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

Grace Bailey Orcid Logo, 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: Grace Bailey Orcid Logo, 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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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 (&#x2265; 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</abstract><type>Journal Article</type><journal>European Journal of Neurology</journal><volume>29</volume><journalNumber>1</journalNumber><paginationStart/><paginationEnd/><publisher>Wiley</publisher><placeOfPublication/><isbnPrint/><isbnElectronic/><issnPrint>1351-5101</issnPrint><issnElectronic>1468-1331</issnElectronic><keywords>dystonia, incidence, mortality, prevalence, socioeconomic factors</keywords><publishedDay>15</publishedDay><publishedMonth>10</publishedMonth><publishedYear>2021</publishedYear><publishedDate>2021-10-15</publishedDate><doi>10.1111/ene.15114</doi><url/><notes/><college>COLLEGE NANME</college><department>Medical School</department><CollegeCode>COLLEGE CODE</CollegeCode><DepartmentCode>MEDS</DepartmentCode><institution>Swansea University</institution><apcterm>Another institution paid the OA fee</apcterm><funders>European Social Fund; KESS 2. 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spelling 2022-01-04T14:59:33.3669367 v2 58005 2021-09-22 Adult‐onset idiopathic dystonia: a national data‐linkage study to determine epidemiological, social deprivation and mortality characteristics 1e09a407fca9e8047e7738b18d381130 0000-0003-4646-3134 Grace Bailey Grace Bailey true false 46366e9ea4e8840d8c216e76c2f85204 NULL Anna Rawlings Anna Rawlings true true f569591e1bfb0e405b8091f99fec45d3 0000-0002-5853-4625 Fatemeh Torabi Fatemeh Torabi true false 1c3044b5ff7a6552ff5e8c9e3901c807 0000-0003-4396-5657 Owen Pickrell Owen Pickrell true false 2021-09-22 MEDS 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 Journal Article European Journal of Neurology 29 1 Wiley 1351-5101 1468-1331 dystonia, incidence, mortality, prevalence, socioeconomic factors 15 10 2021 2021-10-15 10.1111/ene.15114 COLLEGE NANME Medical School COLLEGE CODE MEDS Swansea University Another institution paid the OA fee 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 2022-01-04T14:59:33.3669367 2021-09-22T09:20:30.4087909 Faculty of Medicine, Health and Life Sciences Swansea University Medical School - Medicine Grace Bailey 0000-0003-4646-3134 1 Grace A Bailey 2 Anna Rawlings NULL 3 Fatemeh Torabi 0000-0002-5853-4625 4 Owen Pickrell 0000-0003-4396-5657 5 Kathryn J Peall 6 58005__21313__bd542ffa804146cdbf9897c4d5ef8ca4.pdf 58005.2Aw.pdf 2021-10-27T12:48:05.6960687 Output 2003582 application/pdf Version of Record true This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. true eng https://creativecommons.org/licenses/by/4.0/
title Adult‐onset idiopathic dystonia: a national data‐linkage study to determine epidemiological, social deprivation and mortality characteristics
spellingShingle Adult‐onset idiopathic dystonia: a national data‐linkage study to determine epidemiological, social deprivation and mortality characteristics
Grace Bailey
Anna Rawlings
Fatemeh Torabi
Owen Pickrell
title_short Adult‐onset idiopathic dystonia: a national data‐linkage study to determine epidemiological, social deprivation and mortality characteristics
title_full Adult‐onset idiopathic dystonia: a national data‐linkage study to determine epidemiological, social deprivation and mortality characteristics
title_fullStr Adult‐onset idiopathic dystonia: a national data‐linkage study to determine epidemiological, social deprivation and mortality characteristics
title_full_unstemmed Adult‐onset idiopathic dystonia: a national data‐linkage study to determine epidemiological, social deprivation and mortality characteristics
title_sort Adult‐onset idiopathic dystonia: a national data‐linkage study to determine epidemiological, social deprivation and mortality characteristics
author_id_str_mv 1e09a407fca9e8047e7738b18d381130
46366e9ea4e8840d8c216e76c2f85204
f569591e1bfb0e405b8091f99fec45d3
1c3044b5ff7a6552ff5e8c9e3901c807
author_id_fullname_str_mv 1e09a407fca9e8047e7738b18d381130_***_Grace Bailey
46366e9ea4e8840d8c216e76c2f85204_***_Anna Rawlings
f569591e1bfb0e405b8091f99fec45d3_***_Fatemeh Torabi
1c3044b5ff7a6552ff5e8c9e3901c807_***_Owen Pickrell
author Grace Bailey
Anna Rawlings
Fatemeh Torabi
Owen Pickrell
author2 Grace Bailey
Grace A Bailey
Anna Rawlings
Fatemeh Torabi
Owen Pickrell
Kathryn J Peall
format Journal article
container_title European Journal of Neurology
container_volume 29
container_issue 1
publishDate 2021
institution Swansea University
issn 1351-5101
1468-1331
doi_str_mv 10.1111/ene.15114
publisher Wiley
college_str Faculty of Medicine, Health and Life Sciences
hierarchytype
hierarchy_top_id facultyofmedicinehealthandlifesciences
hierarchy_top_title Faculty of Medicine, Health and Life Sciences
hierarchy_parent_id facultyofmedicinehealthandlifesciences
hierarchy_parent_title Faculty of Medicine, Health and Life Sciences
department_str Swansea University Medical School - Medicine{{{_:::_}}}Faculty of Medicine, Health and Life Sciences{{{_:::_}}}Swansea University Medical School - Medicine
document_store_str 1
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description 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
published_date 2021-10-15T20:05:24Z
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