Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions.
astrocytopathy
autoantibody
glial fibrillary acidic protein (GFAP)
longitudinally extensive spinal cord lesion (LESCL)
myelitis
neuromyelitis optica spectrum disorders (NMOSDs)
Journal
Internal medicine (Tokyo, Japan)
ISSN: 1349-7235
Titre abrégé: Intern Med
Pays: Japan
ID NLM: 9204241
Informations de publication
Date de publication:
01 Nov 2020
01 Nov 2020
Historique:
pubmed:
17
7
2020
medline:
23
3
2021
entrez:
17
7
2020
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 32669494
doi: 10.2169/internalmedicine.5074-20
pmc: PMC7691024
doi:
Substances chimiques
Autoantibodies
0
Glial Fibrillary Acidic Protein
0
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
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