Abstract:
PURPOSE:Benign Childhood Epilepsy with Centrotemporal Spikes (BCECTS) is the most common childhood idiopathic focal epilepsy with a good outcome. However, an increasing number of reports suggest a 'not so benign' outcome showing atypical evolution. METHODS:We analyzed the electroencephalographic (EEG) spike source dipoles of BCECTS patients to compare patients with confirmed typical and atypical outcome showing intractable epilepsy and cognitive decline. Thirty-seven patients that were diagnosed for BCECTS and whose clinical information was sufficient enough to confirm long-term outcome were included in the study. Eight patients (22%, 7 patients for poor seizure control and 1 patient for cognitive decline) were classified as showing atypical outcome. Forty-seven averaged spike dipole sources were analyzed using the single equivalent current dipole (ECD) method. The inverse problem was solved using the 4 shells ellipsoidal model. RESULTS:The coordinate value of yori differed significantly between the two groups (mean±standard deviation, 0.32±0.33 for the typical group and -0.33±0.77 for the atypical group, P<0.001). These findings suggest that the averaged spike source dipoles were oriented anteriorly in patients with typical outcome and posteriorly in patients with atypical outcome. Spike source dipoles seemed to be located higher in patients with atypical presentation but this finding was insignificant. CONCLUSION:We can conclude that there are significant differences in spike source dipole in patients with BCECTS that shows atypical outcome, and we can use this finding as a marker for predicting an outcome at the time of diagnosis.
journal_name
Brain Devjournal_title
Brain & developmentauthors
Kim H,Yoo IH,Lim BC,Hwang H,Chae JH,Choi J,Kim KJdoi
10.1016/j.braindev.2016.06.001subject
Has Abstractpub_date
2016-11-01 00:00:00pages
903-908issue
10eissn
0387-7604issn
1872-7131pii
S0387-7604(16)30063-8journal_volume
38pub_type
杂志文章abstract::We report the case of a girl with Tay-Sachs disease who had convulsions and deteriorated rapidly after an upper respiratory infection at the age of 11 months. At the age of 16 months, her seizures became intractable and magnetic resonance imaging of the brain showed high signal intensity on T2-weighted images and mark...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2009.01.007
更新日期:2010-03-01 00:00:00
abstract:OBJECTIVE:Rubinstein-Taybi syndrome (RSTS) is a rare congenital disorder (1:125.000) characterized by growth retardation, psychomotor developmental delay, microcephaly and dysmorphic features. In 25% of patients seizures have been described, and in about 66% a wide range of EEG abnormalities, but studies on neurologica...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2015.12.003
更新日期:2016-06-01 00:00:00
abstract:BACKGROUND:The effect of rituximab on acute disseminated encephalomyelitis (ADEM) followed by recurrent optic neuritis (ON) is not yet known. PATIENT:We are reporting the case of a 4-year-old Japanese girl who was diagnosed with anti-myelin oligodendrocyte glycoprotein (MOG) antibody positive ADEM followed by recurren...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2018.03.011
更新日期:2018-08-01 00:00:00
abstract::Recent postmortem studies have suggested that sudden infant death syndrome (SIDS) might involve an underlying, gradual brain stem injury caused by repeated episodes of transiently compromised brain stem circulation. Autopsy studies have also reported that vertebral artery occlusion due to head rotations, such as occur...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(03)00004-4
更新日期:2003-08-01 00:00:00
abstract:PURPOSE:Polymicrogyria, a malformation of the cerebral cortex, frequently causes epilepsy. Diffuse bilateral polymicrogyria (DBP) is related to poor epilepsy prognosis, but most patients with DBP are not good candidates for resective epilepsy surgery and effectiveness of corpus callosotomy (CC), a palliative surgery, f...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2018.03.007
更新日期:2018-09-01 00:00:00
abstract:BACKGROUND:Pelizaeus-Merzbacher disease (PMD), a hypomyelinating leukodystrophy, and the related but less severe allelic spastic paraplegia 2 (SPG2) are caused by mutations in the proteolipid protein 1 (PLP1) gene. Magnetic resonance imaging (MRI) is pivotal for diagnosing these disorders. The severity of PMD/SPG2 vari...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2014.06.011
更新日期:2015-04-01 00:00:00
abstract::In 1908 a Viennese remedial educator Theodor Heller described six children under the name of dementia infantilis who had insidiously developed a severe mental regression between the 3rd and 4th years of life after normal mental development. Neuropathological and other medical conditions are sometimes associated with t...
journal_title:Brain & development
pub_type: 杂志文章,评审
doi:10.1016/s0387-7604(02)00228-0
更新日期:2003-06-01 00:00:00
abstract::We describe a clinical profile of a male with Rett syndrome who presented initially with significant axial and peripheral hypotonia, head and truncal titubation and global delay. He is non-ambulatory, lost the few words he had learned and gradually developed hand stereotypes, breathing difficulties, seizures, scoliosi...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2005.03.018
更新日期:2005-11-01 00:00:00
abstract::We present a unique 11-year-old girl showing clinical features of Rett-related disorder with distinctive facial features and multiple congenital anomalies including ocular hypertelorism, arched eyebrows, a broad nose, dental anomalies, congenital heart disease, truncal obesity, and epilepsy. A novel de novo mutation i...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2017.12.013
更新日期:2018-05-01 00:00:00
abstract::The recent advances in neonatal neurology which are discussed include i) the improved organization of perinatal medical services, ii) technological advances including ultrasonography, brainstem auditory evoked responses and intracranial pressure monitoring and, iii) new developments in therapeutics. The development of...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(84)80095-9
更新日期:1984-01-01 00:00:00
abstract::Fifty-one consecutive adult patients with epilepsy and early development destructive brain lesions were divided into three main groups according to the topographic distribution of the lesion on magnetic resonance imaging: hemispheric (H) (n=9); main arterial territory (AT) (n=25) and arterial borderzone (Bdz) (n=17). ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(03)00065-2
更新日期:2003-12-01 00:00:00
abstract::The abnormalities of intracranial hemodynamics associated with strokelike episodes in MELAS are variable depend on the time phase from the onset of strokelike episodes and on the progression of the dementia state. To clarify the regional cerebral blood flows (rCBF) in the natural course of MELAS is very important to u...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2007.06.008
更新日期:2008-02-01 00:00:00
abstract::Dihydropyrimidinase deficiency is a rare autosomal recessive disease affecting the second step of pyrimidine degradation. It is caused by mutations in the DPYS gene. Only approximately 30 cases have been reported to date, with a phenotypical variability ranging from asymptomatic to severe neurological illness. We repo...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2018.10.005
更新日期:2019-03-01 00:00:00
abstract::A patient with adrenoleukodystrophy was successfully treated by means of intravenous gammaglobulin injections. The clinical symptoms, especially visual loss, were apparently relieved and no neurological deterioration was observed during a 18-month period following the start of the gammaglobulin treatment. ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(89)80083-x
更新日期:1989-01-01 00:00:00
abstract::Infarct locations in children with arterial ischemic stroke have primarily been reported to be lobar or in the basal ganglia, and those in patients with Down syndrome (DS) and antiphospholipid syndrome (APS) are typically wide and multiple. No solitary brain stem infarctions have ever been reported in children with DS...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2019.02.013
更新日期:2019-06-01 00:00:00
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
pub_type: 杂志文章
doi:10.1016/j.braindev.2014.03.015
更新日期:2015-01-01 00:00:00
abstract::In the acute precollicular-postmammillary decerebrate cat, stimulation of the mesencephalic locomotor region (MLR) induces "controlled locomotion" on a moving treadmill. Stimulation of the dorsal area and of the ventral area of the pons at its midline elicited a long-lasting decrease and an increase in the tone of the...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(86)80062-6
更新日期:1986-01-01 00:00:00
abstract::Ninety-nine cases of Rett syndrome (RTT) diagnosed clinically (age range 3 years 6 months to 29 years 9 months) were evaluated for the ability of language. The presence of meaningful words, vocabularies, and ages at the start and disappearance of speech were assessed. Phenotype/genotype correlation was evaluated in 22...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(01)00367-9
更新日期:2001-12-01 00:00:00
abstract::The sleep patterns of children often cause anxiety to their parents. Some disturbances are unusual, and therefore may cause diagnostic difficulties. Sleep walking and night terrors can be confused with epileptic seizures. The sudden sleep of narcolepsy can lead to false accusations, when in fact the episodes are beyon...
journal_title:Brain & development
pub_type: 杂志文章,评审
doi:10.1016/s0387-7604(12)80262-2
更新日期:1992-05-01 00:00:00
abstract::The juvenile form of neuronal ceroid lipofuscinosis (JNCL) is caused by mutations in the CLN3 gene, and is characterized by progressive loss of vision and development of motor deficits. A few patients exhibit a more protracted clinical course and are diagnosed with protracted JNCL (PJNCL). Here, we report the autopsy ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2005.12.004
更新日期:2006-08-01 00:00:00
abstract::Coffin-Lowry syndrome is an X-linked mental retardation disorder with dysmorphism caused by mutation of the ribosomal S6 kinase (RSK2) gene. Coffin-Lowry syndrome patients can experience unusual drop episodes whereby an abrupt loss of muscle tone and falling down can be induced by sudden, unexpected tactile or auditor...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2004.02.015
更新日期:2005-03-01 00:00:00
abstract::A family in which three siblings were affected with severe cerebral malformations in association with ocular anomalies and muscle disease is reported. One sibling was diagnosed as having Fukuyama type congenital muscular dystrophy (FCMD) because he showed severe hypotonia with dystrophic findings on a muscle biopsy in...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(12)80154-9
更新日期:1992-09-01 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
pub_type: 杂志文章
doi:10.1016/j.braindev.2014.10.013
更新日期:2015-08-01 00:00:00
abstract::MRI of a female patient with xeroderma pigmentosum group A (XP-A) showed progressive cerebral atrophy, but no disease-specific lesion. MR spectroscopy with short TE sequences in the bilateral white matter revealed decreased N-acetyl aspartate (neuro-axonal marker) and increased myo-inositol (astroglial marker) with a ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2018.06.013
更新日期:2018-11-01 00:00:00
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
doi:10.1016/j.braindev.2019.03.013
更新日期:2019-08-01 00:00:00
abstract:OBJECTIVE:Patients with Tourette syndrome (TS) often report characteristic sensory experiences, also called premonitory urges (PUs), which precede tic expression and have high diagnostic relevance. This study investigated the usefulness of a scale developed and validated in children and adolescents-the Premonitory Urge...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2012.12.010
更新日期:2014-01-01 00:00:00
abstract:OBJECTIVE:Acute encephalitis with refractory repetitive partial seizure (AERRPS) is a peculiar type of post-encephalitic/encephalopathic epilepsy. Here we report an analysis of AERRPS in a series of children and propose an effective treatment option for seizure control in these children. METHODS:We retrospectively rev...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2008.09.010
更新日期:2009-09-01 00:00:00
abstract::Six females with Rett syndrome (RS)--all seriously motor disabled with clinical symptomatology indicating not only brain but also spinal cord impairment--were investigated using auditory and somatosensory evoked responses techniques. In all patients the responses representing the pathways through the upper spinal cord...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(87)80076-1
更新日期:1987-01-01 00:00:00
abstract:OBJECTIVE:Cognitive impairments had been reported in childhood acute lymphoblastic leukemia, what caused the impairments needed to be demonstrated, chemotherapy-related or the disease itself. The primary aim of this exploratory investigation was to determine if there were changes in brain function of children with acut...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2017.04.014
更新日期:2017-10-01 00:00:00
abstract:PURPOSE:We studied children younger than 6years old who developed catastrophic epilepsy and were registered in the FACE study group to clarify their clinical characteristics and prevalence of seizure as well as epilepsy types. SUBJECTS:Subjects were prospectively recruited from children with epilepsy who satisfied the...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2013.02.004
更新日期:2013-09-01 00:00:00