Dystonia is a syndrome of sustained muscle contractions that produce writhing movements and abnormal posturing. Primary dystonia occurs without any other neurological conditions. Dystonia is thought to be due to abnormal signal processing in the basal ganglia, part of the brain's motor control system.
A number of medical therapies are used to treat dystonias, but all have limitations. Anticholinergic medications, benzodiazapines and baclofen have all shown clinical benefit. For focal dystonia, denervation with botulinum toxins can be helpful. However, for severe generalized dystonia, these therapies are often only partially effective or cause intolerable side effects.
Recently, chronic deep brain electrical stimulation (DBS) of the globus pallidus, a brain structure that receives the major portion of the output of the basal ganglia, has been shown to be an effective treatment for appropriately chosen patients with generalized dystonia. At this time, there is no clear understanding of the mechanism by which DBS affects dystonia and no full understanding of the physiological action of the globus pallidus in dystonia.
However, empirical evidence has shown that DBS can be highly effective in therapy for the disorder. In patients without fixed skeletal deformities, this procedure is associated with at least a 50 percent improvement in standardized rating scales of dystonia severity one year after surgery.
UCSF Medical Center has the busiest program of surgery for movement disorders in the western United States and is a pioneer in DBS surgery for the treatment of dystonia. The UCSF program utilizes an integrated team of neurologists, neurosurgeons and nurses who specialize in movement disorders surgery. This full-service, multidisciplinary program coordinates care so that patients can be seen by various specialists on the same day.
For more information, call Jill Ostrem, M.D., at (415) 353-2273 or Phillip Starr, M.D., at (415) 353-7500.
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