Cerebral Palsy

Cerebral palsy (CP) is an umbrella term for a group of disorders affecting body movement, balance, and posture. Loosely translated, cerebral palsy means "brain paralysis." Cerebral palsy is caused by abnormal development or damage in one or more parts of the brain that control muscle tone and motor activity (movement). The resulting impairments first appear early in life, usually in infancy or early childhood. Infants with cerebral palsy are usually slow to reach developmental milestones such as rolling over, sitting, crawling, and walking.

Common to all individuals with cerebral palsy is difficulty controlling and coordinating muscles. This makes even very simple movements difficult.

• Cerebral palsy may involve muscle stiffness (spasticity), poor muscle tone, uncontrolled movements, and problems with posture, balance, coordination, walking, speech, swallowing, and many other functions.
• Mental retardation, seizures, breathing problems, learning disabilities, bladder and bowel control problems, skeletal deformities, eating difficulties, dental problems, digestive problems, and hearing and vision problems are often linked to cerebral palsy.
• The severity of these problems varies widely, from very mild and subtle to very profound.
• The magnitude of the problems may wax and wane over time. However, the cause of the condition, the brain abnormality responsible for the cerebral palsy, does not get worse over time. Nevertheless, the clinical picture may show signs of deterioration as the individual ages.

Types of cerebral palsy are as follows:

• Spastic (pyramidal): Increased muscle tone is the defining characteristic of this type. The muscles are stiff (spastic), and movements are jerky or awkward. This type is classified by which part of the body is affected: diplegia (both legs), hemiplegia (one side of the body), or quadriplegia (the entire body). This is the most common type of CP.
• Dyskinetic (extrapyramidal): This includes types that affect coordination of movements. There are two subtypes.
  • Athetoid: The person has uncontrolled movements that are slow and writhing. The movements can affect any part of the body, including the face, mouth, and tongue. About 10% to 20% of cerebral palsy cases are of this type.
  • Ataxic: This type affects balance and coordination. Depth perception is usually affected. If the person can walk, the gait is probably unsteady. He or she has difficulty with movements that are quick or require a great deal of control, such as writing.
• Mixed: This is a mixture of different types of cerebral palsy. A common combination is spastic and athetoid.

Many individuals with cerebral palsy have normal or above average intelligence. Their ability to express their intelligence may be limited by difficulties in communicating. All children with cerebral palsy, regardless of intelligence level, are able to improve their abilities substantially with appropriate interventions. Most children with cerebral palsy require significant medical and physical care, including physical, occupational, and speech/swallowing therapy.

Despite advances in medical care, cerebral palsy remains a significant health problem. The number of people affected by cerebral palsy has increased over time. This may be because more and more premature infants are surviving. Worldwide, about 1.5 to more than 4 children per 1,000 have cerebral palsy. Cerebral palsy affects both sexes and all ethnic and socioeconomic groups.

Cerebral palsy results from damage to certain parts of the developing brain.

• This damage can occur early in pregnancy when the brain is just starting to form, during the birth process as the child passes through the birth canal, or after birth in the first few years of life.
• In many cases, the exact cause of the brain damage is never known.

At one time, problems during birth, usually inadequate oxygen, were blamed for cerebral palsy.

• We now know that fewer than 10% of cases of cerebral palsy begin during birth (perinatal).
• In fact, current thinking is that cases of cerebral palsy begin before birth (prenatal).
• Some cases begin after birth (postnatal).
• In all likelihood, many cases of cerebral palsy are a result of a combination of prenatal, perinatal, and postnatal factors.

Risk factors linked with cerebral palsy include the following:

• Infection, seizure disorder, thyroid disorder, and/or other medical problems in the mother
• Birth defects, especially those affecting the brain, spinal cord, head, face, lungs, or metabolism
• Rh factor incompatibility, a difference in the blood between mother and fetus that can cause brain damage in the fetus (Fortunately, this is almost always detected and treated in women who receive proper prenatal medical care.)
• Certain hereditary and genetic conditions
• Complications during labor and delivery
• Premature birth
• Low birth weight (especially if less than 2 pounds at birth)
• Severe jaundice after birth
• Multiple births (twins, triplets)
• Lack of oxygen (hypoxia) reaching the brain before, during, or after birth
• Brain damage early in life, due to infection (such as meningitis), head injury, lack of oxygen, or bleeding

The signs of cerebral palsy are usually not noticeable in early infancy but become more obvious as the child's nervous system matures. Early signs include the following:

• Delayed milestones such as controlling head, rolling over, reaching with one hand, sitting without support, crawling, or walking
• Persistence of "infantile" or "primitive reflexes," which normally disappear 3 to 6 months after birth
• Developing handedness before age 18 months: This indicates weakness or abnormal muscle tone on one side, which may be an early sign of CP.

Problems and disabilities related to CP range from very mild to very severe. Their severity is related to the severity of the brain damage. They may be very subtle, noticeable only to medical professionals, or may be obvious to the parents and other caregivers.

• Abnormal muscle tone: Muscles may be very stiff (spastic) or unusually relaxed and "floppy." Limbs may be held in unusual or awkward positions. For example, spastic leg muscles may cause legs to cross in a scissor-like position.
• Abnormal movements: Movements may be unusually jerky or abrupt, or slow and writhing. They may appear uncontrolled or without purpose.
• Skeletal deformities: People who have cerebral palsy on only one side may have shortened limbs on the affected side. If not corrected by surgery or a device, this can lead to tilting of the pelvic bones and scoliosis (curvature of the spine).
• Joint contractures: People with spastic cerebral palsy may develop severe stiffening of the joints because of unequal pressures on the joints exerted by muscles of differing tone or strength.
• Mental retardation: Some, although not all, children with cerebral palsy are affected by mental retardation. Generally, the more severe the retardation, the more severe the disability overall.
• Seizures: About one-third of people with cerebral palsy have seizures. Seizures may appear early in life or years after the brain damage that causes cerebral palsy. The physical signs of a seizure may be partly masked by the abnormal movements of a person with cerebral palsy.
• Speech problems: Speech is partly controlled by movements of muscles of the tongue, mouth, and throat. Some individuals with cerebral palsy are unable to control these muscles and thus cannot speak normally.
• Swallowing problems: Swallowing is a very complex function that requires precise interaction of many groups of muscles. People with cerebral palsy who are unable to control these muscles will have problems sucking, eating, drinking, and controlling their saliva. They may drool. An even greater risk is aspiration, the inhalation into the lungs of food or fluids from the mouth or nose. This can cause infection or even suffocation.
• Hearing loss: Partial hearing loss is not unusual in people with cerebral palsy. The child may not respond to sounds or may have delayed speech.
• Vision problems: Three-quarters of people with cerebral palsy have strabismus, which is the turning in or out of one eye. This is due to weakness of the muscles that control eye movement. These people are often nearsighted. If not corrected, strabismus can lead to more severe vision problems over time.
• Dental problems: People with cerebral palsy tend to have more cavities than usual. This results from both defects in tooth enamel and difficulties brushing the teeth.
• Bowel and/or bladder control problems: These are caused by lack of muscle control.

If a child has problems that suggest cerebral palsy, he or she will undergo a very thorough evaluation. There is no medical test that confirms the diagnosis of cerebral palsy. The diagnosis is made on the basis of various types of information gathered by the child's health care professional and, in some cases, other consultants.

• This information includes a detailed medical interview concerning medical histories of both the mother's and father's families, the mother's medical problems before and during pregnancy, and a detailed account of the pregnancy, labor, delivery, and neonatal (newborn) period.
• Parents will be asked to relate in detail the child's medical problems and mental and physical development.
• Parents may be asked other questions as well. It is very important to answer all questions as completely and honestly as possible, as the answers may help the child.

Imaging studies: These studies provide a picture of structures inside the body. Such testing, when used on the brain or spinal cord, is often called neuroimaging. These tests are not always necessary, but in many cases, they may help identify the cause or extent of the cerebral palsy. They should be done as early as possible so that appropriate treatment, if indicated, can be begun immediately. Many individuals with mild cerebral palsy have no visible brain abnormalities.

• Ultrasound of the brain: Ultrasound uses harmless sound waves to detect certain types of structural and anatomic abnormalities. For instance, it can show hemorrhage (bleeding) in the brain or damage caused by lack of oxygen to the brain. Ultrasound is often used on newborns who cannot tolerate more rigorous tests such as CT scans or MRI.
• CT scan of the brain: This scan is similar to an X-ray but shows greater detail and gives a more three-dimensional image. It identifies malformations, hemorrhage, and certain other abnormalities in infants more clearly than ultrasound.
• MRI of the brain: This is the preferred test, since it defines brain structures and abnormalities more clearly than any other method. Children who are unable to remain still for at least 45 minutes may require a sedative to undergo this test.
• MRI of the spinal cord: This may be necessary in children with spasticity of the legs and worsening of bowel and bladder function, which suggest an abnormality of the spinal cord. Such abnormalities may or may not be related to cerebral palsy.

Other tests: Under certain circumstances, a child's health care professional may want to do other tests.

• Electroencephalography (EEG) is important in the diagnosis of seizure disorders. A high index of suspicion is needed in order to detect nonconvulsive or minimally convulsive seizures. This is a potentially treatable cause of a CP-look-alike, which is easier to treat when treated early.
• Electromyography (EMG) and nerve conduction studies (NCS) may be helpful in distinguishing CP from other muscle or nerve disorders.

If a child was born prematurely, had a low birth weight, or was subject to certain complications of pregnancy, labor, or delivery, he or she will be monitored carefully over time for signs of CP. Any of the following warrant a visit to the child's health care professional:

• The child has a seizure.
• The child's movements seem unusually jerky, abrupt, uncoordinated, or slow and writhing.
• The child's muscles seem unusually tense or, on the other hand, limp and "floppy."
• The child does not blink in response to loud noises by age 1 month.
• The child does not turn his or her head toward a sound by age 4 months.
• The child does not reach out for a toy by age 4 months.
• The child does not sit up unsupported by age 7 months.
• The child does not say words by age 12 months.
• The child develops left- or right-handedness before age 12 months.
• The child has strabismus (one eye turned inward or outward).
• The child does not walk or walks with a stiff or abnormal gait, such as toe-walking.

These are only some of the most obvious examples of problems that may signal CP. A parent should speak to the child's health care professional about any problems that suggest a lack of control of muscles or movements.

There is no cure for cerebral palsy. With early and ongoing treatment, however, the disabilities associated with cerebral palsy can be reduced. Many different therapies are available, most under the supervision of a medical specialist or other allied professional. Not all of these therapies are right for every person with cerebral palsy. The therapy regimen for a specific individual with cerebral palsy should be tailored to meet the needs of that individual. A treatment may work for one child but not for another. The parents and the child's care team work together to choose only those treatments that offer some benefit to the child.

The goal of drug therapy is to reduce the effects of cerebral palsy and prevent complications. Medications are prescribed to reduce spasticity and abnormal movements and to prevent seizures.

Medications used to relieve spasticity and abnormal movements include the following:

• Dopaminergic drugs: Widely used in Parkinson's disease, these drugs increase the level of a brain chemical called dopamine. The effect is to decrease rigidity and abnormal movements. Examples include levodopa/carbidopa (Sinemet) and trihexyphenidyl (Artane).
• Muscle relaxants: These agents reduce spasticity by relaxing the muscle directly. Examples include baclofen (Lioresal). This drug can be taken as a pill or be administered automatically via an implantable pump.
• Benzodiazepines: These agents act on brain chemistry to relax muscles. The most widely used of these agents is diazepam (Valium).
• Botulinum toxin type A: This substance is widely known as BOTOX®. When injected, it causes a mild muscle paralysis and reduces contractions. In cerebral palsy, it is used to decrease spasticity of muscles of the arms or legs, which improves range of motion and overall mobility. This can be important in allowing a child to fit into an orthotic (brace or splint) or even to be comfortably positioned in a wheelchair. The effects of BOTOX® injections typically last 3 to 6 months. BOTOX® helps other treatments work better, such as physical therapy or casting the limb. In some cases, using BOTOX® can delay surgery or make surgery unnecessary. Some people have allergic-type reactions to BOTOX® and must limit the number of injections or stop them altogether.

Medications used to relieve seizures include the following:

• Anticonvulsants: These agents stop seizure activity as rapidly as possible and prevent seizure recurrence. There are many difference agents available; they vary in their mechanism of action.
• Benzodiazepines: Agents such as diazepam often are used to stop seizures when they are frequent or prolonged.
• Ketogenic diet: this is a special diet rich in fats that result in the production of excess of ketones which, acting in the brain, can reduce the number of seizures.

While specific therapies help a child develop specific skills and abilities, the overall goal of treatment is to help the individual with cerebral palsy reach his or her greatest potential physically, mentally, and socially. This is accomplished with a variety of different approaches managed by a team of professionals. Care for people with cerebral palsy is complicated, requiring a number of different services and specialists. In some areas, care is available through a single multidisciplinary clinic that oversees all aspects of the child's therapy.

Rehabilitation: A comprehensive rehabilitation program may include physical therapy, use of special equipment, and spasticity treatment. This program is often overseen by a specialist in rehabilitation medicine (sometimes called a physiatrist).

• Physical therapy involves stretching, physical exercises, and other activities that develop muscle strength, flexibility, and control. The goal is to maximize function and minimize disabling contractures. The focus is on developing specific skills such as holding the head up, sitting unsupported, or walking. Braces, splints, and casts may be used to help reach these goals.
• Special equipment that may be helpful to people with CP includes walkers, positioning devices, customized wheelchairs, scooters, and tricycles.
• Spasticity may be treated by injections into the muscles or by medications. Reduction of spasticity can improve range of motion, reduce deformity, improve response to occupational and physical therapy, and delay the need for surgery.

Occupational therapy: The occupational therapist helps the individual learn physical skills he or she needs to function and become as independent as possible in everyday life. Examples are feeding, grooming, and dressing.

Speech/language therapy: This therapy helps the child overcome communication problems. Many children with cerebral palsy have problems speaking because of poor tone or uncontrolled movements in the muscles of the mouth and tongue. Speech therapy helps develop those muscles, improving speech. Speech therapy also benefits children with hearing loss. Children who cannot speak may be able to benefit from communication technologies such as a computerized voice synthesizer.

Vision problems: An ophthalmologist is consulted for children who have strabismus and visual problems.

Medical therapy: This encompasses treatment for all medical problems whether related to CP or not. Various specialists may be called upon to deal with specific problems.

• Seizures: Seizure disorders are common in people with cerebral palsy. These are not always well controlled with medication. A specialist in conditions of the nervous system (neurologist) may be consulted for help in selecting an appropriate regimen.
• Feeding and digestive problems: Individuals with cerebral palsy often have gastroesophageal reflux or GERD (severe heartburn and related symptoms caused by regurgitation of acid from the stomach) as well as swallowing and feeding problems. A team consisting of a doctor who specializes in digestive diseases (gastroenterologist), a nutritionist, and a feeding and swallowing therapist can assess nutritional status and treat problems. Swallowing therapy helps the child eat and drink independently and helps prevent aspiration. The child's diet must be customized to accommodate limitations in swallowing. Children with severe swallowing problems require feeding through a tube.
• Breathing problems: People with cerebral palsy may have breathing problems because the muscles that control expansion and contraction of the lungs are disabled. A specialist in lung disorders (pulmonologist) should be consulted for management of the resulting lung disease.

Educational services: Many children with cerebral palsy, even those of average or above-average intelligence, are challenged in cognitive processes such as thinking, learning, and memory. They can benefit from the services of a specialist in learning disabilities.

• Such specialists can identify the child's specific learning disabilities, direct early interventions and preparation for school, and monitor his or her progress.
• In the United States, these services are provided for children younger than 3 years by an established early intervention system. Representatives of the appropriate agencies will work with parents to develop an Individualized Family Services Plan, or IFSP. This plan describes the child's needs and the services the child will receive to address those needs.
• Educational services for school-aged children are provided by the public school system. The staff at the child's school will work with the parents to develop an Individualized Education Plan (IEP) for the child. This may include services besides classroom teaching.

Navigating all of these different services can be difficult for parents. The child's health care professional can refer parents to a medical social worker who can help them find and enroll in the services their child needs.

Operations used in the treatment of cerebral palsy include the following.

• Dorsal rhizotomy: In this procedure, a few specific nerves are cut at their roots where they branch off the spinal cord. In certain individuals with cerebral palsy, this works very well to reduce spasticity and improve the ability to sit, stand, and walk.
• Implantation of a baclofen pump: The muscle relaxant drug baclofen can be very helpful in reducing spasticity in some people with cerebral palsy, but it works best when it is given continuously. A tiny pump can be placed in the abdominal wall to deliver a continuous dose to the spastic muscles of the limbs.
• Stereotactic surgery to a part of the brain that controls muscle tone and movement may improve rigidity, athetosis, and tremor.
• Reconstructive surgery to an arm can restore muscle balance, release contractures, and stabilize joints. This can improve placement of the hand in space and the important ability to grasp, release, and pinch.
• Skeletal problems such as hip dislocation and scoliosis can be corrected with surgery.
• Severe spasticity can be corrected with a number of surgical procedures, including tenotomy, a tendon-lengthening procedure.
• Surgical operations for intractable epileptic seizures: These procedures are now well-accepted. There is enough experience in the Epilepsy Centers that surgery, in selected cases, may be an excellent option.
• Vagal nerve stimulation (VNS) incorporates an implantable device that stimulates the vagal nerve in the neck. This method has been used with some success in epileptic disorders that are refractory to antiepileptic medications.

Many children with cerebral palsy develop emotional problems, behavior problems, or both. They can benefit from sessions with a psychologist or counselor.

Personal computers and related technologies offer wonderful opportunities for communication, social interaction, education, entertainment, and employment for people with cerebral palsy.

The overall goal for ongoing care of individuals with CP is to help them reach their full physical, mental, and emotional potential. Generally, this includes living as much as possible in the mainstream of their society and culture. People with CP tend to be happiest and most productive when they can go to school with, live with, and work with their peers.

Children with CP require regular sessions with their physical, occupational, and speech/language therapists, as well as frequent checkups with their medical and surgical teams. The exact schedule of visits is determined by the severity of the child's condition and his or her response to treatment. A multidisciplinary CP clinic allows for frequent and complete care with the minimum of inconvenience.

Often the cause of cerebral palsy is not known, and nothing can be done to prevent it. However, some important causes of cerebral palsy can be prevented in many cases, including premature birth, low birth weight, infections, and head injuries.

• Seek appropriate prenatal care as early as possible in the pregnancy. Many women schedule a prepregnancy visit so they can be properly prepared for a healthy pregnancy. Appropriate care is available from physicians, physician assistants, nurse practitioners, and certified nurse-midwives.
• Avoid using cigarettes, alcohol, and illicit drugs during pregnancy; these increase the risk of premature delivery.
• Rubella (measles) during pregnancy or early in life is a cause of cerebral palsy. Testing for rubella immunity before a woman becomes pregnant allows her to be immunized, which protects both the woman and the baby from contracting this potentially devastating illness.
• Appropriate prenatal care includes testing for Rh factor. Rh incompatibility is easily treated but can cause brain damage and other problems if untreated.
• Routine vaccinations of babies can prevent serious infections such as meningitis that can lead to cerebral palsy.
• Make sure that the child is restrained in a properly installed car seat and wears a helmet when riding on a bicycle.

With proper therapy, many people with cerebral palsy can lead near-normal lives. Even those with very severe disabilities can improve their condition significantly, although they will never be able to live independently.

Approximately 25% of children with cerebral palsy have mild involvement with few or no limitations in walking, self-care, and other activities. Approximately half are moderately impaired to the extent that complete independence is unlikely but function is satisfactory. Only 25% are so severely disabled that they require extensive care and are unable to walk.

Of the 75% of children with cerebral palsy who are eventually able to walk, many rely on assistive equipment. The ability to sit unsupported may be a good predictor of whether a child will walk. Many children who can sit unsupported by age 2 years eventually will be able to walk, while those who cannot sit unsupported by age 4 years probably will not walk. These children will use wheelchairs to move around.

The likelihood of medical complications of cerebral palsy is related to the severity of the condition. Generally, the more severe the CP, the more likely are related conditions such as seizures and mental retardation. Individuals with quadriplegia are much more likely than those with diplegia or hemiplegia to have these related conditions.

• Seizure disorders occur in about one third of people with cerebral palsy.
• Mental retardation occurs in about 30% to 50% of people with cerebral palsy. Standardized tests that evaluate primarily verbal skills may underestimate a child's intelligence level.
• Obesity is a common problem in children who are confined to a wheelchair or are unable to move freely.

Life expectancy in people with cerebral palsy also is related to the severity of their condition. People with milder forms of cerebral palsy have the same life expectancy as the general population. Those with severe forms of cerebral palsy typically have a shorter life span, especially if they have many medical complications.

With the advances in medical care and the awareness that children with cerebral palsy merit aggressive treatment of any acute condition that is treatable, the average life expectancy has increased. As a result, it is not unusual to see adults, even those with severe forms of cerebral palsy, reaching 50 and even the 60 years of age.

Adults with cerebral palsy are likely dependent on others for activities of daily living and in need of continuous support. The parents may be no longer alive or with limitations in the ability of providing support. Unfortunately, at the present time there are very few medical services that are oriented to treat adults with cerebral palsy. Historically, cerebral palsy has been a condition of children, however, at the present time there is a sizable and growing number of adults with cerebral palsy. In addition, there are few internists or general practitioners with expertise in CP. Just recently a few academic adult hospitals offer formal training positions in the area of developmental disabilities. These adults with cerebral palsy need strong advocacy to guarantee the services they need.

Some studies have found that abnormalities of muscle tone or movement in the first several weeks or months after birth may gradually improve over the first years of life. In one study, almost 50% of very young infants thought to have cerebral palsy and 66% of those thought to have spastic diplegia "outgrew" these signs of cerebral palsy by age 7 years. Many children do not manifest full motor signs that are suggestive of cerebral palsy until aged 1 to 2 years. Thus, some propose that the diagnosis of cerebral palsy should be deferred until the child is aged 2 years.

End of Life Issues

Counseling and advice is very important when individuals with cerebral palsy present with serious medical conditions. Unfortunately, some people will make decisions believing that persons with cerebral palsy have "a poor quality of life" to start with. This approach is very dangerous since it leads to the limitation of medical services offered to the person. In some instances, it is appropriate to limit some medical options. For example, a person with severe scoliosis with distortions in the shape of the thorax may not be a good candidate for cardiorespiratory resuscitation because it could result in rib fractures and, in the end, it will be ineffective. Hospice care may be appropriate for a person with a terminal disease that has no treatment. However, certain medical conditions including pneumonia, sepsis, and cardiac insufficiency, can be treated. In these instances, the decision to treat or not to treat should not include as a factor the fact that the individual has cerebral palsy or cognitive deficiencies.

Clearly, children with cerebral palsy may have very substantial problems, but almost all have the potential to learn, achieve, succeed, and create a happy life for themselves. This cannot happen without effort, and they need the help of their families. Having a child with cerebral palsy brings many challenges. It is understandable, then, that parents and siblings of a child with cerebral palsy may have significant stress. A parent may feel guilt, anger, anxiety, and/or hopelessness. The parent may feel alone and uncertain about what he or she should do.

Before parents can help themselves or their child, they need to develop appropriate expectations and get organized. Only then can parents learn practical ways to cope with the child's problems and put these methods into practice. But making changes is not always easy. Sometimes it helps to have someone to talk to.

This is the purpose of support groups. Support groups consist of people in similar situations. They come together to help each other and to help themselves. Support groups provide reassurance, motivation, and inspiration. They help parents see that their situation is not unique and not hopeless, and that gives them power. Support groups also provide practical tips on coping with cerebral palsy and navigating the medical, educational, and social systems that parents will rely on for help for themselves and their child. Being in a cerebral palsy support group is recommended by most mental health professionals.

Support groups meet in person, on the telephone, or on the Internet. To find a support group that works, contact the following organizations. Parents can also ask a member of their child's care team or go on the Internet. If parents do not have access to the Internet, they should go to the public library.

• United Cerebral Palsy - (800) 872-5827 or (202) 776-0406
• Pathways Awareness Foundation - (800) 955-2445