高龄初发型重症肌无力患者的临床特点及预后分析

Clinical characteristics and prognosis of initial-onset myasthenia gravis at advanced age

目的探讨高龄初发重症肌无力(MG)患者的临床特点、治疗和预后。方法筛选2009年4月—2020年12月在郑州大学和河南省医药科学研究院行MG相关抗体检测的35例高龄初发型MG患者为研究对象。回顾性分析纳入患者的信息,并通过文献回顾方法观察高龄初发型MG患者的临床特征和预后。结果35例患者中,33例为乙酰胆碱受体抗体阳性,其中12例患者合并Titin抗体阳性,1例为肌肉特异性激酶抗体阳性,1例为双重血清MG阴性,未发现低密度脂蛋白相关受体4抗体阳性患者。登记注册的18例患者中,13例(72.22%)患者最初为眼部受累,其中6例(46.15%)发展为全身型MG,且平均诊断延迟时间为5.30个月,新斯的明试验阳性率为92.31%(12/13),重复性神经电刺激(RNS)阳性率为54.55%(6/11)。胸部CT检查结果显示,23.08%(3/13)的患者伴有胸腺瘤,危象发生率16.67%。随访期间8例患者死亡,病死率为44.44%(8/18)。结论高龄初发型MG患者主要是乙酰胆碱受体抗体为阳性的女性,主要累及眼肌,常因吞咽困难入院治疗,肌无力危象发生率和病死率较高,但当出现多个肌群受累时,使用小剂量泼尼松或联合他克莫司维持治疗可获得良好预后。

Objective To explore the clinical features,treatment and prognosis of initial-onset myasthenia gravis(MG)at an advanced age.Methods A total of 35 elderly patients with initial-onset MG from April 2009 to December 2020 who underwent MG-associated antibody detection in Zhengzhou University and Henan Institute of Medical and Pharmaceutical Sciences were selected as study subjects.The information of included patients was retrospectively analyzed,and the clinical characteristics and prognosis of elderly patients with elderly-onset MG were observed by literature review.Results Of 35 patients,33 were positive for acetylcholine receptor antibody,of which 12 patients were positive for Titin antibody,1 patient was positive for muscle-specific kinase antibody,and 1 patient was double negative for serum MG.No patient with positive LDL associated receptor 4 antibody was found.Of 18 patients registered,13(72.22%)were initially involved in ocular and 6(46.15%)developed systemic MG,with a mean diagnosis delay of 5.30 months.The positive rate was 92.31%(12/13)for neostigmine test and 54.55%(6/11)for repeated electrical nerve stimulation(RNS).Chest CT examination results showed that 23.08%(3/13)patients were associated with thymoma,and the incidence of crisis was 16.67%.During the follow-up,8 patients died,with a fatality rate of 44.44%(8/18).Conclusion Most of initial-onset MG patients at advanced age are women positive for acetylcholine receptor antibody.Most of the cases involve the ocular muscle,are often hospitalized due to dysphagia.The incidence of myasthenic crisis and the mortality rate are high.However,a good prognosis can be obtained using low-dose prednisone alone or tacrolimus in combination as maintenance therapy in patients with multiple affected muscle groups.