:27004463

  13. Disconnecting force from money: effects of basal ganglia damage on incentive motivation.

    Science.gov (United States)

    Schmidt, Liane; d'Arc, Baudouin Forgeot; Lafargue, Gilles; Galanaud, Damien; Czernecki, Virginie; Grabli, David; Schüpbach, Michael; Hartmann, Andreas; Lévy, Richard; Dubois, Bruno; Pessiglione, Mathias

    2008-05-01

    Bilateral basal ganglia lesions have been reported to induce a particular form of apathy, termed auto-activation deficit (AAD), principally defined as a loss of self-driven behaviour that is reversible with external stimulation. We hypothesized that AAD reflects a dysfunction of incentive motivation, a process that translates an expected reward (or goal) into behavioural activation. To investigate this hypothesis, we designed a behavioural paradigm contrasting an instructed (externally driven) task, in which subjects have to produce different levels of force by squeezing a hand grip, to an incentive (self-driven) task, in which subjects can win, depending on their hand grip force, different amounts of money. Skin conductance was simultaneously measured to index affective evaluation of monetary incentives. Thirteen AAD patients with bilateral striato-pallidal lesions were compared to thirteen unmedicated patients with Parkinson's; disease (PD), which is characterized by striatal dopamine depletion and regularly associated with apathy. AAD patients did not differ from PD patients in terms of grip force response to external instructions or skin conductance response to monetary incentives. However, unlike PD patients, they failed to distinguish between monetary incentives in their grip force. We conclude that bilateral striato-pallidal damage specifically disconnects motor output from affective evaluation of potential rewards. PMID:18344560

  14. Interaction between basal ganglia and limbic circuits in learning and memory processes.

    Science.gov (United States)

    Calabresi, Paolo; Picconi, Barbara; Tozzi, Alessandro; Ghiglieri, Veronica

    2016-01-01

    Hippocampus and striatum play distinctive roles in memory processes since declarative and non-declarative memory systems may act independently. However, hippocampus and striatum can also be engaged to function in parallel as part of a dynamic system to integrate previous experience and adjust behavioral responses. In these structures the formation, storage, and retrieval of memory require a synaptic mechanism that is able to integrate multiple signals and to translate them into persistent molecular traces at both the corticostriatal and hippocampal/limbic synapses. The best cellular candidate for this complex synthesis is represented by long-term potentiation (LTP). A common feature of LTP expressed in these two memory systems is the critical requirement of convergence and coincidence of glutamatergic and dopaminergic inputs to the dendritic spines of the neurons expressing this form of synaptic plasticity. In experimental models of Parkinson's disease abnormal accumulation of α-synuclein affects these two memory systems by altering two major synaptic mechanisms underlying cognitive functions in cholinergic striatal neurons, likely implicated in basal ganglia dependent operative memory, and in the CA1 hippocampal region, playing a central function in episodic/declarative memory processes.

  15. Subthalamic, not striatal, activity correlates with basal ganglia downstream activity in normal and parkinsonian monkeys

    Science.gov (United States)

    Deffains, Marc; Iskhakova, Liliya; Katabi, Shiran; Haber, Suzanne N; Israel, Zvi; Bergman, Hagai

    2016-01-01

    The striatum and the subthalamic nucleus (STN) constitute the input stage of the basal ganglia (BG) network and together innervate BG downstream structures using GABA and glutamate, respectively. Comparison of the neuronal activity in BG input and downstream structures reveals that subthalamic, not striatal, activity fluctuations correlate with modulations in the increase/decrease discharge balance of BG downstream neurons during temporal discounting classical condition task. After induction of parkinsonism with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), abnormal low beta (8-15 Hz) spiking and local field potential (LFP) oscillations resonate across the BG network. Nevertheless, LFP beta oscillations entrain spiking activity of STN, striatal cholinergic interneurons and BG downstream structures, but do not entrain spiking activity of striatal projection neurons. Our results highlight the pivotal role of STN divergent projections in BG physiology and pathophysiology and may explain why STN is such an effective site for invasive treatment of advanced Parkinson's disease and other BG-related disorders. DOI: http://dx.doi.org/10.7554/eLife.16443.001 PMID:27552049

  16. Idiopathic basal ganglia calcification-associated PDGFRB mutations impair the receptor signalling

    Science.gov (United States)

    Arts, Florence A; Velghe, Amélie I; Stevens, Monique; Renauld, Jean-Christophe; Essaghir, Ahmed; Demoulin, Jean-Baptiste

    2015-01-01

    Platelet-derived growth factors (PDGF) bind to two related receptor tyrosine kinases, which are encoded by the PDGFRA and PDGFRB genes. Recently, heterozygous PDGFRB mutations have been described in patients diagnosed with idiopathic basal ganglia calcification (IBGC or Fahr disease), a rare inherited neurological disorder. The goal of the present study was to determine whether these mutations had a positive or negative impact on the PDGFRB activity. We first showed that the E1071V mutant behaved like wild-type PDGFRB and may represent a polymorphism unrelated to IBGC. In contrast, the L658P mutant had no kinase activity and failed to activate any of the pathways normally stimulated by PDGF. The R987W mutant activated Akt and MAP kinases but did not induce the phosphorylation of signal transducer and activator of transcription 3 (STAT3) after PDGF stimulation. Phosphorylation of phospholipase Cγ was also decreased. Finally, we showed that the R987W mutant was more rapidly degraded upon PDGF binding compared to wild-type PDGFRB. In conclusion, PDGFRB mutations associated with IBGC impair the receptor signalling. PDGFRB loss of function in IBGC is consistent with recently described inactivating mutations in the PDGF-B ligand. These results raise concerns about the long-term safety of PDGF receptor inhibition by drugs such as imatinib. PMID:25292412

  17. Methyl CpG-binding protein 2 participating in the regulation of differentiation plasticity of nerve regeneration in the basal ganglia after ischemic stroke

    Directory of Open Access Journals (Sweden)

    LI Pan

    2013-11-01

    Full Text Available Background It is accepted that cerebral ischemia induces neurogenesis and neural stem cells (NSCs differentiation in non-neurogenic regions (especially in the basal ganglia. However, the mechanisms possibly involved in modulating the differentiation plasticity of NSCs are still let to known. This study aims to investigate the possible epigenetic mechanisms involved in the differentiation process of NSCs after ischemic stroke. Methods Western blotting analysis was used to detect the protein levels of methyl CpG-binding protien 2 (MeCP2 and phosphorylated MeCP2 (pMeCP2 in the ischemic basal ganglia of rat model at 3 d following middle cerebral artery occlusion (MCAO. Immunohistochemical staining was performed to observe the cellular distribution of MeCP2 and pMeCP2, the cellular colocalization of pMeCP2 with NSCs marker nestin and neuronal marker microtubule?associated protein 2 (MAP-2 in the ischemic basal ganglia of rat brains. Results 1 MeCP2 was phosphorylated in the basal ganglia after ischemic stroke, forming pMeCP2. MeCP2 positive cell number was decreased in the ischemic basal ganglia (t = 12.239, P = 0.000, while pMeCP2 positive cell number was increased in the ischemic basal ganglia (t = 5.808, P = 0.000. 2 Ischemic stroke induced a reduction of MeCP2 levels in the nucleus (t = 14.949, P = 0.000 and an elevation of pMeCP2 levels in the cytoplasm (t = 5.026, P = 0.001. 3 MeCP2 phosphorylation mediated the translocation of MeCP2 from nucleus to cytoplasm. 4 pMeCP2 was colocalized with NSCs marker protein nestin in the ischemic basal ganglia at 3 d after MCAO; pMeCP2 was colocalized with the neuronal marker MAP-2 in the ischemic basal ganglia at 1 week after MCAO. Conclusion Ischemic stroke-induced MeCP2 phosphorylation was able to alter the spatial distribution of MeCP2, transferring it from nucleus to cytoplasm and affecting its biological functions. This study further improved our awareness of brain neurogenesis in adult animals

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

    International Nuclear Information System (INIS)

    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.)

  19. A modular neural-network model of the basal ganglia's role in learning and selecting motor behaviours.

    OpenAIRE

    Baldassarre, Gianluca

    2002-01-01

    This work presents a modular neural-network model (based on reinforcement learning actor-critic methods) that tries to capture some of the most relevant known aspects of the role that basal ganglia play in learning and selecting motor behavior related to different goals. The model uses a mixture of experts network for the critic and a hierarchical network with two levels for the actor. Some simulations with the model show that basal ganglia select "chunks" of behavior whose "details" are spec...

  20. Involvement of Basal Ganglia network in motor disabilities induced by typical antipsychotics.

    Directory of Open Access Journals (Sweden)

    Jonathan Chetrit

    Full Text Available BACKGROUND: Clinical treatments with typical antipsychotic drugs (APDs are accompanied by extrapyramidal motor side-effects (EPS such as hypokinesia and catalepsy. As little is known about electrophysiological substrates of such motor disturbances, we investigated the effects of a typical APD, alpha-flupentixol, on the motor behavior and the neuronal activity of the whole basal ganglia nuclei in the rat. METHODS AND FINDINGS: The motor behavior was examined by the open field actimeter and the neuronal activity of basal ganglia nuclei was investigated using extracellular single unit recordings on urethane anesthetized rats. We show that alpha-flupentixol induced EPS paralleled by a decrease in the firing rate and a disorganization of the firing pattern in both substantia nigra pars reticulata (SNr and subthalamic nucleus (STN. Furthermore, alpha-flupentixol induced an increase in the firing rate of globus pallidus (GP neurons. In the striatum, we recorded two populations of medium spiny neurons (MSNs after their antidromic identification. At basal level, both striato-pallidal and striato-nigral MSNs were found to be unaffected by alpha-flupentixol. However, during electrical cortico-striatal activation only striato-pallidal, but not striato-nigral, MSNs were found to be inhibited by alpha-flupentixol. Together, our results suggest that the changes in STN and SNr neuronal activity are a consequence of increased neuronal activity of globus pallidus (GP. Indeed, after selective GP lesion, alpha-flupentixol failed to induce EPS and to alter STN neuronal activity. CONCLUSION: Our study reports strong evidence to show that hypokinesia and catalepsy induced by alpha-flupentixol are triggered by dramatic changes occurring in basal ganglia network. We provide new insight into the key role of GP in the pathophysiology of APD-induced EPS suggesting that the GP can be considered as a potential target for the treatment of EPS.

  1. Competing basal ganglia pathways determine the difference between stopping and deciding not to go.

    Science.gov (United States)

    Dunovan, Kyle; Lynch, Brighid; Molesworth, Tara; Verstynen, Timothy

    2015-01-01

    The architecture of corticobasal ganglia pathways allows for many routes to inhibit a planned action: the hyperdirect pathway performs fast action cancellation and the indirect pathway competitively constrains execution signals from the direct pathway. We present a novel model, principled off of basal ganglia circuitry, that differentiates control dynamics of reactive stopping from intrinsic no-go decisions. Using a nested diffusion model, we show how reactive braking depends on the state of an execution process. In contrast, no-go decisions are best captured by a failure of the execution process to reach the decision threshold due to increasing constraints on the drift rate. This model accounts for both behavioral and functional MRI (fMRI) responses during inhibitory control tasks better than alternative models. The advantage of this framework is that it allows for incorporating the effects of context in reactive and proactive control into a single unifying parameter, while distinguishing action cancellation from no-go decisions. PMID:26402462

  2. Models of basal ganglia and cerebellum for sensorimotor integration and predictive control

    Science.gov (United States)

    Jabri, Marwan A.; Huang, Jerry; Coenen, Olivier J. D.; Sejnowski, Terrence J.

    2000-10-01

    This paper presents a sensorimotor architecture integrating computational models of a cerebellum and a basal ganglia and operating on a microrobot. The computational models enable a microrobot to learn to track a moving object and anticipate future positions using a CCD camera. The architecture features pre-processing modules for coordinate transformation and instantaneous orientation extraction. Learning of motor control is implemented using predictive Hebbian reinforcement-learning algorithm in the basal ganglia model. Learning of sensory predictions makes use of a combination of long-term depression (LTD) and long-term potentiation (LTP) adaptation rules within the cerebellum model. The basal ganglia model uses the visual inputs to develop sensorimotor mapping for motor control, while the cerebellum module uses robot orientation and world- coordinate transformed inputs to predict the location of the moving object in a robot centered coordinate system. We propose several hypotheses about the functional role of cell populations in the cerebellum and argue that mossy fiber projections to the deep cerebellar nucleus (DCN) could play a coordinate transformation role and act as gain fields. We propose that such transformation could be learnt early in the brain development stages and could be guided by the activity of the climbing fibers. Proprioceptor mossy fibers projecting to the DCN and providing robot orientation with respect to a reference system could be involved in this case. Other mossy fibers carrying visual sensory input provide visual patterns to the granule cells. The combined activities of the granule and the Purkinje cells store spatial representations of the target patterns. The combinations of mossy and Purkinje projections to the DCN provide a prediction of the location of the moving target taking into consideration the robot orientation. Results of lesion simulations based on our model show degradations similar to those reported in cerebellar lesion

  3. Late-Onset Mania in a Patient with Movement Disorder and Basal Ganglia Calcifications: A Challenge for Diagnosis and Treatment

    Directory of Open Access Journals (Sweden)

    Beatrice Roiter

    2016-01-01

    Full Text Available Age of onset can have a significant impact on clinical course and pathophysiological mechanism of bipolar disorder. Late-onset bipolar episodes are more likely linked to medical illnesses and so are frequently classified as “secondary” forms of mood disorder. We discuss the case of a patient who at the age of 58 presented his first delusional-manic episode. He also had mild frontal and occipital cortical atrophy, white matter posterior ischemic lesions, and small basal ganglia calcifications. Seven years later, he presented a second manic episode with new emergent hyperkinetic choreiform symptoms. Taking into account movement disturbances, the presence of basal ganglia calcification, and worsening of cortical atrophy, we performed a differential diagnosis between Fahr disease, Fahr’s syndrome, calcifications due to ageing, supersensitivity psychosis, and dementia. Valproate, quetiapine, and tetrabenazine were sequentially administered and yielded a good therapeutic response as regards manic and movement symptoms. Relationship between medications and course of specific symptoms was observed.

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

    International Nuclear Information System (INIS)

    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

  5. 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.

  6. A spiking Basal Ganglia model of synchrony, exploration and decision making

    Directory of Open Access Journals (Sweden)

    Alekhya eMandali

    2015-05-01

    Full Text Available To make an optimal decision we need to weigh all the available options, compare them with the current goal, and choose the most rewarding one. Depending on the situation an optimal decision could be to either ‘explore’ or ‘exploit’ or ‘not to take any action’ for which the Basal Ganglia (BG is considered to be a key neural substrate. In an attempt to expand this classical picture of BG function, we had earlier hypothesized that the Indirect Pathway (IP of the BG could be the subcortical substrate for exploration. In this study we build a spiking network model to relate exploration to synchrony levels in the BG (which are a neural marker for tremor in Parkinson’s disease. Key BG nuclei such as the Sub Thalamic Nucleus (STN, Globus Pallidus externus (GPe and Globus Pallidus internus (GPi were modeled as Izhikevich spiking neurons whereas the Striatal output was modeled as Poisson spikes. The model is cast in reinforcement learning framework with the dopamine signal representing reward prediction error. We apply the model to two decision making tasks: a binary action selection task (similar to one used by Humphries et al. 2006 and an n-armed bandit task (Bourdaud et al. 2008. The model shows that exploration levels could be controlled by STN’s lateral connection strength which also influenced the synchrony levels in the STN-GPe circuit. An increase in STN’s lateral strength led to a decrease in exploration which can be thought as the possible explanation for reduced exploratory levels in Parkinson’s patients. Our simulations also show that on complete removal of IP, the model exhibits only Go and No-Go behaviors, thereby demonstrating the crucial role of IP in exploration. Our model provides a unified account for synchronization, action section, and explorative behavior.

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

    Science.gov (United States)

    Berthet, Pierre; Lindahl, Mikael; Tully, Philip J; Hellgren-Kotaleski, Jeanette; Lansner, Anders

    2016-01-01

    The brain enables animals to behaviorally adapt in order to survive in a complex and dynamic environment, but how reward-oriented behaviors 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 post-synaptic 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. PMID:27493625

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

    International Nuclear Information System (INIS)

    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

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

    International Nuclear Information System (INIS)

    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 ([123I]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 [123I]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.)

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

    Energy Technology Data Exchange (ETDEWEB)

    Ryu, W. K.; Ryu, Y. H.; Yoon, M. J.; Kim, C. H.; Chun, K. A.; Lee, J. D. [College of Medicine, Univ. of Yonsei, Seoul (Korea, Republic of); Jee, D. Y. [College of Medicine, Univ. of Inhwa, Seoul (Korea, Republic of); Choi, T. H. [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

    2003-07-01

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2003-12-01

    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)-2{beta}-carbomethoxy-3{beta}-(4-chlorophenyl) tropane ([{sup 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 [{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 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.)

  12. 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.

  13. 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.)

  14. Learning processing in the basal ganglia: a mosaic of broken mirrors.

    Science.gov (United States)

    Da Cunha, Claudio; Wietzikoski, Evellyn Claudia; Dombrowski, Patrícia; Bortolanza, Mariza; Santos, Lucélia Mendes; Boschen, Suelen Lucio; Miyoshi, Edmar

    2009-04-12

    In the present review we propose a model to explain the role of the basal ganglia in sensorimotor and cognitive functions based on a growing body of behavioural, anatomical, physiological, and neurochemical evidence accumulated over the last decades. This model proposes that the body and its surrounding environment are represented in the striatum in a fragmented and repeated way, like a mosaic consisting of the fragmented images of broken mirrors. Each fragment forms a functional unit representing articulated parts of the body with motion properties, objects of the environment which the subject can approach or manipulate, and locations the subject can move to. These units integrate the sensory properties and movements related to them. The repeated and widespread distribution of such units amplifies the combinatorial power of the associations among them. These associations depend on the phasic release of dopamine in the striatum triggered by the saliency of stimuli and will be reinforced by the rewarding consequences of the actions related to them. Dopamine permits synaptic plasticity in the corticostriatal synapses. The striatal units encoding the same stimulus/action send convergent projections to the internal segment of the globus pallidus (GPi) and to the substantia nigra pars reticulata (SNr) that stimulate or hold the action through a thalamus-frontal cortex pathway. According to this model, this is how the basal ganglia select actions based on environmental stimuli and store adaptive associations as nondeclarative memories such as motor skills, habits, and memories formed by Pavlovian and instrumental conditioning. PMID:18977393

  15. A case of vitamin B12 deficiency with involuntary movements and bilateral basal ganglia lesions.

    Science.gov (United States)

    Kitamura, Taisuke; Gotoh, Seiji; Takaki, Hayato; Kiyuna, Fumi; Yoshimura, Sohei; Fujii, Kenichiro

    2016-07-28

    An 86-year-old woman with a one-year history of dementia was admitted to our hospital complaining of loss of appetite, hallucinations, and disturbance of consciousness. She gradually presented with chorea-like involuntary movements of the extremities. Diffusion-weighted magnetic resonance imaging (MRI) showed bilateral symmetrical hyperintense signals in the basal ganglia. The serum vitamin B12 level was below the lower detection limit of 50 pg/ml. The homocysteine level was markedly elevated at 115.8 nmol/ml. Anti-intrinsic factor and anti-parietal cell antibody tests were positive. Gastrointestinal endoscopy revealed atrophic gastritis. The patient was diagnosed with encephalopathy due to vitamin B12 deficiency caused by pernicious anemia. Involuntary movements and MRI abnormalities improved with parenteral vitamin B12 supplementation. Bilateral basal ganglia lesions are rare manifestations of adult vitamin B12 deficiency. The present case is considered valuable in identifying the pathophysiology of involuntary movement due to vitamin B12 deficiency. PMID:27356735

  16. Cardiorespiratory fitness and its association with thalamic, hippocampal, and basal ganglia volumes in multiple sclerosis

    Science.gov (United States)

    Motl, Robert W.; Pilutti, Lara A.; Hubbard, Elizabeth A.; Wetter, Nathan C.; Sosnoff, Jacob J.; Sutton, Bradley P.

    2015-01-01

    Background There is little known about cardiorespiratory fitness and its association with volumes of the thalamus, hippocampus, and basal ganglia in multiple sclerosis (MS). Such inquiry is important for identifying a possible behavioral approach (e.g., aerobic exercise training) that might change volumes of deep gray matter (DGM) structures associated with cognitive and motor functions in MS. Purpose This study examined the association between cardiorespiratory fitness and volumes of the thalamus, hippocampus, and basal ganglia in MS. Method We enrolled 35 persons with MS who underwent a maximal exercise test for measuring cardiorespiratory fitness as peak oxygen consumption (VO2peak) and brain MRI. Volumes of the thalamus, hippocampus, caudate, putamen, and pallidum were calculated from 3D T1-weighted structural brain images. We examined associations using partial (pr) correlations controlling for demographic and clinical variables. Results VO2peak was significantly associated with composite scaled volumes of the caudate(pr = .47, p < .01), putamen (pr = .44, p < .05), pallidum (pr = .40, p < .05), and hippocampus (pr = .42, p < .05), but not thalamus (pr = .31, p = .09), when controlling for sex, age, disability, and duration of MS. Conclusion Our results provide novel evidence that cardiorespiratory fitness is associated with volumes of DGM structures that are involved in motor and cognitive functions in MS. PMID:25844320

  17. Trigeminal-basal ganglia interaction: control of sensory-motor gating and positive reinforcement.

    Science.gov (United States)

    Schwarting, R K; Elstermeier, F; Francke, W; Huston, J P

    1991-02-01

    Functional interactions between the basal ganglia and the perioral area were analyzed by means of electrical brain stimulation in the rat. The first experiment showed that unilateral stimulation of the substantia nigra sensitized the contralateral perioral area for a biting reflex upon its tactile stimulation. This biting reflex consists of lip withdrawal, orienting towards and biting into the stimulus source. The same sites in the substantia nigra also produced electrical self-stimulation using bar-pressing as the operant. A positive correlation was found between threshold currents for biting and for self-stimulation. However, the current levels necessary for reinforcement were considerably higher than those to facilitate the biting reflex. In the second experiment, it was found that manipulation of the perioral area by unilateral vibrissae removal reduced the rate of electrical self-stimulation in the substantia nigra. This effect was lateralized, depended on time after vibrissae removal, and could be reversed by systemic injections of the dopamine receptor agonist apomorphine. These results, which provide evidence for a reciprocal interaction between the basal ganglia and the perioral area, are discussed with respect to mechanisms of sensory-motor gating, motivation and reinforcement.

  18. Genetic screening and functional characterization of PDGFRB mutations associated with Basal Ganglia Calcification of Unknown Etiology

    Science.gov (United States)

    Sanchez-Contreras, Monica; Baker, Matthew C.; Finch, NiCole A.; Nicholson, Alexandra; Wojtas, Aleksandra; Wszolek, Zbigniew K.; Ross, Owen A.; Dickson, Dennis W.; Rademakers, Rosa

    2014-01-01

    Three causal genes for Idiopathic Basal Ganglia Calcification (IBGC) have been identified. Most recently, mutations in PDGFRB, encoding a member of the platelet-derived growth factor receptor family type β, and PDGFB, encoding PDGF-B, the specific ligand of PDGFRβ, were found implicating the PDGF-B/PDGFRβ pathway in abnormal brain calcification. In this study we aimed to identify and study mutations in PDGFRB and PDGFB in a series of 26 patients from the Mayo Clinic Florida Brain Bank with moderate to severe basal ganglia calcification (BCG) of unknown etiology. No mutations in PDGFB were found. However, we identified one mutation in PDGFRB, p.R695C located in the tyrosine kinase domain, in one BGC patient. We further studied the function of p.R695C mutant PDGFRβ and two previously reported mutants, p.L658P and p.R987W PDGFRβ in cell culture. We show that, in response to PDGF-BB stimulation, the p.L658P mutation completely suppresses PDGFRβ autophosphorylation whereas the p.R695C mutation results in partial loss of autophosphorylation. For the p.R987W mutation, our data suggest a different mechanism involving reduced protein levels. These genetic and functional studies provide the first insight into the pathogenic mechanisms associated with PDGFRB mutations and provide further support for a pathogenic role of PDGFRB mutations in BGC. PMID:24796542

  19. Longitudinal Assessment of Motor Recovery of Contralateral Hand after Basal Ganglia Infarction Using Functional Magnetic Resonance Imaging

    Directory of Open Access Journals (Sweden)

    Yue Fu

    2016-01-01

    Full Text Available We used functional fMRI to study the brain activation during active finger movements at different time points during the recovery phase following basal ganglia infarction. Four hemiplegic patients with basal ganglia infarction were serially evaluated at different time points spanning the acute and chronic phase using fMRI. To evaluate motor recovery, the patients were asked to perform functional tasks arranged in a block design manner with their hand. On follow-up (chronic phase, three patients achieved significant recovery of motor function of affected limbs. Activation of bilateral sensorimotor cortex (SMC was observed in two of these patients, while activation of cerebellum was observed in all patients. No remarkable recovery of motor function was noted in one patient with left basal ganglia infarction. In this patient, the activation domain was located in SMC of both sides in acute phase and in ipsilateral SMC in chronic phase. Contralateral SMC appears to be involved in the functional rehabilitation following basal ganglia infarction. The cerebellum may act as an intermediary during functional recovery following basal ganglia infarction. The activation domain associated with active finger movement may be bilateral in acute phase; one patient was ipsilateral in the chronic stage.

  20. Involvement of dorsal root ganglia in Fabry's disease.

    OpenAIRE

    Gadoth, N; Sandbank, U.

    1983-01-01

    Bouts of shooting pain along the extremities are common in the early stages of Fabry's disease. No pathological explanation has been advanced to clarify the mechanism of such pain. In the present case neuronal storage of glycolipid was confined to dorsal root ganglia neurones only. It is suggested that this may explain the shooting pain in Fabry's disease. In hereditary sensory radicular neuropathy, familial dysautonomia, and tabes dorsalis, changes in dorsal root ganglia cells cause similar ...

  1. DISTRIBUTION OF PARVALBUMIN,CALBINDIN-D28 AND CALRETININ IMMUNOREACTIVE NEURONS AND FIBERS IN THE MONKEY BASAL GANGLIA

    Institute of Scientific and Technical Information of China (English)

    2002-01-01

    Objective To investigate the cellular localization of parvalbumin(PV),calbindin-D28K(CB)and clretinin(CR)in the monkey basal ganglia.Methods Immunocytochemical technique was used to detect PV,CB and CR immunoreactivity in the basal ganglia.Results In the striatum,CB labeled medium-sized spiny projection neuronsshereas PV and CR marked two separate classes of aspiny interneurons,The striatal matrix compartment was markedly enriched with CB while striatal patches displayed a CR-ich neuropil,In the pallidum,virtually all neurons contained PV but none express CB,CR occured only in a small subpopulation of large and small pallidal neurons.In the subthalamic nucleus,there existed a multitude of PV-positive cells and fibers but the number of CR and CB-postive neuronal elements was small,In the substantia nigra/ventral tegmental area complex.CB and CR occured principally in dopaminergic neurons of the dorsal tier of the pars compacta and in those of the ventral tegmental area.PV was strickly confined to the GABAergic neurons of the pars reticular and lateralis.CB-rich fibers abounded in the pars reticular and lateralis,while CR-positive axons were confined to the pars compacta.Conclusion:CB and PV were distributed according to a strikingly complementary pattern in primate basal ganglia,and the use of CB and PV immunocytochemistry may be considered as an excellent tool to define distinct chemoarchitectonic and functional domains within the complex organization of the basal ganglia ,CR was less ubiquitous but occured in small basal ganglia components where it labeled distinct subsets of neurons.Such highly specific patterns of distribution indicate that CB,PV and CR may work in synery within primate basal ganglia.

  2. [Gait disturbances related to dysfunction of the cerebral cortex and basal ganglia].

    Science.gov (United States)

    Takezawa, Nobuo; Mizuno, Toshiki; Seo, Kazuya; Kondo, Masaki; Nakagawa, Masanori

    2010-11-01

    This review aimed to characterize the gait disturbances in Parkinson disease (PD) and highlight how a rehabilitation program would affect the care of patients with PD. The typical PD gait is a type of hypokinetic gait characterized by reduced stride length and velocity; shortening of the swing phase; and increase in the stance phase, double-limb support duration, and cadence rate. In the advanced phase of PD, start hesitation, shuffling and festinating gait, propulsion, and freezing of gait (FOG) become remarkable. Notably, in PD, attention may influence gait control, and sensory cueing may improve the stride length. Our study on gait impairment in PD by using a three-dimensional motion analysis system revealed that the stride length and walking speed decreased, but there was no change in cadence. The decreased stride length was due to reduction in the range of movement at the leg and pelvic joints. A 4-week physical rehabilitation program for PD improved the stride length and walking speed;this was achieved by increasing the range of movement of at the leg and pelvic joints. We also assessed the effects of a rehabilitation program for patients with PD who experienced FOG. Although the lower limb function was more impaired in patients with PD and FOG than in those with PD without FOG, the rehabilitation program was effective even for patients with PD and FOG. FOG might be associated with functional impairment of the lower limb as well as dysfunction of the fronto-basal ganglia circuit. We also reported 3 cases of camptocormia (bent spine syndrome) with autonomic dysfunction and rapid eye movement (REM) sleep behavior disorders (RBD) and compared their symptoms with those reported elsewhere. We think that the pedunculopontine nuclear area may control the postural muscle tone and locomotion in PD. On the basis of the results of our rehabilitation programs, we speculate that physical modalities may modify synaptic plasticity by utilizing the cerebellar and/or afferent

  3. Basal ganglia circuit loops, dopamine and motivation: A review and enquiry.

    Science.gov (United States)

    Ikemoto, Satoshi; Yang, Chen; Tan, Aaron

    2015-09-01

    Dopamine neurons located in the midbrain play a role in motivation that regulates approach behavior (approach motivation). In addition, activation and inactivation of dopamine neurons regulate mood and induce reward and aversion, respectively. Accumulating evidence suggests that such motivational role of dopamine neurons is not limited to those located in the ventral tegmental area, but also in the substantia nigra. The present paper reviews previous rodent work concerning dopamine's role in approach motivation and the connectivity of dopamine neurons, and proposes two working models: One concerns the relationship between extracellular dopamine concentration and approach motivation. High, moderate and low concentrations of extracellular dopamine induce euphoric, seeking and aversive states, respectively. The other concerns circuit loops involving the cerebral cortex, basal ganglia, thalamus, epithalamus, and midbrain through which dopaminergic activity alters approach motivation. These models should help to generate hypothesis-driven research and provide insights for understanding altered states associated with drugs of abuse and affective disorders. PMID:25907747

  4. 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

  5. Mechanism of Parkinsonian Neuronal Oscillations in the Primate Basal Ganglia: Some Considerations Based on Our Recent Work

    Directory of Open Access Journals (Sweden)

    Atsushi eNambu

    2014-05-01

    Full Text Available Accumulating evidence suggests that abnormal neuronal oscillations in the basal ganglia contribute to the manifestation of parkinsonian symptoms. In this article, we would like to summarize our recent work on the mechanism underlying abnormal oscillations in the parkinsonian state and discuss its significance in pathophysiology of Parkinson’s disease. We recorded neuronal activity in the basal ganglia of parkinsonian monkeys treated with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Systemic administration of L-DOPA alleviated parkinsonian motor signs and decreased abnormal neuronal oscillations (8-15 Hz in the internal (GPi and external (GPe segments of the globus pallidus and the subthalamic nucleus (STN. Inactivation of the STN by muscimol (GABAA receptor agonist injection also ameliorated parkinsonian signs and suppressed GPi oscillations. Blockade of glutamatergic inputs to the STN by local microinjection of a mixture of 3-(2-carboxypiperazin-4-yl-propyl-1-phosphonic acid (glutamatergic NMDA receptor antagonist and 1,2,3,4-tetrahydro-6-nitro-2,3- dioxo-benzo[f]quinoxaline-7-sulfonamide (glutamatergic AMPA/kainate receptor antagonist suppressed neuronal oscillations in the STN. The STN oscillations were further attenuated by the blockade of GABAergic neurotransmission from the GPe to the STN by muscimol inactivation of the GPe. These results suggest that cortical glutamatergic inputs to the STN and reciprocal GPe-STN interconnections are both important for the generation and amplification of the oscillatory activity of GPe and STN neurons in the dopamine-depleted state. The oscillatory activity in the STN is subsequently transmitted to the GPi and may contribute to manifestation of parkinsonian symptoms.

  6. DISTRIBUTION OF PARVALBUMIN, CALBINDIN-D28 AND CALRETININ IMMUNOREACTIVE NEURO NS AND FIBERS IN THE MONKEY BASAL GANGLIA

    Institute of Scientific and Technical Information of China (English)

    刘健; 张巧俊

    2002-01-01

    Objective To investigate the cellular localization of parvalbumin (PV), calbindin-D28k (CB) and calretinin (CR) in the monkey basal ganglia.Methods Immunocytochemica l technique was used to detect PV,CB and CR immunoreactivity in the basal gangl ia. Results In the striatum, CB labeled medium-sized spin y projection neurons whereas PV and CR marked two separate classes of aspiny int erneurons. The striatal matrix compartment was markedly enriched with CB while s triatal patches displayed a CR-rich neuropil. In the pallidum, virtually all ne u rons contained PV but none express CB. CR occured only in a small subpopulation of large and small pallidal neurons. In the subthalamic nucleus, there existed a multitude of PV-positive cells and fibers but the number of CR and CB-positiv e neuronal elements was small. In the substantia nigra / ventral tegmental area co mplex, CB and CR occured principally in dopaminergic neurons of the dorsal tier of the pars compacta and in those of the ventral tegmental area. PV was strickly confined to the GABAergic neurons of the pars reticular and lateralis. CB-rich fibers abounded in the pars reticular and lateralis, while CR-positive axons we re confined to the pars compacta. Conclusion CB and PV were di stributed accordin g to a strikingly complementary pattern in primate basal ganglia, and the use of CB and PV immunocytochemistry may be considered as an excellent tool to define dist inct chemoarchitectonic and functional domains within the complex organization o f the basal ganglia. CR was less ubiquitous but occured in small basal ganglia c omponents where it labeled distinct subsets of neurons. Such highly specific pat terns of distribution indicate that CB, PV and CR may work in synery within prim ate basal ganglia.

  7. Singing-Related Neural Activity Distinguishes Four Classes of Putative Striatal Neurons in the Songbird Basal Ganglia

    OpenAIRE

    Goldberg, Jesse H.; Fee, Michale S

    2010-01-01

    The striatum—the primary input nucleus of the basal ganglia—plays a major role in motor control and learning. Four main classes of striatal neuron are thought to be essential for normal striatal function: medium spiny neurons, fast-spiking interneurons, cholinergic tonically active neurons, and low-threshold spiking interneurons. However, the nature of the interaction of these neurons during behavior is poorly understood. The songbird area X is a specialized striato-pallidal basal ganglia nuc...

  8. Basal ganglia volumes in drug-naive first-episode schizophrenia patients before and after short-term treatment with either a typical or an atypical antipsychotic drug

    DEFF Research Database (Denmark)

    Glenthoj, Andreas; Glenthøj, Birte Yding; Mackeprang, Torben;

    2007-01-01

    The present study examined basal ganglia volumes in drug-naive first-episode schizophrenic patients before and after treatment with either a specific typical or atypical antipsychotic compound. Sixteen antipsychotic drug-naive and three minimally medicated first-episode schizophrenic patients and...... in caudate volume in patients suggests intrinsic basal ganglia pathology in schizophrenia, most likely of neurodevelopmental origin....

  9. 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

    2012-08-01

    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 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. PMID:22875921

  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

    2012-12-01

    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. 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.

  12. The Basal Ganglia Select the Expected Sensory Input Used for Predictive Coding

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    Brian eColder

    2015-09-01

    Full Text Available While considerable evidence supports the notion that lower-level interpretation of incoming sensory information is guided by top-down sensory expectations, less is known about the source of the sensory expectations or the mechanisms by which they are spread. Predictive coding theory proposes that sensory expectations flow down from higher-level association areas to lower-level sensory cortex, and deviations from those expectations (error signals flow back up to association areas. A separate theory of the role of prediction in cognition describes emulations as linked representations of potential actions and their associated expected sensation that are hypothesized to play an important role in many aspects of cognition. The expected sensations in active emulations are proposed to be the top-down expectation used in predictive coding. Representations of the potential action and expected sensation in emulations are thought to be instantiated in distributed cortical networks. Combining predictive coding with emulations thus provides a theoretical link between the top-down expectations that guide sensory expectations and the cortical networks representing potential actions. Now moving to theories of action selection, the basal ganglia has long been proposed to select between potential actions by reducing inhibition to the cortical network instantiating the desired action plan. Integration of these isolated theories

  13. Model-based action planning involves cortico-cerebellar and basal ganglia networks

    Science.gov (United States)

    Fermin, Alan S. R.; Yoshida, Takehiko; Yoshimoto, Junichiro; Ito, Makoto; Tanaka, Saori C.; Doya, Kenji

    2016-01-01

    Humans can select actions by learning, planning, or retrieving motor memories. Reinforcement Learning (RL) associates these processes with three major classes of strategies for action selection: exploratory RL learns state-action values by exploration, model-based RL uses internal models to simulate future states reached by hypothetical actions, and motor-memory RL selects past successful state-action mapping. In order to investigate the neural substrates that implement these strategies, we conducted a functional magnetic resonance imaging (fMRI) experiment while humans performed a sequential action selection task under conditions that promoted the use of a specific RL strategy. The ventromedial prefrontal cortex and ventral striatum increased activity in the exploratory condition; the dorsolateral prefrontal cortex, dorsomedial striatum, and lateral cerebellum in the model-based condition; and the supplementary motor area, putamen, and anterior cerebellum in the motor-memory condition. These findings suggest that a distinct prefrontal-basal ganglia and cerebellar network implements the model-based RL action selection strategy. PMID:27539554

  14. Making working memory work: a computational model of learning in the prefrontal cortex and basal ganglia.

    Science.gov (United States)

    O'Reilly, Randall C; Frank, Michael J

    2006-02-01

    The prefrontal cortex has long been thought to subserve both working memory (the holding of information online for processing) and executive functions (deciding how to manipulate working memory and perform processing). Although many computational models of working memory have been developed, the mechanistic basis of executive function remains elusive, often amounting to a homunculus. This article presents an attempt to deconstruct this homunculus through powerful learning mechanisms that allow a computational model of the prefrontal cortex to control both itself and other brain areas in a strategic, task-appropriate manner. These learning mechanisms are based on subcortical structures in the midbrain, basal ganglia, and amygdala, which together form an actor-critic architecture. The critic system learns which prefrontal representations are task relevant and trains the actor, which in turn provides a dynamic gating mechanism for controlling working memory updating. Computationally, the learning mechanism is designed to simultaneously solve the temporal and structural credit assignment problems. The model's performance compares favorably with standard backpropagation-based temporal learning mechanisms on the challenging 1-2-AX working memory task and other benchmark working memory tasks. PMID:16378516

  15. Role of beta-arrestin 2 downstream of dopamine receptors in the basal ganglia

    Directory of Open Access Journals (Sweden)

    Thomas eDel'Guidice

    2011-09-01

    Full Text Available Multifunctional scaffolding protein beta-arrestins (βArr and the G protein receptor kinases (GRK are involved in the desensitization of several G protein coupled-receptors (GPCR. However, arrestins can also contribute to GPCR signaling independently from G proteins. In this review, we focus on the role of βArr in the regulation of dopamine receptor functions in the striatum. First, we present in vivo evidence supporting a role for these proteins in the regulation of dopamine receptor desensitization. Second, we provide an overview of the roles of βArr-2 in the regulation of ERK/MAPkinases and Akt/GSK3 signaling pathways downstream of the D1 and D2 dopamine receptors. Thereafter, we examine the possible involvement of βArr-mediated signaling in the action of dopaminergic drugs used for the treatment of mental disorders. Finally, we focus on different potential cellular proteins regulated by βArr-mediated signaling which could contribute to the regulation of behavioral responses to dopamine. Overall, the identification of a cell signaling function for βArr downstream of dopamine receptors underscores the intricate complexity of the intertwined mechanisms regulating and mediating cell signaling in the basal ganglia. Understanding these mechanisms may lead to a better comprehension of the several roles played by these structures in the regulation of mood and to the development of new psychoactive drugs having better therapeutic efficacy.

  16. 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.

  17. Model-based action planning involves cortico-cerebellar and basal ganglia networks.

    Science.gov (United States)

    Fermin, Alan S R; Yoshida, Takehiko; Yoshimoto, Junichiro; Ito, Makoto; Tanaka, Saori C; Doya, Kenji

    2016-01-01

    Humans can select actions by learning, planning, or retrieving motor memories. Reinforcement Learning (RL) associates these processes with three major classes of strategies for action selection: exploratory RL learns state-action values by exploration, model-based RL uses internal models to simulate future states reached by hypothetical actions, and motor-memory RL selects past successful state-action mapping. In order to investigate the neural substrates that implement these strategies, we conducted a functional magnetic resonance imaging (fMRI) experiment while humans performed a sequential action selection task under conditions that promoted the use of a specific RL strategy. The ventromedial prefrontal cortex and ventral striatum increased activity in the exploratory condition; the dorsolateral prefrontal cortex, dorsomedial striatum, and lateral cerebellum in the model-based condition; and the supplementary motor area, putamen, and anterior cerebellum in the motor-memory condition. These findings suggest that a distinct prefrontal-basal ganglia and cerebellar network implements the model-based RL action selection strategy. PMID:27539554

  18. Functional lateralization in cingulate cortex predicts motor recovery after basal ganglia stroke.

    Science.gov (United States)

    Li, Yao; Chen, Zengai; Su, Xin; Zhang, Xiaoliu; Wang, Ping; Zhu, Yajing; Xu, Qun; Xu, Jianrong; Tong, Shanbao

    2016-02-01

    The basal ganglia (BG) is involved in higher order motor control such as movement planning and execution of complex motor synergies. Neuroimaging study on stroke patients specifically with BG lesions would help to clarify the consequence of BG damage on motor control. In this paper, we performed a longitudinal study in the stroke patients with lesions in BG regions across three motor recovery stages, i.e., less than 2week (Session 1), 1-3m (Session 2) and more than 3m (Session 3). The patients showed an activation shift from bilateral hemispheres during early sessions (3m), suggesting a compensation effect from the contralesional hemisphere during motor recovery. We found that the lateralization of cerebellum(CB) for affected hand task correlated with patients' concurrent Fugl-Meyer index (FMI) in Session 2. Moreover, the cingulate cortex lateralization index in Session 2 was shown to significantly correlate with subsequent FMI change between Session 3 and Session 2, which serves as a prognostic marker for motor recovery. Our findings consolidated the close interactions between BG and CB during the motor recovery after stroke. The dominance of activation in contralateral cingulate cortex was associated with a better motor recovery, suggesting the important role of ipsilesional attention modulation in the early stage after BG stroke. PMID:26742641

  19. 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.

  20. 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.

  1. Consequences of nigrostriatal denervation on the functioning of the basal ganglia in human and nonhuman primates: an in situ hybridization study of cytochrome oxidase subunit I mRNA.

    Science.gov (United States)

    Vila, M; Levy, R; Herrero, M T; Ruberg, M; Faucheux, B; Obeso, J A; Agid, Y; Hirsch, E C

    1997-01-15

    To examine the consequences of nigrostriatal denervation and chronic levodopa (L-DOPA) treatment on functional activity of the basal ganglia, we analyzed, using in situ hybridization, the cellular expression of the mRNA encoding for cytochrome oxidase subunit I (COI mRNA), a molecular marker for functional neuronal activity, in the basal ganglia. This analysis was performed in monkeys rendered parkinsonian by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) Intoxication, some of which had been receiving L-DOPA, and in patients with Parkinson's disease (PD). In MPTP-intoxicated monkeys compared with control animals, COI mRNA expression was increased in the subthalamic nucleus (STN) and in the output nuclei of the basal ganglia, i.e., the internal segment of the globus pallidus and the substantia nigra pars reticulata. This increase was partially reversed by L-DOPA treatment. COI mRNA expression remained unchanged in the external segment of the globus pallidus (GPe). In PD patients, all of whom had been treated chronically by L-DOPA, COI mRNA expression in the analyzed basal ganglia structures was similar to that in control subjects. These results are in agreement with the accepted model of basal ganglia organization, to the extent that the output nuclei of the basal ganglia are considered to be overactive after nigrostriatal denervation, partly because of increased activity of excitatory afferents from the STN. Yet, our results would also seem to contradict this model, because the overactivity of the STN does not seem to be attributable to a hypoactivation of the GPe.

  2. Basal ganglia stroke due to mild head trauma in pediatric age - clinical and therapeutic management: a case report and 10 year literature review

    Directory of Open Access Journals (Sweden)

    Salvati Maurizio

    2011-01-01

    Full Text Available Abstract Ischemia of the basal ganglia as an immediate consequence of minor head injury in children is rare ( Young patients should be closely monitored and treated conservatively with osmotic diuretics to reduce perilesional edema. At the same time, however, it is very important to exclude, by means of instrumental and laboratory studies, conditions that could favour the onset of ischemia, including emboligen heart disease, thrombophilia and acute traumatic arterial dissections. Generally speaking, the prognosis in these cases is good. The authors describe their experience treating a 10-month old baby girl, with a left lenticular nucleus ischemia and report a literature review.

  3. Tailored keyhole surgery for basal ganglia cavernous malformation with preoperative three-dimensional pyramidal tracts assessment and intraoperative electrophysiological monitoring

    Institute of Scientific and Technical Information of China (English)

    Kai Quan; Geng Xu; Fan Zhao; Wei Zhu

    2016-01-01

    Background:Accurately mapping the pyramidal tracts preoperatively and intraoperatively is the primary concern when operating on cavernous malformations (CMS) in the basal ganglia.We have conducted new methods for preoperative planning and have tailored lesion resection to prevent the damage of pyramidal tracts.Patients and methods:Eleven patients harboring cavernous malformations in basal ganglia were treated surgically from April 2008 to January 2015.Surgical planning was based on three-dimensional diffusion tensor pyramidal tractography and Virtual Reality system.Intraoperative detecting of pyramidal tracts with subcortical stimulation mapping and motor evoked potential monitoring were performed.The extent of resection and postoperative neurological function were assessed in each case.Results:Total removal of the cavernous malformations were achieved in each case.Four of the total eleven cases presented temporary neurological deficits,including one occurrence of hemiparesis and three occurrences of hemianesthesia.No permanent neurological deficit was developed in this series of cases.Conclusion:Three-dimensional diffusion tensor pyramidal tractography is quite helpful for preoperative planning of basal ganglia cavernous malformations,especially in choosing a suitable surgical approach.Intraoperative detection of pyramidal tracts with subcortical stimulation mapping and motor evoked potential monitoring play important roles in preventing damage to pyramidal tracts during lesion resection.

  4. A movable microelectrode array for chronic basal ganglia single-unit electrocorticogram co-recording in freely behaving rats.

    Science.gov (United States)

    Zheng, Xiaobin; Zeng, Jia; Chen, Ting; Lin, Yuanxiang; Yu, Lianghong; Li, Ying; Lin, Zhangya; Wu, Xiyue; Chen, Fuyong; Kang, Dezhi; Zhang, Shizhong

    2014-09-01

    The basal ganglia-cortical circuits are important for information process to brain function. However, chronic recording of single-unit activities in the basal ganglia nucleus has not yet been well established. We present a movable bundled microwire array for chronic subthalamic nucleus (STN) single-unit electrocorticogram co-recording. The electrode assembly contains a screw-advanced microdrive and a microwire array. The array consists of a steel guide tube, five recording wires and one referenced wire which form the shape of a guiding hand, and one screw electrode for cortico-recording. The electrode can acquire stable cortex oscillation-driven STN firing units in rats under different behaving conditions for 8 weeks. We achieved satisfying signal-to-noise ratio, portions of cells retaining viability, and spike waveform similarities across the recording sections. Using this method, we investigated neural correlations of the basal ganglia-cortical circuits in different behaving conditions. This method will become a powerful tool for multi-region recording to study normal statements or movement disorders.

  5. 肝豆状核变性患者中文书写时皮质下结构的功能影像研究%The role of the basal ganglia in processing of Chinese writing: evidence from a PET study in Wilson's disease

    Institute of Scientific and Technical Information of China (English)

    陈东; 刘晓加; 吴湖炳; 梁秀龄; 李洵桦

    2009-01-01

    ObjectiveTo investigate the role of basal ganglia in processing of Chinese writing by Wilson' disease(WD). Methods7 WD patients were divided into two groups which were normal writing group and dysgraphia group. They were scanned by 18F-fluorodeoxyglucose(18F-FDG) positron emission tomography respectively while performing two tasks: 1) pseudo-writing,2) Chinese character writing. Data were analyzed with Statistical Parametric Mapping. ResultsCompared with pseudo-writing,patients in normal writing group showed greater activation of bilateral lateral globus pallidus and right putamen,whereas patients in dysgraphia group showed greater activation of right ventral lateral nucleus,claustrum,left putamen and lateral globus pallidus(P<0.01). Conclusions1) The results indicate that Chinese writing of WD patients involves in bilateral subcortical structure. Right basal ganglia plays more important role. 2) Activated areas in bilateral basal ganglia of WD patients with agraphia are different with WD patients with normal writing and right thalamus play a compensatory role when WD patients with agraphia are writing.%目的 通过观察脑型肝豆状核变性患者中文字词书写的皮质下结构激活特点,为基底神经节在书写中的作用机制提供实验数据.方法 将7例脑型肝豆状核变性患者分成正常书写组和书写障碍组,分别进行假写作业、中文字词书写作业的18氟脱氧葡萄糖(18F-fluorodeoxyglucose,18F-FDG)脑功能成像,用统计参数图软件(SPM2)得出基底神经节变化区域.结果 正常书写组的皮质下结构激活区包括双侧苍白球和右侧壳核,书写障碍组包括右侧丘脑腹外侧核、屏状核和左侧壳核、苍白球,均差异有显著性(P<0.01).结论 1)脑型肝豆状核变性患者的中文书写涉及双侧基底神经节,右侧基底神经节可能发挥更重要的作用.2)伴有书写障碍的肝豆状核变性患者双侧基底神经节激活点与正常书写的患

  6. Recovery of language function in Korean-Japanese crossed bilingual aphasia following right basal ganglia hemorrhage.

    Science.gov (United States)

    Lee, Boram; Moon, Hyun Im; Lim, Sung Hee; Cho, Hyesuk; Choi, Hyunjoo; Pyun, Sung-Bom

    2016-06-01

    Few studies have investigated language recovery patterns and the mechanisms of crossed bilingual aphasia following a subcortical stroke. In particular, Korean-Japanese crossed bilingual aphasia has not been reported. A 47-year-old, right-handed man was diagnosed with an extensive right basal ganglia hemorrhage. He was bilingual, fluent in both Korean and Japanese. After his stroke, the patient presented with crossed aphasia. We investigated changes in the Korean (L1) and Japanese (L2) language recovery patterns. Both Korean and Japanese versions of the Western Aphasia Battery (WAB) were completed one month after the stroke, and functional magnetic resonance imaging (fMRI) was performed using picture-naming tasks. The WAB showed a paradoxical pattern of bilingual aphasia, with an aphasia quotient (AQ) of 32 for Korean and 50.6 for Japanese, with Broca's aphasia. The patient scored better in the Japanese version of all domains of the tests. The fMRI study showed left lateralized activation in both language tasks, especially in the inferior frontal gyrus. After six months of language therapy targeting L1, the Korean-WAB score improved significantly, while the Japanese-WAB score showed slight improvement. In this case, the subcortical lesion contributed to crossed bilingual aphasia more highly affecting L1 due to loss of the cortico-subcortical control mechanism in the dominant hemisphere. The paradoxical pattern of bilingual aphasia disappeared after lengthy language therapy targeting L1, and the therapy effect did not transfer to L2. Language recovery in L1 might have been accomplished by reintegrating language networks, including the contralesional language homologue area in the left hemisphere.

  7. Clinical characteristics and prognosis of traumatic basal ganglia hematomas: A retrospective analysis of 40 cases

    Institute of Scientific and Technical Information of China (English)

    Jialiang Li; Chunjiang Yu

    2006-01-01

    AIM: To retrospectively analyze the pathogenesis, clinical characteristics, treatment and prognostic characteristics in patients with traumatic basal ganglia hematomas (TBGH).METHODS: A retrospective analysis of the clinical data was performed in 40 patients with TBGH who were selected from 1 250 patients with closed brain injury, who admitted to the Department of Neurosurgery of Shangqiu First People's Hospital from January 1990 to January 2004. The pathogenesis, clinical characteristics and signs, results of radiological examination, treatment and prognostic characteristics were analyzed. The patients all had definite history of brain injury, manifested by neurological functional disturbance to different extent after brain injury, and basal ganglia hemorrhage was identified by CT after brain injury, and hemorrhagic volume were more than or equal to 2 mL. Totally 34 males and 6 females were enrolled, aged 16-72 years and 28 cases of them were younger than 40 years old. The prognosis of the patients was evaluated with Glasgow outcome scale (GOS) at 6 months after injury, and GOS scoring standard was 1-5 points (1 for dead; 2 for vegetative survival, long-term coma, manifestations of decorticate rigidity or decerebrate rigidity; 3 for severely disabled, should be look after by others; 4 for moderately disabled, be able in self-care; 5 for good recovery, adults can work and study).RESULTS: The enrolled cases accounted for 3.20% of the 1250 patients with closed brain injury admitted at the same period. ① The causes of injury included traffic accident in 36 cases, fall in 2 cases, and assault in 2 cases. ② At admission, the Glasgow coma scale (GCS) scores were as follow: 13-15 scores (mild) in 10 cases,9-12 scores (moderate)in 20 cases, and 3-8 scores (severe) in 10 cases. Hemiplegia presented in 37 cases,aphasia in 20 cases, conscious disturbance in 10 cases, unilateral mydriasis in 6 cases, and decerebrate rigidity in 2 cases. ③ TBGH was detected by CT within

  8. Singing-related neural activity distinguishes two putative pallidal cell types in the songbird basal ganglia: comparison to the primate internal and external pallidal segments

    OpenAIRE

    Goldberg, Jesse H.; Adler, Avital; Bergman, Hagai; Fee, Michale S

    2010-01-01

    The songbird area X is a basal ganglia homologue that contains two pallidal cell types—local neurons that project within the basal ganglia and output neurons that project to the thalamus. Based on these projections, it has been proposed that these classes are structurally homologous to the primate external (GPe) and internal (GPi) pallidal segments. To test the hypothesis that the two area X pallidal types are functionally homologous to GPe and GPi neurons, we recorded from neurons in area X ...

  9. Loss of function of Slc20a2 associated with familial idiopathic basal ganglia calcification in humans causes brain calcifications in mice

    DEFF Research Database (Denmark)

    Jensen, Nina; Daa Schrøder, Henrik; Kildall Hejbøl, Eva;

    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....

  10. Methylphenidate exposure induces dopamine neuron loss and activation of microglia in the basal ganglia of mice.

    Directory of Open Access Journals (Sweden)

    Shankar Sadasivan

    Full Text Available BACKGROUND: Methylphenidate (MPH is a psychostimulant that exerts its pharmacological effects via preferential blockade of the dopamine transporter (DAT and the norepinephrine transporter (NET, resulting in increased monoamine levels in the synapse. Clinically, methylphenidate is prescribed for the symptomatic treatment of ADHD and narcolepsy; although lately, there has been an increased incidence of its use in individuals not meeting the criteria for these disorders. MPH has also been misused as a "cognitive enhancer" and as an alternative to other psychostimulants. Here, we investigate whether chronic or acute administration of MPH in mice at either 1 mg/kg or 10 mg/kg, affects cell number and gene expression in the basal ganglia. METHODOLOGY/PRINCIPAL FINDINGS: Through the use of stereological counting methods, we observed a significant reduction (∼20% in dopamine neuron numbers in the substantia nigra pars compacta (SNpc following chronic administration of 10 mg/kg MPH. This dosage of MPH also induced a significant increase in the number of activated microglia in the SNpc. Additionally, exposure to either 1 mg/kg or 10 mg/kg MPH increased the sensitivity of SNpc dopaminergic neurons to the parkinsonian agent 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP. Unbiased gene screening employing Affymetrix GeneChip® HT MG-430 PM revealed changes in 115 and 54 genes in the substantia nigra (SN of mice exposed to 1 mg/kg and 10 mg/kg MPH doses, respectively. Decreases in the mRNA levels of gdnf, dat1, vmat2, and th in the substantia nigra (SN were observed with both acute and chronic dosing of 10 mg/kg MPH. We also found an increase in mRNA levels of the pro-inflammatory genes il-6 and tnf-α in the striatum, although these were seen only at an acute dose of 10 mg/kg and not following chronic dosing. CONCLUSION: Collectively, our results suggest that chronic MPH usage in mice at doses spanning the therapeutic range in humans, especially at

  11. 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.)

  12. Effect of Levodopa Chronic Administration on Behavioral Changes and Fos Expression in Basal Ganglia in Rat Model of PD

    Institute of Scientific and Technical Information of China (English)

    徐岩; 孙圣刚; 曹学兵

    2003-01-01

    To study behavioral character and changes of neuronal activity in the basal ganglia of ratmodel of levodopa-induced dyskinesia, unilateral 6-hydroxydopamine lesioned rat model of Parkin-son disease (PD) was treated with levodopa/benserazide twice daily for 4 weeks and the behaviorobserved on the 1st, 3rd, 4th, 7th, 9th, 10th, 14th, 21st and 28th day. The animals were sacri-ficed and immunohistochemical technique was used to measure the changes of Fos expression in thecaudate putamen (CPU), globus pallidus (GP) and sensorimotor area of cerebral cortex 2 h afterthe last treatment. The results showed that pulsatile treatment with a subthreshold dose of levodo-pa gradually induced abnormal involuntary movement (AIM), including stereotypy (limb dyskine-sia, axial dystonia and masticatory dyskinesia) towards the side contralateral to the dopamine-den-ervated striatum and increased contraversive rotation. The motor pattern of each subtype was highlystereotypic across individual rats, and the proportion of each subtype was not consistent among in-dividual rats. Fos positive nuclei in the CPU and GP were increased by levodopa acute administra-tion, and more remarkably in the CPU, but not in the cerebral cortex. After repeated levodopatreatment, Fos positive nuclei were reduced remarkably in the CPU, but were increased in the GPand cerebral cortex. It was concluded that the neural mechanisms underlying levodopa induced AIMin rat model of PD was very similar to those seen in levodopa-induced dyskinesia (LID) in PD pa-tients and MPTP-lesioned monkeys, and increased striatopallidal neuronal activity might be involvedin occurrence of LID.

  13. Measurement of basal ganglia volume of patients with Parkinson disease using 3.0T MR susceptibility-weighted imaging%3.0T MR磁敏感加权成像测量帕金森病患者基底节区体积

    Institute of Scientific and Technical Information of China (English)

    刘芳; 范国光; 王慈; 徐克; 孙文阁

    2011-01-01

    Objective To analyze changes of basal ganglia volume of patients with Parkinson disease (PD ) with 3. OT MR SWI . Methods Totally 20 patients with early PD , 20 patients with advanced PD and 20 age-matched healthy suhjects underw ent MRI . The volumes of' caudate nucleus , putamen and globus pallidus were measured with SWI . Correlation analysis of basal ganglia volume with Hoehn & Yahr grading and the course of PD was carried out . Results Compared with that of normal volunteers . the volume of' putamen in patients with early PD decreased 10. 79% ( Pdisease . Conclusion MRI volumetric. measurement technique can provide certain assistance f'or the diagnosis of PD .%目的 应用3.0T磁共振SWI分析帕金森病患者基底节区体积的变化,探讨其诊断帕金森病的价值.方法 采用3.0T MRI对40例早期、中晚期帕金森病患者和年龄匹配的20名正常人进行扫描,分别测量尾状核、壳核及苍白球的体积,并与Hoehn & Yahr分级及病程行相关性分析.结果 早期、中晚期帕金森病患者壳核体积较正常人分别下降10.79%(P<0.05)和22.66%(P<0.05),中晚期患者较早期患者下降13.31%(P<0.05).中晚期患者苍白球体积较正常人下降11.65%(P<0.05),较早期患者下降8.51%(P<0.05).帕金森病患者壳核体积与Hoehn & Yahr分级呈负相关 (r=-0.987, P<0.001),与病程无相关性.结论 MRI体积测量技术能为帕金森

  14. Chromosome 10p deletion in a patient with hypoparathyroidism, severe mental retardation, autism and basal ganglia calcifications.

    Science.gov (United States)

    Verri, Annapia; Maraschio, Paola; Devriendt, Koen; Uggetti, Carla; Spadoni, Emanuela; Haeusler, Edward; Federico, Antonio

    2004-01-01

    Chromosome 10p terminal deletions have been associated with a DiGeorge like phenotype. Haploinsufficiency of the region 10p14-pter, results in hypoparathyroidism, sensorineural deafness, renal anomaly, that is the triad that features the HDR syndrome. Van Esch (2000) identified in a HDR patient, within a 200 kb critical region, the GATA3 gene, a transcription factor involved in the embryonic development of the parathyroids, auditory system and kidneys. We describe a new male patient, 33-year-old, with 10p partial deletion affected by hypocalcemia, basal ganglia calcifications and a severe autistic syndrome associated with mental retardation. Neurologically he presented severe impairment of language, hypotonia, clumsiness and a postural dystonic attitude. A peripheral involvement of auditory pathways was documented by auditory evoked potentials alterations. CT scan documented basal ganglia calcifications. Hyperintensity of the lentiform nuclei was evident at the MRI examination. Renal ultrasound scan was normal. Haploinsufficiency for GATA3 gene was documented with FISH analysis using cosmid clone 1.2. Phenotypic spectrum observed in del (10p) is more severe than the classical DGS spectrum. GATA3 has been found to regulate the development of serotoninergic neurons. A serotoninergic dysfunction may be linked with autism in this patient. PMID:15337474

  15. Incomplete and inaccurate vocal imitation after knockdown of FoxP2 in songbird basal ganglia nucleus Area X.

    Directory of Open Access Journals (Sweden)

    Sebastian Haesler

    2007-12-01

    Full Text Available The gene encoding the forkhead box transcription factor, FOXP2, is essential for developing the full articulatory power of human language. Mutations of FOXP2 cause developmental verbal dyspraxia (DVD, a speech and language disorder that compromises the fluent production of words and the correct use and comprehension of grammar. FOXP2 patients have structural and functional abnormalities in the striatum of the basal ganglia, which also express high levels of FOXP2. Since human speech and learned vocalizations in songbirds bear behavioral and neural parallels, songbirds provide a genuine model for investigating the basic principles of speech and its pathologies. In zebra finch Area X, a basal ganglia structure necessary for song learning, FoxP2 expression increases during the time when song learning occurs. Here, we used lentivirus-mediated RNA interference (RNAi to reduce FoxP2 levels in Area X during song development. Knockdown of FoxP2 resulted in an incomplete and inaccurate imitation of tutor song. Inaccurate vocal imitation was already evident early during song ontogeny and persisted into adulthood. The acoustic structure and the duration of adult song syllables were abnormally variable, similar to word production in children with DVD. Our findings provide the first example of a functional gene analysis in songbirds and suggest that normal auditory-guided vocal motor learning requires FoxP2.

  16. 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.

  17. 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.)

  18. 帕金森病患者基底节区的磁共振氨基质子转移成像研究%Amide proton transfer MR imaging at 3.0 T of the basal ganglia in Parkinson's disease

    Institute of Scientific and Technical Information of China (English)

    王蕊; 李春媚; 陈敏; 张晨; 周进元; 苏闻

    2015-01-01

    目的 探讨磁共振氨基质子转移成像(APT)技术对于发现帕金森病患者基底节异常改变的可行性.方法 收集27例帕金森病患者和23名年龄及性别相匹配的健康对照者进行头颅APT成像和常规磁共振检查.测量双侧苍白球、壳核和尾状核的酰胺质子不对称磁化转移率(MTRasym),分别采用独立样本t检验和配对样本t检验比较帕金森病患者与健康对照者、帕金森病患者起病侧和对侧各脑结构MTRasym (3.5 ppm)的差异.使用单因素方差分析比较健康对照者和不同严重程度帕金森病患者间各脑结构MTRasym(3.5 ppm)的差异.结果 帕金森病患者苍白球、壳核和尾状核的MTRasym (3.5 ppm)均高于健康对照者[分别为(0.89±0.12)%与(0.57 ±0.16)%,(1.05±0.11)%与(0.82±0.15)%,(1.15±0.13)%与(0.78 ± 0.19)%;t=3.311、2.562和3.277,均P<0.05].健康对照组、轻度和中重度帕金森病组基底节各脑结构MTRasym(3.5 ppm)的差异具有统计学意义,且轻度帕金森病患者苍白球、壳核和尾状核的MTRasym(3.5 ppm)明显高于健康对照者.帕金森病患者基底节各脑结构起病侧的MTRasym(3.5 ppm)虽均略低于对侧,但差异均无统计学意义.结论 APT成像技术可以敏感地显示早期帕金森病患者和健康对照者基底节各脑结构MTRasym(3.5 ppm)的差异,是一种评价帕金森病患者脑代谢异常的有效工具.%Objective To explore the feasibility of amide proton transfer (APT) MR imaging for the detection of basal ganglia abnormalities in patients with Parkinson' s disease (PD).Methods Twentyseven patients with PD and twenty-three age-matched normal control subjects underwent cerebral APT and structural MR imaging.The magnetic resonance ratio asymmetry (MTRasym) values at 3.5 ppm of bilateral globus pallidus,putamen and caudate were measured on APT images.MTRasym (3.5 ppm) values of cerebral structures between PD patients and control subjects were compared with

  19. Probing the Role of Medication, DBS Electrode Position, and Antidromic Activation on Impulsivity Using a Computational Model of Basal Ganglia.

    Science.gov (United States)

    Mandali, Alekhya; Chakravarthy, V Srinivasa

    2016-01-01

    Everyday, we encounter situations where available choices are nearly equally rewarding (high conflict) calling for some tough decision making. Experimental recordings showed that the activity of Sub Thalamic Nucleus (STN) increases during such situations providing the extra time needed to make the right decision, teasing apart the most rewarding choice from the runner up closely trailing behind. This prolonged deliberation necessary for decision making under high conflict was absent in Parkinson's disease (PD) patients who underwent Deep Brain Stimulation (DBS) surgery of STN. In an attempt to understand the underlying cause of such adverse response, we built a 2D spiking network model (50 × 50 lattice) of Basal ganglia incorporating the key nuclei. Using the model we studied the Probabilistic learning task (PLT) in untreated, treated (L-Dopa and Dopamine Agonist) and STN-DBS PD conditions. Based on the experimental observation that dopaminergic activity is analogous to temporal difference (TD) and induces cortico-striatal plasticity, we introduced learning in the cortico-striatal weights. The results show that healthy and untreated conditions of PD model were able to more or less equally select (avoid) the rewarding (punitive) choice, a behavior that was absent in treated PD condition. The time taken to select a choice in high conflict trials was high in normal condition, which is in agreement with experimental results. The treated PD (Dopamine Agonist) patients made impulsive decisions (small reaction time) which in turn led to poor performance. The underlying cause of the observed impulsivity in DBS patients was studied in the model by (1) varying the electrode position within STN, (2) causing antidromic activation of GPe neurons. The effect of electrode position on reaction time was analyzed by studying the activity of STN neurons where, a decrease in STN neural activity was observed for certain electrode positions. We also observed that a higher antidromic

  20. Probing the Role of Medication, DBS Electrode Position, and Antidromic Activation on Impulsivity Using a Computational Model of Basal Ganglia

    Science.gov (United States)

    Mandali, Alekhya; Chakravarthy, V. Srinivasa

    2016-01-01

    Everyday, we encounter situations where available choices are nearly equally rewarding (high conflict) calling for some tough decision making. Experimental recordings showed that the activity of Sub Thalamic Nucleus (STN) increases during such situations providing the extra time needed to make the right decision, teasing apart the most rewarding choice from the runner up closely trailing behind. This prolonged deliberation necessary for decision making under high conflict was absent in Parkinson's disease (PD) patients who underwent Deep Brain Stimulation (DBS) surgery of STN. In an attempt to understand the underlying cause of such adverse response, we built a 2D spiking network model (50 × 50 lattice) of Basal ganglia incorporating the key nuclei. Using the model we studied the Probabilistic learning task (PLT) in untreated, treated (L-Dopa and Dopamine Agonist) and STN-DBS PD conditions. Based on the experimental observation that dopaminergic activity is analogous to temporal difference (TD) and induces cortico-striatal plasticity, we introduced learning in the cortico-striatal weights. The results show that healthy and untreated conditions of PD model were able to more or less equally select (avoid) the rewarding (punitive) choice, a behavior that was absent in treated PD condition. The time taken to select a choice in high conflict trials was high in normal condition, which is in agreement with experimental results. The treated PD (Dopamine Agonist) patients made impulsive decisions (small reaction time) which in turn led to poor performance. The underlying cause of the observed impulsivity in DBS patients was studied in the model by (1) varying the electrode position within STN, (2) causing antidromic activation of GPe neurons. The effect of electrode position on reaction time was analyzed by studying the activity of STN neurons where, a decrease in STN neural activity was observed for certain electrode positions. We also observed that a higher antidromic

  1. 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.

  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...... subjects, the MSA patients had a substantially lower number of neurons in the substantia nigra (p=0.001), putamen (p=0.001), and globus pallidus (pputamen (p=0.......04) and globus pallidus (p=0.01). In the MSA brains the total number of astrocytes was significantly higher in the putamen (p=0.04) and caudate nucleus (p=0.01). In all examined regions a higher number of microglia were found in the MSA brains with the greatest difference observed in the otherwise unaffected red...

  3. Mean-field modeling of the basal ganglia-thalamocortical system. I Firing rates in healthy and parkinsonian states.

    Science.gov (United States)

    van Albada, S J; Robinson, P A

    2009-04-21

    Parkinsonism leads to various electrophysiological changes in the basal ganglia-thalamocortical system (BGTCS), often including elevated discharge rates of the subthalamic nucleus (STN) and the output nuclei, and reduced activity of the globus pallidus external (GPe) segment. These rate changes have been explained qualitatively in terms of the direct/indirect pathway model, involving projections of distinct striatal populations to the output nuclei and GPe. Although these populations partly overlap, evidence suggests dopamine depletion differentially affects cortico-striato-pallidal connection strengths to the two pallidal segments. Dopamine loss may also decrease the striatal signal-to-noise ratio, reducing both corticostriatal coupling and striatal firing thresholds. Additionally, nigrostriatal degeneration may cause secondary changes including weakened lateral inhibition in the GPe, and mesocortical dopamine loss may decrease intracortical excitation and especially inhibition. Here a mean-field model of the BGTCS is presented with structure and parameter estimates closely based on physiology and anatomy. Changes in model rates due to the possible effects of dopamine loss listed above are compared with experiment. Our results suggest that a stronger indirect pathway, possibly combined with a weakened direct pathway, is compatible with empirical evidence. However, altered corticostriatal connection strengths are probably not solely responsible for substantially increased STN activity often found. A lower STN firing threshold, weaker intracortical inhibition, and stronger striato-GPe inhibition help explain the relatively large increase in STN rate. Reduced GPe-GPe inhibition and a lower GPe firing threshold can account for the comparatively small decrease in GPe rate frequently observed. Changes in cortex, GPe, and STN help normalize the cortical rate, also in accord with experiments. The model integrates the basal ganglia into a unified framework along with an

  4. 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)

  5. 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.

  6. fMRI of Cocaine Self-Administration in Macaques Reveals Functional Inhibition of Basal Ganglia

    OpenAIRE

    Mandeville, Joseph B.; Choi, Ji-Kyung; Jarraya, Bechir; Rosen, Bruce R.; Jenkins, Bruce G.; Vanduffel, Wim

    2011-01-01

    Disparities in cocaine-induced neurochemical and metabolic responses between human beings and rodents motivate the use of non-human primates (NHP) to model consequences of repeated cocaine exposure in human subjects. To characterize the functional response to cocaine infusion in NHP brain, we employed contrast-enhanced fMRI during both non-contingent injection of drug and self-administration of cocaine in the magnet. Cocaine robustly decreased cerebral blood volume (CBV) throughout basal gang...

  7. 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

  8. Exploring the cognitive and motor functions of the basal ganglia: an integrative review of computational cognitive neuroscience models

    Science.gov (United States)

    Helie, Sebastien; Chakravarthy, Srinivasa; Moustafa, Ahmed A.

    2013-01-01

    Many computational models of the basal ganglia (BG) have been proposed over the past twenty-five years. While computational neuroscience models have focused on closely matching the neurobiology of the BG, computational cognitive neuroscience (CCN) models have focused on how the BG can be used to implement cognitive and motor functions. This review article focuses on CCN models of the BG and how they use the neuroanatomy of the BG to account for cognitive and motor functions such as categorization, instrumental conditioning, probabilistic learning, working memory, sequence learning, automaticity, reaching, handwriting, and eye saccades. A total of 19 BG models accounting for one or more of these functions are reviewed and compared. The review concludes with a discussion of the limitations of existing CCN models of the BG and prescriptions for future modeling, including the need for computational models of the BG that can simultaneously account for cognitive and motor functions, and the need for a more complete specification of the role of the BG in behavioral functions. PMID:24367325

  9. The arbitration-extension hypothesis: a hierarchical interpretation of the functional organization of the basal ganglia

    Directory of Open Access Journals (Sweden)

    Iman eKamali Sarvestani

    2011-03-01

    Full Text Available Based on known anatomy and physiology, we present a hypothesis where the basal gangliamotor loop is hierarchically organized in two main subsystems: the arbitration system andthe extension system. The arbitration system, comprised of the subthalamic nucleus, globuspallidus, and pedunculopontine nucleus, serves the role of selecting one out of several candidateactions as they are ascending from various brain stem motor regions and aggregated in thecentromedian 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 findsthe strongest response among several candidates to execute. This decision is communicatedback to the brain stem by facilitating the desired action via cholinergic/glutamatergic projectionsand suppressing conflicting alternatives via GABAergic connections. The extension system,comprised of the striatum and, again, globus pallidus, can extend the repertoire of responsesby 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 Booleanlogic rules using striatal neurons that only fire given specific constellations of inputs (BooleanAND and pallidal neurons that are silenced by any striatal input (Boolean OR. We demonstratethe capabilities of this hierarchical system by a computational model where a simulatedgeneric animal interacts with an environment by selecting direction of movement basedon combinations of sensory stimuli, some being appetitive, others aversive or neutral. Whilethe arbitration system can autonomously handle conflicting actions proposed by brain stemmotor nuclei, the extension system is required to execute learned actions not suggested byexternal motor centers. Being precise in the functional role of each component of the system,this hypothesis generates several readily testable predictions.

  10. Usefulness of computed tomography in the diagnosis of cryptococcal meningoencephalitis. Multiple low density lesions in the basal ganglia and corona radiata

    Energy Technology Data Exchange (ETDEWEB)

    Tokumaru, Yukio; Kojima, Shigeyuki; Yamada, Tatsuo; Ito, Naoki; Hirayama, Keizo (Chiba Univ. (Japan). School of Medicine)

    1982-11-01

    In 2 cases of cryptococcal meningoencephalitis, we found multiple round low density lesions in the basal ganglia and corona radiata by CT scan. Both cases were treated successfully with amphotericin B and 5-fluorocytosine. Pathologically, cryptococcal meningoencephalitis usually shows two types of lesions: one being gelatinous and the other granulomatous. The former is a cystic lesion which mainly invades the cerebral cortex, dentate nucleus and basal ganglia; the latter is a granuloma as a result of histological reaction common to any of fungal organism. In granulomatous lesions, CT scan usually shows a high density or ring enhancement by contrast medium. In our 2 cases, CT scan showed multiple low density spots with no enhancement. We thought that they might represent gelatinous lesions. We stressed the importance of checking serial CT scans for the diagnosis of chronic meningoencephalitis of unknown etiology.

  11. 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.

  12. 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. PMID:25389391

  13. Believer-Skeptic Meets Actor-Critic: Rethinking the Role of Basal Ganglia Pathways during Decision-Making and Reinforcement Learning

    OpenAIRE

    Dunovan, Kyle; Verstynen, Timothy

    2016-01-01

    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 inste...

  14. Combining self-organizing maps with mixtures of experts: Application to an Actor-critic model of reinforcement learning in the Basal Ganglia

    OpenAIRE

    Khamassi, Mehdi; Martinet, Louis-Emmanuel; Guillot, Agnés

    2006-01-01

    International audience In a reward-seeking task performed in a continuous environment, our previous work compared several Actor-Critic architectures implementing dopamine-like reinforcement learning mechanisms in the rat's basal ganglia. The task complexity imposes the coordination of several submodules, each module being an expert trained in a particular subset of the task. Our results illustrated the consequences of different hypotheses about the management of Actor-Critic submodules. We...

  15. A network model of basal ganglia for understanding the roles of dopamine and serotonin in reward-punishment-risk based decision making

    OpenAIRE

    Pragathi Priyadharsini Balasubramani; Srinivasa eChakravarthy; Balaraman eRavindran; Moustafa, Ahmed A.

    2015-01-01

    There is significant evidence that in addition to reward-punishment based decision making, the Basal Ganglia (BG) contributes to risk-based decision making (Balasubramani et al., 2014). 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 (RL)-based model of the BG that simulates the interactions between dopamine (DA) and serotonin (5HT) in a diverse ...

  16. 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.

  17. Basal ganglia stroke due to mild head trauma in pediatric age - clinical and therapeutic management: a case report and 10 year literature review.

    Science.gov (United States)

    Landi, Alessandro; Marotta, Nicola; Mancarella, Cristina; Marruzzo, Daniele; Salvati, Maurizio; Delfini, Roberto

    2011-01-06

    Ischemia of the basal ganglia as an immediate consequence of minor head injury in children is rare (< 2% of all ischemic stroke in childhood) and is due to vasospasm of the lenticulostriate arteries. The clinical history of these lesions is particularly favourable because they are usually small, and also because the facial-brachial-crural hemiparesis typical of this pathology usually regresses after a period ranging from several weeks to several months, despite the persistence of an ischemic area on MRI. This is due to the well known neuronal plasticity of the CNS, in particular, of the primary motor cortex. The most effective therapeutic approach appears to be the conservative one, although the best treatment regimen is still not well defined.Young patients should be closely monitored and treated conservatively with osmotic diuretics to reduce perilesional edema. At the same time, however, it is very important to exclude, by means of instrumental and laboratory studies, conditions that could favour the onset of ischemia, including emboligen heart disease, thrombophilia and acute traumatic arterial dissections. Generally speaking, the prognosis in these cases is good. The authors describe their experience treating a 10-month old baby girl, with a left lenticular nucleus ischemia and report a literature review.

  18. 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.

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

    Science.gov (United States)

    Berthet, Pierre; Lansner, Anders

    2014-01-01

    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. PMID:24614169

  20. Action selection performance of a reconfigurable Basal Ganglia inspired model with Hebbian-Bayesian Go-NoGo connectivity

    Directory of Open Access Journals (Sweden)

    Pierre eBerthet

    2012-10-01

    Full Text Available Several studies have shown a strong involvement of the basal ganglia (BG in action selection and dopamine dependent learning. The dopaminergic signal to striatum, the input stage of the BG, has been commonly described as coding a reward prediction error (RPE, i.e. the difference between the predicted and actual reward. The RPE has been hypothesized to be critical in the modulation of the synaptic plasticity in cortico-striatal synapses in the direct and indirect pathway. We developed an abstract computational model of the BG, with a dual pathway structure functionally corresponding to the direct and indirect pathways, and compared its behaviour to biological data as well as other reinforcement learning models. The computations in our model are inspired by Bayesian inference, and the synaptic plasticity changes depend on a three factor Hebbian-Bayesian learning rule based on co-activation of pre- and post-synaptic units and on the value of the RPE. The model builds on a modified Actor-Critic architecture and implements the direct (Go and the indirect (NoGo pathway, as well as the reward prediction (RP system, acting in a complementary fashion. We investigated the performance of the model system when different configurations of the Go, NoGo and RP system were utilized, e.g. using only the Go, NoGo, or RP system, or combinations of those. Learning performance was investigated in several types of learning paradigms, such as learning-relearning, successive learning, stochastic learning, reversal learning and a two-choice task. The RPE and the activity of the model during learning were similar to monkey electrophysiological and behavioural data. Our results, however, show that there is not a unique best way to configure this BG model to handle well all the learning paradigms tested. We thus suggest that an agent might dynamically configure its action selection mode, possibly depending on task characteristics and also on how much time is available.

  1. Action selection performance of a reconfigurable basal ganglia inspired model with Hebbian-Bayesian Go-NoGo connectivity.

    Science.gov (United States)

    Berthet, Pierre; Hellgren-Kotaleski, Jeanette; Lansner, Anders

    2012-01-01

    Several studies have shown a strong involvement of the basal ganglia (BG) in action selection and dopamine dependent learning. The dopaminergic signal to striatum, the input stage of the BG, has been commonly described as coding a reward prediction error (RPE), i.e., the difference between the predicted and actual reward. The RPE has been hypothesized to be critical in the modulation of the synaptic plasticity in cortico-striatal synapses in the direct and indirect pathway. We developed an abstract computational model of the BG, with a dual pathway structure functionally corresponding to the direct and indirect pathways, and compared its behavior to biological data as well as other reinforcement learning models. The computations in our model are inspired by Bayesian inference, and the synaptic plasticity changes depend on a three factor Hebbian-Bayesian learning rule based on co-activation of pre- and post-synaptic units and on the value of the RPE. The model builds on a modified Actor-Critic architecture and implements the direct (Go) and the indirect (NoGo) pathway, as well as the reward prediction (RP) system, acting in a complementary fashion. We investigated the performance of the model system when different configurations of the Go, NoGo, and RP system were utilized, e.g., using only the Go, NoGo, or RP system, or combinations of those. Learning performance was investigated in several types of learning paradigms, such as learning-relearning, successive learning, stochastic learning, reversal learning and a two-choice task. The RPE and the activity of the model during learning were similar to monkey electrophysiological and behavioral data. Our results, however, show that there is not a unique best way to configure this BG model to handle well all the learning paradigms tested. We thus suggest that an agent might dynamically configure its action selection mode, possibly depending on task characteristics and also on how much time is available. PMID:23060764

  2. 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.

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

    Directory of Open Access Journals (Sweden)

    Kenji eMorita

    2014-04-01

    Full Text Available 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 towards 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

  4. 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

  5. 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

  6. Signal enhancement in the output stage of the basal ganglia by synaptic short-term plasticity in the direct, indirect and hyper direct pathways

    Directory of Open Access Journals (Sweden)

    Mikael eLindahl

    2013-06-01

    Full Text Available Many of the synapses in the basal ganglia display short-term plasticity. Still, computational models have not yet been used to investigate how this affects signaling. Here we use a model of the basal ganglia network, constrained by available data, to quantitatively investigate how synaptic short-term plasticity affects the substantia nigra reticulata (SNr, the basal ganglia output nucleus. We find that SNr becomes particularly responsive to the characteristic burst-like activity seen in both direct and indirect pathway striatal medium spiny neurons (MSN. As expected by the standard model, direct pathway MSNs are responsible for decreasing the activity in SNr. In particular, our simulations indicate that bursting in only a few percent of the direct pathway MSNs is sufficient for completely inhibiting SNr neuron activity. The standard model also suggests that SNr activity in the indirect pathway is controlled by MSNs disinhibiting the subthalamic nucleus (STN via the globus pallidus externa (GPe. Our model rather indicates that SNr activity is controlled by the direct GPe-SNr projections. This is partly because GPe strongly inhibits SNr but also due to depressing STN-SNr synapses. Furthermore, depressing GPe-SNr synapses allow the system to become sensitive to irregularly firing GPe subpopulations, as seen in dopamine depleted conditions, even when the GPe mean firing rate does not change. Similar to the direct pathway, simulations indicate that only a few percent of bursting indirect pathway MSNs can significantly increase the activity in SNr. Finally, the model predicts depressing STN-SNr synapses, since such an assumption explains experiments showing that a brief transient activation of the hyperdirect pathway generates a tri-phasic response in SNr, while a sustained STN activation has minor effects. This can be explained if STN-SNr synapses are depressing such that their effects are counteracted by the (known depressing GPe-SNr inputs.

  7. 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...

  8. Basal ganglia volumes in drug-naive first-episode schizophrenia patients before and after short-term treatment with either a typical or an atypical antipsychotic drug

    DEFF Research Database (Denmark)

    Glenthoj, Andreas; Glenthoj, Birte Y; Mackeprang, Torben;

    2007-01-01

    The present study examined basal ganglia volumes in drug-naive first-episode schizophrenic patients before and after treatment with either a specific typical or atypical antipsychotic compound. Sixteen antipsychotic drug-naive and three minimally medicated first-episode schizophrenic patients...... or intracranial volume, the only significant difference between patients and controls was a Hemisphere x Group interaction for the caudate nucleus at baseline, with controls having larger left than right caudate nuclei and patients having marginally larger right than left caudate volumes. Within patients, the two...

  9. 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

    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...

  10. The microdissection in cerebrum insula and basal ganglia region%大脑岛叶与基底节区的显微解剖

    Institute of Scientific and Technical Information of China (English)

    冯三平

    2012-01-01

    Objective To have an intimate knowledge of the corresponding anatomic correlation among cerebrum insular cortex, basal ganglia region and internal capsule in order to provide some important anatomic basis for cleaning hypertensive hemorrhage in basal ganglia region via lateral fissure-insular approach. Methods Dissection and measurement were performed in insular lobe and basal ganglia region of 10 adult cadaveric heads ( 20 hemispheres ). Results The insular cortex,viewed from a lateral side, was triangular ( widest superiorly and narrowest inferiorly ), with an apical elevation that gave it an overall pyramidal configuration. The insular lobe was located in the depth of the sylvian fissure, covered by the frontal, parietal and temporal, and the depths covered the deep basal nuclei, internal capsule and thalamus. On the middle horizontal section of the insular lobe,the sulci and gyri of insular lobe were all corresponding inward the putamen, in which the middle and posterior short gyri were corresponding the widest part of the putamen and genu of internal capsule. The central sulcus of insula was the closest distance to the putamen, corresponding inward posterior part of putamen and posterior limb of internal capsule as well as thalamus. Conclusion To have an intimate knowledge of the anatomy of the insular lobe and basal ganglia region is helpful to clean intracerebral hematomas in basal ganglia region via sylvian fissure insular surgical approach, and can further reduce the incidence of complications caused by internal capsule injury.%目的 熟悉大脑岛叶和基底节区的解剖,探讨岛叶皮质和基底节、内囊等的对应解剖关系,为经外侧裂-岛叶入路,清除高血压基底节出血时,提供一些重要的解剖依据.方法 对10例(20侧)成人尸头标本进行岛叶和基底节区的解剖与测量.结果 从侧面看,岛叶皮层是一个上宽,下窄的三角形,加上岛顶的高度,整体看岛叶就是一个金字塔结构.岛叶

  11. 脑梗死后基底节性失语的临床分析%Clinical analysis of basal ganglia aphasia after cerebral infarction

    Institute of Scientific and Technical Information of China (English)

    丁杰

    2013-01-01

    目的:探讨脑梗死后基底节性失语临床特点,为提高患者诊断与治疗效果提供可靠依据。方法9例脑梗死后基底节性失语患者均出现音韵节律、语调、看图命名、动作描述以及书写障碍,部分患者发生听理解及复述障碍,患者自发性语言可表现为流畅性或非流畅性。结果治疗后,其听、说、读能力均较治疗前显著提高,3例患者书写能力明显改善,6例患者书写能力未改善;临床治愈2例,显效7例,治疗总有效率为100.00%。结论脑梗死后基底节性失语患者均可出现不同程度的表达障碍,不利于其保持积极心态尽快恢复健康,根据患者具体症状采用针对性的康复训练措施,可显著提高患者语言能力,保障患者生活质量。%Objective To investigate the clinical characteristics of basal ganglia aphasia after cerebral infarction, and to provide reliable basis for diagnosis and treatment. Methods 9 patients with basal ganglia aphasia after cerebral infarction all appeared phonological rhythm,intonation, picture naming, action description and writing disorders, some patients appeared listen understand and repeat disorders,spontaneous language can be expressed as smooth or non-fluency. Results After the treatment,their listening,speaking,reading ability improved significantly,3 patients'ability to write were significantly improved,6 patients did not improve writing skills.2 cases were cured,7 cases were markedly,the total effective rate was 100.00%. Conclusion The basal ganglia aphasia after infarction patients all may have varying degrees of expression barriers,it's detrimental to their health restored as soon as possible,according to specific symptoms in patients to use targeted rehabilitation measures can significantly improve patients' language ability,protect the quality of patients'life.

  12. A network model of basal ganglia for understanding the roles of dopamine and serotonin in reward-punishment-risk based decision making.

    Science.gov (United States)

    Balasubramani, Pragathi P; Chakravarthy, V Srinivasa; Ravindran, Balaraman; Moustafa, Ahmed A

    2015-01-01

    There is significant evidence that in addition to reward-punishment based decision making, the Basal Ganglia (BG) contributes to risk-based decision making (Balasubramani et al., 2014). 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 (RL)-based model of the BG that simulates the interactions between dopamine (DA) and serotonin (5HT) in a diverse set of experimental studies including reward, punishment and risk based decision making (Balasubramani et al., 2014). Starting with the classical idea that the activity of mesencephalic 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 the neural substrates for value computation, we propose that DA D1R and D2R co-expressing MSNs are capable of computing risk. Though the existence of MSNs that co-express D1R and D2R are reported by various experimental studies, prior existing computational models did not include them. Ours is the first model that accounts for the computational possibilities of these co-expressing D1R-D2R MSNs, and describes how DA and 5HT mediate activity in these classes of neurons (D1R-, D2R-, D1R-D2R- MSNs). Starting from the assumption that 5HT modulates all MSNs, our study predicts significant modulatory effects of 5HT on D2R and co-expressing D1R-D2R MSNs which in turn

  13. A clinico-radiological phenotype of voltage-gated potassium channel complex antibody-mediated disorder presenting with seizures and basal ganglia changes.

    Science.gov (United States)

    Hacohen, Yael; Wright, Sukhvir; Siddiqui, Ata; Pandya, Nikki; Lin, Jean-Pierre; Vincent, Angela; Lim, Ming

    2012-12-01

    In childhood, central nervous system (CNS) presentations associated with antibodies to voltage-gated potassium channel (VGKC) complex include limbic encephalitis, status epilepticus, epileptic encephalopathy, and autistic regression. We report the cases of two individuals (a 6-year-old male and an 11-year-old female) who presented with an acute-onset explosive seizure disorder with positive VGKC complex antibodies and bilateral basal ganglia changes on magnetic resonance imaging (MRI). Both patients made a complete clinical recovery, without immunotherapy, with resolution of the MRI changes and normalization of the antibody levels. Extended antibody testing, including testing for leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein 2, and contactin-2 was negative. This could suggest that the clinico-radiological phenotype in our patients may in fact be associated with a novel autoreactive target(s) within the VGKC complex, as may be the case in other children with VGKC complex-mediated CNS disorders.

  14. Believer-Skeptic Meets Actor-Critic: Rethinking the Role of Basal Ganglia Pathways during Decision-Making and Reinforcement Learning.

    Science.gov (United States)

    Dunovan, Kyle; Verstynen, Timothy

    2016-01-01

    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. PMID:27047328

  15. 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.

  16. 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.

  17. Believer-Skeptic Meets Actor-Critic: Rethinking the Role of Basal Ganglia Pathways during Decision-Making and Reinforcement Learning

    Science.gov (United States)

    Dunovan, Kyle; Verstynen, Timothy

    2016-01-01

    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. PMID:27047328

  18. Analysis on Surgery for Hypertensive Cerebral Hemorrhage in Basal Ganglia Regions%基底节区高血压脑出血手术治疗分析

    Institute of Scientific and Technical Information of China (English)

    汤秉洪; 覃宗明; 杨明彬; 陈建刚

    2012-01-01

    Objective To study the surgical timing, method and curative effect of surgery on hypertensive cerebral hemorrhage in basal ganglia regions. Methods We reviewed the clinical data of 168 patients undergoing operation cures for hypertensive cerebral hemorrhage in basal ganglia regions from January 2006 to January 2011. There were 98 males and 70 females with their age ranging from 35 to 84 years old averaging at 65.2 years. The time between onset of the disease and admission to hospital ranged from 0.5 to 48 hours averaging 7.1 hours. At admission, the conscious status was classified as class I in 32 patients, II in 46, III in 41, IV in 28, and V in 21. Head CT examination at admission showed the lateral type in 51 patients, medial type in 71, and mixed type in 46. The volume of hematoma was 25 to 50 mL in 76 patients, 50 to 80 mL in 53, and larger than 80 mL in 39. The small window craniotomy was performed in 127 cases, and lines of bone flap craniotomy was performed in 41 cases. Results Among the 168 patients, 16 died (9.52%). Re-hemorrhage occurred in 8 patients 4 to 28 hours after operation, among whom immediate operation was performed to remove the hematoma in 6 patients, non operation treatment in 2 cases, and 4 patients died. Six patients died of large volume of hematoma or hemiation. Pulmonary or urinary tract infection occurred in 3 patients, and multiple organ failure in 3 patients. According to Glasgow outcome scale (GOS) score at discharge, the outcome was good in 82 patients, moderate disability in 46, severe disability in 16, persistent vegetative in 8, and 16 died. Patients were followed up for 3 to 6 months, and according to the daily work capacity (ADL) classification, there were 33 cases of class I , 49 of class Ⅱ , 54 of class Ⅲ , 8 of class Ⅳ , and 8 of class Ⅴ . Conclusion Ultra early or early operation done under direct vision, clearing hematoma completely, and reliable coagulation of the bleeding arteries responsible for the hematoma

  19. 基底节缺血性卒中对认知功能的影响%A study on cognitive function after ischemic basal ganglia's stroke

    Institute of Scientific and Technical Information of China (English)

    王久武; 孙月吉; 庞鑫鑫; 林媛; 于亮; 李倩; 婉思莹; 周世煜; 郇明明

    2009-01-01

    目的 探讨基底节缺血性卒中导致的认知功能损害特点.方法 基底节缺血性卒中住院患者46例为观察组,所有病例均符1995年10月中华医学会第四届脑血管病学术研讨会通过的脑卒中诊断标准;对照组为性别、年龄和教育程度与观察组相匹配的健康人46例.认知评价采用一般问卷、韦氏成人智力量表的词汇及数字符号测试、韦氏记忆量表、工作记忆课题及威斯康星卡片等,共收集了20项认知功能相关指标.结果 观察组的连线作业A[(54.04±5.66)分]、执行完成分类数[(3.56±0.12)分]、执行错误应答数[(16.17±0.58)分]、执行非持续性错误数[(10.17±0.58)分]的得分显著高于对照组(t=4.67,5.03,9.45,9.5;P0,P<0.05),与顺背与倒背呈负相关(r=-0.857,-0.811;P=0.014,0.027);左侧基底节缺血性卒中体积与词汇测试、经历、视觉再认呈负相关(r=-0.764,-0.907,-0.747;P=0.027,0.002,0.033);右侧基底节缺血性卒中体积与词汇测试、数字符号、视觉再生、执行完成分类数呈负相关(r=-0.747,-0.770,-0.798;P=0.033,0.026,0.011).结论 基底节缺血性卒中可以引起言语智能、执行功能及记忆等认知功能改变,两侧基底节在操作智能、长时记忆及执行功能方面发挥作用不同,基底节卒中体积越大,认知功能损害越明显.%Objective To find the correlation factors of cognitive disorder after ischemic basal ganglia's stroke. Methods 46 cases of ischemic basal ganglia's stroked patients by MRI. And 46 cases health control were tested by Wechsler Adult Intelligence Scale (WAIS),Wechsler Memory Scale (WMS),Trail Making Test A and B,Wisconsin Card Sorting Test (WCST).t test,chi-square,two independent samples and spearmancorrelation were used to analyze the data. Results 1)Group of thalamic stroke compare with health control for recognition index,there were significant different between the two groups,there were higher score in the stroke group at

  20. Neurochemical organization of the human basal ganglia: anatomofunctional territories defined by the distributions of calcium-binding proteins and SMI-32.

    Science.gov (United States)

    Morel, Anne; Loup, Fabienne; Magnin, Michel; Jeanmonod, Daniel

    2002-01-28

    The distribution of the calcium-binding proteins calbindin-D28K (CB), parvalbumin (PV) and calretinin (CR), and of the nonphosphorylated neurofilament protein (with SMI-32) was investigated in the human basal ganglia to identify anatomofunctional territories. In the striatum, gradients of neuropil immunostaining define four major territories: The first (T1) includes all but the rostroventral half of the putamen and is characterized by enhanced matriceal PV and SMI-32 immunoreactivity (-ir). The second territory (T2) encompasses most part of the caudate nucleus (Cd) and rostral putamen (PuT), which show enhanced matriceal CB-ir. The third and fourth territories (T3 and T4) comprise rostroventral parts of Cd and PuT characterized by complementary patch/matrix distributions of CB- and CR-ir, and the accumbens nucleus (Acb), respectively. The latter is separated into lateral (prominently enhanced in CB-ir) and medial (prominently enhanced in CR-ir) subdivisions. In the pallidum, parallel gradients also delimit four territories, T1 in the caudal half of external (GPe) and internal (GPi) divisions, characterized by enhanced PV- and SMI-32-ir; T2 in their rostral half, characterized by enhanced CB-ir; and T3 and T4 in their rostroventral pole and in the subpallidal area, respectively, both expressing CB- and CR-ir but with different intensities. The subthalamic nucleus (STh) shows contrasting patterns of dense PV-ir (sparing only the most medial part) and low CB-ir. Expression of CR-ir is relatively low, except in the medial, low PV-ir, part of the nucleus, whereas SMI-32-ir is moderate across the whole nucleus. The substantia nigra is characterized by complementary patterns of high neuropil CB- and SMI-32-ir in pars reticulata (SNr) and high CR-ir in pars compacta (SNc) and in the ventral tegmental area (VTA). The compartmentalization of calcium-binding proteins and SMI-32 in the human basal ganglia, in particular in the striatum and pallidum, delimits anatomofunctional

  1. Brain MR imaging in patients with hepatic cirrhosis: relationship between high intensity signal in basal ganglia on T{sub 1}-weighted images and elemental concentrations in brain

    Energy Technology Data Exchange (ETDEWEB)

    Maeda, H. [Department of Radiology, Wakayama Medical College, 27-7, Wakayama City, 640 (Japan); Sato, M. [Department of Radiology, Wakayama Medical College, 27-7, Wakayama City, 640 (Japan); Yoshikawa, A. [Department of Radiology, Wakayama Medical College, 27-7, Wakayama City, 640 (Japan); Kimura, M. [Department of Radiology, Wakayama Medical College, 27-7, Wakayama City, 640 (Japan); Sonomura, T. [Department of Radiology, Wakayama Medical College, 27-7, Wakayama City, 640 (Japan); Terada, M. [Department of Radiology, Wakayama Medical College, 27-7, Wakayama City, 640 (Japan); Kishi, K. [Department of Radiology, Wakayama Medical College, 27-7, Wakayama City, 640 (Japan)

    1997-08-01

    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 ({mu}g/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.

  2. 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.

  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.

    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.

  4. 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.

  5. [Hypomyelination with atrophy of the basal ganglia and cerebellum. Contribution of two new cases to a recently reported entity].

    Science.gov (United States)

    Tomás-Vila, Miguel; Menor, Francisco; Ley-Martos, Myriam; Jumillas-Luján, M José; Marco-Hernández, Ana V; Barbero, Pedro

    2014-02-16

    Introduccion. La hipomielinizacion con atrofia de ganglios basales y de cerebelo (H-ABC) es una rara entidad descrita recientemente. Se presentan dos nuevos casos pertenecientes a una misma familia. Casos clinicos. Caso 1: niño de 17 meses con retraso grave en todas las areas, ausencia de lenguaje y de contacto visual. En la exploracion destacaba una microcefalia con tetraparesia espastica. En la resonancia magnetica cerebral se apreciaba atrofia cerebelosa de predominio vermiano con perdida de volumen de ambos nucleos del putamen y la cabeza del caudado, y patron de hipomielinizacion de la sustancia blanca. En la electromiografia se objetivo un patron de polineuropatia cronica de predominio motor. Presento un descenso de los valores de acido homovalinico y de acido 5-hidroxindolacetico. El tratamiento con levodopa/carbidopa no fue efectivo. Caso 2: niña de 11 meses, hermana del caso anterior. Presentaba un retraso grave en todas las areas y en la exploracion clinica se detecto una microcefalia con tetraparesia espastica. La resonancia magnetica cerebral mostro hallazgos superponibles a los del hermano, con hipomielinizacion, atrofia cerebelosa y afectacion putaminal y de ambos caudados; en la electromiografia, hallazgos compatibles con polineuropatia motora de caracter desmielinizante. Presento un descenso de los valores de acido homovalinico y acido 5-hidroxindolacetico en el liquido cefalorraquideo. El tratamiento con levodopa/carbidopa resulto ineficaz. Conclusiones. Estos dos nuevos casos ayudan a caracterizar mejor esta entidad y refuerzan la hipotesis del origen genetico del sindrome, dado que se trata de dos casos pertenecientes a una misma familia.

  6. A novel mutation in TTC19 associated with isolated complex III deficiency, cerebellar hypoplasia, and bilateral basal ganglia lesions.

    Science.gov (United States)

    Melchionda, Laura; Damseh, Nadirah S; Abu Libdeh, Bassam Y; Nasca, Alessia; Elpeleg, Orly; Zanolini, Alice; Ghezzi, Daniele

    2014-01-01

    Isolated complex III (cIII) deficiency is a rare biochemical finding in mitochondrial disorders, mainly associated with mutations in mitochondrial DNA MTCYB gene, encoding cytochrome b, or in assembly factor genes (BCS1L, TTC19, UQCC2, and LYRM7), whereas mutations in nuclear genes encoding cIII structural subunits are extremely infrequent. We report here a patient, a 9 year old female born from first cousin related parents, with normal development till 18 months when she showed unsteady gait with frequent falling down, cognitive, and speech worsening. Her course deteriorated progressively. Brain MRI showed cerebellar vermis hypoplasia and bilateral lentiform nucleus high signal lesions. Now she is bed ridden with tetraparesis and severely impaired cognitive and language functions. Biochemical analysis revealed isolated cIII deficiency in muscle, and impaired respiration in fibroblasts. We identified a novel homozygous rearrangement in TTC19 (c.213_229dup), resulting in frameshift with creation of a premature termination codon (p.Gln77Argfs*30). Western blot analysis demonstrated the absence of TTC19 protein in patient's fibroblasts, while Blue-Native Gel Electrophoresis analysis revealed the presence of cIII-specific assembly intermediates. Mutations in TTC19 have been rarely associated with mitochondrial disease to date, being described in about ten patients with heterogeneous clinical presentations, ranging from early onset encephalomyopathy to adult forms with cerebellar ataxia. Contrariwise, the biochemical defect was a common hallmark in TTC19 mutant patients, confirming the importance of TTC19 in cIII assembly/stability. Therefore, we suggest extending the TTC19 mutational screening to all patients with cIII deficiency, independently from their phenotypes. PMID:25452764

  7. Preserved dichotomy but highly irregular and burst discharge in the basal ganglia in alert dystonic rats at rest.

    Science.gov (United States)

    Kumbhare, Deepak; Chaniary, Kunal D; Baron, Mark S

    2015-10-22

    Despite its prevalence, the underlying pathophysiology of dystonia remains poorly understood. Using our novel tri-component classification algorithm, extracellular neuronal activity in the globus pallidus (GP), STN, and the entopeduncular nucleus (EP) was characterized in 34 normal and 25 jaundiced dystonic Gunn rats with their heads restrained while at rest. In normal rats, neurons in each nucleus were similarly characterized by two physiologically distinct types: regular tonic with moderate discharge frequencies (mean rates in GP, STN and EP ranging from 35-41 spikes/s) or irregular at slower frequencies (17-20 spikes/s), with a paucity of burst activity. In dystonic rats, these nuclei were also characterized by two distinct principal neuronal patterns. However, in marked difference, in the dystonic rats, neurons were primarily slow and highly irregular (12-15 spikes/s) or burst predominant (14-17 spikes/s), with maintained modest differences between nuclei. In GP and EP, with increasing severity of dystonia, burstiness was moderately further increased, irregularity mildly further increased, and discharge rates mildly further reduced. In contrast, these features did not appreciably change in STN with worsening dystonia. Findings of a lack of bursting in GP, STN and EP in normal rats in an alert resting state and prominent bursting in dystonic Gunn rats suggest that cortical or other external drive is normally required for bursting in these nuclei and that spontaneous bursting, as seen in dystonia and Parkinson's disease, is reflective of an underlying pathophysiological state. Moreover, the extent of burstiness appears to most closely correlate with the severity of the dystonia. PMID:26210616

  8. Characterization and distribution of [125I]epidepride binding to dopamine D2 receptors in basal ganglia and cortex of human brain

    International Nuclear Information System (INIS)

    The distribution and pharmacology of the binding of 125I-epidepride, a substituted benzamide with high affinity and selectivity for dopamine (DA) D2 receptors in rat brain is described in human brain. Saturation analysis of the binding of 125I-epidepride to membranes derived from striatum and regions of cortex demonstrated similar Kd values (34 and 28-33 pM, respectively) but differing maximum density of binding site values (152 and 3-8 fmol/mg of protein, respectively). The pharmacological profile of binding in cortex was also similar to striatum (epidepride greater than spiperone greater than butaclamol = flupenthixol greater than clozapine) except that an additional low-affinity site, blocked by the alpha-2 adrenergic antagonist idazoxan, was present in cortex. Quantification by autoradiography also demonstrated the greatest binding in the basal ganglia, with the striatum exhibiting greater binding than the pallidal complex or midbrain regions. For the pallidum, binding in the external segment was higher than the internal segment. Within the midbrain the binding of 125I-epidepride correlated well with the known distribution of DA-containing cell bodies, with the substantia nigra (pars compacta and pars lateralis) and ventral tegmental area (A10) higher than area A8 and central gray. Binding in frontal and parietal cortex was highest in the internal layers (layers V and VI). Temporal cortex showed a 2-fold higher density of binding than other cortical regions and a trilaminar pattern; binding was greater in the external (layers I and II) and internal layers than in the middle layers (III and IV). This pattern changed in the parahippocampal complex. Within the lateral occipitotemporal cortex, binding was densest in layers I to III and very low in layers IV to VI, but binding was almost nonexistent in the adjacent entorhinal cortex

  9. Characterization and distribution of (125I)epidepride binding to dopamine D2 receptors in basal ganglia and cortex of human brain

    Energy Technology Data Exchange (ETDEWEB)

    Joyce, J.N.; Janowsky, A.; Neve, K.A. (Department of Psychiatry, University of Pennsylvania School of Medicine, Philadelphia (USA))

    1991-06-01

    The distribution and pharmacology of the binding of {sup 125}I-epidepride, a substituted benzamide with high affinity and selectivity for dopamine (DA) D2 receptors in rat brain is described in human brain. Saturation analysis of the binding of {sup 125}I-epidepride to membranes derived from striatum and regions of cortex demonstrated similar Kd values (34 and 28-33 pM, respectively) but differing maximum density of binding site values (152 and 3-8 fmol/mg of protein, respectively). The pharmacological profile of binding in cortex was also similar to striatum (epidepride greater than spiperone greater than butaclamol = flupenthixol greater than clozapine) except that an additional low-affinity site, blocked by the alpha-2 adrenergic antagonist idazoxan, was present in cortex. Quantification by autoradiography also demonstrated the greatest binding in the basal ganglia, with the striatum exhibiting greater binding than the pallidal complex or midbrain regions. For the pallidum, binding in the external segment was higher than the internal segment. Within the midbrain the binding of {sup 125}I-epidepride correlated well with the known distribution of DA-containing cell bodies, with the substantia nigra (pars compacta and pars lateralis) and ventral tegmental area (A10) higher than area A8 and central gray. Binding in frontal and parietal cortex was highest in the internal layers (layers V and VI). Temporal cortex showed a 2-fold higher density of binding than other cortical regions and a trilaminar pattern; binding was greater in the external (layers I and II) and internal layers than in the middle layers (III and IV). This pattern changed in the parahippocampal complex. Within the lateral occipitotemporal cortex, binding was densest in layers I to III and very low in layers IV to VI, but binding was almost nonexistent in the adjacent entorhinal cortex.

  10. Human-specific increase of dopaminergic innervation in a striatal region associated with speech and language: A comparative analysis of the primate basal ganglia.

    Science.gov (United States)

    Raghanti, Mary Ann; Edler, Melissa K; Stephenson, Alexa R; Wilson, Lakaléa J; Hopkins, William D; Ely, John J; Erwin, Joseph M; Jacobs, Bob; Hof, Patrick R; Sherwood, Chet C

    2016-07-01

    The dopaminergic innervation of the striatum has been implicated in learning processes and in the development of human speech and language. Several lines of evidence suggest that evolutionary changes in dopaminergic afferents of the striatum may be associated with uniquely human cognitive and behavioral abilities, including the association of the human-specific sequence of the FOXP2 gene with decreased dopamine in the dorsomedial striatum of mice. To examine this possibility, we quantified the density of tyrosine hydroxylase-immunoreactive axons as a measure of dopaminergic innervation within five basal ganglia regions in humans, great apes, and New and Old World monkeys. Our results indicate that humans differ from nonhuman primate species in having a significant increase in dopaminergic innervation selectively localized to the medial caudate nucleus. This region of the striatum is highly interconnected, receiving afferents from multiple neocortical regions, and supports behavioral and cognitive flexibility. The medial caudate nucleus also shows hyperactivity in humans lacking a functional FOXP2 allele and exhibits altered dopamine concentrations in humanized Foxp2 mice. Additionally, striatal dopaminergic input was not altered in chimpanzees that used socially learned attention-getting sounds versus those that did not. This evidence indicates that the increase in dopamine innervation of the medial caudate nucleus in humans is a species-typical characteristic not associated with experience-dependent plasticity. The specificity of this increase may be related to the degree of convergence from cortical areas within this region of the striatum and may also be involved in human speech and language. J. Comp. Neurol. 524:2117-2129, 2016. © 2015 Wiley Periodicals, Inc. PMID:26715195

  11. Can minimal invasive puncture and drainage for hypertension spontaneous basal ganglia intracerebral hemorrhage improve patient outcome: A prospective non-randomized comparative study

    Directory of Open Access Journals (Sweden)

    Guo-qiang WANG

    2014-08-01

    Full Text Available Objective The treatment of hypertensive spontaneous intracranial hemorrhage (ICH is still controversial. The purpose of the present study was to investigate whether minimally invasive puncture and drainage (MIPD could provide improved patient outcome compared with decompressive craniectomy (DC. Methods Eligible, consecutive patients with ICH (≥30 ml, in basal ganglia, within 24 hours of ictus were non-randomly assigned to receive MIPD (group A or to undergo DC (group B hematoma evacuation. The primary outcome was death at 30 days after onset. Functional independence was assessed at 1 year using the Glasgow Outcome Scale (GOS, scores range from 1 to 5, score 1 indicating death, ≥4 indicating functional independence, with lower scores indicating greater disability. Results A total of 198 patients met the per protocol analysis (84 cases in group A and 114 cases in group B, including 9 cases lost during follow-up (2 cases in group A and 7 cases in group B. For these 9 patients, their last observed data were used as their final results for intention-to-treat analysis. The mean age of all patients was 57.1 years (range of 31-95 years, and 144 patients were male. The initial Glasgow Coma Scale (GCS score was 8.1±3.4, and the National Institutes of Health Stroke Scale (NIHSS score was 20.8±5.3. The mean hematoma volume (HV was 56.7±23.0 ml (range of 30-144 ml, and there was extended intraventricular hemorrhage (IVH in 134 patients (67.7%. There were no significant intergroup differences in the above baseline data, except group A had a higher mean age (59.4±14.5 years than the mean age of group B (55.3±11.1 years, P =0.025. The total cumulative mortalities at 30 days and 1 year were 32.3% and 43.4%, respectively, and there were no significant differences between groups A and B (30 days: 27.4% vs 36.0%, P =0.203; 1 year: 36.1% vs 48.2%, P =0.112, respectively. However, the mortality for patients ≤60 years, NIHSS60 ml, deep coma and severe

  12. 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

  13. 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.

  14. Functional magnetic resonance imaging of basal ganglia. Activation mapping with FLASH sequences for BOLD contrast and high resolution; Funktionelle Magnetresonanztomographie der Basalganglien. Einsatz von FLASH-Sequenzen zum Aktivitaetsmapping mit BOLD-Kontrast und Hochaufloesung

    Energy Technology Data Exchange (ETDEWEB)

    Seelos, K.C. [Inst. fuer Radiologische Diagnostik, Klinikum Grosshadern, Univ. Muenchen (Germany); Bucher, S.F. [Neurologische Klinik und Poliklinik, Klinikum Grosshadern, Univ. Muenchen (Germany); Stehling, M.K. [Inst. fuer Radiologische Diagnostik, Klinikum Grosshadern, Univ. Muenchen (Germany); Oertel, W.H. [Neurologische Klinik und Poliklinik, Klinikum Grosshadern, Univ. Muenchen (Germany); Reiser, M. [Inst. fuer Radiologische Diagnostik, Klinikum Grosshadern, Univ. Muenchen (Germany)

    1995-04-01

    The activation pattern of putamen, internal and external division of globus pallidus was investigated during rapid pronation and supination of the right and left hand in 12 normal volunteers using a FLASH sequence with high resolution for functional magnetic resonance imaging (fMRI) at 1.5 T. The chosen paradigm for motor function led to a signal increase within the basal ganglia between 3 and 23%, depending on the structure and individual subject. In all cases significant activation could be found contralateral to the moving hand. In six cases activation was also found on the ipsilateral side. The activated areas within putamen, internal and external division of globus pallidus were less than 5 mm{sup 2}. These first results indicate that fMRI studies of basal ganglia are feasible and might be suitable for analyzing basal ganglia disorders. (orig.) [Deutsch] Das Aktivierungsmuster von Putamen, Globus pallidus internus und externus waehrend schneller Pronation und Supination von rechter und linker Hand wurde bei 12 normalen Probanden mit Hilfe einer fuer die funktionelle Magnetresonanztomographie (fMRT) geeigneten hochaufloesenden FLASH-Sequenz bei 1,5 Tesla untersucht. Der durch das Bewegungsparadigma verursachte Signalanstieg innerhalb der Basalganglien lag je nach Struktur und untersuchtem Individuum zwischen 3 und 23%. In allen Faellen war kontralateral zur bewegten Hand ein signifikanter Aktivitaetsanstieg nachweisbar. In 6 Faellen war auch auf der ipsilateralen Seite eine Aktivitaet nachweisbar. Die aktivierten Areale innerhalb von Putamen, Globus pallidus internus und externus waren nicht groesser als 5 mm{sup 2}. Diese ersten Ergebnisse zeigen, dass magnetresonanztomographische Funktionsuntersuchungen im Bereich der Basalganglien moeglich sind und geeignet erscheinen, um Erkrankungen dieser Systeme zu analysieren. (orig.)

  15. Clinlc and lmaging of the hypoxia cerebropathy in bilateral basal ganglia%双侧基底节区缺氧性脑病的临床与影像学特征

    Institute of Scientific and Technical Information of China (English)

    钟望涛; 郑华; 邢永前; 王义刚

    2001-01-01

    目的探讨双侧基底节区缺氧性脑病的临床表现及影像学特点。方法回顾性分析8例经头MRI证实为双侧基底节区缺氧性脑病患者的临床资料及影像学改变。结果8例均以肌张力增高、运动减少、姿势反射异常等帕金森综合征为主要表现,头MRI表现为双侧基底节区的壳核、苍白球、尾状核头部对称性长T1长T2信号改变。结论双侧基底节缺氧性脑病与皮层下型分水岭梗死(CWI)有完全不同的临床表现和影像学改变。%Objective To investigate the aetiology,pathogeny,clinic,and imaging of the hypoxia cerebropathy in bilateral basal ganglia.Methods The clinical features and imaging in 8 cases were analysed retrospectively after confirmed by MRI,with the hypoxia cerebropathy in bilateral basal ganglia.Results The main manifestation of 8 cases were Parkinsonian syndrome including hypertonia,hypomotor,and anomaly of the posture reflex,MRI scan shows the bilateral long T1 long T2 variation signal in putamens,pallidums and caput nuclei caudatis.Conclusion The clinical manifestation and imaging of the hypoxia cerebropathy in bilateral basal ganglia were different from the subcortical watershed infarct.

  16. Fahr′s disease and psychiatric syndromes: A case series

    Directory of Open Access Journals (Sweden)

    Deepak Ghormode

    2011-01-01

    Full Text Available Fahr′s disease is characterized by basal ganglia calcification with clinical manifestations in the form of neuropsychiatric disorders, neurological symptoms, and cognitive symptoms. In this case series, we describe two cases of basal ganglia calcification, one of whom presented with psychotic symptoms and the other with mood symptoms, and discuss the literature with regard to psychiatric manifestations of basal ganglia calcification.

  17. Clinical analysis of symmetrical pathological changes involving bilateral basal ganglia in 28 children.%儿童基底节对称性病变28例临床分析

    Institute of Scientific and Technical Information of China (English)

    徐三清; 刘艳; 方峰; 周华; 罗小平

    2009-01-01

    Objective To explore and analyze the pathogenesis,clinical characteristics and prognosis of symmetrical pathological changes involving bilateral basal ganglia in children. Methods Analyzing retrospectively clinical data of 28 inpatients with the performance of brain damage and symmetrical low-density lesions on plain CT scans and/or low-signal on MRI T1 weighted imaging, high-signal on MRI T2 weighted imaging involving bilateral basal ganglia. Results Six patients first had fever,cough and (or) vomiting,diarrhea and subsequently progressed rapidly to convulsions, coma and also had marked acidosis, increased blood lactate and pyruvate levels,in which three cases were diagnosed as methylmalonic acidaemia,two were diagnosed as α-keto-glutaric aciduria,one was diagnosed as lactic academia;One 7-month-old infant with delayed motor development,feeding difficulties and repeated seizure was diagnosed as lactic academia;One simple breast-feeding patient with cerebral vitamine Bl deficiency had hoarse cry,muscular weakness,convulsion and good effect to vitamine Bl intramuscular injection;Eighteen cases with hepatolenticular degeneration had muscular hypertonia,tremor, salivation, ataxia, speech unclear and memory decline, in which 13 cases were accompanied by hepatomegaly, 10 cases were accompanied by splenomegaly,two cases were accompanied by liver cirrhosis and two cases were accompanied by hypersplenism; One case with moldy sugarcane poisoning and one case with carbon monoxide-induced toxic encephopathy had cognitive and motor dysfunction which recovered slowly. Conclusion Many causes can lead to symmetrical pathological changes involving bilateral basal ganglia with diverse symptoms in children. The diseases should be diagnosed early by illness history, clinical features, imaging study and laboratory tests including the screening for metabolic disorders, which can help treat them effectively and improve the prognosis.%目的 探讨和分析儿童基底节区对称性

  18. 经外侧裂岛叶入路显微清除高血压性基底节区脑出血%Transsylvian-transinsular approach for the microsurgical removal of hypertensive basal ganglia hemorrhage

    Institute of Scientific and Technical Information of China (English)

    刘永; 张玉海; 何升学; 赵金兵; 朱海涛; 张光绪; 邹元杰

    2015-01-01

    目的:探讨在显微镜下经外侧裂-岛叶入路清除高血压性基底节区脑出血的手术要点及脑组织和血管保护。方法回顾性分析18例经外侧裂-岛叶入路显微手术清除基底节区高血压脑出血患者临床资料。术后复查头颅CT了解血肿清除情况。采用Karnofsky功能状态评分评估术后3~6个月患者功能状态。结果本组血肿清除>90%16例,80%~90%1例;再出血1例。其中1例患者因术后严重肺部感染死亡。随访3~6个月,KPS 90~100分3例,60~80分9例,30~50分4例,10~20分1例。结论经外侧裂-岛叶入路有利于保护重要的大脑皮层和血管,是显微清除高血压性基底节区脑出血安全有效的手术方式。%Objective To explore the key points of transsylvian-transinsular approach to remove the hypertensive basal ganglia hemorrhage and adjacent brain tissue and blood vessels protection . Methods The clinical data of 18 patients who underwent the transsylvian-transinsular approach to remove the hypertensive basal ganglia hemorrhage were analyzed retrospectively .The volume of remaining hematoma was evaluated by postoperative CT scan .The Karnofsky performance score was used to evaluated patient outcome of 3-6 months after the surgery .Results The evacuation rate of hematoma was >90% in 16 patients and 80%-90% in 1.One case occured rehemorrhage .One patient died from severe pulmonary infection .The follow-up from 3 to 6 months showed KPS was 90~100 in 3 patients,60~80 in 9,30 ~50 in 4 and 10 ~20 in 1.Conclusion The transsylvian-transinsular approach was benefit to protect important cerebral cortex and vessels ,thus being a safe and effective operation method for microscopic removal of hypertensive basal ganglia hemorrhage .

  19. Prognostic factors of analysis on patients with nonoperative treatment of intracerebral hemorrhage in basal ganglia%非手术治疗自发性基底节区脑出血预后因素分析

    Institute of Scientific and Technical Information of China (English)

    周焜; 黄冠又; 梁郸; 乔志立; 陈冲; 王恒福; 饶正西; 王诚; 卓志平

    2013-01-01

    Objective To investigate the factors influencing prognosis of nonoperative treatment of intracerebral hemorrhage in basal ganglia. Methods The clinical data and survival status of 109 patients with intracerebral hemorrhage in basal ganglia who were admitted to Neurosurgery of Guiyang Second People' s Hospital during the period from April 2005 to June 2012 were reviewed retrospectively. The survival analysis was analyzed with Kaplan-Meier method. The univariate analysis was used to determine the prognositic factors related with survival rate by Log-rank test. Multivariate factors for the survival rates were analyzed using the Cox proportional hazards regression model. Results Univariate analysis revealed that GOS scale, GCS scale, hypertension, hemorrhage volume, intraventricu-lar hemorrhage, pulmonary infection and glucose were the factors influencing prognostic factors of hypertensive brainstem hemorrhage. Multivariate analysis showed that GCS scale, hemorrhage volume and glucose were independent prognostic factors. Conclusions GCS scale, hemorrhage volume and glucose were important prognostic factors of intracerebral hemorrhage in basal ganglia.%目的 探讨非手术治疗自发性基底节区出血预后相关的因素.方法 回顾性分析贵阳市第二人民医院神经外科2005年4月至2012年6月收治的109例随访资料完整的患者,采用Kaplan-Meier法进行单因素分析.Log-rank法进行生存率显著性检验,Cox比例风险回归模型作多因素分析.结果 单因素分析显示入院时GOS评分、GCS评分、高血压、出血量、出血破入脑室、肺部感染及血糖与预后有关联.多因素分析显示GCS评分、出血量和血糖是自发性基底节区出血预后相关的独立危险因素.结论 发病时GCS评分、出血量和血糖水平是影响患者预后的重要因素.

  20. Behavior Cognition Computational Model Based on Cerebellum and Basal Ganglia Mechanism%基于小脑-基底神经节机理的行为认知计算模型

    Institute of Scientific and Technical Information of China (English)

    陈静; 阮晓钢; 戴丽珍

    2012-01-01

    针对智能体的行为认知问题,提出一种小脑与基底神经节相互协调的行为认知计算模型.该模型核心为操作条件学习算法,包括评价机制、行为选择机制、取向机制及小脑与基底神经节的协调机制.初期的学习信号来自于下橄榄体和黑质两部分,在熵的意义上说明该算法是收敛的.采用该学习方法为自平衡两轮机器人建立运动神经认知系统,利用RBF网络逼近行为和评价网络.仿真实验表明该方法改善仅有基底神经节作用的行为-评价算法学习速度慢和失败次数多的问题,学习后期通过温度的不断降低,加快学习速度,震荡逐渐消失,改善学习效果.%Aiming at agent' s behavioral cognition problem, a behavior cognition computational model based on the coordination of cerebellum and basal ganglia is proposed. Operant conditioning learning algorithm is the central algorithm including evaluation mechanism, action selection mechanism, tropism mechanism, and the coordination mechanism between cerebellum and basal ganglia. The learning signals come from not only the Inferior Olive but also the Substantia Nigra in the beginning. The convergence of the algorithm can be guaranteed in the sense of entropy. With the proposed method, a motor nerve cognitive system for the self-balancing two-wheeled robot has been built using the RBF neural network as the actor and evaluation function approximator. The simulation results show that the learning speed is increased as well as the failure times are reduced by the proposed method than by the Actor-Critic method with the only Basal Ganglia mechanism. Through decreasing temperature in the late stage, the learning speed is increased and the vibration disappeares eventually, and the learning effect is improved.

  1. 经侧裂-岛叶入路治疗高血压性基底节血肿疗效观察%Effect of Surgical Treatment for Hypertensive Basal Ganglia Hematomas Through Transsylvian-insular Approach

    Institute of Scientific and Technical Information of China (English)

    张一; 杨常春; 王强; 董博; 王卉; 张峰极

    2012-01-01

      Objective:To summarize the clinical experience of surgical treatment through transsylvian—insular approach for hypertensive basal ganglia hematomas in 26 patients from the year 2009~2011.Methods: A total of 26 patients with hypertensive basal ganglia hematomas underwent surgical treatment through transsylvian—insular approach.Results: After surgery, hematoma was nearly total evacuated in 19 cases, more than 90% in 3 patients,less than 80% in 4 cases. Two patients died.Follow—up assessment according to GOS for 21~31 months revealed good in 13 patients,moderate handicapped in 8 patients,severely handicapped in 4 patients and death in 2 patients. Conclusions: Surgical treatment through transsylvian-insular approach was effective for hypertensive basal ganglia hematomas patients.%  目的:分析经侧裂-岛叶入路治疗高血压基底节血肿疗效及手术要点。方法:回顾性分析我院2009年~2011年26例经侧裂-岛叶入路治疗的高血压基底节血肿患者的临床资料,手术方法及疗效。结果:26例患者中血肿完全清除19例,血肿清除率>90%3例,血肿清除率<80%4例。本组患者死亡2例,1例死于再出血,1例死于严重肺部感。存活患者随访21~31个月,按格拉斯哥预后评分(GOS评分),良好者13例,中残8,重残4,死亡1例。结论:经侧裂岛叶入路是治疗高血压性基底节出血的良好手术方式。

  2. The anatomy of transsylvian fissure-insular surgical approach for removal of intracerebral hematoma in basal ganglia region%经外侧裂-岛叶清除基底节血肿手术入路的解剖

    Institute of Scientific and Technical Information of China (English)

    冯三平; 冯继

    2012-01-01

    目的 探讨基底节脑出血手术中,经外侧裂岛叶入路的相关结构的解剖,做到既能彻底的清除血肿,又能最大程度地保护脑组织,减少术后并发症发生.方法 对10例(20侧)成人尸头标本进行外侧裂、岛叶、岛盖、大脑中动脉、基底节的相关解剖.结果 ①外侧裂的表面有3个分支与岛盖关系密切,为暴露岛叶提供有利条件;②岛叶呈一个金字塔外形,表面由4~7个岛回组成,周围以环岛沟为界与额顶颞叶分隔开;③大脑中动脉的M1沿蝶骨嵴向外走形,多在岛顶前下方的岛阈附近分叉;M2段彼此“(11)V”字形,或“网眼状”,在岛叶表面向后上扇形展开,并发出许多微小的穿支血管供应岛叶皮层.④岛叶向内对应了基底节、内囊,丘脑等结构,本研究中发现,在岛叶的中部水平切面上,岛叶的中后短回,垂直向内对应了壳核最为宽阔的部分和内囊的膝部.结论 熟悉外侧裂-岛叶手术入路中的解剖,有利于基底节血肿的清除.%Objective To study the anatomy via transsylvian fissure-insular surgical approach in the surgery of intracerebral hematomas in basal ganglia region for a good removal of hematoma, well preservation of brain tissues and decrease of post-operation complications. Methods Dissection and relative measurement were performed in the sylvian fissure, operculum insulae, insular, middle cerebral artery and basal ganglia of 10 adult cadaveric heads (20 hemispheres). Results There were 3 branches in the surface of sylvian fissure, which had a close relationship with the operculum insulae, making the exposure of the insular more easy; the insular had a shape of the incompletely inverted pyramid, had 4-7 insular gyri in its surface, and the anterior, superior and inferior limiting sulcus separated from frontal, parietal and temporal lobe along the periphery of the insula; the M1 of the middle cerebral artery (MCA) coursing outward the sphenoidal crest, and the

  3. 精神分裂症患者基底节功能连接的静息态 fMRI 研究%Resting - state functional magnetic resonance imaging study of functional connectivity of basal ganglia in schizophrenia

    Institute of Scientific and Technical Information of China (English)

    蒋宇超; 陈琳; 段明君; 陈曦; 杨宓; 邓佳燕; 赖永秀; 尧德中; 罗程

    2015-01-01

    Objective To explore the difference of functional connectivity of basal ganglia in schizophrenia during a resting state by functional magnetic resoncance imaging(fMRI). Methods 3. 0T fMRI was used to assess the whole brain activity of 15 schizophrenia patients and 12 health controls. Functional connectivity analysis based on basal ganglia was performed to obtain the significant differ-ence between two groups. Results Compared with the health controls,the patients showed significantly increased functional connectiv-ity between media superior frontal gyrus,posterior cingulate and caudate;increased functional connectivity between left superior frontal gyrus,right anterior cingulate and left pallidum;increased functional connectivity between left medial frontal gyrus and right pallidum;increased functional connectivity between left superior frontal gyrus and left putamen. Conclusion This study discovers increased func-tional connectivity between basal ganglia and crucial regions of Default Model Network(DMN). The results imply that basal ganglia -DMN loop altered aberrantly,which might be associated with the pathological mechanisms of schizophrenia.%目的:通过功能磁共振(fMRI)技术,探讨精神分裂症患者静息状态下与基底节异常连接的脑区。方法采用3.0T 功能磁共振成像技术检测15例精神分裂症患者与12例正常对照组在静息状态下的全脑功能活动。采用功能连接分析对比两组被试的基底节(双侧尾状核、壳核和苍白球共6个区域)与全脑功能连接的差异。结果与对照组相比,精神分裂症患者的内侧额上回、后扣带与尾状核的功能连接上升;左侧额上回、右侧前扣带与左侧苍白球功能连接上升;左内侧额上回与右侧苍白球功能连接上升;左侧额上回与左侧壳核功能连接上升。差异均有统计学意义。结论精神分裂症患者的基底节区域与默认网络的重要节点功能连接上升,提

  4. Evaluation of depression status following basal ganglia infarction by diffusion tensor magnetic resonance imaging%磁共振弥散张量成像对基底节区梗死后抑郁状况的评估

    Institute of Scientific and Technical Information of China (English)

    涂加善; 刘振华; 黄凡衡; 陈爱敏; 蔡卫卫; 祝淑贞; 赵连旭

    2012-01-01

    Objective To study the anatomical abnormalities of basal ganglia and research their influence on depression status in patients with post stroke depression (PSD)by diffusion tensor imaging (DTI) of MRI.Methods Patients with basal ganglia infarction were recruited,and divided into groups of PSD and non depression control group by Hamilton Depression Rating Scale (HAMD) assessment. All the patients were evaluated with National Institute of Health Stroke Scale ( NIHSS). And the patients were checked by DTI sequence.Fractional anisotropy (FA),average diffusion coefficient (ADC) values and the number of nerve fiber were measured in bilateral caudatum,pallidum,putamen and thalamus.Results The score of NIHSS (6.29 ± 3.45 ) was significantly higher in PSD group than that in non-depression group (3.95 ± 1.90 ;t =2.219,P =0.036). No significant difference was found between the two groups for the DTI data of the basal ganglia nuclei ( t =0.056-1.618,all P > 0.05 ). Compared with contralateral construction (0.40 ± 0.02 ),the FA value decreased in the left putamen ( 0.37 ± 0.03 ) in the PSD group ( t =2.243,P =0.045 ).By Spearman correlations analysis,the HAMD score was positively correlated with NIHSS score ( r =0.464,P =0.017 ),and negatively correlated with the FA values of left pallidum (r=-0.563,P=0.005),right pallidum (r=-0.416,P=0.035) and left putamen (r =-0.428,P =0.029).Conclusions The occurrence of PSD was associated with neurological functional deficit following basal ganglia infarction.The depression level was correlated with the increasing of NIHSS score,the reductions in bilateral pallidum and left putamen FA values.This research contributes to evaluation of the PSD status in patients with basal ganglia infarction.%目的 利用磁共振弥散张量成像(DTI)技术研究基底节区梗死患者相关解剖结构的异常改变,评估卒中后抑郁( PSD)的状况.方法 选取基底节区梗死患者,依汉密尔顿抑郁量表(HAMD-24)

  5. MRI上抽动秽语综合征患者基底节核团体积的变化%MRI volume measurement of basal ganglia volumes in patients with Tourette's syndrome

    Institute of Scientific and Technical Information of China (English)

    卢洁; 李坤成; 曹燕翔; 张晓华; 张苗; 隋昕

    2009-01-01

    目的 探讨MR测量基底节核团体积对抽动秽语综合征(Tourette's syndrome,TS)病因诊断的价值.方法 选取10例TS患者(TS组)和10名健康志愿者(对照组)进行MR扫描,分别测量双侧尾状核、壳核、苍白球的体积,对两侧的基底节体积采用配对t检验进行比较分析;将大脑ROI体积数值进行标准化处理,对TS组和对照组之间基底节体积采用独立样本t检验进行比较分析.结果 正常对照组左侧尾状核、壳核、苍白球体积及3者之和均大于右侧(P值均<0.05),TS组上述结构两侧比较差异尤统计学意义(P值均>0.05).根据大脑体积进行标准化处理后,TS患者左侧尾状核、壳核、苍白球体积分别为(7.06±0.48)、(8.81±1.01)、(2.64±0.38)cm3,正常对照组分别为(11.05±1.86)、(9.97±1.11)、(3.04 ± 0.37)cm3,TS组较对照组减小(t值分别为-6.577、-2.457、-2.376,P值均<0.05);TS组右侧尾状核体积[(7.32 ± 0.26)cm3]较对照组[(9.81 ±1.83)cm3]减小(t=-4.258,P<0.01),右侧壳核、苍白球体积与对照组比较差异尤统计学意义(P值均>0.05).结论 MRI显示TS患者基底节核团体积减小,这对研究其病理生理机制及神经病理变化有一定的价值.%Objective To evaluate MRI measurement of basal ganglia volumes in patients with Tourette's syndrome.Methods Ten patients with Tourette's syndrome(TS)and 10 healthy volunteers were studied.Volumes of bilateral candate,putamen and pallidum were measured,and the results were analyzed using paired t test.The basal ganglia volume was normalized according to individual brain volume.The basal ganglia volumes of TS patients were compared with normal control group using independent-sample t tesL Results In 10 healthy volunteers,volumes of the left caudate,putamen,pallidum were significantly larger compared with those of the right side(P<0.05).However,there were no significant differenees between bilateral basal ganglia volumes(P>0.05)in TS patients

  6. 印度人基底神经节英语书写功能的PET-CT研究%The function of basal ganglia in English writing:A PET-CT study on Indians

    Institute of Scientific and Technical Information of China (English)

    邢一兰; 刘晓加; 吴湖柄; 陈东

    2009-01-01

    Objective To explore the neuropsychological mechanism of basal ganglia in English writing and elucidate law of alphabetic writing by means of PET-CT. Methods Subjects were six healthy Indian overseas students,sophomore and junior undergraduate. PET-CT examinations of the pseudo-writing and the English writing were carried out successively with an interval of 3~5 days. Statistical parametric mapping (SPM) was used to compare the datum of the two tasks through paired-t test. After analyzing areas where the metabolism of glucose changed the images about activated cerebral regions in English writing were obtained. Results The metabolism of glucose in leftpatamen(x=-30,y=-11,z=4,t=2.34, Z=1.84, P=0.033) andcaudatehead(x=-8,y= 17, z=-4, t=2.08, Z=1.70, P=0.045) were increased in the comparison between English writing and English pseudo-writing. Conclusion Left basal ganglia participates in English writing with contralateral cerebral domi-nance in right-handedness.%目的 应用正电子发射计算机体层显像-计算机体层显像(PET-CT)探讨基底神经节在印度人英语书写中的神经心理学机制,并在此基础七阐释拼音文字书写规律.方法 以6名健康的大学本科印度留学生为研究对象,每人分别执行假写和英语书写作业,采用PET-CT进行扫描并获得数据,通过统计参数图对假写和英语书写的图像数据进行配对t检验.分析葡萄糖代谢变化的区域,获得英语书写所引起的脑功能激活图.结果 英语书写与假写比较时,左侧壳核(x=-30,Y=-11,z=4,t值=2.34,Z值=1.84,P值=0.033)、尾状核头(X=-8,Y=17,z=-4,t值=2.08,z值=1.70,P值=0.045)葡萄糖代谢增加.结论 左侧基底神经节参与英语书写过程,并在右利手人群中具有对侧优势性.

  7. 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

  8. Basal ganglia structure abnormalities of the children with the first-episode tic disorder: A magnetic resonance imaging study%首发抽动障碍儿童基底节结构异常的磁共振成像研究

    Institute of Scientific and Technical Information of China (English)

    孙锦华; 黄明金; 袁爱花; 李茜茜; 黄晓琦; 龚启勇; 郭兰婷

    2012-01-01

    patients with TD according to the Diagnostic and Statistical Manual of Mental Dis-orders. Fourth Edition (DSM-IV) diagnostic criteria, and 9 healthy children, age of 8 -10 years to conduct the MRI scans. The scale of Yale Global Tic Severity Scale (YGTSS) was used to assess the tic severity. The volumes of total basal ganglia and its subregions including caudate nucleus, putamen, globus pallidus were measured using the method of region of interest. The original volumes, relative volumes (the ratio between the original volume and total intracranial volume) and asymmetry index (AI) of the basal ganglia and its subregions between the two groups were compared. Finally, the correlations were analyzed between the relative volume changes of the basal ganglia and its subregions and the illness duration or the total scores of YGTSS. Results: The original volume of the right globus pallidus [(1.4±0.2) mL vs. (1. 1 ±0.1) mL]and relative volume [(0.9 ±0. 1) × 10-3 vs. (0.7 ±0.2) × 10 'J] in patients with TD were respectively significantly larger than those of controls (Ps 0.05). The orginal volume of the left caudate nucleus was larger than that of right caudate nucleus in the group of TD patients [ (4. 9 ± 0.4) mL vs. (4.7 ±0.5) mL] or controls [(4.7 ±0.5) mL vs. (4.5 ±0.5) mL], while there was no significant difference between left and right volume within other subregions (Ps 0.05). The relative volumes in the basal ganglia or its subregions did not significantly correlate with the severity of tic (r = -0.08 -0.63, Ps > 0.05) and the duration of the disease (r = -0.09 -0. 59, Ps >0.05). Conclusion:The right globus pallidus in TD patients is possibly larger than controls, which may be the abnormal brain structure of TD. The relative volume of any structure of basal ganglia has no significant correlation with the severity or course of disease.

  9. Basal cell carcinomas in a young woman with Steinert’s disease.

    OpenAIRE

    Miraglia, E; Cantisani, C.; Giustini, S; Ambrifi, M; Soda, G.; Calvieri, S.

    2014-01-01

    Steinert’s disease or Myotonic dystrophy type I (DM1) is an autosomal dominant disease characterized by myotonia, muscular dystrophy, cataracts, hypogonadism, frontal balding, and electrocardiographic alterations.Several tumors have been associated with DM1 such as pilomatricoma, thymomas and insulinomas. Herein, we describe the unusual onset of multiple basal cell carcinomas in a young woman with DM1.

  10. Neuropsychiatric manifestations of Fahr′s disease pathogenesis and potential for treatment

    Directory of Open Access Journals (Sweden)

    Raheel Mushtaq

    2013-01-01

    Full Text Available Fahr′s disease (FD is a rare neuropsychiatric disease consisting of bilateral basal ganglia calcification with neurological, cognitive, and psychiatric manifestations. We report here a sporadic case of FDs with its neuropsychology.

  11. 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. PMID:19056429

  12. 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...

  13. Association Between Depressive Disorders and Early Progressive Motor Deficits Among Patients with Cerebral Infarcts in Basal Ganglia Region%抑郁障碍与基底节区脑梗死患者早期运动障碍加重的关系

    Institute of Scientific and Technical Information of China (English)

    伍明; 李梅笑

    2013-01-01

    Objective To explore the impact of depressive disorders on early progressive motor deficits among patients with cerebral infarcts of basal ganglia region.Methods Eighty-five patients with first cerebral infarcts in basal ganglia region were enrolled into this study.All patients were examined with brain magnetic resonance imaging (MRI),magnetic resonance angiography (MRA) and diffusion weighted imaging (DWI).According to the Hamilton Depression Rating Scale scores on admission,patients were divided into non-depressive disorders group and depressive disorders group.Based on NIHSS scores of early progressive motor deficits,each above-mentioned group was further divided into stable subgroup and progressive subgroup.The occurrence rate of early progressive motor deficits,front-to-back ratio of volume of lesions (V2/V1),pathological changes in the middle cerebral artery (MCA),blood pressure,blood lipids and fasting blood glucose were compared between non-depressive disorders and depressive disorders groups.Results The occurrence rate of early progressive motor deficits in depressive disorders group was significantly higher than that of non-depressive disorders group (10/27,37.04 % vs 9/58,15.52%,x2 =4.92,P =0.03).MCAs were obviously stenosing or occlusive (37/85,43.53 %) in cerebral infarcts of basal ganglia region,but no statistically significant difference was found in the pathological changes in the MCA between non-depressive disorders and depressive disorders groups (x2 =0.34,P =0.56).V2/V1 of progressive subgroups was larger than that of stable subgroups,and V2/V1 of depressive disorders groups was significantly different from that of non-depressive disorders in progressive subgroups (F =167.39,P =0.00).Systolic blood pressure and fasting blood glucose were significantly higher in the progression of the disease,and there was a significantly correlation between fasting blood glucose and depressive disorders (r =0.425,P =0.000).Conclusions Depressive disorders

  14. 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.

  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. 基底节区血肿经外侧裂-岛叶手术入路的解剖学研究%Anatomical Study of the Surgical Approach to the Lateral Fissure of the Hematoma in the Basal Ganglia Region

    Institute of Scientific and Technical Information of China (English)

    贾振锋

    2015-01-01

    目的:研究基底节区血肿经外侧裂-岛叶手术入路的解剖学特点。方法对20例40侧成人尸头标本的外侧裂、岛叶、大脑基底节等进行相关解剖。结果岛叶的供血动脉为大脑中动脉 M2段,扇形展开,方向为从岛叶表面向后上,并将很多微小的穿支血管发出来对岛叶皮层进行供应;岛叶的中后短回在其中部水平切面上垂直向内和内囊膝部及壳核最为宽阔的部分相对应。结论深入研究基底节区血肿经外侧裂-岛叶手术入路的解剖学特点能够为临床手术治疗基底节区血肿提供科学有效的依据。%Objective To study the basal ganglia hematoma through lateral fissure-the insular cortex anatomy surgical approach. Methods 20 cases of 40 adult cadaver specimens side lateral fissure, insular cortex, basal ganglia and other brain related anatomy. Results For insular artery middle cerebral artery M2 segment, fan, backward direction from the island leaf surface, and a lot of small penetrating vessels issued to the supply of the insular cortex conducted, after a short return to the island leaves in which the upper portion of the horizontal section vertical knee inwards and the internal capsule and putamen most broad section correspond. Conclusion Depth study by the lateral fissure basal ganglia hematoma-the insular cortex anatomy surgical approach for clinical surgery can basal ganglia hematoma provide scientifically valid basis.

  17. Fronto-striatal grey matter contributions to discrimination learning in Parkinson's disease

    NARCIS (Netherlands)

    C. O'Callaghan; A.A. Moustafa; S. de Wit; J.M. Shine; T.W. Robbins; S.J.G. Lewis; M. Hornberger

    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

  18. 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%).

  19. 成人自发性基底节区脑出血患者日常生活活动能力的影响因素%Factors Affecting Activities of Daily Living in Patients with Spontaneous Basal Ganglia Hemorrhage

    Institute of Scientific and Technical Information of China (English)

    张志竖; 邹耀兵; 肖静; 江思德; 潘成德; 饶富兰; 张建新; 唐明山

    2011-01-01

    Objective To investigate the factors affecting activities of daily life (ADD in patients with first basal ganglia hemorrhage, and to formulate intervention strategies for improving the capability of ADL. Methods A prospective study was conducted on 97 patients with the first spontaneous intracerebral hemorrhage who survived with no surgical treatment. Demographic risk factors for stroke were examined and National Institute of Health stroke scale (NIHSS) and Glasgow coma score (GCS) were recorded on the day of admission. White blood cell(WBC) count and plasma glucose (PG) were measured on the second day of hospitalization. NIHSS score and Barthel index (BI) were recorded 3 weeks after onset. Occurrences of urinary tract and lung infection were determined after discharge from hospital. BI was recorded by clinic or telephone follow-up 3 months after onset. Results The amount of bleeding,initial PG levels, WBC count and initial NIHSS score were independently associated with BI at 3 weeks and 3 months after spontaneous intracerebral hemorrhage. Furthermore, urinary tract infection and the history of ischemic stroke were associated with BI at 3 months after intracerebral hemorrhage. Conclusion Positive measures should be taken to control risk factors so as to improve the capability of ADL in patients with spontaneous intracerebral hemorrhage.%目的 探讨未行手术治疗的成人首发基底节区脑出血患者日常生活活动能力的影响因素,以期早期制定干预措施,提高患者日常生活活动能力.方法 采用前瞻性队列研究连续收集成人首发基底节区脑出血未行手术治疗且存活的患者97例.入院当天记录人口基线资料,进行卒中危险因素调查,行美国国立卫生研究所脑卒中评分(national institute of health stroke scale,NIHSS)和格拉斯哥昏迷评分(GCS);入院次日清晨行白细胞计数、空腹血糖等多项实验室指标的测定;发病3周行NIHSS评分及Barthel指数(Barthelindex

  20. 基底神经节区梗死后失写症的神经心理学分析%The Neuropsychological Analysis of Agraphia After Basal Ganglia Infarction

    Institute of Scientific and Technical Information of China (English)

    金梅; 刘晓加; 陈东; 尹文刚

    2008-01-01

    目的:研究基底神经节(BG)区梗死所致汉语失写症的神经心理学特点.方法:采用汉语失写检查法(CAB)测试40例BG区梗死患者的书写能力,计算各项书写得分和失写指数.对失写组和非失写组头颅CT或MRI进行标准化处理,显示病灶并进行二维平面叠加,直观显示其病灶的集中趋势.结果:在40例患者中,左侧BG区损害21例,失写17例;右侧损害19例,失写4例.神经影像学二维叠加显示,BG区梗死致失写的病灶多位于左侧BG区,包括左侧壳核、尾状核头部和尾状核体;而较少位于右侧壳核和右侧尾状核体.BG区梗死所致失写以失语性失写为主,其特点为构字障碍、字词错写和语法错误.结论:BG区梗死可导致失语性失写症,提示BG参与了书写加工过程,是书写这一高级神经功能的皮质下中枢.%Objective:To investigate the neuropsychological characteristics of Chinese agraphia caused by basal ganglia(BG)infarction.Methods:The writing abilities of 40 patients with BG infarction were detected by Chinese agraphia battery(CAB),and all the writing scores and agraphia quotient were calculated.The head CT/MRI images in agraphia and non-agraphia groups were standardized,the infarction were revealed and the superposition of two-dimensional arrays were performed,so that the central tendency of infarction was visually displayed.Results: Among the 40 patients,21 had left BG infraction,and 17 had agraphia;19 had right BG infraction,and 4 had agraphia.The two-dimensional superimposing neuroimages showed that BG infarctions caused agraphia was mostly in the left BG,including the left putamen,the head and body of the caudate nucleus,but there were fewer infarctions in the right putamen and the body of the candate nucleus.BG infarction caused agraphia was mostly aphasic agraphia,which was characterized by the orthographic disorders,paragraphia,and grammar mistakes.Conclusions: BG infarction may result in aphasic agraphia

  1. Ganoderma Species Associated with Basal Stem Rot Disease of Oil Palm

    Directory of Open Access Journals (Sweden)

    Ling-Chie Wong

    2012-01-01

    Full Text Available Problem statement: Basal Stem Rot disease (BSR is one of the most serious diseases that have been causing major losses in the oil palm industry in Southeast Asia, especially in Malaysia and Indonesia. Several species of Ganoderma have been reported pathogenic to oil palm, however, the diversity and differentiation of the Ganoderma species were not widely studied and the identity of these species are still unclear which may lead to inaccurate and inefficient decision-making in disease management. Approach: In this study, several isolates of Ganoderma were collected in Sarawak, Malaysia and the Multiplex Polymerase Chain Reaction was carried out to differentiate the isolates into species level. This was followed by morphological studies of basidiocarp of the Ganoderma isolates cultivated via artificial cultivation whereby parameters, such as basidiocarp and spore size, color and physical morphology were recorded. Results: Multiplex PCR could be used to differentiate the Ganoderma isolates, however, optimization had to be done to obtain convincing results. Morphology studies of the Ganoderma isolates showed that spore length could be used to distinguish among the Ganoderma species. Conclusion: Three Ganoderma species viz., G. boninense, G. zonatum and G. miniatocinctum are associated with the basal stem rot disease in Sarawak. Further studies on Ganoderma morphological traits is suggested so that immediate identification method can be developed to give guidance in deciding the most suitable way for control measures without any delay, leading to reduced palm deaths and disease control cost, thus, reducing losses in the oil palm industry.

  2. 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. PMID:26525918

  3. 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.

  4. 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.

  5. Multivoxel magnetic resonance spectroscopy study of the bilateral basal ganglia regions in unilateral temporal lobe epilepsy patients%单侧颞叶癫病人双侧基底节区多体素MRS研究

    Institute of Scientific and Technical Information of China (English)

    刘兰; 刘筠; 许亮

    2016-01-01

    Objebtive To detect the metabolic changes in the bilateral basal ganglia regions of unilateral temporal lobe epilepsy (TLE) with multivoxel magnetic resonance spectroscopy. Methods Ten patients with left temporal lobe epilepsy, ten patients with right temporal lobe epilepsy, and 10 healthy volunteers were selected. Unilateral TLE were diagnosed based on clinical onset symptoms and EEG. The Liverpool seizure severity scale (LSSS) 2.0 was measured from all patients. Multivoxel 1H-MRS data were acquired by SIEMENS 3.0 superconducting MR scanner. Concentrations of N-acetyl aspartate (NAA), choline (Cho), and creatine (Cr) were measured symmetrically from bilateral head of caudate nucleus, putamen, and thalamus. The NAA/Cr and Cho/Cr ratios of each area of interest were calculated for inter-group statistical analysis. Pearson correlation analysis between metabolite ratios and LSSS 2.0 score were carried out. Results The NAA/Cr ratios of the left and right thalamus were 1.92 ±0.15 and 2.02 ±0.26, respectively, in the left TLE group;and 2.19 ±0.16 and 1.79±0.16, respectively, in the right TLE group. The ratios were significantly lower (P<0.05) than that of the control group which were 2.37±0.14 and 2.36±0.10, respectively. In the right TLE group, the NAA/Cr ratios of the ipsilateral thalamus was significant lower than that of the contralateral thalamus (1.79 ±0.16 vs 2.19 ±0.16, P<0.05). For TLE patients, the NAA/Cr ratios of the ipsilateral thalamus negatively correlated with LSSS 2.0 score (left TLE r=-0.667;right TLE r=-0.643, all P<0.05). Conclusion Patients with unilateral TLE have neuronal loss and/or dysfunction of bilateral thalamus, and both NAA/Cr ratios of the ipsilateral thalamus and LSSS 2.0 score can reflect the severity of seizures.%目的:采用多体素MRS探讨单侧颞叶癫日(TLE)病人双侧基底节区代谢物改变。方法选取根据临床发作症状和脑电图综合诊断的左侧TLE病人10例,右侧TLE病人10

  6. MR measurement of the basal ganglia volume in the Tourette syndrome%抽动秽语综合征患者双侧基底节结构体积的MR测量

    Institute of Scientific and Technical Information of China (English)

    廖凯兵; 黎桂平; 杨波; 冯敢生

    2014-01-01

    Objective To compare the volume of the basal ganglia in patients with Tourette syndrome(T S) and the normal volunteers and to explore the underlying anatomical basis of TS.Methods Thirty-one cases of TS (TS subjects),31 gender and age-matched subjects (the control subjects) were examined on a 3.0 T MRI system.The volume of the caudate nucleus,globus pallidus,putamen of the two sides and the brain volume were measured with volume analysis software,and the data were normalized according to the individual brain volume.Statistical analysis was performed using t test to compare between the TS subjects and the controls.Results The volume of the both sides of the caudate nucleus,putamen and globus pallidus of TS subjects were (4.11 ±0.12) and (3.76 ±0.11),(2.28 ±0.12)and(2.35 ±0.28),(4.98 ±0.20) and (4.89 ±0.31)cm3,while they were (4.88 ±0.19) and (4.30 ±0.12),(2.28 ±0.12)and (2.35 ± 0.28),(4.98 ± 0.20) and (4.89 ± 0.31) cm3 in the controls,respectively.There were significant differences in the bilateral caudate nucleus and globus pallidus between the TS subjects and control subjects (t =2.97,1.74,3.72,3.93,P < 0.05),but there were no significant differences of the volume in the bilateral putamen between the TS and control subjects(t =0.47,1.31,P >0.05).The volume was not significantly different between the left and right caudate nucleus in the TS subjects (t =1.81,P >0.05),but the left volume of the caudate nucleus was bigger in the control subjects compared with the right volume,however,there was significant difference between the bilateral caudate nucleus in the control subjects (t =2.34,P < 0.05).There were no differences of volume between the bilateral globus pallidus and putamen in both the TS and control subjects (t =1.12,1.44,1.68,0.38,P > 0.05).Conclusion The abnormal volume of caudate nucleus,putamen,and the globus pallidus may be involved in the pathogenesis of TS.%目的 对比研究抽动秽语综合征(TS)患者与健康志愿者基底

  7. Iodine-123 IMP SPECT before and after by-pass surgery in a patient with occlusion of left anterior and middle cerebral arteries with basal abnormal telangiectasis (unilateral Moyamoya disease)

    Energy Technology Data Exchange (ETDEWEB)

    Honda, Norinari; Machida, Kikuo; Takishima, Teruo; Kaizu, Hiroyuki; Sugimoto, Eiichi

    1987-09-01

    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 by-pass 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.

  8. Altered Disease Development in the eui Mutants and Eui Overexpressors Indicates that Gibberellins Negatively Regulate Rice Basal Disease Resistance

    Institute of Scientific and Technical Information of China (English)

    Dong-Lei Yang; Qun Li; Yi-Wen Deng; Yong-Gen Lou; Mu-Yang Wang; Guo-Xing Zhou; Ying-Ying Zhang; Zu-Hua He

    2008-01-01

    Gibberellins (GAs) form a group of important plant tetracyclic diterpenoid hormones that are involved in many aspects of plant growth and development. Emerging evidence implicates that GAs also play roles in stress responses. However, the role of GAs in biotic stress is largely unknown. Here, we report that knockout or overexpression of the Elongated uppermost internode (Eui) gene encoding a GA deactivating enzyme compromises or increases, respectively, disease resistance to bacterial blight (Xanthomonas oryzae pv. oyrzae) and rice blast (Magnaporthe oryzae). Exogenous application of GA and the inhibitor of GA synthesis (uniconazol) could increase disease susceptibility and resistance, respectively, to bacterial blight. Similarly, uniconazol restored disease resistance of the eui mutant and GA3 decreased disease resistance of the Eui overexpressors to bacterial blight. Therefore, the change of resistance attributes to GA levels. In consistency with this, the GA metabolism genes OsGA2Oox2 and OsGA2oxl were down-regulated during pathogen challenge. We also found that PR1a induction was enhanced but the SA level was decreased in the Eui overexpressor, while the JA level was reduced in the eui mutant. Together, our current study indicates that GAs play a negative role in rice basal disease resistance, with EUI as a positive modulator through regulating the level of bioactive GAs.

  9. Fahr disease with atypical presentation: A report of two cases

    Directory of Open Access Journals (Sweden)

    Suzan Tunç

    2010-05-01

    Full Text Available Fahr’s disease is a rare disorder where bilateral, almost symmetric, calcium and other mineral deposits occur in basal ganglia, cerebellar dentate nucleus and white matter. Common clinical findings of the disease are characterizing parkinsonism, dystonia, chorea, ataxia and psychiatric symptoms. Fahr’s disease is associated with various metabolic disorders especially with parathyroid disorders. In this article a 65 year old female patient with vision loss and headache, bilateral basal ganglia and cerebellar calsification on Computerized Tomography examination and a 45 year old female patient with convulsive state and bilateral caudat nucleus calcification on Computerized Tomography examination were reported.

  10. The recent prognosis after operation of hypertensive basal ganglia hemorrhage by using CT perfusion imaging%应用 CT 灌注成像预测基底节区高血压脑出血术后近期预后的研究

    Institute of Scientific and Technical Information of China (English)

    郑念东; 张道宝; 万晓强; 王山; 卫正洪

    2016-01-01

    目的:探讨CT灌注成像对基底节区高血压脑出血术后近期预后的评估作用。方法2012年6月至2013年9月高血压脑出血术后患者81例,术后两周行基底节区CT灌注成像,术后3月为患者行日常生活能力( ADL)分级评估,了解患者近期预后与术区局部脑血容量( rCBV)、局部脑血流量( rCBF)、平均通过时间( MTT)的相关性。结果 Logistic多元回归分析结果显示,rCBV、rCBF及MTT是影响患者预后的独立危险因素,患者近期预后与rCBV、rCBF呈正相关,与MTT呈负相关( P<0.05)。结论 CT灌注成像对预测患者近期预后有指导意义,患者的近期预后和术区周围脑组织的灌注状态密切相关,灌注越好,患者的近期预后越好。%Objective To investigate the evaluation effect of CT perfusion imaging on recent prognosis of hypertensive basal ganglia hemorrhage after operation .Methods Eighty-one patients with hypertensive basal ganglia hemorrhage after operation from June 2012 to September 2013 received CT perfusion imaging examination after 2 weeks of operation .Ability of daily life was estimated after 3 months of operation .The correlation between the recent prognosis and rCBV ,rCBF and MTT of operation area was explored .Results Logistic multivariate regression analysis showed that rCBV ,rCBF and MTT were independent risk factors of prognosis .The recent prog-nosis was positively correlated with rCBV and rCBF but negatively correlated with MTT ( P<0.05 ) .Conclusion The CT perfusion imaging has a guiding significance for recent prognosis of the patients .The recent prognosis was closely related to the brain tissue perfu-sion status in the operation area .The better perfusion the patients have ,they may have better recent prognosis .

  11. Proton MRS in Behcet's disease with and without neurological findings

    International Nuclear Information System (INIS)

    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.)

  12. 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

  13. 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

  14. [The role of the basal forebrain cholinergic dysfunction in pathogenesis of declarative memory disorder in Alzheimer's disease].

    Science.gov (United States)

    Mukhin, V N

    2013-06-01

    Alzheimer's disease is the most common cause of the declarative memory disorder: 30-40% cases of dementia among all of age groups, and 50-60% among the people older 65 years. In addition, disorder of declarative memory is the genuine symptom of the disease, which certainly appears on early stage of the disease and it is an obligate diagnostic symptom. Proponents of the "cholinergic theory" of pathogenesis of Alzheimer's disease suggest that the basis disorder of declarative memory is cholinergic dysfunction. Several neurodynamic mechanisms associated with declarative memory depend on the level of acetylcholine in hippocampus and neocortex. It is believed that dysfunction of the basal cholinergic system in Alzheimer's disease leads to the impairment of these mechanisms. In this review, we summarize available literature data concerning the mechanisms of Alzheimer's disease. PMID:24459876

  15. Mental Symptoms in Huntington's Disease and a Possible Primary Aminergic Neuron Lesion

    Science.gov (United States)

    Mann, J. John; Stanley, Michael; Gershon, Samuel; Rossor, M.

    1980-12-01

    Monoamine oxidase activity was higher in the cerebral cortex and basal ganglia of patients dying from Huntington's disease than in controls. Enzyme kinetics and multiple substrate studies indicated that the increased activity was due to elevated concentrations of monoamine oxidase type B. Concentrations of homovanillic acid were increased in the cerebral cortex but not in the basal ganglia of brains of patients with Huntington's disease. These changes may represent a primary aminergic lesion that could underlie some of the mental symptoms of this disease.

  16. Fahr's Disease Presenting with Dementia at Onset: A Case Report and Literature Review

    Science.gov (United States)

    Calabrò, Rocco Salvatore; Spadaro, Letteria; Marra, Angela; Bramanti, Placido

    2014-01-01

    Fahr's disease (FD) is characterized by sporadic or familiar idiopathic calcification of the basal ganglia, dentate nuclei of the cerebellum, and centrum semiovale, mainly presenting with movement disorder, dementia, and behavioral abnormalities. We described a rare case of Fahr's disease presenting at onset only with behavioral and neuropsychological alterations, whose diagnosis was supposed only after a brain CT, which showed extensive bilateral calcifications in the dentate nuclei of the cerebellum and basal ganglia. Since the onset of Fahr's disease may be a dysexecutive syndrome with behavioral abnormalities, the clinical and radiological features are really important to do the appropriate diagnosis. PMID:24803731

  17. Distribution of protoporphyrin IX in Bowen's disease and basal cell carcinomas treated with topical 5-aminolaevulinic acid

    Science.gov (United States)

    Roberts, David J.; Stables, G. I.; Ash, D. V.; Brown, Stanley B.

    1995-03-01

    We have used ultra-low light level fluorescence microscopy to examine the suggestion that the relatively poor response of human basal cell carcinomas (BCC) to topical 5-aminolaevulinic acid (ALA)-based photodynamic therapy (PDT) arises from limited drug penetration into the lesion. The distribution of ALA-induced protoporphyrin IX (PpIX) in human BCC and Bowen's disease was examined and, in almost all cases, was found to be most intense in those regions of tumor immediately adjacent to the dermis. This distribution was independent of tumor type, and did not appear to be affected by tumor depth in the skin. It is suggested that ALA penetration may not limit the efficacy of ALA-PDT in the treatment of BCC. Failure of superficial ALA-based PDT in basal cell carcinoma may, instead, be related to the histological structure of this type of lesion.

  18. 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.)

  19. 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…

  20. ESC-Derived Basal Forebrain Cholinergic Neurons Ameliorate the Cognitive Symptoms Associated with Alzheimer’s Disease in Mouse Models

    Directory of Open Access Journals (Sweden)

    Wei Yue

    2015-11-01

    Full Text Available Degeneration of basal forebrain cholinergic neurons (BFCNs is associated with cognitive impairments of Alzheimer’s disease (AD, implying that BFCNs hold potentials in exploring stem cell-based replacement therapy for AD. However, studies on derivation of BFCNs from embryonic stem cells (ESCs are limited, and the application of ESC-derived BFCNs remains to be determined. Here, we report on differentiation approaches for directing both mouse and human ESCs into mature BFCNs. These ESC-derived BFCNs exhibit features similar to those of their in vivo counterparts and acquire appropriate functional properties. After transplantation into the basal forebrain of AD model mice, ESC-derived BFCN progenitors predominantly differentiate into mature cholinergic neurons that functionally integrate into the endogenous basal forebrain cholinergic projection system. The AD mice grafted with mouse or human BFCNs exhibit improvements in learning and memory performances. Our findings suggest a promising perspective of ESC-derived BFCNs in the development of stem cell-based therapies for treatment of AD.

  1. 早期抗抑郁治疗对基底节区脑出血患者预后的影响研究%Effect of early anti-depression therapy on prognosis of patients with intracerebral hemorrhage in basal ganglia

    Institute of Scientific and Technical Information of China (English)

    吴丽华; 王怡雯; 郝磊; 田洪; 张玉波; 周虎传; 刘磊

    2015-01-01

    目的:探讨早期抗抑郁治疗对基底节区脑出血患者预后的影响。方法103例患者随机分为两组。对照组给予常规治疗和护理,实验组在对照组基础上给予早期抗抑郁治疗,包括早期心理干预和抗抑郁药物治疗。采用汉密尔顿抑郁量表评分(HAMD-17)、临床神经功能缺损评分(NFA)、简式Fugl-Meyer评分(FMA)及Barthel 指数BI评定,比较入院时和治疗后3个月的评分变化,评估患者抑郁状态、神经功能缺损、运动功能以及日常生活能力。结果治疗后3个月,与对照组比较,实验组HAMD评分、FMA评分、BI指数均优于对照组(P<0.01),两组NFA评分无显著差异(P>0.05)。结论早期抗抑郁治疗能够缓解基底节区脑出血患者的不良情绪,并能提高患者的预后和日常生活能力。%Objective To investigate the effect of early anti-depression therapy on the prognosis of patients with intracerebral hemorrhage in the basal ganglia .Methods 103 patients were randomly divided into 2 groups:anti-depression group and control group;routine treatment was provided to patients in both groups ,while anti-depression therapy was added to patients in anti-depression group , including early psychological intervention and anti-depression drugs;Hamilton Depression Scale ( HAMD-17 ) , Neurological Function Assessment(NFA),Fugl-Meyer Assessment(FMA),and Bathel Index(BI)were used to evaluated the conditions of patients in both groups and the changes of the scores at admission and 3 months after treatment were comparatively analyzed , the evaluation of depression,neurological deficits,motor function and activities of daily living was made .Results 3 months after treatment,the scores of HAMD,FMA and BI of the patients in anti-depression group were superior to those of the patients in control group (P0.05).Conclusion Early anti-depression therapy can relieve the unhealthy mood of the patients with

  2. 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).

  3. 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…

  4. Elevated basal intestinal mucosal cytokine levels in asymptomatic first-degree relatives of patients with Crohn's disease

    Institute of Scientific and Technical Information of China (English)

    Anant VK Indaram; Santa Nandi; Sam Weissman; Sing Lam; Beverly Bailey; Meyer Blumstein; Ronald Greenberg; Simmy Bank

    2000-01-01

    AIM To determine levels of cytokines in colonic mucosa of asymptomatic first degree relatives of Crohn's disease patients.METHODS Cytokines (Interleukin (IL) 1-Beta,IL-2, IL-6 and IL-8) were measured using ELISA in biopsy samples of normal looking colonic mucosa of first degree relatives of Crohn's disease patients (n = 9) and from normal controls (n=10) with no family history of Crohn's disease.RESULTS Asymptomatic first degree relatives of patients with Crohn's disease had significantly higher levels of basal intestinal mucosal cytokines (IL-2, IL-6 and IL-8) than normal controls. Whether these increased cytokine levels serve as phenotypic markers for a genetic predisposition to developing Crohn's disease later on, or whether they indicate early (preclinical) damage has yet to be further defined.CONCLUSION Asymptomatic first degree relatives of Crohn's disease patients have higher levels of cytokines in their normal-looking intestinal mucosa compared to normal controls.This supports the hypothesis that increased cytokines may be a cause or an early event in the inflammatory cascade of Crohn's disease and are not merely a result of the inflammatory process.

  5. Reduced brain perfusion in basal forebrain associated with cognitive decline in Alzheimer's diseases: a Tc-99m HMPAO SPECT study

    International Nuclear Information System (INIS)

    Aim: Reduction of regional cerebral blood flow (rCBF) in various cerebral regions and decline of cognitive function have been reported in Alzheimer's disease (AD) patients. The aim of this study was to identify the brain areas showing correlation between longitudinal changes of rCBFs and decline of general mental function, measured by Mini-Mental State Examination (MMSE) in probable Alzheimer's disease patients. Materials and Methods: Nine probable AD patients according to NINCDS-ADRDA criteria and DSM-IV were studied with Tc-99m HMPAO SPECT at an initial point and at the follow-up after a period of average 1.8 year. MMSE score was obtained in both occasions (average MMSE 16.4 at initial study; average MMSE = 8.1 at follow-up). Single SPECT was performed in 30 age-matched normal controls. Each SPECT image was normalized to the cerebellar activity. Using statistical parametric mapping (SPM99), correlation was analyzed between individual changes in rCBF of two SPECT scans and the MMSE scores at the time of each study in AD patients. In addition, the SPECT images of the initial study and the follow-up study were compared with SPECT images of the age-matched normal group respectively. Results: Significant correlation between longitudinal changes of rCBFs and MMSE scores was found in left basal forebrain region including substantia innominata (x, y, z = -24, 16, -23; P < .05, corrected). Within a short follow-up period of 1.8 years, cerebral hypoperfusion extended to various cortical regions from bilateral temporo-parietal to bilateral frontal regions and cingulate cortex, compared to normal controls. Conclusion: The decline of cognitive function in individual AD patients was correlated with rCBF reduction in left basal forebrain. This finding supports the cholinergic hypothesis of AD since hypoperfusion in basal forebrain region might indicate deterioration of cholinergic neurons in nucleus basalis of Meynert or substantia innominata

  6. Reduced basal autophagy and impaired mitochondrial dynamics due to loss of Parkinson's disease-associated protein DJ-1.

    Directory of Open Access Journals (Sweden)

    Guido Krebiehl

    Full Text Available BACKGROUND: Mitochondrial dysfunction and degradation takes a central role in current paradigms of neurodegeneration in Parkinson's disease (PD. Loss of DJ-1 function is a rare cause of familial PD. Although a critical role of DJ-1 in oxidative stress response and mitochondrial function has been recognized, the effects on mitochondrial dynamics and downstream consequences remain to be determined. METHODOLOGY/PRINCIPAL FINDINGS: Using DJ-1 loss of function cellular models from knockout (KO mice and human carriers of the E64D mutation in the DJ-1 gene we define a novel role of DJ-1 in the integrity of both cellular organelles, mitochondria and lysosomes. We show that loss of DJ-1 caused impaired mitochondrial respiration, increased intramitochondrial reactive oxygen species, reduced mitochondrial membrane potential and characteristic alterations of mitochondrial shape as shown by quantitative morphology. Importantly, ultrastructural imaging and subsequent detailed lysosomal activity analyses revealed reduced basal autophagic degradation and the accumulation of defective mitochondria in DJ-1 KO cells, that was linked with decreased levels of phospho-activated ERK2. CONCLUSIONS/SIGNIFICANCE: We show that loss of DJ-1 leads to impaired autophagy and accumulation of dysfunctional mitochondria that under physiological conditions would be compensated via lysosomal clearance. Our study provides evidence for a critical role of DJ-1 in mitochondrial homeostasis by connecting basal autophagy and mitochondrial integrity in Parkinson's disease.

  7. Volumetric changes in the Basal Ganglia after antipsychotic monotherapy

    DEFF Research Database (Denmark)

    Ebdrup, B H; Nørbak, H; Borgwardt, S;

    2013-01-01

    monotherapy. Material and Methods: We systematically searched PubMed for longitudinal MRI studies of patients with schizophrenia or non-affective psychosis who had undergone a period of antipsychotic monotherapy. We used specific, predefined search terms and extracted studies were hand searched for additional...... 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....... Unexpectedly, no studies found that specific FGAs induce significant BG volume increases. Conversely, both volumetric increases and decreases in the BG have been associated with SGA monotherapy. Discussion: Induction of striatal volume increases is not a specific feature of FGAs. Except for clozapine treatment...

  8. 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.

  9. Fahr's disease: current perspectives

    Directory of Open Access Journals (Sweden)

    Tai XY

    2015-07-01

    Full Text Available Xin You Tai, Amit BatlaUCL Institute of Neurology, Queen Square, London, UKAbstract: Based on original descriptions of brain calcification by Theodor Fahr, brain calcification, and more specifically basal ganglia calcification, is referred to as Fahr's syndrome. Recent identification of genetic mutations has concerted the description of this erstwhile heterogeneous condition which we refer to here as Fahr's disease. Fahr's disease refers to idiopathic calcification of the basal ganglia without a secondary (non-genetic cause. Idiopathic basal ganglia calcification (IBGC is another term, which offers a more accurate description of this condition. Within the last 2 years, genetic mutations for IBGC have been described in SLC20A2, PDGFB, and PDGFRB. These findings broaden our understanding of the pathophysiology and encourage a search for specific treatment options in this rare but disabling condition. Clinically, parkinsonism, dystonia, and other movement disorders are the most common clinical features, but psychiatric features can predominate. Cases with confirmed genetic mutations reveal new clinical features linked with Fahr's disease, such as headaches. Computed tomography or magnetic resonance imaging is usually the trigger for suspecting Fahr's disease and important for identifying the calcification pattern. Symmetric calcification of the globus pallidus, thalamus, and dentate nucleus is the most common pattern in IBGC, but other parts of the brain such as the cerebellum are involved. Treatment of Fahr's disease is currently limited and is largely symptomatic. A better understanding of this condition in light of genetic findings is important to improve the clinical diagnosis and develop specific treatment options.Keywords: Fahr's disease, basal ganglia calcification, parkinsonism, calcium

  10. 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

  11. 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).

  12. A family of conserved bacterial effectors inhibits salicylic acid-mediated basal immunity and promotes disease necrosis in plants.

    Science.gov (United States)

    DebRoy, Sruti; Thilmony, Roger; Kwack, Yong-Bum; Nomura, Kinya; He, Sheng Yang

    2004-06-29

    Salicylic acid (SA)-mediated host immunity plays a central role in combating microbial pathogens in plants. Inactivation of SA-mediated immunity, therefore, would be a critical step in the evolution of a successful plant pathogen. It is known that mutations in conserved effector loci (CEL) in the plant pathogens Pseudomonas syringae (the Delta CEL mutation), Erwinia amylovora (the dspA/E mutation), and Pantoea stewartii subsp. stewartii (the wtsE mutation) exert particularly strong negative effects on bacterial virulence in their host plants by unknown mechanisms. We found that the loss of virulence in Delta CEL and dspA/E mutants was linked to their inability to suppress cell wall-based defenses and to cause normal disease necrosis in Arabidopsis and apple host plants. The Delta CEL mutant activated SA-dependent callose deposition in wild-type Arabidopsis but failed to elicit high levels of callose-associated defense in Arabidopsis plants blocked in SA accumulation or synthesis. This mutant also multiplied more aggressively in SA-deficient plants than in wild-type plants. The hopPtoM and avrE genes in the CEL of P. syringae were found to encode suppressors of this SA-dependent basal defense. The widespread conservation of the HopPtoM and AvrE families of effectors in various bacteria suggests that suppression of SA-dependent basal immunity and promotion of host cell death are important virulence strategies for bacterial infection of plants. PMID:15210989

  13. 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.)

  14. Fahr's disease: a rare neurological presentation in a tropical setting

    Science.gov (United States)

    Otu, Akaninyene Asuquo; Anikwe, Jude Chinedu; Cocker, Derek

    2015-01-01

    Key Clinical Message While rare, Fahr's disease should be considered as a differential diagnosis for seizures, movement disorders, or cognitive impairment in tropical settings. Classically, bilateral calcification of the basal ganglia is seen on CT. Endemic infections, metabolic, and toxic causes should be excluded. Treatment using Levodopa is often beneficial. PMID:26509011

  15. Multiple skin cancers in a single patient: Multiple pigmented Bowen′s disease, giant basal cell carcinoma, squamous cell carcinoma

    Directory of Open Access Journals (Sweden)

    Ravi Saini

    2015-01-01

    Full Text Available Basal cell carcinoma (BCC and squamous cell carcinoma are the most common type of nonmelanoma skin cancers (NMSCs. Bowen′s disease (BD, a premalignant condition, has a marginal potential (3-5% to progress to invasive carcinoma. We report here a rarest of a rare case of multiple pigmented BD with overlying squamous cell cancer along with a giant neglected BCC on the scalp of a 76-year-old man. The occurrence of multiple BD and NMSC in a single patient compelled us to explore the following hypothesis: (1 The multiple precancerous and cancerous lesions can be due to common etiopathogenesis. Chronic ultraviolet exposure, immunosupresssion, human papillomavirus infection, dietary factors, and environmental factors including arsenic exposure were probed in to. (2 There is evolution of precancerous lesions into a different type of cancers in different time frame. (3 The new cancerous lesions are subsequent cancers that developed after neglected untreated primary cancer.

  16. 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 exam

  17. Micrographia in Parkinson's disease.

    Science.gov (United States)

    Contreras-Vidal, J L; Teulings, H L; Stelmach, G E

    1995-10-23

    A computational neural model of movement production in normal and Parkinson's disease (PD) is used to provide a neural account for the source of micrographia in PD handwriting. It is hypothesized that smaller than normal pallido-thalamic signals, due to dopamine depletion, are responsible for the observed overall smallness, slowness and variability in PD handwriting. Experimental data from PD patients that show micrographia support this hypothesis and imply the functional segregation of basal ganglia neural populations. PMID:8580447

  18. 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…

  19. Loss of dopamine phenotype among midbrain neurons in Lesch-Nyhan disease

    NARCIS (Netherlands)

    Gottle, M.; Prudente, C.N.; Fu, R.; Sutcliffe, D.; Pang, H.; Cooper, D.; Veledar, E.; Glass, J.D.; Gearing, M.; Visser, J.E.; Jinnah, H.A.

    2014-01-01

    OBJECTIVE: Lesch-Nyhan disease (LND) is caused by congenital deficiency of the purine recycling enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGprt). Affected patients have a peculiar neurobehavioral syndrome linked with reductions of dopamine in the basal ganglia. The purpose of the curre

  20. Stimulation of the subthalamic region facilitates the selection and inhibition of motor responses in Parkinson's disease

    NARCIS (Netherlands)

    W.P.M. van den Wildenberg; G.J.M. van Boxtel; M.W. van der Molen; D.A. Bosch; J.D. Speelman; C.H.M. Brunia

    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

  1. 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 (rCBF) an...

  2. The Treatment of Intracranial Hematoma Drainage by Drilling Cranium at the Frontal Region for Basal Ganglia Hemorrhage in 109 Cases%额部钻颅血肿引流术治疗高血压基底节区脑出血109例

    Institute of Scientific and Technical Information of China (English)

    罗德群

    2012-01-01

    目的 探索一种新的手术方法治疗高血压脑出血,既能避开重要功能区及重要血管分布区,又能达到与其他穿刺术式引流效果相仿. 方法 回顾性分析我科2010年8月至2011年10月行新术式穿刺的高血压脑出血患者109例,所有患者均经头颅CT确诊,根据多田公式计算出血量,分别采用局麻或全麻下额部钻颅软通道引流血肿78例,额部钻颅血肿引流配合脑室外引流20例,单纯行脑室外引流术11例. 结果 术后2~5d复查头颅CT示:血肿完全清除19例;血肿清除90%以上37例,血肿清除80%以上43例,清除50%以上8例,血肿清除小于50%2例;未发生切口及颅内感染;术后死亡6例. 结论 额部钻颅血肿引流术治疗高血压基底节区脑出血能有效避免对重要脑组织的损伤,减少手术并发症;血肿清除率高,提高了患者的生存率和生存质量.%Objective To explore a new operative method for the treatment of hypertensive cerebral hemorrhage which can avoid the damage to the functional area and important vascular area while also can a-chieve the same therapeutic equivalence as traditional operations. Methods The clinical results were retrospectively analyzed in 109 cases of hypertensive cerebral hemorrhage underwent the new operative method in our department from August, 2010 to October, 2011. All the cases were confirmed by CT scam as the basal ganglia hemorrhage. Intracranial hematoma drainage by drilling cranium at the frontal region was done for all the cases. Results Two to five days after operation, the intracranial hematoma was completely disappeared in 19 patients, 90% above cleared in 37 patients, 80% above cleared in 43 patients, 50% above cleared in 8 patients and less than 50% decreased in 2 patients. Neither intracranial infection nor wound infection was observed. Six patients died after the operation. Conclusion It can effectively avoid the damage to the important brain tissue, decrease complications

  3. Comparative histochemical study of Bowen’s disease and actinic keratosis: preserved normal basal cells in Bowen’s disease

    OpenAIRE

    Ishida, H; Kumakiri, M.; Ueda, K.; LM Lao; M Yanagihara; Asamoto, K.; Imamura, Y; S Noriki; Fukuda, M

    2009-01-01

    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 ...

  4. Leigh’s Disease: A case Report

    Directory of Open Access Journals (Sweden)

    Nikhil Verma

    2014-12-01

    Full Text Available Leigh disease is a progressive degenerative, mitochondrial disorder of childhood with most cases become apparent during infancy. In most cases it presents as a progressive neurological disease with motor and intellectual developmental delay, developmental regression and signs and symptoms of brain stem and/or basal ganglia involvement. Raised lactate levels in blood and/or cerebrospinal fluid is noted. It is the neuro imaging, mainly the Magnetic Resonance Imaging showing characteristic symmetrical necrotic lesions in the basal ganglia and/or brain stem that leads to the diagnosis. Here, we report a case of 3years old male child presenting to us with status epilepticus, delayed developmental milestones and regression of the achieved milestones suspected to be a case of neurodegenerative disorder, which on MRI was diagnosed as Leigh’s disease.

  5. Frontal Cognitive Function and Memory in Parkinson’s Disease: Toward a Distinction between Prospective and Declarative Memory Impairments?

    OpenAIRE

    Tröster, A. I.; Fields, J. A.

    1995-01-01

    Memory dysfunction is a frequent concomitant of Parkinson's disease (PD). Historically, two classes of hypotheses, focusing on different cognitive mechanisms, have been advanced to explain this memory impairment: one postulating retrieval deficits (common to several neurodegenerative disorders involving the basal ganglia), and the other postulating frontally mediated executive deficits as fundamental to memory impairment. After outlining empirical support for the retrieval deficit hypothesis,...

  6. Frequency-selectivity of a thalamocortical relay neuron during Parkinson's disease and deep brain stimulation: a computational study

    NARCIS (Netherlands)

    H. Cagnan; H.G.E. Meijer; S.A. van Gils; M. Krupa; T. Heida; M. Rudolph; W.J. Wadman; H.C.F. Martens

    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 neu

  7. EFFECT OF MICRONUTRIENTS-ENRICHED FERTILIZERS ON BASAL STEM ROT DISEASE INCIDENCE AND SEVERITY ON OIL PALM (ELAEIS GUINEENSIS JACQ.) SEEDLINGS

    OpenAIRE

    Fabien Fonguimgo Tengoua; Hanafi, Mohamed M.; A. S. Idris; Kadir Jugah; Jamaludin Nurul Mayziatul Azwa; Mohidin Hasmah; Syed Rastan Syed-Omar

    2014-01-01

    Basal stem rot caused by Ganoderma boninense constitutes a serious threat to oil palm industry in Southeast Asia, especially in Malaysia and Indonesia and in Papua New Guinea and Pacific Islands. It is also expanding in some oil palm growing countries in Latin America and Africa and will soon become a worldwide concern to oil palm cultivation. To date, none of the various control measures developed and tested to control the disease since many decade gives entire satisfactio...

  8. Evaluation of Basal Serum Adrenocorticotropic Hormone and Cortisol Levels and Their Relationship with Nonalcoholic Fatty Liver Disease in Male Patients with Idiopathic Hypogonadotropic Hypogonadism

    Institute of Scientific and Technical Information of China (English)

    Wen-Bo Wang; Fei She; Li-Fang Xie; Wen-Hua Yan; Jin-Zhi Ouyang; Bao-An Wang; Hang-Yun Ma

    2016-01-01

    Background: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.Methods: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.Results: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.Conclusions:The male IHH patients showed higher basal serum ACTH levels and lower cortisol levels than matched healthy controls.NAFLD was

  9. Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin.

    NARCIS (Netherlands)

    Berghe, P.V. van den; Stapelbroek, J.M.; Krieger, E.; Bie, P. de; Graaf, S.F. van de; Groot, R.E. de; Beurden, E. van; Spijker, E.; Houwen, R.H.; Berger, R.; Klomp, L.W.

    2009-01-01

    Wilson disease (WD) is an autosomal recessive copper overload disorder of the liver and basal ganglia. WD is caused by mutations in the gene encoding ATP7B, a protein localized to the trans-Golgi network that primarily facilitates hepatic copper excretion. Current treatment comprises reduction of ci

  10. [Basal cell carcinoma of the skin--biological behaviour of the tumor and a review of the most important molecular predictors of disease progression in pathological practice].

    Science.gov (United States)

    Bartos, V; Adamicová, K; Kullová, M; Péc, M

    2011-01-01

    Basal cell carcinoma of the skin is currently the most frequent malignancy in human population. Basal cell carcinoma represents a heterogeneous group of tumors with a variable clinical and morphological picture. Based on its biological behaviour, we generally differentiate between indolent (superficial and nodular) and aggressive type (infiltrative, micronodular, and metatypical) of basal cell carcinoma. Because of the different biological characteristics of these tumors, it is questionable whether they are a part of a continuous spectrum of carcinogenesis, starting with indolent and ending with aggressive forms, or they represent separate developmental lines. In the current clinical practice, there is an increasing demand for identification of tumors that are prognostically more adverse and their impact on the overall health status of patients is more serious. Recent advances in pathology and molecular medicine allow identification of various biomarkers from tumor tissue that are significantly involved in the mechanisms of malignant cell transformation. Detection of these biomarkers is of great importance in predicting further clinical behaviour of the cancer. The authors of the paper present basic information about biological behaviour of cutaneous basal cell carcinoma and provide an overview of the most important biomarkers that influence the clinical outcome and disease progression and are detectable through a routine biopsy tissue examination. It is now necessary to search for novel histological and molecular parameters that, in the future, could have a prognostic value in diagnostic and therapeutic process of this disorder. PMID:21542271

  11. Category and Perceptual Learning in Subjects with Treated Wilson's Disease

    OpenAIRE

    Xu, Pengjing; Lu, Zhong-Lin; Wang, Xiaoping; Dosher, Barbara; Zhou, Jiangning; Zhang, Daren; Zhou, Yifeng

    2010-01-01

    To explore the relationship between category and perceptual learning, we examined both category and perceptual learning in patients with treated Wilson's disease (WD), whose basal ganglia, known to be important in category learning, were damaged by the disease. We measured their learning rate and accuracy in rule-based and information-integration category learning, and magnitudes of perceptual learning in a wide range of external noise conditions, and compared the results with those of normal...

  12. Skeletal muscle pathology in Huntington's disease

    OpenAIRE

    Zielonka, Daniel; Piotrowska, Izabela; Marcinkowski, Jerzy T.; Mielcarek, Michal

    2014-01-01

    Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by the expansion of a polyglutamine stretch within the huntingtin protein (HTT). The neurological symptoms, that involve motor, cognitive and psychiatric disturbances, are caused by neurodegeneration that is particularly widespread in the basal ganglia and cereberal cortex. HTT is ubiquitously expressed and in recent years it has become apparent that HD patients experience a wide array of peripheral organ dysfunction ...

  13. 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.

  14. 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.

  15. TGF-β1 induces an age-dependent inflammation of nerve ganglia and fibroplasia in the prostate gland stroma of a novel transgenic mouse.

    Directory of Open Access Journals (Sweden)

    David A Barron

    Full Text Available TGF-β1 is overexpressed in wound repair and in most proliferative disorders including benign prostatic hyperplasia and prostate cancer. The stromal microenvironment at these sites is reactive and typified by altered phenotype, matrix deposition, inflammatory responses, and alterations in nerve density and biology. TGF-β1 is known to modulate several stromal responses; however there are few transgenic models to study its integrated biology. To address the actions of TGF-β1 in prostate disorders, we targeted expression of an epitope tagged and constitutively active TGF-β1 via the enhanced probasin promoter to the murine prostate gland epithelium. Transgenic mice developed age-dependent lesions leading to severe, yet focal attenuation of epithelium, and a discontinuous basal lamina. These changes were associated with elevated fibroplasia and frequency of collagenous micronodules in collapsed acini, along with an induced inflammation in nerve ganglia and small vessels. Elevated recruitment of CD115+ myeloid cells but not mature macrophages was observed in nerve ganglia, also in an age-dependent manner. Similar phenotypic changes were observed using a human prostate epithelium tissue recombination xenograft model, where epithelial cells engineered to overexpress TGF-β1 induced fibrosis and altered matrix deposition concurrent with inflammation in the stromal compartment. Together, these data suggest that elevated TGF-β1 expression induces a fibroplasia stromal response associated with breach of epithelial wall structure and inflammatory involvement of nerve ganglia and vessels. The novel findings of ganglia and vessel inflammation associated with formation of collagenous micronodules in collapsed acini is important as each of these are observed in human prostate carcinoma and may play a role in disease progression.

  16. Conditional Routing of Information to the Cortex: A Model of the Basal Ganglia’s Role in Cognitive Coordination

    OpenAIRE

    Stocco, Andrea; Lebiere, Christian; Anderson, John R.

    2010-01-01

    The basal ganglia play a central role in cognition and are involved in such general functions as action selection and reinforcement learning. Here, we present a model exploring the hypothesis that the basal ganglia implement a conditional information-routing system. The system directs the transmission of cortical signals between pairs of regions by manipulating separately the selection of sources and destinations of information transfers. We suggest that such a mechanism provides an account f...

  17. 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

    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...... fifty years andtoday, neuroscience reveals promising newstrategies to deal with disorders of thenervous system.Some of these results have been implementedwith success in the treatment of Parkinson'sdisease, a common neurodegenerative illnessaffecting approximately 1% of the populationaged seventy...... 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...

  18. Assessment of Cerebral Hemodynamic Changes in Pediatric Patients with Moyamoya Disease Using Probabilistic Maps on Analysis of Basal/Acetazolamide Stress Brain Perfusion SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ho Young; Lee, Jae Sung; Kim, Seung Ki; Wang, Kyu Chang; Cho, Byung Kyu; Chung, June Key; Lee, Myung Chul; Lee, Dong Soo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2008-06-15

    To evaluate the hemodynamic changes and the predictive factors of the clinical outcome in pediatric patients with moyamoya disease, we analyzed pre/post basal/acetazolamide stress brain perfusion SPECT with automated volume of interest (VOIs) method. Total fifty six (M:F=33:24, age 6.7{+-}3.2 years) pediatric patients with moyamoya disease, who underwent basal/acetazolamide stress brain perfusion SPECT within 6 before and after revascularization surgery (encephalo-duro-arterio-synangiosis (EDAS) with frontal encephalo-galeo-synangiosis (EGS) and EDAS only followed on contralateral hemisphere), and followed-up more than 6 months after post-operative SPECT, were included. A mean follow-up period after post-operative SPECT was 33{+-}21 months. Each patient's SPECT image was spatially normalized to Korean template with the SPM2. For the regional count normalization, the count of pons was used as a reference region. The basal/acetazolamide-stressed cerebral blood flow (CBF), the cerebral vascular reserve index (CVRI), and the extent of area with significantly decreased basal/acetazolamide- stressed rCBF than age-matched normal control were evaluated on both medial frontal, frontal, parietal, occipital lobes, and whole brain in each patient's images. The post-operative clinical outcome was assigned as good, poor according to the presence of transient ischemic attacks and/or fixed neurological deficits by pediatric neurosurgeon. In a paired t-test, basal/acetazolamide-stressed rCBF and the CVRI were significantly improved after revascularization (p<0.05). The significant difference in the pre-operative basal/acetazolamide-stressed rCBF and the CVRI between the hemispheres where EDAS with frontal EGS was performed and their contralateral counterparts where EDAS only was done disappeared after operation (p<0.05). In an independent student t-test, the pre-operative basal rCBF in the medial frontal gyrus, the post-operative CVRI in the frontal lobe and the parietal

  19. Application of arbuscular mycorrhizal fungi with Pseudomonas aeruginosa UPMP3 reduces the development of Ganoderma basal stem rot disease in oil palm seedlings.

    Science.gov (United States)

    Sundram, Shamala; Meon, Sariah; Seman, Idris Abu; Othman, Radziah

    2015-07-01

    The effect of arbuscular mycorrhizal fungi (AMF) in combination with endophytic bacteria (EB) in reducing development of basal stem rot (BSR) disease in oil palm (Elaeis guineensis) was investigated. BSR caused by Ganoderma boninense leads to devastating economic loss and the oil palm industry is struggling to control the disease. The application of two AMF with two EB as biocontrol agents was assessed in the nursery and subsequently, repeated in the field using bait seedlings. Seedlings pre-inoculated with a combination of Glomus intraradices UT126, Glomus clarum BR152B and Pseudomonas aeruginosa UPMP3 significantly reduced disease development measured as the area under disease progression curve (AUDPC) and the epidemic rate (R L) of disease in the nursery. A 20-month field trial using similar treatments evaluated disease development in bait seedlings based on the rotting area/advancement assessed in cross-sections of the seedling base. Data show that application of Glomus intraradices UT126 singly reduced disease development of BSR, but that combination of the two AMF with P. aeruginosa UPMP3 significantly improved biocontrol efficacy in both nursery and fields reducing BSR disease to 57 and 80%, respectively. The successful use of bait seedlings in the natural environment to study BSR development represents a promising alternative to nursery trial testing in the field with shorter temporal assessment.

  20. 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.

  1. 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.

  2. Huntington's disease: effect of cysteamine, a somatostatin-depleting agent.

    Science.gov (United States)

    Shults, C; Steardo, L; Barone, P; Mohr, E; Juncos, J; Serrati, C; Fedio, P; Tamminga, C A; Chase, T N

    1986-08-01

    Somatostatin levels in the basal ganglia are elevated in Huntington's disease. A controlled therapeutic trial of the somatostatin-depleting agent, cysteamine, was therefore conducted in five patients, including one with the rigid-akinetic form. Maximum tolerated dosage for 2 weeks produced no consistent change in extrapyramidal or dementia scores. Somatostatin concentrations were not significantly altered in plasma or CSF. Growth hormone levels, on the other hand, more than doubled, suggesting a functionally significant decrease in central somatostatin levels. PMID:2874527

  3. Skeletal muscle pathology in Huntington’s Disease.

    OpenAIRE

    Daniel eZielonka; Izabela ePiotrowska; Marcinkowski, Jerzy T.; Michal eMielcarek

    2014-01-01

    Huntington’s disease (HD) is a hereditary neurodegenerative disorder caused by the expansion of a polyglutamine stretch within the huntingtin protein (HTT). The neurological symptoms, that involve motor, cognitive and psychiatric disturbances, are caused by neurodegeneration that is particularly widespread in the basal ganglia and cereberal cortex. HTT is ubiquitously expressed and in recent years it has become apparent that HD patients experience a wide array of peripheral organ dysfunction ...

  4. 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.

  5. Basal Cell Carcinoma (BCC)

    Science.gov (United States)

    ... epithelioma, is the most common form of skin cancer. Basal cell carcinoma usually occurs on sun-damaged skin, especially ... other health issues. Infiltrating or morpheaform basal cell carcinomas: Infiltrating basal cell carcinomas can be more aggressive and locally destructive ...

  6. Neuroaxonal dystrophy in aging human sympathetic ganglia.

    OpenAIRE

    Schmidt, R.E.; Chae, H. Y.; Parvin, C. A.; Roth, K A

    1990-01-01

    Autonomic dysfunction is an increasingly recognized problem in aging animals and man. The pathologic changes that produce autonomic dysfunction in human aging are largely unknown; however, in experimental animal models specific pathologic changes have been found in selected sympathetic ganglia. To address whether similar neuropathologic changes occur in aging humans, the authors have examined paravertebral and prevertebral sympathetic ganglia from a series of 56 adult autopsied nondiabetic pa...

  7. Noopept efficiency in experimental Alzheimer disease (cognitive deficiency caused by beta-amyloid25-35 injection into Meynert basal nuclei of rats).

    Science.gov (United States)

    Ostrovskaya, R U; Belnik, A P; Storozheva, Z I

    2008-07-01

    Experiments on adult Wistar rats showed that injection of beta-amyloid25-35 (2 microg) into Meynert basal nuclei caused long-term memory deficiency which was detected 24 days after this injection by the memory trace retrieval in conditioned passive avoidance reflex (CPAR). The effects of noopept, an original nootropic and neuroprotective dipeptide, on the severity of this cognitive deficiency were studied. Preventive (for 7 days before the injury) intraperitoneal injections of noopept in a dose of 0.5 mg/kg completely prevented mnestic disorders under conditions of this model. Noopept exhibited a significant normalizing effect, if the treatment was started 15 days after the injury, when neurodegenerative changes in the basal nuclei, cortex, and hippocampus were still acutely pronounced. The mechanisms of this effect of the drug are studied, including, in addition to the choline-positive effect, its multicomponent neuroprotective effect and stimulation of production of antibodies to beta-amyloid25-35. Noopept efficiency in many models of Alzheimer disease, its high bioavailability and low toxicity suggest this dipeptide for further studies as a potential agent for the treatment of this condition (initial and moderate phases). PMID:19145356

  8. Hibernation model of tau phosphorylation in hamsters : selective vulnerability of cholinergic basal forebrain neurons - implications for Alzheimer's disease

    NARCIS (Netherlands)

    Haertig, Wolfgang; Stieler, Jens; Boerema, Ate S.; Wolf, Jennifer; Schmidt, Udo; Weissfuss, Jana; Bullmann, Torsten; Strijkstra, Arjen M.; Arendt, Thomas

    2007-01-01

    Neurofibrillar tangles made up of 'paired helical filaments' (PHFs) consisting of hyperphosphorylated microtubule-associated protein tau are major hallmarks of Alzheimer's disease (AD). Tangle formation selectively affects certain neuronal types and systematically progresses throughout numerous brai

  9. Determination and mapping of calcium and magnesium contents using geostatistical techniques in oil palm plantation related to basal stem rot disease

    Directory of Open Access Journals (Sweden)

    Nur Shuhada Tajudin

    2016-02-01

    Full Text Available The basal stem rot (BSR disease has been reported as the most destructive disease of oil palms in Southeast Asia. Adequate contents of nutrient in soil and leaf helps improve the plant health and its productivity. This study aims to determine the spatial variability of calcium (Ca and magnesium (Mg in soil and leaf collected in BSR infected oil palm plantation. The exchangeable calcium (Ca ex and magnesium (Mgex in soil were found low in the study area ranged from 0.03-0.50% and 0.06- 0.35%, respectively. The Ca and Mg content in leaf were also low ranged from 0.09-0.60%, and 0.03-1.87%, respectively. The Ca ex in soil of both blocks showed a negative significant correlation with the disease at p<0.01. However, only Ca content in leaves of one study site (Block 2 showed a negative significant correlations with the disease (p<0.05. The generated map and significant correlations revealed that unbalanced nutrient content occurred in the study area.

  10. Diffusion-weighted imaging and fluid-attenuated inversion recovery sequence in sporadic Creutzfeldt-Jakob disease One-case report

    Institute of Scientific and Technical Information of China (English)

    Rosa Morabito; Placido Bramanti; Silvia Marino; Annalisa Baglieri; Rosella Ciurleo; Francesco Corallo; Rosaria De Luca; Simona De Salvo; Silvia Guerrera; Francesca Timpano; Maria Adele Marino

    2011-01-01

    The diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) is extremely difficult. Diffusion-weighted imaging has been shown to be the most sensitive technique for the detection of signal alterations in sCJD patients. The present study analyzed the diagnostic value of diffusion-weighted imaging and fluid-attenuated inversion recovery sequence in the early stage of sCJD in one female patient and correlated the clinical symptoms during disease course and magnetic resonance manifestations. Thalamic and basal ganglia hyperintensities were observed on magnetic resonance images in a very early stage, i.e., when the clinical typical manifestations of the disease were not present. With the progression of the disease, cortical and basal ganglia hyperintensities were observed on magnetic resonance images, showing an obvious cerebral atrophy. These findings suggest that diffusion-weighted imaging and fluid-attenuated inversion recovery sequence are helpful in diagnosing sCJD.

  11. 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.).

  12. 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

  13. Dysfunctional Sensory Modalities, Locus Coeruleus, and Basal Forebrain: Early Determinants that Promote Neuropathogenesis of Cognitive and Memory Decline and Alzheimer's Disease.

    Science.gov (United States)

    Daulatzai, Mak Adam

    2016-10-01

    Sporadic Alzheimer's disease (AD) is a devastating neurodegenerative disorder. It is essential to unravel its etiology and pathogenesis. This should enable us to study the presymptomatic stages of the disease and to analyze and reverse the antemortem behavioral, memory, and cognitive dysfunction. Prima facie, an ongoing chronic vulnerability involving neural insult may lead normal elderly to mild cognitive impairment (MCI) and then to AD. Development of effective preventive and therapeutic strategies to thwart the disease pathology obviously requires a thorough delineation of underlying disruptive neuropathological processes. Our sensory capacity for touch, smell, taste, hearing, and vision declines with advancing age. Declines in different sensory attributes are considered here to be the primary "first-tier pathologies." Olfactory loss is among the first clinical signs of neurodegenerative diseases including AD and Parkinson's disease (PD). Sensory dysfunction in the aged promotes pathological disturbances in the locus coeruleus, basal forebrain, entorhinal cortex, hippocampus, and several key areas of neocortex and brainstem. Hence, sensory dysfunction is the pivotal factor that may upregulate cognitive and memory dysfunction. The age-related constellation of comorbid pathological factors may include apolipoprotein E (APOE) genotype, obesity, diabetes, hypertension, alcohol abuse, head trauma, and obstructive sleep apnea. The concepts and trajectories delineated here are the dynamic pillars of the current hypothesis presented-it postulates that the sensory decline, in conjunction with the above pathologies, is crucial in triggering neurodegeneration and promoting cognitive/memory dysfunction in aging and AD. The application of this thesis can be important in formulating new multifactorial preventive and treatment strategies (suggested here) in order to attenuate cognitive and memory decline and ameliorate pathological dysfunction in aging, MCI, and AD. PMID

  14. Dopamine Does Not Appear to Affect Mental Rotation in Parkinson’s Disease

    OpenAIRE

    Crucian, Gregory P.; Sheyan Armaghani; Avan Armaghani; Foster, Paul S.; Burks, David W.; Barry Skoblar; Valeria Drago; Heilman, Kenneth M.

    2014-01-01

    Objective Patients with Parkinson’s disease (PD) often have deficits with mental rotation (MR). The neuropathological factors underlying these deficits, however, remain to be elucidated. One hypothesis suggests that dopamine depletion in nigro-striatal systems adversely influences MR. Another hypothesis suggests that deterioration of cortical (fronto-temporo-parietal basal ganglia) networks that mediate this function are responsible for this deficit. The goal of this study was to test the dop...

  15. Multifaceted Effects of Noisy Galvanic Vestibular Stimulation on Manual Tracking Behavior in Parkinson’s Disease

    OpenAIRE

    Daniel Svenkeson; Martin James McKeown

    2015-01-01

    Parkinson’s disease (PD) is a neurodegenerative movement disorder that is characterized clinically by slowness of movement, rigidity, tremor, postural instability, and often cognitive impairments. Recent studies have demonstrated altered cortico-basal ganglia rhythms in PD, which raises the possibility of a role for non-invasive stimulation therapies such as noisy galvanic vestibular stimulation (GVS). We applied noisy GVS to 12 mild-moderately affected PD subjects (Hoehn & Yahr 1.5-2.5) off ...

  16. Multifaceted effects of noisy galvanic vestibular stimulation on manual tracking behavior in Parkinson’s disease

    OpenAIRE

    Lee, Soojin; Kim, Diana J.; Svenkeson, Daniel; Parras, Gabriel; Oishi, Meeko Mitsuko K.; Mckeown, Martin J.

    2015-01-01

    Parkinson’s disease (PD) is a neurodegenerative movement disorder that is characterized clinically by slowness of movement, rigidity, tremor, postural instability, and often cognitive impairments. Recent studies have demonstrated altered cortico-basal ganglia rhythms in PD, which raises the possibility of a role for non-invasive stimulation therapies such as noisy galvanic vestibular stimulation (GVS). We applied noisy GVS to 12 mild-moderately affected PD subjects (Hoehn and Yahr 1.5–2.5) of...

  17. EFFECT OF MICRONUTRIENTS-ENRICHED FERTILIZERS ON BASAL STEM ROT DISEASE INCIDENCE AND SEVERITY ON OIL PALM (ELAEIS GUINEENSIS JACQ. SEEDLINGS

    Directory of Open Access Journals (Sweden)

    Fabien Fonguimgo Tengoua

    2014-01-01

    Full Text Available Basal stem rot caused by Ganoderma boninense constitutes a serious threat to oil palm industry in Southeast Asia, especially in Malaysia and Indonesia and in Papua New Guinea and Pacific Islands. It is also expanding in some oil palm growing countries in Latin America and Africa and will soon become a worldwide concern to oil palm cultivation. To date, none of the various control measures developed and tested to control the disease since many decade gives entire satisfaction. An experiment was carried out to see whether incorporation of micronutrients, Copper (Cu, Boron (B and Manganese (Mn could reduce the incidence and severity of this disease on oil palm seedlings inoculated with G. boninense. The concentrations tested were 2 mg B/kg of soil, 2 mg Cu/kg of soil and 2 mg Mn/kg of soil incorporated into the basic fertilizer NPKMg 14-10-10-2. Treatments were applied in solution for three months before inoculation, followed by soil application for eight months after inoculation. The results showed that although no significant difference was detected among treatments, the double combinations of these micronutrients, B+Cu, B+Mn and Cu+Mn, performed better than the single nutrients in reducing the incidence and the severity of BSR, while their triple combination rather increased these pathological parameters. These double combinations could therefore be field-tested for their further integration in oil palm fertilization programme.

  18. Morphological changes of glutamatergic synapses in animal models of Parkinson’s disease

    Directory of Open Access Journals (Sweden)

    Rosa M Villalba

    2015-09-01

    Full Text Available The striatum and the subthalamic nucleus are the main entry doors for extrinsic inputs to reach the basal ganglia circuitry. The cerebral cortex, thalamus and brainstem are the key sources of glutamatergic inputs to these nuclei. There is functional and neurochemical evidence that glutamatergic neurotransmission is altered in the striatum and subthalamic nucleus of animal models of Parkinson’s disease, and that these changes may contribute to aberrant network neuronal activity in the basal ganglia-thalamocortical circuitry. Postmortem studies of animal models and PD patients have revealed significant pathology of glutamatergic synapses, dendritic spines and microcircuits in the striatum of parkinsonians. More recent findings have also demonstrated a significant breakdown of the glutamatergic corticosubthalamic system in parkinsonian monkeys. In this review, we will discuss evidence for synaptic glutamatergic dysfunction and pathology of cortical and thalamic inputs to the striatum and subthalamic nucleus in models of Parkinson’s disease. The potential functional implication of these alterations on synaptic integration, processing and transmission of extrinsic information through the basal ganglia circuits will be considered. Finally, the significance of these pathological changes in the pathophysiology of motor and non-motor symptoms in Parkinson’s disease will be examined.

  19. Declarative and procedural learning in Parkinson's disease patients having tremor or bradykinesia as the predominant symptom.

    Science.gov (United States)

    Vakil, E; Herishanu-Naaman, S

    1998-09-01

    The distinction between procedural and declarative memory is widely accepted in the memory literature. Converging evidence makes a strong case that the medial aspects of the temporal lobes and the diencephalon subserve the declarative memory system. However, the neuroanatomy of procedural memory is much less clear. In animal studies, damage to the basal ganglia has been found to affect procedural memory, but studies of patients suffering from degenerative diseases of the basal ganglia (e.g., Parkinson's and Huntington's disease) are less conclusive. Two groups of Parkinson's disease subtypes, with tremor (PDt) and bradykinesia (PDb) as the predominant motor symptom, were compared to controls on declarative and procedural memory tasks. The two patient groups did not differ from each other on the declarative tasks. However, in the procedural learning tasks, the PDb but not the PDt group, was significantly impaired compared to the control group. The results are discussed in terms of the differential involvement of discrete neuroanatomic loops connecting the basal ganglia and the prefrontal cortex. PMID:9800094

  20. Cardiovascular effects of basal insulins.

    Science.gov (United States)

    Mannucci, Edoardo; Giannini, Stefano; Dicembrini, Ilaria

    2015-01-01

    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 regard to cardiovascular safety will provide definitive evidence. PMID:26203281

  1. Evaluation of Basal Serum Adrenocorticotropic Hormone and Cortisol Levels and Their Relationship with Nonalcoholic Fatty Liver Disease in Male Patients with Idiopathic Hypogonadotropic Hypogonadism

    Directory of Open Access Journals (Sweden)

    Wen-Bo Wang

    2016-01-01

    Conclusions: The male IHH patients showed higher basal serum ACTH levels and lower cortisol levels than matched healthy controls. NAFLD was an independent associated factor for ACTH levels in male IHH patients. These preliminary findings provided evidence of the relationship between basal serum ACTH and NAFLD in male IHH patients.

  2. Prepulse inhibition is associated with attention, processing speed, and 123I-FP-CIT SPECT in Parkinson's disease

    DEFF Research Database (Denmark)

    Zoetmulder, Marielle; Biernat, Heidi B; Nikolic, Miki;

    2014-01-01

    BACKGROUND: Prepulse inhibition is a measure of sensorimotor gating, which reflects the ability to filter or 'gate' irrelevant information. Prepulse inhibition is dramatically altered in basal ganglia disorders associated with dysfunction in the midbrain dopaminergic system, and corresponding...... cognitive information processing deficits such as slowed processing speed. Parkinson's disease is characterised by the degeneration of the midbrain dopaminergic system and is associated with cognitive dysfunction, including slowed information processing. Although sensorimotor processes in Parkinson...

  3. Cerebral neurotransmission in huntington's disease and wilson's disease

    International Nuclear Information System (INIS)

    Huntington's disease and Wilson's disease are hereditary disorders with different neuropsychiatric symptoms. In both cases, these symptoms are mainly attributed to functional alterations of neurons, which are located in the basal ganglia. According deficits have been found by investigating the dopaminergic neurotransmission with different PET and SPECT tracers. For both diseases, these deficits revealed to concordantly involve the pre- and postsynaptic compartment. Apart from the dopaminergic system, more recent studies showed alterations of other neurotransmitter systems, like the serotonergic, GABA-ergic and opioide system. Except for scientific studies, nuclear medicine imaging is not regularly required for primary diagnosis of both disorders. In the case of Huntington's disease, however, imaging can be helpful for differential diagnosis to other diseases with similar initial symptoms and to determine the organic manifestation of the gene defect. In addition, neurotransmitter imaging with radiortracers could gain more relevance in the future in supporting decisions on specific treatments or for therapy monitoring in both diseases. (orig.)

  4. Gene Therapy for Parkinson's Disease

    Directory of Open Access Journals (Sweden)

    Rachel Denyer

    2012-01-01

    Full Text Available Current pharmacological and surgical treatments for Parkinson's disease offer symptomatic improvements to those suffering from this incurable degenerative neurological disorder, but none of these has convincingly shown effects on disease progression. Novel approaches based on gene therapy have several potential advantages over conventional treatment modalities. These could be used to provide more consistent dopamine supplementation, potentially providing superior symptomatic relief with fewer side effects. More radically, gene therapy could be used to correct the imbalances in basal ganglia circuitry associated with the symptoms of Parkinson's disease, or to preserve or restore dopaminergic neurons lost during the disease process itself. The latter neuroprotective approach is the most exciting, as it could theoretically be disease modifying rather than simply symptom alleviating. Gene therapy agents using these approaches are currently making the transition from the laboratory to the bedside. This paper summarises the theoretical approaches to gene therapy for Parkinson's disease and the findings of clinical trials in this rapidly changing field.

  5. Disease: H00076 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available gion: cerebral cortex, cerebellum, basal ganglia Microscopic lesion: accumulate of DNA lesions, tigroid-type...cision repair cross complementing, group 6 (mutation) [HSA:2074] [KO:K10841] Prodarsan (Phase I) Affected re

  6. Nevoid Basal Cell Carcinoma Syndrome

    Science.gov (United States)

    ... Nevoid Basal Cell Carcinoma Syndrome Request Permissions Nevoid Basal Cell Carcinoma Syndrome Approved by the Cancer.Net Editorial Board , 04/2016 What is Nevoid Basal Cell Carcinoma Syndrome? Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is ...

  7. Correlation of CT perfusion and CT volumetry in patients with Alzheimers disease

    International Nuclear Information System (INIS)

    Background: Both brain atrophy and decrease of perfusion are observed in dementive diseases. The aim of the study was to correlate the results of brain perfusion CT (pCT) and CT volumetry in patients with Alzheimers disease (AD). Material/Methods: Forty-eight patients with AD (mean age of 71.3 years) underwent brain pCT and CT volumetry. The pCT was performed at the level of basal ganglia after the injection of contrast medium (50 ml, 4 ml/sec.) with serial scanning (delay 7 sec, 50 scans, 1 scan/sec). Volumetric measurements were carried out on the basis of source images, with the use of a dedicated CT software combined with manual outlining of the regions of interest in extracerebral and intraventricular CSF spaces. Perfusion parameters of the cerebral blood flow (CBF) and cerebral blood volume (CBV) from the grey matter of frontal and temporal as well as basal ganglia were compared statistically with the volumetric measurements of frontal and temporal cortical atrophy as well as subcortical atrophy. Results: A statistically significant positive correlation was found between the values of CBF and CBV in the basal ganglia and the volumes of the lateral and third ventricles. The comparison of CBF and CBV results with the volumetric measurements in the areas of the frontal and temporal lobes showed mostly negative correlations, but none of them was of statistical significance. Conclusions: In patients with AD, the degree of cortical atrophy is not correlated with the decrease of perfusion in the grey matter and subcortical atrophy is not correlated with the decrease of perfusion in the basal ganglia region. It suggests that functional and structural changes in AD are not related to each other. (authors)

  8. Correlation of CT perfusion and CT volumetry in patients with Alzheimer’s disease

    Science.gov (United States)

    Czarnecka, Anna; Zimny, Anna; Sąsiadek, Marek

    2010-01-01

    Summary Background: Both brain atrophy and decrease of perfusion are observed in dementive diseases. The aim of the study was to correlate the results of brain perfusion CT (pCT) and CT volumetry in patients with Alzheimer’s disease (AD). Material/Methods: Forty-eight patients with AD (mean age of 71.3 years) underwent brain pCT and CT volumetry. The pCT was performed at the level of basal ganglia after the injection of contrast medium (50 ml, 4 ml/sec.) with serial scanning (delay 7 sec, 50 scans, 1 scan/sec). Volumetric measurements were carried out on the basis of source images, with the use of a dedicated CT software combined with manual outlining of the regions of interest in extracerebral and intraventricular CSF spaces. Perfusion parameters of the cerebral blood flow (CBF) and cerebral blood volume (CBV) from the grey matter of frontal and temporal as well as basal ganglia were compared statistically with the volumetric measurements of frontal and temporal cortical atrophy as well as subcortical atrophy. Results: A statistically significant positive correlation was found between the values of CBF and CBV in the basal ganglia and the volumes of the lateral and third ventricles. The comparison of CBF and CBV results with the volumetric measurements in the areas of the frontal and temporal lobes showed mostly negative correlations, but none of them was of statistical significance. Conclusions: In patients with AD, the degree of cortical atrophy is not correlated with the decrease of perfusion in the grey matter and subcortical atrophy is not correlated with the decrease of perfusion in the basal ganglia region. It suggests that functional and structural changes in AD are not related to each other. PMID:22802771

  9. [Dysexecutive syndromes and degenerative diseases].

    Science.gov (United States)

    Pillon, B; Czernecki, V; Dubois, B

    2004-04-01

    A dysexecutive syndrome is observed not only in frontotemporal lobar degeneration, but also in subcortical degenerative diseases, and even in Alzheimer's disease whose lesions predominate in temporoparietal associative areas. The association between a dysexecutive syndrome and various cerebral localisations may be explained by the fact that cognitive and behavioral organisation recruits anatomofunctional frontostriatal and frontoparietal circuits. Both animal experimentation and human clinical observation argue in favour of a functional continuity and complementarity among these loops. The prefrontal cortex would be particularly needed in new situations, to inhibit old programs of action not adapted to the present context and to elaborate new ones; the basal ganglia would be rather required by the repetition of the situation to progressively transform the new program in routine. If we refer to Shallice model, we can hypothesize that optimal executive functions require the preservation not only of the Supervisory Attentional System, mainly dependent on the prefrontal cortex, but also of the Contention Scheduling, recruiting the basal ganglia, and of the Schemas of Action, represented in parietal and premotor areas. Therefore, the neuropsychological assessment of patients with degenerative diseases contributes to the understanding of the anatomofunctional architecture of executive functions.

  10. Basal Reinforced Piled Embankments

    NARCIS (Netherlands)

    Van Eekelen, S.J.M.

    2015-01-01

    A basal reinforced piled embankment consists of a reinforced embankment on a pile foundation. The reinforcement consists of one or more horizontal layers of geosynthetic reinforcement (GR) installed at the base of the embankment. The design of the GR is the subject of this thesis. A basal reinforce

  11. Neural dynamics of short and medium-term motor control effects of levodopa therapy in Parkinson's disease.

    Science.gov (United States)

    Contreras-Vidal, J L; Poluha, P; Teulings, H L; Stelmach, G E

    1998-05-01

    A neural network model of movement control in normal and Parkinson's disease (PD) conditions is proposed to simulate the time-varying dose-response relationship underlying the effects of levodopa on movement amplitude and movement duration in PD patients. Short and long-term dynamics of cell activations and neurotransmitter mechanisms underlying the differential expression of neuropeptide messenger RNA within the basal ganglia striatum are modeled to provide a mechanistic account for the effects of levodopa medication on motor performance (e.g. the pharmacodynamics). Experimental and neural network simulation data suggest that levodopa therapy in Parkinson's disease has differential effects on cell activities, striatal neuropeptides, and motor behavior. In particular, it is shown how dopamine depletion in the striatum may modulate differentially the level of substance P and enkephalin messenger RNA in the direct and indirect basal ganglia pathways. This dissociation in the magnitude and timing of peptide expression causes an imbalance in the opponently organized basal ganglia pathways which results in Parkinsonian motor deficits. The model is validated with experimental data obtained from handwriting movements performed by PD subjects before and after medication intake. The results suggest that fine motor control analysis and network modeling of the effects of dopamine in motor control are useful tools in drug development and in the optimization of pharmacological therapy in PD patients. PMID:9654379

  12. CT hypodensity on cerebral white matter in Wilson's disease

    Directory of Open Access Journals (Sweden)

    Laura B. Jardim

    1991-06-01

    Full Text Available Wilson's disease in an autosomal recessive disorder of copper metabolism where systemic manifestations are secondary to thei accumulation of copper in hepatic, nervous and other tissues. In CNS, the structural lesions most commonly found by CT scan are ventricular dilatation, cortical atrophy, basal ganglia hyperdensities, and brainstem and cerebellar atrophy. Degenerative changes of cerebral white matter seen on early anatomo-pathologic studies, but were almost never found on CT scan from recently described patients. We report a case of Wilson's disease with an unusually rapid deterioration where asymmetric low-densities in the subcortical white matter were disclosed by CT scan.

  13. Perfusion and metabolism imaging studies in Parkinson's disease

    DEFF Research Database (Denmark)

    Borghammer, Per

    2012-01-01

    Positron emission tomography (PET) and single photon emission computed tomography (SPECT) are important tools in the evaluation of brain blood flow and glucose metabolism in Parkinson's disease (PD). However, conflicting results are reported in the literature depending on the type of imaging data....... It is concluded that PD most likely is characterized by widespread cortical hypometabolism, probably even at early disease stages. Widespread subcortical hypermetabolism is probably not a feature of PD, although certain small basal ganglia structures, such as the external pallidum, may display true...

  14. Masked assessment of MRI findings: is it possible to differentiate neuro-Behcet`s disease from other central nervous system

    Energy Technology Data Exchange (ETDEWEB)

    Coban, O.; Bahar, S.; Akman-Demir, G.; Tasci, B.; Serdaroglu, P. [Univ. of Istanbul (Turkey). Dept. of Neurology; Yurdakul, S.; Yazici, H. [Univ. of Istanbul (Turkey). Dept. of Internal Medicine

    1999-04-01

    Two neuroradiologists reviewed MRI studies of 34 patients with neuro-Behcet`s disease (NBD), 22 with multiple sclerosis (MS) and 7 with systemic lupus erythematosus (SLE) with central nervous system involvement, masked to the clinical diagnosis, age and sex of the patients. Of the patients with NBD 12 were in an acute attack; the others had chronic disease. MRI was assessed using a set of criteria, looking at atrophy, the site of discrete parenchymal lesions, regions of predominant involvement and the extent of the lesion(s). The observers also made a guess at the clinical diagnosis. The brain stem and/or basal ganglia were the most predominantly involved sites in all patients with acute NBD; 75 % of these lesions were large and confluent, mainly extending from the brain stem to the diencephalon and basal ganglia. However, in chronic cases, the predominant involvement was in the brain stem and/or basal ganglia in only 36 %, and in cerebral hemisphere white matter in another 36 %; 27 % of these patients showed no parenchymal lesion. Hemisphere white-matter lesions were equally distributed between periventricular and other areas in NBD, while in MS more were periventricular, and in SLE more were nonperiventricular. Brain-stem atrophy was seen in 21 % of patients with NBD, with a specificity of 96.5 %. In the absence of cortical atrophy, its specificity was 100 %. The attempt at making a radiological diagnosis was successful in all cases of acute NBD and 95.5 % of patients with MS, but in only 40 % of patients with chronic NBD. Most of this latter groups MRI studies were interpreted as MS. An extensive lesion involving the brain stem and basal ganglia seemed to be diagnostic of acute NBD. However, hemisphere white-matter lesions could not be differentiated from those in MS. (orig.) With 3 figs., 6 tabs., 18 refs.

  15. Computational model of precision grip in Parkinson’s disease: A Utility based approach

    Directory of Open Access Journals (Sweden)

    Ankur eGupta

    2013-12-01

    Full Text Available We propose a computational model of Precision Grip (PG performance in normal subjects and Parkinson’s Disease (PD patients. Prior studies on grip force generation in PD patients show an increase in grip force during ON medication and an increase in the variability of the grip force during OFF medication (Fellows et al 1998; Ingvarsson et al 1997. Changes in grip force generation in dopamine-deficient PD conditions strongly suggest contribution of the Basal Ganglia, a deep brain system having a crucial role in translating dopamine signals to decision making. The present approach is to treat the problem of modeling grip force generation as a problem of action selection, which is one of the key functions of the Basal Ganglia. The model consists of two components: 1 the sensory-motor loop component, and 2 the Basal Ganglia component. The sensory-motor loop component converts a reference position and a reference grip force, into lift force and grip force profiles, respectively. These two forces cooperate in grip-lifting a load. The sensory-motor loop component also includes a plant model that represents the interaction between two fingers involved in PG, and the object to be lifted. The Basal Ganglia component is modeled using Reinforcement Learning with the significant difference that the action selection is performed using utility distribution instead of using purely Value-based distribution, thereby incorporating risk-based decision making. The proposed model is able to account for the precision grip results from normal and PD patients accurately (Fellows et. al. 1998; Ingvarsson et. al. 1997. To our knowledge the model is the first model of precision grip in PD conditions.

  16. Computational model of precision grip in Parkinson's disease: a utility based approach.

    Science.gov (United States)

    Gupta, Ankur; Balasubramani, Pragathi P; Chakravarthy, V Srinivasa

    2013-01-01

    We propose a computational model of Precision Grip (PG) performance in normal subjects and Parkinson's Disease (PD) patients. Prior studies on grip force generation in PD patients show an increase in grip force during ON medication and an increase in the variability of the grip force during OFF medication (Ingvarsson et al., 1997; Fellows et al., 1998). Changes in grip force generation in dopamine-deficient PD conditions strongly suggest contribution of the Basal Ganglia, a deep brain system having a crucial role in translating dopamine signals to decision making. The present approach is to treat the problem of modeling grip force generation as a problem of action selection, which is one of the key functions of the Basal Ganglia. The model consists of two components: (1) the sensory-motor loop component, and (2) the Basal Ganglia component. The sensory-motor loop component converts a reference position and a reference grip force, into lift force and grip force profiles, respectively. These two forces cooperate in grip-lifting a load. The sensory-motor loop component also includes a plant model that represents the interaction between two fingers involved in PG, and the object to be lifted. The Basal Ganglia component is modeled using Reinforcement Learning with the significant difference that the action selection is performed using utility distribution instead of using purely Value-based distribution, thereby incorporating risk-based decision making. The proposed model is able to account for the PG results from normal and PD patients accurately (Ingvarsson et al., 1997; Fellows et al., 1998). To our knowledge the model is the first model of PG in PD conditions. PMID:24348373

  17. Similarities and differences of MR findings between Japanese encephalitis and Wilson's disease

    Energy Technology Data Exchange (ETDEWEB)

    Saha, Manash; Kumar, Sunil; Gupta, Rakesh K. [Department of Radiodiagnosis, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow (India); Das, Ashish [Department of Neurology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow (India)

    2002-04-01

    Although Japanese Encephalitis (JE) and Wilson's disease (WD) are different entities, MR findings in both these conditions are quite similar. The purpose of this retrospective study was to find out the similarities and differences between JE and WD on MR imaging. The study group comprised 25 proven cases of JE and 10 cases of WD. Spin echo (SE) TI- and T2-weighted imaging was performed on a 1.5-T MR system. Fourteen of these 35 cases (10 JE, 4 WD) were also examined using T1-weighted magnetization transfer (MT) SE sequence before and after contrast administration. Although both JE and WD showed similar topographical distribution of lesions, predominant involvement of the basal ganglia and thalami, there were some differences. Brain stem lesion was more frequent for WD than for JE, and posteromedial part of the thalami was spared in WD. The lesion characteristics were also different between both; in WD mixed intensity in the basal ganglia and hyperintense linear rim at the peripheral putamen was observed frequently, whereas hyperintense basal ganglia on T2-weighted images, subacute hemorrhage in the thalami and meningeal enhancement were seen only in the patients with JE. These characteristic lesion criteria may help in differentiation of JE from WD on MR imaging. (orig.)

  18. The role of frontostriatal impairment in freezing of gait in Parkinson’s disease

    Directory of Open Access Journals (Sweden)

    James eShine

    2013-10-01

    Full Text Available Freezing of gait (FOG is a disabling symptom of advanced Parkinson’s disease (PD that leads to an increased risk of falls and nursing home placement. Interestingly, multiple lines of evidence suggest that the manifestation of FOG is related to specific deficits in cognition, such as set shifting and the ability to process conflict-related signals. These findings are consistent with the specific patterns of abnormal cortical processing seen during functional neuroimaging experiments of FOG, implicating increased neural activation within cortical structures underlying cognition, such as the Cognitive Control Network. In addition, these studies show that freezing episodes are associated with abnormalities in the BOLD response within key structures of the basal ganglia, such as the striatum and the subthalamic nucleus. In this article, we discuss the implications of these findings on current models of freezing behaviour and propose an updated model of basal ganglia impairment during FOG episodes that integrates the neural substrates of freezing from the cortex and the basal ganglia to the cognitive dysfunctions inherent in the condition.

  19. Cognitive and neuropsychiatric disorders in extrapyramidal diseases

    Directory of Open Access Journals (Sweden)

    O. S. Levin

    2012-01-01

    Full Text Available The affliction of basal ganglia and their associations, which underlies extrapyramidal diseases, leads not only to diverse movements, but also to a broad spectrum of neuropsychological disorders, including cognitive, emotional-personality, and psychotic ones. The basis for these disorders are most commonly dysfunction of one or a few parallel frontostriatal circuits combining the basal ganglia with the thalamus, limbic structures, frontal and other cortical regions into the uniform functional system. The pattern of neuropsychological disorders has both specific and common features, the discussion of which is the subject matter of this paper. Analysis of the specific features of neuropsychological and behavioral disorders allows one to specify the localization of a pathological process and its extent, which may be of important differential diagnostic value. Cognitive and emotional-personality impairments sometimes develop earlier than motor disorders and their detection may be of significance for the early diagnosis of Parkinson’s disease or Huntington’s disease in persons who are predisposed to these diseases. Moreover, to establish the nature of mental disorders and the degree of their disadapting effect and to identify the affected and preserved components in the psychic processes are important for the choice of adequate pharmacotherapy and the elaboration of neuropsychological rehabilitation programs.

  20. Basal gene expression by lung CD4+ T cells in chronic obstructive pulmonary disease identifies independent molecular correlates of airflow obstruction and emphysema extent.

    Directory of Open Access Journals (Sweden)

    Christine M Freeman

    Full Text Available Lung CD4+ T cells accumulate as chronic obstructive pulmonary disease (COPD progresses, but their role in pathogenesis remains controversial. To address this controversy, we studied lung tissue from 53 subjects undergoing clinically-indicated resections, lung volume reduction, or transplant. Viable single-cell suspensions were analyzed by flow cytometry or underwent CD4+ T cell isolation, followed either by stimulation with anti-CD3 and cytokine/chemokine measurement, or by real-time PCR analysis. In lung CD4+ T cells of most COPD subjects, relative to lung CD4+ T cells in smokers with normal spirometry: (a stimulation induced minimal IFN-γ or other inflammatory mediators, but many subjects produced more CCL2; (b the T effector memory subset was less uniformly predominant, without correlation with decreased IFN-γ production. Analysis of unstimulated lung CD4+ T cells of all subjects identified a molecular phenotype, mainly in COPD, characterized by markedly reduced mRNA transcripts for the transcription factors controlling TH1, TH2, TH17 and FOXP3+ T regulatory subsets and their signature cytokines. This mRNA-defined CD4+ T cell phenotype did not result from global inability to elaborate mRNA; increased transcripts for inhibitory CD28 family members or markers of anergy; or reduced telomerase length. As a group, these subjects had significantly worse spirometry, but not DLCO, relative to subjects whose lung CD4+ T cells expressed a variety of transcripts. Analysis of mRNA transcripts of unstimulated lung CD4+ T cell among all subjects identified two distinct molecular correlates of classical COPD clinical phenotypes: basal IL-10 transcripts correlated independently and inversely with emphysema extent (but not spirometry; by contrast, unstimulated IFN-γ transcripts correlated independently and inversely with reduced spirometry (but not reduced DLCO or emphysema extent. Aberrant lung CD4+ T cells polarization appears to be common in advanced

  1. DISCONNECTION OF A BASAL GANGLIA CIRCUIT IN JUVENILE SONGBIRDS ATTENUATES THE SPECTRAL DIFFERENTIATION OF SONG SYLLABLES

    OpenAIRE

    Elliott, Kevin C.; Wu, Wei; Bertram, Richard; Johnson, Frank

    2013-01-01

    Similar to language acquisition by human infants, juvenile male zebra finches (Taeniopygia guttata) imitate an adult (tutor) song by transitioning from repetitive production of one or two undifferentiated protosyllables to the sequential production of a larger and spectrally heterogeneous set of syllables. The primary motor region that controls learned song is driven by a confluence of input from two pre-motor pathways: a posterior pathway that encodes the adult song syllables and an anterior...

  2. Hyporesponsive Reward Anticipation in the Basal Ganglia following Severe Institutional Deprivation Early in Life

    Science.gov (United States)

    Mehta, Mitul A.; Gore-Langton, Emma; Golembo, Nicole; Colvert, Emma; Williams, Steven C. R.; Sonuga-Barke, Edmund

    2010-01-01

    Severe deprivation in the first few years of life is associated with multiple difficulties in cognition and behavior. However, the brain basis for these difficulties is poorly understood. Structural and functional neuroimaging studies have implicated limbic system structures as dysfunctional, and one functional imaging study in a heterogeneous…

  3. Motion and Emotion : Functional In Vivo Analyses of the Mouse Basal Ganglia

    OpenAIRE

    Arvidsson, Emma

    2014-01-01

    A major challenge in the field of neuroscience is to link behavior with specific neuronal circuitries and cellular events. One way of facing this challenge is to identify unique cellular markers and thus have the ability to, through various mouse genetics tools, mimic, manipulate and control various aspects of neuronal activity to decipher their correlation to behavior. The Vesicular Glutamate Transporter 2 (VGLUT2) packages glutamate into presynaptic vesicles for axonal terminal release. In ...

  4. Severity of Dysfluency Correlates with Basal Ganglia Activity in Persistent Developmental Stuttering

    Science.gov (United States)

    Giraud, Anne-Lise; Neumann, Katrin; Bachoud-Levi, Anne-Catherine; von Gudenberg, Alexander W.; Euler, Harald A.; Lanfermann, Heinrich; Preibisch, Christine

    2008-01-01

    Previous studies suggest that anatomical anomalies [Foundas, A. L., Bollich, A. M., Corey, D. M., Hurley, M., & Heilman, K. M. (2001). "Anomalous anatomy of speech-language areas in adults with persistent developmental stuttering." "Neurology," 57, 207-215; Foundas, A. L., Corey, D. M., Angeles, V., Bollich, A. M., Crabtree-Hartman, E., & Heilman,…

  5. Interacting cortical and basal ganglia networks underlying finding and tapping to the musical beat.

    Science.gov (United States)

    Kung, Shu-Jen; Chen, Joyce L; Zatorre, Robert J; Penhune, Virginia B

    2013-03-01

    Humans are able to find and tap to the beat of musical rhythms varying in complexity from children's songs to modern jazz. Musical beat has no one-to-one relationship with auditory features-it is an abstract perceptual representation that emerges from the interaction between sensory cues and higher-level cognitive organization. Previous investigations have examined the neural basis of beat processing but have not tested the core phenomenon of finding and tapping to the musical beat. To test this, we used fMRI and had musicians find and tap to the beat of rhythms that varied from metrically simple to metrically complex-thus from a strong to a weak beat. Unlike most previous studies, we measured beat tapping performance during scanning and controlled for possible effects of scanner noise on beat perception. Results showed that beat finding and tapping recruited largely overlapping brain regions, including the superior temporal gyrus (STG), premotor cortex, and ventrolateral PFC (VLPFC). Beat tapping activity in STG and VLPFC was correlated with both perception and performance, suggesting that they are important for retrieving, selecting, and maintaining the musical beat. In contrast BG activity was similar in all conditions and was not correlated with either perception or production, suggesting that it may be involved in detecting auditory temporal regularity or in associating auditory stimuli with a motor response. Importantly, functional connectivity analyses showed that these systems interact, indicating that more basic sensorimotor mechanisms instantiated in the BG work in tandem with higher-order cognitive mechanisms in PFC.

  6. The role of basal ganglia and cerebellum in motor learning. A computational model

    OpenAIRE

    Senatore, Rosa

    2012-01-01

    2010 - 2011 Our research activity investigates the computational processes underlying the execution of complex sequences of movements and aims at understanding how different levels of the nervous system interact and contribute to the gradual improvement of motor performance during learning. Many research areas, from neuroscience to engineering, investigate, from different perspectives and for diverse purposes, the processes that allow humans to efficiently perform skilled movem...

  7. ANAESTHETIC MANAGEMENT OF A GERIATRIC PATIENT WITH PARKINSON`S DISEASE AND DIABETES MELLITUS POSTED FOR EMERGENCY LAPAROTOMY

    Directory of Open Access Journals (Sweden)

    Prajwal Patel

    2014-10-01

    Full Text Available Parkinson's disease (PD, one of the most common disabling neurological diseases, affects about 1% of the population over 60 years of age. It is a degenerative disease of the central nervous system caused by the loss of dopaminergic fibers in basal ganglia of the brain. PD is an important cause of perioperative morbidity and with an increasingly elderly population; it is being encountered with greater frequency in surgical patients. Here we report a case of 79year old male with Parkinsonism and diabetes mellitus posted for emergency laparotomy, which we managed successfully with general anesthesia.

  8. Understanding Parkinson Disease: A Complex and Multifaceted Illness.

    Science.gov (United States)

    Gopalakrishna, Apoorva; Alexander, Sheila A

    2015-12-01

    Parkinson disease is an incredibly complex and multifaceted illness affecting millions of people in the United States. Parkinson disease is characterized by progressive dopaminergic neuronal dysfunction and loss, leading to debilitating motor, cognitive, and behavioral symptoms. Parkinson disease is an enigmatic illness that is still extensively researched today to search for a better understanding of the disease, develop therapeutic interventions to halt or slow progression of the disease, and optimize patient outcomes. This article aims to examine in detail the normal function of the basal ganglia and dopaminergic neurons in the central nervous system, the etiology and pathophysiology of Parkinson disease, related signs and symptoms, current treatment, and finally, the profound impact of understanding the disease on nursing care.

  9. Creutzfeldt-Jakob disease: Magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Puvaneswary, M.; Floate, D. [John Hunter Hospital, NewCastle, NSW (Australia). Departments of Medical Imaging and Neurology; Harper, C. [Royal Prince Alfred Hospital, Sydney, NSW (Australia). Department of Neuropathology

    1999-02-01

    Rapidly progressive dementia in an adult with findings of bilateral, symmetric high signal intensity on T2-weighted sequences and normal findings on T1-weighted sequences predominantly in the deep grey matter is suggestive of Creutzfeldt-Jakob disease (CJD). The peripheral cortex may be involved, as it was in the present case. The absence of subcortical periventricular white matter high signal intensity suggests that symmetric high signal intensities within the basal ganglia and cortical grey matter are more likely to be due to a degenerative process rather than due to ischaemia, infection or tumour. Copyright (1999) Blackwell Science Pty Ltd 17 refs., 6 figs.

  10. Imaging and clinical characteristics of sporadic Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    HAN Shun-chang

    2013-04-01

    Full Text Available Five patients with sporadic Creutzfeldt-Jakob disease (sCJD presented rapidly progressive dementia which were subacute onset from 1 to 4 months. Among these cases, periodic synchronous discharge (PSD of electroencephalography (EEG was seen in 2 patients. Besides, 4 patients obtained positive results in cerebrospinal fluid (CSF analysis for 14-3-3 protein. The cranial MRI examination showed symmetrical or asymmetrical colored-ribbon-shaped high signals in cerebral cortex or basal ganglia by diffusion weighted imaging (DWI, suggesting that DWI had high sensitivity and specificity for the diagnosis of sCJD as a preferred method in the clinical examination of sCJD.

  11. A study of 99mTc-HM-PAO brain SPECT in the senile parkinson's disease

    International Nuclear Information System (INIS)

    Thirty-three cases of senile Parkinson's disease (PD) imaged by 99mTc-HM-PAO brain SPECT were reported. 66.7% of the patients had cortical hypoperfusion and 18.2% showed asymmetrical hypoperfusion in the basal ganglia. Such a finding was not related with the Hoehn-Yahr stage and the laterality of motor symptoms. If complicated with dementia, the SPECT brain imaging showed similar pattern in Alzheimer's disease with diffuse hypoperfusion in cortical area reflecting widespread pathological changes in tremor paralysis

  12. Hypopituitarism Presenting as Adrenal Insufficiency and Hypothyroidism in a Patient with Wilson's Disease: a Case Report.

    Science.gov (United States)

    Lee, Hae Won; Kang, Jin Du; Yeo, Chang Woo; Yoon, Sung Woon; Lee, Kwang Jae; Choi, Mun Ki

    2016-08-01

    Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.

  13. Dopaminergic therapy in Parkinson's disease decreases cortical beta band coherence in the resting state and increases cortical beta band power during executive control.

    Science.gov (United States)

    George, Jobi S; Strunk, Jon; Mak-McCully, Rachel; Houser, Melissa; Poizner, Howard; Aron, Adam R

    2013-01-01

    It is not yet well understood how dopaminergic therapy improves cognitive and motor function in Parkinson's disease (PD). One possibility is that it reduces the pathological synchronization within and between the cortex and basal ganglia, thus improving neural communication. We tested this hypothesis by recording scalp electroencephalography (EEG) in PD patients when On and Off medication, during a brief resting state epoch (no task), and during performance of a stop signal task that is thought to engage two partially overlapping (or different) frontal-basal-ganglia circuits. For resting state EEG, we measured pair-wise coherence between scalp electrodes in several frequency bands. Consistent with previous studies, in the Off medication state, those patients with the greatest clinical impairment had the strongest coherence, especially in the beta band, indicating pathological over-synchronization. Dopaminergic medication reduced this coherence. For the stop signal task, On vs. Off medication increased beta band power over right frontal cortex for successful stopping and over bilateral sensorimotor cortex for going, especially for those patients who showed greater clinical improvement. Thus, medication reduced pathological coherence in beta band at rest and increased task related beta power for two potentially dissociable cortico-basal ganglia circuits. These results support the hypothesis that dopaminergic medication in PD improves neural communication both at rest and for executive and motor function.

  14. Computed tomography of neurodegenerative disease in childhood. Serial CT findings and their diagnostic values

    Energy Technology Data Exchange (ETDEWEB)

    Kataoka, Kenkichi; Nakagawa, Yoshihiro; Hojo, Hiroatsu

    1984-12-01

    Serial computed tomographic scans were performed on seven children with neurodegenerative disorders. In two cases of white-matter diseases (Krabbe's disease and metachromatic leukodystrophy), diffuse, low-density lesions of white matter were visible in the early stage of the diseases. In one case of adrenoleukodystrophy, regional low-density lesions of the white matter around the posterior horns and peculiar high-density strip lesions were visible in the early stage. In two cases of storage-type gray-matter diseases (Tay-Sachs' and infantile Gaucher's disease), there were no abnormalities in the early stage, but diffuse cortical atrophies in the late stage. In one case of Leigh's disease, there were small, low-density lesions of the basal ganglia and multiple low-density lesions of the gray matter in the early stage. In one case of subacute sclerosing panencephalitis, there were no abnormalities in the early stage, but small, low-density lesions of the basal ganglia and diffuse cerebral atrophies in the late stage. Diagnostic values were recognized dominantly in two cases of adrenoleukodystrophy and Leigh's disease. In the other cases, however, serial CT scans were useful in the diagnostic process. (author).

  15. Fahr's disease in two siblings in a family: A case report

    Science.gov (United States)

    WANG, HONG; SHAO, BEI; WANG, LIUQING; YE, QIANG

    2015-01-01

    Idiopathic basal ganglia calcification, also known as Fahr's disease, is a rare neurological disease characterized by basal ganglia calcification, Parkinsonism and psychiatric symptoms. The majority of patients with Fahr's disease are adults. The present study describes the cases of two patients with Fahr's disease. The patients were brother and sister and their parents were close relatives. The onset age of Fahr's disease in these two patients was early, with the onset age of the brother being in the teens and the sister in early childhood. The patients exhibited different clinical manifestations. The main symptoms of the male patient were Parkinson's disease appearance and the loss of the ability to carry out simple calculations, while the main symptoms of the female patient were grand mal seizures and cerebellar ataxia. Although the two patients had distinct clinical manifestations, they both had similar intracranial multiple calcifications. The computed tomography scan remains the main method used in the diagnosis of Fahr's disease. Following treatment with dopamine and a dopamine receptor agonist, the extra-pyramidal symptoms of the male were significantly relieved. The female patient was administered antiepileptic drugs and there was no recurrence of epilepsy following treatment. PMID:26136916

  16. Two additional cases of metformin-associated encephalopathy in patients with end-stage renal disease undergoing hemodialysis.

    Science.gov (United States)

    Kang, Yeo-Jin; Bae, Eun Jin; Seo, Jong Woo; Jeon, Dae-Hong; Cho, Hyun Seop; Kim, Hyun-Jung; Chang, Se-Ho; Park, Dong Jun

    2013-01-01

    We report on two additional cases of metformin-associated encephalopathy in patients with end-stage renal disease (ESRD) undergoing hemodialysis. Two patients were seen at our hospital with abnormal neurological signs and symptoms. Magnetic resonance imaging (MRI) revealed the same pattern of high signal intensity in both basal ganglia in T2-weighted images in the two patients. The two patients had started taking metformin 5 and 6 weeks earlier at the same dose of 1000 mg per day. Metformin was immediately stopped, and regular hemodialysis was conducted. Their signs and symptoms resolved completely after these measures. The high signal intensity in both ganglia in T2-weighted MRI also disappeared. We should suspect metformin-induced encephalopathy and withdraw the drug when presented with diabetic patients with chronic kidney disease and neurological signs and symptoms of unknown cause.

  17. Altered Striatocerebellar Metabolism and Systemic Inflammation in Parkinson’s Disease

    Directory of Open Access Journals (Sweden)

    Chiun-Chieh Yu

    2016-01-01

    Full Text Available Parkinson’s disease (PD is the most second common neurodegenerative movement disorder. Neuroinflammation due to systemic inflammation and elevated oxidative stress is considered a major factor promoting the pathogenesis of PD, but the relationship of structural brain imaging parameters to clinical inflammatory markers has not been well studied. Our aim was to evaluate the association of magnetic resonance spectroscopy (MRS measures with inflammatory markers. Blood samples were collected from 33 patients with newly diagnosed PD and 30 healthy volunteers. MRS data including levels of N-acetylaspartate (NAA, creatine (Cre, and choline (Cho were measured in the bilateral basal ganglia and cerebellum. Inflammatory markers included plasma nuclear DNA, plasma mitochondrial DNA, and apoptotic leukocyte levels. The Cho/Cre ratio in the dominant basal ganglion, the dominant basal ganglia to cerebellum ratios of two MRS parameters NAA/Cre and Cho/Cre, and levels of nuclear DNA, mitochondrial DNA, and apoptotic leukocytes were significantly different between PD patients and normal healthy volunteers. Significant positive correlations were noted between MRS measures and inflammatory marker levels. In conclusion, patients with PD seem to have abnormal levels of inflammatory markers in the peripheral circulation and deficits in MRS measures in the dominant basal ganglion and cerebellum.

  18. Basal Cell Carcinoma

    Science.gov (United States)

    ... resources Meet our partners Español Donate Diseases and treatments Acne and rosacea Bumps and growths Color problems Contagious skin diseases ... cell carcinoma public SPOT Skin Cancer™ Diseases and treatments Acne and rosacea Bumps and growths Color problems Contagious skin diseases ...

  19. Comparison of clinical types of Wilson's disease and glucose metabolism in extrapyramidal motor brain regions.

    Science.gov (United States)

    Hermann, W; Barthel, H; Hesse, S; Grahmann, F; Kühn, H-J; Wagner, A; Villmann, T

    2002-07-01

    In Wilson's disease a disturbed glucose metabolism especially in striatal and cerebellar areas has been reported. This is correlated with the severity of extrapyramidal motor symptoms (EPS). These findings are only based on a small number of patients. Up to now it is unknown whether EPS are caused by various patterns of disturbed basal ganglia glucose metabolism. We investigated 37 patients and 9 normal volunteers to characterize the disturbed glucose metabolism in Wilson's disease more precisely. The glucose metabolism was determined in 5 cerebellar and cerebral areas (putamen, caput nuclei caudati, cerebellum, midbrain and thalamic area) by using (18)F-Fluorodesoxyglucose-Positron-Emission-Tomography ( [(18)F]FDG-PET). The database was evaluated by a cluster analysis. Additionally, the severity extrapyramidal motor symptoms were judged by a clinical score system. Three characteristic patterns of glucose metabolism in basal ganglia were obtained. Two of them may be assigned to patients with neurological symptoms whereas the third cluster corresponds to most patients without EPS or normal volunteers. The clusters can be identified by characteristic consumption rates in this 5 brain areas. The severity of EPS can not clearly be assigned to one of the clusters with disturbed glucose metabolism. However, the most severe cases are characterized by the lowest consumption in the striatal area. When there is marked improvement of EPS impaired glucose consumption reveals a persistent brain lesion. Finally, the neurological symptoms in Wilson's disease are caused by (at least) two different patterns of disturbed glucose metabolism in basal ganglia and cerebellum. The severity of EPS seems to be determined by a disturbed consumption in the striatal area. PMID:12140675

  20. Estrogen Intake and Copper Depositions: Implications for Alzheimer's Disease

    Directory of Open Access Journals (Sweden)

    Florian Amtage

    2014-06-01

    Full Text Available We present a patient with chronic postmenopausal estrogen intake with presence of Kayser-Fleischer ring in the cornea and Alzheimer's disease and discuss the pathophysiological mechanisms of estrogen intake and copper accumulation in various tissues, including the central nervous system. Sonography was compatible with copper accumulation in the basal ganglia, but the patient showed no clinical signs of Wilson's disease. Magnetic resonance imaging and positron emission tomography revealed a typical pattern for Alzheimer's disease. We propose increased copper levels as a direct effect of estrogen intake due to an augmented ATP7A-mRNA in the intestine. Moreover, we discuss the impact of elevated free serum copper on accompanying Alzheimer's disease, knowing that copper plays a crucial role in the formation of amyloid plaques and tau aggregation. This might offer a partial explanation for the observation that postmenopausal estrogen therapy is associated with a higher risk of mild cognitive impairment and Alzheimer's disease.

  1. Migration and maturation pattern of fetal enteric ganglia: A study of 16 cases

    Directory of Open Access Journals (Sweden)

    Ranjana Bandyopadhyay

    2011-01-01

    Full Text Available Aims: To study the migration and developmental pattern of ganglion cells in fetuses aged 9-21 weeks, and to document whether the migration was occurring circumferentially equally in the entire axis or if there were discrepancies in different portions at the same level. Settings and Design: The hypothesis regarding the pathogenesis of Hirschsprung′s disease mainly revolves around two schools. One is the single gradient migration of ganglia and the other is a dual gradient migration theory. Understanding the embryological development of enteric ganglia is necessary to study the pathogenesis of intestinal innervation disorders. Materials and Methods: We studied the development of intestinal ganglia in fetuses aged 9-21 weeks. Serial longitudinal sections from the colon were studied, the first one including the squamo-columnar junction, for the presence and the nature of ganglion cells with Hematoxylin and Eosin, and neurone-specific enolase immunostaining. Transverse sections from proximal gut were studied in a similar fashion. Thus, we evaluated the migration pattern as well as the nature of ganglia in the fetuses. We also measured the length of distal aganglionic segment in these growing fetuses. Results: We noted that ganglion cells appear first in the myenteric plexus followed by deep and superficial submucous plexus. We also found evidences in favor of dual migration theory, and the distal aganglionic segment varies around the circumference of the rectal wall. Conclusions: We got evidences in support of a dual migration pattern of intestinal ganglion cells. The level of distal aganglionic segments when measured from squamo-columnar junction varied with the age of gestation and the length was incongruous. The description of distal aganglionic segment may help surgeons while taking biopsies or during operative procedures.

  2. rCBF SPECT in Parkinson's disease patients with mental dysfunction

    International Nuclear Information System (INIS)

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

  3. A case of schizophrenia like psychosis due to Fahr′s disease

    Directory of Open Access Journals (Sweden)

    Satyakam Mohapatra

    2016-01-01

    Full Text Available Fahr′s disease (FD is a rare idiopathic degenerative neurological disorder, which can be present in different heterogeneous manifestations and characterized by bilateral symmetrical cerebral calcification. We present a case of a 55-year-old male who presented with the psychotic feature, bilateral tremors of hand and bilateral symmetrical calcification of basal ganglia. Hence our case suggests that psychiatrists should evaluate the cases of psychosis thoroughly when the age of presentation is atypical, and they should consider the diagnosis of FD when psychosis presents with motor abnormalities.

  4. Brainstem lesion revealed by MRI in a case of Leigh's disease with respiratory failure

    International Nuclear Information System (INIS)

    This 6-year-old girl was admitted with insufficient involuntary breathing and generalized hypotonia. T2-weighted MR images showed bilateral symmetrical tubular hyperintense lesions in the medio-caudal part of the medulla oblongata that correlated well with demyelination and glosses in the regions of the reticular formation and the nucleus solitarius found at autopsy. The typical lesions of Leigh's disease in the basal ganglia were not present, which made the diagnosis uncertain prior to the histopathological findings. MRI was very helpful in deciding on further management of the patient. (orig.)

  5. Adult onset Hallervorden-Spatz disease with psychotic symptoms.

    Science.gov (United States)

    del Valle-López, Pilar; Pérez-García, Rosa; Sanguino-Andrés, Rosa; González-Pablos, Emilio

    2011-01-01

    Hallervorden-Spatz disease is a rare neurological disorder characterized by pyramidal and extrapyramidal manifestations, dysarthria and dementia. Its onset is usually in childhood and most patients have a fatal outcome in few years. A high percentage of cases are hereditary with a recessive autosomal pattern. In the majority of the patients reported, a mutation of the gene that encodes the pantothenate kinase (PANK2) located in the 20p13-p12.3 chromosome that causes iron storage in the basal ganglia of the brain has been found. Its diagnosis is based on clinical symptoms as well as specific MRI imaging findings. The most common psychiatric features are cognitive impairment as well as depressive symptoms. There are few documented cases with psychotic disorders. We present the case of a patient with late onset Hallervorden-Spatz disease and psychotic symptoms that preceded the development of neurological manifestations. The pathophysiology and the treatment of psychotic symptomatology are presented and discussed. Key words: Psicosis, Hallervorden-Spatz, late onset, Basal ganglia.

  6. Procedural learning in Parkinson's disease and cerebellar degeneration.

    Science.gov (United States)

    Pascual-Leone, A; Grafman, J; Clark, K; Stewart, M; Massaquoi, S; Lou, J S; Hallett, M

    1993-10-01

    We compared procedural learning, translation of procedural knowledge into declarative knowledge, and use of declarative knowledge in age-matched normal volunteers (n = 30), patients with Parkinson's disease (n = 20), and patients with cerebellar degeneration (n = 15) by using a serial reaction time task. Patients with Parkinson's disease achieved procedural knowledge and used declarative knowledge of the task to improve performance, but they required a larger number of repetitions of the task to translate procedural knowledge into declarative knowledge. Patients with cerebellar degeneration did not show performance improvement due to procedural learning, failed to achieve declarative knowledge, and showed limited use of declarative knowledge of the task to improve their performance. Both basal ganglia and cerebellum are involved in procedural learning, but their roles are different. The normal influence of the basal ganglia on the prefrontal cortex may be required for timely access of information to and from the working memory buffer, while the cerebellum may index and order events in the time domain and be therefore essential for any cognitive functions involving sequences. PMID:8215247

  7. Hedgehog pathway inhibitor in combination with radiation therapy for basal cell carcinomas of the head and neck. First clinical experience with vismodegib for locally advanced disease

    Energy Technology Data Exchange (ETDEWEB)

    Schulze, Bjoern; Roedel, Claus; Balermpas, Panagiotis [University Hospital Johann Wolfgang Goethe University, Department of Radiation Oncology, Frankfurt (Germany); Meissner, Markus [University Hospital Johann Wolfgang Goethe University, Department of Dermatology, Frankfurt (Germany); Ghanaati, Shahram [University Hospital Johann Wolfgang Goethe University, Department of Craniofacial and Plastic Surgery, Frankfurt (Germany); Burck, Iris [University Hospital Johann Wolfgang Goethe University, Department of Diagnostic and Interventional Radiology, Frankfurt (Germany)

    2016-01-15

    Definitive radiotherapy and vismodegib, an oral inhibitor of the hedgehog pathway, are both established treatment options for locally advanced basal cell carcinomas (BCC). Both have shown good results in local tumor control; however, the effects concerning advanced tumors are often not of a lasting nature and to date no systematic data about the combination of the two modalities are available. We retrospectively analyzed four patients who received vismodegib and radiotherapy in combination. Radiation doses varied between 50.4 Gy and 66.0 Gy. Three patients had recurrent BCC. One patient had locoregional lymph node involvement. Vismodegib was taken once a day (150 mg) during the entire time of irradiation and beyond upon instructions of the attending dermatologist. In three cases a persistent complete response was observed, in one case the tumor remained stable for approximately 6 months until further tumor progression was documented. The combined therapy was well tolerated in all cases. No exceptional side effects pointing at a drug-radiation interaction were observed. The combination of vismodegib and radiation seems feasible and the initial results are promising. In our cohort, there was no increase in unexpected side effects. Further research is needed to evaluate the significance of this combined therapy. (orig.) [German] Sowohl definitive Radiotherapie als auch Vismodegib, ein oraler Inhibitor der Hedgehog-Signalkaskade, sind etablierte Behandlungsoptionen fuer lokal fortgeschrittene Basalzellkarzinome (BCC). Beide Therapien zeigen fuer sich gute Ansprechraten, aber die lokale Tumorkontrolle ist oft nicht dauerhaft und bis heute existieren kaum Daten ueber eine Kombination der beiden Modalitaeten. Wir analysierten retrospektiv vier Patientenfaelle nach simultaner Applikation von Vismodegib und Bestrahlung. Die Bestrahlungsdosis variierte zwischen 50,4 Gy und 66,0 Gy. Drei der Patienten hatten ein rezidiviertes BCC. Ein Patient hatte einen befallenen regionalen

  8. Basal Gene Expression by Lung CD4+ T Cells in Chronic Obstructive Pulmonary Disease Identifies Independent Molecular Correlates of Airflow Obstruction and Emphysema Extent

    OpenAIRE

    Freeman, Christine M.; McCubbrey, Alexandra L; Crudgington, Sean; Nelson, Joshua; Martinez, Fernando J; Han, MeiLan K.; George R. Washko; Chensue, Stephen W.; Arenberg, Douglas A.; Meldrum, Catherine A.; McCloskey, Lisa; Curtis, Jeffrey L.

    2014-01-01

    Lung CD4+ T cells accumulate as chronic obstructive pulmonary disease (COPD) progresses, but their role in pathogenesis remains controversial. To address this controversy, we studied lung tissue from 53 subjects undergoing clinically-indicated resections, lung volume reduction, or transplant. Viable single-cell suspensions were analyzed by flow cytometry or underwent CD4+ T cell isolation, followed either by stimulation with anti-CD3 and cytokine/chemokine measurement, or by real-time PCR ana...

  9. Neurodevelopment. Parasympathetic ganglia derive from Schwann cell precursors.

    Science.gov (United States)

    Espinosa-Medina, I; Outin, E; Picard, C A; Chettouh, Z; Dymecki, S; Consalez, G G; Coppola, E; Brunet, J-F

    2014-07-01

    Neural crest cells migrate extensively and give rise to most of the peripheral nervous system, including sympathetic, parasympathetic, enteric, and dorsal root ganglia. We studied how parasympathetic ganglia form close to visceral organs and what their precursors are. We find that many cranial nerve-associated crest cells coexpress the pan-autonomic determinant Paired-like homeodomain 2b (Phox2b) together with markers of Schwann cell precursors. Some give rise to Schwann cells after down-regulation of PHOX2b. Others form parasympathetic ganglia after being guided to the site of ganglion formation by the nerves that carry preganglionic fibers, a parsimonious way of wiring the pathway. Thus, cranial Schwann cell precursors are the source of parasympathetic neurons during normal development. PMID:24925912

  10. Radiological assessment of Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Tschampa, Henriette J.; Urbach, Horst [University of Bonn, Department of Radiology, Bonn (Germany); Zerr, Inga [University of Goettingen, National Reference Center for TSE Surveillance at the Department of Neurology, Goettingen (Germany)

    2007-05-15

    Creutzfeldt-Jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. There is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. Additionally, it is of public interest to differentiate the sporadic form of the disease from the variant CJD type (vCJD), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (BSE). High signal in the striatum on T2-weighted, FLAIR and diffusion weighted (DW) MRI as well as cortical high signal in FLAIR and DW MRI are the classical findings in sCJD. The ''pulvinar sign'', defined as high signal in the pulvinar thalami that is brighter than potential additional high signal in the basal ganglia, is considered pathognomonic for vCJD. (orig.)

  11. Acute hypertensive encephalopathy with widespread small-vessel disease at MRI in a diabetic patient: pathogenetic hypotheses

    Energy Technology Data Exchange (ETDEWEB)

    Cotton, F. [Centre Hospitalier Lyon Sud, Department of Radiology, Pierre Benite (France); Universite Claude-Bernard Lyon-I, CREATIS, UMR CNRS (France); Universite Claude-Bernard Lyon-I, Laboratoire d' Anatomie, Laennec (France); Kamoun, S.; Rety-Jacob, F.; Tran-Minh, V.A. [Centre Hospitalier Lyon Sud, Department of Radiology, Pierre Benite (France); Nighoghossian, N. [Hopital Neurologique et Neurochirurgical, Department of Neurology, Bron (France); Universite Claude-Bernard Lyon-I, CREATIS, UMR CNRS (France); Hermier, M. [Hopital Neurologique et Neurochirurgical, Department of Neuroradiology and MRI, Bron (France); Universite Claude-Bernard Lyon-I, CREATIS, UMR CNRS (France)

    2005-08-01

    We report unusual magnetic resonance imaging (MRI) findings in a diabetic patient with neglected hypertension and hyperglycemia, presenting with seizures and coma. Outcome was fatal despite intensive care. The MRI findings included bilateral insular and temporo-occipital grey and white matter involvement, and numerous, scattered, lacunar-like lesions involving the peripheral and deep white matter, basal ganglia grey matter, and brainstem. Lesions had a low apparent diffusion coefficient, and some enhanced following contrast injection. Hypertensive encephalopathy with widespread and severe acute small-vessel disease was considered. Pathophysiology is discussed. (orig.)

  12. CT and MRI findings of Creutzfeldt-Jakob disease in the early stage. The usefulness of diffusion-weighted images

    Energy Technology Data Exchange (ETDEWEB)

    Ukisu, Ryutaro; Kushihashi, Tamio; Gokan, Takehiko [Showa Univ., Tokyo (Japan). School of Medicine] [and others

    2001-02-01

    To detect subtle CT and MRI features of Creutzfeldt-Jacob disease (CJD) in the early stage is important to prevent a human-to-human transmission. This study included 10 patients of CJD who underwent CT and/or MRI in its early stage. CT, T1- and T2-weighted MRI, DWI, and FLAIR images were obtained in 10, 6, 4, and 2 patients respectively. On DWI, abnormal hyperintensities were observed in both cerebral cortex, and in basal ganglia in all patients. On FLAIR images, abnormal hyperintensies were observed in one patient. Detection of abnormal intensities may be possible in the early stage of CJD using MRI, particularly with DWI. (author)

  13. Intramural ganglia in diverticular disease of the colon

    Science.gov (United States)

    Macbeth, W. A. A. G.; Hawthorne, J. H. R.

    1965-01-01

    Intramural plexuses were studied in 30 colons, and a plethora of ganglionic tissue was observed in specimens with diverticula when compared with a control series. This alteration in the ganglionic pattern is considered real rather than apparent; the changes are confined to the region of the colon where muscular hypertrophy is present. Images PMID:14247702

  14. I-123 IMP SPECT in Parkinson's disease

    International Nuclear Information System (INIS)

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

  15. COMPARATIVE ANATOMICAL STUDIES ABOUT CHICKEN SUB-BASAL CONNECTIONS

    Directory of Open Access Journals (Sweden)

    CARMEN BERGHES

    2013-07-01

    Full Text Available The studies aimed to describe the nervous formations from the base of the cranium in the hen and domestic duck. These clarifications are necessary in order to disclose some unknown facts regarding this region in the poultry species used preponderantly in laboratory studies of the aviary flu. The vegetative connections from the base of the skull have been studied on 10 poultry specimens, 5 hens and 5 ducks. The animals have been euthanatized using chloroform and a special dye has been injected through the heart in order to achieve a better differentiation of the nervous formations. Dissection was performed under a magnifying glass using instruments adequate to highly fine dissections. Photos and sketches of the dissected pieces have been taken. Nomina Anatomica (2003 was used to describe the observed formations.The studies showed that the cranial cervical ganglia around which is the sub-basal nervous tissue, is located on the border of the occipital hole, at the basis of the temporal pyramid, much deeper than in mammalians; it is better developed in the duck (3-4 mm than in the hen (1-2 mm; the cranial cervical ganglia has the shape of a globe in gallinaceans and it is long in shape in the ducks. A multitude of connecting branches were observed around the lymph node, linking it to the vague nerve, to the hypoglossal nerve, to the glossopharyngeal nerve and to the transversal paravertebral chain which is specific to poultry; an obvious branch detaches from the cranial pole, which is the sub-basal connective, while the cervical connective detaches from the caudal pole, connecting it to the cervical-thoracic lymph node.

  16. Diffusion-weighted MR imaging in biopsy-proven Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyo Cheol; Chang, Kee Hyun; Song In Chan; Lee, Sang Hyun; Kwon, Bae Ju; Han, Moon Hee; Kim, Sang Yun [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2001-12-01

    To compare conventional and diffusion-weighted MR imaging in terms of their depiction of the abnormalities occurring in Creutzfeldt-Jakob disease. We retrospectively analyzed the findings of conventional (T2-weighted and fluid-attenuated inversion recovery) and diffusion-weighted MR imaging in four patients with biopsy-proven Creutzfeldt-Jakob disease. The signal intensity of the lesion was classified by visual assessment as markedly high, slightly high, or isointense, relative to normal brain parenchyma. Both conventional and diffusion-weighted MR images demonstrated bilateral high signal intensity in the basal ganglia in all four patients. Cortical lesions were observed on diffusion-weighted MR images in all four, and on fluidattenuated inversion recovery MR images in one, but in no patient on T2-weighted images. Conventional MR images showed slightly high signal intensity in all lesions, while diffusion-weighted images showed markedly high signal intensity in most. Diffusion-weighted MR imaging is more sensitive than its conventional counterpart in the depiction of Creutzfeldt-Jakob disease, and permits better detection of the lesion in both the cerebral cortices and basal ganglia.

  17. Localized 1H-MR spectroscopy in moyamoya disease before and after revascularization surgery

    International Nuclear Information System (INIS)

    To evaluate, using localized proton magnetic resonance spectroscopy (1H-MRS), the cerebral metabolic change apparent after revascularization surgery in patients with moyamoya disease. Sixteen children with moyamoya disease and eight age-matched normal controls underwent MR imaging, MR angiography, conventional angiography, and 99mTc- ECD SPECT. Frontal white matter and the basal ganglia of both hemispheres were subjected to localized 1H-MRS, and after revascularization surgery, four patients underwent follow-up 1H-MRS. Decreased NAA/Cr ratios (1.35±0.14 in patients vs. 1.55±0.24 in controls) and Cho/Cr ratios (0.96±0.13 in patients vs. 1.10±0.11 in controls) were observed in frontal white matter. After revascularization surgery, NAA/Cr and Cho/Cr ratios in this region increased. In the basal ganglia, there is no abnormal metabolic ratios. Localized 1H-MRS revealed abnormal metabolic change in both hemispheres of children with moyamoya disease. Because of its non-invasive nature, 1H-MRS is potentially useful for the preoperative evaluation of metabolic abnormalities and their postoperative monitoring

  18. Elevated Basal Pre-infection CXCL10 in Plasma and in the Small Intestine after Infection Are Associated with More Rapid HIV/SIV Disease Onset

    Science.gov (United States)

    Ploquin, Mickaël J.; Casrouge, Armanda; Huot, Nicolas; Passaes, Caroline; Lécuroux, Camille; Essat, Asma; Boufassa, Faroudy; Jacquelin, Béatrice; Jochems, Simon P.; Petitjean, Gaël; Angin, Mathieu; Gärtner, Kathleen; Garcia-Tellez, Thalía; Booiman, Thijs; Boeser-Nunnink, Brigitte D.; Roques, Pierre; Saez-Cirion, Asier; Vaslin, Bruno; Dereudre-Bosquet, Nathalie; Barré-Sinoussi, Françoise; Ghislain, Mathilde; Rouzioux, Christine; Lambotte, Olivier; Albert, Matthew L.; Goujard, Cécile; Kootstra, Neeltje; Meyer, Laurence; Müller-Trutwin, Michaela C.

    2016-01-01

    Elevated blood CXCL10/IP-10 levels during primary HIV-1 infection (PHI) were described as an independent marker of rapid disease onset, more robust than peak viremia or CD4 cell nadir. IP-10 enhances the recruitment of CXCR3+ cells, which include major HIV-target cells, raising the question if it promotes the establishment of viral reservoirs. We analyzed data from four cohorts of HIV+ patients, allowing us to study IP-10 levels before infection (Amsterdam cohort), as well as during controlled and uncontrolled viremia (ANRS cohorts). We also addressed IP-10 expression levels with regards to lymphoid tissues (LT) and blood viral reservoirs in patients and non-human primates. Pre-existing elevated IP-10 levels but not sCD63 associated with rapid CD4 T-cell loss upon HIV-1 infection. During PHI, IP-10 levels and to a lesser level IL-18 correlated with cell-associated HIV DNA, while 26 other inflammatory soluble markers did not. IP-10 levels tended to differ between HIV controllers with detectable and undetectable viremia. IP-10 was increased in SIV-exposed aviremic macaques with detectable SIV DNA in tissues. IP-10 mRNA was produced at higher levels in the small intestine than in colon or rectum. Jejunal IP-10+ cells corresponded to numerous small and round CD68neg cells as well as to macrophages. Blood IP-10 response negatively correlated with RORC (Th17 marker) gene expression in the small intestine. CXCR3 expression was higher on memory CD4+ T cells than any other immune cells. CD4 T cells from chronically infected animals expressed extremely high levels of intra-cellular CXCR3 suggesting internalization after ligand recognition. Elevated systemic IP-10 levels before infection associated with rapid disease progression. Systemic IP-10 during PHI correlated with HIV DNA. IP-10 production was regionalized in the intestine during early SIV infection and CD68+ and CD68neg haematopoietic cells in the small intestine appeared to be the major source of IP-10. PMID:27509048

  19. P2X7 receptor of rat dorsal root ganglia is involved in the effect of moxibustion on visceral hyperalgesia

    OpenAIRE

    Liu, Shuangmei; Shi, Qingming; Zhu, Qicheng; Zou, Ting; Li, Guilin; Huang, An; Wu, Bing; Peng, Lichao; Song, Miaomiao; Wu, Qin; Xie, Qiuyu; Lin, Weijian; XIE, Wei; Wen, Shiyao; Zhang, Zhedong

    2014-01-01

    Irritable bowel syndrome (IBS) and inflammatory bowel disease often display visceral hypersensitivity. Visceral nociceptors after inflammatory stimulation generate afferent nerve impulses through dorsal root ganglia (DRG) transmitting to the central nervous system. ATP and its activated-purinergic 2X7 (P2X7) receptor play an important role in the transmission of nociceptive signal. Purinergic signaling is involved in the sensory transmission of visceral pain. Moxibustion is a therapy applying...

  20. Pain management in patients with Parkinson's disease: challenges and solutions

    Directory of Open Access Journals (Sweden)

    Skogar O

    2016-09-01

    Full Text Available Orjan Skogar,1,2 Johan Lokk2 1Academy for Health and Care (FUTURUM, Region Jönköping County, Jönköping, 2Department of Neurobiology, Care Sciences and Society, Karolinska Institutet, Huddinge, Stockholm, Sweden Abstract: This review focuses on the diagnosis and management of Parkinson-related pain which is one of the more frequently reported nonmotor symptoms in Parkinson’s disease (PD, which is the second most common neurodegenerative disease after Alzheimer’s disease. Pain is ranked high by patients as a troublesome symptom in all stages of the disease. In early-stage PD, pain is rated as the most bothersome symptom. Knowledge of the correct diagnosis of pain origin and possible methods of treatments for pain relief in PD is of great importance. The symptoms have a great negative impact on health-related quality of life. Separating PD-related pain from pain of other origins is an important challenge and can be characterized as “many syndromes under the same umbrella”. Among the different forms of PD-related pain, musculoskeletal pain is the most common form, accounting for 40%–90% of reported pain in PD patients. Augmentation by pathophysiological pathways other than those secondary to rigidity, tremor, or any of the other motor manifestations of the disease seems most probable. In PD, the basal ganglia process somatosensory information differently, and increased subjective pain sensitivity with lower electrical and heat-pain thresholds has been reported in PD patients. The mechanism is assumed to be diminished activity of the descending inhibitory control system of the basal ganglia. PD pain, like many of the nonmotor symptoms, remains underdiagnosed and, thus, poorly managed. A systematic collection of patient descriptions of type, quality, and duration of pain is, therefore, of utmost importance. Recent studies have validated new and more specific and dedicated pain scales for PD-related symptoms. Symptomatic treatments based

  1. Survey of Saccadic Parameters Using Videonystagmography in Patients with Idiopathic Parkinson's Disease and Normal Subjects

    Directory of Open Access Journals (Sweden)

    Reza Hosseinabadi

    2008-06-01

    Full Text Available Background and Aim: Patients with Parkinson’s disease manifest oculomotor abnormalities. This is the consequence of basal ganglia impairment. The most common abnormalities include increased saccade latency, hypometric saccades and decreased saccade velocity. The purpose of this study was comparison of saccadic parameters using videonystagmography in patients with idiopathic Parkinson’s disease and normal subjects.Materials and Methods: In this cross sectional study, saccadic movements were investigated in thirty patients with idiopathic Parkinson’s disease and thirty age matched subjects were 35-70 years old. Saccade latency, velocity and accuracy were quantitatively analyzed. Results: Results of this study indicated increased saccade latency, reduction of saccade velocity and accuracy in patients with Parkinson’s disease(P<0.001.Conclusion: This study showed that patients with Parkinson’s disease manifest saccadic deficits. This suggests dopaminergic control of these ocular movements.

  2. Cortical information flow in Parkinson's disease: a composite network/field model

    Directory of Open Access Journals (Sweden)

    Cliff C. Kerr

    2013-04-01

    Full Text Available The basal ganglia play a crucial role in the execution of movements, as demonstrated by the severe motor deficits that accompany Parkinson's disease (PD. Since motor commands originate in the cortex, an important question is how the basal ganglia influence cortical information flow, and how this influence becomes pathological in PD. To explore this, we developed a composite neuronal network/neural field model. The network model consisted of 4950 spiking neurons, divided into 15 excitatory and inhibitory cell populations in the thalamus and cortex. The field model consisted of the cortex, thalamus, striatum, subthalamic nucleus, and globus pallidus. Both models have been separately validated in previous work. Three field models were used: one with basal ganglia parameters based on data from healthy individuals, one based on data from individuals with PD, and one purely thalamocortical model. Spikes generated by these field models were then used to drive the network model. Compared to the network driven by the healthy model, the PD-driven network had lower firing rates, a shift in spectral power towards lower frequencies, and higher probability of bursting; each of these findings is consistent with empirical data on PD. In the healthy model, we found strong Granger causality in the beta and low gamma bands between cortical layers, but this was largely absent in the PD model. In particular, the reduction in Granger causality from the main "input" layer of the cortex (layer 4 to the main "output" layer (layer 5 was pronounced. This may account for symptoms of PD that seem to reflect deficits in information flow, such as bradykinesia. In general, these results demonstrate that the brain's large-scale oscillatory environment, represented here by the field model, strongly influences the information processing that occurs within its subnetworks. Hence, it may be preferable to drive spiking network models with physiologically realistic inputs rather than

  3. Modulation of Cortical-subcortical Networks in Parkinson’s Disease by Applied Field Effects

    Directory of Open Access Journals (Sweden)

    Christopher William Hess

    2013-09-01

    Full Text Available Studies suggest that endogenous field effects may play a role in neuronal oscillations and communication. Non-invasive transcranial electrical stimulation with low-intensity currents can also have direct effects on the underlying cortex as well as distant network effects. While Parkinson's disease (PD is amenable to invasive neuromodulation in the basal ganglia by deep brain stimulation, techniques of non-invasive neuromodulation like transcranial direct current stimulation (tDCS and transcranial alternating current stimulation (tACS are being investigated as possible therapies. tDCS and tACS have the potential to influence the abnormal cortical-subcortical network activity that occurs in PD through sub-threshold changes in cortical excitability or through entrainment or disruption of ongoing rhythmic cortical activity. This may allow for the targeting of specific features of the disease involving abnormal oscillatory activity, as well as the enhancement of potential cortical compensation for basal ganglia dysfunction and modulation of cortical plasticity in neurorehabilitation. However, little is currently known about how cortical stimulation will affect subcortical structures, the size of any effect, and the factors of stimulation that will influence these effects.

  4. Nevoid basal cell carcinoma syndrome; Naevoid Basalzellkarzinom-Syndrom

    Energy Technology Data Exchange (ETDEWEB)

    Grgic, A.; Heinrich, M.; Heckmann, M.; Kramann, B. [Universitaetsklinikum des Saarlandes, Homburg/Saar (Germany). Abt. fuer Diagnostische und Interventionelle Radiologie; Aliani, S. [Universitaetsklinikum des Saarlandes, Homburg/Saar (Germany). Klinik fuer Kinder- und Jugendmedizin; Dill-Mueller, D. [Universitaetsklinikum des Saarlandes, Homburg/Saar (Germany). Hautklinik und Poliklinik; Uder, M. [Erlange-Nuernberg Univ. (Germany). Inst. fuer Diagnostische Radiologie

    2005-07-01

    Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal-dominant disorder characterized by multiple basal cell carcinomas, jaw cysts, palmar/plantar pits, calcification of the falx cerebri, and spine and rib anomalies. The combination of clinical, imaging, and histological findings is helpful in identifying NBCCS patients. Imaging plays a crucial role in evaluation of these patients. We present a wide variety of clinical and radiological findings characteristic of this disease. (orig.)

  5. Cognitive-motor learning in Parkinson's disease.

    Science.gov (United States)

    Haaland, K Y; Harrington, D L; O'Brien, S; Hermanowicz, N

    1997-04-01

    Procedural learning deficits are common in Parkinson's disease (PD), but contradictory results have been reported in rotary pursuit learning. This article compared rotary pursuit learning in 2 nondemented PD groups and 2 normal control (NC) groups, using a between-subjects group design in which 3 rotation speeds were presented either randomly or in blocks. The pattern of learning differed between the randomized and the blocked conditions in the NC, but not in the PD groups. Learning was impaired in the PD group in the random condition only. Memory, visuospatial, or executive skills were not associated with the PD group's poorer learning in the randomized context. Results show that procedural learning deficits are not universal with basal ganglia abnormalities but rather depend on the specific cognitive requirements of the learning context. PMID:9110325

  6. Proton MR spectroscopy in three children with Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Aydin, Kubilay; Bakir, Baris; Terzibasioglu, Ege [Istanbul University, Neuroradiology Division, Department of Radiology, Istanbul (Turkey); Tatli, Burak; Ozmen, Meral [Istanbul University, Department of Paediatric Neurology, Istanbul (Turkey)

    2005-11-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM{sub 2} gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease. (orig.)

  7. Dysfunctions of the stomatognathic system and vocal aspects in Fahr disease: case report.

    Science.gov (United States)

    Santos, Karoline Weber dos; Fraga, Bruno Francisco de; Cardoso, Maria Cristina de Almeida Freitas

    2014-01-01

    The aim of this study is to report the case of a patient with Fahr's Disease in order to describe the main stomatognathic and vocal changes that can be found in individuals with this disease. In order to establish the diagnosis, an assessment of the conditions of orofacial motor system and speech production, as well the efficiency of swallowing, was realized. Based on these assessments, there were difficulties in coordinating and sustaining muscle during speech and presence of oropharyngeal dysphagia. Speech disorders found in Fahr's disease manifest themselves in complex and cover various aspects of phonological knowledge and the diseases that affect the basal ganglia have similar frames of speech-language disorders of the stomatognathic system, being able to present a picture of dysarthria.

  8. Patients with Parkinson's disease learn to control complex systems-an indication for intact implicit cognitive skill learning.

    Science.gov (United States)

    Witt, Karsten; Daniels, Christine; Daniel, Victoria; Schmitt-Eliassen, Julia; Volkmann, Jens; Deuschl, Günther

    2006-01-01

    Implicit memory and learning mechanisms are composed of multiple processes and systems. Previous studies demonstrated a basal ganglia involvement in purely cognitive tasks that form stimulus response habits by reinforcement learning such as implicit classification learning. We will test the basal ganglia influence on two cognitive implicit tasks previously described by Berry and Broadbent, the sugar production task and the personal interaction task. Furthermore, we will investigate the relationship between certain aspects of an executive dysfunction and implicit learning. To this end, we have tested 22 Parkinsonian patients and 22 age-matched controls on two implicit cognitive tasks, in which participants learned to control a complex system. They interacted with the system by choosing an input value and obtaining an output that was related in a complex manner to the input. The objective was to reach and maintain a specific target value across trials (dynamic system learning). The two tasks followed the same underlying complex rule but had different surface appearances. Subsequently, participants performed an executive test battery including the Stroop test, verbal fluency and the Wisconsin card sorting test (WCST). The results demonstrate intact implicit learning in patients, despite an executive dysfunction in the Parkinsonian group. They lead to the conclusion that the basal ganglia system affected in Parkinson's disease does not contribute to the implicit acquisition of a new cognitive skill. Furthermore, the Parkinsonian patients were able to reach a specific goal in an implicit learning context despite impaired goal directed behaviour in the WCST, a classic test of executive functions. These results demonstrate a functional independence of implicit cognitive skill learning and certain aspects of executive functions. PMID:16806313

  9. Identification of bladder and colon afferents in the nodose ganglia of male rats.

    Science.gov (United States)

    Herrity, April N; Rau, Kristofer K; Petruska, Jeffrey C; Stirling, David P; Hubscher, Charles H

    2014-11-01

    The sensory neurons innervating the urinary bladder and distal colon project to similar regions of the central nervous system and often are affected simultaneously by various diseases and disorders, including spinal cord injury. Anatomical and physiological commonalities between the two organs involve the participation of shared spinally derived pathways, allowing mechanisms of communication between the bladder and colon. Prior electrophysiological data from our laboratory suggest that the bladder also may receive sensory innervation from a nonspinal source through the vagus nerve, which innervates the distal colon as well. The present study therefore aimed to determine whether anatomical evidence exists for vagal innervation of the male rat urinary bladder and to assess whether those vagal afferents also innervate the colon. Additionally, the relative contribution to bladder and colon sensory innervation of spinal and vagal sources was determined. By using lipophilic tracers, neurons that innervated the bladder and colon in both the nodose ganglia (NG) and L6/S1 and L1/L2 dorsal root ganglia (DRG) were quantified. Some single vagal and spinal neurons provided dual innervation to both organs. The proportions of NG afferents labeled from the bladder did not differ from spinal afferents labeled from the bladder when considering the collective population of total neurons from either group. Our results demonstrate evidence for vagal innervation of the bladder and colon and suggest that dichotomizing vagal afferents may provide a neural mechanism for cross-talk between the organs. PMID:24845615

  10. The Design of Ubiquitous Learning System with Embedded Ganglia Agent

    Directory of Open Access Journals (Sweden)

    Fu-Chien Kao

    2011-05-01

    Full Text Available This research proposes a context-aware computing ubiquitous learning system architecture design. The system integrates data grid, the ability to perform context-awareness computing, and embedded Ganglia Agent design, structuring an architecture that is able to perform context awareness mobile network, creating a ubiquitous learning environment. The embedded Ganglia Agent could provide context information on system network traffic, the CPU load of the content server, and hard disk capacity, and utilize the information to balance the load of back-end content server, providing a flexible expandability mechanism for the back-end content server. The framework of the proposed ubiquitous learning system that has context-awareness computing ability is consisted of 3 major parts: Learning Management System (LMS, Learning Content Management System (LCMS and the embedded Ganglia Agent (EGA. LMS is responsible for managing the learners basic personal information and studying records, LCMS is responsible for the management and storage of back-end learning contents, and EGA is responsible for the management network traffic, CPU load and hard disk capacity. With the three, the load of the back-end content server could be balanced, offering a flexible mechanism for the expansion of the server.

  11. Nonsurgical Treatment Options for Basal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Mary H. Lien

    2011-01-01

    Full Text Available Basal cell carcinoma (BCC remains the most common form of nonmelanoma skin cancer (NMSC in Caucasians, with perhaps as many as 2 million new cases expected to occur in the United States in 2010. Many treatment options, including surgical interventions and nonsurgical alternatives, have been utilized to treat BCC. In this paper, two non-surgical options, imiquimod therapy and photodynamic therapy (PDT, will be discussed. Both modalities have demonstrated acceptable disease control rates, cosmetically superior outcomes, and short-term cost-effectiveness. Further studies evaluating long-term cure rates and long-term cost effectiveness of imiquimod therapy and PDT are needed.

  12. Dopaminergic receptor agents and the basal ganglia : pharmacological properties and interactions with the GABA-ergic system

    NARCIS (Netherlands)

    Timmerman, Wigerline

    1992-01-01

    In the present series of studies, attention was focussed particularly on dopaminergic D2 receptor compounds, with emphasis on the enantiomers of the potent and selective dopamine D2 receptor agonist N-0437. Drugs that display activity at D2 receptors are of great interest as potentially new therapeu

  13. In vivo labelling and axonal transport of monoamine oxidase in the rat basal ganglia using radioactive pargyline

    International Nuclear Information System (INIS)

    The enzyme monoamine oxidase was labelled in the rat striatum or substantia nigra with locally injected radioactive pargyline. The binding was prevented by a pretreatment with non-radioactive pargyline, or with a combination of clorgyline and deprenyl. Most of the MAO labelled with 3H-pargyline was of the B-type, but also some MAO-A was labelled, as shown in rats pretreated with clorgyline or deprenyl separately. Seven days after the injection of (3H)-pargyline into the striatum a significant labelling was observed in the substantia nigra. This labelling was clorgyline sensitive, indicating type A MAO, and was not present when striatal neurons were destroyed with kainic acid. Labelling of the striatum following 3H-pargyline injection into the substantia nigra was also less in kainate intoxicated striata. Damage of nigral dopamine neurons with 6-hydroxydopmaine did not influence the distribution of the label. Thus by using 3H-pargyline, specific labelling and axonal transport of type A MAO in striatal neurons projecting to the substantia nigra was demonstrated. (Author)

  14. [Obsessive-compulsive disorder, a new model of basal ganglia dysfunction? Elements from deep brain stimulation studies].

    Science.gov (United States)

    Haynes, W I A; Millet, B; Mallet, L

    2012-01-01

    Deep brain stimulation was first developed for movement disorders but is now being offered as a therapeutic alternative in severe psychiatric disorders after the failure of conventional therapies. One of such pathologies is obsessive-compulsive disorder. This disorder which associates intrusive thoughts (obsessions) and repetitive irrepressible rituals (compulsions) is characterized by a dysfunction of a cortico-subcortical loop. After having reviewed the pathophysiological evidence to show why deep brain stimulation was an interesting path to take for severe and resistant cases of obsessive-compulsive disorder, we will present the results of the different clinical trials. Finally, we will provide possible mechanisms for the effects of deep brain stimulation in this pathology. PMID:22898561

  15. GENSAT BAC Cre-recombinase driver lines to study the functional organization of cerebral cortical and basal ganglia circuits

    OpenAIRE

    Gerfen, Charles R.; Paletzki, Ronald; Heintz, Nathaniel

    2013-01-01

    Recent development of molecular genetic techniques are rapidly advancing understanding of the functional role of brain circuits in behavior. Critical to this approach is the ability to target specific neuron populations and circuits. The collection of over 250 BAC Cre-recombinase driver lines produced by the GENSAT project provides a resource for such studies. Here we provide characterization of GENSAT BAC-Cre driver lines with expression in specific neuroanatomical pathways within the cerebr...

  16. Integrating cortico-limbic-basal ganglia architectures for learning model-based and model-free navigation strategies

    Directory of Open Access Journals (Sweden)

    Mehdi eKhamassi

    2012-11-01

    Full Text Available Behaviour in spatial navigation is often organised into map-based (place-driven versus map-free (cue-driven strategies; behaviour in operant conditioning research is often organised into goal-directed versus habitual strategies. Here we attempt to unify the two. We review one powerful theory for distinct forms of learning during instrumental conditioning, namely model-based (maintaining a representation of the world and model-free (reacting to immediate stimuli learning algorithms. We extend these lines of argument to propose an alternative taxonomy for spatial navigation, showing how various previously identified strategies can be distinguished as model-based or model-free depending on the usage of information and not on the type of information (e.g. cue vs place. We argue that identifying model-free learning with dorsolateral striatum and model-based learning with dorsomedial striatum could reconcile numerous conflicting results in the spatial navigation literature. From this perspective, we further propose that the ventral striatum plays key roles in the model-building process. We propose that the core of the ventral striatum is positioned to learn the probability of action selection for every transition between states of the world. We further review suggestions that the ventral striatal core and shell are positioned to act as critics contributing to the computation of a reward prediction error for model-free and model-based systems, respectively.

  17. Nevoid basal cell carcinoma syndrome

    Directory of Open Access Journals (Sweden)

    Kannan Karthiga

    2006-01-01

    Full Text Available Binkley and Johnson first reported this syndrome in 1951. But it was in 1960, Gorlin-Goltz established the association of basal cell epithelioma, jaw cyst and bifid ribs, a combination which is now frequently known as Gorlin-Goltz syndrome as well as Nevoid Basal Cell Carcinoma Syndrome (NBCCS. NBCCS is inherited as an autosomal dominant trait with high penetrance and variable expressivity. NBCCS is characterized by variety of cutaneous, dental, osseous, opthalmic, neurologic and sexual abnormalities. One such case of Gorlin-Goltz syndrome is reported here with good illustrations.

  18. Expression pattern of cannabinoid receptor 1 in the basal ganglia of Parkinson disease rat model with levodopa-induced dyskinesia%大麻素CB1受体在左旋多巴诱导的异动症大鼠基底节的表达研究

    Institute of Scientific and Technical Information of China (English)

    马雅萍; 宋璐; 刘振国

    2010-01-01

    目的 观察大麻素CB1受体在长期左旋多巴治疗诱导的异动症(LID)大鼠模型基底节表达的特点,探讨LID与CB1受体表达变化的关系.方法 帕金森病(PD)模型大鼠接受左旋多巴腹腔注射21 d,建立LID大鼠模型.采用免疫组化和Western Blot方法检测基底节不同部位CB1受体表达.结果 经左旋多巴治疗的PD大鼠出现类似人类LID的行为学表现.免疫组化结果显示LID组纹状体CB1受体损伤侧与未损侧的累积吸光度(IOD)比值下降,而苍白球和黑质网状部该比值升高(均P<0.01);Western blot检测结果与免疫组化显示了相同变化趋势, LID组纹状体CB1受体损伤侧/未损侧条带密度比值降低(P<0.01).结论 长期左旋多巴治疗可引起基底节纹状体CB1受体表达下调,这种改变可能与LID的发生发展有关.

  19. [Extensive basal cell cancer of the scalp - case reports].

    Science.gov (United States)

    Olędzki, Szymon; Modrzejewski, Andrzej; Department Of Surgery And Emergency Nursing Pomeranian Medical University In Szczecin Poland, Ryszard

    2016-08-01

    Basal-cell canceris a slow growing, rarely metastasizes, locally malignant skin cancer. Patients with this neoplasm usually have excellent prognosis. Potentially, in some cases, a good prognosis cause a delay in therapy. Delay or withdrawal from treatment might lead to higher local extension of tumour with the destruction of the surrounding tissue. In this article we are presenting two patients with extensive basal cell cancer. The first patient underwent plastic surgery for extensive basal-cell carcinoma located in the parietal and temporal area. The second patient was observed due to recurrence of extensive basal cell carcinoma in the parietal region. Local advancement of the primary tumor could be a reason for the lack of radicality of surgery. Such advancement is rarely seen nowadays. The cases demonstrate the need for awareness about the possible severe course of the disease. PMID:27591446

  20. Risk factors for small-vessel disease revealed by magnetic resonance imaging of the brain

    Energy Technology Data Exchange (ETDEWEB)

    Kohriyama, Tatsuo; Yamaguchi, Shinya; Yamamura, Yasuhiro; Nakamura, Shigenobu [Hiroshima Univ. (Japan). School of Medicine; Tanaka, Eiji

    1996-02-01

    In total, 133 patients with asymptomatic or symptomatic cerebral infarction were randomly selected for the study (64 males, 69 females). Among them 91 patients had a history of symptomatic cerebral infarction, 46 patients of hypertension, and 28 patients of diabetes mellitus. The MRI scans were reviewed for areas with increased signal intensity on T2-weighted images. The grade of periventricular lesions, and the number of small infarctions in the subcortical white matter, basal ganglia and brain stem increased significantly with advancing age. It was thus reconfirmed that age is an important risk for demonstrating small-vessel disease on brain MRI. In addition, the degree of small-vessel disease on brain MRI was more extensive in patients with symptomatic cerebral infarction than with asymptomatic cerebral infarction. The detailed results suggest that small-vessel disease on brain MRI in patients with asymptomatic cerebral infarction might represent preclinical lesions for symptomatic cerebral infarction. The numbers of small infarctions in both the subcortical white matter and basal ganglia associated with advancing age, and histories of cerebrovascular accident and hypertension, suggest that common underlying mechanisms may exist in small-vessel disease in both the medullary arteries, which arise from cortical arteries, and perforating arteries. In the subcortical white matter, the number of patchy lesions was more strongly correlated with histories of hypertension and diabetes mellitus than was the number of spotty lesions, suggesting that the risk factors differed depending on the size of the lesions. The present study revealed that the degree of small-vessel disease on brain MRI was not correlated with the serum concentration of total cholesterol, triglyceride or HDL-cholesterol. The data thus indicate that the risk factors for small-vessel disease are distinct from those for large-vessel disease. (J.P.N.)

  1. Risk factors for small-vessel disease revealed by magnetic resonance imaging of the brain

    International Nuclear Information System (INIS)

    In total, 133 patients with asymptomatic or symptomatic cerebral infarction were randomly selected for the study (64 males, 69 females). Among them 91 patients had a history of symptomatic cerebral infarction, 46 patients of hypertension, and 28 patients of diabetes mellitus. The MRI scans were reviewed for areas with increased signal intensity on T2-weighted images. The grade of periventricular lesions, and the number of small infarctions in the subcortical white matter, basal ganglia and brain stem increased significantly with advancing age. It was thus reconfirmed that age is an important risk for demonstrating small-vessel disease on brain MRI. In addition, the degree of small-vessel disease on brain MRI was more extensive in patients with symptomatic cerebral infarction than with asymptomatic cerebral infarction. The detailed results suggest that small-vessel disease on brain MRI in patients with asymptomatic cerebral infarction might represent preclinical lesions for symptomatic cerebral infarction. The numbers of small infarctions in both the subcortical white matter and basal ganglia associated with advancing age, and histories of cerebrovascular accident and hypertension, suggest that common underlying mechanisms may exist in small-vessel disease in both the medullary arteries, which arise from cortical arteries, and perforating arteries. In the subcortical white matter, the number of patchy lesions was more strongly correlated with histories of hypertension and diabetes mellitus than was the number of spotty lesions, suggesting that the risk factors differed depending on the size of the lesions. The present study revealed that the degree of small-vessel disease on brain MRI was not correlated with the serum concentration of total cholesterol, triglyceride or HDL-cholesterol. The data thus indicate that the risk factors for small-vessel disease are distinct from those for large-vessel disease. (J.P.N.)

  2. Dopamine Does Not Appear to Affect Mental Rotation in Parkinson’s Disease

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    Gregory P. Crucian

    2014-10-01

    Full Text Available ObjectivePatients with Parkinson’s disease (PD often have deficits with mental rotation (MR. The neuropathological factors underlying these deficits, however, remain to be elucidated. One hypothesis suggests that dopamine depletion in nigro-striatal systems adversely influences MR. Another hypothesis suggests that deterioration of cortical (fronto-temporo-parietal basal ganglia networks that mediate this function are responsible for this deficit. The goal of this study was to test the dopamine hypothesis by determining if dopamine abstinence negatively influences MR performance. MethodsThirty three non-demented right-handed individuals with PD were assess for their ability to perform a pencil and paper MR test while “on” and “off” dopaminergic medications. Dopamine abstinence followed the typical overnight withdrawal procedures. ResultsNo differences in mental rotation abilities were found between “on” and “off” dopaminergic medications. ConclusionsThese results suggest that other neuropathological factors, such as cortical-basal ganglia neurodegeneration, or dysfunction of other neurotransmitters systems, might account for these cognitive deficits and future research will have to test these alternative hypotheses.

  3. Correlation between relaxometry and diffusion tensor imaging in the globus pallidus of Huntington's disease patients.

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    Michael Syka

    Full Text Available Huntington's disease (HD is an inherited neurodegenerative disorder with progressive impairment of motor, behavioral and cognitive functions. The clinical features of HD are closely related to the degeneration of the basal ganglia, predominantly the striatum. The main striatal output structure, the globus pallidus, strongly accumulates metalloprotein-bound iron, which was recently shown to influence the diffusion tensor scalar values. To test the hypothesis that this effect dominates in the iron-rich basal ganglia of HD patients, we examined the globus pallidus using DTI and T2 relaxometry sequences. Quantitative magnetic resonance (MR, clinical and genetic data (number of CAG repeats were obtained from 14 HD patients. MR parameters such as the T2 relaxation rate (RR, fractional anisotropy (FA and mean diffusivity (MD were analysed. A positive correlation was found between RR and FA (R2=0.84, between CAG and RR (R2=0.59 and between CAG and FA (R2=0.44. A negative correlation was observed between RR and MD (R2=0.66. A trend towards correlation between CAG and MD was noted. No correlation between MR and clinical parameters was found. Our results indicate that especially magnetic resonance FA measurements in the globus pallidus of HD patients may be strongly affected by metalloprotein-bound iron accumulation.

  4. Correlation between automated writing movements and striatal dopaminergic innervation in patients with Wilson's disease.

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    Hermann, Wieland; Eggers, Birk; Barthel, Henryk; Clark, Daniel; Villmann, Thomas; Hesse, Swen; Grahmann, Friedrich; Kühn, Hans-Jürgen; Sabri, Osama; Wagner, Armin

    2002-08-01

    Handwriting defects are an early sign of motor impairment in patients with Wilson's disease. The basal ganglia being the primary site of copper accumulation in the brain suggests a correlation with lesions in the nigrostiatal dopaminergic system. We have analysed and correlated striatal dopaminergic innervation using [(123)I]beta-CIT-SPECT and automated handwriting movements in 37 patients with Wilson's disease. There was a significant correlation of putaminal dopaminergic innervation with fine motor ability (p < 0,05 for NIV [number of inversion in velocity], NIA [number of inversion in acceleration], frequency). These data suggest that loss of dorsolateral striatal dopaminergic innervation has a pathophysiological function for decreased automated motor control in Wilson's disease. Furthermore analysis of automated handwriting movements could be useful for therapy monitoring and evaluation of striatal dopaminergic innervation. PMID:12195459

  5. HALLERVORDEN-SPATZ DISEASE - A RARE CASE REPORT - “Eye of th e Tiger” Sign

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    Mallikarjunaiah H. S.

    2013-08-01

    Full Text Available Background:Hallervorden-Spatz disease (HSD is a rare neurological disease characterized by progressivedegeneration of basal ganglia, globuspallidus and reticular part of the substantianigra, produced byironaccumulation. The defect has been found in the pantothenate kinase 2 (PANK2 producing gene locatedinchromosome 20p13-p12.3. Clinical presentations include dystonia, dysarthria, dysphasia, dementia, severemental retardation and severe movement disability may develop at later stages. Rare clinical features includerigidity, choreoathetosis, seizures, optic atrophy and pigmentary retinopathy. The characteristic MRI brainpattern of HSD shows the “eye of the tiger ” pattern. Treatment is symptomatic. We present the case of apatient, 19 years old boy with Hallervorden-Spatz disease who came to our physiotherapy department withfeatures of spasticity, dystonia and gait difficulty. He was diagnosed on the basis of clinical findings and typicalMRI brain of “eye of the tiger” pattern. His detailed evaluation was carried out and physiotherapy treatmentwas started.

  6. MRI in sporadic Creutzfeldt-Jakob disease: Correlation with clinical and neuropathological data

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    Urbach, H.; Solymosi, L. [Department of Neuroradiology, University of Wuerzburg (Germany); Klisch, J.; Brechtelsbauer, D. [Department of Neuroradiology, University of Bonn, Bonn (Germany); Wolf, H.K. [Department of Neuropathology, University of Bonn, Bonn (Germany); Gass, S. [Department of Neurology, University of Bonn, Bonn (Germany)

    1998-02-01

    To ascertain whether increased grey matter signal intensity on T2-weighted images in patients with sporadic Creutzfeldt-Jakob disease (CJD) corresponds to the stage and severity of this disease, we correlated MRI findings in four of our own and previously reported patients with sporadic CJD with the clinical variants, neuropathological changes at autopsy, duration of the disease and survival time after MRI examination. Of 15 patients with the extrapyramidal type of CJD, 10 showed increased signal in the basal ganglia on T2-weighted images. One of seven patients with the Heidenhain variant had increased signal in the occipital cortex. Patients without increased grey matter signal intensity had a longer overall duration of CJD (P = 0.035). Although the interval between onset of neurological symptoms and MRI was not different, patients without increased grey matter signal also survived longer after MRI examination (P = 0.022). (orig.) With 5 figs., 2 tabs., 23 refs.

  7. Error processing in Huntington's disease.

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    Christian Beste

    Full Text Available BACKGROUND: Huntington's disease (HD is a genetic disorder expressed by a degeneration of the basal ganglia inter alia accompanied with dopaminergic alterations. These dopaminergic alterations are related to genetic factors i.e., CAG-repeat expansion. The error (related negativity (Ne/ERN, a cognitive event-related potential related to performance monitoring, is generated in the anterior cingulate cortex (ACC and supposed to depend on the dopaminergic system. The Ne is reduced in Parkinson's Disease (PD. Due to a dopaminergic deficit in HD, a reduction of the Ne is also likely. Furthermore it is assumed that movement dysfunction emerges as a consequence of dysfunctional error-feedback processing. Since dopaminergic alterations are related to the CAG-repeat, a Ne reduction may furthermore also be related to the genetic disease load. METHODOLOGY/PRINCIPLE FINDINGS: We assessed the error negativity (Ne in a speeded reaction task under consideration of the underlying genetic abnormalities. HD patients showed a specific reduction in the Ne, which suggests impaired error processing in these patients. Furthermore, the Ne was closely related to CAG-repeat expansion. CONCLUSIONS/SIGNIFICANCE: The reduction of the Ne is likely to be an effect of the dopaminergic pathology. The result resembles findings in Parkinson's Disease. As such the Ne might be a measure for the integrity of striatal dopaminergic output function. The relation to the CAG-repeat expansion indicates that the Ne could serve as a gene-associated "cognitive" biomarker in HD.