High Prevalence of Dystonia in the Faroe Islands: A Population-Based Study

Abstract

Background: There are fewer than 5 population-based studies of dystonia worldwide. Only one utilized a movement disorders neurologist. Given the potential for founder effects, and the highly genetic nature of dystonia, the Faroe Islands provide a particularly interesting setting to study the prevalence of dystonia.

Objective: To estimate the prevalence of dystonia.

Methods: We used a 2-phase, population-based design, screening 1,334 randomly selected Faroese individuals aged ≥40 years from which a subsample of 227 participated in an in-person clinical evaluation. Dystonia was assessed by 2 movement disorder neurologists using videotaped examinations.

Results: Two of 227 (0.88%, 95% CI -0.33 to 2.09%) were diagnosed with cervical or segmental dystonia. An unusual form of thumb flexion dystonia was noted in 75 more, yielding a combined prevalence of 33.92% (95% CI 27.73-40.11%).

Conclusions: The prevalence of cervical or segmental dystonia was as high as in one prior population-based study using similar methods. Furthermore, an unusual form of thumb flexion dystonia was uncovered, which yielded an extraordinarily high prevalence of dystonia in this population. Although our methods likely contributed to more complete capture of subtle dystonia, founder effects are highly likely to have been an additional major contributor to these findings.

Keywords: Dystonia; Faroe Islands; Prevalence; Two-phase population-based design.