CHAPTER 28

Evaluation, Treatment Planning, and Nonsurgical Treatment of Cerebral Palsy

Ann Tilton and Daniella Miller

DEFINITION OF CEREBRAL PALSY

Cerebral palsy (CP) is a clinical syndrome, rather than a specific disease. Although older definitions focused exclusively on the motor disorder, the newest definition from the Executive Committee for the Definition of Cerebral Palsy from the American Academy for Cerebral Palsy and Developmental Medicine broadens the focus to include accompanying disorders:

Thus, the label of CP does not imply anything specific about etiology, and the child with CP may or may not have significant impairments of other systems beyond the motor system. This chapter primarily focuses on the motor aspects of CP, and their nonsurgical treatment.

ETIOLOGY AND EPIDEMIOLOGY

The prevalence of CP is approximately 2 to 3 in 1,000 live births, making it the most common cause of physical disability in children in developed countries. Prenatal disturbances including infection, clotting disorders, and inflammation are the most common cause of CP, and are often the most difficult to diagnose. Perinatal asphyxia is no longer considered a leading cause of CP, likely accounting for less than 10% of cases (2).

Although the brain injury causing CP is static, the consequences for the child often are not, due to developmental changes. Spastic posturing and muscle contracture, for instance, may become more severe as the child grows.

CLINICAL PRESENTATIONS OF CP

The movement disorders of CP may be classified based on their distribution and the types of movements present, as indicated in Table 28.1. The classification may be used to help guide therapy decisions, as discussed in more detail in the following paragraphs.

The motor symptoms of CP can also be grouped into four categories based on their functional consequences (3):

TABLE 28.1

Note: CP, cerebral palsy.

EVALUATION

A comprehensive evaluation of the child with CP is the cornerstone of treatment planning. Although we confine ourselves here to motor evaluation, it is of paramount importance that the child be fully evaluated in all realms, including sensory, language, social, family, school, and recreational activities. The entire treatment team—physicians, allied health therapists, orthotists, dieticians, nurses, and caregivers, as well as surgeons and other professionals as needed—should have input into the evaluation process. Likewise, short- and long-term goals for important issues such as functional mobility, self-care, and communication must be established and reevaluated at each visit. Beginning in adolescence there should be an emphasis on individualized transition planning.

Evaluation of muscle tone is typically done using the Modified Ashworth Scale (MAS; [4]). This clinically useful scale grades tone from 0 to 4, as indicated in Table 28.2. Of note, it does not measure function. The goal of measuring excess tone is not simply to document its presence, but also to determine whether it is interfering with some aspect of function, care, comfort, or cosmesis. If it is, treatment may be warranted. If it is not, or if reducing tone would present new problems (such as increased difficulty with transfers) that outweigh the benefits, then a specific treatment should not proceed.

TABLE 28.2

Source: From Ref. (4). Bohannon RW, Smith MB. Interrater reliability of a modified Ashworth scale of muscle spasticity. Phys Ther. 1987;67(2):206–207.

TABLE 28.3

Source: From Ref. (5). Palisano R, Rosenbaum P, Walter S, et al. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol. 1997;39(4):214–223.

Evaluation should also include determining the child’s level of function, which is often done using the Gross Motor Function Classification System (GMFCS), as shown in Table 28.3 (5). Other useful scales include the Functional Independence Measure (FIM; [6]), and the Barthel Index (7). GMFCS is good for gross motor classification; however, the other scores may be valuable in combination with physical exam (MAS and Tardieu Scale) to better document the child’s functional status as it changes over time, and in response to therapy. When choosing a measure to use for longitudinal evaluation, it is critical that it be relatively easy to administer, in order that it be used routinely during clinical visits.

TREATMENT PLANNING

Once the decision to treat has been made, multiple considerations come into play in determining the choice and timing of treatment. The goal in all cases is to maximize the functional independence of the child, and to facilitate efforts by the family to aid in the goal. For example, the therapeutic program for the school-aged child should be tailored to maximizing the child’s ability to participate in school activities—sitting in class, taking part in play, carrying out fine motor activities such as writing, and especially communicating with others.

Factors to consider in choosing therapy include the age of the child; any comorbidities including ophthalmologic, cognitive, or seizure disorders; distribution of excess tone; existing or potential spasticity-related complications (eg, contracture); mobility; and recreational activities that may be incorporated into the therapeutic program. Additionally, financial and transportation concerns may impact the decision on the most appropriate treatments.

Depending on the child, treatment goals may include:

Treatment options include:

PHYSICAL TREATMENTS

Stretching is an essential part of any treatment program for muscle overactivity in CP. Spasticity leads to adoption of fixed postures, setting the stage for muscle contracture. Stretching counteracts contracture development by maintaining the full range of motion of affected muscles. The normal or adapted activities of play of an active child can often provide a significant portion of the needed range of motion and stretch. Special attention must be given to those joints limited by muscle overactivity.

Physical therapy goes far beyond stretching, however; programs include motor retraining, and strengthening. The physical therapist aids in the selection and modification of orthotics and mobility aids, such as walkers or wheelchairs. The physical therapy program may also include activities such as horseback riding, hydrotherapy, and modalities such as biofeedback and electrical stimulation. How to deliver therapy is often debated. Bower et al have shown that intensive therapy (1 hr/d, 5 d/wk) was not more effective in improving function than routine amounts of therapy (8). However, contrary to the traditional approach to therapy, studies suggest that periods of intense therapy for a few weeks with several weeks off between sessions may be equally as effective as continuous therapy one to three times per week (9).

An ankle-foot orthosis (AFO) is often used to treat dynamic equinus in CP, and has been shown to reduce ankle excursion and increase dorsiflexion at foot strike, along with other biomechanical benefits (10). For preambulatory children whose ability to stand is impaired by equinus, an AFO can also aid the sit-to-stand transition (11).

Hand splints are commonly used to either improve hand function, or to prevent or correct muscle contracture. A systematic review with meta-analysis of six studies investigated the effectiveness of hand splints in conjunction with therapy in CP patients. Moderate-quality evidence showed a very small positive effect on upper limb skills when combined with therapy; however, this effect is diminished and possibly not maintained at 2 to 3 months after splint removal. Therefore, the small potential benefits of this device must be balanced with the potential costs, including negative cosmesis and discomfort for the child (12).

Children with balance difficulties are often aided with a walker or crutches. In patients with spastic diplegia, posterior balance is usually the major limitation to mobility; a posterior walker or crutches may allow the child to ambulate independently.

When stretching is inadequate to regain full range of motion in a tight or contracted joint, serial casting or prolonged splinting may be indicated. The cast is typically applied at a fraction of the desired final angle, and then reapplied at an increased angle every 4 to 7 days. Once the desired joint range of motion is achieved, an orthosis may be fabricated to use as a splint to maintain the desired angle. Although there is debate as to the optimal technique, serial casting is often used in combination with BoNT injections, with the toxin first weakening the overactive muscle, and the cast use beginning 7 to 10 days later to apply prolonged stretch. In comparative trials, toxin injection alone has been shown to be as effective as casting alone (13,14). The two treatments together were proven to be superior to either alone in one trial (15), but other trials have not supported this (16,17). This important issue is reviewed in more detail by Romeiser Logan and Gaebler-Spira elsewhere (18).

Recently, interest in constraint-induced therapy (CIT) has undergone a renaissance, based on increased understanding of the brain’s inherent capacity for “rewiring,” known as plasticity. The underlying idea is to prevent the patient from using his or her more functional extremity, forcing the brain to strengthen firing patterns that work around the damaged motor control region to activate and control the impaired limb. Taub et al studied 18 children with hemiparesis, ages 7 months to 8 years, randomly assigned to CIT or conventional PT. Constraint was applied for 21 days, 6 hours per day (19). CIT-treated children acquired significantly more new classes of motor skills (9 vs 2), and used the affected limb more and with better-quality movements. These benefits were sustained at 6 months, suggesting the occurrence of true plasticity-dependent brain changes. Similar beneficial results have been seen in other recent trials (20,21). However, a CIT program may be a significant burden to the child and the family, and more research is needed to define the least intrusive and most effective regimen.

In contrast to CIT, hand arm bimanual intensive training (HABIT) provides intensive repetitive task training of bimanual coordination. This approach was developed in response to limitations in the inability of CIT to enable practice of functional activities that are inherently bimanual, for example, pulling apart objects, stabilizing a piece of paper while drawing, or tying shoelaces. In a randomized clinical trial of 42 participants with hemiplegic CP between the ages of 3.5 and 10 years, bimanual training was shown to be equally as effective as CIT when changes in Jebsen–Taylor Test of Hand Function (JTTHF) and Assisting Hand Assessment (AHA) scores were measured (22).

ORAL MEDICATIONS

Oral medications can effectively reduce muscle overactivity in the child with CP, but often at the expense of significant adverse effects, especially sedation. For this reason, their role is not as large as that of other treatment options. They also reduce tone globally, which, for many children with focal spasticity, is not desirable. Thus, oral medications may be most appropriate for those with widespread spasticity for whom some sedation is not a contraindication. Relatively few double-blind trials of oral medications have been conducted in children. Additionally, older studies measured technical measures such as tone but did not address functional gains.

Related posts:

Measurement Tools and Treatment Outcomes in Patients With Spasticity Emerging Technologies in the Management of Upper Motor Neuron Syndromes Botulinum Toxin in the Treatment of Upper Limb Spasticity Assessment of Spasticity in the Upper Extremity Techniques and Scales for Measuring Spastic Paresis Pharmacologic Management of Spasticity: Oral Medications

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