Clinical features of the first attack with leukodystrophy-like phenotype in children with myelin oligodendrocyte glycoprotein antibody-associated disorders

作者全名："Jiang, Yan; Tan, Chengbing; Li, Xiujuan; Jiang, Li; Hong, Siqi; Yuan, Ping; Zheng, Heling; Fan, Xiao; Han, Wei"

作者地址："[Jiang, Yan; Tan, Chengbing; Li, Xiujuan; Jiang, Li; Hong, Siqi; Yuan, Ping; Han, Wei] Chongqing Med Univ, Key Lab Child Dev & Disorders, Natl Clin Res Ctr Child Hlth & Disorders, Chongqing Key Lab Pediat,Dept Neurol,Childrens Hos, Chongqing, Peoples R China; [Zheng, Heling; Fan, Xiao] Chongqing Med Univ, Dept Radiol, Childrens Hosp, Chongqing, Peoples R China; [Han, Wei] Chongqing Med Univ, Dept Neurol, Childrens Hosp, 136 Zhongshan 2nd Rd, Chongqing 400014, Peoples R China"

通信作者："Han, W (通讯作者)，Chongqing Med Univ, Dept Neurol, Childrens Hosp, 136 Zhongshan 2nd Rd, Chongqing 400014, Peoples R China."

来源：INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

ESI学科分类：NEUROSCIENCE & BEHAVIOR

WOS号：WOS:000952119700001

JCR分区：Q3

影响因子：1.7

年份：2023

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期号：　

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结束页：　

文献类型：Article; Early Access

关键词：children; leukodystrophy-like phenotype; MOGAD

摘要："BackgroundMyelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) is identified autoimmune disorder with a predominance in paediatric patients, and the disease spectrum has expanded with clinical and radiological patterns. The aim of the study was to describe the clinical characteristics of the first attack with leukodystrophy-like phenotype with MOGAD in children. MethodsPatients hospitalized at the Children's Hospital of Chongqing Medical University from June 2017 to October 2021 with positive MOG antibodies and phenotype of leukodystrophy-like (symmetric white matter lesions) were retrospectively analyzed. Cell-based assays (CBAs) were used to test MOG antibodies. ResultsFour cases from 143 MOGAD patients were recruited, with two females and two males. The age of onset is all under 6 years old. At the last follow-up, four cases exhibited a monophasic course, including ADEM in three patients and encephalitis in one patient. The mean EDSS score at onset was 4.62 +/- 2.93, and the modified Rankin score (mRS) was 3.00 +/- 1.82. First-attack symptoms include fever, headache, vomiting, seizure, loss of consciousness, emotional and behavioural disorder, and ataxia. The brain MRI showed prominent extensive and essentially symmetric distribution lesions in the white matter. All patients showed clinical and partial radiological improvement after intravenous immunoglobulin and/or glucocorticoid treatment. ConclusionThe first attack with MOGAD onset of leukodystrophy-like phenotype was more frequently seen in younger children than other phenotype patients. The patients may show impressive neurologic disorders, but most patients who receive immunotherapy have a good prognosis."

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