bluuebunny/crossref_metadata_2025_split huggingface dataset:
Huntington's disease like‐2 neuropathology
Authors: Greenstein Penny E. , Vonsattel Jean‐Paul G. , Margolis Russell L. , Joseph Jeffrey T. 
Published: May 2007

AbstractHuntington's disease like‐2 (HDL‐2) neurodegeneration is a recently described autosomal dominant disorder with features similar to Huntington's disease (HD). Only one case report has described neuropathology from an affected patient. We describe the clinical presentation and illustrate the pathology in two additional molecularly confirmed patients, compare these with the previously published case, and contrast them with HD. We examined two patients with HDL‐2. Their charts were reviewed, their brains were examined using standard neuropathology techniques, including immunoperoxidase stains, and their diagnoses were confirmed with a PCR‐based assay for repeat length. The first patient presented with obsessive suspiciousness, while the second had depression and decreased visual acuity. Both patients developed increased tone and cogwheel rigidity, but neither developed choreoathetosis. Extensive degeneration affected the caudate nucleus and putamen, especially dorsally and laterally. In addition, the first patient showed lateral temporal, lateral frontal, and orbitofrontal cortical atrophy, while the second patient displayed marked degeneration in the occipital and parietal cortices. Neither patient showed significant changes in the cerebellum or brainstem. Both cases had ubiquitin‐immunoreactive neuronal intranuclear inclusions (NII). The patients with of HDL‐2 reviewed here were remarkable for significant frontal inhibition with parkinsonism, a lack of choreiform movements, and African ancestry. Pathologically, HDL‐2 is similar to HD in its effect on the neostriatum but may differ, at least in some cases, in its degree of focal cortical involvement, including the occipital lobe.
Paul Blocq and (psychogenic) astasia abasia
Authors: Okun Michael S. , Koehler Peter J. 
Published: May 2007

AbstractThe Greek term astasia–abasia literally translates to mean inability to stand and to walk. Although today we would classify the syndrome as a conversion disorder, it was considered a separate disease by Paul Blocq (1860–1896), who described this phenomenon as the inability to maintain an upright posture, despite normal function of the legs in the bed. Paul Blocq's original 1888 articles on astasia abasia were read, partly translated from French to English, and the cases were summarized. A review of a selection of the literature following Blocq's description on astasia abasia was performed. Present day literature was consulted as well. Blocq reviewed what was known about astasia abasia in two papers. Although he was the first to use the combined term astasia abasia, he referred to similar descriptions of Charcot, Richer, Mitchell, Jaccoud, Roméi, and Erlenmeyer. Blocq's contribution was in compiling the experiences and observations of preeminent 19th century neurologists. He recognized that paralysis, jumping, fits, tremor, and bizarre behavior could all be associated with the syndrome. He distinguished the disorder from hysteria. Moreover, he presented a formal delineation of the gait disorder and provided a pathophysiologic concept. Prognosis was considered favorable. Paul Blocq directed attention to psychogenic gait disorders by publishing a small case series on astasia abasia. Today, these case descriptions would be likely considered cases of conversion or psychogenic gait disorder, with or without other conversion/psychogenic movement disorders. © 2007 Movement Disorder Society
Thirty days complication rate following surgery performed for deep‐brain‐stimulation
Authors: Voges Jürgen , Hilker Rüdiger , Bötzel Kai , Kiening Karl L. , Kloss Manja , Kupsch Andreas , Schnitzler Alfons , Schneider Gerd‐Helge , Steude Ulrich , Deuschl Günther , Pinsker Markus O. 
Published: May 2007

AbstractSerious adverse events (SAEs) during the first 30 postoperative days after stereotactic surgery for Deep‐Brain‐Stimulation performed in 1,183 patients were retrospectively collected from five German stereotactic centers. The mortality rate was 0.4% and causes for death were pneumonia, pulmonary embolism, hepatopathy, and a case of complicated multiple sclerosis. The permanent surgical morbidity rate was 1%. The most frequently observed SAEs were intracranial hemorrhage (2.2%) and pneumonia (0.6%). Skin infection occurred in 5 of 1,183 patients (0.4%). Surgical complications caused secondary AEs (e.g. pneumonia) preferentially in older patients and in patients treated for Parkinson's disease (PD). Complication rates did not differ among the five centers. © 2007 Movement Disorder Society
Pathophysiological concepts of restless legs syndrome
Authors: Paulus Walter , Dowling Pascal , Rijsman Roselyne , Stiasny‐Kolster Karin , Trenkwalder Claudia , de Weerd Al 
Published: May 2007

AbstractPathophysiological concepts of restless legs syndrome (RLS) are based mainly on neuroimaging and on neurophysiological data. Furthermore treatment effects contribute essentially to the present understanding of the disease, unless the genetic progress expected in the near future will clarify substantially open issues. The concept agreed on assumes a dysfunction of the dopaminergic system, possibly on the level of striatal and/or spinal dopamine receptors, and the A11 neuron group localized in the hypothalamus as an integrated part of the system. These neurons modulate spinal excitability, alterations of which in turn affect sensory processing predominantly of leg afferents in brain stem structures. Neurophysiologically excitability alterations can be measured by a variety of methods such as determination of pain thresholds, H‐reflex testing, and quantitative sensory testing. © 2007 Movement Disorder Society
The suitability of patient‐based measures in the field of Parkinson's disease: A systematic review
Authors: Den Oudsten Brenda L. , Van Heck Guus L. , De Vries Jolanda 
Published: May 2007

AbstractThe aim of this study is to appraise the suitability of current quality of life (QOL) questionnaires for use in the field of Parkinson's disease (PD). Computerized bibliographic databases were screened for publications from 1960 to December 2006. Predefined selection criteria were used to identify QOL questionnaires in PD studies. Two investigators independently assessed and, subsequently, agreed on a set of multidimensional generic and PD‐specific QOL questionnaires. Data were extracted concerning the internal structure, reliability, validity, and responsiveness of the included questionnaires. Sixteen‐questionnaires were found, of which 14 questionnaires were included (six generic measures and eight PD‐specific). In general, the psychometrics of all the questionnaires were adequately described. Sensitivity to change, however, has been reported for only a limited number of instruments. Almost all included questionnaires used QOL as a keyword, but only two questionnaires fitted the broad QOL definition used in this review. Considering the few “real ” QOL questionnaires, we conclude that there is a strong need for such instruments. © 2007 Movement Disorder Society
Bright light therapy in Parkinson's disease: A pilot study
Authors: Paus Sebastian , Schmitz‐Hübsch Tanja , Wüllner Ullrich , Vogel Antje , Klockgether Thomas , Abele Michael 
Published: May 2007

AbstractSeveral observations suggest a beneficial effect of melatonin antagonism for Parkinson's disease (PD). Although bright light therapy (BLT) suppresses melatonin release and is an established treatment for depression and sleep disturbances, it has not been evaluated in PD. We examined effects of BLT on motor symptoms, depression, and sleep in PD in a randomized placebo‐controlled double‐blind study in 36 PD patients, using Parkinson's Disease Rating Scale (UPDRS) I–IV, Beck's Depression Inventory, and Epworth Sleepiness Scale. All patients received BLT for 15 days in the morning, 30 min daily. Illuminance was 7.500 lux in the active treatment group and 950 lux in the placebo group. Although group differences were small, BLT led to significant improvement of tremor, UPDRS I, II, and IV, and depression in the active treatment group but not in the placebo group. It was very well tolerated. Follow up studies in more advanced patient populations employing longer treatment durations are warranted. © 2007 Movement Disorder Society
Alien limb following posterior cerebral artery stroke: Failure to recognize internally generated movements?
Authors: Coulthard Elizabeth , Rudd Anthony , Playford E. Diane , Husain Musad 
Published: May 2007

AbstractWe describe two rare cases of alien limb syndrome following right posterior cerebral artery (PCA) stroke. Both patients present with left hemianopia, visual neglect and proprioceptive loss in addition to their alien limb symptoms. Lesion subtraction from seven control PCA patients revealed that medial parietal‐occipital and thalamic areas were selectively damaged in the alien limb patients. We propose that loss of the sense of motor intention and internal model of the current state of the arm, combined with deficient proprioceptive and visual feedback of the moving limb, are critical for genesis of posterior alien limb and discuss how affected regions normally function to ensure awareness of self‐generated motor activity. © 2007 Movement Disorder Society
Does ageing influence deep brain stimulation outcomes in Parkinson's disease?
Authors: Ory‐Magne Fabienne , Brefel‐Courbon Christine , Simonetta‐Moreau Marion , Fabre Nelly , Lotterie Jean Albert , Chaynes Patrick , Berry Isabelle , Lazorthes Yves , Rascol Olivier 
Published: May 2007

AbstractWe sought to define the influence of ageing in clinical, cognitive, and quality‐of‐life outcomes after subthalamic nucleus deep brain stimulation (STN‐DBS) in Parkinson's disease (PD). We performed motor assessment (UPDRS), mood tests, cognitive, and quality of life evaluation (PDQ‐39) on PD patients before surgery, and 12 and 24 months after, and we recorded adverse events. The variations of these parameters after surgery were correlated with age using regression statistical tests. Cerebral bleeding risk was evaluated by a nonparametric test. We enrolled 45 patients (mean age 60 ± 9 years, range 40–73). No significant correlation was found between age and motor scores and PDQ‐39 improvements at 12 months. At 24 months, there was a significant negative correlation between age and the improvement of three dimensions of PDQ 39 (mobility, activities of daily life, and cognition). Cognitive impairment showed no correlation, but apathy and depression were positively correlated with age. Significant statistical difference was observed between cerebral bleeding and age. STN‐DBS is an effective treatment for elderly patients with advanced PD. A longer follow‐up duration and a larger population seem necessary to better assess the quality of life perception in elderly patients and to determinate the real risk of hemorrage. © 2007 Movement Disorder Society
Oral festination in Parkinson's disease: Biomechanical analysis and correlation with festination and freezing of gait
Authors: Moreau Caroline , Ozsancak Canan , Blatt Jean‐Louis , Derambure Philippe , Destee Alain , Defebvre Luc 
Published: May 2007

AbstractIn Parkinson's disease (PD), festination corresponds to a tendency to speed up when performing repetitive movements. First described in gait (and then in handwriting and speech), festination is one of the most disabling axial symptoms. To establish the phenomenology of oral festination (OF) and the condition's potential links with other axial disorders, we submitted a simple, rhythmic, repetitive, vocal motor task to 40 PD patients and 20 controls. Forty‐five percent of the 40 patients presented OF, which was strongly associated with gait festination but not with the severity of freezing of gait (FOG) or dysarthria. With respect to the two pathophysiological hypotheses that have been put forward, a possible link with tremor (as previously suggested in tapping) was not confirmed in this study and so, in view of the significant increase in variability observed, we conclude that OF shares the same pathophysiology as gait disorders. © 2007 Movement Disorder Society
Adaptive multiresolution refinement with distance fields
Authors: Tsukanov I. , Shapiro V. 
Published: May 2007

AbstractThis paper describes a multiresolution approach to field modeling that can be used with any meshfree or mesh‐based method for adaptive solution refinement. The refined solution is represented as a superposition of a coarse (unrefined) solution and a sequence of refinements that provide additional degrees of freedom with higher spatial or functional resolution. Each refinement is treated as a solution to a boundary‐value problem within a specified refinement window. The proposed approach is based on the meshfree method with distance fields (Comput. Mech. 2000; 25:305–316, Eng. Comput. 2002; 18(4):295–311) and guarantees Cm continuity of the refined solutions with matching or non‐matching grids. The method does not restrict the shape of the refinement window and does not place any constraints on the type of basis functions, or relative position and resolution of the refinement grids. Combining the proposed approach with hierarchical space decompositions and a posteriori error estimators results in an effective tool for automatic solution refinement. Carefully chosen numerical examples illustrate the power and advantages of the proposed approach. Copyright © 2007 John Wiley & Sons, Ltd.
Liberation regimes and land reform in Africa. Land politics transcending enmity in South Africa
Authors: Gran Thorvald 
Published: May 2007

AbstractTruthfulness, the norm of science, is etymologically related to trust. Both concepts are related to ‘tree’, as a symbol for grounded knowledge, for differentiation (the tree of knowledge), for uprightness and reliability (Searle, 1995). Truthfulness generates trust. Trust generates community. The land politics and trust project in South Africa (Askvik and Bak, 2005) investigated how trust relations intervened within and between government institutions engaged in redistribution and management of land. It enquired into trust relations between the land state and stakeholders in land. It assessed how the new ANC‐controlled state intervened into the relation between market‐oriented urban industry and subsistence‐oriented livelihoods on communal land. Could that intervention explain the slow pace of land redistribution? The field work was done in the Northern and the Western Cape provinces. A hypothesis is that unconditional personal trust across institutional boundaries is a condition for post‐colonial, post‐liberation community building. In 2001, aspects of political democracy were in place. Trust relations between government institutions and to stakeholders in land varied, but were limited. Trusted mediators between the institutions, across cultures, were few and far apart. Subsistence‐oriented livelihoods on communal land were there to be transformed to commercial farming. The three‐step Government strategy, growth in the urban economy, commercialisation of rural subsistence production and rural welfare from the urban surplus, augmented separation, disbelief and distrust. Copyright © 2007 John Wiley & Sons, Ltd.
Relative impact of polar‐orbiting and geostationary satellite radiances in the Aladin/France numerical weather prediction system
Authors: Montmerle Thibaut , Rabier Florence , Fischer Claude 
Published: May 2007

AbstractFor its short‐range forecasts over Western Europe, Météo‐France runs the limited‐area model ALADIN operationally with four daily analyses obtained with a 3D‐Var data assimilation system. This system includes, among other observation types, radiances from AMSU‐A, AMSU‐B, HIRS and SEVIRI radiometers. SEVIRI is on board the geostationary platform Meteosat‐8 and provides continuous observations in space and in time over the region of interest at several wavelengths, while the others, which are on board polar‐orbiting satellites, have poorer temporal and horizontal resolutions but a better spectral resolution than SEVIRI. Observing System Experiments (OSEs) have been performed with the operational 3D‐Var to assess the impact of such satellite data on analyses and on forecasts. DFS (Degrees of Freedom for Signal) have been computed and have shown the complementarity between WV channels from the different radiometers. In the operational version of the 3D‐Var, DFS values show that analyses are strongly controlled by SEVIRI data in the mid to high troposphere. This is consistent with the large number of assimilated SEVIRI radiances. HIRS and AMSU‐B WV data would provide more information if SEVIRI data were not assimilated and if ATOVS data were used with a higher density. However, using ATOVS data with a higher horizontal resolution makes the analyses more dependent on these data, and it does not appear to be beneficial in this particular context, probably because of a non‐optimal bias correction. In that case however, the individual impact of each pixel decreases because of the horizontal correlation lengths of the structure functions. Forecast scores and predicted precipitation patterns display the positive impact of SEVIRI data. Copyright © 2007 Royal Meteorological Society
Spectral and spatial localization of background‐error correlations for data assimilation
Authors: Buehner Mark , Charron Martin 
Published: May 2007

AbstractIn this study, the localization of background‐error correlations in both the spectral and the spatial domains is examined. While spatial localization has become a standard approach for reducing the sampling error of background‐error correlations, localization of spectral correlations has not yet been fully explored. It is shown that spectral localization results in a spatial smoothing of the correlation functions in grid‐point space. The use of correlations that are diagonal in spectral space, resulting in globally homogeneous correlations, has been frequently employed with data assimilation applications for numerical weather prediction (NWP). More recently, correlations that are diagonal in the space defined by an expansion of wavelet functions have been used to implicitly localize the correlations in a particular way in both spectral and grid‐point spaces simultaneously. In this study, the explicit localization of correlations by varying amounts in both the spatial and the spectral domains is applied, to evaluate their complementary ability to reduce sampling error.Spectral and spatial localization are first applied to an idealized one‐dimensional problem where the true correlations are known. In this context it is found that there is an optimal combination of spectral and spatial localization that minimizes the sampling error for a given ensemble of error realizations. Then the implementation of spectral localization is demonstrated in both a realistic three‐dimensional variational assimilation system for NWP and an ensemble‐based data assimilation system applied to an idealized model of the atmosphere's mesoscale dynamics. Two very different practical approaches are used to implement spectral localization for these two types of data assimilation systems. For the application to the ensemble‐based data assimilation system, spectral localization is shown to systematically reduce analysis error without requiring additional model forecasts to be performed. Copyright © 2007 Crown in the right of Canada. Published by John Wiley & Sons, Ltd
Aerosol optical depth and Ångström exponent climatology at El Arenosillo AERONET site (Huelva, Spain)
Authors: Toledano C. , Cachorro V. E. , Berjon A. , de Frutos A. M. , Sorribas M. , de la Morena B. A. , Goloub P. 
Published: May 2007

AbstractRoutine aerosol measurements have been carried out at INTA–El Arenosillo (Huelva, Spain) since February 2000 with a Cimel sun photometer integrated in the global aerosol monitoring network AERONET. A 5‐year aerosol database allows characterization and classification of aerosol properties, which define the local aerosol climatology. In this work two basic parameters for aerosol characterization, aerosol optical depth (AOD) and Ångström exponent, α, are used for aerosol analysis. The mean AOD is 0.18 ( ± 0.14) and the mean α is 1.05 ( ± 0.43). The AOD presents two peaks during the year, at the end of the winter and during the summer, which are related to the seasonal patterns of the desert dust aerosols which arrive at the south‐western Iberian Peninsula from North Africa. The Ångström exponent presents two frequency modes, related to the two main aerosol types present at El Arenosillo: coastal marine aerosols and desert dust. A main aerosol scenario (66% of data) is defined as coastal marine aerosols, with the influence of local sources of continental and polluted aerosols. Continental aerosols are present in 11% of cases, while the desert dust has a very relevant occurrence in around 20% of the data. Copyright © 2007 Royal Meteorological Society
A comparison of cloud‐resolving model simulations of trade wind cumulus with aircraft observations taken during RICO
Authors: Abel S. J. , Shipway B. J. 
Published: May 2007

AbstractThis paper presents results from simulations of trade wind cumulus with the Met Office Large Eddy Model (LEM) based on observed environmental profiles from the Rain In Cumulus over the Ocean (RICO) field experiment. Comparisons of updraught core parameters are made with the in situ data collected onboard the three research aircraft that participated in RICO. The default set‐up of the LEM was unable to produce sufficient amounts of rainwater content when compared to measurements by the aircraft. As a main aim of RICO was to quantify the importance of precipitation in the trade cumulus regime, we test the sensitivity of the model to changes in the rain microphysics, the large‐scale forcing, and horizontal resolution. By changing these model variables we are able to obtain reasonably good agreement with the observations of updraught core vertical velocity, cloud and rainwater contents. Furthermore, the LEM produces comparable surface precipitation rates to those derived from radar measurements during RICO in a previous study. This gives us some confidence in the ability of the LEM to simulate realistic precipitating trade cumulus. Copyright © 2007 Royal Meteorological Society
Adaptive bias correction for satellite data in a numerical weather prediction system
Authors: Auligné T. , McNally A. P. , Dee D. P. 
Published: May 2007

AbstractAdaptive bias corrections for satellite radiances need to separate the observation bias from the systematic errors in the background in order to prevent the analysis from drifting towards its own climate. The variational bias correction scheme (VarBC) is a particular adaptive scheme that is embedded inside the assimilation system.VarBC is compared with an offline adaptive and a static bias correction scheme. In simulation, the three schemes are exposed to artificial shifts in the observations and the background. The VarBC scheme can be considered as a good compromise between the static and the offline adaptive schemes. It shows some skill in distinguishing between the background‐error and the observation biases when other unbiased observations are available to anchor the system. Tests of VarBC in a real numerical weather prediction (NWP) environment show a significant reduction in the misfit with radiosonde observations (especially in the stratosphere) due to NWP model error. The scheme adapts to an instrument error with only minimal disruption to the analysis.In VarBC, the bias is constrained by the fit to observations—such as radiosondes—that are not bias‐corrected to the NWP model. In parts of the atmosphere where no radiosonde observations are available, the radiosonde network still imposes an indirect constraint on the system, which can be enhanced by applying a mask to VarBC. Copyright © 2007 Royal Meteorological Society