The simplified ‘Gold Coast’ criteria for the diagnosis of amyotrophic lateral sclerosis (ALS) should be implemented into clinical practice and clinical trial settings, its developers say.
According to an opinion article in Muscle & Nerve, authored by an international team including Australians Professor Steve Vucic and Professor Matthew Kiernan, the Gold Coast criteria merged four diagnostic categories from previous models into a single ‘presence of ALS’ entity.
The criteria, established at an international consensus meeting held on the Gold Coast in 2019, has also been tested retrospectively in a cohort of more than 500 Australians with suspected ALS attending two major ALS centres in Sydney.
The study, published early this year in Annals of Neurology, confirmed 350 cases of ALS after “extensive clinical, laboratory, neuro-physiological, and radiological assessment” including >6 months follow-up for evidence of progression.
The study found the sensitivity of the Gold Coast criteria (92%) was comparable to the Awaji (90.3%; p = 0.39) and revised El Escorial (88.6%; p = 0.15) criteria.
The specificity of the Gold Coast criteria also remained high (88.5%), although it was lower than that of the Awaji (95.5%) and rEEC criteria (96.2%).
“The diagnostic utility of the Gold Coast criteria was maintained irrespective of functional status, disease duration or site of disease onset,” the Muscle & Nerve article said.
The Gold Coast criteria also effectively excluded people with primary lateral sclerosis (PLS).
The authors said the Gold Coast criteria had demonstrated clinical utility.
“Importantly, clinical utility may be evident even in the absence of effective therapies, in that diagnostic uncertainty is resolved, thereby enabling patients and their carers to plan and cope with the illness.”
“As such, earlier diagnosis may lead to improvement in daily functioning and quality of life in ALS patients, as well as better outcomes in some clinical trials.”
“Furthermore, patients will be prevented from undergoing unnecessary additional investigations, thereby reducing any further burden.”
Speaking to the limbic, Professor Steve Vucic from Westmead Hospital and the University of Sydney, said the previous diagnostic categories were confusing and didn’t have much meaning.