Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
Mult Scler. 2011 Sep;17(9):1107-12. doi: 10.1177/1352458511404917. Epub 2011 May 4.
Although brain abnormalities are being recognized more frequently in patients with neuromyelitis optica spectrum disorder (NMOSD), most brain lesions in previous reports have accompanied pre-existing NMOSD. Here, we describe clinical and imaging characteristics of patients with NMOSD who presented with brain symptoms as their first manifestation of the condition.
Anti-aquaporin-4 antibody was measured using cell-based assays and/or enzyme-linked immunosorbent assay in the sera of 257 patients with inflammatory diseases of the central nervous system who attended the multiple sclerosis clinic of the National Cancer Center, Korea, between May 2005 and December 2009. Eighty-three were seropositive, and 15 of these who presented with brain symptoms were included in this study. We retrospectively reviewed these individuals' clinical and radiological findings.
Patients with NMOSD were followed for a mean of 90 months. Median age at onset was 24 years (6-54 years) and there was a female preponderance (94%). The initial manifestation was classified into two groups according to clinical characteristics: encephalopathy mimicking acute disseminated encephalomyelitis or posterior reversible encephalopathy syndrome and characteristic brainstem symptoms such as intractable hiccup and vomiting. The majority of brain symptoms and lesions resolved. Intriguingly, eight patients (53%) experienced brain relapses that followed a similar pattern during the course of their disease.
It is important to recognize that NMO or NMOSD can initially present with brain symptoms.
尽管视神经脊髓炎谱系疾病（NMOSD）患者的脑异常越来越被认识，但以前的报道中大多数脑病变都伴有先前存在的 NMOSD。在此，我们描述了以脑症状为首发表现的 NMOSD 患者的临床和影像学特征。
在 2005 年 5 月至 2009 年 12 月期间，韩国国家癌症中心多发性硬化症诊所收治的 257 例中枢神经系统炎症性疾病患者的血清中使用基于细胞的测定法和/或酶联免疫吸附试验测量抗水通道蛋白 4 抗体。83 例血清阳性，其中 15 例有脑症状的患者纳入本研究。我们回顾性分析了这些患者的临床和影像学发现。
NMOSD 患者的平均随访时间为 90 个月。发病年龄中位数为 24 岁（6-54 岁），女性居多（94%）。根据临床特征，初始表现分为两组：表现为类似于急性播散性脑脊髓炎或后部可逆性脑病综合征的脑病和特征性脑干症状，如顽固性呃逆和呕吐。大多数脑症状和病变得到缓解。有趣的是，8 名患者（53%）在疾病过程中经历了类似模式的脑部复发。
重要的是要认识到 NMO 或 NMOSD 最初可能出现脑症状。
