Mandatory | Alerts | Exclusionary | |
---|---|---|---|
Seizures | Focal sensory visual seizure triggered by photic stimuli | Prolonged seizures > 15 minutes | Eyelid myoclonia Worsening myoclonus |
EEG | Occipital epileptiform abnormality facilitated by eye closure and intermittent photic stimulation | Focal slowing (outside the postictal period) Photoparoxysmal response at low flash frequencies (suggests CLN2) |
|
Age at onset | <4 or >17 years | <1 or >50 years | |
Development at onset | Moderate or greater impairment | Regression | |
Neurological exam | Abnormal exam | Permanent visual field deficit | |
Imaging | Structural cause for the epilepsy | ||
Are MRI or ictal EEG required for diagnosis? An MRI is required for diagnosis to exclude other causes. An ictal EEG is not required. |
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Syndrome without laboratory confirmation: in resource-limited regions, this syndrome cannot be diagnosed without EEG or MRI |
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