Michaud M, Delrieu J, Astudillo L
Service de médecine interne, CHU de Purpan, Toulouse cedex , France.
Rev Med Interne. 2011 Dec;32(12):742-50. doi: 10.1016/j.revmed.2011.04.003. Epub 2011 Nov 12.
Autoimmune channelopathies are rare neuromuscular diseases that have been characterized clinically for several decades but for which the evidence of associated antibodies has only been recently demonstrated. Ion channels have an important role of activation, inhibition and regulation in neuromuscular transmission. Myasthenia gravis, generally associated with the presence of anti-acetylcholine receptor antibody, is the best-known channelopathy. Other anti-channel antibodies, including voltage-dependent, are associated with several neurological diseases, as illustrated by anti-voltage-gated calcium channels found in Lambert-Eaton myasthenic syndrome and paraneoplastic cerebellar ataxia, and anti-voltage-gated potassium channels found in neuromyotonia, Morvan's syndrome and limbic encephalitis. The treatment of autoimmune channelopathies is logically based on corticosteroids, immunosuppressant drugs, intravenous immunoglobulins and plasmapheresis.
自身免疫性离子通道病是罕见的神经肌肉疾病，临床上已被认识数十年，但相关抗体的证据直到最近才得到证实。离子通道在神经肌肉传递中具有激活、抑制和调节的重要作用。重症肌无力通常与抗乙酰胆碱受体抗体的存在有关，是最著名的离子通道病。其他抗通道抗体，包括电压依赖性抗体，与多种神经系统疾病有关，如在兰伯特-伊顿肌无力综合征和副肿瘤性小脑共济失调中发现的抗电压门控钙通道，以及在神经性肌强直、莫旺综合征和边缘性脑炎中发现的抗电压门控钾通道。自身免疫性离子通道病的治疗理论上基于皮质类固醇、免疫抑制药物、静脉注射免疫球蛋白和血浆置换。
