Patterns of Cerebral Atrophy in Primary Progressive Aphasia

Abstract

Illustrates the spectrum of clinical and imaging patterns in primary progressive aphasia (PPA), a syndrome of slowly progressive speech and language impairment occurring with neurodegenerative disease. Although PPA presents with relatively isolated impairment in language, it is noted that many patients progress to global cognitive or behavioral dysfunction. This syndrome may be associated with frontotemporal dementia (FTD)- or Alzheimer disease (AD)-type changes. This study describes the clinical presentation in 3 patients (aged 52-85 yrs) with PPA and analyzes the pattern of cerebral atrophy in each case with voxel-based morphometry. Two Ss presented with nonfluent progressive aphasia. Subtle differences in the clinical features were suggestive of FTD in one case and AD in the other. Neuroimaging revealed a predominance of frontal atrophy in the first case and temporo-parietal atrophy in the second. The third case presented with the syndrome of semantic dementia and shows the typical behavioral problems associated with FTD and a pattern of left-greater-than-right temporal atrophy. It is concluded that different clinical syndromes in PPA are associated with different patterns of atrophy. (PsycINFO Database Record (c) 2016 APA, all rights reserved)