Table 6

Comparison of incidence and prevalence rate of patients with amyotrophic lateral sclerosis (ALS) between various countries

Study locationRaceData sourceStudy designDiagnostic criteria
(for incidence)
YearNumber of
new patients with ALS
(per 105)
(per 105)
HKSAR10 ChineseCMS (clinical management system)Hospital-based
MND on the revised EEC+ICD-9 code 335.2, 335.20–335.241997–2002980.60†3.04†
Japan21 JapaneseNationwide mail surveyPopulation-basedDefinite, probable or possible ALS based on the revised EEC200922642.29.9
Taiwan9 ChineseHealth insurancePopulation-basedRepeated claim with ICD-9 code 335.20 (amyotrophic lateral sclerosis) either outpatient or inpatient20073380.511.97
MND registers
Population-basedDefinite, probable, possible and suspected MND/ALS using the original EEC2004–20051091.89§4.99
England25 European origin/African originThe South East England ALS (SEALS)Population-basedData derived from SEALS register2002–2008881.97§ ¶ **
1.35§ ** ††
5.79¶ ¶
4.94 ††
Netherlands26 CaucasianPopulation-based registryPopulation-basedDefinite, probable, possible or suspected ALS according to the EEC+PMA‡‡, PLS§§, PBP¶¶2006–200912172.77§10.32
France27 CaucasianHealth insurancePopulation-basedAt least one delivery of riluzole or hospitalisation for MND (claim with ICD-10 code G122)2012–201465533.32§
2.72§ **
Italy22 CaucasianPARALS***Community-based
Cohort study
Definite, probable or possible laboratory-supported ALS based on the revised EEC (EEC before 2000)1995–201427023.03
CaucasianThe ALS registry SwabiaCommunity-based
cohort study
Definite, probable or possible ALS based on the revised EEC (cases comprise the ICD-10 code G12.2)2008–20104382.5† §NA
Worldwide12 All racesFrom 44 studiesMeta-analysis13 1461.75§
1.68† §
South Korea
(current study)
KoreanHealth insurancePopulation-basedFirst service claim with KCD-6 code G12.21 (amyotrophic lateral sclerosis)2011–201530491.203.43
  • MND, motor neuron disease; EEC, El Escorial criteria; NA, not applicable.

  • *Crude rate.

  • †Age-standardised incidence rates.

  • ‡Both Northern Ireland and the Republic of Ireland.

  • §Per 100 000 person-years.

  • ¶European ancestry group live in inner city London.

  • **The age-adjusted and sex-adjusted incidence rate.

  • ††African ancestry group live in inner city London.

  • ‡‡Progressive muscular atrophy.

  • §§Primary lateral sclerosis.

  • ¶¶Progressive bulbar palsy.

  • ***The Piemonte and Valle d’Aosta Register for ALS.