Amyotrophic lateral sclerosis in the Faroe Islands: a genealogical study

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

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)

Abstract

In the Faroe Islands, a clustering of amyotrophic lateral sclerosis (ALS) was observed on the geographically isolated island, Suðuroy. 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 Suðuroy 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.
Original languageEnglish
Pages (from-to)1-6
Number of pages6
JournalAmyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Volume[ahead of print]
DOIs
Publication statusPublished - 4 Sep 2020

Keywords

  • Genealogical database
  • pedigree
  • familial ALS
  • genetics
  • sclerosis
  • Faroe Islands

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