Xing Yan, Cui Li-ying, Guan Yu-zhou, Li Ben-hong, Du Hua
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Zhonghua Yi Xue Za Zhi. 2007 Dec 4;87(45):3188-91.
To investigate the clinical and electrophysiological characteristics of myasthenia gravis with thymoma (MGT).
The clinical and repetitive nerve stimulation (RNS) data of 72 patients with MGT, 36 males and 36 females, and 63 patients with myasthenia gravis without thymoma (NMGT) were analyzed retrospectively.
The onset age of 52 of the 72 MGT patients (72.2%) was 35-59. The average onset age of the MGT patients was 39.5, older than that of the NMGT patients (29.4 yrs), and the course of the MGT patients was 13.1 months, shorter than that of the NMGT patients (29.1 months)The attack rate of the males was not significantly different than that of the females. The proportion of generalized presentation type among the MGT patients was 72.8%, higher than that among the NMGT patients (66.7%), particularly concerning the respiratory muscle (20.8% vs. 9.5%). The average time interval between the onset of symptoms and respiratory or bulbar muscle involvement of the NMGT patients were 12 and 7 months respectively, both remarkably shorter than those of the NMGT patients (26.4 and 11.6 months respectively). The abnormal RNS test yield rate of the MGT patients was 86.9%, higher than, however, not significantly that of the NMGT patients (75.0%). 97.2% of the thymoma and mediastinal abnormality could be detected through computed tomography (CT) scan on thymus. The positive rate of AchRab titer of the MGT patients was 50.0%, not significantly different from that of the NMGT patients (52.4%).
MGT is common in middle-aged people, both male and female. The Course is quickly progressive and generalized skeletal muscles, particularly respiratory muscles, are more involved in the MGT patients than in the NMGT patients. RNS study is a sensitive tool to identify MGT. AchRab may not be the specific antibody for recognizing MGT. CT scan is useful in differentiating thymoma preoperatively.
探讨伴胸腺瘤的重症肌无力(MGT)的临床及电生理特征。
回顾性分析72例MGT患者(男36例,女36例)及63例无胸腺瘤的重症肌无力(NMGT)患者的临床资料及重复神经电刺激(RNS)结果。
72例MGT患者中52例(72.2%)发病年龄为35 - 59岁。MGT患者平均发病年龄为39.5岁,高于NMGT患者(29.4岁);MGT患者病程为13.1个月,短于NMGT患者(29.1个月)。男性与女性的发病率无显著差异。MGT患者中全身型的比例为72.8%,高于NMGT患者(66.7%),尤其是呼吸肌受累方面(20.8%对9.5%)。NMGT患者症状出现至呼吸或延髓肌受累的平均时间间隔分别为12个月和7个月,均显著短于MGT患者(分别为26.4个月和11.6个月)。MGT患者RNS检查异常阳性率为86.9%,高于NMGT患者(75.0%),但差异无统计学意义。通过胸腺计算机断层扫描(CT)可检测出97.2%的胸腺瘤及纵隔异常。MGT患者乙酰胆碱受体抗体(AchRab)滴度阳性率为50.0%,与NMGT患者(52.4%)差异无统计学意义。
MGT在中年人群中常见,男女均可发病。病程进展迅速,全身骨骼肌尤其是呼吸肌受累在MGT患者中比NMGT患者更常见。RNS检查是诊断MGT的敏感方法。AchRab可能不是诊断MGT的特异性抗体。CT扫描有助于术前鉴别胸腺瘤。