To the Editor: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurodegenerative condition with no cure. Only two treatments with class I evidence exist — riluzole1 and edaravone2 — both with unclear mechanisms of action and modest survival benefits. In Australia, riluzole remains the only treatment approved by the Therapeutic Goods Administration. The Pharmaceutical Benefits Scheme limits initiation of riluzole to patients with at least 60% of predicted forced vital capacity, although facial weakness may make this an unreliable target. Initial and continuing treatment requires patients to be ambulant; or to have good upper limb function or to be able to swallow; and not to have respiratory failure.
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- 2. Abe K, Aoki M, Tsuji S, et al. Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double‐blind, placebo‐controlled trial. Lancet Neurol 2017; 16: 505–512.
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Matthew Kiernan is editor‐in‐chief of the Journal of Neurology, Neurosurgery and Psychiatry.