AUTHOR=Arai Yuto , Okanishi Tohru , Kanai Sotaro , Nakamura Yuko , Sunada Hiroshi , Murakami Hinata , Yamauchi Kazuki , Noma Hisashi , Maegaki Yoshihiro 

TITLE=Prediction model for long-term seizure and developmental outcomes among children with infantile epileptic spasms syndrome

JOURNAL=Frontiers in Neurology

VOLUME=Volume 14 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2023.1195252

DOI=10.3389/fneur.2023.1195252

ISSN=1664-2295

ABSTRACT=Introduction: Children with infantile epileptic spasm syndrome (IESS) are likely to experience poor outcomes. Researchers have investigated the factors related to its long-term prognosis; however, none of them developed a predictive model. Objective: To clarify the factors that influence the long-term prognosis of seizures and their development and to create a prediction model. Materials and methods: We conducted a retrospective cohort study enrolling participants diagnosed with IESS at the Tottori University Hospital. We examined the seizure and developmental status at 3 and 7 years after the IESS onset and divided the participants into favorable and poor outcome groups. Subsequently, we analyzed the factors associated with the poor outcome group and developed a prediction model at 3 years by setting cut-off values using the rating operation characteristic curve. Results: Data were obtained from 44 patients with IESS (19 women and 25 men). Three years after epileptic spasm (ES) onset, seizure and development were the poor outcomes in 15 (34.9%) and 27 (61.4%) patients, respectively. The persistence of ES or tonic seizures (TS) after 90 days of onset, moderate or severe magnetic resonance imaging abnormalities, and developmental delay before IESS onset were significantly associated with poor outcomes. Seven years after the onset of ES, seizures and development were the poor outcomes in 9 (45.0%) and 13 (72.2%) patients, respectively. No factor was significantly associated with poor seizure outcomes, and only developmental delay before IESS onset was significantly associated with poor developmental outcomes. Our prediction model demonstrated 86.7% sensitivity and 64.3% specificity for predicting poor seizure outcomes and 88.9% sensitivity and 100% specificity for predicting poor developmental outcomes. Conclusions: Our prediction model may be useful for predicting long-term prognosis of seizures and their development after 3 years. Understanding the long-term prognosis during initial treatment may facilitate appropriate treatment selection.