Researchers from Beth Israel Medical Center in New York have demonstrated that deep brain stimulation of the globus pallidus (near the thalamus) is a safe and highly effective therapy in patients with generalized torsion dystonia. Results of their study are being presented at the American Academy of Neurology Annual Meeting in Honolulu, March 29-April 5, 2003.
Generalized torsion dystonia is a progressively disabling movement disorder that generally develops around age 9 and affects approximately 5 in 100,000 people. Among Ashkenazi Jews, the incidence rate is approximately five times higher than among non-Jews due to a common ancestral gene. This form of dystonia is evidenced by involuntary movements, prolonged muscle contractions, twisting body motions and tremors -- symptoms that will eventually confine most patients to a wheelchair.
To determine the effectiveness of deep brain stimulation (DBS), bilateral DBS leads were implanted in seven patients with intractable primary dystonia. All patients were evaluated with the Burke-Fahn-Marsden Dystonia Rating Scale before and at several intervals after surgery, and were followed for at least six months.
All patients showed improvement in their Rating Scale scores, which was progressive over time. Muscle spasms and dyskinetic movements showed the most rapid improvement, with resolution of dystonic postures improving more slowly, and only partially in a few cases. One patient suffered an infection with the implanted hardware; no other complications occurred in any patients.
"We observed near-complete resolution of symptoms in three patients and significant improvements among the others," noted study author Michele Tagliati, MD, assistant professor of neurology at Albert Einstein College of Medicine. "Ongoing experiences in ours and other centers show that DBS may be a safe, effective therapy for a disease that is otherwise untreatable and grossly debilitating."
The American Academy of Neurology, an association of more than 18,000 neurologists and neuroscience professionals, is dedicated to improving patient care through education and research. For more information about the American Academy of Neurology, visit its online press room at www.aan.com/press/index.cfm.
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