摘要：

目的 基于2015 年版视神经脊髓炎谱系疾病诊断标准，探讨血清NMO-IgG 阳性和阴性患者临床和影像学特征。方法 回顾分析96 例视神经脊髓炎谱系疾病患者（包括NMO-IgG 阳性64 例、NMO-IgG 阴性32 例）的临床和影像学表现。结果 NMO-IgG 阳性和阴性患者首次发病均以脊髓受累常见，发生率分别为48.44%（31/64）和56.25%（18/32），其中感觉障碍（肢体麻木）发生率最高。NMO-IgG 阳性组首发视神经受累症状以单侧视力下降常见（12/16）、NMO-IgG 阴性组以双侧视力下降常见（6/9），NMO-IgG 阳性组最后区受累的顽固性呃逆、呕吐症状较NMO-IgG 阴性组多见（11/18 对2/7），组间差异均无统计学意义（P = 0.087，0.202）。两组患者首次发病时脊髓病灶均以颈髓常见［41.67%（15/36）和11/17］，NMO-IgG 阳性组颈髓-胸髓连续病灶发生率［41.67%（15/36）对1/17］和病灶≥ 7 个椎体节段比例［36.11%（13/36）对1/17］均高于NMO-IgG 阴性组（P = 0.008，0.022），而颅内病灶发生率［90.63%（29/32）对14/17］和延髓-颈髓连续病灶发生率（6/11 对1/2）组间差异无统计学意义（P = 0.702，1.000）。两组患者病程中脊髓病灶发生率［84.38%（54/64）对84.38%（27/32）］、延髓-颈髓连续病灶发生率（9/19 对3/6）和颅内病灶发生率［86.54%（45/52）对83.33%（25/30）］差异均无统计学意义（P = 1.000，1.000，0.934）。结论 基于2015 年版诊断标准分析我国血清NMO?IgG 阳性和阴性的视神经脊髓炎谱系疾病患者的临床和影像学特征，有助于进一步提高疾病的诊断与治疗水平。

关键词: 视神经脊髓炎, 水通道蛋白质4, 参考标准, 磁共振成像

Abstract:

Objective  To analyze the clinical and imaging features of serum NMO-IgG-positive and NMO-IgG-negative Chinese patients with neuromyelitis optica spectrum disorders (NMOSDs) based on the 2015 version of diagnostic criteria.  Methods  Retrospective analyses of clinical and imaging data were held on 96 patients with NMOSDs (64 NMO-IgG-positive patients and 32 NMO-IgG-negative patients) who were admitted to Tangdu Hospital of the Fourth Military Medical University from January 2013 to December 2015.  Results  Gender ratio, age, age of onset, duration and annual relapse rate between NMO-IgG-positive and NMO-IgG-negative patients were of no significant difference. Symptoms of spinal cord involvement were most common during the first attack, with incidence rate of 48.44% (31/64) and 56.25% (18/32), respectively, for NMO-IgG-positive and NMO-IgG-negative patients, while limb numbness was most frequent. Among the first-episode optic nerve symptoms, unilateral visual loss was more common in NMO-IgG-positive patients (12/16) and bilateral visual loss was more frequent in NMO-IgG-negative patients (6/9). Intractable hiccups and vomiting suggestive of area postrema involvement was more common in NMO-IgG-positive group than that in NMO-IgG-negative group (11/18 vs 2/7), but the difference between 2 groups was not significant (P = 0.087, 0.202). The first-episode lesions of spinal cord were most frequently located in the cervical cord in both groups [41.67% (15/36) vs 11/17]. The incidence rates of both cervicothoracic cord lesions [41.67% (15/36) vs 1/17] and spinal cord lesions ≥ 7 vertebral segments [36.11% (13/36) vs 1/17] were significantly higher in NMO-IgG-positive group than those in NMO-IgG-negative group (P = 0.008, 0.022). The occurrence rates of intracranial lesions [90.63% (29/32) vs 14/17] and contiguous lesions involving medulla and cervical cord (6/11 vs 1/2) between 2 groups had no significant difference (P = 0.702, 1.000). During the disease course, there were no significant differences in the incidence of spinal cord [84.38% (54/64) vs 84.38% (27/32)], contiguous lesions involving medulla and cervical cord (9/19 vs 3/6) and intracranial lesions [86.54% (45/52) vs 83.33% (25/30)] between 2 groups (P = 1.000, 1.000, 0.934).  Conclusions  Analysis of clinical and imaging features between NMO-IgG-positive and NMO-IgG-negative Chinese patients with NMOSDs according to the 2015 version of diagnostic criteria is helpful to further improve diagnostic level of the disease.

Key words: Neuromyelitis optica, Aquaporin 4, Reference standards, Magnetic resonance imaging