Bonardi Claudia Maria, Furlanis Giulia Melinda, Toldo Irene, Guarrera Brando, Luisi Concetta, Pettenazzo Andrea, Nosadini Margherita, Boniver Clementina, Sartori Stefano, Landi Andrea
Paediatric Intensive Care Unit, Department of Woman's and Child's Health, Padua University Hospital, Padua, Italy.
Paediatric and Functional Neurosurgery Unit, Padua University Hospital, Padua, Italy.
Brain Dev. 2023 May;45(5):293-299. doi: 10.1016/j.braindev.2023.01.004. Epub 2023 Jan 30.
Febrile infection-related epilepsy syndrome (FIRES) is a rare and catastrophic clinical syndrome occurring in previously healthy patients. Aetiology is still unknown and outcome usually poor. We describe a case of myoclonic prolonged super refractory status epilepticus (P-SRSE) in FIRES in a patient admitted to the paediatric intensive care unit of Padova, Italy.
A previously healthy 14-year-old girl with onset of myoclonic status epilepticus after a mild febrile illness was admitted to our hospital with a diagnosis of FIRES. Extensive diagnostic work-up was inconclusive. Status epilepticus and electroclinical seizures recurred every time weaning from anaesthetic agents was attempted. Eventually, a vagal nerve stimulator (VNS) was implanted and cannabidiol (CBD) administered, 43 days and 70 days after P-SRSE onset, respectively. Two days after CBD introduction, status epilepticus weaned and the girl rapidly regained complete consciousness showing a brilliant and unexpected recovery. At last follow-up, 12 months later, she is 8-months seizure free on multiple antiseizure medications, has only mild neuropsychological impairment with no neurological and intellective deficit.
To our knowledge, this represents a unique case with an extremely favourable evolution with a possible effect of the association of VNS and CBD to traditional antiseizure medications.
发热感染相关癫痫综合征（FIRES）是一种发生在既往健康患者中的罕见且灾难性的临床综合征。病因仍不明，预后通常较差。我们描述了一名意大利帕多瓦儿科重症监护病房收治的FIRES患者出现肌阵挛性长时间超难治性癫痫持续状态（P-SRSE）的病例。
一名既往健康的14岁女孩，在轻度发热性疾病后出现肌阵挛性癫痫持续状态，以FIRES诊断入院。广泛的诊断性检查未得出结论。每次尝试停用麻醉剂时癫痫持续状态和电临床发作都会复发。最终，分别在P-SRSE发作后43天和70天植入了迷走神经刺激器（VNS）并给予了大麻二酚（CBD）。在给予CBD两天后，癫痫持续状态缓解，女孩迅速恢复了完全意识，显示出惊人且意想不到的康复。在最后一次随访时，即12个月后，她在多种抗癫痫药物治疗下已无癫痫发作8个月，仅有轻度神经心理损害，无神经和智力缺陷。
据我们所知，这是一个具有极其良好转归的独特病例，VNS和CBD与传统抗癫痫药物联合使用可能起到了作用。
