Ragonese Paolo, Filippini Graziella, Salemi Giuseppe, Beghi Ettore, Citterio Antonietta, D'Alessandro Roberto, Marini Carmine, Monsurrò Maria Rosaria, Aiello Isidoro, Morgante Letterio, Tempestini Antonella, Fratti Cesare, Ragno Michele, Pugliatti Maura, Epifanio Antonio, Testa Daniela, Savettieri Giovanni
Institute of Neuropsychiatry, University of Palermo, Palermo, Italy.
Neuroepidemiology. 2004 Jan-Apr;23(1-2):73-7. doi: 10.1159/000073978.
To evaluate the accuracy of death certificates (DCs) for amyotrophic lateral sclerosis (ALS) in different parts of Italy. Studies based on DC diagnosis for ALS have shown a reduced mortality comparing northern with southern Italy. These data are in contrast with results from other surveys on the incidence of ALS performed in Italy and other countries.
Archives of neurological clinics from northern (Milano, Monza, Pavia, and Bologna) and southern Italy including islands (Napoli, Sassari, Palermo, and Messina) were searched for patients discharged with a diagnosis of ALS in the period 1970-1995. Subjects affected by definite/probable ALS according to the Scottish Motor Neuron Disease Research Group diagnostic criteria were included. DCs were obtained from the vital statistic bureau. True positive rates (TPRs) and 95% confidence intervals (CIs) for proportions were calculated for northern and southern Italy separately. Multiple logistic regression analysis was performed according to gender, age at onset, age and year of death, and interval between onset and death.
We found 651 patients affected by definite/probable ALS; 573 of them had died by December 31, 1996. DCs were available for 566 subjects (411 from northern Italy and 155 from southern Italy). TPR was 66.7% (95% CI 61.9-71.2) for northern Italy and 51.6% (95% CI 43.5-59.7) for southern Italy (chi(2) = 10.9, p = 0.001). Logistic regression analysis showed an association between a lower accuracy of DCs and the interval between onset of symptoms and death. TPR calculations considering different death periods (1970-1982 and 1983-1996) showed comparable rates of accuracy over time.
Mortality statistics based on official death records do not accurately reflect interregional mortality for ALS in Italy.
评估意大利不同地区肌萎缩侧索硬化症（ALS）死亡证明的准确性。基于死亡证明诊断ALS的研究显示，意大利北部与南部相比死亡率较低。这些数据与在意大利和其他国家进行的关于ALS发病率的其他调查结果形成对比。
检索意大利北部（米兰、蒙扎、帕维亚和博洛尼亚）和包括岛屿在内的南部（那不勒斯、萨萨里、巴勒莫和墨西拿）神经科诊所的档案，查找1970 - 1995年期间诊断为ALS出院的患者。纳入根据苏格兰运动神经元疾病研究组诊断标准确诊为明确/可能ALS的患者。从生命统计局获取死亡证明。分别计算意大利北部和南部的真阳性率（TPR）及比例的95%置信区间（CI）。根据性别、发病年龄、死亡年龄和年份以及发病与死亡之间的间隔进行多因素逻辑回归分析。
我们发现651例明确/可能患有ALS的患者；其中573例在1996年12月31日前死亡。566名受试者有死亡证明（411名来自意大利北部，155名来自意大利南部）。意大利北部的TPR为66.7%（95% CI 61.9 - 71.2），意大利南部为51.6%（95% CI 43.5 - 59.7）（χ² = 10.9，p = 0.001）。逻辑回归分析显示死亡证明准确性较低与症状出现至死亡之间的间隔有关。考虑不同死亡时期（1970 - 1982年和1983 - 1996年）的TPR计算显示，随着时间推移准确率相当。
基于官方死亡记录的死亡率统计不能准确反映意大利各地区ALS的死亡率。
