abstract：:A 1-year-old male began suffering from West syndrome at 3 months of age, when electroencephalography revealed hypsarrhythmia accompanied by a periodic, brief suppression phase. The administration of adrenocorticotropic hormone was partially effective for stopping the condition, and the seizure type evolved into brief ...

journal_title：Brain & development

authors： Nonoda Y,Saito Y,Nagai S,Sasaki M,Iwasaki T,Matsumoto N,Ishii M,Saitsu H

更新日期：2013-03-01 00:00:00

abstract：:Relative to their numbers, more than twice the number of disabled children fell victim to the Great East Japan Earthquake than did normal people. It was important to find out needs and provide support, as the needs of disabled children vulnerable to the disaster, such as a shortage of diapers of the right size for dis...

journal_title：Brain & development

authors： Tanaka S

更新日期：2013-03-01 00:00:00

abstract：:We examined the specific nerve conduction deficits distinguishing spinal muscular atrophy (SMA) subtypes I and II. Five SMA I patients (age, 0.2-1.1 years) and 10 SMA II patients (age, 1.0-2.8 years) were examined. Patients were compared to age-matched controls for motor and sensory conduction velocity (MCV and SCV) c...

journal_title：Brain & development

authors： Yonekawa T,Komaki H,Saito Y,Sugai K,Sasaki M

更新日期：2013-02-01 00:00:00

abstract：:Magnetic resonance (MR) findings in cases of total asphyxia, whose lesions are mainly in the brainstem and deep nuclei, have not been clarified. In this study, we investigated MR images in neonates with total asphyxia. MR images of six infants (three males and three females; gestational age, 35-39 weeks; birth weights...

journal_title：Brain & development

authors： Sugiura H,Kouwaki M,Kato T,Ogata T,Sakamoto R,Ieshima A,Yokochi K

更新日期：2013-01-01 00:00:00

abstract：:We investigated the neuroprotective effects of human placental extracts (HPE) and the effects of HPE on recovery of cognitive and behavioral function on hypoxic-ischemic brain injury in the newborn rat. The right common carotid arteries of 7-day-old rats were coagulated, and rats were then exposed to 8% oxygen. Immedi...

journal_title：Brain & development

authors： Park JY,Byeon JH,Park SW,Eun SH,Chae KY,Eun BL

更新日期：2013-01-01 00:00:00

abstract：BACKGROUND:The abducens nucleus directly innervates the lateral rectus muscle and plays a role in controlling conjugate horizontal eye movements. Although the neuronal cytoarchitecture of the abducens nucleus has been extensively investigated in various species of vertebrates, few studies have been undertaken in humans...

journal_title：Brain & development

authors： Yamaguchi K,Honma K

更新日期：2012-10-01 00:00:00

abstract：:Anti-muscle-specific tyrosine kinase antibody (MuSK-Ab) is the second most frequent autoantibody identified in adult patients with myasthenia gravis (MG). Adult patients with MuSK-Ab demonstrate characteristic clinical features but very little information is available for childhood-onset patients with MuSK-positive MG...

journal_title：Brain & development

authors： Takahashi Y,Sugiyama M,Ueda Y,Itoh T,Yagyu K,Shiraishi H,Ukeba-Terashita Y,Nakanishi M,Nagashima T,Imai T,Motomura M,Saitoh S

更新日期：2012-10-01 00:00:00

abstract：:Self-induced seizures by somatosensory stimulation are rare. We describe two epileptic patients with self-induced seizures presumably by peri-orbital somatosensory stimulation. Two infants with severely delayed psychomotor development and poor visual acuity after acute subdural hemorrhage in early infancy had been dia...

journal_title：Brain & development

authors： Takayama R,Takahashi Y,Mogami Y,Ikegami M,Mukaida S,Ikeda H,Imai K,Shigematsu H,Suzuki Y,Inoue Y

更新日期：2012-09-01 00:00:00

abstract：:A 10-year-old boy with psychomotor developmental delay and cerebellar vermis atrophy developed right hemiplegia with vomiting, unconsciousness, convulsions, and late-onset fever. Slow delta activity was noted over the left hemisphere on electroencephalography, and neuroimaging revealed swelling of the left temporo-occ...

journal_title：Brain & development

authors： Ohmura K,Suzuki Y,Saito Y,Wada T,Goto M,Seto S

更新日期：2012-09-01 00:00:00

abstract：OBJECTIVES:Acute encephalopathy with reduced subcortical diffusion (AED) covers a spectrum including not only typical acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) but also atypical AESD with monophasic clinical course, or more severe subtypes. Aim of this study is to analyze prognostic ...

journal_title：Brain & development

authors： Hayashi N,Okumura A,Kubota T,Tsuji T,Kidokoro H,Fukasawa T,Hayakawa F,Ando N,Natsume J

更新日期：2012-09-01 00:00:00

abstract：:Rett syndrome (RTT) is a neurodevelopmental disorder that is one of the most common causes of mental retardation in females. RTT diagnosis is based on distinct clinical criteria. We describe here a female patient with severe phenotype of congenital variant RTT. The patient originally presented with severe developmenta...

journal_title：Brain & development

authors： Kobayashi Y,Ohashi T,Akasaka N,Tohyama J

更新日期：2012-08-01 00:00:00

abstract：:This is the first study to report differences between Japanese children with and without dyslexia in the way string-length and lexicality effects are manifested when reading Japanese kana. These children were asked to read kana words and non-words consisting of either two or five kana characters. The results showed th...

journal_title：Brain & development

authors： Sambai A,Uno A,Kurokawa S,Haruhara N,Kaneko M,Awaya N,Kozuka J,Goto T,Tsutamori E,Nakagawa K,Wydell TN

更新日期：2012-06-01 00:00:00

abstract：:We report on a 9-year-old boy who presented with acute encephalopathy and hemophagocytic lymphohistiocytosis (HLH). The patient was referred to our hospital because of fever, seizures, and decreased consciousness. He showed moderately elevated levels of proinflammatory cytokines in the cerebrospinal fluid and plasma, ...

journal_title：Brain & development

authors： Wada T,Nishiura K,Kuroda M,Asai E,Vu QV,Toma T,Niida Y,Yachie A

更新日期：2012-05-01 00:00:00

abstract：:A research committee supported by the Japanese government conducted a nationwide survey on the epidemiology of acute encephalopathy in Japan using a questionnaire. A total of 983 cases reportedly had acute encephalopathy during the past 3 years, 2007-2010. Among the pathogens of the preceding infection, influenza viru...

journal_title：Brain & development

authors： Hoshino A,Saitoh M,Oka A,Okumura A,Kubota M,Saito Y,Takanashi J,Hirose S,Yamagata T,Yamanouchi H,Mizuguchi M

更新日期：2012-05-01 00:00:00

abstract：PURPOSE:We performed this study to evaluate the long-term efficacy and tolerability of ethosuximide (ESX), valproic acid (VPA), and lamotrigine (LTG) as initial monotherapies for patients with newly diagnosed childhood absence epilepsy. METHODS:We retrospectively reviewed the medical records of 128 patients (45 boys a...

journal_title：Brain & development

authors： Hwang H,Kim H,Kim SH,Kim SH,Lim BC,Chae JH,Choi JE,Kim KJ,Hwang YS

更新日期：2012-05-01 00:00:00

abstract：:Neurofibrillary tangles (NFTs) have been shown in 20% of subacute sclerosing panencephalitis (SSPE) cases. NFTs contain paired helical filaments formed by hyperphosphorylated tau. The intraneuronal tau metabolism and the rate of formation of paired helical filaments can be regulated by interactions between tau and iso...

journal_title：Brain & development

authors： Yüksel D,Ichiyama T,Yilmaz D,Anlar B

更新日期：2012-04-01 00:00:00

abstract：BACKGROUND:Amplitude-integrated electroencephalogram (aEEG) at <6 h is the best single outcome predictor in term infants with perinatal asphyxia at normothermia. Hypothermia treatment has changed the cutoff values for outcome prediction by using time at onset of normal trace and SWC. Cerebral hemodynamics and oxygenati...

journal_title：Brain & development

authors： Gucuyener K,Beken S,Ergenekon E,Soysal S,Hirfanoglu I,Turan O,Unal S,Altuntas N,Kazanci E,Kulali F,Koc E,Turkyilmaz C,Onal E,Atalay Y

更新日期：2012-04-01 00:00:00

abstract：:Spinal muscular atrophy (SMA) is a common autosomal recessive neuromuscular disorder that is caused by loss of the survival motor neuron gene, SMN1. SMA treatment strategies have focused on production of the SMN protein from the almost identical gene, SMN2. Valproic acid (VPA) is a histone deacetylase inhibitor that c...

journal_title：Brain & development

authors： Harahap IS,Saito T,San LP,Sasaki N,Gunadi,Nurputra DK,Yusoff S,Yamamoto T,Morikawa S,Nishimura N,Lee MJ,Takeshima Y,Matsuo M,Nishio H

更新日期：2012-03-01 00:00:00

abstract：:Altered maternal micronutrients (folic acid, vitamin B(12)) are suggested to be at the heart of intra-uterine programming of adult diseases. We have recently described interactions of folic acid, vitamin B(12) and docosahexaenoic acid in one carbon metabolism that is considered to play a key role in regulation oxidati...

journal_title：Brain & development

authors： Roy S,Kale A,Dangat K,Sable P,Kulkarni A,Joshi S

更新日期：2012-01-01 00:00:00

abstract：:Molecular background of neurogenetic disease is briefly reviewed. Importance and usefulness of genetic testing are emphasized. Molecular genetics is a powerful tool for investigation of epileptic syndromes. Diagnosis based on gene analysis will give a new insight for pathophysiology and clinical outcome of the patient...

journal_title：Brain & development

authors： Suzuki Y

更新日期：2011-10-01 00:00:00

abstract：:We characterized the neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy and measured differences in IQ between children with different types of epilepsy. The Korean Education Development Institute-Wechsler Intelligence Scale for Children (KEDI-WISC) was administered to 72 patients...

journal_title：Brain & development

authors： Jeong MH,Yum MS,Ko TS,You SJ,Lee EH,Yoo HK

更新日期：2011-09-01 00:00:00

abstract：:Alexander disease is a major entity of leukodystrophy; magnetic resonance imaging (MRI) studies of the brain typically show extensive changes in the cerebral white matter with frontal predominance. Heterozygous missense mutations of GFAP are thought to be sufficient for the molecular diagnosis, which has widened the A...

journal_title：Brain & development

authors： Shiihara T,Yoneda T,Mizuta I,Yoshida T,Nakagawa M,Shimizu N

更新日期：2011-08-01 00:00:00

abstract：:We describe a 14-month-old girl who presented with arterial ischemic stroke due to moyamoya disease, unilateral renal agenesis and external iliac artery stenosis. The association of moyamoya disease with renal agenesis and external iliac artery stenosis has not been described before. This report expands the spectrum o...

journal_title：Brain & development

authors： Ramesh K,Sharma S,Raju V,Kumar A,Gulati S

更新日期：2011-08-01 00:00:00

abstract：:We report a case involving a 15-year-old boy with MELAS (G13513A mutation) who developed several stroke-like episodes in a short period of time. Intravenous administration of l-arginine during the acute phase of the stroke-like episodes reduced symptoms immediately, and oral supplementation of l-arginine successfully ...

journal_title：Brain & development

authors： Shigemi R,Fukuda M,Suzuki Y,Morimoto T,Ishii E

更新日期：2011-06-01 00:00:00

abstract：:The objective of this pilot study was to test the validity and reliability of a new scale, the Ability for Basic Movement Scale for Children (ABMS-C). A total of 45 pediatric patients with disabilities (aged 0.1-8.8 years; 29 males, 16 females) participated in this prospective study. To prove the validity and reliabil...

journal_title：Brain & development

authors： Miyamura K,Hashimoto K,Honda M

更新日期：2011-06-01 00:00:00

abstract：:Propionic acidemia manifesting with hyperglycemia is rare. Few cases have been reported mainly of the neonatal-onset form associated with high mortality. We report a 9-month-old Palestinian boy who manifested with coma, severe hyperglycemia and ketoacidosis mimicking diabetic ketoacidosis. Family history of unexplaine...

journal_title：Brain & development

authors： Dweikat IM,Naser EN,Abu Libdeh AI,Naser OJ,Abu Gharbieh NN,Maraqa NF,Abu Libdeh BY

更新日期：2011-05-01 00:00:00

abstract：:We evaluated the cyclic alternating pattern (CAP) during the first year of life in order to obtain information on the maturation of arousal mechanisms during NREM sleep and to provide normative data for CAP parameters in this age range (5-16months). Eleven healthy children (mean age 7.9±3.3months, seven boys) were stu...

journal_title：Brain & development

authors： Miano S,Peraita-Adrados R,Montesano M,Castaldo R,Forlani M,Villa MP

更新日期：2011-05-01 00:00:00

abstract：OBJECTIVE:To analyze the electroclinical features of children with childhood absence epilepsy (CAE) and discuss the diagnostic criteria for CAE. METHODS:The video-electroencephalogram (VEEG) database in our hospital was searched using "absence seizures" and "3-Hz generalized spike and waves (GSW)" as key-words. Other ...

journal_title：Brain & development

authors： Ma X,Zhang Y,Yang Z,Liu X,Sun H,Qin J,Wu X,Liang J

更新日期：2011-02-01 00:00:00

abstract：:We report a 9-year-old girl with acute autonomic sensory and motor neuropathy (AASM) associated with human parvovirus B19 (HPV-B19) infection. The patient presented with fever, erythema of the entire body, and abdominal pain with vomiting. The titer of HPV-B19 IgM antibody was significantly elevated. Symptoms such as ...

journal_title：Brain & development

authors： Hanai S,Komaki H,Sakuma H,Nakagawa E,Sugai K,Sasaki M,Oya Y,Higurashi N,Hamano S

更新日期：2011-02-01 00:00:00

abstract：OBJECTIVE:We retrospectively reviewed six patients with incontinentia pigmenti (IP) who had encephalopathic manifestations during early infancy. METHODS:We enrolled six patients who met the following criteria from the mailing list of the Annual Zao Conference: (1) diagnosis of IP; (2) encephalopathic manifestations wi...

journal_title：Brain & development

authors： Abe S,Okumura A,Hamano S,Tanaka M,Shiihara T,Aizaki K,Tsuru T,Toribe Y,Arai H,Shimizu T

更新日期：2011-01-01 00:00:00

abstract：:A rare case of a congenital brain tumor was diagnosed by sonography in a fetus at 37weeks' gestation. The ultrasound examination showed a large area of both increased echogenicity and echolucency in one hemisphere suggestive of brain tumor or hemorrhage. Extensive surgical removal of the tumor was performed and reveal...

journal_title：Brain & development

authors： Yang CY,Hsu JF,Lin KL,Jung SM,Lien R,Chang YL

更新日期：2010-11-01 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：:This study examined paroxysmal abnormalities and epilepsy in EEG for individuals with pervasive developmental disorders (PDD) in two parts: first with a large number of subjects (n=1624); and second with extracted subjects followed from 5 years into adolescence and beyond (n=92). Many paroxysms in PDD patients in thei...

journal_title：Brain & development

authors： Kawasaki Y,Shinomiya M,Takayanagi M,Niwa S

更新日期：2010-10-01 00:00:00

abstract：:Xeroderma pigmentosum (XP), a genetic disorder in DNA nucleotide excision repair, is characterized by skin hypersensitivity to sunlight and progressive neurological impairment. Laryngeal dystonia and vocal cord paralysis are complications that can arise in older XP group A (XPA) patients. We report three patients with...

journal_title：Brain & development

authors： Miyata R,Sasaki T,Hayashi M,Araki S,Shimohira M,Kohyama J

更新日期：2010-09-01 00:00:00

abstract：:Although hypothalamic hamartomas (HHs) have been shown to be intrinsically epileptogenic and to participate in the generation of gelastic seizures, no evidence has been reported regarding its contribution to the pathogenesis of infantile spasms. We describe a male infant with Pallister-Hall syndrome who had a large HH...

journal_title：Brain & development

authors： Wakamoto H,Sumi A,Motoki T,Ohmori H

更新日期：2010-09-01 00:00:00

abstract：:The aim of this research was to compare the Strengths and Difficulties Questionnaire (SDQ) scores and subscale scores in children with high-functioning autism spectrum disorder (HFASD) and attention-deficit/hyperactivity disorder (AD/HD), and also to clarify the differences between parent- and teacher-assessed SDQ sco...

journal_title：Brain & development

authors： Iizuka C,Yamashita Y,Nagamitsu S,Yamashita T,Araki Y,Ohya T,Hara M,Shibuya I,Kakuma T,Matsuishi T

更新日期：2010-09-01 00:00:00

abstract：:Neuroimaging findings are usually normal in children with benign partial epilepsy in infancy. However, we found a transient reduction of water diffusion in the corpus callosum in two patients with probable benign partial epilepsy in infancy. The patients were admitted to our hospital because of seizure clusters. No de...

journal_title：Brain & development

authors： Okumura A,Abe S,Hara S,Aoyagi Y,Shimizu T,Watanabe K

更新日期：2010-08-01 00:00:00

abstract：AIM:To report on two children with encephalopathy caused by dietary thiamine deficiency due to newly developing nutritional problems in contemporary Japan. SUBJECTS:A 1-year-old boy who had consumed 1l of isotonic drinks per day for 4 months after an episode of diarrhea, and presented with ocular movement disorder, dy...

journal_title：Brain & development

authors： Saeki K,Saito Y,Komaki H,Sakakibra T,Nakagawa E,Sugai K,Sakuma H,Sasaki M,Honda T,Hayashi H,Katori N,Miyahara Y

更新日期：2010-08-01 00:00:00

abstract：:A 14-year-old female had repeated vomiting, headache, abdominal pain, visual field deficit and lethargy at the onset of hypertensive encephalopathy. Cerebrospinal fluid (CSF) test revealed a high level of IgG and protein. MRI demonstrated no supratentorial cerebral lesions but hyperintense lesions were observed from t...

journal_title：Brain & development

authors： Nagato M,Takahashi Y,Yoshioka M,Nambu M

更新日期：2010-08-01 00:00:00

abstract：:A 5-year-old female case of 5p-syndrome exhibited pontine hypoplasia on magnetic resonance imaging. A high-pitched cry characteristic of 5p-syndrome disappeared after 2 years. 5p-syndrome should be considered as a differential diagnosis for brainstem, especially pontine, hypoplasia. Older patients with brainstem hypop...

journal_title：Brain & development

authors： Ninchoji T,Takanashi J

更新日期：2010-08-01 00:00:00