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 case of a juvenile male with muscle rigidity caused by cerebral palsy who experienced intraoperative sudden death due to pulmonary fat embolism after multiple muscle-release and tenotomy of the bilateral lower limbs. Data were obtained through review of the surgical and anesthesia records, as well as from autopsy and histopathological examination. All surgical procedures were performed within the same operation, beginning with the right lower limb and then proceeding with the left lower limb, with application of a pneumatic tourniquet to avoid intraoperative hemorrhage. Slight changes in the hemodynamics were noticed after release of the right tourniquet. Further, sudden onset of hypotension, severe bradycardia, and a marked decrease in percutaneously monitored oxygen saturation occurred just after release of the left tourniquet when the left limb was raised for casting. The patient died despite immediate and vigorous cardiopulmonary resuscitation. At autopsy performed 20 h after death, examination of the lungs revealed a pale surface, slight edema, and obvious fat droplets in the vessels at the cut surfaces. Histopathological examination with fat staining was notable for the presence of pulmonary fat embolism. These results suggest that restoration of venous return after removal of the tourniquet combined with massive fat embolism from dead spaces was the likely cause of death.
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PMID:An autopsy case of intraoperative death due to pulmonary fat embolism--possibly caused by release of tourniquet after multiple muscle-release and tenotomy of the bilateral lower limbs. 1692 Mar 5

We report on a patient who developed two episodes of severe muscle rigidity, increased endtidal CO2 and increased creatine phosphate kinase associated with sevoflurane anesthesia. Dysrhythmias and hyperthermia were not observed and dantrolene was not administered. Genetic testing for the 17 known mutations associated with malignant hyperthermia (MH) was negative. Although we cannot rule out MH or other neuromuscular diseases we suggest that this rare event may be a direct effect of sevoflurane.
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PMID:Repeat episodes of severe muscle rigidity in a child receiving sevoflurane. 1697 40

Thiafentanil oxalate, previously known as A-3080, is a synthetic opioid used for chemical immobilization of a variety of nondomestic hoofstock species. This study compared the combination of thiafentanil oxalate, medetomidine, and ketamine (TMK; 0.09 +/- 0.02 mg/kg, 0.01 +/- 0.003 mg/kg, and 1.36 +/- 0.33 mg/kg, respectively) with the combination of medetomidine and ketamine (MK; 0.09 +/- 0.02 mg/kg and 3.48 +/- 0.55 mg/kg, respectively) for anesthetization of 17 captive male axis deer (Axis axis) for vasectomy. Nine deer received TMK and eight deer received MK via projectile syringe during the months of January and February, 2005. Mean induction and arousal times, vital signs, and arterial blood gas values were monitored and compared. All animals received supplemental oxygen during the surgical procedure. Animals receiving TMK were reversed with naltrexone (100 mg/mg thiafentanil) and atipamazole (5 mg/mg medetomidine). Animals receiving MK were reversed with atipamazole (5 mg/mg medetomidine). Two MK animals and three TMK animals required supplementation with ketamine i.v. immediately upon handling. Six of the nine animals immobilized with TMK required intubation for positive-pressure ventilation. Two of these six animals also required isoflurane to maintain anesthesia. Mean induction time was 3.5 +/- 2.0 min in the TMK group, and 9.8 +/- 6.7 min in the MK group. Despite shorter mean induction times, animals anesthetized with TMK experienced unpredictable inductions, apnea, muscle rigidity, limb movement, and significant respiratory and metabolic lactic acidosis. MK resulted in smoother inductions, better respiratory function, and less adverse metabolic disturbances, and thus was considered superior to TMK for anesthesia in captive axis deer at the dosages tested.
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PMID:Anesthesia of male axis deer (Axis axis): evaluation of thiafentanil, medetomidine, and ketamine versus medetomidine and ketamine. 1731 36

Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle that presents as a hypermetabolic response to potent volatile anesthetic gases such as halothane, sevoflurane, desflurane and the depolarizing muscle relaxant succinylcholine, and rarely, in humans, to stresses such as vigorous exercise and heat. The incidence of MH reactions ranges from 1:5,000 to 1:50,000-100,000 anesthesias. However, the prevalence of the genetic abnormalities may be as great as one in 3,000 individuals. MH affects humans, certain pig breeds, dogs, horses, and probably other animals. The classic signs of MH include hyperthermia to marked degree, tachycardia, tachypnea, increased carbon dioxide production, increased oxygen consumption, acidosis, muscle rigidity, and rhabdomyolysis, all related to a hypermetabolic response. The syndrome is likely to be fatal if untreated. Early recognition of the signs of MH, specifically elevation of end-expired carbon dioxide, provides the clinical diagnostic clues. In humans the syndrome is inherited in autosomal dominant pattern, while in pigs in autosomal recessive. The pathophysiologic changes of MH are due to uncontrolled rise of myoplasmic calcium, which activates biochemical processes related to muscle activation. Due to ATP depletion, the muscle membrane integrity is compromised leading to hyperkalemia and rhabdomyolysis. In most cases, the syndrome is caused by a defect in the ryanodine receptor. Over 90 mutations have been identified in the RYR-1 gene located on chromosome 19q13.1, and at least 25 are causal for MH. Diagnostic testing relies on assessing the in vitro contracture response of biopsied muscle to halothane, caffeine, and other drugs. Elucidation of the genetic changes has led to the introduction, on a limited basis so far, of genetic testing for susceptibility to MH. As the sensitivity of genetic testing increases, molecular genetics will be used for identifying those at risk with greater frequency. Dantrolene sodium is a specific antagonist of the pathophysiologic changes of MH and should be available wherever general anesthesia is administered. Thanks to the dramatic progress in understanding the clinical manifestation and pathophysiology of the syndrome, the mortality from MH has dropped from over 80% thirty years ago to less than 5%.
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PMID:Malignant hyperthermia. 1745 35

Stiff-person syndrome is an autoimmune disease characterized by muscle rigidity accompanied by decreased respiratory function. We report a patient with this syndrome who underwent thymectomy under general anesthesia. A 79-year-old woman complaining of increasing muscle rigidity over the past four months was transferred to this hospital. Marked speech disturbance and dysphagia were observed on admission. The diagnosis of stiff-person syndrome was made based on an increase in serum anti-glutamic acid decarboxylase antibody level and thymoma in the anterior mediastinum. Following alleviation of muscle rigidity by high-dose gamma-globulin, thymectomy was scheduled. General anesthesia was given with propofol, fentanyl and epidural ropivacaine. Propofol was continuously infused to maintain BIS below 60 and vecuronium was intermittently administered when muscle contraction was observed in response to electrical stimulation of the ulnar nerve. Despite full recovery of muscle contractility following surgery, tidal volume was too low to remove the tracheal tube, and mechanical ventilation was continued in ICU. One hour after admission to ICU, the tracheal tube was removed, with no marked changes in respiratory condition thereafter. Since many anesthetics are respiratory suppressants that can delay the recovery of respiratory function, careful monitoring of respiratory condition is required postoperatively.
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PMID:[Anesthetic management for thymectomy in a patient with stiff-person syndrome]. 1796 28

Most opioids used in anaesthesia are of the anilidopiperidine family, including fentanyl, alfentanil, sufentanil and remifentanil. While all share similar pharmacological properties, remifentanil, the newest one, is probably the most original, which is the reason this review focusses especially on this drug. Remifentanil is a potent mu-agonist that retains all the pharmacodynamic characteristics of its class (regarding analgesia, respiratory depression, muscle rigidity, nausea and vomiting, pruritus, etc.) but with a unique pharmacokinetic profile that combines a short onset and the fastest offset, independent of the infusion duration. Consequently, it offers a unique titratability when its effects need to be quickly achieved or suppressed, but it requires specific drug delivery schemes such as continuous infusion, target-controlled infusion and anticipated postoperative pain treatment. Kinetic differences between opioids used in anaesthesia and some clinical uses of remifentanil are reviewed in this chapter.
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PMID:Remifentanil and other opioids. 1817 97

Freeman-Sheldon syndrome, or distal arthrogryposis type 2A, is a rare congenital myopathy and dysplasia characterised by multiple contractures, abnormalities of the head and face, defective development of the hands and feet and skeletal malformations. The facial muscle contracture produces the typical 'whistling face' appearance. Anaesthetic issues include difficult intravenous access, difficult airway and postoperative pulmonary complications. Although an association with malignant hyperthermia has been suggested, this has not been confirmed. We report the management of a seven-year-old girl with Freeman-Sheldon syndrome undergoing anterior and posterior spinal surgery and describe a successful anaesthetic regimen based on a total intravenous anaesthesia technique with remifentanil and propofol without neuromuscular blocking agents. The child had an uneventful anaesthetic and postoperative course. We believe the presence of the myopathy warranted the use of a 'non-triggering' anaesthetic, as suxamethonium and volatile agents may be associated with significant complications such as muscle rigidity and rhabdomyolysis in myopathic patients, even in the absence of malignant hyperthermia.
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PMID:Anaesthetic management of a child with Freeman-sheldon syndrome undergoing spinal surgery. 1836 Oct 19

Stiff-person syndrome is a rare disease characterized by muscle rigidity and painful spasms in the axial and limb muscles. The authors reported here a case of an axilally lymphadenectomy in a 46-year-old woman with stiff-person syndrome. With train of four ratio (TOFR) monitoring at the ulnar nerve, general anesthesia was induced and maintained with fentanyl, vecuronium and propofol with target controlled infusion. A TOFR, BIS monitor and invasive arterial pressure monitoring were employed. During the operation, there was no muscle rigidity and spasm. Ten minutes after the operation, she was fully awake and train of four ratio recovered to 95%, and extubated uneventfully. We chose propofol, because of previous reports about prolonged hypotonicity by interaction of baclofen and isoflurane. Preoperative good symptom control, choice of total intravenous anesthesia (TIVA), and application of the electrical nerve stimulator prevented postoperative hypotonia and resulted in safe anesthetic management.
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PMID:[Anesthesia for a patient with stiff-person syndrome]. 1841 1

A 32-year-old man underwent subtotal thyroidectomy for Basedow's disease under general anesthesia. Preoperatively, the free thyroxine (fT4) and free triiodothyronine (fT3) levels were suppressed and thyroid stimulating hormone level was elevated with administration of iodine and propylthiouracil. Heart rate was 52 beats x min(-1) in sinus rhythm. General anesthesia was induced with fentanyl, propofol and vecuronium, and maintained with nitrous oxide, oxygen and sevoflurane. Systolic blood pressure was controlled within 100 and 130 mmHg. Rectal temperature was 36.5 degrees C after anesthesia induction, gradually rising at a range of 0.4 and 0.7 degrees C per hour, up to 38.6 degrees C four hours after the operation. Arterial blood gas showed bicarbonate 17.1 mEq x l(-1) and base excess -8.1 mmol x l(-1). The metabolic acidosis with normal anion gap lasted during and after the operation. We cooled his body with cold acetyl linger fluid and cooling mattress, and administered sodium bicarbonate. Heart rate increased to 96 beats x min(-1) before the end of operation. Subtotal thyroidectomy was finished in 5 hours 16 minutes. The amount of blood loss was 950 ml. Postoperatively, the serum fT4 and fT3 were suppressed. The serum creatine kinase and lactate dehydrogenase levels increased slightly. He did not show muscle rigidity and neurological disorders. We suspect that he has developed thyroid storm-like symptoms such as hyperthermia and tachycardia induced by subtotal thyroidectomy. Metabolic acidosis might be the result of distal tubular acidosis, which rarely accompanies Basedow's disease. Arterial blood gas analysis and urinalysis should be performed, preoperatively.
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PMID:[Hyperthermia and metabolic acidosis during subtotal thyroidectomy for a patient with Basedow's disease]. 1864 49

This case report details the onset of masseter muscle rigidity, elevated creatine kinase levels, and rhabdomyolysis following a sevoflurane mask induction and succinylcholine administration in a 12-year-old boy. The patient had no family or personal history of neuromuscular disease or malignant hyperthermia. Hyperkalemia, metabolic acidosis, and rhabdomyolysis occurred within 75 minutes of masseter muscle rigidity. Subsequent to this event, it was recommended that the patient undergo a workup for neuromuscular disease and malignant hyperthermia with muscle biopsy. Until this workup is completed, the family should advise anesthesia providers that the patient is "malignant hyperthermia susceptible." Masseter muscle rigidity, elevated creatine kinase levels, and rhabdomyolysis will be thoroughly discussed in this article.
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PMID:Masseter muscle rigidity, elevated creatine kinase, and rhabdomyolysis following succinylcholine administration: a case report. 1894 62


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