bluuebunny/crossref_metadata_2025 huggingface dataset:
Predictors of Ventriculoperitoneal Shunt Among Babies With Intraventricular Hemorrhage
Authors: Hansen Anne R. , Allred Elizabeth N. , Leviton Alan
Published: December 2008

We aimed to identify medical care practices that influence the need for ventriculoperitoneal shunt among infants who develop intraventricular hemorrhage. We reviewed the medical records of 82 babies with ultrasonographically documented intraventricular hemorrhage. We compared the 10 babies who required a ventriculoperitoneal shunt to the 72 controls who had intraventricular hemorrhage, but did not require a ventriculoperitoneal shunt or die, prior to discharge. We considered maternal, perinatal, and neonatal risk factors as potential predictive variables. Maternal preeclampsia, prenatal steroids, and cesarean delivery were associated with a reduced risk of shunt. Patients who did require a shunt were more likely than their nonshunted peers to be treated with dopamine, to receive greater volumes of total intravenous fluid, largely as albumin and red blood cells, and to have a higher incidence of acidosis, patent ductus arteriosus and systolic hypertension. Previously identified antecedents and correlates of intraventricular hemorrhage appear also to be the antecedents and correlates of progression to ventriculoperitoneal shunt among infants with intraventricular hemorrhage. These findings are consistent with the possibility that prenatal and postnatal care practices influence the risk for ventriculoperitoneal shunt among babies with intraventricular hemorrhage. This offers the promise that changes in obstetric and neonatal care will reduce the need for ventriculoperitoneal shunt in very low birthweight infants. (J Child Neurol 1997;12:381-386).
Vitamin D Levels in Noninstitutionalized Children With Cerebral Palsy
Authors: Henderson Richard C.
Published: December 2008

Calcitriol (1,25-dihydroxy vitamin D) is an important hormone in calcium and phosphate metabolism. Levels of calcitriol and its precursor, 25-hydroxy vitamin D (calcidiol), were measured in a heterogeneous group of 125 noninstitutionalized children and adolescents with spastic cerebral palsy. Levels of each were correlated with: (1) clinical factors including mobility, prior fracture, and use of anticonvulsants; (2) nutrition and growth parameters including skinfolds, body mass index, and use of vitamin supplements; and (3) other serum analyses including osteocalcin as a marker of bone formation, calcium, and alkaline phosphatase. Levels of calcidiol and calcitriol did not correlate with any of the various clinical, nutritional, or growth parameters examined. The prevalence of low (< 10 ng/mL) levels of calcidiol was significant (19%), and dependent on the season of the year in which the level was measured. In contrast, less than 2% of the patients were found to have a low (< 20 pg/mL) level of calcitriol and the mean was comparable to normal pediatric subjects. Levels of calcitriol are maintained in noninstitutionalized children with cerebral palsy despite anticonvulsants, poor nutrition, and calcidiol levels that vary greatly with the seasons. (J Child Neurol 1997;12:443-447).
Landau-Kleffner Syndrome: Metabolic Abnormalities in Temporal Lobe Are a Common Feature
Authors: da Silva Ednéa A. , Chugani Diane C. , Muzik Otto , Chugani Harry T.
Published: December 2008

Landau-Kleffner syndrome (acquired epileptic aphasia) is characterized by language regression following normal acquisition of language skills, accompanied by epileptiform abnormalities on the electroencephalogram (EEG) with or without clinical seizures. Continuous spikes and waves during slow wave sleep may be seen on the EEG, but are not required to make the diagnosis. Structural neuroimaging with computed tomography (CT) and magnetic resonance imaging (MRI) is typically normal. We have evaluated 17 children (aged 2.4 to 10.6 yr) with Landau-Kleffner syndrome using positron emission tomography (PET) with 2-deoxy-2-[18F]fluoro-D-glucose (FDG) in order to determine whether there are metabolic abnormalities common to this syndrome. Patients were awake for the uptake period of FDG, and the EEG was monitored. On a visual analysis of the PET images, patients showed metabolic abnormalities in the temporal lobes. Two children had focal hypermetabolism in the left temporal cortex, one of whom also showed right temporal cortex hypometabolism. The remaining patients (n = 15) showed bilateral temporal hypometabolism, and comparison of these patients with a neurologically normal age-matched control group (n = 8) demonstrated significantly reduced glucose metabolism bilaterally in middle temporal gyrus (P < .02). In addition, other cortical regions displayed hypometabolism, although these regions were not consistently abnormal in all patients. The finding of temporal lobe abnormalities in all Landau-Kleffner syndrome patients suggests that temporal lobe structures are important in the pathophysiology of this syndrome, whereas the presence of additional cortical abnormalities in many patients indicates that extensive brain functional disturbances are common. (J Child Neurol 1997; 12:489-495).
Topical Review: Head Injury in Children
Authors: Adelson P. David , Kochanek Patrick M.
Published: December 2008

Trauma in the United States is the leading cause of death and disability in the pediatric population. Differences of age and development affect recovery and outcome following head injury. Mechanisms and pathophysiology of head injury are varied in both the pediatric and adult populations. Assessments of injury are varied and some measurements are more sensitive than others as well as more specific with regard to certain types of injury. Treatment and management should be tailored to each case in order to effect a positive outcome with respect to brain functioning. Aggressive intervention for prevention of primary and secondary injury must be continued and understanding of the impact of these injuries should provide for a brighter future for these patients. (J Child Neurol 1998; 13:2-15).
Transient Cerebral Arteriopathy: A Disorder Recognized by Serial Angiograms in Children With Stroke
Authors: Chabrier Stéphane , Rodesch Georges , Lasjaunias Pierre , Tardieu Marc , Landrieu Pierre , Sébire Guillaume
Published: December 2008

Repeated clinical evaluation and cerebral arteriography during the evolution of ischemic strokes of idiopathic origin allowed us to characterize a transient cerebral arteriopathy. We retrospectively studied the clinical characteristics, course, and neuroimaging features of this disorder in nine children. Of 34 children with ischemic strokes seen consecutively between 1984 and 1995, 9 (26%) were diagnosed as having transient attack of the cerebral arterial wall, termed transient cerebral arteriopathy. All of these patients had previously been in good health. The mean age at the time of the first stroke was 6 years (range, 29/12 years to 134/12 years). All children presented with acute hemiplegia. A recurrence of the stroke took place 3 months at the latest after the initial infarct in three children (mean clinical follow-up 27/12 years). Cerebral imaging in all the patients showed small subcortical infarcts located in basal ganglia or internal capsule. Arteriography revealed multifocal lesions of the arterial wall (focal stenosis or segmental narrowing), mostly located in the initial parts of basal arteries of the carotid system. Longitudinal arteriographic follow-up showed initial worsening of these arterial lesions (n = 5) for a maximum duration of 7 months followed by complete regression (n = 2), improvement (n = 5), or stabilization of the lesions (n = 2). Five patients had a complete clinical recovery. Further studies are necessary to confirm a presumed inflammatory cause of this arteriopathy. (J Child Neurol 1998; 13:27-32).
Alternating Hemiplegia of Childhood: Insights Into Its Pathophysiology
Authors: Rho Jong M. , Chugani Harry T.
Published: December 2008

Alternating hemiplegia of childhood is a rare disorder characterized by episodic paroxysmal attacks of neurologic dysfunction and has historically been thought to represent a migraine equivalent, an unusual form of epilepsy, or a movement disorder. Medical treatment with a variety of anticonvulsants and migraine agents is largely unsuccessful, and progressive mental deterioration is universally expected. Despite extensive laboratory studies, the pathophysiologic basis of this condition has thus far eluded identification. Recently, an international workshop was held to generate testable hypotheses regarding the pathophysiology of alternating hemiplegia of childhood. This paper summarizes the major proceedings of that workshop, and hopes to stimulate further interest in elucidating the molecular and cellular mechanisms underlying this unusual disorder. (J Child Neurol 1998;13:39-45).
Psychosocial Adjustment of Children With Spina Bifida
Authors: Zurmohle Uwe-Martin , Homann Thomas , Schroeter Christiane , Rothgerber Hans , Hommel Gerhard , Ermert Johann August
Published: December 2008

It was the aim of the present prospective study to investigate the influence of age, sex, intellectual function, and school type as well as of hydrocephalus, the level of lesion, and of the degree of handicap on the psychosocial adjustment of children with spina bifida. Seventy-five patients with spina bifida, aged 6 to 16 years were assessed concerning their psychosocial adjustment and their intellectual function by use of standardized instruments. The findings were compared with those of nondisabled controls, matched for age and sex. Children with spina bifida showed a tendency to be at an increased risk for psychosocial maladjustment. Influencing factors were age, sex, and the degree of handicap. Twelve- to 16-year-old boys and girls displayed significant adjustment problems in specific areas in comparison with their controls. There was a tendency for children with spina bifida to be attending inappropriate school types according to their intellectual abilities. Perhaps the most striking finding of our study was that children with spina bifida who attended a school for disabled children, even though it might be an IQ-appropriate setting, had a higher rate of psychosocial maladjustment than the disabled children in mainstream schools. (J Child Neurol 1998;13:64-70).
Intravenous Immunoglobulin Treatment of Children With Autism
Authors: Plioplys Audrius V.
Published: December 2008

Since autism has been associated with immunologic abnormalities suggesting an autoimmune cause of autistic symptoms in a subset of patients, this study was undertaken to investigate whether intravenous immunoglobulin (IVIg) would improve autistic symptoms. Ten autistic children with immunologic abnormalities, demonstrated on blood tests, were enrolled in this study. Their ages ranged from 4 to 17 years, with two girls and eight boys. Eight children (1 female and 7 male) historically had undergone autistic regression. Intravenous immunoglobulin, 200 to 400 mg/kg, was administered every 6 weeks for an intended treatment program of four infusions. In five children, there was no detectable change in behavior during the treatment program. In four children, there was a mild improvement noted in attention span and hyperactivity. In none of these children did the parents feel that the improvement was sufficient to warrant further continuation of the infusions beyond the termination of the program. Only in one child was there a very significant improvement, with almost total amelioration of autistic symptoms over the time period of the four infusions. Once the treatment program was completed, this child gradually deteriorated over a 5-month time period and fully reverted to his previous autistic state. In this treatment program, five children had no response to intravenous immunoglobulin. In the four children who showed mild improvements, those improvements may simply have been due to nonspecific effects of physician intervention and parental expectation (ie, placebo effect). However, in one child there was a very significant amelioration of autistic symptoms. There were no distinguishing historic or laboratory features in this child who improved. Given a positive response rate of only 10% in this study, along with the high economic costs of the immunologic evaluations and the intravenous immunoglobulin treatments, the use of intravenous immunoglobulin to treat autistic children should be undertaken only with great caution, and only under formal research protocols. (J Child Neurol 1998;13:79-82).
Drug Therapy in Juvenile Dermatomyositis: Follow-Up Study
Authors: Ng Yu-tze , Ouvrier Robert A. , Wu Tammy
Published: December 2008

A series of 33 patients with juvenile dermatomyositis was reviewed in terms of their prognosis in relation to their drug therapy. This retrospective study was intended to help clarify the use of various therapies in this rare, heterogeneous disease from our hospital's experience in the last 24 years. The results confirmed that oral corticosteroids should remain the undisputed first line of treatment. For more refractory, chronic patients, the results suggest that azathioprine should be the favored drug of first choice (in addition to corticosteroids). There may be a role for cyclosporine as a "rescue" treatment, but this needs to be further defined. (J Child Neurol 1998;13:109-112).
Acute-Phase Neurologic Complications of Haemophilus Influenzae Type b Meningitis: Association With Developmental Problems at School Age
Authors: Taylor H. Gerry , Schatschneider Christopher , Watters Gordon V. , Mills Elaine L. , Gold Ronald , MacDonald Noni , Michaels Richard H.
Published: December 2008

The purposes of this study were to describe the incidence of acute-phase neurologic complications in a sample of 126 children with Haemophilus influenzae type b meningitis, and to determine if these complications were associated with higher rates of learning and behavior problems at school age. Risks were assessed by comparing rates of adverse psychoeducational outcomes in the 53 children in the sample with complications to corresponding outcome rates in the 67 children who were free of neurologic complications and who did not have abnormal electroencephalograms (EEGs) or computed tomographic (CT) scans. Comparisons were made by means of logistic regression analysis. Twenty-nine children (23% of the sample) had seizures, 16 (13%) were comatose or obtunded, 15 (12%) had sensorineural hearing loss, 8 (6%) had hemiparesis, and 7 (6%) had cranial nerve deficits other than hearing loss. Relative to children without complications, those with complications had higher rates of grade repetition and substandard performance on neuropsychological and achievement testing. Adverse outcomes, however, consisted primarily of more subtle cognitive and learning problems; only two of the children in the sample obtained prorated IQ scores below 70. Sequelae were associated with persistent neurologic deficits and bilateral hearing loss, as well as with transient symptoms including seizures, coma, and hemiparesis. While study findings argue against adverse consequences for the vast majority of children treated for this disease, the results clarify learning and behavior outcomes and indicate which children are at greatest risk. ( J Child Neurol 1997;13:113-119).
Regional Development of Glutamate-N-Methyl-D-Aspartate Receptor Sites in Asphyxiated Newborn Infants
Authors: Andersen Danielle L. , Tannenberg Anthony E.G. , Burke Christopher J. , Dodd Peter R.
Published: December 2008

The N-methyl-D-aspartate (NMDA) subclass of glutamate receptors was examined in newborn infants dying between 25 weeks' gestation and term, either from acute cerebral hypoxia, or from other noncerebral conditions incompatible with life. Frontal, occipital, temporal, and motor cortex tissue samples were obtained at autopsy (post mortem delay: median, 45.9 hr; range, 24-96 hr) and frozen for subsequent [ 3H]MK801 homogenate binding assays. Whereas no significant variation was observed in ligand affinity (KD), in all cases receptor density (BMAX) increased with gestational age, in occipital cortex (27 weeks, BMAX = 222 ± 44 fmol·mg protein-1; 39 weeks, 439 ± 42 fmol·mg protein-1), but not in motor or temporal cortex. The gestational-age increase also occurred in control frontal cortex (27 weeks, 284 ± 80; 39 weeks, 567 ± 40 fmol·mg protein-1), but was significantly less marked in frontal cortex in hypoxia cases (27 weeks, 226 ± 90; 39 weeks, 326 ± 47 fmol·mg protein-1). In all cortical areas except temporal, the maximal response to glutamate did not vary across case groups. Hypoxia cases showed an increased response to glutamate enhancement selectively in temporal cortex. Binding site density did not correlate with degree of hypoxia as assessed pathologically, suggesting that receptor differences preceded the hypoxic episode. Regional differences in glutamate-NMDA receptor sites may underlie increased vulnerability to hypoxia at birth. (J Child Neurol 1998;13:149-157).
Abnormal Frequency Mismatch Negativity in Mentally Retarded Children and in Children With Developmental Dysphasia
Authors: Holopainen Irma E. , Korpilahti Pirjo , Juottonen Kirsi , Lang Heikki , Sillanpää Matti
Published: December 2008

The auditory event-related potential waveform termed "mismatch negativity" was examined in 12 mentally retarded children with delayed development of speech and language (aged 5-8 years) and in 13 children with developmental dysphasia (aged 5-9 years). The mismatch negativity waves were elicited with pure sine-wave tone stimuli using the oddball paradigm. We measured the peak latency and peak amplitude of mismatch negativity responses to frequency (500/553 Hz) difference. The mismatch negativity patterns were compared with those of 10 children with normal development of linguistic skills (aged 5-9 years). In both the mentally retarded and dysphasic groups, the peak amplitude of the frequency mismatch negativity was significantly attenuated when compared with the control group, but no significant difference was observed between the mentally retarded and dysphasic groups. Attenuated frequency mismatch negativity was related to impairment of linguistic skills irrespective of the child's cognitive skills. Because the mismatch negativity response reflects central auditory processing and is modal specific for auditory stimuli, this change-specific response can serve as an objective tool to elucidate central auditory deficits in children. (J Child Neurol 1998;13:178-183).
Congenital Muscular Dystrophy With Complete Laminin-α2-Deficiency, Cortical Dysplasia, and Cerebral White-Matter Changes in Children
Authors: Tsao Chang-Yong , Mendell Jerry R. , Rusin Jerome , Luquette Mark
Published: December 2008

Congenital muscular dystrophy consists of Fukuyama congenital muscular dystrophy, Walker-Warburg syndrome, muscle-eye-brain disease, and occidental congenital muscular dystrophy, which is further divided into laminin-α2-positive and laminin-α2-negative subgroups. These forms of congenital muscular dystrophy are frequently associated with abnormal white-matter changes, whereas the Fukuyama form, Walker-Warburg syndrome, and muscle-eye-brain disease are also frequently found to have polymicrogyria. We now report two infants with complete laminin-α2-deficiency who have not only abnormal cerebral white-matter lesions, but also bioccipital polymicrogyria. There are significant similarities in the clinical and cerebral manifestations among the various types of congenital muscular dystrophy. The diagnosis of the Fukuyama form, laminin-α2-deficiency, Walker-Warburg syndrome, and muscle-eye-brain disease cannot always be established on radiological studies alone. (J Child Neurol 1998;13:253-256)
REM-Sleep Motor Disorder in Children
Authors: Sheldon Stephen H. , Jacobsen John
Published: December 2008

In 1986, Schenck and coworkers described REM-sleep behavior disorder as a treatable parasomnia affecting older males. This disorder is characterized by intermittent loss of the muscle atonia, which normally characterizes the rapid eye movement (REM) sleep state. Complex motor behaviors occur that are often injurious and are associated with dreaming. We have identified five children who meet the criteria for REM-sleep behavior disorder and describe the clinical and polysomnographic characteristics of these patients, along with corresponding polysomnographic characteristics of matched comparison subjects. (J Child Neurol 1998; 13:257-260).
Asthma and Epilepsy: Are They Related? A Retrospective Study
Authors: Castaneda Glenn Y. , Heilbroner Peter L. , Shah Nishant , Forem Sandra , Fish Irving
Published: December 2008

Numerous studies have suggested that epilepsy and asthma may be related conditions. There has, however, been little epidemiologic data published to support this association. We conducted a retrospective study to determine whether the prevalence of epilepsy is increased among children with asthma, and the prevalence of asthma is increased among children with epilepsy, in comparison with the general pediatric population. We reviewed the medical records, at a large city hospital, of two groups of pediatric outpatients: (1) 400 consecutive patients with asthma followed regularly at the asthma and allergy clinic; and (2) 201 consecutive patients with idiopathic epilepsy followed regularly at the pediatric neurology clinic. Patients with a history of birth prior to 36 weeks' gestational age were excluded. Among the 400 cases of asthma, there were three patients with idiopathic epilepsy (prevalence of 0.75%). The prevalence of epilepsy was similar in mild (0.79%) and moderate-to-severe (0.73%) asthma. Among the 201 cases of idiopathic epilepsy, there were 12 patients with asthma (prevalence of 5.97%). Similar percentages of epilepsy patients with and without asthma reported generalized tonicclonic, complex partial, simple partial, and myoclonic seizures as their predominant type. The prevalence values in this study are consistent with the prevalence of epilepsy and asthma in the general pediatric population. Our findings therefore suggest that idiopathic epilepsy and asthma are not etiologically related or mutually predisposing conditions. Small samples, failure to exclude patients born prematurely, and the equation of electroencephalographic (EEG) abnormalities with epilepsy may account for the results of previous studies. (J Child Neurol 1998; 13:283-285).
Topical Review: Whimpers and Bangs: Of Death and Dying in the Nervous System
Authors: Schor Nina Felice
Published: December 2008

Mechanisms of cell death in general and in the nervous system in particular have received increasing attention over the past several years. Recent studies have begun to address the pathogenetic and therapeutic implications of these mechanisms vis-a-vis human disease. This review is aimed at putting the rapidly evolving vocabulary and body of knowledge regarding cell death in the perspective of neurologic disease of childhood. (J Child Neurol 1998; 13:307-312).
Proton Magnetic Resonance Spectroscopy in Children With Sturge-Weber Syndrome
Authors: Moore Gregory J. , Slovis Thomas L. , Chugani Harry T.
Published: December 2008

Quantitative proton magnetic resonance spectroscopy was performed on six children with Sturge-Weber syndrome following gadolinium enhanced magnetic resonance imaging (MRI). MRI revealed only unilateral involvement in all cases. The mean concentration (mmol/kg wet weight) of the neuronal marker N-acetyl-aspartate was significantly reduced by 37% in the ipsilateral gadolinium enhanced volume of interest compared to a similarly placed contralateral volume of interest (5.39 ± 1.70 [SD] vs 8.50 ± 1.14, P < .005, two-tailed paired Student's t-test). Decreased N-acetyl-aspartate in the ipsilateral volume of interest was observed in all patients studied. No significant differences were found in the concentrations of creatine/phosphocreatine or choline compounds between the ipsilateral and contralateral volumes of interest. These findings give possible new insight into the pathophysiology of this disease and suggest that quantitative proton magnetic resonance spectroscopy may be useful for the early characterization and monitoring of neuronal dysfunction or loss in infants and children with Sturge-Weber syndrome. (J Child Neurol 1998; 13:332-335).
Topical Review: Pathologic Basis of the Symptomatic Epilepsies in Childhood
Authors: Duncan Armstrong Dawna , Mizrahi Eli M.
Published: December 2008

The epilepsies in childhood are classified as primary (or idiopathic) and secondary (or symptomatic). The primary epilepsies account for two thirds of all childhood epilepsies and are presumed to be genetically determined. In the remaining one third of cases, a neuropathologic lesion can be identified. This paper summarizes the etiologies of the symptomatic epilepsies. They are classified according to the pathologic processes; malformative, metabolic, neoplastic and phakomatoses, hypoxic-ischemic, infectious, and of unknown pathologic process. (J Child Neurol 1998;13:361-371).
Children With Familial Cryptogenic Epilepsy Have a Favorable Seizure Prognosis
Authors: MacIntosh Donald S. , Camfield Peter R. , Camfield Carol S.
Published: December 2008

We examined the prognosis for children with epilepsy not conforming to a genetic syndrome, who also had close relatives with epilepsy. Probands, with no identified cause for epilepsy except a first-degree relative with epilepsy, were identified from a population-based cohort of 504 children in Nova Scotia, with onset of seizures between 1977 and 1985. The primary outcome measure was seizure remission after an average of 15 years follow-up for probands (n = 27) and 26 years for their affected relatives (n = 32). Of probands, 92% were seizure free for 3 or more years at the end of follow-up, compared with 76% of relatives. When seizures began before age 12 years, 96% of probands and 94% of affected relatives were seizure free at the end of follow-up. There was little concordance for the details of the clinical course between probands and affected family members. This high level of remission was considerably better than for similar patients from the original Nova Scotian cohort (P < .02). We conclude that children with epilepsy not conforming to a well-defined genetic syndrome, but with an affected first-degree relative, have a remarkably good prognosis. (J Child Neurol 1998; 13:372-376).
Auditory Hallucinations in Nonpsychotic Children With Affective Syndromes and Migraines: Report of 13 Cases
Authors: Schreier Herbert A.
Published: December 2008

This report describes the discovery of a possible association between auditory hallucinations, migraine, and affective/anx iety disorders in nonpsychotic children. The cases were culled by a review of all consultations in an outpatient practice in an 8-month period. Thirteen cases of nonpsychotic children who experienced hallucinations (auditory in 12) were found. All but one suffered from a variety of major affective or anxiety/panic disorders and migraine headaches. The family histories were strongly positive for affective/anxiety disorders and migraine, and four of the parents also had a history of hearing voices. The age of onset of the auditory hallucinations, where known (8 cases), was between 4 and 8 years. In only two cases did the voices accompany the migraine attacks, and these two children also heard voices at other times. Although a strong association between migraine and anxiety, panic, and affective syndromes in adults has been repeatedly found in epidemiologic study, no such association has been studied in children, and this is the first known report of a possible association between migraine, affective/anxiety disorders, and auditory hallucinations in nonpsychotic children. It suggests the need for epidemiologic study. (J Child Neurol 1998; 13:377-382).
Temperament Profiles of Children With Vein of Galen Malformations
Authors: Nass Ruth , Melnick John , Berenstein Alejandro
Published: December 2008

Unlike many brain injured children who are often rather difficult, toddlers and school-aged children with congenital vein of Galen malformations (n = 20; age range, 4 months to 12 years with a mean of 4 years) evidence a relatively positive temperament profile (based on the Temperament Scales of Carey, McDevitt, Fullard, Hegvik, Medoff-Cooper). They do not, however, differ from normal children with regard to the frequency of easy, difficult, slow to warm up, and intermediate temperament clusters. The temperament profile of children with vein of Galen malformations appears unaffected by additional neurologic abnormalities (hydrocephalus, abnormal developmental quotient, seizure disorder, focal cortical lesions) or other factors including age at testing, sex, or socioeconomic status. Contrary to the findings in adults of right hemisphere dominance for attention, presence of additional right-sided focal cortical pathology was not associated with greater activity level, distractibility, or impersistence. In contrast to the findings in adults and children of right hemisphere dominance for emotions in general, children with vein of Galen malformations and additional right hemisphere cortical damage are not more difficult than those with additional left cortical pathology or no additional focal cortical pathology. The fact that the pathology in vein of Galen malformations is subcortical may explain their relatively easier temperament and the absence of lateralization effects. (J Child Neurol 1998;13:387-390).
Neurobehavioral Development in Joubert Syndrome
Authors: Gitten Jill , Dede Duane , Fennell Eileen , Quisling Ronald , Maria Bernard L.
Published: December 2008

Research on children with Joubert syndrome has focused on brain structural abnormalities and associated clinical symptoms. The degree of developmental delay has not been objectively reported. We investigated the neurobehavioral development of children with Joubert syndrome through neurobehavioral assessment in the largest sample to date. Thirty-two parents of children with Joubert syndrome completed the Child Development Inventory and magnetic resonance imaging (MRI) data was gathered on 17 of these children. Results indicate that 94% were severely impaired according to the Child Development Inventory, with age being positively correlated with degree of neurobehavioral impairment. The average developmental age of our sample was 19 months (63% below chronological age). Severity of illness as measured by the General Development scale of the Child Development Inventory and severity of illness as measured by MRI (overall severity rating) did not yield consistent data regarding severity of the midbrain and cerebellar malformations. Similarly, markers of abnormal cerebral development such as cortical atrophy and delayed myelination were independent of severity of illness ratings on the Child Development Inventory. The degree of developmental delay in Joubert syndrome and the severity of gross central nervous system malformations appear independent. (J Child Neurol 1998;13:391-397).
Frequency of Neurologic Disorders in the Neonatal Intensive Care Unit
Authors: Sheth Raj D.
Published: December 2008

Neonatal intensive care unit survival rates have improved significantly over the past decade. This improvement primarily reflects declining mortality rates among preterm infants. Neurologic morbidity increases with prematurity and is the major predictor of long-term disability. Accordingly, concern has been expressed that the burden of neurologic dysfunction among contemporary neonatal intensive care unit survivors may be increasing. To define the trends of neurologic disorders in the contemporary neonatal intensive care unit, all 4164 admissions between 1986 and 1995 to a tertiary neonatal intensive care unit were examined. Neonatal intensive care unit admissions (413 ± 49 per year), proportion of births at less than 37 weeks (70 ± 3% per year), and referral patterns were stable between 1986 and 1995. Over the study period, 773 (18%) of 4164 neonatal intensive care unit infants had a total of 1062 neurologic disorders. The neonatal intensive care unit mortality rate declined from 12% in 1986 to 4.2% in 1995 (P < .01). Neurologic disorders declined, from 27% of infants born in 1986 to 12% in 1995 (P < .001): 356 had seizures (14% in 1986 to 4% in 1995; P < .001), 235 had hypoxic-ischemic encephalopathy (8% in 1986 to 4% in 1995, P < .01), and 167 had intraventricular hemorrhage (7% in 1986 to 1.4% in 1995, P < .005). Frequency of congenital or chromosomal aberration affecting the nervous system was relatively constant (4.5% per year). Despite a three-fold improvement in neonatal intensive care unit survival between 1986 and 1995, the frequency of perinatally acquired neurologic disorders declined by more than 50%. (J Child Neurol 1998;13:424-428).
Respiratory Sinus Arrhythmia in Children With Severe Cyanotic and Pallid Breath-Holding Spells
Authors: DiMario Francis J. , Bauer Lance , Baxter David
Published: December 2008

In this study we investigated centrally mediated parasympathetic regulation of modulated cardiac vagal tone among children with severe cyanotic and pallid breath-holding spells by examining respiratory sinus arrhythmia. Respiratory sinus arrhythmia was evaluated in 41 children; 17 subjects with cyanotic breath-holding spells (6 boys, 11 girls; mean age 37.1 months), 7 subjects with pallid breath-holding spells (2 boys, 5 girls; mean age 33.0 months), and 17 controls (8 boys, 9 girls; mean age 41.2 months). Subjects had recurrent (more than 3) severe breath-holding spells. Each subject's electrocardiogram was recorded in a quiet room and digitized by an 80386 personal computer during five 1-minute periods. R-R intervals within each 1-minute period were converted to heart rate in 120 successive 0.5-second intervals. The resultant heart rate time series was converted to its underlying frequency composition by a fast Fourier transform and averaged across minutes. Respiratory sinus arrhythmia was defined as the variability in the time series over a frequency range (0.096 Hz to 0.48 Hz) corresponding to a range of respiratory rates from 6 to 30 breaths per minute. ANCOVA adjusting for age and sex was conducted with the subject group as the independent measure. There were no significant differences between subjects with cyanotic breath-holding spells and controls. Pallid breath-holding spell subjects had a marked difference in respiratory sinus arrhythmia from either controls or subjects with cyanotic breath-holding spells, demonstrating less variability in respiratory sinus arrhythmia (P < .042) This study supports the hypothesis that there exists autonomic dysregulation in pallid breath-holding spells, caused by a primary central parasympathetic disturbance distinct from the dysregulation found in cyanotic breath-holding spells. (J Child Neurol 1998;13:440-442).
Topical Review: Understanding Sleep Ontogeny to Assess Brain Dysfunction in Neonates and Infants
Authors: Scher Mark S.
Published: December 2008

The ontogenetic framework onto which a child's sleep is constructed undergoes significant developmental alterations during early life. Sleep state behaviors, in large part, reflect continuities from fetal through neonatal time periods. Major changes in sleep organization subsequently occur throughout infancy. Maturational expressions of sleep behaviors must be understood by the pediatric neurologist before specific physiologic phenomena can be assessed as transient sleep disturbances or clinically relevant sleep disorders. The first part of this two-part review article focuses on the major aspects of developmental sleep physiology in the first few months of life. Recognition of age-specific electroencephalographic/polysomnographic patterns will facilitate the child neurologist's evaluation of the newborn with suspected seizures and interictal encephalopathies, as well as the prediction of neurologic sequelae. ( J Child Neurol 1998;13:467-474).
An Epidemiologic, Clinical, and Therapeutic Study of Childhood Guillain-Barré Syndrome in Kuwait: Is It Related to the Oral Polio Vaccine?
Authors: Ismail Essam A. R. , Shabani Issa S. , Badawi Mona , Sanaa Hala , Madi Samir , Al-Tawari Asmaa , Nadi Hani , Zaki Mohamed , Al-saleh Qusai
Published: December 2008

We studied Guillain-Barré syndrome, affecting children 12 years old or less, throughout Kuwait, in the period between January 1, 1992, and March 31, 1997. Nineteen children had the diagnostic criteria of Guillain-Barré syndrome, with an overall annual incidence rate of 0.95/100,000 population at risk. Female patients outnumbered male patients with a sex ratio of 1.4:1. There was a clustering of cases in winter and spring and in the year 1996. The disease symptoms were relatively severe in our patients because only 16% (3 of 19) of them were able to walk at the height of their illness, whereas the rest were bed or chair bound or needed assisted ventilation. Two patients had the electrodiagnostic features of axonal neuropathy and both had residual deficits on follow-up, whereas the rest recovered fully. All the patients received intravenous immunoglobulin. The mean time to walk unaided was 23.5 days (range, 2-84 days) after intravenous immunoglobulin and excluding the two patients with axonal neuropathy, and full recovery was achieved in a mean time of 103 days (range, 30-300 days). Contrary to previous studies, we found no correlation between oral polio vaccine administration and Guillain-Barré syndrome in 2 successive years (1995 and 1996) during a nationwide campaign targeting children less than 5 years old. (J Child Neut-ol 1998; 13:488-492).
Need for Electroencephalogram Video Confirmation of Atypical Absence Seizures in Children With Lennox-Gastaut Syndrome
Authors: Bare Mary A. , Glauser Tracy A. , Strawsburg Richard H.
Published: December 2008

Children with childhood epileptic encephalopathy (Lennox-Gastaut syndrome) frequently have both multiple seizure types and nonepileptic stereotyped events that are difficult to differentiate. We hypothesize that electroencephalogram (EEG) video monitoring is essential for correct identification of atypical absence seizures in this population. All video/EEG monitoring records on patients with confirmed Lennox-Gastaut syndrome between September 1992 and December 1996 were reviewed for clinical events and EEG changes. A subset of patients with suspected atypical absence seizures during the video/EEG formed the cohort for analysis. Thirty-eight patients had 48 monitoring periods ranging from 1 to 4 days (mean, 2.2 days). Twenty-six monitoring periods captured suspected atypical absence seizures and formed the study cohort. Suspected atypical absence seizures were epileptic seizures in only 27% (7 of 26) of the study cohort. By contrast, parents reliably and correctly identified tonic, atonic, and tonic-clonic seizures in the study cohort. Reliable diagnosis and subsequent counting of atypical absence seizures in patients with Lennox-Gastaut syndrome cannot be made on the basis of observation and/or history alone. Future outpatient studies of investigational anticonvulsant medications for patients with Lennox-Gastaut syndrome should consider parental counts of atypical absence seizures unreliable. We recommend that video/EEG monitoring be done on all Lennox-Gastaut syndrome patients with suspected atypical absence seizures not controlled by medication. ( J Child Neurol 1998;13:498-500).
Melatonin's Role as an Anticonvulsant and Neuronal Protector: Experimental and Clinical Evidence
Authors: Muñoz-Hoyos Antonio , Sánchez-Forte Miguel , Molina-Carballo Antonio , Escames Germaine , Martin-Medina Encarnación , Reiter Russel J. , Molina-Font Juan A. , Acuña-Castroviejo Darío
Published: December 2008

The pineal gland classically has been considered as a vestigial and mystic organ. In the last decades, and with the incorporation of new methodologic procedures, it could be proved that it also has physiologic actions that vary depending on the level of the phylogenetic scale. Its best-known secretion, melatonin, has been related to many different actions, such as sleep promotion, control of biologic rhythms, hormonal inhibition, and an inhibiting action on central nervous system regulation mechanisms. In animal experimentation, there are papers even accepting an anticonvulsant effect. In humans, evidence is reduced to few experiences. In addition to this clinical experience, there is other evidence that clearly relates melatonin to convulsive phenomena. This relationship must be mediated by the following mechanisms attributed to melatonin: altered brain GABAergic neurotransmission, its known interaction with benzodiazepinic brain receptors, through tryptophan metabolite activity (kynurenine, kynurenic acid), or even by its efficacy as a free-radical scavenger. ( J Child Neurol 1998;13:501-509).
Fosphenytoin in Infants
Authors: Takeoka Masanori , Krishnamoorthy Kalpathy S. , Soman Teesta B. , Caviness Verne S.
Published: December 2008

In comparison with phenytoin preparations, which have a pH value of 11, fosphenytoin, a phosphorylated prodrug of phenytoin, has a pH value of only 8.6, which decreases the risk of cardiovascular and cutaneous side effects. The near-neutral pH value of fosphenytoin allows effective intravenous or intramuscular administration. A 1-mg phenytoin equivalent (PE) of fosphenytoin is converted to 1 mg of phenytoin in adults. We describe four infants whose seizures were treated with intravenous fosphenytoin. We had difficulty maintaining therapeutic serum phenytoin levels of 10 to 20 μg/mL on doses of 5 to 8 mgPE/kg/day, and many bolus doses of 5 to 10 mgPE/kg or maintenance doses of more than 10 mgPE/kg/day were given. Despite increased doses in three out of the four patients, a therapeutic serum phenytoin level was not maintained. From our experience, careful and individual dosing of fosphenytoin in this age group can be considered. (J Child Neurol 1998;13:537-540).
β-Interferon Plus Inosiplex in the Treatment of Subacute Sclerosing Panencephalitis
Authors: Anlar Banu , Yalaz Kalbiye , Köse Gülsen , Saygi Serap
Published: December 2008

We treated seven patients with subacute sclerosing panencephalitis with β-interferon and oral inosiplex for 2 to 15 months. Stabilization or improvement was observed in three patients. The effect of treatment was equivocal in two other patients who became stable. The disease continued its progression in the remaining two patients who died. Treatment shorter than 2 months was not effective. Changes in electroencephalograms (EEG), magnetic resonance images (MRI), or cerebrospinal fluid measles antibody levels did not have a close correlation with clinical course. These results suggest that β-interferon might be efficient in some patients with subacute sclerosing panencephalitis and justify its trial in larger studies with longer follow-up. ( J Child Neurol 1998; 13:557-559).
Chronic Meningoencephalitis due to Cytomegalovirus Infection in a Nonimmunocompromised Patient: An Autopsy Case
Authors: Hirabayashi Shin-ichi , Shiobara Junko , Iwasaki Yuji , Ogiso Yoshifumi , Kubota Noriko , Oka Akira , Takashima Sachio
Published: December 2008

Congenital cytomegalovirus infection, which is the most common of the intrauterine viral infections, is well known to involve various organs, including the central nervous system. On the other hand, encephalitis due to persistent or acquired infection by cytomegalovirus in the central nervous system is thought to be rare, and to be seen exclusively in immunocompromised patients such as those under immunosuppressant therapy or those with acquired immunodeficiency syndrome (AIDS). We describe chronic meningoencephalitis possibly due to cytomegalovirus seen in an immunocompetent patient with behavior problems.
Vein of Galen Malformation with Diencephalic Syndrome: A Clinical Pathologic Report
Authors: Squires Liza A. , Thomas Sutha , Betz Bradford W. , Cottingham Sandra
Published: December 2008

A vein of Galen malformation is a dural extracranial vascular anomaly resulting in direct arteriovenous fistulae between the choroid or quadrigeminal arteries and an overlying single venous sac. The disorder can be identified on prenatal ultrasound examination and presents with macrocrania, with or without congestive heart failure, or cranial bruit. Therapeutic measures have included surgical clipping of feeding arteries and transarterial placement of wire, coils, or "bird cages. "2 Recently, newer endovascular techniques involving both a transarterial and transvenous approach have been promising. Prognosis for infants with the disorder remains poor, although improved with new endovascular techniques. Morbidity and mortality have improved to approximately 40% with mild to severe neurologic deficits.3 One cause of significant pre- and posttreatment morbidity might be the presence of vascular steal leading to visual deterioration, seizures, and signs of parenchymal loss, at times with striking progression. 4 The diencephalic syndrome of emaciation or failure to gain weight in an infant with extreme irritability or euphoria has not been reported previously with the vein of Galen malformation and is usually associated with a hypothalamic or chiasmatic glioma.
Tuberous Sclerosis Complex Consensus Conference: Revised Clinical Diagnostic Criteria
Authors: Roach E.S. , Gomez Manuel R. , Northrup Hope
Published: December 2008

At the recent tuberous sclerosis complex consensus conference, the clinical diagnostic criteria for tuberous sclerosis complex were simplified and revised to reflect both new clinical information about tuberous sclerosis complex and an improved understanding of the disorder derived from molecular genetic studies. Based on this new information, some clinical signs once regarded as pathognomonic for tuberous sclerosis complex are now known to be less specific. No single sign is present in all affected patients, and there is no proof that any single clinical or radiographic sign is absolutely specific for tuberous sclerosis complex. Accordingly, the clinical and radiographic features of tuberous sclerosis complex have now been divided into major and minor categories based on the apparent degree of specificity for tuberous sclerosis complex of each feature. A definitive diagnosis of tuberous sclerosis complex now requires two or more distinct types of lesions, rather than multiple lesions of the same type in the same organ system. Although diagnosis on purely clinical grounds can continue to be difficult in a few patients, there should be little doubt about the diagnosis for those individuals who fulfill these strict criteria. Couples with more than one child with tuberous sclerosis complex, no extended family history, and no clinical features of tuberous sclerosis complex are more likely to have germline mosaicism for tuberous sclerosis than nonexpression of the mutation. Germline mosaicism, while fortunately rare, will not be suspected from either diagnostic criteria or molecular testing until a couple has multiple affected children. Genetic counseling for families with one affected child should include a small (1% to 2%) possibility of recurrence, even for parents who have no evidence of tuberous sclerosis complex after a thorough diagnostic evaluation. ( J Child Neurol 1998;13:624-628).
A Preliminary Validation of Strategies that Support the Transition from School to Adult Life
Authors: Hughes Carolyn , Hwang Bogseon , Kim Jin-Ho , Killian Daniel J. , Harmer Melinda L. , Alcantara Paulo R.
Published: December 2008

The purpose of this study was to extend the empirical literature by identifying and socially validating strategies that support the transition from school to adult life. A three-step process was conducted: (a) reviewing 113 empirical articles that addressed secondary transition, (b) developing a candidate list of all support strategies that were empirically supported in the literature, and (c) conducting a national survey of 54 researchers who had published studies in the area of secondary transition (response rate = 92%) to establish the social validity of the proposed list of support strategies. Results of the survey corroborated multiple strategies that support students in the transition to adult life (e.g., family and peer support, student choice and preference, student self-determination). Findings are compared to those derived from the literature review and implications for research and practice are discussed.