Leukencephalopathy represents 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 leukencephalopathy, there are impairments of sensory and motor functions and processes. Basically, leukencephalopathy primarily affects individuals who suffer from a weakness of the immune system.
What is leukencephalopathy?
Leukencephalopathy occurs primarily in those people who have lowered T-cell immunity. Far less commonly, the disease affects those individuals who exhibit only weakened humoral immune functions. It is also significant that a large proportion of patients with leukoencephalopathy have AIDS at the same time. More rarely, leukoencephalopathy develops after artificial or drug-induced suppression of the natural defenses (medical term immunosuppression). Bone marrow transplantation also partially increases the risk of the disease. Basically, leukencephalopathy affects the central nervous system. The virus responsible for the disease belongs to the group of so-called polyomaviruses. In the course of the disease, the functions and processes of the central nervous system change, which manifests itself, for example, in motor function disorders.
Causes
Leukoencephalopathy develops as a result of infection with the JC virus. The name of the virus is derived from the first letters of the person who first described it. Characteristically, the initial infection with the virus is without symptoms. Only a new infection leads to the development of leukoencephalopathy. Already in children, the pathogen spreads in the organism. The virus remains in the organism of infected persons for the whole life. It is supposed that the corresponding virus spreads from the bones or kidneys to the central nervous system in patients with weakness of the immune system. The transport medium may be leukocytes. Replication of the virus occurs 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 belongs to the so-called demyelinating diseases, which are called demyelinating diseases by medical term. In this case, the nerve sheaths become diseased and degenerate. Inflammatory processes are involved. Since the virus remains in the body of the infected person for the rest of his or her life, the pathogens affect more and more areas of the brain over time. The myelin sheaths are particularly affected by the demyelination processes.
Symptoms, complaints, and signs
The symptoms of leukoencephalopathy are varied. The expression of the individual symptoms depends primarily on which areas of the central nervous system are affected by the pathogens. In this way, various demyelinating lesions are formed within the nervous system. In the case of an infection of those areas of the brain that are responsible for motor function, disturbances of movement 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. It is not uncommon for confusion and impaired concentration to occur. Even dementia is possible. In addition, some patients suffering from leukoencephalopathy suffer from epileptic seizures.
Diagnosis and course of the disease
If a person suffers from the typical symptoms of leukoencephalopathy, a visit to a physician is recommended. The latter discusses with the patient his or her medical history, individual lifestyle, and possible chronic diseases. Following the anamnesis, various examinations are performed. The focus is on the clinical appearance of the leukoencephalopathy. Neuropathological analyses allow the detection of a specific protein of the JC virus. It is also possible to detect the genome of the virus. An examination of the urine, on the other hand, is insufficient. Although the virus can often be detected, this has little significance with regard to an actual disease. This is because approximately one fifth of all people permanently excrete the virus in their urine without suffering from leukencephalopathy.The diagnosis can be confirmed by magnetic resonance imaging. However, differentiation from multiple sclerosis or posterior reversible encephalopathy syndrome is difficult here without a thorough history. In addition, JC virus can be identified in brain tissue by electron microscopic examination. In principle, a meticulous differential diagnosis is highly relevant. If leukoencephalopathy is suspected in association with AIDS, patients should be examined for various encephalitides. These types of diseases include, for example, cryprococcosis, toxoplasmosis, and HIV encephalopathy.
Complications
First and foremost, affected individuals suffer from a severely weakened immune system as a result of leukoencephalopathy. This results in more frequent infections or inflammations, so that the patient’s quality of life and ability to cope with stress are considerably reduced by the disease. Furthermore, disturbances of motor function or movement may occur. Patients often appear clumsy and are unable to perform ordinary everyday tasks. In some cases, patients are then dependent on the help of other people in their daily lives. Likewise, there may be discomfort with speech, so that communication with other people is equally severely limited. There is a lack of comprehension and, furthermore, disturbances in concentration and coordination. If the leukoencephalopathy is not treated, it can also lead to dementia and further to epileptic seizures. Causal treatment of leukoencephalopathy is not possible in many cases. It may be necessary to remove an organ responsible for the disease. Without treatment, this usually results in the death of the patient. Complications occur mainly in people with a weakened immune system, so they need additional treatment.
When should you go to the doctor?
If symptoms such as bloody urine or feces are noticed, medical advice is indicated. Other warning signs that need to be clarified quickly include cramping in the abdomen, discomfort, and diarrhea and vomiting. General malaise should also be investigated if it persists for more than a few days. If the feeling of illness occurs in conjunction with skin changes and reduced performance, a physician must be consulted. If left untreated, leukoencephalopathy can lead to complications such as impaired consciousness and cardiac arrhythmias. If these symptoms occur, an emergency physician must be alerted. The affected person must then be treated in a specialist clinic, as there is an acute danger to life in the advanced stages of the nerve disease. Depending on the symptoms, the general practitioner will consult other doctors such as cardiologists, neurologists, gastroenterologists and rheumatologists. If there are psychological complaints accompanying the illness or if the patient’s health is generally rather poor, a therapist may be called in. Individuals who have recently experienced ammonia poisoning should call 911 immediately.
Treatment and therapy
Treatment of leukoencephalopathy focuses on strengthening the immune system of affected patients. For AIDS patients, so-called high-dose antiretroviral treatment increases longevity. This also reduces the symptoms of the disease at the same time. This is because the number of T cells increases after the therapy. If immunosuppression occurs as a result of transplantation of an organ, it is sometimes necessary to remove the organ in question. Causal therapeutic options for leukoencephalopathy are not known. The prognosis of the disease is also not very positive. Only an improvement and strengthening of the immune functions has a positive effect on the outcome of the disease. Otherwise, diseased patients die after approximately three months to two years.
Outlook and prognosis
Without medical care, there is a steady increase in existing symptoms and health irregularities. The disease trigger can continue to spread in the organism and trigger a continuous weakening of the immune system. In severe cases, there are permanent impairments of organ activity, a permanent malaise and various functional disorders. Irregularities in motor function as well as locomotion set in and have an increasing disease course.If left untreated, the affected person will ultimately die prematurely, as the immune system as a whole is so weakened that it can no longer stand up to external influences. With early medical care, the patient’s survival can be ensured. Nevertheless, there is a risk of lifelong disturbances of brain activity or movement remaining. In some cases, an organ transplant is needed because the body can no longer defend itself against the pathogens on its own. In order to secure the life of the affected person, a strengthening of the immune system is needed. Despite all efforts, the mortality rate of leukoencephalopathy is very high. A large number of patients die within two years of diagnosis. Often, other diseases are present that also result in a weakening of the immune system. This development worsens the prognosis and further shortens the expected lifespan.
Prevention
No effective measures for prevention of leukoencephalopathy are yet known.
Follow-up
There are no follow-up measures that can be taken when the disease is present. Rather, follow-up measures must be based on the different conditions that result in the diagnosis. Especially patients who have AIDS at the same time have hardly any possibilities to positvi influence the course of the disease. However, in some cases the condition of the patients can be improved by a consistent drug therapy. Follow-up examinations consist of monitoring the CD4 values and the viral load. Therefore, regular check-ups with the treating physician are necessary to keep track of the course of the disease. This means checking blood values to detect and monitor secondary infections. The use of immunosuppressants (where appropriate) should be urgently reconsidered. To prevent further opportunistic infections, individuals should live a very healthy lifestyle that strengthens the immune system as best as possible. This includes a healthy diet, exercise, and abstaining from substances that negatively affect the immune system-such as alcohol and nicotine.
Here’s what you can do yourself
Patients with leukoencephalopathy suffer from limitations in movement as well as sensory function. As a result, they are no longer able to manage their daily lives independently as the disease progresses. As a result, the quality of life and mental well-being of those affected suffer. To cope with everyday life, patients seek help from relatives or external care services in order to live in their own homes for as long as possible. However, as soon as the affected person is permanently dependent on care, a move to a nursing facility is usually necessary. Due to the reduction in motor skills, many patients develop inferiority complexes and even depression. All psychological complaints require treatment, so that patients turn to a psychotherapist. It is particularly important for the course of the disease that the ill persons consult the responsible medical professionals at regular intervals for the purpose of control examinations. Patients are prescribed and administered various drugs that strengthen the immune system. In order to support the drug therapy in the best possible way, those affected also strengthen their defenses by adopting a healthy lifestyle. This includes a special diet plan and advice on physical activity. At best, sport takes place within the framework of physiotherapy, whereby the therapist responds to the patient’s condition and promotes his or her motor skills.