DEVELOPMENTAL AND/OR EPILEPTIC ENCEPHALOPATHY WITH SPIKE-WAVE ACTIVATION IN SLEEP (DEE-SWAS, EE-SWAS)
- Lennox-Gastaut syndrome - slow spike-waves are frequent both in awake and asleep states, the sleep EEG shows generalized paroxysmal fast (≥10Hz) activity and tonic seizures may be captured, focal motor seizures are uncommon.
- Structural brain abnormality - spike-wave activation in sleep can occur without associated cognitive or behavioural regression.
- Self-limited focal epilepsy syndromes of childhood - spike-wave activation in sleep can occur without associated cognitive or behavioural regression.
- Etiologies with regression (e.g. autism spectrum disorder) - spike-wave activation in sleep can occur, but regression is not temporally associated with the emergence of the spike-wave activation in sleep.
CAUTION This syndrome is rare, accounting for only ~0.5% of all epilepsy presentations, it should not be over-diagnosed, there should be clear and persisting regression temporally related to the emergence of spike-wave activation in non-REM sleep.