bluuebunny/crossref_metadata_2025 huggingface dataset:
Brain Organization of Motor and Language Functions Following Hemispherectomy: A [15O]-Water Positron Emission Tomography Study
Authors: Müller Ralph-Axel , Chugani Harry T. , Muzik Otto , Mangner Thomas J.
Published: December 2008

The capacity of the developing brain for compensatory reorganization after early hemispherectomy has been previously shown in neurobehavioral studies, above all with regard to language recovery. The present study examines the organization of motor and language areas by means of [15O]-water positron emission tomography (PET) in a 6-year-old boy who underwent right functional hemispherectomy at age 3 years. The results suggest that compensatory allocation for movement of the weak hand primarily involves the premotor, inferior frontal, and insular cortices, and the supplementary motor area in the retained hemisphere, as well as the bilateral cerebellum. Receptive language and prosodic functions primarily activated the left perisylvian cortices. However, language and motor activations were also seen in cortical and subcortical remains on the hemispherectomized side suggesting incomplete disconnection by functional hemispherectomy (J Child Neurol 1998; 13:16-22).
Refractory Status Epilepticus in Children: Role of Continuous Diazepam Infusion
Authors: Singhi Sunit , Banerjee Sanjay , Singhi Pratibha
Published: December 2008

To determine efficacy of continuous diazepam infusion in the treatment of refractory status epilepticus in a retrospective study, we analyzed data of 62 children admitted consecutively to our Pediatric Intensive Care Unit with a diagnosis of refractory status epilepticus. The unit followed a standard treatment protocol for diazepam infusion; if it failed, thiopental infusion was used. The mean age of patients was 2.80 years (range, 1.5 to 11.5 yr). Thirty-six patients (60%) had acute infections of the central nervous system and 10 (16%) had idiopathic epilepsy. Diazepam infusion was used in 57 patients. This treatment controlled seizures in 86% of patients (49/57), on average within 40 minutes (median, 30 min; range, 10-120 min), at a mean infusion rate of 0.017 mg/kg/min (range, 0.01-0.03 mg/kg/min). The mean total duration of infusion was 68 hours (range, 12-220 hr). Diazepam infusion was associated with hypotension in one patient, respiratory depression requiring ventilatory support in 12% of patients (6/49), and death in 14% of patients (7/49). Thiopental infusion was used in nine patients, including eight in whom diazepam infusion had failed. Thiopental infusion controlled seizures in all nine patients, but all of them needed mechanical ventilation, and seven needed vasopressor support for hypotension; four patients (44%) died. We conclude that continuous diazepam infusion is a reasonably effective modality to control refractory status epilepticus in children and is associated with reduced need for ventilatory and vasopressor support. (J Child Neurol 1998;13:23-26).
Frontal Lobe Epilepsy Associated With Tuberous Sclerosis: Electroencephalographic-Magnetic Resonance Image Fusioning
Authors: Seri Stefano , Cerquiglini Antonella , Pisani Francesco , Michel Christoph M. , Pascual Marqui Roberto D. , Curatolo Paolo
Published: December 2008

We studied the topographic relationships between cortical and subcortical lesions shown on magnetic resonance images (MRI) and sources of epileptiform activity in a series of nine children with intractable epilepsy and tuberous sclerosis complex. Although video-electroencephalographic (EEG) monitoring was suggestive of a frontal seizure onset, interictal EEG was, in seven of nine cases, in the form of apparently bisynchronous discharges. In all cases, the use of a short time lag estimation procedure based on a nonlinear correlation function between surface recorded EEG signals allowed the detection of a lateralized onset of EEG paroxysmal activity. Furthermore, a computerized method based on a source localization EEG-MRI image fusioning procedure, has revealed a topographic concordance between well-defmed frontal cortical lesions shown on MRI and site of onset of paroxysmal discharges. Lennox-like EEG patterns frequently reported in children with tuberous sclerosis complex could be the result of the tendency of frontal tubers to induce secondary bilateral synchrony, with implications in the medical and eventually surgical management of the often drug-resistant associated seizures. (J Child Neurol 1998;13:33-38).
Quantification of Cerebrospinal Fluid Proteins in Children by High-Resolution Agarose Gel Electrophoresis
Authors: Barnard Karin , Herold Ralf , Siemes Hartmut , Siegert Martin
Published: December 2008

Physiologic alterations in cerebrospinal fluid proteins occur inter alia with aging. Agarose gel electrophoresis discriminates many cerebrospinal fluid proteins and in addition quantifies concentration alterations. This study aimed to investigate the time course of these alterations in children and to establish normative values for cerebrospinal fluid protein properties. In 202 children without diseases known to alter cerebrospinal fluid, normative protein properties were quantified using nephelometry, ultrafiltration, high-resolution electrophoresis, and Gaussian curve fit densitometry. Total protein and protein concentrations (albumin and γ-globulins) decreased from birth until 7 months age, and, from then on, increased slightly (transthyretin, albumin, and α2-proteins) or strongly (γ-globulins). Protein proportions (transthyretin and transferrin) increased until about 3 years of age and decreased from then on. These normative values for children as quantified by high-resolution agarose gel electrophoresis are presented in a significance-structured percentile table. The time courses of these cerebrospinal fluid properties reflect physiologic alterations of the blood-brain barrier function during childhood. (J Child Neurol 1998;13:51-58).
Hand and Foot Growth Failure in Rett Syndrome
Authors: Schultz Rebecca , Glaze Daniel , Motil Kathleen , Hebert David , Percy Alan
Published: December 2008

Growth failure is a major aspect of the developmental arrest in Rett syndrome. Small hands and feet have been reported anecdotally, but the pattern of hand and foot growth has not been studied rigorously. The aims of this study were to characterize the hand and foot growth of 28 girls with Rett syndrome and to examine the relationship between their hand or foot size and height. Median hand length deviated to the 5th percentile at 11 years of age whereas median foot length and height deviated below the 5th percentile at 5.5 years of age. After adjusting for height-age, a greater proportion of foot lengths than hand lengths was less than the 50th percentile. In conclusion, the rate of hand and foot growth of girls with Rett syndrome is slower than that of the normal female. Moreover, the rate of deceleration of foot, but not hand, growth relative to height growth is greater. Thus, many girls with Rett syndrome have "small" feet, but not hands, for their height. (J Child Neurol 1998;13:71-74).
Influence of Eye Movements on Rett Stereotypies: Evidence Suggesting a Stage-Specific Regression
Authors: Umansky Richard , Watson John S.
Published: December 2008

The influence of eye movements on hand use and pervasive stereotypies in Rett syndrome was studied, to better understand the developmental abnormality in this disorder. Nine patients in the post-regression phase were offered objects. As expected, the girls looked at the objects but usually did not reach for them. Nor were their characteristic hand stereotypies altered by such looking. But the occurrence of stereotypies was promoted by overall shifts in gaze, ie, eye movements in any direction. This finding may be relevant to the late-infancy loss of voluntary hand use in Rett syndrome associated with onset of pervasive stereotypies. It suggests a stage-specific regression to a level characteristic of normal 3-month-old infants who do not reach and grasp, but whose hand clasping and mouthing may be triggered by the perceptual consequences of exploratory eye movements and shifts in gaze. (J Child Neurol 1998;13:158-162).
Children Who Can't Smell the Coffee: Isolated Congenital Anosmia
Authors: Assouline Sarit , Shevell Michael I. , Zatorre Robert J. , Jones-Gotman Marilyn , Schloss Melvin D. , Oudjhane Kalmadine
Published: December 2008

Two children with isolated congenital anosmia, a rare syndrome of deficient restricted neuronal migration, are presented with early diagnosis confirmed by standardized smell testing and detailed neuroimaging studies. Recognition of this disorder and its spectrum of presentations provides important insights into the molecular mechanisms underlying the development of the olfactory system. (J Child Neurol 1998;13:168-172).
Early Diagnosis of Subependymal Giant Cell Astrocytoma in Patients With Tuberous Sclerosis
Authors: Torres Orlando A. , Roach E.S. , Delgado Mauricio R. , Sparagana Steven P. , Sheffield Eugene , Swift Dale , Bruce Derek
Published: December 2008

We present 19 patients with tuberous sclerosis complex and subependymal giant cell astrocytoma. The mean age at the time of tumor diagnosis was 9.4 years (range, 1.5 to 21 years). Computed cranial tomography (CT) or cranial magnetic resonance imaging (MRI) identified the lesion which was resected in all cases. Seven patients had hydrocephalus and there was an interval increase in the tumor size or a large tumor without hydrocephalus in 12 patients. Surgical criteria included: (1) presence of hydrocephalus; (2) interval increase in tumor size; (3) new focal neurologic deficit attributable to the tumor; and/or (4) symptoms of increased intracranial pressure. Eight patients were identified through a surveillance program involving annual computed cranial tomography. All of these eight patients had their tumor removed prior to the development of symptoms, none had neurologic deficits which persisted after surgery, and none has so far developed recurrent subependymal giant cell astrocytoma. In contrast, of the 11 patients from the non-surveillance group 7 were symptomatic at tumor diagnosis, 1 had a complicated postoperative course, 2 developed recurrent giant cell astrocytoma, and 1 had an extensive lesion that could not be completely excised. Periodic cranial imaging may help to identify subependymal giant cell astrocytomas in tuberous sclerosis patients before they become symptomatic. Earlier diagnosis and treatment could reduce surgical morbidity and the risk of tumor recurrence. (J Child Neurol 1998;13:173-177).
Trends in Incidence and Severity of Intraventricular Hemorrhage
Authors: Sheth Raj D.
Published: December 2008

The incidence of germinal matrix-intraventricular hemorrhages declined from 50% in 1977 to 24% in 1985. Over the last decade intraventricular hemorrhage rates ranging from 8% to 56% were reported, leaving uncertainty as to the direction of recent intraventricular hemorrhage trends. Records of all 1950 neonates weighing 2250 g or less at birth (867 weighing 1500 g or less and 1083 weighing 1501-2250 g) at a university neonatal intensive care unit between 1986 and 1995 were studied. Intraventricular hemorrhage rate declined by 53%, from 11.5% in 1986 to 5.5% in 1995 (P < .01), and was consistent across all birthweight groups: 750 g or less from 36% to 24%, 751-1000 g from 38% to 22%, 1001-1250 g from 19% to 13%, 1251-1500 g from 12% to 2% and 1551-2250 g from 3% to 0.2% (P < .05). Proportionately, severe intraventricular hemorrhage (grades 3 ± intraparenchymal hemorrhage) declined from 70% of all intraventricular hemorrhages in 1986 to 23% in 1995 (P < .005). Overall mortality declined by 65% between 1986 and 1995 (P < .001), whereas mortality associated with intraventricular hemorrhage declined by 30% (P = .34). Despite dramatic declines in intraventricular hemorrhage rates, 21% of infants weighing less than 1000 g and 12% of those weighing less than 1500 g at birth were affected in 1995. (J Child Neurol 1998;13:261-264).
Neurologic Perspectives of Crigler-Najjar Syndrome Type I
Authors: Shevell Michael I. , Majnemer Annette , Schiff David
Published: December 2008

Limited information exists on the neurologic sequelae of Crigler-Najjar syndrome type I despite this being the major morbidity of this rare autosomal recessive disorder of bilirubin conjugation that results in chronic unconjugated hyperbilirubinemia. Two patients with identical underlying genetic mutations resulting in Crigler-Najjar syndrome type I were assessed from a neurodevelopmental perspective in late childhood using age appropriate standardized measures. In addition, the English language literature of case reports and series describing the outcomes of patients with this disorder was reviewed (descriptive meta-analysis) and summarized with particular reference to neurologic symptomatology, pattern of neurologic disability, age of onset of symptoms, and therapeutic interventions. Despite radically different therapeutic interventions, our two patients did not differ in outcome measures. Review of the literature reveals distinct, often age-related, patterns of neurologic sequelae reflecting injury to basal ganglia, cerebellar, and likely hippocampal structures. Definitive prevention of the neurologic sequelae that often occur within the context of Crigler-Najjar syndrome type I requires that curative treatment (hepatic transplantation, presently, and gene therapy in the future) be applied prior to the possible onset of neurologic symptoms in adolescence. (J Child Neurol 1998;13:265-269).
Evaluation of Cerebellar Size in Attention-Deficit Hyperactivity Disorder
Authors: Mostofsky Stewart H. , Reiss Allan L. , Lockhart Paula , Denckla Martha Bridge
Published: December 2008

Evidence from animal and human research suggests that the cerebellum may play a role in cognition. This includes domains of executive function that are normally attributed to the prefrontal cortex and are typically deficient in individuals with attention-deficit hyperactivity disorder (ADHD). To investigate cerebellar structure in ADHD, magnetic resonance imaging morphometry was used to measure the area of the cerebellar vermis in 12 males with ADHD and 23 male controls matched for age and Wechsler Full-Scale IQ. Analyses were conducted to evaluate group differences, as well as differences between matched pairs of subjects with ADHD and those without ADHD. All measurements were corrected for overall brain size. Both analyses revealed that the size of the posterior vermis was significantly decreased in males with ADHD (P < .05 in both analyses), and that within the posterior vermis, the inferior posterior lobe (lobules VIII-X) was involved in this reduction (P < .05 for group analysis, P < .005 for matched pair analysis), while the superior posterior lobe (lobules VI/VII) was not involved in the reduction. The finding of abnormal inferior posterior vermal size suggests that dysfunction within this region of the cerebellum may underlie clinical deficits seen in individuals with ADHD. (J Child Neurol 1998; 13:434-439).
Rett Syndrome: Significant Clinical Overlap With Angelman Syndrome but Not With Methylation Status
Authors: Ellaway Carolyn , Buchholz Tina , Smith Arabella , Leonard Helen , Christodoulou John
Published: December 2008

Rett syndrome and Angelman syndrome are neurodevelopmental disorders characterized by severe intellectual disability, microcephaly, speech disturbance, movement disorders with gait and/or truncal ataxia, and occasionally a similar facial appearance. Both conditions can be difficult to diagnose in girls early in their clinical course and can be difficult to distinguish from each other. Genomic imprinting is a known association in Angelman syndrome and previously has been suggested in Rett syndrome. Our aim was to evaluate the methylation status in a cohort of classical patients with Rett syndrome, using a methylation system for chromosome 15q11-13. Methylation analysis of chromosome 15 has not been previously reported in Rett syndrome. Furthermore, we document the clinical features of 31 girls with classical Rett syndrome and confirm the phenotypic similarities between Rett syndrome and Angelman syndrome. The methylation studies in these girls with Rett syndrome were normal. This excludes an imprinting error of the Angelman syndrome critical region on chromosome 15 (15q11-13) as an association with Rett syndrome, and indicates that methylation studies may be useful in distinguishing Rett syndrome from Angelman syndrome in young patients with an overlapping clinical phenotype. A normal methylation pattern, however, does not exclude the diagnosis of Angelman syndrome and clear distinction between the two syndromes will evolve over time. (J Child Neurol 1998;13:448-451).
Dihydropteridine Reductase Deficiency in a Large Consanguineous Tunisian Family: Clinical, Biochemical, and Neuropathologic Findings
Authors: Miladi Najoua , Larnaout Abdelmajid , Dhondt Jean-Louis , Vincent Marie-Françoise , Kaabachi Naziha , Hentati Fayçal
Published: December 2008

We report the case of a large consanguineous Tunisian family of seven siblings suffering from dihydropteridine reductase deficiency with either typical clinical, biochemical, or autopsy findings. Two cousins also were reported to have the same symptoms. This metabolic disorder is characterized by severe microcephaly, psychomotor regression, and progressive basal ganglia calcifications. Dihydropteridine reductase assay on samples collected from the two brothers still alive did not show measurable activity. The sister and four brothers died between the ages of 3 years and 7 years. A neuropathology study done on the sister showed diffuse demyelination throughout the white matter and spongy vacuolation in the subthalamic nuclei, the superior cerebellar peduncles and the tegmentum tracts of the brain stem. The anterointernal part of the putamen was completely necrotic with nearly total nerve cell loss. Abnormal vascular proliferation and calcification of the walls of small, medium, and large arteries and veins, as well as diffusely scattered pericapillary and isolated calcospherites, were seen in this necrotic region. We think that folate deficiency may be involved in the pathogenesis of the basal ganglia calcification. ( J Child Neurol 1998;13:475-480).
Congenital Muscular Dystrophy, White-Matter Abnormalities, and Neuronal Migration Disorders: The Expanding Concept
Authors: Mackay Mark T. , Kornberg Andrew J. , Shield Lloyd , Phelan Ethna , Kean Michael J. , Coleman Lee T. , Dennett Xenia
Published: December 2008

The congenital muscular dystrophies are a heterogeneous, recessively inherited group of disorders that have been subclassified on the basis of clinical central nervous system involvement. We report two children with "pure" congenital muscular dystrophy, one merosin negative and one merosin positive with extensive white matter and occipital cortical neuromigration abnormalities on magnetic resonance imaging (MRI). The first patient (merosin-negative congenital muscular dystrophy) presented with hypotonia and weakness in the neonatal period and subsequently was found to have a leukoencephalopathy and occipital cortical dysplasia on magnetic resonance imaging. The second patient presented with developmental delay without definite weakness. Initial investigations revealed a leukoencephalopathy and cortical dysplasia, but the patient subsequently was shown to have merosin-positive congenital muscular dystrophy. These patients illustrate that white-matter changes are not specific for merosin-negative congenital muscular dystrophy alone and that extensive cortical abnormality can be found in both groups of patients. In addition, our second patient illustrates a non-muscular mode of congenital muscular dystrophy presentation that should be considered in patients with a "nonprogressive leukodystrophy." (J ChiLd Neurol 1998;13:481-487).
Language Deficit With Attention-Deficit Disorder: A Prevalent Comorbidity
Authors: Tirosh Emanuel , Cohen Ayala
Published: December 2008

The aim of this study was to delineate the prevalence and behavioral patterns of children with attention-deficit and language problems as compared to children with attention-deficit hyperactivity disorder (ADHD) only Out of a cohort of 3208 children 6 to 11 years old, 5.2% were identified as having a primary ADHD. A teacher's behavioral questionnaire, pediatric interview and assessment, IQ, attention tests, and language evaluation were employed. A 45% rate of language problems was identified. This comorbidity is more prevalent among girls (P = .02). Sequencing and short-term memory were significantly related to attention-deficit and language problems, but the attention scores were not. Language performance was the best predictor of group assignment and was superior to IQ in that regard. Correlation analysis revealed a different behavioral pattern for the two groups. It appears that a significant proportion of children with ADHD have a language comorbidity not reflected by IQ assessments; therefore, language tests should be considered as part of their routine assessment. Children with attention-deficit and language problems appear to have a different neurocognitive pattern underlying their problems as compared with their peers with ADHD only. ( J Child Neurol 1998;13:493-497).
Serum Carnitine Levels in Epileptic Children Before and During Treatment With Valproic Acid, Carbamazepine, and Phenobarbital
Authors: Castro-Gago Manuel , Eirís-Puñal Jesús , Novo-Rodríguez Maria Inés , Couceiro José , Camiña Félix , Rodríguez-Segade Santiago
Published: December 2008

Serum levels of free, acyl, and total carnitine were determined in 32 patients with seizures, before and after 3, 6, and 12 months of treatment with valproic acid (17 patients), carbamazepine (10 patients), or phenobarbital (5 patients). In all three treated groups, both free and total carnitine levels showed a significant decline with respect to pretreatment levels. This decline was most marked and most consistent in patients treated with valproic acid. In 35% of the patients in this group, carnitine deficiency (ie, total camitine < 30 μmol/L) was observed by month 12. In none of the three groups were serum carnitine levels significantly correlated with the serum concentration of the drug. These findings suggest a need to monitor serum carnitine levels in children treated with any of these drugs. (J Child Neurol 1998;13:546-549).
Developmental Changes of Cortical and Cerebellar Motor Control: A Clinical Positron Emission Tomography Study With Children and Adults
Authors: Müller Ralph-Axel , Rothermel Robert D. , Behen Michael E. , Muzik Otto , Mangner Thomas J. , Chugani Harry T.
Published: December 2008

Functional neuroimaging data regarding the development of motor organization in normal children and adolescents are virtually unavailable because of ethical concerns. As an alternative approach, we studied child and adult lesion patients, focusing on movement of the hand ipsilateral to the lesion and on brain activations in the contralesional hemisphere. [15O]-water positron emission tomography was performed during rest and sequential finger-thumb tapping in 10 children (aged 6 to 14 years) and 15 adults (aged 18 to 74 years) with unilateral lesion. We expected more distinct activation/deactivation patterns during movement in adults than in children. While there were no group differences in activation of primary and secondary motor cortices, deactivations in nonmotor cortex were significantly more pronounced in adults than in children. This indirectly supports our hypothesis of developmental focalization of cerebral motor control. Activations in the cerebellum and vermis were significantly stronger in the adults than in the children, possibly reflecting normal developmental patterns. (J Child Neurol 1998; 13:550-556).
A Large-Scale Mitochondrial DNA Deletion Causing Progressive Ataxia
Authors: Saitoh Shigeko , Momoi Mariko Y. , Ohki Takehiro , Yamagata Takanori , Tsuru Tomohiko , Mizuguchi Masashi , Arima Kunimasa
Published: December 2008

Mitochondrial diseases are characterized by heteroplasmic mitochondrial DNA mutations and multisystemic dysfunction. Base substitutions of mitochondrial DNA have been reported in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), 1,2 myoclonus epilepsy with ragged red fibers (MERRF), 3 and other neuromuscular disorders.4 Heteroplasmic mitochondrial DNA deletions are usually detected in chronic external ophthalmoplegia, including Kearns-Sayre syndrome, 5 and on rare occasions in Pearson marrow-pancreas syndrome.6 A few unusual clinical complexes associated with single mitochondrial DNA deletions have been reported, such as maternally transmitted diabetes and deafness,7 diffuse leukodystrophy,8 MELAS, Fanconi's syndrome,9 pure myopathy and neuropathy,10 myopathy with lipomatosis, 11 and Leigh-type neuropathology.12

We report here a patient with a large-scale mitochondrial DNA deletion, which was located at a novel site and produced an atypical Kearns-Sayre syndrome phenotype. The major clinical sign at the early stage was not ophthalmoplegia, but cerebellar ataxia.
Phenytoin-Induced Dermatomyositis: Case Report and Literature Review
Authors: Dimachkie Mazen M. , Vriesendorp Francine J. , Heck Kent A.
Published: December 2008

A muscle biopsy done to evaluate for ragged red fibers in a patient with progressive myoclonic epilepsy unexpectedly showed prominent inflammation and perifascicular atrophy. Brain biopsy demonstrated Lafora bodies. Lafora body disease is a progressive, fatal myoclonic epilepsy presenting usually in the first or second decade with mental regression, generalized and myoclonic seizures,1,2 ataxia, spasticity, and involuntary movement. It is an autosomal recessive3 disorder localized to chromosome 6q. Phenytoin is herein implicated in the pathogenesis of dermatomyositis.
Topical Review: Use of Midazolam for Refractory Status Epilepticus in Pediatric Patients
Authors: Pellock John M.
Published: December 2008

Status epilepticus is more common among children than young adults. Children might be less likely to die and might be resistant to permanent neurologic damage due to status epilepticus, but significant sequelae also have been demonstrated. Aggressive intervention and rapid termination of seizures contribute significantly to better prognosis and reduced mortality from status epilepticus. Initial treatment of status epilepticus typically consists of either diazepam or lorazepam, immediately followed by phenytoin or phenobarbital. However, approximately 10% to 15% of status epilepticus episodes are refractory to these conventional therapies. Traditionally, refractory status epilepticus is treated with barbiturate coma or general anesthetics, both of which require invasive cardiorespiratory and hemodynamic monitoring and are associated with significant complications. Midazolam is a water-soluble benzodiazepine with a fast onset of action, a short half-life, and inactive metabolites that has been very effective in terminating seizures refractory to diazepam, lorazepam, phenytoin, and phenobarbital in pediatric patients. Midazolam is a valuable treatment option for refractory status epilepticus, especially in pediatric patients. ( J Child Neurol 1998;13:581-587).
High Prevalence of Bihemispheric Structural and Functional Defects in Sturge-Weber Syndrome
Authors: Maria Bernard L. , Neufeld Jennifer A. , Rosainz Louis C. , Ben-David Kfir , Drane Walter E. , Quisling Ronald G. , Hamed Latif M.
Published: December 2008

Abnormal cerebral venous drainage is associated with hypoxia and glucose deprivation, which can account for progressive neurologic deterioration in Sturge-Weber syndrome. Although developmental delay is common in Sturge-Weber syndrome, bihemispheric calcification is uncommon. Computed tomography (CT) and magnetic resonance imaging (MRI) were used to study the neuroanatomy, while single photon emission computed tomography (SPECT) was used concurrently to evaluate perfusion and glucose metabolism using 99mTc hexamethylpropyleneamine oxime (HMPAO) and [18F] fluorodeoxyglucose (FDG), respectively. Ten patients (10 to 22 years of age) with previously diagnosed Sturge-Weber syndrome, port-wine nevi, and clinical evidence of seizures or stroke-like episodes were studied. Five children with onset of seizures in the first year of life had overall clinical severity comparable to that of children with later-onset seizures. Calcification was present in both hemispheres in one patient; six additional patients had other radiologic evidence of bihemispheric disease; SPECT studies detected bihemispheric disease in four cases. Our study is the first to concurrently evaluate structure, perfusion, and glucose metabolism in Sturge-Weber syndrome and to show a mismatch between functional and structural brain imaging in both cerebral hemispheres. Widespread abnormalities of cerebral perfusion and glucose metabolism might explain the high prevalence of developmental delay associated with Sturge-Weber syndrome. Longitudinal studies are needed to define better the natural history of neurologic deterioration and radiologic progression that relates to central nervous system circulatory dysfunction in Sturge-Weber syndrome. ( J Child Neurol 1998;13:595-605).
Employment and the Americans with Disabilities Act of 1990
Authors: Linthicum Elena , Cole Jack T. , D'Alonzo Bruno J.
Published: December 2008

MOST LIFE ROLES that are important in the career development of individuals with disabilities are affected by the Americans with Disabilities Act of 1990 (ADA). In their roles as students, employers and employees, consumers, family members, and as citizens, individuals with disabilities will have more opportunities for participating, benefiting, and contributing in such roles than were previously available. It is imperative that individuals with disabilities, and their service providers, be aware of these opportunities and how the United States Congress intended them to be made available. The authors' purpose in this article is to provide an overview of how the ADA affects employment of individuals with disabilities. The examples are taken directly from congressional reports on the ADA.
Sharing The Journey: An Individual and Integrated Systems Approach to Self-Determination
Authors: Lehmann Jean P. , Deniston Terry L. , Tobin Romie , Howard Dawn
Published: December 2008

The purpose of this article is to describe a model for fostering the self-determination of adolescents with disabilities during the transition process. There are three phases of the model: (1) assessment, (2) planning, and (3) strategy implementation. During each phase, factors relating to individuals and the systems within which they interact are evaluated as either enhancing or preventing self-determination. Issues emerging from the use of this model are also discussed.
Differential Coursework of Students with Disabilities in High School: Implications for Performance on the ACT Assessment
Authors: Shookhi-Yekta Mohsen , Retish Paul M.
Published: December 2008

The purpose of this study is to investigate whether the more high school coursework completed by students with disabilities results in higher performance on the American College Testing entrance examination (ACT). Subjects include a total of 10,835 examinees with a diagnosed disability who took the ACT in 1991. The data were collected by ACT and includes the School Course/Grade Information questionnaire. Demographics including types of disabilities as well as the average ACT scores are presented. Multiple regression procedures are used to identify the unique contribution of each of the following variables in the prediction of the ACT scores: gender, disability, high school average, high school grade, credits earned, grade classification/ level, and type of school. The results indicate that, only for math, high school coursework completed is the best predictor of the corresponding ACT score' indicating that more time spent in math courses is associated with higher ACT Math performance. For English, however, high school credits earned do not appear to play a significant role in determining the ACT English score. Other findings, interpretations of the results, and practical implications are also discussed.
A Follow-Up Study of Former Special Education Students from a Rural and Urban County School System
Authors: Dunn Caroline , Shumaker Linda
Published: December 2008

This study examined the employment status of young adults with mental retardation, behavior disorders, or learning disabilities who had exited a rural (n = 25) or urban (n = 43) county school system within the past year. Additionally, the relationship between employment and demographic and secondary school variables were evaluated. The employment rate for students from the rural school system was 72%, while the employment rate for students from the urban school system was 81.4%. Subjects in rural settings were employed primarily in trade and industry (43.5%), whereas the subjects in the urban area were employed almost equally in service occupations (23.3%), and trade and industry (27.9%). The results of Chi-square analyses were significant for employment status and gender, employment status and disability, and employment status and paid employment during school.
Translating, Research, Innovation, and Policy into Practice: Interdisciplinary Transition Leadership Training (MS #97.05)
Authors: Flexer Robert W. , Baer Robert M. , Simmons Thomas J. , Shell Doreen
Published: December 2008

Transition leadership competencies that address outcome-based and student centered services stem from direct experience in delivering transition services across special education, vocational education, and rehabilitation disciplines. Doctoral trainees in transition at Kent State University are provided a program of studies and opportunities for leadership activities which are built upon their experiences as teachers, coordinators, counselors, and program developers. The doctoral program in Transition Leadership at Kent State University uses in-depth study and intensive field work to instill values and competencies which cross special education, rehabilitation, and vocational education. Through the use of an Individualized Leadership Career Plan, leadership goals and interdisciplinary competencies of each student are identified and focused on activities which foster translation of research into practice. A program description and formative evaluation are presented to demonstrate impacts of the program on research and practice.
Systems in Transition: Are We There Yet?
Authors: Guy Barbara , Schriner Kay
Published: December 2008

States receiving transition systems change projects from the Office of Special Education and Rehabilitative Services (OSERS) have been field sites for the development and testing of strategies intended to improve the abilities of systems to respond to new and continually changing demands for educational improvement. In this article, we describe results from a multi-year evaluation effort conducted by the National Transition Network. We found that change did occur in the delivery of transition services. Specifically, we found that states were successful in (a) increasing stakeholder awareness of transition needs and issues, (b) increasing the participation of students with disabilities and parents in transition activities, (c) enhancing collaborative relationships among school and community agencies, and (d) establishing and improving policies to support better transition services and outcomes. The strategies that were most likely to be effective in producing this change included (a) inducements and capacity-building activities; (b) sustained commitment of highly skilled individuals; (c) strategic integration of transition activities and resources within complementary initiatives; (d) involvement of all stakeholder systems; and (e) planning and using evaluation information.
The Statewide Systems Change Initiative as a Federal Policy Mechanism for Promoting Educational Reform
Authors: Cobb Brian , Johnson David R.
Published: December 2008

Since the late 1980s, the statewide systems change granting process has emerged as a mechanism in education by which federal interests in systemic educational reform are promoted. These reforms have occurred across the educational spectrum, with initiatives in special education (i.e., transition), vocational education (i.e., school-to-work), and general education (i.e., Goals 2000). While there have been differences in form and structure across these initiatives, there have been many commonalities, including, for example, application processes by states; federal intent on eventually funding all states; large-scale, multi-year funding; flow-through dollars to state agencies or a consortium of state agencies; an expectation of state funding of local demonstration efforts; meta-evaluation projects; and technical assistance to state grantees. This article highlights similarities and differences among these three major systems change initiatives. Discussions of these similarities and differences are presented along with thoughts about the efficacy of different elements of the statewide systems change initiative as a policy mechanism for promoting educational reform.
School Dropout among Youth with and without Learning Disabilities
Authors: Kortering Larry J. , Braziel Patricia M.
Published: December 2008

The purpose of this study was to compare the family background, schooling experiences, and postschool outcomes of youths with LD and peers without LD who all had dropped out of school. A total of 35 youth with learning disabilities (LD) and 60 youth with LD participated in this research. The two groups were comparable on most measures of family background, schooling experiences, and postschool outcomes. These results suggest that, at least in this study, youth with LD and youth without LD who had dropped out of school were reasonably equivalent. Thus, literature and research on school dropouts without LD from general education could have relevance for youth with LD and the way in which secondary special education programs are conducted. These implications are discussed and suggestions for practice are provided.
Post School Results For Youth With Disabilities: Key Indicators And Policy Implications
Authors: Colley Debra A. , Jamison Doris
Published: December 2008

This article presents information on the post school results of 720 former students who had received special education services. The results focused on employment, postsecondary education, and community living outcomes of former students and identified program components (including transition planning) that contribute to success, including work experiences, occupational education, and mainstreamed academics. Implications for policy deliberations and school practices are discussed.
Welfare Clients' Volunteering as a Means of Empowerment
Authors: Cohen Ayala
Published: December 2008

The population approaching social services for help is often characterized by poverty, passivity, helplessness, and an external locus of control. This population expects improvement in its situation but develops a dependent relationship with the help agents. The literature shows that volunteer work contributes toward developing empowerment among volunteers, but to date only a relatively small number of welfare clients have been activated as volunteers, serving more as the object of others' volunteer work. This article describes a classic experimental study, checking the connection between welfare clients' volunteering and their individual feeling of empowerment. The main and most significant finding in the study demonstrated that individual feeling of empowerment among clients who engaged in volunteer work was higher than that of clients not engaged in such work. The findings suggest that volunteer work may serve as an effective intervention tool in social work, whose aim is change in welfare clients' feelings of empowerment.
Synthesis and Characterization of Fluorinated Poly (imide siloxane) Copolymers Containing Anthracene Moieties in the Main Chain
Authors: Ghosh Anindita , Banerjee Susanta , Voit Brigitte
Published: December 2008

Five new co-poly(imide siloxane)s have been prepared by reacting two diamine monomers 9, 10-bis [3'-trifluoromethyl-4' (4''-aminobenzoxy) benzyl] anthracene (TFAA) and amino-propyl terminated polydimethylsiloxane (APPS) together with different commercially available dianhydrides like benzene-1,2,4,5-tetracarboxylic dianhydride (PMDA), 4,4'-(hexafluoro-isopropylidene)diphthalic anhydride (6FDA), 4,4'-oxydiphthalic anhydride (ODPA), 4,4'-(4,4' -Isopropylidenediphenoxy)bis(phthalic anhydride) (BPADA) and benzophenone-3,3',4,4'-tetracarboxylic dianhydride (BTDA). In each case the siloxane loading was adjusted to about 40 wt.% in the respective copolymers. The polymers were either synthesized by the one-pot solution imidization method or by the thermal imidization route. The synthesized polymers show predominantly good solubility in different organic solvents. The resulting polymers have been characterized by gel permeation chromatography, infrared and nuclear magnetic resonance techniques. These polyimides show good thermal stability with decomposition temperature (5% weight loss) up to 463 °C in nitrogen. Transparent thin films of these polyimides exhibit tensile strengths up to 31 MPa, a modulus of elasticity up to 0.63 GPa and elongation at break up to 110%.
Thermal, Catalytic and Antimicrobial Aspects of Poly[(2-hydroxy,4-ethoxyacetophenone)butylene] Resin and its Polychelates with 4f — block Elements
Authors: Kapadia Malav , Patel Mihir , Joshi Jayantilal
Published: December 2008

The polymeric ligand was synthesized by condensation of 2-hydroxy-4-ethoxyacetophenone with 1,4-butane diol in presence of polyphosphoric acid as a catalyst at 145 °C for 10 h. The synthesized polymeric ligand was used to synthesize the corresponding polychelates with 4f-block elements. The polymeric ligand and its polychelates were characterized by elemental analyses, electronic spectra, magnetic susceptibilities, Fourier transform infrared spectroscopy, nuclear magnetic resonance and thermogravimetric analyses. Morphological study of the polymeric ligand and polychelates were carried out by scanning electron microscopy. Number average molecular weight ( Mn) was determined by a vapor pressure osmometry method. Antimicrobial activity for all polychelates and catalytic activity of selected polychelates in organic synthesis were examined. It was observed from the study that the polychelates acted as an efficient catalysts and antimicrobial agents.
Respiratory Tract Reactions to Western Red Cedar
Authors: Blainey A.D. , Graham V.A.L. , Phillips M.J. , Davies R.J.
Published: December 2008

1 Asthma due to western red cedar (Thuja plicata) is well recognized, but has not been described frequently in the UK. Two patients who developed asthma and rhinitis due to occupational contact with western red cedar were studied. Both patients developed late asthmatic responses following bronchial challenge with western red cedar.

2 The challenge technique and the results of comparison between different wood dusts and dust extract are described.

3 The technique of anterior rhinometry was used to follow the nasal response to challenge in one patient, and demonstrated a late nasal reaction which followed a similar time course to the bronchial response.
Assessment of Lead Intakes and Dose-response for a Population in Ayr Exposed to a Plumbosolvent Water Supply
Authors: Sherlock J. , Smart G. , Forbes G.I. , Moore M.R. , Patterson W.J. , Richards W.N. , Wilson T.S.
Published: December 2008

1 Dietary lead intakes, blood lead concentrations and water lead concentrations were measured and their relationships investigated for 31 adults and 11 infants living in dwellings in Ayr with lead plumbing.

2 For adults, some lead intakes were found to be higher than the provisional tolerable weekly intake for lead, and for infants most of the intakes were high.

3 A cube root relationship fitted the data on blood lead versus water lead better than a linear relationship. Similarly, blood lead varied with the cube root of weekly dietary lead intake.

4 These cube root equations provided a means of estimating the impact on blood lead concentrations of exposure to lead from food and water. If cube root relationships correctly describe the association between these parameters, then the curve fitted to the results for adults indicates that the contribution to the blood lead concentrations from sources other than the diet and water was relatively small.
Induction of Hamster Hepatic Peroxisomal β-oxidation and Peroxisome Proliferation-associated 80000 mol. wt. Polypeptide by Hypolipidemic Drugs
Authors: Reddy M.K. , Lalwani N.D. , Qureshi S.A. , Reddy J.K.
Published: December 2008

1 The effects of hypolipidemic drugs fenofibrate (isopropyl[4-( p-chlorobenzoyl)-2-phenoxy-2-methyl]propionate), pyrinixil (BR-931; [4-chloro-6-(2,3-xylidino)-2-pyrimidinylthio( N-β-hydroxyethyl) acetamide]), methyl clofenapate (2-methyl-2[4-( p-chlorophenyl)phenoxy]2-methyl-propionate), and clofibrate (ethyl α- p-chloro-phenoxyisobutyrate) on plasma triglyceride levels, hepatic peroxisome proliferation and peroxisome-associated enzymes in hamsters were investigated.

2 Fenofibrate, pyrinixil and methyl clofenapate were administered in the diet at 0.2% level (w/w) for 6 weeks. Clofibrate was fed at 0.5% level.

3 Fenofibrate, pyrinixil and methyl clofenapate induced a marked proliferation of peroxisomes in hamster liver cells which was comparable to that observed in the rat and mouse liver, whereas the peroxisome proliferative effect of clofibrate was less pronounced. Peroxisomal fatty acid β-oxidation system was found in the hamster liver and its activity was enhanced significantly by hypolipidemic drugs. The magnitude of induction of [1-14C]palmitoyl CoA oxidation, heat-labile enoyl-CoA hydratase and peroxisome proliferation-associated 80000 mol. wt. polypeptide in the hamster appeared to parallel the extent of peroxisome proliferation.

4 All four hypolipidemic compounds increased hepatic catalase and carnitine acetyltransferase activities and decreased plasma triglyceride levels in the hamster. The observed hepatic effects of hypolipidemic drugs in hamster are identical to those induced by peroxisome proliferators in the rat and mouse.

5 These observations suggest that hypolipidemic agents identified as peroxisome proliferators in rats, mice and now hamsters would very likely enhance the peroxisomal enzyme system in other species. Additional information on the interspecies responses to peroxisome proliferators, however, is necessary to assess the role of peroxisome proliferation in carcinogenesis.
Comparison of the Inhibitory Action of Delta-9-tetrahydrocannabinol and Petroleum Spirit Extract of Herbal Cannabis on Human Sperm Motility
Authors: Hong C.Y. , Chaput de Saintonge D.M. , Turner P. , Fairbairn J.W.
Published: December 2008

1 Using the trans-membrane migration method to measure drug effects on human sperm motility, it is found that Δ-9-tetrahydrocannabinol is the major constituent of cannabis which inhibits sperm motility and unlike the cataleptic effect, other constituents in the petroleum spirit extract do not potentiate this effect significantly.

2 It is suggested that the mechanisms for sperm immobilization and cataleptic effects are different.
Effect of Pesticide Exposure on Human Microsomal Enzyme Induction
Authors: Uppal R. , Sharma P.R. , Chaudhury R.R.
Published: December 2008

1 The antipyrine half-life, metabolic clearance rate and the apparent volume of distribution were measured in six subjects handling malathion. These results were compared with six appropriate controls.

2 Occupational exposure of malathion in subjects reduced the antipyrine half-life from 9.18 ± 2.16 h in controls to 4.73 ± 1.65 h.

3 It is concluded that malathion exposure increases the rate of antipyrine elimination, possibly by inducing the hepatic microsomal enzymes.