Copper poisening - Wilson's desease

Copper is important for building bones, connective tissue and pigment in the hair. In addition, it is important for a properly functioning immune system that protects the body against micro-organisms such as viruses, fungi and bacteria.
Copper is absorbed through the intestinal wall from food such as grain products, vegetables and meat. The liver excretes excess copper through the bile ducts. An excess of copper can cause irritated intestinal and mucous membranes.

Copper storage disease

In people with Wilson's disease, this process does not go well and copper accumulates in the liver. This is because these people lack an enzyme (adenosine triphosphatase).
Wilson's disease is an inherited digestive disease that can also affect the brain in cases.

The disease bears the name of British physician Samuel Alexander Kinnier Wilson (1878–1937), a neurologist who described the condition, including the pathological changes in the brain and liver, in 1912. See here.
Both parents have passed on an abnormal gene (autosomal recessive inheritance), which can cause the disease to manifest itself over time.
The gene for Wilson's disease is located on chromosome 13 and encodes a copper-transporting P-type ATPase, -ATP7B.
Wilson's disease is present at birth but only manifests itself when copper has accumulated in the liver, brain and other organs.
The disease usually manifests itself between the ages of ten and thirty. It can also reveal itself earlier, after the age of five.
This may be accompanied by anemia due to the breakdown of blood cells, jaundice (icterus) or chronic or acute liver inflammation (hepatitis). If scar tissue develops in the liver due to long-term liver inflammation, it will function poorly (liver cirrhosis). In 70% of people with Wilson's disease, osteoporosis and/or osteoarthritis occur and these people break a bone more often. A yellow-green-brown ring around the iris of the eye is often seen (Kayser-Fleischer ring).

Image from https://commons.wikimedia.org/w/index.php?curid=2746925

Neurological and sometimes neuropsychiatric complaints

Copper can also build up in the brain. In approximately 40-50% of patients, the first symptoms of Wilson's disease manifest themselves in the central nervous system. In 10% of people with Wilson's disease, the first complaints are psychiatric in nature. It can cause damage to the brain with persistent neurological or neuropsychiatric complaints.

The following complaints may occur:

• increased muscle tension
  
• movement and gait disorders: involuntary movements and coordination disorders
  
• muscle cramps
• shaking, tremors
  
• disorders in fine motor skills that change handwriting and/or cause problems with writing.
  
• speech disorders (severe course)
  
• hallucinations (severe course)
  
• neuropsychiatric disorders; personality changes, depression, manic depressive syndrome (bipolar disorder), schizophrenic syndrome or
  psychosis (severe course).

Where in the brain

Copper overload (accumulation) in the brain leads to disturbances in the function of the basal ganglia (basal nuclei). The basal ganglia includes a group of structures that regulate, among other things, balance, posture and initiation of movements. (See our specific page on the basal ganglia).
As a result, patients with Wilson's disease usually exhibit movement disorders (extrapyramidal symptoms), with complaints similar to Parkinson's disease (parkinsonism) being very common.
MRI research shows that neurological damage with motor problems (tremor) is caused by damage (lesions) to 'parts' of the basal ganglia, namely: the globus pallidus, the head of the caudate nucleus and the substantia nigra.

Treatment

Treatment with medication is possible at an early stage.
The medications (chelating agents/chelation therapy) bind copper so that organs release the copper into the bloodstream. The kidneys then filter the blood and copper can be excreted. Another medicine is zinc. Zinc inhibits the absorption of copper from food. Because the enzyme is not produced, this therapy will have to take place throughout life.
A low-copper diet is necessary.
Copper is found in liver, crustaceans, mushrooms, nuts and chocolate.
If the liver is too damaged, a liver transplant should be considered.
It happens that neurological complaints in people disappear with medication and a low-copper diet. In others they remain and we speak of brain damage.