Abstract
Clinical trials for amyotrophic lateral sclerosis (ALS) have failed, except for riluzole, which is expected to prolong survival by 3 months without effects to the symptoms, and edaravone, which was shown to be efficacious to slightly retard the progression of the disease without data of prolonging survival. This has at least two factors: ALS is diverse in its pathogenesis as exemplified by increasing number of causative genes, and treatment is started too late to rescue remaining motor neurons. Awaji criteria were developed for earlier diagnosis of ALS, to facilitate entry of patients at early stages.
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