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.

• Authors: Eoin P Flanagan, Joseph R Duffy, Jennifer L Whitwell, Prashanthi Vemuri, Dennis W Dickson, Keith A Josephs
• Journal: Neurocase (Neurocase) Vol. 22 Issue 1 Pg. 55-9 ( 2016) ISSN: 1465-3656 [Electronic] England
• PMID: 25929342 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)

Chemical References

• DNA-Binding Proteins
• TARDBP protein, human
• tau Proteins

Topics

• Aged
• Aphasia, Primary Progressive (metabolism, pathology)
• Atrophy (metabolism, pathology)
• DNA-Binding Proteins (metabolism)
• Female
• Frontotemporal Lobar Degeneration (metabolism, pathology)
• Humans
• Magnetic Resonance Imaging
• TDP-43 Proteinopathies (metabolism, pathology)
• Tauopathies (metabolism, pathology)
• tau Proteins (metabolism)