Leukoencephalopathy is a disease of the central nervous system. It is usually caused by the so-called JC virus. It is an acute disease characterized by a progressive course. In the context of leukoencephalopathy, impairments of sensory and motor functions and processes occur. Basically, leukoencephalopathy primarily affects people who suffer from a weakness of the immune system.
What is Leukoencephalopathy?
If symptoms such as bloody urine or faeces are noticed, medical advice is indicated. Other warning signs that need to be clarified quickly are cramps in the abdomen, abnormal sensations, diarrhea and vomiting. See AbbreviationFinder for abbreviations related to Leukoencephalopathy.
Leukoencephalopathy occurs primarily in people who have reduced T-cell immunity . Far more rarely, the disease affects people who only show weakened humoral immune functions. It is also significant that a large proportion of patients with leukoencephalopathy also have AIDS.
Less frequently, leukoencephalopathy develops after an artificial or drug-induced suppression of the natural defenses (medical term immunosuppression). Bone marrow transplantation also partially increases the risk of the disease.
Basically, leukoencephalopathy affects the central nervous system. The virus responsible for the disease belongs to the group of so-called polyomaviruses. As part of the disease, the functions and processes of the central nervous system change, which manifests itself, for example, in motor disorders.
Causes
Leukoencephalopathy arises as a result of infection with the JC virus. The virus’ name is derived from the initials of the person who first described the virus. It is characteristic that the initial infection with the virus proceeds without symptoms.
Only renewed infection leads to the development of leukoencephalopathy. Even in children, the pathogen spreads in the organism. The virus stays in the organism of the infected person for a lifetime. It is suspected that the corresponding virus spreads from the bones or kidneys into the central nervous system in patients with a weakened immune system.
The transport medium may be leukocytes. The virus replicates in the white tissue of various brain areas, including the cerebrum and cerebellum. The same process is also possible in the spinal cord. In principle, leukoencephalopathy is one of the so-called demyelinating diseases, which are called demyelination diseases with the medical term.
The nerve sheaths become diseased and degenerate. Inflammatory processes are involved in this. Since the virus remains in the body of the infected person for a lifetime, the pathogens infect more and more areas of the brain over time. The myelin sheaths in particular are affected by the demyelination processes.
Symptoms, Ailments & Signs
The symptoms of leukoencephalopathy are varied. The severity of the individual symptoms depends primarily on which areas of the central nervous system are affected by the pathogens. In this way, various demyelination foci form within the nervous system.
If those areas of the brain that are responsible for motor function are infected, movement disorders develop. The same applies to impairments of the speech center, which often result in aphasia. The more the disease spreads over time, the more pronounced the cognitive impairments become.
Not infrequently, states of confusion and disturbances in the ability to concentrate occur. Even dementia is possible. In addition, some patients suffering from leukoencephalopathy suffer from epileptic seizures.
Diagnosis & course of disease
If a person suffers from the typical symptoms of leukoencephalopathy, a visit to a doctor is recommended. He discusses the patient’s medical history, individual lifestyle and possible chronic diseases with the patient. Following the anamnesis, various examinations are carried out.
The focus is on the clinical appearance of leukoencephalopathy. A special protein of the JC virus can be identified in neuropathological analyses. It is also possible to detect the genome of the virus. However, an examination of the urine is insufficient.
The virus can often be found here, but this has little significance with regard to an actual disease. Because around one fifth of all people excrete the virus permanently through the urine without suffering from leukoencephalopathy. The diagnosis can be confirmed by magnetic resonance imaging.
However, it is difficult to differentiate from multiple sclerosis or a posterior reversible encephalopathy syndrome without a thorough medical history. In addition, the JC virus can be identified with the help of electron microscopic examinations in the tissue of the brain.
Basically, a meticulous differential diagnosis is highly relevant. If leukoencephalopathy is suspected to be associated with AIDS, patients should be evaluated for diverse encephalitis. This type of disease includes, for example, cryprococcosis, toxoplasmosis and HIV encephalopathy.
Complications
First and foremost, those affected by leukoencephalopathy suffer from a severely weakened immune system. Infections or inflammations occur more frequently, so that the patient’s quality of life and resilience is significantly reduced by the disease. Motor and movement disorders can also occur. The patients often seem clumsy and cannot carry out normal everyday things.
In some cases, patients then turn to the help of other people in theireveryday lifereliant. It can also cause problems when speaking, so that communication with other people is just as severely restricted. It comes to an adeptness and furthermore also to disturbances of the concentration and the coordination. If the leukoencephalopathy is not treated, it can also lead to dementia and further to an epileptic seizure.
In many cases, causal treatment of leukoencephalopathy is not possible. An organ that is responsible for the disease may have to be removed. Without treatment, the patient usually dies. Complications mainly occur in people with a weakened immune system, which means that they need additional treatment.
When should you go to the doctor?
If symptoms such as bloody urine or faeces are noticed, medical advice is indicated. Other warning signs that need to be clarified quickly are cramps in the abdomen, abnormal sensations, diarrhea and vomiting. A general malaise should also be investigated if it persists for more than a few days. If the feeling of illness occurs in connection with skin changes and loss of performance, a doctor must be consulted. Left untreated, leukoencephalopathy can lead to complications such as impaired consciousness and cardiac arrhythmia. If these symptoms appear, an emergency doctor must be alerted.
The affected person must then be treated in a specialist clinic, since the advanced stage of the nerve disease is acutely life-threatening. Depending on the symptoms, the family doctor will consult other doctors such as cardiologists, neurologists, gastroenterologists and rheumatologists . If there are psychological complaints accompanying the illness or if the patient’s general state of health is rather poor, a therapist can be called in. People who have recently suffered from ammonia poisoning should call an ambulance immediately.
Treatment & Therapy
Treatment of leukoencephalopathy focuses onimmune systemof the affected patients. In the case of AIDS patients, the lifespan is increased by so-called high-dose antiretroviral treatment. This also reduces the symptoms of the disease at the same time. After the therapy, the number of T-cells increases.
If immunosuppression occurs as a result of organ transplantation, it is sometimes necessary to remove the organ in question. Causal treatment options for leukoencephalopathy are not known. Also theforecastof the disease is not very positive.
Only an improvement and strengthening of the immune functions has an effectpositiveon the outcome of the disease. Otherwise, the sick patients die after about three months to two years.
Outlook & Forecast
Without medical care, there is a steady increase in existing complaints and health irregularities. The disease trigger can spread further in the organism and trigger a continuous weakening of the immune system. In severe cases, there are permanent impairments of organ activity, permanent malaise and various functional disorders.
Unregelmäßigkeiten der Motorik sowie der Fortbewegung setzen ein und haben einen zunehmenden Krankheitsverlauf. Unbehandelt kommt es letztlich zu einem vorzeitigen Ableben des Betroffenen, da das Immunsystem insgesamt so geschwächt ist, dass es sich gegen keine äußeren Einflüsse mehr behaupten kann. Bei einer frühzeitigen ärztlichen Versorgung kann das Überleben des Patienten gesichert werden. Dennoch besteht das Risiko, lebenslange Störungen der Gehirntätigkeit oder der Bewegungsabläufe bestehen bleiben.
In some cases, an organ transplant is required because the body can no longer defend itself against the pathogens on its own. In order to secure the life of the person concerned, a strengthening of the immune system is required. Despite all efforts, the death rate from leukoencephalopathy is very high. A large number of patients die within two years of being diagnosed. There are often other diseases that also weaken the immune system. Thesedevelopmentworsens the prognosis and additionally shortens the expected lifespan.
Prevention
There are still no known effective measures to prevent leukoencephalopathy.
Aftercare
There are no follow-up measures that can be taken in the clinical picture. Rather, the aftercare measures must be based on the different conditions that result in the diagnosis. Above all, patients suffering from AIDS at the same time have hardly any possibilities of positively influencing the course of the disease. In some cases, however, the patient’s condition can still be improved by consistent drug therapy. Follow-up examinations consist of monitoring CD4 counts and viral load everywhere.
Therefore, regular check-ups by the doctor treating you are necessary to monitor the course of the disease. This means checking blood values to detect and monitor subsequent infections. The use of immunosuppressants (where appropriate) should be urgently reconsidered.
To prevent further opportunistic infections, those affected should live a very healthy lifestyle that strengthens the immune system as much as possible. This includes a healthy diet, exercise and avoiding substances that negatively affect the immune system, such as alcohol and nicotine.
You can do that yourself
Patients with leukoencephalopathy suffer from restricted mobility and sensory functions. As a result, as the disease progresses, they are no longer able to cope with everyday life independently. As a result, the quality of life and mental well-being of those affected suffer. In order to cope with everyday life, patients seek help from relatives or external nursing services in order to live in their own home for as long as possible. However, as soon as those affected are constantly dependent on care, it is usually necessary to move to a care facility.
Due to the reduction in motor skills, many patients develop inferiority complexes and even depression. All psychological complaints require treatment, so patients turn to a psychotherapist.
It is particularly important for the course of the disease that the sick person consults the responsible doctor at regular intervals for check-ups. Patients are prescribed and administered various medications that strengthen the immune system. In order to support the drug therapy in the best possible way, those affected also strengthen their immune system through a healthy lifestyle. This includes a special diet plan and advice on physical activity. At best, sport takes place as part of physiotherapy, whereby the therapist responds to the patient’s condition and promotes his or her motor skills.
