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)

Circular dichroism (CD) of serum alpha1-acid glycoprotein, urinary Bence Jones protein, human carbonic anhydrase B, deoxyribonuclease from bovine pancreas, porcine pepsinogen, and plasminogen from human serum was tested in the absence and presence of 0.005-0.05 M sodium dodecyl sulfate. It was found that in all cases the CD spectra of these proteins were modified by the dodecyl sulfate into spectra indicating the presence of a moderate content of alpha-helix. The transitions were enhanced by addition of acid (pH 2.1-4.4) in all cases tested. Comparison of the various proteins with respect to the amount of reconstruction of the main chain conformation showed that the amount of helix formed depended on the amino acid composition of the protein. Rigidity due to cross-linking by disulfide bridges is the strongest deterrant to the conformational change of the main chain. The CD bands of the native proteins in the 250-350 nm spectral zone were extinguished by sodium dodecyl sulfate, and new weak bands were observed the positions of which corresponded approximately to those of the native proteins. In all cases, except the carbonic anhydrase B, the bands of thus denatured proteins were negative.
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PMID:Conformational transitions of non-helical proteins effected by dodecyl sulfate. Circular dichroism of alpha1-acid glycoprotein, Bence Jones protein, carbonic anhydrase B, deoxyribonuclease, pepsinogen, and plasminogen. 5 6

A case of malignant hyperthermia in a Black boy is presented. He developed this condition during repair of a cleft palate, with halothane as the triggering agent. The importance of the high incidence of malignant hyperthermia in patients with certain musculoskeletal abnormalities is stressed. Despite a cool and well air-conditioned theatre, the patient's temperature was 41 degree C when the condition was suspected. At that stage general muscle rigidity was present. The patient was successfully treated with procainamide, sodium bicarbonate and hydrocortisone; surface cooling (with ice packs) was instituted and the stomach was washed out with ice-cold Ringer's solution. Over a period of 14 days serum creatine phosphokinase values decreased from 630 IU (on the day of the incident) to 12 IU. A muscle biopsy showed variation in muscle fibre size. Electron microscopical studies showed myofibrillar disruption and folding of the basement membrane. A modified version of Denborough's technique was used for the in vitro exposure of muscle strips to halothane and suxamethonium. Isometric contraction was measured and recorded. A severe contraction followed the exposure of muscle strips to halothane, which confirmed the diagnosis.
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PMID:[Malignant hyperthermia in a black child. A case report]. 69 25

The effect of normal and artificially induced rigor mortis on the vascular passage of erythrocytes and fluid through isolated dog hearts was studied. Increased rigidity of 6-mm thick transmural sections through the centre of the posterior papillary muscle was used as an indication of rigor. The perfusibility of the myocardium was tested by injecting 10 ml of 1% sodium fluorescein in Hanks solution into the circumflex branch of the left coronary artery. In prerigor hearts (20 minute incubation) fluorescein perfused the myocardium evenly whether or not it was preceded by an injection of 10 ml of heparinized dog blood. Rigor mortis developed in all hearts after 90 minutes incubation or within 20 minutes of perfusing the heart with 50 ml of 5 mM iodoacetate in Hanks solution. Fluorescein injected into hearts in rigor did not enter the posterior papillary muscle and adjacent subendocardium whether or not it was preceded by heparinized blood. Thus the vascular occlusion caused by rigor in the dog heart appears to be so effective that it prevents flow into the subendocardium of small soluble ions such as fluorescein.
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PMID:The effect of rigor mortis on the passage of erythrocytes and fluid through the myocardium of isolated dog hearts. 72 86

After 9 years of treatment for Parkinson's disease, a 68-year-old woman developed the complications of neuroleptic malignant syndrome (NMS) and disseminated intravascular coagulation (DIC) while she was still receiving levodopa, bromocriptine and amantadine hydrochloride. The patient displayed a high fever (40 degrees C), impaired consciousness, marked systemic muscle rigidity, tremor and bloody stools. The diagnosis of NMS and DIC was made on the basis of the symptoms and the results of blood serological tests. The antiparkinsonian drugs that had been administered until her admission to our hospital were continued unchanged, while the NMS was treated with dantrolene sodium and the DIC, with nafamostat mesilate. Both of the above-mentioned therapies were effective. The present case is rare in that the patient developed NMS and DIC during treatment and not after the discontinuation of the antiparkinsonian drugs.
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PMID:Successful treatment of levodopa-induced neuroleptic malignant syndrome (NMS) and disseminated intravascular coagulation (DIC) in a patient with Parkinson's disease. 129 27

The main features of the Neuroleptic Malignant Syndrome (NMS), a complication of neuroleptic therapy, are fever, muscle rigidity, autonomic dysfunction, and an alteration in consciousness level. We describe five cases of NMS comprising 0.6% of acute neuroleptically-treated admissions to a psychiatric hospital over a one-year period. All patients, four females aged 26 to 63 years, and one male, aged 65 years, were of African origin and received multiple neuroleptic drugs, at least one of which was a depot preparation. Four were being treated for functional psychiatric disorders while one had dementia. All patients had fever and depressed consciousness level while four had rigidity and autonomic dysfunction. Serum creatine phosphokinase was elevated in 4 cases, and there was indirect evidence of myoglobinuria in 3 cases suggested by a positive urine dipstick test for blood despite the absence of red cells on microscopy. Rhabdomyolysis was associated with renal failure in one case. Both bromocriptine mesylate and dantrolene sodium were given in two cases. Three patients died in hospital, one with persistent rigidity and progressive decubitus ulceration, one from peritonitis following peritoneal dialysis, and another suddenly. Early recognition of NMS is important; it should be considered in any patient on neuroleptic therapy who develops fever, rigidity or alteration in consciousness level.
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PMID:Neuroleptic malignant syndrome among acute psychiatric admissions in Barbados. 156 88

Malignant hyperthermia (MH) is an adverse reaction most frequently associated with the administration of halogenated inhalational anesthetic agents and the depolarizing muscle relaxant succinylcholine. Characteristic findings are a hypermetabolic state accompanied by extreme hyperpyrexia, acidosis, rhabdomyolysis, and generalized muscle rigidity, often involving the masseter muscles. Dantrolene sodium, which was approved in 1979 by the FDA for use in the prevention of MH in high-risk patients, has neurologic and gastrointestinal side effects. At the Children's National Medical Center (CNMC), 24 children identified as being at risk for the development of a MH reaction were anesthetized for otolaryngic procedures by using "non-triggering" anesthetics and without use of dantrolene sodium. These patients represent 56% of all patients at risk for MH or masseter muscle rigidity (MMR) reactions during an 8-year period at the CNMC. There were no complications. Concomitant muscle biopsies were performed, and caffeine/halothane contracture studies were completed in 18 of these patients, demonstrating 11 susceptible or equivocal responses. The data suggest that children undergoing common otolaryngic procedures who are at risk for development of MH may be safely anesthetized without the use of prophylactic dantrolene sodium.
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PMID:Management of otolaryngic patients susceptible to malignant hyperthermia without dantrolene. 175 51

Malignant hyperthermia is a threat to the life of the surgical patient. It is a pharmacogenic disease that is brought on by contact with certain drugs and is manifest by a hypermetabolic crisis with tachycardia, ventricular ectopy, metabolic acidosis, and a rapid rise in body temperature. Muscle rigidity may or may not be present. Thanks to a reliable porcine animal model of malignant hyperthermia, dantrolene sodium has been found to be effective in the prevention and treatment of malignant hyperthermia. In this article a case report of malignant hyperthermia occurring in an office surgery suite is presented. The patient was 37 years old and underwent a routine septorhinoplasty under general anesthesia. The operation was complicated by ventricular ectopy, rapid rise in body temperature, and muscle rigidity at the end of the case. The malignant hyperthermia aborted spontaneously after 30 minutes; dantrolene was not given.
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PMID:Malignant hyperthermia in an office surgery suite: a case report. 274 52

Stiff-man syndrome is a rare disorder of the central nervous system consisting of progressive, fluctuating muscle rigidity with painful spasms. It is occasionally associated with endocrine disorders, including insulin-dependent diabetes, and with epilepsy. We investigated the possible existence of autoimmunity against the nervous system in a patient with stiff-man syndrome associated with epilepsy and Type I diabetes mellitus. Levels of IgG, which had an oligoclonal pattern, were elevated in the cerebrospinal fluid. The serum and the cerebrospinal fluid produced an identical, intense staining of all gray-matter regions when used to stain brain sections according to an indirect light-microscopical immunocytochemical procedure. The staining patterns were identical to those produced by antibodies to glutamic acid decarboxylase (the enzyme responsible for the synthesis of gamma-aminobutyric acid). A band comigrating with glutamic acid decarboxylase in sodium dodecyl sulfate-polyacrylamide gels appeared to be the only nervous-tissue antigen recognized by cerebrospinal fluid antibodies, and the predominant antigen recognized by serum antibodies. These findings support the idea that an impairment of neuronal pathways that operate through gamma-aminobutyric acid is involved in the pathogenesis of stiff-man syndrome, and they raise the possibility of an autoimmune pathogenesis.
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PMID:Autoantibodies to glutamic acid decarboxylase in a patient with stiff-man syndrome, epilepsy, and type I diabetes mellitus. 328 Oct 11

Small length changes were imposed on pairs of sartorius muscles from Rana temporaria and Rana pipiens in rigor and the mechanical and thermal responses studied. Rigor was induced by soaking the muscles overnight at 0 degrees C in a physiological salt solution containing 1.5 mM sodium azide and 0.4 mM sodium iodoacetate. Tension was measured at both the tibial and the pelvic ends of the preparation. Muscles were held at a steady tension of 20 to 76 kN m-2 and stretches or releases of 0.02 to 0.6 mm applied in pairs, with the initial change reversed several hundred milliseconds later. Single stretches resulted in heat absorption and releases in heat production by the preparation. Net heat production resulted from complete cycles of length changes larger than 0.1 mm, whether the initial change was a stretch or a release. The heat produced by the complete cycle was attributed to the movement of the muscles over the thermopile. It was proportional to the difference in tension between the tibial and pelvic ends of the preparation and increased with the size and speed of the length change. Half the heat produced by a complete cycle of length changes was subtracted from the thermal response recorded in the first half-cycle to obtain the reversible component of the response. The reversible component was linearly related to the tension change for all sizes and speeds of length change which were studied, with the heat:tension ratio ranging from -0.0093 to -0.0179 in eleven muscles (mean -0.0128 +/- 0.0009). The constancy of the heat:tension ratio in rigor muscles over a wide range of mechanical conditions indicates that the source of the thermal changes is the normal elasticity of the preparation. Since the size of the ratio is approximately the same as that measured in active muscles, the tension-dependent component of the thermal response to length changes applied to active muscles is probably also of elastic origin.
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PMID:The thermoelastic effect in rigor muscle of the frog. 348 55

Anesthetic-induced malignant hyperthermia in pigs and humans is characterized by muscle rigidity and rapid, often fatal, increases in body temperature. A defect in Ca2+ homeostasis has been suspected as underlying the disease, based on the preventive effect of dantrolene sodium, an agent thought to reduce Ca2+ levels in the cytoplasm. We describe here direct measurements of cytoplasmic ionized Ca2+ levels in lymphocytes from seven normal and 12 malignant hyperthermia-susceptible pigs, using the fluorescent indicator quin2. No differences in the concentration of cytoplasmic ionized Ca2+ were found in cells from malignant hyperthermia-susceptible pigs (160 +/- 10 nM) relative to the controls (150 +/- 10 nM). However, addition of halothane in vitro caused a significant increase (to 270 +/- 30 nM) in lymphocytes from malignant hyperthermia-susceptible pigs, but not from normal pigs (180 +/- 10 nM). The halothane-mediated increase in cytoplasmic ionized Ca2+ required extracellular Ca2+. It is suggested that general anesthetics such as halothane increase the permeability of the cell surface to Ca2+, and that this increase may, on its own or indirectly, increase the cytoplasmic level of ionized Ca2+ during a malignant hyperthermia crisis. The detection of a halothane-dependent increase in cytoplasmic ionized Ca2+ selectively in malignant hyperthermia-susceptible pigs could be the basis for a noninvasive test for malignant hyperthermia.
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PMID:Selective increase in cytoplasmic calcium by anesthetic in lymphocytes from malignant hyperthermia-susceptible pigs. 357 44


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