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2277 Diagnostic utility of gold coast criteria in amyotrophic lateral sclerosis
  1. Andrew Hannaford1,
  2. Nathan Pavey1,
  3. Mehdi Van den Bos1,
  4. Nimesh Geevasinga2,
  5. Parvathi Menon1,
  6. Jeremy Shefner3,
  7. Matthew Kiernan4 and
  8. Steve Vucic1
  1. 1Brain and Nerve Research Center, Concord Clinical School, University of Sydney, Concord, NSW, Australia
  2. 2Neurology, Westmead Hospital, Westmead, NSW, Australia
  3. 3Barrow Neurological Institute, Phoenix, AZ, USA
  4. 4Brain and Mind Center, University of Sydney, Sydney, NSW, Australia


Objective The diagnosis of amyotrophic lateral sclerosis (ALS) remains problematic, with current diagnostic criteria (revised El Escorial [rEEC] and Awaji) being complex and prone to error. Consequently, the diagnostic utility of the recently proposed Gold Coast criteria was determined in ALS.

Methods We retrospectively reviewed 506 patients (302 males, 204 females) to compare the diagnostic accuracy of the Gold Coast criteria to that of the Awaji and rEEC criteria (defined by the proportion of patients categorized as definite, probable, or possible ALS) in accordance with standards of reporting of diagnostic accuracy criteria.

Results The sensitivity of Gold Coast criteria (92%, 95% confidence interval [CI] = 88.7–94.6%) was comparable to that of Awaji (90.3%, 95% CI = 86.69–93.2%) and rEEC (88.6, 95% CI = 84.8–91.7%) criteria. Additionally, the Gold Coast criteria sensitivity was maintained across different subgroups, defined by site of onset, disease duration, and functional disability. In atypical ALS phenotypes, the Gold Coast criteria exhibited greater sensitivity and specificity.

Interpretation The present study established the diagnostic utility of the Gold Coast criteria in ALS, with benefits evident in bulbar and limb onset disease patients, as well as atypical phenotypes. The Gold Coast criteria should be considered in clinical practice and therapeutic trials.

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