Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative disease characterized by the progressive loss of motor neurons, which are nerve cells that control the voluntary muscles. This leads to symptoms that include overall muscle weakness, causing difficulties with moving, breathing, eating, and speaking.

ALS has no cure at present, but there are treatments to help manage the condition. Medications are available to help ease specific disease symptoms. 

The U.S. Food and Drug Administration (FDA) has approved three treatments that specifically aim to slow the progression of ALS: Rilutek (riluzole tablet), Tiglutik (riluzole suspension), and Radicava (edaravone).

Rilutek has also been approved in Canada, Australia, and across Europe. Tiglutik is approved in Europe under the name Teglutik. Radicava is still under review in many countries.

None of these treatments have been shown to reverse the damage already caused by ALS, but it is thought they might increase patients’ life expectancy. They often are taken in combination with symptomatic treatments to improve patients’ quality of life.

Rilutek (riluzole)

Rilutek is an oral tablet that can help delay the decline in breathing ability and extend life expectancy in those with advanced ALS, usually by three to five months.

The treatment is thought to prevent the release of glutamate, a neurotransmitter (cell-signaling molecule) thought to rise to toxic levels in the brain and spinal cord of ALS patients, damaging nearby nerve cells. By reducing the buildup of glutamate, it is thought that Rilutek prevents or slows the glutamate-induced deterioration of motor neurons.

Rilutek was approved by the FDA in 1995 as the first systemic treatment  to slow ALS progression and prolong survival. It remained the sole systemic ALS therapy in the U.S for more than 20 years, until Radicava became available.

Other countries also have approved the therapy to treat ALS. For example, Rilutek was approved in Europe by the European Medicines Agency (EMA) in 1996.

Tiglutik (riluzole oral suspension)

Tiglutik is an oral suspension that can be given using a syringe. Many ALS patients will develop dysphagia (trouble swallowing) as their disease progresses and this can lead to difficulty taking pills such as Rilutek.

Tiglutik has a similar proposed mechanism of action to Rilutek because both contain riluzole.

Radicava (edaravone)

Radicava is thought to work through a different process than Rilutek, although its exact mechanism of action is not fully understood. It is thought to act as an antioxidant or a so-called free radical scavenger. Free radicals are formed as a byproduct of energy production by the cell. Normally, free radicals are removed quickly by the body; if not, they can cause damage and lead to cell death through a process called oxidative stress. Oxidative stress is defined as an imbalance between free radical production and the antioxidants that work to neutralize them.

Damage from free radicals is thought to be one of the mechanisms in the progressive nerve cell death that marks ALS. It is thought that by helping to remove free radicals quickly, Radicava can reduce damage to the nervous system and slow disease progression.

Radicava, approved by the FDA in May 2017, is administered as an injection into a vein. The treatment also was approved for marketing by Health Canada as of October 2018. In Japan and South Korea, the treatment has been marketed under the brand name Radicut since 2015.

While Radicava is not yet available in Europe, the EMA accepted a new filing for edaravone on May 24, 2018, to consider marketing authorization.

Radicava is not currently available in Australia, and no application has been made to the Therapeutic Goods Administration (TGA) for its approval.

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ALS News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.