In the 1860s, an English surgeon named William Little wrote the first medical descriptions of a puzzling disorder that struck children in the first years of life, causing stiff, spastic muscles in their legs and, to a lesser degree, their arms. These children had difficulty grasping objects, crawling, and walking. They did not get better as they grew up nor did they become worse. Their condition, which was called Little's disease for many years, is now known as spastic diplegia. It is one of several disorders that affect control of movement and are grouped together under the term cerebral palsy.

Because it seemed that many of these children were born following premature or complicated deliveries, Little suggested their condition resulted from a lack of oxygen during birth. This oxygen shortage damaged sensitive brain tissues controlling movement, he proposed. But in 1897, the famous psychiatrist Sigmund Freud disagreed. Noting that children with cerebral palsy often had other problems such as mental retardation, visual disturbances, and seizures, Freud suggested that the disorder might sometimes have roots earlier in life, during the brain's development in the womb. "Difficult birth, in certain cases," he wrote, "is merely a symptom of deeper effects that influence the development of the fetus."

Despite Freud's observation, the belief that birth complications cause most cases of cerebral palsy was widespread among physicians, families, and even medical researchers until very recently. In the 1980s, however, scientists analyzed extensive data from a government study of more than 35,000 births and were surprised to discover that such complications account for only a fraction of cases -- probably less than 10 percent. In most cases of cerebral palsy, no cause of the factors explored could be found. These findings from the NINDS perinatal study have profoundly altered medical theories about cerebral palsy and have spurred today's researchers to explore alternative causes.

At the same time, biomedical research has also led to significant changes in understanding, diagnosing, and treating persons with cerebral palsy. Risk factors not previously recognized have been identified, notably intrauterine exposure to infection and disorders of coagulation, and others are under investigation. Identification of infants with cerebral palsy very early in life gives youngsters the best opportunity to receive treatment for sensory disabilities and for prevention of contractures. Biomedical research has led to improved diagnostic techniques such as advanced brain imaging and modern gait analysis. Certain conditions known to cause cerebral palsy, such as rubella (German measles) and jaundice, can now be prevented or treated. Physical, psychological, and behavioral therapy that assist with such skills as movement and speech and foster social and emotional development can help children who have cerebral palsy to achieve and succeed. Medications, surgery, and braces can often improve nerve and muscle coordination, help treat associated medical problems, and either prevent or correct deformities.

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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 term cerebral refers to the brain's two halves, or hemispheres, and palsy describes any disorder that impairs control of body movement. Thus, these disorders are not caused by problems in the muscles or nerves. Instead, faulty development or damage to motor areas in the brain disrupts the brain's ability to adequately control movement and posture.

Symptoms of cerebral palsy lie along a spectrum of varying severity. An individual with cerebral palsy may have difficulty with fine motor tasks, such as writing or cutting with scissors; experience trouble with maintaining balance and walking; or be affected by involuntary movements, such as uncontrollable writhing motion of the hands or drooling. The symptoms differ from one person to the next, and may even change over time in the individual. Some people with cerebral palsy are also affected by other medical disorders, including seizures or mental impairment. Contrary to common belief, however, cerebral palsy does not always cause profound handicap. While a child with severe cerebral palsy might be unable to walk and need extensive, lifelong care, a child with mild cerebral palsy might only be slightly awkward and require no special assistance.

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Infantile cerebral palsy (ICP) is one of the most common diseases of the nervous system in children. The incidence ranges from 2.5 to 5.9 cases per 1,000 newborns (L.O. Badalyan, 1984), but, taking into account the so-called perinatal and early postnatal encephalopathies, the number of affected children may be even higher.

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.

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Cerebral palsy is classified into four broad categories -- spastic, athetoid, ataxic, and mixed forms -- in accordance with the type of movement disturbance.

Spastic cerebral palsy is the most common and affects 70 to 80 percent of patients. The muscles are stiffly and permanently contracted. The type of spastic cerebral palsy a patient has, is 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.

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 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 is a rare form, affecting 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.

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Many individuals who have cerebral palsy have no associated medical disorders. However, disorders that involve the brain and impair its motor function can also cause seizures and impair an individual's intellectual development, attentiveness to the outside world, activity and behavior, and vision and hearing. Medical disorders associated with cerebral palsy include:

• Mental Impairment

About one-third of children who have cerebral palsy are mildly intellectually impaired, one-third are moderately or severely impaired, and the remaining third are intellectually normal. Mental impairment is even more common among children with spastic quadriplegia.

• Seizures or Epilepsy

As many as half of all children with cerebral palsy have seizures. In the person who has cerebral palsy and epilepsy, this disruption may be spread throughout the brain and cause varied symptoms all over the body -- as in tonic-clonic seizures -- or may be confined to just one part of the brain and cause more specific symptoms -- as in partial seizures.

Tonic-clonic seizures generally cause patients to cry out and are followed by loss of consciousness, twitching of both legs and arms, convulsive body movements, and loss of bladder control.

Partial seizures are classified as simple or complex. In simple partial seizures, the individual has localized symptoms, such as muscle twitches, chewing movements, and numbness or tingling. In complex partial seizures, the individual may hallucinate, stagger, perform automatic and purposeless movements, or experience impaired consciousness or confusion.

• Growth Problems

A syndrome called failure to thrive is common in children with moderate-to-severe cerebral palsy, especially those with spastic quadriparesis. Failure to thrive is a general term physicians use to describe children who seem to lag behind in growth and development despite having enough food. In babies, this lag usually takes the form of too little weight gain; in young children, it can appear as abnormal shortness; in teenagers, it may appear as a combination of shortness and lack of sexual development.

Failure to thrive probably has several causes, including, in particular, poor nutrition and damage to the brain centers controlling growth and development. In addition, the muscles and limbs affected by cerebral palsy tend to be smaller than normal. This is especially noticeable in some patients with spastic hemiplegia, because limbs on the affected side of the body may not grow as quickly or as large as those on the more normal side. This condition usually affects the hand and foot most severely. Since the involved foot in hemiplegia is often smaller than the unaffected foot even among patients who walk, this size difference is probably not due to lack of use. Scientists believe the problem is more likely to result from disruption of the complex process responsible for normal body growth.

• Impaired Vision or Hearing

A large number of children with cerebral palsy have strabismus, a condition in which the eyes are not aligned because of differences in the left and right eye muscles. In an adult, this condition causes double vision. In children, however, the brain often adapts to the condition by ignoring signals from one of the misaligned eyes. Untreated, this can lead to very poor vision in one eye and can interfere with certain visual skills, such as judging distance. In some cases, physicians may recommend surgery to correct strabismus.

Children with hemiparesis may have hemianopia, which is defective vision or blindness that impairs the normal field of vision of one eye. For example, when hemianopia affects the right eye, a child looking straight ahead might have perfect vision except on the far right. In homonymous hemianopia, the impairment affects the same part of the visual field of both eyes. Impaired hearing is also more frequent among those with cerebral palsy than in the general population.

• Abnormal Sensation and Perception

Some children with cerebral palsy have impaired ability to feel simple sensations like touch and pain. They may also have stereognosia, or difficulty perceiving and identifying objects using the sense of touch. A child with stereognosia, for example, would have trouble identifying a hard ball, sponge, or other object placed in his hand without looking at the object

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Most students of cerebral palsy agree that the causes are more often found in events during pregnancy than birth (see: British Medical Journal 1999;319:1054-9). Others dispute this hypothesis (see: BMJ 2000;320:1626 17 June), arguing that an important minority of neurodevelopmental problems in children are secondary to perinatal hypoxic-ischaemic damage (lack of oxygen during birth). The ongoing argument is to set the proportion related to pregnancy versus the proportion due to events during birth. Clearly an infant that is compromised during pregnancy is more vulnerable to the stress of birth, so the argument may be somewhat pointless.

Dr. Peter Dear, consultant and senior lecturer in neonatal medicine at St James's Hospital, Leeds, said of the British Medical Journal's consensus statement that most cerebral palsy is related to pregnancy events:

"It is based upon an inadequate and eclectic appraisal of the available literature. The notion of ... rigid statements about causation based upon outcome do not marry well with the duty of the clinician or medicolegal experts to form a balanced view based upon available evidence, without prejudice.

This consensus document attempts to define criteria in such a way as to disallow consideration of an intrapartum cause for damage in many cases. The arguments are not well founded and add little to the discussion in this controversial area."

Support for the consensus statement comes from Professor Alastair MacLennan, Associate Professor in Obstetrics and Gynecology, University of Adelaide, Australia, who calls for re-education of the public on the issue, emphasizing that no proof exists that any obstetric policies can be used to reduce cerebral palsy rates at term.

We would argue that against this statement, saying that the avoidance of analgesia, anesthesia, and other medications during labor and birth, would have some positive effect, though how much is unknown.

Dr. MacLennan argues that, "...[O]bstetricians and midwives should not be used as a de facto social welfare insurance scheme for children with cerebral palsy. Children with neurological disability would be better served with a limited speedy no-fault system run by the government. We need to re-educate the public and some paediatricians to the fact that the neuropathology of cerebral palsy is established in the large majority of cases antenatally."

Professor MacLennan also points out that magnetic resonance imaging is still not a good enough tool to judge adequately if a neuropathological event has occurred. Arnold Simanowitz, chief executive of the Action for Victims of Medical Accidents, said of the importance of this issue: "To know that it [a child's cerebral palsy] could have been avoided simply adds to the burden of parents."

The gist of this is that arguments exist about the degree to which birth events results in cerebral palsy. Our view is that both are correct.. Severe birth asphyxia (lack of oxygen) can damage children. But so can chronic events during pregnancy. We cannot dispute the many children who have normal, easy labors, but still have cerebral palsy. Nor can we dispute the children who have terribly traumatic labors, and are diagnosed with cerebral palsy.

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• 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.

Parents should not become too alarmed if their child has one or more of these factors. Most such children do not have and do not develop cerebral palsy.

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Several of the causes of cerebral palsy that have been identified through research are preventable or treatable:

Head injury can be prevented by regular use of child safety seats when driving in a car, wearing helmets during bicycle rides, and by the elimination of child abuse. In addition, common sense measures around the household - like close supervision during bathing and keeping poisons out of reach - can reduce the risk of accidental injury.

Jaundice of newborn infants can be treated with phototherapy. In phototherapy, babies are exposed to special blue lights that break down bile pigments, preventing them from building up and threatening the brain. In the few cases in which this treatment is not enough, physicians can correct the condition with a special form of blood transfusion.

Rh incompatibility is easily identified by a simple blood test routinely performed on expectant mothers and, if indicated, expectant fathers. This incompatibility in blood types does not usually cause problems during a woman's first pregnancy, since the mother's body generally does not produce the unwanted antibodies until after delivery. In most cases, a special serum given after each childbirth can prevent the unwanted production of antibodies. In unusual cases, such as when a pregnant woman develops the antibodies during her first pregnancy or antibody production is not prevented, doctors can help minimize problems by closely watching the developing baby and, when needed, performing a transfusion to the baby while in the womb or an exchange transfusion (in which a large volume of the baby's blood is removed and replaced) after birth.

Rubella, or German measles, can be prevented if women are vaccinated against this disease before becoming pregnant. Be sure to see our comprehensive section on Vaccines.

In addition, it is always good to work toward a healthy pregnancy through regular prenatal care and good nutrition and by eliminating smoking, alcohol consumption, and drug abuse. Despite the best efforts of parents and physicians, however, children will still be born with cerebral palsy. Since in most cases the cause of cerebral palsy is unknown, only a small amount can currently be done to prevent it. As investigators learn more about the causes of cerebral palsy through basic and clinical research, doctors and parents will be better equipped to help prevent this disorder.

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Early signs of cerebral palsy usually appear before 3 years of age, and parents are often the first to suspect that their infant is not developing motor skills normally. Infants with cerebral palsy are frequently slow to reach developmental milestones, such as learning to roll over, sit, crawl, smile, or walk. This is sometimes called developmental delay.

Some affected children have abnormal muscle tone. Decreased muscle tone is called hypotonia; the baby may seem flaccid and relaxed, even floppy. Increased muscle tone is called hypertonia, and the baby may seem stiff or rigid. In some cases, the baby has an early period of hypotonia that progresses to hypertonia after the first 2 to 3 months of life. Affected children may also have unusual posture or favor one side of their body.

Parents who are concerned about their baby's development for any reason should contact their physician, who can help distinguish normal variation in development from a developmental disorder.

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Doctors diagnose cerebral palsy by testing an infant's motor skills and looking carefully at the infant's medical history. In addition to checking for those symptoms described above - slow development, abnormal muscle tone, and unusual posture - a physician also tests the infant's reflexes and looks for early development of hand preference.

Reflexes are movements that the body makes automatically in response to a specific cue. For example, if a newborn baby is held on its back and tilted so the legs are above its head, the baby will automatically extend its arms in a gesture, called the Moro reflex, that looks like an embrace. Babies normally lose this reflex after they reach 6 months, but those with cerebral palsy may retain it for abnormally long periods. This is just one of several reflexes that a physician can check.

Doctors can also look for hand preference - a tendency to use either the right or left hand more often. When the doctor holds an object in front and to the side of the infant, an infant with hand preference will use the favored hand to reach for the object, even when it is held closer to the opposite hand. During the first 12 months of life, babies do not usually show hand preference. But infants with spastic hemiplegia, in particular, may develop a preference much earlier, since the hand on the unaffected side of their body is stronger and more useful.

The next step in diagnosing cerebral palsy is to rule out other disorders that can cause movement problems. Most importantly, doctors must determine that the child's condition is not getting worse. Although its symptoms may change over time, cerebral palsy by definition is not progressive. If a child is continuously losing motor skills, the problem more likely springs from elsewhere - possibly including genetic diseases, muscle diseases, disorders of metabolism, or tumors in the nervous system. The child's medical history, special diagnostic tests, and, in some cases, repeated check-ups can help confirm that other disorders are not at fault.

The doctor may also order specialized tests to learn more about the possible cause of cerebral palsy. One such test is computed tomography, or CT, a sophisticated imaging technique that uses X-rays and a computer to create an anatomical picture of the brain's tissues and structures. A CT scan may reveal brain areas that are underdeveloped, abnormal cysts (sacs that are often filled with liquid) in the brain, or other physical problems. With the information from CT scans, doctors may be better equipped to judge the long-term outlook for an affected child.

Magnetic resonance imaging, or MRI, is a relatively new brain imaging technique that is rapidly gaining widespread use for identifying brain disorders. This technique uses a magnetic field and radio waves, rather than X-rays. MRI gives better pictures of structures or abnormal areas located near bone than CT scans.

A third test that can expose problems in brain tissues is ultrasonography. This technique bounces sound waves off the brain and uses the pattern of echoes to form a picture, or sonogram, of its structures. Ultrasonography can be used in infants before the bones of the skull harden and close. Although it is less precise than CT and MRI scanning, this technique can detect cysts and structures in the brain, is less expensive, and does not require long periods of immobility.

Finally, physicians may want to look for other conditions that are linked to cerebral palsy, including seizure disorders, mental impairment, and vision or hearing problems.

When the doctor suspects a seizure disorder, an electroencephalogram, or EEG, may be ordered. An EEG uses special patches called electrodes placed on the scalp to record the natural electrical currents inside the brain. This recording can help the doctor see telltale patterns in the brain's electrical activity that suggest a seizure disorder.

Intelligence tests are often used to determine if a child with cerebral palsy is mentally impaired. It is important to note, however, that sometimes a child's intelligence may be underestimated because problems with movement, sensation, or speech due to cerebral palsy make it difficult for him or her to perform well on these tests.

If problems with vision are suspected, the doctor may refer the patient to an ophthalmologist for examination; if hearing impairment seems likely, an otologist may be called in.

Identifying these accompanying conditions is important and is becoming more accurate as ongoing research yields advances that make diagnosis easier. Many of these conditions can then be addressed through specific treatments, improving the long-term outlook for those with cerebral palsy.

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Conventional medical wisdom holds that cerebral palsy cannot be cured, with treatment aimed at improving the child's capabilities. There is no standard therapy that works for all patients.

Some approaches that can be included in this plan are drugs to control seizures and muscle spasms, special braces to compensate for muscle imbalance, surgery, mechanical aids to help overcome impairments, counseling for emotional and psychological needs, and physical, occupational, speech, and behavioral therapy. In general, the earlier treatment begins, the better chance a child has of overcoming developmental disabilities or learning new ways to accomplish difficult tasks.

A typical treatment team for a child with cerebral palsy might include:

• A physician, such as a family physician, pediatrician, pediatric neurologist, or pediatric physiatrist, trained to help developmentally disabled children. This physician, often the leader of the treatment team, works to synthesize the professional advice of all team members into a comprehensive treatment plan, implements treatments, and follows the patient's progress over a number of years.

• An orthopedist, a surgeon who specializes in treating the bones, muscles, tendons, and other parts of the body's skeletal system. An orthopedist might be called on to predict, diagnose, or treat muscle problems associated with cerebral palsy.

• A physical therapist, who designs and implements special exercise programs to improve movement and strength.

• An occupational therapist, who can help patients learn skills for day-to-day living, school, and work.

• A speech and language pathologist, who specializes in diagnosing and treating communication problems.

• A social worker, who can help patients and their families locate community assistance and education programs.

• A psychologist, who helps patients and their families cope with the special stresses and demands of cerebral palsy. In some cases, psychologists may also oversee therapy to modify unhelpful or destructive behaviors or habits.

• An educator, who may play an especially important role when mental impairment or learning disabilities present a challenge to education.

Individuals who have cerebral palsy and their family or caregivers should be intimately involved in all steps of planning, making decisions, and applying treatments. Studies have shown that family support and personal determination are two of the most important predictors of which individuals who have cerebral palsy will achieve long-term goals.

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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.

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