RT Book, Section A1 Wolf, Nicole I. A1 Surtees, Robert A2 Duchowny, Michael A2 Cross, J. Helen A2 Arzimanoglou, Alexis SR Print(0) ID 1138410145 T1 Errors of Metabolism in the Neonatal Period T2 Pediatric Epilepsy YR 2017 FD 2017 PB McGraw-Hill Education PP New York, NY SN 9780071496216 LK accesspediatrics.mhmedical.com/content.aspx?aid=1138410145 RD 2024/03/28 AB Seizures in the neonatal period are a frequent and ominous sign of a multitude of conditions. Quick and effective treatment is important to prevent late sequelae in cognitive development; treatment efficacy however depends on the underlying cause. It is also hampered by the sometimes-difficult recognition of seizures in the setting of a neonatal intensive care unit in often critically ill children, as neonatal seizures might be very subtle in their manifestation. Simultaneous video EEG studies are at times necessary to correctly identify all seizure events. They may present as focal clonic seizures, oral automatisms, grimacing, complex movements like pedaling, myoclonic seizures or tonic seizures, and rarely as apnea. The most important cause of neonatal seizures is birth asphyxia. Inborn errors of metabolism as a group are also important, and prompt recognition of the few treatable disorders is crucial for preserving the potential of normal development. In this chapter, we would like to present the inborn errors of metabolism presenting with neonatal seizures (see also Tables 15–1 and 15–2) and focus on treatable disorders, offering algorithms for treatment and the diagnostic approach.