Neuropathology of late cortical cerebellar atrophy in Japan: distribution of cerebellar change on an autopsy case and review of Japanese cases.

We report a Japanese autopsy case of late cortical cerebellar atrophy. The patient had showed clinically transient remission during thyrotropin-releasing hormone therapy. Our case suggests that thyrotropin-releasing hormone therapy is worth trying as a treatment of late cortical cerebellar atrophy. Neuropathological examination showed diffuse cerebellar cortical lesions and absence of neuronal loss in the dorsomedial part of the inferior olives. We studied qualitatively the detailed distribution of the cerebellar cortical lesions in 6 sections of the right cerebellum. The cerebellar lesions were more conspicuous in the most lateral hemisphere than in the vermis. We also reviewed 7 Japanese autopsy cases of late cortical cerebellar atrophy.