Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026837 (muscle rigidity)
 1,077 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a family in which two sisters with myotonia congenita (MyC) were referred for malignant hyperthermia (MH) evaluation after each developed muscle rigidity with anesthesia. Halothane contracture testing of skeletal muscle in both was consistent with MH susceptibility. A third sister without clinical evidence of MyC was negative on contracture testing. These results suggest an association between MyC and MH susceptibility.
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PMID:Malignant hyperthermia in myotonia congenita. 336 83

The use of dantrolene to reverse severe unexplained postanaesthetic muscle rigidity in a previously "healthy" 13-year-old male is described. Anaesthesia was induced with thiopentone. After intubation with pancuronium, the patient had an entirely uneventful nitrous oxide, oxygen and halothane anaesthetic. Immediately following reversal of the relaxant, the patient developed generalized muscle tightness and rigidity involving the trunk and extremities. This was prolonged and severe enough to interfere with adequate ventilation. The patient also had a prolonged recovery from the anaesthetic. After ruling out malignant hyperthermia and some other causes of rigidity, a tentative diagnosis of myotonia was made. The symptoms responded to IV dantrolene in a total dose of 2.0 mg.kg-1. Further testing failed to establish a definite diagnosis. Dantrolene could be a useful drug in treating such unexplained muscle rigidity.
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PMID:Reversal of prolonged postoperative muscle rigidity by dantrolene: a case report. 340 20

Reduction in the anesthetic requirement of halothane by narcotics has been studied extensively in humans and animals. Problems of respiratory depression, cardiovascular depression, muscle rigidity, and abuse potential make narcotics less than ideal as supplements to general anesthesia with inhalational agents. Spiradoline, a clinical candidate, is a highly potent and selective kappa-agonist. As such it was considered important to study the effects of spiradoline on the minimum anesthetic concentration (MAC) of halothane required to block responses to noxious stimulation. The results of these experiments in rats showed a dose and plasma concentration-dependent reduction in halothane MAC over a wide range of subcutaneous doses of spiradoline (0.03 to 300 mg/kg). A maximum MAC reduction of 70% was obtained. Plasma levels of spiradoline (6 to 1800 ng/ml) were linearly related to dose. Measurement of blood pressure, heart rate, and PCO2 determined over the course of each experiment showed minor variations which would be acceptable if observed in a clinical setting. It is concluded that spiradoline has promise as an anesthetic supplement.
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PMID:Enhancement of anesthetic effect of halothane by spiradoline, a selective kappa-agonist. 341 91

Dantrolene sodium acts primarily by affecting calcium flux across the sarcoplasmic reticulum of skeletal muscle. Recently, dantrolene has been used very successfully in the treatment of several rare hypercatabolic syndromes which have previously been associated with high mortality rates. In malignant hyperthermia, where early diagnosis and treatment usually with intravenous dantrolene in association with other supportive measures (and often subsequent dantrolene therapy) is performed, recovery is seen in virtually 100% of patients. There is a rapid resolution of hyperthermia, dysrhythmias, muscle rigidity, tachycardia, hypercapnia, mottled or cyanotic skin, and metabolic acidosis, and a slower normalisation of myoglobinuria and elevated serum creatine phosphokinase levels. In patients with family history or previous episodes of malignant hyperthermia, prophylactic treatment with dantrolene prior to anaesthesia prevents the syndrome occurring in most cases. Where malignant hyperthermia has developed patients have been successfully treated with further dantrolene therapy. Dantrolene has also been used successfully in the treatment of a few cases of heat stroke and the neuroleptic malignant syndrome--both of which have many similarities to malignant hyperthermia. Dantrolene is well established in the treatment of patients with muscle spasticity where it generally improves at least some of the components of spasticity (i.e. hyper/hypotonia, clonus, muscle cramps and spasms, resistance to stretch and flexor reflexes, articular movement, neurological and motor functions and urinary control). However, in some patients, particularly those with multiple sclerosis, dantrolene may not be effective, and in many cases muscular strength may diminish. Long term dantrolene therapy has been associated with hepatic toxicity and may cause problems in patients treated for disorders of muscle spasticity. Thus, dantrolene offers a unique advance in the therapy available for the treatment of hypercatabolic disorders and is also useful in the treatment of muscle spasticity of various aetiology.
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PMID:Dantrolene. A review of its pharmacodynamic and pharmacokinetic properties and therapeutic use in malignant hyperthermia, the neuroleptic malignant syndrome and an update of its use in muscle spasticity. 352 59

A case of postoperative neuroleptic malignant syndrome is presented. A healthy 23-year-old male underwent a shoulder repair under uneventful fentanyl, halothane, nitrous oxide and oxygen anaesthesia. He received droperidol 5 mg IV and metoclopramide 10 mg IV intraoperatively to prevent postoperative nausea. Postoperatively, the patient developed autonomic instability, fever and generalized muscle rigidity. His level of consciousness was depressed. These findings were consistent with the diagnosis of neuroleptic malignant syndrome. The supportive treatment of the patient included active cooling measures, muscle relaxation and mechanical ventilation. The ability of anti-dopaminergic agents, including metoclopramide and droperidol, to precipitate the neuroleptic malignant syndrome is discussed. Treatment of the neuroleptic malignant syndrome is briefly discussed.
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PMID:Postoperative neuroleptic malignant syndrome. A case report. 366 20

Trace amounts of [14C]2-deoxyglucose (2-DG) were used to detect regions of the brainstem involved in forelimb stepping in thalamic and low spinal cats. Under ether anesthesia, cats were transected at the stereotaxic A12 level and T10 segment. Two hours later, 50 microCi/kg of 2-DG was infused i.v. and one of 4 procedures was followed: 3 cats stepped on a motor-drive treadmill (Stepping), 3 were kept in a stationary standing position (Rigidity), 2 were anesthetized with sodium pentobarbital (Anesthetized), and 2 were stimulated in the mesencephalic locomotor region (MLR-induced). Absolute optical densities of the autoradiograms corresponding to identified anatomical structures of the brainstem were generally in the following order: Stepping greater than Anesthetized greater than MLR-induced greater than Rigidity. The 2-DG uptake relative to the pyramidal tract (2-DG ratio) also was compared for each of the 4 experimental procedures. In the Stepping cats, the 2-DG ratio was highest in the vestibular nuclei, periaqueductal gray, red nucleus and thalamic nuclei. In the Rigid cats, the 2-DG ratio was highest in the medial vestibular nucleus and subthalamic and thalamic nucleus. These findings suggest that the 2-DG tracer method can be useful in associating neural structures with specific kinds of motor functions within a cat. This is particularly true when using the relative activities of different neural structures and in comparing specific neural structures across cats under different experimental conditions when the amount of 2-DG infused is standardized and the optical densities of the autoradiograms are calibrated to a specific level of 2-DG.
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PMID:Application of autoradiographic analysis of 2-deoxyglucose in the study of locomotion. 368 80

We report the case of a 4.9-year-old boy with congenital hydrocephalus and obstruction of his shunt who just after the induction of anaesthesia suddenly developed generalized muscle rigidity that made intubation impossible. Because of temporary mydriasis the situation could hardly be differentiated from acute cerebral herniation. The lack of any decrease in muscle tone after emergency trephination and drainage of the right lateral ventricle and the immediate improvement following intravenous dantrolene left no doubt about the diagnosis of malignant hyperthermia. The diagnosis was confirmed by the increase in serum creatine phosphokinase and the documentation of massive myoglobinuria. Reconstruction of the course of the first anaesthetic necessary for shunt implantation at the age of 62 days revealed that the same symptoms already had occurred. However, they then were not attributed to malignant hyperthermia but interpreted as symptoms of acute herniation. A detailed description of this first anaesthetic is given which again elucidates the problems associated with the abrupt onset of muscular hypertonus in a patient with neurologic disorder; moreover this may well be the first published case report of malignant hyperthermia at the age of just 2 months.
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PMID:[Symptoms of acute cerebral hernia following induction of anesthesia in hydrocephalus--malignant hyperthermia in reality]. 378 92

Seventy-seven patients who developed masseter muscle rigidity (MMR) after receiving succinylcholine to facilitate tracheal intubation were evaluated for malignant hyperthermia (MH) susceptibility by in vitro halothane and caffeine contracture tests. Thirty-nine patients were diagnosed as MH-susceptible. Neither age, sex, nor type of surgery or anesthesia distinguished MH-susceptible from nonsusceptible patients. Two susceptible and two nonsusceptible patients had evidence of a myopathy. Fifty-two patients had serum creatine phosphokinase (CPK) levels measured in the perioperative period. Although all values were above normal, CPK values equal to or greater than 20,000 IU within 24 hr of trismus (in the absence of myopathy) were observed in six of 30 patients diagnosed as MH-susceptible, but were found in none of the nonsusceptible patients. Considering the high percentage of patients exhibiting MMR that are indeed susceptible to MH (approximately 50%) compared to estimates of MH in the population as a whole (approximately 0.005%), MMR should be considered a presumptive sign of MH. Perioperative CPK values greater than 20,000 IU are highly suggestive of MH susceptibility. Patients exhibiting MMR should be evaluated for MH susceptibility and myopathies. Succinylcholine should be avoided for subsequent anesthetics in patients with a history of MMR.
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PMID:Masseter muscle rigidity and malignant hyperthermia susceptibility. 394 3

The safety of etomidate for induction of anesthesia in malignant hyperthermia-susceptible (MHS) pigs was evaluated in a two-phase experiment. Two litters of Purebred Poland China pigs, one MHS (n = 4) and the other malignant hyperthermia-resistant (MHR) (n = 4) were used. Phase I compared MHS vs MHR animals in terms of cardiovascular, metabolic, and skeletal muscle rigidity responses to etomidate and fentanyl anesthesia and to a subsequent malignant hyperthermia (MH) challenge with halothane-succinylcholine. When three of the four criteria for the diagnosis of MH occurred (rigidity, tachycardia, or increases in temperature or end-tidal CO2) in an animal, phase I was terminated. In phase II, only the MHS animals were used and experimental procedures were as in phase I except thiopental replaced etomidate. In phase I, evidence was inadequate to support the diagnosis of MH based upon responses of MHS pigs to the infusion of etomidate even though the infusion of etomidate in MHS pigs was associated with statistically significant increases in body temperature and plasma lactate levels above those observed in MHR pigs. Heart rate and bicarbonate levels were lower in MHS than in MHR pigs during etomidate infusion. With discontinuation of etomidate and a subsequent challenge with halothane-succinylcholine, all four pigs developed the MH syndrome within 15-30 min. Thiopental replacement of etomidate in the phase II experiment resulted in a twofold greater time (45-75 min) for halothane-succinylcholine to trigger MH in the susceptible pigs.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Malignant hyperthermia: is etomidate safe? 398 91

A report is made of a 44-year-old female who died of malignant hyperthermia during general anesthesia for an operation of left subtrochanteric femoral fracture. Symptoms began with increases in heart rate and blood pressure after the introduction of anesthesia with halothane and were accelerated by the administration of succinylcholine, followed by muscle rigidity and high temperature. The typical wine red urine was observed. The oral temperature of 42 degrees C was recorded within 1 h after succinylcholine. The rectal temperature was 30 degrees C 22 h after death. Severe pulmonary edema and tubular necrosis of the kidney were found by postmortem examination. No latent myopathy was observed. The creatine phosphokinase levels in plasma obtained after the onset of malignant hyperthermia and at the time of autopsy were abnormally high and were 5632 and 34,854 mU/ml, respectively. Plasma myoglobin levels were 130,000 and 2.8 X 10(6) ng/ml, respectively.
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PMID:An autopsy case of malignant hyperthermia. 398 93


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