Abstract
Objectives
To elucidate the electroclinical characteristics of infantile epileptic spasms syndrome (IESS) and to determine any potential association among these with underlying etiologies and response to therapy.
Methods
Sixty-eight, treatment-naive children with IESS underwent long-term video electroencephalogram (EEG) recording, which was used to characterize the semiology, ictal, and inter-ictal EEG patterns. Children were further followed up to assess electroclinical predictors of etiologies and short-term therapeutic response.
Results
Of 68 children enrolled (69% boys), the median age at enrollment was 10.5 mo (IQR-8). Eighty-eight percent of children had flexor spasms, followed by mixed (7%) and extensor (4.4%). Asymmetrical spasms were noted in 17.6% children, and all of them had underlying structural etiology. Two children had the status of epileptic spasms. In the present cohort, authors recognized five distinct ictal EEG correlates of epileptic spasms; the frontocentral dominant slow wave was the most prevalent (32%), followed by the generalized slow-wave complex with superimposed fast rhythm in 29.4%. The occipital dominant slow wave complex was a peculiar pattern in 16%. The major underlying etiologies were hypoxic-ischemic brain injuries (36.7%) and neonatal hypoglycemic brain injuries (22%). Besides asymmetric spasms, authors could not identify any significant association among electroclinical characteristics, underlying etiologies and response to therapy in this study.
Conclusions
The electroclinical landscape of IESS is peculiar and diverse in developing countries. The presence of asymmetrical spasms indicated underlying structural etiology.
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Acknowledgements
SN (Grant reference No.3/1/3/147/Neuro/2021-NCD-1) was supported by the Indian Council of Medical Research (ICMR). JKS received grant from Indian Council of Medical Research paid to his Institute (Reference No F.N.7/1/3/2018-RMC). The authors also acknowledge Vinay Kumar, Isheeta Shyam, Baljit Singh and Seema Nain who performed video EEG recordings. They also acknowledge families of all children with infantile epileptic spasms syndrome who participated in the study.
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PP: study design, drafting the work, data collection, data analysis, data interpretation, manuscript writing and approval of the manuscript; SN, PM, CA: drafting the work, data collection, data analysis, data interpretation, manuscript writing and approval of the manuscript; JB, AGS, RS, NS: study design, drafting the work, data collection, data interpretation, manuscript writing and approval of the manuscript; JKS: conceptualization of study and design, drafting the work, data collection, data interpretation, manuscript writing and approval of the manuscript. JKS will act as guarantor for this manuscript.
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Pal, P., Negi, S., Baishya, J. et al. Electroclinical Landscape of Infantile Epileptic Spasms Syndrome. Indian J Pediatr (2024). https://doi.org/10.1007/s12098-023-05017-6
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DOI: https://doi.org/10.1007/s12098-023-05017-6