26 - Neuromuscular disorders

Summary

This chapter discusses the general medical care of patients with neuromuscular disease, and gives an account of the more common conditions seen in the intensive care setting. It reviews the general principles of the complex and challenging management of patients with neuromuscular disease. In patients with respiratory failure due to acute neuromuscular disease, ventilation is usually initiated with synchronized intermittent mandatory ventilation until recovery of respiratory muscle function occurs. Respiratory failure due to neuromuscular disease may be caused by diseases affecting the anterior horn cells, peripheral nerves, neuromuscular junction or the respiratory muscles themselves. The neuromuscular junction (NMJ) forms the link between myelinated motor nerves and skeletal muscle. The NMJ consists of pre-synaptic, synaptic and post-synaptic components. A number of neurological diseases affect the pre-synaptic and post-synaptic components of the NMJ. Primary muscle diseases include the muscular dystrophies, congenital muscle disease, metabolic muscle disease and the periodic paralyses.