Baraka A
Department of Anesthesiology, American University of Beirut, Lebanon.
Middle East J Anaesthesiol. 1993 Feb;12(1):9-35.
Myasthenia gravis is an autoimmune disease, resulting from the production of antibodies against the acetylcholine receptors of the endplate. These antibodies reduce the number of active receptors, brought about either by functional block of the receptors, by increased rate of receptor degradation, or by complement-mediated lysis. In myasthenic muscles, the miniature endplate potential amplitude is decreased, and a large proportion of the endplate potentials are subthreshold. Repetitive nerve stimulation results in a decremental response. The disease is frequently associated with morphological abnormalities of the thymus. In young patients, thymic hyperplasia is common while thymoma is more frequent in elderly patients. Medical treatment of myasthenia gravis aims at improving of neuromuscular transmission by anticholinesterases, suppressing the immune system by corticosteroids and immunosuppressents, or by decreasing the circulating antibodies by plasmapheresis. Adults with generalized myasthenia should have a transsternal thymectomy. A balanced technique of general anaesthesia which includes the use of muscle relaxants can be safely used, provided neuromuscular transmission is monitored. Myasthenic patients are sensitive to nondepolarizing relaxants but intermediate-acting nondepolarizing relaxants such as atracurium and vecuronium are eliminated rapidly, and can be titrated to achieve the required neuromuscular block that can be completely reversed at the end of surgery. Postoperatively, ventilatory support may be required in high-risk patients. Also, medical treatment may be maintained, tapered or discontinued depending on the outcome of surgery. Thymectomy benefits nearly 96% of patients, 46% develop complete remission and 50% are asymptomatic or improve on therapy.
重症肌无力是一种自身免疫性疾病,由针对终板乙酰胆碱受体的抗体产生所致。这些抗体通过受体功能阻断、受体降解速率增加或补体介导的溶解作用,减少了活性受体的数量。在重症肌无力患者的肌肉中,微小终板电位幅度降低,且大部分终板电位低于阈值。重复神经刺激会导致递减反应。该疾病常与胸腺的形态学异常相关。在年轻患者中,胸腺增生常见,而在老年患者中胸腺瘤更为常见。重症肌无力的医学治疗旨在通过抗胆碱酯酶改善神经肌肉传递,通过皮质类固醇和免疫抑制剂抑制免疫系统,或通过血浆置换减少循环抗体。患有全身型重症肌无力的成年人应进行经胸骨胸腺切除术。只要监测神经肌肉传递,就可以安全地使用包括使用肌肉松弛剂在内的平衡全身麻醉技术。重症肌无力患者对非去极化肌松药敏感,但阿曲库铵和维库溴铵等中效非去极化肌松药消除迅速,可以滴定以达到所需的神经肌肉阻滞,且在手术结束时可完全逆转。术后,高危患者可能需要通气支持。此外,根据手术结果,可能维持、逐渐减少或停止药物治疗。胸腺切除术使近96%的患者受益,46%的患者实现完全缓解,50%的患者无症状或病情在治疗后改善。