Mandatory | Alerts | Exclusionary | |
---|---|---|---|
Seizures | Focal autonomic seizures | Seizure frequency greater than monthly | |
EEG | High amplitude focal or multifocal epileptiform abnormality that increases in drowsiness and sleep | Sustained focal slowing (outside the postictal period) Persistent unifocal abnormality |
|
Age at onset | <3 years or > 8 years | <1 year or > 14 years | |
Development at onset | Moderate or greater impairment | Regression with spike-wave activation in sleep (consider DEE-SWAS) | |
Neurological exam | Abnormal exam | ||
Imaging | Structural cause for the epilepsy | ||
Course of illness | Remission by early to mid 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 mandatory for diagnosis but should be considered in the presence of any alerts An ictal EEG is not required |
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Syndrome without laboratory confirmation: in resource-limited regions, an interictal EEG is required to diagnose this syndrome |
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