Long-term epilepsy control, motor function, cognition, sleep and quality of life in children with West syndrome

•Preponderance of structural etiology, male gender, and a long treatment lag.•High rate of transition from infantile spasms to Lennox-Gastuat syndrome.•Structural etiology: Associated with poor sleep outcomes.•Age at onset of spasms (≤ 5 mo) and structural etiology predicted unfavourable long-term epilepsy outcomes.•Age at onset of spasms ≤ 5 mo: Associated with severe intellectual disability. To assess epilepsy, motor function, cognitive, sleep, and quality of life outcomes and their predictors in a follow-up cohort with West syndrome (WS) at ≥5 years of age. Cross-sectional evaluation in a follow-up cohort of WS (aged 5–14 years), between July 2018 and December 2019, was performed at a tertiary-care referral center in Northern India. 164 children were assessed in-person for epilepsy severity, functional status (gross motor and hand function), social quotient, behavioral comorbidities, sleep problems, and quality of life (QoL) using Early Childhood Epilepsy Severity Scale, Gross Motor Function Classification System, Manual Ability Classification System, Vineland Social Maturity Scale, Diagnostic and Statistical Manual of Mental disorders-5 criteria, Children’s Sleep Habits Questionnaire, and PedsQL-Epilepsy module respectively. Furthermore, 238 children with the inability to visit the hospital were assessed through telephonic interview along with retrospective case record review for epilepsy control, gross and fine motor measures. 402 children with WS (75 % boys) with regular follow-up were studied and the majority (80 %) had underlying structural etiology. The median age (interquartile range) of the cohort was 92 (78–107) months. Of these, 60 % had evolved to Lennox-Gastaut syndrome (LGS). The following long-term outcomes were observed: ongoing epilepsy (261/402), unfavorable motor status (130/402), moderate to profound intellectual disability (111/164), autistic spectrum disorder (42/164), attention-deficit hyperactivity disorder (18/164), poor sleep (135/164), and impaired QoL (115/164). Non-structural etiology (odds ratio [OR] = 3.8, 95 % confidence interval [CI]: 2.1−5.5, p=5 months) at the onset of epileptic spasms (OR = 2·9, 95 % CI: 1.5−5.0, p=