Mitochondrial-toxic AEDs may trigger or worsen an MID or may be even fatal in single cases. The AED with the most well-known mitochondrial toxicity is valproic acid (VPA), which has been known to exhibit a deleterious effect in patients with POLG1 mutations and patients with myoclonic epilepsy with ragged red fibers syndrome and VPA should only be applied in MIDs in case of a drug-resistant status epilepticus. AEDs other than VPA, which may affect the mitochondrial metabolism, include phenobarbital, carbamazepine, phenytoin, oxcarbazepine, ethosuximide, zonisamide, topiramate, gabapentin and vigabatrin. AEDs which interfere with mitochondrial function should be avoided whenever justifiable to the patient's well-being. Collateral beneficial effects of AEDs should also influence their choice in MIDs.

Epilepsy is a common phenotypic feature of syndromic as well as non-syndromic mitochondrial disorders. Treatment of epilepsy in mitochondrial disorders is not at variance from treatment of epilepsy due to other causes but mitochondrion-toxic drugs should be avoided.

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