Comparison of incidence and prevalence rate of patients with amyotrophic lateral sclerosis (ALS) between various countries
| Study location | Race | Data source | Study design | Diagnostic criteria (for incidence) | Year | Number of new patients with ALS | Incidence* (per 105) | Prevalence* (per 105) |
| HKSAR10 | Chinese | CMS (clinical management system) | Hospital-based (multicentre) | MND on the revised EEC+ICD-9 code 335.2, 335.20–335.24 | 1997–2002 | 98 | 0.60† | 3.04† |
| Japan21 | Japanese | Nationwide mail survey | Population-based | Definite, probable or possible ALS based on the revised EEC | 2009 | 2264 | 2.2 | 9.9 |
| Taiwan9 | Chinese | Health insurance | Population-based | Repeated claim with ICD-9 code 335.20 (amyotrophic lateral sclerosis) either outpatient or inpatient | 2007 | 338 | 0.51 | 1.97 |
| Ireland24‡ | Caucasian | Ireland‡ MND registers | Population-based | Definite, probable, possible and suspected MND/ALS using the original EEC | 2004–2005 | 109 | 1.89§ | 4.99 |
| England25 | European origin/African origin | The South East England ALS (SEALS) | Population-based | Data derived from SEALS register | 2002–2008 | 88 | 1.97§ ¶ ** 1.35§ ** †† | 5.79¶ ¶ 4.94 †† |
| Netherlands26 | Caucasian | Population-based registry | Population-based | Definite, probable, possible or suspected ALS according to the EEC+PMA‡‡, PLS§§, PBP¶¶ | 2006–2009 | 1217 | 2.77§ | 10.32 |
| France27 | Caucasian | Health insurance | Population-based | At least one delivery of riluzole or hospitalisation for MND (claim with ICD-10 code G122) | 2012–2014 | 6553 | 3.32§ 2.72§ ** | NA |
| Italy22 | Caucasian | PARALS*** | Community-based Cohort study | Definite, probable or possible laboratory-supported ALS based on the revised EEC (EEC before 2000) | 1995–2014 | 2702 | 3.03 2.78** | 10.54 |
| Southern Germany23 | Caucasian | The ALS registry Swabia | Community-based cohort study | Definite, probable or possible ALS based on the revised EEC (cases comprise the ICD-10 code G12.2) | 2008–2010 | 438 | 2.5† § | NA |
| Worldwide12 | All races | From 44 studies | Meta-analysis | 13 146 | 1.75§ 1.68† § | NA | ||
| South Korea (current study) | Korean | Health insurance | Population-based | First service claim with KCD-6 code G12.21 (amyotrophic lateral sclerosis) | 2011–2015 | 3049 | 1.20 | 3.43 |
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.