CT FEATURES OF OLIVOPONTOCEREBELLAR ATROPHY IN CHILDREN

Between 1990 and 1992. 14 children were seen in whom a clinical diagno sis of olivopontocerebellar atrophy (OPCA) had been made. The majority of patients presented with cerebellar ataxia and hypotonia. Five chil dren had a family history of a similar illness in first-degree relativ es. All cases had undergone clinical and neurologic examinations, rout ine laboratory tests and cranial CT. CT features were graded to quanti tate the degree of atrophy in each cerebellar hemisphere, vermis and b rain stem. All patients had varying degrees of atrophic changes of cer ebellum, brain stem and cerebrum. These CT features appear to be disti nctive enough to enable the diagnosis of OPCA to be made.