The Gold Coast criteria increases the diagnostic sensitivity for amyotrophic lateral sclerosis in a Chinese population

Abstract

Objectives 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. Methods 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). Results 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%. Conclusions 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.

Synucleinopathy in Amyotrophic Lateral Sclerosis: A Potential Avenue for Antisense Therapeutics?

“…The Gold Coast criteria, proposed in 2020, simplifies ALS diagnosis into a dichotomy, ALS or not ALS [ 37 , 38 ]. Through this method, the Gold Coast criteria are expected to correct sensitivity issues from past criteria, as well as facilitate early diagnosis in clinical practice [ 39 ]. Following diagnosis, the ALS Functional Rating Scale (ALSFRS) or the ALSFRS-revised (ALSFRS-R) have been the gold standard measure for monitoring the rate of change in a patient’s physical function over time [ 40 ].…”

Blood biomarkers in ALS : challenges, applications and novel frontiers

“…Biomarkers may also aid in the identification of a pre‐symptomatic and/or of a prodromal phase of the disease, where novel therapeutic approaches stand more chance to attenuate or revert a pathological process that is confined to a smaller number of motor cells. The latest Gold Coast diagnostic criteria propose that nerve conduction studies and electromyography (EMG); magnetic resonance imaging (MRI); and studies of blood and/or cerebrospinal fluid (CSF) for biomarkers of disease initiation and progression, should be used to exclude other disease processes, improve accuracy and timeliness of diagnosis, and to select more clinically homogeneous ALS patients for clinical trials 15–17 …”

Characterising ALS disease progression according to El Escorial and Gold Coast criteria

“…Second, patients without upper motor neuron dysfunction, but with lower motor neuron dysfunction in ≥2 body regions, will fulfil the diagnostic criteria for ALS 7. Consequently, the GCC have a higher sensitivity for ALS diagnosis compared with the rEEC, as the group of patients that is additionally diagnosed as ALS is proportionally larger than the group that does not fulfil the GCC 9–11. The GCC have, therefore, been proposed as a means of selecting participants for future clinical trials 11.…”

“…Also, patients with upper motor neuron dysfunction, but without lower motor neuron involvement, are no longer diagnosed as ALS. The additional number of patients fulfilling the GCC criteria exceeds the number no longer considered to have ALS,5 9–12 resulting in a net increase in the number of patients classifying as ALS.…”