Anti-NMDA receptor encephalitis - symptoms, causes. Limbic encephalitis

Encephalitis is a common brain disease that takes several forms. The most common patients are those with brain inflammation caused by a tick bite from infected ticks, severe infections, or vaccinations. A little over 10 years ago a new form of encephalitis of autoimmune origin was discovered. This disease is called receptor encephalitis.

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is one of the most severe and dangerous forms of the disease, mostly affecting the female half of humanity. Statistics show that more than 90% of patients are women aged 20 to 40 years, although the disease was also diagnosed in young children and the elderly.

Causes and manifestations of pathology

Since there is still very little time since the description of the first officially registered case of manifestations of anti-receptor encephalitis, scientists are still trying to establish the main reasons for the triggering of the pathological process. But, as in the case of other autoimmune diseases, it is difficult to track the entire process and mechanism of development of receptor encephalitis.

At the moment, it is believed that the main causes of the development of the disease are malignant tumors and infectious diseases. Doctors believe that patients with cancer are at risk of developing receptor encephalitis.

This is due to the fact that half of the patients with antibodies to NMDA receptors were diagnosed with malignant neoplasms in the ovaries. In some cases, the disease is preceded by prolonged infections, but in a third of patients it is not possible to determine the cause of the autoimmune process.

The clinical picture also develops differently in each patient. In most cases, the first symptoms appear as an influenza state:

  • the head begins to hurt;
  • temperature rises;
  • the patient is sick;
  • there is a breakdown.

Later signs of a neuropsychiatric disorder begin to appear:

  1. Aggression.
  2. Memory losses.
  3. Incoherence of speech.
  4. Involuntary muscle twitching.
  5. Motor impairment.
  6. Convulsive seizures.
  7. Loss of consciousness.
  8. Impaired respiratory function.

Suspecting the disease is quite difficult, especially at an early stage. Some patients begin to manifest mild and not always noticeable to outsiders cognitive impairment:

  1. Disorientation in space.
  2. Apathy.
  3. Intrusive movements.
  4. Hallucinations.
  5. Anxiety.

Gradually, symptoms increase and turn into serious mental disorders. And if the symptoms of infection are not connected to them, then most patients turn to a psychiatrist, where they are given the wrong diagnosis: schizophrenia, bipolar disorder and others. This is a dangerous disease - it is not known how many patients with receptor encephalitis have been in psychiatric clinics for years and receive the wrong treatment.

In childhood, the main symptoms are convulsive seizures, behavioral disorders and motor disorders. In adolescents, under the influence of hormonal surges, they are rapidly developing:

  • hallucinations;
  • hyper excitability;
  • paranoid disorders.

With late diagnosis or lack of treatment, patients develop serious symptoms:


If you deal with the treatment of anti-receptor encephalitis at an early stage, then it is possible to achieve remission, but not a complete recovery, since the disease is incurable. In a third of patients it is possible to achieve a complete (or with minor impairment) elimination of symptoms, but in 25% of patients persistent mental deviations remain, dementia or death occurs in the first year of illness.

Diagnosis and treatment

In the diagnosis of receptor encephalitis, it is important not only to distinguish it from mental disorders, but also from other forms of the disease. If the patient suspects encephalitis, it is urgent to conduct the necessary examinations:

Auxiliary diagnostic methods:

  1. C-reactive protein.
  2. Rheumatoid factor.
  3. Antinuclear bodies.
  4. Blood biochemistry.
  5. Analysis of urine.
  6. Examination of the body (for the presence of an insect bite).

When making the diagnosis of “anti-receptor encephalitis”, it is necessary to refer the patient to an oncologist for a full examination to exclude malignant tumors - the entire treatment process will depend on the result. In most patients with oncology, improvement was observed after removal of the tumor. There was not only a decrease in the severity of symptoms, but there was a remission.

The main methods of therapy are taking or administering the following drugs:


Sedative and sedative drugs that relieve anxiety (Sedafiton, Sonnat), improve sleep and help reduce the symptoms of a mental disorder are mandatory.

Properly selected treatment allows you to stop the progression of the autoimmune process, but patients should remember that any malfunction in the body can again trigger the pathological mechanism. Therefore, it is extremely important to carefully monitor the state of your health and timely treat any emerging diseases.

Encephalitis associated with autoaggression of the immune system is not fully understood. This is a rather serious disease - inflammation of the very substance of the brain. It has been proven precisely that its development is associated with the development in the body of antibodies to receptors of its own nerve cells (neurons) located in different parts of the brain.

Causes of the disease

The first cases of this type of encephalitis were detected in women with ovarian tumors. Therefore, encephalitis was regarded as paraneoplastic, that is, developing with a malignant tumor.

Indeed, in many cases, encephalitis precedes a cancerous tumor (sometimes for several months and even years), and often develops already against the background of a malignant disease.

Scientists have managed to identify almost 30 antigens associated with the development of malignant processes in the body and associated with damage to nerve cells. A malignant tumor is detected in 60% of patients with clinical manifestations of encephalitis.

But in some cases, the neoplastic process is not detected, and encephalitis as a result of a malfunction in the immune system develops for some reason. And what causes the immune system to produce antibodies against CNS own nerve cells is not yet clear.

Typical manifestations of encephalitis in pediatric patients in many cases are not associated with any tumor. Antibodies in completely healthy children of different ages are produced spontaneously and also bind to the NMDA receptors of nerve cells in the brain.

By blocking these receptors, antibodies lead to the slow development of mental disorders, motor disorders and seizures.

These data confirm that many mechanisms leading to autoaggression and synthesis of antibodies to NMDA receptors of brain cells, to neuroimmune conflict, have not been studied, have not been established.

Epidemiology

Mostly women are affected, in men this type of encephalitis develops in isolated cases. The disease develops in youth, the average age of patients is about 25 years. About 40% of cases are teenagers under 18 years of age. It was noted that in male patients and at a younger age, encephalitis develops more often without a malignant neoplasm.

The role of autoimmune antibodies

The importance of antireceptor antibodies in the development of encephalitis is conclusively proved by the following data:

  1. In each case of encephalitis antibodies  to NMDA receptors of brain cells were found in the acute period of the disease in cerebrospinal fluid and blood serum. Moreover, a decrease in the titer of these antibodies was noted in the stage of recovery and recovery. In addition, there was a clear relationship between the number of antibodies and the outcome of the disease.
  2. Preparations Keiamin,  Phencyclidine and others in the NMDA receptor antagonist group are capable of causing manifestations similar to the symptoms of this encephalitis.
  3. In most cases, patients with anti-NMDA receptor encephalitis develop central hypoventilation.  This can be linked to the fact that the targets for autoimmune antibodies are neurons located mainly in the forebrain, which is affected by anti-NMDA receptor encephalitis. This leads to respiratory disorders.
  4. Hyperkinesis  manifested with this encephalitis are not associated with epilepsy. Proof of this is that sedatives and antiepileptic drugs are not effective. This is confirmed by the results of electroencephalographic observations of such patients.
  5. In each case, encephalitis was excluded viral infection,  as a cause of damage to brain matter: the results of a study of cerebrospinal fluid, blood, intravital biopsy of the brain substance and pathological analyzes did not detect viral markers.

Summarizing these facts and the results of numerous studies in patients, we can no doubt confirm the leading role of autoimmune aggression in the mechanism of development of anti-NMDA receptor encephalitis.

Clinical symptoms

The disease develops slowly at any age of the patient.

In its development, several stages are distinguished.

Prodromal symptoms

Not manifested in all patients (86% of patients), this period lasts about 5 days.

The prodromal stage is characterized by nonspecific manifestations resembling symptoms of SARS:

  • headache;
  • temperature rise;
  • weakness.

Psychotic stage of the disease

It manifests itself as psychopathological behavioral disorders, which prompts the relatives of patients to seek help from a psychiatrist. But even a doctor can suspect organic brain damage in this period of the disease. Typical symptoms are:

  • minimization emotional  manifestations (patients are lethargic, withdrawn into themselves, depression is often noted);
  • decline congruent skills - the ability to process information coming from outside: short-term memory, the ability to use the phone and other devices, etc .;
  • schizophrenic  symptoms: delirium, hallucinations (auditory and visual), compulsive behavior (repeated obsessive actions as a result of an irresistible desire), a decrease in the critical assessment of one’s condition;
  • disorders of memory  (amnesia) and speech are more rare;
  • violation sleep

The duration of this stage is about 2 weeks. Attacks of seizures indicate the progress of the disease.

Areactive stage of encephalitis

It manifests itself as a violation of consciousness, resembling catatonia (motor disorders in the form of a stupor or arousal). At the same time, patients in the stage of excitement automatically repeat other people's words and phrases, make sweeping movements, are distinguished by foolishness, laugh unreasonably.

It is often noted:

  • mutism  (the patient does not make contact, does not answer questions and does not fulfill requests and commands);
  • akinesia  (impossibility of active, arbitrary movements);
  • atetoid  movements (fanciful movements of limbs or fingers, often repeated flexion-extension, unnatural postures, etc.);
  • cataleptic  symptoms (decreased sensitivity to external stimuli);
  • paradoxical  phenomena (for example, there is no reaction to a pain stimulus).

Hyperkinetic stage

Manifested by the gradual development of hyperkinesis. Hyperkinesis can have different localization and speed, similar to psychogenic reactions.

It can be:

  • oro-lingual dyskinesias (prolonged chewing movements, licking the lips, impaired opening of the mouth, excessive compression of the teeth);
  • movements of limbs and fingers;
  • abduction or reduction of eyeballs, etc.

At this stage, signs of autonomic disturbances are noted in the form of increased or decreased heart rate, fluctuations in blood pressure, fever, and excessive sweating. Of particular danger are respiratory disorders, which sometimes require resuscitation. Hypoventilation and hemodynamic disorders develop in all patients.

Symptom regression stage - long stage

The reverse development of manifestations occurs within 2 months, but hyperkinesis can regress for more than 6 months, and be resistant to ongoing therapy. Along with the elimination of hyperkinesis, the psychosomatic status of the patient improves. All patients are characterized by persistent amnesia about the condition.

Diagnosis of anti NMDA receptor encephalitis

In the presence of clinical manifestations characteristic of encephalitis, laboratory and hardware diagnostic methods can be used to confirm the autoimmune nature of the disease.

Laboratory methods include:

  1. Study  cerebrospinal fluid (cerebrospinal fluid). Clinical analysis will give a moderately pronounced increase in the number of cells due to lymphocytes (up to 480 cells / ml), an increase in protein levels (within 49-213 mg / ml). Such changes are not specific, but they are observed in all patients with anti-NMDA receptor encephalitis.
  2. Serological  blood and cerebrospinal fluid tests. Allows you to get a specific test confirming the diagnosis - reveals antibodies to NMDA receptors. Moreover, the higher the titer of antibodies, the more severe neurological disorders. Antibody titers in cerebrospinal fluid are higher than in blood. In a dynamic study, titers decrease in patients with recovery, and in the absence of an effect in the treatment, the level of specific antibodies remains high in the blood and cerebrospinal fluid.
  3. Immunological  cerebrospinal fluid analysis. Shows an increase in class G immunoglobulins.
  4. Virological  studies of any biosubstrate give a negative result.

Hardware diagnostic methods:

  1. MRI  carried out in standard mode or with contrast (the introduction of gadolinium), according to experts, most often does not detect changes. When conducting a study in the FLAIR mode, some patients may have focal signal amplification in the temporal lobes, less often in the brain stem. Upon recovery, these changes disappear.
  2. Positron emission tomography  with fluorodeoxyglucose (SPECT and FDG-PET), according to experts, in some cases it can reveal an accumulation of contrast in the motor zones with the manifestation of dyskinesia (no accumulation is noted in recovery), hypoperfusion (insufficient blood supply) in the frontotemporal temporal regions of the cerebral cortex.
  3. EEG  (electroencephalography) in the reactive and hyperkinetic stages of encephalitis reveals diffuse (but with a predominance of lobewise) d- and q-activity.

In the clinical manifestations of anti-NMDA receptor encephalitis, the maximum possible examination must be carried out to identify the oncopathology of any organs and systems, including children. Oncological screening is an important component of the diagnostic algorithm for such encephalitis.

Treatment

Treatment of patients should be carried out in a resuscitation unit, taking into account the likelihood of developing hemodynamic disorders and respiratory disorders. Comprehensive treatment should include pathogenetic and symptomatic therapy.

As a pathogenetic therapy, antiviral drugs (Acyclovir) are treated until the results of the study exclude the viral nature of encephalitis.

It is very important to conduct an examination in terms of cancer monitoring as soon as possible: the sooner the radical treatment of cancer is carried out, the easier and faster it will be possible to cope with the treatment of encephalitis.

To suppress the synthesis of specific antibodies are used:

  • corticosteroid drugs (methylprednisolone);
  • immunoglobulins (for intravenous administration);
  • monoclonal antibodies (rituximab);
  • plasmapheresis (purification of blood from harmful substances by passing it through special filters);
  • cytostatics (Azathioprine, Cyclophosphamide) in rare cases.

As a symptomatic treatment, the following are used:

  • anticonvulsants (phenobarbital, clobazam, phenytoin, clonazepam, etc.) with the development of convulsive seizures;
  • with dyskinesias, antipsychotics are prescribed and, in addition, Midazolam, Propofol.

Disease outcomes

The average duration of inpatient treatment is 2.5 months. Of the residual neuropsychiatric manifestations after discharge, every fifth patient has sleep disturbances, 85% may have symptoms of frontal lobe dysfunction:

  • impulsiveness;
  • incontinence;
  • planning difficulties;
  • attenuation of attention, etc.

According to statistics, complete cure for adult patients occurs in 47% of cases. With mild but persistent residual changes, treatment ends in 28% of patients. In 18% of patients, more severe manifestations remain. In 7% of cases, a fatal outcome was noted.

Paraneoplastic encephalitis is associated with the formation in the body of antibodies to NMDA receptors of brain nerve cells. Psychopathological disorders often lead to hospitalization of patients in a psychiatric hospital.

The sooner paraneoplastic encephalitis is suspected and the correct diagnosis is made, an examination is carried out to identify oncopathology and its radical treatment in case of detection, the more effective the patient's treatment will be and the more chances of recovery.

69 - instability of the autonomic nervous system, 66 - hypoventilation. Antitumor therapy was associated with a greater number of remissions and fewer subsequent exacerbations. 75 patients recovered without consequences or with slight residual deviations; for 26, the result was severe impairment or death.

Often young women with this disease are admitted to psychiatric hospitals with a preliminary diagnosis of schizophrenia, catatonia, drug addiction or simulation, and only with the onset of neurological symptoms do they begin to suspect an organic disorder. In more rare cases, the disease makes its debut with severe short-term memory impairments resembling limbic encephalopathy. Increasingly, the disease is diagnosed in children and adolescents: a 2009 study said that out of 81 people, 32 (40%) were under the age of 18, and more often than not, tumors were found in younger patients and in male patients. In one small study published in 2009, of the 19 women with sudden onset of epilepsy of unclear etiology, 5 patients had antibodies to the NMDA receptor, and this, according to the authors, suggests that anti-NMDA receptor encephalitis can make up a noticeable proportion of unexplained cases of epilepsy with psychiatric symptoms.

Disease example

A brief description of a clinical case from an abstract of an article in the journal Nature Clinical Practice Neurology, 2007:

A 34-year-old woman went to the doctors for a headache, fever and anxiety. Soon, these symptoms were followed by ideas of harming, aggressive agitation, convulsions, hypoventilation, hyperthermia, and severe instability of the autonomous system, requiring intubation and sedation. There were episodes of hypotension and bradycardia with asystolic periods of up to 15 seconds. With the cancellation of sedatives - opening the eyes without reaction to external stimuli. Muscular rigor was noted, frequent facial grimaces, rhythmic contractions of the abdominal muscles, kicking leg movements, alternating dystonic poses of the right hand.

Story

Alternative names

The names used in the literature to describe the complex of symptoms before the discovery of communication with the NMDA receptor:

  • acute diffuse lymphocytic meningoencephalitis - Eng. acute diffuse lymphocytic meningoencephalitis
  • acute transient limbic encephalitis - English. acute reversible limbic encephalitis
  • acute early female nonherpetic encephalitis - English. acute juvenile female nonherpetic encephalitis
  • acute nonherpetic encephalitis of the young juvenile acute nonherpetic encephalitis

see also

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Notes

  1.   Iizuka T, Sakai F, Ide T, Monzen T, Yoshii S, Iigaya M, Suzuki K, Lynch DR, Suzuki N, Hata T, Dalmau J (February 2008). "". Neurology 70   (7): 504–11. DOI:. PMID 17898324.
  2.   Dalmau J, Tüzün E, Wu HY, Masjuan J, Rossi JE, Voloschin A, Baehring JM, Shimazaki H, Koide R, King D, Mason W, Sansing LH, Dichter MA, Rosenfeld MR, Lynch DR (January 2007). "". Ann. Neurol. 61   (1): 25–36. DOI:. PMID 17262855.
  3.   Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, Dessain SK, Rosenfeld MR, Balice-Gordon R, Lynch DR (December 2008). "". Lancet neurol 7   (12): 1091–8. DOI:. PMID 18851928.
  4.   Shimazaki H, Ando Y, Nakano I, Dalmau J (March 2007). "". J. Neurol. Neurosurg. Psychiatr. 78   (3): 324–5. DOI:. PMID 17308294.
  5.   Florance NR, Davis RL, Lam C, Szperka C, Zhou L, Ahmad S, Campen CJ, Moss H, Peter N, Gleichman AJ, Glaser CA, Lynch DR, Rosenfeld MR, Dalmau J (July 2009). "". Ann. Neurol. 66   (1): 11–8. DOI:. PMID 19670433.
  6.   Niehusmann P, Dalmau J, Rudlowski C, Vincent A, Elger CE, Rossi JE, Bien CG (April 2009). "". Arch. Neurol. 66   (4): 458–64. DOI:. PMID 19364930.
  7. "Glutamate (NMDAr) hypothesis of schizophrenia":
    •   - Bita Moghaddam, the portal "Forum for the study of schizophrenia";
    •   - translation blog neuroscience.ru
  8.   Nasky KM, Knittel DR, Manos GH (August 2008). "". CNS Spectr 13 (8): 699–703.
  9.   Sansing LH, Tüzün E, Ko MW, Baccon J, Lynch DR, Dalmau J (May 2007). "". Nat Clin Pract Neurol 3   (5): 291–6. DOI:. PMID 17479076.
  10.   Neurology Today 15 May 2008; Volume 8 (10); p 18-19

References

  •   - O. A. Levada, Zaporizhzhya Medical Academy of Postgraduate Education
  •   - “quick therapy reverses anti-NMDA receptor encephalitis”; Medscape portal, October 20, 2008, ed. Allison gandey
  •   - pharmed.uz, medical news
  •   - The educational article “The Case of Sudden Psychosis”, demonstrating the typical course of the disorder in a young woman. Current Psychiatry, 2009; author Anthony Cavalieri, MD, Cathy Southammakosane, MD, and Christopher White, MD, JD, FCLM
  •   “My Mysterious Lost Month of Madness,” an article by Suzanne Kalahan in the newspaper New York PostOctober 4, 2009.

Excerpt from Anti-NMDA Receptor Encephalitis

- Surely! This is the simplest thing you can do. You just don’t believe in yourself, therefore you don’t try ...
  - I don’t try it?! .. - I gasped from such terrible injustice ... - I just do what I try! Only maybe not that ...
  Suddenly I remembered how many times, many times, Stella repeated that I could do much more ... But I could - what?! .. I had no idea what they were all talking about, but now I felt that I was starting to calm down and think a little that in any difficult circumstances always helped me. Life suddenly seemed not so unjust at all, and little by little I began to come to life ...
  Inspired by the positive news, of course, all the following days I “tried” ... I completely spared myself, and tormenting my physical body, which was already exhausted, I went to the “floors” dozens of times before showing myself to Stella , because she wanted to make her a pleasant surprise, but did not hit her face in the dirt, making some stupid mistake.
  But, finally, she decided - stop hiding and decided to visit her little girlfriend.
  “Oh, it's you?! ..”, a familiar voice immediately sounded with happy bells. - Is it really you ?! But how did you come here? .. Have you come yourself?
  Questions, as always, rained from her in a hail, a cheerful face shone, and for me it was a genuine pleasure to see this bright, beating fountain, joy.
  - Well, let's go for a walk? - smiling, I asked.
  But Stella still could not calm down from the happiness that I was able to come myself, and that now we can already meet when we wish, and even without outside help!
  “You see, I told you that you can do more! ..” - the baby tweeted happily. - Well, now everything is fine, now we don’t need anyone! Oh, and it’s just very good that you came, I wanted to show you something and was waiting for you very much. But for this we have to take a walk to a place where it’s not very pleasant ...
  “Do you mean the lower floor?” - Realizing what she was talking about, I asked immediately.
  Stella nodded.
  “What did you lose there?”
  “Oh, I didn’t lose, I found! ..” the baby exclaimed triumphantly. “Remember, I told you that there are also good entities, but you didn’t believe me then?”
  Frankly, I didn’t really believe now, but, not wanting to offend my happy girlfriend, she nodded.
  “Well, now you will believe! ..” Stella said fairly. - Let's go?
This time, apparently having gained some experience, we easily “slipped” down the “floors”, and again I saw, very similar to the depressing picture seen earlier ...
  Some black, smelly liquid slurped underfoot, and streams of muddy, reddish water flowed from it ... The scarlet sky darkened, blazing with bloody glare, and, still hanging very low, drove off a crimson mass of heavy clouds. .. And those, not giving in, hung heavy, swollen, pregnant, threatening to give birth to a terrible, sweeping waterfall ... From time to time a wall of brown-red, opaque water broke out of them with a booming roar, hitting the ground so hard that it seemed - the sky is crumbling ...
  The trees stood bare and faceless, lazily moving drooping, spiky branches. Further beyond them stretched a bleak, burnt-out steppe, lost in the distance behind a wall of dirty, gray fog ... Many gloomy, drooping human beings roamed recklessly back and forth, meaninglessly searching for something, not paying any attention to the world around them, which, and True, it did not cause the slightest pleasure, so that he would like to look at him ... The whole landscape brought about horror and longing, seasoned with hopelessness ...
  “Oh, how scary it is here ...” Stella whispered, shivering. “No matter how many times I come here, I just can’t get used to it ... How can these poor things live here ?!”
  - Well, probably, these “poor things” were too guilty once, if they were here. After all, no one sent them here - they just got what they deserved, right? - still not giving up, I said.
  “Now look ...” Stella whispered mysteriously.
  Before us suddenly appeared a cave overgrown with grayish greenery. And from her, squinting, came a tall, stately man who in no way fit into this wretched, chilling landscape ...
  - Hello, Sad! - affectionately greeted the stranger Stella. - So I brought my friend! She does not believe that you can find good people here. And I wanted to show her to you ... Aren't you against it?
  “Hello dear ...” the man answered sadly, “I’m not so good as to show me to someone.” In vain are you this ...
  Oddly enough, but I really liked this sad person right away. Strength and warmth blew from him, and it was very pleasant to be with him. In any case, he did not at all look like those weak-willed, heartbroken, surrendered to the mercy of the fate of the people with whom this “floor” was jam-packed.
“Tell us your story, sad man ...” Stella asked, smiling brightly.
  “There’s nothing to tell there, and there’s nothing much to be proud of ...” the stranger shook his head. - And what do you need it for?
  For some reason I felt sorry for him ... Still not knowing anything about him, I was almost sure that this person could not do something really bad in any way. Well, I just couldn’t! .. Stela, smiling, watched my thoughts, which she apparently really liked ...
  “Well, okay, I agree - you're right! ..” - seeing her contented face, I finally honestly admitted.
  “But you still don’t know anything about him, and yet it’s not so simple with him,” Stella said quite slyly. - Well, please tell her, Sad ...
  The man smiled sadly at us, and said quietly:
  “I am here because I killed ... I killed many.” But not by desire, but by need it was ...
  I was terribly upset right away - I was killing! .. But I, stupid, believed! .. But for some reason I persistently did not have the slightest feeling of rejection or hostility. I clearly liked the man, and no matter how hard I tried, I couldn’t do anything about it ...
  “Is it the same guilt — killing at will or out of necessity?” I asked. “Sometimes people don't have a choice, do they?” For example: when they have to defend themselves or protect others. I always admired the heroes - warriors, knights. I generally always adored the latter ... Is it possible to compare ordinary killers with them?
  He looked at me for a long time and sadly, and then also answered quietly:
  - I don’t know, dear ... The fact that I am here says that the guilt is the same ... But by the way I feel this guilt in my heart, it’s not ... I never wanted to kill, I just he defended his land, I was a hero there ... But here it turned out that I was just killing ... Is that right? I think no...
  “So you were a warrior?” I asked hopefully. “But then, it's a big difference - you defended your home, your family, your children!” And you don’t look like a killer! ..
  - Well, we all are not like the ones that others see us ... Because they see only what they want to see ... or just what we want to show them ... And about the war - I, too, first just as you thought, even proud ... But here it turned out that there was nothing to be proud of. Murder - it is murder, and it does not matter how it happened.
  “But this is not right! ..” I was indignant. - What then turns out - the maniac killer turns out the same as the hero?! .. This simply cannot be, this should not be!
Everything was raging in me with indignation! And the man sadly looked at me with his sad, gray eyes, in which understanding was read ...
  “The hero and the killer take life in the same way.” Only, probably, there are “mitigating circumstances”, since a person who protects someone, even if it takes life, is for a bright and righteous reason. But, one way or another, they both have to pay for it ... And pay very bitterly, you really believe me ...

Encephalitis is a whole group of diseases manifested by the inflammatory process of the brain. The disease is characterized by severe symptoms and can be caused by a number of factors, for example, the autoimmune process that causes Anti-NMDA receptor encephalitis. Inflammation of the brain requires qualified and timely treatment, otherwise the risk of death is high.

Encephalitis is an extensive group of diseases that manifest as an inflammatory process in the brain. They cause various pathological changes in the body and lead to the development of dementia (dementia). The disease can affect not only the brain, but also internal organs and joints.

Pathology can be caused by a number of factors. For the reason that provokes the development of the disease, the following types of pathology are distinguished:

  • infectious inflammation;
  • encephalitis of a bacterial or fungal breed;
  • illness caused by toxic effects on the body;
  • encephalitis of an autoimmune nature.

The disease affects different parts of the brain. The inflammatory process can be localized in the cerebral cortex, subcortical nuclei or cerebellum.

Each type is characterized by its own signs and symptoms, as well as treatment methods.

Infections of an infectious and bacterial nature

The causative agents of infectious encephalitis are the following viruses and bacteria:

  • herpes;
  • HIV infection;
  • encephalitis viruses;
  • tuberculosis bacteria;
  • streptococci and staphylococci;
  • toxoplasma.

Tick-borne encephalitis is a serious problem in some regions. This disease is of a viral nature, the virus is transmitted by some kind of ticks. The virus enters the human blood only by an insect bite, but it is not transmitted by airborne droplets. However, tick-borne encephalitis does not always affect the brain. In about half the cases, symptoms of fever are observed, and there are no symptoms of inflammation of the cerebral cortex.

Another type of viral encephalitis is Japanese. The disease is extremely dangerous and results in death in seven out of ten cases. The disease is characterized by a rapid course, as a result, a coma develops a few days after infection.

Encephalitis caused by the herpes virus causes death in nine out of ten cases. This is a very dangerous disease that is difficult to treat.

A striking example of bacterial encephalitis is a disease provoked by the action of meningococci. Pathology is characterized by the development of meningitis and the further spread of inflammation in the cerebral cortex.

Autoimmune diseases

There is a group of encephalitis caused by autoimmune processes in the body, in which a person’s own immunity begins to attack brain cells.

It is extremely difficult to diagnose and treat. As a rule, the disease causes rapid dementia and leads to impaired brain function and peripheral nervous system activity. In addition to dementia, the disease accompanies paralysis and seizures similar to epileptic.

Such diseases include limbic encephalitis. The disease causes an autoimmune response of the body to cancer cells, or to any infectious or viral disease. According to the rate of development, limbic encephalitis is divided into acute and subacute.

The acute syndrome develops rapidly, within three to five days, while the first symptoms in the subacute course become noticeable a few weeks after the onset of the development of the pathology.

Typical symptoms of pathology:

  • memory impairment;
  • cognitive impairment;
  • epileptic seizures;
  • mental disorders: depression, anxiety, panic attacks;
  • behavioral disorders.

The disease is characterized by progressive dementia. Patients often suffer from sleep disturbances, and epileptic seizures may be accompanied by hallucinations.

Very often, autoimmune brain damage is associated with the presence of cancer.  In the vast majority of cases, such encephalitis is caused by lung cancer.

Anti NMDA Receptor Encephalitis

Anti-NMDA receptor inflammatory process or encephalitis is an autoimmune disease that young women are more susceptible to. In men, pathology is extremely rare.

Pathological Features Anti-NMDA receptor process or encephalitis are severe symptoms. The disease causes a number of psychoneurotic changes, so it is often confused with schizophrenia.

Women suffering from this disease showed psychiatric abnormalities, such as a growl, lack of coherent speech, impaired consciousness. Another characteristic symptom that allows you to diagnose the Anti-NMDA receptor process or encephalitis is short-term memory impairment, characteristic of the limbic form of the disease.

Another feature of the pathology is a violation of muscle function. So, patients for no reason begin to contract their abdominal muscles, kick surrounding objects or try to harm others like in another way.

As a rule, pathology is associated with cancer of the ovaries. In about half of the cases, this cancer was discovered in patients.

It should be noted that they did not know about the disease until recently, what doctors did not find out more than ten years ago.

Diagnosis of autoimmune brain inflammation

An experienced doctor will suspect encephalitis when examining a patient. However, additional tests are needed to determine the nature of the disease.

Often prescribed magnetic resonance imaging. This method allows you to confirm suspected inflammation of the brain, however, to identify the cause of encephalitis will not help.

To determine the cause, a biochemical blood test is needed. In autoimmune diseases, such as the Anti-NMDA receptor process or encephalitis, it is necessary to analyze the presence of antibodies to the nmda receptor.

In some cases, a brain biopsy is also indicated. A biopsy is prescribed only as a last resort, when it is impossible to identify the cause of the pathology by other methods. A consultation with an oncologist and therapist is required.

Possible complications

Autoimmune inflammation of the brain is difficult to diagnose. As a result, many patients end up in a psychiatric hospital due to an incorrect diagnosis.

Without proper treatment, the disease leads to the development of psychiatric disorders and dementia, which are often irreversible. There is also a high risk of developing coma, from which the patient may not exit.
  Without treatment, a vegetative state quickly develops, and in a third of cases - a fatal outcome.

Treatment

For the diagnosis, consultation and examination by a neurologist is necessary. Diagnosis is based on the presence of specific antibodies.

The peculiarity of such brain lesions is that they are often mistaken for schizophrenia and appropriate treatment is prescribed in a psychiatric clinic. However, if an autoimmune encephalitis is suspected, an oncologist consultation is also necessary. In most cases, cancer treatment allows for long-term remission.

A good stable result is achieved by treatment with immunomodulators. However, such treatment is effective only if the suspicion of cancer has not been confirmed.

To reduce the symptoms of mental disorder, patients are shown sedatives. They help normalize sleep and reduce symptoms. If seizures are observed, it is necessary to take antispasmodic drugs.

Corticosteroids are prescribed to relieve acute inflammation. They are administered intramuscularly, and the duration of the course of treatment is selected by the doctor.

Autoimmune encephalitis in most cases cannot be completely cured. Therapy helps stop the further progression of the disease and avoid the development of irreversible neurological disorders. However, if the disease is caused by cancer, the removal of the tumor gives a stable result and in 70% of cases a full recovery occurs.

Prevention

Prevention of viral and bacterial encephalitis consists in careful outdoor behavior during the encephalitis tick migration. Wear closed clothing and use special spray repellents.

Pathology can develop against the background of a serious viral disease, so it is important not to let the disease go by its own accord and contact the clinic in time for qualified help.

Autoimmune brain diseases cannot be prevented.

In Western countries, there is an outbreak of neurological diseases, including early dementia. It turns out that some brain diseases are accompanied by such unusual symptoms that they are sometimes mistaken for mental disorders.

10. Anti-NMDA receptor encephalitis

In many patients, anti-NMDA receptor encephalitis (a newly discovered autoimmune disease that causes cerebral edema) exhibits psychiatric symptoms such as hallucinations, outbreaks of aggression and delirium. Patients seem to be possessed by demons, and within a few days most of them develop various types of seizures, as well as involuntary and uncontrollable human movements.

But neurological symptoms can be hidden, they are easy to miss. Dr. Sukhel Najjar, a specialist in the disease, estimates that 90% of these cases are misdiagnosed. “Perhaps people who are now in a coma, or people stuck in mental hospitals, suffer from this disease and are not receiving proper treatment,” said Emily Gavigan, a patient with anti-NMDA receptor encephalitis.

24-year-old Suzanne Kahalan spent more than $ 1 million on treatment at the hospital with leading doctors, but they repeatedly gave her erroneous diagnoses. The patient had attacks with hallucinations, during which she growled like an animal, pushed and beat people. It constantly seemed to the girl that the announcers on TV were constantly discussing her.

Relief came when Dr. Najar took care of her. He asked her to draw a watch dial, and when she drew the numbers on one side only, the doctor realized that Susanne had inflammation of the right hemisphere of the brain. Timely treatment started saved her from coma and death.

Although Suzanne managed to fully recover, not everyone is so lucky. Even when treatment arrives on time, about 7% of patients die, others suffer brain damage of varying degrees. Anti-NMDA receptor encephalitis can be treated with immunotherapy, but without the possibility of healing, only remission. Relapse requires even more care.

All this prompted Dr. Najar to start researching other mental illnesses - bipolar disorder, depression, obsessive-compulsive syndrome and schizophrenia - to check if they are also physical illnesses caused by brain inflammation.

9. Othello syndrome

Othello Syndrome (SB) got its name from the Shakespearean hero Othello, who killed his wife Desdemona due to suspicion of treason. Patients with CO demonstrate enviable obstinacy when it comes to delusional suspiciousness and jealousy of their spouses, constantly accusing them of infidelity. Some even have hallucinations about their partner having sex with someone else.

Typically, CO occurs at the age of about 68 and is observed in almost 77% of patients with a neurological disease that affects one of the frontal lobes of the brain - most often the right one. Sometimes CO appears as a result of treatment with Parkinson's dopamine. In this case, reducing the dose or stopping the medication can alleviate the symptoms of Othello syndrome.

In dementia with Levy bodies (DTL), symptoms of CO can continue to manifest (or even just start) after the death of the spouse. DTL is a syndrome of parkinsonism and dementia caused by protein deposits called Levy bodies in nerve cells in the brain.

One 42-year-old man who took dopamine for Parkinson's disease began to insist on frequent sex with his wife. He accused her of treason and obsessedly watched the driveway to the house, as he was convinced that a fictional lover was going to call for her to have sex together. The patient lost thousands of dollars due to sudden gambling impulses and lost the ability to control his expenses.

Like Othello, patients with this syndrome can become dangerously violent. Men with CO tried to strangle their wives or engage in fights with neighbors suspected of being their lovers.

8. "PH". Sensory desynchronization

The 60-year-old retired pilot was the first patient with a sensory disorder in which a person hears voices before people speak. Life for PH is like watching a movie with sound ahead of the picture. He even hears his own voice before he feels the movement of his lips. Tomography of the pilot’s brain showed two injuries: one in the midbrain and the other in its trunk. Both of these departments are responsible for hearing, movement and synchronization.

Scientists believe that our brain processes visual and auditory information at different speeds to compensate for the difference in the speeds of light and sound. The brain of a healthy person synchronizes the sound of the voice with the movement of the lips. But in patients with PH, a delay of a quarter second (210 milliseconds) between them is noticeable.

No one knows how the human brain integrates our visual and sound perceptions. But this means that each of us is equipped with more than one pair of watches in our heads. If this watch does not work smoothly, the soundtrack to our life may not correspond to our visual reality.

7. Ecstatic epileptic seizures

An ecstatic epileptic seizure, or an ecstatic aura, was described by the famous novelist, who suffered from epilepsy, Fedor Dostoevsky: “For a few moments I experience such happiness that is simply impossible in the ordinary state, and which other people have no idea about. I feel complete harmony in myself and throughout the world, this feeling is so strong and sweet that in a few seconds of such bliss you can give ten years of life, and perhaps all my life. ”

Here is what a 53-year-old female teacher told about her ecstatic seizure: “The feeling was as if not from this world. There was a feeling of complete serenity, absolute peace, no worries; it was beautiful, everything seemed wonderful ... Probably the closest sensation I experienced was an orgasm. But there was nothing sexual about that feeling ... it was almost religious. ” She also said that she is no longer afraid of death and sees the world brighter than before.

Some scientists believe that ecstatic seizures explain the feelings of patients who survived clinical death. No one knows for sure what happens to a person at such moments, but researchers have estimated that only 1-2% of patients with temporal lobe epilepsy have an ecstatic aura. All these patients report a feeling of euphoria and a deepening of self-awareness. Some also speak of a sense of stopping time, serenity and bliss, but sometimes they also report congestion due to the intensity of what is happening.

Often these attacks begin in one of the temporal lobes of the brain. But some neurologists believe that in fact, activity occurs in the islet lobe. Unlike the temporal lobes, under which the islet is located, its anterior lobe is responsible for our feelings - both good and bad.

6. Misophonia

Those suffering from misophony become furious when they hear sounds that most of us ignore or simply do not notice: chewing gum, sipping soup, soft steps. Their hearts begin to pop out of their chest, their fists clench, and their bodies seem ready to explode from uncontrollable anger or anxiety. Unlike patients with hyperacusis - who perceive all sounds as unbearably loud - patients with misophony calmly respond to noise. They can not stand only quiet sounds.

Misophonia usually occurs in late childhood or early adolescence. Over time, the condition worsens, and more and more sounds cause a trigger effect in patients - even breathing can knock some out of the rut. But patients can not do anything about it. As a patient named Ada Tsyganova said: “It's all about the reaction. Fury. Anger. Inability to stop it. For people with this disorder, one sound is similar to the way 200 people simultaneously fingernail a blackboard. It is very overwhelming and annoying. "

Many of these patients were mistakenly diagnosed with mental disorders, including post-traumatic syndrome. But some doctors are beginning to recognize misophony as a neurological disease that can be caused by damage to the brain “wiring” in the area responsible for emotions. But many doctors still do not believe in the existence of misophony - to date, treatment options are limited and largely ineffective. Most patients are forced to cope with the disease as much as possible by eating alone or relieving stress by screaming. Some use ear plugs to block sounds.

5. Progressive topographic disorientation

Imagine that you get lost and it repeats every day, even in your own home, and perhaps you will understand how the 60-year-old Sharon Roseman from Littleton, Colorado feels. Since she was 5 years old, Sharon has been struggling with progressive topographic disorientation (PTD). This is a rare neurological disease in which a person loses the ability to navigate in space. As a child, Sharon did not recognize her home, then her mother warned her: "Do not tell anyone, they will say that you are a witch and burn you at the stake."

Sharon obeyed, not even telling her husband about her condition. It got to the point that she could hardly find her children when they woke up and cried at night. When she drives a car, curved streets and crossroads confuse her and cause her confusion, as do the oceans, lakes and pools. Sharon herself describes her feelings in this way: "It is almost as if someone had lifted the whole world, swept it and put it in place."

At the age of 29, Sharon first sought medical help and visited a psychologist. But he could not save her from disorientation. The woman was later told that she might have a brain tumor or epilepsy. This also turned out to be a mistake. She then met Giuseppe Airia, a professor of neurobiology at the University of Calgary. In 2008, Dr. Airia published his first article on PDD, so he knew exactly what Sharon was dealing with.

Doctors are not sure what exactly happens to the patient’s brain. Scanning does not reveal any atrophied or depleted areas. But Jeffrey Taub, a professor at Dartmouth College, believes that different areas of the disoriented brain do not interact with each other properly, without coordinating information transformation processes.

The cure for PTD has not yet been invented. But Sharon is simply relieved, because he can finally tell others about his condition, not being afraid to be called crazy.

4. Musical hallucinations

One day, a woman known as Sylvia heard the sounds of playing the piano behind a house. But there was no instrument, in fact, Sylvia was experiencing musical hallucinations. They sound real enough to convince the patient that live music is playing in the next room or the choir is singing. Over time, the hallucinations of Sylvia became almost constant, playing long excerpts from the works of classical composers, such as Rachmaninov.

Mental disorders such as depression, obsessive-compulsive syndrome, and schizophrenia can cause musical hallucinations. But in most cases, the point is not psychosis. But simply in hearing impairment in an elderly person whose brain provides incorrect information about what he hears. At least this is the theory of the doctors who examined Sylvia and other patients with similar symptoms.

In the end, Sylvia found that listening to real music halts hallucinations for a short time. By scanning her brain in the normal state and while playing live music, doctors were able to determine which areas showed increased activity in hallucinations. Based on these studies, today, experts believe that it is enough for our brain to “hear” only one external note or chord, and then it begins to predict subsequent ones based on existing experience. If the predicted information is incorrect, the next external sound will provoke our brain to make a completely new prediction in order to minimize errors.

In case of hearing loss, the patient’s brain receives less sound information and makes more prediction errors. With an increase in their number, "fictional" sounds begin to seem very real to the patient. Doctors believe that a person hallucinates music most likely because it has a certain organization and is thus more easily predicted by the brain than random noise.

3. Huntington's disease

Caused by a mutation in the IT-15 gene, Huntington’s disease (BH) is a rare genetic disease that destroys brain nerve cells over time, affecting the patient’s behavior and motility. American musician Woody Guthrie died of Huntington’s chorea after years of improper treatment. So far, the disease remains incurable.

Some people, such as Katherine Moser, are still being tested for a genetic defect that causes BH, which usually does not appear before middle age. Unfortunately, many patients with Huntington's syndrome are afraid to admit that they have a disease, because they do not want to face the truth and fear discrimination at work and in life. As Katherine's mother said: “People are deprived of compassion. They look at you as if you were not from this world.”

Young Miss Moser observed the effects of the destructive effects of the disease on her grandfather's body. The man had involuntary twitching of limbs and outbursts of anger. Once he entered the kitchen completely without clothes, with the exception of underwear on his head.
In the early stages, symptoms of HD are not always the same. But, as a rule, the sooner they appear, the faster the disease progresses. Mood swings are one of the earliest symptoms. Huntington syndrome sufferers may become depressed, irritable, lethargic, or angry. BH can also affect human memory, judgment and learning. Over time, the patient’s intelligence decreases more and more.

For other patients, the first signs of the disease are uncontrolled movements of the muscles of the face, legs, fingers or torso. Slowness and balance problems may also appear. Over time, difficulties arise when performing such basic body functions as nutrition, communication and movement. Today, Huntington's disease will inevitably lead to death.

2. Frontotemporal dementia

While Alzheimer's disease begins with memory loss, which later gives way to impaired behavior, with frontotemporal dementia (LHD), everything happens exactly the opposite. First, having dealt with the nerve cells of the frontal lobes, LVD is manifested by behavioral disorders. Then, with the spread of the disease throughout the brain, the patient's memory also fades. Over time, senile dementia of the Alzheimer's type and LVD begin to proceed in almost the same scenario.

HDL often catches up with its victims between the ages of 45-65, earlier than Alzheimer's usually does. The behavioral variant of LHD is manifested by early symptoms, most often imitating mental disorders. In physical terms, LVD leads to atrophy of the frontal and temporal lobes of the brain. But, like all forms of dementia, it deprives its carriers of life, love and dignity. As a relative of one patient said: "Performing the role of a nurse in this disease is mourning, while a person is still alive."

Barbara Whitmarsh, a former fellow at the US National Institute of Health, has been married for three decades and raised six children with her husband John. In the end, the husband nevertheless noticed serious changes in his wife, provoked by the Ministry of Internal Affairs. He said: "Her ability to empathize, her personality, over time, she simply disappeared." The woman also gained 15 kilograms in one year.

Patients with a behavioral variant of LVD may develop an increased appetite for sweets. They are also prone to outbursts of rage, loss of inhibitions, and weak value judgments. They can also become hyperactive, hypersexual, and impulsive. But the most difficult thing for families of patients is, perhaps, to put up with their loss of feelings for others. To make matters worse, people with HFD usually do not notice changes in their behavior.

Barbara Whitmarsh no longer recognizes members of her family and speaks only occasionally. She was placed in a closed-type nursing home, where she never stops moving.

1. Macleod's Syndrome

Caused by a hereditary mutation in the HC gene, Macleod's syndrome is a neurological disease, usually manifested in middle age. To date, only about 150 patients with this anomaly have been registered in the world. Half of them complain of cramps, while other symptoms include muscle weakness and atrophy, involuntary twitching of legs and arms, grimacing and vocalizations like grunts, and mental degradation.

Strange changes in behavior can make doctors make a mistake by accepting the Macleod phenotype as a mental illness. Some of the early symptoms are depression, anxiety, and deep emotional instability - including a lack of restraint. This disease is incurable, but medical care can bring some relief.

According to researchers from Southern Methodist University, Macleod’s syndrome could be the true reason why the English king Henry VIII beheaded two of his six wives. Initially, the monarch was strong, athletic and generous. At the age of about 40, he began to feel weakness and atrophy of the leg muscles, which ultimately caused a decrease in mobility. He also plunged into psychotic paranoia, the result of which was the execution of his wives.

Macleod's syndrome is associated with the Kell blood group system, which can also explain pregnancy problems in the spouses and lovers of Henry VIII. They carried at least 11 of his children, but only four of them survived. If Henry VIII had a positive kell antigen and his women had a negative one, then they could give birth to a healthy baby only during their first pregnancy, faced with the threat of miscarriage in the following attempts.

+ Alien syndrome

The corpus callosum is a bundle of nerve fibers that connects the right and left hemispheres of the brain, it is it that allows them to interact. Sometimes the surgeon has to cut this connecting link to help the patient with epilepsy stop seizures. Most patients recover safely after this operation, but some remain with two halves of the brain, functioning independently of each other. It is possible, in the end, this will result in a real war, as if an alien took control of one side of the body. Such a disorder, quite appropriately, is called the syndrome of an alien hand (HRS).

The Nobel laureate, scientist Roger Sperry, filmed as a patient with SCR trying to arrange the cubes in accordance with the sample in the picture. With the left hand - controlled by the right hemisphere of the brain - the patient did an excellent job, but he could not do it with his right hand. Moreover, when the left hand tried to help the right, they began to fight with each other, like a couple of quarreled children.

Karen Byrne got rid of epilepsy by dissecting the corpus callosum. But one fine day, her doctor witnessed a woman, not noticing herself, unbuttoning her shirt with her left hand. After she buttoned all the buttons with her right hand, her left again tried to undress her.

Sometimes a “strange hand” strikes or slaps the patient. And if his legs decide to go in different directions, the person begins to walk in circles, as the two halves of the brain are included in the struggle for power over the body. Fortunately, Karen’s doctors finally managed to find a way to control these symptoms with medication.

Material prepared by Leanna - according to an article from the site

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