Tag: Amyotrophic lateral sclerosis

Riluzole was the first drug approved by the FDA to treat amyotrophic lateral sclerosis (ALS) [1.7.3]. For patients and their families, a primary concern is understanding the financial aspect of this treatment, specifically: **How much does riluzole cost for ALS?**

Every year, approximately 5,000 people in the U.S. receive a diagnosis of Amyotrophic Lateral Sclerosis (ALS), a progressive and fatal neurodegenerative disease. When faced with this news, many want to know, 'What is the best drug for ALS?' The answer is not a single drug, but a personalized treatment plan combining multiple medications and supportive therapies.

In the United States, approximately 2% of ALS cases are the genetic form known as SOD1-ALS [1.8.3]. For these patients, a targeted therapy approved in 2023 represents a major advancement. So, what is the newest drug for ALS, and how does it fit into the evolving landscape of treatment?

Motor neuron disease (MND) is a progressive neurological disorder that damages motor neurons over time. The medication most commonly identified as an MND tablet is Riluzole, which is approved to help slow the disease's progression, particularly in patients with amyotrophic lateral sclerosis (ALS), the most common form of MND.

Approved by the FDA in 1995, riluzole was the first drug available for treating amyotrophic lateral sclerosis (ALS) and is one of the only medications shown to slow the disease's progression [1.8.4, 1.8.5]. So, **what is the pharmacology and mechanism of action of riluzole** that grants it this neuroprotective effect?

In a combined analysis of the VALOR trial and its open-label extension, starting tofersen earlier slowed the decline in clinical function, respiratory function, and muscle strength in SOD1-ALS patients compared to delayed initiation. This critical finding helps clarify **how effective is tofersen** beyond the initial trial results.