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首页> 美国卫生研究院文献>SpringerPlus >Pronounced reversible hyperammonemic encephalopathy associated with combined valproate–topiramate therapy in a 7-year-old girl
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Pronounced reversible hyperammonemic encephalopathy associated with combined valproate–topiramate therapy in a 7-year-old girl

机译：与一名丙戊酸-托吡酯联合疗法联合治疗的7岁女孩明显可逆性高氨性脑病

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摘要

Valproate is one of the most frequently used anticonvulsive drugs in children and adults. Valproate is a generally well tolerated medication. However, encephalopathy with or without hyperammonemia is one of its rare adverse events. We present a 7-year-old girl who suffered from epilepsy with generalized tonic–clonic seizures and absence epilepsy. She was initially treated with topiramate. Methylprednisolone pulse therapy and long-term therapy with valproate were initiated due to an increase of seizure frequency. At day 5 of therapy, a further increase of seizure frequency was observed followed by lethargy and somnolence. Liver enzymes remained within normal range, but ammonia serum levels increased to a maximum of 544 mmol/l. Discontinuing valproate and starting potassium-benzoate and sodium-phenylbutyrate improved the clinical condition and ammonia serum levels. Haemodialysis was not required. Cranial magnetic resonance imaging ruled out brain edema. The patient was further on successfully treated with a combination of both, topiramate and levetiracetam. Seizures did not recur and development was normal until now (3 years later). To the best of our knowledge, we observed the highest ammonia serum levels ever reported in valproate-induced hyperammonemia with a complete remission of the subsequent encephalopathy. Topiramate might increase the risk of valproate-induced encephalopathy by carbonic anhydrase inhibition.
机译：丙戊酸盐是儿童和成人中最常用的抗惊厥药之一。丙戊酸盐是一种耐受性良好的药物。然而，伴或不伴高氨血症的脑病是其罕见的不良事件之一。我们介绍了一个患有癫痫病的7岁女孩，患有全身性强直-阵挛性癫痫发作和无癫痫病。她最初接受托吡酯治疗。由于癫痫发作频率增加，开始使用甲基强的松龙脉冲疗法和丙戊酸长期治疗。在治疗的第5天，观察到癫痫发作频率进一步增加，随后出现嗜睡和嗜睡。肝酶保持在正常范围内，但氨血清水平最高增加到544 mmol / l。停用丙戊酸盐并开始使用苯甲酸钾和苯丁酸钠可改善临床状况和氨氮水平。不需要血液透析。颅磁共振成像排除了脑水肿。该患者进一步接受托吡酯和左乙拉西坦的联合治疗。直到现在（3年后）癫痫发作都没有复发并且发展正常。据我们所知，我们观察到丙戊酸盐诱发的高氨血症报告的氨血清水平最高，随后的脑病得以完全缓解。托吡酯可能通过抑制碳酸酐酶增加丙戊酸盐引起的脑病的风险。

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