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Primary progressive aphasia 

Primary progressive aphasia
Chapter:
Primary progressive aphasia
Author(s):

Jonathan D. Rohrer

and Jason D. Warren

DOI:
10.1093/med/9780199655946.003.0034
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date: 30 May 2020

This chapter overviews clinical and neuroimaging features and pathological and genetic associations of the primary progressive aphasias. Three major forms of progressive aphasia are recognized, with characteristic clinical syndromes, regional brain atrophy profiles and pathological substrates of abnormal protein deposition. Semantic dementia is characterized by loss of vocabulary with supervening more generalized breakdown of nonverbal semantic memory, generally associated with selective asymmetric (predominantly left-sided) anterior temporal lobe atrophy and TDP-43 pathology. Progressive nonfluent aphasia is characterized by disintegration of speech output, most often associated with predominantly left-sided peri-Sylvian atrophy and with tau or TDP-43 pathology. Logopenic aphasia is characterized by word-finding pauses with reduced phonological memory, most often associated with predominantly left-sided temporo-parietal atrophy and underlying Alzheimer’s pathology. Progressive nonfluent aphasia may be associated with progranulin or C9ORF72 mutations; other genetic associations are uncommon. Despite recent progress, the underlying brain mechanisms, classification and diagnosis of these diseases present unresolved problems.

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