Department of Neurosurgery, Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, India.
Department of Neurosurgery, Aster MIMS Hospital, Kerala, India.
Neurosurg Rev. 2024 Nov 6;47(1):836. doi: 10.1007/s10143-024-03054-z.
Sodium valproate is used for the management of seizures, status epilepticus, chronic pain syndrome, bipolar, and other affective disorders. Even with an acceptable safety profile, severe idiosyncratic reactions can occur with valproate use. A rare, serious, and life-threatening side effect of valproate is valproate-induced hyperammonemic encephalopathy (VHE). We intend to analyze the clinical presentation, diagnosis, treatment options, and outcome of VHE in neurosurgical patients and review the pertinent literature on this rare sequelae. We retrospectively reviewed patients who developed VHE following valproate use, either for the treatment of epilepsy or for seizure prophylaxis in our centre. We analyzed the demographic details, clinical presentation, diagnosis, management, and outcomes. A total of four patients with a mean age of 26.3 ± 5.1 (range 19 - 32 years). Valproate was prescribed for primary seizure prophylaxis in 2 patients (50%). The commonest etiology for valproate prescription was for brain tumors (3, 75%) followed by drug-refractory epilepsy (DRE) (1, 25%). None of the patients were documented to have urea cycle disorder. The mean daily prescribed dosage of valproate was 1250 ± 559 mg and the mean duration of administration was 13 ± 13.3 months (range 4 months - 36 months). The mean serum NH3 level was 136,5 ± 44.2 µmol/L (range 107 - 212.8) and all patients (4, 100%) had hyperammonemia with a mortality rate of 50% (2 patients). The hyperammonemia was treated by stopping the valproate use (4, 100%) and dialysis (2, 50%). Normalization of ammonia levels led to clinical improvement in 50% (2 patients). Neurological deterioration in the postoperative period is a diagnostic challenge. VHE is a rare and life-threatening sequelae of Valproate-associated Hyperammonemia (VAH) in neurosurgical patients. A high index of suspicion is required due to its ambiguous presentation. Early diagnosis and prompt treatment can change the course of this life-threatening sequelae.
丙戊酸钠用于治疗癫痫发作、癫痫持续状态、慢性疼痛综合征、双相情感障碍和其他情感障碍。即使具有可接受的安全性特征，丙戊酸盐的使用也会发生严重的特发性反应。丙戊酸盐引起的高氨血症性脑病（VHE）是丙戊酸盐罕见的严重且危及生命的副作用。我们旨在分析神经外科患者 VHE 的临床表现、诊断、治疗选择和结果，并回顾该罕见后遗症的相关文献。我们回顾性分析了在我们中心因癫痫发作或癫痫发作预防而使用丙戊酸钠后出现 VHE 的患者。我们分析了人口统计学细节、临床表现、诊断、治疗和结果。共有 4 名患者，平均年龄为 26.3±5.1 岁（范围 19-32 岁）。2 名患者（50%）开丙戊酸钠用于原发性癫痫预防。开丙戊酸钠的最常见病因是脑肿瘤（3 例，75%），其次是药物难治性癫痫（DRE）（1 例，25%）。无患者有尿素循环障碍的记录。丙戊酸钠的平均日剂量为 1250±559mg，平均给药时间为 13±13.3 个月（范围 4 个月-36 个月）。平均血清 NH3 水平为 136.5±44.2µmol/L（范围 107-212.8），所有患者（4 例，100%）均有高血氨症，死亡率为 50%（2 例）。通过停用丙戊酸钠（4 例，100%）和透析（2 例，50%）来治疗高氨血症。50%（2 例）患者的氨水平正常化导致临床改善。术后神经功能恶化是一个诊断挑战。VHE 是神经外科患者丙戊酸盐相关高氨血症（VAH）的罕见且危及生命的后遗症。由于其表现不明确，需要高度怀疑。早期诊断和及时治疗可以改变这种危及生命的后遗症的病程。
