From the Guidelines
Motor neuron diseases, such as Amyotrophic Lateral Sclerosis (ALS), should be diagnosed and managed with a multidisciplinary approach, focusing on supportive and symptomatic treatment, as the current evidence suggests that this approach improves quality of life and modestly slows disease progression 1. The most common motor neuron disease is ALS, representing 85% of all cases, with an overall annual incidence of 1 to 2/100,000 and a median survival of 3 to 4 years after symptom onset 1. Key characteristics of motor neuron diseases include:
- Degeneration of upper and lower motor neurons in the brain and spinal cord
- Hypertonicity and hyperreflexia (upper motor neuron degeneration)
- Muscle fasciculations, weakness, and atrophy (lower motor neuron degeneration) Electromyography and nerve conduction velocity are essential tests in diagnosing ALS, while imaging is mainly used to exclude other conditions with similar clinical presentations 1. Treatment options for ALS include:
- Riluzole (50mg twice daily)
- Edaravone These medications have been shown to modestly slow disease progression, and their use should be considered in the management of motor neuron diseases 1. A multidisciplinary care approach, involving neurologists, respiratory therapists, physical therapists, speech therapists, and nutritionists, is crucial for managing symptoms and maintaining quality of life for patients with motor neuron diseases.
From the FDA Drug Label
Riluzole tablets is indicated for the treatment of amyotrophic lateral sclerosis (ALS). The answer to the question about motor neuron diseases is that Riluzole is indicated for the treatment of Amyotrophic Lateral Sclerosis (ALS), which is a type of motor neuron disease.
- Key points:
- Riluzole is used to treat ALS.
- ALS is a type of motor neuron disease.
- The drug label does not provide information on other motor neuron diseases. 2
From the Research
Motor Neuron Diseases
- Motor neuron diseases, such as amyotrophic lateral sclerosis (ALS), are progressive neurodegenerative diseases caused by the death of motor neurons.
- Riluzole is a benzothiazole derivative that blocks glutamatergic neurotransmission in the CNS, which is thought to exert neuroprotective effects 3.
- Riluzole has been approved for the treatment of ALS in most countries, but questions persist about its clinical utility due to high cost and modest efficacy 4, 5, 6.
Efficacy of Riluzole
- Riluzole 100 mg per day has been shown to provide a benefit in prolonging tracheostomy-free survival in patients with ALS, with a 9% gain in the probability of surviving one year 4, 5, 6.
- The treatment effect of riluzole is modest, prolonging median survival by about two to three months in patients with ALS 4, 5, 6.
- Riluzole has also been shown to have a small beneficial effect on both bulbar and limb function, but not on muscle strength 4, 5, 6.
Safety and Tolerability
- Riluzole is generally safe and well tolerated in clinical practice, with a threefold increase in serum alanine transferase being more frequent in riluzole-treated patients than controls 4, 5, 6.
- Riluzole treatment has been shown to be well tolerated in humans and animals, with relatively minor effects on normal respiration parameters 7.
Mechanism of Action
- The mechanism of action of riluzole in treating ALS is not fully understood, but it is thought to involve the inhibition of glutamatergic neurotransmission in the CNS 3, 7.
- Riluzole has been shown to have a wide range of effects on factors influencing neural activity, including the inhibition of persistent Na(+) current, potentiation of calcium-dependent K(+) current, and inhibition of neurotransmitter release 7.