WILSTIM – DBS (WILson STIMulation – Deep Brain Stimulation)

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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