Abstract

BackgroundAt the onset, differentiation between abnormal non-epileptic movements, and epileptic seizures presenting in early life is difficult as is clinical diagnosis and prognostic evaluation of the various seizure disorders presenting at this age. Seizures starting in the first year of life including the neonatal period might have a favorable course, such as in infants presenting with benign familial neonatal epilepsy, febrile seizures simplex or acute symptomatic seizures. However, in some cases, the onset of seizures at birth or in the first months of life have a dramatic evolution with severe cerebral impairment. Seizure disorders starting in early life include the “epileptic encephalopathies”, a group of conditions characterized by drug resistant seizures, delayed developmental skills, and intellective disability. This group of disorders includes early infantile epileptic encephalopathy also known as Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, infantile spasms syndrome (also known as West syndrome), severe myoclonic epilepsy in infancy (also known as Dravet syndrome) and, myoclonic encephalopathies in non-progressive disorder.Here we report on seizures manifesting in the first year of life including the neonatal period. Conditions with a benign course, and those with severe evolution are presented. At this early age, clinical identification of seizures, distinction of each of these disorders, type of treatment and prognosis is particularly challenging.The aim of this report is to present the clinical manifestations of each of these disorders and provide an updated review of the conditions associated with seizures in the first year of life.

Highlights

  • At the onset, differentiation between abnormal non-epileptic movements, and epileptic seizures presenting in early life is difficult as is clinical diagnosis and prognostic evaluation of the various seizure disorders presenting at this age

  • Seizure types have been recently classified according to their site of onset in focal, generalized and unknown

  • This report aims to present the clinical manifestation of each of these disorders and provide an updated review of the conditions associated with seizures in the first year of life

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Summary

Introduction

Differentiation between abnormal non-epileptic movements, and epileptic seizures presenting in early life is difficult as is clinical diagnosis and prognostic evaluation of the various seizure disorders presenting at this age. Seizure disorders starting in early life include the “epileptic encephalopathies”, a group of conditions characterized by drug resistant seizures, delayed developmental skills, and intellective disability. This group of disorders includes early infantile epileptic encephalopathy known as Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, infantile spasms syndrome ( known as West syndrome), severe myoclonic epilepsy in infancy ( known as Dravet syndrome) and, myoclonic encephalopathies in non-progressive disorder. Conditions with a benign course, and those with severe evolution are presented At this early age, clinical identification of seizures, distinction of each of these disorders, type of treatment and prognosis is challenging. The focal seizures are further distinguished in: aware or with impaired awareness, with motor or non-motor onset, and focal to bilateral tonic-clonic; the generalized, in Pavone et al Italian Journal of Pediatrics (2018) 44:54

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