Valproic acid aggravates epilepsy due to MELAS in a patient with an A3243G mutation of mitochondrial DNA.

Epilepsy is one of the most common presentations of patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). MELAS is typically caused by an A-to-G substitution at nucleotide position 3243 of mitochondrial DNA. Valproic acid, a common anticonvulsant, can actually increase the frequency of seizures in individuals with MELAS. Here, we report a single case-study of a 38-year-old man who presented with focal seizures and had MELAS Syndrome due to the A3243G mitochondrial DNA mutation. Manifestation of epilepsia partialis continua was aggravated by use of valproic acid. Convulsions abated after discontinuation of valproic acid. Our experience suggests that valproic acid should be avoided for the treatment of epilepsy in individuals with mitochondrial disease.
AuthorsChih-Ming Lin, Peterus Thajeb
JournalMetabolic brain disease (Metab Brain Dis) Vol. 22 Issue 1 Pg. 105-9 (Mar 2007) ISSN: 0885-7490 [Print] United States
PMID17226098 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Anticonvulsants
  • DNA, Mitochondrial
  • Valproic Acid
  • Adult
  • Anticonvulsants (adverse effects)
  • DNA, Mitochondrial (genetics)
  • Electroencephalography
  • Epilepsy (diagnosis, drug therapy, etiology)
  • Humans
  • MELAS Syndrome (complications, genetics)
  • Magnetic Resonance Imaging
  • Male
  • Valproic Acid (adverse effects)

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