Cerebral injury in children undergoing cardiopulmonary bypass (CPB) remains a major source of morbidity. The effect of cardiopulmonary bypass temperature on cerebral function in terms of serum S100beta protein level and cerebral oxygenation monitored by near infrared spectroscopy (NIRO-300) in children is not known. In this study, 18 children undergoing open-heart surgery at the Hospital for Sick Children in London were equally assigned by minimisation to warm (35 +/- 1 degrees C) or cold (25 +/- 1 degrees C) CPB.
View Article and Find Full Text PDFUnlabelled: PURPOSE OF REVIEW Childhood: stroke is more common than brain tumor, but because there is a wide spectrum in terms of etiology and most centers see only a few cases every year, there have been few large studies of genetic and environmental risk factors until recently. This review focuses on the clinical and radiologic methodology required to distinguish phenotypes in patients, and it focuses on the available data on genetic predisposition.
Recent Findings: A number of conditions with Mendelian inheritance (eg, sickle cell disease) predispose to childhood stroke, but the search for epistatic polymorphisms that explain why some but not all of these patients are affected has been hampered by our poor understanding of the pathophysiology.
Conditions associated with arterial ischemic stroke (AIS) in children include congenital heart malformations, sickle cell disease, and meningitis, although around half of all cases are cryptogenic. Up to 80% of children with ischemic stroke have cerebrovascular disease, and case control studies demonstrate an association of arterial ischemic stroke in children with hereditary prothrombotic risk factors and infections such as Varicella. Conventional risk factors, such as hypertension and dyslipidemia, may also play a role and most children have several potential triggers rather than a single cause.
View Article and Find Full Text PDFMoyamoya disease (M-M) is characterized by progressive obstruction of the supraclinoid portion of internal carotid arteries and the proximal middle, anterior and posterior cerebral arteries, associated with the formation of a characteristic net of collateral vessels in the basal ganglia region. Clinical manifestations in childhood include transient ischaemic attacks, seizures and multiple infarcts. Approximately 7% of M-M cases are familial.
View Article and Find Full Text PDFCauses of stroke in children include congenital heart malformations, sickle cell disease, infections, and metabolic disorders. Up to 80% of children with ischemic stroke have cerebrovascular disease, and case control studies demonstrate an association of ischemic stroke in children with hereditary prothrombotic risk factors. There have been no randomized, clinical trials for primary prevention, short-term treatment, or secondary prevention of pediatric ischemic stroke.
View Article and Find Full Text PDFWe present data on the known risk factors encountered in children presenting with a first arterial ischemic stroke to a single tertiary center over 22 years. Two hundred twelve patients (54% male; median age, 5 years) were identified. One hundred fifteen (54%) were previously healthy.
View Article and Find Full Text PDFObjective: To ascertain whether posterior circulation stroke in children has distinctive clinical or radiologic features.
Methods: Patients were identified retrospectively from two pediatric neurology centers. Clinical details were ascertained by chart review, and radiologic data were reviewed by three neuroradiologists.
The pathogenesis of acute painful crisis in children with sickle cell disease is poorly understood; suggested risk factors include sickle cell type, severity of anemia, fetal hemoglobin concentration, and hypoxemia from upper airway obstruction. In a cohort study of 95 patients the relationship between clinical, laboratory, and sleep study data and frequency of painful crisis was investigated. Both univariate and multiple regression modeling showed that low nocturnal oxygen saturation was highly significantly associated with a higher rate of painful crisis in childhood (P <.
View Article and Find Full Text PDFThe aim of this study was to describe the functional consequences of childhood stroke in terms of activity limitation and to explore the relationship between extent of brain damage, impairment, and functional sequelae. A further aim was to describe the health of the parents of these children. Seventeen children and adolescents with cerebral infarction in the territory of the middle cerebral artery were enrolled in the study.
View Article and Find Full Text PDFJapanese encephalitis (JE) causes at least 10 000 deaths each year. Death is presumed to result from infection, dysfunction and destruction of neurons. There is no antiviral treatment.
View Article and Find Full Text PDFBackground And Purpose: Ischemic symptoms in patients with moyamoya syndrome (MMS) are usually due to hemodynamically mediated perfusion failure, and identification of abnormal tissue perfusion in these patients is therefore clinically important. Although dynamic susceptibility contrast (DSC) MRI can be used to study tissue perfusion, there are potential technical problems in MMS. This study investigates the scope and limitations of perfusion MRI in the clinical evaluation of such patients.
View Article and Find Full Text PDFIdiopathic "benign" intracranial hypertension is an uncommon but important cause of headache that can lead to visual loss. This study was undertaken to review our experience in the diagnosis and management of idiopathic intracranial hypertension, giving special attention to treatments used. A retrospective chart review was conducted on 32 patients diagnosed with idiopathic intracranial hypertension between 1984 and 1995.
View Article and Find Full Text PDFBackground: Central-nervous-system (CNS) events, including strokes, transient ischaemic attacks, and seizures are common in sickle-cell disease. Stroke can be predicted by high velocities in the internal-carotid or middle-cerebral arteries on transcranial doppler ultrasonography. We tested the hypothesis that nocturnal hypoxaemia can predict CNS events better than clinical or haematological features, or transcranial doppler sonography.
View Article and Find Full Text PDFNeurological complications are common in sickle cell disease (SCD). However, it is often difficult to relate the clinical presentation to conventional neuroimaging, because subclinical infarction is common and stroke has been described in the absence of large-vessel disease. We studied 48 patients with SCD aged 4-34 (median 13) years with T2-weighted, diffusion and perfusion magnetic resonance imaging (MRI) and with MR angiography.
View Article and Find Full Text PDFIn this study of 118 children (median age 5.1 years; range 6 months to 17 years) with ischaemic stroke or transient ischaemic attack (TIA), 22 children (19%) were homozygous for the thermolabile variant of the methylenetetrahydrofolate reductase allele (t-MTHFR), compared with nine of 78 (12%) of a reference population (p=0.18, OR 1.
View Article and Find Full Text PDFBackground: Seizures are a prominent feature of childhood cerebral malaria, and are associated with an increased risk of death and neurological sequelae. We present the electroencephalographic (EEG) findings from a detailed clinical and electrophysiological study.
Methods: Children with cerebral malaria had EEGs recorded within six hours of admission, and at 12 hourly intervals until recovery of consciousness.
Proc Natl Acad Sci U S A
February 2001
Choline acetyltransferase (ChAT; EC ) catalyzes the reversible synthesis of acetylcholine (ACh) from acetyl CoA and choline at cholinergic synapses. Mutations in genes encoding ChAT affecting motility exist in Caenorhabditis elegans and Drosophila, but no CHAT mutations have been observed in humans to date. Here we report that mutations in CHAT cause a congenital myasthenic syndrome associated with frequently fatal episodes of apnea (CMS-EA).
View Article and Find Full Text PDFWe hypothesized that vaso-occlusive events in childhood sickle cell disease (SCD) may relate to inflammatory cell activation as well as interactions between sickle erythrocytes and vascular endothelium. Peripheral blood was examined from 24 children with SCD, of whom 12 had neurological sequelae and seven had frequent painful crises, and 10 control subjects. Platelet (CD62P and CD40L expression) and granulocyte (CD11b expression) activation and levels of platelet-erythrocyte and platelet-granulocyte complexes were determined by flow cytometry.
View Article and Find Full Text PDFA parental questionnaire was used to investigate the outcome for children who had had ischaemic stroke, who were seen at Great Ormond Street Hospital, London between 1990 and 1996. The results of functional assessments carried out by a physiotherapist and an occupational therapist, and of quantitative evaluations carried out by a neuropsychologist were used for validation where possible. The relationship between clinical and radiological factors and outcome were examined.
View Article and Find Full Text PDFWe report the cases of two children with cranial diabetes insipidus who were treated with lamotrigine for seizures and who had accompanying changes in desmopressin requirements. Lamotrigine is a new anticonvulsant chemically unrelated to other existing antiepileptic drugs. Studies suggest it acts at voltage-sensitive sodium channels and also decreases calcium conductance.
View Article and Find Full Text PDFReview of published clinical and neuropsychologic outcome studies reveals limited information about intellectual functioning after childhood stroke. The extant data are supplemented here by analysis of intelligence quotient (IQ) results obtained from 38 children in an ongoing study of unilateral middle cerebral artery ischemic stroke. Evidence so far indicates that, after stroke, mean IQ falls significantly below the population mean but remains within the average range.
View Article and Find Full Text PDFSince early recurrence occurs in at least 10% of patients presenting with their first stroke in childhood in the reported series, the search for modifiable risk factors should be a priority. Risk factors for stroke in adults include hypertension, diabetes, and smoking, as well as cardiac disease and sickle cell anemia; asymptomatic cerebrovascular disease and transient ischemic events may predict stroke in this age group. The investigation of a child with a stroke has traditionally focused on finding a single cause rather than looking for risk factors to which the patient may be exposed life long.
View Article and Find Full Text PDF