Trevathan E, Murphy CC, Yeargin-Allsopp M. Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. Epilepsia. 1997 Dec. 38(12):1283-8. [QxMD MEDLINE Link].
Arzimanoglou A, French J, Blume WT, Cross JH, Ernst JP, Feucht M, et al. Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol. 2009 Jan. 8(1):82-93. [QxMD MEDLINE Link]. [Full Text].
Engel J Jr; International League Against Epilepsy (ILAE). A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia. 2001 Jun. 42(6):796-803. [QxMD MEDLINE Link].
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 Apr. 51 (4):676-85. [QxMD MEDLINE Link].
Heiskala H. Community-based study of Lennox-Gastaut syndrome. Epilepsia. 1997 May. 38(5):526-31. [QxMD MEDLINE Link].
Oguni H, Hayashi K, Osawa M. Long-term prognosis of Lennox-Gastaut syndrome. Epilepsia. 1996. 37 Suppl 3:44-7. [QxMD MEDLINE Link].
Ohtsuka Y, Amano R, Mizukawa M, Ohtahara S. Long-term prognosis of the Lennox-Gastaut syndrome. Jpn J Psychiatry Neurol. 1990 Jun. 44(2):257-64. [QxMD MEDLINE Link].
Bare MA, Glauser TA, Strawsburg RH. Need for electroencephalogram video confirmation of atypical absence seizures in children with Lennox-Gastaut syndrome. J Child Neurol. 1998 Oct. 13(10):498-500. [QxMD MEDLINE Link].
Al-Banji MH, Zahr DK, Jan MM. Lennox-Gastaut syndrome. Management update. Neurosciences (Riyadh). 2015 Jul. 20 (3):207-12. [QxMD MEDLINE Link].
van Rijckevorsel K. Treatment of Lennox-Gastaut syndrome: overview and recent findings. Neuropsychiatr Dis Treat. 2008 Dec. 4(6):1001-19. [QxMD MEDLINE Link]. [Full Text].
Wheless JW, Clarke DF, Arzimanoglou A, Carpenter D. Treatment of pediatric epilepsy: European expert opinion, 2007. Epileptic Disord. 2007 Dec. 9(4):353-412. [QxMD MEDLINE Link].
Conry JA, Ng YT, Paolicchi JM, Kernitsky L, Mitchell WG, Ritter FJ, et al. Clobazam in the treatment of Lennox-Gastaut syndrome. Epilepsia. 2009 May. 50(5):1158-66. [QxMD MEDLINE Link].
Ng YT, Collins SD. Clobazam. Neurotherapeutics. 2007 Jan. 4(1):138-44. [QxMD MEDLINE Link].
Ng YT, Conry JA, Drummond R, Stolle J, Weinberg MA. Randomized, phase III study results of clobazam in Lennox-Gastaut syndrome. Neurology. 2011 Oct 11. 77(15):1473-1481. [QxMD MEDLINE Link].
Feucht M, Brantner-Inthaler S. Gamma-vinyl-GABA (vigabatrin) in the therapy of Lennox-Gastaut syndrome: an open study. Epilepsia. 1994 Sep-Oct. 35(5):993-8. [QxMD MEDLINE Link].
You SJ, Kang HC, Kim HD, Lee HS, Ko TS. Clinical efficacy of zonisamide in Lennox-Gastaut syndrome: Korean multicentric experience. Brain Dev. 2008 Apr. 30(4):287-90. [QxMD MEDLINE Link].
Motte J, Trevathan E, Arvidsson JF, Barrera MN, Mullens EL, Manasco P. Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. Lamictal Lennox-Gastaut Study Group. N Engl J Med. 1997 Dec 18. 337(25):1807-12. [QxMD MEDLINE Link].
Glauser TA. Topiramate. Epilepsia. 1999. 40 Suppl 5:S71-80. [QxMD MEDLINE Link].
Glauser TA, Levisohn PM, Ritter F, Sachdeo RC. Topiramate in Lennox-Gastaut syndrome: open-label treatment of patients completing a randomized controlled trial. Topiramate YL Study Group. Epilepsia. 2000. 41 Suppl 1:S86-90. [QxMD MEDLINE Link].
Sachdeo RC, Glauser TA, Ritter F, Reife R, Lim P, Pledger G. A double-blind, randomized trial of topiramate in Lennox-Gastaut syndrome. Topiramate YL Study Group. Neurology. 1999 Jun 10. 52(9):1882-7. [QxMD MEDLINE Link].
Pellock JM. Felbamate. Epilepsia. 1999. 40 Suppl 5:S57-62. [QxMD MEDLINE Link].
Glauser T, Kluger G, Sachdeo R, Krauss G, Perdomo C, Arroyo S. Rufinamide for generalized seizures associated with Lennox-Gastaut syndrome. Neurology. 2008 May 20. 70(21):1950-8. [QxMD MEDLINE Link].
Kluger G, Kurlemann G, Haberlandt E, Ernst JP, Runge U, Schneider F, et al. Effectiveness and tolerability of rufinamide in children and adults with refractory epilepsy: first European experience. Epilepsy Behav. 2009 Mar. 14(3):491-5. [QxMD MEDLINE Link].
Perucca E, Cloyd J, Critchley D, Fuseau E. Rufinamide: clinical pharmacokinetics and concentration-response relationships in patients with epilepsy. Epilepsia. 2008 Jul. 49(7):1123-41. [QxMD MEDLINE Link].
Wheless JW, Isojarvi J, Lee D, Drummond R, Benbadis SR. Clobazam is efficacious for patients across the spectrum of disease severity of Lennox-Gastaut syndrome: Post hoc analyses of clinical trial results by baseline seizure-frequency quartiles and VNS experience. Epilepsy Behav. 2014 Oct 2. 41C:47-52. [QxMD MEDLINE Link].
Devinsky O, Patel AD, Cross JH, Villanueva V, Wirrell EC, Privitera M, et al. Effect of Cannabidiol on Drop Seizures in the Lennox-Gastaut Syndrome. N Engl J Med. 2018 May 17. 378 (20):1888-1897. [QxMD MEDLINE Link].
Thiele EA, Marsh ED, French JA, Mazurkiewicz-Beldzinska M, Benbadis SR, Joshi C, et al. Cannabidiol in patients with seizures associated with Lennox-Gastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet. 2018 Mar 17. 391 (10125):1085-1096. [QxMD MEDLINE Link].
Ferrie CD, Patel A. Treatment of Lennox-Gastaut Syndrome (LGS). Eur J Paediatr Neurol. 2009 Nov. 13(6):493-504. [QxMD MEDLINE Link].
Lerner JT, Salamon N, Sankar R. Clinical profile of vigabatrin as monotherapy for treatment of infantile spasms. Neuropsychiatr Dis Treat. 2010 Nov 8. 6:731-40. [QxMD MEDLINE Link]. [Full Text].
Ohtsuka Y, Yoshinaga H, Shirasaka Y, Takayama R, Takano H, Iyoda K. Rufinamide as an adjunctive therapy for Lennox-Gastaut syndrome: A randomized double-blind placebo-controlled trial in Japan. Epilepsy Res. 2014 Nov. 108(9):1627-36. [QxMD MEDLINE Link].
Banzel (rufinamide) [package insert]. Woodcliff Lake, NJ: Eisai Inc. 2/2015. Available at [Full Text].
Lowes R. Clobazam (Onfi) can cause serious skin reactions, FDA warns. Medscape Medical News. December 3, 2013. Available at http://www.medscape.com/viewarticle/815303. Accessed: December 8, 2013.
FDA Drug Safety Communication. FDA warns of serious skin reactions with the anti-seizure drug Onfi (clobazam) and has approved label changes. December 3, 2013. Available at http://www.fda.gov/Drugs/DrugSafety/ucm377204.htm. Accessed: December 8, 2013.
Thiele E, Bebin EM, Bhathal H, et al. Cannabidiol (CBD) treatment in patients with seizures associated with tuberous sclerosis complex: A randomized, double-blind, placebo-controlled phase 3 trial (GWPCARE6) (Abst 1.293). Presented at the American Epilepsy Society 73rd Annual Meeting. 2019 Dec 6-10. Baltimore, MD. [Full Text].
Cukiert A, Burattini JA, Mariani PP, Câmara RB, Seda L, Baldauf CM, et al. Extended, one-stage callosal section for treatment of refractory secondarily generalized epilepsy in patients with Lennox-Gastaut and Lennox-like syndromes. Epilepsia. 2006 Feb. 47(2):371-4. [QxMD MEDLINE Link].
Ben-Menachem E, Hellström K, Waldton C, Augustinsson LE. Evaluation of refractory epilepsy treated with vagus nerve stimulation for up to 5 years. Neurology. 1999 Apr 12. 52(6):1265-7. [QxMD MEDLINE Link].
Lancman G, Virk M, Shao H, Mazumdar M, Greenfield JP, Weinstein S, et al. Vagus nerve stimulation vs. corpus callosotomy in the treatment of Lennox-Gastaut syndrome: a meta-analysis. Seizure. 2013 Jan. 22 (1):3-8. [QxMD MEDLINE Link].
Neal EG, Chaffe H, Schwartz RH, Lawson MS, Edwards N, Fitzsimmons G, et al. The ketogenic diet for the treatment of childhood epilepsy: a randomised controlled trial. Lancet Neurol. 2008 Jun. 7(6):500-6. [QxMD MEDLINE Link].
Freeman JM, Vining EP, Kossoff EH, Pyzik PL, Ye X, Goodman SN. A blinded, crossover study of the efficacy of the ketogenic diet. Epilepsia. 2009 Feb. 50(2):322-5. [QxMD MEDLINE Link].
De Los Reyes EC, Sharp GB, Williams JP, Hale SE. Levetiracetam in the treatment of Lennox-Gastaut syndrome. Pediatr Neurol. 2004 Apr. 30(4):254-6. [QxMD MEDLINE Link].

Media Gallery

Patient with Lennox-Gastaut syndrome wearing a helmet with face guard to protect against facial injury from atonic seizures
Slow spike wave pattern in a 24-year-old awake male with Lennox-Gastaut syndrome. The slow posterior background rhythm has frequent periods of 2- to 2.5-Hz discharges, maximal in the bifrontocentral areas, occurring in trains as long as 8 seconds without any clinical accompaniment.

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Author

Koshi A Cherian, MD Assistant Professor, Department of Neurology and Pediatrics, Albert Einstein College of Medicine; Attending Physician, Department of Neurology, Division of Child Neurology and Epilepsy, Montefiore Medical Center; Attending Physician, Department of Pediatrics, Division of Child Neurology, Jacobi Medical Center; Staff Physician (Courtesy), Department of Pediatrics, Division of Child Neurology, St Barnabas Hospital

Koshi A Cherian, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, Child Neurology Society, Medical Society of the State of New York

Disclosure: Nothing to disclose.

Coauthor(s)

Tracy A Glauser, MD Professor, Departments of Pediatrics and Neurology, University of Cincinnati College of Medicine; Director, Comprehensive Epilepsy Center, Co-Director, Genetic Pharmacology Service, Cincinnati Children's Hospital Medical Center

Tracy A Glauser, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, Child Neurology Society

Disclosure: Received consulting fee from Eisai, ucb Pharma, Supernus, SK Life Science, Claritas, and Sunovion for consulting; Received royalty from AssureRx for license.

Diego A Morita, MD Assistant Professor of Pediatrics and Neurology, Department of Pediatrics, Division of Neurology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine

Diego A Morita, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association

Disclosure: Nothing to disclose.

Karen Mary Stannard, MD, FRCPC Pediatric Neurologist, Milford Regional Medical Center, Boston Children's Hospital

Karen Mary Stannard, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA Professor of Pediatrics, Neurology, Neurosurgery, and Psychiatry, Medical Director, Tulane Center for Autism and Related Disorders, Tulane University School of Medicine; Pediatric Neurologist and Epileptologist, Ochsner Hospital for Children; Professor of Neurology, Louisiana State University School of Medicine

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, American Neurological Association, Association of Military Surgeons of the US, Child Neurology Society, Southern Pediatric Neurology Society

Disclosure: Nothing to disclose.

Acknowledgements

David A Griesemer, MD Professor, Departments of Neuroscience and Pediatrics, Medical University of South Carolina

David A Griesemer, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Epilepsy Society, Child Neurology Society, and Society for Neuroscience

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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