1/28. Cortical potentials preceding voluntary finger movement in patients with focal cerebellar lesion.OBJECTIVE: To elucidate influences of the dentate nucleus on the generation of the related cortical potentials (MRCPs) preceding voluntary movement, Bereitschaftspotential (BP) and negative slope (NS). DESIGN AND methods: MRCPs preceding self-paced voluntary finger movement were recorded in 5 cases with localized cerebellar lesions due to stroke or tumor. Comparing 3 of them involving the dentate nucleus and 2 others sparing it, as judged from CT or MRI findings. RESULTS: BP and NS preceding voluntary finger movement ipsilateral to the lesion were absent or markedly reduced in amplitude in those 3 cases with the dentate nucleus lesion, whereas those negative components were present in the 2 cases with no evidence of dentate lesion. CONCLUSION: The dentate nucleus has a facilitatory effect on the generation of BP and NS.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
2/28. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis.Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination.- - - - - - - - - - ranking = 6.6001533148985keywords = basal ganglion, ganglion (Clic here for more details about this article) |
3/28. Palatal myoclonus--a case report.Palatal myoclonus is usually due to a brainstem or cerebellar lesion disrupting the dentato-rubro-olivary pathway. Rarely it may be caused by a cortical lesion. The precipitating factor in 70% of all cases is an infarct. We describe an unusual case of a patient with palatal myoclonus who had an old ipsilateral cerebellar infarct and a new contralateral subcortical (corona radiata) infarct. We postulate that the new infarct caused disinhibition of the old cerebellar infarct, resulting in palatal myoclonus. magnetic resonance imaging (MRI) of the brain did not show any hypertrophy of the inferior olivary nucleus. Her myoclonus proved refractory to clonazepam, valproate and phenytoin.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
4/28. Cerebellar dysfunction in chronic toluene abuse: beneficial response to amantadine hydrochloride.CASE REPORT: A 21-year-old man who had sniffed toluene since the age of 13 presented with a 4-year history of progressive cerebellar dysfunction and visual deterioration. The patient's condition did not improve despite 5 months of abstinence. magnetic resonance imaging revealed cerebral atrophy and hypointensity signals in the white matter and bilaterally in the globus pallidus, thalamus, red nucleus, and substantia nigra. amantadine hydrochloride therapy (100 mg/d, then 200 mg/d) resulted in dramatic improvement of his cerebellar and visual symptoms.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
5/28. Cerebellar cortical degeneration disrupts discrimination learning but not delay or trace classical eyeblink conditioning.The authors investigated classical eyeblink conditioning in a relatively rare patient, B.R., with extensive cerebellar cortical atrophy and marked sparing of the dentate nucleus. Patient B.R.'s ability to acquire and extinguish simple associations (delay and trace conditioning tasks) as well as her ability to acquire more complex associations (temporal and simple discrimination tasks) were examined. There are 2 primary findings from this study. First, B.R. showed normal acquisition and extinction in delay and trace conditioning. Second, she demonstrated a complete inability to learn associative discriminations, even in the case of a simple 2-tone discrimination within the context of a delay paradigm. The latter finding was unexpected because of the sparing of her deep cerebellar nuclei. These data suggest that the cerebellar cortex, or pathways traversing cerebellar cortex, play an important role in classical eyeblink discrimination learning.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
6/28. Mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes (MELAS) with prominent degeneration of the intestinal wall and cactus-like cerebellar pathology.A 67-year-old woman had frequent subacute ileus, hearing difficulty, muscle atrophy and stroke-like episodes. Computed tomography revealed multiple low-density areas, which did not correlate with the vascular supply, in the cerebral cortex. She had metabolic disturbance comprising lactic acidosis and elevated pyruvate level. Her skeletal muscle biopsy specimen showed ragged-red fibers, and mitochondrial dna analysis revealed a point mutation at position 3243, findings consistent with MELAS. Examination of her small intestine revealed a necrotic zone and numerous abnormal large mitochondria in the smooth muscle cells, vascular media and endothelium, and intestinal ganglion cells. The cerebral cortex showed multiple microcystic necrotic foci in cerebral cortex. Cactus-like pathology resembling the changes associated with Menkes' kinky hair disease and torpedoes were observed in the cerebellar purkinje cells. The intestinal dysmotility due to MELAS and cerebellar changes were presumed to be associated with a disturbance of copper metabolism.- - - - - - - - - - ranking = 0.51741361381619keywords = ganglion (Clic here for more details about this article) |
7/28. Dysembryoplastic neuroepithelial tumour and cerebellar atrophy: case report.Dysembryoplastic neuroepithelial tumours (DNET) are mainly benign cortical lesions. DNET in the caudate nucleus, thalamus, hypothalamus, pons and cerebellar hemispheres has also been reported. We describe a fronto-temporo-parietal DNET extending to the ipsilateral thalamus and internal capsule, associated with cerebellar lobe atrophy. Involvement of the internal capsule and complication of DNET with cerebellar atrophy have not been reported previously. We emphasise the importance of early diagnosis and treatment of this rare condition.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
8/28. A case of isolated nodulus infarction presenting as a vestibular neuritis.We reported a patient with cerebellar infarction who presented with purely isolated vertigo, ipsilesional spontaneous nystagmus, and contralesional axial lateropulsion without usual symptoms or signs of cerebellar dysfunction. An MRI of the brain showed a small left cerebellar infarct selectively involving the nodulus. A pure vestibular syndrome in our patient may be explained by ipsilateral involvement of nodulo-vestibular inhibitory projection to vestibular nucleus. Clinicians should be aware of the possibility of a nodulus infarction in patient with acute vestibular syndrome, even if the pattern of nystagmus and lateropulsion is typical of a vestibular neuritis.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
9/28. Optico-cochleo-dentate degeneration associated with severe peripheral neuropathy and caused by peroxisomal D-bifunctional protein deficiency.The clinical, neuroradiological, neuropathological and biochemical findings in a patient with optico-cochleo-dentate degeneration (OCDD; OMIM 258700) are presented in a severe case succumbing at the age of 4 years. The electron microscopic and biochemical data showed for the first time that OCDD may occur as the phenotypic expression of D-bifunctional protein deficiency, i.e., a peroxisomal disorder. The boy was born as the first child of healthy, consanguineous parents of Turkish origin. No other family members were affected. The main clinical symptoms consisted of muscle hypotonia ("floppy infant"), generalized epileptic fits, hypacusis, rotatory nystagmus, insufficient pupillary reactions, and mental retardation. Fibroblast cultures revealed D-bifunctional protein deficiency. Neuropathological examination displayed moderate frontoparietal and insular microgyria, and atrophy of the cerebellum. Loss of neurons was severe in the granular layer, the Purkinje cell band of the cerebellum, and rather complete in the dentate nucleus. A corresponding loss of myelinated fibers associated with characteristic periodic acid-Schiff-positive macrophages was most prominent in the white matter of the cerebellum. There was additional severe loss of myelinated fibers in the central portions of the optic nerve, reduction of the nerve fiber density in the cochlear nerve, and reduction of myelinated nerve fibers by about 80-90% in the sural nerve, which has not been studied in previous cases. At the electron microscopic level, characteristic inclusions mainly in perivascular macrophages and astrocytes were the most prominent finding. The inclusions usually showed a bilaminar structure, whereas trilaminar structures, typically seen in adrenoleukodystrophy, and multilaminar structures were less frequently seen.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
10/28. Positional nystagmus and vertigo due to a solitary brachium conjunctivum plaque.The authors describe two patients suffering from demyelinating central nervous system disease who developed intense vertigo and downbeat nystagmus upon tilting their heads relative to gravity. brain MRI revealed in both cases a single, small active lesion in the right brachium conjunctivum. The disruption of otolithic signals carried in brachium conjunctivum fibres connecting the fastigial nucleus with the vestibular nuclei is thought to be causatively involved, in agreement with a recently formulated model simulating central positional nystagmus. Insufficient otolithic information results in erroneous adjustment of the Listing's plane in off-vertical head positions, thus producing nystagmic eye movements.- - - - - - - - - - ranking = 0.25keywords = nucleus (Clic here for more details about this article) |
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