Authors :
Presenting Author: Gozde Erdemir, MD – Penn State University
Yunting Fu, MLS – University of Maryland
Jessica Carpenter, MD – University of Maryland
Chethan Rao, DO, MS – University of Maryland
Rationale:
Infantile Epileptic Spasms Syndrome (IESS) is a severe epileptic disorder that presents in early infancy, characterized by a distinct type of seizures. Early diagnosis and prompt initiation of treatment are critical to improving neurological outcomes. Despite its well-recognized clinical features, there remains variability in the management of IESS across different countries and healthcare settings, leading to potential disparities in patient outcomes. The primary objective of this review is to compare existing guidelines on the management of IESS, highlighting their similarities, differences, and areas where further research or consensus is needed.
Methods:
This study was registered on PROSPERO and conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. EMBASE (Elsevier), MEDLINE (Ovid), Scopus and Cochrane were searched for the terms “epileptic spasm”, 'infantile spasms”, “west syndrome”, “infantile epileptic spasms syndrome”, “epileptic spasms” AND “guidelines”, “best practice”, “consensus”, “recommendations”, “‘position paper”, “commission”. Guidelines and expert consensus papers available in English were included in the review. Two pediatric epileptologists independently screened and identified studies for eligibility.
Results:
The initial search yielded 349 references. Twelve full-text articles published between 2014 and August 2024 met the inclusion and exclusion criteria. Four guidelines focused specifically on diagnosis and management of IESS during the COVID-19 pandemic. The authors, publication years, and topics of the guidelines are summarized in Table 1. All manuscripts emphasize the need for urgent EEG within days of suspected epileptic spasms and immediate treatment upon diagnosis. Oral prednisolone, ACTH and vigabatrin are the mainstays of recommended treatment. ACTH is not available in some regions. Vigabatrin is consistently preferred for TSC patients, but treatment duration is poorly defined. There is no consensus on dual therapy and evidence for effectiveness of combining standard medications is lacking. Recommendations also differ regarding metabolic/genetic workups, infectious prophylaxis, and timing of follow-up EEGs. Associated health concerns like vaccination schedule modification, gastric protection, and parental education of immune suppression concepts are inconsistently discussed. COVID-19 guidelines suggested outpatient evaluation and oral prednisolone use can be effective for non-TSC cases.
Conclusions:
Guidelines on IESS share core recommendations but vary by region and resource availability. Key uncertainties include optimal dosing and tapering of ACTH and steroids, vigabatrin duration, and side effect management. The benefits of combining hormonal therapy with vigabatrin also remains unclear. Future research should aim to unify guidelines and tailor treatments to specific genetic or metabolic causes of IESS.
Funding: none