Progressive paralysis is a severe consequence of cerebral syphilis. Tiganov A.S.

Progressive paralysis(synonym for Bayle's disease) - organic disease brain of syphilitic origin, determined by the rapid development of dementia (dementia) with neurological symptoms and cachexia. Progressive paralysis most often develops between the ages of 30 and 55 years, 10-15 years after infection with syphilis. Initial symptoms progressive paralysis, lasting from 1 to 6 months, is manifested by asthenia (see Asthenic syndrome) or depression. These disorders are always combined with memory loss, complaints of headaches, dizziness, tinnitus, lethargy, indifference to loved ones and one’s responsibilities, and irritability. There is no criticism of one’s wrong actions, physical and especially mental state. In some cases, only the symptoms of total dementia (see) increase - the so-called simple, or dementia, form progressive paralysis. In others, psychoses develop over months, at most a year: depressive with hypochondriacal-nihilistic delusions, manic with delusions of grandeur (see. Affective syndromes), states resembling catatonia (see Catatonic syndrome), hallucinatory-delusional pictures. Any of these psychoses that occur during progressive paralysis are always accompanied by symptoms of severe dementia. The latter give all delusional statements of patients an absurd character (see also Pseudoparalytic syndrome). Subsequently, the symptoms of psychosis disappear and increasing dementia comes to the fore.
Neurological disorders always exist and are manifested by dysarthric slurred speech, Argyll Robertson's symptom (see Tabes dorsalis), unsteadiness of gait, imprecision of movements, decreased or absent knee and Achilles reflexes, reversible mono- or hemiparesis.
Of the somatic disorders, especially in advanced cases, the phenomena of cachexia (see), trophic ulcers, fragility of bones, paralysis of the sphincters are noted. The Wasserman reaction in the blood and cerebrospinal fluid is almost always positive; the number of cellular elements is increased, the amount of protein is increased. The combination of progressive paralysis and tabes dorsalis (see) is called taboparalysis.
Treatment: Mandatory and immediate hospitalization with malaria treatment and antibiotics. With early treatment, recovery can be achieved.

Progressive paralysis (synonymous with Bayle's disease) is a parenchymal form of neurosyphilis, later syphilitic damage to the brain.
Progressive paralysis usually occurs 10-15 years after infection with syphilis, more often at 40-50 years, but it also occurs in younger people. In the past, progressive paralysis was a relatively common disease. Since the 30s of the current century, the incidence of P. p. both in the USSR and abroad has been decreasing due to the successes in the prevention and treatment of syphilis.
Pathologist and Chesky P. p. is characterized by the phenomena of chronic leptomeningitis, granular ependymatitis of the ventricles of the brain, internal and external hydrocephalus, and atrophy of the cerebral convolutions. In the initial stage of P. p. are noted inflammatory phenomena; later, dystrophic changes in the brain tissue occur.
IN clinical picture progressive paralysis combines symptoms of psychopathological, neurological and somatic disorders. According to the degree of clinical manifestations, three stages can be distinguished during P. p.: “neurasthenic” (initial), developed manifestations of the disease, and marantic.
In the initial stage of progressive paralysis, so-called neurasthenic phenomena are noted in the foreground in the form of increased fatigue, irritability, decreased performance, and headaches. The second stage occurs after a few weeks or months. It is characterized by deeper personality changes. Complacency or an unmotivated change in mood from euphoria to outbursts of irritability or a weak-willed reaction is revealed. Frequently observed absurd crazy ideas greatness: patients consider themselves the owners of enormous sums of money, countless treasures, rulers of the world, etc. Depressed mood is less common. A peculiar coarsening of the personality is also revealed, which is characterized by a loss of a sense of tact in communicating with people, a violation of social and personal connections. Patients make pointless purchases and spend money, regardless of the financial capabilities of the family. Impairments in memory and counting gradually increase, learning new things becomes impossible, and previous knowledge and skills are lost. The possibility of abstract thinking is reduced. Patients exhibit an inability to sufficiently assess the situation and regulate their behavior. There is no awareness of the disease. Due to the emergence of suggestibility and the weakening of moral inhibitions, patients sometimes commit criminal acts that bear the stamp of thoughtlessness and frivolity. Thus, at this stage the main clinical symptom progressive paralysis - diffuse dementia (dementia paralytica). Fluctuations in the state of consciousness in the form of varying degrees of stupor often aggravate the impression of the depth of dementia.
In the third, marantic stage of P. p., which occurs in the absence of treatment 2-3 years after the onset of the disease, phenomena of deep dementia and sharp exhaustion of patients are observed; they become untidy and develop bedsores.
Several forms of P. p. are described. The most common is simple dementia form. Other forms are distinguished depending on the predominance of additional symptoms (delusions of grandeur, epileptiform or apoplectiform seizures, agitation, delusions of persecution, harm). Accordingly, they are divided into expansive, epileptic, agitated, paranoid, and depressive forms. These forms are not stable and may change during the course of the disease. In addition, some atypical forms are also observed: juvenile P. p., developing in connection with congenital syphilis in children and adolescents with a clinical picture of dementia; taboparalysis, characterized by the addition of progressive paralysis to tabes; paralysis Lissauer, characterized by predominant damage to the posterolateral parts of the brain, according to which there are clinical focal symptoms(aphasia, apraxia, agnosia); Korsakov form with severe memory impairment and confabulations. The last two atypical forms are characterized by a slow increase in symptoms and resistance to therapy. In the post-war years, malignant current forms of P. p. with weakly expressed specific serological changes were described; these forms occur in the early stages of syphilis under the influence of additional hazards.

In parallel with the increase in mental changes, somatic and neurological disorders are gradually revealed - progressive weight loss, trophic disorders (ulcers, arthropathy, osteoporosis), hypersalivation, spontaneous fluctuations in body temperature, syphilitic damage to the aorta. One of the early neurological signs is Argyll Robertson syndrome (see Argyll Robertson syndrome). The pupils are usually sharply constricted (miosis). TO early symptoms include speech disorders such as so-called dysarthria (see). When writing, frequent omissions of syllables or words and shaky handwriting are noted. A mask-like appearance of the face and asymmetry of the nasolabial folds, characteristic of progressive paralysis, are observed. There are frequent tabetic symptoms (and not only in cases of taboparalysis) - the absence of patellar and Achilles reflexes, impaired sensitivity and coordination of movements. The Wasserman, Sachs-Vitebsky and Kahn reactions in the blood give a positive result in almost 100% of cases.
In the cerebrospinal fluid, the Wasserman reaction is positive in 100% of cases, there is an increase in the amount of protein to 1.0‰ and higher, positive or strongly positive protein reactions(Nonne - Appelt, Pandi, Weichbrodt), cytosis - several dozen cells per 1 mm3. The Lange reaction curve for progressive paralysis graphically looks like a tooth specific to P. p. (the so-called paralytic), and in digital designation - 66655432100. All these serological changes are so typical for untreated P. p. that negative serological data cast doubt on this diagnosis.
Diagnosis and differential diagnosis. The greatest diagnostic difficulties arise in the early stage of the disease, which is nevertheless distinguished from neurasthenia by the rapid progression of all painful manifestations and the presence of euphoria, Argyle Robertson syndrome, and characteristic serological changes. P. p. can be distinguished from the pseudoparalytic (most similar to progressive paralysis) form of cerebral syphilis on the basis of signs of profound dementia, characteristic neurological and serological changes, and a more rapid increase in all painful manifestations than with cerebral syphilis. The differentiation of P. p. from a brain tumor of the frontal localization, which is sometimes similar in clinical picture to it, is possible with a detailed clinical examination on the basis of congestive nipples, a negative Wasserman reaction, and the presence of protein-cell dissociation in the cerebrospinal fluid.
The prognosis for progressive paralysis is better when treatment is started early in the disease.
Treatment. WITH therapeutic purpose the patient is vaccinated with a pathogen tertian malaria. After 10-12 attacks, quinine and other antimalarials are prescribed (see Malaria, treatment). Other pyrogenic agents are also used (2% sulfur suspension, pyrogenal). A 2% suspension of sulfur in peach oil, after heating for 1 hour and shaking, is slowly injected intramuscularly into the upper outer quadrant of the buttock; initial dose 0.2 ml; subsequently the dose is increased by 0.2 to 1 ml or higher (depending on the reaction); The course of treatment involves 10-12 injections at intervals of 2-4 days. The domestic drug pyrogenal is prescribed as an intramuscular injection into the upper outer quadrant of the buttock in doses of 10-20-30 mcg with a further gradual increase in the dose to 80-150 mcg. The course of treatment is 8-12 injections at intervals of 2-3 days. The therapeutic effect of a 2% suspension of sulfur and pyrogenal is less than that of malaria therapy. After completion of pyrotherapy (in cases of malaria vaccination - from the middle of its course), combined specific treatment with penicillin, bioquinol, novarsenol is carried out according to special regimens (see Syphilis).
Mental and physical state Sometimes it improves immediately after a course of pyrotherapy, but more often gradually, over several months. As a result successful treatment mental disorders (including manifestations of dementia) are smoothed out, personality is restored. It is also possible to smooth out some neurological symptoms. Cerebrospinal fluid and blood are sanitized more slowly (from 1/2 year to 3-4 years). 1/2 - 1 year after infectious and specific therapy, a control study of cerebrospinal fluid and blood should be performed. In the absence of any clinical and serological effect, repeated pyrogenic therapy should be resorted to, and it is recommended to change the infection or use another pyrogenic agent.
The frequency of good remissions (full restoration of ability to work with minor personality changes) reaches 20%. Recovery with a defect and incomplete ability to work is observed in 30-40% of cases, in the rest - unstable improvement or no effect.

Progressive paralysis or Bayle's disease is a pathology that occurs as a complication after syphilis. This disease is an organic lesion of one of the most important organs – the brain.

Development mechanism

The disease does not begin immediately after infection with syphilis, but after a fairly long period of time (on average 10-15 years). The pathology occurs more often in men. From the primary focus (genital organs), the disease spreads throughout the body. In some cases it affects the brain.

A characteristic feature of progressive paralysis is that it affects the main substance of the brain (parenchyma) and does not spread to the membranes and vascular formations, surrounding him.

This makes it possible to distinguish between cerebral syphilis and progressive paralysis. Violation of the structure of organ tissue leads to the appearance of a clinical picture of the disease. It is worth noting that progressive paralysis does not occur in all patients with syphilis. IN Lately

the number of its occurrences has decreased significantly. This is explained by the fact that the pathology is associated with insufficient treatment of the primary disease - syphilis.

Thanks to modern medicine, with timely treatment of this disease, serious complications, including progressive paralysis, can be avoided.

Stages of the disease

1. The clinical picture of the disease has three main stages, each of which is characterized by certain psychological symptoms. These include:
2. neurasthenic stage;
3. severe dementia;

stage of insanity.

Let's take a closer look at each stage of the disease.

At the initial stages of progressive paralysis, patients experience a clinical picture characteristic of neurasthenia. The patient notes the appearance of the following symptoms:

• Sleep disorders (insomnia, daytime sleepiness);
• Severe irritability for no reason;
• Rudeness, impudence;
• Increased fatigue, chronic fatigue;
• Decreased activity (apathy, lethargy).

At this stage of the disease, one can notice the first signs of a change in the patient’s personality. People around him notice that the person becomes more rude and irritable, and primitive drives become more important to him.

Performance also changes: as the pathology progresses, it becomes increasingly difficult for the patient to perform his duties. Over time, a person will have to change his job to an easier one.

Dementia

At the stage of dementia, the patient's personality changes even more. At first, this may manifest itself in absurd, incomprehensible actions to others. For example, a patient may buy something that he does not need at all, or give an expensive gift to a complete stranger.

Many patients develop kleptomania, and the patient may openly take and try to take away some goods from the store.

An important sign of dementia is severe violation memory and loss of simple skills. A person cannot solve the simplest arithmetic problem or does not remember what city and what country he is in. Often the patient forgets where he lives, loses keys, money, documents.

A person may not recognize his loved ones because he does not remember their faces. Patients lose their criticality towards surrounding events and cannot analyze what is happening.

The following psychiatric symptoms are also characteristic of the stage of dementia:

1. The appearance of delirium;
2. Depressive states;
3. Increased anxiety;
4. Psycho-emotional arousal.

Depending on the patient’s behavior, there are several main forms of dementia:

1. Expansive form. The main manifestation is “delusions of grandeur.” The patient considers himself infinitely rich and famous, and tells ridiculous stories about wagons of gold and huge bank accounts. The patient feels euphoria, is pleased with what is happening, and feels like the center of the universe.
2. Dementia form. The patient is constantly in a good mood. He treats those around him kindly and calmly.
3. Depressive form. Patients show signs of depression and blame themselves for some wrongdoing. In some cases, ridiculous nonsense appears that they have already died, they have an incurable disease, etc.
4. Agitated form. With this course, the patient constantly experiences psycho-emotional arousal. He constantly wants to do something, run somewhere, tears off his clothes. Patients often shout meaningless phrases that they think explain the reason for their “rush.”

Thus, at the second stage, the change in the patient’s personality and brain damage are much more pronounced. In the absence of proper treatment, progressive paralysis can progress to the terminal stage, which is characterized by even more severe violations

psyche.

Stage of insanity

At the third stage of the disease, marasmus develops - a condition in which the patient loses the simplest skills. A patient with severe progressive paralysis cannot remember his name, where he lives, and does not recognize those around him.

His movements become chaotic, in many ways they are similar to children’s: the patient puts into his mouth everything he sees and can pick up.

Patients cannot move independently, they develop severe weakness and general exhaustion. That's why they are constantly in bed. Due to a sedentary lifestyle, accompanied by poor muscle nutrition, they develop muscle contractures, paresis or paralysis. Diagnostics

Diagnosis of the disease is usually not difficult. If a patient with a diagnosis of syphilis develops symptoms of psychiatric disorders, then he should consult a neurologist.

Brain paralysis is characterized by a number of neurological symptoms that can appear already in the initial stages of the disease and allow it to be diagnosed. So, speech disorders appear almost immediately. The patient cannot pronounce long and complex words normally, and cannot pronounce the simplest tongue twisters when the doctor asks him to. Another neurological symptom of the disease is constriction of the pupils. Sometimes they may not be the same size, which is also a sign of a pathological process. The reaction of the pupils to light is usually absent, but accommodation (sharpening, focusing) is preserved.

This combination is called the Argyll-Robertson symptom. This sign is characteristic symptom progressive paralysis and allows it to be diagnosed in time.

The disease is also characterized by impaired sensitivity. The patient often does not notice the appearance of scratches and abrasions. One more laboratory research. Signs of the disease can be detected by testing blood and cerebrospinal fluid. Based on the data obtained, the doctor makes a diagnosis and immediately prescribes treatment appropriate to the stage of the disease.

Treatment

Progressive syphilitic paralysis is a very serious disease, which for a long time was considered incurable. On this moment there is an effective therapy, but it has a strong side effect on the body.

Used to treat progressive paralysis complex therapy. First of all, the patient must undergo antisyphilitic therapy to prevent progression of the disease. However, specific treatment alone will not be enough.

Used in the treatment of syphilitic paralysis infectious therapy– infection of the patient with another pathogen in order to activate his immunity and eliminate the syphilitic lesion.

To do this, the patient is injected with a special serum containing malarial plasmodia.

After 1-2 weeks, the patient develops symptoms of malaria. The disease occurs in the form of frequent attacks. Doctors allow the patient to endure about 10 attacks of malaria, after which it is cured with the help of specific therapy. At the moment, infectious therapy is the only effective way

treatment of progressive paralysis.

It must be carried out as early as possible, since in the initial stages there is a greater likelihood that the functional activity of the brain will be restored. Thus, syphilitic paralysis is a serious disease that is characterized by a combination of psychiatric and neurological symptoms. In order to prevent the occurrence of such a serious complication, it is necessary to undergo anti-syphilitic therapy in a timely manner. This will help the patient maintain normal cognitive abilities. 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

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. A restraining role of the immune system is assumed, blocking the proliferation 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 an inflammatory process that covers all 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. Trophic disorders and dystrophic changes in somatic organs are typical. 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, lesion upper incisors). The manifestation of symptoms may be preceded by mental retardation.
• Senile. The debut occurs after 60 years. Typically long-term 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 activity, as symptoms progress, they lose the ability to recognize them. Sleep disorders worsen: hypersomnia is noted during the day, insomnia at night. Violations occur eating behavior(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 are characterized by polymorphism, euphoria, depression, delusions, hallucinations, and depersonalization are found. In speech, difficulties arise in pronouncing complex words, then dysarthria develops - 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 of death.

His movements become chaotic, in many ways they are similar to children’s: the patient puts into his mouth everything he sees and can pick up.

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. The list of necessary examinations includes:

• Neurological examination. At the onset stage, attention is paid to mild anisocoria, paresis oculomotor 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. Characteristic of varying severity dementia, 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. In most patients, RIF and RIBT reactions with cerebrospinal fluid are positive.
• 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 makes it possible to exclude the presence of a space-occupying lesion (intracerebral tumor, brain abscess), and to carry out a differential diagnosis of 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. In the future, 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. It was discovered experimentally healing effect high body temperature. 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 complete recovery biological media. Subsequently, the patient is recommended to be monitored and periodically examined for early detection of late forms of syphilis.

Progressive paralysis (syn. Bayle's disease) is considered a rather rare form of cerebral syphilis, since it is diagnosed on average in 5% of people who have suffered such a disease. It is noteworthy that most often males suffer from this disease.

The disease does not begin immediately after infection with syphilis - on average, it takes 10-15 years. The main causative agent of this pathology is treponema pallidum from the primary focus, that is, from the genital organs, which spreads throughout the body.

The clinical picture includes a large number of signs, including:

• severe headaches;
• noise in ears;
• disruption of food digestion processes;
• insomnia;
• muscle weakness;
• problems with coordination of movements;
• hallucinations.

To make a correct diagnosis, a comprehensive examination of the body is necessary. This means that diagnosis will consist not only of laboratory and instrumental studies, but also of activities performed directly by the clinician.

Treatment in this case is carried out only conservatively and consists of long-term use antibacterial agents. The prognosis directly depends on the stage of the disease and the time of initiation of specific therapy.

Etiology

The disease is provoked by Treponema pallidum, which acts as a causative agent. Such a microorganism from the primary focus invades the nervous system and has a destructive effect on it. In extremely rare cases, it reaches the brain. Against this background, progressive paralysis actually develops.

This disease has some features: it affects only the parenchyma (the main substance of the brain) and does not spread to the membranes or vascular formations that surround this organ.

There are several ways for pathogenic bacteria to enter the brain:

• lymphogenous - penetration occurs through the human body, namely through damaged mucous membranes or skin. The infection spreads through the bloodstream;
• hematogenous - in such situations, the pathogen infiltrates into cerebrospinal fluid. Against this background, not only the brain, but also the spinal cord is involved in the pathological process.

The main risk group consists of people of working age, namely from 30 to 50 years. It is noteworthy that the disease does not develop immediately after infection with syphilis. This is due to the fact that only over time human body stops fighting pathogenic bacteria.

Classification

Progressive paralysis in its clinical picture has several stages of progression, each of which has its own specific symptoms. The stages of progression are:

• initial stage or ;
• advanced stage or severe dementia;
• terminal stage or marasmus.

There are also several forms of the disease:

• expansive – the occurrence of manic symptoms, euphoria and meaningless ideas. The person considers himself completely healthy, moreover, he is satisfied with the fact that he is in a psychiatric institution;
• depressive – depressed mood combined with ridiculous hypochondriacal delirium;
• Dementia – an increase in dementia, the patients’ mood is cheerful and complacent;
• tabetic - the addition of signs of tabes dorsalis to the symptoms of paralytic dementia;
• agitated – malignant course. A person's confusion is clearly expressed and mental disintegration quickly develops.

This pathology also includes the following stages:

• latent - begins to develop a year after infection. However, there are no specific clinical manifestations. Headaches and dizziness may occur;
• acute – manifests itself on average after 2 years and has nonspecific symptoms, including nausea and vomiting, a slight decrease in hearing and visual acuity;
• meningovascular - diagnosed in the 7th year of its course. There are complaints from personality disorder and emotional instability;
• meningomyelitis – there is a disruption in the functioning of the pelvic organs and lower extremities, problems with sensitivity appear;
• tabes dorsalis - often manifests itself after 20 years of the disease. The presence of neurogenic disorders is noted;
• paralytic dementia - begins to develop 10-20 years after infection with syphilis. It is characterized by rapid progression, which often leads to death.

Infantile and juvenile progressive paralysis is also distinguished - it is extremely rare in persons from 6 to 16 years old. In the vast majority of situations, it acts as a rare form of congenital syphilis.

Symptoms

The clinical picture of such a disease will progress along with the severity of its course. So, at the initial stages of development there are:

• sleep disorders – this includes insomnia or daytime drowsiness;
• causeless irritability;
• chronic fatigue;
• constant weakness;
• severe headaches;
• decreased ability to work - due to the fact that a person cannot fully perform his duties, he is forced to change his job to an easier one;
• frequent mood swings - rudeness and arrogance often come to the fore;
• decreased visual acuity.

At the stage of dementia there are:

• severe memory impairment;
• loss of simple skills;
• inability to recognize loved ones and friends;
• loss of criticality to surrounding events;
• inability to analyze what is happening;
• disorientation;
• lack of initiative;
• increased anxiety;
• personal changes;
• psycho-emotional arousal.

On later stages join:

• clouding of consciousness;
• speech dysfunction;
• loss of ability to count;
• muscle weakness in the upper and lower extremities, as well as in other parts of the body;
• loss of short-term memory;
• delusions and hallucinations;
• capriciousness and short temper;
• seizures;
• changes in pupil size and reactivity;
• weakness and general exhaustion - for this reason a person is forced to constantly stay in bed;
• paresis and paralysis;
• attacks of loss of consciousness.

If such symptoms are ignored, profound dementia develops and the likelihood of death increases significantly.

His movements become chaotic, in many ways they are similar to children’s: the patient puts into his mouth everything he sees and can pick up.

This pathology is expressed in a large number of quite specific external manifestations, namely mental disorders. In order to make a correct diagnosis, it is necessary to carry out wide range diagnostic measures. First of all, the clinician must personally perform several manipulations, including:

• studying the medical history to confirm the fact of infection with syphilis;
• collection and analysis of life history;
• assessment of the condition of the pupils and visual acuity;
• neurological examination;
• a detailed survey of the patient or his relatives to establish the first time of onset and severity of characteristic symptoms.

Laboratory research in in this case presented:

• general clinical blood test;
• microscopic examination of cerebrospinal fluid;
• blood biochemistry;
• specific tests with blood serum;
• blood tests for syphilis RIF and RIT.

As for instrumental diagnostics, it is limited to:

• CT and MRI of the head;
• lumbar puncture– this is necessary to obtain liquor;
• procedures aimed at studying nerve conduction.

This disease must be differentiated from:

• tumor lesions or vascular pathologies of the brain;
• serous;
• cerebral;
• malignant forms.

Treatment

Getting rid of such a disease is carried out only by conservative methods. Treatment is based on long-term use of antibacterial substances; patients are usually prescribed 8 courses of such drugs. Often this technique is supplemented with “Biyoquinol”.

Previously, three-day malaria vaccinations were widely used in therapy, which were aimed at increasing temperature indicators. This was due to the fact that this was the only way to achieve the death of Treponema pallidum.

Today, substances such as Sulfozin and Pyrogenal are used for this. Patients may also be prescribed:

• vitamin complexes;
• a nicotinic acid;
• amino acids with antioxidant, neurometabolic and neuroprotective properties;
• iron supplements;
• general strengthening substances.

In addition, treatment may include:

• limb massage;
• electrophoresis and other physiotherapeutic procedures;
• gymnastics according to Frenkel;
• homeopathy;
• folk remedies.

Surgical therapy is not used in the treatment of this disease.

Possible complications

If a person or his relatives ignore the clinical manifestations of the disease and refuse qualified help, then the possibility of developing the following complications cannot be ruled out:

• complete loss of skills;
• incapacity;
• violations of communicative behavior;
• extreme degree of exhaustion;
• injuries sustained due to loss of consciousness.

Prevention and prognosis

Since progressive paralysis is a form of syphilis, prevention, first of all, will be aimed specifically at preventing the occurrence of this particular disease.

Thus, to preventive measures relate:

• avoidance of casual sexual relationships;
• engaging in protected sex only;
• compliance with the rules of personal and intimate hygiene;
• refusal to use household items together with a person with syphilis;
• regular preventive examinations in medical institution with mandatory visits to all clinicians, including a neurologist.

The prognosis of the disease is generally unfavorable - only every fifth patient achieves complete remission. In 30% of situations, recovery will be accompanied by minor mental manifestations. In other cases, even specific treatment turns out to be ineffective, and the complications described above develop, leading to disability and death.

Is everything in the article correct from a medical point of view?

Answer only if you have proven medical knowledge

Until the middle of the 20th century, this disease was not considered as an independent nosology; it was mistakenly interpreted as the consequences of epidemic encephalitis, which was widespread at that time. The fact is that a large number of cases of polymorphic postencephalitic parkinsonism masked rarer pathologies that were considered atypical forms.

Progressive supranuclear palsy as an independent neuropathology was identified in 1963-1964. a group of Canadian doctors: neurologists J. Steel and J. Richardson and pathologist J. Olszewski. They described and analyzed 7 cases of neurodegeneration with a characteristic clinical picture. In the USSR, progressive supranuclear palsy was first mentioned in 1980 by doctors at the Clinic of Nervous Diseases of the Moscow Medical Academy. THEM. Sechenov, who observed two patients.

Subsequently, the disease continued to be studied and was identified in domestic and world classifications as a separate nosological unit. In ICD-10, progressive supranuclear palsy is classified as a disease nervous system(section of extrapyramidal and other movement disorders, subsection of other degenerative diseases of the basal ganglia), coded G23.1.

A52.1

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.

Progressive supranuclear palsy (PSP) is a degenerative brain disorder unknown etiology. Along with Alzheimer's disease, multiple system atrophy, corticobasal degeneration, Pick's disease, PSP refers to tauopathies characterized by the formation of tau protein inclusions in neurons and glial cells.

Progressive supranuclear palsy was first described in detail in 1963-64 by Canadian neurologists Steele and Richardson in collaboration with pathologist Olszewski, after whom the Steele-Richardson-Olszewski syndrome is named. The prevalence of the disease, according to various information sources, varies between 1.4-6.4 cases per 100 thousand population.

Prevalence

The disease is based on the progressive degeneration of neurons, resulting from the accumulation of pathological protein in them.

According to modern medical statistics, progressive supranuclear palsy is the cause of 4-7% of cases of diagnosed parkinsonism. But even now some patients with this disease have misdiagnoses, especially in the early stages. The overall prevalence of progressive supranuclear palsy in the population averages 5 cases per 100 thousand population, from 1.4 to 6.4 cases in different countries.

The disease is detected mainly in people of the older age group; even hereditary forms usually appear at the age of 50 years.

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.

The etiofactors that trigger degenerative processes of a certain cerebral localization remain unknown. Most cases of the disease are sporadic. Individual familial variants with presumed autosomal dominant inheritance have been identified since 1995. Molecular genetic studies have shown that some forms of PSP are caused by defects in the tau protein-encoding gene located at the 17q21.31 locus. The most likely is a multifactorial mechanism for the occurrence of pathology, which is realized against the background of genetic predisposition.

Etiology: why this disease develops

In 1913, H. Noguchi proved the syphilitic etiology of P. p. by discovering treponema pallidum in brain tissue.

Pathogenesis has not been studied enough. K. Levaditi, based on observations when both spouses or several persons infected with syphilis from the same source fell ill with syphilis, suggested the existence of a special neurotropic treponema. which, however, has not been proven. Perhaps disturbances in the reactivity of the body (see) with sensitization of brain tissue play a role in pathogenesis, as a result of which in some cases treponemes penetrate into the brain tissue.

The causes of progressive supranuclear palsy are not reliably known. This disease is not associated with any infections, previous injuries or exposure to external adverse factors.

Progressive supranuclear palsy is considered a sporadic pathology (occurring randomly in the population). At the same time, since 1995, information has been published on the presence of rare familial cases of the disease with an autosomal dominant type of inheritance. This variant of the disease is associated with a heterozygous mutation of the gene encoding tau protein and located on 17q21.31.

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. Trophic disorders and dystrophic changes in somatic organs are typical. 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.
• Depressive - 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 - the 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 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 undergoing 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 onset of symptoms may be preceded by impaired mental function.
• Senile. The debut occurs after 60 years. Typically long-term asymptomatic course. Possible development Korsakov's syndrome. The senile form is extremely difficult to differentiate from senile dementia.

Pathogenesis: what happens in the brain?

The development of symptoms of progressive supranuclear palsy is associated with irreversible and steadily increasing degeneration of neurons in certain areas of the brain. The basis of this destructive process is the excessive intracellular accumulation of neurofibrillary tangles and neuropil threads that have lost their structure. They disrupt the functioning of neurons and promote their premature apoptosis (programmed self-destruction).

Neurofibrillary tangles in the cytoplasm of brain neurons are formed by a special τ protein (tau protein), which is in a pathological hyperphosphorylated state. Normally, it is attached to tubulin microtubules and is responsible for their polymerization and stabilization of microtubules and fixation of some intracellular enzymes.

The main functions of normal tau protein include:

1. participation in the processes of maintaining the neuronal cytoskeleton (nerve cell framework);
2. formation and elongation of axonal processes;
3. restoration of neurons after damage;
4. regulation of intracellular transport of vesicles (cytoplasmic vesicles) with synthesized neuropeptides.

Hyperphosphorylated tau protein is no longer able to maintain the microtubule structure. They disintegrate, and the protein that has become abnormal forms filaments (tubules) of irregular shape, which gather in the cytoplasm into neurofibrillary tangles. In the affected cell, biochemical contact with other neurons is disrupted, the ability to form and maintain axonal connections is lost, the cytoskeleton becomes unstable, and the lifespan is significantly reduced. Such neurodegeneration is irreversible and progressive, gradually spreading from the characteristic primary zones to the entire brain.

Neurofibrillary tangles in neurons are formed not only during progressive supranuclear palsy. Similar degenerative changes in the brain are also found in Alzheimer's disease, corticobasal degeneration, frontotemporal dementia and some other, more rare diseases.

The specially created Reisenburg Institute is engaged in the study of various tauopathies, the development of issues of their differential diagnosis and the clarification of nosological criteria. working group(Reisensburg Working Group for Tauopathies With Parkinsonism).

Symptoms of PSP

Progressive supranuclear palsy is characterized by a nonspecific clinical onset. Symptoms of this period include unusual fatigue, decreased performance, cephalgia, dizziness, low mood, narrowing of interests, sleep disorders, including insomnia at night and hypersomnia during the day. Subsequently, symptoms of akinetic-rigid parkinsonism appear. Postural tremor is absent in most patients. Muscle rigidity is expressed mainly in the axial muscles - the muscles running along the cervical spine, connecting it to the skull. Patients complain of stiffness in the neck and back. Increased tone in the posterior neck muscles leads to a typical “proud” position of the patient’s head. Parkinsonian ataxia is characteristic, caused by a disorder of coordination of the position of the trunk and lower extremities relative to the center of gravity. Difficulty maintaining balance while walking leads to frequent falls backwards.

A distinctive feature of PSP is ophthalmoplegia, which occurs on average 2-3 years after the onset of the disease. Against the background of slow movement of the eyeballs, gaze paralysis occurs in the vertical plane, the patient cannot lower his eyes down. Due to infrequent blinking, the patient feels discomfort and burning in the eyes. Possible blurred vision, convergence disorder, blepharospasm. Progressive supranuclear ophthalmoparesis is accompanied by limitation of gaze down and up, and over time can lead to oculomotor disorders in the horizontal plane. With the development of complete ophthalmoplegia, retraction of the upper eyelids is formed, which gives the face a surprised expression.

In the clinical picture of PSP, pseudobulbar manifestations appear relatively early: dysarthria, dysphagia, forced crying or laughter. Changes occur in the personal-emotional sphere, patients become withdrawn, apathetic, demotivated, and indifferent. Cognitive impairment in most cases occurs at the height of the disease, in 10-30% of cases - at the debut stage. Characterized by intellectual decline, disorders of abstract thinking and memory, visuospatial apraxia, elements of agnosia. Dementia is observed in 60% of patients with a 3-year history of the disease.

The disease begins with nonspecific, slowly increasing symptoms, similar to neurasthenic manifestations. Patients complain of decreased performance, unusual fatigue, irritability, headaches, memory impairment, 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, 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 are characterized by polymorphism, euphoria, depression, delusions, hallucinations are found, depersonalization. In speech, difficulties arise in pronouncing complex words, then dysarthria develops - 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, epileptic seizures are observed (usually of the type Jacksonian epilepsy). 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 bone fragility, dystrophic 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, increasing bone fragility causes fractures.

Patients need etiotropic and psychocorrective therapy. The therapeutic effect of high body temperature was experimentally discovered. 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.
• Psychotropic treatment. 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.

What is affected by progressive supranuclear palsy?

With progressive supranuclear palsy, neurodegeneration in most cases begins in the subcortical and brainstem formations. The cerebral cortex is initially affected to a lesser extent, but as the disease progresses, the process steadily spreads to it. In this case, the anterior parts of the cerebral hemispheres are predominantly affected.

Localization of main changes:

• substantia nigra;
• subthalamic and peduncular nuclei;
• pale globe;
• thalamus;
• midbrain tegmentum;
• stem part of the reticular formation;
• temporal and prefrontal areas of the cerebral cortex.

Primary damage to these areas explains the characteristic order of onset of symptoms and the typical Parkinson-like onset of the disease. And the obligatory presence of oculomotor disorders is associated with degeneration of the dorsal part of the midbrain, which leads to a disconnection of connections between the gaze centers in the cortex and brain stem. The nuclei of the cranial nerves themselves, which are responsible for the functioning of the muscles of the eyeballs, remain intact. That's why the paralysis is called supranuclear.

Are obligatory manifestation diseases and are characterized by a characteristic combination of symptoms:

• Loss of ability to voluntary movement eyeballs: usually first in the horizontal and then in the vertical plane. The outcome is complete ophpalmoplegia with the inability to purposefully move the gaze.
• Decreased convergence, which already in the early stages of the disease is accompanied by complaints of blurred vision and double vision when looking at objects at different distances.
• Preservation of reflex friendly movements of the eyeballs.
• The appearance of the phenomenon of doll's eyes, when the eyeballs, when moving the head, continue to involuntarily fixate the object. This is explained by the lack of suppression of vestibulo-ocular syndrome. At earlier stages, there is intermittency and “lag” in the gaze’s tracking of a moving object (on examination, a neurological hammer), which leads to the appearance of spasmodic “catching up” movements of the eyeballs.
• Gradual decay of the amplitude and speed of voluntary saccadic movements of the eyeballs. During a neurological examination, this is revealed when checking tracking movements; each repeated shift of gaze to the extreme lateral leads is accompanied by an increasing limitation of eye mobility (hypometry).
• Absence of spontaneous nystagmus.

The method of provoking optokinetic nystagmus can be used by rotating the striped drum in front of the patient's face. With progressive supranuclear palsy, the fast phase of nystagmus in the vertical plane initially slows down, and in later stages of the disease it is not caused at all.

The first signs of ophthalmopathy usually appear in the early stages of the disease. Moreover, a decrease in convergence, changes in optokinetic nystagmus and a decrease in voluntary vertical saccades are often detected even when the patient has no complaints of visual impairment. To make a presumptive diagnosis, it is necessary to have at least a limitation of downward gaze, in combination with other signs of a neurodegenerative process.

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 in later stages - bedsores.

IN initial period Falls of the patient without the ability to coordinate their movements lead to bruises and fractures. After several years, progressive oligobradykinetic syndrome leaves patients bedridden. In the absence of proper care, immobility is dangerous for the development of joint contractures, bedsores, and congestive pneumonia.

Progressive pseudobulbar palsy causes choking on food with the risk of asphyxia and aspiration pneumonia. Sleep apnea can cause sudden death in a dream. A serious complication is the addition of intercurrent infections (pneumonia, cystitis, pyelonephritis), since against the background of reduced immunity there is high risk development of sepsis.

Clinical picture

P. p. is a serious disease of the whole organism, and its most striking manifestations are disturbances in mental activity. The main syndrome is progressive total dementia (see): the intellect suffers severely, disorders of judgment appear early, criticism and especially self-criticism disappear. There is no awareness of the disease, memory sharply decreases, and confabulations occur (see Confabulosis).

Manifestations of dementia intensify due to the often observed euphoria (see Psychoorganic syndrome). Neurol. the symptoms consist of speech disorders, primarily in violation of articulation - dysarthria (see). Speech becomes unclear and slurred, especially when pronouncing long words, the patient skips or rearranges syllables, does not pronounce the endings of words.

Handwriting becomes uneven, individual letters and syllables drop out of words. The timbre of the voice changes, it becomes dull. The patient's face is expressionless and mask-like, because the innervation of the facial muscles is disrupted, and blepharoptosis occurs (see Ptosis). Tendon reflexes are often increased and uneven, except in cases of taboparalysis (see).

Based on psychopathol. manifestations, four stages of the disease are distinguished: latent (from infection with syphilis to manifestations of P. p.), the stage of initial manifestations, the stage of full development of the disease and the stage of marasmus (see). In the latent stage, headaches, dizziness, fainting may occur, and in some cases characteristic changes in the cerebrospinal fluid (see).

The stage of initial manifestations is characterized by increased fatigue, irritability, and weakness. Patients complain of loss of strength and decreased performance, although they can still perform their usual work to some extent. Previously, such conditions, due to their external resemblance to neurotic symptoms, were incorrectly called preparalytic neurasthenia.

In some cases, at the stage of initial manifestations, depressive and delusional disorders are observed - anxious depression with hypochondriacal statements, anxious-agitated depression, delusional ideas of jealousy, persecution, poisoning; as the symptoms of dementia increase, these endoform disorders disappear.

Memory loss is detected very early. Certain actions indicate a violation of criticism. The sphere of desires is upset, patients become gluttonous and erotic. The increase in these disorders indicates the transition of the disease to the stage of full development, the edges manifest themselves in different wedges. forms.

The expansive, or classic, form (previously widespread) is more common in men. It is characterized by the presence of manic excitement with the manifestation of anger, grandiose delusions of grandeur (see Delirium). The dementia form is characterized by increasing dementia against the background of inactive euphoria. In the depressive form, a depressed mood develops, often with anxiety and a desire for suicide (see.

Depressive syndromes), often there is an absurd hypochondriacal delusion of nihilistic content. The circular form, first described by S. S. Korsakov, occurs with alternating states of excitement and depression. The hallucinatory-paranoid form is characterized by the development of paranoid syndrome (see) with predominantly auditory hallucinations and delusions of persecution.

In the catatonic form, a stuporous state occurs (see) with phenomena of mutism and negativism (see Catatonic syndrome). In the stage of insanity, conscious activity ceases, speech disappears, patients make inarticulate sounds, and cannot stand or move. At this stage they die from an intercurrent disease.

Along the way, they distinguish a particularly malignant agitated form (galloping paralysis) with sharp motor agitation and disturbance of consciousness of the amentive type and the so-called. stationary paralysis, in which there is a slow course with a gradual decrease in intelligence and lethargy.

Atypical forms of P. p. are youthful and senile P. p., as well as Lissauer’s palsy and taboparalysis (see). Juvenile P. p. develops on the basis of congenital syphilis; usually begins at the age of 10-15 years. Sometimes it is preceded by signs of congenital syphilis, in other cases it occurs in children who were previously considered healthy.

Most often occurs in the form of dementia; Local symptoms are often observed, for example, optic nerve atrophy. Senile P. p. occurs over the age of 60 years and is characterized, first of all, by a long latent stage (up to 40 years). Wedge, the picture reminds me senile dementia(see) with severe memory disorders; sometimes the disease occurs like Korsakov's syndrome (see).

Lissauer's palsy and taboparalysis are characterized by a relatively slow progression of dementia. With Lissauer's palsy, there is a tendency for local brain damage, predominantly parietal lobes, while aphasia (see), agnosia (see), apraxia (see), apoplectiform and epileptiform seizures develop.

Rest tremor is not typical for progressive supranuclear palsy.

All symptoms of progressive supranuclear palsy are combined into several groups:

• Oculomotor disorders in the form of gaze paralysis and a number of other symptoms, accompanied by retraction (lifting) of the upper eyelids with the formation of a characteristic “surprised” facial expression.
• Parkinsonism (akinetic-rigid form). Moreover, extrapyramidal disorders in the classic course of progressive supranuclear palsy have a number of features that allow for correct differential diagnosis. Characterized by the predominance of rigidity of the muscles of the neck and shoulder girdle with the formation of a characteristic “proud” posture, bradykinesia (slowness of movements), symmetry of disorders even in the initial stages, and the early appearance of postural instability. These extrapyramidal symptoms are not corrected by antiparsinsonian drugs. Not typical rest tremor, falls, obvious autonomic and pelvic disorders.
• Walking disorders, usually of the subcortical astasia type with a pronounced influence of postural instability. At the same time, the length of the step, the area of ​​support and the initiation of movements do not initially change; friendly movements arms and legs. People with progressive supranuclear palsy, already in the early stages of the disease, easily lose stability when turning, changing speed, pushing, or walking on an inclined surface. During the first year of the disease, falls backward occur, without attempts to maintain balance.
• Cognitive impairment, with fairly rapid development of dementia of the frontal-subcortical type. Speech becomes impoverished, the ability to abstract and generalize is lost, apathy, field behavior, low speech activity, and echopraxia are characteristic.
• Pseudobulbar syndrome due to lesion frontal cortex and the regulatory pathways coming from it. Dysarthria (unclear sound pronunciation), dysphagia (swallowing disorders, with a preserved or even increased pharyngeal reflex), symptoms of oral automatism, forced laughter and crying develop early.

Progressive supranuclear palsy is not characterized by illusions, hallucinatory-delusional syndrome, qualitative and quantitative violations consciousness, vivid affective disorders.

Atypical clinical forms of progressive supranuclear palsy are also possible: with a predominance of parkinsonism and the appearance of asymmetrical dystonia of the limbs, with a debut in the form of rapidly increasing cognitive impairment, with a predominance of primary progressive aphasia.

Prognosis and prevention

With supranuclear palsy, there is a non-stop progression of symptoms. The therapy provided does not have a significant effect on the course of the disease. The life expectancy of patients ranges from 5-15 years. Death caused by intercurrent infections, prolonged sleep apnea, aspiration pneumonia.

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.