ArticleEuropean journal of neurology2022
Adult-onset idiopathic dystonia: A national data-linkage study to determine epidemiological, social deprivation, and mortality characteristics.
Article in European journal of neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 22 citations in OpenAlex.
- Structural magnetic resonance imaging in dystonia: A systematic review of methodological approaches and findings.European journal of neurology · 2022Pooled it
- Developmental, Neuroanatomical and Cellular Expression of Genes Causing Dystonia.Annals of clinical and translational neurology · 2026Article
- The Gut Microbiota Regulates Motor Deficits via Butyrate in a GnalAdvanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Transcriptomic disruption and hypoactivity in DYT-SGCE medial ganglionic eminence-patterned inhibitory neurons.Brain : a journal of neurology · 2026Article
- Video-based quantification of hand postural tremor without external references. Integrating postural tremor quantification into visionMD.NPJ Parkinson's disease · 2025Article
- Non-Motor Symptoms and Health-Related Quality of Life in Patients with Isolated Dystonia: A Cross-Sectional Study.Healthcare (Basel, Switzerland) · 2025Article
- Retrospective chart review of inherited and idiopathic dystonia.Frontiers in genetics · 2025Article
- Prevalence and temporal relationship of clinical co-morbidities in idiopathic dystonia: a UK linkage-based study.Journal of neurology · 2024Article
- Neurosurgical and pharmacological management of dystonia.World journal of psychiatry · 2024Review
- Macro- and micro-structural insights into primary dystonia: a UK Biobank study.Journal of neurology · 2024Article
- Accelerometer-derived sleep measures in idiopathic dystonia: A UK Biobank cohort study.Brain and behavior · 2023Article
- Cortical neuronal hyperexcitability and synaptic changes in SGCE mutation-positive myoclonus dystonia.Brain : a journal of neurology · 2023Article
- Longitudinal analysis of the relationship between motor and psychiatric symptoms in idiopathic dystonia.European journal of neurology · 2022Article
- Clinical and genotypic analysis in determining dystonia non-motor phenotypic heterogeneity: a UK Biobank study.Journal of neurology · 2022Article
- Cognitive and Neuropsychiatric Impairment in Dystonia.Current neurology and neuroscience reports · 2022Review
- The Prevalence of Idiopathic or Inherited Isolated Dystonia: A Systematic Review and Meta-Analysis.Movement disorders clinical practice · 2022Review
- Adult-onset idiopathic dystonia: A national data-linkage study to determine epidemiological, social deprivation, and mortality characteristics.European journal of neurology · 2022Article
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Authors and funding
5 authors at 3 institutions in 1 country.
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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 (≥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.
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