Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: An Emerging Cause of Meningoencephalomyelitis in Children and Adolescents
Document Type
Journal Article
Publication Date
12-1-2023
Journal
Journal of child neurology
Volume
38
Issue
13-14
DOI
10.1177/08830738231214301
Keywords
GFAP; astrocytopathy; glial fibrillary acidic protein; linear radial enhancement; meningoencephalomyelitis
Abstract
We describe 5 children with GFAP astrocytopathy with the goal of further characterizing this rare form of meningoencephalomyelitis. Retrospective chart review of patients diagnosed with GFAP astrocytopathy between 2019 and 2021. Patients were 8-17 years old, and all were male. Fever, headache, and vomiting were common presenting symptoms, and weakness, tremor, and ataxia were common initial examination findings. Initial magnetic resonance imaging (MRI) showed spinal cord abnormalities in 2 patients and leptomeningeal enhancement in 1. Most patients had cerebral spinal fluid pleocytosis, and all screened negative for malignancy. Three patients progressed to coma, and all were treated with immunosuppressant therapy. By discharge, all patients had improved over their clinical nadir, although none had returned to baseline. GFAP astrocytopathy is a recently recognized cause of meningoencephalomyelitis in children. Here, we expand our understanding of this entity with the goal of aiding those treating children with GFAP astrocytopathy.
APA Citation
Uppal, Prayerna; Smith, Jacklyn; Castillo-Pinto, Carlos; Wells, Elizabeth M.; Jayakar, Anuj; and Harrar, Dana, "Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: An Emerging Cause of Meningoencephalomyelitis in Children and Adolescents" (2023). GW Authored Works. Paper 4044.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/4044
Department
Neurology