What treatment options are available for ALS with speech impairment?

Difficulties with speech (dysarthria) and swallowing (dysphagia) are common symptoms of ALS and are experienced as particularly distressing. With dextromethorphan/quinidine (DMC) and pyridostigmine, medication is available that can improve or stabilize the symptoms. The response to the medication can vary greatly from individual to individual and is to be expected above all for mild and moderate symptoms.

DMC is approved in the USA for the symptomatic treatment of ALS and is available there under the name “Nuedexta”. In Europe (and Germany), DMC can be produced and supplied by ALS pharmacies on the basis of a doctor’s prescription. The drug acts on the connection (synapses) between motor nerves in the bulbar brain, where speech and swallowing functions are regulated. DMC is a drug from the group of NMDA antagonists that influence the neurotransmitter glutamate. DMC is particularly effective in cases of increased muscle tension (spasticity, “pseudobulbar symptoms”).

A small clinical study (24 subjects, observation interval 28 days) showed a 12% improvement in bulbar symptoms with DMC. The expected effect is moderate, but may represent a relief of the stressful symptoms.

Pyridostigmine is another treatment option – a drug that is approved for the treatment of the muscle disease myasthenia. The drug targets the connection between motor nerves and the muscles (neuromuscular endplate) and leads to a strengthening of the existing muscles. Pyridostigmine is preferred when there is weakness without recognizable spasticity of the tongue and pharyngeal muscles (bulbar symptoms without a pseudobulbar component). Due to the pharmacological properties of pyridostigmine, increased salivation may occur as a side effect, which is particularly undesirable in ALS. This potential side effect requires an additional medication against salivation or discontinuation of pyridostigmine.