Familial dementia with Lewy bodies with an atypical clinical presentation

• Published in: Journal of geriatric psychiatry and neurology Vol. 16; no. 1; p. 59
• Main Authors: Bonner, Lauren T, Tsuang, Debby W, Cherrier, Monique M, Eugenio, Charisma J, Du Jennifer, Q, Steinbart, Ellen J, Limprasert, Pornprot, La Spada, Albert R, Seltzer, Benjamin, Bird, Thomas D, Leverenz, James B
• Format: Journal Article
• Language: English
• Published: United States 01.03.2003
• Subjects: Alzheimer Disease - genetics; Alzheimer Disease - pathology; Humans; Lewy Body Disease - genetics; Lewy Body Disease - pathology; Male; Middle Aged; Neuropsychological Tests
• ISSN: 0891-9887
• DOI: 10.1177/0891988702250585

The authors report a case of a 64-year-old male with Alzheimer's disease (AD) and dementia with Lewy bodies (DLB) pathology at autopsy who did not manifest the core symptoms of DLB until very late in his clinical course. His initial presentation of early executive and language dysfunction suggested a cortical dementia similar to frontotemporal lobar degeneration (FTLD). Core symptoms of DLB including dementia, hallucination, and parkinsonian symptoms were not apparent until late in the course of his illness. Autopsy revealed both brainstem and cortical Lewy bodies and AD pathology. Family history revealed 7 relatives with a history of dementia including 4 with possible or probable DLB. This case is unique because of the FTLD-like presentation, positive family history of dementia, and autopsy confirmation of DLB.