Treating amyotrophic lateral sclerosis (ALS)

Tips and tricks for managing amyotrophic lateral sclerosis (ALS) in your patients. Click here to read more!
Last update7th Jan 2021

There is no definitive treatment for amyotrophic lateral sclerosis (ALS). Most treatment regimens are supportive and symptom-relief oriented with a focus on airway management and feeding.

How to treat ALS with medications

Medications to prevent the decline

While most treatments for ALS only relieve symptoms, riluzole is a bit different. Riluzole is recommended for ALS and can somewhat slow disease progression, potentially extending life by a few months to a year. Riluzole acts to diminish glutamate release by blocking specific sodium ion channels. Glutamate is a neurotransmitter that is cytotoxic to nervous system cells in increased concentrations.

Figure 1. Riluzole is a medication that can somewhat slow the progression of amyotrophic lateral sclerosis (ALS) by diminishing glutamate release.

Other experimental medications are on the horizon. Several researchers seek to alter glutamate availability and toxicity through various mechanisms.

Edaravone has also been invoked in the care of patients with ALS and should be considered. This medication is a free radical scavenger and is thought to slow functional decline in patients with ALS.

Medications for stiffness and cramping

Stiffness and cramping are common complaints from patients with ALS. These symptoms can be treated with various muscle relaxants such as baclofen and tizanidine.

Figure 2. Muscle relaxants such as baclofen and tizanidine may help relieve amyotrophic lateral sclerosis (ALS)-associated stiffness and cramping.

Medications for depression

Depression is common in patients with ALS, and selective serotonin reuptake inhibitors (SSRIs) should be strongly considered. The disease carries a level of certitude and hopelessness both for the patients and their loved ones.

Figure 3. Depression is common in patients with amyotrophic lateral sclerosis (ALS), and antidepressants should be considered.

What new therapies for ALS are being developed?

Other therapies on the horizon involve the removal or suppression of mutated superoxide dismutase 1 (SOD1) proteins in motor neurons. Research has also been conducted on the replacement of lost motor neurons with stem cells.

Taking another approach, brain-digital interfaces may promise some hope by allowing patients with ALS to control robotic exoskeletons with thought. For the time being, however, most interventions for ALS are supportive.

What other therapies may benefit patients with ALS?

If available, ALS is ideally treated in specialized multi-disciplinary clinics that offer tracheostomy, mechanical ventilation, and feeding tube support. Aggressive physical, speech, and occupational therapy should be employed to optimize the patient’s mobility and activities of daily living.

Respiratory complications are the main cause of death for these patients. Both respiratory drive and airway clearing are compromised by ALS. Consider noninvasive ventilatory support, such as a continuous positive airway pressure (CPAP) machine, particularly at night for patients beginning to exhibit respiratory failure.

Figure 4. The use of continuous positive airway pressure (CPAP) machines, particularly at night, may help patients with amyotrophic lateral sclerosis (ALS) who are starting to show signs of respiratory failure.

Tracheostomy and mechanical ventilation may have to be considered with the progression of ALS. Some authors recommend a relatively early transition to tracheostomy and mechanical ventilation. However, many patients elect not to resort to this level of support and dependency.

Figure 5. Tracheostomy and mechanical ventilation should be considered as amyotrophic lateral sclerosis (ALS) progresses.

Lower cranial nerve dysfunction leads to increasing difficulties with swallowing. Therefore, aspiration becomes a persistent challenge and many patients tend to become poorly nourished. Good nutrition is therefore a mainstay of supportive care.

Consult nutritional services early and consider placing a feeding tube to maintain good nutrition in your patient. A feeding tube should be considered relatively early in the disease progression. Percutaneous endoscopic gastronomy (PEG) tubes are preferred since nasogastric tubes increase the chances of aspiration in patients with ALS.

Figure 6. A percutaneous endoscopic gastronomy (PEG) feeding tube should be considered early in the progression of amyotrophic lateral sclerosis (ALS) to avoid aspiration and help maintain good nutrition.

Psychosocial and behavioral difficulties are also common with ALS and may include inappropriate behaviors, impaired decision making, involuntary laughing, and crying. Reach out to psychosocial support systems early to initiate psychiatric and psychological support.

Other management challenges must be considered as the disease progresses and additional symptoms arise:

  • Hypersalivation and drooling
  • Thickened secretions
  • Limb stiffness
  • Anxiety
  • Pain
  • Skin breakdown
  • Anorexia
  • Sleep difficulties

Nursing care facility placement may become necessary. Families caring for a patient at home will need extensive support. Social services should become involved early in the course of the disease.

Figure 7. Common disease management considerations as amyotrophic lateral sclerosis (ALS) progresses.

What is the prognosis of patients with ALS?

Sadly, most patients with ALS die within 3–5 years. Therefore, it is incumbent on the provider to encourage contact with end-of-life services and experts relatively early in the course of the disease.

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Recommended reading

  • Armon, C. 2020. Amyotrophic lateral sclerosis. Medscapehttps://emedicine.medscape.com/
  • Devlin, VJ. 2012. Spine Secrets Plus. 2nd edition. Missouri: Mosby.
  • Louis, ED, Mayer, SA, and Rowland, LP. 2015. Merritt’s Neurology. 13th edition. Philadelphia: Wolters Kluwer.
  • Mitchell, JD and Borasio, GD. 2007. Amyotrophic lateral sclerosis. Lancet369: 2031–2041. PMID: 17574095
  • Rowland, LP and Shneider, NA. Amyotrophic lateral sclerosis. N Engl J Med344: 1688–1700. PMID: 11386269

About the author

Gary R. Simonds, MD MHCDS FAANS
Professor at Virginia Tech School of Neuroscience / Virginia Tech Carilion School of Medicine and Program Director for the Division of Neurosurgery at Virginia Tech Carilion Clinic.
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