bluuebunny/crossref_metadata_2025 huggingface dataset:
Novel Gene Silencer Pyrrole-Imidazole Polyamide Targeting Lectin-Like Oxidized Low-Density Lipoprotein Receptor-1 Attenuates Restenosis of the Artery After Injury
Authors: Yao En-Hui , Fukuda Noboru , Ueno Takahiro , Matsuda Hiroyuki , Matsumoto Koichi , Nagase Hiroki , Matsumoto Yoshiaki , Takasaka Ayako , Serie Kazuo , Sugiyama Hiroshi , Sawamura Tatsuya
Published: June 2008

Lectin-like oxidized low-density lipoprotein receptor-1 (LOX-1) is a membrane protein that can support the binding, internalization, and proteolytic degradation of oxidized low-density lipoprotein. The LOX-1 expression increases in the neointima after balloon injury. To develop an efficient compound to inhibit LOX-1, we designed and synthesized a novel gene silencer pyrrole-imidazole (PI) polyamide targeting the rat LOX-1 gene promoter (PI polyamide to LOX-1) to the activator protein-1 binding site. We examined the effects of PI polyamide to LOX-1 on the LOX-1 promoter activity, the expression of LOX-1 mRNA and protein, and neointimal hyperplasia of the rat carotid artery after balloon injury. PI polyamide to LOX-1 significantly inhibited the rat LOX-1 promoter activity and decreased the expression of LOX-1 mRNA and protein. After balloon injury of the arteries, PI polyamide to LOX-1 was incubated for 10 minutes. Fluorescein isothiocyanate–labeled PI polyamide was distributed to almost all of the nuclei in the injured artery. PI polyamide to LOX-1 (100 μg) significantly inhibited the neointimal thickening by 58%. PI polyamide preserved the re-endothelialization in the injured artery. PI polyamide significantly inhibited the expression of LOX-1, monocyte chemoattractant protein-1, intercellular adhesion molecule-1, and matrix metalloproteinase-9 mRNAs in the injured artery. The synthetic PI polyamide to LOX-1 decreased the expression of LOX-1 and inhibited neointimal hyperplasia after arterial injury. This novel gene silencer PI polyamide to LOX-1 is, therefore, considered to be a feasible agent for the treatment of in-stent restenosis.
Between Science and Religion: An Astrological Interpretation of the Asian Tsunami in India
Authors: Yongjia Liang
Published: June 2008

Astrology plays an important role in Indian social life. Indian astrologers' claim to have accurately predicted the 2004 Indian Ocean Tsunami, or the Asian Tsunami, was an effort to legitimize astrology as a full science. This effort demonstrates a difficulty in knowledge categorization, for in India, astrology is neither classified as a science nor as a religion. This is a result of the idea of an Indian nation-state, which rests upon both science and religion as foundations, but at the expense of expelling astrology from religion for not being scientific. However, as astrology continues to be important in India, the astrological interpretation of the Indian Ocean Tsunami drew substantial public attention. Astrology's significant presence in Indian society shows the role of a mature civil society in India as well.
Global Rise of Suicide Terrorism: An Overview
Authors: Hassan Riaz
Published: June 2008

Suicide attacks are the targeted use of self-destructing humans against a perceived enemy for political ends. After reviewing terrorism and suicide terrorism trends between 1980 and 2003 the paper shows that suicide attacks have increased dramatically between 2004 and 2005 and have become a global phenomenon. Three main sites of suicide terrorism namely, Iraq, Israel and Sri Lanka are examined in some detail including information about the main terrorist groups responsible for sponsoring suicide attacks and some profiles of individuals involved. The paper then examines the main sociological explanations of suicide attacks, including a description of the strategic logic behind these atrocities, and the notion of life being a weapon. It concludes with an overview of the recommendations emerging from studies that seek to prevent suicide terrorism.
The Role of Government in Technology Transfer to SME Clusters in Indonesia: Micro-level Evidence from the Metalworking Industry Cluster in Tegal (Central Java)
Authors: Tambunan Tulus
Published: June 2008

It is evident everywhere that levels of productivity are higher in large enterprises (LEs) and foreign-owned enterprises than in small and medium enterprises (SMEs), partly because they have higher levels of technology capacity. Thus increasing the productivity of SMEs might be facilitated through improved knowledge or technology. The literature on the development of SME clusters in developing countries argues that clusters are an effective means for technology transfer to SMEs and that government can play as the main source of technology transfer to the clusters, especially in regions where production linkages between LEs and SMEs are not yet well developed. This study indeed shows that in Indonesia government agencies are currently the largest providers of training and similar assistance. However, these programmes are marred by a low level of coverage, lack of effective evaluation and assessment, and a supply rather than demand orientation. The case study of the Tegal metalworking industry also shows that the most important channels for the transfer of technology to SME clusters are not only government agencies but also sub-contracting arrangements with LEs.
Theorising the UN Convention on the Rights of the Child within Canadian Post-Secondary Education: A Grounded Theory Approach
Authors: Mitchell Richard , McCusker Shawna
Published: June 2008

The following exploratory study was conducted in 2005 within Canadian post-secondary institutions for those intending to gain employment within public education for children and related professions. Data are comprised from thirteen interviews and draw upon students and educators from education, child health, and child and youth studies programmes. The researchers adopted a qualitative, grounded theory methodology to analyze documentary themes and those that emerged during theoretical sampling. Although there is variance of opinion, the majority confirm there is a limited theoretical appreciation of the Convention, and of concepts related to 'childhood' with a resultant lack of knowledge within post-secondary education. Interview findings were corroborated by policy analyses and data from non-governmental surveys, the Concluding Observations from the UN Committee on the Rights of the Child, and a contemporaneous parliamentary review undertaken by Canada's Standing Senate Committee on Human Rights. A number of implications stemming from the ongoing violation of international human rights law in Canada are discussed as well as directions for future research.
New Zealand Children and Young People's Perspectives on Citizenship
Authors: Smith Anne , Gollop Megan , Taylor Nicola
Published: June 2008

The perspectives of 66 children and young people (aged 8/9 and 14/15 years) on their understanding of citizenship issues were obtained through focus group discussions in New Zealand schools. The children reported on their rights and responsibilities in the everyday contexts of home, school and community. An imaginary country exercise was also utilized to compare children's responses with their current lives in New Zealand. Children generally conceived of the new land as safer, happier and healthier, with greater freedoms and respect than they currently enjoyed.
"It's Like Mental Torture": Participation and Mental Health Services
Authors: LeFrançois Brenda
Published: June 2008

This article discusses findings from an ethnographic research study looking at the ways in which children's participation rights are incorporated within an adolescent mental health inpatient unit. The practitioners working within this setting have reinterpreted the concept of 'participation' to suit a coercive agenda associated with the authoritarian medical model of treatment used within child psychiatry. Indeed, the use of the term 'participation' has become a tool to enforce children's compliance with adult (and practitioner) determined treatment plans. For the most part, the barriers to according children's participation rights within this setting relate to the institution's and practitioners' protectionist stance vis-à-vis children who are deemed informally to be both vulnerable and incompetent.
'Me and the 5 P's': Negotiating Rights-Based Critical Disabilities Studies and Social Inclusion
Authors: Moore Shannon , Davis John , Melchior Luke
Published: June 2008

This paper presents an integration of insights from critical disabilities studies, child rights and social exclusion in theory and practice and is conceptualized from two keynote presentations given by Dr. John Davis and Mr. Luke Melchior at Investment and Citizenship Towards a Transdisciplinary Dialogue in Child Rights, at Brock University Canada. In addition, the lived experience of disability is at the centre of this discussion rather than being the object of inquiry, providing a nuanced experiential perspective. The aim of this collaboration is to emphasise that young peoples' rights from the perspective of critical disabilities studies may be informed by an analysis of power relations and the five principles of protection, prevention, provision, participation, and perception (5 P's).
An Assessment of Errors in the Simulation of Atmospheric Interannual Variability in Uncoupled AGCM Simulations
Authors: Kumar Arun , Zhang Qin , Schemm J-K. E. , L’Heureux Michelle , Seo K-H.
Published: June 2008

For the uncoupled atmospheric general circulation model (AGCM) simulations, the quantification of errors due to the lack of coupled ocean–atmospheric evolution on the characteristics of the atmospheric interannual variability is important for various reasons including the following: 1) AGCM simulations forced with specified SSTs continue to be used for understanding atmospheric interannual variability and 2) there is a vast knowledge base quantifying the global atmospheric influence of tropical SSTs that traditionally has relied on the analysis of AGCM-alone simulations. To put such results and analysis in a proper context, it is essential to document errors that may result from the lack of a coupled ocean–atmosphere evolution in the AGCM-alone integrations.

Analysis is based on comparison of tier-two (or uncoupled) and coupled hindcasts for the 1982–2005 period, and interannual variability for the December–February (DJF) seasonal mean is analyzed. Results indicate that for the seasonal mean variability, and for the DJF seasonal mean, atmospheric interannual variability between coupled and uncoupled simulations is similar. This conclusion is drawn from the analysis of interannual variability of rainfall and 200-mb heights and includes analysis of SST-forced interannual variability, analysis of El Niño and La Niña composites, and a comparison of hindcast skill between tier-two and coupled hindcasts.
Response of Regional Sea Level to Atmospheric Pressure Loading in a Climate Change Scenario
Authors: Stammer Detlef , Hüttemann Sören
Published: June 2008

The regional response of the global ocean to low-frequency changes in atmospheric pressure loading, ηib, is analyzed as it occurs in the Max Planck Institute for Meteorology (MPI-M) coupled ocean–atmosphere climate model in response to increased atmospheric CO2 concentrations. Results suggest that long-term changes in ηib can lead to increases in high-latitude sea level by up to 5 and 10 cm, respectively, after doubling and quadrupling the atmospheric CO2 content. At low latitudes, sea level will decrease simultaneously between 2 and 4 cm through the combined effects of changes in the atmospheric circulation and through the increase of its moisture content. In subpolar regions, associated rates of sea level increase are of the order of 0.4–0.6 mm yr−1 for quadrupled atmospheric CO2 concentrations, while in mid- and low latitudes, sea level will decrease at a rate of 0.2 mm yr−1. Differences between doubling and quadrupling CO2 concentrations indicate regionally dependent nonlinearities in the changing climate system. The analysis suggests that in some regions (including the coasts of northern Europe) low-frequency ηib changes could be as large as 10%–20% of a global sea level increase anticipated over the next 100 yr. While not being a dominant effect, amplitudes of long-period ηib changes are large enough to be included in future estimates of climate-related regional sea level change. Increasing the vertically integrated atmospheric CO2 content by 4 and 12 kg m−2 (on global average), in response to doubling and quadrupling atmospheric CO2 concentrations, suggests associated reductions of global sea level by 0.6 and 1.7 cm, respectively. The differences between two different model solutions are significant, especially in the Southern Ocean, where they show significantly different atmospheric mass and pressure distributions, and at low latitudes, where differences resemble the contribution of increased moisture content added to the inverted-barometer (IB) effect in the MPI-M solution.
North American Droughts in ERA-40 Global and NCEP North American Regional Reanalyses: A Palmer Drought Severity Index Perspective
Authors: Karnauskas Kristopher B. , Ruiz-Barradas Alfredo , Nigam Sumant , Busalacchi Antonio J.
Published: June 2008

The Palmer drought severity index (PDSI) monitors meteorological and surface hydrological parameters to represent the severity of drought conditions. PDSI datasets are developed for the NCEP North American Regional Reanalysis (NARR) and the 40-yr European Centre for Medium-Range Weather Forecasts (ECMWF) Re-Analysis (ERA-40) to facilitate North American drought research with these datasets. The drought index calculation, in particular, allows diagnostic assessment of the relative contributions of various surface water balance terms in generation of drought conditions by selectively holding these terms to their climatological value in PDSI computations. The length of the diagnosed PDSI permits analysis of subdecadal time-scale variability, such as ENSO, whose influence on North American drought evolution is investigated. ENSO’s considerable drought impact is potentially predictable, especially in the southern half of the United States.
Response of Thermohaline Circulation to Freshwater Forcing under Present-Day and LGM Conditions
Authors: Hu Aixue , Otto-Bliesner Bette L. , Meehl Gerald A. , Han Weiqing , Morrill Carrie , Brady Esther C. , Briegleb Bruce
Published: June 2008

Responses of the thermohaline circulation (THC) to freshwater forcing (hosing) in the subpolar North Atlantic Ocean under present-day and the last glacial maximum (LGM) conditions are investigated using the National Center for Atmospheric Research Community Climate System Model versions 2 and 3. Three sets of simulations are analyzed, with each set including a control run and a freshwater hosing run. The first two sets are under present-day conditions with an open and closed Bering Strait. The third one is under LGM conditions, which has a closed Bering Strait. Results show that the THC nearly collapses in all three hosing runs when the freshwater forcing is turned on. The full recovery of the THC, however, is at least a century earlier in the open Bering Strait run than the closed Bering Strait and LGM runs. This is because the excessive freshwater is diverged almost equally toward north and south from the subpolar North Atlantic when the Bering Strait is open. A significant portion of the freshwater flowing northward into the Arctic exits into the North Pacific via a reversed Bering Strait Throughflow, which accelerates the THC recovery. When the Bering Strait is closed, this Arctic to Pacific transport is absent and freshwater can only be removed through the southern end of the North Atlantic. Together with the surface freshwater excess due to precipitation, evaporation, river runoff, and melting ice in the closed Bering Strait experiments after the hosing, the removal of the excessive freshwater takes longer, and this slows the recovery of the THC. Although the background conditions are quite different between the present-day closed Bering Strait run and the LGM run, the THC responds to the freshwater forcing added in the North Atlantic in a very similar manner.
Is the Upper Ocean Warming? Comparisons of 50-Year Trends from Different Analyses*
Authors: Carson Mark , Harrison D. E.
Published: June 2008

There is great interest in World Ocean temperature trends, yet the historical global ocean database has very uneven coverage in space and time. Previous work on 50-yr upper ocean temperature trends from the NOAA ocean data archive is extended here. Trends at depths from 50 to 1000 m are examined, based on observations gridded over larger regions than in the earlier study. Despite the use of larger grid boxes, most of the ocean does not have significant 50-yr trends at the 90% confidence level (CL). In fact only 30% of the ocean at 50 m has 90% CL trends, and the percentage decreases significantly with increasing depth. As noted in the previous study, there is much spatial structure in 50-yr trends, with areas of strong warming and strong cooling. These trend results are compared with trends calculated from data interpolated to standard levels and from a highly horizontally interpolated version of the dataset that has been used in previous heat content trend studies. The regional trend results can differ substantially, even in the areas with statistically significant trends. Trends based on the more interpolated analyses show more warming. Together with major temporal and spatial sampling limitations, the previously described strong interdecadal and spatial variability of trends makes it very difficult to formally estimate uncertainty in World Ocean averages, but these results suggest that upper ocean heat content integrals and integral trends may be substantially more uncertain than has yet been acknowledged. Further exploration of uncertainties is needed.
Reconsideration of the True versus Apparent Arctic Oscillation
Authors: Itoh Hisanori
Published: June 2008

The physical reality of the Arctic Oscillation (AO; or northern annular mode) is considered. The data used are mainly the monthly mean sea level pressure (SLP).

A schematic figure is first presented to illustrate the relationship between the North Atlantic Oscillation (NAO)–Pacific–North American Oscillation (PNA) system and the AO–negative correlation mode between the Atlantic and the Pacific (AO–NCM) system. Although the NAO–PNA (apparent AO–NCM) and true AO–NCM systems give rise to the same EOFs, the probability density functions for the time coefficients of the two leading modes are different. Therefore, the discrimination of the two systems is possible. Several pieces of evidence indicate that, in the real world, the NAO–PNA and the AO–NCM are located on almost the same plane in phase space. This means that the NAO–PNA and AO–NCM systems have the same variations on the plane in common, implying that when the NAO–PNA system is real, the AO–NCM is unlikely to be real. Simple independent component analysis is carried out to distinguish between the true and apparent AO–NCM systems, indicating that the NAO and PNA are independent oscillations, that is, true ones.

The analysis is extended to the winter mean SLP field, for which the EOF shows the NAO–PNA but not the AO–NCM. This may be due to the fact that the winter mean NAO and PNA patterns have little spatial correlation. Calculations using randomly selected samples also indicate that when the NAO and PNA patterns have little spatial correlation, the AO never appears as EOF1.

All the preceding results show that almost all characteristics of the AO–NCM can be explained from those of the NAO–PNA. Hence it is concluded that the AO, which is extracted by EOF analysis from the temporarily independent but spatially overlapping variations of the NAO and PNA, is almost apparent.
Cirrus Microphysical Properties and Air Motion Statistics Using Cloud Radar Doppler Moments. Part II: Climatology
Authors: Deng Min , Mace Gerald G.
Published: June 2008

The algorithm described in Part I has been applied to the millimeter cloud radar observations from January 1999 to December 2005 at the Atmospheric Radiation Measurement Program (ARM) Southern Great Plains (SGP) and Tropical Western Pacific (including Manus and Nauru) sites. Approximately 10 000 cirrus hours from each of these sites were analyzed. Retrieved cloud properties including condensed mass, particle size, optical depth, and in-cloud vertical air motions were analyzed in terms of their geographical, seasonal, and diurnal variations. The analysis shows that tropical ice clouds observed by millimeter radar are very different from ice clouds at SGP, with the tropical clouds having slightly larger particle sizes and greater ice masses and being more likely to be associated with ascending air motions, in addition to being colder and higher in altitude. A positive residual of derived in-cloud air motion found in the tropical data likely provides evidence for lofting of air into the tropopause transition layer as a result of radiative heating. The midlatitude cirrus demonstrate strong seasonal variations with more frequent, thicker clouds occurring during the summer than during the winter. Very subtle seasonal variations are found for tropical ice clouds, and evidence is presented that cirrus properties vary interannually and are correlated with El Niño oscillations. In addition, it is found that tropical cirrus demonstrate a stronger diurnal cycle than cirrus of the midlatitudes, with the in-cloud updrafts peaking in the early afternoon.
To Study or Not to Study Religion and Society
Authors: Hjelm Titus
Published: June 2008

In the work of many of the classics of sociology, religion plays a major role. In post-World War II sociology, however, the study of religion gradually ended up in a marginal position. This article discusses the status of the sociological study of religion in Finland from the post-war period to the present. The focus in the article is on the institutional evolution of the field and the influence of that evolution on the role of the sociology of religion within Finnish sociology. Finnish sociology of religion is divided into three categories: studies carried out in sociology departments, sociological studies carried out within religious studies and church and social studies. The article examines the development and characteristics of each of these disciplines as well as the consequences of institutional scattering for the sociology of religion. The aversion of religion within sociology, the focus on folklore in religious studies and the public identification of church sociology with theology are identified as the reasons for the marginal position of the sociology of religion in Finland. The article closes with a review of recent studies in the sociology of religion and the `new rise' of the field due to the rise in the societal significance of religion.
Market formation and governance in international financial markets: The case of OTC derivatives¹
Authors: Morgan Glenn
Published: June 2008

How are markets developed and governed? The article reviews four perspectives on the formation and development of markets and market governance — the political perspective (Fligstein), the network perspective (White), the framing perspective (Callon) and the soft law/expert power perspective (Slaughter). It then utilizes these perspectives in order to understand the development of the market for over-the-counter (OTC) derivatives. It finds that this market was stabilized and governed through the operation of all of these features — power, networks, framing and expert power. The International Swaps and Dealers Association (ISDA) to which all market participants belong has played a central role in this process. It has created a Master Agreement that is the common basis to all OTC derivative contracts across the world and seeks to overcome potentially problematic areas such as netting and collateral. It brings together all market participants and uses their expertise within its committees to develop new rules. However, the article shows that ISDA as a private international association cannot sustain governance by itself. It has to influence national governments in order to ensure that its Master Agreement is enforceable even though the agreement favours ISDA members above other actors. This has the potential for political controversy though mainly governments deal with it as an issue of technical expertise. At the public, international level, the central bankers forum in Basle is also concerned with the oversight of the sector and have issued a number of warnings about potential sources of instability arising from the derivatives market. The article illustrates that the market formation process is highly complex and that the governance of the market is multi-levelled and precarious. The research raises questions about how other complex markets are being created and how their governance structures are determined.
The resilience of `institutionalized capitalism': Managing managers under `shareholder capitalism' and `managerial capitalism'
Authors: Morris Jonathan , Hassard John , McCann Leo
Published: June 2008

This article identifies two, nation-wide, forms of governance or organizing capital,`shareholder' capitalism and `managerial' capitalism, epitomized by the USA and Japan respectively. Furthermore, it identifies the implications for managers in these systems. The article argues that both varieties of capitalism have been forced to modify, due to pressures from globalization. Shareholder capitalism in the USA has been intensified with an even greater emphasis on `shareholder value'. In Japan, meanwhile, poor national economic performance has led to pressures upon managerial capitalism to take on features more akin to the shareholder variety. Such pressures have been transmitted to managers with a more intensified work regime. However, many of the HRM features of the Japanese model remain, despite these pressures.
Simulation and Games Forum
Authors: Fletcher Jerry L.
Published: June 2008

The purpose of the "Simulation and Games Forum" is to provide a medium for informal and informative discussion about games and simulation. The forum may contain comments on papers previously published or may initiate a topic for discussion, unrelated to previous publications in SIMULATION & GAMES. This section is intended to generate debate and to lay out issues on which readers might differ. Communications will be published in the issue following receipt. They should be limited to 700 words and sent in letter form addressed to the Editors.
Simulation and Games Forum
Authors: Taylor John L. , Walford Rex , Kidd John B. , Bonoma Thomas V.
Published: June 2008

The purpose of the "Simulation and Games Forum" is to provide a medium for informal and informative discussion about games and simulation. The forum may contain comments on papers previously published; initiate a topic for discussion, unrelated to previous publications in SIMULATION AND GAMES; or give a prospectus of research projects, prior to their initiation, so that researchers with similar interests may exchange ideas and possibly arrange to share data. This section is intended to generate debate, to lay out issues on which readers might differ, and to foster communication among readers. Submissions will be published in the issue following receipt. They should be limited to 700 words (approximately 21/2 pages, typed double-space) and sent in letter form addressed to the Editors.
Simulation and Games Forum
Authors: Cobb Anthony T.
Published: June 2008

The purpose of the "Simulation and Games Forum" is to provide a medium for informal and informative discussion about games and simulation. The forum may contain comments on papers previously published; initiate a topic for discussion, unrelated to previous publications in SIMULATION AND GAMES; or give a prospectus of research projects, prior to their initiation, so that researchers with similar interests may exchange ideas and possibly arrange to share data. This section is intended to generate debate, to lay out issues on which readers might differ, and to foster communication among readers. Submissions will be published in the issue following receipt. They should be limited to 700 words (approximately 21/2 pages, typed double-space) and sent in letter form addressed to the Editors.
Evaluation of the Treatment Tradeoff Method in Rectal Cancer Patients: Is Surgery Preference Related to Outcome Utilities?
Authors: Bossema Ercolie R. , Marijnen Corrie A. M. , Baas-Thijssen Monique C. M. , van de Velde Cock J. H. , Stiggelbout Anne M.
Published: June 2008

Background. The treatment tradeoff method (TTM) has been developed specifically for decision making at the level of the individual patient. The task is tailored to the clinical decision problem at hand and may therefore be more relevant to patients than methods of outcome valuation. Despite its wide use in oncology research, few methodological studies regarding validity have been conducted. Objective and Methods. The present study evaluates the validity of the TTM in rectal cancer patients who had undergone either 1 of 2 surgery types: 1 requiring a permanent stoma (stoma group) and 1 involving a postoperative risk of fecal incontinence (no-stoma group). The authors relate the surgery preference scores to the utilities of the 2 main surgery outcome states as well as to their utility difference. Results. Surgery preference was more strongly associated with the utility difference (r > 0.54 in the total patient group) than with the utilities of the surgery outcome states per se (r < 0.44 in the total patient group). In the stoma group, surgery preference was especially related to the utility of incontinence and in the no-stoma group especially to the utility of a permanent stoma. Conclusions. Patients indeed use their valuations of treatment outcomes states, especially those they are less familiar with, in determining their preference for one treatment over another. In clinical practice, the TTM may be used to obtain an indication of the treatment preference of an individual patient and may also be helpful to detect patients' motives to choose one treatment over another.
Proton Magnetic Resonance Spectroscopic Observations of Epilepsia Partialis Continua in Children
Authors: Park Yong D. , Allison Jerry D. , Weiss Kenneth L. , Smith Joseph R. , Lee Mark R. , King Don W.
Published: June 2008

We performed magnetic resonance spectroscopy in three pediatric patients (two boys and one girl, ages 11 to 17 years) with epilepsia partialis continua. Single-voxel proton magnetic resonance spectroscopy was performed on each patient. Data were acquired from voxels of 4 or 8 cm3 from the affected hemisphere and from contralateral homologous regions. The spectral peaks of several metabolites (N-acetyl-aspartate, choline, creatine, and lactate) were measured. Neuropathologic findings revealed Rasmussen's syndrome in two children and gliosis in one. We observed increased lactate-to-creatine ratios and reduced N-acetyl-aspartate-to-creatine ratios in the affected hemispheres in all three children with epilepsia partialis continua. These data support previous reports. The largest increase in the lactate-to-creatine ratio was detected in a patient with Rasmussen's syndrome and ongoing epilepsia partialis continua at the time of measurement. The other two patients had an increase in the lactate-to-creatine ratio and a decrease in the N-acetyl-aspartate-to-creatine ratio in the affected area. The increased lactate-to-creatine ratio was associated with recurrent focal seizures from different underlying pathologies. (J Child Neurol 2000;15:729-733).
Progression in a Case of Kearns-Sayre Syndrome
Authors: Ishikawa Yukitoshi , Goto Yu-ichi , Ishikawa Yuka , Minami Ryoji
Published: June 2008

The quantitative relationship between deleted mitochondrial DNA and the clinical course of patients with Kearns-Sayre syndrome is poorly understood. We investigated this point using tissue samples obtained at age 10 years when the patient was diagnosed as Kearns-Sayre syndrome and at age 20 years when he died of disseminated intravascular coagulation. By long polymerase chain reaction, a shortened mitochondrial genome (8.8 kb; normal, 16.6 kb) was detected in the patient. By quantitative competitive polymerase chain reaction, the percentage of deletion-carrying mitochondrial DNA was not increased as expected and did not differ significantly by tissue type or sampling time or correlate with clinical course. Although we could not demonstrate that the amounts of wild-type mitochondrial DNA decreased with accelerating progression, it was emphasized that such a reduction of mitochondrial DNA in various tissues, including those of the central nervous system, could play a significant pathogenetic role, since only wild-type mitochondrial DNA is functional in patients with large-scale deletions of mitochondrial DNA. ( J Child Neurol 2000;15:750-755).
Endocrine Disorders in Two Sisters Affected by MELAS Syndrome
Authors: Balestri Paolo , Grosso Salvatore
Published: June 2008

A variety of endocrine and metabolic defects, including hypothalamopituitary hypofunction and diabetes mellitus, has been reported in association with mitochondrial disorders. We describe two sisters affected by mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome in whom DNA analysis showed an A→G transition at the 3243rd nucleotide position on the transfer RNA Leu(UUR) gene with 65% and 45% of mutant-type mitochondrial DNA present in the blood cells of the younger and the older sister, respectively. The younger sister had severe involvement of the central nervous system with mental retardation, epilepsia partialis continua, and strokelike episodes. Endocrine investigations showed an extensive neuroendocrine dysfunction with growth hormone deficiency, hypothalamopituitary hypothyroidism, prepubertal gonadotropin levels, and absence of any secondary sexual characteristics at the age of 12 6/12 years. The neurologically normal older sister was affected by diabetes mellitus and had normal hypothalamopituitary function. Our report confirms that the endocrine system can be affected differently by the same mitochondrial DNA mutation, depending on the heteroplasmia phenomenon. A complete endocrine evaluation must be performed in patients affected by mitochondrial disease and the existence of a mitochondrial disorder should be taken into account in patients with endocrine abnormalities, even if neuromuscular signs are lacking. ( J Child Neurol 2000;15:755-758).
G8363A Mutation in the Mitochondrial DNA Transfer Ribonucleic Acid Lys Gene: Another Cause of Leigh Syndrome
Authors: Shtilbans Alexander , Shanske Sara , Goodman Svetlana , Sue Carolyn M. , Bruno Claudio , Johnson Theodore L. , Lava Neil S. , Waheed Nasir , DiMauro Salvatore
Published: June 2008

We identified a G→A transition at nt-8363 in the mitochondrial DNA transfer ribonucleic acid Lys gene in blood and muscle from a 13-month-old girl who had clinical and neuroradiologic evidence of Leigh syndrome and died at age 27 months. The mutation was less abundant in the same tissues from the patient's mother, who developed myoclonus epilepsy with ragged red fibers (MERRF) in her late 20s. In both mother and daughter, muscle histochemistry showed ragged red and cytochrome c oxidase-negative fibers and biochemical analysis showed partial defects of multiple respiratory-chain enzymes. A maternal half-sister of the proband had died at 2.5 years of age from neuropathologically proven Leigh syndrome. The G8363A mutation, which previously had been associated with cardiomyopathy and hearing loss, MERRF, and multiple lipomas, also should be included in the differential diagnosis of maternally inherited Leigh syndrome. ( J Child Neurol 2000;15:759-761).
Purple Glove Syndrome Caused by Oral Administration of Phenytoin
Authors: Yoshikawa Hideto , Abe Tokinari , Oda Yoshihiko
Published: June 2008

A severely handicapped boy had been treated with phenytoin and his seizures were controlled well. At 10 years of age, a pharmacy gave about 1000 mg of phenytoin instead of the prescribed 100 mg of the drug per day. Several hours after the initial administration, the patient became drowsy and his hands and feet turned dark purple with marked swelling. Four days later, his mother stopped administering the phenytoin to him and took him to hospital. After fluid therapy was started, the swelling and discoloration of both his hands and feet improved gradually and disappeared 11 days after drug discontinuation. Purple glove syndrome is defined as the edema, discoloration, and pain occurring in the distal limb where intravenous phenytoin has been administered. This might be the first report of purple glove syndrome caused by the oral administration of a large quantity of phenytoin. ( J Child Neurol 2000;15:762).
Central Auditory Processing and Attention Deficit Disorders
Authors: Gascon Generoso G. , Johnson Ron , Burd Larry
Published: June 2008

Nineteen children who met criteria for attention deficit disorder (ADD) received neurodevelopmental attention testing and central auditory processing (CAP) tests. The CAP tests consisted of the Staggered Spondaic Word (SSW) test and the Willeford battery of tests. Teacher and parent questionnaires (originally devised at the Learning Disabilities Clinic, The Children's Hospital, Boston) were obtained. There was a very high concordance between the questionnaires and the CAP tests, but a low concordance between the neurodevelopmental measures and the questionnaires. After treatment with central nervous system stimulants, there was again a high concordance between teacher/parent reports and CAP measures, and less than 50% concordance between neurodevelopmental measures and teacher/parent observations. We conclude that the SSW and Willeford battery are sensitive performance indicators of attention disorder. Furthermore, these tests are more useful in titrating response to CNS stimulants than neurodevelopmental assessments and provide objective support for the subjective teacher and parent observations. (J Child Neurol 1986;1:27-33)
Choroid Plexus Papilloma in a 4-Month-Old Child: A Case Report
Authors: Ivan Leslie P. , Martin David J. , Mallya Kasargod B. , Schneider Elizabeth
Published: June 2008

We report a case of choroid plexus papilloma arising from the right lateral ventricle in a 4-month-old infant. Because of intermittent vomiting, abdominal pain, and diarrhea, the child was initially treated with intravenous rehydration, which aggravated the symptoms, and a bulging fontanel developed. Before a planned lumbar puncture, cranial ultrasound was performed, which disclosed a choroid plexus papilloma. The tumor was removed surgically, and the child made an uneventful recovery. We discuss the diagnostic value of cranial ultrasound compared to computed tomographic (CT) scan and angiography. (J Child Neurol 1986:1;53-55)
Spongy Degeneration of the Central Nervous System (Van Bogaert-Bertrand Disease): Report of a Case in a Saudi Family
Authors: Mahdi Awad H. , Elidirssy A.T.H. , Wright Eric A.
Published: June 2008

Spongy degeneration of the central nervous system in infancy (Van Bogaert-Bertrand disease) is a disorder usually thought to occur in Ashkenazi Jewish infants. We report a case occurring in an Arab infant of consanguineous parents. (J Child Neurol 1986;1:61-63)
Dandy-Walker Malformation Associated With Occipital Meningocele and Cardiac Anomalies: A Rare Complex Embryologic Defect
Authors: Sautreaux Jean Louis , Giroud Maurice , Dauvergne Michel , Nivelon Jean Louis , Thierry André
Published: June 2008

The authors present the second case in the literature of Dandy-Walker malformation associated with occipital meningocele and congenital cardiac defect. This association suggests that the time of origin of this posterior fossa malformation is before the sixth postconception week; however, the pathogenesis remains unsettled. (J Child Neurol 1986;1:64-66)
Magnetic Resonance Imaging in Moyamoya Disease
Authors: Rolak Loren A. , Rokey Roxann
Published: June 2008

Magnetic resonance imaging of the brain in a patient with moyamoya disease demonstrated multiple scattered white matter lesions, but was not sufficiently specific to confirm the diagnosis without other techniques. (J Child Neurol 1986;1:67-70)
Ischemic Strokes in Children
Authors: Dusser Anne , Goutières Françoise , Aicardi Jean
Published: June 2008

Of 44 infants and children (neonates excluded) with ischemic strokes of arterial origin documented by CT scan and/or cerebral angiography, idiopathic strokes, occurring in 22 cases, accounted for half the total. Eight of these patients had basal ganglia and/or capsular infarcts without cortical involvement. The outcome in the idiopathic group was favorable: after an average follow-up duration of 48 months, no child had recurrence, two children developed secondary epilepsy, and only two were severely mentally retarded. However, residual dystonia and dyskinesia constituted an incapacitating handicap, having been observed in 14 children of the idiopathic group and in 18 of the whole series. It may be concluded from the present study that the long-term prognosis of ischemic strokes in children is excellent except for the risk of secondary evolutive dystonia in the absence of any detectable cause. (J Child Neurol 1986;1:131-136)
Marginal Glioneuronal Heterotopias in Nine Cases With and Without Cortical Abnormalities
Authors: Dambska M. , Wisniewski K.E. , Sher J.H.
Published: June 2008

Nine cases of marginal glioneuronal heterotopias over the cerebral cortex were reviewed from the morphological point of view. There were developmental disabilities in all cases except one (case 8), who was stillborn. All subjects died before 1 year of age except one (case 5). The common features of small glioneuronal heterotopias and abundant heterotopic glioneuronal proliferation are described. The correlation of glioneuronal heterotopias with polymicrogyria and other cortical malformations, as well as their appearance over a normal cortex, are described. The glioneuronal heterotopias are considered to be a separate type of malformation that could arise during the second half of intrauterine life. A breach of the neuropial border seems to be the most acceptable pathomechanism for our presented cases. Their morphological features indicate that damage to this barrier leads to involvement of glioneuronal heterotopias in fusion of opposite cortical convolutions. (J Child Neurol 1986;1:149-157)
A Diagnostic Approach to Developmental Specific Learning Disorders
Authors: Weinberg Warren A. , McLean Anne
Published: June 2008

Children and adolescents who are not doing well in school are often referred to physicians for evaluation. Many of these children have average or above-average intelligence but are academically frustrated, low achievers. Parents state that although they are convinced that their child is bright, he or she does not seem to learn, and does not behave acceptably in school and at home. Results of the usual pediatric and neurologic examinations are frequently unrevealing. In evaluating children who are doing poorly in school, the clinician must determine whether the child has a developmental specific learning disorder and whether primary affective illness, disturbed vigilance, or hyperactivity complicates the picture. Children who are intelligent but failing in school frequently manifest either one or several of these conditions. Although there are reasonably well-established criteria for affective illness in children, clinically useful criteria for evaluating the variety of childhood learning problems have not been available. We present criteria for the diagnosis of developmental specific learning disorders and briefly mention some aspects of management and treatment. We also describe a primary disorder of vigilance that becomes manifest in school, worsening the learning and performance process, which is further worsened by affective illness. (J Child Neurol 1986;2:158-172).
Review Article: Anesthesia for Pediatric Neurological and Neuromuscular Diseases
Authors: McLeod M.E. , Creighton R.E.
Published: June 2008

Children with neurological and neuromuscular diseases often present anesthetic problems in the perioperative period. The anesthetic technique can play a significant role in altering the state of the brain during neurosurgical procedures through effects on the cerebral circulation and metabolism. Pre-existing neuromuscular disease may also have specific anesthetic implications such as cardiorespiratory involvement (eg, myotonia dystrophica), the potential for drug interactions (eg, myasthenia gravis) or abnormal responses to commonly used drugs (eg, malignant hyperthermia). In this review, the perioperative anesthetic considerations in a number of common neurological and neuromuscular conditions in the pediatric patient are discussed. ( J Child Neurol 1986;1:189-197)
Transient Post-traumatic Cortical Blindness: Brief v Prolonged Syndromes in Childhood
Authors: Kaye Edward M. , Herskowitz Joel
Published: June 2008

Six children who sustained head trauma of varying degrees experienced self-limited cortical blindness—complete bilateral visual loss associated with normal pupillary responses to light. In five cases associated with relatively minor head trauma, full visual function returned within 24 hours. In the other case, associated with parietal and occipital contusions, the period of complete blindness was prolonged, lasting 1 week, followed by a striking recovery over an additional week. A bilateral inferior altitudinal visual field deficit has persisted over 6 years, with emergence of a complicated migraine syndrome. Sequential computed tomographic (CT) scans over a 4-year period demonstrate bioccipital and right parietal injury, correlating clinical and anatomic findings. This case is the first description of prolonged transient post-traumatic cortical blindness in a child with CT correlation. It underscores the clinical, radiographic, and pathophysiologic differences between syndromes of brief and prolonged transient post-traumatic cortical blindness in childhood. ( J Child Neurol 1986;1:206-210).
Review Article: Electromyography in Infants and Children
Authors: Jablecki Charles K.
Published: June 2008

Electromyography (EMG) is of proven value in the diagnosis of acute and chronic neuromuscular diseases in infants and children. When this technique is combined with nerve conduction studies, including repetitive nerve stimulation studies, it is often possible upon completion of the studies to identify the disorder as one of nerve, neuromuscular junction, or muscle. The purpose of this article is to review the principles and techniques of EMG in infants and children and to describe the EMG findings in several neuromuscular disorders. ( J Child Neurol 1986;1:297-318)