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https://www.readbyqxmd.com/read/28728838/kcnq2-encephalopathy-a-case-due-to-a-de-novo-deletion
#1
Carlotta Spagnoli, Grazia Gabriella Salerno, Alessandro Iodice, Daniele Frattini, Francesco Pisani, Carlo Fusco
KCNQ2 encephalopathy is characterized by severely abnormal EEG, neonatal-onset epilepsy and developmental delay. It is caused by mutations (typically missense) in the KCNQ2 gene, encoding the voltage gated potassium channel Kv7.2 and leading to a negative-dominant effect. We present one case experiencing recurrent neonatal seizures with changing hemispheres of origin, reminiscent of epilepsy of infancy with migrating focal seizures. At 9months of age the patient is still seizure-free on carbamazepine, although he is developing a spastic-dystonic tetraplegia with severe dysphagia...
July 17, 2017: Brain & Development
https://www.readbyqxmd.com/read/28711233/review-of-sleep-eeg-in-preterm-and-term-neonates
#2
Anneleen Dereymaeker, Kirubin Pillay, Jan Vervisch, Maarten De Vos, Sabine Van Huffel, Katrien Jansen, Gunnar Naulaers
Neonatal sleep is a crucial state that involves endogenous driven brain activity, important for neuronal survival and guidance of brain networks. Sequential EEG-sleep analysis in preterm infants provides insights into functional brain integrity and can document deviations of the biologically pre-programmed process of sleep ontogenesis during the neonatal period. Visual assessment of neonatal sleep-EEG, with integration of both cerebral and non-cerebral measures to better define neonatal state, is still considered the gold standard...
July 12, 2017: Early Human Development
https://www.readbyqxmd.com/read/28691593/electrographic-seizures-in-preterm-neonates-in-the-neonatal-intensive-care-unit
#3
Ersida Buraniqi, Arnold J Sansevere, Kush Kapur, Ann M Bergin, Phillip L Pearl, Tobias Loddenkemper
OBJECTIVE: Characterize clinical and electroencephalography (EEG) characteristics of preterm neonates undergoing continuous EEG in the neonatal intensive care unit. METHODS: Retrospective study of preterm neonates born less than 37 weeks' gestational age undergoing continuous EEG in the neonatal intensive care unit at Boston Children's Hospital over a 2-year period. RESULTS: Fifty-two preterms (46% male) had a mean gestational age of 32.8 weeks (standard deviation = 4...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28687180/a-patient-with-early-myoclonic-encephalopathy-eme-with-a-de-novo-kcnq2-mutation
#4
Karin Kojima, Kentaro Shirai, Mizuki Kobayashi, Akihiko Miyauchi, Hirotomo Saitsu, Naomichi Matsumoto, Hitoshi Osaka, Takanori Yamagata
BACKGROUND: The potassium voltage-gated channel subfamily Q member 2 (KCNQ2) gene has been reported to be associated with various types of epilepsy, including benign familial neonatal seizure (BFNS), early infantile epileptic encephalopathy (EIEE), and unclassified early onset encephalopathies. We herein report a patient with early myoclonic encephalopathy (EME) caused by a KCNQ2 mutation. CASE REPORT: A male infant started to exhibit erratic myoclonus several days after birth and apnea attacks lasting for seconds with desaturation...
July 4, 2017: Brain & Development
https://www.readbyqxmd.com/read/28685437/relation-between-eeg-activity-and-brain-oxygenation-in-preterm-neonates
#5
Alexander Caicedo, Liesbeth Thewissen, Anne Smits, Gunnar Naulaers, Karel Allegaert, Sabine Van Huffel
This study investigates the relationship between brain oxygenation, assessed by means of near infrared spectroscopy (NIRS), and brain function, assessed by means of electroencephalography (EEG). Using NIRS signals measuring the regional cerebral oxygen saturation (rScO2) and computing the fractional tissue oxygen extraction (FTOE), we compared how these variables relate to different features extracted from the EEG, such as the inter-burst interval (IBI) duration and amplitude, the amplitude of the EEG, and the amplitude of the burst...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28660303/the-role-of-eeg-recordings-in-children-undergoing-cardiac-surgery-for-congenital-heart-disease
#6
Sascha Meyer, Martin Poryo, Mohammed Shatat, Ludwig Gortner, Hashim Abdul-Khaliq
BACKGROUND: Neurological dysfunction may occur after corrective cardiac surgery using cardio-pulmonary bypass (CPB) with or without circulatory arrest. Different neurophysiological monitoring systems have been employed to detect neurological complications and possible brain injury in infants and children during and after cardiac surgery. The value of Elecetroencephalogram (EEG) in infants and children at risk for neurological sequelae has not been systematically studied. METHODS: Sequential performance of two EEGs before and after cardiac surgery at a tertiary University Hospital to screen for possible brain injury after cardiac surgery in neonates and children undergoing CPB surgery...
June 28, 2017: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28644831/inflammatory-and-vascular-placental-lesions-are-associated-with-neonatal-amplitude-integrated-eeg-recording-in-early-premature-neonates
#7
Dorit Paz-Levy, Letizia Schreiber, Offer Erez, Sharon Goshen, Justin Richardson, VIadimir Drunov, Orna Staretz Chacham, Eilon Shany
INTRODUCTION: Placental histologic examination can assist in revealing the mechanism leading to preterm birth. Accumulating evidence suggests an association between intrauterine pathological processes, morbidity and mortality of premature infants, and their long term outcome. Neonatal brain activity is increasingly monitored in neonatal intensive care units by amplitude integrated EEG (aEEG) and indices of background activity and sleep cycling patterns were correlated with long term outcome...
2017: PloS One
https://www.readbyqxmd.com/read/28627083/a-review-of-important-eeg-features-for-the-assessment-of-brain-maturation-in-premature-infants
#8
REVIEW
Elena Pavlidis, Rhodri O Lloyd, Sean Mathieson, Geraldine B Boylan
This review describes the maturational features of the baseline electroencephalogram (EEG) in the neurologically healthy preterm infant. Features such as continuity, sleep state, synchrony and transient waveforms are described, even from extremely preterm infants and includes abundant illustrated examples. The physiological significance of these EEG features and their relationship to neurodevelopment is highlighted where known. This review also demonstrates the importance of multichannel conventional EEG monitoring for preterm infants as many of the features described are not apparent if limited channel EEG monitors are used...
June 18, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28623069/neonatal-hypoglycemia-a-wide-range-of-electroclinical-manifestations-and-seizure-outcomes
#9
Ebru Arhan, Zeynep Öztürk, Ayşe Serdaroğlu, Kürşad Aydın, Tuğba Hirfanoğlu, Yılmaz Akbaş
PURPOSE: We examined the various types of epilepsy in children with neonatal hypoglycemia in order to define electroclinical and prognostic features of these patients. METHOD: We retrospectively reviewed the medical records of patients with a history of symptomatic neonatal hypoglycaemia who have been followed at Gazi University Hospital Pediatric Neurology Department between 2006 and 2015. Patients with perinatal asphyxia were excluded. Details of each patient's perinatal history, neurological outcome, epilepsy details, seizure outcome and EEG and brain MRI findings were reviewed...
June 1, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28605362/recording-eeg-in-freely-moving-neonatal-rats-using-a-novel-method
#10
Qing-Wei Huo, Sidra Tabassum, Afzal Ali Misrani, Cheng Long
EEG is a useful method to detect electrical activity in the brain. Moreover, it is a widely used diagnostic tool for various neurological conditions, such as epilepsy and neurodegenerative disorders. However, it is technically difficult to obtain EEG recordings in neonates as it requires specialized handling and great care. Here, we present a novel method to record EEG in neonatal rat pups (P8-P15). We designed a simple and reliable electrode using computer pin loci; it can be easily implanted into the skull of a rat pup to record high-quality EEG signals in the normal and epileptic brain...
May 29, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28587899/anticonvulsant-effect-of-flupirtine-in-an-animal-model-of-neonatal-hypoxic-ischemic-encephalopathy
#11
Dayalan Sampath, Robert Valdez, Andrew M White, Yogendra H Raol
Research studies suggest that neonatal seizures, which are most commonly associated with hypoxic-ischemic injury, may contribute to brain injury and adverse neurologic outcome. Unfortunately, neonatal seizures are often resistant to treatment with current anticonvulsants. In the present study, we evaluated the efficacy of flupirtine, administered at clinically relevant time-points, for the treatment of neonatal seizures in an animal model of hypoxic-ischemic injury that closely replicates features of the human syndrome...
June 3, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28576730/short-term-deep-brain-stimulation-of-the-thalamic-reticular-nucleus-modifies-aberrant-oscillatory-activity-in-a-neurodevelopment-model-of-schizophrenia
#12
Víctor Manuel Magdaleno-Madrigal, Gerardo Contreras-Murillo, Israel Camacho-Abrego, José Vicente Negrete-Díaz, Alejandro Valdés-Cruz, Rodrigo Fernández-Mas, Salvador Almazán-Alvarado, Gonzalo Flores
Dysfunction of thalamo-cortical networks involving particularly the thalamic reticular nucleus (TRN) is implicated in schizophrenia. In the neonatal ventral hippocampal lesion (NVHL), a heuristic animal model of schizophrenia, brain oscillation changes similar to those of schizophrenic patients have been reported. The aim of this study was to analyze the effects of short-term deep brain stimulation (DBS) in the thalamic reticular nucleus on electroencephalographic (EEG) activity in the NVHL. Male and female Sprague-Dawley rats were used and the model was prepared by excitotoxicity damage of the ventral hippocampus on postnatal day 7 (PD-7)...
May 31, 2017: Neuroscience
https://www.readbyqxmd.com/read/28551036/a-patient-with-muenke-syndrome-manifesting-migrating-neonatal-seizures
#13
Yukimune Okubo, Taro Kitamura, Mai Anzai, Wakaba Endo, Takehiko Inui, Yusuke Takezawa, Sato Suzuki-Muromoto, Takuya Miyabayashi, Noriko Togashi, Hiroshi Oba, Hirotomo Saitsu, Naomichi Matsumoto, Kazuhiro Haginoya
We report a patient with Muenke syndrome who had repetitive apneic spell followed by focal status epilepticus in the early infancy. Ictal EEG showed focal spikes bursts originated from the left hemisphere and sifted to the right hemisphere, during which he had migrating tonic seizures from right side of the body to the left side of the body. Brain MRI showed abnormal development of bilateral hippocampus, which was characterized as abnormal folding of hippocampal gyri. However, the long-term seizure prognosis was favorable...
May 24, 2017: Brain & Development
https://www.readbyqxmd.com/read/28549636/amplitude-integrated-electroencephalography-improves-the-identification-of-infants-with-encephalopathy-for-therapeutic-hypothermia-and-predicts-neurodevelopmental-outcomes-at-2-years-of-age
#14
Janne Helen Skranes, Gro Løhaugen, Eva Margrethe Schumacher, Damjan Osredkar, Andres Server, Frances Mary Cowan, Tom Stiris, Drude Fugelseth, Marianne Thoresen
OBJECTIVES: To examine whether using an amplitude-integrated electroencephalography (aEEG) severity pattern as an entry criterion for therapeutic hypothermia better selects infants with hypoxic-ischemic encephalopathy and to assess the time-to-normal trace for aEEG and magnetic resonance imaging (MRI) lesion load as 24-month outcome predictors. STUDY DESIGN: Forty-seven infants meeting Norwegian therapeutic hypothermia guidelines were enrolled prospectively. Eight-channel EEG/aEEG was recorded from 6 hours until after rewarming, and read after discharge...
May 23, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28506426/heterogeneity-of-fhf1-related-phenotype-novel-case-with-early-onset-severe-attacks-of-apnea-partial-mitochondrial-respiratory-chain-complex-ii-deficiency-neonatal-onset-seizures-without-neurodegeneration
#15
Nathalie Villeneuve, Affef Abidi, Pierre Cacciagli, Cécile Mignon-Ravix, Brigitte Chabrol, Laurent Villard, Mathieu Milh
INTRODUCTION/OBJECTIVES: We report the case of a child prospectively followed in our institution for a severe, neonatal onset epilepsy presenting with severe attacks of apnea that were not initially recognized as seizure since they were not associated with any abnormal movement and since interictal EEG was normal. Recording of attacks using prolonged video-EEG recording allowed to confirm the diagnosis of epileptic seizures. RESULTS: Using whole exome sequencing we found a de novo heterozygous, missense mutation of FHF1 (p...
April 29, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28504308/brainstem-as-a-developmental-gateway-to-social-attention
#16
Ronny Geva, Ayelet Dital, Dan Ramon, Jessica Yarmolovsky, Maor Gidron, Jacob Kuint
BACKGROUND: Evolution preserves social attention due to its key role in supporting survival. Humans are attracted to social cues from infancy, but the neurobiological mechanisms for the development of social attention are unknown. An evolutionary-based, vertical-hierarchical theoretical model of self-regulation suggests that neonatal brainstem inputs are key for the development of well-regulated social attention. METHODS: Neonates born preterm (N = 44, GA 34 w...
May 15, 2017: Journal of Child Psychology and Psychiatry, and Allied Disciplines
https://www.readbyqxmd.com/read/28504050/how-long-is-sufficient-for-optimal-neuroprotection-with-cerebral-cooling-after-ischemia-in-fetal-sheep
#17
Joanne O Davidson, Vittoria Draghi, Sean Whitham, Simerdeep K Dhillon, Guido Wassink, Laura Bennet, Alistair J Gunn
The optimal duration of mild "therapeutic" hypothermia for neonates with hypoxic-ischemic encephalopathy is surprisingly unclear. This study assessed the relative efficacy of cooling for 48 h versus 72 h. Fetal sheep (0.85 gestation) received sham ischemia (n = 9) or 30 min global cerebral ischemia followed by normothermia (n = 8) or delayed hypothermia from 3 h to 48 h (n = 8) or 72 h (n = 8). Ischemia was associated with profound loss of electroencephalogram (EEG) power, neurons in the cortex and hippocampus, and oligodendrocytes and myelin basic protein expression in the white matter, with increased Iba-1-positive microglia and proliferation...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28503704/use-of-eeg-in-critically-ill-children-and-neonates-in-the-united-states-of-america
#18
Marina Gaínza-Lein, Iván Sánchez Fernández, Tobias Loddenkemper
The objective of the study was to estimate the proportion of patients who receive an electroencephalogram (EEG) among five common indications for EEG monitoring in the intensive care unit: traumatic brain injury (TBI), extracorporeal membrane oxygenation (ECMO), cardiac arrest, cardiac surgery and hypoxic-ischemic encephalopathy (HIE). We performed a retrospective cross-sectional descriptive study utilizing the Kids' Inpatient Database (KID) for the years 2010-2012. The KID is the largest pediatric inpatient database in the USA and it is based on discharge reports created by hospitals for billing purposes...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28482764/amplitude-integrated-electroencephalography-compared-with-conventional-video-eeg-for-neonatal-seizure-detection-a-diagnostic-accuracy-study
#19
Abhijeet Rakshasbhuvankar, Shripada Rao, Linda Palumbo, Soumya Ghosh, Lakshmi Nagarajan
This diagnostic accuracy study compared the accuracy of seizure detection by amplitude-integrated electroencephalography with the criterion standard conventional video EEG in term and near-term infants at risk of seizures. Simultaneous recording of amplitude-integrated EEG (2-channel amplitude-integrated EEG with raw trace) and video EEG was done for 24 hours for each infant. Amplitude-integrated EEG was interpreted by a neonatologist; video EEG was interpreted by a neurologist independently. Thirty-five infants were included in the analysis...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28463933/brief-potentially-ictal-rhythmic-discharges-b-i-rds-in-noncritically-ill-adults
#20
Ji Yeoun Yoo, Lara V Marcuse, Madeline C Fields, Jillian L Rosengard, Maria Vittoria Traversa, Nicolas Gaspard, Lawrence J Hirsch
INTRODUCTION: Brief potentially ictal rhythmic discharges (B(I)RDs) have been described in neonates and critically ill adults, and their association with seizures has been demonstrated. Their significance in noncritically ill adults remains unclear. We aimed to investigate their prevalence, electrographic characteristics, and clinical significance. METHODS: We identified adult patients with B(I)RDs who received long-term EEG recordings either in the epilepsy monitoring unit or in the ambulatory setting...
May 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
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