Fleury Marie-Céline, Tranchant Christine
Service de neurologie, Hôpitaux universitaires, 67091 Strasbourg Cedex, France.
Rev Prat. 2008 Dec 31;58(20):2217-24.
Myasthenia gravis is a rare, auto-immune neuromuscular junction disorder. Prevalence rates is about 50/1,000000. The disease results from circulating auto-antibody attacks against post-synaptic targets (acetylcholine receptor [AChR] in 80% cases) on the endplate region of the postsynaptic membrane. The diagnosis is supported clinically by transient weakness, increased by activity that can affect eye movements, swallowing, speech, upper and lower limbs, and trunk. There are generalized or focalized forms (as ocular myasthenia). The course is variable and evolved either with attacks or more chronically. Helpful tests for diagnosis are serologic antibodies detection against AChR, decrement of muscle action potential after repetitive nerve stimulations, identification of thymus gland abnormality (frequently associated with myasthenia) by chest computed tomography. Myasthenia gravis treatment is based on oral form of cholinesterase inhibitors, corticosteroids and other immunosuppressive drugs in severe forms. During myasthenia crisis, intraveinous immune globulines or plasma exchanges can be used. Thymectomy is proposed in case of thymus abnormality.
重症肌无力是一种罕见的自身免疫性神经肌肉接头疾病。患病率约为50/1000000。该病是由循环自身抗体攻击突触后膜终板区域的突触后靶点(80%的病例中为乙酰胆碱受体[AChR])所致。临床上,短暂性肌无力可支持诊断,活动后肌无力会加重,可累及眼球运动、吞咽、言语、上肢和下肢以及躯干。有全身型或局限型(如眼肌型重症肌无力)。病程多变,可呈发作性或慢性进展。有助于诊断的检查包括检测抗AChR血清抗体、重复神经刺激后肌肉动作电位递减、通过胸部计算机断层扫描识别胸腺异常(常与重症肌无力相关)。重症肌无力的治疗基于口服胆碱酯酶抑制剂、皮质类固醇以及严重病例使用的其他免疫抑制药物。在重症肌无力危象期间,可使用静脉注射免疫球蛋白或进行血浆置换。若存在胸腺异常,则建议行胸腺切除术。