1/7. motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration.The frontotemporal dementias are a group of relatively new and evolving clinical and pathologic entities. The predominant frontal-temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech. The motor neuron disease-inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin-immunoreactive inclusions with the complete absence of tau immunoreactivity. We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia. His premorbid diagnosis was cortical-basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/7. Corticobasal ganglionic degeneration with Balint's syndrome.Corticobasal ganglionic degeneration (CBGD) is a neurodegenerative dementia characterized by asymmetric parkinsonism, ideomotor apraxia, myoclonus, dystonia, and the alien hand syndrome. This report describes a patient with CBGD who developed Balint's syndrome with simultanagnosia, oculomotor apraxia, and optic ataxia.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
3/7. Post-stroke violent adventitial movement responsive to levo-dopa/carbi-dopa therapy.Previous reports in the literature concerning cerebrovascular accident have illustrated cases of post-stroke tremor. Treatments of these studies have varied. Trials of levo-dopa have been reported in two such cases. This case study reports on a case of a patient with a left thalamic, left superior cerebellar artery infarction with a lacunar infarction in the basal ganglia. The patient developed a violent tremor/movement disorder which was unresponsive to haloperidol. With this failure, and with the evidence of a basal ganglion lesion, levo-dopa/carbi-dopa was introduced as an intervention. The amplitude of the tremor was dramatically reduced, with protective devices removed, and with complete cessation of the tremor at rest. The medication was withdrawn and reintroduced with a reduction and subsequent resolution of the symptoms. A discussion of the previous studies of movement disorder with cerebrovascular accident is included.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
4/7. The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients.Acute extrapyramidal movement disorders have rarely been reported in uremic patients. We had previously presented three cases of acute movement disorders with bilateral basal ganglia lesions in uremia, and had proposed that it is not a rare condition. The objective of this study is to establish a more accurate clinical profile of this rarely described clinical syndrome, and to call attention to its common occurrence. We prospectively studied six patients we encountered from March 1996 to June 2001. We also reviewed the clinical records of a large population of uremic patients and identified six more cases. The clinical manifestations, laboratory findings, neuroimages, and clinical outcomes of these 12 patients were analyzed. When possible, each patient was followed up to the present time. Twelve patients had acute onset of movement disorders and bilateral basal ganglia lesions. All of the patients were diabetic. They had acute-onset Parkinsonism or dyskinesias, together with various symptoms such as consciousness disturbance, dysarthria, dysphagia, or ataxia. The main laboratory test results of abnormalities consisted of elevated blood urea nitrogen, creatinine, and metabolic acidosis. They had uniform neuroimaging findings of symmetrical bilateral basal ganglion changes. These changes regressed or disappeared during follow-up. The clinical prognoses were diverse. We believe that this group of patients represents a well-demarcated clinical syndrome, which is not uncommon but has previously been rarely addressed. The underlying mechanism of such lesions may be associated with metabolic, as well as vascular factors.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
5/7. Cerebral neurocytoma without ganglion cell differentiation.Neurocytomas are rare neuronal tumours, and are defined as tumours comprising cells with neuronal differentiation; they commonly arise in the ventricles. We report a case of a cerebral neurocytoma in a young man who presented with hemiparesis. The tumour was a radiologically a well-circumscribed large cyst with solid mass; the histopathology showed a well-differentiated lesion comprising uniform, round cells with perinuclear halos in a neuropil background, and immunohistochemically positive for neuronal markers (synaptophysin, neuron-specific enolase and neurofilaments). We suggest that cerebral neurocytomas should be considered among the uncommon causes of a large intra-axial cystic mass with a solid component in young adults.- - - - - - - - - - ranking = 0.8keywords = ganglion (Clic here for more details about this article) |
6/7. movement disorders associated with chronic GM2 gangliosidosis. Case report and review of the literature.A 24-year-old man presented with dystonia, dementia, amyotrophy, choreoathetosis, and ataxia. Partial hexosaminidase a deficiency was documented in serum and leukocytes and confirmed by rectal biopsy with ganglion cells containing membranous cytoplasmic bodies. A brief review of the literature reveals that tremor, dystonia and choreoathetosis are common but neglected symptoms associated with chronic GM2 gangliosidosis.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
7/7. Hypometria with hemispatial and limb motor neglect.A patient with a haemorrhage in the right mesial frontal lobe exhibited hypokinesia, bradykinesia and a hypometric movement disorder of his contralateral limbs. This movement disorder was characterized by reduced amplitude of otherwise normally formed movements (hypometria). In addition, the left limb hypokinesia improved with placement of the left forelimb into right hemispace (hemispatial motor neglect). We postulate that the hypokinesia, bradykinesia and hypometria were induced by reduction in activation of forebrain dopaminergic and basal ganglion motor systems, which in conjunction with the supplementary motor area are critical for setting the activational level for motor output.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |