High Prevalence of Dystonia in the Faroe Islands: A Population-Based Study.
Dystonia
Faroe Islands
Prevalence
Two-phase population-based design
Journal
Neuroepidemiology
ISSN: 1423-0208
Titre abrégé: Neuroepidemiology
Pays: Switzerland
ID NLM: 8218700
Informations de publication
Date de publication:
2019
2019
Historique:
received:
25
06
2019
accepted:
27
07
2019
pubmed:
21
8
2019
medline:
9
6
2020
entrez:
21
8
2019
Statut:
ppublish
Résumé
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. To estimate the prevalence of dystonia. 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. 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%). 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.
Sections du résumé
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.
Identifiants
pubmed: 31430749
pii: 000502455
doi: 10.1159/000502455
pmc: PMC6881520
mid: NIHMS1045710
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
220-224Subventions
Organisme : NINDS NIH HHS
ID : R01 NS039422
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS094607
Pays : United States
Informations de copyright
© 2019 S. Karger AG, Basel.
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