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Cerebral palsy
Introduction:
Cerebral palsy (CP) is the most common chronic disability of
childhood today all over the world. In developed nations, the incidence is
about 1 - 2 per 1000 live-births. In spite of the improved obstetrical and
perinatal care, CP remains. As a result of injury to the developing brain,
these children will have motor deficits which will affect them for their entire
lifetime. Treatment often starts when they are infants and continues
throughout their life, even into adulthood.
The problems involved are complex. Not only do these children have
problems of mobility but they can also have seizure disorders,
gastrointestinal problems, learning and perceptual difficulties, visual and
hearing problems and growth deficiency.
Cerebral palsy cannot be cured with the present knowledge but life
can be made better for these children. Even small degrees of improvement
make a great difference. Getting a child to walk, be in crutches, in braces or
with a walker is better than having him in a wheelchair. Having a child be
able to live in a wheelchair, as is true for children with total body
involvement, is better than having him be on a stretcher or in a bed for the
rest of his life. These are important things to consider.
Definition:
Cerebral palsy has been defined as a non-progressive injury to the
immature brain leading to motor dysfunction. Although the lesion is not
progressive, the clinical manifestations change over time.
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Cerebral palsy is a disorder of movement and posture that appears
during infancy or early childhood. It is caused by non-progressive damage to
the brain before, during or shortly after birth. It is not a single disease but a
name given to a wide variety of static neuromotor impairment syndromes,
occurring secondary to a lesion in the developing brain.
The damage to the brain is permanent and cannot be cured but the
consequences may be minimized. The lesion in the brain may occur during
the prenatal, perinatal or postnatal periods. Generally, any non-progressive
central nervous system injury occurring during the first 3 years of life is
referred to as “cerebral palsy” (CP).
Epidemiology:
Cerebral palsy is the most common cause of childhood disability in
the world. The incidence is 2 - 2.5 / 1000 live-births. Some affected children
do not survive and the prevalence varies between 1 - 5 / 1000 babies in
different countries. It was previously thought that improvements in perinatal
and obstetric care would decrease the incidence of CP. However, the
incidence has not declined and the overall prevalence increased during the
1980s and 1990s. This is explained by increased survival of prematures and
very low-birth-weight infants and by a rise in the number of multiple births.
Etiology:
The etiology can be identified only in 50 % of the cases. Certain
factors in the history of the child increase the risk of CP. The incidence of
CP among babies who have one or more of these risk factors is higher than
those among the normal population. The clinician should therefore be alerted
to the possibility of the presence of CP in a patient having these factors.
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Risk factors:
Risk factors associated with CP are grouped into prenatal, perinatal
and postnatal factors. Prematurity and low birth-weight is the two most
important risk factors in the developed countries with high standards of
obstetrical care. Postnatal risk factors additionally play a major role in other
countries. Rubella and toxoplasmosis cause severe effects on the developing
CNS. Severe maternal illnesses and hypothermia or hypoglycemia of the
neonate causes a reduction in the levels of oxygen and nutrients available to
the fetus. Breech presentation also can increase the risk.
Pathological findings in the CNS:
Specific brain lesions related to CP can be identified in most of the
cases. These lesions occur in regions that are particularly sensitive to
disturbances in blood supply, called “hypoxic ischemic encephalopathy”.
Clinical findings:
Children with CP present with three types of motor problems.
“Primary impairments” of muscle tone, balance and strength selectivity are
directly related to damage in the CNS. “Secondary impairments” of muscle
contractures and deformities develop over time in response to the primary
problems and musculoskeletal growth. “Tertiary impairments” are adaptive
mechanisms and coping responses that the child develops to adapt to the
primary and secondary problems. One typical example is gastrocnemius
spasticity as a primary impairment, leads to secondary ankle plantar flexion
contracture and knee hyperextension in stance as an adaptive mechanism.
CP children have abnormalities of muscle tone and reflexes, showing delay
in developmental milestones with posture and movement problems.
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Classification:
Clinicians classify patients to describe the specific problem, to predict
prognosis and to guide treatment. Classification is based on the change in
muscle tone, anatomical region of involvement and severity of the problem.
Classification provides a clearer understanding of the specific patient and
directs management.
* Spastic CP:
“Spasticity” is defined as an increase in the physiological resistance
of the muscle to passive motion. It is a part of the upper motor neuron
syndrome characterized by hyperreflexia, clonus and primitive reflexes.
Spastic CP is the most common form of CP; approximately 70 - 80 % of
children with CP are spastic, anatomically distributed into three types.
- Hemiplegia: In hemiplegia, one side of the body is involved with the upper
extremity generally more affected than the lower.
- Diplegia: In diplegia, the lower extremities are severely involved and the
arms are mildly involved. A history of prematurity and low birth-weight is
common causes of this type.
- Quadriplegia: In quadriplegia, all four limbs, the trunk and muscles that
control the mouth, tongue and pharynx are involved. The total body
involvement may be termed as “Tetraplegia”.
* Dyskinetic CP:
It shows signs of extra-pyramidal involvement with involuntary
movements (athetosis, chorea and dystonia). Dysarthria, dysphagia and
drooling accompany the movement problem. Mental status is generally
normal. “Dyskinesia” accounts for approximately 10 - 15 % of all cases of
CP. Hyper-bilirubinemia or severe anoxia causes basal ganglia dysfunction
and results in dyskinetic CP.
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*Ataxic CP:
It shows signs of cerebellar involvement. “Ataxia” is loss of balance,
coordination and fine motor control. Ataxic children cannot coordinate their
movements. They are hypotonic during the first 2 years of life. Muscle tone
becomes normal and ataxia becomes apparent toward the age of 2 to 3 years.
Children who can walk have a wide-based gait and a mild intention tremor
(dysmetria). Dexterity and fine motor control is poor. Ataxia is associated
with cerebellar lesions.
* Hypotonic CP:
There is an often severe depression of motor function and weakness.
* Mixed CP:
Children with a mixed type of CP commonly have mild spasticity,
dystonia and / or athetoid movements. Ataxia may be a component of the
motor dysfunction in patients belonging to this group. Ataxia and spasticity
often occur together. “Spastic ataxic diplegia” is a common mixed type that
often is associated with hydrocephalus.
Associated problems:
A number of associated problems occur which increases with severity
of the disease. Cortical blindness, sensory loss, deafness, mental retardation
and epilepsy are primary impairments, while malnutrition, psychosocial
problems and intellectual impairment may accompany. Language and
cognitive disturbance lead to mental retardation and learning disability.
Mental retardation accompanies 30 - 65 % in all cases of CP. It is most
common in spastic quadriplegia.
- Epileptic seizures: Seizures affect about 30 - 50 % of patients. They are
most common in the total body involvement and hemiplegic patients.
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- Respiratory problems: Aspiration in small quantities leads to pneumonia in
children who have difficulty in swallowing. Premature babies have bronchopulmonary dysplasia, which leads to frequent upper respiratory tract
infections. Respiratory muscle spasticity contributes to the pulmonary
problems.
- Bladder and bowel dysfunction: Loss of coordination of bowel and bladder
sphincters results in constipation and / or incontinence. Enuresis, frequency,
urgency, urinary tract infections and incontinence are common problems.
Common musculoskeletal problems in different types:
- Hemiplegia:
“Hemiplegia” is the involvement of the arm and leg on one side of
the body. The upper extremity is more severely involved than the lower.
Spastic hemiplegia constitutes 20 % of cases with spastic CP. These children
generally have very few associated problems. Communication is unimpaired
most of the time; they may have seizures, learning and behavioral problems.
Functional prognosis is good compared to other types because one side of
the body is normal. All hemiplegic children learn to walk by the age of three
years. They become independent in the activities of daily living. Seizures,
mild mental retardation, learning difficulties and behavioral disturbances
may complicate the management and integration into the society.
The shoulder is adducted and internally rotated, the elbow is flexed
and pronated, the wrist and fingers are flexed and the thumb is in the palm.
The hip is flexed and internally rotated, the knee is flexed or extended and
the ankle is in plantar flexion. The foot is generally in varus although valgus
deformity may also be seen. The hemiplegic side is short and atrophic,
depending on the severity of involvement.
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- Diplegia:
“Diplegia” is defined as gross motor involvement of the lower
extremities and fine motor involvement of the upper ones. It constitutes 50
% of the spastic CP population. The main problem in spastic diplegia is
walking difficulty. Balance disturbance, muscle weakness, spasticity and
deformities result in abnormal gait pattern, typical for diplegic children.
Abnormal gait increases energy consumption, causing fatigue. Most
diplegic children start cruising at 2 years of age and walk by the age of 4.
Neuromotor function improves until the age of 7. Children who cannot walk
by then in spite of appropriate treatment usually become limited walkers.
Among all types of CP, diplegic children benefit most from treatment
procedures. Unlike hemiplegic children, they cannot reach their potential if
left untreated. Every effort is worth spending when treating a diplegic child.
- Quadriplegia:
“Quadriplegia” is the involvement of the neck, trunk and all four
extremities. Quadriplegics have severe motor impairment and other signs
and symptoms of CNS dysfunction such as cognitive impairments, seizures,
speech and swallowing difficulties. Some call this total body involvement
(tetraplegia) because the trunk, neck and oro-facial muscles are affected as
well as the extremities. Prognosis is the worse. The spectrum of severity is
variable, from having no sitting ability or head control to being able to walk
with extensive assistive devices.
The majority of quadriplegics cannot be independent and need
lifelong, all day assistance in daily life. They are wheelchair bound; the
survivors face the late complications related to hip instability and spinal
deformity. Spinal and hip deformities such as hip instability, pelvic obliquity
and scoliosis are very common and interfere with sitting balance.
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- Dyskinesia:
“Athetosis, dystonia and chorea” are the main movement disorders
seen in dyskinetic children. These children are initially hypotonic but as they
get older, muscle tone begins to fluctuate. Involuntary movements occur
when the child tries to move. Sometimes, involuntary movements may also
occur at rest. When the child is totally relaxed in the supine position or
asleep, there is full range of motion and decreased muscle tone. When the
child wakes up or is excited, he becomes rigid. Lack of coordination is even
more prominent during strenuous activities. The dyskinetic child spends
excessive energy because of the continuous uncontrolled movements.
Abnormal contractions of many muscles occurring with the slightest
voluntary motion increase the energy demand considerably.
Physical examination
History:
History is a key component in evaluating the child as it provides
valuable information for diagnosis. In children with a definite diagnosis, the
timing of achievement of developmental milestones and the presence of
associated impairments help to decide a functional prognosis.
Clinical Examination:
Observing the child’s movements is the initial and most crucial part of
the examination. The principal rule is “Observe before you touch”. If the
child is young, apprehensive or tearful, let him or her stay on the mother’s
lap while you watch and talk to the mother. As the child adapts to the
environment, slowly place him on the mat and watch him moving around. If
the child cries a lot and does not cooperate, continue let him in mother’s lap.
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Neurological examination:
Neurological evaluation of the infant and the child requires adequate
knowledge of neurological developmental stages, which include assessment
of motor development and reflexive maturation.
* Reflexes:
The persistence of primitive reflexes and the absence of advanced
postural reactions should be evaluated. The presence of primitive reflexes
beyond 6 months of age is a sign of poor prognosis.
* Muscle tone and involuntary movements:
The child must be calm for assessment of muscle tone. The head
should be placed in the neutral position because turning or flexion can
trigger tonic neck reflexes and interfere with muscle tone. “Spasticity” is the
resistance felt while moving the joint through a passive range of motion. The
“modified Ashworth or Tardieu scales” are utilized to grade spasticity.
Tremor, chorea, athetosis, dystonia and ataxia should be also thoroughly
noted.
* Muscle strength and selective motor control:
Many children with CP cannot voluntarily contract or relax their
muscles in isolation; therefore they are unable to move their joints
separately. For example, when the child attempts to extend his elbow, he
involuntarily moves his whole arm. Lack of selective motor control makes it
impossible to determine muscle strength using simple manual muscle
testing. Muscle strength can be observed by watching the child during
performing certain tasks, such as throwing or hitting a ball.
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Musculoskeletal examination:
This examination reveals contractures and deformities that interfere
with mobility. The examination is performed in a comfortable room with
adequate space and props to attract the child’s attention.
* Range of motion:
ROM test must be done in a slow and smooth manner. Sudden stretch
of the muscle increases spasticity, creating the false impression of a fixed
joint contracture.
* Balance:
Balance and equilibrium reactions are prerequisites for walking.
Balance should be evaluated in all children by pushing the standing child
gently from the front, back and side to determine whether he can promptly
regain balance.
* Mobility:
A crucial part of the examination is the observation of the child’s
walking pattern. Photographs or video recordings of their child are useful to
understand how the child functions at home. Computerized gait analysis is
possible in advanced centers. The non-ambulatory child is placed on the
floor to assess his mobility (rolling, creeping or crawling).
* Gait:
Some children with CP cannot walk, while others have walking
difficulty. This is generally the basic reason for seeking medical advice in
CP and probably one of the most difficult to affect. To understand the gait
pathology associated with CP, normal gait should be firstly understood.
Walking is one of the most important functions of the human
musculoskeletal system. Efficient walking requires complete coordination of
the brain, spinal cord, peripheral nerves, muscles, bones and joints.
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Physical treatment
The clinical picture ranges from very mild to very severe depending
on the extent of the CNS lesion. This wide spectrum of clinical findings
makes it difficult to predict prognosis. Predicting prognosis forms the basis
of management. The parents want to know about the prognosis of their child
in order to shape their lives in the following years. The physicians and
therapists want to know about the prognosis in order to make sound
treatment decisions.
Treatment team:
Much impairment, associated with the primary neurological lesion,
accompany the motor disorder seen in CP. A large team of experts who
work together for treatment is necessary because the impairments vary, both
in severity and in character. The team consists of physicians, surgeons and
allied health professionals such as physical therapists, occupational
therapists and child development professionals, who apply various treatment
procedures. The team must work together in harmony for success. The
members must be aware of all the needs and limitations of the child and
know what each should do for the child.
The ultimate goal in the management is to minimize disability while
promoting independence and full participation in society. All efforts have to
be directed toward gaining independence in activities of daily living, ability
to go to school, earn a living and a successful integration with the
community.
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Treatment strategy:
Everyone is a child only once. Childhood should not be sacrificed for
therapy, so make the child live a childhood that is as close to normal as
possible. Treatment strategy should be based on a realistic evaluation of the
child’s present functional status and possible future prognosis. Goals must
be set for each child, to be explained to the family. The child has to
participate in the goal-setting process when he is old enough.
Treatment should be individualized for each child. The strategy
depends also on the child’s age. Family education, addressing the specific
needs of the infant, providing adequate support for optimal growth and
development are priorities in management.
Rehabilitation:
Rehabilitation is the name given to all diagnostic and therapeutic
procedures, aiming to develop maximum physical, social and vocational
function in a diseased or injured person. The goal of rehabilitation is to gain
independence in activities of daily living, school or work and social life.
This is possible to the extent of the person’s impairments. CP rehabilitation
consists of physical therapy, occupational therapy, bracing, assistive devices,
adaptive technology and sports.
* Physical therapy:
Physical therapy helps to improve mobility. It is the basic treatment in
all children with CP. It consists of exercises, bracing and activities towards
reaching specific functional goals. It aims to bring the child to an erect
position, give the child independent mobility and prevent deformity.
Physiotherapy should be organized to fit into the family’s lifestyle.
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Therapeutic exercises help the child learn how to sit, stand, walk and
use his upper extremity for function. The child also learns how to use his
remaining potential to compensate for the movements he cannot perform.
Decreasing spasticity, gaining muscle strength and improving joint
alignment decrease deformity. Parents should encourage their children to
participate in daily living activities by using the functional skills they
learned during therapy.
General principles of physical therapy:
Physiotherapy begins in early infancy and continues throughout
adolescence. The primary purpose is to facilitate normal neuromotor
development. With the help of correct positioning, appropriate stimulation
and intensive exercise, the therapist tries to gain head control, postural
stability and good mobility in the child. This is possible only to the extent of
the child’s neurological capacity. Even with vigorous physiotherapy, many
children remain functionally impaired in varying degrees.
There are different methods of therapy for children with neurological
impairments. Even though they differ in the techniques they use, basic
principles remain the same. The problems of neuromotor development are
difficult flexion and extension of the body against gravity and difficult
sitting and functional ambulation.
For functional ambulation, the child needs motivation to move and
explore the world around him. He must have enough muscle strength and
control. He must be able to shift his body weight and have an awareness of
body position in space at rest and during movement. Visual and vestibular
systems must be efficient. There must not be any deformities interfering
with joint function.
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In physical therapy sessions, the therapist works with the child in
supine and prone positions to improve head and trunk control. He supports
the child in the sitting position to develop weight shifting and unilateral
balance, ability to rotate the body and the ability to respond to sudden
changes in position. The rehabilitation team strives for long-term functional
mobility in a variety of environments so that the child will integrate into the
community and social life in a healthy way.
Common approaches used in CP treatment:
* Bobath neurodevelopmental technique:
This is the most commonly used therapy approach in CP worldwide. It
aims to normalize muscle tone, inhibit abnormal primitive reflexes and
stimulate normal movement. It uses the idea of reflex inhibitory positions to
decrease spasticity and stimulation of key points of control to promote the
development of advanced postural reactions.
It is believed that through positioning and stimulation, a sense of
normal movement will develop. An important part of treatment of the infant
is teaching the mother how to position the child at home during feeding and
other activities. The baby is held in the anti-spastic position to prevent
formation of contractures.
* Neuro-facilitation techniques:
Sensory input to the CNS produces reflex motor output. The various
neuro-facilitation techniques are based on this basic principle. All of the
techniques aim to normalize muscle tone, to establish advanced postural
reactions and to facilitate normal movement patterns.
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* Vojta method of technique:
Vojta established 18 points in the body for stimulation and used the
positions of reflex crawling and reflex rolling. He proposed that placing the
child in these positions and stimulation of the key points in the body would
enhance CNS development. In this way, the child is presumed to learn
normal movement patterns in place of abnormal motions. Positioning and
stimulation techniques are different from NDT. Vojta stated that therapy
should be applied by the primary caregiver at home at least 4 - 5 times daily
and stopped after a year if there is no improvement.
* Conductive education:
This approach, developed by professor Peto in Hungary depends on
educational principles which become the core stone during treatment. A
conductor is responsible to treat the child in integrated pattern in group
therapy. Each task is divided into steps and learned to child one by one,
using special rhythm of songs and verbal commands.
* Occupational therapy and play:
Occupational therapy (OT) aims to improve hand and upper extremity
function in the child through play and purposeful activity. There are defined
systematic treatment methods for occupational therapy. Ayres sensory
integration therapy aims to enhance the child’s ability to organize and
integrate sensory information. In response to sensory feedback, CNS
perception and execution functions may improve and the motor planning
capacity of the child may increase.
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* Bracing:
Braces are devices which hold the extremities in a stable position. The
goals of bracing are to increase function, prevent deformity, keep the joint in
the functional position, stabilize the trunk and extremities, facilitate selective
motor control and decrease spasticity.
- Lower extremity bracing:
Orthoses are usually named according to the body parts they cover.
Various kinds of ankle foot orthoses (AFOs) are the most common braces
used in CP. Static braces immobilize the joint, while flexible ones use body
weight to stretch the muscles of the leg and ankle. AFOs provide appropriate
contact with the ground during stance and foot clearance during swing.
Knee immobilizing splints and hip abduction splints are prescribed
both for non-ambulatory and ambulatory children. Knee-ankle foot orthoses
(KAFOs) work in children who use them. AFOs are not very useful as night
splints because they do not prevent knee flexion.
* Oral medications:
Various pharmacological agents decrease spasticity such as baclofen,
benzodiazepines and trizanidine are commonly used in children.
* Botulinum toxin:
Botulinum toxin (Botox), produced by the anaerobic bacteria
“Clostridium botulinum”, is one of the most potent poisons known to man.
It enables physical therapists to perform range of motion and strengthening
exercises in an intensive manner to obtain maximum benefits from the
injection. Intensive exercises and electrical stimulation after the injection
may increase toxin uptake by the nerve terminal and potentates the effect.
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* Orthopedic surgery:
Orthopedic surgery is widely used in the management of children with
CP to prevent or correct certain musculoskeletal problems such as muscle
shortening and bone deformities. The goal of orthopedic surgery in a child
with walking potential is to improve functional ambulation. For nonambulatory children, the goal of orthopedic surgery is to facilitate sitting,
improve hygiene, prevent pain and obtain plantigrade stable feet.
* Post-operative physical therapy:
A significant change in all the primary impairments is expected after
surgery. There is a need for gentle return to function. Range of motion and
strength has to be regained as early as possible after surgery. Mobilization
should be started as soon as the child is comfortable and painless, usually on
the second to fourth day after soft tissue procedures. Training with range of
motion exercises starts and gradually progress to strengthening as healing
allows. Keep in mind that a spastic muscle is also a weak muscle, so
strengthen the muscles after muscle lengthening. The ultimate aim is to
improve the ambulatory capacity. It usually takes approximately 3 months to
regain the preoperative muscle strength after multi-level surgery. Immediate
postoperative physical therapy re-introduces movement and the new
alignment. The skills that the patient acquires are established in 3 - 6 months
after surgery.
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