Browsing by Subject "Motor Skills"

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Open Access
Early cognitive development in children with infantile Pompe disease.
(Molecular genetics and metabolism, 2012-03) Spiridigliozzi, Gail A; Heller, James H; Case, Laura E; Jones, Harrison N; Kishnani, Priya S
This report describes the cognitive development of 17 children with infantile Pompe disease who participated in a 52-week clinical trial of enzyme replacement therapy (ERT) via biweekly infusion of Myozyme® (alglucosidase alfa). Subjects were six months of age or younger (adjusted for gestational age) upon initiation of ERT. The Mental Scale of the Bayley Scales of Infant Development-Second Edition (BSID-II) was administered to obtain a Mental Development Index (MDI) at baseline and weeks 12, 26, 38, and 52 of ERT to assess cognitive development in this treated cohort. Data regarding motor development were also obtained at the same visits and these were used to determine correlations between cognitive and motor development. Over the course of the study, two subgroups of subjects emerged: high responders who were sitting independently and/or ambulating by week 52 (n=13) and limited responders who showed minimal motor gains throughout the first year of ERT (n=4). In the high responder group, MDI scores on the BSID-II remained stable throughout the study and were within normal limits. Positive correlations between cognitive and motor development were also present. These data suggest that the cognitive function of infants up to 18 months of age with Pompe disease is unaffected by the possible presence of glycogen in the central nervous system. Continued investigation of the cognitive development of older survivors is warranted.
Open Access
Effect of Autologous Cord Blood Infusion on Motor Function and Brain Connectivity in Young Children with Cerebral Palsy: A Randomized, Placebo-Controlled Trial.
(Stem cells translational medicine, 2017-12) Sun, Jessica M; Song, Allen W; Case, Laura E; Mikati, Mohamad A; Gustafson, Kathryn E; Simmons, Ryan; Goldstein, Ricki; Petry, Jodi; McLaughlin, Colleen; Waters-Pick, Barbara; Chen, Lyon W; Wease, Stephen; Blackwell, Beth; Worley, Gordon; Troy, Jesse; Kurtzberg, Joanne
Cerebral palsy (CP) is a condition affecting young children that causes lifelong disabilities. Umbilical cord blood cells improve motor function in experimental systems via paracrine signaling. After demonstrating safety, we conducted a phase II trial of autologous cord blood (ACB) infusion in children with CP to test whether ACB could improve function (ClinicalTrials.gov, NCT01147653; IND 14360). In this double-blind, placebo-controlled, crossover study of a single intravenous infusion of 1-5 × 10⁷ total nucleated cells per kilogram of ACB, children ages 1 to 6 years with CP were randomly assigned to receive ACB or placebo at baseline, followed by the alternate infusion 1 year later. Motor function and magnetic resonance imaging brain connectivity studies were performed at baseline, 1, and 2 years post-treatment. The primary endpoint was change in motor function 1 year after baseline infusion. Additional analyses were performed at 2 years. Sixty-three children (median age 2.1 years) were randomized to treatment (n = 32) or placebo (n = 31) at baseline. Although there was no difference in mean change in Gross Motor Function Measure-66 (GMFM-66) scores at 1 year between placebo and treated groups, a dosing effect was identified. In an analysis 1 year post-ACB treatment, those who received doses ≥2 × 10⁷ /kg demonstrated significantly greater increases in GMFM-66 scores above those predicted by age and severity, as well as in Peabody Developmental Motor Scales-2 Gross Motor Quotient scores and normalized brain connectivity. Results of this study suggest that appropriately dosed ACB infusion improves brain connectivity and gross motor function in young children with CP. Stem Cells Translational Medicine 2017;6:2071-2078.
Open Access
Mapping the structure of perceptual and visual-motor abilities in healthy young adults.
(Acta Psychol (Amst), 2015-05) Wang, Lingling; Krasich, Kristina; Bel-Bahar, Tarik; Hughes, Lauren; Mitroff, Stephen R; Appelbaum, L Gregory
The ability to quickly detect and respond to visual stimuli in the environment is critical to many human activities. While such perceptual and visual-motor skills are important in a myriad of contexts, considerable variability exists between individuals in these abilities. To better understand the sources of this variability, we assessed perceptual and visual-motor skills in a large sample of 230 healthy individuals via the Nike SPARQ Sensory Station, and compared variability in their behavioral performance to demographic, state, sleep and consumption characteristics. Dimension reduction and regression analyses indicated three underlying factors: Visual-Motor Control, Visual Sensitivity, and Eye Quickness, which accounted for roughly half of the overall population variance in performance on this battery. Inter-individual variability in Visual-Motor Control was correlated with gender and circadian patters such that performance on this factor was better for males and for those who had been awake for a longer period of time before assessment. The current findings indicate that abilities involving coordinated hand movements in response to stimuli are subject to greater individual variability, while visual sensitivity and occulomotor control are largely stable across individuals.
Open Access
Neurodevelopmental outcomes of umbilical cord blood transplantation in metachromatic leukodystrophy.
(Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation, 2013-04) Martin, Holly R; Poe, Michele D; Provenzale, James M; Kurtzberg, Joanne; Mendizabal, Adam; Escolar, Maria L
Metachromatic leukodystrophy (MLD) is an inherited demyelinating disease that causes progressive neurologic deterioration, leading to severe motor disability, developmental regression, seizures, blindness, deafness, and death. The disease presents as a late-infantile, juvenile, or adult form. Hematopoietic stem cell transplantation has been shown to slow disease progression. The purpose of this longitudinal study was to evaluate long-term treatment outcomes after unrelated donor umbilical cord blood (UCB) transplantation in pediatric patients according to disease burden and age at onset (ie, late-infantile versus juvenile). Engraftment, survival, treatment-related toxicity, graft-versus-host disease, neurophysiologic measures, and neurodevelopmental function were assessed. To evaluate whether signal intensity abnormalities on magnetic resonance imaging (ie, modified Loes scores) predict post-transplant cognitive and gross motor development, a general linear mixed model was fit to the data. Twenty-seven patients underwent transplantation after myeloablative chemotherapy; 24 patients engrafted after the initial transplantation. Seven patients died of infection, regimen-related toxicity, or disease progression. Twenty patients (6 with late-infantile onset and 14 with juvenile onset) were followed for a median of 5.1 years (range, 2.4 to 14.7). We found that patients with motor function symptoms at the time of transplant did not improve after transplantation. Brainstem auditory evoked responses, visual evoked potentials, electroencephalogram, and/or peripheral nerve conduction velocities stabilized or improved in juvenile patients but continued to worsen in most patients with the late-infantile presentation. Pretransplant modified Loes scores were highly correlated with developmental outcomes and predictive of cognitive and motor function. Children who were asymptomatic at the time of transplantation benefited most from the procedure. Children with juvenile onset and minimal symptoms showed stabilization or deterioration of motor skills but maintained cognitive skills. Overall, children with juvenile onset had better outcomes than those with late-infantile onset. As in other leukodystrophies, early intervention correlated with optimal outcomes. We conclude that UCB transplantation benefits children with presymptomatic late-infantile MLD or minimally symptomatic juvenile MLD.
Open Access
Visual abilities distinguish pitchers from hitters in professional baseball.
(Journal of sports sciences, 2018-01) Klemish, David; Ramger, Benjamin; Vittetoe, Kelly; Reiter, Jerome P; Tokdar, Surya T; Appelbaum, Lawrence Gregory
This study aimed to evaluate the possibility that differences in sensorimotor abilities exist between hitters and pitchers in a large cohort of baseball players of varying levels of experience. Secondary data analysis was performed on 9 sensorimotor tasks comprising the Nike Sensory Station assessment battery. Bayesian hierarchical regression modelling was applied to test for differences between pitchers and hitters in data from 566 baseball players (112 high school, 85 college, 369 professional) collected at 20 testing centres. Explanatory variables including height, handedness, eye dominance, concussion history, and player position were modelled along with age curves using basis regression splines. Regression analyses revealed better performance for hitters relative to pitchers at the professional level in the visual clarity and depth perception tasks, but these differences did not exist at the high school or college levels. No significant differences were observed in the other 7 measures of sensorimotor capabilities included in the test battery, and no systematic biases were found between the testing centres. These findings, indicating that professional-level hitters have better visual acuity and depth perception than professional-level pitchers, affirm the notion that highly experienced athletes have differing perceptual skills. Findings are discussed in relation to deliberate practice theory.
Open Access
Visual-motor expertise in athletes: Insights from semiparametric modelling of 2317 athletes tested on the Nike SPARQ Sensory Station.
(Journal of sports sciences, 2020-02) Burris, Kyle; Liu, Sicong; Appelbaum, Lawrence
Elite athletes not only run faster, hit harder, and jump higher, but also see and react better. However, the specific visual-motor skills that differentiate high-achieving athletes are still not well understood. In this paper we examine 2317 athletes (1871 male) tested on the Nike SPARQ Sensory Station, a digital test battery measuring visual, perceptual and motor skills relevant for sports performance. We develop a multivariate Gaussian transformation model to robustly estimate visual-motor differences by level, gender, and sport type. Results demonstrate that visual-motor performance is superior for athletes at higher levels, with males faster at near-far eye movements and females faster at eye-hand reaction times. Interestingly, athletes who play interceptive sports such as baseball and tennis exhibit better measures of visual clarity, contrast sensitivity and simple reaction time, while athletes from strategic sports like soccer and basketball have higher measures of spatial working memory. These findings provide quantitative evidence of domain-specific visual expertise in athletes.