Perioral myoclonia with absences? A case report with EEG and voltage mapping analysis

The author presents clinical and electroencephalographic data of an epileptic girl, whose absences started at the age of 2. Based on seizure semiology, serial EEG and video-EEG recordings, 7 years of follow-up and other clinical data, the patient was diagnosed as having the PMA (‘perioral myoclonus with absences’) syndrome. There were some disagreement between clinical and EEG data, however, Finally, she became completely seizure free on valproate + lamotrigine medication. Detailed EEG and voltage mapping analysis disclosed that spike movements of ictal and interictal generalized spike-wave discharges showed always the same, very consistent, peculiar topographic pattern. This pattern was fundamentally different from spike patterns associated with typical absence seizures (recorded in other patients). Analysis of clinical, EEG and voltage mapping results suggest that actually this case might be rather classified as myoclonic epilepsy. The conjuction of this case to the PMA syndrome has been discussed.