Abstract:
:Animal models have assisted in understanding the mechanisms of brain injury underlying cerebral palsy. Nevertheless, no such models replicate every aspect of the human disease. This review summarizes the classic and more recent studies of the neuropathology of human perinatal brain injury most commonly associated with cerebral palsy, for use by researchers and clinicians alike who need to analyze published animal models with respect to their fidelity to the human disorder. The neuropathology underlying cerebral palsy includes white-matter injury, known as periventricular leukomalacia, as well as germinal matrix hemorrhage with intraventricular extension, and injury to the cortex, basal ganglia, and thalamus. Each has distinctive features while sharing some risk factors, such as prematurity and/or hypoxia-ischemia in the perinatal period. Periventricular leukomalacia consists of diffuse injury of deep cerebral white matter, with or without focal necrosis. Recent work directly in human postmortem tissue has focused on the role of free radical injury, cytokine toxicity (especially in light of the epidemiologic association of periventricular leukomalacia with maternofetal infection), and excitotoxicity in the development of periventricular leukomalacia. Premyelinating oligodendrocytes, which predominate in periventricular regions during the window of vulnerability to periventricular leukomalacia (24-34 postconceptional weeks), are the targets of free radical injury, as determined by immunocytochemical markers of lipid peroxidation and protein nitration. This maturational susceptibility can be attributed in part to a relative deficiency of superoxide dismutases in developing white matter. Microglia, which respond to cytokines and to bacterial products such as lipopolysaccharide via Toll-like receptors, are increased in periventricular leukomalacia white matter and can contribute to cellular damage. Indeed, several cytokines, including tumor necrosis factor-a and interleukins 2 and 6, as well as interferon-g, have been demonstrated in periventricular leukomalacia. Preliminary work suggests a role for glutamate receptors and glutamate transporters in periventricular leukomalacia based on expression in human developing oligodendrocytes. Germinal matrix hemorrhage, with or without intraventricular hemorrhage, occurs in premature infants and can coexist with periventricular leukomalacia. Studies in human germinal matrix tissue have focused on maturation-based vascular factors, such as morphometry and expression of molecules related to the structure of the blood-brain barrier. Gray-matter injury, seen more commonly in term infants, includes cortical infarcts and status marmoratus. Subtle cortical injury overlying periventricular leukomalacia is the subject of current interest as a possible substrate for the cognitive difficulties seen in patients with cerebral palsy. In summary, it is hoped that work in human tissue, in conjunction with experimental animal models, will lead to eventual therapeutic or preventive strategies for the perinatal brain injury underlying cerebral palsy.
journal_name
J Child Neuroljournal_title
Journal of child neurologyauthors
Folkerth RDdoi
10.1177/08830738050200120301subject
Has Abstractpub_date
2005-12-01 00:00:00pages
940-9issue
12eissn
0883-0738issn
1708-8283journal_volume
20pub_type
杂志文章,评审abstract::Gelastic seizure is a rare symptom often associated with hypothalamic hamartoma. We present here a 4-year-old girl with gelastic epilepsy caused by hypothalamic hamartoma and report the magnetic resonance spectrometry and electroencephalographic (EEG) findings. At the age of 2 1/2 years, she developed brief, repetitiv...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307380201700111
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abstract::Isolated cranial nerve paresis in childhood hepatitis A virus infection is rare. The authors report an instance of concomitant right-hand side palatal and abducens palsy, developing in the course of an otherwise uncomplicated hepatitis A virus infection in a 5-year-old girl. The neurological complications were transie...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073808323026
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abstract::Autosomal recessive spinal muscular atrophy is caused by mutations in the survival motoneuron (SMN) gene. There are two nearly identical copies of this gene present on chromosome 5q13; however, only the telomeric copy of this gene is affected in spinal muscular atrophy. In this study, we describe a new method to detec...
journal_title:Journal of child neurology
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doi:10.1177/08830738030180041301
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abstract::MECP2 duplication syndrome is an X-linked genomic disorder that is characterized by infantile hypotonia, intellectual disability, and recurrent respiratory infections. Regression affects a subset of individuals, and the etiology of regression has yet to be examined. In this study, alterations in the hypothalamus-pitui...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073815585577
更新日期:2016-02-01 00:00:00
abstract::A 13-month-old boy presented with repeated episodes of tongue biting during sleep. On evaluation, he was found to have hereditary chin trembling, a rare autosomal dominant condition characterized by continuous or intermittent tremulous activity of the mentalis muscle. This is the first report of this kind from India. ...
journal_title:Journal of child neurology
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doi:10.1177/08830738060210111101
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abstract::Autosomal inherited mitochondrial diseases have been of increasing interest among clinicians and mitochondrial research groups because these diseases are caused through a secondary effect on the mitochondrial DNA. It was thought that the genetic stability of mitochondrial DNA relies on the accuracy of DNA polymerase g...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073808324539
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abstract::Girls with Rett syndrome display signs of neuronal dysfunction including mental retardation, seizures, stereotyped movements, and abnormal breathing and autonomic control. Decelerating head growth during infancy might reflect a disorder in production or pruning of neuronal synapses or both. Recent immunocytochemical s...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/08830738030180100501
更新日期:2003-10-01 00:00:00
abstract::Discrimination of odorous molecules in amniotic fluid occur after 30 weeks' gestation; fetuses exhibit differential responses to maternal diet. Olfactory reflexes enable reliable neonatal testing. Olfactory bulbs can be demonstrated reliably by MRI after 30 weeks' gestation, and their hypoplasia or aplasia also docume...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/0883073817690867
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abstract::Primary spinal atypical teratoid/rhabdoid tumor is extremely rare. The authors present a case of atypical teratoid/rhabdoid tumor occurring in a 4-year-old girl. Magnetic resonance imaging The authors showed an intramedullary mass extending from the bulbomedullary junction to T1 with leptomeningeal dissemination. The ...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073808319319
更新日期:2008-12-01 00:00:00
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journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073807309232
更新日期:2008-04-01 00:00:00
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journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307389100600207
更新日期:1991-04-01 00:00:00
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journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307389601100305
更新日期:1996-05-01 00:00:00
abstract::Four unrelated infants with neonatal thrombocytopenia associated with congenital blindness and porencephaly have been seen over an 18-year period. The association of congenital blindness with neonatal thrombocytopenia has not previously been reported. All children had clinical purpura in the neonatal period; in three ...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307388800300208
更新日期:1988-04-01 00:00:00
abstract::Actinopathies are defined by missense mutations in the ACTA1 gene coding for sarcomeric actin, of which some 70 families have, so far, been identified. Often, but not always, muscle fibers carry large patches of actin filaments. Many such patients also have nemaline myopathy, qualifying actinopathies as a subgroup of ...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/08830738040190021201
更新日期:2004-02-01 00:00:00
abstract::Neonatal alloimmune thrombocytopenia results from platelet-antigen incompatibility between mother and fetus, leading to antibody-mediated destruction of fetal platelets. With a prevalence of 1 in 1000 births, approximately 4000 infants born in the United States each year develop neonatal alloimmune thrombocytopenia. T...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/08830738010160111001
更新日期:2001-11-01 00:00:00
abstract::Executive dysfunction occurs in sickle cell anemia, but there are few early data. Infants with sickle cell anemia (n = 14) and controls (n = 14) performed the "A-not-B" and Object Retrieval search tasks, measuring precursors of executive function at 9 and 12 months. Significant group differences were not found. Howeve...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073812453495
更新日期:2013-10-01 00:00:00
abstract::In this era of changing priorities, regulations, and resources, it is useful to look both back and forward at the building of a division of child neurology in the context of the emergence of child neurology as a nationally and internationally recognized distinct subspecialty of both pediatrics and neurology. Both Pitt...
journal_title:Journal of child neurology
pub_type: 传,历史文章
doi:10.1177/08830738030180020401
更新日期:2003-02-01 00:00:00
abstract::Brain tissue from three patients with a clinical diagnosis of Reye syndrome was compared with tissue from three control patients. All Reye syndrome patients demonstrated cytotoxic cerebral edema, with swelling of astrocyte foot processes, which was not seen in controls. Myelin sheath splitting was seen both in control...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307389100600108
更新日期:1991-01-01 00:00:00
abstract::To investigate the significance of electroencephalogram (EEG) discharges and their treatment, we retrospectively reviewed the charts of 22 children with atypical cognitive development that did not respond to standard educational therapy and demonstrated discharges on EEG. Most children demonstrated no obvious symptoms...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/0883073809344743
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abstract::Paroxysmal kinesigenic dyskinesia is a rare episodic movement disorder that can be isolated or associated with benign infantile seizures as part of choreoathetosis syndrome. Mutations in the PRRT2 gene have been recently identified as a cause of paroxysmal kinesigenic dyskinesia and infantile convulsion and choreoathe...
journal_title:Journal of child neurology
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journal_title:Journal of child neurology
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doi:10.1177/088307389901400206
更新日期:1999-02-01 00:00:00
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journal_title:Journal of child neurology
pub_type: 杂志文章,评审
doi:10.1177/0883073891006001s12
更新日期:1991-01-01 00:00:00
abstract::The aim of this work was to evaluate the cognitive, language, and motor development, after 18 months of life, of nonmicrocephalic children born to mothers with Zika virus infection during pregnancy. Participants were 37 children aged 18-29 months divided into 2 groups: 17 nonmicrocephalic children born to mothers who ...
journal_title:Journal of child neurology
pub_type: 杂志文章
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更新日期:2020-03-01 00:00:00
abstract::This article reports on an 11-year-old boy who was diagnosed with unilateral auditory neuropathy. After failing his annual medical and school hearing screenings, he was referred for audiologic testing, which identified a profound sensorineural hearing loss in his left ear that has remained stable for the past 3 1/2 ye...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/088307380201700415
更新日期:2002-04-01 00:00:00
abstract::The potential benefits of functional magnetic resonance imaging (MRI) for the investigation of normal development have been limited by difficulties in its use with children. We describe the practical aspects, including failure rates, involved in conducting large-scale functional MRI studies with normal children. Two h...
journal_title:Journal of child neurology
pub_type: 杂志文章
doi:10.1177/08830738020170122201
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abstract::Genetic factors play an important role in childhood autism. This study is to determine the association of single-nucleotide polymorphisms in dopa decarboxylase (DDC) and dopamine receptor-1 (DRD1) genes with childhood autism, in a Chinese Han population. A total of 211 autistic children and 250 age- and gender-matched...
journal_title:Journal of child neurology
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更新日期:2016-04-01 00:00:00
abstract::Corpus callosotomy is a palliative procedure performed to reduce the severity of drug-resistant epilepsy. The authors assessed its efficacy on different seizure types in 20 subjects (age range 5-19 years); 8 with active vagus nerve stimulator. Fifteen had complete callosotomy, 3 had anterior 2/3, and 2 had anterior 2/...
journal_title:Journal of child neurology
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abstract::The authors provide an update on most issues related to biology, diagnosis, and treatment of children with ependymoma based on a literature review. Ependymoma is the third most common brain tumor in children and overall survival ranges from 24% to 75% at 5 years. The extent of surgical resection remains the principal ...
journal_title:Journal of child neurology
pub_type: 杂志文章,评审
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journal_title:Journal of child neurology
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更新日期:2015-07-01 00:00:00
abstract::A 14-year-old boy with an episode of acute weakness resembling acute demyelinating encephalomyelitis and polyradiculoneuritis after a febrile illness is described. Molecular analysis showed a mutation at codon 164 of the connexin 32 gene. Neuroradiological and neurophysiological follow-up is reported during acute and ...
journal_title:Journal of child neurology
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更新日期:2010-06-01 00:00:00