CEREBRAL PALSY

Definition:
A new definition in 2006 describes cerebral palsy as a “group of permanent
disorders of the development of movement and posture, causing activity limitation,
that are attributed to non-progressive disturbances that occurred in the developing
foetal or infant brain”. In addition to motor disorders, the condition often involves
disturbances of sensation, perception, communication, cognition, and behaviour;
secondary musculoskeletal problems; and epilepsy.
Incidence:
It is estimated to be 7 per 1,000 live births per year. The incidence may in
fact be increasing because many very low birth weight infants are now surviving.
Factors associated with cerebral palsy:
1. Prenatal:
✓ Maternal diabetes
✓ Rh and ABO blood type incompatibility
✓ Rubella in the first trimester
✓ Genetic or chromosomal abnormalities
✓ Congenital brain abnormality
✓ Multiple foetuses
✓ Exposure to teratogens
✓ Toxoplasmosis
✓ Cytomegalovirus
✓ Ineffective placenta causing insufficient nutrition and oxygen delivery to
foetus

2. Perinatal:
✓ Asphyxia
✓ Low birth weight
✓ Prematurity
✓ Precipitous delivery
✓ Pregnancy-induced hypertension
✓ Birth trauma
✓ Anoxia
✓ Prolonged labour
✓ Perinatal metabolic condition (diabetes)
✓ Intracranial haemorrhage.
3. Postnatal:
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 ✓ Infections
✓ Trauma
✓ Stroke
✓ Poisoning.
Pathophysiology:
A number of neuromuscular disabilities are associated with cerebral palsy.
The alteration in voluntary muscular control is related to a cerebral insult. The area
of the brain that has been injured determines the type of neuromuscular disability.
Dyskinetic palsy refers to an injury in the basal ganglia. Slow, writhing,
uncontrolled, involuntary movements involving all extremities characterized this
type.
In spastic cerebral palsy the affected area of the brain is the cortex. It is
characterized by increased deep tendon reflexes, hypertonia, flexion, and
sometimes contractures. The child’s muscles are very tense and any stimulus may
cause a sudden jerking movement.
In ataxic cerebral palsy, the affected area of the brain is the cerebellum. This
is characterized by a loss of coordination, equilibrium, and kinaesthetic sense.
Overall, the child appears clumsy.
Approximately half of children with cerebral palsy have some degree of
mental retardation and other disabilities. Other than epilepsy and mental
retardation, learning problems, poor attention span, hyperactivity, hearing or visual
loss, and emotional problems may be seen. Gastroesophageal reflux may be a
problem. Intense movements cause a high expenditure of calories, and difficulty
feeding leads to a calorie deficit.
Clinical classification of cerebral palsy
Spastic (Pyramidal)
Characterized by persistent primitive reflexes, positive Babinski, ankle clonus,
exaggerated stretch reflexes, eventual development of contractures.
✓ 70% to 80% of all cases of cerebral palsy
✓ Diplegia- All extremities affected; lower more than upper (30% to 40% of
spastic cerebral palsy)
✓ Quadriplegia- All four extremities involved: legs and trunk, mouth, pharynx,
and tongue (10% to 15% of spastic cerebral palsy)
✓ Triplegia-Three limbs involved
✓ Hemiplegia- Motor dysfunction on one side of the body; upper extremity
more affected than lower (20% to 30% of spastic CP)

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 Other features-
✓ Hypertonicity with poor control of posture, balance, and coordinated motion
✓ Impairment of fine and gross motor skills.

Dyskinetic (Nonspastic, Extrapyramidal)

Athetoid-Chorea (involuntary, irregular, jerking movements);
Characterized by slow, wormlike, writhing movements that usually involve
the extremities, trunk, neck, facial muscles, and tongue
Dystonia-Slow, twisting movements of the trunk or extremities; abnormal
posture Involvement of the pharyngeal, laryngeal, and oral muscles causing
drooling and dysarthria (imperfect speech articulation)

Ataxic (Nonspastic, Extrapyramidal)

• Wide-based gait
• Rapid, repetitive movements performed poorly
• Disintegration of movements of the upper extremities when the child reaches
for objects.

Mixed type
Combination of spastic CP and dyskinetic CP
May be labelled mixed when no specific motor pattern is dominant; however,
this term is losing favour to more precise descriptions of motor function and
affected area of brain involved.

Clinical signs and symptoms

Spastic type
➢ Increased muscle tone (hypertonicity)
➢ Increased deep tendon reflexes and clonus (sudden dorsiflexion of the ankle or
rapid distal movement of the patella resulting in alternating spasm and
relaxation of the muscles being stretched)
➢ Flexor, adductor, and internal rotator muscles more involved than extensor,
abductor, and external rotator muscles
➢ Difficulty with fine and gross motor skills
➢ Most common contracture: that of the heelcord
➢ Hip adductor contractures leading to progressive subluxation and dislocation
➢ Knee contractures
➢ Scoliosis common
➢ Typical gait crouched, intoeing, scissoring
➢ Elbow, wrists, and fingers in flexed position with thumb adductor
➢ Motor weakness of antagonist muscle groups

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Dyskinetic type
➢ Purposeless, involuntary, uncontrollable movements of face and extremities
➢ Increased movements with stress and voluntary movements, absent during
sleep.
➢ Contractures less
➢ Normal deep tendon reflexes

Ataxic type
➢ Disturbed coordination
➢ Lack of equilibrium
➢ Unsteady gait
➢ Few orthopaedic problems
➢ Hyporeflexia
➢ Loss of ability to gauge distance, speed, power of movement
➢ Muscles hypotonic
➢ Speech slurred, jerky, explosive
➢ Nystagmus common

Other manifestations
➢ Visual deficits (most common in spastic type)
➢ Hearing impairment (most common in dyskinetic type)
➢ Oral motor involvement resulting in drooling and feeding problems
➢ Developmental delay (40% to 60%; most common in spastic quadriplegia)
➢ Sensory impairment
➢ Seizures (approximately 40% of those with spastic hemiplegia affected)

Early signs of cerebral palsy

➢ Failure to meet any developmental milestones such as rolling over, raising head,
sitting up, crawling
➢ Persistent primitive reflexes such as Moro, atonic neck
➢ Poor head control (head lag) and clenched fists after 3 months of age
➢ Stiff or rigid arms or legs; scissoring legs
➢ Pushing away or arching back; stiff posture
➢ Floppy or limb body posture, especially while sleeping
➢ Inability to sit up without support by 8 months
➢ Using only one side of the body, or only the arms to crawl
➢ Feeding difficulties
➢ Persistent gagging or choking when fed
➢ After 6 months of age, tongue pushing soft food out of the mouth
➢ Extreme irritability or crying
➢ Failure to smile by 3 months
➢ Lack of interest in surroundings
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Diagnostic evaluation
❖ Careful assessment during early infancy to identify the signs of muscular
dysfunction as early as possible.
❖ The neurologic examination and history are the primary modalities for
diagnosis
❖ Neuroimaging of the child with suspected brain abnormality and cerebral palsy
❖ Magnetic resonance imaging of the brain and also of the spinal cord
❖ Metabolic and genetic testing is recommended if no structural abnormality is
identified by neuroimaging
❖ Infants with known etiologic risk factors should be monitored and evaluated
closely in the first 2 years of life.
❖ Analysis of the child’s chromosomes, including karyotype analysis and specific
DNA testing, may be needed to rule out a genetic syndrome.
❖ Electromyography (EMG) and nerve conduction studies (NCS) may be helpful
in distinguishing CP from other muscle or nerve disorders

Therapeutic management
The goals of therapy for children with CP are early recognition and
promotion of optimal development to enable affected children to attain
normalization and their potential within the limits of their existing health problems.
The disorder is permanent, and therapy is primarily preventive and symptomatic.
Therapy has five broad aims:
1. To establish locomotion, communication, and self-help skills
2. To gain optimal appearance and integration of motor functions
3. To correct associated defects as effectively as possible
4. To provide educational opportunities adapted to the child’s needs and
capabilities
5. To promote socialization experiences with other affected and unaffected
children.

Medical Treatment

While specific therapies help a child develop specific skills and abilities, the
overall goal of treatment is to help the individual with cerebral palsy reach his or
her greatest potential physically, mentally, and socially. This is accomplished with
a variety of different approaches managed by a team of professionals. Care for
people with cerebral palsy is complicated, requiring a number of different services
and specialists. In some areas, care is available through a single multidisciplinary
clinic that oversees all aspects of the child’s therapy.
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- Rehabilitation: A comprehensive rehabilitation program may include physical
therapy, use of special equipment, and spasticity treatment. Physical therapy
involves stretching, physical exercises, and other activities that develop muscle
strength, flexibility, and control. The goal is to maximize function and
minimize disabling contractures. The focus is on developing specific skills such
as holding the head up, sitting unsupported, or walking. Braces, splints, and
casts may be used to help reach these goals.

- Spasticity may be treated by injections into the muscles or by medications.

Reduction of spasticity can improve range of motion, reduce deformity,
improve response to occupational and physical therapy, and delay the need for
surgery.

- Occupational therapy: The occupational therapist helps the individual learn

physical skills he or she needs to function and become as independent as
possible in everyday life. Examples are feeding, grooming, and dressing.

- Speech/language therapy: This therapy helps the child overcome

communication problems. Many children with cerebral palsy have problems
speaking because of poor tone or uncontrolled movements in the muscles of the
mouth and tongue. Speech therapy helps develop those muscles, improving
speech. Speech therapy also benefits children with hearing loss. Children who
cannot speak may be able to benefit from communication technologies such as
a computerized voice synthesizer.

- Vision problems: An ophthalmologist is consulted for children who have

strabismus and visual problems.

- Medical therapy: This encompasses treatment for all medical problems

whether related to CP or not. Various specialists may be called upon to deal
with specific problems.

- Seizures: Seizure disorders are common in people with cerebral palsy. These
are usually well controlled with medication. A specialist in conditions of the
nervous system (neurologist) may be consulted for help in selecting an
appropriate regimen.

- Feeding and digestive problems: Individuals with cerebral palsy often have
gastroesophageal reflux or GERD as well as swallowing and feeding problems.
A team consisting of a doctor who specializes in digestive diseases
(gastroenterologist), a nutritionist, and a feeding and swallowing therapist can
assess nutritional status and treat problems. Swallowing therapy helps the child
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 eat and drink independently and helps prevent aspiration. The child’s diet must
be customized to accommodate limitations in swallowing. Children with severe
swallowing problems require feeding through a tube.

- Breathing problems: People with cerebral palsy may have breathing problems
because the muscles that control expansion and contraction of the lungs are
disabled. A specialist in lung disorders (pulmonologist) should be consulted for
management of the resulting lung disease.

- Educational services: Many children with cerebral palsy, even those of

average or above-average intelligence, are challenged in “cognitive” processes
such as thinking, learning, and memory. They can benefit from the services of a
specialist in learning disabilities. Such specialists can identify the child’s
specific learning disabilities, direct early interventions and preparation for
school, and monitor his or her progress.
Medications

The goal of drug therapy is to reduce the effects of cerebral palsy and
prevent complications. Medications are prescribed to reduce spasticity and
abnormal movements and to prevent seizures.
Medications used to relieve spasticity and abnormal movements include the
following:

- Dopaminergic drugs: Widely used in Parkinson disease, these drugs increase

the level of a brain chemical called dopamine. The effect is to decrease rigidity
and abnormal movements. Examples include levodopa/carbidopa (Sinemet) and
trihexyphenidyl (Artane).

- Muscle relaxants: These agents reduce spasticity by relaxing the muscle

directly. Examples include baclofen (Lioresal). This drug can be taken as a pill or
be administered automatically via an implantable pump.

- Benzodiazepines: These agents act on brain chemistry to relax muscles. The

most widely used of these agents is diazepam (Valium).

- Botulinum toxin type A: This substance is widely known as BOTOX. When

injected, it causes a mild muscle paralysis and reduces contractions. In cerebral
palsy, it is used to decrease spasticity of muscles of the arms or legs, which
improves range of motion and overall mobility. This can be important in allowing
a child to fit into an orthotic (brace or splint) or even to be comfortably positioned
in a wheelchair. The effects of BOTOX injections typically last 3-6 months.
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BOTOX helps other treatments work better, such as physical therapy or casting
the limb. In some cases, using BOTOX can delay surgery or make surgery
unnecessary. Some people have allergic-type reactions to BOTOX and must limit
the number of injections or stop them altogether.

Medications used to relieve seizures include the following:

Anticonvulsants: These agents stop seizure activity as rapidly as possible and

prevent seizure recurrence. There are many difference agents available; they
vary in their mechanism of action.

Benzodiazepines: Agents such as diazepam often are used to stop seizures when
they are frequent or prolonged.

Surgery

Operations used in the treatment of cerebral palsy include the following.

1. Dorsal rhizotomy: In this procedure, a few specific nerves are cut at their
roots where they branch off the spinal cord. In certain individuals with cerebral
palsy, this works very well to reduce spasticity and improve the ability to sit,
stand, and walk.
2. Implantation of a baclofen pump: The muscle relaxant drug baclofen can be
very helpful in reducing spasticity in some people with cerebral palsy, but it
works best when it is given continuously. A tiny pump can be placed in the
abdominal wall to deliver a continuous dose to the spastic muscles of the
limbs.
3. Stereotactic surgery to a part of the brain that controls muscle tone and
movement may improve rigidity, athetosis, and tremor.
4. Reconstructive surgery to an arm can restore muscle balance, release
contractures, and stabilize joints. This can improve placement of the hand in
space and the important ability to grasp, release, and pinch.
5. Skeletal problems such as hip dislocation and scoliosis can be corrected with
surgery.
6. Severe spasticity can be corrected with a number of surgical procedures,
including tenotomy, a tendon-lengthening procedure.

Other Therapy

Many children with cerebral palsy develop emotional problems, behaviour

problems, or both. They can benefit from sessions with a psychologist or
counsellor. Personal computers and related technologies offer wonderful

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opportunities for communication, social interaction, education, entertainment, and
employment for people with cerebral palsy.
Follow-up

The overall goal for ongoing care of individuals with CP is to help them
reach their full physical, mental, and emotional potential. Generally, this includes
living as much as possible in the mainstream of their society and culture. People
with CP tend to be happiest and most productive when they can go to school with,
live with, and work with their peers.
Children with CP require regular sessions with their physical, occupational,
and speech/language therapists, as well as frequent check-ups with their medical
and surgical teams. The exact schedule of visits is determined by the severity of the
child’s condition and his or her response to treatment. A multidisciplinary CP
clinic allows for frequent and complete care with the minimum of inconvenience.
Nursing management:
- The nurse should ensure the infant’s or child’s body is in the best possible
alignment, using pillows and bolsters as supports.
- Special care should be taken to protect bony prominences as they are prone to
breakdown.
- Handling and moving the child can be challenging. Caregivers as well as
therapists may assist the nurse in determining the best way to handle and move
these children.
- Feeding may prove challenging as chewing and swallowing problems are
common. Techniques such as stroking the throat may help.
- If the infant or child is hospitalized, the at-home regime should be followed as
much as possible, and physical, occupational, and speech therapy departments
should be contacted so that therapy sessions can be scheduled.
- Respect and dignity need to be integrated into all interventions.
- The diet should be tailored to the child’s activity and metabolic needs.
Gastrostomy feedings may be necessary to supplement regular feedings and
ensure adequate weight gain, particularly in the child who is at risk for growth
failure and chronic malnutrition.
- Safety precautions are implemented, such as having children wear protective
helmets if they are subject to falls or capable of injuring their heads on hard
objects.
- Appropriate immunizations should be administered to prevent childhood
illnesses and protect against respiratory tract infections such as influenza.
- Meticulous attention to all aspects of dental care is needed as dental problems
are very common in children with CP.

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- As in all aspects of care, educational requirements are determined by the child’s
needs and potential.
- Recreational outlets and after-school activities should be considered for the
child who is unable to participate in the regular athletic programs and other peer
activities.
- Any accomplishment that helps children approach a “normal” way of life
enhances their self-concept.

Family teaching- nurses must work with the families

- Providing supportive counselling directed toward understanding the
implications of the diagnosis and all of the feelings that it engenders.
- The family may require assistance in modifying the home environment for care
of the child.
- The nurse needs to support the parents in their frustration, problem solving,
concerns, approaches to helping the child, and lack of gratification, as well as
the positive approaches they use.
- Providing them with support and resources to meet their child’s needs.
- Referring them to developmental clinic or centre
- Encouraging the family how to do the exercises and follow the techniques used
by the rehabilitation specialists so their child receives consistent therapy at
home
- They may also need to learn about braces or walkers, and purchase adaptive
feeding equipment.
Prevention

Often the cause of cerebral palsy is not known, and nothing can be done to
prevent it. However, some important causes of cerebral palsy can be prevented in
many cases, including premature birth, low birth weight, infections, and head
injuries.

- Seeking appropriate prenatal care as early as possible in the pregnancy.

- Avoid using cigarettes, alcohol, and illicit drugs during pregnancy: these
increase the risk of premature delivery.
- Rubella (measles) during pregnancy or early in life is a cause of cerebral palsy.
Testing for rubella immunity before pregnant is important.
- Appropriate prenatal care includes testing for Rh factor. Rh incompatibility is
easily treated but can cause brain damage and other problems if untreated.
- Routine vaccinations of babies can prevent serious infections such as meningitis
that can lead to cerebral palsy.
- Make sure the child is restrained in a properly installed car seat and wears a
helmet when riding on a bicycle.

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Prognosis
With proper therapy, many people with cerebral palsy can lead near-normal
lives. Even those with very severe disabilities can improve their condition
significantly, although they will never be able to live independently.
Approximately 25% of children with cerebral palsy have mild involvement with
few or no limitations in walking, self-care, and other activities. Approximately half
are moderately impaired to the extent that complete independence is unlikely but
function is satisfactory. Only 25% are so severely disabled that they require
extensive care and are unable to walk. Of the 75% of children with cerebral palsy
who are eventually able to walk, many rely on assistive equipment. The ability to
sit unsupported may be a good predictor of whether a child will walk. Many
children who can sit unsupported by age 2 years eventually will be able to walk,
while those who cannot sit unsupported by age 4 years probably will not walk.
These children will use wheelchairs to move around. The likelihood of medical
complications of cerebral palsy is related to the severity of the condition.
Generally, the more severe the CP, the more likely are related conditions such as
seizures and mental retardation. Individuals with quadriplegia are much more
likely than those with diplegia or hemiplegia to have these related conditions.
Seizure disorders occur in about one third of people with cerebral palsy. Mental
retardation occurs in about 30-50% of people with cerebral palsy. Standardized
tests that evaluate primarily verbal skills may underestimate a child’s intelligence
level. Obesity is a common problem in children who are confined to a wheelchair
or are unable to move freely. Life expectancy in people with cerebral palsy also is
related to the severity of their condition. People with milder forms of cerebral
palsy have the same life expectancy as the general population. Those with severe
forms of cerebral palsy typically have a shorter life span, especially if they have
many medical complications.

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