A Clinical Study of 38 Cases of Olivopontocerebellar Atrophy

ABSTRACT

Olivopontocerebellar atrophy (OPCA) is a form of degenerative disease associated with neuronal degeneration in the cerebellar cortex, pons, and inferior olives. The authors have reviewed 38 cases, clinically diagnosed as OPCA, admitted in department of neurology, Seoul National University Hospital From January, 1985 to August, 1989. Seven cases of familial and 31 cases of sporadic forms have been gathered. Gait ataxia and dizziness are the most frequent initial manifestations and the cerebellar ataxia and pyramidal tract signs are frequent neurologic findings in both familial and sporadic forms. Age of onset is earlier in the familial form (mean 31.4y) than in the sporadic form (mean 47.5y). The duration of the disease is longer in the former(6.8y) than in the latter (2.9y). Abnormal ocular movement and nystagmus are more frequent in the familial form, while autonomic changes and parkinsonian features in the sporadic form.