Confluent Thalamic Hyperintensities in CADASIL

Background: CADASIL is responsible for diffuse hyperintensities in the white matter on FLAIR images. These lesions are often associated with focal lesions in the basal ganglia such as lacunar infarctions. The prevalence and significance of diffuse or confluent thalamic hyperintensities (CTH) remain unknown. Methods: The frequency of hyperintensities on FLAIR images in the thalamus was assessed in 147 CADASIL patients, and signal abnormalities on both FLAIR and T(1)-weighted images were categorized as focal/punctuate or diffuse/confluent by the same reader. The areas of increased diffusion were also analyzed on apparent diffusion coefficient maps. The association of CTH with vascular risk factors, the main clinical manifestations of the disease and MRI markers (brain parenchymal fraction, volume of white matter hyperintensities, volume of lacunar infarcts and number of microbleeds) was analyzed with generalized linear regression models. Results: CTH were detected in 12% of the CADASIL subjects in association with hypointensities on T(1)-weighted images. CTH corresponded to areas of increased diffusion apparent diffusion coefficient maps. CTH were found significantly associated with age and independently related to the volume of white matter hyperintensities but not to that of lacunar infarctions or to cerebral atrophy after adjustment for age and sex. No significant association was found between CTH and global cognitive performances. Conclusion: CTH are observed on FLAIR images in a sizeable proportion of CADASIL patients. They are mainly related to the extent of white matter hyperintensities and do not correlate with cognitive decline. Demyelination and/or loss of glial cells appear to be the most plausible cause of these confluent signal changes in the thalamus. Copyright (C) 2010 S. Karger AG, Base

Quelques éléments sur le thermalisme dans la Sicile et l'Espagne musulmanes

Aunque se trata de un coloquio esencialmente consagrado a la AntigiJedad, queremos presentar algunas referencias médiévales, principalmente isiâmicas. Conviene tener en cuenta que si el mundo musulman ha tomado prestado mucho de la Antiguedad, algunos aspectos bâsicos de su civilizaciôn son totalmente opuestos. La relaciôn estrecha que existia en el mundo antiguo entre el agua y las creencias o prâcticas religiosas, de una parte, y lo récréative, por otra, no tienen este sentido en el mundo musulman médiéval. La utilizaciôn del agua, en el sentido de abluciôn, es necesaria para la purificaciôn del creyente para la oraciôn. En principio, el Islam excluye todo nexo entre el agua y lo sagrado. Se mantiene la utilizaciôn del agua para banarse, prâctica que pasa del mundo antiguo al mundo musulman médiéval, trente al olvido, en parte, del mundo cristiano. Existen referencias a los bafios en gênerai como la de Ibn al-Kalib, politico granadino del sigio XIV; o la del geôgrafo al-ldirisi, consagrada a las aguas termales de Alhama de Almeria, sobre las que trata también al-Himyari, con gran detalle. También hay alusiones a Alhama de Murcia y a termas en Sicilia, ademâs de a los frescos del palacio de Qusayr 'Amra (Jordania)

Radiochirurgie des malformations arterio-veineuses du tronc cerebral stereotactic radiosurgery for brainstem arteriovenous malformations

Introduction: Les malformations artério-veineuses ou angiomes cérébraux sont des anomalies vasculaires congénitales qui se définissent par la présence d’un shunt artério-veineux anormal.Matériels et méthodes: Nous avons mené une étude rétrospective entre Janvier 1992 et Décembre 2010 au service de radiochirurgie de l’hôpital de la Timone, 1557 patients ont été traités par radiochirurgie pour une malformation artérioveineuse dont 61 patients pour une localisation du tronc cérébral (3.91%).Résultats: L’âge moyen de 35.8-/+16.6 ans (4-75).16 patients avaient bénéficié d’une embolisation préradiochirurgicale (26.23%). Le mode de découverte était une hémorragie pour 53 patients (87%). Le volume moyen de traitement était de 1.43+/-1.96 cm3 (0.09-14.20). Le score RBAS était en moyenne de 1.36 +/- 0.34 (0.64-2.35). La dose moyenne de prescription à l’enveloppe l’isodose 50% délivrée était de 22.9 +/-2.9 Gy (12-30), la dose médiane était de 24 Gy).Notre taux d’oblitération global après une ou deux procédures était de 67.27%, pour un délai moyen de suivi est de 56.7 mois+/-43.3 (8.1-251.7). Les facteurs en rapport avec une oblitération) : score RBAS bas (p=0.012), une dose de traitement supérieur à 20Gy (p=0.034). Nous avons eu une mortalité de 1.63% et un pourcentage de saignement après la procédure de 6.55% et 3.2% déficits neurologiques radio-induits.Conclusion: La radiochirurgie est maintenant devenue incontournable dans le traitement des malformations du tronc cérébral même s’il existe un risque hémorragique persistant jusqu’à la guérison (3.5%). Le taux d’oblitération complète est de 67.27%Mots clés: malformations artério-veineuses, tronc cérébral, radiochirurugieEnglish Title: Stereotactic radiosurgery for brainstem arteriovenous malformationsEnglish AbstractIntroduction: Arterioveinous malformations (AVM) or cerebral angiomas are congenital vascular abnormalities which are defined by the presence of abnormal arterio-veinous shunt.Methods:  We lead a retrospective study recruiting 1557 patients treated between January 1992 and December 2010 in the Radiosurgery Unit of La Timone hospital in Marseille. All patients received radiosurgery for management of an AVM, 61 of them being brainstem AVMs (3.91%).Results: Mean age was 35.8 +/- 16.6 years. Sixteen patients underwent arterial embolization before surgery (26.23%). Hemorrhage was the primary onset for 53 patients (87%). Mean volume before treatment was 1.43+/-1.96 cm3 (0.09-14.20). Average RBAS score was 1.36 +/-0.34 (0.64-2.35). Average prescripted dose for 50% delivered iso-dose was 22.9 +/-2.9 Gy (12-30), mean dose was 24 Gy. Our global occlusion rate after one or two treatments was 67.27%, considering an average follow-up of 56.7 months +/- 43.3 (8.1- 251.7). Occlusion related factors were: low RBAS score (p=0.012), received dose higher than 20Gy (p=0.034). Our death rate was 1.63% and our post-procedure bleeding rate was 6.55% with 3.2% of neurological post-procedure deficience.Conclusion: Having regard of remaining hemorrhage risk until complete recovery (3.5%), radiosurgery is nonetheless now a key treatment in the management brainstem AVMS. Complete occlusion rate in our study was 67.27%.Keywords: arteriovenous malformations, brainstem, radiosurger

J Clin Sleep Med

Non-24-hour sleep-wake disorder is 1 of several chronic circadian rhythm sleep-wake disorders. It is defined as progressive daily shifts in sleep onset and wake times. It mainly affects patients who are sight-impaired, is relatively rare in sighted patients, and is difficult to treat, with no guidelines. This case report discusses non-24-hour sleep-wake disorder in a sighted young man who complained of alternating severe insomnia and excessive sleepiness, with a sleep agenda and actigraphic data showing a daily delay of approximately 2 hours. A novel therapy by total sleep deprivation followed by a combination of morning light therapy and nocturnal melatonin administration was efficient in stopping his free-running sleep-wake pattern both immediately and in the long term. The treatment combination for 6 months resulted in stable circadian entrainment to a 24-hour cycle. Compliance with chronotherapy was maintained over the course of follow-up

Dilated perivascular spaces in small-vessel disease: A study in CADASIL

BACKGROUND AND AIM Dilated perivascular spaces (dPVS) have previously been associated with aging and hypertension-related cerebral microangiopathy. However, their risk factors, radiological features and clinical relevance have been poorly evaluated in CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy), a unique model to investigate the pathophysiology of ischemic small-vessel disease. The purpose of this study was to investigate these different aspects in a large cohort of patients with this disorder. METHODS Demographic and MRI data of 344 patients from a prospective cohort study were analyzed. The severity of dPVS was evaluated separately in the anterior temporal lobes, subinsular areas, basal ganglia and white matter, using validated semiquantitative scales. Logistic and multiple linear regression models were used to determine the risk factors associated with the severity of dPVS in these different regions and their relationships with cognition, disability and the MRI markers of the disease (white matter hyperintensities (WMH) lacunar infarcts, microbleeds and brain parenchymal fraction (BPF)). RESULTS The severity of dPVS was found to increase with age regardless of cerebral area (p\textless0.001). In contrast with dPVS in other locations, the severity of dPVS in the temporal lobes or subinsular areas was also found strongly and specifically related to the extent of WMH (p\textless0.001). Conversely, no significant association was detected with lacunar volume, number of microbleeds or BPF. A high degree of dPVS in the white matter was associated with lower cognitive performances independently of age and other MRI markers of the disease including BPF (p≤0.04). CONCLUSIONS In CADASIL, the progression of the hereditary microangiopathy with aging may promote the dilation of perivascular spaces throughout the whole brain but with variable extent according to cerebral location. In temporal lobes and subinsular areas, dPVS are common MRI features and may share a similar pathogenesis with the extension of WMH during the course of the disease. dPVS may also participate in the development of cognitive decline in this model of small-vessel disease, and their large number in white matter may alert clinicians to a higher risk of cognitive decline in CADASIL

Construire sa vie (Life designing) : un paradigme pour l’orientation au 21e siècle

Ce texte est la traduction française de l’article publié dans le Journal of Vocational Behavior, 75(3) : Savickas, M. L., Nota, L., Rossier, J., Dauwalder, J.-P., Duarte, M. E., Guichard, J., Soresi, S., Van Esbroeck, R., & van Vianen, A. E. M., « Life designing: A paradigm for career construction in the 21st century » (239-250), © 2009, avec la permission de Elsevier. http://www.sciencedirect.com/science/journal/00018791 ***** La nouvelle organisation sociale du travail du début du 21e siècle pose une série de questions et lance de nombreux défis aux spécialistes de l’accompagnement en orientation professionnelle. Compte tenu de la mondialisation du conseil en orientation, c’est dans un cadre international que nous avons décidé d’aborder ces questions, puis de formuler des réponses potentiellement innovantes. Cette approche permet d’éviter les difficultés que soulève la création de modèles et de méthodes dans un pays donné en tentant ensuite de les exporter dans d’autres, où ils doivent être adaptés pour être utilisés. Cet article présente le premier résultat tangible de cette collaboration : un modèle et des méthodes d’accompagnement des personnes. Le modèle « Construire sa vie », destiné à des interventions d’accompagnement en orientation se fonde sur cinq présupposés concernant les personnes et leur vie professionnelle : des possibilités liées aux contextes, des processus dynamiques, une progression non linéaire, des perspectives multiples et des configurations individuelles. En partant de ces présupposés, nous avons bâti un modèle en contexte, se réclamant de l’épistémologie du constructionnisme social, reconnaissant en particulier que les connaissances et l’identité d’un individu sont le produit d’interactions sociales et que le sens est co-construit, via la médiation du discours. Le cadre général « construire sa vie » s’appuie sur les théories de la construction de soi (Guichard, 2005) et de la construction des parcours professionnels (Savickas, 2005), qui décrivent les conduites d’orientation et leurs développements. Ce cadre concerne toute la vie, il est holiste, tient compte des contextes et est préventif.At the beginning of the 21st century, a new social arrangement of work poses a series of questions and challenges to scholars who aim to help people develop their working lives. Given the globalization of career counseling, we decided to address these issues and then to formulate potentially innovative responses in an international forum. We used this approach to avoid the difficulties of creating models and methods in one country and then trying to export them to other countries where they would be adapted for use. This article presents the initial outcome of this collaboration, a counseling model and methods. The life-designing model for career intervention endorses five presuppositions about people and their work lives: contextual possibilities, dynamic processes, non-linear progression, multiple perspectives, and personal patterns. Thinking from these five presuppositions, we have crafted a contextualized model based on the epistemology of social constructionism, particularly recognizing that an individual’s knowledge and identity are the product of social interaction and that meaning is co-constructed through discourse. The life-design framework for counseling implements the theories of self-constructing (Guichard, 2005) and career construction (Savickas, 2005) that describe vocational behavior and its development. Thus, the framework is structured to be life-long, holistic, contextual, and preventive

Patterns of sequence polymorphism in the fleshless berry locus in cultivated and wild Vitis vinifera accessions

<p>Abstract</p> <p>Background</p> <p>Unlike in tomato, little is known about the genetic and molecular control of fleshy fruit development of perennial fruit trees like grapevine (<it>Vitis vinifera </it>L.). Here we present the study of the sequence polymorphism in a 1 Mb grapevine genome region at the top of chromosome 18 carrying the <it>fleshless berry </it>mutation (<it>flb</it>) in order, first to identify SNP markers closely linked to the gene and second to search for possible signatures of domestication.</p> <p>Results</p> <p>In total, 62 regions (17 SSR, 3 SNP, 1 CAPS and 41 re-sequenced gene fragments) were scanned for polymorphism along a 3.4 Mb interval (85,127-3,506,060 bp) at the top of the chromosome 18, in both <it>V. vinifera cv</it>. Chardonnay and a genotype carrying the <it>flb </it>mutation, <it>V. vinifera cv</it>. Ugni Blanc mutant. A nearly complete homozygosity in Ugni Blanc (wild and mutant forms) and an expected high level of heterozygosity in Chardonnay were revealed. Experiments using qPCR and BAC FISH confirmed the observed homozygosity. Under the assumption that <it>flb </it>could be one of the genes involved into the domestication syndrome of grapevine, we sequenced 69 gene fragments, spread over the <it>flb </it>region, representing 48,874 bp in a highly diverse set of cultivated and wild <it>V. vinifera </it>genotypes, to identify possible signatures of domestication in the cultivated <it>V. vinifera </it>compartment. We identified eight gene fragments presenting a significant deviation from neutrality of the Tajima's D parameter in the cultivated pool. One of these also showed higher nucleotide diversity in the wild compartments than in the cultivated compartments. In addition, SNPs significantly associated to berry weight variation were identified in the <it>flb </it>region.</p> <p>Conclusions</p> <p>We observed the occurrence of a large homozygous region in a non-repetitive region of the grapevine otherwise highly-heterozygous genome and propose a hypothesis for its formation. We demonstrated the feasibility to apply BAC FISH on the very small grapevine chromosomes and provided a specific probe for the identification of chromosome 18 on a cytogenetic map. We evidenced genes showing putative signatures of selection and SNPs significantly associated with berry weight variation in the <it>flb </it>region. In addition, we provided to the community 554 SNPs at the top of chromosome 18 for the development of a genotyping chip for future fine mapping of the <it>flb </it>gene in a F2 population when available.</p

Evolution of Surface Hydrology in the Sahelo-Sudanian Strip: An Updated Review

In the West African Sahel, two paradoxical hydrological behaviors have occurred during the last five decades. The first paradox was observed during the 1968–1990s ‘Great Drought’ period, during which runoff significantly increased. The second paradox appeared during the subsequent period of rainfall recovery (i.e., since the 1990s), during which the runoff coefficient continued to increase despite the general re-greening of the Sahel. This paper reviews and synthesizes the literature on the drivers of these paradoxical behaviors, focusing on recent works in the West African Sahelo/Sudanian strip, and upscaling the hydrological processes through an analysis of recent data from two representative areas of this region. This paper helps better determine the respective roles played by Land Use/Land Cover Changes (LULCC), the evolution of rainfall intensity and the occurrence of extreme rainfall events in these hydrological paradoxes. Both the literature review and recent data converge in indicating that the first Sahelian hydrological paradox was mostly driven by LULCC, while the second paradox has been caused by both LULCC and climate evolution, mainly the recent increase in rainfall intensity

ADC Histograms from Routine DWI for Longitudinal Studies in Cerebral Small Vessel Disease: A Field Study in CADASIL.

Diffusion tensor imaging (DTI) histogram metrics are correlated with clinical parameters in cerebral small vessel diseases (cSVD). Whether ADC histogram parameters derived from simple diffusion weighted imaging (DWI) can provide relevant markers for long term studies of cSVD remains unknown. CADASIL patients were evaluated by DWI and DTI in a large cohort study overa6-year period. ADC histogram parameters were compared to those derived from mean diffusivity (MD) histograms in 280 patients using intra-class correlation and Bland-Altman plots. Impact of image corrections applied to ADC maps was assessed and a mixed effect model was used for analyzing the effects of scanner upgrades. The results showed that ADC histogram parameters are strongly correlated to MD histogram parameters and that image corrections have only limited influence on these results. Unexpectedly, scanner upgrades were found to have major effects on diffusion measures with DWI or DTI that can be even larger than those related to patients' characteristics. These data support that ADC histograms from daily used DWI can provide relevant parameters for assessing cSVD, but the variability related to scanner upgrades as regularly performed in clinical centers should be determined precisely for longitudinal and multicentric studies using diffusion MRI in cSVD

The BLLAST field experiment: Boundary-Layer late afternoon and sunset turbulence

Due to the major role of the sun in heating the earth's surface, the atmospheric planetary boundary layer over land is inherently marked by a diurnal cycle. The afternoon transition, the period of the day that connects the daytime dry convective boundary layer to the night-time stable boundary layer, still has a number of unanswered scientific questions. This phase of the diurnal cycle is challenging from both modelling and observational perspectives: it is transitory, most of the forcings are small or null and the turbulence regime changes from fully convective, close to homogeneous and isotropic, toward a more heterogeneous and intermittent state. These issues motivated the BLLAST (Boundary-Layer Late Afternoon and Sunset Turbulence) field campaign that was conducted from 14 June to 8 July 2011 in southern France, in an area of complex and heterogeneous terrain. A wide range of instrumented platforms including full-size aircraft, remotely piloted aircraft systems, remote-sensing instruments, radiosoundings, tethered balloons, surface flux stations and various meteorological towers were deployed over different surface types. The boundary layer, from the earth's surface to the free troposphere, was probed during the entire day, with a focus and intense observation periods that were conducted from midday until sunset. The BLLAST field campaign also provided an opportunity to test innovative measurement systems, such as new miniaturized sensors, and a new technique for frequent radiosoundings of the low troposphere. Twelve fair weather days displaying various meteorological conditions were extensively documented during the field experiment. The boundary-layer growth varied from one day to another depending on many contributions including stability, advection, subsidence, the state of the previous day's residual layer, as well as local, meso- or synoptic scale conditions. Ground-based measurements combined with tethered-balloon and airborne observations captured the turbulence decay from the surface throughout the whole boundary layer and documented the evolution of the turbulence characteristic length scales during the transition period. Closely integrated with the field experiment, numerical studies are now underway with a complete hierarchy of models to support the data interpretation and improve the model representations.publishedVersio