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Parents are often appointed a passive role in the care for their hospitalised child. In the family-integrated care (FICare) model, parental involvement in neonatal care is emulated. Parental participation in medical rounds, or family-centred rounds (FCR), forms a key element. A paucity remains of randomised trials assessing the outcomes of FCR (embedded in FICare) in families and neonates, and outcomes on an organisational level are relatively unexplored. Likewise, biological mechanisms through which a potential effect may be exerted are lacking robust evidence. Ten level two Dutch neonatal wards are involved in this stepped-wedge cluster-randomised trial FCR (embedded in FICare) by one common implementation strategy. Parents of infants hospitalised for at least 7 days are eligible for inclusion. The primary outcome is parental stress (PSS:NICU) at discharge. Secondary outcomes include parental, neonatal, healthcare professional and organisational outcomes. Biomarkers of stress will be analysed in parent–infant dyads. With a practical approach and broad outcome set, this study aims to obtain evidence on the possible (mechanistic) effect of FCR (as part of FICare) on parents, infants, healthcare professionals and organisations. The practical approach provides (experiences of) FICare material adjusted to the Dutch setting, available for other hospitals after the study. [ABSTRACT FROM AUTHOR]

Background: Feeding supplemented mother milk during hospital stay improves neurodevelopment in preterm infants. Yet the composition of mother milk varies widely between subjects. The relationship between this variation and outcome is unknown., Objective: To determine whether the protein content in native breast milk (BM) correlates with 2-year infant outcome., Design: In a monocentric prospective observational study, LACTACOL, preterm infants born between 28 and 34 weeks of gestation, whose mothers decided to exclusively breastfeed, were enrolled during the first week of life. Samples of expressed breast milk obtained at several times of the day were pooled over a 24-h period, and such pool was used for macronutrient analysis, using mid-infrared analyzer. Age and Stages questionnaire (ASQ) was used to assess 2-year neurodevelopmental outcome. We analyzed the relationship between protein content in BM, and (i) infant neurodevelopment at 2-year (primary outcome), and (ii) growth until 2-year (secondary outcome)., Results: 138 infants were enrolled. The main analysis concerned 130 infants (including 40 twin infants) and 110 mothers with BM samples collected at week 3 after birth. Native BM samples were ranked in three tertiles of protein content (g/100 ml): 0.91 ± 0.09 (lower), 1.14 ± 0.05 (middle) and 1.40 ± 0.15 (upper); 48, 47 and 35 infants were ranked, respectively, in these three tertiles. Infants in the upper tertile were more often singleton (P = 0.012) and were born with lower birth weight and head circumference Z-scores (P = 0.005 and 0.002, respectively). Differences in weight and head circumference were no longer observed at 2-year. ASQ score at age 2 did not differ between the three tertiles (P = 0.780). Sensitivity analyses with imputations, including all 138 infants, confirmed the main analysis as well as analyses based on fortified BM as exposure., Conclusions: Protein content of BM (native or fortified) is not associated with preterm infant neurodevelopment at 2-year. Higher protein content was associated with a lower birth weight., (© 2021. Springer-Verlag GmbH Germany, part of Springer Nature.)

Variants in SCN2A are a known risk factor for developing autism spectrum disorder (ASD). Catatonia is a complex neuropsychiatric syndrome, which occurs at a higher rate in individuals with ASD. Catatonia has also been associated with COVID-19 infection, though the majority of these cases are associated with increased serum inflammatory markers. We present a case of a 15-year-old female with ASD and corticosteroid responsive stuporous catatonia to explore the relationship between SCN2A variants, ASD, COVID-19 exposure, and treatment refractory catatonia. Despite a lack of significantly elevated serum or CSF inflammatory markers, this patient showed significant improvement following initiation of corticosteroid therapy. This case presents a novel approach to the work-up and treatment of catatonia in individuals with SCN2A variants independent of elevated inflammatory markers., (© 2024 The Author(s). American Journal of Medical Genetics Part C: Seminars in Medical Genetics published by Wiley Periodicals LLC.)

Aim: This study compared neurodevelopmental screening questionnaires completed when preterm-born children reached 2 years of corrected age with social communication skills at 5.5 years of age., Methods: Eligible subjects were born in 2011 at 24-34 weeks of gestation, participated in a French population-based epidemiological study and were free of motor and sensory impairment at 2 years of corrected age. The Ages and Stages Questionnaire (ASQ) and the Modified Checklist for Autism in Toddlers (M-CHAT) were used at 2 years and the Social Communication Questionnaire (SCQ) at 5.5 years of age., Results: We focused on 2119 children. At 2 years of corrected age, the M-CHAT showed autistic traits in 20.7%, 18.5% and 18.2% of the children born at 24-26, 27-31 and 32-34 weeks of gestation, respectively (p = 0.7). At 5.5 years of age, 12.6%, 12.7% and 9.6% risked social communication difficulties, with an SCQ score ≥90th percentile (p = 0.2). A positive M-CHAT score at 2 years was associated with higher risks of social communication difficulties at 5.5 years of age (odds ratio 3.46, 95% confidence interval 2.04-5.86, p < 0.001). Stratifying ASQ scores produced similar results., Conclusion: Using parental neurodevelopmental screening questionnaires for preterm-born children helped to identify the risk of later social communication difficulties., (© 2024 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

Research Findings: This study investigated children's feelings about school and their adaptation to primary school after leaving kindergarten. Altogether, 28,334 Chinese children (ages 6–8 years) were sampled and surveyed online. The descriptive statistic, latent class analysis (LCA) approach, and logistic regression analysis indicated that: (1) children generally had positive feelings toward school, especially about teacher-student relationships. However, the self-perception of academic abilities was less positive; (2) children's social adaptation had the highest scores, followed by life and physical adaptation, and then academic adaptation; (3) there were significant differences between children from only child and non-only child families both in school feelings and adaptation; (4) three latent classes of children's school adaptation emerged: the Well-Adapted Children were girls, only children in eastern China; the Moderately-Adapted Children were boys, non-only children in central China; the Poorly-Adapted Children were boys, non-only children in western China; and (5) children's school feelings significantly predicted their class membership for adaptation. Practice or Policy: These findings suggest that we need to focus more on children's feelings and academic school adjustment issues. Targeted support is needed for children at risk of school adaptation to help them transition smoothly to primary school. [ABSTRACT FROM AUTHOR]

Unilateral spastic cerebral palsy (USCP) is caused by damage to the developing brain and affects motor function, mainly lateralized to one side of the body. Children with USCP have difficulties grasping objects, which can affect their ability to perform daily activities. Although cerebral palsy is typically classified according to motor function, sensory abnormalities are often present as well and may contribute to motor impairments, including grasping. In this review, we show that the integrity and connectivity pattern of the corticospinal tract (CST) is related to execution and anticipatory control of grasping. However, as this may not explain all the variance of impairments in grasping function, we also describe the potential roles of sensory and sensorimotor integration deficits that contribute to grasp impairments. We highlight studies measuring fingertip forces during object manipulation tasks, as this approach allows for the dissection of the close association of sensory and motor function and can detect the discriminant use of sensory information during a complex, functional task (i.e., grasping). In addition, we discuss the importance of examining the interactions of the sensory and motor systems together, rather than in isolation. Finally, we suggest future directions for research to understand the underlying mechanisms of grasp impairments. [ABSTRACT FROM AUTHOR]

A neonatal stroke is a cerebrovascular process caused by interruption of cerebral blood flow that occurs with an incidence between 1 per 1600 and 1 per 2660 live births. Relative higher incidence in the neonatal period compared to later childhood is favored by the hypercoagulability state of the mother, mechanical stress during delivery, transient right to left intracardiac shunt, high hematocrit, blood viscosity, and risk of dehydration during the first few days of life. The exact cause of a neonatal stroke remains unclear in many cases. About 80% of neonatal strokes are due to arterial ischemic events involving the middle cerebral artery. Typical clinical manifestations in a neonatal stroke are usually seizures that appear immediately after birth or after several days of life, but many of the cases may remain asymptomatic. We present the case of a late preterm infant diagnosed with a thalamic stroke on the fifth day of life with no clinical signs except for repeated episodes of apnea. The anamnesis and clinical context, in this case, revealed a SARS-CoV-2 infection in late pregnancy and early bacterial neonatal sepsis. Early identification of a perinatal stroke and increasing awareness of physicians about this condition in the neonatal period have paramount importance to reduce developmental postischemic damage. [ABSTRACT FROM AUTHOR]

Background: Many children born very low birth weight (VLBW) experience school struggles with preparedness requiring adequate physical, social, behavioural, cognitive and communication skills. A global assessment of proficiency is necessary to identify those at risk in any such area and direct early intervention accordingly. Study objectives were to characterize developmental vulnerability and school readiness scores in these key domains in a sample of children born VLBW versus their provincial public school system peers and identify early‐life infant and parent factors related to suboptimal school readiness. Methods: The Early Development Instrument teacher assessments of school readiness were collected for a Canadian VLBW sample (NCT02759809). Comparisons between children born VLBW and peers were made. Group differences between children born VLBW considered vulnerable (<10th percentile, not developmentally ready for learning) and not vulnerable were tested and linear regression explored associations between early‐life factors and domain scores. Results: Of 77 available Early Development Instrument assessments, median (interquartile range) assessment age was 6.0 (5.7, 6.2) years, birth weight 950 (793, 1250) grammes and birth gestation 27.4 (25.6, 29.7) weeks. A higher proportion of children born VLBW versus peers exhibited vulnerability in Physical Health and Well‐being (24.7% vs. 16.1%, p = 0.04), Communication Skills and General Knowledge (23.4% vs. 10.2%, p = 0.0001) and vulnerability in ≥2 domains (26.0% vs. 14.4%, p = 0.004). Children born VLBW classified as vulnerable versus not vulnerable had lower birth gestation and 5‐min Apgar. Adjusted regression models found Apgar <7 associated with lower scores for Physical Health and Well‐being (−0.86; 95%CI: −1.71, −0.00; p = 0.049), Social Competence (−1.77; 95%CI: −2.92, −0.62; p = 0.003), Emotional Maturity (−1.55; 95%CI: −2.43, −0.66; p = 0.0009) and Communication Skills and General Knowledge (−1.63; 95%CI: −3.19, −0.06; p = 0.04). Conclusions: This VLBW sample exhibited poor school readiness in multiple domains. Identification of lower birth gestation and Apgar may assist targeted early interventions to mitigate vulnerability. [ABSTRACT FROM AUTHOR]

Introduction: Preterm birth and cerebral hemorrhage have adverse effects on brain development. Alterations in regional brain size on magnetic resonance imaging (MRI) can be assessed using 2D biometrical analysis, an easily applicable technique showing good correlation with 3D brain volumes. Methods: This retrospective study included 74 preterm neonates with intraventricular hemorrhage (IVH) born <32+0 weeks of gestation between 2011 and 2019. Cerebral MRI was performed at term-equivalent age, and 2D measurement techniques were used for biometrical analysis and compared to normative data of two control groups. Finally, the correlation and association of brain parameters and patterns of impaired brain growth and outcome at 2 and 3 years of age were evaluated. Results: Interhemispheric distance (IHD), the 3rd ventricle, and lateral ventricles presented larger, in contrast, cerebral biparietal width (cBPW), fronto-occipital diameter (FOD), and the length of the corpus callosum were smaller in IVH patients compared to respective controls. The strongest correlations with outcome were observed for the parameters FOD, anteroposterior diameter of the vermis, transverse cerebellar diameter (tCD), corpus callosum, 3rd ventricle, and left ventricular index. Patients with the small FOD, small BPW, and increased IHD pattern reached overall lower outcome scores at follow-up. Discussion: Preterm neonates with IVH showed reduced total brain sizes and enlarged pericerebral spaces compared to neurologically healthy controls. Biometric analysis revealed that several 2D brain parameters as well as different patterns of impaired brain growth were associated with neurodevelopmental impairment in early childhood. These findings may support prediction of long-term outcome and parental counseling in patients with IVH. [ABSTRACT FROM AUTHOR]

Aim: The aetiology of perinatal stroke is poorly understood. This study aimed to prospectively confirm the risk factors and identify any previously unknown variables. Methods: A prospective case–control study was conducted in Australia. Univariate odds ratios (ORs), associated 95% confidence intervals (CIs) and multivariable logistic regression models fitted with backwards stepwise variable selection were used. Results: Sixty perinatal stroke cases reported between 2017 and 2019 included 95% (57/60) with multiple risk factors. Univariate analysis identified emergency caesarean section rather than NVD (P < 0.01), low Apgar score (<7) at 1, 5 and 10 min of age (P < 0.01), resuscitation at birth (P < 0.01), abnormal cord blood gas (P < 0.01), neonatal infection/sepsis (P < 0.01), congenital heart disease (P < 0.01) and hypoglycaemia (P < 0.01) as significant risk factors. Multivariate analysis found smoking during pregnancy (OR: 1.48; 95% CI: 1.09–1.99), 1‐min Apgar score < 7 (OR: 1.54; 95% CI: 1.15–2.08), 10‐min Apgar score < 7 (OR: 1.26; 95% CI: 1.02–1.54) and hypoglycaemia (OR: 1.49; 95% CI: 1.07–2.06). Conclusions: Perinatal stroke is associated with multiple risk factors. Exposure to smoking, 10‐min Apgar score < 7, neonatal infection and hypoglycaemia were independent risk factors. Emergency caesarean section, resuscitation at birth and abnormal cord blood gas were additional risk factors. [ABSTRACT FROM AUTHOR]

Background: The 3q29 microduplication syndrome is a rare genomic disorder characterized by an extremely variable neurodevelopmental phenotype usually involving a genomic region ranging from 1.6 to 1.76 Mb. A small microduplication of 448.8 Kb containing only two genes was recently described in a patient with a 3q29 microduplication that was proposed as the minimal critical region of overlap of this syndrome. Methods: Molecular karyotyping (array‐CGH) was performed on DNA extracted from peripheral blood samples using Agilent‐California USA Human Genome CGH Microarray 4 × 180 K. The proband and his younger brother were further tested with a next generation sequencing (NGS) panel including genes implicated in autism spectrum disorder and in neurodevelopmental disorders. Quantitative real‐time PCR was applied to verify the abnormal array‐CGH findings. Results: Here, we report on a family with two males with neurodevelopmental disorders and an unaffected sibling with a small 3q29 microduplication (432.8 Kb) inherited from an unaffected mother that involves only two genes: DGL1 and BDH1. The proband had an additional intragenic duplication inherited from the unaffected father. Further testing was negative for Fragile X syndrome and for genes implicated in autism spectrum disorder and in neurodevelopmental disorders. Conclusion: To the best of our knowledge, one of the family members here analyzed is the second reported case of a patient carrying a small 3q29 microduplication including only DGL1 and BDH1 genes and without any additional genetic aberration. The recognition of the clinical spectrum in patients with the critical region of overlap associated with the 3q29 duplication syndrome should prove valuable for predicting outcomes and providing more informed genetic counseling to patients with duplications in this region. [ABSTRACT FROM AUTHOR]

Comorbidities are common in children with epilepsy, with nearly half of the patients having at least one comorbidity. Attention deficit hyperactivity disorder (ADHD) is a psychiatric disorder characterized by hyperactivity and inattentiveness level disproportional to the child's developmental stage. The burden of ADHD in children with epilepsy is high and can adversely affect the patients' clinical outcomes, psychosocial aspects, and quality of life. Several hypotheses were proposed to explain the high burden of ADHD in childhood epilepsy; the well-established bidirectional connection and shared genetic/non-genetic factors between epilepsy and comorbid ADHD largely rule out the possibility of a chance in this association. Stimulants are effective in children with comorbid ADHD, and the current body of evidence supports their safety within the approved dose. Nonetheless, safety data should be further studied in randomized, double-blinded, placebo-controlled trials. Comorbid ADHD is still under-recognized in clinical practice. Early identification and management of comorbid ADHD are crucial to optimize the prognosis and reduce the risk of adverse long-term neurodevelopmental outcomes. The identification of the shared genetic background of epilepsy and ADHD can open the gate for tailoring treatment options for these patients through precision medicine. [ABSTRACT FROM AUTHOR]

Children in Africa are disproportionately burdened by the neurosurgical condition hydrocephalus. In Blantyre, Malawi, paediatric hydrocephalus represents the majority of surgical procedures performed in the neurosurgical department at Queen Elizabeth Central Hospital. To reduce morbidity and mortality, timely detection followed by referral from surrounding primary health centres is crucial. Aiming to explore perceptions and identify enablers and barriers to detection and referral, we conducted a qualitative study among primary healthcare providers (n = 30) from ten health centres in Blantyre district. Using a semi-structured interview-guide, we audio-recorded and transcribed the interviews before conducting a thematic analysis. One main finding is that there is a potential to improve detection through head circumference measurements, which is the recommended way to detect hydrocephalus early, yet healthcare providers did not carry this out systematically. They described the health passport provided by the Malawian Ministry of Health as an important tool for clinical communication. However, head circumference growth charts are not included. To optimise outcomes for paediatric hydrocephalus we suggest including head circumference growth charts in the health passports. To meet the need for comprehensive management of paediatric hydrocephalus, we recommend more research from the continent, focusing on bridging the gap between primary care and neurosurgery. [ABSTRACT FROM AUTHOR]

This research analyzed the temporal trend of stroke mortality in children aged 0–14 years, from 1990 to 2019, in Brazil and its federative units. This ecological study used data from the Global Burden of Disease, a study led by the Institute for Health Metrics and Evaluation. Stroke definition considered the International Classification of Diseases according to codes G45, G46, and I60–I69. Age-standardized mortality rates and the mean annual percentage change (APC) in mortality rates were estimated. Stroke mortality trends decreased, with an APC of − 3.9% (95% CI − 4.5; − 3.3; p < 0.001). Reducing trends were found in all but two states, where they were stationary. Maranhão (− 6.5%; 95% CI − 7.6; − 5.4; p < 0.001) had the greatest reduction and Rondônia, the smallest (− 1.2%; 95% CI − 2.3; − 0.1, p = 0.027). Decrease was more important in children < 5 (− 5.8%; 95% CI − 6.3; − 5.2; p < 0.001) compared to 5–14 years old (− 2.1%; 95% CI − 2.9; − 1.3; p < 0.001); additionally, it was greater in girls (− 4.1%; 95% CI − 4.6; − 3.5; p < 0.001) than in boys (− 3.8%; 95% IC − 4.5; − 3.1; p < 0.001). Ischemic stroke had the highest APC (− 6.1%; 95% CI − 6.8; − 5.3; p < 0.001), followed by intracranial hemorrhage (− 5.3%; 95% CI − 6.1; − 4.5; p < 0.001) and subarachnoid hemorrhage (− 2.7%; 95% CI − 3.3; − 2.1; p < 0.001). Largest reductions were seen in states with more vulnerable socioeconomic contexts. The stationary trends and lowest APCs were concentrated in the northern region, which had greater impact of diseases and less favorable outcomes. [ABSTRACT FROM AUTHOR]

Objective: Amplitude-integrated electroencephalography (aEEG) is a simplified bedside neurophysiology tool that has been implemented in the neonatal intensive care unit and studied in an extensive range of clinical applications in the past decade. This critical review aimed to evaluate a variety of clinical applications of aEEG monitoring in diagnosis, clinical management, and prognosis assessment in critically ill neonates. Sources: The databases of Pubmed, SciELO, Lilacs, and Cochrane, books, and other online resources were consulted, as well as sources of professional experiences. Summary of findings: The clinical use of aEEG to access real-time brain function, background activity, and utility in seizures detection has been described. A critical review was realized considering the authors' professional experience. Newborns with hypoxic-ischemic encephalopathy and seizures screening represent the most common studied population. However, several studies have shown interesting applications on preterm infants, newborns with congenital heart disease, and other clinical situations of high risk of injury to the developing brain. Conclusion: The aEEG has shown to be a useful non-invasive bedside monitor that aids in evaluating brain function, background activity, and cyclicity. aEEG findings have also demonstrated good prognostic value in a group of critically ill neonates. The aEEG seizure diagnosis capability has limitations, which have been already well established. The use of neonatal brain monitoring such as aEEG was shown to give valuable information in several high-risk clinical situations. [ABSTRACT FROM AUTHOR]

Intracranial volume (ICV) is frequently used in volumetric magnetic resonance imaging (MRI) studies, both as a covariate and as a variable of interest. Findings of associations between ICV and age have varied, potentially due to differences in ICV estimation methods. Here, we compared five commonly used ICV estimation methods and their associations with age. T1‐weighted cross‐sectional MRI data was included for 651 healthy individuals recruited through the NORMENT Centre (mean age = 46.1 years, range = 12.0–85.8 years) and 2410 healthy individuals recruited through the UK Biobank study (UKB, mean age = 63.2 years, range = 47.0–80.3 years), where longitudinal data was also available. ICV was estimated with FreeSurfer (eTIV and sbTIV), SPM12, CAT12, and FSL. We found overall high correlations across ICV estimation method, with the lowest observed correlations between FSL and eTIV (r =.87) and between FSL and CAT12 (r =.89). Widespread proportional bias was found, indicating that the agreement between methods varied as a function of head size. Body weight, age, sex, and mean ICV across methods explained the most variance in the differences between ICV estimation methods, indicating possible confounding for some estimation methods. We found both positive and negative cross‐sectional associations with age, depending on dataset and ICV estimation method. Longitudinal ICV reductions were found for all ICV estimation methods, with annual percentage change ranging from −0.293% to −0.416%. This convergence of longitudinal results across ICV estimation methods offers strong evidence for age‐related ICV reductions in mid‐ to late adulthood. [ABSTRACT FROM AUTHOR]

Myotonia congenita is a rare neuromuscular disorder caused by CLCN1 mutations resulting in delayedmuscle relaxation. Extramuscularmanifestations are not considered to be present in chloride skeletal channelopathies, although recently some cardiac manifestations have been described. We report a family with autosomal dominant myotonia congenita and Brugada syndrome. Bearing in mind the previously reported cases of cardiac arrhythmias in myotonia congenita patients, we discuss the possible involvement of the CLCN1-gene mutations in primary cardiac arrhythmia. [ABSTRACT FROM AUTHOR]

Objective: To determine the birth prevalence of perinatal stroke in term born infants at our high-volume delivery center and assess the frequency of both gross and histologic placental pathologies associated with perinatal stroke using the Amsterdam Placental Workshop Group Consensus Statement guidelines and definitions. Study Design: A single-center retrospective cohort study spanning 2010-2020. Results: There were 129,759 live births at Parkland Hospital during the study period and a total of 18 term born infants leading to a birth prevalence of 1 in 6,829 infants. Perinatal risk factors were found in all but one patient, and 74% presented with seizures. Pathologic placental examination was available in 56% of the cohort and only one patient had normal placental examination. Acute histologic chorioamnionitis was described in five placentas (50%) and an additional two had isolated umbilical and/or chorionic plate vasculitis with or without funisitis compared to a rate of 28% with acute inflammation in a Control group. Chronic inflammation in the form of villitis of unknown etiology was described in three of the acutely inflamed placentas and was high-grade in each of those while none of the placentas from our Control group showed evidence of any chronic lesion. Conclusion: Both acute and chronic placental inflammation are common in perinatal stroke; placental examination should be considered an essential component to the diagnostic workup. [ABSTRACT FROM AUTHOR]

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Functional Magnetic Resonance Imaging (fMRI) is an essential tool for the pre-surgical planning of brain tumor removal, which allows the identification of functional brain networks to preserve the patient's neurological functions. One fMRI technique used to identify the functional brain network is the resting-state-fMRI (rs-fMRI). This technique is not routinely available because of the necessity to have an expert reviewer who can manually identify each functional network. The lack of sufficient unhealthy data has so far hindered a data-driven approach based on machine learning tools for full automation of this clinical task. In this article, we investigate the possibility of such an approach via the transfer learning method from healthy control data to unhealthy patient data to boost the detection of functional brain networks in rs-fMRI data. The end-to-end deep learning model implemented in this article distinguishes seven principal functional brain networks using fMRI images. The best performance of a 75 % correct recognition rate is obtained from the proposed deep learning architecture, which shows its superiority over other machine learning algorithms that were equally tested for this classification task. Based on this best reference model, we demonstrate the possibility of boosting the results of our algorithm with transfer learning from healthy patients to unhealthy patients. This application of the transfer learning technique opens interesting possibilities because healthy control subjects can be easily enrolled for fMRI data acquisition since it is non-invasive. Consequently, this process helps to compensate for the usual small cohort of unhealthy patient data. This transfer learning approach could be extended to other medical imaging modalities and pathology. [ABSTRACT FROM AUTHOR]

Objective  The aim of the study is to model amplitude-integrated electroencephalography (aEEG) utility to diagnose seizures in common clinical scenarios. Study Design  Using reported neonatal seizure prevalence and aEEG sensitivities and specificities, likelihood ratios (LRs) and post-test probabilities were calculated to quantify aEEG utility to diagnose seizures in three typical clinical scenarios. Results  Prevalence data supported pretest probabilities for neonatal seizures of 0.4 in neonatal hypoxic ischemic encephalopathy (HIE), 0.27 in bacterial meningitis, and 0.05 in extreme prematurity. Reported sensitivity of 85% and specificity of 90% for seizures with expert aEEG interpretation yielded a positive likelihood ratio (LR+) of 8.7 and a negative likelihood ratio (LR−) of 0.17. Reported sensitivity of 65% and specificity of 70% with intermediate interpretation yielded LR+ 2.17 and LR− 0.5. Reported sensitivity of 40% and sensitivity of 50% with inexperienced interpretation gave LR+ 0.8 and LR− 1.2. These translate the ability to move pretest to post-test probability highly dependent on user expertise. For HIE, a pretest probability of seizure of 0.4 moves to a post-test probability of 0.85 when aEEG is positive for seizures by expert interpretation, and down to 0.1 when aEEG is negative. In contrast, no useful information was gained between pretest and post-test probability by aEEG interpreted as negative or positive for seizure at the inexperienced user level. Similarly, in the models of meningitis or extreme prematurity, incremental information gained from aEEG ranged widely based on interpreter experience. Conclusion  aEEG is most useful to screen for neonatal seizures when used in conditions with high seizure prevalence, and when interpretation has a sensitivity and specificity as reported for expert users. In contrast, aEEG can become negligible in providing meaningful clinical information when applied in conditions having lower seizure prevalence or when interpretation has low accuracy. Appropriate patient selection and high quality interpretation are essential for aEEG utility in neonatal seizure detection. Key Points aEEG utility for neonatal seizure screening relies on patient selection and quality interpretation. Utility of aEEG is highest with high seizure prevalence and expert interpretation. Utility of aEEG can be negligible with lower seizure prevalence or low accuracy interpretation. [ABSTRACT FROM AUTHOR]

Background: Artefact removal in neonatal electroencephalography (EEG) by visual inspection generally depends on the expertise of the operator, is time consuming and is not a consistent pre-processing step to the pipeline for the automated EEG analysis. Therefore, there is the need for the automated detection and removal of artefacts in neonatal EEG, especially of distinct and predominant artefacts such as flat line segments (mainly caused by instrumental error where contact between electrodes and head box is lost) and large amplitude fluctuations (related to neonatal movements). Method: A threshold-based algorithm for the automated detection and removal of flat line segments and large amplitude fluctuations in neonatal EEG of infants at term-equivalent age is developed. The algorithm applies thresholds to the absolute second difference, absolute amplitude, absolute first difference and the ratio between the frequency content above 50 Hz and the frequency content across all frequencies. Results: The algorithm reaches a median accuracy of 0.91, a median hit rate of 0.91 and a median false discovery rate of 0.37. Also, a significant improvement (≈10%) in the performance of a four-stage sleep classifier is observed after artefact removal with the proposed algorithm as compared to before its application. Significance: An automated artefact removal method contributes to the pipeline of automated EEG analysis. The proposed algorithm has shown to have good performance and to be effective in neonatal EEG applications. [ABSTRACT FROM AUTHOR]

In a cohort of children born very preterm (VPT), we investigated the association between breast milk feeding (BMF) initiation and its duration on cognitive development at 2 years of corrected age. Data were obtained from the Effective Perinatal Intensive Care in Europe population‐based prospective cohort of children born <32 weeks of gestation, in 11 European countries, in 2011–2012. The study sample included 4323 children. Nonverbal cognitive ability was measured applying the Parental Report of Children's Abilities, except for France where the problem‐solving domain of the Ages & Stages Questionnaire was used. Verbal cognition was based on the number of words the child could say. To determine the association between BMF (mother's own milk) and nonverbal and verbal cognition (outcome categorized as optimal and suboptimal), adjusted risk ratios (aRRs) were estimated fitting Poisson regression models, with inverse probability weights to account for nonresponse bias. Overall, 16% and 11% of the children presented suboptimal nonverbal and verbal cognition, respectively. Never BMF was associated with a significantly increased risk for suboptimal nonverbal (aRR = 1.29, 95% confidence interval [CI] = 1.09–1.53) and verbal (aRR = 1.45, 95% CI = 1.09–1.92) cognitive development compared with those ever breastfed, after adjustment for perinatal and sociodemographic characteristics. Compared with children breastfed 6 months or more, children with shorter BMF duration exhibited a statistically nonsignificant elevated aRR. VPT children fed with breast milk had both improved nonverbal and verbal cognitive development at 2 years in comparison with never breastfed, independently of perinatal and sociodemographic characteristics. This study encourages targeted interventions to promote BMF among these vulnerable children. Key messages: In a European cohort of children born very preterm, 16% and 11% presented suboptimal nonverbal and verbal cognitive development, respectively.Never‐breastfed children faced a higher risk of having suboptimal nonverbal and verbal cognition at 2 years of corrected age when compared with those who were breastfed, independently of perinatal and sociodemographic characteristics.Breastfeeding support is a modifiable factor regardless of perinatal and sociodemographic characteristics, which reinforces the importance of specifically targeted interventions to protect, promote and support breast milk feeding in neonatal intensive care units and after discharge. [ABSTRACT FROM AUTHOR]

Background: It is important to assess the degree of brain injury and predict long-term outcomes in neonates diagnosed with hypoxic-ischemic encephalopathy (HIE). However, routine studies, including magnetic resonance imaging (MRI) and conventional encephalography (EEG) or amplitude-integrated EEG (aEEG), have their own limitations in terms of availability and accuracy of evaluation. Recently, quantitative EEG (qEEG) has been shown to improve the predictive reliability of neonatal HIE and has been further refined with brain mapping techniques.Methods: We investigated background EEG activities in 29 neonates with HIE who experienced therapeutic hypothermia, via qEEG using a distributed source model. MRI images were evaluated and classified into two groups (normal-to-mild injury vs moderate-to-severe injury), based on a scoring system. Non-parametric statistical analysis using standardized low-resolution brain electromagnetic tomography was performed to compare the current density distribution of four frequency bands (delta, theta, alpha, and beta) between the two groups.Results: Electrical neuronal activities were significantly lower in the moderate-to-severe injury group compared with the normal-to-mild injury group. Background EEG activities in moderate-to-severe HIE were most significantly reduced in the temporal and parietal lobes. Quantitative EEG also revealed a decrease in background activity at all frequency bands, with a maximum in decrease in the delta component. The maximum difference in current density was found in the inferior parietal lobule of the right parietal lobe for the delta frequency band.Conclusions: Our study demonstrated quantitative and topographical changes in EEG in moderate-to-severe neonatal HIE. They also suggest possible implementation and evaluation of conventional EEG and aEEG in neonatal HIE. The findings have implications as biomarkers in the assessment of neonatal HIE. [ABSTRACT FROM AUTHOR]

Aim: This retrospective, longitudinal study examined the predictive value of the ages and stages questionnaire (ASQ) in late infancy for identifying children who progressed to have low cognitive ability at 5 years of age. Methods: The ASQ was performed on 755 participants from the Irish BASELINE birth cohort at 24 or 27 months of age. Intelligence quotient was measured at age 5 with the Kaufmann Brief Intelligence Test, Second Edition, and low cognitive ability was defined as a score more than 1 standard deviation below the mean. The ASQ's predictive value was examined, together with other factors associated with low cognitive ability at 5 years. Results: When the ASQ was performed at 24 or 27 months, the overall sensitivity for identifying low cognitive ability at 5 years was 20.8% and the specificity was 91.1%. Using a total score cut‐off point increased the sensitivity to 46.6% and 71.4% at 24 and 27 months, but specificity fell to 74.1% and 67.2%, respectively. After adjusting for ASQ performance, maternal education and family income were strongly associated with cognitive outcomes at 5 years. Conclusion: The ASQ did not detect the majority of children with low cognitive ability at age 5. Alternative methods need investigation. [ABSTRACT FROM AUTHOR]

This study aimed to evaluate the association of neonatal transfer with the risk of neurodevelopmental outcomes at 3 years of age. Data were obtained from the Japan Environment and Children's Study. A general population of 103,060 pregnancies with 104,062 fetuses was enrolled in the study in 15 Regional Centers between January 2011 and March 2014. Live-born singletons at various gestational ages, including term infants, without congenital anomalies who were followed up until 3 years were included. Neurodevelopmental impairment was assessed using the Ages and Stages Questionnaire, third edition (ASQ-3) at 3 years of age. Logistic regression was used to estimate the adjusted risk and 95% confidence interval (CI) for newborns with neonatal transfer. Socioeconomic and perinatal factors were included as potential confounders in the analysis. Among 83,855 live-born singletons without congenital anomalies, 65,710 children were studied. Among them, 2780 (4.2%) were transferred in the neonatal period. After adjustment for potential confounders, the incidence of neurodevelopmental impairment (scores below the cut-off value of all 5 domains in the ASQ-3) was higher in children with neonatal transfer compared with those without neonatal transfer (communication: 6.5% vs 3.5%, OR 1.42, 95% CI 1.19–1.70; gross motor: 7.6% vs 4.0%, OR 1.26, 95% CI 1.07–1.49; fine motor: 11.3% vs 7.1%, OR 1.19, 95% CI 1.03–1.36; problem solving: 10.8% vs 6.8%, OR 1.29, 95% CI 1.12–1.48; and personal-social: 6.2% vs 2.9%, OR 1.52, 95% CI 1.26–1.83). Conclusion: Neonatal transfer was associated with a higher risk of neurodevelopmental impairment at 3 years of age. What is Known: • Neonatal transfer after birth in preterm infants is associated with adverse short-term outcomes. • Long-term outcomes of outborn infants with neonatal transfer in the general population remain unclear. What is New: • This study suggests that neonatal transfer at birth is associated with an increased risk of neurodevelopmental impairment. • Efforts for referring high-risk pregnant women to higher level centers may reduce the incidence of neonatal transfer, leading to improved neurological outcomes in the general population. [ABSTRACT FROM AUTHOR]

Although migraine in adult and pediatric patients are overall very similar to each other, differences in prevalence, presentation, and treatment efficacy may reflect slight differences in the pathophysiological processes underlying migraine in these patient groups, perhaps because of ongoing development of the nervous system during childhood and adolescence. Although major gains have been made in understanding the pathophysiology of migraine in adults in recent years, equivalent research on migraine in pediatric patients continues to lag behind. In this review, we will describe the current state of migraine research in pediatric patients with regard to presentation and frequency of prodromal and postdromal symptoms, ictal and interictal calcitonin gene-related peptide elevation, and evidence for cortical spreading depression, thus covering all phases of migraine, and discuss how the findings seen here may relate to possible underlying pathophysiological mechanisms of migraine. We aim to elucidate possible differences between migraine in children and adults, and the need for further research specific to pediatric patients with migraine in order to improve treatment in this patient group. [ABSTRACT FROM AUTHOR]

Background: There are few data concerning the neonatal and long-term prognosis of monochorionic biamniotic twin pregnancies (MCBA) complicated by selective intrauterine growth restriction (sIUGR). The aim of the study is to assess the neurological outcomes at two years of age of these newborns and compares these outcomes to those of newborns resulting from intrauterine growth restriction (IUGR) pregnancies. Methods: The study focuses on a cross-sectional prospective cohort of patients treated between 2012 and 2019 in Marseille, France. The primary endpoint is the overall score of the Ages and Stages questionnaires (ASQ) at two years, which assesses the global neurodevelopment. The secondary endpoint is the assessment of neonatal morbi-mortality for both groups (composite endpoint). Results: In total, 251 patients were included in the analysis: 67 in the sIUGR group and 184 in the IUGR group. There was no statistically significant difference in the overall ASQ score at two years but there was the finest motor skills impairment in the IUGR group. The areas most often impaired were communication and fine motor skills. There were no significant differences between the neonatal morbi-mortality of the two groups (adjusted OR = 0.95, p = 0.9). Conclusions: Newborns from MCBA pregnancies with sIUGR appear to have similar overall neurological development to IUGR. Notably, IUGR seems to have the most moderate neurobehavioral disorder (fine motor) as a consequence of impaired antenatal brain development due to placenta insufficiency leading to chronic hypoxia. [ABSTRACT FROM AUTHOR]

This study's objective was to correlate the abnormalities in brain MRIs performed at corrected-term age for minor or moderate neurocognitive disorders in children school-age born extremely premature (EPT) and without serious sequelae such as autism, cerebral palsy, mental impairment. Data were issued from a cross-sectional multicenter study (GP-Qol study, number NCT01675726). Clinical examination and psychometric assessments were performed when the children were between 7 and 10 years old during a day-long evaluation. Term-equivalent age brain MRIs on EPT were analyzed with a standardized scoring system. There were 114 children included in the study. The mean age at the time of evaluation, was 8.47 years old (± 0.70). 59% of children with at least one cognitive impairment and 53% who had a dysexecutive disorder. Only ten EPT (8.7%) presented moderate to severe white and grey matter abnormalities. These moderate to severe grey matter abnormalities were associated with at least two abnormal executive functions [OR 3.08 (95% CI 1.04–8.79), p = 0.04] and language delay [OR 3.25 (95% CI 1.03–9.80), p = 0.04]. These results remained significant in the multivariate analysis. Moderate to severe ventricular dilatation abnormalities (15%, n = 17) were associated with ideomotor dyspraxia [OR 7.49 (95% CI 1.48–35.95), p = 0.02] and remained significant in multivariate analysis [OR 11.2 (95% CI 1.45–131.4), p = 0.02]. Biparietal corrected diameters were moderate abnormal in 20% of cases (n = 23) and were associated to visuo spatial integration delay [OR 4.13 (95% CI 1.23–13.63), p = 0.02]. Cerebral MRI at term-equivalent age with scoring system analysis can provide information on long-term neuropsychological outcomes at school-age in EPTs children having no severe disability. [ABSTRACT FROM AUTHOR]

Aim: To evaluate quantitative electroencephalogram (EEG) measures as predictors of long‐term neurodevelopmental outcome in infants with a postconceptional age below 46 weeks, including typically developing infants born at term, infants with heterogeneous underlying pathologies, and infants born preterm. Method: A comprehensive search was performed using PubMed, Embase, and Web of Science from study inception up to 8th January 2021. Studies that examined associations between neonatal quantitative EEG measures, based on conventional and amplitude‐integrated EEG, and standardized neurodevelopmental outcomes at 2 years of age or older were reviewed. Significant associations between neonatal quantitative EEG and long‐term outcome measures were grouped into one or more of the following categories: cognitive outcome; motor outcome; composite scores; and other standardized outcome assessments. Results: Twenty‐four out of 1740 studies were included. Multiple studies showed that conventional EEG‐based absolute power in the delta, theta, alpha, and beta frequency bands and conventional and amplitude‐integrated EEG‐related amplitudes were positively associated with favourable long‐term outcome across several domains, including cognition and motor performance. Furthermore, a lower presence of discontinuous background pattern was also associated with favourable outcomes. However, interpretation of the results is limited by heterogeneity in study design and populations. Interpretation: Neonatal quantitative EEG measures may be used as prognostic biomarkers to identify those infants who will develop long‐term difficulties and who might benefit from early interventions. [ABSTRACT FROM AUTHOR]

Infants born in early term (37–38 weeks gestation) experience slower neurodevelopment than those born at full term (40–41 weeks gestation). While this could be due to higher perinatal morbidity, gestational age at birth may also have a direct effect on the brain. Here we characterise brain volume and white matter correlates of gestational age at birth in healthy term‐born neonates and their relationship to later neurodevelopmental outcome using T2 and diffusion weighted MRI acquired in the neonatal period from a cohort (n = 454) of healthy babies born at term age (>37 weeks gestation) and scanned between 1 and 41 days after birth. Images were analysed using tensor‐based morphometry and tract‐based spatial statistics. Neurodevelopment was assessed at age 18 months using the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley‐III). Infants born earlier had higher relative ventricular volume and lower relative brain volume in the deep grey matter, cerebellum and brainstem. Earlier birth was also associated with lower fractional anisotropy, higher mean, axial, and radial diffusivity in major white matter tracts. Gestational age at birth was positively associated with all Bayley‐III subscales at age 18 months. Regression models predicting outcome from gestational age at birth were significantly improved after adding neuroimaging features associated with gestational age at birth. This work adds to the body of evidence of the impact of early term birth and highlights the importance of considering the effect of gestational age at birth in future neuroimaging studies including term‐born babies. [ABSTRACT FROM AUTHOR]

Introduction: Non-dystrophic myotonias (NDMs) are skeletal muscle ion channelopathies caused by CLCN1 or SCN4A mutations. This study aimed to describe the clinical, myopathological, and genetic analysis of NDM in a large Chinese cohort. Methods: We reviewed the clinical manifestations, laboratory results, electrocardiogram, electromyography, muscle biopsy, genetic analysis, treatment, and follow-up of 20 patients (from 18 families) with NDM. Results: Cases included myotonia congenita (MC, 17/20) and paramyotonia congenita (PMC, 3/20). Muscle stiffness and hypertrophy, grip and percussion myotonia, and the warm-up phenomenon were frequently observed in MC and PMC patients. Facial stiffness, eye closure myotonia, and cold sensitivity were more common in PMC patients and could be accompanied by permanent weakness. Nine MC patients and two PMC patients had cardiac abnormalities, mainly manifested as cardiac arrhythmia, and the father of one patient died of sudden cardiac arrest. Myotonic runs in electromyography were found in all patients, and seven MC patients had mild myopathic changes. There was no difference in muscle pathology between MC and PMC patients, most of whom had abnormal muscle fiber type distribution or selective muscle fiber atrophy. Nineteen CLCN1 variants were found in 17 MC patients, among which c.795T>G (p.D265E) was a new variant, and two SCN4A variants were found in three PMC patients. The patients were treated with mexiletine and/or carbamazepine, and the symptoms of myotonia were partially improved. Conclusions: MC and PMC have considerable phenotypic overlap. Genetic investigation contributes to identifying the subtype of NDM. The muscle pathology of NDM lacks specific changes. [ABSTRACT FROM AUTHOR]

Purpose of Review: To review the literature on the clinical characteristics of the symptoms other than headache that occurs during a migraine attack in childhood and adolescence. Recent Findings: Premonitory symptoms (42–67%) and postdrome phase (82%) are frequent. The most frequent auras were visual. There was no association between age or sex and the occurrence of auras. Cranial autonomic symptoms are also frequent (40–70%) and are most often bilateral. Most studies suggest that age is not associated with the frequency of nausea, vomiting, photophobia, and phonophobia. Cephalic cutaneous allodynia (15–37%) and osmophobia (20–53%) are common symptoms in children with migraine. Osmophobia has low sensitivity and high specificity for the diagnosis of migraine and is associated with the severity of the migraine. Summary: Migraine is a complex disease, and although headache is its best-known symptom, other symptoms also occur frequently during migraine attacks in children and adolescents. [ABSTRACT FROM AUTHOR]

The goal of this study was to examine executive functioning, math performance, and visuospatial processing skills of children with perinatal stroke, which have not been well explored in this population. Participants included 18 children with perinatal stroke (aged 6-16 years old) and their primary caregiver. Each child completed standardized tests of executive function and visuospatial processing skills, Intelligence Quotient (IQ), and math achievement. Performance on executive function, IQ, math, and visuospatial processing tests was significantly lower in children with perinatal stroke when compared to normative means. Poorer inhibitory control was associated with worse math performance. Increased age at testing was associated with better performance on visuospatial ability (using standardized scores), and females performed better than males on a test of inhibitory control. Children with perinatal stroke displayed a range of neuropsychological impairments, and difficulties with executive function (inhibition) may contribute to math difficulties in this population. [ABSTRACT FROM AUTHOR]

Aim: This study sought to evaluate the accuracy of the Ages and Stages Questionnaires 3rd Edition (ASQ-3) in identifying developmental delay (DD) in children with congenital heart disease (CHD) born at term who underwent surgical repair. Methods: Participants had to complete ASQ-3 and Bayley Scales of Infant and Toddler Development 3rd Edition (BSID-III) at 12 and 24 months. A child was considered at risk of DD for a ASQ-3 domain when he scored below the cutoff (≤-1SD or ≤-2SD). A child had a DD in a BSID-III domain when the score was ≤-1SD. The validity for each ASQ-3 domain and for overall ASQ-3 was measured. Results: At 12 months (n = 64), overall ASQ-3 (≤-2SD) sensitivity was 88%, specificity 74%. At 24 months (n = 82), overall ASQ-3 (≤-2SD) sensitivity was 74%, specificity 88%. Conclusion: The results support the utility of the ASQ-3 for screening the overall risk of DD in children with CHD. [ABSTRACT FROM AUTHOR]

Background: The lateral spread response (LSR) is an electromyography feature of hemifacial spasm; intraoperative reduction in the LSR is associated with positive surgical outcomes. This study examined the effects of different minimum alveolar concentrations (MACs) and durations of sevoflurane inhalation on the LSR., Methods: Eighty patients undergoing microvascular decompression surgery for hemifacial spasm were randomly allocated to receive propofol-remifentanil total intravenous anesthesia alone or in combination with sevoflurane at 0.5, 0.75, or 1 MAC. The LSR and orbicularis oculi muscle wave were recorded before and at 15 and 30 minutes after the start of sevoflurane administration., Results: Sevoflurane reduced the LSR amplitude in a dose-dependent and duration-dependent manner. The curve representing the LSR amplitude preservation ratio change according to sevoflurane concentration is best fitted by regression analysis using a cubic model, as the cubic equations had the largest coefficient of determination; at 15 minutes ( R2 =0.76, F =78.36, P <0.05) and at 30 minutes ( R2 =0.882, F =189.94, P <0.05). The inhibitory effect of sevoflurane on the LSR amplitude was greater in the first 15 minutes than in the second 15 minutes of sevoflurane administration. Sevoflurane at 1 MAC for 30 minutes mildly decreased the amplitude of the orbicularis oculi muscle wave. The latencies of the LSR and the orbicularis oculi muscle wave were not affected by sevoflurane at all MACs studied., Conclusions: The combination of intravenous propofol-remifentanil anesthesia with 0.5 MAC sevoflurane allows reliable intraoperative LSR monitoring in hemifacial spasm patients. Our findings support the central rather than peripheral hypothesis of the LSR., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)