Abstracts

Rationale: Rapid initiation of first-line treatment of infantile spasms (IS) is essential, as little as 1 week treatment delay can affect developmental outcomes. Therefore, it is important to ascertain the precise timing of spasms onset from caregivers in order to uncover potential treatment delay. In this single-center study, we aimed to determine the percentage of cases the date of onset of spasms (DOS) could be determined exactly or estimated within one week, based on caregiver report in new onset IS. We also analyzed the reasons for the inability to identify IS onset to the nearest week and propose suggestions to improve identification of timing of spasms onset.

Methods: Retrospective chart review of children with new onset IS at Boston Children’s Hospital between 2019-2022. Demographic and clinical information was collected. Four reviewers (different levels of training) independently collected data regarding timing of IS onset to verify accuracy with two independent reviewers cross-verifying data. Multivariate logistic regression was performed to evaluate for predictors of difficulty estimating DOS within 1 week of spasms onset to those in whom such a determination was not possible (Table 1). Reasons for failure to identify timing of spasms onset to the nearest week were categorized as either physician-related, disease-related or caregiver-related (Table 2).

Results: Total of 100 new onset IS patients (43% female) were identified. Developmental delay was noted in 69; etiology was known in 76. Spasms were preceded by other seizures in 45 patients and intermixed with other seizure types in 32. Based on caregiver report, the timing of the spasms onset could accurately be determined to the exact date or estimated to within 1 week in only 53% of new onset IS.  Estimating the timing of spasms onset to within a month was possible in 79% and was indeterminate in the remainder. On univariate analysis, age at IS onset, race, developmental delay, epileptic spasms intermixed with other seizures in sequence and lack of clustering of spasms were associated with inability to establish the timing of the spasms to nearest week (Table 1). On multivariate analysis, failure to ascertain the timing of spasms onset was related to spasms characteristics: epileptic spasms were intermixed with other seizures in sequence (p=0.004) and lack of clustering of spasms (p=0.003). Difficulties in ascertaining the timing was physician-related (n=22) with 5/22 being due to poor documentation, caregiver-related (n=20), disease-related (n=29), and 34 cases having multiple contributing factors (Table 2).

Conclusions: Treatment lag influences short-term treatment response and affects development. We find that in nearly half of new onset IS cases, uncovering timing onset of spams to nearest week was not possible, thereby hindering the determination of the treatment lag accurately. A lack of clarity in timing of IS onset was related to spasms characteristics in addition to some modifiable factors such as parental and clinician awareness on recognition of this condition. We propose interventions that could lead to improvement in DOS determination and thereby potentially decreasing delays to treatment.

Funding: Not applicable