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以惊厥发作为核心症状的中枢神经系统神经元表面抗体综合征10例患儿临床特征分析

Clinical features analysis in 10 children with seizures as core symptoms of neuronal surface antibody syndromes
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摘要 目的探讨儿童以惊厥发作为核心症状的中枢神经系统神经元表面抗体综合征的临床特征方法收集2015年12月-2016年12月在华中科技大学同济医学院附属武汉儿童医院神经内科收治10例中枢神经系统神经元表面抗体综合征的患儿,所有患儿均以惊厥发作为核心症状人院、结果男1例,女9例,就诊年龄3~13岁,病程3~14d。10例患儿就诊时主要症状均为惊厥发作,2例强直发作,1例强直阵挛发作,7例部分性发作;其中6例患儿出现惊厥持续状态,丛集性发作,后续在病程5~30d时出现精神行为异常。7例患儿有手不自主动作,1例出现肌张力不全,2例出现多种锥体外系症状。1O例患儿行脑脊液自身免疫性抗体检测,9例抗N-甲基-D-天冬氨酸受体(N-methy-D-aspartate recptor,NMDAR)抗体阳性,1例抗LGI1抗体阳性。7例脑电图(EEG)表现为慢波、尖慢波,2例棘波,1例慢波。1例患儿头颅核磁共振(MRI)异常。10例患儿急性期行丙种球蛋白联合激素冲击治疗,8例临床症状作免疫治疗后7~21d好转,随访3~6个月,未再发作。2例抗NMDAR抗体相关性癫痫患儿急性期抗癫痫药物(AEDs)单药治疗效果差,联合一线免疫疗法,发作控制可,但后期复发,一线治疗失败,予二线免疫疗法环磷酰胺定期冲击治疗有效。结论惊厥发作可为儿童中枢神经系统神经元表面抗体综合征首次发病形式,对于临床上起病急骤的惊厥发作,合并有惊厥持续状态,应注意早期筛查惊厥发作的免疫因素。常见的神经元表面抗体以NMDAR抗体为主,惊厥发作时EEG表现以慢波、尖慢波为主,头颅MRI多无特异性。AEDs联合免疫治疗有效,在发病初期及时给予免疫治疗,对癲痫发作控制效果较好。一线治疗失败,可行二线免疫疗法环磷酰胺定期冲击治疗。 Objective To study the clinical features of children with seizures as core symptoms of neuronal surface antibody syndromes. Methods The clinical data of neuronal surface antibody syndromes between December 2015 and December 2016 were obtained and analyzed. All children presented to hospital with seizures as core symptoms. Results There were 1 male and 9 females in this study. The ages ranged from 3 years to 13 years. The disease course was between 3 and 14 days. All children presented to hospital with seizures as core symptoms. Two children had tonic seizures.one had tonic-clonic seizure. Seven had partial seizures. Among them, six children had status epilepticus and cluster attack. The other symptoms in the course of the disease were psychiatric symptoms and extrapyramidal symptoms. The anti-NMDAR antibody were found in 9 patients' CSF and blood. The LGI1 antibody was found in one patients' CSF and blood. The EEG test of 7 patients showed slow wave and sharp slow wave. Two showed spike wave. One showed slow wave. The MRI test of one patient showed abnormal. Ten cases were treated with IVIG and methylprednisolone during acute stage. The patients had been followed up for 3 to 6 months. Eight of them recovered completely. Two cases had seizures. Two cases diagnosed with anti-NMDAR related epilepsy received sound effects after treated with cyclophosphamide. Conclusions Convulsion may be the first common symptom of neuronal surface antibody syndromes in children. Immune factors should be screened when children with acute seizures and status epilepticus. Accompanying psychiatric symptoms, autoimmune epilepsy should be considered. The most common neuronal surface antibody in children with neuronal surface antibody syndromes is NMDAR antibody. EEG usually shows slow wave and sharp slow wave during seizures. Brain MRI is usually normal. Immunotherapy is effective in the majority of patients as the first line treatment. When the first-line treatment failed, second-line immunotherapy such as cyclophosphamide shock therapy
作者 胡春辉 孙丹 胡家胜 刘智胜 HU Chun-hui;SUN Dan;HU Jia-sheng;LIU Zhi-sheng;Department of Neurology,Wuhan Children’s Hospital,Tongji Medical College,Huazhong University of Science & Technology
出处 《癫痫杂志》 2017年第6期479-483,共5页 Journal of Epilepsy
基金 武汉市科技创新平台-儿童神经疾病临床医学研究中心资助项目(2014-160)
关键词 惊厥发作 神经元表面抗体综合征 儿童 Convulsion Neuronal surface antibody syndrome Children
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