Gold Coast Criteria Diagnostic Accuracy in Amyotrophic Lateral Sclerosis: A Systematic Review and Meta-analysis
Enzo Von Quednow1, Aishwarya Koppanatham2, Paweł Łajczak3, Luini Garcia-Quiroa4
1Albacete University Hospital Complex, 2Andhra Medical College, 3Medical University of Silesia, 4Brigham Young University
Objective:
To perform a meta-analysis of the literature comparing the diagnostic accuracy of the Gold Coast Criteria (GCC) with Revised El Escorial Criteria (rEEC) and Awaji Criteria (AC) in the diagnosis of amyotrophic lateral sclerosis (ALS).
Background:

Diagnosing ALS remains challenging due to the lack of definitive biomarkers, prompting the development of clinical criteria, such as the EEC, its revisions, and later the AC. However, these criteria showed high specificity but low sensitivity. In 2020, the GCC were introduced to simplify diagnosis and improve early-stage sensitivity.

Design/Methods:

We performed a systematic literature search in PubMed-MEDLINE, EMBASE, and Cochrane databases until September 2024. We focused on studies comparing GCC with rEEC and/or AC in the entire study population to minimize heterogeneity. We defined "possible+" which includes possible, probable, and definite categories for AC, and possible, probable, probable laboratory-supported and definite for rECC. “Probable+” includes probable and definite categories for AC, and probable, probable laboratory-supported and definite for rECC. Summary receiver operating characteristic (sROC) curves were constructed, and areas under the curve (AUCs) were calculated.

Results:

Four studies met inclusion criteria, comprising 2696 patients. SROC curves for GCC showed superior sensitivity 94.3% (95%CI:0.903, 0.967) with specificity 82.4% (95%CI:0.353, 0.976). AC “possible+” category demonstrated a pooled sensitivity 86.6% (95%CI:0.821, 0.901) and specificity 84.3% (95%CI:0.454, 0.972). AC “probable+” category exhibited lower sensitivity 55.8% (95%CI:0.456, 0.655) but high specificity 99.0% (95%CI:0.639, 1.0). And rEEC “possible+” category had a sensitivity 85.7% (95%CI:0.811, 0.892) and specificity 85.0% (95%CI:0.459, 0.974), while rEEC “probable+” category reflected sensitivity 59.6% (95%CI:0.457, 0.720) and specificity 99.0% (95%CI:0.505, 1.0).

Conclusions:
The GCC showed greater sensitivity than AC and rEEC in diagnosing ALS, highlighting its potential for improving early-stage detection. Our findings suggest GCC should be used for ALS diagnosis in clinical practice and trial inclusion, however, further studies with larger populations are needed to confirm these results.
10.1212/WNL.0000000000210856
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