PROGRESSIVE MYOCLONUS EPILEPSIES (PME)

	Mandatory	Alerts	Exclusionary
Seizures	Myoclonic seizures
EEG	Generalized spike-/polyspike-wave		Persistent focal epileptiform abnormality, other than occipital
Age at onset	2-50 years	>20 years
Development at onset	Normal
Neurological exam	Normal
Imaging	Normal at onset
Course of illness	Progressive: worsening of seizures cognitive regression neurological abnormality e.g. cerebellar signs EEG background slowing and/or increased EEG epileptiform abnormality
Are MRI or ictal EEG required for diagnosis? MRI or ictal EEG is not required
Syndrome without laboratory confirmation: in resource-limited regions, a person who meets mandatory criteria and does not meet alert or exclusionary criteria, and who shows a progressive worsening of myoclonic seizures and neurological and cognitive decline, can be suspected of having 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