Amyotrophic lateral sclerosis in the Faroe Islands – a genealogical study

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

doi:10.1080/21678421.2020.1813311

Cited by 6 publications

(5 citation statements)

References 10 publications

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“…Studies on the incidence of ALS and the identification of geographical clusters have taken place in many Western countries, including the USA ( Taylor and Davis, 1989 ; Sienko et al, 1990 ; Turabelidze et al, 2008 ; Caller et al, 2009 ; Reddy, 2020 ), Italy ( Giagheddu et al, 1983 , 1993 ; Grainieri et al, 1989 ; Uccelli et al, 2007 ), Sweden ( Gunnarsson et al, 1996 ), Finland ( Sabel et al, 2003 ), Denmark ( Johansen et al, 2021 ), United Kingdom ( Mitchell et al, 1998 ; Scott et al, 2009 ), Greece ( Kalfakis et al, 1991 ), and France ( Boumédiène et al, 2011 ; Lagrange et al, 2021b ). While an aggregation of patients in a given geographic area theoretically may occur by chance or be the consequence of a statistical bias in the process of patient selection ( Malaspina et al, 2002 ), their intensive study has the potential of discovering important environmental associations, as demonstrated by the experience with Western Pacific ALS.…”

Section: Resultsmentioning

confidence: 99%

“…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: Denmark Sweden and Finlandmentioning

confidence: 99%

“…While mutations are suspected and often found in familial ALS, the operation of culpable trans-generational environmental exposures cannot be excluded ( Johansen et al, 2021 ). For example, in the case of the former ultra-high incidence of familial and sporadic ALS among the Chamorro people of Guam, where the disorder was first thought to result from a dominantly inherited genetic trait ( Plato et al, 1969 ), disease rates fell steadily in the second half of the twentieth century such that the high incidence had essentially disappeared ( Garruto et al, 1985 ; Chen, 1995 ).…”

Section: Introductionmentioning

confidence: 99%

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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%

Section: Denmark Sweden and Finlandmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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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“…16 We have previously reported on a geographical cluster of ALS in the Faroe Islands, where the occurrence of the disease was three times higher on the isolated island of Suðuroy compared to the rest of the islands. On the same note, the rate of familial ALS in the Faroe Islands is, depending on defining criteria, 17 between 14 and 28%, 18 which is relatively high. A next step would be to look into the genetic risk factors of ALS in the Faroes.…”

Section: Discussionmentioning

confidence: 98%

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

Johansen

Svenstrup

Joensen

et al. 2022

Ann Clin Transl Neurol

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The Faroese population isolate harbors epidemiological and genetic characteristics that likely differ from outbred populations. This population-based register study found that the Faroese 2010-2020 crude incidence of amyotrophic lateral sclerosis (ALS) was 4.9/100,000 person-years (95% confidence interval [CI], 3.3-7.0) and the age-and sex-standardized incidence (US 2010 Census Population) was 4.1/100,000 person-years (95% CI, 2.7-6.0), which is a 68% increase from the 1987-2009 estimate. The 2020 crude prevalence was 9.5/100,000 (95% CI, 3.0-19.6) in a population of 52,912 inhabitants. Incidence and prevalence estimates of ALS in the Faroes are high and further research is warranted to uncover the genetic or environmental determinants of ALS in this population.

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“…It is associated with a degeneration of the neuromuscular junctions that lead to skeletal muscle atrophy ( 2 ), difficulty performing voluntary movements, decreased motor autonomy and impaired oral communication, swallowing and breathing ( 3 ). It occurs in adults aged between 55 and 65, with peak incidence between 50 and 75 ( 4 ), although cases have been identified in patients younger than 25 ( 5 ). There is a crude prevalence and incidence of ALS worldwide of 4.42 (95% CI 3.92–4.96) per 100,000 population and 1.59 (95% CI 1.39–1.81) per 100,000 person-years, respectively ( 6 ).…”

Section: Introductionmentioning

confidence: 99%

Effect of the Mediterranean diet supplemented with nicotinamide riboside and pterostilbene and/or coconut oil on anthropometric variables in amyotrophic lateral sclerosis. A pilot study

Carrera-Juliá,

Estrela,

Zacarés

et al. 2023

Front. Nutr.

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Amyotrophic Lateral Sclerosis (ALS) is a chronic and progressive neurodegenerative disease that causes the death of motor neurons and alters patients’ body composition. Supplementation with the antioxidants nicotinamide riboside (NR) and pterostilbene (PTER) can combat associated oxidative stress. Additionally, coconut oil is an alternative energy substrate that can address mitochondrial dysfunction. The aim of the present study is to assess the impact of a Mediterranean Diet supplemented with NR and PTER and/or with coconut oil on the anthropometric variables of patients with ALS. A prospective, mixed, randomized, analytical and experimental pilot study in humans was performed through a clinical trial (registered with ClinicalTrials.gov under number NCT03489200) with pre- and post-intervention assessments. The sample was made up of 40 subjects categorized into four study groups (Control, Antioxidants, Coconut oil, and Antioxidants + Coconut oil). Pre- and post-intervention anthropometric assessments were carried out to determine the following data: weight, percentage of fat and muscle mass, skinfolds, body perimeters, Body Mass Index (BMI), Waste-to-Hip Index (WHI) and Waist-Height Ratio (WHR). Compared to the Control group, GAx significantly increased muscle mass percentage and decreased fat mass percentage, triceps, iliac crest, and abdominal skinfolds. GCoco significantly increased muscle mass percentage and decreased fat mass percentage, subscapular skinfolds, and abdominal skinfolds. GAx + coco significantly increased muscle mass percentage and decreased abdominal skinfolds. Therefore, our results suggest that the Mediterranean Diet supplemented with NR and PTER and the Mediterranean Diet supplemented with coconut oil (ketogenic diet) are the two nutritional interventions that have reported the greatest benefits, at anthropometric level.

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Amyotrophic lateral sclerosis in the Faroe Islands – a genealogical study

Cited by 6 publications

References 10 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

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

Effect of the Mediterranean diet supplemented with nicotinamide riboside and pterostilbene and/or coconut oil on anthropometric variables in amyotrophic lateral sclerosis. A pilot study

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