Beghi E, Millul A, Micheli A, Vitelli E, Logroscino G
Clinica Neurologica, Monza, Italy.
Neurology. 2007 Jan 9;68(2):141-5. doi: 10.1212/01.wnl.0000250339.14392.bb.
To assess the incidence and trends of ALS in a large population at risk.
This study was performed in nine provinces of Lombardy in Northern Italy (population 4,947,554). Patients with newly diagnosed ALS were enrolled during the period 1998 to 2002 through a prospective regional register. For each patient, the main demographic and clinical information was collected by the caring physicians and reviewed by a panel of experts according to the original and revised El Escorial diagnostic criteria. Overall, age- and sex-specific and standardized annual incidence rates were calculated for the entire population and for each year and province separately.
We studied 517 patients (M:F ratio 1.3) aged 18 to 92 years (mean 63.6). Onset of symptoms was bulbar in 29% of cases. ALS was definite in 45%, probable in 27%, probable laboratory supported in 3.5%, possible in 15%, and suspected in 10%. Mean disease duration at diagnosis was 10.6 months. The standardized incidence rate was 2.09 per 100,000/year (95% CI: 1.17 to 3.18). The rate, which was 2.43 in men and 1.76 in women, tended to increase up to ages 65 to 74 and to decrease thereafter. The rate was unchanged over time and presented moderate variations across provinces. The incidence rate of definite ALS was 0.93 (spinal-onset ALS 1.35; bulbar-onset ALS 0.74) and was consistently higher in men with spinal-onset ALS vs men with bulbar-onset ALS and women.
The incidence of ALS varied according to age, sex, and site of onset. No temporal and geographic clusters were detected over a 5-year period.
评估一大群高危人群中肌萎缩侧索硬化症（ALS）的发病率及趋势。
本研究在意大利北部伦巴第大区的九个省份进行（人口4,947,554）。1998年至2002年期间，通过前瞻性区域登记系统纳入新诊断为ALS的患者。每位患者的主要人口统计学和临床信息由护理医生收集，并由一组专家根据原始及修订后的埃尔埃斯科里亚尔诊断标准进行审核。总体而言，分别计算了整个人口以及每年和每个省份的年龄和性别特异性标准化年发病率。
我们研究了517例年龄在18至92岁（平均63.6岁）的患者（男女性别比为1.3）。29%的病例症状首发于延髓。ALS确诊病例占45%，很可能病例占27%，可能有实验室支持病例占3.5%，可能病例占15%，疑似病例占10%。诊断时的平均病程为10.6个月。标准化发病率为每100,000人/年2.09（95%置信区间：1.17至3.18）。该发病率男性为2.43，女性为1.76，在65至74岁之前呈上升趋势，此后下降。发病率随时间无变化，且各省之间存在适度差异。确诊ALS的发病率为0.93（脊髓起病型ALS为1.35；延髓起病型ALS为0.74），脊髓起病型男性ALS患者的发病率始终高于延髓起病型男性及女性患者。
ALS的发病率因年龄、性别和起病部位而异。在5年期间未发现时间和地理聚集现象。
