NGLY1 Deficiency is an ultra-rare autosomal recessively inherited disorder. Characteristic symptoms include among others, developmental delays, movement disorders, liver function abnormalities, seizures, and problems ...NGLY1 Deficiency is an ultra-rare autosomal recessively inherited disorder. Characteristic symptoms include among others, developmental delays, movement disorders, liver function abnormalities, seizures, and problems with tear formation. Movements are hyperkinetic and may include dysmetric, choreo-athetoid, myoclonic and dystonic movement elements. To date, there have been no quantitative reports describing arm movements of individuals with NGLY1 Deficiency. This report provides quantitative information about a series of arm movements performed by an individual with NGLY1 Deficiency and an aged-matched neurotypical participant. Three categories of arm movements were tested: 1) open ended reaches without specific end point targets;2) goal-directed reaches that included grasping an object;3) picking up small objects from a table placed in front of the participants. Arm movement kinematics were obtained with a camera-based motion analysis system and “initiation” and “maintenance” phases were identified for each movement. The combination of the two phases was labeled as a “complete” movement. Three-dimensional analysis techniques were used to quantify the movements and included hand trajectory pathlength, joint motion area, as well as hand trajectory and joint jerk cost. These techniques were required to fully characterize the movements because the NGLY1 individual was unable to perform movements only in the primary plane of progression instead producing motion across all three planes of movement. The individual with NGLY1 Deficiency was unable to pick up objects from a table or effectively complete movements requiring crossing the midline. The successfully completed movements were analyzed using the above techniques and the results of the two participants were compared statistically. Almost all comparisons revealed significant differences between the two participants, with a notable exception of the 3D initiation area as a percentage of the complete movement. The statistical tests of these measures revealed no significant differences between the two participants, possibly suggesting a common underlying motor control strategy. The 3D techniques used in this report effectively characterized arm movements of an individual with NGLY1 deficiency and can be used to provide information to evaluate the effectiveness of genetic, pharmacological, or physical rehabilitation therapies.展开更多
Tunnel heading stability in two dimensions(2D)has been extensively investigated by numerous scholars in the past decade.One significant limitation of 2D analysis is the absence of actual tunnel geometry modeling with ...Tunnel heading stability in two dimensions(2D)has been extensively investigated by numerous scholars in the past decade.One significant limitation of 2D analysis is the absence of actual tunnel geometry modeling with a considerable degree of idealization.Nevertheless,it is possible to study the stability of tunnels in three dimensions(3D)with a rectangular shape using finite element limit analysis(FELA)and a nonlinear programming technique.This paper employs 3D FELA to generate rigorous solutions for stability numbers,failure mechanisms,and safety factors for rectangular-shaped tunnels.To further explore the usefulness of the produced results,multivariate adaptive regression spline(MARS)is used for machine learning of big dataset and development of design equations for practical design applications.The study should be of great benefit to tunnel design practices using the developed equations provided in the paper.展开更多
The focusing tomography is presented to reconstruct 3 dimensional irradiance distribution. A 3 dimensional luminous body could be considered as the combination of many 2 dimensional parallel luminous sections. Focu...The focusing tomography is presented to reconstruct 3 dimensional irradiance distribution. A 3 dimensional luminous body could be considered as the combination of many 2 dimensional parallel luminous sections. Focus on these discrete sections respectively by a single camera, a group of images would be captured to form governing equations of irradiance. After inversion procedure, the irradiance distribution of different sections could be decoded. In this experimentation two lightbulbs are used to simulate two luminous sections. Reasonable results demonstrate that this technique could be a useful method in irradiance reconstruction after further development.展开更多
文摘NGLY1 Deficiency is an ultra-rare autosomal recessively inherited disorder. Characteristic symptoms include among others, developmental delays, movement disorders, liver function abnormalities, seizures, and problems with tear formation. Movements are hyperkinetic and may include dysmetric, choreo-athetoid, myoclonic and dystonic movement elements. To date, there have been no quantitative reports describing arm movements of individuals with NGLY1 Deficiency. This report provides quantitative information about a series of arm movements performed by an individual with NGLY1 Deficiency and an aged-matched neurotypical participant. Three categories of arm movements were tested: 1) open ended reaches without specific end point targets;2) goal-directed reaches that included grasping an object;3) picking up small objects from a table placed in front of the participants. Arm movement kinematics were obtained with a camera-based motion analysis system and “initiation” and “maintenance” phases were identified for each movement. The combination of the two phases was labeled as a “complete” movement. Three-dimensional analysis techniques were used to quantify the movements and included hand trajectory pathlength, joint motion area, as well as hand trajectory and joint jerk cost. These techniques were required to fully characterize the movements because the NGLY1 individual was unable to perform movements only in the primary plane of progression instead producing motion across all three planes of movement. The individual with NGLY1 Deficiency was unable to pick up objects from a table or effectively complete movements requiring crossing the midline. The successfully completed movements were analyzed using the above techniques and the results of the two participants were compared statistically. Almost all comparisons revealed significant differences between the two participants, with a notable exception of the 3D initiation area as a percentage of the complete movement. The statistical tests of these measures revealed no significant differences between the two participants, possibly suggesting a common underlying motor control strategy. The 3D techniques used in this report effectively characterized arm movements of an individual with NGLY1 deficiency and can be used to provide information to evaluate the effectiveness of genetic, pharmacological, or physical rehabilitation therapies.
基金supported by the Thailand Science Research and Innovation Fundamental Fund fiscal year 2023The fifth author (V.Kamchoom)acknowledges the financial support from the National Science,Research and Innovation Fund (NSRF)at King Mongkut's Institute of Technology Ladkrabang (KMITL),Thailand (Grant No.FRB66065/0258-RE-KRIS/FF66/53)+1 种基金the Climate Change and Climate Variability Research in Monsoon Asia (CMON3)from the National Research Council of Thailand (NRCT) (Grant No.N10A650844)the National Natural Science Foundation of China (NSFC).
文摘Tunnel heading stability in two dimensions(2D)has been extensively investigated by numerous scholars in the past decade.One significant limitation of 2D analysis is the absence of actual tunnel geometry modeling with a considerable degree of idealization.Nevertheless,it is possible to study the stability of tunnels in three dimensions(3D)with a rectangular shape using finite element limit analysis(FELA)and a nonlinear programming technique.This paper employs 3D FELA to generate rigorous solutions for stability numbers,failure mechanisms,and safety factors for rectangular-shaped tunnels.To further explore the usefulness of the produced results,multivariate adaptive regression spline(MARS)is used for machine learning of big dataset and development of design equations for practical design applications.The study should be of great benefit to tunnel design practices using the developed equations provided in the paper.
文摘The focusing tomography is presented to reconstruct 3 dimensional irradiance distribution. A 3 dimensional luminous body could be considered as the combination of many 2 dimensional parallel luminous sections. Focus on these discrete sections respectively by a single camera, a group of images would be captured to form governing equations of irradiance. After inversion procedure, the irradiance distribution of different sections could be decoded. In this experimentation two lightbulbs are used to simulate two luminous sections. Reasonable results demonstrate that this technique could be a useful method in irradiance reconstruction after further development.
