Riluzole is indicated for use in ALS to extend life (on average by around three months) or the time to needing mechanical ventilation.

Its mode of action is thought to lie in inhibiting glutamate neurotransmitter processes in the CNS, with glutamate particularly involved in exciting motor neurones. Inhibition may protect cells from glutamate-mediated damage.^21,22

Tofersen, a short-chain nucleotide, is designed to address a fault with the SODI-1 gene which affects breakdown of toxic byproducts in normal cellular function, a gene fault seen in 2% of people with MND. Tofersen has limited availability in the UK through an Early Access Programme (EAP) and is awaiting NICE appraisal.^23,24,25