bluuebunny/crossref_metadata_2025 huggingface dataset:
A Constructivist Approach to College-Level Economic Education
Authors: Ford Edward J. , Leclerc Daniel C.
Published: June 2007

This paper begins with a critique of the prevailing stimulus/response approach to economic education in which instructors communicate (typically via lecture) their view of economic reality and the student is expected to replicate that view. In the alternative constructivist approach, economic reality is understood by building on the student's own economic experiences. This process results in active and engaged learning. Initial class experimentation is discussed along with future research plans.
Global accumulation and accounting for national economic identity
Authors: Bryan Dick
Published: June 2007

As accumulation has globalized, national economic indicators such as the balance of payments have been increasingly called on to provide popular measures of national economic performance. Yet at the same time the possibility of defining the national economy as a unit is becoming more ambiguous. Reliance on conventional national indicators in the context of globally integrated accumulation is leading systematically to reinforce the subordination of labor to capital on a global scale.
One hundred years of???
Authors: McDermott John
Published: June 2007

Marxian socialism has bequeathed contradictory ideas about the course of capitalist development. There is "the proletariat," the anticipation that workers were being simplified into a uniform mass, living at the bottom of society, and "having no country of their own." On the other hand, there is an historical tendency for the relations and even the forces of production to become more deeply social in character, ultimately to burst the confining integument of private property and capitalism itself. Modem socialism continues to be influenced by echoes of that proletariat, but has tended to ignore opportunities provided by the increasingly social character of our mode of production, assuming that those are tasks to be taken up on some distant tomorrow. If the decline of socialism is to be radically reversed, the proletarian echo will have to be muted even further, and the increasing socialization of the mode of production taken up as an existing threat and a striking opportunity.

The expectation of a revolutionary proletariat neither can nor does function as the underpinning for socialist strategies of change. Of course, that is not the same thing as socialist sensitivity to the very poor and to inequality generally, but the two are often confused. If there were such a thing as proletarianization, then a realistic and ultimately dominant political movement could be based upon it, but contemporary socialist sympathy for the poor and the denied has much more morality than strategy to it, and to that extent does not supply a sufficiently acute political perspective. I am not saying, "Devil take the poor," nor denying their permanence while capitalism still exists. But an anticapitalist transformation of society now requires a different socialist fulcrum.
The impact of EEOC enforcement on the wages of black and white women: does class matter?
Authors: Wilhelm Sarah
Published: June 2007

The procedures used by the EEOC have been broadly criticized. The critiques are that the EEOC does not completely investigate all charges, and does not apply the law to the fullest benefit of discrimination victims. This paper contributes to these critiques with a finding that, among women, benefits from EEOC enforcement are funneled to white women and black women with relatively high levels of education. This distribution of benefits is due to the EEOC's investigation procedures.
You can't beat something with nothing: getting an alternative into the curculum
Authors: Goodwin Neva R.
Published: June 2007

This paper describes an alternative paradigm, "contextual economics," which is being developed, initially, in the form of an introductory microeconomics textbook. This paradigm gives special attention to the goals of economic actors and of the discipline of economics; and to the physical and the social/psychological contexts within which economies function.
To market, to market: Imperial capitalism's destruction of social capital and the family
Authors: Ciscel David H. , Heath Julia A.
Published: June 2007

The market, far from being the sine qua non for modem society in general, and the family in particular, has eroded the social fiber that gives shape and resilience to the experiences of individuals, families, and ultimately, the market itself. Reviewing both historical and feminist analysis of the family in a capitalist market society, it is clear that the modem market is imperial. Not only does it transform every human interaction into a transient market exchange, it undermines the basis for social reproduction through the family. Using the concept of social capital as a primary analytical tool, this paper argues that the distinction between home and market labor has been unnecessarily polarized, limiting policy options. Thus, society's ability to produce and maintain long-standing social networks is put at risk, paradoxically reducing the market's ability to perform efficiently.
Healing ourselves, healing our economy: paid work, unpaid work, and the next stage of feminist economic transformation
Authors: Matthaei Julie
Published: June 2007

This article presents a broad overview of U.S. economic history which identifies a next stage of feminist economic transformation. It identifies three semihistorical stages in the development of the economic self: gender polarization, gender freedom, and gender integration. Contemporary economic values, practices, and institutions-which were constructed on gender polarization between masculine paid economy-centered work and feminine unpaid family-centered work-are currently being undermined by the process of gender integration as individuals of both sexes straddle economy and family, masculinity and femininity, and as social movements organize to inject feminine caring into an increasingly heartless masculine economy. This process is leading to the healing of individuals, male-female relationships, and economic and social institutions, constituting a revolutionary new stage of feminist-inspired change.
Productive and unproductive labor: a reply to Houston and Laibman
Authors: Mohun Simon
Published: June 2007

Responding to criticisms of Mohun (1996) by Houston (1997) and Laibman (1999) in the RRPE, this paper explores the empirical consequences of the competing definitions proposed for productive labor, and concludes that abandoning the productive-unproductive labor distinction renders the Marxian theory of capitalist accumulation an unconvincing account of U.S. accumulation from 1948 to 1989.
Depths below depths: the intensification, multiplication, and spread of capitalism's destructive force from Marx's time to ours
Authors: Dowd Doug
Published: June 2007

The Marxian analytical framework remains essential for the understanding of contemporary capitalism; however, given the enormous changes 4ince his time, its constitutent elements are not only in need of "updating," but the relationships between them require important shifts in emphasis. Most especially is that so regarding the relationships between "base and superstructure." It is the strength of the latter that has enabled capitalism's ongoing "triumphs," despite its ever-increasing destructiveness to humanity, society, and nature. There has always been an urgency to our work as radical economists; today's existing and threatened calamities require us to intensify and integrate our analytical and political efforts, in ways urged by Gramsci long ago.
The profit rate: where and how much did it fall? Did it recover? (USA 1948-2000)
Authors: Dumenil Gerard , Levy Dominique
Published: June 2007

The decline of the profit rate since World War II, up to the early 1980s, is now widely acknowledged, and a new upward trend has been identified. This study presents new computations of profit rates using a definition like that used by Marx in Volume III of Capital, where profits correspond to total surplus-value, as well as a definition of the rate of profit that impacts individual firms, taking account of interest, taxes, and inventories. We show that both the level and trend of a specific group of industries (such as Railroads), called Highly Capital Intensive Industries, whose capital-labor ratio is very large, are totally different from that of other industries. (Their profit rate is very low and did not decline.) When these industries are removed from the data set, the downward trend of the profit rate and the limited recovery can be more clearly observed. Overall, between 1948 and 1982, the profit rate was divided by a coefficient ranging between 2 and 7, depending on the sector and definition of the profit rate. The profit rate in 2000 is still only half of its value in 1948. Finally, we show that the decline of the productivity of capital was the main factor in the fall of the profit rate, though the decline of the share of profits also contributed to this evolution.
Wages fund, high wages, and social conflict in a classical model of unemployment equilibrium
Authors: Davanzati Guglielmo Forges
Published: June 2007

The aim of the paper is to provide an explanation of involuntary unemployment in a classical (post-Ricardian) framework. The model describes a three-stage bargaining process with four agents (governments, firms, unions, workers). In the first stage, firms determine the wage offered, following a short-run version of the wages fund theory. In the second stage, unionists determine the wage demanded, which depends on workers' tendency to conflict, while firms decide the number of employed under a "right to manage" hypothesis. In the third stage, governments tax the wages fund and pay unemployment benefits. Public intervention is subject to a trade-off between accumulation and legitimation.
Labor Relations and Productivity Growth in Advanced Capitalist Economies
Authors: Buchele Robert , Christiansen Jens
Published: June 2007

In this paper, we claim that worker rights (including collective bargaining rights, employment protection, and income security) promote productivity growth. We argue that cooperative labor-management relations encourage workers to make positive contributions to technical and organizational innovations that raise labor productivity, and that an industrial relations system that secures strong worker rights fosters cooperative labor-management relations. These arguments are supported by an empirical analysis of long-run productivity growth in 15 advanced capitalist countries. We first develop an index of worker rights and show its positive effect on several indicators of labor-management cooperation. We then develop an index combining measures of worker rights and labor-management cooperation and show its positive effect on the rate of growth of labor productivity.
Profitability in Business Cycle Theory and Forecasting
Authors: Van Lear William
Published: June 2007

Given the important connections among profitability, investment, and economic activity, a profitability indicator can be used to assess where the economy is in the business cycle. Rising profitability suggests that the economy is on a secular growth path, while a peak or fall in profitability suggests that growth is slowing and the economy is headed for recession. One measure of profitability is to divide total business sector profit by total wages paid to labor. Let this variable be called the PW ratio.

This paper's research shows that the PW ratio leads recessions, and that it takes two to six quarters of decline in PW before the onset of recession. PW clearly peaks in stage three of the business cycle. The evidence demonstrates that the PW ratio compares favorably with other indicators used by forecasters. The paper concludes that wages are not responsible for squeezing profits until stage seven on average, and fluctuations in profit over the cycle exceed that of wages and the gap grows in late expansion.
A Predictive Model for the Intention to Implement Home Modifications: A Pilot Study
Authors: Yuen Hon Keung , Carter Rickey E.
Published: June 2007

This study was conducted to explore determinants related to older adults' intention to implement home modifications that can prevent falls. Eighty-seven community-dwelling older adults aged 60 or older completed a questionnaire examining potential determinants to predict an intention to implement home modifications. Multiple logistic regression suggests that the odds ratios (OR) for the participants' intention to implement home modifications were higher for those (a) who have higher levels of belief that home modifications are beneficial (adjusted OR = 2.41, p = .015), (b) who believe that environmental adaptation can help prevent future falls (adjustedOR= 4.61, p = .036), and (c) who have taken past action concerning changes in home environment (adjusted OR = 4.79, p = .029). Findings serve as a preliminary step toward developing a comprehensive predictive model to identify determinants in predicting the degree of adherence to recommended home modifications for fall prevention among older adults.
Perceived Social Support, Hassles, and Coping Among the Elderly
Authors: Fiksenbaum Lisa M. , Greenglass Esther R. , Eaton Judy
Published: June 2007

An essential aspect of aging is successful coping. This entails learning how to deal effectively with change, losses, disappointments, and decline. The present study examined the relationship between coping, social support, daily hassles, functional disability, and physical and psychological health status in a sample of 224 community-residing older adults. Data were collected using a confidential and anonymous questionnaire. Results of structural equation analyses showed that social support was associated with fewer daily hassles. Social support was also indirectly related to daily hassles—that is, by increasing proactive coping. Further results indicated that proactive coping was inversely related to health hassles and functional disability. Proactive coping also was indirectly related to somatization and functional disability through health hassles. Results also showed that greater functional disability was associated with greater somatization. Implications of the results for healthy psychological functioning in the elderly are discussed.
Medical Communication in Older American Indians: Variations by Ethnic Identity
Authors: Garroutte Eva Marie , Kunovich Robert M. , Buchwald Dedra , Goldberg Jack
Published: June 2007

The authors analyzed audiotapes from 102 patients of American Indian race (≥50 years) to explore how ethnic identity influences medical communication. A standardized interaction analysis system was used to classify patient utterances into categories: information-giving, questions, social talk, positive talk, negative talk. The authors identified patient subgroups distinguished by level of identification with American Indian and White identity and explored whether some subgroups devoted more communication to certain categories of talk. Patients highly affiliated with American Indian identity devoted a significantly greater percentage of communication to “positive talk”—including statements of optimism, reassurance, and agreement—than patients identifying at lower levels (p > .05). They devoted less communication to “negative talk,” including corrections, disagreements, and anxiety statements (p < .05). Effects persisted after adjustment for confounders, including health status. Patterns may encourage providers to underestimate distress and overestimate satisfaction and comprehension in patients highly affiliated with American Indian identity.
Variations in Latino Parenting Practices and Their Effects on Child Cognitive Developmental Outcomes
Authors: De Von Figueroa-Moseley Colmar , Ramey Craig T. , Keltner Bette , Lanzi Robin G.
Published: June 2007

This research examines variations in parenting and its effects on child cognitive outcomes across Latino subgroups from a national sampling that utilized a subset of 995 former Head Start Latino parents and children. Comparisons of the Parenting Dimension Inventory scaled scores revealed Latino subgroup differences on nurturance and consistency. Puerto Rican parental caregivers scored higher on nurturance and consistency than Mexican Americans and El Salvadorans. Pearson’s product-moment correlation revealed a positive relationship between responsiveness to child inputs and higher academic achievement scores for each Latino subgroup. Examination of the Woodcock-Johnson Test of Achievement also revealed a main effect of Latino subgroup differences. Findings suggest that there exist intracultural variations in parenting and academic achievement within the Latino population and confirm previous findings that parenting is related to positive child cognitive outcomes.
Acculturation and Teaching Styles Among Young Mainland Puerto Rican Mothers
Authors: Teichman Jennifer R. , Contreras-Grau Josefina M.
Published: June 2007

This study examined associations between acculturation and teaching styles among 55 young mainland Puerto Rican mothers. Mothers ( M age = 19.2 years) and their children ( M age = 19.2 months) were videotaped during a puzzle-completion teaching task. Three aspects of acculturation were assessed: language, cultural involvement, and exposure to Euro-American culture. More highly acculturated mothers used less modeling and more verbal inquiry and encouragement than relatively unacculturated mothers. The overall teaching styles were also associated with acculturation: Whereas the least acculturated mothers employed a predominantly nonverbal and directive style, the style of more acculturated mothers was more verbal in nature, in both directive and nondirective modes. The findings suggest that an increase in acculturation may be related more strongly to an increase in the use of verbal tactics than an increase in nondirective ones.
Culture, Consumption, and Adult Education: Refashioning Consumer Education for Adults as a Political Site Using a Cultural Studies Framework
Authors: Sandlin Jennifer A.
Published: June 2007

The field of adult education exists within a context of consumer capitalism, although adult educators have failed to acknowledge how central consumption is to today’s society. Traditional consumer education has typically focused on technical skills, and thus positions itself outside the social, political, and cultural realms. In this article, the author retheorizes consumer education into a more critical enterprise using the framework of cultural studies. She argues that consumer education is a political site that creates consumers with a range of reactions to consumer culture. From this perspective, consumer education for adults is reconceived to include a variety of informal sites of learning including those focusing on curbing consumption, fighting consumer capitalism, and “jamming” corporate-sponsored consumer culture.
Cross-National Gender Differences in Mathematics Achievement, Attitude, and Self-Efficacy within a Common Intrinsic Structure
Authors: Randhawa Bikkar S. , Gupta Ashum
Published: June 2007

Because of a marked increase in the Canadian immigrant and refugee population in recent years, school psychologists today are frequently called upon to make recommendations concerning the learning needs of students from many diverse backgrounds and cultures. The purpose of this study was to examine possible gender as well as cultural differences in mathematics attitude, achievement, and self-efficacy between Canadian high school students and English-speaking high school students from a northern city in India. Although the participants, tested in their home countries, were administered the same battery of measures, the findings revealed, among other things, significant multivariate and corresponding univariate country, gender, and country x gender interaction effects. The importance of these findings for the practice of school psychology is discussed.
The Calculation of the WISC-Ill General Ability Index Using Canadian Norms
Authors: Weiss Lawrence G. , Saklofske Donald H. , Prifitera Aurelio , Chen Hsin-Yi , Hildebrand Denise
Published: June 2007

Since the publication of the Wechsler Intelligence Scalefor Children - Third Edition (WISC-III, 1991), several alternative scoring frameworks have been developed based on American norms. One such framework is the General Ability Index (GAI). The GAI provides a composite score using the eight subtests from the Verbal Comprehension Index (VCI) and the Perceptual Organization Index (POI). The GAI is an appropriate substitute for the Full Scale IQ score if the VCI and POI are considered better estimates of the child's verbal and nonverbal reasoning abilities than the Verbal IQ and the Performance IQ scores. The tables for calculating the GAI using Canadian norms are presented.
Febrile Seizures
Authors: Shinnar Shlomo , Glauser Tracy A.
Published: June 2007

Febrile seizures are the most common form of childhood seizures, occurring in 2 to 5% of children in the United States. Most febrile seizures are considered simple, although those with focal onset, prolonged duration, or that occur more than once within the same febrile illness are considered complex. Risk factors for a first febrile seizure, recurrence of febrile seizures, and development of future epilepsy are identifiable and varied. Children with febrile seizures encounter little risk of mortality and morbidity and have no association with any detectable brain damage. Recurrence is possible, but only a small minority will go on to develop epilepsy. Although antiepileptic drugs can prevent recurrent febrile seizures, they do not alter the risk of subsequent epilepsy. This has led to a changing view of how we approach the treatment of these common and largely benign seizures. This chapter will review the current understanding of the prognosis and management of febrile seizures. (J Child Neurol 2002;17:S44—S52).
Three New Drugs for Epilepsy: Levetiracetam, Oxcarbazepine, and Zonisamide
Authors: Leppik Ilo E.
Published: June 2007

During the last decade, nine new antiepileptic drugs were approved for use by the US Food and Drug Administration. The characteristics of three of these new antiepileptic drugs—levetiracetam, oxcarbazepine, and zonisamide—are reviewed here. Their individual characteristics, including mechanism of action, efficacy, safety, and pharmacokinetics, are compared, and their effectiveness in treating specific seizure types is noted. As with all antiepileptic drugs, the efficacy, side-effect, and pharmacokinetic profiles must be matched to each individual's clinical profile to attain the maximum benefit. All three are unique and will be useful in expanding the aggregate of therapies available to clinicians treating diverse epilepsy syndromes and seizure types. This review focuses on results in adults; pediatric experience is reported in other articles in this supplement. (J Child Neurol 2002; 17:S53—S57).
Advancing the Medical Management of Epilepsy: Disease Modification and Pharmacogenetics
Authors: Glauser Tracy A.
Published: June 2007

Despite the recent development of new antiepilepsy drugs, a significant number of children are still unable to achieve seizure freedom without side effects. Understanding the factors behind individual variability in antiepilepsy drug tolerability and dose response and incorporating these factors into a treatment plan would represent an important advance in epilepsy pharmacotherapy. A more thorough understanding of the epileptogenic process may allow clinicians to select antiepilepsy drugs that interrupt or modify various steps in the epileptogenic progression (ie, disease modification). Additionally, advances in the understanding of human genetics may allow for selection of antiepilepsy drugs and dosage regimens based on a patient's clinical characteristics and genotype (ie, pharmacogenetics). This article focuses on these two areas of potential improvement in the medical treatment of patients with epilepsy. Such methods of tailoring antiepilepsy drug therapy would be preferable to the trial-and-error system that is currently used. (J Child Neurol 2001;17:S85—S93).
Test Reliability and Stability of Children's Cognitive Functioning
Authors: Spencer Fiona H. , Bornholt Laurel J. , Ouvrier Robert A.
Published: June 2007

This study addresses the test reliability of a screening test and stability of children's cognitive functioning. Children aged 5 to 8 years in western Sydney were assessed on three occasions. The first assessment provided a baseline, with the second assessment at 2-, 4-, or 12-week intervals. The final assessment was 4 weeks later. Indicators of reliability and stability suggested that a distinction can be made between test reliability and the phenomenon (cognitive functioning) stability. Cognitive functioning was assessed using the School-Years Screening Test for the Evaluation of Mental Status (SYSTEMS). The findings have implications for indicators of reliability and stability of cognitive assessments in developmental research and clinical practice. ( J Child Neurol 2003; 18: 5—11).
Mental Retardation and Early Onset of Weakness in a Girl With a Dystrophinopathy and a Large Xp21-23 Deletion
Authors: Falsaperla Raffaele , Romeo Giusi , Sorge Anna , Bianchini Rio , DiGiorgio Angelo , Trigilia Tatiana , Mattina Teresa , Connolly Anne M.
Published: June 2007

A 2-year-old girl presented with severe global developmental delay, weakness, and an elevated serum creatine kinase level. Her muscle biopsy was consistent with an active dystrophy with absence of dystrophin in about half of the muscle fibers. Fluorescent in situ hybridization analysis showed her karyotype to be 46, X, del X p23.1-p21.1. This large deletion includes the dystrophin gene as well as the region involved in X-linked mental retardation. The genetic mechanism for the manifestation of both diseases is likely non-random inactivation of the X chromosome. To our knowledge, the combination of this dystrophinopathy in association with severe mental retardation has not been described in a girl. ( J Child Neurol 2003; 18: 79—81).
Molecular Biology and Ontogeny of γ-Aminobutyric Acid (GABA) Receptors in the Mammalian Central Nervous System
Authors: Simeone Timothy A. , Donevan Sean D. , Rho Jong M.
Published: June 2007

γ-Aminobutyric acid (GABA) is the predominant inhibitory neurotransmitter in the mammalian central nervous system. After release from nerve terminals, GABA binds to at least two classes of postsynaptic receptors (ie, GABAAand GABAB), which are nearly ubiquitous in the brain. GABAAreceptors are postsynaptic heteropentameric complexes that display unique physiologic and pharmacologic properties based on subunit composition. Activation of GABAAreceptors in mature neurons results in membrane hyperpolarization, which is mediated principally by inward chloride flux, whereas in early stages of brain development, GABAAreceptor activation causes depolarization of the postsynaptic membrane. GABABreceptors reside both presynaptically and postsynaptically, exist as heterodimers and are coupled to voltage-dependent ion channels through interactions with heterotrimeric G proteins. This review summarizes the molecular biology and ontogeny of GABAAand GABABreceptors, highlighting some of their putative roles during normal brain development as well as in disease states such as epilepsy. ( J Child Neurol 2003; 18: 39—48).
Quantitative Analysis of Gray- and White-Matter Volumes and Glucose Metabolism in Sturge-Weber Syndrome
Authors: Pfund Zoltán , Kagawa Kenji , Juhász Csaba , Chenggang Shen  , Joon Soo Lee  , Chugani Diane C. , Muzik Otto , Chugani Harry T.
Published: June 2007

The progressive nature of Sturge-Weber syndrome is well known, but the mechanisms of focal cortical and subcortical degeneration in this disorder are poorly understood. In the present study, we assessed the structural and functional integrity of gray and white matter in unihemispheric Sturge-Weber syndrome using quantitative magnetic resonance imaging (MRI) volumetry and MRI-based partial volume correction of [18F]fluorodeoxyglucose positron emission tomographic (PET) images. Gray- and white-matter volumes and glucose metabolism were measured in three brain regions (parieto-occipital underneath the angioma, temporal, and frontal) in six children with Sturge-Weber syndrome (two infants, ages 6 and 9 months; four older children, ages 4 to 14 years), all with unilateral parieto-occipital leptomeningeal angiomatosis. The gray-matter volumes ipsilateral to the angioma were smaller in all children, with the posterior regions underneath the angioma the most affected. In the infants, the white-matter volumes were increased in the region of the angioma, whereas in the regions remote from the angioma in the infants and in all regions of the older children, there were large decreases in white-matter volume. The decreases of frontal and temporal white-matter volume were more pronounced than the corresponding gray-matter volume decreases. The PET studies showed severe hypometabolism in the parieto-occipital region underneath the angioma in all of the children. However, the two infants showed glucose hypermetabolism in the frontal and temporal cortical gray matter, whereas these regions had relatively preserved metabolism in the older patients. These results demonstrate differential involvement of gray and white matter in Sturge-Weber syndrome. Both structural and functional abnormalities extend well beyond the angioma, indicating widespread abnormalities of growth and development of the affected hemisphere. Furthermore, whereas increased white-matter volume underlying the angioma may be seen in infants, ipsilateral white-matter regions outside the angioma show volume loss both in infants and in older patients. Extensive gray- and white-matter volume loss and hypometabolism ipsilateral to the angioma likely contribute to the frequently observed progressive cognitive dysfunction in these patients, regardless of the extent of the angioma. ( J Child Neurol 2003; 18: 119—126).
Cardiac Complications of Childhood Myopathies
Authors: Muntoni Francesco
Published: June 2007

The management of individuals with a neuromuscular disorder is usually focused on the skeletal muscle weakness and resulting complications, such as respiratory failure. Long-term prognosis of a number of neuromuscular conditions is, however, also determined by the type and severity of cardiac involvement. Early recognition and treatment of the cardiovascular complications are part of the task of the multidisciplinary team involved in the care of these patients. Although for several of the common conditions, there is general consensus on the cardiac investigations and treatments, in the rarer disorders, evidence-based recommendations are not available, and suggestions from experts provide an acceptable solution. This review summarizes the recent advances in our understanding of the pathogenesis and phenotypic diversity of cardiac complications associated with pediatric myopathies and provides a rational framework for planning the monitoring and therapeutic intervention in individual conditions. ( J Child Neurol 2003; 18: 191—202).
Hypotonia, Congenital Hearing Loss, and Hypoactive Labyrinths
Authors: Bodensteiner John B. , Smith Shelly D. , Schaefer G. Bradley
Published: June 2007

The records of all patients attending a neurosensory genetics clinic over an 11-year period were reviewed. Of the 450 patients seen, 31 presented with sensorineural hearing loss, hypotonia, and delay in the acquisition of motor milestones. Of these, 4 children were found who did not have an etiologic diagnosis such as Down syndrome or cerebral palsy. Vestibular testing revealed hypoactive labyrinthine function in all 4 of the cases, and careful imaging of the temporal bone showed anomalous development of the cochlea, vestibule, and semicircular canals in 3 of the 4 cases. None of the patients had ataxia, tremor, or significant nystagmus. Over time, the hypotonia improved in all, and none were felt to have cognitive deficits. These cases demonstrate that hypoactive labyrinthine function may be associated with hypotonia that is severe enough to result in delayed acquisition of motor milestones. The patients followed the typical remitting course of “benign congenital hypotonia.” The distinguishing clinical feature is the presence of moderate to profound sensorineural hearing loss in all of the patients. ( J Child Neurol 2003;18:171—173).
Pontocerebellar Hypoplasia Type 1: New Leads for an Earlier Diagnosis
Authors: Salman Michael S. , Blaser Susan , Buncic J. Raymond , Westall Carol A. , Héon Elise , Becker Laurence
Published: June 2007

Pontocerebellar hypoplasia type 1 is a rare disease characterized by pontocerebellar hypoplasia and anterior horn cell degeneration. The oldest reported child died at the age of 26 months. Two siblings were diagnosed with pontocerebellar hypoplasia type 1 after the death of the second sibling at 40 months of age from respiratory failure and the unexpected finding of anterior horn cell degeneration on her autopsy. The older sibling was a boy who was labeled as having cerebral palsy. He died at 14 months of age from pneumonia following a clinical course similar to his sister's, who was born 5 years after his death. Both siblings had significant global developmental delay with axial and peripheral hypotonia initially. Peripheral hypertonia with brisk reflexes developed later but were absent prior to death. Extensive investigations in the second sibling ruled out known metabolic (including congenital disorders of glycosylation) and mitochondrial diseases using skin fibroblast cultures and enzyme analysis. Genetic testing for Friedreich's ataxia; neuropathy, ataxia, and retinitis pigmentosa (NARP); spinal muscular atrophy; and spinocerebellar ataxia type 1, 2, 3, 6, 7, and 8 gene abnormalities was negative. The electroretinogram showed a previously unreported finding of abnormal and progressive rod/cone response. Our cases provide clinical and previously unreported electroretinographic evidence for neuro-degeneration in pontocerebellar hypoplasia type 1 and call for the expansion of the disease phenotype. ( J Child Neurol 2003; 18:220—225).
Speed of Performance of Children With Developmental Right Hemisphere Syndrome and With Attention-Deficit Hyperactivity Disorder
Authors: Landau Yael E. , Auerbach Judith G. , Gross-Tsur Varda , Shalev Ruth S.
Published: June 2007

Slowness is a common complaint in children with attention-deficit hyperactivity disorder (ADHD) and with developmental right hemisphere syndrome. However, it was our clinical impression that slowness in developmental right hemisphere syndrome was more prominent than in ADHD. Our objective was to assess slowness as operationalized by speed of performance in children with developmental right hemisphere syndrome, children with ADHD, and controls. The research sample comprised 19 children in each group, matched for age, gender, socioeconomic status, IQ, and handedness. The subjects were administered a reaction time battery assessing speed of performance. Overall, the average performance differed among the three study groups ( F(2,53) = 2.40, P < .01). Children with developmental right hemisphere syndrome were slower than their peers with ADHD ( t(35) = 1.99, P < .05) and slower than controls ( t(35) = 4.55, P < .001). Children with ADHD performed more slowly than controls, although for the majority of tasks, this was nonsignificant. We conclude that slowness is an integral and consistent component of developmental right hemisphere syndrome and cannot be attributed only to the ADHD symptomatology. ( J Child Neurol 2003;18:264—268).
Ocular Motor Behavior of Children With Neurofibromatosis 1
Authors: Lasker Adrian G. , Denckla Martha B. , Zee David S.
Published: June 2007

Patients with neurofibromatosis 1 show neurocognitive deficits including abnormal visuospatial performance, but oculomotor behavior has not been studied. We recorded saccades in neurofibromatosis 1 and normal children, ages 6 to 12 years. Patients showed increased latency and diminished amplitude to more eccentric targets for both visually guided and memory-guided saccades. Subtracting the latencies of visually guided saccades from memory-guided saccades produced no differences between groups. For predictive saccades (0.5 Hz), patients with neurofibromatosis 1 generated fewer anticipatory saccades than normal subjects, but patients with neurofibromatosis 1 made more direction errors in an antisaccade task. Patients with neurofibromatosis 1 show saccade abnormalities that are not easily ascribed to dysfunction within single cortical areas. Rather, the findings likely reflect (1) partial involvement of different components of a widely distributed cerebral network that controls saccades, (2) impaired visuomotor maturation in a brain abnormal early in development, and (3) excessive γ-aminobutyric acid—mediated inhibition, which could influence basal ganglia pathways that modulate superior colliculus activity. ( J Child Neurol 2003;18:348—355).
Topical Review: Acute Ataxia in Childhood
Authors: Ryan Monique M. , Engle Elizabeth C.
Published: June 2007

Acute childhood ataxia is a common cause of presentation to the pediatric emergency room or child neurologist. The primary concern on initial assessment is to exclude serious causes of this clinical syndrome, including central nervous system infections and mass lesions, while recognizing the essentially benign nature of acute ataxia in most children. Childhood ataxia can be diagnostically approached by consideration of the temporal course and presence or absence of associated neurologic abnormalities. In all forms of childhood ataxia, outcome is largely determined by etiology. In this review, the various causes and syndromes of acute ataxia in childhood are described, with discussion of diagnostic considerations and an approach to investigation, treatment, and prognosis. ( J Child Neurol 2003;18:309—316).
Moyamoya Disease Presented With Migrainelike Headache in a 4-Year-Old Girl
Authors: Aydin Kürşad , Okuyaz Çetin , Gücüyener Kvlcm , Serdaroğlu Ayşe , Akpek Sergin
Published: June 2007

A 4-year-old girl was admitted to our department with the complaints of severe headache, nausea, vomiting, and photophobia. She had a 1-year history of migrainelike headache that occurred every 1 to 2 months. Her neurologic examination was normal, but T2-weighted axial magnetic resonance imaging (MRI) of the brain showed flow void within the basal ganglia owing to parenchymal vascular collaterals. Magnetic resonance angiography and digital substraction carotid angiography showed both occlusion of the internal carotid artery in the supraclinoid portion and extensive parenchymal vascular collaterals. Because there was no evidence of risk factors for cerebral arterial occlusion and cerebral infarct or hemorrhage, she was diagnosed as having moyamoya disease. In any case of atypical migrainelike headache, a detailed investigation should be kept in mind to detect an underlying vascular disease such as moyamoya. ( J Child Neurol 2003;18:361—363).
Delayed Visual Maturation Associated With Auditory Neuropathy/Dyssynchrony
Authors: Aldosari Mohammed , Mabie Ann , Husain Aatif M.
Published: June 2007

Delayed visual maturation is a term used to describe infants who initially seem blind but subsequently have a marked improvement. The mechanism of visual loss and the subsequent improvement remains unknown. Auditory neuropathy/dyssynchrony is a condition of hearing impairment associated with absent or severely abnormal brainstem auditory evoked potentials but normal cochlear functions as measured by otoacoustic emissions. In this report, a 9-month-old infant who had no visual fixation for the first 3 months of life and congenital hearing impairment is described. Her brainstem auditory evoked potential study at 2.5 months of age showed no response to click stimuli presented at 90 dB nHL, whereas her otoacoustic emissions were normal. Subsequently, her vision and hearing improved. A brainstem auditory evoked potential study at 9 months of age showed reproducible waveforms. This case suggests the need for a detailed hearing evaluation of children with delayed visual maturation. Furthermore, this case highlights the need for follow-up brainstem auditory evoked potential testing prior to pursuing any audiologic intervention. ( J Child Neurol 2003; 18: 358—361).
Costello Syndrome and Chiari I Malformation: Apropos of a Case With a Review of the Literature Regarding a Potential Association
Authors: Tubbs R. Shane , Oakes W. Jerry
Published: June 2007

We report a child with Costello syndrome and Chiari I malformation. The medical literature has several case reports of findings peculiar for each of these two clinical entities that, when investigated, can actually be found in both processes. Recent reports have shown additional medical coincidences for the Chiari I malformation, which, unlike the Chiari II malformation, was once thought not to have many additional associations. We propose that both Costello syndrome and other clinical entities that have potential mesodermal or ectodermal deficiencies (eg, Chiari I malformation and phakomatoses, respectively) could have common dysgeneses. Further case reports from other institutions regarding Costello syndrome and Chiari I malformation are now necessary to confirm our findings. Our hope is that these data will potentially add to our knowledge of the etiology of both Costello syndrome and Chiari I malformation and potentially aid in the definition of a genetic locus for both entities. ( J Child Neurol 2003;18:496—498).
Unusual Presentation of Lafora's Disease
Authors: Al Otaibi Suad F. , Minassian Berge A. , Ackerley Cameron A. , Logan William J. , Weiss Shelly
Published: June 2007

Lafora's disease is a progressive myoclonus epilepsy with onset in adolescence and a gradual decline in cognitive functions and increase in seizure intractability. We present the case of a 16-year-old with precipitous dementia within 6 months of onset. Peripheral biopsies and EPM2A mutation analysis were negative. The diagnosis could be established only by brain biopsy. ( J Child Neurol 2003;18:499—501).
Serum Carnitine Levels During Oxcarbazepine and Carbamazepine Monotherapies in Children With Epilepsy
Authors: Kurul Semra , Dirik Eray , Iİscan Akin
Published: June 2007

Prolonged antiepilepsy drug treatment can result in secondary carnitine deficiency. The effect of oxcarbazepine on carnitine metabolism has not been reported previously. In this study, serum concentrations of total and free carnitine were measured in 20 children with epilepsy treated with oxcarbazepine monotherapy and were compared with 20 children with epilepsy who were taking carbamazepine as monotherapy. The assays were performed between 3 and 6 months of anticonvulsant treatment. The mean values of serum total and free carnitine levels in patients receiving carbamazepine monotherapy were 63.0 ± 20.7 μmol/L and 49.1 ± 16.7 μmol/L, respectively. The mean values of serum total and free carnitine levels in patients receiving oxcarbazepine monotherapy were 64.2 ± 17.4 μmol/L and 50.3 ± 13.7 μmol/L, respectively. The values were all between normal ranges. No significant difference was observed in the level of total and free carnitine levels between the two groups. Our results suggest that neither oxcarbazepine nor carbamazepine as monotherapy causes carnitine deficiency in otherwise healthy children with primary idiopathic epilepsy. ( J Child Neurol 2003;18:552—554).
Favorable Seizure Outcome in Kabuki Make-up Syndrome Associated With Epilepsy
Authors: Ogawa Atsushi , Yasumoto Sawa , Tomoda Yasuko , Ohfu Masaharu , Mitsudome Akihisa , Kuroki Yoshikazu
Published: June 2007

Kabuki make-up syndrome is a mental retardation—malformation syndrome affecting multiple organ systems, with a broad spectrum of neuromuscular dysfunction and mental ability. The incidence of seizures associated with this syndrome ranges from 10 to 40%. However, details of the seizures in this syndrome have not been adequately reported or thoroughly evaluated. In this study, we analyzed seizure characteristics and clinical outcomes in nine patients with Kabuki make-up syndrome. Four patients had generalized seizures and two patients had complex partial seizures, extended to secondary generalized seizures. West's syndrome, complex partial seizure, and atonic seizure were seen in one case each, respectively. Electroencephalograms showed focal spikes in seven cases, diffuse spike and wave burst in one case, and hypsarrhythmia in one case. Seizures were well controlled in eight cases and incompletely controlled in only one case. Together with mental retardation, epilepsy can be a primary feature of Kabuki make-up syndrome. Epilepsy associated with Kabuki make-up syndrome is mainly localization-related epilepsy with a favorable seizure outcome. ( J Child Neurol 2003;18:549—551).
Clinical Correlates of Frontal Intermittent Rhythmic Delta Activity in Children
Authors: Watemberg Nathan , Gandelman Revital , Neufeld Miri Y. , Ginsberg Mira , Lerman-Sagie Tally , Kramer Uri
Published: June 2007

Frontal intermittent rhythmic delta activity is an electrographic pattern of unclear origin. Previously thought to correlate with deep midline and infratentorial pathology, rather, it appears to be associated with encephalopathy states in adults. The significance of frontal intermittent rhythmic delta activity in children has not been studied. We analyzed the electrographic characteristics and clinical associations of pediatric frontal intermittent rhythmic delta activity. This pattern was rarely detected, occurring in 20 of 1500 electroencephalographic (EEG) studies. Patients' ages ranged between 1.5 and 17 years. Most patients were awake and showed no signs of acute encephalopathy when frontal intermittent rhythmic delta activity occurred. Half of the children were cognitively impaired, and half had a history of epilepsy. Epileptiform activity was present in 55% of the EEG recordings. However, frontal intermittent rhythmic delta activity was part of the epileptiform discharge in only a minority of cases. The duration of frontal intermittent rhythmic delta activity bursts was longer in the mentally retarded group. Most patients did not have structural brain pathology. None had deep midline or infratentorial lesions. In conclusion, frontal intermittent rhythmic delta activity is rare in children, is not associated with acute encephalopathy or with deep midline or infratentorial lesions, and tends to occur during wakefulness. The electrographic characteristics of frontal intermittent rhythmic delta activity appear to differ between cognitively normal and mentally retarded children. ( J Child Neurol 2003;18:525—529).
Acute Necrotizing Encephalopathy Associated With Parainfluenza Virus in a Caucasian Child
Authors: Mastroyianni Sotiria D. , Voudris Konstantinos A. , Katsarou Efstathia , Gionnis Dimitrios , Mavromatis Panagiotis , Vagiakou Eleni A. , Skardoutsou Angeliki
Published: June 2007

Acute necrotizing encephalopathy is a severe parainfectious disorder with a clear racial predilection for Oriental children living in the Far East. The prognosis was originally reported as grave; however, a mild form of the disease has recently been described. A case of parainfluenza virus—associated acute necrotizing encephalopathy in a Caucasian child with a mild clinical course and excellent prognosis is presented. In this patient, the initial clinical picture was not very impressive, and the diagnosis was delayed until the third week of the illness, when neuroimaging was performed. Two months later, clinical and neuroimaging findings had almost completely resolved. Suggested criteria for a benign prognosis, such as normal liver function and cerebrospinal fluid protein levels, asymmetric thalamic lesions, and no brainstem involvement, were relevant in the present case. An extended diagnostic work-up for metabolic, vascular, coagulation, and infectious diseases was negative apart from a seroconversion for parainfluenza virus. To our knowledge, this is the first reported case of acute necrotizing encephalopathy associated with parainfluenza virus infection. Acute necrotizing encephalopathy, especially in the mild form, might not be fully recognized and could be underdiagnosed in Europe, where the reported incidence of the syndrome is very low. ( J Child Neurol 2003;18:570—572).