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The spectrum of symptoms of <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> is continuous and varies from neonatal death to progressive symmetrical muscle weakness first appearing in adulthood. The disease is produced by degeneration of spinal motor neurons and can be described in three or more categories: <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> I with onset of symptoms before 6\u00a0months of age; <jats:styled-content style=\"fixed-case\">SMAII<\/jats:styled-content> with onset between 6 and 18\u00a0months and <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> III, which presents later in childhood. <jats:italic>Genetics<\/jats:italic>: The disease is in more than 95% of cases caused by a homozygous deletion in survival motor neuron gene 1 (<jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content>1). <jats:italic>Pathophysiology<\/jats:italic>: The loss of full\u2010length functioning <jats:styled-content style=\"fixed-case\">SMN<\/jats:styled-content> protein leads to a degeneration of anterior spinal motor neurons which causes muscle weakness. <jats:italic>Anesthetic risks: <jats:bold>Airway<\/jats:bold><\/jats:italic>: Tracheal intubation can be difficult. <jats:italic><jats:bold>Respiration<\/jats:bold><\/jats:italic>: Infants with <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> I almost always need postoperative respiratory support. Patients with <jats:styled-content style=\"fixed-case\">SMA II<\/jats:styled-content> sometimes need support, while <jats:styled-content style=\"fixed-case\">SMA III<\/jats:styled-content> patients seldom need support. <jats:italic><jats:bold>Circulation<\/jats:bold><\/jats:italic>: Circulatory problems during anesthesia are rare. <jats:italic>Anesthetic drugs: <jats:bold>Neuromuscular blockers<\/jats:bold><\/jats:italic>: Patients with <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content> may display increased sensitivity to and prolonged effect of nondepolarizing neuromuscular blockers. Intubation without muscle relaxation should be considered. Succinylcholine should be avoided. <jats:italic><jats:bold>Opioids<\/jats:bold><\/jats:italic>: These should be titrated carefully. <jats:italic><jats:bold>Anesthetic techniques<\/jats:bold><\/jats:italic>: All types of anesthetic technique have been used. Although none is absolutely contraindicated, none is perfect: anesthesia must be individualized. <jats:italic><jats:bold>Conclusion<\/jats:bold><\/jats:italic>: The perioperative risks can be considerable and are mainly related to the respiratory system, from respiratory failure to difficult\/impossible intubation.<\/jats:p>","DOI":"10.1111\/pan.12159","type":"journal-article","created":{"date-parts":[[2013,4,19]],"date-time":"2013-04-19T12:21:55Z","timestamp":1366374115000},"page":"804-816","source":"Crossref","is-referenced-by-count":52,"title":["Anesthesia and spinal muscle atrophy"],"prefix":"10.1111","volume":"23","author":[{"given":"Gunilla","family":"Islander","sequence":"first","affiliation":[{"name":"Department of Intensive and Perioperative Care Sk\u00e5ne University Hospital Lund 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