Department of Neurology, Peking Union Medical College Hospital, Beijing, 100730, China.
Neuroscience Center, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Transl Neurodegener. 2021 Aug 9;10(1):28. doi: 10.1186/s40035-021-00253-2.
The aim of this study was to assess and compare the diagnostic utility of a new diagnostic criteria for amyotrophic lateral sclerosis (ALS), abbreviated as the 'Gold Coast Criteria', with the revised El Escorial (rEEC) and Awaji criteria.
Clinical and electrophysiological data of 1185 patients from January 2014 to December 2019 in the Peking Union Medical College Hospital ALS database were reviewed. The sensitivity of the Gold Coast criteria was compared to that of the possible rEEC and Awaji criteria (defined by the proportion of patients categorized as definite, probable, or possible ALS).
A final diagnosis of ALS was recorded in 1162 patients. The sensitivity of the Gold Coast criteria (96.6%, 95% confidence interval [CI] = 95.3%-97.5%) was greater than that of the rEEC (85.1%, 95%CI = 82.9%-87.1%) and Awaji (85.3%, 95%CI = 83.2%-87.3%). In addition, the sensitivity of the novel criteria maintained robust across subgroups, and the advantage was more prominent in limb-onset ALS patients and those who completed electromyographic tests. In those who did not achieve any of the rEEC diagnostic categories, the sensitivity of Gold Coast criteria was 84.4%.
The current study demonstrated that the Gold Coast criteria exhibited greater diagnostic sensitivity than the rEEC and Awaji criteria in a Chinese ALS population. The application of the Gold Coast criteria should be considered in clinical practice and future therapeutic trials.
本研究旨在评估和比较新的肌萎缩侧索硬化症(ALS)诊断标准(简称“黄金海岸标准”)与修订后的埃尔埃斯克里瓦(rEEC)和淡路岛标准的诊断效用。
回顾 2014 年 1 月至 2019 年 12 月北京协和医院 ALS 数据库中 1185 例患者的临床和电生理数据。比较了黄金海岸标准的敏感性与可能的 rEEC 和 Awaji 标准(根据归类为明确、可能或可能 ALS 的患者比例定义)。
1162 例患者记录了最终的 ALS 诊断。黄金海岸标准的敏感性(96.6%,95%置信区间 [CI] = 95.3%-97.5%)高于 rEEC(85.1%,95%CI = 82.9%-87.1%)和 Awaji(85.3%,95%CI = 83.2%-87.3%)。此外,新标准的敏感性在亚组中保持稳健,在肢体起病的 ALS 患者和完成肌电图检查的患者中优势更为明显。在未达到任何 rEEC 诊断类别的患者中,黄金海岸标准的敏感性为 84.4%。
本研究表明,在中国人群中,黄金海岸标准在诊断敏感性方面优于 rEEC 和 Awaji 标准。在临床实践和未来的治疗试验中,应考虑应用黄金海岸标准。