Jeavons syndrome

Eyelid myoclonia with absences or Jeavons syndrome is clinically characterized by rapid eyelid myoclonia with or without loss of consciousness. Seizures are provoked after eye closure with generalized discharges, mainly polyspikes in EEG and photosensitivity. Patients may occasionally have generalized tonic-clonic seizures. It is a lifelong disorder that begins in childhood with a striking female predominance. We report on three children (two female and one male) with eyelid myoclonia, observed at the age of 6 years in the boy and one girl, and at the age of 14 years in the older girl. In the youngest patient (the boy), the diagnosis was made after 2 months, in young girl after 2.5 years, and in older girl after 1.5 year of eyelid myoclonia persistence. The boy was referred to the hospital because of pathologic EEG and a „tic“ that manifested as eyelid myoclonia and occasional eyeball deviation, the young girl because of recurrent syncope and generalized epileptic changes in EEG, and the older girl because of syncope. Eyelid myoclonia after closure of the eyes followed by generalized epileptic discharges typical for Jeavons syndrome was confirmed by video EEG. All patients had normal neurologic status and intellectual development. The disease was stabilized by valproic acid and ethosuximide. In conclusion, proper knowledge of Jeavons syndrome is necessary to avoid inaccurate diagnosis of tics or other idiopathic generalized epileptic syndrome with absence type seizures.

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