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PHOTOSENSITIVE OCCIPITAL LOBE EPILEPSY (POLE)

  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.
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

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