This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Bonner, L. T. Articles by Leverenz, J. B. Search for Related Content PubMed PubMed Citation Articles by Bonner, L. T. Articles by Leverenz, J. B. Social Bookmarking                         What's this?

Familial Dementia with Lewy Bodies with an Atypical Clinical Presentation

Department of Veterans Affairs Northwest Network Mental Illness Research, Education, and Clinical Center (MIRECC), Department of Psychiatry and Behavioral Sciences, University of Washington, Seattle

Debby W. Tsuang, MD, MSc

Department of Veterans Affairs Northwest Network Mental Illness Research, Education, and Clinical Center (MIRECC), Department of Psychiatry and Behavioral Sciences, University of Washington, Seattle

Monique M. Cherrier, PhD

Department of Psychiatry and Behavioral Sciences, University of Washington, Seattle

Charisma J. Eugenio, BS

Department of Veterans Affairs Northwest Network Mental Illness Research, Education, and Clinical Center (MIRECC), University of Washington, Seattle

Jennifer Q. Du, RN

Department of Veterans Affairs Northwest Network Mental Illness Research, Education, and Clinical Center (MIRECC), University of Washington, Seattle

Ellen J. Steinbart, RN

Department of Geriatric Research, Education and Clinical Centers, University of Washington, Seattle

Pornprot Limprasert, MD, PhD

Department of Laboratory Medicine, University of Washington, Seattle

Albert R. La Spada, MD, PhD

Department of Laboratory Medicine, Department of Neurology, Department of Medicine (Medical Genetics), University of Washington, Seattle

Benjamin Seltzer, MD

Tulane University School of Medicine, Department of Psychiatry and Neurology and the Department of Veterans Affairs South Central MIRECC, New Orleans, Louisiana

Thomas D. Bird, MD

Department of Geriatric Research, Education and Clinical Centers, Department of Neurology, Department of Medicine (Medical Genetics), University of Washington, Seattle

James B. Leverenz, MD

Department of Veterans Affairs Northwest Network Mental Illness Research, Education, and Clinical Center (MIRECC), Department of Parkinson’s Disease Research, Education, and Clinical Center, Department of Neurology, Department of Psychiatry and Behavioral Sciences, University of Washington, Seattle

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. (J Geriatr Psychiatry Neurol 2003; 16:59-64)

Key Words: Lewy bodies • familial dementia • Alzheimer’s disease

Journal of Geriatric Psychiatry and Neurology, Vol. 16, No. 1, 59-64 (2003)
DOI: 10.1177/0891988702250585


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				V. Bogaerts, S. Engelborghs, S. Kumar-Singh, D. Goossens, B. Pickut, J. van der Zee, K. Sleegers, K. Peeters, J.-J. Martin, J. Del-Favero, et al. A novel locus for dementia with Lewy bodies: a clinically and genetically heterogeneous disorder Brain, September 1, 2007; 130(9): 2277 - 2291. [Abstract] [Full Text] [PDF]

				H. Ohtake, P. Limprasert, Y. Fan, O. Onodera, A. Kakita, H. Takahashi, L. T. Bonner, D. W. Tsuang, I. V.J. Murray, V. M.-Y. Lee, et al. {beta}-Synuclein gene alterations in dementia with Lewy bodies Neurology, September 14, 2004; 63(5): 805 - 811. [Abstract] [Full Text] [PDF]