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At least 991 records · Page 55Linked to original sources
PubMed1997

[A case of severe involvement of the motor neuron system accompanied with cerebellar ataxia].

Here we report a sporadic case of severe involvement of the motor neuron system accompanied with cerebellar ataxia. A 55-year-old Japanese woman was admitted to our hospital because of unstable gait and clumsiness of hands. Since she had prominent ataxia, she was initially diagnosed as late onset cortical cerebellar atrophy (LCCA). However, mild muscular weakness and atrophy were pointed out. Weakness in extremities progressed slowly and she became unable to walk in two years. On the second admission, in addition to cerebellar ataxia, she had moderate to severe muscular weakness and atrophy with fasciculation in extremities. Although she had no sensory impairment, micturitional disturbance nor orthostatic hypotension, she had impaired skin sweating response. MRI imaging revealed moderate cerebellar and brain stem atrophy. Neurophysiological examination revealed upper and lower motor neuron damage. Beta-D-N acetylglucosaminidase activity was normal and SCA1, DRPLA and Machado-Joseph disease were excluded by DNA studies. Combination of motor neuron disease and cerebellar degeneration has been very rare. Only two cases similar to our case were reported before. Our patient had anti GM1-ganglioside antibody in her serum, suggesting that motor neuron disease and cerebellar degeneration may occur with the same pathophysiological mechanism.

Cerebellar Ataxia↗
PubMed1982

Brain pyruvate carboxylase and the pathophysiology of biotin-dependent diseases.

Given the cerebellar symptomatology of biotin-dependent diseases and other lactic acidoses, we hypothesized that cerebellar pyruvate carboxylase activity might be differentially low or especially sensitive to cofactor deprivation. Accordingly, pyruvate carboxylase activity was measured in selected areas of normal and biotin-deficient rat brain. Control cerebellar hemisphere and vermis specific activities were identical, and slightly higher than cerebral and brainstem activities. In biotin-deficient rats, hepatic pyruvate carboxylase activity was 3% of control, whereas pyruvate carboxylase activities of all brain sections were 53 to 71% of control. Brain histology was normal. Cerebellar pyruvate carboxylase activity is therefore not distinctly low or labile and is in fact preferentially maintained despite severe cofactor deprivation.

Animals↗
PubMed1984

Rebound nystagmus in olivocerebellar atrophy: a clinicopathological correlation.

Rebound nystagmus was recorded in a patient with cerebellar dysfunction. Neuropathological examination showed degeneration of the inferior olivary nuclei and the cerebellar cortex. The basis pontis and the brainstem tegmentum were spared. Rebound nystagmus is attributed to a smooth eye movement bias that is generated within intact vestibuloocular pathways to oppose gaze-evoked centripetal drift of the eyes. The source of the rebound bias was not visual, because rebound nystagmus normally occurs in darkness, smooth pursuit was paretic, and the olivocerebellar visual relay pathway was degenerated. An extraretinal, efference copy signal of the eye position error must detect the ocular drift and generate the rebound bias. The neuropathological findings indicate that rebound nystagmus can be a sign of involvement confined to the olivocerebellar circuit and that nonvisual signals of eye position are monitored independently of this cerebellar system.

Aged↗
PubMed1964

CEREBELLAR ATAXIA IN HAMSTERS INOCULATED WITH RAT VIRUS.

Chronic ataxia associated with hypoplasia of the cerebellum was induced by intracerebral inoculation of newborn hamsters with various strains of rat virus. The condition usually became recognizable within 3 weeks of inoculation and remained static as the animals matured. Preliminary studies suggest that hypoplasia is induced by the rat virus acting on the outer germinal layer of the cerebellum at a critical time in its ontogenic development.

Animals↗