Gordon N
Brain Dev. 1994 Mar-Apr;16(2):153-5. doi: 10.1016/0387-7604(94)90054-x.
Loss of consciousness in childhood may be due to cardiovascular causes, and the Long Q-T syndromes can present with seizures. The Romano-Ward syndrome is of autosomal dominant inheritance, and the Jervell and Lange-Nielson syndrome, with associated deafness, of autosomal recessive inheritance. The diagnosis is often delayed, but a careful history can avoid this. The syndromes can appear to be due to an imbalance in the sympathetic nerve to the ventricular myocardium, and precipitating causes such as stress suggest a CNS influence on this. The electrocardiogram can confirm the prolonged Q-T interval, but this is not always present, at least without an exercise test. Treatment with beta-blockers is often successful. If a wrong diagnosis of epilepsy is made a chance may be missed of avoiding sudden death, quite apart from all the medical, and social consequences that can result from such a diagnosis.
儿童期意识丧失可能由心血管原因引起，长Q-T综合征可伴有癫痫发作。 Romano-Ward综合征为常染色体显性遗传，而伴有耳聋的Jervell和Lange-Nielson综合征为常染色体隐性遗传。诊断往往会延迟，但仔细询问病史可避免这种情况。这些综合征似乎是由于支配心室心肌的交感神经失衡所致，诸如压力等诱发因素提示中枢神经系统对此有影响。心电图可证实Q-T间期延长，但并非总是如此，至少在未进行运动试验时不会出现。使用β受体阻滞剂治疗往往有效。如果误诊为癫痫，除了这种诊断可能带来的所有医学和社会后果外，还可能错过避免猝死的机会。
