DEEP BRAIN STIMULATION FOR SEVERE DYSTONIA ASSOCIATED WITH WILSON'S DISEASE. A Prospective Multicenter Meta-analysis of Nof1 Trials

Status: Unknown status
Location: See all (2) locations...
Intervention Type: Device
Study Type: Interventional
Study Phase: Not Applicable
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.

Eligibility
Participation Requirements
Sex: All
Minimum Age: 18
Maximum Age: 60
Healthy Volunteers: No
View:

• 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)

Locations
Other Locations
France
Hospices Civils de Lyon
Recruiting
Lyon
Hopital Lariboisiere
Recruiting
Paris
Contact Information
Primary
Stéphane THOBOIS, MD
stephane.thobois@chu-lyon.fr
+33 4 72 35 72 18
Backup
Segolene GAILLARD
segolene.gaillard@chu-lyon.fr
+33 4 72 35 75 51
Time Frame
Start Date: January 2016
Completion Date: January 2022
Participants
Target number of participants: 5
Treatments
Experimental: Stimulation on
The deep brain stimulation is on
Sham Comparator: Stimulation off
The deep brain stimulation is off
Sponsors
Leads: Hospices Civils de Lyon

This content was sourced from clinicaltrials.gov

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