Researchers at the Hospital for Sick Children, Toronto, studied the interictal, ictal, and clinical findings on video-EEG in 12 children (11 female; mean age 4.9 years, range 1.5-9 years) with Jeavons syndrome (JS). All met the diagnostic criteria of JS: eyelid myoclonia, eye closure-induced seizures/EEG paroxysms, and photosensitivity. Six (50%) had a previous diagnosis of absence seizures and 10 were taking antiepileptic medications. All 12 had normal posterior dominant alpha rhythm, reactive to eye opening and closure. Six had spiky posterior alpha activity with sustained eye closure, 10 (83%) had generalized epileptiform discharges (ED) interictally, 11 (92%) had focal posterior ictal EDs, and 9 (75%) had eyelid myoclonia and/or paroxysmal EDs induced by photic stimulation and by hyperventilation in 7 (58%). JS patients show focal posterior interictal EDs and focal posterior ictal EDs preceding generalized EDs. The findings suggest that seizures and EEG paroxysms induced by eye closure, intermittent photic stimulation, and hyperventilation are indicative of the occipital cortex initiating the generalized epilepsy network in JS. [1]

COMMENT. Jeavons syndrome (JS) is referred to as an idiopathic "eye closure-induced" reflex idiopathic generalized epilepsy. In the 2006 ILAF classification of epileptic syndromes, JS was not included as an epileptic syndrome, but eyelid myoclonia was classified as a generalized seizure. Panayiotopoulos (2005) proposed the inclusion of JS as a reflex epilepsy, and Striano et al (2009) as an idiopathic generalized epilepsy. Under the revised terminology (2005-2009) for the ILAE, proposed by Berg AT et al [2], JS would probably be included as an electroclinical syndrome and a distinctive "constellation" of "genetic" or "unknown" etiology. Four (33%) of the patients in the above series had a family history of epilepsy.