Jeavons Syndrome

Abdulla, Hafsa; Mirza, Mahwish; Osman, Shatha

doi:10.12816/0047610

Cited by 3 publications

(1 citation statement)

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“…Literature regarding the treatment of EEM supports the use of broad-spectrum ASMs, including valproic acid, lamotrigine, ethosuximide, levetiracetam, zonisamide, clobazam, and clonazepam. [5][6][7][11][12][13] There was a strong consensus for the use of valproic acid as first-line therapy, with lamotrigine and levetiracetam also determined to be efficacious and reasonable first-line alternatives to valproic acid for women of childbearing age. There was a moderate consensus for the use of ethosuximide or clobazam.…”

Section: Discussionmentioning

confidence: 99%

Management of epilepsy with eyelid myoclonia: Results of an international expert consensus panel

Smith,

Wirrell,

Andrade

et al. 2023

Epilepsia

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ObjectiveThere 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).MethodsAn 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.ResultsThere 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.SignificanceThis 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.

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