Yang Chun-Sheng, Zhang Da-Qi, Wang Jing-Hua, Jin Wei-Na, Li Min-Shu, Liu Jie, Zhang Cun-Jin, Li Ting, Shi Fu-Dong, Yang Li
Department of Neurology and Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin, China.
CNS Neurosci Ther. 2014 Jan;20(1):32-9. doi: 10.1111/cns.12156. Epub 2013 Jul 27.
To investigate the clinical characteristics and sera anti-aquaporin 4 (AQP4) antibody positivity in patients with inflammatory demyelinating disorders (IDDs) of the central nervous system (CNS) in Tianjin, China.
We retrospectively evaluated 234 patients with IDDs including neuromyelitis optica (NMO), recurrent optic neuritis (rON), longitudinally extensive transverse myelitis (LETM), clinically isolated syndrome (CIS), and multiple sclerosis (MS) groups. Sera from 217 patients were determined for AQP4-Ab. The clinical characteristics and sera anti-AQP4 positivity were compared.
The IDDS comprised 63 MS, 51 NMO, 56 LETM, 10 rON, and 54 CIS. Compared with MS, NMO had a higher frequency of occurrence in women, intractable hiccup and nausea (IHN), medullospinal lesion, longitudinally extensive spinal cord lesions (LESCL) and bilateral ON, disease onset at a later age, and worsening residual disability. AQP4-Ab-positive rates were 84.1% and 69% in NMO and NMO spectrum disorders (NMOSD), respectively, whereas it was undetectable in all of the MS sera samples.
We comprehensively contrast the distinct clinical features of MS, NMO, and NMOSD in our center. A sensitive AQP4-Ab assay is necessary for the early diagnosis of NMOSD in our patients. Neither medullospinal lesion nor IHN is unique in NMO.
调查中国天津中枢神经系统(CNS)炎性脱髓鞘疾病(IDDs)患者的临床特征及血清抗水通道蛋白4(AQP4)抗体阳性情况。
我们回顾性评估了234例IDDs患者,包括视神经脊髓炎(NMO)、复发性视神经炎(rON)、长节段横贯性脊髓炎(LETM)、临床孤立综合征(CIS)和多发性硬化(MS)组。测定了217例患者的血清AQP4-Ab。比较了临床特征和血清抗AQP4阳性情况。
IDDs包括63例MS、51例NMO、56例LETM、10例rON和54例CIS。与MS相比,NMO在女性中的发生率更高,有顽固性呃逆和恶心(IHN)、延髓脊髓病变、长节段脊髓病变(LESCL)和双侧视神经炎,发病年龄较晚,残留残疾恶化。NMO和NMO谱系障碍(NMOSD)的AQP4-Ab阳性率分别为84.1%和69%,而所有MS血清样本中均未检测到。
我们全面对比了本中心MS、NMO和NMOSD的不同临床特征。灵敏的AQP4-Ab检测对我们患者中NMOSD的早期诊断是必要的。延髓脊髓病变和IHN在NMO中均不具有独特性。