Clinical Features And Follow-up Study Of Neuromyelitis Optica Spectrum Disorders In Children

Objective:To analyze the clinical features and prognosis of children with neuromyelitis optica spectrum disorders(NMOSD),and to improve the understanding of the disease.Methods:Retrospectively analyzed The clinical manifestations,auxiliary examination results and follow-up data of 8 children with NMO and NMOSD admitted to the Pediatrics Department of Wuhan Tongji Hospital from March 2012 to October 2018.Describe the disease status and prognosis using EDSS scores and ARR.The relationship between serum complement and severity of acute phase of the disease was analyzed using SPSS statistical software.Results:Among the 8 children,1 was a boy and 7 were girls.The age of onset ranged from 4 years and 11 months to 10 years and 10 months.There are 3 cases of infection history before illness.2 cases had a single-phase course during the observation period,and 6 cases had recurrence.The first symptoms of all cases were optic neuritis,4 cases with blindness or near blindness in one or both eyes,6 cases with encephalopathy,and no cases with myelitis.There were 2 cases of serum aquaporin 4(AQP4)antibody positive,1 case of cerebrospinal fluid serum aquaporin 4 antibody positive,and 2 cases of serum myelin oligodendrocyte glycoprotein(MOG)antibody positive.One of these cases was positive for both serum AQP4 and MOG antibodies.No patients were found to have other autoimmune diseases.There were 7 patients with abnormal MRI,including 7 cases with abnormal MRI in the head,7 cases with frontal and parietal involvement,and 4 cases with unilateral or bilateral semi-oval center involvement.Two patients had similar changes to acute disseminated encephalomyelitis.Only one patient had abnormal MRI in the spinal cord,which was manifested by cervical spinal cord involvement.Six patients had immunological function test data at the beginning of the disease.Among them,4 patients had lower serum C4 levels,and there was a negative correlation between serum complement C4 level and acute EDSS score,but there was no statistical significance.7 patients were treated with methylprednisolone and intravenous immunoglobulin(IVIG)in the acute phase,of which 5 patients had improved symptoms and 2 patients had no remission;4 patients in the maintenance phase were treated with immunosuppressive agents,three of whom had different degrees of improvement and did not relapse.The other patient received only IVIG shock therapy,who relapsed after 4 months and still only received IVIG shock therapy and was lost after discharge.Seven patients were followed up for a median follow-up of 2 years(7 months to 6 years and 6 months).As of the end of the observation period,the extended disability status scale(EDSS)score was 0 to 3 points,with a median of 2 points.The total recurrence rate was 75% in 8 patients.The average annual recurrence rate(ARR)is 0~3.4 times,with a median of 0.65 times per year.Conclusions:Children’s NMOSD is mainly characterized by optic neuritis and brain lesions,and half of them are blind or near blind;anti-AQP4 and MOG antibodies are important for the diagnosis,and AQP4 and MOG antibody double positive children are found for the first time;these cases are given in the acute phase.In the acute phase,methylprednisolone combined with IVIG shock therapy is effective in these cases,but the recurrence rate is also high.The use of immunosuppressive maintenance therapy can help control the disease and reduce recurrence.