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Epilepsy with eyelid myoclonia (EM) or Jeavons syndrome (JS) is an epileptic syndrome related to the spectrum of genetic generalized epilepsies (GGE). We report two untreated children on which EEGs were performed several hours after a generalized tonic-clonic seizure (GTCS). These showed a unilateral, nearly continuous posterior slowing. This slow-wave activity was associated with contralateral epileptiform activity in one case, while in the second case, it was associated with an ipsilateral activity. However, in the latter child, a few months later an independent focus on the contralateral side was observed. A diagnosis of focal occipital lobe epilepsy was proposed in both cases, and one child underwent a left occipital lobectomy at 3.5 years of age. Despite surgery, absences with EM persisted in this child, and a marked photosensitivity to photic stimulation was observed two years later. The focal slow wave activity of one occipital lobe several hours after a GTCS in these two subjects was in favor of a focal onset preceding the generalization. The EEG evidence for independent left and right posterior focus in these two cases, the persistence of EM, and the development of a marked photosensitivity to photic stimulation in the child who underwent an occipital lobectomy, allow us to suggest that JS is associated with a network of bi-occipital hyperexcitability that rapidly engages bilaterally to produce generalized seizures., Competing Interests: Declaration of competing interest Dr. Gelisse received support for teaching programs from Sanofi, UCB, EISAI and Psicofarma. He received a research grant from Janssen-Cilag. He worked as a consultant for Eisai-France in 2011. Dr. Crespel received support for teaching programs from Sanofi, UCB, GSK, Shire and is an advisory board member for Eisai-France. Dr. Genton received speaker invitations and honoraria from Sanofi-Aventis, Novartis, GSK, Pfizer, Janssen-Cilag, UCB, Eisai, and Actelion. He received support for teaching programs from Sanofi-Aventis and UCB. Dr Nilo received support as a consultant from Eisai-Italy. Dr. Gallegos, Dr Macorig report no conflicts of interest. All co-authors have been substantially involved in the study and preparation of the manuscript. No undisclosed persons have had a primary role in the study or manuscript preparation. All co-authors have approved the submitted version of the paper and accept responsibility for its content., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Eyelid myoclonia with absences is classified as a unique type of generalized seizure. Its pathogenesis is proposed to involve the functional abnormalities in cortical–subcortical networks. Here, we describe the case of a 7-year-old boy who had eyelid myoclonia with absences, along with focal motor seizures. Video-EEG monitoring demonstrated eyelid myoclonia associated with 4- to 5-Hz generalized polyspike–waves preceded by focal frontal discharges. Interictal EEG showed focal epileptiform discharges over the frontal regions. Our case suggests an important role of the frontal lobe in the generation of eyelid myoclonia with absences.

Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare.

Objective: In this study, our aim was to demonstrate the effect of sleep deprivation, short sleep, and awakening on photoparoxysmal responses (PPR) and eyelid myoclonia (EM) in patients with Epilepsy with Eyelid Myoclonia (E-EM)., Methods: E-EM patients with at least 1 year of follow-up in our clinic were included in the study. Video EEG(v-EEG) analyses were divided into three periods of wakefulness, sleep, and awakening. The PPR and onset of EMs were investigated., Results: 32 patients met the study criteria, of which 56.3% (n = 18) were male. The mean age at disease onset was 7.7 ± 4.1 years. The mean age at EEG recording was 12.4 ± 4.0 years. EM was observed only on awakening in 78.1% of patients (n = 22), of which it was seen only during intermittent photic stimulation (IPS) in 43.7% (n = 14). Eye closure (EC) sensitivity was detected in all patients. The proportion of patients with a PPR was significantly higher on awakening than before sleep (p = 0.01)., Conclusions: This study showed that EM is most prominent and sometimes can only be detected in the awakening period in E-EM. In order to detect E-EM, v-EEG recordings including both pre-sleep and post-sleep wakefulness periods should be recorded, with intermittent photic stimulation performed in both periods., Competing Interests: Declaration of Competing Interest None of the authors have potential conflicts of interest to be disclosed., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Purpose: Eye closure sensitivity (ECS) has been described as a reflex trait in juvenile myoclonic epilepsy (JME). However, there is no consensus regarding its significance on prognosis. The aim of this study is to clarify the long-term impact of ECS documented by a clinical interview and a video-EEG neuropsychological protocol (VNPP) in a series of 133 JME patients., Methods: Data from 22 JME patients with ECS confirmed by a VNPP (Group 1) were compared with those of 20 JME patients without any reflex traits (Group 2). They were followed for a mean of 8.21 years (SD=±5.044). The frequency of seizures was assessed using a diary. Except for photosensitivity (PS), any other reflex traits occurrence, drugs/alcohol abuse intake, noncompliance, and Jeavons syndrome, were considered exclusion criteria., Results: Group 1 had a lower age at epilepsy onset (p = 0.028), higher incidence of febrile seizures (13.6%), and familial history of epilepsy (p = 0.023). Only 18.2% had self-perception of eyelid myoclonia (EM) (kappa coefficient = 0.193), which persisted in 77.3% of patients. Limb myoclonia, tonic-clonic seizures (TCS) and/or myoclonic-tonic-clonic seizures (MTCS), as well as absences were more frequent (p = 0.015; p = 0.013; p = 0.011, respectively) in Group 1. PS did not influenced frequency of EM (p = 1.0), absences (p = 0.648), or TCS/MTCS (p = 0.934). Psychiatric comorbidities were not different between groups., Conclusions: ECS is related to a worse outcome regarding control of all seizure types, persistence of EM, and higher frequency of limb myoclonia, as well as the total number of TCS and/or MTCS., (Copyright © 2018 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Purpose: Eyelid myoclonia (EM) is considered a seizure type and has been described in several epileptic conditions. Previous studies reported that EM are precipitated only by slow eye closure, but little is known about the characteristics of blinking in patients with EM seizures and differences in precipitation of EM by different kinds of eye closure. We analyzed by video-EEG the characteristics of blinking and eye closure in these patients., Method: Twenty patients with EM had a video-EEG protocol with eyelid sensors. Semiology and rate of blinking and EM were analyzed. Statistical analysis was performed and p-values <0.05 were considered significant., Results: Seventeen cases (mean age 20.7, range 3-35) were women, 10 had EM as the main seizure type and between the others, all, but one, had criteria to Juvenile Myoclonic Epilepsy. All patients presented EM, 18 spontaneously during awake, 10 on eye closure and one only during intermittent photic stimulation. EM assumed the form of flicker, flutter or jerk, accompanied by generalized discharges, spiky posterior alpha, theta rhythm or absence of any EEG abnormality. Analysis of the characteristics of blinking had no statistics differences between patients and healthy subjects. The rate of blinks and EM increased during speech and decreased during reading. EM never occurred during blinking or in the dark., Conclusions: Despite normal physiology of blinking, EM can manifest as jerk, flicker or flutter, with or without EEG abnormalities and independently of IPS, suggesting that eye closure sensitivity seems to include both, a motor and a visual component., (Copyright © 2014 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

We report on a 25 year old female patient who had a diagnosis of Jeavons syndrome since her childhood. Although valproate led to seizure freedom, she developed persistent reproductive endocrine disorders attributed to this drug. The withdrawal of valproate in parallel with an initiation of levetiracetam monotherapy resulted in a maintenance of clinical remission and a resolution of these abnormalities. This case together with relevant literature data supports the view that the use of levetiracetam might be of benefit for female patients with Jeavons syndrome., (Copyright © 2014 Elsevier B.V. All rights reserved.)