Propofol reduces succinylcholine-induced muscle rigidity in a patient with paramyotonia congenita
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چکیده
منابع مشابه
A variety of paramyotonia congenita.
Paramyotonia congenita was first described by Eulenberg (1886). It is a rare, hereditary disorder of muscle in which severe loss of power is induced by degrees of cooling which are insufficient to affect normal muscle. Myotonia also appears on cooling. However, the paralysis produced by cooling distinguishes paramyotonia from Thomsen's disease. The weakness and myotonia can easily be produced u...
متن کاملMuscle stiffness and electrical activity in paramyotonia congenita.
To investigate the pathomechanism of paramyotonic stiffness, the mechanogram of isometric finger force and the electromyogram of the flexor digitorum muscle were simultaneously recorded in five unrelated paramyotonia congenita patients. Cooling of the forearm provoked "spontaneous" electrical activity, but the accompanying force was less than 5% of the maximal voluntary isometric contraction am...
متن کاملA defect in skeletal muscle sodium channel deactivation exacerbates hyperexcitability in human paramyotonia congenita.
1. Paramyotonia congenita (PC) is a human hereditary disorder wherein missense mutations in the skeletal muscle sodium channel lead to cold-exacerbated muscle hyperexcitability. The most common site for PC mutations is the outermost arginine of domain i.v. segment 4 (human R1448, rat R1441). 2. We examined the rat homologues of two PC mutants with changes at this site: R1441P and R1441C. The R-...
متن کاملParamyotonia congenita and skeletal sodium channelopathy.
Paramyotonia congenita, the major characteristics of which are cold-induced and exercise-induced myotonia, is an autosomal-dominant muscle disease which is classified into one of a group of muscle diseases, so-called muscle "sodium channelopathies" caused by missense mutations in the gene coding for the skeletal muscle sodium channel a-subunit (SCN4A) (1-4). Such muscle sodium channelopathies s...
متن کاملHypothermic cardiopulmonary bypass in paramyotonia congenita: a case report.
Paramyotonia congenita is a non-progressive disease characterised by myotonia (failure of muscle relaxation) induced by the exposure of the affected muscles to cold. Episodes of flaccid paralysis occur and may be related to hyperkalaemic periodic paralysis. Proximal myopathy or muscular hypertrophy may occur. The condition is inherited as an autosomal dominant.' Severe paralysis may occur with ...
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ژورنال
عنوان ژورنال: Anesthesia: Essays and Researches
سال: 2017
ISSN: 0259-1162
DOI: 10.4103/0259-1162.200242