Deep brain stimulation effective for patients with primary generalized dystonia

A new study from Mount Sinai School of Medicine finds that deep brain stimulation (DBS) is a safe and effective treatment for patients with primary generalized dystonia (PGD).

The study appears in the April 13 issue of Archives of Neurology, and was supported in part by a grant from the Bachmann-Strauss Dystonia and Parkinson Foundation.

Michele Tagliati, MD, Chief of Division of Movement Disorders and Associate Professor of Neurology at Mount Sinai School of Medicine, and Ron Alterman, MD, Associate Professor of Neurosurgery and Director of Functional and Restorative Neurosurgery at Mount Sinai School of Medicine, co-authored the study.

Primary generalized dystonia is a movement disorder that causes muscles to contract and spasm involuntarily and is characterized by twisting movement and abnormal postures. Deep brain stimulation involves the implantation of a series of electrodes into specific targets within the brain. The electrodes are then connected to pacemakers that are placed beneath the skin of the chest wall. The pacemakers send electrical impulses to the brain that help control the symptoms of dystonia and other neurological disorders.

For this study, 30 consecutive patients with PGD were followed for at least two and as many as eight years after DBS surgery. Overall improvement as measured at one year was maintained in all at successive years, and was still greater than 80% in the five patients who reached seven years of follow-up. There were no intraoperative complications and hardware-related adverse events were infrequent. Rare stimulation-related adverse events primarily affected speech and clinical outcome did not depend on high stimulation intensities.

"Our research proves that deep brain stimulation is helping many people who suffer from this painful and debilitating disease," said Dr. Tagliati. "DBS is a crucial treatment because it's the best therapeutic option for patients with primary dystonia that do not respond well to medication."

"The long-term results of this intervention are important because many patients with PGD undergo DBS implantation in childhood or adolescence," said Dr. Alterman. "Clinical results beyond three years after DBS surgery are not previously documented in significant numbers of patients. One of the patients in our study has enjoyed sustained improvement in motor function and her quality of life for eight years since surgery. This is good news for those who must live with this disabling disease."

Drs. Alterman and Tagliati have received speaking honoraria and consulting fees from Medtronic Inc., unrelated to the conduct of this study. Medtronic Inc., manufactures the DBS implant.

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