Objectives: The establishment of variations in the incidence of amyotrophic lateral sclerosis (ALS) in the Faroese population from that found in other general populations may point to risk factors for the development of this disease among the Faroese. The aim of this study was to estimate the annual incidence of ALS during the period 1987-2009 and to compare the occurrence of ALS in the Faroe Islands with data from three European countries.
Method: All Faroese patients diagnosed with ALS in this period are documented in the current longitudinal prospective study.
Results: The incidence of ALS in the Faroe Islands during the period 1987-2009 is 2.6 (1.7-3.7) per 100,000 annually. The results yielded no strong evidence of a difference (P = 0.09) in the incidence of ALS between Faroe Islands and Europe. The sample population is small, and this, of course, impacts the statistical precision of the findings.
Conclusion: The data clearly suggest, however, that the Faroese population is probably not subject to an increased risk of ALS, even though certain risk factors are present in the general population: (i) a fish-based diet contaminated with mercury and polychlorinated biphenyl; (ii) the high occurrence of the recessive carnitine transporter genetic defect; and (iii) the anticipated high degree of inbreeding at the fifth generation.
© 2011 John Wiley & Sons A/S.