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EEG stabilization and cognitive gains after pulse steroid therapy in developmental and/or epileptic encephalopathy
Neetha Balaram1, Rajith Ravindren2, S R Ratheesh3
1Department of Neurology, Government Medical College, Kozhikode, Kerala, India.
Insights
Pulse steroid therapy significantly reduced seizures and EEG abnormalities in children with D/EE-SWAS, with cognitive improvements seen mainly in idiopathic cases. Early EEG response predicted long-term outcomes.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neuroscience
Background:
- Developmental and/or epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS) is a rare pediatric condition causing seizures and cognitive decline.
- Corticosteroid use is common, but data on structured pulse steroid regimens and long-term neurocognitive outcomes are limited, especially in resource-limited settings.
Purpose of the Study:
- To characterize the clinical, EEG, and neuropsychological profile of children with D/EE-SWAS.
- To evaluate the electroclinical and cognitive impact of a six-month pulse intravenous methylprednisolone (IVMP) regimen.
Main Methods:
- A prospective cohort study involving 29 children with D/EE-SWAS in India, followed for two years.
- Monthly pulse IVMP for six months, with stable anti-seizure medications.
- Serial EEGs to assess spike-wave index (SWI) and spike dipole stability quotient (SQ); neuropsychological evaluations at baseline and two years.
Main Results:
- Pulse IVMP significantly reduced seizure frequency and SWI over 24 months, with a progressive increase in SQ.
- Early EEG response (SWI reduction, SQ increase) correlated with seizure control; early dipole stabilization showed prognostic value.
- Neuropsychological outcomes showed overall improvements in attention and verbal learning, with better cognitive recovery in the idiopathic D/EE-SWAS group.
Conclusions:
- A six-month pulse IVMP regimen effectively suppressed epileptiform discharges and stabilized brain networks in D/EE-SWAS.
- Etiology (idiopathic vs. symptomatic) critically impacted long-term EEG stability and cognitive recovery.
- Early stabilization of brain network activity (dipole stability) may predict long-term seizure control.
Background:
Developmental and/or epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS) is a rare paediatric epileptic encephalopathy characterized by marked activation of epileptiform discharges during non-REM sleep, leading to seizures and neurocognitive regression. Although corticosteroids are frequently used, prospective data on structured pulse steroid regimens and long-term neurocognitive outcomes from resource-limited settings are scarce.
Objective:
To characterize the clinical, electroencephalographic, and neuropsychological profile of children with D/EE-SWAS and to evaluate the electroclinical and cognitive impact of a six-month pulse intravenous methylprednisolone (IVMP) regimen.
Methods:
In this prospective cohort study, 29 children with D/EE-SWAS were enrolled from two tertiary centers in India and followed for two years. Monthly IVMP (25 mg/kg/day for 3 days) was administered for six months, with stable anti-seizure medications. Serial overnight EEGs assessed spike-wave index (SWI) and spike dipole stability quotient (SQ), a qualitative marker of cortical source coherence. Neuropsychological evaluations covering attention, memory, executive, visuospatial, and adaptive functioning were conducted at baseline and at two years. Repeated-measures ANOVA, paired t-tests and correlation statistics were used for statistical analysis.
Results:
Pulse IVMP therapy resulted in a significant and sustained reduction in seizure frequency and SWI over 24 months (p < 0.001), with a progressive increase in SQ. Improvement in SWI and seizure control occurred early, while SQ improved significantly only after 12 months. Idiopathic D/EE-SWAS showed durable electroclinical improvement, whereas symptomatic cases demonstrated transient gains with partial relapse at two years. Early EEG response was prognostic- higher SWI and lower SQ at six months predicted higher seizure frequency at 24 months. Neuropsychological outcomes revealed significant improvements in attention and verbal learning overall, with meaningful domain-specific cognitive recovery predominantly confined to the idiopathic group.
Conclusion:
A structured six-month pulse IVMP regimen was associated with early spike suppression and delayed network stabilisation. Etiology significantly influenced long-term EEG stability and cognitive recovery. Early dipole stabilization may serve as a prognostic biomarker for long-term seizure control beyond spike burden alone.
