延髓型重症肌无力166例的临床特点  

Bulbar myasthenia gravis:a clinical analysis of 166 patients

作　　者：王俊玲[1] 陈黛琪[1] 杨勇[1] 王雪贞[1] 杨明山[1] 卜碧涛[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院神经科,武汉北京博爱医院急诊科430030

出　　处：《中华神经科杂志》2007年第6期395-398,共4页Chinese Journal of Neurology

摘　　要：目的研究延髓型重症肌无力(MG)的临床特点。方法回顾性分析1983年5月至2005年10月间我院诊治的166例延髓型 MG 患者的临床资料,并与本院 MG 资料库中其他类型的MG 患者2722例进行比较。结果延髓型 MG 在本组患者中的发生率为5.7%(166/2888)。女性多于男性(男:女=1:1.35),发病年龄高峰为20～40岁。危象发生率高(44/166,26.5%),18例(10.8%)延髓型 MG 患者半年内发生危象,总病死率达6.0%(10/166)。肺部感染发生率高(30/166,18.1%)。早期误诊率高达19.9%(33/166)。治疗相对困难,激素"中剂量冲击、小剂量维持"疗法和中剂量环磷酰胺疗法短期疗效较好,胸腺切除术远期缓解达80.0%(20/25)。结论本组结果显示,延髓型 MG 具有不同于其他类型 MG 的临床特点,充分认识其临床特点有助于降低误诊率,提高诊断水平和治疗效果。Objective To study the clinical characteristics of bulbar myasthenia gravis （MG）. Methods Retrospective review was performed on 166 patients with bulbar type of myasthenia gravis, diagnosed at Tongji Hospital in the period of May 1983 through October 2005. Results Bulbar MG was a relatively rare type of MG, accounting for 5. 7% （ 166/2888 ） of MG classifications. Females were more often affected than males （ the ratio of male： female was 1 ： 1.35 ）. The peak of onset age was at 20-40 years. The incidence of myasthenia crisis in the group was 26.5% （44/166）. Myasthenic crisis occurred in 10. 8% （18/166） of the bulbar MG patients within 6 months after onset, resulting in a mortality rate of 6. 0% （10/166） in the group. Out of the group, 30 cases experienced pulmonary infections （ 18. 1% ）. Thirty cases were initially misdiagnosed as other diseases such as nasopharyngeal disorders （33/166, 19. 9% ）. The routine therapy was not very satisfactory. Median dose cyclophosphamide therapy appeared to be effective for ameliorating refractory MG. Thymectomy was performed in 25 patients, with optimistic efficacy rate up to 80. 0% （ 20/25 ） in a 3-year follow-up. Conclusions The clinical analysis in the current study suggested that the bulbar MG had its own characteristics in such aspects as progression of the disease, complications, treatment and prognosis. The information of the clinical manifestations presented in this study may be useful in diagnosing and treating bulbar MG.

关 键 词：重症肌无力 胸腺瘤 回顾性研究 

分 类 号：R746.1[医药卫生—神经病学与精神病学]