Adult-onset idiopathic dystonia: A national data-linkage study to determine epidemiological, social deprivation, and mortality characteristics.
Adult-onset idiopathic dystonia: A national data-linkage study to determine epidemiological, social deprivation, and mortality characteristics.
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成人发作的特发性肌张力障碍：一项国家数据链接研究，用于确定流行病学，社会剥夺和死亡率特征。
DOI：
10.1111/ene.15114
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发表时间：
2022-01
影响因子：
5.1
通讯作者：
中科院分区：
文献类型：
--
作者：
准确的流行病学信息对于提高对肌张力障碍综合征的理解，以及更好地提供临床服务和为诊断决策提供背景至关重要。在这里，我们确定了威尔士人群中成人发病的特发性肌张力障碍的流行病学、社会剥夺和死亡率特征。使用威尔士匿名电子医疗保健数据进行了一项基于人群的回顾性队列研究，以确定1994年1月1日至2017年12月31日期间患有肌张力障碍的个体。我们开发了一种病例确定算法，以确定肌张力障碍的发病率和患病率，以及基于社会剥夺和死亡率的肌张力障碍队列特征。病例确定算法（79%的灵敏度）确定了54,966例；在这些病例中，41,660例为成人发病的特发性肌张力障碍（≥20岁）。在成人发病形式中，诊断时的中位年龄为41岁，男性在诊断时明显大于女性。患病率从1994年的0.02%到2017年的1.2%不等。年平均发病率为87.7/10万/年，由1994年的49.9/10万/年上升至2017年的96.21/10万/年。2017年，肌张力障碍患者的预期寿命与威尔士人口相似。我们开发了一种病例确定算法，通过引入神经学家审查的验证队列来支持，为未来基于人群的肌张力障碍研究提供了一个平台。我们已经建立了成年人发病的特发性肌张力障碍在人群水平上的患病率和发病率值，这反映了越来越多的临床认识和病因基因的鉴定。潜在的死亡原因反映了一般人群的死亡原因，包括循环系统疾病、呼吸系统疾病、癌症和痴呆。我们已经开发并验证了一种病例确定算法，为未来基于人群的肌张力障碍研究提供了一个平台。我们发现肌张力障碍的患病率和发病率比之前估计的要高，分别为1220/100,000/年（1.2%）和96/100,000/年。我们已经证明，肌张力障碍的诊断似乎不会对社会经济地位产生不利影响，也没有证据表明预期寿命会缩短。
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. A 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. 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. 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 those of the general population, including circulatory disorders, respiratory disorders, cancers, and dementia. We have developed and validated a case‐ascertainment algorithm, providing a platform for future population‐based dystonia studies. We demonstrate that prevalence and incidence rates of dystonia are higher than previously estimated, 1220/100,000/year (1.2%) and 96/100,000/year, respectively. We have shown that a diagnosis of dystonia does not appear to have a detrimental impact on socioeconomic status, and there was no evidence of decreased life expectancy.
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影响因子：
8.6
作者：
Cossu, Giovanni;Mereu, Alessandra;Contu, Paolo
通讯作者：
Contu, Paolo
影响因子：
3
作者：
Fonferko-Shadrach, Beata;Lacey, Arron S.;Pickrell, W. Owen
通讯作者：
Pickrell, W. Owen
影响因子：
4.5
作者：
Lee, Sze Chim;DelPozo-Banos, Marcos;John, Ann
通讯作者：
John, Ann
DOI：
10.1192/bjp.2019.153
发表时间：
2020-02
期刊：
The British journal of psychiatry : the journal of mental science
影响因子：
--
作者：
Demmler JC;Brophy ST;Marchant A;John A;Tan JOA
通讯作者：
Tan JOA
影响因子：
3.5
作者：
Lyons RA;Jones KH;John G;Brooks CJ;Verplancke JP;Ford DV;Brown G;Leake K
通讯作者：
Leake K