Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy
Status PubMed-not-MEDLINE Jazyk angličtina Země Švýcarsko Médium electronic-ecollection
Typ dokumentu časopisecké články
PubMed
31920619
PubMed Central
PMC6918864
DOI
10.3389/fnagi.2019.00336
Knihovny.cz E-zdroje
- Klíčová slova
- TDP-43 proteinopathy, dementia, globular glial inclusions, primary progressive aphasia, tauopathy,
- Publikační typ
- časopisecké články MeSH
Globular glial tauopathies (GGTs) have heterogeneous presentations with little available information regarding typical clinical manifestations. We report on a case of atypical primary progressive aphasia (PPA) due to comorbid GGT and limbic transactive response DNA binding protein of 43 kDa (TDP-43) proteinopathy. The initial clinical phenotype was compatible with the nonfluent-agrammatical variant of PPA and early hippocampal amnesia. Progressively, parkinsonism and supranuclear oculomotor impairment occurred, and finally, late mutism with frontal-type dementia, impaired comprehension, and behavioral manifestations developed. The neuropathology was characteristic of GGT type I with vascular changes and comorbid limbic-predominant age-related TDP-43 encephalopathy (LATE). Our findings expand the clinical spectrum of GGTs to include a complex progressive aphasia syndrome. The extraordinary feature, in this case, was the combination of two progressive aphasia subtypes, that is, the early nonfluent-agrammatical variant and the late semantic variant. Our findings also expand the spectrum of neuropathological comorbidities in GGT.
Department of Communication Disorders Comenius University Bratislava Slovakia
Department of Nursing 2nd Faculty of Medicine Charles University Prague Czechia
Department of Radiology Na Homolce Hospital Prague Czechia
Laboratory Medicine Program and Krembil Brain Institute University Health Network Toronto ON Canada
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