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Neurology 2002;59:1079-1082
© 2002 American Academy of Neurology
Brief Communications

Familial dementia with Lewy bodies

Clinicopathologic analysis of two kindreds

J.E. Galvin, MD MSc*;, S.L. Lee, MD PhD*;, A. Perry, MD, N. Havlioglu, MD, D.W. McKeel, Jr., MD and J.C. Morris, MD

*These authors contributed equally to this article.
From the Departments of Neurology (Drs. Galvin, Lee, and Morris) and Pathology and Immunology (Drs. Perry, Havlioglu, McKeel, and Morris), Washington University School of Medicine; and Department of Pathology (Dr. Havlioglu), St. Louis University, MO.

Address correspondence and reprint requests to Dr. James E. Galvin, Alzheimer Disease Research Center, Washington University School of Medicine, 4488 Forest Park, Suite 130, St. Louis, MO 63108; e-mail: galvinj{at}neuro.wustl.edu

Familial cases of dementia with Lewy bodies (DLB) are rare. The authors describe two small kindreds with familial DLB: one with pure DLB meeting consensus criteria for DLB and one with coexistent AD pathology that did not fulfill DLB criteria. The authors call attention to the diverse features of DLB and suggest that current clinical criteria may not detect all cases. Familial DLB is clinically heterogeneous and occurs with or without coexistent AD, suggesting the relevance of LB pathology for the developing dementia.




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