Cerebral palsy (CP)

Cerebral palsy (CP) is the term used for a group of nonprogressive disorders of movement and posture caused by abnormal development of, or damage to, motor control centers of the brain. CP is caused by events before, during, or after birth. The abnormalities of muscle control that define CP are often accompanied by other neurological and physical abnormalities.

Types of Cerebral Palsy:

• Spastic cerebral palsy

Spastic hemiplegeia

A child with spastic hemiplegeia will typically have spasticity (muscle stiffness) on one side of the body – usually just a hand and arm, but may also involve a leg. The side that is affected may not develop properly. The child may have speech problems. In the majority of cases intelligence is not affected. Some children will have seizures.

Spastic diplegia

The lower limbs are affected, and there is no or little upper body spasticity. The child’s leg and hip muscles are tight. Legs cross at the knees, making walking more difficult. The crossing of the legs when the child is upright is often referred to as scissoring.

Spastic quadriplegia

The child’s legs, arms, and body are affected. This is the severest from of spastic cerebral palsy. Children with this kind of cerebral palsy are more likely to have mental retardation. Walking and talking will be difficult. Some children have seizures.

• Ataxic cerebral palsy

The child’s balance and depth perception are affected. Depth perception refers to a person’s ability to judge where objects are in relation to where he/she is. It is the least diagnosed type of cerebral palsy. The child will find it difficult to tie his/her shoelaces, button up shirts, cut with scissors, and other fine motor skills. Because of balance difficulties, the child may walk with the feet far apart. There may be intention tremors – a shaking that starts with a voluntary movement, such as reaching out for a toy, the closer he/she gets to the toy the worse the tremors become. Most children with ataxic cerebral palsy are of normal intelligence and have good communication skills. Some may have erratic speech.

• Athetoid or dyskinetic (or athetoid dyskinetic) cerebral palsy

This is the second most common type of cerebral palsy. Intelligence will nearly always be normal, but the whole body will be affected by muscle problems. Muscle tone is weak or tight – causing random and uncontrolled body movements. The child will have problems walking, sitting, maintaining posture, and speaking clearly (tongue and vocal cords are hard to control). Some children drool if they have problems controlling facial muscles.

• Hypotonic cerebral palsy

Muscle problems will appear much earlier. The baby’s head is floppy, and he/she cannot control the head when sitting up. Some parents have described their child’s movements as similar to that of a rag doll. The baby gives only a moderate amount of resistance when an adult tries to move their limbs. The baby may rest with his/her elbows and knees loosely extended, compared to other infants whose elbows/knees will be flexed. Some babies may have breathing difficulties.

Injury to the cerebellum can result in this type of cerebral palsy.

What causes cerebral palsy?

The control of muscles takes place in the cerebrum. Cerebral palsy may appear to be a muscle condition, but it is, in fact, caused by damage to the cerebrum. The cerebrum is also responsible for our memory, ability to learn, and communication skills – that is why some people with cerebral palsy have problems with communication and learning. Cerebrum damage can sometimes affect vision and hearing.

Some babies are deprived of oxygen during labor and delivery (birth). Because of this, doctors used to think that asphyxia (oxygen deprivation) during birth was the cause of the brain damage. However, scientists discovered during the 1980s that less than one tenth of cerebral palsy cases were caused by oxygen deprivation during birth. Most cases of damage to the brain among cerebral palsy children occurred before they were born – more specifically, during the first six months of pregnancy. Experts believe the brain damage happened because of three possible reasons:

Periventricular leukomalacia (PVL)

This refers to the damage of the brain’s white matter. Experts believe that lack of oxygen may have caused destruction of the unborn baby’s brain cells. PVL may have been caused by the pregnant mother catching an infection, such as rubella (German measles), having very low blood pressure, giving birth too early (premature birth), or consuming an illegal drug during the pregnancy.

Abnormal development of the brain

If the development of the brain is altered, the way it communicates with the body’s muscles can be affected, as can other functions. Experts say that any abnormality in brain development has the potential to cause cerebral palsy. During the first six months of pregnancy the embryo/fetus is particularly vulnerable to abnormal brain development. This can be caused by mutations in the genes responsible for brain development, some infections, such as toxoplasmosis (parasite infection), herpes and herpes-like viruses, and trauma to the unborn baby’s head.

Intracranial hemorrhage

This refers to bleeding inside the brain caused by the unborn baby having a stroke. Bleeding in the brain can stop the supply of blood to vital brain tissue, which consequently becomes damaged or dies. The escaped blood itself can clot and damage surrounding tissue. Several factors can cause a stroke in a baby during pregnancy and during the birth:

A blood clot in the placenta that blocked the flow of blood

The baby had a clotting disorder

There were interruptions in arterial blood flow to the baby’s brain

The mother had pre-eclampsia that was not treated

An emergency cesarean had to be performed

The second stage of labor was prolonged

A vacuum extraction was used during delivery

The baby had heart anomalies

There was inflammation of the placenta

There were umbilical cord abnormalities

The mother had a pelvic inflammatory infection

Experts say that anything which tends to cause either a premature birth or a low-weight baby who is not developed enough to cope with the stresses of life outside the womb will raise the risk of cerebral palsy. These factors may also contribute to a higher risk of cerebral palsy:

Multiple births

Damaged placenta

STDs (sexually transmitted diseases)

Consumption of alcohol by the pregnant mother

Consumption of illegal drugs by the pregnant mother

Exposure to other toxic substances by the pregnant mother

The pregnant mother did not eat properly

Random malformation of the baby’s brain

Small pelvic structure of the mother

Breech delivery

Brain damage after birth

A small proportion of cerebral palsy cases happen because of damage after birth. This could have happened because of an infection, such as meningitis, a head injury, a drowning accident, or poisoning. When damage does happen, it will do so soon after the birth. Later on in life the human brain is much more resilient and can withstand far more damage.

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 or a wide 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

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, although the shortening of muscles and muscle rigidity may worsen if not treated aggressively.

Other neurological problems:

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

Impairments also Associated with CP:

In addition to affecting muscles and motor skills, CP may cause a range of other impairments in some children. These include:

• Mental impairment (such as intellectual disability or learning disability)

• Seizures

• Vision difficulties

• Shortened limbs

• Curvature of the spine (scoliosis)

• Dental problems

• Complete or partial hearing loss

• Problems with joints (contracture)

Diagnosis:

The signs of CP are not usually noticeable at birth. Children normally progress through a predictable set of developmental milestones through the first 18 months of life. Children with CP, however, tend to develop these skills more slowly because of their motor impairments, and delays in reaching milestones are usually the first symptoms of CP. Babies with more severe cases of CP are usually diagnosed earlier than others.

Selected developmental milestones, and the ages for normally acquiring them, are given below. If a child does not acquire the skill by the age shown in parentheses, there is some cause for concern.

• sits well unsupported—6 months (8-10 months)

• babbles—six months (eight months)

• crawls—nine months (12 months)

• finger feeds, holds bottle—nine months (12 months)

• walks alone—12 months (15-18 months)

• uses one or two words other than dada/mama—12 months (15 months)

• walks up and down steps—24 months (24-36 months)

• turns pages in books; removes shoes and socks—24 months (30 months)

Children do not consistently favor one hand over the other before 12-18 months, and doing so may be a sign that the child has difficulty using the other hand. This same preference for one side of the body may show up as asymmetric crawling or, later on, favoring one leg while climbing stairs.

It must be remembered that children normally progress at somewhat different rates, and slow beginning accomplishment is often followed by normal development. Other causes for developmental delay—some benign, some serious—should be excluded before considering CP as the answer. CP is nonprogressive, so continued loss of previously acquired milestones indicates that CP is not the cause of the problem.

No one test is diagnostic for CP, but certain factors increase suspicion. The Apgar score measures a baby’s condition immediately after birth. Babies that have low Apgar scores are at increased risk for CP. Presence of abnormal muscle tone or movements may indicate CP, as may the persistence of infantile reflexes. Imaging of the brain using ultrasound, x rays, MRI, and/or CT scans may reveal a structural anomaly. Some brain lesions associated with CP include scarring, cysts, expansion of the cerebral ventricles (hydrocephalus), periventricular leukomalacia (an abnormality of the area surrounding the ventricles), areas of dead tissue (necrosis), and evidence of an intracerebral hemorrhage or blood clot. Blood and urine biochemical tests, as well as genetic tests, may be used to rule out other possible causes, including muscle and peripheral nerve diseases, mitochondrial and metabolic diseases, and other inherited disorders. Evaluations by a pediatric developmental specialist and a geneticist may be of benefit

•

Top of page

Doctor Examination

Medical History and Physical Examination

Give the doctor your child’s complete medical history. This may help to rule out other disorders that can cause movement problems, such as genetic or muscle diseases, metabolism disorders, and tumors of the nervous system.

Your doctor will evaluate your child’s muscle tone and reflexes, and may want to watch your child walk, crawl, sit, and lie down.

If your child is less than one year old, your doctor may need to wait a few months before confirming the diagnosis of cerebral palsy in order to see whether your child develops specific problems with movement.

During the physical examination, your doctor also may check for other conditions linked to CP, such as mental impairment, seizures, and vision problems.

Tests

Other conditions may have similar symptoms and need to be ruled out, such as a tumor or muscular dystrophy. The following tests will help the doctors carry out their diagnosis:

Blood tests

Cranial ultrasound –

an ultrasound scan can help doctors see an image of the child’s brain tissue

MRI (magnetic resonance image) scan –

this uses nuclear magnetic resonance of protons to produce proton density images

CT (computed tomography) scan –

a series of X-rays are compiled by the computer to create a 3-D image of the baby’s brain

When a child is two to three years old a more comprehensive diagnosis of cerebral palsy can usually be made. However, its severity is not usually fully assessed until the child is about four or five years old.

A diagnosis of cerebral palsy requires regular assessments of the child. The assessments are used to make comparisons and determine what the developmental needs and issues are. A comprehensive and confident diagnosis is possible after time has been taken to carefully assess and evaluate various factors several times.There is no specific blood test or imaging study that can make the diagnosis of cerebral palsy.

However, your doctor may order a magnetic resonance imaging (MRI) scan of your child’s brain. This test can show damage or abnormalities in the brain.

Top of page

Treatment

Individuals who have cerebral palsy often need help from their families and medical specialists throughout their lives. Many types of healthcare professionals are involved in treating those with CP. Depending upon the type of CP your child has, as well as any additional impairments, the medical treatment team may include:

• Orthopaedic surgeons to treat problems with bones, muscles, tendons, nerves or joints

• Physical therapists to improve movement and strength

• Occupational therapists to teach daily living skills, such as eating and dressing

• Speech and language specialists to treat communication problems

• Social workers to help locate community assistance and education programs

• Psychologists to help patients and families cope with stress

• Behavioral therapists to foster social and emotional development

• Other medical specialists such as eye doctors, neurologists, and nutritionists

Nonsurgical Treatment

Physical therapy.

Physical therapy may be recommended shortly after the diagnosis is made in order to help your child learn skills such as sitting, walking, or using a wheelchair. It also may help improve muscle strength, balance, and coordination, as well as prevent muscles from becoming too tight. Physical therapy may involve fun activities to tone muscles, like swimming and horseback riding.

(Left) A physical therapist teaches a child with CP to use walking sticks. (Right) A young child with CP learns how to use a harness walker.

Reproduced with permission from Sussman MD: The orthopaedic management of cerebral palsy. Orthopaedic Knowledge Online Journal 2009. Accessed August 2013

Braces, splints, and casts.

Using braces, splints, or casts may improve range of motion in joints and joint stability, prevent contracture, and improve hand or leg function. Braces can compensate for muscle imbalance.

Botox.

This drug can be injected into spastic muscles to loosen them. It is especially helpful for children younger than 5 years old, and when used in combination with casting.

Medication.

Some medications that your doctor may prescribe can control or prevent seizures or muscle spasms, ease muscle stiffness, or reduce abnormal movements.

Education:

Parents of a child newly diagnosed with CP are not likely to have the necessary expertise to coordinate the full range of care their child will need. Although knowledgeable and caring medical professionals are indispensable for developing a care plan, a potentially more important source of information and advice is other parents who have dealt with the same set of difficulties. Support groups for parents of children with CP can be significant sources of both practical advice and emotional support. Many cities have support groups that can be located through the United Cerebral Palsy Association, and most large medical centers have special multidisciplinary clinics for children with developmental disorders.

Surgical Treatment

If contractures are severe, surgery to lengthen affected muscles can improve a child’s ability to move and walk. This surgery may also help if tightly contracted muscles cause stress to joints and lead to deformities or dislocations.

Some children with CP need surgery to correctly position their arms or legs, or to correct curvature of the spine (scoliosis).

Severe spasticity and muscle stiffness may be helped with an intrathecal baclofen pump. In this procedure, a small pump is surgically implanted under the skin to deliver doses of a muscle relaxant.

If other treatments cannot effectively manage severe spasticity, your doctor may recommend selective dorsal rhizotomy. During this surgery of the spine, specific nerves that control spastic muscles are cut to help the muscles relax, as well as to relieve associated pain. This particular type of surgery is done infrequently.

Can cerebral palsy be prevented?

There are some things people can do to minimize the risk. However, in many cases cerebral palsy cannot be prevented. The pregnant mother should:

Make sure all her vaccinations are up-to-date

Go to all her antenatal appointments

Abstain from drinking alcohol throughout the whole pregnancy

Abstain from smoking throughout the whole pregnancy

Take regular exercise throughout the whole pregnancy (check with the doctor what you can do)

Eat a healthy diet throughout the whole pregnancy

Identify potential Rh incompatibility (usually for second and subsequent pregnancies)

Key terms

Asphyxia — Lack of oxygen. In the case of cerebral palsy, lack of oxygen to the brain.

Ataxia — A deficiency of muscular coordination, especially when voluntary movements are attempted, such as grasping or walking.

Athetosis — A condition marked by slow, writhing, involuntary muscle movements.

Coagulopathy — A disorder in which blood is either too slow or too quick to coagulate (clot).

Contracture — A tightening of muscles that prevents normal movement of the associated limb or other body part.

Cytokine — A protein associated with inflammation that, at high levels, may be toxic to nerve cells in the developing brain.

Diplegia — Paralysis affecting like parts on both sides the body, such as both arms or both legs.

Dorsal rhizotomy — A surgical procedure that cuts nerve roots to reduce spasticity in affected muscles.

Dyskinesia — Impaired ability to make voluntary movements.

Hemiplegia — Paralysis of one side of the body.

Hypotonia — Reduced or diminished muscle tone.

Quadriplegia — Paralysis of all four limbs.

Serial casting — A series of casts designed to gradually move a limb into a more functional position.

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

Spasticity — Increased mucle tone, or stiffness, which leads to uncontrolled, awkward movements.

Static encephalopathy — A disease of the brain that does not get better or worse.

Tenotomy — A surgical procedure that cuts the tendon of a contracted muscle to allow lengthening