Variants Cause Spastic Paraplegia Associated with Cerebral Hypomyelination

Oculodentodigital dysplasia is an autosomal dominant disorder due to variants characterized by dysmorphic features. Neurologic symptoms have been described in some patients but without a clear neuroimaging pattern. To understand the pathophysiology underlying neurologic deficits in oculodentodigital dysplasia, we studied 8 consecutive patients presenting with hereditary spastic paraplegia due to variants. Clinical disease severity was highly variable. Cerebral MR imaging revealed variable white matter abnormalities, consistent with a hypomyelination pattern, and bilateral hypointense signal of the basal ganglia on T2-weighted images and/or magnetic susceptibility sequences, as seen in neurodegeneration with brain iron accumulation diseases. Patients with the more prominent basal ganglia abnormalities were the most disabled ones. This study suggests that -related hereditary spastic paraplegia is a complex neurodegenerative disease affecting both the myelin and the basal ganglia. variants should be considered in patients with hereditary spastic paraplegia presenting with brain hypomyelination, especially if associated with neurodegeneration and a brain iron accumulation pattern

How does fusion hindrance show up in medium-light systems? The case of 48Ca + 48Ca

The fusion excitation function of 48Ca + 48Ca has been measured above and well below the Coulomb barrier, thereby largely extending the energy range of a previous experiment down to very low cross sections. This system has a negative Q-value for compound nucleus formation. The fusion cross section decreases steadily below the barrier with no conspicuous change of slope below 300 μb. Coupled-channels calculations using a Woods–Saxon potential indicate that a large diffuseness parameter is needed to reproduce the sub-barrier cross sections. A close analogy with the case of 36S + 48Ca, with Q>0, is pointed out. The sign of the Q-value does not influence fusion cross sections down to the 300–600 nb leve

Selected nondigestible carbohydrates and prebiotics support the growth of probiotic fish bacteria mono-cultures in vitro

To search for nondigestible but fermentable (NDF) carbohydrates and prebiotics with a potency to promote the growth of selected bacteria in vitro. The growth of three reference bacteria strains Bacillus subtilis LMG 7135(T), Carnobacterium piscicola LMG 9839, Lactobacillus plantarum LMG 9211 and one candidate probiotic bacteria Lactobacillus delbrueckii subsp. lactis was investigated over a minimum period of 48 h in the presence of beta-glucan, xylo-oligosaccharide, arabinoxylo-oligosaccharide, inulin, oligofructose and glucose. Besides the capability to grow on inulin and oligofructose containing media, a distinct high growth in beta-glucan based substrates and a low growth in (arabino)xylooligosaccharide containing media were evident for most bacteria tested. With the exception of B. subtilis and L. plantarum, other bacteria grew equally well or even better on different substrates than on glucose. The fermentation of studied carbohydrates by these micro-organisms was dominated by the production of acetic acid as the main short chain fatty acid. Selected bacteria are able to ferment and grow on NDF and prebiotic carbohydrates but in a substrate dependent manner. This study delivers a first screening of which NDF or prebiotic carbohydrates are the most promising for aquaculture feed supplementations

Restricted Application of Insecticides: A Promising Tsetse Control Technique, but What Do the Farmers Think of It?

Restricted application of insecticides to cattle is a cheap and safe farmer-based method to control tsetse and the diseases they transmit, i.e. human and animal African trypanosomoses. The efficiency of this new control method has been demonstrated earlier but no data is available on its perception and adoption intensity by farmers. We studied these two features in Burkina Faso, where the method has diffused thanks to two development projects. The study allowed identifying three groups of farmers with various adoption intensities, of which one was modern and two traditional. The economic benefit and the farmers' knowledge of the epidemiological system appeared to have a low impact on the early adoption process whereas some modern practices, as well as social factors appeared critical. The quality of technical support provided to the farmers had also a great influence on the adoption rate. The study highlighted individual variations in risk perceptions and benefits, as well as the prominent role of the socio-technical network of cattle farmers. The results of the study are discussed to highlight the factors that should be taken into consideration, to move discoveries from bench to field for an improved control of trypanosomoses vectors

Deformation of the N=Z nucleus 76Sr using beta-decay studies

A novel method of deducing the deformation of the N=Z nucleus 76Sr is presented. It is based on the comparison of the experimental Gamow-Teller strength distribution B(GT) from its beta decay with the results of QRPA calculations. This method confirms previous indications of the strong prolate deformation of this nucleus in a totally independent way. The measurement has been carried out with a large Total Absorption gamma Spectrometer, "Lucrecia", newly installed at CERN-ISOLDE.Comment: Accepted in Phys. Rev. Letter

Binary reaction decays from 24Mg+12C

Charged particle and gamma decays in 24Mg* are investigated for excitation energies where quasimolecular resonances appear in 12C+12C collisions. Various theoretical predictions for the occurence of superdeformed and hyperdeformed bands associated with resonance structures with low spin are discussed within the measured 24Mg* excitation energy region. The inverse kinematics reaction 24Mg+12C is studied at E_lab(24Mg) = 130 MeV, an energy which enables the population of 24Mg states decaying into 12C+12C resonant break-up states. Exclusive data were collected with the Binary Reaction Spectrometer in coincidence with EUROBALL IV installed at the VIVITRON Tandem facility at Strasbourg. Specific structures with large deformation were selectively populated in binary reactions and their associated gamma decays studied. Coincident events associated with inelastic and alpha-transfer channels have been selected by choosing the excitation energy or the entry point via the two-body Q-values. The analysis of the binary reaction channels is presented with a particular emphasis on 24Mg-gamma, 20Ne-gamma and 16O-gamma coincidences. New information (spin and branching ratios) is deduced on high-energy states in 24Mg and 16O, respectively.Comment: 27 pages, 8 figures, 1 tabl

Reaction mechanisms in 24Mg+12C and 32S+24Mg

The occurence of "exotic" shapes in light N=Z alpha-like nuclei is investigated for 24Mg+12C and 32S+24Mg. Various approaches of superdeformed and hyperdeformed bands associated with quasimolecular resonant structures with low spin are presented. For both reactions, exclusive data were collected with the Binary Reaction Spectrometer in coincidence with EUROBALL IV installed at the VIVITRON Tandem facility of Strasbourg. Specific structures with large deformation were selectively populated in binary reactions and their associated $\gamma$-decays studied. The analysis of the binary and ternary reaction channels is discussed.Comment: 7 pages, 4 figures, Paper presented at the Fusion08 International Conference on New Aspects of Heavy Ion Collisions Near the Coulomb Barrier, Chicago. Proceedings to be published by AIP Conference Proceedings Illinois, USA, September 22-26, 200

Search for highly excited states in 28Si

The theoretical and experimental determination of superdeformed states in nuclei in the mass region A≤40 has been since a long time one of the major challenges of nuclear structure studies. Despite the considerable experimental and theoretical work dedicated to this topic, up to now superdeformed bands have been found in only two nuclei, 36Ar and 40Ca. While the experimental signature of the superdeformed nature of those states is irrefutable, their theoretical interpretation is still uncertain. In particular, it is not clear whether clusterisation is responsible of the onset of superdeformation. For this reason, we wanted to investigate an even lighter system, 28Si, where a number of theoretical calculations predict the presence of superdeformation as an effect of the cluster structure of the nucleus

Clusters in Light Nuclei

A great deal of research work has been undertaken in the alpha-clustering study since the pioneering discovery, half a century ago, of 12C+12C molecular resonances. Our knowledge of the field of the physics of nuclear molecules has increased considerably and nuclear clustering remains one of the most fruitful domains of nuclear physics, facing some of the greatest challenges and opportunities in the years ahead. In this work, the occurence of "exotic" shapes in light N=Z alpha-like nuclei is investigated. Various approaches of superdeformed and hyperdeformed bands associated with quasimolecular resonant structures are presented. Results on clustering aspects are also discussed for light neutron-rich Oxygen isotopes.Comment: 12 pages, 5 figures. Invited Talk presented by C. Beck at the Zakopane Conference on Nuclear Physics "Extremes of the Nuclear Landscape" XLV in the series of Zakopane Schools of Physics - International Symposium - Zakopane, Poland, August 30 - September 5, 2010.To be publihed in Acta Physica Polonica B42 no 3, March 201

Fusion reactions of 58,64Ni+124Sn

International audience; In order to better understand the influence of transfer in sub-barrier nuclear reactions, cross sections for the system ^58,64Ni+^124Sn have been measured down to 0.5-1 mub and compared to detailed coupledchannel calculations. In agreement with a phenomenological Q-value systematics, calculations show the importance of including the coupling to the transfer channel for these heavy systems. No clear evidence of fusion hindrance is observed, probably due to the fact that the cross sections measured in this experiment are not low enough for the appearance of that phenomenon