Anti-NMDA receptor encephalitis

The anti- NMDA receptor encephalitis is an inflammation of the brain (encephalitis), are detectable in the antibodies against the NMDA receptor.

Classification, incidence, and risk groups

The anti- NMDA receptor encephalitis was first described in 2007. Data on the frequency of the disease are not yet available. There are currently (April 2011) described about 420 cases. It ill mainly women and girls (about 80 % of sufferers are female ), the average age of onset is 23 years ( age approximately between 22 months and 79 years ).

Symptoms

The disease often begins with a flu-like preliminary, insomnia, loss of appetite, confusion, followed by psychological symptoms such as anxiety, agitation, bizarre behavior, delusions and hallucinations. A large proportion of patients passes first psychiatric treatment. Within weeks, seizures and catatonia -like disturbances of consciousness are added.

At this stage, involuntary movements, swallowing disorders can to other symptoms (food and drink can not be even brought to them ), immobility, breathing disorders, occurring cardiac arrhythmia, blood pressure and temperature fluctuations as well as hands- outs (complete loss of speech ). It is a serious disease, patients are often treated for long periods in intensive care units.

According to anecdotal reports, the anti - NMDA receptor encephalitis can result in older children to acute beginning, autism -like symptoms ( late onset autism ).

Cause, diagnosis,

The anti- NMDA receptor encephalitis is an autoimmune disease. The body forms defensive substances (antibodies) against a protein that plays an important role in signal transmission in the brain, the NMDA receptor. In approximately 60 % of adult patients one finds a ovarian tumor ( teratoma ) which contains, among other nerve cells. In these patients, anti - NMDA receptor encephalitis is a paraneoplastic syndrome. Teratomas are very rarely found in children and adolescents. In other patients, one finds no underlying disease.

The suspicion is made based on the clinical syndrome and a mostly elevated cell count in the cerebrospinal fluid ( CSF) and secured by the detection of antibodies against NMDA receptors in the spinal fluid or serum ( blood).

Many of the patients have an abnormal EEG. Only about half of the patients has visible changes in the brain in magnetic resonance imaging.

Treatment, prognosis

You try to suppress with the help of the immunosuppressive drugs misdirected immune response of the body. The most common glucocorticoids and intravenous immunoglobulin administration may be used. If one finds a tumor, it will be removed. A so-called plasmapheresis, the antibodies will be filtered from the blood and replaced with a replacement antibody.

Approximately 75% of patients are healthy or survive with only minor neurological problems. Approximately 21 % of patients survive with severe neurological damage, estimated 4% of patients die from the disease. Recent research shows more than half of patients who are treated with an immunosuppressive therapy, after four weeks of improvement. More than 80 % complete or at least very good recovery was observed in this study and 12% were within two years of a relapse.

The prognosis is better for patients who have a tumor is detected and removed. It is in most patients, a consistent loss of memory ( amnesia) for the duration of the disease. Relapses are possible. If the disease is detected early and treated properly, the prospect of full recovery is very good. Patients need to learn everything, physiotherapy (movement), speech therapy ( speech and swallowing ) and occupational therapy ( Pictorial perception and memory ) in various therapies again.