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Lennox-Gastaut Syndrome (LGS)

PAGE CONTENTS

Care Team

Lennox-Gastaut syndrome (LGS) is a rare, complex neurological condition that primarily manifests in early childhood. It is one of the epileptic encephalopathies, in which multiple seizure types and abnormal interictal brain wave patterns contribute to the development of cognitive impairment.1

Children affected by LGS have unique medical needs and require a multidisciplinary care team that spans fields of expertise. Because of the rarity of LGS, local care teams often may not be as familiar with the condition as comprehensive care centers that uniquely offer a concentration of LGS understanding and experience. To help parents and patients find the closest care center, the LGS Foundation has compiled a searchable database of comprehensive LGS care centers throughout the United States.2

The multidisciplinary team caring for patients with LGS may include: pediatric neurologists/epileptologists, pediatricians, cardiologists, neurosurgeons, orthopedists, geneticists, sleep specialists, developmental psychologists or neuropsychologists, dietitians (especially in Keto clinics), physical/occupational/speech therapists, social workers, and other providers.2 Communication and collaboration among all members of the team are essential for optimal patient care, and when all providers are located in one center, access to care is more convenient for patients and their caregivers.

Pediatric Neurologist/Epileptologist

A pediatric neurologist/epileptologist specializes in the care of children with epilepsy. They receive additional training and education in the diagnosis and treatment of diverse types of seizures.3

Children with LGS have multiple types of seizures that are often resistant to antiepileptic drugs (AEDs). Frequently, a combination of multiple AEDs is required to reduce the frequency and intensity of seizures.4

It is important that a child with LGS receive care from a knowledgeable provider who is familiar with the available antiepileptic treatments, can identify those that are most effective, and can customize them according to the patient’s presentation and response to treatment.4

Read more about LGS therapies

Pediatrician

Because the signs of LGS predominantly emerge during early childhood, the primary care physician of most patients with LGS is a pediatrician. As the patient becomes an adolescent and then a young adult, strategies for a smooth transition of care from a pediatrician and pediatric neurology teams to adult care teams are critical to maintaining the continuity and quality of care.5

Cardiologist

Patients with LGS are at increased risk for the development of cardiac abnormalities. Cardiac arrhythmias and repolarization abnormalities occur frequently in patients with either focal or generalized refractory epilepsy, such as LGS.6

Seizures are known to alter the heart rate and also have been found to cause ventricular tachyarrhythmias and asystole.6-10 This association may explain one of the life-threatening complications of LGS — sudden unexplained death in epilepsy (SUDEP) — which may be caused by cardiac or pulmonary disturbances or shutdown of the cerebrum during a seizure episode.6,9,10 Patients with ictal bradycardia may require a pacemaker.6,10 

A cardiologist who is knowledgeable about how LGS can affect cardiac function can conduct a thorough evaluation, treat cardiac dysfunction effectively, and prevent poor outcomes in patients with LGS.

Read more about LGS comorbidities

Neurosurgeon

If a patient’s seizures are refractory to AED therapy, another potential treatment is neurosurgery. A neurosurgeon experienced in the treatment of LGS can undertake interventions such as corpus callosotomy, implantation of a vagus nerve stimulator, and focal resection of a specific region of the brain triggering epilepsy.11

Orthopedist

An orthopedist specializes in injuries affecting the bones, muscles, soft tissues, and joints.12 They are an important member of the multidisciplinary team who provides care for patients with LGS, particularly those who are injured in seizure-related falls during atonic or grand mal seizures.13-15

Read more about LGS complications

Geneticist/Genetic Counselor

A geneticist can perform genetic tests to determine if mutations in certain genes are responsible for a patient’s LGS. The epilepsy of approximately 25% of patients with LGS has a genetic cause.16 

Additionally, results of genetic testing may guide decisions regarding more effective treatments. The treatments of 12% to 50% of patients with LGS are switched following genetic testing. A genetic counselor can help patients and their caregivers understand the results of genetic tests and why a change of treatments might be beneficial.16 

Read more about LGS genetics

Sleep Specialist

Seizures occur in patients with LGS during both wakefulness and sleep. Tonic seizures are the seizure type that most often occurs during sleep17,18 and are the predominant seizure type in adults with LGS.19 

A patient who experiences sleep disruption due to seizures must be treated by a sleep specialist familiar with LGS. Additionally, a sleep specialist may prevent SUDEP by detecting and treating obstructive and central sleep apnea, which has been found to be temporally linked to seizure activity on electroencephalography. Sleep apnea can be managed with positive airway pressure therapy.20

Developmental Neuropsychologist

Cognitive impairment and developmental delay are major features of epileptic encephalopathies, including LGS. These signs manifest before the onset of seizures in approximately 70% to 80% of children.1 A developmental neuropsychologist can evaluate children with LGS to determine the severity of cognitive impairment or developmental delay and how this can affect learning and growth.21 

With testing, a developmental neuropsychologist can identify children with rehabilitative potential and formulate treatment plans to mitigate behavioral problems. The neuropsychologist can also recommend cognitive therapy, occupational therapy, and/or pharmacotherapy.21

Read more about LGS clinical features

Dietitian

A dietitian, especially one working in a Keto clinic, can develop meal plans and educate patients and caregivers about the ketogenic diet, which has proved beneficial in reducing seizure frequency in individuals with refractory epilepsy.22,23

Read more about LGS diet and nutrition

Physical/Occupational/Speech Therapists 

A speech pathologist/therapist, occupational therapist, and physical therapist are all important parts of the care team who provide ongoing nonpharmacological support that may benefit children and adults with LGS.24 

Given their cognitive and learning disabilities, children with LGS often require an individualized education plan (IEP) that allows their caregivers, health care professionals (in particular speech therapists), and schools to collaborate and develop a curriculum that specifically targets their needs. As part of an IEP, the patient’s need for the special services provided by a speech/language pathologist and an occupational/physical therapist is continually re-evaluated.24,25

Read more about LGS treatment

Social Worker

Children and adults with LGS require significant resources and attention from their caregivers. A social worker can help parents and caregivers identify supportive resources and provide respite care to the families of individuals with LGS.24

Other Health Care Providers

A gastroenterologist, endocrinologist, metabolic specialist, and pulmonologist may also be required for individuals with LGS who have certain comorbidities, such as mitochondrial disorders,26,27 metabolic disorders contributing to seizure activity,28 and pulmonary hypertension as a side effect of certain AEDs, especially Fintepla® (fenfluramine).29

References

  1. Lennox-Gastaut syndrome LGS. Epilepsy Foundation. Accessed February 27, 2023.
  2. Comprehensive care centers. LGS Foundation. Accessed February 27, 2023.
  3. Childhood seizures and epilepsy. DukeHealth. Accessed February 27, 2023.
  4. van Rijckevorsel K. Treatment of Lennox-Gastaut syndrome: overview and recent findings. Neuropsychiatr Dis Treat. 2008;4(6):1001-1019. doi:10.2014/ndt.s1668
  5. Camfield PR, Gibson PA, Douglass LM. Strategies for transitioning to adult care for youth with Lennox-Gastaut syndrome and related disorders. Epilepsia. 2011;52(s5):21-27. doi:10.1111/j.1528-1167.2011.03179.x
  6. Nei M, Sperling MR, Mintzer S, Ho RT. Long-term cardiac rhythm and repolarization abnormalities in refractory focal and generalized epilepsy. Epilepsia. 2012;53(8):e137-e140. doi:10.1111/j.1528-1167.2012.03561.x
  7. Espinosa PS, Lee JW, Tedrow UB, Bromfield EB, Dworetzky BA. Sudden unexpected near death in epilepsy: malignant arrhythmia from a partial seizure. Neurology. 2009;72(19):1702-1703. doi:10.1212/WNL.0b013e3181a55f90
  8. Nei M. Cardiac effects of seizures. Epilepsy Curr. 2009;9(4):91-95. doi:10.1111/j.1535-7511.2009.01303.x
  9. Tomson T, Nashef L, Ryvlin P. Sudden unexpected death in epilepsy: current knowledge and future directions. Lancet Neurol. 2008;7(11):1021-1031. doi:10.1016/S1474-4422(08)70202-3
  10. Rugg-Gunn FJ, Simister RJ, Squirrell M, Holdright DR, Duncan JS. Cardiac arrhythmias in focal epilepsy: a prospective long-term study. Lancet. 2004;364(9452):2212-2219. doi:10.1016/S0140-6736(04)17594-6
  11. Thirunavu V, Du R, Wu JY, Berg AT, Lam SK. The role of surgery in the management of Lennox–Gastaut syndrome: a systematic review and meta-analysis of the clinical evidence. Epilepsia. 2021;62(4):888-907. doi:10.1111/epi.16851
  12. Orthopedist. Cleveland Clinic. Accessed February 27, 2023.
  13. Mollaoğlu M, Bolayir E. Injuries in patients with epilepsy and some factors associated with injury. Noro Psikiyatr Ars. 2013;50(3):269-273. doi:10.4274/npa.y6632
  14. Yen WW, Falik N, Passfall LG, et al. Management of lower extremity orthopaedic injuries in epileptic patients: a systematic review. J Orthop. 2021;25:278-282. doi:10.1016/j.jor.2021.05.005
  15. Yen WW, Cloud GW, Wasserburg JR, et al. A systematic review of the management of upper extremity orthopaedic injuries in epileptic patients. Arch Bone Jt Surg. 2022;10(4):301-310. doi:10.22038/ABJS.2021.56488.2803
  16. Genetic testing in LGS. LGS Foundation. Accessed February 27, 2023.
  17. Cherian KA. Lennox-Gastaut syndrome: practice essentials. Medscape. Updated August 6, 2020. Accessed February 27, 2023.
  18. Eisensehr I, Parrino L, Noachtar S, Smerieri A, Terzano MG. Sleep in Lennox–Gastaut syndrome: the role of the cyclic alternating pattern (CAP) in the gate control of clinical seizures and generalized polyspikes. Epilepsy Res. 2001;46(3):241-250. doi:10.1016/S0920-1211(01)00280-7
  19. Ferlazzo E, Nikaronova M, Italiano D, et al. Lennox–Gastaut syndrome in adulthood: clinical and EEG features. Epilepsy Res. 2010;89(2):271-277. doi:10.1016/j.eplepsyres.2010.01.012
  20. Schwartz K, Lastra AC, Balabanov AJ. Obstructive and central sleep apnoea in a patient with medically intractable epilepsy. BMJ Case Rep. 2022;15(9):e245564. doi:10.1136/bcr-2021-245564
  21. Neuropsychological evaluation FAQ. UNC School of Medicine Department of Neurology. Accessed February 27, 2023.
  22. Caraballo RH, Fortini S, Fresler S, et al. Ketogenic diet in patients with Lennox–Gastaut syndrome. Seizure. 2014;23(9):751-755. doi:10.1016/j.seizure.2014.06.005
  23. 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. doi:10.3389/fneur.2017.00505
  24. How is LGS treated? LGS Foundation. Accessed February 27, 2023.
  25. Speech therapy mandate? Administrators and IEPs. Global Teletherapy. Accessed February 27, 2023.
  26. Na JH, Kim HD, Lee YM. Effective and safe diet therapies for Lennox-Gastaut syndrome with mitochondrial dysfunction. Ther Adv Neurol Disord. 2020;13:1756286419897813. doi:10.1177/1756286419897813
  27. Lee S, Baek MS, Lee YM. Lennox-Gastaut syndrome in mitochondrial disease. Yonsei Med J. 2019;60(1):106-114. doi:10.3349/ymj.2019.60.1.106
  28. Almannai M, Al Mahmoud RA, Mekki M, El-Hattab AW. Metabolic seizures. Front Neurol. 2021;12:640371. doi.10.3389/fneur.2021.640371
  29. Ostendorf AP, Ng YT. Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions. Neuropsychiatr Dis Treat. 2017;13:1131-1140. doi:10.2147/NDT.S115996

Reviewed by Harshi Dhingra, MD, on 2/27/2023.

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