Therapies of last resort in paediatric super-refractory status epilepticus: a case report on combined selective amygdalohippocampectomy, vagus nerve stimulation, and anterior corpus callosotomy

儿童难治性癫痫持续状态的最后治疗手段:选择性杏仁核海马切除术、迷走神经刺激术和前部胼胝体切开术联合治疗的病例报告

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Abstract

Super-refractory status epilepticus (SRSE) is a rare and life-threatening neurological emergency associated with substantial morbidity and mortality. Defined as persistent or recurrent seizures lasting beyond 24 h despite anaesthetic and antiseizure pharmacotherapy, SRSE poses significant therapeutic challenges. Management strategies aim to suppress seizures, provide neuroprotection, and mitigate excitotoxic injury. While emergency epilepsy surgery is traditionally reserved as a last resort due to limited supporting evidence, it has demonstrated efficacy in selected cases. We report the case of an 8-year-old girl with cerebral palsy, drug-resistant epilepsy, cortical visual impairment, and bilateral hearing loss, who developed SRSE unresponsive to maximal antiepileptic and anaesthetic therapy. Following multidisciplinary evaluation, a sequential multimodal neurosurgical approach was implemented, comprising left selective amygdalohippocampectomy (SAH), vagus nerve stimulation (VNS), and anterior corpus callosotomy. SAH targeted the epileptogenic mesial temporal structures while preserving surrounding neocortex and neurovascular anatomy, resulting in immediate seizure cessation. Subsequent VNS implantation and callosotomy were performed following recurrent episodes, achieving sustained reduction in seizure frequency and severity, with Engel class II outcome. This case underscores the potential role of integrating resective, neuromodulatory, and disconnection procedures in paediatric SRSE when conventional treatments fail. Early consideration of sequential surgical strategies may enhance seizure control and improve quality of life in this high-risk patient group.

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