Mandatory | Alerts | Exclusionary | |
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Seizures | Tonic and/or myoclonic seizures | ||
EEG | Burst suppression or multifocal discharges Diffuse slowing |
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Age at onset | Birth to 3 months (adjusted for prematurity) | ||
Development at onset | Reported normal development | ||
Neurological exam | Normal exam at onset | ||
Early Comorbidities | Developmental impairment evident at or shortly after seizure onset | ||
Course of illness | Abnormal development persists, and later includes intellectual impairment | ||
Are MRI or ictal EEG required for diagnosis? An MRI is not required for diagnosis but is strongly recommended to exclude a structural cause An ictal EEG is not required in an infant with characteristic clinical features where the interictal EEG shows burst-suppression, multifocal discharges and diffuse slowing |
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Syndrome without laboratory confirmation: in resource-limited regions, this syndrome cannot be diagnosed without an interictal EEG |
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