Tiganov A.S. (ed.) ‹‹Exogenous-organic mental diseases. Mental disorders in syphilitic brain damage Treatment of progressive paralysis

Syphilitic infection, as is known, affects all organs and tissues, including the brain. In clinical psychiatry, two separate diseases are traditionally distinguished: syphilis of the brain itself and progressive paralysis (PP). Sometimes these diseases are combined under the general name “” (a52.1, f02.8). Syphilis (from the name of the poem by the Italian doctor G. Fracastoro « Syphilis Sive De Morbo Gallico» - “syphilis, or French disease”, 1530) of the brain most often occurs 2 - 4 years after infection, it is classified as an early form of neurolues, and progressive paralysis - as a late one. In early neurolysis, tissue of mesodermal origin (vessels, membranes) is primarily affected; in late neurolysis (progressive paralysis), along with these changes, extensive dystrophic and atrophic changes in the neurocytes of the cerebral cortex occur.

Term Lues Venerea was introduced. Fernel in 1554 to refer to contagious infections; In the Middle Ages, syphilis was called the “Italian disease” in France, and the “French disease” in Italy. Subsequently, it was noted that syphilitic psychoses developed in only 5-7% of those infected. The introduction of antibiotics to treat infections in the 20th century led to a significant decrease in the incidence of syphilis in the USSR, but since 1990 there has been a sharp increase in incidence, and the incidence rate has increased 3-4 times.

Syphilis of the brain and PP are progressive diseases and occur, as a rule, in cases of untreated or undertreated disease. Brain injuries and alcoholism are noted as predisposing factors.

Syphilis of the brain (lues cerebri)

Syphilis of the brain (meningovascular syphilis) - specific inflammatory disease with predominant damage to blood vessels and membranes of the brain. The onset of the disease is earlier than PP (four to five years after infection). The diffuse nature of brain damage corresponds to a significant polymorphism of symptoms, which resembles manifestations of nonspecific vascular diseases brain

The onset of the disease is gradual, with the appearance of symptoms characteristic of neuroses, primarily reminiscent of. Patients develop irritability, headaches, increased fatigue, and decreased performance. With special research different forms mental activity can usually be detected by their mild decline. Neurological examination reveals mildly expressed signs of stigmatization: anisocoria with a sluggish reaction of the pupils to light, asymmetry of the facial muscles, uneven tendon reflexes, and their increase. In contrast to similar symptoms observed during the development of cerebral atherosclerosis, the disease with syphilis begins at a young age and shows steady progression in the absence of the “flickering” symptoms typical of vascular disorders.

Against this background, when the meninges are damaged, signs of meningism are detected, or a picture of typical meningitis develops, which can be acute or chronic. In acute cases, general cerebral phenomena (dizziness, headaches, vomiting) come to the fore, body temperature rises, neck stiffness and Kernig's symptom are typical. Damage to the cranial nerves is typical; epileptiform seizures and symptoms of impaired consciousness such as stupor, confusion, or delirium may occur. However, more often in the membranes of the brain

A chronic inflammatory process develops, with penetration of the latter in some cases into the substance of the brain (chronic syphilitic meningitis and meningoencephalitis). Irritability and affective instability may increase, and depressed mood is often observed.

If meningitis develops on the convex (convexital) surface of the brain, the most pronounced symptoms are disturbances of consciousness and convulsive paroxysms, which are in the nature of Jacksonian or generalized seizures. The typical Argyle-Robertson symptom does not always occur. In some cases, meningitis is asymptomatic; the disease manifests itself only by characteristic changes in the cerebrospinal fluid.

In the apoplectiform course of syphilis of the brain, clinical manifestations are characterized by frequent strokes followed by focal lesions of the brain tissue.

At first, focal lesions are unstable and reversible, then they become more numerous and stable. At the same time, extensive neurological symptoms are constantly noted, its diversity is due to the difference in the localization of lesions; Paralysis and paresis of the limbs, damage to the cranial nerves, phenomena, and pseudobulbar disorders may develop. Almost all patients experience a weakening of the pupillary response to light.

In addition to the presence of focal symptoms, patients constantly experience persistent headaches, dizziness, memory loss, irritability, dysphoria, or weakness.

Some patients develop paroxysmal states with clouding of consciousness, mainly of the twilight disorder type. As the disease progresses and the neurological symptoms become more severe, dysmnestic dementia progresses.

Gummoznaya form of syphilis of the brain is manifested by the formation of chronic infiltrates in the brain in the form of nodes with different localization, which determines the characteristics of the symptoms of the disease. Gummas can be single or multiple, small in size.

The gummous form is characterized by signs of increased intracranial pressure with vomiting, painful headaches, adynamia, and sometimes the phenomena of clouding of consciousness and convulsive paroxysms may develop. When examining the fundus, congestive papillae of the optic nerves may be observed.

Syphilitic Plauta refers to the exogenous type of reactions, according to K. Bongeffer. Such manifestations cannot immediately be distinguished from, while syphilitic paranoid kraepelin is characterized by a predominance. Currently, both variants are grouped as a hallucinatory-paranoid form of syphilis with the manifestation of deceptions of feelings and the occurrence of delusional ideas, with either delusional symptoms predominating. More often observed or self-accusation. Delusional ideas are simple, associated with the patient’s immediate environment, with specific life situations.

Progressive paralysis

Progressive paralysis of the insane was first described by A. Bayle in 1822 as an independent disease, which later served as the basis for the development of a nosological direction in psychiatry. Much later a. Wasserman (1883) determined the presence of a spirochete in the blood, and x. Noguchi (1913) discovered it in the brain.

The disease is a syphilitic meningoencephalitis, which leads to progressive global destruction and disintegration of the personality and the entire psyche as a whole with the development of various psychotic disorders, polymorphic neurological disorders and the appearance of typical serological changes in the blood and cerebrospinal fluid. Untreated progressive paralysis in most cases leads to the development of marasmus and death after four to five years.

According to P.B. Posvyansky (1954), the frequency of progressive paralysis in patients admitted to psychiatric hospitals, tends to decrease from 13.7% in 1885-1900 and 10.8% in 1900-1913 to 2.8% in 1935-1939 and 0.78% in 1944-1948.

Frequency of progressive paralysis, according to a. c. Kosovo (1970), amounted to 0.5% in 1960-1964, according to x. Muller (1970) - 0.3%.

Clinical manifestations

The disease usually develops 10-15 years after infection with syphilis and is characterized by a slow, gradual onset of symptoms. This imperceptible creeping of the disease is very accurately described by Mr. Schule: “silently and quietly, sharply different from the tragic course and ending, the onset of the disease occurs. A person who has been hardworking and true to his word until now begins to cope somewhat worse with his affairs, ordinary things are more difficult for him, his excellent memory begins to stumble, mainly in things that until now belonged to the most ordinary, most familiar things for him. But who would suspect anything special about this? The patient's behavior is the same as before. His character has not changed, his wit has not suffered. Nevertheless, some change occurred in the patient. His mood was not what it had been before. The patient is neither gloomy nor excited, he still expresses his former sympathies and inclinations, but he has become more irritable. The slightest trifle can make him lose his temper, and, moreover, with such a temper that has never been seen in him before, he can forget himself to such an extent that he gives free rein to his hands, while before he had excellent control over his feelings and words.”

Such symptoms resemble manifestations; along with irritability, there is increased fatigue, forgetfulness, decreased performance, and sleep disturbances. Still, one cannot help but notice that such pseudoneurasthenic symptoms are combined with various progressive personality changes. Patients show indifference to their family members, lose their characteristic sensitivity and delicacy, they show previously uncharacteristic sloppiness, wastefulness, lose modesty, and may, to the surprise of their acquaintances, suddenly use obscene language.

In the next stage of the full development of progressive paralysis, the main symptom illness - dementia, pronounced disorders of memory and ability to memorize become obvious, weakness of judgment, loss of criticism are revealed. External manifestations diseases at this time can be different, which makes it possible to describe them as separate forms of progressive paralysis, which manifest themselves quite clearly at this stage of the disease.

Expansive form considered classic, manifests itself in manic excitement with lush delusions of grandeur of an absurdly grandiose nature. The mood of patients is elevated, it is sometimes euphorically complacent, sometimes accompanied by a feeling of happiness, sometimes agitated and angry. Patients express pompous, absurd, incredible in their senselessness ideas of greatness, which are in absolute contradiction with the real state of affairs. A complete loss of criticism, inadequate arousal, and disinhibition of drives are revealed.

Euphoric form These are cases in which dementia of the total type gradually increases against the background of a complacent-euphoric mood and the presence of fragmentary, mostly confabulatory ideas of greatness in the absence of acute manic excitement characteristic of expansive paralysis.

Depressive form characterized by a depressed mood and absurd hypochondriacal ideas (patients claim that they have no insides, they have long died and are decomposing, etc.).

Dementia (simple) form- the most common, it is characterized by progressive dementia, complacency in the absence of bright mental symptoms and relatively slow flow.

Agitated form characterized by a state of incessant senseless excitement with confusion, malignancy of the course, and rapid disintegration of the personality.

Other forms (hallucinatory-paranoid, catatonic, circular) are much less common.

Juvenile progressive paralysis occurs due to the presence of congenital syphilis during transplacental infection of the fetus from a sick mother. This type of disease is now extremely rare. In such cases, as a rule, other signs of congenital syphilis occur - parenchymal keratitis, deformation of the anterior

Teeth, lesions inner ear(Hutchinson's triad). Paralytic disorders are often combined with symptoms of juvenile tabes dorsalis. Juvenile paralysis manifests itself no earlier than six years of age, most often between 10 and 15 years of age. It may be preceded by a delay mental development, but sometimes the illness begins as if in the midst of complete health. Possible acute onset with epileptiform seizures, followed by dementia with manifestations of dysarthria, sometimes speech is completely lost.

Diagnosis of progressive paralysis is based not only on the characteristics of psychopathology, but also relies on data from neurological symptoms, somatic disorders and laboratory research. In most patients, the Argyle-Robertson symptom is determined with a weakening or absence of the pupils' reaction to light while maintaining their reaction to convergence and accommodation. Much less common are the absolute absence of pupillary reaction, narrowing (miosis) or dilation (mydriasis) of the pupils, in some cases their unevenness (anisocoria) and deformation. To frequent and early symptoms include dysarthria, slurred speech, or chanting. In approximately 60% of cases of progressive paralysis, signs of syphilitic damage to the aorta develop. Frequent bone fractures are caused by a combination with tabes dorsalis.

Laboratory data. Serological reactions to syphilis (for example, the Wassermann reaction) are positive in the blood and cerebrospinal fluid in most cases of progressive paralysis already at a dilution of 0.2. More sensitive reactions to syphilis have been proposed and used - the immobilization reaction of pallidum treponema (ribt), the immunofluorescence reaction (rif). An increase in the number of cells in the cerebrospinal fluid (pleocytosis), mainly lymphocytes, is characteristic, but an increase in plasma cells is also found. All globulin reactions (None-Appelt, Pandi, Weichbrodt) are positive. The total protein content in the cerebrospinal fluid is two to three times higher than normal. The globulin-albumin ratio (normally 1:4) is sharply changed due to an increase in globulins. The Lange reaction shows a “paralytic curve” with maximum loss in the first tubes.

Etiology and pathogenesis. The syphilitic etiology of progressive paralysis is proven clinically and laboratory. Japanese x. Noguchi (1913) discovered Treponema pallidum in the brains of patients with progressive paralysis. But the pathogenesis of the disease itself remains unclear. Only about 5% of people infected with syphilis suffer from progressive paralysis. Predisposing factors include hereditary burden, alcoholism, skull trauma, etc. However, most researchers believe that the absence or insufficiency of treatment can contribute to the development of the disease.

Differential diagnosis

The most important thing is to recognize progressive paralysis in the early stages of the disease, since it has been established that only those mental disorders that arise before the destruction of brain tissue can be eliminated with treatment.

Considering the non-specificity of “pseudoneurasthenic” manifestations at the onset, if signs of even a mild decline in the level of personality according to the organic type, epileptiform paroxysms, or transient apoplectiform states are detected, incipient progressive paralysis should be excluded. In such cases, it is necessary to conduct a thorough neurological, somatic, and serological examination. Difficulties may arise when distinguishing progressive paralysis from cerebrovascular pathology (atherosclerosis, hypertonic disease), as well as from . In such cases, neurological and serological examination data become a diagnostic aid.

Treatment

The introduction of malaria therapy and other types of pyrotherapy by Wagner von Jauregg (1917) became an important stage in the treatment of syphilis and progressive paralysis. Since the 40s of the 20th century, penicillin therapy has become the main method of therapy. Its effectiveness depends on the severity clinical manifestations illness and start date of treatment. Good-quality remissions develop in at least 50% of cases. Mental condition against the background of penicillin therapy, it improves in three to four weeks; blood sanitation can be completed in a period of two to five years. An average course of treatment requires 14 million units of penicillin. It is advisable to use a depot drug. It is recommended to carry out 6 - 8 courses of penicillin therapy with an interval of one to two months. If you are intolerant to penicillin, you can use erythromycin 5 times a day, 300 oooo units in combination with courses of bijoquinol or bismoverol. In treated patients, states of inpatient dementia, chronic expansive states, and psychotic variants of the defect are distinguished (P. B. Posvyansky, 1954). After therapy, a control study of cerebrospinal fluid is indicated to diagnose a possible relapse. An indicator of the stability of remission is evidence-based sanitization of the cerebrospinal fluid for at least two years.

Progressive paralysis (paralytic dementia, Bayle's disease) is a pathology of organic nature, marked by progressive disturbances in psychological and neurological activity, up to the onset. All processes can be accompanied by somatic and neurological disorders.

The probability of a patient developing progressive paralysis varies from 1 to 5% of all people who have suffered. Among other things, the male part of patients suffers from the disease more often - according to statistics, 5 times than women. Himself incubation period pathology is 10-15 years from the moment of infection.

The reason for the development of this disease there is syphilis. However, the risks may increase with the development of other sexually transmitted diseases, such as gonorrhea.

Stages of the pathology

In its course, Bayle's disease goes through 3 stages, namely:

Clinical forms of progressive paralysis

Each of the presented forms of the disease is marked by its own special clinical form the course of the pathology and it is on this issue that it is worth dwelling in more detail:

Symptoms of pathology

Symptoms of progressive paralysis will manifest themselves as follows:

• and a failure in the ability to connect complex words;
• a patient diagnosed with progressive paralysis loses the ability to do basic math, and his motivation decreases significantly;
• the patient's consciousness is clouded;
• – both short-term and long-term types;
• in the upper and lower limbs observed muscle atrophy and weakness, as well as weakness in other parts of the body;
• attacks of delirium and hallucinations, increased attacks of irritability and irascibility, which are followed by attacks of depression and moodiness;
• muscle cramp.

Complex of medical measures

The treatment process is reduced, for the most part, to a course of antibiotics and, accordingly, the fight against the root cause that provoked the course of the disease.

To date effective treatment progressive paralysis with Penicillin and drugs containing it. In case of individual intolerance or allergy to Penicillin, Ceftriaxone is used, which is administered intramuscularly or into a vein for 2-3 weeks. A repeated, control course of treatment, in the absence of deterioration of the patient’s condition, is indicated once every 1-2 years.

Most often, the course of treatment itself takes place in a psychiatric hospital, when, in addition to a course of antibiotics, a course of pyrotherapy, treatment with leeches, and drugs that suppress the causative agent of malaria are prescribed.

In this case, quinine or other antimalarial drugs, as well as compounds such as 2% sulfur solutions, are most often prescribed. In addition, Pyrogenal is also prescribed - it is administered by injection, intramuscularly, in a dosage of 10 to 30 mcg, gradually increasing to 80 - 150 mcg. The course of treatment ranges from 8 to 12 injections with an interval of 1 to 3 days.

After a course of pyrotherapy, doctors prescribe a combined course that is specific in nature and direction of treatment with using Penicillin, Bioquinol, Novarsenol.

After half a year or a year, doctors can prescribe a course of infectious and specific therapy, as well as a control study of cerebrospinal fluid and blood. If there is no positive dynamics of treatment, doctors recommend changing the infectious course of treatment using another pyrogenic drug.

In the absence of timely diagnosis and treatment, the patient develops disability, and the risk of developing other infectious diseases increases. Permanent incapacity, failure to communicate, and injury due to falls or convulsions may occur.

Not only the central nervous system, but also the entire organism as a whole. Therefore, the symptoms of progressive paralysis consist of a number of mental and somatic symptoms. Compared to cerebral syphilis, progressive paralysis has a longer period of time from the moment of infection to the appearance of the first signs of the disease, usually five to ten years. This gives the right to call it late syphilitic psychosis. The incidence of progressive paralysis in persons who have had syphilis ranges from 2 to 4% (according to old authors). Behind last years in the Soviet Union, this percentage decreased significantly due to active treatment of syphilis.

Progressive paralysis is one of the most well-known and accurately described mental illnesses. This form was isolated from other psychoses back in the 30s years XIX centuries based on the study of the course of the disease and post-mortem changes found in the brains of paralytics.

After the reaction to syphilis in the blood and cerebrospinal fluid was discovered in 1906, and then in 1913 a method was found for detecting the syphilitic spirochete in the brain of paralytics, all doubts about the causes of progressive paralysis were eliminated. It became clear that progressive paralysis was a special syphilitic disease of the brain.

Symptoms and course of the disease. The picture of mental changes during progressive paralysis increases imperceptibly. The first signs of the disease are very similar to neurasthenia: patients exhibit decreased ability to work, insomnia, increased irritability, absent-mindedness, complain of headaches, fatigue, and weakness. Already in the neurasthenic stage of progressive paralysis, changes are noted in the character of the patients, instability of mood that goes beyond simple irritability.

Soon the first harbingers of decreased criticism and weakness of judgment appear. Patients cease to notice the upcoming changes, and incorrect actions that were previously unusual for them appear in their behavior. Gradually, this is accompanied by memory and attention disorders, comprehension decreases, and patients become stupid and frivolous. The defects of criticism reach such a degree that patients are no longer tolerated either in the family or in society. They commit immoral acts, sexually harass women and children, make unnecessary purchases, etc. In the mood of patients, either excitement or depression increases more and more. complete absence consciousness of his illness.

Intellectual weakness progresses quickly, patients cannot cope with the simplest tasks, cannot perform usual actions, put on a suit, fasten buttons, etc. They walk around looking unkempt, shabby, half dressed, showing suggestibility and dementia, are easily influenced by bad influences, cry easily and laugh just as easily. Against the background of such mental decline, paralytics often develop ridiculous delusions of grandeur. Patients talk about their incalculable wealth, enormous abilities, great strength, and their activities that extend to the whole world. From all these manifestations of impoverishment of the psyche - weakness of judgment, decreased criticism, faint-heartedness, suggestibility - one develops paralytic dementia.

From physical symptoms with progressive paralysis, one can already early notice a mask-like face due to immobility of facial expressions, loss of dexterity and flexibility of movements, trembling of the eyelids, tongue and hands. Important signs of progressive paralysis are: absence pupillary reactions to light and slurred speech (dysarthria)) with omissions of syllables and individual consonants. The same can be seen in the writing of paralytics (shaking, omissions, repetition).

The course of progressive paralysis is often complicated seizures, after which patients experience short-term paralysis and paresis (incomplete paralysis).

Found in the blood and cerebrospinal fluid from the very beginning of the disease positive reaction for syphilis, which always helps in diagnosing the disease.

Clinical forms. Depending on the predominance of certain symptoms, several most typical forms are distinguished in the picture of progressive paralysis. Most common simple dementia form. The symptoms of dementia in it are most clearly expressed. The patients are uncritical, faint-hearted, their memory impairments are pronounced, the speech of the patients becomes more and more incomprehensible, helplessness and untidiness increase. The course of this form can be slow and prolonged. Classical expansive or manic form progressive paralysis. It proceeds with cheerful, euphoric excitement and delusional ideas of greatness, wealth, and power. The expansive form of paralysis will often give independent remissions (improvement).

Next depressive form of progressive paralysis is rare. It begins with depressive and hypochondriacal ideas associated with motives of self-blame, sinfulness, and fears of illness.

Finally, it should be mentioned youth uniform progressive paralysis, developing due to congenital syphilis at a young age.

Untreated progressive paralysis in most cases relatively quickly (within two to three years) results in an increase in severe dementia, as well as general physical exhaustion, which leads to death. The organs and tissues of a paralytic undergo changes: the skin becomes vulnerable, susceptible to non-healing abrasions and bedsores, bones become fragile and brittle. In the last stage of the disease, general weakness, loss of speech, inability to stand, and complete helplessness occur, which confines patients to bed.

Influenced treatment Patients first of all experience physical improvement: they get better, gain weight, their muscle strength strengthens, trembling, weakness, and headaches disappear. Mentally they become calmer, more focused, more serious. Their memory and attention are restored, delusional ideas and mood swings disappear, interest in life appears, and their ability to work returns.

Depending on the degree of recovery, it is customary to distinguish full recovery(such cases are relatively rare), improvement with defect and predominantly physical improvement.

This is a late form of syphilis, occurring in the form of progressive inflammatory-dystrophic total damage to cerebral structures. It differs in the stages of development; at first, symptoms of pseudoneurasthenia are observed, then psychical deviations against the background of intellectual decline, at the final stage - severe dementia. Diagnosis is carried out on the basis of the conclusions of a neurologist, psychiatrist, results of blood and cerebrospinal fluid tests for syphilis, and cerebral MRI data. Treatment is carried out comprehensively with pyrogenic, antitreponemal, psychocorrective, and neurometabolic drugs.

ICD-10

A52.1 Neurosyphilis with symptoms

General information

Progressive paralysis is a separate nosological form of late neurosyphilis. It is a syphilitic meningoencephalitis - an inflammatory lesion of the membranes and parenchyma of the brain. The disease was described in detail in 1822 by the French psychiatrist Antoine Bayle, after whom Bayle's disease was named in neurology. In 1913, Japanese pathologist H. Noguchi proved the syphilitic etiology of the disease by discovering treponema pallidum in the brain tissue of patients. Progressive paralysis occurs in 1-5% of patients with syphilis. The time range for the development of symptoms ranges from 3 to 30 years from the moment of infection, most often 10-15 years. The peak incidence occurs in the age range of 35-50 years. In modern neurological practice Bayle's disease is a fairly rare pathology, which is due to timely diagnosis and treatment of syphilis.

Causes

The etiological factor of the disease is the causative agent of syphilis - Treponema pallidum. The source of infection is a sick person, the main route of infection is sexual and injection. The pathogen can be transmitted through saliva. The introduction of an infectious agent into cerebral tissue occurs through hematogenous and lymphogenous routes. The reason for the long-term persistence of the pathogen, which is associated with progressive paralysis, is not known with certainty. Possible predisposing factors are the absence or insufficiency of correct treatment of early forms of syphilis, hereditary predisposition, and alcoholism. Triggers that provoke the activation of infection are considered to be traumatic brain injury, weakened immunity against the background of acute and chronic somatic diseases.

Pathogenesis

The mechanism of infection activation is unclear. Assumes a deterrent role immune system, blocking the reproduction of the pathogen. Impaired body reactivity and immunosuppressive conditions lead to the spread of Treponema pallidum in cerebral tissues. In the initial stage, progressive paralysis is characterized by inflammatory process covering everything brain structures- panencephalitis, then inflammatory changes are replaced by atrophic ones.

Macroscopically, there is clouding of the soft cerebral membrane, its fusion with the underlying brain parenchyma. Typical is ependymitis - inflammation of the lining of the ventricles of the brain. Hydrocephalus, diffuse atrophy of the cortex with widening of the furrows between the gyri, is observed. The microscopic picture is represented by vasculitis of small cerebral vessels with plasmacytic perivascular infiltration, shrinkage and death of neurons, demyelination and atrophic changes in nerve fibers, proliferation of glia, and the appearance of amoeboid cells.

Classification

Clinical symptoms are variable, weakly manifested at the onset of the disease, change and worsen as it progresses. Understanding the stage of the process is necessary to select the most effective therapy. In this regard, in neurological practice, progressive paralysis is divided into three clinical periods:

• Initial. Corresponds to the onset of clinical symptoms. Symptoms of neurasthenia, sleep disturbances, and decreased performance are typical. The initial stage lasts from several weeks to 2-3 months. By the end of the period, personality changes become apparent, with a tendency toward a loss of ethical standards.
• Development of the disease. Increasing personality changes, behavioral disorders, speech, gait and handwriting disorders, and intellectual decline predominate. Typical trophic disorders dystrophic changes somatic organs. Without treatment, the stage lasts 2-3 years.
• Dementia. The progressive collapse of the cognitive sphere is accompanied by apathy, a complete loss of interest in the environment. Epileptic seizures and strokes are often observed. Trophic disorders worsen, bone fragility increases. The patient dies from a stroke or intercurrent infections.

The following classification is based on a syndromic approach. Taking into account the prevailing symptom complex, a whole list of disease variants has been identified. The main ones are the following clinical forms:

• Dementia(simple) - increasing dementia with a predominance of euphoria. The most common. Not accompanied by severe mental disorders.
• Expansive- pronounced excitement with a predominance of delusional thoughts about one’s own greatness and wealth. The euphoric-comfortable mood abruptly gives way to anger. Typical verbosity, criticism is completely lost.
• Euphoric- progressive dementia is accompanied by fragmentary ideas of greatness. Unlike the expansive form, it occurs without pronounced neuropsychic excitement.
• Depressed- dementia progresses against the background of absurd hypochondriacal complaints. It seems to patients that they have no insides, that they have already died. Elements of depersonalization, derealization up to Cotard's syndrome are characteristic.
• Agitated- excitement is pronounced, there is a tendency to aggression. Visual and auditory hallucinations are observed. Consciousness is confused, a rapid disintegration of personality occurs.
• Circulatory- similar to manifestations of bipolar disorder. The period of manic excitement gives way to severe depression. There is an opinion that this form is realized against the background of a genetic predisposition to mental illness.
• Hallucinatory-paranoid- psychosis with a predominance of hallucinatory syndrome, delusions of persecution, catatonia. It has been suggested that this variant of the disease occurs in patients with schizoid psychopathy.

The most typical onset of Bayle's disease is in middle age. Progressive paralysis, manifesting in childhood and old age, is distinguished by a number of features. Accordingly, there are 2 age-related forms of the disease:

• Juvenile. Debuts at the age of 6-16 years after suffering congenital syphilis resulting from transplacental infection. More often it occurs in the form of dementia with Hutchinson's triad (keratitis, hearing loss, damage to the upper incisors). The manifestation of symptoms may be preceded by mental retardation.
• Senile. The debut occurs after 60 years. Typically a long asymptomatic course. The development of Korsakov's syndrome is possible. The senile form is extremely difficult to differentiate from senile dementia.

Symptoms of progressive paralysis

The disease begins with nonspecific, slowly increasing symptoms, similar to neurasthenic manifestations. Patients complain of decreased performance, unusual fatigue, irritability, headaches, memory impairment, and sleep disturbances. Over time, the patient’s relatives notice changes in his personality: indifference to the problems of others, loss of a sense of tact, behavior beyond ethical standards. Patients begin to make gross mistakes in their usual professional activities, and as symptoms progress, they lose the ability to recognize them. Sleep disorders worsen: hypersomnia is noted during the day, and insomnia at night. Eating disorders occur (anorexia, bulimia). Speech speeds up or slows down excessively. Criticism of one's behavior decreases.

At the stage of development of the disease, progressive paralysis is accompanied by worsening personality and behavioral disorders. Inappropriate behavior, ridiculous actions, and flat jokes are revealed. Mental disorders characterized by polymorphism, euphoria, depression, delusions, hallucinations, and depersonalization are detected. Difficulties in pronunciation occur in speech difficult words, then dysarthria is formed - speech becomes slurred, blurred, with the omission of individual sounds.

The gait is unsteady, loose, the handwriting is uneven, dysgraphia with missing letters is noted. Fainting and epileptic seizures are observed (usually of the Jacksonian type). Appetite disturbances cause weight loss or weight gain. Trophic disorders appear: decreased skin turgor, brittle nails, mild infection with the development of boils and abscesses. Characterized by increased fragility of bones, degenerative changes in the heart (cardiomyopathy) and liver (hepatosis).

During the period of dementia, there is an increase in dementia up to the point of insanity. The patient stops serving himself, interacting with others, and voluntarily controlling the function of the pelvic organs. Refusal to eat leads to significant weight loss. Dysphagia, a swallowing disorder, is sometimes noted. Numerous trophic ulcers form, increasing bone fragility causes fractures.

Complications

Cerebral vasculitis accompanying Bayle's disease is the cause of frequent strokes. As a result of a stroke, a persistent neurological deficit is formed: paresis, aphasia, apraxia. Trophic changes lead to a decrease in the barrier function of the skin with the development of pyoderma, abscesses, and more late stages- bedsores. Possible pneumonia, inflammation urinary tract(cystitis, pyelonephritis). The penetration of infectious agents into the blood causes the occurrence of sepsis. Strokes and infectious complications are the most common cause lethal outcome.

Diagnostics

The disease is most difficult to diagnose in the initial period. Some patients do not have anamnestic data on the presence of syphilis in the past, which also makes it difficult to determine the nosology. The diagnosis is established by laboratory confirmation of syphilitic etiology. Add to list necessary examinations includes:

• Neurological examination. In the debut stage, attention is paid to mild anisocoria, paresis of the extraocular muscles, asymmetry of reflexes, instability, unsteadiness of gait, monotony of speech. At the height of symptoms, the neurologist reveals a decrease in the photoreaction of the pupils, dysarthria, and severe cognitive dysfunction. Paresis, aphasia and other focal symptoms caused by strokes are possible.
• Neuropsychological testing. Carried out by a psychiatrist, neuropsychologist. Includes assessment of cognitive abilities and mental status. Characterized by dementia of varying severity, lack of criticism, psychomotor agitation, depressive-phobic, hallucinatory, delusional syndromes. Possible twilight consciousness.
• Laboratory diagnostics. Treponemal blood tests (Wassermann reaction, RPR test) are positive in almost 100% of patients. In doubtful cases, additional repeated analyzes are carried out in dynamics, RIF, RIT.
• Cerebrospinal fluid examination. Liquor is obtained by lumbar puncture. The analysis reveals an increased amount of protein, positive globulin reactions, and cytosis due to lymphocytes. The Lange reaction gives a specific waveform in the form of a tooth. Most patients have positive reactions of RIF and RIBT with cerebrospinal fluid.
• MRI of the brain. Depending on the phase of the disease, it reveals diffuse inflammatory or atrophic changes affecting the membranes, brain matter and ventricular ependyma. Scanning eliminates the presence volumetric education(intracerebral tumor, brain abscess), carry out differential diagnosis with progressive degenerative diseases.

The initial stage of Bayle's disease should be differentiated from neurasthenia. Features of the neurological status, the presence of epileptic episodes, and early changes in the patient’s personality noticed by loved ones can help. Subsequently, differential diagnosis is carried out with mental disorders, degenerative cerebral processes (vascular dementia, Alzheimer's disease, multiple system atrophy, Pick's disease). The most reliable criterion confirming progressive paralysis is positive treponemal reactions in the blood and cerebrospinal fluid.

Treatment of progressive paralysis

Patients need etiotropic and psychocorrective therapy. A therapeutic effect was experimentally discovered high temperature bodies. Modern treatment includes four main components:

• Pyrotherapy. Previously, it was carried out by introducing malaria pathogens followed by antimalarial therapy. Currently, pyrogenic drugs are used.
• Antibacterial therapy. Penicillin antibiotics, ceftriaxone, quinine iodobismuthate, and bismuth nitrate have an antitreponemal effect. Etiotropic therapy begins with large doses and continues for 2-3 weeks. At the same time, probiotics, multivitamin preparations, and, if necessary, hepatoprotectors are prescribed.
• . Indicated in the second phase of Bayle's disease. Individual selection of pharmaceuticals (sedatives, antipsychotics, antipsychotics, antidepressants) is carried out in accordance with the symptoms.
• Neurotropic therapy. Aimed at improving metabolic processes in the brain and restoring its functions. Includes pharmaceuticals with vascular, nootropic, and neurometabolic effects. At the stage of dementia, neurotropic treatment does not have a significant effect.

Prognosis and prevention

The results of treatment depend on the timeliness of its initiation. In 20% of cases, correct therapy allows one to achieve almost complete restoration of health. In 30-40% of patients, it is possible to achieve stable remission with moderate mental deviations, allowing them to adapt to society. In 40% of cases, persistent organic dementia persists. The process of sanitation of cerebrospinal fluid takes from six months to 3-4 years. Progressive paralysis can be prevented by maintaining personal hygiene, avoiding casual sexual intercourse, and unprotected sexual intercourse. When syphilis is first diagnosed, correct treatment is necessary until the biological environment is completely sanitized. Subsequently, the patient is recommended to be monitored and periodically examined for early detection of late forms of syphilis.

Progressive paralysis is a mental illness characterized by an increase in various paralysis and severe dementia, arising from a primary lesion of the brain substance itself, although tissue of mesodermal origin (vessels and membranes) also suffers. In this case, we are talking about the appearance and increase in the process of the disease of complete unresponsiveness, and therefore the normal automated reactions of the body disappear (coughing, swallowing, urination, etc.). At the same time, immunobiological reactivity decreases.

The classic picture of progressive paralysis includes three stages: the initial stage, the prime stage of the disease, and the final stage (the stage of insanity). Each of these stages is characterized by certain mental, neurological and somatic disorders. Currently, progressive paralysis has not only become rare, but has also changed its clinical picture. Nowadays there are practically no patients in a state of paralytic marasmus (stage III). The picture of progressive paralysis has noticeably changed due to the sharp predominance of the dementia form over all others. Due to active treatment, progressive paralysis is no longer considered, as before, a disease with a mandatory outcome of severe dementia.

Mental disorders

initial stage

The initial (neurasthenic) stage of progressive paralysis is characterized by the appearance of neurosis-like symptoms in the form of ever-increasing headaches, increased fatigue, irritability, bad sleep, decreased performance. These symptoms are soon joined by disturbances, which at first can be characterized as a loss of previous ethical skills. Patients become cheeky, sloppy, rude and tactless, and display previously unusual cynicism and a casual attitude towards their responsibilities. Then these personal changes become more and more brutal and conspicuous - the second stage begins (the heyday of the disease).

The heyday of the disease

Increasing memory disorders and weakness of judgment are revealed, patients can no longer fully criticize their condition and the environment, and behave incorrectly. They often reveal gross sexual promiscuity and completely lose their sense of shame.

There is often a tendency to waste, patients borrow money and buy completely random things. The appearance of talkativeness and boastfulness is typical.

The emotionality of patients also changes dramatically. A pronounced lability of emotions appears, patients easily have outbursts of irritation, up to severe anger, but these angry outbursts are usually fragile, and the patient can quickly be switched to something extraneous. These patients just as easily move from tears to laughter and vice versa. The predominant mood color may be euphoria that appears quite early. In some cases, on the contrary, there is a pronounced depressive state with possible suicidal tendencies. The emergence of delusional ideas is also possible, especially delusions of grandeur and one of its varieties - delusions of wealth.

The rapid increase in dementia leaves a clear imprint on the delusional creativity of patients. Paralytic delirium is usually characterized by absurdity and grandiose proportions. Less frequently, ideas of persecution, hypochondriacal delusions, etc. appear, usually also of absurd content. Hallucinations, mainly auditory, occur occasionally.

Over time, the decline in intelligence becomes more and more noticeable. Memory is sharply impaired, weakness of judgment and loss of criticism are increasingly revealed. Dementia with progressive paralysis, in contrast to cerebral syphilis, is diffuse and general in nature.

Marasmic stage

For the third (senile) stage of the disease (to which, as already indicated, the disease process now usually does not reach), the most characteristic is deep dementia, complete mental insanity (along with physical insanity).

Somatoneurological disorders

Quite often there is a combination of progressive paralysis with syphilitic mesaortitis. Specific lesions of the liver, lungs, and sometimes skin and mucous membranes may also be observed. General metabolic disorders are very characteristic. Trophic skin disorders, including the formation of ulcers, increased fragility of nails, hair loss, and swelling are possible. With a good or even increased appetite, there can be sharply progressive exhaustion. The body's resistance decreases; in patients with progressive paralysis, intercurrent (concomitant) diseases, in particular purulent processes, easily arise.

Clinical picture

A characteristic and one of the first neurological signs is Argyll-Robertson syndrome - the absence of pupillary response to light while maintaining it for convergence and accommodation. Other pupillary disorders are also characteristic: a sharp narrowing of the size of the pupils (miosis), sometimes to the size of a pinhead, anisocoria or deformation of the pupils is possible. Often with progressive paralysis, asymmetry of the nasolabial folds, ptosis, mask-like face, deviation of the tongue to the side, isolated fibrillary twitching of the tongue muscles and orbicularis muscles mouth (so-called lightning). Dysarthria that appears quite early is very typical. In addition to slurred and unclear speech, patients may miss certain words or, conversely, get stuck on a syllable, repeating it many times (logoclonus). Often speech becomes nasal (rhinolalia).

TO early manifestations There are also characteristic changes in handwriting (it becomes uneven, trembling) and impaired coordination of fine movements. As the illness progresses, more and more serious errors appear in the letters of patients in the form of omissions or rearrangements of syllables, replacement of some letters by others, repetition of the same syllables, etc.

Loss of coordination may also become more severe. Quite often, changes in tendon reflexes are observed, as well as a more or less pronounced decrease in sensitivity. Pathological reflexes may appear.

Disorders of the innervation of the pelvic organs are common. The most severe neurological symptoms are characteristic of the last, third stage of the disease. Mostly at this stage, apoplectiform seizures are observed, leaving behind initially smoothing and then increasingly persistent focal disturbances in the form of paresis and paralysis of the limbs, phenomena of aphasia, apraxia, etc.

A common pathology in progressive paralysis is epileptiform seizures, which in the third stage of the disease usually occur in series or in the form of statuses with a possible fatal outcome.

Forms of progressive paralysis

The expansive (classic, manic) form was one of the first to be described, not only due to its vivid clinical picture, but also its wide distribution. Now this form, especially in pure form, is quite rare. Characterized by the appearance of a manic state and absurd ideas of greatness. Against the background of a complacent-euphoric mood, outbursts of anger can sometimes suddenly arise, but this irritation is usually short-lived, and the patient soon becomes euphoric and complacent again.

Currently, the most common form is dementia (up to 70% of all cases). A vivid picture of general (total) dementia comes to the fore. The mood is dominated by either complacency or lethargy, indifference to everything around you.

In addition, there are depressive, agitated, seizure, circular, paranoid and atypical forms progressive paralysis.

In most cases, the Wasserman reaction in the blood and cerebrospinal fluid is positive already at a dilution of 0.2. Treponema pallidum immobilization reactions (RIBT and RIT), as well as the immunofluorescence reaction (RIF), are used. Lange reaction 7777765432111 is typical.

Untreated paralysis after 2-5 years leads to complete insanity and death. The phenomena of general insanity increase especially quickly with the so-called galloping paralysis, which is characterized by a catastrophic course of the disease. Most often this happens in the agitated form. The epileptic form is also very unpleasant.

The prognosis (both in relation to life and the further course of the disease and recovery) is determined, as a rule, by the results of treatment, which must be started as early as possible.

Treatment of progressive paralysis involves the use of specific combination therapy: antibiotics and bismuth and iodine preparations are prescribed in repeated courses according to the regimen.