Mandatory | Alerts | Exclusionary | |
---|---|---|---|
Seizures | Generalised tonic-clonic seizures | Consistent focal features at seizure onset | Generalised myoclonic-tonic-clonic seizures (suggests juvenile myoclonic epilepsy) All other seizure types |
EEG | 3-5.5Hz generalised spike- or polyspike-wave | Focal slowing Consistently unifocal epileptiform abnormality Generalised slow (<2.5Hz) spike-wave (measured at the beginning of the spike-wave discharge) Diffuse background slowing (outside the postictal period) |
|
Age at onset | 5-9 or 26-40 years | <5 or >40 years | |
Development at onset | Mild intellectual impairment | Moderate or greater intellectual impairment | |
Neurological exam | Abnormal, potentially clinically significant | ||
Imaging | Abnormal, potentially clinically significant | Structural cause for the epilepsy | |
Course of illness | Progressive cognitive decline | ||
Are MRI or ictal EEG required for diagnosis? An MRI is not required for diagnosis, but should be considered in patients who have alerts or clinical concern for a structural aetiology An ictal EEG is not required |
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Syndrome without laboratory confirmation: in resource-limited regions, this syndrome cannot be diagnosed without interictal EEG showing generalised spike-wave, as a focal epilepsy cannot be excluded |
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