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Riluzole: real-world evidence supports significant extension of median survival times in patients with amyotrophic lateral sclerosis

Authors Hinchcliffe M, Smith A

Received 28 February 2017

Accepted for publication 21 April 2017

Published 29 May 2017 Volume 2017:7 Pages 61—70

DOI https://doi.org/10.2147/DNND.S135748

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 2

Editor who approved publication: Prof. Dr. Thomas Müller

Michael Hinchcliffe,1 Alan Smith2

1Paracelsis Ltd, 2PharmaSci Consulting Ltd, Nottingham, UK

Abstract: Amyotrophic lateral sclerosis (ALS) is the commonest form of motor neuron disease and is a fatal, degenerative, multisystem disorder affecting upper and/or lower motor neurons in the motor cortex, brain stem, and spinal cord. ALS is characterized by progressive atrophy of associated bulbar, limb, thoracic, and abdominal muscles and supporting cells manifesting in a range of muscular symptoms such as weakness and wasting and eventual paralysis; the majority of patients will die from respiratory failure within 2–5 years of onset. Riluzole, a synthetic benzothiazole drug with glutamine antagonist activity, is indicated for the treatment of patients with ALS and is the only drug that has been shown to slow the course of the disease and extend survival in ALS patients. The original analyses, and subsequent meta-analyses, of data obtained from randomized controlled trials (RCTs) suggest that riluzole typically extends survival by 2–3 months and increases the chance of an additional year of survival by ~9%. However, published real-world evidence (RWE) from 10 clinical ALS databases indicates that riluzole therapy may afford much greater extension of survival, and improvements in median survival times of more than 19 months have been reported in the overall ALS patient population. This article will review the available data from RCTs and RWE on riluzole therapy.

Keywords: ALS, survival, randomized controlled trial, RCT, RWE

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