Clinical spectrum and treatment outcome of 95 children with continuous spikes and waves during sleep (CSWS)

Area of Science:

• Pediatric Neurology
• Epileptology
• Clinical Neuroscience

Background:

• Continuous spikes and waves during sleep (CSWS) is a severe epileptic encephalopathy in children, marked by generalized epileptiform activity and neurocognitive decline.
• Etiologies and treatment outcomes for CSWS are highly variable, often necessitating empirical therapeutic approaches.

Purpose of the Study:

• To analyze the clinical characteristics, etiological factors, and treatment outcomes in a large cohort of children diagnosed with CSWS.
• To investigate trends in the identification of genetic causes and the effectiveness of various treatments for CSWS.

Main Methods:

• Retrospective analysis of clinical and electroencephalogram (EEG) data from 95 children diagnosed with CSWS between 1998 and 2018 at a single university hospital.
• Evaluation of etiological factors (structural, metabolic, genetic) and treatment responses, including the number and types of interventions received.

Main Results:

• A structural or metabolic cause was identified in 43.2% of patients, genetic alterations in 17.9%, and the etiology remained unknown in 38.9%.
• The proportion of patients with identified genetic causes significantly increased from 10.3% in 1998-2007 to 22.8% in 2008-2018.
• Over 70% of cases were refractory to treatment, with steroids and neurosurgery demonstrating the most significant efficacy. No significant difference in outcomes was noted between CSWS and Near-CSWS classifications.

Conclusions:

• CSWS is confirmed as an age-dependent epileptic encephalopathy where structural brain abnormalities are prevalent, but genetic etiologies are increasingly recognized.
• The findings underscore the need for more precise diagnostic criteria and evidence-based treatment goals for CSWS.
• This study provides valuable data for clinical decision-making in the diagnosis and management of pediatric CSWS.