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Deep Brain Stimulation in DYT1 Dystonia: A 10-Year Experience

Panov, Fedor MD*; Gologorsky, Yakov MD*; Connors, Grayson BA; Tagliati, Michele MD§; Miravite, Joan RN; Alterman, Ron L. MD

doi: 10.1227/01.neu.0000429841.84083.c8
Research-Human-Clinical Studies: Editor's Choice
Editor's Choice

BACKGROUND: Globus Pallidus Interna (GPi) deep brain stimulation (DBS) is an effective treatment for DYT1-associated dystonia, but long-term results are lacking.

OBJECTIVE: To evaluate the long-term effects of GPi DBS in patients with DYT1 dystonia.

METHODS: A retrospective chart review (cohort study) of 47 consecutive DYT1+ patients treated by a single surgical team over a 10-year period and followed for up to 96 months (mean, 46 months) was performed. Symptom severity was quantified with the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor (M) and disability (D) sub-scores.

RESULTS: As measured with the BFMDRS (M), symptom severity was reduced to less than 20% of baseline after 2 years of DBS therapy (P = .001). The disability scores were reduced to <30% of baseline (P = .001). Symptomatic improvement was durable throughout available follow-up. Sixty-one percent of patients had discontinued all dystonia-related medications at their last follow-up. Ninety-one percent had discontinued at least 1 class of medication. Infections requiring removal and later reimplantation of hardware occurred in 4 of 47 patients (8.5%). Hardware malfunction including lead fractures occurred in 4 of 47 cases (8.5%). Lead revision to address poor clinical response was performed in 2 of 92 implanted leads (2.2%).

CONCLUSION: GPi DBS is an effective therapy for DYT1-associated torsion dystonia. Statistically significant efficacy is maintained for up to 7 years. Neurologic complications are rare, but long-term hardware-related complications can be significant.

ABBREVIATIONS: BFMDRS, Burke-Fahn-Marsden Dystonia Rating Scale

DBS, deep brain stimulation

GPi, globus pallidus interna

MCP, mid-commissural point

PGD, primary generalized dystonia

*Department of Neurosurgery, Mount Sinai School of Medicine, New York, New York;

New York College of Osteopathic Medicine, Old Westbury, New York;

§Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, California;

Department of Neurology, Beth Israel Medical Center, New York, New York;

Division of Neurosurgery, Beth Israel Deaconess Medical Center, Boston, Massachusetts

Correspondence: Ron L. Alterman, MD, Department of Surgery, Beth Israel Deaconess Medical Center, West Campus, Lowry Office Building, Suite 3B, 110 Francis Street, Boston, MA 02215. E-mail:

Received October 28, 2012

Accepted March 07, 2013

Copyright © by the Congress of Neurological Surgeons