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 journal › Journal article › Research › peer-review
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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