Format

Send to

Choose Destination
Neurocase. 2016;22(1):55-9. doi: 10.1080/13554794.2015.1041534. Epub 2015 May 1.

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

Author information

1
a Department of Neurology, Divisions of Behavioral Neurology , Mayo Clinic , Rochester , MN , USA.
2
b Department of Neurology, Divisions of Speech Pathology , Mayo Clinic , Rochester , MN , USA.
3
c Radiology Research , Mayo Clinic , Rochester , MN , USA.
4
d Department of Laboratory Medicine and Pathology , Mayo Clinic , Jacksonville , FL , USA.

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.

KEYWORDS:

TDP-43; argyrophilic grain disease; frontotemporal lobar degeneration; primary progressive aphasia; tau

PMID:
25929342
PMCID:
PMC4628904
DOI:
10.1080/13554794.2015.1041534
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for PubMed Central
Loading ...
Support Center