Spinocerebellar degeneration with parkinsonian features: a clinical and pathological report

A 20-year-old patient had spinocerebellar degeneration and parkinsonian tremor that improved with L-dopa treatment. Postmortem examination revealed posterior column atrophy in the spinal cord and severe degeneration in the substantia nigra. Atrophic changes were noted in deep cerebellar nuclei and superior cerebellar peduncles. This study confirms the relationships between clinical parkinsonism, substantia nigra atrophy, and the therapeutic effect of L-dopa.