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.

在法罗群岛，在地理上与世隔绝的岛屿 Suðuroy 上观察到一组肌萎缩侧索硬化症 (ALS)。本研究旨在估计法罗群岛中家族性 ALS (fALS) 的发生率，包括 43 名被诊断患有 ALS 的患者。fALS 患者是通过医疗记录和法罗群岛多代登记册确定的。首先，当发生在一级或二级亲属之间时，fALS 会被识别。其次，通过系谱（3代内≥3例）识别出患有fALS的家庭和个人。与全国患病率相比，Suðuroy 的 ALS 患病率高出 3 倍。 与散发性 ALS 相比，fALS的发生率至少为 14%（n = 6），并且 fALS 的平均生存时间短 1.7 年（p = 0.01。SD = 0.5，范围 1.0–2.2）。这项研究表明家族聚集性超过了 ALS 的预期，并支持法罗群岛 ALS 的遗传贡献，尽管不能排除家庭内的环境暴露。