Department of Pediatrics, Medical College and Hospital, Kolkata, West Bengal, India.
Pediatr Neurol. 2011 Sep;45(3):203-5. doi: 10.1016/j.pediatrneurol.2011.04.011.
A 3-year-old boy receiving valproate for 1.5 months presented with sudden-onset unprovoked seizures and unconsciousness. Hypoketotic hypoglycemia, hyperammonemia, and deranged liver function were detected. Elevated medium-chain urinary acylglycines and plasma acylcarnitine were detected. His serum valproate level was elevated. Valproate toxicity had been precipitated in presence of medium-chain acyl-CoA dehydrogenase deficiency. Cranial magnetic resonance imaging brain indicated unilateral basal ganglia ischemia instead of the bilateral changes expected in metabolic disease.
一名 3 岁男孩因接受丙戊酸钠治疗 1.5 个月而突发无诱因癫痫发作和意识丧失。检测到低酮性低血糖、高血氨和肝功能异常。检测到中等链尿酰基甘油脂和血浆酰基肉碱升高。血清丙戊酸钠水平升高。在存在中链酰基辅酶 A 脱氢酶缺乏的情况下，丙戊酸毒性已被引发。头颅磁共振成像脑显示单侧基底节区缺血，而不是代谢性疾病中预期的双侧变化。
