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Announcements and reportsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - September 2, 2009 Category: Neurology Source Type: journals

ContentsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - September 2, 2009 Category: Neurology Source Type: journals

Editorial BoardEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - September 2, 2009 Category: Neurology Source Type: journals

Announcement: The 2010 JSCN International Education FellowshipEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - August 4, 2009 Category: Neurology Source Type: journals

Announcements and reportsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - August 4, 2009 Category: Neurology Source Type: journals

ContentsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - August 4, 2009 Category: Neurology Source Type: journals

Editorial BoardEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - August 4, 2009 Category: Neurology Source Type: journals

ContentsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - July 23, 2009 Category: Neurology Source Type: journals

Announcements and reportsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - July 7, 2009 Category: Neurology Source Type: journals

OBC (Contents)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - July 7, 2009 Category: Neurology Source Type: journals

Special Issue Title PageEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - July 7, 2009 Category: Neurology Source Type: journals

Editorial BoardEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - July 7, 2009 Category: Neurology Source Type: journals

Founders of child neurology in Japan – Kazuo BabaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Professor Kazuo Baba was born in 1920 in Tokyo. He graduated from Tokyo University School of Medicine in 1944. Following graduation, he served on the navy hospital medical staff, and then went on to join the Tokyo University Department of Pediatrics in 1945. In addition to his affiliations with the Department of Pediatrics, Komagome Metropolitan Hospital and the Sanikukai Hospital as director of the pediatric department, he assumed a position as an assistant professor at the Tokyo University in 1956 and became an associate professor in 1959. Later, he moved to the Nihon University to become the chairman and professor of th...
Source: Brain & Development - June 11, 2009 Category: Neurology Authors: Yukihiko Fujita Tags: Founders Source Type: journals

Early neuropsychological signs of childhood adrenoleukodystrophy (ALD)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Conclusions: Follow-up of asymptomatic ALD boys with neuropsychological tests along with previously reported neurophysiological tests may help in determining the timing of therapeutic intervention. Neuropsychological abnormalities seemed to precede the appearance of clinical and MRI alterations.
Source: Brain & Development - June 4, 2009 Category: Neurology Authors: Makiko Kaga, Wakana Furushima, Masumi Inagaki, Masako Nakamura Tags: Original articles Source Type: journals

Child & brain – Evidence based practice meets neuroscience 3rd German–Japanese Symposium on Pediatric Neurology, 2008, Munich, GermanyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
It was for the third time that the Japanese and German pediatric neurologists got together for a lively scientific dialog and exchange, this time in Munich, Germany. The meeting was hosted by the German Society of Neuropediatrics and the Medical School of the Ludwig-Maximilian’s University – one of the largest and most prestigious academic institutions in Germany. Lectures and discussion seminars covered a wide range of topics such as disorders of the grey and white matter, apoptosis, neuroprotection, clinical system physiology, brain development, cerebral palsy, and epilepsy.
Source: Brain & Development - June 1, 2009 Category: Neurology Authors: Florian Heinen Tags: Short notes Source Type: journals

Sodium and potassium channel dysfunctions in rare and common idiopathic epilepsy syndromesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Mutations in the SCN1A gene are found in up to 80% of individuals with severe myoclonic epilepsy of infancy (SMEI), and mutations in KCNQ2 and KCNQ3 were identified in benign familial neonatal convulsions (BFNC) as well as in single families with Rolandic epilepsy (RE) and idiopathic generalized epilepsies (IGE). This paper summarizes recent findings concerning sodium (SCN1A) and potassium channel (KCNQ2 and KCNQ3) dysfunctions in the pathogenesis of rare and common idiopathic epilepsies (IE). SMEI, severe idiopathic generalized epilepsy of infancy (SIGEI), and myoclonic–astatic epilepsy (MAE) are rare IE. Beca...
Source: Brain & Development - May 25, 2009 Category: Neurology Authors: Andreas Hahn, Bernd A. Neubauer Tags: Review articles Source Type: journals

Genetic regulation of proliferation/differentiation characteristics of neural progenitor cells in the developing neocortexEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Brain size variation among different mammals is tightly associated with different levels of cerebral function. Mechanisms that regulate the number of neurons and hence the size of the brain must be at least partially embedded within the very early phase of neocortical development, that is, embedded in proliferation/differentiation characteristics of the neural progenitor cells (NPCs) of the neocortex. Here we review a sequence of critical events through which the neocortex is formed in the embryonic forebrain, with particular emphasis on cell cycle kinetics of the NPCs that produce non-GABAergic projection neuron...
Source: Brain & Development - May 25, 2009 Category: Neurology Authors: Takayuki Mitsuhashi, Takao Takahashi Tags: Review articles Source Type: journals

Perspective of Child Neurology in JapanEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Japan is facing major socioeconomic changes which include rapid aging and dwindling birth rate. Given such a situation, Japan has been promoting activities for fundamental structural reforms in diverse areas especially national universities, post-graduate clinical training systems, educational systems for children with disabilities and medical and long-term nursing care insurance systems. But after these structural reforms, several problems have emerged. In this paper the problems and perspectives associated with child neurology in present-day Japan are discussed.
Source: Brain & Development - May 14, 2009 Category: Neurology Authors: Kousaku Ohno Tags: Short notes Source Type: journals

IBCEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - May 7, 2009 Category: Neurology Source Type: journals

OBC (Contents)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - May 7, 2009 Category: Neurology Source Type: journals

Announcements and reportsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - May 7, 2009 Category: Neurology Source Type: journals

Effective prophylactic therapy for cyclic vomiting syndrome in children using valproateEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: This trial sought to evaluate our experience using the antimigraine prophylactic drug, use of valproate for the prophylactic management of cyclic vomiting syndrome (CVS) in children. Thirteen children diagnosed with severe CVS were enrolled. Prophylactic therapy consisted of valproate administered at a dose of 10–40mg/kg/day. Upon enrollment in the study, all patients underwent diagnostic tests to rule out organic causes of their symptoms. Vomiting was severe enough in all patients to cause dehydration requiring hospitalization for intravenous rehydration. Nine of 13 patients did not respond to numerous previou...
Source: Brain & Development - May 7, 2009 Category: Neurology Authors: Toshiyuki Hikita, Hiroko Kodama, Natsue Nakamoto, Fumiaya Kaga, Kaori Amakata, Kaori Ogita, Sono Kaneko, Yasushi Fujii, Yukishige Yanagawa Tags: Original articles Source Type: journals

Editorial BoardEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - May 7, 2009 Category: Neurology Source Type: journals

The role of hypoxia-inducible transcription factors in the hypoxic neonatal brainEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Hypoxia-inducible transcription factors (HIF)-1 and HIF-2, composed of an oxygen-dependent α-subunit and a constitutive β-subunit, have been characterized as the most important regulators of oxygen homeostasis during physiological and pathological conditions. During embryonic, fetal and postnatal brain development, HIFs and specific HIF target genes are involved in early and highly active maturational processes by modulating cell differentiation, vascular development, angiogenesis and metabolic homeostasis. Under hypoxic conditions, activation of the HIF system reflects an immediate and cell-specific response t...
Source: Brain & Development - April 27, 2009 Category: Neurology Authors: Regina Trollmann, Max Gassmann Tags: Review articles Source Type: journals

Review of Alexander disease: Beyond the classical concept of leukodystrophyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Alexander disease is classified as one of the leukodystrophies, which are degenerative diseases primarily affecting the cerebral white matter. Formal diagnosis is achieved by showing diffuse accumulation of Rosenthal fibers in the brain by biopsy or autopsy. Showing a heterozygous mutation in the glial fibrillary acidic protein (GFAP) gene is currently sufficient for diagnosis. The mechanisms of Rosenthal fiber formation remain unclear. However, both the quality and quantity of GFAP are important. GFAP-ε (rodent homologous GFAP-δ), one of the alternatively spliced GFAP isoforms, may also play a modulating role ...
Source: Brain & Development - April 22, 2009 Category: Neurology Authors: Yukio Sawaishi Tags: Review articles Source Type: journals

Cerebral palsy updateEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: A common language on CP has been developed for the European registers by the SCPE (Surveillance of Cerebral Palsy in Europe) working group and the common database allows prevalence analyses on a larger basis. CP prevalence increases with lower birthweight and higher immaturity. Increase of survival after preterm birth has first also increased CP rates. But already in the 80s this trend was reversed for LBW infants, and in the 90s also for VLBW or very immature infants. The outcome with respect to CP in the group of extremely LBW or immature infants remains a matter of specific concern, as prevalence seems to be r...
Source: Brain & Development - April 22, 2009 Category: Neurology Authors: Ingeborg Krägeloh-Mann, Christine Cans Tags: Review articles Source Type: journals

Announcements and reportsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - April 20, 2009 Category: Neurology Source Type: journals

IBCEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - April 20, 2009 Category: Neurology Source Type: journals

OBC (Contents)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - April 20, 2009 Category: Neurology Source Type: journals

Molecular basis of severe myoclonic epilepsy in infancyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Severe myoclonic epilepsy (SMEI) or Dravet syndrome is caused by mutations of the SCN1A gene that encodes voltage-gated sodium channel alpha-1 subunit. Recently, we generated and characterized a knock-in (KI) mice with an SCN1A nonsense mutation that appeared in three independent SMEI patients. The SCN1A-KI mice well reproduced the SMEI disease phenotypes. Both homozygous and heterozygous knock-in mice developed epileptic seizures within the first postnatal month. In heterozygous knock-in mice, trains of evoked action potentials in inhibitory neurons exhibited pronounced spike amplitude decrement late in the burs...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Kazuhiro Yamakawa Tags: Review articles Source Type: journals

Dravet syndrome or genetic (generalized) epilepsy with febrile seizures plus?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Dravet syndrome and genetic epilepsy with febrile seizures plus (GEFS+) can both arise due to mutations of SCN1A, the gene encoding the alpha 1 pore-forming subunit of the sodium channel. GEFS+ refers to a familial epilepsy syndrome where at least two family members have phenotypes that fit within the GEFS+ spectrum. The GEFS+ spectrum comprises a range of mild to severe phenotypes varying from classical febrile seizures to Dravet syndrome. Dravet syndrome is a severe infantile onset epilepsy syndrome with multiple seizure types, developmental slowing and poor outcome. More than 70% of patients with Dravet syndro...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Ingrid E. Scheffer, Yue-Hua Zhang, Floor E. Jansen, Leanne Dibbens Tags: Review articles Source Type: journals

The role of interleukin-1β in febrile seizuresEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Febrile seizures (FS) occur in children as a result of fever. Despite their prevalence, the pathophysiology of FS has remained unclear. Recent evidence from clinical and experimental studies has highlighted a potential role of immune generated products in the genesis of FS. Of particular interest are the pro-inflammatory cytokine, interleukin-1beta (IL-1β) and its naturally occurring antagonist, interleukin 1 receptor antagonist (IL-1ra). Using a novel animal model of FS, involving the generation of physiological fever, we investigated the role of the IL-1β/IL-1ra system in the genesis of FS. We found that anim...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: James G. Heida, Solomon L. Moshé, Quentin J. Pittman Tags: Review articles Source Type: journals

The long-term effects of febrile seizures on the hippocampal neuronal plasticity – Clinical and experimental evidenceEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Febrile seizures are the most common seizure disorder in childhood, but their long-term effects on the developing brains especially neuronal injury and neurocognitive function remain unresolved. Recent epidemiological studies reassure that most febrile seizures do not adversely affect global intelligence and hippocampal function, such as memory. However, there are concerns regarding those children who experience febrile seizures during the first postnatal year, having prior developmental delay and pre- or peri-natal events. Magnetic resonance imaging (MRI) studies confirmed that prolonged and focal FS can occasio...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Chao-Ching Huang, Ying-Chao Chang Tags: Review articles Source Type: journals

Neurobiological and physiological mechanisms of fever-related epileptiform syndromesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Febrile seizures (FS) are the most common type of convulsive events in children. FS have been extensively studied using animal models, where rat and mice pups are placed in a hyperthermic environment. Such work has largely focused on the consequences rather than on the mechanisms of experimental febrile seizures (eFS). We have recently shown that eFS are preceded by a dramatic rise in the rate of respiration. The consequent respiratory alkalosis affecting the brain and increasing neuronal excitability is a direct cause of the eFS . If a similar mechanism contributes to human FS and other fever-related epileptifor...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Sebastian Schuchmann, Sampsa Vanhatalo, Kai Kaila Tags: Review articles Source Type: journals

Register-based studies on febrile seizures in DenmarkEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: During a short period of brain development, one out of 25 children experience seizures when exposed to fever. The risk and consequences of these febrile seizures remain incompletely understood. We have conducted a number of studies within a population-based cohort of 1.6 million children born in Denmark (1977–2004). We constructed the cohort by linking registers on civil service, health, and cause of death. We followed the cohort for up to 28years with virtually no loss to follow-up. The aetiology of febrile seizures depends on a genetic susceptibility that can be transmitted through both parents. The risk of f...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Mogens Vestergaard, Jakob Christensen Tags: Review articles Source Type: journals

Febrile seizures: Mechanisms and relationship to epilepsyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Studies of febrile seizures have been driven by two major enigmas: first, how these most common of human seizures are generated by fever has not been known. Second, epidemiological studies have linked prolonged febrile seizures with the development of temporal lobe epilepsy, yet whether long or recurrent febrile seizures cause temporal lobe epilepsy has remained unresolved. To investigate these questions, a model of prolonged (complex) febrile seizures was developed in immature rats and mice, permitting mechanistic examination of the potential causal relationships of fever and seizures, and of febrile seizures an...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Céline M. Dubé, Amy L. Brewster, Tallie Z. Baram Tags: Review articles Source Type: journals

Progress in searching for the febrile seizure susceptibility genesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Febrile seizures (FS) represent the most common form of childhood seizures. They affect 2–5% of infants in the Caucasian population and are even more common in the Japanese population, affecting 6–9% of infants. Some familial FS are associated with a wide variety of afebrile seizures. Generalized epilepsy with febrile seizures plus (GEFS+) is a familial epilepsy syndrome with a spectrum of phenotypes including FS, atypical FS (FS+) and afebrile seizures. A significant genetic component exists for susceptibility to FS and GEFS+: extensive genetic studies have shown that at least nine loci are responsible for F...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Junko Nakayama Tags: Review articles Source Type: journals

Special Section Title PageEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - April 20, 2009 Category: Neurology Tags: SPECIAL SECTION Source Type: journals

Neuroimaging findings of Sturge–Weber Syndrome in a child with Tuberous SclerosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We describe a new patient with confirmed diagnosis of TSC and MRI appearance of SWS and review the pertinent literature. We discuss these findings on the basis of the new classifications of brain malformations, which take into account the role of neural-crest. The coexistence of signs of both diseases in the same individuals could be explained by common altered pathways that could lead to an anomalous angiogenesis.
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: P. Curatolo, A. Lo-Castro, M. Pinci, R. Moavero, R. Bombardieri Tags: Case reports Source Type: journals

Professor Norimitsu Yoshikura (1902–1988). A leading Japanese authority in paediatric neurologyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Dr. Norimitsu Yoshikura, Professor of Pediatrics, Surugadai Hospital, Nihon University School of Medicine, was born in Tokyo on 18th January, 1902. He first graduated from the Faculty of Literature, Waseda University, and thereafter from the Jikei University School of Medicine in 1933. He was a member of the committee of the Japanese Pediatric Neurology Association from 1964 to 1976, and an honorary member of the French Neurological Society. In 1966 he was president at the 6th annual meeting of the Japanese Society of Child Neurology. He retired from Nihon University in 1967 and became visiting Professor at the Department ...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Komei Kumagai Tags: Founders Source Type: journals

Early surgery of hamartoma of the floor of the fourth ventricle: A case reportEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report a 3-month-old boy with episodes of medically intractable abnormal eyelid blinking and hemifacial seizure. The episodes began from the first day of life and were unresponsive to medication. A magnetic resonance imaging scan revealed mass lesion on the floor of fourth ventricle, with extended cerebellar peduncle and cerebellar hemisphere. Surgery had been performed two times previously for treatment; only a subtotal resection was performed due to severe bradycardia during the first operation. The patient underwent second operation for gross total removal of tumor. Complete resection of the mass after second surgery...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Young Seok Park, Min-Chul Oh, Heung Dong Kim, Dong-Seok Kim Tags: Case reports Source Type: journals

Merosin-deficient congenital muscular dystrophy in KoreaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report, for the first time in Korea, eight patients with merosin-deficient CMD, confirmed by immunohistochemical staining of muscle or skin samples. We also describe their wide spectrum of clinical features and neuroimaging findings. Among 35 patients diagnosed as CMD, almost 23% of them were proved to have MDCMD with typical phenotypic presentation. We infer that prevalence of MDCMD in Korea may not be as low as expected. One of the patients was diagnosed by skin biopsy, which is good alternative for diagnosis of MDCMD.
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Jong-Hee Chae, Jin Sook Lee, Hee Hwang, Ki Joong Kim, Yong Seung Hwang, June Dong Park, Jung-Eun Cheon, In-One Kim, Ghee Young Choe, Sung Hye Park Tags: Original articles Source Type: journals

Editorial BoardEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Source: Brain & Development - April 20, 2009 Category: Neurology Source Type: journals

Basic fibroblast growth factor stimulates the proliferation and differentiation of neural stem cells in neonatal rats after ischemic brain injuryEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: A little is known about the proliferation and fate of neural stem cells in the subventricular zone (SVZ) after cerebral ischemia. However, how endogenous neural stem cells are activated in the premature brain is not clear, although basic fibroblast growth factor (bFGF) is important in neurogenesis. To investigate the effect of bFGF on the proliferation and differentiation of neural stem cells after brain ischemia, we observed cellular changes in the subventricular zone (SVZ) of 3-day-old rats (approximately equivalent to premature infants) using immunofluorescence assays, Western blot analysis, and real-time quan...
Source: Brain & Development - April 20, 2009 Category: Neurology Authors: Sun Jin-qiao, Sha Bin, Zhou Wen-hao, Yang Yi Tags: Original articles Source Type: journals

Molecular analysis of a presymptomatic case of carnitine palmitoyl transferase capital I, Ukrainian (CPT I) deficiency detected by tandem mass spectrometry newborn screening in Japan.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report herein a presymptomatic CPT capital I, Ukrainian deficiency detected in a Japanese female newborn by tandem mass spectrometry newborn screening. A mutation analysis of the CPT1A gene revealed two novel mutations, p.R446X and p.G719D. PMID: 19345525 [PubMed - as supplied by publisher]
Source: Brain & Development - April 2, 2009 Category: Neurology Authors: Tsuburaya R, Sakamoto O, Arai N, Kobayashi H, Hasegawa Y, Yamaguchi S, Shigematsu Y, Takayanagi M, Ohura T, Tsuchiya S Tags: Brain Dev Source Type: journals

Postnatal evolution of cortical malformation in the "non-affected" hemisphere of hemimegalencephaly.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
A boy with epilepsy of neonatal onset was diagnosed with hemimegalencephaly (HME) based on the finding of an enlarged left cerebral hemisphere with dysplastic cortex over the frontal, parietal, and temporal lobes. Magnetic resonance imaging (MRI) showed an essentially unremarkable gyration pattern in the contralateral hemisphere. Ictal electroencephalography and magnetoencephalography revealed epileptic foci in the left hemisphere. Hyperperfusion and hypermetabolism were noted in comparison to the contralateral hemisphere, and these findings were consistent with the diagnosis of HME. Soon after hemispherotomy was perfo...
Source: Brain & Development - April 1, 2009 Category: Neurology Authors: Kometani H, Sugai K, Saito Y, Nakagawa E, Sakuma H, Komaki H, Sasaki M, Adachi Y, Kaneko Y, Otsuki T, Hamano SI Tags: Brain Dev Source Type: journals

Two newly proposed infectious encephalitis/encephalopathy syndromesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Abstract: Two newly proposed infectious encephalitis/encephalopathy syndromes, in which magnetic resonance imaging (MRI) is essential for the diagnosis, have been reviewed. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is reported only in East Asian infants, characterized by a febrile seizure (usually >30min) as the initial neurological symptom on day 1, followed by secondary seizures at day 4 to 6; affected children display variable levels of neurological sequelae. MRI shows no acute abnormality during the first two days; reduced diffusion appears in the frontal or fronto-parietal subcortic...
Source: Brain & Development - April 1, 2009 Category: Neurology Authors: Jun-ichi Takanashi Tags: Review articles Source Type: journals

The effects of pilocarpine-induced status epilepticus on oxidative stress/damage in developing animals.Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Pilocarpine (PC), a muscarinic receptor agonist, is used for the induction of experimental models of status epilepticus (SE) for studying the type of seizure-induced brain injury and other neuropathophysiological mechanisms of related disorder. PC was administered to day-old Taiwan Native Breeder chicks and induced severe prolonged seizures (PC+PS) and repeated seizures (PC+RS) during 4h behavioral observations. Results showed that PC+PS group had excessive levels of reactive oxygen species (ROS) and malondialdehyde (MDA) production and lower activities of superoxide dismutase (SOD) and catalase (CAT) compared to the P...
Source: Brain & Development - March 31, 2009 Category: Neurology Authors: Tsai HL, Chang CN, Chang SJ Tags: Brain Dev Source Type: journals

Pathogenesis of leukodystrophy for Krabbe disease: Molecular mechanism and clinical treatmentEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We reported the basic concept of the pathology of leukodystrophy with emphasis on Krabbe disease. First, the normal process of myelination and the pathology of demyelination will be described, emphasizing the course inducing neuro-inflammation in its progression. After classifying metabolic leukodystrophy, the features of Krabbe disease (globoid-cell leukodystrophy) are explained as well as molecular cloning and mutation analysis of the galactocerebrosidase (GALC) gene. Finally, the experience of hematopoietic stem cell transplantation for patients with Krabbe disease is reported and the future possibility of therapy for t...
Source: Brain & Development - March 31, 2009 Category: Neurology Authors: Norio Sakai Tags: Review articles Source Type: journals

Reflections on the brainstem dysfunction in neurologically disabled childrenEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This article deals with the neurological basis of brainstem-related symptoms in disabled children. Synaptic interactions of respiratory and swallowing centers, which are briefly reviewed in this study, highlight the significance of the nucleus of solitary tract (NTS) in the stereotyped motor events. Coordination mechanisms between these two central pattern generators are also studied with a focus on the inhibitory action of decrementing expiratory neurons that terminate the inspiratory activity and become activated during swallowing. Dorsal brainstem lesions in hypoxic–ischemic encephalopathy (HIE) affect the area includ...
Source: Brain & Development - March 31, 2009 Category: Neurology Authors: Yoshiaki Saito Tags: Review articles Source Type: journals

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