What is it?

Dystonia is a type of movement control problem that can occur after a brain injury. It refers to repetitive twisting, abnormal posturing or having the body 'lock in' to various positions. Dystonia can affect just one body area including the neck, mouth, jaw, trunk or a limb.

It is caused by abnormal signals from the basal ganglia part of the brain to the muscles. These wrong messages result in sustained muscle contractions. Dystonia is made worse with voluntary movement.  When your child is at rest or asleep, the muscles are relaxed and appear normal.

Dystonia can vary in severity from mild to severe. It can be focal, involving just one muscle group or body part; or it can be generalised, involving the whole body. It can be present alongside other motor problems such as spasticity, and its presence may not always be readily recognised.

This particular motor disorder can have a delayed onset, e.g. weeks or even months after the brain injury occurred. Dystonia may sometimes become more obvious over time, and this may lead to a loss of motor skills. Severe dystonia can lead to pain, joint damage and deformity.

What is the treatment?

Therapy

Dystonia is not a well-understood problem, and it can be very difficult and frustrating to treat. This is despite the skill of the therapist or the frequency of therapy or excercising. Therapy techniques that can work well for other movement problems such as spasticity and weakness often do not work for individuals with dystonia.

Techniques

Dystonia makes it difficult for a person to control his or her own body movements and posture, and efforts to do so often make the problem worse. Often, the more a person tries to control their posture the more locked in they may become, or the more out of control the affected limb gets. Some individuals are able to develop their own techniques (triggers) for managing their dystonic posturing.  Therefore, it is possible for some individuals to gain some control.

Orthoses, splints and lycra

The presence of dystonia can cause difficulties with the fitting, making and wearing of splints or orthoses, and creative solutions are often needed. Protective splints to minimise pain, joint damage and for functional benefits can be useful and important in the management of dystonia. Lycra garment splinting may be worth trialing as it can work well for some individuals, especially in the upper limbs and trunk.

Medical treatments

• Focal dystonias can sometimes be relieved by botulinum toxin injections.
• Generalised dystonia may respond to oral drug treatments such as L-dopa, baclofen, benztropine, benhexol and others.
• Generalised dystonias are best treated with oral or intrathecal medications. Intrathecal medications are delivered directly to the space surrounding the spinal cord. Intrathecal baclofen is a relatively new, expensive but effective treatment for severe generalised spasticity and dystonia.

Key points to remember

• Dystonia is a movement control problem that can occur after an injury to an area of the brain called the basal ganglia.
• Dytonia refers to involuntary muscle contractions that can cause repetitive twisting, abnormal posturing or the 'locking in' to positions by the body or a body part such as the neck, mouth, trunk, or a limb.
• Dystonia may have a delayed onset after brain injury.
• Treatment will vary depending on the type of dystonia and can include splinting and medical intervention.

For more information

The Royal Children's Hospital
Paediatric Rehabilitation Service
T: (03) 9345 5283
E: rehab.service@rch.org.au