Incidence and Predictors of Later Epilepsy in Neonates with Encephalopathy: The Impact of Electrographic Seizures.

Objectives: To determine the incidence of later epilepsy in full-term infants with neonatal encephalopathy (NE) who undergo continuous electroencephalography (cEEG) monitoring in the neonatal period and to identify potential predictors of later epilepsy both in infants with and without electrographic neonatal seizures (ENS).

Methods: This was a retrospective observational study performed at Cork University Maternity Hospital, Cork, Ireland, between 2003 and 2019. All term infants with NE had a minimum of 2 h of cEEG monitoring in the neonatal period.

Synergistic Clinico-Radiological Assessment of Term Newborns Afflicted by Birth Asphyxia in a Tertiary Medical Center: A Prospective Observational Study.

Background and objectives Hypoxic-ischemic encephalopathy (HIE) remains a critical concern in newborns, with therapeutic hypothermia (TH) serving as a key treatment strategy. However, assessing long-term outcomes requires a comprehensive approach, as children may develop neuropsychological and learning challenges even in the absence of noticeable motor impairments. This study aims to explore the correlation between electroencephalography (EEG) and neuroimaging findings with the clinical severity of HIE.

Predictors of Death or Severe Impairment in Neonates With Hypoxic-Ischemic Encephalopathy.

Importance: Outcomes after hypoxic-ischemic encephalopathy (HIE) are variable. Predicting death or severe neurodevelopmental impairment (NDI) in affected neonates is crucial for guiding management and parent communication.

Objective: To predict death or severe NDI in neonates who receive hypothermia for HIE.

Novel compound heterozygous P4HTM variants in a girl with developmental and epileptic encephalopathy: First case report of P4HTM variant-associated epileptic encephalopathy.

Background: HIDEA syndrome (MIM: #618493) is a rare autosomal recessive disorder characterized by hypotonia, hypoventilation, intellectual disability, dysautonomia, epilepsy, and eye anomalies. We present the case of a Turkish female with developmental and epileptic encephalopathy, highlighting a novel compound heterozygous variation in the P4HTM gene.

Case Presentation: A 6-year and 11-month-old girl with early infantile epileptic encephalopathy and abnormal eye movements since the neonatal period has been presented to our clinic.

AAV9/SLC6A1 gene therapy rescues abnormal EEG patterns and cognitive behavioral deficiencies in Slc6a1-/- mice.

The SLC6A1 gene encodes the gamma-aminobutyric acid (GABA) transporter GAT-1, the deficiency of which is associated with infantile encephalopathy with intellectual disability. We designed two AAV9 vectors, with either the JeT or MeP promoter, and conducted preclinical gene therapy studies using heterozygous and homozygous Slc6a1 KO mice at different developmental ages and various routes of administration. Neonatal intracerebroventricular administration of either vector resulted in significantly normalized EEG patterns in Slc6a1-/- or Slc6a1+/- mice, as well as improvement in several behavioral phenotypes of Slc6a1-/- mice.

Chorioamnionitis and Two-Year Outcomes in Infants with Hypoxic-Ischemic Encephalopathy.

Objective: To determine if chorioamnionitis is associated with an increased risk of adverse 2-year outcomes among infants with hypoxic-ischemic encephalopathy (HIE).

Study Design: This cohort study included all infants with moderate to severe HIE treated with therapeutic hypothermia and enrolled on the High-dose Erythropoietin for Asphyxia and Encephalopathy Trial. Clinical chorioamnionitis (CC) was defined as a diagnosis made by a treating obstetrician and histologic chorioamnionitis (HC) was defined as placental inflammation observed on histology.

Assessing Early Severity of Hypoxic-Ischemic Encephalopathy: The Role of Electroencephalogram Background in Addition to Sarnat Exam.

Objective: To assess the relationship between the Sarnat exam, early electroencephalogram (EEG) background, and death or neurodevelopmental impairment (NDI) at age 2 years among neonates with moderate to severe hypoxic-ischemic encephalopathy treated with therapeutic hypothermia.

Study Design: Neonates enrolled in the High-dose Erythropoietin for Asphyxia and Encephalopathy trial with EEG (n = 463) or amplitude-integrated electroencephalogram (n = 15) reports available on the first day after birth were included in this cohort study. A Sarnat exam was performed between 1 and 6 hours after birth, and neonates were classified into 3 groups of increasing severity based on the number of severe features (none, 1-2, or 3+).

A Systematic Review of EEG and MRI Features for Predicting Long-Term Neurological Outcomes in Cooled Neonates With Hypoxic-Ischemic Encephalopathy (HIE).

Neonatal hypoxic-ischemic encephalopathy (HIE) represents a significant global disease burden, but more importantly, it leaves a lasting impact of disability on individual children and their families. HIE outcome prognostication is important for guiding clinical interventions and counseling families. The objective of this study was to systematically review early electroencephalogram (EEG) and magnetic resonance imaging (MRI) features associated with long-term neurological outcomes in infants after perinatal HIE.

Altered sleep and inflammation are related to outcomes in neonatal encephalopathy.

Aim: Immune dysregulation and delayed onset of sleep wake cycling (SWC) are associated with worse outcome in neonatal encephalopathy (NE), however the association between sleep and immune dysfunction in NE remains unclear. Aimed to evaluate association of sleep and systemic inflammation with outcomes in NE.

Methods: Amplitude-integrated electroencephalography (aEEG) recordings were collected on infants undergoing therapeutic hypothermia (TH).

Adjuvant High-Dose Erythropoietin With Delayed Therapeutic Hypothermia in Neonatal Hypoxic-Ischemic Encephalopathy.

Background: To evaluate the benefits of high-dose erythropoietin (EPO) combined with therapeutic hypothermia (TH) on brain magnetic resonance imaging (MRI) scores and neurodevelopmental outcomes in neonates with moderate to severe hypoxic-ischemic-ecephalopathy (HIE), especially in neonates who received TH between six to 12 hours of birth.

Methods: This prospective, single-blind, randomized, placebo-controlled trial enrolled term newborns with moderate to severe HIE admitted to neonatal intensive care unit between April 2018 and April 2022. Hypothermia was started within 12 hours of birth.

Efficacy of Phenobarbital, Italept, and Topamax in the Treatment of Neonatal Seizures:A Double-Blinded Cross-Sectional Study of the Iranian Population.

Objectives: Seizures are changes in the electrical activity of the brain. These changes can cause significant or otherwise asymptomatic symptoms. Phenobarbital and phenytoin are known drugs for treating neonatal seizures, but little clinical experience exists using other drugs.

Clinical, developmental and serotonemia phenotyping of a sample of 70 Italian patients with Phelan-McDermid Syndrome.

Background: Phelan-McDermid syndrome (PMS) is caused by monoallelic loss or inactivation at the SHANK3 gene, located in human chr 22q13.33, and is often associated with Autism Spectrum Disorder (ASD).

Objectives: To assess the clinical and developmental phenotype in a novel sample of PMS patients, including for the first time auxometric trajectories and serotonin blood levels.

The Clinical Utility of Finding Unexpected Subclinical Spikes Detected by High-Density EEG During Neurodiagnostic Investigations.

This study aimed to analyze the frequency of unexpected subclinical spikes (USCS) in pediatric patients who underwent high-density electroencephalogram (HD-EEG). Of the 4481 successful HD-EEG studies, 18.5% (829) were abnormal, and 49.

Infant sleep spindle measures from EEG improve prediction of cerebral palsy.

Objective: Early identification of infants at risk of cerebral palsy (CP) enables interventions to optimize outcomes. Central sleep spindles reflect thalamocortical sensorimotor circuit function. We hypothesized that abnormal infant central spindle activity would predict later contralateral CP.

Persistent pulmonary hypertension and short-term neurological outcomes in infants with moderate to severe hypoxic ischemic encephalopathy.

Background: Our aim was to investigate the relationship between persistent pulmonary hypertension of the newborn (PPHN), short-term brain injury or death, and clinical parameters in infants with moderate to severe hypoxic ischemic encephalopathy (HIE).

Methods: Retrospective single-center cohort study of 274 HIE infants, 230 underwent Therapeutic Hypothermia (TH). Primary outcome was severe HIE brain injury on MRI and/or death within the first month of life in relation to presence and severity of PPHN (clinical or echocardiographic).

Natural history and outcome of nonketotic hyperglycinemia in China.

Introduction: Nonketotic hyperglycinemia (NKH) is a rare, life-threatening genetic disorder. The patients usually show heterogeneous and nonspecific symptoms, resulting in diagnosis challenges using conventional approaches. Here, the clinical presentation and genetic features of 20 Chinese patients were examined and reported in order to clarify the natural history and prognosis of NKH in China.

Early automated classification of neonatal hypoxic-ischemic encephalopathy - An aid to the decision to use therapeutic hypothermia.

Objective: The study aimed to address the challenge of early assessment of neonatal hypoxic-ischemic encephalopathy (HIE) severity to identify candidates for therapeutic hypothermia (TH). The objective was to develop an automated classification model for neonatal EEGs, enabling accurate HIE severity assessment 24/7.

Methods: EEGs recorded within 6 h of life after perinatal anoxia were visually graded into 3 severity groups (HIE French Classification) and quantified using 6 qEEG markers measuring amplitude, continuity and frequency content.

Examining Clinical Features and Severe Neurologic Disease of Parechovirus Infection in Young Infants: A Multistate Cohort Study.

Background: Human parechovirus (HPeV) infection can result in severe disease in infants, including sepsis, seizures, brain injury, and death. In 2022, a resurgence of HPeV was noted in young infants. The spectrum of illness and outcomes remain to be fully described.

Significant correlation between serum biomarkers and outcome in neonatal hypoxic-ischaemic encephalopathy.

Aim: Associations between serum biomarkers S100 calcium-binding protein B (S100B) and neuron-specific enolase (NSE) in neonates with hypoxic-ischaemic encephalopathy (HIE) offer contradicting data in regard to neurocognitive outcome. The aim of our study was to provide another dataset to answer this question if S100B or NSE correlates to outcome in neonatal HIE. In addition, we investigate whether amplitude-integrated EEG (aEEG) or magnetic resonance imaging (MRI) also has predictive value.

[Genotype and phenotype of WWOX gene related developmental and epileptic encephalopathy].

To summarize the genotype and clinical phenotype of children with WWOX gene related developmental and epileptic encephalopathy (DEE). Case series studies. The clinical data of 12 children with WWOX gene related DEE who were admitted to the Neurological Department of Children's Medical Center, Peking University First Hospital from June 2019 to December 2023 were analyzed.