Pyridoxal phosphate-responsive seizures

Orpha code: 79096OMIM code: 610090

Definition

A very rare neonatal epileptic encephalopathy disorder characterized clinically by onset of severe seizures within hours of birth that are not responsive to anticonvulsants, but are responsive to treatment with pyridoxal phosphate.

Disease data
Classification

Disease

Synonyms
PNPO deficiency
Napady zależne od fosforanu pirydoksalu
Niedobór 5'-oksydazy pirydoksaminy
Niedobór oksydazy 5'-fosforanu pirydoksyaminy
Niedobór PNPO
Noworodkowa encefalopatia epileptyczna związana z PNPO
PNPO-related neonatal epileptic encephalopathy
Pyridoxal phosphate-dependent seizures
Pyridoxamine 5'-oxidase deficiency
Pyridoxamine 5'-phosphate oxidase deficiency
ORPHA code
79096
OMIM code
610090
ICD10 code
G40.8
ICD11 code
8A61.00

No additional description.

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