Familial dementia with lewy bodies: a clinical and neuropathological study of 2 families.

Background: Dementia with Lewy bodies (DLB) is characterized by early dementia and associated visual hallucinations, parkinsonism, and fluctuations in cognition. Few families with DLB have been described with detailed clinical, pathological, and genetic assessments.

Objective: To investigate the clinical, neuropathological, and genetic characteristics of families with 2 or more autopsy-proven cases of DLB.