Epilepsy with continuous spikes and waves during sleep

In epilepsy with continuous spike and waves during slow wave sleep (or electrical status epilepticus during slow sleep), continuous sleep-related EEG discharges, persisting for months to years, are associated with cognitive decline. The syndrome appears in previously healthy or in delayed children. Brain lesions, especially polymicrogyria and porencephaly, are found in 30–50% of patients. Onset is insidious. Seizures start at 3–5 years as nocturnal and focal attacks are similar to rolandic epilepsy. After a few months, continuous spike and waves during slow wave sleep and atypical or atonic absences appear. There is marked decrease in intelligence quotient  scores with attention deficit and hyperactivity, sometimes with language disturbances and autistic features. Long‑term course of epilepsy is favourable, but cognitive impairment, persists in most children. A long duration of continuous spike and waves during slow wave sleep is the major factor of a poor prognosis. The benign atypical partial epilepsy syndrome bears a close relation to continuous spike and waves during slow wave sleep. Drug treatment is the same as in Landau-Kleffner syndrome.
 

If you have a child who you think may have this condition and you would like us to review the diagnosis or provide genetic testing please contact us at neuroscience@meyer.it.