TY - JOUR
T1 - Management of epilepsy with eyelid myoclonia
T2 - Results of an international expert consensus panel
AU - Smith, Kelsey M.
AU - Wirrell, Elaine C.
AU - Andrade, Danielle M.
AU - Choi, Hyunmi
AU - Trenité, Dorothée Kasteleijn Nolst
AU - Jones, Hannah
AU - Knupp, Kelly G.
AU - Mugar, Jon
AU - Nordli, Douglas R.
AU - Riva, Antonella
AU - Stern, John M.
AU - Striano, Pasquale
AU - Thiele, Elizabeth A.
AU - Zawar, Ifrah
N1 - Publisher Copyright:
© 2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.
PY - 2023/9
Y1 - 2023/9
N2 - Objective: 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). Methods: 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. Results: 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. Significance: 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.
AB - Objective: 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). Methods: 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. Results: 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. Significance: 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.
KW - drug-resistant epilepsy
KW - epilepsy with eyelid myoclonia
KW - eyelid myoclonia
KW - genetic generalized epilepsy
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U2 - 10.1111/epi.17682
DO - 10.1111/epi.17682
M3 - Article
C2 - 37326215
AN - SCOPUS:85162875734
SN - 0013-9580
VL - 64
SP - 2342
EP - 2350
JO - Epilepsia
JF - Epilepsia
IS - 9
ER -