意大利弗留利-威尼斯朱利亚地区 2002 年至 2009 年肌萎缩侧索硬化症的发病率：一项回顾性基于人群的研究。

The incidence of amyotrophic lateral sclerosis in Friuli Venezia Giulia, Italy, from 2002 to 2009: a retrospective population-based study.

机构信息

Department of Medical and Biological Sciences, University of Udine, Udine, Italy.

出版信息

Neuroepidemiology. 2013;41(1):54-61. doi: 10.1159/000350015. Epub 2013 May 25.

DOI:10.1159/000350015

PMID:23711404

Abstract

BACKGROUND

We conducted a retrospective population-based study to estimate the incidence of amyotrophic lateral sclerosis (ALS) in Friuli Venezia Giulia, Italy, from 2001 to 2009.

METHODS

Multiple sources were used for case ascertainment: Health databases, archives of the neurology departments and of the regional chapter of the Italian ALS Association. The diagnosis was validated through clinical documentation review. Crude and standardized incidence rates (IRs) per 100,000 person-years were calculated.

RESULTS

We identified 262 incident ALS cases, 50.4% men, 4.2% familial. Half of the patients had spinal onset (56.8% in men) and 25.2% bulbar (29% in women). Bulbar onset had a similar frequency in women (31.7%) and men (31.5%) aged 67 or above at diagnosis. The crude IR was 2.72 (95% confidence interval, 95% CI, 2.39-3.05) and the male:female ratio 1.08. The IR peaked in the 65-74 age group, with a second increase in men 85 years and older. The IR standardized to the 2001 Italian population was 2.38 (95% CI 2.13-2.63) and to the 2000 European population 2.58 (95% CI 2.34-2.81).

CONCLUSIONS

This retrospective study found IRs of ALS in the range of Italian and European prospective population-based registries, suggesting an almost complete case ascertainment.

摘要

背景

我们进行了一项回顾性基于人群的研究，以估计 2001 年至 2009 年意大利弗留利-威尼斯朱利亚地区的肌萎缩侧索硬化症（ALS）的发病率。

方法

使用多种来源进行病例确认：健康数据库、神经病学部门的档案以及意大利 ALS 协会的地区分会。通过临床文件回顾验证诊断。计算每 100,000 人年的粗发病率（IR）和标准化发病率（IR）。

结果

我们确定了 262 例新发 ALS 病例，其中 50.4%为男性，4.2%为家族性。一半的患者为脊髓型（男性中 56.8%），25.2%为延髓型（女性中 29%）。在诊断时，67 岁或以上的女性和男性延髓型发病率相似（分别为 31.7%和 31.5%）。粗发病率为 2.72（95%置信区间，95%CI，2.39-3.05），男女比例为 1.08。发病率峰值出现在 65-74 岁年龄组，男性 85 岁及以上年龄段再次增加。根据 2001 年意大利人口标准化的发病率为 2.38（95%CI 2.13-2.63），根据 2000 年欧洲人口标准化的发病率为 2.58（95%CI 2.34-2.81）。