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Brain & Development

Natan Gadoth
No abstract text is available yet for this article.
May 17, 2019: Brain & Development
Sachiyo Fukushima, Ichiro Morioka, Shohei Ohyama, Kosuke Nishida, Sota Iwatani, Kazumichi Fujioka, Tsurue Mandai, Hisayuki Matsumoto, Yuji Nakamachi, Masashi Deguchi, Kenji Tanimura, Kazumoto Iijima, Hideto Yamada
OBJECTIVE: This study aimed to evaluate the neurodevelopmental outcomes of infants with symptomatic congenital cytomegalovirus (SCCMV) disease after antiviral treatment and investigate the symptoms at birth associated with a developmental quotient (DQ) < 70. METHODS: In this prospective study conducted from 2009 to 2018, infants with SCCMV disease who received oral valganciclovir (VGCV; 32 mg/kg/day) for 6 weeks (November 2009 to June 2015) or 6 months (July 2015 to March 2018) were evaluated for their neurodevelopmental outcomes at around 18 months of corrected age...
May 7, 2019: Brain & Development
Eugenia Maranella
No abstract text is available yet for this article.
May 2, 2019: Brain & Development
Vania Aldrete-Cortez, Adrián Poblano, Silvia A Tafoya, Luz Angélica Ramírez-García, Cesar Casasola
BACKGROUND: The Polyvagal theory argues that behavioral modulation is a fundamental neurodevelopmental process that depends on autonomic regulation. OBJECTIVE: The present study aimed to assess sleep architecture in newborns with fetal growth restriction (FGR) using polysomnography as an indicator of Polyvagal theory. METHODS: We studied polysomnography recordings from 68 preterm infants, 34 with FGR and 34 born with appropriate growth for gestational age (AGA), who were matched according to the corrected age for prematurity (CA)...
May 2, 2019: Brain & Development
A Iodice, M Carecchio, G Zorzi, B Garavaglia, C Spagnoli, G G Salerno, D Frattini, N E Mencacci, F Invernizzi, L Veneziano, E Mantuano, M Angriman, C Fusco
No abstract text is available yet for this article.
April 30, 2019: Brain & Development
Longkai He, Wei Zhou, Xiaopeng Zhao, Xiaoping Liu, Xiao Rong, Yanyan Song
OBJECTIVE: We sought to develop and validate a novel scoring system for the prediction of severe intraventricular hemorrhage (SIVH) in very low birth weight infants (VLBWI). METHODS: This retrospective cohort multicenter study included 615 VLBWI born between 24 and 32 weeks gestational age (GA). Multivariable logistic regression analyses were used to determine which factors evaluated within the first 5 days of life were associated with SIVH and the weights of these variables...
April 30, 2019: Brain & Development
Yali Wang, Yitong Yuan, Yuantao Gao, Xiao Li, Feng Tian, Fang Liu, Ruochen Du, Pengfei Li, Fei Wang, Suming Xu, Xueqing Wu, Chunfang Wang
Apoptosis is a highly conservative energy demand program for non-inflammatory cell death, which is extremely significant in normal physiology and disease. There are many techniques used for studying apoptosis. MicroRNA (miRNA) is closely related to cell apoptosis, and especially microRNA-31 (miR-31) is involved in apoptosis by regulating a large number of target genes and signaling pathways. In many neurological diseases, cell apoptosis or programmed cell death plays an important role in the reduction of cell number, including the reduction of neurons in spinal cord injuries...
April 26, 2019: Brain & Development
Ayumi Matsumoto, Masako Nagashima, Kazuhiro Iwama, Takeshi Mizuguchi, Shinji Makino, Takahiro Ikeda, Kazuhiro Muramatsu, Naomichi Matsumoto, Takanori Yamagata, Hitoshi Osaka
INTRODUCTION: Neuronal ceroid lipofuscinoses (NCLs; CLN) are mainly autosomal recessive neurodegenerative disorders characterized by the accumulation of autofluorescent lipopigments in neuronal and other cells. Symptoms include visual disabilities, motor decline, and epilepsy. Causative genes are CLN1, CLN2, CLN3, CLN5, CLN6, CLN7, CLN8, CLN10, CLN11, CLN12, CLN13, and CLN14. We present the fourth Japanese case with a CLN6 mutation. CASE PRESENTATION: At 3 years of age, our patient became clumsy and fell down easily...
April 24, 2019: Brain & Development
Takuji Nakamura, Muneaki Matsuo
No abstract text is available yet for this article.
April 23, 2019: Brain & Development
Takuya Kosaka, Genrei Ohta, Hiroshi Kometani, Masao Kawatani, Yusei Ohshima
BACKGROUND: Early myoclonic encephalopathy (EME) is an epileptic syndrome that develops in neonates, commonly within 1 month of birth. The condition is characterized by irregular, partial, and asynchronous myoclonus. The seizures in EME are generally refractory to antiepileptic drugs and no effective treatment for EME has been established. We encountered a case of EME in which oral high-dose phenobarbital therapy effectively alleviated seizures. CASE REPORT: A male infant developed erratic myoclonus in the face and limbs, exhibited upward gaze and facial flushing 20-30 times a day since 1 week of age...
April 23, 2019: Brain & Development
Kyosuke Ibi, Katsunori Fujii, Hironobu Kobayashi, Masayoshi Senda, Katsuhiko Kitazawa, Akihito Honda
BACKGROUND: Non-paraneoplastic limbic encephalitis is characterized by attention deficit, loss of emotion control, and impaired memory. Viral infection can cause acute encephalitis in children, occasionally exhibiting clinical features of limbic dysfunction. However, how viral infection affects the limbic system remains to be elucidated. CASE DESCRIPTION: A 5-year-old Japanese boy was admitted to our hospital because of high fever and status epilepticus. After seizures were controlled by diazepam, he exhibited attention deficit, loss of emotion control, and impaired memory, suggesting acute limbic encephalitis...
April 17, 2019: Brain & Development
Yukie Arahata, Katsunori Fujii, Tatsuya Nishimura, Tomoko Uchida, Katsuhiko Kitazawa, Akihito Honda
BACKGROUND: Japanese encephalitis is a flavivirus that can cause pandemic encephalitis, and is prevalent in Southeast Asia and Australia. Brain images of patients with Japanese encephalitis are characterized by thalamic lesions, distinct from those seen in viral encephalopathies caused by the herpes simplex virus and West Nile virus. AIM: Herein, we describe for the first time a time-dependent magnetic resonance imaging pattern in Japanese encephalitis in a 10-month-old Japanese boy...
April 16, 2019: Brain & Development
Gonca Bektaş, Osman Kipoğlu, Edibe Pembegül Yıldız, Nur Aydınlı, Mine Çalışkan, Meral Özmen, Serra Sencer
PURPOSE: To identify the frequency of epilepsy and whether the association of epilepsy with clinical and neuroimaging findings in children with presumed perinatal arterial ischemic stroke (PPAIS). METHODS: We performed a retrospective analysis of 37 children with PPAIS followed-up at a tertiary referral center between January 1, 2000, and October 31, 2016. Clinical data including demographic features, age at onset of symptoms and seizures, initial clinical presentation, epilepsy features, used antiepileptic drugs, and thrombophilia screening results were abstracted from medical records...
April 16, 2019: Brain & Development
Marie Glombova, Borivoj Petrak, Jiri Lisy, Josef Zamecnik, David Sumerauer, Petr Liby
PURPOSE: To evaluate the incidence and clinical importance of brain gliomas - optic pathway gliomas (OPGs) and especially gliomas outside the optic pathway (GOOP) for children with neurofibromatosis type 1 (NF1), additionally, to assess the causes of obstructive hydrocephalus in NF1 children with an emphasis on cases caused by idiopathic aqueduct stenosis. SUBJECTS AND METHODS: We analysed data from 285 NF1 children followed up on our department from 1990 to 2010 by the same examination battery...
April 15, 2019: Brain & Development
Tomonori Ishiguro, Yuichiro Sugiyama, Kazuto Ueda, Yukako Muramatsu, Hiroyuki Tsuda, Tomomi Kotani, Toshimi Michigami, Kanako Tachikawa, Tomoyuki Akiyama, Masahiro Hayakawa
Hypophosphatasia (HPP) is a rare disorder caused by low serum tissue non-specific alkaline phosphatase (ALP) activity due to hypomorphic mutations in the ALPL gene. HPP is characterized by defective bone mineralization. It frequently accompanies pyridoxine-responsive seizures. Because alkaline phosphatase change pyridoxal 5' phosphate (PLP) into pyridoxal (PL), which can cross the blood brain barrier and regulates inhibitory neurotransmitter gamma-aminobutyric acid. The female patient was born at a gestational age of 37 weeks 2 days...
April 15, 2019: Brain & Development
Kenji Yamada, Michinori Ito, Hironori Kobayashi, Yuki Hasegawa, Seiji Fukuda, Seiji Yamaguchi, Takeshi Taketani
Multiple acyl-CoA dehydrogenase deficiency (MADD), also known as glutaric acidemia type II, is classically caused by a congenital defect in electron transfer flavoprotein (ETF) or ETF dehydrogenase (ETFDH). Flavin adenine dinucleotide synthase (FADS) deficiency caused by mutations in FLAD1 was recently reported as a novel riboflavin metabolism disorder resembling MADD. Here, we describe a Japanese boy with FADS deficiency due to a novel mutation (p.R249*) in FLAD1. In the asymptomatic male infant born at full term, newborn screening showed positive results with elevated C5 and C14:1 acylcarnitine levels and an increased C14:1/C2 ratio...
April 11, 2019: Brain & Development
Jung Min Ko, Woo Joong Kim, Soo Yeon Kim, Jun Hwa Lee, Jong Hee Chae, Ki Joong Kim, Byung Chan Lim
Herpes simplex encephalitis (HSE) is a widely accepted risk factor for anti N-methyl-d-aspartate receptor (NMDAR) encephalitis. Association of inherited metabolic disease has never been reported in a patient with HSE and anti-NMDAR encephalitis. Herein, we report a case of pediatric HSE complicated by development of anti-NMDAR encephalitis; this patient showed subsequent recurrent, unexplained episodes of encephalopathy associated with hyperammonemia. The patient was diagnosed with lysinuric protein intolerance (LPI), a rare inborn metabolic disorder...
April 5, 2019: Brain & Development
Yuichi Takami, Taku Nakagawa
OBJECTIVE: This study was performed to evaluate the efficacy and safety of intravenous phenobarbital (PB) for benign convulsions with mild gastroenteritis (CwG). METHODS: A randomized, single-blind, placebo-controlled trial involving patients with CwG was conducted at the Japanese Red Cross Society Himeji Hospital. Patients with CwG who had experienced two or more seizures were eligible. Patients were excluded if any anticonvulsant was used before enrollment. Patients who were allocated to the PB group were administered 10 mg/kg of PB intravenously...
April 3, 2019: Brain & Development
Robertino Dilena, Paola De Liso, Matteo Di Capua, Dario Consonni, Giuseppe Capovilla, Francesco Pisani, Agnese Suppiej, Giovanna Vitaliti, Raffaele Falsaperla, Dario Pruna
BACKGROUND: A targeted treatment approach is increasingly promoted in epilepsy management. AIM: To investigate if etiology (both established or initially presumed) influences antiepileptic drug choice of experts in neonatal seizures. METHODS: An invitation to participate to a web-based questionnaire was sent to Italian pediatric neurologists affiliated to the Italian Society of Pediatric Neurology (SINP). RESULTS: 19 pediatric neurologists from different centers, all consultants of third level Neonatal Intensive Care Units (NICUs) answered...
April 3, 2019: Brain & Development
Yan Xiang, Hua Han, Shengfeng Ji, Liang Wei, Pengbo Yang, Junfeng Zhang
BACKGROUNDS: Metabotropic glutamate receptors, besides ionotropic receptors, mediate the complicated effect of glutamate on neurogenesis. Previous studies showed that metabotropic glutamate receptor 4 (mGluR4) regulated the proliferation and differentiation of neural stem/progenitor cells in vitro. However, little is known about the expression pattern of mGluR4 on prenatal central nervous system in vivo, especially the human being. METHODS: The normal brain tissues of human fetus were collected and divided into 4 groups according to the gestational age: 9-11 W, 14-16 W, 22-24 W and 32-36 W...
April 3, 2019: Brain & Development
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