28 Wilson's Disease

• Teenage years
Signs and Symptoms
• Silent disease until intra-hepatic and extra-hepatic copper
deposition begins to manifest clinically
– Age 6-30
• Incidental elevated LFT Overt liver failure
• 282 patients:
– Neurologic 69%
• Parkinsonism, Dystonia, Cerebellar and Pyramidal sings
– Hepatic 15%
– Hepato-neurologic 2.5%
– Osseomuscular 2%
– Pure psychiatric 2%
– Asymptomatic 5%