CAJ | 학술논문

目的观察眼肌型重症肌无力(OMG)向全身型重症肌无力(GMG)转化患者的临床特征,分析影响GMG转化的预后因素.方法对2000年1月至2013年6月在本院诊治的770例OMG患者进行门诊随访,将在病程中出现眼外肌以外肌群受累者归为GMG转化组(197例),将在末次门诊时维持OMG者纳入OMG不变(R-OMG)组(573例),采用Logistic回归方法分析GMG转化的预后因素.结果 (1) GMG转化组较R-OMG组起病年龄大、低频重复神经电刺激(RNS)异常以及胸腺瘤比例高、早期激素治疗比例低;(2)770例OMG患者中,197例(26％)发生GMG转化,延髓型转化占GMG转化的81％ (160/197);67％ (132/197) GMG转化在2年内、84％(166/197)在5年内发生;起病年龄≥50岁、15 ～49岁较≤14岁的患者GMG转化率高、转化快,上述3个年龄段GMG转化中位数分别为起病后6 5个月、1年、10年;(3)副神经RNS异常(OR=6.650,95％ CI 3.547 ～ 12.471)、胸腺瘤(OR =7.924,95％CI2.554 ～24.585)是GMG转化的危险因素(均P＜0.05),早期激素治疗(OR=0.232,95％CI0.119～0.452,P＜0.05)是GMG转化的保护因素.结论肢体近端肌肉低频RNS异常、胸腺瘤、早期激素治疗以及15岁以上起病可能是OMG向全身型转化的预后因素.
목적 관찰안기형중증기무력(OMG)향전신형중증기무력(GMG)전화환자적림상특정,분석영향GMG전화적예후인소.방법 대2000년1월지2013년6월재본원진치적770례OMG환자진행문진수방,장재병정중출현안외기이외기군수루자귀위GMG전화조(197례),장재말차문진시유지OMG자납입OMG불변(R-OMG)조(573례),채용Logistic회귀방법분석GMG전화적예후인소.결과 (1) GMG전화조교R-OMG조기병년령대、저빈중복신경전자격(RNS)이상이급흉선류비례고、조기격소치료비례저;(2)770례OMG환자중,197례(26％)발생GMG전화,연수형전화점GMG전화적81％ (160/197);67％ (132/197) GMG전화재2년내、84％(166/197)재5년내발생;기병년령≥50세、15 ～49세교≤14세적환자GMG전화솔고、전화쾌,상술3개년령단GMG전화중위수분별위기병후6 5개월、1년、10년;(3)부신경RNS이상(OR=6.650,95％ CI 3.547 ～ 12.471)、흉선류(OR =7.924,95％CI2.554 ～24.585)시GMG전화적위험인소(균P＜0.05),조기격소치료(OR=0.232,95％CI0.119～0.452,P＜0.05)시GMG전화적보호인소.결론 지체근단기육저빈RNS이상、흉선류、조기격소치료이급15세이상기병가능시OMG향전신형전화적예후인소.
Objective To explore thc clinical manifestation of secondary generalized myasthenia gravis(GMG) and analyze the factors affecting the progression from ocular myasthenia gravis(OMG) to GMG.Methods This research constitutes a single-center,retrospectively-collected prospective cohort study.We comprehensively reviewed our self-managed myasthenia gravis (MG) database drawn from personal clinical experience from January 2000 to Junc 2013.Patients underwent series of examination including repetitive nerve stimulation (RNS) tests,measurement of serum acetylcholine receptors antibody and serum muscle-specific tyrosine kinase antibodies,thymus computer tomography scan etc.Patients were treated with pyridostigmine bromide,corticosteroid therapy and (or) thymectomy based on a nonrandomization pattern and they were documented for their respective symptoms of OMG and GMG and date of GMG conversion.Logistic regression analysis was adopted to determine the influencing factors correlated with the development of GMG during the follow-up.Results Totally 770 patients initially diagnosed with OMG were included,among whom 573 (74％) patients remained with OMG (R-OMG group) and 197(26％) patients developed into GMG (GMG group) during the follow-up.(1) In comparison with their R-OMG counterparts,patients with secondary GMG were older at onset; Displayed more frequent RNS abnormality of facial nerve,accessory nerve and ulnar nerve ; Showed higher incidence of thymoma and were less treated by early corticosteroids.(2) For GMG group,81％ (160/197) of them displayed bulbar MG; 67％ (132/197) of GMG conversion occurred within 2 years,and 84％ (166/197) within 5 years.In comparison with the patients with onset of " ≤ 14-year-old",both of patients with " 15-49-year-old" and " ≥ 50-year-old" displayed higher conversion rate and shorter conversion duration (median:10 years versus 1 year and 6.5 months).(3) RNS abnormality of accessory nerve(OR =6.650,95％ CI 3.547-12.471 ; P ＜ 0.05) and thymoma(OR =7.924,95％ CI 2.554-24.585 ; P ＜ 0.05) were prognostic factors for the development of GMG,while early corticosteroid(OR =0.232,95％ CI 0.119-0.452 ; P ＜ 0.05) predicted the reduction of the risk of generalization.Conclusions Multiple factors including abnormal RNS of proximal limb muscles,thymoma,early corticosteroids therapy and possibly even onset age of over 15-year-old may involve the generalization in patients with OMG at onset.