Deep brain stimulation (DBS) is a treatment for neurological conditions such as essential tremor, Parkinson’s disease, epilepsy, and obsessive-compulsive disorder. The treatment is also being developed for Huntington’s disease.

DBS involves the surgical implantation of an electrode in the brain, specifically in a region called the globus pallidus. Wires passing through the neck connect the electrode to a stimulator in the chest. The stimulator is a small device that delivers a controlled electronic pulse to the brain.

How DBS works

Although DBS has been used for decades to treat neurological and psychiatric disorders, scientists are not entirely sure how it works. The technique was originally developed based on the observation of its effects, rather than an understanding of the mechanism leading to those effects.

Researchers are now beginning to better understand exactly what DBS does to the brain and how it leads to therapeutic benefits. Research suggests that it involves multiple mechanisms, including shaping electrical and neurochemical activity of brain cells near the electrode, as well as modulating neural network activity more broadly.

DBS in clinical trials for Huntington’s disease

A small trial in Germany found that Huntington’s patients treated with DBS showed reduced symptoms of chorea (involuntary jerking or writhing movements), but not dystonia (muscle problems, such as rigidity). The patients also showed some improvements in mood, disability, and quality of life. The study concluded that DBS was safe for use in Huntington’s patients.

A Phase 2 clinical trial (NCT02535884) is currently recruiting adult-onset Huntington’s patients with moderate progression of the disease to participate in a study testing the efficacy and safety of DBS in Austria, Germany, and Switzerland. All participants will be surgically implanted with an electrode in the globus pallidus region of the brain, as well as a stimulator. Participants will be randomly assigned to one of two groups. In the first group, stimulation will begin immediately. In the second group — also known as the sham group — the stimulator will not be turned on to start with.

After three months, both groups will be assessed using the Unified Huntington’s Disease Rating Scale. At this three-month mark, stimulators will also be turned on in the sham group. Both groups will be assessed again at six months following implantation using a variety of assessment tools to measure motor function, dementia, and psychiatric symptoms. The trial is expected to be completed in October 2020.


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