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October 1970

Striatonigral DegenerationClinical-Pathological Correlations and Response to Stereotaxic Surgery

Author Affiliations

Los Angeles; Bronx, NY
From the departments of neurology and pathology (neuropathology), UCLA Center for the Health Sciences, Los Angeles (Dr. Andrews), and the Department of Pathology, Albert Einstein College of Medicine, Bronx, NY (Drs. Terry and Spataro).

Arch Neurol. 1970;23(4):319-329. doi:10.1001/archneur.1970.00480280033004

IN 1961, Adams et al1 described 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.2,3 Adams4 has 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.5-8 The 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  CASE 1.—This 54-year-old

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