Epilepsy pp 127-140 | Cite as

Juvenile Myoclonic Epilepsy in Humans

  • David W. McCandless


Juvenile myoclonic epilepsy is a common epilepsy, most often seen in the adolescent population. There are two distinct forms of adolescent myoclonic epilepsy: juvenile myoclonic epilepsy and a more severe type called progressive myoclonic epilepsy. Both these forms will be considered in this chapter.


Myoclonic Epilepsy Juvenile Myoclonic Epilepsy Myoclonic Seizure Temporal Lobe Epilepsy Patient Idiopathic Generalize Epilepsy 
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  1. Berkovic, S. (2008) Progressive myoclonic epilepsies. In Pediatric Epilepsy Pellock, J., et al., eds. Demos, NY, pp 367-375Google Scholar
  2. Berkovic, S., et al. (1986) Progressive myoclonus epilepsies: specific causes and diagnosis. N. Eng J. Med. 315:296–305CrossRefGoogle Scholar
  3. Opeskin, K., et al. (2000) Idiopathic generalized epilepsy: lack of significant microdysgenesis. Neurology 55:1101–1106PubMedGoogle Scholar
  4. Hirose, S., et al. (2005) Genetics of idiopatic epilepsy. Epilepsia 46:38–43PubMedCrossRefGoogle Scholar
  5. Panayiotopoulos, C., Obeid, T., and Tahan, A. (1994) Juvenile myoclonic epilepsy: a 5 year prospective study. Epilepsia 35:258–296CrossRefGoogle Scholar
  6. Obeid, T. (1994) Clinical and genetic aspects of juvenile absence epilepsy. J. Neurol. 241:487–491PubMedCrossRefGoogle Scholar
  7. Nair, R., and Thomas, S. (2004) Genetic liability to epilepsy in Kerala State, India. Epil. Res. 62:163–170CrossRefGoogle Scholar
  8. Dhanuka, A., et al. (2001) Juvenile myoclonic epilepsy: a clinical and sleep EEG study. Seizure 10:374–378PubMedCrossRefGoogle Scholar
  9. Bourgeois, B., et al. (1987) Monotherapy with valproate in primary generalized epilepsies. Epilepsia 28:S8-S11PubMedCrossRefGoogle Scholar
  10. Rees, M., et al. (1994) Linkage analysis of idiopathic generalized epilepsy in families of probands with juvenile myoclonic epilepsy and marker loci in the region of EPM 1 on chromosome 21 q. Neuroped 25:20–25CrossRefGoogle Scholar
  11. Pal, D., et al. (2003) BRD2 is a probable major susceptibility gene for common juvenile myoclonic epilepsy. J. Hum. Genet. 73:261–270CrossRefGoogle Scholar
  12. Woermann, F., et al. (1999) Abnormal cerebral structure in juvenile myoclonic epilepsy demonstrated with voxel based analysis of MRI. Brain 122:2101–2108PubMedCrossRefGoogle Scholar
  13. Martinez-Juarez, I., et al. (2006) Juvenile myoclonic epilepsy subsyndromes. Brain 129:1269–1280PubMedCrossRefGoogle Scholar
  14. Shorvon, S., and Walker, M. (2005) Status epilepticus in idiopathic generalized epilepsy. Epilepsia 46:73–79PubMedCrossRefGoogle Scholar
  15. Koepp, M. (2005) Juvenile myoclonic epilepsy-a generalized epilepsy syndrome? Acta Neurol Scand 112:57–62CrossRefGoogle Scholar
  16. Delgado-Escuetta, A., and Enrile-Bacsal, F. (1984) Juvenile myoclonic epilepsy of Janz. Neurol. 34:285–294Google Scholar
  17. Savic, I., et al. (2000) MR spectroscopy shows reduced frontal lobe concentrations of N-acetyl asparate in patients with juvenile myoclonic epilepsy. Epilepsia 41:290–296PubMedCrossRefGoogle Scholar
  18. Woermann, F., et al. (1998) Quantitative MRI in patients with idiopathic generalized epilepsy. Evidence of widespread cerebral structural changes. Brain 121:1661–1667Google Scholar
  19. Pung, T., Schmitz, B. (2006) Circadian rhythm and personality profile in juvenile myoclonic epilepsy. Epilepsia 47:111–114PubMedCrossRefGoogle Scholar
  20. Janz, D,and Christian, W. (1957) Impulsiv-Petit mal. Dtsch Nervenheilkd 176:346–386Google Scholar
  21. Fittipaldi, F., et al. (2001) EEG discharges on awakening: a marker of idiopathic generalized epilepsy. Neurology 56:123–126PubMedGoogle Scholar
  22. Delgado-Escueta, A. (2007) Advances in genetics of juvenile myoclonic epilepsies. Curr. Rev Clin Sci. 7:61–67Google Scholar
  23. Krauss, G., and Morrison, P. (2007) Understanding and treating a channelopathy: severe myoclonic epilepsy of infancy. Neurology 69:233–234PubMedCrossRefGoogle Scholar
  24. Striano, P., et al. (2007) An open label trial of levetiracetam in severe myoclonic epilepsy of infancy. Neurology 69:250–254PubMedCrossRefGoogle Scholar
  25. Meldrum, B., and Rogawski, M. (2007) Molecular targets for antiepileptic drug development. Neurother. 4:18–61CrossRefGoogle Scholar
  26. Piazzini, A., et al. (2008) Frontal cognitive dysfunction in juvenile myoclonic epilepsy. Epilepsy 49:657–662CrossRefGoogle Scholar
  27. Kim, J. et al. (2007) Regional grey matter abnormalities in juvenile myoclonic epilepsy: a voxel based morphometric study. Neuroimage 37:1132–1137PubMedCrossRefGoogle Scholar
  28. Specchio, N., et al. (2008) Effects of levetiracetam on EEG abnormalities in juvenile myoclonic epilepsy. Epilepsia 49:663–669PubMedCrossRefGoogle Scholar
  29. Gelisse, P., et al. (2001) Clinical factors of drug resistance in juvenile myoclonic epilepsy. J. Neurol. Neurosurg. Psychiat. 70:240–243PubMedCrossRefGoogle Scholar
  30. Labate, A., et al. (2007) Usefulness of a morning routine EEG recording in patients with juvenile myoclonic epilepsy. Epil. Res. 77:17–21CrossRefGoogle Scholar
  31. Noachtar, S., et al. (2008) Levetiracetam for the treatment of idiopathic generalized epilepsy with myoclonic seizures. Neurology 70:607–616PubMedCrossRefGoogle Scholar
  32. Hirano, Y., et al. (2009) Differentiation of myoclonic seizures in epileptic syndromes: a video polygraphic study of 26 patients. Epilepsia 50:1525–1535PubMedCrossRefGoogle Scholar
  33. Lin, K., et al. (2009) Magnetic resonance spectroscopy reveals an epileptic network in juvenile myoclonic epilepsy. Epilepsia 50:1191–1200PubMedCrossRefGoogle Scholar
  34. Filho, G., et al. (2009) Are personality traits of juvenile myoclonic epilepsy related to frontal lobe dysfunctions? A protein MRS study. Epilepsia 50:1201–1209CrossRefGoogle Scholar
  35. Holmes, M., Quiring, J., and Tucker, D. (2010) Evidence that juvenile myoclonic epilepsy is a disorder of frontotemporal corticothalamic networks. Neuroimage 49:80–93PubMedCrossRefGoogle Scholar
  36. McCormick, D. (2002) Cortical and subcortical generators of normal and abnormal rhythmicity Int. Rev. Neurobiol. 22:2323–2334Google Scholar
  37. Steriade, M. (2003) Neuronal substrate of sleep and epilepsy. Cambridge Univ. Press, Ny pp 215-227Google Scholar
  38. Tae, W., et al. (2008) Cortical thickness abnormality in juvenile myoclonic epilepsy. J. Neurol. 255:561–566PubMedCrossRefGoogle Scholar
  39. Badawy, R., et al. (2010) Can changes in cortical excitability distinguish progressive from juvenile myoclonic epilepsy? Epilepsia 51:Google Scholar
  40. Manganotti, P., et al. (2011) Highly focal BOLD activation on functional MRI in a patient with progressive myoclonic epilepsy and diffuse giant somatosensory evoked potentials Epil. Behav. PMID 21339087 Feb 19Google Scholar

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© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • David W. McCandless
    • 1
  1. 1.The Chicago Medical School Department of Cell Biology and AnatomyRosalind Franklin UniversityChicagoUSA

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