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• Drug-resistant epilepsies (DRE) are a challenge for paediatricians. • The steroid must be considered a therapeutic option in these forms of epilepsy. • 62.68 % of patients presented a reduction in epileptic seizures greater than 50 %. • DREs with structural and immune aetiology respond better than genetic forms. • The most effective steroid protocol is methylprednisolone 30 mg/kg/day for 3 days. Our study aimed to evaluate the effectiveness of corticosteroids on seizure control in drug-resistant epilepsies (DREs). Our primary goal was to assess the response to steroids for various underlying etiologies, interictal electroencephalographic (EEG) patterns and electroclinical seizure descriptions. Our second goal was to compare steroid responsiveness to different treatment protocols. This is a retrospective multicentre cohort study conducted according to the STROBE guidelines (Strengthening the Reporting of Observational Studies in Epidemiology). The following data were collected for each patient: epilepsy etiology, interictal EEG pattern, seizure types and type of steroid treatment protocol administered. Thirty patients with DRE were included in the study. After 6 months of therapy, 62.7 % of patients experienced reduced seizure frequency by 50 %, and 6.6 % of patients experienced complete seizure cessation. Findings associated with favourable response to steroids included structural/lesional etiology of epilepsy, immune/infectious etiology and focal interictal abnormalities on EEG. Comparing four different steroid treatment protocols, the most effective for seizure control was treatment with methylprednisolone at the dose of 30 mg/kg/day administered for 3 days, leading to greater than 50 % seizure reduction at 6 months in 85.7 % of patients. Treatment with dexamethasone 6 mg/day for 5 days decreased seizure frequency in 71.4 % of patients. Hydrocortisone 10 mg/kg administered for 3 months showed a good response to treatment in 71 %. In our study, two-thirds of patients with DRE experienced a significant seizure reduction following treatment with steroids. We suggest considering steroids as a potential therapeutic option in children with epilepsy not responding to conventional antiseizure medicines (ASM). [ABSTRACT FROM AUTHOR]

Psychopathology has not yet been studied beyond pediatric age for all degrees of prematurity, including late-preterm, particularly in those who grew up with no apparent neurodevelopmental sequelae. This study aimed to examine psychopathological outcome following preterm birth and admission to neonatal intensive care in young adults without major neurodevelopmental and psychopathological problems that emerged during childhood. An Italian single-center prospective cohort study. Eighty-nine young adults (40 admitted to neonatal intensive care unit with less than 37 weeks of gestation and no medical history of other neurological or psychiatric conditions in childhood and 49 healthy peers born at term, matched by age, sex, and education) underwent neuropsychiatric interviews at the age of 20 ± 1 years; MINI International Neuropsychiatric Interview, Beck Depression Inventory and Barratt Impulsive Scale, results were correlated to individual neonatal data and cognitive measures. We found a significantly higher prevalence of psychopathology at MINI score (22.5% vs. 4.2%; χ2 = 6.7; p = 0,010) and prevalence of previous stressful life events in the preterm compared to at-term group. B.D.I. (testing depression) and BIS-11(testing impulsivity) did not highlight a statistically significant difference between the groups. All patients had average I.Q., a statistically significant difference (p < 0.001) was observed between groups with a better performance in controls than cases. Preterm infants attaining young adult age with otherwise typical development during childhood are at risk of psychopathology and lower resilience to stressful life events. The MINI interview could be a useful tool to highlight the psychopathology of preterm infants attaining adult age. • Young adults born late-preterm NICU admitted are at risk for psychopathology similarly to those born very preterm. • The MINI interview could be a useful tool to highlight the psychopathology of ex-preterm adults. • Mood disorders and anxiety can emerge in adults born preterm without psichopatology in childhood. • A reduced resilience to stressful life events seems to be associated with the psychopathological outcome of the preterm birth. • Early dysregulation of mechanisms that modulate emotional and social adjustment may affect mental health in adulthood. [ABSTRACT FROM AUTHOR]

Acute necrotizing encephalopathy (ANE) is a rare disease not yet described in children with Covid-19. RANBP2 gene variations are implicated in recurrences in the genetic form of ANE, the so called ANE1. We report the first case of pediatric ANE1 following Sars-CoV-2 infection. She had a first episode at 2 years of age following influenza type A with full recovery, many other respiratory and non-respiratory febrile viral infections without recurrences and a severe recurrence following Sars-CoV-2 infection, suggesting a potentiation effect on cytokine cascade. Her MRI showed the typical pattern of injury resembling that of mitochondrial disorders, and supported the role of RANBP2 in mitochondrial homeostasis. This case rises attention on diagnostic challenges and offers several interesting tips for discussion about new perspectives in pathogenesis and targeted treatments. • Acute necrotizing encephalomyelitis can complicate Covid 19. • Genetic tests are recommended in recurrent ANE for prognostic and therapeutic implications. • RANBP2 genotype should be looked for in recurrent in acute necrotizing encephalomyelitis. [ABSTRACT FROM AUTHOR]

Inherited retinal dystrophies (IRDs) are a major cause of childhood blindness. Timely diagnosis requires a high level of clinical suspicion from both neurologists and ophthalmologists and is increasingly important given recent advancements in gene therapy. We focused our study on genotype-phenotype associations in very early-onset forms of retinal dystrophy, the least well characterized and most challenging diagnostic subgroup. From January 12, 2015 to March 31, 2017, we prospectively performed whole exome sequencing targeted on the phenotype of non-syndromic IRDs and phenotype characterization in a cohort of 68 children affected by very early-onset inherited retinal dystrophies, defined by the onset before five years of age. Phenotype parameters included age at onset, clinical presentation, ophthalmic evaluation, electrophysiological patterns and clinical course. A genetically confirmed diagnosis was achieved in 50 out of 60 (83%) families. The median age at onset was 4 months (<6 m in 70%, < 2 y in 82% of the cases). Clinical presentation was associated with visual loss and nystagmus in the majority of patients. Three (CNGB3, CNGA3 and CACNA1F) out of 22 genes considered pathogenic in the cohort, accounted for 51% of all IRD's, all within the class of stationary IRDs. This study reports on the largest cohort of very early-onset retinal dystrophies, including a description of electroretinography patterns. The electro-clinical phenotype coupled with genetic diagnosis provided additional clues for child neurologists dealing with low vision and nystagmus in infancy. A high level of clinical suspicion improves the diagnosis with important implications for the future of the affected child. • Diagnosis of low vision in infancy bridges and challenges paediatric neurology and ophthalmology. • Inherited retinal dystrophies are major causes of inherited childhood blindness. • Very early onset forms of retinal dystrophy are the least well characterized and most difficult diagnostic subgroup. • Exome analysis performed in clinically focused tiered fashion allowed a detection rate of 82%, the highest reported to date. • We propose an electro-clinical diagnostic algorithm for low vision and nystagmus in infancy. [ABSTRACT FROM AUTHOR]

Major deficits in the autonomic nervous system function, detected by measuring heart rate variability (HRV), are reported in neonatal hypoxic-ischemic encephalopathy (HIE)). However, it is unknown if they will recover in the long-term. Because of the possible implications for the neurological outcome, this study aimed to evaluate the HRV at school age, in a cohort of children who survived HIE managed with therapeutic hypothermia. A cross-sectional study of HRV in 40 children: 20 HIE survivors and 20 healthy peers. All underwent 5-min plethysmography using the PPG Stress Flow device (BioTekna Italy). Absolute and normalized HRV spectral power in the very low frequency (VLF), low frequency (LF), and high frequency (HF) bands and total power were compared between patients and healthy children. The outcome evaluation included neurological, cognitive (WISC-IV), and psychosocial (Parent Stress Index-Short Form-PSI-SF and psychosocial interview) measures. All mean HRV values were significantly higher in survivors of HIE, compared to healthy peers, with the larger effect size for the HF band (Total Power 8.57 ± 0.59 vs 7.82 ± 0.77 ms2, p.003 ES 0.21; HF 7.82 + 0.77 vs 8.57 + 0.59 ms2, p.001 EF 0.24). None of the children had major health, neurological and psychosocial (PSI-SF/interview) problems. The IQ (WISC-IV) was normal in 17/20 patients, borderline in 2, and <70 in 1. HRV measures highlight autonomic dysfunction at school age in survivors of neonatal HIE, in the absence of major neurodevelopmental and psychosocial problems. The significance of this finding for children's future life needs further neuropsychiatric investigations and longer follow-up. • Heart rate variability is a reliable measure of autonomic function in children. • Plethysmography informs on heart rate variability changes at school-age, in survivors of hypoxic-ischemic encephalopathy. • Frequency analysis of heart rate variability highlighted autonomic over activation in patients compared to healthy peers. • In the absence of major neurological disability, autonomic dysfunction point to neurobiological vulnerability. [ABSTRACT FROM AUTHOR]

Background Neurocognitive deficits at school starting age may affect as many as 50% of children who underwent cardiac surgery for complex congenital heart disease (CHD). The aim of this study was to identify which phases of cardiopulmonary bypass (CPB) are associated with an increased risk of impaired neurodevelopmental skills in children with complex CHD. This was assessed by means of glial fibrillary acidic protein (GFAP) plasma levels during CPB for CHD surgery, as a marker of neurologic insult. We correlated GFAP levels with clinical parameters and neurodevelopmental outcome. Methods We studied 45 children undergoing surgery for complex CHD. We measured plasma GFAP levels by enzyme-linked immunosorbent assay at the following steps: anesthesia induction, CPB start, end of hypothermia, end of rewarming, and end of CPB. Neurologic assessment and Vineland Adaptive Behavior Scales (VABS-I) were administered to patients at least 18 months after surgery. Results GFAP was undetectable before surgery and it peaked at the end of hypothermia or rewarming. Multiple regression analyses showed that GFAP peak level and preoperative neurologic comorbidity were significant independent predictors of neurologic impairment, as showed by VABS-I communication domain intelligence quotient (IQ). Receiver operating characteristic curve showed that the model was highly significant. Conclusions Impaired neurodevelopment was associated with increase of GFAP plasma levels during cardiac surgery in infants. The identification of the neurologic high-risk phases of CPB run could support the application of new neuroprotective strategies for CHD repair. [ABSTRACT FROM AUTHOR]

Background Acute diplopia (AD) is an uncommon and distressing symptom of numerous ocular and neurological conditions, with potentially serious sequelaes. No data are present in pediatrics on the presentation and management of AD. Aim This study investigated characteristics, etiology and health care utilization of the pediatric population with AD accessed to pediatric Emergency Departments (ED), trying to identify “red flags” associated with potentially life -threatening (LT) conditions. Methods We conducted a cohort multicenter study on children with AD in ten Italian hospitals. Patients were classified into diagnostic categories, comparing children with and without LT disease. Results 621 children presented AD at a rate of 3.6 per 10.000. The most frequent diagnosis among no-LT conditions (81.2%) were headache, ocular disorders and minor post-traumatic disease, while LT conditions (18.8%) were represented by brain tumors, demyelinating conditions, idiopathic intracranial hypertension and major post-traumatic diseases. The LT group showed a significantly higher age, with the odds increased by 1% for each month of age. Monocular diplopia occurred in 16.1%, but unlike adult one-fifth presented LT conditions. Binocular diplopia, associated ocular manifestations or extraocular neurological signs were significantly more common in the LT group. At regression logistic analysis strabismus and ptosis were associated with LT conditions. Conclusion The majority of children presented no-LT conditions and more than one-fourth of patients had headache. Monocular diplopia in the LT group was never isolated but associated with other signs or symptoms. Our study was able to identify some specific ocular disturbances or neurologic signs potentially useful for ED physician to recognize patients with serious pathologies. [ABSTRACT FROM AUTHOR]

Early markers of neurological outcome in the absence of overt brain damage are scarce in extreme prematurity. The aim of this study was to compare spectral EEG values of infants born near term with those of infants born at extremely low gestational age (ELGA) but having attained near term age. We aimed also to evaluate whether spectral EEG features were related with neurological outcome. The ELGA group consisted of 12 neonates born between 23 + 2 and 27 + 6 weeks; the control group consisted of nine infants born 34–35 + 2 weeks, tested within the first week of life. All neonates underwent multichannel EEG recordings at 35 weeks post-conception. None of the subjects had apparent neurological abnormalities or risk factors at the time of recording. EEG data were transformed into the frequency domain and divided into delta (0.5–4 Hz), theta (5–7 Hz), alpha (8–13 Hz), beta (14–20 Hz) frequency bands; relative EEG power values were calculated. ELGA group was compared with the control group using a mixed analysis of variance. Outcome was evaluated at one year of age by Griffiths’ scales. A principal effect of frequency and an interaction effect of frequency * group was found. The total relative power of the delta band was significantly higher in ELGA than in control group, whereas in the remaining frequency bands total relative power was lower in ELGA than in control group. Higher values of delta and lower values of alpha and beta spectral power correlated with poor outcome. We provide preliminary results suggesting that, as early as 35 weeks post conception, infants born extremely preterm fail to develop the age specific pattern of EEG spectral activity, in the absence of neurological neonatal risk. [ABSTRACT FROM AUTHOR]

Background Moyamoya syndrome represents an etiologically heterogeneous cerebral evolutive angiopathy. It can be associated with both well-characterized and recently described genetic conditions with mendelian inheritance. Case report We report the case of a moyamoya angiopathy in a prematurely born girl affected by congenital heart defect, mild facial dysmorphism, mild neurodevelopmental delay and borderline cognitive profile, associated to a de novo complex rearrangement involving the terminal segment of the short arm of chromosome 6. Conclusion To the best of our knowledge, this is the second case described of pediatric moyamoya syndrome associated with a 6p complex rearrangement. Adding this case to the pertinent literature, we discuss the pathogenic role of rearrangements in 6p region in moyamoya syndrome and suggest to investigate in this region potential genes involved in angiogenesis or vascular homeostasis. [ABSTRACT FROM AUTHOR]

Summary Objective To evaluate whether wearing a wool cap, a routine practice used to prevent heat loss in premature infants, affects interpretation of electroencephalogram spectral analysis. Methods Eighteen premature infants (median gestational age 28 weeks, range 23–32) without neurological complications were randomized to two channel (C3, C4 referred to Cz) digital electroencephalogram recordings with (90 min) and without (90 min) wearing wool cap, at 4 days of life. Electroencephalogram was analyzed automatically by measurement of burst suppression ratio and asymmetry index and by Fast Fourier Transform to calculate total absolute spectral power; relative spectral power in the δ (0.5–3.5 Hz), θ (4–7.5 Hz), α (8–12.5 Hz), and β (13–30 Hz) frequency bands; spectral edge frequency; and mean dominant frequency. Results The use of wool cap had no effect on all electroencephalogram parameters considered. Gestational age showed an effect on relative spectral power of all considered bands, spectral edge frequency and mean dominant frequency, while no effect was seen on burst suppression ratio and asymmetry index. Neonates born at gestational weeks lower than 28 had significantly higher relative power in the δ band and lower relative power in the α and β bands. Conclusions Heat loss prevention using wool cap does not affect interpretation of spectral electroencephalogram. Spectral values in our group of very premature infants without neurological complications correspond to normal data reported in the literature. Maturation changes consist of reduction of relative power of the δ band, spectral edge frequency and mean dominant frequency. [ABSTRACT FROM AUTHOR]

Abstract: Background: Acute disseminated encephalomyelitis is an inflammatory-demyelinating disorder of the central nervous system usually with a monophasic course and a favorable neurological outcome. Long-term neurocognitive sequelae and quality of life have not yet been fully investigated. Aim: To examine neurocognitive outcome and quality of life in pediatric monophasic acute disseminated encephalomyelitis. Methods: Of the 36 patients diagnosed with acute disseminated encephalomyelitis at our institution, six were lost to follow-up and eight relapsed (two with multiphasic forms and six with multiple sclerosis). The outcome of the 22 remaining patients was evaluated using four subscales of the Wechsler Intelligence Scales for estimation of IQ, a battery of neuropsychological tests, and semistructured and PedsQL questionnaires for quality of life. The effect of age at onset, neuroradiological recovery, and time elapsed from the acute event on outcome was also investigated. Results: Estimated IQ, neuropsychological mean group scores, and quality of life at follow-up were within the normal range, but 23% of the patients had pathological scores in various neuropsychological functions, among which attention was the most clearly affected. The neuroradiological recovery was not correlated with the result of the neuropsychological tests. Age at onset correlated with linguistic skills, whereas the time elapsed from the acute event had a significant effect on attention tasks: scores were worse in the group of patients with a follow-up shorter than 7 years. Conclusion: Our results suggest that pediatric monophasic acute disseminated encephalomyelitis has a favorable neurocognitive outcome. Patients with longer follow-up had a better outcome, suggesting a neurocognitive course that is different from that of multiple sclerosis and a potential for long-term recovery of affected functions. [Copyright &y& Elsevier]

Abstract: Objectives: To evaluate the extent of oxidative stress in neonates born from multiple gestation pregnancies who are at high risk of prematurity and growth abnormalities. Design and methods: Blood samples were collected from umbilical cord of 72 twins, born at gestational age of 28–38weeks, and 20 consecutive control singletons. Oxidative stress parameters (15-F2t-isoprostane, a marker of lipid peroxidation, and total antioxidant capacity, tAOC), were measured in cord plasma. Results: Levels of 15-F2t-isoprostane showed a moderate negative correlation with birth weight and were higher in small co-twins of discordant pairs; tAOC was positively correlated with birth weight but no significant difference was found between co-twins. Conclusions: Oxidative stress levels in twins are mainly influenced by birth weight and weight discordance. We suggest that evaluation of cord blood 15-F2t-isoprostane might be of clinical value as maker of pre- and perinatal distress in twinning. [Copyright &y& Elsevier]

Abstract: Auditory processing during sleep was investigated in premature infants by auditory event related potentials (AERPs). Twenty-six premature infants (mean GA 30week– range 25–35) admitted to a neonatal intensive care unit were studied, prior to discharge, in active and quiet sleep at a mean post-conceptional age of 35weeks. Infant state was determined by behavioral observation according to standard criteria. An auditory odd-ball paradigm was used with frequently occurring ‘standard’ tones at 1000Hz and infrequent ‘deviant’ tones at 2000Hz. Waveforms were recorded at Fz, Cz, Pz, T3 and T4 scalp locations. Measurements were performed in 18 patients because 8 preterm infants were excluded since they had less than the required artifact-free deviant trials in each sleep state. The responses to standard tones were equally recorded in both active and quiet sleep, but auditory responses to deviant tones consisting of an increased frontal negativity in the time period from 200 to 300ms after the stimulus were recorded only in active sleep. A significant effect of electrode placement, for frontal location by sleep condition and sleep condition by 50ms time windows was shown by repeated measures analyses of variance. The significance of these findings on evoked potential methodology in preterm infants admitted to neonatal intensive care unit is discussed. [ABSTRACT FROM AUTHOR]

Abstract: Bilateral loss of cortical somatosensory evoked potential (SEP) is considered the single best indicator of adverse outcome in acute encephalopathy of adult patients and older children. This study determines whether the presence or absence of the neonatal cortical SEP can predict cerebral palsy at two years in survivors of neonatal encephalopathy scored according to Sarnat criteria. We also compare SEPs with visual evoked potentials (VEPs), the EEG and neonatal neurological status. Fifty-nine neonates admitted to the neonatal intensive care unit had SEP, VEP and EEG recordings analysed according to the presence (n =37, 63%) or absence (n =22, 37%) of neonatal encephalopathy (score ≥1). Cortical SEP was always present in the perinatal period in those surviving without major neurological disability, while it was bilaterally absent in all but one patient with a subsequent diagnosis of cerebral palsy. Multivariate analysis using the logistic regression model showed that bilateral loss of cortical SEP and Sarnat Score correctly classified the neurological outcome in all patients. Bilateral absence of cortical SEP indicates early identification of neonates at risk of cerebral palsy indicating that EPs have a clinical role in the workup of neonatal encephalopathy. [Copyright &y& Elsevier]

Summary: Introduction: The contribution of clinical neurophysiology in the neurological prognosis of hypoxic-ischemic coma has been well established in adults: the bilateral absence of cortical somatosensory evoked potentials (SEP) is considered the single best indicator of adverse outcome, while the presence of the auditory mismatch negativity (MMN) is thought to herald arousal. Study aim: To use MMN combined with serial EEG recordings, somatosensory and brainstem auditory evoked potentials (BAEP) in a paediatric case of postanoxic coma managed with hypothermia, since they have not yet been described in children. Methods: We report the case of a nine-year-old boy with hypoxic-ischemic encephalopathy due to cardiorespiratory arrest after accidental burial in sand, who was treated with therapeutic hypothermia for 72hours. Serial EEG recordings, evoked potentials, brain CT scan and brain MRI were performed in the first few days after the event. Results: SEP to median nerve stimulation showed bilateral absence of the N20 component, while the N13 and P14 peaks were preserved; BAEP showed normal I–V interpeak latency and normal hearing threshold. At the same time, the MMN component of auditory event related potentials, recorded in the classical oddball paradigm, was absent. Seventeen months after the accident, the patient is alive in persistent vegetative state. Conclusions: This case illustrates the particular significance of SEP and MMN together with EEG in gaining prognostic information, even in sedated and hypothermic patients, and encourages systematic study of these prognostic tools in paediatric postanoxic coma. [Copyright &y& Elsevier]

This study investigated the possible prognostic factors for relapse, and the diagnostic criteria for multiple sclerosis and related disorders, in pediatric acute disseminated encephalomyelitis. The study population comprised 24 Italian children with a mean age at onset of 6.9 years, and a mean follow-up time of 52.8 months (range, 12-180). Clinical, neurophysiologic, spinal-fluid, neuroradiologic, and outcome features were investigated. All patients but 2, who were reclassified as exhibiting clinically isolated syndromes, fulfilled the new classification criteria for acute disseminated encephalomyelitis recently proposed by the International Pediatric Multiple Sclerosis Study Group. Three patients relapsed after 3 months, 2 years, and 8 years, respectively. By the second attack, the diagnosis of multiple sclerosis, as well as of multiphasic disseminated encephalomyelitis, could be rendered using the revised criteria of McDonald et al. Long-term follow-up seemed to confirm a chronic disease course in 2 children. We could not identify features at onset to predict outcomes of patients. However, early in follow-up, the appearance of oligoclonal immunoglobulin G bands in spinal fluid and the persistence of visual-evoked potential abnormalities were associated with poor outcomes. [Copyright &y& Elsevier]

Abstract: Objective: To compare the diagnostic reliability of automated transient evoked otoacoustic emissions (a-TEOAE), automated auditory brainstem response (a-ABR) and conventional brainstem auditory evoked potential (BAEP/ABR) for identification of hearing loss in high-risk neonates. Methods: Two hundred and six neonatal intensive care unit (NICU) admitted neonates were tested pre-discharge. Follow-up included a-TEOAE in all children, repetition of a-ABR or BAEP if failed in NICU. Sensitivity and specificity were compared and correlated with auditory risk factors. Results: BAEP had the highest sensitivity (100%) and specificity (90.8%), a-ABR the lowest (88.9% and 70.6%). A statistically significant difference in risk factors for temporary hearing loss was observed between normal and false positive a-TEOAE and BAEP, but not a-ABR outcome. Differences in specificity between a-ABR and a-TEOAE explain the pattern of ‘absent a-ABR/present a-TEOAE’ in 13.8% of ears. Conclusions: The BAEP appears the more reliable test for hearing screening of high-risk neonates because of highest sensitivity and specificity and should be used to confirm the diagnosis of ‘auditory neuropathy’ in high-risk neonates. The reliability of a-ABR devices in critically ill neonates needs further investigation. Significance: This is, to our knowledge, the first attempt to compare the diagnostic reliability of a-TEOAE, a-ABR and BAEP in high-risk neonates. [Copyright &y& Elsevier]

Summary: We investigated whether histological chorioamnionitis (HCA) is a risk factor predisposing to leukemoid reaction (LR) and whether LR is associated with the preterm parturition syndrome and the systemic fetal inflammation response syndrome. A prospective histological study on placentas was performed in preterm infants (≤32+6 weeks'' gestation) admitted to level III neonatal intensive care unit of Padua University from January 1998 to December 2003. Development of LR was related to evidence of HCA. Among the placentas of the 223 preterm examined infants, 61 (27%) were diagnosed with HCA. Overall incidence of LR was 5.3%. Of 61 preterm neonates with HCA, 8 (13%) developed LR; moreover, 4 (2%) of 162 preterm infants without HCA developed LR (odds ratio = 7.4). Neonates with both LR and HCA differ from neonates with LR and without HCA in occurrence of prolonged rupture of membranes (62.5% versus 0%, P < .01), mode of delivery (37.5% versus 100% cesarean delivery, P < .01), gestational age (26 ± 1.6 versus 29.7 ± 1.7 gestational weeks, P < .01), and bronchopulmonary dysplasia incidence (63% versus 25%, P < .01). Histological chorioamnionitis placental findings increase the risk for neonatal LR. The LR in low-birth-weight neonates with HCA is significantly associated with earlier delivery and bronchopulmonary dysplasia. Pathogenetic mechanisms of neonatal LR in low-birth-weight infants without HCA remain undefined. [Copyright &y& Elsevier]

Abstract: The role of histological chorioamnionitis in neonatal neurological outcome is not yet fully understood. The present study aimed to assess the neurodevelopmental outcome of preterm babies born after pregnancy complicated by histological chorioamnionitis. Clinical data were prospectively collected for consecutive premature neonates born before 32 weeks of gestation, admitted to Neonatal Intensive Care Unit of Padua University from January 1998 to December 2001. Placental histology was performed. Outcome at 18 months of corrected age was evaluated by a standardized postal parental questionnaire. Among 104 placentas examined, 41 (39.4%) were diagnosed with histological chorioamnionitis. Reply to the postal questionnaire was available from 76.1% of the families. The relative risk of disability in vision, hearing, speech and motor development was higher in the histological chorioamnionitis than in the non-histological chorioamnionitis group, with statistical significance in speech delay (relative risk 2.37; 95% confidence interval: 1.33–4.22) and hearing loss (relative risk 2.76; 95% confidence interval:1.64–4,64). To our knowledge this is the first report suggesting preterm histological chorioamnionitis as a possible risk factor for hearing loss and speech delay. [Copyright &y& Elsevier]

Objective: To evaluate clinical data and associated risk conditions of noncerebral systemic venous thromboembolism (VT), arterial thromboembolism (AT), and intracardiac thromboembolism (ICT) in neonates.Study Design: Data analysis of first systemic thromboembolism occurring in 75 live neonates (0-28 days), enrolled in the Italian Registry of Pediatric Thrombosis from neonatology centers between January 2007 and July 2013.Results: Among 75 events, 41 (55%) were VT, 22 (29%) AT, and 12 (16%) ICT; males represented 65%, and 71% were preterm. In 19 (25%), thromboembolism was diagnosed on the first day of life. In this "early onset" group, prenatal-associated risk conditions (maternal/placental disease) were reported in 70% and inherited thrombophilia in 33%. Postnatal risk factors were present in 73%; infections and central vascular catheters in 56% and 54% VT, respectively, and in 67% ICT vs 27% AT (<.05). Overall mortality rate was 15% and significant thromboembolism-related sequelae were reported in 16% of discharged patients.Conclusions: This report from the Registro Italiano Trombosi Infantili, although limited by representing an uncontrolled case series, can be used to develop future clinical trials on appropriate management and prevention of neonatal thrombosis, focusing on obstetrical surveillance and monitoring of critically ill neonates with vascular access. A thrombosis risk prediction rule specific for the neonatal population should be developed through prospective controlled studies. [ABSTRACT FROM AUTHOR]

Objective To present a case of LBSL with neonatal onset and with rapid progression and to review the literature focused on LBSL with early onset (before one year of age). Methods We report a case of an infant admitted at our Child Neurology Unit in November 2014 with LBSL, investigated with blood and cerebrospinal spinal fluid (CSF) tests, brain and spinal magnetic resonance imaging (MRI). Results The patient was admitted at our hospital at 2 months of age because of severe hypotonia. She had regular antenatal and perinatal story. At birth physical and neurological examination were normal. At two weeks of age nystagmus was noticed and after few weeks, she started to be hyporeactive and hypotonic. At our examination she had severe and diffuse hypotonia, brisk reflex at legs and nystagmus. CSF examination showed an increased lactate (7.4 mmol/L, range 1.2–2.2 mmol/L). Cerebral (including spectroscopy) and spinal MRI showed changes in white matter consistent with LBSL. Two weeks later she developed respiratory arrest and needed mechanical ventilatory support. Steroid therapy was administered without results. Genetic test is in progress. Conclusion LBSL is a rare disease described for the first time in 2003 by Van der Knaap et al. as a mild disorder with childhood and adolescent onset and slow progression. In the Literature we found two cases with neonatal onset, rapid deterioration and death respectively at 2 and 3 year of age and 16 cases with onset before 12 months (mean 8 months) and early death in 6/16 cases. We add to the pertinent literature a new case of LBSL with neonatal onset having a rapid and deteriorating course. This condition should be suspected in neonates or infants with leukoencephalopathy on brain MRI and lactic acidosis in CSF. Spinal MRI and specific genetic tests are needed to confirm the diagnosis. [ABSTRACT FROM AUTHOR]

Objective Given the rarity of this condition, especially in children, there is a paucity of literature reporting large case series of paediatric anti-N-methyl-D-aspartate receptor encephalitis. Our goal is to contribute to define the features of paediatric anti-N-methyl-D-aspartate receptor encephalitis. Methods We describe a new nationwide case series of 20 children (50% females), referred by 13 Italian centers. Results Mean age at onset was 8 years (range 3–17). Prodromal symptoms were reported in 31.6%; onset was with neurological symptoms in 70%, and with behavioral/psychiatric disturbances in 30%. Most patients developed a severe clinical picture (90%), and 41% experienced medical complications; children 12–18 years old seemed to be more severe and symptomatic than younger patients. All children received first-line immune therapy; second-line treatment was administered to 45%. Relapses occurred in 15%. Most patients recovered substantially (80%); this rate was higher among older patients, and in those receiving first immune therapy within 1 month. Conclusion Our case series confirms a symptomatologic core of paediatric anti-N-methyl-D-aspartate receptor encephalitis, even though displaying some distinctive features that may be explained by a specific genetic background or by the limited number of the patients. The growing incidence of this condition, the relative age-dependent variability of its manifestations, the availability of immunotherapy and the possible better outcome with early treatment impose a high index of clinical suspicion be maintained. Paediatricians should consider this diagnosis for children presenting with neurological and/or behavioral or psychiatric disturbances, regardless of age and gender, especially in the absence of data suggesting other specific etiologies. Other authors: F. Beccaria, L. Giunta, C. Boniver, M.G. Natali Sora, N. Zamponi. [ABSTRACT FROM AUTHOR]