Journal
EUROPEAN JOURNAL OF NEUROLOGY
Volume 29, Issue 1, Pages 91-104Publisher
WILEY
DOI: 10.1111/ene.15114
Keywords
dystonia; incidence; mortality; prevalence; socioeconomic factors
Categories
Funding
- KESS2
- European Social Fund
- Cardiff University
- HDR UK - UK Medical Research Council, Engineering and Physical Sciences Research Council [HDR-9006]
- Economic and Social Research Council
- Department of Health and Social Care (England)
- Chief Scientist Office of the Scottish Government Health and Social Care Directorates
- Health and Social Care Research and Development Division (Welsh Government)
- Public Health Agency (Northern Ireland)
- British Heart Foundation
- Wellcome Trust
- Brain Repair and Intracranial Neurotherapeutics (BRAIN) Unit Infrastructure Award [UA05]
- Welsh Government through Health and Care Research Wales
- MRC [MR/P008593/1]
- MRC [MR/P008593/1] Funding Source: UKRI
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The study identified epidemiological, social deprivation, and mortality characteristics of adult-onset idiopathic dystonia in the Welsh population using a case-ascertainment algorithm. The results showed an increasing trend in prevalence and incidence rates, with individuals with dystonia having a life expectancy similar to the general Welsh population.
Background and purpose Accurate epidemiological information is essential for the improved understanding of dystonia syndromes, as well as better provisioning of clinical services and providing context for diagnostic decision-making. Here, we determine epidemiological, social deprivation, and mortality characteristics of adult-onset idiopathic dystonia in the Welsh population. Methods A retrospective population-based cohort study using anonymized electronic health care data in Wales was conducted to identify individuals with dystonia between 1 January 1994 and 31 December 2017. We developed a case-ascertainment algorithm to determine dystonia incidence and prevalence, as well as characterization of the dystonia cohort, based on social deprivation and mortality. Results The case-ascertainment algorithm (79% sensitivity) identified 54,966 cases; of these cases, 41,660 had adult-onset idiopathic dystonia (>= 20 years). Amongst the adult-onset form, the median age at diagnosis was 41 years, with males significantly older at time of diagnosis compared to females. Prevalence rates ranged from 0.02% in 1994 to 1.2% in 2017. The average annual incidence was 87.7/100,000/year, increasing from 49.9/100,000/year (1994) to 96.21/100,000/year (2017). In 2017, people with dystonia had a similar life expectancy to the Welsh population. Conclusions We have developed a case-ascertainment algorithm, supported by the introduction of a neurologist-reviewed validation cohort, providing a platform for future population-based dystonia studies. We have established robust population-level prevalence and incidence values for adult-onset idiopathic forms of dystonia, with this reflecting increasing clinical recognition and identification of causal genes. Underlying causes of death mirrored those of the general population, including circulatory disorders, respiratory disorders, cancers, and dementia.
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