Chapter 4 - Neuromuscular disorders

Summary

Neuromuscular diseases include disorders of anterior horn cells, anterior and posterior roots, plexus, peripheral nerves, neuromuscular junctions, and muscles. Detailed history and physical examination most often provide reliable information to localize the neuromuscular disorder. Motor neuron diseases have in common the dysfunction of the superior or inferior motor neuron. Immune myasthenia gravis is caused by autoantibodies interfering with the normal neuromuscular transmission. Most myopathies are slowly progressing diseases involving predominantly proximal muscles. Corticosteroids are the first-line immunosuppressive treatment in most chronic immune-mediated neuromuscular diseases. Neurological disorders may cause respiratory failure by impairing pulmonary ventilation. For normal ventilation to occur, multiple central and peripheral nervous system structures need to be intact. Respiratory management of neuromuscular respiratory failure requires differentiating between slowly developing conditions in which respiratory failure occurs as an exacerbation of a chronic condition, and rapidly progressive diseases.