Abstract
Autoimmune glial fibrillary acidic protein astrocytopathy is a novel form of autoimmune meningoencephalitis related to GFAP autoantibodies. This condition is still being characterized, and few pediatric patients have been identified. Here, we report three patients presenting with fever, nausea, and headache, following progressive disturbance of consciousness, limb weakness, dyspnea, or urine retention. MRI analysis revealed that T2-hyperintense lesions, or enhancement of the meninges and spinal cord. CSF and serum analyses revealed they were positive for GFAP antibody, confirming GFAP astrocytopathy diagnosis. Treating the patients with IVIG, with or without intravenous steroids, gradually improved their clinical symptoms. Our findings indicate that GFAP astrocytopathy should be considered in children who are clinically diagnosed with meningoencephalitis, whether or not myelitis is present, and if the MRI reveals enhancement of meninges or spinal cord, T2-hyperintense lesions, or a pattern of linear perivascular gadolinium enhancement. Suspected cases should be tested for GFAP antibody as soon as possible because these patients may benefit from immunotherapy.
Highlights
Glial fibrillary acidic protein (GFAP) astrocytopathy is a recently identified type of autoimmune meningoencephalitis related to GFAP autoantibodies
Serum and cerebrospinal fluid (CSF) were examined for GFAP IgG using a cell-based assay that relies on GFAP Autoantibodies In Pediatric Patients
The patients were initially diagnosed with infectious meningoencephalitis, with or without myelitis, and treated with antibiotics and antivirals, but their conditions did not improve
Summary
Glial fibrillary acidic protein (GFAP) astrocytopathy is a recently identified type of autoimmune meningoencephalitis related to GFAP autoantibodies. Its clinical presentations include encephalopathy, headache, myelopathic symptoms, abnormal vision/optic neuritis, postural tremor, and cerebellar ataxia. GFAP astrocytopathy has almost exclusively been described in adults [1,2,3,4], with very few reported pediatric cases. Its clinical features in children remain to be defined. We present three pediatric cases of GFAP astrocytopathy and provide a literature review regarding this novel disorder
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