Luker Jackson, Woodman Richard, Schultz David
College of Medicine and Public Health, Flinders University, Adelaide, Australia and.
Flinders Medical Centre, Bedford Park, Australia.
Amyotroph Lateral Scler Frontotemporal Degener. 2023 May;24(3-4):195-202. doi: 10.1080/21678421.2022.2108326. Epub 2022 Aug 8.
Motor neurone disease (MND) is a neurodegenerative disease that leads to significant morbidity and mortality. The Global Burden of Disease (GBD) project estimated the MND all-age global prevalence is 4.5 (95% confidence interval (CI)=4.1-5.0) per 100,000 persons and the all-age incidence of 0.78 (95% CI = 0.71-0.86) per 100,000 person-years. Whether the prevalence and incidence of MND within South Australia using registry data is in keeping with these global estimates has not been studied.
To describe the prevalence and annual incidence rates (IRs) of MND in South Australia between 2017 and 2019.
A retrospective cohort study calculating the point prevalence of MND on 31 December 2017, 2018 and 2019 utilizing data from the Australasian Motor Neurone Disease Registry (AMNDR). The annual IRs between 2017 and 2019 were calculated using population data reported in the 2016 Australian census and age-standardized rates utilized the world population from the United Nations Population Division's World Population Prospects.
A total of 256 MND patients were identified, of whom 114 were alive on 31 December 2019. Based on the 2016 population of 1,676,653 persons, the estimated prevalence was 6.79 per 100,000 persons. The crude IR for the all-age South Australian population was 3.34 per 100,000 person-years (95% CI, 2.85-3.88). The estimated annual IRs based on those ≥20 yo were 4.98 (3.84-6.35), 3.68 (2.71-4.88), and 4.21 (3.17-5.49) per 100,000 person-years for 2017, 2018, and 2019, respectively.
The crude prevalence and incidence of MND in South Australia were considerably higher than global estimates. This may reflect higher rates of the disease or higher rates of case ascertainment compared to the GBD project.
运动神经元病（MND）是一种神经退行性疾病，会导致严重的发病和死亡。全球疾病负担（GBD）项目估计，MND在全年龄段的全球患病率为每10万人中有4.5例（95%置信区间（CI）=4.1 - 5.0），全年龄段发病率为每10万人年中有0.78例（95%CI = 0.71 - 0.86）。南澳大利亚州使用登记数据得出的MND患病率和发病率是否与这些全球估计值相符尚未得到研究。
描述2017年至2019年南澳大利亚州MND的患病率和年发病率（IR）。
一项回顾性队列研究，利用澳大利亚运动神经元病登记处（AMNDR）的数据计算2017年、2018年和2019年12月31日MND的点患病率。2017年至2019年的年发病率使用2016年澳大利亚人口普查报告的人口数据进行计算，年龄标准化率采用联合国人口司《世界人口展望》中的世界人口数据。
共识别出256例MND患者，其中114例在2019年12月31日时存活。根据2016年1,676,653人的人口数据，估计患病率为每10万人中有6.79例。南澳大利亚州全年龄段人口的粗发病率为每10万人年中有3.34例（95%CI，2.85 - 3.88）。基于20岁及以上人群的估计年发病率，2017年、2018年和2019年分别为每10万人年中有4.98例（3.84 - 6.35）、3.68例（2.71 - 4.88）和4.21例（3.17 - 5.49）。
南澳大利亚州MND的粗患病率和发病率显著高于全球估计值。这可能反映出该疾病的发病率较高，或者与GBD项目相比病例确诊率较高。
