6.  Treatment options for Dystonia

There are six main approaches to the treatment of dystonia:

  1. Oral medications
  2. Injection Therapy
  3. Intrathecal medications
  4. Deep brain stimulation
  5. Ablative Surgery
  6. Complementary therapies

These therapies may be administered individually, or a combination of treatments may be deemed appropriate.

Oral medications

There are a number of pharmacological treatment options available to treat dystonia. Drugs such as benzodiazepines, baclofen, dopamine-blocking or dopamine-depleting agents or anticholinergics may be used ?either alone or in combination ?to treat patients with dystonia. Drugs may also be combined with other forms of treatment.

Your doctor will be able to discuss the individual benefits and side effects of different drug therapies with you.

Injection therapy

Botulinum toxin is a biological therapeutic agent that acts against dystonia. Botulinum toxin is a toxic protein that is produced by the bacterium Clostridium botulinum. When a minute amount of drug is injected directly into an overactive muscle, it relaxes the muscle, decreasing inappropriate or excessive muscle contractions and allowing the affected area to assume a more normal position or posture.

Intrathecal medications

Intrathecal drug therapy has been successfully used in the treatment of secondary dystonia caused by stroke, cerebral palsy, multiple sclerosis, and acquired brain and spinal cord injuries.

The drug is delivered directly into the intrathecal space (the area around the spinal cord) using a three-part system:

  • a surgically placed, programmable pump with a reservoir or storage area for the drug;
  • a flexible silicone tube or catheter; and
  • a programming device.

Because the drug is administered directly to the site where it will act instead of being taken orally, much less is needed. The reduced volume results in fewer side effects, such as drowsiness and sedation. Intrathecal drug therapy is usually combined with physical therapy and other forms of rehabilitation.

Candidates for this therapy usually suffer from severe dystonia that has not responded to more conservative drug treatments, or cannot tolerate the side effects of these drugs at therapeutic doses.

To help determine whether intrathecal drug therapy might produce a helpful response, youll first undergo a screening test. This involves administering the drug in a test dose into the intrathecal space. The peak effect of the drug will usually occur within four hours, and you must be closely monitored in a fully equipped and staffed setting to mitigate the risk of possible side effects. If you respond positively to the test dose, you can be considered for the therapy.

Once you've been deemed suitable for the therapy, a surgeon will perform an operation to implant the battery-powered pump and catheter. The entire hospital stay is usually four to seven days. In this time, the pump is programmed to deliver the best possible drug dose to reduce muscle tone and provide relief from the spasticity. Some tenderness or soreness is common after the operation, but this is usually treated with pain medication. The implanted pump can also cause a slight bulge in the abdominal wall, but many patients stop noticing this after a few weeks.

A decrease in the tone of dystonia-affected muscles is usually noticeable within several days of the operation, but evidence of significant improvements in function can often take longer.

The metered drug dose can be adjusted whenever necessary by reprogramming the pump in your doctors office. The pump also contains a programmable alarm that beeps softly when either the drug reservoir or the batteries run low. The reservoir can be refilled as needed with an injection, and this is usually needed every one to six months dependant on dosage settings. The batteries will run low after about seven years, and the entire pump is replaced at this time.

The side effects of intrathecal drug therapy are similar to those for drugs given orally, but are typically milder because of the lower doses of medicine required. About 5 percent of intrathecal drug therapy patients develop infections that require the temporary removal of their pump.

Equipment-related risks include pump failure, catheter kinking or breakage, and movement (dislodgement) of the catheter so that the drug no longer reaches the intrathecal space. Mechanical defects or failure to refill the pump reservoir can lead to sudden interruption of drug treatment. In rare cases, this can cause a life-threatening withdrawal syndrome. Your family must be educated about the signs of drug withdrawal, and theyll need a plan for managing such an emergency.

Although it rarely happens, it is possible for a person receiving this therapy to receive an overdose of their medication. This may cause drowsiness, lightheadedness, slowed or difficult breathing, seizures, loss of consciousness, and coma. In the event of an overdose, it is very important for the patient or caregiver to immediately contact the patients physician.

Deep brain stimulation

Deep brain stimulation (DBS) uses an implantable medical device similar to a cardiac pacemaker, which treats the main symptoms of dystonia by delivering electrical stimulation to a precisely targeted area deep within the brain. Fully reversible, this implant surgery helps control the main symptoms of dystonia.

It is most suitable for patients whose symptoms are not well controlled by drug treatment, or who cannot tolerate the side-effects of their recommended medication.

The electrical signals delivered by DBS de-activate the target site within the brain without destroying any tissue. The goal is to restore the balance of electrical activity within the brain, by blocking the brain signals that cause dystonic spasms.

The electrical signals are also adjustable, allowing more precise calibration and targeting of the effect. This can help improve your symptom control and reduce the risks of accidental damage to neighbouring areas.

The surgery is conducted under general anaesthetic. The use of a rigid metal frame, which holds your head perfectly still, helps ensure precise guidance of the instruments during surgery.

DBS requires repeat visits to the neurologist, so that the electrodes can be precisely programmed to deliver the maximum benefit. The benefits of DBS can take some time to become apparent with dystonia.

If the person implanted with DBS is a child or adolescent, further surgery may be required to extend the surgical leads as the patient grows.

Any surgery is a major procedure. The risks during and immediately after surgery include infection, haemorrhage, and even death. Complications from surgery may include cognitive impairment, swallowing difficulty, visual impairment, seizures, and headache. While many of these complications will improve over time, they can persist without improvement in some patients.

You, your caregivers, and your movement disorder specialist must work together to determine if surgery is the right option for you. Your neurologist and a neurosurgeon will then help you choose the right procedure. When all of these factors are considered carefully, surgery does have the potential to significantly improve your quality of life with Dystonia.

There is also the chance that any part of the hardware ?the electrodes in the brain, battery-powered pulse generator in the chest, and wire connecting them under the skin ?may malfunction or break. If this does happen the dystonia may rebound? the original symptoms returning with the same or increased severity. Any malfunction demands urgent medical attention and the equipment may require surgical removal or replacement.

Ablative Surgery

Ablative surgery destroys a select region of the brain. Pallidotomy and thalamotomy are types of ablative surgery involving lesioning or surgical destruction of specific brain cells controlling the initiation and generation of movement. Compared to their use in patients with Parkinson's disease, these procedures have been performed in relatively few cases of dystonia.

Ablative surgery may be a suitable treatment for some patients. However, the lesioning is irreversible and non-adjustable, so the destroyed tissue cannot be replaced and the side effects cannot be adjusted. Ablating some sites also runs the risk of damaging brain cells that influence vision, swallowing, and verbal ability.

Selective myectomy or myotomy is another surgical procedure, in which a portion of an overactive muscle is removed. Selective myectomies are rarely performed.

Ramisectomy and rhizotomy are surgical procedures that involve the cutting of the nerve(s) that direct an overactive muscle. These types of surgeries are also rarely performed. However they may be effective in certain, properly selected patients.

Peripheral denervation, is the removal of selected nerves at the point where they enter the hyperactive muscle. The activity of uninvolved muscles is maintained. This procedure may be an option for some patients whose treatment has not been successful, or who have not responded to injection therapy.

Any surgery is a major procedure. The risks during and immediately after surgery include infection, haemorrhage, and even death. Complications from surgery may include cognitive impairment, swallowing difficulty, visual impairment, seizures, and headache. While many of these complications will improve over time, they can persist without improvement in some patients.

You, your caregivers, and your movement disorder specialist must work together to determine if surgery is the right option for you. Your neurologist and a neurosurgeon will then help you choose the right procedure.

Complementary therapies

Physiotherapy for dystonia is used to supplement many of the therapies mentioned above. The treatment will be designed to meet your specific needs and will maintain muscle tone, and maintain or improve range of motion and mobility, increase strength and coordination, and improve care and comfort.

Stretching exercises may be important for maintaining or recovering the range of motion in affected joints.

Some patients use 'sensory tricks' to temporarily relieve their symptoms. These frequently involve touching or stroking a particular spot on the skin. Exactly how these 'sensory tricks' obtain their relief is not understood. It is possible that the skin stimulation may interrupt the abnormal nerve impulses causing muscle overactivity.

Certain types of braces can also provide the same type of stimulation, and be equally effective for some people.


If you are suffering from a movement disorder, discuss treatment options with your doctor, and ask for a referral to a movement disorder specialist near you.

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