IRCCS Istituto delle Scienze Neurologiche di Bologna, U.O.C. Neuropsichiatria dell'età pediatrica, Member of the ERN EpiCare, Bologna, , Italy; Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum - University of Bologna, Bologna, Italy.
Pediatric Hematology and Oncology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
Seizure. 2024 Oct;121:85-90. doi: 10.1016/j.seizure.2024.08.001. Epub 2024 Aug 5.
Acute central nervous system (CNS) complications are common and well described among pediatric patients undergoing haematopoietic cell transplantation (HCT). However, their long-term outcomes are not known. The aim of this study is to describe the incidence, characteristics, and risk factors of long-term epilepsy in pediatric patients with acute CNS complications of HCT.
This retrospective study included pediatric patients who developed acute CNS complications from autologous or allogeneic HCT between 2000 and 2022. Clinical, therapeutic and prognostic data including long-term outcomes were analyzed. A diagnosis of epilepsy was provided if unprovoked seizures occurred during follow-up.
Ninety-four patients (63 males, 31 females, median age 10 years, range 1-21 years) were included. The most common acute CNS complications were posterior reversible encephalopathy syndrome (n = 43, 46 %) and infections (n = 15, 16 %). Sixty-five patients (69 %) had acute symptomatic seizures, with 14 (16 %) having one or more episodes of status epilepticus (SE). Nine patients (9.6 %) were diagnosed with long-term focal epilepsy during the follow-up (5-year cumulative incidence from the acute complication, 13.3 %). Acute symptomatic SE during neurological complications of HCT was associated with an increased risk of long-term epilepsy (OR=14, 95 % CI 2.87-68.97).
A higher occurrence of epilepsy has been observed in our cohort compared to the general population. Acute symptomatic SE during HCT was associated with a higher risk of long-term epilepsy. Pediatric patients with CNS complications during HCT could benefit from specific neurological follow-up. Further studies are needed to characterize mechanisms of epileptogenesis in pediatric patients undergoing HCT.
急性中枢神经系统（CNS）并发症在接受造血细胞移植（HCT）的儿科患者中很常见且已有详细描述。然而，其长期预后尚不清楚。本研究旨在描述 HCT 后发生急性 CNS 并发症的儿科患者中，长期癫痫的发生率、特征和危险因素。
本回顾性研究纳入了 2000 年至 2022 年间发生自体或异基因 HCT 后出现急性 CNS 并发症的儿科患者。分析了临床、治疗和预后数据，包括长期结局。如果在随访期间出现无诱因癫痫发作，则诊断为癫痫。
94 例患者（63 例男性，31 例女性，中位年龄 10 岁，范围 1-21 岁）纳入研究。最常见的急性 CNS 并发症为后部可逆性脑病综合征（n = 43，46%）和感染（n = 15，16%）。65 例患者（69%）出现急性症状性癫痫发作，其中 14 例（16%）有一次或多次癫痫持续状态（SE）发作。9 例患者（9.6%）在随访期间被诊断为长期局灶性癫痫（从急性并发症开始的 5 年累积发生率为 13.3%）。HCT 神经并发症期间出现急性症状性 SE 与长期癫痫风险增加相关（OR=14，95%CI 2.87-68.97）。
与一般人群相比，我们的队列中观察到癫痫的发生率更高。HCT 期间出现急性症状性 SE 与长期癫痫风险增加相关。接受 HCT 的 CNS 并发症患儿可能受益于特定的神经随访。需要进一步研究以明确接受 HCT 的儿科患者癫痫发生的机制。
