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NEUROLOGY 1991;41:62
© 1991 American Academy of Neurology

Early-onset Alzheimer's disease in 2 large Belgian families

J. J. Martin, MD, J. Gheuens, MD, M. Bruyland, MD, P. Cras, MD, A. Vandenberghe, PhD, C. L. Masters, MD, K. Beyreuther, PhD, R. Dom, MD, C. Ceuterick, PhD, U. Lübke, H. Van Heuverswijn, PhD, G. De Winter and C. Van Broeckhoven, PhD

Department of Neurology (Drs. Martin, Gheuens, Cras, Ceuterick, and U. Lübke), University Hospital and Laboratory of Neuropathology, Born-Bunge Foundation and Universitaire Instelling Antwerpen, Wilrijk, Belgium; the Department of Neurology (Dr. Bruyland), City Hospital of Ronse, Ronse, Belgium; the Department of Biochemistry (Dr. Vandenberghe, G. De Winter, and Dr. Van Broeckhoven), Universitaire Instelling Antwerpen, Wilrijk, Belgium; the Department of Pathology (Dr. Masters), University of Melbourne. Australia; the Center for Molecular Biology (Dr. Beyreuther). University of Heidelberg, Germany; the Department of Neurology (Dr. Dom). Laboratory of Neuropathology, University Hospital Gasthuisherg, Leuven, Belgium; and Innogenetics (Dr. Van Heuverswijn and G. De Winter). Ghent. Belgium.

Familial Alzheimer's disease (FAD) is a dominantly inherited condition that may present with an early onset, and myoclonus occurs frequently in the course of the disease. We report clinical and neuropathologic data on 2 large Belgian families with FAD in which we obtained 17 autopsies of the CNS. In family A, each of 11 autopsies had the typical neuropathologic features of Alzheimer's disease (AD), and there were a few cerebellar plaques in the molecular layer. In family B, in addition to the typical characteristics of AD in 6 autopsies, there were numerous amyloid plaques in the cortical cerebellar layers. In both families, we immunostained the amyloid deposits for the A4 protein, and they were negative for prion-associated protein immunoreactivity.

Address correspondence and reprint requests to Dr. J.J. Martin, Gebouw T. Lokaal 5-18, Universitaire Instelling Antwerpen, Universiteitsplein 1, B-2610, Wilrijk, Antwerpen. Belgium.

Supported by the Belgian Fonds voor Geneeskundig Wetenschappelijk Onderzoek (grant no. 3.0019.86).

Received September 20, 1989. Accepted for publication in final form June 1.1990.




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