After Creutzfeldt-Jakob disease and mad cow disease were rampant a few years ago, these diseases had become quieter. But now there is a new variant of Creutzfeldt-Jakob disease (vCJD), the human form of BSE, so to speak, which begins insidiously with atypical symptoms.
What is the new variant of Creutzfeldt-Jakob disease?
There has not yet been a reported case of vCJD in Germany, but there are around 200 cases worldwide, most of them in Great Britain. While older people were primarily affected by the actual Creutzfeldt-Jakob disease, the age at vCJD is much lower. See percomputer for Semicircular Canal Dehiscence Explanation.
In order to curb the spread of vCJD, various consumer laws have been enacted, e.g. B. BSE tests on all slaughtered cattle over a certain age limit.
The new variant of Creutzfeld-Jakob disease is caused by misshapen proteins (prions) that can cause brain damage. The symptoms of vCJD closely resemble those of Creutzfeldt-Jakob disease. This type of diseases belongs to the “communicable, spongy brain diseases”. They are also called prion diseases because they are caused by prions.
The first case of Creutzfeldt-Jakob became known in 1995. About a year later, connections were recognized between this disease and mad cow disease (BSE), which occurs in cattle. In 1997 it was proven that prions are responsible for both BSE and Creutzfeldt-Jakob disease.
Causes
The new variant of Creutzfeldt-Jakob disease is triggered by faulty proteins (prions) found in nerve and defense cells.
Why they arise is not clear. It might be due to genetic changes. The prions have a different sequence of amino acids than normal proteins.
It has not yet been clarified how the prions damage the brain. Since they also do not trigger any immune reactions, diagnosis is very difficult.
Symptoms, Ailments & Signs
The new variant of Creutzfeldt-Jakob disease does not show any very specific symptoms at the beginning of the disease. Affected people experience mood changes and anxiety. These can be periodic at first and then manifest themselves. Depressive moods often occur. Hallucinations occur. Uncertainty when walking is the first motor sign.
In the further course, there is a lack of concentration and a steadily declining cognitive performance. Those affected suffer from an increasingly poor memory and thus show the symptoms of dementia. This is also accompanied by all other symptoms of dementia – such as disorientation and sudden aggressiveness. The ability to read, write and speak can be completely lost. Sometimes there is total indifference.
vCJD leads to further motor symptoms. Uncontrolled shaking occurs. Movements become more uncoordinated and can appear particularly expansive or paralyzed. The muscle disorders also lead to sudden movements and can further limit the ability to speak.
Diagnosis & History
To date, there is no diagnostic procedure that reliably detects the new Creutzfeldt-Jakob disease. Symptoms and complaints cannot be unequivocally assigned to vCJD and can be similar to those of other diseases. You can gain a little more certainty with a magnetic resonance tomography (MRT), in which damage to the brain tissue of those affected could be detected. A tonsil biopsy can be performed to detect prions.
It is a particular risk when people have contracted the new Jakob-Creutzfeldt disease but do not yet have any symptoms. If they donate blood, the prions can be passed on. For this reason, research is increasingly working on being able to detect the causative agent of vCJD using blood tests.
The difficult thing is that in the early stages of vCJD there are no characteristic symptoms that would allow conclusions to be drawn about vCJD. There are rather non-specific symptoms such as depressive moods, states of anxiety, concentration disorders, decrease in mental performance. Movement disorders and uncontrolled shaking (tremor) can also occur. As the disease progresses, paralysis, muscle twitching and uncontrolled movements can occur.
Reading and speaking skills are getting out of control. In the final stage, those suffering from vCJD can no longer contact their environment at all. They become apathetic, eventually pass out, and eventually die.
Complications
The complications of the new variant of Creutzfeldt-Jakob disease (vCJD) arise in direct connection with the symptoms of the disease. They occur both with and without treatment of the disease, for which there are no fundamentally effective drugs, only symptom-relieving drugs.
Psychological symptoms such as confusion, anxiety or depression often lead to misdiagnoses at the beginning of the disease. After just a few months, those affected are often no longer able to cope with normal everyday life on their own. Motor disorders such as unsteady gait or uncontrolled falling also increase the risk of accidents as a complication as the disease progresses.
Movement disorders and epileptic seizures are typical as the disease progresses and, as complications, can also lead to accidents and injuries to the patient. Urinary and fecal incontinence and progressive dementia characterize the more advanced new variant of Creutzfeldt-Jakob disease and ultimately require intensive around-the-clock care and nursing for the patient.
In the final stage, the patients are then no longer able to make contact with their environment. They are often in a state of complete spastic paralysis (so-called cerebral rigidity). In this terminal state, complications often include respiratory paralysis or pneumonia. In most cases, this leads to the death of the patient.
When should you go to the doctor?
If irregularities in well-being occur, special care should be taken with regard to one’s own feelings. A diffuse experience of fear, changing moods or a peculiarity of behavior are the first signs of a health disorder.
Being depressed, withdrawing from social life, or breaking off close contacts is cause for concern. This applies in particular if there are no incidents that could explain such a decision. The observations and changes should be discussed with a doctor so that a check-up can be initiated.
In the case of a decrease in mental performance or a lack of concentration, a doctor’s consultation is indicated. If the symptoms persist over a longer period of time or if they increase in intensity, medical help is required. Clarification of the cause is necessary so that a treatment plan can be drawn up.
Aggressive behavior, changes in personality and loss of memory should be examined. If there is disorientation or the loss of learned skills such as reading, writing or arithmetic, the person concerned needs help. In the case of disorders of the muscular system, an unnatural decrease in physical resilience and trembling limbs, a doctor’s visit is necessary.
Treatment & Therapy
The new variant of Creutzfeldt-Jakob disease cannot yet be treated effectively with medication. However, medicine is researching intensively to find drugs that can successfully combat vCJD. Until now, people suffering from vCJD have been treated with various medications, some of which can delay the course.
Muscle twitches can be treated with appropriate medication. However, since there is no medicine on the market for vCJD, vCJD is still fatal. The average life expectancy after infection with vCJD is about 14 months.
Outlook & Forecast
The new variant of Creutzfeldt-Jakob disease has an unfavorable prognosis. The health disorder is characterized by a strongly progressive course. Despite all efforts, no effective therapy has been found to stop the progression of the disease. Regardless of whether treatment is initiated or not, serious health irregularities occur that contribute to a reduced quality of life. The cognitive performance is severely reduced and means that intensive care is necessary.
Ultimately, after a severe course of the disease, the patient dies prematurely. Statistically, the patient is predicted to have a life expectancy of approximately 14 months after diagnosis. In addition to the cognitive problems, motor irregularities appear within a few months.
If the course of the disease is favorable, drug therapy delays the progression of the disease. However, this cannot be achieved in all patients and depends on the stage of the disease. Changes in behavior and personality can be expected. There are problems with orientation, states of confusion and immense loss of memory. The disease is therefore difficult to bear for the patient and especially for his relatives. In most cases, due to the severity of the symptoms, inpatient treatment is necessary at an advanced stage of the disease.
Prevention
Just as there is no cure, there is no way to prevent vCJD. Although there are consumer protection regulations, according to which beef and products made from it are paid close attention to the origin of the animals, this is not a guarantee. Cooking doesn’t help either, because the prions cannot be destroyed due to their heat resistance. However, since January 2001 there has been an EU decision that all slaughtered cattle intended for human consumption must be tested for BSE.
Aftercare
There are hardly any aftercare options for the new variant of Creutzfeldt-Jakob disease, since the disease cannot be cured and cannot be treated to any significant extent. It always ends with the death of the person affected and spontaneous healing has not occurred so far. So it cannot be assumed that medical follow-up care will play a role in vCJD in the future.
The many symptoms of brain failure cannot be treated either and therefore do not constitute a basis for aftercare. Adequate care for the patient is much more important – especially in the last months of his life. However, the rare cases of this disease sometimes bring new insights into the pathogen and the possible transmission routes. Research into Creutzfeldt-Jakob disease and the prevention of further outbreaks can therefore be seen as follow-up care.
More important is the psychological aftercare or care of the relatives. Since the new variant of Creutzfeldt-Jakob disease often affects comparatively young people, the shock is often deep, especially for the parents. Accordingly, relatives should not be left alone after the death of the patient and should be able to seek help if they wish. This depends heavily on the individual case. Understanding the illness can help in overcoming the loss.
You can do that yourself
There are no self-help measures that would counteract the new variant of Creutzfeldt-Jakob disease in any way. All self-help attempts that may be advisable using questionable substances, homeopathic remedies or alternative therapies have so far had no positive effect. Once the disease has broken out, it is considered incurable.
The only positive aspect that can still improve the quality of life of those affected is the fact that the course of the disease is well known. When the first symptoms appear, those affected know what to expect. It is then up to the patient himself how he would like to use his remaining months. Because those affected can live almost normally – albeit with restrictions – for a few months despite symptoms, they are well advised to use this time extensively.
Especially in the early stages, which is characterized by anxiety and mood swings, human support is all the more important. The quality of life of those affected is improved by the fact that they are taken care of. Activities should be included. As long as those affected are still able to move, they should be encouraged to do so.
The other stages, which are also associated with pain, also offer no scope for medical self-help measures. Here, too, only human attention comes into consideration.
