儿童视神经脊髓炎的临床特征及预后

Clinical characteristics and prognosis of pediatric neuromyelitis optica

目的分析儿童视神经脊髓炎(p-NMO)的临床特征及预后。方法回顾性分析2009-01-2012-10中国人民解放军总医院诊治的14例p-NMO的临床资料。结果 14例p-NMO首次发病年龄为5.0～13.6周岁,中位数为9.7岁,四分位数间距为4.3岁,男女比例为1∶6。4例p-NMO存在可能的诱发因素。以视神经炎(ON)为首发症状者6例,以ON及横贯性脊髓炎(TM)首发2例,以TM首发3例,以脑部症状首发2例,以ON合并脑部症状首发1例。视觉诱发电位(VEP)均异常;12例行水通道蛋白4抗体(AQP4-Ab)检测,6例阳性;均行血清风湿抗体检测,6例阳性。均为复发-缓解病程。通过电话随访(随访时间中位数为14.5个月),1例借助轮椅行走,1例双目失明,其余日常生活基本不受影响;EDSS评分中位数为2.0分,四分位数间距为1.3分。结论 p-NMO女性多见且首发症状可能以ON为主,尤以双侧ON突出。AQP4-Ab对p-NMO敏感度可能较成人低。p-NMO的预后可能较成人NMO好;后遗症可能以视力障碍为主,脊髓炎后遗症较轻。

Objective To analyze the clinical characteristics and prognosis of pediatric neuromyelitis optica（p-NMO）. Methods A retrospective analysis was performed evaluating 14 p-NMO patients presenting in chinese PLA General hospital from January of 2009 to October of 2012. Results Median（interquartile range） age at symptom onset was 9.7（4.3） years old,and age of onset ranged from 5.0 to 13.6 years old.Gender ratio（m∶f） was 1∶6.There were possible precipitating factors in 4 cases of p-NMO.The patients had attacks of optic neuritis（ON）（n=6）,transverse myelitis（TM）（n=3）,both of the above（n=2）,brain symptom（n=2） or ON combining brain symptom（n=1） at disease onset.The visual evoked potentials（VEP）of all patients were abnormal;AQP4-Ab was positive in 6 patients of the 12 detected.Additional autoantibodies were measured in all patients,and 6 had positive autoimmune antibodies.Attacks were recurrent in all patients.One patient walks with wheelchair assisting,one blind with two eyes,while the others live almost normally（the median of follow-up duration is 14.5 months by telephone）.The median（interquartile range） of Expanded Disability Status Scale（EDSS） scores was 2.0（1.3） after 39.5（21.5）months. Conclusions P-NMO is more common in girls,and the first symptom onset mainly presents as ON,especially bilateral ON.The positive rate of AQP4-Ab in childhood is probably less sensitive than that in adult.NMO and AQP4-Ab may be related to the prognosis.The prognosis of p-NMO is better,and residual disability is mainly visual impairment.