Dysautonomia Center, Department of Neurology, New York University School of Medicine, 530 First Avenue Suite 9Q, New York, NY, 10016, USA.
Auton Neurosci. 2011 Oct 28;164(1-2):87-8. doi: 10.1016/j.autneu.2011.06.005. Epub 2011 Jul 13.
Autoimmune autonomic ganglionopathy (AAG) and myasthenia gravis (MG) are both autoimmune channelopathies mediated by antibodies directed against nicotinic acetylcholine receptors. While both diseases target acetylcholine receptors, skeletal muscle and ganglionic receptor subtypes have key immunologic and genetic distinctions, and reports of patients with both AAG and MG are rare. We report a patient with antibody-confirmed AAG and elevated levels of ACh binding antibodies that did not meet clinical or electrodiagnostic criteria for MG. We presume that his skeletal muscle nAChR seropositivity was a false positive, perhaps due to the cross reactivity of the patient's ganglionic nAChR antibodies with skeletal nAChR subtypes.
自身免疫性自主神经节神经病 (AAG) 和重症肌无力 (MG) 都是由针对烟碱型乙酰胆碱受体的抗体介导的自身免疫性通道病。虽然这两种疾病都针对乙酰胆碱受体,但骨骼肌和神经节受体亚型具有关键的免疫和遗传差异,同时患有 AAG 和 MG 的患者报告非常罕见。我们报告了一例经抗体确认的 AAG 患者,其乙酰胆碱结合抗体水平升高,但不符合 MG 的临床或电诊断标准。我们推测他的骨骼肌 nAChR 血清阳性是假阳性,可能是由于患者的神经节 nAChR 抗体与骨骼肌 nAChR 亚型的交叉反应所致。