Hereditary neuralgic amyotrophy and hereditary neuropathy with liability to pressure palsies: two distinct clinical, electrophysiologic, and genetic entities

Fistulas secondary to Crohn's disease occur in about 33% of patients. A colobronchial fistula complicating Crohn's disease is very rare, having been reported only twice previously. We present an unusual fistula secondary to Crohn's colitis that originated from the splenic flexure and crossed the diaphragm to involve the bronchial tree.     

Ionoluminescence decay measured with single ions

A new ion beam analysis-based, single ion technique called the time to first photon has been developed to measure the decay of the luminescence signal of phosphors. Such measurements are currently needed to study luminescence decay mechanisms following high-density excitations and to identify strongly luminescent phosphor coatings with short lifetimes for ion photon emission microscopy (IPEM). The samples for this technique consist of thin phosphor layers placed or coated on the surface of PIN diodes. Single ions from an accelerator strike this sample and simultaneously create ion beam induced luminescence (IBIL) from the phosphor that is measured by a single-photon-detector, and an ion beam induced charge collection (IBICC) signal in the PIN diode. In this case, the IBICC signal provides the start pulse and the IBIL signal the stop pulse to a time to amplitude converter. It is straightforward to show that this approach also measures a signal proportional to activity versus time with an accuracy of 5% as long as the number of detected photons per ion is less than 0.1, which usually requires the use of absorbers for the IBIL detector or electronic discrimination for the IBIL signals. Details of the new analysis are given together with examples of luminescence decay measurements of several ceramic phosphors being considered to coat IPEM samples. IPEM is currently being developed at Sandia National Laboratory (SNL), the University of North Texas in Denton, and the Universities and INFN of Padova and Torino.     

Correlation between left ventricular hypertrophy and GAA trinucleotide repeat length in Friedreich's ataxia

BACKGROUND: Friedreich's ataxia (FA), the most common inherited ataxia, is associated frequently with cardiac hypertrophy, and death is often cardiac related. Recently, the disease has been associated with a mutation that consists of an unstable expansion of GAA repeats in the first intron of the gene encoding frataxin on chromosome 9. METHODS AND RESULTS: We studied 44 consecutive patients with FA, determined the size of GAA expansions in the frataxin gene, and examined the relation between the genotype and cardiac phenotype assessed by M-mode and two-dimensional echocardiography. All the patients were homozygous for the mutation. The size of the GAA expansion on the smaller allele varied from 270 to 1200. We found a correlation between the size of GAA expansion and the left ventricular wall thickness (r = .51, P < .001) and the left ventricular mass index (r = .45, P = .002). Left ventricular hypertrophy was observed in 81% of patients with a number of GAA repeats above the median value of 770 compared with only 14% in the other group (P = .002). CONCLUSIONS: These data demonstrate that in FA, the severity of left ventricular hypertrophy is related to the number of GAA repeats. These results suggest that abnormalities of the gene encoding frataxin, a protein of unknown function highly expressed in the normal heart, may play an important role in the modulation of cardiac hypertrophy.     

The right view from the wrong location: depth perception in stereoscopic multi-user virtual environments

Stereoscopic depth cues improve depth perception and increase immersion within virtual environments (VEs). However, improper display of these cues can distort perceived distances and directions. Consider a multi-user VE, where all users view identical stereoscopic images regardless of physical location. In this scenario, cues are typically customized for one "leader" equipped with a head-tracking device. This user stands at the center of projection (CoP) and all other users ("followers") view the scene from other locations and receive improper depth cues. This paper examines perceived depth distortion when viewing stereoscopic VEs from follower perspectives and the impact of these distortions on collaborative spatial judgments. Pairs of participants made collaborative depth judgments of virtual shapes viewed from the CoP or after displacement forward or backward. Forward and backward displacement caused perceived depth compression and expansion, respectively, with greater compression than expansion. Furthermore, distortion was less than predicted by a ray-intersection model of stereo geometry. Collaboration times were significantly longer when participants stood at different locations compared to the same location, and increased with greater perceived depth discrepancy between the two viewing locations. These findings advance our understanding of spatial distortions in multi-user VEs, and suggest a strategy for reducing distortion.     

An approach using Dempster–Shafer theory to fuse spatial data and satellite image derived crown metrics for estimation of forest stand leading species

Leading species at the forest stand level is a required forest inventory attribute. Information regarding leading species enables the calculation of volume and biomass in support of forest monitoring and reporting activities. In this study, approaches for leading species estimation based upon very high spatial resolution (pixel sided <1 m) have been developed and implemented, with opportunities for improving attribute accuracy using data fusion methods. Over a study region located in the Yukon Territory, Canada, we apply the Dempster–Shafer Theory (DST) to integrate multiple resolutions of satellite imagery (including spatial and spectral), topographic information, and fire disturbance history records for the estimation of leading species.Among the data source combinations tested in the study, the QuickBird panchromatic combined with selected optical channels from Landsat-5 Thematic Mapper (TM) imagery provided the highest overall accuracy (70.4%) for identifying leading species and improved the accuracy by 3.1% over a baseline from a classification-tree based method applied on all data sources. Additional insights to the application of DST to fuse satellite imagery with ancillary data sources to map leading stand species in a boreal environment are also elaborated upon, including the range and distribution of training data and DST mass function establishment.     

Segment-constrained regression tree estimation of forest stand height from very high spatial resolution panchromatic imagery over a boreal environment

Mean stand height is an important parameter for forest volume and biomass estimation in support of monitoring and management activities. Information on mean stand height is typically obtained through the manual interpretation of aerial photography, often supplemented by the collection of field calibration data. In remote areas where forest management practices may not be spatially exhaustive or where it is difficult to acquire aerial photography, alternate approaches for estimating stand height are required. One approach is to use very high spatial resolution (VHSR) satellite imagery (pixels sided less than 1 m) as a surrogate for air photos. In this research we demonstrate an approach for modelling mean stand height at four sites in the Yukon Territory, Canada, from QuickBird panchromatic imagery. An object-based approach was used to generate homogenous segments from the imagery (analogous to manually delineated forest stands) and an algorithm was used to automatically delineate individual tree crowns within the segments. A regression tree was used to predict mean stand height from stand-level metrics generated from the image grey-levels and within-stand objects relating individual tree crown characteristics. Heights were manually interpreted from the QuickBird imagery and divided into separate sets of calibration and validation data. The effects of calibration data set size and the input metrics used on the regression tree results were also assessed. The approach resulted in a model with a significant R² of 0.53 and an RMSE of 2.84 m. In addition, 84.6% of the stand height estimates were within the acceptable error for photo interpreted heights, as specified by the forest inventory standards of British Columbia. Furthermore, residual errors from the model were smallest for the stands that had larger mean heights (i.e., > 20 m), which aids in reducing error in subsequent estimates of biomass or volume (since stands with larger trees contribute more to overall estimates of volume or biomass). Estimated and manually interpreted heights were reclassified into 5-metre height classes (a schema frequently used for forest analysis and modelling applications) and compared; classes corresponded in 54% of stands assessed, and all stands had an estimated height class that was within ± 1 class of their actual class. This study demonstrates the capacity of VHSR panchromatic imagery (in this case QuickBird) for generating useful estimates of mean stand heights in unmonitored, remote, or inaccessible forest areas.     

The contribution of visual and proprioceptive information to the perception of leaning in a dynamic motorcycle simulator

Compared with driving or flight simulation, little is known about self-motion perception in riding simulation. The goal of this study was to examine whether or not continuous roll motion supports the sensation of leaning into bends in dynamic motorcycle simulation. To this end, riders were able to freely tune the visual scene and/or motorcycle simulator roll angle to find a pattern that matched their prior knowledge. Our results revealed idiosyncrasy in the combination of visual and proprioceptive information. Some subjects relied more on the visual dimension, but reported increased sickness symptoms with the visual roll angle. Others relied more on proprioceptive information, tuning the direction of the visual scenery to match three possible patterns. Our findings also showed that these two subgroups tuned the motorcycle simulator roll angle in a similar way. This suggests that sustained inertially specified roll motion have contributed to the sensation of leaning in spite of the occurrence of unexpected gravito-inertial stimulation during the tilt. Several hypotheses are discussed.

Practitioner Summary: Self-motion perception in motorcycle simulation is a relatively new research area. We examined how participants combined visual and proprioceptive information. Findings revealed individual differences in the visual dimension. However, participants tuned the simulator roll angle similarly, supporting the hypothesis that sustained inertially specified roll motion contributes to a leaning sensation.     

One-dimensional axial simulation of electric double layer overlap effects in devices combining micro- and nanochannels

This paper presents a numerical steady-state model of ion transport in micro- and nanofluidic devices with widely varying geometric scale, such as transitions between micro- and nanochannels. Finite element or finite volume simulation of such problems is challenging, due to the number of elements needed to produce a satisfactory mesh. Here, only the lengthwise channel dimension is meshed; standard analytical approximations are used to incorporate cross-channel properties. Singularly perturbed cases are built up by continuation. The method is shown to reproduce our previously reported measurements of electric double-layer effects on conductivity, ion concentration, and ion enhancement and depletion. Comparison with 2-D simulations reported in the literature shows that effects on accuracy due to the 1-D approximation are small. The model incorporates analytical models of surface charge density taken from the literature. This enables predictive simulation with reasonable accuracy using published parameter values, or these values may be tuned based on experiment to give improved results. Use of the model for iterative design and parameter estimation is demonstrated.     

Short-Term Land use Planning and Optimal Subsidies

Networks and Spatial Economics - Urban planning is a complex problem which includes choosing a social objective for a city, finding the associated optimal allocation of agents and identifying...