Pascuzzi R M, Kim Y I
Indiana University Medical Center, Indianapolis 46202.
Semin Neurol. 1990 Mar;10(1):35-41. doi: 10.1055/s-2008-1041251.
LES is an autoimmune disorder of the neuromuscular junction in which autoantibodies directed against voltage-dependent Ca2+ channels block nerve-evoked Ca2+ entry at the motor nerve terminal. The pathogenic IgG is likely to produce a similar inhibitory effect on the Ca2+ channel function in other cholinergic synapses of the autonomic nervous system. This pathophysiology is sufficient to account for the distinctive clinical, immunologic, and electrophysiologic manifestations in patients with LES. Etiology of this disease is uncertain but in view of its frequent association with small cell lung cancer, this specific type of neoplasm may be implicated in the initiation of autoimmune response. Recent studies indeed support the possibility that the antigenic stimulus in the neoplastic form of LES may arise from voltage-dependent Ca2+ channels found in the lung cancer cells.
兰伯特-伊顿肌无力综合征（LES）是一种神经肌肉接头的自身免疫性疾病，其中针对电压依赖性Ca2+通道的自身抗体在运动神经末梢阻断神经诱发的Ca2+内流。致病性IgG可能对自主神经系统其他胆碱能突触中的Ca2+通道功能产生类似的抑制作用。这种病理生理学足以解释LES患者独特的临床、免疫和电生理表现。该疾病的病因尚不确定，但鉴于其常与小细胞肺癌相关，这种特定类型的肿瘤可能与自身免疫反应的启动有关。最近的研究确实支持这样一种可能性，即LES肿瘤形式中的抗原刺激可能来自肺癌细胞中发现的电压依赖性Ca2+通道。
