Striatonigral Degeneration

IN 1961, Adams et al¹described a series of patients with clinical features of parkinsonism, but characterized pathologically by a predominance of neuronal degeneration in the striatum (especially the putamen) and substantia nigra. They termed this condition striatonigral degeneration, thus distinguishing it from the more frequently encountered cases of parkinsonism with lesions principally in the substantia nigra and/or globus pallidus.²,³Adams⁴has recently reviewed the present status of this disease based on a study of six known cases and has considered in detail its clinical features, neuropathology, and nosological relationship to other degenerative diseases of the basal ganglia. Five additional cases, probably representing this condition, have also appeared from other sources.⁵⁻⁸The purpose of this report is to describe four cases of this uncommon clinical-pathological entity and to document the response of three patients to stereotaxic thalamic lesions. Report of Cases CASE1.—This 54-year-old