Cognition and anatomy in three variants of primary progressive aphasia

Annals of Neurology - Tập 55 Số 3 - Trang 335-346 - 2004
Maria Luisa Gorno‐Tempini1, Nina F. Dronkers2,3, Katherine P. Rankin1, Jennifer Ogar2, La Phengrasamy1, Howard J. Rosen1, Julene K. Johnson1, Michael W. Weiner1,4, Bruce L. Miller1
1Department of Neurology, University of California San Francisco, San Francisco
2Center for Aphasia and Related Disorders, VA Northern California Health Care System, Martinez
3Department of Neurology and Linguistics, University of California Davis, Davis
4Magnetic Resonance Unit, VA San Francisco, San Francisco, CA

Tóm tắt

AbstractWe performed a comprehensive cognitive, neuroimaging, and genetic study of 31 patients with primary progressive aphasia (PPA), a decline in language functions that remains isolated for at least 2 years. Detailed speech and language evaluation was used to identify three different clinical variants: nonfluent progressive aphasia (NFPA; n = 11), semantic dementia (SD; n = 10), and a third variant termed logopenic progressive aphasia (LPA; n = 10). Voxel‐based morphometry (VBM) on MRIs showed that, when all 31 PPA patients were analyzed together, the left perisylvian region and the anterior temporal lobes were atrophied. However, when each clinical variant was considered separately, distinctive patterns emerged: (1) NFPA, characterized by apraxia of speech and deficits in processing complex syntax, was associated with left inferior frontal and insular atrophy; (2) SD, characterized by fluent speech and semantic memory deficits, was associated with anterior temporal damage; and (3) LPA, characterized by slow speech and impaired syntactic comprehension and naming, showed atrophy in the left posterior temporal cortex and inferior parietal lobule. Apolipoprotein E ε4 haplotype frequency was 20% in NFPA, 0% in SD, and 67% in LPA. Cognitive, genetic, and anatomical features indicate that different PPA clinical variants may correspond to different underlying pathological processes.

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Annals of Neurology

Tập 55 Số 3

335-346

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