Clinical, cognitive and neuropsychiatric correlates of personality in Wilson’s disease

Introduction: Wilson's disease (WD) is an autosomal recessive disorder characterized by hepatic, neurologic, or psychiatric symptoms. Compared to other recognized clinical symptoms, the personality profile of WD patients remains under-investigated. This study compared personality traits in a sample of adults with WD and healthy controls (HC). Moreover, we assessed the cognitive and affective correlates of personality within the WD group. Methods: We enrolled 20 patients affected with genetically confirmed WD, with prevalent extrapyramidal symptoms, and 20 age and education-matched HC. Participants completed the revised Eysenck Personality Questionnaire, neuropsychological tests, and psychological questionnaires. Results: WD patients scored significantly higher on neuroticism (Cohen's d = 1.28; Confidence Interval: 0.61-1.95) and psychoticism (Cohen's d = 0.72; Confidence Interval: 0.08-1.36) compared to HC, but no differences were observed for extraversion. Among WD patients, neuroticism correlated with global disability, trail making test, depression, and mental health status; psychoticism correlated with memory and executive functions (all r > ± 0.5). Conclusion: We observed that WD patients are characterized by high neuroticism and psychoticism, and this personality profile is related to other clinical, cognitive, and neuropsychiatric manifestations. Identification of personality traits can enhance early detection, improve monitoring, and guide targeted management strategies for individuals with WD.