A genetic disorder called Wilson’s disease affects copper metabolism and leads to low serum and hair copper levels but high levels in the liver and brain. This can be a serious and even fatal problem unless treated by chelating agents; BAL or penicillamine is most often used as it binds copper in the gut and carries it out.
Signs, symptoms & indicators of Wilson's Disease
Blue and brown fingernails
Conditions that suggest Wilson's Disease
Schizophrenia
In Wilson’s disease the small intestine absorbs too much copper and the liver excretes too little of it, resulting in a copper buildup in the liver and brain. Onset is slow and begins between 11 and 25 years of age. A wide array of symptoms occur, fitting a number of psychiatric diagnoses, including major depression, schizophrenia, and hysteria. Children with Wilson’s disease can appear to be mentally retarded. Appetite loss and weight loss can appear along with hallucinations and delusions. The physical manifestations of Wilson’s disease do not appear until the late stages, thus it is easily misdiagnosed as psychiatric illness.
Wilson's Disease can lead to
Schizophrenia
In Wilson’s disease the small intestine absorbs too much copper and the liver excretes too little of it, resulting in a copper buildup in the liver and brain. Onset is slow and begins between 11 and 25 years of age. A wide array of symptoms occur, fitting a number of psychiatric diagnoses, including major depression, schizophrenia, and hysteria. Children with Wilson’s disease can appear to be mentally retarded. Appetite loss and weight loss can appear along with hallucinations and delusions. The physical manifestations of Wilson’s disease do not appear until the late stages, thus it is easily misdiagnosed as psychiatric illness.
Recommendations for Wilson's Disease
Zinc
Supplemental zinc therapy (in capsules or tablets of 50mg of elemental zinc, 3 times daily, separated from food by at least 1 hour) was found to be an effective sole therapy in the long-term treatment of Wilson’s disease. This study was a ten year follow-up of 141 Wilson’s disease patients. Zinc helps block the absorption of copper and acts to remove accumulated copper from the body as well as prevent its accumulation. [J Lab Clin Med, 1998;132: pp.264-278]
Copper
People with Wilson’s disease should eliminate copper as far as possible from their diet, environment and supplement sources.
Key
| Weak or unproven link |
| Strong or generally accepted link |
| Likely to help |
| Highly recommended |
| Avoid absolutely |
Glossary
Copper
An essential mineral that is a component of several important enzymes in the body and is essential to good health. Copper is found in all body tissues. Copper deficiency leads to a variety of abnormalities, including anemia, skeletal defects, degeneration of the nervous system, reproductive failure, pronounced cardiovascular lesions, elevated blood cholesterol, impaired immunity and defects in the pigmentation and structure of hair. Copper is involved in iron incorporation into hemoglobin. It is also involved with vitamin C in the formation of collagen and the proper functioning in central nervous system. More than a dozen enzymes have been found to contain copper. The best studied are superoxide dismutase (SOD), cytochrome C oxidase, catalase, dopamine hydroxylase, uricase, tryptophan dioxygenase, lecithinase and other monoamine and diamine oxidases.
Metabolism
The chemical processes of living cells in which energy is produced in order to replace and repair tissues and maintain a healthy body. Responsible for the production of energy, biosynthesis of important substances, and degradation of various compounds.
Serum
The cell-free fluid of the bloodstream. It appears in a test tube after the blood clots and is often used in expressions relating to the levels of certain compounds in the blood stream.
Schizophrenia
Any of a group of psychotic disorders usually characterized by withdrawal from reality, illogical patterns of thinking, delusions, and hallucinations, and accompanied in varying degrees by other emotional, behavioral, or intellectual disturbances. Schizophrenia is associated with dopamine imbalances in the brain and defects of the frontal lobe and is caused by genetic, other biological, and psychosocial factors.
Hallucination
A false or distorted perception of objects or events, including sensations of sight, sound, taste, smell or touch, typically accompanied by a powerful belief in their reality.