Cargando…
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...
Autores principales: | , , , |
---|---|
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 |
_version_ | 1784905695250874368 |
---|---|
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. |
format | Online Article Text |
id | pubmed-10008168 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | BMJ Publishing Group |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT goynechristophere radiographicevolutionofmyelitisinacaseofglialfibrillaryacidicproteingfapastrocytopathy AT piccionidavid radiographicevolutionofmyelitisinacaseofglialfibrillaryacidicproteingfapastrocytopathy AT handwerkerjason radiographicevolutionofmyelitisinacaseofglialfibrillaryacidicproteingfapastrocytopathy AT rajagopalamutha radiographicevolutionofmyelitisinacaseofglialfibrillaryacidicproteingfapastrocytopathy |