ALS Consortium of Epidemiologic Studies (ACES)–

Descriptive Epidemiology

Epidemiology of Sporadic ALS

The incidence of ALS in the United States and most European countries ranges from 1.5-2.5 ALS cases per 100,000 population members per year (1-9). Internationally, the incidence rates for ALS or motor neuron disease (MND) worldwide range from 0.86 to 2.5/100,000/year (10-25). Because almost all patients with ALS die of their disease, mortality rates for ALS should parallel incidence rates. However, reported mortality rates for ALS in the United States are lower than incidence rates (approximately 1.5/100,000/year for all ages combined compared to incidence rates of 2/100,000/year), possibly due of underreporting of ALS on death certificates. Recent studies report higher incidence and mortality rates for MND than were previously reported in industrialized countries (4, 8, 26-29). This increase may be accounted for by improved ascertainment, better reporting and loss of competing causes of mortality in a susceptible cohort (30-32). Few studies have focused on the prevalence of ALS. With a median survival of three years, the prevalence of ALS is approximately 5 per 100,000, or nearly three times the incidence rate of 2 per 100,000 per year (6, 8, 13). Most epidemiologic studies report incidence rates among men that are 20-60% higher than incidence rates among women (5-7, 9, 33), although in the most recent studies, the differences in male and female incidence rates are less striking. Age-specific incidence rates of ALS increase with age, peaking at ages 65-74 years for both men and women (1-2, 5-9, 34). In referral-center based studies, the age distribution of ALS peaks earlier at 55-60 years (11-12), probably due to a form of selection bias where younger onset patients with ALS are more likely than older onset patients to seek the specialty care that ALS referral centers can provide. Studies suggest that ALS incidence and mortality may be lower among African, Asian, and Hispanic Americans than among non-Hispanic Caucasians (12, 21, 35). However, the degree of case ascertainment among African-Americans and Hispanics may be less than complete and may result in underestimation of the ALS incidence in these groups. Further research is needed to obtain estimates of the incidence of ALS in larger studies that can be conducted in multiracial populations with uniform access to medical care.
Beghi et al. (36) noted the importance of population-based registries, which could provide answers to unanswered questions regarding the descriptive epidemiology of ALS, including whether the incidence of ALS is increasing among women and whether there is an increasing temporal trend of ALS. To this end, the European ALS Consortium (EURALS) was established in 2004 as a consortium of population-based registries from four countries and clinic-based cohorts from six countries, with the promise of identifying 500 new cases each year for study.

       Epidemiology of Sporadic ALS References:

  1. Yoshida S, Mulder DW, Kurland LT, Chu CP, Okazaki H. Follow-up study on amyotrophic lateral sclerosis in Rochester, Minnesota, 1925-1984. Neuroepidemiology 1986;5:61-70.
  2. Sorenson EJ, Stalker AP, Kurland LT, Windebank AJ. Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925 to 1998. Neurology 2002;59:280-282.
  3. Kurtzke JF. Which "neurodegenerative diseases" are on the rise? Health Environ Digest 1989;3:3-8. Article referenced in SciCentral
  4. Lilienfeld DE, Perl DP. Projected neurodegenerative disease mortality in the United States, 1990-2040. Neuroepidemiology 1993;12:219-228.
  5. McGuire V, Longstreth WT Jr, Koepsell TD, van Belle G. Incidence of amyotrophic lateral sclerosis in three counties in western Washington State. Neurology 1996;47:571-573.
  6. Traynor BJ, Codd MB, Corr B, Forde C, Frost E, Hardiman O. Incidence and prevalence of ALS in Ireland, 1995-1997: a population-based study. Neurology 1999;52:504-509.
  7. Piemonte and Valle d'Aosta Register for Amyotrophic Lateral Sclerosis (PARALS). Incidence of ALS in Italy: evidence for a uniform frequency in Western countries. Neurology 2001;56:239-244.
  8. Mandrioli J, Faglioni P, Merelli E, Sola P. The epidemiology of ALS in Modena, Italy. Neurology 2003;60:683-689.
  9. Logroscino G, Zoccolella S, Palagano R, Fraddosio A, Simone IL, Lamberti P, Lepore B, Serlenga L, the SLAP registry. Incidence of amyotrophic lateral sclerosis in southern Italy: a population based study. J Neurol Neurosurg Psychiatry 2005;76;1094-1098.
  10. Armon C, Kurland LT. Classic and western Pacific amyotrophic lateral sclerosis: epidemiologic comparisons. In: Hudson AJ, ed. Amyotrophic lateral sclerosis: Concepts in Pathogenesis and Etiology. Toronto: University of Toronto Press, 1989:144-165. Article referenced in SciCentral
  11. Gunnarsson L-G, Lindberg G, Soderfeldt B, Axelson O. Amyotrophic lateral sclerosis in Sweden in relation to occupation. Acta Neurol Scand 1991 ;83:394-398.
  12. Kurtzke JF. Risk factors in amyotrophic lateral sclerosis. Adv in Neurol 1991;56:245-270.
  13. Chancellor AM, Warlow CP. Adult onset motor neuron disease: worldwide mortality, incidence and distribution since 1950. J Neurol Neurosurg Psychiatry 1992;55:1106-1115.
  14. Kahana E, Zilber N. Changes in incidence of amyotrophic lateral sclerosis in Israel. Arch Neurol 1984;41:157-160.
  15. Rosati G, Pinna L, Granieri E, Aiello I, Tola R, Agnetti V, Pirisi A, de Bastiani P. Studies on epidemiological, clinical, and etiological aspects of ALS disease in Sardinia, Southern Italy. Acta Neurol Scand 1977;55:231-244.
  16. Forsgren L, Almay BG, Holmgren G, Wall S. Epidemiology of motor neuron disease in northern Sweden. Acta Neurol Scand 1983;68:20-29.
  17. Gunnarsson LG, Palm R. Motor neuron disease and heavy manual labor: an epidemiologic survey of Varmland County, Sweden. Neuroepidemiology 1984;3:195-206. Article referenced in SciCentral
  18. Hojer-Pedersen E, Christensen PB, Jensen NB. Incidence and prevalence of motor neuron disease in two Danish counties. Neuroepidemiology 1989;8:151-159.
  19. Murros K, Fogelholm R. Amyotrophic lateral sclerosis in Middle-Finland: an epidemiological study. Acta Neurol Scand 1983;67:41-47.
  20. Hudson AJ, Davenport A, Hader WJ. The incidence of amyotrophic lateral sclerosis in southwestern Ontario, Canada. Neurology 1986;36:1524-1528.
  21. Annegers JF, Appel S, Lee JR, Perkins P. Incidence and prevalence of amyotrophic lateral sclerosis in Harris County Texas, 1985-1988. Arch Neurol 1991;48:589-593.
  22. Scottish Motor Neuron Disease Research Group. The Scottish motor neuron disease register: a prospective study of adult onset motor neuron disease in Scotland. Methodology, demography and clinical features of incident cases in 1989. J Neurol Neurosurg Psychiatry 1992;55:536-541.
  23. Chiņ A, Tribolo A, Oddenino E, Schiffer. A cross-sectional and cohort study of motor neuron disease in Piedmont, Italy. In: Clifford Rose F, ed. New Evidence in MND/ALS Research. London: Smith-Gordon, 1991:59-62. Article referenced in SciCentral
  24. Guidetti D, Bondavalli M, Sabadini R, Marcello N, Vinceti M, Cavalletti S, Marbini A, Gemignani F, Colombo A, Ferrari A, Vivoli G, Solime F. Epidemiological survey of amyotrophic lateral sclerosis in the province of Reggio Emilia, Italy: influence of environmental exposure to lead. Neuroepidemiology 1996;15:301-312.
  25. Gunnarsson LG, Lygner PE, Veiga-Cabo J, de Pedro-Cuesta J. An epidemic-like cluster of motor neuron disease in a Swedish county during the period 1973-1984. Neuroepidemiology 1996;15:142-152.
  26. Seljeseth YM, Vollset SE, Tysnes OB. Increasing mortality from amyotrophic lateral sclerosis in Norway? Neurology 2000;55:1262-1266.
  27. Maasilta P, Jokelainen M, Loytonen, M Sabel CE, Gatrell AC. Mortality from amyotrophic lateral sclerosis in Finland, 1986-1995. Acta Neurol Scand 2001;104:232-235.
  28. Noonan CW, White MC, Thurman D, Wong LY. Temporal and geographic variation in United States motor neuron disease mortality, 1969-1998. Neurology 2005;64;7:1215-1221.
  29. Sejvar JJ, Holman RC, Bresee JS, Kochanek KD, Schonberger LB. Amyotrophic lateral sclerosis mortality in the United States, 1979-2001. Neuroepidemiology 2005;25;3:144-152.
  30. Gompertz B. On the nature of the function expressive of the law of human mortality. Philos Trans R Soc London 1825;115:513-585. Article referenced in SciCentral
  31. Riggs JE. Longitudinal Gompertzian analysis of amyotrophic lateral sclerosis mortality in the U.S., 1977-1986: evidence for an inherently susceptible population subset. Mech Ageing Dev 1990;55:207-220.
  32. Nielson S, Robinson I, Hunter M. Longitudinal Gompertzian analysis of ALS mortality in England and Wales, 1963-1989: estimates of susceptibility in the general population. Mech Ageing Dev 1992,64:210-216.
  33. Armon C. Motor neuron disease. In: Gorelick PB, Alter M, eds. Handbook of Neuroepidemiology. New York: Marcel Dekker, Inc., 1994:407-456. Article referenced in SciCentral
  34. Christensen PB, Hojer-Pedersen E, Jensen NB. Survival of patients with amyotrophic lateral sclerosis in two Danish counties. Neurology 1990;40:600-604.
  35. Leone M, Chandra V, Schoenberg BS. Motor neuron disease in the United States, 1971 and 1973-1978: patterns of mortality and associated conditions at the time of death. Neurology 1987;37:1339-1343.
  36. Beghi E, Logroscino G, Chio A, Hardiman O, Mitchell D, Swingler R, Traynor BJ, EURALS Consortium. The epidemiology of ALS and the role of population-based registries. Biochim Biophys Acta 2006;1762:1150-1157.

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