Diagnostic utility of Gold Coast criteria for amyotrophic lateral sclerosis in Asia

Abstract

Objective: This study aimed to reveal the diagnostic utility of Gold Coast (GC) criteria in Japanese patients with amyotrophic lateral sclerosis (ALS) by comparing the sensitivity/specificity with revised El Escorial (R-EE) and Awaji criteria, because its utility has not been studied in Asian ALS. Methods: Consecutive 639 patients (529 with ALS and 110 with ALS mimics), who were suspected of ALS and referred to three Japanese ALS centers, were enrolled. Diagnostic accuracy and characteristics of false positive and negative in GC criteria were compared with those of the Awaji and R-EE criteria. Patients were categorized as definite, probable or possible ALS according to each criterion. Results: The sensitivity of GC criteria (96.8%, 95% confidence interval [CI]: 95.3–98.3%) was higher than that of Awaji (89.6%, 95% CI: 87.0–92.2%) and R-EEC (89.2, 95% CI: 86.6–91.8%) criteria (both, p < 0.001). The specificity was also higher with GC criteria (77.3%, 95% CI: 69.5–85.1%) than Awaji (65.5%, 95% CI: 56.6–74.4%) and R-EEC (66.4, 95% CI: 57.6–75.2%) criteria (both, p < 0.01). Using GC criteria, patients with cervical spondylosis and Parkinson’s syndrome tended to be diagnosed with ALS (i.e. “false positive”). Additionally, ALS patients diagnosed only by GC criteria less frequently had upper motor neuron (UMN) signs, compared with the other two criteria. Conclusion: Gold Coast criteria improve diagnostic accuracy for ALS in an Asian population, especially in patients with subtle UMN signs.

Keywords:

• Amyotrophic lateral sclerosis
• Gold Coast criteria
• Awaji criteria
• El Escorial criteria

Acknowledgements

Kazumoto Shibuya grateful to Ryo Morishima, Tomoya Kawazoe, Yuri Asano, Daisuke Nakashima, and Kentaro Hayashi at the Tokyo Metropolitan Neurological Hospital for their cooperation in the collection date.

Ethical approval

This study was approved by the Ethics Committees of Chiba University School of Medicine, Tokyo Metropolitan Neurological Hospital and Graduate School of Medical Science, Kyoto Prefectural University of Medicine. In consultation with the Ethics Committee of each facility, consent was obtained using an opt-out approach.

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article. Drs. Shibuya, Shimizu, Noto, Suichi, Nakamura, and Aotsuka receive research support from the Ministry of Education, Culture, Sports, Science, and Technology of Japan. Dr. Kuwabara receives research support from the Ministry of Education, Culture, Sports, Science, and Technology of Japan, and Grants-in-Aid from the Research Committee of CNS Degenerative Diseases, the Ministry of Health, Labor and Welfare of Japan.