Abstract

BackgroundAlthough seizures in neonates are common and often due to acute brain injury, 10-15% are unprovoked from congenital brain malformations. A better understanding of the risk of neonatal-onset epilepsy by the type of brain malformation is essential for counseling and monitoring. MethodsIn this retrospective cohort study, we evaluated 132 neonates with congenital brain malformations and their risk of neonatal-onset epilepsy. Malformations were classified into one of five categories based on imaging patterns on prenatal or postnatal imaging. Infants were monitored with continuous video EEG (cEEG) for encephalopathy and paroxysmal events in addition to abnormal neuroimaging. ResultsSeventy-four of 132 (56%) neonates underwent EEG monitoring, and 18 of 132 (14%) were diagnosed with neonatal-onset epilepsy. The highest prevalence of epilepsy was in neonates with disorders of neuronal migration/organization (9/34, 26%; 95% confidence interval [CI] = 13-44%), followed by disorders of early prosencephalic development (6/38, 16%; 95% CI = 6-31%), complex total brain malformations (2/16, 13%; 95% CI = 2-38%), and disorders of midbrain/hindbrain malformations (1/30, 3%; 95% CI = 0-17%). Of neonates with epilepsy, 5 of 18 (28%) had only electrographic seizures, 13 of 18 (72%) required treatment with two or more antiseizure medicines (ASMs), and 7 of 18 (39%) died within the neonatal period. ConclusionOur results demonstrate that disorders of neuronal migration/organization represent the highest-risk group for early-onset epilepsy. Seizures are frequently electrographic only, require treatment with multiple ASMs, and portend a high mortality rate. These results support American Clinical Neurophysiology Society recommendations for EEG monitoring during the neonatal period for infants with congenital brain malformations.

Highlights

  • Seizures in neonates are common with an incidence of 1-3.5/1000,1 the risk of neonatal-onset epilepsy related to particular brain malformations is not well understood

  • Most neonatal seizures are caused by hypoxic ischemic encephalopathy or other acute brain injuries; approximately 10-15% are unprovoked seizures due to early-onset epilepsy caused a by a genetic epileptic encephalopathy or congenital brain malformation.[2,3]

  • Mortality was significantly higher for neonates diagnosed with epilepsy (7/18 [39%]) than for children who did not have seizures in the neonatal period (13/114 [11%], P 1⁄4 0.003)

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Summary

Introduction

Seizures in neonates are common with an incidence of 1-3.5/1000,1 the risk of neonatal-onset epilepsy related to particular brain malformations is not well understood. Epileptogenesis in the setting of congenital brain malformations has been explained as an abnormality in the excitatory-to-inhibitory synaptic ratio, which can arise from multiple mechanisms including disrupted cell division, neuronal migration, development of the synaptic bouton, and dendritic morphogenesis.[5,6,7] A better understanding of the relationship between risk of neonatal-onset epilepsy and type of brain malformation is essential for prenatal counseling and clinical decision-making regarding postnatal electroencephalogram (EEG) monitoring. Seizures are frequently electrographic only, require treatment with multiple ASMs, and portend a high mortality rate These results support American Clinical Neurophysiology Society recommendations for EEG monitoring during the neonatal period for infants with congenital brain malformations.

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