S.A. Klyushnikov
Candidate of Medical Sciences
State Research Center of Neurology RAMS
The first mention of an unusual disease, now called Huntington's disease (HD), is found in Western European historical documents XVI-XVIII centuries. Many paid attention to the most striking external manifestation of the disease - involuntary movements of the arms, legs, torso of patients, often resembling a kind of dance. Neurologists call this type of violent (that is, not amenable to voluntary control) movements chorea, from which the common synonymous name for HD, Huntington's chorea, came from. From the deep Middle Ages, another name for the disease has come down to our days - "St. Vitus' dance"; this unusual term is known to many people who are not related to the history of medicine and neurology. Saint Vitus was a historical character and lived in Sicily at the beginning of the decline of the Roman Empire. This young Christian was martyred by the Romans in 303 during the persecution of Christians launched by the emperor Diocletian. After 1200 years (since the 16th century), his name became associated with "dance". Then, for unknown reasons, a belief spread throughout Germany that anyone who dances in front of the statue of St. Vitus on his day (June 15) will receive a charge of vivacity for the whole year. Thousands of people crowded around the statues of the saint on this day, and their dances were often very expansive, emotional. In the end, they began to call the chorea "the dance of St. Vitus" and even tried to resort to the help of this saint in order to heal.
The modern scientific stage in the study of BG dates back to 1872, when at a meeting of the Medical scientific society State of Ohio (USA) George Huntington (George Huntington) presented a brilliant clinical detailed description of the disease, based on the analysis of many of his own observations. It is no coincidence that hereditary chorea was named after this American physician. The following decades were a period of accumulation of clinical facts and the results of the analysis of pedigrees, attempts to systematize them. In 1983, HD became the first hereditary neurological disease in which the exact localization of a pathological gene on a particular chromosome was established. After 10 years, scientists were able to decipher the exact structure of the pathological gene of the disease. At the same time, DNA diagnostic methods were developed that made it possible to establish the carriage of the pathological HD gene long before the onset of symptoms of the disease.
HD is one of the most common hereditary diseases in terms of prevalence. nervous system- an average of 5-7 cases per 100,000 population. HD is a hereditary progressive brain disease that usually begins in middle age (about 40 years), the main external clinical manifestation which are involuntary movements of the arms, legs, torso, often facial muscles of the face, called choreic hyperkinesis. At the beginning of the disease, they are barely noticeable, "random" in nature. Often on early stages diseases of obvious hyperkinesis are not visible, but some motor anxiety of a person is captured, he "does not sit still." In the future, as the disease develops, choreic hyperkinesis intensifies, affecting more and more muscle groups. Constant muscle activity takes a lot of strength from patients, which is often manifested by a characteristic complaint of patients about general weakness. With a long-term course of the disease, the intensity of hyperkinesis often decreases, and excessive motor activity is replaced by general lethargy, reminiscent of that in Parkinson's disease. Characteristic manifestations of the disease are also violations of memory, thinking, intellectual activity, that is, cognitive impairments (or disorders of the cognitive function of the brain). First of all, short-term memory suffers, concentration of attention is disturbed, a person becomes distracted. At the same time, the analytical function of the brain is lost, the ability to abstract, generalize and logical conclusions, thinking becomes primitive, there is a loss of habitual interests. Personal characteristics of a person suffer, character changes, emotional-volitional and often mental disorders appear. The course of HD is characterized by a slow but steady progression over 15-20 years. The development of HD inevitably leads to disability and the need for external household assistance.
HD is inherited in an autosomal dominant manner, meaning inheritance from one of the affected parents (regardless of their gender) with a probability of 50% for each child. Persons of both sexes are also affected. An important characteristic The autosomal dominant mechanism of inheritance of the HD gene is the so-called vertical transmission path, with the presence of cases in each generation, without gaps. If in some generation all relatives are genetically healthy, then further inheritance of the disease in the genus stops - BG does not "jump" through the generation. In each subsequent generation, an earlier onset of the disease and its more severe course are observed. This phenomenon is called "anticipation". Close to this phenomenon is the so-called "paternal transmission effect", which consists in the fact that the disease with more early start and more severe course develops predominantly through paternal transmission.
Many subtle molecular mechanisms of HD development have been elucidated over the past decade. A specific dynamic mutation in the gene (an increase in the number of copies of trinucleotide repeats) leads to disruption of the normal spatial folding of the corresponding protein product and the acquisition of toxic properties by this mutant protein. The abnormal protein accumulates in certain brain cells, causing them to die. Unfortunately, at present there are no means of directly influencing the mechanisms of the development of the disease. The modern drugs used (such as haloperidol, tiapride, semax, memantine, etc.) are aimed at alleviating the condition of patients, reducing violent movements, and alleviating mental and intellectual disorders. However complex treatment BG allows in some cases to slow down the development of the disease and improve the quality of life of patients.
DNA diagnostics (gene diagnostics) of HD, like any other hereditary disease, is the basis of the system of medical genetic counseling - a special type of specialized medical care aimed at preventing the occurrence of repeated cases of hereditary diseases in burdened families. Medical genetic counseling allows you to establish an accurate diagnosis, calculate the genetic risk of consulted relatives, including the accurate determination of their genetic status using prognostic testing, determine the prognosis for offspring, including
including using prenatal DNA diagnostics of the fetus on early dates pregnancy, as well as to help the consulted family in solving a number of other issues related to life planning, reproductive behavior and the possibility of childbearing, psychological support, social adaptation.
In 1995 on the basis of the neurogenetic department of the Institute of Neurology of the Russian Academy of Medical Sciences (now - Science Center neurology of the Russian Academy of Medical Sciences), the Russian Association for the Fight against GD was created. It is integrated into the structure of the World Association for the Fight against HD. The Russian Association was founded by neurogeneticists and works in close connection with the neurogenetic clinic and the DNA laboratory of the Scientific Center for Neurology of the Russian Academy of Medical Sciences. Based on the experience gained, the Association has worked out its own ethical and organizational principles for conducting medical genetic counseling in burdened families. Patients suffering from HD and being in the field of view of the Association have the opportunity to periodically undergo inpatient treatment at the neurogenetic clinic of the Scientific Center for Neurology of the Russian Academy of Medical Sciences and receive outpatient counseling assistance. The Huntington's Disease Association of Russia also considers its goal to be comprehensive information and educational support for burdened families, for which special booklets are issued, seminars are held for family members with HD and other events.
According to the modern medical reference book, Chorea (from the Greek choreia - dance) is a disease of the nervous system, characterized by violent movements, which are characterized by disorder, fast pace, sweeping, irregularity, lack of stereotype, the possibility of simultaneous contraction of muscles of different location and function. It seems to be nothing ordinary, you never know there are human ailments. However, an interesting fact is that many centuries ago, such a disease massively affected entire villages. Considering that chorea is not a contagious disease, let's figure out where is the truth and where is fiction.
So in 1374, the inhabitants of dozens of villages along the Rhine River suddenly felt the symptoms of a deadly disease - the dance plague - choreomania (or the dances of St. Witt). On these streets settlements hundreds of people could be seen jumping and kneeling for no reason. And they did it without musical accompaniment, although, perhaps, the music sounded in their inflamed minds. And they continued their dances until, exhausted from hunger, insomnia and fatigue, they fell to the ground. The heels of the unfortunate patients were rubbed into the blood. And then this strange plague suddenly ended - almost the same as it began.
In 1518 there was another outbreak. A woman named Frau Troffea, for unknown reasons, suddenly went out into the streets of the city of Strasbourg and began to dance. She could not complete her dance for several days. In a week, the number of dancing patients increased to 35 people, and by the end of the month, 400 people were already moving in a strange dance. Dozens of people have had heart attacks, strokes or exhaustion. This caused them to drop dead. And in this case, the dancing plague stopped just as suddenly.
Many scientists have tried to explain this "dancing" riddle. For some time, the following hypothesis was popular: people were poisoned with bread affected by a fungus growing on wet rye stalks - ergot. Once in the body, it causes fever, convulsions, delirium.
John Waller, professor of history at the University of Michigan, believes that this version is implausible, because the speech movements of the patients were precisely dances, and not convulsions. Another popular theory - the involvement of the victims in a certain dance cult - the professor also rejected.
Waller proposed a third theory: these were psychogenic (caused by mental trauma) mass diseases that caused fear and depression. Both outbreaks occurred during times of famine, crop failure, and flooding—what could be taken as signs of a biblical catastrophe. Fear of the supernatural could provoke a state of a kind of trance in people.
In addition, the dancing plague is associated with St. Vitus, a Christian martyr. According to legend, one could gain health by dancing in front of the statue of St. Vitus on his name day. For some, these dances could be the last chance to recover from a deadly disease, such as the plague, smallpox or cholera that raged in those days. So, the idea of saving dances was already in the subconscious of people. And to start this marathon during the epidemic, one person was enough.
The Strasbourg outbreak of the dancing plague was not the last; something similar could be observed in Madagascar in 1840.
The Middle Ages "gave" Europe a huge number of dangerous and harmful superstitions. Moreover, their creators and distributors were the most authoritative categories: doctors and Catholic priests, since they received at least some education. This knowledge was quite enough for self-confident "specialists" to impose their opinion on an even more ignorant population.
Among the monasteries there was a struggle for the flock: after all, it was a source of income, so all kinds of miraculous objects, parts of the bodies of saints, stories about incredible cures were born. Priests argued that it is preferable for saints when pilgrims move by some in a strange way e.g. by jumping backwards. In this case, the believer was more likely to get what he wanted.
Historical examples
The religious rite dedicated to St. Vitus originated in the 14th century in Germany. To get new opportunities, strength, health, it was required to dance in front of the statue of this character on his day (June 15). Soon this method spread throughout Europe and became the most popular version of communication with higher powers.
It is not surprising that religious superstitions were especially pronounced in Europe in the 14th century. In the middle of the century, several plague epidemics swept through. Due to heavy rains, there were several lean years, and the Rhine overflowed its banks and flooded cities, villages and arable land.
The German historian of the 19th century, Justus Hecker, reports a number of recorded cases of mass psychosis, when people, in the process of their convulsive dances, lost all adequacy and resembled epileptics.
One of the first cases of mass dancing took place near convent Kolbig.
In Utrecht, about two hundred dancers gathered on a bridge over the Rhine, as a result, the bridge collapsed and all the dancers drowned.
In Erfurt, the mania of crazy dancing seized more than a hundred children.
In 1374, believers gathered for a prayer service to John the Baptist, but religious celebrations unexpectedly turned into crazy historical dances.
More than 500 people danced in Cologne. Groups of people who were in the grip of delusions and hallucinations united in round dances, which moved along the streets, accompanying their path with outlandish body movements. They rolled on the ground, foaming at the mouth, tearing out their hair and uttering wild screams.
The streets of the city of Metz were filled with a mass of jumping and screaming people: at the same time, about 1,500 people jumped through the streets in a complete frenzy. Many were visited by hallucinations, which were taken for religious insight.
In Strasbourg in 1518, “hundreds of men and women danced and jumped in the marketplace, in the lanes and in the streets. Women took shameless poses, tumbled and stood on their heads. Many did not eat anything for several days until the disease subsided.
The Hungarian writer and historian Istvan Rath-Weg quotes an entry in the Great Belgian Chronicle from 1374, which reads: “This year, crowds of outlandish people arrived in Aachen and moved from there to France. Beings of both sexes, inspired by the devil, danced hand in hand in the streets, in houses, in churches, jumping and screaming without any shame. Exhausted from dancing, they complained of pain in their chests and, wiping themselves with handkerchiefs, lamented that it was better to die. Finally, in Lüttich, they managed to get rid of the infection thanks to prayers and blessings.
It is clarified that these obsessed people were driven into a frenzy by the red color and attracted to water, so they often threw themselves into the water and drowned. Many could not stand the heart and they fell dead. After the passage of such processions, the lifeless bodies of people who died from heart attacks or from exhaustion remained on the ground.
In 1418, the dancers were declared insane and possessed by a demon, they were caught and locked up in a church under lock and key.
Crazy dances: how they attracted people
A person's pleasure is associated with the production of certain hormones in the body. Can be enjoyed different ways: through sex, Tasty food, shopping, travel, beautiful music, achievements in sports or science. There are people who take pleasure in anti-social actions: offending the weak, stealing something. In any case, the corresponding hormone, dopamine, enters the brain; the reflex is fixed, and the person again seeks to perform the same actions for the sake of feeling joy and satisfaction. Hormones of pleasure are necessary for a person to maintain health: if life is boring and bleak, they develop various diseases such as Parkinson's disease.
There are also workarounds for getting the same sensations: through drugs or alcohol. However, such artificial way leads to disruption of metabolic processes in the structure of the brain and as a result, the same diseases arise as with a lack of positive energy.
St. Witt's dance - chaotic physical activity, accompanied by unloading of the brain, also stimulated the production of the hormone of pleasure. From this point of view, the statement that the rite gives additional strength was correct. Another thing is that it was the same artificial way as drugs, and it had the same destructive effect on the brain. Psychosis and hysterical trance developed.
Poisoning as the cause of the dance of St. Vitus
Before the introduction of potatoes from America, the basis of the diet of Europeans was Rye bread. In rainy years, low temperatures and high humidity, a fungus called "ergot" developed in the grains. It was difficult to recognize it in dark grains of rye, so it got into food and caused severe poisoning: ergotism. There were two types of symptoms:
1. As a result of taking low doses, patients developed an aggressive type of behavior, mental disorders, severe pain and blockage of capillary blood vessels, because of this, gangrene could begin;
2. High doses of ergot alkaloids led to involuntary muscle contractions, uncontrolled convulsive movements and painful death.
Because of the similarity of the movements, ergot poisoning and the conscious ritual were called the same: the Dance of St. Vitus. Often these two factors were mixed, and when dangerous symptoms people began to dance vigorously in the hope that this ritual would help them heal.
Scientists state the fact that the symptoms of poisoning had differences and were regional in nature. It is assumed that the cause is ergot mutations and a different composition of alkaloids.
Saint Vitus and paganism
How historical figure, Saint Vitus lived in Sicily during the time of Emperor Diocletian. He converted to Christianity as a child under the influence of his mentor. Witt was given to be torn to pieces by lions, but the predators were not interested in him, and then the young Christian was thrown into a cauldron of boiling oil. It happened in 303, and after 1200 years a bizarre Catholic ritual was named after him.
The dances were reminiscent of the insane rituals of the Bacchantes, Bassarids, Maenads, Corybants and other priests of pagan antiquity. For many hours the dancers made erratic, convulsive movements, jumping in all directions; rolled on the ground and uttered inarticulate cries. In ancient times, there was even a name for such a dance: "chorea". It is now a neurological term for the symptoms of diseases leading to uncontrolled, erratic movements.
It is known that during antiquity, most participants first needed to “warm themselves up” with wine or other stimulants, and then their active and meaningless actions completely suppressed the brain. The dancers went into an uncontrollable frenzy, and there were frequent cases of murder during these rituals. According to legend, the singer and mystic Orpheus fell under hot hand dancing maenads and was torn to pieces by them.
I am not a doctor, but I can look, and this wonderful video seemed to me symptomatic, like many things.
How amazingly this video presents fatigue, loneliness, the desire for impulsive irrational acts (I act because I feel this way - this, by the way, is also shown in many films - I feel, I decide, I go to the end ...), and the desire for destruction is also present
How can you not remember the medieval dance of St. Vitus)
In the 14th century, a plague epidemic swept through Europe, claiming more than 20 million lives.
The main method of treatment remained, as usual, earnest prayer, repentance, kissing the cross and scrupulous administration of all church rites.
In the midst of this disaster came the feast of St. Vitus, which was always accompanied by massive feasts and dances.
Exhausted and desperate people, having drunk wine, began to dance rhythmically, brought themselves to a hysterical state and, no longer able to stop, fell dead.
An ominous, contagious amusement was transmitted from one urban area to another, from village to village, leaving lifeless human bodies behind.
The nightmare episode recorded by the chroniclers was reflected in fiction(Pushkin’s play Feast during the Plague is best known to Russians), as well as in modern psychological and medical terminology, where the dance of St. Vitus means a well-known clinical symptom:
"Involuntary movements of the arms, legs, torso, and often the facial muscles of the face are called choreic hyperkinesias.
At the beginning of the disease, they are barely noticeable, “random” in nature.
Often, in the early stages of the disease, obvious hyperkinesis is not visible, but some motor anxiety of a person is captured, he “does not sit still”.
In the future, as the disease develops, choreic hyperkinesis intensifies, affecting more and more muscle groups.
Characteristic manifestations of the disease are also violations of memory, thinking, intellectual activity, that is, cognitive impairments (or disorders of the cognitive function of the brain).
First of all, short-term memory suffers, concentration of attention is disturbed, a person becomes distracted. At the same time, the analytical function of the brain is lost, the ability to abstract, generalize and logical conclusions, thinking becomes primitive, there is a loss of habitual interests.
Personal characteristics of a person suffer, character changes, emotional-volitional and often mental disorders appear.
About what Vita's "dancing madness" looked like, medieval historical sources allow.
An entry in the Great Belgian Chronicle (Magnum Chronicon Belgicum) from 1374, which reads:
“This year, crowds of outlandish people arrived in Aachen and moved from there to France. Beings of both sexes, inspired by the devil, danced hand in hand in the streets, in houses, in churches, jumping and screaming without any shame. Exhausted from dancing, they complained of pain in their chests and, wiping themselves with handkerchiefs, lamented that it was better to die. Finally, in Lüttich, they managed to get rid of the infection thanks to prayers and blessings.
There are more detailed descriptions mass dances:
“Eight days before the feast of Mary Magdalene (July 14), a frantic dance appeared for the first time in one woman.
The magistrate sent her to the chapel of St. Vita to Zabern, where she calmed down.
But over the next four days, thirty-four more men and women fell ill.
The magistrate forbade drumming and trumpeting.
All the sick were also taken to St. Vita.
But, despite these measures, their number in a few days increased to two hundred.
The newly ill, in turn, were sent in separate batches to the chapel of St. Vita to Zabern and Rothenstein, some on foot, and others in carriages, in order to influence them with prayers and other sacred rites.
Saint Vitus (lat. Santus Vitus) was a historical character and lived in Sicily at the beginning of the decline of the Roman Empire. According to legend, from childhood he was endowed with the gift of healing the sick, and people, impressed by these miracles, converted to the faith of Vita - Christianity. Among the miracles performed by St. Vitus, the healing of the son of the Roman emperor Diocletian from demonic possession is mentioned. Apparently, therefore, later he was considered the patron saint of patients with epilepsy, since all convulsions at that time were considered demonic tricks.
In the Middle Ages, it was believed that anyone who danced in front of the statue of St. Vitus on his memorial day (June 15) would be in good health all year.
St. Vitus is considered the patron saint of dancers, actors, epileptics, as well as coppersmiths and brewers, helps not to oversleep and saves from thunderstorms and attacks by wild animals.
Chronology of dances
Such phenomena are surprising in themselves, but their number and prevalence are really striking (quoted from Chizhevsky’s monograph “The Earth Embraced by the Sun”):
1021 year. Epidemic in Dessau (Germany).
Several peasants began to dance and continued dancing until some of them fell dead; in others who survived, the manifestations of the disease remained in the form of convulsions.
1237.
Furious dance in Utrecht (Netherlands).
Two hundred people gathered on the Moselle bridge and danced until the bridge collapsed and they all perished in the river.
1337.
More than a hundred children jumped and danced through Steigerwald (a province in southern Germany) to the nearby town of Arystad, where they collapsed in exhaustion.
Many of these children died, others remained forever subject to the dance of St. - Vita.
1370 year.
In Europe, there was a mania for dancing.
"Dancers" filled the streets, especially in Germany and the Netherlands.
1375 year.
The epidemic spread from Aachen throughout Germany, mainly along the Rhine and Moselle, reaching the borders of Holland.
Patients "began to dance frantically and whirl ... for many hours, until they had such a strong tightness in the chest that they fell prostrate."
Many claimed that they saw streams of blood flowing along the ground under their feet, from which they tried to jump out and therefore tossed their legs high.
1479-1480
In Italy, an epidemic of frantic dancing took on a general distribution, behind which the name “tarantism” was strengthened.
1728.
An epidemic of convulsioners in Paris at the Saint-Medor cemetery.
The patients indulged in fantastic dancing, antics and twitches.
For repetition and a large number of participants, such mass dances in modern literature have received the name of psychopathic epidemics. But these epidemics were not only dance. Such researchers as Vladimir Bekhterev, Viktor Kandinsky and Karl Jaspers refer to cases of mass insanity as demonic possession, group self-immolation, self-flagellation, outbreaks of suicide, and similar phenomena. Of course, the actual data on these events are incomplete and insufficient. In addition, the historical sources from which they can be obtained are in most cases distorted by the religious ideas of those times, because the chronicles of events were mainly kept by the clergy.
Hypotheses
Thus, the data on dance and other mental epidemics are not sufficient to develop a fundamental scientific theory, but researchers offer several hypotheses.
Option 1. Hysteria
Psychiatrist Vladimir Bekhterev considered mass dances to be hysterical attacks. In his monograph "Suggestion and its role in public life” (1898) rightly noted the similarity of the clinical picture, as well as the possibility of induction, that is, the “contagiousness” of hysteria.
Option 2. Sun
Alexander Chizhevsky gives a very detailed study and chronological description of dance epidemics and connects their occurrence with a change solar activity. It is very difficult to argue with this theory, but nevertheless it is interesting why dance epidemics subsided by the 20th century—after all, the sun has not gone anywhere.
Option 3. Nervous diseases
From a clinical point of view, obsessive dance syndrome with clouding of consciousness, of course, seems to be associated with hereditary nervous diseases such as the aforementioned Huntington's disease or Tourette's syndrome, in which the extrapyramidal system and the basal ganglia of the brain are affected. But given that the highest frequency of their occurrence is 5-7 per 100 thousand of the population, it can be assumed that the connection of these diseases with the dance of St. Vitus is limited to the name and a similar clinic.
To recurring and mass diseases with similar clinical picture infection or intoxication may result.
lack of either one or the other medieval Europe did not have.
Option 4. Ergot
The idea of a connection between ergotism and the dance of St. Vitus was put forward in 1952 by the English researcher Eugene Backman (Backman Eugene Louis). Ergotism can indeed explain both the mass dance and psychopathic syndromes. Moreover, ergot was not only present in Europe, but was also considered a sign good harvest, that is, they did not fight it, and infected rye could well have been eaten, and poor nutrition and beriberi, characteristic of the Middle Ages, only exacerbated the clinical picture.
Ergot (uterine horns) is one of the long-known mushrooms. All ergot alkaloids are highly toxic. Symptoms of clavicepstoxicosis (or ergotism) are due to the ability of alkaloids to cause smooth muscle contraction and vasoconstriction and to have an effect on the nervous system. There are two forms of ergotism: gangrenous - "Antonov's fire" (which is accompanied by a violation of tissue trophism due to the irreversible narrowing of capillaries mainly in the limbs and auricles), and convulsive - "evil writhing". The factors responsible for these two forms of ergotism have not been fully elucidated. The convulsive variant is thought to be related to vitamin A deficiency.
Option 5. Fires
During the preparation of the article, I had my own version: perhaps the dance of St. Vitus was associated with damage to the nervous system carbon monoxide and toxic combustion products. Here we can again recall the connection with plague epidemics. As you know, in order to stop the infection, not only the corpses of the dead were burned, but everything that the sick had touched. The city during the plague was a grandiose conflagration. It is highly probable that this combustion took place under conditions of oxygen deficiency with the release of carbon monoxide.
In 1497, frightened residents of Luxembourg, in order to protect themselves from the terrible dances of St. Vitus, began to dance their dance in honor of the local saint Willibrord, the founder of the city, asking him to get rid of the disease. You can watch these relic dances of St. Willibrord even now - accompanied by an unpretentious melody played on violins, mandolins, trumpets and drums, they have survived to this day and every year they gather many tourists and pilgrims in Echternach.
Given the incompleteness and inaccuracy of the data, it is currently impossible to determine which of the proposed theories is closest to the truth.
It is also impossible to say for sure whether all the episodes mentioned were due to one reason or had a purely external similarity.
In any case, the dance of St. Vitus represents big interest as a medical, cultural and historical phenomenon.