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Mixed tau and TDP-43 pathology in a patient with unclassifiable primary progressive aphasia.


ABSTRACT: Classifying primary progressive aphasia (PPA) into variants that may predict the underlying pathology is important. However, some PPA patients cannot be classified. A 78-year-old woman had unclassifiable PPA characterized by anomia, dysarthria, and apraxia of speech without agrammatism. Magnetic resonance imaging revealed left mesial temporal atrophy and 18-flourodeoxy-glucose positron emission tomography showed left anterior temporal and posterior frontal (premotor) hypometabolism. Autopsy revealed a mixed tauopathy (argyrophilic grain disease) and transactive response-DNA-binding-protein-43 proteinopathy. Dual pathologies may explain the difficulty classifying some PPA patients and recognizing this will be important as new imaging techniques (particularly tau-positron emission tomography) are introduced and patients begin enrollment in clinical trials targeting the underlying proteinopathy.

SUBMITTER: Flanagan EP 

PROVIDER: S-EPMC4628904 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature

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Mixed tau and TDP-43 pathology in a patient with unclassifiable primary progressive aphasia.

Flanagan Eoin P EP   Duffy Joseph R JR   Whitwell Jennifer L JL   Vemuri Prashanthi P   Dickson Dennis W DW   Josephs Keith A KA  

Neurocase 20150501 1


Classifying primary progressive aphasia (PPA) into variants that may predict the underlying pathology is important. However, some PPA patients cannot be classified. A 78-year-old woman had unclassifiable PPA characterized by anomia, dysarthria, and apraxia of speech without agrammatism. Magnetic resonance imaging revealed left mesial temporal atrophy and 18-flourodeoxy-glucose positron emission tomography showed left anterior temporal and posterior frontal (premotor) hypometabolism. Autopsy reve  ...[more]

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