Dystonia - Medical, Surgical & Supportive Therapies

Brain
Brain. Wikimedia Commons

Dystonia involves involuntary contraction of muscles that normally work in cooperation, so that a body part is held in an unusual and often painful position as a result. Dystonia can affect any body part, and can result in both embarrassment and the inability to perform daily activities. Fortunately, there are a number of different ways that dystonia can be improved.

Physical and Occupational Therapy

Seeing a physical or occupational therapist can help people with dystonia learn to work around their disorder, although it doesn't directly treat the problem.

Many people with dystonia also find that they are able to relieve their symptoms temporarily by touching part of their body in some way. This is known as geste antagoniste, and is one of the more mysterious aspects of dystonia.

Oral Medications

Unfortunately, few medications are completely effective for the treatment of dystonia. Exceptions to this rule include the use of Benadryl to treat acute drug-induced dystonia and the use of dopamine to treat certain forms of inherited dystonia, such as Segawa syndrome. For this reason, all children or adolescents who have dystonia should be given a trial of dopamine.

Artane (trihexyphenidyl) is one of the best-studied medications for dystonia. This medication is from the family of anticholinergics. Younger patients tend to benefit most from this medication. Adults may be more sensitive to the side effects of anticholinergics, including dry mouth, confusion, sedation, memory loss and hallucinations.

Benzodiazepines, like clonazepam, may also be used, usually in conjunction with another medication as well. Baclofen, a muscle relaxant, is generally not very helpful in the treatment of dystonia, but may be useful in treating leg dystonia, especially among children. The main side effect of these medications is sedation.

Dopamine-depleting agents like tetrabenazine are the exact opposite of giving dopamine, but can also have a place in treating dystonia. Side effects include depression and dysphoria, as well as parkinsonism. If these medications are used, the doses should only be increased very slowly.

Injected Medications

In focal dystonia affecting only one part of the body, injections of botulinum toxins may be helpful. In fact, in some kinds of dystonia, such as blepharospasm (excessive eye blinks) and cervical torticollis (neck dystonia), botulinum toxin injection is considered the first-line therapy. In torticollis, 70-90% of patients reported some benefit. Injections are repeated every 12 to 16 weeks. Under this treatment plan, the effects can remain robust and safe for many years.

Botulinum injections work by blocking the release of acetylcholine, the neurotransmitter that signals between peripheral nerves and muscles. This leads to a weakening of the muscle. Side effects of botulinum toxin injections include excessive weakness, which can become especially bothersome if injecting around the eyes for blepharospasm, or around the neck and throat, as this can lead to swallowing problems.

The injections must be targeted very precisely in order to maximize benefit while minimizing the risk of side effects.

Surgical Options

When medical options fail and if the dystonia is truly impairing someone’s life, surgical options may be considered.

In the past, these surgeries involved intentionally damaging either the peripheral nerve that leads from the brain to the affected muscles (thereby weakening the muscles and relieving the dystonia) or ablating part of the brain. Now, most people prefer a less permanent solution in the form of deep brain stimulation (DBS).

Deep brain stimulation is most indicated for medically refractory primary generalized dystonia. The people who suffer from this type of dystonia tend to be young, including children. Responses to deep brain stimulation can vary widely. In general, the response of dystonia to DBS is less predictable than the response of Parkinson’s disease and essential tremor, and the improvement may only be seen many months after treatment.

About twelve months after DBS, most patients with dystonia show improvement in movement by about 50 percent. Children and people who have had dystonia for a relatively short period of time tend to do better than average. Secondary dystonia does not tend to respond as predictably to deep brain stimulation. Similarly, if the dystonia has led to fixed postures rather than fluctuating in severity, the dystonia is less likely to respond to deep brain stimulation.

Sources:

Kathleen Poston, Overview of Common Movement Disorders, Continuum : Movement Disorders Volume 16, Number 1, February 2010

Mustafa Saad Siddiqui, Ihstsham Ul Haq, Michael S Okun, Deep Brain Stimulation in Movement Disorders, Continuum : Movement Disorders Volume 16, Number 1, February 2010

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