Neuromuscular disorders

Abstract

Neuromuscular disorders are a heterogeneous group of neurologic diseases that affect a number of neural structures including the motor nerves, neuromuscular junctions or the muscles themselves. Although many of the diseases are rare, the total number of individuals who present to a respiratory care provider is significant. Many neuromuscular diseases lead to progressive muscle weakness that compromises the function of the respiratory system including upper airway tone, cough and chest wall support. Respiratory failure is the most common cause of mortality in patients with neuromuscular diseases. These patients still die prematurely or are hospitalized and undergo tracheostomy because of failure to assist respiratory muscles to prevent respiratory failure. Approaches to care include regular and careful clinical follow-up of symptoms of sleep-disordered breathing, daytime hypoventilation, as well as cough and swallowing effectiveness. Chronic invasive or non-invasive ventilation and airway clearance techniques are the cornerstone of the treatment of patients with neuromuscular disorders.