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NEUROLOGY 1991;41:820
© 1991 American Academy of Neurology

New pathologic observations in juvenile onset parkinsonism with dystonia

W.R.G. Gibb, MD, H. Narabayashi, MD, M. Yokochi, MD, R. Iizuka, MD and A. J. Lees, MD

National Hospital for Nervous Diseases (Drs. Gibb and Lees), Queen Square, London, UK; and Department of Neurology (Drs. Narabayashi, Yokochi, and Iizuka), Juntendo University School of Medicine, Tokyo, Japan.

A patient with hereditary juvenile onset parkinsonism with dystonia died at age 39. There were Lewy bodies and regionally selective neuronal damage in the substantia nigra pars compacta. These changes closely resemble those seen in Parkinson's disease, and emphasize the selective vulnerability of the ventral tier of the pars compacta in these degenerations.

Address correspondence and reprint requests to Dr. W.R.G. Gibb, University Department of Neurology, Institute of Psychiatry, De Crespigny Park, Denmark ?ill, London SE5 8AF, UK.

Presented in part at the 41st annual meeting of the American Academy of Neurology, Chicago, IL, April 1989.

Received January 18, 1990. Accepted for publication in final form November 9, 1990.




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