Newer ALS diagnostic criteria more sensitive than older approaches
Study findings support clinical adoption of the Gold Coast criteria
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- ALS involves progressive motor neuron damage, causing symptoms like voluntary movement loss and muscle weakness.
- The Gold Coast criteria demonstrated superior sensitivity in diagnosing ALS compared to older methods.
- Clinical adoption of the Gold Coast criteria should serve as the standard for prompt and accurate ALS diagnosis.
A newer set of diagnostic criteria for amyotrophic lateral sclerosis (ALS) was significantly more sensitive at identifying people with the disease than two older diagnostic approaches, a real-world study in Germany found.
Among more than 400 people ultimately diagnosed with ALS, these Gold Coast criteria correctly identified 92.5% of ALS cases, compared with less than 75% using the older Awaji or revised El Escorial criteria.
Even when researchers added markers of nerve cell damage that are typically elevated in people with ALS to the older criteria, their sensitivity still did not match that of Gold Coast.
The findings support the clinical adoption of the Gold Coast criteria, which have been increasingly, but not universally, used in recent years.
“Continued clinical use of the [Gold Coast criteria] should be considered the standard for ALS diagnosis,” researchers wrote in the study, “Gold coast criteria but not neurofilaments improve diagnostic sensitivity in a specialized ALS university center,” which was published in Clinical Neurophysiology Practice.
ALS diagnostic criteria has evolved over the years
Diagnosing ALS involves looking for hallmark signs of progressive damage to motor neurons, the nerve cells controlling voluntary movement, while ruling out conditions that can cause similar symptoms. Because there is no single test that confirms ALS, diagnosis is often delayed.
To guide the diagnostic process, several sets of criteria have been developed. The revised El Escorial criteria, initially published in 1994 and updated in 2000, classify patients as having definite, probable, or possible ALS based largely on evidence of disease progression and signs of both upper and lower motor neuron damage.
The Awaji criteria were introduced in 2008 to improve diagnostic sensitivity by assigning signs of lower motor neuron damage detected by electromyography as equivalent to findings from a neurological exam.
The Gold Coast criteria, emerging in 2020, further simplified the diagnosis by classifying a person as having ALS or not, rather than assigning different levels of certainty. Diagnosis requires progressive motor impairment plus evidence of both upper and lower motor neuron damage, or lower motor neuron damage alone in at least two body regions. These criteria are thought to be more sensitive to catching early cases of ALS.
Gold Coast criteria showed the highest sensitivity
Researchers at Hannover Medical School in Germany set out to directly compare how well the three sets of criteria identified ALS in routine clinical practice. They retrospectively analyzed data from 431 adults with suspected ALS referred to a specialized neuromuscular center for comprehensive diagnostic testing between August 2010 and July 2023. Ultimately, 426 were diagnosed with ALS, a median of one year after symptoms began.
Researchers evaluated how many of these patients would have qualified for an ALS diagnosis under each set of diagnostic criteria.
The Gold Coast criteria showed the highest sensitivity — the ability to correctly identify people with ALS — at 92.5%, compared with 72.5% for the Awaji criteria and 71.1% for the revised El Escorial criteria. Even when “possible ALS” was counted as a diagnosis under the older criteria, their sensitivity remained lower, at about 84%.
Gold Coast also performed well among people who had experienced symptoms for less than 18 months, identifying 93% of ALS cases. Their sensitivity was particularly high for people whose symptoms began in the limbs and those with predominantly lower motor neuron involvement.
Biomarkers such as neurofilaments could be used to refine the diagnostic criteria, ensuring coverage of the entire ALS spectrum.
That could be because the Gold Coast criteria can diagnose ALS based on lower motor neuron damage in at least two body regions without upper motor neuron signs, according to the authors. However, Gold Coast was less sensitive than older criteria in people with predominantly upper motor neuron involvement.
Adding blood levels of neurofilament light chain (NfL) and/or spinal fluid levels of phosphorylated neurofilament heavy chain (pNfH) — two biomarkers of nerve damage often elevated in ALS — to the older criteria did not increase their sensitivity beyond that of the Gold Coast criteria. In 127 patients analyzed, the modified Awaji and revised El Escorial criteria each had a sensitivity of 89%, compared with 97.3% for Gold Coast.
Still, neurofilament measurements may help in some cases that Gold Coast misses. Of three such patients with available data, two had markedly elevated levels of both NfL and pNfH and pure upper motor neuron involvement, suggesting these biomarkers could complement current diagnostic criteria in certain patients.
Overall, the researchers concluded that the Gold Coast criteria “may facilitate early diagnosis of ALS,” while “biomarkers such as neurofilaments could be used to refine the diagnostic criteria, ensuring coverage of the entire ALS spectrum.”
“Prospective multicenter studies are needed to confirm these findings and to further define the role of biomarkers within diagnostic criteria for ALS,” the team added.
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