¿Se han modificado las preferencias de los ciudadanos sobre las políticas de bienestar en España (1985-2005)?

Este trabajo es uno de los frutos empíricos del proyecto de investigación Reformas en el Estado de Bienestar: Actores y Apoyos Ciudadanos (REBAAC), http://www.iesam.csic.es/proyecto/rebaac.htm, dirigido por Luis Moreno, que se completará en los próximos meses con otros escritos sobre el tema.[EN] In recent years pressures for the reform of the Welfare State have been voiced despite that public opinion has kept opposed to the retrenchment of social policies. Some studies have pointed out that governments have learned how to avoid popular resistance to the reforms due to a change in citizens’ attitudes. This Working Paper explores the attitudes of the Spaniards with respect to the process and outcomes of the welfare policies implemented in Spain in the last 20 years. Whenever possible this paper takes a comparative. Time series have been worked out covering the period under analysis. Likewise, preferences towards the various welfare policies and these and other public policies are also compared. The two main conclusions are that the support for the welfare policies is solid (despite some qualifications) and that the public preference for the meso or intermediate level of government as provider of social policies has increased.[ES] En los últimos años parece que las presiones para la reforma del Estado de Bienestar están siendo más insistentes que una opinión pública hasta ahora opuesta a cualquier tipo de recorte de los programas sociales. Algunos estudios sugieren que los gobiernos parecen haber aprendido como esquivar la resistencia de los ciudadanos a las reformas aprovechando un supuesto cambio en sus actitudes durante los últimos años. Con esta hipótesis de cambio en las actitudes ciudadanas se exploran en este trabajo las preferencias de los españoles hacia el proceso y los resultados de las políticas del bienestar en los últimos veinte años. Siempre que es posible se sigue una estrategia comparativa. Por un lado, se han construido series temporales que cubren todo el periodo estudiado y, por otro, se comparan las preferencias entre las políticas de bienestar y entre éstas y el resto de las políticas públicas. Las dos conclusiones más significativas son que, si bien con algunos matices, el apoyo a la provisión pública del bienestar continúa siendo muy sólido y crece también la preferencia por el nivel intermedio de gobierno como proveedor de tales programas de bienestar

Development of phasic attention in children: Temporal analysis of alert during a detection task

This paper focuses on the maturation of the temporal aspect of phasic attention. 96 children (age range from 6 to 10 years) performed a detection task either alone or preceded by a visual alert signal. The Stimulus Onset Asynchrony (SOA) between the alert and the visual target was manipulated (100, 450 and 800 ms). Analysis of the mean RT (taking into account the SOA) and the response distribution (delta plot) converged on two points: (1) 6-year-old children experienced difficulties using the alert signal, and (2) alertness capacities emerged around the age of 7-8 years associated with a decrease of the delay beyond which the alert signal became efficient with increasing age. Distributional analysis distinguished erroneous or impulsive responses in reaction to the alert signal from those for which the alert was used correctly to prepare detection of the target.Le développement des aspects temporels de l’attention phasique a été étudié dans ce travail. 96 enfants (âgés de 6 à 10 ans) ont réalisé une tâche de détection simple précédée ou non par une alerte. L’intervalle de temps entre alertes et cibles visuelles était manipulé (“Stimulus Onset Asynchrony”: SOA de 100, 450 et 800 ms). L’analyse des temps de réponse (prenant en compte le SOA) et leur distribution (delta plot) convergent sur deux points : (1) à 6 ans, les enfants ne semblent pas capables d’utiliser efficacement l’alerte, et (2) les capacités d’alerte deviendraient efficientes à partir de 7-8 ans, pouvant être mobilisées de plus en plus rapidement à mesure que l’age des enfants augmente. L’analyse distributionnelle permet de distinguer les réponses erronées ou impulsives qui sont données en réponse à l’alerte de celles pour lesquelles l’alerte a été utilisée de façon correcte pour préparer la détection de la cible

Retrospective chart review study of use of cannabidiol (CBD) independent of concomitant clobazam use in patients with Lennox-Gastaut syndrome or Dravet syndrome

PURPOSE: This retrospective chart review study (GWEP20052) evaluated plant-derived highly purified cannabidiol (CBD; Epidyolex®; 100 mg/mL oral solution) use without clobazam as add-on therapy in patients aged ≥2 years with Lennox-Gastaut syndrome (LGS) or Dravet syndrome (DS) enrolled in a European Early Access Program. METHODS: Data were extracted from patient charts covering a period starting 3 months before CBD treatment and concluding after 12 months of CBD treatment, or sooner if a patient discontinued CBD or started clobazam. RESULTS: Of 114 enrolled patients, data were available for 107 (92 LGS, 15 DS) who received CBD without clobazam for ≥3 months. Mean age: 14.5 (LGS) and 10.5 (DS) years; female: 44% (LGS) and 67% (DS). Mean time-averaged CBD dose: 13.54 (LGS) and 11.56 (DS) mg/kg/day. Median change from baseline in seizure frequency per 28 days over 3-month intervals varied from -6.2% to -20.9% for LGS and 0% to -16.7% for DS. Achievement of ≥50% reduction in drop (LGS) or convulsive (DS) seizures at 3 and 12 months: LGS, 19% (n = 69) and 30% (n = 53); DS, 21% (n = 14) and 13% (n = 8). Retention on CBD without clobazam (enrolled set): 94%, 80%, 69%, and 63% at 3, 6, 9, and 12 months. Adverse event (AE) incidence was 31%, most commonly somnolence, seizure, diarrhea, and decreased appetite. Two patients discontinued CBD owing to AEs, and four patients with LGS experienced elevated liver enzymes. CONCLUSION: Results support favorable effectiveness and retention of CBD without concomitant clobazam for up to 12 months in clinical practice

Sporadic Infantile Epileptic Encephalopathy Caused by Mutations in PCDH19 Resembles Dravet Syndrome but Mainly Affects Females

Dravet syndrome (DS) is a genetically determined epileptic encephalopathy mainly caused by de novo mutations in the SCN1A gene. Since 2003, we have performed molecular analyses in a large series of patients with DS, 27% of whom were negative for mutations or rearrangements in SCN1A. In order to identify new genes responsible for the disorder in the SCN1A-negative patients, 41 probands were screened for micro-rearrangements with Illumina high-density SNP microarrays. A hemizygous deletion on chromosome Xq22.1, encompassing the PCDH19 gene, was found in one male patient. To confirm that PCDH19 is responsible for a Dravet-like syndrome, we sequenced its coding region in 73 additional SCN1A-negative patients. Nine different point mutations (four missense and five truncating mutations) were identified in 11 unrelated female patients. In addition, we demonstrated that the fibroblasts of our male patient were mosaic for the PCDH19 deletion. Patients with PCDH19 and SCN1A mutations had very similar clinical features including the association of early febrile and afebrile seizures, seizures occurring in clusters, developmental and language delays, behavioural disturbances, and cognitive regression. There were, however, slight but constant differences in the evolution of the patients, including fewer polymorphic seizures (in particular rare myoclonic jerks and atypical absences) in those with PCDH19 mutations. These results suggest that PCDH19 plays a major role in epileptic encephalopathies, with a clinical spectrum overlapping that of DS. This disorder mainly affects females. The identification of an affected mosaic male strongly supports the hypothesis that cellular interference is the pathogenic mechanism

Mutations and Deletions in PCDH19 Account for Various Familial or Isolated Epilepsies in Females

Mutations in PCDH19, encoding protocadherin 19 on chromosome X, cause familial epilepsy and mental retardation limited to females or Dravet-like syndrome. Heterozygous females are affected while hemizygous males are spared, this unusual mode of inheritance being probably due to a mechanism called cellular interference. To extend the mutational and clinical spectra associated with PCDH19, we screened 150 unrelated patients (113 females) with febrile and afebrile seizures for mutations or rearrangements in the gene. Fifteen novel point mutations were identified in 15 female patients (6 sporadic and 9 familial cases). In addition, qPCR revealed two whole gene deletions and one partial deletion in 3 sporadic female patients. Clinical features were highly variable but included almost constantly a high sensitivity to fever and clusters of brief seizures. Interestingly, cognitive functions were normal in several family members of 2 families: the familial condition in family 1 was suggestive of Generalized Epilepsy with Febrile Seizures Plus (GEFS+) whereas all three affected females had partial cryptogenic epilepsy. These results show that mutations in PCDH19 are a relatively frequent cause of epilepsy in females and should be considered even in absence of family history and/or mental retardation. © 2010 Wiley-Liss, Inc

Mutations in Eml1 lead to ectopic progenitors and neuronal heterotopia in mouse and human.

Neuronal migration disorders such as lissencephaly and subcortical band heterotopia are associated with epilepsy and intellectual disability. DCX, PAFAH1B1 and TUBA1A are mutated in these disorders; however, corresponding mouse mutants do not show heterotopic neurons in the neocortex. In contrast, spontaneously arisen HeCo mice display this phenotype, and our study revealed that misplaced apical progenitors contribute to heterotopia formation. While HeCo neurons migrated at the same speed as wild type, abnormally distributed dividing progenitors were found throughout the cortical wall from embryonic day 13. We identified Eml1, encoding a microtubule-associated protein, as the gene mutated in HeCo mice. Full-length transcripts were lacking as a result of a retrotransposon insertion in an intron. Eml1 knockdown mimicked the HeCo progenitor phenotype and reexpression rescued it. We further found EML1 to be mutated in ribbon-like heterotopia in humans. Our data link abnormal spindle orientations, ectopic progenitors and severe heterotopia in mouse and human

La stimulation du nerf vague dans le traitement de l'épilepsie

L'objectif de ce travail est l'évaluation de l'efficacité de la stimulation du nerf vague dans les épilepsies pharmacorésistantes non chirurgicales. L'étude porte sur 15 patients (14 enfants et 1 jeune adulte) implantés d'un stimulateur vagal, suivis dans le service de pédiatrie de Amiens. Les résultats mettent en évidence une réduction de la fréquence des crises et une amélioration de la qualité de vie dans les épilepsies partielles et généralisées à l'exclusion des spasmes. L'efficacité se maintient dans le temps. Les paramètres de stimulation optimaux semblent être une intensité comprise entre 1,5 et 2,5 mA, une durée ON de stimulation de 30 ou 60 secondes et une durée OFF comprise entre 1,1 et 5 minutes. Les effets secondaires cliniques sont peu importants et occasionnels. Ainsi la stimulation du nerf vague semble donc un traitement antiépileptique efficace, indiquée dans les épilepsies pharmacorésistante non chirurgicales, à l'exclusion des spasmesAMIENS-BU Santé (800212102) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

La thrombose du tronc basilaire chez l'enfant (à propos de deux cas)

OBJECTIFS : la thrombose du tronc basilaire est une pathologie rare à l origine d AVCI souvent gravissime chez l enfant et l adulte. Notre travail a deux objectifs : comprendre les caractéristiques et les spécificités de cette pathologie chez l enfant, et discuter l intérêt thérapeutique de la thrombolyse pendant phase aigue de ces AVCI en pédiatrie. MATERIELS ET METHODES : Après une revue générale de l AVCI chez l enfant, nous présentons et analysons deux cas pédiatriques de thrombose du tronc basilaire. Le 1er cas clinique rapporte une thrombose vertébro-basilaire d origine inconnue, chez une adolescente de 14 ans, dont l évolution a été particulièrement favorable après une héparinothérapie. Le 2ème cas clinique décrit une thrombose complète du tronc basilaire, dont l issue a été fatale, chez une adolescente de 14 ans, malgré l utilisation d un traitement thrombolytique. DISCUSSION ET CONCLUSIONS : Les AVCI secondaires à une thrombose du troc basilaire chez l enfant, se caractérisent par une clinique riche et peu spécifique et par un diagnostic et une prise en charge souvent tardifs. En ce qui concerne la thrombolyse, il semble légitime de la proposer, dans les cas graves ou le pronostic vital est engagé, compte tenu de son efficacité démontrée chez l adulte. Par ailleurs, notre travail met en évidence la nécessité de travaux s intéressant spécifiquement à cette pathologie et à l évaluation de l efficacité de la thrombolyse dans la population pédiatrique.AMIENS-BU Santé (800212102) / SudocSudocFranceF