EPILEPSY WITH EYELID MYOCLONIA (EEM)

	Mandatory	Alerts	Exclusionary
Seizures	Eyelid myoclonia	Eyelid myoclonia not elicited with slow eye closure in bright light in an untreated patient Myoclonic jerks of the limbs (suggests juvenile myoclonic epilepsy)	Myoclonic-absence seizures Focal seizures
EEG	Eye closure and intermittent photic stimulation elicit fast (3-6Hz) generalised polyspike or polyspike-wave		Focal slowing Consistently unifocal spikes Generalized slow (<2.5Hz) spike-wave (measured at the beginning of a spike-wave discharge) Diffuse background slowing (outside the postictal period) Typical clinical event with no ictal EEG pattern
Age at onset			<2 or > 14 years
Neurological exam		Abnormal examination
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 An ictal EEG is not required, provided mandatory criteria have been met, with the eyelid myoclonia observed by the diagnosing clinician. However, most untreated patients will have seizures on a routine EEG performed with intermittent photic stimulation
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed without EEG if all other mandatory criteria and no exclusionary criteria are met and eyelid myoclonia has been witnessed (in person or captured on video)

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