Wilson's Disease
NeurologyOverview
Wilson's disease (hepatolenticular degeneration) is an autosomal recessive disorder of copper metabolism in which copper accumulates in the body, mainly affecting the brain and liver. Symptoms include liver disease and neurological features such as tremor, muscle rigidity, and speech difficulty.
Symptoms
- Limb tremor
- Dysarthria
- Dystonia
- Jaundice
- Kayser-Fleischer corneal rings
Treatment
Lifelong low-copper diet along with copper-chelating drugs such as penicillamine or zinc, with regular monitoring of copper metabolism.
Dietary advice
Strict low-copper diet, avoiding animal organs, nuts, shellfish, and chocolate.