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. 2023 Mar 1;18:42. doi: 10.1186/s13023-023-02626-4

Table 3.

Epidemiology: Incidence/prevalence and mortality in patients with LGS

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

CI confidence interval; CPRD UK Clinical Practice Research Datalink; GKV German population covered by statutory health insurance (Gesetzliche Krankenversicherung; GKV); NR not reported

*Converted to per 100,000 people