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Electrophysiological patterns and therapeutic outcomes in DEE-SWAS: A cohort study from a tertiary pediatric
1Division of Child Neurology, Department of Pediatrics, Kanuni Education and Training Hospital, Trabzon University Medical School, Trabzon, Turkey.
Insights
Early reduction in spike-wave index (SWI) in pediatric patients with developmental and epileptic encephalopathy with SWAS indicates a better prognosis. Persistent high SWI and abnormal EEG activity predict long-term neurological impairment.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Electrophysiology
Background:
- Developmental and epileptic encephalopathy with spike-wave activation during sleep (DEE-SWAS) is a severe condition in children.
- Understanding prognostic factors is crucial for effective management of DEE-SWAS.
Purpose of the Study:
- To characterize clinical, electrophysiological, and radiological features of pediatric DEE-SWAS.
- To identify predictors of prognosis in children with DEE-SWAS.
Main Methods:
- Retrospective cohort study of 25 pediatric patients with DEE-SWAS and a spike-wave index (SWI) ≥50%.
- Analysis of clinical, EEG, and neuroimaging data over a minimum 2-year follow-up.
- Assessment of factors associated with residual neurological or electrophysiological abnormalities.
Main Results:
- Patients with residual findings showed higher SWI at 3, 6, and 24 months (p<0.05).
- Lower EEG background frequency and higher amplitude during wakefulness were observed in those with residual findings (p<0.05).
- Abnormal neurological examination correlated significantly with residual findings (p=0.02).
Conclusions:
- A significant early reduction in SWI is linked to a better prognosis in pediatric DEE-SWAS.
- Persistent elevated SWI and abnormal background EEG activity predict long-term neurological deficits.
- Early identification and tailored treatment are vital for managing DEE-SWAS.
Objective:
This study aimed to evaluate the clinical, electrophysiological, and radiological characteristics of pediatric patients diagnosed with developmental and epileptic encephalopathy with spike-wave activation during sleep (DEE-SWAS) and to identify factors associated with prognosis.
Methods:
Twenty-five pediatric patients with a spike-wave index (SWI) ≥50% and a minimum of 2 years of follow-up were analyzed in this retrospective cohort study. Clinical data, electroencephalogram (EEG) findings, and neuroimaging results were assessed, and their relationship with residual neurological or electrophysiological abnormalities was examined.
Results:
The patients' mean age was 9.4 ± 2.97 years, with a mean age at seizure onset of 6.6 ± 2.32 years. Etiologically, 60% of patients were unknown, and 40% were known. The baseline mean SWI was 67.1%, decreasing to 13.8% by the 24th month. Patients with residual findings had significantly higher SWI values at months 3, 6, and 24 (p = .04, p = .006, and p = .01, respectively). Additionally, these patients exhibited lower EEG background frequency and higher amplitude during wakefulness (p = .026 and p = .001). Abnormal neurological examination was significantly associated with the presence of residual findings (p = .02).
Significance:
Early and substantial reduction in SWI is associated with better prognosis. Persistent high SWI and abnormal background EEG activity may serve as predictors of long-term neurological impairment. These findings underscored the importance of early identification of high-risk patients and personalized treatment strategies in the management of DEE-SWAS.
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