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Behavioral features in semantic dementia vs other forms of progressive aphasias

From the University of California at San Francisco Department of Neurology (H.J.R., S.C.A., S.A., K.R., B.L.M., M.L.G.-T.); USCF Memory and Aging Center (H.J.R., S.C.A., S.A., K.R., B.L.M., M.L.G.-T.); VA Northern California Health Care System (J.M.O., N.D.); University of California, Davis (J.M.O., N.D.); and University of California, San Diego (J.M.O., N.D.).

Address correspondence and reprint requests to Dr. Howard J. Rosen, UCSF Department of Neurology, Memory and Aging Center, 350 Parnassus Ave., Box 1207, Suite 706, San Francisco, CA 94143-1207; e-mail: howie{at}itsa.ucsf.edu

Objective: To compare the behavioral profiles in different variants of primary progressive aphasia (PPA).

Methods: We classified 67 patients with PPA into three clinical variants: semantic dementia (SEMD), progressive nonfluent aphasia (PNFA), and logopenic progressive aphasia (LPA), and we compared the severity of behavioral dysfunction, as measured by the Neuropsychiatric Inventory, in these groups and patients with frontotemporal dementia (FTD) and Alzheimer disease (AD).

Results: SEMD was associated with significantly more socioemotional behavioral dysfunction than the other two variants of PPA and than AD, specifically more disinhibition, aberrant motor behavior, and eating disorders—behaviors that are typical of FTD. In contrast, PNFA and LPA did not differ from each other or from AD in the type or severity of behavioral dysfunction. Behavioral abnormalities increased in severity with disease duration in SEMD, but this association was not detected in PNFA or LPA.

Conclusions: Semantic dementia is associated with significantly more behavioral dysfunction than other variants of primary progressive aphasia, specifically behavioral features typical of frontotemporal dementia.

Editorial, see page 1738

See also page 1849

Supported by NIA grants 1K08AG020760-01, AG10129, P50-AG05142, and AG16570, National Institute of Neurologic Disorders and Stroke grant NS050915, the State of California Department of Health Services (DHS) AD Research Center of California (ARCC) grant 01-154-20, State of California DHS grant 04-35516, NIH grant number M01 RR00079 (UCSF General Clinical Research Center), and the Hillblom Network.

Disclosure: The authors report no conflict of interest.

Received February 7, 2006. Accepted in final form September 12, 2006.

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