What is cerebral palsy

Cerebral palsy is a group of neurological disorders caused by brain damage in the area that controls muscle tone (the motor cortex) resulting from an insult (adverse effect) in the time before birth (prenatal), during birth (perinatal) or shortly after birth (postnatal). In some cases, the motor cortex fails to develop normally in the fetus. This nervous system damage affects a person’s motor skills (ability to coordinate body movements), posture, balance and muscle tone. Cerebral palsy often shows up as either floppy or stiff muscles, or involuntary muscle movements.

Cerebral palsy is not hereditary. Depending on the damage, cerebral palsy affects people in different ways and to different extents.

Cerebral palsy can affect movement, coordination, muscle tone and posture. Cerebral palsy can also be associated with impaired vision, hearing, speech, eating and learning.

The majority of children with cerebral palsy are born with it, although it may not be detected until months or years later. Children with cerebral palsy tend to miss developmental milestones such as crawling, walking and talking. Usually, a confirmed diagnosis of cerebral palsy is made by the time a child is 2.

Cerebral palsy is a common problem, occurring in about 2 to 2.5 per 1,000 live births. The occurence of cerebral palsy is slowly increasing most probably because of the improved survival rates of pre-term infants. More than 100,000 Americans under the age of 18 years are estimated to have some degree of neurologic impairment associated with cerebral palsy.

The early signs of cerebral palsy usually appear before a child reaches 3 years of age. The most common are:

• a lack of muscle coordination when performing voluntary movements (ataxia);
• stiff or tight muscles and exaggerated reflexes (spasticity);
• walking with one foot or leg dragging;
• walking on the toes, a crouched gait, or a “scissored” gait; and
• muscle tone that is either too stiff or too floppy.

Other neurological symptoms that commonly occur in individuals with cerebral palsy include seizures, hearing loss, problems swallowing, eye muscle imbalance  (in which the eyes don’t focus on the same object) and impaired vision, bladder and bowel control issues, and pain and abnormal sensations. People with cerebral palsy also may suffer reduced range of motion at various joints of their bodies due to muscle stiffness.

Cerebral palsy’s effect on functional abilities varies greatly. Some affected people can walk while others can’t. Some people show normal or near-normal intellectual capacity, but others may have intellectual disabilities. Epilepsy, blindness or deafness also may be present.

The causes of cerebral palsy may be multiple but in most the precise cause of damage in an individual child may be difficult to determine. Current evidence suggests that events during pregnancy are responsible for 70-80% of cases of cerebral palsy. Factors such as prematurity, exposure to toxins and infections, congenital abnormalities may influence the risk of developing cerebral palsy. Maternal factors such as pre-existing disease, previous pregnancy loss, and complications during pregnancy and birth are also thought to be important.

A small number of children have cerebral palsy as the result of brain damage in the first few months or years of life, brain infections such as bacterial meningitis or viral encephalitis, or head injury from a motor vehicle accident, a fall, or child abuse. The disorder isn’t progressive, meaning that the brain damage typically doesn’t get worse over time. Risk factors associated with cerebral palsy do not cause the disorder but can increase a child’s chance of being born with the disorder.

If you think your child is showing some of the symptoms of cerebral palsy, or their development may be delayed, see your early childhood nurse or healthcare provider.

Types of cerebral palsy

There are four main types of cerebral palsy:

• Spastic cerebral palsy is characterized by increased muscle tone, resulting in stiffness of affected limbs;
• Athetoid cerebral palsy is characterized by involuntary movements;
• Ataxic cerebral palsy occurs when the cerebellum (part of the brain) has been damaged, thus causing lack of coordination and jerky movements;
• Infantile hemiparesis is characterized by seizures and one side of the body being affected more than the other.

Cerebral palsy may also be mixed, with components of 2 or more of the above. Spastic cerebral palsy is the most common type of cerebral palsy and in present in about 50% of people with cerebral palsy.

Cerebral palsy prognosis

Cerebral palsy doesn’t always cause profound disabilities and for most people with cerebral palsy the disorder have a normal life expectancy. Many children with cerebral palsy have average to above average intelligence and attend the same schools as other children their age.

On the other hand, some patients who have severe cerebral palsy have a reduced life span. The severity of the cerebral palsy and its associated complications will affect prognosis. Patients with paralysis of all four limbs (quadriplegia), severe intellectual impairment and epilepsy have a worse outcome.

Supportive treatments, medications, and surgery can help many individuals improve their motor skills and ability to communicate with the world. While one child with cerebral palsy might not require special assistance, a child with severe cerebral palsy might be unable to walk and need extensive, lifelong care.

Approximately one quarter of cerebral palsy patients will have minimal or no functional limitation. Half will be moderately impaired and although their functional capacity will be satisfactory, achieving complete independence is unlikely. The remaining quarter will be severely disabled and require full time care.

Cerebral palsy complications

Muscle weakness, muscle spasticity and coordination problems can contribute to a number of complications either during childhood or later during adulthood, including:

• Contracture. Contracture is muscle tissue shortening due to severe muscle tightening (spasticity). Contracture can inhibit bone growth, cause bones to bend, and result in joint deformities, dislocation or partial dislocation.
• Malnutrition. Swallowing or feeding problems can make it difficult for someone who has cerebral palsy, particularly an infant, to get enough nutrition. This may cause impaired growth and weaker bones. Some children may need a feeding tube for adequate nutrition.
• Mental health conditions. People with cerebral palsy may have mental health (psychiatric) conditions, such as depression. Social isolation and the challenges of coping with disabilities can contribute to depression.
• Lung disease. People with cerebral palsy may develop lung disease and breathing disorders.
• Neurological conditions. People with cerebral palsy may be more likely to develop movement disorders or worsened neurological symptoms over time.
• Osteoarthritis. Pressure on joints or abnormal alignment of joints from muscle spasticity may lead to the early onset of painful degenerative bone disease (osteoarthritis).
• Osteopenia. Fractures due to low bone density (osteopenia) can stem from several common factors such as lack of mobility, nutritional shortcomings and antiepileptic drug use.
• Eye muscle imbalance. This can affect visual fixation and tracking; an eye specialist should evaluate suspected imbalances.

Cerebral palsy causes

The cause remains unknown for most babies with cerebral palsy. There is no single cause of cerebral palsy.

The damage to the brain does not worsen with age, but it’s permanent. There is no cure. Life expectancy is normal, but the effects of cerebral palsy can cause stress to the body and premature ageing.

Cerebral palsy is caused by an abnormality or disruption in brain development, usually before a child is born. In many cases, the exact trigger isn’t known.

Factors that may lead to problems with brain development include:

• Mutations in genes that lead to abnormal brain development
• Maternal infections that affect the developing fetus
• Fetal stroke, a disruption of blood supply to the developing brain
• Infant infections that cause inflammation in or around the brain
• Traumatic head injury to an infant from a motor vehicle accident or fall
• Lack of oxygen to the brain (asphyxia) related to difficult labor or delivery, although birth-related asphyxia is much less commonly a cause than historically thought

Risk factors for cerebral palsy

A number of factors are associated with an increased risk of cerebral palsy.

Maternal health

Certain infections or health problems during pregnancy can significantly increase cerebral palsy risk to the baby. Infections of particular concern include:

• German measles (rubella). Rubella is a viral infection that can cause serious birth defects. It can be prevented with a vaccine.
• Chickenpox (varicella). Chickenpox is a contagious viral infection that causes itching and rashes, and it can cause pregnancy complications. It too can be prevented with a vaccine.
• Cytomegalovirus. Cytomegalovirus is a common virus that causes flu-like symptoms and may lead to birth defects if a mother experiences her first active infection during pregnancy.
• Herpes. Herpes infection can be passed from mother to child during pregnancy, affecting the womb and placenta. Inflammation triggered by infection may then damage the unborn baby’s developing nervous system.
• Toxoplasmosis. Toxoplasmosis is an infection caused by a parasite found in contaminated food, soil and the feces of infected cats.
• Syphilis. Syphilis is a sexually transmitted bacterial infection.
• Exposure to toxins. Exposure to toxins, such as methyl mercury, can increase the risk of birth defects.
• Zika virus infection. Infants for whom maternal Zika infection causes microcephaly can develop cerebral palsy.
• Other conditions. Other conditions may increase the risk of cerebral palsy, such as thyroid problems, intellectual disabilities or seizures.

Infant illness

Illnesses in a newborn baby that can greatly increase the risk of cerebral palsy include:

• Bacterial meningitis. This bacterial infection causes inflammation in the membranes surrounding the brain and spinal cord.
• Viral encephalitis. This viral infection similarly causes inflammation in the membranes surrounding the brain and spinal cord.
• Severe or untreated jaundice. Jaundice appears as a yellowing of the skin. The condition occurs when certain byproducts of “used” blood cells aren’t filtered from the bloodstream.

Other factors of pregnancy and birth

While the potential contribution from each is limited, additional pregnancy or birth factors associated with increased cerebral palsy risk include:

• Breech births. Babies with cerebral palsy are more likely to be in a feet-first position (breech presentation) at the beginning of labor rather than headfirst.
• Complicated labor and delivery. Babies who exhibit vascular or respiratory problems during labor and delivery may have existing brain damage or abnormalities.
• Low birth weight (birth weight less than 1,500 grams). Babies who weigh less than 5.5 pounds (2.5 kilograms) are at higher risk of developing cerebral palsy. This risk increases as birth weight drops.
• Multiple babies (twins, triplets, etc.). Cerebral palsy risk increases with the number of babies sharing the uterus. If one or more of the babies die, the chance that the survivors may have cerebral palsy increases.
• Premature birth. A normal pregnancy lasts 40 weeks. Babies born fewer than 37 weeks into the pregnancy are at higher risk of cerebral palsy. The earlier a baby is born, the greater the cerebral palsy risk.
• Rh blood type incompatibility between mother and child. If a mother’s Rh blood type doesn’t match her baby’s, her immune system may not tolerate the developing baby’s blood type and her body may begin to produce antibodies to attack and kill her baby’s blood cells, which can cause brain damage.

Cerebral palsy prevention

Most cases of cerebral palsy can’t be prevented, but you can lessen risks. If you’re pregnant or planning to become pregnant, you can take these steps to keep healthy and minimize pregnancy complications:

• Make sure you’re vaccinated. Vaccination against diseases such as rubella may prevent an infection that could cause fetal brain damage.
• Take care of yourself. The healthier you are heading into a pregnancy, the less likely you’ll be to develop an infection that may result in cerebral palsy.
• Seek early and continuous prenatal care. Regular visits to your doctor during your pregnancy are a good way to reduce health risks to you and your unborn baby. Seeing your doctor regularly can help prevent premature birth, low birth weight and infections.
• Practice good child safety. Prevent head injuries by providing your child with a car seat, bicycle helmet, safety rails on beds and appropriate supervision.

Cerebral palsy symptoms

Signs and symptoms can vary greatly. Movement and coordination problems associated with cerebral palsy may include:

• Variations in muscle tone, such as being either too stiff or too floppy
• Stiff muscles and exaggerated reflexes (spasticity)
• Stiff muscles with normal reflexes (rigidity)
• Lack of muscle coordination (ataxia)
• Tremors or involuntary movements
• Slow, writhing movements (athetosis)
• Delays in reaching motor skills milestones, such as pushing up on arms, sitting up alone or crawling
• Favoring one side of the body, such as reaching with only one hand or dragging a leg while crawling
• Difficulty walking, such as walking on toes, a crouched gait, a scissors-like gait with knees crossing, a wide gait or an asymmetrical gait
• Excessive drooling or problems with swallowing
• Difficulty with sucking or eating
• Delays in speech development or difficulty speaking
• Difficulty with precise motions, such as picking up a crayon or spoon
• Seizures

The disability associated with cerebral palsy may be limited primarily to one limb or one side of the body, or it may affect the whole body. The brain disorder causing cerebral palsy doesn’t change with time, so the symptoms usually don’t worsen with age. However, muscle shortening and muscle rigidity may worsen if not treated aggressively.

Brain abnormalities associated with cerebral palsy also may contribute to other neurological problems. People with cerebral palsy may also have:

• Difficulty with vision and hearing
• Intellectual disabilities
• Seizures
• Abnormal touch or pain perceptions
• Oral diseases
• Mental health (psychiatric) conditions
• Urinary incontinence.

Cerebral palsy diagnosis

If your family doctor or pediatrician suspects your child has cerebral palsy, he or she will evaluate your child’s signs and symptoms, review your child’s medical history, and conduct a physical evaluation. Your doctor may refer you to a specialist trained in treating children with brain and nervous system conditions (pediatric neurologist).

Your doctor will also order a series of tests to make a diagnosis and rule out other possible causes.

Brain scans

Brain-imaging technologies can reveal areas of damage or abnormal development in the brain. These tests may include the following:

• Magnetic resonance imaging (MRI). An MRI uses radio waves and a magnetic field to produce detailed 3-D or cross-sectional images of your child’s brain. An MRI can often identify any lesions or abnormalities in your child’s brain. This test is painless, but it’s noisy and can take up to an hour to complete. Your child will likely receive a mild sedative beforehand. An MRI is usually the preferred imaging test.
• Cranial ultrasound. This can be performed during infancy. A cranial ultrasound uses high-frequency sound waves to obtain images of the brain. An ultrasound doesn’t produce a detailed image, but it may be used because it’s quick and inexpensive, and it can provide a valuable preliminary assessment of the brain.

Electroencephalogram (EEG)

If your child has had seizures, your doctor may order an electroencephalogram (EEG) to determine if he or she has epilepsy, which often occurs in people with cerebral palsy. In an EEG test, a series of electrodes are affixed to your child’s scalp.

The EEG records the electrical activity of your child’s brain. If he or she has epilepsy, it’s common for there to be changes in normal brain wave patterns.

Laboratory tests

Laboratory tests may also screen for genetic or metabolic problems.

Additional tests

If your child is diagnosed with cerebral palsy, you’ll likely be referred to specialists for assessments of other conditions often associated with the disorder. These tests may identify:

• Vision impairment
• Hearing impairment
• Speech delays or impairments
• Intellectual disabilities
• Other developmental delays
• Movement disorders.

Cerebral palsy treatment

While there is no cure for cerebral palsy, many things can be done to improve the quality of the life for the person with cerebral palsy and their family. This includes the treatment of seizures with anticonvulsant medications, medications to reduce muscle stiffness and control involuntary movements.

In general, the earlier treatment begins the better chance children have of overcoming developmental disabilities or learning new ways to accomplish the tasks that challenge them. Early intervention, supportive treatments, medications, and surgery can help many individuals improve their muscle control. Treatment may include physical and occupational therapy, speech therapy, drugs to control seizures, relax muscle spasms, and alleviate pain; surgery to correct anatomical abnormalities or release tight muscles; braces and other orthotic devices; wheelchairs and rolling walkers; and communication aids such as computers with attached voice synthesizers.

Physiotherapy and massage may be important in maintaining a range of movement and muscle strength, and occupational therapy can be invaluable in helping a child affected by cerebral palsy to develop skills needed for daily living. In the case of severe muscle spasticity, surgery may be a valuable option. Tendon release procedures, usually performed by an orthopedic surgeon, allow improved range of motion in some cases.

Children and adults with cerebral palsy require long-term care with a medical care team. This team may include:

• Physical medicine and rehabilitation physician or physiatrist. A physiatrist oversees the treatment plan and medical care.
• Pediatric neurologist. A doctor trained to diagnose and treat children with brain and nervous system (neurological) disorders may be involved in your child’s care.
• Orthopedic surgeon. A doctor trained to treat muscle and bone disorders may be involved to diagnose and treat muscle conditions.
• Physical therapist. A physical therapist may help your child improve strength and walking skills, and stretch muscles.
• Occupational therapist. An occupational therapist can provide therapy to your child to develop daily skills and to learn to use adaptive products that help with daily activities.
• Speech-language pathologist. A doctor trained to diagnose and treat speech and language disorders may work with your child if your child suffers from speech, swallowing or language difficulties.
• Developmental therapist. A developmental therapist may provide therapy to help your child develop age-appropriate behaviors, social skills and interpersonal skills.
• Mental health specialist. A mental health specialist, such as a psychologist or psychiatrist, may be involved in your child’s care. He or she may help you and your child learn to cope with your child’s disability.
• Recreation therapist. Participation in art and cultural programs, sports, and other events that help children expand physical and cognitive skills and abilities. Parents of children often note improvements in a child’s speech, self-esteem and emotional well-being.
• Social worker. A social worker may assist your family to find services and plan for care transitions.
• Special education teacher. A special education teacher addresses learning disabilities, determines educational needs and identifies appropriate educational resources.

Medications

Medications that can lessen the tightness of muscles may be used to improve functional abilities, treat pain and manage complications related to spasticity or other cerebral palsy symptoms.

It’s important to talk about drug treatment risks with your doctor and discuss whether medical treatment is appropriate for your child’s needs. Medication selection depends on whether the problem affects only certain muscles (isolated) or the whole body (generalized). Drug treatments may include the following:

• Isolated spasticity. When spasticity is isolated to one muscle group, your doctor may recommend onabotulinumtoxinA (Botox) injections directly into the muscle, nerve or both. Botox injections may help to improve drooling. Your child will need injections about every three months. Side effects may include pain, mild flu-like symptoms, bruising or severe weakness. Other more-serious side effects include difficulty breathing and swallowing.
• Generalized spasticity. If the whole body is affected, oral muscle relaxants may relax stiff, contracted muscles. These drugs include diazepam (Valium), dantrolene (Dantrium) and baclofen (Gablofen). Diazepam carries some dependency risk, so it’s not recommended for long-term use. Its side effects include drowsiness, weakness and drooling. Dantrolene side effects include sleepiness, weakness, nausea and diarrhea. Baclofen side effects include sleepiness, confusion and nausea. Note that baclofen may also be pumped directly into the spinal cord with a tube. The pump is surgically implanted under the skin of the abdomen.

Your child also may be prescribed medications to reduce drooling. Medications such as trihexyphenidyl, scopolamine or glycopyrrolate (Robinul, Robinul Forte) may be helpful, as can Botox injection into the salivary glands.

Therapies

A variety of nondrug therapies can help a person with cerebral palsy enhance functional abilities:

• Physical therapy. Muscle training and exercises may help your child’s strength, flexibility, balance, motor development and mobility. You’ll also learn how to safely care for your child’s everyday needs at home, such as bathing and feeding your child. For the first 1 to 2 years after birth, both physical and occupational therapists provide support with issues such as head and trunk control, rolling, and grasping. Later, both types of therapists are involved in wheelchair assessments. Braces or splints may be recommended for your child. Some of these supports help with function, such as improved walking. Others may stretch stiff muscles to help prevent rigid muscles (contractures).
• Occupational therapy. Using alternative strategies and adaptive equipment, occupational therapists work to promote your child’s independent participation in daily activities and routines in the home, the school and the community. Adaptive equipment may include walkers, quadrupedal canes, seating systems or electric wheelchairs.
• Speech and language therapy. Speech-language pathologists can help improve your child’s ability to speak clearly or to communicate using sign language. Speech-language pathologists can also teach your child to use communication devices, such as a computer and voice synthesizer, if communication is difficult. Another communication device may be a board covered with pictures of items and activities your child may see in daily life. Sentences can be constructed by pointing to the pictures. Speech therapists may also address difficulties with muscles used in eating and swallowing.
• Recreational therapy. Some children may benefit from recreational therapies, such as therapeutic horseback riding. This type of therapy can help improve your child’s motor skills, speech and emotional well-being.

Surgical or other procedures

Surgery may be needed to lessen muscle tightness or correct bone abnormalities caused by spasticity. These treatments include:

• Orthopedic surgery. Children with severe contractures or deformities may need surgery on bones or joints to place their arms, hips or legs in their correct positions. Surgical procedures can also lengthen muscles and tendons that are proportionally too short because of severe contractures. These corrections can lessen pain and improve mobility. The procedures may also make it easier to use a walker, braces or crutches.
• Severing nerves. In some severe cases, when other treatments haven’t helped, surgeons may cut the nerves serving the spastic muscles in a procedure called selective dorsal rhizotomy. This relaxes the muscle and reduces pain, but can also cause numbness.

Coping and support

When a child is diagnosed with a disabling condition, the whole family faces new challenges. Here are a few tips for caring for your child and yourself:

• Foster your child’s independence. Encourage any effort at independence, no matter how small. Just because you can do something faster or more easily than your child doesn’t mean you should.
  Be an advocate for your child. You are an important part of your child’s health care team. Don’t be afraid to speak out on your child’s behalf or to ask tough questions of your physicians, therapists and teachers.
• Find support. A circle of support can make a big difference in helping you cope with cerebral palsy and its effects. As a parent, you may feel grief and guilt over your child’s disability. Your doctor can help you locate support groups, organizations and counseling services in your community. Your child may also benefit from family support programs, school programs and counseling.