The Patrimony Atlas of Seine-Saint-Denis

The Patrimony Atlas of Seine-Saint-Denis (north of Paris) is an information tool related to the archaeological, architectural and landscape patrimony of Seine-Saint-Denis, accessible on the Internet address http://www.atlas-patrimoine93.fr/. It is distributed by the Cultural Patrimony Service of the Department of Seine-Saint-Denis, and is registered in the National Patrimony Atlas project of the Ministry of Culture. It is organized into three categories. The documentation platforms gather: a geographical catalogue which includes 50 levels of information geographically referenced, which are free of copyrights and can circulate online; a bibliography with 3700 references classified according to borough, subject and time period; an iconographic catalogue with 2800 images, the issues of archaeological maps and patrimony inventory that collect and collate past data. The “territorial views” offer a rapid access to a selection of ancient maps that represent a selected point on the territory on a contemporary basis, like the Napoleonic cadastral tables. The “Documents” file gathers the documents in a PDF format: university projects, thematic studies, articles and monographs, methodologies, summary charts, as well as hypertext documents, a presentation of the evolution of the road network in the latter part of the 18th century, a “Mathériauthèque numérique” dedicated to construction materials, and an “Atlas des colleges” of Seine-Saint-Denis. In its present version (2.5.3, put online in March 2008), the Seine-Saint-Denis Patrimony Atlas receives between 12,000 and 14,000 hits per month

Efficient laser-overdense plasma coupling via surface plasma waves and steady magnetic field generation

International audienceThe efficiency of laser overdense plasma coupling via surface plasma wave excitation is investigated. Two-dimensional particle-in-cell simulations are performed over a wide range of laser pulse intensity from 10 15 to 10 20 W cm À2 lm 2 with electron density ranging from 25 to 100n c to describe the laser interaction with a grating target where a surface plasma wave excitation condition is fulfilled. The numerical studies confirm an efficient coupling with an enhancement of the laser absorption up to 75%. The simulations also show the presence of a localized, quasi-static magnetic field at the plasma surface. Two interaction regimes are identified for low (Ik 2 10 17 W cm À2 lm 2) laser pulse intensities. At " relativistic " laser intensity, steady magnetic fields as high as $580 MG lm/k 0 at 7 Â 10 19 W cm À2 lm 2 are obtained in the simulations

Neuroinflammation, Mast Cells, and Glia: Dangerous Liaisons

The perspective of neuroinflammation as an epiphenomenon following neuron damage is being replaced by the awareness of glia and their importance in neural functions and disorders. Systemic inflammation generates signals that communicate with the brain and leads to changes in metabolism and behavior, with microglia assuming a pro-inflammatory phenotype. Identification of potential peripheral-to-central cellular links is thus a critical step in designing effective therapeutics. Mast cells may fulfill such a role. These resident immune cells are found close to and within peripheral nerves and in brain parenchyma/meninges, where they exercise a key role in orchestrating the inflammatory process from initiation through chronic activation. Mast cells and glia engage in crosstalk that contributes to accelerate disease progression; such interactions become exaggerated with aging and increased cell sensitivity to stress. Emerging evidence for oligodendrocytes, independent of myelin and support of axonal integrity, points to their having strong immune functions, innate immune receptor expression, and production/response to chemokines and cytokines that modulate immune responses in the central nervous system while engaging in crosstalk with microglia and astrocytes. In this review, we summarize the findings related to our understanding of the biology and cellular signaling mechanisms of neuroinflammation, with emphasis on mast cell-glia interactions

Increase in Ocular Syphilis Cases at Ophthalmologic Reference Center, France, 2012–2015

We describe the frequency, demographic and clinical features, and visual outcomes of ocular syphilis infections observed during 2012–2015 at a tertiary reference center in Paris, France. Twenty-one cases (29 eyes) were identified. The occurrence of ocular syphilis increased from 1 case in 2012 to 5 cases in 2013, 6 cases in 2014, and 9 cases in 2015 (2.22–25.21/1,000 individual patients/year for the period). Among case-patients, an annual 20%–33% were co-infected with HIV. Seventy-six percent of ocular syphilis infections occurred in men who have sex with men. Seventy-five percent of case-patients had a good final visual outcome (best-corrected visual acuity >0.3 logMAR score). Visual outcome was worse for HIV-positive patients than for HIV-negative patients (p = 0.0139). At follow-up, the best visual outcomes were observed in patients whose mean time from first ocular symptom to consultation was 15 days (SD +19 days)

Creatine and guanidinoacetate reference values in a French population

Creatine and guanidinoacetate are biomarkers of creatine metabolism. Their assays in body fluids may be used for detecting patients with primary creatine deficiency disorders (PCDD), a class of inherited diseases. Their laboratory values in blood and urine may vary with age, requiring that reference normal values are given within the age range. Despite the long known role of creatine for muscle physiology, muscle signs are not necessarily the major complaint expressed by PCDD patients. These disorders drastically affect brain function inducing, in patients, intellectual disability, autistic behavior and other neurological signs (delays in speech and language, epilepsy, ataxia, dystonia and choreoathetosis), being a common feature the drop in brain creatine content. For this reason, screening of PCDD patients has been repeatedly carried out in populations with neurological signs. This report is aimed at providing reference laboratory values and related age ranges found for a large scale population of patients with neurological signs (more than 6 thousand patients) previously serving as a background population for screening French patients with PCDD. These reference laboratory values and age ranges compare rather favorably with literature values for healthy populations. Some differences are also observed, and female participants are discriminated from male participants as regards to urine but not blood values including creatine on creatinine ratio and guanidinoacetate on creatinine ratio values. Such gender differences were previously observed in healthy populations; they might be explained by literature differential effects of testosterone and estrogen in adolescents and adults, and by estrogen effects in prepubertal age on SLC6A8 function. Finally, though they were acquired on a population with neurological signs, the present data might reasonably serve as reference laboratory values in any future medical study exploring abnormalities of creatine metabolism and transport

Search for the best indicators for the presence of a VPS13B gene mutation and confirmation of diagnostic criteria in a series of 34 patients genotyped for suspected Cohen syndrome

BACKGROUND: Cohen syndrome is a rare autosomal recessive inherited disorder that results from mutations of the VPS13B gene. Clinical features consist of a combination of mental retardation, facial dysmorphism, postnatal microcephaly, truncal obesity, slender extremities, joint hyperextensibility, myopia, progressive chorioretinal dystrophy, and intermittent neutropenia.PATIENTS AND METHODS: The aim of the study was to determine which of the above clinical features were the best indicators for the presence of VPS13B gene mutations in a series of 34 patients with suspected Cohen syndrome referred for molecular analysis of VPS13B. RESULTS: 14 VPS13B gene mutations were identified in 12 patients, and no mutation was found in 22 patients. The presence of chorioretinal dystrophy (92% vs 32%, p=0.0023), intermittent neutropenia (92% vs 5%, p<0.001), and postnatal microcephaly (100% vs 48%, p=0.0045) was significantly higher in the group of patients with a VPS13B gene mutation compared to the group of patients without a mutation. All patients with VPS13B mutations had chorioretinal dystrophy and/or intermittent neutropenia. The Kolehmainen diagnostic criteria provided 100% sensibility and 77% specificity when applied to this series. CONCLUSION: From this study and a review of more than 160 genotyped cases from the literature, it is concluded that, given the large size of the gene, VPS13B screening is not indicated in the absence of chorioretinal dystrophy or neutropenia in patients aged over 5 years. The follow-up of young patients could be a satisfactory alternative unless there are some reproductive issues

Observation and modelling of stimulated Raman scattering driven by an optically smoothed laser beam in experimental conditions relevant for shock ignition

We report results and modelling of an experiment performed at the Target Area West Vulcan laser facility, aimed at investigating laser-plasma interaction in conditions that are of interest for the shock ignition scheme in inertial confinement fusion (ICF), that is, laser intensity higher than impinging on a hot (1$]]> keV), inhomogeneous and long scalelength pre-formed plasma. Measurements show a significant stimulated Raman scattering (SRS) backscattering (of laser energy) driven at low plasma densities and no signatures of two-plasmon decay (TPD)/SRS driven at the quarter critical density region. Results are satisfactorily reproduced by an analytical model accounting for the convective SRS growth in independent laser speckles, in conditions where the reflectivity is dominated by the contribution from the most intense speckles, where SRS becomes saturated. Analytical and kinetic simulations well reproduce the onset of SRS at low plasma densities in a regime strongly affected by non-linear Landau damping and by filamentation of the most intense laser speckles. The absence of TPD/SRS at higher densities is explained by pump depletion and plasma smoothing driven by filamentation. The prevalence of laser coupling in the low-density profile justifies the low temperature measured for hot electrons (keV), which is well reproduced by numerical simulations

Psychiatric and cognitive phenotype in children and adolescents with myotonic dystrophy

Myotonic dystrophy type 1 (DM1) is the most frequent inherited neuromuscular disorder. The juvenile form has been associated with cognitive and psychiatric dysfunction, but the phenotype remains unclear. We reviewed the literature to examine the psychiatric phenotype of juvenile DM1 and performed an admixture analysis of the IQ distribution of our own patients, as we hypothesised a bimodal distribution. Two-thirds of the patients had at least one DSM-IV diagnosis, mainly attention deficit/ hyperactivity disorder and anxiety disorder. Two-thirds had learning disabilities comorbid with mental retardation on one hand, but also attention deficit, low cognitive speed and visual spatial impairment on the other. IQ showed a bi-modal distribution and was associated with parental transmission. The psychiatric phenotype in juvenile DM1 is complex. We distinguished two different phenotypic subtypes: one group characterised by mental retardation, severe developmental delay and maternal transmission; and another group characterised by borderline full scale IQ, subnormal development and paternal transmission

SLC35A2â CDG: Functional characterization, expanded molecular, clinical, and biochemical phenotypes of 30 unreported Individuals

Pathogenic de novo variants in the Xâ linked gene SLC35A2 encoding the major Golgiâ localized UDPâ galactose transporter required for proper protein and lipid glycosylation cause a rare type of congenital disorder of glycosylation known as SLC35A2â congenital disorders of glycosylation (CDG; formerly CDGâ IIm). To date, 29 unique de novo variants from 32 unrelated individuals have been described in the literature. The majority of affected individuals are primarily characterized by varying degrees of neurological impairments with or without skeletal abnormalities. Surprisingly, most affected individuals do not show abnormalities in serum transferrin Nâ glycosylation, a common biomarker for most types of CDG. Here we present data characterizing 30 individuals and add 26 new variants, the single largest study involving SLC35A2â CDG. The great majority of these individuals had normal transferrin glycosylation. In addition, expanding the molecular and clinical spectrum of this rare disorder, we developed a robust and reliable biochemical assay to assess SLC35A2â dependent UDPâ galactose transport activity in primary fibroblasts. Finally, we show that transport activity is directly correlated to the ratio of wildâ type to mutant alleles in fibroblasts from affected individuals.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/150498/1/humu23731_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/150498/2/humu23731-sup-0001-Supp_Mat__2019.2.10_.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/150498/3/humu23731.pd