Department of Clinical Neurophysiology, Aarhus University Hospital, Aarhus, Denmark.
Department of Neurology, Saint-Etienne University Hospital, Saint-Etienne, France.
Clin Neurophysiol. 2021 Dec;132(12):3183-3189. doi: 10.1016/j.clinph.2021.08.014. Epub 2021 Sep 8.
This study evaluates diagnostic accuracy of the proposed 'Gold Coast' (GC) diagnostic criteria for amyotrophic lateral sclerosis (ALS).
Five European centres retrospectively sampled consecutive patients referred for electromyography on suspicion of ALS. Patients were classified according to the GC criteria, the revised El Escorial (rEE) criteria and the Awaji (AW) criteria without and with the 'Possible' category (+ Poss). Reference standard was ALS confirmed by disease progression at follow-up.
Of 404 eligible patients 272 were diagnosed as ALS, 94 had mimicking disorders, 35 were lost for follow-up, and three had insufficient data. Sensitivity for the GC criteria was 88.2% (95% CI: 83.8-91.8%), which was higher than for previous criteria, of which the AW + Poss criteria reached the highest sensitivity of 77.6% (95% CI: 72.2-82.4%) (p < 0.001). Specificity was high for all criteria. The increase in sensitivity for the GC criteria was mainly due to the inclusion of 28 patients with progressive muscular atrophy (PMA).
The simpler GC criteria increase the sensitivity, primarily due to considering PMA as a form of ALS with high specificity preserved.
This validation study supports that GC criteria should be used in clinical practice and may be used for inclusion in trials.
本研究评估了“黄金海岸”（GC）诊断标准对肌萎缩侧索硬化症（ALS）的诊断准确性。
五个欧洲中心回顾性地选择了连续因疑似 ALS 而行肌电图检查的患者。根据 GC 标准、修订后的埃尔埃斯克里尔（rEE）标准和 Awaji（AW）标准，以及有无“可能”类别（+ Poss）对患者进行分类。参考标准是根据随访时疾病进展确定的 ALS。
404 名符合条件的患者中，272 例被诊断为 ALS，94 例为类似疾病，35 例失访，3 例数据不足。GC 标准的敏感性为 88.2%（95%CI：83.8-91.8%），高于以往的标准，其中 AW+Poss 标准的敏感性最高，为 77.6%（95%CI：72.2-82.4%）（p<0.001）。所有标准的特异性均较高。GC 标准敏感性的提高主要是由于纳入了 28 例进行性肌萎缩症（PMA）患者。
简化的 GC 标准提高了敏感性，主要是因为将 PMA 视为具有高特异性的 ALS 形式。
这项验证研究支持 GC 标准应在临床实践中使用，并且可能用于临床试验的纳入。
