(FDA approved in bold)

• Amyotrophic lateral sclerosis (ALS)

• The mode of action is unknown, but the effect in ALS is felt to be from glutamate antagonism. Putative mechanisms include inhibition of glutamate release, interference with transmitter binding at excitatory amino acid receptors, and inactivation of voltage-gated sodium channels. In animal models, appears to be neuroprotective in mice with human superoxide dismutase mutations and has sedative and myorelaxant properties at large doses

• Steady state is reached in 5 days, but it can take months to assess any clinical effect from the drug

• ALS is a degenerative disease and deterioration is the general rule. Riluzole can increase survival or time to tracheostomy but is not a cure

• It is difficult to determine if the treatment is effective, especially because ALS progression varies greatly from patient to patient. Supportive care is the mainstay of current ALS treatment. This may include monitoring and treatment of gait, swallowing and respiratory difficulties

• No other medication is indicated for the treatment of ALS progression

• Measure serum transaminases, including ALT levels, at baseline and monthly for 3 months. Then evaluate every 3 months for the first year and periodically after that. Once ALT exceeds 5 times normal, begin checking weekly, and discontinue if ALT exceeds 10 times normal or clinical symptoms, such as jaundice, occur