Abstract
ObjectiveTo describe the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children.MethodData from 11 pediatric patients with autoimmune GFAP astrocytopathy were retrospectively analyzed.ResultsAll of the patients showed encephalitis and meningoencephalitis or meningoencephalomyelitis with or without myelitis. 45.4% of the patients had fever, 27.3% headaches, 18.2% dizziness, 18.2% drowsiness, and 18.2% mental disorders. Cerebrospinal fluid (CSF) was detected in all patients. The white blood cell counts (WBC) (90.9%), lactic dehydrogenase levels (72.7%), protein level (36.4%), and adenosine deaminase activity (ADA) level (27.3%) were elevated, and the CSF glucose levels (72.7%) were slightly reduced. Nine patients (90%) were found to have brain abnormalities, of which five (50.0%) patients had abnormal symmetrical laminar patterns or line patterns hyperintensity lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the basal ganglia, hypothalamus, subcortical white matter and periventricular white matter. The linear radial enhancement pattern of the cerebral white matter was only seen in two patients, with the most common being abnormal enhancement of leptomeninges (50%). Five patients had longitudinally extensive spinal cord lesions.ConclusionThe findings of pediatric patients with autoimmune GFAP astrocytopathy are different from previous reports.
Highlights
In 2016, autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy was first reported as a kind of meningoencephalomyelitis associated with GFAP-IgG, a spectrum of autoimmune inflammatory central nervous system (CNS) disorders, and was subsequently confirmed internationally [1]
Patients We enrolled 11 pediatric patients who were admitted to our hospital (Department of Neurology) between January 2018 and April 2021 with encephalitis and meningoencephalitis or meningoencephalomyelitis who only responded to corticosteroid treatment
Among these 11 patients, two had other common antibodies; one patient was positive for myelin oligodendrocyte glycoprotein (MOG) antibodies, and the other was positive for N-methyl-D-aspartate receptor (NMDAR) antibodies
Summary
In 2016, autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy was first reported as a kind of meningoencephalomyelitis associated with GFAP-IgG, a spectrum of autoimmune inflammatory central nervous system (CNS) disorders, and was subsequently confirmed internationally [1]. This disorder involves meningeal, brain parenchymal, spinal cord, or optic nerve inflammation and injury, and is characterized by corticosteroid response. Patients and methodology This study protocol was approved by the medical ethics committee of the Hunan Children’s hospital of the University of South China This retrospective study was approved by the institutional review board.
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