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26 - Neuromuscular disorders

from Section 4 - Neurointensive care

Published online by Cambridge University Press:  05 December 2011

Basil F. Matta
Affiliation:
Addenbrooke's Hospital, Cambridge
David K. Menon
Affiliation:
Addenbrooke's Hospital, Cambridge
Martin Smith
Affiliation:
Department of Neuroanaesthesia and Neurocritical Care, the National Hospital for Neurology and Neurosurgery, University College London Hospitals
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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.
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Publisher: Cambridge University Press
Print publication year: 2011

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