PDF(Pubmed)
Abstract：
Hyperammonemic encephalopathy is a neurological emergency that can lead to seizures and cerebral edema. Although early interventions have been suggested, no clear criteria have been established. Herein, we report a case of severe non-hepatic hyperammonemia resulting in refractory status epilepticus within a day. A 79-year-old woman presented with acute altered mental status. Initial evaluation revealed septic shock and hyperammonemia due to fecal bowel obstruction with congenital portosystemic shunt. The patient was unresponsive to medical treatment and developed refractory status epilepticus. After surgical drainage with colostomy and a decrease in ammonia level, the patient developed cerebral edema and did not recover from the coma. Severe hyperammonemia warrants early intervention, especially in critically ill patients, with treatment of the cause and augmented removal of ammonia with renal replacement therapy.
摘要：
高血氨脑病是一种神经系统急症，可导致癫痫发作和脑水肿。尽管已经提出了早期干预措施，没有明确的标准。在这里，我们报告一例严重的非肝性高氨血症,导致1天内出现难治性癫痫持续状态.一名79岁的妇女出现了严重的精神状态改变。初步评估显示，由于先天性门体分流导致的粪便肠梗阻引起的感染性休克和高氨血症。患者对药物治疗无反应，并出现难治性癫痫持续状态。结肠造口术和氨水平降低后，患者出现脑水肿，昏迷未恢复。严重的高氨血症需要早期干预，尤其是危重病人,通过肾脏替代疗法治疗原因和增加氨的去除。
