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NEUROLOGY 1993;43:2031
© 1993 American Academy of Neurology

Fulminant multiple system atrophy in a young adult presenting as motor neuron disease

A.A.F. Sima, MD, PhD, M. Caplan, MD, C. J. D'Amato, BS, M. Pevzner, MD and J. W. Furlong, MD

From the Departments of Pathology (Drs. Sima and Caplan, and C.J. D'Amato) and Internal Medicine (Dr. Sima), University of Michigan, Ann Arbor, and the Departments of Internal Medicine (Dr. Pevzner) and Pathology (Dr. Furlong), St. Joseph Mercy Hospital. Pontiac, MI.

A 34-year-old man demonstrated rapidly progressive motor neuron disease and, late in his 9-month clinical course, exhibited ophthalmoplegia and dysautonomic symptoms. Neuropathology showed spinal and bulbar motor neuron disease with severe involvement of extraocular motor nuclei, degeneration of spinal sympathetic and bulbar parasympathetic nuclei, striatonigral degeneration, and early olivopontocerebellar atrophy. This case underscores the diversity of multiple system atrophy and demonstrates an unusually rapid course in a young patient.

Address correspondence and reprint requests to Dr. Anders A.F. Sima, University of Michigan, 1331 E. Ann Street, Box 0580, Ann Arbor, MI 48109.

Received December 8,1992. Accepted for publication in final form February 24, 1993.




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