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 Optica Spectrum Disorders

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。

Objective： 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.