Validation of a Quality of Life Assessment Tool in Adult Generalized Dystonodyskinetic Syndromes Treated by Continuous Electrical Neuromodulation (DBS-QoLs)

Validation of a Quality of Life Assessment Tool in Adult Generalized Dystonodyskinetic Syndromes Treated by Continuous Electrical Neuromodulation

Validation of a Quality of Life Assessment Tool in Generalized Dystonodyskinetic Syndromes Treated With Continuous Electrical Neuromodulation (CEN): The "Dystono-dyskinetic BrethoméSanrey Quality of Life" Scale (DBS-QoLs)

In spite of a growing interest in the evaluation of health-related quality of life in movement's disorders management, there is no tool specifically dedicated to dystonia and related syndromes that measures both the objective severity of the handicap and the patient's feelings about the surgical treatment in terms of subjective improvement. We have been working for several years at the bedside of patients operated on in the " pathologies cérébrales résistantes " Unit on the development of a related questionnaire to assess both the motor severity and the patients' feelings about the evolution of their disease under Deep Brain Stimulation (DBS). The self-questionnaire designed would allow to a certain extent to get away from the two gold standards currently used in the field, i.e. the Burke, Fahn & Marsden Dystonia Rating Scale (BFMDRS) and the SF-36, thus facilitating the evaluation and allowing a homogenization of the practices for the different implanting centers on the french territory.

The construction of the tool in its particular design, including various targeted modules, stems from an experience of more than ten years acquired with patients treated by deep brain stimulation in the unit. The self-questionnaire thus obtained will be sent to 146 patients with generalized dystonodyskinetic syndrome, operated and followed in the unit, and submitted to various statistical analyses. The scores of the identified dimensions will be compared with the etiology of the abnormal movements (primary vs. secondary) as well as with the results obtained with the two scales currently used to evaluate abnormal movements (BFMDRS) and/or quality of life (SF-36) and finally with the patients' mood during their follow-up (BDI, STAI). Tolerance to surgery will also be measured.

The DBS-QoLs questionnaire will be sent to the patients for answer: The questonnaire will be sent at : the inclusion 15 days after the inclusion 1 year after the inclusion

Ability to perform different activities assessed by the Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale (DBS-QOLs) with the evaluation of the ability to perform different activities (from impossible to autonomous) and then to estimate the evolution of this ability since the intervention (worsening, no change, improvement)

Psychosocial assessment by the "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs) with yes/no/partially questions related to the emotional and psychological perception of patients and then to estimate the evolution of this thought since the intervention (worsening, no change, improvement)

Intervention's assessment by "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs) with yes/no question related to the intervention.

Life's Quality assessment by "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs) with squales from 0 (worst case) to 10 (best case).

Comparison between the "Functional" subscale of the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and the total score obtained on the Functional dimension (Mobility + Activity of Daily Living) of the "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs).

Comparison of the Thumia score assessed by the Beck Depression Inventory score & the "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs) through the answer the the emotional and psychological perception of patients which have 12 items (Impossible, third person help, technology help, autonomous)

Comparison of the Life's quality assessed by the Short Form (36) Health Survey (SF-36) and by the "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs) with different yes/no question

Comparison of the sentivity to change between the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and the "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs) through the evolution of the answer of DBS-QoLs and BFMDRS during the following of the patient.

Assessment of the correlation between the functional fain & the benefit felt by the patient with the DBS-QOLs

Assessment of the correlation between the measure of benefit in terms of functional gain and the benefit experienced by the patient with all the answer of all module in "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs).

Assessment of the tolerance to surgery through the "surgical" module of the "Dystono-dyskinetic Brethomé-Sanrey Quality of Life scale" (DBS-QOLs) which is composed of yes/no questions.

Inclusion Criteria: Adulte between 18 an 80 years Patient with a generalized dystono-dyskinetic syndrome treated by Electrical Neuromodulation Continue and follow-up in the unit Subject affiliated to the French social security system Exclusion Criteria: non-French speaker patients who are physically unable to answer the questionnaire and who have no trusted person to help severe cognitive impairment (MoCA <10, MDRS <123, PM-38 <70) lack of informed consent