Cerebral palsy

Ataxic Cerebral Palsy, Spastic Cerebral Palsy

What is Cerebral palsy?

Cerebral palsy (CP) is a term used to describe a group of nervous system (neurological) disorders characterized by impaired body movement and muscle coordination. CP can be caused by injury to or abnormal development of the brain during pregnancy, birth or the first two to three years of life.

Motor skills are controlled primarily by structures in the middle regions of the brain, including the frontal motor cortex, basal ganglia, thalamus, cerebellum and brainstem. Damage to or impaired development of any of these structures can lead to problems with normal motor functions, movement and posture.

CP is most often present at birth and may go unnoticed until there are signs of developmental delays. Babies may appear normal at birth. However, as they age, parents may notice developmental delays (e.g., failure to sit up, roll over, crawl or walk at the normal stages) and signs of poor coordination or stiff or tight muscles.

CP is not progressive, meaning it does not worsen with time. However, a child’s symptoms can become more pronounced over time and the developmental disabilities may become more obvious. In addition, secondary symptoms such as muscle spasms may become better, worsen or remain the same over time.

Children with CP often have other disorders related to brain injury or cerebral dysfunction. These can include mental retardation, behavioral or emotional disorders, seizures and visual and speech impairments. The severity of these disorders can affect the child’s treatment and/or progress in physical, occupational and speech therapy. Generally, the earlier that therapy and other treatment begins, the better the child’s opportunity to overcome or adapt to disabilities.

According to the United Cerebral Palsy Foundation, there are between 1.5 and 2 million children and adults with CP in the United States. Some 10,000 infants and babies are diagnosed with the disorder each year. Another 1,200 to 1,500 preschool aged children are diagnosed with CP annually.

Researchers continue to investigate the causes of CP. They are focusing on the roles of early brain development, genetic defects and abnormalities in the development of the condition. Other research areas include the influence of traumatic events during gestation (e.g., fetal stroke), and evaluating therapeutic methods to develop the most effective therapy.

Types and differences of cerebral palsy

Cerebral palsy (CP) is used to describe several different neurological disorders involving body movement and motor skills. The condition may manifest in different types of movement or muscle abnormalities (palsies), depending on the area of the brain affected by the disorder.

The four major types of CP are:

  • Spastic (or pyramidal). Muscles are stiff, permanently contracted and unable to relax. Infants with spastic CP may also have periods of poor muscle tone (hypotonia). Spastic CP is the most common form, affecting 70 to 80 percent of patients with the disorder, according to the United Cerebral Palsy Foundation (UCPF). The spasticity (stiffness) and/or weakness (paresis or plegia) can affect both legs (spastic diplegia or paraplegia), the arms and legs (spastic quadriplegia), the left side of the body (left hemi-paresis), the right side (right hemi-paresis), one extremity (monoplegia) or both arms and one leg (triplegia).
  • Athetoid (or dyskinetic). According to the UCPF, this form of CP affects an estimated 10 to 20 percent of patients. It is characterized by uncontrolled, slow, writhing movements typically affecting the hands, feet, arms, legs or face and tongue muscles. Patients with athetoid CP may drool or grimace. They may also have difficulty coordinating the facial muscles needed for speech (dysarthria).
  • Ataxic. A rare form of CP that affects 5 to 10 percent of patients, according to the UCPF. Poor coordination, tremor, an unsteady walk with a wide gait and difficulty executing precise movements such as writing or buttoning a shirt are common characteristics.
  • Mixed. Some people have a combination of the three major types of CP in varying degrees. The most common mix is spastic and athetoid disorders, but other combinations are possible.

Risk factors and causes of cerebral palsy

Scientists do not completely understand what causes most cases of cerebral palsy (CP). There may be a number of factors contributing to the disorder, but researchers believe that abnormal brain development during pregnancy (prenatal) may be responsible in most cases. A small number of cases are believed to be due to injury to the brain before, during or after birth (perinatal).


Possible causes that may occur during pregnancy include:

  • Genetic or chromosomal disorders (thrombophilia) that lead to abnormal brain development.
  • Intrauterine infection (infection that occurs in the womb) in the mother, including streptoccocal, syphilis and varicella infections.
  • Systemic infection in the mother, including German measles (rubella).
  • Premature birth. Babies born prematurely are at risk for a number of complications, including bleeding in the brain (intraventricular hemorrhaging or IVH) and brain damage (e.g., periventricular leukomalacia or PVL).
  • Blood type incompatibility. Occurs when the blood Rh factor (positive or negative) between the fetus and mother are mismatched. However, this is unlikely to occur with modern prenatal care. Routine testing of Rh factor occurs during first trimester laboratory tests, and incompatibility is easily treated with immune globulin to prevent complications.

Possible causes that may occur before, during and immediately after birth include:

  • Perinatal asphyxia. Lack of oxygen to a baby’s brain (ischemia) and/or blood (hypoxia). This can be caused by a number of circumstances, including prolonged labor, umbilical cord or placental trauma, breech births, and multiple births. Multiple births are a particular risk if one twin dies in the uterus.
  • Coagulation (blood-clotting) disorders that cause fetal strokes.
  • Severe newborn jaundice (yellowing of the skin and eyes caused by excess buildup of the pigment bilirubin in the blood). Excessive amounts of bilirubin can kill brain cells in the basal ganglia (which controls motor skills) and brain stem, a condition called kernicterus.

Possible causes that may occur during the first two to three years of life include:

  • Trauma. A brain injury from a fall, accident or from child abuse such as intentional shaking (shaken baby syndrome).
  • Asphyxia. A near-drowning accident that deprives the brain of oxygen.
  • Infection. Meningitis or encephalitis that damages brain tissue or attacks the child’s central nervous system.
  • Brain tumors.

There are several factors generally recognized to increase the risk of a fetus, newborn or young child developing CP.


They include:

  • Labor and delivery complications. Any conditions that deprive the fetus or newborn of oxygen, blood or nutrients. This may include breech births, prolonged labor and physical birth defects in the newborn. Use of forceps or other instruments to pry the baby out of the birth canal increases the risk of trauma to the newborn’s brain. Placental abruption (when the placenta separates from the walls of the uterus) can disrupt the fetal oxygen supply if it occurs before birth.
  • Maternal infection (e.g., German measles, cytomegalovirus and toxoplasmosis), especially during the first 6 months of pregnancy. Uterine or birth canal infections also increase the risk of placenta inflammation (chorioamnionitis) which can impede normal fetal brain development.

Signs and symptoms of cerebral palsy

Signs and symptoms may be mild to severe and will vary depending on the type of cerebral palsy (CP) a child has. Generally, the patient will have problems coordinating muscle and body movements and posture. Children with severe cases of CP may also have other problems such as seizures and mental retardation.


Early symptoms of the disorder may include:

  • Difficulty feeding or coordinating sucking and swallowing actions.
  • Developmental delays. Parents or caregivers typically notice that infants appear to progress less rapidly than other children, exhibiting developmental delays. Most newborns can roll over by 4 or 5 months, sit without support by 6 months and walk by 14 months. A pediatrician can determine if children are reaching standard milestones for motor skill development.
  • Poor or stiff muscle tone. The baby’s head may flop over or the entire body may be flaccid or relaxed, which is an indication of lack of control over body muscles. The child may also appear rigid or stiff. In some cases, the infant may be floppy for the first two to three months followed by a period of stiffness. Some children will show signs of favoring one side of the body over another, always using the right or left hand and dragging the opposite limb.

Some children born with CP may appear normal at birth. The motor skills and muscle control problems associated with the disorder may not become evident until the child is toddler age. However, signs that a child has CP usually appear by 18 months of age.

As the child gets older, physical disabilities tend to become more obvious.


These include:

  • Dragging one or both feet
  • Walking on the toes
  • Crouching while walking
  • A scissor-like gait

Diagnosis methods for cerebral palsy

To diagnose cerebral palsy (CP), a pediatrician or pediatric neurologist will perform a complete physical examination and medical history, paying attention to prenatal development, birth and the mother’s health status during the pregnancy. A neurological examination will also be used to diagnose the condition.

During well-baby checkups (these occur at 2, 4, 6, 9 and 12 months of age), the physician performs a visual examination of the baby and checks the ability to move the neck, hands, feet, arms and legs. The physician also rotates the baby’s legs to check for hip dislocation. In addition, the physician will check several basic responses and reflexes, such as a startle response, to assess the baby’s development.

The checkups will also focus on assessing the child’s motor, speech and language and neurological (nervous system) development. The physician will focus on basic skills such as rolling over, sitting up and standing as well as the use of sounds and words.

During the examination at 12 months, the physician will inspect a child’s feet and observe the child’s gait to make sure the bones in the legs and feet are normal. The physician will assess the child’s basic motor movements, such as grasping and clapping, to determine if there are any difficulties. The physician will discuss the child’s communication skills with the parent, including the child’s ability to understand and whether or not the child is speaking. In addition, the physician will examine the child’s ears for any signs of infection and mouth for the presence of teeth.

The physician will also evaluate the child’s ability to visually track or follow objects (e.g., vision test) and generally assess the child’s hearing.

In addition to the medical history, neurological and physical examination, the physician may also order diagnostic tests for the child. These may include:


X-ray

A painless test in which an image is created of part of the body by using low doses of radiation that are reflected on film paper or fluorescent screens. An x-ray is effective at diagnosing skull fractures, although it is less effective at diagnosing specific problems within the brain. For this purpose, a computed axial tomography (CAT) scan or magnetic resonance imaging (MRI) may be used.

CAT scan

A noninvasive or minimally invasive test that uses a rotating x-ray device to create three-dimensional cross-sectional images (or slices) of different body parts, including the brain and skull. CAT scans can be used to diagnose numerous conditions and complications which may arise from a head injury, such as blood clots and skull fractures. They can also be used to guide a biopsy.

MRI

A noninvasive procedure that uses powerful magnets and radio waves to produce clear, cross-sectional or three-dimensional images of body tissues. A certain type of MRI is particularly useful in cases of head injuries because of the ability to assess how the brain is functioning. This type of MRI is called a functional MRI (fMRI). During an fMRI, the patient may be asked to perform a task while the machine scans the brain and records its activity. This information can then be used to determine which parts of the brain may be damaged.

Blood test

A sample of blood is drawn from the newborn’s heel or the veins in the arm of older children. These tests can determine possible chromosomal and genetic reasons for the child’s condition.

Urine test

A sample of the child’s urine is collected in a laboratory cup. The urine sample may identify metabolic problems.

Electroencephalogram (EEG)

Performed if the child has seizures. During an EEG, activity in the brain is monitored through electrodes that are placed on the scalp. People who have seizures may show abnormally slow frequencies or spikes and other disruptions, even when they are not having a seizure. The physician may request that the EEG be performed both while the child is sleeping and awake in order to give the most accurate picture of normal brain activity.

During the mother’s pregnancy, the treating physician routinely performs a number of diagnostic tests to monitor fetal development. These tests can be early warning signals of risk factors for CP. The tests may include:

Treatment options for cerebral palsy

There is no cure for cerebral palsy (CP), but there are several treatment options designed to improve symptoms, the child’s quality of life, physical abilities and independence. Some children with CP may require minimal physical therapy whereas others may need surgery and extensive physical therapy as well as medications to control symptoms such as seizures. The type and duration of treatment will depend on the type and severity of the motor skill dysfunction.

The child’s team of physicians and specialists may include the pediatrician and pediatric neurologist as well as an orthopedic surgeon, various physical, speech and occupational therapists, nurses, school psychologists, social workers, nutritionists and mental health professionals. Caregivers are also an integral part of any successful treatment plan.

Common treatment options for CP may include:


Oral medication

Anticonvulsants to reduce seizures and muscle relaxants to ease stiff muscles (spasticity) may be prescribed. As with any medication, parents should discuss the benefits, risks and possible side effects (e.g., sedation, hyperactivity or other cognitive impairment) with physicians prior to giving the drugs to their children. It is also important to inform the physician of any other prescription drugs, over-the-counter medications, herbal supplements or vitamins given to the child since these substances can cause drug interactions.

Intraspinal fluid catheter

Studies have shown some patients with severe symptoms experienced reduction in spasticity by administering skeletal muscle relaxant medication directly into the spinal fluid via a pump inserted into the lumbar vertebrae in the lower back. However, this treatment has been associated with complications from use of the catheter (e.g., infections) and side effects.

Intramuscular injections

Botulinum toxin (Botox) injected into affected muscles can act as a muscle relaxant to help control severe spasticity at least temporarily. Studies have shown the injections may help to lengthen muscles (which can atrophy over time) or delay their degeneration. Most of the injections are used in the calf muscles and are repeated every three to eight months. This treatment is not recommended for children under 18 months of age.

Surgery

Children with severe muscle contractures (that limit the ability to flex and fully extend limbs) may benefit from surgical procedures on muscles, tendons, nerves and joints. Depending on the severity of the dysfunction, one or multiple surgeries may be required. Surgeons may also advise parents to wait until children reach a certain minimum age before some procedures are performed, whereas others may be conducted while the child is very young. Types of surgical procedures include:

  • Muscle-tendon surgery. When joints (e.g., knees, elbows, ankles) have a restricted range of movement, surgically releasing or lengthening the tendon or muscle from the bone may improve functioning of the joint and the child’s gait.
  • Hip realignment surgery. Hip disorders (subluxation, dislocation and degeneration) that cause pain are common complications for a person with spastic CP.
  • Selective dorsal rhizotomy. Spinal cord surgery that involves identifying and cutting a small number of the lower spinal nerve roots that control the leg muscles. This helps to reduce spasticity without causing paralysis.
  • Stereotactic encephalotomy. Neurosurgery on the basal ganglia (one of the regions of the brain believed to be responsible for impaired motor function in CP) that involves inserting a probe into the brain and cutting specific tracts of nerve fibers. The procedure is only considered for children with severe muscle spasticity (dystonia).

Orthopedic aids

Braces, crutches, walkers or wheelchairs may be prescribed to aid the child’s mobility.

Ergonomic aids

Numerous devices and products are available to assist a person with physical limitations to accomplish everyday tasks. These include hand controls to operate motor vehicles, velcro ties to help grip eating utensils, and computers that can be activated and controlled with simple hand signals or by blowing on a tube.

Physical therapy

Building up muscles that may have atrophied or weakened is an important part of continued mobility for a person with CP. Regular training and exercise will improve the child’s chances of learning to walk, run, sit or stand. Since many people with CP have difficulty controlling posture and balancing, these skills are also developed during physical therapy. Experts agree this treatment must begin as soon as the child is diagnosed and may continue throughout life.

Speech therapy

Regular sessions with a speech therapist from an early age will help children whose bulbar muscles (which control the vocal cords, tongue, swallowing and chewing functions) are affected learn to properly sound out words and talk or communicate as best they can.

Occupational therapy

Learning how to function day-to-day and perform personal tasks is critical for a child with CP. These lessons include holding a pencil, spoon or fork, feeding themselves, using buttons, zippers or belts, brushing their teeth and/or grooming.

Psychotherapy

Mental health counseling for the child, siblings and parents or caregivers is an essential part of the treatment and learning to cope with the disorder.

Ongoing evaluation

The child may need periodic x-rays (on hips and other joints), blood tests and vision, hearing and speech exams to determine their progress or monitor the effect of certain medications.

Prevention methods for CP

Primary prevention methods for cerebral palsy (CP) focus on insuring a healthy pregnancy for the woman. This may include:


Prenatal care to prevent premature birth

Although there are identifiable risk factors that indicate if a woman is more likely to deliver prematurely, there are no known methods of prevention. Perhaps the most important way to lower the risk of a premature birth is to receive early and adequate prenatal care. Women may be advised to maintain a healthy weight and lifestyle (e.g., avoiding alcohol and tobacco). Rh blood factor testing is necessary during the first trimester. In addition, consuming the recommended amount of prenatal vitamins, including folic acid, may reduce the risk of premature birth, as well as birth defects and other complications. If an obstetrician feels the woman is in danger of a pre-term birth, bed rest, fluids and labor-inhibiting medications may be prescribed.

Immunizations to prevent viral and other systemic infections

Women who are planning to become pregnant may be advised to get immunized against German measles (rubella) if they have never had the disease. Pregnant women who have not been immunized may reduce the risk of exposure by limiting their contact with people who have not had the measles or other infectious viral diseases. Other infections to ward against include cytomegalovirus (CMV) and toxoplasmosis.

Practice safe sex to prevent pelvic infections

Bacterial or viral infections of the woman’s urinary tract and reproductive system (vagina and uterus) may be prevented through the use of latex condoms during sexual intercourse.

For CP that develops in the first few years of a child’s life, prevention can focus on safeguarding the home against accidental drowning (by putting fencing around backyard swimming pools) and falls (by putting child safety equipment such as toddler gates to block the top of staircases). Use of car safety seats and infant carriers when transporting children in vehicles has been shown in numerous highway safety studies to prevent serious head trauma and injury during traffic accidents.

In addition, wearing helmets while biking, skating or participating in recreational sports activities may also prevent serious head injuries. Enrolling infants and toddlers in swimming classes can also be effective prevention measures.

Lifestyle considerations for cerebral palsy

Having a child or loved one with cerebral palsy (CP) can present various psychological, social, financial and emotional challenges for the individual, family and caregivers. There is no cure for CP but the vast majority of children with the disorder live into adulthood (past 20 years of age). With surgery and physical therapy, people with mild to moderate CP can lead relatively normal and independent lives, gaining employment and driving cars. For severe cases, patients may require a lifetime of round-the-clock care.

Homes and cars may have to be customized with special equipment (e.g., wheelchairs and wheelchair lifts, ramps and handrails in hallways, stairways and bathrooms). The impact on the family life may be significant as well, especially if one parent has to stop working in order to care for the child.

Parents and family members of children with CP are often urged to seek family and individual and/or group counseling to help them cope with caregiving responsibilities. Many experience guilt associated with blaming themselves for the child’s condition. The child will also need counseling to help adapt to life as a person with disabilities who may be unable to do what other children do.


Questions for your doctor about cerebral palsy

Preparing questions in advance can help patients and parents have more meaningful discussions with healthcare professionals regarding their or their child's treatment options. The following questions related to cerebral palsy (CP) may be helpful:

  • What should I do if I suspect my baby may have CP?
  • What tests will my baby have to undergo to determine if he or she has CP?
  • What are the risks associated with the various tests?
  • Which type of CP does my child have?
  • What caused my child’s CP?
  • When will we know for certain if mental retardation accompanies my child’s condition?
  • How long will my child need physical, occupational or speech therapy?
  • Does my child require special braces and other orthopedic appliances? How long will he or she have to wear these appliances?
  • Will my child require surgery? How many operations do you anticipate he or she will need?
  • What are the chances that my child can lead a normal, independent life in adulthood? Will he or she be able to have children of their own?

Additional Information

Medications

http://www.nmihi.com/b/baclofen.html

Seizure medications

What might complicate it?

Individuals with cerebral palsy frequently experience neurological problems including epilepsy, mental retardation, learning disabilities, attention deficit hyperactivity disorder, visual impairment, swallowing difficulties, speech impairment (dysarthria), and hearing loss. Potential orthopedic problems include scoliosis, hip dislocation, muscle and joint stiffening, and discrepancy in leg length.

Predicted outcome

The severity of cerebral palsy varies widely. An individual may have high intelligence despite major muscular disability. Individuals with less severe impairment can lead productive, near-normal lives. For those with severe forms of cerebral palsy, special care may be required.

Alternatives

Differential diagnoses include spinal cord injury, spina bifida, congenital spinal cord malformation, phenylketonuria, amino-acidopathies, subdural hematoma, brain tumor, hemiparesis, Tay-Sachs disease, metachromatic leukodystrophy, and mucopolysaccharidosis.

Rehabilitation

Physical therapy, occupational therapy and speech therapy, three times a week, indefinitely.

Appropriate specialists

Neonatologist, neurologist, developmental pediatrician, child psychologist, neuropsychologist, speech therapist, occupational therapist, physical therapist, audiologist, orthopedic surgeon.