Neurocognitive Profile and Electroclinical Outcome of Steroids In Children With Dee-Swas - A Prospective Cohort Study

Introduction Prospective assessment of behavioral pattern in DEE-SWAS (Developmental/Epileptic Encephalopathy with Spike-Wave Activation In Sleep) and analysis of electroclinical response to steroids at 12 weeks.

Methods Children (2-12 years) with steroid therapy naïve DEE-SWAS (spike-wave-index(SWI>=50)) were enrolled from 2019-2023. Presenting complaints, SQ/IQ(VSMS/MISIC), behavioral problems(CBCL) and neuroimaging findings were documented. EEG re-evaluated for SWI calculation. 3-month steroid course (monthly pulses or single pulse followed by oral steroids in tapering doses) was given. Electroclinical responders defined as 25% absolute reduction in SWI with 5-point improvement in atleast one behavioral domain(CBCL). Ongoing anti-seizure medications were kept unchanged during the study period. The study was funded by AIIMS intramural research grant.

Results Forty participants with DEE-SWAS enrolled(30 boys; mean age 94.8 +/- 24.4 months. Presenting complaints were problem behaviors 40/40(100%): inattention/hyperactivity(33/40), autistic(15/40), aggression(14/40); cognitive decline 37/40(92.5%) with a mean SQ of 46.55 +/- 18.5, and ongoing seizures 26/40(65%). Etiology: structural 29/40(72.5%) and presumed genetic 11/40(27.5%). Seizures preceded neurocognitive regression by 36 months(IQR-45 months). Diagnostic delay was 12 months (IQR-30 months). The Electroclinical response was observed in 23/40(57.5%): isolated EEG response (25% absolute reduction in SWI)- 28/40(70%); isolated behavioral response(5 point change in CBCL) - 28/40(70%). SWI in EEG reduced from 87.5% +/- 13.8% to 39.32 +/- 29.91; p<0.01. 5-point improvement in inattention/hyperactivity was noted in 11/33(33.3%), autistic traits in 8/15 (53.3%) aggression in 4/14(28.5%).

Conclusion Significant diagnostic delay exists in children with DEE-SWAS. These children are hyperactive, aggressive and may mimic autistic behavior. Short-term electroclinical response to steroids is observed in 57.5% cases.