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Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies?
Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP‐A) is a steroid‐responsive meningoencephalomyelitis, sometimes presenting with atypical clinical signs such as movement disorders or psychiatric and autonomic features. Beyond clinical presentation and imaging, diagnosis relies on detec...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Blackwell Publishing Ltd
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8935272/ https://www.ncbi.nlm.nih.gov/pubmed/35130372 http://dx.doi.org/10.1002/acn3.51513 |
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author | Friedrich, Maximilian Hartig, Johannes Prüss, Harald Ip, Chi Wang Volkmann, Jens |
author_facet | Friedrich, Maximilian Hartig, Johannes Prüss, Harald Ip, Chi Wang Volkmann, Jens |
author_sort | Friedrich, Maximilian |
collection | PubMed |
description | Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP‐A) is a steroid‐responsive meningoencephalomyelitis, sometimes presenting with atypical clinical signs such as movement disorders or psychiatric and autonomic features. Beyond clinical presentation and imaging, diagnosis relies on detection of GFAP‐antibodies (AB) in CSF. Using quantitative behavioral, serologic, and immunohistochemical analyses, we characterize two patients longitudinally over 18–24 months who presented with rapidly progressive neurocognitive deterioration in the context of GFAP‐AB in CSF and unremarkable cranial MRI studies. Intensified immunotherapy was associated with clinical stabilization. The value of GFAP‐AB screening in selected cases of rapidly progressive dementias is discussed. |
format | Online Article Text |
id | pubmed-8935272 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Blackwell Publishing Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-89352722022-03-24 Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies? Friedrich, Maximilian Hartig, Johannes Prüss, Harald Ip, Chi Wang Volkmann, Jens Ann Clin Transl Neurol Case Study Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP‐A) is a steroid‐responsive meningoencephalomyelitis, sometimes presenting with atypical clinical signs such as movement disorders or psychiatric and autonomic features. Beyond clinical presentation and imaging, diagnosis relies on detection of GFAP‐antibodies (AB) in CSF. Using quantitative behavioral, serologic, and immunohistochemical analyses, we characterize two patients longitudinally over 18–24 months who presented with rapidly progressive neurocognitive deterioration in the context of GFAP‐AB in CSF and unremarkable cranial MRI studies. Intensified immunotherapy was associated with clinical stabilization. The value of GFAP‐AB screening in selected cases of rapidly progressive dementias is discussed. Blackwell Publishing Ltd 2022-02-07 /pmc/articles/PMC8935272/ /pubmed/35130372 http://dx.doi.org/10.1002/acn3.51513 Text en © 2022 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made. |
spellingShingle | Case Study Friedrich, Maximilian Hartig, Johannes Prüss, Harald Ip, Chi Wang Volkmann, Jens Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies? |
title | Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies? |
title_full | Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies? |
title_fullStr | Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies? |
title_full_unstemmed | Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies? |
title_short | Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies? |
title_sort | rapidly progressive dementia: extending the spectrum of gfap‐astrocytopathies? |
topic | Case Study |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8935272/ https://www.ncbi.nlm.nih.gov/pubmed/35130372 http://dx.doi.org/10.1002/acn3.51513 |
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