Eyelid myoclonia with absences related to epileptic negative myoclonus

Eyelid myoclonia with absences (EMA) is an epileptic syndrome characterised by eyelid myoclonia with or without absences, eye closure-induced paroxysms, and photosensitivity. The relationship between EMA and epileptic negative myoclonus has not previously been reported. Herein, we describe a case of a 10-year-old girl who presented with eyelid myoclonia, eye closure-induced paroxysms, and photosensitivity, which was compatible with the diagnosis of EMA. Ictal EEG depicted an eye closure-induced diffuse 3.0-4.5-Hz polyspike-and-wave complex, which was accompanied by eye fluttering (eyelid myoclonia). EMG disclosed a brief interruption (60-140 mseconds) of tonic contraction of the orbicularis oculi muscle, which was associated with the polyspike-and-wave complex on EEG. The findings led to the diagnosis of epileptic negative myoclonus. Eye closure induced eyelid epileptic negative myoclonus, demonstrated in this patient, may be an atypical seizure type of EMA that represents an intermediate between eyelid myoclonia and epileptic negative myoclonus.