THE CHILD WITH DEVELOPMENTAL DISABILITIES 0031-3955/93 $0.00 + .

20

CEREBRAL PALSY
Peggy S. Eicher, MO, and Mark L. Batshaw, MO

Cerebral palsy is the term used to describe a collection of nonprogressive

disorders that manifest as abnormalities of motion ,and posture and result from
a central nervous system (CNS) injury sustained in the early period of brain
development, usually defined as the first 3 to 5 years of life. 2 ! Because the
injury is central in origin, the motor involvement is commonly accompanied
by other CNS impairments, which may be cognitive, sensory, or communica-
tive. Because motor function is the earliest assessable developmental process,
cerebral palsy is usually the first identifiable developmental disability. It is
often an important marker for other developmental problems that may coexist
but are not yet manifest.
The child suspected of having cerebral palsy deserves not only an investi-
gation for etiology but, just as importantly, a comprehensive evaluation to
detect impairment in other CNS functions. This enables better directed inter-
disciplinary therapy with the goal of maximizing the child's potential. Although
by definition cerebral palsy is nonprogressive, its manifestations can change
over time, reflecting the effects of growth and development on the child. This
necessitates ongoing reassessment and monitoring to maximize function and
prevent secondary impairments. This article reviews what is currently known
about the etiology of cerebral palsy and discusses aspects of assessment and
treatment.

A CLASSIFICATION SYSTEM

Many classification systems have been proposed for cerebral palsy. The
bases of these systems included neuropathology, etiology, or clinical features.
No system has been universally accepted because none has been able to capture
the distinctions between the subtypes of cerebral palsy without becoming

From the Children's Seashore House; Department of Pediatrics, and Division of Child
Development and Rehabilitation Medicine, The Children's Hospital of Philadelphia;
and University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania

PEDIATRIC CLINICS OF NORTH AMERICA

VOLUME 40· NUMBER 3· JUNE 1993 537

538 EICHER & BATSHAW

overly cumbersome. One problem is that unlike adults, there is no specific

correlation between the brain injury and its clinical presentation, because of
the nature of the injurious agents and immaturity and plasticity of the devel-
oping brain.
We use the Swedish classification system, which divides cerebral palsy
into spastic, dyskinetic, ataxic, and mixed types. 25 The most common form,
spastic cerebral palsy, involves abnormalities of the pyramidal tract, manifesting
as increased deep tendon reflexes and increased tone with a claspknife quality.
Any voluntary muscle group theoretically can be affected, but the most common
patterns are quadriplegia (27% of all cerebral palsy), diplegia (21 %), and
hemiplegia (21 %).41 The second type of cerebral palsy is dyskinetic and manifests
as choreoathetosis with variable tone or rigidity/dystonia, in which the increased
tone has a "lead-pipe" quality. The dyskinetic form combined with the even
less common ataxic forms of cerebral palsy accounts for 13% of the cerebral
palsy population. 23 Children with both spastic and dyskinetic features account
for about 20%.14
Spastic diplegia implies minimal upper extremity involvement but spasticity
of both lower extremities. Hemiplegia indicates involvement of one side of the
body, with the upper extremity tending to be more involved than the lower
extremity. Quadriplegia means all four extremities are involved, lower extrem-
ities more so than the upper.
The dyskinetic forms are classified by type of associated movement, not
topography, because all four extremities are affected. Choreoathetosis involves
slow writhing movements, mostly involving distal muscles. The upper extrem-
ities are more involved than the lower extremities, and there is often prominent
facial grimacing and oromotor difficulties. These involuntary movements are
accentuated by emotion, position change, and volitional movement. Dystonia
indicates the maintenance of a twisted, usually extended, positioning of the
torso and extremities. Ataxia is associated with a broad based gait, truncal
titubation, and dysmetria.
Etiology may suggest the topographic" pattern. For example, premature
infants are most likely to exhibit spastic diplegia because of the location of
germinal matrix hemorrhages that involve the medially placed pyramidal fibers
supplying the lower extremities. Kernicterus results in choreoathetoid cerebral
palsy as a consequence of bilirubin deposition in the basal ganglia. Hemiplegias
may result from a traumatic brain injury localized to one cerebral hemisphere.
Finally, spastic quadriplegia implicates diffuse and severe brain damage, such
as is associated with prolonged hypoxia-ischemia.
It is important to recognize that each type of cerebral palsy has its own
course, and the clinical presentation may evolve over time. For example,
children with spastic diplegia may be hypotonic as infants, with the appearance
of increased tone later in the first year of life. Children with dyskinetic cerebral
palsy typically do not manifest choreoathetosis until the second year of life.
The classification of these children does not need to change, although the
clinical expression evolves over time.

PREVALENCE

The prevalence of cerebral palsy among children at school entry is about 2

per 1000 live births, with little variation across developed countries. 46 ,53 It is
much higher in developing countries, however. The prevalence actually has
 CEREBRAL PALSY 539

risen slightly between 1970 and 1990, and there has been a change in the
frequency of various subtypes. 36 The lack of an overall decrease in prevalence
is likely to be a function of both the large number of cases, which are attributable
to congenital brain malformations and are unresponsive to improvements in
medical care, and the improved survival of very low birth weight infants who
have an increased risk of cerebral palsy.24 With the decreased incidence of
kernicterus, choreoathetoid cerebral palsy has become much less common,
whereas spastic diplegia, associated with prematurity, has increased.

ETIOLOGY

In 1862 William John Little, an orthopedic surgeon in London, presented

his observations of a group of children with tonal and developmental abnor-
malities, which he described as "spastic rigidity." 34 Many of these children had
a history of prolonged labor or preterm or breech delivery and demonstrated
stupor or convulsions in the first days of life. Because of the frequency of these
perinatal problems, Little postulated that the motor defects resulted directly
from difficulties in the birth process. This opinion was widely held for over a
century. Yet there were early critics, chief among them Sigmund Freud, who
speculated that perinatal difficulties were the result of preexisting abnormalities
in the fetus rather than the cause of cerebral palsy. 20
The question of which came first, the brain damage or the asphyxia, was
eventually resolved by the National Collaborative Perinatal Project (NCPP). In
this study, almost 38,000 infants born between 1959 and 1966 were followed
prospectively to 7 years of age in order to determine the incidence and risk
factors contributing to the development of cerebral palsy.41 Of the children
enrolled in the study, 0.5% were diagnosed as having cerebral palsy by 3 years
of age. An association was found between cerebral palsy and both low birth
weight and severe birth asphyxia. The risk of cerebral palsy was increased 20-
fold in infants weighing 1500 g or less and 250-fold in normal birth weight
infants who had sustained severe asphyxia, defined as an Apgar score at 20
minutes of 3 or less. Many of the infants who sustained asphyxia had congenital
brain anomalies, however; asphyxia alone accounted for less than 10% of all
cases of cerebral palsy.39 This supported Freud's view that CNS abnormalities
preceded birth in most children with cerebral palsy.
It is known that many diseases and conditions can injure the developing
brain and lead to cerebral palsy. Yet, approximately one quarter of all cases
still have no definable cause. Problems during intrauterine development account
for the largest proportion of known causes of cerebral palsy.37, 46 The most
common antecedents of cerebral palsy are described later, but it is important
to emphasize that most children with these known risk factors do not develop
this disability (Table 1).

Prematurity

The risk of cerebral palsy rises steadily as birthweight declines. Data from
the NCPP showed the risk to be 3.4 per 1000 in infants 2500 g and over, 13.9
per 1000 in infants 1501 g to 2500 g, and 90.4 per 1000 in infants less than or
equal to 1500 g.39 In both the NCPP study and one from Sweden, a history of
either preterm delivery or small for gestational age was present in about 40%
 540 EICHER & BATSHAW

Table 1. CAUSES OF CEREBRAL PALSY

Causes % of Cases
Prenatal 44
1st trimester Teratogens
Genetic syndromes
Chromosomal abnormalities
Brain malformations
2nd-3rd trimester Intrauterine infections
Problems in fetal/placental functioning
Labor and delivery Preeclampsia 19
Complications of labor and delivery
Perinatal Sepsis/CNS infection 8
Asphyxia
Prematurity
Childhood Meningitis 5
Traumatic brain injury
Toxins
Not obvious 24

Adapted from Hagberg S, Hagberg G: Prenatal and perinatal risk factors in a survey of 681 Swedish
cases. In Stanley F, Alberman E (eds): The Epidemiology of the Cerebral Palsied. Philadelphia, JS
Lippincott, 1984, pp 116-134.

of children with cerebral palsy, and the presence of both risk factors was found
in almost 25%.24

Asphyxia

For premature babies a strong relationship exists between spastic diplegia

and hypoxic-ischemic induced periventricular leukomalacia (see the article by
Allen elsewhere in this issue). The influence of hypoxia on the development of
cerebral palsy is harder to isolate in full-term infants. In the NCPP, the Apgar
score was used as an indicator of perinatal asphyxia. Infants of normal
birthweight with a 5-minute Apgar score of 3 or less had a 5% probability of
developing cerebral palsy. Similar scores at 10 minutes increased the risk to
17%, and scores of 3 or less at 20 minutes were associated with a 57% risk of
cerebral palsy. Meconium passage, toxemia, and middle and high forceps use
also were associated with increased risk of cerebral palsy, but only in the
presence of a low 5-minute Apgar score. 39, 40

Prenatal Abnormalities

Children with low Apgar scores represent only a small fraction of all cases
of cerebral palsy. In the NCPP study, only 25% of full-term infants with cerebral
palsy had 5-minute Apgar scores below 7. Several investigators have suggested
that prenatal events not evident at birth may be responsible. 13,47 Paneth47 notes
that in many series of infants with cerebral palsy, there is a more frequent than
expected prenatal history of maternal disorders likely to interfere with normal
fetal nutrition/oxygenation such as abnormal uterine bleeding or placental
infarction. Furthermore, cystic degenerative lesions such as porencephaly,

-
 CEREBRAL PALSY 541

hydrencephaly, or cystic encephalomalacia have been found in children with

cerebral palsy in the absence of a history of perinatal insult. These could result
from vascular accidents or ischemic events occurring after the development of
the major cerebral structures but before parturition. 47
Congenital malformations of the brain, including neuronal migration de-
fects and cerebral dysgenesis, also can manifest as cerebral palsy, most com-
monly as spastic quadriplegia. 6 On brain imaging studies there may be lissen-
cephaly, pachygyria, or absence of the corpus callosum. These children also
may have associated facial dysmorphisms. 13

Biochemical Abnormalities

The classic biochemical abnormality causing cerebral palsy is bilirubin

encephalopathy or kernicterus. Affected children have a distinct clinical tetrad
of choreoathetoid movements, high-frequency sensorineural hearing loss, up-
ward gaze palsy, and tooth enamel dysplasia. 12 In these children there is relative
cognitive sparing and a distinct neuropathologic picture, with bilirubin depo-
sition in the basal ganglia. With the advent of Rh immunization and improved
clinical management of severe hyperbilirubinemia by exchange transfusion and
phototherapy, bilirubin levels high enough to cause kernicteric brain damage
are now rare. Bilirubin encephalopathy still occasionally occurs in infants with
hyperbilirubinemia combined with the additional risk factors of low birthweight,
acidosis, and infection.
Although hypoglycemia was previously believed to be an important risk
factor for cerebral palsy, it now seems likely to be a secondary phenomenon. 56
Infants who have suffered asphyxia, sepsis, or cerebral hemorrhage also are
likely to manifest hypoglycemia. Additionally, hypoglycemia is frequent in
premature and small for gestational age infants. Unless the degree of hypogly-
cemia is severe and the duration prolonged, however, it does not seem to lead
to the development of cerebral palsy.

Genetic Causes

Most cases of cerebral palsy are not of genetic origin. Certain subtypes are
more likely to be inherited, however. One third of ataxic cerebral palsy appears
to be inherited as an autosomal recessive disorder.22 Children with certain
neonatal onset inborn errors of amino acid and organic acid metabolism also
are at increased risk for developing cerebral palsy. For example, children with
urea cycle disorders who have recovered from hyperammonemic coma may
exhibit spastic quadriplegia,35 and children with glutaric acidemia Type 1 often
manifest extrapyramidal cerebral palsy.26, 58 Overall, the empiric risk figure of
recurrence of cerebral palsy in an affected family is about 10%.27 The rarity of
genetic origin of cerebral palsy distinguishes it from other disorders of move-
ment such as myopathies, torsion dystonia, and the muscular dystrophies,
which are all genetically inherited traits.

Environmental Toxins

Although environmental toxins have been touted as causing cerebral palsy,

few cases actually have been proven. Radiation to pregnant women, in the
542 EICHER & BATSHAW

form of the atomic bomb, was found to result in children who had microcephaly,
mental retardation, and cerebral palsy.62 Medical radiation of tumors in the
pelvis during the second trimester was found to produce similar effects. 15
Methylmercury ingestion prenatally has resulted in isolated cases of spastic
quadriplegia and mental retardation in Japan-where the exposure was derived
from fish contaminated with industrial waste-and in Iraq-where the source
was fungicide-contaminated bread. 4, 19 Cerebral palsy also has been reported in
children with fetal alcohol syndrome. In Sweden, one case of hemiplegia and
three of congenital ataxia were found among 48 children born to women with
chronic alcoholism. 44

Congenital Infections

Each of the intrauterine viral infections can cause sufficient CNS injury to
be manifest as cerebral palsy, most commonly spastic quadriplegia. Since the
development of the rubella vaccine, cytomegalovirus has become the most
common of these infections. Approximately 10% of cytomegalovirus infected
children manifest cerebral palsy, especially when hydrocephalus is present. ll

Postnatal Events

Postnatal causes of cerebral palsy account for approximately 10% of all

cases and most often occur in infancyY,47 Viral (e.g., herpesvirus) and bacterial
(e.g., Beta streptococcus) infections affecting the CNS are major risks. 61 In older
children, bacterial meningitis (most commonly meningococcal) and viral en-
cephalitis can also result in the development of cerebral palsy.4s Greater risks
in older children are traumatic brain injury, from falls from heights, motor
vehicle accidents, and assaults (see article by Michaud et al elsewhere in this
issue),

EARLY IDENTIFICATION

In high-risk infants, early detection of cerebral palsy is important to initiate

therapy (see the article by Allen elsewhere in this issue). Certain behavioral
symptoms suggest the possibility of cerebral palsy in early infancy, including
excessive lethargy or irritability; a high pitched cry; poor head control; weak
suck together with tongue thrust, tonic bite, and oral hypersensitivity; de-
creased interest in surroundings; unusual posturing; and asymmetric move-
ments.3
In later infancy, gross and fine motor development is not only delayed but'
deviant. Hands should be unclenched by 3 months, rolling over accomplished
by 4 to 5 months, and independent sitting by 7 months. Significant delays
in these milestones suggest cerebral palsy, especially if there is a dis-
crepancy between motor and cognitive skills. Deviant development includes
hand preference prior to 12 months, "bunny hopping," W sitting, and "log
rolling."
As is true of other developmental disabilities, the diagnosis of cerebral
palsy is easier to arrive at in the more severely affected child. 41 In approximately
 CEREBRAL PALSY 543

43% of cases the diagnosis of cerebral palsy is made by 6 months and in 70%
by 1 year. In some infants, it is obvious in the first weeks of life, but in more
mildly affected children, the diagnosis may not become clear until nursery
schoo!. The inverse is also true; some children who manifest signs suggestive
of cerebral palsy in infancy "grow out" of it by 2 years of age. 38. 49 This has
been especially true of premature infants whose spastic diplegia becomes
functionally insignificant by school age. It should be noted that these children
show residual language, attention, and learning deficits suggestive of persist-
ence of cortical dysfunction, however.

DIFFERENTIAL DIAGNOSIS

Because there is no specific diagnostic test for cerebral palsy, it becomes a

diagnosis of exclusion. Its principal features are that it is nonprogressive and
involves a brain-based movement disorder. Thus, it must be distinguished from
a number of other neurologic conditions, including neurodegenerative disor-
ders, lesions of the spinal cord, neuromuscular disorders, inborn errors of
metabolism, and mental retardation. s
Repeated examinations may be necessary to demonstrate that the disorder
is nonprogressive. Even then, it may not be clear cut because contractures,
dislocations, and poor nutrition may contribute to a picture of "pseudode-
generation" in certain instances. Yet a number of generalizations about differ-
ential diagnosis can be made. Weakness and decreased deep tendon reflexes
are found in neuromuscular disorders. Loss of cognitive skills together with
motor abnormalities suggest a neurodegenerative process, for example, Lesch-
Nyhan syndrome with choreoathetosis, ataxia telangiectasia with progressive
ataxia, and leukodystrophies with quadriplegia. Intermittent episodes of vom-
iting and lethargy suggest an inborn error of amino acid or organic acid
metabolism. Finally, mental retardation alone may be associated with motor
deficits, but these are usually no more severe than the cognitive deficits.
Overall, infants having a neurologic condition masquerading as cerebral palsy
usually present with a confusing clinical picture that is incongruous with the
topography and physiology of cerebral palsy.

ASSOCIATED DEFICITS

In addition to problems with movement and posture, almost all children

with cerebral palsy have at least one additional disability associated with
damage to the CNS.31 The most common of these associated deficits are
cognitive impairments, sensory deficits, communication disorders, seizures,
feeding problems, and behavioral or emotional problems. In general, the more
limbs involved, the greater the degree of spasticity and the higher the likelihood
of associated deficits and cognitive impairment.
Approximately two thirds of children with cerebral palsy have mental
retardation. Among those with normal intelligence, most have perceptual
impairments that place them at risk for learning disabilities. Outcome is
somewhat correlated with topography. Children with hemiplegia have the best
intellectual outcome, with more than 60% having normal intelligence. Less
than 30% of children with spastic quadriplegia or mixed-type cerebral palsy
have normal intelligence. Among those children with cerebral palsy and mental
544 EICHER & BATSHAW

retardation, approximately 15% have mild retardation, 35% moderate retarda-

tion, and 50% severe retardation. 52
Sensory deficits include both vision and hearing problems. Visual deficits
include refractive errors, field defects, amblyopia, nystagmus, and abnormalities
of visual pursuit. Strabismus is found in more than half of all children with
cerebral palsy. 57 Other defects often relate to the underlying etiology. For
example, premature infants are likely to have residua of retinopathy of pre-
maturity, ranging from myopia to blindness, and children with hemiplegia may
have homonomous hemianopsia. Hearing loss occurs in about 10% of children
with cerebral palsy, predominantly in those whose disability derives from
prenatal or postnatal CNS infections! Communication disorders in children
with cerebral palsy include speech motor deficits and central processing
problems. Speech motor problems, in the form of dysarthria, are common in
extrapyramidal cerebral palsy.50 Expressive and receptive language disorders
often parallel the cognitive impairments in affected children.
Growth is often stunted in children with cerebral palsy, and, in hemiplegia,
there may be growth arrest of an affected limb. There also may be superimposed
failure to thrive. Feeding problems may result from various motor problems
including hypotonia, weak suck, poor coordination of the swallowing mecha-
nism, tonic bite reflex, hyperactive gag reflex, and exaggerated tongue thrust. 3()
There may be drooling or aspiration resulting from the oromotor problems.
Gastroesophageal reflux also may be present. In addition, the child may be
unable to independently access food or communicate hunger. Finally, consti-
pation may interfere with the child's willingness to eat. If unattended, these
problems lead to poor nutrition and may require the use of alternate feeding
methods, such as tube-feeding. 51
Approximately one third of children with cerebral palsy develop a seizure
disorder. 2 Seizures have been noted to be most frequent in spastic hemiplegia,
ranging from 55% to 72%, and least common in choreoathetosis and ataxic
forms (23%)." Most seizures manifest by 2 years of age. As in the general
population, tonic-clonic and partial complex seizures predominate.
Finally, behavioral and emotional problems may develop. In high function-
ing children, the most common behavioral symptoms are attentional deficits
and impulsivity; the diagnosis of attention-deficit hyperactivity disorder
(ADHD) is appropriate in some cases. During adolescence there may be
emotional lability, depression, dependency, and low self-esteem. 29 There are
also concerns about sexuality, independence, vocation, and socialization. Coun-
seling may be indicated to address these issues, or pharmacotherapy may be
used to treat ADHD or depression. In children with severe retardation there
may be stereo typic or self-injurious behavior. This may respond to behavior
management techniques or medication (see the article by Parrish elsewhere in
this issue).

ASSESSMENT

Because of the multitude of problems related both to the motor impairment

and associated deficits, an interdisciplinary approach to treatment is essential. 2 !
This approach requires the talents of both medical specialists and allied health
professionals. A typical team in a cerebral palsy clinic includes a neurodevel-
opmental pediatrician, orthopedist, physical therapist, occupational therapist,
speech pathologist, and social worker. Prior to intervention, an assessment is
 CEREBRAL PALSY 545

completed by the various team members. This is usually followed by a case

management conference, during which various intervention strategies are
discussed. Follow-up is generally arranged at 3- to 6-month intervals in young
children and yearly in older school-aged children.
The concern that brings most parents to a cerebral palsy clinic is abnormal
tone or motor delays. The pediatrician's initial role is to attempt to make a
diagnosis, determine an etiology, and identify neuromotor and cognitive
abnormalities. The etiologic search starts with the pregnancy and birth, seeking
a history of bleeding, infection, or factors that may have led to brain injury in
the fetus or neonate. A family history, identification of intercurrent illnesses or
accidents, and description of developmental milestones are also important. This
may help detect an etiology as well as identify the age of onset of the
developmental delay and its progression.
The physical examination should include observations of the child in prone
and supine positions. Dysmorphisms and congenital anomalies should be
identified. Special attention should be paid to the head, hips, and spine as well
as to the neurodevelopmental examination, focusing on tone, deep tendon
reflexes, primitive reflexes, and functional skills (see the article by Levy and
Hyman elsewhere in this issue).
In pyramidal cerebral palsy, the motor signs are persistent and consistent.
Tone is increased in a clasp-knife fashion; on flexion or extension of an affected
limb, there is initial resistance to stretch followed by a sudden giving way.
Because of the persistent tonal abnormalities, contractures frequently develop
over time, most commonly at the hips, knees, and ankles. Deep tendon reflexes
are markedly increased, usually with sustained ankle clonus. There are also
pathologic reflexes such as the Babinski and Chaddock signs. Additionally,
there is persistence of primitive reflexes. These are normally found in infants
during the first 3 to 6 months of life but are subsequently suppressed by
increased cortical activity. Common primitive reflexes include the Moro, asym-
metric tonic neck reflex, tonic labyrinthine response, and positive support. s In
the child with cerebral palsy these reflexes are not only persistent but often
obligatory, that is, the child is unable to independently get out of the reflexive
posture.
In dyskinetic cerebral palsy, signs are more variable, changing with tension,
sleep, and volitional movement. Tone may be increased or decreased; there
may be "lead pipe" rigidity or less commonly, dystonia. In rigidity there is a
constant but malleable increase in tone that can be briefly "shaken out" by
rapidly flexing and extending the affected limb. Because of the variability of
tone, contractures are less common. Whereas spastic cerebral palsy may be
asymmetric, extrapyramidal forms are usually symmetric. Deep tendon reflexes
may be normal or increased, and there is usually no clonus or Babinski sign.
Primitive reflexes may be more prominent and persist longer than in spastic
cerebral palsy.
In addition to the physical examination, a neurodevelopmental assessment
also should be performed (see the article by Levy and Hyman elsewhere in
this issue). Observation is informative regarding a child's motor and cognitive
function and is often best done before the child's clothes are removed or much
handling has occurred.
Depending on the history, symptoms, and physical findings, an investi-
gative examination may include an electroencephalogram (EEG), electromy-
ogram (EMG), brain imaging study, or metabolic investigation. These studies
are only indicated in the pursuit of specific etiologies, however, and should
not be used as a screen. EEG would be performed if symptoms suggest seizure
546 EICHER & BATSHAW

activity. EMG may help differentiate a myopathy from cerebral palsy. Neuroim-
aging studies may reveal specific brain abnormalities, for example, cortical
atrophy in children with spastic diplegia 59 and in children with hemiplegia. to
Formal hearing and vision tests also should be performed to document sensory
impairments, and a formal ophthalmologic examination may be appropriate
(see the article by Menacker elsewhere in this issue).
Psychological testing should be done to assess intellectual function (see
the article by Batshaw elsewhere in this issue), although this may prove difficult
because of motor and communicative deficits. Infant intelligence tests, which
principally rely on visual and motor activity, may give an inaccurate picture,
and testing in older children may be compromised by speech motor and
language problems. Certain nonverbal intelligence tests, such as the Leiter
scales, may be appropriate. Speech and language, occupational therapy, and
physical therapy evaluations help to document these deficits and suggest
compensatory strategies for testing cognitive function as well as designing a
treatment program.
An assessment of the functioning of the family by social work is also
important. Like news of any developmental disability, giving the diagnosis of
cerebral palsy to a family can be devastating. It is all the more difficult because
of the lack of a clear understanding of etiology in many cases and the difficulty
in predicting functional outcome. Family coping must be addressed from the
beginning in order for the parents to be an effective part of the therapy program
(see the article by Thomas et al elsewhere in this issue).

APPROACHES TO INTERVENTION

Once the assessment is completed an intervention strategy can be devel-

oped. The goals are to improve function, develop compensatory strategies, and
encourage independence. From the beginning it is essential to involve the
family and the external agencies that will carry through the suggested program.
This usually implies family meetings and contacting the school system, health
department, social service agency, and early intervention program.
Therapy may include the use of medication for seizures, spasticity, consti-
pation, or gastroesophageal reflux. Antiepileptic medication is used under the
same guidelines as for other children with seizure disorders and is generally
effective. The most commonly used antispasticity drugs are the benzodiaze-
pines, dantrolene,33 and baclofen (see the article by Mercugliano elsewhere in
this issue). They have had only limited benefit; although they may decrease
spasticity, they do not improve incoordination. Furthermore, side effects,
including lethargy, increased salivation, and stomach upset, may interfere with
other aspects of the therapy program. An innovative approach has been the
use of intrathecal baclofen in severe spasticity in adults; it has not been tested
in children with cerebral palsy. 55 Antireflux medication may be needed if there
are feeding problems associated with gastroesophageal reflux. The most com-
monly used medications are metoclopramide to increase gastric emptying and
cimetidine or ranitidine to decrease stomach acidity. A medical regimen to
decrease constipation, including adding fiber to the diet and using stool
softeners and laxatives, also may be helpful.
The principal approaches to treating abnormalities of tone and posture are
occupational and physical therapy (see the article by Kurtz and Scull elsewhere
in this issue). The occupational therapist generally focuses on developing

-
 CEREBRAL PALSY 547

approaches to oral motor function, visual-perceptual problems, and activities

of daily living. The physical therapist is involved in the development of posture
and ambulation, including the use of adaptive and seating devices. In addition
to these formal therapies, exercise and sports are important to strengthen
muscles and bones and enhance motor skills. Participation in sports also adds
a recreational aspect to therapy, is enjoyable, and enhances self-esteem.
Swimming, dancing, and horseback riding are examples of sports that can be
modified to permit children with cerebral palsy to participate. 32
The speech therapist provides speech and language therapy if communi-
cation deficits exist. This may include the use of computers with microswitches
to permit augmentative communicationY The speech therapist also may be
involved, together with the occupational therapist and nutritionist, in devel-
oping an effective feeding program. This may include adapting positioning
techniques and special seating apparatus and modifying the texture and
quantity of feedings. 2s They also may work on excessive drooling, using
behavior management or biofeedback approaches. '6 If oral approaches to
nutrition are ineffective, placement of a gastrostomy feeding tube may be
required. Using special formula feedings this approach has resulted in signifi-
cant weight gain and improved general health and lifespan in severely affected
children. 30
While these therapy programs are ongoing, there is also the need for
periodic consultation by a number of medical specialists. The orthopedic
surgeon examines the child for scoliosis, contractures, and dislocations that
may require bracing, serial casting, or corrective surgery (see the article by
Dormans elsewhere in this issue). The orthopedic surgeon also may prescribe
adaptive equipment such as sidelyers, prone boards, and supine standers to
promote skeletal alignment, to compensate for abnormal postures, or to prepare
the child for independent mobility. Mobility devices such as walkers, scooters,
tricycles, and wheelchairs may be needed. Splints or braces, designed by an
orthotist, may be prescribed to maintain range of motion, prevent contractures,
provide stability, or control involuntary movements (see the article by Kurtz
and Scull elsewhere in this issue).
Neurosurgeons also may become involved, especially in certain cases of
spastic diplegia in which the use of selective dorsal rhizotomy has been
advocated to decrease spasticity. 1 This procedure involves isolating the posterior
roots from the second lumbar to first sacral spinal nerves and providing selective
electrical stimulation to discover which rootlets make the greatest contribution
to the spasticity and then cutting them. This is still considered an experimental
procedure.
It is important to develop the treatment plan together with family members.
If they do not agree with the plan, or are financially or emotionally unable to
follow through with it, the entire program will fail. If possible, the therapeutic
program should be carried out in the home for infants as part of an early
intervention program. 45 Federal law (PL 99-457) mandates the provision of these
free services to high-risk infants. The problem is inadequate funding to meet
the needs and resultant long waiting lists. In the interim, therapy services near
the home should be arranged. In school-aged children, therapy should be
provided as part of the school program as mandated by law (PL 94-142). In
adolescence, prevocational training should be started.
Even once an effective treatment program has been initiated, there is the
need for periodic reassessment. As the child moves from preschool to school
age, therapy must shift from being directed at increasing motor development
and minimizing contractures to helping the child function in school. The
548 EICHER & BATSHAW

educational placement should be based on cognitive abilities. Other consider-

ations such as developing a proper seating apparatus and permitting enhanced
mobility with appliances or a wheelchair need to be addressed. A word
processor with a microswitch may need to be used in place of handwriting, or
an augmentative communication system may need to be developed. Activities
of daily living, for example, dressing, feeding, and toileting, also must be
addressed.

Outcome

More than 90% of children with cerebral palsy live to adulthood, although
the life expectancy of severely involved individuals is significantly less than
that of the general population. '8 The outlook varies somewhat with the topog-
raphy of the cerebral palsy. The more limbs involved, the worse the prognosis.
Children with spastic diplegia and hemiplegia do relatively well, whereas those
with choreoathetosis and quadriplegia do less well. 60 Virtually all children with
hemiplegia or ataxia are ambulators. In general, if a child has not walked by 6
years, he or she is unlikely to walk independently.
Eventual employability seems to depend principally on intellectual function
and severity of the cerebral palsy. Associated deficits, family support, quality
of educational programs, and availability of community-based training and
technical support also can influence outcome, howeverY, 54 Aggressive and
earlier orthopedic surgery, improved adaptive equipment, and computers all
have increased the potential for independent functioning. Despite these im-
provements, the percentage of individuals with cerebral palsy living and
working independently remains low. Yet, it seems likely that with improved
treatment techniques and protection under the Americans with Disabilities Act,
the next generation of individuals with cerebral palsy will have a more
independent outcome.

SUMMARY

Over the last century, our understanding of cerebral palsy has broadened.
For example, we now know that it results more commonly from prenatal
abnormalities than from perinatal difficulties. Yet, in most cases we are still no
closer to understanding the operant mechanism of injury or how the injury
results in the expressed motor disorder. Hopefully, the strides being made in
neurodevelopmental physiology and neurotransmitter communication will help
elucidate the mechanism of injury in cerebral palsy and thereby lead to methods
of prevention. Meanwhile, comprehensive clinical evaluation and treatment
and periodic reassessment will help tailor strategies to the individual needs of
the child. This should enable the child with cerebral palsy to optimize his or
her function in society.

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Peggy S. Eicher, MD
Children's Seashore House
3405 Civic Center Boulevard
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