Objective: Development and validation of the Relationship Self-Questionnaire (RSQ), specific to Huntington’s disease (HD), which aims tomeasurepatients self-perceived quality of their relationships (RS).

Background: In HD, social cognition disorders cause relational difficulties and contribute to social withdrawal of patients, often more than motor or cognitive symptoms. Existing tools assess the negative aspects (symptoms), relying on the expertise of health professionals or opinions of the patient’s relatives, but rarely the patient’s insight.

Method: A focus group including health professionals, relatives, representatives of HD associations and interviews with patients identified 122 questions qualifying the patients relationship. Then, experts in social cognition excluded redundant and ambiguous sentences and established a list of 49 questions, to be evaluated on a 6-point Likert scale (the RS questionnaire, RSQ). It was administered to 24 pre-manifest HD, 68 manifest HD and 71 controls. Exploratory factor analyses (EFA), performed with gene carriers’ outcomes, allowed characterizing scale dimensionality of the RSQ. We also explored psychometric characteristics of the scale using general least-squares linear models and convergent validity (Spearman) between the RSQ dimensions and scores of the Unified Huntington’s Disease Rating Scale (UHDRS), Problem Behaviours Assessment Scale (PBA), Hospital Anxiety and Depression Scale with Snaith Irritability Scale(HAD-SIS)).

Results: The EFA identified 16 items to group into 2 dimensions, D1 and D2, focusing on positive or negative aspects of RS, respectively. A RSQscore was computed by subtracting D1 to D2 scores. The RSQscore was lower in pre-HD than in controls and in HD and controls score lower than in the one of HD (p<0.05). Inverse results are observed for the D1(pre-HD > controls > HD). Pre-HD D2 score tended to be lower than in HD ones (p=0.06). The RSQscore correlated with each UHDRS scores (total functional capacity (p=0.01), total motor score (p<0.05), dependence (p=0.01)). All RSQ scores correlated with psychiatric scales (PBA, HAD-SIS (p<0.05)).

Conclusion: The RSQ allows a measure relationship of HD gene carriers without focusing on symptoms. HD perceive their relationship more negatively than pre-HD and controls while pre-HD perceive them more positively. It will be invaluable in personalized care.

References: Bachoud-Levi AC et al. (2006). Lancet Neurol, 5(4):303-309. Bora et al.(2016). Behavioural Brain Research, 297; 131-140 Craufurd et al.(2001). Neuropsychiatry Neuropsychol Behav Neurol, 14 : 219-226

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MDS Abstracts - https://www.mdsabstracts.org/abstract/measure-of-social-relation-in-huntingtons-disease-the-relationship-self-questionnaire-rsq/