Does Wilson's disease determine specific personality traits? Analysis of patients with the neurological form of the disease

Background: In the autosomal recessive disorder of copper metabolism, Wilson's disease (WD), patients present hepatic, neurological, and psychiatric symptoms combined in different and unpredictable ways. Brain pathology primarily affects the basal ganglia and may cause movement, cognitive, affective, and behavioral disturbances. Personality traits have been rarely studied in the neurological form of the disease. Objective: To investigate whether patients with the neurological form of WD (WDn) have specific personality traits, especially those typical of frontobasal loop dysfunction. Methods: The personality of 29 adult WDn patients (without significant cognitive and psychiatric disorders) and 29 matched healthy controls was assessed using the Minnesota Multiphasic Personality Inventory (MMPI-2). Results: Compared to healthy individuals, patients with WDn scored higher on the following MMPI-2 scales: hypochondriasis, physical malfunctioning, bizarre sensory experiences, health concerns, and general health concerns, with no differentiation between patients and controls on the remaining MMPI-2 scales. Conclusions: Long-term treated patients with WDn did not appear to have any specific personality traits reflecting unambiguously frontobasal psychopathology, if self-report measurement is used. The more strongly exhibited features concerning their actual physical ailments and health anxiety, such as hypochondria, chronic fatigue, and poor psychophysical well-being, could result from psychological reactions to a chronic disabling brain disease. Further study of personality traits and personality disorders is warranted in patients with clearly defined forms of WD, using various measurement tools. Psychopathological disorders in different forms of WD warrant further studies.