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目的：分析中国西北部重症肌无力患者的临床特点。方法：收集2006 年1 月～2013 年9 月第四军医大学唐都医院神经内科及胸外科收治的重症肌无力患者，应用重症肌无力定量评分表(QMGs)进行病情评分，胸腺组织学类型以WHO分类为主，同时对部分患者进行随访观察。结果：共纳入1221 例重症肌无力患者，包括男性595 例，女性626 例。Ⅰ型MG 患者的起病年龄最轻，其次是Ⅲa；各临床类型MG患者的病程比较均无明显统计学差异(P>0.05)；Ⅳb 型MG患者的QMGs 评分最高，病情最严重，Ⅰ型最低，病情最轻。合并胸腺增生的MG 患者起病年龄最轻，其次是胸腺正常患者，合并胸腺瘤患者的起病年龄最大，显著大于合并胸腺增生者(P<0.05)；不同胸腺病理状态MG 患者的QMGs比较无统计学差异(P>0.05)。合并不同类型胸腺瘤MG患者的起病年龄、QMGs 比较均无明显统计学差异(P>0.05)。与住院期间QMGs相比，随访患者各组QMGs 均较前明显下降，且以胸腺瘤各组 QMGs 评分下降显著。结论：①眼肌型MG患者起病年龄最轻，临床症状也最轻；②胸腺增生患者起病年龄较胸腺瘤明显早，而胸腺瘤不同病理分型间起病年龄无明显区别；③胸腺手术切除患者病情恢复明显优于未切除胸腺患者，提示手术切除胸腺对于治疗MG 是行之有效的方法。

英文摘要:

Objective:To analyze the clinical characteristics in 1221 Northwestern Chinese myasthenia gravis patients.Methods:Collect data of myasthenia gravis patients in inpatient Neurology Department and Thoracic Surgery, Tangdu Hospital, Fourth Military Medical University, from January 2006 to 2013 September. All the patients were assessed the clinical severity by quantitative myasthenia gravis score (QMGs). Thymus histology was based on WHO classification. Some patients were followed up. P <0.05 was considered statistically significant.Results:The enrolled MG patients in Neurology Department and Thoracic Surgery were 1221, 595 males and 626 females. The onset age in Ⅰ was lowest. There was no difference was found in different clinical type. The highest QMGs was found in type Ⅳb and lowest in Ⅰ . The onset age in thymic hyperplasic patients was younger than that in thymoma. However, no difference of QMGs was found between thymic hyperplasic and thymoma. The follow up study showed lower QMGs in thymoma.Conclusion:① The onset age of ocular MG patients was lowest and their severity of clinical symptoms as well. ② The onset age of thymic hyperplasia patients were lower than that of thymoma patients. There was no significant difference among the onset age in all type thymoa. ③ The outcome of thymectomy in MG patients was better than that without operation which indicate that surgery for MG patients is an effective way.

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