Amyotrophic lateral sclerosis in the Faroe Islands - a genealogical study

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 m...

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Bibliographic Details
Published in:Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Main Authors: Johansen, Malan, Svenstrup, Kirsten, Mortensen, Olavur, Andorsdottir, Guorio, Steig, Bjarni A., Joensen, Poul, Hansen, Torben, Petersen, Maria Skaalum
Format: Article in Journal/Newspaper
Language:English
Published: 2021
Subjects:
Genealogical database
pedigree
familial ALS
genetics
ALS
Faroe Islands
Faroes
Online Access:https://curis.ku.dk/portal/da/publications/amyotrophic-lateral-sclerosis-in-the-faroe-islands--a-genealogical-study(fd7309b9-5c0e-4067-8d07-09e6f98af3ad).html
https://doi.org/10.1080/21678421.2020.1813311
Description
Summary: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.

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