Cerebral Palsy

Cerebral Palsy
Cerebral palsy (CP) is an umbrella term for a group of disorders affecting body movement, balance, and posture. Loosely translated, cerebral palsy means “brain paralysis.” Cerebral palsy is caused by abnormal development or damage in one or more parts of the brain that control muscle tone and motor activity (movement). The resulting impairments first appear early in life, usually in infancy or early childhood. Infants with cerebral palsy are usually slow to reach developmental milestones such as rolling over, sitting, crawling, and walking.

Common to all individuals with cerebral palsy is difficulty controlling and coordinating muscles. This makes even very simple movements difficult.
Cerebral palsy may involve muscle stiffness (spasticity), poor muscle tone, uncontrolled movements, and problems with posture, balance, coordination, walking, speech, swallowing, and many other functions.

Mental retardation, seizures, breathing problems, learning disabilities, bladder and bowel control problems, skeletal deformities, eating difficulties, dental problems, digestive problems, and hearing and vision problems are often linked to cerebral palsy.

The severity of these problems varies widely, from very mild and subtle to very profound.

Although the magnitude of the problems may wax and wane over time, the condition does not get worse over time.
Types of cerebral palsy are as follows:

Spastic (pyramidal): Increased muscle tone is the defining characteristic of this type. The muscles are stiff (spastic), and movements are jerky or awkward. This type is classified by which part of the body is affected: diplegia (both legs), hemiplegia (one side of the body), or quadriplegia (the entire body). This is the most common type of CP, accounting for about 70-80% of cases.

Dyskinetic (extrapyramidal): This includes types that affect coordination of movements. There are 2 subtypes.


Athetoid: The person has uncontrolled movements that are slow and writhing. The movements can affect any part of the body, including the face, mouth, and tongue. About 10-20% of cerebral palsy cases are of this type.
Ataxic: This type affects balance and coordination. Depth perception is usually affected. If the person can walk, the gait is probably unsteady. He or she has difficulty with movements that are quick or require a great deal of control, such as writing. About 5-10% of cases of cerebral palsy are of this type.

Mixed: This is a mixture of different types of cerebral palsy. A common combination is spastic and athetoid.
Many individuals with cerebral palsy have normal or above average intelligence. Their ability to express their intelligence may be limited by difficulties in communicating. All children with cerebral palsy, regardless of intelligence level, are able to improve their abilities substantially with appropriate interventions. Most children with cerebral palsy require significant medical and physical care, including physical, occupational, and speech/swallowing therapy.

Despite advances in medical care, cerebral palsy remains a significant health problem. The number of people affected by cerebral palsy has increased over time. This may be because more and more premature infants are surviving. In the United States, about 2-3 children per 1000 have cerebral palsy. As many as 1,000,000 people of all ages are affected. Cerebral palsy affects both sexes and all ethnic and socioeconomic groups.

Cerebral Palsy Causes

Cerebral palsy results from damage to certain parts of the developing brain.

This damage can occur early in pregnancy when the brain is just starting to form, during the birth process as the child passes through the birth canal, or after birth in the first few years of life.

In many cases, the exact cause of the brain damage is never known.
At one time, problems during birth, usually inadequate oxygen, were blamed for cerebral palsy.

We now know that fewer than 10% of cases of cerebral palsy begin during birth (perinatal).

In fact, current thinking is that at least 70-80% of cases of cerebral palsy begin before birth (prenatal).

Some cases begin after birth (postnatal).

In all likelihood, many cases of cerebral palsy are a result of a combination of prenatal, perinatal, and postnatal factors.
Risk factors linked with cerebral palsy include the following:

Infection, seizure disorder, thyroid disorder, and/or other medical problems in the mother

Birth defects, especially those affecting the brain, spinal cord, head, face, lungs, or metabolism

Rh factor incompatibility, a difference in the blood between mother and fetus that can cause brain damage in the fetus (Fortunately, this is almost always detected and treated in women who receive proper prenatal medical care.)

Certain hereditary and genetic conditions

Complications during labor and delivery

Premature birth

Low birth weight (especially if less than 2 pounds at birth)

Severe jaundice after birth

Multiple births (twins, triplets)

Lack of oxygen (hypoxia) reaching the brain before, during, or after birth

Brain damage early in life, due to infection (such as meningitis), head injury, lack of oxygen, or bleeding

Cerebral Palsy Symptoms

The signs of cerebral palsy are usually not noticeable in early infancy but become more obvious as the child’s nervous system matures. Early signs include the following:

Delayed milestones such as controlling head, rolling over, reaching with one hand, sitting without support, crawling, or walking

Persistence of “infantile” or “primitive” reflexes, which normally disappear 3-6 months after birth

Developing handedness before age 18 months: This indicates weakness or abnormal muscle tone on one side, which may be an early sign of CP.
Problems and disabilities related to CP range from very mild to very severe. Their severity is related to the severity of the brain damage. They may be very subtle, noticeable only to medical professionals, or may be obvious to the parents and other caregivers.

Abnormal muscle tone: Muscles may be very stiff (spastic) or unusually relaxed and “floppy.” Limbs may be held in unusual or awkward positions. For example, spastic leg muscles may cause legs to cross in a scissor-like position.

Abnormal movements: Movements may be unusually jerky or abrupt, or slow and writhing. They may appear uncontrolled or without purpose.

Skeletal deformities: People who have cerebral palsy on only one side may have shortened limbs on the affected side. If not corrected by surgery or a device, this can lead to tilting of the pelvic bones and scoliosis (curvature of the spine).

Joint contractures: People with spastic cerebral palsy may develop severe stiffening of the joints because of unequal pressures on the joints exerted by muscles of differing tone or strength.

Mental retardation: Some, although not all, children with cerebral palsy are affected by mental retardation. Generally, the more severe the retardation, the more severe the disability overall.

Seizures: About one third of people with cerebral palsy have seizures. Seizures may appear early in life or years after the brain damage that causes cerebral palsy. The physical signs of a seizure may be partly masked by the abnormal movements of a person with cerebral palsy.

Speech problems: Speech is partly controlled by movements of muscles of the tongue, mouth, and throat. Some individuals with cerebral palsy are unable to control these muscles and thus cannot speak normally.

Swallowing problems: Swallowing is a very complex function that requires precise interaction of many groups of muscles. People with cerebral palsy who are unable to control these muscles will have problems sucking, eating, drinking, and controlling their saliva. They may drool. An even greater risk is aspiration, the inhalation into the lungs of food or fluids from the mouth or nose. This can cause infection or even suffocation.

Hearing loss: Partial hearing loss is not unusual in people with cerebral palsy. The child may not respond to sounds or may have delayed speech.

Vision problems: Three quarters of people with cerebral palsy have strabismus, which is the turning in or out of one eye. This is due to weakness of the muscles that control eye movement. These people are often nearsighted. If not corrected, strabismus can lead to more severe vision problems over time.

Dental problems: People with cerebral palsy tend to have more cavities than usual. This results from both defects in tooth enamel and difficulties brushing the teeth.

Bowel and/or bladder control problems: These are caused by lack of muscle control.

when to Seek Medical Care

If your child was born prematurely, had a low birth weight, or was subject to certain complications of pregnancy, labor, or delivery, he or she will be monitored carefully over time for signs of CP. Any of the following warrant a visit to your child’s health care provider:

Your child has a seizure.
Your child’s movements seem unusually jerky, abrupt, uncoordinated, or slow and writhing.
Your child’s muscles seem unusually tense or, on the other hand, limp and “floppy.”
Your child does not blink in response to loud noises by age 1 month.
Your child does not turn his or her head toward a sound by age 4 months.
Your child does not reach out for a toy by age 4 months.
Your child does not sit up unsupported by age 7 months.
Your child does not say words by age 12 months.
Your child develops left- or right-handedness before age 12 months.
Your child has strabismus (one eye turned inward or outward).
Your child does not walk or walks with a stiff or abnormal gait, such as toe-walking.
These are only some of the most obvious examples of problems that may signal CP. You should speak to your child’s health care provider about any problems that suggest a lack of control or muscles or movements.

Exams and Tests

If your child has problems that suggest cerebral palsy, he or she will undergo a very thorough evaluation. There is no medical test that confirms the diagnosis of cerebral palsy. The diagnosis is made on the basis of various types of information gathered by the child’s health care provider and, in some cases, other consultants.

This information includes a detailed medical interview concerning medical histories of both the mother’s and father’s families, the mother’s medical problems before and during pregnancy, and a detailed account of the pregnancy, labor, delivery, and neonatal (newborn) period.

You will be asked to relate in detail the child’s medical problems and mental and physical development.

You may be asked other questions as well. It is very important to answer all questions as completely and honestly as possible, as the answers may help your child.
Lab studies: Various blood and urine tests may be ordered if your child’s health care provider suspects that the child’s difficulties are due to chemical, hormonal, or metabolic problems. Analysis of the child’s chromosomes, including karyotype analysis and specific DNA testing, may be needed to rule out a genetic syndrome.

Imaging studies: These studies provide a picture of structures inside the body. Such testing, when used on the brain or spinal cord, is often called neuroimaging. These tests are not always necessary, but in many cases, they may help identify the cause or extent of the cerebral palsy. They should be done as early as possible so that appropriate treatment, if indicated, can be begun immediately. Many individuals with mild cerebral palsy have no visible brain abnormalities.
Ultrasound of the brain: Ultrasound uses harmless sound waves to detect certain types of structural and anatomic abnormalities. For instance, it can show hemorrhage (bleeding) in the brain or damage caused by lack of oxygen to the brain. Ultrasound is often used on newborns who cannot tolerate more rigorous tests such as CT scans or MRI.

CT scan of the brain: This scan is similar to an x-ray but shows greater detail and gives a more 3-dimensional image. It identifies malformations, hemorrhage, and certain other abnormalities in infants more clearly than ultrasound.

MRI of the brain: This is the preferred test, since it defines brain structures and abnormalities more clearly than any other method. Children who are unable to remain still for at least 45 minutes may require a sedative to undergo this test.

MRI of the spinal cord: This may be necessary in children with spasticity of the legs and worsening of bowel and bladder function, which suggest an abnormality of the spinal cord. Such abnormalities may or may not be related to cerebral palsy.
Other tests: Under certain circumstances, your child’s health care provider may want to do other tests.

Electroencephalography (EEG) is important in the diagnosis of seizure disorders. A high index of suspicion is needed in order to detect non-convulsive or minimally convulsive seizures. This is a potentially treatable cause of a CP-look-alike, which is easier to treat when treated early.

Electromyography (EMG) and nerve conduction studies (NCS) may be helpful in distinguishing CP from other muscle or nerve disorders.

Cerebral Palsy Treatment

There is no cure for cerebral palsy. With early and ongoing treatment, however, the disabilities associated with cerebral palsy can be reduced. Many different therapies are available, most under the supervision of a medical specialist or other allied professional. Not all of these therapies are right for every person with cerebral palsy. The therapy regimen for a specific individual with cerebral palsy should be tailored to meet the needs of that individual. A treatment may work for one child but not for another. The parents and the child’s care team work together to choose only those treatments that offer some benefit to the child

Medical Treatment

While specific therapies help a child develop specific skills and abilities, the overall goal of treatment is to help the individual with cerebral palsy reach his or her greatest potential physically, mentally, and socially. This is accomplished with a variety of different approaches managed by a team of professionals. Care for people with cerebral palsy is complicated, requiring a number of different services and specialists. In some areas, care is available through a single multidisciplinary clinic that oversees all aspects of the child’s therapy.

Rehabilitation: A comprehensive rehabilitation program may include physical therapy, use of special equipment, and spasticity treatment. This program is often overseen by a specialist in rehabilitation medicine (sometimes called a physiatrist).
Physical therapy involves stretching, physical exercises, and other activities that develop muscle strength, flexibility, and control. The goal is to maximize function and minimize disabling contractures. The focus is on developing specific skills such as holding the head up, sitting unsupported, or walking. Braces, splints, and casts may be used to help reach these goals.

Special equipment that may be helpful to people with CP includes walkers, positioning devices, customized wheelchairs, scooters, and tricycles.

Spasticity may be treated by injections into the muscles or by medications. Reduction of spasticity can improve range of motion, reduce deformity, improve response to occupational and physical therapy, and delay the need for surgery.
Occupational therapy: The occupational therapist helps the individual learn physical skills he or she needs to function and become as independent as possible in everyday life. Examples are feeding, grooming, and dressing.

Speech/language therapy: This therapy helps the child overcome communication problems. Many children with cerebral palsy have problems speaking because of poor tone or uncontrolled movements in the muscles of the mouth and tongue. Speech therapy helps develop those muscles, improving speech. Speech therapy also benefits children with hearing loss. Children who cannot speak may be able to benefit from communication technologies such as a computerized voice synthesizer.

Vision problems: An ophthalmologist is consulted for children who have strabismus and visual problems.

Medical therapy: This encompasses treatment for all medical problems whether related to CP or not. Various specialists may be called upon to deal with specific problems.
Seizures: Seizure disorders are common in people with cerebral palsy. These are usually well controlled with medication. A specialist in conditions of the nervous system (neurologist) may be consulted for help in selecting an appropriate regimen.

Feeding and digestive problems: Individuals with cerebral palsy often have gastroesophageal reflux or GERD (severe heartburn and related symptoms caused by regurgitation of acid from the stomach) as well as swallowing and feeding problems. A team consisting of a doctor who specializes in digestive diseases (gastroenterologist), a nutritionist, and a feeding and swallowing therapist can assess nutritional status and treat problems. Swallowing therapy helps the child eat and drink independently and helps prevent aspiration. The child’s diet must be customized to accommodate limitations in swallowing. Children with severe swallowing problems require feeding through a tube.

Breathing problems: People with cerebral palsy may have breathing problems because the muscles that control expansion and contraction of the lungs are disabled. A specialist in lung disorders (pulmonologist) should be consulted for management of the resulting lung disease.
Educational services: Many children with cerebral palsy, even those of average or above-average intelligence, are challenged in “cognitive” processes such as thinking, learning, and memory. They can benefit from the services of a specialist in learning disabilities.

Such specialists can identify the child’s specific learning disabilities, direct early interventions and preparation for school, and monitor his or her progress.

In the United States, these services are provided for children younger than 3 years by an established early intervention system. Representatives of the appropriate agencies will work with you to develop an Individualized Family Services Plan, or IFSP. This plan describes the child’s needs and the services the child will receive to address those needs.

Educational services for school-aged children are provided by the public school system. The staff at your child’s school will work with you to develop an Individualized Education Plan (IEP) for your child. This may include services besides classroom teaching.
Navigating all of these different services can be difficult for parents. Your child’s health care provider can refer you to a medical social worker who can help you find and enroll in the services your child needs.

Medications

The goal of drug therapy is to reduce the effects of cerebral palsy and prevent complications. Medications are prescribed to reduce spasticity and abnormal movements and to prevent seizures.

Medications used to relieve spasticity and abnormal movements include the following:
Dopaminergic drugs: Widely used in Parkinson disease, these drugs increase the level of a brain chemical called dopamine. The effect is to decrease rigidity and abnormal movements. Examples include levodopa/carbidopa (Sinemet) and trihexyphenidyl (Artane).


Muscle relaxants: These agents reduce spasticity by relaxing the muscle directly. Examples include baclofen (Lioresal). This drug can be taken as a pill or be administered automatically via an implantable pump.


Benzodiazepines: These agents act on brain chemistry to relax muscles. The most widely used of these agents is diazepam (Valium).


Botulinum toxin type A: This substance is widely known as BOTOX®. When injected, it causes a mild muscle paralysis and reduces contractions. In cerebral palsy, it is used to decrease spasticity of muscles of the arms or legs, which improves range of motion and overall mobility. This can be important in allowing a child to fit into an orthotic (brace or splint) or even to be comfortably positioned in a wheelchair. The effects of BOTOX® injections typically last 3-6 months. BOTOX® helps other treatments work better, such as physical therapy or casting the limb. In some cases, using BOTOX® can delay surgery or make surgery unnecessary. Some people have allergic-type reactions to BOTOX® and must limit the number of injections or stop them altogether.
Medications used to relieve seizures include the following:

Anticonvulsants: These agents stop seizure activity as rapidly as possible and prevent seizure recurrence. There are many difference agents available; they vary in their mechanism of action.


Benzodiazepines: Agents such as diazepam often are used to stop seizures when they are frequent or prolonged.

Fever

Fever


A fever is usually a sign that something out of the ordinary is going on in your body. For an adult, a fever may be uncomfortable, but fever usually isn't dangerous unless it reaches 103 F (39.4 C) or higher. For very young children and infants, a slightly elevated temperature may indicate a serious infection.



But the degree of fever doesn't necessarily indicate the seriousness of the underlying condition. A minor illness may cause a high fever, and a more serious illness may cause a low fever.



Usually a fever goes away within a few days. A number of over-the-counter medications lower a fever, but sometimes it's better left untreated. Fever seems to play a key role in helping your body fight off a number of infections.



Symptoms

By Mayo Clinic staff



You have a fever when your temperature rises above its normal range. What's normal for you may be a little higher or lower than the average normal temperature of 98.6 F (37 C).



Depending on what's causing your fever, additional fever signs and symptoms may include:

Sweating

Shivering

Headache

Muscle aches

Loss of appetite

Dehydration

General weakness



High fevers between 103 F (39.4 C) and 106 F (41.1 C) may cause:

Hallucinations

Confusion

Irritability

Convulsions

Dehydration



When to see a doctor

Fevers by themselves may not be a cause for alarm — or a reason to call a doctor. Yet there are some circumstances when you should seek medical advice for your baby, your child or yourself.



Taking a temperature

To check your or your child's temperature, you can choose from several types of thermometers, including oral, rectal and ear (tympanic) thermometers.



Although it's not the most accurate way to take a temperature, you can use an oral thermometer for an armpit (axillary) reading:

Place the thermometer in the armpit and cross your arms or your child's arms over the chest.

Wait four to five minutes. The axillary temperature is slightly lower than an oral temperature.

If you call your doctor, report the actual number on the thermometer and where on the body you took the temperature.



Use a rectal thermometer for infants:

Place a dab of petroleum jelly on the bulb.

Lay your baby on his or her tummy.

Carefully insert the bulb one-half inch to one inch into your baby's rectum.

Hold the bulb and your baby still for three minutes.

Don't let go of the thermometer while it's inside your baby. If your baby squirms, the thermometer could go deeper and cause an injury.



Infants

An unexplained fever is greater cause for concern in infants and in children than in adults. Call your baby's doctor if your baby has a fever of 101 F (38.3 C) or higher. Also call your baby's doctor if your baby:

Has a fever and is younger than 3 months of age.

Refuses to eat or drink.

Has a fever and unexplained irritability, such as marked crying during a diaper change or when moved.

Has a fever and seems lethargic and unresponsive. In infants and children younger than age 2, these may be signs of meningitis — an infection and inflammation of the membranes and fluid surrounding the brain and spinal cord. If you're worried that your baby might have meningitis, take your baby to the doctor right away.

Is a newborn and has a lower than normal temperature — less than 97 F (36.1 C). Very young babies may not regulate their body temperature well when they are ill and may become cold rather than hot.



Children

There's probably no cause for alarm if your child has a fever but is responsive — making eye contact with you and responding to your facial expressions and to your voice — and is drinking fluids and playing.



Call your child's doctor if your child:

Is listless or irritable, vomits repeatedly, has a severe headache or stomachache, or has any other symptoms causing significant discomfort.

Has a fever after being left in a hot car. Seek medical care immediately.

Has a fever that persists longer than a day (in children younger than age 2) or longer than three days (in children ages 2 and older).



Ask your child's doctor for guidance in special circumstances, such as a child with immune system problems or with a pre-existing illness. Your child's doctor also may recommend precautions if your child has just started taking a new prescription medicine.



Adults

Call your doctor if:

Your temperature is more than 103 F (39.4 C)

You've had a fever for more than three days



In addition, seek immediate medical attention if any of these signs or symptoms accompanies a fever:

Severe headache

Severe throat swelling

Unusual skin rash, especially if the rash rapidly worsens

Unusual sensitivity to bright light

Stiff neck and pain when you bend your head forward

Mental confusion

Persistent vomiting

Difficulty breathing or chest pain

Extreme listlessness or irritability

Abdominal pain or pain when urinating

Any other unexplained signs or symptoms



Causes

By Mayo Clinic staff



Your normal body temperature varies throughout the day — it's lower in the morning and higher in the late afternoon and evening. In fact, your normal temperature can range from about 97 F (36.1 C) to 99 F (37.2 C). Although most people consider 98.6 F (37 C) normal, your temperature may vary by a degree or more. Other factors, such as your menstrual cycle or heavy exercise, can affect your temperature.



A fever might be caused by:

A virus

A bacterial infection

Heat exhaustion

Extreme sunburn

Certain inflammatory conditions such as rheumatoid arthritis — inflammation of the lining of your joints (synovium)

A malignant tumor

Some medications, such as antibiotics and drugs used to treat high blood pressure or seizures

Some immunizations, such as the diphtheria, tetanus and acellular pertussis (DTaP) or pneumococcal vaccines



Sometimes it's not possible to identify the cause of a fever. If you have a temperature of 101 F (38.3 C) or higher for more than three weeks and your doctor isn't able to find the cause after extensive evaluation, the diagnosis may be fever of unknown origin.





Complications

By Mayo Clinic staff



Complications of a fever may include:

Severe dehydration

Hallucinations

Fever-induced seizure (febrile seizure), in a small number of children ages 6 months to 5 years



Febrile seizures

Febrile seizures usually involve loss of consciousness and shaking of limbs on both sides of the body. Although alarming for parents, the vast majority of febrile seizures cause no lasting effects.



If a seizure occurs:

Lay your child on his or her side or stomach on the floor or ground

Remove any sharp objects that are near your child

Loosen tight clothing

Hold your child to prevent injury

Don't place anything in your child's mouth or try to stop the seizure



Most seizures stop on their own. Take your child to the doctor as soon as possible after the seizure to determine the cause of the fever