Antiepileptic treatment and blood lactate level alteration in patients with myoclonic epilepsy with ragged-red fibers (MERRF) syndrome in a Chinese family
- Author:
Fei Xiao
;
Jia Li
;
Xiaogang Zhang
;
Xuefeng Wang
- Publication Type:Journal Article
- From:Neurology Asia
2013;18(1):47-51
- CountryMalaysia
- Language:English
-
Abstract:
Background: Myoclonic epilepsy with ragged-red fi bers (MERRF) is a type of mitochondrial
encephalomyopathy, clinical experience with the antiepileptic treatment for myoclonus in MERRF is
still limited. Myoclonus appears to be intractable, and some antiepileptic drugs may change the blood
lactate level. Objective: In this study, we report on two patients, a girl and her mother, both with
MERRF in a Chinese family. We aimed to study their myoclonus attack, response to AEDs and blood
lactate level. Methods: The diagnosis was based on muscle biopsies and a genetic test. We recorded
their myoclonus and detected alterations of blood lactate when the patients received antiepileptic
drugs. Results: The patients displayed substantial differences in their responses to antiepileptic drugs.
The mother exhibited a good response to valproic acid, although valproic acid is not recommended
for mitochondrial disease; however, her daughter was refractory to many antiepileptic drugs until
she received a combination treatment of levetiracetam and topiramate. We did not fi nd valproic acid,
levetiracetam or topiramate affected the blood lactate levels.
Conclusion: These fi ndings imply that not all MERRF patients are resistant to antiepileptic drugs, and
for those who are intractable, combination treatment involving levetiracetam and topiramate may be
effective for treating myoclonus in MERRF and does not worsen lactic acidosis.
- Full text:P020150703589053608479.pdf
