早发型和迟发型重症肌无力的临床特点分析  

Clinical differences between early- and late-onset myasthenia

作　　者：母艳蕾[1] 张华[1] 国红[1] 陈海波[1] 侯世芳[1] 殷剑[1] 郝洪军[2] 高宇 Mu Yanlei;Zhang Hua;Guo Hong;Chen Haibo;Hou Shi fang;Yin J ian;Hao Hongj un;Gao Yu(Department of Neurology, Beij ing Hospital, National Center of Gerontology, Beij ing 100730, China （Mu YL, Zhang H, Guo H, Chen HE, Hou SF,Yin J, Gao Y;Department of Neurology, Peking University First Hospital, Beijing 100034, China（ Hao HJ)

机构地区：[1]北京医院国家老年医学中心神经内科,100730 [2]北京大学第一医院神经内科实验室,100034

出　　处：《中华老年医学杂志》2018年第5期510-513,共4页Chinese Journal of Geriatrics

摘　　要：目的分析早发型重症肌无力（EOMG）和迟发型重症肌无力（LOMG）患者的临床特点。方法回颐性研究，纳入157例重症肌无力（MG）患者，根据发病年龄分为年龄小于等于50岁为EOMG组85例，大于等于50岁为LOMG组72例。对两组患者的临床症状、肌无力分型、重复电刺激（RNS）、单纤维肌电图（SFEMG）、乙酰胆碱受体抗体（Ach—RAb）、肌肉特异性激酶抗体（MuSKAb）、抗连接蛋白抗体（Titin Ab）和抗兰尼碱受体抗体（RyRAb）及甲状腺功能、外科手术情况、胸腺病理、治疗方案进行分析。结果EOMG组和LOMG组患者平均起病年龄为（40．9±9．7）岁、（62．0±12．2）岁。LOMG组和EOMG组患者眼肌型E36例（50．0％）比28例（32．9％）]、全身型E36例（50．0％）比57例（67．1％）]、球部症状F30例（41．7％）比20例（23．5％）]差异有统计学意义（P〈0．05）。两组患者RNS、SFEMG阳性率和AchRAb（＋）、MuSKAb（＋）和双抗体均阴性表达，差异无统计学意义（P〉0．05）。EOMG组患者甲状腺功能异常率和外科胸腺手术比例均高于LOMG组患者（P〈0．05）。激素、他克莫司和血浆置换EOMG组患者使用频率高于LOMG组患者（P〈0．05）。结论EOMG和LOMG组患者在临床分型、甲状腺功能、纹状抗体表达情况、外科手术比例及免疫用药方面存在差异。Objective To explore the clinical differences between patients with early-onset myasthenia gravis （EOMG）and those with late-onset myasthenia gravis（LOMG）. Methods This was a retrospective study enrolling 157 MG patients. Based on the age of onset, patients were divided into the EOMG group（n= 85）and the LOMG group （n = 72 ）. The groups were compared on clinical characteristics, including clinical manifestations, MG classification, electrophysiological findings on repetitive nerve stimulation（RNS）, single fiber electromyography（SFEMG）,levels of antibody against aeetylcholine receptors（Ach-R Ab）, antibody to muscle-specific kinase（MuSK Ab） ,titin antibody（Titin Ab）, ryanodine receptor antibody（RyR Ab） ,thyroid function,thymectomy, thymus pathology and responses to treatment. Results The mean ages of onset were markedly different [（ 40. 9± 9.7 ） years vs. （62.0 ± 12.2 ） years, P〈 0.05 ] between the EOMG and LOMG groups. The LOMG group was associated with a significantly higher rate of the ocular form（50. 0%, n=36 w 32.9% ,n=28, P〈0.05）,a lower rate of the general form（50.0% ,n=36 vs.67.1% ,n=57, P〈 0. 05）,and an increased risk of bulbar involvement（41.7% n=30 vs. 23.5% ,n=20, P〈0. 05）than those in the EOMG group. There was no significant difference in positive rates of RNAS and SFEMG, and levels of AChR Ab, MuSK Ab and double serum negative （DSN）MG between the groups （P〉 0. 05）. Moreover, patients in the EOMG group were more likely to have abnormal thyroid function and higher percentages of receiving steroids, tacrolimus, plasma exchange therapy, and thymectomy （ P 〈 0. 05）. Conclusions The clinical profiles of LOMG are different from those of EOMG in clinical manifestations, thyroid function, thymectomy frequency,striational antibody levels and disease-modifying drug options.

关 键 词：重症肌无力 受体 胆碱能 

分 类 号：R746.1[医药卫生—神经病学与精神病学]