Olivopontocerebellar atrophy. A review of 117 cases.

Fifty-four cases of familial OPCA (FOPCA) and sixty-three cases of sporadic OPCA (SOPCA) have been gathered from the literature. The data concerning age at onset, duration of the disease, frequency of symptoms and the various localizations of lesions have been evaluated. In comparison with SOPCA, the disease begins earlier in FOPCA and lasts longer (P less than 0.

[Thrombosis of intracraneal sinuses in homocystinuria (author's transl)].

The case of a child affected with piridoxinresistant homocystinuria who developed early on the disease an aseptic thrombosis of intracraneal sinuses, of chronic evolution is presented. Pathogenesis and therapy of the thrombosis are discussed emphasizing the value of measures directed to avoid the aggregation of platelets.