کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
5628694 | 1579887 | 2017 | 7 صفحه PDF | دانلود رایگان |
- First-line treatment of mt epilepsy relies on low mitochondrion-toxic AEDS (LTG, LEV, GBT, ZNS, CBD).
- If these AEDs are ineffective, AEDs with higher mitochondrion-toxic potential (VPA, CBZ, PHT, PB, RGB) must be tried.
- VPA should be avoided in MERRF and patients carrying POLG1 mutations.
- If seizures are associated with stroke-like episodes, add l-arginine or l-citrulline.
ObjectivesAntiepileptic drugs (AEDs) exhibit adverse and beneficial effects on mitochondria, which have a strong impact on the treatment of patients with a mitochondrial disorder (MID) with epilepsy (mitochondrial epilepsy). This review aims at summarizing and discussing recent findings concerning the effect of AEDs on mitochondrial functions and the clinical consequences with regard to therapy of mitochondrial epilepsy and of MIDs in general.MethodsLiterature review.ResultsAEDs may interfere with the respiratory chain, with non-respiratory chain enzymes, carrier proteins, or mitochondrial biogenesis, with carrier proteins, membrane-bound channels or receptors and the membrane potential, with anti-oxidative defense mechanisms, with morphology, dynamics and survival of mitochondria, and with the mtDNA. There are AEDs of which adverse effects outweigh beneficial effects, such as valproic acid, carbamazepine, phenytoin, or phenobarbital and there are AEDs in which beneficial effects dominate over mitochondrial toxic effects, such as lamotrigine, levetiracetam, gabapentin, or zonisamide. However, from most AEDs only little is known about their interference with mitochondria.ConclusionsMitochondrial epilepsy might be initially treated with AEDs with low mitochondrial toxic potential. Only in case mitochondrial epilepsy is refractory to these AEDs, AEDs with higher mitochondrial toxic potential might be tried. In patients carrying POLG1 mutations AEDs with high mitochondrial toxic potential are contraindicated.
Journal: Epilepsy Research - Volume 136, October 2017, Pages 5-11