The Dystonia Society

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Cerebral palsy with dystonia (dystonic cerebral palsy)

Cerebral palsy is a general term for a group of movement and posture disorders that arise as a result of non-progressive damage to the developing brain of a foetus or infant. The symptoms of cerebral palsy are different in each person affected and will be very dependent on the extent of the damage to the developing brain. The impact on movement and other activity will also be different for each individual. Some may only be mildly affected with minimal disability whereas others may have a lot of challenges to their development. However, there is a range of medical and therapeutic support that can assist in managing the condition.

There are a number of types of cerebral palsy – the most common is spasticity which causes the muscles to be stiff and inflexible (in this case the muscle is described as having “high tone”). Spasticity is present in around 80% of cerebral palsy cases. Alternatively in ataxic cerebral palsy, the muscle can be too flexible making the child’s limb or body appear floppy (the muscle is described as having low tone). The child is unsteady in their posture and when trying to reach.

In around one in six cases, cerebral palsy causes involuntary muscle spasms and unwanted movement - this is described as dystonic cerebral palsy (or alternatively dyskinetic or choreoathetoid cerebral palsy). The symptoms of dystonic cerebral palsy are usually of muscle spasms which can be difficult to control and sometimes painful.  These are a result of incorrect signals from the brain. The spasms cause a range of different unwanted movements.

Dystonic cerebral palsy often appears in combination with other symptoms of cerebral palsy – usually the dystonic movements appear in combination with high and/or low muscle tone. For this reason, the condition is often described as cerebral palsy with dystonia.
 
Causes

Basal gangliaDystonic cerebral palsy is generally caused by damage to the basal ganglia - part of the brain involved in the recruitment of muscles of movement. Anything that changes or affects the normal development of the brain can lead to problems with the way it transmits information to the muscles, and therefore can cause cerebral palsy.

Risk factors can include problems with the placenta, oxygen loss during birth, premature delivery, low birth weight as well as some health issues affecting the mother during pregnancy including some bacterial infections and some viral diseases such as German measles. On occasion, cerebral palsy can be caused by damage to the brain that occurs within the first few weeks after birth. Exposure to any of these factors does not mean that the infant will necessarily develop cerebral palsy – but they do increase the risk.

Symptoms

No two people will be affected by dystonic cerebral palsy in the same way. A child with this type of cerebral palsy can appear restless and constantly on the move. The muscle spasms may cause twisting, repetitive movements or abnormal postures (dystonia) abrupt, irregular, jerky movements (chorea) or slower sinuous movements (athetosis) or sometimes a combination of these movements (choreoathetosis). The child may also have difficulty holding him / herself upright when sitting or walking.

The child may also have difficulty controlling movements of the face, tongue, lips, jaw, throat and muscles of respiration. This will affect someone’s facial expressions, speech, eating, drinking and saliva control.

The abnormal movements often increase with raised emotions, such as excitement or stress.  Relaxation can help reduce these symptoms. The movements usually disappear completely when a person is asleep.

An assessed scoring scale can be used to determine the impact of cerebral palsy on the child’s motor function at any age (motor function means the ability to use hands, arms and legs as well as the ability to sit). The scale is called Gross Motor Function Classification Score (GMFCS). The GMFCS ranges from 1 (limited impact) to 5 (severe impact). Using the GMFCS, it is possible to project forwards to estimate how the cerebral palsy may affect the child at a later age.

There are other function classification systems that may be used these include the Manual Ability Classification System; Communication Function Classification System and Eating and Drinking Ability Classification System. It is important to know which system is being used and how your child’s development is being measured.

Intelligence may or may not also be affected depending on whether there is damage to the parts of the brain that controls intelligence.

Therapeutic support

Click here for more information on therapies for dystonic cerebral palsy.

Treatment for all forms of cerebral palsy is focussed on enabling the child to live as normal a life as possible. Once a diagnosis has been made (often by the local community disability team) the child is referred to a team of health care professionals who have experience in planning and delivering care to children and young people with cerebral palsy.

This team will usually include a number of healthcare professionals including developmental paediatricians, physiotherapists, occupational therapists, speech and language therapists and nurses.  Regular assessments and reviews are used to monitor progress and identify problems early. These should always involve the patient and the family.  

Click to read more about different types of therapeutic support for dystonic cerebral palsy.

Therapies can help with a range of daily activites including (click for information):

Treatments

Click here for more information on treatments for dystonic cerebral palsy.

Medication is often used to manage the muscle spasms and pain. If your child's muscles are particularly stiff and overactive, they may need medication to help relax their muscles. As many people with cerebral palsy experience a lot of pain associated with gastrointestinal problems including gastric reflux and reduced gut motility causing constipation. It is important to treat pain and discomfort such as that caused by gastric reflux and constipation as these symptoms can aggravate the symptoms of dystonia. Click here to read more about medication.

Other treatments

Other treatments for dystonic cerebral palsy are (click to read more):

Deep Brain Stimulation is not usually effective for those with athetoid or dystonic cerebral palsy but is sometimes appropriate.

Multidisciplinary Team Approach

There is likely to be an extensive team involved in a newly diagnosed patient with cerebral palsy this may change over time but initially it may include:

  • paediatrician
  • neuro-developmental paediatrician
  • physiotherapist
  • occupational therapist
  • speech and language therapist
  • psychologist/psychotherapist, who helps to support the child and family with any aspect of cognitive and emotional development
  • health visitor
  • social worker
  • incontinence advisor

Some or all of the above will be involved with the child and the family in the development of the care plan to address any needs and problems the child or young person has.
This care plan will be regularly reviewed and the child reassessed as necessary and changes made as required to cope with altered needs.

Your child should be allocated a key worker who as the name implies is the person responsible for coordinating the care plan and is also the person who the other professionals report issues or concerns to.

Transition from childhood to adulthood is a key time and it is important that children and adult service providers are working together with the young person and the family as well as the school or college/university.

Possible complications of dystonic cerebral palsy

Whilst dystonic cerebral palsy is not a condition that gets worse over time although some deterioration in tone may occur with growth spurts, excessive wear and tear on the joints and bones can sometimes occur because of the condition.

Contracture (shortening of the muscle) is a risk in children who have problems stretching their muscles because of muscle stiffness. If the muscles cannot stretch, they do not grow as fast as the bones. This can lead to deformities, causing pain and discomfort. Physiotherapy is important in reducing the risk of contractures - however it cannot prevent them completely.

Problems can occur at joints due to the muscle spasms which can lead to spine and hip deformities.

Nutrition problems - Swallowing or feeding problems can make it difficult for someone who has cerebral palsy, particularly an infant, to get enough nutrition for growth and development as they use far more calories for any movement than those without cerebral palsy. Finidng a suitable diet is therefore critical.

Mental Health Issues such as depression, social isolation and body image and the challenges of coping with disabilities can contribute to the difficulties faced by young people with cerebral palsy. This is very much a personal experience as many children cope very well.

Osteoarthritis - Pressure on joints or abnormal alignment of joints from muscle spasticity may result in the early development of osteoarthritis.

Epilepsy – can be more common in young people with CP than the normal population.

Education

Every child and young person is entitled to an education and must not be discriminated against because of a disability and there is a variety of legislation in place to protect them. Clck here to see our guide for schools.

For help and support you can call the Dystonia Society helpline on  0845 458 6322 or 020 7793 3658 or email support@dystonia.org.uk

For more general information on children's disability:

SCOPE
6 Market Road
London
N7 9PW
England
UK
Helpline 0808 800 3333
response@scope.org.uk 

For information and support on hemiplegia:

HemiHelp
6 Market Road,
London
N7 9PW
Helpline 0845 123 2372 open 10am-1pm Monday to Friday during termtime.
helpline@hemihelp.org.uk
support@helpline.org.uk

Disclaimer
The Dystonia Society provides the information on this page as general information only. It is not intended to provide instruction and you should not rely on this information to determine diagnosis, prognosis or a course of treatment. It should not be used in place of a professional consultation with a doctor.
The Dystonia Society is not responsible for the consequences of your decisions resulting from the use of this information, including, but not limited to, your choosing to seek or not to seek professional medical care, or from choosing or not choosing specific treatment based on the information. You should not disregard the advice of your physician or other qualified health care provider because of any information you receive from us. If you have any health care questions, please consult the relevant medical practitioner.


References
World Health Organisation, 2001. International classification of functioning, disability and health. World Health Organisation, Geneva.
http://www.nice.org.uk/nicemedia/live/11220/52084/52084.pdf
http://www.nice.org.uk/nicemedia/live/13803/60029/60029.pdf
http://research.ncl.ac.uk/sparcle/

 

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