Unknown prevalence: it may account for up to 10 % of all generalized idiopathic epilepsies. Unknown etiology but recent studies indicate modifications of some genes including CHD2 (chromodomain helicase DNA-binding protein 2), KCNB1, KIAA2022 and NAA10. It could be that the occipital cortex is playing an important role in the genesis of seizures.

Onset during childhood, with a peak at 6-8 years of age. More common in girls (80 %) than in boys.

Characteristic sign: myoclonus of the eyelids associated or not with brief absences (less than 6 seconds) (63 %). Seizures are triggered mainly by closing the eyes in the presence of uninterrupted light. Some patients (23 %) have uncommon generalized tonic-clonic seizures. Intellectual development is generally normal but mild to moderate intellectual deficiency has been observed.

Diagnosis: based on a video-encephalogram that shows myoclonus of the eyelids (with or without absences), accompanied by photosensitivity and EEG discharges triggered by closing the eyes.

Antiepileptic treatments (valproate, benzodiazepines, levetiracetam and zonisamide) can control the seizures in 50 % of cases. Cases with tonic-clonic seizures are more often resistant to treatment.

Anesthetic implications:

epilepsy, avoiding light stimulations and strong lights

References :

-        Smith KM , Paul E Youssef PE, Wirrell EC, Nickels  KC, Payne ET, Britton JW, Shin J et al.
Jeavons syndrome: clinical features and response to treatment.
Pediatr Neurol 2018 ; 86 :46-51.

Updated: October 2022