Printer Friendly Version

What is Dystonia?
Dystonia is a syndrome of sustained involuntary muscle contractions, frequently causing twisting and repetitive movements, or abnormal postures.

Under normal circumstances, when some mucles contract, others need to relax in  order to allow a smooth and controlled movement of the particular part of the body. In dystonia, various muscles contract in opposition to each other in an uncontrolled fashion.

What are the main types of dystonia?
Dystonia may affect widespread areas of the body (generalised dystonia), or may be confined to a particular region (focal dystonias).

Types of focal dystonia include cervical dystonia (spasmodic torticollis or 'wry neck'), writer's cramp, blepharospasm and golfer's 'yips'.

The onset of dystonia in childhood or adulthood imparts a significant risk of spread to other areas than adult-onset dystonia.

Most cases of dystonia occur spontaneously or are genetic (primary dystonia), but some are due to an environmental insult, such as cerebral palsy or medications (secondary dystonia).

How is it dystonia treated?
Dystonia is usually treated with medications and/or Botox injections. Many patients continue to progress despite these therapies.

Some specialists offer a destructive procedure, known as denervation, however such procedures carry significant risks.

In patients with primary dystonia, particularly generalised dystonia and spasmodic torticollis, deep brain stimulation (DBS) offers a high chance of benefit with a low risk of complications. DBS is also used in ome cases of secondary dystonia, such as post-traumatic dystonia.

What are the reasons for surgery?
When medications and Botox are no longer working well or their side effects are to severe, surgery may be of value. The procedure most commonly used to treat Dystonia is DBS of a part of the brain known as the pallidum (‘globus pallidus internus, or GPi’).

What are the alternatives to surgery?
At present, the only alternatives to surgery are medications and Botox.

You should check with your neurologist to ensure that you have tried all of the appropriate medications before seriously considering surgery. Your neurologist may choose to refer you to another neurologist with expertise in the management of such conditions.

What happens before surgery?
Several weeks before surgery you will undergo an MRI scan of your brain. This will be used to plan and execute your operation. You will also be seen by a neuropsychologist and frequently by one of our movement disorder surgery neurologists. We will arrange all of this for you.

What do you need to tell the doctor before surgery?
It is important that you tell your surgeon if you:

• Have blood clotting or bleeding problems
• Are taking aspirin, warfarin, or anything else (even some herbal supplements) that might thin your blood
• Have high blood pressure
• Have any allergies
• Have any other health problems

What is deep brain stimulation?
Deep brain stimulation works by implanting fine wires (electrodes) into the pallidum. DBS works by reducing the activity of this tiny area that may be overactive in Dystonia. The electrical pulse inhibits the activity in the brain surrounding the wire.

  More information on deep brain stimulation (DBS) for movement disorders

How is deep brain stimulation performed?
Before surgery you will have a special frame attached to your head. This is usually done with local anaesthetic and is not too bothersome. Then you will have a brain scan before being taken to the operating theatre. Some more local anaesthetic and some light sedation will be given before one or two small holes are shaved in your skull. This does not hurt, and is usually much less uncomfortable than going to the dentist! The electrode (wire) is then slowly inserted and the electrical activity will be monitored to guide its final placement. Once we are confident that we are in the right position, we will stimulate through the tip of the electrode to look for side effects. You will then undergo another brain scan and, if the results are satisfactory, you will be taken back to the operating theatre (sometimes on the same day) for the second stage. This involves giving you a general anaesthetic and running the wires under your skin from your head to your chest. They are connected to a battery placed under the skin just below your collarbone (sometimes the battery is placed in your abdominal region or flank). You will be discharged from hospital several days later. Your neurologist will adjust your stimulation settings and medications progressively over a number of months.

Who may benefit from surgery?
Patients with generalised dystonia or spasmodic torticollis have a high likelihood of success following DBS. Patients benefit the most if surgery is performed early, before rigid deformities have set in.

What are the benefits of surgery?
Reduction or abolition of abnormal dystonic movements, improved mobility, reduced pain, and improved quality of life are the most important benefits of surgery. These benefits typically take around 6-12 months to reach their maximum.

What are the risks of surgery?
There is a very small risk of infection, haemorrhage (bleeding), stroke, and seizures (epilepsy). The risk that the surgery could cause death is extremely small (much less than 1 in 100). Over 95% of patients come through surgery without significant complications.

How successful is surgery?
Surgery for dystonia frequently results in a reduction in the symptoms and signs of this condition, and an improvement in quality of life. Some patients are able to return to their previous occupation following successful treatment. Surgery does not return you to "normal" however, and it is important that you understand this before going ahead. Overall, of the patients selected for surgery, around 70% will significantly benefit. The magnitude of this benefit is typically 50-70%.

How long will the benefits last?
This will vary from patient to patient. These benefits are typically long-lasting, and many patients have been followed up for longer than 6-8 years with ongoing benefit. You will need to have you battery changed (this takes around 20 minutes and is done under local anaesthesia) every 18 months or so.

What happens when you go home?
You will need to take it easy for 6 weeks. You should do an hour of gentle exercise such as walking, every day or two.

Your GP should check your wounds 4 days after discharge. You should not sign or witness any legal documents until you have been seen by your GP. You will be given instructions about when the staples need to be removed (this is done by your GP or the Precision Neurosurgery Practice Nurse).

You will be reviewed after a number of weeks by your neurologist and neurosurgeon. You should not drive a motor vehicle or operate heavy machinery until they give you the go ahead.

If you have had deep brain stimulation you will also be given some detailed information about things you must avoid, such as metal detectors at airports. It is critical that you read such information thoroughly.

What should you notify your doctor of after surgery?

• Increasing headache
• Fever
• Swelling or infection of the wounds
• Leakage of fluid from the wound
• Fitting (seizures)
• Abnormal sensations or movements in your face, arms or legs
• Weakness or numbness
• Drowsiness
• Any other concerns

The consent process
You will be asked to sign a consent form before surgery. This form confirms that you understand all of the treatment options, as well as the risks and potential benefits of surgery. You should also understand the costs involved with surgery before going ahead, and should discuss this with your surgeon. If you are unsure, you should ask for further information and only sign the form when you are completely satisfied.