recommended using high-dose corticosteroid therapy to retreat patients with recurrence, although the dose of corticosteroid should then be slowly reduced after at least 3 months of therapy. Results == A total of 35 children, including 23 males and 12 females with a mean age of 6.3 0.6 years, manifested clinical symptoms of fever (62.9%), headache (42.9%), convulsions (42.9%), abnormal mental behavior (51.4%), disorders of consciousness (54.3%), visual disturbance (22.9%), ataxia (11.4%), paralysis (40%), and autonomic dysfunction (25.7%). One child exhibited only the clinical symptom of peripheral facial nerve palsy. Eleven out of 35 children were also positive for other antibodies. In addition to the common overlapping autoimmune syndromes, one case of autoimmune GFAP-A also manifested as Bickerstaffs brainstem encephalitis. Linear periventricular enhancement upon MRI was significantly less frequent in children (8.5%) than in adults. In pediatric patients, MRI contrast enhancement was principally seen in the meninges and brain lobes. Although repeated relapse (17.1%) and sequelae symptoms (20%) occurred in some cases, most children showed a favorable prognosis. Spearmans rank correlation showed that the antibody titer was not significantly associated with the severity of the initial disease conditions. == Conclusions == The disease diagnosis in children seropositive for GFAP antibodies only should receive a comprehensive diagnosis based on their clinical symptoms, imaging, electroencephalographic characteristics, and treatment responses. Some patients with relapses should receive repeated gamma globulin and corticosteroid therapy or the addition of immunosuppressants to their therapeutic regimen, and slow-dose tapering of corticosteroids and extended treatment are recommended for patients with overlapping autoimmune syndromes. Keywords:autoimmune glial fibrillary acidic protein astrocytopathy, GFAP-IgG antibodies, Il16 overlapping autoimmune syndromes, children, GFAP-A == 1 Introduction == Glial fibrillary acidic protein (GFAP) is an intermediate filament protein in astrocytes, with a size between that of microfilaments and microtubules. It is a biomarker for astrocytes, and participates in a variety of biological functions in astrocytes. Through a BM212 cell-based assay (CBA) and/or a tissue-based assay (TBA), specific immunoglobulin (Ig)G autoantibodies BM212 that selectively target GFAP in astrocytes have been detected in the serum and the cerebrospinal fluid of patients with autoimmune GFAP astrocytopathy (GFAP-A) (1). Autoimmune GFAP-A BM212 is an immune (IgG)-mediated inflammation within the central nervous system; its clinical characteristics include signs and symptoms involving the meninges, brain, and spinal cord, including abnormal vision. Although autoimmune GFAP-A has been reported more frequently in adults (especially those over 40 years old) than in children, there are still no standard diagnostic criteria available for this syndrome. Other coexisting neuronal antibodies can also be identified in patients, making disease diagnosis challenging. The specificity of serum GFAP antibodies is still uncertain and requires additional data for further evaluation. A previous study showed the presence of serum GFAP antibodies in patients with Alzheimers disease and cancer using ELISA (2). However, it is difficult to determine BM212 the significance of positive GFAP antibodies in serum alone in an autoimmune GFAP-A diagnosis. In the present study, we reported the clinical manifestations, characteristics of imaging and electroencephalography (EEG), treatment, and prognostic characteristics of 35 children with autoimmune GFAP-A and positive GFAP antibodies in serum and/or cerebrospinal fluid (CSF). This will provide a reference basis for determining the significance of GFAP antibodies in serum and CSF, and thus allow the formulation of standard diagnostic criteria for autoimmune GFAP-A. == 2 Materials and Methods == == 2.1 Patient Information and Research Methods == == 2.1.1 Patient Information == Children hospitalized in the Department of Neurology at Hunan Childrens Hospital (Hunan Province, China) from January 2015 to June 2021 owing to autoimmune diseases of the central nervous system were subjected to CBA to identify 40 children positive for GFAP-IgG antibodies in serum and/or cerebrospinal fluid, followed by collection of their clinical data. Five children only seropositive for GFAP-IgG antibodies were excluded, and the remaining 35 children were diagnosed with autoimmune GFAP-A. The extended stability status scale.