Abstract：
:Several studies have demonstrated the slowing effect of corticosteroids on the decline of muscle strength in Duchenne muscular dystrophy (DMD). Deflazacort (DFC) is supposed to have fewer side effects than prednisone (PRED). An ongoing double blind multicenter study is comparing the effects and side effects of deflazacort (0.9 mg/kg/day) and prednisone (0.75 mg/kg/day) in DMD. This interim report includes data for 67 boys between age 5 years and loss of ambulation. Besides the common clinical and laboratory data for chronic corticoid treatment, motor performance has been tested. Interim results, 3-15 months after starting the medication, show some scattering but no grouping of data for all the functions tested: timed motor functions, sum of the strength of 20 muscles according to a 10-point scale on manual testing, weight gain, osteocalcin and alkaline phosphatase. Only the changes in CK activity after 3 months medication might reflect two equal groups without any correlation with the initial activity or with other parameters. On average, there was no clear-cut loss of muscle strength or performance. Except for in 4 patients, who were excluded due to unacceptable weight gain and/or loss of ambulation, there were no side effects considered to be serious. The results suggest that (i) DFC and PRED in equal anti-inflammatory dosage are similarly or equally efficient in slowing down the decline of muscle strength in DMD; (ii) benefits outweigh the side effects. This allows the study to continue as designed.
journal_name
Brain Devjournal_title
Brain & developmentauthors
Reitter Bsubject
Has Abstractpub_date
1995-01-01 00:00:00pages
39-43eissn
0387-7604issn
1872-7131pii
0387-7604(95)00015-1journal_volume
17 Supplpub_type
临床试验,杂志文章,多中心研究abstract：:Monitoring brain function by EEG is an important means of preventing cerebral insults in pediatric cardiovascular surgery. We studied intraoperative EEG changes and their regional differences associated with hypothermia and brain ischemia. The subjects of this study consisted of 13 children ranging in age from 4 month...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(01)00192-9
更新日期：2001-03-01 00:00:00
abstract：:Germ cell tumors originating in the posterior fossa are very rare. Described herein is a case of primary germ cell tumor (yolk sac tumor) found in the cerebellar vermis. A 5-year-old boy who complained of headache was admitted. CT and MRI revealed a tumor with diffuse enhancement by contrast medium in the right cerebe...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/0387-7604(94)90128-7
更新日期：1994-09-01 00:00:00
abstract：:The electroencephalographic features and their evolutional changes with age were described in three cases of lissencephaly syndrome diagnosed by CT scan. The case with more severe lissencephaly displayed very similar EEG findings. In early or middle infancy when infantile spasms began, EEG showed very high amplitude (...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(79)80042-x
更新日期：1979-01-01 00:00:00
abstract：:Cerebral blood flow was assessed by ultrasound in 12 children with intractable epilepsy who were treated with ACTH. The average maximal blood velocity (A/L) and end-diastolic blood velocity (d) of the internal carotid artery were measured, before, during and after ACTH therapy in each subject. The right and left mean ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(85)80060-7
更新日期：1985-01-01 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
pub_type： 杂志文章
doi：10.1016/j.braindev.2009.07.003
更新日期：2010-08-01 00:00:00
abstract：OBJECTIVE:To evaluate the efficacy and safety of intravenous (i.v.) thiamylal in pediatric magnetic resonance imaging (MRI) sedation. METHODS:Infants and children from 1 month up to 8 years of age who underwent MRI in our hospital between April 2017 and March 2019 were included in this prospective observational study....
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2020.04.005
更新日期：2020-08-01 00:00:00
abstract：BACKGROUND:Few studies have targeted psychomotor development and associated perinatal risk factors in Japanese very low birth weight (VLBW) infants who are severely small for gestational age (SGA). DESIGN/SUBJECTS:A single-center study was conducted in 104 Japanese VLBW infants who were born preterm, due to maternal, ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2015.07.008
更新日期：2016-02-01 00:00:00
abstract：:Muscle ultrasound scanning is a non-invasive and painless technique for evaluating muscle disorders in childhood. We have performed ultrasound scans of the quadriceps muscle in 26 children with various forms of congenital muscular dystrophies. There were 8 patients clearly showing selective involvement within the comp...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(12)80091-x
更新日期：1992-03-01 00:00:00
abstract：:Two brothers, offspring of an Arab inbred family suffered from both mucolipidosis III (ML-III) and Bardet-Biedl syndrome (BBS). Other members of this family were affected either with ML-III, or with BBS. It seems that this unique combination is probably coincidental. ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(12)80072-6
更新日期：1990-01-01 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：: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
pub_type： 杂志文章
doi：10.1016/j.braindev.2013.01.010
更新日期：2013-09-01 00:00:00
abstract：:It is sometimes difficult to discriminate high functioning pervasive developmental disorders (HFPDD) from attention deficit/hyperactivity disorders (AD/HD) in young children because of the behavioral similarities between the two. For adequate diagnosis, understanding fundamental differences in their social cognitive a...
journal_title：Brain & development
pub_type： 临床试验,杂志文章
doi：10.1016/S0387-7604(03)00170-0
更新日期：2004-08-01 00:00:00
abstract：:We report three typical cases of Landau-Kleffner syndrome with varied courses. The very frequent discharges in sleep EEGs, often showing the patterns of CSWS (continuous spike-waves during slow-wave sleep), either typical (spike-wave complex occupying over 85% of slow-wave sleep duration) or atypical (spike-waves occu...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/0387-7604(95)00168-9
更新日期：1996-05-01 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
pub_type： 杂志文章
doi：10.1016/j.braindev.2010.11.003
更新日期：2011-09-01 00:00:00
abstract：:We report a 2-month-old boy who presented with apneic attacks as a manifestation of epileptic seizures at onset and eventually progressed to infantile spasms. At onset, at 2 months of age, apneic attacks were the sole symptom of epileptic fits. Although these seizures were accompanied by cyanosis, bradycardia was not ...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/0387-7604(95)00148-4
更新日期：1996-05-01 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
pub_type： 杂志文章
doi：10.1016/s0387-7604(01)00215-7
更新日期：2001-07-01 00:00:00
abstract：:Twelve patients with early infancy onset megalencephaly and leukodystrophy with a mild clinical course are reported. The neuroradiological, clinical, and genetic aspects of this recently recognized familial leukodystrophy syndrome were reviewed. Five were affected siblings, and all patients had consanguineous parents....
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(98)00002-3
更新日期：1998-04-01 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
pub_type： 杂志文章
doi：10.1016/j.braindev.2015.04.012
更新日期：2016-01-01 00:00:00
abstract：:713 children (from newborn to 12-month-old) with delayed motor development were carefully examined and classified into normal, very light cerebral coordination disturbance (CCD, Vojta), light CCD, moderate CCD, severe CCD, suspected cerebral palsy (CP) and other diseases at their first visit, and were followed up care...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(83)80024-2
更新日期：1983-01-01 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
pub_type： 杂志文章
doi：10.1016/j.braindev.2010.07.013
更新日期：2011-06-01 00:00:00
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 dip...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2016.06.001
更新日期：2016-11-01 00:00:00
abstract：AIM:The prevalence of cerebral palsy (CP) has not decreased in developed countries over the past 30 years. We examined gestational age-specific trends in the prevalence of CP. METHODS:This unselected, population-based study was conducted in Miyazaki prefecture, Japan (10,000 deliveries annually), where 102,999 deliver...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2015.10.002
更新日期：2016-04-01 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：: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：:Schizencephaly is an uncommon structural disorder of cerebral cortical development, characterized by congenital clefts spanning the cerebral hemispheres from the pial surface to the lateral ventricles and lined by cortical gray matter. Either an antenatal environmental incident or a genetic origin could be responsible...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/s0387-7604(00)00173-x
更新日期：2000-12-01 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
pub_type： 杂志文章
doi：10.1016/j.braindev.2009.04.010
更新日期：2010-08-01 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：:Tourette syndrome (TS) is a neurodevelopmental disorder defined by tics, but most patients also experience bothersome sensory phenomena, in the form of premonitory urges and/or sensory hypersensitivity. Whereas premonitory urges are temporally paired with tics, sensory hypersensitivity is a constant, heightened awaren...
journal_title：Brain & development
pub_type： 杂志文章,评审
doi：10.1016/j.braindev.2020.06.003
更新日期：2020-10-01 00:00:00
abstract：:Mutations in the mitochondrial tRNAMet gene have been reported in only five patients to date, all of whom presented with muscle weakness and exercise intolerance as signs of myopathy. We herein report the case of a 12-year-old girl with focal epilepsy since the age of eight years. At age 11, the patient developed sudd...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/j.braindev.2019.01.006
更新日期：2019-05-01 00:00:00
abstract：:A 10-year-old male patient, who had suffered from intractable convulsions from the neonatal period, is presented. Serial brain images suggested slow, gradual destruction of neural elements. Human cytomegalovirus (HCMV) DNA was detected in his cerebrospinal fluid (CSF) by means of the polymerase chain reaction. Intrath...
journal_title：Brain & development
pub_type： 杂志文章
doi：10.1016/0387-7604(93)90086-n
更新日期：1993-11-01 00:00:00
