June 29, 2020 e-Pearl of the Week: Lennox gastaut syndrome (LGS)

Lennox gastaut syndrome (LGS)

Lennox gastaut syndrome (LGS) presents with multiple seizures types—tonic, atonic, atypical absence—and intellectual disability. EEG shows slow-spike-and-wave (<2.5Hz),1 which may normalize by adulthood, and generalized paroxysmal fast activity during tonic seizures. LGS comprises 2–5% of childhood epilepsies with peak incidence at 3–5 years.2 Symptomatic LGS (70–80%) results from multiple etiologies such as cortical dysplasia, congenital infections, or genetic mutations (CHD2, SCN2A). 10–30% of cases evolve from West syndrome, which is characterized by infantile spasms, hypsarrhythmia, intellectual disability.3 Therapy includes valproate, epidiolex, rufinamide, clobazam,4 ketogenic diet, corpus callosotomy, and VNS.5 Remission is rare6 with 3–7% mortality.

References

  1. Markand ON. Lennox-Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol 2003;20:426–441.
  2. Archer JS, Warren AEL, Jackson GD, Abbott DF. Conceptualizing Lennox–Gastaut syndrome as a secondary network epilepsy. Front Neurol 2014;5:225.
  3. Glauser TA. Following catastrophic epilepsy patients from childhood to adulthood. Epilepsia 2004;45 Suppl 5:23–26.
  4. Gresham J, Eiland LS, Chung AM. Treating Lennox-Gastaut syndrome in epileptic pediatric patients with third-generation rufinamide. Neuropsychiatr Dis Treat 2010;6:639–645.
  5. Freeman, John M. (February 2009). Seizures, EEG events, and the ketogenic diet. Epilepsia 2009;50:329–330.
  6. Hancock EC, Cross JH. Treatment of Lennox-Gastaut Syndrome. Cochrane Database Syst Rev 2013(2):CD0032.

Submitted by Tanay Satarkar, Medical Graduate, Bukovinian State Medical University (Chernivtsi, Ukraine) and Faisal Khan, MD, DABSM, DABPN, Consultant Neurologist, Sugar Land Neurology and Sleep (Sugar Land, Texas)

Tanay Satarkar and Dr. Khan report no disclosures.