Epileptic Disorders

, Volume 13, Issue 3, pp 308–312 | Cite as

Perioral myoclonia with absences and myoclonic status aggravated by oxcarbazepine

  • Pascal Vrielynck
  • Nino Rostomashvili
  • Elodie Degroote
  • Sophie Ghariani
  • Kenou van Rijckevorsel
Clinical Commentary with Video Sequences


Perioral myoclonia with absences belongs to the “idiopathic generalised epilepsy syndromes in development”, currently not yet cited in the ILAE classification. This epilepsy syndrome is associated with a seizure type that appears to be specific. Here, we report polygraphic recordings of this seizure type in a young boy, previously misdiagnosed with focal epilepsy. EEG and clinical features were useful to differentiate diagnosis of his seizures from other absence or myoclonic seizures. Interestingly, some seizures were associated with neck myoclonia. Home video recording of myoclonic status aggravated by inappropriate treatment is also presented.

Key words

perioral myoclonia with absences idiopathic generalised epilepsy myoclonic status neck myoclonia oxcarbazepine AEDs aggravation 


  1. Baykan B, Noachtar S. Perioral myoclonia with absences: an overlooked and misdiagnosed generalized seizure type. Epilepsy Behav 2005; 6:460–462.PubMedCrossRefGoogle Scholar
  2. Berg A, Berkovic S, Brodie M, et al. Revised terminology and concepts for organization of the epilepsies: Report of the Commission on Classification and Terminology. ILAE website. July 2009.
  3. Bilgiç B, Baykan B, Gürses C, Gökyigit A. Perioral myoclonia with absence seizures: a rare epileptic syndrome. Epileptic Disord 2001; 3:23–27.PubMedGoogle Scholar
  4. Bureau M, Tassinari CA. Epilepsy with myoclonic absences. Brain Dev 2005; 27:178–184.PubMedCrossRefGoogle Scholar
  5. Capovilla G, Rubboli G, Beccaria F, et al. A clinical spectrum of the myoclonic manifestations associated with typical absences in childhood absence epilepsy. A videopolygraphic study. Epileptic Disord 2001; 3:57–62.PubMedGoogle Scholar
  6. Caraballo RH, Fontana E, Darra F, et al. Astudy of 63 cases with eyelid myoclonia with or without absences: type of seizure or an epileptic syndrome? Seizure 2009; 18: 440–445.PubMedCrossRefGoogle Scholar
  7. d’Orsi G, Demaio V, Trivisano M, Pascarella MG, Specchio LM. Ictal video-polygraphic features of perioral myoclonia with absences. Epilepsy Behav 2011; 21:314–317.PubMedCrossRefGoogle Scholar
  8. Gelisse P, Genton P, Kuate C, et al. Worsening of seizures by oxcarbazepine in juvenile idiopathic generalized epilepsies. Epilepsia 2004; 45:1282–1286.PubMedCrossRefGoogle Scholar
  9. Mayer TA, Schroeder F, May TW, Wolf PT. Perioral reflex myoclonias: a controlled study in patients with JME and focal epilepsies. Epilepsia 2006; 47:1059–1067.PubMedCrossRefGoogle Scholar
  10. Panayiotopoulos CP, Ferrie CD, Giannakodimos SE, Robinson RO. Perioral myoclonia with absences: a new syndrome. In: Wolf P. Epileptic Seizures and Syndromes. London: John Libbey & Company Ltd Publishers, 1994: 143–153.Google Scholar
  11. Rubboli G, Gardella E, Capovilla G. Idiopathic generalized epilepsy (IGE) syndromes in development: IGE with absences of early childhood, IGE with phantom absences, and perioral myoclonia with absences. Epilepsia 2009; 50:24–28.PubMedCrossRefGoogle Scholar
  12. Usui N, Kotagal P, Matsumoto R, et al. Focal semiologic and electroencephalographic features in patients with juvenile myoclonic epilepsy. Epilepsia 2005; 46:1668–1676.PubMedCrossRefGoogle Scholar
  13. Yang Z, Liu X, Qin J, Jiang Y. Neck myoclonia with absence seizures: report of 3 cases. J Child Neurol 2009; 24:1026–1029.PubMedCrossRefGoogle Scholar

Copyright information

© John Libbey Eurotext and Springer 2011

Authors and Affiliations

  • Pascal Vrielynck
    • 1
    • 3
  • Nino Rostomashvili
    • 1
  • Elodie Degroote
    • 2
  • Sophie Ghariani
    • 1
  • Kenou van Rijckevorsel
    • 1
  1. 1.Centre Neurologique William Lennox, Reference Centre for Refractory EpilepsyUniversité Catholique de LouvainOttigniesBelgium
  2. 2.Centre Hospitalier Régionalservice de pédiatrieMonsBelgium
  3. 3.Centre Neurologique William LennoxOttigniesBelgium

Personalised recommendations