title: Familial dementia with Lewy bodies with an atypical clinical presentation.
abstract: 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.
authors.affiliation: Department of Veterans Affairs Northwest Network Mental Illness Research, Education, and Clinical Center, University of Washington, Seattle 98108, USA.
authors.forename: Lauren T
authors.initials: LT
authors.lastname: Bonner
authors.forename: Debby W
authors.initials: DW
authors.lastname: Tsuang
authors.forename: Monique M
authors.initials: MM
authors.lastname: Cherrier
authors.forename: Charisma J
authors.initials: CJ
authors.lastname: Eugenio
authors.forename: Q
authors.initials: Q
authors.lastname: Du Jennifer
authors.forename: Ellen J
authors.initials: EJ
authors.lastname: Steinbart
authors.forename: Pornprot
authors.initials: P
authors.lastname: Limprasert
authors.forename: Albert R
authors.initials: AR
authors.lastname: La Spada
authors.forename: Benjamin
authors.initials: B
authors.lastname: Seltzer
authors.forename: Thomas D
authors.initials: TD
authors.lastname: Bird
authors.forename: James B
authors.initials: JB
authors.lastname: Leverenz
mesh_terms: D000544:Alzheimer Disease / Q000235:genetics* / Q000473:pathology*; D006801:Humans; D020961:Lewy Body Disease / Q000235:genetics* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests