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Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy

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)...

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Autores principales: Rusina, Robert, Csefalvay, Zsolt, Kovacs, Gabor G., Keller, Jiri, Javurkova, Alena, Matej, Radoslav
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6918864/
https://www.ncbi.nlm.nih.gov/pubmed/31920619
http://dx.doi.org/10.3389/fnagi.2019.00336
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author Rusina, Robert
Csefalvay, Zsolt
Kovacs, Gabor G.
Keller, Jiri
Javurkova, Alena
Matej, Radoslav
author_facet Rusina, Robert
Csefalvay, Zsolt
Kovacs, Gabor G.
Keller, Jiri
Javurkova, Alena
Matej, Radoslav
author_sort Rusina, Robert
collection PubMed
description 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.
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spelling pubmed-69188642020-01-09 Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy Rusina, Robert Csefalvay, Zsolt Kovacs, Gabor G. Keller, Jiri Javurkova, Alena Matej, Radoslav Front Aging Neurosci Neuroscience 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. Frontiers Media S.A. 2019-12-11 /pmc/articles/PMC6918864/ /pubmed/31920619 http://dx.doi.org/10.3389/fnagi.2019.00336 Text en Copyright © 2019 Rusina, Csefalvay, Kovacs, Keller, Javurkova and Matej. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Rusina, Robert
Csefalvay, Zsolt
Kovacs, Gabor G.
Keller, Jiri
Javurkova, Alena
Matej, Radoslav
Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy
title Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy
title_full Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy
title_fullStr Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy
title_full_unstemmed Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy
title_short Globular Glial Tauopathy Type I Presenting as Atypical Progressive Aphasia, With Comorbid Limbic-Predominant Age-Related TDP-43 Encephalopathy
title_sort globular glial tauopathy type i presenting as atypical progressive aphasia, with comorbid limbic-predominant age-related tdp-43 encephalopathy
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6918864/
https://www.ncbi.nlm.nih.gov/pubmed/31920619
http://dx.doi.org/10.3389/fnagi.2019.00336
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