bluuebunny/crossref_metadata_2025 huggingface dataset:
Differential Use of Orthographic and Phonological Coding Strategies by Students with Symmetrical and Asymmetrical Reading and Spelling Skills
Authors: Harrison Gina
Published: March 2008

This study investigated the orthographic and phonological coding strategies of students with symmetrical and asymmetrical reading and spelling achievement. Based on performance on a standardized achievement test, fourth-and fifth-grade students (N = 50) were classified as having no difficulties with reading and spelling, difficulties with both reading and spelling, or difficulties with spelling but not with reading. Students were administered the following tasks to assess their use of orthographic and phonological strategies: 1) rhyme judgment, 2) orthographic and phonological similarity in memory, 3) reading pronounceable pseudowords, and 4) deciding which pseudoword looked most like a real word. Compared to the poor readers and spellers, the results indicated a greater use of phonological coding strategies by the good readers regardless of spelling skill. Additionally, students who were poor readers and spellers demonstrated greater use of orthographic than phonological coding strategies. However, students with asymmetrical good reading but poor spelling skills recalled fewer orthographically distinct words than the good or poor readers and spellers, thereby, showing some differential use of an orthographic coding strategy. The results highlight the importance of considering students' orthographic in addition to phonological processing strategies when assessing and identifying specific learning disabilities. The results also provide evidence for the differential use of these strategies by students with either symmetrically poor or asymmetrically good reading but poor spelling skills.
An Evaluation of a Bullying Prevention Program for Elementary Schools
Authors: Beran Tanya N. , Tutty Leslie , Steinrath Greg
Published: March 2008

This research evaluates an anti-bullying program, Dare to Care: Bully Proofing Your School. Students in Grades 4-6 (N = 197, 77 boys, 120 girls) from four Calgary elementary schools completed the Colorado School Climate Survey (Garrity, Jens, Porter, Sager, & Short-Camilli, 2000) and the Provictim Scale - Short Version (Rigby & Slee, 1991). The first research component was a pretest - 3-month posttest comparison between a school that was about to implement the program and one that was not planning to do so. At pretest, students in the no-program school gave more positive reports on many variables than the school about to implement the program, making the intended across-school statistical comparison unfeasible. Within the 3-month program school, however, the frequency of bullying witnessed by the students significantly decreased, but remained stable in the no-program comparison school. Further, attitudes towards victims significantly worsened in the no-program school, but remained stable in the program school. The second research component compared three schools that had implemented the anti-bullying program for various lengths of time. Students in the schools with the longer program duration reported more positive attitudes towards victims than students in the school with shorter program duration; however, other indicators of program effectiveness were not significantly different. The results raise questions about evaluating anti-bullying programs.
School Psychology: How Universal are Ethical Principles Approved by International Associations?
Authors: Pettifor Jean L.
Published: March 2008

Globalization is a dominant issue in all aspects of business and professional activities in the 21 st Century. The International School Psychology Association and the International Test Commission have adopted ethics and competency guidelines to raise the standards of practice for their members. Other international organizations are doing likewise. Challenges exist in accommodating values related to the individualism, democracy, secularism, science, and wealth of western societies with the collectivism, authoritarianism, theocracy, folklore, and scarcity of resources of many developing countries. The first approach may be to acknowledge values based on common humanity rather than to define culturally specific behaviours. Vignettes are provided against which school psychologists may evaluate the utility of current ethical guidelines.
Educating the ADHD Child: Debunking the Myths
Authors: Schwean V.L. , Parkinson M. , Francis G. , Lee F.
Published: March 2008

Recent statistics show that children exhibiting Attention Deficit Hyperactivity Disorder (ADHD) represent a significant number of school-based referrals for psychoeducational assessment and intervention. Despite the pervasiveness and salience of this disorder, educators continue to labor under a number of misconceptions about the nature of ADHD and what constitutes appropriate school-based assessment and intervention practice. In this article, common myths with regard to conceptual issues and assessment and intervention strategies are generated and responded to with practical research-based information.
Correlations Between Objective Tests of Attention and Third Factor in WISC-R
Authors: McLarty Miclielle L. , Das J.P.
Published: March 2008

The relationship between the Third Factor of the Wechsler Intelligence Scale for Children-Revised (WISC-R) and selective attention subtests from the Das-Naglieri Cognitive Assessment System (CAS) were examined. Thirty-three children, ages 8 to 10, from resource classes participated in the study. Results of correlations between the Third Factor and CAS were as follows: With expressive attention tasks, r=-41, p <.01, with the number of correct responses in both visual and auditory selective attention tasks r=.45, p <.01. As expected, positive correlations were obtained between similar CAS subtests. Specific feedback provided by CAS regarding children's attention was regarded by both parents and teachers to be helpful. These findings have important implications relating to the diagnostic value of objective tests of attention.
WISC-III Third Factor: Critical Measurement Issues
Authors: Lowman Mary G. , Schwanz Kerry A. , Kamphaus Randy W.
Published: March 2008

Content validity of the Third Factor of the Wechsler Intelligence Scale for Childre-n-Third Edition (WISC-III) was evaluated by asking undergraduates to label the factor represented by items like those on the Arithmetic and Digit Span subtests. Participants unfamiliar with the content of the two subtests choose labels that corresponded with terminology associated with problem solving, memory, and quantitative skills. The concurrent validity of the Third Factor with behavioral measures of attention, hyperactivity, and learning problems was assessed in a sample comprising consecutive referrals to a university-based psychoeducational clinic. There was a moderate relationship between Freedom From Distractibility (FFD) and teacher ratings of learning problems on the Behavior Assessment System for Children-Teacher Rating Scales. There were no significant correlations to suggest concurrent validity between the Third Factor and behavioral ratings of attention problems and hyperactivity. Theoretical and practical implications of the FFD factor are discussed.
Diagnosis Based on the WISC-III Processing Speed Factor
Authors: Tiholov Tihol T. , Zawallich Andre , Janzen Henry L.
Published: March 2008

The primary intent of this research was to determine whether the Processing Speed Factor scores of the WISC-III would distinguish between groups of clinically referred children. A total of 311 elementary, junior high, and senior high students were grouped according to ADHD, VMI, behavioral, low academic achievement categories, and physical health problems or absence of such problems. Multiple comparisons using the Newman-Keuls and multiple regression techniques indicated that processing speed was a significant diagnostic factor differing between clinical groups. Anderson's (1992) model of the cognitive architecture of the mind was used to explain the specfic findings.
The Wechsler Adult Intelligence Scale—Third Edition: A Canadian Standardization Study
Authors: Hildebrand Denise , Saklofske Donald H.
Published: March 2008

The Wechsler Adult Intelligence Scale (WAIS) is presently undergoing revision in the United States. As Caruzdian children perfornwd differently than American children on the recent reuision of the WISC-III, a Canadian standardization study is currently being undertaken to determine whether Carsadian adults perform differently than American adults. Canadian norms will be developed for the WAIS-III if differenres are found.
School-Based Intergenerational Programs and the School Psychologist
Authors: Mester Fiore , Kokai Maria
Published: March 2008

The authors examine the types and benefits of school-based intergenerational programs (the interaction of senior volunteers and children) specifically from the school psychologist's perspective, and explore the possible roles psychologists may play in these programs.
Differential Validity of the BASC Student Observation System and the BASC Teacher Rating Scale
Authors: Lett Nancy J. , Kamphaus Randy W.
Published: March 2008

This study evaluated the differerrtial validity of the BASC Teacher Rating Scale (TRS) and the BASC Student Observation System (SOS) in the differentintion of nondisabled children from children with an ADHD diagnosis, and children with only an ADHD dingrrosis from children with ADHD and comorbid dingnoses. The results indicated that the TRS and SOS exhibited good differential validity. The group differences that were found were consistent with the definition of ADHD, and a good classification rate was found for the TRS and SOS (73%). The Predictive Discriminant Analysis also indicated, however, that the SOS does not contribute a sufficient amount of information above and beyond that obtained from the TRS alone.
Applying a Narrative Frame to the Assessment of Children
Authors: Korn Carol
Published: March 2008

Psychological assessment has been sharply criticized for its role in sorting children, especially nunority children (Jitendra & Rohena-Diaz, 1996; Malgady, 1996), and for representing the views of the dominant school structure (Milofsky, 1989). This article calls for reconceptualization of psychological assessment of children as narrative, representing both the process and product of constructing the story of a child's growth. Psychological assessment and report writing are reframed here as hermeneutic activities that seek to understand the meanings of children's behavior in the social and cultural contexts in which children grow. Narrative is proposed as an alternate framework to that provided by the diagnostic/prescriptive model, grounding psychological assessment in a humanistic rather than positivistic tradition of identification and remediation of pathology. The psychological report is explored as a particular kind of writing that shares with ofher narrative genres an emphnsis On developing a plausible life story by creating a coherent whole out of disparate events. Special consideration is given to the role responsibility of psychologists as narrators of children's life storirs. Finally, the implications of a narrative framework on the practice of psychological assessment of children, and on the training of psychologists who work with children are raised.
Treatment Effect of a School Based Cognitive-Behavioral Program for Aggressive Children
Authors: Phillips Dale R. , Schwean Vicki L. , Saklofske Donald H.
Published: March 2008

The Day Treatment Program was designed as a teacher operated, school-based cognitive-behavioral program. Initiated to address the needs of behaviorally disordered children, it has a strong parenting component. This study found that the program had a significant treatment effect. Aggressive popular children did not respond to the program as well as aggressive unpopular children.
The Underlying Structures of Autism: An Exploratory Factor Analytic Study
Authors: Soucy Michelle , Andrews Jac
Published: March 2008

Data from several psychometric variables was collected for 24 autistic children from a multidisciplinary center in Calgary, Alberta. The purpose of this study was to explore the underlying nature of autism. Variables were factor and duster analyzed to discover underlying structures of the disorder and to empirically derive homogeneous subtypes of children with a diagnosis of autism. Factor analysis indicated a 4 factor solution to account for the variance among variables. The groups were subsequently labeled Social Cognition, Language, Deviant Behavior, and Developmental. The clustering solution indicated the presence of two distinct groups labeled Low Functioning and High Functioning. Validity checks confirmed significance between group differences on measures of overall development, socinl functioning, and behaviors. The potential utility of empirical research is discussed along with limitations of the present study and suggestions for future research.
Consultation in Canada
Authors: Sladeczek Ingrid E. , Heath Nancy Lee
Published: March 2008

In the United States there has been an increasing focus on consultation as an indirect service delivery model that is cost effective and preventive. Despite the substantive nature of the consultation field in the United States, the status of consultation in Canada remains less clear. In reviewing the Canadian literature on consultation, it became apparent that the conceptualization of consultation is varied. In order to obtain clarification, a systematic evaluation of the consultation literature in Canada was initiated. The present review describes consultation in Canada from its origin in 1960 to the present. Canadian journals relevant to the education field were examined and articles extracted that dealt with school consultation. This resulted in a sample of 24 articles drawn from 12 Canadianjournals. Chronological trends and themes are noted and it is concluded that school consultation in Canada differs from that in the United States, both quantitatively and qualitatively. Although less empirical research has been conducted on school consultation in Canada than in the United States, the Canadian literature on school consultation is characterized by a multitude of types of consultation.
An Empirically Derived Basis for Using Geometric-Form Copy Tasks to Identify Kindergarten Children at Risk for Early School Failure
Authors: Simner Marvin L.
Published: March 2008

An item analysis was performed on the geometric-form copy tasks used to identify kindergarten children at risk for early school failure. The outcome of this analysis revealed that only eight of the 15 forms found on these tasks and only certain of the scoring criteria applied to these forms were effective in identifying children with potential learning problems. The findings further revealed an important limitation in the use of these eight forms. The implications of this limitation are discussed in terms of a possible unique role that these forms might play in the development of an effective early identification program.
Vacuolating Megalencephalic Leukoencephalopathy in 12 Israeli Patients
Authors: Ben-Zeev Bruria , Gross Varda , Kushnir Tamar , Shalev Ruth , Hoffman Chen , Shinar Yael , Pras Eilo , Brand Nathan
Published: March 2008

Leukodystrophy with macrocephaly as the main features of infantile neurodegenerative disease are characteristics of Canavan's disease, L-2-hydroxyglutaric aciduria, type I glutaric aciduria, and Alexander's disease. Also occasionally described are occidental congenital muscular dystrophy, GM2-gangliosidosis, metachromatic leukodystrophy, Krabbe's disease, and mucopolysaccharidosis. Since 1995, over 60 patients with a new syndrome, vacuolating megalencephalic leukoencephalopathy, have been described. The syndrome is characterized by macrocephaly, a slowly progressive clinical course of ataxia, spastic paraparesis, and seizure disorder with relatively spared cognition. Unlike other leukodystrophies with macrocephaly (except Alexander's disease), no metabolic marker has been found. We describe a similar group of 12 patients from two different Jewish ethnic origins in whom consanguinity is prominent. These patients have neuroimaging features and magnetic resonance spectroscopy findings indicating that there is an initial increase in white-matter edema with subsequent cystic formation. Consistent with loss of tissue in these areas, brain metabolites are reduced. The familial incidence in this group of patients is suggestive of autosomal-recessive inheritance. (J Child Neurol 2001;16:93-99).
Encephalopathy Associated With Respiratory Syncytial Virus Bronchiolitis
Authors: Ng Yu-tze , Cox Christopher , Atkins Jane , Butler Ian J.
Published: March 2008

Respiratory syncytial virus is an extremely common cause of childhood respiratory infections resulting in significant morbidity and mortality. Although apnea is a well-known complication in young infants with respiratory syncytial virus bronchiolitis, the encephalopathy associated with this infection is not well recognized. Our study reveals an incidence of encephalopathy of 1.8% in a total of 487 patients with respiratory syncytial virus bronchiolitis studied over a period of almost 4 years. Seizures were the presenting complication. Based on our study of a cohort of children with respiratory syncytial virus bronchiolitis, we believe that neurologic complications, although relatively uncommon, represent a significant component of this common childhood illness. Furthermore, respiratory syncytial virus has been shown to release several mediators that could directly or indirectly be neurotoxic and induce an encephalopathy associated with the respiratory illness. (J Child Neurol 2001;16:105-108).
Benign Myoclonus of Early Infancy: An Imitator of West's Syndrome
Authors: Maydell Bernhard V. , Berenson Frank , Rothner A. David , Wyllie Elaine , Kotagal Prakash
Published: March 2008

Benign myoclonus of early infancy is a rare condition characterized by nonepileptic spasms that may resemble the epileptic spasms seen in West's syndrome. The spells in benign myoclonus of early infancy begin before age 1 year and are self-limited. The electroencephalogram (EEG) is invariably normal, and neurologic development is not affected. West's syndrome is characterized by infantile spasms that appear before 1 year of age, an abnormal EEG with hypsarrhythmia, and a poor prognosis. We describe six infants who presented for evaluation of clusters of head, trunk or extremity spasms, eye blinking, brief jerking of upper extremities or trunk, and head nodding episodes. In most, a presumptive diagnosis of West's syndrome was made prior to the referral. One infant had been placed on valproate. Routine EEG recordings or prolonged video EEG monitoring were normal both during and between episodes. After the negative evaluations, the diagnosis of benign myoclonus of early infancy was made in each infant. Subsequently, no infant was treated with anticonvulsants. Follow-up revealed complete resolution of the episodes in all children within 2 weeks to 8 months of onset. All had normal neurologic development. Based on our cases and review of the literature, the prognosis for this disorder is excellent. Care should be taken to recognize this rare entity and avoid unnecessary and potentially harmful antiepileptic therapy. (J Child Neurol 2001;16:109-112).
Acute Cognitive Effects of Nonconvulsive Difficult-to-Detect Epileptic Seizures and Epileptiform Electroencephalographic Discharges
Authors: Aldenkamp Albert P. , Arends Johan , Overweg-Plandsoen Truus C.G. , van Bronswijk Karin C. , Schyns-Soeterboek Angelique , v.d. Linden Inge , Diepman Leonie
Published: March 2008

This study compares the acute cognitive effects of short nonconvulsive seizures with the effects of interictal epileptiform electroencephalographic (EEG) discharges in children. The study is a prospective, standardized, nonrandomized, and open clinical comparative study. Eligible patients were included when they had (a) unclear seizures and fluctuations in cognitive performance and (b) frequent epileptiform EEG discharges in a recent EEG. All children were assessed with EEG/video (Brainlab®) simultaneously with computerized neuropsychologic testing (FePsy®) assessing motor speed/alertness, mental speed/attention, and memory function. Eleven patients with short nonconvulsive seizures during cognitive testing were included and compared with 11 matched patients with interictal epileptiform EEG discharges during cognitive testing but without seizures. Patients included in both groups had a reconfirmed diagnosis of epilepsy. Cognitive performance for both groups was compared. Statistical analysis showed significant correlations beween the number of seizures (during cognitive testing) and impaired alertness and between the duration of the ictal period and memory impairment. Interictal epileptiform EEG discharges do not have an additional independent effect on cognitive function. The results demonstrate the accumulating cognitive effect of seizures and illustrate that frequent seizures, even when these are short in duration and with subtle symptomatology, can have a substantial impact on daily life and can lead to state-dependent learning impairment. Alertness and short-term memory appeared to be the functions that are most vulnerable for the acute effects of seizures. (J Child Neurol 2001;16:119-123).
Prolonged but Reversible Respiratory Failure in a Newborn With Prader-Willi Syndrome
Authors: MacDonald John T. , Camp Diane
Published: March 2008

A 29-week premature infant with severe central hypoventilation secondary to Prader-Willi syndrome required mechanical ventilation until 55 days of age. This disorder must be considered in the differential diagnosis of a premature infant who does not have primary lung disease but has significant hypotonia because the respiratory failure may require prolonged supportive care. (J Child Neurol 2001;16:153-154).
Pediatric Patients With Undetectable Anticonvulsant Blood Levels: Comparison With Compliant Patients
Authors: Snodgrass S. Robert , Vedanarayanan V.V. , Parker Colette C. , Parks Bruce R.
Published: March 2008

Undetectable anticonvulsant blood levels indicate sustained noncompliance (several consecutive doses missed). We compared 91 consecutive outpatients with epilepsy and undetectable anticonvulsant blood levels to 100 patients seen during the same time period, verified as compliant by acceptable serum levels. We hypothesized that pay status, application for Supplemental Security Income, patient age, history of missed appointments, and functional status would differ between compliant and noncompliant patients. We were surprised to find large differences between clinic and insurance patients and between Caucasian and non-Caucasian patients. The 100 compliant patients included 44 Caucasian and 56 non-Caucasian patients, whereas only 9 of 91 noncompliant patients were Caucasian, and only 9 had insurance, compared to 32 compliant patients. Applications for Supplemental Security Income and history of missed appointments were significantly associated with noncompliance, but patient age, seizure type, and seizure control were not. Uninsured Caucasians were more often compliant than non-Caucasians were. Many noncompliant patients had mild epilepsy, which was reportedly doing well. Race and pay status were closely correlated. Several noncompliant females became pregnant, whereas no compliant patients did. Compliant patients were much more likely to be accompanied by a parent or caretaker on clinic visits than noncompliant patients. Noncompliant patients had at least one acceptable subsequent serum level, although 2 patients with intractable epilepsy had undetectable serum levels on three or more occasions. Noncompliance may respond to discussion and advice. We reviewed 124 episodes of undetectable drug levels in the 91 noncompliant patients. Eighteen of these resulted in hospitalization, but in 25 cases, we were told that there had been no seizures since the preceding visit. Many noncompliant patients have infrequent seizures, even if they take little or no medication. Socioeconomic status influences health, life expectancy, and educational success, but it has been claimed to be irrelevant to compliance and adherence issues in epilepsy. Our data and the experience of other centers with childhood diabetes suggest that socioeconomic, racial, and family factors influence compliance or adherence to treatment for many chronic conditions. Educational efforts and support for parents at the start of anticonvulsant treatment may improve compliance. Uninsured patients missed more appointments and were much more likely to be noncompliant than insured patients. Attention to the special problems of Medicaid and minority children is needed. ( J Child Neurol 2001;16:164-168).
Progressive Intracranial Vascular Disease with Strokes and Seizures in a Boy with Progeria
Authors: Rosman N. Paul , Anselm Irina , Bhadelia Rafeeque A.
Published: March 2008

Progeria, a rare genetic disorder, is characterized by severe growth failure, premature aging, and very early atherosclerosis with coronary artery and cerebrovascular disease. There has been no detailed description of progressive cerebrovascular changes in progeria or any attempted neurologic correlation of those changes. A 5-year-old boy developed signs of progeria at 4 months and hypertension at 4 years, treated with atenolol and dipyridamole. Left-sided seizures with a left hemiparesis occurred at 5 years. Magnetic resonance imaging (MRI) showed bilateral acute, subacute, and chronic cerebral infarctions. Magnetic resonance angiography disclosed severe stenosis of the left internal carotid artery. The child was also found to have an aortic valve vegetation and was anticoagulated. He subsequently developed right-sided seizures, and treatment with gabapentin was started. Later, severe stenosis also of the right internal carotid artery was found. MRI showed new left cerebral infarction. The child's neurologic symptoms almost certainly were caused by cerebral infarctions from progressive atherosclerosis of major intracranial vessels, but clinical-neuroradiologic correlations were imprecise. There were multiple cerebral infarctions of different ages, some asymptomatic, others ipsilateral to the child's neurologic findings. No therapy has halted progression of the child's cerebrovascular disease. ( J Child Neurol 2001; 16:212-215).
Hemihydranencephaly: Case Report and Literature Review
Authors: Greco Filippo , Finocchiaro Maria , Pavone Piero , Trifiletti Rosario R. , Parano Enrico
Published: March 2008

Hydranencephaly is a severe brain condition characterized by complete or almost complete absence of cerebral cortex with preservation of meninges, basal ganglia, pons, medulla, cerebellum, and falx. It has been ascribed to different causes (infections, irradiations, fetal anoxia, medications, twin-twin transfusion), all leading to vascular disruption. Hemihydranencephaly is an extremely rare condition in which the vascular anomaly is unilateral. We report on a patient who was suspected to have hydrocephalus in utero; a brain magnetic resonance imaging scan showed left-sided hydranencephaly with preservation of basal ganglia. The patient developed signs of right hemiparesis but notably has only mild language delay. The available literature on hemihydranencephaly is reviewed. ( J Child Neurol 2001;16:218-221).
Early Cognitive and Communication Development in Children With Focal Brain Lesions
Authors: Chilosi Anna M. , Cipriani Paola , Bertuccelli Barbara , Pfanner Lucia , Cioni Giovanni
Published: March 2008

Early cognitive and language development of children with congenital focal brain lesions, documented by magnetic resonance imaging, was studied in 18 cases, 9 with left-hemisphere damage and 9 with right-hemisphere damage, at about 2 (Time 1) and 4 years of age (Time 2). All of the children showed normal cognitive development, but their global Griffiths Developmental Scales scores were lower at Time 2, and developmental profiles across individual subscales revealed side-specific effects, resembling the adult left/right cerebral hemisphere lesion model. Expressive lexicon and grammar were delayed, more often in left-hemisphere-damaged than in right-hemisphere-damaged children, at Time 1 and Time 2. Functional findings were not related to the size and location of the brain lesion, whereas the presence of epilepsy was a highly significant predictor of cognitive and language outcome, irrespective of the side of the lesion. The stable disadvantage in the verbal domain shown by left-hemisphere-damaged children within the age range of this study might suggest that the left hemisphere has some initial bias for language learning. The effects of right-hemisphere damage were more variable and emerged at a later stage of language development. (J Child Neurol 2001;16:309-316).
Definite and Suspected Multiple Sclerosis in Children: Long-Term Follow-up and Magnetic Resonance Imaging Findings
Authors: Belopitova Liliana , Guergueltcheva V. , Bojinova V.
Published: March 2008

Twenty-five children at the ages of 3 to 18 years with an initial diagnosis of acute disseminated encephalomyelitis were followed in the Clinic of Child Neurology for a period of 2 to 8 years. In 10 children, there were data for clinically definite or laboratory-supported definite multiple sclerosis. The other 15 children in our study were considered as having suspected multiple sclerosis. Brain magnetic resonance imaging (MRI) performed in 15 children disclosed multiple hyperintense lesions on T2-weighted imaging in 13 children: 10 with definite multiple sclerosis and 3 with suspected multiple sclerosis. The clinical manifestations did not always correspond to the size and location of the MRI lesions of demyelination. Follow-up revealed normalization of the neurologic examination in 18 patients (72%) and abnormal neurologic findings in 7 patients (28%) (6 children with definite multiple sclerosis and 1 with suspected multiple sclerosis). Magnetic resonance imaging follow-up in children with definite multiple sclerosis disclosed a reduction in the size of the lesions in 3; enlargement or new lesions were established in the other 7 cases, and 2 cases were without clinical signs of new attacks. Correlation was done concerning the findings of the cerebrospinal fluid examination, transcranial magnetic stimulation, evoked potentials, computed tomography, and MRI. The role of MRI for an early diagnosis of multiple sclerosis in children is discussed. The dynamic follow-up of the pathologic changes is of prognostic significance for the course of the disease that could be a definite cessation of the process in acute disseminated encephalomyelitis cases or transition to multiple sclerosis. (J Child Neurol 2001;16:317-324).
Brain Activity and Cognitive Status in Pediatric Patients: Development of a Clinical Assessment Protocol
Authors: Byrne Joseph M. , Connolly John F. , MacLean Shannon E. , Beattie Tricia L. , Dooley Joseph M. , Gordon Kevin E.
Published: March 2008

The purpose of this study was to test the validity of a new computerized task to assess children's cognitive problem-solving skills using the brain event-related potentials. This event-related potential-computerized cognitive problem-solving task does not require a child to give a verbal or motor (ie, pointing) response. The event-related potential waveforms were recorded from 20 typically developing children. Two nonverbal, problem-solving tasks (tasks 1 and 2) were developed for each of two age groups (5 and 6 years). For each task, single pictures, taken from an existing standardized test of nonverbal problem solving, were individually and sequentially presented on a computer screen. One of the seven pictures was classified as incongruent or outside category; it did not belong with the other pictures. As predicted, the event-related potential amplitudes were significantly larger to the outside- versus within-category pictures. This effect was found for tasks 1 and 2 for the 5- and 6-year-old children. Children as young as 5 years of age reliably exhibit brain activity, which can be used to infer cognitive problem-solving skill. This assessment paradigm may eventually serve as a clinically useful adjunct to a thorough neurologic and neurodevelopmental assessment of selected pediatric populations, such as those presenting with moderate-severe cerebral palsy whose expressive language and motor skills are notably impaired. (J Child Neurol 2001;16:325-332).
Ictal Single Photon Emission Computed Tomography in Absence Seizures: Apparent Implication of Different Neuronal Mechanisms
Authors: Iannetti Paola , Spalice Alberto , De Luca Francesca , Boemi Sergio , Festa Anna , Maini Carlo Ludovico
Published: March 2008

Absence seizures represent a complex group of epilepsy, characterized by lapse of consciousness with staring. Bilateral, synchronous, and symmetric bursts of 3-Hz spike-and-wave discharges are observed on the electroencephalogram, whereas interictal background activity is normal. This kind of epilepsy has to be differentiated from other generalized epilepsies such as juvenile absence epilepsy and juvenile myoclonic epilepsy. Moreover, absence seizures, together with generalized spike-and-wave discharges, may coexist with other types of epilepsy such as frontal lobe epilepsy, temporal lobe epilepsy, benign epilepsy with centrotemporal spikes, and childhood epilepsy with occipital paroxysms. We have carried out ictal single photon emission computed tomography (SPECT) in 10 patients with clinical evidence of absence seizures with the aim to better understand and to distinguish this kind of seizure as primarily or secondarily generalized to a specific area and to obtain more information on the neuronal mechanisms involved in the different types of seizures, usually not identifiable at the first appearance. During the long follow-up period (9 months to 14 years), 7 of the 10 examined patients underwent interictal SPECT when they became seizure free. Our data permitted, in two patients, the diagnosis of childhood absence seizures; in three patients, they suggested the possibility of later appearance of other seizure types, on the basis of focal hyperperfusion indicating a possible focal firing. In three of the examined patients, the diagnosis of idiopathic localization-related epilepsies mimicking childhood absence seizures could be performed. In the last two patients, the hypothesis of a coexistence of absences with partial and generalized seizures was considered. From our results, it can be presumed that ictal SPECT findings may contribute to the physiopathologic classification of the different types of epilepsies. Moreover, anticonvulsant treatment more appropriate to the different forms of seizures can be used. (J Child Neurol 2001;16:339-344).
Effect of Antiepileptic Drugs on Plasma Lipoprotein (a) and Other Lipid Levels in Childhood
Authors: Müjgan Aynaci F. , Orhan Fazil , Orem Asim , Yildirmis Sermet , Gedik Yusuf
Published: March 2008

Antiepileptic drugs may alter plasma lipid status in epileptic patients. We conducted a study to assess the effect of phenobarbital, carbamazepine, and valproate on plasma levels of lipoprotein (a), total cholesterol, triglycerides, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, apolipoprotein A, and apolipoprotein B in 22 epileptic children. The children were separated as group 1, seven children, mean age 1.6 ± 0.2 years, treated with phenobarbital, 5 mg/kg/day, twice daily; group 2, seven children, mean age 9.8 ± 1.2 years, treated with carbamazepine, 20 mg/kg/day, twice daily; and group 3, eight children, mean age 6.8 ± 0.6 years, treated with valproate, 20 mg/kg/day, twice daily. Plasma lipoprotein (a) and other lipid levels were studied before (pretreatment) and at 3 and 6 months of treatment. Friedman two-way analysis of variance and Wilcoxon's signed-rank test were used for statistical analysis, and the results were expressed as the mean and standard error of the mean. The mean age of children in group 1 was significantly low, compared with groups 2 and, 3 (P < .001). The mean pretreatment lipid levels between the groups were not significant. The increase in lipoprotein (a) at 3 and 6 months and high-density lipoprotein cholesterol at 6 months was statistically significant in group 1 (P <.025). We suggest a careful monitoring of plasma levels of lipoprotein (a) and other lipids in epileptic children treated with antiepileptic drugs. (J Child Neurol 2001;16:367-369).
Rasmussen Encephalitis Associated With Segmental Vitiligo of the Scalp: Clinicopathologic Report
Authors: Yacubian Elza Márcia T. , Rosemberg Sérgio , Garrido Neto Theodomiro L. , Marie Suely K.N. , Valério Rosa M.F , Jorge Carmen L.
Published: March 2008

A 3-year-old boy with segmental vitiligo, poliosis, and alopecia over the right side of the scalp developed epilepsia partialis continua involving the left side of the body and progressive atrophy of the right cerebral hemisphere. There was a right ear dysacusia and a perilimbal vitiligo associated with an area of iris depigmentation in the right eye. Pleocytosis and hypergammaglobulin were detected in cerebrospinal fluid. Because medical treatment that included phenobarbital, phenytoin, carbamazepine, oxcarbazepine, benzodiazepines, corticosteroids, γ-globulin, and a ketogenic diet was ineffective, he underwent a right hemispherectomy. Neuropathologic examination showed a widespread scattered inflammatory process with numerous microglial nodules and perivascular lymphocytic cuffing associated with degenerative changes with severe neuronal loss, loosening of the neuropil, and microcystic changes with tissue collapse. The coexistence of vitiligo and possibly Vogt-Koyanagi-Harada syndrome in this child reinforces the autoimmune theory as the pathogenesis of Rasmussen syndrome. (J Child Neurol 2001;16:374-377).
Clonidine Treatment Increases Tics in Patients With Tourette Syndrome: Case Report
Authors: Kessler Abraham R.
Published: March 2008

Clonidine usually serves as a tic-suppressing agent in patients with Tourette syndrome. A case study is presented in which clonidine at subclinical concentrations led to an exacerbation of the tics and caused severe systemic heat sensations. Involvement of functional abnormalities of the hypothalamus in Tourette syndrome is hypothesized. (J Child Neurol 2001;16:380-381).
Risperidone Monotherapy in Preschool Children With Pervasive Developmental Disorders
Authors: Masi Gabriele , Cosenza Angela , Mucci Maria , De Vito Giuseppe
Published: March 2008

The aim of this preliminary study was to examine the short-term efficacy and safety of the atypical antipsychotic risperidone in preschool autistic children. The sample consisted of 10 subjects (7 males and 3 females) aged 39/12 to 66/12 years (mean age 4.7 years). A 16-week open-label trial with risperidone monotherapy was initiated at a starting dose of 0.25 mg daily and was increased to a maximum dose of 0.50 mg (0.027 mg/kg daily). Outcome measures were the Childhood Autism Rating Scale, the Children's Psychiatric Rating Scale, Clinical Global Impression (improvement score), and the Children's Global Assessment of Functioning. Two subjects did not complete the trial because of side effects (tachycardia and flushes, fever and hyporexia). After the 16-week treatment, data from the eight children who completed the trial indicated a modest improvement in the Childhood Autism Rating Scale total score, Children's Psychiatric Rating Scale total score, and Children's Global Assessment of Functioning. According to the Clinical Global Impression, the global improvement score for four subjects was much improved or very much improved; the score for the other four children was minimally improved. None of the children exhibited behavioral deterioration. The side effects in the eight children were not severe. (J Child Neurol 2001;16:395-400).
Neurologic Course of Congenital Disorders of Glycosylation
Authors: Pearl Phillip L. , Krasnewich Donna
Published: March 2008

Congenital disorders of glycosylation, formerly called carbohydrate-deficient glycoprotein syndrome, may present in infancy with slowly progressive neurologic deficits including cognitive impairment, ataxia, pigmentary retinal degeneration, and neuropathy. The metabolic defect is in N-linked oligosaccharide synthesis, and diagnosis is made by a serum transferrin isoelectric focusing. We reviewed the neurologic course of 10 children with congenital disorders of glycosylation (ages 13 months to 7 years). All had severe developmental delay and ataxia; none walked unassisted, and the highest level of communication was simple sign language in one patient. Five of 10 children had seizures (absence, complex partial, tonic clonic). Only one patient has had strokelike episodes, despite reports that they are common in this population. The underlying basis of these episodes has been hypothesized to be coagulopathy due to dysfunctional, incorrectly glycosylated coagulation factors. This 5-year-old patient with congenital disorders of glycosylation type Ia had two strokelike episodes, with evolving hemiparesis over 5 to 6 days' duration, followed by focal tonic-clonic seizures. Coagulation studies were normal. Electroencephalography showed transient hemispheric polymorphous delta-range slowing and suppression. Magnetic resonance imaging revealed corresponding cortical swelling. Magnetic resonance angiography was normal. Magnetic resonance spectroscopy revealed a decrease in the N-acetylaspartate peak, suggesting neuronal loss, with normal lactate peak. The neuroradiologic data do not support a thrombotic, embolic, or hemorrhagic basis for strokelike episodes in carbohydrate-deficient glycoprotein syndrome; other mechanisms must be considered. (J Child Neurol 2001;16:409-413).
Long-Term Follow-up of an Epidemiologically Defined Cohort of Patients With Tourette Syndrome
Authors: Burd Larry , Kerbeshian Jacob , Barth Amy , Klug Marilyn G. , Avery Karilyn , Benz Becky
Published: March 2008

The goal of this study was to collect prospective longitudinal information on the development of an epidemiologically defined cohort of patients with Tourette syndrome. These data may improve prognostic understanding of the condition. This information will also be important for specification of an adult phenotype for genetic marker studies. A prospective longitudinal cohort study was conducted. Fifty-four of 73 patients from our 1986 prevalence study of Tourette syndrome in North Dakota school-aged children were eligible for inclusion. The subjects were diagnosed in 1984 and 1985. We were able to interview 39 of 54 eligible patients for 507 person-years of follow-up. For the cohort, tic severity declined by 59%, global assessment of functioning improved by 50%, and the average number of comorbidities decreased by 42%. Forty-four percent of patients were essentially symptom free at follow-up. Only 22% were on medication as adults. Tourette syndrome is a developmental neuropsychiatric disorder with a long-term course that is favorable for most patients. Males demonstrated substantially more variability in improvement but overall demonstrated more improvement than females. (J Child Neurol 2001;16:431-437).
Evidence for Apoptosis in the Fetal Down Syndrome Brain
Authors: Seidl Rainer , Bidmon Bettina , Bajo Michael , Yoo Byon C. , Cairns Nigel , LaCasse Eric C. , Lubec Gert
Published: March 2008

In Down syndrome, enhanced apoptosis (programmed cell death) may play a role in the pathogenesis of characteristic early mental retardation and precocious neurodegeneration of Alzheimer type. Various apoptosis-associated proteins (Bax, Bcl-2, Fas, p53, Hsp70, neuronal apoptosis inhibitory protein-like immunoreactivity) were investigated in four different cortical regions and the cerebellum of one fetal Down syndrome (35 weeks' gestation) postmortem brain sample compared with a control brain sample. The most impressive finding was an at least fivefold elevation of Bax protein together with decreased Bcl-2 values in all Down syndrome cerebral regions investigated. In addition, antiapoptotic, presumably caspase-inhibitory, principles like heat shock protein 70 and neuronal apoptosis inhibitory protein were also reduced. Whereas Fas protein, an important member of receptor-mediated apoptosis, was inconsistently altered, a rather surprising finding was reduced proapoptotic, regulatory protein p53 in four of five regions. The findings are in good agreement with the proposed role of the Bcl-2 protein family in regulating developmental (naturally occurring) apoptotic neuronal death and further suggest that developmental apoptosis may be inappropriately commandeered by so far undefined pathologic processes in Down syndrome. (J Child Neurol 2001;16:438-442).
Internal Carotid Artery Thrombus: An Underdiagnosed Source of Brain Emboli in Neonates?
Authors: Alfonso Israel , Prieto Genoveva , Vasconcellos Elza , Aref Karim , Pacheco Esperanza , Yelin Karina
Published: March 2008

We report a full-term neonate with a left middle cerebral artery infarct, narrowing of the internal carotid artery detected by magnetic resonance angiography and B-mode ultrasonography, and a large thrombus at the origin of the internal carotid artery detected by B-mode ultrasonography. Internal carotid arterial thrombus is seldom considered the source of middle cerebral arterial embolus in neonates. We suggest that B-mode ultrasonography of the carotid artery be included in the diagnostic evaluation of middle cerebral artery infarcts in neonates. (J Child Neurol 2001;16:446-447).
Three New Patients With Congenital Unilateral Facial Nerve Palsy due to Chromosome 22q11 Deletion
Authors: Eirís Puñal Jesús , Siebert Manuel Fuster , Angueira Francisco Barros , Castro-Gago Manuel
Published: March 2008

We report three unrelated patients with congenital facial nerve palsy and chromosome 22q11 deletion, a condition hitherto poorly recognized. In the first case, facial palsy was associated with aortic coarctation, ductus arteriosus, and ostium secundum atrial septal defect. In the second case, facial palsy was associated with ostium secundum atrial septal defect, obstruction of the ureteropelvic junction, double ureteropelvic-calicial system, and distal metaphyseal widening of the forearm and leg bones. In both cases, facial palsy was the presenting feature. In the third case, an ostium secundum atrial septal defect was also present, but involvement of cranial nerves III, VI, and VIII, in addition to hypoplastic structures of cerebellar and cerebral peduncles, were the predominant features. There were no inherited deletions within chromosome band 22q11 and the de novo deletions detected in each case belonged to the paternally derived chromosome 22. Association of facial nerve palsy and congenital heart disease versus cardiofacial syndrome are different only on clinical grounds, so both conditions can be genetically identical and form part of the spectrum of defects associated with chromosome 22q11 1 deletions. We recommend investigation for chromosome 22q11 deletions in patients with complete nerve facial palsy. (J Child Neurol 2001;16:450-452).
Magnetic Resonance Imaging Findings in Patients With Severe Neonatal Indirect Hyperbilirubinemia
Authors: Yilmaz Yüksel , Alper Gülay , Kiliçoglu Gamze , Çelik Levent , Karadeniz Leyla , Yilmaz-Degirmenci Serpil
Published: March 2008

The aim of this study was to document the magnetic resonance imaging (MRI) findings of cases with a history of severe neonatal indirect hyperbilirubinemia. Ten cases (eight cases with neurologic findings, two normal cases) with a history of severe neonatal indirect hyperbilirubinemia were studied. Neurologic findings and MRI results were described and correlated. Seven of eight cases with neurologic findings demonstrated symmetric and uniform increased T2 signal changes limited to globus pallidi. MRI scans of two cases without neurologic findings showed no abnormality. Severe neonatal indirect hyperbilirubinemia should be considered in the differential diagnosis of bilateral symmetric hyperintense signal changes in the globus pallidus on MRI. However, high levels of unconjugated bilirubin concentrations in the neonatal period may not always cause such lesions of globus pallidus on MRI despite the presence of neurologic findings. (J Child Neurol 2001;16:452-455).