Polygraphical study on age dependent epileptic encephalopathy--relationship between body movements during sleep and prognosis.


:Body movements (BMs) during sleep in patients with age dependent epileptic encephalopathy (ADEE) were studied polysomnographically in order to clarify the underlying mechanism of intractability and the age dependent trend. Twenty patients were divided into two groups according to the prognosis of convulsions. In the good prognosis group, BMs were nearly normal except for a low frequency in some cases. In the intractable group with seizures which were uncontrollable by medication and recurred within a year, BMs showed abnormalities as follows: abnormal distribution according to sleep stages, and/or a low frequency; increased BMs on therapy with prednisolone or ACTH. Moreover, a paradoxical increase of BMs with age and recurrence of seizures concomitantly occurred in the course of the disease. Status epilepticus appeared in cases under 1-DOPA administration or with a strikingly high frequency of BMs. Since electrophysiological evidence indicates that BMs during sleep are modulated by the dopaminergic (DA) system, the present data might suggest that prognosis of convulsions in ADEE depend upon, at least in part, the DA system. And denervated supersensitivity of that system might give rise to recurrence of seizures and status epilepticus.


Brain Dev


Brain & development


Iwakawa Y,Ogiso M,Suzuki H,Kawano Y,Koyama J,Shimohira M




Has Abstract


1986-01-01 00:00:00














  • Congenital ataxia of parietal origin? Report of two cases.

    abstract::Congenital cerebellar ataxia is usually thought to be of cerebellar origin. We report two children with congenital cerebellar ataxia, in whom neuroimaging investigations suggest the possibility of a parietal etiology. The two boys showed hypotonia, delayed motor and cognitive development followed by marked, truncally ...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Steinlin M,Schmitt B,Ferrini B

    更新日期:1998-06-01 00:00:00

  • Predictors of unprovoked seizure after febrile seizure: short-term outcomes.

    abstract:INTRODUCTION:We performed this study to confirm the known risk factors and to identify possible new risk factors for subsequent unprovoked seizure after febrile seizure (FS) on Jeju Island, South Korea. METHODS:A population-based retrospective study of 204 children with FS, whose first FS developed between March 2003 ...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Hwang G,Kang HS,Park SY,Han KH,Kim SH

    更新日期:2015-03-01 00:00:00

  • Electroencephalographic changes before the onset of symptomatic West syndrome.

    abstract::To clarify the characteristics of the mode of appearance and morphology of epileptiform discharges before the onset of West syndrome (WS). The subjects were 25 infants whose electroencephalograms (EEGs) were recorded before the onset of WS and whose first EEG was recorded before 6 months of corrected age (CA). We exte...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Endoh F,Yoshinaga H,Kobayashi K,Ohtsuka Y

    更新日期:2007-11-01 00:00:00

  • The test of variables of attention (TOVA) is useful in the diagnosis of Japanese male children with attention deficit hyperactivity disorder.

    abstract::The purpose of this study was to evaluate the ability of the test of variables of attention (TOVA) to distinguish between 6- to 12-year-old Japanese male children with attention deficit hyperactivity disorder (ADHD group; n=17) meeting DSM-IV and ICD-10 criteria, and age-matched, normal Japanese male controls (control...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Wada N,Yamashita Y,Matsuishi T,Ohtani Y,Kato H

    更新日期:2000-09-01 00:00:00

  • Apnea associated with hypoxia in preterm infants: impact on cerebral blood volume.

    abstract::The present study analyzed changes in cerebral blood volume (CBV) during apnea associated with hypoxia compared to apnea without hypoxia. Hypoxia was defined as pulsoxymetric oxygen saturation <80%>10 s. The employed technique was near infrared spectroscopy combined with electrocardiogram, electroocologram, pulsoxymet...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Payer C,Urlesberger B,Pauger M,Müller W

    更新日期:2003-01-01 00:00:00

  • Hyperammonemia in a case of herpes simplex and anti-N-methyl-d-aspartate receptor encephalitis.

    abstract::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 a...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Ko JM,Kim WJ,Kim SY,Lee JH,Chae JH,Kim KJ,Lim BC

    更新日期:2019-08-01 00:00:00

  • Efficacy of phenobarbital for benign convulsions with mild gastroenteritis: A randomized, placebo-controlled trial.

    abstract:OBJECTIVE:This study was performed to evaluate the efficacy and safety of intravenous phenobarbital (PB) for benign convulsions with mild gastroenteritis (CwG). METHODS:A randomized, single-blind, placebo-controlled trial involving patients with CwG was conducted at the Japanese Red Cross Society Himeji Hospital. Pati...

    journal_title:Brain & development

    pub_type: 杂志文章,随机对照试验


    authors: Takami Y,Nakagawa T

    更新日期:2019-08-01 00:00:00

  • HLA antigens, epilepsy and cytomegalovirus infection.

    abstract::Thirty-one epileptic patients, selected from among 900 children with previous febrile convulsions and subsequent epilepsy, were typed for HLA antigens. In 16 of the 31 patients CMV was isolated from the urine shortly after the appearance of spontaneous fits; in the remaining 15 patients the virus was never detected. A...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Iannetti P,Morellini M,Raucci U,Cappellacci S

    更新日期:1988-01-01 00:00:00

  • Neurodegeneration in hereditary nucleotide repair disorders.

    abstract::Both xeroderma pigmentosum group A (XPA) and Cockayne syndrome (CS) are rare autosomal disorders, have a genetic defect in the step of nucleotide repair, and involve various neurological abnormalities caused by progressive neurodegeneration. We performed comprehensive neuropathological analysis of five cases of XPA an...

    journal_title:Brain & development

    pub_type: 临床试验,杂志文章


    authors: Itoh M,Hayashi M,Shioda K,Minagawa M,Isa F,Tamagawa K,Morimatsu Y,Oda M

    更新日期:1999-07-01 00:00:00

  • Lennox-Gastaut syndrome after a further attenuated live measles vaccination.

    abstract::We reported a 2-year-old boy with Lennox-Gastaut syndrome, of which the cause could be an adverse effect of further attenuated live (FL) measles vaccine. The pre- and peri-natal histories of the patient were uneventful, except that he was one of monozygotic twins. He had developed normally until 24 months of life, whe...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Ishikawa T,Ogino C,Chang S

    更新日期:1999-12-01 00:00:00

  • Tourette syndrome and chronic tics in a sample of children and adolescents.

    abstract::Forty-eight subjects with Tourette syndrome (M 36, F 12; mean age 11.2 years) and 48 with chronic tic disorder (M 33, F 15; mean age 12.1 years) were recruited in order to study the vertical transmission within families of a vulnerability to tic disorders or to other psychiatric disorders, the role of adverse pre- and...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Saccomani L,Fabiana V,Manuela B,Giambattista R

    更新日期:2005-08-01 00:00:00

  • Influence of ACTH therapy on overnight sleep polygrams in infantile spasms.

    abstract::Overnight sleep polygrams were recorded before and during therapy in nine patients with infantile spasms. Results showed that ACTH therapy increased the waking time and decreased rapid eye movement sleep. Thus it caused sleep disturbance in patients with infantile spasms. During ACTH therapy the number of rapid eye mo...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Hashimoto T,Hiura K,Suzue J,Nokawa T,Fukuda K,Endo S,Tayama M,Tamura Y,Miyao M

    更新日期:1981-01-01 00:00:00

  • A group of very preterm children characterized by atypical gaze patterns.

    abstract:OBJECTIVE:Very preterm (VP) children are at risk for social difficulties, including autism spectrum disorder (ASD). This study used eye tracking to determine viewing behaviors that may reflect these difficulties. DESIGN:The gaze patterns of 47 VP (mean gestational age: 28weeks, mean birth weight: 948g, and mean chrono...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Sekigawa-Hosozawa M,Tanaka K,Shimizu T,Nakano T,Kitazawa S

    更新日期:2017-03-01 00:00:00

  • Missense mutation of the sodium channel gene SCN2A causes Dravet syndrome.

    abstract::Mutations of the gene encoding the alpha2 subunit of the neuronal sodium channel, SCN2A, have been found in benign familial neonatal-infantile seizures (BFNIS). In Dravet syndrome, only one nonsense mutation of SCN2A was identified, while hundreds of mutations were found in the paralogue gene, SCN1A, which encodes the...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Shi X,Yasumoto S,Nakagawa E,Fukasawa T,Uchiya S,Hirose S

    更新日期:2009-11-01 00:00:00

  • Electroencephalographic features of epileptic drop attacks and absence seizures: a case study.

    abstract::A study of epileptic drop attacks (EDA) by simultaneous video-polygraphic recordings was carried out in one epileptic patient with myoclonic astatic seizures (Doose syndrome). EDA was shown to correspond to a burst of generalized bilaterally synchronous spike and wave complexes (GBSSW) at 3 Hz. Absence seizures were a...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Oguni H,Imaizumi Y,Uehara T,Oguni M,Fukuyama Y

    更新日期:1993-05-01 00:00:00

  • The role of the central monoaminergic system and rapid eye movement sleep in development.

    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

    pub_type: 杂志文章


    authors: Mirmiran M

    更新日期:1986-01-01 00:00:00

  • Clinical and MRI findings in a case of D-2-hydroxyglutaric aciduria.

    abstract::We report the 3rd case in the literature of a 3-year-old boy with D-2-hydroxyglutaric (D-2-HG) aciduria, who presented primarily generalized hypotonia and feeding difficulty during the neonatal period, with eventual development of generalized myoclonic seizures. Gas chromatographic analysis of urinary organic acids sh...

    journal_title:Brain & development

    pub_type: 杂志文章,评审


    authors: Sugita K,Kakinuma H,Okajima Y,Ogawa A,Watanabe H,Niimi H

    更新日期:1995-03-01 00:00:00

  • Inflammatory neuropathology of infantile Alexander disease: A case report.

    abstract:BACKGROUND:Alexander disease (AxD) is a rare fatal leukodystrophy caused by a dominant missense mutation in the glial fibrillary acidic protein. In a mouse model of AxD, the pathological astrocyte causes a pronounced immune response. The inflammatory environment in the brain might play an important role in the neuronal...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Kora K,Kato T,Ide M,Tanaka T,Yoshida T

    更新日期:2020-01-01 00:00:00

  • Long-term effectiveness of ethosuximide, valproic acid, and lamotrigine in childhood absence epilepsy.

    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

    pub_type: 杂志文章


    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

  • Prognostic significance of failure of the initial antiepileptic drug in children with benign childhood epilepsy with centrotemporal spikes.

    abstract:BACKGROUND:Benign epilepsy with centrotemporal spikes is the most common partial epilepsy syndrome in children. The long-term prognosis for children with BECTS is believed to be generally excellent with seizures usually responding well to AEDs. The goal of the present study was to determine the risk factors associated ...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Incecik F,Altunbasak S,Herguner OM,Mert G,Sahan D

    更新日期:2015-01-01 00:00:00

  • Metabolic epilepsy: an update.

    abstract::Inborn errors of metabolism comprise a large class of genetic diseases involving disorders of metabolism. Presentation is usually in the neonatal period or infancy but can occur at any time, even in adulthood. Seizures are frequent symptom in inborn errors of metabolism, with no specific seizure types or EEG signature...

    journal_title:Brain & development

    pub_type: 杂志文章,评审


    authors: Papetti L,Parisi P,Leuzzi V,Nardecchia F,Nicita F,Ursitti F,Marra F,Paolino MC,Spalice A

    更新日期:2013-10-01 00:00:00

  • MECP2 mutations are an infrequent cause of mental retardation associated with neurological problems in male patients.

    abstract::Mutations in the methyl-CpG-binding protein 2 (MECP2) gene located on Xq28, cause Rett syndrome (RTT) in female patients. Meanwhile, nonmosaic MECP2 mutations unknown in girls have been found in an increasing number of male patients with a normal 46, XY karyotype. They can cause a broad spectrum of neurodevelopmental ...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Moog U,Van Roozendaal K,Smeets E,Tserpelis D,Devriendt K,Buggenhout GV,Frijns JP,Schrander-Stumpel C

    更新日期:2006-06-01 00:00:00

  • A case of intraneural perineurioma presenting with monomelic atrophy in a child.

    abstract::We report the case of an 11-year-old girl who developed slowly progressive atrophy of the left lower extremity. She suffered from mild dilated cardiomyopathy of unknown cause since 4years of age. When she was 7years old, her family noticed that her left extremity was thinner compared to the right one. Computed tomogra...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Miyahara-Katayama A,Ohya Y,Omi T,Komaki H,Nonaka I,Sato N,Sasaki M

    更新日期:2010-04-01 00:00:00

  • White matter abnormalities in an adult patient with l-2-hydroxyglutaric aciduria.

    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

    pub_type: 杂志文章


    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

  • Wolf-Hirschhorn syndrome with posterior intraorbital coloboma cyst: an unusual case.

    abstract::Wolf-Hirschhorn syndrome (WHS) is associated with partial deletion of short arm of chromosome 4, and characterized by severe growth retardation. Other characteristic features are microcephaly, intellectual handicap, Greek helmet facies and closure deficits such as cleft lip or cleft palate, coloboma of the eye, and ca...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Tutunculer F,Acunas B,Hicdonmez T,Deviren A,Pelitli V

    更新日期:2004-04-01 00:00:00

  • Pedoscope studies on neonatal activity and center of gravity after delivery.

    abstract::Seventeen normal term infants delivered at the Jikei University School of Medicine were placed daily on a pedoscope in the supine and prone position after birth, and the movement of the gravity center and changes in the activities of the extremities were assessed. The results indicated that both the activity of the ex...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Maekawa K,Soeda A,Yokoi S,Maekawa N,Usui N,Kiryu T,Hirasawa Y,Akamatsu H,Kamanaka A,Wada M

    更新日期:1986-01-01 00:00:00

  • Nutritional status among women whose pregnancy outcome was afflicted with neural tube defects in Tigray region of Ethiopia.

    abstract:BACKGROUND:Nutritional deficiency in pregnant women is a confirmed cause of neural tube defects (NTDs). Alongside to this background, We sought to determine the nutritional status and level of awareness on the issue of the NTDs as well as folic acid (FA) utilization among women who born infants with NTDs in Tigray regi...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Welderufael AL,Berihu BA,Berhe Y,Magana T,Asfaw S,Gebreselassie K,Belay E,Kebede H,Mulugeta A

    更新日期:2019-05-01 00:00:00

  • Abnormalities of joint mobility and gait in children with autism spectrum disorders.

    abstract:AIMS:Abnormalities of gross motor function in children with autism are well known to clinicians but have not received much empirical documentation and, with the exception of stereotypies, are not among its diagnostic criteria. We recorded the characteristics of gait and prevalence of toe walking, the range of passive j...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Shetreat-Klein M,Shinnar S,Rapin I

    更新日期:2014-02-01 00:00:00

  • Increased fetal heart rate variability in periventricular leukomalacia.

    abstract:OBJECTIVE:This study used quantitative analysis to determine whether increased variability in fetal heart rate (FHR) is related to the risk of developing periventricular leukomalacia (PVL). METHODS:We analyzed 124 FHR traces of neonates delivered preterm at 27-33 weeks' gestation to 105 mothers. FHR traces 1-3h before...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Kurahashi H,Okumura A,Kubota T,Kidokoro H,Maruyama K,Hayakawa M,Itakura A,Matsuzawa K,Yamamoto H,Kato T,Hayakawa F,Watanabe K

    更新日期:2016-02-01 00:00:00

  • A study on epileptic negative myoclonus in atypical benign partial epilepsy of childhood.

    abstract:OBJECTIVE:To investigate the clinical and neurophysiological characteristics, particularly therapeutic considerations, of epileptic negative myoclonus (ENM) in atypical benign partial epilepsy (ABPE) of childhood. METHODS:From 1998 to 2006, 14/242 patients with benign children epilepsy with centrotemporal spikes (BECT...

    journal_title:Brain & development

    pub_type: 杂志文章


    authors: Yang Z,Liu X,Qin J,Zhang Y,Bao X,Chang X,Wang S,Wu Y,Xiong H

    更新日期:2009-04-01 00:00:00