Cerebral Palsy
Content
Table I: Summary of clinical symptoms of impaired muscle function in spastic paresis 4 Impairment of muscle activation Deficit symptoms (signs of reduction or loss of normal voluntary muscle activation) Paresis Inadequate force Loss of selective motor control Impaired ability to activate and control selective or isolated movements across specific joints Loss of dexterity of movement Impaired ability to coordinate temporal and spatial activation of many muscles Enhanced fatigability Inadequate sustained force Excess symptoms (signs of abnormal involuntary muscle activation) Passive movement Hypertonia (TSR) Non-velocity-dependent resistance to passive movement experienced by the examiner as increased muscle tone, caused by continuous muscle activation (Tonic Stretch Reflex activity) Spasticity Velocity-dependent resistance to passive movement experienced by the examiner as increased muscle tone, caused by an increase in stretch reflex activity Clonus Involuntary rhythmic muscle contraction Active movement Mirror movements Involuntary, simultaneous, contralateral movement of a muscle caused by voluntary movement of the same muscle on the ipsilateral side of the body Associated abnormal postures Involuntary abnormal muscle activity related to posture or the performance of any task Co-contraction Simultaneous involuntary contraction of the antagonist at voluntary contraction of the agonist Abnormal reflexes Hyperreflexia of tendon jerks Raised reaction to tendon tap, extension of the reflexogenic zone, spread of reflexes Abnormal nociceptive flexion reflexes Abnormal pathological reflexes to painful stimuli (i.e. Babinski reflex)
Abnormal musculocutaneous reflexes Abnormal pathological reflexes to cutaneous stimuli (i.e. adductor reflex) Changes in biomechanical properties of muscle and other connective tissues Contractures Fixed shortening of muscle and tendon, resulting in a reduced range of motion Hypertonia Non-velocity-dependent resistance to passive stretch experienced by examiner as increased muscle tone, caused by biomechanical changes within muscle itself What is Cerebral palsy? The brain controls all that we do. Different parts of the brain control the movement of every muscle of the body. In cerebral palsy, there is damage to, or lack of development in, one of these areas of the brain. 'Cerebral' – refers to the brain. 'Palsy' – can mean weakness or paralysis or lack of muscle control. Therefore cerebral palsy is a disorder of muscle control which results from some damage to part of the brain. The term cerebral palsy is used when the problem has occurred early in life, to the developing brain. Children with cerebral palsy can have problems such as muscle weakness, stiffness, awkwardness, slowness, shakiness, and difficulty with balance. These problems can range from mild to severe. In mild cerebral palsy, the child may be slightly clumsy in one arm or leg, and the problem may be barely noticeable. In severe cerebral palsy, the child may have a lot of difficulties in performing everyday tasks and movements. movement sight balance
speech 2There are several different types of cerebral palsy: Spastic cerebral palsy This is the most common type of cerebral palsy. Spasticity means stiffness or tightness of muscles. The muscles are stiff because the messages to the muscles are relayed incorrectly from the damaged parts of the brain. When people without cerebral palsy perform a movement, groups of muscles contract whilst the opposite groups of muscles relax or shorten in order to perform the movement. In children with spastic cerebral palsy, both groups of muscles may contract together, making the movement difficult. Dyskinetic cerebral palsy This refers to the type of cerebral palsy with abnormal involuntary movements. It is divided into two types of movement problems, called dystonia and athetosis. • Dystonia – this is the term used for sustained muscle contractions that frequently cause twisting or repetitive movements, or abnormal postures. • A t h e t o s i s – this is the word used for the uncontrolled extra movements that occur particularly in the arms, hands and feet, and around the mouth. The lack of control is often most noticeable when the child starts to move – for example, when the child attempts to grasp a toy or a spoon. In addition, children with athetoid cerebral palsy often feel floppy when carried.
Ataxic cerebral palsy This is the least common type of cerebral palsy. Ataxic (or ataxia) is the word used for unsteady shaky movements or tremor. Children with ataxia also have problems with balance. Mixed types Many children do not have just one type, but a mixture of several of these movement patterns. Some of these concepts are difficult to understand. Please discuss them further with your therapist or doctor. What are the Various types of Cerebral palsy? Which part of the body is affected? Again, this varies greatly from one child to another. Certain words are used to describe the parts affected: Hemiplegia – the leg and arm on one side of the body are affected (also described as hemiparesis). Diplegia – both legs are predominantly affected. Children with diplegia usually also have some difficulties with their arm and hand movements. Quadriplegia – both arms and both legs, and the trunk, are affected (also described as quadriparesis). The muscles of the face, mouth and throat can also be involved. How severe is the movement problem? The Gross Motor Function Classification System (GMFCS) is a method of describing the range of gross motor function in children with cerebral palsy. The
GMFCS describes five 'levels' of motor function, with a particular emphasis on abilities and limitations in the areas of sitting, standing and walking. Children with GMFCS levels I and II walk independently, children with GMFCS level III require sticks, elbow crutches GMFCS for children aged 6-12 yeas GMFCS Level I Children walk indoors and climb stairs without limitation. Children perform gross motor skills including running and jumping, but speed, balance, and coordination are impaired. GMFCS Level II Children walk indoors and climb stairs holding onto a railing but experience limitations walking on uneven surfaces and inclines and walking in crowds or confined spaces. GMFCS Level III Children walk indoors or outdoors on a level surface with an assistive mobility device. Children may climb stairs holding onto a railing. Children may propel a wheelchair manually or are transported when traveling for long distances or outdoors on uneven terrain. GMFCS Level IV Children may continue to walk for short distances on a walker or rely more on wheeled mobility at home and school and in the community. GMFCS Level V Physical impairment restricts voluntary control of movement and the ability to maintain antigravity head and trunk postures. All areas of motor function are limited. Children have no means of independent mobility and are transported Cerebral palsy occurs in about two in every thousand
children. There are many different causes. A problem with the brain can occur: 1. If the brain does not grow or form properly. The result is that children may have brain malformations. 2. In the early months of pregnancy – for example, if the mother is exposed to certain infections such as Rubella (German Measles), or Cytomegalovirus (CMV). 3. During labour or at birth – for example, if the baby does not receive enough oxygen. 4. In the period shortly after birth – for example, when an infant develops a severe infection, such as meningitis, in the first few days or weeks of life. 5. In children having accidents in the early years of life, causing permanent brain injury. These children are also considered to have cerebral palsy. In some children it is important to note that, despite a careful review and various tests, the cause of cerebral palsy remains unknown. With technologies such as MRI brain scans and sophisticated blood tests, more causes are slowly being identified. Current research suggests that approximately 75% of all cerebral palsy is caused by problems that occur during pregnancy, 10–15% by difficulties at birth or in the newborn period, and a further 10% by illnesses or accidents in the early weeks, months or years of life. Problems that lead to permanent neurological deficits up to the age of five to six years are included in the cerebral palsy group. Children particularly at risk for cerebral palsy
are those infants who have been born extremely prematurely. It is sometimes difficult to be sure whether the neurological problem predated the premature delivery or whether the problems that occurred due to prematurity are responsible for the child’s cerebral palsy. Almost all families continue to worry about the cause and why it happened. This is understandable and a natural response. Parents often blame themselves for something they may or may not have done during the pregnancy or birth. But usually the event for which the family blame themselves is either not the cause or could not have been prevented. It is helpful if families can discuss the problem and share their concerns with each other and with the people involved in the care of their child. Sometimes, the child may have additional difficulties. These could include: 1. Problems with hearing – all children should be seen by the specialist in hearing (audiologist) to check for hearing difficulties. 2. Problems with eye sight – children with cerebral palsy very commonly have squints. They can also have other problems with vision. Most children are checked by the eye specialist (ophthalmologist) at some time during their early years. 3. Epilepsy – epilepsy may develop in about one in three of all children with cerebral palsy. There are various types of epilepsy. Some children may only have very occasional seizures whereas in others the
problem may be more persistent, and may require the advice of a paediatric neurologist. 4. Intellectual or learning disability – it must be stressed that there is a wide range of intellectual ability in children with cerebral palsy. Unfortunately it is often difficult to assess learning ability in the early years of life. Children with severe physical disabilities may have normal intelligence. If there is a delay in learning in the early years of life, the term 'developmental delay' is sometimes used. Developmental delay is a descriptive term used when a young child’s development is delayed in one or more areas compared to that of other children. 5. Perceptual difficulties – problems such as judging the size and shape of objects are termed perceptual difficulties, and may not be apparent until school age. 6. Gastro-oesophageal reflux – food comes back up the oesophagus (gullet) more commonly in children with cerebral palsy. Symptoms may include vomiting and discomfort during feeds. A complication of gastro-oesophageal reflux is inflammation of the lower oesophagus (called oesophagitis). Children with this problem may be very unsettled or irritable. 7. Orthopaedic problems – as children grow and develop, muscles with spasticity or stiffness may become shortened, causing muscle or joint contractures. This is most likely to occur at the ankle, knee, hip, elbow and wrist. In addition, children with cerebral palsy are at risk
for developing hip subluxation (movement of the head of the thigh bone out of the hip socket) and dislocation. This is most likely to occur in children that are not walking independently (GMFCS levels III, IV and V). Regular monitoring and surveillance with hip X-rays is important. Physical examination of the hips is not enough so all children with cerebral palsy affecting both lower limbs (diplegia, quadriplegia) require regular hip X-rays (particularly children with GMFCS levels III, IV and V). Scoliosis (curvature of the spine) is a more unusual problem. (Continued next page) 8. Constipation – this is common in children with cerebral palsy. The cause is not always clear. Sometimes it relates to the lack of usual mobility, or it may be related to the difficulty eating a high fibre diet. 9. Nutritional difficulties – some children with severe cerebral palsy may have difficulties with chewing and coordinating their swallowing, causing prolonged or difficult meal times. This in turn may lead to inadequate food intake. Other children have a tendency to put on weight because of reduced physical activity. 10. Saliva control problems – children often dribble in the early years of life. Dribbling can persist in children with cerebral palsy. 11. Recurrent chest infections – this is a problem in only a small group of children with cerebral palsy. It is most likely to occur in children who have
difficulties with chewing and swallowing. Some of the food and drink may inadvertently pass in to the lungs causing the child to cough and wheeze. These episodes may mimic asthma. If these episodes are severe and persistent the child may develop recurrent attacks of pneumonia. Of course, just like other children, those with cerebral palsy may occasionally develop a chest infection, pneumonia or asthma. 12. Bone disease – some children with cerebral palsy are not able to be as active as children without disabilities and have some degree of osteoporosis. Fractures can occur with very minor injuries and sometimes during normal activities such as napkin change or putting an arm through a sleeve. Occasionally children need special medication to promote bone mineralisation. Will my child’s condition deteriorate? The answer is 'No'. The damage done to the brain early in life does not worsen. Sometimes it may seem that the child’s condition is becoming worse. There are many reasons for this apparent deterioration and some are listed below. 1. As children grow older, more is expected of them. For a child with cerebral palsy, simple tasks such as learning to dress and eat independently may take a longer time and may be achieved later than usual. This delay in their development might make it seem that deterioration is occurring, but this is not the case.
2. As the child grows and uses his muscles more and more, they may become tighter. During growth spurts, the child’s bones may grow more quickly than his muscles, which can lead to a tightening of muscles, and a problem such as toe walking may become more apparent. Stiffness in muscles may be due to spasticity, contractures or a mixture of both. It can be difficult to tell the difference but it is important to distinguish between spasticity and shortening because they have different treatments. 3. Any common childhood illness, such as an ear or throat infection, can cause a child’s progress to come to a standstill for a while. 4. Emotional stress. When a child feels he is being 'pushed' to achieve a skill, he may react by becoming stubborn or refusing to cooperate. In a child with cerebral palsy this can certainly be a reason for lack of progress, but it does not mean that his condition has deteriorated. If your child loses previous skills, discuss this with your therapist and/or doctor. WTherapy is often incorporated in an early intervention program which addresses not only the movement problems but aims to optimise the child’s progress in all areas of development. The most commonly used approaches by therapists in Victoria are listed below. . Wh a t th e ra p i e s a re a va i la b l e t o a s s i st m y c h i ld ?
1. Neuro-Developmental therapy (often abbreviated
to 'NDT' and also known as Bobath therapy) is a therapeutic approach to the assessment and management of movement dysfunction in children with neurological dysfunction. The ultimate goal of treatment and management is to maximise the child’s functional ability. The therapy was first developed by Dr and Mrs Bobath in the 1940s and hence is sometimes known as 'Bobath therapy'. Following a thorough assessment, the treatment focuses on making desired movements more possible and preventing undesired movements. Family members and other caregivers receive education in NDT principles to maximise quality of movements and implementation of the program at home, preschool, school and in other community environments. 2. Programs based on the principles of Conductive Education. Conductive Education is a Hungarian system for educating children and adults with movement disorders. In 1940, Professor Andras Peto established the approach by recognising that such disorders are learning difficulties to be overcome rather than conditions to be treated. Conductive Education provides an integrated group program where children and their carers/parents learn to develop skills in all areas of life, for example, daily living, physical, social, emotional, cognitive and communication skills. There are some programs that apply the principles of Conductive Education in Victoria. The professionals involved in these
programs include special education teachers, therapists and occasionally Hungarian 'conductors'. 3. Constraint induced movement therapy, often abbreviated to 'CIMT' is a therapy for children with hemiplegic cerebral palsy that aims to increase the child’s use of their hemiplegic arm and hand. This therapy approach has developed from studies of the effects of constraining the non-affected arm and hand of adults following stroke to 'force' the use of their hemiplegic arm and hand. CIMT involves constraining a child’s unaffected hand and/or arm for a period of time, usually in a modified glove or mitt, while they are encouraged to use their affected hand and arm in play activities. 4. Goal directed training involves the child and/or family identifying specific tasks that the child may need to, want to or have to do at home, school or in their leisure. The approach developed from our understanding of how children learn motor skills. The therapist works with the child and family to identify specific goals or tasks and to assess the child’s performance. The therapist may structure aspects of the task or environment to optimize the child’s performance. Skills required by the child are identified and developed. Repeated task practice is an important part of the approach and requires the child and family to be active partners in the therapy process
TABLE 2
Common Complications of Cerebral Palsy Conditions
Condition
Complications
Care Caregivers and patients should protect the joints and related soft tissues during movement, including avoidance of head injury.
Abnormal neurologic Lack selective control of muscle activity and control anticipatory regulation
Abnormal sensation Some children have impaired sensations to Mittens may be needed during teething to and perception touch and pain with or without astereognosis. prevent damage to fingers and hands. Gastrointestinal problems (e.g., vomiting, constipation, or bowel obstruction) Hearing and vision abnormalities Caused by delayed gastric emptying, abnormal autonomic control of gastrointestinal mobility, immobilization, inadequate oral intake, and prolonged colonic transit Children may present with strabismus or hemianopia. Visual defects occur in 25 to 39 percent of adult patients. Eight to 18 percent of adults with cerebral palsy have hearing problems. Impaired oral-motor functions Can cause hypoxemia, temporomandibular joint contractures, vomiting, and aspiration pneumonia associated with gastroesophageal reflux, poor nutrition, failure to thrive, drooling, and communication difficulties For feeding difficulties, use special diets, positioning, new feeding techniques, gastrostomy, or nasogastric tube feeding.*Medications, surgery, and biofeedback have been used to control drooling. Speech therapy and the use of computer voice synthesizers can help impaired communication. Markedly reduced bone mass in nonambulatory adults and children Can cause osteopenia, osteoporosis, fracture, scoliosis, or pain Assess clinical conditions by physical examination and radiographic studies. Use stool softeners with narcotic pain medications. Perform bowel hygiene.Increase fluids and fiber with or without laxatives. Screen early and periodically.
Use medications, vitamins, and mineral supplementation to reduce bone loss. Encourage exercise. Ask about or use instruments to qualify and monitor pain. Adequately treat pain. Mental health Cognitive impairment is present in two thirds of patients with cerebral palsy.Neurosis and psychosis also can occur. Encourage functionality and independence with living accommodations, transportation, exercise, mechanical aids, or employment opportunities. Provide counseling for emotional and psychological challenges. Monitor for needed medications. Seizures Spasticity and contractures One half of children with cerebral palsy demonstrate seizure activity. Spasticity prevents the stretching of muscles and tendons. Consequently, they do not grow at the same rate as lengthening bones, forming contractures and difficulty with Monitor and control with medication. Prevent with physical therapy with or without orthotic devices.Treat with drugs, surgery, or cerebral stimulation.Assess clinical condition by physical examination
Condition
Complications ambulation and fine- or gross-motor movements.Pain is created by hip dislocations, repetitive use syndromes, and degenerative joint disease.
Care and radiographic studies.Ask or use instruments to qualify and monitor pain.Adequately treat the pain. Special exercises Biofeedback Prescription medications Surgery Surgically implanted devices to replace or aid muscles Specially designed undergarments
Urinary incontinence Caused by impaired control of bladder muscles
*—Long-term use of nasogastric tube feeding is associated with nasal discomfort, recurrent aspiration pneumonia, and decreased survival. Information from references 3 and 5 through 7.
Assessment Instruments
Several assessment instruments are available to quantify and monitor developmental milestones and skills and to assess the quality of life of patients and their caregivers (Table 3 ). Readily available and useful assessment instruments include the Child Health Questionnaire,10 the Wong-Baker FACES Pain Rating Scale, and the Gross Motor Function Classification System for Cerebral Palsy. Functional scales such as the Gross Motor Function Classification System for Cerebral Palsy (Table 4 ) standardize self-initiated movements and measure change in gross motor function over time, and this particular scale is widely accepted and easy to administer in the primary care office. Other functional scales include: the Pediatric Evaluation of Disability Inventory, a judgment-based, standardized instrument using parent report through a structured interview measuring both fine- and gross-motor movements related to self-care and mobility; the Functional Independence Measure (FIM) for adults and the WeeFIM for children, which measure the amount of assistance a person would require to perform activities of daily living; and the Ashworth and Modified Ashworth scales, which grade muscle spasticity.
8–14 14 12 12 12,15 13 16 11 8,9 9
TABLE 3
Clinical Assessment Instruments for Child Development and Quality of Life
Name of instrument Ashworth Scale and Modified Ashworth Scale
8,9 9
Age range Any Age Any age
Use Method of measuring muscle spasticity Cerebral palsy–specific measure of the effect of interventions on personal care, positioning, transferring, comfort, integration, and communication from the perspective of the caregiver Assesses a child’s physical, emotional, and social well-being from the perspective of the parent or guardian and, in some instances, from that of the child. Measures functional performance in three domains: self-care, mobility, and cognition Assesses self-initiated movements (e.g., walking and sitting) over time and categorizes patients based on functional abilities and limitations; useful for planning interventions and evaluating outcomes Measures capability and performance of functional activities in three domains: self-care, mobility, and social function
Caregiver questionnaire
10
Child Health Questionnaire
10
Child form: five to 18 yearsParent/caregiver form: any age Six months to seven years
Functional Independence Measure (WeeFIM) for children
11
Gross Motor Function Classification System for Cerebral Palsy patients
12
One to 12 years
Pediatric Evaluation of Disability Inventory
13
Six months to seven years
Name of instrument
14
Age range
Use Assesses intensity of pain using drawings of six faces whose expressions represent varying degrees of pain
Wong-Baker FACES Pain Three years and older Rating Scale
Information from references 8 through 14.
TABLE 4
Gross Motor Function Classification System for Cerebral Palsy
Before second birthday Level I Infants move in and out of sitting and floor sit with both hands free to manipulate objects. Infants crawl on hands and knees, pull to stand, and take steps holding onto furniture. Infants walk between 18 months and two years of age without the need for any assistive mobility device. Level II Infants maintain floor sitting but may need to use their hands for support to maintain balance. Infants creep on their stomachs or crawl on hands and knees. Infants may pull to stand and take steps holding onto furniture. Level III Level IV Level V Infants maintain floor sitting when the low back is supported. Infants roll and creep forward on their stomachs. Infants have head control but trunk support is required for floor sitting. Infants can roll to supine and may roll to prone. Physical impairments limit voluntary control of movement. Infants are unable to maintain antigravity head and trunk postures in prone and sitting. Infants require adult assistance to roll.
Between second and fourth birthdays Level I Children floor sit with both hands free to manipulate objects. Movements in and out of floor sitting and standing are performed without adult assistance. Children walk as the preferred method of mobility without the need for any assistive mobility device. Level II Children floor sit but may have difficulty with balance when both hands are free to manipulate objects. Movements in and out of sitting are performed without adult assistance. Children pull to stand on stable surface. Children crawl on hands and knees with a reciprocal pattern, cruise holding onto furniture, and walk using an assistive mobility device as preferred methods of mobility. Children maintain floor sitting often by “W-sitting” (sitting between flexed and internally rotated hips and knees) and may require adult assistance to assume sitting. Children creep on the stomach or crawl on hands and knees (often without reciprocal leg movements) as their primary methods of self-mobility. Children may pull to stand on a stable surface and cruise short distances. Children may walk short distances indoors using an assistive mobility device and adult assistance for steering and turning. Children floor sit when placed but are unable to maintain alignment and balance without use of their hands for support. Children commonly require adaptive equipment for sitting and standing. Self-mobility for short distances (within a room) is achieved through rolling, creeping on the stomach, or crawling on hands and knees without reciprocal leg movement. Physical impairments restrict voluntary control of movement and the ability to maintain antigravity head and trunk postures. All areas of motor function are limited. Functional limitations in sitting and standing are not fully compensated for through the use of adaptive equipment and assistive technology. Children at level V have no means of independent mobility and are transported. Some children achieve selfmobility using a power wheelchair with extensive adaptations.
Level III
Level IV
Level V
Between fourth and sixth birthdays Level I Children get into and out of, and sit in, a chair without the need for hand support. Children move from the floor and from chair sitting to standing without the need for objects for support. Children walk indoors and outdoors and climb stairs. Emerging ability to run and jump.
Level II
Children sit in a chair with both hands free to manipulate objects. Children move from the floor to standing and from chair sitting to standing but often require a stable surface to push or pull up on with their arms. Children walk without the need for any assistive mobility device indoors and for short distances on level surfaces outdoors. Children climb stairs holding onto a railing but are unable to run or jump. Children sit on a regular chair but may require pelvic or trunk support to maximize hand function. Children move in and out of chair sitting using a stable surface to push or pull up on with their arms. Children walk with an assistive mobility device on level surfaces and climb stairs with assistance from an adult. Children commonly are transported when traveling for long distances or outdoors on uneven terrain. Children sit on a chair but need adaptive seating for trunk control and to maximize hand function. Children move in and out of chair sitting with assistance from an adult or a stable surface to push or pull up on with their arms. At best, children may walk short distances with a walker and adult supervision but have difficulty turning and maintaining balance on uneven surfaces. Children are transported in the community. Children may achieve self- mobility using a power wheelchair. Same as between second and fourth birthday.
Level III
Level IV
Level V
Between sixth and twelfth birthdays Level I Children walk indoors and outdoors and climb stairs without limitations. Children perform gross motor skills including running and jumping but speed, balance, and coordination are reduced. Level II Level III Level IV Level V Children walk indoors and outdoors and climb stairs holding onto a railing, but they experience limitations walking on uneven surfaces and inclines, and walking in crowds or confined spaces. Children have at best only minimal ability to perform gross-motor skills such as running and jumping. Children walk indoors and outdoors on a level surface with an assistive mobility device. Children may climb stairs holding onto railing. Depending on upper limb function, children propel a wheelchair manually or are transported when traveling for long distances or outdoors on uneven terrain. Children may maintain levels of function achieved before six years of age or rely more on wheeled mobility at home, school, and in the community. Children may achieve self-mobility using a power wheelchair. Same as between second and fourth birthdays.
Distinctions between levels I and II Compared with children in level I, children in level II have limitations in the ease of performing movement transitions, walking outdoors and in the community, the need for assistive mobility devices when beginning to walk, quality of movement, and the ability to perform gross-motor skills such as running and jumping. Distinctions between levels II and III Differences are seen in the degree of achievement of functional mobility. Children in level III need assistive mobility devices and often need orthoses to walk, whereas children in level II do not require assistive mobility devises after four years of age. Distinctions between levels III and IV Differences in sitting ability and mobility exist, even allowing for extensive use of assistive technology. Children in level III sit independently, have independent floor mobility, and walk with assistive mobility devices. Children in level IV function in sitting (usually supported), but independent mobility is very limited. Children in Level IV are more likely to be transported or to use power mobility. Distinctions between levels IV and V Children in level V lack independence even in basic antigravity postural control. Self-mobility is achieved only if the child can learn how to operate an electronically powered wheelchair.
Spastic
Main article: Spastic cerebral palsy
Spastic cerebral palsy is by far the most common type of overall cerebral palsy, occurring in 80% of all cases.[11] People with this type of CP are hypertonic and have what is essentially aneuromuscular mobility impairment (rather than hypotonia or paralysis) stemming from an upper motor neuron lesion in the brain as well as the corticospinal tract or the motor cortex. This damage impairs the ability of some nerve receptors in the spine to properly receive gamma amino butyric acid, leading to hypertonia in the muscles signaled by those damaged nerves. As compared to other types of CP, and especially as compared to hypotonic or paralytic mobility disabilities, spastic CP is typically more easily manageable by the person affected, and medical treatment can be pursued on a multitude of orthopedic and neurological fronts throughout life. Spastic CP is classified by topography dependent on the region of the body affected; these include:
Spastic hemiplegia is one side being affected. Generally, injury to muscle-nerves controlled by the brain's left side will cause a right body deficit, and vice versa. Typically, people that have spastic hemiplegia are the most ambulatory of all the forms, although they generally have dynamic equinus (a limping instability) on the affected side and are primarily prescribed ankle-foot orthoses to prevent said equinus.[12] Spastic diplegia is the lower extremities affected, with little to no upper-body spasticity. The most common form of the spastic forms (70-80% of known cases), most people with spastic diplegia are fully ambulatory, but are "tight" and have a scissors gait. Flexed knees and hips to varying degrees, and moderate to severe adduction (stemming from tight adductor muscles and comparatively weak abductor muscles), are present. Gait analysis is often done in early life on a semi-regular basis, and assistive devices are often provided like walkers, crutches or canes; any ankle-foot orthotics provided usually go on both legs rather than just one. In addition, these individuals are often nearsighted. The intelligence of a person with spastic diplegia is unaffected by the condition. Over time, the effects of the spasticity sometimes produce hip problems and dislocations (see the main article and spasticity for more on spasticity effects). In threequarters of spastic diplegics, also strabismus (crossed eyes) can be present as well.
Spastic monoplegia is one single limb being affected. Spastic triplegia is three limbs being affected. Spastic quadriplegia is all four limbs more or less equally affected. People with spastic quadriplegia are the least likely to be able to walk, or if they can, to desire to walk, because their muscles are too tight and it is too much of an effort to do so. Some children with spastic quadriplegia also have hemiparetic tremors, an uncontrollable shaking that affects the limbs on one side of the body and impairs normal movement.
In any form of spastic CP, clonus of the affected limb(s) may sometimes result, as well as muscle spasms resulting from the pain and/or stress of the tightness experienced. The spasticity can and usually does also lead to very early onset of muscle-stress symptoms like arthritis and tendinitis, especially in ambulatory individuals in their mid-20s and early-30s. Physical therapy and occupational therapy regimens of assisted stretching, strengthening, functional tasks, and/or targeted physical activity and exercise are usually the chief ways to keep spastic CP well-managed, although if the spasticity is too much for the person to handle, other remedies may be considered, such as various antispasmodic medications, botox, baclofen, or even a neurosurgery known as a selective dorsal rhizotomy (which eliminates the spasticity by eliminating the nerves causing it). [edit]Ataxic Main article: Ataxic cerebral palsy Ataxia type symptoms can be caused by damage to the cerebellum. The forms of ataxia are less common types of cerebral palsy, occurring in at most 10% of all cases. [citation needed] Some of these individuals have hypotonia and tremors. Motor skills such as writing, typing, or using scissors might be affected, as well as balance, especially while walking. It is common for individuals to have difficulty with visual and/or auditory processing. [edit]Athetoid/Dyskinetic Main article: Athetoid cerebral palsy Athetoid cerebral palsy or dyskinetic cerebral palsy is mixed muscle tone – both hypertonia and hypotonia mixed with involuntary motions. People with Dyskinetic CP have trouble holding themselves in an upright, steady position for sitting or walking, and often show involuntary motions. For some people with dyskinetic CP, it takes a lot of work and concentration to get their hand to a certain spot (like scratching their nose or reaching for a cup). Because of their mixed tone and trouble keeping a position, they may not be able to hold onto objects, especially small ones requiring fine motor control(such as a toothbrush or pencil). About 10% of individuals with CP are classified as dyskinetic CP but some have mixed forms with spasticity and dyskinesia.[13] The damage occurs to theextrapyramidal motor system and/or pyramidal tract and to the basal ganglia. In newborn infants, high bilirubin levels in the blood, if left untreated, can lead to brain damage in in the basal ganglia (kernicterus), which can lead to dyskinetic cerebral palsy. [edit]Signs
and symptoms
All types of cerebral palsy are characterized by abnormal muscle tone (i.e. slouching over while sitting), reflexes, or motor development and coordination. There can be joint and bone deformities andcontractures (permanently fixed, tight muscles and joints). The classical symptoms are spasticities, spasms, other involuntary movements (e.g. facial gestures), unsteady gait, problems with balance, and/or soft tissue findings consisting largely of decreased muscle mass. Scissor walking (where the knees come in and cross) and toe walking (which can contribute to a gait reminiscent of a marionette) are common among people with CP who are able to walk, but taken on the whole, CP symptomatology is very diverse. The effects of cerebral palsy fall on a continuum of motor dysfunction which may range from slight clumsiness at the mild end of the spectrum to impairments so severe that they render coordinated movement virtually impossible at the other end the spectrum. Babies born with severe CP often have an irregular posture; their bodies may be either very floppy or very stiff. Birth defects, such as spinal curvature, a small jawbone, or a small head sometimes occur along with CP. Symptoms may appear or change as a child gets older. Some babies born with CP do not show obvious signs right away. Classically, CP becomes evident when the baby reaches the developmental stage at six and a half to 9 months and is starting to mobilise, where preferential use of limbs, asymmetry or gross motor developmental delay is seen. Secondary conditions can include seizures, epilepsy, apraxia, dysarthria or other communication disorders, eating problems, sensory impairments, mental retardation, learning disabilities, urinary incontinence, fecal incontinence and/or behavioral disorders. Speech and language disorders are common in people with cerebral palsy. The incidence of dysarthria is estimated to range from 31% to 88%. Speech problems are associated with poor respiratorycontrol, laryngeal and velopharyngeal dysfunction as well as oral articulation disorders that are due to restricted movement in the oral-facial muscles. There are three major types of dysarthria in cerebral palsy: spastic, dyskinetic (athetosis) and ataxic. Speech impairments in spastic dysarthria involves four major abnormalities of voluntary movement: spasticity, weakness, limited range of motion and slowness of movement. Speech mechanism impairment in athetosis involves a disorder in the regulation of breathing patterns, laryngeal dysfunction (monopitch, low, weak and breathy voice quality). It is also associated with articulatory dysfunction (large range of jaw movements), inappropriate positioning of the tongue, instability of velar elevation. Athetoid dysarthria is caused by disruption of the internal sensorimotor feedback system for appropriate motor commands, which leads to the generation of faulty movements that are perceived by others as involuntary. Ataxic dysarthria is uncommon in cerebral palsy. The speech characteristics are: imprecise consonants, irregular articulatory breakdown, distorted vowels, excess and equal stress, prolonged phonemes, slow rate, monopitch, monoloudness and harsh voice.[14] Overall language delay is associated with problems of mental retardation, hearing impairment and learned helplessness.[3] Children with cerebral palsy are at risk oflearned helplessness and becoming passive communicators, initiating little communication.[3] Early
intervention with this clientele often targets situations in which children communicate with others, so that they learn that they can control people and objects in their environment through this communication, including making choices, decisions and mistakes.[3] [edit]Skeleton In order for bones to attain their normal shape and size, they require the stresses from normal musculature. Osseous findings will therefore mirror the specific muscular deficits in a given person with CP. The shafts of the bones are often thin (gracile) and become thinner during growth. When compared to these thin shafts (diaphyses), the centers (metaphyses) often appear quite enlarged (ballooning). With lack of use, articular cartilage may atrophy, leading to narrowed joint spaces. Depending on the degree of spasticity, a person with CP may exhibit a variety of angular joint deformities. Because vertebral bodies need vertical gravitational loading forces to develop properly, spasticity and an abnormal gait can hinder proper and/or full bone and skeletal development. People with CP tend to be shorter in height than the average person because their bones are not allowed to grow to their full potential. Sometimes bones grow to different lengths, so the person may have one leg longer than the other. [edit]Pain
and sleep disorders
Pain is common, and may result from the inherent deficits associated with the condition, along with the numerous procedures children typically face.[15] There is also a high likelihood of suffering from chronic sleep disorders associated with both physical and environmental factors.[16] Pain is also associated with tight and/or shortened muscle, abnormal posture, stiff joints, unsuitable orthosis etc. [edit]Causes
Micrograph showing a fetal (placental) vein thrombosis, in a case of fetal thrombotic vasculopathy (FTV). FTV is associated with cerebral palsy and is suggestive of ahypercoagulable state as an underlying cause. H&E stain.
While in certain cases there is no identifiable cause, typical causes include problems in intrauterine development (e.g. exposure to radiation, infection),asphyxia before birth, hypoxia of the brain, and birth trauma during labor and delivery, and complications in the perinatal period or during childhood. [3] CP is also more common in multiple births. Between 40% and 50% of all children who develop cerebral palsy were born prematurely. Premature infants are vulnerable, in part because their organs are not fully developed, increasing the risk of hypoxic injury to the brain that may manifest as CP. A problem in interpreting this is the difficulty in differentiating between cerebral palsy caused by damage to the brain that results from inadequate oxygenation and CP that arises from prenatal brain damage that then precipitates premature delivery. Recent research has demonstrated that intrapartum asphyxia is not the most important cause, probably accounting for no more than 10 percent of all cases; rather, infections in the mother, even infections that are not easily detected, may triple the risk of the child developing the disorder, mainly as the result of the toxicity to the fetal brain of cytokines that are produced as part of the inflammatory response.[17] Low birthweight is a risk factor for CP—and premature infants usually have low birth weights, less than 2.0 kg, but full-term infants can also have low birth weights. Multiple-birth infants are also more likely than single-birth infants to be born early or with a low birth weight. After birth, other causes include toxins, severe jaundice, lead poisoning, physical brain injury, shaken baby syndrome, incidents involving hypoxia to the brain (such as near drowning), and encephalitis or meningitis. The three most common causes of asphyxia in the young child are: choking on foreign objects such as toys and pieces of food, poisoning, and near drowning. Some structural brain anomalies such as lissencephaly may present with the clinical features of CP, although whether that could be considered CP is a matter of opinion (some people say CP must be due to brain damage, whereas people with these anomalies didn't have a normal brain). Often this goes along with rare chromosome disorders and CP is not genetic or hereditary. It has been hypothetized that many cases of cerebral palsy are caused by the death in very early pregnancy of an identical twin.[18] [edit]Diagnosis
Abnormal musculocutaneous reflexes Abnormal pathological reflexes to cutaneous stimuli (i.e. adductor reflex) Changes in biomechanical properties of muscle and other connective tissues Contractures Fixed shortening of muscle and tendon, resulting in a reduced range of motion Hypertonia Non-velocity-dependent resistance to passive stretch experienced by examiner as increased muscle tone, caused by biomechanical changes within muscle itself What is Cerebral palsy? The brain controls all that we do. Different parts of the brain control the movement of every muscle of the body. In cerebral palsy, there is damage to, or lack of development in, one of these areas of the brain. 'Cerebral' – refers to the brain. 'Palsy' – can mean weakness or paralysis or lack of muscle control. Therefore cerebral palsy is a disorder of muscle control which results from some damage to part of the brain. The term cerebral palsy is used when the problem has occurred early in life, to the developing brain. Children with cerebral palsy can have problems such as muscle weakness, stiffness, awkwardness, slowness, shakiness, and difficulty with balance. These problems can range from mild to severe. In mild cerebral palsy, the child may be slightly clumsy in one arm or leg, and the problem may be barely noticeable. In severe cerebral palsy, the child may have a lot of difficulties in performing everyday tasks and movements. movement sight balance
speech 2There are several different types of cerebral palsy: Spastic cerebral palsy This is the most common type of cerebral palsy. Spasticity means stiffness or tightness of muscles. The muscles are stiff because the messages to the muscles are relayed incorrectly from the damaged parts of the brain. When people without cerebral palsy perform a movement, groups of muscles contract whilst the opposite groups of muscles relax or shorten in order to perform the movement. In children with spastic cerebral palsy, both groups of muscles may contract together, making the movement difficult. Dyskinetic cerebral palsy This refers to the type of cerebral palsy with abnormal involuntary movements. It is divided into two types of movement problems, called dystonia and athetosis. • Dystonia – this is the term used for sustained muscle contractions that frequently cause twisting or repetitive movements, or abnormal postures. • A t h e t o s i s – this is the word used for the uncontrolled extra movements that occur particularly in the arms, hands and feet, and around the mouth. The lack of control is often most noticeable when the child starts to move – for example, when the child attempts to grasp a toy or a spoon. In addition, children with athetoid cerebral palsy often feel floppy when carried.
Ataxic cerebral palsy This is the least common type of cerebral palsy. Ataxic (or ataxia) is the word used for unsteady shaky movements or tremor. Children with ataxia also have problems with balance. Mixed types Many children do not have just one type, but a mixture of several of these movement patterns. Some of these concepts are difficult to understand. Please discuss them further with your therapist or doctor. What are the Various types of Cerebral palsy? Which part of the body is affected? Again, this varies greatly from one child to another. Certain words are used to describe the parts affected: Hemiplegia – the leg and arm on one side of the body are affected (also described as hemiparesis). Diplegia – both legs are predominantly affected. Children with diplegia usually also have some difficulties with their arm and hand movements. Quadriplegia – both arms and both legs, and the trunk, are affected (also described as quadriparesis). The muscles of the face, mouth and throat can also be involved. How severe is the movement problem? The Gross Motor Function Classification System (GMFCS) is a method of describing the range of gross motor function in children with cerebral palsy. The
GMFCS describes five 'levels' of motor function, with a particular emphasis on abilities and limitations in the areas of sitting, standing and walking. Children with GMFCS levels I and II walk independently, children with GMFCS level III require sticks, elbow crutches GMFCS for children aged 6-12 yeas GMFCS Level I Children walk indoors and climb stairs without limitation. Children perform gross motor skills including running and jumping, but speed, balance, and coordination are impaired. GMFCS Level II Children walk indoors and climb stairs holding onto a railing but experience limitations walking on uneven surfaces and inclines and walking in crowds or confined spaces. GMFCS Level III Children walk indoors or outdoors on a level surface with an assistive mobility device. Children may climb stairs holding onto a railing. Children may propel a wheelchair manually or are transported when traveling for long distances or outdoors on uneven terrain. GMFCS Level IV Children may continue to walk for short distances on a walker or rely more on wheeled mobility at home and school and in the community. GMFCS Level V Physical impairment restricts voluntary control of movement and the ability to maintain antigravity head and trunk postures. All areas of motor function are limited. Children have no means of independent mobility and are transported Cerebral palsy occurs in about two in every thousand
children. There are many different causes. A problem with the brain can occur: 1. If the brain does not grow or form properly. The result is that children may have brain malformations. 2. In the early months of pregnancy – for example, if the mother is exposed to certain infections such as Rubella (German Measles), or Cytomegalovirus (CMV). 3. During labour or at birth – for example, if the baby does not receive enough oxygen. 4. In the period shortly after birth – for example, when an infant develops a severe infection, such as meningitis, in the first few days or weeks of life. 5. In children having accidents in the early years of life, causing permanent brain injury. These children are also considered to have cerebral palsy. In some children it is important to note that, despite a careful review and various tests, the cause of cerebral palsy remains unknown. With technologies such as MRI brain scans and sophisticated blood tests, more causes are slowly being identified. Current research suggests that approximately 75% of all cerebral palsy is caused by problems that occur during pregnancy, 10–15% by difficulties at birth or in the newborn period, and a further 10% by illnesses or accidents in the early weeks, months or years of life. Problems that lead to permanent neurological deficits up to the age of five to six years are included in the cerebral palsy group. Children particularly at risk for cerebral palsy
are those infants who have been born extremely prematurely. It is sometimes difficult to be sure whether the neurological problem predated the premature delivery or whether the problems that occurred due to prematurity are responsible for the child’s cerebral palsy. Almost all families continue to worry about the cause and why it happened. This is understandable and a natural response. Parents often blame themselves for something they may or may not have done during the pregnancy or birth. But usually the event for which the family blame themselves is either not the cause or could not have been prevented. It is helpful if families can discuss the problem and share their concerns with each other and with the people involved in the care of their child. Sometimes, the child may have additional difficulties. These could include: 1. Problems with hearing – all children should be seen by the specialist in hearing (audiologist) to check for hearing difficulties. 2. Problems with eye sight – children with cerebral palsy very commonly have squints. They can also have other problems with vision. Most children are checked by the eye specialist (ophthalmologist) at some time during their early years. 3. Epilepsy – epilepsy may develop in about one in three of all children with cerebral palsy. There are various types of epilepsy. Some children may only have very occasional seizures whereas in others the
problem may be more persistent, and may require the advice of a paediatric neurologist. 4. Intellectual or learning disability – it must be stressed that there is a wide range of intellectual ability in children with cerebral palsy. Unfortunately it is often difficult to assess learning ability in the early years of life. Children with severe physical disabilities may have normal intelligence. If there is a delay in learning in the early years of life, the term 'developmental delay' is sometimes used. Developmental delay is a descriptive term used when a young child’s development is delayed in one or more areas compared to that of other children. 5. Perceptual difficulties – problems such as judging the size and shape of objects are termed perceptual difficulties, and may not be apparent until school age. 6. Gastro-oesophageal reflux – food comes back up the oesophagus (gullet) more commonly in children with cerebral palsy. Symptoms may include vomiting and discomfort during feeds. A complication of gastro-oesophageal reflux is inflammation of the lower oesophagus (called oesophagitis). Children with this problem may be very unsettled or irritable. 7. Orthopaedic problems – as children grow and develop, muscles with spasticity or stiffness may become shortened, causing muscle or joint contractures. This is most likely to occur at the ankle, knee, hip, elbow and wrist. In addition, children with cerebral palsy are at risk
for developing hip subluxation (movement of the head of the thigh bone out of the hip socket) and dislocation. This is most likely to occur in children that are not walking independently (GMFCS levels III, IV and V). Regular monitoring and surveillance with hip X-rays is important. Physical examination of the hips is not enough so all children with cerebral palsy affecting both lower limbs (diplegia, quadriplegia) require regular hip X-rays (particularly children with GMFCS levels III, IV and V). Scoliosis (curvature of the spine) is a more unusual problem. (Continued next page) 8. Constipation – this is common in children with cerebral palsy. The cause is not always clear. Sometimes it relates to the lack of usual mobility, or it may be related to the difficulty eating a high fibre diet. 9. Nutritional difficulties – some children with severe cerebral palsy may have difficulties with chewing and coordinating their swallowing, causing prolonged or difficult meal times. This in turn may lead to inadequate food intake. Other children have a tendency to put on weight because of reduced physical activity. 10. Saliva control problems – children often dribble in the early years of life. Dribbling can persist in children with cerebral palsy. 11. Recurrent chest infections – this is a problem in only a small group of children with cerebral palsy. It is most likely to occur in children who have
difficulties with chewing and swallowing. Some of the food and drink may inadvertently pass in to the lungs causing the child to cough and wheeze. These episodes may mimic asthma. If these episodes are severe and persistent the child may develop recurrent attacks of pneumonia. Of course, just like other children, those with cerebral palsy may occasionally develop a chest infection, pneumonia or asthma. 12. Bone disease – some children with cerebral palsy are not able to be as active as children without disabilities and have some degree of osteoporosis. Fractures can occur with very minor injuries and sometimes during normal activities such as napkin change or putting an arm through a sleeve. Occasionally children need special medication to promote bone mineralisation. Will my child’s condition deteriorate? The answer is 'No'. The damage done to the brain early in life does not worsen. Sometimes it may seem that the child’s condition is becoming worse. There are many reasons for this apparent deterioration and some are listed below. 1. As children grow older, more is expected of them. For a child with cerebral palsy, simple tasks such as learning to dress and eat independently may take a longer time and may be achieved later than usual. This delay in their development might make it seem that deterioration is occurring, but this is not the case.
2. As the child grows and uses his muscles more and more, they may become tighter. During growth spurts, the child’s bones may grow more quickly than his muscles, which can lead to a tightening of muscles, and a problem such as toe walking may become more apparent. Stiffness in muscles may be due to spasticity, contractures or a mixture of both. It can be difficult to tell the difference but it is important to distinguish between spasticity and shortening because they have different treatments. 3. Any common childhood illness, such as an ear or throat infection, can cause a child’s progress to come to a standstill for a while. 4. Emotional stress. When a child feels he is being 'pushed' to achieve a skill, he may react by becoming stubborn or refusing to cooperate. In a child with cerebral palsy this can certainly be a reason for lack of progress, but it does not mean that his condition has deteriorated. If your child loses previous skills, discuss this with your therapist and/or doctor. WTherapy is often incorporated in an early intervention program which addresses not only the movement problems but aims to optimise the child’s progress in all areas of development. The most commonly used approaches by therapists in Victoria are listed below. . Wh a t th e ra p i e s a re a va i la b l e t o a s s i st m y c h i ld ?
1. Neuro-Developmental therapy (often abbreviated
to 'NDT' and also known as Bobath therapy) is a therapeutic approach to the assessment and management of movement dysfunction in children with neurological dysfunction. The ultimate goal of treatment and management is to maximise the child’s functional ability. The therapy was first developed by Dr and Mrs Bobath in the 1940s and hence is sometimes known as 'Bobath therapy'. Following a thorough assessment, the treatment focuses on making desired movements more possible and preventing undesired movements. Family members and other caregivers receive education in NDT principles to maximise quality of movements and implementation of the program at home, preschool, school and in other community environments. 2. Programs based on the principles of Conductive Education. Conductive Education is a Hungarian system for educating children and adults with movement disorders. In 1940, Professor Andras Peto established the approach by recognising that such disorders are learning difficulties to be overcome rather than conditions to be treated. Conductive Education provides an integrated group program where children and their carers/parents learn to develop skills in all areas of life, for example, daily living, physical, social, emotional, cognitive and communication skills. There are some programs that apply the principles of Conductive Education in Victoria. The professionals involved in these
programs include special education teachers, therapists and occasionally Hungarian 'conductors'. 3. Constraint induced movement therapy, often abbreviated to 'CIMT' is a therapy for children with hemiplegic cerebral palsy that aims to increase the child’s use of their hemiplegic arm and hand. This therapy approach has developed from studies of the effects of constraining the non-affected arm and hand of adults following stroke to 'force' the use of their hemiplegic arm and hand. CIMT involves constraining a child’s unaffected hand and/or arm for a period of time, usually in a modified glove or mitt, while they are encouraged to use their affected hand and arm in play activities. 4. Goal directed training involves the child and/or family identifying specific tasks that the child may need to, want to or have to do at home, school or in their leisure. The approach developed from our understanding of how children learn motor skills. The therapist works with the child and family to identify specific goals or tasks and to assess the child’s performance. The therapist may structure aspects of the task or environment to optimize the child’s performance. Skills required by the child are identified and developed. Repeated task practice is an important part of the approach and requires the child and family to be active partners in the therapy process
TABLE 2
Common Complications of Cerebral Palsy Conditions
Condition
Complications
Care Caregivers and patients should protect the joints and related soft tissues during movement, including avoidance of head injury.
Abnormal neurologic Lack selective control of muscle activity and control anticipatory regulation
Abnormal sensation Some children have impaired sensations to Mittens may be needed during teething to and perception touch and pain with or without astereognosis. prevent damage to fingers and hands. Gastrointestinal problems (e.g., vomiting, constipation, or bowel obstruction) Hearing and vision abnormalities Caused by delayed gastric emptying, abnormal autonomic control of gastrointestinal mobility, immobilization, inadequate oral intake, and prolonged colonic transit Children may present with strabismus or hemianopia. Visual defects occur in 25 to 39 percent of adult patients. Eight to 18 percent of adults with cerebral palsy have hearing problems. Impaired oral-motor functions Can cause hypoxemia, temporomandibular joint contractures, vomiting, and aspiration pneumonia associated with gastroesophageal reflux, poor nutrition, failure to thrive, drooling, and communication difficulties For feeding difficulties, use special diets, positioning, new feeding techniques, gastrostomy, or nasogastric tube feeding.*Medications, surgery, and biofeedback have been used to control drooling. Speech therapy and the use of computer voice synthesizers can help impaired communication. Markedly reduced bone mass in nonambulatory adults and children Can cause osteopenia, osteoporosis, fracture, scoliosis, or pain Assess clinical conditions by physical examination and radiographic studies. Use stool softeners with narcotic pain medications. Perform bowel hygiene.Increase fluids and fiber with or without laxatives. Screen early and periodically.
Use medications, vitamins, and mineral supplementation to reduce bone loss. Encourage exercise. Ask about or use instruments to qualify and monitor pain. Adequately treat pain. Mental health Cognitive impairment is present in two thirds of patients with cerebral palsy.Neurosis and psychosis also can occur. Encourage functionality and independence with living accommodations, transportation, exercise, mechanical aids, or employment opportunities. Provide counseling for emotional and psychological challenges. Monitor for needed medications. Seizures Spasticity and contractures One half of children with cerebral palsy demonstrate seizure activity. Spasticity prevents the stretching of muscles and tendons. Consequently, they do not grow at the same rate as lengthening bones, forming contractures and difficulty with Monitor and control with medication. Prevent with physical therapy with or without orthotic devices.Treat with drugs, surgery, or cerebral stimulation.Assess clinical condition by physical examination
Condition
Complications ambulation and fine- or gross-motor movements.Pain is created by hip dislocations, repetitive use syndromes, and degenerative joint disease.
Care and radiographic studies.Ask or use instruments to qualify and monitor pain.Adequately treat the pain. Special exercises Biofeedback Prescription medications Surgery Surgically implanted devices to replace or aid muscles Specially designed undergarments
Urinary incontinence Caused by impaired control of bladder muscles
*—Long-term use of nasogastric tube feeding is associated with nasal discomfort, recurrent aspiration pneumonia, and decreased survival. Information from references 3 and 5 through 7.
Assessment Instruments
Several assessment instruments are available to quantify and monitor developmental milestones and skills and to assess the quality of life of patients and their caregivers (Table 3 ). Readily available and useful assessment instruments include the Child Health Questionnaire,10 the Wong-Baker FACES Pain Rating Scale, and the Gross Motor Function Classification System for Cerebral Palsy. Functional scales such as the Gross Motor Function Classification System for Cerebral Palsy (Table 4 ) standardize self-initiated movements and measure change in gross motor function over time, and this particular scale is widely accepted and easy to administer in the primary care office. Other functional scales include: the Pediatric Evaluation of Disability Inventory, a judgment-based, standardized instrument using parent report through a structured interview measuring both fine- and gross-motor movements related to self-care and mobility; the Functional Independence Measure (FIM) for adults and the WeeFIM for children, which measure the amount of assistance a person would require to perform activities of daily living; and the Ashworth and Modified Ashworth scales, which grade muscle spasticity.
8–14 14 12 12 12,15 13 16 11 8,9 9
TABLE 3
Clinical Assessment Instruments for Child Development and Quality of Life
Name of instrument Ashworth Scale and Modified Ashworth Scale
8,9 9
Age range Any Age Any age
Use Method of measuring muscle spasticity Cerebral palsy–specific measure of the effect of interventions on personal care, positioning, transferring, comfort, integration, and communication from the perspective of the caregiver Assesses a child’s physical, emotional, and social well-being from the perspective of the parent or guardian and, in some instances, from that of the child. Measures functional performance in three domains: self-care, mobility, and cognition Assesses self-initiated movements (e.g., walking and sitting) over time and categorizes patients based on functional abilities and limitations; useful for planning interventions and evaluating outcomes Measures capability and performance of functional activities in three domains: self-care, mobility, and social function
Caregiver questionnaire
10
Child Health Questionnaire
10
Child form: five to 18 yearsParent/caregiver form: any age Six months to seven years
Functional Independence Measure (WeeFIM) for children
11
Gross Motor Function Classification System for Cerebral Palsy patients
12
One to 12 years
Pediatric Evaluation of Disability Inventory
13
Six months to seven years
Name of instrument
14
Age range
Use Assesses intensity of pain using drawings of six faces whose expressions represent varying degrees of pain
Wong-Baker FACES Pain Three years and older Rating Scale
Information from references 8 through 14.
TABLE 4
Gross Motor Function Classification System for Cerebral Palsy
Before second birthday Level I Infants move in and out of sitting and floor sit with both hands free to manipulate objects. Infants crawl on hands and knees, pull to stand, and take steps holding onto furniture. Infants walk between 18 months and two years of age without the need for any assistive mobility device. Level II Infants maintain floor sitting but may need to use their hands for support to maintain balance. Infants creep on their stomachs or crawl on hands and knees. Infants may pull to stand and take steps holding onto furniture. Level III Level IV Level V Infants maintain floor sitting when the low back is supported. Infants roll and creep forward on their stomachs. Infants have head control but trunk support is required for floor sitting. Infants can roll to supine and may roll to prone. Physical impairments limit voluntary control of movement. Infants are unable to maintain antigravity head and trunk postures in prone and sitting. Infants require adult assistance to roll.
Between second and fourth birthdays Level I Children floor sit with both hands free to manipulate objects. Movements in and out of floor sitting and standing are performed without adult assistance. Children walk as the preferred method of mobility without the need for any assistive mobility device. Level II Children floor sit but may have difficulty with balance when both hands are free to manipulate objects. Movements in and out of sitting are performed without adult assistance. Children pull to stand on stable surface. Children crawl on hands and knees with a reciprocal pattern, cruise holding onto furniture, and walk using an assistive mobility device as preferred methods of mobility. Children maintain floor sitting often by “W-sitting” (sitting between flexed and internally rotated hips and knees) and may require adult assistance to assume sitting. Children creep on the stomach or crawl on hands and knees (often without reciprocal leg movements) as their primary methods of self-mobility. Children may pull to stand on a stable surface and cruise short distances. Children may walk short distances indoors using an assistive mobility device and adult assistance for steering and turning. Children floor sit when placed but are unable to maintain alignment and balance without use of their hands for support. Children commonly require adaptive equipment for sitting and standing. Self-mobility for short distances (within a room) is achieved through rolling, creeping on the stomach, or crawling on hands and knees without reciprocal leg movement. Physical impairments restrict voluntary control of movement and the ability to maintain antigravity head and trunk postures. All areas of motor function are limited. Functional limitations in sitting and standing are not fully compensated for through the use of adaptive equipment and assistive technology. Children at level V have no means of independent mobility and are transported. Some children achieve selfmobility using a power wheelchair with extensive adaptations.
Level III
Level IV
Level V
Between fourth and sixth birthdays Level I Children get into and out of, and sit in, a chair without the need for hand support. Children move from the floor and from chair sitting to standing without the need for objects for support. Children walk indoors and outdoors and climb stairs. Emerging ability to run and jump.
Level II
Children sit in a chair with both hands free to manipulate objects. Children move from the floor to standing and from chair sitting to standing but often require a stable surface to push or pull up on with their arms. Children walk without the need for any assistive mobility device indoors and for short distances on level surfaces outdoors. Children climb stairs holding onto a railing but are unable to run or jump. Children sit on a regular chair but may require pelvic or trunk support to maximize hand function. Children move in and out of chair sitting using a stable surface to push or pull up on with their arms. Children walk with an assistive mobility device on level surfaces and climb stairs with assistance from an adult. Children commonly are transported when traveling for long distances or outdoors on uneven terrain. Children sit on a chair but need adaptive seating for trunk control and to maximize hand function. Children move in and out of chair sitting with assistance from an adult or a stable surface to push or pull up on with their arms. At best, children may walk short distances with a walker and adult supervision but have difficulty turning and maintaining balance on uneven surfaces. Children are transported in the community. Children may achieve self- mobility using a power wheelchair. Same as between second and fourth birthday.
Level III
Level IV
Level V
Between sixth and twelfth birthdays Level I Children walk indoors and outdoors and climb stairs without limitations. Children perform gross motor skills including running and jumping but speed, balance, and coordination are reduced. Level II Level III Level IV Level V Children walk indoors and outdoors and climb stairs holding onto a railing, but they experience limitations walking on uneven surfaces and inclines, and walking in crowds or confined spaces. Children have at best only minimal ability to perform gross-motor skills such as running and jumping. Children walk indoors and outdoors on a level surface with an assistive mobility device. Children may climb stairs holding onto railing. Depending on upper limb function, children propel a wheelchair manually or are transported when traveling for long distances or outdoors on uneven terrain. Children may maintain levels of function achieved before six years of age or rely more on wheeled mobility at home, school, and in the community. Children may achieve self-mobility using a power wheelchair. Same as between second and fourth birthdays.
Distinctions between levels I and II Compared with children in level I, children in level II have limitations in the ease of performing movement transitions, walking outdoors and in the community, the need for assistive mobility devices when beginning to walk, quality of movement, and the ability to perform gross-motor skills such as running and jumping. Distinctions between levels II and III Differences are seen in the degree of achievement of functional mobility. Children in level III need assistive mobility devices and often need orthoses to walk, whereas children in level II do not require assistive mobility devises after four years of age. Distinctions between levels III and IV Differences in sitting ability and mobility exist, even allowing for extensive use of assistive technology. Children in level III sit independently, have independent floor mobility, and walk with assistive mobility devices. Children in level IV function in sitting (usually supported), but independent mobility is very limited. Children in Level IV are more likely to be transported or to use power mobility. Distinctions between levels IV and V Children in level V lack independence even in basic antigravity postural control. Self-mobility is achieved only if the child can learn how to operate an electronically powered wheelchair.
Spastic
Main article: Spastic cerebral palsy
Spastic cerebral palsy is by far the most common type of overall cerebral palsy, occurring in 80% of all cases.[11] People with this type of CP are hypertonic and have what is essentially aneuromuscular mobility impairment (rather than hypotonia or paralysis) stemming from an upper motor neuron lesion in the brain as well as the corticospinal tract or the motor cortex. This damage impairs the ability of some nerve receptors in the spine to properly receive gamma amino butyric acid, leading to hypertonia in the muscles signaled by those damaged nerves. As compared to other types of CP, and especially as compared to hypotonic or paralytic mobility disabilities, spastic CP is typically more easily manageable by the person affected, and medical treatment can be pursued on a multitude of orthopedic and neurological fronts throughout life. Spastic CP is classified by topography dependent on the region of the body affected; these include:
Spastic hemiplegia is one side being affected. Generally, injury to muscle-nerves controlled by the brain's left side will cause a right body deficit, and vice versa. Typically, people that have spastic hemiplegia are the most ambulatory of all the forms, although they generally have dynamic equinus (a limping instability) on the affected side and are primarily prescribed ankle-foot orthoses to prevent said equinus.[12] Spastic diplegia is the lower extremities affected, with little to no upper-body spasticity. The most common form of the spastic forms (70-80% of known cases), most people with spastic diplegia are fully ambulatory, but are "tight" and have a scissors gait. Flexed knees and hips to varying degrees, and moderate to severe adduction (stemming from tight adductor muscles and comparatively weak abductor muscles), are present. Gait analysis is often done in early life on a semi-regular basis, and assistive devices are often provided like walkers, crutches or canes; any ankle-foot orthotics provided usually go on both legs rather than just one. In addition, these individuals are often nearsighted. The intelligence of a person with spastic diplegia is unaffected by the condition. Over time, the effects of the spasticity sometimes produce hip problems and dislocations (see the main article and spasticity for more on spasticity effects). In threequarters of spastic diplegics, also strabismus (crossed eyes) can be present as well.
Spastic monoplegia is one single limb being affected. Spastic triplegia is three limbs being affected. Spastic quadriplegia is all four limbs more or less equally affected. People with spastic quadriplegia are the least likely to be able to walk, or if they can, to desire to walk, because their muscles are too tight and it is too much of an effort to do so. Some children with spastic quadriplegia also have hemiparetic tremors, an uncontrollable shaking that affects the limbs on one side of the body and impairs normal movement.
In any form of spastic CP, clonus of the affected limb(s) may sometimes result, as well as muscle spasms resulting from the pain and/or stress of the tightness experienced. The spasticity can and usually does also lead to very early onset of muscle-stress symptoms like arthritis and tendinitis, especially in ambulatory individuals in their mid-20s and early-30s. Physical therapy and occupational therapy regimens of assisted stretching, strengthening, functional tasks, and/or targeted physical activity and exercise are usually the chief ways to keep spastic CP well-managed, although if the spasticity is too much for the person to handle, other remedies may be considered, such as various antispasmodic medications, botox, baclofen, or even a neurosurgery known as a selective dorsal rhizotomy (which eliminates the spasticity by eliminating the nerves causing it). [edit]Ataxic Main article: Ataxic cerebral palsy Ataxia type symptoms can be caused by damage to the cerebellum. The forms of ataxia are less common types of cerebral palsy, occurring in at most 10% of all cases. [citation needed] Some of these individuals have hypotonia and tremors. Motor skills such as writing, typing, or using scissors might be affected, as well as balance, especially while walking. It is common for individuals to have difficulty with visual and/or auditory processing. [edit]Athetoid/Dyskinetic Main article: Athetoid cerebral palsy Athetoid cerebral palsy or dyskinetic cerebral palsy is mixed muscle tone – both hypertonia and hypotonia mixed with involuntary motions. People with Dyskinetic CP have trouble holding themselves in an upright, steady position for sitting or walking, and often show involuntary motions. For some people with dyskinetic CP, it takes a lot of work and concentration to get their hand to a certain spot (like scratching their nose or reaching for a cup). Because of their mixed tone and trouble keeping a position, they may not be able to hold onto objects, especially small ones requiring fine motor control(such as a toothbrush or pencil). About 10% of individuals with CP are classified as dyskinetic CP but some have mixed forms with spasticity and dyskinesia.[13] The damage occurs to theextrapyramidal motor system and/or pyramidal tract and to the basal ganglia. In newborn infants, high bilirubin levels in the blood, if left untreated, can lead to brain damage in in the basal ganglia (kernicterus), which can lead to dyskinetic cerebral palsy. [edit]Signs
and symptoms
All types of cerebral palsy are characterized by abnormal muscle tone (i.e. slouching over while sitting), reflexes, or motor development and coordination. There can be joint and bone deformities andcontractures (permanently fixed, tight muscles and joints). The classical symptoms are spasticities, spasms, other involuntary movements (e.g. facial gestures), unsteady gait, problems with balance, and/or soft tissue findings consisting largely of decreased muscle mass. Scissor walking (where the knees come in and cross) and toe walking (which can contribute to a gait reminiscent of a marionette) are common among people with CP who are able to walk, but taken on the whole, CP symptomatology is very diverse. The effects of cerebral palsy fall on a continuum of motor dysfunction which may range from slight clumsiness at the mild end of the spectrum to impairments so severe that they render coordinated movement virtually impossible at the other end the spectrum. Babies born with severe CP often have an irregular posture; their bodies may be either very floppy or very stiff. Birth defects, such as spinal curvature, a small jawbone, or a small head sometimes occur along with CP. Symptoms may appear or change as a child gets older. Some babies born with CP do not show obvious signs right away. Classically, CP becomes evident when the baby reaches the developmental stage at six and a half to 9 months and is starting to mobilise, where preferential use of limbs, asymmetry or gross motor developmental delay is seen. Secondary conditions can include seizures, epilepsy, apraxia, dysarthria or other communication disorders, eating problems, sensory impairments, mental retardation, learning disabilities, urinary incontinence, fecal incontinence and/or behavioral disorders. Speech and language disorders are common in people with cerebral palsy. The incidence of dysarthria is estimated to range from 31% to 88%. Speech problems are associated with poor respiratorycontrol, laryngeal and velopharyngeal dysfunction as well as oral articulation disorders that are due to restricted movement in the oral-facial muscles. There are three major types of dysarthria in cerebral palsy: spastic, dyskinetic (athetosis) and ataxic. Speech impairments in spastic dysarthria involves four major abnormalities of voluntary movement: spasticity, weakness, limited range of motion and slowness of movement. Speech mechanism impairment in athetosis involves a disorder in the regulation of breathing patterns, laryngeal dysfunction (monopitch, low, weak and breathy voice quality). It is also associated with articulatory dysfunction (large range of jaw movements), inappropriate positioning of the tongue, instability of velar elevation. Athetoid dysarthria is caused by disruption of the internal sensorimotor feedback system for appropriate motor commands, which leads to the generation of faulty movements that are perceived by others as involuntary. Ataxic dysarthria is uncommon in cerebral palsy. The speech characteristics are: imprecise consonants, irregular articulatory breakdown, distorted vowels, excess and equal stress, prolonged phonemes, slow rate, monopitch, monoloudness and harsh voice.[14] Overall language delay is associated with problems of mental retardation, hearing impairment and learned helplessness.[3] Children with cerebral palsy are at risk oflearned helplessness and becoming passive communicators, initiating little communication.[3] Early
intervention with this clientele often targets situations in which children communicate with others, so that they learn that they can control people and objects in their environment through this communication, including making choices, decisions and mistakes.[3] [edit]Skeleton In order for bones to attain their normal shape and size, they require the stresses from normal musculature. Osseous findings will therefore mirror the specific muscular deficits in a given person with CP. The shafts of the bones are often thin (gracile) and become thinner during growth. When compared to these thin shafts (diaphyses), the centers (metaphyses) often appear quite enlarged (ballooning). With lack of use, articular cartilage may atrophy, leading to narrowed joint spaces. Depending on the degree of spasticity, a person with CP may exhibit a variety of angular joint deformities. Because vertebral bodies need vertical gravitational loading forces to develop properly, spasticity and an abnormal gait can hinder proper and/or full bone and skeletal development. People with CP tend to be shorter in height than the average person because their bones are not allowed to grow to their full potential. Sometimes bones grow to different lengths, so the person may have one leg longer than the other. [edit]Pain
and sleep disorders
Pain is common, and may result from the inherent deficits associated with the condition, along with the numerous procedures children typically face.[15] There is also a high likelihood of suffering from chronic sleep disorders associated with both physical and environmental factors.[16] Pain is also associated with tight and/or shortened muscle, abnormal posture, stiff joints, unsuitable orthosis etc. [edit]Causes
Micrograph showing a fetal (placental) vein thrombosis, in a case of fetal thrombotic vasculopathy (FTV). FTV is associated with cerebral palsy and is suggestive of ahypercoagulable state as an underlying cause. H&E stain.
While in certain cases there is no identifiable cause, typical causes include problems in intrauterine development (e.g. exposure to radiation, infection),asphyxia before birth, hypoxia of the brain, and birth trauma during labor and delivery, and complications in the perinatal period or during childhood. [3] CP is also more common in multiple births. Between 40% and 50% of all children who develop cerebral palsy were born prematurely. Premature infants are vulnerable, in part because their organs are not fully developed, increasing the risk of hypoxic injury to the brain that may manifest as CP. A problem in interpreting this is the difficulty in differentiating between cerebral palsy caused by damage to the brain that results from inadequate oxygenation and CP that arises from prenatal brain damage that then precipitates premature delivery. Recent research has demonstrated that intrapartum asphyxia is not the most important cause, probably accounting for no more than 10 percent of all cases; rather, infections in the mother, even infections that are not easily detected, may triple the risk of the child developing the disorder, mainly as the result of the toxicity to the fetal brain of cytokines that are produced as part of the inflammatory response.[17] Low birthweight is a risk factor for CP—and premature infants usually have low birth weights, less than 2.0 kg, but full-term infants can also have low birth weights. Multiple-birth infants are also more likely than single-birth infants to be born early or with a low birth weight. After birth, other causes include toxins, severe jaundice, lead poisoning, physical brain injury, shaken baby syndrome, incidents involving hypoxia to the brain (such as near drowning), and encephalitis or meningitis. The three most common causes of asphyxia in the young child are: choking on foreign objects such as toys and pieces of food, poisoning, and near drowning. Some structural brain anomalies such as lissencephaly may present with the clinical features of CP, although whether that could be considered CP is a matter of opinion (some people say CP must be due to brain damage, whereas people with these anomalies didn't have a normal brain). Often this goes along with rare chromosome disorders and CP is not genetic or hereditary. It has been hypothetized that many cases of cerebral palsy are caused by the death in very early pregnancy of an identical twin.[18] [edit]Diagnosis
