仅表现声带麻痹的MuSK抗体阳性重症肌无力一例并文献复习  

MuSK myasthenia gravis presenting with only bilateral vocal cord abduction paresis:a case report and literature review

作　　者：杨梦婷 姜梦迪 赵凌云 殷剑[1] 侯世芳[1] 张华[1] YANG Mengting;JIANG Mengdi;ZHAO Lingyun;YIN Jian;HOU Shifang;ZHANG Hua(不详;Department of Neurology,Beijing Hospital,National Center of Gerontology,Institute of Geriatric Medicine,China Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]北京医院神经内科、国家老年医学中心、中国医学科学院老年医学研究院,100730 [2]山西省运城同德医院内分泌科,044000

出　　处：《中国神经免疫学和神经病学杂志》2023年第3期205-210,共6页Chinese Journal of Neuroimmunology and Neurology

基　　金：北京医院院级科研课题(BJ-2009-021)。

摘　　要：目的报道1例表现为双侧声带麻痹的肌肉特异性酪氨酸激酶(muscle skeletal receptor tyrosine kinase,MuSK)抗体阳性重症肌无力(myasthenia gravis,MG)患者的临床特点并进行文献复习。方法总结我院收治的1例主要累及双侧声带的MuSK抗体阳性MG患者的临床资料,同时检索中国知网、万方数据库、维普数据库、Pubmed、Cochrone图书馆、Embase数据库自建库以来至2022年6月公开发表的表现为声带麻痹的MuSK抗体阳性MG的个案报道,系统回顾伴声带麻痹的MuSK抗体阳性MG患者的临床特点、辅助检查、治疗及预后。结果共检索伴声带麻痹的MuSK抗体阳性MG患者5例,包括本文报道的病例共6例纳入研究。其中男性2例,女性4例。发病年龄22~55岁,平均(45.17±12.09)岁。出现声带麻痹的年龄24~59岁,平均(47.17±12.61)岁。喉镜检查结果均显示声带外展受限。重复神经电刺激检测显示,5例患者低频递减。4例患者行新斯的明试验,其中2例阴性。4例患者使用溴吡斯的明治疗效果均不佳。2例经丙种球蛋白治疗,其中1例效果不佳。5例经糖皮质激素治疗,其中4例改善。2例采用利妥昔单抗治疗,均有所改善。3例行气管插管或切开辅助治疗,均有所改善。结论MuSK抗体阳性MG患者发生双侧声带麻痹较为罕见,当患者出现伴疲劳现象的发音困难和喘鸣需要考虑抗MuSK阳性MG的可能。新斯的明试验阴性不能排除诊断,需要完善MG抗体、重复神经电刺激和单纤维肌电图检查进一步确诊。糖皮质激素、血浆置换和利妥昔单抗对抗MuSK阳性MG有一定疗效,多数患者需要辅助呼吸支持治疗。Objective To report a patient with muscle skeletal receptor tyrosine kinase(MuSK)antibody positive myasthenia gravis(MG)presenting with bilateral vocal cord paresis,and summarize the clinical characteristics of related literatures.Methods The clinical characteristics of a patient with MuSK MG presenting with bilateral vocal cord paresis was reported.We searched China National Knowledge Internet,Wanfang data,Weipu data,PubMed,Embase,and Cochrane Library for studies of patients with MuSK MG presenting with vocal cold paralysis published up to June 2022.We summarized the clinical characteristics,assistant examination,treatment and prognosis of reported cases and related literatures.Results We included 6 cases with MuSK MG presenting with vocal cord paralysis,5 of which identified from the literatures and 1 from our report,including 2 males and 4 females.Age of onset ranged from 22 to 55 years.The average onset age was(45.17±12.09)years.Age of vocal cord paralysis ranged from 24 to 59 years.The average age of vocal cord paralysis was(47.17±12.61)years.Laryngoscopy of all patients revealed vocal cold abduction paresis.Low-frequency repetitive nerve stimulation performed in all patients.Five patients showed a significant decrementing response.Neostigmine tests performed in 4 patients.Neostigmine tests were negative in 2 patients.All(4/4)patients treated with pyridostigmine showed no improvement.Two patients treated with intravenous immunoglobulin,1 of whom showed no improvement.Five cases treated with hormone,4 of whom improved.Two patients treated with rituximab showed improvement.Three patients treated with invasive respiratory support treatment also improved.Conclusions Bilateral vocal cord paralysis is a rare manifestation of MuSK MG.Presence of dysphonia and stridor that accompanied with fatigue should be suspected MG.Negative results of neostigmine test could not be used to exclude MG.Appropriate antibody testing,repetitive electrical stimulation and single fiber electromyography could help.The treatment of ste

关 键 词：声带麻痹 肌肉特异性酪氨酸激酶抗体 重症肌无力 蛋白酪氨酸激酶类 

分 类 号：R746.1[医药卫生—神经病学与精神病学]