Cargando…

Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a...

Descripción completa

Detalles Bibliográficos
Autores principales: Li, Xue-Lin, Han, Jinming, Zhao, Hao-Tian, Long, You-Ming, Zhang, Bing-Wei, Wang, Hai-Yang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7270931/
https://www.ncbi.nlm.nih.gov/pubmed/32547640
http://dx.doi.org/10.1177/1756286420909973
_version_ 1783541990513704960
author Li, Xue-Lin
Han, Jinming
Zhao, Hao-Tian
Long, You-Ming
Zhang, Bing-Wei
Wang, Hai-Yang
author_facet Li, Xue-Lin
Han, Jinming
Zhao, Hao-Tian
Long, You-Ming
Zhang, Bing-Wei
Wang, Hai-Yang
author_sort Li, Xue-Lin
collection PubMed
description Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a 21-year-old girl presenting with dysuria and weariness, who subsequently developed blurry vision, slight dysphagia, slurred speech, and sensory abnormality. GFAP-IgG was detected in her cerebrospinal fluid. Magnetic resonance imaging using both T2-weighted and contrast-enhanced T1-weighted images revealed a rare finding of lesions distributed mainly in the entire spinal cord rather than typical brain lesions. After treating with corticosteroids, her clinical symptoms were alleviated, and the spinal cord lesion enhancement was reduced. Our observations extend the clinical spectrum of autoimmune GFAP astrocytopathy. We suggest that rare distributed lesions in the entire spinal cord in patients with autoimmune GFAP astrocytopathy cannot be ignored by neurologists. The identification of potential atypical lesions broadens the understanding of autoimmune GFAP astrocytopathy.
format Online
Article
Text
id pubmed-7270931
institution National Center for Biotechnology Information
language English
publishDate 2020
publisher SAGE Publications
record_format MEDLINE/PubMed
spelling pubmed-72709312020-06-15 Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord Li, Xue-Lin Han, Jinming Zhao, Hao-Tian Long, You-Ming Zhang, Bing-Wei Wang, Hai-Yang Ther Adv Neurol Disord Case Report Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a 21-year-old girl presenting with dysuria and weariness, who subsequently developed blurry vision, slight dysphagia, slurred speech, and sensory abnormality. GFAP-IgG was detected in her cerebrospinal fluid. Magnetic resonance imaging using both T2-weighted and contrast-enhanced T1-weighted images revealed a rare finding of lesions distributed mainly in the entire spinal cord rather than typical brain lesions. After treating with corticosteroids, her clinical symptoms were alleviated, and the spinal cord lesion enhancement was reduced. Our observations extend the clinical spectrum of autoimmune GFAP astrocytopathy. We suggest that rare distributed lesions in the entire spinal cord in patients with autoimmune GFAP astrocytopathy cannot be ignored by neurologists. The identification of potential atypical lesions broadens the understanding of autoimmune GFAP astrocytopathy. SAGE Publications 2020-06-03 /pmc/articles/PMC7270931/ /pubmed/32547640 http://dx.doi.org/10.1177/1756286420909973 Text en © The Author(s), 2020 https://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Li, Xue-Lin
Han, Jinming
Zhao, Hao-Tian
Long, You-Ming
Zhang, Bing-Wei
Wang, Hai-Yang
Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord
title Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord
title_full Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord
title_fullStr Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord
title_full_unstemmed Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord
title_short Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord
title_sort autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7270931/
https://www.ncbi.nlm.nih.gov/pubmed/32547640
http://dx.doi.org/10.1177/1756286420909973
work_keys_str_mv AT lixuelin autoimmuneglialfibrillaryacidicproteinastrocytopathywithlesionsdistributedpredominantlyintheentirespinalcord
AT hanjinming autoimmuneglialfibrillaryacidicproteinastrocytopathywithlesionsdistributedpredominantlyintheentirespinalcord
AT zhaohaotian autoimmuneglialfibrillaryacidicproteinastrocytopathywithlesionsdistributedpredominantlyintheentirespinalcord
AT longyouming autoimmuneglialfibrillaryacidicproteinastrocytopathywithlesionsdistributedpredominantlyintheentirespinalcord
AT zhangbingwei autoimmuneglialfibrillaryacidicproteinastrocytopathywithlesionsdistributedpredominantlyintheentirespinalcord
AT wanghaiyang autoimmuneglialfibrillaryacidicproteinastrocytopathywithlesionsdistributedpredominantlyintheentirespinalcord