DEFINITION Cerebral palsy (CP) is a neuro-developmental impairment caused by a non-progressive defect or lesion in single or multiple locations in the immature

brain. This defect causes cognitive and motor impairment and possible sensory deficits that are usually evident in early childhood. In cerebral palsy, faulty development or damage to motor areas in the brain impair the bodys ability to control movement and posture. This results in a number of chronic neurological disorders. Cerebral palsy is usually associated with events that occur before or during birth, but may be acquired during the first few months or years of life as the result of head trauma or infection. Cerebral palsy is neither contagious nor inherited, nor is it progressive. The symptoms ofcerebral palsy (CP) differ from person to person and change as children and their nervous systems mature. Some persons with severe CP are completely disabled and require lifelong care, while others display only slight awkwardness and need no special assistance. Complications associated with CP include learning disabilities, gastrointestinal dysfunction, tooth decay (dental caries), sensory deficits, and seizures. Types Cerebral palsy (CP) is classified as spastic, athetoid, ataxic, or mixed. These classifications reflect the type of movement disturbance displayed by the patient. Spastic CPstiff, permanently contracted muscles; 50% to 75% of cases Athetoid CP (also called dyskinetic cerebral palsy)slow, uncontrolled, writhing movements; 10% to 20% of cases Ataxic CPpoor coordination, balance, and depth perception; 5% to 10% of cases Mixed CPtwo or more types present; 10% of cases (percentage may be higher) Incidence Approximately 1 million people in the United States have CP. Improvements in prenatal, pediatric, and intensive care over the past 30 years have enabled more critically premature and frail babies to survive infancy. Many of these surviving children suffer developmental disorders and neurological damage. Causes Acquired Approximately 10% to 20% of children with cerebral palsy acquire it after birth, typically from brain damage sustained in the first few months or years of life. In such cases, the disorder may result from brain infections like bacterial meningitis or viral encephalitis, or from head trauma sustained from an accident, fall, or inflicted injuries (e.g., shaken baby syndrome).

Congenital The cause or causes of congenital CPthe type that is present at birthoften are unknown and many cases go undetected for months. Certain events during pregnancy, labor, and delivery can damage motor centers in the developing brain and cause cerebral palsy. However, birth complications account for only about 313% of congenital CP cases. Infections during pregnancy, such as German measles (rubella), can damage the fetuss developing nervous system. Other potentially damaging infections include cytomegalovirus and toxoplasmosis. Severe, untreated jaundice (hyperbilirubinemia) can damage brain cells in newborns and infants. Deprivation of oxygen to the brain (asphyxia) or head trauma sustained during labor and/or delivery can cause CP. Severe asphyxia for a lengthy period can produce brain damage called hypoxic-ischemic encephalopathy, which causes many infant deaths. Birth asphyxia is associated with spastic quadriplegia. Brain hemorrhage, or bleeding, can occur in the fetus during pregnancy or in newborns around the time of birth, damaging fetal brain tissue and causing neurological problems, including congenital CP. These hemorrhages are a type of stroke that may be caused by broken, abnormal, or clogged blood vessels in or leading to the brain, or by respiratory distress, a common breathing disorder in premature infants. Risk Factors Infants at the highest risk for developing cerebral palsy exhibit one or more of these factors: premature; low birth weight (<5 lb 7 1/2 oz); do not cry within 5 minutes of delivery; sustained on a ventilator more than 4 weeks; brain hemorrhage. Other risk factors include the following: Complications in pregnant mother (vaginal bleeding after 6th month, proteinuria, hyperthyroidism, high blood pressure, Rh incompatibility, mental retardation, seizures) Breech birth (born feet or buttocks first) Labor and delivery complications (vascular or respiratory problems; may indicate brain damage or abnormal brain development) Multiple births (twins, triplets, etc.; CP may be due to prematurity or intrauterine growth retardation) Birth defects (malformation of spinal bones, hernia in groin area, abnormally small jawbone, microcephaly) Newborn seizures Low Apgar score Infant heart rate, breathing, muscle tone, reflexes, and skin color are each scored as 0 (low), 1 (intermediate), or 2 (normal) after delivery. A total score of 7-10 at 5 minutes is considered normal; 4-6, intermediate; and 0-3, low. Scores that remain low 10-20 minutes after delivery indicate increased risk for CP.

Complications Some people with CP have associated disorders, such as impaired intellectual development, seizures, failure to grow and thrive, and vision and sense of touch problems. Roughly a third of patients with CP also have mild intellectual impairment; another third are moderately or severely impaired; and the remainder, intellectually normal. Mental impairment is most common in children with spastic quadriplegia. As many as half of all patients with cerebral palsy have seizures in which uncontrolled bursts of electricity disrupt the brains normal pattern of electrical activity. Seizures that recur without a direct trigger, such as a fever, are classified as epilepsy. Seizures generally are tonic-clonic or partial. Tonic-clonic seizures spread throughout the brain, typically causing the patient to cry out, followed by unconsciousness, twitching legs and arms, convulsive body movements, and loss of bladder control. Partial seizures are confined to one part of the brain and may be simple or complex. Simple partial seizures cause muscle twitching, chewing movement, and numbness or tingling. Complex partial seizures can produce hallucinations; staggering, random movement; and impaired consciousness or confusion. Children with moderate-to-severe cerebral palsy, especially those with spastic quadriplegia, often experience failure to grow or thriveinfants fail to gain weight normally; young children may be abnormally short; and teenagers may be short for their age and may have slow sexual development. These phenomena may be caused by a combination of poor nutrition and damage to the brain centers that control growth. Some patients, particularly those with spastic hemiplegia, have muscles and limbs that are smaller than normal. Limbs on the side of the body affected by CP may grow slower than those on the other side. Hands and feet are most severely affected. The affected foot in cases of hemiplegia usually is the smaller of the two, even in patients who walk, suggesting the size difference is due not to disuse but to a disrupted growth process. Vision and hearing problems are more common in people with cerebral palsy than in the general population. Differences in the left and right eye muscles often cause the eyes to be misaligned. This condition, called strabismus, causes double vision; in children, however, the brain often adapts by ignoring signals from one eye. Because strabismus can lead to poor vision and impaired depth perception, some physicians recommend corrective surgery. Patients with hemiparesis may have hemianopia, a condition marked by impaired vision or blindness in half of the visual field in one or both eyes. A related condition, called homonymous hemianopia, causes impairment in the right or left half of the visual fields in both eyes. Sensations of touch or pain may be impaired. A patient with stereognosis, for example, has difficulty perceiving or identifying the form and nature of an object placed in their hand using the sense of touch alone.

Hip dislocation, curvature of the spine (scoliosis), incontinence, constipation, tooth decay (dental caries), bronchitis, skin sores, and asthma are other complications commonly experienced by people with CP. PATHOPHYSIOLOGY Cerebral palsy involves insult to the higher brain centers such as cerebral cortex and/or corticospinal tracts (spastic), basal ganglia (athetoid), and cerebellum (ataxic). Spastic CP involves the descending tracts (corticospinal tract) that provide inhibition to the reflex arc. The spasticity results because there is too much facilitatory input from the spinal reflex arc generated by a stretch put on the muscle spindle. The spindle sends information to the spinal cord that usually is controlled by the descending tracts. When there is an injury to these tracts, there is an uncontrolled facilitation that causes the muscle to contract to take up the length on the spindle. The basal ganglia, a contributor to the extrapyramidal system, is a major center for movements related to posture, automatic movements (such as reciprocal arm swing during gait), and skilled volitional movements. It also monitors progress of movements and execution of learned motor plans. Insults to this area result symptoms such as akinesia, difficulty starting and stopping motion, rigidity, tremor or chorea. The cerebellum is the center for coordination of movement. Incerebral palsy, insults to this area result in such symptoms as hypotonia, disorders of balance and proprioception, ataxia, tremors, and dysmetria.

LINICAL MANIFESTATION Symptoms of cerebral palsy can be as simple as having difficulty with fine motor tasks like writing or using scissors, or as profound as being unable to maintain balance or walk. Severely afflicted patients may have involuntary movements, such as uncontrollable hand motions and drooling. Others suffer from associated medical disorders, such as seizures and mental retardation. Spastic CP is the most common type of cerebral palsy. It causes the muscles to be stiff and permanently contracted. Spastic cerebral palsy is often subclassified as one of five types that describe the affected limbs. The names of these types combine a Latin prefix describing the

number of affected limbs (e.g., di- means two) with the term plegia or paresis, meaning paralyzed or weak: Diplegiaeither both arms or both legs Hemiplegialimbs on only one side of the body Quadriplegiaall four limbs Monoplegiaone limb (extremely rare) Triplegiathree limbs (extremely rare) Spastic diplegia affects the legs more than the arms. The legs often turn in and cross at the knees. This causes a scissors gait, in which the hips are flexed, the knees nearly touch, the feet are flexed, and the ankles turn out from the leg, causing toe-walking. Learning disabilitiesand seizures are less common than in spastic hemiplegia. Persons with spastic hemiplegia (hemiparesis) also may experience hemiparetic tremors uncontrollable shaking of the limbs on one side of the body. Severe hemiparetic tremors can seriously impair movement. The arm is generally affected more than the leg. Learning disabilities, vision problems, seizures, and dysfunction of the muscles of the mouth and tongue are classic symptoms. Spastic quadriplegia involves all four limbs. There is dysfunction of the muscles of the mouth and tongue, seizures, medical complications, and increased risk for cognitive difficulties. Athetoid (or dyskinetic) cerebral palsy is characterized by slow, uncontrolled, writhing movements of the hands, feet, arms, or legs (athetosis). Patients also may have abrupt, irregular, jerky movements (chorea), a combination (choreoathetosis), or slow rhythmic movements with muscle tone abnormalities and abnormal postures (dystonia). The muscles of the face and tongue may be affected, causing grimacing and/or drooling. When the muscles that control speech are affected, the patient experiences dysarthria (abnormal pronunciation of speech). Hearing loss is commonly associated with this form of CP. Ataxic cerebral palsy affects balance and depth perception. Persons with ataxic CP have poor coordination and walk unsteadily, usually placing their feet far apart. Many have trouble with quick or precise movements, like writing or buttoning a shirt. Some also have intention tremor, in which a voluntary movement, like reaching for an object, sets off trembling in the limb. The tremor becomes more intense as the person nears the target object. Mixed CP involves two or more types of cerebral palsy. While any mix of types and subtypes can occur, the most common are athetodic-spastic-diplegic and athetoid-spastic-hemiplegic; the least common is athetoid-ataxic. It is possible to have a mix of all three (spastic-athetoid-ataxic).

MEDICAL MANAGEMENT A multidisciplinary team of health care professionals develops an individualized treatment plan based on the patients needs and problems. It is imperative to involve patients, families, teachers, and caregivers in all phases of planning, decision making, and treatment. A pediatrician, pediatric neurologist, or pediatric physiatrist (physician who specializes in physical medicine) provides primary care for children with CP. A family doctor, neurologist, or physiatrist provides primary care for adults with CP. The primary care provider gathers input from the health care team, synthesizes the information into a comprehensive treatment plan, and follows the patients progress. Other specialists on the team may include: Orthopedist or orthopedic surgeon to predict, diagnose, and treat associated muscle, tendon, and bone problems Physical therapist to design and supervise special exercise programs for improving movement and strength Speech and language pathologist to diagnose and treat communication problems Occupational therapist to help the patient learn life skills for home, school, and work Social worker to help patients and their families obtain community assistance, education, and training programs Psychologist to help address negative or destructive behaviors, and guide the patient and his/her family through the stresses and demands presented by cerebral palsy.

The need for and types of therapy change over time. Adolescents with CP may need counseling to cope with emotional and psychological challenges. Physical therapy may be supplemented with special education, vocational training, recreation, and leisure programs. Adults may benefit from attendant care, special living accommodations, and transportation and employment assistance services, depending upon his or her intellectual and physical capabilities. Physical Therapy It is important for physical therapy to begin soon after diagnosis is made. Daily range of motion exercises help prevent muscles from growing weak and atrophied or rigidly fixed from contracture. Normally, muscles and tendons stretch and grow at the same rate as bones. Spasticity can prevent stretching, and muscle growth may not keep up with bone growth. The muscles can become fixed in stiff, abnormal positions. Physical therapy, often in combination with special braces, helps prevent contracture by stretching spastic muscles. It also can improve a childs motor development. To prepare a child for school, the focus of therapy gradually shifts toward activities associated with daily living and communication. Exercises are designed to improve the childs ability to sit, move independently, and perform tasks such as dressing, writing, and using the bathroom. Orthotics can help control limb position, and walkers can help some patients walk. Mastering such skills reduces demands on caregivers and helps the child obtain some degree of selfreliance, which helps build self-esteem. Speech therapy Children with athetoid (dyskinetic) CP often have trouble pronouncing words (dysarthria), and swallowing (dysphagia). Difficulty with swallowing causes eating problems and drooling. Speech therapy can help improve swallowing and communication. A speech therapist also can work with the child to learn to use special communication devices like computers with voice synthesizers. Psychotherapy Behavioral therapy can complement physical therapy, employing psychological techniques that encourage the mastery of tasks that promote muscular and motor development. Praise, positive reinforcement, and small rewards can encourage a child to learn to use weak limbs, overcome speech deficits, and stop negative behaviors like hair pulling and biting. Medication As with many forms of drug therapy, a certain amount of experimentation may be required before optimum results are achieved. Seizures No single drug controls all types of seizures, and no two patients respond identically to any

given drug. Medications are divided into first-generation anticonvulsants (older medications) and second-generation anticonvulsants (more recently developed). Spasticity The muscle relaxants diazepam (Valium) and dantrolene (Dantrium) may be prescribed to control muscle contraction (myoclonus). These drugs reduce spasticity for short periods, and their long-term value is uncertain. The long-term effect of these drugs on a childs developing nervous system is unknown. Side effects of diazepam include drowsiness, slurred speech, constipation, nausea, and incontinence. Common side effects of dantrolene include drowsiness, dizziness, general weakness, and diarrhea. The most common side effect of phenobarbitol is drowsiness. Other effects include dizziness, slowed heart rate, confusion, constipation, and nausea. Phenobarbitol and the other barbiturates can become habit forming.

Baclofen is a muscle relaxant and antispastic medication that is available in tablet and injectable forms. Intrathecal baclofen uses a very small implanted pump to deliver a steady supply of medication into the fluid around the spinal cord. Strict compliance with the refill schedule is imperative to avoid abrupt withdrawal and resulting severe complications, including death. Side effects associated with baclofen include confusion, dizziness, drowsiness, headache, insomnia, nausea, hypotension, and urinary frequency. Anticholinergics may be prescribed to control the abnormal movements associated with athetoidcerebral palsy. These drugs inhibit the effects of acetylcholine, a chemical in brain cells that triggers muscle contraction. The most commonly prescribed anticholinergic drugs are trihexyphenidyl (Artane), benztropine (Cogentin), and procyclidine hydrochloride (Kemadrin). Side effects associated with anticholinergic drugs include dry mouth, constipation, agitation, and painful urination (dysuria). Botulinum toxin, or BOTOX Cosmetic, is injected directly into muscle. BOTOX Cosmetic blocks acetylcholine and alleviates muscle spasm for 3-6 months. Botulinum toxin may produce muscle weakness. In some cases physicians may try to reduce spasticity or correct a developing contracture by injecting phenol into a muscle. This weakens the muscle and gives physicians andtherapists a chance to stretch and lengthen the muscle with therapy, bracing, or casts. If the contracture is treated early enough, the need for surgery may be avoided. Mechanical Aids A variety of devices and mechanical aids can help patients with cerebral palsy overcome physical limitations. These range from simple Velcro shoe straps to motorized wheelchairs and computerized communication devices.

Computers can transform the lives of cerebral palsy patients. Fitted with a light pointer attached to a headband and a voice synthesizer, they can give a child unable to speak or write the power of communication using nothing but simple head movements. Casting and splinting for 2-3 months can improve range of motion (ROM) of a joint and decrease muscle tone for 3-4 months. Orthopaedic Surgery Orthopaedic surgery can prevent spinal deformities and contractures but requires particular expertise and appropriate rehabilitation following surgery. When contractures cause severe movement problems, surgery to lengthen muscles and tendons may be recommended. Gait analysis is used to identify problem muscles, eliminate compensation factors associated with walking, and check surgical results. Cameras and computers record and analyze the patients walk, and electromyography records muscle activity. Surgically lengthening a muscle makes it weaker and may require months of recovery. Whenever possible, therefore, doctors try to fix affected muscles with a single surgery. If more than one procedure is required, operations usually are scheduled as close together as possible. Neurosurgery Dorsal root rhizotomy treats spasticity in the legs by selectively severing nerves that overstimulate leg muscles. The procedure is controversial, but it can benefit some CP patients, particularly those with spastic diplegia. Stereotactic neurosurgery may improve rigidity, choreoathetosis, and tremor. The area of the brain treated controls the part of the body meant to be improved. A head CT scan is performed to produce images of the brain. Coordinates of the location to be treated are moved to a stereotactic frame. The frame is used like a map to guide an electrode to the target area in the brain. An electrical impulse is sent through the electrode and into the brain tissue to change the brain cells. Stereotaxic thalamotomy can reduce some hemiparetic tremors. This procedure involves cutting parts of the thalamus, the brains center for relaying messages from the muscles and sensory organs. There is significant risk with this procedure. Chronic cerebellar stimulation is an experimental technique that uses electrodes surgically implanted on the surface of the cerebellum the part of the brain that coordinates movement to decrease spasticity and improve motor function by stimulating certain nerves. Some studies claim the procedure improves spasticity, while others report less satisfactory results. Treating Complications Incontinence, caused by faulty control over the bladder muscles, can be treated with exercises, biofeedback, prescription drugs, surgery, or surgically implanted devices that replace or aid muscles. Drooling, caused by poor functioning of the muscles of the throat, mouth, and tongue, can be difficult to control. Anticholinergic drugs reduce the flow of saliva but may produce side effects like mouth dryness and poor digestion. Surgery sometimes helps, but also can worsen

swallowing problems. Biofeedback has been proven helpful in patients who are intellectually unimpaired and motivated. Eating and swallowing problems may require the caregiver to prepare food in a semisolid fashion, such as strained vegetables and fruits. Physical therapy can support and promote proper positioning while eating or drinking, or help extend the neck away from the body to reduce the risk of choking. Severe swallowing problems may necessitate the use of a tube to deliver food down the throat and into the stomach. Gastrostomy, which involves making a surgical opening in the abdomen that allows tube-feeding directly into the stomach, is sometimes necessary.

Prevention The best prescription for having a healthy baby is to have regular prenatal care and good nutrition, and to avoid smoking, alcohol consumption, and drug abuse. Other forms of prevention include: Prevent head injuries by using child safety seats in automobiles and helmets when riding bicycles, skateboards, etc. Supervise young children closely during bathing and swimming. Prevent child abuse. Keep poisons out of reach. Treat jaundice in newborns with phototherapy. Exposing jaundiced babies to special blue lights that break down bile pigments and prevent them from building up and causing brain damage. In rare cases, a blood transfusion may be necessary. Identify potential Rh incompatibility in expectant mothers. Incompatible Rh factor usually is not problematic with first pregnancies, as the mothers body typically produces the unwanted antibodies only after delivery. A special serum given after each delivery can prevent the production of antibodies. If antibodies develop during a first pregnancy, or if antibody production is not prevented, the developing baby is monitored closely. If necessary, a transfusion may be given to the infant in the womb or an exchange transfusion may be given after birth, removing and replacing a large volume of the infants blood. Be vaccinated against German measles (rubella) before becoming pregnant. Alternative Therapies Adults with CP may benefit from alternative therapies such as acupuncture, massage, osteopathy, and homeopathy. Support Groups To meet the challenges of cerebral palsy, patients, families, and caregivers need help and support. There are many government-supported and private voluntary groups that provide information about prevention, diagnosis, and treatment, as well as clinical and support services. Prognosis

Most people with cerebral palsy experience a normal life span. Those with severe forms of CP may have a reduced life span. As people with cerebral palsy age, they may experience longterm effects of chronic physical impairment, such as the following: Increase in spasms Increase in shortening of muscles (contractures) Joint problems (e.g., pain, loss of flexibility) Tight muscles Increase in back pain Emergence of incontinence Increase in incontinence Reduced energy levels Factors that contribute to these effects include: Poor wheelchair seating and posture Spinal deformities Weight gain Severe learning disability Discontinuance of physical therapy Walking when it is increasingly difficult Inappropriate orthopaedic surgery Physical exhaustion from inadequate rest or recovery from illness or injury can lead to an overall decline in physical and mental function. Ways to maintain physical function include the following: Appropriate wheelchair seating and posture Assume various positions out of the wheelchair Use a wheelchair when fatigued and when walking is difficult Regular and appropriate exercise, including stretching exercises and exercises to maintain flexibility in joints Maintain ideal weight Have regular medical check-ups Avoid sustained mental stress Carefully consider proposed surgery Plan for appropriate rehabilitation after surgery NURSING MANAGEMENT A child with cerebral palsy may be hospitalized for orthopedic surgery to correct contractures and for treatment of other complications. Assign the child a room with children in the same age-group. Speak slowly and distinctly. Encourage the child to ask for things he wants. Listen patiently and dont rush him.

During meals, maintain a quiet, unhurried atmosphere with as few distractions as possible. The child may need special utensils and a chair with a solidfootrest. Teach him to place food far back in his mouth to facilitate swallowing. Encourage the child to chew food thoroughly, drink through a straw, and suck on a lollipop between meals to develop the muscle control needed to minimize drooling. Allow the child to wash and dress independently, assisting only as needed. Give all care in an unhurried manner; otherwise, muscle spasticity may increase. Encourage the child and his family to participate in the care plan so they can continue it at home. Minimize muscle spasms that increases postoperative pain by moving and turning the child very carefully after surgery. After orthopedic surgery, give good cast care. Wash and dry the skin at the edge of the cast frequently, and rub it with alcohol. Reposition the child often, check for foul odor, and ventilate under the cast with a blow dryer. Use a flashlight to check for skin breakdown beneath the cast. Help the child relax, perhaps by giving a warm bath, before reapplying a bivalved cast. Help parents deal with their childs handicap. A good understanding of normal growth and development will enable you to work with parents to set realistic goals. Assist in planning crafts and other activities. Stress the childs need to develop peer relationships; warn against being overprotective. Identify and deal with family stress. Parents may feel unreasonable guilt about their childs handicap and may need psychologic counseling. Make a referral to supportive community organizations.