Introduction

Rehabilitation is the process of restoration following injury or disease, with the goal of maximizing an individual’s ability to function in a normal manner. The application of rehabilitation extends far beyond the bounds of traditional medicine since one of its chief aims is to protect or restore personal and social identity [Ward and McIntosh, 2003]. Neurorehabilitation therefore encompasses two distinct, but overlapping, concepts. The first is that neurorehabilitation is a medical subspecialty that treats patients with disabling (and often chronic) diseases of the central and peripheral nervous system [Selzer, 1992]. So defined, neurorehabilitation translates into the active process designed to reduce the effects of a primary neurological condition on performance of activities in daily life. A second aspect stresses the specific therapeutic modalities that are implemented to overcome or improve any neurological impairment that interferes with daily life [Ward and McIntosh, 2003]. Both aspects clearly have practical implications for neurorehabilitation as it is carried out in children [Taylor, 1991].

The principles, goals, and challenges of both pediatric neurorehabilitation and rehabilitation in general are best understood in the context of the International Classification of Functioning, Disability and Health, which replaces the previous scheme entitled the International Classification of Impairments, Disabilities and Handicaps [Thomas-Stonell et al., 2006]. As originally defined by the World Health Organization, impairment is the loss or abnormality of physiologic, psychologic, or anatomic structure or function, while disability, the lack of ability or restricted ability to perform a functional task, is very dependent upon the individual’s environment. Thus, one of the ways in which neurorehabilitation can be carried out is to modify or adapt the environment. Appropriate neurorehabilitation of disability in childhood must be predicated upon a clear understanding of normal development, as well as the ramifications of abnormal development. Finally, it must be recognized that disability does not flow solely or directly from the type and degree of impairment. Instead, disability is best viewed as a three-dimensional construct, consisting of impairment, activity limitations, and participation restrictions [WHO, 1997]. Each dimension is the result of interaction between the biologic features intrinsic to the individual’s medical condition and the child’s physical and social environment [Bickenbach et al., 1999].

The concept of dependency also plays a fundamental role in medical rehabilitation [Ward and McIntosh, 2003]. Dependency is more readily quantifiable than disability, and degree of dependency is a critical element in the cost of on-going medical and rehabilitation care, as well as in eventual quality of life. While a variety of scales have been developed to measure a patient’s potential autonomy, or conversely, the burden imposed on a caregiver, use in pediatric patients is limited because of the inter-related issues of anticipated development and maturation. In our experience and that of others, the WeeFIM (Functional Independence Measure) is a very useful rehabilitation tool [Rice et al., 2005].

The disablement/dependency paradigm in pediatric neurorehabilitation emphasizes movement towards appropriate functional outcomes, as well as diagnosis, and thus is more informative than traditional neurodiagnostic categories [Taylor, 1991]. There also appear to be other distinct advantages to conceptualizing neurorehabilitation in this manner. First, therapeutic interventions and treatment modalities should be based primarily on the disablement and resultant activity limitations, as opposed to the underlying pathologic process producing the impairment. In practical terms, this means that rehabilitation techniques often may be transferred successfully from one child to another despite differences in their primary neurological diagnosis [Ward and McIntosh, 2003]. Similarly, the process of neurorehabilitation logically involves a multidisciplinary team, comprised of physicians, nurses, therapists, and other healthcare professionals, who work in collaboration with the patient and the patient’s family. Finally, the paradigm provides a framework for patients that can be expanded to encompass all of the multifaceted aspects of neurorehabilitation inherent in the practice of child neurology. For example, the paradigm works just as well in describing an adolescent with seizures (impairment) who is prohibited from driving an automobile (disablement) and thus relies on his parents for rides (dependency), as it does for a child with a lower thoracic spinal cord injury causing paralysis of the legs and thus a limited capacity to ambulate. In both cases, there are resultant activity limitations that will affect social life and future employment opportunities.

Mechanisms Underlying Functional Recovery in the Nervous System

Implicit in any consideration of the rehabilitation process is the recognition that the potential for recovery of function exists. The degree of recovery following injury to the nervous system is variable but rarely complete. None the less, a better understanding of the cellular and molecular bases of neuronal dysfunction, as well as the potential mechanisms by which recovery of function occurs, is the framework upon which development of any pediatric neurorehabilitation program must rest [Noetzel, 2003]. Since each proposed mechanism of recovery follows a different time course, the implications are very important in defining an appropriate therapy program.

Mechanisms regarding potential recovery of neurological function are, at best, poorly understood. However, several general conclusions, based upon an increasing amount of scientific evidence, can be drawn. First, significant capabilities of biologic modification in response to injury and developmental aberrations exist within the nervous system. The potential mechanisms by which the nervous system can respond to injury are exceedingly varied and more dynamic than suggested by prior studies [Taub et al., 2002]. Finally, meaningful recovery of function continues for an extended period of time, one that is longer than previously recognized [Graham, 1999]. These findings stand in contrast to former beliefs that recovery essentially was a biphasic process, in that any improvement that occurred within the first few months following an injury was felt to be due to reversibility of factors affecting dysfunctional, as opposed to dead, tissue. Improvement in functional capabilities at later time periods was attributed to nonphysiologic factors, such as learning and behavior modification. Presently, however, there is strong evidence that changes in neuronal circuitry, as opposed to psychologic factors, mediate recovery of function [Johnston, 2009]. The level of scientific information does not allow us to separate out one set of factors from another. Concerning acquired injury to the central nervous system (CNS), it is appropriate to acknowledge that there is ongoing debate concerning the relative contributions of biological healing and learned adaptations to the process of functional recovery.

Principles of Pediatric Neurorehabilitation

Disorders involving the CNS represent a large proportion of all severe and complex disabilities, especially those secondary to trauma and other acquired injury. This situation is especially true in the pediatric population, where injury to or malformation of the brain and spinal cord accounts for the vast majority of children referred for rehabilitation. Pediatric neurologists therefore are uniquely positioned to contribute in a meaningful way to the discipline of pediatric rehabilitation [Taylor, 1991].

Pediatric rehabilitation has several guiding principles based upon the nature of the discipline, as well as the age and developmental level of the patients requiring treatment (Box 105-1) [Noetzel, 2003]. A fundamental principle of neurorehabilitation is that the process mandates a coordinated transdisciplinary team working in unison to provide integrated evaluations and therapeutic interventions. A variety of “nonmedical” issues, such as home and school accessibility, psychosocial adaptation to disability, and school reintegration, mandates that the team include individuals who can address these all-important considerations. A second essential principle is that the rehabilitation process must concentrate on strategies designed to effect true functional improvement, as opposed to enacting treatments that merely decrease symptoms without resulting in improvement in a patient’s capabilities. In pediatric rehabilitation, this means establishing practical management decisions that are endorsed not only by the patient, but also by the family. In order to accomplish this goal, the team must have a clear understanding of the physical, emotional, cognitive, and social consequences of a child’s injury [Ylivsaker et al., 1999]. The degree, extent, and rate of recovery, which varies significantly among children, as well as differences in functioning from setting to setting and task to task in a single child, mandate continual reassessment. As previously described, the immature brain’s response to injury is both varied and dynamic. This evolving biologic recovery, in combination with substitute/alternative cognitive processes and movement patterns, necessitates an on-going program of assessment and reassessment. Additionally, progress from the baseline condition hopefully will be observed as a direct response to the therapeutic interventions. Rehabilitation, especially as it pertains to nervous system injury, has been described as a spiral management process in which, following an initial evaluation, a treatment program is initiated; this, in turn, is constantly revised and updated, based on successive reassessments, taking into consideration therapy-mediated improvements [Ward and McIntosh, 2003].

Box 105-1 Principles of Pediatric Neurorehabilitation

Well-coordinated transdisciplinary therapy team

Interventions initiated as early as possible

Therapeutic strategies predicated on achieving functional improvement

On-going reassessment of the child and revision of the treatment program

Re-evaluations guided by an understanding of normative development

Another fundamental and distinguishing principle of pediatric rehabilitation is that these frequent reassessments of cognitive, motor, and psychosocial deficits, especially when resulting from acquired nervous system injury, must be guided by an understanding of normative development. Detailed understanding of the patterns of cognitive development during maturation is especially important in the rehabilitation of infants and children for several reasons. First, normative developmental milestones can be identified as sequential goals within an individual’s therapy program [Ylivsaker et al., 1999]. In addition, as a result of injury or illness, the pediatric patient typically loses age-appropriate developmental capabilities. As such, the manner in which therapy can be provided to the patient and the individual’s ability to respond to it will be limited. This is most notable in children and adolescents early in the recovery process, who lack the developmental skills necessary to learn complex strategies upon which functional substitution is predicated [Noetzel, 2003]. Finally, an understanding of cognitive development allows more accurate prediction of the long-term effects of cerebral injury sustained by young children. This is most pertinent for areas of brain subserving functions that normally mature later in childhood, such as executive functioning within the frontal lobes.

A final guiding principle of rehabilitation in the pediatric patient is that intervention should begin as soon as possible. Coma is not a contraindication to the initiation of rehabilitation management strategies, and thus, once the patient is medically stable, therapy can and should be instituted, even while the child is still in the intensive care unit [Michaud et al., 1993]. This early phase of intervention is designed to limit maladaptive behavioral habits and movement patterns, and to prevent or at least minimize complications that can take months to resolve, if not properly and promptly addressed following the acute injury. Specific goals include: prevention of physical deformities, such as contractures due to abnormal spasticity and prolonged immobilization; maintenance of skin integrity; and reduction in the manifestations of dysautonomia secondary to fluctuations in muscle tone or bladder distinction [Noetzel, 2003].

Medical Aspects of Acute Pediatric Rehabilitation Management

Nutritional support is a key element of the acute management process [McLean et al., 1995]. In any child with an acquired nervous system injury, the risk for compromised nutrition is very high. There are a variety of causes that contribute to this risk, such as a hypermetabolic state resulting from systemic trauma and/or infection [Feldman et al., 1993]; delayed gastric emptying and intestinal dysmotility and stasis; oral pharyngeal dysfunction, which, even if recognized, can lead to aspiration pneumonia; and underestimation of caloric requirements necessary for repair and growth [Phillips et al., 1987]. Feeding through a nasogastric tube should be implemented while the patient is in the intensive care unit. An exception would be cases of major intra-abdominal injury; in these instances, hyperalimentation may be warranted. Oropharyngeal dysfunction and resultant swallowing difficulties secondary to bilateral cerebral hemisphere injury and/or lower cranial nerve involvement are an almost universal complication of acquired brain injury in the pediatric population [Bruce, 1990]. Thus, a detailed evaluation by a rehabilitation feeding team is mandatory [Logemann and Ylvisaker, 1998]. As neurological recovery proceeds, gradual introduction of oral feeding begins, with a commensurate decrease in supplemental alimentation based upon assessment of caloric need by a pediatric dietician.

Other medical issues often impact on acute neurorehabilitation management and, in addition, can impair the accuracy of on-going reassessment by the team of rehabilitation specialists. Fever and associated infection are a near-universal complication seen in pediatric patients with acute neurorehabilitation needs [Suz et al., 2006]. In those with intracranial trauma, the possibility of CNS infection must be considered [Bell et al., 1992]. In patients with penetrating cranial injury and basilar or compound skull fractures, the risk of bacterial meningitis and brain abscess is increased significantly. Sinusitis is a relatively common infection secondary to the frequent need for nasotracheal intubation or long-term nasogastric tube utilization. Other equally obvious sources of infection include indwelling urinary catheters and intravascular lines. Fever of central origin secondary to hypothalamic injury also has been documented in children with acquired brain injury. The setting commonly is one in which other clinical features of dysautonomia are observed, most notably elevated heart rate and blood pressure [Krach et al., 1997]. However, the diagnosis of fever of central origin is not a tenable one until all other sources of infection have been carefully evaluated and excluded, including drug-induced fever.

Dysautonomia, a syndrome characterized by simultaneous and paroxysmal sympathetic and muscle overactivity, typically follows severe traumatic, as well as other forms of acquired brain injury. The incidence varies between 5 and 12 percent in children [Blackman et al., 2004], its presence correlating with the most severe injury and the worst prognosis. Autonomic changes include marked tachycardia, hyperventilation, hypertension, fever, and increased sweating; motor changes include (decerebrate or decorticate) posturing, dystonia, rigidity, and spasticity [Pranzatelli et al., 1991]. Evidence-based treatment paradigms for dysautonomia in pediatric patients are nonexistent, with all evidence being anecdotal in nature. Gabapentin and bromocriptine have been reported to reduce the number and severity of paroxysms in pediatric patients [Russo and O’Flaherty, 2000], while in adults there is evidence that additional medications, such as propranolol, labetalol, clonidine, morphine, and midazolam, can be beneficial [Baguley, 2008]. Intrathecal baclofen also has been shown to be quite effective, even in children who are resistant to oral medications [Turner, 2003].

Disturbances in endocrine function, although less common than infection, also can complicate severe acquired brain injury [Einaudi and Bondone, 2007]. Damage to the pituitary and hypothalamic glands can produce the syndrome of inappropriate antidiuretic hormone secretion (SIADH), or conversely, central diabetes insipidus, in which ADH secretion is insufficient [Lin et al., 2009]. Although generally transient, both of these conditions mandate judicious fluid and electrolyte management, as well as pharmacologic treatment with desmopressin acetate in cases of diabetes insipidus.

Gastrointestinal disorders are also common in the pediatric population with acquired nervous system injury. These range from delayed gastric emptying and intestinal stasis to hemorrhage secondary to reflux esophogitis, gastric hyperacidity, and stress ulcers [Cochran et al., 1992]. Additionally, any child with a feeding tube is at risk for damage to the gastric mucosa. Thus, prophylactic management with H₂ receptor blockers is indicated. Finally, in children with severe traumatic injuries involving the nervous system, there is a high incidence of skeletal fractures, typically of the long bones [Brown et al., 2006]. Such fractures may not be apparent early on in the comatose patient or in children with spinal cord injury, but reveal themselves during the early course of neurorehabilitation treatment [Sobus et al., 1993]. Close coordination between the orthopedic surgeons and the therapy team in regard to range of motion exercises and weight-bearing status is essential to optimize the response to therapy.

Comprehensive Pediatric Rehabilitation Programs

Once the above noted medical and surgical issues have stabilized, a decision has to be rendered regarding the direction in which the rehabilitation process should proceed. In some children, recovery has been sufficiently rapid for discharge from the acute care setting and institution of outpatient therapy or a day treatment program to be appropriate. For others, progress has been and likely will continue to be very slow. In these cases, a subacute nursing center with less intense physiotherapy, occupational therapy, and speech therapy is a reasonable consideration. The final alternative of a dedicated pediatric rehabilitation program is indicated for the vast majority of children and adolescents with moderate to severe acute neurological injury. Typical management issues demonstrated by these patients include:

immobility – inability to walk, climb stairs, or transfer to/from a wheelchair

dependence in self-care activities – drinking, eating, dressing, maintaining personal hygiene, and applying a brace or prosthesis

aphasia with significant receptive or expressive dysfunction

cognitive or perceptual motor dysfunction

incontinence of bowel or bladder

limited functional performance due to poorly controlled pain.

Another group recognized to benefit from an intensive inpatient program of rehabilitation therapy are those patients with severe neuromuscular deconditioning secondary to prolonged intubation and ventilatory support, with the concomitant need for pharmacological paralysis and sedation. This latter group includes many children who have received heart or lung transplants, even in the absence of any overt CNS injury.

Over the last several years, eligibility criteria for admission to comprehensive neurorehabilitation services have been developed and refined (Box 105-2). At most major pediatric hospitals, these criteria include the following: a primary condition that is medically stable, but one that mandates intensive and multidisciplinary rehabilitation care; the premorbid physical and cognitive level of the child indicate potential for meaningful recovery; the patient is responsive to verbal or visual stimuli and has sufficient alertness to participate in the rehabilitation process; and the injury/illness diagnosis allows a reasonable expectation for improvement, with the potential to achieve clearly identifiable treatment goals within a reasonable period of time.

A comprehensive pediatric rehabilitation program should provide a very highly intensive service through a transdisciplinary coordinated team approach. Most programs mandate an initial minimum of 3 hours of therapy each day; as recovery proceeds and endurance improves, the duration and scope of treatment are expanded to a full day of activity. The program usually is supervised by a physician with specialized training and experience in one of the disciplines of rehabilitation medicine. Rehabilitation nursing care/supervision must be provided 24 hours a day. Although therapeutic interventions carried out by physical and occupational therapists and speech-language pathologists are the mainstay of any treatment program, most rehabilitation teams also include psychologists, social workers, child-life specialists, music therapists, chaplains, orthotists, and dieticians. In any program with a large number of children with acquired brain injury, a pediatric neuropsychologist is an essential member of the team. Our rehabilitation service also benefits from having horticultural and art therapists participate in the care of our patients. Formal written team evaluations typically occur at least every other week to assess progress and impediments to recovery; consider possible solutions to the impediments; reassess and adjust established goals; and develop and implement discharge plans.

Management of Spasticity

Overview

Almost every single patient requiring acute neurorehabilitation services will manifest alterations in tone as a result of injury or illness. In addition, spasticity is a characteristic feature of many chronic motor disorders affecting infants, children, and adolescents. In the pediatric population, spasticity is most commonly found in children with hypertonic forms of cerebral palsy. However, spasticity can result from any disorder causing damage to or abnormal development of nerve cells or pathways controlling motor movements, either in the brain or in the spinal cord [Sanger et al., 2003]. Thus, spasticity is commonly observed in children with brain tumors and those with vascular, traumatic, infectious, and hypoxic injury to the brain or spinal cord.

In the early phase following CNS injury, absent or reduced tone typically is encountered; over time, the pattern often evolves into spasticity as one part of the upper motor neuron syndrome [Noetzel and Miller, 1998]. Spasticity is defined as an exaggerated response to passive movement of a limb, inducing a velocity-dependent involuntary resistance of the stretched muscle. Spasticity is characterized by excessive and inappropriately timed activation of skeletal muscles, which often interferes with a child’s ability to move voluntarily in a normal fashion. The clinical manifestations of spasticity vary, depending upon the sites of injury within the CNS. Extent of damage, as well as intrinsic characteristics of recovery, influences the presentation of spasticity. However, in almost all pediatric patients, spasticity will impair passive movement and static postural alignment [Noetzel and Miller, 1998]. In addition, impaired activation of voluntary movement, resulting in weakness and clumsiness, often may accompany spasticity [Tilton, 2009]. Spasticity must be differentiated from other manifestations of impaired movement, such as dystonia and rigidity, both of which typically occur within the context of an associated or a secondary injury due to ischemia and hypoxia [Sanger et al., 2003].

Spasticity to any significant degree interferes with a child’s control of voluntary movement, coordination, exercise tolerance, and range of motion in the joints. Invariably, this results in a state of excessive energy expenditure, compared to able-bodied children [Rose et al., 1990]. Spasticity typically impedes a child’s independence in activities of daily living, and may cause pain and disturb sleep. In the more severely affected individuals, patient care often is especially difficult. Over time, spasticity reduces protein synthesis in muscles and impairs longitudinal growth; the end result typically is permanent shortening of muscles (contractures) and the development of bony deformities [Lieber et al., 2004].

Treatment of spasticity should be predicated upon establishing goals that improve a child’s functional capabilities [Tilton, 2009]. Thus, the number one reason for attempting to decrease spasticity in the pediatric population is to improve functional movement, either upper-extremity use in activities of daily living, or leg and trunk movement to achieve ambulation. Improvement of head, neck, and trunk posture by reducing spasticity may have additional benefits in some children: namely, better oral feeding and breath support for speech. It must be recognized, however, that spasticity may impart advantages in certain individual cases, including maintenance of muscle bulk and tone; support of circulatory function; assistance in transfers and ambulation; and assistance in activities of daily living. Once the goals of treatment have been established, decisions can be made as to which treatment option is most likely to achieve the desired success (Figure 105-1).

Fig. 105-1 Treatment options for a child with spasticity.

(Modified from Tilton AH, Maria BL. Consensus statement on pharmacotherapy for spasticity. J Child Neurol 16:66–67, 2001.)