Contents
Prions are infectious protein molecules that arise from proteins in our body that are initially not dangerous. When it comes to a natural conformation, they become an infectious prion protein.
Prion diseases and their symptoms
Prion diseases are groups of infectious diseases that occur in humans and animals. It is caused by an accumulation of abnormal infectious protein particles, or prions. Diseases caused by prions include:
- Creutzfeldt-Jakob disease, which has several forms,
- Gerstmann, Straussler and Scheinker disease,
- choroba kuru,
- fatal family insomnia.
Unfortunately, diseases caused by prions remain untreated, despite the fact that medicine is already very well developed. Diseases can be sporadic, inherited, and also acquired. In the latter case, they are considered infectious diseases and are transmitted through the food or blood. Fortunately, prion diseases are very rare. For example, the kuru disease was present only in New Guinea, and with the ban on cannibalism, it ceased to affect people. Unfortunately, it is difficult to recognize prion diseases in the early stages. There are various neurological, dementia and psychiatric symptoms.
Symptoms of individual diseases:
- sporadic Creutzfeldt-Jakob disease – the disease progresses rapidly, leading to death within 8 months. At first, the patient experiences progressive dementia and disturbances in gait and speech. These symptoms are also associated with behavioral disturbances, tremors, limb stiffness, seizures, and visual disturbances.
- variant of Creutzfeldt-Jakob disease – the course of the disease is more aggressive than the case described above. Cerebellar (gait and speech) disorders occur in all sick people. The accompanying symptoms are dementia, irritability, sleep disturbances, depressed mood, sensory disturbances, convulsions, difficulty swallowing, urinary and faecal incontinence, and high blood pressure.
- Gerstmann, Straussler and Scheinker disease – the disease leads to death within 3-8 years. The disease occurs in families that are burdened with the gene mutation. The first symptoms include progressive dementia, movement disorders, as well as convulsions, nystagmus, visual and hearing disturbances,
- fatal family insomnia – like the previous disease, it affects people with a gene mutation. The disease can be fatal in six months. The main symptoms are insomnia, disturbances in temperature regulation, hypertension, increased heart rate, rapid breathing and sweating.
People who experience the above-mentioned symptoms should see a doctor. Unfortunately, diagnosing prion diseases is difficult. The doctor must conduct a detailed interview with the patient. Most likely, they will also order an MRI. A cerebrospinal fluid test is also performed. Occasionally, a brain biopsy should be performed. All these tests are needed to rule out other diseases that may manifest themselves in a similar way.
Treatment of diseases caused by prions
Prion diseases progress very quickly. Therefore, treatment begins before the final diagnosis of the disease. The patient is under specialist neurological or psychiatric care. Unfortunately, there is no specific drug that can cure a sick person. Therefore, treatment is symptomatic. The patient is given anticonvulsants, antipsychotics, drugs that improve memory and alleviate parkinsonian symptoms.
Can you avoid getting sick?
Prion diseases are very rare, but there is no way to prevent sporadic or inherited forms. The risk of developing Creutzfeldt and Jakob variants is very small, since cattle spongiform encephalopathy occurs much less frequently. In addition, careful selection of blood donors is to help in not passing the disease on. It is worth knowing that prion proteins are not destroyed despite sterilization. Therefore, the neurosurgical instruments used to examine sick people cannot be reused.