Abstract

BackgroundThere is a lack of studies of Huntington’s disease (HD) in immigrants.ObjectiveTo study the association between country of birth and incident HD in first-generation immigrants versus Swedish-born individuals and in second-generation immigrants versus Swedish-born individuals with Swedish-born parents.MethodsStudy populations included all adults aged 18 years and older in Sweden, i.e., in the first-generation study 6,042,891 individuals with 1034 HD cases and in the second-generation study 4,860,469 individuals with 1001 cases. HD was defined as having at least one registered diagnosis of HD in the National Patient Register. The incidence of HD in different first-generation immigrant groups versus Swedish-born individuals was assessed by Cox regression, expressed as hazard ratios (HRs) and 95% confidence intervals (CI). The models were stratified by sex and adjusted for age, geographical residence in Sweden, educational level, marital status, and neighborhood socioeconomic status.ResultsMean age-standardized incidence rates per 100,000 person-years were for all Swedish-born 0.82 and for all foreign born 0.53 and for all men 0.73 and for all women 0.81, with the highest incidence rates for the group 80–84 years of age. After adjusting for potential confounders, the HRs were lower in women in the first- and second-generation, i.e., 0.49 (95% CI 0.36–0.67) and 0.63 (95% 0.45–0.87), respectively, and also among women from Finland or with parents from Finland.SignificanceIn general, the risk of HD was lower in first-generation and second-generation immigrant women but not among male immigrants.

Highlights

  • Huntington’s disease (HD) is an autosomal dominant inherited neurodegenerative disease first described in 1872

  • The nationwide registers used in the present study were the Total Population Register and the National Patient Register (NPR)

  • A diagnosis of HD from the NPR was registered at the age 18 years and above

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Summary

Introduction

Huntington’s disease (HD) is an autosomal dominant inherited neurodegenerative disease first described in 1872. The disease is caused by an expanded CAG repeat within the Huntington’s gene on chromosome 4 [2, 3], with a mean onset of symptoms at around 40 years of age, but with earlier as well as later onset [1]. In Caucasian populations, prevalence rate is estimated to 5.7 per 100,000 in Europe, North America, and Oceania [5] and up to 10 per 100,000 in Western Europe, North America, and Australia [4]. The prevalence rate among men and women in this study was similar, with a mean average prevalence in 1990 and 2010 of men 9.4 and of women 10.4 per 100,000 and with the highest prevalence in the ages 51–60 years [6]. There is a lack of studies of Huntington’s disease (HD) in immigrants

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