Wilson's disease, also known as hepatocerebral degeneration, is a hereditary disease, characterized by excessive copper accumulation in the body.This disease often affects the brain and liver, causing different nerve and liver-related symptoms.Nowadays, with the advancement of medical science, the understanding of Wilson's disease is becoming more and more profound.However, when the disease shows neurological symptoms, it can often be misdiagnosed or ignored.
Wilson's disease occurs due to mutations in the ATP7B gene, which is responsible for transporting excess copper into bile for excretion.When the gene is mutated, the body cannot effectively eliminate copper, causing its accumulation in the liver and brain.
Wilson's disease has a variety of neurological symptoms, and about half of the patients will experience problems such as cognitive decline, behavioral changes, and muscle stiffness.The most common neurological symptoms include:
Many patients are not clear about their status when they first develop neurological symptoms, and may even attribute these conditions to other causes, missing out on the opportunity for an early diagnosis.
The liver symptoms of Wilson's disease usually precede neurological symptoms, manifesting as fatigue, jaundice, ascites and coagulation problems.Patients with liver damage often seek medical help in adolescence, however, many patients associated with neurological symptoms are often diagnosed in their twenties or even later.
Diagnosis of Wilson's disease usually includes blood tests, urine tests, and liver biopsy.Because the symptoms of Wilson's disease may be similar to many other diseases, the diagnosis is relatively complicated.The testing items will involve the copper content, ceruloplasmin level, etc.
In all confirmed cases, bronze protein levels were significantly reduced in about 80% to 95% of patients.This indicator is not only used for the diagnosis of Wilson's disease, but also helps doctors track the progress of the disease.
Treatment of Wilson's disease usually involves dietary adjustment and medication.Patients should follow a low-copper diet to avoid copper-rich foods such as nuts and seafood.In addition, options for drug treatment include copper removal agents and zinc supplements, which are designed to reduce the copper content in the body.
In addition to medicine and diet, patients' lifestyles also need to be adjusted.For example, avoiding copper kitchenware is an important life detail.In addition, early diagnosis and treatment can greatly improve the prognosis and reduce the risk of later development into serious health problems.
In short, the impact of Wilson's disease on the nervous system cannot be underestimated.If a relative has been diagnosed with Wilson's disease, genetic screening and health assessment should be carried out as soon as possible so that corresponding prevention and treatment measures should be taken.Have you also considered whether you or your family have potential health risks?