Treatment strategy

Treatment with conventional seizure medications may reduce seizures initially, but will seldom produce complete remission as the disease progresses Those medications that are usually used for myoclonic seizures generally are more efficacious: valproic acid, lamotrigine, zonisamide, levetiracetam, and benzodiazepines However, medication efficacy tends to be patient specific, and no prospective studies have been performed In a small study of five patients with mitochondrial disease due to respiratory chain dysfunction, vagus nerve stimulation did not produce reduction in myoclonic seizure frequency This suggests that placement of the vagus nerve stimulator device be undertaken with caution

The addition of L-carnitine and coenzyme Q10 has been advocated by some to improve mitochondrial function. However, no prospective studies have been performed to support this assertion Standard pharmacologic therapy is used to treat other specific organ involvement, such as cardiac symptoms Currently, there is no treatment for the genetic defect

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