What is cerebral palsy?

Cerebral palsy is a long term disorder causing impaired control of movement. The symptoms develop over the first few years of life, and generally do not worsen thereafter. The disorder is caused by damage to the motor control areas of the brain which disrupts the brain's ability to control movement and posture. The term cerebral refers to the brain, and the term palsy refers to impaired control of body movement.

There is a spectrum of symptoms for cerebral palsy. Someone with cerebral palsy may have difficulty with fine motor tasks, such as writing; or experience trouble with maintaining balance and walking; or be affected by involuntary movements, such as uncontrollable writhing motion of the hands or drooling. The symptoms differ from one person to the next, and may even change over time. Unfortunately people with cerebral palsy are often affected by other medical disorders, including epilepsy or mental impairment. Contrary to common belief, however, cerebral palsy does not always cause profound handicap.

Cerebral palsy is not contagious nor is it usually inherited. At this time, cerebral palsy cannot be cured, although scientific research continues to yield improved treatments.

Classification and incidence of cerebral palsy

[incidence]

There are three broad types of cerebral palsy:

• spastic
  In this form of cerebral palsy, which affects 70 – 80% of patients, the muscles are stiffly and permanently contracted. [more]
  
  Spastic cerebral palsy (and sometimes other types of cerebral palsy) is further classified by the limbs affected. In diplegia either the arms or legs are affected. In hemiplegia the arm and leg on one side of the body is affected. In quadriplegia all four limbs are affected.
  
  When both legs are affected by spasticity, the legs may turn in and cross at the knees. This causes a characteristic walking rhythm, known as the scissors gait in which the legs move awkwardly and stiffly and nearly touch at the knees.
  
  Individuals with spastic hemiplegia may also experience hemiplegic tremors, in which uncontrollable shaking affects the limbs on one side of the body. If these tremors are severe, they can seriously impair movement.

• athetoid
  This form of cerebral palsy affects some 10 – 20% of patients. It is characterised by uncontrolled, slow, writhing movements. These abnormal movements usually affect the hands, feet, arms, or legs and, in some cases, the muscles of the face and tongue, causing grimacing or drooling. The movements often increase during periods of emotional stress and disappear during sleep. Patients may also have problems co-ordinating the muscle movements needed for speech, a condition known as dysarthria.
  
  In dystonia [more]

• ataxic
  This form of cerebral palsy affects 5 – 10% of patients and involves the sense of balance and depth perception. Affected individuals often have poor co-ordination; walk unsteadily with a wide-based gait, placing their feet unusually far apart; and experience difficulty when attempting quick or precise movements, such as writing or buttoning a shirt. They may also have intention tremor. In this form of tremor, beginning a voluntary movement, such as reaching for a book, causes a trembling that affects the body part being used and that worsens as the individual gets nearer to the desired object.

It must be remembered that this is a classification, and largely represents a convenient labelling system for the medical profession. The symptoms of cerebral palsy are highly individual, and it is common for a patient to combine any of the above symptoms - to the extent that there is often a fourth type of cerebral palsy that is recognised – mixed. The most common mixed form is spasticity with athetoid movements, but other combinations are also possible.

What are the early signs of cerebral palsy?

Babies with cerebral palsy are frequently slow to reach developmental milestones, such as learning to roll over, sit, crawl, smile, or walk. This is sometimes called developmental delay.

Some affected babies have abnormal muscle tone. Decreased muscle tone is called hypotonia; the baby may seem flaccid and relaxed, even floppy. Increased muscle tone is called hypertonia, and the baby may seem stiff or rigid. In some cases, the baby has an early period of hypotonia that progresses to hypertonia after the first two to three months of life. Affected babies may also have unusual posture or favour one side of their body.

Parents who are concerned about their baby's development for any reason should contact their doctor, who can help distinguish normal variation in development from a developmental disorder.

How is cerebral palsy diagnosed?

Doctors diagnose cerebral palsy by testing a child’s motor skills and looking carefully at the child's medical history. In addition to checking for slow development, abnormal muscle tone, and unusual posture - a doctor also tests the child's reflexes and looks for early development of hand preference.

Reflexes are movements that the body makes automatically in response to a specific cue. For example, if a new-born baby is held on its back and tilted so the legs are above its head, the baby will automatically extend its arms in a gesture, called the Moro reflex, that looks like an embrace. Babies normally lose this reflex after they reach 6 months, but those with cerebral palsy may retain it for abnormally long periods. This is just one of several reflexes that a doctor can check.

Doctors can also look for hand preference -- a tendency to use either the right or left hand more often. When the doctor holds an object in front and to the side of the child, a child with hand preference will use the favoured hand to reach for the object, even when it is held closer to the opposite hand. During the first year, babies do not usually show hand preference. But babies with spastic hemiplegia, in particular, may develop a preference much earlier, since the hand on the unaffected side of their body is stronger and more useful.

The next step in diagnosing cerebral palsy is to rule out other disorders that can cause movement problems. Most important, doctors must determine that the child's condition is not getting worse. Although its symptoms may change over time, cerebral palsy is not progressive. If a child is continuously losing motor skills, the problem is more likely to be genetic diseases, muscle diseases, disorders of metabolism, or tumours in the nervous system. The child's medical history, special diagnostic tests, and, in some cases, repeated check-ups can help confirm that other disorders are not at fault.

The doctor may also order specialised scans of the brain, including CT (computerised tomography), MRI (magnetic resonance imaging), EEG (electro encephalogram), or Ultrasound, to learn more about the possible cause of cerebral palsy.

Finally, doctors may want to look for other conditions that are linked to cerebral palsy, including epilepsy, mental impairment, and vision or hearing problems.

How is cerebral palsy managed?

Cerebral palsy can not be cured, but treatment can often improve a child's capabilities. There is no standard therapy that works for all patients. Instead, the doctor must work with a team of health care professionals, first to identify a child's unique needs and impairments, and then to create an individual treatment plan that addresses these.

Some approaches that can be included in this plan are drugs to control fits and muscle spasms, special braces to compensate for muscle imbalance, surgery, mechanical aids to help overcome impairments, counselling for emotional and psychological needs, and physical, occupational, speech, and behavioural therapy. In general, the earlier treatment begins, the better chance a child has of overcoming developmental disabilities or learning new ways to accomplish difficult tasks.

The members of the treatment team for a child with cerebral palsy should be knowledgeable professionals with a wide range of specialities. A typical treatment team might include:

• A doctor, such as a paediatrician, a paediatric neurologist, or a paediatric psychiatrist, trained to help developmentally disabled children. This doctor, often the leader of the treatment team, works to synthesise the professional advice of all team members into a comprehensive treatment plan, implements treatments, and follows the patient's progress over a number of years.

• An orthopaedist, a surgeon who specialises in treating the bones, muscles, tendons, and other parts of the body's skeletal system. An orthopaedist might be called on to predict, diagnose, or treat muscle problems associated with cerebral palsy.

• A physical therapist, who designs and implements special exercise programmes to improve movement and strength.

• An occupational therapist, who can help patients learn skills for day-to-day living, school, and work.

• A speech and language therapist, who specialises in diagnosing and treating communication problems.

• A social worker, who can help patients and their families locate community assistance and education programmes.

• A psychologist, who helps patients and their families cope with the special stresses and demands of cerebral palsy. In some cases, psychologists may also oversee therapy to modify unhelpful or destructive behaviours or habits.

• An educator, who may play an especially important role when mental impairment or learning disabilities present a challenge to education.

The children who have cerebral palsy and their family or caregivers are also key members of the treatment team, and they should be closely involved in all steps of planning, making decisions, and applying treatments. Studies have shown that family support and personal determination are two of the most important predictors of which children with cerebral palsy will achieve long-term goals.

Too often, however, doctors and parents may focus primarily on an individual symptom -- especially the inability to walk. While mastering specific skills is an important focus of treatment on a day-to-day basis, the ultimate goal is to help individuals grow to adulthood and have maximum independence in society.

Physical, behavioural, and other therapies

Therapy - whether for movement, speech, or practical tasks - is the cornerstone of cerebral palsy treatment. Physical therapy should begin as soon as the diagnosis is made. Physical therapy programmes use specific sets of exercises to work toward two important goals: preventing the weakening or deterioration of muscles that can follow lack of use (called disuse atrophy) and avoiding contracture, in which muscles become fixed in a rigid, abnormal position.

Contracture is one of the most common and serious complications of cerebral palsy. Normally, a child whose bones are growing stretches the body's muscles and tendons through running and walking and other daily activities. This ensures that muscles will grow at the same rate. But in children with spastic cerebral palsy, the spasticity prevents this stretching and, as a result, muscles do not grow fast enough to keep up with lengthening bones. The resulting contracture can disrupt balance and trigger loss of previous abilities. Physical therapy alone, or in combination with special braces, works to prevent this complication by stretching spastic muscles. For example, if a child has spastic hamstrings (the tendons located behind the knee), the therapist and parents should encourage the child to sit with the legs extended to stretch them.

A third goal of some physical therapy programmes is to improve the child's motor development. A widespread programme of physical therapy that works toward this goal is the Bobath technique, named after a husband and wife team who pioneered this approach in the UK. This programme is based on the idea that the primitive reflexes retained by many children with cerebral palsy present major roadblocks to learning voluntary control. A therapist using the Bobath technique tries to counteract these reflexes by positioning the child in an opposing movement. So, for example, if a child with cerebral palsy normally keeps his arm flexed, the therapist would repeatedly extend it.

A second approach to physical therapy is "patterning," which is based on the principle that motor skills should be taught in more or less the same sequence that they develop normally. In this controversial approach, the therapist guides the child with movement problems along the path of normal motor development. For example, the child is first taught elementary movements like pulling himself to a standing position and crawling before he is taught to walk - regardless of his age. Some experts, have expressed strong reservations about the patterning approach, because studies have not established its value.

Physical therapy is usually just one element of a development programme that should include efforts to provide a varied and stimulating environment. Like all children, the child with cerebral palsy needs new experiences and interactions with the world around him in order to learn. Stimulation programmes can bring this valuable experience to the child who is physically unable to explore.

As the child with cerebral palsy approaches school age, the emphasis of therapy shifts away from early motor development. Efforts now focus on preparing the child for the classroom, helping the child master activities of daily living, and maximising the child's ability to communicate.

Physical therapy can now help the child with cerebral palsy prepare for the classroom by improving his or her ability to sit, move independently or in a wheelchair, or perform precise tasks, such as writing. In occupational therapy, the therapist works with the child to develop such skills as feeding, dressing, or toilet. This can help reduce demands on caregivers and boost self-reliance and self-esteem. For the many children who have difficulty communicating, speech therapy works to identify specific difficulties and overcome them through a programme of exercises. Speech therapy can also work to help the child learn to use special communication devices, such as a computer with voice synthesisers.

Behavioural therapy provides yet another avenue to increase a child's abilities. This therapy, which uses psychological theory and techniques, can complement physical, speech, or occupational therapy. For example, behavioural therapy might include hiding a toy inside a box to reward a child for learning to reach into the box with his weaker hand. In other cases, therapists may try to discourage unhelpful or destructive behaviours, such as hair-pulling or biting, by selectively presenting a child with rewards and praise during other, more positive activities.

As the child with cerebral palsy grows older, the need for and types of therapy and other support services will continue to change. Continuing physical therapy addresses movement problems and is supplemented by vocational training, recreation and leisure programmes, and special education when necessary. Counselling for emotional and psychological challenges may be needed at any age, but is often most critical during adolescence.

Ultimately, depending on their physical and intellectual abilities, adults with cerebral palsy may need attendant care, living accommodations, transportation, or employment opportunities.

Regardless of the patient's age and which forms of therapy are used, treatment does not end when the patient leaves the treatment centre - most of the work is often done at home. The therapist functions as a coach, providing parents and patients with the strategy and drills that can help improve performance at home, at school, and in the world. As research continues, doctors and parents can expect new forms of therapy and better information about which forms of therapy are most effective for individuals with cerebral palsy.

Drug therapy

Drugs are also sometimes used to control spasticity, particularly following surgery. The three medications that are used most often are diazepam, which acts as a general relaxant of the brain and body; baclofen, which blocks signals sent from the spinal cord to contract the muscles; and dantrolene, which interferes with the process of muscle contraction. Given by mouth, these drugs can reduce spasticity for short periods, but their value for long-term control of spasticity has not been clearly demonstrated. They may also trigger significant side effects, such as drowsiness, and their long-term effects on the developing nervous system are largely unknown. One possible solution to avoid such side effects may lie in current research to explore new routes for delivering these drugs.

Patients with athetoid cerebral palsy may sometimes be given drugs that help reduce abnormal movements. Most often, the prescribed drug belongs to a group of chemicals called anticholinergics that work by reducing the activity of acetylcholine. Acetylcholine is a chemical messenger that helps some brain cells communicate and that triggers muscle contraction. Anticholinergic drugs include trihexyphenidyl, benztropine, and procyclidine hydrochloride.

Occasionally, doctors may use alcohol "washes" -- or injections of alcohol into a muscle -- to reduce spasticity for a short period. This technique is most often used when doctors want to correct a developing contracture. Injecting alcohol into a muscle that is too short weakens the muscle for several weeks and gives doctors time to work on lengthening the muscle through bracing, therapy, or casts. In some cases, if the contracture is detected early enough, this technique may avert the need for surgery.

Surgery

Surgery is often recommended when contractures are severe enough to cause movement problems. In the operating room, surgeons can lengthen muscles and tendons that are proportionately too short. First, however, they must determine the exact muscles at fault, since lengthening the wrong muscle could make the problem worse.

Finding problem muscles that need correction can be a difficult task. To walk two strides with a normal gait requires more than 30 major muscles working at exactly the right time and exactly the right force. Furthermore, the natural adjustments the body makes to compensate for muscle problems can be misleading. A new tool that enables doctors to spot gait abnormalities, pinpoint problem muscles, and separate real problems from compensation is called gait analysis. Gait analysis combines cameras that record the patient while walking, computers that analyse each portion of the patient's gait, force plates that detect when feet touch the ground, and a special recording technique that detects muscle activity (electromyography).

Because lengthening a muscle makes it weaker, surgery for contractures is usually followed by months of recovery. For this reason, doctors try to fix all of the affected muscles at once when it is possible or, if more than one surgical procedure is unavoidable, they may try to schedule operations close together.

A second surgical technique, known as selective dorsal root rhizotomy, aims to reduce spasticity in the legs by reducing the amount of stimulation that reaches leg muscles via nerves. In the procedure, doctors try to locate and selectively sever overactivated nerves controlling leg muscles. Although there is scientific controversy over how selective this technique actually is, recent research results suggest it can reduce spasticity in some patients, particularly those who have spastic diplegia. Experimental surgical techniques include chronic cerebellar stimulation and stereotaxic thalamotomy. In chronic cerebellar stimulation, electrodes are implanted on the surface of the cerebellum -- the part of the brain responsible for co-ordinating movement -- and are used to stimulate certain cerebellar nerves. While it was hoped that this technique would decrease spasticity and improve motor function, results of this invasive procedure have been mixed. Some studies have reported improvements in spasticity and function, others have not.

Stereotaxic thalamotomy involves precise cutting of parts of the thalamus, which serves as the brain's relay station for messages from the muscles and sensory organs. This has been shown effective only for reducing hemiparetic tremors (see glossary).

Mechanical aids

Whether they are as humble as velcro shoes or as advanced as computerised communication devices, special machines and gadgets in the home, school, and workplace can help the child or adult with cerebral palsy overcome the limitations of his body.

The computer is probably the most dramatic example of a new device that can make a difference in the lives of those with cerebral palsy. For example, a child who is unable to speak or write but can make head movements may be able to learn to control a computer using a special light pointer that attaches to a headband. Equipped with a computer and voice synthesiser, this child could communicate with others. In other cases, technology has led to new versions of old devices, such as the traditional wheelchair and its modern offspring that runs on electricity.

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