Table 3 Epidemiology: Incidence/prevalence and mortality in patients with LGS
From: The burden of illness in Lennox–Gastaut syndrome: a systematic literature review
Study | Country | Study type | Year | Population (n) | Incidence/prevalence | Mortality | |
|---|---|---|---|---|---|---|---|
Parameter | Value | ||||||
Piña-Garza 2017 [46] (Full) | USA | Retrospective claims analysis | 2012/13 | Probable LGS patients N=14,712 | Proportion of epilepsy patients with probable LGS (10-year age groups) | 8.4% at age 10 decreasing to 2% in the 60-year-old cohort | NR |
Strzelczyk 2021 [47] (Full) | Germany | Retrospective claims analysis | 2016 | Broad LGS N=545 Narrow LGS N=102 | Prevalence (standardized to German GKV population) | Broad LGS: 39.2 per 100,000 people Narrow LGS: 6.5 per 100,000 people | Broad LGS: 10.01% [157 events] Narrow LGS: 2.88% [six events] (p < 0.001 vs control) Control: 0.01% [one event] |
Chin 2021 [40] (Full) | UK | Retrospective analysis of electronic medical records | 2017 | Confirmed LGS: N=74 for prevalence Probable LGS: N=106 for prevalence Full cohort: N=180 for prevalence; N=122 for mortality | Prevalence (standardized to UK CPRD population) | Confirmed LGS: 2.89 per 100,000 people* Probable LGS: 4.20 per 100,000 people* Full cohort: 5.78 per 100,000 people* | Crude mortality rate per 1000 person-years: Confirmed LGS: 6.12 [11 events] Probable LGS: 4.17 [7 events] Full cohort: 18 events |
Hollenack 2019 [41] (Abstract) | USA | Retrospective claims analysis | 2016/ 2017 | Probable LGS: Commercial: N=2273 Medicaid: N=4786 | Prevalence | Commercial: 13.0 per 100,000 people* Medicaid: 60.8 per 100,000 people* | NR |
Trevathan 1997 [44] MADDS study (Full) | USA | Population-based, cross-sectional study | 1975–1977 | LGS: N=23 | Prevalence of LGS at age 10 years Proportion of epilepsy patients with LGS | 26 per 100,000 people* 4% of epilepsy patients | NR |
Autry 2010 [48] MADDS study (Full) | Original cohort: 1975–1977 Follow-up: 1975 to 2001 | LGS: N=34 | NR | NR | Mortality ratio (95% CI): 13.92 (7.19-24.31) for LGS vs 3.11 (2.39-3.98) for all epilepsy | ||
Sidenvall 1996 [43] (Full) | Northern Sweden | Observational | 1985 | LGS: N=9 | Prevalence N (%) of epilepsy patients with LGS | 20 per 100,000 people* n=9 (5.8%) of all epilepsy cases | NR |
Rantala 1999 [42] (Full) | Finland | Retrospective | 1976–1993 | LGS: N=25 | Prevalence | 28 per 100,000 people (95% CI: 18–41)* | NR |
Annual incidence | 1.93 per 100,000 children <15 years (95% CI: 1.25-2.85/100,000) | ||||||
Heiskala 1997 [45] (Full) | Finland | Retrospective | 1975–1985 | LGS (broad definition): N=75 LGS children 0–14 years: N=NR | Annual incidence | Broad definition: 2.1 per 100,000 people Children 0–14 years: 1.9 per 100,000 | NR |
Beilmann 1999 [39] (Full) | Estonia | Observational | 1997 | LGS: N=NR | Prevalence | 10 per 100,000 people* | NR |