bluuebunny/crossref_metadata_2025 huggingface dataset:
Reporting Inclusivity: The Northern Ireland Women's Coalition, the News Media and the North Ireland Peace Process
Authors: Spencer Graham
Published: January 2017

This article is an investigation into how the news media dealt with the politics and ideas of the Northern Ireland Women's Coalition in the build-up to the Good Friday Agreement of 1998. Drawing on interview material carried out with representatives of the NIWC who were actively involved within the peace process at that time, it assesses the responsiveness of news towards positions taken by the NIWC and considers the implications of reporting in relation to NIWC discourse about inclusiveness and equality. This article brings to light a general lack of interest in party ideas by the news media, which is symptomatic of journalism obsessed with the competitiveness and negativity of male dominated politics.
Occupational Change and Social Polarisation in Ireland: Further Evidence
Authors: Breathnach Proinnsias
Published: January 2017

This paper extends to the year 2002 that section of an earlier paper (Breathnach 2002b) which considered whether the pattern of occupational change occurring in Ireland during the period 1991–1996 indicated the operation of processes of social polarisation. The occupational categories contained in the Census of Population were recast in order to create a set of broad groups which facilitated analysis from a social polarisation perspective. This analysis demonstrated strong growth, on the one hand, in the numbers of employers & managers and professional & technical workers and, on the other, in certain unskilled occupational groups (personal services and retail sales) – the latter following the allocation to these groups of the bulk of the greatly-expanded number of workers who failed to state their occupation in the 2002 census. Meanwhile, key middle-income groups, including blue-collar industrial, clerical and public service workers experienced contraction in their overall share of employment. These findings provide considerable support for the social polarisation hypothesis, albeit in the context of overall professionalisation of the occupational structure. Female workers have been to the fore in driving these processes of change, in that there has been above-average growth of female employment in those occupational categories at both ends of the occupational spectrum.
Patriarchs and Partners in Spousal Bequests in Ireland in 1951 and 2000: The Life Interest as a Dying Practice
Authors: Whitaker Teresa
Published: January 2017

The gendered nature of property ownership, succession and inheritance has always been a concern for feminists. In Ireland, the 1965 Succession Act effectively abolished gender as an issue in property ownership when it curbed testamentary freedom and granted a spouse a legal right to a proportion of his/her spouse's estate. This article compares spousal bequeathing practices in Irish society in 1951 and 2000 through a small sample of qualitatively analysed probated wills. The findings suggest that patriarchy has declined in transmissions between spouses and that there is an increasing tendency for marriage to be based on democratic principles and confluent love rather than on the prerogatives of property consolidation (Giddens 1992).
Is Contemporary Ulster Unionism in Crisis? Changes in Unionist Identity during the Northern Ireland Peace Process
Authors: White Andrew
Published: January 2017

This paper addresses the way in which the Northern Ireland Peace Process has impacted on unionist identity. In particular, it offers a critique of the three constituent philosophies of unionism – cultural unionism, liberal unionism and economic unionism – and suggests that a new form of unionism that reflects the altered polity of Northern Ireland must be constructed.
Against Unspoilt Authenticity: A Re-Appraisal of Helmuth Plessner's The Limits of Community (1924)
Authors: Hess Andreas
Published: January 2017

Community rhetoric has made a strange comeback. More than half a century after Arensberg and Kimball's study Family and Community in Ireland (1940) hardly a day passes without a politician, social scientist or public intellectual referring or appealing to some sense of community. However, what appears to be a genuine search for meaning and an attempt to make sense of social life also has its downsides – as Helmuth Plessner's classic study The Limits of Community (1924) reveals. This article reconstructs Plessner's main argument and locates Plessner's reflections in the context of the relevant international debates. With explicit reference to recent debates in Ireland the article warns against some of the non-reflective use of community rhetoric.
Thinking Critically about Social Capital
Authors: Farrell Clare
Published: January 2017

Although clearly sociological in origin, the concept of social capital has become popular in a number of academic disciplines, and prominent in discourses about tackling social inequality. It has managed to cross over into popular consciousness and the vocabulary of political leaders and it has been invoked as a sort of cure-all for an assortment of societal problems. Putnam (1998) has linked social capital to ‘better schools, safer streets, faster economic growth, more effective government, and even healthier and longer lives’. Given its rapid journey into political, policy and popular discourse, this paper seeks to critically explore the concept, and its application in research and social policy terms. It calls for a critical and thoughtful approach to the application of the concept, particularly in the context of addressing the problems faced by communities experiencing deprivation or disadvantage.
Social Capital in Disadvantaged Neighbourhoods: A Diversion from Needs or a Real Contribution to the Debate on Area-Based Regeneration?
Authors: Humphreys Eileen
Published: January 2017

The most enthusiastic proponents of social capital identify it as the key explanatory factor in economically prosperous and well-governed territorial communities. The tendency is to reduce the concept to ‘sense of community’ and voluntary association. Use of social capital as a policy tool, however, requires a clear understanding of what social capital is and a stronger evidence base to examine its association with positive outcomes. This paper presents an approach to measuring social capital in local territorial communities alongside selected findings from an empirical examination of social capital and its relationship to quality of life in disadvantaged urban neighbourhoods (Humphreys, 2005). Variations in levels of social capital in neighbourhoods are associated with both compositional and contextual conditions of place. Socio-economic characteristics of people including higher levels of education and home ownership are positively associated with social capital. Better conditions of place such as good quality local services and a developed community and social infrastructure are also positively associated with social capital. However, this does not mean that the problems of disadvantaged neighbourhoods can be attributed simply to deficits of social capital or certain types of social capital – i.e. bridging and linking social capital. I argue that a more sophisticated understanding is required of the conditions that shape social capital and that are shaped by it in specific spatial contexts, before considering the deployment of social capital as a policy tool.
Cosmopolitans versus the Locals: Community-Based Protest in the Age of Globalisation
Authors: Murray Michael
Published: January 2017

Processes of deterritorialisation and cosmopolitanism are, according to some authors, diminishing the influence of location on identity formation in the age of globalisation. This paper disputes these claims by arguing that ‘locality’, as a social construction, remains a key determinant of identity, especially in relation to community-based protest. In doing so, it draws on a case study of members of a Dublin community who are resisting government plans to locate a municipal incinerator in close proximity to their neighbourhood. An alternative view is offered which employs Roudometof's (2005) concept of a continuum of ‘degrees of attachment’ and suggests that transnational forces/discourses are ‘critically filtered’ by members of the community in terms of the primacy of local concerns and meaning.
Investigating the classroom: Journeying from consuming to producing the scholarship of teaching and learning
Authors: Lowney Kathleen S.
Published: January 2017

This article discusses one faculty member’s journey from reading about others’ pedagogical research to her use of data from her own classes to create new scholarship of teaching and learning.
Unemployment and Imprisonment: Examining and Interpreting the Relationship in the Republic of Ireland
Authors: Cullagh Ciaran Mc
Published: January 2017

There is a tradition in the sociological study of punishment that emphasises the relationship between conditions in the economy, in particular the level of unemployment, and the numbers sent to prison. This paper examines this relationship in the Irish context using data from the period 1951 to 1988. It finds that the relationship only holds in the period from the late 1970s onwards. It suggests that an examination of why this relationship exists needs to look at the ‘vocabulary of motives’ used by key decision-makers in the criminal justice system, and in particular by the judiciary.
Labour Relations: Mothers and Hospital Procedure
Authors: Carr Patricia
Published: January 2017

This paper is an examination of how two groups of women, i.e. those who attend hospital ante-natal classes and those who attend ante-natal classes outside the hospital, cope with giving birth and with breastfeeding in an institutional setting. Using the concept of ‘rule’ developed by Anthony Giddens, it will be shown how women from both class settings actively drew on the ‘rules of childbirth’ that are learned in ante-natal classes, that these rules are enabling rather than constraining and that Giddens’ assertion that institutions do not just work ‘behind the backs’ of the social actors is thus justified. Feminist critiques of health care have argued that the unequal power relations that exist between medical personnel and pregnant women are based on gender, i.e. male doctors trying to control female bodies. However, I would argue that examining gender relations between doctors and patients is not enough. We must also examine the social context in which birth occurs and the resources which women bring to the hospital.
Ourselves Alone: Household Work Strategies in a Deprived Community
Authors: Leonard Madeleine
Published: January 2017

This paper is concerned with examining the nature of household work strategies in a low income estate in Belfast. It is based on a survey of one in four households within the estate and interviews with one hundred and twenty two school pupils from the area. The paper is divided into three parts. The first section assesses the extent to which male unemployment leads to a renegotiation of the domestic division of labour within the household. The second part of the paper relates to the presence or absence of consumer durables within the household and assesses whether this is connected to the employment status of household members. It examines whether the presence of consumer durables influences the time spent on household labour. The final part of the paper focuses on male involvement in non-routine household tasks.
Replaying the ‘Match’: Marriage Settlements in North Galway
Authors: Curtin Chris , Devereux Eoin , Shields Dan
Published: January 2017

In this paper, marriage settlement records from a north Galway legal practice are utilised to provide additional insights into the socio-legal aspects of the marriage practices of west of Ireland farmers. The significant new findings include: the complexity and variety of forms of property transfer; the wide range of actors and interests involved in the settlements; and the far from universal presence of the dowry. While the empirical base of the paper is limited, the findings are such as to question some of the taken-for-granted elements of farmer's marriage patterns as they are described in previous accounts.
Framing Nature: First Steps into the Wilderness for a Sociology of the Landscape
Authors: Bell Desmond
Published: January 2017

We give a sociological reading of the landscape as a formation not primarily of the natural world but of the social. Landscape is best seen as a terrain on which socially constructed and historically determined significatory practices are at work shaping our perception of nature. Accordingly we must attend to the media forms and practices through which landscape has been represented if we are to understand the inter-textual nature of our experience of nature. We explore in particular sociological attempts to understand tourist representations of the landscape. We engage with Urry's postmodern analysis of picturesque tourism and argue that a more historically informed analysis is needed that links representations of landscape to power relations in colonised societies.
Household Structure in Early Twentieth Century Ireland
Authors: Corrigan Carmel
Published: January 2017

This paper aims to provide a count of households by structural type in Ireland in the early twentieth century. In doing so it addresses some of the issues which have been raised in the debate on the structure of Irish households at that time. The question of the classification of households by structural type is also addressed and attempts made to clarify some of the more ambiguous definitions. Some commonly accepted and some modified definitions are then applied to a national sample of 2,495 households drawn from the 1911 Census of Ireland. The paper concludes that the nuclear or simple household was the dominant from of household in Ireland at that time and that while the extended and multiple household certainly existed, its prevalence has largely been overstated in previous writings.
A Study of Social Welfare and Law: The Recovery of Overpayments
Authors: Cousins Mel
Published: January 2017

This article argues that for many years the official practice in relation to the recovery of overpaid social welfare benefits did not coincide with the legal rules in this area, i.e. overpayments were assessed against claimants and repayment of money was sought where there was no legal obligation on the claimant to repay the benefit. The implications of this practice in two areas are discussed: first, why such a divergence between the legal rules and the official practice can arise in the area of social welfare administration, and secondly, the meaning of ‘the law’ from a sociological point of view in the light of this case study.
Feminist Research Methodologies — A Separate Paradigm? Notes for a Debate
Authors: Lentin Ronit
Published: January 2017

This paper has three central components. One, it examines the basic tenets of feminist research methodologies — the commitment to making visible women's lived experiences, to gender and gender relations as socially constructed and historicially specific, to reflexivity and the inclusion of the researcher and the research process as researchable topics, and to the emancipation of women —and argues that they constitute feminist research methodologies as a separate paradigm. Two, by describing the methodological choices made for my study of Israeli daughters of Holocaust survivors, it examines the discontents of feminist research methodologies and how we can address them. Three, by asking why so few published studies by Irish feminist sociologists and Women's Studies scholars have adhered to feminist research methodologies, it hopes to launch a debate about feminist research methodologies in Ireland.
Converting Patients into Consumers: Consumerism and the Charter of Rights for Hospital Patients
Authors: O'Donovan Orla , Casey Dympna
Published: January 2017

This paper examines consumerism in public health policy and focuses on a specific strategy to make Irish hospital services more consumer-oriented, namely, patients' charters. The first part of the paper examines different conceptualisations of the ‘new consumerism’ in the social policy literature and locates its emergence within the broader context of the ‘marketisation’ of the welfare state. A brief review of the literature on the merits and limitations of public sector consumerism is then presented. The second part of the paper concentrates on the emergence of the new consumerism in Irish public health policy, and the results of a study that examined the implementation of the Charter of Rights for Hospital Patients are presented. A key finding of the study was that none of the hospitals in the study area had fully implemented the provisions of the Charter. Furthermore, only 26 per cent of a sample of one hundred hospital patients had heard of the Charter and only 10 per cent could recall any of the rights that it conferred on patients. The paper concludes by suggesting that the Charter of Rights for Hospital Patients is less concerned with empowering patients than it is with other agendas, such as creating a semblance of closeness to the users of health services and counterbalancing medical authority.
Massachusetts nua darb ainm Caillimh?Athchultúrú fhochomhlachtaí: Digital Equipment Corporation
Authors: Canainn Aodh ó
Published: January 2017

This paper examines the acculturation programme which Digital Equipment Corporation has used with success to co-ordinate its world-wide activities. It is critical of the misuse of cultural concepts by some management authors and questions their talk of ‘shared values’. Employees accept the official values of the company as coordinates of work rather than internalising them. In Digital this is not difficult as the declared values are such that most people are happy to accept them as guidelines for management practices. Taking the former hardware manufacturing operation of the Ballybrit factory as an example, the aims of an acculturation programme are examined. The question of whether or not a local culture can be a match for a strong organisational culture is considered. The author concludes that Irish culture is no match for Digital culture in the workplace but that company culture has little or no influence outside it.
School-Leavers' Career Choices: An Investigation of the Relationship between Action and Structure
Authors: Canny Angela
Published: January 2017

School-leavers' career choices are discussed from the perspective of both structure and action. Very little research has attempted to reconcile the structural conditioning of students' choices with students' own worldview orientations. To help bridge this gap this paper presents a broader analysis of school-leavers' choices, by investigating the combined effects of gender, social class, geographical locality together with students' own worldview orientations and expectations. An attempt will be made to operationalise the link between opportunity structure and worldview by examining the students' educational and occupational aspirations, their migration intentions and their perceptions of the labour market. Using discriminant analysis, the final section seeks to explicate the main factors predicting higher education intentions.
The Future of Child Neurology
Authors: Polsky Daniel , Werner Rachel M.
Published: January 2017

Current workforce projections estimate that there is a shortage of child neurologists. We surveyed child neurology residents to learn more about the entry point for a career in child neurology: what attracts current residents to the field of child neurology and what the future career plans of child neurology residents are. Most respondents (52%) were exposed to child neurology for the first time in their third or fourth year of medical school, with 41% reporting that they chose the specialty at that time. US medical graduates identified having a mentor as one of the most influential exposures in their career choice. Respondents predict that they will spend less time on patient care and more time on research than current practicing child neurologists report. When asked about what could improve the attractiveness of the field, residents responded that medical students should get increased and earlier exposure to child neurology. Given the declining number of individuals pursuing a career in child neurology and that current residents predict that they will spend less time seeing patients than their predecessors do, understanding how to attract more candidates to child neurology will be essential to alleviate future shortages in child neurology. ( J Child Neurol 2004; 19:6-13).
Neonatal General Movements
Authors: Zuk Luba , Harel Shaul , Leitner Yael , Fattal-Valevski Aviva
Published: January 2017

Intrauterine growth retardation plays a significant role in neurodevelopmental outcome. The assessment of general movements during the first 20 weeks is a new method for early detection of brain dysfunction. General movements in 31 infants with asymmetric intrauterine growth retardation and their appropriate for gestational age—matched controls were examined. General movements were scored as normal or abnormal by sequential videotape recordings in the writhing (term to 2 weeks), early fidgety (9—11 weeks), and late fidgety (14—16 weeks) periods. Scores were compared between the groups and correlated with neurodevelopmental outcome at 2 years. The incidence of normal general movements was lower in the intrauterine growth retarded infants than in the controls ( P < .001). Significant correlations were found between general movement quality and neurodevelopmental scores in the intrauterine growth retarded group. The fidgety movements were the most sensitive and specific for prediction of neurologic outcome. The general movement assessment can, therefore, serve as an additional tool for examining the neurologic status of the preterm and term intrauterine growth retarded infant. ( J Child Neurol 2004;19:14—18).
Progressive Multifocal Leukoencephalopathy Successfully Treated With Highly Active Antiretroviral Therapy and Cidofovir in an Adolescent Infected With Perinatal Human Immunodeficiency Virus (HIV)
Authors: Robinson Lisa-Gaye , Chiriboga Claudia A. , Champion Susan E. , Ainyette Ivan , Abrams Elaine J.
Published: January 2017

Progressive multifocal leukoencephalopathy is an infectious demyelinating brain disease caused by the JC virus that is associated with significant morbidity and mortality in the immunocompromised host. We report a case of progressive multifocal leukoencephalopathy successfully treated with highly active antiretroviral therapy and cidofovir in an adolescent patient perinatally infected with human immunodeficiency virus (HIV). ( J Child Neurol 2004;19:35—38).
Developmental Dyscalculia
Authors: Shalev Ruth S.
Published: January 2017

Developmental dyscalculia is a specific learning disability affecting the normal acquisition of arithmetic skills. Genetic, neurobiologic, and epidemiologic evidence indicates that dyscalculia, like other learning disabilities, is a brain-based disorder. However, poor teaching and environmental deprivation have also been implicated in its etiology. Because the neural network of both hemispheres comprises the substrate of normal arithmetic skills, dyscalculia can result from dysfunction of either hemisphere, although the left parietotemporal area is of particular significance. The prevalence of developmental dyscalculia is 5 to 6% in the school-aged population and is as common in girls as in boys. Dyscalculia can occur as a consequence of prematurity and low birthweight and is frequently encountered in a variety of neurologic disorders, such as attention-deficit hyperactivity disorder (ADHD), developmental language disorder, epilepsy, and fragile X syndrome. Developmental dyscalculia has proven to be a persisting learning disability, at least for the short term, in about half of affected preteen pupils. Educational interventions for dyscalculia range from rote learning of arithmetic facts to developing strategies for solving arithmetic exercises. The long-term prognosis of dyscalculia and the role of remediation in its outcome are yet to be determined. ( J Child Neurol 2004;19:765—771).
Attention-Deficit Hyperactivity Disorder (ADHD)
Authors: Voeller Kytja K. S.
Published: January 2017

Approaches to the diagnosis and treatment of attention-deficit hyperactivity disorder (ADHD) are undergoing a major change as a result of information from studies on the genetics of ADHD and the use of new neuroimaging technologies. Moreover, pharmacogenomics, although still in its infancy, will provide a basis for much more sophisticated treatment strategies for ADHD, particularly once more information is available about the genetics of ADHD. Even at this point in time, there is some pertinent information available that, although not ready for application in clinical settings, nonetheless provides a broader perspective for the clinician. In terms of etiology, ADHD is a neuropsychiatric disorder. There is a genetic basis in about 80% of the cases, involving a number of different genes, and in about 20% of the cases, ADHD is the result of an acquired insult to the brain. Some individuals likely have both genetic and acquired forms. Although medication works well in many cases of ADHD, optimal treatment of ADHD requires integrated medical and behavioral treatment. The family plays a crucial role in the management of children with ADHD. Because there is often a very high degree of comorbidity between ADHD and learning disabilities, teachers also have a great deal to contribute in the day-to-day management of these children. Early recognition and treatment prevent the development of more serious psychopathology in adolescence and adulthood. ( J Child Neurol 2004;19:798—814).
Psychiatric Implications of Language Disorders and Learning Disabilities: Risks and Management
Authors: Sundheim Suzanne T. P. V. , Voeller Kytja K. S.
Published: January 2017

This article reviews the relationship between different learning disabilities, language disorders, and the psychiatric disorders that are commonly associated with learning disabilities and language disorder: attention-deficit hyperactivity disorder (ADHD), anxiety disorders, depression, and conduct or antisocial personality disorder. The complex associations between language disorders and specific learning disabilities—dyslexia, nonverbal learning disorder, dyscalculia—and the various psychiatric disorders are discussed. Clinical vignettes are presented to highlight the impact of these disorders on a child's social and psychological development and the importance of early recognition and treatment. ( J Child Neurol 2004;19:814—826).
Chronic Daily Headaches in Pediatric Neurology Practice
Authors: Moore Alison J. , Shevell Michael
Published: January 2017

Using a comprehensive computerized database for a single general pediatric neurology ambulatory practice, the clinical profile, together with the precipitating features and outcomes, in a consecutive series of children with chronic daily headache was ascertained. Chronic daily headache was defined as persistent or daily headaches of at least 3 months' duration. Children with persistent headaches owing to a serious medical condition were specifically excluded from analysis. Of 1669 children (24% of total seen) referred over an 11-year inclusive interval for the evaluation of headaches, 50 (3%) merited a diagnosis of chronic daily headache. The overwhelming majority were female (45/50; 90%), with a mean age at diagnosis of 12.9 years (range 7—16 years). Transformed migraine was determined to be the etiology in the majority (35/50; 70%), with a minority attributable either to postconcussion syndrome (7/50; 14%) or new daily persistent headaches (6/50; 12%). Analgesic abuse was evident in a majority (26/50; 52%). Treatment consisted predominantly of migraine and analgesic education, with a majority of children (42/50; 84%) also receiving daily prophylaxis. Four fifths (41/50; 82%) returned for at least one follow-up. Of these, 56% (23/41) were improved, 32% (13/41) unchanged, and 12% (5/41) worse. Eventually, almost half (20/41) experienced complete resolution of their headache symptoms, with a mean time to resolution of 8 months (range 2—48 months). Children with chronic daily headache are thus a small subset of children with headache seen in general ambulatory practice. They tend to be females in the midteen years experiencing a transformed migraine complicated by analgesic abuse, suggesting potential preventability. Simple measures, which can include the use of prophylactic agents, can be expected to result in improvement and eventual resolution of headache symptoms. ( J Child Neurol 2004;19:925—929).
Predictors of Intellectual Function After a Unilateral Cortical Lesion: Study of 635 Patients From Infancy to Adulthood
Authors: Montour-Proulx Isabelle , Braun Claude M. J. , Daigneault Sylvie , Rouleau Isabelle , Kuehn Sally , Bégin Jean
Published: January 2017

This study investigated potential predictors of intellectual outcome in 417 children and 218 adults who had sustained a unilateral cortical lesion. Of these, 295 cases were collected from the scientific literature and 340 from medical records at seven hospitals in Canada. Different sets of predictors emerged for the Wechsler Verbal and Performance IQ values, accounting for differing variances (ie, 12.4% and 20.1%, respectively). The volume of the lesion was the factor that explained the most variance (ie, 4.95% and 11.7%, respectively). Age at lesion onset was significantly and positively correlated with verbal intelligence scores. This variable, considered independently or in interaction with other predictors, did not account for a large portion of the variance explained in intelligence. This refutes the commonly held notion that early onset of the insults results in a better prognosis. ( J Child Neurol 2004; 19:935-943).
Retrospective Study of the Use of Levetiracetam in Childhood Seizure Disorders
Authors: Koukkari Mark W. , Guarino Edward J.
Published: January 2017

The purpose of this study was to assess the efficacy and safety of levetiracetam in a diverse pediatric epilepsy population. A retrospective chart review of 52 consecutive children age 8 months to 16 years who were treated with levetiracetam was performed. The data include patients with partial and generalized seizures, monotherapy, and concomitant antiepileptic drug use. Levetiracetam was dosed to efficacy or unacceptable side effects, with a range of 8 to 315 mg/kg/day. Two patients discontinued levetiracetam prior to assessment of efficacy owing to side effects. Of the remaining 50 patients, 12 had ≥ 75% seizure reduction, 3 had ≥ 50% and < 75% seizure reduction, 8 had ≥ 25% and < 50% seizure reduction, and 27 had < 25% seizure reduction. A variety of pediatric epilepsy syndromes and seizure types were represented. Adverse events, seen in 17 patients, were determined to be tolerable or resolved over time with continued dosing, dosage reduction, or discontinuation. This open-label, retrospective study of 52 consecutive pediatric patients treated with levetiracetam indicates at least partial efficacy in a variety of pediatric epilepsy syndromes. Tolerability was surprisingly favorable, even at doses far exceeding 40 mg/kg/day. ( J Child Neurol 2004;19:944—947).
Relationship Between the Clinical-Neurophysiologic Pattern, Disability, and Quality of Life in Adolescents With Spina Bifida
Authors: Padua Luca , Rendeli Claudia , Ausili Emanuele , Aprile Irene , Caliandro Pietro , Tonali Pietro , Salvaggio Elio
Published: January 2017

A number of studies have focused on quality of life in children with spina bifida, concentrating for the most part on younger children. We assessed health-related quality of life and disability in adolescents with spina bifida and correlated these with the clinical and neurophysiologic picture. Twelve consecutive spina bifida cases were evaluated through a multiperspective protocol by means of (1) clinical and neurophysiologic assessment, (2) a self-administered questionnaire for general health—Short Form-36 (SF-36)—and (3) standardized disability measurements (Functional Independence Measure, Barthel Index, Deambulation Index). Relationships between disability measurement, the adolescents' perspective, and conventional clinical and neurophysiologic assessment were evaluated. The Functional Independence Measure is strongly related to the clinical measurement of muscle strength ( P < .03, r > .6). The Barthel Index and Deambulation Index are related to clinical measurement of muscle strength ( P < .03, r > .6), as well as to electromyographic findings in the proximal muscles of inferior limbs ( P < .05, r > .6). Although the function of lower limb muscles, especially the proximal muscles, is closely related to impairment of physical aspects of quality of life, it is only partially related to the mental aspects ( P < .03, r > .6). The findings at clinical examination, especially proximal deficit of inferior limbs, were usually related to higher disability and lower physical aspects of quality of life. The multiperspective evaluation of adolescents with spina bifida shows a high correlation between conventional neurologic-neurophysiologic measurements and the adolescents' own perspective of their quality of life. Our data demonstrated that the conventional clinical and neurophysiologic measurements are useful not only because they provide a measure of lower limb impairment but also because they appear to be related to physical aspects of the adolescent's quality of life. ( J Child Neurol 2004;19:952—957).
Autonomic Dysfunction in Mental Retardation and Spastic Paraparesis With MECP2 Mutation
Authors: Dotti Maria Teresa , Guideri Francesca , Acampa Maurizio , Orrico Alfredo , Battisti Carla , Federico Antonio
Published: January 2017

Autonomic nervous system involvement in female patients with classic Rett syndrome usually manifests as breathing abnormalities, peripheral vasomotor disturbances, and cardiac sympathetic imbalance, the latter a possible cause of sudden death. MECP2 gene mutations responsible for Rett syndrome have also been found in male patients with mental retardation, sometimes associated with different neurologic abnormalities. However, autonomic nervous system functions have never been investigated in male patients with X-linked mental retardation owing to MECP2 mutations. We studied heart rate variability, a marker of autonomic activity, in a family with the MECP2 mutation in male patients, one of whom had died suddenly. Cardiovascular features similar to those observed in a Rett syndrome variant with preserved speech were found, suggesting sympathetic imbalance. ( J Child Neurol 2004; 19:964-966).
Standardization of the Capute Scales: Methods and Results
Authors: Visintainer Paul F. , Leppert Mary , Bennett Anna , Accardo Pasquale J.
Published: January 2017

The Capute Scales is a 100-item developmental assessment tool to quantitatively measure expressive and receptive language and nonverbal problem-solving skills in infants from birth to 3 years of age. The present multisite study standardized the instrument on a population of 1055 typically developing children balanced for age, sex, and race and generated normative tables to document the instrument's utility. There were no significant performance differences by race or sex. The achieved age levels for individual test items and for total scores were accurately predicted by the age levels assigned to the test items. The conversion of age levels to standard scores was found to be unnecessary because the developmental quotients derived from age ratios did not differ significantly from the derived scores. ( J Child Neurol 2004;19:967—972).
Ketamine Increases Opening Cerebrospinal Pressure in Children Undergoing Lumbar Puncture
Authors: Yoram Ben Yehuda  , Watemberg Nathan
Published: January 2017

Intravenous ketamine, often administered concomitantly with midazolam, is one of the most commonly used drugs for procedural sedation in children. Although it is considered safe for use in children, ketamine can increase intracranial pressure. Hence, this study was carried out to determine whether adding ketamine to midazolam prior to performing lumbar puncture would be associated with a higher cerebrospinal fluid opening pressure in children with suspected aseptic meningitis. Thirty-nine children aged 6 months to 14 years were included: 26 in group A (intravenous midazolam plus ketamine) and 13 in group B (intravenous midazolam only). Mean cerebrospinal fluid opening pressure in group A was 24.4 cm H2O (median 23 cm H2O) compared with 20 cm H2O (median 19.8 cm H2O) in group B ( P = .011). Intravenous ketamine prior to performing lumbar puncture is associated with a significantly higher lumbar cerebrospinal fluid opening pressure in children with aseptic meningitis. However, no outcome differences have been found between the two groups. ( J Child Neurol 2006;21:441—443;
Familial Robertsonian 13;14 Translocation With Mental Retardation and Epilepsy
Authors: Buoni Sabrina , Zannolli Raffaella , Macucci Francesca , Pucci Lucia , Mogni Massimo , Pierluigi Mauro , Fois Alberto
Published: January 2017

Familial reports of a robertsonian translocation in more than two generations are rare. We report three generations (a daughter, the mother, and the mother's father) with a heterozygous, balanced robertsonian translocation t(13;14)(q11;q11). Central nervous system disease was present, but differentially expressed, in generations I and III. The daughter presented with mental delay and epilepsy, and the mother was apparently healthy, whereas the mother's father was again symptomatic, with borderline intelligence. Fluorescent in situ hybridization analysis was performed to exclude a loss or gain of chromosomal material. No uniparental disomy was present. We concluded that genetic counseling in the presence of this rearrangement was extremely difficult, independent of the affected parent being symptomatic or asymptomatic. ( J Child Neurol 2006;21:531—533; DOI 10.2310/7010.2006.00098).
Risperidone in Children With Autism: Randomized, Placebo-Controlled, Double-Blind Study
Authors: Nagaraj Ravishankar , Singhi Pratibha , Malhi Prahbhjot
Published: January 2017

Some open-label studies suggest that risperidone can be useful in the treatment of certain target symptoms in children with autism. We aimed to study whether the use of risperidone in comparison with placebo improved functioning in children with autism with regard to behavior (aggressiveness, hyperactivity, irritability), social and emotional responsiveness, and communication skills. We conducted a randomized, double-blind, placebo-controlled trial with 40 consecutive children with autism, whose ages ranged from 2 to 9 years, who were receiving either risperidone or placebo given orally at a dose of 1 mg/day for 6 months. Autism symptoms were monitored periodically. The outcome variables were total scores on the Childhood Autism Rating Scale (CARS) and the Children's Global Assessment Scale (CGAS) after 6 months. Of the 40 children enrolled, 39 completed the trial over a period of 18 months; 19 received risperidone, and 20 received placebo. In the risperidone group, 12 of 19 children showed improvement in the total Childhood Autism Rating Scale score and 17 of 19 children in the Children's Global Assessment Scale score compared with 0 of 20 children for the Childhood Autism Rating Scale score and 2 of 20 children for the Children's Global Assessment Scale score in the placebo group ( P < .001 and P = .035, respectively). Risperidone also improved social responsiveness and nonverbal communication and reduced the symptoms of hyperactivity and aggression. Risperidone was associated with increased appetite and a mild weight gain, mild sedation in 20%, and transient dyskinesias in three children. Risperidone improved global functioning and social responsiveness while reducing hyperactivity and aggression in children with autism and was well tolerated. ( J Child Neurol 2006;21:450—455; DOI 10.2310/7010.2006.00099).
Neurodevelopmental Outcome of Severe Neonatal Hemolytic Hyperbilirubinemia
Authors: Chen Wen-Xiong , Wong Virginia C. N. , Wong Kar-Yin
Published: January 2017

We recruited 128 neonates with hyperbilirubinemia over a 5-year period (1995—2000) to study the short- and long-term effects of hemolytic hyperbilirubinemia on the auditory brainstem pathway and neurodevelopmental status. These children were divided into two groups: (1) a hemolytic group ( n = 29; ABO incompatibility [ n = 19], Rh incompatibility [ n = 1], glucose-6-phosphate dehydrogenase deficiency [ n = 8] and both ABO incompatibility and glucose-6-phosphate dehydrogenase deficiency [ n = 1]) and (2) a nonhemolytic group ( n = 99). All received phototherapy. Exchange transfusions were performed for four (13.8%) in the hemolytic group and three (3%) in the nonhemolytic group. The brainstem auditory evoked potential was recorded at a mean age of 3.2 months in the hemolytic group and 3.1 months in the nonhemolytic group. Serial brainstem auditory evoked potential assessments were performed until 2 years of age (3 in the hemolytic group and 18 in the nonhemolytic group). All had regular physical, neurologic, visual, and auditory evaluation until 3 years of age. The rate of exchange transfusion was significantly higher in the hemolytic group than in the nonhemolytic group ( P< .05). Brainstem auditory evoked potential abnormalities at the initial assessment occurred in three (10.4%) in the hemolytic group (all related to ABO incompatibility) and nine (9.1%) in the nonhemolytic group. At 2 years, the brainstem auditory evoked potential returned to normal except in three cases with a slightly increased hearing threshold (one [3.5%] in the hemolytic group at 60 dB nHL and two [2%] in the nonhemolytic group at 50 dB nHL]). There were no significant differences in the rate of brainstem auditory evoked potential abnormalities at the initial or subsequent assessments between both groups. All except five cases had a normal neurodevelopmental outcome at 3 years (three [two with ABO incompatibility and one with glucose-6-phosphate dehydrogenase deficiency] in the hemolytic group [10.4%] and two [2%] in the nonhemolytic group). All had mild motor delay and hypotonia, which returned to normal at 3 years. The rate of abnormal neurodevelopmental outcome was higher in the hemolytic group than in the nonhemolytic group, although with no significant difference between both groups ( P= .08). All five cases in both groups with abnormal neurodevelopment had a normal brainstem auditory evoked potential at the initial assessment. There was no relationship between the abnormal initial brainstem auditory evoked potential and the final neurodevelopmental outcome. The toxic effect of hyperbilirubinemia on the auditory brainstem pathway and neurodevelopmental status in our cohort was transient. The prognosis of neonatal hemolytic hyperbilirubinemia in our Chinese cohort is excellent, possibly owing to an aggressive early-intervention approach. ( J Child Neurol 2006;21:474—479; D
Deletion of the Long Arm of Chromosome 6: Report on a New Case With Intractable Epilepsy
Authors: Cerminara Caterina , Bombardieri Roberta , Pinci Mariangela , Seri Stefano , Curatolo Paolo
Published: January 2017

Interstitial deletions in the terminal region of chromosome 6 are rare. The deletion most often occurs de novo. Mental retardation is always described. The most characteristic manifestations are microcephaly, micrognathia, hypotonia, typical facial appearance, strabismus, and congenital heart defects. Although this chromosomal syndrome does not appear to have a distinctive phenotype, epileptic seizures are uncommon in affected individuals. We report on a novel finding in a patient with the 46 XX karyotype and del(6)(q25-q26) who developed intractable epilepsy. ( J Child Neurol 2006;21:527—531; DOI 10.2310/7010.2006.00112).
Long-Term Use of Oxcarbazepine Oral Suspension in Childhood Epilepsy: Open-Label Study
Authors: Rufo-Campos Miguel , Casas-Fernández Carlos , Martínez-Bermejo Antonio
Published: January 2017

Studies designed specifically for the pediatric population are needed to assess the tolerability and safety of the new antiepileptic drugs. The purpose of this study was to document the safety, ease of dosing, and acceptance of oxcarbazepine oral suspension in pediatric patients in monotherapy and polytherapy. A prospective, multicenter, open-label study was conducted at the neurology services of three pediatric university hospitals over 12 months. After obtaining signed informed consent, we enrolled a series of 62 patients with epilepsy aged between 2 months and 14 years who began oxcarbazepine treatment in monotherapy or in combination with other antiepileptic drugs to assess the seizure frequency, safety (adverse events), and acceptance of the pharmaceutical form by the patient's family. Fifty patients (80.6%) reduced seizures by at least 50%, 44 (71%) saw a reduction in seizure frequency of over 75%, and 29 (46.8%) were seizure free at the end of the study. The difference in the number of seizures before and after the study was statistically significant, both overall and by type of pathology. Adverse events occurred in four patients (6.4%) and required withdrawal of the drug in two cases (skin rash); three patients (4.8%) withdrew for inefficacy. Five patients (8.1%) withdrew from the treatment. We concluded that, in this series of patients, oxcarbazepine in oral suspension form was seen to help reduce seizure frequency, to have few side effects, and to be accepted by parents and patients. ( J Child Neurol 2006;21:480—485; DOI 10.2310/7010.2006.00120).
Clinical and Imaging Study of Human Immunodeficiency Virus-1—Infected Youth Receiving Highly Active Antiretroviral Therapy: Pilot Study Using Magnetic Resonance Spectroscopy
Authors: Gabis Lidia , Belman Anita , Wei Huang  , Milazzo Maria , Nachman Sharon
Published: January 2017

The objective of this study was to correlate clinical and neuropsychologic findings with neuroimaging studies to assess the value of magnetic resonance spectroscopy in detection and monitoring of central nervous system disease of children vertically infected with human immunodeficiency virus (HIV)-1 and receiving highly active antiretroviral therapy. Eight children (four boys) with vertically transmitted stable HIV infection had a neurologic examination, neuropsychologic testing, and a neuroimaging study. The structural magnetic resonance imaging (MRI) and metabolic changes (magnetic resonance spectroscopy) were compared with the cognitive, clinical, and laboratory results. Our results for the eight children who completed the magnetic resonance spectroscopic study showed that a high N-acetylaspartate (NAA) to choline ratio correlated significantly with IQ subtests of arithmetic ( r = .74; P < .034) and comprehension ( r = .77; P = .025). Our results suggest that lower NAA and higher choline values represent neuronal dysfunction and inflammation that can be recognized before anatomic changes appear on MRI. In addition, a low NAA to Cho ratio correlated with poor performance. These data suggest that magnetic resonance spectroscopy can be used as a follow-up tool in addition to the structural MRI. Moreover, a change in a child's performance with a concomitant change in NAA and choline, as measured with magnetic resonance spectroscopy, could indicate the need for more aggressive intervention. ( J Child Neurol 2006;21:485—490; DOI 10.2310/7010.2006.00126).