Jeavons syndrome – updated review

Abstract

Summary Introduction. Eyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy. Aim and method. The aim is to consider the updated electro-clinical pathophysiology and to discuss terminology, classification and differential diagnosis. The review includes our own research and relevant papers on the subject of Jeavons syndrome (JS). Review and differential diagnosis. Definition: Jeavons syndrome is a generalized idiopathic (genetic) epilepsy syndrome (IGE) characterized by eyelid myoclonia, other seizures (absences, myoclonic and or generalized tonic-clonic) and EEG paroxysms induced by voluntary or on command eye closure, in the light and photosensitivity. Demographical data: The prevalence of JS has been reported to vary from 7.3% to 12.9% among idiopathic generalized epilepsies and 2.5% to 2.7% among all patients with epileptic disorders. Etiology: JS, as is the case for all idiopathic generalized epilepsies, is genetic and the familial preponderance and concordance is high. Pathophysiology: Three factors are important in order for JS to manifest clinically; the genetic predisposition, the voluntary or on command eye closure and the light input. Clinical forms of JS: we have identified four forms of JS; early onset (< 4 years), mild form, classical form and an ELMA-JME form. Diagnosis: the diagnosis of JS is based on the history, clinical observation and provocation and the confirmation with an EEG. Differential diagnosis: is easily made from tics, other idiopathic generalized or focal cryptogenic/symptomatic epilepsies. Conclusion. JS is characterized by unique electro-clinical features evoked by voluntary or on command eye closure in the light and photosensitivity.