Department of Radiology, Neuroradiology Section, University of California, San Francisco, California 94143-1207, USA.
Epilepsia. 2010 Mar;51(3):473-7. doi: 10.1111/j.1528-1167.2009.02287.x. Epub 2009 Sep 22.
Voltage-gated potassium channels are widely expressed throughout the entire nervous system. These channels play a critical role in establishing the resting membrane potential and generation of neuronal action potentials. There is mounting evidence that autoantibodies reactive to neuronal cell surface antigens, such as voltage-gated potassium channels, play a pathogenic role in a wide spectrum of central and peripheral nervous system disorders. We report a case of new-onset drug-refractory seizure disorder associated with the presence of high levels of serum anti-voltage-gated potassium channel antibodies that responded only to immunotherapy. As demonstrated by this case report, anti-voltage-gated potassium channel antibody associated drug-refractory seizure disorder, although rare, should be considered in patients with unexplained adult-onset seizure activity. Once the diagnosis has been established the initiation of immunotherapy should be undertaken without delay.
电压门控钾通道广泛表达于整个神经系统。这些通道在建立静息膜电位和产生神经元动作电位方面发挥着关键作用。越来越多的证据表明，针对神经元细胞表面抗原（如电压门控钾通道）的自身抗体在中枢和外周神经系统疾病的广泛谱中发挥着致病作用。我们报告了一例新诊断的药物难治性癫痫发作疾病，与高水平的血清抗电压门控钾通道抗体有关，而这些抗体仅对免疫治疗有反应。正如本病例报告所表明的，尽管抗电压门控钾通道抗体相关的药物难治性癫痫发作疾病很少见，但对于原因不明的成年期癫痫发作患者，应考虑这种疾病。一旦确诊，应立即开始免疫治疗。
