Pénisson-Besnier I
Département de Neurologie, Centre de Référence des Maladies Neuromusculaires, CHU d'Angers, 4 Rue Larrey, 49933 Angers Cedex 09, France.
Rev Neurol (Paris). 2010 Apr;166(4):400-5. doi: 10.1016/j.neurol.2009.09.006.
Myasthenia gravis is the most common neuromuscular junction disorder and the best understood autoimmune disease of the nervous system. The autoimmune attack leads to decreased concentrations of the AChR and results in fatigability of skeletal muscles increasing with activity and improving with rest. The treatment of myasthenia has improved dramatically over the last few decades, with an increasing number of immunotherapies used in management although not all of them have been formally tested in double-blind, prospective trials. The principles of treatment consist in optimizing neuromuscular junction function by use of cholinesterase inhibitors, inducing an immunologic remission and then maintaining that remission by long-term immunotherapies. Prednisone and/or azathioprine are the most effective. Short-term immunotherapies, i.e. intravenous immunoglobulin or plasmapheresis, are indicated for disease exacerbation. For patients with nonthymomatous autoimmune myasthenia, the effectiveness of thymectomy remains uncertain. The overall objective of therapy is to enable patients to lead a normal life as rapidly as possible, while limiting side effects and costs if possible. Treatment should be individualized. The aggressiveness of therapy should be balanced against a number of factors including distribution of muscle involvement, rate of progression, degree of functional impairment, lifestyle choice, and coexisting disease. In all cases, adequate education, for the patient and the physician, is most helpful in facilitating management of this chronic disease.
重症肌无力是最常见的神经肌肉接头疾病,也是神经系统中了解最为透彻的自身免疫性疾病。自身免疫攻击导致乙酰胆碱受体浓度降低,进而导致骨骼肌疲劳,活动时加重,休息后改善。在过去几十年里,重症肌无力的治疗有了显著改善,越来越多的免疫疗法用于治疗,尽管并非所有疗法都经过了双盲前瞻性试验的正式检验。治疗原则包括使用胆碱酯酶抑制剂优化神经肌肉接头功能,诱导免疫缓解,然后通过长期免疫疗法维持缓解。泼尼松和/或硫唑嘌呤最为有效。短期免疫疗法,即静脉注射免疫球蛋白或血浆置换,适用于疾病加重期。对于非胸腺瘤性自身免疫性重症肌无力患者,胸腺切除术的疗效仍不确定。治疗的总体目标是使患者尽快过上正常生活,同时尽可能限制副作用和费用。治疗应个体化。治疗的积极程度应与多种因素相平衡,包括肌肉受累分布、进展速度、功能损害程度、生活方式选择和并存疾病。在所有情况下,对患者和医生进行充分教育,对促进这种慢性病的管理最有帮助。