ABSTRACT
A wide variety of progressive neuromuscular disorders can result in dysfunction of the ventilatory muscles that in turn can lead to respiratory failure, pneumonia and even death. Breathing disorders are recognized as the leading cause of mortality in neuromuscular disease.1 Neuromuscular disease can affect almost any part of the neurological system leading to changes in the respiratory system. In this chapter we will focus on a number of different conditions that affect the respiratory system in a progressive manner and review the pathophysiology and data pertinent to respiratory function where available.
