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Prospective evaluation of pallidal deep brain stimulation in Huntington’s disease

S. Zittel, V. Tadic, C. Moll, A. Fellbrich, N. Brüggemann, D. Rasche, T. Bäumer, V. Tronnier, A. Münchau (Luebeck, Germany)

Meeting: 2016 International Congress

Abstract Number: 122

Keywords: Chorea (also see specific diagnoses, Deep brain stimulation (DBS), etc): Treatment, Huntingtons disease

Session Information

Date: Monday, June 20, 2016

Session Title: Surgical therapy: Other movement disorders

Session Time: 12:30pm-2:00pm

Location: Exhibit Hall located in Hall B, Level 2

Objective: To prospectively evaluate the effect of bilateral pallidal deep brain stimulation (DBS) on motor symptoms, activities of daily living and quality of life in Huntington’s disease (HD).

Background: Globus pallidus internus (GPi) DBS is a well-established treatment for dyskinesias in Parkinson’s disease. It has recently also been applied in a small number of patients with HD but experience is limited so far.

Methods: Six HD patients with disabling treatment-refractory chorea were assessed preoperatively and six months after GPi DBS. The primary endpoint was improvement in chorea. To evaluate motor symptoms the motor score of the Unified Huntington’s disease rating scale (UHDRS) was applied. Activities of daily living were assessed with the Total Functional Capacity score (TFC) and the Functional Assessment score (FA). The 26-item version of the World Health Organisation questionnaire (WHOQOL-Bref) was applied in four patients to evaluate quality of life.

Results: Two patients underwent high frequency (130 Hz) and four patients low-frequency (40 Hz) DBS. The chorea subscore of the UHDRS motor score improved by 47% (range 21%-86%) (p < 0.01). UHDRS total motor score was reduced by 17% (p = 0.02). Dystonia and bradykinesia subscores remained unchanged. Antihyperkinetic medication could be reduced in all of the patients. TFC and FA did not show an improvement in activities of daily living. Six months postoperatively, the WHOQOL-Bref total score increased from 207.89 ± 21.91 to 277.61 ± 37.39 (p = 0.02). There was a significant improvement in domain 2 (psychological) (47.92 ± 7.98 vs. 77.08 ± 12.96, p < 0.01) and domain 4 (environment) (53.13 ± 9.20 vs. 79.69 ± 5.98, p < 0.01).

Conclusions: Bilateral pallidal DBS is a therapeutic option for well-selected HD patients with treatment-refractory chorea and can not only improve chorea but also quality of life.

To cite this abstract in AMA style:

S. Zittel, V. Tadic, C. Moll, A. Fellbrich, N. Brüggemann, D. Rasche, T. Bäumer, V. Tronnier, A. Münchau. Prospective evaluation of pallidal deep brain stimulation in Huntington’s disease [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/prospective-evaluation-of-pallidal-deep-brain-stimulation-in-huntingtons-disease/. Accessed October 5, 2026.
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