Zoccolella S, Beghi E, Palagano G, Fraddosio A, Guerra V, Samarelli V, Lepore V, Simone I L, Lamberti P, Serlenga L, Logroscino G
Department of Neurological Sciences, University of Bari, Italy.
J Neurol Neurosurg Psychiatry. 2008 Jan;79(1):33-7. doi: 10.1136/jnnp.2007.118018. Epub 2007 Jun 5.
To measure survivorship and predictors of prognosis of amyotrophic lateral sclerosis (ALS).
Incident cases, diagnosed in the 1998-1999 period and classified according to the El Escorial criteria, were enrolled from a prospective population based registry established in Puglia, Southern Italy, with a reference population of 4,025,329. Cases were followed up until death or 30 June 2004.
We identified 130 incident cases of ALS while four were lost to follow-up. Median survival was 28 months from first symptoms and 16 months from diagnosis, while cumulative survivorship at 4 years was approximately 30%. Advanced age (>75 years: hazard ratio (HR) 7.5; 95% CI 1.9 to 29.6; p = 0.004) and bulbar or generalised (HR 1.8; 95% CI 1.1 to 3.0; p = 0.01) onset of symptoms were independent predictors of adverse survival. After stratifying patients according to site of first symptoms, age was a predictor of death among spinal (HR for patients aged >75 years compared with patients aged 45 years or less: HR 11; 95% CI 1.5 to 78.5; p = 0.01) but not among bulbar ALS (HR 4.5; 95% CI 0.4 to 46.5; p = 0.2). Among spinal onset cases, cases with predominant upper motoneuronal (UMN) involvement presented with a borderline significant better survivorship (HR 0.5; 95% CI 0.2 to 1.3; p = 0.1)
Bulbar signs and advanced age among subjects with spinal onset were indicators of poor prognosis while El Escorial category at entry did not predict survival. Among subjects with spinal onset of the disease, a trend for a better survivorship of subjects with UMN signs was noted.
测量肌萎缩侧索硬化症（ALS）的生存率及预后预测因素。
选取1998 - 1999年确诊、根据埃尔埃斯科里亚尔标准分类的新发病例，这些病例来自意大利南部普利亚地区建立的前瞻性人群登记处，参考人群为4,025,329人。对病例进行随访直至死亡或2004年6月30日。
我们确定了130例ALS新发病例，4例失访。从首次出现症状起的中位生存期为28个月，从诊断起为16个月，4年时的累积生存率约为30%。高龄（>75岁：风险比（HR）7.5；95%置信区间1.9至29.6；p = 0.004）以及延髓或全身症状发作（HR 1.8；95%置信区间1.1至3.0；p = 0.01）是不良生存的独立预测因素。根据首发症状部位对患者进行分层后，年龄是脊髓型患者死亡的预测因素（>75岁患者与45岁及以下患者相比的HR：HR 11；95%置信区间1.5至78.5；p = 0.01），但在延髓型ALS患者中并非如此（HR 4.5；95%置信区间0.4至46.5；p = 0.2）。在脊髓型发病病例中，主要表现为上运动神经元（UMN）受累的病例生存期有临界显著改善趋势（HR 0.5；95%置信区间0.2至1.3；p = 0.1）
脊髓型发病患者的延髓体征和高龄是预后不良的指标，而入院时的埃尔埃斯科里亚尔分类不能预测生存情况。在脊髓型发病的患者中，注意到UMN体征患者生存期有改善的趋势。
