2016 年 8 巻 1 号 p. 1-8
Perioral myoclonia with absences, which is currently not recognized officially by the International League Against Epilepsy classification, is generalized epilepsy characterized by short absences with constant rhythmic contraction of the perioral muscles. The long-term outcome and features of this disease are not clear. We describe a patient with perioral myoclonia with absences, brief axial tonic seizures, and atypical absence status epilepticus, who was followed for 21 years. The efficacy of lamotrigine in this case was remarkable. This report suggests that when clinicians see a patient with absences accompanied by myoclonia limited to the facial area, perioral myoclonia with absences should be considered in terms of appropriate treatment and care for atypical absence status epilepticus.