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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1.03.5。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 features and prognosis of optic neuromyelitis (NMO) in children so as to improve the understanding of this disease. Methods The subjects were 7 neonates with NMO who were hospitalized in our hospital from April 1997 to May 2010 and their clinical manifestations, laboratory and imaging examination, treatment and prognosis were retrospectively analyzed. Results 7 cases of NMO children were the first confirmed cases. 2 males and 5 females, the ratio of males to females was 1.03.5. Age onset 1 year 5 months to 15 years old, the median age of 10 years. 5 cases of history of infection before the disease. The first symptom of optic neuritis in 3 cases, 4 cases of myelitis. Optic neuritis and myelitis interval 1 d ~ 7 months, the median time 8 d. 2 cases of unilateral optic neuritis, bilateral optic neuritis in 5 cases. Traumatic spinal cord injury in 5 cases, incomplete traumatic injury in 2 cases. Visual evoked potential (VEP) abnormalities in 7 cases. Spinal cord MRI showed thoracic lesions in 7 cases, involving the lower cervical segment, lumbosacral segment in 1 case; lesion length of 4 to 11 spinal segments, median 7 spinal segments. Abnormal cranial MRI examination in 1 case. Autoantibodies abnormalities in 1 case. Cerebrospinal fluid examination increased the number of cells in 3 cases, are mainly lymphocytes, etiological examination (-). Seven patients were treated with oral prednisone after intravenous methylprednisolone in acute phase, while 3 patients with intravenous gamma globulin. In addition to 1 patient gave up treatment, more than 6 cases have recovered. Follow-up of 4 cases of children, 2 cases of recurrence, recurrence of symptoms showed only optic neuritis or myelitis and optic neuritis have emerged. Conclusion Children with NMO can develop in early childhood, and some of them can relieve the pathological process of recurrence. Early detection and follow-up of clinical lesions should be paid attention to.