About this Journal Submit a Manuscript Table of Contents
Epilepsy Research and Treatment
Volume 2012 (2012), Article ID 642725, 6 pages
http://dx.doi.org/10.1155/2012/642725
Review Article

Epileptic Encephalopathies with Status Epilepticus during Sleep: New Techniques for Understanding Pathophysiology and Therapeutic Options

National Neurological Institute C. Mondino, Child Neuropsychiatry Unit, University of Pavia, 27100 Pavia, Italy

Received 19 March 2012; Revised 9 July 2012; Accepted 9 July 2012

Academic Editor: Nicola Specchio

Copyright © 2012 Daniela Brazzo et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Linked References

  1. G. Patry, S. Lyagoubi, and C. A. Tassinari, “Subclinical electrical status epilepticus induced by sleep in children. A clinical and electroencephalographic study of six cases.,” Archives of Neurology, vol. 24, no. 3, pp. 242–252, 1971. View at Scopus
  2. C. A. Tassinari, O. Daniele, and F. Gambarelli, “Excessive 7–14 sec positive spikes during REM sleep in monozygotic non epileptic twins with speech retardation,” Revue d'E.E.G. et de Neuro-Physiologie Clinique, vol. 7, no. 2, pp. 192–193, 1977. View at Scopus
  3. Commission on Classification and Terminology of the International League against Epilepsy, “Proposal for revised classification of epilepsies and epileptic syndromes,” Epilepsia, vol. 30, no. 4, pp. 389–399, 1989.
  4. E. R. Perez, “Syndromes of acquired epileptic aphasia and epilepsy with continuous spike-waves during sleep: models for prolonged cognitive impairment of epileptic origin,” Seminars in Pediatric Neurology, vol. 2, no. 4, pp. 269–277, 1995. View at Scopus
  5. W. M. Landau and F. R. Kleffner, “Syndrome of acquired aphasia with convulsive disorder in children,” Neurology, vol. 7, no. 8, pp. 523–530, 1957. View at Scopus
  6. A. S. Galanopoulou, A. Bojko, F. Lado, and S. L. Moshé, “The spectrum of neuropsychiatric abnormalities associated with electrical status epilepticus in sleep,” Brain and Development, vol. 22, no. 5, pp. 279–295, 2000. View at Publisher · View at Google Scholar · View at Scopus
  7. N. Fejerman, “Atypical rolandic epilepsy,” Epilepsia, vol. 50, no. 7, pp. 9–12, 2009. View at Publisher · View at Google Scholar · View at Scopus
  8. C. A. Tassinari, G. Rubboli, L. Volpi et al., “Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia,” Clinical Neurophysiology, vol. 111, no. 2, pp. S94–S102, 2000. View at Publisher · View at Google Scholar · View at Scopus
  9. C. A. Tassinari, R. Michelucci, A. Forti et al., “The electrical status epilepticus syndrome.,” Epilepsy Research, vol. 6, pp. 111–115, 1992. View at Scopus
  10. P. G. Rossi, A. Parmeggiani, A. Posar, M. C. Scaduto, S. Chiodo, and G. Vatti, “Landau-Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES),” Brain and Development, vol. 21, no. 2, pp. 90–98, 1999. View at Publisher · View at Google Scholar · View at Scopus
  11. M. Inutsuka, K. Kobayashi, M. Oka, J. Hattori, and Y. Ohtsuka, “Treatment of epilepsy with electrical status epilepticus during slow sleep and its related disorders,” Brain and Development, vol. 28, no. 5, pp. 281–286, 2006. View at Publisher · View at Google Scholar · View at Scopus
  12. A. Beaumanoir, “EEG data,” in Continuous Spikes and Waves during Slow Sleep, A. Beaumanoir, M. Bureau, T. Deonna, L. Mira, and C. A. Tassinari, Eds., pp. 217–223, John Libbey, London, UK, 1995.
  13. M. Van Hirtum-Das, E. A. Licht, S. Koh, J. Y. Wu, W. D. Shields, and R. Sankar, “Children with ESES: variability in the syndrome.,” Epilepsy Research, vol. 70, pp. S248–258, 2006. View at Scopus
  14. C. A. Tassinari and G. Rubboli, “Cognition and paroxysmal EEG activities: from a single spike to electrical status epilepticus during sleep,” Epilepsia, vol. 47, no. 2, pp. 40–43, 2006. View at Publisher · View at Google Scholar · View at Scopus
  15. B. K. Bölsterli, B. Schmitt, T. Bast et al., “Impaired slow wave sleep downscaling in encephalopathy with status epilepticus during sleep (ESES),” Clinical Neurophysiology, vol. 122, no. 9, pp. 1779–1787, 2011. View at Publisher · View at Google Scholar · View at Scopus
  16. K. Nickels and E. Wirrell, “Electrical status epilepticus in sleep,” Seminars in Pediatric Neurology, vol. 15, no. 2, pp. 50–60, 2008. View at Publisher · View at Google Scholar · View at Scopus
  17. C. Billard, A. Autret, F. Laffont, B. Lucas, and F. Degiovanni, “Electrical status epilepticus during sleep in children: a reappraisal from eight new cases,” in Sleep and Epilepsy, M. B. Sterman, M. N. Shouse, and P. Passouant, Eds., pp. 481–491, Academic Press, New York, NY, USA, 1982.
  18. P. DeMarco, “Electrical status epilepticus during slow sleep: one case with sensory aphasia,” Clinical EEG Electroencephalography, vol. 19, no. 2, pp. 111–113, 1988. View at Scopus
  19. C. G. Fowler, J. E. Kile, and K. E. Hecox, “Electrical status epilepticus in slow wave sleep: prospective case study of a cortical hearing impairment,” Journal of the American Academy of Audiology, vol. 12, no. 4, pp. 174–182, 2001. View at Scopus
  20. W. Fröscher, “Sleep and prolonged epileptic activity (status epilepticus),” Epilepsy Research, vol. 2, pp. 165–176, 1991. View at Scopus
  21. G. L. Holmes and P. P. Lenck-Santini, “Role of interictal epileptiform abnormalities in cognitive impairment,” Epilepsy and Behavior, vol. 8, no. 3, pp. 504–515, 2006. View at Publisher · View at Google Scholar · View at Scopus
  22. B. Dalla Bernardina, E. Fontana, B. Michelizza, V. Colamaria, G. Capovilla, and C. A. Tassinari, “Partial epilepsies of childhood, bilateral synchronization, continuous spike-waves during slow sleep,” in Advances in Epileptology, S. Manelis, E. Bental, J. N. Loeber, and F. E. Dreifuss, Eds., pp. 295–302, Raven Press, New York, NY, USA, 1989.
  23. T. Loddenkemper, G. Cosmo, P. Kotagal et al., “Epilepsy surgery in children with electrical status epilepticus in sleep,” Neurosurgery, vol. 64, no. 2, pp. 328–337, 2009. View at Publisher · View at Google Scholar · View at Scopus
  24. P. Veggiotti, C. Termine, E. Granocchio, S. Bova, G. Papalia, and G. Lanzi, “Long-term neuropsychological follow-up and nosological considerations in five patients with continuous spikes and waves during slow sleep,” Epileptic Disorders, vol. 4, no. 4, pp. 243–249, 2002. View at Scopus
  25. X. De Tiège, S. Goldman, D. Verheulpen, A. Aeby, N. Poznanski, and P. Van Bogaert, “Coexistence of idiopathic rolandic epilepsy and CSWS in two families,” Epilepsia, vol. 47, no. 10, pp. 1723–1727, 2006. View at Publisher · View at Google Scholar · View at Scopus
  26. T. Morikawa, M. Seino, Y. Watanabe, M. Watanabe, and K. Yagi, “Clinical relevance of continuous spike-waves during slow wave sleep,” in Advances in Epileptology, S. Manelis, E. Bental, J. N. Loeber, and F. E. Dreifuss, Eds., pp. 359–363, Raven Press, New York, NY, USA, 1989.
  27. P. Veggiotti, F. Beccaria, R. Guerrini, G. Capovilla, and G. Lanzi, “Continuous spike-and-wave activity during slow-wave sleep: syndrome or EEG pattern?” Epilepsia, vol. 40, no. 11, pp. 1593–1601, 1999. View at Publisher · View at Google Scholar · View at Scopus
  28. R. Guerrini, P. Genton, M. Bureau et al., “Multilobar polymicrogyria, intractable drop attack seizures, and sleep- related electrical status epilepticus,” Neurology, vol. 51, no. 2, pp. 504–512, 1998. View at Scopus
  29. F. Guzzetta, D. Battaglia, C. Veredice et al., “Early thalamic injury associated with epilepsy and continuous spike-wave during slow sleep,” Epilepsia, vol. 46, no. 6, pp. 889–900, 2005. View at Publisher · View at Google Scholar · View at Scopus
  30. T. Nakayama, S. Nabatame, Y. Saito et al., “8p deletion and 9p duplication in two children with electrical status epilepticus in sleep syndrome,” Seizure, vol. 21, no. 4, pp. 295–299, 2012. View at Publisher · View at Google Scholar
  31. S. H. G. Kevelam, F. E. Jansen, E. V. Binsbergen et al., “Copy number variations in patients with electrical status epilepticus in sleep,” Journal of Child Neurology, vol. 27, no. 2, pp. 178–182, 2012. View at Publisher · View at Google Scholar
  32. M. Broli, F. Bisulli, M. Mastrangelo et al., “Definition of the neurological phenotype associated with dup (X)(p11.22-p11.23),” Epileptic Disorders, vol. 3, no. 3, pp. 240–251, 2011.
  33. A. P. Aldenkamp, J. Arends, S. Verspeek, and M. Berting, “The cognitive impact of epileptiform EEG-discharges; relationship with type of cognitive task,” Child Neuropsychology, vol. 10, no. 4, pp. 297–305, 2004. View at Publisher · View at Google Scholar · View at Scopus
  34. I. Sanchez Fernandez, J. Peters, M. Takeoka, et al., “Patients with electrical status epilepticus in sleep share similar clinical features regardless of their focal or generalized sleep potentiation of epileptiform activity,” Journal of Child Neurology. In press. View at Publisher · View at Google Scholar
  35. A. Ray, J. X. Tao, S. M. Hawes-Ebersole, and J. S. Ebersole, “Localizing value of scalp EEG spikes: a simultaneous scalp and intracranial study,” Clinical Neurophysiology, vol. 118, no. 1, pp. 69–79, 2007. View at Publisher · View at Google Scholar · View at Scopus
  36. A. J. Bell and T. J. Sejnowski, “An information-maximization approach to blind separation and blind deconvolution.,” Neural Computation, vol. 7, no. 6, pp. 1129–1159, 1995. View at Scopus
  37. S. Makeig, T. P. Jung, A. J. Bell, D. Ghahremani, and T. J. Sejnowski, “Blind separation of auditory event-related brain responses into independent components,” Proceedings of the National Academy of Sciences of the United States of America, vol. 94, no. 20, pp. 10979–10984, 1997. View at Publisher · View at Google Scholar · View at Scopus
  38. K. Kobayashi, T. Akiyama, T. Nakahori, H. Yoshinaga, and J. Gotman, “Systematic source estimation of spikes by a combination of independent component analysis and RAP-MUSIC I: principles and simulation study,” Clinical Neurophysiology, vol. 113, no. 5, pp. 713–724, 2002. View at Publisher · View at Google Scholar · View at Scopus
  39. K. Kobayashi, T. Akiyama, T. Nakahori, H. Yoshinaga, and J. Gotman, “Systematic source estimation of spikes by a combination of independent component analysis and RAP-MUSIC: II: preliminary clinical application,” Clinical Neurophysiology, vol. 113, no. 5, pp. 725–734, 2002. View at Publisher · View at Google Scholar · View at Scopus
  40. D. F. Sobel, M. Aung, H. Otsubo, and M. C. Smith, “Magnetoencephalography in children with Landau-Kleffner syndrome and acquired epileptic aphasia,” American Journal of Neuroradiology, vol. 21, no. 2, pp. 301–307, 2000. View at Scopus
  41. R. Paetau, “Magnetoencephalography in landau-kleffner syndrome,” Epilepsia, vol. 50, no. 7, pp. 51–54, 2009. View at Publisher · View at Google Scholar · View at Scopus
  42. J. Jacobs, E. Kobayashi, R. Boor et al., “Hemodynamic responses to interictal epileptiform discharges in children with symptomatic epilepsy,” Epilepsia, vol. 48, no. 11, pp. 2068–2078, 2007. View at Publisher · View at Google Scholar · View at Scopus
  43. S. Vulliemoz, R. Thornton, R. Rodionov et al., “The spatio-temporal mapping of epileptic networks: combination of EEG-fMRI and EEG source imaging,” NeuroImage, vol. 46, no. 3, pp. 834–843, 2009. View at Publisher · View at Google Scholar · View at Scopus
  44. S. Seri, J. N. Thai, D. Brazzo, F. Pisani, and A. Cerquiglini, “Neurophysiology of CSWS-associated cognitive dysfunction,” Epilepsia, vol. 50, no. 7, pp. 33–36, 2009. View at Publisher · View at Google Scholar · View at Scopus
  45. M. Siniatchkin, K. Groening, J. Moehring et al., “Neuronal networks in children with continuous spikes and waves during slow sleep,” Brain, vol. 133, no. 9, pp. 2798–2813, 2010. View at Publisher · View at Google Scholar · View at Scopus
  46. X. De Tiège, S. Goldman, and P. Van Bogaert, “Insights into the pathophysiology of psychomotor regression in CSWS syndromes from FDG-PET and EEG-fMRI,” Epilepsia, vol. 50, no. 7, pp. 47–50, 2009. View at Publisher · View at Google Scholar · View at Scopus
  47. X. De Tiège, N. Ligot, S. Goldman, N. Poznanski, A. de Saint Martin, and P. Van Bogaert, “Metabolic evidence for remote inhibition in epilepsies with continuous spike-waves during sleep,” NeuroImage, vol. 40, no. 2, pp. 802–810, 2008. View at Publisher · View at Google Scholar · View at Scopus
  48. R. Ribacoba, J. Salas-Puig, D. M. Solar, B. Otero, and J. L. Herranz, “The efficacy of the valproic acid-ethosuximide association in the continuous slow point-wave syndrome during sleep,” Neurologia, vol. 12, no. 8, pp. 335–338, 1997. View at Scopus
  49. A. Yasuhara, H. Yoshida, T. Hatanaka, T. Sugimoto, Y. Kobayashi, and E. Dyken, “Epilepsy with continuous spike-waves during slow sleep and its treatment,” Epilepsia, vol. 32, no. 1, pp. 59–62, 1991. View at Scopus
  50. M. Inutsuka, K. Kobayashi, M. Oka, J. Hattori, and Y. Ohtsuka, “Treatment of epilepsy with electrical status epilepticus during slow sleep and its related disorders,” Brain and Development, vol. 28, no. 5, pp. 281–286, 2006. View at Publisher · View at Google Scholar · View at Scopus
  51. M. De Negri, M. G. Baglietto, F. M. Battaglia, R. Gaggero, A. Pessagno, and L. Recanati, “Treatment of electrical status epilepticus by short diazepam (DZP) cycles after DZP rectal bolus test,” Brain and Development, vol. 17, no. 5, pp. 330–333, 1995. View at Publisher · View at Google Scholar · View at Scopus
  52. S. B. Wang, W. C. Weng, P. C. Fan, and W. T. Lee, “Levetiracetam in continuous spike waves during slow-wave sleep syndrome,” Pediatric Neurology, vol. 39, no. 2, pp. 85–90, 2008. View at Publisher · View at Google Scholar · View at Scopus
  53. S. Chhun, P. Troude, N. Villeneuve et al., “A prospective open-labeled trial with levetiracetam in pediatric epilepsy syndromes: continuous spikes and waves during sleep is definitely a target,” Seizure, vol. 20, no. 4, pp. 320–325, 2011. View at Publisher · View at Google Scholar · View at Scopus
  54. U. Kramer, L. Sagi, H. Goldberg-Stern, N. Zelnik, A. Nissenkorn, and B. Ben-Zeev, “Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES),” Epilepsia, vol. 50, no. 6, pp. 1517–1524, 2009. View at Publisher · View at Google Scholar · View at Scopus
  55. D. B. Sinclair and T. J. Snyder, “Corticosteroids for the treatment of Landau-Kleffner syndrome and continuous spike-wave discharge during sleep,” Pediatric Neurology, vol. 32, no. 5, pp. 300–306, 2005. View at Publisher · View at Google Scholar · View at Scopus
  56. M. Buzatu, C. Bulteau, C. Altuzarra, O. Dulac, and P. Van Bogaert, “Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep,” Epilepsia, vol. 50, no. 7, pp. 68–72, 2009. View at Publisher · View at Google Scholar · View at Scopus
  57. M. A. Mikati, R. Saab, M. N. Fayad, and R. N. Choueiri, “Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome,” Pediatric Neurology, vol. 26, no. 4, pp. 298–300, 2002. View at Publisher · View at Google Scholar · View at Scopus
  58. M. Nikanorova, M. J. Miranda, M. Atkins, and L. Sahlholdt, “Ketogenic diet in the treatment of refractory continuous spikes and waves during slow sleep,” Epilepsia, vol. 50, no. 5, pp. 1127–1131, 2009. View at Publisher · View at Google Scholar · View at Scopus
  59. T. Loddenkemper, G. Cosmo, P. Kotagal et al., “Epilepsy surgery in children with electrical status epilepticus in sleep,” Neurosurgery, vol. 64, no. 2, pp. 328–337, 2009. View at Publisher · View at Google Scholar · View at Scopus
  60. F. Morrell, W. W. Whisler, M. C. Smith et al., “Landau-Kleffner syndrome: treatment with subpial intracortical transection,” Brain, vol. 118, no. 6, pp. 1529–1546, 1995. View at Scopus
  61. R. Caraballo, E. Fontana, and B. Michelizza, “Carbamazepina, “assenze atipiche”, crisi “atoniche” e stato di PO continua del sonno (POCS),” Bollettino della Lega Italiana contro l’Epilessia, vol. 66-67, pp. 379–381, 1989.
  62. P. Lerman, “Seizures induced or aggravated by anticonvulsants,” Epilepsia, vol. 27, no. 6, pp. 706–710, 1986. View at Scopus
  63. A. Van Lierde, “Therapeutic data,” in Epileptic Syndrome in Infancy, Childhood and Adolescence, J. Roger, M. Bureau, and C. Dravet, Eds., pp. 225–227, John Libbey, Eastleigh, UK, 1992.
  64. A. T. Berg, S. F. Berkovic, M. J. Brodie et al., “Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE commission on classification and terminology, 2005–2009,” Epilepsia, vol. 51, no. 4, pp. 676–685, 2010. View at Publisher · View at Google Scholar · View at Scopus
  65. C. Urbain, T. Di Vincenzo, P. Peigneux, and P. Van Bogaert, “Is sleep-related consolidation impaired in focal idiopathic epilepsies of childhood? A pilot study,” Epilepsy & Behavior, vol. 22, no. 2, pp. 380–384, 2011.
  66. P. Veggiotti, M. C. Pera, F. Teutonico, D. Brazzo, U. Balottin, and C. A. Tassinari, “Therapy of encephalopathy with status epilepticus during sleep (ESES/CSWS syndrome): an update,” Epileptic Disorders, vol. 14, no. 1, pp. 1–11, 2012.
  67. M. Buzatu, C. Bulteau, C. Altuzarra, O. Dulac, and P. Van Bogaert, “Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep,” Epilepsia, vol. 50, no. 7, pp. 68–72, 2009. View at Publisher · View at Google Scholar · View at Scopus
  68. J. Praline, C. Hommet, M. A. Barthez et al., “Outcome at adulthood of the continuous spike-waves during slow sleep and Landau-Kleffner syndromes,” Epilepsia, vol. 44, no. 11, pp. 1434–1440, 2003. View at Publisher · View at Google Scholar · View at Scopus
  69. T. Tsuru, M. Mori, M. Mizuguchi, and M. Y. Momoi, “Effects of high-dose intravenous corticosteroid therapy in Landau-Kleffner syndrome,” Pediatric Neurology, vol. 22, no. 2, pp. 145–147, 2000. View at Publisher · View at Google Scholar · View at Scopus
  70. Ç. Okuyaz, K. Aydin, K. Gücüyener, and A. Serdaroǧlu, “Treatment of electrical status epilepticus during slow-wave sleep with high-dose corticosteroid,” Pediatric Neurology, vol. 32, no. 1, pp. 64–67, 2005. View at Publisher · View at Google Scholar · View at Scopus