Cerebral palsy is an umbrella-like term used to describe a group of chronic disorders impairing control of movement that appear in the first few years of life and generally do not worsen over time. The disorders are caused by faulty development of or damage to motor areas in the brain that disrupts the brain's ability to control movement and posture. Symptoms of cerebral palsy include difficulty with fine motor tasks (such as writing or using scissors), difficulty maintaining balance or walking, involuntary movements. The symptoms differ from person to person and may change over time. Some people with cerebral palsy are also affected by other medical disorders, including seizures or mental impairment, but cerebral palsy does not always cause profound handicap. Early signs of cerebral palsy usually appear before 3 years of age. Infants with cerebral palsy are frequently slow to reach developmental milestones such as learning to roll over, sit, crawl, smile, or walk. Cerebral palsy may be congenital or acquired after birth. Several of the causes of cerebral palsy that have been identified through research are preventable or treatable: head injury, jaundice, Rh incompatibility, and rubella (German measles). Doctors diagnose cerebral palsy by testing motor skills and reflexes, looking into medical history, and employing a variety of specialized tests. Although its symptoms may change over time, cerebral palsy by definition is not progressive, so if a patient shows increased impairment, the problem may be something other than cerebral palsy.

Is there any treatment?

There is no standard therapy that works for all patients. Drugs can be used to control seizures and muscle spasms, special braces can compensate for muscle imbalance. Surgery, mechanical aids to help overcome impairments, counseling for emotional and psychological needs, and physical, occupational, speech, and behavioral therapy may be employed.

What is the prognosis?

At this time, cerebral palsy cannot be cured, but due to medical research, many patients can enjoy near-normal lives if their neurological problems are properly managed.

What research is being done?

Research suggests that cerebral palsy results from incorrect cell development early in pregnancy. For example, a group of researchers has recently observed that more than one-third of children with cerebral palsy also have missing enamel on certain teeth. Scientists are also examining other events—such as bleeding in the brain, seizures, and breathing and circulation problems—that threaten the brain of a newborn baby. Some investigators are conducting studies to learn whether certain drugs can help prevent neonatal stroke, and other investigators are examining the causes of low birth-weight. Other scientists are exploring how brain insults (like brain damage from a shortage of oxygen or blood flow, bleeding in the brain, and seizures) can cause the abnormal release of brain chemicals and trigger brain disease.

How Many People Have This Disorder?

The United Cerebral Palsy Associations estimate that more than 500,000 Americans have cerebral palsy. Despite advances in preventing and treating certain causes of cerebral palsy, the number of children and adults it affects has remained essentially unchanged or perhaps risen slightly over the past 30 years. This is partly because more critically premature and frail infants are surviving through improved intensive care. Unfortunately, many of these infants have developmental problems of the nervous system or suffer neurological damage. Research is under way to improve care for these infants, as in ongoing studies of technology to alleviate troubled breathing and trials of drugs to prevent bleeding in the brain before or soon after birth.

What Are the Different Forms?

Spastic diplegia, the disorder first described by Dr. Little in the 1860s, is only one of several disorders called cerebral palsy. Today doctors classify cerebral palsy into four broad categories -- spastic, athetoid, ataxic, and mixed forms -- according to the type of movement disturbance.

Spastic cerebral palsy. In this form of cerebral palsy, which affects 70 to 80 percent of patients, the muscles are stiffly and permanently contracted. Doctors will often describe which type of spastic cerebral palsy a patient has based on which limbs are affected. The names given to these types combine a Latin description of affected limbs with the term plegia or paresis, meaning paralyzed or weak. The four commonly diagnosed types of spastic cerebral palsy are illustrated in the figure.

When both legs are affected by spasticity, they may turn in and cross at the knees. As these individuals walk, their legs move awkwardly and stiffly and nearly touch at the knees. This causes a characteristic walking rhythm, known as the scissors gait.

Individuals with spastic hemiparesis may also experience hemiparetic tremors, in which uncontrollable shaking affects the limbs on one side of the body. If these tremors are severe, they can seriously impair movement.

Athetoid, or dyskinetic, cerebral palsy. This form of cerebral palsy is characterized by uncontrolled, slow, writhing movements. These abnormal movements usually affect the hands, feet, arms, or legs and, in some cases, the muscles of the face and tongue, causing grimacing or drooling. The movements often increase during periods of emotional stress and disappear during sleep. Patients may also have problems coordinating the muscle movements needed for speech, a condition known as dysarthria. Athetoid cerebral palsy affects about 10 to 20 percent of patients.

Ataxic cerebral palsy. This rare form affects the sense of balance and depth perception. Affected persons often have poor coordination; walk unsteadily with a wide-based gait, placing their feet unusually far apart; and experience difficulty when attempting quick or precise movements, such as writing or buttoning a shirt. They may also have intention tremor. In this form of tremor, beginning a voluntary movement, such as reaching for a book, causes a trembling that affects the body part being used and that worsens as the individual gets nearer to the desired object. The ataxic form affects an estimated 5 to 10 percent of cerebral palsy patients.

Mixed forms. It is common for patients to have symptoms of more than one of the previous three forms. The most common mixed form includes spasticity and athetoid movements but other combinations are also possible.

What Causes Cerebral Palsy?

Cerebral palsy is not one disease with a single cause, like chicken pox or measles. It is a group of disorders with similar problems in control of movement, but probably with different causes. When physicians try to uncover the cause of cerebral palsy in an individual child, they look at the form of cerebral palsy, the mother's and child's medical history, and onset of the disorder.

In the United States, about 10 to 20 percent of children who have cerebral palsy acquire the disorder after birth. (The figures are higher in underdeveloped countries.) Acquired cerebral palsy results from brain damage in the first few months or years of life and can follow brain infections, such as bacterial meningitis or viral encephalitis, or results from head injury -- most often from a motor vehicle accident, a fall, or child abuse.

Congenital cerebral palsy, on the other hand, is present at birth, although it may not be detected for months. In most cases, the cause of congenital cerebral palsy is unknown. Thanks to research, however, scientists have pinpointed some specific events during pregnancy or around the time of birth that can damage motor centers in the developing brain. Some of these causes of congenital cerebral palsy include:

• Infections during pregnancy. German measles, or rubella, is caused by a virus that can infect pregnant women and, therefore, the fetus in the uterus, to cause damage to the developing nervous system. Other infections that can cause brain injury in the developing fetus include cytomegalovirus and toxoplasmosis. There is relatively recent evidence that placental and perhaps other maternal infection can be associated with cerebral palsy.
• Jaundice in the infant. Bile pigments, compounds that are normally found in small amounts in the bloodstream, are produced when blood cells are destroyed. When many blood cells are destroyed in a short time, as in the condition called Rh incompatibility (see below), the yellow-colored pigments can build up and cause jaundice. Severe, untreated jaundice can damage brain cells.
• Rh incompatibility. In this blood condition, the mother's body produces immune cells called antibodies that destroy the fetus's blood cells, leading to a form of jaundice in the newborn.
• Severe oxygen shortage in the brain or trauma to the head during labor and delivery. The newborn infant's blood is specially equipped to compensate for low levels of oxygen, and asphyxia (lack of oxygen caused by interruption in breathing or poor oxygen supply) is common in babies during the stresses of labor and delivery. But if asphyxia severely lowers the supply of oxygen to the infant's brain for lengthy periods, the child may develop brain damage called hypoxic-ischemic encephalopathy. A significant proportion of babies with this type of brain damage die, and others may develop cerebral palsy, which is then often accompanied by mental impairment and seizures.

• Stroke. Coagulation disorders in mothers or infants can produce stroke in the fetus or newborn baby. Bleeding in the brain has several causes -- including broken blood vessels in the brain, clogged blood vessels, or abnormal blood cells -- and is one form of stroke. Although strokes are better known for their effects on older adults, they can also occur in the fetus during pregnancy or the newborn around the time of birth, damaging brain tissue and causing neurological problems. Ongoing research is testing potential treatments that may one day help prevent stroke in fetuses and newborns.

What are the Risk Factors?

Research scientists have examined thousands of expectant mothers, followed them through childbirth, and monitored their children's early neurological development. As a result, they have uncovered certain characteristics, called risk factors, that increase the possibility that a child will later be diagnosed with cerebral palsy:

• Breech presentation. Babies with cerebral palsy are more likely to present feet first, instead of head first, at the beginning of labor.
• Complicated labor and delivery. Vascular or respiratory problems of the baby during labor and delivery may sometimes be the first sign that a baby has suffered brain damage or that a baby's brain has not developed normally. Such complications can cause permanent brain damage.
• Low Apgar score. The Apgar score (named for anesthesiologist Virginia Apgar) is a numbered rating that reflects a newborn's condition. To determine an Apgar score, doctors periodically check the baby's heart rate, breathing, muscle tone, reflexes, and skin color in the first minutes after birth. They then assign points; the higher the score, the more normal the baby's condition. A low score at 10-20 minutes after delivery is often considered an important sign of potential problems.
• Low birthweight and premature birth. The risk of cerebral palsy is higher among babies who weigh less than 2500 grams (5 lbs., 7 1/2 oz.) at birth and among babies who are born less than 37 weeks into pregnancy. This risk increases as birthweight falls.
• Multiple births. Twins, triplets, and other multiple births are linked to an increased risk of cerebral palsy.
• Nervous system malformations. Some babies born with cerebral palsy have visible signs of nervous system malformation, such as an abnormally small head (microcephaly). This suggests that problems occurred in the development of the nervous system while the baby was in the womb.
• Maternal bleeding or severe proteinuria late in pregnancy. Vaginal bleeding during the sixth to ninth months of pregnancy and severe proteinuria (the presence of excess proteins in the urine) are linked to a higher risk of having a baby with cerebral palsy.
• Maternal hyperthyroidism, mental retardation, or seizures. Mothers with any of these conditions are slightly more likely to have a child with cerebral palsy.
• Seizures in the newborn. An infant who has seizures faces a higher risk of being diagnosed, later in childhood, with cerebral palsy.

What Specific Treatments Are Available?

Physical, Behavioral, and Other Therapies

Therapy -- whether for movement, speech, or practical tasks -- is a cornerstone of cerebral palsy treatment. The skills a 2-year-old needs to explore the world are very different from those that a child needs in the classroom or a young adult needs to become independent. Cerebral palsy therapy should be tailored to reflect these changing demands.

Physical therapy usually begins in the first few years of life, soon after the diagnosis is made. Physical therapy programs use specific sets of exercises to work toward two important goals: preventing the weakening or deterioration of muscles that can follow lack of use (called disuse atrophy) and avoiding contracture, in which muscles become fixed in a rigid, abnormal position.

Contracture is one of the most common and serious complications of cerebral palsy. Normally, a child whose bones are growing stretches the body's muscles and tendons through running and walking and other daily activities. This ensures that muscles will grow at the same rate. But in children with cerebral palsy, spasticity prevents this stretching and, as a result, muscles do not grow fast enough to keep up with lengthening bones. The resulting contracture can disrupt balance and trigger loss of previous abilities. Physical therapy alone, or in combination with special braces (sometimes called orthotic devices), works to prevent this complication by stretching spastic muscles. For example, if a child has spastic hamstrings (tendons located behind the knee), the therapist and parents should encourage the child to sit with the legs extended to stretch them.

A third goal of some physical therapy programs is to improve the child's motor development. A widespread program of physical therapy that works toward this goal is the Bobath technique, named for a husband and wife team who pioneered this approach in England. This program is based on the idea that the primitive reflexes retained by many children with cerebral palsy present major roadblocks to learning voluntary control. A therapist using the Bobath technique tries to counteract these reflexes by positioning the child in an opposing movement. So, for example, if a child with cerebral palsy normally keeps his arm flexed, the therapist would repeatedly extend it.

A second such approach to physical therapy is "patterning," which is based on the principle that motor skills should be taught in more or less the same sequence that they develop normally. In this controversial approach, the therapist guides the child with movement problems along the path of normal motor development. For example, the child is first taught elementary movements like pulling himself to a standing position and crawling before he is taught to walk -- regardless of his age. Some experts and organizations, including the American Academy of Pediatrics, have expressed strong reservations about the patterning approach, because studies have not documented its value.

Physical therapy is usually just one element of an infant development program that also includes efforts to provide a varied and stimulating environment. Like all children, the child with cerebral palsy needs new experiences and interactions with the world around him in order to learn. Stimulation programs can bring this valuable experience to the child who is physically unable to explore.

As the child with cerebral palsy approaches school age, the emphasis of therapy shifts away from early motor development. Efforts now focus on preparing the child for the classroom, helping the child master activities of daily living, and maximizing the child's ability to communicate.

Physical therapy can now help the child with cerebral palsy prepare for the classroom by improving his or her ability to sit, move independently or in a wheelchair, or perform precise tasks, such as writing. In occupational therapy, the therapist works with the child to develop such skills as feeding, dressing, or using the bathroom. This can help reduce demands on caregivers and boost self-reliance and self-esteem. For the many children who have difficulty communicating, speech therapy works to identify specific difficulties and overcome them through a program of exercises. For example, if a child has difficulty saying words that begin with "b," the therapist may suggest daily practice with a list of "b" words, increasing their difficulty as each list is mastered. Speech therapy can also work to help the child learn to use special communication devices, such as a computer with voice synthesizers.

Behavioral therapy provides yet another avenue to increase a child's abilities. This therapy, which uses psychological theory and techniques, can complement physical, speech, or occupational therapy. For example, behavioral therapy might include hiding a toy inside a box to reward a child for learning to reach into the box with his weaker hand. Likewise, a child learning to say his "b" words might be given a balloon for mastering the word. In other cases, therapists may try to discourage unhelpful or destructive behaviors, such as hair-pulling or biting, by selectively presenting a child with rewards and praise during other, more positive activities.

As a child with cerebral palsy grows older, the need for and types of therapy and other support services will continue to change. Continuing physical therapy addresses movement problems and is supplemented by vocational training, recreation and leisure programs, and special education when necessary. Counseling for emotional and psychological challenges may be needed at any age, but is often most critical during adolescence. Depending on their physical and intellectual abilities, adults may need attendant care, living accommodations, transportation, or employment opportunities.

Regardless of the patient's age and which forms of therapy are used, treatment does not end when the patient leaves the office or treatment center. In fact, most of the work is often done at home. The therapist functions as a coach, providing parents and patients with the strategy and drills that can help improve performance at home, at school, and in the world. As research continues, doctors and parents can expect new forms of therapy and better information about which forms of therapy are most effective for individuals with cerebral palsy. Drug Therapy

Physicians usually prescribe drugs for those who have seizures associated with cerebral palsy, and these medications are very effective in preventing seizures in many patients. In general, the drugs given to individual patients are chosen based on the type of seizures, since no one drug controls all types. However, different people with the same type of seizure may do better on different drugs, and some individuals may need a combination of two or more drugs to achieve good seizure control.

Drugs are also sometimes used to control spasticity, particularly following surgery. The three medications that are used most often are diazepam, which acts as a general relaxant of the brain and body; baclofen, which blocks signals sent from the spinal cord to contract the muscles; and dantrolene, which interferes with the process of muscle contraction. Given by mouth, these drugs can reduce spasticity for short periods, but their value for long-term control of spasticity has not been clearly demonstrated. They may also trigger significant side effects, such as drowsiness, and their long-term effects on the developing nervous system are largely unknown. One possible solution to avoid such side effects may lie in current research to explore new routes for delivering these drugs.

Patients with athetoid cerebral palsy may sometimes be given drugs that help reduce abnormal movements. Most often, the prescribed drug belongs to a group of chemicals called anticholinergics that work by reducing the activity of acetylcholine. Acetylcholine is a chemical messenger that helps some brain cells communicate and that triggers muscle contraction. Anticholinergic drugs include trihexyphenidyl, benztropine, and procyclidine hydrochloride.

Occasionally, physicians may use alcohol "washes" -- or injections of alcohol into a muscle -- to reduce spasticity for a short period. This technique is most often used when physicians want to correct a developing contracture. Injecting alcohol into a muscle that is too short weakens the muscle for several weeks and gives physicians time to work on lengthening the muscle through bracing, therapy, or casts. In some cases, if the contracture is detected early enough, this technique may avert the need for surgery. Surgery

Surgery is often recommended when contractures are severe enough to cause movement problems. In the operating room, surgeons can lengthen muscles and tendons that are proportionately too short. First, however, they must determine the exact muscles at fault, since lengthening the wrong muscle could make the problem worse.

Finding problem muscles that need correction can be a difficult task. To walk two strides with a normal gait, it takes more than 30 major muscles working at exactly the right time and exactly the right force. A problem in any one muscle can cause abnormal gait. Furthermore, the natural adjustments the body makes to compensate for muscle problems can be misleading. A new tool that enables doctors to spot gait abnormalities, pinpoint problem muscles, and separate real problems from compensation is called gait analysis. Gait analysis combines cameras that record the patient while walking, computers that analyze each portion of the patient's gait, force plates that detect when feet touch the ground, and a special recording technique that detects muscle activity (known as electromyography). Using these data, doctors are better equipped to intervene and correct significant problems. They can also use gait analysis to check surgical results.

Because lengthening a muscle makes it weaker, surgery for contractures is usually followed by months of recovery. For this reason, doctors try to fix all of the affected muscles at once when it is possible or, if more than one surgical procedure is unavoidable, they may try to schedule operations close together.

A second surgical technique, known as selective dorsal root rhizotomy, aims to reduce spasticity in the legs by reducing the amount of stimulation that reaches leg muscles via nerves. In the procedure, doctors try to locate and selectively sever overactivated nerves controlling leg muscles. Although there is scientific controversy over how selective this technique actually is, recent research results suggest it can reduce spasticity in some patients, particularly those who have spastic diplegia. Ongoing research is evaluating this surgery's effectiveness.

Experimental surgical techniques include chronic cerebellar stimulation and stereotaxic thalamotomy. In chronic cerebellar stimulation, electrodes are implanted on the surface of the cerebellum -- the part of the brain responsible for coordinating movement -- and are used to stimulate certain cerebellar nerves. While it was hoped that this technique would decrease spasticity and improve motor function, results of this invasive procedure have been mixed. Some studies have reported improvements in spasticity and function, others have not.

Stereotaxic thalamotomy involves precise cutting of parts of the thalamus, which serves as the brain's relay station for messages from the muscles and sensory organs. This has been shown effective only for reducing hemiparetic tremors (see glossary). Mechanical aids

Whether they are as humble as velcro shoes or as advanced as computerized communication devices, special machines and gadgets in the home, school, and workplace can help the child or adult with cerebral palsy overcome limitations.

The computer is probably the most dramatic example of a new device that can make a difference in the lives of those with cerebral palsy. For example, a child who is unable to speak or write but can make head movements may be able to learn to control a computer using a special light pointer that attaches to a headband. Equipped with a computer and voice synthesizer, this child could communicate with others. In other cases, technology has led to new versions of old devices, such as the traditional wheelchair and its modern offspring that runs on electricity.

Many such devices are products of engineering research supported by private foundations and other groups. What Other Major Problems Are Associated with Cerebral Palsy?

Poor control of the muscles of the throat, mouth and tongue sometimes leads to drooling. Drooling can cause severe skin irritation and, because it is socially unacceptable, can lead to further isolation of affected children from their peers. Although numerous treatments for drooling have been tested over the years, there is no one treatment that always helps. Drugs called anticholinergics can reduce the flow of saliva but may cause significant side effects, such as mouth dryness and poor digestion. Surgery, while sometimes effective, carries the risk of complications, including worsening of swallowing problems. Some patients benefit from a technique called biofeedback that can tell them when they are drooling or having difficulty controlling muscles that close the mouth. This kind of therapy is most likely to work if the patient has a mental age of more than 2 or 3 years, is motivated to control drooling, and understands that drooling is not socially acceptable.

Difficulty with eating and swallowing -- also triggered by motor problems in the mouth -- can cause poor nutrition. Poor nutrition, in turn, may make the individual more vulnerable to infections and cause or aggravate "failure to thrive" -- a lag in growth and development that is common among those with cerebral palsy. To make swallowing easier, the caregiver may want to prepare semisolid food, such as strained vegetables and fruits. Proper position, such as sitting up while eating or drinking and extending the individual's neck away from the body to reduce the risk of choking, is also helpful. In severe cases of swallowing problems and malnutrition, physicians may recommend tube feeding, in which a tube delivers food and nutrients down the throat and into the stomach, or gastrostomy, in which a surgical opening allows a tube to be placed directly into the stomach.

A common complication is incontinence, caused by faulty control over the muscles that keep the bladder closed. Incontinence can take the form of bed-wetting (also known as enuresis), uncontrolled urination during physical activities (or stress incontinence), or slow leaking of urine from the bladder. Possible medical treatments for incontinence include special exercises, biofeedback, prescription drugs, surgery, or surgically implanted devices to replace or aid muscles. Specially designed undergarments are also available.

Information Source

National Institute of Neurological Disorders and Stroke

NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.