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

Grace A Bailey, Anna Rawlings Orcid Logo, Fatemeh Torabi Orcid Logo, Owen Pickrell Orcid Logo, Kathryn J Peall

European Journal of Neurology, Volume: 29, Issue: 1

Swansea University Authors: Anna Rawlings Orcid Logo, Fatemeh Torabi Orcid Logo, Owen Pickrell Orcid Logo

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DOI (Published version): 10.1111/ene.15114

Abstract

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 mort...

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Published in: European Journal of Neurology
ISSN: 1351-5101 1468-1331
Published: Wiley 2021
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URI: https://cronfa.swan.ac.uk/Record/cronfa58005
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Abstract: 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
Keywords: dystonia, incidence, mortality, prevalence, socioeconomic factors
College: Swansea University Medical School
Funders: European Social Fund; KESS 2. Grant Number: 517008; Public Health Agency of Northern Ireland; Wellcome Trust; Cardiff University; Health Data Research UK Ltd. Grant Number: HDR-9006; Health and Social Care Research and Development Division (Welsh Government); Cheif Scientist Office of the Scottish Government Health and Social Care Directorates; Department of Health and Social Care; Medical Research Council. Grant Number: MR/P008593/1; Health and Care Research Wales; Brain Repair and Intracranial Neurotherapeutics. Grant Number: UA05; Engineering and Physical Sciences Research Council; Economic and Social Research Council; British Heart Foundation
Issue: 1