Cases reported "Basal Ganglia Diseases"

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1/59. magnetic resonance imaging findings in corticobasal degeneration.

    Two women (patient 1, 77 years old, and patient 2, 63 years old) with strong clinical evidence for corticobasal degeneration (CBD) are presented. Patient 2 was in an early stage of the disease with only a mild disability of her left hand. In addition to the clinical characteristics, both patients presented the typical cortical reflex myoclonus. magnetic resonance imaging studies for both patients revealed nearly identical hyperintense lesions somatotopic from the left-hand primary motor cortex (M1), extending to the midline and possibly supplementary motor area (SMA) in patient 2. To our knowledge, this has not been previously described in patients with CBD. These lesions may play a role in the etiology and the development of CBD with involvement of the M1 and may correspond to the underlying pathology of demyelination or gliosis.
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keywords = cortex
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2/59. Dyspraxia in a patient with corticobasal degeneration: the role of visual and tactile inputs to action.

    OBJECTIVES: To investigate the roles of visual and tactile information in a dyspraxic patient with corticobasal degeneration (CBD) who showed dramatic facilitation in miming the use of a tool or object when he was given a tool to manipulate; and to study the nature of the praxic and neuropsychological deficits in CBD. methods: The subject had clinically diagnosed CBD, and exhibited alien limb behaviour and striking ideomotor dyspraxia. General neuropsychological evaluation focused on constructional and visuospatial abilities, calculation, verbal fluency, episodic and semantic memory, plus spelling and writing because impairments in this domain were presenting complaints. Four experiments assessed the roles of visual and tactile information in the facilitation of motor performance by tools. Experiment 1 evaluated the patient's performance of six limb transitive actions under six conditions: (1) after he described the relevant tool from memory, (2) after he was shown a line drawing of the tool, (3) after he was shown a real exemplar of the tool, (4) after he watched the experimenter perform the action, (5) while he was holding the tool, and (6) immediately after he had performed the action with the tool but with the tool removed from his grasp. Experiment 2 evaluated the use of the same six tools when the patient had tactile but no visual information (while he was blindfolded). Experiments 3 and 4 assessed performance of actions appropriate to the same six tools when the patient had either neutral or inappropriate tactile feedback-that is, while he was holding a non-tool object or a different tool. RESULTS: Miming of tool use was not facilitated by visual input; moreover, lack of visual information in the blindfolded condition did not reduce performance. The principal positive finding was a dramatic facilitation of the patient's ability to demonstrate object use when he was holding either the appropriate tool or a neutral object. Tools inappropriate to the requested action produced involuntary performance of the stimulus relevant action. CONCLUSIONS: Tactile stimulation was paramount in the facilitation of motor performance in tool use by this patient with CBD. This outcome suggests that tactile information should be included in models which hypothesise modality specific inputs to the action production system. Significant impairments in spelling and letter production that have not previously been reported in CBD have also been documented.
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keywords = visual
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3/59. Case report of pallido-pyramidal disease with supplementary motor area involvement.

    An C-flumazenil positron emission tomography (PET) study in a patient with pallido-pyramidal disease revealed a marked decrease in benzodiazepine-receptor density in the precentral gyrus cortex and the mesial frontal cortex. We suggest that, in addition to dysfunction of basal ganglia-dependent systems, degeneration of the supplementary motor area could also be involved in the patient's bradykinesia.
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keywords = cortex
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4/59. Involuntary hand levitation associated with parietal damage: another alien hand syndrome.

    The alien hand syndrome (AHS) usually consists of an autonomous motor activity perceived as an involuntary and purposeful movement, with a feeling of foreignness of the involved limb, commonly associated with a failure to recognise ownership of the limb in the absence of visual clues. It has been described in association to lesions of the frontal lobes and corpus callosum. However, parietal damage can promote an involuntary, but purposeless, hand levitation, which, sometimes, resembles AHS. In the present study, four patients (cortico-basal ganglionic degeneration - n=2; Alzheimer's disease - n=1 and parietal stroke - n=1) who developed alien hand motor behaviour and whose CT, MRI and/or SPECT have disclosed a major contralateral parietal damage or dysfunction are described. These results reinforce the idea that parietal lobe lesions may also play a role in some patients with purposeless involuntary limb levitation, which is different from the classic forms of AHS.
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keywords = visual
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5/59. Unilateral periodic limb movements during sleep in corticobasal degeneration.

    Periodic limb movements in sleep (PLM) have been associated with several degenerative disorders and other focal or diffuse diseases. We present a patient with clinical diagnosis of corticobasal degeneration and sleep complaints, in whom video recording and polygraphic study confirmed the presence of right PLM. The unilaterality of the movements and the positron emission tomography findings (hypometabolism in the left frontoparietal, basal ganglia, and thalamic areas) suggest that the loss of inhibitory descending central pathways, with origin in the cortex or basal ganglia, may be involved in the pathogenesis of PLM.
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keywords = cortex
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6/59. Corticobasal degeneration and frontotemporal dementia presentations in a kindred with nonspecific histopathology.

    We report the clinical, neuropsychological, electroencephalographic and radiologic findings in a kindred with varying clinical presentations of a neurodegenerative disorder. Postmortem examination of one member with clinically suspected corticobasal degeneration (CBD) revealed nonspecific histopathology maximally involving the frontoparietal cortex with negligible degenerative changes in the basal ganglia and substantia nigra. The findings in this and other kindreds demonstrate that (1) similar findings on ancillary testing can occur in relatives presumably suffering from the same pathophysiologic process despite dissimilar clinical presentations, (2) the 'CBD syndrome' is not specific for CBD, (3) extrapyramidal dysfunction can exist in the absence of appreciable basal ganglia and nigral degeneration, (4) nonspecific histopathology can underlie familial focal/asymmetric cortical degeneration syndromes and (5) many of the findings in CBD are comparable to those reported in frontotemporal dementia.
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7/59. Distribution of basal ganglia lesions in diffuse neurofibrillary tangles with calcification: a clinicopathological study of five autopsy cases.

    We investigated five Japanese autopsy cases of diffuse neurofibrillary tangles with calcification (DNTC), both clinically and pathologically, and examined the degree and distribution of the basal ganglia lesions, especially in the amygdala, striatum, pallidum, and substantia nigra. The lesions in the amygdala, striatum, and pallidum were classified into three categories (mild, moderate, and severe). The lesions in the substantia nigra were qualitatively judged, compared with normal controls. Severe dementia was observed in four cases neuropathologically showing pronounced neuronal loss in the cerebral cortex, but one case without neuronal loss in the cerebral cortex showed mild memory disturbance. Extrapyramidal signs were evident in three cases. Obvious neuronal loss in the substantia nigra with the presence of lewy bodies was noticed in four cases. basal ganglia lesions in all five cases were uniform: the amygdala showed severe to moderate lesions, the caudate nucleus moderate to slight lesions, and the putamen and pallidum slight lesions to normal. Furthermore, the lesions in the amygdala were more prominent in the basolateral group than in the corticomedial group, inconsistent with those in the amygdala of Alzheimer's disease. Moderate lesions were evident in the basolateral group of the amygdala in the case without neuronal loss in the cerebral cortex. In DNTC, the degree and distribution of the basal ganglia lesions, except for nigral lesions, were analogous to those found in Pick's disease with Pick bodies. These clinicopathological findings may contribute to the elucidation of the clinicopathological hallmarks in this disorder.
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keywords = cortex
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8/59. Macro-aneurysm in the basal ganglia region.

    A 59-year-old female patient suddenly developed vomiting and gait disturbances followed by decreasing consciousness. CT scans revealed a hemorrhage within the left basal ganglia region with rupture into the ventricles and consecutive hydrocephalus. On angiography an aneurysm in the region of the caput nuclei caudati was shown to be the source of the bleeding. On repeat-angiography 4 months later the aneurysm was no longer visualized, probably due to thrombosis. This is an extraordinary case of a basal ganglia aneurysm comparable with the aneurysms of Willis' circle, but located in a region where generally microaneurysms--mostly combined with hypertension or moyamoya disease--can be found.
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ranking = 0.017284014788733
keywords = visual
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9/59. basal ganglia involvement in a child with cat-scratch disease.

    Catch-scratch disease (CSD) is a self-limiting zoonotic illness. Encephalopathy is the most common neurologic manifestation of CSD. neuroimaging is usually normal with occasional abnormalities reported in children involving the cerebral cortex and thalamus but not previously described in the basal ganglia. Here we report a 7-year-old boy with CSD encephalopathy who developed choreoathetosis with high intensity changes in basal ganglia on magnetic resonance imaging scan.
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keywords = cortex
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10/59. Focal dystonia secondary to cavernous angioma of the basal ganglia: case report and review of the literature.

    The case of a young woman with focal dystonia of the hand due to a cavernous angioma of the basal ganglia is presented. The lesion involved the anterior third of the lentiform nucleus and a large portion of white matter anterior to this nucleus and lateral to the head of the caudate, as shown by magnetic resonance imaging; it was completely removed through a computed tomography-assisted stereotactic craniotomy by microsurgical technique, resulting in the cure of the patient. These facts support the pathophysiological hypothesis of a disruption of the striatopallidothalamic projection to the premotor cortex as the cause of symptomatic dystonia. A review of the reported cases of cavernous angiomas of the deep cerebral gray nuclei shows that this is the first case of cavernous angioma associated with movement disorder.
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