Objective:

To examine mortality rates and patient-related characteristics in patients with Lennox-Gastaut Syndrome (LGS) or Dravet Syndrome (DS) using US claims data.

Background:

Patients with LGS or DS are at risk of premature mortality. Large mortality studies in DS and LGS are limited.

Design/Methods:

This retrospective study used the Komodo database from 1/1/2015–12/31/2024. Eligible patients had: ≥2 LGS (ICD-10, G40.81) or DS (ICD-10, G40.83) claims ≥1 month apart in patient qualification period (1/1/2015–12/31/2023), and 12 months of pre-index data. The index date was 1/1/2018 or the second LGS or DS claim date (if claim date ≥30 days after first claim), whichever occurred later. Study exit date was date of death, last recorded claim, or study end date. The primary endpoint evaluated LGS and DS mortality rates per 1000 person-years (PY) and standardized mortality ratios (SMRs), stratified by age and healthcare resource utilization (HCRU) severity score.

Results:

Overall, 33,404 and 2781 patients with LGS and DS were identified, respectively. Mortality rates (95% CI) in LGS and DS: 14.2 (13.6; 14.8) and 7.3 (5.6; 9.4) per 1000 PY, respectively; SMRs (95% CI): 7.5 (7.2; 7.8) and 7.8 (6.0; 10.1), respectively. In LGS, pediatric (<18 years; n=16,984) and adult (≥18 years; n=16,420) mortality rates: 12.1 (11.3; 12.9) and 16.3 (15.4; 17.2) per 1000 PY, respectively. In DS, pediatric (n=2045) and adult (n=736) mortality rates: 6.7 (4.8; 9.1) and 9.1 (5.5; 14.2) per 1000 PY, respectively. In LGS and DS, SMRs were higher in pediatric patients vs adults. Higher mortality rates and SMRs were observed in patients with higher HCRU severity scores. In combined LGS and DS populations, Cox models indicated significant associations with clinical and demographic characteristics and mortality.

Conclusions:

Patients with LGS or DS are at an increased risk of mortality, especially in younger patients and those with severe disease.