Skip to main content
SpringerLink
Log in

Myoclonic Status Epilepticus

  • Chapter
  • First Online:
Status Epilepticus

Part of the book series: Current Clinical Neurology ((CCNEU))

Abstract

Myoclonic status epilepticus (MSE) is an epileptic condition in which myoclonic seizures persist for more than 30 min. Based on etiology, MSE has been classified by Gastaut into primary, secondary, and symptomatic forms of MSE. The clinical and electrographic characteristics of different syndromes that can present with MSE will be discussed in this chapter, as well as implications and treatment of this complex condition.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Shibasaki H, Hallett M. Electrophysiological studies of myoclonus. Muscle Nerve. 2005;31(2):157–74.

    Article  PubMed  Google Scholar 

  2. Gastaut H. Classification of status epilepticus. Adv Neurol. 1983;34:15–35.

    CAS  PubMed  Google Scholar 

  3. Gerard EE, Hirsch LJ. Generalized myoclonic status epilepticus. In: Panayiotopoulos CP, editor. Atlas of epilepsies. London: Springer; 2010. p. 523–31.

    Chapter  Google Scholar 

  4. Brown P, Ridding MC, Werhahn KJ, Rothwell JC, Marsden CD. Abnormalities of the balance between inhibition and excitation in the motor cortex of patients with cortical myoclonus. Brain. 1996;119(Pt 1):309–17.

    Article  PubMed  Google Scholar 

  5. Shibasaki H, Kuroiwa Y. Electroencephalographic correlates of myoclonus. Electroencephalogr Clin Neurophysiol. 1975;39(5):455–63.

    Article  CAS  PubMed  Google Scholar 

  6. Guerrini R, Bonanni P, Rothwell J, Hallett M. Myoclonus and epilepsy. In: Guerrini R, Aicardi J, Andermann F, Hallett M, editors. Epilepsy and movement disorders. Cambridge: Cambridge University Press; 2002. p. 165–210.

    Google Scholar 

  7. Wilkins DE, Hallett M, Erba G. Primary generalised epileptic myoclonus: a frequent manifestation of minipolymyoclonus of central origin. J Neurol Neurosurg Psychiatry. 1985;48(6):506–16.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Tassinari CA, Rubboli G, Shibasaki H. Neurophysiology of positive and negative myoclonus. Electroencephalogr Clin Neurophysiol. 1998;107(3):181–95.

    Article  CAS  PubMed  Google Scholar 

  9. Guerrini R, Dravet C, Genton P, Bureau M, Roger J, Rubboli G, et al. Epileptic negative myoclonus. Neurology. 1993;43(6):1078–83.

    Article  CAS  PubMed  Google Scholar 

  10. Tassinari CA, Rubboli G, Parmeggiani L, Valzania F, Plasmati R, Riguzzi P, et al. Epileptic negative myoclonus. Adv Neurol. 1995;67:181–97.

    CAS  PubMed  Google Scholar 

  11. Tassinari C, Michelucci R, Rubboli G. Negative epileptic myoclonus. Mov Disord. 1990;5(Suppl 1):44.

    Google Scholar 

  12. Berg AT, Berkovic SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE commission on classification and terminology, 2005–2009. Epilepsia. 2010;51(4):676–85.

    Article  PubMed  Google Scholar 

  13. Dziewas R, Kellinghaus C, Lüdemann P. Nonconvulsion status epilepticus in patients with juvenile myoclonic epilepsy: types and frequencies. Seizure. 2002;11(5):335–9.

    Article  PubMed  Google Scholar 

  14. Thomas P, Valton L, Genton P. Absence and myoclonic status epilepticus precipitated by antiepileptic drugs in idiopathic generalized epilepsy. Brain. 2006;129(Pt 5):1281–92.

    Article  PubMed  Google Scholar 

  15. Panayiotopoulos CP, Obeid T, Tahan AR. Juvenile myoclonic epilepsy: a 5-year prospective study. Epilepsia. 1994;35(2):285–96.

    Article  CAS  PubMed  Google Scholar 

  16. Larch J, Unterberger I, Bauer G, Reichsoellner J, Kuchukhidze G, Trinka E. Myoclonic status epilepticus in juvenile myoclonic epilepsy. Epileptic Disord. 2009;11(4):309–14.

    PubMed  Google Scholar 

  17. Salas Puig J, Tunon A, Vidal JA, Mateos V, Guisasola LM, Lahoz CH. Janz’s juvenile myoclonic epilepsy: a little-known frequent syndrome. A study of 85 patients. Med Clin (Barc). 1994;103(18):684–9.

    Google Scholar 

  18. Asconape J, Penry JK. Some clinical and EEG aspects of benign juvenile myoclonic epilepsy. Epilepsia. 1984;25(1):108–14.

    Article  CAS  PubMed  Google Scholar 

  19. Fanella M, Egeo G, Fattouch J, Casciato S, Lapenta L, Morano A, et al. Oxcarbazepine-induced myoclonic status epilepticus in juvenile myoclonic epilepsy. Epileptic Disord. 2013;15(2):181–7.

    PubMed  Google Scholar 

  20. Usui N, Kotagal P, Matsumoto R, Kellinghaus C, Luders HO. Focal semiologic and electroencephalographic features in patients with juvenile myoclonic epilepsy. Epilepsia. 2005;46(10):1668–76.

    Article  PubMed  Google Scholar 

  21. Montalenti E, Imperiale D, Rovera A, Bergamasco B, Benna P. Clinical features, EEG findings and diagnostic pitfalls in juvenile myoclonic epilepsy: a series of 63 patients. J Neurol Sci. 2001;184(1):65–70.

    Article  CAS  PubMed  Google Scholar 

  22. Benbadis SR, Lin K. Errors in EEG interpretation and misdiagnosis of epilepsy. Which EEG patterns are overread? Eur Neurol. 2008;59(5):267–71.

    Article  PubMed  Google Scholar 

  23. Gélisse P, Crespel A. Mixed myoclonic-absence status epilepticus in juvenile myoclonic epilepsy. Epileptic Disord. 2015;17(1):95–6.

    PubMed  Google Scholar 

  24. Liu L, Zheng T, Morris MJ, Wallengren C, Clarke AL, Reid CA, et al. The mechanism of carbamazepine aggravation of absence seizures. J Pharmacol Exp Ther. 2006;319(2):790–8.

    Article  CAS  PubMed  Google Scholar 

  25. Zheng T, Clarke AL, Morris MJ, Reid CA, Petrou S, O’Brien TJ. Oxcarbazepine, not its active metabolite, potentiates GABAA activation and aggravates absence seizures. Epilepsia. 2009;50(1):83–7.

    Article  CAS  PubMed  Google Scholar 

  26. Biraben A, Allain H, Scarabin JM, Schuck S, Edan G. Exacerbation of juvenile myoclonic epilepsy with lamotrigine. Neurology. 2000;55(11):1758.

    Article  CAS  PubMed  Google Scholar 

  27. Liu Z, Vergnes M, Depaulis A, Marescaux C. Involvement of intrathalamic GABAB neurotransmission in the control of absence seizures in the rat. Neuroscience. 1992;48(1):87–93.

    Article  CAS  PubMed  Google Scholar 

  28. Crespel A, Genton P, Berramdane M, Coubes P, Monicard C, Baldy-Moulinier M, et al. Lamotrigine associated with exacerbation or de novo myoclonus in idiopathic generalized epilepsies. Neurology. 2005;65(5):762–4.

    Article  CAS  PubMed  Google Scholar 

  29. Capovilla G, Striano P, Gambardella A, Beccaria F, Hirsch E, Casellato S, et al. Eyelid fluttering, typical EEG pattern, and impaired intellectual function: a homogeneous epileptic condition among the patients presenting with eyelid myoclonia. Epilepsia. 2009;50(6):1536–41.

    Article  PubMed  Google Scholar 

  30. Millichap JJ, Koh S, Laux LC, Nordli DR Jr. Child Neurology: Dravet syndrome: when to suspect the diagnosis. Neurology. 2009;73(13):e59–62.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Claes L, Del-Favero J, Ceulemans B, Lagae L, Van Broeckhoven C, De Jonghe P. De novo mutations in the sodium-channel gene SCN1A cause severe myoclonic epilepsy of infancy. Am J Hum Genet. 2001;68(6):1327–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Dravet C, Oguni H. Dravet syndrome (severe myoclonic epilepsy in infancy). Handb Clin Neurol. 2013;111:627–33.

    Article  PubMed  Google Scholar 

  33. Fontana E, Dalla Bernardina B, Sgrò V, Mastella L, Filippi A, Torniero C, et al. [Severe myoclonic epilepsy in infancy (SMEI) and/or Dravet syndrome: longitudinal electroclinical study of 53 subjects.] Epilessia mioclonica severa (EMS) e/o sindrome di Dravet: studio elettroclinico longitudinale di 53 soggetti. Boll Lega It Epil. 2004;125–6:337–40 (Article in Italian).

    Google Scholar 

  34. Akiyama M, Kobayashi K, Yoshinaga H, Ohtsuka Y. A long-term follow-up study of Dravet syndrome up to adulthood. Epilepsia. 2010;51(6):1043–52.

    Article  PubMed  Google Scholar 

  35. Yakoub M, Dulac O, Jambaque I, Chiron C, Plouin P. Early diagnosis of severe myoclonic epilepsy in infancy. Brain Dev. 1992;14(5):299–303.

    Article  CAS  PubMed  Google Scholar 

  36. Dalla Bernardina B, Trevisan C, Bondavalli S, Colamaria V, Bureau M, Roger J, Dravet C. Une forme particulière d’epilepsie myoclonique chez des enfants porteurs d’encephalopathie fixée. Boll Lega It Epil. 1980;29–30:183–6 (Article in French).

    Google Scholar 

  37. Dulac O, N’Guyen T. The Lennox-Gastaut syndrome. Epilepsia. 1993;34(Suppl 7):S7–17.

    Article  PubMed  Google Scholar 

  38. Guerrini R, Belmonte A, Parmeggiani L, Perucca E. Myoclonic status epilepticus following high-dosage lamotrigine therapy. Brain Dev. 1999;21(6):420–4.

    Article  CAS  PubMed  Google Scholar 

  39. Dulac O, Plouin P, Shewmon A. Myoclonus and epilepsy in childhood: 1996 Royaumont meeting. Epilepsy Res. 1998;30(2):91–106.

    Article  CAS  PubMed  Google Scholar 

  40. Kaminska A, Ickowicz A, Plouin P, Bru MF, Dellatolas G, Dulac O. Delineation of cryptogenic Lennox-Gastaut syndrome and myoclonic astatic epilepsy using multiple correspondence analysis. Epilepsy Res. 1999;36(1):15–29.

    Article  CAS  PubMed  Google Scholar 

  41. Oguni H, Tanaka T, Hayashi K, Funatsuka M, Sakauchi M, Shirakawa S, et al. Treatment and long-term prognosis of myoclonic-astatic epilepsy of early childhood. Neuropediatrics. 2002;33(3):122–32.

    Article  CAS  PubMed  Google Scholar 

  42. Doose H, Gerken H, Leonhardt R, Volzke E, Volz C. Centrencephalic myoclonic-astatic petit mal. Clinical and genetic investigation. Neuropadiatrie. 1970;2(1):59–78.

    Article  CAS  PubMed  Google Scholar 

  43. Lortie A, Chiron C, Mumford J, Dulac O. The potential for increasing seizure frequency, relapse, and appearance of new seizure types with vigabatrin. Neurology. 1993;43(11 Suppl 5):S24–7.

    CAS  PubMed  Google Scholar 

  44. Tassinari CA, Lyagoubi S, Santos V, Gambarelli G, Roger J, Dravet C, et al. Study on spike and wave discharges in man. II. Clinical and electroencephalographic aspects of myoclonic absences. Rev Neurol (Paris). 1969;121(3):379–83.

    Google Scholar 

  45. Mizuguchi M, Tsukamoto K, Suzuki Y, Nakagome Y. Myoclonic epilepsy and a maternally derived deletion of 15pter → q13. Clin Genet. 1994;45(1):44–7.

    Article  CAS  PubMed  Google Scholar 

  46. Sgro V, Riva E, Canevini MP, Colamaria V, Rottoli A, Minotti L, et al. 4p(-) syndrome: a chromosomal disorder associated with a particular EEG pattern. Epilepsia. 1995;36(12):1206–14.

    Article  CAS  PubMed  Google Scholar 

  47. Dalla Bernardina B, Fontana E, Darra F. Myoclonic status in non-progressive encephalopathies. In: Bureau M, Genton P, Dravet C, Delgado-Escueta AV, Tassinari CA, Thomas P, Wolf P, editors. Epileptic syndromes in infancy, childhood and adolescence, 5th ed. Montrouge, France: John Libbey Eurotext; 2012. p. 431–40.

    Google Scholar 

  48. Caraballo RH, Cersosimo RO, Espeche A, Arroyo HA, Fejerman N. Myoclonic status in nonprogressive encephalopathies: study of 29 cases. Epilepsia. 2007;48(1):107–13.

    Article  PubMed  Google Scholar 

  49. Thomas JE, Reagan TJ, Klass DW. Epilepsia partialis continua. A review of 32 cases. Arch Neurol. 1977;34(5):266–75.

    Article  CAS  PubMed  Google Scholar 

  50. Obeso JA, Rothwell JC, Marsden CD. The spectrum of cortical myoclonus. From focal reflex jerks to spontaneous motor epilepsy. Brain. 1985;108(Pt 1):193–224.

    Article  PubMed  Google Scholar 

  51. Cockerell OC, Rothwell J, Thompson PD, Marsden CD, Shorvon SD. Clinical and physiological features of epilepsia partialis continua. Cases ascertained in the UK. Brain. 1996;119(Pt 2):393–407.

    Article  PubMed  Google Scholar 

  52. Shorvon S, Baulac M, Cross H, Trinka E, Walker M. Task force on status epilepticus of the ILAE commission for European affairs. The drug treatment of status epilepticus in Europe: consensus document from a workshop at the first London colloquium on status epilepticus. Epilepsia. 2008;49(7):1277–85.

    PubMed  Google Scholar 

  53. Pereira LS, Muller VT, da Mota Gomes M, Rotenberg A, Fregni F. Safety of repetitive transcranial magnetic stimulation in patients with epilepsy: a systematic review. Epilepsy Behav. 2016;57(Pt A):167–76.

    Google Scholar 

  54. Binelli S, Agazzi P, Canafoglia L, Scaioli V, Panzica F, Visani E, et al. Myoclonus in Creutzfeldt-Jakob disease: polygraphic and video-electroencephalography assessment of 109 patients. Mov Disord. 2010;25(16):2818–27.

    Article  PubMed  Google Scholar 

  55. Shibasaki H. Neurophysiological classification of myoclonus. Neurophysiol Clin. 2006;36(5–6):267–9.

    Article  CAS  PubMed  Google Scholar 

  56. Kalviainen R. Progressive myoclonus epilepsies. Semin Neurol. 2015;35(3):293–9.

    Article  PubMed  Google Scholar 

  57. Frei KP, Schiffmann R. Myoclonus in Gaucher disease. Adv Neurol. 2002;89:41–8.

    PubMed  Google Scholar 

  58. Miyahara A, Saito Y, Sugai K, Nakagawa E, Sakuma H, Komaki H, et al. Reassessment of phenytoin for treatment of late stage progressive myoclonus epilepsy complicated with status epilepticus. Epilepsy Res. 2009;84(2–3):201–9.

    Article  CAS  PubMed  Google Scholar 

  59. Vaca GF, Lenz T, Knight EM, Tuxhorn I. Gaucher disease: successful treatment of myoclonic status epilepticus with levetiracetam. Epileptic Disord. 2012;14(2):155–8.

    PubMed  Google Scholar 

  60. Eldridge R, Iivanainen M, Stern R, Koerber T, Wilder BJ. “Baltic” myoclonus epilepsy: hereditary disorder of childhood made worse by phenytoin. Lancet. 1983;2(8354):838–42.

    Article  CAS  PubMed  Google Scholar 

  61. Knake S, Klein KM, Hattemer K, Wellek A, Oertel WH, Hamer HM, et al. Pregabalin-induced generalized myoclonic status epilepticus in patients with chronic pain. Epilepsy Behav. 2007;11(3):471–3.

    Article  CAS  PubMed  Google Scholar 

  62. Vollmar C, Noachtar S. Tiagabine-induced myoclonic status epilepticus in a nonepileptic patient. Neurology. 2007;68(4):310.

    Article  PubMed  Google Scholar 

  63. Vartanian MG, Radulovic LL, Kinsora JJ, Serpa KA, Vergnes M, Bertram E, et al. Activity profile of pregabalin in rodent models of epilepsy and ataxia. Epilepsy Res. 2006;68(3):189–205.

    Article  CAS  PubMed  Google Scholar 

  64. Grill MF, Maganti RK. Neurotoxic effects associated with antibiotic use: management considerations. Br J Clin Pharmacol. 2011;72(3):381–93.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  65. Badhwar A, Berkovic SF, Dowling JP, Gonzales M, Narayanan S, Brodtmann A, et al. Action myoclonus-renal failure syndrome: characterization of a unique cerebro-renal disorder. Brain. 2004;127(Pt 10):2173–82.

    Article  PubMed  Google Scholar 

  66. Perandones C, Micheli FE, Pellene LA, Bayly MA, Berkovic SF, Dibbens LM. A case of severe hearing loss in action myoclonus renal failure syndrome resulting from mutation in SCARB2. Mov Disord. 2012;27(9):1200–1.

    Article  PubMed  Google Scholar 

  67. Rubboli G, Franceschetti S, Berkovic SF, Canafoglia L, Gambardella A, Dibbens LM, et al. Clinical and neurophysiologic features of progressive myoclonus epilepsy without renal failure caused by SCARB2 mutations. Epilepsia. 2011;52(12):2356–63.

    Article  CAS  PubMed  Google Scholar 

  68. Pezzella M, Yeghiazaryan NS, Veggiotti P, Bettinelli A, Giudizioso G, Zara F, et al. Galloway-Mowat syndrome: an early-onset progressive encephalopathy with intractable epilepsy associated to renal impairment. Two novel cases and review of literature. Seizure. 2010;19(2):132–5.

    Article  PubMed  Google Scholar 

  69. Ohbo Y, Fukuzako H, Takeuchi K, Takigawa M. Argyria and convulsive seizures caused by ingestion of silver in a patient with schizophrenia. Psychiatry Clin Neurosci. 1996;50(2):89–90.

    Article  CAS  PubMed  Google Scholar 

  70. Iwasaki S, Yoshimura A, Ideura T, Koshikawa S, Sudo M. Elimination study of silver in a hemodialyzed burn patient treated with silver sulfadiazine cream. Am J Kidney Dis. 1997;30(2):287–90.

    Article  CAS  PubMed  Google Scholar 

  71. Mirsattari SM, Hammond RR, Sharpe MD, Leung FY, Young GB. Myoclonic status epilepticus following repeated oral ingestion of colloidal silver. Neurology. 2004;62(8):1408–10.

    Article  CAS  PubMed  Google Scholar 

  72. Gyori J, Kiss T, Shcherbatko AD, Belan PV, Tepikin AV, Osipenko ON, et al. Effect of Ag+ on membrane permeability of perfused Helix pomatia neurons. J Physiol. 1991;442:1–13.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  73. Hero B, Schleiermacher G. Update on pediatric opsoclonus myoclonus syndrome. Neuropediatrics. 2013;44(6):324–9.

    Article  PubMed  Google Scholar 

  74. Blaes F, Pike MG, Lang B. Autoantibodies in childhood opsoclonus-myoclonus syndrome. Neuroimmunol. 2008;201–202:221–6.

    Article  Google Scholar 

  75. Jacobs DA, Fung KM, Cook NM, Schalepfer WW, Goldberg HI, Stecker MM. Complex partial status epilepticus associated with anti-Hu paraneoplastic syndrome. J Neurol Sci. 2003;213(1–2):77–82.

    Article  PubMed  Google Scholar 

  76. Espay AJ, Kumar V, Sarpel G. Anti-Hu-associated paraneoplastic limbic encephalitis presenting as rapidly progressive non-convulsive status epilepticus. J Neurol Sci. 2006;246(1–2):149–52.

    Article  CAS  PubMed  Google Scholar 

  77. Takahashi Y, Mori H, Mishina M, Watanabe M, Fujiwara T, Shimomura J, et al. Autoantibodies to NMDA receptor in patients with chronic forms of epilepsia partialis continua. Neurology. 2003;61(7):891–6.

    Article  CAS  PubMed  Google Scholar 

  78. Veciana M, Becerra JL, Fossas P, Muriana D, Sansa G, Santamarina E, et al. EEG extreme delta brush: an ictal pattern in patients with anti-NMDA receptor encephalitis. Epilepsy Behav. 2015;49:280–5.

    Article  CAS  PubMed  Google Scholar 

  79. Baykan B, Martinez-Juarez IE, Altindag EA, Camfield CS, Camfield PR. Lifetime prognosis of juvenile myoclonic epilepsy. Epilepsy Behav. 2013;28(Suppl 1):S18–24.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Feinberg School of Medicine, Northwestern University, 710N Lake Shore Drive, Abbott Hall #1425, Chicago, IL, 60611, USA

    Anna Serafini, Elizabeth E. Gerard & Stephan U. Schuele

Authors
  1. Anna Serafini
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Elizabeth E. Gerard
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Stephan U. Schuele
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Stephan U. Schuele .

Editor information

Editors and Affiliations

  1. Department of Neurology, Harvard Medical School, Comprehensive Epilepsy Center, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA

    Frank W. Drislane

  2. Department of Neurology, Johns Hopkins Bayview Medical Center, Baltimore, Maryland, USA

    Peter W. Kaplan MBBS

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer Science+Business Media LLC

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Serafini, A., Gerard, E.E., Schuele, S.U. (2018). Myoclonic Status Epilepticus. In: Drislane, F., Kaplan MBBS, P. (eds) Status Epilepticus. Current Clinical Neurology. Springer, Cham. https://doi.org/10.1007/978-3-319-58200-9_12

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-58200-9_12

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-58198-9

  • Online ISBN: 978-3-319-58200-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation