Wilson's Disease

Key points

• Wilson's disease is an autosomal recessive disorder characterised by excessive copper deposition in the tissues.
• liver: hepatitis, cirrhosis
• neurological: basal ganglia degeneration, speech and behavioural problems are often the first manifestations. Also: asterixis, chorea, dementia
• Kayser-Fleischer rings
• renal tubular acidosis (esp. Fanconi syndrome)