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Cerebral palsy

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Cerebral palsy



Introduction 

Cerebral palsy is the general term for a number of neurological conditions that affect movement and co-ordination.

Neurological conditions are caused by problems in the brain and nervous system.

Specifically, cerebral palsy is caused by a problem in the parts of the brain responsible for controlling muscles. The condition can occur if the brain develops abnormally or is damaged before, during or shortly after birth.

Causes of cerebral palsy include:



an infection caught by the mother during pregnancy



a difficult or premature birth



bleeding in the baby’s brain



changes (mutations) in the genes that affect the brain's development



 

It is estimated that 1 in 400 people in the UK is affected by cerebral palsy.

Symptoms of cerebral palsy

The symptoms of cerebral palsy normally become apparent during the first three years of a child's life.

The main symptoms are:



muscle stiffness or floppiness



muscle weakness



random and uncontrolled body movements



balance and co-ordination problems



These symptoms can affect different areas of the body and vary in severity from person to person. Some people will only have minor problems, whereas others will be severely disabled.

Many people with cerebral palsy also have a number of associated problems, including repeated seizures or fits, drooling problems and swallowing difficulties. Some people with the condition may have communication and learning difficulties, although intelligence is often unaffected.

 

When to seek medical advice

A child with cerebral palsy may be slower in achieving important developmental goals, such as learning to crawl, walk or speak.

You should see your GP if you are concerned about your child's development. If necessary, they can refer you to a paediatrician (a doctor who specialises in the treatment of children), who can help identify any problems.

 

How cerebral palsy is treated

There is no cure for cerebral palsy. However, there are numerous treatments available, which can treat many of its symptoms and help people with the condition to be as independent as possible.

These treatments include physiotherapy, occupational therapy and medication to relieve muscle stiffness and spasms. In some cases, surgery may also be needed.

 

Outlook

Cerebral palsy is not a progressive condition. This means the original problem in the brain doesn't get worse with age, and life expectancy is usually unaffected.

However, the physical and emotional strain of living with a long-term condition such as cerebral palsy can put a great deal of stress on the body, which can cause further problems in later life.

 

Cerebral palsy


About one in 400 children born alive has cerebral palsy. An expert explains the causes, symptoms and treatment


Symptoms of cerebral palsy 

The symptoms of cerebral palsy normally become apparent during the first three years of a child's life.

A child with the condition may be slower in achieving important developmental goals, such as learning to crawl, walk or talk.

 

Main symptoms

The main symptoms largely depend on the specific form of cerebral palsy a person has.

The four main types of cerebral palsy are:



spastic cerebral palsy – when the muscles are weak and stiff (hypertonia), especially if moving them rapidly



dyskinetic cerebral palsy – when muscle tone (the unconscious ability to contract or relax muscles) varies between stiffness and floppiness (hypotonia), causing random and uncontrolled body movements (choreo-athetoid cerebral palsy), or involuntary spasms and postures (dystonic cerebral palsy) 



ataxic cerebral palsy – when a person has balance and co-ordination problems, resulting in jerky and clumsy movements; they may also experience tremors (involuntary shaking) in their hands



mixed cerebral palsy – when a person has features of more than one of the types mentioned above



The symptoms of cerebral palsy differ in severity from person to person. Some people will only have mild problems, while others will be severely disabled.

The areas affected by cerebral palsy can also vary. Some cases only affect one side of the body, some affect primarily the legs and some affect both the arms and legs.

 

Associated problems

People with cerebral palsy can also have a range of related conditions or problems, including:



repeated seizures or fits (epilepsy)



drooling and swallowing difficulties (dysphagia)



gasto-oesophageal reflux disease (GORD)



skeletal abnormalities, particularly hip dislocation or an abnormally curved spine (scoliosis)



difficulty controlling their bladder (urinary incontinence)



constipation



difficulties speaking (dysarthria)



visual impairment



hearing loss



learning difficulties (although intelligence is often unaffected)



 

When to seek medical advice

If you are concerned about your child's development, see your GP. If necessary, they can refer you to a paediatrician (a doctor who specialises in the treatment of children), who can help identify the problem.

 

Causes of cerebral palsy 

Cerebral palsy is caused by a problem in the parts of the brain responsible for controlling movement. It can occur if the brain is damaged in early life or develops abnormally, although the exact cause is not always obvious.

Parts of the brain responsible for other important functions – such as communication, hearing, vision and the ability to learn – may also be affected. This is why people with the condition can have various other problems, not just ones involving the muscles.

What causes the problems in the brain?

In the past, doctors believed that cerebral palsy was caused by brain damage sustained during birth – the direct result of being temporarily deprived of oxygen (asphyxiation). Asphyxiation can sometimes occur during a difficult or complicated birth.

However, a major research project carried out in the 1980s showed that asphyxiation was only responsible for up to 1 in 10 cases of cerebral palsy. Most were due to problems with the brain that developed before the child was born.

Researchers believe there are three main problems that can affect the brain before birth and cause cerebral palsy. These are discussed below.

 

Periventricular leukomalacia (PVL)

Periventricular leukomalacia (PVL), also known as white matter damage of immaturity, refers to damage of the brain's white matter. This part of the brain comprises many nerve fibres, which are protected by a white fatty protein, known as myelin. The white matter is responsible for directing communication between the thought-processing sections of the brain (known as grey matter) and the rest of the body.

It is thought the brain damage is caused by a reduction in the child’s blood or oxygen supply, which damages the brain cells. This has serious consequences in later life, as the white matter is responsible for transmitting signals from the brain to the muscles.

It is not clear exactly why PVL occurs, but it has been linked to:



an infection caught by the mother



the mother having abnormally low blood pressure – for example, due to a caesarean section



premature birth, especially if a child is born at 32 weeks of pregnancy or earlier



 

Abnormal development of the brain

Anything that changes or affects the brain's normal development can lead to problems with the way it transmits information to the muscles. If this happens, a child can develop cerebral palsy.

Brain development can be affected by:



changes (mutations) in the genes that play a role in the brain's development



an infection caught by the mother



trauma or injury to the unborn baby's head



Intracranial haemorrhage and stroke

Intracranial haemorrhage is bleeding in the brain. This can be dangerous because the brain can be deprived of blood, which can cause parts of the brain to die – the build-up of blood itself can damage brain tissue.

Intracranial haemorrhage is normally seen in babies born prematurely, although it sometimes occurs in unborn babies after they have had a stroke. Factors that increase the risk of an unborn baby having a stroke include:



pre-existing weaknesses or abnormalities in the baby's blood vessels or the mother’s placenta



high blood pressure in the mother



the mother having an infection during pregnancy, particularly pelvic inflammatory disease (an infection of the female upper genital tract)



 

Brain damage during or after birth

A few cases of cerebral palsy are caused by brain damage that occurs during or soon after birth.

The damage normally occurs during the first few months of a baby’s life, before the brain develops its ability to withstand and adapt to a moderate degree of damage.

Damage can be caused by asphyxiation when the child is born, an infection of the brain (such as meningitis), a particularly low blood sugar level, a serious head injury or a stroke.

 

Diagnosing cerebral palsy 

If you are concerned about your child's development, see your GP. They can refer you to a paediatrician (a doctor specialising in treating children), if necessary.

The paediatrician will ask about your child's medical history and their development. They will also study your child's reflexes, posture, movements and muscle tone.

Depending on your child's age, you may also be referred to an educational psychologist so your child's intellectual development can be assessed.

 

Tests and scans

Further tests may be recommended to rule out other problems with similar symptoms to cerebral palsy. These can include a general delay in development or a specific medical condition, such as muscular dystrophy (a group of inherited conditions that gradually weaken the muscles).

In some cases, further testing will also be able to confirm a diagnosis of cerebral palsy. This is because the condition can cause changes to the brain's structure, which can be detected by tests.

Tests your child may have include:



a magnetic resonance imaging (MRI) scan, which uses radio and magnetic waves to study the brain in more detail



an ultrasound scan, which uses sound waves to build up a picture of your child's brain tissue



a computerised tomography (CT) scan, which uses a series of X-rays that are then assembled by a computer to create a detailed 3-D model of your child's brain



an electroencephalogram (EEG), where small electrodes are placed on the scalp to monitor brain activity



an electromyogram (EMG), where muscle activity and the function of the peripheral nerves (the network of nerves that run from your brain and spinal cord to other areas of the body) is tested.



blood tests



In some cases, when a baby requires special care in hospital after being born, it may be possible for a confident diagnosis of cerebral palsy to be made relatively quickly. In many cases, however, a clear diagnosis is only possible after a few months or years of screening.

It may not be possible to determine the type and severity of your child's condition until they reach four or five years old.

 

Treating cerebral palsy 

There is no cure for cerebral palsy, but a range of treatments are available to help treat many of the symptoms.

Treatment for cerebral palsy will usually involve a team of health professionals with different areas of expertise. The team may include:



a paediatrician



a health visitor



a social worker



a physiotherapist, who helps with movement and co-ordination



an orthotist, who specialises in the use of devices (orthoses) to correct deformities and support weakened joints



a speech and language therapist



an occupational therapist, who helps with the skills and abilities needed for daily activities, such as washing or dressing



a teacher specialising in helping children with visual impairment



an educational psychologist, who specialises in helping people with learning difficulties



The care team will help draw up an individual care plan to address any needs or problems your child has. The plan will be continually reassessed as your child gets older and their needs change.

You and your child will also be assigned a key worker, who will be the first point of contact between you and the various support services available. When your child is young, the key worker is likely to be a health visitor. As your child gets older and their needs become more complex, the key worker is likely to be a social worker.

There is no single treatment plan for a child with cerebral palsy. Instead, there are a wide range of treatments available, which are designed to improve your child's symptoms and let them be as independent as possible. Some of the main treatments are outlined below.

 

Physiotherapy

Physiotherapy is normally started when your child is diagnosed with cerebral palsy. It is one of the most important ways of helping your child manage their condition.

The two main goals of physiotherapy are:



to prevent the weakening of muscles that are not normally used by your child



to prevent muscles shortening and losing their normal range of movement (known as a contracture)



There is a risk of contracture in children who have problems stretching their muscles as a result of muscle stiffness. If the muscles cannot stretch, they do not grow as fast as the bones. This can lead to deformities, causing your child pain and discomfort.

A physiotherapist will teach your child a number of physical exercises to strengthen and stretch their muscles, which they can carry out every day. Special arm or leg braces (orthoses) may also be used to help stretch their muscles and improve their posture.

Speech therapy

Speech therapy can help children who have trouble communicating, by teaching them a series of exercises that can help them speak clearly.

If they have severe speech difficulties, the therapist may be able to teach them an alternative method of communication, such as sign language.

Special equipment to help your child communicate may also be available, such as a computer connected to a voice synthesizer.

Younger children can be given a device similar to a laptop that is covered with symbols of everyday objects and activities. The child then presses a combination of symbols to communicate.

 

Occupational therapy

Occupational therapy involves a therapist identifying problems your child may have carrying out everyday tasks.

They can advise your child on the best way to carry out tasks that require movement skills, such as going to the toilet or getting dressed.

Occupational therapy can be extremely useful in boosting your child's self-esteem and independence, especially as they get older.

Medications for muscle stiffness

If your child's muscles are particularly stiff, and are causing discomfort or are preventing them from doing everyday tasks, medication to relax their muscles may be offered.

 

Diazepam

If fast and short-term treatment for muscle pain and stiffness is needed, diazepam can be recommended. This medication can be taken in liquid or tablet form.

Side effects of diazepam can include:



drowsiness



slurred speech



dizziness (lightheadedness)



clumsiness or loss of co-ordination



forgetfulness



confusion



If diazepam is not effective, there are alternative muscle relaxants that can be used, such as dantrolene or tizanidine. These have similar side effects to diazepam, although regular blood tests will be needed to check for  more severe side effects, such as liver damage.

 

Baclofen

For longer-term treatment, a medication called baclofen may be recommended. Like diazepam, this can be taken in liquid or tablet form.

Side effects of baclofen can include:



drowsiness



feeling sick



constipation or diarrhoea



confusion



clumsiness or loss of co-ordination



In some cases, treatment with a baclofen pump may be recommended instead of tablets. This involves surgically implanting a small pump under the skin near the waist, which is connected through tubes to the spinal cord.

The pump delivers regular doses of baclofen directly into the fluid surrounding the spinal cord. This means it has fewer side effects than baclofen tablets and is more effective at reducing muscle stiffness.

 

Botulinum toxin

If your child has muscle stiffness affecting a specific muscle or group of muscles, injections of a medication called botulinum toxin can be offered.

The effects of botulinum toxin injections normally last between three and six months, but repeat injections can be given. The treatment is most effective when a programme of stretching and physiotherapy follows the injections.

In rare cases, botulinum toxin can cause serious swallowing and breathing difficulties. Your child's care team will explain how to recognise these problems and you will be advised to got to the hospital immediately if they develop.

 

Treating feeding and drooling problems

Children who have problems controlling their mouth will often find it difficult to swallow food and control their saliva production. Both of these can be potentially serious and require treatment.

If your child has problems swallowing their food (dysphagia), there is a risk that small pieces of food could enter their lungs. This can damage the lungs and cause an infection (pneumonia).

If your child's dysphagia is mild, it may be possible for a speech and language therapist to teach them techniques to deal with it. A diet of soft foods may also be advised.

If the problem is more serious, a feeding tube may be needed. This can be placed into their stomach, either through their nose and throat (nasogastric tube) or directly through their abdominal wall (gastrostomy tube).

If your child has drooling problems, the excess saliva can irritate the skin around the mouth, chin and neck. This will increase the risk of these areas becoming infected.

A number of treatments can help children control their drooling, including:



anticholinergic medication, given as a tablet or skin patch, which reduces the body's production of saliva



botulinum toxin injections into the salivary glands (although this is only a temporary solution)



surgery to redirect the saliva gland, so the saliva runs towards the back of the mouth rather than the front



devices placed in the mouth to encourage a better tongue position and regular swallowing



biofeedback training, where the child is taught to recognise when they are drooling and to swallow accordingly



 

Surgery

Surgery may sometimes be used to correct problems with bones and joints, by lengthening any muscles and tendons that are too short and causing problems. 

This type of surgery, known as orthopaedic surgery, may be recommended if your child's cerebral palsy is causing them pain when they move. It can also improve their posture and movement, as well as their confidence and self-esteem. 

However, it can take a while for your child to experience the full benefits of orthopaedic surgery, and a long course of physiotherapy will usually be needed after the operation. For example, if your child has surgery to improve their ability to walk, it may take up to two years for the full effects of the treatment to be achieved.

Surgery can also be used to treat other problems, including curvature of the spine (scoliosis) and urinary incontinence. See treating scoliosis and surgery for urinary incontinence for more information.

Your child will be routinely monitored and may need regular hip and spine X-rays to check for any problems that need to be corrected with surgery.

 

Selective dorsal rhizotomy (SDR)

Selective dorsal rhizotomy (SDR) is a surgical procedure that can help children with particularly severe muscle stiffness in their legs to improve their walking. It is usually only recommended if scans have shown the child has damage to the brain's white matter (periventricular leukomalacia) and other treatments for muscle stiffness have failed.

The operation involves cutting some of the nerves in the lower spinal column, which can help relieve leg stiffness.

However, extensive physiotherapy lasting several months will be needed after the operation, to help your child "relearn" how to control their leg muscles.

As with all types of surgery, the SDR procedure also carries the risk of complications, including temporary difficulty emptying the bladder (urinary retention), scoliosis and a change in the way their legs feel.

You and your child (if they are able to understand the implications of surgery) should discuss the potential benefits and risks with your surgeon.

For more information about SDR, see the guidelines from the National Institute for Health and Care Excellence (NICE) on selective dorsal rhizotomy for spasticity in cerebral palsy.

 

Complications of cerebral palsy 

The problem in the brain that causes cerebral palsy doesn't get worse with age. However, as a person with the condition grows, physical and emotional problems can develop.

 

Physical problems

Many adults with cerebral palsy develop further physical problems (such as osteoarthritis) as a result of their condition, which can cause pain, fatigue and weakness.

These problems are largely caused by the bone and muscle abnormalities associated with cerebral palsy, which can place a lot of physical stress on the body. As a result, a person with the condition can use up much more energy carrying out everyday tasks than someone who doesn't.

Further physiotherapy and equipment to help you move about, such as a wheelchair or walking frame, may help relieve some of the physical problems that can develop later on as a result of cerebral palsy.

 

Depression

The daily challenges of living with cerebral palsy can be difficult to cope with emotionally, and adults with the condition are at an increased risk of developing depression.

You should see your GP or care team if you think you may be depressed. There are several treatments that can help, such as cognitive behavioural therapy (CBT).

Talking to other people living with cerebral palsy may also help. Scope, a charity for people with cerebral palsy, operates an internet forum for people with the condition.

Scope also has more detailed information about ageing and cerebral palsy.


Cerebral palsy