Benign and severe early-life seizures: a round in the first year of life

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.

PRRT2 gene variant in a child with dysmorphic features, congenital microcephaly, and severe epileptic seizures: genotype-phenotype correlation?

“…At an early age, the PRRT2 mutations have been associated to benign familial infantile epilepsy, benign infantile epilepsy, and benign myoclonus of early infancy [2, 4, 5]. The clinical features of infants with benign infantile epilepsy are relativity typical, with seizures beginning in the first days or months of life and tending to disappear very early, usually within the first few years [10]. In these infants, their developmental stages and physical examination are within the normal range.…”

Etiology and Types of Seizures in Patients Presenting to a Tertiary Care Hospital in Karachi: A Cross-Sectional Study

“…Other causes include head injury, CNS infections and neurodegenerative disorders [7]. In children, the most common causes of seizures include high-grade fever, cerebral palsy, hypoxic-ischemic encephalopathy, infections, intracranial hemorrhage and hydrocephalus [8]. In our study, 58% of seizures were primary while 42% were secondary.…”

Report on advances for pediatricians in 2018: allergy, cardiology, critical care, endocrinology, hereditary metabolic diseases, gastroenterology, infectious diseases, neonatology, nutrition, respiratory tract disorders and surgery

“…Pavone et al provided an updated review of the conditions associated with seizures in the first year of life [142]. BFNE is a condition inherited as an autosomal dominant trait (mutation in the KCN gene, chromosome 20q13.33) and is characterized by seizures in the first days of life in otherwise healthy looking babies and are typically associated with a family history of neonatal seizures, neonatal prognosis is usually benign and the seizures tend to gradually disappear within the first months of life [143].…”