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OBJECTIVE: To determine if amantadine use in pediatric patients with traumatic brain injury is well tolerated and to attempt to assess its effectiveness. DESIGN: This was a retrospective, case-controlled study. RESULTS: Of the 54 patients, aged 3-18 yrs, who were administered amantadine, five (9%) had side effects that might have been related to the drug. These included hallucinations, delusions, increased aggression, and nausea/vomiting. The side effects were reversed when the medication was stopped or the dosage decreased. Patients in the amantadine group had a greater increase in Ranchos Los Amigos level during their admission than those in the control group (median, 3 vs. 2; P < 0.01). This difference may be, at least in part, explained by the fact that the amantadine group started at a lower Ranchos Los Amigos level (median, 3 vs. 4; P < 0.01). There were subjective improvements noted in 29 of the 46 patients (63%) in the amantadine group whose full charts were available for review. CONCLUSION: Amantadine is a well tolerated medication when it is used in pediatric patients with traumatic brain injury. Subjective improvements were noted in the majority of the patients administered amantadine, and the amantadine group showed a greater improvement in Ranchos Los Amigos level during admission, suggesting that it may be effective. [ABSTRACT FROM AUTHOR]

Becker's muscular dystrophy (BMD) is associated with abnormal cardiac findings in 75% of cases; up to one third will develop ventricular dilatation leading to congestive heart failure, at times necessitating cardiac transplant. Candidates are selected from a base of heart failure patients who are usually New York Heart Association (NYHA) class III or IV. Treatment in a phase II cardiac rehabilitation program after transplantation is associated with functional improvement in patients without BMD, but there are no reports of patients with this disorder. We present the case of a 38-year-old man diagnosed with BMD with associated dilated cardiomyopathy. The patient was a NYHA class IIIa and underwent orthotopic cardiac transplantation for intractable heart failure followed by treatment in a phase II cardiac rehabilitation program. At the end of cardiac rehabilitation, his 12-minute walking distance had improved from 716.28 to 929.64 m (30% improvement), he had increased his conditioning metabolic equivalent level from 3.5 to 5.5 (55% improvement), he had a weight loss from 81.65 to 78.93 kg, and his body mass index changed from 23 to 22 kg/m2. The patient now has returned to work, is using a stationary bicycle once a day for 30 minutes, and is walking 1 hour a day. This suggests that treatment in a cardiac rehabilitation program is effective in patients with BMD after cardiac transplant. Copyright © 2005 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation [ABSTRACT FROM AUTHOR]

Background: The American Academy of Physical Medicine and Rehabilitation (AAPM&R) conducted a comprehensive review in 2021 to identify opportunities for enhancing the care of adult and pediatric patients with spasticity. A technical expert panel (TEP) was convened to develop consensus-based practice recommendations aimed at addressing gaps in spasticity care., Objective: To develop consensus-based practice recommendations to identify and address gaps in spasticity care., Methods: The Spasticity TEP engaged in a 16-month virtual meeting process, focusing on formulating search terms, refining research questions, and conducting a structured evidence review. Evidence quality was assessed by the AAPM&R Evidence, Quality and Performance Committee (EQPC), and a modified Delphi process was employed to achieve consensus on recommendation statements and evidence grading. The Strength of Recommendation Taxonomy (SORT) guided the rating of individual studies and the strength of recommendations., Results: The TEP approved five recommendations for spasticity management and five best practices for assessment and management, with one recommendation unable to be graded due to evidence limitations. Best practices were defined as widely accepted components of care, while recommendations required structured evidence reviews and grading. The consensus guidance statement represents current best practices and evidence-based treatment options, intended for use by PM&R physicians caring for patients with spasticity., Conclusion: This consensus guidance provides clinicians with practical recommendations for spasticity assessment and management based on the best available evidence and expert opinion. Clinical judgment should be exercised, and recommendations tailored to individual patient needs, preferences, and risk profiles. The accompanying table summarizes the best practice recommendations for spasticity assessment and management, reflecting principles with little controversy in care delivery., (© 2024 American Academy of Physical Medicine and Rehabilitation.)

This international multidisciplinary document is intended to guide electrophysiologists, cardiologists, other clinicians, and health care professionals in caring for patients with arrhythmic complications of neuromuscular disorders (NMDs). The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2; myotonic dystrophy type 1 and type 2; Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B; facioscapulohumeral muscular dystrophy; and mitochondrial myopathies, including Friedreich ataxia and Kearns-Sayre syndrome, with an emphasis on managing arrhythmic cardiac manifestations. End-of-life management of arrhythmias in patients with NMDs is also covered. The document sections were drafted by the writing committee members according to their area of expertise. The recommendations represent the consensus opinion of the expert writing group, graded by class of recommendation and level of evidence utilizing defined criteria. The recommendations were made available for public comment; the document underwent review by the Heart Rhythm Society Scientific and Clinical Documents Committee and external review and endorsement by the partner and collaborating societies. Changes were incorporated based on these reviews. By using a breadth of accumulated available evidence, the document is designed to provide practical and actionable clinical information and recommendations for the diagnosis and management of arrhythmias and thus improve the care of patients with NMDs., (Copyright © 2022 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: The prevalence of obesity in adolescents with Down syndrome (DS) far exceeds that in the general population. Cortisol, an adrenal hormone, can be obesogenic when dysregulated. However, the diurnal patterns of this hormone have not been examined among individuals with DS. Variations in adiposity may also mediate cortisol regulation. This study sought to examine diurnal cortisol patterns in adolescents with DS as well as associations between cortisol function and obesity., Method: A total of 32 adolescents, including 16 with DS and 16 controls with typical development (TD) of similar sex, age and Tanner pubertal stage (P > 0.05), participated in this preliminary study. Participants completed a dual-energy X-ray absorptiometry scan to measure body composition and collected saliva samples for cortisol measurements in the morning, afternoon and night. Linear mixed models with random intercepts and repeated measures were used to examine the daily trajectory of log-transformed cortisol concentrations between adolescents with and without DS. A second model examined the interaction between DS and presence of elevated body fatness., Results: Adolescents with DS had higher morning cortisol concentrations (intercept = 0.37 μg/dL), but this was not significantly different than in TD (0.35 μg/dL, P = 0.16). Cortisol significantly declined across hours (b = -0.026 μg/dL/h, P < 0.001), but this decline also did not differ from that observed in TD (b = -0.024 μg/dL/h, P = 0.43). While cortisol levels were slightly higher among adolescents with elevated body fatness, this difference was not statistically significant (P > 0.05; d = 0.30)., Conclusions: This study is the first to examine diurnal cortisol in DS but is limited in sample size. These preliminary findings suggest that diurnal cortisol patterns are not significantly different between adolescents with DS and TD and that cortisol levels are not associated with adiposity in this population. Despite these non-significant differences, youth with DS continue to be an 'at-risk' population for paediatric obesity in need of clinical intervention., (© 2019 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.)

Purpose: Obesity is highly prevalent among adolescents with Down syndrome (DS); however, reported associations between body composition and moderate-to-vigorous physical activity (MVPA) have been small and nonsignificant. The purpose of this study was to compare group differences between adolescents with and without DS, including dual-energy x-ray absorptiometry (DXA) measured body composition and accelerometer-measured physical activity, and then examine associations within adolescents with DS., Methods: Thirty-nine adolescents (22 with DS and 17 typically developing controls) 12-18 yr of age participated in the study. Groups had similar distributions of age, sex, and Tanner pubertal stage. Body composition was assessed by DXA, body mass index (BMI), and BMI percentile. MVPA was measured with ActiGraph GT3X+ accelerometers., Results: Adolescents with DS had significantly higher BMI, BMI percentile, and DXA-derived percent body fat (%BF) as well as lower MVPA compared with controls (P < 0.05). Associations between MVPA and %BF in adolescents with DS were moderate (r = -0.39, P = 0.07) but substantially stronger than BMI (r = -0.19, P = 0.40). However, linear regression analyses identified Tanner stage (β = -0.77, P < 0.001) and MVPA (β = -0.34, P = 0.047) as significant predictors of %BF. No relevant associations between body composition and MVPA were observed in adolescents with typical development (P > 0.05)., Conclusions: Our findings suggest that MVPA is associated with adiposity when measured with DXA among adolescents with DS.

The muscular dystrophies (MD) are a heterogeneous group of inherited disorders characterized by findings on muscle biopsy. In general, they feature progressive muscle wasting and weakness. In addition to the musculoskeletal system, direct and indirect effects can be seen in a variety of organ systems. These issues create challenges in patients with MD for ambulation and mobility, self-care, pain, fatigue, and community involvement. Because of its progressive nature and wide variety of pathophysiological mechanisms, patients with MD require individualized rehabilitation care. This chapter reviews specific rehabilitation needs and treatment of patients with MD., (Copyright © 2013 Elsevier B.V. All rights reserved.)

The purpose of this study was to examine the physical activity patterns of children with Down syndrome. A cross-sectional approach and accelerometry were used to measure the time children with Down syndrome (N = 104) spent in sedentary, light, and moderate-to-vigorous physical activity. Results indicated that adolescents from ages 14 to 15 years were the most sedentary and spent the least amount of time in light and moderate-to-vigorous physical activity. A general trend of decreasing physical activity as children increase in age was found. This trend is similar to that found among typically developing youth. Participants in this study were found to spend a majority of their day engaged in sedentary activities. Results indicate that most participants were not accumulating the recommended 60 minutes of moderate or vigorous physical activity.

Background: People with Down syndrome (DS) display consistent patterns of physical inactivity. If these sedentary behaviors continue over extended periods of time, there will be negative health consequences., Objective: The objective of this study was to investigate the physical activity and health-related outcomes of teaching children with DS to ride a 2-wheel bicycle., Design: This study was a randomized intervention in which the control group waited 1 year to receive the intervention., Setting: This intervention study was conducted in a community setting., Participants: The participants were children who were 8 to 15 years of age and who had been diagnosed with DS. Intervention The participants were randomly assigned to an experimental group (bicycle intervention) or a control group (no intervention)., Measurements: Measurements were obtained in the month before the intervention (preintervention), at 7 weeks after the intervention, and at 12 months after the preintervention measurement for all participants., Results: The results indicated no group differences at the preintervention session. Fifty-six percent of the participants in the experimental group successfully learned to ride a 2-wheel bicycle during the 5-day intervention. Analysis showed that participants who learned to ride spent significantly less time in sedentary activity at 12 months after the preintervention measurement and more time in moderate to vigorous physical activity than participants in the control group. Body fat appeared to be positively influenced over time in participants who learned to ride., Limitations: It is unknown how frequently the children in the experimental group rode their bicycles after the intervention., Conclusions: Most children who are 8 to 15 years of age and who have been diagnosed with DS can learn to ride a 2-wheel bicycle. Learning to ride can reduce time spent in sedentary activity and increase time spent in moderate to vigorous physical activity, which may influence the health and functioning of these children.

In this review, the current evidence is examined regarding neuropharmacologic treatment for children and adolescents (under the age of 18 years) who sustained a traumatic brain injury (TBI). Although the focus is on the pediatric TBI population, there is a paucity of empirical data related to the role of medication with children and adolescents after brain injury. Therefore, findings from the adult TBI literature are incorporated where appropriate so as to identify potential agents that warrant further examination in pediatric populations. This review addresses specific sequelae of TBI from the earliest stages of neurologic recovery to long-term comorbidities, including disorders of impaired consciousness, post-TBI agitation, cognitive decline, and post-TBI depression. The evidence regarding the role of medication in neuroprotection and neurorecovery in this population is also explored. Medication classes reviewed include excitatory amino acids, antagonists to the N-methyl-D-aspartate receptor, dopamine agonists, benzodiazepines, β-blockers, anticonvulsants, and antidepressants. It is hoped that this review will guide future research, and ideas as to how this may be accomplished within a pediatric population are suggested., (© 2010 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.)

Background/objective: To determine the effects of spasticity on anthropometrics, body composition (fat mass [FM] and fat-free mass [FFM]), and metabolic profile (energy expenditure, plasma glucose, insulin concentration, and lipid panel) in individuals with motor complete spinal cord injury (SCI)., Methods: Ten individuals with chronic motor complete SCI (age, 33 +/- 7 years; BMI, 24 +/- 4 kg/m2; level of injury, C6-T11; American Spinal Injury Association A and B) underwent waist and abdominal circumferences to measure trunk adiposity. After the first visit, the participants were admitted to the general clinical research center for body composition (FFM and FM) assessment using dual energy x-ray absorptiometry. After overnight fasting, resting metabolic rate (RMR) and metabolic profile (plasma glucose, insulin, and lipid profile) were measured. Spasticity of the hip, knee, and ankle flexors and extensors was measured at 6 time points over 24 hours using the Modified Ashworth Scale., Results: Knee extensor spasticity was negatively correlated to abdominal circumferences (r = -0.66, P = 0.038). After accounting for leg or total FFM, spasticity was negatively related to abdominal circumference (r = -0.67, P = 0.03). Knee extensor spasticity was associated with greater total %FFM (r = 0.64; P = 0.048), lower % FM (r = -0.66; P = 0.03), and lower FM to FFM ratio. Increased FFM (kg) was associated with higher RMR (r = 0.89; P = 0.0001). Finally, spasticity may indirectly influence glucose homeostasis and lipid profile by maintaining FFM (r = -0.5 to -0.8, P < 0.001)., Conclusion: Significant relationships were noted between spasticity and variables of body composition and metabolic profile in persons with chronic motor complete SCI, suggesting that spasticity may play a role in the defense against deterioration in these variables years after injury. The exact mechanism is yet to be determined.

Various guidelines have been proposed for returning to sport after concussion or mild TBI. However, no such guidelines exist for severe TBI. This study presents three cases of athletes who sustained severe TBIs and returned to competition. The rational for their clearance will be discussed.

Neuromuscular diseases are a broad group of disorders that affect the motor unit. Recent advances in genetics and molecular biology have greatly furthered understanding of these diseases. Unfortunately, this has not greatly modified treatment strategies. This article addresses some common features of these diseases, and some less commonly addressed issues.

Qualitative and quantitative measures of mitochondrial function were performed in rats selectively bred 15 generations for intrinsic aerobic high running capacity (HCR; n = 8) or low running capacity (LCR; n=8). As estimated from a speed-ramped treadmill exercise test to exhaustion (15 degrees slope; initial velocity of 10 m/min, increased 1 m/min every 2 min), HCR rats ran 10 times further (2,375+/-80 m) compared with LCR rats (238+/-12 m). Fiber bundles were obtained from the soleus and chemically permeabilized. Respiration was measured 1) in the absence of ADP, 2) in the presence of a submaximally stimulating concentration of ADP (0.1 mM ADP, with and without 20 mM creatine), and 3) in the presence of a maximally stimulating concentration of ADP (2 mM). Although non-ADP-stimulated and maximally ADP-stimulated rates of respiration were 13% higher in HCR compared with LCR, the difference was not statistically significant (P>0.05). Despite a similar rate of respiration in the presence of 0.1 mM ADP, HCR rats demonstrated a higher rate of respiration in the presence of 0.1 mM ADP+20 mM creatine (HCR 33% higher vs. LCR, P<0.05). Thus mitochondria from HCR rats exhibit enhanced mitochondrial sensitivity to creatine (i.e., the ability of creatine to decrease the Km for ADP). We propose that increased respiratory sensitivity to ADP in the presence of creatine can effectively increase muscle sensitivity to ADP during exercise (when creatine is increased) and may be, in part, a contributing factor for the increased running capacity in HCR rats.

An inverse relationship between initial level of physical capacity and the magnitude of response to training is termed the principle of initial value. We tested the operation of this principle under experimental conditions of minimal genetic and environmental variation. Inbred rat strains previously identified as genetic models of low [Copenhagen (COP)] and high [Dark Agouti (DA)] intrinsic (untrained) exercise capacity were trained for 8 wk on a treadmill using two disparate protocols: 1) a relative mode where each rat exercised daily according to its initial capacity, and 2) an absolute mode where both strains received the same amount of training independent of initial capacity. Response to exercise was the change in running capacity as estimated by meters run to exhaustion before and after training. When trained with the relative mode, COP rats gained 88 m (+21%; NS) whereas DA rats increased distance run by 228 m (+36%; P < 0.001). When each strain trained with the same absolute amount of training, the COP strain showed essentially no change (-6 m, -2%) and the DA strain gained 325 m (+49%; P < 0.009). Differences in response to exercise between the COP and DA could not be explained by body mass differences, oxidative enzyme activity (citrate synthase or ATP), or spontaneous behavioral activity. Our data demonstrate that genetic factors causative of high response to exercise are not uniquely associated with genetic factors for low intrinsic capacity and thus are not in accord with the principle of initial value.