ICD·index Chapters ENESDE
8A61.00 category

Pyridoxal dependent epilepsy

Definition

Pyridoxal 5-phosphate dependent epilepsy usually presents with neonatal intractable seizures and is diagnosed by cerebrospinal fluid (CSF) analysis, gene testing, and clinical response. The majority of patients have pyridoxamine 5'-phosphate oxidase (PNPO) gene disease causing mutations. Early diagnosis and effective treatment can lead to a relatively favourable neurodevelopmental outcome.

Also indexed as

Pyridoxal dependent epilepsy

Nearby in Genetic epileptic syndromes with neonatal onset