February 23, 2016: Primary progressive aphasia
February 23, 2016
Primary Progressive Aphasia
Primary progressive aphasia is a frontotemporal lobar degeneration with three clinical subtypes. Semantic variant PPA is characterized by a fluent but anomic aphasia and single-word comprehension impairment.1 Nonfluent variant PPA features halting or agrammatic speech, with impaired comprehension of complex sentences.2 The logopenic subtype presents with impairment of repetition, phonologic paraphasias and word retrieval difficulty but without agrammatism. Pathophysiologically, two findings predominate: tau-positive or ubiquitin/TDP43-positive pathology, especially in the semantic and agrammatic variants. The logopenic subtype has been correlated with Alzheimer's type pathology, however, all three report cases with these pathologic findings.2
- Matthews BR. Memory dysfunction. Continuum 2015; 21: 613-626.
- Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011; 76: 1006-1014.
Submitted by Ilena George, MD, Department of Neurology, Yale School of Medicine, New Haven.
Disclosures: Dr. George is a member of the Resident and Fellow Section of Neurology.