Childhood disintegrative disorder.

abstract：:Neuromyelitis optica (NMO) is an inflammatory demyelinating disease with a poor prognosis that is characterized by inflammatory optic neuritis and myelitis. Although it is commonly misdiagnosed as multiple sclerosis (MS), distinguishing NMO from MS is important, as therapeutic approaches approved for MS are ineffectiv...

journal_title：Brain & development

authors： Numata Y,Uematsu M,Suzuki S,Miyabayashi T,Oyama T,Kubota S,Itoh T,Hino-Fukuyo N,Takahashi T,Kure S

更新日期：2015-01-01 00:00:00

abstract：OBJECTIVE:Autonomic nervous system activity is recognized as a major component of emotional responses. Future reward/punishment expectations depend upon the process of decision making in the frontal lobe, which is considered to play an important role in executive function. The aim of this study was to investigate the r...

journal_title：Brain & development

authors： Hosaka H,Aoyagi K,Kaga Y,Kanemura H,Sugita K,Aihara M

更新日期：2017-08-01 00:00:00

abstract：:Transmission of information in the brain is of a chemical nature. Neurotransmitters are present at very early stages of brain development, having trophic effects on maturation of target neurons as well as mediating the behavioral repertoire of the immature brain. Many centrally acting psychoactive drugs which are comm...

journal_title：Brain & development

authors： Mirmiran M

更新日期：1986-01-01 00:00:00

abstract：:Becker muscular dystrophy (BMD) was diagnosed in a male patient with Klinefelter's syndrome (47, XXY karyotype). The BMD was confirmed by (i) immunohistological methods and Western blotting, showing decreased quantity of dystrophin in muscle biopsy specimen and (ii) molecular genetic analysis which demonstrated a homo...

journal_title：Brain & development

authors： Zeitoun O,Ketelsen UP,Wolff G,Müller CR,Korinthenberg R

更新日期：1997-07-01 00:00:00

abstract：:Oxidative stress resulting from increased free radical production and/or defects in antioxidant defences may be the cause of various neurodegenerative disorders. In this study, the roles of oxygen free radicals, nitric oxide, superoxide dismutase, vitamin E and vitamin C were investigated in pure and complicated hered...

journal_title：Brain & development

authors： Gücüyener K,Pinarli FG,Erbaş D,Hasanoğlu A,Serdaroğlu A,Topaloğlu H

更新日期：2010-02-01 00:00:00

abstract：:Event-related potential (ERP) studies with infants are often limited by a small number of measurements. We introduce a weighted general linear mixed model analysis with a time-varying covariate, which allows for the efficient analysis of all available event-related potential data of infants. This method allows control...

journal_title：Brain & development

authors： Stahl D,Parise E,Hoehl S,Striano T

更新日期：2010-04-01 00:00:00

abstract：INTRODUCTION:Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most common subtype of acute encephalopathy among children in Japan. The pathogenesis of AESD is mostly delayed cerebral edema caused by excitotoxic injury. It is difficult to discriminate AESD and complex febrile seizure ...

journal_title：Brain & development

authors： Yokochi T,Takeuchi T,Mukai J,Akita Y,Nagai K,Obu K,Kakuma T,Matsuishi T

更新日期：2016-02-01 00:00:00

abstract：:To determine whether or not dystrophin really exists in the outer plexiform layer (OPL) of the retina, we studied control and mdx mice, using four kinds of polyclonal antibodies (DMDP-II, 60 kd, 30 kd and DMDP-IV) against dystrophin. Although control OPL showed a positive immunohistochemical reaction with all four ant...

journal_title：Brain & development

authors： Zhao J,Uchino M,Yoshioka K,Miyatake M,Miike T

更新日期：1991-01-01 00:00:00

abstract：:The aim of this study is to investigate predictive factors of post-traumatic syndrome in children with minor head injury. Prospective neurological, electroencephalographic and psychological investigations were performed in 98 children aged 3-13 years within 24 h after the trauma and 4-6 weeks later. Inclusion criteria...

journal_title：Brain & development

authors： Korinthenberg R,Schreck J,Weser J,Lehmkuhl G

更新日期：2004-03-01 00:00:00

abstract：:Oral motor patterns during feeding were investigated in 58 patients with severe physical disability. Five patients showed a pattern resembling sucking. Twenty-nine exhibited an up-and-down movement of the jaw and protrusion of the tongue. Among these, the mouth opened when the food entered and the lips closed before s...

journal_title：Brain & development

authors： Yokochi K

更新日期：1997-12-01 00:00:00

abstract：:Since 1988, we have diagnosed 6 cases of hereditary progressive dystonia with marked diurnal fluctuation (HPD) in Taiwan. All cases presented with clinical features similar to those described by Segawa. They consisted of four sporadic and two familial cases. The age at onset ranged from 18 months to 8 years. There is ...

journal_title：Brain & development

authors： Wang PJ,Ko YM,Young C,Hwu WL,Shen YZ

更新日期：1994-03-01 00:00:00

abstract：:In this study a comparison of the myelination rate in humans in normal and pathologic conditions was made. The progress of myelination was examined on slides stained by the Klüver-Barrera method and evaluated as to four degrees. The prenatal myelination in the brain stem in a group of newborns who died of pregnancy pa...

journal_title：Brain & development

authors： Dambska M,Laure-Kamionowska M

更新日期：1990-01-01 00:00:00

abstract：OBJECTIVE:To analyze and to discuss whether by paying attention to the many recent advancements in the field of pediatric epilepsy surgery catastrophic childhood epilepsies caused by definitive or suspected structural lesions can be prevented more often these days in comparison to the past. METHODS:Based on data from ...

journal_title：Brain & development

authors： Holthausen H,Pieper T,Kudernatsch M

更新日期：2013-09-01 00:00:00

abstract：:l-2-Hydroxyglutaric aciduria (l-2-HGA) is a rare inborn error of metabolism. Mainly, patients with this disorder exhibit neurological symptoms and characteristic neuroradiological findings, such as subcortical white matter abnormalities, which are believed to be caused by the toxicity of the accumulation of l-2-hydrox...

journal_title：Brain & development

authors： Yamamoto T,Yoshioka S,Tsurusaki Y,Shino S,Shimojima K,Shigematsu Y,Takeuchi Y,Matsumoto N

更新日期：2016-01-01 00:00:00

abstract：OBJECTIVE:In order to evaluate whether face perception is intact or not in Williams syndrome (WS), the face inversion effects (FIE) in the event-related potential (ERP) or magnetoencephalography (MEG) were investigated in three teenaged patients with WS. METHODS:Responses to the inverted faces and upright faces were c...

journal_title：Brain & development

authors： Nakamura M,Watanabe S,Inagaki M,Hirai M,Miki K,Honda Y,Kakigi R

更新日期：2013-04-01 00:00:00

abstract：:The abnormal patterns of cerebral convolutions range from severe to small anomalies restricted to tertiary gyri and sulci. Lesions within Glial-Pial barrier were found in examined cases with cortical developmental abnormalities. Anomalies and impaired function of vessels penetrating the cortex from meningeal plexus co...

journal_title：Brain & development

authors： Dambska M,Laure-Kamionowska M

更新日期：2001-07-01 00:00:00

abstract：:The correlation between the functional and morphological maturation of the auditory pathway was studied in preterm and term infants, children and adults. As to the auditory brainstem response (ABR), peak latencies and I-V interpeak latencies (central transmission) gradually decreased during the third trimester and the...

journal_title：Brain & development

authors： Inagaki M,Tomita Y,Takashima S,Ohtani K,Andoh G,Takeshita K

更新日期：1987-01-01 00:00:00

abstract：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 patien...

journal_title：Brain & development

authors： Kojima K,Shirai K,Kobayashi M,Miyauchi A,Saitsu H,Matsumoto N,Osaka H,Yamagata T

更新日期：2018-01-01 00:00:00

abstract：:We report herein the case of a 9-year-old girl with life-threatening hyperkinetic involuntary movement of unknown etiology. Medical treatment was ineffective for her stereotypy and choreoathetotic/ballistic movements, but bilateral stimulation of the globus pallidus immediately alleviated these symptoms. Pallidal deep...

journal_title：Brain & development

authors： Sato K,Nakagawa E,Saito Y,Komaki H,Sakuma H,Sugai K,Sasaki M,Kaido T,Nakama H,Otsuki T

更新日期：2009-06-01 00:00:00

abstract：:We examined seizure, cognitive, and motor outcomes in patients with Rasmussen syndrome or Rasmussen encephalitis (RS), after recent initiation of immunomodulatory therapies. Among 53 patients with a diagnosis of RS referred from all over Japan, 49 patients (male 22, female 27) with symptoms and findings characteristic...

journal_title：Brain & development

authors： Takahashi Y,Yamazaki E,Mine J,Kubota Y,Imai K,Mogami Y,Baba K,Matsuda K,Oguni H,Sugai K,Ohtsuka Y,Fujiwara T,Inoue Y

更新日期：2013-09-01 00:00:00

abstract：:The authors report a case of early infantile epileptic encephalopathy (EIEE) associated with hemimegalencephaly. The etiological factors in previously published cases of EIEE are reviewed and attention is focused on the high proportion of cases associated with neuronal migration disorders. We suggest that idiopathic c...

journal_title：Brain & development

authors： Bermejo AM,Martin VL,Arcas J,Perez-Higueras A,Morales C,Pascual-Castroviejo I

更新日期：1992-11-01 00:00:00

abstract：:We report seizures induced by adrenocorticotropic hormone (ACTH), which were demonstrated clinically and electro-encephalographically, in a severely handicapped 7-month-old infant with West syndrome due to perinatal hypoxicischemic encephalopathy. Although tonic spasms (original seizures) decreased soon after starting...

journal_title：Brain & development

authors： Kanayama M,Ishikawa T,Tauchi A,Kobayashi M,Takasaka Y,Shibata H

更新日期：1989-01-01 00:00:00

abstract：OBJECTIVES:To explore the relationship between autistic regression (AR) with and without EEG abnormalities and favourable outcome. METHODS:Follow up data on children with favourable outcome in a series of 534 cases aged below 5 years and diagnosed as ASD. RESULTS:Cases with regression were 167 (31.8%), usually with p...

journal_title：Brain & development

authors： Zappella M

更新日期：2010-10-01 00:00:00

abstract：:The aim of this study was to evaluate respiratory function of severely handicapped children. Tidal volumes and respiratory rates were determined in a total of 130 children with different clinical motor abilities. Tidal volume of non-sitters (n = 39) was significantly lower than ambulators (n = 49) or sitters (n = 42) ...

journal_title：Brain & development

authors： Ishida C,Fujita M,Umemoto H,Taneda M,Sanae N,Tazaki T

更新日期：1990-01-01 00:00:00

abstract：PURPOSE:Ictal fear is an uncommon condition in which fear manifests as the main feature of epileptic seizures. The literature has suggested that ictal fear is generally associated with poor seizure outcomes. We wanted to clarify the variability in seizure outcome of children with ictal fear. SUBJECTS AND METHODS:We id...

journal_title：Brain & development

authors： Akiyama M,Kobayashi K,Inoue T,Akiyama T,Yoshinaga H

更新日期：2014-10-01 00:00:00

abstract：:Bedside monitoring of cerebral circulation or oxygen metabolism in infants to appropriately manage circulation and establish the oxygen dose, aiming at improving the neurological prognosis, is needed in general clinical practice. Near-infrared spectroscopy is used for measurements of neonatal cerebral Hb oxygen satura...

journal_title：Brain & development

authors： Kusaka T,Isobe K,Yasuda S,Koyano K,Nakamura S,Nakamura M,Ueno M,Miki T,Itoh S

更新日期：2014-04-01 00:00:00

abstract：:We report on the clinical, neuropathological, and genetic findings of a Japanese case with myocerebrohepatopathy spectrum (MCHS) disorder due to polymerase gamma (POLG) mutations. A girl manifested poor sucking and failure to thrive since 4 months of age and had frequent vomiting and developmental regression at 5 mont...

journal_title：Brain & development

authors： Montassir H,Maegaki Y,Murayama K,Yamazaki T,Kohda M,Ohtake A,Iwasa H,Yatsuka Y,Okazaki Y,Sugiura C,Nagata I,Toyoshima M,Saito Y,Itoh M,Nishino I,Ohno K

更新日期：2015-08-01 00:00:00

abstract：:Three cases of Lesch-Nyhan syndrome (LNS) were examined by polysomnography to assess the brainstem function, and to determine the causes of the neurological manifestations and sudden death in this syndrome. In the two older cases, the amount of slow wave and rapid eye movement (REM) sleep, the REM density and the freq...

journal_title：Brain & development

authors： Saito Y,Hanaoka S,Fukumizu M,Morita H,Ogawa T,Takahashi K,Ito M,Hashimoto T

更新日期：1998-12-01 00:00:00

abstract：:The association between measurements of lateral ventricle dilatation determined by serial ultrasound and brain specific creatine-kinase isoenzyme patterns (CK-BB) is studied in 60 very low birth weight preterm neonates of 1,500 g birth weight or 32 weeks gestation or less. The patients were divided into three groups a...

journal_title：Brain & development

authors： Amato M,Hüppi P,Gambon R

更新日期：1992-07-01 00:00:00

abstract：:MRI appearance of Sturge-Weber Syndrome (SWS) in patients with Tuberous Sclerosis (TSC) has been rarely reported. 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 malformat...

journal_title：Brain & development

authors： Curatolo P,Lo-Castro A,Pinci M,Moavero R,Bombardieri R

更新日期：2009-05-01 00:00:00