Department of Family Medicine, Chi-Mei Medical Center, Yong Kang Campus, 901 Chung Hwa Road, Tainan, Taiwan.
Pediatr Neonatol. 2010 Dec;51(6):347-52. doi: 10.1016/S1875-9572(10)60067-8.
Neuromyelitis optica (NMO, Devic's syndrome) is a demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. It is often confused with multiple sclerosis. Early discrimination between NMO and multiple sclerosis is important because the two diseases have different natural histories and treatment regimens. Seropositivity for NMO-IgG and longitudinally extensive spinal cord lesions (3 or more spinal segments) are characteristic of NMO. Despite the absence of a definitive therapeutic strategy for NMO syndrome, methylprednisolone pulse therapy is recommended in the acute phase. Treatment strategies in relapse phases are aimed at preventing relapses, and increasing evidence shows a better clinical response of immunosuppressive therapy than immuno-modulating therapy (a standard multiple sclerosis-modulating therapy). We describe a 10-year-old girl who had visual loss due to acute optic neuritis at 6 years old and suffered repetitive myelitis 2 years later. NMO was diagnosed because of characteristic longitudinal myelitis and positive NMO-IgG. After combining therapy with prednisolone and an immunosuppressant (cyclophosphamide), the patient's medical condition was stable and no relapse symptoms were observed.
视神经脊髓炎(NMO,Devic 综合征)是一种中枢神经系统脱髓鞘疾病,主要影响脊髓和视神经。它常与多发性硬化症混淆。早期区分 NMO 和多发性硬化症很重要,因为这两种疾病具有不同的自然病史和治疗方案。NMO-IgG 血清阳性和纵向广泛脊髓病变(3 个或更多脊髓节段)是 NMO 的特征。尽管 NMO 综合征没有明确的治疗策略,但建议在急性期使用甲基强的松龙脉冲治疗。复发期的治疗策略旨在预防复发,越来越多的证据表明免疫抑制治疗比免疫调节治疗(一种标准的多发性硬化症调节治疗)有更好的临床反应。我们描述了一位 10 岁女孩,她在 6 岁时因急性视神经炎导致视力丧失,2 年后又因反复性脊髓炎而发病。由于特征性的纵向脊髓炎和 NMO-IgG 阳性,诊断为 NMO。在联合使用泼尼松和免疫抑制剂(环磷酰胺)治疗后,患者病情稳定,未出现复发症状。