Developmental outcomes and prevalence of SLC2A1 variants in young infants with hypoglycorrhachia
The neurodevelopmental outcomes of young infants with hypoglycorrhachia that is comparable to glucose transporter 1 deficiency syndrome (GLUT1DS), i.e. cerebrospinal fluid (CSF) glucose ≤40 mg/dL and CSF lactate (Source: Brain and Development)
Source: Brain and Development - July 17, 2019 Category: Neurology Authors: Wen-Hao Yu, Li-Wen Chen, Shan-Tair Wang, Yi-Fang Tu, Chao-Ching Huang Tags: Original article Source Type: research

Breastfeeding and risk of febrile seizures in infants: The Japan Environment and Children ’s Study
Our study was conducted to examine the association between breastfeeding and febrile seizures (FS) in the first year of life. (Source: Brain and Development)
Source: Brain and Development - July 16, 2019 Category: Neurology Authors: Naomi Mitsuda, Takatoshi Hosokawa, Masamitsu Eitoku, Mikiya Fujieda, Narufumi Suganuma, Japan Environment and Children's Study (JECS) Group Tags: Original article Source Type: research

ARX-associated infantile epileptic-dyskinetic encephalopathy with responsiveness to valproate for controlling seizures and reduced activity of muscle mitochondrial complex IV
ARX genetic defect is associated with a spectrum of neurodevelopmental disorders that exhibit a high degree of phenotypic heterogeneity. (Source: Brain and Development)
Source: Brain and Development - July 16, 2019 Category: Neurology Authors: Anna Ka-Yee Kwong, Vanessa Loi-Yan Chu, Richard J.T. Rodenburg, Jan Smeitink, Cheuk-Wing Fung Tags: Case Report Source Type: research

Automatic calculation of Mercuri grades from CT and MR muscle images
Mercuri grading of muscle images is a useful method to evaluate the progression of muscular dystrophies. However, because Mercuri grading is skill-based, few competent experts are available. We therefore developed an automated method for Mercuri grade calculations. (Source: Brain and Development)
Source: Brain and Development - July 12, 2019 Category: Neurology Authors: Takahiro Nakayama, Akihiko Ishiyama, Terumi Murakami, En Kimura, Satoshi Kuru Tags: Original article Source Type: research

Single-fiber electromyography-based diagnosis of CACNA1A mutation in children: A potential role of the electrodiagnosis in the era of whole exome sequencing
A loss-of-function mutation in CACNA1A, which encodes P/Q-type Ca channels, causes various diseases. As most of the Ca channels at neuromuscular junctions are of the P/Q type, patients with loss-of-function CACNA1A mutations exhibit disturbed neuromuscular transmission. The associated jitters and blocking in such patients can be detected by single-fiber electromyography (SFEMG). (Source: Brain and Development)
Source: Brain and Development - July 6, 2019 Category: Neurology Authors: Ayaka Hirasawa-Inoue, Akihiko Ishiyama, Eri Takeshita, Yuko Shimizu-Motohashi, Takashi Saito, Hirofumi Komaki, Eiji Nakagawa, Shota Yuasa, Hirotomo Saitsu, Kohei Hamanaka, Satoko Miyatake, Naomichi Matsumoto, Masayuki Sasaki Tags: Case Report Source Type: research

Long-term outcome of a group of Japanese children with myelin-oligodendrocyte glycoprotein encephalomyelitis without preventive immunosuppressive therapy
There is increasing evidence that immunosuppressive therapy is essential for reducing disease activity and avoiding further attacks in patients positive for anti-myelin-oligodendrocyte glycoprotein (MOG) antibodies. However, to date, no placebo-controlled trial has been published. (Source: Brain and Development)
Source: Brain and Development - July 4, 2019 Category: Neurology Authors: Naomi Hino-Fukuyo, Kazuhiro Haginoya, Toshiyuki Takahashi, Ichiro Nakashima, Kazuo Fujihara, Yoshiki Takai, Manami Akasaka, Shigeo Kure Tags: Original article Source Type: research

A severe case of Menkes disease with repeated bone fracture during the neonatal period, followed by multiple arterial occlusion
We present an unusually severe case of MD with skull fractures at the birth and repeated fractures during the neonatal period, with further examinations leading to diagnosis. The patient died due to hemorrhagic shock, due to multiple arterial occlusion despite initiation of copper- histidine therapy in early infancy. (Source: Brain and Development)
Source: Brain and Development - July 3, 2019 Category: Neurology Authors: Satoru Kobayashi, Kyoko Yokoi, Naomi Kamioka, Naoki Hamajima, Kyoko Ban, Hiroko Kodama, Satoshi Suzuki Tags: Case Report Source Type: research

Novel RASA1 mutations in Japanese pedigrees with capillary malformation-arteriovenous malformation
Capillary malformation-arteriovenous malformation (CM-AVM, MIM#608354) is a rare autosomal dominant disorder characterized by multiple cutaneous capillary malformations co-occurring with fast-flow vascular anomalies, such as arteriovenous malformation or fistula. Despite the identification of RASA1 as the first causative gene in Western patients with CM-AVM, there have been no literature reports of Japanese patients with this gene mutation. We herein report two Japanese pedigrees harboring multiple affected members with CM-AVM. (Source: Brain and Development)
Source: Brain and Development - June 20, 2019 Category: Neurology Authors: Yosuke Moteki, Hiroyuki Akagawa, Yasunari Niimi, Yoshikazu Okada, Takakazu Kawamata Tags: Case Report Source Type: research

Gender differences in occurrence of behavioral and emotional problems at the lower grades of elementary school: Association with developmental and behavioral characteristics at 5  years
We examined whether developmental status and behavioral problems at age five were related to the occurrence of behavioral and emotional problems in the lower grades of elementary school among non-clinical children who did not receive specialized support for developmental problems in early childhood. (Source: Brain and Development)
Source: Brain and Development - June 18, 2019 Category: Neurology Authors: Yui Zen, Tomohiro Chiyonobu, Mariko Yuge, Isao Yokota, Masafumi Morimoto, Satoshi Teramukai, Hajime Hosoi Tags: Original article Source Type: research

Cumulative jerk as an outcome measure in nonambulatory Duchenne muscular dystrophy
Quantitative or semiquantitative outcome measures for patients with Duchenne muscular dystrophy (DMD) are important, as they can be objective indicators of the natural history of DMD; these measures also aid in the evaluation of the efficacy of various treatments. However, the most widely used standard outcome measures in patients with DMD, such as the North Star Ambulatory Assessment and the 6-min walk test, cannot be applied after patients have become nonambulatory. We evaluated the utility and reliability of accelerometric analysis of motor activity in nonambulatory patients with DMD. (Source: Brain and Development)
Source: Brain and Development - June 15, 2019 Category: Neurology Authors: Tatsuya Fujii, Eri Takeshita, Yasuyuki Iwata, Hiroyuki Yajima, Fumihito Nozaki, Mioko Mori, Tomohiro Kumada Tags: Original article Source Type: research

Central nervous system vasculitis from Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disorder in children: A case report
Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disorders (EBV-T/NK-LPD) is a group of rare disorders resulting from EBV-infected T/NK-cells. It manifests as a broad spectrum of clinical symptoms according to immunologic status and viral load of an infected patient. Here, we report a boy who developed central nervous system (CNS) vasculitis and myelopathy as possible neurologic manifestations of EBV-T/NK-LPD. (Source: Brain and Development)
Source: Brain and Development - June 14, 2019 Category: Neurology Authors: Mi Seon Lee, Su-Kyeung Hwang, Yeong Eun Kim, Jin Kyung Suh, Hyery Kim, So Mi Lee, Ji Yun Jeong, Soonhak Kwon, Yun Jeong Lee Tags: Case Report Source Type: research

Association of inattention with slow-spindle density in sleep EEG of children with attention deficit-hyperactivity disorder
We evaluated the power of slow sleep spindles during sleep stage 2 to clarify their relationship with executive function, especially with attention, in children with attention deficit-hyperactivity disorder (ADHD). (Source: Brain and Development)
Source: Brain and Development - June 13, 2019 Category: Neurology Authors: Yoshihiko Saito, Yoshimi Kaga, Eiji Nakagawa, Mariko Okubo, Kosuke Kohashi, Mikimasa Omori, Ayako Fukuda, Masumi Inagaki Tags: Original article Source Type: research

Usefulness of diagnostic tools in a GLUT1 deficiency syndrome patient with 2 inherited mutations
We report a child with 2 inherited mutations suggesting an autosomal recessive transmission of SLC2A1 mutations. (Source: Brain and Development)
Source: Brain and Development - June 10, 2019 Category: Neurology Authors: Blandine Dozi ères-Puyravel, Sasha Zaman, Steven Petrou, Laurent François, Sandrine Vuillaumier-Barrot, Fanny Mochel, Domitille Gras, Stéphane Auvin Tags: Case Report Source Type: research

Transition from Leigh syndrome to MELAS syndrome in a patient with heteroplasmic MT-ND3 m.10158T > C
An m.10158T>C mutation in MT-ND3, encoding a subunit of respiratory complex I, causes early-onset Leigh syndrome (LS), mitochondrial encephalomyopathy with lactic acid and stroke-like episodes (MELAS) syndrome, and LS and MELAS overlapping syndrome, presumably dependent on the ratio of heteroplasmy. Herein, we report a 4-year-old girl with heteroplasmic m.10158T>C mutation, showing an evolving age-dependent phenotype from LS to MELAS syndromes. She showed mild developmental delay during infancy, which was associated with magnetic resonance imaging lesions in the brain stem and basal ganglia. (Source: Brain and Development)
Source: Brain and Development - June 6, 2019 Category: Neurology Authors: Atsuko Kori, Ikumi Hori, Tatsushi Tanaka, Kohei Aoyama, Koichi Ito, Ayako Hattori, Kyoko Ban, Yasushi Okazaki, Kei Murayama, Shinji Saitoh Tags: Case Report Source Type: research

Genomic backgrounds of Japanese patients with undiagnosed neurodevelopmental disorders
Recently, many genes related to neurodevelopmental disorders have been identified by high-throughput genomic analysis; however, a comprehensive understanding of the mechanism underlying neurodevelopmental disorders remains to be established. To further understand these underlying mechanisms, we performed a comprehensive genomic analysis of patients with undiagnosed neurodevelopmental disorders. (Source: Brain and Development)
Source: Brain and Development - June 3, 2019 Category: Neurology Authors: Toshiyuki Yamamoto, Taichi Imaizumi, Keiko Yamamoto-Shimojima, Yongping Lu, Tomoe Yanagishita, Shino Shimada, Pin Fee Chong, Ryutaro Kira, Riyo Ueda, Akihiko Ishiyama, Eri Takeshita, Ken Momosaki, Shiro Ozasa, Tomoyuki Akiyama, Katsuhiro Kobayashi, Hiroo Tags: Original article Source Type: research

Reply to the comments on “Spontaneous spinal epidural hematoma mimicking Guillain-Barre Syndrome”
We appreciate your comments on our paper, “spontaneous spinal epidural hematoma mimicking Guillain-Barre Syndrome”. We would like to clarify some of your concerns. (Source: Brain and Development)
Source: Brain and Development - June 1, 2019 Category: Neurology Authors: Hiroshi Yamaguchi, Azusa Maruyama Tags: Commentary Source Type: research

Perampanel attenuates myoclonus in a patient with neuronal ceroid lipofuscinoses type 2 disease
Neuronal ceroid lipofuscinoses type 2 disease (CLN2) is a very rare, autosomal recessive neurodegerative disease caused by deficient activity of the enzyme tripeptidyl peptidase 1 (TPP1). The seizures in CLN2 are polymorphic and resistant to antiepileptic drugs. In particular, myoclonus (epileptic and non-epileptic) predominant as the disease progresses. Herein, we present a child of CLN2 disease, who had near-continuous myoclonus, and was subsequently attenuated by administration of Perampanel. (Source: Brain and Development)
Source: Brain and Development - May 21, 2019 Category: Neurology Authors: Lee Chin Wong, Chia-Jui Hsu, Wang-Tso Lee Source Type: research

Phenotypic manifestations between male and female children with CDKL5 mutations
Cyclin-dependent kinase-like 5 (CDKL5), which maps to chromosome Xp22.13 and contains 20 coding exons, has been recognized as the gene responsible for early-onset epileptic encephalopathy (EoEE). A retrospective study is carried out to analyze potential genotypic and phenotypic differences between male and female patients with CDKL5 mutations. (Source: Brain and Development)
Source: Brain and Development - May 20, 2019 Category: Neurology Authors: Jao-Shwann Liang, Hsin Huang, Jinn-Shyan Wang, Jyh-Feng Lu Tags: Original article Source Type: research

Letter to the editor
I have read with interest the recently published case report by Kondo et al. “spontaneous spinal epidural hematoma mimicking Guillain-Barre Syndrome” [1]. (Source: Brain and Development)
Source: Brain and Development - May 17, 2019 Category: Neurology Authors: Natan Gadoth Source Type: research

Prediction of poor neurological development in patients with symptomatic congenital cytomegalovirus diseases after oral valganciclovir treatment
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)   (Source: Brain and Development)
Source: Brain and Development - May 7, 2019 Category: Neurology Authors: 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 Tags: Original article Source Type: research

Fetal growth restriction: From Polyvagal theory to developmental impairments?
The Polyvagal theory argues that behavioral modulation is a fundamental neurodevelopmental process that depends on autonomic regulation. (Source: Brain and Development)
Source: Brain and Development - May 2, 2019 Category: Neurology Authors: Vania Aldrete-Cortez, Adri án Poblano, Silvia A. Tafoya, Luz Angélica Ramírez-García, Cesar Casasola Tags: Original article Source Type: research

Response to “Causal connection between methamphetamine and neurotoxicity not established”
We appreciate the letter by Medina-Kirchner and Torres [1]. Their suggestions improve the interpretation of our case. (Source: Brain and Development)
Source: Brain and Development - May 2, 2019 Category: Neurology Authors: Eugenia Maranella Tags: Letter to the Editor Source Type: research

Development and validation of a novel scoring system to predict severe intraventricular hemorrhage in very low birth weight infants
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). (Source: Brain and Development)
Source: Brain and Development - April 30, 2019 Category: Neurology Authors: Longkai He, Wei Zhou, Xiaopeng Zhao, Xiaoping Liu, Xiao Rong, Yanyan Song Tags: Original article Source Type: research

Corrigendum to “Restless legs syndrome in NKX2-1-related chorea: An expansion of the disease spectrum” [Brain Dev. 41 (2019) 250–256]
The authors would like to revise the printed version of the above article, because it contained an error regarding gene nomenclature in one mutation reported. In the section “Results” and in Table 2, we described a mutation in NKX2-1 (NM_001079668.2) c.300C>G p.Tyr100*, although c.300 is a G not a C and codon 100 is ATG coding for Met not Tyr. For that reason we would like to specify that the reported mutation was referred to the “short” form of the protein (NM_003317) while the correct numbering referred to the “long” form (NM_001079668.2) is p.Tyr130* c.390C>G. (Source: Brain and Development)
Source: Brain and Development - April 30, 2019 Category: Neurology Authors: 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 Tags: Corrigendum Source Type: research

MicroRNA-31 regulating apoptosis by mediating the phosphatidylinositol-3 kinase/protein kinase B signaling pathway in treatment of spinal cord injury
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. (Source: Brain and Development)
Source: Brain and Development - April 26, 2019 Category: Neurology Authors: Yali Wang, Yitong Yuan, Yuantao Gao, Xiao Li, Feng Tian, Fang Liu, Ruochen Du, Pengfei Li, Fei Wang, Suming Xu, Xueqing Wu, Chunfang Wang Tags: Review article Source Type: research

Editorial Board
(Source: Brain and Development)
Source: Brain and Development - April 25, 2019 Category: Neurology Source Type: research

Announcements and reports
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Source: Brain and Development - April 25, 2019 Category: Neurology Source Type: research

Cover
(Source: Brain and Development)
Source: Brain and Development - April 25, 2019 Category: Neurology Source Type: research

Contents
(Source: Brain and Development)
Source: Brain and Development - April 25, 2019 Category: Neurology Source Type: research

Rapid progression of a walking disability in a 5-year-old boy with a CLN6 mutation
We present the fourth Japanese case with a CLN6 mutation. (Source: Brain and Development)
Source: Brain and Development - April 24, 2019 Category: Neurology Authors: Ayumi Matsumoto, Masako Nagashima, Kazuhiro Iwama, Takeshi Mizuguchi, Shinji Makino, Takahiro Ikeda, Kazuhiro Muramatsu, Naomichi Matsumoto, Takanori Yamagata, Hitoshi Osaka Tags: Case Report Source Type: research

A case of early myoclonic encephalopathy with intractable seizures successfully treated with high-dose phenobarbital
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. (Source: Brain and Development)
Source: Brain and Development - April 23, 2019 Category: Neurology Authors: Takuya Kosaka, Genrei Ohta, Hiroshi Kometani, Masao Kawatani, Yusei Ohshima Tags: Case Report Source Type: research

Reply to the Letter, “Methotrexate myelopathy: A mimicker of subacute combined degeneration of the spinal cord”
Thank you for providing us with valuable comments regarding our manuscript entitled, “A case of subacute combined degeneration of the spinal cord due to folic acid and copper deficiency”. (Source: Brain and Development)
Source: Brain and Development - April 23, 2019 Category: Neurology Authors: Takuji Nakamura, Muneaki Matsuo Tags: Letter to the Editor Source Type: research

Anterior cingulate cortex involvement in non-paraneoplastic limbic encephalitis
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. (Source: Brain and Development)
Source: Brain and Development - April 17, 2019 Category: Neurology Authors: Kyosuke Ibi, Katsunori Fujii, Hironobu Kobayashi, Masayoshi Senda, Katsuhiko Kitazawa, Akihito Honda Tags: Case Report Source Type: research

Longitudinal magnetic resonance imaging changes in Japanese encephalitis
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. (Source: Brain and Development)
Source: Brain and Development - April 16, 2019 Category: Neurology Authors: Yukie Arahata, Katsunori Fujii, Tatsuya Nishimura, Tomoko Uchida, Katsuhiko Kitazawa, Akihito Honda Tags: Case Report Source Type: research

Epileptic spasm and other forms of epilepsy in presumed perinatal arterial ischemic stroke in Turkey after more than 10  years follow-up: A single centre study
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). (Source: Brain and Development)
Source: Brain and Development - April 16, 2019 Category: Neurology Authors: Gonca Bekta ş, Osman Kipoğlu, Edibe Pembegül Yıldız, Nur Aydınlı, Mine Çalışkan, Meral Özmen, Serra Sencer Tags: Original article Source Type: research

Brain gliomas, hydrocephalus and idiopathic aqueduct stenosis in children with neurofibromatosis type 1
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. (Source: Brain and Development)
Source: Brain and Development - April 15, 2019 Category: Neurology Authors: Marie Glombova, Borivoj Petrak, Jiri Lisy, Josef Zamecnik, David Sumerauer, Petr Liby Tags: Original article Source Type: research

Findings of amplitude-integrated electroencephalogram recordings and serum vitamin B6 metabolites in perinatal lethal hypophosphatasia during enzyme replacement therapy
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. (Source: Brain and Development)
Source: Brain and Development - April 15, 2019 Category: Neurology Authors: Tomonori Ishiguro, Yuichiro Sugiyama, Kazuto Ueda, Yukako Muramatsu, Hiroyuki Tsuda, Tomomi Kotani, Toshimi Michigami, Kanako Tachikawa, Tomoyuki Akiyama, Masahiro Hayakawa Tags: Case Report Source Type: research

Flavin adenine dinucleotide synthase deficiency due to FLAD1 mutation presenting as multiple acyl-CoA dehydrogenation deficiency-like disease: A case report
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 C1...
Source: Brain and Development - April 11, 2019 Category: Neurology Authors: Kenji Yamada, Michinori Ito, Hironori Kobayashi, Yuki Hasegawa, Seiji Fukuda, Seiji Yamaguchi, Takeshi Taketani Tags: Case Report Source Type: research

Hyperammonemia in a case of herpes simplex and anti-N-methyl-d-aspartate receptor encephalitis
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. (Source: Brain and Development)
Source: Brain and Development - April 5, 2019 Category: Neurology Authors: Jung Min Ko, Woo Joong Kim, Soo Yeon Kim, Jun Hwa Lee, Jong Hee Chae, Ki Joong Kim, Byung Chan Lim Tags: Case Report Source Type: research

Influence of etiology on treatment choices for neonatal seizures: A survey among pediatric neurologists
A targeted treatment approach is increasingly promoted in epilepsy management. (Source: Brain and Development)
Source: Brain and Development - April 3, 2019 Category: Neurology Authors: Robertino Dilena, Paola De Liso, Matteo Di Capua, Dario Consonni, Giuseppe Capovilla, Francesco Pisani, Agnese Suppiej, Giovanna Vitaliti, Raffaele Falsaperla, Dario Pruna Tags: Original article Source Type: research

The developmental expression of metabotropic glutamate receptor 4 in prenatal human frontal lobe and neurogenesis regions
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. (Source: Brain and Development)
Source: Brain and Development - April 3, 2019 Category: Neurology Authors: Yan Xiang, Hua Han, Shengfeng Ji, Liang Wei, Pengbo Yang, Junfeng Zhang Tags: Original article Source Type: research

Efficacy of phenobarbital for benign convulsions with mild gastroenteritis: A randomized, placebo-controlled trial
This study was performed to evaluate the efficacy and safety of intravenous phenobarbital (PB) for benign convulsions with mild gastroenteritis (CwG). (Source: Brain and Development)
Source: Brain and Development - April 3, 2019 Category: Neurology Authors: Yuichi Takami, Taku Nakagawa Tags: Original article Source Type: research

Epilepsy in patients with EAST syndrome caused by mutation in the KCNJ10
EAST syndrome comprises of epilepsy, ataxia, sensorineural deafness, and tubulopathy. It is caused by a mutation in KCNJ10 gene. Less than thirty cases have been reported in the literature with emphasis on genetic mutation and renal tubulopathy. In this article, our goal is to present a comprehensive description of epilepsy and its management. A literature review is also presented to consolidate and compare our findings with the previously reported cases. (Source: Brain and Development)
Source: Brain and Development - April 2, 2019 Category: Neurology Authors: Ali Mir, Mohammed Chaudhary, Hani Alkhaldi, Rami Alhazmi, Raidah Albaradie, Yousef Housawi Tags: Original article Source Type: research

Age-related differences in frontal lobe function in children with ADHD
The neural correlates of executive function disorders are thought to be predominantly localized within the prefrontal cortex (PFC). However, no study to date has investigated changes in this system across different age groups in children with attention deficit hyperactivity disorder (ADHD). Thus, this study aimed to explore changes in PFC function in children with ADHD. (Source: Brain and Development)
Source: Brain and Development - April 2, 2019 Category: Neurology Authors: Akira Yasumura, Mikimasa Omori, Ayako Fukuda, Junichi Takahashi, Yukiko Yasumura, Eiji Nakagawa, Toshihide Koike, Yushiro Yamashita, Tasuku Miyajima, Tatsuya Koeda, Masao Aihara, Masumi Inagaki Tags: Original article Source Type: research

Exclusive affection of the brain in m.4450G > A carriers rather suggests Leigh syndrome than MELAS
With interest we read the article by Kuwajima et al. about a 12yo female with MELAS due to the mtDNA variant m.4450G>A in the tRNA(Met) gene [1]. The study raises concerns. (Source: Brain and Development)
Source: Brain and Development - April 2, 2019 Category: Neurology Authors: Josef Finsterer Tags: Letter to the Editor Source Type: research

Editorial Board
(Source: Brain and Development)
Source: Brain and Development - March 31, 2019 Category: Neurology Source Type: research

Announcements and reports
(Source: Brain and Development)
Source: Brain and Development - March 31, 2019 Category: Neurology Source Type: research

Cover
(Source: Brain and Development)
Source: Brain and Development - March 31, 2019 Category: Neurology Source Type: research

Contents
(Source: Brain and Development)
Source: Brain and Development - March 31, 2019 Category: Neurology Source Type: research

Reply to comments on “The association between brain morphological development and the quality of general movements”
I appreciate the comments of Saini et al. on our paper. As they mentioned, the longitudinal general movements assessments (GMA) is more informative than the cross-sectional, and GMA in fidgety period is the most useful for predicting cerebral palsy. (Source: Brain and Development)
Source: Brain and Development - March 29, 2019 Category: Neurology Authors: Tomoki Maeda Tags: Opinion Source Type: research