Critical Care Medicine-Neurologic Disorders>>>>>Brain Death and Degenerative Diseases
Question 4#

A 58-year-old woman with amyotrophic lateral sclerosis (ALS) presents to the emergency department complaining of increased weakness and difficulty with feeding herself. Her daughter inquires if there are any interventions which have been proven to increase life expectancy in ALS patients.

Which of these is the MOST appropriate answer?

a. Amantadine
b. Noninvasive ventilation
c. Colostomy
d. Prophylactic antibiotics
e. Indwelling nasogastric tube

Correct Answer is B

Comment:

Correct Answer: B

Patients with ALS have progressive degeneration of both upper and lower motor neurons causing weakness, difficulty swallowing, and respiratory insufficiency. Although many different approaches have been tried to manage these patients, there are only a few which have shown benefits in increasing life expectancy. Noninvasive ventilation, specifically with optimized bilevel positive airway pressure (BiPAP) protocols, helps to avoid hypercarbia, secondary to diaphragm and respiratory muscle weakness. Other interventions include riluzole, a medication that is thought to work on the neuronal level and shown to increase survival by few months and delay the onset of tracheostomy and ventilator dependence in selected ALS patients. Amantadine is used to promote alertness in patients with neurological injury; however, it has not been shown to be of benefit in ALS. Although patients with ALS often get gastrostomy tubes for safer feeding, colostomy and indwelling nasogastric tubes have not been shown to increase life expectancy. Prophylactic antibiotics are not recommended for ALS as there is no immune suppression.

References:

  1. Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. 2019. Available at https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet. Retrieved January 24, 2019.
  2. Karam CY, Paganoni S, Joyce N, et al. Palliative care issues in amyotrophic lateral sclerosis: an evidenced-based review. Am J Hosp Palliat Care. 2016;33(1):84-92.
  3. Hardiman O, Al-Chalabi A, Chio A, et al. Amyotrophic lateral sclerosis. Nat Rev Dis Primers. 2017;3:17071.