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Malignant Hyperthermia

Malignant Hyperpyrexia

Malignant hyperthermia is a rare genetic condition occuring during general anesthesia characterized by severe hyperthermia, acidosis, rigid muscles and rhabdomyolysis.


PBB Protein RYR1 image
PBB Protein RYR1 image
PBB Protein RYR1 image, Public Domain
MH drill cognitive aid mixing
MH drill cognitive aid mixing
MH drill cognitive aid mixing, CC BY-SA 4.0

Presentation
  • We also discuss the reasons for the shift in typical clinical presentation.[ncbi.nlm.nih.gov]
  • Signs of an MH reaction are non-specific and may be confused with the presentation of other problems such as sepsis and overheating of a patient. A high index of suspicion is needed to be aware of an early presentation of MH.[ncbi.nlm.nih.gov]
  • Hyperthermia was not a presenting sign of MH.[ncbi.nlm.nih.gov]
  • We present a case with unusual presentation. A boy aged 3 years and 9 months who was scheduled for Hotz's operation presented normally before the operation. Anesthesia was induced by atropine, thiopental and sevoflurane.[ncbi.nlm.nih.gov]
  • Present case describes rare delayed postoperative presentation of MH after off-pump coronary bypass surgery. Prompt recognition and immediate treatment with dantrolene can effectively treat the fatal syndrome.[ncbi.nlm.nih.gov]
Fever
  • In both cases, the discontinuation of rocuronium therapy was followed by the rapid diminution of fever over 12-36 hours.[ncbi.nlm.nih.gov]
  • This case underlines the importance of considering nonanesthetic MH susceptibility in the child who has a history of unspecified myopathy and who presents with fever and total body stiffness.[ncbi.nlm.nih.gov]
  • J Pediatr Orthop 1994; 14:799–801Angel, JD Blasier, RD Allison, R Lenhardt R, Negishi C, Sessler DI: Perioperative fever.[doi.org]
  • Many features of a typical malignant hyperthermia episode were present, including tachydysrhythmia, tachypnea, and fever in association with metabolic acidosis, hyperCKemia, myglobinemia, and rapid recovery without residual effects upon administration[ncbi.nlm.nih.gov]
  • A healthy 6-year-old boy developed lower extremity rigidity, trismus, and fever after playing in a splash pool. On arrival in the emergency department, he appeared to be seizing. An endotracheal tube was emergently placed using succinylcholine.[ncbi.nlm.nih.gov]
High Fever
  • He later developed high fever with core temperature reaching a peak of 42.2 C, rapid breathing, and died after unsuccessful attempts to stabilize him.[ncbi.nlm.nih.gov]
  • Muscle rigidity, breakdown of muscle fibers ( rhabdomyolysis ), a high fever, increased acid levels in the blood and other tissues (acidosis), and a rapid heart rate are some of the effects of this potentially life-threatening condition.[medicinenet.com]
  • This exposure can lead to muscle rigidity, very high fever, failure of other organs (kidney, lung), abnormal blood clotting and other life-threatening complications.[wakehealth.edu]
  • If given these drugs, people at risk for malignant hyperthermia may experience muscle rigidity, breakdown of muscle fibers (rhabdomyolysis), a high fever, increased acid levels in the blood and other tissues (acidosis), and a rapid heart rate.[web.archive.org]
Rigor
  • 1.75% halothane or an ambient temperature of 42 degrees C characterized by increased rectal temperature, respiratory rate, and inspiratory effort, with significant blood biochemical changes indicating metabolic acidosis, ending in death and hyperacute rigor[ncbi.nlm.nih.gov]
  • 1.25–1.75% halothane or an ambient temperature of 42 C characterized by increased rectal temperature, respiratory rate, and inspiratory effort, with significant blood biochemical changes indicating metabolic acidosis, ending in death and hyperacute rigor[doi.org]
  • ., 2000 ), the effect of caffeine on the Ca 2 and Mg 2 affinities of the A- and I-sites of the MHS and normal RyR1 have not been rigorously examined.[web.archive.org]
  • Despite possible links between exertional heat stroke and MH however, treatment with dantrolene has not been rigorously examined.[doi.org]
Tachypnea
  • Many features of a typical malignant hyperthermia episode were present, including tachydysrhythmia, tachypnea, and fever in association with metabolic acidosis, hyperCKemia, myglobinemia, and rapid recovery without residual effects upon administration[ncbi.nlm.nih.gov]
  • Clinical signs include unexplained elevation of end-tidal carbon dioxide, muscle rigidity, acidosis, tachycardia, tachypnea, hyperthermia, and evidence of rhabdomyolysis.[doi.org]
  • All demonstrated signs characteristic of acute MH, including generalized rigidity, hypercapnia and/or tachypnea, tachycardia, and hyperthermia. No subject demonstrated hyperthermia as the presenting sign.[ncbi.nlm.nih.gov]
  • In response to the acidosis, patients breathing spontaneously develop tachypnea that only partially compensates.[msdmanuals.com]
Tachycardia
  • Characteristic signs of malignant hyperthermia (MH) include unexplained tachycardia, increased end-tidal carbon dioxide (Etco₂) concentration, metabolic and respiratory acidosis, and an increase in body temperature above 38.8 C.[ncbi.nlm.nih.gov]
  • Pathologic response in MH include increase in oxygen consumption, increase in endtidal CO2, tachycardia, hyperthermia, hyperkalemia and muscle rigidity. Immediate recognition and treatment are crucial to avoid lethal outcome.[ncbi.nlm.nih.gov]
  • The following tachycardia, hypercapnia and hyperthermia led to the diagnosis of malignant hyperthermia. Symptoms were relieved dramatically after the discontinuation of sevoflurane.[ncbi.nlm.nih.gov]
  • […] anesthetics and/or the depolarizing muscle relaxant succinylcholine may induce this hypermetabolic muscular syndrome due to uncontrolled sarcoplasmic calcium release via functionally altered calcium release receptors, resulting in hypoxemia, hypercapnia, tachycardia[ncbi.nlm.nih.gov]
  • Twenty minutes after the start of CO(2) insufflation, increased end-tidal CO(2) and tachycardia were observed, which did not improve even with increased ventilation and release of insufflation.[ncbi.nlm.nih.gov]
Hypertension
  • After a preprocedural pain block for elective right shoulder arthroscopy, a 53-year-old hypertensive white man developed chest pain. In the operating room, he had increased blood pressure.[ncbi.nlm.nih.gov]
  • Disease (1) increased ETCO2 (2) tachycardia (3) tachypnoea (4) masseter spasm (if develops this convert to a MH safe anaesthetic) (5) muscle rigidity (6) temp increase (late) – 1 C\15min intra-op & 4\24 post op tachyarrhythmia difficulty ventilation hypertension[lifeinthefastlane.com]
  • In patient 10, the AMRA was accompanied by a narrative statement: “Pt developed muscular rigidity, tachypnea, tachycardia, and hypertension just as he was extubated.[doi.org]
  • A fast rate of breathing (in a spontaneously breathing patient), cyanosis, hypertension, abnormal heart rhythms, and high blood potassium may also be seen.[en.wikipedia.org]
  • He had suffered from hypertension and gastroesophageal reflux disease, but he had no history of neuromuscular disease and surgical operations. Also, he had no significant family history. He had been a heavy drinker and a smoker.[omicsonline.org]
Hypotension
  • ., central core disease and multiminicore disease ) also have MH susceptibility. 0003236 Hypotension Low blood pressure 0002615 Mixed respiratory and metabolic acidosis 0005967 Rhabdomyolysis Breakdown of skeletal muscle 0003201 Rigidity Muscle rigidity[rarediseases.info.nih.gov]
  • After 10 minutes of halothane anesthesia, the patient became tachycardic, hypotensive, hypoxemic, and hyperthermic, and the halothane was discontinued. He was packed in ice and subsequently recovered uneventfully.[newswise.com]
  • Later signs include complex arrhythmias, cyanosis, hypoxia, hypotension, electrolyte abnormalities, rhabdomyolysis and severe hyperthermia. Hyperthermia is a late sign. Rhabdomyolysis is a sign of the severity of the condition.[patient.info]
  • Signs and Symptoms Approximate number of patients (when available) Abnormality of the coagulation cascade - Autosomal dominant inheritance - Elevated serum creatine phosphokinase - Fever - Hyperkalemia - Hyperphosphatemia - Hypotension - Malignant hyperthermia[web.archive.org]
  • Ca 2 blockers viz verapamil should not be used along with dantrolene, since hyperkalemia and profound hypotension may occur with such a drug combination [ 16, 158 ]. The steps in the treatment of acute MH are shown in Table 2.[doi.org]
Erythema
  • Less than twenty minutes after initiation of volatile anesthesia, profound skin erythema and hyperthermia were installed, with the body temperature reaching 41 Celsius degrees.[pubs.sciepub.com]
  • […] reconstitution, dantrolene is stable at room temperature for 6 hours when protected from light. 18 Adverse effects associated with dantrolene include loss of grip strength, muscle weakness, drowsiness, dizziness, and injection site reactions, including pain, erythema[healio.com]
Myalgia
  • We present four recently referred cases to illustrate this point: one late-onset case, two patients with slowly progressive hypercapnia as the sole sign and a fourth patient with postoperative myalgias and elevated creatine kinase.[ncbi.nlm.nih.gov]
  • Several uncommon muscle disorders have a high association with MH, and postoperative myalgia unrelated to suxamethonium can be a sign which is associated with MH.[ncbi.nlm.nih.gov]
  • Most complained about myalgia or cramps, but all had a normal neurological examination. They persistently presented with hyperCKemia from three months to ten years, with a mean (SD) CK value of 788 (507) IU·L -1 ranging from 210-1,667 IU·L -1 .[ncbi.nlm.nih.gov]
  • Before an anaesthetic ask about unclear complications from previous anaesthesia, muscle disorders in the family, myalgia, muscle cramps and dark urine. If there is suspicion, check CK. If in doubt, use a trigger-free anaesthetic.[patient.info]
  • Jungbluth, Mutations in RYR1 are a common cause of exertional myalgia and rhabdomyolysis, Neuromuscular Disorders, 10.1016/j.nmd.2013.03.008, 23, 7, (540-548), (2013). Allison Billings, Skeletal Muscle, Equine Clinical Pathology, (153-179), (2013).[doi.org]
Flexion Contracture
  • Clinical features of the Freeman-Sheldon syndrome include flexion contractures and characteristic facial features, including microstomia and a whistling shape to the lips.[ncbi.nlm.nih.gov]
Confusion
  • Signs of an MH reaction are non-specific and may be confused with the presentation of other problems such as sepsis and overheating of a patient. A high index of suspicion is needed to be aware of an early presentation of MH.[ncbi.nlm.nih.gov]
  • Flushed skin Sweating An abnormally rapid or irregular heartbeat Rapid breathing or uncomfortable breathing Brown or cola-colored urine Very low blood pressure (shock) Confusion Muscle weakness or swelling after the event Many people who carry a gene[drugs.com]
  • Some degree of increased muscular tone is usually present, which may be confused with the lead-pipe rigidity seen with NMS.[calpoison.org]
  • Hyperkalemic cardiac arrest in patients with muscular dystrophy A syndrome often confused with MH is sudden hyperkalemic cardiac arrest during or shortly after anesthesia in young males.[doi.org]
  • A syndrome often confused with MH is sudden hyperkalemic cardiac arrest during or shortly after anesthesia in young males.[doi.org]
Altered Mental Status
  • Serotonin toxicity is characterized by a triad of altered mental status, autonomic effects, and neuromuscular excitation.[calpoison.org]
  • Neuroleptic malignant syndrome is characterized by muscle rigidity, hyperthermia, hyperkalemia, acidosis, autonomic instability, and altered mental status most often occurring after the use of neuroleptic agents such as haloperidol, but it has also been[healio.com]
Gowers Sign
  • On neurological examination, he had mild proximal muscle weakness and atrophy, decreased deep tendon reflexes and Gowers' sign, but his intelligence was normal. The electromyogram showed myopathic pattern.[ncbi.nlm.nih.gov]

Workup
  • Neurology workup revealed the presence of a known causative ryanodine receptor (RYR1) mutation for MH, c.7522C T; p.R2508C. Furthermore, the neurology workup diagnosed the child with King-Denborough syndrome (KDS).[ncbi.nlm.nih.gov]
  • She was administered 2 liters of normal saline and a workup for possible infection ensued. Her sister later arrived and stated that her paroxetine dose was doubled from 20 to 40 mg last week and that she may have taken a few extra pills.[calpoison.org]
Pneumoperitoneum
  • We assumed that this was due to a prolonged carbon dioxide pneumoperitoneum until he developed ST segment depression on his electrocardiogram and a rapid rise in temperature.[ncbi.nlm.nih.gov]
Hypercapnia
  • The following tachycardia, hypercapnia and hyperthermia led to the diagnosis of malignant hyperthermia. Symptoms were relieved dramatically after the discontinuation of sevoflurane.[ncbi.nlm.nih.gov]
  • Volatile anesthetics and/or the depolarizing muscle relaxant succinylcholine may induce this hypermetabolic muscular syndrome due to uncontrolled sarcoplasmic calcium release via functionally altered calcium release receptors, resulting in hypoxemia, hypercapnia[ncbi.nlm.nih.gov]
  • We present four recently referred cases to illustrate this point: one late-onset case, two patients with slowly progressive hypercapnia as the sole sign and a fourth patient with postoperative myalgias and elevated creatine kinase.[ncbi.nlm.nih.gov]
  • Because it is necessary to initiate treatment in the early stage of MH, as soon as possible, although MH prevalence is low in the elderly, it is important to suspect MH when hypercapnia and/or hyperthermia are seen.[ncbi.nlm.nih.gov]
  • All demonstrated signs characteristic of acute MH, including generalized rigidity, hypercapnia and/or tachypnea, tachycardia, and hyperthermia. No subject demonstrated hyperthermia as the presenting sign.[ncbi.nlm.nih.gov]
Amino Acids Increased
  • Ca(2 )-release experiments showed that exchanges of these highly conserved amino acids increased the sensitivities for the substances tested (except 4-CmC with p.R2458H and p.R3348C) when compared with the MH-negative control group.[ncbi.nlm.nih.gov]

Treatment
  • Dantrolene is the drug of choice for treatment. Recurrence may occur in up to 20% of cases after the initial event treatment.[ncbi.nlm.nih.gov]
  • If these complications occur even with initial treatment with dantrolene, our experiences may be useful adjunctive treatments to consider.[ncbi.nlm.nih.gov]
  • FDA-Approved Treatments The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.[rarediseases.info.nih.gov]
  • This case suggests that low dose dantrolene by mouth could be considered for the treatment of chronic muscle pain in individuals with MH susceptibility.[ncbi.nlm.nih.gov]

Prognosis
  • […] inotropes as needed HCO3 2-4mEq/kg Dantrolene 2.5mg/kg every 5min (total dose 10mg/kg/day – continuous infusion or treat recurrence (25%)) cardiac arrhythmias - beta blockers & lignocaine. high K - glucose-insulin & frusemide watch for DICCK Q6hrly PROGNOSIS[lifeinthefastlane.com]
  • […] surgery, tell your surgeon, primary care doctor and anesthesiologist if you: Have a family history of malignant hyperthermia Have had one or more episodes of heat stroke or exercise-induced hyperthermia Have muscle symptoms or a known muscle disease Prognosis[drugs.com]
  • […] in 291 MH episodes recorded in the North American Malignant Hyperthermia Registry database between 1987 and 2006, there were eight cardiac arrests and four deaths, and the median age of patients experiencing cardiac arrest or death was 20 years. [7] Prognosis[emedicine.medscape.com]
  • The use of dantrolene has considerably influenced the prognosis of such a disorder, but other appropriate symptomatic measures have to be honored, with discontinuation of offending drug, treatment of acidosis, electrolyte disbalance and heart rhythm disorders[pubs.sciepub.com]
  • Prognosis is poor if this condition is not aggressively treated. In the 1970s, mortality was greater than 80%; with the current management, however, mortality is now less than 5%.[en.wikipedia.org]

Etiology
  • DNA samples were collected from 885 patients in 4 groups: severe statin myopathy (n 197), mild statin myopathy (n 163), statin-tolerant controls (n 133), and non-drug-induced myopathies of unknown etiology characterized by exercise-induced muscle pain[ncbi.nlm.nih.gov]
  • He developed a postoperative left brachial vein thrombus of unclear etiology, possibly related to dantrolene administration or his underlying susceptibility to MH.[ncbi.nlm.nih.gov]
  • As a result of extensive research on the mechanisms of excitation-contraction coupling and recent functional characterization of several disease-causing mutations in heterologous expression systems, much is known today about the molecular etiology of[ncbi.nlm.nih.gov]
  • […] high creatine kinase (CK), 9 with exercise-induced rhabdomyolysis and/or exercise intolerance, 2 with high CK and exercise-induced rhabdomyolysis and/or exercise intolerance, 15 with postviral chronic fatigue, and 14 with muscle weakness of unknown etiology[ncbi.nlm.nih.gov]
  • This prospective study included horses with suspected MH, undetermined etiology of sudden death, death within hours of onset of rhabdomyolysis, muscle rigidity, stiffness, intermittent sweating, and persistent increases in serum muscle enzyme activities[ncbi.nlm.nih.gov]

Epidemiology
  • Epidemiologic data on the geographic variation in MH prevalence is scant. The objective of this study is to examine the prevalence of recorded MH diagnosis in patients discharged from hospitals in four states in the United States.[ncbi.nlm.nih.gov]
  • Epidemiologic research on MH is largely limited to inpatients. In this study, we examined the prevalence of recorded MH diagnosis in patients discharged from ambulatory surgery centers (ASCs).[ncbi.nlm.nih.gov]
  • This systematic review and meta-analysis will be performed according to the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guidelines and reported according to the Preferred Reporting Items for Systematic reviews and Meta-Analysis (PRISMA[ncbi.nlm.nih.gov]
  • METHODS: Patients in an MH centre were followed from 1997-2012, and their epidemiologic, clinical, and laboratory data were analyzed, including IVCT, muscle histochemical analysis, and next-generation sequencing molecular analysis.[ncbi.nlm.nih.gov]
  • Specialists in the field discussed the most up-to-date findings from the point of view of clinical classification, history, and incidence based on the evidence of epidemiology, diagnostic muscle testing, genetics, and biochemistry.[books.google.com]
Sex distribution
Age distribution

Pathophysiology
  • Dantrolene sodium is a specific antagonist of the pathophysiologic changes of MH and should be available wherever general anesthesia is administered.[ncbi.nlm.nih.gov]
  • Increased understanding of the clinical manifestation and pathophysiology of the syndrome, has lead to the mortality decreasing from 80 % thirty years ago to 5 % in 2006.[ncbi.nlm.nih.gov]
  • Pathophysiology, genetic basis of and testing for MH-susceptibility as well as the role of the critical care nurse in the management of this potentially fatal disorder will be discussed.[ncbi.nlm.nih.gov]
  • The pathophysiologic underpinnings of idiopathic hypersomnia and its interactions with anesthetic medications remain poorly understood. There is a scarcity of literature describing this patient population in the surgical setting.[ncbi.nlm.nih.gov]
  • The objective of this exploratory study was to elucidate the pathophysiology of exercise intolerance in patients tested positive for MH with the caffeine-halothane contracture test.[ncbi.nlm.nih.gov]

Prevention
  • This prevented us from making a definitive diagnosis in our patient.[ncbi.nlm.nih.gov]
  • In the EXIT (ex utero intrapartum treatment) procedure, after uterine incision, uterine relaxation is maintained to prevent placental separation and the fetus is supported via the placenta until the airway is successfully established.[ncbi.nlm.nih.gov]
  • Potentially fatal operating room events have become largely preventable with advances in anesthesia and surgical technique. Nonetheless, some lethal emergencies remain unpredictable and can occur whenever general anesthesia is given.[ncbi.nlm.nih.gov]
  • Our primary objective was to detect malignant hyperthermia (MH)-susceptible persons and thereby prevent MH episodes. We identified variants in the ryanodine receptor isoform 1 using molecular pedigree analysis.[ncbi.nlm.nih.gov]
  • CONCLUSION: When investigating patients with idiopathic hyperCKemia, susceptibility to MH should be taken into account, and guidance should be offered to prevent anesthetic complications in the family.[ncbi.nlm.nih.gov]

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Last updated: 2019-06-28 10:43