Journal of Neurology

, Volume 264, Issue 7, pp 1388–1396 | Cite as

Understanding Lennox–Gastaut syndrome: insights from focal epilepsy patients with Lennox–Gastaut features

  • Sophie DupontEmail author
  • Raluca Banica-Wolters
  • Isabelle An-Gourfinkel
  • Virginie Lambrecq
  • Vincent Navarro
  • Claude Adam
  • Vi-Huong Nguyen-Michel
Original Communication


To delineate the clinical and EEG features of adults with focal epilepsy associated with a generalized paroxysmal fast activity (GPFA) pattern on EEG who developed refractory seizures, notably drop attacks, but do not fulfill the classical triad for the diagnosis of Lennox–Gastaut syndrome (LGS) and provide further insight into LGS mechanisms. Among 957 patients admitted to video-EEG monitoring between 2002 and 2015, we retrospectively research adult patients with refractory focal epilepsy, drop attacks and GPFA on EEG. We collected demographic, anamnestic, and clinical data from medical records. We reviewed for all patients the interictal and ictal video-EEG recordings. We identified ten patients with focal epilepsy and electro-clinical features of LGS. As compared to classical LGS patients, our patients: (1) began epilepsy later (15.4 ± 8 years); (2) exhibited exclusively focal onset seizures, including drop attacks seizures linked to focal asymmetrical tonic posturing seizures; (3) had a stable cognition over time and (4) evolved favourably with a good secondary response to treatments in 80% of cases. Interestingly, all patients exhibited apparent diffuse interictal and ictal EEG abnormalities but a detailed analysis revealed that 50% had asymmetrical GPFA and 70% secondary bilateral synchrony processes. We may hypothesize here that a process of “secondary LGS” occurred which produced a worsening of seizures with the apparition of drop attacks and GPFA on EEG. This study brings arguments to consider that some cases of LGS could be linked to the development of a “secondary epileptic network” driven by a primary focal epileptic zone.


Focal epilepsy Drop attacks Generalized paroxysmal fast activity Secondary Lennox–Gastaut 


Compliance with ethical standards

Conflicts of interest

The authors have nothing to declare.

Ethical standard

The study has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.


  1. 1.
    Arzimanoglou A, French J, Blume WT et al (2009) Lennox–Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol 8:82–93CrossRefPubMedGoogle Scholar
  2. 2.
    Camfield P, Camfield C (2002) Epileptic syndromes in childhood: clinical features, outcomes, and treatment. Epilepsia 43(Suppl. 3):27–32CrossRefPubMedGoogle Scholar
  3. 3.
    Bourgeois BF, Douglass LM, Sankar R (2014) Lennox–Gastaut syndrome: a consensus approach to differential diagnosis. Epilepsia 55(Suppl. 4):4–9CrossRefPubMedGoogle Scholar
  4. 4.
    Gastaut H, Roger J, Soulayrol R et al (1966) Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise known as “petit mal variant”) or Lennox syndrome. Epilepsia 7:139–179CrossRefGoogle Scholar
  5. 5.
    Markand ON (1977) Slow spike-wave activity in EEG and associated clinical features: often called ‘Lennox’ or “Lennox–Gastaut’ syndrome. Neurology 27:746–757CrossRefPubMedGoogle Scholar
  6. 6.
    Archer JS, Warren AEL, Jackson GD, Abbott DF (2014) Conceptualizing Lennox–Gastaut syndrome as a secondary network epilepsy. Font Neurol 5:1–11Google Scholar
  7. 7.
    Fakhoury T, Abou-Khalil B (1999) Generalized absence seizures with 10–15 Hz fast discharges. Clin Neurophysiol 110:1029–1035CrossRefPubMedGoogle Scholar
  8. 8.
    Guye M, Bartolomei Gastaut L et al (2001) Absence epilepsy with fast rhythmic discharges during sleep: an intermediary form of generalized epilepsy? Epilepsia 42:351–356CrossRefPubMedGoogle Scholar
  9. 9.
    Shih TT, Hirsch LJ (2003) Tonic-absence seizures: an underrecognized seizure type. Epilepsia 44:461–465CrossRefPubMedGoogle Scholar
  10. 10.
    Kaminska A, Ickowicz A, Plouin P, Bru MF, Dellatolas G, Dulac O (1999) Delineation of cryptogenic Lennox-Gastaut syndrome and myoclonic astatic epilepsy using multiple correspondence analysis. Epilepsy Res 36(1):15–29CrossRefPubMedGoogle Scholar
  11. 11.
    Kuruvilla A, Flink R (2002) Focal fast rhythmic epileptiform discharges on scalp EEG in a patient with cortical dysplasia. Seizure 11:330–334CrossRefPubMedGoogle Scholar
  12. 12.
    Chassoux F, Landré E, Mellerio C et al (2012) Type II focal cortical dysplasia: electroclinical phenotype and surgical outcome related to imaging. Epilepsia 53:349–358CrossRefPubMedGoogle Scholar
  13. 13.
    Mohammadi M, Okanishi T, Okanari K et al (2015) Asymmetrical generalized paroxysmal fast activities in children with intractable localization-related epilepsy. Brain Dev 37:59–65CrossRefPubMedGoogle Scholar
  14. 14.
    Oguni H, Hayashi K, Osawa M (1996) Long-term prognosis of Lennox–Gastaut syndrome. Epilepsia 37(Suppl. 3):44–47CrossRefPubMedGoogle Scholar
  15. 15.
    Blume WT (2004) Lennox–Gastaut syndrome: potential mechanisms of cognitive regression. Ment Retard Dev Disabil Res Rev 10(2):150–153CrossRefPubMedGoogle Scholar
  16. 16.
    Pedersen M, Curwood EK, Archer JS, Abbott DF, Jackson GD (2015) Brain regions with abnormal network properties in severe epilepsy of Lennox–Gastaut phenotype: multivariate analysis of task-free fMRI. Epilepsia 56(11):1767–1773CrossRefPubMedGoogle Scholar
  17. 17.
    Berg AT, Berkovic SF, Brodie MJ et al (2010) Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia 51(4):676–685CrossRefPubMedGoogle Scholar
  18. 18.
    Ohtahara S, Ohtsuka Y, Kobayashi K (1995) Lennox–Gastaut syndrome: a new vista. Psychiatry Clin Neurosci 49(3):S179–S183CrossRefPubMedGoogle Scholar
  19. 19.
    Gastaut H, Zifkin BG (1992) The Lennox–Gastaut syndrome. In: Roger J, Bureau M, Dravet Ch et al (eds) Epileptic syndromes in infancy, childhood and adolescence, 4th edn. John Libbey, LondonGoogle Scholar
  20. 20.
    Beaumanoir A (1982) The Lennox–Gastaut syndrome: a personal study. Electroencephalogr Clin Neurophysiol 35:85–99Google Scholar
  21. 21.
    Tinuper P, Cerullo A, Marini C et al (1998) Epileptic drop attacks in partial epilepsy: clinical features, evolution, and prognosis. J Neurol Neurosurg Psychiatry 64:231–237CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Blume WT (2001) Pathogenesis of Lennox–Gastaut syndrome: considerations and hypotheses. Epileptic Disord 3(4):183–196PubMedGoogle Scholar
  23. 23.
    Badawy RA, Macdonell RA, Vogrin SJ, Lai A, Cook MJ (2012) Cortical excitability decreases in Lennox–Gastaut syndrome. Epilepsia 53(9):1546–1553CrossRefPubMedGoogle Scholar
  24. 24.
    Goldsmith IL, ZupancML BuchhalterJR (2000) Long-term seizure outcome in 74 patients with Lennox–Gastaut syndrome: effects of incorporating MRI head imaging in defining the cryptogenic subgroup. Epilepsia 41(4):395–399CrossRefPubMedGoogle Scholar
  25. 25.
    Li L, Fish D, Sisodiya S, Shorvon S, Alsanjari N, Stevens J (1995) High resolution magnetic resonance imaging in adults with partial or secondary generalized epilepsy attending a tertiary referral unit. J Neurol Neurosurg Psychiatry 59(4):384–387CrossRefPubMedPubMedCentralGoogle Scholar
  26. 26.
    Ohtahara S, Ohtsuka Y, Tyoshinga H, Iyoda K, Amano R, Yamatogi Y et al (1988) Lennox–Gastaut syndrome: etiological considerations. In: Niedermeyer E, Degen R (eds) The Lennox–Gastaut syndrome. Alan R. Lissp, New York, pp 47–63Google Scholar
  27. 27.
    GurR C, Sussman NM, Alavi A, Gur RE, Rosen AD, O’Connor M et al (1982) Positron emission tomography in two cases of childhood epileptic encephalopathy (Lennox–Gastaut syndrome). Neurology 32(10):1191–1194CrossRefGoogle Scholar
  28. 28.
    Iinuma K, Yanai K, Yanagisawa T, Fueki N, Tada K, Ito M et al (1987) Cerebral glucose metabolism in five patients with Lennox–Gastaut syndrome. Pediatr Neurol 3(1):12–18CrossRefPubMedGoogle Scholar
  29. 29.
    Yanai K, Iinuma K, Matsuzawa T, Ito M, Miyabayashi S, Narisawa K et al (1987) Cerebral glucose utilization in pediatric neurological disorders determined by positron emission tomography. Eur J Nucl Med 13(6):292–296CrossRefPubMedGoogle Scholar
  30. 30.
    Miyauchi T, Nomura Y, Ohno S, Kishimoto H, Matsushita M (1988) Positron emission tomography in three cases of Lennox–Gastaut syndrome. Psychiatry Clin Neurosci 42(4):795–804CrossRefGoogle Scholar
  31. 31.
    Seri S, Cerquiglini A, Pisani F, Michel CM, Pascual Marqui RD, Curatolo P (1998) Frontal lobe epilepsy associated with tuberous sclerosis: electroencephalographic-magnetic resonance image fusioning. J Child Neurol 13(1):33–38CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Epileptology UnitAP-HP, GH Pitie-Salpêtrière-Charles FoixParisFrance
  2. 2.Rehabilitation UnitAP-HP, GH Pitie-Salpêtrière-Charles Foix, Hôpital de la Pitié-SalpêtrièreParisFrance
  3. 3.Reference Center of Rare EpilepsiesParisFrance
  4. 4.Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC)ParisFrance
  5. 5.Department of Clinical NeurophysiologyAP-HP, GH Pitie-Salpêtrière-Charles FoixParisFrance
  6. 6.Sorbonne University, UPMC Univ. Paris 06ParisFrance
  7. 7.Hôpital de la SalpêtrièreParis Cedex 13France

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