However, there is a wide-ranging estimate of MH incidence. Many physicians may believe that MH is so rare that most professionals will probably never face a case. It was first described in 1962 as cases occurring in a single family by Denborough et al., when the authors reported a 21-year-old student who, when admitted to the Royal Melbourne Hospital in Australia for a leg fracture, was more concerned about receiving GA than about his fracture because 10 of his family members had died during or after GA, usually administered for minor procedures. Malignant hyperthermia (MH) is a rare but life-threatening heterogeneous pharmacogenetic disorder due to the dysfunction of skeletal muscle calcium channels, triggered by exposure to volatile halogenated anesthetics (desflurane, isoflurane, sevoflurane, halothane) and the depolarizing muscle relaxant succinylcholine, which results in abnormal contraction and a hypermetabolic state at the level of the myocyte that rapidly leads to ATP depletion, rhabdomyolysis, and heat production. Since the beginning of the 20th century, cases of increased body temperature related to general anesthesia (GA) have been observed, and several reports referred to complications and anesthesia-related deaths, often described as “ether convulsions”. This review shall consider all these aspects and summarize the most recent updates. Based on epidemiological data, many national scientific societies have produced consistent guidelines, but many misconceptions are common among physicians and healthcare workers. However, a personalized approach and an effective prevention policy focused on clearly recognizing the high-risk population, defining perioperative trigger-free hospitalization, and rapid activation of supportive therapy should be improved. In the last few decades, synergistic efforts among leading anesthesiology, pediatrics, and neurology associations have produced new evidence concerning the diagnostic pathway, avoiding unnecessary testing and limiting false diagnoses. Although this occurrence could affect virtually any patient during the perioperative time, the pediatric population is particularly vulnerable, and it has a five-fold higher incidence in children compared to adults. Malignant hyperthermia is a rare but life-threatening pharmacogenetic disorder triggered by exposure to specific anesthetic agents.
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