Early Globus Pallidus Internus Stimulation in Pediatric Patients With Generalized Primary Dystonia: Long-Term Efficacy and Safety

Primary generalized dystonia presents mainly at a young age and commonly is severely disabling. The authors report the long-term follow-up (mean, 73 months; range, 50-101 months) of 5 pediatric patients (mean age at surgery 13 years; range, 8-16 years) undergoing globus pallidus internus deep brain stimulation. Mean improvement in the Burke-Fahn-Marsden movement score was 67.4% (range, 47.0%-87.5%), 75.4% (range, 61.5%-91.7%), and 83.5% (range, 72.0%-93.3%) at 3 months, 12 months, and long-term follow-up (>36 months), respectively. Hardware problems (electrode dislocation/breakage of extension cable, and imminent perforation of extension cable) were observed in 2 patients (operative revision without sequelae). Except for mild dysarthria in 2 patients, no other therapy-related morbidity was observed. The authors found globus pallidus internus stimulation to offer a very effective and safe therapy in pediatric patients with primary dystonia. Early neurosurgical intervention seems to be crucial to prevent irreversible impairment of motor function

FOOD HABITS OF MOUNTAIN LIONS IN THE TRANS-PECOS REGION OF TEXAS

Information regarding mountain lion (Felis concolor) food habits is relatively scarce overall, and this is particularly true in the Trans-Pecos region of Texas. Most information currently available is from Big Bend National Park where livestock are excluded and game animals are not actively managed. This study involved the analysis of 32 mountain lion stomachs collected throughout the Trans-Pecos over a 14 month period. Deer (Odocoileus spp.) and javelina (Tayassu tajacu) were the predominate prey species, each occurring in 39% of the stomachs analyzed. Domestic livestock was found in 9% of the total stomachs and non-game wildlife in 13%. Samples taken from areas with and without livestock differed significantly (P\u3c0.05). Samples from areas with livestock contained deer (50%), javelina (19%), small game (19%), and livestock (12%). Samples from areas without livestock contained javelina (86%) and deer (14%). No differences (P\u3e0.05) in food habits were found between sexes or among seasons of the year

FOOD HABITS OF MOUNTAIN LIONS IN THE TRANS-PECOS REGION OF TEXAS

Information regarding mountain lion (Felis concolor) food habits is relatively scarce overall, and this is particularly true in the Trans-Pecos region of Texas. Most information currently available is from Big Bend National Park where livestock are excluded and game animals are not actively managed. This study involved the analysis of 32 mountain lion stomachs collected throughout the Trans-Pecos over a 14 month period. Deer (Odocoileus spp.) and javelina (Tayassu tajacu) were the predominate prey species, each occurring in 39% of the stomachs analyzed. Domestic livestock was found in 9% of the total stomachs and non-game wildlife in 13%. Samples taken from areas with and without livestock differed significantly (P\u3c0.05). Samples from areas with livestock contained deer (50%), javelina (19%), small game (19%), and livestock (12%). Samples from areas without livestock contained javelina (86%) and deer (14%). No differences (P\u3e0.05) in food habits were found between sexes or among seasons of the year

Loop-shaping for reset control systems -- A higher-order sinusoidal-input describing functions approach

The ever-growing demands on speed and precision from the precision motion industry have pushed control requirements to reach the limitations of linear control theory. Nonlinear controllers like reset provide a viable alternative since they can be easily integrated into the existing linear controller structure and designed using industry-preferred loop-shaping techniques. However, currently, loop-shaping is achieved using the describing function (DF) and performance analysed using linear control sensitivity functions not applicable for reset control systems, resulting in a significant deviation between expected and practical results. We overcome this major bottleneck to the wider adaptation of reset control with two contributions in this paper. First, we present the extension of frequency-domain tools for reset controllers in the form of higher-order sinusoidal-input describing functions (HOSIDFs) providing greater insight into their behaviour. Second, we propose a novel method which uses the DF and HOSIDFs of the open-loop reset control system for the estimation of the closed-loop sensitivity functions, establishing for the first time - the relation between open-loop and closed-loop behaviour of reset control systems in the frequency domain. The accuracy of the proposed solution is verified in both simulation and practice on a precision positioning stage and these results are further analysed to obtain insights into the tuning considerations for reset controllers

Lynch Syndrome-associated Mutations in MSH2 Alter DNA Repair and Checkpoint Response Functions In Vivo

The DNA mismatch repair (MMR) pathway is essential in maintaining genomic stability through its role in DNA repair and the checkpoint response. Loss of DNA MMR underlies the hereditary cancer disease Lynch Syndrome (LS). Germline mutations in MSH2 account for approximately 40% of LS patients and of these, 18% are missense variants. One important clinical challenge has been discriminating between missense variants that are pathogenic and those that are not. Current analysis of missense mutations in MSH2 is performed using a combination of clinical, biochemical, and functional data; however, suitable cell culture models to test the various functions of the DNA MMR proteins are lacking. Here, we have generated human cell lines stably expressing a subset of MSH2 missense mutants and tested their effect on DNA repair and checkpoint response functions. We have expanded on previous biochemical and functional analyses performed in non-human systems to further understand defects conferred by this subset of single amino acid alterations. The functional characterization of MSH2 missense mutants combined with clinical and biochemical data is essential for appropriate patient management and genetic counseling decisions. ©2010 Wiley-Liss, Inc

Chemical element detection in thin sections of soils with the Laser Microprobe Mass Analyzer (LAMMA 500).

Components from thin sections of soils developed on weathered granite were analysed with the Laser Microprobe Mass Analyzer. Fragments of thin sections were mounted on sandwich grids, and perforated with the laser from the edges inwards (laser milling), using the laser light at grazing incidence. Laser-induced mass spectra of secondary titanium compounds and other constituents in the weathered granite were obtained in this manner. Positive and negative laser desorption mass spectra were recorded with such a speed and accuracy that in spite of the relatively large volume of analysed materials, minute changes in composition could be detected over very short distances. This allowed total chemical element analysis of spots in which titanium compounds concentrated during weathering, giving information on changes in purity of amorphous and semi-crystalline materials at such sites. Characteristic analysis possibilities of the Ion Microprobe Mass Analyzer (IMMA), Laser Microspectral Analyzer (LMA) and Laser Microprobe Mass Analyzer (LAMMA 500) are compared. (Abstract retrieved from CAB Abstracts by CABI’s permission

Geometry Relaxation and Transition State Search throughout Chemical Compound Space with Quantum Machine Learning

We use energies and forces predicted within response operator based quantum machine learning (OQML) to perform geometry optimization and transition state search calculations with legacy optimizers. For randomly sampled initial coordinates of small organic query molecules we report systematic improvement of equilibrium and transition state geometry output as training set sizes increase. Out-of-sample S$_\mathrm{N}$2 reactant complexes and transition state geometries have been predicted using the LBFGS and the QST2 algorithm with an RMSD of 0.16 and 0.4 \r{A} -- after training on up to 200 reactant complexes relaxations and transition state search trajectories from the QMrxn20 data-set, respectively. For geometry optimizations, we have also considered relaxation paths up to 5'500 constitutional isomers with sum formula C$_7$H$_{10}$O$_2$ from the QM9-database. Using the resulting OQML models with an LBFGS optimizer reproduces the minimum geometry with an RMSD of 0.14~\r{A}. For converged equilibrium and transition state geometries subsequent vibrational normal mode frequency analysis indicates deviation from MP2 reference results by on average 14 and 26\,cm$^{-1}$, respectively. While the numerical cost for OQML predictions is negligible in comparison to DFT or MP2, the number of steps until convergence is typically larger in either case. The success rate for reaching convergence, however, improves systematically with training set size, underscoring OQML's potential for universal applicability

Performance and modeling of superconducting ring resonators at millimeter-wave frequencies

Microstrip ring resonators operating at 35 GHz were fabricated from laser ablated YBCO thin films deposited on lanthanum aluminate substrates. They were measured over a range of temperatures and their performance compared to identical resonators made of evaporated gold. Below 60 Kelvin the superconducting strip performed better than the gold, reaching an unloaded Q approximately 1.5 times that of gold at 25 K. A shift in the resonant frequency follows the form predicted by the London equations. The Phenomenological Loss Equivalence Method is applied to the ring resonator and the theoretically calculated Q values are compared to the experimental results