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NEUROLOGY 1984;34:1213
© 1984 American Academy of Neurology

Familial multisystem atrophy with possible thalamic dementia

David A. Katz, MD, Attia Naseem, MD, Dikran S. Horoupian, MD, A. David Rothner, MD and Peter Davies, PhD

From the Department of Pathology (Neuropathology) and Rose F. Kennedy Center for Research in Mental Retardation and Human Development (Drs. Katz, Horoupian, and Davies), Albert Einstein College of Medicine, and the Bronx Municipal Hospital Center, Bronx, NY; the Department of Pathology (Dr. Naseem), Mt. Vernon Hospital, Mt. Vernon, NY; and the Department of Neurology (Dr. Rothner), The Cleveland Clinic Foundation, Cleveland, OH.

We studied a family with progressive dementia, optic atrophy, and spastic paraparesis. Autopsy of one family member revealed multisystem atrophy with widespread degeneration of the thalamus and marked attenuation of hemispheric white matter to account for the-dementia. This family may suffer from a unique disorder, but the findings overlap with other reported cases of thalamic dementia or familial spastic paraparesis.

Address correspondence and reprint requests to Dr. Horoupian, Department of Pathology (Neuropathology), Rose F. Kennedy Center, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461.

Accepted for publication January 17, 1984.