Clinical features of Infantile Epileptic Spasms Syndrome: a systematic review

Abstract Introduction Infantile Epileptic Spasms Syndrome (IESS) is a severe epilepsy in children between one month and two years of age. Urgent referral of possible IESS cases from pediatricians and family physicians who care for children to neurologists for diagnosis and treatment has been shown to significantly improve patient health outcomes. Yet, a comprehensive list of IESS clinical features is lacking. We conducted a systematic review of studies to report IESS clinical features. Methods Articles from the MEDLINE Complete and Embase databases were searched from the earliest years of databases availability (1979) to July 31, 2024. We followed the Preferred Reporting for Systematic Reviews and Meta-Analysis guidelines to conduct this systematic review with 140 articles, all of which were descriptive studies. Results We identified 25 clinical features among the 3,786 children with IESS who were included in 140 studies. No single clinical descriptor was reported in over half of the children. The six most commonly reported clinical features included flexor spasms (48.28%), mixed spasms (30.19%), extensor spasms (10.49%), symmetric spasms (8.49%), asymmetric/focal spasms (6.50%), and head nodding (2.48%). Conclusions There were a wide variety of IESS presentation descriptions. This review will help future researchers to develop an IESS screening tool that can assist pediatricians and family physicians who care for children in making urgent referrals.