Abstracts

Rationale:
Considering the paucity of literature on long-term outcomes in infantile spasms (IS), the current study was designed to objectively assess epilepsy, motor function, cognition, sleep and quality of life outcomes and their predictors in a follow-up cohort with IS at  5 years of age.
Method:
Clinic records were reviewed to identify the children more than five years who were treated for IS. Children, who subsequently visited the hospital, were enrolled after informed consent and assessed face-to-face(n=164) for epilepsy severity, functional status (gross motor and fine motor), social quotient, behavioral comorbidities {Autism Spectrum Disorder (ASD) and Attention Deficit Hyperactivity Disorder (ADHD)}, sleep problems and QoL using Early Childhood Epilepsy Severity Scale (E-Chess), Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), Vineland Social Maturity Scale (VSMS), Diagnostic and Statistical manual of Mental disorders-5 (DSM-5) criteria, Children’s Sleep Habits Questionnaire (CSHQ), and PedsQL-Epilepsy module respectively. Those who did not follow-up for more than a year were requested to visit. If the visit was not feasible, they were interviewed telephonically(n=238) for epilepsy control, gross motor and hand function after verbal informed consent. The predictors of these outcomes and their correlations were also evaluated.
Results:
Among 402 children [302 boys; median age (IQR): 92(78-107) months] enrolled, one-third had persistent cessation of spasms (at day 42) after first-line therapy, while one-third were seizure-free (for at least two years) at the time of assessment and 60% evolved to Lennox-Gastaut syndrome (LGS). Comorbidities included unfavorable motor status (130/402), moderate to profound intellectual disability (111/164), ASD (42/164), ADHD (18/164), poor sleep (135/164) and impaired quality of life (115/164). The predictors of long-term epilepsy control were etiology and age at onset of spasms (OR[Odds Ratio]=2·83, p=0·035). Children with age at onset of spasms more than five months [Hazard Ratio (HR)=3·75, p=< 0·0001], and non-structural etiology (HR=3·6, p=0·001] had early remission (censored at five years of age). Also, etiology predicted long-term sleep outcome (F-3·21, p-0·006). Age at onset of spasms predicted long-term intellectual outcome (F=2·38, p=0·03), at or beyond five years of age. PedsQL score has a significant negative correlation with motor (GMFCS[ρ= -0.25] and MACS[ρ= -0.23]) and sleep outcomes(ρ= -0.32) and positive correlation with intellectual functioning (ρ= 0·62).
Conclusion:
The current study revealed a preponderance of structural etiology and a higher rate of transition from IS to LGS. The etiology and age at onset of IS were significant predictors of long-term epilepsy control. Age at onset of spasms more than five months and probable genetic etiology were the predictors of early remission by five years of age. Further follow-up studies are required for assessing the outcomes in adults with IS.
Funding:
:None