Benign congenital myopathy

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