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How does dystonia work in the brain?

Extracts from this document...

Introduction

Dystonia Primary dystonia is believed to be due to abnormal functioning of the basal ganglia which are deep brain structures involved with the control of movement. The basal ganglia assists in initiating and regulating movement. What goes wrong in the basal ganglia is still unknown. An imbalance of dopamine, a neurotransmitter in the basal ganglia, may underlie several different forms of dystonia, but much more research needs to be done for a better understanding of the brain mechanisms involved with dystonia. Secondary forms of dystonia arise from and can be attributed to numerous causes, such as birth injury, trauma, toxins, or stroke. Secondary dystonia can be symptomatic and can also occur in association with other disorders such as Wilson's disease. When dystonia is secondary to certain injuries or small strokes, we often find lesions (areas of damage) in the putamen, one nucleus in the basal ganglia, as well as in certain nearby structures. Even though we can see no microscopic abnormalities of the brain in the great majority of cases of dystonia, including those with generalized dystonia, the evidence is so clear in the secondary dystonias that we believe the same part of the brain is involved in all types. How does dystonia work in the brain? Dystonia is a disorder that has to do with the way we move. The control of our movements is very complicated and involves many areas in the brain. The area of the brain that is involved in dystonia is called the basal ganglia. The basal ganglia is a deep region of the brain that controls the speed of movement and prevents unwanted movements. If there is a small change in the way the basal ganglia works, it can cause movements to occur even if you don't want them to. This small change is not found by medical tests or pictures of the brain on an MRI scan. ...read more.

Middle

However, these instances are quite rare and usually treatable. Dystonia has a variable nature, therefore making it difficult to predict the prognosis of the disorder. Currently, no medication or therapy can prevent progression from happening. The dystonia (both generalized and focal forms) will usually stabilize within five years of onset, but symptoms may fluctuate-for example, stressful situations may make symptoms temporarily worse. The following questions provide an overview of the prognosis and effects of dystonia. More specific information about the various forms of dystonia can be found on the Forms of Dystonia page. My dystonic symptoms have been stable with no change for five years. Can I expect them to remain the same, or will they progress to becoming more debilitating? Often dystonia will stabilize and not progress. In some patients with cervical dystonia, there may even be a remission. As a general rule, the older one is when dystonia develops, the more likely it will not progress beyond a certain point but will plateau. The younger one is when dystonia develops, the more likely that it will progress over time, particularly if the dystonia begins in a leg. And in such patients, the disorder can stabilize eventually and not progress any further. However, we always advise patients that they should not take dystonia for granted. There is no guarantee that the disease will not progress even though it has stabilized itself for a number of years. There is always a degree of uncertainty that something may develop and that the dystonia will start to flair up in the future and become worse again. Can childhood dystonia go into remission and reappear as an adult? Yes. But this happens quite rarely The figure used to be quoted that as many as 10% of patients (child and adult onset combined) might have such a spontaneous remission. It is probably much less than that. More frequently, instead of a true remission, the severity of dystonic postures may become much less for months or years in a small minority of patients. ...read more.

Conclusion

There are a variety of therapeutic programs that have been suggested to be useful for treatment of tardive dystonia and while some are clearly successful in some patients, there is no therapy that is so uniformly successful that it might be considered a standard of care. Patients who are affected by tardive dystonia need to work closely with their doctors to try to find a successful regimen. What drugs can cause tardive dystonia? Drugs belonging to a class called "dopamine receptor blocking agents," also referred to as "neuroleptics," can cause dystonia. The following is a list of such drugs that can cause dystonia (trade name listed in parenthesis): Acetohenazine (Tindal(r)), Amoxapine (Asendin), Chlorpromazine (Thorazine(r)), Fluphenazine (Permitil(r), Prolixin(r)), Haloperidol (Haldol(r)). Loxapine (Loxitane(r), Daxolin(r)), Mesoridazine (Serentil(r)), Metaclopramide (Reglan(r)), Molindone (Lindone(r), Moban(r)), Perphanzine (Trilafrom(r) or Triavil(r)), Piperacetazine (Quide(r)), Prochlorperzine (Compazine(r), Combid(r)), Promazine (Sparine(r)), Promethazine (Phenagran(r)), Thiethylperazine (Torecan(r)), Thioridazine (Mellaril(r)), Thiothixene (Navane(r)),Trifluoperazine (Stelazine(r)), Triflupromazine (Vesprin(r)), and Trimeprazine (Temaril(r)). Is Thorazine (chlorpromazine) a cause of or treatment for dystonia? Thorazine is a dopamine receptor blocker. Many forms of dystonia (especially dopa-responsive dystonia) are associated with the role of dopamine in the brain. By blocking dopamine, Thorazine is capable of causing tardive (i.e. drug-induced) dystonia. In some instances dopamine blockers may help dystonia. It is generally best for people with dystonia to avoid medications that block dopamine receptors. Are some drugs better suited to treat secondary rather than primary dystonia? Wilson's disease requires a very specific treatment; therefore in a case of secondary dystonia caused by Wilson's disease (which is a copper abnormality), the dystonia will be alleviated by treating the Wilson's disease. Tetrabenazine, a dopamine depleter, may be appropriate to treat tardive dystonia. For the most part, many of the drugs of choice for secondary dystonia also work well for primary dystonia and vice versa. Can Artane (trihexyphenidyl), and/or Klonopin (clonazepam) be used during pregnancy? They are not recommended. Treatment for dystonia during pregnancy is very individual. In some cases doses can be reduced; in other cases the woman may (gradually) stop taking oral drugs completely. There is no single recommendation for all women patients. ...read more.

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