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Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy

Autoimmune glial fibrillar acidic protein (GFAP) astrocytopathy is a rare autoimmune neuroinflammatory disorder that affects the central nervous system. We present a case of GFAP astrocytopathy in a middle-aged male who presented with constitutional symptoms, encephalopathy and lower extremity weakn...

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Autores principales: Goyne, Christopher E, Piccioni, David, Handwerker, Jason, Rajagopal, Amutha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10008168/
https://www.ncbi.nlm.nih.gov/pubmed/36898710
http://dx.doi.org/10.1136/bcr-2022-248921
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author Goyne, Christopher E
Piccioni, David
Handwerker, Jason
Rajagopal, Amutha
author_facet Goyne, Christopher E
Piccioni, David
Handwerker, Jason
Rajagopal, Amutha
author_sort Goyne, Christopher E
collection PubMed
description Autoimmune glial fibrillar acidic protein (GFAP) astrocytopathy is a rare autoimmune neuroinflammatory disorder that affects the central nervous system. We present a case of GFAP astrocytopathy in a middle-aged male who presented with constitutional symptoms, encephalopathy and lower extremity weakness and numbness. Initially MRI of the spine was normal, but he subsequently developed longitudinally extensive myelitis and meningoencephalitis. Workup for infectious aetiologies was negative and the patient’s clinical course worsened despite broad antimicrobial coverage. Ultimately, he was found to have anti-GFAP antibodies in his cerebral spinal fluid consistent with GFAP astrocytopathy. He was treated with steroids and plasmapheresis with clinical and radiographic improvement. This case demonstrates the temporal evolution of myelitis on MRI in a case of steroid-refractory GFAP astrocytopathy.
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spelling pubmed-100081682023-03-13 Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy Goyne, Christopher E Piccioni, David Handwerker, Jason Rajagopal, Amutha BMJ Case Rep Case Reports: Rare disease Autoimmune glial fibrillar acidic protein (GFAP) astrocytopathy is a rare autoimmune neuroinflammatory disorder that affects the central nervous system. We present a case of GFAP astrocytopathy in a middle-aged male who presented with constitutional symptoms, encephalopathy and lower extremity weakness and numbness. Initially MRI of the spine was normal, but he subsequently developed longitudinally extensive myelitis and meningoencephalitis. Workup for infectious aetiologies was negative and the patient’s clinical course worsened despite broad antimicrobial coverage. Ultimately, he was found to have anti-GFAP antibodies in his cerebral spinal fluid consistent with GFAP astrocytopathy. He was treated with steroids and plasmapheresis with clinical and radiographic improvement. This case demonstrates the temporal evolution of myelitis on MRI in a case of steroid-refractory GFAP astrocytopathy. BMJ Publishing Group 2023-03-10 /pmc/articles/PMC10008168/ /pubmed/36898710 http://dx.doi.org/10.1136/bcr-2022-248921 Text en © BMJ Publishing Group Limited 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) .
spellingShingle Case Reports: Rare disease
Goyne, Christopher E
Piccioni, David
Handwerker, Jason
Rajagopal, Amutha
Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy
title Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy
title_full Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy
title_fullStr Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy
title_full_unstemmed Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy
title_short Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy
title_sort radiographic evolution of myelitis in a case of glial fibrillary acidic protein (gfap) astrocytopathy
topic Case Reports: Rare disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10008168/
https://www.ncbi.nlm.nih.gov/pubmed/36898710
http://dx.doi.org/10.1136/bcr-2022-248921
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