Zoccolella S, Beghi E, Serlenga L, Logroscino G
Department of Neurological Sciences, University of Bari, Italy.
Neurol Sci. 2005 Dec;26(5):330-3. doi: 10.1007/s10072-005-0501-y.
Amyotrophic lateral sclerosis (ALS) diagnosis is based exclusively on clinical grounds because of the absence of biological markers and of specific neuroradiological and neurophysiological diagnostic features. A clinical classification system of cases has been introduced (El Escorial Criteria, EEC) and then revised after the inclusion of the neurophysiologic assessment (Airlie House Criteria, AHC) for enrollment of patients in clinical trials. The aim of this study is to present cases at presentation in the early stages of the disease that have difficult allocation both in EEC and AHC. All cases were subjects enrolled through SLAP, a population-based registry based in Puglia, Southern Italy. Although differential diagnosis excluded ALS-mimic syndromes, we identified four cases (out of 130 cases, 3.1%) that did not meet the EEC and AHC at the first visit. Even though the number of unclassifiable cases is small, both EEC and AHC may be restrictive. This precludes the enrollment of ALS cases at an early stage both in observational studies and clinical trials.
肌萎缩侧索硬化症（ALS）的诊断完全基于临床依据，因为缺乏生物学标志物以及特定的神经放射学和神经生理学诊断特征。已经引入了一种病例临床分类系统（埃尔埃斯科里亚尔标准，EEC），之后在纳入神经生理学评估（艾利屋标准，AHC）后进行了修订，以便将患者纳入临床试验。本研究的目的是呈现疾病早期就诊时在EEC和AHC中都难以分类的病例。所有病例均为通过SLAP招募的受试者，SLAP是意大利南部普利亚地区一个基于人群的登记处。尽管鉴别诊断排除了ALS模拟综合征，但我们在首次就诊时发现了4例（在130例病例中，占3.1%）不符合EEC和AHC的病例。尽管无法分类的病例数量较少，但EEC和AHC可能都具有局限性。这使得在观察性研究和临床试验中都难以在早期阶段纳入ALS病例。
