What is Lennox-Gastaut Syndrome?

Lennox-Gastaut Syndrome (LGS) is a rare, severe form of epilepsy characterized by the presence of tonic seizures and at least one additional seizure type. It typically starts during childhood and persists into adulthood.4  Approximately 50% of infants with a severe infantile epilepsy syndrome evolve over time to LGS.5 

LGS has far-reaching effects beyond seizures; impairments with developmental delay culminating in cognitive impairment, communication, psychiatric symptoms, sleep, behavioral challenges and mobility are common.5

Seizures largely remain uncontrolled on currently available medications.8 Sudden unexpected death in epilepsy (SUDEP) is a major concern for patients and families with LGS.9

 

Learn more about LGS

Burden of disease

LGS has wide-ranging affects, which include:

Behavior disorders5

Persistence through childhood and adolescence to 
adult years5

Intellectual disability5

Impacted quality of life due to seizure frequency and number of seizure free days14

LGS impacts the entire family—parents, caregivers, and siblings—including:15

  • Feelings of isolation, which can lead to depression15 
  • Restriction of social life and relationship problems between partners and other family members15
  • Physical exhaustion and disrupted sleep15
  • Financial concerns13

LGS by the numbers

There are approximately: 

~48K LGS patients in the U.S.17

~1M LGS patients worldwide17

How is LGS treated?

Treatment options vary for patients with LGS.

Several ways to treat LGS include anti-seizure medicines, dietary therapy, neuromodulation and other management options.16

Safety concerns for patients with LGS

LGS is characterized by many different seizure types. Some of these seizure types—tonic, atonic, generalized tonic-clonic, atypical absence, and myoclonic— may result in frequent falls and injuries. As a result, protective equipment such as helmets are often needed.1,3,5,15 

Moreover, the cognitive impairment in patients with LGS may impact their use of language.10 Some patients may place themselves in potentially dangerous situations, if not properly supervised. Importantly, families and caregivers may need to take appropriate precautions to ensure their loved one diagnosed with LGS is safe.11

References
  1. Strzelczyk A, Schubert-Bast S. Expanding the Treatment Landscape for LGS: Current and Future Strategies. CNS Drugs. 2021;35(1):61-83. 
  2. Scheffer IE, Liao J. Deciphering the concepts behind “Epileptic encephalopathy” and “Developmental and epileptic encephalopathy”. Eur J Paediatr Neurol. 2020;24:11-14. 
  3. Mastrangelo M. LGS: A State of the Art Review. Neuropediatrics. 2017;48(3):143-151.
  4. NORD. LGS. https://rarediseases.org/rare-diseases/lennox-gastaut-syndrome. Accessed 17 July 2022. 
  5. International League Against Epilepsy. Specchio, N, Wirrell, EC, Scheffer, IE, et al. Draft guideline: ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions. https://www.ilae.org/files/dmfile/CHILDApril6withfigs.pdf. Accessed 17 July 2022. 
  6. LGS Foundation. LGS Characteristics and Major Concerns Survey. https://www.lgsfoundation.org/wp-content/uploads/2021/08/2019-PFDD-Caregiver-Survey-1.pdf, Accessed October 17, 2021. 
  7. LGS. Child Neurology Foundation. https://www.childneurologyfoundation.org/disorder/lennox-gastaut-syndrome. Accessed 17 July 2022. 
  8. Specchio N, Wirrell EC, Scheffer IE, Nabbout R, Riney K, Samia P, et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022;63:1398–1442. https://doi.org/10.1111/epi.17241 
  9. LGS Foundation. Sudden Unexpected Death in Epilepsy (SUDEP). https://www.lgsfoundation.org/sudep/. Accessed November 18, 2022.
  10. Gallop K, Wild D, Verdian L, et al. LGS: development of conceptual models of health-related quality of life (HRQL) for caregivers and children. Seizure. 2010;19(1):23-30.
  11. LGS Foundation. Seizures and Safety. https://www.lgsfoundation.org/seizure-safety. Accessed 17 July 2022. 
  12. NIH. LGS. https://medlineplus.gov/genetics/condition/lennox-gastaut-syndrome. Accessed 17 July 2022. 
  13. Reaven NL, Funk SE, Montouris GD, Saurer TB, Story TJ. Burden of illness in patients with possible Lennox-Gastaut syndrome: A retrospective claims-based study. Epilepsy Behav. 2018;88:66-73.
  14. Auvin S, Damera V, Martine M, et al. The impact of seizure frequency on quality of life in patients with Lennox-Gastaut syndrome or Dravet syndrome. Epilepsy & Behavior. 2021 ; 123 :108239. 
  15. Cross JH, Auvin S, Falip M, Striano P, Arzimanoglou A. Expert Opinion on the Management of Lennox-Gastaut Syndrome: Treatment Algorithms and Practical Considerations. Front Neurol. 2017;8:505. Published 2017 Sep 29. 
  16. LGS Foundation. How is LGS treated? https://www.lgsfoundation.org/about-lgs-2/how-is-lgs-treated/. Accessed 17 July 2022. 
  17. LGS Foundation. How Many People Have LGS? https://www.lgsfoundation.org/about-lgs-2/how-many-people-have-lgs/ Accessed September 6, 2022.