MERRF syndrome 

(acronym for Myoclonic Epilepsy with Ragged Red Fibers)

Mitochondrial cytopathy (mutation A8344G of the ARNtLys gene of the mitochondrial DNA) with clinical manifestations beginning in adolescence. Progressive myoclonic epilepsy with ragged red fibers at muscle biopsy. Progressive ataxia but very  mild myopathy. Occasionally, hypoventilation of central origin.


Anesthetic implications:

anesthetic management of a mitochondrial cytopathy (see this topic)


References : 

 Vilela H, Garcia-Fernandez J, Parodi E, Reinoso-Barbero F, Duran P, Gilsanz F. 

Anesthetic management of a patient with MERFF syndrome. 

Pediatr Anesth 2005 ; 15 : 77-9.


Updated: January 2019