Journal of Pediatric Rehabilitation Medicine - Volume 13, issue 1

Authors: Crisp, Kelly D. | Case, Laura E. | Kravitz, Richard M. | Kishnani, Priya S. | Jones, Harrison N.

Article Type: Case Report

Abstract: BACKGROUND: Respiratory muscle weakness is a primary cause of morbidity and mortality in patients with Pompe disease. We previously described the effects of our 12-week respiratory muscle training (RMT) regimen in 8 adults with late-onset Pompe disease [1 ] and 2 children with infantile-onset Pompe disease [2 ]. CASE REPORT: Here we describe repeat enrollment by one of the pediatric participants who completed a second 12-week RMT regimen after 7 months of detraining. We investigated the effects of two 12-week RMT regimens (RMT #1, RMT #2) using a single-participant A-B-A experimental design. Primary outcome measures were maximum inspiratory …pressure (MIP) and maximum expiratory pressure (MEP). Effect sizes for changes in MIP and MEP were determined using Cohen’s d statistic. Exploratory outcomes targeted motor function. RELEVANCE : From pretest to posttest, RMT #2 was associated with a 25% increase in MIP and a 22% increase in MEP, corresponding with very large effect sizes (d = 2.92 and d = 2.65, respectively). Following two 12-week RMT regimens over 16 months, MIP increased by 69% and MEP increased by 97%, corresponding with very large effect sizes (d = 3.57 and d = 5.10, respectively). MIP and MEP were largely stable over 7 months of detraining between regimens. Magnitude of change was greater for RMT #1 relative to RMT #2. Show more

Keywords: Pompe disease, glycogen storage disease type II, skeletal muscle, neuromuscular disease, rehabilitation, respiratory muscle training

DOI: 10.3233/PRM-190601

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 13, no. 1, pp. 71-80, 2020

Authors: Clayton, Gerald | Davis, Natalie | Holliday, Adam | Joffe, David | Oakley, David S. | Palermo, Francis X. | Poddar, Sourav | Rueda, Miguel

Article Type: Research Article

Abstract: PURPOSE: Electrophysiological event-related potentials (ERP’s) have been reported to change after concussion. The objective of this study is to use a simple 2-tone auditory P300 ERP in routine clinical settings to measure changes from baseline after concussion and to determine if these changes persist at return to play when other standard measures have normalized. METHODS: Three-hundred sixty-four (364) student athletes, aged 17–23 years, participating in contact sports were tracked over consecutive years. In this blinded study P300, plus physical reaction times and Trail Making tests, were collected alongside standard clinical evaluations. Changes in these measures after concussion …were compared to clinical outcomes over various stages of post-injury recovery. RESULTS: Concussed players experienced significant reaction time and/or P300 amplitude changes compared to pre-concussion baseline measurements (p < 0.005). P300 changes persisted in 38% of the players after standard measures, including reaction times, had cleared. Many of those players slow to normalize were part of the sub-concussive symptom group and/or appeared more prone to repeat concussions. CONCLUSION: These data suggest significant P300 amplitude changes after concussion that are quantifiable and consistent. These changes often normalized slower than other standard assessments. More data are needed to determine if slow normalization relates to sub-concussive or repeated events. Show more

Keywords: Concussion, sports, electroencephalogram, event related potentials, P300, reaction time, trail making, return to play

DOI: 10.3233/PRM-190620

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 13, no. 1, pp. 81-92, 2020

Article Type: Other

DOI: 10.3233/PRM-200018

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 13, no. 1, pp. 93-, 2020

Article Type: Other

DOI: 10.3233/PRM-200001

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 13, no. 1, pp. 95-103, 2020