Clinical Analysis And Literature Review Of 5 Cases Of Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy In Children

Objective: To summarize and analyze the clinical characteristics of autoimmune glial fibrillary acidic protein astrocytopathy and review the literature to provide ideas for the diagnosis and treatment of the disease.Methods: Retrospective collection of data on 5 confirmed cases of autoimmune astrocytic fibroblastosis astrocyte patients admitted by pediatrics in our hospital from March 2020 to December 2020,including clinical manifestations,imaging examination,laboratory examination,treatment and prognosis of children.Results: The age of 5 patients with autoimmune GFAP-A was 3-15 years old,and the ratio was 4:1.Two patients showed signs of pre-emplacement infection and four cases of elevated myoplasmic titer of pneumonia,2 of which were newly infected.Four of the patients were clinically present with meningococcal meningitis or meningococcal meningoencephalitis and one with epilepsy.In 5 cases,abnormality occurred in the MRI of the skull,of which 1 case was seen to be reinforced by radiation-like patterns around the ventricle.5 cases of serum and cerebrospinal fluid anti-GFAP antibodies were positive,the highest antibody titer cases at the same time the most severe clinical symptoms.1 case combined with other nervous system autoantibodies.No tumors were found in all pediatric tumor screenings.4 cases of immunotherapy,including high-dose hormone shock,intravenous cprotein application,of which 1 case of severe patients with plasma replacement treatment,all prognosis is good,follow-up did not recur.Conclusion: The etiology and pathogenesis of autoimmune GFAP-A are not clear.The combination of meningitis,encephalitis,spinal corditis or the above syndrome of acute or subacute disease is the main clinical manifestation of autoimmune GFAP-A.However,because its clinical performance and laboratory examination are non-specific,there is still no uniform standard of diagnosis and treatment,easy to misdiagnose and delay treatment.The central nervous system MRI can provide ideas for diagnosis and treatment,when the emergence of ventricle or peripheral peripheral radiopathy,serum and cerebrospinal fluid GFAP antibody examination should be perfected as soon as possible for further diagnosis,as soon as possible to be immunotherapy.