An open-label trial of levetiracetam in 35 patients (23 girls) with eyelid myoclonia (EM) was conducted at the Epilepsy Center, Federico II University, Napoli, and several additional epilepsy centers in Italy. Levetiracetam dosage was 10 mg/kg/day up to 50-60 mg/kg/day in 2 doses in a 12-18 week titration and evaluation phase. Mean dose was 1985 mg/day. Patients' mean age was 19 +/- 6 yrs. Of 28 (80%) patient responders, 6 were seizure-free, 15 had a >75% and 7 a >50% seizure reduction. Associated generalized tonic clonic seizures (GTCS) in 21 patients were controlled in 14 (66%). The number of days with EM and GTCS was significantly reduced compared to baseline. Paroxysmal abnormalities at eye closure and photoparoxysmal response disappeared or were reduced in 20 responders. Mean follow-up was 24 months. [1]
COMMENT. Eyelid myoclonia with absences (Jeavons syndrome, 1977) is characterized by the triad of eyelid myoclonia with or without absences (EMA), seizures and EEG paroxysms induced by eye closure, and photosensitivity (Panayiotopoulos 1996, and others), Onset is in childhood, 2-14 years (mean 8 yrs), especially in girls. Marked intermittent jerking of eyelids after eye closure is associated with jerky upward deviation of eyeballs and retropulsion of the head. Generalized tonic clonic seizures are often associated. AED therapy is usually disappointing, and levetiracetam is the most promising. EMA is not yet recognized as a definite epileptic syndrome by the DLAE.