Anti-NMDA Receptor Encephalitis: Causes, Symptoms & Treatment

Anti-NMDA receptor encephalitis is an inflammation of the brain. The medical term for brain inflammation is encephalitis. Because antibodies against the NMDA receptor are present in this particular form of inflammation, it is called anti-NMDA receptor encephalitis.

What is anti-NMDA receptor encephalitis?

Anti-NMDA receptor encephalitis was classified as a disease only a few years ago. Along with various other infectious diseases of the brain that have been largely unrecognized, the disease has been the subject of increased research since 2000. However, there is currently little confirmed data regarding the incidence of anti-NMDA receptor encephalitis. However, it can be determined with a relatively high degree of probability that approximately 80 percent of all patients are female. The average age at which the disease occurs is 23 years. The age spectrum of patients ranges from 22 months to 79 years. It is also noteworthy that not only humans can contract anti-NMDA receptor encephalitis, but also other mammals. Polar bear Knut from the Berlin Zoo also died from anti-NMDA receptor encephalitis.

Causes

The causes responsible for the development of anti-NMDA receptor encephalitis have not yet been adequately explored. However, based on current knowledge, it can be assumed that specific genetic factors influence the disease. This is because anti-NMDA receptor encephalitis is an autoimmune disease. Medical research shows that autoimmune diseases have a genetic component in most cases. This argument is also supported by the enormous difference in the frequency of the disease in women and men. It must also be taken into account that certain inflammations promote the development and onset of anti-NMDA receptor encephalitis. This is because a large proportion of those affected suffer from infections at the onset of the disease. However, anti-NMDA receptor encephalitis is not an infectious disease. Most importantly, the disease is not transmissible to other individuals, as is the case with viral infections. Since anti-NMDA receptor encephalitis is an autoimmune disease, the organism forms antibodies against the so-called NMDA receptor in the brain. This is a protein that is important for the transmission of signals in the brain. In addition, about 60 percent of adults affected suffer from an ovarian tumor, which is associated with anti-NMDA receptor encephalitis. In turn, other sufferers show no underlying disease.

Symptoms, complaints, and signs

In a large number of cases, anti-NMDA receptor encephalitis announces itself with flu-like symptoms. Patients also complain of loss of sleep, appetite, and confusion. In addition, there are often psychological symptoms in the form of anxiety and bizarre behavior up to delusions and hallucinations. For this reason, the majority of patients first seek psychiatric treatment. Over the course of a few weeks, epileptic seizures and impaired consciousness often occur. Other potential symptoms of anti-NMDA receptor encephalitis include dysphagia and involuntary movements up to and including immobility. Cardiac arrhythmias, respiratory impairment, and fluctuations in blood pressure and body temperature are also possible. Observations suggest that anti-NMDA receptor encephalitis may lead to autism-like symptoms in older children.

Diagnosis and course

A number of investigative methods are available for the definitive diagnosis of anti-NMDA receptor encephalitis. The treating specialist decides on the diagnostic procedure depending on the individual case. So far, immunoglobulins from class G have been found in all patients. In addition, immunoglobulins from class A were detected in 30 percent of the patients. Currently, the performance of these tests is not yet standardized worldwide. Due to different laboratory methods, it is possible that anti-NMDA receptor encephalitis is overlooked, even if the affected persons show corresponding symptoms. Suspicion of the presence of anti-NMDA receptor encephalitis is usually based on the clinical syndrome. An increased number of cells in the cerebrospinal fluid also indicates the disease.The detection of endogenous antibodies against the NMDA receptors in both serum and cerebrospinal fluid confirms the diagnosis. In addition, many patients show changes in the EEG. Approximately half of the individuals also show changes in the brain that are visible by magnetic resonance imaging.

When should you see a doctor?

Unfortunately, anti-NMDA receptor encephalitis is detected relatively late because the symptoms closely resemble the flu or a cold, so the disease is diagnosed late. However, the affected person should consult a doctor if there is a loss of appetite or insomnia. A doctor should be consulted especially if the symptoms persist for a longer period of time. Likewise, changes and disturbances of the personality, anxiety states, or a versatility can indicate anti-NMDA receptor encephalitis and should therefore be examined by a physician in any case. It is also not uncommon to experience cardiac or respiratory symptoms, so sufferers may require a physical examination. Furthermore, hallucinations or swallowing difficulties may also indicate anti-NMDA receptor encephalitis. Especially in children, early diagnosis and treatment is necessary to avoid further complications. Children may also show symptoms of autism in the process. In general, anti-NMDA receptor encephalitis can be treated by a general practitioner in the first instance. In most cases, treatment of this disease is by surgery to remove the tumor. Furthermore, psychological treatments are also necessary. The relatives or the parents may also need psychological treatment in this case.

Treatment and therapy

In the course of anti-NMDA receptor encephalitis, the use of psychotropic drugs is usually necessary. The goal of this drug treatment is to calm the patient and reduce anxiety and psychotic symptoms. In addition, immunosuppressive arnze drugs are used to try to stop the organism’s faulty defense reactions. In most cases, intravenous immunoglobulins and glucocorticoids are used. If a patient suffers from a tumor, its removal is necessary. The prognosis of anti-NMDA receptor encephalitis is predominantly positive. Approximately 75 percent of those with the disease can be cured or carry only mild neurological damage from the disease. Approximately 21 percent carry severe neurological impairment from the disease. Approximately four percent of those affected die from anti-NMDA receptor encephalitis. Generally, the prognosis is better for individuals who have a tumor that is eventually removed. If the disease is detected and treated early, the chances for a complete cure are very good.

Outlook and prognosis

A sufferer can expect relief and cure of symptoms only in inpatient treatment. Without it, there will be an increase in existing symptoms and further symptoms. If the disease remains undiagnosed and untreated, it can have a fatal course. Without intervention, the inflammatory process of the brain can continue unhindered and lead to a failure of individual systems. Ultimately, there is a threat of systemic collapse resulting in death. Without competent medical care, the risk of loss of life and the presence of permanent damage increases considerably. If intensive medical treatment takes place in time, the prospects of recovery depend on the progress of the disease. In cases of anti-NMDA receptor encephalitis detected at an early stage, there are good drug treatment options that can be applied. In these cases, the patient can expect to be discharged from the hospital after a few weeks. However, follow-up treatments are necessary and there is a risk of relapse. It is not yet possible to give a long-term prognosis because the disease is a recently discovered disease. It was first diagnosed a few years ago, so research on it has not yet been fully completed. What is known so far is that even several years after the onset of the disease, targeted therapies can be used to improve the current state of health.

Prevention

Because anti-NMDA receptor encephalitis has not yet been adequately studied, no firm conclusions about measures to prevent the disease are possible to date.Because it is an autoimmune disease with a genetic component, it can be assumed that preventive measures other than screening may not exist.

Follow-up

Follow-up care usually proves relatively difficult in anti-NMDA receptor encephalitis, so affected individuals rely primarily on medical treatment for this disease. In this regard, a complete cure cannot always be guaranteed, so life expectancy may be reduced. The treatment of anti-NMDA receptor encephalitis is usually carried out with the help of medication. Patients should take care to take the medication regularly and correctly and also consider possible interactions with other medications in order to avoid complications and other complaints. The earlier the anti-NMDA receptor encephalitis is detected, the higher the chance of a complete cure. If anti-NMDA receptor encephalitis is caused by a tumor, it usually requires surgical removal. After such a surgical procedure, affected individuals should always rest and not exert their bodies unnecessarily. Stress should also always be avoided. Furthermore, regular examinations of the body for cancer are useful to prevent the tumor from spreading. In general, contact with other anti-NMDA receptor encephalitis sufferers can also have a positive effect on the course of the disease, as this often leads to an exchange of information.

What you can do yourself

Anti-NMDA receptor encephalitis is a genetic inflammatory disease of the brain. Affected individuals suffer increased infections until symptom onset, which are not transmissible. With this symptom, self-help methods can only be applied to a limited extent. First of all, bad lifestyle habits such as smoking, medication, drug and alcohol abuse should be discarded. A light diet rich in vitamins and foods rich in omega-3 fatty acids support the immune system, especially when psychotropic drugs are used. As soon as states of exhaustion and loss of appetite become more frequent, a nutritionally based diet is an important support in the psychological management of the symptom. Immunosuppressive preparations curb the defective defense reaction of the organism. More than half of adult women affected by the symptom have an ovarian tumor. Therefore, when the disease appears, a thorough gynecological examination should be performed as a self-help measure. If massive swallowing and cardiovascular difficulties occur, emergency medical services should be called. In order to be able to cope with everyday life for the time being if the symptom progresses, it is advisable to seek help from relatives or third parties. Assisted living or a temporary stay in a psychiatric rehabilitation clinic is recommended. As the disease progresses, it can lead to confusion, panic behavior with uncontrollable movements, delusions, hallucinations, and epileptic seizures. Thus, the use of sedative medications with concomitant psychological care is explained.