High incidence of amyotrophic lateral sclerosis in the Faroe Islands 2010–2020

Johansen, Malan; Svenstrup, Kirsten; Joensen, Poul; Steig, Bjarni á; Andorsdóttir, Guðrið; Hansen, Torben; Petersen, Maria Skaalum

doi:10.1002/acn3.51501

Cited by 7 publications

(4 citation statements)

References 21 publications

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“…The incidence and prevalence of ALS is high in the Danish Faroe Islands ( Johansen et al, 2022 ), particularly the southernmost island of Suðuroy, where the prevalence is three times higher than the nationwide prevalence ( Johansen et al, 2021 ). While familial clustering (14%) was in excess of that expected for ALS on Suðuroy, a result suggestive of genetic contribution, environmental factors were not excluded or sought.…”

Section: Resultsmentioning

confidence: 99%

Early-onset, conjugal, twin-discordant, and clusters of sporadic ALS: Pathway to discovery of etiology via lifetime exposome research

et al. 2023

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The identity and role of environmental factors in the etiology of sporadic amyotrophic lateral sclerosis (sALS) is poorly understood outside of three former high-incidence foci of Western Pacific ALS and a hotspot of sALS in the French Alps. In both instances, there is a strong association with exposure to DNA-damaging (genotoxic) chemicals years or decades prior to clinical onset of motor neuron disease. In light of this recent understanding, we discuss published geographic clusters of ALS, conjugal cases, single-affected twins, and young-onset cases in relation to their demographic, geographic and environmental associations but also whether, in theory, there was the possibility of exposure to genotoxic chemicals of natural or synthetic origin. Special opportunities to test for such exposures in sALS exist in southeast France, northwest Italy, Finland, the U.S. East North Central States, and in the U.S. Air Force and Space Force. Given the degree and timing of exposure to an environmental trigger of ALS may be related to the age at which the disease is expressed, research should focus on the lifetime exposome (from conception to clinical onset) of young sALS cases. Multidisciplinary research of this type may lead to the identification of ALS causation, mechanism, and primary prevention, as well as to early detection of impending ALS and pre-clinical treatment to slow development of this fatal neurological disease.

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Section: Resultsmentioning

confidence: 99%

Early-onset, conjugal, twin-discordant, and clusters of sporadic ALS: Pathway to discovery of etiology via lifetime exposome research

et al. 2023

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“…Faroese diet has traditionally contained high amounts of seafood, sheep and potatoes but recently has shifted to a more western‐style diet 5 . The Faroese population is genetically homogeneous as a consequence of being a geographically isolated island group, leading to an increased rate of some diseases amongst the Faroese, 6‐10 while other diseases are less common, for example, Huntington disease has until now not been found in persons of Faroese origin.…”

Section: Introductionmentioning

confidence: 99%

“…4 Faroese diet has traditionally contained high amounts of seafood, sheep and potatoes but recently has shifted to a more western-style diet. 5 The Faroese population is genetically homogeneous as a consequence of being a geographically isolated island group, leading to an increased rate of some diseases amongst the Faroese, [6][7][8][9][10] while other diseases are less common, for example, Huntington disease has until now not been found in persons of Until now, there has been no systematic reporting of cancer survival in the Faroe Islands, apart from estimations for singular cancer entities. 13 Previously, survival estimations have not been performed due to incomplete data on follow-up and missing cases in the cancer registry.…”

Section: Introductionmentioning

confidence: 99%

Cancer survival in the Faroe Islands over the last 50 years compared to the other Nordic countries

Kristiansen

Mikkelsen

Kristiansdóttir

et al. 2023

Intl Journal of Cancer

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As sustained development in cancer treatment protocols have led to improved survival in most areas of the world, surveillance is needed to ensure that small populations follow suit. Our study reports age‐standardized relative cancer survival in the Faroe Islands compared to the other Nordic countries. We present 1‐ and 5‐year survival estimates and corresponding 95% confidence intervals for the Faroe Islands and compare them with estimates for the Nordic countries. The data for this article has been obtained through the NORDCAN collaboration (2019 data). Age‐standardized relative survival was estimated using shared R codes on individual‐level data within each country. Ten‐year calendar inclusion periods were used in addition to the usual 5‐year calendar periods to include cancer sites with few cases, which is especially beneficial to the smaller populations. The primary findings were that 1‐ and 5‐year survival were consistently lower in the Faroes for the summary group all sites but non‐melanoma skin cancer for both women and men. Further, 5‐year survival was lower for women with ovarian cancer and men with lung cancer than in other Nordic countries. Previously, breast cancer survival was low in the Faroes but has improved to a comparable level over the last few years. Colorectal cancer survival was relatively high for both sexes. The reported estimates in this article call for further research to investigate the cancers with lower survival and should call for actions to improve the survival of Faroese cancer patients.

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“…In contrast to the extreme variation in the whole world, the incidence is relatively homogeneous in studies from North America, New Zealand and Europe. Outside of ALS clusters reported in the past like in Guam or Kiji peninsula [ 22 , 23 ], the highest reported ALS incidence so far has been recently reported from Faroe island [ 24 ▪ ], a small archipelago of 18 islands with a population of about 50 000 people: 4.9/100 000 in the period 2010–2020 raising from 2.6 in the period 1987–2000. This almost 70% increase has been attributed to possible environmental factors as seafood, including whale meat and blubber, components of the traditional Faroese diet.…”

Section: Introductionmentioning

confidence: 99%

The challenge of amyotrophic lateral sclerosis descriptive epidemiology: to estimate low incidence rates across complex phenotypes in different geographic areas

Logroscino

Urso

Tortelli

2022

Current Opinion in Neurology

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Purpose of review Amyotrophic lateral sclerosis (ALS) is a rare progressive neurodegenerative disease of motor neurons with a fatal outcome. The rareness of the disease and the rapidly fatal course are the main challenges for the ALS epidemiological research. The understanding of ALS has clearly advanced in the recent years both in the genetics and in the leading pathways of disease determinants. Epidemiological research has played a primary role in these discoveries. Recent findingsEpidemiological studies have shown a variation of incidence, mortality and prevalence of ALS between geographical areas and different populations, supporting the notion that genetic factors, linked to populations ancestries, along with environmental and lifestyle factors, play a significant role in the occurrence of the disease. The burden of motor neuron diseases is increasing and currently more relevant in high-income countries but increasing at the highest rate in low and middle-income countries. The ALS phenotype is not restricted to motor functions. C9orf72 repeat expansion seems to present a recognizable phenotype characterized by earlier disease onset, the presence of cognitive and behavioural impairment.

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High incidence of amyotrophic lateral sclerosis in the Faroe Islands 2010–2020

Cited by 7 publications

References 21 publications

Early-onset, conjugal, twin-discordant, and clusters of sporadic ALS: Pathway to discovery of etiology via lifetime exposome research

Early-onset, conjugal, twin-discordant, and clusters of sporadic ALS: Pathway to discovery of etiology via lifetime exposome research

Cancer survival in the Faroe Islands over the last 50 years compared to the other Nordic countries

The challenge of amyotrophic lateral sclerosis descriptive epidemiology: to estimate low incidence rates across complex phenotypes in different geographic areas

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