The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK
The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK
复制标题
进行性核上性麻痹（Steele-Richardson-Olszewski 综合征）在英国的发病率
DOI：
10.1093/brain/124.7.1438
复制
发表时间：
2001-07-01
期刊：
影响因子：
14.5
通讯作者：
Burn, DJ
中科院分区：
文献类型：
--
作者：
Nath, U;Ben-Shlomo, Y;Burn, DJ
我们进行了一项研究，以估计进行性核上性麻痹（PSP）在英国国家、地区和社区层面的点患病率。使用了“俄罗斯娃娃”设计，其中三个研究中的每个研究的总体分母依次变小，而病例确定方法变得越来越严格。NINDS-SPSP（国家神经疾病研究所和进行性核上性麻痹学会）诊断标准在整个研究中用于病例定义。国家研究确定了使用被动转诊机制的病例[例如，英国神经监测单位（BNSU）， PSP（欧洲）协会患者登记]。我们确定了577例PSP病例，给出了每10万人中1.0例的全国患病率估计[95%置信区间（CI） 0.9-1.1]。英格兰北部地区的研究使用了积极的“多源”病例确定，来自神经学家和非神经学家的合作网络，我们在本研究中确定了80例PSP，给出了粗患病率和年龄调整患病率分别为3.1 （95% CI 2.4-3.8）和2.4（1.9-3.0）每10万人。在这80例病例中，51例患者（65%）最初转诊给非神经科医生，10例患者（13%）在其疾病的任何阶段都没有见过神经科医生。该地区女性病例比例显著高于全国研究（61%比44%，P < 0.02），转介给非神经科医生的病例明显大于转介给神经科医生的病例（中位年龄73比69.5岁，P < 0.01）。社区研究中的患者是通过来自泰恩河畔纽卡斯尔全科医院的代表性样本的诊断和治疗登记来确定的，我们确定了17例PSP，得出的粗患病率和年龄调整患病率分别为每10万人6.5例（95% CI 3.4-9.7）和5.0例（95% CI 2.5-7.5）。17例中有7例（41%）以前没有被诊断为PSP。这项研究表明，PSP比以前认为的更常见，通常被误诊，并且大多数病例最初没有被转诊给神经科医生。该研究还证实了积极和详细的病例确定对于确保可靠的患病率估计的重要性。
We performed a study to estimate the point prevalence of progressive supranuclear palsy (PSP) in the UK at national, regional and community levels. A 'Russian doll' design was used in which the population denominator for each of the three studies was successively smaller, whilst the method of case ascertainment became increasingly more rigorous. The NINDS-SPSP (National Institute of Neurological Disorders and the Society for Progressive Supranuclear Palsy) diagnostic criteria for PSP were applied throughout the study for case definition. The national study identified cases using passive referral mechanisms [e.g. the British Neurological Surveillance Unit (BNSU), PSP (Europe) Association patient register]. We identified 577 cases of PSP, giving a national prevalence estimate of 1.0 per 100 000 [95% confidence interval (CI) 0.9-1.1]. The North of England regional study used active 'multiple source' case ascertainment from a collaborative network of neurologists and non-neurologists, We identified 80 cases of PSP in this study, giving a crude and age-adjusted prevalence of 3.1 (95% CI 2.4-3.8) and 2.4 (1.9-3.0) per 100 000, respectively. Of these 80 cases, 51 patients (65%) were referred initially to non-neurologists and 10 patients (13%) had not seen a neurologist at any stage of their illness. The proportion of female cases was significantly greater in the regional than in the national study (61% versus 44%; P < 0.02), Cases referred to non-neurologists were significantly older than those referred to neurologists in the regional study (median age 73 versus 69.5 years; P < 0.01). Patients in the community study were identified via diagnostic and therapeutic registers from a representative sample of general practices in Newcastle upon Tyne, We identified 17 cases of PSP, yielding crude and age-adjusted prevalences of 6.5 (95% CI 3.4-9.7) and 5.0 (95% CI 2.5-7.5) per 100 000, respectively. Seven of the 17 cases (41%) had not previously been diagnosed as PSP This study suggests that PSP is more common than previously considered, is commonly misdiagnosed and that the majority of cases are not initially referred to neurologists. The study also confirms the importance of active and detailed case ascertainment in ensuring reliable prevalence estimates.