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 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 other 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.

Symptoms

The symptoms of CP are usually not noticeable at birth. As children develop through 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 the newborn's heart rate, cry, color, muscle tone, and motor reactions. Apgar scores of less than three out 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. Ultrasound 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 deaths 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 .

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 as the associated problems the child has. 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 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 for most people.

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 overpowering position may help.

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 antiseizure 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.

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.

Key Terms

Ataxic

Ataxic refers to a condition called ataxia, in which balance and coordination are impaired.

Athetoid

The type of CT that is marked by slow, writhing, involuntary muscle movements.

Attention-deficit/hyperactivity disorder

A behavioral disorder marked by inattentiveness, hyperactivity, and impulsivity.

Augmentative communication devices

Computers, picture boards, and other devices that increase the ability to communicate, either with or without speech.

Contracture

Shortening of a muscle caused by an imbalance of force between opposing muscles.

Diplegia

Paralysis of corresponding parts on both sides of the body.

Dorsal rhizotomy

Surgical procedure that cuts nerve roots to reduce spasticity in affected muscles.

Dystonic

Dystonic refers to a condition called dystonia, in which fine motor control is confused.

Equinovarus

A condition in which the foot is commonly pulled inward.

Equinus

The most common postural deformity, the foot is extended by the strong pull of the rear calf muscles, causing the toes to point.

Hemiplegia

Paralysis of one side of the body.

Hypotonic

Hypotonic refers to a condition called hypotonia, in which fine motor control is floppy, without tone.

IEP

Individualized Educational Plan; a plan that guides the delivery of services to a child with special education needs.

Ketogenic diet

A specialized diet designed to increase the blood levels of breakdown products known as ketone bodies. For unknown reasons, this aids in seizure control.

Perinatal asphyxia

Asphyxia, or lack of oxygen, that occurs during birth.

Quadriplegia

Paralysis of all four limbs.

Serial casting

A series of casts designed to gradually move a limb into a more functional position, as opposed to doing it all at once with one cast, as would be done in setting a broken bone.

Spastic

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

Tenotomy

Surgical procedure that cuts the tendon of a contractured muscle to allow lengthening.

Further Reading

For Your Information

Books

• The Burton Goldberg Group. "Cerebral Palsy." Alternative Medicine: The Definitive Guide. Tiburon, CA: Future Medicine Publishing, Inc., 1998.
• "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.
• Kuban, KCK, and A. Leviton. "Cerebral Palsy." New England Journal of Medicine 1994: 188-95.

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.

Gale Encyclopedia of Alternative Medicine. Gale Group, 2001.