The Second Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Neurol Sci. 2023 Oct;44(10):3669-3673. doi: 10.1007/s10072-023-06865-y. Epub 2023 May 27.
Valproic acid (VPA) is a prevalent antiseizure medication (ASM) used to treat epilepsy. Valproate-related hyperammonemic encephalopathy (VHE) is a type of encephalopathy that can occur during neurocritical situations. In VHE, the electroencephalogram (EEG) displays diffuse slow waves or periodic waves, and there is no generalized suppression pattern.
We present a case of a 29-year-old female with a history of epilepsy who was admitted for convulsive status epilepticus (CSE), which was controlled by intravenous VPA, as well as oral VPA and phenytoin. The patient did not experience further convulsions but instead developed impaired consciousness. Continuous EEG monitoring revealed a generalized suppression pattern, and the patient was unresponsive. The patient's blood ammonia level was significantly elevated at 386.8 μmol/L, indicating VHE. Additionally, the patient's serum VPA level was 58.37 μg/ml (normal range: 50-100 μg/ml). After stopping VPA and phenytoin and transitioning to oxcarbazepine for anti-seizure and symptomatic treatment, the patient's EEG gradually returned to normal, and her consciousness was fully restored.
VHE can cause the EEG to display a generalized suppression pattern. It is crucial to recognize this specific situation and not to infer a poor prognosis based on this EEG pattern.
丙戊酸（VPA）是一种常用的抗癫痫药物（ASM），用于治疗癫痫。丙戊酸盐相关高氨血症性脑病（VHE）是一种在神经危重症情况下可能发生的脑病。在 VHE 中，脑电图（EEG）显示弥漫性慢波或周期性波，没有广泛抑制模式。
我们报告了一例 29 岁女性癫痫病史，因癫痫持续状态（CSE）入院，静脉注射 VPA 以及口服 VPA 和苯妥英钠控制了癫痫发作。患者没有再次抽搐，但出现意识障碍。连续 EEG 监测显示出广泛抑制模式，患者无反应。患者的血氨水平显著升高至 386.8μmol/L，提示 VHE。此外，患者的血清 VPA 水平为 58.37μg/ml（正常范围：50-100μg/ml）。停用 VPA 和苯妥英钠，并改用奥卡西平进行抗癫痫和对症治疗后，患者的 EEG 逐渐恢复正常，意识完全恢复。
VHE 可导致 EEG 显示出广泛抑制模式。识别这种特殊情况非常重要，不要根据这种 EEG 模式推断预后不良。
