CHILDHOOD OCCIPITAL VISUAL EPILEPSY (COVE)

	Mandatory	Alerts	Exclusionary
Seizures	Focal sensory visual seizures with elementary visual phenomena (multicoloured circles), predominantly or exclusively in wakefulness.	Prolonged seizure > 15 minutes Generalized tonic-clonic seizure while awake	Tonic, atonic, or atypical absence seizures Worsening myoclonus
EEG	Occipital spikes or spike-wave	Focal slowing (outside the postictal period)
Age at onset		<6 or >14 years	<1 or >19 years
Development at onset		Intellectual impairment	Regression
Neurological exam		Abnormal, clinically significant	Persistent visual field deficit
Imaging			Structural cause for the epilepsy Occipital lobe calcification
Course of illness			Regression, myoclonic seizures, spasticity, ataxia (consider CLN2)
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, as a minimum, an EEG and MRI are required to confidently diagnose this syndrome.

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