Facioscapulohumeral Dystrophy (FSHD) results in slowly progressive strength impairment, especially the upper extremities. Recent discoveries regarding pathophysiology have led to exciting novel therapeutic strategies. To further facilitate drug development, improved FSHD outcome measures that are functionally-relevant and sensitive to longitudinal change will be critical. Recently, a motion sensor (Kinect)-based upper extremity outcome called 'reachable workspace' that provides a quantitative reconstruction of an individual's reachability was developed. In this study, changes in reachable workspace were tracked upwards for 5-years in 18 FSHD subjects. Results show −1.63%/year decline in total reachable workspace (p=0.144); with most notable decline in the above-the-shoulder level quadrants (upper-lateral Q3: −9.5%/year, p<0.001 and upper-medial Q1: −6.8%/year, p=0.063) with no significant changes in the lower quadrants (Q2,Q4). Reachable workspace declined more significantly if the subjects were challenged with 500g wrist weights: total reachable workspace: −1.82%/year, p=0.039; Q1: −7.20%/year, p=0.041; Q3: −8.09%/year, p=0.001. Importantly, reachable workspace outcome was also able to distinguish subgroups in FSHD: mildlyand severely-affected with essentially unchanging reachability over years, and moderately-affected who demonstrate the most detectable changes longitudinally. The study demonstrates utility for measuring declines in upper quadrant

Thalamic dementia is an uncommon type of stroke that presents with disorientation, behavioral changes, and impairment of executive functions, with relative preservation of motor functions. It is typically caused by paramedian territory infarctions of the thalamus, most often due to ischemic insult at the tip of the basilar artery. In this report, we present a case of bilateral thalamic infarcts resulting in thalamic dementia with severe behavioral manifestations in a 64-yr-old man with no preexisting neuropsychiatric comorbidities. A trial of amantadine, a dopamine-promoting agent, in the acute rehabilitation unit in an attempt to manage his agitation led to multiple weeks of dramatic behavioral improvement and increased participation in therapies. Dopamine receptors are believed to be present at increased densities in thalamic nuclei with mesolimbic projections, suggesting that they are able to modulate limbic functions such as arousal, emotion, and memory. This case report, aimed both to increase the awareness of this uncommon stroke syndrome and describe the observed effect of amantadine, will ultimately help clinicians properly recognize thalamic dementia, minimize unnecessary investigations, and develop effective neurorehabilitation strategies in these patients.

A method to analyze the variation of the jamming power received by an unmanned aerial vehicle (UAV) receiver according to the change in the attitude of the small UAV is presented. The main interest of the study is in the variation of the polarization loss factor (PLF) in the UAV jamming link due to attitude change, which may be caused by the wind or intended movement. The attitude change is modeled by a combination of three successive rotations using roll, pitch, and yaw, which are defined as UAV rotations. The proposed method is applied to a jamming link, where a small UAV with an omnidirectional antenna is jammed by a jammer antenna on the ground. The method is applied to a plane ground and over a simulated terrain. The variation of the PLF according to the change of UAV attitude may be higher than the generally expected PLF at locations where the height difference between the UAV and the jammer is large, and near the locations where the jammer is located close to the vertical plane containing the rotation axis when the attitude change is represented by one rotation.

No abstract