Antimyoclonic effect of levetiracetam in MERRF syndrome

Michelangelo Mancuso; Renato Galli; Chiara Pizzanelli; Massimiliano Filosto; Gabriele Siciliano; Luigi Murri

doi:10.1016/j.jns.2005.11.021

Antimyoclonic effect of levetiracetam in MERRF syndrome

J Neurol Sci. 2006 Apr 15;243(1-2):97-9. doi: 10.1016/j.jns.2005.11.021. Epub 2006 Jan 18.

Authors

Michelangelo Mancuso¹, Renato Galli, Chiara Pizzanelli, Massimiliano Filosto, Gabriele Siciliano, Luigi Murri

Affiliation

¹ Neurophysiopathology Unit, Hospital of Pontedera, Italy. mmancuso@inwind.it

PMID: 16414077
DOI: 10.1016/j.jns.2005.11.021

Abstract

The treatment of progressive myoclonic epilepsy (PME) is largely empirical, even though valproic acid (VPA) is usually considered the drug of first choice. However, VPA should be used with caution in PME due to mitochondrial dysfunction, i.e. in MERRF (myoclonic epilepsy with ragged red fibers) syndrome, because of its interaction with mitochondrial respiration and metabolism. Levetiracetam (LEV) treatment was started in combination with VPA in a patient with typical clinical, histological, and biochemical features of MERRF due to a mutation on the tRNA of Phenilalanine gene. The average myoclonus score improved dramatically, as well as the quality of life and no side effects were observed, even after having withdrawn VPA. LEV may benefit myoclonus in PME of mitochondrial origin without altering mitochondrial function, and it could be considered the drug of first choice for the treatment of myoclonus in MERRF.

Publication types

Case Reports

MeSH terms

Activities of Daily Living
Anticonvulsants / pharmacology*
Anticonvulsants / therapeutic use
Atrophy / genetics
Atrophy / metabolism
Atrophy / pathology
Brain / metabolism
Brain / pathology
Brain / physiopathology
Drug Therapy, Combination
Female
Genetic Predisposition to Disease / genetics
Humans
Levetiracetam
MERRF Syndrome / drug therapy*
MERRF Syndrome / genetics
MERRF Syndrome / physiopathology
Middle Aged
Mitochondria / drug effects
Mitochondria / genetics
Mitochondria / metabolism
Muscle, Skeletal / metabolism
Muscle, Skeletal / pathology
Muscle, Skeletal / physiopathology
Mutation / genetics
Myoclonus / drug therapy*
Myoclonus / genetics
Myoclonus / physiopathology
Phenylalanine / genetics
Phenylalanine / metabolism
Piracetam / analogs & derivatives*
Piracetam / pharmacology
Piracetam / therapeutic use
Quality of Life
RNA, Transfer / genetics
Treatment Outcome
Ubiquinone / therapeutic use
Valproic Acid / adverse effects

Substances

Anticonvulsants
Ubiquinone
Levetiracetam
Phenylalanine
Valproic Acid
RNA, Transfer
Piracetam