a Medical Area Department , University of Udine , Udine , Italy.
b Neurologic Clinic, Department of Medical, Surgical and Health Sciences , Azienda Sanitaria Universitaria Integrata di Trieste, University Hospital of Trieste , Trieste , Italy.
Amyotroph Lateral Scler Frontotemporal Degener. 2019 Feb;20(1-2):90-99. doi: 10.1080/21678421.2018.1511732. Epub 2018 Nov 15.
To describe the epidemiology of Amyotrophic Lateral Sclerosis (ALS) in Friuli-Venezia Giulia (FVG) region, Italy, over a 13-year period (2002-2014), estimating ALS (a) incidence, prevalence, and clinical features; (b) mortality, also comparing Udine municipality to the rest of FVG.
We conducted a retrospective population-based study. ALS incident cases were ascertained using multiple sources and validated through expert review. We calculated crude and standardized incidence rate (IR), point prevalence and mortality rate (MR), each with 95% confidence interval. Standardized incidence (SIR) and mortality (SMR) ratio were calculated to compare Udine to FVG.
Among 444 incident cases (50.0% men, median age 68.5 years), onset was bulbar in 30.2%, spinal in 59.9%, mixed in 9.9%; 3.6% had familial ALS. Crude and 2000 European population standardized IR was respectively 2.81 (2.56-3.09) and 2.09 (1.89-2.29) per 100,000 person-years. Standardized male-to-female incidence ratio was 1.05. IR peaked at age 65-74 years (men: 9.93, 8.04-12.32; women: 7.74, 6.18-9.67) and decreased thereafter. Prevalence was 8.36 (6.74-9.97) cases per 100,000 inhabitants on 30 June 2009 and 7.98 (6.40-9.56) on 30 June 2014. SIR was 1.20 and SMR 1.11.
When assessed over a long period, incidence of ALS was in the range of Italian and European population-based registries and showed a consistent pattern by age and sex. IR and MR were only slightly higher in Udine vs. FVG.
描述意大利弗留利-威尼斯朱利亚（FVG）地区 13 年来（2002-2014 年）肌萎缩侧索硬化症（ALS）的流行病学情况，估计 ALS 的（a）发病率、患病率和临床特征；（b）死亡率，并将乌迪内市与 FVG 其他地区进行比较。
我们进行了一项回顾性的基于人群的研究。使用多种来源确定 ALS 发病例，并通过专家审查进行验证。我们计算了粗发病率和标准化发病率（IR）、点患病率和死亡率（MR），每个值都有 95%置信区间。计算标准化发病率（SIR）和死亡率（SMR）比值，以比较乌迪内与 FVG。
在 444 例发病例中（50.0%为男性，中位年龄 68.5 岁），发病形式为延髓性 30.2%，脊髓性 59.9%，混合性 9.9%；3.6%为家族性 ALS。粗发病率和 2000 年欧洲人口标准化发病率分别为 2.81（2.56-3.09）和 2.09（1.89-2.29）/10 万人年。标准化男女发病率比值为 1.05。发病率在 65-74 岁年龄组达到高峰（男性：9.93，8.04-12.32；女性：7.74，6.18-9.67），此后逐渐下降。2009 年 6 月 30 日的患病率为每 10 万人 8.36（6.74-9.97）例，2014 年 6 月 30 日为 7.98（6.40-9.56）例。SIR 为 1.20，SMR 为 1.11。
从长期来看，ALS 的发病率在意大利和欧洲基于人群的登记处范围内，且呈年龄和性别一致的模式。乌迪内与 FVG 相比，IR 和 MR 仅略高。
