Highlights
Treatment of PNPO deficiency related neonatal status epilepticus is challenging.
Pyridoxine responsiveness is seen in huge number of cases of PNPO deficiency.
Various phenotypic variants in terms of response to pyridoxine are known in PNPO disorder making the treatment complex.
Immediate complete cessation of seizures or normalization of EEG on pyridoxine or pyridoxal 5 phosphate therapy is not necessary for the diagnosis, and treatment should be continued till the genetic tests are available.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.