Management of epilepsy in MERRF syndrome - RIIP - Réseau International des Instituts Pasteur Access content directly
Journal Articles Seizure - European Journal of Epilepsy Year : 2017

Management of epilepsy in MERRF syndrome


Myoclonic epilepsy with ragged-red fibers (MERRF) syndrome is a rare syndromic mitochondrial disorder (MID) with a broad phenotypic but narrow genotypic heterogeneity. One of the predominant phenotypic features in addition to myopathy is epilepsy. The most frequent seizure type in MERRF is generalised myoclonic seizure but also focal myoclonic, focal atonic, generalised tonic-clonic, generalised atonic, generalised myoclonic-atonic, typical absences, or tonic-clonic seizures of unknown onset have been reported. There are no guidelines available for the management of epilepsy in MERRF syndrome but several expert opinions and general recommendations for the treatment of mitochondrial epilepsy have been published. According to these recommendations the antiepileptic drugs (AEDs) of choice are levetiracetam, topiramate, zonisamide, piracetam, and benzodiazepines. Perampanel has not been applied in MERRF patients but is promising in non-mitochondrial myoclonic epilepsy. Mitochondrion-toxic agents, including mitochondrion-toxic AEDs, such as valproate, carbamazepine, phenytoin, and barbiturates, should be avoided as well as AEDs potentially enhancing the frequency of myoclonus, such as phenytoin, carbamazepine, lamotrigine, vigabatrin, tiagabine, gabapentin, pregabalin, and oxcarbazepine.

Dates and versions

pasteur-02010870 , version 1 (07-02-2019)



Josef Finsterer, Sinda Zarrouk-Mahjoub. Management of epilepsy in MERRF syndrome. Seizure - European Journal of Epilepsy, 2017, 50, pp.166-170. ⟨10.1016/j.seizure.2017.06.010⟩. ⟨pasteur-02010870⟩


17 View
0 Download



Gmail Facebook Twitter LinkedIn More