雷诺丁受体抗体阳性的重症肌无力临床特点

Clinical features of myasthenia gravis with positive ryanodine receptor antibody

雷诺丁受体(ryanodine receptor,RyR)是存在于内质网/肌质网中的一种重要钙离子通道,在骨骼肌兴奋收缩偶联机制中起重要作用。RyR抗体阳性的重症肌无力(myasthenia gravis,MG)患者常合并胸腺瘤,对常规治疗不敏感,会导致延误临床早期识别及治疗。血清RyR抗体水平与患者临床症状的严重程度显著相关。该文就4例RyR抗体阳性MG患者的临床特点及治疗过程进行讨论并文献复习,旨在提高对RyR抗体阳性MG的认识及诊疗水平。

Ryanodine receptor(RyR)is an important calcium channel in the endoplasmic reticulum/sarcoplasmic reticulum and plays an important role in the excitation⁃contraction coupling mechanism of skeletal muscle.Patients with myasthenia gravis(MG)and positive RyR antibody often have thymoma and are not sensitive to conventional treatment,which may delay early identification and treatment in clinical practice.The serum level of RyR antibody is significantly associated with the severity of clinical symptoms.This article discusses the clinical features and treatment process of four MG patients with positive RyR antibody,so as to improve the awareness and diagnosis and treatment levels of this disease.