| 邓兵梅，康健捷，杨红军，邓文婷，项薇，熊铁根，彭凯润.血清NMO-IgG阳性视神经脊髓炎谱系疾病患者的
临床分析[J].神经损伤功能重建,2018,13(9):448-452 |
| 血清NMO-IgG阳性视神经脊髓炎谱系疾病患者的
临床分析 |
| Analysis of Clinical Features of NMO-IgG-Positive Patients with Neuromyelitis OpticaSpectrum Disorders |
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| DOI： |
| 中文关键词: 视神经脊髓炎谱系疾病 神经脊髓炎-免疫球蛋白G 磁共振成像 诊断 |
| 英文关键词: neuromyelitis optica spectrum disorders NMO-IgG magnetic resonance imaging diagnosis |
| 基金项目:广东省科技计划项
目（No.2015077） |
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| 中文摘要: |
| 目的：探讨血清视神经脊髓炎-免疫球蛋白G（NMO-IgG）阳性的视神经脊髓炎谱系疾病（NMOSDs）患
者的临床和影像特点。方法：纳入34例NMO-IgG阳性NMOSDs患者，回顾性分析其临床表现、实验室指标
和影像学结果。结果：34例NMOSDs患者中，男女比例为1∶16，首发年龄为（44.65±5.27）岁。首发症状为感
觉异常（52.94%），肢体无力（44.12%），视力下降（29.41%），低热、嗜睡（8.82%），顽固性呃逆呕吐（8.82%），复
视（2.94%），行走不稳（2.94%）。临床综合征为脊髓炎（82.35%），视神经炎（50.00%），最后区综合征
（14.70%），间脑综合征（8.82%），大脑综合征伴有 NMOSD 特征性大脑病变（17.65%），其他脑干综合征
（14.70%）。临床发作次数1～8次，平均（2.41± 0.63）次，24例（70.59%）为复发-缓解病程，10例（29.41%）为
单相病程。MRI 表现：脑内病灶的发生率依次为脑干 35.29%、大脑半球 17.65%、间脑 8.82%、视交叉
8.82%。脊髓病灶以颈胸髓（50.00%）同时累及最为常见，其次为胸髓（17.65%）、颈髓（14.70%）。长节段脊
髓受累（≥3个节段）24例（70.59%），其中11例（32.35%）≥10个椎体节段。免疫指标：血清NMO⁃IgG阳性率
100%，脑脊液NMO-IgG阳性率29.41%；抗核抗体阳性率38.24 %；甲状腺过氧化物酶抗体增高率32.35%；甲
状腺球蛋白增高率17.65%。结论：NMO-IgG阳性NMOSDs患者以中年女性多见，首发症状以感觉异常、肢
体无力及视力下降为多，脊髓炎和神经炎是最常见的临床综合征，多表现为复发-缓解病程；MRI病灶以颈
胸髓同时受累最为常见，且多≥3个脊柱节段；常合并多种免疫指标异常 |
| 英文摘要: |
| To explore the clinical manifestations and MRI features in serum NMO-IgG-positive
patients with neuromyelitis optica spectrum disorders (NMOSDs). Methods: Thirty-four serum NMO ⁃
IgG-positive patients with NMOSDs were recruited to the study. Retrospective analyses of clinical material,
laboratory statistics, and imaging data were performed. Results: Among the 34 patients with NMOSDs, the
male to female ratio was 1∶16, and the average onset age was (44.65±5.27) years old. Symptoms during the first
attack were abnormal sensation (52.94%), limb weakness (44.12%), diminished vision (29.41%), low-grade fever
and somnolence (8.82% ), intractable hiccups and vomiting (8.82% ), diplopia (2.94% ), and stumble (2.94% ).
Clinical syndromes included myelitis (82.35% ), optic neuritis (50.00% ), area postrema syndrome (14.70% ),
diencephalic syndrome (8.82% ), cerebral syndrome with NMOSD-typical brain lesions (17.65% ), and other
brainstem syndromes (14.70% ). The number of attacks was 1~8, with an average of (2.41±0.63). Twenty-four
patients (70.59% ) experienced a relapsing-remitting disease course, and 10 patients experienced a monophasic
disease course. MRI showed occurrence rates of intracranial lesions to be 35.29% in the brainstem, 17.65% in the
cerebral hemisphere, 8.82% in the diencephalon, and 8.82% in the optic chiasma. Spinal cord lesions were most
frequently located in the cervicothoracic cord (50.00%) and were also commonly in the thoracic cord (17.65%)
and cervical cord (14.70% ). Twenty-four patients (70.59% ) showed long spinal cord lesions (≥3 vertebral
segments), and among these, the lesions of 11 patients (32.35% ) extended over 10 vertebral segments. Serum
NMO ⁃ IgG-positive rate was 100% , and cerebrospinal fluid (CSF) NMO ⁃ IgG-positive rate was 29.41% .
Antinuclear antibody (ANA) positive rate was 38.24%; thyroid peroxidase antibody increase rate was 32.35%;
thyroglobulin antibody increase rate was 17.65%. Conclusion: Serum NMO-IgG positivity with NMOSDs is
commonly seen in middle-aged females. Abnormal sensation, limb weakness, or visual loss is usually one of the
primary symptoms during onset. Myelitis and optic neuritis are common clinical symdromes. Most patients
experience a relapsing-remitting course of disease. The focal lesion is usually located in the cervicothoracic cord,
spanning ≥3 spinal segments. Additionally, abnormal immune parameters are frequently seen. |
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