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Joseph disease

A multisystem degenerative disorder of the nervous system

Departments of Neurology (Dr. Sachdev) and Pathology (Dr. Forno), Palo Alto Veterans Administration Medical Center, Stanford University Medical Center, Palo Alto, CA, and Division of Neurology, The Permanente Medical Group. Kaiser Hospital (Dr. Kanei, Hay ward, CA.

We studied Joseph disease clinically and pathologically in two patients of Portuguese ancestry, but from different families. We found involvement of spinocerebellar tracts, Clarke's column, anterior horn cells, motor cranial nerve nuclei, and substantia nigra. One patient also had pallidosubthalamic and pontocerebellar degeneration with normal inferior olives. The second patient, a Joseph family member, had nerve cell loss in the subthalamic nucleus. The neostriatum appeared normal in both cases. The pigmented nuclei contained a few Lewy bodies. The almost identical pathology in two families support the hypothesis that Joseph disease is a genetic entity.

Address correspondence and reprint requests to Dr. Forno, Department of Pathology, Veterans Administration Medical Center. 3801 Miranda Avenue, Palo Alto, CA 94304.

This study was supported by the Veterans Administration Medical Research Program.

Accepted for publication August 4, 1981.

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