Benign congenital myopathy
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Unknown incidence.
General and vague term used by French-speaking neurologists to name an unspecific muscle disease:
- present from birth: congenital
- non-progressive: benign
- poorly symptomatic: moderate hypotonia, significant fatigability
- without structural abnormalities at biopsy
- without biological abnormalities: CPK blood level within the limits of normal.
From an etiological point of view, it can be:
- a variant of the normal
- the mild phenotype of an usually more severe myopathy
- a congenital myopathy the histological signs of which were not yet present at the time of the biopsy
Anesthetic implications:
unknown risk of malignant hyperthermia or rhabdomyolysis induced by halogenated and / or succinylcholine. Use preferably total IV propofol-based anesthesia (or ketamine and/or dexmedetomidine, or locoregional anesthesia). The short-term use of a halogenated agent is probably safe.
References :
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Updated: June 2021