特发性脱髓鞘性视神经炎的临床分析和长期随访观察

Analysis on the clinical feature of idiopathic demyelinating optic neuritis and long term follow-up

目的:了解特发性脱髓鞘性视神经炎(idiopathetic demyelinated optic neuritis,IDON)的自然病程、发病特点及临床转归,并与西方国家视神经炎的发病特征进行比较。方法:对2009-05/2010-12就诊于解放军总医院确诊为特发性脱髓鞘性视神经炎的137例患者的临床资料进行回顾性分析。结果:特发性脱髓鞘性视神经炎男女发病率之比为1∶1.54,74例患者以双眼发病(其中29例双眼同时发病),63例患者单眼发病。28.9%患者伴有眼球转动痛,69.7%眼表现为球后视神经炎,30.3%眼表现为视神经乳头炎。58.4%患者预后视力>0.5,仍有21.8%患者的预后视力<0.1。低年龄组(≤18岁)患者视力预后较好,约73.1%患者视力>0.5;中年龄组视力预后次之,约56.2%患者视力>0.5;高年龄组(≥46岁)患者视力预后较差,仅47.3%患者视力>0.5,三组统计学比较有显著性差异(P<0.05)。137例患者中共89例进行了水通道蛋白-4(AQP-4)的检测,28例(31.5%)结果阳性。我们发现AQP-4阳性组视力预后较差,和AQP-4阴性组比较统计学有显著性差异(P<0.05)。在随后2a的随访中,28例AQP-4阳性组的患者中,5例发展为视神经脊髓炎(optic neuromyelitis,NMO),1例发展为多发性硬化(multiple sclerosis,MS);在61例AQP-4阴性患者中,仅1例患者发展为NMO。结论:特发性脱髓鞘性视神经炎的自然病程和主要临床特点与西方国家报告较为相似,但也有不同之处。AQP-4可能对孤立性视神经炎患者具有诊断和提示预后的价值,AQP-4阳性提示视力预后较差,可能发展为NMO的危险性增加。

AIM： To investigate the course of disease, clinical characteristic and clinical outcome of patients with idiopathic demyelinating optic neuritis （IDON） in final diagnosis and compare the results with those in western countries. METHODS： Clinical features of 137 cases of IDON with final diagnosis in the General Hospital of Chinese PLA form May 2009 to December 2010 were retrospectively analyzed. RESULTS： With a female to male ratio of 1：1.54, 74 cases had onset in both eyes or one after another, and 63 cases had onset with simple eye. There were 28. 9% patients accompanied with oculogyria pain, 69.7% eyes with retrobulbar neuritis and 30.3% eyes with papillitis. There were 58.4% cases with visual acuity recovered to more than 0.5; but there were also 21.8% cases with visual acuity recovered to less than 0.1, The patients in lower age group （〈18 years） had a better recovery ofvisual acuity and those in higher age group （ 〉146 year） at the same time had a bad visual acuity. There were significant differences between two groups （ P〈 0.05）. There were 89 cases performed aquaporin - 4 （ AQP- 4 ） autoantibodies assay. The results of 28 cases （31.5%） were seropositivity. The patients in seropositivity group had better recovery of visual acuity and there were significant differences compared to seronegativity group. In the two follow- up years, 5 out of 28 seropositive patients were diagnosed as optical neuromyelitis （NMO） and 1 out of 28 was diagnosed as multiple sclerosis （MS） finally~ 1 out of 61 seronegative patients were diagnosed as NMO finally. ~ CONCLUSION： Despite of minor differences of clinical feature, the course of IDON is similar to that reported in western countries, AQP- 4 autoantibodies may have diagnostic and prognostic value in patients who present with isolated ON. The seropositivity of AQP-4 may mean the worse recovery of sight and increase the chances of the development of NMO.