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Eyelid myoclonia with absences related to epileptic negative myoclonus Volume 23, issue 6, December 2021

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  • Eyelid myoclonia with absences related to epileptic negative myoclonus
  • Eyelid myoclonia with absences related to epileptic negative myoclonus

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  • Figure 1

  • Figure 2
Authors
1 Department of Pediatrics, St. Luke's International Hospital, 9-1 Akashi-cho, Chuo-ku, Tokyo, Japan
2 Division of Neurology, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo-ku, Saitama, Japan
3 Department of Pediatrics and Adolescent Medicine, Tokyo Medical University, 6-7-1, Nishi-shinjuku, Shinjuku-ku, Tokyo, Japan
4 Department of Pediatrics, Taipei Medical University-Shuang Ho Hospital, No. 291, Zhogzheng Rd., Zhonghe District, New Taipei City, Taiwan
5 Ogihara Clinic, 2-11-10, Egoda, Nakano-ku, Tokyo, Japan
* Correspondence: Atsuro Daida Department of Pediatrics, St. Luke's International Hospital, 9-1 Akashi-cho, Chuo-ku, Tokyo, 104-8560 Japan

Eyelid myoclonia with absences (EMA) is an epileptic syndrome characterised by eyelid myoclonia with or without absences, eye closure-induced paroxysms, and photosensitivity. The relationship between EMA and epileptic negative myoclonus has not previously been reported. Herein, we describe a case of a 10-year-old girl who presented with eyelid myoclonia, eye closure-induced paroxysms, and photosensitivity, which was compatible with the diagnosis of EMA. Ictal EEG depicted an eye closure-induced diffuse 3.0-4.5-Hz polyspike-and-wave complex, which was accompanied by eye fluttering (eyelid myoclonia). EMG disclosed a brief interruption (60-140 mseconds) of tonic contraction of the orbicularis oculi muscle, which was associated with the polyspike-and-wave complex on EEG. The findings led to the diagnosis of epileptic negative myoclonus. Eye closure-induced eyelid epileptic negative myoclonus, demonstrated in this patient, may be an atypical seizure type of EMA that represents an intermediate between eyelid myoclonia and epileptic negative myoclonus.