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Psychiatric and behavioral abnormalities in Wilson's disease
1Department of Psychiatry, University of Pittsburgh, Western Psychiatric Institute and Clinic, Pennsylvania 15213.
Advances in Neurology
|January 1, 1995
Summary
Psychiatric and behavioral abnormalities are common and often initial symptoms of Wilson's disease (WD), frequently leading to delayed diagnosis. Some symptoms improve with WD-specific treatment.
Area of Science:
- Neurology
- Psychiatry
- Genetics
Background:
- Wilson's disease (WD) is a rare genetic disorder characterized by copper accumulation.
- Psychiatric and behavioral abnormalities are recognized but often overlooked manifestations of WD.
Purpose of the Study:
- To outline the spectrum and significance of psychiatric and behavioral symptoms in Wilson's disease.
- To highlight the diagnostic challenges and delays associated with the psychiatric presentation of WD.
- To discuss the reversibility of these symptoms with treatment.
Main Methods:
- Literature review and clinical experience with Wilson's disease patients.
- Analysis of psychiatric and behavioral manifestations and their diagnostic timelines.
Main Results:
- Psychiatric and behavioral abnormalities occur in a high percentage of symptomatic WD patients (30-100%).
- These symptoms are often the initial presentation, leading to diagnostic delays of 1-5 years.
- Common symptoms include personality changes, depression, cognitive decline, and psychosis; some are reversible with treatment.
Conclusions:
- The psychiatric presentation of Wilson's disease is crucial for early diagnosis.
- Recognizing these symptoms and including WD in the differential diagnosis can prevent significant delays.
- Early diagnosis and treatment are essential for favorable outcomes, with potential for symptom reversal.