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KMID : 0352819940100010049
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Kosin Medical Journal
1994 Volume.10 No. 1 p.49 ~ p.58
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Three Cases of Dominantly inherited Olivopontocerebellar Atrophy
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Abstract
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Dominantly inherited olivopontocerebellar atrophy(OPCA) is a progressive autosomal dominant neurodegenerative disorder characterized clinically by ataxia and histologically by neuronal degeneration in the inferior olives, pons, and cerebellar
cortex.
Three patients in a family with hereditary OPCA are described. The mode of inheritance was autosomal dominant. The clinical maifestations were insidious progressive cerebellar ataxia, gait disturbance, and dysarthria. One patient showed slow eye
movement and pyramidal signs. The brain magnetic resonance imaging(MRI) showed significant atrophy of brainstem and cerebellum with dilatation of the 4th ventricle and prepontine cistern.The evoked potential studies revealed absent bilateral
tibial
and
median nerves somatosensory evoked potentials(SSEP) and prolonged absolute latency of III wave in the left ear brainstem auditory evoked potentials.
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KEYWORD
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