The main purpose of this study is to investigate whether Deep Brain stimulation has a positive effect on the motor and cognitive symptoms in Huntingtons patients. Deep brain stimulation (DBS) is a surgery to implant a device that sends electrical signals to brain areas responsible for body movement. Electrodes are placed deep in the brain and are connected to a stimulator device. Similar to a heart pacemaker, a neurostimulator uses electric pulses to regulate brain activity. DBS treatment has been used for some people with Parkinsons disease. Both safety and efficacy will be investigated in this study with 50 participants. 25 participants will be stimulated with the DBS for the first period of 3 months and their performance on a variety of tests, will be compared with the 25 participants who don’t get any stimulation. After the first 3 months, all participants will be treated with DBS for another 3 months.
Estimated Study Completion Date: 31 December, 2022
Ages Eligible for Study:
18 Years and older (Adult, Older Adult)
Sexes Eligible for Study:
All
Accepts Healthy Volunteers:
No
Inclusion Criteria
Clinically symptomatic and genetically confirmed HD (number of CAG repeats ≥ 36)
Age ≥18 years
Moderate stage of the disease (UHDRS motor score ≥ 30)
Chorea despite best medical treatment (UHDRS chorea subscore ≥ 10)
Mattis Dementia Rating Scale ≥ 120 (or > 80% of items testable independently from motor impairment)
Patient has stable medication prior six weeks before inclusion
Signed informed consent
Exclusion Criteria
Juvenile HD (Westphal variant) or predominant bradykinesia
Postural instability with UHDRS retropulsion score > 2
Severe comorbidity compromising operability and/or life expectancy and/or quality of life during the trial duration (e.g. cancer with life expectancy < 6 months, NYHA 3 and 4 rising the anaesthetic risk according to the anaesthesiologist)
Acute suicidality
Acute psychosis (symptoms within previous 6 months)
Participation in any interventional clinical trial within 2 months before screening
Cortical atrophy grade 3
Patients with risk of coagulopathies and/or increased risk of haemorrhage
Patients with an implanted pacemaker or defibrillator