Download Cerebral palsy` (CP) is the neurologic condition most frequently

CHARTER
20
spasticity management usually’ rely on input from
Cerebral Palsy
physical therapists. The therapist’s influence is not
SANDRA J. ONLNEY, BSc restricted
(P&OT),
Med,
PhDcenter and treatment
to the
medical
MARILYNJ. WRIGHT, BSCPT
NATURE AND CHARACTERISTICS OF
CEREBRAL PALSY
Classification, Etiology and Pathophysiology
Progress in Primary Prevention
Impairment
Determinants of Prognosis or Outcome
EXAMINATION,
INTERVENTION
Infancy
EVALUATIAB,
AND
Cerebral palsy’ (CP) is the neurologic
condition most frequently encountered by pediatric
physical therapists. Jr is a permanent but not
unchanging neurodevelopmental impairment caused by
a non progressive defect or lesion in single or multiple
locations in the immature brain. The defect or lesion
can occur in utero or during or shortly after birth and
produces motor impairment and possible sensory
deficits that are usually evident in early infancy
(Scherzer & Tscharnuter, 1990). CP involves one or
more limbs and frequently the trunk. It causes disturbances of voluntary motor function and produces a
variety of symptoms. Nevertheless, CP is itself an
artificial concept, comprising several causes and clinical syndromes that have been lumped together because
of a commonality of management. The impaired
control and coordination of voluntary muscles is
accompanied by mental retardation or learning
disabilities in 50 to 75% of children and by disorders of
speech (25%), auditory impairments (25%), seizure
disorders
(25-35%), or abnormalities of vision
(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
central role or have as much potential to influence the
outcome of children’s lives. Their interventions have
not only immediate but also lifelong effects, and can be
efficient and cost-effective. Treatment of children is
specialized: therapists provide services that will help
them reach their full potential in their homes and
communities. Furthermore, decisions a bout many
medical interventions such as orthopedic surgery and
Preschool Period
School-Age and Adolescent Period
Transition to Adulthood
RESEARCH NEED
GLOBAL ISSUES
PROFESSIONAL ISSUES
CADE HISTORIES
gymnasium, but frequently includes consultation regarding the child’s functioning in settings within the
home, school, recreation, and community environments. Good therapy not only helps the child with CP
but also can have a positive influence on the child’s
family and caregivers. In summary, parents of children
with disabilities want services that provide general and
specific information about their child, provide
coordinated and comprehensive care, and are provided
by’ respectful and supportive professionals (King et al.,
1998). The pediatric physical therapist is ideally’ suited
to fill these roles.
This chapter discusses the background of
cerebral palsy the classification, etiology, and
pathophysiology; prevention; associated impairments;
and determinants of outcomes. Physical therapy
examination, evaluation, and intervention that are
related to each age between infancy and adulthood are
discussed. The chapter concludes with two case
histories.
NATURE AND CHARACTERISTICS OF
CEREBRAL PALSY
Classification, Etiology,
and Pathophysiology
CP has been classified in a number of ways. A
classification based on the area of the body exhibiting
impairment yields the designations of monoplegia (one
limb), diplegia (lower limbs), hemiplegia (upper and
lower limbs on one side of the body), and quadriplegia
(all limbs). Another classification, based on the most
obvious movement abnormality resulting from
common brain lesions, yields spastic, dyskinetic, and
ataxic types. The spastic type, in which the muscles are
perceived as excessively stiff and taut, especially
during attempted movement, results from involvement
of the motor cortex or white matter projections to and
from cortical sensorimotot areas of the brain.
Involvement of the basal ganglia is reflected in
dyskinesia or athetosis and sometimes in intermittent
muscular tension of the extremities or trunk and
involuntary movement patterns. A cerebellar lesion
produces ataxia, or general instability of movement. A
hypotonic classification, not known to be related to a
particular lesion, is characterized by diminished resting
muscle tone and decreased ability to generate voluntary
muscle force. Symptoms of spasticity and dyskinesia
may both be present in a child, with the type of CP
referred to as mixed. The degree of severity of CP
varies greatly, and the designations mild, moderate,
and severe are often applied within types. The Gross
Motor Disability Classification System is a five-level,
age categorized system that places children with CP
into categories of severity that represent clinically
meaningful distinctions in motor function (Palisano et
al., 1997). Although the proportions of the various subtypes of CP vary with the reporting source, a series
from Sweden noted that hemiplegia accounted for
36.4%; diplegia, 41.5%; quadriplegia. 7.3%; dyskinesia
or athetosis, 10%; and ataxic forms, 5% (Hagberg et
al., 1989a).
CP is a condition with multiple causes leading
to damage within the central nervous system. Although
the causes are not completely understood, certain
prenatal, perihatal, and postnatal factors have been
associated with CP (Torfs et al., 1990). There is general agreement that the majority of cases of CP in term
infants are due to prenatal or unknown causes, whereas
in the vast majority of preterm infants the lesion
causing CP develops during the perinatal period.
Preterm birth, although not believed to be causative, is
associated with up to 33% of all cases, including more
than 50% of diplegia (Pharoah et aL, 1990), 25% of
hemiplegia (Uvebrandt, 1988), and 5% of quadriplegia
(Edebol-Tysk, 1989). Prenatal malnutrItion, intrinsic
developmental problems of the fetus, poor maternal
prenatal condition, and maternal infection are also
associated with CP (Menkes, 1990). Intracranial
hemorrhage, especially among premature infants, is a
wellestablished causal factor. Neonatal asphyxia is a
significant perinatal event, but only a small minority of
cases result from such events (Nelson & Ellenberg,
1986). Infection in the perinacal period is also
important. The effects of hvperbilirubinemia and other
blood incompatibilities frequently resulting in athetoid
CP are a concern, particularly in developing countries.
The statistics from developing countries are in
sharp contrast to those of developed countries. Studies
have suggested that up to 63%. of cases in the former
have preventable causes associated with shortage of
care personnel and inadequate financing for effective
services (Karumuna & Mgone, 1990; Nottidge &
Okogbo, 1991).
The dramatic decrease in perinatal mortality in
developed countries during the 1980s, largely a result
of improved survival rates for low birth weight infants,
has given rise to the fear of increased numbers of
children with neurodevelopmental impairments. In fact,
there was a steady-state incidence of CP of about 2.5
per 1000 live births until about the mid-1950s (Little,
1958), which was followed by a decrease in incidence
to about 1.5 per 1000 for about 15 years. Since then,
the incidence has increased to near mid-1950s Levels,
if Swedish statistics are typical (Hagberg et al., 1989b).
The change in this trend mainly reflects changes in the
live birth rate of preterm infants, especially in those
with spastic diplegia. A study conducted between 1982
and 1994 of a cohort of 2076 consecutively born
infants with birth weights of 500 to 1500 g (O’Shea et
al., 1998) concluded that the increasing survival of
very low birth weight infants has not resulted in an
increased prevalence of cerebral palsy among
survivors. The risk of CP increases sharply with
decreasing birth weight (Atkinson & Stanley, 1983;
Hagberg et al., 1989a; Pharoah et al., 1987) and has
been reported to be as much as 40 times higher in
infants weighing less than 1000 g (Hagberg et al.,
1989b).
The increased risk of CP in preterm infants
must be put in perspective, however. Reports of longterm outcome of extremely premature infants, that is,
infants born between 24 and 28 weeks of gestational
age, suggest about a 75% survival hte, with more than
50% of those who survive free of major neurodevelopmental impairments, about 25% with major
impairments, and about 11% with CP (Msall et al.,
1991).
Autopsies of infants have revealed three types
of neuropathic lesions (Weinstein & Tharp, 1989): neuropathy resulting from hemorrhage below the lining of
the ventricles (subependymal), encephalopathy caused
by anoxia or hypoxia, and neuropathy resulting from
malformations of the central nervous system.
Most subependymal hemorrhages occur in infants of less than 28 weeks of gestational age and those
with low birth weight. Intraventricular hemorrhages,
present in up to 46% of infants weighing less than 1500
g (Papile et al., 1978), are thought to develop
secondary to lesions of ischemic origin. In most cases,
blood ruptures into the lateral ventricle and the ensuing
connective tissue blocks the cerebrospinal fluid flow,
frequently resulting in hydrocephaLus. Anoxic or
hypoxic encephalopathy results in gray matter and
white matter lesions. Gray matter lesions are diffusely
present throughout the cortex, basal ganglia and
thalamus, brainstem, and spinal cord, whereas lesions
in the white matter are frequently in the periventricular
zone. Periventricular atrophy has been identified as the
most common abnormality found in preterm infants
who developed hemiplegic CP, occurring in 50% of
cases (Wiklund et al., 199 lb). It is unclear, however,
whether the lesions occur before, during, or after birth.
Although periventricular atrophy is a bilateral lesion
thought to be responsible for most cases of preterm
spastic diplegia, it has also been reported as an
asymmetric or unilateral lesion or one with bilateral
lesions expressing only unilateral clinical symptoms
(Wiklund et al.. 199 lb).
Malformations of the central nervous system
may generate hemorrhagic and anoxic lesions. Many
factors may be responsible, including drug ingestion,
radiation, and infection by viruses such as herpes
simplex and rubella.
Attempts to relate cerebral lesions to the
extent of disability have had only limited success. With
respect to the side of expression, in the small
percentage of children with hemiplegia with bilateral
morphologic findings, subtle physical abnormalities
were sometimes seen on the nonhemiplegic side
(Wiklund & Uvebrant, 199la). Results suggest the
existence of a continuum between hemiplegia and
diplegià resulting from periventricular lesions.
Magnetic resonance imaging showed that, of several
measures, only the amount of white matter correlated
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
been found, although trends toward the association of
less impairment with smaller lesions have been reported (Molteni et al., 1987; Wiklund & Uvebrant, 199
la). Quadriplegia has been associated with brainsrm
and basal nuclei damage in addition to cortical and
subcortical lesions (Wilson et aL., 1982). Further
discussion of the causes of the various types of CP can
be found in works by Menkes (1990) and Weinstein
and Tharp (1989).
Progress in Primary Prevention
The primary way to reduce the incidence of
CP is through good socioeconomic health of the
population coupled with maternal education. The role
of poverty and low socioeconomic status in the prevalence of CP (Dowding & Barry, 1990) and in determining the need for special educational resources
(Msall et al., 1991) has frequently been overlooked, yet
there is empirical evidence of its importance.
Certain
maternal
prepregnancy
and
pregnancy-related risk factors are associated with
delivery of a child with a disability (Holst et al., 1989).
Studies have suggested that improved intrapartum
diagnosis of risk factors, prevention of asphyxia, and
medical treatment of children with low Apgar scores
would reduce the incidence of disabilities, as would
intervention to prevent premature rupture of membranes. However reasonable these hypotheses may
seem, no studies are known to have tested them.
The role of the obstetrician in preventing CP
before birth occurs is limited (Weinstein & Tharp,
1989). Attention is directed toward developing effective prevention of and intervention for premature
delivery, fetal distress, neonatal asphyxia, and mechanical birth trauma. Methods of inhibiting labor have
met with much success, although the effects on
incidence of CP remain unclear. Methods of antepartum fetal evaluation, including sonographic measurement, electronic fetal monitoring, fetal pH monitoring, and intrauterine pressure monitoring, have
provided the obstetrician with powerful tools for
assessing the need for active intervention id the labor
process. Delivery procedures using high forceps and
certain presentations of breech deliveries that were
found to be associated with increased perinatal morbidity have dramatically decreased in favor of cesarean
section. This is partly due to the increased safety of
cesarean birth for both the mother and the fetus.
Impairment
Early detection of CP facilitates optimal
management by the family and the health care and
educational community. Complicating the picture is the
instability of diagnosis; CP is reported to disappear
over time in many low birth weight infants (Kitchen et
al., 1987). In a study designed to determine the
accuracy of diagnosis of CP at 2 years of age (Kitchen
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
were identified at age 5 to have CP. Of those children
in whom the diagnosis was no longer accurate at age 5,
most had minor neurologic abnormalities and left-hand
preference, but their psychologic test scores were no
different from those of children who had n’ever been
diagnosed as having CP.
Tests of neurologic status, motor function,
primitive reflexes, and posture have been assessed for
their ability to identify CP (Burns et al., 1989).
Although assessments performed at age 1 month failed
to identify several of the infants who later showed CP,
assessments made at 4 months of age resulted in some
overidentification. At 8 months of age, the presence of
three or more abnormal signs was highly predictive of
CP, and the authors concluded that all but the mildest
cases of CP can be identified by that age. Formal tests
have varying abilities to detect motor abnormalities
(Harris, 1989). The sensitivity of the Movement
Assessment of Infants (MAI) has been calculated to be
73.5% in a high-risk population (Harris, 1987). When
compared with the Bayley motor scale (Bayley, 1993),
the MAI identified more than 3 times as many children
with diplegia at 4 months of age, more than 2 times as
many children with hemiplegia, and about 1.5 times as
many children with quadriplegia (Harris, 1989). In
children at 1 year corrected age, however, the Bayley
motor scale demonstrated sensitivities of 100% for
both spastic diplegia and quadriplegia and 75% for
spastic hemiplegia. Furthermore, the MM has a lower
rate. of specificity than does the Bayley motor scale;
that is, a greatez percentage of children with normal
outcomes are identified as being at risk.
Physical Therapy-Related impairments
Impairments in CP are problems with the
neuromuscular and skeletal systems that are either an
immediate result of the existing pathophysiologic process or an indirect consequence that has developed over
time. Impairments can be classified, somewhat
artificially, into single-system impairments and multisystem impairmcnts.
SINGLE-SYSTEM IMPAIRMENTS
Single-system impairrhents are expressed in the
muscular system and the skeletal system, eventhough
the pathophysiologic damage occurred in the, central
nervous system. Primary impairments such as
insufficient force generation, spasticity, abnormal
extensibility, and exaggerated or hyperactive reflexes
are evident in the muscular system; malalignments
such as femoral anteversion and femoral and tibial
torsion (Cusick & Stuberg, 1992) are secondary
impairments evident in the skeletal system.
CP is characterized by insufficient force
generation by affected muscle groups, which is
consistent with low levels of electromyographic
(EMG) activity and decreased moment of force output
(Berger et al., 1982). When an activity leads to an
active contraction, this impairment may be expressed
as a deficiency in power (Olney et al., 1990), or when
considered over time, in work. The term strength may
refer to any of these measurable factors. Strength measurement in neurologic conditions is problematic, but
when measured, strength has frequently been
intimately linked with functional capabilities such as
speed of walking (Bohannon, 1989).
The clinical term tone is used to describe the
impairments of spasticity and abnormal extensibility. A
sensation of abnormally high tone may be caused by
spasticity, a velocity-dependent overactivity that is
proportional to the imposed velocity of limb movement. Spasticity is especially evident in children with
clonus but is frequently mistaken for problems of
extensibility. Supraspinal and interneuronal mechanisms appear to be responsible for spasticity, with
increased “gain” in the muscle spindles and increased
excitation of Ia afferents having been ruled out as a
cause of spasticity (Young, 1994). There is
experimental evidence for three pathophysiologic
mechanisms: reduced reciprocal inhibition of antagonist motor neuron pools by Ia afferents, decreased
presynaptic inhibition of Ia afferents, and decreased
nonreciprocal inhibition by lb afferents. There is
considerable evidence indicating that reciprocal inhibition is reduced in cerebral palsy (Hallett & Alvarez,
1983; Leonard et al., 1991). Adding to this effect are
recent elegant studies using transcranial magnetic
cerebral stimulation that have provided evidence of
simultaneous activation of antagonistic muscle groups
through abnormal alpha motor neuron innervation
(Brouwer & Ashby, 1991). The role of decreased
presynaptic inhibition of Ia afferents in spasticity has
been deduced from experiments showing that
vibration-induced inhibition of the H-reflex is much
less in spastic than in normal muscles, a phenomenon
that has been shown to be mediated by a presynaptic
mechanism in animal models. Finally, nonreciprocal
inhibition has been reported to be reduced and even
replaced by facilitation in persons exhibiting spasticity
with sustained hypertonia (Young, 1994), which
suggests that there may be afurther mechanism
responsible for abnormal alpha motor neuron
excitability.
The sense of abnormally high tone can also
result from hypoextensibility of the muscle because of
abnormal mechanical characteristics. Comparing
healthy children with children with CP, Berger and
colleagues (1982) found that the EMG activity of leg
muscles in nearly all children with CP was reduced in
affected limbs and that there were no indications of
pathologic reflex effects on muscle activity. A force
transducer on the tendo Achilles measured tension that
was disproportionate to muscle activity and could best
be attributed to mechanical changes in the muscle
rather than to increased stretch reflexes from spasticity.
These muscles were also seen to be abnormally stiff
that is, they produced more force for a given length
change than did muscles in non-disabled children
(Tardieu et al., 1982). The most accurate term for this
impairment is hypoextensibility. The muscle offers
resistance to passive stretching at a shorter length than
that expected in a normal muscle. In addition, if greater
than normal amounts of force are required to produce a
change in length, the muscle is said to have increased
stiffness. This is represented as the passive tension
curve for CP (pCP) in Figure 20-lA, in contrast to the
normal passive tension curve (p,N) in Figure 20-iD,
when one moves the ankle from a position of plantar
flexion to one of dorsiflexion. When a clinician finds
that it is not possible to manually stretch the muscle
through a normal range using reasonable amounts of
manual force, the muscle group is deemed to have a
contraçtur e, represented in Figure 20-2 as
“Contracture,” the difference between the joint angle at
which this extreme resistance is encountered in the CP
muscle and that of the normal muscle.
Figure 20-1 shows hypothetical active forcelength characteristics of spastic plantar flexors (a,CP;
see Fig. 20-1B) and normal plantar flexors (a,N; see
Fig. 20-1E), that is, the force generated by the contractile elements of the muscle over the range of
muscle lengths from a shortened position (plantar
flexed) to a longer position (dorsiflexed). Note that the
maximal force is lower for the CP muscle and also that
the peak force occurs at a more plantarflexed position
in the CP muscle than in the normal muscle. The sum
of the combined effects of active force output and
passive stiffness for the CP muscle is shown as total
tension curve CP, and the corresponding curve for the
normal muscle is shown as total tension curve N (see
Fig. 20-2). The complexity of the representation in
Figure 20-2 underlines the difficulty faced by a
physical therapist or physician in correctly assessing
the cause of increased tone through clinical methods
such as passive manipulation of the limb and clinical
assessment of muscle strength.
If a muscle complex has become
overlengthened, which is usually a secondary
impairment resulting from repeated mechanical stretch,
it is termed hyperextensible. The overlengthened
muscle complex may also have decreased
forcegenerating capabilities.
Few studies of the histology and morphology
of spastic muscle have been reported, but they have
shown that differences are present when spastic muscle
is compared with normal muscle (Romanini et al.,
1989). The slowly contracting fibers of the spastic
adductor muscles demonstrate hypertrophy, whereas
the fast fibers show atrophy. Surprisingly, there has
be’m no evidence of increase in endomysial or
perimysial connective tissue at any age of child,
regardless of the clinical picture. The authors concluded that joint restrictions are attributable to the
atrophy of muscle fibers, which makes the muscle less
elastic and extensible, and possibly to an increase of
fibrous tissue in the periarticular structures, although
the latter was not verified.
There are no universally accepted methods of
measuring spasticity (Katz & Rvmer, 1989), although
techniques include measurement of forces in response
to standard passive stretches (tonic) or standard
hammer stimuli (phasic), Hoffmann’s reflex recording
(Jones & Mullev, 1982), and measurement of responses
to sinusoidal cycling or ramp stretches (Lin et al.,
1994; Price et al., 1991). The modified Ashworth scale,
though commonly used in clinical situations, is really
an undifferentiated measure of spasticity and
extensibility. It has been shown to be reliable in adults
with neurolngic conditions, although reliability has not
been established for children (Bohannon & Smith,
1987).
MULTISYSTEM IMPAIRMENTS
In the second group of impairments are three
multisystem impairments expressed in. the neuromuscular system: poor selective control of muscle
activity, poor regulation of activity in muscle groups in
anticipation of postural changes and body movement
(referred to as anticipatory regulation), and decreased
ability to learn unique movements.
In CP there is poor selective control of muscle
activity. Normal movement is characterized by orderly
phasing in and out of muscle activation, coactivation of
muscles with similar biomechanical functions, and
limited coactivation of antagonists during phasic or
free movement. In CP there is abundant evidence of
inappropriate sequencing (Nashner et al., 1983) and
coactivation of svner~ists and antagonists (Knutsson &
Martensson, 1980).
'
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
muscle activity are unknown. Failure of the normal
recipro Cal relationship of activity between agonist and
antagonist muscles during voluntary movements has
been observed (Berger et aL, 1982; Hallett & Alvarez,
1983; Leonard et al., 1990), but whether segmental-or
supraspinal mechanisms or both are involved is unclear. Although Berbrayer and Ashby (1990) clearly
demonstrated the presence of reciprocal inhibition in
CP, it is not possible to exclude the possibility that
other spinal mechanisms may’ be impaired (Harrison,
1988). Direct evidence for a supraspinal origin is scant;
however, researchers have concluded that in CP, the
corticospinal projections are directed equally to the
motoneurons of agonist and antagonist muscles of the
ankle (Brouwer & Ashby, 1991). Reflex overflow to
antagonist muscles in children with CP (Leonard et al.,
1991) has been attributed either to exuberant
motoneuronal projections or to exuberant projections
that extend to motoneurons innervating muscles other
than the one being stimulated. From these studies, it
appears certain that the neuronal “wiring” in CP is not
normal.
Poor anticipatory regulation of muscle
sequencing when postural correction is attempted has
been reported by Nashner and colleagues (1983). In
healthy individuals, changes in posture are preceded by
preparatory muscle contractions that stabilize the body.
In people with CP, the contraction that is needed to
produce stability is frequently interrupted by
destabilizing synergistic or antagonistic muscle
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
children with CP is frequently impaired (Lesny et al.,
1990). This finding is important in considering strategies for teaching movement, but it has received little
attention to date.
Assessment of multisystem impairments
usually involves measurement of a closely associated
variable or number of variables and frequently involves
different dimensions of the disabling process.
Examination of the impairments of poor selective
control of muscle activity, poor anticipatory regulation
of muscle groups, and decreased ability to learn unique
movements includes use of measures of balance,
coordination, and motor control. Most are not in general clinical use. Two approaches to assessing balance
are available: one is to disturb the supporting surface in
a variety of ways (Nashner et al., 1983); the other is to
perturb the subject or environment (Patla et al., 1989).
In each case, kinematic, kinetic, and EMG responses
are measured. Coordination has been documented with
EMG records, which makes it possible to detect
differences from normal records in timing of muscle
activity onset and duration, in sequencing of agonists,
and in cocontraction of antagonists. Gait has been the
most commonly observed activity used to examine
specific impairments of CP (Perry et al., 1976), and the
potential for its wide clinical application has increased
with the advent of fast and efficient computer systems.
Determinants of Prognosis or Outcome.
About 90% of children with CP in developed
countries survive to adulthood (Evans et al., 1990).
Strauss and Shavelle (1998) found that the key
predictors of a reduced life expectancy were lack of
mobility and feeding difficulties. Survival of high
functioning adults was found to be close to that of the
general population, but predictions of lifetime
functional outcomes in CP are limited. A California
study (Anonymous, 1991) reported that only 12 to 17%
of people with CP registered with developmental
services were competitively employed. Positive
prognostic factors for employment included mild
physical involvement, good family support, vocatioaal
training, and having good employment contacts.
Mental retardation, seizures, and wheelchair
dependency were factors reducing the likelihood of
living independently. Senft and colleagues (1990)
reported that more than 60% of registrants in a
neuromuscular disability program were dependent on
aging parents. In a review of the literature, Bleck
(1987) included the following positive predictors of
independence and employment of a person with CP:
regular schooling, completion of secondary schooling,
independence in mobility with the ability to travel
beyond the home, good hand skills, living in a small
rather than a large community, and having a diagnosis
of spasticity rather than one of involuntary movements.
Preliminary studies of life satisfaction suggest more
positive outcomes, but few studies have included this
important variable (Wacker et al., 1983).
Certain factors assist in predicting the ambulation potential of children with CP. Children with the
hemiplegic type of CP usually have a good prognosis
for ambulation, whereas the prognosis is less favorable
for those with rigid or hypotonic types of CP (Crothers
& Paine, 1988). Persistent tonic neck reflexes are
associated with decreased likelihood of walking
(Crothers & Paine, 1988). Some studies have reported
that a remarkably large percentage of children who are
able to sit independently by age 24 months eventually
walk (Crothers & Paine, 1988) and that nearly all
children with CP who ev~mtually walk do so before 8
years of age (Bleck, 1975). Watt and colleagues
(1989), examining all survivors of neonatal intensive
care, have reported that nearly all who sat by 24
months of age walked 15 meters or more with or
without assistive devices or orthos~s by age 8 years.
Independent sitting by 24 months remains the best
predictor of am: bulation, despite inclusion of neonatal
variables, clinical types, primitive reflexes, and
reactions (Watt et al., 1989).
EXAMINATION, EVALUATION, AND
INTERVENTION
At all ages, the physical therapy examination
of the child with CP will focus on the identification of
disabilities, functional limitations, and impairments. In
addition, physical therapy examination is used to
measure change resulting from intervention at all levels
of the disabling process and provide feedback to
clients.
Physical therapists integrate information from
the many aspects of their examination and evaluation
with prognostic knowledge to predict the optimal level
of improvement that can be expected. They then
develop a plan of care that includes long-term and
short-term goals and outcomes, specific interventions,
and duration and frequency of intervention required to
reach the goals and outcomes.
From infancy to adulthood, physical therapy
goals for clients with CP should focus on the prevention of disability by minimizing the effects of functional limitations and impairments, preventing or
limiting secondary impairments, maximizing the gross
motor functions allowed by the organic deficits, and
helping the child compensate for functions when
necessary. Achieving these goals involves the
promotion and maintenance of musculoskeletal integrity, the prevention of secondary impairment and
deformity, the enhancement of optimal postures and
movement to promote functional independence, and
optimal levels of fitness.
The presence of impairments, such as low
levels of force generation, spasticity, abnormal
extensibility, and disturbed reflexes, can result in
abnormal weight bearing and malalignment, which can,
in turn, affect the orthopedic development of the spine
and the extremities. The application of correct forces is
required for optimal skeletal modeling before the skeleton ossifies (LeVeau & Bernhardt, 1984), although the
research reported to date has offered little specific
guidance. Of particular concern is the effect of
increased hip flexion and adduction on acetabular
development and hip joint stability. Neck and trunk
asymmetry can result in torticollis or spinal deformities. At all ages, children with hypoextensibility and
spasticity are prone to developing contractures. Although patt2rns of tightness vary, commonly at risk for
contractures are the shoulder adductors; the elbow,
wrist, and finger flexors; the hip flexors and adductors;
the knee flexors; and the ankle plantar flexors
(Massagli, 1991).
Furthermore, the physical therapist attempts to
prevent environmental deprivation that could increase
existing disabilities and attempts to provide support,
guidance, and education for the child, the family, and
the community. Goals are individualized for the
particular child and family. They should be determined
in collaboration with the family and based on the
needs, expectations, and values of the whole family
(Rosenbaum et aL, 1998). Goal and outcome
attainment should be regularly reassessed so that the
therapy plan is adapted to reflect changes in the child’s
progress and the family’s needs. An important
component of therapy programs is education of the
child and family about the disability to enable them to
become capable of advocating and baking
responsibility for their future.
The involvement of other health care
professionals in the treatment of the infant with CP
depends on the child’s needs and the practices of the
institution where the program occurs. Some facilities
may have professionals from several disciplines
working with the family, whereas at others it may be
thought better to have a primary therapist initially,
bringing in others for assessment or treatment as
necessary. Regardless of practice approach, parents
value coordination of care and consistency of service
providers.
Increasing emphasis on the costs of provision
of services and managed care have led some
institutions to develop critical paths. This is a difficult
task for CP due to the diversity of presentation and the
chronic nature of the condition. An example of an
outline of care for CP is the document Cerebral PalsyCritical Elements of Care which was developed by the
Washington State Department of Health (1997).
Infancy
The life role of an infant is to grow and develop in
response to being loved and nurtured by parents and
caregivers in a home environment. Despite being
dependent in most aspects of life, infants interact with,
and develop an understanding of, the people in their
lives, their surroundings, and’ themselves. From the
time of birth, a child with CP may not experience the
normal activities associated with infancy. As a result,
the parents of an infant with CP may not receive the
positive feedback of a normal nurturing experience and
the satisfaction of observing the development of motor
and social skills, that is the normal rewards of caring
for an infant. The parents must cope with the impact of
the diagnosis and the grieving process that
accompanies the awareness that their expectations of
having a normal child will not be realized. They may
be overwhelmed with the uncertainty that the future
holds for them, their child, and their family. Many
parents are also concerned with the immediate issues of
providing basic infant care and are apprehensive about
incorporating the specialized care necessary for their
child’s optimal development.
Movement is ~n important component in the
learning and interactive processes of infancy. In infants
who have CP, the nature and extent of their
impairments affect their potential to develop and learn
through movement. This may result in functional
limitations in the development of gross motor skills
and may affect their ability to interact with their
parents, themselves, and their environment.
Physical Therapy
Evaluation
Examination
and
Infant examination provides a baseline for the
monitoring of improvement or deterioration, growth,
maturation, and treatment effects. Therapists must
determine the history, living environment, and social
supports of an infant and the knowledge level and
concerns of the family. Examination of impairment
involves qualitative and, when possible, quantitative
evaluation of the single system and multisystem
impairments. Observation of active range of motion
(ROM) provides indirect assessment of the forcegenerating ability of muscle groups and some
information about muscle extensibility. Determination
of the passive ROM, using a slow, maintained stretch
in a position that promotes relaxation, assesses muscle
group extensibility and provides information about
joints, such as the presence of dislocation. Normal
maturational changes in joint range and alignment must
be considered in evaluating the significance of
measures.
Passive movement performed with greater
velocity is used to assess spasticity and the sensitivity
of the stretch reflex. Spasticity can be documented descriptively on the basis of resistance to movement and
observations of spontaneous active movement and
posturing. The severity of spasticity-whether it is mild,
moderate, or severe-its distribution over the body and
limbs, and its variations under different conditions
should be noted. Frequently, there are variations in
spasticity associated with positioning and the infant’s
effort and behavior. The modified Ashworth scale
(Bohannon & Smith, 1987) or the muscle tone section
of the MAI can be used (Chandler et al., 1980).
The presence or persistence of primitive
reflexes and the development of the postural reactions
of equilibrium, righting, and protective extension are
assessed to determine their influence on selective
control and anticipatory regulation of muscle group
activity. The effects these reflexes and postural reactions have on positioning, handling, and the facilitation
or inhibition of functional movement also need to be
evaluated (Bly, 1991). The primitive reflex and the
automatic reaction sections of the MAI (Chandler et
al., 1980) are appropriate to use when evaluating
infants with CP.
Selective control and anticipatory regulation
of muscle groups are assessed in the context of functional evaluation: for the infant, this is indicated by the
assessment of gross motor skills. Standardized tests
used by physical therapists when assessing infant
movement include the MAI (Chandler & Harris, 1985),
the Gross Motor Function Measure (Russell et al.,
1989), the Peabody Developmental Motor Scales
(Palisano etal., 1995), the Bayley Scales of Infant
Development (Bayley, 1993), the Test of Infant Motor
Performance (Murney & Campbell, 1998), and the
Alberta Infant Motor Scale (Piper & Darrah, 1994).
Various elements of movements and posture combine
to produce functional gross motor skills. These include
the ability to align one part of the body on another: to
bear weight through different parts of the body; to shift
weight; to move against gravity; to assume, maintain,
and move into and out of different positions; and to
perform graded, isolated, and variable movements with
an appropriate degree of effort. When examining functional motor skills, proficiency in incorporating these
elements into the achievement of purposeful and
efficient movement must be evaluated.
Specific assessments of seating, feeding
(Evans Morris & Dunn Klein. 1987), or respiratory
problems may be necessary for infants with problems
in these areas. Growth is often affected in children with
CP; therefore, anthropometric measures, including
head circumference, weight, and length, should be
documented. Growth may influence, or be influenced
by, feeding, exercise, and energy efficiency (Campbell
et al., 1989). Other factors to be considered during assessment include the influence of an infant’s temperament and behavior on performance; sensory, social,
communication, and cognitive abilities; and support
from the environment.
Physical Therapy Goals, Outcomes, and
Intervention
Physical therapy in infancy is focused on
educating the family, facilitating caregiving, and
promoting optimal sensorimotor experiences and skills.
Intervention must address current and potential
problems. Early intervention for children with CP has
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,
1996; Campbell, 1990).
FAMILY EDUCATION
The foremost set of goals at all ages is to
educate families about CP, to provide support in their
acceptance of their child’s problems, and to be of assistance when parents make decisions about managing
both their own and their child’s lives. Infancy is an
important time to foster collaborative goal-setting and
programming strategies with the parents and promote
ongoing communication between families and service
providers. These skills empower them to make
decisions, solve problems, and set priorities, as well as
to become effective advocates for their children and
themselves. Although it is recognized that parents
know their children best, at this stage, the parents’
goals may be overly optimistic and hopeful. Therapists
must be realistic about the prognosis and the efficacy
of physical therapy while remaining hopeful and
providing options for intervention. They can break
down overall goals into objectives that are meaningful,
obtainable, sequential, observable, and measurable
(Kolobe, 1992).
HANDLING AND CARE
Abnormal postures and movements resulting
from impairments can make an infant difficult to
handle and potition. These difficulties can affect an
infant’s interaction with the environment, reaction to
caregiving activities, and development of gross motor
skills. Therefore, a second physical therapy goal is to
promote the parents’ skill, ease, and confidence in
handling and caring for their infant. These skills
alleviate unnecessary stress for parents and child and
also help reduce the influence of the impairments,
thereby preventing unnecessary secondary impairments
and limitations. Parents are taught positioning,
carrying, feeding, and dressing techniques that promote
symmetry, limit abnormal posturing and movement,
and facilitate functional motor activity. The principles
guiding these methods are 1) to use a variety of
movements and postures to promote sensory variety, 2)
to frequently include positions that promote the full
lengthening of spastic or hypoextensible muscles, and
3) to use positions that promote functional voluntary
movement of limbs.
FACILITATING OPTIMAL
SENSORIMOTOR DEVELOPMENT
A third physical therapy goal in infancy is to
facilitate optimal sensorimotor experiences and skills,
thereby reducing functional limitations and disabilities.
Therapy should focus on the development of wellaligned postural stability coupled with smooth mobility
to allow the emergence of motor skills such as
reaching, rolling, sitting, cu~w1ing, transitional
movements, standing, and prewalking skills. These
skills promote the development of spatial perception,
body awareness, and mobility to facilitate play, social
interaction, and exploration of the environment.
Movements that include trunk rotation, dissociation of
body segments, weight shifting, weight bearing, and
isolated movements should be incorporated into gross
motor exercises and activitie. These movement
components, if experienced with proper alignment, can
give the sensory feedback of normal movement
patterns and activities. Good sources for the handling
and treatment of infants and children of other ages
include the works of Finnie (1997), Jaeger (1987,
1989), Scherzer and Tscharnuter (1990), and Wilson
(1991). A practical reference for parents is Children
with Cerebral Palsy (Geralis, 1991). Careful instruction
of the family in specific techniques and activities,
ongoing reinforcement, encouragement, and support
are essential. Clearly written, illustrated, and updated
home programs can be beneficial. Computer-generated
programs or videotaping can be used to produce
personalized, effective, and efficient information
regarding activities, positioning, and exercises.
The normal motor developmental sequence
may assist in guiding the progression of motor
activities, although research indicates that motor
milestones and their components develop in
overlapping sequences, with spurts of development
interspersed with some plateaus and even regressions
(Atwater, 1991). The child with CP does not always
proceed along the normal developmental sequence, and
therapy becomes more functionally oriented within the
scope of the child’s physical capabilities (Blv, 1991).
The stage at which this happens depends on the
severity of the impairments; in some children, it may
occur early in life.
Activities or equipment may be used to allow
attainment of functional skills when impairments
otherwise prevent the development of certain skills.
For example, the sitting position promotes visual
attending, upper extremity use, and social interaction.
Infants with CP may be unable to sit independently,
may sit statically only with precarious balance, or may
not even be able to be seated in commercially available
infant equipment. Customized seating or adaptations to
regular infant seats may be necessary to allow function
in other areas of development to progress. Infants with
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,
mouthing activities should be incorporated into
therapy. Toys may need to be adapted to facilitate
developmental activities.
The care of an infant exhibiting asymmetry,
extensor posturing, and shoulder retraction illustrates
these approaches. Such an infant should be carried,
seated, and fed in a symmetric position that does not
allow axial hyperextension and keeps the hips and
knees flexed. Positioning of or playing with the upper
extremities to allow the infant to see his or her hands,
practice midline play, reach for his or her feet, or suck
on fingers can promote sensorimotor awareness. Active
movements, such as the handling of toys that require
two hands and that encourage the infant to develop
flexor control and symmetry, are incorporated into
daily activities. These activities facilitate the use of the
neck and trunk muscles, promoting anterior and
posterior control. The introduction of lateral control is
the next step in achieving functional head and trunk
control. In some severely affected children, slight gains
in head control may be a goal, whereas in minimally
affected children a fairly normal progression of motor
development is expected, even without intervention.
These therapeutic interventions should not limit
infants’ spontaneous desires to move and play and
explore their environments because even very young
children need to be able to assert themselves and
manipulate their world (Campbell, 1997).
Some physical therapists may adhere to
specific treatment philosophies, although differing
treatment approaches often have underlying
similarities. Two approaches used with infants are
neurodevelopmental treatment (NDT) and the Vojta
approach. NDT has been widely used throughout North
America and other parts of the world as a basis for the
treatment of infants with CP. NDT is based on the
theory that inhibiting or modifying impairments of
spasticity and abnormal reflex patterns can improve
movement. For infants, handling techniques encourage
active movement, and thus they experience normal
movement sensations. The ultimate aim of the
treatment is the acquisition of functioiial movements
that permit children the greatest degree of
independence possible to prepare them for as normal
an adolescence and adult life as can he achieved
(Bobath & Bobath, 1984). The Vojta approach, a
European-based practice, uses proprioceptive information from the trunk and extremities to activate the
central nervous system and guide it toward normal
motor ontogenesis by eliciting appropriate movement
patterns (Vojta, 1984).
ROLE OF OTHER DISCIPLINES
Occupational therapists may be involved in
upper extremity function, particularly as it relates to
play. In addition, speech and language pathologists
may be necessary if there are oral-motor problems
interfering with feeding or early language
development. Community infant development workers
may be involved in home-based programs. Social
workers may help the parents through the grieving
process, explain programs, and direct them to
appropriate resources. Likewise it may be helpful to
join parent support groups or meet with parents who
have been through similar experiences.
Preschool Period
During the preschool years, locomotor,
cognitive. communication, fine motor, selfcare, and
social abilities develop to promote functional independence in children. The process is a dynamic one in
which all these areas constantly interact with one
another. The child’s environment remains oriented
toward the parents, family, and home ci ring this
period, but he or she begins to interact with the outside
world. Child care centers, babysitters, nursery schools,
and playmates thus become part of a preschooler’s
world.
For children with CP, the limitations in motor
functioning may create disabilities in learning, socialization, and attainment of independence (Butler,
1991). Concerns of the parents include the impact of
impaired performance on all areas of development : for
example, their child’s ability to participate in and
become integrated into normal preschool activities. the
development of cognition and language, and the longterm effect of disabilities on future life and
independence.
During these years, the child’s attainable level
of motor skills can be predicted with a greater degree
of accuracy, as the influences of motor impairments on
functioning become apparent. A major area of concern
for physical therapists is the child’s ability to achieve
independent mobility. In addition, skills in overall
gross motor development continue to be a focus of
physical therapy to minimize disabilities, such as the
inability to learn and perform the selfcare skills of
toileting, dressing, grooming, and feeding, and the
limitations in play, communication, social skills, and
problem-solving behavior.
Physical
Therapy
Evaluation
Examination
and
Assessment of disability assumes a primary
focus, but it is important to determine the role of
function arid impairment in the production of
disability. Tests should be administered at regular
intervals to document change that is due to treatment
and/or maturation. Within the dimension of
impairment, direct testing of the forcegenerating ability
of muscle groups is not always appropriate because
spasticity, abnormal extensibility, hyperactive reflexes,
and poor selective control affect the assessment. In
such cases, muscle strength should continue to be
considered in a functional context. Observing activities
such as moving between sitting and standing positions
or ascending and descending stairs assess both
concentric and eccentric power. Endurance should be
evaluated by observing the ability to walk ageappropriate distances or propel a wheelchair a
comparable span. During these years, quantitative
measures of joint ROM and skeletal alignment, including the rotational and torsional alignment of the
pelvis and lower extremities (Cusick & Stuberg, 1992;
see Chapter 15), should be documented using
consistent and standardized procedures. Variations of
10 to 150 occur in intrarater goniometric measurement
in children with CP (Stuberg er al., 1988), and caution
must be used to avoid misinterpreting small changes.
Noting the point at which initial resistance is met with
passive range of motion is clinically relevant but
difficult to accurately measure clinically.
Evaluation of function and disability are frequently included in the same assessments. The Gross
Motor Function Measure (Russell et al., 1989) and the
Peabody Developmental Motor Scales (Palisano et al.,
1995) can continue to be used to monitor the child’s
motor progress. When assessing motor skills, the use of
equipment to achieve an activity should be taken into
consideration. For example, the use of orthoses in
ambulation may substantially affect walking abilities.
Function and disability assessment should also
include mobility and transfers, communication, social
function, bowel and bladder control, self-care and the
degree of reliance on caregivers, adaptive equipment,
and environmental modifications in the performance of
activities of daily living (ADL). The Pediatric
Evaluation of Disability Inventory (Reid et al., 1993)
assesses many of these functional skills in young
children. The Functional Independence Measure for
Children (WeeFIM), a pediatric version of the
Functional Independence Measure (Msall et aL, 1990),
measures disability as quantified by burden of care.
Other measures of ADL (Gowland et al., 1991) such as
the Vineland Adaptive Behavior Scales (Sparrow et al.,
1984) can be used. The Canadian Occupational
Performance Measure (Law et aL, 1990) can be used to
ensure that goals are relevant to the family and to
measure outcomes. Goal Attainment Scaling can be
used to evaluate whether specific individualized
treatment goals or outcomes have been met, but this
form of assessment cannot replace standardized
measures, particularly for research (Palisano, 1993).
Disability measures also include attempts to assesr
health-related quality of life. These measures take into
account age, specific disability, and the factors and
values believed to be important by health care
professionals, parents, and children themselves
(Rosenbaum et al., 1990).
Asseisments specific to certain activities or
equipment may be indicated. These include evaluations
of postural stability (Westcott et al., 1997), augmentative communication, mobility, and gait (Olney et et al.,
1990). Gait assessment measures ambulatory function,
and if kinetic and EMG analyses are included, certain
impairments are also evaluated (Fig. 20-3). The ROM
of the hips, knees, and ankles in each phase of gait can
be observed using a videotape. Particular attention
should be paid to the propulsive movement of ankle
plantar flexion during push-off and to the concurrent
hip flexion. These two events are responsible for much
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
activation of each muscle group, the degree of cocontraction. and the
selectivity and degree of sequencing of muscle-group
activity-all indicators of impairment. Upper extremity
activities such as reaching (Kluzik et al., 1990) haVe
also been studied using videotaping and other sensing
systems.
When assessing children in this age group. it
is necessary to be aware of the effects of attention.
cooperation, and the children’s reaction to being
assessed on the evaluation process. Parents or other
caregivers can provide information on whether a
child’s performance is characteristic of his or her
abilities.
Evaluation must take place at regular intervals
to ensure that goals are still appropriate and therapy
intervention is being appropriately directed.
Physical Therapy Goals, Outcomes, and
Intervention
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
electromyography shows patterns of muscle activities
and aind indentification of the presence of cocontraction of muscle groups. Markers at joints allow
computer calculation of joint movements , force
platforms embedded in floor permit measurement of
individual muscle group contributions to the work of
walking. (Courtest of Human Motion Laboratory
School of Rehabilitation Therapy at Queen’s
University, Kingston, Ontarion)
REDUCING PRIMARY IMPAIRMENT
AND
PREVENTING
SECONDARY
IMPAIRMENT
INGREASING FORGE GENERATION. Treatment
to improve force generation of muscles in this age
group is achieved through performing activities that
create increased demands for production of both concentric and eccentric muscie force. Such act~vtt1es
include transitional movements, ball gx’mnastlcs.
games, and practice of functional skills such as using
stairs (Stern & Steidle, 1994)
SPASTICITY. Several options are available
for the management of spasticity. Interventions have
been directed toward decreasing the impairment ot
spasticity with the goals of prevention of secondary
impairment, comfort and ease of positioning. and
improved functional movement. Decreasing spasticity
during the preschool years allows muscle lengthening
and growth (Boyd & Graham, 1997; Rang, 1990) and
may delay or eliminate the need for orthopedic surgery.
Two interventions most appropriate for the preschool
ages are selective dorsal rhizotomies and botulinum
toxin A injections. These interventions are used if
spasticity is interfering with function and conversely
are not used if a child appears to be dependent on
spasticity for function. Ideal candidates have fair to
good trunk control and selective muscle control; good
cognitive abilities, motivation, and parental support
that are conducive to intensive postoperative therapy
programs; and no fixed contractures or deformity.
Severely affected patients, such as children with spastic
quadriplegia, may be appropriate candidates; however,
in these cases the goals are improved positioning, care,
and comfort (McDonald, 1991). Selective dorsal
rhizotomy is a surgical procedure in which the dorsal
nerve rootlets supplying the lower extremity muscles
are selectively cut. Prospective randomized controlled
clinical trials have found spasticity to be substantially
reduced. Function, as measured by the Gross Motor
Performance Measure, has been shown to be improved
in children who have received rhizotomies compared
with those receiving equivalent physical therapy in
some (Steinbok et al., 1997; Wright et al., 1998) but
not in another (McLaughlin et al., 1998).
Gait analysis in children who have had
rhizotomies has shown improved sagittal motion at the
hip, knee, and ankle; however, abnormalities in
patterns of muscle activation have persisted (Giuliani,
1991). This is attributed to continuing problems with
motor control, which prohibit the proper sequencing of
muscle action. Some gait improvements have been
found to remain 10 years after surgery (Subramanian et
al., 1998). Other positive effects that have been noted
include improved oral-motor control, increased voice
volume and endurance, improved temperament and
concentration, improved bowel and bladder control,
and improvement in growth parameters (McDonald,
1991).
Injections of small quantities of botulinum
toxin A into muscles can prevent the presynaptic
release of acetylcholine at the nerve-muscle junction.
The effect peaks at 2 weeks and may last for 1 to 4
months. The drug is expensive but is often covered by
insurance. Targeted muscles are those in which
spasticity interferes with function and those that are
most prone to developing contractures. These include
the calf muscles, hamstrings, hip flexors, and
adductors. Upper extremity muscles have also been
successfully injected. Botox injections can also be used
as a diagnostic measure before orthopedic or rhizotomy
surgery or as an analgesic agent to reduce pain and
spasm postoperatively (Boyd & Graham, 1997). Injections in children with a dynamic component to calf
equinus were successful in improving passive
dorsiflexion, which may allow more opportunity for an
increase in muscle length. The results were comparable
with but longer lasting than those of a control group
that received serial casting and also demonstrated
fewer side effects (Corry et al., 1998).
Therapy focusing on functional outcomes, but
also emphasizing muscle strengthening, is necessary
after the spasticity intervention for optimal effectiveness, because the children’s muscles are “weakened”
without their spasticity (Fig. 20-4).
HYPOEXTENSIBILITY. Various approaches
are used to maintain muscle extensibility and joint
mobility. Some therapists use manual stretching
programs. The usefulness of these passive maneuvers
is difficult to assess because active exercises,
positioning programs, and equipment are usually used
simultaneously. Research on the effectiveness of
manual stretching on extensibility is inconclusive
(Miedaner & Renander, 1987). Tremblav and
colleagues (1990) found that a prolonged stretch of 30
minutes to the plantar flexors of children with CP
reduced the impairment of spasticity and improved the
voluntary activation of the plantar flexors but not the
dorsiflexors. The effect lasted for as long as 35
minutes. In a parallel study, the stretching session did
not produce a functional improvement in gait (Richards
et al., 1991).
The effects of prolonged stretching programs
have been studied (Tardieu et al., 1988), and it was
found that contractures were prevented if the plan-tar
flexor muscles were stretched beyond a minimum
threshold length for at least 6 hours during daily
activity. The threshold length was the length at which
the muscle began to resist a stretch. The data
prompting this statement are suggestive rather than
conclusive, however. Lespargot and colleagues (1994)
found that physiotherapy and a moderate stretch
imposed for 6 hours daily prevented muscle-body
contracture but did not prevent shortening of the
tendon.
CASTING AND ORTHOSES. Plaster or
fiberglass casting has been used as an economical
method of providing stretch and is commonly used in
serial casting to lengthen hypoextensible calf muscles.
Casting for a 3-week period was shown to be effective
if the hypoextensibility was due to imbalance between
the triceps surae and dorsiflexor muscles but not if the
primary impairment was lack of appropriate muscle
growth in response to bone growth (Tardieu et al.,
1982). Serial casting has also been used for calf muscle
and other muscle groups, such as the hamstrings and
elbow flexors.
FIGURE 20-4. Exercises after rhizotomy are
frequently directed toward increasing force generation
of etensor muscles.
Lower extremity orthoses are used to reduce
impairment, prevent secondary impairment, and facilitate function. The specific goals are prevention of
contracture and deformity, provision of optimal joint
alignment, provision of selective motion restriction,
protection of weak muscles, control of tone and tonusrelated deviations, enhancement of function, and
postoperative protection of tissues (Cusick, 1990).
Ricks and Eilert (1993) found that although casts and
orthoses improved ambulation and preambulation
skills, x-ray’s did not show significant changes in the
bony alignment of the foot and ankle during weight
bearing.
Many variations of ankle-foot orthoses
(AFOs) are available, depending on the biomechanical
and functional needs of the individual child (Knutsson
& Clark, 1991). Solid AFOs are used if restriction of
ankle movement is desired. Children who would
benefit from freedom of movement at the joint can use
hinged AFOs. Hinged AFOs frequently prevent plantar
flexion but permit dorsiflexion, which allows stretching
of the plantar flexor muscle group during walking.
Hinged AFOs have been found to promote a more
normal and efficient gait pattern than do rigid orthotics
(Carmick, 1995; Middleton et aL, 1988). Hainsworth
and colleagues (1997) found that that the range of
movement and gait deteriorated during the short
periods without AFOs when compared with periods
during which the AEOs were worn. Foot orthoses, or
supramalleolar orthoses, may be used for children with
pronation who do not require the ankle stabilization of
an AFO (Knutsson & Clark, 1991). Supramalleolar
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, permitted 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 extension support, which is purported to inhibit abnormal
tone or reflex activity. Although clinicians have
claimed that the splints reduce abnormal tone, improve
positioning, and reduce unwanted reflexes, research has
not substantiated the claims (Carlson, 1984).
Orthoses have also been used during sleep to
prevent the secondary impairment of hypoextensibility,
or contracture. Baumann and Zumstein (1985) found
that the use of double-shell foot orthoses as night
splints from age 3 years to the end of the skeletal
growth period prevented calf muscle contractures from
developing and made the need for surgery rare.
Other materials such as Lycra (Blair et al.,
1995), neoprene, and tape have been used for splinting
to assist children biomechanically and facilitate function. Caution must be taken concerning the skin
tolerance of these materials.
ORTHOPEDIC SURGERY
Orthopedic surgery in preschoolers is usually
performed to prevent secondary impairment by limiting
the effects, but not the causes, of hypoextensibility and
spasticity. For example, the lengthening of
hypoextensible or spastic hip adductors (or both) may
be performed to prevent subluxation or dislocation of
the hip joint. Sometimes, however, surgery such as
tendo Achillis lengthening is delayed because of the
tendency for recurrence necessitating repeated surgery
(Tardieu et al., 1982). Ideally, surgery is deferred until
age 6 to 8 years, when multilevel corrections can be
performed if necessary (DeLuca, 1996). Massagli
(1991) has emphasized that musculoskeletal surgery
does not alter the neurologically driven patterns of
muscle activity, although lengthening, releasing, or
transferring a muscle can alter its influence. Decreased
force production is often a complication. Orthopedic
surgery is sometimes combined with neurectomy if
tonic activity of the muscle is present as a result of
spasticity or other neurologic causes. A full discussion
of the role of orthopedic surgery in CP can be found in
the work of Rang (1990). Physical therapists play
important roles in surgical decision making. They are
also involved in the care of the child who is
immobilized and in providing postoperative therapy
(Harryman, 1992), particularly because decreased force
production is a significant complication of surgery.
Frequently forgotten is the importance of transporting
children safely in motor vehicles when they are in
casts, an activity for which various devices are
available (Bull et al., 1989).
POSITIONING. Alignment of the body as a
whole is important. Children should have a variety of
posit tions in which they can optimally function, travel,
and sleep. Varying the positions of children who are
limited in movement also helps prevent the secondary
impairments of positional contractures and deformity,
as well as skin breakdown (Healy et al., 1997).
Decreased ability to change body position during sleep
can cause disrupted rest for children with CP (Kctagal
et al., 1994).
Position changes can also contribute to pulmonary health. Severely involved children are at risk for
chest complications because of chest wall biomechanics, feeding difficulties, immobility, and poor
coughing abilities. Adaptive seating has also been
shown to improve pulmonary functioning (Nwaobi &
Smith, 1986). For the preschooler, sitting, standing,
lying, and a position suitable for playing on the floor
are important. When prescribing seating systems, it is
necessary to be aware of not only the child’s comfort
and functional abilities but also the caregivers’
concerns and needs and the child’s environment.
Seating inserts can be used in a variety of situations
and with equipment such as strollers and wheelchairs,
which are often needed to enable parents to transport
their child easily. Specific suggestions are included in
Chapter 24. Approved car seats and restraints are
necessary for safe and comfortable vehicular
transportation (Shaw, 1987).
Positioning in standing is thought to reduce or
prevent secondary impairments by maintaining lower
extremity muscle extensibility, maintaining or
increasing bone mineral density, and promoting optimal musculoskeletal development (Stuberg, 1992),
including acetabular development. A study of bone
mineralization in children with hemiplegic CP concluded that bone cize and density decrease with increasing neurologic involvement, and weight bearing
may slightly lessen the effect (Lin & Henderson,
1996). Optimally, standing involves movement and
activity to provide intermittent loading and muscle
strain. Standing programs are often started at 1 year of
age if children are not able to bear their weight
effectively on their own. Stuberg (1992) recommended
positioning instanding for 45 minutes two or three
times a day to control lower extremity flexor
contractures, and for 60 minutes four or five times per
week to facilitate bone development, but notes that
there is no definite evidence to support these
guidelines. Maintenance of the child’s ability to bear
full weight through the-legs reduces the need to be
lifted by caregivers.
TREATMENT
LIMITATIONS
OF
FUNCTIONAL
Physical therapy for treatment of functional
limitations is often intensive during the preschool
years. The frequendy of treatment varies, depending on
the resources available, complementary programming,
client goals, parental needs and desires, and the child’s
-response to treatment. Optimal treatment frequency is
unknown, but periods of increased frequencies have
shown improvements in attainment of specific
treatment goals at levels that were maintained when
frequency decreased, provided the skills were
incorporated into daily functional activities. Bower and
McLellan (1992) found that bursts of intensive
physiotherapy directed at achievable specific
measurable goals accelerated the acquisition of motor
skills compared with conventional physical therapy.
Therapy should be challenging and
meaningful to the child and progress to integrating the
skills learned into functional and cognitively directed
skills for carryover. Movement tasks should be goal
oriented and interesting to maintain motivation and
arousal. For example. kicking a soccer ball may be a
more functional and motivating method of developing
balance skills than practicing standing on one foot.
ChIldren with CP are able to perform concrete
perceptuomotor tasks much more readily than abstract
ones, even if the same movements are involved (van
der Weel et al., 1991), because more information is
available from the environment to direct the task.
Motor control and motor-learning principles
(see Chapter 6) can be used to develop treatment strategies for reducing functional limitations. Feedback is
important in the process of learning skilled movement.
Feedback through the child’s sensory receptors
provides intrinsic information, whereas extrinsic
feedback through various forms of biofeedback
provides information from external sources. Knowledge of results contributes information about movement outcome, and knowledge of performance supplies
feedback about the nature of the movement (Poole,
1991).
Peedforward mechanisms must also be
considered, because there is a cognitive component to
movement skills. In some instances, cognitive strategies may be able to compensate for some of the
inherent motor limitations.
Many children with CP do not have normal
cognition and behavior, and activities must be adapted
accordingly. If a child is unable to learn, training using
memorization of solutions may be necessary, although
limited transfer to novel situations will occur (Higgins,
1991). If behavioral factors are negatively affecting
treatment, a behavioral approach using appropriate
motivators may encourage children to work on certain
skills (Horton & Taylor, 1989).
Improvements in functional movement of the
preschool-age child are made by reducing the effects of
the multisystem impairments of selective control,
anticipatory regulation, and learning of unique
movements. Although there is a growing literature on
motor learning (see Chapter 6), on motor control in
skill acquisition (see Chapter 2), and on the biomechanics of movement, the profession is still far from
being able to provide optimal strategies for treatment
that are known to be effective (Fetters, 1991). The
therapist who treats children with CP should modify
approaches as research produces new insights in the
areas of motor learning and motor control.
FEEDING, DROOLING, AND TOILETING.
Some children with CP, particularly those who are
severely affected, may have oral-motor problems, such
as poor mouth closure, retraction or thrusting of the
tongue, poor tongue movements, and poor coordination
in swallowing, which can make speech and feeding
difficult. Feeding problems can be aggravated by other
problems such as impaired self-feeding and difficulties
in expressing hunger or food preferences that may
result in inadequate nutritional intake and poor growth
(Gisel & Patrick, 1988; Reilly & Skuse, 1992).
Gastroesophageal reflux and aspiration also occur in
children with severe CP. Oral-motor programs, proper
positioning, and parent education and support are
important issues to address (Evans Morris & Dunn
Klein, 1987). In extreme cases, gastrostomy and
antireflux procedures may be necessary to improve
growth and enhance the quality of life for the child and
the family (Rempel et al., 1988).
Drooling, a significant problem in about 10%
of children with CP, can cause social embarrassment
and affect the quality of social integration (Blasco et
al., 1992). It can result from dysfunctional oral-motor
activity, oral sensory problems or inefficient and
infrequent swallowing. Management may include
waiting for further neurologic maturation, feeding and
oral stimulation programs, behavior modification
programs, medications, or surgery.
Failure to develop an appropriate toileting
routine during the preschool years can result in an
ongoing disability, because incontinence can provoke
negative reactions from caregivers and peers. Expectations of toileting in children with CP should be
similar to those for children of comparable cognitive
abilities, and therapists should encourage training at a
comparable age and recommend appropriate adaptive
equipment as necessary (Shaw, 1990).
MOBILITY
Ambulation is a major concern of physical
therapists during these years. Emphasis in treatment is
initially on prewalking skills, such as attaining effective and well-aligned weight bearing, promoting dissociation and weight shifting, and improving balance.
Ambulatory aids, such as walkers and crutches, may be
used, either temporarily while the child is progressing
to more advanced gait skills or as long-term aids for
independent mobility. The use of posterior walkers has
been found to encourage a more upright posture during
gait and to promote better gait characteristics than does
the use of anterior walkers (Fig. 20-5) (Logan et al.,
1990).
Children in this age group are becoming
aware of the concept of achievement, and although
ambulation is a coveted skill, it should not become an
allconsuming goal, particularly if it may not be attainable. When interviewing adults with significant
impairment, Kibele (1989) found that they remembered
walking as the most important goal set for them by
their parents and therapists. This resulted in feelings of
failure from an early age and also in a loss of faith in
rehabilitation professionals.
The provision of alternative means to allow
children functional, independent mobility when ambulation is impossible or inefficient is recommended.
Sometimes this need is met with an adapted tricycle
(Fig. 20-6) or manual wheelchair; other children require one of a wide variety of power mobility devices
available (Jones, 1990) and may need special controls
(Fig. 20-7). These enable children with CP to explore
their environment and achieve a sense of independence
and competence. Power mobility may also promote the
development of initiative (Butler, 1991) and the
acquisition of spatial concepts. The lack of selfpropelled locomotion can result in apathy, withdrawal,
passivity, and dependent behavior that can persist into
later life (Butler, 1991).
indication of the implications of power mobility on
housing and transportation needs. For more
information, see Chapter 24.
PLAY
Play, the primary productive activity for
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 ambulationoriented therapy but provides the child with a method
of moving about independently in the meantime. For
children who will continue to be wholly dependent on
power mobility for independence, it provides mobility
at an appropriate age and gives the families an
FIGURE 20-6 An Adapted tricycle may meet a child
need for mobility.
INTERVENTION APPROACHES
FIGURE 20-7 Single – swoith scanning computer
controller for power wheelchair permith indenoendent
mobility for children with limited motor control.
Developed at Hugh MacMillan Rehabilitation Centre,
Toronto, Ontario
It is important that children are not overprotected in their attempts to play. Parents should be
encouraged to let their children enjoy typical play
actisis such as rolling down hills and getting dirtx’ in
the ttttid. Therapists must ensure that therapy and home
programs promote, rather than interfere with, the
novtmtl play experiences parents have with their
children.
Figure 20-8 Therapeutic exercise programs can include
highly motivaTING PLAY ACTIVITIES. Thorowing a
basketball may be more motivating than trunk
extension exercises.
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 improve 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
theory that the difficulties of the child with motor dvsfu
n tion are problems of learning. The goal of treatment
is the ability to function independently in the world
without aids, an important objective in Hungary, where
schools are not adapted for children with disabilities.
Participants are selected for their abilify to learn, which
may make some children ir~eligible for the program.
They are usually treated in group settings that provide
the incentive of competition and allow more time in
therapy than does individual treatment. Functional
goals are broken down into small steps. Children
initiate the activities on their own, with direct
conscious action aided by mental preparation (Bairstow
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 discrepancies 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
when they requested the services, when they were
present during treatment, and when targeted goals were
met. They concluded that the most appropriate
approach for a child would be one that would meet the
needs of the particular child. Parents of children with
CP often consider alternative therapies that may not be
accepted by some professionals and are usually
expensive. If families choose to take an approach other
than the one offered by the therapist, it is necessary to
respect their choice. Reasons for seeking alternatives
include a desire for more therapy, dissatisfaction with
present therapy, and belief that their child could do
better (Milo-Manson et al., 1997). Therapists should
not react defensively but should provide impartial
information about the therapies in question.
FAMILY INVOLVEMENT
The importance of providing family-centered
setvice continues. Planning of interventions should take
into account the child within the context of the family.
Therapists should be sensitive to the family’s stresses,
dynamics, child-rearing practices, coping mechanisms,
privacy, values, and cultural variations. Therapists
should be flexible in their approach and programming
(Kolobe, 1992). Special efforts must be made to deal
with the effects of a child with CP on the siblings
(Powell & Ogle, 1985).
Family involvement is crucial for integrating
treatment into everyday life. Home programs are important for optimal results from therapy programs
because strengthening, extensibility, and motor
learning often require more input than can be provided
by limited treatment resources. It is necessary to find a
balance between providing parents with home
programs that make them an integral part of their
child’s therapy and burdening them with activities they
cannot realistically be expected to carry out. Obstacles
may includ&constraints on time, energy, skills, or
resources or negative effects on the parent-child
relationship. Hinojosa and Anderson (1991) found that
although mothers of preschoolers with CP did not carry
out specific home programs designed by therapists,
they did activities that could be integrated into their
daily routines and interactions and that were not
stressful for the child or the caregiver.
Siblings can be included in home programs.
Craft and colleagues (1990) studied a group of children
with CP whose siblings had been educated about the
condition and ways in which they could encourage
their brother or sister to be more independent. As a
result of this, the children with CP showed increases in
ROM and in functional independence.
ROLE OF OTHER DISCIPLINES
Occupational therapists work closely with the
children at this age to develop independence in activities such as dressing, feeding, toileting, and playing.
Speech and language pathologists continue to develop
efficient methods of communication in children with
CP. Psychologists assess cognitive skills and advise on
the interaction of intellectual abilities with the other
areas of development. Social workers and behavior
therapists continue to provide ongoing support to the
families, because the stresses involved in parenting a
child with a disability persist (Sternisha et aL, 1992).
Team assessment and intervention are imperative for
addressing issues such as feeding problems,
augmentative communication, and transition to school.
School-Age and Adolescent Period
During the school-age and adolescent years,
children typically become more involved in school and
community life while remaining dependent on their
families and living in their parental homes. They refine
and augment the basic functional skills they
have learned and develop life skills that will enable
them to cope effectively with the demands of daily
living and, it is hoped, independent adult life.
These can be difficult years for children with
CP. They become more aware of the reality, extent, and
impact of their disabilities on themselves and their
families. As they strive to contend with the normal
stresses of growing up, particularly those of adolescence, they must also cope with being different, acknowledge the potential obstacles to attaining independence, and work to overcome them.
Parents remain anxious about how their childs
disabilities will affect their participation in educational
environments and in social situations. They may worry
about their child’s future as an adult (Hallum, 1995).
While continuing to be naturally attentive, they have to
avoid being overprotective and begin to allow their
child to take risks and become independent in the
outside world. In some cases, there may be financial
concerns regarding the need for special equipment,
transportation, and home renovations. Parents of
children who are dependent in ADL and transfers may
suffer from physical stresses, such as back problems.
Typical disabilities encountered during these
years include a lack of independent mobility, poor
endurance in performing routine activities, and continued difficulty and slowness with self-care and hygiene skills at a time when privacy is becoming in-
creasingly important. Adolescents may also not have
the opportunity to develop socially and sexually and
may lack the ability to acquire age-appropriate levels of
independence from the family. Societal barriers may
reduce access to community and school facilities, thus
limiting opportunities for participation in social,
cultural, and athletic activities.
Physkal Therapy
Evaluation
Examination
and
Assessment of impairments that could
interfere with function, or lead to further secondary
impairment, such as scoliosis, continues to he
important. Children in this age group may be able to
participate in measurement of force production using
dynamometry. Assessment of gross motor function is
appropriate, because children are often making gross
motor progress during their school years. They may
also change as a result of interventions such as surgery,
the use of orthoses, and periods of intensive therapy.
The development of the Gross Motor Performance
Measure (Boyce et al., 1995) may help in detecting the
fine increments of motor skill gains that are
characteristic of children who are severely affected. For
children with mild degrees of involvement, the
Bruininks-Oseretsky Test of Motor Proficiency
(Bruininks, 1978) may be useful in measuring specific
components of higher levels of motor function. Tools
such as the School Function Assessment may be used
in educational settings (Coster et al., 1994).
Gait analysis continues to be helpful in
assessing ambulatory children, particularly when
decisions about surgery are being made (Lee et al.,
1992), although this opinion is not held by all (Abel,
1995). Endurance and efficiency of movement become
increasingly important during these years as the children venture into the community on their own or with
their peers. Mechanical energy costs can be assessed
with motion analysis equipment (Olney’ et al., 1987).
In the clinical setting, physiologic measures such as the
physiologic cost index for activities such as ambulation
can give energy cost estimates (Butler, 1991; Mossberg
et al., 1990) or assist in selecting assistive devices
(Rose et al., 1985). In a laboratory setting, ergometer
assessment can analyze other aspects of endurance
(Parker et al., 1992). An important consideration in
maximizing endurance for daily activities is the effect
of excessive weight gain, because it can compromise
optimal function and efficiency.
Privacy of individuals at all ages should be respected, but it is particularly important during these
years when children are becoming more aware of their
bodies and their sexuality. Children should be
appropriately dressed when attending therapy sessions
and clinics, particularly if they are being seen by
unfamiliar people or are being photographed or
videotaped. If it is necessary for children to remove
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
converse only with caregivers or other professional
staff.
Physical Therapy Goals, Outcomes, and
Interventions
As goals become oriented toward the child’s
or adolescent’s lifestyle, emphasis is on maintaining or
improving the level of function while considering the
stresses of growth, maturation, and increastna demands
in life skills and participation in communitiy activities.
Although the pathophysiologic impairment of CP is
nonprogressive, there are changes related to the stresses
of increasing size, accumulative physical overuse, and
a more competitive lifestyle. For example, as children
advance to higher grades in school, there may be
longer distances to walk between classes. Contractures
may rapidly develop during periods when boEes are
growing faster than muscles (Tardieu et al., 1982).
Secondary orthopedic and functional problems must be
anticipated and avoided. The maintenance of muscle
extensibility and force generation, joint integrity, and
fitness is important in preventing secondary
impairments that can result from the stresses of aging.
This age group also needs to develop problem-solving
strategies to overcome environmental and societal
barriers to become as independent and active as
possible in home, school, recreational, social, and
community life.
In many cases, adolescents can and should be
involved in setting goals and determining programming. It is important during these years to encourage
them to take responsibility for their own health, care,
and decision making so that they are prepared to
assume these responsibilities in adulthood (Fox et al.,
1992). It is also important to look ahead and set goals
that are appropriate for their later life situations and
independence. Therapists should strive to foster selfesteem and assertiveness in children and adolescents by
emphasizing their abilities, finding areas and activities
in which they can excel, and helping them to
acknowledge their difficulties with a view toward
identifying appropriate compensations and use of
attendant care. In the case of children with severe
(multiple) disorder, goals are oriented toward
minimizing impairments to facilitate caregiving and
comfort.
REDUCING PRIMARY IMPAIRMENT
AND
PREVENTING
SECONDARY
IMPAIRMENT
The impairment of deficient muscle force
generation that may result in functional limitations and
disabilities is important to address in this age group.
Damiano and coworkers (1995) used ankle weights in a
6-week progressive resistive strengthening program for
the quadriceps in 14 children with spastic diplegia.
Stride length increased, and knee flexion at initial
contact decreased. An 8-week isokinetic strengthening
program for mildly affected adolescents effected
strength, gains of 12 to 30% in knee extensors and
flexors but no increase in spasticitv (MacPhail &
Kramer, 1995). Nine of the seventeen subjects showed
improvements in the standing and walking, running,
and jumping dimensions of the Gross Motor Function
Measure. Using free weights in a home program
supervised by’ a family member, Damiano and Abel
1998) reported average strength gains of 69% when the
two weakest muscle groups were trained in children
with spastic diplegia or hemiplegia. The gains were
associated with positive functional outcomes, although,
as with the MacPhail and Kramer study, it is not clear
whether increasing strength alters gait efficiency.
Various methods of electrical stimulation have
been used as an adjunct to the treatment of CP to
reduce spasticity, to increase force production and
extensibility, and to improve function activities such as
gait (Stanger & Bertoti, 1997). This treatment adjunct
is particularly useful in postoperative muscle training
and strengthening. Protocols should be individualized
for patients following careful analysis of movement,
and application should be closely monitored.
It was once thought that individuals had no
control of their spasticity. Subjects with CP, however,
have been able to reduce their responsiveness to a
stretch reflex stimulus imposed during a lower limb
activity (O’Dwyer et al., 1994). These findings encourage further exploration of the possibilities of
reducing spastic responses.
Pharmacologic intervention can be used to
control the impairment of spasticity. The use of oral
medications has been poorlystudied, but medication
may be appropriate for some children (Pranzatelli,
1996). Children must be carefully assessed for
appropriateness and monitored closely for side effects.
Baclofen, a synthetic agonist of aminobutyric acid, has
an inhibitory effect on the presynaptic excitatory
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
proper concentration in the cerebrospinal fluid can
cause side effects such as drowsiness. To circumvent
this problem, baclofen is most effective if given
intrathecally by’ a continuous infusion pump implanted
in the abdomen that releases the drug at a slow,
constant rate into the subarachnoid space. The use of
intrathecal baclofen has been postulated to reduce the
need for orthopedic surgery (Gerszten et al., 1998).
Biofeedback is a useful tool in addressing
single-system and multisystem impairments during
these years (Fig. 20-9) because by this time children
have usually developed abstract thinking and sufficient
cognitive ability to use it optimally. Positive results
have been reported, but carryover is often limited,
generalization to real-life situations is not readily
demonstrated, and treat~rient is time consuming
(James, 1992). Toner and colleagues (1998) found that
a program of biofeedback improved active range of
motion and strength and motor control in dorsiflexion
in a group of children with CP.
The secondary impairment ofjoint or muscle
contracture may occur in this age group, particularly in
the more severely affected patients. This can be a result
of chronic muscle imbalance, abnormal posturing, or
static positioning. Casting may be used to increase the
range of joint movement by lengthening muscles or
tendons or both with nc associated loss in strength
(Brouwer et al., 1998; Cusick, 1990; O’Dwyer et al.,
1989; Tardieu et al., 1982). Interventions of botulinum
toxin A and casting may complement each other in
children with spasticity and contractures. Anderson and
colleagues (1988) found that soft splints made of
polyurethane foam were effective in reducing severe
knee flexor contractures; this may be an attractive
alternative for low-income families.
Orthopedic surgery continues to be used to reduce, the effects of primary and secondary impairment
on mobilirv, posture, cosmesis, and hygiene and to
prevent further secondary impairment. Possible
surgical procedures include muscle lengthenings or
transfers, tenotomies, neurectomies, osteotomies. and
fusions.
Surgery may improve posture and gait, but
outcomes can be unpredictable because muscle weakness often results. As children get older, they should be
active participants in surgical decision making that may
he influenced by their interests, priorities. and concerns
about interruptions in their lives as result of
hospitalizations, immobilizations, and recovery
periods.
The secondary impairment of joint
hypomobility resulting from capsular or ligamentous
tightness can be treated with manual therapy
techniques (Brooks-Scott, 1995). Joint mobilizations
may be used to regain joint mobility, particularly after
immobilization. Therapists must ascertain whether
joint structures are causing movement restriction and
must also be aware of the contraindications and
precautions relevant to using mobilizations in growing
and neurologically involved patients (Harris &
Lundgren, 1991).
Spasticity, abnormal extensibility of muscles,
muscular imbalance, and decreased force generation
can resulr in scoliosis, which, in turn, can affect
positioning and respiratory status. Spinal deformity can
be a particularly difficult problem in the severely
affected patient. Rigid orthoses can result in skin
breakdown arid patient intolerance; by contrast, the use
of a soft orthosis has been found to be beneficial in the
management of scoliosis in patients with CP (Letts et
al, 1992). In some-ases, surgical intervention may be
necessary (see Chapter 10).
wheelchair or a power scooter, as they become larger
and need to travel greater distances to meet their social
and educational objectives. Ambulant children with
cerebral palsy are less active than their peers (van den
Berg-Emons et al., 1995). Availability of power
mobility should not preclude activity to the point of
decreased musculoskeletal integrity or physical fitness.
Mobility devices may require modifications, such as
ramps to buildings or washroom renovations, for
accessibility’. Dnve r training offers the freedom to
travel independen:Iy. For those unable to drive,
instruction in the use of public or special transportation
should be provided.
The functional limitation of low endnrance
levels, frequently related to inefficiency of movement,
has been identified in children with CP. Rose and colleagues (1990) found chat energy expenditure indices
based on oxygen uptake and heart rate measured at a
given walking speed were 3 times higher in children
with CP than in normal control children. Findings that
were similar, but based on mechanical energy analyses,
were reported for children with hemiplegia (Olney et
al., 1987). Rose and colleagues (1985) also found that
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 implemented to promote function and circumvei~t disability
during the school years. The continued use of po’ver
mobility devices is important for independent mobility.
Often children who are able to walk independently
need an alternative form of mobility, such as a manual
FIGURE 20-10
Computer-based communication
system with keyboard on computer screen and head
pointer acting as controller. Developed at Hugh
MacMillan Rehabilitation Centre, Toront, Ontario
SCHOOL AND COMMUNITY
Many children are involved in school based
programs, particularly with continuing application of
the Education for All Handicapped Children Act in
1975 in the United States (Poirier et al., 1988) and the
Charter of Rights and Freedoms in Canada in 1982
(Poirier et al., 1988). In most jurisdictions, the
educational program must be implemented in the least
restrictive environment for that child, and “the child.
mainstreamed to the fullest possible extent” (Clune &
Van Pelt, 1985). Facilities and resources such as
support personnel, equipment for accessibilityi, and
computer-based systems (Fig. 20-10) are necessary to
meet the physical needs of children in the school
system. Therapists working with school personnel may
instruct assistanrs and teachers in positioning, lifting,
and transferring the children; carrying out exercise
programs; and adapting and developing physical
education programs (Wilcox, 1988). Therapists may
also be involved in accessibility, transportation,
evacuation, and other safety issues. Therapists working
in school settings must be sensitive to the physical and
scheduling constraints of the educational environment
and be willing to compromise to meet the educational
priorities of the students. Therapy may range from
consultation and monitoring for students who are
thought to have reached their maximal level of
functioning, to active therapy for children who have
specific treatment goals. When children are primarily
seen through the educational system, effort must be
made to keep the family involved in all aspects of care
and treatment. See Chapter 32 for further information
on this subject based on experiences in the United
States.
In addition to educating school staff about
children with CP, therapists can help educate the other
children in the classroom. Knowledge ahout, and
positive interactions with, people with disabilities can
create positive attitudes, particularly when children are
between 7 and 9 years of age, because their attitudes
about people with disabilities are flexible at this age
(Morrison & Ursprung, 1987).
It is important for children with disabilities to
be involved in community and recreational activities
that provide social as well as therapeutic opportunities.
Many adapted or integrated sports activities are
suitable for people with CP, including horseback riding
(MacKinnon et al., 1995), swimming, skiing, sailing,
canoeing, camping, kayaking, fishing, bun-gee
jumping, yoga, and tai chi. Adapted games can provide
athletic competition and participation in team
experiences and can facilitate the social aspects of
sports (Jones, 1988).
All athletes are at risk for sports-related
injuries, but relatively minor injuries can incapacitate
people with CP. They should be encouraged to be
responsible for their bodies during sports activities by
following appropriate conditioning, warm-up, and
cool-down routines; following comprehensive injury
prevention programs, which include strengthening,
flexibility, and aerobic and anaerobic training activities; and using appropriate protective and orthotic
equipment. Injuries should be treated promptly. The
knee is the most frequently injured body part in athletes
with CP. Shoulders, hands, and ankles are also
vulnerable (Ferrara et al., 1992). For more information,
see Chapter 17.
During these years, children learn about their
bodies, their sexuality, and appropriate interactions
with other people. Children and adults with disabilities
have an increased risk of suffering abuse, including
sexual abuse, which can result in physical, social,
emotional, and behavioral consequences (Hallum,
1995; Sobsey & Doe, 1991). Many of the perpetrators
have relationships with the victims that are similar to
those commonly found among nondisabled victims;
however, some abusers have relationships specifically
related to the victim’s disability. These people can be
personal care, attendants, transportation providers,
residential care staffi and other disabled individuals.
Abuse is a serious matter that must be guarded against
at all times. Physical therapists must know how to
detect the signs of abuse, be sensitive and receptive to
clients who may choose to confide in them, and know
the proper procedures to follow if they suspect abuse.
They must work with other professionals to promote
assertiveness and positive self-esteem in their clients.
All professionals involved with patients who
have CP must educate them in being streetwise. Their
physical and sometimes cognitive limitations can make
them particularly vulnerable to crime. Children and
adolescents with disabilities should be taught to avoid
threatening situations. They should be warned about
carrying valuables (large sums of money or important
medications) with them, particularly in a purse that
attracts attention and is easy to grab) such as one slung
over a wheelchair handle. Attendance at self-defense
courses should be encouraged; some that are
specifically designed for people with disabilities are
becoming available.
Health care professionals must realize that although parents have been coping with their children’s
needs for a number of years, parent education is still
important: their children and their needs are constantly
changing. Continued attention to education in lifting
and transferring is necessary to prevent injury to
caregivers as their children grow larger and heavier and
they themselves are aging.
the family, and the health care team to provide
comprehensive planning to ease the transition to
adulthood.
ROLE OF OTHER DISCIPLINES
Occupational therapists may be involved in promoting independence in ADL. Multidisciplinary lifeskills training may be offered to focus on self-care,
community living, and interpersonal relationships.
Prevocational training and related activities, such as
money management and employment searching, may
be necessary. Psychologists or social workers may be
involved in social and sexual counseling.
Transition to Adulthood
The life role of an adult is to be an
independently
functioning
and
self-sufficient
individual who has a satisfying social and emotional
life and is a contributing member of society. The
natural environment for adults is living independently
in the community, alone or with others, with
employment to support them.
The extent to which people with CP can
realize these goals depends on factors such as level
oficognition, available resources and support, and
independence in self-care activities and mobility. Many
adults with CP continue to live with their families, in
group homes, or in institutions, and a small proportion
of them are employed. In Finland, a study of young
adults who had been followed since childhood revealed
that 31% of those older than 19 years were
competitively employed, but an additional 21% who
were judged to be employable were unemployed
(Sillanpaa et al., 1982). Finding employment can be
particularly difficult when there is considerable unemployment in the able-bodied population.
At a time when most parents are experiencing
freedom from caregiving responsibilities, many parents
of children with CP continue to have these obligations
and have to cope with many anxieties (Hallum, 1995).
Their concerns focus on how their child can function as
an independent adult, how they can continue to care for
their child as they themselves age, and who will care
for the child when they are unable to do so. They, and
their child, may also be coping with a decrease in the
numbei of relatively organized and available programs
that were available for the younger child.
Physical therapy involvement continues to address all levels of the disabling process; however, there
is an emphasis on working together with the individual,
Physical
Therapy
Evaluation
Examination
and
Although there is a continuing need to assess
impairment, adult assessments focus on functicn and
disability, particularly on the level of independent
functioning. A variety of functional disability scales,
such as the Barthel Index, have been developed for
adults in general rather than for adults with a particular
disability (Mahoney & Barthel, 1965). These measure
performance in various self-care, independence. and
mobility functions (Spector, 1990).
Physical Therapy Goals, Outcomes, and
Interventions
The major goal during this period of transition
is to maximize the client’s capabilities to achieve optimal independence and happiness as an adult. Ideally,
the medical, therapeutic, and educational goals of
childhood have had this as a long-term goal in earlier
intervention. In a study of adults with CP health in
terms of acute illnesses was not a problem, but 76%
had multiple musculoskeletal problems, and many
nonambulatory adults had urinary complaints.
Equipment and therapy needs were poorly met
(Murphy et al., 1995). In many situations, professionals, the client, and the family are dealing with
external environmental forces that make it difficult for
a person to overcome disabilities.
REDUCING PRIMARY IMPAIRMENT
AND
PREVENTING
SECONDARY
IMPAIRMENT
Although there is a focus on overall disability,
therapists must still be cognizant of the impact of
impairment on function. Adults with CP must deal with
the normal effects of aging in addition to their existing
impairments (Overeynder & Turk, 1998). Insufficient
force generation and hypoextensibility can still respond
to therapy. Although secondary impairments such as
contracture may appear to be static, there can still b3
deteriorarion, so momtoring and treatment, if
necessary, should be available. If significant deformity
occurs, aggressive salvage surgery may be necessary
for comfort and ease of care. One example is proximal
femoral resection if a hip is painful or if hip mobility is
limited to the extent that sitting and perineal care are
affected; however, this surgery has a high complication
rate (Perlmutter, 1993).
Of particular importance is the prevention of
overuse syndromes, joint degeneration, progression of
contractures, osteoporosis, poor endurance, and
pathologic fractures. Cervical and back pain, nerve
entrapment syndromes, or tendinitis can occur as a
result of excessive and repetitive physical stresses
(Bergman, 1994; Murphy et al., 1995). Such injuries
should be treated with orthopedic therapy techniques,
as they would be in the general population. Preventive
treatment to minimize the long-term effects of the
neuromuscular dysfunction may be beneficial. This
may include use of additional mobility aids or devices,
orthoses, or surgery. Changes to the adult’s
environment may be necessary to maintain optimal
independence (Overeynder & Turk, 1998).
LIFE SKILLS
Ongoing involvement in fitness and
recreational activities should be planned. This will
provide social opportunities, as well as maintain or
improve cardiovascular fitness, weight control, and
integrity of joints and muscles; help prevent
osteoporosis; and generally promote the optimal health
that contributes to independent functioning. Fitness
clubs, swimming, wheelchair aerobics. and adapted
sports are options. Endurance continues to be an important concern. Fernandez and Pitetti (1993) found that
the values for physical work capacity of ambulatory
adults with CP were significantly lower than normal
values and concluded that adults with CP would
possibly experience fatigue before completing a normal
workday. However, physical work capacity and workrelated activities improved with training.
Technology is providing adults with CP many
options that were not previously available. These
include computers for communication, artificial speech
devices, environmental control devices, and mobility
devices. For more information, see Chapter 24.
Society is becoming more conscious of the
rights and needs of the disabled (Bickenbach, 1993).
Human rights legislation now exists to accommodate
people with disabilities and to prevent discrimination
against them in areas such as employment,
accessibiLty, the legal system, and education. Government programs and services are available to people
with disabilities. Theaters, restaurants, libraries, museums, government buildings, educational facilities,
shopping areas, parks, campground facilities, and
parking lots are becoming accessible, where possible,
through the provision of ramps, appropriate washroom
facilities, and other modifications. Air and rail travel is
also becoming more accessible to people with special
mobility needs, and there are now travel organizations
that cater to people with disabilities. In some situations,
funding for assistive devices, living allowances, and
housing and tax exemptions help prevent undue
hardship. Therapists should be aware of the facilities
available to the disabled and the political policies and
issues concerning the disabled and should advocate
their advancement.
TRANSITION PLANNING
Ideally, the planning for the transition to adult
services and lifestyle takes place before the actual
major life changes. Areas to be addressed when planning for the transition include the following: vocational
training or postsecondary educational placement,
which may range from higher education to supported
work models; living arrangements (independent, with
family, institutional, or other supportive care); leisure
and recreation (religious groups, community programs,
and recreational centers); personal management,
including birth control; social skills; and household
management. The continuation of professional health
services must also be dealt with (Fox et. al. 1992). This
includes the provision of therapy when needed,
medical consultation, primary care, and equipment
needs and maintenance. Financial planning and
education about budgeting, tax and other governmental
benefits, advocacy and legal services, guardianship,
conservatorship, wills, and trusts must be addressed
(Hallum, 1995).
RESEARCH NEEDS
Research in CP is urgently needed. The
selection of treatment for an individual with CP
requires predictive information about the effects of
interventions, if any, on the pathophysiology,
impairments, functional limitations, and disabilities of
the person throughout the life span, yet even
descriptive information is limited. To take a simple
example, to decide whether orthoses are appropriate for
a particular child, we should know whether the
impairment of hypoextensibility is preventable by
orthoses, as some studies suggest (Tardieu et al., 1988),
and, if so, under what conditions. Furthermore, we
should know if force generation capability is changed
with orthotic wear, how force output changes with
growth, and what conditions favor successful longterm
outcomes. The multisystem impairments of poor
selective control of muscles, poor anticipatory
regulation of movement, and decreased ability to learn
unique movements have received little attention.
We need better information about the relationships between measures at each level of the disabling
process. This will facilitate specificity of treatment.
There is a need for research that predicts long-term
outcomes. Finally, there are needs for specific and
sensitive measurements of all dimensions of the disabling process to be developed within a focused research program.
GLOBAL ISSUES
The treatment of children with CP varies
throughout the world. Physical therapists use many
different approaches and combinations of approaches,
depending on the facilities available, the child’s and the
family’s needs, the therapisty is training and
background, and the diversity of client values, beliefs,
and priorities. Many of the treatments and technologies
discussed are practiced in developed countries, where
services, although variable in their extent, quality, and
funding, are available and accessible. However, much
of the world’s population lives in underserviced areas,
particularly in developing nations or in remote areas of
developed nations. Often, many of the principles and
equipment ideas developed elsewhere can be adapted
to the various situations (Werner, 1987). Using
indigenous materials to fabricate effective and
affordable equipment, recycling used equipment, and
training local personnel or fostering exchange
programs can help provide resources to underserviced
areas.
It is important to be sensitive to local customs,
cultures, and environmental situations when adapting
programs for different settings. Often, the direct
application of a certain method is impractical or
inappropriate because of economic, geographic, or
cultural differences. There is an increasing emphasis on
corñmunity-based rehabilitation, which promotes
interventions that are practical and functional for the
particular settings, lifestyles, and cultures.
PROFESSIONAL ISSUES
Therapists who care for children (vith CP
must realize that the work can be physically
demanding. They must practice appropriate lifting and
handing precautions and should maintain a suitable
level of fitness if they are actively treating patients.
Working with children and their families can also be
emotionally stressful. Therapists may often be
challenged with ethical issues, unrealistic expectations
and demands, limited resources, and the pressures of
dealing with families during the grieving period and
other times of crisis. Therapists need to concentrate on
what is positive and realize that they cannot control all
the variables in their patients’ conditions and lives.
Professionals must acknowledge their own needs and
reactions and feel comfortable in seeking assistance
and support from others.
This chapter concludes with two case histories
that illustrate some of the management principles
discussed in this chapter.
CASE HISTORY
JAMIE
Jamie is an 8-year-old boy with spastic
diplegia and microcephaly. After a pregnancy
complicated by preeclamptic toxemia, labor began at
37 weeks of gestation, and an emergency cesarean
section became necessary when fetal distress occurred.
Treatment was begun in the hospital. On returning
home with their child, the parents performed routine
stretches daily, and a therapist visited every other
week. A mothers’ support group met every 2 weeks,
which provided general information and guidance, as
well as support.
When Jamie was about 1.5 years of age, the
family moved to a town in another province where
coordinated services were provided. jamie took part in
physical therapy, occupational therapy, and speech
therapy and was assessed routinely by the psychologic
services. When he began attending day care, he
received services from the same therapists, and the
parents, teachers, and therapists worked together on his
treatment.
When Jamie was 3.5 years old, the
impairment of K poextensibility of the hamstrings and
heel cords was present. Functionally, he was able to
walk independently, although he fatigued easily. Toewalking 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
times a day, the goal being to prevent further
hypoextensibility of the ankle plantar flexors.
At age 4.5 years, jamie’s gait was examined in
the Motion Analysis Laboratory at the School of
Rehabilitation Therapy at Queen’s University in
Kingston, Ontario. He walked at an average of 0.71
m/s, compared with-the normal value for his age of
about 1.00 in/s (Sutherland et al., 1980). joint
excursions were reduced (Fig. 20-11). He walked with
marked plantar flex~on of the right foot and with slight
flexion and reduced excursion of both knees, and he
had reduced power generation of the ankle plantar
flexors on both sides (A2-PF, Fig. 20-12), which was
evidence of low force-generating capability,
hypoextensibility, or both. He was compensating for
these deficiencies with larger than normal pull-off by
hip flexors on the left side (H3-F, Fig. 20-12). The
family engaged jamie in an electrostimulation program
from an outside clinic in
which the quadriceps and ankle dorsiflexors were stimulated overnight at a level below that required to
produce muscle contraction. During the next few
months, while electrostimulation and weekly therapy
were continued but the use of casts was not, jam ie’s
ankle plantar flexors became much more hypoextensible. The use of bivalved casts was reintroduced, and
the previous range was regained rapidly.
Clinical examination when jamie was 5.5 years old
revealed that his hip adductors, knee flexors, and ankle
plantar flexors were more hypoextensible; functionally,
he was \.‘alking on his toes with reduced excursions of
his ankles. Although a number of surgical alternatives
were considered, including rhizotomy, the family decided on a lengthening of the right heel cord. The goals
of gait reeducation were toward attaining initial contact
with the heel and an extended knee and achieving
effective push-off with the ankle plantar flexors. His
electrica stimulator was used in treatment postoperatively to strengthen the ankle dorsiflexors and the
surgically weakened plantar flexors. Other treatment
goals and activities included climbing and descending
stairs with one foot per stair and stepping off a stair or
curb without hand support. Because jamie had a keen
interest in sports, movement components such as
weight shifting, balancing, and eye-hand-foot
coordination and skills such as running, starting and
stopping, changing direction, and throwing were
incorporated into sports activities such as baseball,
coccer, and badminton. The improvement noted at a 6month 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
dorsiflexion during late stance reached about 150. The
knees on both sides were more extended at initial
contact and extended more in late stance than was the
case before surgery. The push-off power of the right
ankle was greater after surgery (A2-PF, Fig. 20-1 2)
than before surgery, and right hip power was more
effective (H1-E, hip extensors in early stance; H3-F,
hip flexors at pull-off; Fig. 20-12). Average walking
velocity was 0.82 m/s.jamie was able to join the
community soccer league the following summer.
Jamie attends school in a regular classroom,
although he has some cognitive limitations. He is
visited by a familiar therapist from the treatment center
every 2 or 3 weeks and attends speech therapy weekly.
The main problem the parents have encountered within
the school system is a practice of inflexibly classifying
the children according to their chronologic age only.
Although jamie is cognitively young and physically
small for his age, there has been considerable reluctance to permit him to join younger groups, even for
sports. Outside of school, jamie takes part in a nj.imber
of athletic activities. He is interested in team sports,
and the family has been pleased with the efforts of
coaches and managers to include him in organized
teams.
Update: 5 years later
Jamie is now a happy and likeable 13-year-old about
to face the challenges of adolescence. He is completing
his last year of school at the intermediate level and will
be entering a public high school next year. The family
is very happy with his education to date, and is trying
to help him prepare for the challenges of a large school
in which the children are expected to be independent.
His cognitive impairments are more limiting than his
physical situation. Still small for his age, he has not
been able to continue in team sports. His greatest joy is
karate, which he attends 3 days a week. He expects to
receive his violet belt soon. This activity has helped
him with selfdiscipline, balance, and coordination.
jamie attends physical therapy about every 3 weeks,
where he concentrates on stretching and functional
activities that are complementary to his karate practice.
About once a year, the hypoextensibility of his left calf
group is treated with about 3 weeks of serial casting.
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
NICOLE
Nicole has moderate to sev ore spastic
diplegia. She was born at 29.5 weeks of gestation after
placental separation. She weighed 1300 g and had an
Apgar score of 8 at 5 minutes. She was treated with
ventilatior for 8 hours and then.was wearied off the
ventilator onto continuous positive airway pressure,
and then to oxygen. She received phototherapy for 3
days for an increased bilirubin level and was
administered theophyliine for apnea. Nicole remained
in the neonatal intensive care unit for 6 weeks and then
went home to live with her parents and 3-year-old
sister.
Nicole was followed up at the screening clinic
for high-risk infants. At 3 months of age corrected for
prematurity, she exhibited extensor positioning of her
neck and trunk and hypertonicity was emerging in her
legs, but her family did not have any concerns. At 4
months of corrected age, these findings were discussed
with her mother, and positioning and handling recommendations were made. At 5 months of corrected age,
the diagnosis of CP was made on the basis of hypertonicity in her extremities, affecting lower extremities
more than upper; strong, persisting primitive reflex
activity; and delayed development of head and trunk
control. An ultrasound scan at this time showed the left
lateral ventricle to be slightly enlarged and the right
lateral ventricle to be at the upper limits of normal in
size. The periventricular brain parenchyma appeared
normal.
Management by a developmental pediatrician,
a social worker, an orthopedic surgeon, a physical
therapist, and later an occupational therapist was
coordinated at the local children’s treatment center.
Nicole attended physical therapy sessions weekly. The
basic therapy was based on NDT and encouraged
active control of movement and play. Positions that
reduced the influence of her extensor postuing were
used to encourage active control of movements and
functional skills. Bivalved casts were introduced early
to maintain muscle extensibility and provide optimal
alignment of her feet when she was working on
standing activities. The casts also reduced some of the
extensor posturing in her lower extremities, resulting in
improvements in her alignment in sitting and standing
and improvements in the quality of functional activities
in these positions. These casts were later replaced with
solid AFOs when growth slowed down, and Nicole was
eager to wear regular shoes. Customized seating and a
standing frame gave her a variety of positions that
offered opportunities to interact with others and use her
hands and provided some weight bearing with her body
optimally aligned.
At 2 years of age, the impairments of adductor
hypcextensibility and spasticity were treated surgically
with bilateral adductor muscle releases and anterior
obturator neurectomies. The purpose of the surgery
was to give her more functional motion at her hips and
to put her hip joints in an optimal position for acetabular development; it was also intended to avoid the
potential secondary impairment of hip subluxation or
dislocation.
During her preschool years, Nicole attended
an integrated child care program with governmentsubsidized funding for children with special needs.
This setting allowed for integration with able-bodied
children and also gave Nicole and her family
opportunities to meet other children with special needs
and their families. Nicole’s physical and occupational
therapists visited the center regularly to discuss
Nicole’s abilities, programs, handling, and equipment.
Her resource teacher was invaluable in coordinating
care, supporting the family, and adapting or acquiring
equipment.
When Nicole was 5 years of age, she was
progressing slowly in her gross and fine motor skills.
However, spasticity in her lower extremities that was
clinically apparent was affecting her ability to maintain
well-aligned postural stability, and she could not move
easily using optimal patterns of movement. These restrictions resulted in functional limitations in sitting,
standing, transitional movements, fine motor activities,
and ADL and limited her potential for independence.
Ambulation was not functional, but she could move
about independently in a power chair and had some
limited mobility in a manual wheelchair. The
prominence of spasticity and the generally good forcegenerating capabilities of her musculature prompted
the decision to have a selective dorsal rhizotomy.
After the rhizotomy, Nicole’s lower extremity
tone was greatly reduced, and Nicole participated in
daily inpatient physical therapy and occupational
therapy sessions for 8 weeks postoperatively and then
had sessions twice a week for the next year (Figs. 2013 and 20-14).
A Gross Motor Function Measure evaluation
was done preoperatively and 1 year after the surgery.
Her scores im~oved from 88 to 96% in lying and
rolling; from 78 to 87% in sitting; from 19 to 57% in
crawling and kneeling; from 13 to 32% in standing
(with AFOs); and from 7 to 10% in walking, running,
and jumping (with AFOs). She had been able to walk
10 m at 0.04 m/s preoperatively but could walk 30 m at
0.15 m/s at her 1-year follow-up. Although these
findings indicated that there had been improvements in
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 20-13 Mother and therapist Nocole,
encouragaing force generation of trunk and hip
extensors
FIGURE 20-14. Nicole, wearing ankle-foot arthoses,
doing exercises at school
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
achieved 9/33 before surgery and 1 9/33 when tested 1
year later. She was able to function better with her
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 Developmental 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 exercise 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.
educational,
governmental,
and
community
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
wheelchair. Nicole has attended the community day
camp, Brownies, and the Easter Seals family camp in
the summer.
Resources her family has found useful include
the Easter Seals Society; the Ontario government’s
As~istive Devices Program; parent support groups; and
the Community and Social Services Respite Care
Program, which provides funds for students to work
with Nicole-arid provide parent relief. The Canadian
railroad, VIA Rail, which allows wheelchair escorts to
ride free and offers reduced rates for Nicole, is useful
for traveling to out-of-town appointments.
Nicole and her family face barriers. The
family home is in the country and has a gravel
driveway. The house is not wheelchair accessible
because there are stairs into the house, stairs inside, and
narrow doorways. Thick carpeting makes manual
wheelchair pushing and walker maneuvering difficult.
When Nicole visits friends, their homes have similar
obstacles. Renovations have been made to the school
washroom, and equipment has been provided, but the
entrance doors are too heavy to open, playground
equipment is inaccessible, and classroom door handles
are difficult to turn. There is no public wheelchair
transportation in their township community. Although
obstacles are becoming less common as society
becomes more aware of accommodations necessary for
people with disabilities, barriers such as buildings
without ramps, inadequate parking, and inaccessible
washrooms still exist.
Nicole’s parents have found regular family
conferences invaluable in helping to facilitate effective
family and school involvement in therapy programs. In
these conferences, all the team members meet to
discuss short- and long-term goals. Agendas and
minutes clarify the objectives of the meetings and
document the decisions that are made. Such
conferences help empower parents and allow them to
become effective advocates for their children. They
also reinforce the philosophy of teamwork and
partnership with the therapists and the treatment center.
Nicole’s parents have found Craig Shield’s
book Strategies: A Practical Guide for Dealing with
Professionals and Human Service Systems to be
helpful (Shields, 1987).They believe that home visits
designed to deal with issues of daily routines, integrate
treatment goals into home life, and involve all family
members are important. Similarly, school visits are
important. Nicole’s parents believe that it is
particularly important not to withhold any information
from the family. They also emphasize the need for
feedback and encouragement to the family, especially
to the primary caregiver.
Update: 5 years later
Nicole is now a warm and friendly 13-yearold who will be entering high school next year. Her
parents have worked closely with teachers and
administrators of the school, where she has a half-day
educational attendant who helps with her schoolwork
and gives personal assistance. Her above average
knowledge of computer functions allows her to develop
more efficient ways of completing her school
assignments and communicating with others. Nicole
uses a taxi fitted for wheelchair transport when she
travels by herself to school and to the shopping mall.
She uses a power wheelchair in most places other than
her own home, where she uses a manual wheelchair.
For recreation, Nicole swims at a local pool, and, in the
summer, attends a camp. Like many teenagers, she uses
a chat line on the Internet, and she speaks with her
friends on the telephone.
Because Nicole had received some
professional services (notably the dorsal rhizotomy)
from a nearby city, it was difft cult for the family to
obtain a consensus of prognostic information that
would help them make decisions about their home and
lifestyle. Nicole’s mother arranged a telephone
conference between all of the relevant professionals in
the two cities and Nicole and herself which she felt was
the single most important event in the past several
years. As a result, Nicole’s family has built an addition
to their home, which makes the downstairs fully
accessible. It also has a large deck that allows Nicole to
go outdoors on her own and gives a second entrance to
the house. The driveway is paved to enable her to reach
her taxi or bus. She needs one-person assistance with
transfers, toileting, and dressing, but she showers and
washes her own hair independently using a commode
chair in a wheel-in shower. Nicole eats independently.
Nicole is seen by a physical therapist working
in the school system about once a month, and she
attends a teen group for girls organized by the
professionals of the Child Development Centre.
Stressing life skills, the meetings also provide
opportunities for the teens to discuss items of mutual
interest. Her mother thinks physical therapy may be
needed to maintain the range of moton in her
hamstrings, which is needed for upright standing
during transfers.
Nicole’s parents are very active advocates for
changes that will provide people with disabilities with
a full range of life’s opportunities. They believe parents
must be prepared to play centrr!, responsible leadership
roles within groups and agencies that can assist in these
endeavors.
ACKNOWLEDGMENTS
Acknowledgments are extended to the Child
Development Centre of the Hotel Dieu Hospital,
Kingston, Ontario; the Motion Analysis Laboratory at
the School of Rehabilitation Therapy at Queen’s
University, Kingston, Ontario; the BloorviewMacMillan Rehabilitation Centre, Toronto, On tario,
for assistance in providing data and photographs; and
to the parents of Jamie and Nicole for their support of
and contributions to the case histories.
Recommended Resources
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Medicine:
http://aacpdm.org/
United Cerebral Palsy’: http:// www.ucpa.org/
Ontario
Federation
for
Cerebral
Palsy
:http://www.ofcp.on.ca/
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