21/36. Delayed choreoathetosis following acute carbon monoxide poisoning.Ten days after accidental exposure to carbon monoxide, a 17-year-old youth developed transitory choreoathetosis of both arms, face, and neck, with moderate dysarthria. CT revealed symmetric bilateral infarction in the head of the caudate nucleus, the putamen, and the small parts of the anterolateral globus pallidus.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
22/36. Further observations showing the impact of the probenecid test in movement disorders.Two patients with movement disorders are described and neurochemically studied. The first subject concerns a central anticholinergic syndrome in a 83 years old man treated with l-dopa and amantadine for red nucleus tremor. The second one is an association of parkinsonism and chorea in a 76 years old woman presenting with FAHR's syndrome. In both cases, CSF HVA levels were measured before and after oral probenecid load. The observations confirm the usefulness of this neurochemical strategy in order to classify aging patients with parkinsonism into dopasensitive and doparesistant groups.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
23/36. Hallervorden-Spatz syndrome restricted to the pallidal nuclei.The case history is presented of a man who died at the age of 38 years and had been suffering from severe torsion dystonia and a hypokinetic-rigid motor disturbance since the age of 10. The pathological findings were isolated pallidal degeneration with demyelination and moderate neuronal loss, iron pigment accumulation and spheroid bodies in both pallidal nuclei. In addition the zona reticularis of the substantia nigra was hypoplastic and not affected. The red nucleus, the dentate nucleus and the zona reticularis of the substantia nigra showed iron depigmentation. The rare condition of non-familial Hallervorden-Spatz syndrome, without involvement of the zona reticularis and the cerebral cortex, was considered to be the diagnosis.- - - - - - - - - - ranking = 2keywords = nucleus (Clic here for more details about this article) |
24/36. Calcified basal ganglionic mass 12 years after radiation therapy for medulloblastoma.A patient treated 12 years previously with an operation and radiation therapy for a medulloblastoma developed weakness of the left hand and perivascular calcification involving the right internal capsule and caudate nucleus. These findings are considered possible long-term complications of the radiation therapy.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
25/36. Hemi-dystonia secondary to localised basal ganglia tumour.An 8-year-old boy with an 18 month history of left limb hemi-dystonia due to a right lenticular nucleus astrocytoma originating in the putamen is reported. Subsequent neuropathological study demonstrated that the tumour was mainly localised to the right lenticular nucleus, with cystic necrosis in the infero-lateral putamen. Solid tumour also infiltrated the right hypothalamus, the anterior commisure and the optic chiasm, and there was perivascular spread into the globus pallidus, internal capsule and roof of the right lateral ventricle. This case, and the few other published reports of symptomatic dystonia due to focal brain lesions verified pathologically, indicate that damage to the lenticular nucleus, and to the putamen in particular, can cause limb dystonia in man.- - - - - - - - - - ranking = 3keywords = nucleus (Clic here for more details about this article) |
26/36. Hemichorea-hemiballismus caused by lacunar infarction in the basal ganglia. A case report.Unilateral hemichorea-hemiballismus developed suddenly in a 60-year-old man. Computed tomography revealed a small area of low density in the region of the left anterior limb of the internal capsule and the head of the caudate nucleus which was consistent with a lacunar infarction. haloperidol gradually abolished the symptoms within 7 days of their onset. No further choreoballistic movements were experienced. This report reveals that ballistic movements may occasionally be caused by lesions lying some distance from the subthalamic nucleus of Luys.- - - - - - - - - - ranking = 14.354854757571keywords = subthalamic nucleus, subthalamic, nucleus (Clic here for more details about this article) |
27/36. Autosomal recessive spino-olivo-cerebellar degeneration without ataxia.Five adult siblings from a sibship of ten suffering from an external ophthalmoplegia with a spastic paraplegia are reported. In addition, optic nerve atrophy was present in three of the patients and dementia in two; extrapyramidal signs and cerebellar ataxia were found only in one patient. Contrary to earlier studies of patients with comparable neurological signs the pattern of inheritance was autosomal recessive. Neuropathological investigation of the index case, who had never shown ataxia, nevertheless showed demyelination of the spinocerebellar and the olivocerebellar pathways, and also a severe loss of purkinje cells, of cells in Clarke's column and in the inferior olives. The dentate nucleus was severely gliotic but showed no cell loss. Earlier neuropathological investigations of this disorder, but with an autosomal dominant heredity, were incomplete. It is concluded that the five siblings of this family have a unique autosomal recessive disorder, which should be considered a distinct entity.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
28/36. Paraballism caused by bilateral hemorrhagic infarction in basal ganglia.Bilateral ballism developed gradually in a 75-year-old woman after head trauma. Computerized tomography revealed bilateral hemorrhagic infarction in the head of the caudate nucleus and the putamen. haloperidol abolished the symptoms completely within 2 days. After 18 days, haloperidol was gradually withdrawal over 16 days. The patient remained without ballistic movements thereafter.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
29/36. Joseph disease: a multisystem degenerative disorder of the nervous system.We studied Joseph disease clinically and pathologically in two patients of Portuguese ancestry, but from different families. We found involvement of spinocerebellar tracts, Clarke's column, anterior horn cells, motor cranial nerve nuclei, and substantia nigra. One patient also had pallidosubthalamic and pontocerebellar degeneration with normal inferior olives. The second patient, a Joseph family member, had nerve cell loss in the subthalamic nucleus. The neostriatum appeared normal in both cases. The pigmented nuclei contained a few lewy bodies. The almost identical pathology in two families support the hypothesis that Joseph disease is a genetic entity.- - - - - - - - - - ranking = 15.772447071752keywords = subthalamic nucleus, subthalamic, nucleus (Clic here for more details about this article) |
30/36. Corticobasal degeneration: a disease with widespread appearance of abnormal tau and neurofibrillary tangles, and its relation to progressive supranuclear palsy.The neuropathological findings, including immunohistochemistry and electron microscopy, of two patients with clinical findings consistent with corticobasal degeneration (CBD) are reported. Both patients showed degeneration of the precentral cortex, the substantia nigra, the pallidum, and the thalamus. Many ballooned neurons were seen in the cerebral cortex, and argentophilic, skein-like inclusions suggesting neurofibrillary tangles (NFTs) were found in the brain stem and precentral cortex in patient 1. In contrast, patient 2 clearly showed NFTs in the brain stem and dentate nucleus which were indistinguishable from those seen in progressive supranuclear palsy (PSP), while only a few ballooned neurons were found in the cerebral cortex. Gallyas silver stain showed many argentophilic inclusions suggesting NFTs in the brain stem, subcortical nuclei, and cerebral cortex in both patients. immunohistochemistry for tau showed tau-positive neurons in the cerebral cortex, brain stem, subcortical nuclei and spinal cord, and tau-positive glial cells were seen in the cerebral cortex, white matter and subcortical nuclei, and thread-like structures were seen in the cerebral cortex and white matter. Electron microscopy of the brain stem showed NFTs consisting of paired helical filaments in patient 1, and paired helical filaments and straight tubules in patient 2. Immunoelectron microscopy revealed parallel tau-positive filaments in the cerebral cortex in patient 1. From the two patients, the wide-spread appearance of abnormal tau and NFTs is one of the essential pathological features in CBD, and it also appears that CBD and PSP have some common underlying pathological processes. Patient 2 is closer to PSP than patient 1 and suggests CBD would link to PSP.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
| <- Previous || Next -> |