WILSTIM - DBS (WILson STIMulation - Deep Brain Stimulation)
WILSTIM - DBS (WILson STIMulation - Deep Brain Stimulation)
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.
4 periods of stimulation on and off, sequence randomized at Day 0.
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.