bluuebunny/crossref_metadata_2025_split huggingface dataset:
Supratentorial and infratentorial damage in spinocerebellar ataxia 2: A diffusion‐weighted MRI study
Authors: Salvatore Elena , Tedeschi Enrico , Mollica Carmine , Vicidomini Caterina , Varrone Andrea , Coda Anna Rita Daniela , Brunetti Arturo , Salvatore Marco , De Michele Giuseppe , Filla Alessandro , Pappatà Sabina 
Published: December 2013

ABSTRACTSpinocerebellar ataxia type 2 (SCA2) is an autosomal‐dominant degenerative disorder that is neuropathologically characterized primarily by infratentorial damage, although less severe supratentorial involvement may contribute to the clinical manifestation. Diffusion‐weighted imaging (DWI)–Magnetic Resonance Imaging (MRI) studies of SCA2 have enabled in vivo quantification of neurodegeneration in infratentorial regions, whereas supratentorial regions have been explored less thoroughly. We measured microstructural changes in both infratentorial and supratentorial regions in 13 SCA2 patients (9 men, 4 women; mean age, 50 ± 12 years) and 15 controls (10 men, 5 women; mean age, 49 ± 14 years) using DWI‐MRI and correlated the DWI changes with disease severity and duration. Disease severity was evaluated using the International Cooperative Ataxia Rating Scale and the Inherited Ataxia Clinical Rating Scale. Cerebral diffusion trace (
) values were generated, and regions of interest (ROIs) and voxel‐based analysis with Statistical Parametric Mapping (SPM) were used for data analysis. In SCA2 patients, ROI analysis and SPM confirmed significant increases in
values in the pons, cerebellar white matter (CWM) and middle cerebellar peduncles. Moreover, SPM analysis revealed increased
values in the right thalamus, bilateral temporal cortex/white matter, and motor cortex/pyramidal tract regions. Increased diffusivity in the frontal white matter (FWM) and the CWM was significantly correlated with ataxia severity. DWI‐MRI revealed that both infratentorial and supratentorial microstructural changes may characterize SCA2 patients in the course of the disease and might contribute to the severity of the symptoms. © 2013 International Parkinson and Movement Disorder Society
Executive functions in premanifest Huntington's disease
Authors: You S. Christine , Geschwind Michael D. , Sha Sharon J. , Apple Alexandra , Satris Gabriella , Wood Kristie A. , Johnson Erica T. , Gooblar Jonathan , Feuerstein Jeanne S. , Finkbeiner Steven , Kang Gail A. , Miller Bruce L. , Hess Christopher P. , Kramer Joel H. , Possin Katherine L. 
Published: December 2013

ABSTRACTBackgroundWe investigated the viability of psychometrically robust executive function measures as markers for premanifest Huntington's disease (HD).MethodsFifteen premanifest HD subjects and 42 controls were compared on the NIH EXAMINER executive function battery. This battery yields an overall executive composite score, plus working memory, cognitive control, and fluency scores that are measured on psychometrically matched scales. The scores were correlated with two disease markers, disease burden and striatal volumes, in the premanifest HD subjects.ResultsThe premanifest HD subjects scored significantly lower on the working memory score. The executive composite positively correlated with striatal volumes, and the working memory score negatively correlated with disease burden. The cognitive control and fluency scores did not differ between the groups or correlate significantly with the disease markers.ConclusionsThe NIH EXAMINER executive composite and working memory scores are sensitive markers of cognitive dysfunction, striatal volume, and disease burden in premanifest HD. © 2013 International Parkinson and Movement Disorder Society
Relative exchangeable copper: A promising tool for family screening in Wilson disease
Authors: Trocello Jean‐Marc , El Balkhi Souleiman , Woimant France , Girardot‐Tinant Nadège , Chappuis Philippe , Lloyd Carla , Poupon Joël 
Published: December 2013

ABSTRACTBackgroundFamily screening is a main step for the diagnosis in Wilson disease. This study was undertaken to evaluate the value of relative exchangeable copper for family screening.MethodsData from family screening were collected from the French National Center of Reference for Wilson disease. Subjects who were first‐ or second‐degree relatives of the index case underwent clinical examination and biological parameters.ResultsOf 127 subjects examined, copper abnormalities or low ceruloplasminemia were detected in 21 subjects, corresponding to 5 patients with Wilson disease, 14 heterozygous ATP7B carriers and 2 subjects with no ATP7B mutations. Relative exchangeable copper determination significantly discriminates heterozygous ATP7B carriers and subjects with no ATP7B mutations from WD patients with a cutoff of 15%.ConclusionsExchangeable copper appears to be a promising tool for family screening in Wilson disease. © 2013 International Parkinson and Movement Disorder Society
Idiopathic head tremor in english bulldogs
Authors: Guevar Julien , De Decker Steven , Van Ham Luc M.L. , Fischer Andrea , Volk Holger A. 
Published: December 2013

AbstractIdiopathic head tremor (IHT) syndrome is a recognized but poorly characterized movement disorder in English bulldogs (EBs). The data analyzed were collected via a detailed online questionnaire and video recordings. Thirty‐eight percent of the population demonstrated IHT. The first presentation was early in life. There was no sex or neutered status predisposition. The condition disappeared with time in 50% of the cases. The direction of the head movement was vertical or horizontal. The number of episodes per day and the duration of the episodes were greatly variable. The majority of episodes occurred at rest. Most of the episodes were unpredictable. And there was no alteration of the mental status for most dogs during the episodes. Stress has been reported as a suspected trigger factor. IHT in EBs can be considered an idiopathic paroxysmal movement disorder. © 2013 International Parkinson and Movement Disorder Society
Nizatidine ameliorates gastroparesis in Parkinson's disease: A pilot study
Authors: Doi Hirokazu , Sakakibara Ryuji , Sato Mitsutoshi , Hirai Shigekazu , Masaka Tohru , Kishi Masahiko , Tsuyusaki Yohei , Tateno Akihiko , Tateno Fuyuki , Takahashi Osamu , Ogata Tsuyoshi 
Published: December 2013

ABSTRACTBackgroundThe objective of this work was to perform an open trial of the effects of nizatidine (NZT), a selective histamine H2‐receptor antagonist and a cholinomimetic, on gastroparesis in Parkinson's disease (PD) patients, using objective parameters given by a gastric emptying study using a 13C‐sodium acetate expiration breath test.MethodsTwenty patients with PD were enrolled in the study. There were 13 men and 7 women; aged 68.0 ± 7.72 years; disease duration 5.50 ± 3.62 years. All patients underwent the breath test and a gastrointestinal questionnaire before and after 3 months of administration of NZT at 300 mg/day. Statistical analysis was performed by Student t test.ResultsNZT was well tolerated by all patients and none had abdominal pain or other adverse effects. NZT significantly shortened Tmax (13C) (the peak time of the 13C‐dose‐excess curve) (P < 0.05).ConclusionsAlthough this is a pilot study, we found a significant shortening of gastric emptying time after administration of NZT in PD patients. © 2013 International Parkinson and Movement Disorder Society
Parkinson's disease with Lewy bodies associated with a heterozygous PARKIN dosage mutation
Authors: Sharp Madeleine E. , Marder Karen S. , Côté Lucien , Clark Lorraine N. , Nichols William C. , Vonsattel Jean‐Paul , Alcalay Roy N. 
Published: December 2013

ABSTRACTBackgroundPARKIN‐related disease remains incompletely understood. First, the pathogenicity of heterozygous PARKIN mutations is unclear, although some evidence supports causality. Second, unlike sporadic Parkinson's disease (PD), Lewy bodies are present only in a minority of cases. Only one other heterozygote PARKIN carrier with autopsy findings has been described. Our case adds to the broadening pathological and clinical phenotype of PARKIN‐related disease.MethodsClinical chart, genetic analysis, and pathological findings of a patient with familial PD are reviewed.ResultsA 44‐year‐old man developed slowly progressive tremor‐predominant PD with excellent response to levodopa. Genetic analysis revealed a heterozygous PARKIN exon 3–4 deletion, also present in 2 family members with early‐onset PD. Postmortem examination showed severe neuronal loss in the substantia nigra and nucleus coeruleus with the presence of diffuse Lewy bodies.ConclusionsThe deletion is unlikely an incidental finding considering family history, age at onset, and the presence of clinical and pathological features not typical of sporadic PD. © 2013 International Parkinson and Movement Disorder Society
A noninvasive handheld assistive device to accommodate essential tremor: A pilot study
Authors: Pathak Anupam , Redmond John A. , Allen Michael , Chou Kelvin L. 
Published: December 2013

ABSTRACTBackgroundWe explored whether a noninvasive handheld device using Active Cancellation of Tremor (ACT) technology could stabilize tremor‐induced motion of a spoon in individuals with essential tremor (ET).MethodsFifteen ET subjects (9 men, 6 women) performed 3 tasks with the ACT device turned on and off. Tremor severity was rated with the Fahn‐Tolosa‐Marin Tremor Rating Scale (TRS). Subjective improvement was rated by subjects with the Clinical Global Impression Scale (CGI‐S). Tremor amplitude was measured using device‐embedded accelerometers in 11 subjects.ResultsTRS scores improved with ACT on (versus off) in all 3 tasks: holding (1.00 ± 0.76 vs. 0.27 ± 0.70; P = 0.016), eating (1.47 ± 1.06 vs. 0.13 ± 0.64; P = 0.001), and transferring (1.33 ± 0.82 vs. 0.27 ± 0.59; P = 0.001). CGI‐S improved with eating and transferring, but not the holding task. Accelerometer measurements demonstrated 71% to 76% reduction in tremor with the ACT device on.ConclusionsThis noninvasive handheld ACT device can reduce tremor amplitude and severity for eating and transferring tasks in individuals with ET. © 2013 International Parkinson and Movement Disorder Society
On the linearization of operators related to the full waveform inversion in seismology
Authors: Kirsch Andreas , Rieder Andreas 
Published: December 2013

AbstractIn this work, we analyze the parameter‐to‐solution map of the acoustic wave equation with respect to its parameters wave speed and mass density. This map is a mathematical model for the seismic inverse problem where one wants to recover the parameters from measurements of the acoustic potential. We show its complete continuity and Fréchet differentiability. To this end, we provide necessary existence, stability, and regularity results. Moreover, we discuss various implications of our findings on the inverse problem and comment on the Born series. Copyright © 2013 John Wiley & Sons, Ltd.
Deformation of striped patterns by inhomogeneities
Authors: Jaramillo Gabriela , Scheel Arnd 
Published: December 2013

AbstractWe study the effects of adding a local perturbation in a pattern‐forming system, taking as an example the Ginzburg–Landau equation with a small localized inhomogeneity in two dimensions. Measuring the response through the linearization at a periodic pattern, one finds an unbounded linear operator that is not Fredholm due to continuous spectrum in typical translation invariant or weighted spaces. We show that Kondratiev spaces, which encode algebraic localization that increases with each derivative, provide an effective means to circumvent this difficulty. We establish Fredholm properties in such spaces and use the result to construct deformed periodic patterns using the Implicit Function Theorem. We find a logarithmic phase correction, which vanishes for a particular spatial shift only, which we interpret as a phase‐selection mechanism through the inhomogeneity. Copyright © 2013 John Wiley & Sons, Ltd.
Daily life functioning of community‐dwelling elderly couples: an investigation of the feasibility and validity of Ecological Momentary Assessment
Authors: Rullier Laetitia , Atzeni Thierry , Husky Mathilde , Bouisson Jean , Dartigues Jean‐François , Swendsen Joel , Bergua Valerie 
Published: December 2013

AbstractAlthough ambulatory data collection techniques have been used in elderly populations, their feasibility and validity amongst elderly individuals with cognitive impairment and amongst couples remains unexplored. The main objective of this study is to examine the validity of Ecological Momentary Assessment (EMA) in elderly persons with or without cognitive impairment and their spouses. The sample included 58 retired farmers (mean 77.3 years, standard deviation [SD] 5.5) with or without cognitive impairment, recruited within a French cohort and 60 spouses (mean 73.4 years, SD 6.9). The presence of cognitive impairment determining by a panel of specialized neurologists permitted to define two groups: “The Cognitive Impairment Group” and “The Control Group”. EMA procedures consisted of repeated telephone interviews five times per day during four days for each spouse. Our results demonstrate the validity of EMA procedures through a 92.1% level of compliance, the absence of fatigue effects, and the lack of evidence for major reactivity to the methods. However, the specificity of our sample may explain the acceptance (42%) and response (75%) rates and may reduce the generalizability of the results to the general population of elderly individuals. Finally, the validation of such techniques may contribute to future research examining community‐dwelling elderly individuals and their spouses. Copyright © 2013 John Wiley & Sons, Ltd.
A finite element approach combining a reduced‐order system, Padé approximants, and an adaptive frequency windowing for fast multi‐frequency solution of poro‐acoustic problems
Authors: Rumpler R. , Göransson P. , Deü J.‐F. 
Published: December 2013

SUMMARYIn this work, a solution strategy is investigated for the resolution of multi‐frequency structural‐acoustic problems including 3D modeling of poroelastic materials. The finite element method is used, together with a combination of a modal‐based reduction of the poroelastic domain and a Padé‐based reconstruction approach. It thus takes advantage of the reduced‐size of the problem while further improving the computational efficiency by limiting the number of frequency resolutions of the full‐sized problem. An adaptive procedure is proposed for the discretization of the frequency range into frequency intervals of reconstructed solution. The validation is presented on a 3D poro‐acoustic example. Copyright © 2013 John Wiley & Sons, Ltd.
A high‐order approach for modelling transient wave propagation problems using the scaled boundary finite element method
Authors: Chen D. , Birk C. , Song C. , Du C. 
Published: December 2013

SUMMARYA high‐order time‐domain approach for wave propagation in bounded and unbounded domains is proposed. It is based on the scaled boundary FEM, which excels in modelling unbounded domains and singularities. The dynamic stiffness matrices of bounded and unbounded domains are expressed as continued‐fraction expansions, which leads to accurate results with only about three terms per wavelength. An improved continued‐fraction approach for bounded domains is proposed, which yields numerically more robust time‐domain formulations. The coefficient matrices of the corresponding continued‐fraction expansion are determined recursively. The resulting solution is suitable for systems with many DOFs as it converges over the whole frequency range, even for high orders of expansion. A scheme for coupling the proposed improved high‐order time‐domain formulation for bounded domains with a high‐order transmitting boundary suggested previously is also proposed. In the time‐domain, the coupled model corresponds to equations of motion with symmetric, banded and frequency‐independent coefficient matrices, which can be solved efficiently using standard time‐integration schemes. Numerical examples for modal and time‐domain analysis are presented to demonstrate the increased robustness, efficiency and accuracy of the proposed method. Copyright © 2013 John Wiley & Sons, Ltd.
A direct search approach to optimization for nonlinear model predictive control
Authors: Gibson James D. 
Published: December 2013

SummaryNonlinear model predictive control (NMPC) depends on performing a constrained nonlinear optimization, based on predictions of future system behavior, during a sampling interval to determine the control action to be applied to the system during the next time step. The difficulty in designing an optimization procedure to solve a constrained NMPC problem is due to the finite time horizon to which the predictive model is evaluated, the state and control actuator constraints, and sampling interval length. The resulting objective function, which is to be optimized is typically not differentiable. Although there are many commercial, shareware, and open‐source optimization packages available that can perform a nonlinear constrained optimization for most cases, there are NMPC implementations requiring embedded code or that must meet stringent timing requirements that preclude the use of off‐the‐shelf packages. In cases where the predictive model is known, such as aerodynamic or hydrodynamic systems, a direct‐search optimization algorithm can perform well enough in a real‐time environment. Direct search algorithms are simple to implement and can be made more efficient by applying differential geometric techniques to the search methodology. The typical smoothness of the equations of motion for vehicular systems allows the objective function's stationarities to be handled in a straight‐forward way. Copyright © 2013 John Wiley & Sons, Ltd.
Childhood acute lymphoblastic leukemia in Nicaragua: Long‐term results in the context of an international cooperative program
Authors: Pacheco Carlos , Lucchini Giovanna , Valsecchi Maria Grazia , Malta Alberto , Conter Valentino , Flores Andronica , Biondi Andrea , Tognoni Gianni , Arguello Mercedes , Cavalli Franco , Silvestri Daniela , Lacayo Fulgencio Baez , Masera Giuseppe 
Published: December 2013

BackgroundThe aim of this paper is to describe the results of acute lymphoblastic leukemia (ALL) treatment in Nicaragua from 1995 to 2005 in the context of an international cooperation program.ProceduresPatients <18 years with ALL were treated with two consecutive protocols (1995 and 2000). After a steroid prophase, a three‐drug induction was administered in protocol 1995, and a four‐drug induction, including asparaginase, was administered in protocol 2000. In protocol 2000, a modified BFM phase IB with cyclophosphamide, 6‐mercaptopurine and cytosine arabinoside was administered to patients at high risk (HR), who also received IV methotrexate (500 mg/m2) in the consolidation phase. Reinduction consisted of dexamethasone, vincristine, doxorubicin, cytosine arabinoside, and 6‐thioguanine administered over 7 (protocol 1995) or 4 (protocol 2000) weeks; reinduction was repeated twice for patients at HR. Maintenance consisted of p.o. 6‐mercaptopurine and methotrexate, and vincristine and dexamethasone pulses were added in the 2000 study. The total duration of therapy was 24 months.ResultsIn total, 540 patients were treated. Overall, 7% of patients died during induction, and 9% abandoned treatment. At 5 and 10 years from diagnosis, event‐free survival (EFS) rates of 38.1% and 36.6%, respectively, and overall survival rates of 48.0% and 39.6%, respectively, were obtained, considering abandonment as an event.ConclusionsIn our experience, a 10‐year EFS of 36.6% was achieved in a country with limited resources. Factors limiting a higher success rate were treatment abandonment and a high relapse rate. Pediatr Blood Cancer 2014;61:827–832. © 2013 Wiley Periodicals, Inc.
Coronal approach for measuring both fetal lateral ventricles: is there an advantage over the axial view?
Authors: Katorza Eldad , Duvdevani Nir , Jacobson Jeffrey‐Michael , Gilboa Yinon , Hoffmann Chen , Achiron Reuven 
Published: December 2013

ABSTRACTObjectiveThis study aimed to compare measurement of the lateral cerebral ventricular diameter using either the traditional axial view or the coronal plane.Materials and methodsWe performed a prospective study on 144 fetuses, 77 evaluated as part of a routine fetal scan and 67 referred for a neurosonogram. Distal lateral ventricles were measured both in axial and coronal plane.ResultsGood visualization of the ventricles was achieved in 91% of the cases using the coronal plane (both ventricles) and in 95% of the cases using the axial plane (only the distal ventricle) (p > 0.001). The mean width of the distal lateral ventricle in the axial plane was 7.9 ± 1.9 mm versus 8.2 ± 1.9 mm on the coronal plane (p < 0.001). This larger diameter by 0.3 mm was not dependent on the indication for the scan or the gestational age. Slight asymmetry was present on coronal images in the routine group (0.2 mm), and that was even larger in the referral group (1.6 mm) (p < 0.001).ConclusionCoronal measurement of both ventricles is feasible and has the advantage over measurement in the axial view in which only the distal ventricle is clearly visible and measurable. © 2013 John Wiley & Sons, Ltd.
Dual length morphological model for bulk‐heterojunction, polymer‐based solar cells
Authors: Shen Hao , Zhang Wenluan , Mackay Michael E. 
Published: December 2013

ABSTRACTWe present a dual length morphological model for the active layer of bulk‐heterojunction, polymer‐based solar cells using results from neutron and X‐ray scattering techniques. Two critical characteristic lengths are found in the mixtures composed of poly(3‐hexylthiophene) (P3HT) and [6,6]‐phenyl‐C61‐butyric acid methyl ester (PCBM). A characteristic length at 15 nm is the local characteristic of the P3HT crystals and PCBM agglomerations, which is independent of the bulk composition upon relaxation by thermal annealing. Conversely, a larger bicontinuous structure described by Teubner–Strey model with phase distances between 23 and 35 nm forms only after thermal annealing, which is highly correlated to the bulk compositions. These results suggest phase separation between the polymer and fullerene can only be partially manipulated by simple processing techniques such as coating conditions and annealing, and a more rigorous design of the morphology should be implemented in the future. © 2013 Wiley Periodicals, Inc. J Polym Sci Part B: Polym. Phys. 2014, 52, 387–396
Pd@SnO2 and SnO2@Pd Core@Shell Nanocomposite Sensors
Authors: Gyger Fabian , Sackmann André , Hübner Michael , Bockstaller Pascal , Gerthsen Dagmar , Lichtenberg Henning , Grunwaldt Jan‐Dierk , Barsan Nicolae , Weimar Udo , Feldmann Claus 
Published: December 2013

Pd@SnO2 and SnO2@Pd core@shell nanocomposites are prepared via a microemulsion approach. Both nanocomposites exhibit high‐surface, porous matrices of SnO2 shells (>150 m2 g−1) with very small SnO2 crystallites (<10 nm) and palladium (Pd) nanoparticles (<10 nm) that are uniformly distributed in the porous SnO2 matrix. Although similar by first sight, Pd@SnO2 and SnO2@Pd are significantly different in view of their structure with Pd inside or outside the SnO2 shell and in view of their sensor performance. As SMOX‐based sensors (SMOX: semiconducting metal oxide), both nanocomposites show a very good sensor performance for the detection of CO and H2. Especially, the Pd@SnO2 core@shell nanocomposite is unique and shows a fast response time (τ90 < 30 s) and a very good response at low temperature (<250 °C), especially under humid‐air conditions. Extraordinarily high sensor signals are observed when exposing the Pd@SnO2 nanocomposite to CO in humid air. Under these conditions, even commercial sensors (Figaro TGS 2442, Applied Sensor MLC, E2V MICS 5521) are outperformed.