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Quantifying Kinematic Tremor in an NGLY1-Deficient Individual: A Case Study

Case Reports in Clinical Medicine 2024年第1期13卷 25-36页

作者： Brock Futrell Christopher Alexander Malaya Dacia Martinez Diaz Christian Alfaro hannah elizabeth gustafson Subhalakshmi Chandrasekaran Rhea Mohan Phatak Bernhard Suter Charles Shannon Layne Department of Health and Human Performance University of Houston Houston USA Center for Neuromotor and Biomechanics Research University of Houston Houston USA Department of Computer Science and Engineering Texas A&M University College Station USA Blue Bird Circle Rett Center Texas Children’s Hospital Houston USA Baylor College of Medicine Houston USA Center for NeuroEngineering and Cognitive Science University of Houston Houston USA

NGLY1 Deficiency is an autosomal recessive congenital disorder that has been identified in less than 100 individuals. Most individuals with NGLY1 Deficiency display hyperkinetic movement disorders, including choreiform, athetoid, dystonic myoclonic, dyskinetic, and dysmetric movements. Developing a consistent and concise consensus on the classification and evaluation of tremors is essential to forward the research and treatment of tremors. It has also been reported that some individuals with NGLY1 Deficiency demonstrate tremor, but such tremor has never been formally investigated. The primary objective of this study is to determine if an individual with NGLY1 Deficiency demonstrates an identifiable tremor during a series of arm movements and, if so, describe the frequency and power characteristics of that tremor. Arm movement kinematics were obtained using a 16-camera Vicon system, and time series trajectory waveforms for three planes of a marker placed on the hand were developed. Custom MATLAB scripts were utilized to compute Fast Fourier Transformations of the data within the identified waveform segments. A mean frequency of 2.30 Hz (SD = 1.05) with a mean power of 5.02 |P1(f)| (SD = 4.63) suggests that our participant’s kinematic data did display a persistent tremor in both hands across all tasks and movement planes. Analyses of the reaching hand and the non-reaching hand suggest the participant displayed an action tremor in both postural and intention (kinetic) tremors. Future directions should include assessing additional individuals with NGLY1 Deficiency to determine if the tremor is a distinguishable disorder behavior. Additionally, evaluating other anatomical sites, such as the elbow, head, and lower limbs, would provide further insights into the characteristics of this tremor.

关键词： Tremor NGLY Fast Fourier Transformation Reaching Hyperkinetic Movement

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Quantifying Kinematic Tremor in an NGLY1-Deficient Individual: A Case Study

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Case Reports in Clinical Medicine（临床医学病理报告） 2024年第1期

作者： Brock Futrell Christopher Alexander Malaya Dacia Martinez Diaz Christian Alfaro hannah elizabeth gustafson Subhalakshmi Chandrasekaran Rhea Mohan Phatak Bernhard Suter Charles Shannon Layne

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Using Linear and Non-Linear Techniques to Characterize Gait Coordination Patterns of Two Individuals with NGLY1 Deficiency

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Case Reports in Clinical Medicine 2024年第9期13卷 391-409页

作者： Charles S. Layne Dacia Martinez Diaz Christopher A. Malaya Brock Futrell Christian Alfaro hannah E. gustafson Bernhard Suter Department of Health and Human Performance University of Houston Houston TX USA Center for Neuromotor and Biomechanics Research University of Houston Houston TX USA Center for Neuro Engineering and Cognitive Science University of Houston Houston TX USA Research Center Parker University Dallas TX USA Blue Bird Circle Rett Center Texas Childrens Hospital Houston TX USA Baylor College of Medicine Houston TX USA

Individuals with NGLY1 Deficiency, an inherited autosomal recessive disorder, exhibit hyperkinetic movements including athetoid, myoclonic, dysmetric, and dystonic movements impacting both upper and lower limb motion. This report provides the first set of laboratory-based measures characterizing the gait patterns of two individuals with NGLY1 Deficiency, using both linear and non-linear measures, during treadmill walking, and compares them to neurotypical controls. Lower limb kinematics were obtained with a camera-based motion analysis system and bilateral time normalized lower limb joint time series waveforms were developed. Linear measures of joint range of motion, stride times and peak angular velocity were obtained, and confidence intervals were used to determine if there were differences between the patients and control. Correlations between participant and control mean joint waveforms were calculated and used to evaluate the similarities between patients and controls. Non-linear measures included: joint angle-angle diagrams, phase-portrait areas, and continuous relative phase (CRP) measures. These measures were used to assess joint coordination and control features of the lower limb motion. Participants displayed high correlations with their control counterparts for the hip and knee joint waveforms, but joint motion was restricted. Peak angular velocities were also significantly less than those of the controls. Both angle-angle and phase-portrait areas were less than the controls although the general shapes of those diagrams were similar to those of the controls. The NGLY1 Deficient participants’ CRP measures displayed disrupted coordination patterns with the knee-ankle patterns displaying more disruption than the hip-knee measures. Overall, the participants displayed a functional walking pattern that differed in many quantitative ways from those of the neurotypical controls. Using both linear and non-linear measures to characterize gait provides a more compre

关键词： NGLY1 Gait Disability Kinematics Angle-Angle Diagrams Phase Portraits

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3-Dimensional Kinematic Comparison of Arm Movements between an Individual with NGLY1 Deficiency and a Neurotypical Individual

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Case Reports in Clinical Medicine 2024年第4期13卷 122-146页

作者： Charles S. Layne Christopher A. Malaya Brock Futrell Dacia Martinez Diaz Christian Alfaro hannah E. gustafson Subhalakshmi Chandrasekaran Rhea M. Phatak Bernhard Suter Department of Health and Human Performance University of Houston Houston TX USA Center for Neuromotor and Biomechanics Research University of Houston Houston TX USA Center for Neuro Engineering and Cognitive Science University of Houston Houston TX USA Department of Computer Science and Engineering Texas A&M University College Station TX USA Blue Bird Circle Rett Center Texas Children&#8217s Hospital Houston TX USA Baylor College of Medicine Houston TX USA

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 stati

关键词： NGLY1 Deficiency Developmental Disorders Kinematics 3 Dimensional Analyses

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3-Dimensional Kinematic Comparison of Arm Movements between an Individual with NGLY1 Deficiency and a Neurotypical Individual

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Case Reports in Clinical Medicine（临床医学病理报告） 2024年第4期 122页

作者： Charles S. Layne Christopher A. Malaya Brock Futrell Dacia Martinez Diaz Christian Alfaro hannah E. gustafson Subhalakshmi Chandrasekaran Rhea M. Phatak Bernhard Suter

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Effects of 7 years of field weathering on biochar recalcitrance and solubility

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Biochar 2019年第3期1卷 237-248页

作者： elizabeth K.Williams Davey L.Jones hannah R.Sanders Gabriel V.Benitez Alain F.Plante University of Pennsylvania PhiladelphiaPAUSA Bangor University BangorUK The University of Western Australia PerthAustralia University of Minnesota MinneapolisMNUSA

How weathering affects the physiochemical properties of biochar and its long-term carbon(C)sequestration potential remains *** this study,we measured changes in biochar recalcitrance and solubility after 7 years of weathering in a cultivated *** recalcitrance and biodegradability of weathered and unweathered hardwood biochar mixtures were determined by thermal analysis(differential scanning calorimetry)and evolved gas *** in biochar solubility and the chemical composition of biochar-derived dissolved organic carbon(DOC)were determined by repeated laboratory leaching and UV-Vis *** surface carbon-oxidation state(Cox)of biochar increased by 117.6-158.2%with weather-ing in the field,and there was an average of 0.9-1.2%decrease in biochar C contents per ***,thermal indices of biochar recalcitrance and biodegradability,which suggested intermediate C sequestration potential,were not significantly different between weathered and unweathered *** O:C ratio increased with weathering suggesting an increase in biodegradability,however,both weathered and unweathered biochars were still estimated to have half-lives of over 1000 ***-soluble organic carbon(WSOC)concentrations from the unweathered biochar rapidly decreased during laboratory leaching to levels similar to the field-weathered biochars,and aromaticity(SUVA_(254))of WSOC increased from 5.9%in the unweathered biochar to 42%aromaticity in the biochars weathered for 7 *** conclude that during short-term(years)weathering under field conditions,there is continued solubilization of increasingly aromatic biochar-C compounds,however,this accounts for a relatively small proportion of biochar C such that there is little-to-no change in biochar stability or C sequestration potential after field application.

关键词： Biochar Pyrogenic organic matter Thermal analysis Carbon sequestration Dissolved pyrogenic organic matter

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Salsa dance and Zumba fitness: Acute responses during community-based classes

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Journal of Sport and Health Science 2016年第2期5卷 190-196页

作者： Pablo A.Domene hannah J.Moira elizabeth Pummell Chris Easton Faculty of Science Engineering and Computing Kingston University Institute for Clinical Exercise and Health Science University of the West of Scotland

Background： Research interest in both partnered Latin dance and non-partnered Latin-themed aerobic dance has increased in recent years, likely a result of the gaining popularity of these types of instructor-led group classes among the mainstream dance and fitness audiences; however, the efficacy of these activities for the purposes of health promotion currently remains unclear. The purpose of this study was to simultaneously assess the physiological responses and psychological experiences during salsa dance and Zumba fitness in a community sample of physically inactive women. Methods： Twenty-four participants, aged 22-56 years, visited the laboratory to perform a graded exercise test for determination of maximal oxygen uptake and maximal heart rate. The participants then attended 2 partnered salsa dance and 2 non-partnered Zumba fitness classes each in a counterbalanced order over a 2-week period. The 1-h classes were taught by certified instructors in established venues in the Royal Borough of Kingston and the surrounding communities of London, UK. Physiological data were collected using a wrist-worn ActiGraph wGT3X＋ acceler- ometer with accompanying heart rate monitor and were processed using previously validated dance-specific techniques. Psychological experiences were measured via the Subjective Exercise Experiences Scale. Results： There was a significantly higher （p 〈 0.001） total time spent in moderate-to-vigorous physical activity （51.2± 3.1 vs. 32.6 ±5.9 min）, total energy expenditure （411±66 vs. 210 ± 46 kcal）, and total step count （6773 ± 556 vs. 4108 ±781 steps） during Zumba fitness when compared to salsa dance. Significant pre- to post-class improvements in positive well-being （p 〈 0.01, partial 7/2 = 0.4l） and psychological distress （p 〈 0.001, partial 02 = 0.72） were simultaneously observed for both salsa dance and Zumba fitness. Conclusion： The acute responses to classes of partnered Latin dance and non-partnered Latin-themed aerobic dance suggest tha

关键词： Accelerometry Aerobics Cultural dance Exercise Physical acti,~ity

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A critical literature review of bioretention research for stormwater management in cold climate and future research recommendations

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Frontiers of Environmental Science & Engineering 2017年第4期11卷 23-37页

作者： hannah Kratky Zhan Li Yijun Chen Chengjin Wang Xiangfei Li Tong Yu Department of Civil and Environmental Engineering University of Alberta Edmonton Alberta T6G 1H9 Canada City Planning City of Edmonton Edmonton Alberta T5J 0J4 Canada

Bioretention is a popular best management practice of low impact development that el/ecUvely restores urban hydrologic characteristics to those ofpredevelopment and improves water quality prior to conveyance to surface waters. This is achieved by utilizing an engineered system containing a surface layer of mulch, a thick soil media often amended with a variety of materials to improve water oualitv, a variety of vegetation, and underdrains, depending on the surrounding soil *** systems have been studied quite extensively for warm climate applications, but ctata strongly supporting their long-tema efficacy and application in cold climates is sparse. Although it is apparent that biorelention is an effective stormwater management system, its design in cold climate needs further research. Existing cold climate research has shown that coarser media is required to prevent concrete frost from forming. For spring, summer and fall seasons, if sufficient permeability exists to drain the system prior to freezing, peak flow and volume reduction can be maintained. Additionally. contaminants that are removed via filtration are also not impacted by cold climates. In contrary, dissolved contaminants, nutrients, and organics are significantly more variable in their ability to be removed or degraded via bioretention in colder temperatures. Winter road maintenance salts have been shown to negatively impact the removal of some contaminants and positively impact others, while their effects on properly selected vegetation or bacteria health are also not well understood. Research in these water quality aspects has been inconsistent and therefore requires further study.

关键词： Bioretention Cold climate Low impact development Stormwater

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Effects of primary glaucoma on sleep quality and daytime sleepiness of patients residing at an equatorial latitude

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International Journal of Ophthalmology(English edition) 2020年第9期13卷 1451-1458页

作者： Jacob YH Chin Zhi Hong Toh Ying Tai Lo hannah TY Wang elizabeth YW Poh Chun Hau Chua Owen Kim Hee Boon Ang Lim Vernon KY Yong Augustinus Laude Hon Tym Wong Leonard WL Yip Department of Ophthalmology National Healthcare Group Eye InstituteTan Tock Seng Hospital308433Singapore Monash School of Medicine Monash UniversityVictoria 3800Australia

AIM:To investigate the impact of primary glaucoma on sleep quality and daytime sleepiness of ***:Prospective cross-sectional study with consecutive sampling in South-East Asian population was *** questionnaires:the Pittsburg Sleep Quality Index(PSQI)and Epworth Sleepiness Scale(ESS)were administered *** with nonglaucomatous optic neuropathy or concomitant retinal pathology were *** severity was based on HVF 24-2 *** single vision was represented based on the better *** of and predictive factors for poor sleep quality and excessive daytime sleepiness were ***:A total of 79 primary open angle glaucoma(POAG),27 primary angle-closure glaucoma(PACG)patients,and 89 controls were *** patients had higher median PSQI scores(P=0.004)and poorer sleep quality(P<0.001).Compared to controls,PACG patients were 3.34 times more likely to have poor sleep quality(P=0.008),which remained significant after adjustment for demographics(P=0.016)and predictive variables(P=0.013).PACG patients have poorer sleep quality when visual acuity(VA)was 6/15 or worse(P=0.009).Univariate and multivariate analysis of predictive variables for poor sleep quality and daytime sleepiness did not find statistical ***:PACG patients have poorer sleep quality but not daytime *** is important in South-East Asian population with heavy disease *** on sleep disturbances can be considered to provide more ho istic care.

关键词： sleep disturbances primary glaucoma intrinsically photosensitive retinal ganglion cells daytime sleepiness sleep quality South-East Asia

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Multi-scale phylogenetic structure in coastal dune plant communities across the globe

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Journal of Plant Ecology 2014年第2期7卷 101-114页

作者： Ane Kirstine Brunbjerg Jeannine Cavender-Bares Wolf L.Eiserhardt Rasmus Ejrnæs Lonnie W.Aarssen hannah L.Buckley Estelle Forey Florian Jansen Jens Kattge Cynthia Lane Roy A.Lubke Angela T.Moles Ana Laura Monserrat Robert K.Peet Julissa Roncal Louise Wootton Jens-Christian Svenning Ecoinformatics&Biodiversity Group Department of BioscienceAarhus UniversityNy Munkegade 114DK-8000 Aarhus C.Denmark Department of Bioscience Aarhus UniversityGrenaavej 14DK-8410 RøndeDenmark Department of Ecology Evolution and BehaviorUniversity of Minnesota1987 Upper Buford CircleFalcon HeightsMN 55108USA Department of Biology Queens University116 Barrie StreetKingstonOntario K7L 3N6Canada Department of Ecology Lincoln UniversityPO Box 84Canterbury 7647New Zealand Laboratoire d’Ecologie EA 1293 ECODIVFED SCALEUFR Sciences et TechniquesUniversitéde Rouen76821 Mont Saint Aignan CedexFrance Institute of Botany and Landscape Ecology Soldmannstr.1517487 GreifswaldGermany Functional Biogeography Research Group Max Planck Institute for BiogeochemistryHans Knoell Str.1007745 JenaThuringiaGermany Lake Lily Ecology Ltd.PO Box 8455CanmoreAB T1W 2V2Canada Department of Botany Rhodes UniversityPO Box 94GrahamstownEast Cape 6140South Africa Evolution&Ecology Research Centre School of BiologicalEarth and Environmental SciencesThe University of New South WalesBotany RoadKensingtonSydneyNew South Wales 2034Australia Grupo de Investigación en Sistemas Ambientales Complejos Facultad de IngenieríaUniversidad de FloresPedernera 275Ciudad de Buenos AiresArgentina Department of Biology University of North CarolinaChapel HillNC 27599-3280USA Department of Biology Memorial University of Newfoundland232 Elizabeth AvenueSt.John'sNL A1B 3X9Canada Department of Biology Georgian Court University900 Lakewood AvenueLakewoodNJ 08701USA

Aims Studies integrating phylogenetic history and large-scale community assembly are few,and many questions remain ***,we use a global coastal dune plant data set to uncover the important factors in community assembly across scales from the local filtering processes to the global long-term diversification and dispersal *** dune plant communities occur worldwide under a wide range of climatic and geologic conditions as well as in all biogeographic ***,global patterns in the phylogenetic composition of coastal dune plant communities have not previously been *** The data set comprised vegetation data from 18463 plots in New Zealand,South Africa,South America,North America and *** phylogenetic tree comprised 2241 plant species from 149 *** calculated phylogenetic clustering(Net Relatedness Index,NRI,and Nearest Taxon Index,NTI)of regional dune floras to estimate the amount of in situ diversification relative to the global dune species pool and evaluated the relative importance of land and climate barriers for these diversification patterns by geographic analyses of phylogenetic *** then tested whether dune plant communities exhibit similar patterns of phylogenetic structure within ***,we calculated NRI for local communities relative to the regional species pool and tested for an association with functional traits(plant height and seed mass)thought to vary along sea–inland *** Findings Regional species pools were phylogenetically clustered relative to the global pool,indicating regional *** showed stronger clustering than NRI pointing to the importance of especially recent diversifications within *** species pools grouped phylogenetically into two clusters on either side of the tropics suggesting greater dispersal rates within hemispheres than between *** NRI plot values confirmed that most communities were also phylogenetically clus

关键词： disturbance phylogenetic community structure plant functional traits plant height seed mass

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