Objective

This study evaluates diagnostic accuracy of the proposed ‘Gold Coast’ (GC) diagnostic criteria for amyotrophic lateral sclerosis (ALS).

Methods

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.

Results

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).

Conclusions

The simpler GC criteria increase the sensitivity, primarily due to considering PMA as a form of ALS with high specificity preserved.

Significance

This validation study supports that GC criteria should be used in clinical practice and may be used for inclusion in trials.

中文翻译：

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黄金海岸诊断标准提高了肌萎缩侧索硬化的敏感性

客观的

本研究评估了拟议的“黄金海岸”(GC) 肌萎缩侧索硬化 (ALS) 诊断标准的诊断准确性。

方法

五个欧洲中心对因怀疑 ALS 转诊进行肌电图检查的连续患者进行了回顾性抽样。根据 GC 标准、修订后的 El Escorial (rEE) 标准和淡路 (AW) 标准对患者进行分类，没有和有“可能”类别 (+可能)。参考标准是在随访时通过疾病进展确认的 ALS。

结果

在 404 名符合条件的患者中，272 名被诊断为 ALS，94 名患有模仿障碍，35 名失访，3 名数据不足。GC 标准的灵敏度为 88.2%（95% CI：83.8%-91.8%），高于之前的标准，其中 AW+Poss 标准达到了最高的灵敏度 77.6%（95% CI：72.2%- 82.4%) (p<0.001)。所有标准的特异性都很高。GC 标准敏感性的提高主要是由于纳入了 28 名进行性肌萎缩 (PMA) 患者。

结论

更简单的 GC 标准提高了灵敏度，这主要是因为将 PMA 视为一种保留了高特异性的 ALS。

意义

该验证研究支持 GC 标准应用于临床实践，并可用于纳入试验。