| 陈彬，李尧，王瑞金，王淑辉，张拥波.视神经脊髓炎谱系疾病临床特征分析及
症状异质性研究[J].神经损伤功能重建,2019,14(4):169-172 |
| 视神经脊髓炎谱系疾病临床特征分析及
症状异质性研究 |
| Clinical Analysis and Research on Atypical Manifestation of Neuromyelitis Optica SpectrumDisorders |
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| DOI： |
| 中文关键词: 视神经脊髓炎谱系疾病 非典型临床症状 抗水通道蛋白4抗体 抗髓鞘少突胶质细胞糖蛋白抗体 |
| 英文关键词: neuromyelitis optica spectrum disorders atypical clinical manifestations AQP4-IgG MOG-IgG |
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| 中文摘要: |
| 目的：探讨视神经脊髓炎谱系疾病（NMOSD）的临床特征，分析非典型临床症状的异质性。方法：纳
入符合NMOSD诊断标准的患者36例，收集患者临床资料，包括临床特征、影像学表现、实验室检查、治疗
及随访等，并进行回顾性分析。结果：36例患者中男∶女=7∶29；平均年龄（43.5±6.4）岁；甲状腺功能亢进病
史 2例，过敏性哮喘病史1例，干燥综合征病史1例；首发症状为顽固性恶心，呕吐4例、感觉异常4例、味觉
减退1例、视神经炎表现的10例、脊髓炎表现17例；非典型首发症状比例为5/36。血清抗水通道蛋白抗体
（AQP-4）阳性14例（14/32），抗髓鞘少突胶质细胞糖蛋白抗体（MOG-IgG）阳性10例（10/25）。核磁检查可见
大脑皮质、中脑、脑桥、延髓、脊髓异常病灶。36例患者均行糖皮质激素冲击治疗，行血浆置换治疗1例，后
续使用硫唑嘌呤口服治疗5例。25例患者遗留不同程度的后遗症。结论：NMOSD好发于青年女性，部分可
合并其他自身免疫疾病；首发症状及病程中出现的症状表现复杂多样，病变以视神经，脊髓，脑部受累为
主。对于临床表现不特异的患者需高度警惕NMOSD的可能。 |
| 英文摘要: |
| To explore the clinical features in neuromyelitis optica spectrum disorders (NMOSD)
and analyze the heterogeneity of atypical clinical manifestations. Methods: The clinical data of 36 patients with
NMOSD were collected. Their clinical profiles, MRI features, laboratory examination, treatment, and prognosis
were analyzed. Results: Of the 36 patients, the male to female ratio was 7∶29. The average age was (43.5±6.4)
years. There were 2 cases with history of hyperthyroidism, 1 case with history of allergic asthma, and 1 case with
history of Sjogren’s syndrome. Initial manifestations include intractable nausea and vomiting in 4 cases,
paresthesia in 4 cases, hypogeusia in 1 case, optic neuritis symptoms in 10 cases, and myelitis symptoms in 17
cases. The ratio of atypical initial manifestations was 5/36. There were 14 cases that were positive for serum
AQP4-IgG (14/32) and 10 cases that were positive for serum MOG-IgG (10/25). Magnetic resonance imaging
(MRI) showed abnormal signals in the cerebral cortex, midbrain, pons, oblongata, and spinal cord. All 36 patients
were treated with glucocorticoid; among them, 1 received plasmapheresis and 5 received azathioprine by oral
dosage. Follow up showed that 25 patients experienced different degrees of sequelae. Conclusion:
Neuromyelitis optica spectrum disorders frequently occur in young females. Some cases may exist in
combination with other autoimmune diseases. Manifestations in the initial stage and course of disease vary
greatly. Lesions occur mainly in the optic nerve, spinal cord, and brain. NMOSD should be considered in patients
with atypical clinical manifestations |
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