Abstract Details

Concordance between expert clinical diagnosis of Motor Neurone Disease and the Gold Coast, Awaji and EL-Escorial criteria – implications for clinical practice

Amyotrophic Lateral Sclerosis (ALS) is a progressive heterogenous neurodegenerative disease with a median survival of 3 years from symptom onset. International diagnostic criteria have been published, aiming both to expedite diagnosis, enabling patients to be referred as early as possible to a multidisciplinary specialist clinic, and to improve eligibility of patients for clinical trials.
The established revised El-Escorial (REE, 1998) and Awaji-Shima (AS, 2008) criteria have been in use. However, their suboptimal sensitivity and specificity motivated the development of a new international criteria in 2019: the Gold Coast (GC) Criteria.
In order to determine the sensitivity and specificity of the GC Criteria in the diagnosis of ALS in the setting of the National Health Service (NHS, United Kingdom), all referrals for neurophysiological examination in 2019 and 2020 received at the Royal Victoria Infirmary in Newcastle with the diagnostic question of ALS were analysed, together with clinical outcomes at follow up. Cases were then classified according to the three international criteria, and sensitivity and specificity were calculated.
203 cases were included in the analysis, of which 106 received a final diagnosis of ALS and 97 an alternative diagnosis. For the REE and AS Criteria, all three categories (possible, probable and definite) were considered as positive ALS diagnoses.
Sensitivity and specificity were highest for the GC Criteria (sensitivity 93.58%, specificity 100%) when compared to the REE and AS criteria (sensitivity 66.04%, specificity 100% for both).
We suggest that the improvement in specificity and sensitivity of the GC Criteria results from excluding Progressive Lateral Sclerosis from its diagnostic range, dichotomising the diagnoses into “ALS or not-ALS” and the greater weight given to the neurophysiological examination as evidence to support the diagnosis of ALS. Therefore, this study proposes that the GC Criteria should now be adopted in the United Kingdom.

TitleForenamesSurnameInstitutionLead AuthorPresenter
MrArunachalamSomaNewcastle University
DrTimothyWilliamsDepartment of Neurology, Royal Victoria Infirmary
DrMark BakerNewcastle University
DrHugo OliveiraNewcastle University
Reference
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