Gould R J, Steeg C N, Eastwood A B, Penn A S, Rowland L P, De Vivo D C
Neurology. 1985 Aug;35(8):1208-12. doi: 10.1212/wnl.35.8.1208.
An 11-year-old boy was evaluated for mild periodic muscular weakness exacerbated on separate occasions by disopyramide phosphate and procainamide. He and his mother both had bidirectional ventricular tachydysrhythmia (BVT), short stature, microcephaly, and clinodactyly. The mother, but not the child, had lingual myotonia. The two antiarrhythmic drugs worsened the muscular weakness without benefiting the cardiac dysrhythmia. Potassium loading produced skeletal muscle weakness and transient conversion of the BVT to normal sinus rhythm. Hypokalemia aggravated the BVT without causing weakness. Acetazolamide had no effect. The patient suffered a nonfatal cardiac arrest after several days of increased carbohydrate intake. Imipramine controlled the dysrhythmia without inducing weakness. Periodic paralysis should be considered as the diagnosis in children with BVT, a potentially fatal condition.
一名11岁男孩因轻度周期性肌无力接受评估，磷酸丙吡胺和普鲁卡因胺曾在不同时间加重其肌无力症状。他和他的母亲均患有双向性室性心动过速（BVT）、身材矮小、小头畸形和手指弯曲。母亲有舌肌强直，而孩子没有。这两种抗心律失常药物加重了肌无力，却对心律失常没有益处。补钾导致骨骼肌无力，并使BVT暂时转变为正常窦性心律。低钾血症加重了BVT，但未引起肌无力。乙酰唑胺无效。患者在碳水化合物摄入量增加数天后发生了一次非致命性心脏骤停。丙咪嗪控制了心律失常，且未诱发肌无力。对于患有BVT（一种潜在致命疾病）的儿童，应考虑周期性麻痹作为诊断。
