Continuum Lifelong Learning Neurol 2011;17(4):

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1 Review Article Address correspondence to Dr Cristina L. Sadowsky, Kennedy Krieger Institute, 707 North Broadway, Suite 518, Baltimore, MD 21205, Relationship Disclosure: Drs Sadowsky and Bosques report no disclosures. Dr Becker has received compensation for expert witness testimony and plans to continue to be available for litigation consultation. Dr Becker has also received a grant from the Bryon Riesch Paralysis Foundation for a clinical trial. Ms Dean has received an honorarium for a speaking engagement at the Rare Neuroimmunologic Disorders CME Conference. Dr McDonald may receive a potential royalty related to a patent licensed through Washington University School of Medicine from Restorative Therapies, Inc. Dr Recio has received grants from the US Department of Defense. Dr Frohman has received personal compensation for speaking engagements or consulting activities from Abbott Laboratories, Acorda Therapeutics, Bayer, Biogen Idec, Novartis, and Teva Neuroscience. Unlabeled Use of Products/Investigational Use Disclosure: Dr Sadowsky discusses the use of activity-based restorative therapy and functional electrical stimulation for neural restoration. Drs Becker, Bosques, McDonald, Recio, and Frohman and Ms Dean report no disclosures. Copyright*2011, American Academy of Neurology. All rights reserved. Rehabilitation in Transverse Myelitis Cristina L. Sadowsky, MD; Daniel Becker, MD; Glendaliz Bosques, MD, FAAPMR; Janet M. Dean, MS, RN, CPNP, CRRN; John W. McDonald III, MD, PhD; Albert Recio, MD, RPT, PTRP; Elliot M. Frohman, MD, PhD, FAAN ABSTRACT The consequences of neurologic injuries related to transverse myelitis (TM) are longlasting and require rehabilitative interventions in about two-thirds of cases. Because numerous neural repair mechanisms are dependent on maintenance of an optimal amount of activity both above and below the injury level, rehabilitation and exercise are useful not only for compensatory functional purposes but also as tools in neural system restoration. The application of established neurophysiologic principles to post-tm rehabilitation has substantial impact on optimizing residual functional capabilities while facilitating the processes of central plasticity and reorganization of sensory and motor programming. The process of neurorehabilitation thereby serves both to treat the patient with TM and to help physicians interrogate and dissect the mechanisms involved in spinal cord injury, neuroprotection, and, ultimately, recovery. Post-TM rehabilitation is lifelong and should be integrated into daily living in a home setting as part of the global management of paralysis, a chronic condition with significant comorbidities. Continuum Lifelong Learning Neurol 2011;17(4): INTRODUCTION In medicine, rehabilitation is defined as the process of assisting someone to improve or recover lost function after an event, illness, or injury that has caused functional limitations. The goal of rehabilitation is usually to restore the lost function or return it to a level as close as possible to the one exhibited prior to the injury. Thus, the definition of rehabilitation is closely related to function. Function is described according to the model in which it is studied. Impairment is any loss or abnormality of psychological, physiologic, or anatomical structure or function. Disability is a restriction or lack of ability to perform an activity in the manner or within the range considered normal for a human being as a result of an impairment. A handicap is described as a disadvantage for a given individual (resulting from an impairment or disability) that limits or prevents the fulfillment of a role that is normal for that individual, depending on age, sex, and social and cultural factors. 1 With such a broad spectrum of application, rehabilitation of a neurologic injury and its consequences should focus on restoration of both physiologic (impairment) and day-to-day (disability) function. While ability-restoring rehabilitation (or habilitation when the process addresses function potentially lost from a disease that strikes a child during development, before he or she had a chance to acquire such function) has long been recognized by the current medical model, impairmentreversing (re)habilitation is not a concept widely accepted yet. There are 816 August 2011

2 several reasons for this, including the inability to perfectly repair damage and the lack of correlation between anatomical and physiologic integrity. Additionally, the damaged substrate may be unknown or not visible using current medical assessment tools, making it unable to be repaired. In transverse myelitis (TM), all of the above factors play a role, but rehabilitation approaches can be expanded to include concepts of impairment and ability repair and restoration. TM is defined as a monofocal inflammatory process of the spinal cord. It can exist as part of a multifocal CNS disease (eg, multiple sclerosis, neuromyelitis optica), a CNS infection (eg,syphilis,lymedisease,hiv,human T-cell lymphotropic virus type I, Mycoplasma, herpesviruses), a multisystem disease (eg, systemic lupus erythematosus, sarcoidosis, Sjögren syndrome, Beh0et disease), or as an isolated idiopathic entity. Because of its broad differential diagnosis, etiology, and prognosis, rehabilitation must be tailored to the specific setting in which TM occurred. Age at onset should also be taken into account. This article focuses on idiopathic TM. Long-term follow-up data on patients with TM reveal that approximately onethird of patients recover with little to no sequelae, one-third are left with a moderate degree of permanent disability (eg, mild spasticity but independent ambulation, urgency and/or constipation, some sensory deficits), and one-third have severe disabilities (eg, inability to walk or severe gait disturbance, absence of sphincter control, sensory deficits). 2,3 It is extremely difficult to predict at onset into which category a patient will fall. Certain surrogate markers have been identified to guide this prediction. For example, acute illness requiring ventilator assistance is re lated to later impairment in Continuum Lifelong Learning Neurol 2011;17(4): ambulation. A greater number of white blood cells in CSF predicts worse functional mobility, as does age younger than 3 at onset of disease. 4 AhighCSF interleukin (IL)-6 level in TM predicts acute and long-term disability. 5 Virtually all patients with acute TM require acute rehabilitation. Based on the data referenced above, it can be concluded that approximately 66% of all patients with TM will also require long-term rehabilitation. TRADITIONAL REHABILITATION IN TRANSVERSE MYELITIS Functional goals for traditional TM rehabilitation (the rehabilitation concerned with restoring abilities) include maximizing physical independence, capabilities, and potential. An interdisciplinary team approach is used, and the team leadership varies according to the goal or task (ie, if the goal is gait training, the physical therapist is the leader, with the physician supporting for medical management of tone and spasticity as needed). The rehabilitation team traditionally includes physicians, therapists, nurses, case managers, and family members and may also include home health aides, psychologists, respiratory therapists, speech-language pathologists, and orthotists, depending on specific patient needs and rehabilitation goals. Physical and occupational therapists train patients with TM to increase their strength and joint range of motion (ROM), improve tone, mitigate pain, and maximize functional mobility. Equipment evaluation should be a regular and systematic feature of neurorehabilitation to determine whether any equipment changes or modifications will augment and optimize functional performance. A thorough home rehabilitation program is developed to facilitate the patient s mobility gains in the home setting and complement the KEY POINTS h Rehabilitation of a neurologic injury and its consequences should focus on restoration of both physiologic (impairment) and day-to-day (disability) function. h Approximately one-third of patients with transverse myelitis recover with little to no sequelae, one-third are left with a moderate degree of permanent disability, and one-third have severe disabilities. h Functional goals for traditional transverse myelitis rehabilitation include maximizing physical independence, capabilities, and potential. This type of rehabilitation uses an interdisciplinary team approach. 817

3 Rehabilitation KEY POINTS h Some muscle tightness may be desirable in specific locations, such as tightness of the finger flexors to produce strong tenodesis grasp in an individual with C6 tetraplegia or tightness of back extensors to assist triceps paralysis to regain upright sitting. h A strength-training regimen may include static or dynamic training performed through isometric, isotonic, and isokinetic techniques, according to the targeted goal and the degree of neurologic deficit and adapted specifically for each individual with paralysis. training done at the therapy site. The patient and caregiver are educated on the home program and expectations upon hospital discharge. Achieving adequate joint ROM is necessary and is facilitated through stretching exercises, use of appropriate orthoses, and strengthening exercises. The most common limitations in movement due to contractures in traumatic and nontraumatic spinal cord disease (SCD) involve shoulder external rotation, shoulder elevation, scapular depression, scapular retraction, elbow extension and supination, hip extension, ankle dorsiflexion, and great toe flexion. Some muscle tightness may be desirable in specific locations, such as tightness of the finger flexors to produce strong tenodesis grasp in an individual with C6 tetraplegia, thereby resulting in good wrist extension function, or tightness of back extensors to assist triceps paralysis and to regain upright sitting. A limited amount of overstretching can likewise help in specific areas, such as the increased hip external rotation required to put on socks and shoes. Aggressive ROM exercises are contraindicated or should be done with caution in the presence of unstable fractures, active heterotopic ossification, deep vein thrombosis, and osteoporosis. Daily stretching of muscles with terminal sustained stretch is considered first-line rehabilitative treatment for limitation of motion due to spasticity. To target muscle strengthening and facilitation exercises in individuals with SCD, all spared or intact muscles are given progressive resistive exercises, active-assistive ROM, or active ROM through the maximum range. Adaptive muscle changes seen in SCD with exercise training include increased cross-sectional area, predominantly of type II fibers; increased metabolic capacity due to increased concentrations of adenosine 5 -triphosphate and creatine phosphate; and increased levels of myofibrillar proteins. A strength-training regimen may include static or dynamic training performed through isometric, isotonic, and isokinetic techniques, according to targeted goals and the degree of neurologic deficit. Training programs using the DeLorme or Oxford 6 methods are adapted specifically for individuals with paralysis. Training activities done on an adjustable therapy mat are often composed of sequenced activities that progress from the easiest to the most difficult. The usual progression is from bed mobility to rolling, prone lying, long sitting, short sitting, and sitting up from a lying position. Muscles needed for individuals with lower limb paralysis to be able to move or position their legs in bed are wrist extensors, biceps, anterior deltoid, middle deltoid, and shoulder girdle stabilizers. Individuals with tetraplegia are taught to use their arms, head, and neck for momentum to roll in bed, keeping the elbow straight while the shoulder is flexing across the body. Sitting up from a lying position is a prerequisite for independent dressing and transfers. A thorough evaluation should be conducted to determine the most appropriate transfer technique for any individual with paralysis. Dependent transfers include sliding transfers, the dependent standing pivot technique, or the use of Hoyer lifts. Transfers that require some active patient participation include the two-man lift, the sliding board transfer, or the assisted standing pivot transfer. The goal of an assisted transfer is to gradually reduce the assistance required until the patient can perform the transfer independently. Floor-to-chair transfer training is very important for anyone who falls out of a wheelchair or otherwise ends up on the floor and needs to get back into a chair August 2011

4 Ambulation is a commonly expressed goal of most people with TM. Patients with injuries at T2 and above typically cannot achieve ambulation, whereas patients with T3 to T11 affectation are able to use braces for physiologic standing and therapeutic ambulation. The goal for individuals with a T12 to L2 injury is to achieve ambulation in the household, while patients with injuries at L3 and lower are most likely to achieve ambulation in the community (distance of more than 150 feet at a time). It should be noted, however, that patients with T12 to L2 injuries can also achieve community ambulation if they are young and highly motivated. The attainment of muscle function in the back and lower extremities helps physical therapists predict the bracing needs to aid in ambulation. For example, patients with pelvic control and intact quadratus lumborum and abdominal muscles may walk using long leg braces (knee-ankle-foot orthoses [KAFOs]) and crutches. Patients with some control of muscles crossing the hip joint, such as iliopsoas, hip adductors, sartorius, and gluteus maximus, should be able to walk a limited amount with long leg braces (KAFOs) and crutches. Patients with intact quadriceps can walk full-time with short leg braces (ankle-foot orthoses [AFOs]). Patients with intact tibialis anterior and posterior can walk full-time with short leg braces (AFOs, supramalleolar orthoses) or no braces at all. During the preygait training program, important muscles to strengthen are core trunk and abdominal muscles, shoulder depressors and scapula stabilization muscles, and triceps and wrist extensors. In this phase, a structured progressive resistive exercise program for the latissimus dorsi, pectoralis, lower trapezius, and serratus anterior muscles is very important to achieve good trunk Continuum Lifelong Learning Neurol 2011;17(4): and hip stability (while the shoulder is adducted, the latissimus dorsi can act as a hip extensor, given its fixed insertion and reverse origin, locking the hip in extension against its own ligaments). During gait training, control of the pelvis is a critical factor in successful ambulation. Gait training includes practicing standing activities between parallel bars and practicing the gait pattern inside and outside the bars with assistive devices (ie, canes, bilateral forearm or axillary crutches, walkers), over obstacles, on rough terrain or uneven ground, on ramps, on curbs, going through doorways, and when getting into a car. Training on falling techniques includes learning how to fall safely and how to get up from the floor. Finally, prescribing the appropriate wheelchair for the level of the deficit, the individual s needs and comfort, and any medicalorthopedic comorbidities is essential. ACTIVITY-BASED RESTORATIVE THERAPIES IN TRANSVERSE MYELITIS Activity-based restorative therapy (also known as activity-based therapy or activity-based rehabilitation) addresses the impairment and is based on activity-dependent neural plasticity, in which changes in the nervous and muscular system are driven by repetitive activation of the neuromuscular system above and below the injury level. The tools of activity-based restorative therapy are the same as those used in traditional rehabilitation, but the premise of application is based on the nervous system s dependence on activity for everything from myelination and remyelination to new cell birth and synapse formation and, ultimately, to function. Thus, motor activation (patterned and nonpatterned) and sensory stimulation as part of the rehabilitation process are done with the ultimate goal of KEY POINTS h Individuals with an injury at the T12 to L2 level can master household ambulation, while individuals with L3 and lower injuries can achieve community ambulation. h During a preygait training program, important muscles to strengthen are core trunk and abdominal muscles, shoulder depressors and scapula stabilization muscles, and triceps and wrist extensors. h During gait training, control of the pelvis is a critical factor in successful ambulation. 819

5 Rehabilitation KEY POINTS h Activity-based restorative therapy addresses the impairment and is based on activity-dependent neural plasticity, in which changes in the nervous and muscular system are driven by repetitive activation of the neuromuscular system above and below the injury level. By using activity-based restorative therapyaspartofthe rehabilitation process, the ultimate goal is neural restoration, not just function restoration. h Activity-based restorative therapy tools are used to produce motor activation (patterned and nonpatterned) and sensory activation. h Activity plays a critical role in development and neural plasticity, including influencing gene expression and modifying synaptic strength, synapse elimination, myelination, remyelination, and axonal growth. neural restoration, not just function restoration. Motor Activation Patterned motor activation can be done through both task-specific and nonspecific training. Task-specific training is easy to understand and has been practiced since the beginning of rehabilitation. Locomotor training (ie, gait training) is the best example of patterned, task-specific training. It can be practiced in a partially weight-supported environment using treadmill systems, automatic gait robots, or water. It can also be practiced in the traditional overground way. Two important principles of locomotor training when dealing with neurologic injuries need to be mentioned. First, maximization of load bearing in lower extremities is essential, as the increase in load bearing is associated with an increase in limb EMG activity, and shared load bearing between upper and lower extremities decreases EMG activity in lower limbs. 7 Second, optimizing sensory cues, ensuring normal walking speed, and optimizing kinematics (stance/swing, upper extremities patterned swing) play an essential role in maximizing functional recovery and minimizing compensation. Patterned nonspecific training is based on the principle of neural activation of the central pattern generator (CPG). Circuitry for the CPG is located in the lumbar region of the spinal cord (L2 to L5). 8 Limited input can activate the CPG and produce interlimb CPG activation. 8,9 Nonspecific patterned training is accomplished most easily by using functional electrical stimulation (FES). FES is postulated to promote peripheral and central nervous system repair following injury. Following complete spinal cord transection in rats, lower extremity FES induced an 82% to 86% increase in cell birth in the lumbar spinal cord. In this model, FES doubled the proportion of the newly born cells expressing markers suggestive of tripotential progenitors. These data suggest that controlled electrical activation of the CNS may enhance spontaneous regeneration after neurologic injuries. 10 In another study, electrical stimulation applied to the cortical pyramids in rats enhanced synapse formation in the spinal cord during development and following corticospinal tract injury. 11 Elegant studies have clearly demonstrated that activation of the CNS is an important variable influencing the cellular mechanisms associated with regeneration. In fact, these studies indicate that cellular regeneration is dependent on activity. The role of activity may be even more important in conditions in which normal activity is reduced, as in TM. Examples of activity playing a critical role in development and plasticity include activity-dependent gene expression, modification of synaptic strength, synapse elimination, myelination and maintenance of myelination, and axonal growth. The widespread dependence of development and plasticity in the CNS on neural activity suggests that optimized neural activity might also be important for regeneration, given the common cellular mechanisms participating in both processes. 12 This concept is further supported by evidence that increased neural activity enhances multiple components of spontaneous regeneration, while decreased activity inhibits it. 13 Activity itself has been associated with neurotrophin production; thus, it is conceivable that the elaboration of neurotrophins after FES activation below the injury level underlies the biological basis for neural reorganization and functional improvement. 14 Proven functional electrical stimulation benefits. Numerous publications 820 August 2011

6 have demonstrated the practical benefits of FES in individuals with sequelae of traumatic spinal cord injury (SCI). Some of those benefits include increased muscle mass, improved bone density, enhanced cardiovascular function, improved body composition (ratio of muscle to fat), improved bowel function, decreased spasticity, improved glucose metabolism, and reductions in bladder infection rate. In addition, electrical stimulation can be used in combination with other therapies to enhance functional recovery from SCI. One example is the use of FES to facilitate gait. 15 In a study of 70 patients, Solomonow and colleagues demonstrated that 14 weeks of FES walking (3 h/wk) improved total cholesterol, low-density lipoprotein levels, and hydroxyproline/creatinine ratios and reduced spasticity in patients with paraplegia from SCI. 16,17 In another study, after 10 weeks of FES cycling (2 to 3 sessions/wk), 18 subjects with SCI showed increased lean muscle mass, improved American Spinal Injury Association motor and sensory scores, and reductions in serum levels of IL-6, tumor necrosis factor ", and C-reactive protein (markers for inflammation). Limited clinical data are available on the use of FES in demyelinating disorders such as TM and multiple sclerosis (MS). A pilot trial of 12 patients with MS who underwent FES cycling (3 sessions/wk for 2 weeks) demonstrated improved spasticity but failed to show improvement in strength and walking speed. 18 Longterm FES bracing (3 to 12 months) for footdrop has been shown to increase strength and walking speed, suggesting that it strengthens activation of motor cortical areas and their residual descending connections in patients with MS. 19 A randomized trial of 44 subjects with MS and footdrop who received FES bracing or exercise for Continuum Lifelong Learning Neurol 2011;17(4): weeks demonstrated that exercise may provide a greater training effect on walking speed and endurance than FES, although the FES group performed to a significantly higher level with FES than without for the same outcome measures. Therefore, the authors recommended studying the combined therapeutic effects of FES and exercise for this patient group. 20 In a recent pilot trial, patients with MS showed improvement on a broad array of functional and neurologic outcome measures including gait, upper extremity dexterity, and quality of life. Furthermore, analysis of CSF before FES and 3 months after initiating FES cycling revealed an enhanced neural repair program (increased CSF transforming growth factor $3) and a reduced inflammatory environment within the CNS (decreased interferon-,, IL-7, and IL-8). 21 Functional electrical stimulation characteristics in transverse myelitis. Successful implementation of FES to result in muscle contractions requires an intact lower motor neuron. Traditionally, the FES technology was used to generate purposeful contractions of paralyzed muscles. Newer FES technology, however, is based on applying low-level electrical currents that activate the ascending nerve fibers. Signals enter the spinal cord and are switched either directly or via interneurons to the lower motor neuron, which causes the muscle to contract. The use of FES is related not only to direct and visible muscle contraction but also to the effect of neural activation in the ascending sensory pathways and the effect of electrical current on the histology and biochemical characteristics of the muscle fibers. 22,23 In TM, the lower motor neuron is generally affected at the level of the inflammation. For that reason, muscle contractions in response KEY POINT h Functional electrical stimulation has shown proven benefits in individuals with sequelae of traumatic spinal cord injury, including increased muscle mass, improved bone density, enhanced cardiovascular function, improved body composition, improved bowel function, decreased spasticity, improved glucose metabolism, and reductions in bladder infection rate. 821

7 Rehabilitation KEY POINT h Functional electrical stimulation does not always produce visible activation of muscles in transverse myelitis because of lower motor neuron involvement. In those cases, use of different current characteristics (ie, longer pulse width, bidirectional/rectangular, or even direct current) can prevent muscle atrophy and its sequelae. to FES from levels at the injury site are usually limited or absent. This may be a transient phenomenon related to the acute inflammation. In most patients, some of the lower motor neuron function returns weeks to months following the injury. During that period, it is important to try to avoid muscle atrophy and its sequelae at that level. This may be attempted by using other electrical stimulation devices that utilize an unusually long duration (ie, 200 6s) and unusually shaped (bidirectional, rectangular) current impulse or by activating the muscle from a different spinal level in addition to using traditional rehabilitation approaches (eg, bracing, taping, splinting). Sensory Stimulation Sensory stimulation can be used to activate afferent pathways with the goal of providing information that can subsequentlybeusedtoperformamotortask with the direct effect being improved motor and sensory function. 24,25 An exampleofthisissensorystimulationthat enhances excitatory inflow from muscle spindles to the motor neuron pools and depresses the inhibitory effect of the Golgi tendon organ. Focal sensory activation or stimulation and nonfocal activation (eg, epidural stimulation or intrathecal delivery of neurotrophin-3 or brain-derived neurotrophic factor) can also produce complex lower limb cyclinglike movements. In a doubleblind, randomized controlled clinical trial using whole-body vibration in patients with MS, Schuhfried and colleagues demonstrated improved postural control and walking speed. 26 PEDIATRIC CONSIDERATIONS Approximately 20% of patients with acute transverse myelitis (ATM) are younger than 18 years of age. 27 Pidcock and colleagues 4 analyzed the largest cohort of pediatric patients with ATM ever published using standardized functional measures. According to the study, ATM may have a bimodal distribution of age, with approximately 40% of patients being younger than 3 years of age (compared to 10% reported in previous studies) and another peak in incidence occurring between the ages of 5 and 17 years. Most patients had the greatest impairment during the acute illness period and reached their functional nadir 2 days after the onset of symptoms. Eighty-nine percent of children had acute muscle weakness, were limited to bed or a wheelchair for mobility, or required ventilatory assistance during this stage. Of the patients who were nonambulatory, 52% were able to walk at least 30 feet with or without the help of a walker, while only 39% of the patients who initially needed ventilator assistance were able to do so at the time of follow-up. In the acute phase, 82% of the patients experienced bladder dysfunction requiring catheterization, and 50% of the patients still required catheterization at follow-up. Regarding functional outcome, most patients were independent in the skill areas measured by the Functional Independence Measure (FIM) and WeeFIM (FIM for children) systems: self-care, communication, social cognition, transfers, and locomotion. However, 33% required minimal to total assistance for ambulation or using the wheelchair for more than 160 feet. More than half (54%) of children required minimal to total assistance for sphincter control, while 36% required assistance for mobility with transfers and 27% required assistance for self-care needs. According to this study, even though most patients recover adequate muscle strength and motor control for ambulation, significant impairments remain within important functional domains such as transfers, self-care needs, and sphincter control August 2011

8 Continuum Lifelong Learning Neurol 2011;17(4): These limitations present an opportunity for rehabilitation management to prevent secondary complications and further improve function in the long term. In our clinical practice we have identified a subgroup of children who presented with CSF and multilevel MRI changes consistent with ATM. These children subsequently recovered most of the function that was acutely lost, except for one extremity, either upper or lower, that was left with persistent flaccid monoplegia (see Case 6-1). On physical examination, the affected limb has generally demonstrated intact sensation but severely decreased motor function, rapid and severe muscle atrophy, areflexia, and no muscle contraction with trials of electrical stimulation. Because of this unusual outcome, electrodiagnostic studies have been completed on all of these children. Results of these studies have been consistent with severe motor neuronopathy. We postulate that these children have had an immunologic or inflammatory reaction to either the anterior horn cells or their proximal nerve roots, resulting in a lower motor neuron pattern of paralysis. We anticipated that these children would demonstrate long-term sequelae similar to those of children affected with poliomyelitis or brachial plexus injury, including asymmetric limb growth, weakness, joint dislocation or contracture, scoliosis, and scapular winging or gait abnormalities. 28 Upper extremity fractures (multiple in several children), including buckle fractures in the wrists, have been observed in several children with plegic upper extremities due to rapid onset and progression of disuse or neurogenic osteoporosis. Indeed, children with affected upper extremities demonstrated shoulder subluxation, scapular winging, and mild scoliosis, and children with lower extremity involvement demonstrated laxity of the hip, hyperextension at the knee, poor or absent dorsiflexion, and plantar flexion at the ankle. Bracing and splinting have been prescribed to protect affected joints. During observation of some of these children for over 2 years, recovery of motor function has been poor despite aggressive outpatient and home rehabilitation programs including strengthening, weight-bearing activities, gait training or fine motor skill remediation, joint stabilization, and bracing. 29 All children were given trials of FES cycle ergometry (either upper or lower extremity). In general, muscle contraction was not elicited, and, as sensation was intact, the children s tolerance for FES was poor. Because of poor spontaneous recovery of neurologic function, these children have been evaluated for nerve, muscle, or tendon transfers in the hope of improving function or at least slowing the severe muscle and bone wasting. Three of our patients in this subgroup have undergone nerve transfers. Two have received nerve transfers with or without grafts to the biceps muscle with the goal of restoring elbow flexion. One has had a nerve transfer to the quadriceps with the goal of improving knee extension. Currently, the results of these procedures are unknown. Referral for nerve transfers must be made early in the course of recovery, as experience with the neonatal and traumatic nerve injury populations shows that better outcomes are achieved if these procedures are performed within 3 to 9 months, as fewer muscle fibers and less motor endplate atrophy will be present. 30 The goal of muscle and tendon transfer procedures is to stabilize joints (such as the shoulder or ankle) and to potentially improve function (such as elbow flexion or ankle plantar flexion or dorsiflexion). Because these procedures are not as time-sensitive, 823

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