Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autónoma de Barcelona, Barcelona, Spain.
Amyotroph Lateral Scler Frontotemporal Degener. 2013 May;14(4):278-83. doi: 10.3109/21678421.2012.749915. Epub 2013 Jan 4.
Our objective was to determine the incidence and clinical-epidemiological characteristics of an ALS cohort patient in Catalonia (Spain). We conducted a population based registry for a three-year period (1999-2001) in Catalonia (6,361,365 inhabitants) using several sources of information. The original El Escorial diagnostic criteria (1994) for ALS were applied for the classification of patients. New cases diagnosed between 1 January 1999 and 31 December 2001 were 215 (118 males and 97 females), with an annual crude incidence rate of 1.4/100,000 (95% CI 1.3-1.8). This rate showed a peak age between 75 and 79 years. The incidence rate was 1.6 (95% CI 1.5-2.2) in males and 1.2 (95% CI 1.1-1.7) in females. Prevalence at the end of the period was 5.4/100,000 of the total population. Median age at onset was 64.3 years. Onset of symptoms was bulbar or generalized in 38% of cases. Mean disease duration at diagnosis was 11.0 months. Median time of survival from onset was 30.8 months. In conclusion, ALS incidence in Catalonia is within the range of other countries across Europe with different geographic, environmental and socioeconomic situations. However, as in other studies conducted in the Mediterranean area, Catalonia incidence is in the lower range of rates in Europe.
我们的目的是确定加泰罗尼亚（西班牙）ALS 队列患者的发病率和临床流行病学特征。我们使用多种信息来源，对加泰罗尼亚（636.1365 万居民）进行了为期三年（1999-2001 年）的基于人群的登记。我们应用了原始的埃尔埃斯克里尔（El Escorial）诊断标准（1994 年）对患者进行分类。1999 年 1 月 1 日至 2001 年 12 月 31 日期间新诊断的病例为 215 例（男性 118 例，女性 97 例），粗发病率为 1.4/10 万（95%CI 1.3-1.8）。该发病率在 75-79 岁之间达到峰值。男性发病率为 1.6（95%CI 1.5-2.2），女性为 1.2（95%CI 1.1-1.7）。在研究期末，总人口中患病率为 5.4/10 万。发病中位年龄为 64.3 岁。38%的病例起病症状为延髓或全身性。诊断时疾病平均持续时间为 11.0 个月。从发病到死亡的中位生存时间为 30.8 个月。总之，加泰罗尼亚的 ALS 发病率与欧洲其他具有不同地理、环境和社会经济情况的国家相似。然而，与地中海地区的其他研究一样，加泰罗尼亚的发病率处于欧洲较低的水平。
