CEREBRAL PALSY

DEFINITION

Cerebral palsy (CP) is the name given to a collection of movement disorders. Movement disorders are conditions in which a person's muscles do not respond normally. CP is also known as static encephalopathy (pronounced in-seh-fuh-LAH-puh-thee). It is caused by brain damage that occurs before, during, or just after birth. A person with CP is often also affected by other conditions caused by brain damage.

DESCRIPTION

If a person is affected by CP his or her muscles may become either rigid or very loose. Sometimes an individual may lose control of his or her muscles, resulting in problems with balance and coordination. The condition may affect the legs only, which is called paraplegia (pronounced par-uh-PLEE-jee-uh) or diplegia (pronounced die-PLEE-juh); the arm and leg on one side of the body, which is known as hemiplegia (pronounced hem-i-PLEE-juh); or all four limbs, called quadriplegia (pronounced kwod-ruh-PLEE-jee-uh).

Other problems experienced by someone with CP include visual or hearing problems, mental retardation (see mental retardation entry), learning disabilities (see learning disorders entry), and attention deficit disorder (ADD). Some CP patients experience no problems beyond their movement disorder.

CP affects about 500,000 children and adults in the United States. About 6,000 new cases are diagnosed in newborns and young children each year. CP is not a genetic disorder, and there is currently no way of predicting which children will develop it. CP is not a disease and is not communicable, which means it cannot be passed from one person to another.

CP is a nonprogressive disorder. That is, it does not become better or worse over time. However, some conditions may appear to become worse. For example, when muscles are rigid for a long period, arms and legs may become deformed. In such cases, additional treatments may be necessary.

CAUSES

Cerebral palsy is caused by damage to brain cells that control the movement of muscles. When those cells die, signals can no longer be sent to muscle cells. This loss of muscle control results in the symptoms of CP.

A number of factors can cause brain cell damage. These include lack of oxygen (asphyxia, pronounced az-FIK-see-uh), infection, trauma (shock), malnutrition (poor diet), drugs or other chemicals, or hemorrhage (bursting of blood vessels). In most cases, it is impossible to discover the exact cause for any one person's CP. Premature birth is regarded as one important and common factor. Most researchers now believe that brain cell damage occurs before birth. This damage is also responsible for other conditions that tend to occur along with CP.

Asphyxia may occur as the result of various problems. First, the circulatory system may not develop normally prior to birth. Asphyxia may occur during the birth process, however asphyxia at birth usually indicates that the newborn has other neurological problems. Asphyxia after birth can be caused by choking, exposure to poisons (such as carbon monoxide), or near drowning.

A child's brain may also be damaged prior to birth by infections acquired by the mother. These infections include rubella (German measles; see rubella entry), toxoplasmosis (a blood infection), cytomegalovirus (a virus that causes herpes; pronounced SIE-tuh-MEG-uh-lo-VIE-rus), and HIV (the virus that causes AIDS; see AIDS entry). Two kinds of brain infection, encephalitis (see encephalitis entry) and meningitis (see meningitis entry), can also cause CP in infants.

Physical trauma (shock) to the pregnant mother or fetus may cause brain damage leading to CP. Trauma can be caused by a car accident, violent shaking, or physical abuse. Malnutrition and drug use by the mother can also cause brain damage.

Erika Moore, who suffers from cerebral palsy, sits next to a playground at the Children's Care Hospital and School in Sioux Falls, South Dakota. The hospital plans to add equipment for children with special needs. (Reproduced by permission of

AP/Wide World Photos

)

Differences in certain blood factors (Rh factors) between mother and child was once a major cause of one form of CP. Today these differences can be detected by tests and a pregnant woman can be treated to prevent damage to her child.

SYMPTOMS

The symptoms of cerebral palsy are usually not noticeable at birth. Instead, they begin to show up during the first eighteen months of a child's life.

Normal children pass through a series of developmental stages. At each stage, they learn how to perform one or more common tasks. Infants with CP have difficulty performing these tasks. Such difficulties may indicate that an infant has CP. The tasks considered to be milestones in normal development include the following:

• Babbles (6 to 8 months)
• Sits well with support (8 to 10 months)
• Crawls (9 to 12 months)
• Walks alone (12 to 15 months)
• Uses one or two words other than dada and mama (12 to 15 months)
• Eats with fingers; holds bottle (12 to 18 months)
• Turns pages in books; removes shoes and socks (24 to 30 months)
• Walks up and down steps (24 to 36 months)

Because children develop at different rates, using these milestones to diagnose cerebral palsy (or any other disorder) must be used with great caution. The fact that an otherwise healthy child does not reach one of these milestones at the suggested age is not necessarily a sign that the child has CP. He or she may just be developing at a slower rate. In addition, problems with vision or hearing may cause such delays.

Five forms of cerebral palsy are recognized. They are defined according to the kind of muscle damage and the location of that damage:

• Spastic: Muscles are rigid (tight), posture may be abnormal, and the ability to do delicate work is impaired.
• Athetoid: Muscular movements are slow, twisting, and involuntary (beyond the person's control).
• Hypotonic: Muscles are floppy, without firmness.
• Ataxic: Balance and coordination are impaired.
• Dystonic: Any combination of the above symptoms.

Cerebral palsy is also described according to the parts of the body affected:

• Hemiplegia: One arm and one leg on the same side of the body are involved.
• Diplegia: Both legs are involved; in addition, one or both arms may also be involved.
• Quadriplegia: Both arms and both legs are involved.

A diagnosis of CP usually involves combining terms. For example, someone with spastic diplegia shows symptoms of rigid muscles in both legs. In addition, the terms mild, moderate, and severe are sometimes used to describe the seriousness of the disorder.

Loss of muscle control as a result of CP can cause other problems with bones and muscles. Examples of these problems include scoliosis (curving of the spine; see scoliosis entry), hip dislocation, and contractures. A contracture is a permanent shortening of a muscle. It can cause muscles to become fixed in awkward positions, resulting in clenched fists or equinus (pronounced eh-KWI-nuss). Equinus is a foot deformity that prevents one's heel from touching the ground when walking. Contractures can be painful and can interfere with the normal activities of daily living.

Brain damage that causes cerebral palsy may also cause a variety of other disorders. These include:

• Mental retardation
• Learning disabilities.
• Attention deficit disorder
• Seizure disorder (tendency toward convulsions)
• Visual problems, especially strabismus ("cross-eye")
• Loss of hearing (see hearing loss entry)
• Speech problems

About one-third of children with CP have some moderate-to-severe mental retardation, one-third have mild mental retardation, and one-third have normal to above average intelligence. For some children, disorders related to CP can have an even greater impact on a child's life than the physical handicaps caused by CP.

DIAGNOSIS

Cerebral palsy is usually diagnosed using the described developmental milestones. Using these milestones, most children with CP can be diagnosed by the age of eighteen months. However, differences in the rate of development and the presence of other medical problems must always be taken into account.

Certain tests can also be used to diagnose CP. The Apgar test is used with newborn babies. It measures the newborn's heart rate, cry, color, muscle tone, and motor (muscular) reactions. An Apgar score of less than three (out of a possible ten) is a warning sign that the baby may have cerebral palsy. Babies with abnormal organs or abnormal behaviors may also have CP. A variety of tests, such as an ultrasound test, can be used to diagnose these problems.

As in most medical cases, tests can also be performed to rule out potential problems other than cerebral palsy. X rays can be used to look for abnormal structures in the brain, for example, and blood tests can be used to check for infection.

TREATMENT

Cerebral palsy cannot be cured. However, the physical and other problems it causes can usually be managed through planning and timely care. Treatment plans depend on the type of impairment as well as associated problems the child may have, such as learning disabilities. Many CP patients require the help of physical and occupational therapists only. These professionals help the child learn to deal with loss of muscle control. Other specialists, such as speech-language therapists, special education teachers, nutritionists, and neurosurgeons (nerve specialists) may be needed to help with problems related to CP.

While cerebral palsy does not become worse over time, the needs of a CP child do change and new treatment plans may have to be developed as the child grows older. Most parents do not have the knowledge and skills to provide a CP child with all the care he or she needs. Medical professionals then become essential. Other parents who have CP children are also an important resource. They can provide both practical advice and emotional support for parents of newly-diagnosed CP children. These support groups exist throughout the United States. They can be contacted with the help of the United Cerebral Palsy Association or a local hospital or social service agency.

Posture and Mobility

Cerebral palsy affects both posture and one's ability to move about. Physical therapists work with a child to develop good posture, to move affected arms and legs, and to develop normal body movements. Special equipment, such as wheelchairs, crutches, braces, and walkers, may be needed to achieve this goal.

SPASTICITY. Spasticity is a condition in which muscles become tight and stiff. It can cause muscles to shorten, joints to tighten, and posture to change. It can also affect the ability to walk, use a wheelchair, or sit unaided. Spasticity can prevent a person from being able to feed, dress, or care for himself or herself.

Treatments for spasticity depend on its severity. Mild spasticity is treated with regular stretching exercises. Moderate spasticity may require braces or casts to keep a limb in its normal position. More serious cases of spasticity may require more aggressive treatments. For example, spasticity can be treated with muscle-relaxing drugs, such as diazepam (pronounced di-AZE-uh-pam, trade name Valium) or dantrolene (pronounced DAN-tro-leen, trade name Dantrium). Surgery can also be used. Tendons in the affected muscle are cut. The limb is then placed into a cast until the tendons grow back in a normal position.

SCOLIOSIS. Scoliosis, or curvature of the spine, can develop when back muscles become weak or spastic. When that happens, the vertebrae (bones that make up the spinal column) may be pulled out of alignment. Scoliosis causes pain, changes in posture, and possible damage to internal organs. Scoliosis is usually treated with a brace that holds the back in a normal position. Surgery can also be used to join the vertebrae in a normal position.

Ataxia and Coordination

Ataxia (pronounced uh-TAK-see-uh) is a loss of balance control. It impairs a person's ability to move normally. Physical therapy can help a child with CP regain the sense of balance.

Seizures

Seizures (see epilepsy entry) occur in 30 to 50 percent of children with cerebral palsy. The seizures may occur only in one arm or leg, or throughout the body. They can be treated with various drugs, such as carbamazepine (pronounced KAHR-buh-MAZ-uh-peen, trade name Tegretol) or ethosuximide (pronounced ETH-o-SUK-sih-mide, trade name Zarontin). Some children need antiseizure drugs for a limited time only, while others must continue to use them throughout their lives. Careful control of one's diet can also reduce the risk of having seizures.

Strabismus

Strabismus (cross-eye, pronounced struh-BIZ-muss) is treated with eye patches and corrective lenses. If these treatments do not work, injections of botulinum toxin or surgery on eye muscles may help relieve the disorder.

Nutrition

People with cerebral palsy may have trouble eating because they cannot control the tongue and mouth muscles. They may also have difficulty holding eating utensils. As a result, they may not get enough of the foods needed for normal, healthy development. In such cases, the symptoms of CP may actually become worse.

Twenty-month-old Ashley Neisis who suffers from cerebral palsy, with her service dog, BJ. The dog has warned Ashley's parents when she was having trouble breathing. (Reproduced by permission of

AP/Wide World Photos

)

Nutritionists can help children with CP learn what foods they should eat. Nutritional supplements, such as vitamins and minerals, may also be needed. Speech-language therapists can teach people with CP more effective ways to use their throat and mouth muscles, reducing the risk of aspiration. Aspiration is the inhaling of food and saliva into the airways, causing choking and suffocation. In severe cases, a tube can be inserted through the abdomen and into the stomach to carry food directly into the digestive system.

Other Common Medical Problems

Drooling, dental caries (cavities), and gum disease are more common in people with CP than in the general population. These problems can be prevented to some degree by drugs or with the help of a physical therapist. Constipation is another common problem. It can be relieved with changes in the diet or with enemas or suppositories when needed. Enemas and suppositories help loosen the bowels and make bowel movements easier.

Communication

Poor coordination of tongue and mouth muscles can cause speech problems in people with CP. Problems with speaking can retard a child's mental development. Picture boards can help with speech problems. They allow the CP child to point to objects rather than naming them. A number of mechanical devices are available for school-age children with communication problems. These include typing programs and computer-assisted speech devices. Speech-language therapists can offer valuable advice on the types of equipment available.

Education

Children with mild symptoms of cerebral palsy can often be placed in a regular school classroom. This practice is known as inclusion or mainstreaming. Inclusion has the advantage of making CP children feel less different from other children.

Children with more severe forms of CP may be placed in separate classrooms with teachers trained to work with special education problems. On the federal level, such schools operate under and are financed by the Individuals with Disabilities Education Act (IDEA). Most states have legislation similar to IDEA. Educational specialists can help parents understand the options and opportunities provided for their CP children by these acts.

Behavioral and Mental Health Services

Children with cerebral palsy sometimes develop behavioral or emotional problems. These problems may require special treatments, such as behavioral modification (structured programs for changing behavior) and/or individual and family counseling.

Alternative Treatment

Some alternative treatments that have been effective with some CP individuals include massage therapy, vitamin supplements, herbal medicine, and acupuncture (a Chinese therapy technique where fine needles puncture the body).

PROGNOSIS

Cerebral palsy is a nonprogressive disorder that does not become worse or better over time. However, it does pose different kinds of problems at different stages of life. Treatments that are devised for children with CP usually have to be changed and adjusted as the person grows older. Some form of professional help is usually needed at every stage of the disorder.

Cerebral palsy itself is generally not the cause of death. However, it can shorten a person's life span for other, related reasons. For example, lung infections that can lead to pneumonia (see pneumonia entry) and other diseases are more common among people with CP. Poor nutrition can also contribute to the likelihood of infection. Overall, more than 90 percent of infants with CP survive to adulthood, and the vast majority with mild symptoms live near-normal lifespans.

PREVENTION

The specific cause of cerebral palsy is usually not known, so prevention is difficult or impossible. Difficulties with the birth process itself are no longer thought to be a major risk factor for CP. Instead, problems with normal development before birth seem to be the most common cause of CP. Until these problems are better understood, prevention will be difficult.

Ironically, better health care may actually contribute to an increase in the number of cerebral palsy cases. Doctors have learned to keep premature babies alive more effectively, but these babies are at high risk for the disorder.

The best hope for reducing the risk of cerebral palsy appears to be for pregnant women to follow good health practices. These practices include a healthy diet, avoiding alcohol and tobacco, and prompt treatment for infections.

FOR MORE INFORMATION

Books

Geralis, Elaine, ed. Children With Cerebral Palsy: A Parents' Guide, 2nd edition. Bethesda, MD: Woodbine House, 1998.

Kramer, Laura. Uncommon Voyage: Parenting a Special Needs Child in the World of Alternative Medicine. Winchester, MA: Faber & Faber, 1996.

Leonard, Jane Faulkner, Margaret E. Myers, Sherri Lynn Cadenhead. Keys to Parenting a Child With Cerebral Palsy. Hauppauge, NY: Barron's Educational Series, 1997.

Miller, Freeman, and Steven J. Bachrach. Cerebral Palsy: A Complete Guide for Caregiving. Baltimore: Johns Hopkins University Press, 1995.

Periodicals

Exceptional Parent Magazine. 555 Kinderkamack Road, Oradell, NJ 07649–1517. 800–EPARENT; 201–634–6550.

Organizations

National Information Center for Children and Youth with Disabilities. PO Box 1492, Washington, DC 20013–1492. (800) 695–0285.

United Cerebral Palsy Association. 1660 L Street NW, Suite 700, Washington, DC 20036–5602. (800) 872–5827; (202) 776–0406; (202) 973–7197 (TTY). (202) 776–0414 (fax). ucpnatl@ucpa.org. http://www.ucpa.org.