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Seizure. 2012 Jun;21(5):316-21. doi: 10.1016/j.seizure.2012.03.003. Epub 2012 Mar 27.

Epilepsy in mitochondrial disorders.

Author information

1
Danube University Krems, Krems, Austria. fifigs1@yahoo.de

Abstract

OBJECTIVES:

Information about epilepsy in mitochondrial disorders is scarce although a number or syndromic and non-syndromic mitochondrial disorders frequently manifest with focal or generalized seizures. Aim of the review was to describe epilepsy in syndromic and non-syndromic mitochondrial disorders with epilepsy as a dominant or collateral feature of the phenotype.

METHODS:

Literature search via Pubmed using the key words "mitochondrial", "epilepsy", "seizures", and all acronyms of syndromic mitochondrial disorders.

RESULTS:

Syndromic mitochondrial disorders obligatory associated with epilepsy include Alpers-Huttenlocher-syndrome (AHS), ataxia neuropathy spectrum (ANS), Leigh-syndrome, MELAS-syndrome, myoclonic epilepsy, myopathy, and sensory ataxia (MEMSA) syndrome, and MERRF-syndrome, Occasionally, epilepsy is a phenotypic feature in IOSCA, KSS, LHON, LBSL, or NARP, All types of seizures occur but most frequently generalized tonic-clonic seizures, partial seizures, myoclonic jerks, or West-syndrome was reported. Treatment of epilepsy in patients with mitochondrial disorders is not at variance from epilepsy of other causes but mitochondrion-toxicity of various antiepileptic drugs, such as valproic acid, carbamazepine etc. has to be considered to avoid severe complications or deterioration of the underlying disease.

CONCLUSIONS:

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.

PMID:
22459315
DOI:
10.1016/j.seizure.2012.03.003
[Indexed for MEDLINE]
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