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NEUROLOGY 1989;39:910
© 1989 American Academy of Neurology

Familial dementia with PrP-positive amyloid plaques

A variant of Gerstmann-Sträussler syndrome

D. Nochlin, MD, S. M. Sumi, MD, T. D. Bird, MD, A. D. Snow, PhD, C. M. Leventhal, MD, K. Beyreuther, PhD and C.L. Masters, MD

Department of Pathology, University of Washington School of Medicine, and VA Medical Center (Dr. Bird), Seattle, WA (Dr. Snow)
Department of Neuropathology, University of Washington School of Medicine, and VA Medical Center (Dr. Bird), Seattle, WA (Drs. Nochlin and Sumi)
Department of Medicine Neurology (Drs. Sumi and Bird), University of Washington School of Medicine, and VA Medical Center (Dr. Bird), Seattle, WA
National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD (Dr. Leventhal)
Center of Molecular Biology, University of Heidelberg, Heidelberg, Federal Republic of Germany (Dr. Beyreuther)
Department of Pathology, University of Western Australia. Perth, Australia. (Dr. Masters)

We present a 22-year follow-up of a large and unusual kindred previously reported as familial Alzheimer's disease (FAD). However, detailed clinical and neuropathologic evaluation of family members and brain autopsy on another affected individual now make the diagnosis of FAD unlikely. Our patient, as well as members of this family, had numerous amyloid plaques and rare neurofibrillary tangles. These plaques were quite atypical for Alzheimer's disease (AD); many were quite large (up to 500 µm in diameter) and contained several amyloid cores, some with neuritic components. The plaques were present throughout the cerebral cortex and striatum, but not in the cerebellum. By electron microscopy, they had radiating star-shaped amyloid cores containing 8-to 10-nm fibrils, and a few dystrophic neurites. They were strongly immunoreactive with antiserum to prion protein but did not react with the antiserum to the amyloid A4 protein of AD. Although the cerebellum was uninvolved, this family appears to represent another clinical and neuropathologic variant of Gerstmann-Sträussler syndrome.

Address correspondence and reprint requests to Dr. Nochlin. Department of Pathology, Neuropathology Laboratory, RJ-05, University of Washington School of Medicine, Seattle, WA 98195.

C.L.M. and K.B. are supported in part by grants from the National Health and Medical Research Council (Australia), The Alzheimer's Disease and Related Disorders Association, the Deutsche Forschungsgemeinschaft, and the Fonds der chemischen Industrie. Supported by grant # AG05136 to the Alzheimer's Disease Research Center-University of Washington, American Health Assistance Foundation, and Veterans Administration Medical Research Funds.

Presented in part at the fortieth annual meeting of the American Academy of Neurology. Cincinnati, OH, April 1988.

Received November 22,1988. Accepted for publication in final form January 27,1989.




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