DRAVET SYNDROME (DS)

	Mandatory	Alerts	Exclusionary
Seizures	Recurrent focal clonic (hemiclonic) febrile and afebrile seizures (which often alternate sides from seizure to seizure), focal to bilateral tonic-clonic and/or generalised clonic seizures	No history of prolonged seizures > 10 minutes Lack of fever sensitivity as a seizure trigger	Epileptic spasms Early infantile SCN1A developmental and epileptic encephalopathy
EEG		Normal background No interictal epileptiform abnormality after age 2 years
Age at onset	1-20 months	1-2 months or 15-20 months
Development at onset		Impairment present at seizure onset
Neurological exam		Abnormal exam (excluding Todd's paresis)
Imaging			Structural cause for the epilepsy
Other testing		Lack of pathogenic variant in SCN1A or other causal variant
Course of illness	Drug resistant epilepsy Intellectual impairment	Efficacy of sodium-channel blocking antiseizure medication
Are MRI or ictal EEG required for diagnosis? An MRI is not required for diagnosis but is highly recommended to exclude other causes An ictal EEG is not required for diagnosis
Syndrome in evolution: in a child <12 months who presents with a prolonged hemiclonic or tonic-clonic seizure with fever, and no other cause is found, this syndrome should be considered. Further convulsive seizures, especially if with fever, hemiclonic or prolonged, allows syndrome diagnosis. Diagnosis is further supported by the presence of a pathogenic variant in SCN1A
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed without EEG, MRI or genetic testing if all inclusion criteria are met and no exclusion criteria or alerts are met

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