eMedicine Specialties > Neurology > Pediatric Neurology

Lennox-Gastaut Syndrome: Follow-up

Author: Tracy A Glauser, MD, Professor, Departments of Pediatrics and Neurology, University of Cincinnati College of Medicine, Children's Comprehensive Epilepsy Program, Children's Hospital Medical Center of Cincinnati
Coauthor(s): 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
Contributor Information and Disclosures

Updated: Apr 10, 2006

Follow-up

Further Inpatient Care

  • Patients with LGS experience frequent exacerbations of their seizures that may require inpatient adjustment of AEDs.

Complications

  • Death (either sudden unexplained death in epilepsy or related to an accident involving a seizure)
  • Injuries (especially facial) from seizures resulting in falls
  • Dose-related, idiosyncratic, or long-term adverse effects from AEDs
  • Renal, cardiac, or metabolic complications from the ketogenic diet

Prognosis

  • Long-term prognosis overall is unfavorable but variable.
  • Longitudinal studies of children with LGS found a minority of patients eventually could work normally, but 47-76% still had typical characteristics (mental retardation, treatment resistant seizures) many years after onset and required significant help (eg, home care, institutionalization).
  • Patients with symptomatic LGS, particularly those with an early onset of seizures, prior history of West syndrome, higher frequency of seizures, or constant slow EEG background activity, have a worse prognosis than those with idiopathic seizures.
  • Tonic seizures may persist and be more difficult to control over time, while myoclonic and atypical absences appear easier to control.
  • The characteristic diffuse slow spike wave pattern of LGS gradually disappears with age and is replaced by focal epileptic discharges, especially multiple independent spikes.

Miscellaneous

Medicolegal Pitfalls

  • Failure to inform the patient and his or her family of the risk for severe idiosyncratic reactions from 3 commonly used AEDs for LGS constitutes a medical/legal risk.
    • Valproate - Hepatotoxicity, pancreatitis
    • Lamotrigine - Stevens-Johnson syndrome, toxic epidermal necrolysis
    • Felbamate - Aplastic anemia, hepatotoxicity
  • Failure to recognize the signs and symptoms of LGS, which could result in failure to select an appropriate AED with proven efficacy, presents a medical/legal risk. This may increase the risk of uncontrolled seizures, increasing the risk for injury and death.
  • Failure to prescribe a medication for emergency intervention (eg, rectal diazepam) may pose a medical/legal risk. Patients with LGS have a recognized high risk for status epilepticus.
 


More on Lennox-Gastaut Syndrome

Overview: Lennox-Gastaut Syndrome
Differential Diagnoses & Workup: Lennox-Gastaut Syndrome
Treatment & Medication: Lennox-Gastaut Syndrome
Follow-up: Lennox-Gastaut Syndrome
Multimedia: Lennox-Gastaut Syndrome
References
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References

  1. Alvarez N, Besag F, Iivanainen M. Use of antiepileptic drugs in the treatment of epilepsy in people with intellectual disability. J Intellect Disabil Res. Dec 1998;42 Suppl 1:1-15. [Medline].

  2. Bare MA, Glauser TA, Strawsburg RH. Need for electroencephalogram video confirmation of atypical absence seizures in children with Lennox-Gastaut syndrome. J Child Neurol. Oct 1998;13(10):498-500. [Medline].

  3. Beaumanoir A. The Lennox-Gastaut syndrome: a personal study. Electroencephalogr Clin Neurophysiol Suppl. 1982;(35):85-99. [Medline].

  4. Ben-Menachem E, Hellstrom K, Waldton C. Evaluation of refractory epilepsy treated with vagus nerve stimulation for up to 5 years. Neurology. Apr 12 1999;52(6):1265-7. [Medline].

  5. Conry JA. Pharmacologic treatment of the catastrophic epilepsies. Epilepsia. 2004;45 Suppl 5:12-6. [Medline].

  6. Crumrine PK. Lennox-Gastaut syndrome. J Child Neurol. Jan 2002;17 Suppl 1:S70-5. [Medline].

  7. De Los Reyes EC, Sharp GB, Williams JP. Levetiracetam in the treatment of Lennox-Gastaut syndrome. Pediatr Neurol. Apr 2004;30(4):254-6. [Medline].

  8. Dulac O, N''Guyen T. The Lennox-Gastaut syndrome. Epilepsia. 1993;34 Suppl 7:S7-17. [Medline].

  9. Feucht M, Brantner-Inthaler S. Gamma-vinyl-GABA (vigabatrin) in the therapy of Lennox-Gastaut syndrome: an open study. Epilepsia. Sep-Oct 1994;35(5):993-8. [Medline].

  10. Freeman JM, Vining EP. Seizures decrease rapidly after fasting: preliminary studies of the ketogenic diet. Arch Pediatr Adolesc Med. Sep 1999;153(9):946-9. [Medline].

  11. Gastaut H. The Lennox-Gastaut syndrome: comments on the syndrome''s terminology and nosological position amongst the secondary generalized epilepsies of childhood. Electroencephalogr Clin Neurophysiol Suppl. 1982;(35):71-84. [Medline].

  12. Glauser TA, Levisohn PM, Ritter F. 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].

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

  14. Glauser TA. Pediatric epilepsy syndromes. Curr Opin Pediatr. Dec 1995;7(6):640-9. [Medline].

  15. Glauser TA. Following catastrophic epilepsy patients from childhood to adulthood. Epilepsia. 2004;45 Suppl 5:23-6. [Medline].

  16. Hancock E, Cross H. Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev. 2003;CD003277. [Medline].

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

  18. Livingston JH. The Lennox-Gastaut syndrome. Dev Med Child Neurol. Aug 1988;30(4):536-40. [Medline].

  19. Lynch BJ, Glauser TA, Canter C. Neurologic complications of pediatric heart transplantation. Arch Pediatr Adolesc Med. Sep 1994;148(9):973-9. [Medline].

  20. Motte J, Trevathan E, Arvidsson JF. Lamotrigine for generalized seizures associated with the Lennox-Gastaut syndrome. Lamictal Lennox-Gastaut Study Group [published erratum appears in N Engl J Med 1998 Sep 17;339(12):851-2]. N Engl J Med. Dec 18 1997;337(25):1807-12. [Medline].

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

  22. Ohtahara S. Lennox-Gastaut syndrome. Considerations in its concept and categorization. Jpn J Psychiatry Neurol. Sep 1988;42(3):535-42. [Medline].

  23. Ohtsuka Y, Amano R, Mizukawa M. Long-term prognosis of the Lennox-Gastaut syndrome. Jpn J Psychiatry Neurol. Jun 1990;44(2):257-64. [Medline].

  24. Pellock JM. Managing pediatric epilepsy syndromes with new antiepileptic drugs. Pediatrics. Nov 1999;104(5 Pt 1):1106-16. [Medline].

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

  26. Roger J, Dravet C, Bureau M. The Lennox-Gastaut syndrome. Cleve Clin J Med. 1989;56 Suppl Pt 2:S172-80. [Medline].

  27. Sachdeo RC, Glauser TA, Ritter F. A double-blind, randomized trial of topiramate in Lennox-Gastaut syndrome. Topiramate YL Study Group. Neurology. Jun 10 1999;52(9):1882-7. [Medline].

  28. Shields WD, Sankar R. Vigabatrin. Semin Pediatr Neurol. Mar 1997;4(1):43-50. [Medline].

  29. Shields WD. Diagnosis of infantile spasms, Lennox-Gastaut syndrome, and progressive myoclonic epilepsy. Epilepsia. 2004;45 Suppl 5:2-4. [Medline].

  30. Trevathan E, Murphy CC, Yeargin-Allsopp M. Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. Epilepsia. Dec 1997;38(12):1283-8. [Medline].

  31. Wheless JW, Constantinou JE. Lennox-Gastaut syndrome. Pediatr Neurol. Oct 1997;17(3):203-11. [Medline].

  32. Wheless JW. The ketogenic diet: Fa(c)t or fiction [editorial]. J Child Neurol. Nov 1995;10(6):419-23. [Medline].

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Further Reading

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Keywords

childhood epileptic encephalopathy with diffuse slow spike waves, Lennox-Gastaut syndrome, LGS, pediatric epilepsy syndrome, childhood epilepsy, seizures, mental retardation

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Contributor Information and Disclosures

Author

Tracy A Glauser, MD, Professor, Departments of Pediatrics and Neurology, University of Cincinnati College of Medicine, Children's Comprehensive Epilepsy Program, Children's Hospital Medical Center of Cincinnati
Tracy A Glauser, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Coauthor(s)

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
Diego A Morita, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

David A Griesemer, MD, Professor, Departments of Neurology and Pediatrics, Medical University of South Carolina
David A Griesemer, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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