Publication

Abstract : 1 PREAMBLE The World Health Organization (WHO) intersectoral global action plan (IGAP) aims to improve the quality of life and access to treatment for people affected by neurological disorders, including epilepsy.1 The goal is also to promote and optimize brain health across the lifespan. In that regard, infantile epileptic spasms syndrome (IESS) deserves special attention as the most common developmental and epileptic encephalopathy of infantile onset.2 Timely and appropriate treatment is crucial to achieve electroclinical remission and the best possible neurodevelopmental outcomes. The preferred treatment options include hormonal therapy in the form of adrenocorticotrophic hormone (ACTH), oral corticosteroids, and vigabatrin. Critical challenges of IESS management in South Asia and other low- and middle-income countries (LMICs) include a high disease burden, the long diagnostic lag, a major treatment gap, preponderance of structural etiologies, paucity of electroencephalographic (EEG) facilities and specialist care, the need for pragmatic etiological classification, lack of standardized management practices, limited availability of ACTH in the synthetic form and vigabatrin, and socioeconomic barriers faced by families.2-6 These circumstances create significant diagnostic and treatment delays, negatively impacting the clinical outcomes and quality of life of those affected. Our research group has previously demonstrated a pooled treatment lag of 2.4 months (95% confidence interval [CI] = 2.1–2.6 months) for IESS in South Asian studies. In Indian studies, it was nearly two times higher (4.4 months, 95% CI = 4–4.8 months).3 These data are of significant concern, as treatment lag is an independent predictor of poor therapeutic response, and with each month of treatment lag, the possibility of electroclinical response decreases by 2.9% (p = .09).7 Similarly, O'Callaghan et al.,8 in their International Collaborative Infantile Spasms Study, demonstrated that longer treatment lag was associated with poorer developmental and epilepsy outcomes. The main factors for this long treatment lag in LMICs include limited knowledge and awareness among health care personnel (resulting in misdiagnoses and suboptimal treatment) and late consultation by families, due to factors such as limited access to quality care and poor health literacy among the general population.9 Furthermore, political unrest, economic slowdowns, and natural calamities often escalate treatment lag. Telemedicine harbors excellent potential to enhance IESS care in such situations but remains underutilized.10 To address these issues, we propose consensus-based Delphi statements that inform key recommendations to mitigate IESS treatment lag, thus improving the care and outcome of this condition in South Asia and other LMICs. 2 METHODS We developed the recommendations through an iterative process among the research group. Subsequently, we performed a web-based Delphi survey using the Survey Monkey Platform to achieve consensus on the recommendations. We followed CHERRIES (Checklist for Reporting Results of Internet E-Surveys) wherever applicable.11 This was a closed group survey, and the Delphi panel (n = 27) comprised members of the South Asia allied IESS Research Group, the International Bureau for Epilepsy, the International League Against Epilepsy, and the International Child Neurology Association across five geographical regions, with representation of health care professionals, researchers, epilepsy advocates, and persons with lived experience, including people with epilepsy and caregivers (Table S1). We used a 9-point Likert scale to rate the statements. The threshold to establish agreement on the recommendation was as follows: a median score of ≥7 was considered appropriate, ≤5 as inappropriate, and between 5 and 7 as needing revision. 3 RESULTS Five key recommendations (Figure 1) were developed and consensus was achieved in the first round among all 27 members with a median score of >7 (Table 1).