Autoimmune Encephalitis

Neurologist Dr. Doping speaks about studying autoimmune diseases, encephalitis and diagnosing tumor effect on the nervous system.

Autoimmune encephalitis - a condition in which a person in a matter of days and weeks developing quite vivid memory impairment, behavioral disorders, psychosis, any unusual movement. This is due to the formation of antibodies against its own nerve cells. Perhaps most clearly, this situation has been described in 2012 in a book by Suzanne Kehelen that she was ill with the disease. She has released a book "My Month of Madness", and, in fact, in this title is the essence of this disease. The author - a young girl who became ill, doctors have long tried to find out the causes of her condition. She was in a frenzy, as she herself says, and then, thanks to the correct diagnosis, is fully recovered - recovered so that I could write an interesting and fascinating book, which became a bestseller in America.

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The disease itself was described a few years earlier. In 2005, the first cases were identified among young women, who both suffered from behavioral disorders and unexplained nature of ovarian tumor. Accordingly, it has been suggested the so-called paraneoplastic nature of the disease, ie a disease that is caused by cross-reaction of the immune system between the tumor and its own nervous system.

What is paraneoplastic nervous system, what they are and why there was such an assumption? They were described and other paraneoplastic disease. There are diseases that damage the brain and spinal cord, damaging the neuromuscular division, damaging peripheral nerves. At the same time developing the clinic, ranging from encephalitis, that is an acute condition in which a person properly behaved: he psychosis, his unusual movements, he loses his memory, it can run into catatonia, coma, - up to myelitis, when human damaged spinal cord. It can be nerve damage and neuropathy dipole when developing weakness or disorder of neuromuscular transmission when coming develops fatigue and impaired muscle strength.

All this is due to formation of autoantibodies to various structures of the nervous system, namely, to intracellular antigens - is an antibody which in practice is called Anti-Ku, Anti-Jo, Anti-Ro, is intracellular antigens of the nervous system. Any antigen located on the surface of nerve cells of the central nervous system. One of them - it is the NMDA-receptor, which we will now speak in detail. There antigens located on neuromuscular synapses as calcium-dependent channels in the syndrome of Lambert - Eaton, for example.

Thus, in order to confirm the nature of the disease paraneoplastic need several factors, namely the presence of the tumor itself, the presence of antibodies and evidence that these antibodies bind directly to the immune system and cause the same neurological syndrome that we see in the patient. And in 2007, ie two years after the first description of acute cases of mental disorders of young women with a tumor, Professor Dalmau was confirmed this relationship between tumor formation of antibodies and the corresponding clinical manifestations. He gets in antibodies derived from blood, cerebrospinal fluid of patients in the sections of the nervous tissue of animals and noted that the antibodies of these biological fluids are associated primarily with hippocampal tissue. And then, thanks to the method of colocalization with known antibodies added to the same cuts, it became clear that this antibody to the NMDA-receptor.

NMDA-receptor - it receptors that transmit signals in the nervous system. Neurotransmitter is glutamate, and they are well represented in all parts of the nervous system. Currently cases sets 300-500 person been described, and that are directed toward the body NMDA-receptors, determines the clinical picture follows. These antibodies bind to receptors and cause their internalization, that is dipping into neurons, respectively, they go to the surface, and it turns out that degrades transmission through these receptors and attenuates the signal transmitted through the glutamate receptors. Glutamate - This excitatory neurotransmitter that serves conditionally positive signal between nerve cells. But since many of these receptors are inhibitory neurons and excites - GABA neurons, it turns out that the inhibition, ie a decrease in the excitation of inhibitory neurons, leading to disinhibition of front-strialnic ties in the system. It turns out, reducing the negative impact, that is, the inhibitory effect, leads to the fact that becoming more active front-strialnic system, which lead to the development of psychosis clinics, catatonia, to the whole of the unusual clinical picture that we see.

It is known that the determination of these antibodies in patients newly diagnosed with schizophrenia, such as schizophrenia, which was considered before, a positive result and 10% of cases. You can speculate and say that up to 10% of newly diagnosed schizophrenia in adulthood may be autoimmune, that is completely treatable, that at one time made a rather dramatic shift in consciousness as psychiatrists and neurologists regarding the diagnosis and treatment of mental illness. This disease is primarily manifested in women under 30 years, and more than half of them have a tumor, as we said at the beginning. Tumors - is primarily ovarian teratoma and teratoma, which are a variety of tissues. They are a generator of these antibodies, which subsequently cross-react with the immune system.

Treatment of this disease treatment corresponds to many autoimmune diseases, namely: immunosuppression with corticosteroids, leaching of plasmapheresis antibodies decrease B-cell activity, antibody producing monoclonal antibodies by introducing, for example, to receptors CD20, located on the B-cells; drug "Rituximab" or less-specific immuno-suppressive therapy in the form of injections "cyclophosphamide". And with regard to diagnosis and treatment is very important is to identify the primary tumor and remove it.

The most striking example to date of treatment known to me as a result of removal of the tumor is the case of a young girl who was 6 months in refractory, that is not curable, non-convulsive status epilepticus, which in itself is a very rare and life-threatening situation. Do not be effective or immuno-suppressive therapy, or antiepileptic therapy. She was diagnosed encephalitis induced antibodies NMDA-receptors, ovarian studies gave mixed results, but she, even though she was in a serious condition, laparoscopic surgery to remove the ovaries was carried out, and she came out of this status and relatively well recovered . That is such a dramatic improvement in the detection of possible tumors and their timely treatment.

Accordingly, in our country we are now conducting diagnostics of autoimmune encephalitis, and how researchers from around the world, and it becomes clear that not all antibodies are still unknown. Since, as in addition to those in the 2007, new NMDA-receptors as antigens these antibodies have been described, now apparently wider spectrum of these antibodies, which is known to us. Therefore, our work is that we diagnose some patients autoimmune encephalitis, but we collect the material, which is necessary in order to, in cases in which a high probability of the presence of a human autoimmune disease caused by antibodies to the cells of the nervous system, to accumulate the material to further identify new, hitherto unknown antibodies and hence the development of drugs, and more accurate diagnosis of these conditions. A treatment of these conditions, as seen in the example, can dramatically affect human life.