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ALS is a rare neurological disease without a cure. However, there are drug treatments that can help relieve symptoms of the disease such as the FDA-approved riluzole and edaravone. This article outlines drug treatments that are available for ALS patients.

Unfortunately, there is no cure for ALS. However, scientists have developed treatments that can help manage the disease. In particular, there are two FDA-approved medications for ALS that, while not being able to reverse disease progression, have been shown to improve patient survival. These two medications are called riluzole (Rilutek) and edaravone (Radicava).

There are also other medications that are often prescribed to patients to help ease specific disease symptoms. In fact, riluzole and edaravone are often taken in combination with symptomatic treatments to improve quality of life. These medications include quinine bisulphate (Qualaquin), baclofen (Gablofen), and tizanidine (Zanaflex), gabapentin (Neurontin), Neurodex, tricyclic antidepressants, nonsteroidal anti-inflammatory drugs (NSAIDs), glycopyrrolate (Robinul), trihexyphenidyl (Artane) and morphine.

Here is a list of medications that can be prescribed to patients with ALS:

1. Riluzole

  • Riluzole is an oral drug that has been shown to slow down the decline in breathing ability and prolong life expectancy in patients with advanced ALS. The average length of time through which riluzole extends life expectancy is three to five months.
  • Riluzole works by preventing the buildup of a chemical called glutamate, which is present in high levels in the brain and spinal cord of patients with ALS. By decreasing levels of glutamate, researchers believe that riluzole stops or slows the deterioration of motor neurons.
  • The FDA granted approval to riluzole in 1995 as the first treatment that can slow down ALS progression and increase survival. Since then, other countries, such as Canada, Australia, and most European countries, have granted approval to riluzole for treatment of ALS.

2. Edaravone

  • Edaravone is administered through an injection into a vein and works to improve the condition through a different mechanism from riluzole. However, edaravone’s exact mechanism is not fully understood at this time.
  • Researchers postulate that edaravone improves ALS by working as an antioxidant, which attacks molecules called free radicals. Free radicals are toxic molecules that are naturally formed in cells but are quickly removed from the body. However, if they are not expelled from the body then they can cause cell damage. Hence, free radicals are thought to contribute to the progressive nerve cell death that is seen in ALS. Researchers believe that edaravone works by removing these free radicals, thereby reducing damage to the nervous system and slowing down disease progression.
  • Edaravone was approved by the FDA in 2017, and later by Health Canada in 2018. Furthermore, edaravone has also been approved in Japan and South Korea, where it is marketed under the brand name Radicut. Currently, edaravone is not available in Europe, though the European Medicines Agency (EMA) has accepted the application for approval of edaravone in 2018. edaravone is also not available in Australia.

3. Baclofen

  • Baclofen helps relieves muscle spasms by relaxing the body’s muscles.
  • Studies have shown that baclofen is especially effective at relieving muscle spasms when it is used alongside assisted or unassisted range of motion physical therapy.
  • It is imperative to closely monitor baclofen dosing in order to avoid limb and torso weakness which can develop when patients are given high doses (such as 40 to 80 mg) too soon.
  • When a patient stops taking baclofen, then can often develop withdrawal and must be monitored for signs of hallucinations, insomnia, nausea, anxiety and other side effects.

4. Tizanidine

  • Tizanidine helps relieve muscle spasms by relaxing muscles.
  • Tizanidine works by blocking nerve signals that are sent from the brain to the muscle.
  • Tizanide is administered at doses that range from 2-10 mg daily.
  • In general, it is thought that tizanide is the best of anti-spasmodic, muscle relaxation option for patients with ALS.
  • While uncommon, side effects can sometimes include weakness, constipation, dizziness, and other issues.

5. Quinine bisulphate

  • Quinine bisulphate has been shown to be effective for muscles symptoms, particularly rigidity and spasticity (continuous muscle contraction).  
  • Quinine bisulphate has been shown to be as at least as efficacious as baclofen as an anti-spasmodic medication (a type of medicine that helps relax muscles).
  • It is available in 324 mg capsules at local pharmacies and can be taken 1-2 times per day.
  • Generally, quinine bisulphate is used when tizanidine and/or baclofen have not been successful for patients with ALS.
  • Side effects to the medication can include allergic reaction, thrombosis (formation of a blood clot inside a blood vessel) or kidney issues.

6. NSAIDs

  • As inflammation is thought to contribute to the development and progression of ALS, researchers have postulated that NSAIDs (which are anti-inflammatory) can have a protective effect. However, several clinical trials have not found any beneficial effects on the overall survival of ALS patients
  • NSAIDs, such as ibuprofen or naproxen, may help relieve general pain and discomfort. However, due to the potential gastrointestinal and cardiovascular side effects, NSAIDs should only be taken as instructed.

7. Gabapentin

  • Gabapentin is used an anti-seizure medication for diseases such as epilepsy, but can also be used for ALS.
  • Animal studies have shown that gabapentin improves survival and clinical trials have shown that it decreases muscle cramps and twitching in ALS patients.
  • Gabapentin works by regulating glutamate levels (similar to riluzole).
  • Side effects for gabapentine include tiredness, weight gain, indigestion, drowsiness, dizziness, ataxia, and tremor.

8. Neurodex

  • Neurodex is a a combination of vitamins B-1, B-6, and B-12
  • Neurodex is used as a treatment of pseudobulbar effect, an usual symptom in patients with ALS in which they experience uncontrollable and extreme emotions.

9. Tricyclic antidepressants

  • These drugs are widely used for treatment of ALS as they have multiple effects.
  • In particular, depression and anxiety are common in ALS and an adequate dose of a tricyclic agent can relieve depression.
  • Side effects of these drugs, such as dry mouth and weight gain, can also help other ALS symptoms such as too much saliva in the mouth and weight loss.

10. Glycopyrrolate

  • Glycopyrrolate can help reduce excessive saliva, which is a frustrating ALS to manage.
  • The recommended dose of Glycopyrrolate is 1-2 mg every four hours, and the drug is also available in injectable form of 0.1 mg every four hours or 3-4 times/day.

11. Trihexyphenidyl

  • Trihexyphenidyl also helps manage excessive saliva.
  • Side effects of trihexyphenidyl can include cognitive issues, dry mouth, dizziness, sleepiness and hallucinations.

12. Morphine

  • Morphine, a type of opioid, can be used to treat pain.
  • Morphine can help the feeling of not getting enough air, which occurs in patients with advanced ALS.

  • Aggarwal, Swati, and Merit Cudkowicz. "ALS drug development: httpreflections from the past and a way forward." Neurotherapeutics 5.4 (2008): 516-527.
  • Bradley, Walter G., et al. "Current management of ALS Comparison of the ALS CARE Database and the AAN Practice Parameter." Neurology 57.3 (2001): 500-504.
  • Miller, Robert G., et al. "Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND)." Cochrane Database of Systematic Reviews 1 (2007).
  • A Forshew, Dallas, and Mark B Bromberg. "A survey of clinicians' practice in the symptomatic treatment of ALS." Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders 4.4 (2003): 258-263.
  • Photo courtesy of SteadyHealth.com

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