Psychosocial Adjustment

One-third of children with Wilson's disease will initially present with psychiatric symptoms, and nearly 60% will eventually have psychiatric manifestations of the disorder. Patients with Wilson's disease have at least one of the following problems at some point in the course of the disorder: dementia, psychosis, impulsivity, and oppositional defiant disorders that can later develop into disturbances of conduct. It is not unusual for patients with impulsive and acting-out behaviors to eventually become involved with police and the courts. Studies in adult patients have noted that psychosis is relatively rare in patients with Wilson's disease. The disease instead presents with personality change, depression, and cognitive impairment (Dening and Berrios

1990). In summary, Wilson's disease should be considered when a young patient has a family history of jaundice, evidence of neuropsychiatric disorder and premature deaths in siblings, sudden onset of psychiatric disorder with extrapyramidal symptoms, bleeding symptoms, recurrent or pathological fractures, and poor response to therapeutic interventions (Srinivas et al. 2008).

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