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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 features of medullary myasthenia gravis (MG). Methods The clinical data of 166 patients with medullary MG diagnosed and treated in our hospital from May 1983 to October 2005 were retrospectively analyzed and compared with 2722 patients with other types of MG in our MG database. Results The incidence of medullary MG in this group was 5.7% (166/2888). More women than men (male: female = 1: 1.35), the peak age of onset of 20 to 40 years old. Crisis rates were high (44/166, 26.5%). 18 (10.8%) patients with medullary MG developed a crisis within six months with a total case fatality rate of 6.0% (10/166). High incidence of pulmonary infection (30 / 166,18.1%). Early misdiagnosis rate as high as 19.9% (33/166). Treatment is relatively difficult, hormone “medium dose impact, low dose maintenance” therapy and medium dose of cyclophosphamide therapy is good short-term efficacy, thymectomy long-term relief of up to 80.0% (20/25). Conclusion The results of this study show that medullary MG has clinical features different from those of other types of MG. To fully understand its clinical features may help to reduce the rate of misdiagnosis, improve the diagnostic level and therapeutic effect.