Mandatory | Alerts | Exclusionary | |
---|---|---|---|
Seizures | Focal seizures | Generalized seizures | |
EEG | Generalized epileptiform abnormality | ||
Age at onset | Neonatal onset | ||
Development at onset | Moderate or greater intellectual impairment | ||
Neurological exam | Abnormal exam | ||
Imaging | Normal or focal cortical dysplasia | ||
Other | Family history of individuals with focal seizures arising from different cortical regions in different family members | Family history of focal seizures exclusively seen <20 months of age | |
Are MRI or ictal EEG required for diagnosis? An MRI is required for diagnosis to exclude other causes An ictal EEG is not required for diagnosis |
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Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed without EEG in a patient if other mandatory and exclusionary criteria are met. However, an MRI or CT is required to exclude other structural causes |
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