What is Amyotrophic Lateral Sclerosis (ALS)?

From the Guidelines

Amyotrophic lateral sclerosis (ALS) patients should prioritize maintaining a healthy weight and nutritional status, as malnutrition and significant weight loss are associated with increased risk of death and poorer survival outcomes, as evidenced by a study published in 2018 1. The management of ALS involves a multidisciplinary approach, including neurologists, respiratory therapists, physical therapists, speech therapists, and nutritionists.

• Key aspects of ALS management include:
  • Symptom management and supportive care
  • Medications such as riluzole (Rilutek) and edaravone (Radicava) to slow disease progression
  • Assistive devices like braces, walkers, and wheelchairs
  • Respiratory support as the disease progresses
• Nutritional status plays a crucial role in ALS prognosis, with studies indicating that:
  • Weight loss and malnutrition are associated with increased risk of death
  • Maintaining a healthy weight and body mass index (BMI) is essential for better survival outcomes
  • Bioelectrical impedance analysis (BIA) can be used to assess body composition and nutritional status in ALS patients
• Gastrostomy feeding may be recommended for patients with severe dysphagia, as it can provide long-term enteral nutrition therapy and improve nutritional outcomes, as suggested by a study published in 2018 1.
• The timing of gastrostomy insertion is crucial, and patients may benefit from early gastrostomy, i.e., before substantial weight loss occurs, as evidenced by a study published in 2018 1.
• Respiratory function is also an essential factor to consider in ALS management, and patients with moderate to severe respiratory impairment may require non-invasive ventilation support during gastrostomy placement, as noted in a study published in 2018 1.

From the FDA Drug Label

Riluzole tablets is indicated for the treatment of amyotrophic lateral sclerosis (ALS) The efficacy of riluzole tablets was demonstrated in two studies (Study 1 and 2) that evaluated riluzole tablets 50 mg twice daily in patients with amyotrophic lateral sclerosis (ALS) The answer to the question is:

• Riluzole is indicated for the treatment of amyotrophic lateral sclerosis (ALS).
• The efficacy of riluzole was demonstrated in two studies that evaluated riluzole 50 mg twice daily in patients with ALS.
• The studies showed that riluzole improved survival in patients with ALS, but did not show a benefit in measures of muscle strength and neurological function 2.

From the Research

Definition and Symptoms of Amyotrophic Lateral Sclerosis (ALS)

• Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper and lower motor neurons, leading to paralysis and eventually death 3.
• ALS is characterized by progressive motor symptoms, such as muscle weakness, muscle atrophy, and spasticity 4.
• Symptoms may include muscle cramps, spasticity, and pain, as well as bulbar symptoms such as dysphagia or dysarthria 5.

Current Treatments for ALS

• To date, riluzole and edaravone are the only two drugs approved by the Food and Drug Administration for the treatment of ALS in a few countries 3.
• Riluzole is a benzothiazole derivative that blocks glutamatergic neurotransmission in the CNS, which is thought to exert neuroprotective effects 6.
• Edaravone has demonstrated statistically significant slowing of ALS disease progression 7.
• Sodium phenylbutyrate and taurursodiol (PB-TURSO) was conditionally approved in Canada in 2022, having shown significant slowing of disease progression and prolonged survival 7.

Management and Care of ALS Patients

• Nurses play a critical role in the clinical management of ALS and may be involved in coordinating the activities of the team, facilitating treatment, and helping patients and caregivers in making informed treatment and end-of-life decisions 5.
• Care in specialized ALS centers is recommended to ensure optimal treatment regarding symptomatic medication, assistive devices, nutrition support, and ventilation therapy 4.
• Optimal care is achieved by interdisciplinary collaboration of general practitioners, specialized physicians, neurologists, and ALS experts being integrated in multiprofessional care networks 4.

Future Directions for ALS Treatment

• Identification of novel therapeutic strategies is urgently needed, including therapies modulating autophagy, stem cell therapies, gene therapies, and newly-developed biomaterials 3.
• Clinical trials utilizing stem cell transplantation and other biologics have emerged, providing renewed hope to finding a cure for ALS 7.
• The validity of the biomarker neurofilament light chain (NF-L) for estimating prognosis is currently being investigated 4.