Mandatory | Alerts | Exclusionary | |
---|---|---|---|
Seizures | Focal sensory auditory seizures and/or focal cognitive seizures with receptive aphasia | Generalized seizures Other focal seizures |
|
EEG | Generalized epileptiform abnormality | ||
Age at onset | |||
Development at onset | Moderate or greater intellectual impairment | ||
Neurological exam | Abnormal exam | ||
Imaging | Normal or focal cortical dysplasia | ||
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 |
|||
Syndrome without laboratory confirmation: in resource-limited regions, MRI is still required to exclude other 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