Rasmussen encephalitis is a very rare form of non- infectious inflammation in the cerebral cortex. An autoimmune process is suspected. The disease usually occurs in children and is almost always fatal if left untreated.
What is Rasmussen encephalitis?
Rasmussen encephalitis is named after Canadian neurologist Theodore Rasmussen. Rasmussen introduced surgical methods to cure epileptic diseases and in this context also dealt with the inflammatory disease of the cerebral cortex named after him. In English-speaking countries, Rasmussen encephalitis is also known as chronic focal encephalitis or CFE. For what is hypothyroidism disease, please visit beautyphoon.com.
This term describes chronic focal encephalitis. The disease then begins with a small focal inflammation in a specific, limited, small area of the brain and from there spreads more and more to the neighboring brain tissue. Usually only one cerebral hemisphere is affected.
In most cases, the inflammation does not spread to the opposite hemisphere. The disease is always associated with an irreversible loss of nerve cells. It leaves scars and, if left untreated, leads to death. Depending on the extent of the inflammatory processes, those affected also suffer from severe lifelong disabilities after treatment.
Rasmussen encephalitis usually occurs for the first time in children under the age of ten. In rare cases, however, the first symptoms of the disease are only found in adolescents during puberty or even in adults. Overall, the disease is very rare. Every year, for example, 50 new cases are registered in Germany. A prevalence of one to two people per 1,000,000 is assumed.
Causes
An autoimmune process is assumed to be the cause of Rasmussen encephalitis. So-called CD8 T cells play a special role here. CD8 T cells are T cells with an extra CD8 receptor protein in the cell membrane. The CD8 receptor is a co-receptor of the T cell receptor.
The cytotoxic T-cells recognize foreign protein structures through the CD8 receptors and stimulate an immune reaction, as a result of which the antigen-presenting cells are destroyed. It has now been recognized that CD8 T cells specifically seek out cells with specific antigenic structures and destroy them. It has not yet been possible to determine which structures are involved.
It is not clear whether these structures are of a viral nature or whether they are based on endogenous proteins. In the case of Rasmussen encephalitis, the CD8 T cells attack and destroy nerve cells and astrocytes. The CD8 T cells multiply very rapidly and can be detected in the blood for years. They enter the brain via the blood-brain barrier and cling to nerve cells and astrocytes.
There they specifically destroy cells with a specific antigen structure. Once cell destruction begins, this process can no longer be stopped. The inflammation progresses and leads to the destruction of other areas of the affected cerebral hemisphere. So far, immunosuppressants have only been able to reduce the inflammation, but still stop it.
Symptoms, Ailments & Signs
Rasmussen encephalitis usually begins with focal epileptic seizures. The seizures are localized on one side of the body. Later, neurological impairments such as hemiplegia, visual disturbances, speech disorders and mental retardation increasingly develop. The epileptic seizures can then generalize (grand mal).
In rare cases, both cerebral hemispheres are finally affected, which leads to bilateral failure symptoms. If no treatment is carried out, the prognosis of the disease is very poor. Ultimately, it always ends fatally. Treatment leads to better prognosis. However, depending on the extent of the brain damage that has already occurred, severe physical and mental disabilities can sometimes remain.
Diagnosis & course of disease
Imaging methods such as MRI examination, EEG and histological examination of brain tissue can be used to diagnose Rasmussen encephalitis. The EEG is about locating the area of the brain that triggers the seizure. The histological examination of the brain tissue is used for the differential diagnosis to rule out infectious causes for the encephalitis.
Complications
Serious complications always occur during the course of Rasmussen encephalitis. Typical of the disease are neurological impairments such as hemiplegia and mental retardation. As the disease progresses, visual and speech disorders as well as epileptic seizures also occur more frequently. In severe attacks, those affected often bite their lips and tongue until they bleed and suffer from severe twitching of their arms and legs.
Patients usually also suffer psychologically from the symptoms – anxiety disorders, depression or inferiority complexes occur. If left untreated, Rasmussen encephalitis is always fatal. Severe brain damage usually occurs beforehand, which in the further course leads to deficits, pain and finally to a coma. Various side effects and interactions can occur during treatment with immunosuppressants.
Possible symptoms are blood pressure fluctuations, increased blood fat levels and muscle weakness. In addition, late effects such as diabetes mellitus, osteoporosis and damage to the kidneys and liver can occur. Cardiovascular diseases and the development of skin and lymph node tumors can also occur in the long term. The concomitantly prescribed antiepileptic drugs and glucocorticoids harbor similar risks. Brain surgery can cause serious complications.
When should you go to the doctor?
Patients previously diagnosed with Rasmussen encephalitis should receive medical attention. If there is no diagnosis, there are some indications that a doctor’s visit makes sense. If a person affected has convulsions, it is advisable to consult a doctor or a neurologist straight away. First, he can clarify the cause of the cramps and second, he can prescribe appropriate medication that, on the one hand, reduces the general susceptibility to seizures and, on the other hand, an emergency medication that provides relief in the event of acute cramps.
Apart from the most obvious signs, it is also advisable to note changes in everyday life and, if necessary, to use them as an opportunity to see a doctor. For example, impaired vision or slurred, halting speech can already be signs of Rasmussen encephalitis. An accumulation of cognitive difficulties is also a warning signal that should be clarified by a doctor. Once the disease has been diagnosed, the doctor should be consulted regularly so that the medication can be adjusted. Blood tests and, if necessary, EEG’s are necessary for an optimal drug setting.
Treatment & Therapy
So far, there is no clear treatment recommendation for Rasmussen encephalitis. Treatment is often very difficult, especially as the disease progresses inexorably. An attempt is made to stop the encephalitis with a combination therapy of immunosuppressants. For this purpose, among other things, combinations of immunoglobulins, high-dose glucocorticoids and tacrolimus are administered.
The inflammation can probably be reduced, but the disease still progresses. The treatment of epileptic seizures is also very difficult. First of all, this is a symptomatic treatment that has no influence on the course of Rasmussen encephalitis.
However, it was also found that even the antiepileptic drugs used to date have made little progress in combating epilepsy. Real curative success, however, opens up the surgical therapy. The affected section of the cerebral hemisphere is removed. In severe generalized cases, however, the entire affected hemisphere must be removed to avoid spreading to the entire brain.
Without surgery, the prognosis of the disease is always poor. After surgery, the course of Rasmussen encephalitis can be stopped. Whether and which disabilities remain depends on how far the disease has progressed and how the child’s brain is able to regenerate to compensate for severe damage. In medical research, the focus is on finding effective drugs that can stop the progression of the disease without surgical intervention.
Prevention
There is currently no recommendation for the prophylaxis of Rasmussen encephalitis. The cause of the disease has not yet been fully elucidated.
Aftercare
Rasmussen encephalitis causes motor disorders and signs of paralysis. In severe cases, consequential damage remains in the affected area of the brain; if left untreated, the disease leads to death. For this reason, follow-up care is necessary. A recurrence of the symptoms should be avoided in the long term. During follow-up, the patient’s condition is controlled, the duration depends on the extent of the disease.
A neurologist performs follow-up care during regular check-ups. The effect of the medication administered is determined, side effects must be recognized and averted in good time. If the disease progresses severely, a brain operation is necessary. During aftercare, the healing process is monitored by a doctor, and after discharge, the specialist continues to monitor the patient. The goal is a successful elimination of the encephalitis without long-term effects.
If the inflammation has already caused physical or mental limitations, the patient receives support in everyday life. These measures are also part of the aftercare. The person concerned learns how to deal appropriately with the new situation. In addition to a specialist, it is advisable to visit an appropriate advice center. Severe disabilities require professional help from trained staff. As part of the aftercare, the patient is cared for professionally.
You can do that yourself
The treatment of Rasmussen syndrome can only be supported to a limited extent. Sick people have to learn how to deal with the disease in a healthy way, which is possible through literature and documentation, but also by visiting a specialist center for neurological diseases. Participation in self-help groups and talking to other affected people are important measures to make it easier to deal with the disease.
Under certain circumstances, drug treatment can be supported by various general measures such as exercise or a change in diet. Natural remedies can also provide relief. However, taking alternative medicine should be discussed with the doctor beforehand to avoid complications. It is best for those who are ill to go to a naturopath or a well-stocked drugstore to find the right medication for the respective symptoms. The antiepileptic treatment is primarily to be supported by attentively observing the person concerned, because then action can be taken immediately in the event of an attack.
In the long term, Rasmussen encephalitis patients must be hospitalized. Self-help measures are no longer effective in the later stages of the disease. However, relatives and friends are an important support for those affected.
