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)

Malignant hyperthermia is an autosomal dominant disorder with variable expressivity that is caused by a membrane defect in the sarcolemma of myofibrils. A patient with strabismus (esotropia) had tachycardia and masseter muscle rigidity on exposure to succinylcholine chloride and halothane, but because of rapid recognition of the condition and discontinuation of the procedure, the potentially lethal complications of malignant hyperthermia did not develop. A serum creatine phosphokinase level showed a substantial increase above normal. Two weeks later, the patient underwent successful correction of the strabismus under general anesthesia, using morphine sulfate and thiopental sodium without complication. This condition is of interest to ophthalmologists because it occurs with increasing frequency in patients with strabismus and ptosis, and it may be triggered by certain local anesthetic agents often used by ophthalmologists.
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PMID:Suspected malignant hyperthermia in a strabismus patient. A case report. 725 98

The anaesthetic properties of ketamine in sheep were evaluated and compared with the results of a combination of ketamine/xylazine and ketamine/xylazine/atropine. Premedication of xylazine/atropine followed by intravenous injection of ketamine hydrochloride appeared to result in satisfactory immobilisation and anaesthesia for surgical operations of short duration. This combination effectively reduced some of the undesirable effects of ketamine, such as muscle rigidity, insufficient suppression of reflexes and tachycardia. The action on haematological factors was studied and significant increases in blood glucose content were detected during anaesthesia.
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PMID:Evaluation of the anaesthetic properties of ketamine and a ketamine/xylazine/atropine combination in sheep. 726 83

The incidence, etiology, clinical manifestations, and management of malignant hyperthermia (MH) are reviewed. The syndrome of MH is recognized as one of the causes of anesthesia-related deaths. It is considered pharmacogenetic because both an abnormal gene and precipitating environmental factors are necessary to produce an acute reaction. Metabolic defects, involving a derangement of calcium dynamics, appear to be the common characteristic of susceptible individuals. Calcium release and uptake from the sarcoplasmic reticulum is altered when an individual with MH is exposed to certain anesthetic agents or triggering physical and emotional stresses. Muscle rigidity, tachycardia, tachypnea, and high fever can lead to other complications and death. Management of an acute reaction of MH includes cooling methods to lower body temperature, hyperventilation, sodium bicarbonate control of acidosis, maintenance of fluid and electrolyte balance, and the administration of dantrolene sodium. A thorough family history, baseline CPKs, caffeine-halothane contracture tests, and ultramicroscopic examination of muscle biopsy specimens are recommended as screening techniques. The early administration of dantrolene sodium in acute reaction of MH has been shown to rapidly alleviate the symptoms and ensuing severe complications. Individuals with a strong family history of MH or previous episodes may be treated with oral dantrolene sodium before surgery to effectively prevent a crisis, and after surgery to prevent recurrence.
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PMID:Malignant hyperthermia: current perspectives. 728 92

The behaviour of 48 children ranging from weeks to eight years was observed and compared after four different anaesthesia methods. Either ethrane or halothane was used with or without induction with ketamine i.m. (5 mg/kg bodyweight). Restlessness, the depth of postanaesthetic sleep, shivering, muscle rigidity and vomiting were evaluated every 15 min. up to one hour postoperatively using a graduation from 1--4. Ketamine combined with halothane showed significantly less postoperative restlessness than all other methods. No statistically proven differences were seen in the other criteria, which were noticed more than once. The psychic effects as well as the practical clinical application of this method are discussed.
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PMID:[Steal-induction with ketamine in childhood: comparison of the postanaesthetic period (author's transl)]. 746 56

The objective of this study was an evaluation of the prevalence of myopathies in paediatric patients scheduled for orthopaedic surgery (clubfoot) performed under regional anaesthesia. Seventeen infants scheduled for lower limb orthopaedic surgery were studied to verify coexisting neuromuscular disorders with electromyography and muscle biopsy during surgery. All surgical procedures were performed under caudal block or spinal anaesthesia, associated with light general anaesthesia. No major cardiorespiratory, neurological or malignant hyperthermic complications (muscle rigidity, arrhythmias, hyperpyrexia) were observed. Combined neurological, electromyographic and biopsy studies showed a high rate of myopathic changes (70%). Performance of clubfoot surgery under light general anaesthesia with regional techniques was free from any problems. The high rate of myopathic changes (70%) observed in the muscle biopsies suggests that precautions should be taken with paediatric patients for clubfoot surgery and a regional anaesthesia technique with adequate monitoring may be helpful to prevent possible malignant hyperthermia related problems.
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PMID:Prevalence of unsuspected myopathy in infants presenting for clubfoot surgery. 748 36

Dexmedetomidine is a new alpha 2 adrenergic agonist anaesthetic adjuvant. In animal studies, dexmedetomidine produced muscle flaccidity and prevented opioid-induced muscle rigidity, apparently via a central mechanism. The effect of dexmedetomidine on the neuromuscular junction or on non-depolarizing neuromuscular block during anaesthesia has not been reported. We have studied in the anaesthesized rat, the effects of dexmedetomidine on vecuronium-induced twitch depression. Wistar rats (n = 35) were anaesthetized and their lungs ventilated to maintain normocapnia. An infusion of vecuronium of 2.3 (SEM 0.1) micrograms kg-1 min-1 produced a stable twitch height (T1) depression of the tibial nerve of 53 (2)% of control in all groups. Rats were allocated randomly to receive either saline or dexmedetomidine 10, 30 or 100 micrograms kg-1 i.v. and T1 height was measured continuously for 60 min. Dexmedetomidine did not significantly affect T1 height during the first 30 min of infusion. At later times there were minor differences between groups. With cessation of the infusion of vecuronium, T1 height recovered rapidly to normal in all groups. These data suggest that the neuromuscular blocking properties of dexmedetomidine are unlikely to be produced by action at the neuromuscular junction.
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PMID:Dexmedetomidine does not modify the neuromuscular blocking action of vecuronium in the anaesthetized rat. 773 68

Malignant hyperthermia (MH) susceptibility remains the commonest cause of death owing to general anaesthesia. In humans, genetically predisposed to MH, anaesthesia can induce skeletal muscle rigidity, hypermetabolism and hyperthermia, which if not immediately reversed can lead to tissue injury and death. In swine, the corresponding condition leads to stress-induced deaths and devalued meat products. Aberrant behaviour in the calcium (Ca2+) release channel (the ryanodine receptor) of skeletal muscle sarcoplasmic reticulum has been implicated in the cause of both the porcine and human syndromes by biochemical, physiological and molecular genetic analysis. In swine, a single mutation in the ryanodine receptor gene (RYR1) can account for all cases of MH in all breeds, but a series of different RYR1 mutation are uncovered in human families with MH. In addition, the lack of linkage between MH and RYR1 in some families indicates a heterogeneous genetic basis for the human MH.
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PMID:The genetic basis of malignant hyperthermia. 797 21

A 42-year-old woman with myotonic dystrophy underwent general anesthesia twice. In the first operation, muscle rigidity was recognized when the patient was placed on lithotomy position under anesthesia with thiopental and nitrous oxide. In the second operation, rigidity was not observed under vecuronium, sevoflurane-nitrous oxide anesthesia. Neuromuscular monitoring of this patient did not show any difference of neuromuscular blockade with vecuronium compared with other 9 normal patients. We conclude that vecuronium and sevoflurane can be used safely in a patient with myotonic dystrophy under neuromuscular monitoring.
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PMID:[General anesthesia conducted twice in myotonic dystrophy]. 809 79

Stereotactic thalamotomy of the VIM (ventral intermediate) nucleus is considered as the best neurosurgical treatment for Parkinsonian and essential tremors. However, this surgery, especially when bilateral, still presents a risk of recurrence and neurological complications. We observed that acute VIM stimulation at frequencies higher than 60 Hz during the mapping phase of the target suppressed the tremor of Parkinson's disease (PD) and essential tremor (ET). This effect was immediately reversible at the end of the stimulation. This was initially proposed as an additional treatment for patients already thalamotomized on the contralateral side, and then extended as a regular procedure for extra-pyramidal dyskinesias. Since January 1987, we implanted 126 thalami in 87 patients (61 PD, 13 ET, 13 dyskinesias of various origins). Deep brain stimulation electrodes were stereotactically implanted under local anaesthesia, using stimulation and micro-recording to delineate the best site of stimulation. Electrodes were subsequently connected to implantable programmable stimulators. The optimal frequency was around 130 to 185 Hz. The results (evaluated by a neurologist from 0 = no effect to 4 = perfect relief) are related to the type of tremor. Altogether, 71% of the 80 patients benefited from the procedure with grade 3 and 4 results. In 88% of the PD cases, the results were good (grade 3) or excellent (grade 4) and stable with time. Rigidity was moderately for a long improved but akinesia was not. The same level of improvement was observed in 68% of the ET patients and only in 18% of the other types of dyskinesias.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Chronic VIM thalamic stimulation in Parkinson's disease, essential tremor and extra-pyramidal dyskinesias. 810 99

A young man underwent anaesthesia and surgery after multiple fractures. After 2 hours of anaesthesia, the patient developed hypercapnia, acidosis, hyperpyrexia and mild muscle rigidity. He was treated for malignant hyperthermia. Muscle tension studies with caffeine-halothane and muscle histology proved normal. The differential diagnosis of this abnormal metabolic response is briefly discussed.
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PMID:A hypermetabolic reaction during anaesthesia and surgery. A case report. 821 15


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