Scialò Carlo, Novi Giovanni, Bandettini di Poggio Monica, Canosa Antonio, Sormani Maria Pia, Mandich Paola, Origone Paola, Truffelli Romina, Mancardi Giovanni Luigi, Caponnetto Claudia
a Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
b ALS Centre 'Rita Levi Montalcini' Department of Neuroscience, University of Torino, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI) , University of Genova, IRCCS AOU San Martino-IST , Genova.
Amyotroph Lateral Scler Frontotemporal Degener. 2016 Oct-Nov;17(7-8):535-542. doi: 10.1080/21678421.2016.1197942. Epub 2016 Jul 11.
Our objectives were: (1) to assess amyotrophic lateral sclerosis (ALS) incidence and its trend over time in Liguria, an Italian north-western region, performing an analysis of data prospectively collected from 1 January 2009 to 31 December 2014; (2) to determine the mean and median survival in the 2009-2014 Ligurian ALS incident cases; and (3) to evaluate the presence of disease prognostic factors. The Liguria Register for ALS (LIGALS) is an ongoing, multicentre prospective register enrolling all ALS incident cases in Liguria. Cases were identified using several concurrent sources. ALS diagnosis was based on El Escorial revised criteria (EEC-R). Two hundred and ninety-eight patients were enrolled in this study. The mean annual crude incidence rate in the 2009-2014 period was 3.11/100,000 population (95% CI 2.77-3.49); the point prevalence at 31 December 2014 was 7.85/100,000 (95% CI 6.54-9.36) population. Survival analysis demonstrated a median survival from symptom onset of 37.0 months (95% CI 32.0-42.0). In conclusion, ALS crude incidence in Liguria is higher compared to other Italian regions. Clinical and epidemiological data are comparable with those of the Italian ALS population. Survival analysis showed that higher age at onset, bulbar onset, definite EEC-R diagnostic category and a shorter diagnostic delay are related with worse outcomes.
（1）评估意大利西北部利古里亚地区肌萎缩侧索硬化症（ALS）的发病率及其随时间的变化趋势，对2009年1月1日至2014年12月31日期间前瞻性收集的数据进行分析；（2）确定2009 - 2014年利古里亚ALS发病病例的平均和中位生存期；（3）评估疾病预后因素的存在情况。利古里亚ALS登记册（LIGALS）是一个正在进行的多中心前瞻性登记册，登记利古里亚所有ALS发病病例。通过多种并行来源识别病例。ALS诊断基于埃尔埃斯科里亚尔修订标准（EEC - R）。本研究纳入了298例患者。2009 - 2014年期间的年平均粗发病率为3.11/10万人口（95%置信区间2.77 - 3.49）；2014年12月31日的点患病率为7.85/10万（95%置信区间6.54 - 9.36）人口。生存分析显示，从症状出现开始的中位生存期为37.0个月（95%置信区间32.0 - 42.0）。总之，利古里亚的ALS粗发病率高于意大利其他地区。临床和流行病学数据与意大利ALS人群的数据相当。生存分析表明，发病年龄较大、延髓发病、明确的EEC - R诊断类别以及诊断延迟较短与较差的预后相关。
