Cerebral Palsy a comprehensive guide

An umbrella term covering a number of different neurological conditions, cerebral palsy is used to denote cases where there are difficulties with co-ordination and movement.

Although the brain and nervous system is involved in all neurological conditions, in cerebral palsy it’s the section of the brain which controls muscle movement which is specifically affected. The damage can arise either antenatally whether the brain doesn’t develop as it should or during birth or shortly after.

Causes

Although it has been possible to narrow down the part of the brain which is responsible for causing the symptoms of cerebral palsy, determining the exact cause of the abnormality is not always quite as clear.

The neurological abnormality may be due to an antenatal lack of development or due to a brain injury incurred either during birth or very early postnatally.

Although the primary part of the brain affected is that which is responsible for bodily moment, other areas within the brain may also be affected too. This may commonly be the areas which are responsible for controlling functions such as vision, hearing, speech, communication and the capacity to learn. This is why individuals with cerebral palsy can sometimes present with much more diverse symptoms than just a lack of co-ordination and muscle control.

There have been many theories about the exact causes of the abnormalities within the brain. In the past, there was a strong belief that the majority of cases were being caused by temporary asphyxiation during birth. This certainly can be the case as during traumatic or complicated births, asphyxia is a real risk.

But around 30 years ago a study was carried out which conclusively demonstrated that just one in 10 cases of cerebral palsy were due to a birth asphyxiation. The research revealed that the majority of cases of cerebral palsy were due to a brain which failed to properly develop in utero.

The current school of thought suggests that the majority of cases of cerebral palsy which develop antenatally are due to one of three causes:

Stroke and intracranial haemorrhage

Bleeding in the brain can be life-threatening, but can also cause parts of the brain to die due to either deprivation of blood or damage to tissue caused by am accumulation of too much blood.

Intracranial haemorrhages are a risk for infants born prematures but can also occur in foetuses who have suffered a stroke in utero. This can occur when the mother either suffers from high blood pressure, contracts an infection during pregnancy (especially pelvic inflammatory disease) or when there’s a congenital weakness in either foetus’s own blood vessels or the placenta.

Periventricular leukomalacia (PVL)

Periventricular leukomalacia, or PVL as it is more commonly known is a term used to describe damage which has occurred in the white matter of the brain. There’s a large number of nerve fibres in the region of the brain, all covered in myelin protein. The white matter is instrumental in acting as an intermediary, routing communicating between the grey matter within the brain (the area responsible for the processing of thoughts) and the body.

A reduction in the supply of oxygen or blood is believed to be the cause of this particular brain damage. The consequences of damage to the white matter can be very significant as this area of the brain controls the signals to the muscles.

The exact cause for PVL is unclear but studies suggest that it could be either seriously low blood pressure, such as during a caesarian section birth, an infection in the mother or a premature birth, especially one occurring at 32 weeks or earlier.

Abnormal brain development

A whole host of causes which impair the development of the brain prior to birth can result in cerebral palsy. This could be mutations within the genes, an infection or some kind of trauma or injury to the baby’s head while in utero.

Any impairment or injury to the part of the brain which is responsible for the transmission of messages to the muscles carries the possibility of cerebral palsy developing.

Presentation

The spectrum of symptoms associated with cerebral palsy is extremely diverse, ranging from those who are severely disabled to others who are only mildly affected.

This diversity means that not all cases are diagnosed at birth, but typically become apparent during the first three years of life.

The symptoms of cerebral palsy are often first noticed as the infant fails to develop as expected. Delays in crawling, walking and talking can often be markers of the condition. Other features which may be present include increased or decreased tone, especially in the limbs, balance difficulties and generalised muscle weakness. Involuntary movements may also be observed but an absence of these would not preclude a diagnosis.

Any failure to reach developmental goals should be discussed with the child’s physician who can make an onward referral to a paediatrician for further assessment and investigations.

The condition can present in a variety of ways, but this is typically dictated by the type of cerebral palsy. Most cases can be classified into one of four groups:

Ataxic cerebral palsy

Balance and co-ordination problems are present which manifest as clumsy and jerky movements, accompanied by tremors in the hands.

Dyskinetic cerebral palsy

Abnormal muscle tone is one of the defining presentations of this classification, typically fluctuating between rigidity and hypotonia. This can results in a lack of control over the body, causing random and unplanned movements (choreo-athetoid cerebral palsy) and/or uncontrolled and involuntary postures and spasms (dystonic cerebral palsy).

Spastic cerebral palsy

Manifested by muscles which are weakened and also more rigid (hypertonia), particularly during rapid movements.

Mixed cerebral palsy

A type of cerebral palsy where the features are a combination of the various other types.

Regardless of the sub-classification of cerebral palsy, there is the potential for a wide spectrum of variation in the severity of the symptoms. In some cases there is only a very mild presentation while in others, the symptoms are one sided, or alternately affect just the arms, or just the legs. In the most severe cases, both the arms and the legs are affected with other features or associated problems (see below).

Common complications and associated conditions

Some of the most severe problems arise not from the cerebral palsy itself, but from other conditions which present in tandem.

Some of the most common complications and associated conditions which present themselves include:

1. dysphagia
2. dysarthria
3. gastro-oesophageal reflux disease
4. epilepsy
5. scoliosis and other abnormalities of the skeleton, particularly in the pelvic region
6. urinary incontinence
7. constipation
8. impairment of vision
9. loss of hearing
10. learning difficulties (although IQ may be unaffected)

Although all of the above complications may be present in combination with the cerebral palsy, as the individual begins to grow older and ultimately age, additional difficulties may develop.

Cerebral palsy is not a degenerative condition so there’s no reason for the extent of the disability to grow worse with age. However, the presence of associated conditions such as arthritis may make the symptoms of cerebral palsy appear to grow worse.

Most individuals with cerebral palsy have substantial skeletal and muscle abnormalities which can place undue stress on other body parts. If appropriate aids aren’t used to relieve the excess stress, additional musculoskeletal problems such as back pain, knee disorders and arthritis can develop.

In addition, because of the abnormalities, it can be much harder for an individual with cerebral palsy to get around simply because of the sheer effort being ambulatory can take.

There are lots of different types of equipment which can be provided to assist with movement, and to ease the burden on the rest of the body. This can help to either delay or avoid complications in the long run but early intervention is key.

The challenges of living with cerebral palsy can be extremely difficult for the individual and it’s not uncommon for depression to develop in adulthood. There are different interventions which can help with this such as cognitive behavioural therapy or anti-depressants. Individuals affected should discuss how they are feeling with their GP so the most appropriate course of action can be taken, which may be a combination of different treatments.

Talking to others who are facing similar difficulties can also be helpful. Scope is a charity run specifically for individuals with cerebral palsy and include in their services an internet forum, which is particularly convenient for those who struggle to get out of the house.

Diagnosis

Cerebral palsy typically becomes apparent within the first three year’s of the child’s life and may be first identified when developmental delays are observed, typically with walking, talking or crawling.

The GP will refer the child to a paediatrician for further assessment if abnormalities are noted, where a full medical history will be taken and the child’s posture, movements, muscle tone and reflexes will be tested.

Children who are slightly older may be referred to an educational psychologist so their intellectual development can be checked.

The differential diagnosis could be muscular dystrophy, which differs from cerebral palsy significantly because it’s degenerative, or a more generalised condition such as a developmental delay. It’s therefore essential for investigations to be carried out to confirm or exclude other conditions.

In addition to blood tests, the investigations which may be carried out include an MRI scan, an ultrasound, a CT scan, and EEG or an EMG (which tests muscle activity and the peripheral nerves).

Diagnosing cerebral palsy can sometimes take a while, and it may not be possible to reach a definite conclusion until the child is 4 or 5 years old. This may follow an extended period of observation and repeated scans.

In other cases the diagnosis may be much more swift, typically in those cases where the baby is admitted to the SCBU after birth.

Even in children who receive an early diagnosis, it can be difficult for doctors to be able to accurately predict the extent of the disability until the child is around 5 years old.

Treatment

Cerebral palsy is an incurable condition but there are a number of different treatments and therapies available which can bring symptomatic relief or improve quality of life.

Because of the wide-ranging nature of cerebral palsy, a multi-discipline approach is typically required with a number of different healthcare professionals and doctors involved in the care. This may include some or all of the following:

1. a paediatrician
2. a social worker
3. a health visitor
4. a physiotherapist
5. a speech and language therapist
6. an orthotist
7. an occupational therapist
8. an educational psychologist (when learning difficulties are present)

Each child will have a individual care plan created which addresses their specific needs and problems. As the child grows and develops, this plan will need to be reassessed and adjusted as required.

A key worker is normally assigned to each family to act as the intermediary between the various services and the act as the first point of contact. Initially this may be the health visitor but as the child’s needs become more complex as they grow up, a social worker will normally take over.

Helping the child fulfil their potential, enjoy a good quality of life and be as independent as possible are the main goals in the treatment of cerebral palsy.

Here are some of the main types of treatment offered.

Physiotherapy

This is one of the earliest types of treatment given, typically as soon as a diagnosis is made. Physiotherapy can play a very significant role in the ongoing management of cerebral palsy.

Regardless of the type, extent or severity of the cerebral palsy, physiotherapy typically focuses on two main goals: to stop muscles contracting and becoming unable to complete their full range of movement (contracture), and to stop any weakening of the muscles, particularly those which are not strong enough to be used.

Muscle spasticity can prevent children from properly stretching their muscles which in turn can increase the risk of contracture. As the bones grow, the muscles must stretch to keep up. If they’re not able to do this, it can cause significant discomfort and pain, inhibiting movement further.

A physiotherapist can provide exercises which will stretch and strengthen the muscles at risk, which will need to be carried out daily. Orthoses for the arms and legs may also be provided to improve posture and stretch muscles further.

Occupational therapy

A great number of everyday activities require a degree of movement and this may not be as easy for a person diagnosed with cerebral palsy. An occupational therapist can provide help with tasks such as getting dressed or going to the toilet.

Occupational therapy can be vital in helping individuals to achieve as much independence as possible, and to boost self confidence.

Speech therapy

The extent of speech problems can be wide ranging from mild to severe but speech therapy can be useful for anyone with communication difficulties.

The therapist can provide a series of exercises that can individuals to vocalise more clearly. At the more severe end of the spectrum, alternative types of communication, sign language for example, can be taught by the speech therapist.

Technology can also be helpful, such as a computer and voice synthesizer.

In children too young to use a regular laptop, a computer-like device which instead uses symbols of routine activities and objects can be provided to help them communicate more effectively.

Oral difficulties

When the cerebral palsy affects the muscles in the mouth, it can be difficult for the child to control the production of saliva and also to swallow food. These complications need treatment as they have the potential to cause serious problems.

When a child is suffering from dysphagia, the risk is that tiny fragments of food could enter into the lungs, causing damage and leading to infections such as pneumonia.

For mild cases of dysphagia, a combination of a soft food diet along with techniques, which the speech and language therapist can teach, may be sufficient to manage the problems. However, for more severe cases of dysphagia, either a gastrostomy or nasogastric feeding tube may be required.

Drooling may accompany dysphagia causing irritation around the chin, neck and mouth which can go on to become infected unless action is taken. There are a number of options which can help to control drooling:

1. the reduction of the production of saliva via anticholinergic medication (tablets or skin patch)
2. saliva gland surgery, altering the flow of saliva from facing forward to backwards
3. biofeedback training to help the child to recognise drooling and to swallow as necessary
4. use of medical devices to improve tongue position and help with swallowing
5. temporary solution - injection of botulinum toxins directly into the salivary glands

Medication

Medication won’t cure cerebral palsy but it can help to provide some relief from the spasticity and rigidity which are typical features of the condition. The stiffness can not just cause discomfort and pain, but it can also prevent movement and consequently activities from being carried out.

A range of different medications can be provided as a means to ease the muscle stiffness, these include:

Diazepam

A type of benzodiazepine, Diazepam is a fast-acting medication that is useful for the short-term treatment of muscular stiffness and pain.

Available in either liquid or tablet form, diazepam is a muscle relaxant but can cause a number of side effects which includes:

1. slurring of speech
2. drowsiness
3. dizziness
4. loss of co-ordination
5. temporary dyspraxia
6. confusion
7. forgetfulness

Tizanidine and Dantrolene have a similar effect to Diazepam, with similar side effects and can be offered as an alternative if Diazepam doesn’t provide sufficient relief. These two drugs require frequent blood tests to check for more potentially serious side effects such as liver damage.

Baclofen

For relief in the longer term, Baclofen can be offered; the possible side effects include:

1. nausea
2. drowsiness
3. bowel disturbances such as diarrhoea or constipation
4. confusion
5. loss of co-ordination

Baclofen is usually offered as either a liquid or a tablet, but it can also be provided in the form of a pump. This requires the surgical implantation of a small pump, close to the wrist, under the skin which can be hooked up directly to the spinal cord via a series of tubes.

Opting for the pump allows regular doses of the drug to be delivered directly to the spinal cord fluid. This means it is not only more effective but also minimises side effects too.

Botulinum toxin

If certain muscle groups are affected by stiffness, injections of botulinum toxin may be offered. These only provide transient relief, with each injection lasting between 3-6 months. Repeat injections can be provided.

Botulinum toxin injections combined with other types of treatments such as physiotherapy provide the optimum results.

There is a small is of botulinum injections causing serious breathing and swallowing difficulties; if these occur, immediate hospital treatment is required.

Surgery

Other than operations to re-direct the saliva glands, or implant the Baclofen pump, surgery may be offered as a means of correcting deformities within the joints and bones. More specifically, lengthening tendons and muscles which are excessively short to the extent of causing serious problems.

This type of orthopaedic surgery is usually only indicated if the child experiences pain upon movement. However, when carried out, it can improve not just overall function, but also provide a sense of self-esteem and confidence too.

Despite these benefits, orthopaedic surgery is not a type of treatment which should be undertaken without serious consideration as the operation is invasive and requires a significant period for recovery, which will also require additional physiotherapy. It will also take a length of time for the full extent of the surgery to be evident. For example, corrective surgery to assist with walking could take up to two years for the effects to be seen.

Other complications of cerebral palsy which can be treated with surgery include scoliosis and urinary incontinence. There may be additional problems which accompany either of these conditions and any case where this kind of surgery is being contemplated will require a series of other investigations, such as scans of the hips and spine to see if there are any other areas which will need to be simultaneously corrected.

Selective dorsal rhizotomy (SDR)

Children who suffer from particularly excessive stiffness in their legs may be candidates for selective dorsal rhizotomy (SDR) to help improve their ability to walk. This type of surgery is normally only indicated in cases where the cerebral palsy has been caused as a result of periventricular leukomalacia (see above) and where other treatments have been already tried or ruled out.

SDR involves slicing the nerves in the lower part of the spinal column but intensive physiotherapy will be required in every case following the surgery, as the child will need to be trained how to achieve control of the muscles in their legs once more.

As might be expected with this type of surgery, there is the risk of complications which include:

1. permanent altered sensation in the legs
2. scoliosis
3. temporary urinary retention

If successful SDR can help to significant improve spasticity in the lower limbs but the potential risks and benefits of this type of surgery should be carefully weighed up in every case.