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

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Abstract

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

ABBREVIATIONS:

DBS, deep brain stimulation

ABBREVIATIONS:

GPi, globus pallidus interna

ABBREVIATIONS:

MCP, mid-commissural point

ABBREVIATIONS:

PGD, primary generalized dystonia

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