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Evidence-Based Respiratory Muscle Training in Amyotrophic Lateral Sclerosis

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1.  Patients with ALS frequently develop dystussia (an impaired, ineffective cough), which severely impacts airway clearance. Which of the following best explains the primary cause of dystussia?
  1. Isolated diaphragm weakness
  2. Airway bronchospasm and abdominal spasticity
  3. Weakness of the inspiratory, expiratory, and bulbar muscles
  4. Upper motor neuron degeneration of the intercostals
2.  Historically, rehabilitation professionals approached resistance exercise in the ALS population with caution primarily due to the theoretical fear of causing which of the following?
  1. Exercise-induced hypertension
  2. Overwork weakness
  3. Spontaneous pneumothorax
  4. Pulmonary embolism
3.  According to recent randomized controlled trials, which of the following respiratory measures demonstrates statistically significant improvement following Expiratory Muscle Strength Training (EMST) in patients with ALS?
  1. Maximum Expiratory Pressure (MEP)
  2. Forced Vital Capacity (FVC)
  3. Total ALSFRS-R score
  4. Lung diffusion capacity
4.  What is the validated "Rule of Fives" dosing protocol commonly used for Expiratory Muscle Strength Training (EMST) in the ALS population?
  1. 5 minutes of continuous training, 5 times a day
  2. 5 minutes of continuous training, 5 times a day
  3. 5 repetitions every 5 hours, 5 days per week
  4. 5 sets of 5 repetitions, 5 days per week
5.  In addition to preserving expiratory force, moderate-intensity Expiratory Muscle Strength Training (EMST) has been shown to improve which of the following?
  1. Maximum hyoid displacement
  2. Esophageal peristalsis
  3. Gastric emptying
  4. Saliva production