ABSTRACT
OBJECTIVE: The aim was to elucidate the personality traits of patients with treated Wilsons disease (WD) in comparison to healthy volunteers. METHOD: Twenty-five WD patients, ten females and 15 males, with a mean age of 35.2 +/- 8.3 years completed the Karolinska Scales of Personality (KSP), a self-report inventory comprising 15 separate scales. The results were compared to a control series comprising 200 men and 200 women drawn from the general population. RESULTS: The patients with treated WD scored significantly lower than the healthy controls on aggressivity-hostility-related scales and the scale measuring Psychic Anxiety. Patients with predominantly hepatic symptoms had the lowest aggressivity-related scores and patients with predominantly neurological symptoms had the lowest Irritability, Guilt and Detachment scores and the highest Impulsiveness and Muscular Tension scores. Both groups scored low on the Somatic Anxiety scale. CONCLUSION: The present results illustrate that patients with treated WD have significant deviations in personality traits, especially in aggressivity-hostility-related scales and Psychic Anxiety, compared to healthy controls when investigated by means of a self-report inventory, the KSP. The deviations were not related to age, age at onset or duration of the disease.
Subject(s)
Aggression/drug effects , Arousal/drug effects , Hepatolenticular Degeneration/drug therapy , Hostility , Penicillic Acid/analogs & derivatives , Penicillic Acid/therapeutic use , Personality Inventory , Trientine/therapeutic use , Zinc Acetate/therapeutic use , Adult , Female , Follow-Up Studies , Hepatolenticular Degeneration/diagnosis , Hepatolenticular Degeneration/psychology , Humans , Male , Middle Aged , Penicillic Acid/adverse effects , Psychometrics , Reproducibility of Results , Trientine/adverse effects , Zinc Acetate/adverse effectsABSTRACT
The study aimed to describe the neuropsychological profiles in patients with treated Wilson's disease (WD). The series included 19 symptomatic and 2 asymptomatic patients with a mean age of 35.3 +/- 9.2 years. They were tested with the Automated Psychological Test system (APT), a comprehensive computerised neuropsychological test battery. APT comprised eleven separate tests and assessed five essential types of neuropsychological functions: motor functions, basic neuropsychological functions, specific cognitive functions, memory, and executive functions. The results were compared to current norms of the test battery. The symptomatic WD patients had significantly lower performance than the norms on all finger tapping tasks, the simple reaction time, the simultaneous capacity background task, the short-term memory test, the index of word decoding speed, the grammatical reasoning test, and the perceptual maze test. They were significantly higher on the index of impulsive errors, and used a significantly more global processing mode in the test of selective attention. The female symptomatic patients displayed more pronounced neuropsychological deficits than the males in the complex tasks. WD patients displayed a specific profile of moderate neuropsychological impairment. The results are theoretically interesting and have practical implications for the management of WD patients, e.g. some patients confronted with the results have had increased compliance.
Subject(s)
Copper/metabolism , Metal Metabolism, Inborn Errors/psychology , Adult , Age of Onset , Aging/psychology , Cognition/physiology , Copper/blood , Copper/urine , Female , Humans , Liver Diseases/etiology , Liver Diseases/psychology , Male , Maze Learning/physiology , Memory/physiology , Metal Metabolism, Inborn Errors/physiopathology , Middle Aged , Nervous System Diseases/etiology , Nervous System Diseases/psychology , Neuropsychological Tests , Psychomotor Performance/physiology , Reaction Time/physiology , Sex Characteristics , SyndromeABSTRACT
OBJECTIVE: To examine the occurrence and severity of psychopathological symptoms in patients with treated Wilson's disease (WD) and to evaluate the clinical utility of a self-assessment. METHOD: Twenty-six consecutive patients with confirmed WD were investigated using the Comprehensive Psychopathological Rating Scale (CPRS) and the CPRS Self-rating Scale. RESULTS: The total CPRS scores ranged from 2.5 to 59.0 (mean 29.4 +/- 15.5). Most common symptoms were: autonomic disturbances, observed muscular tension, fatiguability, reduced sexual interest, lack of appropriate emotion, concentration difficulties, reduced sleep, aches and pains, hostile feelings, apparent sadness and failing memory. Agreement between interview-based ratings and self-ratings was low. CONCLUSION: The patients with treated WD have prominent psychopathology in the same range as in patients with moderate to severe depressive disorders. A specific symptom profile has been identified. If confirmed, the identification of the typical symptom profile might be of great importance. The patients with WD tend to underestimate the presence of psychopathological symptoms.
