DEVELOPMENTAL AND/OR EPILEPTIC ENCEPHALOPATHY WITH SPIKE-WAVE ACTIVATION IN SLEEP (DEE-SWAS, EE-SWAS)

	Mandatory	Alerts	Exclusionary
Seizures		Tonic seizures from sleep	Epileptic spasms
EEG	Slow (1.5-2Hz) spike-wave in non-REM sleep Abnormalities are markedly activated in sleep	Generalized paroxysmal fast activity in sleep Generalized slow (<2.5Hz) spike-wave, awake and asleep
Age at onset		1-2 years	<1 year, >12 years
Development at onset	Cognitive, behavioural or motor plateau/regression temporally related to spike-wave activation in sleep on EEG
Course of illness	Remission of spike-wave activitation in sleep by mid adolescence
Are MRI or ictal EEG required for diagnosis? An MRI is not required for diagnosis but is often performed to evaluate for underlying cause A sleep EEG is required for diagnosis
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome cannot be diagnosed without a sleep EEG

NOTE Alert criteria are absent in the vast majority of patients with the syndrome, but rarely can be seen. Their presence should result in caution in diagnosing the syndrome and consideration of other conditions