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NEUROLOGY 1994;44:1015
© 1994 American Academy of Neurology

Progressive supranuclear palsy

Neuropathologic and clinical heterogeneity

Maria Gearing, PhD, David A. Olson, MD, Ray L. Watts, MD and Suzanne S. Mirra, MD

Veterans Affairs Medical Center and the Department of Pathology and Laboratory Medicine (Drs. Gearing and Mirra) and the Department of Neurology (Drs. Olson and Watts), Emory University School of Medicine, Atlanta, GA.

To investigate potential heterogeneity in progressive supranuclear palsy (PSP), we examined 13 patients with neuropathologically diagnosed PSP. The clinical diagnosis of PSP was made in eight of these individuals, whereas probable AD was the primary diagnosis in the remaining five. In addition to PSP neuropathology, seven of the 13 patients (54%) showed concomitant pathologic changes of Alzheimer's disease (AD), Parkinson's disease (PD), or both disorders. These observations indicate that AD and PD changes coexist with PSP neuropathology in a substantive proportion of patients and provide further evidence of clinical and neuropathologic heterogeneity in neurodegenerative disorders. Moreover, our results suggest that PSP may be underdiagnosed and deserves more prominence in the differential diagnosis of dementing illness.

Address correspondence and reprint requests to Dr. Suzanne S. Mirra, VA Medical Center (1131, 1670 Clairmont Road, Decatur, GA 30033.

Supported by the Veterans Affairs Merit Award and NIH grants AG06790 and AG10130.

Received August 2, 1993. Accepted for publication in final form November 22, 1993.




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