核磁共振阴性视神经脊髓炎谱系病1例报告.docVIP

核磁共振阴性视神经脊髓炎谱系病1例报告 　　摘 要 目的：报告少见的核磁共振（MRI）阴性的视神经脊髓炎谱系病（NMOSD）1例。方法：对1例MRI阴性的NMOSD患者的临床资料进行回顾性总结。结果：女患者于2012年9月曾因视神经炎口服强的松后好转。2013年1月因顽固性呃逆、呕吐住我院，查血水通道蛋白4（AQP4）抗体呈强阳性，头颅及脊髓MRI检查均阴性，消化系统检查无异常发现，结合“视神经炎”病史，诊断为NMOSD，予甲强龙冲击治疗后呕吐呃逆消失。结论： 顽固性呃逆、呕吐是NMOSD的特异性症状，为脑干背侧呕吐中枢受累所致，但有时头颅MRI未必在相应部位有所发现，此时仍应警惕NMOSD的可能。 关键词 视神经脊髓炎谱系病 核磁共振 水通道蛋白4 中图分类号：R744.52 文献标识码：B 文章编号：1006-1533（2014）11-0028-03 Neuromylitis optica spectrum disorder with normal brain and spinal cord MRI images： a case report* CAI Tongjia1**， XUE Yuanyuan1， QUAN Chao2*** （1.Department of Neurology， Shanghai Jingan District Central Hospital； 2.Department of Neurology， Huashan Hospital， Fudan University， Shanghai 200040， China） ABSTRACT Objective： To report a rare case of neuromylitis optica spectrum disorder （NMOSD） with normal brain and spinal cord MRI images. Methods： Clinical data on a patient with NMOSD but without positive findings on MRI were retrospectively summarized. Results： A 30 years old female had suffered from optic neuritis in September 2012 but was improved after treatment with oral prednisone. In January 2013 she was hospitalized again because of intractable hiccups， vomiting. Serum AQP-4 antibody was strongly positive， while cranial and spinal MRI were normal and examinations of digestive system showed no abnormalities. She was diagnosed as NMOSD， and the hiccups and vomiting disappeared after pulse therapy with high-dose methylprednisolone. Conclusion： Intractable hiccups， vomiting are characteristic symptoms of NMOSD due to the lesions in dorsal brainstem. Sometimes these lesions would not cause alterations of MRI signals， however， the possibilities of NMOSD should still be kept in mind. KEY WORDS neuromyelitis optica spectrum disorder； aquaporin 4； magnetic resonance imaging 视神经脊髓炎谱系病（neuromyelitis optica spectrum disorders，NMOSD）是一组潜在免疫病理机制与视神经脊髓炎（Neuromyelitis optica，NMO）相近，但临床受累局限，不完全符合NMO诊断标准的一组神经系统脱髓鞘疾病[1]。以顽固性呃逆、呕吐为主要表现，头颅MRI检查阴性的NMOSD少见报道，现将本院收治的1例报道如下。 1