Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Friedrich, Maximilian, Hartig, Johannes, Prüss, Harald, Ip, Chi Wang, Volkmann, Jens
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Blackwell Publishing Ltd 2022
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
_version_ 1784672012727222272
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
work_keys_str_mv AT friedrichmaximilian rapidlyprogressivedementiaextendingthespectrumofgfapastrocytopathies
AT hartigjohannes rapidlyprogressivedementiaextendingthespectrumofgfapastrocytopathies
AT prussharald rapidlyprogressivedementiaextendingthespectrumofgfapastrocytopathies
AT ipchiwang rapidlyprogressivedementiaextendingthespectrumofgfapastrocytopathies
AT volkmannjens rapidlyprogressivedementiaextendingthespectrumofgfapastrocytopathies