Cerebral Palsy

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CHARTER

20
Cerebral Palsy
spasticity management usually’ rely on input from
physical therapists. The therapist’s influence is not
SANDRA J. ONLNEY, BSc restricted
(P&OT), Med,
to the PhDcenter and treatment
medical
MARILYNJ. WRIGHT, BSCPT

NATURE AND CHARACTERISTICS OF Preschool Period


School-Age and Adolescent Period
CEREBRAL PALSY Transition to Adulthood
Classification, Etiology and Pathophysiology
Progress in Primary Prevention
RESEARCH NEED
Impairment GLOBAL ISSUES
Determinants of Prognosis or Outcome PROFESSIONAL ISSUES
EXAMINATION, EVALUATIAB, AND CADE HISTORIES
INTERVENTION
Infancy

Cerebral palsy’ (CP) is the neurologic


condition most frequently encountered by pediatric gymnasium, but frequently includes consultation re-
physical therapists. Jr is a permanent but not garding the child’s functioning in settings within the
unchanging neurodevelopmental impairment caused by home, school, recreation, and community environ-
a non progressive defect or lesion in single or multiple ments. Good therapy not only helps the child with CP
locations in the immature brain. The defect or lesion but also can have a positive influence on the child’s
can occur in utero or during or shortly after birth and family and caregivers. In summary, parents of children
produces motor impairment and possible sensory with disabilities want services that provide general and
deficits that are usually evident in early infancy specific information about their child, provide
(Scherzer & Tscharnuter, 1990). CP involves one or coordinated and comprehensive care, and are provided
more limbs and frequently the trunk. It causes dis- by’ respectful and supportive professionals (King et al.,
turbances of voluntary motor function and produces a 1998). The pediatric physical therapist is ideally’ suited
variety of symptoms. Nevertheless, CP is itself an to fill these roles.
artificial concept, comprising several causes and clin- This chapter discusses the background of
ical syndromes that have been lumped together because cerebral palsy the classification, etiology, and
of a commonality of management. The impaired pathophysiology; prevention; associated impairments;
control and coordination of voluntary muscles is and determinants of outcomes. Physical therapy
accompanied by mental retardation or learning examination, evaluation, and intervention that are
disabilities in 50 to 75% of children and by disorders of related to each age between infancy and adulthood are
speech (25%), auditory impairments (25%), seizure discussed. The chapter concludes with two case
disorders (25-35%), or abnormalities of vision histories.
(40-50%) (Batshaw. & Perret, 1992; Schanzenbacher,
1989). Social and family problems may occur
secondary to the presence of primary deficits.
In few conditions do therapists play such a NATURE AND CHARACTERISTICS OF
central role or have as much potential to influence the CEREBRAL PALSY
outcome of children’s lives. Their interventions have
not only immediate but also lifelong effects, and can be Classification, Etiology,
efficient and cost-effective. Treatment of children is
specialized: therapists provide services that will help and Pathophysiology
them reach their full potential in their homes and
communities. Furthermore, decisions a bout many CP has been classified in a number of ways. A
medical interventions such as orthopedic surgery and classification based on the area of the body exhibiting
impairment yields the designations of monoplegia (one cases result from such events (Nelson & Ellenberg,
limb), diplegia (lower limbs), hemiplegia (upper and 1986). Infection in the perinacal period is also
lower limbs on one side of the body), and quadriplegia important. The effects of hvperbilirubinemia and other
(all limbs). Another classification, based on the most blood incompatibilities frequently resulting in athetoid
obvious movement abnormality resulting from CP are a concern, particularly in developing countries.
common brain lesions, yields spastic, dyskinetic, and The statistics from developing countries are in
ataxic types. The spastic type, in which the muscles are sharp contrast to those of developed countries. Studies
perceived as excessively stiff and taut, especially have suggested that up to 63%. of cases in the former
during attempted movement, results from involvement have preventable causes associated with shortage of
of the motor cortex or white matter projections to and care personnel and inadequate financing for effective
from cortical sensorimotot areas of the brain. services (Karumuna & Mgone, 1990; Nottidge &
Involvement of the basal ganglia is reflected in Okogbo, 1991).
dyskinesia or athetosis and sometimes in intermittent The dramatic decrease in perinatal mortality in
muscular tension of the extremities or trunk and developed countries during the 1980s, largely a result
involuntary movement patterns. A cerebellar lesion of improved survival rates for low birth weight infants,
produces ataxia, or general instability of movement. A has given rise to the fear of increased numbers of
hypotonic classification, not known to be related to a children with neurodevelopmental impairments. In fact,
particular lesion, is characterized by diminished resting there was a steady-state incidence of CP of about 2.5
muscle tone and decreased ability to generate voluntary per 1000 live births until about the mid-1950s (Little,
muscle force. Symptoms of spasticity and dyskinesia 1958), which was followed by a decrease in incidence
may both be present in a child, with the type of CP to about 1.5 per 1000 for about 15 years. Since then,
referred to as mixed. The degree of severity of CP the incidence has increased to near mid-1950s Levels,
varies greatly, and the designations mild, moderate, if Swedish statistics are typical (Hagberg et al., 1989b).
and severe are often applied within types. The Gross The change in this trend mainly reflects changes in the
Motor Disability Classification System is a five-level, live birth rate of preterm infants, especially in those
age categorized system that places children with CP with spastic diplegia. A study conducted between 1982
into categories of severity that represent clinically and 1994 of a cohort of 2076 consecutively born
meaningful distinctions in motor function (Palisano et infants with birth weights of 500 to 1500 g (O’Shea et
al., 1997). Although the proportions of the various sub- al., 1998) concluded that the increasing survival of
types of CP vary with the reporting source, a series very low birth weight infants has not resulted in an
from Sweden noted that hemiplegia accounted for increased prevalence of cerebral palsy among
36.4%; diplegia, 41.5%; quadriplegia. 7.3%; dyskinesia survivors. The risk of CP increases sharply with
or athetosis, 10%; and ataxic forms, 5% (Hagberg et decreasing birth weight (Atkinson & Stanley, 1983;
al., 1989a). Hagberg et al., 1989a; Pharoah et al., 1987) and has
CP is a condition with multiple causes leading been reported to be as much as 40 times higher in
to damage within the central nervous system. Although infants weighing less than 1000 g (Hagberg et al.,
the causes are not completely understood, certain 1989b).
prenatal, perihatal, and postnatal factors have been The increased risk of CP in preterm infants
associated with CP (Torfs et al., 1990). There is gen- must be put in perspective, however. Reports of long-
eral agreement that the majority of cases of CP in term term outcome of extremely premature infants, that is,
infants are due to prenatal or unknown causes, whereas infants born between 24 and 28 weeks of gestational
in the vast majority of preterm infants the lesion age, suggest about a 75% survival hte, with more than
causing CP develops during the perinatal period. 50% of those who survive free of major neuro-
Preterm birth, although not believed to be causative, is developmental impairments, about 25% with major
associated with up to 33% of all cases, including more impairments, and about 11% with CP (Msall et al.,
than 50% of diplegia (Pharoah et aL, 1990), 25% of 1991).
hemiplegia (Uvebrandt, 1988), and 5% of quadriplegia Autopsies of infants have revealed three types
(Edebol-Tysk, 1989). Prenatal malnutrItion, intrinsic of neuropathic lesions (Weinstein & Tharp, 1989): neu-
developmental problems of the fetus, poor maternal ropathy resulting from hemorrhage below the lining of
prenatal condition, and maternal infection are also the ventricles (subependymal), encephalopathy caused
associated with CP (Menkes, 1990). Intracranial by anoxia or hypoxia, and neuropathy resulting from
hemorrhage, especially among premature infants, is a malformations of the central nervous system.
wellestablished causal factor. Neonatal asphyxia is a Most subependymal hemorrhages occur in in-
significant perinatal event, but only a small minority of fants of less than 28 weeks of gestational age and those
with low birth weight. Intraventricular hemorrhages, Progress in Primary Prevention
present in up to 46% of infants weighing less than 1500
g (Papile et al., 1978), are thought to develop The primary way to reduce the incidence of
secondary to lesions of ischemic origin. In most cases, CP is through good socioeconomic health of the
blood ruptures into the lateral ventricle and the ensuing population coupled with maternal education. The role
connective tissue blocks the cerebrospinal fluid flow, of poverty and low socioeconomic status in the preva-
frequently resulting in hydrocephaLus. Anoxic or lence of CP (Dowding & Barry, 1990) and in deter-
hypoxic encephalopathy results in gray matter and mining the need for special educational resources
white matter lesions. Gray matter lesions are diffusely (Msall et al., 1991) has frequently been overlooked, yet
present throughout the cortex, basal ganglia and there is empirical evidence of its importance.
thalamus, brainstem, and spinal cord, whereas lesions Certain maternal prepregnancy and
in the white matter are frequently in the periventricular pregnancy-related risk factors are associated with
zone. Periventricular atrophy has been identified as the delivery of a child with a disability (Holst et al., 1989).
most common abnormality found in preterm infants Studies have suggested that improved intrapartum
who developed hemiplegic CP, occurring in 50% of diagnosis of risk factors, prevention of asphyxia, and
cases (Wiklund et al., 199 lb). It is unclear, however, medical treatment of children with low Apgar scores
whether the lesions occur before, during, or after birth. would reduce the incidence of disabilities, as would
Although periventricular atrophy is a bilateral lesion intervention to prevent premature rupture of mem-
thought to be responsible for most cases of preterm branes. However reasonable these hypotheses may
spastic diplegia, it has also been reported as an seem, no studies are known to have tested them.
asymmetric or unilateral lesion or one with bilateral The role of the obstetrician in preventing CP
lesions expressing only unilateral clinical symptoms before birth occurs is limited (Weinstein & Tharp,
(Wiklund et al.. 199 lb). 1989). Attention is directed toward developing effec-
Malformations of the central nervous system tive prevention of and intervention for premature
may generate hemorrhagic and anoxic lesions. Many delivery, fetal distress, neonatal asphyxia, and me-
factors may be responsible, including drug ingestion, chanical birth trauma. Methods of inhibiting labor have
radiation, and infection by viruses such as herpes met with much success, although the effects on
simplex and rubella. incidence of CP remain unclear. Methods of antepar-
Attempts to relate cerebral lesions to the tum fetal evaluation, including sonographic mea-
extent of disability have had only limited success. With surement, electronic fetal monitoring, fetal pH mon-
respect to the side of expression, in the small itoring, and intrauterine pressure monitoring, have
percentage of children with hemiplegia with bilateral provided the obstetrician with powerful tools for
morphologic findings, subtle physical abnormalities assessing the need for active intervention id the labor
were sometimes seen on the nonhemiplegic side process. Delivery procedures using high forceps and
(Wiklund & Uvebrant, 199la). Results suggest the certain presentations of breech deliveries that were
existence of a continuum between hemiplegia and found to be associated with increased perinatal mor-
diplegià resulting from periventricular lesions. bidity have dramatically decreased in favor of cesarean
Magnetic resonance imaging showed that, of several section. This is partly due to the increased safety of
measures, only the amount of white matter correlated cesarean birth for both the mother and the fetus.
with the severity of disability (Yokochi et al., 1991). In
children with hemiplegia, no significant correlations
between size of lesion and severity of impairment have Impairment
been found, although trends toward the association of
less impairment with smaller lesions have been re- Early detection of CP facilitates optimal
ported (Molteni et al., 1987; Wiklund & Uvebrant, 199 management by the family and the health care and
la). Quadriplegia has been associated with brainsrm educational community. Complicating the picture is the
and basal nuclei damage in addition to cortical and instability of diagnosis; CP is reported to disappear
subcortical lesions (Wilson et aL., 1982). Further over time in many low birth weight infants (Kitchen et
discussion of the causes of the various types of CP can al., 1987). In a study designed to determine the
be found in works by Menkes (1990) and Weinstein accuracy of diagnosis of CP at 2 years of age (Kitchen
and Tharp (1989). et al., 1987), only 55% of those so diagnosed at age 2
were deemed to have CP at age 5, but the diagnosis of
those with moderate or severe involvement did not
change. Only 1% of children not diagnosed at age 2 are evident in the muscular system; malalignments
were identified at age 5 to have CP. Of those children such as femoral anteversion and femoral and tibial
in whom the diagnosis was no longer accurate at age 5, torsion (Cusick & Stuberg, 1992) are secondary
most had minor neurologic abnormalities and left-hand impairments evident in the skeletal system.
preference, but their psychologic test scores were no CP is characterized by insufficient force
different from those of children who had n’ever been generation by affected muscle groups, which is
diagnosed as having CP. consistent with low levels of electromyographic
Tests of neurologic status, motor function, (EMG) activity and decreased moment of force output
primitive reflexes, and posture have been assessed for (Berger et al., 1982). When an activity leads to an
their ability to identify CP (Burns et al., 1989). active contraction, this impairment may be expressed
Although assessments performed at age 1 month failed as a deficiency in power (Olney et al., 1990), or when
to identify several of the infants who later showed CP, considered over time, in work. The term strength may
assessments made at 4 months of age resulted in some refer to any of these measurable factors. Strength mea-
overidentification. At 8 months of age, the presence of surement in neurologic conditions is problematic, but
three or more abnormal signs was highly predictive of when measured, strength has frequently been
CP, and the authors concluded that all but the mildest intimately linked with functional capabilities such as
cases of CP can be identified by that age. Formal tests speed of walking (Bohannon, 1989).
have varying abilities to detect motor abnormalities The clinical term tone is used to describe the
(Harris, 1989). The sensitivity of the Movement impairments of spasticity and abnormal extensibility. A
Assessment of Infants (MAI) has been calculated to be sensation of abnormally high tone may be caused by
73.5% in a high-risk population (Harris, 1987). When spasticity, a velocity-dependent overactivity that is
compared with the Bayley motor scale (Bayley, 1993), proportional to the imposed velocity of limb move-
the MAI identified more than 3 times as many children ment. Spasticity is especially evident in children with
with diplegia at 4 months of age, more than 2 times as clonus but is frequently mistaken for problems of
many children with hemiplegia, and about 1.5 times as extensibility. Supraspinal and interneuronal mecha-
many children with quadriplegia (Harris, 1989). In nisms appear to be responsible for spasticity, with
children at 1 year corrected age, however, the Bayley increased “gain” in the muscle spindles and increased
motor scale demonstrated sensitivities of 100% for excitation of Ia afferents having been ruled out as a
both spastic diplegia and quadriplegia and 75% for cause of spasticity (Young, 1994). There is
spastic hemiplegia. Furthermore, the MM has a lower experimental evidence for three pathophysiologic
rate. of specificity than does the Bayley motor scale; mechanisms: reduced reciprocal inhibition of antag-
that is, a greatez percentage of children with normal onist motor neuron pools by Ia afferents, decreased
outcomes are identified as being at risk. presynaptic inhibition of Ia afferents, and decreased
nonreciprocal inhibition by lb afferents. There is
considerable evidence indicating that reciprocal in-
Physical Therapy-Related impairments hibition is reduced in cerebral palsy (Hallett & Alvarez,
1983; Leonard et al., 1991). Adding to this effect are
Impairments in CP are problems with the recent elegant studies using transcranial magnetic
neuromuscular and skeletal systems that are either an cerebral stimulation that have provided evidence of
immediate result of the existing pathophysiologic pro- simultaneous activation of antagonistic muscle groups
cess or an indirect consequence that has developed over through abnormal alpha motor neuron innervation
time. Impairments can be classified, somewhat (Brouwer & Ashby, 1991). The role of decreased
artificially, into single-system impairments and mul- presynaptic inhibition of Ia afferents in spasticity has
tisystem impairmcnts. been deduced from experiments showing that
vibration-induced inhibition of the H-reflex is much
SINGLE-SYSTEM IMPAIRMENTS less in spastic than in normal muscles, a phenomenon
that has been shown to be mediated by a presynaptic
Single-system impairrhents are expressed in the mechanism in animal models. Finally, nonreciprocal
muscular system and the skeletal system, eventhough inhibition has been reported to be reduced and even
the pathophysiologic damage occurred in the, central replaced by facilitation in persons exhibiting spasticity
nervous system. Primary impairments such as with sustained hypertonia (Young, 1994), which
insufficient force generation, spasticity, abnormal suggests that there may be afurther mechanism
extensibility, and exaggerated or hyperactive reflexes responsible for abnormal alpha motor neuron
excitability.
The sense of abnormally high tone can also overlengthened, which is usually a secondary
result from hypoextensibility of the muscle because of impairment resulting from repeated mechanical stretch,
abnormal mechanical characteristics. Comparing it is termed hyperextensible. The overlengthened
healthy children with children with CP, Berger and muscle complex may also have decreased
colleagues (1982) found that the EMG activity of leg forcegenerating capabilities.
muscles in nearly all children with CP was reduced in Few studies of the histology and morphology
affected limbs and that there were no indications of of spastic muscle have been reported, but they have
pathologic reflex effects on muscle activity. A force shown that differences are present when spastic muscle
transducer on the tendo Achilles measured tension that is compared with normal muscle (Romanini et al.,
was disproportionate to muscle activity and could best 1989). The slowly contracting fibers of the spastic
be attributed to mechanical changes in the muscle adductor muscles demonstrate hypertrophy, whereas
rather than to increased stretch reflexes from spasticity. the fast fibers show atrophy. Surprisingly, there has
These muscles were also seen to be abnormally stiff be’m no evidence of increase in endomysial or
that is, they produced more force for a given length perimysial connective tissue at any age of child,
change than did muscles in non-disabled children regardless of the clinical picture. The authors con-
(Tardieu et al., 1982). The most accurate term for this cluded that joint restrictions are attributable to the
impairment is hypoextensibility. The muscle offers atrophy of muscle fibers, which makes the muscle less
resistance to passive stretching at a shorter length than elastic and extensible, and possibly to an increase of
that expected in a normal muscle. In addition, if greater fibrous tissue in the periarticular structures, although
than normal amounts of force are required to produce a the latter was not verified.
change in length, the muscle is said to have increased There are no universally accepted methods of
stiffness. This is represented as the passive tension measuring spasticity (Katz & Rvmer, 1989), although
curve for CP (pCP) in Figure 20-lA, in contrast to the techniques include measurement of forces in response
normal passive tension curve (p,N) in Figure 20-iD, to standard passive stretches (tonic) or standard
when one moves the ankle from a position of plantar hammer stimuli (phasic), Hoffmann’s reflex recording
flexion to one of dorsiflexion. When a clinician finds (Jones & Mullev, 1982), and measurement of responses
that it is not possible to manually stretch the muscle to sinusoidal cycling or ramp stretches (Lin et al.,
through a normal range using reasonable amounts of 1994; Price et al., 1991). The modified Ashworth scale,
manual force, the muscle group is deemed to have a though commonly used in clinical situations, is really
contraçtur e, represented in Figure 20-2 as an undifferentiated measure of spasticity and
“Contracture,” the difference between the joint angle at extensibility. It has been shown to be reliable in adults
which this extreme resistance is encountered in the CP with neurolngic conditions, although reliability has not
muscle and that of the normal muscle. been established for children (Bohannon & Smith,
Figure 20-1 shows hypothetical active force- 1987).
length characteristics of spastic plantar flexors (a,CP;
see Fig. 20-1B) and normal plantar flexors (a,N; see MULTISYSTEM IMPAIRMENTS
Fig. 20-1E), that is, the force generated by the con-
tractile elements of the muscle over the range of In the second group of impairments are three
muscle lengths from a shortened position (plantar multisystem impairments expressed in. the neuro-
flexed) to a longer position (dorsiflexed). Note that the muscular system: poor selective control of muscle
maximal force is lower for the CP muscle and also that activity, poor regulation of activity in muscle groups in
the peak force occurs at a more plantarflexed position anticipation of postural changes and body movement
in the CP muscle than in the normal muscle. The sum (referred to as anticipatory regulation), and decreased
of the combined effects of active force output and ability to learn unique movements.
passive stiffness for the CP muscle is shown as total In CP there is poor selective control of muscle
tension curve CP, and the corresponding curve for the activity. Normal movement is characterized by orderly
normal muscle is shown as total tension curve N (see phasing in and out of muscle activation, coactivation of
Fig. 20-2). The complexity of the representation in muscles with similar biomechanical functions, and
Figure 20-2 underlines the difficulty faced by a limited coactivation of antagonists during phasic or
physical therapist or physician in correctly assessing free movement. In CP there is abundant evidence of
the cause of increased tone through clinical methods inappropriate sequencing (Nashner et al., 1983) and
such as passive manipulation of the limb and clinical coactivation of svner~ists and antagonists (Knutsson &
assessment of muscle strength. Martensson, 1980).
If a muscle complex has become
'

FIGURE 20-1. Representation of force capabilities of ankle plantar flexor muscle at different joint angles in normal
muscle (N) and spastic muscle (CP). A. Resistance to passive stretch of spastic muscle (p,CP) increasing with more
dorsiflexion. B, The force of active contraction (a/ZP) varying with the joint angle, l denoting resting length. C, The
sum of the passive and active effects in spastic muscle. D, Resistance to passive stretch in normal muscle (p,N). F,
Force of active contraction in normal muscle (a,N). F, The total tension curve comprising the sum of the passive and
active effects in normal muscle. Note that 1) the slope (i.e., the stiffness) of p,CP in A is greater for the spastic muscle
than for the normal muscles (p,N) in D; 2) the maximal active force achieved by the spastic muscle (a,CP) in B is less
than the maximal active force of normal muscle (a,N) in F; and 3) the maximal active force for spastic muscle (a,CP)
shown in B occurs at a more plantar-flexed position than that of the normal muscle (a,N) shown in F.

The reasons for poor selective control of antagonist muscles in children with CP (Leonard et al.,
muscle activity are unknown. Failure of the normal 1991) has been attributed either to exuberant
recipro Cal relationship of activity between agonist and motoneuronal projections or to exuberant projections
antagonist muscles during voluntary movements has that extend to motoneurons innervating muscles other
been observed (Berger et aL, 1982; Hallett & Alvarez, than the one being stimulated. From these studies, it
1983; Leonard et al., 1990), but whether segmental-or appears certain that the neuronal “wiring” in CP is not
supraspinal mechanisms or both are involved is un- normal.
clear. Although Berbrayer and Ashby (1990) clearly Poor anticipatory regulation of muscle
demonstrated the presence of reciprocal inhibition in sequencing when postural correction is attempted has
CP, it is not possible to exclude the possibility that been reported by Nashner and colleagues (1983). In
other spinal mechanisms may’ be impaired (Harrison, healthy individuals, changes in posture are preceded by
1988). Direct evidence for a supraspinal origin is scant; preparatory muscle contractions that stabilize the body.
however, researchers have concluded that in CP, the In people with CP, the contraction that is needed to
corticospinal projections are directed equally to the produce stability is frequently interrupted by
motoneurons of agonist and antagonist muscles of the destabilizing synergistic or antagonistic muscle
ankle (Brouwer & Ashby, 1991). Reflex overflow to activity.
FIGURE 20-2. Complete representation of forces capabilities of ankle plantar flexor muscle at different joint angle in
normal muscle (N) and spastic muscle (CP) shown in Figure 20-1. a, CP = Force of active contraction of spastic
muscle, 10, CP = resting length of spatic muscle, 10, N = resting length of normal muscle, an,N = force of active
cobtraction of normal muscle, p, CP = resistence to passive stretch of spastic muscle, p,N = resistance to passsiva
stretch of normal muscle.

There is some evidence that motor memory in potential for its wide clinical application has increased
children with CP is frequently impaired (Lesny et al., with the advent of fast and efficient computer systems.
1990). This finding is important in considering strat-
egies for teaching movement, but it has received little
attention to date. Determinants of Prognosis or Outcome.
Assessment of multisystem impairments
usually involves measurement of a closely associated About 90% of children with CP in developed
variable or number of variables and frequently involves countries survive to adulthood (Evans et al., 1990).
different dimensions of the disabling process. Strauss and Shavelle (1998) found that the key
Examination of the impairments of poor selective predictors of a reduced life expectancy were lack of
control of muscle activity, poor anticipatory regulation mobility and feeding difficulties. Survival of high
of muscle groups, and decreased ability to learn unique functioning adults was found to be close to that of the
movements includes use of measures of balance, general population, but predictions of lifetime
coordination, and motor control. Most are not in gen- functional outcomes in CP are limited. A California
eral clinical use. Two approaches to assessing balance study (Anonymous, 1991) reported that only 12 to 17%
are available: one is to disturb the supporting surface in of people with CP registered with developmental
a variety of ways (Nashner et al., 1983); the other is to services were competitively employed. Positive
perturb the subject or environment (Patla et al., 1989). prognostic factors for employment included mild
In each case, kinematic, kinetic, and EMG responses physical involvement, good family support, vocatioaal
are measured. Coordination has been documented with training, and having good employment contacts.
EMG records, which makes it possible to detect Mental retardation, seizures, and wheelchair
differences from normal records in timing of muscle dependency were factors reducing the likelihood of
activity onset and duration, in sequencing of agonists, living independently. Senft and colleagues (1990)
and in cocontraction of antagonists. Gait has been the reported that more than 60% of registrants in a
most commonly observed activity used to examine neuromuscular disability program were dependent on
specific impairments of CP (Perry et al., 1976), and the
aging parents. In a review of the literature, Bleck goals for clients with CP should focus on the preven-
(1987) included the following positive predictors of tion of disability by minimizing the effects of func-
independence and employment of a person with CP: tional limitations and impairments, preventing or
regular schooling, completion of secondary schooling, limiting secondary impairments, maximizing the gross
independence in mobility with the ability to travel motor functions allowed by the organic deficits, and
beyond the home, good hand skills, living in a small helping the child compensate for functions when
rather than a large community, and having a diagnosis necessary. Achieving these goals involves the
of spasticity rather than one of involuntary movements. promotion and maintenance of musculoskeletal in-
Preliminary studies of life satisfaction suggest more tegrity, the prevention of secondary impairment and
positive outcomes, but few studies have included this deformity, the enhancement of optimal postures and
important variable (Wacker et al., 1983). movement to promote functional independence, and
Certain factors assist in predicting the ambula- optimal levels of fitness.
tion potential of children with CP. Children with the The presence of impairments, such as low
hemiplegic type of CP usually have a good prognosis levels of force generation, spasticity, abnormal
for ambulation, whereas the prognosis is less favorable extensibility, and disturbed reflexes, can result in
for those with rigid or hypotonic types of CP (Crothers abnormal weight bearing and malalignment, which can,
& Paine, 1988). Persistent tonic neck reflexes are in turn, affect the orthopedic development of the spine
associated with decreased likelihood of walking and the extremities. The application of correct forces is
(Crothers & Paine, 1988). Some studies have reported required for optimal skeletal modeling before the skel-
that a remarkably large percentage of children who are eton ossifies (LeVeau & Bernhardt, 1984), although the
able to sit independently by age 24 months eventually research reported to date has offered little specific
walk (Crothers & Paine, 1988) and that nearly all guidance. Of particular concern is the effect of
children with CP who ev~mtually walk do so before 8 increased hip flexion and adduction on acetabular
years of age (Bleck, 1975). Watt and colleagues development and hip joint stability. Neck and trunk
(1989), examining all survivors of neonatal intensive asymmetry can result in torticollis or spinal deformi-
care, have reported that nearly all who sat by 24 ties. At all ages, children with hypoextensibility and
months of age walked 15 meters or more with or spasticity are prone to developing contractures. Al-
without assistive devices or orthos~s by age 8 years. though patt2rns of tightness vary, commonly at risk for
Independent sitting by 24 months remains the best contractures are the shoulder adductors; the elbow,
predictor of am: bulation, despite inclusion of neonatal wrist, and finger flexors; the hip flexors and adductors;
variables, clinical types, primitive reflexes, and the knee flexors; and the ankle plantar flexors
reactions (Watt et al., 1989). (Massagli, 1991).
Furthermore, the physical therapist attempts to
prevent environmental deprivation that could increase
EXAMINATION, EVALUATION, AND existing disabilities and attempts to provide support,
INTERVENTION guidance, and education for the child, the family, and
the community. Goals are individualized for the
At all ages, the physical therapy examination particular child and family. They should be determined
of the child with CP will focus on the identification of in collaboration with the family and based on the
disabilities, functional limitations, and impairments. In needs, expectations, and values of the whole family
addition, physical therapy examination is used to (Rosenbaum et aL, 1998). Goal and outcome
measure change resulting from intervention at all levels attainment should be regularly reassessed so that the
of the disabling process and provide feedback to therapy plan is adapted to reflect changes in the child’s
clients. progress and the family’s needs. An important
Physical therapists integrate information from component of therapy programs is education of the
the many aspects of their examination and evaluation child and family about the disability to enable them to
with prognostic knowledge to predict the optimal level become capable of advocating and baking
of improvement that can be expected. They then responsibility for their future.
develop a plan of care that includes long-term and The involvement of other health care
short-term goals and outcomes, specific interventions, professionals in the treatment of the infant with CP
and duration and frequency of intervention required to depends on the child’s needs and the practices of the
reach the goals and outcomes. institution where the program occurs. Some facilities
From infancy to adulthood, physical therapy may have professionals from several disciplines
working with the family, whereas at others it may be
thought better to have a primary therapist initially, maturation, and treatment effects. Therapists must
bringing in others for assessment or treatment as determine the history, living environment, and social
necessary. Regardless of practice approach, parents supports of an infant and the knowledge level and
value coordination of care and consistency of service concerns of the family. Examination of impairment
providers. involves qualitative and, when possible, quantitative
Increasing emphasis on the costs of provision evaluation of the single system and multisystem
of services and managed care have led some impairments. Observation of active range of motion
institutions to develop critical paths. This is a difficult (ROM) provides indirect assessment of the force-
task for CP due to the diversity of presentation and the generating ability of muscle groups and some
chronic nature of the condition. An example of an information about muscle extensibility. Determination
outline of care for CP is the document Cerebral Palsy- of the passive ROM, using a slow, maintained stretch
Critical Elements of Care which was developed by the in a position that promotes relaxation, assesses muscle
Washington State Department of Health (1997). group extensibility and provides information about
joints, such as the presence of dislocation. Normal
maturational changes in joint range and alignment must
Infancy be considered in evaluating the significance of
measures.
The life role of an infant is to grow and develop in Passive movement performed with greater
response to being loved and nurtured by parents and velocity is used to assess spasticity and the sensitivity
caregivers in a home environment. Despite being of the stretch reflex. Spasticity can be documented de-
dependent in most aspects of life, infants interact with, scriptively on the basis of resistance to movement and
and develop an understanding of, the people in their observations of spontaneous active movement and
lives, their surroundings, and’ themselves. From the posturing. The severity of spasticity-whether it is mild,
time of birth, a child with CP may not experience the moderate, or severe-its distribution over the body and
normal activities associated with infancy. As a result, limbs, and its variations under different conditions
the parents of an infant with CP may not receive the should be noted. Frequently, there are variations in
positive feedback of a normal nurturing experience and spasticity associated with positioning and the infant’s
the satisfaction of observing the development of motor effort and behavior. The modified Ashworth scale
and social skills, that is the normal rewards of caring (Bohannon & Smith, 1987) or the muscle tone section
for an infant. The parents must cope with the impact of of the MAI can be used (Chandler et al., 1980).
the diagnosis and the grieving process that The presence or persistence of primitive
accompanies the awareness that their expectations of reflexes and the development of the postural reactions
having a normal child will not be realized. They may of equilibrium, righting, and protective extension are
be overwhelmed with the uncertainty that the future assessed to determine their influence on selective
holds for them, their child, and their family. Many control and anticipatory regulation of muscle group
parents are also concerned with the immediate issues of activity. The effects these reflexes and postural reac-
providing basic infant care and are apprehensive about tions have on positioning, handling, and the facilitation
incorporating the specialized care necessary for their or inhibition of functional movement also need to be
child’s optimal development. evaluated (Bly, 1991). The primitive reflex and the
Movement is ~n important component in the automatic reaction sections of the MAI (Chandler et
learning and interactive processes of infancy. In infants al., 1980) are appropriate to use when evaluating
who have CP, the nature and extent of their infants with CP.
impairments affect their potential to develop and learn Selective control and anticipatory regulation
through movement. This may result in functional of muscle groups are assessed in the context of func-
limitations in the development of gross motor skills tional evaluation: for the infant, this is indicated by the
and may affect their ability to interact with their assessment of gross motor skills. Standardized tests
parents, themselves, and their environment. used by physical therapists when assessing infant
movement include the MAI (Chandler & Harris, 1985),
Physical Therapy Examination and the Gross Motor Function Measure (Russell et al.,
1989), the Peabody Developmental Motor Scales
Evaluation (Palisano etal., 1995), the Bayley Scales of Infant
Development (Bayley, 1993), the Test of Infant Motor
Infant examination provides a baseline for the Performance (Murney & Campbell, 1998), and the
monitoring of improvement or deterioration, growth, Alberta Infant Motor Scale (Piper & Darrah, 1994).
Various elements of movements and posture combine themselves. Although it is recognized that parents
to produce functional gross motor skills. These include know their children best, at this stage, the parents’
the ability to align one part of the body on another: to goals may be overly optimistic and hopeful. Therapists
bear weight through different parts of the body; to shift must be realistic about the prognosis and the efficacy
weight; to move against gravity; to assume, maintain, of physical therapy while remaining hopeful and
and move into and out of different positions; and to providing options for intervention. They can break
perform graded, isolated, and variable movements with down overall goals into objectives that are meaningful,
an appropriate degree of effort. When examining func- obtainable, sequential, observable, and measurable
tional motor skills, proficiency in incorporating these (Kolobe, 1992).
elements into the achievement of purposeful and
efficient movement must be evaluated.
Specific assessments of seating, feeding HANDLING AND CARE
(Evans Morris & Dunn Klein. 1987), or respiratory
problems may be necessary for infants with problems Abnormal postures and movements resulting
in these areas. Growth is often affected in children with from impairments can make an infant difficult to
CP; therefore, anthropometric measures, including handle and potition. These difficulties can affect an
head circumference, weight, and length, should be infant’s interaction with the environment, reaction to
documented. Growth may influence, or be influenced caregiving activities, and development of gross motor
by, feeding, exercise, and energy efficiency (Campbell skills. Therefore, a second physical therapy goal is to
et al., 1989). Other factors to be considered during as- promote the parents’ skill, ease, and confidence in
sessment include the influence of an infant’s temper- handling and caring for their infant. These skills
ament and behavior on performance; sensory, social, alleviate unnecessary stress for parents and child and
communication, and cognitive abilities; and support also help reduce the influence of the impairments,
from the environment. thereby preventing unnecessary secondary impairments
and limitations. Parents are taught positioning,
carrying, feeding, and dressing techniques that promote
Physical Therapy Goals, Outcomes, and symmetry, limit abnormal posturing and movement,
Intervention and facilitate functional motor activity. The principles
guiding these methods are 1) to use a variety of
Physical therapy in infancy is focused on movements and postures to promote sensory variety, 2)
educating the family, facilitating caregiving, and to frequently include positions that promote the full
promoting optimal sensorimotor experiences and skills. lengthening of spastic or hypoextensible muscles, and
Intervention must address current and potential 3) to use positions that promote functional voluntary
problems. Early intervention for children with CP has movement of limbs.
been advocated to help infants organize potential
abilities in the most normal way for them, although
there is no definitive support for its efficacy (Barry, FACILITATING OPTIMAL
1996; Campbell, 1990). SENSORIMOTOR DEVELOPMENT
A third physical therapy goal in infancy is to
FAMILY EDUCATION facilitate optimal sensorimotor experiences and skills,
thereby reducing functional limitations and disabilities.
The foremost set of goals at all ages is to Therapy should focus on the development of well-
educate families about CP, to provide support in their aligned postural stability coupled with smooth mobility
acceptance of their child’s problems, and to be of assis- to allow the emergence of motor skills such as
tance when parents make decisions about managing reaching, rolling, sitting, cu~w1ing, transitional
both their own and their child’s lives. Infancy is an movements, standing, and prewalking skills. These
important time to foster collaborative goal-setting and skills promote the development of spatial perception,
programming strategies with the parents and promote body awareness, and mobility to facilitate play, social
ongoing communication between families and service interaction, and exploration of the environment.
providers. These skills empower them to make Movements that include trunk rotation, dissociation of
decisions, solve problems, and set priorities, as well as body segments, weight shifting, weight bearing, and
to become effective advocates for their children and isolated movements should be incorporated into gross
motor exercises and activitie. These movement on fingers can promote sensorimotor awareness. Active
components, if experienced with proper alignment, can movements, such as the handling of toys that require
give the sensory feedback of normal movement two hands and that encourage the infant to develop
patterns and activities. Good sources for the handling flexor control and symmetry, are incorporated into
and treatment of infants and children of other ages daily activities. These activities facilitate the use of the
include the works of Finnie (1997), Jaeger (1987, neck and trunk muscles, promoting anterior and
1989), Scherzer and Tscharnuter (1990), and Wilson posterior control. The introduction of lateral control is
(1991). A practical reference for parents is Children the next step in achieving functional head and trunk
with Cerebral Palsy (Geralis, 1991). Careful instruction control. In some severely affected children, slight gains
of the family in specific techniques and activities, in head control may be a goal, whereas in minimally
ongoing reinforcement, encouragement, and support affected children a fairly normal progression of motor
are essential. Clearly written, illustrated, and updated development is expected, even without intervention.
home programs can be beneficial. Computer-generated These therapeutic interventions should not limit
programs or videotaping can be used to produce infants’ spontaneous desires to move and play and
personalized, effective, and efficient information explore their environments because even very young
regarding activities, positioning, and exercises. children need to be able to assert themselves and
The normal motor developmental sequence manipulate their world (Campbell, 1997).
may assist in guiding the progression of motor Some physical therapists may adhere to
activities, although research indicates that motor specific treatment philosophies, although differing
milestones and their components develop in treatment approaches often have underlying
overlapping sequences, with spurts of development similarities. Two approaches used with infants are
interspersed with some plateaus and even regressions neurodevelopmental treatment (NDT) and the Vojta
(Atwater, 1991). The child with CP does not always approach. NDT has been widely used throughout North
proceed along the normal developmental sequence, and America and other parts of the world as a basis for the
therapy becomes more functionally oriented within the treatment of infants with CP. NDT is based on the
scope of the child’s physical capabilities (Blv, 1991). theory that inhibiting or modifying impairments of
The stage at which this happens depends on the spasticity and abnormal reflex patterns can improve
severity of the impairments; in some children, it may movement. For infants, handling techniques encourage
occur early in life. active movement, and thus they experience normal
Activities or equipment may be used to allow movement sensations. The ultimate aim of the
attainment of functional skills when impairments treatment is the acquisition of functioiial movements
otherwise prevent the development of certain skills. that permit children the greatest degree of
For example, the sitting position promotes visual independence possible to prepare them for as normal
attending, upper extremity use, and social interaction. an adolescence and adult life as can he achieved
Infants with CP may be unable to sit independently, (Bobath & Bobath, 1984). The Vojta approach, a
may sit statically only with precarious balance, or may European-based practice, uses proprioceptive infor-
not even be able to be seated in commercially available mation from the trunk and extremities to activate the
infant equipment. Customized seating or adaptations to central nervous system and guide it toward normal
regular infant seats may be necessary to allow function motor ontogenesis by eliciting appropriate movement
in other areas of development to progress. Infants with patterns (Vojta, 1984).
limited upper extremity movement may be unable to
bring their hands or toys to their mouths to provide
normal oral-motor sensory input. In these cases, ROLE OF OTHER DISCIPLINES
mouthing activities should be incorporated into
therapy. Toys may need to be adapted to facilitate Occupational therapists may be involved in
developmental activities. upper extremity function, particularly as it relates to
The care of an infant exhibiting asymmetry, play. In addition, speech and language pathologists
extensor posturing, and shoulder retraction illustrates may be necessary if there are oral-motor problems
these approaches. Such an infant should be carried, interfering with feeding or early language
seated, and fed in a symmetric position that does not development. Community infant development workers
allow axial hyperextension and keeps the hips and may be involved in home-based programs. Social
knees flexed. Positioning of or playing with the upper workers may help the parents through the grieving
extremities to allow the infant to see his or her hands, process, explain programs, and direct them to
practice midline play, reach for his or her feet, or suck
appropriate resources. Likewise it may be helpful to and poor selective control affect the assessment. In
join parent support groups or meet with parents who such cases, muscle strength should continue to be
have been through similar experiences. considered in a functional context. Observing activities
such as moving between sitting and standing positions
or ascending and descending stairs assess both
Preschool Period concentric and eccentric power. Endurance should be
evaluated by observing the ability to walk age-
During the preschool years, locomotor, appropriate distances or propel a wheelchair a
cognitive. communication, fine motor, selfcare, and comparable span. During these years, quantitative
social abilities develop to promote functional indepen- measures of joint ROM and skeletal alignment, in-
dence in children. The process is a dynamic one in cluding the rotational and torsional alignment of the
which all these areas constantly interact with one pelvis and lower extremities (Cusick & Stuberg, 1992;
another. The child’s environment remains oriented see Chapter 15), should be documented using
toward the parents, family, and home ci ring this consistent and standardized procedures. Variations of
period, but he or she begins to interact with the outside 10 to 150 occur in intrarater goniometric measurement
world. Child care centers, babysitters, nursery schools, in children with CP (Stuberg er al., 1988), and caution
and playmates thus become part of a preschooler’s must be used to avoid misinterpreting small changes.
world. Noting the point at which initial resistance is met with
For children with CP, the limitations in motor passive range of motion is clinically relevant but
functioning may create disabilities in learning, so- difficult to accurately measure clinically.
cialization, and attainment of independence (Butler, Evaluation of function and disability are fre-
1991). Concerns of the parents include the impact of quently included in the same assessments. The Gross
impaired performance on all areas of development : for Motor Function Measure (Russell et al., 1989) and the
example, their child’s ability to participate in and Peabody Developmental Motor Scales (Palisano et al.,
become integrated into normal preschool activities. the 1995) can continue to be used to monitor the child’s
development of cognition and language, and the long- motor progress. When assessing motor skills, the use of
term effect of disabilities on future life and equipment to achieve an activity should be taken into
independence. consideration. For example, the use of orthoses in
During these years, the child’s attainable level ambulation may substantially affect walking abilities.
of motor skills can be predicted with a greater degree Function and disability assessment should also
of accuracy, as the influences of motor impairments on include mobility and transfers, communication, social
functioning become apparent. A major area of concern function, bowel and bladder control, self-care and the
for physical therapists is the child’s ability to achieve degree of reliance on caregivers, adaptive equipment,
independent mobility. In addition, skills in overall and environmental modifications in the performance of
gross motor development continue to be a focus of activities of daily living (ADL). The Pediatric
physical therapy to minimize disabilities, such as the Evaluation of Disability Inventory (Reid et al., 1993)
inability to learn and perform the selfcare skills of assesses many of these functional skills in young
toileting, dressing, grooming, and feeding, and the children. The Functional Independence Measure for
limitations in play, communication, social skills, and Children (WeeFIM), a pediatric version of the
problem-solving behavior. Functional Independence Measure (Msall et aL, 1990),
measures disability as quantified by burden of care.
Physical Therapy Examination and Other measures of ADL (Gowland et al., 1991) such as
the Vineland Adaptive Behavior Scales (Sparrow et al.,
Evaluation 1984) can be used. The Canadian Occupational
Performance Measure (Law et aL, 1990) can be used to
Assessment of disability assumes a primary ensure that goals are relevant to the family and to
focus, but it is important to determine the role of measure outcomes. Goal Attainment Scaling can be
function arid impairment in the production of used to evaluate whether specific individualized
disability. Tests should be administered at regular treatment goals or outcomes have been met, but this
intervals to document change that is due to treatment form of assessment cannot replace standardized
and/or maturation. Within the dimension of measures, particularly for research (Palisano, 1993).
impairment, direct testing of the forcegenerating ability Disability measures also include attempts to assesr
of muscle groups is not always appropriate because health-related quality of life. These measures take into
spasticity, abnormal extensibility, hyperactive reflexes, account age, specific disability, and the factors and
values believed to be important by health care selectivity and degree of sequencing of muscle-group
professionals, parents, and children themselves activity-all indicators of impairment. Upper extremity
(Rosenbaum et al., 1990). activities such as reaching (Kluzik et al., 1990) haVe
Asseisments specific to certain activities or also been studied using videotaping and other sensing
equipment may be indicated. These include evaluations systems.
of postural stability (Westcott et al., 1997), augmenta- When assessing children in this age group. it
tive communication, mobility, and gait (Olney et et al., is necessary to be aware of the effects of attention.
1990). Gait assessment measures ambulatory function, cooperation, and the children’s reaction to being
and if kinetic and EMG analyses are included, certain assessed on the evaluation process. Parents or other
impairments are also evaluated (Fig. 20-3). The ROM caregivers can provide information on whether a
of the hips, knees, and ankles in each phase of gait can child’s performance is characteristic of his or her
be observed using a videotape. Particular attention abilities.
should be paid to the propulsive movement of ankle Evaluation must take place at regular intervals
plantar flexion during push-off and to the concurrent to ensure that goals are still appropriate and therapy
hip flexion. These two events are responsible for much intervention is being appropriately directed.
of the forward movement of the body and are indirect
measures of force generation of muscle groups. EMG
recordings during walking show the general level of Physical Therapy Goals, Outcomes, and
activation of each muscle group, the degree of co- Intervention
contraction. and the
The impact and extent of the child’s
impairments become more established during the
preschool years. Treatment focused specifically on
reducing impairments and preventing secondary effects
of impairment provides a backdrop for interventions
aimed at higher levels of the disabling process to
prevent isolation from the typical experiences of early
childhood and family life. Optimal postural alignment
and movements of the body that are conducive to
musculoskeletal development, neurophvsioiogic
control, and function, through exercise, positioning.
and equipment, are the aims of many interventions. In
many cases, physical therapy goals ma serve as the
building blocks for global interdisciplinary goals in
communication, play, social interaction, and self-care
activities. Therapists must be willing to respect the
priorities of families and other professionals when
determining goals, because it may not be possible to
work on all areas at once. They must also be sure that
treatment is cQnducive to the goals chosen and is
motivating and fun for the child.

Figure 20-30 Cinild taking part in gait analysis REDUCING PRIMARY IMPAIRMENT
electromyography shows patterns of muscle activities AND PREVENTING SECONDARY
and aind indentification of the presence of co- IMPAIRMENT
contraction of muscle groups. Markers at joints allow
computer calculation of joint movements , force INGREASING FORGE GENERATION. Treatment
platforms embedded in floor permit measurement of to improve force generation of muscles in this age
individual muscle group contributions to the work of group is achieved through performing activities that
walking. (Courtest of Human Motion Laboratory create increased demands for production of both con-
School of Rehabilitation Therapy at Queen’s centric and eccentric muscie force. Such act~vtt1es
University, Kingston, Ontarion) include transitional movements, ball gx’mnastlcs.
games, and practice of functional skills such as using insurance. Targeted muscles are those in which
stairs (Stern & Steidle, 1994) spasticity interferes with function and those that are
SPASTICITY. Several options are available most prone to developing contractures. These include
for the management of spasticity. Interventions have the calf muscles, hamstrings, hip flexors, and
been directed toward decreasing the impairment ot adductors. Upper extremity muscles have also been
spasticity with the goals of prevention of secondary successfully injected. Botox injections can also be used
impairment, comfort and ease of positioning. and as a diagnostic measure before orthopedic or rhizotomy
improved functional movement. Decreasing spasticity surgery or as an analgesic agent to reduce pain and
during the preschool years allows muscle lengthening spasm postoperatively (Boyd & Graham, 1997). In-
and growth (Boyd & Graham, 1997; Rang, 1990) and jections in children with a dynamic component to calf
may delay or eliminate the need for orthopedic surgery. equinus were successful in improving passive
Two interventions most appropriate for the preschool dorsiflexion, which may allow more opportunity for an
ages are selective dorsal rhizotomies and botulinum increase in muscle length. The results were comparable
toxin A injections. These interventions are used if with but longer lasting than those of a control group
spasticity is interfering with function and conversely that received serial casting and also demonstrated
are not used if a child appears to be dependent on fewer side effects (Corry et al., 1998).
spasticity for function. Ideal candidates have fair to Therapy focusing on functional outcomes, but
good trunk control and selective muscle control; good also emphasizing muscle strengthening, is necessary
cognitive abilities, motivation, and parental support after the spasticity intervention for optimal effective-
that are conducive to intensive postoperative therapy ness, because the children’s muscles are “weakened”
programs; and no fixed contractures or deformity. without their spasticity (Fig. 20-4).
Severely affected patients, such as children with spastic HYPOEXTENSIBILITY. Various approaches
quadriplegia, may be appropriate candidates; however, are used to maintain muscle extensibility and joint
in these cases the goals are improved positioning, care, mobility. Some therapists use manual stretching
and comfort (McDonald, 1991). Selective dorsal programs. The usefulness of these passive maneuvers
rhizotomy is a surgical procedure in which the dorsal is difficult to assess because active exercises,
nerve rootlets supplying the lower extremity muscles positioning programs, and equipment are usually used
are selectively cut. Prospective randomized controlled simultaneously. Research on the effectiveness of
clinical trials have found spasticity to be substantially manual stretching on extensibility is inconclusive
reduced. Function, as measured by the Gross Motor (Miedaner & Renander, 1987). Tremblav and
Performance Measure, has been shown to be improved colleagues (1990) found that a prolonged stretch of 30
in children who have received rhizotomies compared minutes to the plantar flexors of children with CP
with those receiving equivalent physical therapy in reduced the impairment of spasticity and improved the
some (Steinbok et al., 1997; Wright et al., 1998) but voluntary activation of the plantar flexors but not the
not in another (McLaughlin et al., 1998). dorsiflexors. The effect lasted for as long as 35
Gait analysis in children who have had minutes. In a parallel study, the stretching session did
rhizotomies has shown improved sagittal motion at the not produce a functional improvement in gait (Richards
hip, knee, and ankle; however, abnormalities in et al., 1991).
patterns of muscle activation have persisted (Giuliani, The effects of prolonged stretching programs
1991). This is attributed to continuing problems with have been studied (Tardieu et al., 1988), and it was
motor control, which prohibit the proper sequencing of found that contractures were prevented if the plan-tar
muscle action. Some gait improvements have been flexor muscles were stretched beyond a minimum
found to remain 10 years after surgery (Subramanian et threshold length for at least 6 hours during daily
al., 1998). Other positive effects that have been noted activity. The threshold length was the length at which
include improved oral-motor control, increased voice the muscle began to resist a stretch. The data
volume and endurance, improved temperament and prompting this statement are suggestive rather than
concentration, improved bowel and bladder control, conclusive, however. Lespargot and colleagues (1994)
and improvement in growth parameters (McDonald, found that physiotherapy and a moderate stretch
1991). imposed for 6 hours daily prevented muscle-body
Injections of small quantities of botulinum contracture but did not prevent shortening of the
toxin A into muscles can prevent the presynaptic tendon.
release of acetylcholine at the nerve-muscle junction. CASTING AND ORTHOSES. Plaster or
The effect peaks at 2 weeks and may last for 1 to 4 fiberglass casting has been used as an economical
months. The drug is expensive but is often covered by method of providing stretch and is commonly used in
serial casting to lengthen hypoextensible calf muscles. (Carmick, 1995; Middleton et aL, 1988). Hainsworth
Casting for a 3-week period was shown to be effective and colleagues (1997) found that that the range of
if the hypoextensibility was due to imbalance between movement and gait deteriorated during the short
the triceps surae and dorsiflexor muscles but not if the periods without AFOs when compared with periods
primary impairment was lack of appropriate muscle during which the AEOs were worn. Foot orthoses, or
growth in response to bone growth (Tardieu et al., supramalleolar orthoses, may be used for children with
1982). Serial casting has also been used for calf muscle pronation who do not require the ankle stabilization of
and other muscle groups, such as the hamstrings and an AFO (Knutsson & Clark, 1991). Supramalleolar
elbow flexors. orthoses, however, may not improve ankle motion in
the sagittal plane (Carlsun et al., 1997). Another variant
is the posterior leaf spring orthosis, which is intended
to prevent excessive equinus while mechanically
augmenting push-off. A kinetic gait analysis of 31
children found that it reduced equinus in swing, per-
mitted ankle dorsiflexion in stance, absorbed more
energy during midstance, but reduced the desirable
power-generating capabilities at push-off (Oonpuu et
al., 1996).
Bivalved casts or therapist-fabricated splints
have been used in place of AFOs as a less expensive
alternative for children who are growing quickly, do
not have access to funding, or require a period of
evaluation. The bivalved casts, popular during the
1980s, incorporate design features such as toe exten-
sion support, which is purported to inhibit abnormal
tone or reflex activity. Although clinicians have
FIGURE 20-4. Exercises after rhizotomy are claimed that the splints reduce abnormal tone, improve
frequently directed toward increasing force generation positioning, and reduce unwanted reflexes, research has
of etensor muscles. not substantiated the claims (Carlson, 1984).
Orthoses have also been used during sleep to
Lower extremity orthoses are used to reduce prevent the secondary impairment of hypoextensibility,
impairment, prevent secondary impairment, and facili- or contracture. Baumann and Zumstein (1985) found
tate function. The specific goals are prevention of that the use of double-shell foot orthoses as night
contracture and deformity, provision of optimal joint splints from age 3 years to the end of the skeletal
alignment, provision of selective motion restriction, growth period prevented calf muscle contractures from
protection of weak muscles, control of tone and tonus- developing and made the need for surgery rare.
related deviations, enhancement of function, and Other materials such as Lycra (Blair et al.,
postoperative protection of tissues (Cusick, 1990). 1995), neoprene, and tape have been used for splinting
Ricks and Eilert (1993) found that although casts and to assist children biomechanically and facilitate func-
orthoses improved ambulation and preambulation tion. Caution must be taken concerning the skin
skills, x-ray’s did not show significant changes in the tolerance of these materials.
bony alignment of the foot and ankle during weight ORTHOPEDIC SURGERY
bearing. Orthopedic surgery in preschoolers is usually
Many variations of ankle-foot orthoses performed to prevent secondary impairment by limiting
(AFOs) are available, depending on the biomechanical the effects, but not the causes, of hypoextensibility and
and functional needs of the individual child (Knutsson spasticity. For example, the lengthening of
& Clark, 1991). Solid AFOs are used if restriction of hypoextensible or spastic hip adductors (or both) may
ankle movement is desired. Children who would be performed to prevent subluxation or dislocation of
benefit from freedom of movement at the joint can use the hip joint. Sometimes, however, surgery such as
hinged AFOs. Hinged AFOs frequently prevent plantar tendo Achillis lengthening is delayed because of the
flexion but permit dorsiflexion, which allows stretching tendency for recurrence necessitating repeated surgery
of the plantar flexor muscle group during walking. (Tardieu et al., 1982). Ideally, surgery is deferred until
Hinged AFOs have been found to promote a more age 6 to 8 years, when multilevel corrections can be
normal and efficient gait pattern than do rigid orthotics performed if necessary (DeLuca, 1996). Massagli
(1991) has emphasized that musculoskeletal surgery mineralization in children with hemiplegic CP con-
does not alter the neurologically driven patterns of cluded that bone cize and density decrease with in-
muscle activity, although lengthening, releasing, or creasing neurologic involvement, and weight bearing
transferring a muscle can alter its influence. Decreased may slightly lessen the effect (Lin & Henderson,
force production is often a complication. Orthopedic 1996). Optimally, standing involves movement and
surgery is sometimes combined with neurectomy if activity to provide intermittent loading and muscle
tonic activity of the muscle is present as a result of strain. Standing programs are often started at 1 year of
spasticity or other neurologic causes. A full discussion age if children are not able to bear their weight
of the role of orthopedic surgery in CP can be found in effectively on their own. Stuberg (1992) recommended
the work of Rang (1990). Physical therapists play positioning instanding for 45 minutes two or three
important roles in surgical decision making. They are times a day to control lower extremity flexor
also involved in the care of the child who is contractures, and for 60 minutes four or five times per
immobilized and in providing postoperative therapy week to facilitate bone development, but notes that
(Harryman, 1992), particularly because decreased force there is no definite evidence to support these
production is a significant complication of surgery. guidelines. Maintenance of the child’s ability to bear
Frequently forgotten is the importance of transporting full weight through the-legs reduces the need to be
children safely in motor vehicles when they are in lifted by caregivers.
casts, an activity for which various devices are
available (Bull et al., 1989).
POSITIONING. Alignment of the body as a TREATMENT OF FUNCTIONAL
whole is important. Children should have a variety of LIMITATIONS
posit tions in which they can optimally function, travel,
and sleep. Varying the positions of children who are Physical therapy for treatment of functional
limited in movement also helps prevent the secondary limitations is often intensive during the preschool
impairments of positional contractures and deformity, years. The frequendy of treatment varies, depending on
as well as skin breakdown (Healy et al., 1997). the resources available, complementary programming,
Decreased ability to change body position during sleep client goals, parental needs and desires, and the child’s
can cause disrupted rest for children with CP (Kctagal -response to treatment. Optimal treatment frequency is
et al., 1994). unknown, but periods of increased frequencies have
Position changes can also contribute to pulmo- shown improvements in attainment of specific
nary health. Severely involved children are at risk for treatment goals at levels that were maintained when
chest complications because of chest wall biome- frequency decreased, provided the skills were
chanics, feeding difficulties, immobility, and poor incorporated into daily functional activities. Bower and
coughing abilities. Adaptive seating has also been McLellan (1992) found that bursts of intensive
shown to improve pulmonary functioning (Nwaobi & physiotherapy directed at achievable specific
Smith, 1986). For the preschooler, sitting, standing, measurable goals accelerated the acquisition of motor
lying, and a position suitable for playing on the floor skills compared with conventional physical therapy.
are important. When prescribing seating systems, it is Therapy should be challenging and
necessary to be aware of not only the child’s comfort meaningful to the child and progress to integrating the
and functional abilities but also the caregivers’ skills learned into functional and cognitively directed
concerns and needs and the child’s environment. skills for carryover. Movement tasks should be goal
Seating inserts can be used in a variety of situations oriented and interesting to maintain motivation and
and with equipment such as strollers and wheelchairs, arousal. For example. kicking a soccer ball may be a
which are often needed to enable parents to transport more functional and motivating method of developing
their child easily. Specific suggestions are included in balance skills than practicing standing on one foot.
Chapter 24. Approved car seats and restraints are ChIldren with CP are able to perform concrete
necessary for safe and comfortable vehicular perceptuomotor tasks much more readily than abstract
transportation (Shaw, 1987). ones, even if the same movements are involved (van
Positioning in standing is thought to reduce or der Weel et al., 1991), because more information is
prevent secondary impairments by maintaining lower available from the environment to direct the task.
extremity muscle extensibility, maintaining or Motor control and motor-learning principles
increasing bone mineral density, and promoting op- (see Chapter 6) can be used to develop treatment strate-
timal musculoskeletal development (Stuberg, 1992), gies for reducing functional limitations. Feedback is
including acetabular development. A study of bone
important in the process of learning skilled movement. the family (Rempel et al., 1988).
Feedback through the child’s sensory receptors Drooling, a significant problem in about 10%
provides intrinsic information, whereas extrinsic of children with CP, can cause social embarrassment
feedback through various forms of biofeedback and affect the quality of social integration (Blasco et
provides information from external sources. Knowl- al., 1992). It can result from dysfunctional oral-motor
edge of results contributes information about move- activity, oral sensory problems or inefficient and
ment outcome, and knowledge of performance supplies infrequent swallowing. Management may include
feedback about the nature of the movement (Poole, waiting for further neurologic maturation, feeding and
1991). oral stimulation programs, behavior modification
Peedforward mechanisms must also be programs, medications, or surgery.
considered, because there is a cognitive component to Failure to develop an appropriate toileting
movement skills. In some instances, cognitive strate- routine during the preschool years can result in an
gies may be able to compensate for some of the ongoing disability, because incontinence can provoke
inherent motor limitations. negative reactions from caregivers and peers. Expec-
Many children with CP do not have normal tations of toileting in children with CP should be
cognition and behavior, and activities must be adapted similar to those for children of comparable cognitive
accordingly. If a child is unable to learn, training using abilities, and therapists should encourage training at a
memorization of solutions may be necessary, although comparable age and recommend appropriate adaptive
limited transfer to novel situations will occur (Higgins, equipment as necessary (Shaw, 1990).
1991). If behavioral factors are negatively affecting
treatment, a behavioral approach using appropriate
motivators may encourage children to work on certain MOBILITY
skills (Horton & Taylor, 1989).
Improvements in functional movement of the Ambulation is a major concern of physical
preschool-age child are made by reducing the effects of therapists during these years. Emphasis in treatment is
the multisystem impairments of selective control, initially on prewalking skills, such as attaining effec-
anticipatory regulation, and learning of unique tive and well-aligned weight bearing, promoting dis-
movements. Although there is a growing literature on sociation and weight shifting, and improving balance.
motor learning (see Chapter 6), on motor control in Ambulatory aids, such as walkers and crutches, may be
skill acquisition (see Chapter 2), and on the bio- used, either temporarily while the child is progressing
mechanics of movement, the profession is still far from to more advanced gait skills or as long-term aids for
being able to provide optimal strategies for treatment independent mobility. The use of posterior walkers has
that are known to be effective (Fetters, 1991). The been found to encourage a more upright posture during
therapist who treats children with CP should modify gait and to promote better gait characteristics than does
approaches as research produces new insights in the the use of anterior walkers (Fig. 20-5) (Logan et al.,
areas of motor learning and motor control. 1990).
FEEDING, DROOLING, AND TOILETING. Children in this age group are becoming
Some children with CP, particularly those who are aware of the concept of achievement, and although
severely affected, may have oral-motor problems, such ambulation is a coveted skill, it should not become an
as poor mouth closure, retraction or thrusting of the allconsuming goal, particularly if it may not be attain-
tongue, poor tongue movements, and poor coordination able. When interviewing adults with significant
in swallowing, which can make speech and feeding impairment, Kibele (1989) found that they remembered
difficult. Feeding problems can be aggravated by other walking as the most important goal set for them by
problems such as impaired self-feeding and difficulties their parents and therapists. This resulted in feelings of
in expressing hunger or food preferences that may failure from an early age and also in a loss of faith in
result in inadequate nutritional intake and poor growth rehabilitation professionals.
(Gisel & Patrick, 1988; Reilly & Skuse, 1992). The provision of alternative means to allow
Gastroesophageal reflux and aspiration also occur in children functional, independent mobility when ambu-
children with severe CP. Oral-motor programs, proper lation is impossible or inefficient is recommended.
positioning, and parent education and support are Sometimes this need is met with an adapted tricycle
important issues to address (Evans Morris & Dunn (Fig. 20-6) or manual wheelchair; other children re-
Klein, 1987). In extreme cases, gastrostomy and quire one of a wide variety of power mobility devices
antireflux procedures may be necessary to improve available (Jones, 1990) and may need special controls
growth and enhance the quality of life for the child and
(Fig. 20-7). These enable children with CP to explore indication of the implications of power mobility on
their environment and achieve a sense of independence housing and transportation needs. For more
and competence. Power mobility may also promote the information, see Chapter 24.
development of initiative (Butler, 1991) and the
acquisition of spatial concepts. The lack of self-
propelled locomotion can result in apathy, withdrawal, PLAY
passivity, and dependent behavior that can persist into Play, the primary productive activity for
later life (Butler, 1991). children, should be intrinsically motivating and
pleasurable. The benefits of play include the children’s
discovering the effects they can have on objects and
people in their environment; developing social skills;
and promoting the development of perceptual,
conceptual, intellectual, and language skills.
Limitations in the play of children with physical
disabilities may affect their experiential learning
derived from play and result in decreased
independence, motivation, imagination, creativity,
assertiveness, social skills, and selfesteem (Blanche,
1997; Missiuna & Pollock, 1991). Therapy should
provide and demonstrate play opportunities (Fig. 20-8).
Appropriate toys and play methods should be
suggested to parents and caregivers. If children are
physically unable to play with regular toys, a variety of
adaptations, such as switch accessing, can make their
toys usable (Langley, 1990). Environmental control
equipment also can be introduced to preschoolers.

FIGURE 20-5. Child using a posterior walker,


reported to promote upright posture and higher walking
speeds than an anterior walker

If power mobility is being considered, fine


motor control, cognitive abilities, behavior,
environment. visual and auditory abilities, and
financial resources must all be taken into account.
Children with motor limitations have become safely
and effectively mobile in power wheelchairs at as
young as 17 months of age (Butler, 1991). Parents may
initially be hesitant about introducing power mobility
to young children, fearing that it signifies giving up on
walking. Power mobility does not preclude ambulation-
oriented therapy but provides the child with a method
of moving about independently in the meantime. For FIGURE 20-6 An Adapted tricycle may meet a child
children who will continue to be wholly dependent on need for mobility.
power mobility for independence, it provides mobility
at an appropriate age and gives the families an
INTERVENTION APPROACHES
NOT is a treatment approach that is used with
the age group and that continues to evolve with
advances in the field (Bly, 1991). Research studies on
the effectiveness of NDT are available but controver~
sial and reflect the difficulty in conducting clinical
research (lThiisano, 1991). However, NDT strategies
incorporated into treatment have been shown to im-
prove control of functional movements (Kluzik et al..
1990). Strategies such as practice may need to be
integrated into NDT for optimal im2rovement in
specific skills (Jonsdottir or al., 1997).
Conductive Education (CE), an approach
originated by Andras Peto in Hungary, is based on the
FIGURE 20-7 Single – swoith scanning computer theory that the difficulties of the child with motor dvsfu
controller for power wheelchair permith indenoendent n tion are problems of learning. The goal of treatment
mobility for children with limited motor control. is the ability to function independently in the world
Developed at Hugh MacMillan Rehabilitation Centre, without aids, an important objective in Hungary, where
Toronto, Ontario schools are not adapted for children with disabilities.
Participants are selected for their abilify to learn, which
It is important that children are not overpro- may make some children ir~eligible for the program.
tected in their attempts to play. Parents should be They are usually treated in group settings that provide
encouraged to let their children enjoy typical play the incentive of competition and allow more time in
actisis such as rolling down hills and getting dirtx’ in therapy than does individual treatment. Functional
the ttttid. Therapists must ensure that therapy and home goals are broken down into small steps. Children
programs promote, rather than interfere with, the initiate the activities on their own, with direct
novtmtl play experiences parents have with their conscious action aided by mental preparation (Bairstow
children. et al., 1991). Research on CE is limited; however, a
study by Reddihough and colleagues (1997) found that
children in a CE program made progress similar to that
of children involved in neurodevelopmental programs.
Knowledge of a variety of theoretic models,
not necessarily pediatric in origin, can help form a
therapist’s approach to treating children with cerebral
palsy. Cart and Shepherd’s task-related movement
science-based approach (Shepherd, 1995), the motor
control theories of Shumway-Cook and Woollacott
(1995), and the dynamic systems theory (Darrah &
Bartlett, 1995) can be integrated into physical therapy
interventions for children with CP. The family-centered
service philosophy of service provision can be applied
to all treatment approaches.
It is difficult to compare the effectiveness of
different approaches because there are also discrepan-
cies in parameters such as frequency, skill level of
individual therapists, compliance of families, and age
and abilities of the patients. In a study comparing four
treatment approaches, Bower and colleagues (1996)
found that parents were most pleased with therapy
Figure 20-8 Therapeutic exercise programs can include when they requested the services, when they were
highly motivaTING PLAY ACTIVITIES. Thorowing a present during treatment, and when targeted goals were
basketball may be more motivating than trunk met. They concluded that the most appropriate
extension exercises.
approach for a child would be one that would meet the ROLE OF OTHER DISCIPLINES
needs of the particular child. Parents of children with
CP often consider alternative therapies that may not be Occupational therapists work closely with the
accepted by some professionals and are usually children at this age to develop independence in activ-
expensive. If families choose to take an approach other ities such as dressing, feeding, toileting, and playing.
than the one offered by the therapist, it is necessary to Speech and language pathologists continue to develop
respect their choice. Reasons for seeking alternatives efficient methods of communication in children with
include a desire for more therapy, dissatisfaction with CP. Psychologists assess cognitive skills and advise on
present therapy, and belief that their child could do the interaction of intellectual abilities with the other
better (Milo-Manson et al., 1997). Therapists should areas of development. Social workers and behavior
not react defensively but should provide impartial therapists continue to provide ongoing support to the
information about the therapies in question. families, because the stresses involved in parenting a
child with a disability persist (Sternisha et aL, 1992).
Team assessment and intervention are imperative for
FAMILY INVOLVEMENT addressing issues such as feeding problems,
augmentative communication, and transition to school.
The importance of providing family-centered
setvice continues. Planning of interventions should take
into account the child within the context of the family. School-Age and Adolescent Period
Therapists should be sensitive to the family’s stresses,
dynamics, child-rearing practices, coping mechanisms, During the school-age and adolescent years,
privacy, values, and cultural variations. Therapists children typically become more involved in school and
should be flexible in their approach and programming community life while remaining dependent on their
(Kolobe, 1992). Special efforts must be made to deal families and living in their parental homes. They refine
with the effects of a child with CP on the siblings and augment the basic functional skills they
(Powell & Ogle, 1985). have learned and develop life skills that will enable
Family involvement is crucial for integrating them to cope effectively with the demands of daily
treatment into everyday life. Home programs are im- living and, it is hoped, independent adult life.
portant for optimal results from therapy programs These can be difficult years for children with
because strengthening, extensibility, and motor CP. They become more aware of the reality, extent, and
learning often require more input than can be provided impact of their disabilities on themselves and their
by limited treatment resources. It is necessary to find a families. As they strive to contend with the normal
balance between providing parents with home stresses of growing up, particularly those of adoles-
programs that make them an integral part of their cence, they must also cope with being different, ac-
child’s therapy and burdening them with activities they knowledge the potential obstacles to attaining inde-
cannot realistically be expected to carry out. Obstacles pendence, and work to overcome them.
may includ&constraints on time, energy, skills, or Parents remain anxious about how their childs
resources or negative effects on the parent-child disabilities will affect their participation in educational
relationship. Hinojosa and Anderson (1991) found that environments and in social situations. They may worry
although mothers of preschoolers with CP did not carry about their child’s future as an adult (Hallum, 1995).
out specific home programs designed by therapists, While continuing to be naturally attentive, they have to
they did activities that could be integrated into their avoid being overprotective and begin to allow their
daily routines and interactions and that were not child to take risks and become independent in the
stressful for the child or the caregiver. outside world. In some cases, there may be financial
Siblings can be included in home programs. concerns regarding the need for special equipment,
Craft and colleagues (1990) studied a group of children transportation, and home renovations. Parents of
with CP whose siblings had been educated about the children who are dependent in ADL and transfers may
condition and ways in which they could encourage suffer from physical stresses, such as back problems.
their brother or sister to be more independent. As a Typical disabilities encountered during these
result of this, the children with CP showed increases in years include a lack of independent mobility, poor
ROM and in functional independence. endurance in performing routine activities, and con-
tinued difficulty and slowness with self-care and hy-
giene skills at a time when privacy is becoming in-
creasingly important. Adolescents may also not have years when children are becoming more aware of their
the opportunity to develop socially and sexually and bodies and their sexuality. Children should be
may lack the ability to acquire age-appropriate levels of appropriately dressed when attending therapy sessions
independence from the family. Societal barriers may and clinics, particularly if they are being seen by
reduce access to community and school facilities, thus unfamiliar people or are being photographed or
limiting opportunities for participation in social, videotaped. If it is necessary for children to remove
cultural, and athletic activities. their clothing, their permission should be asked and a
reason given for doing so. It is important to include the
clients in conversations that involve them and not to
Physkal Therapy Examination and converse only with caregivers or other professional
Evaluation staff.

Assessment of impairments that could


interfere with function, or lead to further secondary Physical Therapy Goals, Outcomes, and
impairment, such as scoliosis, continues to he Interventions
important. Children in this age group may be able to
participate in measurement of force production using As goals become oriented toward the child’s
dynamometry. Assessment of gross motor function is or adolescent’s lifestyle, emphasis is on maintaining or
appropriate, because children are often making gross improving the level of function while considering the
motor progress during their school years. They may stresses of growth, maturation, and increastna demands
also change as a result of interventions such as surgery, in life skills and participation in communitiy activities.
the use of orthoses, and periods of intensive therapy. Although the pathophysiologic impairment of CP is
The development of the Gross Motor Performance nonprogressive, there are changes related to the stresses
Measure (Boyce et al., 1995) may help in detecting the of increasing size, accumulative physical overuse, and
fine increments of motor skill gains that are a more competitive lifestyle. For example, as children
characteristic of children who are severely affected. For advance to higher grades in school, there may be
children with mild degrees of involvement, the longer distances to walk between classes. Contractures
Bruininks-Oseretsky Test of Motor Proficiency may rapidly develop during periods when boEes are
(Bruininks, 1978) may be useful in measuring specific growing faster than muscles (Tardieu et al., 1982).
components of higher levels of motor function. Tools Secondary orthopedic and functional problems must be
such as the School Function Assessment may be used anticipated and avoided. The maintenance of muscle
in educational settings (Coster et al., 1994). extensibility and force generation, joint integrity, and
Gait analysis continues to be helpful in fitness is important in preventing secondary
assessing ambulatory children, particularly when impairments that can result from the stresses of aging.
decisions about surgery are being made (Lee et al., This age group also needs to develop problem-solving
1992), although this opinion is not held by all (Abel, strategies to overcome environmental and societal
1995). Endurance and efficiency of movement become barriers to become as independent and active as
increasingly important during these years as the chil- possible in home, school, recreational, social, and
dren venture into the community on their own or with community life.
their peers. Mechanical energy costs can be assessed In many cases, adolescents can and should be
with motion analysis equipment (Olney’ et al., 1987). involved in setting goals and determining program-
In the clinical setting, physiologic measures such as the ming. It is important during these years to encourage
physiologic cost index for activities such as ambulation them to take responsibility for their own health, care,
can give energy cost estimates (Butler, 1991; Mossberg and decision making so that they are prepared to
et al., 1990) or assist in selecting assistive devices assume these responsibilities in adulthood (Fox et al.,
(Rose et al., 1985). In a laboratory setting, ergometer 1992). It is also important to look ahead and set goals
assessment can analyze other aspects of endurance that are appropriate for their later life situations and
(Parker et al., 1992). An important consideration in independence. Therapists should strive to foster self-
maximizing endurance for daily activities is the effect esteem and assertiveness in children and adolescents by
of excessive weight gain, because it can compromise emphasizing their abilities, finding areas and activities
optimal function and efficiency. in which they can excel, and helping them to
Privacy of individuals at all ages should be re- acknowledge their difficulties with a view toward
spected, but it is particularly important during these identifying appropriate compensations and use of
attendant care. In the case of children with severe appropriateness and monitored closely for side effects.
(multiple) disorder, goals are oriented toward Baclofen, a synthetic agonist of aminobutyric acid, has
minimizing impairments to facilitate caregiving and an inhibitory effect on the presynaptic excitatory
comfort. neurotransmitter release. It has been shown to reduce
spasticity in individuals with CP (Campbell et al.,
1995). If taken orally, doses high enough to give the
REDUCING PRIMARY IMPAIRMENT proper concentration in the cerebrospinal fluid can
AND PREVENTING SECONDARY cause side effects such as drowsiness. To circumvent
this problem, baclofen is most effective if given
IMPAIRMENT intrathecally by’ a continuous infusion pump implanted
in the abdomen that releases the drug at a slow,
The impairment of deficient muscle force constant rate into the subarachnoid space. The use of
generation that may result in functional limitations and intrathecal baclofen has been postulated to reduce the
disabilities is important to address in this age group. need for orthopedic surgery (Gerszten et al., 1998).
Damiano and coworkers (1995) used ankle weights in a Biofeedback is a useful tool in addressing
6-week progressive resistive strengthening program for single-system and multisystem impairments during
the quadriceps in 14 children with spastic diplegia. these years (Fig. 20-9) because by this time children
Stride length increased, and knee flexion at initial have usually developed abstract thinking and sufficient
contact decreased. An 8-week isokinetic strengthening cognitive ability to use it optimally. Positive results
program for mildly affected adolescents effected have been reported, but carryover is often limited,
strength, gains of 12 to 30% in knee extensors and generalization to real-life situations is not readily
flexors but no increase in spasticitv (MacPhail & demonstrated, and treat~rient is time consuming
Kramer, 1995). Nine of the seventeen subjects showed (James, 1992). Toner and colleagues (1998) found that
improvements in the standing and walking, running, a program of biofeedback improved active range of
and jumping dimensions of the Gross Motor Function motion and strength and motor control in dorsiflexion
Measure. Using free weights in a home program in a group of children with CP.
supervised by’ a family member, Damiano and Abel The secondary impairment ofjoint or muscle
1998) reported average strength gains of 69% when the contracture may occur in this age group, particularly in
two weakest muscle groups were trained in children the more severely affected patients. This can be a result
with spastic diplegia or hemiplegia. The gains were of chronic muscle imbalance, abnormal posturing, or
associated with positive functional outcomes, although, static positioning. Casting may be used to increase the
as with the MacPhail and Kramer study, it is not clear range of joint movement by lengthening muscles or
whether increasing strength alters gait efficiency. tendons or both with nc associated loss in strength
Various methods of electrical stimulation have (Brouwer et al., 1998; Cusick, 1990; O’Dwyer et al.,
been used as an adjunct to the treatment of CP to 1989; Tardieu et al., 1982). Interventions of botulinum
reduce spasticity, to increase force production and toxin A and casting may complement each other in
extensibility, and to improve function activities such as children with spasticity and contractures. Anderson and
gait (Stanger & Bertoti, 1997). This treatment adjunct colleagues (1988) found that soft splints made of
is particularly useful in postoperative muscle training polyurethane foam were effective in reducing severe
and strengthening. Protocols should be individualized knee flexor contractures; this may be an attractive
for patients following careful analysis of movement, alternative for low-income families.
and application should be closely monitored. Orthopedic surgery continues to be used to re-
It was once thought that individuals had no duce, the effects of primary and secondary impairment
control of their spasticity. Subjects with CP, however, on mobilirv, posture, cosmesis, and hygiene and to
have been able to reduce their responsiveness to a prevent further secondary impairment. Possible
stretch reflex stimulus imposed during a lower limb surgical procedures include muscle lengthenings or
activity (O’Dwyer et al., 1994). These findings en- transfers, tenotomies, neurectomies, osteotomies. and
courage further exploration of the possibilities of fusions.
reducing spastic responses. Surgery may improve posture and gait, but
Pharmacologic intervention can be used to outcomes can be unpredictable because muscle weak-
control the impairment of spasticity. The use of oral ness often results. As children get older, they should be
medications has been poorlystudied, but medication active participants in surgical decision making that may
may be appropriate for some children (Pranzatelli, he influenced by their interests, priorities. and concerns
1996). Children must be carefully assessed for about interruptions in their lives as result of
hospitalizations, immobilizations, and recovery wheelchair or a power scooter, as they become larger
periods. and need to travel greater distances to meet their social
The secondary impairment of joint and educational objectives. Ambulant children with
hypomobility resulting from capsular or ligamentous cerebral palsy are less active than their peers (van den
tightness can be treated with manual therapy Berg-Emons et al., 1995). Availability of power
techniques (Brooks-Scott, 1995). Joint mobilizations mobility should not preclude activity to the point of
may be used to regain joint mobility, particularly after decreased musculoskeletal integrity or physical fitness.
immobilization. Therapists must ascertain whether Mobility devices may require modifications, such as
joint structures are causing movement restriction and ramps to buildings or washroom renovations, for
must also be aware of the contraindications and accessibility’. Dnve r training offers the freedom to
precautions relevant to using mobilizations in growing travel independen:Iy. For those unable to drive,
and neurologically involved patients (Harris & instruction in the use of public or special transportation
Lundgren, 1991). should be provided.
Spasticity, abnormal extensibility of muscles, The functional limitation of low endnrance
muscular imbalance, and decreased force generation levels, frequently related to inefficiency of movement,
can resulr in scoliosis, which, in turn, can affect has been identified in children with CP. Rose and col-
positioning and respiratory status. Spinal deformity can leagues (1990) found chat energy expenditure indices
be a particularly difficult problem in the severely based on oxygen uptake and heart rate measured at a
affected patient. Rigid orthoses can result in skin given walking speed were 3 times higher in children
breakdown arid patient intolerance; by contrast, the use with CP than in normal control children. Findings that
of a soft orthosis has been found to be beneficial in the were similar, but based on mechanical energy analyses,
management of scoliosis in patients with CP (Letts et were reported for children with hemiplegia (Olney et
al, 1992). In some-ases, surgical intervention may be al., 1987). Rose and colleagues (1985) also found that
necessary (see Chapter 10). children who were ambulatory with wheeled walkers or
quadruped canes had high physiologic wotkloads when
walking for 5 minutes, suggesting that it is impractical
for such children to walk long distances. Orthoses may
also influence energy expenditure. Mossberg and
colleagues (1990) showed that the use of AFOs
significantly reduced the energy demands of walking in
children with spastic diplegia.

FIGURE 20-9. Biofeedback is a useful teatment tool


when precise objectives can be indentified

MOBILITY AND ENDURANCE


Many compensatory strategies can be imple-
mented to promote function and circumvei~t disability FIGURE 20-10 Computer-based communication
during the school years. The continued use of po’ver system with keyboard on computer screen and head
mobility devices is important for independent mobility. pointer acting as controller. Developed at Hugh
Often children who are able to walk independently MacMillan Rehabilitation Centre, Toront, Ontario
need an alternative form of mobility, such as a manual
SCHOOL AND COMMUNITY sports (Jones, 1988).
All athletes are at risk for sports-related
Many children are involved in school based injuries, but relatively minor injuries can incapacitate
programs, particularly with continuing application of people with CP. They should be encouraged to be
the Education for All Handicapped Children Act in responsible for their bodies during sports activities by
1975 in the United States (Poirier et al., 1988) and the following appropriate conditioning, warm-up, and
Charter of Rights and Freedoms in Canada in 1982 cool-down routines; following comprehensive injury
(Poirier et al., 1988). In most jurisdictions, the prevention programs, which include strengthening,
educational program must be implemented in the least flexibility, and aerobic and anaerobic training activi-
restrictive environment for that child, and “the child. ties; and using appropriate protective and orthotic
mainstreamed to the fullest possible extent” (Clune & equipment. Injuries should be treated promptly. The
Van Pelt, 1985). Facilities and resources such as knee is the most frequently injured body part in athletes
support personnel, equipment for accessibilityi, and with CP. Shoulders, hands, and ankles are also
computer-based systems (Fig. 20-10) are necessary to vulnerable (Ferrara et al., 1992). For more information,
meet the physical needs of children in the school see Chapter 17.
system. Therapists working with school personnel may During these years, children learn about their
instruct assistanrs and teachers in positioning, lifting, bodies, their sexuality, and appropriate interactions
and transferring the children; carrying out exercise with other people. Children and adults with disabilities
programs; and adapting and developing physical have an increased risk of suffering abuse, including
education programs (Wilcox, 1988). Therapists may sexual abuse, which can result in physical, social,
also be involved in accessibility, transportation, emotional, and behavioral consequences (Hallum,
evacuation, and other safety issues. Therapists working 1995; Sobsey & Doe, 1991). Many of the perpetrators
in school settings must be sensitive to the physical and have relationships with the victims that are similar to
scheduling constraints of the educational environment those commonly found among nondisabled victims;
and be willing to compromise to meet the educational however, some abusers have relationships specifically
priorities of the students. Therapy may range from related to the victim’s disability. These people can be
consultation and monitoring for students who are personal care, attendants, transportation providers,
thought to have reached their maximal level of residential care staffi and other disabled individuals.
functioning, to active therapy for children who have Abuse is a serious matter that must be guarded against
specific treatment goals. When children are primarily at all times. Physical therapists must know how to
seen through the educational system, effort must be detect the signs of abuse, be sensitive and receptive to
made to keep the family involved in all aspects of care clients who may choose to confide in them, and know
and treatment. See Chapter 32 for further information the proper procedures to follow if they suspect abuse.
on this subject based on experiences in the United They must work with other professionals to promote
States. assertiveness and positive self-esteem in their clients.
In addition to educating school staff about All professionals involved with patients who
children with CP, therapists can help educate the other have CP must educate them in being streetwise. Their
children in the classroom. Knowledge ahout, and physical and sometimes cognitive limitations can make
positive interactions with, people with disabilities can them particularly vulnerable to crime. Children and
create positive attitudes, particularly when children are adolescents with disabilities should be taught to avoid
between 7 and 9 years of age, because their attitudes threatening situations. They should be warned about
about people with disabilities are flexible at this age carrying valuables (large sums of money or important
(Morrison & Ursprung, 1987). medications) with them, particularly in a purse that
It is important for children with disabilities to attracts attention and is easy to grab) such as one slung
be involved in community and recreational activities over a wheelchair handle. Attendance at self-defense
that provide social as well as therapeutic opportunities. courses should be encouraged; some that are
Many adapted or integrated sports activities are specifically designed for people with disabilities are
suitable for people with CP, including horseback riding becoming available.
(MacKinnon et al., 1995), swimming, skiing, sailing, Health care professionals must realize that al-
canoeing, camping, kayaking, fishing, bun-gee though parents have been coping with their children’s
jumping, yoga, and tai chi. Adapted games can provide needs for a number of years, parent education is still
athletic competition and participation in team important: their children and their needs are constantly
experiences and can facilitate the social aspects of changing. Continued attention to education in lifting
and transferring is necessary to prevent injury to
caregivers as their children grow larger and heavier and the family, and the health care team to provide
they themselves are aging. comprehensive planning to ease the transition to
adulthood.

ROLE OF OTHER DISCIPLINES


Physical Therapy Examination and
Occupational therapists may be involved in pro- Evaluation
moting independence in ADL. Multidisciplinary life-
skills training may be offered to focus on self-care, Although there is a continuing need to assess
community living, and interpersonal relationships. impairment, adult assessments focus on functicn and
Prevocational training and related activities, such as disability, particularly on the level of independent
money management and employment searching, may functioning. A variety of functional disability scales,
be necessary. Psychologists or social workers may be such as the Barthel Index, have been developed for
involved in social and sexual counseling. adults in general rather than for adults with a particular
disability (Mahoney & Barthel, 1965). These measure
performance in various self-care, independence. and
Transition to Adulthood mobility functions (Spector, 1990).

The life role of an adult is to be an


independently functioning and self-sufficient Physical Therapy Goals, Outcomes, and
individual who has a satisfying social and emotional Interventions
life and is a contributing member of society. The
natural environment for adults is living independently The major goal during this period of transition
in the community, alone or with others, with is to maximize the client’s capabilities to achieve opti-
employment to support them. mal independence and happiness as an adult. Ideally,
The extent to which people with CP can the medical, therapeutic, and educational goals of
realize these goals depends on factors such as level childhood have had this as a long-term goal in earlier
oficognition, available resources and support, and intervention. In a study of adults with CP health in
independence in self-care activities and mobility. Many terms of acute illnesses was not a problem, but 76%
adults with CP continue to live with their families, in had multiple musculoskeletal problems, and many
group homes, or in institutions, and a small proportion nonambulatory adults had urinary complaints.
of them are employed. In Finland, a study of young Equipment and therapy needs were poorly met
adults who had been followed since childhood revealed (Murphy et al., 1995). In many situations, pro-
that 31% of those older than 19 years were fessionals, the client, and the family are dealing with
competitively employed, but an additional 21% who external environmental forces that make it difficult for
were judged to be employable were unemployed a person to overcome disabilities.
(Sillanpaa et al., 1982). Finding employment can be
particularly difficult when there is considerable un-
employment in the able-bodied population. REDUCING PRIMARY IMPAIRMENT
At a time when most parents are experiencing
freedom from caregiving responsibilities, many parents AND PREVENTING SECONDARY
of children with CP continue to have these obligations IMPAIRMENT
and have to cope with many anxieties (Hallum, 1995).
Their concerns focus on how their child can function as Although there is a focus on overall disability,
an independent adult, how they can continue to care for therapists must still be cognizant of the impact of
their child as they themselves age, and who will care impairment on function. Adults with CP must deal with
for the child when they are unable to do so. They, and the normal effects of aging in addition to their existing
their child, may also be coping with a decrease in the impairments (Overeynder & Turk, 1998). Insufficient
numbei of relatively organized and available programs force generation and hypoextensibility can still respond
that were available for the younger child. to therapy. Although secondary impairments such as
Physical therapy involvement continues to ad- contracture may appear to be static, there can still b3
dress all levels of the disabling process; however, there deteriorarion, so momtoring and treatment, if
is an emphasis on working together with the individual, necessary, should be available. If significant deformity
occurs, aggressive salvage surgery may be necessary with disabilities. Theaters, restaurants, libraries, mu-
for comfort and ease of care. One example is proximal seums, government buildings, educational facilities,
femoral resection if a hip is painful or if hip mobility is shopping areas, parks, campground facilities, and
limited to the extent that sitting and perineal care are parking lots are becoming accessible, where possible,
affected; however, this surgery has a high complication through the provision of ramps, appropriate washroom
rate (Perlmutter, 1993). facilities, and other modifications. Air and rail travel is
Of particular importance is the prevention of also becoming more accessible to people with special
overuse syndromes, joint degeneration, progression of mobility needs, and there are now travel organizations
contractures, osteoporosis, poor endurance, and that cater to people with disabilities. In some situations,
pathologic fractures. Cervical and back pain, nerve funding for assistive devices, living allowances, and
entrapment syndromes, or tendinitis can occur as a housing and tax exemptions help prevent undue
result of excessive and repetitive physical stresses hardship. Therapists should be aware of the facilities
(Bergman, 1994; Murphy et al., 1995). Such injuries available to the disabled and the political policies and
should be treated with orthopedic therapy techniques, issues concerning the disabled and should advocate
as they would be in the general population. Preventive their advancement.
treatment to minimize the long-term effects of the
neuromuscular dysfunction may be beneficial. This
may include use of additional mobility aids or devices, TRANSITION PLANNING
orthoses, or surgery. Changes to the adult’s
environment may be necessary to maintain optimal Ideally, the planning for the transition to adult
independence (Overeynder & Turk, 1998). services and lifestyle takes place before the actual
major life changes. Areas to be addressed when plan-
ning for the transition include the following: vocational
LIFE SKILLS training or postsecondary educational placement,
which may range from higher education to supported
Ongoing involvement in fitness and work models; living arrangements (independent, with
recreational activities should be planned. This will family, institutional, or other supportive care); leisure
provide social opportunities, as well as maintain or and recreation (religious groups, community programs,
improve cardiovascular fitness, weight control, and and recreational centers); personal management,
integrity of joints and muscles; help prevent including birth control; social skills; and household
osteoporosis; and generally promote the optimal health management. The continuation of professional health
that contributes to independent functioning. Fitness services must also be dealt with (Fox et. al. 1992). This
clubs, swimming, wheelchair aerobics. and adapted includes the provision of therapy when needed,
sports are options. Endurance continues to be an impor- medical consultation, primary care, and equipment
tant concern. Fernandez and Pitetti (1993) found that needs and maintenance. Financial planning and
the values for physical work capacity of ambulatory education about budgeting, tax and other governmental
adults with CP were significantly lower than normal benefits, advocacy and legal services, guardianship,
values and concluded that adults with CP would conservatorship, wills, and trusts must be addressed
possibly experience fatigue before completing a normal (Hallum, 1995).
workday. However, physical work capacity and work-
related activities improved with training.
Technology is providing adults with CP many RESEARCH NEEDS
options that were not previously available. These
include computers for communication, artificial speech Research in CP is urgently needed. The
devices, environmental control devices, and mobility selection of treatment for an individual with CP
devices. For more information, see Chapter 24. requires predictive information about the effects of
Society is becoming more conscious of the interventions, if any, on the pathophysiology,
rights and needs of the disabled (Bickenbach, 1993). impairments, functional limitations, and disabilities of
Human rights legislation now exists to accommodate the person throughout the life span, yet even
people with disabilities and to prevent discrimination descriptive information is limited. To take a simple
against them in areas such as employment, example, to decide whether orthoses are appropriate for
accessibiLty, the legal system, and education. Govern- a particular child, we should know whether the
ment programs and services are available to people impairment of hypoextensibility is preventable by
orthoses, as some studies suggest (Tardieu et al., 1988), PROFESSIONAL ISSUES
and, if so, under what conditions. Furthermore, we
should know if force generation capability is changed Therapists who care for children (vith CP
with orthotic wear, how force output changes with must realize that the work can be physically
growth, and what conditions favor successful longterm demanding. They must practice appropriate lifting and
outcomes. The multisystem impairments of poor handing precautions and should maintain a suitable
selective control of muscles, poor anticipatory level of fitness if they are actively treating patients.
regulation of movement, and decreased ability to learn Working with children and their families can also be
unique movements have received little attention. emotionally stressful. Therapists may often be
We need better information about the relation- challenged with ethical issues, unrealistic expectations
ships between measures at each level of the disabling and demands, limited resources, and the pressures of
process. This will facilitate specificity of treatment. dealing with families during the grieving period and
There is a need for research that predicts long-term other times of crisis. Therapists need to concentrate on
outcomes. Finally, there are needs for specific and what is positive and realize that they cannot control all
sensitive measurements of all dimensions of the dis- the variables in their patients’ conditions and lives.
abling process to be developed within a focused re- Professionals must acknowledge their own needs and
search program. reactions and feel comfortable in seeking assistance
and support from others.
This chapter concludes with two case histories
GLOBAL ISSUES that illustrate some of the management principles
discussed in this chapter.
The treatment of children with CP varies
throughout the world. Physical therapists use many
different approaches and combinations of approaches, CASE HISTORY
depending on the facilities available, the child’s and the JAMIE
family’s needs, the therapisty is training and
background, and the diversity of client values, beliefs, Jamie is an 8-year-old boy with spastic
and priorities. Many of the treatments and technologies diplegia and microcephaly. After a pregnancy
discussed are practiced in developed countries, where complicated by preeclamptic toxemia, labor began at
services, although variable in their extent, quality, and 37 weeks of gestation, and an emergency cesarean
funding, are available and accessible. However, much section became necessary when fetal distress occurred.
of the world’s population lives in underserviced areas, Treatment was begun in the hospital. On returning
particularly in developing nations or in remote areas of home with their child, the parents performed routine
developed nations. Often, many of the principles and stretches daily, and a therapist visited every other
equipment ideas developed elsewhere can be adapted week. A mothers’ support group met every 2 weeks,
to the various situations (Werner, 1987). Using which provided general information and guidance, as
indigenous materials to fabricate effective and well as support.
affordable equipment, recycling used equipment, and When Jamie was about 1.5 years of age, the
training local personnel or fostering exchange family moved to a town in another province where
programs can help provide resources to underserviced coordinated services were provided. jamie took part in
areas. physical therapy, occupational therapy, and speech
It is important to be sensitive to local customs, therapy and was assessed routinely by the psychologic
cultures, and environmental situations when adapting services. When he began attending day care, he
programs for different settings. Often, the direct received services from the same therapists, and the
application of a certain method is impractical or parents, teachers, and therapists worked together on his
inappropriate because of economic, geographic, or treatment.
cultural differences. There is an increasing emphasis on When Jamie was 3.5 years old, the
corñmunity-based rehabilitation, which promotes impairment of K poextensibility of the hamstrings and
interventions that are practical and functional for the heel cords was present. Functionally, he was able to
particular settings, lifestyles, and cultures. walk independently, although he fatigued easily. Toe-
walking was common and accompanied by slight knee
flexion and adduction and internal rotation of the hips.
His right limb showed more impairment than his left.
Bivalved short leg casts were worn for 2hours three dorsiflexion during late stance reached about 150. The
times a day, the goal being to prevent further knees on both sides were more extended at initial
hypoextensibility of the ankle plantar flexors. contact and extended more in late stance than was the
At age 4.5 years, jamie’s gait was examined in case before surgery. The push-off power of the right
the Motion Analysis Laboratory at the School of ankle was greater after surgery (A2-PF, Fig. 20-1 2)
Rehabilitation Therapy at Queen’s University in than before surgery, and right hip power was more
Kingston, Ontario. He walked at an average of 0.71 effective (H1-E, hip extensors in early stance; H3-F,
m/s, compared with-the normal value for his age of hip flexors at pull-off; Fig. 20-12). Average walking
about 1.00 in/s (Sutherland et al., 1980). joint velocity was 0.82 m/s.jamie was able to join the
excursions were reduced (Fig. 20-11). He walked with community soccer league the following summer.
marked plantar flex~on of the right foot and with slight Jamie attends school in a regular classroom,
flexion and reduced excursion of both knees, and he although he has some cognitive limitations. He is
had reduced power generation of the ankle plantar visited by a familiar therapist from the treatment center
flexors on both sides (A2-PF, Fig. 20-12), which was every 2 or 3 weeks and attends speech therapy weekly.
evidence of low force-generating capability, The main problem the parents have encountered within
hypoextensibility, or both. He was compensating for the school system is a practice of inflexibly classifying
these deficiencies with larger than normal pull-off by the children according to their chronologic age only.
hip flexors on the left side (H3-F, Fig. 20-12). The Although jamie is cognitively young and physically
family engaged jamie in an electrostimulation program small for his age, there has been considerable reluc-
from an outside clinic in tance to permit him to join younger groups, even for
which the quadriceps and ankle dorsiflexors were stim- sports. Outside of school, jamie takes part in a nj.imber
ulated overnight at a level below that required to of athletic activities. He is interested in team sports,
produce muscle contraction. During the next few and the family has been pleased with the efforts of
months, while electrostimulation and weekly therapy coaches and managers to include him in organized
were continued but the use of casts was not, jam ie’s teams.
ankle plantar flexors became much more hypoexten-
sible. The use of bivalved casts was reintroduced, and
the previous range was regained rapidly. Update: 5 years later
Clinical examination when jamie was 5.5 years old
revealed that his hip adductors, knee flexors, and ankle Jamie is now a happy and likeable 13-year-old about
plantar flexors were more hypoextensible; functionally, to face the challenges of adolescence. He is completing
he was \.‘alking on his toes with reduced excursions of his last year of school at the intermediate level and will
his ankles. Although a number of surgical alternatives be entering a public high school next year. The family
were considered, including rhizotomy, the family de- is very happy with his education to date, and is trying
cided on a lengthening of the right heel cord. The goals to help him prepare for the challenges of a large school
of gait reeducation were toward attaining initial contact in which the children are expected to be independent.
with the heel and an extended knee and achieving His cognitive impairments are more limiting than his
effective push-off with the ankle plantar flexors. His physical situation. Still small for his age, he has not
electrica stimulator was used in treatment postopera- been able to continue in team sports. His greatest joy is
tively to strengthen the ankle dorsiflexors and the karate, which he attends 3 days a week. He expects to
surgically weakened plantar flexors. Other treatment receive his violet belt soon. This activity has helped
goals and activities included climbing and descending him with selfdiscipline, balance, and coordination.
stairs with one foot per stair and stepping off a stair or jamie attends physical therapy about every 3 weeks,
curb without hand support. Because jamie had a keen where he concentrates on stretching and functional
interest in sports, movement components such as activities that are complementary to his karate practice.
weight shifting, balancing, and eye-hand-foot About once a year, the hypoextensibility of his left calf
coordination and skills such as running, starting and group is treated with about 3 weeks of serial casting.
stopping, changing direction, and throwing were
incorporated into sports activities such as baseball,
coccer, and badminton. The improvement noted at a 6-
month postsurgical gait analysis (see Fig. 20-11) was
more marked on the right side, but several
improvements were noted on the left. On the right side,
initial contact was made with the heel, and ankle
FIGURE 20-11. Plots of joint angles from gait analysis of one stride for Jamic, shown presurgically and postsurgically
and with normal values (dotted line). Each plot represents one stride beginning and ending with initial contact of the
foot on the floor. Plotted upward (positively) are hip flexion, knee flexion, and ankle dorsiflexion. Note limited knee
extension on both sides, extreme limitation of ankle dorsiflexion on the right, and normalization of postsurgical values.

FIGURE 20-12. Plots ofjoint power from gait analysis of one stride forJamie, shown presurgically and postsurgically
and with normal values (dotted line). Each plot represents one stride beginning and ending with initial contact of the
foot on the floor. Plotted upward (positively) are muscle power generations (concentric contractions), and downward
(negatively) are muscle power absorptions (eccentric contractions). Plots ofJamie’s right side show low force
generation of right hip extensors in early stance (H1-E), of hip flexors at pull-off (H3-F), and Gf ankle plantar flexors
(A2-PF) presurgically, and increases in all of these after surgery. Plots of Jamie’s left side show compensatory increase
in H3-F (pull-off of hip flexors) presurgically, and normalization after surgery.
CASE HISTORY optimally aligned.
NICOLE At 2 years of age, the impairments of adductor
hypcextensibility and spasticity were treated surgically
Nicole has moderate to sev ore spastic with bilateral adductor muscle releases and anterior
diplegia. She was born at 29.5 weeks of gestation after obturator neurectomies. The purpose of the surgery
placental separation. She weighed 1300 g and had an was to give her more functional motion at her hips and
Apgar score of 8 at 5 minutes. She was treated with to put her hip joints in an optimal position for acetab-
ventilatior for 8 hours and then.was wearied off the ular development; it was also intended to avoid the
ventilator onto continuous positive airway pressure, potential secondary impairment of hip subluxation or
and then to oxygen. She received phototherapy for 3 dislocation.
days for an increased bilirubin level and was During her preschool years, Nicole attended
administered theophyliine for apnea. Nicole remained an integrated child care program with government-
in the neonatal intensive care unit for 6 weeks and then subsidized funding for children with special needs.
went home to live with her parents and 3-year-old This setting allowed for integration with able-bodied
sister. children and also gave Nicole and her family
Nicole was followed up at the screening clinic opportunities to meet other children with special needs
for high-risk infants. At 3 months of age corrected for and their families. Nicole’s physical and occupational
prematurity, she exhibited extensor positioning of her therapists visited the center regularly to discuss
neck and trunk and hypertonicity was emerging in her Nicole’s abilities, programs, handling, and equipment.
legs, but her family did not have any concerns. At 4 Her resource teacher was invaluable in coordinating
months of corrected age, these findings were discussed care, supporting the family, and adapting or acquiring
with her mother, and positioning and handling recom- equipment.
mendations were made. At 5 months of corrected age, When Nicole was 5 years of age, she was
the diagnosis of CP was made on the basis of hyperto- progressing slowly in her gross and fine motor skills.
nicity in her extremities, affecting lower extremities However, spasticity in her lower extremities that was
more than upper; strong, persisting primitive reflex clinically apparent was affecting her ability to maintain
activity; and delayed development of head and trunk well-aligned postural stability, and she could not move
control. An ultrasound scan at this time showed the left easily using optimal patterns of movement. These re-
lateral ventricle to be slightly enlarged and the right strictions resulted in functional limitations in sitting,
lateral ventricle to be at the upper limits of normal in standing, transitional movements, fine motor activities,
size. The periventricular brain parenchyma appeared and ADL and limited her potential for independence.
normal. Ambulation was not functional, but she could move
Management by a developmental pediatrician, about independently in a power chair and had some
a social worker, an orthopedic surgeon, a physical limited mobility in a manual wheelchair. The
therapist, and later an occupational therapist was prominence of spasticity and the generally good force-
coordinated at the local children’s treatment center. generating capabilities of her musculature prompted
Nicole attended physical therapy sessions weekly. The the decision to have a selective dorsal rhizotomy.
basic therapy was based on NDT and encouraged After the rhizotomy, Nicole’s lower extremity
active control of movement and play. Positions that tone was greatly reduced, and Nicole participated in
reduced the influence of her extensor postuing were daily inpatient physical therapy and occupational
used to encourage active control of movements and therapy sessions for 8 weeks postoperatively and then
functional skills. Bivalved casts were introduced early had sessions twice a week for the next year (Figs. 20-
to maintain muscle extensibility and provide optimal 13 and 20-14).
alignment of her feet when she was working on A Gross Motor Function Measure evaluation
standing activities. The casts also reduced some of the was done preoperatively and 1 year after the surgery.
extensor posturing in her lower extremities, resulting in Her scores im~oved from 88 to 96% in lying and
improvements in her alignment in sitting and standing rolling; from 78 to 87% in sitting; from 19 to 57% in
and improvements in the quality of functional activities crawling and kneeling; from 13 to 32% in standing
in these positions. These casts were later replaced with (with AFOs); and from 7 to 10% in walking, running,
solid AFOs when growth slowed down, and Nicole was and jumping (with AFOs). She had been able to walk
eager to wear regular shoes. Customized seating and a 10 m at 0.04 m/s preoperatively but could walk 30 m at
standing frame gave her a variety of positions that 0.15 m/s at her 1-year follow-up. Although these
offered opportunities to interact with others and use her findings indicated that there had been improvements in
hands and provided some weight bearing with her body her gait, the distance and velocity of her walking were
still much below age norms and did not result in
functional ambulation. She had improved isolated
muscle control, which was demonstrated by improved
active ROM of her quadriceps, dorsiflexors, and
plantar flexors. Her passive ROM improved in her
hips, knees, and ankles, particularly in motions
involving the hamstring muscles. Nicole continues to
have decreased muscle force production, particularly in
her hip and knee extensor muscles (Fig. 20-15).

FIGURE 20-15. Despite many improvement after


posterior rhizotomy, Nicole still shows diminished
forcegenerating ability hip and knee muscles.
Nicole showed improvements in her self-care
skills, particularly in dressing her lower extremities,
because she was able to move one leg independently of
the other. On a modified Klein-Bell test of ADL, she
FIGURE 20-13 Mother and therapist Nocole, achieved 9/33 before surgery and 1 9/33 when tested 1
encouragaing force generation of trunk and hip year later. She was able to function better with her
extensors hands in activities such as opening jars, printing, and
propelling her manual wheelchair. The improvements
were believed to be the result of better trunk control
and co-contraction in the shoulder musculature rather
than of changes in the intrinsic muscles of her hands,
motor planning, or visuoperceptual skills-areas that
continue to be problems for Nicole. The Beery Devel-
opmental Test of visuomotor integration score was at
an age equivalency of 4.1 both preoperatively and
postoperatively.
Nicole now attended her neighborhood school.
Transportation was provided by the school board in a
school bus with a wheelchair lift so that she could
travel to school with her peers. The educational system
also provided a full-time educational assistant to help
Nicole with transfers, toileting, schoolwork, and exer-
cise and sports programs. She played with her friends
on the playground at lunch and recess in her power
chair. She used a typewriter to increase the speed at
which she could put information down on paper.
Nicole’s family has worked with medical.
FIGURE 20-14. Nicole, wearing ankle-foot arthoses, educational, governmental, and community
doing exercises at school organizations to gain access to services and programs
that provide her with normal childhood experiences
and minimize disabilities. Nicole has been involved
with horseback riding, swimming programs, and
adapted games. She rides in her family’s boat using a
Tumble Form seat, goes on an annual vacation to
Florida, and traveled to England to be a flower girl at
her aunt’s wedding. She attends her peers’ birthday
parties, where her friends or their parents help her as
necessary. She has modeled in fashion shows in her Update: 5 years later
wheelchair. Nicole has attended the community day
camp, Brownies, and the Easter Seals family camp in Nicole is now a warm and friendly 13-year-
the summer. old who will be entering high school next year. Her
Resources her family has found useful include parents have worked closely with teachers and
the Easter Seals Society; the Ontario government’s administrators of the school, where she has a half-day
As~istive Devices Program; parent support groups; and educational attendant who helps with her schoolwork
the Community and Social Services Respite Care and gives personal assistance. Her above average
Program, which provides funds for students to work knowledge of computer functions allows her to develop
with Nicole-arid provide parent relief. The Canadian more efficient ways of completing her school
railroad, VIA Rail, which allows wheelchair escorts to assignments and communicating with others. Nicole
ride free and offers reduced rates for Nicole, is useful uses a taxi fitted for wheelchair transport when she
for traveling to out-of-town appointments. travels by herself to school and to the shopping mall.
Nicole and her family face barriers. The She uses a power wheelchair in most places other than
family home is in the country and has a gravel her own home, where she uses a manual wheelchair.
driveway. The house is not wheelchair accessible For recreation, Nicole swims at a local pool, and, in the
because there are stairs into the house, stairs inside, and summer, attends a camp. Like many teenagers, she uses
narrow doorways. Thick carpeting makes manual a chat line on the Internet, and she speaks with her
wheelchair pushing and walker maneuvering difficult. friends on the telephone.
When Nicole visits friends, their homes have similar Because Nicole had received some
obstacles. Renovations have been made to the school professional services (notably the dorsal rhizotomy)
washroom, and equipment has been provided, but the from a nearby city, it was difft cult for the family to
entrance doors are too heavy to open, playground obtain a consensus of prognostic information that
equipment is inaccessible, and classroom door handles would help them make decisions about their home and
are difficult to turn. There is no public wheelchair lifestyle. Nicole’s mother arranged a telephone
transportation in their township community. Although conference between all of the relevant professionals in
obstacles are becoming less common as society the two cities and Nicole and herself which she felt was
becomes more aware of accommodations necessary for the single most important event in the past several
people with disabilities, barriers such as buildings years. As a result, Nicole’s family has built an addition
without ramps, inadequate parking, and inaccessible to their home, which makes the downstairs fully
washrooms still exist. accessible. It also has a large deck that allows Nicole to
Nicole’s parents have found regular family go outdoors on her own and gives a second entrance to
conferences invaluable in helping to facilitate effective the house. The driveway is paved to enable her to reach
family and school involvement in therapy programs. In her taxi or bus. She needs one-person assistance with
these conferences, all the team members meet to transfers, toileting, and dressing, but she showers and
discuss short- and long-term goals. Agendas and washes her own hair independently using a commode
minutes clarify the objectives of the meetings and chair in a wheel-in shower. Nicole eats independently.
document the decisions that are made. Such
conferences help empower parents and allow them to Nicole is seen by a physical therapist working
become effective advocates for their children. They in the school system about once a month, and she
also reinforce the philosophy of teamwork and attends a teen group for girls organized by the
partnership with the therapists and the treatment center. professionals of the Child Development Centre.
Nicole’s parents have found Craig Shield’s Stressing life skills, the meetings also provide
book Strategies: A Practical Guide for Dealing with opportunities for the teens to discuss items of mutual
Professionals and Human Service Systems to be interest. Her mother thinks physical therapy may be
helpful (Shields, 1987).They believe that home visits needed to maintain the range of moton in her
designed to deal with issues of daily routines, integrate hamstrings, which is needed for upright standing
treatment goals into home life, and involve all family during transfers.
members are important. Similarly, school visits are Nicole’s parents are very active advocates for
important. Nicole’s parents believe that it is changes that will provide people with disabilities with
particularly important not to withhold any information a full range of life’s opportunities. They believe parents
from the family. They also emphasize the need for must be prepared to play centrr!, responsible leadership
feedback and encouragement to the family, especially roles within groups and agencies that can assist in these
to the primary caregiver. endeavors.
ACKNOWLEDGMENTS Canada, MSB 1Y3.

Acknowledgments are extended to the Child


Development Centre of the Hotel Dieu Hospital, WEB SITES
Kingston, Ontario; the Motion Analysis Laboratory at
the School of Rehabilitation Therapy at Queen’s American Academy for Cerebra. Palss and Developmental
University, Kingston, Ontario; the Bloorview- Medicine:
http://aacpdm.org/
MacMillan Rehabilitation Centre, Toronto, On tario, United Cerebral Palsy’: http:// www.ucpa.org/
for assistance in providing data and photographs; and Ontario Federation for Cerebral Palsy
to the parents of Jamie and Nicole for their support of :http://www.ofcp.on.ca/
and contributions to the case histories.

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