Mixed tau and TDP-43 pathology in a patient with unclassifiable primary progressive aphasia. Issue 1 (2nd January 2016)

• Record Type: Journal Article
• Authors: Flanagan, Eoin P.; Duffy, Joseph R.; Whitwell, Jennifer L.; Vemuri, Prashanthi; Dickson, Dennis W.; Josephs, Keith A.
• Is Part Of: Neurocase. Volume 22:Issue 1(2016)
• Page Start: 55
• Page End: 59
• Publication Date: 2016-01-02
• Subjects: frontotemporal lobar degeneration -- primary progressive aphasia -- argyrophilic grain disease -- tau -- TDP-43; Neurology -- Periodicals; Neuropsychology -- Case Report; Mental Disorders -- Case Report; Nervous System Diseases -- Case Report; 616.8
• DOI: 10.1080/13554794.2015.1041534
• Languages: English
• ISSNs: 1355-4794

Abstract

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.