Literature DB >> 2248848

Are myotonias and periodic paralyses associated with susceptibility to malignant hyperthermia?

F Lehmann-Horn1, P A Iaizzo.   

Abstract

Excised muscles from patients with myotonia or periodic paralysis were subjected to the in vitro contracture test for susceptibility to malignant hyperthermia (MH). In a group of 44 patients, this standard test gave four positive, 10 equivocal and 30 negative results. The results for 27 control muscles from normal subjects were negative. When the test was performed with less than normal concentrations of contracture-triggering substances (caffeine less than or equal to 2 mmol litre-1, less than or equal to 2% halothane), 70% of the muscles from the patients and only 15% of the controls responded with small contractures (less than 0.2 g). These results should not be taken to indicate that the patients have the genetic trait for MH. The positive and equivocal test results, in addition to the slight contractures, may be accounted for by the electrical after-activity in the cases of pure myotonia, and by increased resting myoplasmic [Ca2+] in myotonic dystrophy. This shows that the in vitro contracture test lacks specificity.

Entities:  

Mesh:

Substances:

Year:  1990        PMID: 2248848     DOI: 10.1093/bja/65.5.692

Source DB:  PubMed          Journal:  Br J Anaesth        ISSN: 0007-0912            Impact factor:   9.166


  15 in total

Review 1.  Muscle channelopathies and critical points in functional and genetic studies.

Authors:  Karin Jurkat-Rott; Frank Lehmann-Horn
Journal:  J Clin Invest       Date:  2005-08       Impact factor: 14.808

2.  Evidence for genetic heterogeneity of malignant hyperthermia susceptibility.

Authors:  T Deufel; A Golla; D Iles; A Meindl; T Meitinger; D Schindelhauer; A DeVries; D Pongratz; D H MacLennan; K J Johnson
Journal:  Am J Hum Genet       Date:  1992-06       Impact factor: 11.025

3.  Genetic epidemiology of malignant hyperthermia in the UK.

Authors:  D M Miller; C Daly; E M Aboelsaod; L Gardner; S J Hobson; K Riasat; S Shepherd; R L Robinson; J G Bilmen; P K Gupta; M-A Shaw; P M Hopkins
Journal:  Br J Anaesth       Date:  2018-08-17       Impact factor: 9.166

4.  Malignant-hyperthermia susceptibility is associated with a mutation of the alpha 1-subunit of the human dihydropyridine-sensitive L-type voltage-dependent calcium-channel receptor in skeletal muscle.

Authors:  N Monnier; V Procaccio; P Stieglitz; J Lunardi
Journal:  Am J Hum Genet       Date:  1997-06       Impact factor: 11.025

5.  Paramyotonia and MH.

Authors:  G C Allen
Journal:  Can J Anaesth       Date:  1993-06       Impact factor: 5.063

Review 6.  Adverse effects of depolarising neuromuscular blocking agents. Incidence, prevention and management.

Authors:  W J Book; M Abel; J B Eisenkraft
Journal:  Drug Saf       Date:  1994-05       Impact factor: 5.606

Review 7.  Malignant Hyperthermia in the Post-Genomics Era: New Perspectives on an Old Concept.

Authors:  Sheila Riazi; Natalia Kraeva; Philip M Hopkins
Journal:  Anesthesiology       Date:  2018-01       Impact factor: 7.892

8.  Anaesthetic management of a patient with hypokalemic periodic paralysis- a case report.

Authors:  S Chitra; Grace Korula
Journal:  Indian J Anaesth       Date:  2009-04

9.  Malignant hyperthermia susceptibility in a patient with mitochondrial disorder.

Authors:  Josef Finsterer; Andrea Michalek-Sauberer; Romana Höftberger
Journal:  Metab Brain Dis       Date:  2009-09       Impact factor: 3.584

Review 10.  Hypokalemic periodic paralysis: a model for a clinical and research approach to a rare disorder.

Authors:  Bertrand Fontaine; Emmanuel Fournier; Damien Sternberg; Savine Vicart; Nacira Tabti
Journal:  Neurotherapeutics       Date:  2007-04       Impact factor: 7.620

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.