Levison Lotte Sahin, Blicher Jakob Udby, Andersen Henning
Department of Neurology, Aarhus University Hospital, Aarhus, Denmark.
J Neurol. 2024 Dec 12;272(1):44. doi: 10.1007/s00415-024-12743-0.
Recent studies have suggested that the incidence rate (IR) and the rate of death (MR) of amyotrophic lateral sclerosis (ALS) are increasing. Still, it remains unclear whether this is due to improved case ascertainment or represents a true increase. We examined the development in the incidence and mortality of ALS in Denmark for 42 years.
We retrieved individual-level data of all patients aged above 18 years with first-time ALS diagnosed at any Danish department of neurology. The IR and MR were calculated based on data from 1980 to 2021, stratified by gender and age.
We identified 5,943 patients with ALS and identified a total of 5,069 deaths in the nationwide population. Overall, the IR was 3.4 per 100,000 persons per year (95% CI 3.4-3.5). ALS incidence rose gradually during the study period, and the IR was 2.8 times higher (95% CI 2.4-3.2) when comparing the latest period (2018-2021) with the first (1980-1983). Parallel to the IR, the MR increased over time and was associated with male gender and rose with age at diagnosis, peaking in the 70-79-year age group.
In Denmark, the IR and MR of ALS increased threefold from 1980 to 2021, with steadily increasing risk related to male gender and in particular to higher age. Considering our aging societies, the number of elderly patients with ALS can be expected to increase considerably.
近期研究表明，肌萎缩侧索硬化症（ALS）的发病率（IR）和死亡率（MR）正在上升。然而，目前尚不清楚这是由于病例确诊率提高所致，还是代表了真正的增长。我们研究了丹麦42年间ALS发病率和死亡率的变化情况。
我们检索了丹麦各神经内科首次诊断为ALS的所有18岁以上患者的个体水平数据。根据1980年至2021年的数据计算发病率和死亡率，并按性别和年龄分层。
我们共识别出5943例ALS患者，在全国范围内共确定了5069例死亡病例。总体而言，发病率为每年每10万人3.4例（95%置信区间3.4 - 3.5）。在研究期间，ALS发病率逐渐上升，将最近时期（2018 - 2021年）与第一个时期（1980 - 1983年）相比，发病率高出2.8倍（95%置信区间2.4 - 3.2）。与发病率相似，死亡率也随时间增加，与男性性别有关，且在诊断时随年龄增长而上升，在70 - 79岁年龄组达到峰值。
在丹麦，1980年至2021年期间，ALS的发病率和死亡率增加了两倍，与男性性别尤其是高龄相关的风险稳步增加。考虑到我们社会的老龄化，预计老年ALS患者的数量将大幅增加。
