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.

Department

Neurology

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