Malignant hyperthermia (MH) may lead to metabolic crisis of skeletal muscle in susceptible individuals following exposure to triggering agents such as volatile anesthetics or depolarizing muscle relaxants. MH is a rare and a potentially lethal disease, which can lead to cardiac arrest. We report a case of severe MH, in which the rapidly evolving signs of hypermetabolism eventually resulted in cardiac arrest. Despite conventional treatments following cardiopulmonary resuscitation, the patient's vital signs did not improve. Therefore, we applied extracorporeal membrane oxygenation for providing hemodynamic support.
Anesthetics ; Cardiopulmonary Resuscitation* ; Dantrolene ; Extracorporeal Membrane Oxygenation* ; Heart Arrest* ; Hemodynamics ; Humans ; Malignant Hyperthermia* ; Muscle, Skeletal ; Neuromuscular Depolarizing Agents ; Vital Signs

Paramyotonia congenita is a rare hereditary skeletal muscle disease characterized by exercise- or cold-induced myotonia. Anesthesiologists should make any efforts to prevent perioperative myotonic attack and muscle weakness in patients with this kind of disorder. Specifically, the administration of depolarizing muscle relaxants should be avoided and serum potassium level as well as body temperature should be carefully managed. The present report describes our experiences with successful epidural anesthesia in a patient with paramyotonia congenita who underwent a lumbar discectomy.
Analgesia, Epidural ; Anesthesia, Epidural* ; Body Temperature ; Diskectomy* ; Humans ; Muscle Weakness ; Muscle, Skeletal ; Myotonia ; Myotonic Disorders* ; Neuromuscular Depolarizing Agents ; Potassium

Sugammadex is belonging to a new class of drugs: the selective relaxant binding agents. Sugammadex can reverse residual paralysis by encapsulating free circulating non depolarizing muscle relaxants. The mains advantages of sugammadex when compared with conventional anticholinesterase agents are a much faster recovery time and the unique ability, for the first time, to reverse rapidly and efficiently deep levels of neuromuscular blockade. However it only works for reversal of rocuronium or vecuronium-induced neuromuscular blockade. When administered 3 min after rocuronium the use of a large dose (16 mg/kg) can even reverse rocuronium significantly faster than the spontaneous recovery after succinylcholine.
Cholinesterase Inhibitors ; Cyclodextrins ; Neostigmine ; Neuromuscular Blockade ; Neuromuscular Depolarizing Agents ; Paralysis ; Succinylcholine

Malignant hyperthermia (MH) is an inherited disorder of skeletal muscle manifested as a life threatening hypermetabolic crisis in susceptible individuals following exposure to commonly used inhaled anesthetics and depolarizing muscle relaxants. We experienced a suspicious case of MH in 34-year-old male during transfemoral cerebral angiography embolization under general anesthesia with desflurane. The episode emerged 15 minutes after induction of general anesthesia using propofol, rocuronium, remifentanil, desflurane. Desflurane is a recently developed inhaled anesthetics and there has been no case report of MH related with it in Korea. When we suspected episode, vigorous treatment was carried out, symptoms were resolved without dantrolene administration.
Androstanols ; Anesthesia, General ; Anesthetics ; Cerebral Angiography ; Dantrolene ; Humans ; Isoflurane ; Korea ; Male ; Malignant Hyperthermia ; Muscle, Skeletal ; Neuromuscular Depolarizing Agents ; Piperidines ; Propofol

Abstract: Suxamethonium chloride is a depolarizing muscle relaxant used in general anesthesia. In overdose, it causes adverse reactions such as bradycardia, arrhythmia, cardiac arrest, and death. The article reviews the progress on testing methods of suxamethonium chloride such as infrared spectroscopy, chemical color reaction, chemical titration, enzyme electrode, chromatography and mass spectrometry.
Anesthesia, General ; Arrhythmias, Cardiac/chemically induced* ; Biosensing Techniques ; Bradycardia/chemically induced* ; Chromatography ; Drug Overdose ; Heart Arrest/chemically induced* ; Humans ; Mass Spectrometry ; Neuromuscular Depolarizing Agents/analysis* ; Spectrophotometry, Infrared ; Succinylcholine/analysis*

Malignant hyperthermia is manifestated in susceptible individuals exposed to triggering drugs, such as depolarizing muscle relaxants and inhalational anesthetics. Various musculoskeletal abnormalities, such as scoliosis, hernias and strabismus, have been associated with malignant hyperthermia susceptibility. During cesarean section of the patient who had undergone scoliosis correction surgery, we experienced malingnant hyperthermia due to succinylcholine and inhalation anesthetics. In our case, as soon as we suspected the episode, all anesthetics were stopped and anesthetic machines were changed to unexposed anesthetic machine for inhalational anesthetics. Dantrolene was given intravenously and the patient was cooled by tepid sponging, cooled fluids. The patient recovered normal temperature and consciousness without any complications.
Anesthetics ; Anesthetics, Inhalation ; Cesarean Section ; Consciousness ; Dantrolene ; Female ; Fever ; Hernia ; Humans ; Malignant Hyperthermia ; Musculoskeletal Abnormalities ; Neuromuscular Depolarizing Agents ; Pregnancy ; Scoliosis ; Strabismus ; Succinylcholine

Malignant hyperthermia (MH) is a potentially fatal pharmacogenetic disorder manifested as a life threatening hypermetabolic crisis in susceptible individuals following exposure to inhalation anesthetics and to depolarizing muscle relaxants. The preoperative diagnosis of MH susceptibility is difficult. The gold standard for determination of MH susceptibility is the in vitro contracture test. However, it is invasive, requiring skeletal muscle biopsy and is not widely available. Recent advances in genetic testing for mutations that result in MH during anesthesia have helped some genetic test have limitations in clinical application due to the diversity of mutations. In Korea, we found the RYR1 genetic mutation by molecular genetic testing for MH susceptibility in a family for which MH had occurred. Based on the results of genetic testing, we could known MH susceptibility of 2 patients. We believe that the genetic testing for MH can be developed and used with some limitations in clinical settings in Korea.
Anesthesia ; Anesthetics, Inhalation ; Biopsy ; Contracture ; Genetic Testing ; Humans ; Korea ; Malignant Hyperthermia ; Molecular Biology ; Muscle, Skeletal ; Neuromuscular Depolarizing Agents ; Ryanodine Receptor Calcium Release Channel

BACKGROUND: Malignant hyperthermia (MH) is a disorder of the skeletal muscle manifested as a life threatening hypermetabolic crisis in susceptible individuals following exposure to inhalation anesthetics and depolarizing muscle relaxants. The gold standard for determination of MH susceptibility is the in vitro contracture test (IVCT). However, it is invasive, requiring skeletal muscle biopsy and is not widely available. We attempted to reevaluate the MH in Korea using a Clinical Grading Scale (CGS) developed by Larach and colleagues to assist in clinical diagnosis. We intend to study CGS as a standardized means for estimating the qualitative likelihood of MH and establishing a Korean MH registry system. METHODS: We obtained twenty-seven case reports from the Korean Journal of Anesthesiology and applied a CGS developed by Larach and colleagues. The raw score of each case was obtained by scoring rules for the MH clinical grading scale, translated to a MH rank, and ranked by the MH likelihood. RESULTS: The overall mortality rate of reported MH was 44.4%. We described for MH rank and likelihood by CGS of cases. The CGS was missing a process in 13 cases for process II, 5 cases for process III and 4 cases for others. CONCLUSIONS: MH CGS is useful to aid the objective definition of this disease and for establishing a national registry system.
Anesthesiology ; Anesthetics, Inhalation ; Biopsy ; Contracture ; Korea ; Malignant Hyperthermia ; Muscle, Skeletal ; Neuromuscular Depolarizing Agents

OBJECTIVEThe aim of this study was to analyses the clinicopathologic features of the patient with myasthenia gravis (MG) occurring after resection of thymoma.

METHODSData of 15 patients were collected. The follow-up range from 8 to 178 (average 76.7) months. A retrospective analysis was performed through comparison with data of all 112 cases without MG, which had not occurred MG during our average 5.5 years follow-up, operated for thymoma in same period. The statistics analysis adopted chi(2) and t test.

RESULTS(1) According to Masaoka's classification of thymoma, stage I in 7 cases, stage II in 4, stage III in 4. Histologic Bernatz's classification: lymphocyte predominant type in 4, epithelial type in 3, mixed type in 7, unknown in 1. According to Osserman's classification of MG, grade I in 7, IIa in 4, IIb in 3, III in 1. The MG onset times was the postoperative narcotic waking duration-137 (average 33.9) months, and the average remission time was 30.9 (0.5 - 120) months. (2) 4 cases who occur MG as soon as pull up narcotic tube, all adopted nondepolarizing muscular relaxants. (3) MG was discovered in 3 cases (3/67) during postoperative radiotherapy until a average dosage of 36 Gy was received in average 24 days. (4) The tendency of occurring MG following resection was found in female patients with longer duration of disease, mixed type, larger and later stage thymoma as compared with the thymoma group.

CONCLUSIONSThe factors including the operation, relatively using overdose relaxation control, choosing unfavorable muscle relaxant and postoperative radiotherapy could induce postoperative MG. An intensive care should be put on the cases with the tendency of occurring postoperative MG.

Adult ; Combined Modality Therapy ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; etiology ; Neuromuscular Depolarizing Agents ; adverse effects ; Postoperative Period ; Radiotherapy, Adjuvant ; adverse effects ; Retrospective Studies ; Sex Factors ; Thymectomy ; adverse effects ; Thymoma ; radiotherapy ; surgery ; Thymus Neoplasms ; radiotherapy ; surgery

BACKGROUND: Malignant hyperthermia (MH) is a disorder of the skeletal muscle manifested as a life threatening hypermetabolic crisis in susceptible individuals following exposure to inhalation anesthetics and depolarizing muscle relaxants. MH susceptibility (MHS) is inherited as an autosomal dominant trait and mutations in the gene encoding skeletal muscle ryanodine receptor (RYR1) are considered a common cause of the disorder. To date, more than 25 RYR1 mutations have been reported in families from Europe and North America. In Korea, however, little is known about the mutations in this candidate gene. METHODS: For the analysis of novel or previously published known RYR1 mutations in 13 exons of the RYR1 gene, PCR amplification and direct sequencing were performed in the proband. After identification of RYR1 mutation in the proband, we performed an extended pedigree study with informed consent from 160 members of a Korean MH family. DNA from 160 members of this family was screened for the presence or absence of RYR1 mutation identified in the proband. RESULTS: We identified a heterozygous G7304A mutation (Arg2435His) in exon 45 of the RYR1 gene in the proband. Six members of the family suffered fatal MH reaction and died. Two members including this proband had a fulminant MH episodes but survived. Two members of the family had presented with severe muscle hypotonia. PCR amplification for the screening of the site yields a fragment of 256 base pair (bp), which is fully cleaved into two fragments of 169 bp and 87 bp by Hga1 in normal individuals and 50% cleaved in individuals with a heterozygous mutation. We found the heterozygous G7304A mutation in 30 individuals from the 160 family members. CONCLUSIONS: This result is the first report to identify the mutation of RYR1 in patients with malignant hyperthermia in Korea. Further larger scale studies will provide important data regarding the frequency of occurrence of the RYR1 mutations in Korea and insight into the practicality of genetic screening relative to diagnosis of MHS and prevention of MH episodes and MH-related problems.
Anesthetics, Inhalation ; Base Pairing ; Diagnosis ; DNA ; Europe ; Exons ; Genetic Testing ; Humans ; Informed Consent ; Korea ; Malignant Hyperthermia* ; Mass Screening ; Muscle Hypotonia ; Muscle, Skeletal ; Neuromuscular Depolarizing Agents ; North America ; Pedigree* ; Polymerase Chain Reaction ; Ryanodine Receptor Calcium Release Channel* ; Ryanodine*