Musculoskeletal

Hip assessment should include evaluation for contractures involving flexion, internal and external rotation, and adduction and leg length discrepancies. To evaluate for hip flexion contractures, the Thomas test is performed by bringing both legs up to the chest. One leg is then extended until resistance is felt, and the angle of the femur to the table is recorded. Normally, the angle should be zero. In the Ely test, the patient lies prone and the knee is quickly flexed. The test is positive if the buttocks rises off the table, and indicates a tight quadriceps. Testing for adductor contracture and hip internal and external rotation is performed by evaluating range of motion with the hip in flexion and extension. Excessive internal rotation may be caused by femoral anteversion. Leg length should be measured, generally from the anterior–superior iliac spine to the medial malleolus. However, leg length should never be measured over a contracted joint. If necessary, the measurement should be broken down into segments and added together to ensure accurate results.

The knee should be tested for hamstring contracture by measuring the popliteal angle. The contralateral leg should be stabilized on the table and then the ipsilateral hip flexed to 90°. The lower leg is then extended until resistance is felt and the angle between the tibia and the line of full extension is recorded. Normally this angle should be close to zero.

The foot and ankle should be tested for contractures and tibial torsion. To test for gastrocnemius/soleus contracture, ankle dorsiflexion range should be checked with both the knee flexed and extended. If the range is greater in flexion, the gastrocnemius is the most likely site of contracture; otherwise, the soleus is the most likely site. The posterior tibialis and peroneal muscles should be evaluated. A spastic posterior tibialis may cause varus of the heel along with plantar flexion spasticity. A spastic peroneus may cause a valgus deformity. To test for tibial torsion with the patient in prone position, the thigh–foot angle is measured with the knee flexed to 90°.

The back should be examined for posture, spinal curves, or asymmetries. The upper extremities should be assessed for resting posture and spontaneous movement. Active and passive range of motion should be measured and positioning of the thumb, wrist, and fingers evaluated.

Neurologic

Focus should be placed on tone. The head must be kept in neutral because the tonic neck reflexes may influence tone. If movement has a velocity-dependent component, it is spastic. In addition to the deep tendon reflexes, which are often increased in spastic CP, and the Babinski’s or plantar response, which is often upgoing, the following reflexes can be useful to assess a child whose presentation is concerning for CP: Moro reflex, asymmetric tonic neck reflex, neck righting reflex, vertical suspension, grasping reflex, tonic labyrinthine reflex, protective reflexes, and symmetric tonic neck reflex .

Motor delay

CP is, by definition, a disorder of movement, usually resulting in a delay of gross motor skills. This delay is caused by abnormal tone in some muscle groups, weakness in other muscle groups, and the lack of coordination between muscle groups to allow for directed movement. Treating the above abnormalities can most easily treat motor delay.

Children who have hemiplegia exhibit definite hand preference before 1 year of age, compared with most children who display handedness at about 2 years of age. Hemiplegic children often display an asymmetric crawl, or they might not crawl at all. Almost all children who have spastic hemiplegia do ambulate. Most become independent with activities of daily living, using some aids.

Children who have spastic diplegia are late in attaining all gross motor skills, but the delay in standing and walking are most notable. Generally, only mild functional impairment of the upper extremity occurs. In a prospective study, being able to sit independently at 2 years of age was a good predictive sign of future ambulation. Children who could not sit by 4 years of age did not ambulate . See Table 2 for other predictive signs .

Children who have spastic quadriplegia present a much broader spectrum of disability. The severity of motor involvement affects the delay in developmental milestones. Approximately one fourth of children who have spastic quadriplegia have only mild involvement, with minimal functional limitation in mobility or self care . Approximately one half are moderately impaired. These patients are unable to achieve complete independence, but are able to function reasonably well. About one quarter of patients are severely involved, require total care, and are unable to ambulate . Independent sitting at 2 years and suppression of obligatory reflexes are good prognostic signs for eventual ambulation .

Children who have dyskinetic CP typically experience severe neuromuscular dysfunction. The abnormal movements are first noticed in the hands and fingers, but abnormal movements are usually noted in all extremities by 18 months of age. The writhing, involuntary movements give way to dystonia. As a result, all of their gross motor milestones are significantly delayed. Only approximately one half of these children are able to walk, and this normally occurs after 3 years of age . Those children who are able to walk usually develop the coordination necessary to perform their activities of daily living. Those who do not walk typically require total care.

A fairly broad body of literature now suggests that early intervention programs work to optimize development . The goals of early intervention are to improve function and encourage independence. The programs improve family interaction, provide family support, and provide education in how to promote development. The Individuals with Disabilities Education Act mandates early intervention for all children aged 0 to 3 years who show developmental delay. These programs are typically multidisciplinary and may occur either in centers or the family’s home. The therapists either directly provide hands-on therapy to the child or they educate the family on how to provide the interventions themselves.

Various methods of therapy have been used in CP. The neurodevelopmental treatment approach, developed by Bobath , is commonly used in patients who have CP. The goal is to alter abnormal postures, reduce or increase tone, improve balance in antigravity postures, and develop fundamental movement patterns following the normal developmental sequence. The Brunnstrom method advocates facilitation of muscle groups within synergy, with progression toward development of voluntary control . Constraint-induced therapy, developed by Taub and colleagues , has been adapted for use in children who have hemiplegic CP. This therapy focuses on the theory of learned nonuse and involves restraining the less-involved arm for periods each day while the more involved arm performs various functional tasks along with shaping activities. This program has been found be beneficial in a randomized clinical trial of 18 children who had hemiparesis comparing constraint-induced therapy with conventional therapy . Strengthening programs using progressive resistive exercise have been advocated for children who have CP. Damiano and colleagues showed increased force production and improved gait parameters after strengthening.

Increasing numbers of individuals who have CP are competing in sporting activities, either through programs such as the Special Olympics or in programs with normally developing individuals, allowing them to develop healthy lifestyles that include higher levels of physical activity. This participation must be encouraged in individuals who have CP, who have generally been found to have decreased physical activity levels and decreased levels of fat-free mass compared with normally developing individuals. Studies also indicate a lower level of cardiorespiratory fitness in individuals who have CP compared with the typically developing population . Training protocols have been shown to be beneficial .

Families of children who have cerebral palsy are seek a wide variety of therapies, with more than 50% trying some form of complementary and alternative medicine. Massage, hippotherapy, and aquatherapy are among the most popular . Some forms of therapy, such as hippotherapy, are based on physiologic theories of how the horse affects the child’s nervous system but may simply be more interesting and interactive ways of doing therapy. Various suits, intensive therapy programs, and other methods are widely acclaimed by families, with perhaps their greatest benefit in increasing parental involvement in the child’s therapy program . Hyperbaric oxygen treatment has also been popular, but well-done randomized controlled trials have failed to show benefit , and this therapy is associated with possible complications .

Spasticity

Managing spasticity is a large part of treating children who have CP. The treatment of spasticity normally begins with the application of therapeutic modalities . Daily range of motion has been shown to decrease muscle tone for several hours after it is performed, and it may delay or prevent contractures . The use of heat or cold can modify tone in the short term, but either modality is impractical for long-term use . Casting and splinting are often used as adjuncts to regular range of motion. Casting for 2 to 3 weeks can decrease tone and improve range of motion for several months . Cyclical use of electrical stimulation has been found to decrease upper-extremity contractures and improve agonist/antagonist balance, but these affects only last for hours .

Several medications have been found to affect spasticity ( Table 3 ). All of these medications may decrease spasticity but have not been shown to improve coordination. Trihexyphenidyl has been found to improve upper-extremity function, expressive language, and drooling in children who have dystonic CP. Younger children are more likely to respond to this medication. Doses range from 0.04 to 0.3 mg/kg/d .

In people who have more focal spasticity or whose regional problem overlies general spasticity, local injections may be appropriate. A careful physical examination must be performed to ensure that the proper muscles are selected for therapy. Botulinum toxin blocks neuromuscular transmission by inhibiting the release of acetylcholine, essentially causing reversible denervation atrophy. The onset of action is typically within 24 to 72 hours, with peak effect experienced at approximately 2 weeks. The duration of action is 3 to 6 months . Side effects include fever, excessive weakness, pain, and bruising.

Phenol is the other substance that is most frequently injected as a local agent. Phenol denatures proteins and causes tissue necrosis. Its onset of action is within 1 hour and its duration of action is 2 to 36 months. Side effects include pain during the injection, chronic dysesthesias, and chronic pain. Other side effects include edema, deep venous thrombosis, excessive motor weakness, and sensory loss. Phenol is much less expensive than botulinum toxin, but the injection process is more complicated and painful. Children who undergo phenol injections, and many who undergo botulinum toxin injections, often require sedation or general anesthesia. Phenol and botulinum toxin are sometimes given to patients in the same injection session to maximize effect without increasing risk through increased dose. This technique has not led to increased adverse effects .

For patients whose spasticity is refractory to more conservative measures, or those whose spasticity has progressed to contracture, referral to orthopedic surgery may be necessary. Surgery alters the musculotendinous unit in a way that decreases tension. Selecting the appropriate procedures to perform is critical, because surgery permanently alters the function of the muscle groups involved . An attempt should be made to wait until the major growth spurt has finished. The patient should undergo a thorough evaluation, often involving a physical therapist, before any course of action is decided.

Some neurosurgical procedures can be useful in reducing tone for people who have CP. Selective dorsal rhizotomy has been shown to improve range of motion and gait in young children who have spastic diplegic CP, but many patients still have difficulty with motor control . An intensive therapy program is recommended to maximize the functional gains after surgery. A meta-analysis of three controlled trials of rhizotomy plus therapy versus therapy only suggested that the surgery has beneficial effects on gross motor function . Risks associated with surgery include hypotonia, weakness, sensory changes, and bladder dysfunction. Although some postoperative weakness may occur, generally good recovery is experienced with increased strength noted . Patient selection (ages 3–6 or 3–8 years, good trunk control, good underlying strength) is important for best outcomes.

A recent advancement in the treatment of spasticity has been the development of an implantable pump that infuses baclofen directly into the thecal sac surrounding the spinal cord. Intrathecal baclofen (ITB) binds to receptors in the spinal cord causing inhibition of spinal reflexes. It is sometimes used in patients who have spastic CP . Because the medication is infused at the target site of action, a smaller amount of it is needed than in oral dosing . ITB decreases lower-extremity spasticity much more than upper-extremity spasticity, although upper-extremity function has been found to improve in patients treated with ITB . The decreased spasticity has been shown to improve the gait in ambulatory patients treated with ITB . Patients have also noted a pattern of weight gain, which has been beneficial for underweight individuals . ITB has also been found to improve the dystonia in patients who have dyskinetic CP. Quality of life, ease of care, and speech were also improved in dyskinetic patients . The most common side effects are drowsiness, dizziness, nausea, hypotension, and headache, but are much less frequent than with oral dosing. Unfortunately, complications can occur and may be life-threatening . Therefore, any physician caring for a patient receiving ITB should be aware of the signs, symptoms, and treatment of these complications.

Overdose can occur with incorrect pump filling or programming and may result in respiratory depression and reversible coma. If overdose occurs, emergent measures are required to maintain airway, breathing, and circulation. The pump will need to be urgently stopped with a specialized device. If this is not available, the reservoir can be emptied using a 22-guage needle. The pump is easily palpable in the abdominal wall. The central port of the pump can be found by palpating the edge of the pump and measuring in toward the center, as described in the protocols for each pump. Intravenous physostigmine or withdrawal of 30 to 40 mL of cerebral spinal fluid can be tried in severe overdoses.

Withdrawal may occur if tube dysfunction, pump failure, or incorrect pump programming stops ITB dosing. Withdrawal may cause increased spasticity, severe hyperthermia, and rhabdomyolysis followed by multisystem failure and death . Early symptoms of withdrawal include itching, dysphoria, irritability, a dramatic increase in spasticity, tachycardia, fever, and changes in blood pressure. A patient who develops these symptoms should be advised to immediately begin oral baclofen and go to the emergency department. Intravenous benzodiazepines may be helpful for symptomatic relief, but the best and safest form of management is early reinitiation of ITB, if necessary through lumbar puncture boluses.

Seizures

Approximately one half of people who have CP have a seizure disorder . Seizures are most common in children who have hemiplegia; 70% of the children who acquired hemiplegia postnatally and 50% of those who have congenital hemiplegia have seizure disorders . Focal motor seizures are common in this patient population. Of people who have spastic quadriplegia 50% experience seizures, most commonly the grand mal type . Seizures are less common in people who have diplegic or athetoid forms of CP . When seizures are uncontrolled, they may lead to further delay in cognitive development in patients who have CP. Compared with children who have seizures but are otherwise neurologically normal, children who have CP are more likely to develop seizures within the first year of life, have a history of neonatal seizures, have a history of status epilepticus, and require treatment with second-line agents. They are less likely to remain seizure-free after discontinuing antiepileptic mediations . Because the management of seizures in these children can be complicated, children who have CP who have a seizure disorder should be referred to a neurologist for management.

Cognitive delay

Mental retardation is commonly found in CP, although it is by no means universal. Approximately 30% to 50% of the patients who have CP have mental retardation . An even higher percentage of patients have milder cognitive deficits and learning disabilities. Of those who have mental retardation, approximately 50% fall into the severe-to-profound mentally retarded group . The patients who have the most severe retardation are those who have severe spastic quadriplegic CP, although many who have severe spastic quadriplegia have normal or near-normal intelligence. Approximately 30% of the patients who have mental retardation fall into the mild range, and approximately 30% of those who have diplegic CP have cognitive impairment . Some cognitive impairment is noted in 28% of the patients who have hemiplegic CP . People who have athetosis as their main motor deficit tend to have normal intelligence. Early intervention followed by appropriate schooling may improve the cognitive outcome of children who have CP.

Speech and language

Patients who have slow development of language often also have cognitive delay. However, oromotor deficits may also cause patients to have difficulty with the coordination of articulation, phonation, and respiration. Dysarthria is almost universal in patients who have dyskinetic forms of CP . Dysarthria may be so severe as to preclude functional speech. Referral to speech language pathology for augmentative communication devices should be considered for patients who have severe dysarthria who are cognitively able to communicate. Also, difficulty with hearing may lead to delayed development of speech in children who have CP. A thorough evaluation by a speech language pathologist will help to determine whether language delay is secondary to oromotor difficulties or cognitive impairment.

Sensory changes

Many people who have CP have problems with vision. Visual deficits are present in 63% of people who have diplegic CP. Strabismus is present in 50% of children who have diplegic CP , and an even higher incidence of visual impairment is seen in children who have spastic quadriplegic CP. Approximately 25% of people who have spastic hemiplegic CP have visual deficits, and the most common deficit is homonymous hemianopsia. Convergent strabismus is also common in this patient population . In patients who have dyskinetic CP, accommodation may be impaired . In many patients, the cause of visual impairment is not obvious and is believed to be cerebral in origin . Visual–perceptual impairments are also common in children who have all types of CP . Vision should be tested at least as frequently in children who have CP as in other children, and early referral to an appropriate specialist should be arranged if any concerns develop.

Hearing loss is a common finding in children who have CP. Conductive hearing loss is often caused by anatomic abnormalities or frequent otitis media. Sensorineural impairment also occurs. It may be caused by congenital nervous system infection. Treatment with extracorporeal membrane oxygenation, prolonged artificial respiration after birth, and persistent pulmonary hypertension are other risk factors . Hearing should be tested in children who have CP and referral to an audiologist should be considered for those who are found to have sensorineural hearing loss.

Tactile sensation, particularly proprioception, may be impaired in children who have diplegic and quadriplegic forms of CP . Among people who have spastic hemiplegia, 68% have sensory deficits on the same side as their motor deficits. Stereognosis, graphesthesia, two-point discrimination, and proprioception are often affected .

Gait

Almost all people who have CP have some abnormality of their gait. Spasticity management is an important component in any program designed to improve gait in cerebral palsy. An ankle-foot orthosis (AFO) is commonly prescribed to help stabilize gait. AFOs can be used to control spastic equinus, promote alignment of the hindfoot, and control foot and knee position. Walkers, lofstrand crutches, or canes are also often prescribed to help improve stability. Children who are unable to ambulate because of energy requirement or instability should be prescribed a wheelchair. Even the most severely involved children are often able to propel a powered wheelchair with supervision, giving them some mobility independence. Increased mobility and independent exploration of one’s environment has been shown to improve self-esteem .

Changing neurologic status

CP is, by definition, a nonprogressive lesion. If a patient who has CP exhibits a neurologic decline, the cause of that decline must be investigated. Cervical myelopathy develops more frequently and at a younger age in people who have dyskinetic CP . Neurologic tumors, simple disk herniations, peripheral neuropathy, mononeuropathies, myopathy, dementia, and systemic illnesses are other potential causes for decline.

School

The physiatrist who treats children who have CP will often serve as an advocate for them when they and their families are dealing with the public school system. All children, regardless of disability, are entitled to a free and appropriate education, including special education and related services provided at public expense. Related services may include transportation, speech pathology, audiology, psychological services, physical and occupational therapy, recreational services, appropriate medical services for diagnosis and evaluation, school health services, extended school year and school day services, social work, and parent counseling and training. For children who have significant developmental disabilities, education must be provided from birth until 21 years of age or older, depending on state and local laws. Education must be in the least restrictive environment possible.

The individualized education plan (IEP) is supposed to ensure timely compliance with these rules. To initiate the IEP process, parents must request a special education assessment in writing. A written letter by the physician may be critical to the case because it provides medical documentation to evaluate and support the request. It also provides information to help determine who will be involved in the IEP assessment. When the educational plan is established, it may be helpful for parents to review the plan with their physician to ensure that it meets all of the child’s needs. If the plan is not acceptable, there is an appeal process. Recommendations made by a physician that include appropriate clinical findings and relevant literature are more likely to be accepted.

Self-esteem

Some research suggests that children who have CP have more difficulty with self-esteem than their peers. In one longitudinal study of self-esteem, adolescent girls who had CP scored significantly lower than age-matched controls on physical, social, and personal self-esteem, but adults showed no significant difference. Adult male subjects who had CP had self-esteem scores similar to their nondisabled peers. The increase in self-esteem was believed to be caused by a greater breadth of relationships and experiences as adults than as adolescents. This same study found no relationship between self-image and severity of disability. Having friends who were disabled and friends who were not disabled improved self-image . Other studies have reported no difference in self-esteem between children with CP and their nondisabled peers .

Sexuality issues

The sexuality of people who have CP has not been adequately researched or discussed. One study showed no significant difference in sexual desire between a population of women who had physical disabilities and a nondisabled group, although significant differences were seen in level of sexual activity, sexual response, and satisfaction. This study also found no difference between degree of disability and level of sexual activity in women who had CP . In another study of disabled young adults, most having CP, half had never dated and a little more than half had no sexual experience. Of those who had dated, they generally began dating at a later age than a control group .

No published reports could be found specifically examining the sexuality of men who have CP. Men who have CP are able to father children, although fertility rates have not been adequately studied. Difficulties with motor control and sensation may make sexual activity more difficult for men who have CP.

Females who have CP develop regular menstrual cycles. In females who are more severely involved, maintaining adequate hygiene during the menstrual period may be difficult because of contractures and poor muscle control. Frequently these women will need assistance in placing pads or tampons. Occasionally medications such as Depo-Provera may be used to prevent monthly menstruation. Women who have CP may also experience an increase in spasticity and incontinence during their menstrual cycles .

Women who have CP are able to get pregnant, although their rates of fertility have not been adequately studied. Typically their pregnancies are uncomplicated and they give birth to healthy children, although vaginal delivery may be more difficult because of poor muscle control and contracture. One study showed a slightly higher incidence of elective termination and cesarean section among women who have CP .

Women who have CP should begin to undergo pelvic examinations when they become sexually active. The position for these examinations may have to be adapted, depending on the presence of contracture and poor muscle control. Barriers to quality reproductive health care include inaccessible equipment and facilities, insensitivity and lack of knowledge about disabilities on the part of health care providers, and lack of information tailored to the patients’ special needs. Patients report that providers often seemed surprised that they would be sexually active and would not ask about contraceptive use or discuss risks for sexually transmitted diseases. Some patients report that accessing health care was so difficult that they avoided regular gynecologic visits .

Abuse is a serious concern for people who have CP. One study showed no difference in the prevalence of emotional, physical, or sexual abuse between women who had disabilities and those who did not. However, women who had disabilities were more likely to experience abuse by attendants or health care providers, primarily because they had increased exposure to institutional or medical settings. Factors that make women who have disabilities susceptible to abuse include inability to leave an abusive situation because of mobility impairments or dependency on a caregiver and perceived vulnerability because of physical, mental, and emotional impairments. These women stay in abusive situations longer than their nondisabled counterparts because interventions that are available, such as shelters, may not be accessible. They may also have no opportunity to report the abuse or may be afraid that they will be placed in a more restrictive living environment if they do .