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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, Pages: 91 - 104

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 purposeAccurate 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 mortalit...

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Published in: European Journal of Neurology
ISSN: 1351-5101 1468-1331
Published: Wiley 2022
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URI: https://cronfa.swan.ac.uk/Record/cronfa58972
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fullrecord <?xml version="1.0"?><rfc1807><datestamp>2021-12-31T16:52:49.6587400</datestamp><bib-version>v2</bib-version><id>58972</id><entry>2021-12-08</entry><title>Adult&#x2010;onset idiopathic dystonia: A national data&#x2010;linkage study to determine epidemiological, social deprivation, and mortality characteristics</title><swanseaauthors><author><sid>46366e9ea4e8840d8c216e76c2f85204</sid><ORCID>NULL</ORCID><firstname>Anna</firstname><surname>Rawlings</surname><name>Anna Rawlings</name><active>true</active><ethesisStudent>true</ethesisStudent></author><author><sid>f569591e1bfb0e405b8091f99fec45d3</sid><ORCID>0000-0002-5853-4625</ORCID><firstname>Fatemeh</firstname><surname>Torabi</surname><name>Fatemeh Torabi</name><active>true</active><ethesisStudent>false</ethesisStudent></author><author><sid>1c3044b5ff7a6552ff5e8c9e3901c807</sid><ORCID>0000-0003-4396-5657</ORCID><firstname>Owen</firstname><surname>Pickrell</surname><name>Owen Pickrell</name><active>true</active><ethesisStudent>false</ethesisStudent></author></swanseaauthors><date>2021-12-08</date><abstract>Background and purposeAccurate 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.MethodsA 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.ResultsThe 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.ConclusionsWe 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.</abstract><type>Journal Article</type><journal>European Journal of Neurology</journal><volume>29</volume><journalNumber>1</journalNumber><paginationStart>91</paginationStart><paginationEnd>104</paginationEnd><publisher>Wiley</publisher><placeOfPublication/><isbnPrint/><isbnElectronic/><issnPrint>1351-5101</issnPrint><issnElectronic>1468-1331</issnElectronic><keywords>dystonia; incidence; mortality; prevalence; socioeconomic factors</keywords><publishedDay>1</publishedDay><publishedMonth>1</publishedMonth><publishedYear>2022</publishedYear><publishedDate>2022-01-01</publishedDate><doi>10.1111/ene.15114</doi><url/><notes/><college>COLLEGE NANME</college><department>Biosciences</department><CollegeCode>COLLEGE CODE</CollegeCode><institution>Swansea University</institution><apcterm/><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. 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spelling 2021-12-31T16:52:49.6587400 v2 58972 2021-12-08 Adult‐onset idiopathic dystonia: A national data‐linkage study to determine epidemiological, social deprivation, and mortality characteristics 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 2021-12-08 Background and purposeAccurate 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.MethodsA 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.ResultsThe 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.ConclusionsWe 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. Journal Article European Journal of Neurology 29 1 91 104 Wiley 1351-5101 1468-1331 dystonia; incidence; mortality; prevalence; socioeconomic factors 1 1 2022 2022-01-01 10.1111/ene.15114 COLLEGE NANME Biosciences COLLEGE CODE Swansea University 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 2021-12-31T16:52:49.6587400 2021-12-08T12:32:51.3957815 Faculty of Medicine, Health and Life Sciences Swansea University Medical School - Medicine Grace A. Bailey 1 Anna Rawlings NULL 2 Fatemeh Torabi 0000-0002-5853-4625 3 Owen Pickrell 0000-0003-4396-5657 4 Kathryn J. Peall 5 58972__21982__e82d1409596f4263be49189df51ff84b.pdf 58972.pdf 2021-12-31T16:49:23.4351072 Output 1075073 application/pdf Version of Record true © 2021 The Authors. This is an open access article under the terms of the Creative Commons Attribution License true eng http://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
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 46366e9ea4e8840d8c216e76c2f85204
f569591e1bfb0e405b8091f99fec45d3
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author_id_fullname_str_mv 46366e9ea4e8840d8c216e76c2f85204_***_Anna Rawlings
f569591e1bfb0e405b8091f99fec45d3_***_Fatemeh Torabi
1c3044b5ff7a6552ff5e8c9e3901c807_***_Owen Pickrell
author Anna Rawlings
Fatemeh Torabi
Owen Pickrell
author2 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
container_start_page 91
publishDate 2022
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 purposeAccurate 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.MethodsA 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.ResultsThe 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.ConclusionsWe 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.
published_date 2022-01-01T04:15:55Z
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