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 prevented further stroke-like episodes. This is the first report on effective l-arginine therapy in MELAS associated with the G13513A mutation.
journal_name
Brain Devjournal_title
Brain & developmentauthors
Shigemi R,Fukuda M,Suzuki Y,Morimoto T,Ishii Edoi
10.1016/j.braindev.2010.07.013subject
Has Abstractpub_date
2011-06-01 00:00:00pages
518-20issue
6eissn
0387-7604issn
1872-7131pii
S0387-7604(10)00196-8journal_volume
33pub_type
杂志文章abstract::Clobazam was added to the previous antiepileptic drug therapy of 90 children suffering from drug resistant epilepsy. Ten patients became seizure free, although four of these later developed tolerance. Thirty-three patients experienced a decrease in seizure frequency, and 24 of these, too, developed tolerance. Forty-fo...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(12)80025-8
更新日期:1991-05-01 00:00:00
abstract::This is the first case report on the diagnosis of spinal cord injury due to hemorrhage during the neonatal period using magnetic resonance imaging (MRI). Somatosensory evoked potentials are also helpful in the functional demonstration of this lesion. When discrepant signs, alert consciousness and intact cranial nerves...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/0387-7604(94)90114-7
更新日期:1994-01-01 00:00:00
abstract:INTRODUCTION:Chilaiditi syndrome is a rare pathophysiology in which the colon or other organs are interposed between the diaphragm and liver, and respiratory or digestive symptoms sometimes manifest. Although there have been some cases of Chilaiditi syndrome complicating neuromuscular disorders, none have described res...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2017.11.001
更新日期:2018-04-01 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
pub_type: 杂志文章
doi:10.1016/j.braindev.2010.03.003
更新日期:2011-02-01 00:00:00
abstract::We studied the relationship between theophylline and memory/learning using an elevated plus-maze test and measuring spontaneous locomotor activity in developing mice. There were no significant differences in transfer latency (TL) in 21-, 30- and 42-day-old mice in the acquisition trial, but theophylline significantly ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2003.12.008
更新日期:2004-10-01 00:00:00
abstract::Hypoxic ischemic brain can result in cerebral palsy, mental retardation, and learning disabilities in surviving children. The purpose of this study was to elucidate the cerebral blood flow volume in infants complicated with brain damage after the birth. Nine term infants with hypoxic ischemic encephalopathy and 41 nor...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2007.08.008
更新日期:2008-04-01 00:00:00
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
doi:10.1016/j.braindev.2014.02.008
更新日期:2015-01-01 00:00:00
abstract::Norrie disease is an X-linked recessive disorder that is characterized by congenital blindness. Although epileptic seizures are observed in some patients with Norrie disease, little is known about this phenomenon. Here, we report the manifestation of epilepsy in siblings with Norrie disease to increase our knowledge o...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2015.04.004
更新日期:2015-11-01 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
pub_type: 杂志文章
doi:10.1016/j.braindev.2011.11.007
更新日期:2012-09-01 00:00:00
abstract::Adrenocorticotropic hormone (ACTH) has been the first-line drug for the treatment of West syndrome, although the therapy has various adverse effects. ACTH depresses resistance to a variety of bacterial, viral, protozoal, and fungal agents. The timing of the various vaccinations is delayed after ACTH therapy in Japan, ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2008.11.007
更新日期:2009-11-01 00:00:00
abstract::Neonatal focal cerebral arterial infarction has been rarely reported in the literature, in contrast to the watershed infarctions, which are common entities among asphyxiated infants. In neonatal postmortem series, thromboembolism was the commonest cause of cerebral arterial occlusion; the source of emboli was associat...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(12)80344-5
更新日期:1992-11-01 00:00:00
abstract::A 7-year-old boy began to complain that his pitch perception was decreased just after oral medication with carbamazepine was initiated for the treatment of epilepsy. When he played the piano, he felt as if he had played a musical note of almost a half pitch lower than he had. His pitch perception recovered soon after ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(02)00155-9
更新日期:2003-03-01 00:00:00
abstract::Blink reflex can be elicited by sudden strong auditory stimulation. Using a special transducer wer recorded this reflex which appears as a microvibration of the eyelid, and named it auditory-evoked eyelid microvibration (AMV). As the reflex pathway of AMV exists in the brainstem, AMV is an easy and useful way of knowi...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(84)80009-1
更新日期:1984-01-01 00:00:00
abstract:PURPOSE:This study aimed to analyze the topological characteristics of brain structural network in pediatric epilepsy patients with vagus nerve stimulation (VNS) by applying graph theoretical approaches. METHODS:Nine patients with generalized seizures and eight normal controls (NC) were enrolled. Based on diffusion te...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2020.07.006
更新日期:2021-01-01 00:00:00
abstract:BACKGROUND:The initial presentation of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is indistinguishable from that of complex febrile seizures (FS), which poses a great diagnostic challenge for clinicians. Excitotoxicity is speculated to be the pathogenesis of AESD. Vitamin B6 (VB6) is ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2020.02.002
更新日期:2020-05-01 00:00:00
abstract::Childhood leukodystrophies are a growing category of neurological disorders in pediatric neurology practice. With the help of new advanced genetic studies such as whole exome sequencing (WES) and whole genome sequencing (WGS), the list of childhood heritable white matter disorders has been increased to more than one h...
journal_title:Brain & development
pub_type: 杂志文章,评审
doi:10.1016/j.braindev.2017.01.001
更新日期:2017-05-01 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
pub_type: 杂志文章
doi:10.1016/j.braindev.2013.11.011
更新日期:2014-10-01 00:00:00
abstract::The common factor causing congenital arthrogryposis is lack of fetal movements. This can result from a large number of disorders. They may be neuropathic, affecting the brain, the spinal cord, or the peripheral nerves; they may be abnormalities of the muscles, such as myasthenia gravis, congenital muscular dystrophies...
journal_title:Brain & development
pub_type: 杂志文章,评审
doi:10.1016/s0387-7604(98)00037-0
更新日期:1998-10-01 00:00:00
abstract::Phelan-McDermid syndrome (PMS) is a 22q13.3 deletion syndrome. Most PMS patients show global developmental delay and some of them suffer from developmental regression. The deleted region contains ARSA, which is responsible for metachromatic leukodystrophy (MLD). Here we report an extremely rare case of PMS characteriz...
journal_title:Brain & development
pub_type:
doi:10.1016/j.braindev.2020.02.003
更新日期:2020-05-01 00:00:00
abstract::The clinical profile of 33 children (19 boys, 14 girls) with multiple congenital contractures has been studied. The majority (54%) belong to arthrogryposis multiplex congenita with a static clinical course. Children were classified into three groups: group I (limb involvement only; n = 21) having arthrogryposis multip...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(96)00557-8
更新日期:1997-04-01 00:00:00
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
doi:10.1016/s0387-7604(00)00168-6
更新日期:2000-09-01 00:00:00
abstract::We present the clinical and neuropathological findings in a female patient with early onset Cockayne syndrome and a chromosomal anomaly (47XXX). The girl was the only child of healthy, unrelated parents. She was born with a birth weight of 1,930 gm. She had progeroid facial features with bilateral cataracts. A diagnos...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(12)80282-8
更新日期:1992-01-01 00:00:00
abstract:OBJECTIVE:To clarify changes in clinical practice for infantile spasms, including West syndrome, in Japan over the past two decades. METHODS:We investigated common treatment strategies for infantile spasms among 157 pediatric neurologists from a designated training facility for pediatric neurology and/or a designated ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2018.04.006
更新日期:2018-09-01 00:00:00
abstract::Cystic lesions of the posterior fossa remain a controversial subject as to clinical classification and diagnosis, especially for those in combination with other intracranial abnormalities. During the period of November 1985 to June 1991, 16 patients with cystic lesions of the posterior fossa were retrospectively revie...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/0387-7604(95)00067-4
更新日期:1995-11-01 00:00:00
abstract::Factors useful to predict seizure frequency and duration of antiepileptic treatment of children with benign partial epilepsy and rolandic spikes were retrospectively evaluated in 72 patients seizure-free for at least 5 years and off antiepileptic drugs for at least 2 years. Three groups were considered: Group I, 11 pa...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(87)80049-9
更新日期:1987-01-01 00:00:00
abstract::To investigate the development of the voluntary control of saccadic eye movement, we examined eye movements in 99 normal children (4-13 years of age). Subjects were asked to fixate a central light for 3-5 s. A target was then presented, either to its right or left. In visually guided saccades, the mean latencies of th...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(00)00101-7
更新日期:2000-05-01 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::Long-term social prognosis of 120 Japanese patients with West syndrome was surveyed using structured questionnaire at the age of 25.4 years in average. The survey revealed that ADL score remained null in one-fifth of cases; the majority entered in special education course for disabled children; only eleven cases were ...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/s0387-7604(01)00269-8
更新日期:2001-11-01 00:00:00
abstract:BACKGROUND:Takuto Rehabilitation Center for Children is located in Sendai, the capital of the Miyagi prefecture, and faces the Pacific Ocean. The tsunami caused by the Great East Japan Earthquake resulted in tremendous damage to this region. Many physically handicapped patients with epilepsy who are treated at our hosp...
journal_title:Brain & development
pub_type: 杂志文章
doi:10.1016/j.braindev.2016.01.005
更新日期:2016-08-01 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
pub_type: 杂志文章
doi:10.1016/j.braindev.2011.08.007
更新日期:2012-05-01 00:00:00