Limbic Encephalitis: Causes, Symptoms & Treatment

Limbic encephalitis is a disease of the central nervous system associated with inflammatory processes. The term ‘limbic encephalitis‘ encompasses a number of different subacute conditions that affect the central nervous system. Limbic encephalitis predominantly manifests in adult individuals who suffer from symptoms such as epilepsy, mental health problems, or memory difficulties as a result of the disease.

What is limbic encephalitis?

The disease term originated from several neurologists in the United Kingdom who identified inflammation in the limbic system in some ill people. Basically, limbic encephalitis is associated with cancer in about 60 percent of all patients. The medical term for this is paraneoplastic limbic encephalitis. Likewise, non-paraneoplastic limbic encephalitides exist, which in most cases represent diseases of the human immune system.

Causes

The causes of limbic encephalitis are differentiated according to the two subcategories of the disease:

Paraneoplastic limbic encephalitis is a type of disease often triggered by cancer. In more than half of affected individuals, this form of limbic encephalitis develops as a result of carcinoma. The inflammatory processes then occur as a direct result of the cancer. The disease of non-paraneoplastic limbic encephalitis, on the other hand, is not due to malignant tumors. Instead, in this case, the affected individuals suffer from diseases of the body’s own defense system. It is also possible that a possible cancer has not yet been diagnosed. The exact reasons for the development of the autoimmune disorder have not yet been sufficiently researched. In some patients, certain antibodies are found that attack specific structures of the central nervous system and neurons.

Symptoms, complaints, and signs

The symptoms of limbic encephalitis are relatively general in the majority of cases and also occur in a variety of other diseases. For this reason, diagnosis of limbic encephalitis is often difficult. Typically, individuals suffering from limbic encephalitis experience seizures of an epileptic nature, for example, or exhibit memory difficulties. In addition, some individuals also experience psychological problems, such as euphoria, depression, apathy, or personality changes. Basically, there are no differences in symptoms between the paraneoplastic and nonparaneoplastic forms of limbic encephalitis.

Diagnosis and course of the disease

It often takes a long time for limbic encephalitis to be correctly diagnosed. This is because the typical symptoms of the disease also occur in various other diseases, as they are primarily common general symptoms. It was only a few years ago that appropriate standards for diagnosing the disease were established. In any case, the patients suffer from the so-called limbic syndrome. The onset of this phenomenon is not more than five years ago. Decisive criteria consist of affective disorders, difficulties with new memory, and seizures of the temporal lobes. The affected patient is usually seen and examined by a neurologist. The anamnesis before the examination clarifies the individual complaints in connection with the person’s lifestyle. Information about any existing chronic diseases or specific diseases in the patient’s past helps the physician to approximate the disease at hand. Also relevant are familial and genetic dispositions of limbic encephalitis in the affected patient. Clinical examination usually consists of an MRI scan and histologic analysis. Based on certain criteria, limbic encephalitis can be diagnosed comparatively clearly. The decisive factor is, among other things, the detection of a so-called chronic temporomedial encephalitis. Laboratory examinations primarily involve analysis of the cerebrospinal fluid. The so-called cerebrospinal fluid comes from a lumbar puncture. In the majority of cases of limbic encephalitis, there is evidence of inflammatory processes. Another characteristic feature of the disease is the proliferation of lymphocytes, also known as lymphocytic pleocytosis.Oligoclonal bands as well as barrier defects are also sometimes detected.

Complications

In most cases, this disease is diagnosed relatively late, resulting in late treatment of the disease. Likewise, the complaints are not particularly characteristic and can occur thereby also with various other diseases. Those affected usually suffer from epileptic seizures and severe muscle cramps. In most cases, these are associated with severe pain. The patient may also become confused and have memory problems. Those affected can no longer remember events correctly and are thus significantly restricted in their daily lives. Furthermore, the disease also leads to depression and other psychological upsets. The personality of the affected person can also change negatively as a result of the disease, with social discomfort being the main cause. The treatment of the disease can take place with various medications. It is not uncommon for the relatives or parents to have to be treated as well, since it is not uncommon for them to experience psychological discomfort as well. As a rule, a complete cure cannot be achieved. However, special complications do not occur.

When should one go to the doctor?

In the event of an epileptic seizure, a visit to the doctor is generally necessary to clarify the cause. Seizures any doctor should be examined and treated by a doctor. If there are changes in memory activity, restrictions in the usual ability to remember, or if acquired knowledge cannot be recalled as usual, a physician should be consulted. Reduced performance or slight fatigue when recalling memory content must be examined more closely by a doctor. If objectively false memories are repeatedly noticed or if the person suffers from memory lapses, a physician is needed. Sudden changes in the usual personality of the affected person or abnormalities in his usual behavior should be clarified by a doctor. If people in the immediate environment notice strong changes in the demeanor, expressions or attitudes of the patient, a doctor should be alerted. Social withdrawal, depressive moods or apathetic behavior are considered worrisome. They should be presented to a physician as soon as they persist over several weeks or increase in intensity. A strong euphoric appearance without reason or increased irritability should also be discussed with a physician. If the affected person experiences a diffuse feeling of illness, a general malaise, or changes in his or her perception, the complaints should be discussed with a physician.

Treatment and therapy

In both forms of limbic encephalitis, immunotherapy is used first. Even a suspicion of the disease is reason enough to initiate appropriate treatment measures. As soon as the MRI examination gives indications of limbic encephalitis, immunotherapy also begins in numerous cases. This usually involves the use of substances such as immunoglobulins and glucocorticoids. In addition, it is possible to treat limbic encephalitis with plasmapheresis. As soon as more precise knowledge is available about the antibodies or carcinomas present in the individual case, the physician makes appropriate changes in treatment. When a tumor is identified, efforts focus on its therapy. In cases without cancer, the success of immunotherapy is evaluated after a quarter of a year. If there are no positive effects, the type of immunotherapy is usually changed. If treatment still does not have the desired effect after that, continuation of immunotherapy is appropriate only in cases of limbic encephalitis associated with VGKC antibodies.

Outlook and Prognosis

Limbic encephalitis is characterized by problems of diagnosis. Often, because of the parallel nature of complaints of different diseases, the patient undergoes a variety of tests and conjectures of causes until complete clarification occurs and final diagnosis is established. This circumstance makes an early and comprehensive therapy more difficult. The physical as well as the mental condition is therefore often excessively battered at the time of diagnosis. In addition, a complete recovery is often not achieved despite all efforts and use of various therapeutic approaches.Nevertheless, the symptoms that have occurred can be treated symptomatically with the current medical options. This makes it possible to live with the disease and leads to an improvement in the quality of life. The prognosis worsens if a tumor is noticed as the cause of the encephalitis. The course of the disease and the treatment options are linked to the size of the tumor and the general health of the affected person. In unfavorable cases, premature death of the affected person occurs. Without seeking medical care, a steady increase in symptoms is to be expected. In addition to the physical irregularities, emotional stress situations become apparent. Therefore, psychological sequelae may occur. These significantly worsen the patient’s general state of health and can trigger further impairments in the organization of life.

Prevention

Current options for prevention of limbic encephalitis are limited. The causes identified to date, such as autoimmune diseases and cancer, are themselves pathologic phenomena that have not been adequately studied. Patients with characteristic symptoms of limbic encephalitis are advised to consult a specialist immediately and have the origin of the symptoms clarified.

What you can do yourself

Patients with limbic encephalitis have no way to achieve a cure for the disease on their own. Various measures can be taken in everyday life to achieve an improvement in well-being, but recovery is only possible with the cooperation of physicians. For strengthening the psyche, conversations with friends and confidants help. Through them, the patient can express his emotions and receive feedback as well as valuable tips that can help change his own thoughts. In self-help groups or forums, sufferers can exchange ideas and share their experiences with each other. In addition, relaxation techniques are suitable for reducing the stress of everyday life and creating an inner calm and balance. Techniques such as yoga, meditation, autogenic training or Qi Gong can be used by the patient independently as well as in small groups and experience a stabilization of the inner balance. With a positive basic attitude and a basically optimistic attitude towards the challenges of life, the affected person can achieve a significant improvement of his situation. Sufficient exercise, various leisure activities and a healthy lifestyle help to build up the body’s defenses as well as overcome the psychological symptoms. Sufficient sleep and avoidance of harmful influences at various levels can be a valuable support for the patient in coping with the disease.