Le syndrome des myoclonies peribuccales avec absences: a propos de 2 cas.

Perioral myoclonia with absences (POMA) was first described in 1994 by CP Panayotopoulos who identified 6 cases that did not fit with the classical syndrome of absence epilepsy in children and whose predominant symptom during the absence seizure was the occurrence of myoclonia of perioral muscles. The POMA belongs to the group of generalized idiopathic epilepsies. It begins in childhood and there is a female predominance. It may be accompanied by tonic-clonic generalized seizures as well as absence status epilepticus. It has the EEG characteristics of typical absence seizures and therefore remains currently considered as such. The clinical manifestations of POMA are often misdiagnosed as focal motor seizures. This syndrome can be pharmacoresistant and is not likely to regress spontaneously. We present two clinical observations of perioral myoclonia with absences. The first case illustrates the typical electro-clinical features of this syndrome while the second illustrates its pharmacoresistance.