Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: An Emerging Cause of Meningoencephalomyelitis in Children and Adolescents.
Prayerna UppalJacklyn SmithCarlos Castillo-PintoElizabeth M WellsAnuj JayakarDana B HarrarPublished in: Journal of child neurology (2023)
Introduction: We describe 5 children with GFAP astrocytopathy with the goal of further characterizing this rare form of meningoencephalomyelitis. Methods: Retrospective chart review of patients diagnosed with GFAP astrocytopathy between 2019 and 2021. Results: 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. Discussion: 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.
Keyphrases
- end stage renal disease
- magnetic resonance imaging
- ejection fraction
- spinal cord
- newly diagnosed
- chronic kidney disease
- prognostic factors
- stem cells
- young adults
- peritoneal dialysis
- magnetic resonance
- small cell lung cancer
- computed tomography
- cross sectional
- neuropathic pain
- mesenchymal stem cells
- contrast enhanced
- brain injury
- cell therapy
- diffusion weighted imaging