Hannaford et al. Diagnostic Utility of Gold Coast Criteria in Amyotrophic Lateral Sclerosis. Ann Neurol. 2021;89(5):979-986. doi:10.1002/ana.26045

The Gold Coast criteria were designed in 2020 as a new simplified framework for diagnosing Amyotrophic Lateral Sclerosis (ALS) (Shefner et al 2020). Our publication is the first to establish the accuracy of these new Gold Coast diagnostic criteria by applying them to 506 patients with possible ALS referred to two large ALS centers in Sydney (Westmead Hospital and Brain and Mind Center), Australia.

The Gold Coast criteria has abandoned the previous diagnostic categories of possible, probable, and definite ALS. These categories were commonly misinterpreted by patients and care givers as the likelihood that ALS was causing their symptoms. In fact, most people diagnosed with possible ALS according to these older criteria unfortunately continue to progress and finally die from ALS.

In our study 350 out of our 506 referred patients were ultimately proven to have ALS. We compared the accuracy of the Gold Coast criteria to the older revised El Escorial (rEEC) and Awaji criteria. The Gold Coast criteria was highly accurate in diagnosing ALS, which was comparable to the rEEC/Awaji, but importantly much simpler to use.

The manifestations of ALS are highly variable, with a number of subgroups such as those with predominant weakness of speech and swallowing known as “bulbar onset”. This variability is a key challenge to accurate diagnosis in ALS. Importantly, we were able to show that the Gold Coast criteria was highly accurate for diagnosing ALS in all of these varied subgroups, and in some categories outperformed the existing rEEC/Awaji criteria. In addition, the Gold Coast criteria were accurate across a range of disease severity and duration.

In summary, we have established the accuracy of these new Gold Coast criteria. Moving forward, it is anticipated they will lead to early and definitive diagnosis in ALS, and ultimately allow more patients to be fast-tracked into clinical trials.

References: Shefner JM, Al-Chalabi A, Baker MR, et al. A proposal for new diagnostic criteria for ALS. Clin Neurophysiol 2020;131:1975-1978.

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