视神经脊髓炎的临床特点及预后分析

Clinical Features and Prognosis Analysis of Neuromyelitis Optica in Children

目的探讨儿童视神经脊髓炎(NMO)的临床特点及预后,以提高对此病的认识。方法研究对象为1997年4月-2010年5月在本院住院确诊的7例NMO患儿,对其临床表现、实验室及影像学检查、治疗及预后进行回顾性分析。结果 7例NMO患儿均为首次确诊病例。男2例,女5例,男女比例为1.03.5。起病年龄1岁5个月～15岁,中位年龄10岁。病前有感染史5例。首发症状为视神经炎3例,脊髓炎4例。视神经炎与脊髓炎间隔时间1 d～7个月,中位时间8 d。单侧视神经炎2例,双侧视神经炎5例。脊髓横贯性损害5例,不完全横贯性损害2例。视觉诱发电位(VEP)检查异常7例。脊髓MRI检查示胸段病灶7例,同时累及下颈段、腰骶段各1例;病灶长度4～11个脊髓节段,中位值7个脊髓节段。头颅MRI检查异常1例。自身抗体检查异常1例。脑脊液检查细胞数增高3例,均以淋巴细胞为主,病原学检查(-)。7例患儿急性期均静脉滴注甲泼尼龙冲击后改为口服泼尼松治疗,3例患儿同时静脉滴注丙种球蛋白。除1例患儿放弃治疗外,余6例病情均有恢复。跟踪随访的4例患儿中,2例复发,复发症状表现为仅视神经炎或脊髓炎与视神经炎均出现。结论儿童NMO可于幼儿期发病,部分有缓解复发的阶梯病程,应注意临床病灶的早发现及随访。

Objective To investigate the clinical feature,treatment response and prognosis in children with neuromyelitis optica（NMO）,in order to improve the recognition of NMO.Methods A retrospective study of 7 children with NMO was performed.All of them were admitted and diagnosis in Tianjin Children＇s Hospital from Apr.1997 to May 2010.The clinical features,laboratory tests,imaging modalities,treatment response and prognosis were all collected and evaluated.Results There were 2 boys and 5 girls in the 7 patients with NMO,and the ratio of male to female was 1.03.5.The onset was from 17 months to 15 years old,with average age at 10 years old.Among 3 cases,optica neuritis were initial symptoms.The distance between neuromyelitis and optica neuritis was 1 day to 7 months,with average 8 days.Bilateral optic neuritis were in 5 cases.Complete transverse myelitis was dominant in 5 cases.Visual evoked potential（VEP） were all examined abnormal.The thoracic spinal cord were involved in all 7 cases.Magnetic resonance imaging（MRI） with T2 signal abnormality of the spinal cord extending over 4 to 11 vertebral segments,with average 7 vertebral segments.Brain MRI lesions were found in 1 case,and another case was found positive serum autoantibodies.The number of cerebrospinal fluid cells increased slightly in 3 cases.Seven cases were treated with intravenous methylprednisolone,and following with oral prednisone.Three cases of them were also given intravenous immunoglobulin,1 case was given azathioprine.Neuromyelitis and optica neuritis symptoms were improved in 6 cases,despite 1 case gave up.Long-term follow-up showed 2 cases had relapsing disease,characterized by recurrent attacks of optica neuritis,or both neuromyelitis and optica neuritis.Conclusions NMO can occur in very young children,and some is relapsing NMO.Finding subclinical abnormality and follow-up are necessary.