Abstract:
Autoimmune neuromuscular junction disorders are rare. However, myasthenia gravis is being increasingly recognised in people older than 50 years. In the past 5-10 years, epidemiological studies worldwide suggest an incidence of acetylcholine receptor antibody-positive myasthenia gravis of up to 29 cases per 1 million people per year. Muscle-specific tyrosine kinase antibody-positive myasthenia gravis and Lambert-Eaton myasthenic syndrome are about 20 times less common. Several diagnostic methods are available for autoimmune neuromuscular junction disorders, including serological antibody, electrophysiological, imaging, and pharmacological tests. The course of disease can be followed up with internationally accepted clinical scores or patient-reported outcome measures. For prognostic purposes, determining whether the disease is paraneoplastic is of great importance, as myasthenia gravis can be associated with thymoma and Lambert-Eaton myasthenic syndrome with small-cell lung cancer. However, despite well defined diagnostic parameters to classify patients into subgroups, objective biomarkers for use in the clinic or in clinical trials to predict the course of myasthenia gravis and Lambert-Eaton myasthenic syndrome are needed.
摘要:
自身免疫性神经肌肉接头疾病很少见。然而,重症肌无力在50岁以上的人群中得到越来越多的认可。在过去的5-10年里,全世界的流行病学研究表明,每年每100万人中,乙酰胆碱受体抗体阳性重症肌无力的发病率高达29例.肌肉特异性酪氨酸激酶抗体阳性的重症肌无力和Lambert-Eaton肌无力综合征约20倍不常见。有几种诊断方法可用于自身免疫性神经肌肉接头疾病,包括血清学抗体,电生理学,成像,和药理试验。病程可以通过国际公认的临床评分或患者报告的结果测量来随访。出于预后目的,确定该疾病是否为副肿瘤非常重要,重症肌无力可与胸腺瘤和Lambert-Eaton肌无力综合征伴小细胞肺癌相关。然而,尽管明确定义了将患者分为亚组的诊断参数,需要在临床或临床试验中使用客观的生物标志物来预测重症肌无力和Lambert-Eaton肌无力综合征的病程。