Perioral myoclonia with absences: an overlooked and misdiagnosed generalized seizure type

The goal of this report is to draw attention to perioral myoclonia with absences, a rare and underrecognized generalized seizure type.

We describe a 31-year-old, mildly retarded patient who had absence seizures with prominent perioral movements and rare generalized tonic-clonic convulsions. He had an attack of status epilepticus consisting of asymmetrical perioral myoclonic jerks and was misdiagnosed with epilepsia partialis continua.

The perioral myoclonia were associated with brief lapses of consciousness and with a generalized EEG pattern as shown by the synchronous video/EEG investigation with polygraphic recordings. The patient was diagnosed as having the syndrome of perioral myoclonia with absences described by C.P. Panayiotopoulos, C.D. Ferrie, S.E. Giannakodimos, and R.O. Robinson (in: Wolf P, editor. Epileptic seizures and syndromes. London: John Libbey; 1994, p. 143-53).

We emphasize that this rare and peculiar seizure type associated with generalized epilepsy can be misdiagnosed as focal epilepsy.