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Cerebral Palsy

Cerebral Palsy

Definition

Cerebral palsy (CP) is the term used for a group of nonprogressive disorders of movement and posture caused by abnormal development of, or damage to, motor control centers of the brain. CP is caused by events before, during, or after birth. The abnormalities of muscle control that define CP are often accompanied by other neurological and physical abnormalities.

Description

Voluntary movement (walking, grasping, chewing, etc.) is primarily accomplished using muscles that are attached to bones, known as the skeletal muscles. Control of the skeletal muscles originates in the cerebral cortex, the largest portion of the brain. Palsy means paralysis, but may also be used to describe uncontrolled muscle movement. Therefore, cerebral palsy encompasses any disorder of abnormal movement and paralysis caused by abnormal function of the cerebral cortex. In truth, however, CP does not include conditions due to progressive disease or degeneration of the brain. For this reason, CP is also referred to as static (nonprogressive) encephalopathy (disease of the brain). Also excluded from CP are any disorders of muscle control that arise in the muscles themselves and/or in the peripheral nervous system (nerves outside the brain and spinal cord).

CP is not a specific diagnosis, but is more accurately considered a description of a broad but defined group of neurological and physical problems.

The symptoms of CP and their severity are quite variable. Those with CP may have only minor difficulty with fine motor skills, such as grasping and manipulating items with their hands. A severe form of CP could involve significant muscle problems in all four limbs, mental retardation, seizures, and difficulties with vision, speech, and hearing.

Muscles that receive defective messages from the brain may be constantly contracted and tight (spastic), exhibit involuntary writhing movements (athetosis), or have difficulty with voluntary movement (dyskinesia). There can also be a lack of balance and coordination with unsteady movements (ataxia). A combination of any of these problems may also occur. Spastic CP and mixed CP constitute the majority of cases. Effects on the muscles can range from mild weakness or partial paralysis (paresis), to complete loss of voluntary control of a muscle or group of muscles (plegia). CP is also designated by the number of limbs affected. For instance, affected muscles in one limb is monoplegia, both arms or both legs is diplegia, both limbs on one side of the body is hemiplegia, and in all four limbs is quadriplegia. Muscles of the trunk, neck, and head may be affected as well.

CP can be caused by a number of different mechanisms at various timesfrom several weeks after conception, through birth, to early childhood. For many years, it was accepted that most cases of CP were due to brain injuries received during a traumatic birth, known as birth asphyxia. However, extensive research in the 1980s showed that only 5-10% of CP can be attributed to birth trauma. Other possible causes include abnormal development of the brain, prenatal factors that directly or indirectly damage neurons in the developing brain, premature birth, and brain injuries that occur in the first few years of life.

Advances in the medical care of premature infants in the last 20 years have dramatically increased the rate of survival of these fragile newborns. However, as gestational age at delivery and birth weight of a baby decrease, the risk for CP dramatically increases. A term pregnancy is delivered at 37-41 weeks gestation. The risk for CP in a preterm infant (32-37 weeks) is increased about five-fold over the risk for an infant born at term. Survivors of extremely preterm births (less than 28 weeks) face as much as a 50-fold increase in risk. About 50% of all cases of CP now being diagnosed are in children who were born prematurely.

Two factors are involved in the risk for CP associated with prematurity. First, premature babies are at higher risk for various CP-associated medical complications, such as intracerebral hemorrhage, infection, and difficulty in breathing, to name a few. Second, the onset of premature labor may be induced, in part, by complications that have already caused neurologic damage in the fetus. A combination of both factors almost certainly plays a role in some cases of CP. The tendency toward premature delivery runs in families, but the genetic mechanisms are far from clear.

An increase in multiple pregnancies in recent years, especially in the United States, is blamed on the increased use of fertility drugs. As the number of fetuses in a pregnancy increases, the risks for abnormal development and premature delivery also increase. Children from twin pregnancies have four times the risk of developing CP as children from singleton pregnancies, owing to the fact that more twin pregnancies are delivered prematurely. The risk for CP in a child of triplets is up to 18 times greater. Furthermore, recent evidence suggests that a baby from a pregnancy in which its twin died before birth is at increased risk for CP.

Approximately 500,000 children and adults in the United States have CP, and it is newly diagnosed in about 6,000 infants and young children each year. The incidence of CP has not changed much in the last 20-30 years. Ironically, while advances in medicine have decreased the incidence from some causesRh disease for examplethey have increased it from others, notably, prematurity and multiple pregnancies. No particular ethnic groups seem to be at higher risk for CP. However, people of disadvantaged back-ground are at higher risk due to poorer access to proper prenatal care and advanced medical services.

Causes and symptoms

As noted, CP has many causes, making a discussion of the genetics of CP complicated. A number of hereditary/genetic syndromes have signs and symptoms similar to CP, but usually also have problems not typical of CP. Put another way, some hereditary conditions "mimic" CP. Isolated CP, meaning CP that is not a part of some other syndrome or disorder, is usually not inherited.

It might be possible to group the causes of CP into those that are genetic and those that are non-genetic, but most would fall somewhere in between. Grouping causes into those that occur during pregnancy (prenatal), those that happen around the time of birth (perinatal), and those that occur after birth (postnatal), is preferable. CP related to premature birth and multiple pregnancies (twins, triplets, etc., not "many pregnancies") is somewhat different and considered separately.

Prenatal causes

Although much has been learned about human embryology in the last few decades, a great deal remains unknown. Studying prenatal human development is difficult because the embryo and fetus develop in a closed environmentthe mother's womb. However, the relatively recent development of a number of prenatal tests has opened a window on the process. Add to that more accurate and complete evaluations of newborns, especially those with problems, and a clearer picture of what can go wrong before birth is possible.

The complicated process of brain development before birth is susceptible to many chance errors that can result in abnormalities of varying degrees. Some of these errors will result in structural anomalies of the brain, while others may cause undetectable, but significant, abnormalities in how the cerebral cortex is "wired." An abnormality in structure or wiring is sometimes hereditary, but is most often due to chance, or a cause unknown at this time. Whether and how much genetics played a role in a particular brain abnormality depends to some degree on the type of anomaly and the form of CP it causes.

Several maternal-fetal infections are known to increase the risk for CP, including rubella (German measles, now rare in the United States), cytomegalovirus (CMV), and toxoplasmosis. Each of these infections is considered a risk to the fetus only if the mother contracts it for the first time during that pregnancy. Even in those cases, though, most babies will be born normal. Most women are immune to all three infections by the time they reach childbearing age, but a woman's immune status can be determined using the so-called TORCH (for Toxoplasmosis, Rubella, Cytomegalovirus, and Herpes) test before or during pregnancy.

Reserchers continue to study the role of perinatal infection in development of cerebral palsy. New evidence in 2004 linked inflammatory cytokines to possible cerebral injury that could lead to CP. Scientists suggested new research with intravenous immunoglobulin to limit inflammatory damage.

Just as a stroke can cause neurologic damage in an adult, so too can this type of event occur in the fetus. A burst blood vessel in the brain followed by uncontrolled bleeding (coagulopathy), known as intracerebral hemorrhage, could cause a fetal stroke, or a cerebral blood vessel could be obstructed by a clot (embolism ). Infants who later develop CP, along with their mothers, are more likely than other mother-infant pairs to test positive for factors that put them at increased risk for bleeding episodes or blood clots. Some coagulation disorders are strictly hereditary, but most have a more complicated basis.

A teratogen is any substance to which a woman is exposed that has the potential to harm the embryo or fetus. Links between a drug or other chemical exposure during pregnancy and a risk for CP are difficult to prove. However, any substance that might affect fetal brain development, directly or indirectly, could increase the risk for CP. Furthermore, any substance that increases the risk for premature delivery and low birth weight, such as alcohol, tobacco, or cocaine, among others, might indirectly increase the risk for CP.

The fetus receives all nutrients and oxygen from blood that circulates through the placenta. Therefore, anything that interferes with normal placental function might adversely affect development of the fetus, including the brain, or might increase the risk for premature delivery. Structural abnormalities of the placenta, premature detachment of the placenta from the uterine wall (abruption), and placental infections (chorioamnionitis) are thought to pose some risk for CP.

Certain conditions in the mother during pregnancy might pose a risk to fetal development leading to CP. Women with autoimmune anti-thyroid or anti-phospholipid (APA) antibodies are at slightly increased risk for CP in their children. A potentially important clue uncovered recently points toward high levels of cytokines in the maternal and fetal circulation as a possible risk for CP. Cytokines are proteins associated with inflammation, such as from infection or autoimmune disorders, and they may be toxic to neurons in the fetal brain. More research is needed to determine the exact relationship, if any, between high levels of cytokines in pregnancy and CP. A woman has some risk of developing the same complications in more than one pregnancy, slightly increasing the risk for more than one child with CP.

Serious physical trauma to the mother during pregnancy could result in direct trauma to the fetus as well, or injuries to the mother could compromise the availability of nutrients and oxygen to the developing fetal brain.

Perinatal causes

Birth asphyxia significant enough to result in CP is now uncommon in developed countries. Tight nuchal cord (umbilical cord around the baby's neck) and prolapsed cord (cord delivered before the baby) are possible causes of birth asphyxia, as are bleeding and other complications associated with placental abruption and placenta previa (placenta lying over the cervix).

Infection in the mother is sometimes not passed to the fetus through the placenta, but is transmitted to the baby during delivery. Any such infection that results in serious illness in the newborn has the potential to produce some neurological damage.

Postnatal causes

The remaining 15% of CP is due to neurologic injury sustained after birth. CP that has a postnatal cause is sometimes referred to as acquired CP, but this is only accurate for those cases caused by infection or trauma.

Incompatibility between the Rh blood types of mother and child (mother Rh negative, baby Rh positive) can result in severe anemia in the baby (erythroblastosis fetalis ). This may lead to other complications, including severe jaundice, which can cause CP. Rh disease in the newborn is now rare in developed countries due to routine screening of maternal blood type and treatment of pregnancies at risk. The routine, effective treatment of jaundice due to other causes has also made it an infrequent cause of CP in developed countries. Rh blood type poses a risk for recurrence of Rh disease if treatment is not provided.

Serious infections that affect the brain directly, such as meningitis and encephalitis, may cause irreversible damage to the brain, leading to CP. A seizure disorder early in life may cause CP, or may be the product of a hidden problem that causes CP in addition to seizures. Unexplained (idiopathic) seizures are hereditary in only a small percentage of cases. Although rare in infants born healthy at or near term, intracerebral hemorrhage and brain embolism, like fetal stroke, are sometimes genetic.

Physical trauma to an infant or child resulting in brain injury, such as from abuse, accidents, or near drowning/suffocation, might cause CP. Likewise, ingestion of a toxic substance such as lead, mercury, poisons, or certain chemicals could cause neurological damage. Accidental overdose of certain medications might also cause similar damage to the central nervous system.

By definition, the defect in cerebral function causing CP is nonprogressive. However, the symptoms of CP often change over time. Most of the symptoms of CP relate in some way to the abnormal control of muscles. To review, CP is categorized first by the type of movement/postural disturbance(s) present, then by a description of which limbs are affected, and finally by the severity of motor impairment. For example, spastic diplegia refers to continuously tight muscles that have no voluntary control in both legs, while athetoid quadraparesis describes uncontrolled writhing movements and muscle weakness in all four limbs. These three-part descriptions are helpful in providing a general picture, but cannot give a complete description of any one person with CP. In addition, the various "forms" of CP do not occur with equal frequencyspastic diplegia is seen in more individuals than is athetoid quadraparesis. CP can also be loosely categorized as mild, moderate, or severe, but these are very subjective terms with no firm boundaries between them.

A muscle that is tensed and contracted is hypertonic, while excessively loose muscles are hypotonic. Spastic, hypertonic muscles can cause serious orthopedic problems, including scoliosis (spine curvature), hip dislocation, or contractures. A contracture is shortening of a muscle, aided sometimes by a weak-opposing force from a neighboring muscle. Contractures may become permanent, or "fixed," without some sort of intervention. Fixed contractures may cause postural abnormalities in the affected limbs. Clenched fists and contracted feet (equinus or equinovarus) are common in people with CP. Spasticity in the thighs causes them to turn in and cross at the knees, resulting in an unusual method of walking known as a "scissors gait." Any of the joints in the limbs may be stiff (immobilized) due to spasticity of the attached muscles.

Athetosis and dyskinesia often occur with spasticity, but do not often occur alone. The same is true of ataxia. It is important to remember that "mild CP" or "severe CP" refers not only to the number of symptoms present, but also to the level of involvement of any particular class of symptoms.

Mechanisms that can cause CP are not always restricted to motor-control areas of the brain. Other neurologically based symptoms may include:

  • mental retardation/learning disabilities
  • behavioral disorders
  • seizure disorders
  • visual impairment
  • hearing loss
  • speech impairment (dysarthria)
  • abnormal sensation and perception

These problems may have a greater impact on a child's life than the physical impairments of CP, although not all children with CP are affected by other problems. Many infants and children with CP have growth impairment. About one-third of individuals with CP have moderate-to-severe mental retardation, one-third have mild mental retardation, and one-third have normal intelligence.

Diagnosis

The signs of CP are not usually noticeable at birth. Children normally progress through a predictable set of developmental milestones through the first 18 months of life. Children with CP, however, tend to develop these skills more slowly because of their motor impairments, and delays in reaching milestones are usually the first symptoms of CP. Babies with more severe cases of CP are usually diagnosed earlier than others.

Selected developmental milestones, and the ages for normally acquiring them, are given below. If a child does not acquire the skill by the age shown in parentheses, there is some cause for concern.

  • sits well unsupported6 months (8-10 months)
  • babblessix months (eight months)
  • crawlsnine months (12 months)
  • finger feeds, holds bottlenine months (12 months)
  • walks alone12 months (15-18 months)
  • uses one or two words other than dada/mama12 months (15 months)
  • walks up and down steps24 months (24-36 months)
  • turns pages in books; removes shoes and socks24 months (30 months)

Children do not consistently favor one hand over the other before 12-18 months, and doing so may be a sign that the child has difficulty using the other hand. This same preference for one side of the body may show up as asymmetric crawling or, later on, favoring one leg while climbing stairs.

It must be remembered that children normally progress at somewhat different rates, and slow beginning accomplishment is often followed by normal development. Other causes for developmental delaysome benign, some seriousshould be excluded before considering CP as the answer. CP is nonprogressive, so continued loss of previously acquired milestones indicates that CP is not the cause of the problem.

No one test is diagnostic for CP, but certain factors increase suspicion. The Apgar score measures a baby's condition immediately after birth. Babies that have low Apgar scores are at increased risk for CP. Presence of abnormal muscle tone or movements may indicate CP, as may the persistence of infantile reflexes. Imaging of the brain using ultrasound, x rays, MRI, and/or CT scans may reveal a structural anomaly. Some brain lesions associated with CP include scarring, cysts, expansion of the cerebral ventricles (hydrocephalus ), periventricular leukomalacia (an abnormality of the area surrounding the ventricles), areas of dead tissue (necrosis), and evidence of an intracerebral hemorrhage or blood clot. Blood and urine biochemical tests, as well as genetic tests, may be used to rule out other possible causes, including muscle and peripheral nerve diseases, mitochondrial and metabolic diseases, and other inherited disorders. Evaluations by a pediatric developmental specialist and a geneticist may be of benefit.

Treatment

Cerebral palsy cannot be cured, but many of the disabilities it causes can be managed through planning and timely care. Treatment for a child with CP depends on the severity, nature, and location of the primary muscular symptoms, as well as any associated problems that might be present. Optimal care of a child with mild CP may involve regular interaction with only a physical therapist and occupational therapist, whereas care for a more severely affected child may include visits to multiple medical specialists throughout life. With proper treatment and an effective plan, most people with CP can lead productive, happy lives.

Therapy

Spasticity, muscle weakness, coordination, ataxia, and scoliosis are all significant impairments that affect the posture and mobility of a person with CP. Physical and occupational therapists work with the patient, and the family, to maximize the ability to move affected limbs, develop normal motor patterns, and maintain posture. "Assistive technology," things such as wheelchairs, walkers, shoe inserts, crutches, and braces, are often required. A speech therapist, and high-tech aids such as computer-controlled communication devices, can make a tremendous difference in the life of those who have speech impairments. A new experimental physical therapy treatment was tested on children in 2004 that often is used on adults who have had strokes. Called constraint-induced movement therapy, it consists of placing the child's stronger arm in a case for three weeks and giving him or her 21 straight days to retrain the weaker arm.

Medications

Before fixed contractures develop, muscle-relaxant drugs such as diazepam (Valium), dantrolene (Dantrium), and baclofen (Lioresal) may be prescribed. Botulinum toxin (Botox), a newer and highly effective treatment, is injected directly into the affected muscles. Alcohol or phenol injections into the nerve controlling the muscle are another option. Multiple medications are available to control seizures, and athetosis can be treated using medications such as trihexyphenidyl HCl (Artane) and benztropine (Cogentin).

Surgery

Fixed contractures are usually treated with either serial casting or surgery. The most commonly used surgical procedures are tenotomy, tendon transfer, and dorsal rhizotomy. In tenotomy, tendons of the affected muscle are cut and the limb is cast in a more normal position while the tendon regrows. Alternatively, tendon transfer involves cutting and reattaching a tendon at a different point on the bone to enhance the length and function of the muscle. A neurosurgeon performing dorsal rhizotomy carefully cuts selected nerve roots in the spinal cord to prevent them from stimulating the spastic muscles. Neurosurgical techniques in the brain such as implanting tiny electrodes directly into the cerebellum, or cutting a portion of the hypothalamus, have very specific uses and have had mixed results.

Education

Parents of a child newly diagnosed with CP are not likely to have the necessary expertise to coordinate the full range of care their child will need. Although knowledgeable and caring medical professionals are indispensable for developing a care plan, a potentially more important source of information and advice is other parents who have dealt with the same set of difficulties. Support groups for parents of children with CP can be significant sources of both practical advice and emotional support. Many cities have support groups that can be located through the United Cerebral Palsy Association, and most large medical centers have special multidisciplinary clinics for children with developmental disorders.

Prognosis

Cerebral palsy can affect every stage of maturation, from childhood through adolescence to adulthood. At each stage, those with CP, along with their caregivers, must strive to achieve and maintain the fullest range of experiences and education consistent with their abilities. The advice and intervention of various professionals remains crucial for many people with CP. Although CP itself is not considered a terminal disorder, it can affect a person's lifespan by increasing the risk for certain medical problems. People with mild cerebral palsy may have near-normal lifespans, but the lifespan of those with more severe forms may be shortened. However, more than 90% of infants with CP survive into adulthood.

The cause of most cases of CP remains unknown, but it has become clear in recent years that birth difficulties are not to blame in most cases. Rather, developmental problems before birth, usually unknown and generally undiagnosable, are responsible for most cases. The rate of survival for preterm infants has leveled off in recent years, and methods to improve the long-term health of these at-risk babies are now being sought. Current research is also focusing on the possible benefits of recognizing and treating coagulopathies and inflammatory disorders in the prenatal and perinatal periods. The use of magnesium sulfate in pregnant women with preeclampsia or threatened preterm delivery may reduce the risk of CP in very preterm infants. Finally, the risk of CP can be decreased through good maternal nutrition, avoidance of drugs and alcohol during pregnancy, and prevention or prompt treatment of infections.

KEY TERMS

Asphyxia Lack of oxygen. In the case of cerebral palsy, lack of oxygen to the brain.

Ataxia A deficiency of muscular coordination, especially when voluntary movements are attempted, such as grasping or walking.

Athetosis A condition marked by slow, writhing, involuntary muscle movements.

Coagulopathy A disorder in which blood is either too slow or too quick to coagulate (clot).

Contracture A tightening of muscles that prevents normal movement of the associated limb or other body part.

Cytokine A protein associated with inflammation that, at high levels, may be toxic to nerve cells in the developing brain.

Diplegia Paralysis affecting like parts on both sides the body, such as both arms or both legs.

Dorsal rhizotomy A surgical procedure that cuts nerve roots to reduce spasticity in affected muscles.

Dyskinesia Impaired ability to make voluntary movements.

Hemiplegia Paralysis of one side of the body.

Hypotonia Reduced or diminished muscle tone.

Quadriplegia Paralysis of all four limbs.

Serial casting A series of casts designed to gradually move a limb into a more functional position.

Spastic A condition in which the muscles are rigid, posture may be abnormal, and fine motor control is impaired.

Spasticity Increased mucle tone, or stiffness, which leads to uncontrolled, awkward movements.

Static encephalopathy A disease of the brain that does not get better or worse.

Tenotomy A surgical procedure that cuts the tendon of a contracted muscle to allow lengthening.

Resources

BOOKS

Peacock, Judith. Cerebral Palsy. Mankato, MN: Capstone Press, 2000.

Pimm, Paul. Living With Cerebral Palsy. Austin, TX: Raintree Steck-Vaughn Publishers, 2000.

Pincus, Dion. Everything You Need to Know About Cerebral Palsy. New York: Rosen Publishing Group, Inc., 2000.

PERIODICALS

Chambers, Henry G. "Research in Cerebral Palsy." The Exceptional Parent 29 (July 1999): 50.

"Experimental Treatment Yields New Hope for Children Battling Cerebral Palsy." Drug Week February 27, 2004: 82.

Mohan, P.V., et al. "Can Polyclonal Intravenous Immunoglobulin Limit Cytokine Mediated Cerebral Damage and Chronic Lung Disease in Preterm Infants?" Archives of Disease in Childhood January 2004: pF5-pF9.

Myers, Scott M., and Bruce K. Shapiro. "Origins and Causes of Cerebral Palsy: Symptoms and Diagnosis." The Exceptional Parent 29 (April 1999): 28.

ORGANIZATIONS

Epilepsy Foundation of America. 4351 Garden City Dr., Suite 406, Landover, MD 20785-2267. (301) 459-3700 or (800) 332-1000. http://www.epilepsyfoundation.org.

March of Dimes Birth Defects Foundation. 1275 Mamaroneck Ave., White Plains, NY 10605. (888) 663-4637. resourcecenter@modimes.org. http://www.modimes.org.

National Easter Seal Society. 230 W. Monroe St., Suite 1800, Chicago, IL 60606-4802. (312) 726-6200 or (800) 221-6827. http://www.easter-seals.org.

National Institute of Neurological Disorders and Stroke. 31 Center Drive, MSC 2540, Bldg. 31, Room 8806, Bethesda, MD 20814. (301) 496-5751 or (800) 352-9424. http://www.ninds.nih.gov.

National Society of Genetic Counselors. 233 Canterbury Dr., Wallingford, PA 19086-6617. (610) 872-1192. http://www.nsgc.org/GeneticCounselingYou.asp.

United Cerebral Palsy Association, Inc. (UCP). 1660 L St. NW, Suite 700, Washington, DC 20036-5602. (202) 776-0406 or (800) 872-5827. http://www.ucpa.org.

OTHER

"Cerebral Palsy: Hope Through Research." National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/health_and_medical/pubs/cerebral_palsyhtr.htm.

"Cerebral Palsy Information Page." National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/health_and_medical/pubs/cerebral_palsy.htm.

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Cerebral Palsy

Cerebral palsy

Definition

Cerebral palsy (CP) is the term used for a group of nonprogressive disorders of movement and posture caused by abnormal development of, or damage to, motor control centers of the brain. CP is caused by events before, during, or after birth. The abnormalities of muscle control that define CP are often accompanied by other neurological and physical abnormalities.

Description

Voluntary movement (for example, walking, grasping, chewing) is primarily accomplished using skeletal muscles (muscles attached to bones). Control of the skeletal muscles originates in the cerebral cortex, the largest portion of the brain. Palsy means paralysis but may also be used to describe uncontrolled muscle movement. Therefore, cerebral palsy encompasses any disorder of abnormal movement and paralysis caused by abnormal function of the cerebral cortex. CP does not include conditions due to progressive disease or degeneration of the brain. For this reason, CP is also referred to as static (nonprogressive) encephalopathy (disease of the brain). Also excluded from CP are any disorders of muscle control that arise in the muscles themselves and/or in the peripheral nervous system (nerves outside the brain and spinal cord). CP is not a specific diagnosis but is more accurately considered a description of a broad but defined group of neurological and physical problems.v

Because CP is not one disorder, it is difficult to classify. It has been divided into four general types: spastic, athetoid, ataxic, and mixed. Another general categorization describes spastic, dyskinetic, and ataxic CP as follows:

  • Spastic refers to diplegic impairment of either legs or arms, quadriplegic involving all four extremities, hemiplegic or one-sided involvement of arms and legs, or double hemiplegic impairment of both sides, arms and legs. Spasticity means having an increased stretch reflex.
  • Dyskinetic refers to abnormal movements caused by inadequate regulation of muscle tone and coordination. The category includes athetoid or choreoathetoid CP; both are hyperkinetic forms of the disease.
  • Ataxic refers to disturbances in coordination of voluntary movements; it includes mixed forms of CP, with mixed characteristics and symptoms.

Muscles that receive defective messages from the brain may be constantly contracted and tight (spastic), exhibit involuntary writhing movements (athetosis), or have difficulty with voluntary movement (dyskinesia). A lack of balance and coordination with unsteady movements (ataxia) may also be present. Spastic CP and mixed CP constitute the majority of cases. Effects on the muscles can range from mild weakness or partial paralysis (paresis) to complete loss of voluntary control of a muscle or group of muscles (plegia). CP is also designated by the number of limbs affected. For instance, affected muscles in one limb is monoplegia, both arms or both legs is diplegia, both limbs on one side of the body is hemiplegia, and in all four limbs is quadriplegia. Muscles of the trunk, neck, and head may be affected.

About 50 percent of all cases of CP diagnosed are in children who are born prematurely. Advances in the medical care of premature infants since the 1980s have dramatically increased the rate of survival of these fragile newborns. However, as gestational age at delivery and birth weight of a baby decrease, the risk for CP dramatically increases. A term pregnancy is delivered at 3741 weeks gestation. The risk for CP in a preterm infant (3237 weeks) is increased about five-fold over the risk for an infant born at term. Survivors of extremely preterm births (less than 28 weeks) face as much as a 50-fold increase in risk.

Two factors are involved in the risk for CP associated with prematurity . First, premature babies are at higher risk for various CP-associated medical complications, such as intracerebral hemorrhage, infection, and difficulty in breathing, to name a few. Second, the onset of premature labor may be induced, in part, by complications that have already caused neurologic damage in the fetus. A combination of both factors may play a role in some cases of CP. The tendency toward premature delivery runs in families, but genetic mechanisms are not fully clear.

An increase in multiple births in the early 2000s, especially in the United States, is associated with the increased use of fertility drugs. As the number of fetuses in a pregnancy increases, the risks for abnormal development and premature delivery also increase. Twins , for example, have four times the risk of developing CP as children from singleton pregnancies, owing to the fact that more twin pregnancies are delivered prematurely. The risk for CP in one of triplets is up to 18 times greater. Furthermore, evidence suggests that a baby from a pregnancy in which its twin died before birth is at increased risk for CP.

Although CP is the leading cause of disability in children, its incidence in the United States did not changed much between the 1980s and the early 2000s. Advances in medicine have decreased the incidence from some causes. Rh disease, for example, has been controlled by the advent of anti-Rh globulin; its administration to Rh-negative mothers has reduced one risk factor for CP. The risk has still increased from other causes, however, notably prematurity and multiple-birth pregnancies. The cause of most cases of CP remains unknown, but it has become clear in the early 2000s that birth difficulties are not to blame in most cases. Rather, developmental problems before birth, usually unknown and generally undiagnosable, are largely responsible. The rate of survival for preterm infants has leveled off in the early 2000s, and methods to improve the long-term health of these at-risk babies are being sought.

Demographics

Approximately 500,000 children and adults in the United States have CP, and it is newly diagnosed in about 6,000 infants and young children each year, representing about two to three children in 1,000 live births. No particular ethnic group seems to be at higher risk for CP. However, some low income families may be at higher risk due to poorer access to proper prenatal care and advanced medical services.

Causes and symptoms

CP can be caused by a number of different mechanisms at various times of life, ranging from several weeks after conception, through birth, to early childhood. In the twentieth century, it was accepted that most cases of CP were due to brain injuries received during a traumatic birth, a condition known as birth asphyxia. However, extensive research in the 1980s showed that only 5 to 10 percent of CP can be attributed to birth trauma. Other possible causes include abnormal development of the brain, prenatal factors that directly or indirectly damage neurons in the developing brain, premature birth, and brain injuries that occur in the first few years of life.

The causes of CP could be grouped into those that are genetic and those that are non-genetic, although most would fall somewhere in between. Grouping causes into those that occur during pregnancy (prenatal), those that happen around the time of birth (perinatal), and those that occur after birth (postnatal), is preferable. CP related to premature birth and multiple births is somewhat different and considered separately.

Prenatal causes

Although much was learned about human embryology in the last couple of decades of the twentieth century, a great deal remains unknown in the early 2000s. Studying prenatal human development is difficult because the embryo and fetus develop in a closed environmentthe mother's womb. However, the development of a number of prenatal tests has opened a window on the process. Add to that more accurate and complete evaluations of newborns, especially those with problems, and a clearer picture of what can go wrong before birth is possible.

The complicated process of brain development before birth is susceptible to many chance errors that can result in abnormalities of varying degrees. Some of these errors will result in structural anomalies of the brain, while others may cause undetectable, but significant, abnormalities in how the cerebral cortex is wired. An abnormality in structure or wiring is sometimes hereditary but is most often due to chance or some unknown cause. The possible role genetics plays in a particular brain abnormality depends to some degree on the type of anomaly and the form of CP it causes.

Several maternal-fetal infections are known to increase the risk for CP, including rubella (German measles , now rare in the United States), cytomegalovirus (CMV), and toxoplasmosis . Each of these infections is considered a risk to the fetus only if the mother contracts it for the first time during that pregnancy. Even in those cases, most babies are born normal. Most women are immune to all three infections by the time they reach childbearing age, but a woman's immune status can be determined using the so-called TORCH (for toxoplasmosis, rubella, cytomegalovirus, and herpes) test before or during pregnancy.

Just as a stroke can occur in an adult and cause neurologic damage in an adult, so too can this type of event occur in the fetus. A burst blood vessel in the brain followed by uncontrolled bleeding (intracerebral hemorrhage) can cause a fetal stroke, or a clot (embolism) can obstruct a cerebral blood vessel. Infants who later develop CP, along with their mothers, are more likely than other mother-infant pairs to have coagulation disorders (coagulopathies) that put them at increased risk for bleeding episodes or blood clots. Certain coagulation disorders are inherited while others may be deficiencies in essential clotting factors or defects in the coagulation process.

Any substance that might affect fetal brain development, directly or indirectly, can increase the risk for CP. Likewise, any substance that increases the risk for premature delivery and low birth weight, such as alcohol, tobacco, or cocaine, among others, might indirectly increase the risk for CP. Links between a drug or other chemical exposure during pregnancy and a risk for CP are difficult to prove.

Because the fetus receives all nutrients and oxygen from blood that circulates through the placenta, anything that interferes with normal placental function might adversely affect development of the fetus, including the brain, or might increase the risk for premature delivery. Structural abnormalities of the placenta, premature detachment of the placenta from the uterine wall (abruption), and placental infections (chorioamnionitis) are thought to pose some risk for CP.

Certain conditions in the mother during pregnancy might pose a risk to fetal development leading to CP. Women with autoimmune anti-thyroid or anti-phospholipid (APA) antibodies are at slightly increased risk for CP in their children. A potentially important clue points toward high levels of cytokines in the maternal and fetal circulation as a possible risk for CP. Cytokines are proteins associated with inflammation, such as from infection or autoimmune disorders, and they may be toxic to neurons in the fetal brain.

Serious physical trauma to the mother during pregnancy could result in direct trauma to the fetus as well, or injuries to the mother could compromise the availability of nutrients and oxygen to the developing fetal brain.

Perinatal causes

Birth asphyxia that is significant enough to result in CP is uncommon in developed countries. An umbilical cord around the baby's neck (tight nuchal cord) and the cord delivered before the baby (prolapsed cord) are possible causes of birth asphyxia, as are bleeding and other complications associated with placental abruption and placenta previa (placenta lying over the cervix).

Infection in the mother is sometimes not passed to the fetus through the placenta but is transmitted to the baby during delivery. Any such infection, such as herpes, that results in serious illness in the newborn has the potential to produce some neurological damage.

Postnatal causes

The remaining 15 percent of CP cases are due to neurologic injury sustained after birth. CP that has a postnatal cause is sometimes referred to as acquired CP, but this is only accurate for those cases caused by infection or trauma.

Incompatibility between the Rh blood types of mother and child (mother Rh negative, baby Rh positive) can result in severe anemia in the baby (erythroblastosis fetalis ). This may lead to other complications, including severe jaundice , which can cause CP. Rh disease in the newborn is rare in developed countries due to routine screening of maternal blood type and routine prevention of anti-Rh antibodies in Rh negative women after each birth of an Rh positive infant. The routine, effective treatment of jaundice due to other causes has also made it an infrequent cause of CP in developed countries.

Serious infections that affect the brain directly, such as meningitis and encephalitis , may cause irreversible damage to the brain, leading to CP. A seizure disorder early in life may cause CP or may be the product of a hidden problem that causes CP in addition to seizures. Unexplained (idiopathic) seizures are hereditary in only a small percentage of cases. Although rare in healthy infants born at or near term, intracerebral hemorrhage and brain embolism, like fetal stroke, are sometimes genetic.

Physical trauma to an infant or child resulting in brain injury, such as from abuse, accidents, or near drowning/suffocation, might cause CP. Likewise, ingestion of a toxic substance such as lead, mercury, other poisons, or certain chemicals could cause neurological damage. Accidental overdose of certain medications might also cause similar damage to the central nervous system.

Symptoms

The symptoms of CP and their severity are variable. Those who have CP may have only minor difficulty with fine motor skills , such as grasping and manipulating items with their hands. A severe form of CP could involve significant muscle problems in all four limbs, mental retardation , seizures, and difficulties with vision, speech, and hearing.

Although the defect in cerebral function that causes CP is not progressive, the symptoms of CP often change over time. Most of the symptoms relate in some way to the aberrant control of muscles. CP is categorized first by the type of movement/postural disturbance(s) present, rather than by a description of which limbs are affected. The severity of motor impairment is also a factor. Spastic diplegia, for example, refers to continuously tight muscles that have no voluntary control in both legs, while athetoid quadraparesis describes uncontrolled writhing movements and muscle weakness in all four limbs. These may describe CP symptoms generally but do not describe all people with CP. Spastic diplegia is seen in more individuals than is athetoid quadraparesis. CP can also be loosely categorized as mild, moderate, or severe, but these are subjective terms.

A muscle that is tensed and contracted is hypertonic, while excessively loose muscles are hypotonic. Spastic, hypertonic muscles can cause serious orthopedic problems, including curvature of the spine (scoliosis ), hip dislocation, or contractures. A contracture is shortening of a muscle, aided sometimes by a weak-opposing force from a neighboring muscle. Contractures may become permanent, i.e., fixed, without some sort of intervention. Fixed contractures may cause postural abnormalities in the affected limbs. Clenched fists and contracted feet (equinus or equinovarus) are common in people with CP. Spasticity in the thighs causes them to turn in and cross at the knees, resulting in an unusual method of walking known as scissors gait. Any of the joints in the limbs may be stiff (immobilized) due to spasticity of the attached muscles.

Athetosis and dyskinesia often occur with spasticity but do not often occur alone. The same is true of ataxia. It is important to remember that mild CP or severe CP refers not only to the number of symptoms present but also to the level of involvement of any particular class of symptoms.

Other neurologically based symptoms may include the following:

  • mental retardation/learning disabilities
  • behavioral disorders
  • seizure disorders
  • visual impairment
  • hearing loss
  • speech impairment (dysarthria)
  • abnormal sensation and perception

These problems may have a greater impact on a child's life than the physical impairments of CP, although not all children with CP are affected by other problems. Many infants and children with CP have growth impairment. About one third of individuals with CP have moderate-to-severe mental retardation, one third have mild mental retardation, and one third have normal intelligence .

When to call the doctor

Parents should seek medical advice when they notice what seems to be slow development in movement, speech, or cognitive ability in their young child. If a child does not acquire certain skills within a normal time frame, there may be some cause for concern. However, it is known that children progress at somewhat different rates, and a slow beginning is often followed by normal development.

Normal developmental milestones with typical ages for acquiring them, include the following:

  • sits well unsupported at about six months (eight to ten months)
  • babbles at about six months (up to eight months)
  • crawls at about nine months (up to 12 months)
  • finger feeds, holds bottle at about nine months (up to 12 months)
  • walks alone at about 12 months (up to 1518 months)
  • uses one or two words other than dada/mama at about 12 months (up to 15 months)
  • walks up and down steps at about 24 months (24 to 36 months)
  • turns pages in books and removes shoes and socks at about 24 months (to 30 months)

Children do not consistently favor one hand over the other before 12 to 18 months of age, and doing so may be a sign that the child has difficulty using the other hand. This same preference for one side of the body may show up as asymmetric crawling or, later on, favoring one leg while climbing stairs. Because CP is nonprogressive, continued loss of previously acquired milestones may indicate that CP is not the cause of the problem; medical evaluation is needed to determine the cause.

Diagnosis

The signs of CP are not usually noticeable at birth. Children normally progress through a predictable set of developmental milestones through the first 18 months of life. Children with CP, however, tend to develop these skills more slowly because of their motor impairments, and delays in reaching milestones are usually the first symptoms of CP. Babies with more severe cases of CP are usually diagnosed earlier than others.

No one test is diagnostic for CP, but certain factors increase suspicion. The Apgar score measures a baby's condition immediately after birth. Babies who have low Apgar scores are at increased risk for CP. Presence of abnormal muscle tone or movements may indicate CP, as may the persistence of infantile reflexes. Imaging of the brain using ultrasound, x rays , MRI, and/or CT scans may reveal a structural anomaly. Some brain lesions associated with CP include scarring, cysts, expansion of the cerebral ventricles (hydrocephalus ), abnormality of the area surrounding the ventricles (periventricular leukomalacia), areas of dead tissue (necrosis), and evidence of an intracerebral hemorrhage or blood clot. Blood and urine biochemical tests, as well as genetic tests, may be used to rule out other possible causes, including muscle and peripheral nerve diseases, mitochondrial and metabolic diseases, and other inherited disorders. Evaluations by a pediatric developmental specialist and a geneticist may be of benefit.

Treatment

Cerebral palsy cannot be cured, but many of the disabilities it causes can be managed through planning and timely care. Treatment for a child with CP depends on the severity, nature, and location of the primary muscular symptoms, as well as any associated problems that might be present. Optimal care of a child with mild CP may involve regular interaction with only a physical therapist and occupational therapist, whereas care for a more severely affected child may include visits to multiple medical specialists throughout life. With proper treatment and an effective plan, most people with CP can lead productive, happy lives.

Physical, occupational, and speech therapy

Spasticity, muscle weakness, coordination, ataxia, and scoliosis are all significant impairments that affect the posture and mobility of children and adults with CP. Physical and occupational therapists work with the patient and the family to maximize the patient's ability to move affected limbs, develop normal motor patterns, and maintain posture. Assistive technology, including wheelchairs, walkers, shoe inserts, crutches, and braces, are often required. A speech therapist and high-tech aids such as computer-controlled communication devices can make a tremendous difference in the life of those who have speech impairments.

Drug therapy

Before fixed contractures develop, muscle-relaxant drugs such as diazepam (Valium), dantrolene (Dantrium), and baclofen (Lioresal) may be prescribed. Botulinum toxin (Botox), a highly effective treatment, is injected directly into the affected muscles. Alcohol or phenol injections into the nerve controlling the muscle are another option. Multiple medications are available to control seizures, and athetosis can be treated using medications such as trihexyphenidyl HCl (Artane) and benztropine (Cogentin).

Surgery

Fixed contractures are usually treated with either serial casting or surgery. The most commonly used surgical procedures are tenotomy, tendon transfer, and dorsal rhizotomy. In tenotomy, tendons of the affected muscle are cut, and the limb is cast in a more normal position while the tendon regrows. Alternatively, tendon transfer involves cutting and reattaching a tendon at a different point on the bone to enhance the length and function of the muscle. A neurosurgeon performing dorsal rhizotomy carefully cuts selected nerve roots in the spinal cord to prevent them from stimulating the spastic muscles. Neurosurgical techniques in the brain such as implanting tiny electrodes directly into the cerebellum or cutting a portion of the hypothalamus have very specific uses and have had mixed results.

KEY TERMS

Asphyxia Lack of oxygen.

Ataxia A condition marked by impaired muscular coordination, most frequently resulting from disorders in the brain or spinal cord.

Athetosis A condition marked by slow, writhing, involuntary muscle movements.

Cerebral palsy A nonprogressive movement disability caused by abnormal development of or damage to motor control centers of the brain.

Coagulopathy A disorder in which blood is either too slow or too quick to coagulate (clot).

Contracture A tightening or shortening of muscles that prevents normal movement of the associated limb or other body part.

Cytokines Chemicals made by the cells that act on other cells to stimulate or inhibit their function. They are important controllers of immune functions.

Diplegia Paralysis affecting like parts on both sides the body, such as both arms or both legs.

Dorsal rhizotomy A surgical procedure that cuts nerve roots to reduce spasticity in affected muscles.

Dyskinesia Impaired ability to make voluntary movements.

Hemiplegia Paralysis of one side of the body.

Hypotonia Having reduced or diminished muscle tone or strength.

Quadriplegia Paralysis of all four limbs and the trunk below the level of an associated injury to the spinal cord. Also called tetraplegia.

Serial casting A series of casts designed to gradually move a limb into a more functional position.

Spastic Refers to a condition in which the muscles are rigid, posture may be abnormal, and fine motor control is impaired.

Spasticity Increased mucle tone, or stiffness, which leads to uncontrolled, awkward movements.

Static encephalopathy A disease or disorder of the brain that does not get better or worse.

Tenotomy A surgical procedure that cuts the tendon of a contracted muscle to allow lengthening.

Prognosis

Cerebral palsy can affect every stage of maturation, from childhood through adolescence to adulthood. At each stage, those with CP, along with their caregivers, must strive to achieve and maintain the fullest range of experience and education consistent with their abilities. The advice and intervention of various professionals are crucial for many people with CP. Although CP itself is not considered a terminal disorder, it can affect a person's lifespan by increasing the risk for certain medical problems. People with mild cerebral palsy may have near-normal lifespan, but the lifespan of those with more severe forms may be shortened. However, over 90 percent of infants with CP survive into adulthood.

Prevention

Research in the early 2000s is focused on the possible benefits of recognizing and treating coagulopathies and inflammatory disorders in the prenatal and perinatal periods in order to reduce the incidence of CP and other congenital diseases. The use of magnesium sulfate in pregnant women with preeclampsia or threatened preterm delivery may reduce the risk of CP in very preterm infants. Finally, the risk of CP can be decreased through good maternal nutrition , avoidance of drugs and alcohol during pregnancy, and prevention or prompt treatment of infections.

Parental concerns

Parents of a child diagnosed with CP may not feel that they have the necessary expertise to coordinate the full range of care their child needs. Although knowledgeable and caring medical professionals are indispensable for developing a care plan, a potentially more important source of information and advice can be gained from other parents who have dealt with the same set of difficulties. Support groups for parents of children with CP can be significant sources of both practical advice and emotional support. Many cities have support groups that can be located through the United Cerebral Palsy Association, and most large medical centers have special multidisciplinary clinics for children with developmental disorders.

See also Febrile seizures; TORCH test; Seizure disorder.

Resources

BOOKS

Peacock, Judith. Cerebral Palsy. Mankato, MN: Capstone Press, 2000.

Pimm, Paul. Living with Cerebral Palsy. Austin, TX: Raintree Steck-Vaughn Publishers, 2000.

Pincus, Dion. Everything You Need to Know about Cerebral Palsy. New York: Rosen Publishing Group Inc., 2000.

ORGANIZATIONS

National Institute of Neurological Disorders and Stroke. 31 Center Drive, MSC 2540, Bldg. 31, Room 8806, Bethesda, MD 20814. Web site: <www.ninds.nih.gov>.

National Society of Genetic Counselors. 233 Canterbury Dr., Wallingford, PA 190866617. (610) 8721192. http://www.nsgc.org/GeneticCounselingYou.asp.

United Cerebral Palsy Association Inc. (UCP). 1660 L St. NW, Suite 700, Washington, DC 200365602. Web site: <www.ucpa.org>.

WEB SITES

"Cerebral Palsy: Hope Through Research." National Institute of Neurological Disorders and Stroke, 2004. Available online at <www.ninds.nih.gov/health_and_medical/pubs/cerebral_palsyhtr.htm> (accessed November 29, 2004).

"NINDS Cerebral Palsy Information Page." National Institute of Neurological Disorders and Stroke, October 2004. Available online at <www.ninds.nih.gov/disorders/cerebral_palsy/cerebral_palsy.htm> (accessed November 29, 2004).

L. Lee Culvert Scott J. Polzin, MS

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Culvert, L.; Polzin, Scott. "Cerebral Palsy." Gale Encyclopedia of Children's Health: Infancy through Adolescence. 2006. Encyclopedia.com. 25 Jun. 2016 <http://www.encyclopedia.com>.

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Culvert, L.; Polzin, Scott. "Cerebral Palsy." Gale Encyclopedia of Children's Health: Infancy through Adolescence. 2006. Retrieved June 25, 2016 from Encyclopedia.com: http://www.encyclopedia.com/doc/1G2-3447200114.html

Cerebral Palsy

Cerebral palsy

Definition

Cerebral palsy (CP), or static encephalopathy, is the name for a collection of movement disorders caused by brain damage that occurs before, during, or shortly after birth. A person with CP is often also affected by other conditions caused by brain damage.

Description

The affected muscles of a person with CP may become rigid or excessively loose. The person may lose control of muscles, or have problems with balance and coordination. A combination of these is also possible. Those with CP may be primarily affected in the legs (paraplegia or diplegia), or in the arm and leg of one side of the body (hemiplegia), or all four limbs may be involved (quadriplegia).

A person with CP may also be affected by a number of other problems, including a seizure disorder, visual deficits, hearing problems, mental retardation, learning disabilities, and attention-deficit hyperactivity disorder . None of these is necessarily part of CP, however, they may accompany the disorder.

CP affects approximately 500,000 children and adults in the United States, and is diagnosed in more than 6,000 newborns and young children each year. It is not an inherited disorder, and as of yet there is no way to predict with certainty which children will develop CP. It is not a disease, and is not communicable. CP is a nonprogressive disorder, which means that symptoms neither worsen nor improve over time. However manifestation of the symptoms may become more severe over time. For example, rigidity of muscles can lead to contractures and deformities that require a variety of interventions.

Causes & symptoms

Causes

Cerebral palsy is caused by damage to the motor control centers of the brain. When the nerve cells (neurons) in these regions die, the appropriate signals can no longer be sent to the muscles under their control. The resulting poor control of these muscles causes the symptoms of CP.

The brain damage leading to CP may be caused by lack of oxygen (asphyxia), infection, trauma, malnutrition, drugs or other chemicals, or hemorrhage. In most cases it is impossible to determine the actual cause, although premature birth is recognized as a significant risk factor. It was once thought that difficult or prolonged delivery was responsible for many cases of CP, but most researchers now believe that the great majority of cases result from brain damage occurring before birth. The same injury that damages the motor areas can harm other areas as well, leading to problems commonly associated with CP.

If brain cells do not get enough oxygen because of poor circulation, they may die. Defects in circulation in the developing brain may cause CP in some cases. Asphyxia during birth is also possible, and about half of newborns known to have suffered asphyxia during birth (perinatal asphyxia) develop CP. However, asphyxia during birth is usually considered a symptom of an underlying neurological problem in a newborn, rather than its cause, and the resulting CP may be another sign of that problem. Asphyxia after birth can be caused by choking, poisoning (such as from carbon monoxide or barbiturates), or near-drowning.

The fetal brain may be damaged by an infection contracted by the mother. Infections correlated with CP include rubella (German measles ), toxoplasmosis (often contracted from cat feces), cytomegalovirus (a herpes virus), and HIV (the virus that causes AIDS ). Encephalitis and meningitis , infections of the brain and its coverings, can also cause CP when contracted by infants.

Physical trauma to the pregnant mother or infant may cause brain damage. Blows to the infant's head, as from a motor vehicle accident, violent shaking, or other physical abuse can damage the infant's brain. Maternal malnutrition may cause brain damage, as can the use of drugs, including cocaine or alcohol. Although these factors may cause CP, they may be more likely to cause mental retardation or other impairments.

Incompatibility between the Rh blood types of mother and child was once a major cause of athetoid CP, one type of movement impairment seen in cerebral palsy. In some cases, this incompatibility can cause the mother's defense (immune) system to attack and destroy the child's blood cells during pregnancy , a condition called erythroblastosis fetalis. High levels of a blood cell breakdown product called bilirubin in a child's circulation, leading to yellowish pigmentation of the skin caused by bile (jaundice ) can result in brain damage. This condition is now rare because of testing procedures that identify potential Rh incompatibility, and treatment that prevents the mother's immune system from attacking the child's blood cells. Jaundice that does occur can be treated with special lights that help the breakdown of bilirubin. Blood transfusions for the child are also possible in extreme cases. Despite the virtual elimination of this cause of CP in the last few decades, CP rates have not declined, largely because of the increase of survival of premature babies.

Prematurity is one of the most significant risk factors for CP. About 7% of babies weighing less than three pounds at birth develop CP, and the risk increases dramatically as weight falls. Prematurity may increase the risk of CP because of the increased likelihood of hemorrhaging in the brain associated with low birth weight. Brain hemorrhage is most common in babies weighing less than four pounds at birth, and the risk increases as weight decreases. The hemorrhage may destroy brain tissue, either through asphyxia or release of toxic breakdown products.

Researchers in Sweden reported in 2002 that babies conceived through in vitro fertilization (IVF) were 3.7 times more likely to have CP than babies conceived naturally. Some of the reason can be attributed to a higher rate of twins, low birthweight, and premature births associated with IVF babies, but some single births also have higher rates of CP.

Symptoms

The symptoms of CP are usually not noticeable at birth. As children develop in the first 18 months of life, however, they progress through a predictable set of developmental milestones. Children with CP will develop these skills more slowly because of their motor impairments, and delay in reaching milestones is usually the first symptom of CP. The more severe the CP, the earlier the diagnosis is usually made.

Selected developmental milestones, and the ages at which a child will normally acquire them, are given below. There is some cause for concern if the child does not acquire the skill by the age shown in parentheses:

  • sits well unsupported, 6 months (8-10 months)
  • babbles, 6 months (8 months)
  • crawls, 9 months (12 months)
  • finger feeds, holds bottle, 9 months (12 months)
  • walks alone, 12 months (15-18 months)
  • uses one or two words other than dada/mama, 12 months (15 months)
  • walks up and down steps, 24 months (24-36 months)
  • turns pages in books, removes shoes and socks, 24 months (30 months)

Children do not consistently favor one hand over the other before 18 months, and doing so may be a sign that the child has difficulty using the other hand. This same preference for one side of the body may show up as an asymmetric crawling effort, or continuing to use only one leg for the work of stair climbing after age three.

It must be remembered that children normally progress at somewhat different rates, and slow initial accomplishment is often followed by normal development. There are also other causes for delay in reaching some milestones, including problems with vision or hearing. Because CP is a non-progressive disease, loss of previously acquired milestones indicates that CP is not the cause of the problem.

The impairments of CP become recognizable in early childhood. The type of motor impairment and its location are used as the basis for classification. There are five generally recognized types of impairment:

  • Spastic. Muscles are rigid, posture may be abnormal, and fine motor control is impaired.
  • Athetoid. It is marked by slow, writhing, involuntary movements.
  • Hypotonic. Muscles are floppy, without tone.
  • Ataxic. Balance and coordination are impaired.
  • Dystonic. Impairment is mixed.

The location of the impairment usually falls into one of three broad categories:

  • Hemiplegia. One arm and one leg on the same side of the body are involved
  • Diplegia. Both legs; arms may be partially involved.
  • Quadriplegia. All four extremities are involved.

A person with CP may be said to have spastic diplegia, or ataxic hemiplegia, for instance. CP is also termed mild, moderate, or severe, although these are subjective categories with no firm boundaries.

Loss of muscle control, especially of the spastic type, can cause serious orthopedic problems, including scoliosis (spine curvature), hip dislocation, or contractures. Contracture is shortening of a muscle, caused by an imbalance of opposing force from a neighboring muscle. Contractures begin as prolonged contractions, but can become fixed or irreversible without regular range of motion exercises. A fixed contracture occurs when the contracted muscle adapts by reducing its overall length. Fixed contractures may cause postural abnormalities in the affected limbs, including clenched fists, tightly pressed or crossed thighs, or equinus. In equinus, the most common postural deformity, the foot is extended by the strong pull of the rear calf muscles, causing the toes to point. The foot is commonly pulled inward as well, a condition called equinovarus. Contractures of all kinds may be painful, and may interfere with normal activities of daily living, including hygiene and mobility.

As noted, the brain damage that causes CP may also cause a large number of other disorders. These may include:

  • mental retardation
  • learning disabilities
  • attention-deficit hyperactivity disorder
  • seizure disorder
  • visual impairment, especially strabismus ("cross-eye")
  • hearing loss
  • speech impairment

These problems may have an even greater impact on the child's life than the physical impairment of CP, although not all children with CP are affected by other problems. About one-third of children with CP have moderate to severe mental retardation, one-third have mild mental retardation, and one-third have normal to above average intelligence.

Diagnosis

The tracking of developmental progress is the most important test the physician has in determining whether a child has cerebral palsy. Most children with CP can be confidently diagnosed by 18 months. However, diagnosing CP is not always easy, since variations in child development may account for delays in achieving milestones, and since even children who are obviously delayed may continue to progress through the various developmental stages, attaining a normal range of skills later on. Serious or prolonged childhood illness may cause delays that are eventually caught up.

Evidence of other risk factors may aid the diagnosis. The Apgar score, evaluated immediately after birth, measures a newborn's heart rate, cry, color, muscle tone, and motor reactions. Apgar scores of less than three out of a possible 10 are associated with a highly increased indication of CP. Presence of abnormal muscle tone or movements may signal CP, as may the persistence of infantile reflexes. A child with seizures or congenital organ malformation has an increased likelihood of CP. Ultra-sound examination, a diagnostic technique that creates a two-dimensional image of internal body structures, may help to identify brain abnormalities, such as enlarged ventricles (chambers containing fluid) or periventricular leukomalacia (an abnormality of the area surrounding the ventricles), which may be associated with CP.

X rays, magnetic resonance imaging (MRI) studies, and computed tomography (CT) scans are often used to look for scarring, cysts, expansion of the cerebral ventricles (hydrocephalus), or other brain abnormalities that may indicate the cause of symptoms. Blood tests and genetic tests may be used to rule out other possible causes, including muscular dystrophy (a disease characterized by the progressive wasting of muscles), mitochondrial (cellular) disease, and other inherited disorders or infections.

Treatment

A number of people with cerebral palsy, both children and adults, have found systematic relief and enhanced quality of life from a combination of alternative and complementary treatments, including nutritional therapy, craniosacral therapy , bodywork, herbal therapy, homeopathy , and acupuncture .

General recommendations

Pregnant women should avoid cleaning cat litter, which may contain toxoplasma parasite. This organism causes severe brain damage or death in the unborn fetus. Unprotected sex increases risk of contracting sexually transmitted diseases such as genital herpes , which can infect the unborn child. Women should also vaccinate before getting pregnant to prevent measles and rubella, which can cause severe brain damage to the fetus. They should avoid taking certain drugs, smoking , or drinking alcohol. Cocaine, heroine, nicotine and alcohol are toxic to the developing brain of the fetus.

Nutritional therapy

The following dietary adjustments have been recommended to alleviate some symptoms in patients with cerebral palsy:

  • Those with CP should avoid potential allergenic foods. Allergic foods are believed to worsen symptoms in many CP patients.
  • CP patients should also avoid preservatives and food additives such as MSG (which are potentially toxic to the brain) by eating fresh and unprocessed foods such as whole grains, vegetables, beans, fruits, nuts, and seeds.
  • To improve muscle tone, CP patients should supplement their diets with magnesium, thiamine, pyridoxine, vitamin C , and bioflavonoids . Alternatively, they can take daily multivitamin/mineral supplements that can provide all these helpful nutrients and make sure they are getting adequate protein in diet or supplements.

Osteopathy

Craniosacral therapy, a special form of osteopathic treatment, may be successful in preventing cerebral palsy if performed right after a difficult labor or delivery by forceps. This manipulation of bones of the newborn's skull may prevent stress and distortion of the child's head occurring during traumatic delivery. Craniosacral therapy is less successful, however, in established cerebral palsy in an older child.

Bodywork

Bodywork such as massage, reflexology, Feldenkrais , or rolfing can help improve blood circulation and muscle tone and reduce muscle spasms in patients with cerebral palsy.

Other therapies

Other potentially helpful treatments include acupuncture, homeopathy and herbal therapy, and dance and music therapy . Although still not proven in clinical trials, hyperbaric oxygen therapy (HBOT) has been used to alleviate many symptoms of CP. It provides pure oxygen at higher-tha-normal pressure in an enclosed chamber and is more commonly known for treating divers with compression sickness. A Cornell University study in 19992000 studied the effects of 40 one-hour sessions on 23 children with moderate to severe CP. They noted improvements in motor skills, attention, language, and play.

Allopathic treatment

Cerebral palsy cannot be cured, but many of the disabilities it causes can be managed through planning and timely care. Treatment for a child with CP depends on the severity, nature, and location of the impairment, as well a child's associated problems. Optimal care of a child with mild CP may involve regular interaction with only physical and occupational therapists, whereas care for a more severely affected child may include a speech-language therapist, special education teacher, adaptive sports therapist, nutritionist, orthopedic surgeon, and neurosurgeon.

Parents of a child newly diagnosed with CP are not likely to have the necessary expertise to coordinate the full range of care their child will need. Support groups for parents of physically or mentally impaired children can be significant sources of both practical advice and emotional support. Many cities have support groups that can be located through the United Cerebral Palsy Association or a local hospital or social service agency. Children with CP are also eligible for special education services. The diagnosing doctor should refer parents to the local school district for these services. Even children aged birth to three years are eligible through early intervention programs.

Influence of CP on development

Cerebral palsy may restrict a child's ability to reach for and grasp objects, to move about, to explore the properties of toys, and to communicate with others, which are all central activities in the child's growth and development. Therefore, the disease inhibits acquisition of motor skills, knowledge of the world, and social competence. The family can do much to overcome these restrictions by adapting the child's environment to meet his or her needs and providing challenges within the child's abilities to accomplish. The advice and direction of an occupational therapist can be critical to promoting normal development of the child with CP.

Posture and mobility

Spasticity, muscle coordination, ataxia, and scoliosis are all significant impairments that affect the posture and mobility of a person with cerebral palsy. Physical therapists work with the family to maximize the child's ability to move affected limbs, to develop normal motor patterns, and to maintain posture. Adaptive equipment may be needed, including wheelchairs, walkers, shoe inserts, crutches, or braces. The need for adaptive equipment may change as the person develops, or as new treatments are introduced.

SPASTICITY. Spasticity causes muscles to shorten, joints to tighten, and postures to change. Spasticity can affect the ability to walk, use a wheelchair, and sit unaided; and it can prevent independent feeding, dressing, hygiene, or other activities of daily living. Contracture and dislocations are common consequences of spasticity.

Mild spasticity may be treated by regular stretching of the affected muscles through their full range of motion. This usually is done at least daily. Moderate spasticity may require bracing to keep a limb out of the abnormal position, or serial casting to return it to its normal position. Ankle-foot braces (orthoses) made of lightweight plastic are often used to increase a child's stability and to promote proper joint alignment.

Spasticity may also be treated with muscle relaxing drugs, including diazepam (Valium), dantrolene (Dantrium), and baclofen (Lioresal). A variety of experimental surgeries have been tried for people with cerebral palsy to control spasticity. Most of these have not proven effective.

ATAXIA AND COORDINATION Ataxia, or lack of balance control, is another factor affecting mobility. Physical therapy is an important tool to help the child with CP maximize balance. Coordination can be worsened if one member of a muscle pair is overly strong; bracing or surgical transfer of the muscle to a less over-powering position may help.

SCOLIOSIS. Scoliosis, or spine curvature, can develop when the muscles that hold the spine in place become either weak or spastic. This can cause pain , as well as interfere with normal posture and internal organ function. Scoliosis may be treated with a trunk brace. If this proves unsuccessful, spinal fusion surgery may be needed to join the vertebrae together, which keeps the spine straight.

Seizures

Seizures occur in 30-50% of children with CP. Seizures may be treated with drugs, most commonly carbamazepine (Tegretol) or ethosuximide (Zarontin). A combination of a ketogenic diet and fasting may also be used to control seizures. Although the need for anti-seizure medication is temporary in some children, it may be required throughout life for others.

Strabismus

Strabismus, or squinting and lack of parallelism in the eyes, occurs in nearly half of all people with spastic CP. Strabismus may be treated with patching and corrective lenses. When these do not work, it may be treated with either surgery on the eye muscles causing the problem or by injection of botulinum toxin.

Nutrition

Due to poor muscle coordination, CP children may not take in adequate nutrition for full growth and development, worsening the results of the disorder. Careful attention to nutritional needs and nutritional supplements is required. Poor swallowing coordination may lead to aspiration, or inhaling of food or saliva. A speech-language therapist may be able to teach the person more effective movement patterns to avoid aspiration. In severe cases, a gastrostomy tube may be required to provide adequate nutrition directly into the digestive system while preventing aspiration.

Other common medical problems

Drooling, dental caries (cavities), and gum disease are more common in people with CP than in the general population, partly because of lowered coordination and increased muscle tightness in the mouth and jaw. Each of these can be prevented to some degree, either through behavioral changes alone or in combination with drug therapy. Constipation is more common as well, and may be treated through dietary changes, or with enemas or suppositories when necessary.

Communication

Poor coordination of the tongue and mouth muscles can also affect speech. Children may benefit from picture boards or other communication devices that allow them to point to make their desires known. For school-age children or older persons with CP, there are a large number of augmentative communication devices, including shorthand typing programs and computer-assisted speech devices. A speech-language therapist can offer valuable advice on the types of equipment available.

Education

The best choice of school for the child with CP depends on the presence and degree of mental impairment and physical impairment, as well as the facilities available in the area. "Inclusion," or mainstreaming the child in a regular public school classroom, may work well for the child with mild physical impairment. Separate classrooms or special schools may be needed for more severely involved children. Schooling for disabled students is governed by the Individuals with Disabilities Education Act (IDEA) at the federal level and state special education rules at the local level. An educational specialist within the school system or from a community social services agency may be able to help the family navigate the various bureaucratic pathways that will ensure the best schooling available.

The process of developing an educational plan for a child with CP begins with an assessment of the child's needs. The assessment is carried out under state guidelines by a team of medical professionals. After the assessment, the school district works with the parents and others involved in the child's education and treatment to develop an Individualized Educational Plan (IEP). The IEP states the child's specific needs for special instruction and indicates what services will be provided. The special services may be as simple as allowing extra time to travel between classes or as extensive as individualized instruction, adapted classroom equipment, and special testing procedures. More information about assessments and IEPs is available through the National Information Center for Children and Youth with Disabilities. The United Cerebral Palsy Assocation is another resource for advocacy, information, and legal rights.

Behavioral and mental health services

The child with CP may have behavioral problems or emotional issues that affect psychological development and social interactions. These may require special intervention or treatment, including behavior modification programs or individual and family counseling. Attention-deficit hyperactivity disorder is common in children with CP, and may require behavioral, educational, and medical intervention.

Expected results

Cerebral palsy can affect every stage of maturation, from childhood through adolescence to adulthood. At each stage, the person with CP and his or her caregivers must strive to achieve and maintain the fullest range of experiences and education consistent with the person's abilities. The advice and intervention of professionals remains crucial for many people with CP.

Although CP is not a terminal disorder, it can affect a person's lifespan by increasing the risk of infection, especially lung infections. Poor nutrition can contribute to the likelihood of infection. People with mild cerebral palsy may have near-normal lifespans. The lifespan of those with more severe forms, especially spastic quadriplegia, is often considerably shortened. However, over 90% of infants with CP survive into adulthood.

In 2002, a radical new method for repairing the damage caused by lack of oxygen at delivery of babies with CP was being funded for study. The possible treatment involved transplanting mature stem cells into babies' circulations, which will then migrate to the site of injury caused by oxygen deprivation, hopefully promoting natural repair of the brain damage. However, this treatment was in very early stages of testing in animals only as of late 2002.

Prevention

The cause of most cases of CP is unknown, but it has become clear in recent years that birth difficulties are not to blame in most cases. Developmental problems before birth, usually unknown and generally undiagnosable, are responsible for most cases. Although the incidence of CP caused by Rh factor incompatibility has declined markedly, the incidence of CP as a consequence of prematurity has increased, because of the increasing success of medical intervention in keeping premature babies alive.

The risk of CP can be decreased through good maternal nutrition, avoidance of drugs or alcohol during pregnancy, and prevention or prompt treatment of infections. Recent preliminary research suggests that magnesium sulfate may reduce the risk of CP in mothers taking it for the medical treatment of preeclampsia and preterm labor

Resources

BOOKS

The Burton Goldberg Group. "Cerebral Palsy." Alternative Medicine: The Definitive Guide. Tiburon, CA: Future Medicine Publishing, Inc., 1999.

"Cerebral Palsy." Reader's Digest Guide to Medical Cures and Treatments. Canada: The Reader's Digest Association, Inc., 1996.

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

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

Vickers, Andrew. Health Options: Complementary Therapies for Cerebral Palsy and Related Conditions. Element Publications, 1994.

PERIODICALS

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

"Hyperbaric Oxygen Therapy." The Exceptional Parent (July 2002): 52.

"IVF Babies at Increased Risk of Cerebral Palsy." Contemporary OB/GYN (July 2002): 41.

Kuban, KCK, and A. Leviton. "Cerebral Palsy." New England Journal of Medicine (1994): 188-95.

"Mature Stem Cell Transplants Linked to Treatment." Pain & Central Nervous System Week (July 22, 2002): 11.

ORGANIZATION

Cranial Academy. 3500 Depaw Boulevard. Indianapolis, IN 46268. (317) 879-0713.

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 Washington, DC 20036-5602. (800) USA-5-UCP,(202) 776-0406, (202) 973-7197 (TTY). Fax: (202) 776-0414. ucpnatl@ucpa.org. http://www.ucpa.org.

OTHER

Electronic forum for cerebral palsy. http://neuro-www.mgh.harvard.edu/forum/CerebralPalsyMenu.html.

Mai Tran

Teresa G. Odle

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Cerebral Palsy

Cerebral palsy

Definition

Cerebral palsy is a term used to describe a group of chronic conditions affecting body movements and muscle coordination. It is caused by damage to one or more specific areas of the brain, usually occurring during fetal development or during infancy.

Description

Cerebral palsy (CP) is an umbrella-like term used to describe a group of chronic disorders impairing movement control that appear in the first few years of life and generally do not worsen over time. The disorders are caused by faulty development or damage to motor areas in the brain that disrupt the brain's ability to control movement and posture. The causes of such cerebral insults include vascular, metabolic, infectious, toxic, traumatic, hypoxic (lack of oxygen) and genetic causes. The mechanism that originates cerebral palsy involves multi-factorial causes, but much is still unknown.

Cerebral palsy distorts messages from the brain to cause either increased muscle tension (hypertonus) or reduced muscle tension (hypotonus). Sometimes this tension will fluctuate, becoming more or less obvious.

Symptoms of CP include difficulty with fine motor tasks (such as writing or using scissors) and difficulty maintaining balance or walking. Symptoms differ from person to person and may change over time. Some people with CP are also affected by other medical disorders, including seizures or mental impairment. Early signs of CP usually appear before three years of age. Infants with this disease are frequently slow to reach developmental milestones such as learning to roll over, sit, crawl, smile, or walk.

Causes of CP may be congenital (present at birth) or acquired after birth. Several of the causes that have been identified through research are preventable or treatable: head injury, jaundice, Rh incompatibility, and rubella (German measles). Cerebral palsy is diagnosed by testing motor skills and reflexes, examining the medical history, and employing a variety of specialized tests. Although its symptoms may change over time, this disorder by definition is not progressive. If a patient shows increased impairment, the physician considers an alternative diagnosis.

Demographics

Cerebral palsy is one of the most common causes of chronic childhood disability. About 3,000 babies are born with the disorder each year in the United States, and about 1,500 preschoolers are diagnosed with cerebral palsy during the first three years of life. In almost 70% of cases, CP is found with some other disorder, the most common being

mental retardation . In all, around 500,000700,000 Americans have some degree of cerebral palsy.

The prevalence of CP has remained very stable for many years. The incidence increases with premature or very low-weight babies regardless of the quality of care. Twins are also four times more likely to develop CP than single births.

Despite medical advances, in some cases the incidence of CP has actually increased over time. This may be attributed to medical advances in areas related to premature babies or the increased usage of artificial fertilization techniques.

Causes and symptoms

CP is caused by damage to an infant's brain before, during or shortly after delivery. The part of the brain that is damaged determines what parts of the body are affected.

There are a number of factors which appear to predispose a child to CP including:

  • Exposure of the expectant mother to certain infections like rubella, toxoplasmosis and cytomegalovirus,
  • Exposure of the expectant mother to certain chemicals like alcohol, cigarettes, cocaine and teratogenic (capable of causing birth defects) agents,
  • Severe physical trauma to the mother during pregnancy, multiple births or maternal illness,
  • Children who are born prematurely (less than 32 weeks) or who are very low birth weight (less than 1,500 grams or about 3 pounds),
  • Failure of the brain to develop properly or neurological damage to the infant's developing brain, including hypoxia (lack of oxygen) during birth,
  • Bacterial meningitis and other infections, bleeding in the brain, lack of oxygen, severe jaundice, and head injury during the first few years of a child's life.

Cerebral palsy is categorized into four different groups that are characterized by different symptoms. Generally, babies that are severely affected may have obvious signs immediately following birth. Many infants do not display immediate CP symptoms. Parents are usually able to notice developmental delays, especially if they have another unaffected child. At the age of about three months, parents may notice a lack of facial expressions or that their baby does not respond to some sounds, or does not follow movement with their eyes. Certain other indicative symptoms may appear at around six months of age, including inability to lift the head or roll over and difficulty feeding. An affected child may be unable to crawl, sit, or stand without support and drooling is a common problem because of poor facial and throat muscle control. CP symptoms depend on the individual and the type of CP and, in particular, whether or not there is a mixed form of the condition.

The four main categories of cerebral palsy are:

  • Spastic CP: Children with spastic CP have increased muscle tone. Their muscles are stiff and their movements can be awkward. Seventy to eighty percent of people with this disease have spasticity . Spastic CP is usually described further by what parts of the body are affected. In spastic diplegia, the main effect is found in both legs. In spastic hemiplegia, one side of the person's body is affected. Spastic quadriplegia affects a person's whole body (face, trunk, legs, and arms).
  • Athetoid or dyskinetic CP: Children with athetoid CP have slow, writhing movements that they cannot control. The movements usually affect a person's hands, arms, feet, and legs. Sometimes the face and tongue are affected and the person has a hard time talking. Muscle tone can change from day to day and can vary even during a single day. Ten to twenty percent of people with CP have the athetoid form of the condition.
  • Ataxic CP: Children with ataxic CP have problems with balance and depth perception. They might be unsteady when they walk. They might have a hard time with quick movements or movements that need a lot of control, like writing. Controlling their hands or arms when they reach for something is often difficult. People with ataxic CP can have increased or decreased muscle tone.
  • Mixed CP: Some people have more than one type of CP, but this is most often a mixture of spasticity and athetoid movements, with tight muscle tone and involuntary reflexes.

Diagnosis

Diagnosing CP in an infant is often a difficult and slow process that takes time to establish with certainty, as there other health problems that can mimic the condition. The physician may suspect that the infant has CP because of a history of difficulties at birth, seizures, feeding problems or low muscle tone. Detailed medical and developmental history, including the history of the pregnancy and delivery, medications taken by the mother during fetal development, infections and fetal movement are all considered. A detailed family history, including the mother's history of miscarriage, relatives with similar conditions, ethnic background, and consanguinity (marriage between close blood relatives) can also prove helpful. The child's physician will perform a thorough physical examination and may order vision and hearing testing.

Infants suffering from brain injury are often slow to reach developmental milestones including rolling over, sitting up, crawling, walking and talking. Healthcare professionals are often hesitant to reach an early diagnosis because the child may recover and they may use other, less emotive terms in labeling the condition such as: neuromotor dys-function, developmental delay, motor disability, static encephalopathy and central nervous system dysfunction.

Physicians must test the child's motor skills, using many of the techniques outlined above and looking for evidence of slow development, abnormal muscle tone, and unusual posture. Healthcare professionals will move slowly and carefully towards a positive diagnosis only after eliminating all other possible causes of the child's condition.

Neuroimaging studies can help to evaluate brain damage and to determine those at risk of developing CP. No study exists to support definitive diagnosis of CP. Computed tomography (CT ) scans provide information to help diagnose congenital malformations and intracranial hemorrhages in the infant. Magnetic resonance imaging (MRI ) is most useful after two to three weeks of life, and is also used to detect brain disease in an older child.

Ultrasound in the neonate (newborn) provides information about the structures of the brain as well as diagnostic information on possible hemorrhage or hypoxicischemic (lack of oxygen) injury.

Evoked potentials are used to evaluate the anatomic pathways of the nerves responsible for hearing and vision. Electroencephalogram (EEG) is useful in evaluating severe hypoxic-ischemic injury.

Treatment team

A neurologist may help to differentiate cerebral palsy from other neurological disorders. Consultation with a neurologist also may be helpful in treatment of patients with seizures. Pulmonologists (lung specialists) may help treat the patient with bronchopulmonary dysplasia or frequent aspiration pneumonia. Orthopedic surgery consultation may be needed to help correct any structural deformities. An ophthalmologist may be indicated to follow up with any patient experiencing visual deficits. Audiologists help screen for hearing deficits. A gastroenterologist (specialist on digestive disorders) may help with reflux and constipation and may be helpful in coordinating feedings to regulate weight gain or weight loss if needed. A periodic nutrition consultation is important to make sure the child does not suffer from growth failure or nutritional deficiencies.

Treatment

Drug therapy is used for those who have seizures associated with CP. Anticonvulsant medications are usually very effective in preventing seizures associated with CP. Drugs are also used to control spasticity in some cases. Medications used most often are diazepam , a general relaxant of the brain and body, baclofen, which blocks signals sent from the spinal cord to contract the muscles, and datrolene, which interferes with the process of muscle contraction. These drugs are used for short periods, but long-term control of spasticity is more difficult to achieve.

Persons with athetoid CP are sometimes given drugs to help reduce abnormal movements, usually anticholinergics . Anticholinergics reduce the activity of acetylcholine, a chemical messenger that helps some brain cells communicate and trigger muscle contraction. Physicians may inject drugs directly into a muscle to reduce spasticity for a short period.

Surgery is used when muscle contractures are severe enough to create problems in movement. The surgeons lengthen the muscle that is too short. Lengthening a muscle usually makes it weaker, so surgery for contractures is usually followed by an extended recovery and therapy period. To reduce spasticity in the legs, surgery called selective dorsal root rhizotomy sometimes proves effective. It reduces the amount of stimulation that reaches leg muscles by the nerves.

Recovery and rehabilitation

Cerebral palsy cannot be cured. Treatment can, however, help a person take part in family, school, and work activities as much as possible. There are many treatments, including physical therapy, occupational therapy, medicine, operations, and orthotic devices that help maintain the highest possible state of wellness and activity.

Specialized Therapies

Physical therapy improves infant-caregiver interaction, gives family support, and supplies resources for parental education, as well as promoting motor and developmental skills. Physical therapists teach the parent or caregiver exercises or activities necessary to help the child reach his or her full potential.

Daily range of motion (ROM) exercises are important to prevent or delay contractures (fixed, rigid muscles) secondary to spasticity, and to maintain mobility of joints and soft tissues. Stretching exercises are performed to increase motion. Progressive resistance exercises also increase strength. Age-appropriate play and adaptive toys and games using the desired exercises are important to elicit the child's full cooperation. Strengthening knee extensor muscles helps to improve crouching and stride length. Postural and motor control training is important following the normal developmental sequence of children (i.e., achieve head and neck control if possible before advancing to trunk control).

Occupational therapists keep the child's developmental age in mind and use adaptive equipment as needed to help attain these milestones. For example, if a child is developmentally ready to stand and explore the environment, but is limited by lack of motor control, a stander or modified walker is used. Performance based upon previous success is encouraged to maintain the child's interest and cooperation. Assistive devices and durable medical equipment help attain function that may not be possible otherwise. Orthotic devives frequently are required to maintain functional joint position especially in persons who are non-ambulatory. Frequent reevaluation of orthotic devices is important as children quickly outgrow them and can develop skin irritation from improper use of orthotic devices.

Recreational therapy, especially hippotherapy (horse-back riding therapy) is frequently a well-liked activity of parents and patients alike to help with muscle tone, range

of motion, strength, coordination, and balance. Hippotherapy also offers many potential cognitive, physical, and emotional benefits. Incorporation of play into all of a child's therapies is important. The child should view physical and occupational therapy as fun, not work. Caregivers should seek fun and creative ways to stimulate children, especially those who have decreased ability to explore their own environments.

Many children with dyskinetic CP have involvement of the face and oropharynx causing difficulty swallowing properly, drooling, and speech difficulties. Speech therapy can be implemented to help improve swallowing and communication. Those patients with athetoid CP may benefit the most from speech therapy, as most have normal intelligence and communication is an obstacle secondary to abnormal muscle movements that affect their speech. Adequate communication is probably the most important goal for enhancing function in the athetoid CP patient.

Clinical trials

As of mid-2004, there were numerous open clinical trials for the study and treatment of cerebral palsy, including:

  • "Botulinum Toxin (BOTOX) for CP," "Relaxation Training to Decrease Pain and Improve Function in Adolescents with CP," and "Constraint-based Therapy to Improve Motor Function in Children with CP," sponsored by the National Institute of Child Health and Human Development (NICHD),
  • "Classification of CP Subtypes," "Eye-Hand Coordination in Children with Spastic Diplegia," "Beneficial Effects of Antenatal Magnesium Sulfate (BEAM Trial)," and "Brain Control of Movements in CP," sponsored by National Institute of Neurological Disorders and Stroke (NINDS),
  • Study of Tongue Pressures, sponsored by Warren G. Magnuson Clinical Center.

Updated information about these clinical trials can be found at the National Institutes of Health website for clinical trials at www.clinicaltrials.gov.

Prognosis

The prognosis of persons with CP varies according to the severity of the disorder. Some children have only mild problems in muscle tone and no problems with daily activities, while others are unable to purposefully move any part of the body. Regression, or worsening of long-term symptoms, is not characteristic of CP. If regression occurs, it is necessary to look for a different cause of the child's problems. In order for a child to be able to walk, a major cascade of events in motor control have to occur. A child must be able to hold up his head before he can sit up on his own, and he must be able to sit independently before he can walk on his own. It is generally assumed that if a child is not sitting up by himself by age four or walking by age eight, he will never be an independent walker. But a child who starts to walk at age three will certainly continue to walk unless he has a disorder other than CP.

In people with severe CP, motor problems often lead to medical complications, including more frequent and serious infections, severe breathing problems, feeding intolerance, and skin breakdown. These medical complications can lead to frequent hospitalizations and a shortened life expectancy.

Epilepsy also occurs in about a third of children with CP and is more frequent in patients with spastic quadriplegia or mental retardation. Cognitive impairment occurs more frequently in CP than in the general population, and mental delays or some form of learning disability has been estimated to occur in over two thirds of CP cases.

Resources

BOOKS

Anderson, Mery Elizabeth, Dineen Tom. Taking Cerebral Palsy to School. St. Louis: Jayjo Books, 2000.

Mechan, Merlin L. Cerebral Palsy. Austin, TX: Pro-Ed Publishers, 2002.

Pincus, Dion. Everything You Need to Know About Cerebral Palsy (Need to Know Library). New York: Rosen Publishing Group, 1999.

PERIODICALS

Darrah, J., et al. "Conductive education intervention for children with cerebral palsy: an AACPDM evidence report." Dev Med Child Neurol 46 (March 2004): 187203.

OTHER

"Cerebral PalsyFacts & Figures." United Cerebral Palsy. (May 1, 2004). <http://www.ucp.org/ucp_generaldoc.cfm/1/9/37/37-37/447>.

"NINDS Cerebral Palsy Information Page." National Institute of Neurological Disorders and Stroke. (May 1, 2004). <http://www.ninds.nih.gov/health_and_medical/disorders/cerebral_palsy.htm>.

ORGANIZATIONS

March of Dimes Birth Defects Foundation. 1275 Mamaroneck Avenue, White Plains, NY 10605. (914) 428-7100 or (888) 663-4637; Fax: (914) 428-8203. askus@ marchofdimes.com. <http://www.marchofdimes.com>.

United Cerebral Palsy (UCP). 1600 L Street, NW, Suite 700, Washington, DC 20036. (202) 776-0406 or (800) USA-5UCP (872-5827); Fax: (202) 776-0414. national@ucp.org. <http://www.ucp.org>.

Francisco de Paula Careta

Iuri Drumond Louro

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de Paula Careta, Francisco; Louro, Iuri. "Cerebral Palsy." Gale Encyclopedia of Neurological Disorders. 2005. Retrieved June 25, 2016 from Encyclopedia.com: http://www.encyclopedia.com/doc/1G2-3435200090.html

cerebral palsy

cerebral palsy denotes ‘a disorder of movement and posture resulting from a permanent, non-progressive defect or lesion of the immature brain’. There are a number of causative factors, most of which act before birth, but some can act up until the age of 2 years, to cause a non-progressive disorder of the still-developing brain. The overall incidence of cerebral palsy is about 5 per 2000 infants, although there are quite large variations between countries.

In some cases there are prenatal influences which cause failure of brain development: either definite genetic factors, inadequate supply of oxygenated blood to the fetal brain, rubella in the first trimester of pregnancy, toxoplasmosis transmitted across the placenta, or irradiation.

In about half of all instances cerebral palsy is associated with pre-term delivery and low birthweight. Compared to those at full term and of normal weight, such infants are particularly at risk of developing cerebral palsy if they suffer hypoxia or inadequate blood flow to the brain around the time of birth, or if they suffer brain infection (encephalitis, meningitis) or head injury during early life.

There are many variations in the types of cerebral palsy, and often there is a mixture of neurological abnormalities. The children may have increased muscle tone (spasticity), which most commonly affects all four limbs — the condition of spastic tetraplegia; or there may be spastic paraplegia, when the arms are apparently unaffected; or the so-called cerebral diplegia, often associated with premature birth, when the arms are less affected than the legs. There can also be hemiplegia, affecting one half of the body, or monoplegia, affecting one limb.

There may also be involuntary movements, such as the writhing (athetoid) type and various disturbances of co-ordination, depending on the parts of the brain affected. Less commonly, cerebral palsy takes the form of a hypotonic tetraplegia, with no spasticity, when the child has a mobility problem but with floppy muscles.

Children with cerebral palsy frequently have other severe handicaps. About half of them have an IQ less than 70, whilst 25% have an IQ above 90, compared with 3% below 70 and 75% above 90 in the general population. Epileptic seizures are more common than in the population overall. Retarded speech development may parallel the degree of learning disorder, but it is frequently also complicated by the problems of defective muscle control. Different forms of speech defect (dysarthria) accompany the different types of movement disorder.

Although the brain abnormality underlying the cerebral palsy is permanent and cannot be corrected, much help can be given to affected children and their families by way of physiotherapy, play and occupational therapy, speech therapy, orthopaedic surgery, and a variety of nutritional, mobility, and educational aids.

Forrester Cockburn


See also muscle tone; paralysis.

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COLIN BLAKEMORE and SHELIA JENNETT. "cerebral palsy." The Oxford Companion to the Body. 2001. Encyclopedia.com. 25 Jun. 2016 <http://www.encyclopedia.com>.

COLIN BLAKEMORE and SHELIA JENNETT. "cerebral palsy." The Oxford Companion to the Body. 2001. Encyclopedia.com. (June 25, 2016). http://www.encyclopedia.com/doc/1O128-cerebralpalsy.html

COLIN BLAKEMORE and SHELIA JENNETT. "cerebral palsy." The Oxford Companion to the Body. 2001. Retrieved June 25, 2016 from Encyclopedia.com: http://www.encyclopedia.com/doc/1O128-cerebralpalsy.html

cerebral palsy

cerebral palsy (sərē´brəl pôl´zē), disability caused by brain damage before or during birth or in the first years, resulting in a loss of voluntary muscular control and coordination. Although the exact cause is unknown, apparent predisposing factors include disease (e.g., rubella, genital herpes simplex), very low infant birthweight (less than 3.3 lb [1.5 kg]), and injury or physical abuse. Maternal smoking, alcohol consumption, and ingestion of certain drugs can also contribute. Most cases are associated with prenatal problems; about 10% of the cases are thought to be due to oxygen deficiency during the birth process. The severity of the affliction is dependent on the extent of the brain damage. Those with mild cases may have only a few affected muscles, while severe cases can result in total loss of coordination or paralysis.

There are many different forms of the disability, each caused by damage to a different area of the brain. The spastic type, accounting for over half of the cases, results from damage to the motor areas of the cerebral cortex and causes the affected muscles to be contracted and overresponsive to stimuli. Athetoid cerebral palsy, caused by damage to the basal ganglia, results in continual, involuntary writhing movements. Choreic cerebral palsy is characterized by jerking, flailing movements. Ataxic cerebral palsy, involving the cerebellum, causes either an impaired sense of balance or a lack of coordinated movements. In addition to these types, which may occur singly or together, emotional, visual, and hearing impairments and convulsive seizures may be present. Some of those affected have a degree of mental retardation, but in many the intellect is unimpaired.

There is no cure for the disorder. Treatment usually includes physical, occupational, and speech therapy, and sometimes includes biofeedback and muscle relaxants. Sometimes appliances such as braces and surgery are helpful. Measures that appear to help decrease the incidence of cerebral palsy include maternal immunization against rubella, maternal abstention from smoking and alcohol consumption, magnesium sulfate given in premature labor, treatment for Rh incompatibility (see blood groups), and treatment of hyperbilirubinemia (jaundice) in the newborn.

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Cerebral Palsy

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.

Cerebral Palsy: Words to Know

Ataxia:
A condition in which balance and coordination are impaired.
Athetonia:
A condition marked by slow, twisting, involuntary muscle movements.
Attention-deficit/Hyperactivity Disorder:
A behavior disorder marked by inattentiveness, a high level of activity, and impulsive behavior.
Contracture:
Shortening of a muscle.
Diplegia:
Paralysis of the arm and leg on one side of the body.
Dystonia:
Loss of the ability to control detailed muscle movement.
Hemiplegia:
Paralysis of one side of the body.
Hypotonia:
A condition in which muscles become floppy and lacking in strength.
Quadriplegia:
Paralysis of both arms and both legs.
Spastic:
A condition in which muscles are rigid, posture may be abnormal, and control of muscles may be impaired.

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.

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.

WHAT IS ULTRASOUND?

Ultrasound is a method by which doctors can study organs and tissues inside the human body. The technique was developed during World War II (193945) when scientists used very high pitched sound waves (ultrasound) to search for submarines under the water. The sound waves were sent out from a ship. They traveled through the water and bounced off objects, such as submarines and fish. The reflect sound waves formed characteristic patterns, depending on the objects from which they rebounded. By the 1950s scientists began to realize that ultrasound had many other uses and could be especially helpful in the field of medicine.

A problem doctors face is that they cannot see through the body to find out what organs and tissues look like. They can shine X rays through the body, which does produce photographs. However, only teeth, bone, and other hard substances can be seen. Ultrasound is an ideal method for taking pictures of organs and tissues. It is reflected off these objects in much the same way it reflect off submarines, and it does not harm the organs and tissues it strikes. Today, ultrasound is widely used for many medical purposes, such as diagnosing cerebral palsy.

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.

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 076491517. 800EPARENT; 2016346550.

Organizations

National Information Center for Children and Youth with Disabilities. PO Box 1492, Washington, DC 200131492. (800) 6950285.

United Cerebral Palsy Association. 1660 L Street NW, Suite 700, Washington, DC 200365602. (800) 8725827; (202) 7760406; (202) 9737197 (TTY). (202) 7760414 (fax). ucpnatl@ucpa.org. http://www.ucpa.org.

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cerebral palsy

cerebral palsy n. a disorder of movement and/or posture as a result of nonprogressive but permanent damage to the developing brain, which may occur before, during, or immediately after delivery. Causes include an inadequate supply of oxygen to the brain, low levels of glucose in the blood, and infection. Cerebral palsy is often associated with other problems, such as learning difficulties, hearing difficulties, poor speech, and epilepsy. There are three main types: spastic, in which the limbs are difficult to control; ataxic hypotonic, in which the main problem is poor balance and uncoordinated movements; and dyskinetic, in which there is involuntary movement of the limbs.
www.scope.org.uk Website of Scope

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"cerebral palsy." A Dictionary of Nursing. 2008. Encyclopedia.com. 25 Jun. 2016 <http://www.encyclopedia.com>.

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Cerebral Palsy

Cerebral Palsy

Marshas Story

What Is Cerebral Palsy?

What Are the Signs and Symptoms of Cerebral Palsy?

How Is Cerebral Palsy Diagnosed and Treated?

Living with Cerebral Palsy

Resources

Cerebral palsy (SER-uh-brul PAWL-zee) is a group of conditions characterized by a loss or limitation of movement or other nerve functions caused by brain injuries during fetal development or near the time of birth.

KEYWORDS

for searching the Internet and other reference sources

Cerebellum

Cerebral cortex

Cerebrum

Movement disorders

Spastic syndromes

Marshas Story

Marsha could not help comparing her baby to the others at the park. At ten months old, Sam could hardly sit up on his own, but most of the other babies Sams age were crawling and pulling up to a standing position. Marsha also noticed that Sam often felt stiff when she picked him up. When Marsha took Sam to the doctor and described his symptoms, the doctor suspected that Sam had a form of cerebral palsy. Sams doctor explained that cerebral palsy occurs when parts of the brain that control movement are injured or dont develop properly during pregnancy.

What Is Cerebral Palsy?

Cerebral palsy is not a single condition but instead identifies a group of movement disorders caused by a brain injury. Before birth or shortly after birth, developing brain tissue may be injured by trauma* or by diseases such as meningitis* or encephalitis*. Brain damage may also result from severe dehydration*, lack of oxygen, or a variety of other problems. In most cases, however, the cause of cerebral palsy is unknown.

* trauma
(TRAW-muh), in the broadest sense, refers to a wound or injury, whether psychological or physical. It occurs when a person experiences a sudden or violent injury (physical trauma) or encounters a situation that involves intense fear and loss of control (psychological trauma).
* meningitis
(men-in-JY-tis) is an inflammation of the membranes that surround the brain and the spinal cord.
* encephalitis
(en-sef-uh-LY-tis) is an inflammation of the brain that can range from mild to extremely serious. It is usually caused by one of many viruses.
* dehydration
(dee-hy-DRAY-shun) is loss of fluid from the body.

In the United States, cerebral palsy occurs at a rate of about 5 cases per 2,000 births, and the rate is even higher in premature infants (about 5 percent of premature babies have cerebral palsy). Each year, 10,000 new cases of cerebral palsy are diagnosed in the United States.

What Are the Signs and Symptoms of Cerebral Palsy?

Cerebral palsy is divided into different types based on the symptoms that a person experiences. Spastic (SPAS-tik) syndromes are the most common form of cerebral palsy and account for about 70 percent of cases. People with spastic syndromes move in a stiff or jerky way. Spastic movements may affect one limb, one side of the body, both legs, or both arms and legs, and the affected limbs are usually underdeveloped and have rigid muscles. In mild cases, the symptoms may only show during certain activities such as running. People with spastic syndromes also may experience seizures*, partial or full loss of movement (paralysis), sensory abnormalities, and speech, hearing, and vision problems. About 20 percent of people with cerebral palsy experience slow, writhing, involuntary muscle movements in the arms and legs. The symptoms usually increase with stress and disappear when the person is sleeping. Others (about 10 percent of people with cerebral palsy) have weakness, uncoordinated movements, and shaking; a person with this type of cerebral palsy has difficulty with rapid and fine movements. Some people with cerebral palsy experience a mixture of all of these symptoms.

* seizures
(SEE-zhurz) are sudden episodes of involuntary (uncontrollable) body movements, changes in behavior or sensations, or loss of consciousness that result from bursts of abnormal electrical energy in the brain.

How Is Cerebral Palsy Diagnosed and Treated?

Cerebral palsy is sometimes difficult to diagnose in infants. Clear diagnosis may be delayed until a child is about 2 years old. Children at risk for developing cerebral palsy, such as children who are born with very low birth weights (less than 2 pounds), should be watched closely.

Treatment for cerebral palsy is tailored to each individuals specific symptoms. Doctors and therapists work together to set up a treatment program to help the patient deal with the challenges of day-to-day living, such as getting dressed, grooming, and eating. Physical therapy can help people with cerebral palsy build strength and improve function in their limbs. Special equipment, such as leg braces, walkers, and wheelchairs, can provide mobility. Muscle relaxants may be used to reduce muscle tone and antiseizure medications may control seizures. Glasses and hearing aids may improve sight and hearing, and special education may help a child with cerebral palsy cope with learning problems.

Living with Cerebral Palsy

Cerebral palsy is a lifelong disorder. The extent of disability caused by cerebral palsy varies with the severity of the symptoms. Some people have mild forms that are barely noticeable; for example, a child might just walk and run with a limp. Other people have more severe symptoms; they might require a wheelchair to get around and have severe mental retardation. While long-term care such as institutionalization may be required in severe cases, many people with cerebral palsy lead full and happy lives. Most children with cerebral palsy do many of the things their friends do, such as go to school, go to summer camp, read, listen to music, talk on the phone, and play sports.

See also

Birth Defects and Brain Development

Brain Injuries

Disability

Resources

Organizations

Nemours Center for Childrens Health Media, Alfred I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19803. This organization is dedicated to issues of childrens health and produces the KidsHealth website. Its website has articles about cerebral palsy. http://www.KidsHealth.org

United Cerebral Palsy, 1660 L Street NW, Suite 700, Washington, DC 20036. http://www.ucpa.org

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cerebral palsy

cerebral palsy Disorder mainly of movement and coordination caused by damage to the brain during or soon after birth. It may feature muscular spasm and weakness, lack of coordination and impaired movement or paralysis and deformities of the limbs. Intelligence is not necessarily affected. The condition may result from a number of causes, such as faulty development, oxygen deprivation, birth injury, haemorrhage or infection.

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cerebral palsy

ce·re·bral pal·sy • n. a condition marked by impaired muscle coordination (spastic paralysis) and/or other disabilities, typically caused by damage to the brain before or at birth. See also spastic.

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