摘要
目的探讨重症肌无力危象患者的临床特点和预后。方法回顾74例第一次发生肌无力危象的重症肌无力患者的一般资料,分析Osserman分型、危象发生时间、危象前特点、危象持续时间,评价重症肌无力危象治疗效果及预后。结果伴胸腺瘤的重症肌无力危象患者45例,占60.81%,非胸腺瘤患者29例,占39.19%。60.81%(45/74)的肌无力危象发生在重症肌无力起病的1年之内,胸腺切除术后6个月内发生危象的比例为76.00%(38/50)。呼吸费力和吞咽费力(24/74)是出现危象前最显著的特征,其次为胸腺手术后(11/74)、感染(9/74)、激素相关(7/74)。所有危象患者中Osserman分型ⅡB型所占比例最高,为45.95%(34/74)。重症肌无力危象患者的插管时间为15(7,30)d,44.60%(33/74)的危象患者需要丙种免疫球蛋白和(或)血浆置换联合甲泼尼龙冲击治疗。肌无力危象最常见的并发症为肺部感染(32/74,43.24%),反复气管插管最严重的并发症为支气管扩张伴出血、气胸。随访2~10年,17例患者出现再次或多次危象,死亡率为9.46%(7/74)。结论伴胸腺瘤的重症肌无力患者较不伴胸腺瘤患者出现肌无力危象的比例更高。重症肌无力危象治疗困难,需要多种免疫抑制剂联合治疗。胸腺切除后的半年内,仍然是肌无力危象发生的高峰。肌无力危象并发症、反复危象、胸腺瘤转移是患者死亡的主要原因。
Objectives To explore the clinical features and prognosis of patients with myasthenic crisis (MC). Methods The general data of 74 first-time MC patients with myasthenia gravis (MG) were reviewed for Osserman classification, time of crisis, pre-crisis characteristics, duration of the crisis, and effectiveness and prognosis evaluation of the MC treatment. Results MC patients with thymoma accounted for 60.81% of all participants, and non-thymoma patients accounted for 39.19%. 60.81%(45/74) of MC patients' first MC occurred within 1 year since MG onset, and 76.00%(38/50) of all MC cases occurred within 6 months after thymectomy accounted. Dyspnea and dysphagia (24/74) were the most prominent characteristics before the onset of MC, followed by thymic surgery (11/74), infection (9/74), and hormone-related factors (7/74). Among all MCs, 45.95% are Osserman type IIB, accounting for the highest percentage across all types. The intubation time of MC patients is 15(7,30)days. 44.60%(33/74) of MC patients required gamma globulin and/or plasma exchange combined with methylprednisolone. The most common complication of MC was the pulmonary infection (32/74), accounting for 43.24% of all cases. The most serious complications of repeated tracheal intubation were bronchiectasis with hemorrhage and pneumothorax. After 2-10 years of follow-up, 17 patients had multiple crises after the first MC, with a mortality rate of 9.46%(7/74). Conclusions MG patients with thymoma have a higher likelihood of MC than those without thymoma. Treatment of MCis difficult and requires multiple immunosuppressive combination therapy. MC is most likely to occur until six months after thymectomy. MC complications, repeated crises, and thymoma metastasis are the leading causes of death in patients.
作者
黄玲
王磊
徐芳
张琛
殷春立
尹世敏
Huang Ling;Wang Lei;Xu Fang;Zhang Chen;Yin Chunli;Yin Shimin(PLA Stroke Care and Reseach Center, Department of Neurology, PLA Rocket Characteristic Medical Center,Beijing 100088, China)
出处
《神经疾病与精神卫生》
2019年第5期477-481,共5页
Journal of Neuroscience and Mental Health
