Cerebral Palsy
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In the 1860s, an English surgeon named William Little wrote the first medical
descriptions of a puzzling disorder that struck children in the first years
of life, causing stiff, spastic muscles in their legs and, to a lesser degree,
their arms. These children had difficulty grasping objects, crawling, and
walking. They did not get better as they grew up nor did they become worse.
Their condition, which was called Little's disease for many years, is now
known as spastic diplegia. It is just one of several disorders that affect
control of movement and are grouped together under the term cerebral palsy.
Because it seemed that many of these children were born following premature
or complicated deliveries, Little suggested their condition resulted from
a lack of oxygen during birth. This oxygen shortage damaged sensitive brain
tissues controlling movement, he proposed. But in 1897, the famous psychiatrist
Sigmund Freud disagreed. Noting that children with cerebral palsy often had
other problems such as mental retardation, visual disturbances, and seizures,
Freud suggested that the disorder might sometimes have roots earlier in life,
during the brain's development in the womb. "Difficult birth, in certain cases,"
he wrote, "is merely a symptom of deeper effects that influence the development
of the fetus."
Despite Freud's observation, the belief that birth complications cause most
cases of cerebral palsy was widespread among physicians, families, and even
medical researchers until very recently. In the 1980s, however, scientists
analyzed extensive data from a government study of more than 35,000 births
and were surprised to discover that such complications account for only a
fraction of cases -- probably less than 10 percent. In most cases of cerebral
palsy, no cause of the factors explored could be found. These findings from
the NINDS perinatal study have profoundly altered medical theories about cerebral
palsy and have spurred today's researchers to explore alternative causes.
At the same time, biomedical research has also led to significant changes
in understanding, diagnosing, and treating persons with cerebral palsy. Risk
factors not previously recognized have been identified, notably intrauterine
exposure to infection and disorders of coagulation, and others are under investigation.
Identification of infants with cerebral palsy very early in life gives youngsters
the best opportunity to receive treatment for sensory disabilities and for
prevention of contractures. Biomedical research has led to improved diagnostic
techniques such as advanced brain imaging and modern gait analysis. Certain
conditions known to cause cerebral palsy, such as rubella (German measles)
and jaundice, can now be prevented or treated. Physical, psychological, and
behavioral therapy that assist with such skills as movement and speech and
foster social and emotional development can help children who have cerebral
palsy to achieve and succeed. Medications, surgery, and braces can often improve
nerve and muscle coordination, help treat associated medical problems, and
either prevent or correct deformities.
Much of the research to improve medical understanding of cerebral palsy has
been supported by the National Institute of Neurological Disorders and Stroke
(NINDS), one of the federal government's National Institutes of Health. The
NINDS is America's leading supporter of biomedical research into cerebral
palsy and other neurological disorders. Through this publication, the NINDS
hopes to help the more than 4,500 American babies and infants diagnosed each
year, their families, and others concerned about cerebral palsy benefit from
these research results.
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Cerebral palsy is an umbrella-like term used to describe a group of chronic
disorders impairing control of movement that appear in the first few years
of life and generally do not worsen over time. The term cerebral refers to
the brain's two halves, or hemispheres, and palsy describes any disorder that
impairs control of body movement. Thus, these disorders are not caused by
problems in the muscles or nerves. Instead, faulty development or damage to
motor areas in the brain disrupts the brain's ability to adequately control
movement and posture.
Symptoms of cerebral palsy lie along a spectrum of varying severity. An individual
with cerebral palsy may have difficulty with fine motor tasks, such as writing
or cutting with scissors; experience trouble with maintaining balance and
walking; or be affected by involuntary movements, such as uncontrollable writhing
motion of the hands or drooling. The symptoms differ from one person to the
next, and may even change over time in the individual. Some people with cerebral
palsy are also affected by other medical disorders, including seizures or
mental impairment. Contrary to common belief, however, cerebral palsy does
not always cause profound handicap. While a child with severe cerebral palsy
might be unable to walk and need extensive, lifelong care, a child with mild
cerebral palsy might only be slightly awkward and require no special assistance.
Cerebral palsy is not contagious nor is it usually inherited from one generation
to the next. At this time, it cannot be cured, although scientific research
continues to yield improved treatments and methods of prevention.
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The United Cerebral Palsy Associations estimate that more than 500,000 Americans
have cerebral palsy. Despite advances in preventing and treating certain causes
of cerebral palsy, the number of children and adults it affects has remained
essentially unchanged or perhaps risen slightly over the past 30 years. This
is partly because more critically premature and frail infants are surviving
through improved intensive care. Unfortunately, many of these infants have
developmental problems of the nervous system or suffer neurological damage.
Research is under way to improve care for these infants, as in ongoing studies
of technology to alleviate troubled breathing and trials of drugs to prevent
bleeding in the brain before or soon after birth.
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Spastic diplegia, the disorder first described by Dr. Little in the 1860s,
is only one of several disorders called cerebral palsy. Today doctors classify
cerebral palsy into four broad categories -- spastic, athetoid, ataxic, and
mixed forms -- according to the type of movement disturbance.
Spastic cerebral palsy. In this form of cerebral palsy, which affects 70
to 80 percent of patients, the muscles are stiffly and permanently contracted.
Doctors will often describe which type of spastic cerebral palsy a patient
has based on which limbs are affected. The names given to these types combine
a Latin description of affected limbs with the term plegia or paresis, meaning
paralyzed or weak. The four commonly diagnosed types of spastic cerebral palsy
are illustrated in the figure.
When both legs are affected by spasticity, they may turn in and cross at
the knees. As these individuals walk, their legs move awkwardly and stiffly
and nearly touch at the knees. This causes a characteristic walking rhythm,
known as the scissors gait.
Individuals with spastic hemiparesis may also experience hemiparetic tremors,
in which uncontrollable shaking affects the limbs on one side of the body.
If these tremors are severe, they can seriously impair movement.
Athetoid, or dyskinetic, cerebral palsy. This form of cerebral palsy is characterized
by uncontrolled, slow, writhing movements. These abnormal movements usually
affect the hands, feet, arms, or legs and, in some cases, the muscles of the
face and tongue, causing grimacing or drooling. The movements often increase
during periods of emotional stress and disappear during sleep. Patients may
also have problems coordinating the muscle movements needed for speech, a
condition known as dysarthria. Athetoid cerebral palsy affects about 10 to
20 percent of patients.
Ataxic cerebral palsy. This rare form affects the sense of balance and depth
perception. Affected persons often have poor coordination; walk unsteadily
with a wide-based gait, placing their feet unusually far apart; and experience
difficulty when attempting quick or precise movements, such as writing or
buttoning a shirt. They may also have intention tremor. In this form of tremor,
beginning a voluntary movement, such as reaching for a book, causes a trembling
that affects the body part being used and that worsens as the individual gets
nearer to the desired object. The ataxic form affects an estimated 5 to 10
percent of cerebral palsy patients.
Mixed forms. It is common for patients to have symptoms of more than one
of the previous three forms. The most common mixed form includes spasticity
and athetoid movements but other combinations are also possible.
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Many individuals who have cerebral palsy have no associated medical disorders.
However, disorders that involve the brain and impair its motor function can
also cause seizures and impair an individual's intellectual development, attentiveness
to the outside world, activity and behavior, and vision and hearing. Medical
disorders associated with cerebral palsy include:
- Mental impairment. About one-third of children who have cerebral palsy
are mildly intellectually impaired, one-third are moderately or severely
impaired, and the remaining third are intellectually normal. Mental impairment
is even more common among children with spastic quadriplegia.
- Seizures or epilepsy. As many as half of all children with cerebral palsy
have seizures. During a seizure, the normal, orderly pattern of electrical
activity in the brain is disrupted by uncontrolled bursts of electricity.
When seizures recur without a direct trigger, such as fever, the condition
is called epilepsy. In the person who has cerebral palsy and epilepsy, this
disruption may be spread throughout the brain and cause varied symptoms
all over the body -- as in tonic-clonic seizures -- or may be confined to
just one part of the brain and cause more specific symptoms -- as in partial
seizures.
Tonic-clonic seizures generally cause patients to cry out and are followed
by loss of consciousness, twitching of both legs and arms, convulsive body
movements, and loss of bladder control.
Partial seizures are classified as simple or complex. In simple partial seizures,
the individual has localized symptoms, such as muscle twitches, chewing movements,
and numbness or tingling. In complex partial seizures, the individual may
hallucinate, stagger, perform automatic and purposeless movements, or experience
impaired consciousness or confusion.
- Growth problems. A syndrome called failure to thrive is common in children
with moderate-to-severe cerebral palsy, especially those with spastic quadriparesis.
Failure to thrive is a general term physicians use to describe children
who seem to lag behind in growth and development despite having enough food.
In babies, this lag usually takes the form of too little weight gain; in
young children, it can appear as abnormal shortness; in teenagers, it may
appear as a combination of shortness and lack of sexual development. Failure
to thrive probably has several causes, including, in particular, poor nutrition
and damage to the brain centers controlling growth and development. In addition,
the muscles and limbs affected by cerebral palsy tend to be smaller than
normal. This is especially noticeable in some patients with spastic hemiplegia,
because limbs on the affected side of the body may not grow as quickly or
as large as those on the more normal side. This condition usually affects
the hand and foot most severely. Since the involved foot in hemiplegia is
often smaller than the unaffected foot even among patients who walk, this
size difference is probably not due to lack of use. Scientists believe the
problem is more likely to result from disruption of the complex process
responsible for normal body growth.
- Impaired vision or hearing. A large number of children with cerebral palsy
have strabismus, a condition in which the eyes are not aligned because of
differences in the left and right eye muscles. In an adult, this condition
causes double vision. In children, however, the brain often adapts to the
condition by ignoring signals from one of the misaligned eyes. Untreated,
this can lead to very poor vision in one eye and can interfere with certain
visual skills, such as judging distance. In some cases, physicians may recommend
surgery to correct strabismus. Children with hemiparesis may have hemianopia,
which is defective vision or blindness that impairs the normal field of
vision of one eye. For example, when hemianopia affects the right eye, a
child looking straight ahead might have perfect vision except on the far
right. In homonymous hemianopia, the impairment affects the same part of
the visual field of both eyes. Impaired hearing is also more frequent among
those with cerebral palsy than in the general population.
- Abnormal sensation and perception. Some children with cerebral palsy have
impaired ability to feel simple sensations like touch and pain. They may
also have stereognosia, or difficulty perceiving and identifying objects
using the sense of touch. A child with stereognosia, for example, would
have trouble identifying a hard ball, sponge, or other object placed in
his hand without looking at the object.
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Cerebral palsy is not one disease with a single cause, like chicken pox or
measles. It is a group of disorders with similar problems in control of movement,
but probably with different causes. When physicians try to uncover the cause
of cerebral palsy in an individual child, they look at the form of cerebral
palsy, the mother's and child's medical history, and onset of the disorder.
In the United States, about 10 to 20 percent of children who have cerebral
palsy acquire the disorder after birth. (The figures are higher in underdeveloped
countries.) Acquired cerebral palsy results from brain damage in the first
few months or years of life and can follow brain infections, such as bacterial
meningitis or viral encephalitis, or results from head injury -- most often
from a motor vehicle accident, a fall, or child abuse.
Congenital cerebral palsy, on the other hand, is present at birth, although
it may not be detected for months. In most cases, the cause of congenital
cerebral palsy is unknown. Thanks to research, however, scientists have pinpointed
some specific events during pregnancy or around the time of birth that can
damage motor centers in the developing brain. Some of these causes of congenital
cerebral palsy include:
- Infections during pregnancy. German measles, or rubella, is caused by
a virus that can infect pregnant women and, therefore, the fetus in the
uterus, to cause damage to the developing nervous system. Other infections
that can cause brain injury in the developing fetus include cytomegalovirus
and toxoplasmosis. There is relatively recent evidence that placental and
perhaps other maternal infection can be associated with cerebral palsy.
- Jaundice in the infant. Bile pigments, compounds that are normally found
in small amounts in the bloodstream, are produced when blood cells are destroyed.
When many blood cells are destroyed in a short time, as in the condition
called Rh incompatibility (see below), the yellow-colored pigments can build
up and cause jaundice. Severe, untreated jaundice can damage brain cells.
- Rh incompatibility. In this blood condition, the mother's body produces
immune cells called antibodies that destroy the fetus's blood cells, leading
to a form of jaundice in the newborn.
- Severe oxygen shortage in the brain or trauma to the head during labor
and delivery. The newborn infant's blood is specially equipped to compensate
for low levels of oxygen, and asphyxia (lack of oxygen caused by interruption
in breathing or poor oxygen supply) is common in babies during the stresses
of labor and delivery. But if asphyxia severely lowers the supply of oxygen
to the infant's brain for lengthy periods, the child may develop brain damage
called hypoxic-ischemic encephalopathy. A significant proportion of babies
with this type of brain damage die, and others may develop cerebral palsy,
which is then often accompanied by mental impairment and seizures.
In the past, physicians and scientists attributed most cases of cerebral
palsy to asphyxia or other complications during birth if they could not identify
another cause. However, extensive research by NINDS scientists and others
has shown that very few babies who experience asphyxia during birth develop
encephalopathy soon after birth. Research also shows that a large proportion
of babies who experience asphyxia do not grow up to have cerebral palsy or
other neurological disorders. Birth complications including asphyxia are now
estimated to account for about 6 percent of congenital cerebral palsy cases.
- Stroke. Coagulation disorders in mothers or infants can produce stroke
in the fetus or newborn baby. Bleeding in the brain has several causes --
including broken blood vessels in the brain, clogged blood vessels, or abnormal
blood cells -- and is one form of stroke. Although strokes are better known
for their effects on older adults, they can also occur in the fetus during
pregnancy or the newborn around the time of birth, damaging brain tissue
and causing neurological problems. Ongoing research is testing potential
treatments that may one day help prevent stroke in fetuses and newborns.
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Research scientists have examined thousands of expectant mothers, followed
them through childbirth, and monitored their children's early neurological
development. As a result, they have uncovered certain characteristics, called
risk factors, that increase the possibility that a child will later be diagnosed
with cerebral palsy:
- Breech presentation. Babies with cerebral palsy are more likely to present
feet first, instead of head first, at the beginning of labor.
- Complicated labor and delivery. Vascular or respiratory problems of the
baby during labor and delivery may sometimes be the first sign that a baby
has suffered brain damage or that a baby's brain has not developed normally.
Such complications can cause permanent brain damage.
- Low Apgar score. The Apgar score (named for anesthesiologist Virginia
Apgar) is a numbered rating that reflects a newborn's condition. To determine
an Apgar score, doctors periodically check the baby's heart rate, breathing,
muscle tone, reflexes, and skin color in the first minutes after birth.
They then assign points; the higher the score, the more normal the baby's
condition. A low score at 10-20 minutes after delivery is often considered
an important sign of potential problems.
- Low birthweight and premature birth. The risk of cerebral palsy is higher
among babies who weigh less than 2500 grams (5 lbs., 7 1/2 oz.) at birth
and among babies who are born less than 37 weeks into pregnancy. This risk
increases as birthweight falls.
- Multiple births. Twins, triplets, and other multiple births are linked
to an increased risk of cerebral palsy.
- Nervous system malformations. Some babies born with cerebral palsy have
visible signs of nervous system malformation, such as an abnormally small
head (microcephaly). This suggests that problems occurred in the development
of the nervous system while the baby was in the womb.
- Maternal bleeding or severe proteinuria late in pregnancy. Vaginal bleeding
during the sixth to ninth months of pregnancy and severe proteinuria (the
presence of excess proteins in the urine) are linked to a higher risk of
having a baby with cerebral palsy.
- Maternal hyperthyroidism, mental retardation, or seizures. Mothers with
any of these conditions are slightly more likely to have a child with cerebral
palsy.
- Seizures in the newborn. An infant who has seizures faces a higher risk
of being diagnosed, later in childhood, with cerebral palsy.
Knowing these warning signs helps doctors keep a close eye on children who
face a higher risk for long-term problems in the nervous system. However,
parents should not become too alarmed if their child has one or more of these
factors. Most such children do not have and do not develop cerebral palsy.
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Several of the causes of cerebral palsy that have been identified through
research are preventable or treatable:
- Head injury can be prevented by regular use of child safety seats when
driving in a car and helmets during bicycle rides, and elimination of child
abuse. In addition, common sense measures around the household -- like close
supervision during bathing and keeping poisons out of reach -- can reduce
the risk of accidental injury.
- Jaundice of newborn infants can be treated with phototherapy. In phototherapy,
babies are exposed to special blue lights that break down bile pigments,
preventing them from building up and threatening the brain. In the few cases
in which this treatment is not enough, physicians can correct the condition
with a special form of blood transfusion.
- Rh incompatibility is easily identified by a simple blood test routinely
performed on expectant mothers and, if indicated, expectant fathers. This
incompatibility in blood types does not usually cause problems during a
woman's first pregnancy, since the mother's body generally does not produce
the unwanted antibodies until after delivery. In most cases, a special serum
given after each childbirth can prevent the unwanted production of antibodies.
In unusual cases, such as when a pregnant woman develops the antibodies
during her first pregnancy or antibody production is not prevented, doctors
can help minimize problems by closely watching the developing baby and,
when needed, performing a transfusion to the baby while in the womb or an
exchange transfusion (in which a large volume of the baby's blood is removed
and replaced) after birth.
- Rubella, or German measles, can be prevented if women are vaccinated against
this disease before becoming pregnant.
In addition, it is always good to work toward a healthy pregnancy through
regular prenatal care and good nutrition and by eliminating smoking, alcohol
consumption, and drug abuse. Despite the best efforts of parents and physicians,
however, children will still be born with cerebral palsy. Since in most cases
the cause of cerebral palsy is unknown, little can currently be done to prevent
it. As investigators learn more about the causes of cerebral palsy through
basic and clinical research, doctors and parents will be better equipped to
help prevent this disorder.
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Early signs of cerebral palsy usually appear before 3 years of age, and parents
are often the first to suspect that their infant is not developing motor skills
normally. Infants with cerebral palsy are frequently slow to reach developmental
milestones, such as learning to roll over, sit, crawl, smile, or walk. This
is sometimes called developmental delay.
Some affected children have abnormal muscle tone. Decreased muscle tone is
called hypotonia; the baby may seem flaccid and relaxed, even floppy. Increased
muscle tone is called hypertonia, and the baby may seem stiff or rigid. In
some cases, the baby has an early period of hypotonia that progresses to hypertonia
after the first 2 to 3 months of life. Affected children may also have unusual
posture or favor one side of their body.
Parents who are concerned about their baby's development for any reason should
contact their physician, who can help distinguish normal variation in development
from a developmental disorder.
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Doctors diagnose cerebral palsy by testing an infant's motor skills and looking
carefully at the infant's medical history. In addition to checking for those
symptoms described above -- slow development, abnormal muscle tone, and unusual
posture -- a physician also tests the infant's reflexes and looks for early
development of hand preference.
Reflexes are movements that the body makes automatically in response to a
specific cue. For example, if a newborn baby is held on its back and tilted
so the legs are above its head, the baby will automatically extend its arms
in a gesture, called the Moro reflex, that looks like an embrace. Babies normally
lose this reflex after they reach 6 months, but those with cerebral palsy
may retain it for abnormally long periods. This is just one of several reflexes
that a physician can check.
Doctors can also look for hand preference -- a tendency to use either the
right or left hand more often. When the doctor holds an object in front and
to the side of the infant, an infant with hand preference will use the favored
hand to reach for the object, even when it is held closer to the opposite
hand. During the first 12 months of life, babies do not usually show hand
preference. But infants with spastic hemiplegia, in particular, may develop
a preference much earlier, since the hand on the unaffected side of their
body is stronger and more useful.
The next step in diagnosing cerebral palsy is to rule out other disorders
that can cause movement problems. Most important, doctors must determine that
the child's condition is not getting worse. Although its symptoms may change
over time, cerebral palsy by definition is not progressive. If a child is
continuously losing motor skills, the problem more likely springs from elsewhere
-- including genetic diseases, muscle diseases, disorders of metabolism, or
tumors in the nervous system. The child's medical history, special diagnostic
tests, and, in some cases, repeated check-ups can help confirm that other
disorders are not at fault.
The doctor may also order specialized tests to learn more about the possible
cause of cerebral palsy. One such test is computed tomography, or CT, a sophisticated
imaging technique that uses X rays and a computer to create an anatomical
picture of the brain's tissues and structures. A CT scan may reveal brain
areas that are underdeveloped, abnormal cysts (sacs that are often filled
with liquid) in the brain, or other physical problems. With the information
from CT scans, doctors may be better equipped to judge the long-term outlook
for an affected child.
Magnetic resonance imaging, or MRI, is a relatively new brain imaging technique
that is rapidly gaining widespread use for identifying brain disorders. This
technique uses a magnetic field and radio waves, rather than X rays. MRI gives
better pictures of structures or abnormal areas located near bone than CT.
A third test that can expose problems in brain tissues is ultrasonography.
This technique bounces sound waves off the brain and uses the pattern of echoes
to form a picture, or sonogram, of its structures. Ultrasonography can be
used in infants before the bones of the skull harden and close. Although it
is less precise than CT and MRI scanning, this technique can detect cysts
and structures in the brain, is less expensive, and does not require long
periods of immobility.
Finally, physicians may want to look for other conditions that are linked
to cerebral palsy, including seizure disorders, mental impairment, and vision
or hearing problems.
When the doctor suspects a seizure disorder, an electroencephalogram, or
EEG, may be ordered. An EEG uses special patches called electrodes placed
on the scalp to record the natural electrical currents inside the brain. This
recording can help the doctor see telltale patterns in the brain's electrical
activity that suggest a seizure disorder.
Intelligence tests are often used to determine if a child with cerebral palsy
is mentally impaired. Sometimes, however, a child's intelligence may be underestimated
because problems with movement, sensation, or speech due to cerebral palsy
make it difficult for him or her to perform well on these tests.
If problems with vision are suspected, the doctor may refer the patient to
an ophthalmologist for examination; if hearing impairment seems likely, an
otologist may be called in.
Identifying these accompanying conditions is important and is becoming more
accurate as ongoing research yields advances that make diagnosis easier. Many
of these conditions can then be addressed through specific treatments, improving
the long-term outlook for those with cerebral palsy.
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Cerebral palsy can not be cured, but treatment can often improve a child's
capabilities. In fact, progress due to medical research now means that many
patients can enjoy near-normal lives if their neurological problems are properly
managed. There is no standard therapy that works for all patients. Instead,
the physician must work with a team of health care professionals first to
identify a child's unique needs and impairments and then to create an individual
treatment plan that addresses them.
Some approaches that can be included in this plan are drugs to control seizures
and muscle spasms, special braces to compensate for muscle imbalance, surgery,
mechanical aids to help overcome impairments, counseling for emotional and
psychological needs, and physical, occupational, speech, and behavioral therapy.
In general, the earlier treatment begins, the better chance a child has of
overcoming developmental disabilities or learning new ways to accomplish difficult
tasks.
The members of the treatment team for a child with cerebral palsy should
be knowledgeable professionals with a wide range of specialties. A typical
treatment team might include:
- a physician, such as a pediatrician, a pediatric neurologist, or a pediatric
physiatrist, trained to help developmentally disabled children. This physician,
often the leader of the treatment team, works to synthesize the professional
advice of all team members into a comprehensive treatment plan, implements
treatments, and follows the patient's progress over a number of years.
- an orthopedist, a surgeon who specializes in treating the bones, muscles,
tendons, and other parts of the body's skeletal system. An orthopedist might
be called on to predict, diagnose, or treat muscle problems associated with
cerebral palsy.
- a physical therapist, who designs and implements special exercise programs
to improve movement and strength.
- an occupational therapist, who can help patients learn skills for day-to-day
living, school, and work.
- a speech and language pathologist, who specializes in diagnosing and treating
communication problems.
- a social worker, who can help patients and their families locate community
assistance and education programs.
- a psychologist, who helps patients and their families cope with the special
stresses and demands of cerebral palsy. In some cases, psychologists may
also oversee therapy to modify unhelpful or destructive behaviors or habits.
- an educator, who may play an especially important role when mental impairment
or learning disabilities present a challenge to education.
Individuals who have cerebral palsy and their family or caregivers are also
key members of the treatment team, and they should be intimately involved
in all steps of planning, making decisions, and applying treatments. Studies
have shown that family support and personal determination are two of the most
important predictors of which individuals who have cerebral palsy will achieve
long-term goals.
Too often, however, physicians and parents may focus primarily on an individual
symptom -- especially the inability to walk. While mastering specific skills
is an important focus of treatment on a day-to-day basis, the ultimate goal
is to help individuals grow to adulthood and have maximum independence in
society. In the words of one physician, "After all, the real point of walking
is to get from point A to point B. Even if a child needs a wheelchair, what's
important is that they're able to achieve this goal."
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Physical, Behavioral, and Other Therapies
Therapy -- whether for movement, speech, or practical tasks
-- is a cornerstone of cerebral palsy treatment. The skills a 2-year-old needs
to explore the world are very different from those that a child needs in the
classroom or a young adult needs to become independent. Cerebral palsy therapy
should be tailored to reflect these changing demands.
Physical therapy usually begins in the first few years of life, soon after
the diagnosis is made. Physical therapy programs use specific sets of exercises
to work toward two important goals: preventing the weakening or deterioration
of muscles that can follow lack of use (called disuse atrophy) and avoiding
contracture, in which muscles become fixed in a rigid, abnormal position.
Contracture is one of the most common and serious complications of cerebral
palsy. Normally, a child whose bones are growing stretches the body's muscles
and tendons through running and walking and other daily activities. This ensures
that muscles will grow at the same rate. But in children with cerebral palsy,
spasticity prevents this stretching and, as a result, muscles do not grow
fast enough to keep up with lengthening bones. The resulting contracture can
disrupt balance and trigger loss of previous abilities. Physical therapy alone,
or in combination with special braces (sometimes called orthotic devices),
works to prevent this complication by stretching spastic muscles. For example,
if a child has spastic hamstrings (tendons located behind the knee), the therapist
and parents should encourage the child to sit with the legs extended to stretch
them.
A third goal of some physical therapy programs is to improve the child's
motor development. A widespread program of physical therapy that works toward
this goal is the Bobath technique, named for a husband and wife team who pioneered
this approach in England. This program is based on the idea that the primitive
reflexes retained by many children with cerebral palsy present major roadblocks
to learning voluntary control. A therapist using the Bobath technique tries
to counteract these reflexes by positioning the child in an opposing movement.
So, for example, if a child with cerebral palsy normally keeps his arm flexed,
the therapist would repeatedly extend it.
A second such approach to physical therapy is "patterning," which is based
on the principle that motor skills should be taught in more or less the same
sequence that they develop normally. In this controversial approach, the therapist
guides the child with movement problems along the path of normal motor development.
For example, the child is first taught elementary movements like pulling himself
to a standing position and crawling before he is taught to walk -- regardless
of his age. Some experts and organizations, including the American Academy
of Pediatrics, have expressed strong reservations about the patterning approach,
because studies have not documented its value.
Physical therapy is usually just one element of an infant development program
that also includes efforts to provide a varied and stimulating environment.
Like all children, the child with cerebral palsy needs new experiences and
interactions with the world around him in order to learn. Stimulation programs
can bring this valuable experience to the child who is physically unable to
explore.
As the child with cerebral palsy approaches school age, the emphasis of therapy
shifts away from early motor development. Efforts now focus on preparing the
child for the classroom, helping the child master activities of daily living,
and maximizing the child's ability to communicate.
Physical therapy can now help the child with cerebral palsy prepare for the
classroom by improving his or her ability to sit, move independently or in
a wheelchair, or perform precise tasks, such as writing. In occupational therapy,
the therapist works with the child to develop such skills as feeding, dressing,
or using the bathroom. This can help reduce demands on caregivers and boost
self-reliance and self-esteem. For the many children who have difficulty communicating,
speech therapy works to identify specific difficulties and overcome them through
a program of exercises. For example, if a child has difficulty saying words
that begin with "b," the therapist may suggest daily practice with a list
of "b" words, increasing their difficulty as each list is mastered. Speech
therapy can also work to help the child learn to use special communication
devices, such as a computer with voice synthesizers.
Behavioral therapy provides yet another avenue to increase a child's abilities.
This therapy, which uses psychological theory and techniques, can complement
physical, speech, or occupational therapy. For example, behavioral therapy
might include hiding a toy inside a box to reward a child for learning to
reach into the box with his weaker hand. Likewise, a child learning to say
his "b" words might be given a balloon for mastering the word. In other cases,
therapists may try to discourage unhelpful or destructive behaviors, such
as hair-pulling or biting, by selectively presenting a child with rewards
and praise during other, more positive activities.
As a child with cerebral palsy grows older, the need for and types of therapy
and other support services will continue to change. Continuing physical therapy
addresses movement problems and is supplemented by vocational training, recreation
and leisure programs, and special education when necessary. Counseling for
emotional and psychological challenges may be needed at any age, but is often
most critical during adolescence. Depending on their physical and intellectual
abilities, adults may need attendant care, living accommodations, transportation,
or employment opportunities.
Regardless of the patient's age and which forms of therapy are used, treatment
does not end when the patient leaves the office or treatment center. In fact,
most of the work is often done at home. The therapist functions as a coach,
providing parents and patients with the strategy and drills that can help
improve performance at home, at school, and in the world. As research continues,
doctors and parents can expect new forms of therapy and better information
about which forms of therapy are most effective for individuals with cerebral
palsy.
Drug Therapy
Physicians usually prescribe drugs for those who have seizures associated
with cerebral palsy, and these medications are very effective in preventing
seizures in many patients. In general, the drugs given to individual patients
are chosen based on the type of seizures, since no one drug controls all types.
However, different people with the same type of seizure may do better on different
drugs, and some individuals may need a combination of two or more drugs to
achieve good seizure control.
Drugs are also sometimes used to control spasticity, particularly following
surgery. The three medications that are used most often are diazepam, which
acts as a general relaxant of the brain and body; baclofen, which blocks signals
sent from the spinal cord to contract the muscles; and dantrolene, which interferes
with the process of muscle contraction. Given by mouth, these drugs can reduce
spasticity for short periods, but their value for long-term control of spasticity
has not been clearly demonstrated. They may also trigger significant side
effects, such as drowsiness, and their long-term effects on the developing
nervous system are largely unknown. One possible solution to avoid such side
effects may lie in current research to explore new routes for delivering these
drugs.
Patients with athetoid cerebral palsy may sometimes be given drugs that help
reduce abnormal movements. Most often, the prescribed drug belongs to a group
of chemicals called anticholinergics that work by reducing the activity of
acetylcholine. Acetylcholine is a chemical messenger that helps some brain
cells communicate and that triggers muscle contraction. Anticholinergic drugs
include trihexyphenidyl, benztropine, and procyclidine hydrochloride.
Occasionally, physicians may use alcohol "washes" -- or injections of alcohol
into a muscle -- to reduce spasticity for a short period. This technique is
most often used when physicians want to correct a developing contracture.
Injecting alcohol into a muscle that is too short weakens the muscle for several
weeks and gives physicians time to work on lengthening the muscle through
bracing, therapy, or casts. In some cases, if the contracture is detected
early enough, this technique may avert the need for surgery.
Surgery
Surgery is often recommended when contractures are severe enough to cause
movement problems. In the operating room, surgeons can lengthen muscles and
tendons that are proportionately too short. First, however, they must determine
the exact muscles at fault, since lengthening the wrong muscle could make
the problem worse.
Finding problem muscles that need correction can be a difficult task. To
walk two strides with a normal gait, it takes more than 30 major muscles working
at exactly the right time and exactly the right force. A problem in any one
muscle can cause abnormal gait. Furthermore, the natural adjustments the body
makes to compensate for muscle problems can be misleading. A new tool that
enables doctors to spot gait abnormalities, pinpoint problem muscles, and
separate real problems from compensation is called gait analysis. Gait analysis
combines cameras that record the patient while walking, computers that analyze
each portion of the patient's gait, force plates that detect when feet touch
the ground, and a special recording technique that detects muscle activity
(known as electromyography). Using these data, doctors are better equipped
to intervene and correct significant problems. They can also use gait analysis
to check surgical results.
Because lengthening a muscle makes it weaker, surgery for contractures is
usually followed by months of recovery. For this reason, doctors try to fix
all of the affected muscles at once when it is possible or, if more than one
surgical procedure is unavoidable, they may try to schedule operations close
together.
A second surgical technique, known as selective dorsal root rhizotomy, aims
to reduce spasticity in the legs by reducing the amount of stimulation that
reaches leg muscles via nerves. In the procedure, doctors try to locate and
selectively sever overactivated nerves controlling leg muscles. Although there
is scientific controversy over how selective this technique actually is, recent
research results suggest it can reduce spasticity in some patients, particularly
those who have spastic diplegia. Ongoing research is evaluating this surgery's
effectiveness.
Experimental surgical techniques include chronic cerebellar stimulation and
stereotaxic thalamotomy. In chronic cerebellar stimulation, electrodes are
implanted on the surface of the cerebellum -- the part of the brain responsible
for coordinating movement -- and are used to stimulate certain cerebellar
nerves. While it was hoped that this technique would decrease spasticity and
improve motor function, results of this invasive procedure have been mixed.
Some studies have reported improvements in spasticity and function, others
have not.
Stereotaxic thalamotomy involves precise cutting of parts of the thalamus,
which serves as the brain's relay station for messages from the muscles and
sensory organs. This has been shown effective only for reducing hemiparetic
tremors (see glossary).
Mechanical aids
Whether they are as humble as velcro shoes or as advanced as computerized
communication devices, special machines and gadgets in the home, school, and
workplace can help the child or adult with cerebral palsy overcome limitations.
The computer is probably the most dramatic example of a new device that can
make a difference in the lives of those with cerebral palsy. For example,
a child who is unable to speak or write but can make head movements may be
able to learn to control a computer using a special light pointer that attaches
to a headband. Equipped with a computer and voice synthesizer, this child
could communicate with others. In other cases, technology has led to new versions
of old devices, such as the traditional wheelchair and its modern offspring
that runs on electricity.
Many such devices are products of engineering research supported by private
foundations and other groups.
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Poor control of the muscles of the throat, mouth and tongue sometimes leads
to drooling. Drooling can cause severe skin irritation and, because it is
socially unacceptable, can lead to further isolation of affected children
from their peers. Although numerous treatments for drooling have been tested
over the years, there is no one treatment that always helps. Drugs called
anticholinergics can reduce the flow of saliva but may cause significant side
effects, such as mouth dryness and poor digestion. Surgery, while sometimes
effective, carries the risk of complications, including worsening of swallowing
problems. Some patients benefit from a technique called biofeedback that can
tell them when they are drooling or having difficulty controlling muscles
that close the mouth. This kind of therapy is most likely to work if the patient
has a mental age of more than 2 or 3 years, is motivated to control drooling,
and understands that drooling is not socially acceptable.
Difficulty with eating and swallowing -- also triggered by motor problems
in the mouth -- can cause poor nutrition. Poor nutrition, in turn, may make
the individual more vulnerable to infections and cause or aggravate "failure
to thrive" -- a lag in growth and development that is common among those with
cerebral palsy. To make swallowing easier, the caregiver may want to prepare
semisolid food, such as strained vegetables and fruits. Proper position, such
as sitting up while eating or drinking and extending the individual's neck
away from the body to reduce the risk of choking, is also helpful. In severe
cases of swallowing problems and malnutrition, physicians may recommend tube
feeding, in which a tube delivers food and nutrients down the throat and into
the stomach, or gastrostomy, in which a surgical opening allows a tube to
be placed directly into the stomach.
A common complication is incontinence, caused by faulty control over the
muscles that keep the bladder closed. Incontinence can take the form of bed-wetting
(also known as enuresis), uncontrolled urination during physical activities
(or stress incontinence), or slow leaking of urine from the bladder. Possible
medical treatments for incontinence include special exercises, biofeedback,
prescription drugs, surgery, or surgically implanted devices to replace or
aid muscles. Specially designed undergarments are also available.
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Investigators from many arenas of medicine and health are using their expertise
to help improve treatment and prevention of cerebral palsy. Much of their
work is supported through the National Institute of Neurological Disorders
and Stroke (NINDS), the National Institute of Child Health and Human Development,
other agencies within the Federal Government, nonprofit groups such as the
United Cerebral Palsy Research Foundation, and private institutions.
The ultimate hope for overcoming cerebral palsy lies with prevention. In
order to prevent cerebral palsy, however, scientists must first understand
the complex process of normal brain development and what can make this process
go awry.
Between early pregnancy and the first months of life, one cell divides to
form first a handful of cells, and then hundreds, millions, and, eventually,
billions of cells. Some of these cells specialize to become brain cells. These
brain cells specialize into different types and migrate to their appropriate
site in the brain. They send out branches to form crucial connections with
other brain cells. Ultimately, the most complex entity known to us is created:
a human brain with its billions of interconnected neurons.
Mounting evidence is pointing investigators toward this intricate process
in the womb for clues about cerebral palsy. For example, a group of researchers
has recently observed that more than one-third of children who have cerebral
palsy also have missing enamel on certain teeth. This tooth defect can be
traced to problems in the early months of fetal development, suggesting that
a disruption at this period in development might be linked both to this tooth
defect and to cerebral palsy.
As a result of this and other research, many scientists now believe that
a significant number of children develop cerebral palsy because of mishaps
early in brain development. They are examining how brain cells specialize,
how they know where to migrate, how they form the right connections -- and
they are looking for preventable factors that can disrupt this process before
or after birth.
Scientists are also scrutinizing other events -- such as bleeding in the
brain, seizures, and breathing and circulation problems -- that threaten the
brain of the newborn baby. Through this research, they hope to learn how these
hazards can damage the newborn's brain and to develop new methods for prevention.
Some newborn infants, for example, have life-threatening problems with breathing
and blood circulation. A recently introduced treatment to help these infants
is extracorporeal membrane oxygenation, in which blood is routed from the
patient to a special machine that takes over the lungs' task of removing carbon
dioxide and adding oxygen. Although this technique can dramatically help many
such infants, some scientists have observed that a substantial fraction of
treated children later experience long-term neurological problems, including
developmental delay and cerebral palsy. Investigators are studying infants
through pregnancy, delivery, birth, and infancy, and are tracking those who
undergo this treatment. By observing them at all stages of development, scientists
can learn whether their problems developed before birth, result from the same
breathing problems that made them candidates for the treatment, or spring
from errors in the treatment itself. Once this is determined, they may be
able to correct any existing problems or develop new treatment methods to
prevent brain damage.
Other scientists are exploring how brain insults like hypoxic-ischemic encephalopathy
(brain damage from a shortage of oxygen or blood flow), bleeding in the brain,
and seizures can cause the abnormal release of brain chemicals and trigger
brain damage. For example, research has shown that bleeding in the brain unleashes
dangerously high amounts of a brain chemical called glutamate. While glutamate
is normally used in the brain for communication, too much glutamate overstimulates
the brain's cells and causes a cycle of destruction. Scientists are now looking
closely at glutamate to detect how its release harms brain tissue and spreads
the damage from stroke. By learning how such brain chemicals that normally
help us function can hurt the brain, scientists may be equipped to develop
new drugs that block their harmful effects.
In related research, some investigators are already conducting studies to
learn if certain drugs can help prevent neonatal stroke. Several of these
drugs seem promising because they appear to reduce the excess production of
potentially dangerous chemicals in the brain and may help control brain blood
flow and volume. Earlier research has linked sudden changes in blood flow
and volume to stroke in the newborn.
Low birthweight itself is also the subject of extensive research. In spite
of improvements in health care for some pregnant women, the incidence of low
birth-weight babies born each year in the United States remains at about 7
1/2 percent. Some scientists currently investigating this serious health problem
are working to understand how infections, hormonal problems, and genetic factors
may increase a woman's chances of giving birth prematurely. They are also
conducting more applied research that could yield: 1) new drugs that can safely
delay labor, 2) new devices to further improve medical care for premature
infants, and 3) new insight into how smoking and alcohol consumption can disrupt
fetal development.
While this research offers hope for preventing cerebral palsy in the future,
ongoing research to improve treatment brightens the outlook for those who
must face the challenges of cerebral palsy today. An important thrust of such
research is the evaluation of treatments already in use so that physicians
and parents have the information they need to choose the best therapy. A good
example of this effort is an ongoing NINDS-supported study that promises to
yield new information about which patients are most likely to benefit from
selective dorsal root rhizotomy, a recently introduced surgery that is becoming
increasingly in demand for reduction of spasticity.
Similarly, although physical therapy programs are a popular and widespread
approach to managing cerebral palsy, little scientific evidence exists to
help physicians, other health professionals, and parents determine how well
physical therapy works or to choose the best approach among many. Current
research on cerebral palsy aims to provide this information through careful
studies that compare the abilities of children who have had physical and other
therapy with those who have not.
As part of this effort, scientists are working to create new measures to
judge the effectiveness of treatment, as in ongoing research to precisely
identify the specific brain areas responsible for movement may yield one such
approach. Using magnetic pulses, researchers can locate brain areas that control
specific actions, such as raising an arm or lifting a leg, and construct detailed
maps. By comparing charts made before and after therapy among children who
have cerebral palsy, researchers may gain new insights into how therapy affects
the brain's organization and new data about its effectiveness.
Investigators are also working to develop new drugs -- and new ways of using
existing drugs -- to help relieve cerebral palsy's symptoms. In one such set
of studies, early research results suggest that doctors may improve the effectiveness
of the anti-spasticity drug called baclofen by giving the drug through spinal
injections, rather than by mouth. In addition, scientists are also exploring
the use of tiny implanted pumps that deliver a constant supply of anti-spasticity
drugs into the fluid around the spinal cord, in the hope of improving these
drugs' effectiveness and reducing side effects, such as drowsiness.
Other experimental drug development efforts are exploring the use of minute
amounts of the familiar toxin called botulinum. Ingested in large amounts,
this toxin is responsible for botulism poisoning, in which the body's muscles
become paralyzed. Injected in tiny amounts, however, this toxin has shown
early promise in reducing spasticity in specific muscles.
A large research effort is also directed at producing more effective, nontoxic
drugs to control seizures. Through its Antiepileptic Drug Development Program,
the NINDS screens new compounds developed by industrial and university laboratories
around the world for toxicity and anticonvulsant activity and coordinates
clinical studies of efficacy and safety. To date, this program has screened
more than 13,000 compounds and, as a result, five new antiepileptic drugs
-- carbamazepine, clonazepam, valproate, clorazepate, and felbamate -- have
been approved for marketing. A new project within the program is exploring
how the structure of a given antiseizure medication relates to its effectiveness.
If successful, this project may enable scientists to design better antiseizure
medications more quickly and cheaply.
As researchers continue to explore new treatments for cerebral palsy and
to expand our knowledge of brain development, we can expect significant medical
advances to prevent cerebral palsy and many other disorders that strike in
early life.
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The NINDS is the Federal Government's leading supporter of biomedical research
on brain and nervous system disorders, including cerebral palsy. The NINDS
conducts research in its own laboratories at the National Institutes of Health
in Bethesda, MD, and supports research at institutions worldwide. The Institute
also sponsors an active public information program. Other NINDS publications
that may be of interest to those concerned about cerebral palsy include "Seizures
and Epilepsy: Hope Through Research" and "The Dystonias." The Institute's
address and phone number, as well as information on other organizations that
offer various services to those affected by cerebral palsy, are provided in
the information resources section at the end of this brochure.
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- Apgar score. A numbered score doctors use to assess a
baby's physical state at the time of birth.
- apraxia. Impaired ability to carry out purposeful movements
in an individual who does not have significant motor problems.
- asphyxia. Lack of oxygen due to trouble with breathing
or poor oxygen supply in the air.
- bile pigments. Yellow-colored substances produced by
the human body as a by-product of digestion.
- cerebral. Relating to the two hemispheres of the human
brain.
- computed tomography (CT). An imaging technique that uses
X rays and a computer to create a picture of the brain's tissues and structures.
- congenital. Present at birth.
- contracture. A condition in which muscles become fixed
in a rigid, abnormal position causing distortion or deformity.
- dysarthria. Problems with speaking caused by difficulty
moving or coordinating the muscles needed for speech.
- electroencephalogram (EEG). A technique for recording
the pattern of electrical currents inside the brain.
- electromyography. A special recording technique that
detects muscle activity.
- failure to thrive. A condition characterized by lag in
physical growth and development.
- gait analysis. A technique that uses camera recording,
force plates, electromyography, and computer analysis to objectively measure
an individual's pattern of walking.
- gastrostomy. A surgical procedure to create an artificial
opening in the stomach.
- hemianopia. Defective vision or blindness that impairs
half of the normal field of vision.
- hemiparetic tremors. Uncontrollable shaking affecting
the limbs on the spastic side of the body in those who have spastic hemiplegia.
- hypertonia. Increased tone.
- hypotonia. Decreased tone.
- hypoxic-ischemic encephalopathy. Brain damage caused
by poor blood flow or insufficient oxygen supply to the brain.
- jaundice. A blood disorder caused by the abnormal buildup
of bile pigments in the bloodstream.
- magnetic resonance imaging (MRI) -- an imaging technique
which uses radio waves, magnetic fields, and computer analysis to create
a picture of body tissues and structures.
- neonatal hemorrhage. Bleeding of brain blood vessels
in the newborn.
- orthotic devices. Special devices, such as splints or
braces, used to treat problems of the muscles, ligaments, or bones of the
skeletal system.
- paresis or plegia. Weakness or paralysis. In cerebral
palsy, these terms are typically combined with another phrase that describes
the distribution of paralysis and weakness, e.g., paraparesis.
- palsy. Paralysis, or problems in the control of voluntary
movement.
- reflexes. Movements that the body makes automatically
in response to a specific cue.
- Rh incompatibility. A blood condition in which antibodies
in a pregnant woman's blood can attack fetal blood cells, impairing the
fetus's supply of oxygen and nutrients.
- rubella. Also known as German measles, rubella is a viral
infection that can damage the nervous system in the developing fetus.
- selective dorsal root rhizotomy. A surgical procedure
in which selected nerves are severed to reduce spasticity in the legs.
- spastic diplegia. A form of cerebral palsy in which both
arms and both legs are affected, the legs being more severely affected.
- spastic hemiplegia (or hemiparesis). A form of cerebral
palsy in which spasticity affects the arm and leg on one side of the body.
- spastic paraplegia (or paraparesis). A form of cerebral
palsy in which spasticity affects both legs but the arms are relatively
or completely spared.
- spastic quadriplegia (or quadriparesis). A form of cerebral
palsy in which all four limbs are affected equally.
- stereognosia. Difficulty perceiving and identifying objects
using the sense of touch.
- strabismus. Misalignment of the eyes.
- ultrasonography. A technique that bounces sound waves
off of tissues and structures and uses the pattern of echoes to form an
image, called a sonogram.
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BRAIN
Epilepsy Foundation
March of Dimes Birth
Defects Foundation
Easter Seals
United Cerebral Palsy (UCP)
Children's Hemiplegia
and Stroke Assocn. (CHASA)
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