关键词: 小脑共济失调； 免疫性； 复发性； 儿童

Abstract: Objective To analyze the clinical features, treatment and prognosis of recurrent immune cerebellar ataxia in children. Methods Clinical data of relapsed immune cerebellar ataxia in 11 children diagnosed from January 2016 to June 2020 were collected and analyzed. Results The median onset age of 11 children ( 4 boys and 7 girls) was 2 . 2 years ( 0 . 8 - 10 . 8 years). Seven children had prodromal infection or vaccination during the first course of the disease. All the 11 children had abnormal gait or unsteadiness, 8 had intentional tremor, 5 had dyslexic articulation, 5 had body wobble and 5 had nystagmus. Among the 11 children, 5 children were positive for systemic autoantibodies, 2 had elevated CSF leukocytes, and 2 were positive for paraneoplastic syndrome antibodies. Two children had slow background activity of electroencephalogram. During the course of recurrence, cerebellar atrophy was showed in 3 children by cranial magnetic resonance (MRI) and cerebellar hypometabolism was showed in 2 children by PET-CT. The median follow-up time was 27 months ( 10 - 48 months) in the 11 children who had 21 relapses in total and the median recurrence interval was 4 months ( 3 - 24 months). The causes of recurrence were infection ( 10 times), hormone reduction ( 7 times), Mycophenolate Mofetil reduction ( 1 time) and no obvious inducement ( 3 times). Intravenous infusion of human immunoglobulin and methylprednisolone were the preferred treatment for all the children at the first onset, and their symptoms disappeared or were relieved. After rituximab immunosuppressive therapy, recurrence was reduced in 6 children who had repeated recurrence. Mediastinal ganglioneuroblastoma was found in 2 children with recurrence and surgical resection was performed, and then the symptoms disappeared or alleviated. The median follow-up time after the last attack in the 11 children was 13 months ( 1 - 35 months). Four cases had complete remission, 4 cases had broad basal gait, 3 cases had slight wobble of trunk and limbs, and 2 cases had unclear articulation. The annual recurrence rate after immunosuppressive therapy decreased from 1 . 6 to 0 . 19 . Conclusions The most common predisposing factor of recurrent immune cerebellar ataxia in children is infection. It happens mostly in female. Human immunoglobulin and hormone shock therapy were effective at the first onset, and immunosuppressive therapy could improve the condition and reduce the recurrence in patients with multiple relapses. Some patients with tumors should be treated etiologically.

Key words: cerebellar ataxia; immunity; recurrent; child