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The Sarcomere and Skeletal Muscle Disease pp 92–98Cite as

Acute Quadriplegic Myopathy: An Acquired “Myosinopathy”

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 642))

Abstract

Acquired neuromuscular disorders have been shown to be very common in critically ill patients receiving prolonged mechanical ventilation in the intensive care unit (ICU). Acute Quadriplegic Myopathy (AQM) is a specific acquired myopathy in ICU patients. Patients with AQM are characterized by severe muscle weakness and atrophy of spinal nerve innervated limb and trunk muscles, while cranial nerve innervated craniofacial muscles, sensory and cognitive functions are spared or less affected. The muscle weakness is associated with altered muscle membrane properties and a preferential loss of the motor protein myosin and myosin-associated thick filament proteins. Prolonged mechanical ventilation, muscle unloading, postsynaptic block of neuromuscular transmission, sepsis and systemic corticosteroid hormone treatment have been suggested as important triggering factors in AQM. However, the exact mechanisms underlying the loss of thick filament proteins are not known, though enhanced myofibrillar protein degradation in combination with a downregulation of protein synthesis at the transcriptional level play important roles.

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Author information

Authors and Affiliations

  1. Department of Clinical Neurophysiology, Uppsala University, SE-751 85, Uppsala, Sweden

    Lars Larsson

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  1. Lars Larsson
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Editors and Affiliations

  1. Centre for Medical Research, University of Western Australia, Western Australian Institute for Medical Research, Nedlands, Western Australia, Australia

    Nigel G. Laing PhD

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© 2008 Landes Bioscience and Springer Science+Business Media

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Larsson, L. (2008). Acute Quadriplegic Myopathy: An Acquired “Myosinopathy”. In: Laing, N.G. (eds) The Sarcomere and Skeletal Muscle Disease. Advances in Experimental Medicine and Biology, vol 642. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84847-1_8

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