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The goal of this paper is to describe epilepsy syndromes that begin in childhood (age 2-12 years). Additional syndromes that have a variable age at onset, including in childhood, are described in the paper on epilepsy syndromes with onset at a variable age. 1 The childhood onset syndromes can be broadly divided into three main groups: (1) self-limited focal epilepsies (SeLFEs); (2) generalized epilepsy syndromes, which are thought to have a genetic basis; and (3) developmental and/or epileptic encephalopathies (DEEs), which often have both focal and generalized seizures, including Lennox-Gastaut syndrome (LGS), developmental epileptic encephalopathy with spike-and-wave activation in sleep (DEE-SWAS), and epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS), or may have generalized seizures alone, such as epilepsy with myoclonic atonic seizures (EMAtS), or focal/multifocal seizures alone, such as hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) and febrile infection-related epilepsy syndrome (FIRES).Childhood is also the typical age of onset of childhood absence epilepsy (CAE); this syndrome is covered in a separate paper on the idiopathic generalized epilepsy (IGE) syndromes. 2

Mutations in PNPO are a known cause of neonatal onset seizures that are resistant to pyridoxine but responsive to pyridoxal phosphate (PLP). Mills et al. show that PNPO mutations can also cause neonatal onset seizures that respond to pyridoxine but worsen with PLP, as well as PLP-responsive infantile spasms.