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Vol. 30, No. 2, 2008
Issue release date: April 2008
Neuroepidemiology 2008;30:105–111

Incidence and Prevalence of Amyotrophic Lateral Sclerosis in Uruguay: A Population-Based Study

Vázquez M.C. · Ketzoián C. · Legnani C. · Rega I. · Sánchez N. · Perna A. · Penela M. · Aguirrezábal X. · Druet-Cabanac M. · Medici M.
aInstituto de Neurología, Hospital de Clínicas, Facultad de Medicina, Universidad de la Republica, bHospital de Clínicas, Montevideo, Uruguay; cInstitute of Neurological Epidemiology and Tropical Neurology, Faculty of Medicine, Limoges, France

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Objective: To determine the incidence and prevalence of amyotrophic lateral sclerosis (ALS) in the Republic of Uruguay.Methods: The study was performed in Uruguay (3,241,003 inhabitants) during a 2-year period (2002–2003). To ensure complete case ascertainment, multiple sources of information were used, including all the neurologists, other medical specialties, general physicians, neurophysiology laboratories, hospital medical records and death certificates. ALS diagnosis was based on El Escorial criteria. Although all patients with motor neuron disease were enrolled in the follow-up, only probable and definite cases are included in the study. Results: Between January 1st, 2002, and December 31st, 2003, 89 new patients were diagnosed with probable or definite ALS. The mean annual incidence rate was 1.37 per 100,000 persons. The incidence was higher for men (1.95) than for women (0.84) with a male to female ratio of 2:1. For both, the incidence increased throughout the years showing a peak in the 65–74 age group among men and the 55–64 age group among women. Mean age at onset of ALS disease was 58.7 years. The estimated mean annual incidence for ALS calculated by the capture recapture method was 1.42 (95% CI, 1.13–1.72). On December 31st, 2002, the crude prevalence was 1.9 per 100,000 inhabitants. Conclusions: ALS incidence is within a narrow range across countries despite the genetic, environmental and socioeconomic differences when similar prospective design, diagnosis criteria and data analyses are applied.

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  1. Fong KY, Yu YL, Chan YW, Kay R, Chan J, Yang Z, Kwan MC, Leung KP, Li PCK, Lam TH, Cheung RTF: Motor neuron disease in Hong Kong Chinese: epidemiology and clinical picture. Neuroepidemiology 1996;15:239–245.
  2. Preux PM, Druet-Cabanat M, Couratier P, Debrock C, Marcharia W, Dumas M: Estimation of the amyotrophic lateral sclerosis incidence by capture-recapture method in the Limousin region of France. J Clin Epidemiol 2000;53:1025–1029.
  3. Olivares L, Esteban ES, Alter M: Mexican ‘resistance’ to amyotrophic lateral sclerosis. Arch Neurol 1972;27:397–402.
  4. Gunnarsson LG, Palm R: Motor neuron disease and heavy manual labour: an epidemiologic survey of Varmland county, Sweden. Neuroepidemiology 1984;3:195–206.

    External Resources

  5. Chancellor AM, Warlow CP: Adult onset motor neuron disease: worldwide mortality, incidence and distribution since 1950. J Neurol Neurosurg Psychiatry 1992;55:1106–1115.
  6. Roman GC: Neuroepidemiology of amyotrophic lateral sclerosis: clues to aetiology and pathogenesis. J Neurol Neurosurg Psychiatry 1996;61:131–137.
  7. Worms PM: The epidemiology of motor neuron diseases: a review of recent studies. J Neurol Sci 2001;191:3–9.
  8. Neilson 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;64:201–216.
  9. Neilson S, Robinson I, Alperovitch A: Rising amyotrophic lateral sclerosis mortality in France, 1968–1990: increased life expectancy and interdisease competition as an explanation. J Neurol 1994;241:448–455.
  10. Armon C: Motor neuron disease; in Gorelick PB, Alter M (eds): Handbook of Neuroepidemiology. New York, Marcel Dekker, 1994, pp 407–454.
  11. 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.
  12. 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.
  13. Logroscino G, Beghi E, Zoccolella S, PalaganoR, Fraddosio A, Simone IL, Lamberti P, Lepore V, 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.
  14. Beghi E, Millul A, Micheli A, Vitelli E, Logroscino G: Incidence of ALS in Lombardy, Italy. Neurology 2007;68:141–145.
  15. 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.

    External Resources

  16. McGuire V, Longstreth WT, Koepsell TD, van Belle G: Incidence of amyotrophic lateral sclerosis in three counties in western Washington state. Neurology 1996;47:571–573.
  17. World Federation of Neurology Subcommittee on Motor Neuron Disease: El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 1994;124(suppl):98–107.
  18. Instituto Nacional de Estadística (INE): Censo fase I 2004. Anuario estadístico. Montevideo, INE, 2005.
  19. Hook EB, Regal RR: Capture-recapture methods in epidemiology: methods and limitations. Epidemiol Rev 1995;17:243–264.
  20. Sakamoto Y, Ishiguro M, Kitigawa G: Akaike Information Criterion statistics. Tokyo, KTK Scientific, 1986.
  21. Schwarz G: Estimating the dimension of a model. Ann Stat 1978;6:461–464.

    External Resources

  22. Draper D: Assessment and propagation of a model uncertainty. J R Stat Soc 1995;57:78–79.
  23. United States Census Bureau: 1990 Census of United States. Accessed February, 2006.
  24. Chiò A: Update on ISIS survey: Europe, North America and South America. Amyotroph Lateral Scler Other Motor Neuron Disord 2000;(suppl 1):S9–S11.

    External Resources

  25. Kahana E, Zilber N: Changes in incidence of amyotrophic lateral sclerosis in Israel. Arch Neurol 1984;41:157–160.
  26. Yoshida S, Mulder DW, Kurland LT, Chu C-P, Okazaki H: Follow-up study on amyotrophic lateral sclerosis in Rochester, Minnesota, 1925 through 1984. Neuroepidemiology 1986;5:61–70.
  27. Gunnarsson L-G, Lygner P-E, Viega-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.
  28. Hojer-Pedersen E, Christensen PB, Jensen NB: Incidence and prevalence of motor neuron disease in two Danish counties. Neuroepidemiology 1989;8:151–159.
  29. Guidetti D, Bondavalli M, Sabadini R, Marcello N, Vinceti M, Cavalletti S, Marbini A, Gemignani F, Colombo A, Ferrari A, Vivoli G, Solimè 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.
  30. Sorenson EJ, Stalker AP, Kurland LT, Windebank AJ: Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925 to 1998. Neurology 2002;59:280–282.
  31. De Pedro-Cuesta J, Litvan I: Epidemiology of motor neuron disease; in Anderson DW, Schoenberg DG (eds): Neuroepidemiology: a Tribute to Bruce Schoenberg. Boca Raton, CRC Press, 1991.

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