Cerebral Palsy Definition Cerebral palsy (CP) is a clinical entity characterized by a three-part definition: a disorder of movement and posture

(1) caused by a nonprogressive injury (2) to the immature brain (3). The distinctive characteristic of these syndromes is the change in muscle tone and posture, both at rest and with voluntary activity. The definition of CP implies that the underlying pathologic process in the brain does not progress, and occurred during early development of the brain. The first year or two of life is included in most definitions, although it is unclear what the upper age limit is of a postneonatal brain insult. The definition of CP is being reviewed at present. The diagnosis is made in children who are demonstrating delayed motor development, and is confirmed with MRI. Where indicated, other metabolic, biochemical, or genetic testing might be necessary. There is a subset of children for whom no imaging abnormality is seen. Epidemiology Cerebral palsy is one of the most common disabling conditions affecting children. The reported incidence varies but is approximately 1-2.3 per 1000 live births.203 The Collaborative Perinatal Project measured a prevalence rate of 5.2 per 1000 live births at 1 year of age, but reported resolution in up to half of these children by 7 years of age. Discrepancies in the rate are due to difficulties in diagnosis for a variety of reasons. Diagnosis is not made at any specific age, and can 'resolve' in up to 50% of children diagnosed prior to 2 years of age, or the brain insult might not occur until later in childhood. Various terms are also used, including neonatal encephalopathy, birth asphyxia, periventricular leukomalacia (PVL), hypoxic brain injury, stroke, traumatic brain injury, and shaken infant syndrome. There is also a wide variation in normal attainment of motor milestones, making early diagnosis less reliable. There were hopes that recent improvements in neonatal care would decrease the incidence of CP, but the prevalence in full-term infants has remained relatively constant. Despite improved neonatal outcomes in general, the increased survival of premature low birth weight (<2500 g) to extremely low birth weight (<1000g) infants with higher CP risk has kept the prevalence of CP in childhood relatively constant. Infants born between 32 and 42 weeks' gestation, with a birth weight below the 10th percentile, had

four to six times higher risk of CP compared with those between the 25th and 75th percentiles for birth weight. Maternal mental retardation, maternal seizure disorder, and hyperthyroidism; two or more prior fetal deaths; a sibling with motor deficit; third-trimester bleeding or increased urine protein excretion; and fetal bradycardia, chorioamnionitis, low placental weight, fetal malformations, and neonatal seizures all increase CP risk in term or near-term infants. Multiple birth pregnancies also have a higher risk of CP, and it more often occurs in the second twin.108 Before pregnancy, increased CP risk is associated with long menstrual cycles or repeated fetal loss during pregnancy, fetal growth retardation, congenital malformations, abnormal fetal presentation, or low socioeconomic class. During labor and delivery, only premature separation of the placenta and tight nuchal cord pose an associated CP risk. Despite these many associations, most children with these risk factors do not develop CP. Etiology Risk factors associated with cerebral palsy General • Gestational age <32 weeks • Birth weight <2500 g Maternal history • Mental retardation • Seizure disorder • Hyperthyroidism • Two or more prior fetal deaths • Sibling with motor deficits During gestation • Twin gestation • Fetal growth retardation • Third-trimester bleeding • Increased urine protein excretion • Chorionitis • Premature placenta separation • Low placenta weight Fetal factors • Abnormal fetal presentation « Fetal malformations • Fetal bradycardia • Neonatal seizures

The brain injury that leads to CP can occur in the prenatal, perinatal, or postnatal period. The causes of these lesions have been attributed to a wide variety of brain injury mechanisms. It is now thought that most causes of CP occur in the prenatal period. Prenatal causes include TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes simplex, other) infections, intrauterine stroke, and genetic malformation. The most common currently understood causes are related to brain injury occurring in children born prematurely. The combination of immaturity, fragile brain vasculature, and the physical stresses of prematurity predisposes these children to compromised cerebral blood flow. The blood vessels are particularly vulnerable in the watershed zone next to the lateral ventricles in the capillaries of the germinal matrix. Bleeding in this area is arterial in origin and can occur in differing degrees: cerebral intraventricular hemorrhage isolated to germinal matrix (grade 1), intraventricular hemorrhage with normal ventricular size (grade 2), intraventricular hemorrhage with ventricular dilatation (grade 3), or intraventricular hemorrhage with parenchymal hemorrhage (grade 4). This can be detected early on using transfontanelle cerebral high-resolution ultrasound. Very low birth weight infants also have an increased incidence of periventricular hemorrhagic infarction, which is hemorrhagic necrosis lateral to the external angle of the lateral ventricle. This is thought to be bleeding of venous origin and is usually asymmetric. With healing of this bleeding, symmetric necrosis of white matter adjacent to the external angle of the lateral ventricles (PVL) can develop. PVL is one of the strongest predictors of CP in the premature neonate. PVL is almost always associated with a history of prematurity. Extremely low birth weight infants are also at increased risk of CP, especially with a history of resuscitation and prolonged ventilation. Protective factors were prenatal care and steroids. Almost half of all children with CP were not born prematurely. For term births that result in CP, the cause of brain injury is often elusive. Although uncommon, most known perinatal injuries that cause CP are due to severe anoxic or ischemic brain injury. This can occur with mechanical difficulties of the placenta, umbilical cord, or the actual delivery itself. Intrapartum asphyxia must be severe and prolonged to cause CP. Injuries of this type unfortunately tend to be more global and are more likely to cause a more severe disability. Less that 10% of cases are thought to be due to birth asphyxia, based on specific criteria that include pH of the cord blood Chorioarrmionitis has recently been found to be associated with a 4.1 times increased risk for CP. Maternal autoimmune disease coagulation disorders

are also associated with CP. Inflammatory mediators in cord serum, and other protein markers, have also been noted in small studies. Genetic causes can be associated with other congenital malformations. Athetoid CP is associated with injury to the basal ganglia. This is most often caused by hyperbilirubinemia, and is associated with hearing loss. Incidence has declined since testing treatment for Rh incompatibility has become routine, and re is now relatively rare. There are many potential causes for postnatal cerebral injury and CP. The major causes for this include central nervous system infection, vascular causes, and head injury. Others include anoxia, ischemia, and inflammation. Much work is needed in the areas of prevention of CP in the full-term neonate and in postneonatal causes. When deterioration or regression in developmental milestones is seen, referral for metabolic testing should be considered. Classification: The most commonly used classification system describes the type of tone abnormality as spastic, dyskinetic (dystonic or athetoid), or mixed, and some systems also include ataxic and hypotonic subtypes. The distribution of limb involvement is characterized as monoplegia, diplegia (both legs affected more than arms), hemiplegia (arm frequently more affected than leg), and quadriplegia. Triplegia is a combination of diplegia and hemiplegia. It has been suggested that the terms diplegia, quadriplegia, and hemiplegia be replaced by bilateral and unilateral involvement. As the upper limb involvement becomes more severe, it becomes more difficult to determine whether the upper or lower extremity is more involved. This has led to inconsistencies in classifying diplegia and quadriplegia. For this reason, the Gross Motor Functional Classification System (GMFCS), which stratifies children based on gross motor mobility, is favored by some. The GMFCS classification does not describe the limbs affected, so the GMFCS classification is typically used in addition to describing the tone disorder and the limb distribution. The areas of brain involvement on MRI can also be helpful to predict areas of difficulty. Generally, the vast majority of children (over 85%) with hemiplegia can eventually walk independently. For unclear reasons, isolated right hemiplegia is twice as common as left hemiplegia. The spastic subtype is the most common, affecting about 75% of children with CP. Spasticity is defined as velocity-dependent increased tone, associated with upper motor neuron syndrome findings of increased muscle stretch reflexes, Babinski's response, weakness, and difficulty with

coordination. It can be associated with extensor or flexor posturing (decorticate or decerebrate). Dystonia is defined as a dyskinetic tone abnormality with alternating tone or co contraction in the antagonist and agonist muscle groups, causing varied abnormal postures and often fluctuating tone. The other dyskinetic forms are athetosis, choreiform and choreoathetoid. Athetoid movements are involuntary constant rotatory or writhing movements of the distal extremities, and are associated with basal ganglia involvement. These movements stop during sleep. The incidence of the athetoid type has drastically been reduced by the advent of treatment for Rh incompatibility. The ataxic form is rare, and must be differentiated from degenerative processes of the cerebellum. Spasticity and dystonia frequently coexist in CP, as the mixed form of tone abnormality. Studies to differentiate spasticity from dystonia using clinical assessment or gait analysis are important, as some treatments control spasticity or dystonia more effectively. Surgical outcomes are much less predictable in children with dystonia, as tone fluctuates. A form of dystonia that is responsive to dopaminergic medication is well described. A small percentage of patients have the hypotonic type of CP. These children need to be differentiated from those with identifiable causes of neonatal hypotonia such as muscle disease, metabolic disorders, and genetic syndromes. Many of these children develop spastic or extrapyramidal-type disorders after the first few months of life. Clinical effect: Hypotonia and motor delay are often seen as early signs of CP. The severity of involvement varies widely, depending on the extent of neurologic involvement. Upper motor neuron injury features include positive findings of increased tone and reflexes, positive Babinski's reflex, and negative findings of reduced strength, selective motor control, balance, and coordination. About three-quarters of children with CP have spasticity, although the first several months can be characterized with hypotonia. Early on, assessment reveals tone in the extremities, and retention of primitive reflexes can be noted. Obligatory primitive reflexes that the child cannot emerge from are always abnormal. Examples of these are the asymmetric tonic neck reflex (ATNR), symmetric tonic neck reflex (STNR), and tonic labyrinthine reflex. Other problems in infancy that suggest CP include irritability, lethargy, weak suck with tongue thrust, poor head control, high-pitched cry, oral hypersensitivity, tonic bite, and asymmetric movements or unusual posturing. Later on, the child might be noted to be

rolling for mobility, combat crawling, 'W sitting', 'bunny hopping', or adopting a hand preference before the first birthday. Trunk or central hypotonia often remains throughout life. Abnormalities of muscle tone are frequently accompanied by muscle weakness. As tone develops in children with spasticity, abnormal posturing such as 'scissoring', 'guarding' of the upper extremities, extensor posturing, or proximal 'fixing' can be seen. Scissoring is the simultaneous adduction, knee hyperextension, and plantar flexion of the lower extremities. Flexion synergy patterns of the upper extremities include flexion at the fingers, wrists, and elbows with shoulder abduction. As this upper extremity pattern becomes stronger, the child's hands rise from the waist, producing a Tow, mid, or high guard position. The abnormalities of muscle tone are often accompanied by weakness in individual muscles. Applying the traditional methods of measuring muscle strength is problematic in CP, because the tone abnormalities mask the patient's ability to generate force. Modified sphygmomanometer and hydraulic force measures can provide better reliability than Medical Research Council muscle strength grades. Sensory issues are also well recognized in CP, which affect motor function, such as hand manipulation ability. Hypersensitivity can interfere with development of hand and lower extremity weight-bearing skills. The abnormal sensory experience of disordered motor control can contribute to disordered sensory perception, which further interferes with the child's ability to perform high-level motor activities. Decreased ability to distinguish two-point discrimination has been found in the upper extremities of children with all types of CP. Children with hemiparetic CP have also been found almost universally to have a decrease in stereognosis, with decreased proprioception in about half of patients tested. The side of involvement with cortical lesions is also known to cause specific issues, such as movement planning with left hemisphere injury. Balance difficulties can be due to a combination of weakness, abnormal tone, and sensory issues, including visual perceptual skills. The major secondary effects of disordered muscle tone, control, and balance are changes in joint alignment, leading to contracture and deformity. Contracture (passive shortening that can limit joint and soft tissue movement) frequently affects the adductor, hamstring, and plantar flexor muscles of the lower extremities and the flexors of the upper extremities. This reflects the presence of spasticity, scissoring, or upper extremity flexion patterns, individually or severally, which are present in the majority of children with spastic types of CP.

Bony deformity can occur because the abnormal muscle forces of CP act on a growing skeleton. The integrity of the hips and spine are a principal concern, because of their primary role in weight bearing and positioning. The femur is affected by muscle and gravity-loading forces during growth. Muscle forces in CP can cause increased anteversion of the femur neck. The typical increase in hip flexion, adduction, and internal rotation of the femur acts to influence the femoral head in a superoposterolateral direction out of the acetabulum. The result is coxa valgus, deformation of the femoral head, and a shallow acetabulum, which causes the hip to be more prone to subluxation . Asymmetric muscle pull and immobility can contribute to significant deformity of the spine, including kyphosis, scoliosis, or rotational deformities. These spinal deformities can significantly affect comfort, tone, sitting and standing alignment, and balance. When these are severe, respiratory function can be compromised by the mechanical restriction of the chest, combined with decreased efficiency of available respiratory muscle strength. This can have a significant impact on endurance, health, and longevity. Spondylolisthesis and spondylolysis are not increased in CP. Case reports of atlantoaxial instability in spastic CP have been reported. Bony abnormalities of the feet can occur in a variety of patterns. The most common is deformity of the hind foot with exaggerated heel valgus or varus. Hyperpronation occurs frequently with calcaneovalgus. Occasionally a rocker bottom type of foot can be seen.

Many of the normal developmental milestones, such as reaching for toys (3-4 months), sitting (6-7 months), and walking (10-14 months), are based on motor function. A physician may suspect cerebral palsy in a child whose development of these skills is delayed. In making a diagnosis of cerebral palsy, the physician takes into account the delay in developmental milestones as well as physical findings that might include abnormal muscle tone, abnormal movements, abnormal reflexes and persistent infantile reflexes. Making a definite diagnosis of cerebral palsy is not always easy, especially before the child's first birthday. In fact, diagnosing cerebral palsy usually involves a period of waiting for the definite and permanent appearance of specific motor problems. Most children with cerebral palsy can be diagnosed by the age of 18 months, but eighteen months is a long time for parents to wait for a diagnosis, and this is understandably a difficult period for them. Making a diagnosis of cerebral palsy is also difficult when, for example, a two-year- old has suffered a head injury. The child may immediately appear to be severely injured, and three months after the injury he may have symptoms that are typical of a child

with cerebral palsy. But one year after the injury such a child may be completely normal. This child does not have cerebral palsy. Although he has a scar on his brain, the scar is not permanently impairing his motor activities. After injury, waiting and observing are necessary before the diagnosis can be made. Magnetic resonance imaging (MRI) and Computed Tomography (CT) scans are often ordered when the physician suspects that the child has cerebral palsy. These tests may provide evidence of hydrocephalus (an abnormal accumulation of fluid in the cerebral ventricles), and they may be used to exclude other causes of motor problems. These scans do not prove whether a child has a cerebral palsy; nor do they predict how a specific child will function as she grows. Thus, children with normal scans may have severe cerebral palsy, and children with clearly abnormal scans occasionally appear totally normal or have only mild physical evidence of cerebral palsy. As a group, though, children with cerebral palsy do have brain scars, cysts, and other changes which show up on scans more frequently than in normal children.

Associated medical and functional problem A number of associated medical issues are also commonly seen in children and adults with CP that can interfere with development and general health. These also require attention and management, and often involvement of a number of healthcare professionals. Visual sequelae occur due to the central visual pathways being affected. Strabismus is common in children with diplegia, including exotropia or esotropia. Hemiplegia can be associated with hemianopsia. Premature infants can also have had retinopathy of prematurity. Forty percent of premature children have visual sequelae. Hearing impairment can be related to ototoxic drug exposure, hyperbilirubinemia, or TORCH infection in utero. Early assessment of hearing and vision is important in all children with CP. A large number of children with CP have abnormalities of oral motor function. These deficits include drooling, dysphagia, and dysarthria, due to weakness and incoordination of lips, tongue, and masticatory and facial muscles. Drooling can also reflect poor swallowing ability. It can cause problems socially for schoolage children, and can cause rash and dental problems. Treatment can include behavioral techniques, speech therapy, anticholinergic medications, botulinum toxin A injections, and surgical redirection of the salivary gland ducts. Airway patency can be obstructed in some children due to spastic, floppy, or stenosed tissues in the upper airway. Difficulties with choking or apneic spells during sleep, stridor, or snoring can occur. Tracheostomy is sometimes required to maintain control of the airway.

Dysphagia can be due to a problem in the oral phase (chewing and bolus preparation) or in the laryngeal phase (propulsion and airway protection problems). In one large study of patients with suspected oropharyngeal dysfunction, over half of those tested aspirated. The majority of those patients have no effective cough during aspiration. Swallowing dysfunction is often present only with certain textures of food ingested, as detected by videoflouroscopy. In severe cases, aspiration can cause hypoxemia. Feeding difficulties can contribute to substantial undernutrition or malnutrition. Up to one-third of children with hemiplegia and diplegia, and more than two-thirds with quadriplegia, have been found to be undernourished. The North American Cerebral Palsy Growth Project demonstrated that 27% of children with CP were malnourished, based on triceps skin fold measures below the 10th percentile. Due to the difficulty in maintaining adequate nutrition orally, other methods might be required, such as gastrostomy or gastrojejunostomy tube. Gastrointestinal symptoms are frequent in children with CP. Gastroesophageal reflux, due to weakness of the lower esophageal sphincter, can cause episodic emesis. It can also interfere with adequate ingestion and absorption of nutrients, and compromise adequate nutrition and growth. Medications, or surgical Nissen fundoplication or jejunostomy are options to reduce reflux. Chronic constipation is a significant concern for many children with CP. This can be in part due to neuromuscular control of the bowel, and exaggerated by immobility and abnormal diet and fluid intake. Abnormal segmental colonic transit times have been noted, and are correlated with ambulatory function. Increasing activity, and fluid and fiber intake can improve constipation, along with the judicious use of medications. Cognitive impairments are not obligatory with CP but can occur to varying degrees. The prevalence of mental retardation in all persons with CP is estimated to be 30%. The risk increases with the severity of the motor disability. Specific learning disabilities can be present in 20-30%. Normal birth weight children with CP have had cognitive abnormalities reported in up to 40-50%. Seizure disorders can occur in up to one-third of children with CP. Children with hemiplegia have the highest rate, followed by those with quadriplegia, and then diplegia. Presence of seizures typically reflects a greater extent of cortical brain injury. Newer antiseizure techniques using grid mapping and resection of seizure foci are now part of the standard treatment options.

Osteoporosis is present in children with CP due to multiple factors. Calcium and Vitamin D can be deficient, due to the reduced caloric intake diet of people with CP. Decreased weight bearing and muscle stresses can contribute to osteoporosis. Antiseizure medications can interfere with vitamin D metabolism. Pathologic fractures, or fractures with even minimal transfer activity, are not uncommon and occur more frequently after a period of immobilization. Decreased femoral bone mineral density was found to be associated with feeding difficulty, anticonvulsant use, weight percentile, and low triceps skin fold. Studies on increased weight bearing as an intervention show varying results on bone mineral density. Calcium and vitamin D supplementation show positive effects on bone density. Injuries, abuse, and neglect are also potential causes for morbidity and mortality in children with CP. The usual preventive strategies for childhood injury should be employed. Injuries from use of equipment used for mobility can be of concern. Neglect is the most common form of childhood maltreatment, and is about twice as common in disabled as in non-disabled children. In most jurisdictions, healthcare professionals who suspect that a child is being subject to maltreatment are obligated to report it to the child protective services. Functional prognosis Children typically develop motor skills craniocaudally. A child will first develop neck control, then upper trunk control, and then lower trunk control. Sitting balance follows, then standing. The age at which these skills are developed help to predict the eventual outcome. Those children who will attain independent ambulation typically achieve these skills by the age of 8, and only rarely later. Seventy-five percent of children with spastic CP eventually ambulate, about 85% with diplegia, and 70% with quadriplegia. Most children with hemiplegic or ataxic CP ambulate independently, the majority by age 3 years. Children who are able to sit independently before their second birthday eventually become independent walkers, with or without equipment. Children who have not attained independent sitting by age 4 years rarely walk. However, if the primitive reflexes (ATNR, STNR, tonic labyrinthine, Moro's, positive supporting reflex, or extensor posturing) still occur in an obligatory manner, the prognosis for independent ambulation is poor. Most children who will walk have fewer than three of these reflexes present at age 18 months. The combination of increased tone and underlying muscle weakness greatly increases the energy expended for motor activity. Children with CP who achieve ambulation typically have an inefficient gait, which includes

much shorter step length, decreased range of motion at the hip and knee, more energy expenditure, and decreased velocity as compared with their peers. All these inefficiencies of muscle control contribute to decreased endurance. Although endurance is difficult to measure with conventional methods because of the challenges of various impairments with CP, it seems to correlate with the overall gross motor capabilities of the individual. Energy cost of walking is found to correlate to GMFCS level. Contractures and bone or joint deformities can also greatly limit a child's function. Therapeutic intervention The therapeutic management of the child with CP emphasizes a functional aim- or goal-oriented approach. The two major goals of rehabilitation, according to Molnar are to decrease complications of the CP and to enhance or improve the acquisition of new skills. Rothery and colleagues include parent and caregiver education, decreasing skeletal deformity, and improving mobility. The role of the physician is to provide an accurate assessment of the child's impairments. In the case of the child with CP, this can include learning disabilities, mental retardation, respiratory compromise, fractures, and other comorbidities that can limit function. In addition to the impairments, all precautions should be identified that can pose a risk to the child during therapy. Seizures, osteopenia, and gastroesophageal reflux occur frequently and can have an impact on the treatment.196 From the first evaluation, the family and child should be encouraged to become active participants in the process of setting priorities and goals within the context of the impairment. The institution of therapy and other medical modalities should be approached and prescribed after a thorough discussion of efficacy. The following discussion represents the major therapeutic interventions for children with CP. Early intervention: The rationale for early intervention is closely connected to concepts that stress the importance of the early years for normally developing children, and the role that environmental factors play in development. Parental characteristics have been found to be associated with a child's concurrent or later developmental functioning. These parental characteristics include responsively contingent on child initiations, the quality and quantity

of verbal interactions, the existence of a social support network, and maternal sensitivity. There are two main models for the delivery of developmental early intervention: the direct therapy service model and the consultation model. In the direct model, service delivery can occur as part of interdisciplinary, multidisciplinary, or trans-disciplinary treatment in a center- or home-based program. Physical therapists and occupational therapists have traditionally been providers of therapeutic intervention for the child with CP. Many children are also seen by a speech-language pathologist for feeding difficulties and oromotor problems. A social worker and developmental specialist constitute the members of a typical early intervention team. To reduce the number of therapists handling the child, Haynes has advocated that one professional from the early intervention team integrate assessment data from all the members of the team, developing integrated strategies to meet the goals agreed in the IFSP. Therapy approaches Children with CP who have motor problems that interfere with educational activities have the opportunity to participate in integrated therapy programs in school. The services should be delivered in the least restrictive environment, and are mandated to allow the child to participate in and benefit from the educational experience. The current philosophic trend in schools is to include disabled children as much as possible into the regular classroom, and few 'pull out' services are stressed. Mainstreaming has positive effects on the psychosocial aspects of schooling and increases the academic expectations of the disabled student. Consultative models of PT, occupational therapy (OT), and speech therapy have provided a method of treating and involving more of the children with disabilities into regular education classes. Even though there is a move toward consultative services by PT, OT, and speech therapy in the school systems, Cerebral Palsy direct service can be recommended to augment an overall therapy program. There are a number of therapy systems that have influenced the management of children with CP. Rarely does the clinical delivery of therapy services rely on any one system. An eclectic approach is common—one that offers modalities from several schools of therapy. Such an approach provides the flexibility and individualization necessary to meet the child's and the family's goals. A shift to functional goal setting that involves the parents has become prominent since the 1980s. PT had focused on impairment, with less emphasis on the daily functioning of the parent (caregiver). Caregiver participation provides the key to transferring the physical therapies' impact from clinic to daily home activities. Families are also then put into the crucial role of becoming equal partners in the setting of goals and understanding of

impairment, which promotes empowerment. The relationship of therapist and families is complex, and has been studied to look at compliance and parent stress. There is a trend toward more compliance for therapy that is performed in the home. Parent satisfaction for home therapy is high. Physical therapy has been shown to prevent contractures and deformities, improve postural alignment, improve independence through assistive seating and mobility devices, maintain motor level and improved functional abilities, increase endurance, and improve the ability of the parents to manage the child's disability. Most studies of the effectiveness of PT and other treatments are inconclusive. Investigators have found that the children most likely to improve in motor abilities were those children with higher IQs and lesser involvement of the neuromuscular system. Harris has provided a review of efficacy studies. The effectiveness of any specific intervention for the child with CP is difficult to study, due to the multihandicapping nature of CP, lack of outcome measures, difficulty in obtaining control groups, and historically poor study designs. Individual therapy programs vary in parameters and incorporate subjective as well as objective elements. Methodological difficulties arise from the problems of measuring treatment-related change on a background of development, as well as the heterogeneity of the population. The development of appropriate evaluative measures, adherence to randomized control studies, and the use of metaanalysis portend improved understanding of the use of therapies on the outcome of the child with CP. In a recent review, neurodevelopmental therapy for children with CP did not confer an advantage over other types of therapy in altering abnormal motor responses, slowing or preventing contractures, or facilitating more normal motor development or functional activities. Systematic reviews of literature on speech therapy and OT reveal positive trends to support the use of services but demonstrate no clear treatment effect. The success of therapy can be better measured by individual goal achievement and specific outcomes such as strength. Spasticity was once thought to be increased with strengthening, but that has not been shown to occur. A systematic review of the effectiveness of strength training for people with CP does demonstrate improved strength and motor activity. An intervention that melds educational theory and PT is conductive education. Conductive education is a mode of therapy that integrates rhythmic movement and activities in a group setting, with a class leader. This method has spread in the USA and now is utilized in some areas as a school-based program. The evidence available supports improvement in functional skills such as toileting for children in conductive education programs versus those in regular school therapy.

Constraint-induced (or forced use) therapy has promise and supports the theory of motor plasticity. This therapy restrains the sound limb to increase the use of the affected side. Gains in the areas of motor performance were maintained up to 6 months post intensive training in one study. Functional electrical stimulation and biofeedback can be helpful for training specific muscles. Another modality that is gaining attention for improving strength in the child with CP is therapeutic electrical stimulation. The use of low-voltage, high-frequency electrical stimulation has been shown to increase blood flow and improve muscle growth and strength. Equipment concerns All durable medical equipment should be considered in the context of the functional prognosis. The early introduction of independent mobility, for children who are not yet able to negotiate at a household level, increases exploration of the environment and might also improve self-esteem. When standardized equipment does not meet the postural support needs of the severely involved child, adaptive seating is essential for the attainment of a sitting position Use of a wheelchair for community mobility becomes a practical measure once a child has outgrown commercially available strollers. A specialized seating system preserves a child's capacity to interact in a conventional posture and also improves pulmonary function tests. While power mobility is usable for children as young as 18 months, many children with CP have problems with spatial relationships and problem-solving skills that preclude motorized chair use. Prior to acquisition of a power chair, a careful evaluation of the family's needs, the child's coping skills, and the environment for which the power mobility is necessary should be undertaken. The child who is comfortable and adequately seated typically has better feeding, digestion, and vocal production. Rehabilitation engineers working with therapists can address complex orthopedic and functional problems. Walkers provide external support for strength and balance, and can improve upright position. The early introduction of technology to improve communication, either written or oral, is warranted. The use of an augmentative communication device does not inhibit development of communication skills. In augmentative communication, speech is enhanced by the use of technology. Low technology and high technology are compensation strategies that continue to challenge the rehabilitation professional abilities to maximize function.

Adaptive equipment specialists provide access to computers, environmental control units, and other activities of daily living (ADL) equipment. In addition to specialized equipment, the child with CP is frequently assisted in mobility and ADL tasks by the use of splints or orthoses. Splinting is a common conservative method of managing a spastic but flexible dynamic deformity. The physical therapist can provide tone-reducing ankle-foot orthoses (TRAFOs) whose tone-reducing aspects are incorporated into their construction. TRAFOs have been useful in some children but are not universally recommended. The decision to brace and the type of orthosis to be used are dictated by the age of the child, functional level, motor control, type of deformity, and commitment to use. Ankle-foot orthoses can decrease energy expenditure when used in conjunction with therapy, surgery, and other treatment. Other joint immobilizers can contribute to function and maintenance of range of motion for hygiene and dressing. Compression garments theoretically increase proprioceptive input to the neuromuscular system and improve motor function. Their intermittent use is appropriate for tasks that are challenging and self-limited. Medical and surgical management: Management for hypotonia Management of hypertonia for children with CP has had increasing medical attention over the decade. Distinguishing between spasticity and other hypertonic movement disorders has been highlighted as a problem in evaluating different interventions. Many professionals are involved with the child, and it is critical that terms that are used frequently to describe the motor disorder are clearly defined. Spasticity implies a velocity-dependent resistance to stretch of a muscle, which is separate from dynamic disorders such as dystonia. Careful analysis and measurement of the hypertonia will lead to more precise management. Oral medications are utilized to decrease global hypertonia. The most commonly used drugs are baclofen (Lioresal), dantrolene (Dantrium), and diazepam. The response to these drugs is generally unpredictable, and side effects can limit long-term use. Studies have demonstrated clinically useful reduction in spasticity following the initiation of medication. The pediatric doses can be quite variable and depend on the response of patient. Specific medications for dystonia can impact the movement in additional ways. Motor control can be improved in the oral motor and fine motor areas with trihexyphenidyl hydrochloride (Artane), and levodopa-carbidopa (Sinemet) and other dopaminergic medications are drugs that warrant trial in a child with dystonia.

Reappraisal of spasticity or hypertonia in a growing child with CP every 6 months is necessary. Contractures develop over time and are a complex interaction of spasticity, growth, motor control deficits, weakness, and chronic positioning. Motor point blocks, and recently botulinum toxin injections, can be used as adjunct management of the spastic muscle. Botulinum toxin's effect in children with CP has been studied and promoted in the past decade and is widely utilized in the management of CP. There is a large body of literature that supports its use in children with hypertonia, both spasticity and dystonia. Botulinum toxin Type A (Botox) is effective in improving range of motion and reducing tone, and also potentially effective in improving motor control. The recent enthusiastic utilization of the botulinum toxins is due to the possible positive effect on the growth of spastic muscles, as well as the potential for improved motor control. The use of serial casting with and without botulinum toxin for children with dynamic contractures has been studied. At present, the best results appear to be with those that combine casting with botulinum toxin for dynamic phased contractures. The current suggested indications for the use of botulinum toxin include: • calf injection for dynamic equinus persistent throughout the gait cycle; • hamstring injection for dynamic knee flexion; • adductor injection for scissoring; • diagnostic measures before surgery; • management of focal limb dystonia; • analgesia for pain and spasm in the perioperative period; and • in the upper limb, persistent thumb in palm, wrist posture preventing effective hand use, and elbow flexion. The method of diluting the injectable, as well as exercise (after injection), can improve the efficacy of the botulinum toxin and needs further study. Phenol is an inexpensive neurolytic agent used for over 30 years. The technical difficulties in administration have limited the use for children. Comparisons of phenol to botulinum toxins have been few. The theoretic advantages of using the combination of phenol are greater control over focal spasticity involving more than three muscle groups, longer duration for the phenol, and lower costs. Phenol does have significant adverse clinical effects when compared with botulinum toxin. Botulinum toxin when compared with phenol for adductor spasticity, improves cadence, step length, and velocity during gait.

Neurosurgical intervention Selective dorsal rhizotomy (SDR) and the intrathecal baclofen (ITB) pump are two current neurosurgical procedures that hold promise in reducing spasticity. Reduction in tone, as recorded by the modified Ashworth scale, is improved with both procedures. SDR has been utilized since the early 1980s to reduce spasticity by interrupting the sensory input into the dorsal horn. Three randomized controlled trials have been published that describe a consistent reduction in Ashworth scores for all three study centers. In two of the centers, there was significant improvement in SDR plus PT compared with PT alone, with a change in the score on the Gross Motor Functional Measure (GMFM) of four percentage points. In the study with higher functioning children, the GMFM scores showed that the difference between SDR plus PT and PT alone was insignificant. A gain in GMFM score was obtained for the SDR group, but the effect might not be clinically relevant. The ideal candidate for SDR is the premature child, with spastic diplegia, good balance, and good selective motor skills, aged 4 or 5 years, with minimal contractures, and able to walk unassisted. A group that can benefit is the more involved children where care and comfort are the only goals. Athetosis is a contraindication for SDR as an intervention. Dystonia can become more problematic post SDR. Intrathecal baclofen is another neurosurgical option for hypertonia. In addition to spasticity reduction, it also has an impact on dystonia. ITB was systematically reviewed by the AACPDM Treatment Outcomes Committee. Level 2 evidence is provided for lower extremity spasticity reduction. The AACPDM evidence-based medicine review reported benefits of ITB for those who are severely involved. For those children in GMFCS 4 and 5, improved positioning, decreased pain, and easier caregiving occurred. The ambulatory child will have decreased energy requirement for walking; however, the balance between weakness and spasticity control has to be carefully managed. Neurosurgical procedures, such as stereotactic ablation of selected thalamic nuclei and chronic electrical stimulation of the cerebellum or posterior columns, have also been unsuccessful in reducing spasticity in the child with CP. Deep brain stimulation is a neurosurgery technique that has shown promise in adults with dystonia. Orthopedic intervention

The natural history of CP is one of progressive effect of spasticity or hypertonia on the growing child and progressive degeneration. Detrimental changes in gait and function occur over time spans as short as 1.5 years. The argument could be made that maintaining function over time is a satisfactory goal. Close observation, aggressive bracing, and vigorous PT can temper the effect of dynamic tone and static contractures. Nearly all children with CP develop an abnormality of physical form and/or function. The degree of the orthopedist's involvement with the child and family depends on the complexity and severity of the musculoskeletal system impairments. Children with CP should have regular orthopedic consultations. The physiatrist can facilitate this process by working in conjunction with the orthopedist and therapists involved with the child and family. Although the orthopedic treatment of the child with CP can show variations among different physicians, the fundamental goals of surgery should reflect a functional approach to problems of alignment. If pain or discomfort is present, especially around the hip, surgical relief may be necessary. Surgery sitting Sitting is a realistic functional goal for every child. The necessary postural alignment for sitting includes a level pelvis and a reasonably straight spine. The loss of motion associated with hip dislocation can alter seating. Excessive pelvic obliquity reduces the sitting surface area and causes excessive pressure on the bony prominences of the pelvis. The management of the hip is complex. Early detection of subluxation is possible with sequential radiographs of the pelvis. Physical examination of the hip alone is not sufficient to detect subluxation. If the hip is subluxed, the surgeon might be able to reduce the overpowering muscle forces by lengthening the iliopsoas and adductor muscles around the hip. This is a brief procedure that offers several benefits. It not only improves femoral head coverage under the acetabulum, but also allows easier dressing, diapering, cleaning, and positioning. The child treated early is more likely to maintain well-seated hips. If the hip progresses to dislocation, then a more extensive procedure may be necessary. The femur and acetabulum may benefit from reconstruction, depending on the child's age and the bone pathology. The femur is commonly affected by excessive anteversion, with a valgus orientation. Acetabular dysplasia contributes to the inability of the femoral head to remain covered. A combination of muscle lengthenings, varus derota-tional osteotomy of the femur, and augmentation procedures of the

acetabulum are complex and arduous. Another problem is that postoperative immobilization can be necessary for as long as 6-8 weeks. Complications can arise following these procedures, including femoral fractures, heterotopic ossification, and peripheral neuropathy. Rehabilitation plays an important role following cast removal. If the femoral articular cartilage is eroded and the hip becomes painful, the options for salvage are limited and fraught with problems. Relief of the pain can be obtained by hip fusion or artificial joint replacement. Sometimes, resection of the femoral head is used as a salvage procedure to afford relief of the pain. Painful hips are a chronic problem and need to be managed with multiple modalities. The pelvis is also influenced by the hamstrings. The hamstrings act as hip extensors, but the major pull of this muscle is to tilt the pelvis in a posterior direction. Sacral sitting with constant sliding out of a wheelchair can be partially corrected by a hamstring release. Distal hamstring lengthening is the more common surgery, but a proximal lengthening can also be considered. Extensor contractures of the hips and knees can also cause sitting problems but are addressed surgically less frequently. Scoliosis or kyphosis can be progressive. Early treatment usually involves using a molded thoracic lumbar orthosis. This is intended to slow rather than stop the progression, and improve trunk support. Total contact support can be incorporated into a contoured seating system. If a curve progresses beyond 40°, fusion is considered to avoid compromise of the respiratory system. If the deformity is rigid and extends over a long segment, staged procedures are performed. The risk of anesthesia, infection, blood loss, neurologic compromise, and pseudoarthrosis should be considered and planned for before surgery. Surgery for standing Supported standing and transfers are possible when the ankle can be held in the neutral position and the knee has less than 20° of flexion contracture. The surgical procedures used to improve alignment for these skills are hamstring lengthenings and Achilles tendon lengthenings. Hip flexion contractures can also hinder standing, and need attention if greater than 20°. Surgery for ambulation Orthopedic surgery to improve ambulation should be well timed and planned to maximize the child's strength and motivation. Collaboration with a pediatric orthopedist is essential. Even though orthopedic surgery has been the mainstay of treatment for the child with biomechanical alignment and

lever arm disease, the literature supporting orthopedic surgery has been of a Sackett's level 4 evidence with few randomized and controlled studies. The analysis of orthopedic surgery has expanded to include energy cost-benefits, standardized outcome testing with the GMFM, and cost analysis. Difficulty in predicting outcome for the ambulatory has led to caution in recommending orthopedic surgery. The use of gait analysis has refined the observation of components of the gait cycle and the combined effect of contractures on gait dynamics. Gait analysis defines cadence, velocity, stride length, and ranges of motion of the hip, knee, and ankle during various phases of the gait cycle and the timing of muscular activity. Some gait laboratories also measure the forces generated at each joint. Gait analysis provides consistent objective information that enhances orthopedic decision making and improves outcome studies. The use of gait analysis not only improves decision making prior to surgeries, but also allows a more objective analysis of the outcome following surgery. Energy expenditure for some children with spastic CP can be as high as 350% of normal. Energy expenditure is an important consideration for continued community ambulation. Gait analysis is an appropriate outcome and energy cost for children in GMFCS levels 1 and 2. The goals for children with GMFCS level 3 and 4 are to improve alignment and motor function. For children not expected to change in motor function, gains can still be realized with orthopedic surgery for children who are GMFCS level 5. Response to treatment can be reflected in a care and comfort-measuring instrument or with a quality of life measure. The tendency to scissor at the hips can hinder forward progression of gait. Adductor myotomies in combination with hamstring lengthenings can create a better base of support and a more upright posture. The dynamic hip flexion can inhibit balance and decrease the child's ability to walk. Intramuscular psoas lengthening assists with hip extension and stance. Rectus femoris transfers and lengthenings are used to decrease the problem of stiff knee gait following or at the time of hamstring release. A braceable foot or a foot that allows for foot flat or heel strike is desirable for stance. Gastrocnemius recession surgery and tendo-Achilles lengthening can improve the foot position but should be delayed until 6 years of age. Orthopedic surgery can affect balance, but the central processing of balance reactions remains the same after surgery. Assistive devices can play an important transitional role in household or community ambulation. A reverse walker typically assists with maintaining upright posture better than the traditional forward walker. Upper extremity surgery is uncommonly done to improve the function of the hand. Flexor carpi ulnaris transfers to a wrist extensor are done to reduce the wrist flexion deformity. Rerouting of the brachioradialis can

improve supination and improve upper limb reach. Other surgeries include biceps release for flexion contractures and anterior elbow release. The active use of the hand, however, is dependent on stereo-gnosis and two-point discrimination. The emphasis on the arm and hand is important, because the adult shifts concerns from mobility to ADL and communication. Motion analysis for the arm and hand is a new technology utilized to determine joint kinematics and kinetics of upper extremity function. Spasticity reduction and orthopedic surgery are two distinct and complementary elements in the treatment of gait in the child with CP. Neither one should be viewed as an exclusive intervention option. Children with major structural and biomechanical alignment can benefit from both. It is common, however, to provide the spasticity management first. Rehabilitation is individualized for each child post orthopedic surgery or neurosurgery. The recognition and the evaluation of the other impairments related to CP are central aspects of treatment. In particular, strengthening and improved endurance is important after procedures that have the child immobilized for a period of time. If tone-reducing procedures are done first, then the alignment issues will continue to impact functional gains. Functional goal-focused therapy is the cornerstone of rehabilitation management. In some instances, the goal may be to improve the distribution of weight and change the technique for transfers or even seating, which is a functional benefit. Transition to adulthood and aging with Cerebral Palsy Adolescence is a difficult time for many children. It is a time in which one changes physically and socially, and begins to work toward a more independent future. Adolescents need gradually increasing responsibility and a sense of who they are in the world. Prevocational skills are learned incrementally through helping others, chores, part-time and summer jobs, and volunteer work. Increasing independence with decreasing support in all areas of life, including medical decision making, should be encouraged. Resilience is associated with support (not over protectiveness) of parents and friends, having chores in childhood, and having a mix of disabled and non-disabled friends. Physical activity and fitness levels, employment, and marital rates remain low in this population. Routine primary preventive care is often neglected in adults with CP as well. Organized sports activities were once only rarely recommended for the child with CP. That has changed. There is no evidence that strengthening adversely affects muscle tone. Neither is there evidence to preclude even vigorous activities, such as downhill skiing, that once were thought to

increase orthopedic deformity. The therapeutic benefits of swimming and horseback riding for children with motor disabilities have been established. While sports programs can improve endurance and strength, they also can promote interpersonal growth and improve self-esteem—something that all children experience when appropriately supervised in a team sport with peers. The established organizations for athletes with CP are the United States Cerebral Palsy Athletic Association and the Cerebral Palsy International Sports and Recreation Association. Fitness programs that emphasize flexibility and endurance have been developed for both recreational and therapeutic use. The child with CP ages with a chronic but not unchanging neuromuscular condition. Adults with CP have similar life expectancy to that.-of normal peers, although it becomes poorer in those who lose the ability to ambulate. Ongoing medical issues are exacerbated by aging, such as dysphagia, gastroesophageal reflux, urinary incontinence, constipation, and musculoskeletal pain, which progress and cause a decline in function. Problems of osteoporosis, fractures due to falls, worsening balance, loss of strength and flexibility, and pressure sores are frequently seen. Physical fatigue is increased versus in the normal population. Independence with ADL also typically decreases with age. Spasticity can change as the skeletal system matures. Contractures, scoliosis, and windswept hip deformity can make seating and positioning difficult. The neck, particularly for the adult with athetoid CP, is vulnerable to excessive forces that can narrow the disk spaces or compress the spinal cord. Osteoarthritic changes occur earlier in joints having ongoing abnormal joint mechanics and stresses from spastic or dyskinetic muscle forces. The prevalence of musculoskeletal pain in adults with CP is high, over 80% in some studies. Loss of mobility with aging in those who walk with equipment occurs commonly, but it can be ameliorated or delayed using general strengthening and fitness techniques. Lower extremity strength and endurance training programs have been shown to improve gait parameters in periods as short as 6 weeks. Pain problems can be sorted through and often successfully treated. Positioning equipment, antiinflammatory medication, bursal injections, mobilization and modalities by PT, and spasticity treatment can improve activity tolerance and decrease pain. Hip replacement is now being favored over resection procedures for severe hip dysfunction. Adults with CP rank communication, ADL, and mobility as higher priority goals than ambulation. Vocational rates among adults with CP in the past were only 30-50% but could be increased. The Americans with Disabilities Act requires reasonable access to employment, housing, and

accessibility to the community. Quality of life research demonstrates that participation in community activities of all kinds contributes to improved satisfaction with life. Prognosis