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Management of epilepsy with eyelid myoclonia: Results of an international expert consensus panel
There are limited data about the treatment and management of epilepsy with eyelid myoclonia (EEM). The objective of this study was to determine areas of consensus among an international panel of experts for the management of EEM (formerly known as Jeavons syndrome). An international steering committ...
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Published in: | Epilepsia (Copenhagen) 2023-09, Vol.64 (9), p.2342-2350 |
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creator | Smith, Kelsey M Wirrell, Elaine C Andrade, Danielle M Choi, Hyunmi Trenité, Dorothée Kasteleijn-Nolst Jones, Hannah Knupp, Kelly G Mugar, Jon Nordli, Jr, Douglas R Riva, Antonella Stern, John M Striano, Pasquale Thiele, Elizabeth A Zawar, Ifrah |
description | There are limited data about the treatment and management of epilepsy with eyelid myoclonia (EEM). The objective of this study was to determine areas of consensus among an international panel of experts for the management of EEM (formerly known as Jeavons syndrome).
An international steering committee was convened of physicians and patients/caregivers with expertise in EEM. This committee summarized the current literature and identified an international panel of experts (comprising 25 physicians and five patients/caregivers). This panel participated in a modified Delphi process, including three rounds of surveys to determine areas of consensus for the treatment, other areas of management, and prognosis for EEM.
There was a strong consensus for valproic acid as the first-line treatment, with levetiracetam or lamotrigine as preferable alternatives for women of childbearing age. There was a moderate consensus that ethosuximide and clobazam are also efficacious. There was a strong consensus to avoid sodium channel-blocking medications, except for lamotrigine, as they may worsen seizure control. There was consensus that seizures typically persist into adulthood, with remission occurring in |
doi_str_mv | 10.1111/epi.17682 |
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An international steering committee was convened of physicians and patients/caregivers with expertise in EEM. This committee summarized the current literature and identified an international panel of experts (comprising 25 physicians and five patients/caregivers). This panel participated in a modified Delphi process, including three rounds of surveys to determine areas of consensus for the treatment, other areas of management, and prognosis for EEM.
There was a strong consensus for valproic acid as the first-line treatment, with levetiracetam or lamotrigine as preferable alternatives for women of childbearing age. There was a moderate consensus that ethosuximide and clobazam are also efficacious. There was a strong consensus to avoid sodium channel-blocking medications, except for lamotrigine, as they may worsen seizure control. There was consensus that seizures typically persist into adulthood, with remission occurring in <50% of patients. There was less agreement about other areas of management, including dietary therapy, lens therapy, candidacy for driving, and outcome.
This international expert panel identified multiple areas of consensus regarding the optimal management of EEM. These areas of consensus may inform clinical practice to improve the management of EEM. In addition, multiple areas with less agreement were identified, which highlight topics for further research.</description><identifier>ISSN: 0013-9580</identifier><identifier>EISSN: 1528-1167</identifier><identifier>DOI: 10.1111/epi.17682</identifier><identifier>PMID: 37326215</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>Caregivers ; Disease management ; Epilepsy ; Etiracetam ; Eyelid ; Lamotrigine ; Nutrition therapy ; Patients ; Remission ; Seizures ; Valproic acid</subject><ispartof>Epilepsia (Copenhagen), 2023-09, Vol.64 (9), p.2342-2350</ispartof><rights>2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.</rights><rights>2023. This article is published under http://creativecommons.org/licenses/by-nc-nd/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c348t-5c03a1f5e7c66f1790442f2a8842881630ae65b654e2e1ec62e7f0da2ff50b2c3</citedby><cites>FETCH-LOGICAL-c348t-5c03a1f5e7c66f1790442f2a8842881630ae65b654e2e1ec62e7f0da2ff50b2c3</cites><orcidid>0000-0002-1967-0827 ; 0000-0003-3431-4713 ; 0000-0002-0826-2350 ; 0000-0002-3549-1642 ; 0000-0001-8659-3801 ; 0000-0001-9152-5571 ; 0000-0002-6065-1476 ; 0000-0002-6103-4250 ; 0000-0003-3015-8282 ; 0000-0002-6659-2614</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,778,782,27907,27908</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37326215$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Smith, Kelsey M</creatorcontrib><creatorcontrib>Wirrell, Elaine C</creatorcontrib><creatorcontrib>Andrade, Danielle M</creatorcontrib><creatorcontrib>Choi, Hyunmi</creatorcontrib><creatorcontrib>Trenité, Dorothée Kasteleijn-Nolst</creatorcontrib><creatorcontrib>Jones, Hannah</creatorcontrib><creatorcontrib>Knupp, Kelly G</creatorcontrib><creatorcontrib>Mugar, Jon</creatorcontrib><creatorcontrib>Nordli, Jr, Douglas R</creatorcontrib><creatorcontrib>Riva, Antonella</creatorcontrib><creatorcontrib>Stern, John M</creatorcontrib><creatorcontrib>Striano, Pasquale</creatorcontrib><creatorcontrib>Thiele, Elizabeth A</creatorcontrib><creatorcontrib>Zawar, Ifrah</creatorcontrib><title>Management of epilepsy with eyelid myoclonia: Results of an international expert consensus panel</title><title>Epilepsia (Copenhagen)</title><addtitle>Epilepsia</addtitle><description>There are limited data about the treatment and management of epilepsy with eyelid myoclonia (EEM). The objective of this study was to determine areas of consensus among an international panel of experts for the management of EEM (formerly known as Jeavons syndrome).
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There was a strong consensus for valproic acid as the first-line treatment, with levetiracetam or lamotrigine as preferable alternatives for women of childbearing age. There was a moderate consensus that ethosuximide and clobazam are also efficacious. There was a strong consensus to avoid sodium channel-blocking medications, except for lamotrigine, as they may worsen seizure control. There was consensus that seizures typically persist into adulthood, with remission occurring in <50% of patients. There was less agreement about other areas of management, including dietary therapy, lens therapy, candidacy for driving, and outcome.
This international expert panel identified multiple areas of consensus regarding the optimal management of EEM. These areas of consensus may inform clinical practice to improve the management of EEM. 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Wirrell, Elaine C ; Andrade, Danielle M ; Choi, Hyunmi ; Trenité, Dorothée Kasteleijn-Nolst ; Jones, Hannah ; Knupp, Kelly G ; Mugar, Jon ; Nordli, Jr, Douglas R ; Riva, Antonella ; Stern, John M ; Striano, Pasquale ; Thiele, Elizabeth A ; Zawar, Ifrah</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c348t-5c03a1f5e7c66f1790442f2a8842881630ae65b654e2e1ec62e7f0da2ff50b2c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Caregivers</topic><topic>Disease management</topic><topic>Epilepsy</topic><topic>Etiracetam</topic><topic>Eyelid</topic><topic>Lamotrigine</topic><topic>Nutrition therapy</topic><topic>Patients</topic><topic>Remission</topic><topic>Seizures</topic><topic>Valproic acid</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Smith, Kelsey M</creatorcontrib><creatorcontrib>Wirrell, Elaine C</creatorcontrib><creatorcontrib>Andrade, Danielle M</creatorcontrib><creatorcontrib>Choi, Hyunmi</creatorcontrib><creatorcontrib>Trenité, Dorothée Kasteleijn-Nolst</creatorcontrib><creatorcontrib>Jones, Hannah</creatorcontrib><creatorcontrib>Knupp, Kelly G</creatorcontrib><creatorcontrib>Mugar, Jon</creatorcontrib><creatorcontrib>Nordli, Jr, Douglas R</creatorcontrib><creatorcontrib>Riva, Antonella</creatorcontrib><creatorcontrib>Stern, John M</creatorcontrib><creatorcontrib>Striano, Pasquale</creatorcontrib><creatorcontrib>Thiele, Elizabeth A</creatorcontrib><creatorcontrib>Zawar, Ifrah</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Epilepsia (Copenhagen)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Smith, Kelsey M</au><au>Wirrell, Elaine C</au><au>Andrade, Danielle M</au><au>Choi, Hyunmi</au><au>Trenité, Dorothée Kasteleijn-Nolst</au><au>Jones, Hannah</au><au>Knupp, Kelly G</au><au>Mugar, Jon</au><au>Nordli, Jr, Douglas R</au><au>Riva, Antonella</au><au>Stern, John M</au><au>Striano, Pasquale</au><au>Thiele, Elizabeth A</au><au>Zawar, Ifrah</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Management of epilepsy with eyelid myoclonia: Results of an international expert consensus panel</atitle><jtitle>Epilepsia (Copenhagen)</jtitle><addtitle>Epilepsia</addtitle><date>2023-09-01</date><risdate>2023</risdate><volume>64</volume><issue>9</issue><spage>2342</spage><epage>2350</epage><pages>2342-2350</pages><issn>0013-9580</issn><eissn>1528-1167</eissn><abstract>There are limited data about the treatment and management of epilepsy with eyelid myoclonia (EEM). 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An international steering committee was convened of physicians and patients/caregivers with expertise in EEM. This committee summarized the current literature and identified an international panel of experts (comprising 25 physicians and five patients/caregivers). This panel participated in a modified Delphi process, including three rounds of surveys to determine areas of consensus for the treatment, other areas of management, and prognosis for EEM.
There was a strong consensus for valproic acid as the first-line treatment, with levetiracetam or lamotrigine as preferable alternatives for women of childbearing age. There was a moderate consensus that ethosuximide and clobazam are also efficacious. There was a strong consensus to avoid sodium channel-blocking medications, except for lamotrigine, as they may worsen seizure control. There was consensus that seizures typically persist into adulthood, with remission occurring in <50% of patients. There was less agreement about other areas of management, including dietary therapy, lens therapy, candidacy for driving, and outcome.
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subjects | Caregivers Disease management Epilepsy Etiracetam Eyelid Lamotrigine Nutrition therapy Patients Remission Seizures Valproic acid |
title | Management of epilepsy with eyelid myoclonia: Results of an international expert consensus panel |
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