Cerebral Palsy

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.

1 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.

2 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.

3 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.

4 *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.

5 - Respiratory problems: Aspiration in small quantities leads to pneumonia in children who have difficulty in swallowing. Premature babies have broncho- pulmonary 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.

6 - 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.

7 - 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.

8 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.

9 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.

10 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.

11 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.

12 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.

13 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.

14 * 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.

15 * 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.

16 * 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 non- ambulatory 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.