A comprehensive narrative review of epilepsy with eyelid myoclonia

Smith, Kelsey M.; Wirrell, Elaine; Andrade, Danielle M.; Choi, Hyunmi; Trenité, Dorothee Kasteleijn‐Nolst; Knupp, Kelly G.; Nordli, Douglas R.; Riva, Antonella; Stern, John M.; Striano, Pasquale; Thiele, Elizabeth A.; Zawar, Ifrah

doi:10.1016/j.eplepsyres.2023.107147

Cited by 3 publications

(4 citation statements)

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“…8) Most of the patients with EMA experienced more than one seizure type: absence, GTCS, myoclonic seizures, and other generalized seizure-like tonic and atonic. 3,4,7,8,14) To conclude, this report recommends that VNS is an effective option for patients with refractory epilepsy requiring VPA discontinuation. Women with EMA taking VPA must be aware of PCOS and monitor their menstrual cycles.…”

Section: Discussionmentioning

confidence: 97%

“…In cases of epilepsy, higher-level stimulation is more effective, 11,12) and the population-level target output current of VNS is 1.61 mA. 13) Eyelid myoclonia is usually associated with several seizure types: 3,4,7,8,14) absence seizures (90.6%), GTCS (78.1%), and myoclonic seizures (43.7%). 8) Several reports suggest that VNS therapy may be effective in treating these seizures.…”

Section: Discussionmentioning

confidence: 99%

“…4,6) Since the first description of EMA by Jeavons in 1977, 1) case reports and small studies have been published, [1][2][3][4][5]7,8) but the prognostic and clinical features remain partially uncertain. [1][2][3][4][5][6][7][8]14) A recent study has reported electroclinical endophenotypes and long-term seizure outcomes in a large cohort of patients with EMA. 6) We speculate that PCOS, which can occur in adulthood and is not a direct symptom of EMA itself, has received little attention because EMA is a disease of childhood onset.…”

Section: Discussionmentioning

confidence: 99%

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Epilepsy with Eyelid Myoclonia Complicated by Polycystic Ovary Syndrome: Valproate Withdrawal, Vagus Nerve Stimulation, and Seizure Reduction

TATEBAYASHI,

TAKUMI,

MATSUMORI

et al. 2023

NMC Case Report Journal

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Epilepsy with eyelid myoclonia is a childhood-onset generalized epilepsy, which is more common in women. Over 90% of the patients continue antiseizure medications, especially valproate, and more than 60% of cases are refractory. The efficacy of vagus nerve stimulation in treating eyelid myoclonia is still unknown. Polycystic ovary syndrome is highly prevalent in women with epilepsy receiving valproate; nevertheless, no reports on the complication of polycystic ovary syndrome in women with epilepsy with eyelid myoclonia were found. In this report, a case of a woman with epilepsy with eyelid myoclonia who developed polycystic ovary syndrome while receiving valproate and underwent vagus nerve stimulation is described. A 26-year-old female patient has been administered valproate since the occurrence of generalized seizures at the age of 12 years and then developed polycystic ovary syndrome. When the dose of valproate was reduced as an adult, her epilepsy became intractable. Information from her mother led to a video electroencephalography re-evaluation, and she was finally diagnosed 15 years after onset. The patient underwent vagus nerve stimulation. In a short-term followup, she achieved >50% seizure reduction at low output currents of <1.00 mA. Polycystic ovary syndrome was cured 15 months after valproate withdrawal. There are three key points presented in this case: Vagus nerve stimulation therapy was useful for treating epilepsy with eyelid myoclonia with absence. Women with epilepsy with eyelid myoclonia taking valproate must be aware of the risk of polycystic ovary syndrome and monitor their menstrual cycles. Information from the family, such as home videos, helped with the diagnosis.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Epilepsy with Eyelid Myoclonia Complicated by Polycystic Ovary Syndrome: Valproate Withdrawal, Vagus Nerve Stimulation, and Seizure Reduction

TATEBAYASHI,

TAKUMI,

MATSUMORI

et al. 2023

NMC Case Report Journal

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“…Epilepsy with eyelid myoclonia (EEM) is an underrecognized genetic generalized epilepsy syndrome with childhood onset and a female predominance 1 . The syndrome consists of the following triad: (1) eyelid myoclonia with or without absence seizures, (2) eye closure‐induced seizures or electroencephalographic (EEG) paroxysms, and (3) photosensitivity 2 .…”

Section: Introductionmentioning

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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