ICU Management of Respiratory Disorders in Neuromuscular Diseases

Abstract

Neuromuscular diseases (NMDs) are acquired or inherited conditions that affect parts of the neuromuscular system such as the muscles, the peripheral motor nerves (in arms, legs, neck and face), the neuromuscular junction or the motor neurons in the spinal cord. Most NMDs are characterised by progressive muscular impairment leading to loss of ambulation, being wheelchair-bound, swallowing difficulties, respiratory muscle weakness, and eventually death from respiratory failure. Many NMDs can progress to the point where they compromise respiratory function. Although the diseases have variable causes and clinical courses, in many cases it is a pulmonary complication that is the cause of death or other serious adverse event. Pathophysiology of respiratory complications in neuromuscular diseases depends on many factors which include direct consequences of respirator