Quantifying the burden of disease in patients with Lennox Gastaut syndrome

Jochem K H Spoor; Teresa Greco; Marcel A Kamp; Silvia Faini; Christian Senft; Maxine Dibué

doi:10.1016/j.ebr.2021.100508

Quantifying the burden of disease in patients with Lennox Gastaut syndrome

Epilepsy Behav Rep. 2021 Nov 25:16:100508. doi: 10.1016/j.ebr.2021.100508. eCollection 2021.

Authors

Jochem K H Spoor¹, Teresa Greco², Marcel A Kamp³, Silvia Faini², Christian Senft³, Maxine Dibué^{3

4}

Affiliations

¹ Department of Neurosurgery, Erasmus University, Dr. Molewaterplein 40, 3015 GD, Rotterdam, Netherlands.
² Statistics and Data Management, LIVANOVA - SORIN Group Italia S.r.l., Via Benigno Crespi, 17, 20159 Milano, Italy.
³ Department of Neurosurgery, Medical Faculty, Friedrich Schiller University, Fürstengraben 1, 07743 Jena, Germany.
⁴ Medical Affairs International, Neuromodulation, LivaNova PLC, 20 Eastbourne Terrace, London W2 6LG United Kingdom.

Abstract

Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy but there is limited literature characterizing the disease burden despite this being crucial for disease management strategies, and for designing and interpreting clinical trials. We searched the Vagus Nerve Stimulation (VNS) Therapy Patient Outcome Registry including over 7000 patients with drug‑resistant epilepsy (DRE). Propensity Score Matching (PSM) matched LGS-DRE patients and non-LGS-DRE patients and frequencies of individual seizure types were assessed. The PSM population included 705 and 1410 DRE patients with and without LGS. 40% of the LGS-DRE group had polypharmacy with 3 antiseizure medications (ASM) while 42% in non-LGS-DRE had polypharmacy with 2 ASMs. Median total monthly seizure frequency was over double in the LGS group: 90 (IQR, 28-312) versus 40 (IQR, 10-150); p < 0.001. This analysis suggests that seizure frequency in LGS patients who later receive VNS is more than double than in non-LGS DRE patients with mostly bilateral tonic-clonic seizures contributing to this difference. Furthermore, ASM burden with poorer seizure control may be greater in LGS patients, however data collection ceased in 2003 and therefore does not take recent ASMs approved for LGS into account. This analysis offers quantitative insight into the burden of disease in patients with LGS.

Keywords: ASM, antiseizure medication; Drug-resistant epilepsy; Epileptic encephalopathy; LGS, Lennon-Gastaut Syndrome; Lennox–Gastaut Syndrome; Seizure types; VNS; VNS, vagus nerve stimulation; Vagus nerve stimulation.