Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions

We report a 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year. On neurological examination, he displayed spastic paraplegia and autonomic dysfunctions including dysuria and...

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Autores principales: Kudo, Takuya, Kimura, Akio, Higashida, Kazuhiro, Yamada, Megumi, Hayashi, Yuichi, Shimohata, Takayoshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7691024/
https://www.ncbi.nlm.nih.gov/pubmed/32669494
http://dx.doi.org/10.2169/internalmedicine.5074-20
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author Kudo, Takuya
Kimura, Akio
Higashida, Kazuhiro
Yamada, Megumi
Hayashi, Yuichi
Shimohata, Takayoshi
author_facet Kudo, Takuya
Kimura, Akio
Higashida, Kazuhiro
Yamada, Megumi
Hayashi, Yuichi
Shimohata, Takayoshi
author_sort Kudo, Takuya
collection PubMed
description We report a 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year. On neurological examination, he displayed spastic paraplegia and autonomic dysfunctions including dysuria and constipation. Spinal cord magnetic resonance imaging showed longitudinally extensive spinal cord lesions (LESCLs) extending from the cervical to the thoracic cords. The patient was negative for anti-myelin oligodendrocyte glycoprotein and anti-aquaporin 4 antibodies. Treatment with corticosteroids and intravenous immunoglobulin resulted in a clinical improvement. It is important to distinguish GFAP-A from slowly progressive myelitis with LESCLs.
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spelling pubmed-76910242020-12-03 Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions Kudo, Takuya Kimura, Akio Higashida, Kazuhiro Yamada, Megumi Hayashi, Yuichi Shimohata, Takayoshi Intern Med Case Report We report a 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year. On neurological examination, he displayed spastic paraplegia and autonomic dysfunctions including dysuria and constipation. Spinal cord magnetic resonance imaging showed longitudinally extensive spinal cord lesions (LESCLs) extending from the cervical to the thoracic cords. The patient was negative for anti-myelin oligodendrocyte glycoprotein and anti-aquaporin 4 antibodies. Treatment with corticosteroids and intravenous immunoglobulin resulted in a clinical improvement. It is important to distinguish GFAP-A from slowly progressive myelitis with LESCLs. The Japanese Society of Internal Medicine 2020-07-14 2020-11-01 /pmc/articles/PMC7691024/ /pubmed/32669494 http://dx.doi.org/10.2169/internalmedicine.5074-20 Text en Copyright © 2020 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Kudo, Takuya
Kimura, Akio
Higashida, Kazuhiro
Yamada, Megumi
Hayashi, Yuichi
Shimohata, Takayoshi
Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions
title Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions
title_full Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions
title_fullStr Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions
title_full_unstemmed Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions
title_short Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions
title_sort autoimmune glial fibrillary acidic protein astrocytopathy presenting with slowly progressive myelitis and longitudinally extensive spinal cord lesions
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7691024/
https://www.ncbi.nlm.nih.gov/pubmed/32669494
http://dx.doi.org/10.2169/internalmedicine.5074-20
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