Electrophysiologie clinique des canalopathies musculaires. [Electrophysiological testing in muscle channelopathies]

Détails

ID Serval
serval:BIB_3FA9782C3078
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Electrophysiologie clinique des canalopathies musculaires. [Electrophysiological testing in muscle channelopathies]
Périodique
Revue Neurologique
Auteur(s)
Kuntzer  T.
ISSN
0035-3787 (Print)
Statut éditorial
Publié
Date de publication
05/2004
Peer-reviewed
Oui
Volume
160
Numéro
5 Pt 2
Pages
S49-54
Notes
English Abstract Journal Article Review --- Old month value: May
Résumé
Electrodiagnostic testing should always be tailored to the clinical setting; in the muscle channelopathies, provocative tests are essential to demonstrate the modifications of the muscle excitability. When a permanent muscle weakness is present, a post-exercise (or post-tetanic) potentiation should be search to demonstrate a presynaptic neuromuscular channelopathy; when the weakness is fluctuating it is useful to perform repetitive nerve stimulation to see if a decrement is present or if the jitter is prolonged, indicative of a postsynaptic neuromuscular channelopathy; when the weakness is episodic, the prolonged exercise test is the only test to demonstrate the appearance of a late post-exercise decrement (sodium and calcium channelopathies), and when a myotonic reaction is seen to search the myotonic discharges by needle emg. The concomitant presence of myotonic discharges and abnormal decrement of the motor responses during repetitive nerve stimulation train depend on the type of mutations in the chloride gene and the amount of CTG repeats in myotonic dystrophy type 1. In paramyotonia congenita there a prolonged decrement when the tests are carried out with cooling the recorded muscle. Electrophysiological testings appear therefore useful to diagnose muscle channelopathies but also give information about the prognosis of the disorder; they could also be viewed as useful tests to predict response to treatments.
Mots-clé
Electrophysiology Humans Ion Channels/*physiology Membrane Potentials/physiology Muscle Contraction/physiology Muscle, Skeletal/physiopathology Muscular Diseases/*diagnosis/*physiopathology Myotonia/physiopathology
Pubmed
Web of science
Création de la notice
25/01/2008 13:43
Dernière modification de la notice
03/03/2018 16:25
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