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Pathological fractures in patients with cerebral palsy.

date: 10/01/1996
author: Brunner R, Doderlein L.
publication: J Pediatr Orthop B. 1996 Fall;5(4):232-8. Comment in: J Pediatr Orthop B. 1996 Fall;5(4):223-4.
pubmed_ID: 8897254

A retrospective study was made of 37 patients with 54 fractures that occurred without significant trauma. The morbidity and causes of these pathological fractures in patients with cerebral palsy were analyzed. The major causes for the fractures were long and fragile lever arms and stiffness in major joints, particularly the hips and knees. An additional factor was severe osteoporosis following a long period of postoperative immobilization. Seventy-four percent of the fractures occurred in the femoral shaft and supracondylar region. Stress fractures were rare (7%) and involved only the patella. Conservative treatment was sufficient in most cases but surgical fixation provided a good alternative for fractures of the femoral shaft. Intraarticular fractures with joint incongruity resulted in a decreased level of activity of the patient. Since osteoporosis is a major risk factor, patients with cerebral palsy should bear weight to prevent pathological fractures. Any stiffness of major joints and extended periods of immobilization should be avoided.

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Bone density and metabolism in children and adolescents with moderate to severe cerebral palsy.

date: 07/01/2002
author: Henderson RC, Lark RK, Gurka MJ, Worley G, Fung EB, Conaway M, Stallings VA, Stevenson RD.
publication: Pediatrics. 2002 Jul;110(1 Pt 1):e5.
pubmed_ID: 12093986

OBJECTIVES: Diminished bone density and a propensity to fracture with minimal trauma are common in children and adolescents with moderate to severe cerebral palsy (CP). The purpose of this study was to provide a detailed evaluation of bone mineral density (BMD) and metabolism in this population and to assess the relationship of these measures to multiple other clinical, growth, and nutrition variables. METHODS: The study group consisted of 117 subjects ages 2 to 19 years (mean: 9.7 years) with moderate to severe CP as defined by the Gross Motor Functional Classification scale. Population-based sampling was used to recruit 62 of the participants, which allows for estimations of prevalence. The remaining 55 subjects were a convenience sampling from both hospital- and school-based sources. The evaluation included measures of BMD, a detailed anthropometric assessment of growth and nutritional status, medical and surgical history, the Child Health Status Questionnaire, and multiple serum analyses. BMD was measured in the distal femur, a site specifically developed for use in this contracted population, and the lumbar spine. BMD measures were converted to age and gender normalized z scores based on our own previously published control series (n > 250). RESULTS: Osteopenia (BMD z score <-2.0) was found in the femur of 77% of the population-based cohort and in 97% of all study participants who were unable to stand and were older than 9 years. BMD was not as low in the lumbar spine (population-based cohort mean +/- standard error z score: -1.8 +/- 0.1) as in the distal femur (mean z score: -3.1 +/- 0.2). Fractures had occurred in 26% of the children who were older than 10 years. Multiple clinical and nutritional variables correlated with BMD z scores, but interpretation of these findings is complicated by covariance among variables. In stepwise regression analyses, it was found that severity of neurologic impairment as graded by Gross Motor Functional Classification level, increasing difficulty feeding the child, use of anticonvulsants, and lower triceps skinfold z scores (in decreasing order of importance) all independently contribute to lower BMD z scores in the femur. CONCLUSIONS: Low BMD is prevalent in children with moderate to severe CP and is associated with significant fracture risk. The underlying pathophysiology is complex, with multiple factors contributing to the problem and significant variation between different regions of the skeleton.

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Cardiopulmonary response in spinal cord injury patients: effect of pneumatic compressive devices.

date: 03/01/1983
author: Huang CT, Kuhlemeier KV, Ratanaubol U, McEachran AB, DeVivo MJ, Fine PR.
publication: Arch Phys Med Rehabil. 1983 Mar;64(3):101-6.
pubmed_ID: 6830418

The purpose of this study was to determine the effects of an inflatable abdominal corset and bilateral pneumatic leg splints on certain physiologic parameters during and after postural change in 27 quadriplegic patients. Data reflecting respiratory rate, tidal volume, heart rate, systolic and diastolic blood pressure were collected and analyzed. Measurements were acquired with patients in supine, 20 degrees head-up, 45 degrees head-up, and 20 degrees head-down positions. The study population was divided into 2 groups of cervical spinal cord injured patients: group I included 13 patients with C6 or C7 lesions; group II included 14 patients with C4 or C5 lesions. The mean time between injury and data collection was 47 days. Several trends were identified: (1) the neurologic level of lesion in quadriplegics appears relatively unimportant in determining cardiopulmonary response to postural change; (2) the use of assistive compressive devices does not improve pulmonary ventilatory parameters during postural change, although such devices do help maintain cardiovascular parameters; and (3) the abdominal corset appears more effective than pneumatic leg splints in maintaining blood pressure at pretilt levels. A tidal volume of 350ml to 400ml is most easily maintained by placing patients in a supine position and eschewing assistive compressive devices. Because the pneumatic devices proved successful in helping quadriplegic patients maintain cardiovascular stability during postural changes, therapeutic modalities, such as tilt table treatments, may be initiated at an earlier stage in the rehabilitation process.

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Femoral loads during passive, active, and active-resistive stance after spinal cord injury: a mathematical model.

date: 03/19/2004
author: Frey Law LA, Shields RK.
publication: Clin Biomech (Bristol, Avon). 2004 Mar;19(3):313-21.
pubmed_ID: 15003348

OBJECTIVE: The purpose of this study was to estimate the loading environment for the distal femur during a novel standing exercise paradigm for people with spinal cord injury. DESIGN: A mathematical model based on experimentally derived parameters. BACKGROUND: Musculoskeletal deterioration is common after spinal cord injury, often resulting in osteoporotic bone and increased risk of lower extremity fracture. Potential mechanical treatments have yet to be shown to be efficacious; however, no previous attempts have been made to quantify the lower extremity loading during passive, active, and active-resistive stance. METHODS: A static, 2-D model was developed to estimate the external forces; the activated quadriceps forces; and the overall bone compression and shear forces in the distal femur during passive (total support of frame), active (quadriceps activated minimally), and active-resistive (quadriceps activated against a resistance) stance. RESULTS: Passive, active, and active-resistive stance resulted in maximal distal femur compression estimates of approximately 45%, approximately 75%, and approximately 240% of body weight, respectively. Quadriceps force estimates peaked at 190% of body weight with active-resistive stance. The distal femur shear force estimates never exceeded 24% of body weight with any form of stance. CONCLUSIONS: These results support our hypothesis that active-resistive stance induces the highest lower extremity loads of the three stance paradigms, while keeping shear to a minimum. RELEVANCE: This model allows clinicians to better understand the lower extremity forces resulting from passive, active, and active-resistive stance in individuals with spinal cord injury.

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Standing and its importance in spinal cord injury management.

date: 01/01/1987
author: Axelson P, Gurski D, Lasko-Harvill A.
publication: RESNA 10th Annual Conference San Jose, California 1987
A broad spectrum of physiological problems are associated with lack of gravitational stress in the individual with spinal cord injury. Prolonged immobilization results in systemic de-adaptations which include cardiovascular changes, the alteration of calcium homeostasis which leads to bone de-mineralization and risk of urinary calculi.

Weight bearing in the standing posture has been shown to ameliorate many of these problems and offers physiological advantages for the individual with spinal card injury.

There are also significant psychological and social benefits to standing, including improved self-image, and eye-to-eye interpersonal contact. Increased vocational, recreational and daily living independence are additional benefits of standing.

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Bone loss and muscle atrophy in spinal cord injury: epidemiology, fracture prediction, and rehabilitation strategies.

date: 01/01/2006
author: Giangregorio L, McCartney N.
publication: J Spinal Cord Med. 2006;29(5):489-500.
pubmed_ID: 17274487

Individuals with spinal cord injury (SCI) often experience bone loss and muscle atrophy. Muscle atrophy can result in reduced metabolic rate and increase the risk of metabolic disorders. Sublesional osteoporosis predisposes individuals with SCI to an increased risk of low-trauma fracture. Fractures in people with SCI have been reported during transfers from bed to chair, and while being turned in bed. The bone loss and muscle atrophy that occur after SCI are substantial and may be influenced by factors such as completeness of injury or time post injury. A number of interventions, including standing, electrically stimulated cycling or resistance training, and walking exercises have been explored with the aim of reducing bone loss and/or increasing bone mass and muscle mass in individuals with SCI. Exercise with electrical stimulation appears to increase muscle mass and/or prevent atrophy, but studies investigating its effect on bone are conflicting. Several methodological limitations in exercise studies with individuals with SCI to date limit our ability to confirm the utility of exercise for improving skeletal status. The impact of standing or walking exercises on muscle and bone has not been well established. Future research should carefully consider the study design, skeletal measurement sites, and the measurement techniques used in order to facilitate sound conclusions.

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Moving the arms to activate the legs.

date: 07/01/2006
author: Ferris DP, Huang HJ, Kao PC.
publication: Exerc Sport Sci Rev. 2006 Jul;34(3):113-20.
pubmed_ID: 16829738

Recent studies on neurologically intact individuals and individuals with spinal cord injury indicate that rhythmic upper limb muscle activation has an excitatory effect on lower limb muscle activation during locomotor-like tasks. This finding suggests that gait rehabilitation therapy after neurological injury should incorporate simultaneous upper limb and lower limb rhythmic exercise to take advantage of neural coupling.

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Skeletal adaptations to alterations in weight-bearing activity: a comparison of models of disuse osteoporosis.

date: 01/01/2001
author: Giangregorio L, Blimkie CJ.
publication: Sports Med. 2002;32(7):459-76.
pubmed_ID: 12015807

The removal of regular weight-bearing activity generates a skeletal adaptive response in both humans and animals, resulting in a loss of bone mineral. Human models of disuse osteoporosis, namely bed rest, spinal cord injury and exposure to micro-gravity demonstrate the negative calcium balance, alterations in biochemical markers of bone turnover and resultant loss of bone mineral in the lower limbs that occurs with reduced weight-bearing loading. The site-specific nature of the bone response is consistent in all models of disuse; however, the magnitude of the skeletal adaptive response may differ across models. It is important to understand the various manifestations of disuse osteoporosis, particularly when extrapolating knowledge gained from research using one model and applying it to another. In rats, hindlimb unloading and exposure to micro-gravity also result in a significant bone response. Bone mineral is lost, and changes in calcium metabolism and biochemical markers of bone turnover similar to humans are noted. Restoration of bone mineral that has been lost because of a period of reduced weight bearing may be restored upon return to normal activity; however, the recovery may not be complete and/or may take longer than the time course of the original bone loss. Fluid shear stress and altered cytokine activity may be mechanistic features of disuse osteoporosis. Current literature for the most common human and animal models of disuse osteoporosis has been reviewed, and the bone responses across models compared.

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Neural coupling between upper and lower limbs during recumbent stepping.

date: 10/01/2004
author: Huang HJ, Ferris DP.
publication: J Appl Physiol. 2004 Oct;97(4):1299-308. Epub 2004 Jun 4.
pubmed_ID: 15180979
During gait rehabilitation, therapists or robotic devices often supply physical assistance to a patient’s lower limbs to aid stepping. The expensive equipment and intensive manual labor required for these therapies limit their availability to patients. One alternative solution is to design devices where patients could use their upper limbs to provide physical assistance to their lower limbs (i.e., self-assistance). To explore potential neural effects of coupling upper and lower limbs, we investigated neuromuscular recruitment during self-driven and externally driven lower limb motion. Healthy subjects exercised on a recumbent stepper using different combinations of upper and lower limb exertions. The recumbent stepper mechanically coupled the upper and lower limbs, allowing users to drive the stepping motion with upper and/or lower limbs. We instructed subjects to step with 1) active upper and lower limbs at an easy resistance level (active arms and legs); 2) active upper limbs and relaxed lower limbs at easy, medium, and hard resistance levels (self-driven); and 3) relaxed upper and lower limbs while another person drove the stepping motion (externally driven). We recorded surface electromyography (EMG) from six lower limb muscles. Self-driven EMG amplitudes were always higher than externally driven EMG amplitudes (P < 0.05). As resistance and upper limb exertion increased, self-driven EMG amplitudes also increased. EMG bursts during self-driven and active arms and legs stepping occurred at similar times. These results indicate that active upper limb movement increases neuromuscular activation of the lower limbs during cyclic stepping motions. Neurologically impaired humans that actively engage their upper limbs during gait rehabilitation may increase neuromuscular activation and enhance activity-dependent plasticity.

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Can Using Standers Increase Bone Density In Non-Ambulatory Children?

date: 10/01/2006
author: Katz, Danielle,MD, Snyder, Bryan MD, PhD, Dodek, Anton MD, Holm, Ingrid MD Miller, Claire BS
publication: Abstract as published in the American Academy of Cerebral Palsy and Developmental Medicine (AACPDM) 2006 Conference Proceedings

Purpose: Pathologic fractures are a significant source of morbidity for non-ambulatory children with neuromuscular dysfunction. We hypothesize that increasing weight-bearing in non-ambulatory children will increase bone material density (BMD) and decrease fracture risk. The aim of this pilot study was to demonstrate that non-ambulatory children participating in a standing program for at least two hours a day will experience an increase in BMD in the weight bearing bones. We also evaluate the reliability of measuring BMD at the calcaneous (weight bearing bones) and distal forearm (non-weight bearing bone) using peripheral DXA in delayed, non-ambulatory children.

Methods: After receiving IRB approval, 12 non-ambulatory, quadriplegic children (ages 12-21) consented to participate in a 2 hour/day, 5 day/week standing program. A history, orthopaedic exam, determination of bone age, laboratory tests for metabolic bone disease and BMD at the calcaneal tuberosity and distal forearm metaphyses were obtained. Compliance with the prescribed standing program was monitored for 6 months. BMD was measured using peripheral DXA at baseline and every 3 months. Using Jan. 2003 BMD data as a baseline, the ratio of change in BMD at the calcaneous and distal forearm was evaluated as a function of percent compliance with standing program.

Results: Intrarater reliability for BMD measured by peripheral DXA was good: Pearson correlation for the calcaneous = 0.90 (p=0.01) and for the forearm = 0.96 (p=0.01). Paired t test between two sets of data measured at each site on the same day were not different for calcaneous (t=0.92, df=15, p=0.37) or forearm (t=0.05, df=15, p=0.96). Compliance with the standing program was inconsistent. No patients were 100% compliant. Patients tended to stand longer at the initiation of the study Jan.-April (Jan vs Apr, p = 0.018; Jan vs Jul, p = 0.89; Apr vs Jul, p = 0.063). Compliance (%) was positively correlated (r = -0.62) with increased calcaneous BMD measured in April. This is in contrast to forearm BMD measured at the same time; which was negatively correlated (r = -0.44) with standing compliance. This support the notion that standing preferentially increases bone mass in the weight-bearing bones. However the BMD at the calcaneous measured in July was decreased, perhaps reflecting the decreased compliance the with standing program over the succeeding interval April-July.

Conclusion: It is feasible to have non-ambulatory children participate in a rigorous standing program. The weight bearing ?dose? affects BMD at the calcaneous but the benefit appears to be transient if the intensive standing program is not sustained.

Significance: The intensive use of standers (10 hours/wk) may have a beneficial effect on BMD of weight bearing bones in non-ambulatory children.