Gold Coast Criteria in ALS Diagnosis: A Real-World Experience
<b>Background:</b> Revised El Escorial (rEEC) and Awaji criteria are currently used for diagnosing and categorizing amyotrophic lateral sclerosis (ALS). However, they are complex; their sensitivity is still not optimal for research purposes, and they present high inter-rater variability...
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MDPI AG
2024-10-01
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| author | Lucia Ferullo Barbara Risi Filomena Caria Emanuele Olivieri Loris Poli Stefano Gazzina Ugo Leggio Enrica Bertella Giorgia Giovanelli Beatrice Labella Alessandro Padovani Massimiliano Filosto |
| author_facet | Lucia Ferullo Barbara Risi Filomena Caria Emanuele Olivieri Loris Poli Stefano Gazzina Ugo Leggio Enrica Bertella Giorgia Giovanelli Beatrice Labella Alessandro Padovani Massimiliano Filosto |
| author_sort | Lucia Ferullo |
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| description | <b>Background:</b> Revised El Escorial (rEEC) and Awaji criteria are currently used for diagnosing and categorizing amyotrophic lateral sclerosis (ALS). However, they are complex; their sensitivity is still not optimal for research purposes, and they present high inter-rater variability in clinical practice. To address these points, in 2019, a new set of diagnostic criteria was proposed, namely the Gold Coast criteria (GCC), characterized by a dichotomous diagnostic categorization, i.e., ALS or not ALS. <b>Methods:</b> In order to investigate the sensitivity, specificity, and clinical usefulness of GCC in a practical clinical setting, we retrospectively evaluated 131 patients diagnosed with ALS and 104 control subjects. ALSFRS-R score, electrophysiological tests, neuroradiological investigations, and CSF analysis were obtained. rEEC, Awaji, and GCC were applied at the first and last evaluations. <b>Results:</b> The sensitivity of GCC (93.1%; 96.1%) was greater than rEEC (71.8%; 87%) and Awaji criteria (77.8%; 89.3%) both at the first visit and last follow-up. The GCC’s specificity (28.8%) is lower than that of the other two criteria (rEEC 45.2%; Awaji 43.3%). <b>Conclusions:</b> Our study suggests that in a real-world setting, the GCC are more sensitive and have substantially lower risk of false negative diagnoses than rEEC and Awaji criteria. Although rEEC had the highest specificity, they may delay the diagnosis. Systematically using the GCC could help to achieve an earlier diagnosis and quickly refer patients to the correct management. The low specificity of GCC is likely to not significantly impact patient recruitment in clinical trials; therefore, its use might allow a faster and earlier enrollment. |
| format | Article |
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| institution | Kabale University |
| issn | 2076-3425 |
| language | English |
| publishDate | 2024-10-01 |
| publisher | MDPI AG |
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| series | Brain Sciences |
| spelling | doaj-art-b59a89f9b1c542a8bc137e00f8d5b3d92024-11-26T17:54:47ZengMDPI AGBrain Sciences2076-34252024-10-011411105510.3390/brainsci14111055Gold Coast Criteria in ALS Diagnosis: A Real-World ExperienceLucia Ferullo0Barbara Risi1Filomena Caria2Emanuele Olivieri3Loris Poli4Stefano Gazzina5Ugo Leggio6Enrica Bertella7Giorgia Giovanelli8Beatrice Labella9Alessandro Padovani10Massimiliano Filosto11Department of Clinical and Experimental Sciences, University of Brescia, 25121 Brescia, ItalyNeMO-Brescia Clinical Center for Neuromuscular Diseases, 25064 Brescia, ItalyNeMO-Brescia Clinical Center for Neuromuscular Diseases, 25064 Brescia, ItalyDepartment of Clinical and Experimental Sciences, University of Brescia, 25121 Brescia, ItalyUnit of Neurology, ASST Spedali Civili, 25123 Brescia, ItalyUnit of Neurology, ASST Spedali Civili, 25123 Brescia, ItalyUnit of Neurology, ASST Spedali Civili, 25123 Brescia, ItalyNeMO-Brescia Clinical Center for Neuromuscular Diseases, 25064 Brescia, ItalyNeMO-Brescia Clinical Center for Neuromuscular Diseases, 25064 Brescia, ItalyDepartment of Clinical and Experimental Sciences, University of Brescia, 25121 Brescia, ItalyDepartment of Clinical and Experimental Sciences, University of Brescia, 25121 Brescia, ItalyDepartment of Clinical and Experimental Sciences, University of Brescia, 25121 Brescia, Italy<b>Background:</b> Revised El Escorial (rEEC) and Awaji criteria are currently used for diagnosing and categorizing amyotrophic lateral sclerosis (ALS). However, they are complex; their sensitivity is still not optimal for research purposes, and they present high inter-rater variability in clinical practice. To address these points, in 2019, a new set of diagnostic criteria was proposed, namely the Gold Coast criteria (GCC), characterized by a dichotomous diagnostic categorization, i.e., ALS or not ALS. <b>Methods:</b> In order to investigate the sensitivity, specificity, and clinical usefulness of GCC in a practical clinical setting, we retrospectively evaluated 131 patients diagnosed with ALS and 104 control subjects. ALSFRS-R score, electrophysiological tests, neuroradiological investigations, and CSF analysis were obtained. rEEC, Awaji, and GCC were applied at the first and last evaluations. <b>Results:</b> The sensitivity of GCC (93.1%; 96.1%) was greater than rEEC (71.8%; 87%) and Awaji criteria (77.8%; 89.3%) both at the first visit and last follow-up. The GCC’s specificity (28.8%) is lower than that of the other two criteria (rEEC 45.2%; Awaji 43.3%). <b>Conclusions:</b> Our study suggests that in a real-world setting, the GCC are more sensitive and have substantially lower risk of false negative diagnoses than rEEC and Awaji criteria. Although rEEC had the highest specificity, they may delay the diagnosis. Systematically using the GCC could help to achieve an earlier diagnosis and quickly refer patients to the correct management. The low specificity of GCC is likely to not significantly impact patient recruitment in clinical trials; therefore, its use might allow a faster and earlier enrollment.https://www.mdpi.com/2076-3425/14/11/1055amyotrophic lateral sclerosismotor neuron diseaseGold Coast criteriarevised El Escorial criteriaAwaji criteria |
| spellingShingle | Lucia Ferullo Barbara Risi Filomena Caria Emanuele Olivieri Loris Poli Stefano Gazzina Ugo Leggio Enrica Bertella Giorgia Giovanelli Beatrice Labella Alessandro Padovani Massimiliano Filosto Gold Coast Criteria in ALS Diagnosis: A Real-World Experience Brain Sciences amyotrophic lateral sclerosis motor neuron disease Gold Coast criteria revised El Escorial criteria Awaji criteria |
| title | Gold Coast Criteria in ALS Diagnosis: A Real-World Experience |
| title_full | Gold Coast Criteria in ALS Diagnosis: A Real-World Experience |
| title_fullStr | Gold Coast Criteria in ALS Diagnosis: A Real-World Experience |
| title_full_unstemmed | Gold Coast Criteria in ALS Diagnosis: A Real-World Experience |
| title_short | Gold Coast Criteria in ALS Diagnosis: A Real-World Experience |
| title_sort | gold coast criteria in als diagnosis a real world experience |
| topic | amyotrophic lateral sclerosis motor neuron disease Gold Coast criteria revised El Escorial criteria Awaji criteria |
| url | https://www.mdpi.com/2076-3425/14/11/1055 |
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