Abstract：
:The objective of this study was to compare the efficacy of corticotropin (ACTH) versus vigabatrin in treating infantile spasms and to determine which medication has a more favorable long-term outcome in terms of cognitive function, evolution of epilepsy, and incidence of autism. Patients with infantile spasms were included in the study if they were 3 to 16 months old, had hypsarrhythmia, and had no previous treatment with vigabatrin or corticosteroids. Patient evaluation included electroencephalographic and psychometric measures before and after treatment. Patients were stratified based on etiology (idiopathic or symptomatic) and sex and then randomized between the ACTH and vigabatrin treatment groups. Each of the treatment groups received either ACTH or vigabatrin for 2 weeks. At the end of 2 weeks of treatment, patients were considered responders if spasms and hypsarrhythmia resolved. Nonresponders were crossed over and treated with the alternate drug. Nine patients were included in the study. Three patients received ACTH, one of whom was a responder. Six patients received vigabatrin, three of whom were responders. The five nonresponders received both therapies. All patients had some degree of developmental plateau or regression before the initiation of treatment. Four patients with idiopathic infantile spasms showed improved cognitive function following treatment. The remaining five patients remained significantly delayed. Five patients with symptomatic infantile spasms had epilepsy following treatment; three of them were in the autistic spectrum. The small number of infants in this pilot study is insufficient to determine which of the two drugs is more effective. However, the following trends were identified: vigabatrin may be more effective for patients with symptomatic infantile spasms; patients with idiopathic infantile spasms tend to have a better cognitive outcome; and patients with symptomatic infantile spasms tend to develop both epilepsy and autism.
journal_name
J Child Neuroljournal_title
Journal of child neurologyauthors
Askalan R,Mackay M,Brian J,Otsubo H,McDermott C,Bryson S,Boyd J,Snead C 3rd,Roberts W,Weiss Sdoi
10.1177/08830738030180030801subject
Has Abstractpub_date
2003-03-01 00:00:00pages
165-70issue
3eissn
0883-0738issn
1708-8283journal_volume
18pub_type
临床试验,杂志文章abstract：:Facioscapulohumeral muscular dystrophy is one of the most prevalent muscular dystrophies in the world, resulting from the deletion of tandem repeats on chromosome 4q35. Extramuscular associations include sensorineural hearing loss, mental retardation, and epilepsy. These manifestations are commonly found in those with...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.2310/7010.2006.00054
更新日期：2006-03-01 00:00:00
abstract：:The aim of this study is to investigate the efficacy and tolerability of topiramate in a large number of children with West syndrome. The authors performed a retrospective, questionnaire-based data collection in specialized epilepsy units in Germany. Patients with West syndrome and hypsarrhythmia could be included if ...
journal_title：Journal of child neurology
pub_type： 杂志文章,多中心研究
doi：10.1177/0883073807300535
更新日期：2007-03-01 00:00:00
abstract：:A child suffered from herpes simplex virus encephalitis at the age of 6 months; a late relapse occurred 8.5 years after the initial episode, the longest latency period reported. Radiologic and autopsy findings suggest local reactivation of latent herpes simplex virus as the cause of relapse. All cases of late relapse ...
journal_title：Journal of child neurology
pub_type： 杂志文章,评审
doi：10.1177/0883073807309243
更新日期：2008-03-01 00:00:00
abstract：:Klonopin (clonazepam; Genentech Inc, South San Francisco, California) oral wafers are benzodiazepines with anticonvulsive and anxiolytic properties. Our institution has been prescribing clonazepam wafers for acute treatment of prolonged seizures for years. Patients' size determined dosing at 0.25, 0.5, 1, or 2 mg wafe...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073810368312
更新日期：2010-12-01 00:00:00
abstract：:Tuberous sclerosis complex is a disease that affects many organs, including the central nervous system. Nervous system involvement in the form of hamartomas often results in seizures. In this study we wanted to determine the outcome of epilepsy in tuberous sclerosis complex and determine whether interictal electroence...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/088307380001500203
更新日期：2000-02-01 00:00:00
abstract：:Spinal muscular atrophy type 0 is a severe form of spinal muscular atrophy that is usually fatal in the first months of life. These children present with arthrogryposis multiplex congenita and respiratory compromise. We describe a child with spinal muscular atrophy and arthrogryposis multiplex congenita who has had a ...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/088307380101601213
更新日期：2001-12-01 00:00:00
abstract：:Neurological disorders, including neurodevelopmental disorders, have been identified by the World Health Organization (WHO) as one of the greatest threats to global public health. It is generally believed that these conditions are more prevalent in the developing than the developed world because of multiple known risk...
journal_title：Journal of child neurology
pub_type： 杂志文章,评审
doi：10.1177/0883073809357792
更新日期：2010-04-01 00:00:00
abstract：:Direct perfusion of specific regions of the central nervous system by convection-enhanced delivery is becoming more widely used for the delivery of compounds in the research and treatment of various neural disorders. In contrast to other currently available central nervous system delivery techniques, convection-enhanc...
journal_title：Journal of child neurology
pub_type： 杂志文章,评审
doi：10.1177/0883073808321064
更新日期：2008-10-01 00:00:00
abstract：:A 3 1/2-year-old boy presented with megaloblastic anemia and recurrent episodes of severe lactic acidosis and coma. At age 4 years, he developed sepsis and died; postmortem examination failed to show any gross abnormality in any tissue. Biochemical analysis of muscle showed decreased activities for all respiratory cha...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/088307380401900403
更新日期：2004-04-01 00:00:00
abstract：:A 10-year-old boy with tuberous sclerosis complex and macrodactyly of the right index and middle fingers is described. He also had a fibrous hamartoma at the front of the right wrist. The association of fibrous hamartoma and macrodactyly has not been previously described in tuberous sclerosis complex. ...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073810375616
更新日期：2011-01-01 00:00:00
abstract：:The electrophysiologic findings in an infant with congenital facial diplegia (Möbius syndrome) are presented. As to serial electrically elicited blink reflexes, the R1 amplitude was significantly reduced and the R2 latency was significantly prolonged. The M response of the orbicularis oculi muscle showed slightly redu...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/088307389300800213
更新日期：1993-04-01 00:00:00
abstract：:In addition to the typical infantile spasm symptoms, several other symptoms, such as eye movements, have been reported to be associated with infantile spasms, although the relationship between the typical spasms and these other events is not fully understood. Here we present a case with West syndrome. We observed the ...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073813489169
更新日期：2013-11-01 00:00:00
abstract：:The authors reviewed the medical records of very low-birth-weight infants admitted from 1998 to 2007 and compared neurodevelopmental outcomes with their previously reported data from 1989 to 1997. The recent group included 824 infants, and the previous group included 471 infants. Neurodevelopmental outcomes were class...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073811408606
更新日期：2011-11-01 00:00:00
abstract：:Young children with sickle cell disease are at risk of brain damage, including stroke. We tested the hypothesis that such patients are also at risk of cognitive impairment. We characterized the cognitive ability of kindergarten children to minimize the effect of disease-related school absence. The Memphis City Schools...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/088307380201700204
更新日期：2002-02-01 00:00:00
abstract：:The etiology of mild mental retardation remains undefined in about 60% of cases. Even though the causes of mild mental retardation are likely to be heterogeneous, the evidence for genetic involvement is increasing, along with the development of specific diagnostic techniques. To improve our understanding of the geneti...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/08830738060210091201
更新日期：2006-09-01 00:00:00
abstract：:Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is characterized by acute- or subacute-onset encephalopathy with extrapyramidal, psychiatric, and epileptic manifestations. Diagnosis is confirmed by positive antibodies to NMDA receptor in cerebrospinal fluid and serum. Eleven pediatric cases presented over a 2-y...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073813494474
更新日期：2014-11-01 00:00:00
abstract：:This article describes a case of pyruvate dehydrogenase deficiency in a 3-year-old boy who presented generalized hypotonia, severe psychomotor development delay, and generalized and partial seizures and was refractory to antiepileptic drugs. After the diagnosis, the patient was put on a ketogenic diet. Six months late...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073812436424
更新日期：2012-12-01 00:00:00
abstract：:Children and adolescents with the Diagnostic and Statistical Manual of Mental Disorders-IV (DSM-IV) diagnosis of attention-deficit hyperactivity disorder (ADHD) can have comorbid conditions such as conduct disorder, oppositional defiant disorder, and obsessive-compulsive disorder (comorbid type). The purpose of our st...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/08830738040190020201
更新日期：2004-02-01 00:00:00
abstract：:We previously reported that patients with spinal muscular atrophy do not lose muscle strength over time as measured quantitatively. However, we noted that many patients with spinal muscular atrophy suffer from what they call fatigue. We wondered if we could measure fatigue during a single maximal voluntary contraction...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/088307389701200507
更新日期：1997-08-01 00:00:00
abstract：:Baló concentric sclerosis is a unique and rare phenomenon in demyelinating disease. Typically thought of as a subtype of multiple sclerosis, Baló concentric sclerosis is characterized pathologically by striking rings of demyelination alternating with areas of preserved myelination. Its exact prevalence in adult and es...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073813517294
更新日期：2014-05-01 00:00:00
abstract：:Thromboembolic events during the perinatal period are responsible for irreversible brain damage owing to cerebral hypoxia and neuronal necrosis. We investigated the presence of thrombophilia risk factors in children with congenital neurologic disorders. Nineteen children (9 males and 10 females), aged 1 to 14 years (m...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/08830738050200060701
更新日期：2005-06-01 00:00:00
abstract：:A 2-year-old boy with an initial diagnosis of Hunter syndrome (mucopolysaccharidosis II) had a more severe phenotype than expected, which warranted further evaluation. The patient had severe infantile global neurodevelopmental delays, macrocephaly with a prominent forehead, coarse facial features with clear corneas, c...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073811425860
更新日期：2012-06-01 00:00:00
abstract：:An association between overweight and attention-deficit/hyperactivity disorder (ADHD) in children was previously suggested. We examined the prevalence of overweight, anthropometric changes, and the effect of methylphenidate treatment in 275 children with ADHD without neurological comorbidities and in controls. Data we...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073810380051
更新日期：2011-03-01 00:00:00
abstract：:Managing severe epilepsy syndromes of early childhood is challenging as the seizures are typically resistant to treatment and may cause disabling mental and behavioral problems in later life. A comprehensive treatment plan includes pharmacologic, nonpharmacologic, and surgical options. This article reviews clinical st...
journal_title：Journal of child neurology
pub_type： 杂志文章,评审
doi：10.1177/0883073809338153
更新日期：2009-08-01 00:00:00
abstract：:Idiopathic dystonia with onset in the first year of life has been described as a transient movement disorder, in contrast to other forms of idiopathic dystonia We report on a girl who showed, from her 5th month, episodes of dystonic postures of her neck and arm, which lasted for seconds and occurred several times a da...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/088307380001501212
更新日期：2000-12-01 00:00:00
abstract：:Clinical manifestations were retrospectively assessed in 5 families with tuberous sclerosis complex, including 1 pair of monozygotic twins. Interfamilial variation in tuber count was significantly larger than intrafamilial variation. Severity of epilepsy and cognitive profiles varied both between and within families, ...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073807307093
更新日期：2007-12-01 00:00:00
abstract：:This is the first study to evaluate the efficacy of riboflavin for migraine prophylaxis in children. This was a randomized, double-blind study of riboflavin (200 mg daily) versus placebo in 48 children. The primary efficacy measure was the number of patients achieving a 50% or greater reduction in the number of migrai...
journal_title：Journal of child neurology
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1177/0883073808318053
更新日期：2008-11-01 00:00:00
abstract：:Neurologic toxicity may occur as a direct effect of cancer and its therapy or indirectly because of a dysfunctional immune system. The authors report the development of axonal neuropathy, myelopathy, and leucoencephalopathy associated with glutamic acid decarboxylase-65 (GAD) antibodies in 4 children with progressive ...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073808315617
更新日期：2008-11-01 00:00:00
abstract：:This study assessed metabolic functioning of regional brain areas to address whether there is a neurometabolic profile reflecting the underlying neuropathology in individuals with autism spectrum disorders, and if varied profiles correlate with the clinical subtypes. Thirteen children (7-16 years) with autism spectrum...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073808315423
更新日期：2008-07-01 00:00:00
abstract：:To evaluate the consistency of the Hammersmith Infant Neurological Examination scores of very-low-birth-weight infants at 6 and 12 months of age and its correlation to cranial ultrasonography findings, we designed a prospective study between January 2005 and January 2008, in the tertiary Neonatal Unit of Aristotle Uni...
journal_title：Journal of child neurology
pub_type： 杂志文章
doi：10.1177/0883073813479171
更新日期：2014-11-01 00:00:00
