Dhamija Radhika, Gavrilova Ralitza H, Wirrell Elaine C
Department of Pediatric Neurology, Mayo Clinic, Rochester, MN 55905, USA.
J Child Neurol. 2011 Oct;26(10):1319-21. doi: 10.1177/0883073811402204. Epub 2011 Mar 31.
The use of valproate sodium as an antiepileptic is not advised in children with an undiagnosed metabolic condition because of the increased risk of hepatotoxicity and encephalopathy. Here the authors describe a 2.5-year-old girl with a history of developmental delay, failure to thrive, and a seizure disorder whose seizures worsened after the introduction of valproate sodium. This led to a search for an underlying metabolic disorder, and after extensive investigations, a diagnosis of nonketotic hyperglycinemia was made. In this report, the authors discuss the metabolic conditions that can be worsened by valproate sodium. Valproate sodium interferes with the glycine cleavage enzyme synthesis in the mitochondria, hence increasing glycine levels. The increased glycine levels are responsible for worsening of the underlying metabolic condition and increased seizure frequency.
由于存在肝毒性和脑病风险增加的情况，不建议在患有未确诊代谢疾病的儿童中使用丙戊酸钠作为抗癫痫药物。本文作者描述了一名2.5岁的女孩，有发育迟缓、生长发育不良和癫痫发作病史，在使用丙戊酸钠后癫痫发作恶化。这促使对潜在的代谢紊乱进行排查，经过广泛检查，诊断为非酮症高甘氨酸血症。在本报告中，作者讨论了可因丙戊酸钠而恶化的代谢状况。丙戊酸钠干扰线粒体中甘氨酸裂解酶的合成，从而增加甘氨酸水平。甘氨酸水平升高导致潜在代谢状况恶化和癫痫发作频率增加。
