Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study

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Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study. / Johansen, Malan; Svenstrup, Kirsten; Mortensen, Olavur; Andorsdottir, Guorio; Steig, Bjarni A.; Joensen, Poul; Hansen, Torben; Petersen, Maria Skaalum.

In: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Vol. 22, No. 7-8, 2021, p. 571-575.

Research output: Contribution to journalJournal articleResearchpeer-review

Harvard

Johansen, M, Svenstrup, K, Mortensen, O, Andorsdottir, G, Steig, BA, Joensen, P, Hansen, T & Petersen, MS 2021, 'Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study', Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, vol. 22, no. 7-8, pp. 571-575. https://doi.org/10.1080/21678421.2020.1813311

APA

Johansen, M., Svenstrup, K., Mortensen, O., Andorsdottir, G., Steig, B. A., Joensen, P., Hansen, T., & Petersen, M. S. (2021). Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 22(7-8), 571-575. https://doi.org/10.1080/21678421.2020.1813311

Vancouver

Johansen M, Svenstrup K, Mortensen O, Andorsdottir G, Steig BA, Joensen P et al. Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2021;22(7-8):571-575. https://doi.org/10.1080/21678421.2020.1813311

Author

Johansen, Malan ; Svenstrup, Kirsten ; Mortensen, Olavur ; Andorsdottir, Guorio ; Steig, Bjarni A. ; Joensen, Poul ; Hansen, Torben ; Petersen, Maria Skaalum. / Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study. In: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2021 ; Vol. 22, No. 7-8. pp. 571-575.

Bibtex

@article{fd7309b95c0e40678d0709e6f98af3ad,
title = "Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study",
abstract = "In the Faroe Islands, a clustering of amyotrophic lateral sclerosis (ALS) was observed on the geographically isolated island, Suouroy. This study aims to estimate the frequency of familial ALS (fALS) in the Faroes including 43 patients diagnosed with ALS. Patients with fALS were identified through medical records and the Faroese Multi Generation Register. Firstly, fALS was recognized when occurring between first- or second-degree relatives. Secondly, families and individuals with fALS were recognized through pedigrees (>= 3 cases within 3 generations). The prevalence of ALS was 3 times higher in Suouroy compared to the nationwide prevalence. The frequency of fALS was at least 14% (n = 6) and mean survival time was 1.7 years shorter for fALS compared to sporatic ALS (p = 0.01. SD = 0.5, range 1.0-2.2). This study is suggestive of familial clustering in excess of expected for ALS and supports a genetic contribution to ALS in the Faroe Islands albeit environmental exposure within families cannot be excluded.",
keywords = "Genealogical database, pedigree, familial ALS, genetics, ALS",
author = "Malan Johansen and Kirsten Svenstrup and Olavur Mortensen and Guorio Andorsdottir and Steig, {Bjarni A.} and Poul Joensen and Torben Hansen and Petersen, {Maria Skaalum}",
year = "2021",
doi = "10.1080/21678421.2020.1813311",
language = "English",
volume = "22",
pages = "571--575",
journal = "Amyotrophic Lateral Sclerosis",
issn = "2167-8421",
publisher = "Taylor & Francis",
number = "7-8",

}

RIS

TY - JOUR

T1 - Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study

AU - Johansen, Malan

AU - Svenstrup, Kirsten

AU - Mortensen, Olavur

AU - Andorsdottir, Guorio

AU - Steig, Bjarni A.

AU - Joensen, Poul

AU - Hansen, Torben

AU - Petersen, Maria Skaalum

PY - 2021

Y1 - 2021

N2 - In the Faroe Islands, a clustering of amyotrophic lateral sclerosis (ALS) was observed on the geographically isolated island, Suouroy. This study aims to estimate the frequency of familial ALS (fALS) in the Faroes including 43 patients diagnosed with ALS. Patients with fALS were identified through medical records and the Faroese Multi Generation Register. Firstly, fALS was recognized when occurring between first- or second-degree relatives. Secondly, families and individuals with fALS were recognized through pedigrees (>= 3 cases within 3 generations). The prevalence of ALS was 3 times higher in Suouroy compared to the nationwide prevalence. The frequency of fALS was at least 14% (n = 6) and mean survival time was 1.7 years shorter for fALS compared to sporatic ALS (p = 0.01. SD = 0.5, range 1.0-2.2). This study is suggestive of familial clustering in excess of expected for ALS and supports a genetic contribution to ALS in the Faroe Islands albeit environmental exposure within families cannot be excluded.

AB - In the Faroe Islands, a clustering of amyotrophic lateral sclerosis (ALS) was observed on the geographically isolated island, Suouroy. This study aims to estimate the frequency of familial ALS (fALS) in the Faroes including 43 patients diagnosed with ALS. Patients with fALS were identified through medical records and the Faroese Multi Generation Register. Firstly, fALS was recognized when occurring between first- or second-degree relatives. Secondly, families and individuals with fALS were recognized through pedigrees (>= 3 cases within 3 generations). The prevalence of ALS was 3 times higher in Suouroy compared to the nationwide prevalence. The frequency of fALS was at least 14% (n = 6) and mean survival time was 1.7 years shorter for fALS compared to sporatic ALS (p = 0.01. SD = 0.5, range 1.0-2.2). This study is suggestive of familial clustering in excess of expected for ALS and supports a genetic contribution to ALS in the Faroe Islands albeit environmental exposure within families cannot be excluded.

KW - Genealogical database

KW - pedigree

KW - familial ALS

KW - genetics

KW - ALS

U2 - 10.1080/21678421.2020.1813311

DO - 10.1080/21678421.2020.1813311

M3 - Journal article

C2 - 32885668

VL - 22

SP - 571

EP - 575

JO - Amyotrophic Lateral Sclerosis

JF - Amyotrophic Lateral Sclerosis

SN - 2167-8421

IS - 7-8

ER -

ID: 250071789