Abstract：【Objective】 To investigate the clinical characteristics and short-term prognosis of pediatric patients with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A).【Methods】 A retrospective analysis was conducted on the clinical data of 15 pediatric patients with cerebrospinal fluid positivity for glial fibrillary acidic protein (GFAP).【Results】 The median age of onset was 8 years. The main clinical symptoms included fever, limb weakness, headache, and loss of appetite. The predominant clinical phenotypes were encephalitis, meningitis, and myelitis. Magnetic resonance imaging (MRI) revealed lesions involving brain parenchyma, meninges, optic nerves, and spinal cord, presenting as patchy, linear, punctate, and strip-like T2 hyperintensities. Among 15 patients,13 cases had elevated cerebrospinal fluid white blood cells, mainly lymphocytes, 8 with elevated protein, and 4 with decreased sugar. Fourteen patients responded well to first-line immunotherapy, including corticosteroids and immunoglobulins, while one patient with overlapping aquaporin(AQP-4) antibody experienced relapse and had a poor response to first-line therapy.【Conclusion】Pediatric GFAP-A patients have diverse clinical symptoms and MRI findings, lacking distinct specificity. First-line immunotherapy is generally effective in treatment, although patients with overlapping AQP-4 antibody tend to have a poor prognosis.
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2025, Vol. 42 
