What About Those Oral Meds?

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Before thalamotomy, before deep brain stimulation, before botulinum toxin, there were medications. For years medication offered the only help for patients with dystonia. Whereas many patients now find relief through Botox and Myobloc, and whereas surgery, including DBS, is becoming a good treatment for cervical dystonia and generalized dystonia, many patients need to fall back on medications to get better relief of their symptoms. When chemical denervation and surgery are not helpful, medications often become the only medical treatment available.

Many medications have been tried over the years and many offer some benefit in the treatment of dystonia. Although they offer incomplete relief in most cases, and although sometimes there are side effects associated with them, medications can improve the quality of life quite a bit. I’d like to discuss a few of them here. Much of what I say here can be found on the “WE MOVE” web site.

Benzodiazepines

Benzodiazepines are a class of drugs that interfere with chemical activities in the nervous system, including the brain. To the nerve cells, these molecules look like a naturally occurring molecule called GABA. When GABA is around the nerve cell it tends to turn the nerve cell down a little bit. These nerves then fire less frequently and, therefore, the communication between nerve cells is reduced. Because of this, benzodiazepines may relax muscles and ease symptoms associated with dystonia. In general, these are oral medications. Two of the more popular ones are diazepam (Valium) and clonazepam (Klonopin). The major side effect of these drugs is drowsiness, which sometimes is controlled by lowering the dose. Other side effects in higher doses may occur but none of them life threatening.

Baclofen

Baclofen (Liorisal) is a drug that is generally used to treat spasticity. Its main site of action is the spinal cord, where it inhibits nerves that cause muscle contraction. It may sometimes cause dry mouth but is usually well tolerated. In recent years this medication has been used in a pump into the spinal canal so that much lower doses can be used with a much more even effect.

Anticholinergics

Anticholinergic medications block the action of the neurotransmitter, the acetylcholine, an important neurotransmitter in the brain, especially in the basal ganglia. As you probably remember, the basal ganglia are very important in dystonia. The most widely used anticholinergic medication is trihexyphenidyl (Artane). Artane is very well tolerated in children, but it is not so well tolerated in adults. Children can take much higher doses that adults can tolerate. The main side effects include dry mouth, confusion, and difficulty with memory. Blurred vision and urinary retention can also be a problem.

Dopamine blocking agents

Medications that block the effects of dopamine have also been helpful in some patients with dystonia. Dopamine is an important neurotransmitter in the brain as well, and excessive activity of this chemical is implicated in dystonia. Although not available in the United States, tetrabenazine can be obtained in most cases from ordering it overseas. Other dopamine blocking agents such as Haldol and similar medications have not been helpful in dystonia, and can often make it worse.

Other medications

Certain seizure medications, such as Neurontin, Gabatril, Topamax, and several others have been helpful in some people, but their effectiveness remains uncertain. It is worth discussing other medications with your neurologist. New medications to come along from time to time and they may be helpful for dystonia.

Dr. Matthews GWYNN, Medicine Man
993-F Johnson Ferry Rd. NE Suite 120
Atlanta, GA 30305

My mom and I wanted to thank you for hosting such a great symposium this year. This was our third year and we are looking forward to next years. E. Mathews