home→Diagnostics→ Progressive paralysis - stages, symptoms and treatment of pathology. Tiganov A.S.

Progressive paralysis - stages, symptoms and treatment of pathology. Tiganov A.S.

Against the backdrop of a sharp decline in the incidence of such a terrible disease as syphilis, progressive paralysis is not so common today.

Usually, the pathology develops about ten to fifteen years after the person has become infected.

Syphilis of the brain is characterized by damage to the membranes and blood vessels. Progressive paralysis of the brain is different in that in this case, the brain parenchyma is primarily affected. In this abnormal condition, severe inflammatory processes appear in the vessels and membranes of the brain.

common data

To a greater extent, progressive paralysis affects males who have crossed the forty-year milestone. An important role in the origin of this pathological condition belongs:

brain injuries;
infectious pathologies;
alcohol intoxication (chronic).

According to doctors, people who have not received timely treatment or who have made a mistake in the selection and administration of medicines are subject to the development of this disease.

Features of the course of the disease

Progressive cerebral palsy is a complex and dangerous disease. Physicians who are actively involved in this problem believe that the pathology develops gradually. In total, three periods of progressive paralysis of the brain are known.

First period

The initial symptomatology of a dangerous disease is the presence of neurasthenic complaints. The person becomes hyper-irritable, distracted and forgetful. He suffers from sleep disorders and decreased performance. Often, causeless tears appear in the eyes of the patient.

The difference between progressive paralysis of the brain and neurasthenic manifestations is the increase in unpleasant symptoms even after rest.

The key neurological symptom of this pathological condition is dysarthria. A person "stumbles" on words that are difficult for him to pronounce.

An equally important symptom is dysgraphia. The handwriting of the patient is transformed into real scribbles. In addition, he often misses both letters and entire syllables.

The duration of the first period can be either a few weeks or two or three months.

Second period

This period of progressive paralysis of the brain is characterized by the development of symptoms of the disease. Signs of pronounced dementia come to the fore in this period. So, doctors say:

Lack of ability to critically assess one's own condition (it often seems to a person that he is absolutely healthy).
Euphoria (sometimes there is just a constantly elevated, somewhat excited state).
Doing strange things that attract the attention of others.

Human behavior becomes somewhat eccentric. This can be expressed both in promiscuity in sexual relations, and in the manner of dressing.

An important symptom in this period is physical exhaustion.

Third period

The word "insanity" has become so firmly established in the lexicon of modern man that he rarely attaches importance to it. And the true meaning of this word is terrible: insanity, or the stage of decay, is the third period of progressive paralysis of the brain. When the disease is transformed into this stage, dementia reaches terrifying degrees.

So, a person turns out to be incapable of articulate speech and utters only a set of strange sounds. He also cannot move and serve himself independently, since his limbs are paralyzed. The result of paralysis of the sphincters is the involuntary release of feces and urine.

The patient is extremely emaciated. Trophic manifestations and bedsores are formed on the skin. A person turns into a living skeleton, and the bones, becoming very brittle, are often injured.

The disease develops into this stage about five years after the onset. In the third period, the disease is considered incurable.

How to recognize

Many people are interested in the question of features mental disorders with progressive paralysis. Unfortunately, the presence of certain mental disorders is not the reason for a person to see a doctor. And it often seems to him and his relatives that all "eccentricities" are temporary and do not pose any danger. The following manifestations of a neurological nature help to identify progressive cerebral palsy:

constriction of the pupils;
irregular pupil shape;
lack of response to light stimulus;
the presence of epileptic seizures;
tendon reflex disorder.

Help the sick

In order to cure progressive paralysis of the brain, the doctor prescribes the passage of a special antibiotic therapy. Treatment lasts for eight courses. Antibiotic drugs are combined with biquinol.

A few years ago, a 3-day malaria vaccination method was used to destroy the syphilis pathogen. Today, the doctor prefers the use of pyrogenal or sulfozine to "old" drugs.

The sooner a person sees a doctor, and the more correct the treatment is, the higher his chances of survival.

Syphilitic infection, as you know, 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 title of a poem by the Italian doctor J. Fracastoro « syphilis Sive De Morbo Gallico» - "syphilis, or French disease", 1530) of the brain occurs most often 2 to 4 years after infection, it is classified as an early form of neurolues, and progressive paralysis is a late one. With early neurolues, the tissue of mesodermal origin (vessels, membranes) is primarily affected, with late (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 designate contagious infections; in the Middle Ages in France, syphilis was called the "Italian disease", and in Italy - the "French disease". Subsequently, it was noted that syphilitic psychosis developed only in 5-7% of those infected. The introduction of antibiotics for the treatment of 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 the incidence, the incidence rate has increased by 3-4 times.

Cerebral syphilis and pp are progressive diseases and occur, as a rule, in cases of untreated or undertreated disease. As a predisposition factor, brain injuries and alcoholism are noted.

Syphilis of the brain (lues cerebri)

Syphilis of the brain (meningovascular syphilis) - specific inflammatory disease with a primary lesion of the 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 in nonspecific vascular diseases brain.

The onset of the disease is gradual, with the onset of symptoms characteristic of neuroses, primarily reminiscent of. Patients develop irritability, headaches, increased fatigue, reduced performance. In a special study of various forms of mental activity, one can usually detect their unsharp decrease. Neurological examination reveals mild signs of stigmatization: anisocoria with sluggish pupillary response to light, asymmetry of facial muscles, uneven tendon reflexes, and their increase. In contrast to similar symptoms observed in the development of cerebral atherosclerosis, syphilis disease begins at a young age and shows steady progression in the absence of the "flicker" of symptoms typical of vascular disorders.

Against this background, when the meninges are affected, signs of meningism are found, or a picture of typical meningitis develops, which can occur acutely or chronically. In acute cases, 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 characteristic, epileptiform seizures, symptoms of impaired consciousness in the form of stunning, confusion, or delirium may occur. However, more often in the meninges

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

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

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

Initially, focal lesions are unstable, reversible, then they become more numerous and stable. At the same time, extensive neurological symptoms are constantly noted, their diversity is due to the difference in the localization of lesions; paralysis and paresis of the extremities, lesions of the cranial nerves, phenomena, pseudobulbar disorders can develop. Almost all patients have a weakening of the pupillary reaction to light.

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

Some patients develop paroxysmal conditions with clouding of consciousness, mainly by the type of twilight disorder. As the disease progresses and neurological symptoms worsen, dysmnestic dementia progresses.

gummy the 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 gummy form is characterized by signs of increased intracranial pressure with vomiting, excruciating headaches, adynamia, sometimes clouding of consciousness, convulsive paroxysms can develop. When examining the fundus, congestive nipples of the optic nerves can be observed.

syphilitic plauta refers to the exogenous type of reactions, according to K. Bongeffer. Such manifestations can not immediately be distinguished from, while the syphilitic paranoid of craepelin is characterized by a predominance. Currently, both options are grouped as a hallucinatory-paranoid form of syphilis with the manifestation of deceptions of feelings and the emergence of delusional ideas, with delusional symptoms predominating. More often observed or self-blame. Crazy ideas are simple, associated with the immediate environment of the patient, 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 the nosological trend in psychiatry. Much later a. Wasserman (1883) determined the presence of a spirochete in the blood, and x. Noguchi (1913) found 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 after four to five years leads to the development of insanity and death.

According to paragraph b. Posviansky (1954), the incidence 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.

The frequency of progressive paralysis, according to a. c. Kosov (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 up of the disease is very accurately described by Mr. Schüle: “silently and quietly, sharply different from the tragic course and finale, the beginning of the disease comes. Until now, a person who is industrious and true to his word begins to cope with his affairs somewhat worse, ordinary things are more difficult for him, his excellent memory begins to stumble, mainly in things that until now belonged to the most ordinary for him, the most familiar. But who would suspect anything special in this? The behavior of the patient is the same as before. His character has not changed, his wit has not suffered. Nevertheless, some change occurred with the patient. His mood is not what it used to be. The patient is neither sullen nor excited, he still expresses his former sympathies and inclinations, but he has become more irritable. The slightest trifle can drive him crazy, and, moreover, with such a temper that he had never been noticed before, he can forget himself to such an extent that he gives free rein to his hands, while before he had an excellent command of feelings and words.

Such symptoms resemble manifestations, along with irritability, increased fatigue, forgetfulness, decreased performance, and sleep disturbance are noted. Nevertheless, it is impossible not to notice that such pseudo-neurasthenic symptoms are combined with various progressive personality changes. Patients show indifference to their family members, lose their sensitivity, delicacy, they show previously uncharacteristic carelessness, extravagance, lose their shame, and, to the surprise of acquaintances, unexpectedly use obscene language.

In the next stage of the full development of progressive paralysis, the main symptom of the disease, dementia, comes to the fore, pronounced memory disorders, the ability to memorize become obvious, weakness of judgment, loss of criticism are found. The external manifestations of the disease at this time may 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 It is considered classical, manifested by manic excitement with magnificent delirium of grandeur of an absurdly grandiose nature. The mood of patients is elevated, it is either euphorically complacent, sometimes accompanied by a feeling of happiness, sometimes agitation and anger. Patients express magnificent, absurd ideas of greatness, incredible in their meaninglessness, which are in absolute contradiction with the real state of affairs. A complete loss of criticism, inadequate excitement, disinhibition of drives are found.

Euphoric form call such cases in which dementia of a 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 excitation 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.).

Dement (simple) form- the most frequent, it is characterized by progressive dementia, complacency in the absence of vivid mental symptoms and a relatively slow course.

agitated form characterized by a state of incessant senseless excitement with confusion, malignancy of the course, 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 in transplacental infection of the fetus from a sick mother. This type of disease is now extremely rare. In such cases, as a rule, there are other signs of congenital syphilis - parenchymal keratitis, deformation of the anterior

Teeth, inner ear lesions (Hatchinson's triad). Paralytic disorders are often associated with symptoms of juvenile dorsal tabes. Juvenile paralysis manifests no earlier than six years, most often in the period from 10 to 15 years. It may be preceded by a delay mental development, but sometimes the disease begins as if in the midst of full health. Perhaps an 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 features of psychopathology, but also relies on data from neurological symptoms, somatic disorders and laboratory tests. In most patients, the Argyle-Robertson symptom is determined with a weakening or absence of pupillary response to light while maintaining their response to convergence and accommodation. The absolute absence of pupillary reaction, narrowing (miosis) or dilation (mydriasis) of the pupils, in some cases their unevenness (anisocoria) and deformation are much less common. to frequent and early symptoms include dysarthria, slurring, or chanting of speech. Approximately 60% of cases of progressive paralysis develop signs of syphilitic damage to the aorta. Frequent bone fractures are due to a combination with dorsal tabes.

Data from laboratory studies. Serological tests for syphilis (eg Wassermann test) 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 are being used - the immobilization reaction of pale treponema (ribt), the immunofluorescence reaction (reef). Characterized by an increase in the number of cells in the cerebrospinal fluid (pleocytosis), mainly lymphocytes, but there is also an increase in plasma cells. All globulin reactions (none-appelt, pandy, 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 drastically changed due to an increase in globulins. The Lange reaction exhibits a "paralytic curve" with a maximum dropout in the first tubes.

Etiology and pathogenesis. The syphilitic etiology of progressive paralysis is proven clinically and laboratory. Japanese x. Noguchi (1913) found pale treponemas in the brain of patients with progressive paralysis. However, the pathogenesis of the disease itself remains unclear. Only about 5% of people who contract syphilis suffer from progressive paralysis. Predisposing factors include hereditary burden, alcoholism, skull injuries, etc. Nevertheless, most researchers believe that the absence or insufficiency of treatment can contribute to the development of the disease.

Differential Diagnosis

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

Taking into account the non-specificity of “pseudo-neurasthenic” manifestations in the debut, if signs of even a mild decrease in the level of personality according to the organic type, epileptiform paroxysms, transient apoplectiform states are found, the beginning progressive paralysis should be excluded. In such cases, it is necessary to conduct a thorough neurological, somatic, serological study. Difficulties may arise when delimiting progressive paralysis from cerebral vascular pathology (atherosclerosis, hypertonic disease) as well as from . In such cases, the data of neurological and serological studies become diagnostic help.

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 1940s, penicillin therapy has become the main method of therapy. Its effectiveness depends on the severity clinical manifestations disease and treatment initiation. Good quality remissions develop in at least 50% of cases. The mental state on the background of penicillin therapy improves after three to four weeks, blood sanitation can be completed in a period of two to five years. The course of treatment requires an average of 14 million units of penicillin. It is desirable to use a depot drug. It is recommended to conduct 6-8 courses of penicillin therapy with an interval of one to two months. With intolerance to penicillin, erythromycin can be used 5 times a day for 300 000 units in combination with biyoquinol or bismoverol courses. In treated patients, there are states of stationary dementia, chronic expansive states, psychotic variants of the defect (p. b. Posvyansky, 1954). After therapy, a control study of the cerebrospinal fluid is indicated to diagnose a possible relapse. An indicator of the stability of remission is evidence-based sanitation of the cerebrospinal fluid for at least two years.

Neurosyphilis is an infectious syphilitic lesion of the central nervous system human, caused by pale treponema.

The causative agent is pale treponema (or pale spirochete), which has a spiral structure, like a corkscrew, penetrates through microcracks in the skin or mucous membrane of a person, entering the blood or lymph, and then into the brain, affecting the nervous system.

Infection often occurs during unprotected intercourse with a sick partner, and it can also occur in the household way (through wet towels, dishes), but these cases are rare.

The reasons for the development of human neurosyphilis are considered incorrect or untimely treatment of syphilis, numerous craniocerebral injuries, disorders immune system, hereditary predisposition and more.

Currently, there is a decrease in cases of neurosyphilis, perhaps this is due to a decrease in the neurotropism of pale treponema, a change in the pathogenic properties of the causative agent of the disease.

Classification of neurosyphilis

1. Asymptomatic neurosyphilis

2. Meningovascular neurosyphilis (the membranes and vessels of the brain are affected)

cerebral (meningoencephalitis, cerebral infarction)
spinal (meningomyelitis, spinal cord infarction)

3. Parenchymal neurosyphilis (affects the tissues of the brain or spinal cord)

progressive paralysis
taboparalysis
dorsal tabes
optic nerve atrophy

4. Hummous neurosyphilis (the appearance of specific formations - gumm)

Neurosyphilis divided into asymptomatic (hidden), early and late forms. The latent form is asymptomatic, pathological changes found only in the cerebrospinal fluid of patients (lymphocytic pleocytosis, an increase in protein content by 2-3 times due to globulins).

Early neurosyphilis occurs during the first 3-5 years after infection (more often in the secondary period of syphilis). Such forms are quite rare, due to modern treatment, but they do exist. Mental symptoms in early forms of neurosyphilis are similar to mental symptoms in encephalitis, meningitis, vascular and tumor lesions, syphilitic neurasthenia, neuritis and polyneuritis occur. They are associated with damage to the membranes of the brain and blood vessels.

Late forms of neurosyphilis appear after 5-10-15 years from the onset of the disease (this is the tertiary period of syphilis), there are cases of manifestation of the disease 20 years after infection. These include: dorsal tabes, syphilitic gumma and progressive paralysis.

progressive paralysis - this is meningoencephalitis (inflammation of the membranes and substance of the brain), leading to a violation of cognitive functions (memory, thinking), a change in personality until the total collapse of mental activity, or dementia.

This form of neurosyphilis is associated with the direct penetration of the pathogen into the brain cells and their subsequent destruction. Progressive paralysis occurs in men 3-5 times more often than in women. The average age of the diseased is over 40 years old, it can also occur in old age.

The puffy, mask-like face of the patient is noteworthy, with a characteristic asymmetry of the nasolabial folds, deviation of the tongue to the side, fibrillar twitches of the perioral muscles.

One of the early symptoms of progressive paralysis is dysarthria. At first, it appears when pronouncing difficult words, later it turns into fuzzy, slurred, chanted speech.

In half of the cases of progressive paralysis, neurological symptoms are characteristic: an increase or decrease in pain sensitivity in the Gitzig zones (the zone of innervation of the first branch trigeminal nerve) - area of the nipples, navel, inguinal folds, increased and uneven tendon reflexes, impaired coordination of movements, tremor, change in handwriting, decreased muscle tone.

There are epileptiform seizures, sometimes serial. Occasionally, visual disturbances are noted - Argill-Robertson syndrome: the patient's pupils acquire an uneven shape and react poorly to light. Psychotic symptoms such as manic-depressive states, delusions, or hallucinations may be observed. Often a combination of progressive paralysis and dorsal tabes.

Stages of progressive paralysis

Often the disease begins gradually, imperceptibly, so patients seek help at severe stages, when the cure is difficult.

1. Initial (neurasthenic) stage. Typical complaints about headache, irritability, fatigue, sleep disturbance, nervousness, decreased performance and so on. Nonspecific neurotic symptoms are often combined with gross emotional disturbances of an organic nature: patients become angry, quick-tempered or, conversely, indifferent to the people around them, lose their moral attitudes, shame.

In other cases, there are cases of rapidly increasing decline in intelligence, memory. Patients may appear deafened, inattentive, have poor understanding of the meaning of what is read or said, respond inappropriately, memory for past and current events suffers. Criticism of one's condition decreases, the level of judgments decreases, unreasonable optimism can be observed. Already at the beginning of progressive paralysis, psychotic phenomena can be observed: manic-depressive states, catatonic excitement or stupor, hypochondriacal disorders.

2. Stage of advanced disease (paralytic dementia). There is a further loss of individual personality traits with a complete loss of criticism of one's condition. Patients are in a benevolent euphoria or dull emotional indifference, there is a disinhibition of drives, behavioral disorders. Intellectual decline continues up to the loss of the simplest forms. The neurological and mental disorders described above are found.

3. Initial stage (marasmus stage) occurs at different times, on average after 2-5 years from the onset of the disease. Complete breakdown of mental activity, physical helplessness,, deep - signs of imminent death of patients. Thanks to modern methods treatment, the disease usually does not reach this stage, but remains at the level of paralytic dementia.

Diagnosis of neurosyphilis

The diagnosis of neurosyphilis can be made on the basis of 3 main criteria:

clinical picture of the disease
diagnostic serological samples
examination of the patient's cerebrospinal fluid

The patient should be examined by a neurologist and ophthalmologist to confirm associated neurological and visual impairment. Already in the early stages of the disease, memory and intelligence disorders appear, which is confirmed by psychological tests. (clock drawing test, ).

For diagnostic purposes, serological samples of the patient's blood or cerebrospinal fluid are carried out (Wasserman reaction - RW, RPR, ELISA, RIBT, RPHA).

RPR (Rapid Plasma Reagin) - an anticardiolipin test-screening non-treponemal test that detects antibodies (reagins) of the Ig G and Ig M classes to lipoid and lipoprotein-like material released from damaged cells of a patient with syphilis. This test is a modern analogue Wasserman reactions (RW). In most cases, a positive RPR - blood reaction is observed 7-10 days after the appearance of the primary chancre or 3-5 weeks after infection. The diagnostic value of the sample decreases after the onset of the secondary stage of syphilis. 30% of patients with late syphilis do not respond to the RPR test. Thus, a blood test better detects early forms of syphilis, up to 5 years from the initial infection. To detect late forms of syphilis, the cerebrospinal fluid (CSF) is examined. positive reaction Wasserman in cerebrospinal fluid with progressive paralysis reaches 100%.

This test is not specific because in some autoimmune diseases, diabetes, people who use drugs may experience false positive results. Therefore, the most correct will be the simultaneous appointment of RPR - test with ELISA - analysis.

ELISA (enzymatic immunoassay) detects Syphilis Ig M and Ig G antibodies in the blood of patients. The results of ELISA allow laboratory confirmation of the diagnosis of syphilis from the end of the 3rd week from the moment of infection.

Treponema pallidum immobilization reaction (RIBT)is the most specific serological test. Starting from the second stage of syphilis, RIBT is positive in all patients. This test also detects late forms of syphilis, over 5 years from the onset of infection.

Passive hemagglutination reaction (RPHA)- a test based on the determination of specific antibodies to pale treponema.

To detect neurosyphilis in the absence of symptoms of compression of the brain, the patient undergoes a lumbar puncture and a study of the cerebrospinal fluid. Characteristic features of the cerebrospinal fluid are the presence of pale treponema, lymphocytic pleocytosis, an increase in the protein content over 0.6 g / l).

Usage (MRI or CT) indirectly confirms the diagnosis of neurosyphilis, hydrocephalus, expansion of the subarachnoid spaces, multifocal lesions of the white matter of the brain, its atrophy are noted, and also serves to exclude other diseases.

Treatment of neurosyphilis

Currently, penicillin therapy is used to treat syphilis. Penicillin penetrates the blood-brain barrier well, especially when intravenous administration drug, which allows to achieve a successful cure for the patient.

Benzipenicillin sodium salt is injected intravenously at a dose of 10-12 million units 2 times a day for 14-20 days, then a second course is carried out.

Penicillin is administered intravenously in a stream of 2-4 million units 6 times a day (daily dose of 12-24 million units) for 14-20 days.

In case of intolerance, allergy to penicillin, ceftriaxone is used intramuscularly or intravenously in the treatment daily dose from 1 g to 4 g for 14-20 days.

The first injection of the antibiotic is administered intramuscularly to avoid an adverse Jarisch-Herxheimer reaction due to the breakdown of a large number of pale treponemas. It is manifested by acute fever, headaches, chills, decreased blood pressure, muscle pain, cramps. Usually, the symptoms disappear within a day against the background of the introduction of glucocorticoids (60 mg of prednisolone) and non-steroidal anti-inflammatory drugs.

Criteria for the cure of neurosyphilis are coagulation neurological symptoms and normalization of the patient's cerebrospinal fluid. Further control of cerebrospinal fluid is carried out once every 6 months, within 3 years after the diagnosis. Persistent normalization of cerebrospinal fluid (even with persistent residual neurological symptoms) is an indication for deregistration by a dermatovenereologist.

Video "Neurosyphilis"

Progressive paralysis (synonymous with Bayle's disease) is 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, 10 to 15 years after contracting syphilis. Initial symptoms progressive paralysis, lasting from 1 to 6 months, are manifested by asthenia (see Asthenic syndrome) or. These disorders are always combined with memory loss, complaints of headaches, dizziness, lethargy, indifference to loved ones and their duties, irritability. There is no criticism of their wrong actions, physical and especially mental state. In some cases, only the symptoms of total dementia increase (see) - the so-called simple, or dementia, form of progressive paralysis. In others, lasting months, at most a year, develop psychoses: 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 the delusional statements of patients an absurd character (see also). In the future, the symptoms of psychosis disappear and the growing one comes to the fore.

Neurological disorders always exist and are manifested by dysarthric slurred speech, Argyle Robertson's symptom (see), unsteadiness of gait, inaccuracy of movements, decrease or absence of knee and Achilles reflexes, reversible mono- or hemiparesis.

Of the somatic disorders, especially in advanced cases, there are phenomena of cachexia (see), bone fragility, paralysis of sphincters. The Wasserman reaction is in the blood and is almost always positive; the number of cellular elements is increased, the amount of protein is increased. The combination of progressive paralysis and dorsal tabes (see) is called taboparalysis.

Treatment: Compulsory and urgent admission to a hospital where malaria treatment and antibiotics are administered. With early treatment, recovery can be achieved.

Progressive paralysis (synonymous with Bayle's disease) is a parenchymal form of neurosyphilis, a later syphilitic brain lesion.

Progressive paralysis usually occurs 10-15 years after infection with syphilis, more often at 40-50 years old, but it also occurs in younger individuals. In the past, progressive paralysis was a relatively common disease. Since the 1930s, the incidence of progressive paralysis, both in the USSR and abroad, has been declining due to the success of the prevention and treatment of syphilis.

Pathologically progressive paralysis is characterized by the phenomena of chronic leptomeningitis, granular ependymatitis of the cerebral ventricles, internal and external hydrocephalus, and atrophy of the cerebral convolutions. In the initial stage of progressive paralysis, inflammatory phenomena are noted; later dystrophic changes in the brain tissue join.

The clinical picture of progressive paralysis combines symptoms of psychopathological, neurological and somatic disorders. According to the degree of clinical manifestations, three stages can be distinguished during progressive paralysis: “neurasthenic” (initial), advanced manifestations of the disease and marantic.

In the initial stage of progressive paralysis, the 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 unmotivated mood swings from euphoria to outbursts of irritability or weakness reactions are revealed. Ridiculous delusional ideas of greatness are often observed: patients consider themselves the owners of huge sums of money, innumerable treasures, rulers of the world, etc. A depressed mood is less often noted. 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 ties. Patients make pointless purchases, spend money, regardless of the material resources of the family. Gradually, violations of memory and counting increase, it becomes impossible to assimilate a new one, and former knowledge and skills are lost. The possibility of abstract thinking is reduced. Patients show an inability to adequately assess the situation and regulate their behavior. There is no consciousness of illness. Due to the appearance of suggestibility and the weakening of moral delays, 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 stupefaction often aggravate the impression of the depth of dementia.

In the third, marantic, stage of progressive paralysis, which occurs in the absence of treatment 2-3 years after the onset of the disease, there are phenomena of profound dementia, a sharp exhaustion of patients; they become untidy, they develop bedsores.

Several forms of progressive paralysis have been described. The most common is the simple dementia form. Other forms are distinguished depending on the predominance of additional symptoms (delusions of grandeur, epileptiform or apoplektiform seizures, arousal, delusions of persecution, damage). Accordingly, they are divided into expansive, seizure, agitated, paranoid, depressive forms. These forms are not stable and may change over the course of the illness. In addition, there are also some atypical forms: juvenile progressive paralysis, which develops 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; Lissauer's paralysis, characterized by a predominant lesion of the posterolateral parts of the brain, according to which there are clinical focal symptoms (aphasia, apraxia, agnosia); Korsakov's 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 postwar years, malignant current forms of progressive paralysis with mild specific serological changes are described; these forms arise 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 Argyle Robertson syndrome (see Argyle Robertson syndrome). Pupils are usually sharply narrowed (miosis). Carry disturbances of the speech of type so-called dysarthrias to early symptoms (see). When writing, there are frequent omissions of syllables or words, trembling handwriting. There is a mask-like face characteristic of progressive paralysis, asymmetry of the nasolabial folds. Frequent tabetic symptoms (and not only in cases of taboparalysis) - the absence of patellar and Achilles reflexes, impaired sensitivity and coordination of movements. The reactions of Wasserman, Sachs - Vitebsky and Kahn in the blood in almost 100% of cases give a positive result.

In the cerebrospinal fluid, the Wassermann reaction is positive in 100% of cases, there is an increase in the amount of protein up to 1.0‰ and higher, positive or sharply positive protein reactions (Nonne - Appelt, Pandey, Weichbrodt), cytosis - several tens of cells in 1 mm 3. The Lange reaction curve for progressive paralysis graphically looks like a specific for progressive paralysis (the so-called paralytic) tooth, and in the digital designation - 66655432100. All these serological changes are so typical of untreated progressive paralysis 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 nevertheless differs from neurasthenia by the rapid progression of all painful manifestations and the presence of euphoria, Argyle Robertson syndrome, and characteristic serological changes. From the pseudo-paralytic (most similar to progressive paralysis) form of cerebral syphilis, progressive paralysis can be distinguished on the basis of signs of profound dementia, characteristic neurological and serological changes, a faster increase in all painful manifestations than with cerebral syphilis. Delimitation of progressive paralysis from frontal localization of a brain tumor sometimes similar in clinical picture is possible with a detailed clinical examination based on congestive nipples, a negative Wasserman reaction, and the presence of protein-cell dissociation in the cerebrospinal fluid.

The prognosis of progressive paralysis is better when treatment is started early in the course of the disease.

Treatment. For therapeutic purposes, the patient is vaccinated with the causative agent of three-day malaria. After 10-12 attacks, quinine and other antimalarials are prescribed (see Malaria, treatment). Other pyrogenic agents are also used (2% suspension of sulfur, 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; in the future, the dose is increased by 0.2 to 1 ml and above (depending on the reaction); the course of treatment is carried out 10-12 injections at intervals of 2-4 days. The domestic drug pyrogenal is prescribed as intramuscular injections into the upper outer quadrant of the buttock in doses of 10-20-30 mcg with a further gradual increase in dose to 80-150 mcg. The course of treatment is 8-12 injections at intervals of 2-3 days. Therapeutic effect 2% suspension of sulfur and pyrogenal is less than malaria therapy. After the end 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 schemes (see Syphilis).

The mental and physical condition sometimes improves immediately after the course of pyrotherapy, but more often gradually, over several months. As a result of successful treatment, mental disorders are smoothed out (including manifestations of dementia), personality is restored. It is also possible to smooth out some neurological symptoms. The cerebrospinal fluid and blood are sanitized more slowly (from 1/2 to 3-4 years). After 1/2 year - 1 year after the infectious and specific therapy, a control study of the cerebrospinal fluid and blood should be carried out. In the absence of any clinical and serological effect, repeated pyrogenic therapy should be resorted to, and a change in infection or the use of another pyrogenic agent is recommended.

The frequency of good remissions (complete recovery with minor changes in personality) reaches 20%. Recovery with a defect and incomplete ability to work is noted in 30-40% of cases, in the rest - an unstable improvement or lack of effect.

One of the first cases of undoubted progressive paralysis was described by J. Esquirol. In 1822, A. Bayle singled out Progressive paralysis as an independent disease, emphasizing that it causes both mental disorders and neurological disorders associated with chronic inflammation arachnoid membrane of the brain. Bayle's statement met with objections from some researchers. And only in the 50s of the 19th century progressive paralysis was recognized as an independent disease.

Progressive paralysis affects about 5% of all patients with syphilis (see the complete body of knowledge), and women get sick much less often than men. The disease usually occurs 10-15 years or more after infection. Thus, if we take into account that the period of greatest sexual activity falls by 20-35 years, the age of the diseased is most often 35-50 years. When infected with syphilis at a late age (45-60 years), this interval is usually shortened.

clinical picture. Progressive paralysis is a serious disease of the whole organism, and its most striking manifestations are mental disorders. The main syndrome is progredient total dementia (see the full body of knowledge): the intellect suffers rudely, judgment disorders are indicated early, criticism and especially self-criticism disappear. There is no consciousness of the disease, memory is sharply reduced, confabulations occur (see the full body of knowledge: Confabulosis). Manifestations of dementia are intensified due to the often observed euphoria (see the full body of knowledge: Psycho-organic syndrome). Neurological symptoms are speech disorders, primarily in violation of articulation - dysarthria (see full body of knowledge). Speech becomes unclear, 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 fall out of words. The timbre of the voice changes, it becomes deaf. The patient's face is inexpressive, mask-like, since the innervation of the muscles of the face is disturbed, blepharoptosis occurs (see the full body of knowledge: Ptosis). Tendon reflexes are often elevated and uneven, except in cases of taboparalysis (see full body of knowledge). In connection with disorders of the sphincters, patients do not retain urine and feces. As a result of vasomotor and trophic disorders, edema, bone fragility, muscle atrophy, pressure ulcers appear.

On the basis of psychopathological manifestations, four stages of the disease are distinguished: latent (from infection with syphilis to manifestations of Progressive paralysis), the stage of initial manifestations, the stage of the full development of the disease and the stage of insanity (see the full body of knowledge). In the latent stage, headaches, dizziness, fainting, in some cases there are characteristic changes in the cerebrospinal fluid (see the complete body of knowledge). The stage of initial manifestations is characterized by increased fatigue, irritability, weakness of mind. Patients complain of a breakdown and decreased efficiency, although they can still do their usual work to some extent. Previously, such conditions, due to their external resemblance to neurotic symptoms, were incorrectly called pre-paralytic neurasthenia. In some cases, in the stage of initial manifestations, depressive and delusional disorders are observed - anxious depression with hypochondriacal statements, anxiety-agitated depression, delusional ideas of jealousy, persecution, poisoning; as the symptoms of dementia increase, these endoform disorders disappear. Memory impairment is detected very early. Separate actions indicate a violation of criticism. The sphere of inclinations is upset, patients become gluttonous, erotic. The growth of these disorders indicates the transition of the disease to the stage of full development, which manifests itself in various clinical forms. The expansive, or classic, form (previously common) is more common in men. It is characterized by the presence of manic excitement with the manifestation of anger, grandiose delusions of grandeur (see the full body of knowledge: Delusions). 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 the full body of knowledge: Depressive syndromes), ridiculous hypochondriacal delusions of nihilistic content are often observed. The circular form, first described by S. S. Korsakov, proceeds with a change in states of excitement and depression. The hallucinatory-paranoid form is characterized by the development of a paranoid syndrome (see the complete body of knowledge) with predominantly auditory hallucinations and delusions of persecution. In the catatonic form, a stuporous state occurs (see the full body of knowledge) with the phenomena of mutism and negativism (see the full body of knowledge: Catatonic syndrome). In the stage of insanity, conscious activity ceases, speech disappears, patients make inarticulate sounds, cannot stand and move. At this stage, they die from an intercurrent disease.

Along the course, a particularly malignant agitated form (galloping paralysis) with a sharp motor excitation and impairment of consciousness according to the amental type and the so-called stationary paralysis are distinguished, in which there is a slow course with a gradual decrease in intelligence and lethargy.

Atypical forms of Progressive paralysis are juvenile and senile Progressive paralysis, as well as Lissauer's palsy and taboparalysis (see full body of knowledge). Juvenile Progressive Paralysis 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, at other times it occurs in children who were previously considered healthy. Most often occurs in dementia form; local symptoms are often observed, for example, atrophy of the optic nerves. Senile Progressive paralysis occurs over the age of 60 and is characterized primarily by a long latent stage (up to 40 years). Clinical, the picture resembles senile dementia (see full body of knowledge) with severe memory disorders; sometimes the disease proceeds according to the type of Korsakov's syndrome (see the full body of knowledge).

Lissauer's paralysis and taboparalysis are characterized by a relatively slow increase in dementia. With Lissauer's palsy, there is a tendency to local damage to the brain, mainly parietal lobes, while developing aphasia (see the full body of knowledge), agnosia (see the full body of knowledge), apraxia (see the full body of knowledge), apoplectiform and epileptiform seizures. Taboparalysis is a combination of the symptoms of Progressive palsy and tacis dorsalis (see full body of knowledge), with spinal disorders preceding the development of symptoms of Progressive palsy, usually its dementia form.

Etiology and pathogenesis. In 1913, X. Noguchi proved the syphilitic etiology of Progressive paralysis by finding pale treponema in the brain tissues.

The pathogenesis is not well understood. K. Levaditi, on the basis of observations when both spouses or several persons infected with syphilis from the same source fell ill with Progressive paralysis, suggested the existence of a special neurotropic treponema, which, however, has not been proven. It is possible that disturbances in the body's reactivity play a role in pathogenesis (see full body of knowledge) with sensitization of the brain tissue, as a result of which, in some cases, treponemas penetrate into the brain tissue.

Pathological anatomy. Macroscopically, with progressive paralysis, atrophy of the gyri of the brain and expansion of the furrows are noted. The soft shell of the brain is clouded, fused with the substance of the brain; ependymatitis is observed (see full body of knowledge: Chorioependymatitis). Microscopically, an inflammatory process is detected in the pia mater of the brain and in the vessels, especially small ones. Around the vessels, infiltration by plasma cells is characteristic. In the brain tissue, desolation of the cortex is observed due to the destruction of nerve cells and fibers. In the neuroglia, glial fibers grow, rod-shaped and amoeboid cells appear. Pale treponemas are found near the nerve cells.

Diagnosis in the stage of full development of the disease usually does not cause difficulties. It is established on the basis of psychopathological, neurological manifestations and laboratory data. The increase in the phenomena of total dementia with a lack of criticism, dysarthria, impaired pupillary reactions (see the full body of knowledge: Argyll Robertson syndrome), the constancy of serological indicators - all this together makes the diagnosis of Progressive paralysis reliable. The Wasserman reaction in the blood is in most cases positive (see the full body of knowledge: Wasserman reaction); in the cerebrospinal fluid, it, like protein reactions (Nonne - Apelt, Pandey, Weichbrodt), is sharply positive (see the full body of knowledge: Coagulation tests, Cerebrospinal fluid). The number of cellular elements in the cerebrospinal fluid is increased, sometimes significantly. The total protein content is increased. When staging the Lange reaction (see the full body of knowledge: Cerebrospinal fluid), there is a discoloration of the liquid in the first 4-6 test tubes and an increase in color intensity in the following (the so-called paralytic curve). Treponema immobilization reaction (see Nelson-Meyer reaction) and immunofluorescence reaction (see full body of knowledge) are sharply positive in blood and cerebrospinal fluid.

Differential diagnosis is carried out with syphilis of the brain (see the full body of knowledge: Syphilis), in which dementia is lacunar in nature with more or less preservation of criticism, hallucinations are more often observed; the Lange reaction curve has a so-called syphilitic tooth. progressive paralysis

differentiate with alcoholic pseudo-paralysis (see Alcoholic encephalopathies) and senile dementia (see full body of knowledge) on the basis of serological indicators; with brain injuries (see the full body of knowledge), especially the frontal lobe, according to neurological symptoms and serological indicators. Pseudoparalytic syndrome in brain tumors is accompanied by increased intracranial pressure (see full body of knowledge: Hypertensive syndrome). Psychoses of vascular genesis differ from Progressive paralysis by the development of lacunar dementia (see the complete body of knowledge: Atherosclerosis), which is not characteristic of Progressive paralysis The presence of paralytic dementia makes it possible to distinguish the circular form of Progressive paralysis from manic-depressive psychosis (see the full body of knowledge), and the hallucinatory-paranoid form - from schizophrenia (see full body of knowledge).

Treatment. Pyrotherapy is shown (see the full body of knowledge) - infectious therapy and pyrogenic substances (see the full body of knowledge) in combination, as a rule, with antibiotic therapy. Back in 1845, V. F. Sabler noted the beneficial effect of a number of febrile illnesses on the course of psychoses. Priority in the infectious therapy of psychosis belongs to A. S. Rosenblum, who treated the mentally ill with relapsing fever vaccination. In 1917, the Viennese psychiatrist J. Wagner-Jauregg suggested that patients with progressive paralysis be treated with malaria inoculations. The method has become widespread; it consists in injecting subcutaneously into a patient with progressive paralysis blood taken from a patient with three days of malaria. Incubation period lasts 4-20 days, the first attacks proceed according to the type of three-day malaria, in the future, as a rule, they are observed daily. After 10-12 attacks, they are stopped by taking quinine hydrochloride. Inf. therapy is also carried out by infecting patients with progressive paralysis with European and African relapsing fever. The results of this therapy are less pronounced, but it is convenient, since the inoculation material, previously obtained by infecting a mouse, can be transported over long distances. Somatically weakened patients are vaccinated with Japanese rat typhus - sodoku. In cases where infection fails, as well as with somatic contraindications, pyrogenic substances (pyrogenal and others) are prescribed.

Since the 40s, the 20th century has been spent complex treatment- pyrotherapy in combination with the introduction of antibiotics. At the same time, 40,000,000 units of penicillin are prescribed for the course of treatment; repeated courses of penicillin therapy are carried out at intervals of up to 2 months under the control of serological indicators. In some cases, bicillin is used in combination with bismuth preparations. In combination with antibiotic therapy, infectious therapy (malaria inoculation) is recommended, which contributes to the penetration of antibiotics into the brain tissue, and in addition, to an increase in the body's defenses. Inf. therapy is contraindicated in old age, with marasmus, heart failure, aortic aneurysm, kidney disease, diabetes mellitus. In addition, treatment with penicillin alone has been developed. Several regimens for penicillin treatment have been proposed, but some researchers consider the use of penicillin alone insufficient.

Forecast. The duration of the course of untreated progressive paralysis from its initial manifestations to death is on average about 2½ years. Juvenile Progressive paralysis flows more slowly (5-6 years), stationary paralysis - up to 20 years or more. The agitated form ends with the death of the patient in a few months. With the expansive form, long-term remissions are observed. Remissions after treatment have a different duration. Known remissions over 20 years. According to a number of researchers, remissions of more than 2 years are stable.

Prevention consists in the timely treatment of syphilis, which is carried out with specific and non-specific means according to certain schemes (see the full body of knowledge: Syphilis).

Social and forensic psychiatric significance. Only in isolated cases (persistent remission after treatment) can the patient be allowed to return to the previous professional activity. During a forensic psychiatric examination (see the full body of knowledge), the patient is insane in almost all cases (see the full body of knowledge). Only when treated with progressive paralysis with a stable remission lasting at least 3 years, the patient can be recognized as sane.

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