RT Book, Section A1 Bauman, Loren A. A1 Tobin, Joseph R. A2 Longnecker, David E. A2 Brown, David L. A2 Newman, Mark F. A2 Zapol, Warren M. SR Print(0) ID 56623240 T1 Chapter 12. Evaluation of the Patient with Neuromuscular, Skeletal, or Motion Disorders T2 Anesthesiology, 2e YR 2012 FD 2012 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-178513-6 LK accessanesthesiology.mhmedical.com/content.aspx?aid=56623240 RD 2024/04/19 AB The major cause of death in patients with neuromuscular disorders is respiratory insufficiency. Respiratory involvement varies considerably among the various neuromuscular disorders, and the extent of general muscle weakness does not necessarily correlate with the severity of respiratory muscle involvement.Cardiovascular involvement may manifest as myocardial failure in patients with myopathic disorders and as autonomic dysfunction in patients with neuropathic disorders. Clinical signs of autonomic dysfunction include orthostatic hypotension, resting tachycardia, paralytic ileus, anhidrosis, and constricted pupils. The presence of these clinical signs may indicate profound hemodynamic instability that may manifest during the perioperative period, requiring invasive monitoring to manage intravascular volume status and myocardial contractility.In patients with neuromuscular disorders, the severity of skeletal muscle involvement does not necessarily correlate with the severity of cardiac involvement.Children with asymptomatic, undiagnosed muscular dystrophy are at significant risk for serious, life-threatening anesthetic complications. Specifically, these patients may develop intractable hyperkalemic cardiac arrest after receiving succinylcholine intravenously.Spinal anesthesia has been associated with exacerbation of multiple sclerosis, although the mechanism is unclear. Speculation is that demyelinated areas of the spinal cord are more sensitive to the effects of the local anesthetic, resulting in a relative neurotoxicity.Numerous anesthetics may exacerbate an acute attack of porphyria and should be avoided. Propofol, ketamine, local anesthetics, muscle relaxants, nitrous oxide, isoflurane, and opioids are considered safe.Myasthenic crisis is a rapid deterioration of neuromuscular and respiratory function that may occur at any time perioperatively as a result of infection, stress, or an overdose with anticholinesterase drugs (cholinergic crisis).Patients with myasthenia gravis and myasthenic syndrome are exquisitely sensitive to the effects of nondepolarizing muscle relaxants.Succinylcholine should be avoided in patients with muscular dystrophies; it may further damage the already abnormal muscle membrane and cause the release of intracellular contents.Succinylcholine produces an exaggerated contracture, and its use should be avoided in patients with myotonias. The myotonic response produced by succinylcholine may be so severe that ventilation and tracheal intubation are difficult and may be impossible.Rheumatoid arthritis, osteoarthritis, and ankylosing spondylitis all are associated with possible cervical spine disease and may make airway management difficult.Some musculoskeletal disorders are associated with an abnormal heat dissipation mechanism; central temperature monitoring is essential in these patients.Many etiologies of high creatine kinase concentrations or rhabdomyolysis may be associated with malignant hyperthermia susceptibility.