PedAM
Pediatric Disease Annotations & Medicines
| Disease | epilepsy |
| Phenotype | C0205710|alpers-huttenlocher syndrome |
| Sentences | 1 |
| PubMedID- 26273464 | Valproate is the most well-known mitochondrial toxic anticonvulsant, which is known to exhibit a deleterious effect in patients carrying mitochondrial dna polymerase gamma mutations (e.g., alpers-huttenlocher syndrome) and with myoclonic epilepsy with ragged red fibers syndrome 15. a common mechanism of convulsion in the patients with mitochondrial disease seems to involve cortical damage resulting from respiratory chain dysfunction 16. inhibition and decreased activity of mitochondrial complexes i and iv, inhibition of oxygen and consumption and adenosine triphosphate synthesis are known as the mitochondrial adverse effects associated with valproate 17. therefore, these complex factors could have affected negatively the control of convulsion in our case. |
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