Dulac Olivier, Milh Mathieu, Holmes Gregory L
Department of Pediatric Neurology, Hôpital Necker-Enfants Malades, UMR663, Paris, France.
Handb Clin Neurol. 2013;111:441-6. doi: 10.1016/B978-0-444-52891-9.00047-6.
At full term, both glutamate and gamma-amino-butyric acid (GABA) are excitatory; cortical synapses are beginning to appear, there is little myelin in the cerebral hemispheres, and long tracts hardly start to develop. Neonatal myoclonic encephalopathy can result from premature activation of N-methyl-D-aspartate (NMDA) transmission. Benign neonatal seizures and migrating partial seizures in infancy could involve excessive or premature excitability of deep cortical layers. Benign rolandic epilepsy and continuous spike waves in slow sleep are consistent with an excess of both excitatory and inhibitory cortical synapses. West and Lennox-Gastaut syndromes express age-related diffuse cortical hyperexcitability, the pattern depending on the age of occurrence; synchronization of spikes is becoming possible with maturation of the myelin. Idiopathic generalized epilepsy is itself modulated by maturation that causes frontal hyperexcitability generating myoclonic-astatic seizures, between the ages of infantile and juvenile myoclonic epilepsies. Physiological delay of hippocampo-neocortical pathways maturation could account for the delayed occurrence of mesial temporal epilepsy following infantile damage, whereas premature maturation could contribute to fronto-temporal damage characteristic of fever-induced epileptic encephalopathy in school-age children, a dramatic school-age epileptic encephalopathy.
足月时，谷氨酸和γ-氨基丁酸（GABA）均具有兴奋性；皮质突触开始出现，大脑半球几乎没有髓鞘，长束几乎尚未开始发育。新生儿肌阵挛性脑病可由N-甲基-D-天冬氨酸（NMDA）传递的过早激活引起。良性新生儿惊厥和婴儿期游走性部分性惊厥可能涉及深层皮质层过度或过早的兴奋性。良性罗兰多癫痫和慢波睡眠期持续性棘波与皮质兴奋性和抑制性突触均过多一致。韦斯特和伦诺克斯-加斯东综合征表现出与年龄相关的弥漫性皮质兴奋性过高，其模式取决于发病年龄；随着髓鞘成熟，棘波同步化成为可能。特发性全身性癫痫本身受成熟过程调节，成熟过程导致额叶兴奋性过高，在婴儿肌阵挛性癫痫和青少年肌阵挛性癫痫之间引发肌阵挛-无动性发作。海马-新皮质通路成熟的生理性延迟可解释婴儿期损伤后内侧颞叶癫痫的延迟发生，而早熟可能导致学龄儿童发热性癫痫性脑病（一种严重的学龄期癫痫性脑病）特有的额颞叶损伤。
