Lennox-Gastaut Syndrome

  • Warren T. Blume
Part of the Clinical Medicine and the Nervous System book series (CLIN.MED.NERV.)


The eponym “Lennox-Gastaut syndrome” refers to patients with intractable generalized, usually motor, seizures and bilaterally synchronous slow spike-waves on the electroencephalogram (EEG). Most patients are, or become, mentally subnormal; the seizures usually start in childhood. In one population of patients with epilepsy the syndrome was identified in 10% of those less than 15 years of age (Gastaut et al. 1975).


Status Epilepticus Seizure Onset Infantile Spasm Neuronal Ceroid Lipofuscinosis Tonic Seizure 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Aicardi J (1982) Childhood epilepsies with brief myoclonic, atonic or tonic seizures. In: Laidlaw J, Richens A (eds) A textbook of epilepsy, 2nd edn. Churchill Livingstone, Edinburgh, pp 88–96Google Scholar
  2. Avoli M, Gloor P (1982) Role of the thalamus in generalised penicillin epilepsy: observations on decorticated cats. Exp Neurol 77: 386–402PubMedCrossRefGoogle Scholar
  3. Avoli M, Gloor P, Kostopoulos G, Gotman J (1983) An analysis of penicillin-induced generalized spike and wave discharges using simultaneous recordings of cortical and thalamic single neurons. J Neurophysiol 50: 819–837PubMedGoogle Scholar
  4. Bauer G, Aichner F, Saltuari L (1983) Epilepsies with diffuse slow spikes and waves of late onset. Eur Neurol 22: 344–350PubMedCrossRefGoogle Scholar
  5. Bittencourt PRM, Richens A (1981) Anticonvulsant-induced status epilepticus in Lennox-Gastaut syndrome. Epilepsia 22: 129–134PubMedCrossRefGoogle Scholar
  6. Blume WT, David RB, Gomez MR (1973) Generalized sharp and slow wave complexes. Associated clinical features and long-term follow-up. Brain 96: 289–306PubMedCrossRefGoogle Scholar
  7. Chatrian GE, Lettich E, Wilkus RJ, Vallarta J (1982) Poly graphic and clinical observations on tonic-autonomic seizures. In: Broughton RJ (ed) Henri Gastaut and the Marseilles school’s contribution to the neurosciences (EEG suppl No. 35 ) Elsevier Biomedical, Amsterdam, pp 100–124Google Scholar
  8. Chevrie JJ, Aicardi J (1972) Childhood epileptic encephalopathy with slow spike-wave. A statistical study of 80 cases. Epilepsia 13: 259–271PubMedCrossRefGoogle Scholar
  9. Delgado-Escueta AV, Treiman DM, Walsh GO (1983a) The treatable epilepsies. New Engl J Med 308: 1508–1514PubMedCrossRefGoogle Scholar
  10. Delgado-Escueta AV, Treiman DM, Walsh GO (1983b) The treatable epilepsies. New Engl J Med 308: 1576–1583PubMedCrossRefGoogle Scholar
  11. Doose H, Gerken H, Leonhardt R, Volzke E, Volz C (1970) Centrencephalic myoclonic-astatic petit mal. Neuropadiatrie 2: 59–78PubMedCrossRefGoogle Scholar
  12. Dulac O, Arthuis M (1984) Open trials with valproate in epilepsy. Epilepsia. 25 [suppl 1]: S23–S31PubMedCrossRefGoogle Scholar
  13. Dwyer BE, Wasterlain CG (1983) Regulation of brain protein synthesis during status epilepticus. Adv Neurol 34: 297–304PubMedGoogle Scholar
  14. Eeg-Olofsson O (1980) Treatment of epileptic disorders of children. In: Robb JP (ed) Epilepsy updated: causes and treatment. Yearbook Medical Publishers, Chicago, pp 199–213Google Scholar
  15. Fisher RS, Prince DA (1977a) Spike-wave rhythms in cat cortex induced by parenteral penicillin. I. Electroencephalographic features. Electroencephalogr Clin Neurophysiol 42: 608–624CrossRefGoogle Scholar
  16. Fisher RS, Prince DA (1977b) Spike-wave rhythms in cat cortex induced by parenteral penicillin. II. Cellular features. Electroencephalogr Clin Neurophysiol 42: 625–639CrossRefGoogle Scholar
  17. Gastaut H, Broughton R (1972) Epileptic seizures. Charles C Thomas, Springfield, pp 37–47Google Scholar
  18. Gastaut H, Roger J, Ouahchi S, Timsit M, Broughton R (1963) An electroclinical study of generalized epileptic seizures of tonic expression. Epilepsia 4: 15–44PubMedCrossRefGoogle Scholar
  19. Gastaut H, Roger J, Soulayrol R et al. (1966a) L’encephalopathie epileptique de l’enfant avec pointeondes lentes diffuses (alias “petit mal variant”) ou syndrome de Lennox. Ann Pediatr (Paris) 13: 2093–2103Google Scholar
  20. Gastaut H, Roger J, Soulayrol R et al. (1966b) Childhood epileptic encephalopathy with diffuse spike-waves (otherwise known as “petit mal variant”) or Lennox syndrome. Epilepsia 7: 139–179CrossRefGoogle Scholar
  21. Gastaut H, Gastaut JL, Goncalves e Silva GE, Fernandez Sanchez GR (1975) Relative frequency of different types of epilepsy. A study employing the classification of the International League Against Epilepsy. Epilepsia 16: 457–461Google Scholar
  22. Gibbs FA, Gibbs EL, Lennox WG (1939) Influence of the blood sugar level on the wave and spike formation in petit mal epilepsy. Arch Neurol Psychiatr 41: 1111–1116Google Scholar
  23. Giuditta A, Metafora S, Popoli M, Perrone-Capano C (1983) Brain protein and DNA synthesis during seizures. Adv Neurol 34: 289–295PubMedGoogle Scholar
  24. Gloor P (1979) Generalized epilepsy with spike-and-wave discharge: a reinterpretation of its electrographic and clinical manifestations. Epilepsia 20: 571–588PubMedCrossRefGoogle Scholar
  25. Gloor P, Kalabay O, Giard N (1968) The electroencephalogram in diffuse encephalopathies: electroencephalographic correlates of grey and white matter lesions. Brain 91: 779–802CrossRefGoogle Scholar
  26. Gram L, Bentsen KD (1984) Controlled and comparative trials of valproate performed in Europe and Asia. Epilepsia 25 [Suppl 1]: S32–S39PubMedCrossRefGoogle Scholar
  27. Gur RC, Sussman NM, Alavi A et al. (1982) Positron emission tomography in two cases of childhood epileptic encephalopathy (Lennox-Gastaut syndrome). Neurology 32: 1191–1194PubMedGoogle Scholar
  28. Jeavons PM, Clark JE, Maheshwari MC (1977) Treatment of generalized epilepsies of childhood and adolescence with sodium valproate (‘Epilim’). Dev Med Child Neurol 19: 9–25PubMedCrossRefGoogle Scholar
  29. Komai S (1977) Lennox-Gastaut’s syndrome. Prognosis of the secondary generalized epilepsies. Epilepsia 18: 131Google Scholar
  30. Kostopoulos G, Avoli M, Pellegrini A, Gloor P (1982) Laminar analysis of spindles and of spikes of the spike and wave discharge of feline generalized penicillin epilepsy. Electroencephalogr Clin Neurophysiol 53: 1–13PubMedCrossRefGoogle Scholar
  31. Kreindler A, Zuckermann E, Steriade M, Chimion D (1958) Electroclinical features of convulsions induced by stimulation of the brain stem. J Neurophysiol 21: 430–436PubMedGoogle Scholar
  32. Lennox WG, Davis JP (1950) Clinical correlates of the fast and the slow spike-wave electroencephalogram. Pediatrics 5: 626–644PubMedGoogle Scholar
  33. Margerison JH, Corsellis JAN (1966) Epilepsy and the temporal lobes. Brain 89: 499–530PubMedCrossRefGoogle Scholar
  34. Markand ON (1977) Slow spike-wave activity in EEG and associated clinical features: often called ‘Lennox’ or “Lennox-Gastaut” syndrome. Neurology 27: 746–757PubMedGoogle Scholar
  35. McLachlan RS, Robitaille Y, Gloor P (1982) Effect of anoxia in feline generalized penicillin epilepsy (FGPE). Electroencephalogr Clin Neurophysiol 54: 39PCrossRefGoogle Scholar
  36. Niedermeyer E (1968) The Lennox-Gastaut syndrome: a severe type of childhood epilepsy. Electroencephalogr Clin Neurophysiol 24: 283 (P)PubMedGoogle Scholar
  37. Niedermeyer E (1984) The Lennox-Gastaut syndrome and its frontiers. Paper presented at International Symposium in Honor of Henri Gastaut, Bologna, Italy, Sept. 1984Google Scholar
  38. Ohtahara S, Yamatogi Y, Ohtsuka Y (1976) Prognosis of the Lennox syndrome — long-term clinical and electroencephalographic follow-up study, especially with special reference to relationship with the West syndrome. Folia Psychiatr Neurol Jpn 30: 275–287PubMedGoogle Scholar
  39. Pellegrini A, Musgrave J, Gloor P (1979) Role of afferent input of subcortical origin in the genesis of bilaterally synchronous epileptic discharges of feline generalized penicillin epilepsy. Exp Neurol 64: 155–173PubMedCrossRefGoogle Scholar
  40. Pollen DA (1964) Intracellular studies of cortical neurons during thalamic induced wave and spike. Electroencephalogr Clin Neurophysiol 17: 398–404PubMedCrossRefGoogle Scholar
  41. Prince DA, Connors BW (1984) Mechanisms of epileptogenesis in cortical structures. Ann Neurol 16 [Suppl]: S59–S64PubMedCrossRefGoogle Scholar
  42. Rodin E, Onuma T, Wasson S, Porzak J, Rodin M (1971) Neurophysiological mechanisms involved in grand mal seizures induced by Metrazol and Megimide. Electroencephalogr Clin Neurophysiol 30: 62–72PubMedCrossRefGoogle Scholar
  43. Rodin E, Kitano H, Wasson S, Rodin M (1975) Experimental models of “centrencephalic” epilepsy. EEG and multiple unit recordings. Electroencephalogr Clin Neurophysiol 38: 546 (P)Google Scholar
  44. Smeraldi E, Scorza-Smeraldi R, Cazzullo CL, Guareschi-Cazzullo A, Canger R (1976) A genetic approach to the Lennox-Gastaut syndrome by the “major histocompatibility complex” (MHC). In: Janz D ed) Epileptology: proceedings of the Seventh International symposium on Epilepsy. Thieme, Berlin (West), pp 33–37Google Scholar
  45. Sorel L (1964) L’epilepsie myokinetique grave de la premiere enfance avec pointe-onde lente (Petit mal variant) et son traitement. Rev Neurol 100: 215–223Google Scholar
  46. Tassinari CA, Dravet C, Roger J, Cano JP, Gastaut H (1972) Tonic status epilepticus precipitated by intravenous benzodiazepine in five patients with Lennox-Gastaut syndrome. Epilepsia 13: 421–435PubMedCrossRefGoogle Scholar
  47. Tassinari CA, Terzano G, Capocchi G et al. (1977) Epileptic seizures during sleep in children. In: Penry JK (ed) Epilepsy, The Eighth International Symposium. Raven, New York, pp 345–354Google Scholar
  48. Vasella F, Pavlincova E, Schneider HJ, Rudin HJ, Karbowski K (1973) Treatment of infantile spasms and Lennox-Gastaut syndrome with clonazepam (Rivotril). Epilepsia 14: 165–175CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1987

Authors and Affiliations

  • Warren T. Blume

There are no affiliations available

Personalised recommendations