<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2015-0033_s_001"><h3>Introduction</h3><p>Eyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy.</p></sec><sec id="j_joepi-2015-0033_s_002"><h3>Aim and method</h3><p>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).</p></sec><sec id="j_joepi-2015-0033_s_003"><h3>Review and differential diagnosis</h3><sec id="j_joepi-2015-0033_s_004"><h3>Definition</h3><p>eavons 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.</p></sec></sec><sec id="j_joepi-2015-0033_s_005"><h3>Demographical data</h3><p>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.</p></sec><sec id="j_joepi-2015-0033_s_006"><h3>Etiology</h3><p>JS, as is the case for all idiopathic generalized epilepsies, is genetic and the familial preponderance and concordance is high.</p><sec id="j_joepi-2015-0033_s_007"><h3>Pathophysiology</h3><p>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.</p></sec></sec><sec id="j_joepi-2015-0033_s_008"><h3>Clinical forms of JS</h3><p>we have identified four forms of JS; early onset (&lt; 4 years), mild form, classical form and an ELMA-JME form.</p></sec><sec id="j_joepi-2015-0033_s_009"><h3>Diagnosis</h3><p>the diagnosis of JS is based on the history, clinical observation and provocation and the confirmation with an EEG.</p></sec><sec id="j_joepi-2015-0033_s_010"><h3>Differential diagnosis</h3><p>is easily made from tics, other idiopathic generalized or focal cryptogenic/symptomatic epilepsies.</p></sec><sec id="j_joepi-2015-0033_s_011"><h3>Conclusion</h3><p>JS is characterized by unique electro-clinical features evoked by voluntary or on command eye closure in the light and photosensitivity.</p></sec></abstract>

IntroductionEyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy.Aim and methodThe 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 diagnosisDefinitioneavons 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 dataThe 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.EtiologyJS, as is the case for all idiopathic generalized epilepsies, is genetic and the familial preponderance and concordance is high.PathophysiologyThree 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 JSwe have identified four forms of JS; early onset (< 4 years), mild form, classical form and an ELMA-JME form.Diagnosisthe diagnosis of JS is based on the history, clinical observation and provocation and the confirmation with an EEG.Differential diagnosisis easily made from tics, other idiopathic generalized or focal cryptogenic/symptomatic epilepsies.ConclusionJS is characterized by unique electro-clinical features evoked by voluntary or on command eye closure in the light and photosensitivity.

Jeavons syndrome – updated review

Journal of Epileptology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

{"article-title":"Jeavons syndrome \u2013 updated review"}

IntroductionEyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy.Aim and methodThe aim is...

Jeavons syndrome – updated review

Online veröffentlicht: 19. Okt. 2015

Seitenbereich: 113 - 123

Eingereicht: 12. Aug. 2015

Akzeptiert: 10. Okt. 2015

DOI: https://doi.org/10.21307/joepi-2015-0033

Schlüsselwörter
epilepsy, eyelid myoclonia, absences, epilepsy with myoclonic absences, eyelid myoclonia and absences, myoclonic seizures

© 2015 Athanasios Covanis et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Jeavons syndrome – updated review

Online veröffentlicht: 19. Okt. 2015

Seitenbereich: 113 - 123

Eingereicht: 12. Aug. 2015

Akzeptiert: 10. Okt. 2015

DOI: https://doi.org/10.21307/joepi-2015-0033

Schlüsselwörterepilepsy, eyelid myoclonia, absences, epilepsy with myoclonic absences, eyelid myoclonia and absences, myoclonic seizures

© 2015 Athanasios Covanis et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Schlüsselwörter
epilepsy, eyelid myoclonia, absences, epilepsy with myoclonic absences, eyelid myoclonia and absences, myoclonic seizures