AChR抗体阳性重症肌无力合并舌肌萎缩2例报道并文献分析

目的 探讨重症肌无力(MG)患者合并舌肌萎缩的发病特点及机制。方法 分析武汉同济医院神经内科住院部2例乙酰胆碱受体(AChR)抗体阳性MG患者合并舌肌萎缩的临床资料,检索MG合并舌肌萎缩的文献报道,总结此类疾病的特点、萎缩机制、治疗方法及预后。结果 在临床上MG合并舌肌萎缩并不多见,大多数患者伴有血清肌肉特异性酪氨酸肌酶(MuSK)抗体阳性,伴AChR抗体阳性者较为罕见,肌肉萎缩的发病机制具体不详; 该类患者除发病时间较长外,通常提示更严重的病情、更差的治疗反应、更不良的预后。结论 MG舌肌萎缩临床少见,严重影响着患者的言语、咀嚼和吞咽等功能,除病程较长外,其肌肉萎缩机制暂不清楚,有待临床进一步探索,以改善此类患者预后和生活质量。

AChR-positive myasthenia gravis patients with tongue muscle atrophy(two case reports)and review

ObjectiveTo investigate the clinical characteristics and mechanism of myasthenia gravis(MG)patients with tongue muscle atrophy symptoms.Methods Through analyzing the clinical data of two acetylcholine receptor(AChR)antibody positive MG patients with tongue muscle atrophy in the Neurology Department of Wuhan Tongji Hospital and searching the literature of tongue muscle atrophy in MG patients, the characteristics of this kind of disease, the mechanism of atrophy, the treatment and prognosis of this disease were discussed.Results Tongue muscle atrophy in MG patients was not usual in clinical. Most of them are serum muscle-specific tyrosine kinase(MuSK)antibody positive, and AChR antibody positive patients were rare, however the pathogenesis of muscle atrophy was unknown.In addition to a longer duration of disease,these patients usually suggested a more severe condition, a worse treatment response and a poorer prognosis.Conclusion Tongue muscle atrophy in MG patients was rare with serious impact on patients' speech, chewing, deglutition, and other functions. In addition to the long course of disease, the muscle atrophy mechanisms were not clear. Further research was needed to improve the prognosis and the quality of life.