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Lennox-Gastaut Syndrome Differential Diagnoses

  • Author: Koshi A Cherian, MD; Chief Editor: Amy Kao, MD  more...
 
Updated: Jul 27, 2016
 
 

Diagnostic Considerations

Lennox-Gastaut syndrome must be differentiated from myoclonic-astatic epilepsy (Doose syndrome).

Differential Diagnoses

 
 
Contributor Information and Disclosures
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 for consulting; Received consulting fee from Lundbeck for consulting; Received consulting fee from Questcor for consulting; Received consulting fee from ucb Pharma for consulting; Received consulting fee from Supernus for consulting; Received honoraria from Supernus for speaking and teaching; Received consulting fee from Sunovion for consulting; Received royalty from AssureRx for license; Received consulting fee from Upsher-Smith for consulting; Received consul.

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

Karen Mary Stannard, MD 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

Amy Kao, MD Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.

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

References
  1. 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. [Medline].

  2. 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. [Medline]. [Full Text].

  3. 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. [Medline].

  4. Heiskala H. Community-based study of Lennox-Gastaut syndrome. Epilepsia. 1997 May. 38(5):526-31. [Medline].

  5. Oguni H, Hayashi K, Osawa M. Long-term prognosis of Lennox-Gastaut syndrome. Epilepsia. 1996. 37 Suppl 3:44-7. [Medline].

  6. 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. [Medline].

  7. 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. [Medline].

  8. Al-Banji MH, Zahr DK, Jan MM. Lennox-Gastaut syndrome. Management update. Neurosciences (Riyadh). 2015 Jul. 20 (3):207-12. [Medline].

  9. van Rijckevorsel K. Treatment of Lennox-Gastaut syndrome: overview and recent findings. Neuropsychiatr Dis Treat. 2008 Dec. 4(6):1001-19. [Medline]. [Full Text].

  10. Wheless JW, Clarke DF, Arzimanoglou A, Carpenter D. Treatment of pediatric epilepsy: European expert opinion, 2007. Epileptic Disord. 2007 Dec. 9(4):353-412. [Medline].

  11. 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. [Medline].

  12. Ng YT, Collins SD. Clobazam. Neurotherapeutics. 2007 Jan. 4(1):138-44. [Medline].

  13. 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. [Medline].

  14. 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. [Medline].

  15. 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. [Medline].

  16. 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. [Medline].

  17. Glauser TA. Topiramate. Epilepsia. 1999. 40 Suppl 5:S71-80. [Medline].

  18. 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. [Medline].

  19. 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. [Medline].

  20. Pellock JM. Felbamate. Epilepsia. 1999. 40 Suppl 5:S57-62. [Medline].

  21. 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. [Medline].

  22. 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. [Medline].

  23. 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. [Medline].

  24. Ferrie CD, Patel A. Treatment of Lennox-Gastaut Syndrome (LGS). Eur J Paediatr Neurol. 2009 Nov. 13(6):493-504. [Medline].

  25. 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. [Medline]. [Full Text].

  26. 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. [Medline].

  27. Banzel (rufinamide) [package insert]. Woodcliff Lake, NJ: Eisai Inc. 2/2015. Available at [Full Text].

  28. 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. [Medline].

  29. 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.

  30. 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.

  31. 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. [Medline].

  32. 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. [Medline].

  33. 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. [Medline].

  34. 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. [Medline].

  35. 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. [Medline].

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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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