EPILEPSY WITH MYOCLONIC ABSENCES (EMA)

	Mandatory	Alerts	Exclusionary
Seizures	Myoclonic absence seizures		Atonic, myoclonic-atonic or tonic seizures Focal seizures
EEG	Regular 3Hz generalised spike-wave time-locked with myoclonic jerks		Focal slowing Consistently unifocal spikes Generalized slow (<2.5Hz) spike-wave (measured at the beginning of a spike-wave discharge)
Age at onset			<1 or > 12 years
Development		Moderate or greater impairment
Neurological exam		Abnormal examination
Imaging			Structural cause for the epilepsy
Course of illness			Progressive cognitive decline
Are MRI or ictal EEG required for diagnosis? An MRI should be considered to exclude other causes An ictal EEG is not required, provided myoclonic absences have been observed by the diagnosing clinician and the interictal EEG shows regular 3Hz generalised spike-wave. However, most untreated patients will have myoclonic absence seizures during routine EEG
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 a myoclonic absence seizure 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