Plateaus in amyotrophic lateral sclerosis progression: results from a population‐based cohort. (22nd May 2020)

• Record Type: Journal Article
• Authors: Vasta, R.; D'Ovidio, F.; Canosa, A.; Manera, U.; Torrieri, M. C.; Grassano, M.; De Marchi, F.; Mazzini, L.; Moglia, C.; Calvo, A.; Chiò, A.
• Is Part Of: European journal of neurology. Volume 27:Number 8(2020)
• Page Start: 1397
• Page End: 1404
• Publication Date: 2020-05-22
• Subjects: amyotrophic lateral sclerosis -- cohort studies -- epidemiology -- motor neuron diseases -- progression analysis; Neurology -- Periodicals; Nervous system -- Diseases -- Periodicals; 616.8
• DOI: 10.1111/ene.14287
• Languages: English
• ISSNs: 1351-5101

Abstract

Abstract : Background and purpose: The aim was to assess the frequency of plateaus in amyotrophic lateral sclerosis (ALS) progression using a large population‐based cohort. Methods: Data from the Piemonte and Valle d'Aosta ALS register were used. Patients who were diagnosed between 2007 and 2014 were considered. The follow‐up period was extended until 31 December 2018. Visits after tracheostomy were excluded. A plateau was defined as a stable Amyotrophic Lateral Sclerosis Functional Rating Scale revised (ALSFRSr) score lasting at least 6, 12 or 18 months. Results: Out of 1214 patients, 200 (16.5%), 93 (7.7%) and 52 (4.3%) showed at least one plateau lasting a minimum of 6, 12 and 18 months, respectively. Plateaus occurred mostly at high ALSFRSr scores and were more frequent during the initial phases of the disease course. Spinal onset [odds ratio (OR) 1.83, 95% confidence interval (CI) 1.16–2.95, P value 0.01) and predominant upper motor neuron phenotype (OR 2.18, 95% CI 1.36–3.48, P value 0.001) conferred a higher risk for the subsequent appearance of plateaus; conversely, older age at diagnosis (OR 0.25, 95% CI 0.11–0.54, P value 0.002 for >75 year age class) reduced this risk. Conclusions: Plateaus in ALS progression lasting at least 6 months appear in about one out of six patients and could last even 12, 18 months or more in a smaller subgroup of patients. Plateau occurrence should not lead the neurologist to automatically reconsider ALS diagnosis and should be considered for future clinical trial design.