gms | German Medical Science

Objective: At present, there is no effective treatment or cure for Huntington's Disease HD patients. There is a high level of evidence that deep brain stimulation (DBS) is successful in the symptomatic treatment of other movement disorders but there are only case reports of DBS in Huntington's Disease (HD). Thus, DBS was evaluated in a prospective pilot trial for the symptomatic treatment of HD. Questions remained concerning the optimal target.

Methods: This phase I clinical trial is based on the hypothesis that DBS of the pallidum can reduce choreatic symptoms. In addition, it should demonstrate which part of the pallidum can be used effectively for the specific features of HD. We report on 6 HD cases, who underwent DBS of the pallidum (GPi/GPe region). 2 patients suffered from the Westphal variant of HD. Using stereotaxy the electrodes were implanted under general anaesthesia, followed by the implantation of a generator system (Kinetra™, Medtronic). Patients were randomized as to Gpi or GPe stimulation and crossed over after 6 weeks. Best contact stimulation was applied for 3 months.

Results: No perioperative complications occurred. The coordinates for the active contacts in the GPi/GPe range were adapted to individual anatomical changes. There were no differences in the total UHDRS between GPi and GPe stimulation. However, not counting the 2 Westphal patients, the total UHDRS of the remaining 4 patients improved significantly under DBS (preop 46.7 to 35.5 at 6 mo postop, p < 0.01). The most effective active contacts were in projection to the border of GPi (n = 3) and GPe (n = 3).

Conclusions: A systematic positive influence of DBS and its safety in HD patients is reported for the first time. In the context of the following MCT, it remains to be clarified which patient group is the most suitable for DBS and which long-term results can be obtained.