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Long-term evolution of neuropsychological competences in encephalopathy with status epilepticus during sleep: a
Maria C Pera1, Daniela Brazzo, Nausicaa Altieri
1National Neurologic Institute "C. Mondino," Child Neuropsychiatry Unit, University of Pavia, Pavia, Italy.
Insights
Continuous spikes and waves during slow wave sleep (CSWS) syndrome often leads to worsened cognitive performance in children. Long-term outcomes for CSWS are variable, influenced by treatment, duration, and cause.
Area of Science:
- Pediatric Neurology
- Neurodevelopmental Disorders
- Epilepsy Syndromes
Background:
- Continuous spikes and waves during slow wave sleep (CSWS) is an epilepsy syndrome impacting cognitive development.
- Understanding the long-term cognitive trajectory in CSWS is crucial for patient management.
Observation:
- A review of 25 children with CSWS over a mean follow-up of 13.5 years was conducted.
- Neuropsychological tests were analyzed to assess cognitive changes.
Findings:
- Cognitive performance declined in nearly all patients during CSWS.
- While 28% with nonlesional epilepsy had positive outcomes, 28% with prolonged CSWS (mean 28.1 months) experienced negative cognitive outcomes.
- Permanent cognitive impairment was observed in 44% of children with CSWS.
Implications:
- The long-term cognitive outcome in CSWS is heterogeneous.
- Factors such as treatment response, disease duration, and underlying etiology significantly influence cognitive prognosis.
- Early intervention and tailored treatment strategies may be vital for improving outcomes in pediatric CSWS.
Purpose:
The aim of this study was to evaluate the long-term cognitive outcome in children with continuous spikes and waves during slow wave sleep (CSWS syndrome).
Methods:
We reviewed the neuropsychological tests of 25 children diagnosed with CSWS between 1987 and 2010 and with a mean follow-up of 13.5 years.
Key Findings:
Cognitive performances worsened during CSWS in virtually all patients. Seven patients (28%) with nonlesional epilepsy had a positive outcome; three patients (12%) showed persistence of motor deficit without involvement of cognitive functions; and seven patients (28%) who presented a long duration of CSWS (mean = 28.1 months) had a negative cognitive outcome. In 6 patients (24%) with structural or metabolic disorders before CSWS onset cognitive outcomes did not change; 2 patients (8%) had a negative outcome irrespective of the duration or presence of other neurologic disorders before CSWS onset. Forty-four percent of children with CSWS demonstrated permanent cognitive impairment.
Significance:
The long-term outcome of CSWS syndrome is variable and seems to depend on treatment response, disease duration, and underlying etiology.
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