IRCCS Ospedale Policlinico San Martino, Genova, Italy.
Department of Quantitative Biomedicine, University of Zurich, Zurich, Switzerland.
Amyotroph Lateral Scler Frontotemporal Degener. 2024 Feb;25(1-2):104-111. doi: 10.1080/21678421.2023.2260842. Epub 2024 Jan 23.
This article presents an updated analysis of the LIGALS register, a prospective study conducted over a ten-year period (2009-2018) in Liguria, Italy, aimed at evaluating the incidence, prevalence, clinical presentation, and management of amyotrophic lateral sclerosis (ALS).
We calculated the mean annual crude incidence rate of ALS, assessed the point prevalence of ALS on January 1, 2018, and analyzed demographic factors, clinical characteristics, and clinical management strategies. Data analysis included Cox regression analysis to identify predictors of survival.
The mean annual crude incidence rate of ALS was 3.16/100,000 per year (CI 95%) while the point prevalence of ALS on January 1, 2018, was 9.31/100,000 population (CI 95%). Among the patients, 6.5% were familial ALS, while 93.5% were sporadic cases. Clinical management strategies, including percutaneous endoscopic gastrostomy (PEG) and noninvasive ventilation (NIV), were employed. The study observed a stable frequency of NIV initiation and PEG placement over time, with a growing trend toward earlier PEG positioning. The mean survival from symptom onset was 39 months, whereas from diagnosis, it was 26 months. Cox regression analysis identified several predictors of survival, including gender, age at onset and diagnosis, site of onset, diagnostic category, phenotype, and diagnostic delay.
This comprehensive analysis provides valuable insights into the long-term trends in ALS epidemiology and clinical management in Liguria, Italy. It underscores the importance of continued research efforts in understanding and addressing the challenges posed by ALS, particularly in terms of early diagnosis and optimizing clinical interventions to improve patient outcomes.
本文对 LIGALS 登记处进行了更新分析，这是一项在意大利利古里亚地区进行的为期十年（2009-2018 年）的前瞻性研究，旨在评估肌萎缩侧索硬化症（ALS）的发病率、患病率、临床表现和治疗方法。
我们计算了 ALS 的平均年粗发病率，评估了 2018 年 1 月 1 日的 ALS 点患病率，并分析了人口统计学因素、临床特征和临床管理策略。数据分析包括 Cox 回归分析，以确定生存的预测因素。
ALS 的平均年粗发病率为 3.16/100,000 人/年（95%CI），而 2018 年 1 月 1 日的 ALS 点患病率为 9.31/100,000 人（95%CI）。患者中，6.5%为家族性 ALS，93.5%为散发性病例。采用了经皮内镜胃造口术（PEG）和无创通气（NIV）等临床管理策略。研究观察到 NIV 启动和 PEG 放置的频率在一段时间内保持稳定，且早期 PEG 定位的趋势呈上升趋势。从症状出现到死亡的平均生存时间为 39 个月，从诊断到死亡的平均生存时间为 26 个月。Cox 回归分析确定了几个生存的预测因素，包括性别、发病和诊断时的年龄、发病部位、诊断类别、表型和诊断延迟。
这项全面分析提供了意大利利古里亚地区 ALS 流行病学和临床管理的长期趋势的宝贵见解。它强调了继续努力研究理解和应对 ALS 所带来的挑战的重要性，特别是在早期诊断和优化临床干预以改善患者预后方面。
