An Overview Of Cerebral Palsy And

Evolving Trends In Management

Daniel E. Shanks, M.D.
Daniel E. Shanks, M.D. is a Pediatric Neurologist with Nemours Children's Clinic,
Jacksonville.

The developmental disabilities are a group of disorders differentiated by the pattern of

delay among developmental streams. The four streams of development include language,
problem solving, motor, and social. Cerebral palsy is a disorder of motor development.
Common to the developmental disabilities, cerebral palsy is a static encephalopathy
which is further characterized as a disorder of movement and posture associated with an
abnormal pattern of persistent primitive reflexes and delayed evolution of postural
reactions and consequent motor milestones. The prevalence of cerebral palsy is
approximately 2 per 1000; however, with the increasing survival of very low birth weight
(VLBW) premature infants, the prevalence appears to be increasing slightly. The
prevalence of cerebral palsy in premature infants with birth weight less than 1500 grams
is 60 per 1000. Although the risk of cerebral palsy is increased 30 fold compared to term
infants, the vast majority of children who are VLBW do not have cerebral palsy.

This article will describe the classification of cerebral palsy, features which may alert the
primary physician when to consider the possibility of cerebral palsy, examples of
common conditions which may mimic cerebral palsy, and emerging trends in technology
which offers a greater variety of therapeutic options.

Classification

Not all children with cerebral palsy have spastic quadriplegia. The classification is based
on the predominant motor pattern. Two major categories are used in this scheme: spastic
(pyramidal) and extrapyramidal. Approximately 65% of children with cerebral palsy have
spastic cerebral palsy. The spastic group is further subdivided based on the pattern of
body involvement. Quadriplegia involves all four extremities and should be further
described if there is asymmetry in the degree of involvement among the extremities.
Hemiplegia is restricted to one side of the body with the arm typically more severely
involved than the leg. Diplegia involves the lower extremities predominantly, although
there can be minor fine motor abnormalities. This is the most common pattern in
premature infants. Occasionally a triplegic pattern is seen with a single upper extremity
spared. This likely represents hemiplegia superimposed on diplegia. On occasion a
monoplegia is seen and when in a lower extremity the differentiation from a spinal cord
etiology can be very problematic.

Extrapyramidal cerebral palsy, comprising 25% of cases, almost always represents a

quadriplegic pattern of involvement. The subdivisions of this group are based on the type
of movement disorder which predominates: rigidity, dystonia, choeroathethosis, and
ataxia. Although not a movement disorder, hypotonic cerebral palsy is included in the
extrapyramidal group. Rigidity is the most common of this group that is difficult to
differentiate from spasticity. The critical feature characterizing spasticity is velocity
dependent increase of tone. The faster the attempt to passively move the joint, the
greater the resistance. Gradual slow pressure produces movement more readily. There is
also clasp knife effect: beyond a certain amount of stretch of the muscle, a sudden
relaxation of the muscle allows movement more easily. The presence of a fixed
contracture will interfere with the attempt to elicit these characteristics. Rigidity is
characterized by consistent increased tone unrelated to speed of movement or joint
position. Table 1 describes additional features differentiating spastic and extrapyramidal
features. The final 10% of cases are classified as mixed, displaying prominent spastic and
extrapyramidal features.

The proper classification of cerebral palsy is important for diagnostic and treatment
purposes. The potential etiologies and differential diagnosis varies among the different
subtypes of cerebral palsy. The details of a comprehensive diagnostic work-up for each
type of cerebral palsy is beyond the scope of this paper. As treatment options increase,
the proper characterization of the child's motor patterns becomes more important in
selecting the most appropriate approach. Later in this paper, I will discuss the evolving
options for treating spasticity.

When To Suspect Cerebral Palsy

Motor milestones are the most commonly used features to clinically monitor for cerebral
palsy. Although these can be very informative, they are sometimes misleading. A
developmental quotient can be calculated for motor skills (MQ), just as the intellectual
quotient (IQ) reflects cognitive development:

MOTOR MILESTONES
MOTOR QUOTIENT = CHRONOLOGIC AGE X 100%
When calculating any developmental quotient in the first 24 months of age, half
correction should be given for prematurity.1 For example, a twelve month old who was
two months premature would be assigned a chronological age in the denominator of
eleven months. A MQ of 50 _ 70 % is a borderline to mild range whereas a MQ of less
than 50% is usually associated with significant pathology. The failure to sit by 10 months
or walk by 15 months should trigger consideration of further evaluation. For a
comprehensive review of early motor milestones (gross motor and fine motor) as well as
general developmental milestones, Capute and Accardo2, 3 are recommended.

Prior to twelve months of age, children should use both hands equally. The establishment
of a clear hand preference prior to the first birthday is strongly suggestive of an
abnormality of the "non-dominant" hand. Failure to use the upper extremity in the first
months (usually days) of life suggests a peripheral nerve, spinal cord, or traumatic
disorder. When an unexplained asymmetry is noted after three months (usually four to six
months), a congenital hemiplegia should be suspected.

The persistence of primitive reflexes is often the earliest clue to the diagnosis of cerebral
palsy and may allow identification in the first year of life.4 Primitive reflexes should
gradually extinguish over the first six months. Prominent fisting, obligate positive
support reflex, and obligate asymmetric tonic neck reflex are suggestive of spastic
cerebral palsy. Prominent Moro (myoclonic reflex) and tonic labyrinthine reflexes,
especially when they persist past six months, are early markers for extrapyramidal forms.
Extrapyramidal movements are not present until the second year of life and do not
necessarily represent a progression of disease, but instead a maturing to these movements
in an infant who had previously been hypotonic. Details of the diagnostic features based
on the neurodevelopmental exam can be found in review articles by Ingram5 and
Illingsworth.6

Conditions Which Mimic Cerebral Palsy

By presenting two relatively common benign conditions which mimic cerebral palsy, an
emphasis is placed on the need to remember all the features of the definition in the
diagnosis of cerebral palsy, especially in early and mild cases. Many other less common
conditions must also be borne in mind as part of the differential diagnosis for cerebral
palsy. The first of the benign conditions is characterized by normal to high muscle tone
and hyperreflexia in the first six months of life. This occurs most commonly in infants
who were premature and whose motor system may be mildly disorganized. The infant is
alert and is only mildly delayed. Prominent primitive reflexes and hard neurologic
findings are absent. In the latter half of the first year, developmental progress normalizes.

The second condition often misidentified as cerebral palsy is benign congenital

hypotonia. As the name implies, the child is floppy from birth but has no evidence of
weakness and has easily elicitable DTR's. The motor DQ is approximately 70% for gross
motor skills. Fine motor skills and other areas of development (language, social) are
normal. The hypotonia appears less prominent with age and the prognosis is good without
intervention. There is frequently a family history of a similar developmental pattern. The
critical clue in both the hypertonic and hypotonic conditions is the uniformly good
quality of skills when the gross motor skills emerge. These two groups were likely the
patients who in earlier reports were considered to have "outgrown" their cerebral palsy.

Management

The basic approach to management of cerebral palsy remains the close collaboration
among a wide range of specialties to address the often complex constellation of medical
needs many have. For more information, reviews by Dormans & Pellegrimo7 and
Batshaw8 are recommended. Batshaw9 also provides an excellent resource for parents.

Just as recent advances in technology have revolutionized many aspects of all our lives, it
offers an ever enlarging array of new interventions for individuals with cerebral palsy.
From polymers for making splints and braces, to mechanical advances allowing for better
motorized wheelchairs, to computer advances which allow for better communication
systems and academic participation, many new options are becoming available.10 It
cannot be overemphasized that in addition to medical interventions, Physical and
Occupational therapies remain a critical component in the comprehensive management of
cerebral palsy.

One area which has seen an evolution of medical options for treatment is spasticity
management. These include oral medications, intrathecal baclofen, neurolytic and
neuromuscular blockade, and surgical procedures. The most commonly used oral
medications to reduce spasticity are diazepam, baclofen, and dantrolene. Overall, oral
treatment for spasticity due to cerebral palsy has been unsatisfactory with inconsistent
and only partial improvement.11 The dose required to produce a meaningful effect usually
cannot be tolerated due to excess sedation.

Surgical options for treatment of spasticity include selective dorsal rhizotomy (SDR) and
intrathecal baclofen infusion. Selective dorsal rhizotomy is a procedure in which a
proportion of dorsal rootlets of the lumbosacral plexus are cut in order to interrupt the
afferent reflex arc from muscle spindles to the spinal cord where they stimulate muscle
contraction. The failure of the motor system from above to inhibit this reflex
appropriately is the basis for spasticity. The procedure was initially used to treat cerebral
palsy in 1913. It was subsequently repopularized by Peacock in the 1980's.12 Accurate
assessment of the child with cerebral palsy is essential, because significant
extrapyramidal involvement is a contraindication for SDR. The ideal candidates appear to
be early school age children with spastic diplegia related to prematurity who are
ambulatory and have adequate cognitive ability to participate in vigorous post-rhizotomy
physical therapy.7 Reports of efficacy are largely anecdotal. In a blinded controlled study,
McLaughlin, et al13 compared a group of children who underwent SDR and intense
physical therapy with a group receiving intense physical therapy alone. Although SDR
reduced spasticity, the functional outcome of the groups was similar.

Limitations of SDR include the uncertainty of efficacy, the small percentage of children
with cerebral palsy who are candidates, and its irreversibility. The effect of SDR also is
often not maintained without indefinite aggressive physical therapy. Intrathecal baclofen
by infusion appears to be supplanting some of the utility of SDR.

In the past five years, interest has been generated in the use of an implantable pump for
the infusion of baclofen into the intrathecal space. Although efficacy of oral baclofen is
disappointing, intrathecal administration is efficacious and well tolerated due to the
extremely low dose utilized.14 The pump is generally implanted in the lower abdomen in
a pouch which can be created in the subcutaneous tissue external to the abdominal
musculature. A catheter is then tunneled subcutaneously to the spine where it is inserted
into the subarachnoid space. The pump contains a reservoir where baclofen is stored. It
also contains a computer chip which can be programmed using telemetry to set the
infusion rate and pattern and to calculate remaining reservoir volume.

Intrathecal baclofen reliably reduces spasticity; however, the reduction in spasticity does
not reliably improve functional outcome, especially in the more functionally mobile
children with cerebral palsy. Intrathecal baclofen infusion has been more efficacious in
more severely involved children where the reduced spasticity allows for greater comfort
and makes care and positioning easier. Whether this will lead to less orthopedic
complications and need for orthopedic surgical procedures has yet to be determined.
Advantages over SDR include the ability to easily titrate the degree of tone reduction and
reversibility. Prior to implantation, a test dose administered by lumbar puncture is given
in order to determine response.

In addition to treating spasticity, intrathecal baclofen appears to be effective for dystonia,

although higher doses are required.14 A beneficial effect on upper extremity tone and oral
motor dysfunction may also be attained. As more experience is gained with intrathecal
baclofen, a wider range of indications will likely be found.

In the early 1990's, botulinum toxin became available for treatment of spasticity in
cerebral palsy. The toxin binds with the presynaptic axon terminal at the neuromuscular
junction and blocks the release of acetylcholine. Although this is the toxin responsible for
botulism, botulinum toxin is safe because the dose used is an order of magnitude smaller
than would be required to produce systemic effects. Botulinum toxin binds readily to the
neuromuscular junction and remains localized to the area of injection. Although the toxin
produces an irreversible binding to the nerve ending, duration of effect is generally three
to five months because the nerve sprouts new axon terminals. Numerous trials have
demonstrated a reduction of spasticity in cerebral palsy.15, 16 Due to the limitations of
dosing, the number of muscles which can be injected at a time is only two to three.
Combined with aggressive physical therapy and sometimes serial casting, improved
range of motion can generally be obtained. The goal is to improve range of motion and
function in a less invasive manner than would be accomplished with surgical
interventions. Fixed contractures and bony deformities do not respond to botulinum toxin
injections.

Cerebral palsy remains a very challenging disability to manage, requiring multiple

disciplines for the wide range of potential complications. A variety of new therapeutic
options are becoming available for appropriately selected patients. Unfortunately, therapy
is geared toward the secondary complications of this motor disorder. Presently, no
therapy has been proven effective for the primary brain disorders responsible for cerebral
palsy. Prevention of these disorders, when feasible, remains the only truly effective
treatment.

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March, 2000/ Jacksonville Medicine

http://www.dcmsonline.org/jax-medicine/2000journals/march2000/cerebral.htm