摘要： 目的 分析水通道蛋白4（AQP4）抗体阳性多发性硬化的临床特征。方法 共18 例多发性硬化患者，均符合McDonald 诊断标准（2010 年版），分为AQP4 抗体阳性组（5 例）和AQP4 抗体阴性组（13 例），并门诊随访。结果 5 例AQP4 抗体阳性多发性硬化患者，男性2 例、女性3 例，中位发病年龄43 岁，中位病程4 个月；脊髓病变和视神经受累常见；MRI显示脑组织广泛多发性长T1、长T2异常信号伴颈胸髓长T1、长T2 异常信号（3/5 例）或单纯颈胸髓多发性长T1、长T2 异常信号（2/5 例）；脑脊液IgG 指数（4/4 例）、24 h IgG 鞘内合成率（3/4 例）升高，寡克隆区带阳性（3/4 例）；血清抗核抗体阳性（2/5 例）或合并干燥综合征（1/5 例）。结论 AQP4 抗体阳性多发性硬化有其特异性，发病机制可能与经典多发性硬化不同，在临床诊断与治疗时需加以重视并区别处理。
关键词:
多发性硬化,
水孔蛋白质类
Abstract: Objective To investigate the clinical features of multiple sclerosis (MS) patients with positive serum aquaporin 4 (AQP4) antibody. Methods A total of 18 MS patients who had been diagnosed in accord with McDonald Criteria (2010) were enrolled and were divided into AQP4 positive group (N = 5) and AQP4 negative group (N = 13). In combination with accessory examination, clinical features and laboratory data of MS patients were correlatively studied between 2 groups in association with follow-up study in Outpatient Clinic. Results Five seropositive patients, including 2 men and 3 women, were collected. The median age of onset was 43 years and the median clinical course was 4 months. Compared with patients with negative AQP4 antibody, MS patients with positive AQP4 antibody demonstrated increased spinal cord lesions and optic nerve involvement. MRI revealed multiple abnormal long T1 and long T2 signals in brain and cervicothoracic spinal cord (3/5), and multiple abnormal long T1 and long T2 signals in cervicothoracic spinal cord (2/5) in seropositive group. Relatively more patients in seropositive group had increased cerebral spinal fluid (CSF) IgG index (4/4) and 24 h intrathecal IgG synthesis rate (3/4), and positive oligoclonal bands (OBs, 3/4). Two patients presented with positive anti-nuclear antibody (ANA) and one Sjögren's syndrome (SS) in seropositive group. Conclusions There were some differences in clinical features between AQP4 antibody positive MS and classical MS, suggesting distinctive pathogenesis may exist between these two entities, which need to be emphasized and treated accordingly from the diagnostic and therapeutic points of view.
Key words:
Multiple sclerosis,
Aquaporins
何洋, 杨亭亭, 姜红, 向雅娟, 敖冬慧, 高旭光, 刘广志. 水通道蛋白4 抗体阳性多发性硬化临床特点分析[J]. 中国现代神经疾病杂志, 2015, 15(3): 229-234.
HE Yang, YANG Ting-ting, JIANG Hong, XIANG Ya-juan, AO Dong-hui, GAO Xu-guang, LIU Guang-zhi. Analysis of clinical features of five multiple sclerosis patients with positive serum aquaporin 4 antibody[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2015, 15(3): 229-234.