Brief Title
WILSTIM - DBS (WILson STIMulation - Deep Brain Stimulation)
Official Title
DEEP BRAIN STIMULATION FOR SEVERE DYSTONIA ASSOCIATED WITH WILSON'S DISEASE. A Prospective Multicenter Meta-analysis of Nof1 Trials
Brief Summary
Dystonia in Wilson's disease represent a major issue. The persistence of disabling motor
symptoms despite medical treatments justifies conducting a study on deep brain stimulation
(DBS) in Wilson's disease (WD). For bradykinetic patients, subthalamic nucleus (STN) could be
considered as a better target than the globus pallidus (GPi). For patients with hyperkinetic
dystonia, the internal globus pallidus (GPi) will be chosen as the target of DBS.
The investigators hypothesize that STN DBS will improve Wilson's disease patients, who,
despite copper chelators drugs, are still impaired by severe dystonia and akinesia (more or
less associated with other movement disorders).
The investigators primary objective is to demonstrate the efficacy of STN/GPi DBS on dystonia
associated with Wilson's disease.
Secondary objectives:
- To evaluate the impact of STN/GPi DBS on other movements disorders (tremor,
Parkinsonism, chorea) observed in Wilson's disease.
- To describe cognitive status of patients and to evaluate the consequences of STN/GPi DBS
on cognition and behavioral aspects of the disease.
- To evaluate the consequences of the stimulation on speech and swallowing.
- To evaluate the social impact of STN/GPi DBS in Wilson's disease.
- To evaluate the safety of STN/GPi DBS in the specific context of Wilson's disease.
Detailed Description
4 periods of stimulation on and off, sequence randomized at Day 0.
Study Type
Interventional
Primary Outcome
Change in movement disorder evaluated by the Canadian Occupational Performance Measure (COPM) performance and satisfaction scores
Secondary Outcome
Other movement disorder will be assessed by the reduction of the Burke-Fahn-Marsden (BFM) dystonia scale score
Condition
Severe Dystonia
Intervention
Medtronic, Activa® PC "on"
Study Arms / Comparison Groups
Stimulation "on"
Description: The deep brain stimulation is "on"
Publications
* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.
Recruitment Information
Recruitment Status
Device
Estimated Enrollment
5
Start Date
January 2016
Completion Date
January 2022
Primary Completion Date
January 2022
Eligibility Criteria
Inclusion Criteria:
- Age > 18 and < 60 years.
- Severe neurological form of Wilson's disease with predominant dystonia and
akinetic-rigid syndrome, despite optimized treatment stabilized for at least 6 months.
- Important disability due to abnormal movements (Rankin score=2 to 4).
- Absence of dementia (MMS > 24 and BREF > 15).
- Stable psychiatric status and absence of severe depression (BDI <28).
- Social security coverage.
- Signature of informed consent. (signature of legal guardian for subjects under
protection)
Exclusion Criteria:
- Severe hepatopathy with coagulation disorders (Platelet count < 100 G / l; INR > 1.5;
V factor deficit; low level of fibrinogen < 1g/dL; increased of fibrin degradation
products; low level of antithrombin).
- Liver transplanted patients < 2 years
- Patients under immunosupressive drugs and corticoids regimen.
- Participation to another biomedical research involving any drugs.
- Severe and uncontrolled psychosis or depression.
- Major atrophy on brain MRI that could represent a problem for leads implantation.
- Necrosis of the STN/GPi on brain MRI.
- Female subjects who are pregnant or lactating.
Gender
All
Ages
18 Years - 60 Years
Accepts Healthy Volunteers
No
Contacts
Stéphane THOBOIS, MD, +33 4 72 35 72 18, [email protected]
Location Countries
France
Location Countries
France
Administrative Informations
NCT ID
NCT02552628
Organization ID
69HCL14_0448
Secondary IDs
IDRCB
Responsible Party
Sponsor
Study Sponsor
Hospices Civils de Lyon
Study Sponsor
Stéphane THOBOIS, MD, Principal Investigator, Hospices Civils de Lyon
Verification Date
July 2018