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Review
. 2024 Oct 1;37(5):570-576.
doi: 10.1097/WCO.0000000000001302. Epub 2024 Jul 22.

Diagnostic criteria for amyotrophic lateral sclerosis

Hannah C Timmins  1 , Alexandra E Thompson  1   2 , Matthew C Kiernan  1   3   4
Affiliations
Review

Diagnostic criteria for amyotrophic lateral sclerosis

Hannah C Timmins et al. Curr Opin Neurol. .

Abstract

Purpose of review: The present review will discuss the evolution of diagnostic criteria for amyotrophic lateral sclerosis (ALS) and biomarker considerations.

Recent findings: To address the limitations of existing ALS diagnostic criteria, a consortium of key stakeholders developed the Gold Coast consensus criteria (GCC). The GCC has similar or greater sensitivity compared with the revised El Escorial (rEEC) and Awaji criteria (AC), particularly for atypical phenotypes, maintained across disease duration, severity, and site of onset. In addition to improving diagnostic sensitivity, using the GCC in clinical trials may promote an increased enrolment of up to 50% of ALS patients who do not currently meet the full diagnostic eligibility requirements of the rEEC. Future inclusion of genetic biomarkers may mitigate some limitations of the GCC, to further improve diagnostic utility. In advance of such a process, validation of these biomarkers will be required before inclusion as additional criteria.

Summary: The GCC are simpler to use than previous consensus criteria, with demonstrated greater sensitivity and, enabling an earlier and more definitive ALS diagnosis, thereby facilitating wider enrolment into clinical trials. Broader implementation of the GCC in clinical trial settings is currently underway, globally.

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References

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    1. Michael S, David B, Martin RT, et al. Occasional essay: Upper motor neuron syndrome in amyotrophic lateral sclerosis, Journal of Neurology. Neurosurg Psychiatry 2020; 91:3357–3555.
    1. Turner MR, Eisen A, Kiernan MC, et al. Kinnier Wilson's puzzling features of amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2018; 89:657–666.
    1. Richards D, Morren JA, Pioro EP. Time to diagnosis and factors affecting diagnostic delay in amyotrophic lateral sclerosis. J Neurol Sci 2020; 417:117054.
    1. Ashhurst JF, Tu S, Timmins HC, Kiernan MC. Progress, development, and challenges in amyotrophic lateral sclerosis clinical trials. Expert Rev Neurother 2022; 22:905–913.

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