Zoccolella Stefano, Beghi Ettore, Palagano Guerrino, Fraddosio Angela, Samarelli Vito, Lamberti Paolo, Lepore Vito, Serlenga Luigi, Logroscino Giancarlo
Department of Neurological Sciences, University of Bari, Italy.
J Neurol Sci. 2006 Dec 1;250(1-2):45-9. doi: 10.1016/j.jns.2006.06.027. Epub 2006 Aug 22.
The El Escorial and the revised Airlie House diagnostic criteria for amyotrophic lateral sclerosis (ALS) were introduced to select patients for clinical trials. Heterogeneity of clinical presentation at onset and delay in diagnosis may decrease the likelihood for trial entry.
Identify risk factors for delay in the diagnosis and trial exclusion.
ALS incident cases were identified with El Escorial (EEC) and Airlie House criteria (AHC) through a population-based registry established in Puglia, Southern Italy, in the years 1998-99.
130 ALS incident cases were diagnosed with a median interval between onset of symptoms and diagnosis of 9.3 months and not different across both EEC and AHC categories. Twenty percent of cases were not eligible for clinical trials according to the AHC. About 5% of subjects in this series died with only lower motor neuron signs. Predictors for delay in the diagnosis were age between 65 and 75 years and spinal onset while fasciculations and cramps as first symptoms were predictors of exclusion from trials.
In this population-based series, diagnostic delay was longer in subjects with spinal onset and age between 65 and 75 and fasciculation as first symptoms. About 80% of incident cases were trial eligible with AHC criteria. However, a significant number of subjects with ALS, characterized by a limited spread of signs, were not trial eligible while alive.
引入埃斯科里亚尔（El Escorial）和修订后的阿利屋（Airlie House）肌萎缩侧索硬化症（ALS）诊断标准以选择患者进行临床试验。起病时临床表现的异质性和诊断延迟可能会降低进入试验的可能性。
确定诊断延迟和试验排除的风险因素。
通过1998 - 1999年在意大利南部普利亚建立的基于人群的登记处，使用埃斯科里亚尔（EEC）和阿利屋（AHC）标准确定ALS新发病例。
130例ALS新发病例被诊断，症状出现至诊断的中位间隔为9.3个月，且在EEC和AHC类别中无差异。根据AHC，20%的病例不符合临床试验条件。该系列中约5%的受试者仅出现下运动神经元体征时死亡。诊断延迟的预测因素为年龄在65至75岁之间且起病于脊髓，而束颤和痉挛作为首发症状是试验排除的预测因素。
在这个基于人群的系列中，脊髓起病、年龄在65至75岁之间且以束颤为首发症状的受试者诊断延迟更长。根据AHC标准，约80%的新发病例符合试验条件。然而，相当数量体征扩散有限的ALS受试者在存活时不符合试验条件。
