视神经脊髓炎谱系疾病在长期免疫抑制治疗前的病程特点及预后分析

Characteristics of disease course and prognosis of neuromyelitis optica spectrum disorder patients

目的了解中国视神经脊髓炎谱系疾病(NMOSD)患者有效免疫抑制治疗前的病程特点和预后。方法对122例中国NMOSD患者的首次发病症状、复发情况和神经功能障碍情况进行回顾性分析,应用KaplanMeier方法分析预后。结果 122例NMOSD患者(女性占86%)的首次发病症状,41例有视神经炎表现,43例累及脊髓,41例累及延髓最后区,累及脑部其他位置的15例,视神经和脊髓同时受累的仅2例。122例中116例(95%)病程中有复发,78例(67%)在发病1年内、94例(77%)在发病2年内复发,年复发率中位数(最小值~最大值)1.30(0.05~10.00)次/年。首次发作后,3个月后恢复期扩展残疾状态量表(EDSS)评分中位数(最小值~最大值)为0(0~7),仅6例(5%)不能独立行走(EDSS评分≥6);第2次临床发作后恢复期,99例(81%)无或仅有很轻微神经功能障碍(EDSS评分≤3),8例(7%)EDSS评分≥6。预后分析结果显示发病10年后约20%患者不能独立行走,25%至少单眼盲。结论此组中国NMOSD绝大多数呈复发性病程,虽有较明显的致残性,但前两次发作后仅有小部分患者遗留神经功能障碍。

Objective To explore the characteristics of near natural disease course and prognosis of neuromyelitis optica （NMO） spectrum disorder （NMOSD） in a large Chinese cohort.Methods We performed a retrospective cohort study of 122 Chinese NMOSD patients fulfilling the 2006 NMO criteria or the 2015 NMOSD criteria.Clinical characteristics, disease course before prophylaxis immunosuppressants initiation and disability of these NMOSD patients were analyzed.Results Among the 122 NMOSD patients, 105 patients were female （86%）.Forty-one patients presented（with or without other symptoms, same below） with optic neuritis, 43 with myelitis and 41 with intractable hiccup and nausea.Only 2 patients presented with both optic neuritis and myelitis.Compared with 31 of the 105 female patients（30%）,most of th male patients （10 of 17, 59%） presented with optic neuritis （P=0.026）.The patients with the onset age of 20 or younger were more prone to present with optic neuritis （9 of 19, 47%）, while half of the 8 patients with the onset age older than 60 presented with myelitis （P〈0.05）.Among the 116 NMOSD patients with at least 2 attacks, 78 （67%） relapsed within 1 year and 94（77%） relapsed within 2 years.The median EDSS after the 2nd attack was 1 （0-8）.Only 8 （7%） patients had an EDSS≥6 and 99 （81%） patients had none or only minimal disability （EDSS≤3） 3 months after the relapse.Ten years after disease onset, 20% of the patients could not walk independently （EDSS≥6） and approximately 25% of them legally blind （visual acuity of 20/200 or less） in at least one eye.Conclusions Most of the 122 Chinese NMOSD patients had relapsing courses.Adequate immunosuppressant should be initiated after relapses or soon after disease onset if any sustained disability developed.