Mandatory | Alerts | Exclusionary | |
---|---|---|---|
Seizures | Characteristic focal seizure with mouth and hemiface sensorimotor features, dysarthria and drooling or focal to bilateral tonic-clonic seizure from sleep If seizures occur from sleep, they occur within 1 hour of falling asleep or 1-2 hours prior to waking |
Focal motor or generalised convulsive status epilepticus > 30 minutes Usual seizure frequency > daily Seizures in the awake state only |
Generalized tonic-clonic seizures Atypical absence seizures Focal seizures with gustatory, fear or autonomic symptoms |
EEG | High amplitude centrotemporal biphasic epileptiform abnormality | Sustained focal slowing (outside of the postictal period) Persistent unilateral centrotemporal abnormality Lack of sleep activation of centrotemporal abnormality |
|
Age at onset | >12 years | <3 years or >14 years | |
Development at onset | Moderate or greater impairment | Regression with spike-wave activation in sleep (consider DEE-SWAS) | |
Neurological exam | Abnormal examination | ||
Imaging | Structural cause for the epilepsy | ||
Course of illness | Remission by mid to late adolescence No regression |
Regression with spike-wave activation in sleep (consider DEE-SWAS) | |
Are MRI or ictal EEG required for diagnosis? An MRI is not required An ictal EEG is not required |
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Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed without EEG or MRI in a child who meets all other mandatory and exclusionary clinical criteria and no alert criteria |
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