Reversible acquired epileptic frontal syndrome and CSWS suppression in a child with congenital hemiparesis treated by hemispherotomy

Rapport de synthèse : Cette thèse a étudié en détail le cas d'un enfant souffrant d'une hémiplégie congénitale sur un infarctus prénatal étendu qui a développé une forme particulière d'épilepsie, le syndrome des pointes ondes continues du sommeil (POCS), associé à une régression mentale massive. Les caractéristiques de cette détérioration pointaient vers un dysfonctionnement de type frontal. Une chirurgie de l'épilepsie (hémisphérotomie) a, non seulement, permis la guérison de l'épilepsie mais une récupération rapide sur le plan comportemental et cognitif, suivie d'une reprise plus lente du développement, avec finalement à l'âge de 11 ans un niveau de déficience intellectuelle modérée. L'intérêt de cette étude réside dans le fait que l'enfant a pu être suivi prospectivement entre l'âge de 4.5 ans et 11 ans par des enregistrements électro-encéphalographiques (EEG) ainsi que des tests neuropsychologiques et des questionnaires de comportements sériés, permettant de comparer les périodes pré-, péri- et postopératoires, ce qui est rarement réalisable. Un enregistrement EEG de surface a même pu être effectué durant l'opération sur l'hémisphère non lésé, permettant de documenter l'arrêt des décharges épileptiformes généralisées dès la fin de l'intervention. L'hypothèse que nous avons- souhaité démontrer est que la régression comportementale et cognitive présentée par l'enfant après une période de développement précoce presque normale (retard de langage) était de nature épileptique : nous l'expliquons par la propagation de l'activité électrique anormale à partir de la lésion de l'hémisphère gauche vers les régions préservées, en particulier frontales bilatérales. L'hémisphérotomie a permis une récupération rapide en déconnectant l'hémisphère gauche lésé et épileptogène de l'hémisphère sain, qui a ainsi pu reprendre les fonctions cognitives les plus importantes. Les progrès plus lents par la suite et l'absence de rattrapage au delà d'un niveau de déficience mentale modérée sont plus difficiles à expliquer: on postule ici un effet de l'épilepsie sur le développement de réseaux neuronaux de l'hémisphère initialement non lésé, réseaux qui sont à la fois à un stade précoce de leur maturation et en cours de réorganisation suite à la lésion prénatale. La littérature sur les déficits cognitifs avant et après hemisphérotomie s'est surtout préoccupée du langage et de sa récupération possible. À notre connaissance, notre étude est la première à documenter la réversibilité d'une détérioration mentale avec les caractéristiques d'un syndrome frontal après hémisphérotomie. La chirurgie de l'épilepsie a offert ici une occasion unique de documenter le rôle de l'activité épileptique dans la régression cognitive puisqu'en interrompant brusquement la propagation de l'activité électrique anormale, on a pu comparer la dynamique du développement avant et après l'intervention. La mise en relation des multiples examens cliniques et EEG pratiqués chez un seul enfant sur plusieurs années a permis d'obtenir des informations importantes dans la compréhension des troubles cognitifs et du comportement associés aux épilepsies focales réfractaires. ABSTRACT : A boy with a right congenital hemiparesis due to a left pre-natal middle cerebral artery infarct developed focal epilepsy at 33 months and then an insidious and subsequently more rapid, massive cognitive and behavioural regression with a frontal syndrome between the ages of 4 and 5 years with continuous spike-waves during sleep (CSWS) on the EEG. Both the epilepsy and the CSWS were immediately suppressed by hemispherotomy at the age of 5 years and 4months. A behavioural-cognitive follow-up prior to hemispheratomy, an per-operative EEG and corticography and serial post-operative neuropsychological assessments were performed until the age of 11 years. The spread of the epileptic activity to the "healthy" frontal region was the cause of the reversible frontal syndrome. A later gradual long-term but incomplete cognitive recovery, with moderate mental disability was documented. T9ris outcome is probably explained by another facet of the epilepsy, namely the structural effects of prolonged epileptic dischazges in rapidly developing cerebral networks which are, at the same time undergoing the reorganization imposed by a unilateral early hemispheric lesion. Group studies on the outcome of children before and after hemispherectomy using only single IQ measures, pre- and postoperatively, may miss particular epileptic cognitive dysfunctions as they are likely to be different from case to case. Such detailed and rarely available complementary clinical and EEG data obtained in a single case at different time periods in relation to the epilepsy, including peroperative electrophysiological findings, may help to understand the different cognitive deficits and recovery profiles and the limits of full cognitive recovery.

Sense of time in children with Attention-Deficit/Hyperactivity Disorder (ADHD) : a comparative study

Parents of children with attention-deficit/hyperactivity disorder (ADHD) frequently report that their children have a poor sense of time. Several studies looking at the perception of time mentioned a form of temporal myopia. The present study investigates the sense of time in children with ADHD. Twenty-two French-speaking Swiss children with ADHD and 22 controls between the ages of 6 and 13 years were tested using a conventional time knowledge questionnaire and two Piagetian time conservation tasks. Parents were asked to complete the "It's about time" questionnaire. Better performance was observed in matched control group children than in children with ADHD on a conventional time knowledge questionnaire. For children under 10 years of age, the two Piagetian time conservation tasks were able to differentiate children with and without ADHD. Parents of ADHD children reported more frequently that their children had time-related difficulties in daily activities. This study suggests that children with ADHD take longer to develop several time-related abilities.

Information seeking activity and adherence to therapy: Findings from the Swiss Inflammatory Bowel Disease Cohort Study

Background: In spite of the relapsing nature of inflammatory bowel diseases (IBD), on average, 40% of IBD patients are nonadherent to treatments. On the other hand, they are often actively seeking information on their disease. The relationship between information seeking behaviour and adherence to treatment is poorly documented. The main aim of this study was to examine this association among IBD patients. Methods: We used data from the Swiss IBD cohort study. Baseline data included questions on adherence to ongoing treatments. A survey was conducted in October 2009 to assess information sources and themes searched by patients. Crude odds ratio (OR) and 95% CI were calculated for the association between adherence and information seeking. Adjustment for potential confounders and main known risk factors was performed using multivariate logistic regression. Differences in the proportions of information sources and themes were compared between adherent and non-adherent patients. Results: The number of patients eligible was 488. Nineteen percent (N = 99) were non-adherent to treatment and one third (N = 159) were active information seekers. Crude OR for being non-adherent was 69% higher among information seekers compared to non-seekers (OR = 1.69; 95%CI 0.99 2.87). Adjusted OR for non-adherence was OR = 2.39 (95%CI 1.32 4.34) for information seekers compared to non-seekers. Family doctors were 15.2% more often consulted (p = 0.019) among patients who were adherent to treatment compared to those who were not, as were books and TV (+13.1%; p = 0.048). No difference was observed for internet or gastroenterologists as sources of information. Themes of information linked to tips for disease management were 14.2% more often searched among non-adherent patients (p = 0.028) compared to adherent. No difference was observed for the other themes (research and development on IBD, therapies, basic information on the disease, patients' experiences sharing, miscellaneous). Conclusions: Active information seeking was shown to be strongly associated with non-adherence to treatment in a population of IBD patients in Switzerland. Surprisingly themes related to therapies were not especially those on which nonadherent patients focused. Indeed, management of symptoms and everyday life with the disease seemed to be the most pressing information concerns of patients. Results suggest that the family doctor plays an important role in the multidisciplinary care approach needed for IBD patients.

Acute diarrhoea in a community cohort of children who received an oral rotavirus vaccine in Northeast Brazil

Rotavirus is an important cause of childhood diarrhoea. A monovalent rotavirus vaccine (Rotarix®) was introduced into the Immunization Program of Brazil in 2006. In this study, we describe the incidence and burden of disease of rotavirus diarrhoea in two cohorts of children (vaccinated and unvaccinated). We followed two groups of 250 children under one year old, who were enrolled in December 2006 from a low-income residential area in Northeast Brazil. The children were monitored every two weeks for two years. Stool samples from children with diarrhoea were examined for the presence of rotavirus. Rotaviruses were genotyped using real time-polymerase chain reaction. The mean numbers of all-cause diarrhoea episodes/child (adjusted for age) in the first year were 0.87 and 0.84, in vaccinated and unvaccinated children, respectively. During the second year, the number of episodes/child decreased to 0.52 and 0.42. Only 16 (4.9%) of 330 stool samples were rotavirus-positive (10 vaccinated and 6 unvaccinated children) and only P[4]G2 rotaviruses were identified. All-cause diarrhoea episodes were more severe in unvaccinated children in the first year of age (p < 0.05), while vaccinated children had more severe episodes 18 months after vaccination. Rotavirus diarrhoea incidence was very low in both groups.

Do Fiscal and Political Decentralization Raise Students’ Performance? A Cross-Country Analysis

The low quality of education is a persistent problem in many developed countries. Parallel to in the last decades exists a tendency towards decentralization in many developed and developing countries. Using micro data from the Programme for International Student Assessment (PISA) referred to 22 countries, we test whether there exists an impact of fiscal and political decentralization on student performance in the areas of mathematics, reading skills and science. We observe that fiscal decentralization exerts an unequivocal positive effect on students’ outcomes in all areas, while the effect of political decentralization is more ambiguous. On the one hand, the capacity of the subnational governments to rule on its region has a positive effect on students’ performance in mathematics. On the other hand, the capacity to influence the country as a whole has a negative impact on mathematics achievement. As a general result, we observe that students’ performance in Mathematics is more sensible to these exogenous variations than in Sciences and reading skills. Keywords: School outcomes, PISA, fiscal decentralization, political decentralization JEL codes: H11, H77, I21

The European Board of Physical and Rehabilitation Medicine : the past, the present and the future [Europejska Rada Medycyny Fizykalnej i Rehabilitacji: przeszłość, teraźniejszość i perspektywy]

Right from the beginning of the development of the medical specialty of Physical and Rehabilitation Medicine (PRM) the harmonization of the fields of competence and the specialist training across Europe was always an important issue. The initially informal European collaboration was formalized in 1963 under the umbrella of the European Federation of PRM. The European Academy of PRM and the UEMS section of PRM started to contribute in 1969 and 1974 respectively. In 1991 the European Board of Physical and Rehabilitation Medicine (EBPRM) was founded with the specific task of harmonizing education and training in PRM in Europe. The EBPRM has progressively defined curricula for the teaching of medical students and for the postgraduate education and training of PRM specialists. It also created a harmonized European certification system for medical PRM specialists, PRM trainers and PRM training sites. European teaching initiatives for PRM trainees (European PRM Schools) were promoted and learning material for PRM trainees and PRM specialists (e-learning, books and e-books, etc.) was created. For the future the Board will have to ensure that a minimal specific undergraduate curriculum on PRM based on a detailed European catalogue of learning objectives will be taught in all medical schools in Europe as a basis for the general medical practice. To stimulate the harmonization of national curricula, the existing postgraduate curriculum will be expanded by a syllabus of competencies related to PRM and a catalogue of learning objectives to be reached by all European PRM trainees. The integration of the certifying examination of the PRM Board into the national assessment procedures for PRM specialists will also have to be promoted.

Allergie alimentaire chez l'enfant: quelle prise en charge en 2011 [Food allergy in children: anything new in its management?].

The prevalence of food allergy in children is increasing, in particular in its most severe presentation, i.e. anaphylaxis. Food allergy has an important impact on children's and their parent's quality of life, because of the fear of accidental ingestion and limitations of social activities. Quality of life questionnaires adapted to food allergy are now available, as well as new diagnostic procedures using recombinant technology. Their interpretation and their clinical correlation remain difficult, especially in children, in the absence of references values. Various oral and subcutaneous immunotherapy strategies are currently under evaluation, using modified or native allergens.

Performance of blood pressure-to-height ratio at a single screening visit for the identification of hypertension in children.

BACKGROUND: The diagnosis of hypertension in children is difficult because of the multiple sex-, age-, and height-specific thresholds to define elevated blood pressure (BP). Blood pressure-to-height ratio (BPHR) has been proposed to facilitate the identification of elevated BP in children. OBJECTIVE: We assessed the performance of BPHR at a single screening visit to identify children with hypertension that is sustained elevated BP. METHOD: In a school-based study conducted in Switzerland, BP was measured at up to three visits in 5207 children. Children had hypertension if BP was elevated at the three visits. Sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) for the identification of hypertension were assessed for different thresholds of BPHR. The ability of BPHR at a single screening visit to discriminate children with and without hypertension was evaluated with receiver operating characteristic (ROC) curve analyses. RESULTS: The prevalence of systolic/diastolic hypertension was 2.2%. Systolic BPHR had a better performance to identify hypertension compared with diastolic BPHR (area under the ROC curve: 0.95 vs. 0.84). The highest performance was obtained with a systolic BPHR threshold set at 0.80 mmHg/cm (sensitivity: 98%; specificity: 85%; PPV: 12%; and NPV: 100%) and a diastolic BPHR threshold set at 0.45 mmHg/cm (sensitivity: 79%; specificity: 70%; PPV: 5%; and NPV: 99%). The PPV was higher among tall or overweight children. CONCLUSION: BPHR at a single screening visit had a high performance to identify hypertension in children, although the low prevalence of hypertension led to a low PPV.

Gender and attachment representations in the preschool years: comparisons between five countries

Bowlby proposed that the individual's social experiences, as early as in infancy, contribute to the construction of Internal Working Models (IWMs) of attachment, which will later guide the individual's expectations and behaviors in close relationships all along his or her life. The qualitative, individual characteristics of these models reflect the specificity of the individual's early experiences with attachment figures. The attachment literature globally shows that the qualities of IWMs are neither gender specific nor cultural specific. Procedures to evaluate IWMs in adulthood have been well established, based on narrative accounts of childhood experiences. Narrative procedures at earlier ages (e.g., in the preschool years) have been proposed, such as Bretherton's Attachment Story Completion Task (ASCT), to evaluate attachment representations. More than 500 ASCT narratives of preschoolers, coming from five different countries, have been collected, in the perspective of examining possible interactions between gender and culture regarding attachment representations. A specific Q-Sort coding procedure (CCH) has been used to evaluate several dimensions of the narratives. Girls' narratives appeared as systematically more secure than those of same-age boys, whatever their culture. The magnitude of gender differences, however, varied between countries. Taylor's model of gender-specific responses to stress and Harwood's and Posada's hypothesis on inter-cultural differences regarding caregiving are evoked to understand the differences across gender and countries.

Regional cortical volumes and congenital heart disease: a MRI study in 22q11.2 deletion syndrome.

Children with congenital heart disease (CHD) who survive surgery often present impaired neurodevelopment and qualitative brain anomalies. However, the impact of CHD on total or regional brain volumes only received little attention. We address this question in a sample of patients with 22q11.2 deletion syndrome (22q11DS), a neurogenetic condition frequently associated with CHD. Sixty-one children, adolescents, and young adults with confirmed 22q11.2 deletion were included, as well as 80 healthy participants matched for age and gender. Subsequent subdivision of the patients group according to CHD yielded a subgroup of 27 patients with normal cardiac status and a subgroup of 26 patients who underwent cardiac surgery during their first years of life (eight patients with unclear status were excluded). Regional cortical volumes were extracted using an automated method and the association between regional cortical volumes, and CHD was examined within a three-condition fixed factor. Robust protection against type I error used Bonferroni correction. Smaller total cerebral volumes were observed in patients with CHD compared to both patients without CHD and controls. The pattern of bilateral regional reductions associated with CHD encompassed the superior parietal region, the precuneus, the fusiform gyrus, and the anterior cingulate cortex. Within patients, a significant reduction in the left parahippocampal, the right middle temporal, and the left superior frontal gyri was associated with CHD. The present results of global and regional volumetric reductions suggest a role for disturbed hemodynamic in the pathophysiology of brain alterations in patients with neurodevelopmental disease and cardiac malformations.

Alfabetização de crianças caboverdianas em língua portuguesa como língua não materna : o ensino da leitura

abstract: Cape Verde is a country of bilingual characteristics, where coexist two languages: the mother tongue – the Creole of Cape Verde (CCV) or the Capeverdian Language (LCV) and the Non Maternal language – the Portuguese that is the official language and, therefore, the language used in the process of education and learning. This situation generates conflicts so much to linguistic level as to cultural level. The two languages presents some lexical resemblances, what drives, many times, to misconceptions and linguistics errors that complicate children in the learning, in particular, of reading that constitute the base for the learning of others knowledge. The learning of reading, in the Non Maternal language, requires a development of the oral language in Portuguese Language, which stimulates the reasoning of the child through playful exercises and cognitivists and construtivists approaches. In this way, the competences of phonological processing in the acquisition of the competences of reading are important for the discrimination of written text and favor the learning and the development of reading. The child, through the discovery, begins to elaborate concepts in the way to obtain a relation with the written language, by functional form. Adopting a methodology of case study and through questionnaires, direct observation and collect of documentary information, this dissertation presents and analyzes connected aspects to the literacy of capeverdian children in the beginning of the schooling and to the learning of reading as basic support for the learning of Non Maternal language. The subsidies collected by the study, presented in this dissertation will contribute for the education progress of reading and, also, for implement successfully the learning of reading of the students, developing to practical of reading and the expectations in uncover the multiplicity of the dimensions of experience in that domain and contribute for a relative comprehension of written and reading modes.

Alfabetização de crianças caboverdianas em língua portuguesa como língua não materna : o ensino da leitura

abstract: Cape Verde is a country of bilingual characteristics, where coexist two languages: the mother tongue – the Creole of Cape Verde (CCV) or the Capeverdian Language (LCV) and the Non Maternal language – the Portuguese that is the official language and, therefore, the language used in the process of education and learning. This situation generates conflicts so much to linguistic level as to cultural level. The two languages presents some lexical resemblances, what drives, many times, to misconceptions and linguistics errors that complicate children in the learning, in particular, of reading that constitute the base for the learning of others knowledge. The learning of reading, in the Non Maternal language, requires a development of the oral language in Portuguese Language, which stimulates the reasoning of the child through playful exercises and cognitivists and construtivists approaches. In this way, the competences of phonological processing in the acquisition of the competences of reading are important for the discrimination of written text and favor the learning and the development of reading. The child, through the discovery, begins to elaborate concepts in the way to obtain a relation with the written language, by functional form. Adopting a methodology of case study and through questionnaires, direct observation and collect of documentary information, this dissertation presents and analyzes connected aspects to the literacy of capeverdian children in the beginning of the schooling and to the learning of reading as basic support for the learning of Non Maternal language. The subsidies collected by the study, presented in this dissertation will contribute for the education progress of reading and, also, for implement successfully the learning of reading of the students, developing to practical of reading and the expectations in uncover the multiplicity of the dimensions of experience in that domain and contribute for a relative comprehension of written and reading modes.

Health-related quality of life in migrant preschool children.

BACKGROUND: Minority groups have a lower health-related quality of life (HRQOL), but there is little information if this finding also applies to children. In this study, we compared HRQOL between young children with and without migrant parents. METHODS: Two cross-sectional studies of culturally diverse preschool populations in Switzerland: Ballabeina (40 preschools, 258 girls and 232 boys aged 4 to 6 years) and Youp'là Bouge (58 child care centers, 453 girls and 522 boys aged 2 to 4 years). Most children were born in Switzerland (Ballabeina: 92.3%; Youp'là Bouge: 93.7%). Number of migrant parents was considered as the main exposure. HRQOL was measured using the 23-item Pediatric Quality of Life Inventory. RESULTS: Children of migrant parents had a significantly lower HRQOL total score (mean ± SD, Ballabeina: 84.2 ± 9.1; 82.7 ± 9.6 and 81.7 ± 11.7 for children with none, one or two migrant parents, respectively; Youp'là Bouge: 83.8 ± 8.6; 82.9 ± 9.5; 80.7 ± 11.7, all p < 0.05). Similar results were found in Ballabeina and Youp'là Bouge for social, school and physical functioning (all p < 0.05), but not for emotional functioning. The differences in HRQOL measures were partly mediated by children's place of birth, parental education, paternal occupational level, children's BMI, screen time and physical activity in one study (Ballabeina), but not in the other (Youp'là Bouge). CONCLUSION: In preschoolers, children of migrant parents have lower HRQOL than children of non-migrant parents. These differences are only partly mediated by other sociocultural characteristics or lifestyle behavior. These families may need assistance to prevent further inequalities.

Effect of weight loss on resting energy expenditure in obese prepubertal children.

To assess the effect of weight loss on resting metabolic rate (RMR), the energy expenditure of eight obese prepubertal children (age 9 +/- 1 years; weight 48.7 +/- 9.1 kg; BMI 25.3 +/- 3.9) and of 14 age-matched children of normal body weight (age 9 +/- 1 years; weight 28.8 +/- 5.6 kg; BMI 16.5 +/- 1.7) was measured by indirect calorimetry. The obese children were reinvestigated after a mean weight loss of 5.4 +/- 1.2 kg induced by a six-months mixed hypocaloric diet. Before slimming, the obese group showed a higher daily energy intake than the control group (10.40 +/- 3.45 MJ/day vs 7.97 +/- 2.02 MJ/day respectively; P less than 0.05) but a similar value was observed per unit fat-free mass (FFM) (0.315 +/- 0.032 MJ/kgFFM/day vs 0.329 +/- 0.041 MJ/kgFFM/day respectively). The average RMR of the obese children was greater than that of the control group (5217 +/- 531 kJ/day vs 4477 +/- 506 kJ/day) but similar after adjusting for FFM (4728 +/- 3102 kJ/day vs 4899 +/- 3102 kJ/day). Weight loss resulted in a reduction in RMR (5217 +/- 531 kJ/day vs 4874 +/- 820 kJ/day), each kg of weight loss being accompanied by a decrease of RMR of 64 kJ (15.3 kcal) per day. The changes in RMR induced by weight loss paralleled the changes in FFM. No difference was found in average RQ in obese children vs controls (0.85 +/- 0.03 vs 0.87 +/- 0.03 respectively) and in the obese children before and after weight loss (0.87 +/- 0.02).(ABSTRACT TRUNCATED AT 250 WORDS)

AMCHP Issue Brief: Environmental Scan: State Title V Program Response to Autism Spectrum Disorder and Other Developmental Disabilities, December 2009

The increasing incidence of children identified and diagnosed with Autism Spectrum Disorders (ASD) and other developmental disabilities (DD) poses a major challenge to Title V and other programs as they try to meet the diverse and sometimes complex needs of these children. However, those state that have initiated coordinated efforts to meet the needs of these children cross systems have had the opportunity to form and/or strengthen relationships with new partners. In addition, these coordinated efforts will allow states to develop new policies, programs and financing mechanisms addressing the health of children with ASD, which may also strengthen the system of care for all Children and Youth with Special Health Care Needs.