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Adult-onset idiopathic dystonia: a national data-linkage study to determine epidemiological, social deprivation and mortality characteristics

Bailey, Grace, Rawlings, Anna, Torabi, Fatemeh, Pickrell, Owen and Peall, Kathryn ORCID: 2022. Adult-onset idiopathic dystonia: a national data-linkage study to determine epidemiological, social deprivation and mortality characteristics. European Journal of Neurology 29 (1) , pp. 91-104. 10.1111/ene.15114

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Background and Objectives 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 anonymised electronic healthcare data in Wales was conducted to identify individuals with dystonia between January 1st 1994 and December 31st 2017. We developed a case-ascertainment algorithm to determine dystonia incidence and prevalence, as well as characterisation 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. Discussion 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 that of the general population, including circulatory disorders, respiratory disorders, cancers or dementia.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
MRC Centre for Neuropsychiatric Genetics and Genomics (CNGG)
Additional Information: This is an open access article under the terms of the Creative Commons Attribution License
Publisher: Wiley
ISSN: 1351-5101
Date of First Compliant Deposit: 20 September 2021
Date of Acceptance: 14 September 2021
Last Modified: 08 May 2023 21:30

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