Youth progressive paralysis. Progressive paralysis of the brain: clinical forms, the flow of stages

Etiology and pathogenesis

Syphilitic infection affecting all organs and tissues can spread on the brain. In the nature of the lesions of the tissues, the time of emergence and the peculiarities of mental disorders, two different diseases are distinguished: the syphilis of the brain and progressive paralysis. The syphilis of the brain is usually referring to the early forms of neurolar (with this form, vessels and shells of the brain are affected), and progressive paralysis - to the late (here in the process is involved in the brain substance). This division for early and late forms is based on various morphological changes in brain tissues and does not coincide with the generally accepted division of syphilis for periods.

The syphilis of the brain and progressive paralysis are preventive diseases and occur most often on the basis of an uncutaneous or unrealized syphilis. A large predisposing role in the occurrence of these diseases (especially progressive paralysis) is played by brain injuries, chronic infections and intoxication, of which alcoholism is in the first place.

The syphilis of the brain occurs most often in 5-7 years after infection, although it is possible for a shorter time (from several months to 2-4 years) and longer (10 years and more) the timing of the incubation period. The incubation period of the progressive paralysis is much longer (8-12 years or more), but the earlier appearance of this disease is possible.

Under the generalizing name "syphilis of the brain", various non-verbal disorders associated with a syphilitic lesion of the brain vessels, his shells or, which is much less common, the emergence of gumm. Neriva-mental disorders can also be caused by changes in the brain tissue itself, increasingly due to the spread of the pathological process from the shells on the brain substance (meningoencephalitis), brain lesions due to power impairment, etc.

In almost every case, the diseases in one degree or another there are lesions of both cerebral vessels and shells and the brain substances, but is usually prevailing some one type of violations. The syphilis of the brain is a preventive disease and can lead to a pronounced dismissal (partial) dementary.

Clinical picture

The syphilis of the brain is characterized by a large variety of clinical manifestations, a pronounced polymorphism of neurological and mental disorders, which is often lack of clear boundaries between different forms. Neriva-mental disorders in the defeat of brain shells can manifest itself both with light irritation (phenomenon of meningism) and typical meningitis flowing acutely or chronically. Mental disorders in this case can mimic neurasthenic, as well as occurring during encephalitis, vascular and tumor processes phenomena. The data of neurological and serological studies are crucial in their diagnosis.

Forms of syphilitic disorders

Syphilitic neurasthenia

The so-called syphilitic neurasthenia cannot be considered a separate form, but is essentially a prefabricated concept. Characteristic (especially in the initial stages of the disease) neric-like symptoms in the form of increased irritability, poor sleep, fatigue, anxiety and depression (sometimes to pronounced depression) are explained mainly by two reasons: a person's response to the fact of the disease syphilis and the general intoxication of the body, in particular the head brain. For syphilitic neurasthenia, abundant indefinite sensations (sensenestopathy) are characterized, as well as disorders of consciousness in the form of stunning or its easy degree - revenue, the difficulty of concentration, difficulties in the selection of words.

Epileptiform seizures

Less often, epileptiform seizures are observed, accompanied by fear and amnistic disorders. From the side of neurological status, the sluggish reaction of pupils into light, pronounced hyperesthesia, the positive reaction of the vasserman in the blood and liquor, positive globulin reactions and moderate plea-virocytosis, syphilitic "prong" during the Lange reaction, less often - outlined paralytic or meningeal curves.

Acute syphilic meningitis

Acute syphilitic meningitis develops mainly in the secondary period, is accompanied by general-creation phenomena (headaches, dizziness, vomiting), increased body temperature, typical meningeal symptoms, memory disorders and perception, difficulty in intelligent processes. Characteristic defeat of cranial nerves. Often there are epileptiform convulsions and symptoms of permanent consciousness in the type of stunning, confusion or delirium. Beginning more acute than under progressive paralysis, but the prevention of psycho-organic changes is less, and the preservation of criticism is greater. The course of the disease is wave-like. Neurological and serological changes are approximately the same as in the previous embodiment, the Vasserman reaction may be negative.

However, much more often than acute meningitis, in the brain shell, a chronic inflammatory process is developing, often penetrating and in the brain substance (chronic syphilitic meningitis and meningoencephalitis).

In addition to the pronounced pathology of the cranial nerves, the patients have headaches, irritability, a tendency to affective reactions, often suppressed mood.

Sometimes meningitis develops on the convex surface of the brain. With this localization of the pathological process, the most vivid symptoms are the convulsive states and disturbances. Patients are either stunned or suppressed, or are in a state of confusion and motor excitation. Facial seizures are the character of Jackson, or generalized, seizures. Neurological symptoms for syphilitic meningitis can be represented not only by meningeal symptoms and a variety of pathologies of cranial nerves, but also in such phenomena as Agraphia, apraxia, hemi and monoplegia.

Apoplemic form of the syphilis of the brain

This form meets most often. It develops on the basis of a specific lesion of cerebral vessels. Clinically manifested by frequent strokes with subsequent focal lesions. The first time the focal lesions of the unstable, reversible, then become increasingly multiple, durable and permanent. Extensive neurological disorders can be represented by the most diverse (depending on the localization of the lesion) symptoms: paralycs and lines of limbs, lesion of cranial nerves, apraxia, agnosia, pseudobulbar phenomena, epileptiform seizures, in some cases - Jackson, etc. Almost a constant feature is Weakening of the pupil reaction to the light.

In addition to focal pathology, headaches, dizziness and reduction of memory are very characteristic for this form. Patients become irritable, picky, weak, sometimes angry or depressed. The episodes of permanent consciousness are noted, mainly by type of twilight, verbal hallucinations of commenting and imperative content.

As neurological symptoms are weighted, there is an increasingly distinct increase of dismissal (focal, partial, lacunar) dementia. Dementia is developing close to vascular: the predominance of dismissal disorders and lacunar drops. Within the framework of the pseudo-paralytic dementia, the frontal symptoms are developing: euphoria, ideas of greatness, reduction of criticism, amnistic disorders, disinterested permission. Possible fatal outcome during stroke.

Serological changes in this group of the Pestra and diverse. The reaction of the vasserman in the blood is more often positive, in the lycvore - not always; the increase in cells and globulins moderate; In the reaction of Lange, the syphilitic "prong" is determined or paralytic curves.

Gummime form

In the humming form of syphilis, mental changes depend on the magnitude and localization of the gum. Psycho-organic disorders are characterized with multiple gums and various degrees of permanent of consciousness, focal disorders and exogenous psychosis at large gums, causing an increase in intracranial pressure. Possible strokes and epileptic seizures. Characteristic preciousness of psychoorganic disorders.

In addition to those listed, epileptiform and hallucinatory-paranoid forms of syphilis are also isolated. With a dorsal dry shot, the actual tabetic disorders (the absence of tendon reflexes in the hands and legs, ataxia and sensitivity disturbance) are rare; We are more often observed euphoria and reduction of criticism, the weakening of memory and a decrease in mental activity, less often - depressive and depressive-hypochondriartic states. In the Likvore Plequitosis and an increase in protein content are less pronounced.

Treatment

Treatment of the syphilis of the brain must begin as early as possible. Therapy is made by specific anti-acidic drugs with a comprehensive method according to the scheme. Antibiotics of penicillin rows, macrolides, bismuth and iodine preparations are used. In addition to special treatment, it is shown and a contemplating, especially vitamin therapy.

Progressive paralysis

Progressive paralysis - mental illness, characterized by increasing a variety of paralysis and severe dementia, arises on the basis of the primary damage to the brain itself, although the fabric of mesodermal origin (vessels and shells) suffer. At the same time, we are talking about the appearance and increasing in the process of the disease of complete apeactivity, and therefore the normal automated reactions of the body disappear (cough, swallowing, urination, etc.). Immunobiological reactivity is reduced at the same time.

The classic picture of the progressive paralysis includes three stages: the initial, heyday of the disease and the final (stage of the marasma). For each of these stages, certain mental, neurological and somatic violations are characteristic. Currently, progressive paralysis not only began to meet rarely, but changed his clinical picture. There are practically no patients in a state of paralytic marasm (III stage). The picture of the progressive paralysis was noticeably changed due to the sharp prevailing over all other dementary form. Due to active treatment, progressive paralysis is no longer considered as before, the disease with a mandatory outcome in severe dementia.

Mental violations

initial stage

The initial (neurasthenic) stage of progressive paralysis is characterized by the emergence of neurosis-like symptoms in the form of all increasing headaches, increased fatigue, irritability, poor sleep, reduce performance. In this symptoms, violations are quite soon to be joined, which at first can be characterized as the loss of previous ethical skills. Patients become unulcated, sloppy, rude and neutactic, detect the cynicism inconspicuously with them earlier and a slight attitude towards their duties. Then these personality changes are becoming increasingly brutal and striking - the second stage comes (the period of the heyday).

Disease breakdown period

More increasing memory disorders and weakness of judgments are detected, patients cannot with full criticism to evaluate their condition and the environment, incorrectly behave. Often we find rude sexual successes, completely lose the feeling of shame.

Often there is a tendency to empty spent, patients take money and buy completely random things. Typically appearance of chatty and boastfulness.

The emotionality of the patients changes dramatically. There is a pronounced lability of emotions, patients easily arise outbreaks of irritation up to pronounced ammunition, but these evil flares are usually fragile, and the patient can be quickly switched to something outsuen. Also, these patients are easily moving from tears to laughter and vice versa. The prevailing color of the mood can be quite early appearing euphoria. In some cases, on the contrary, a pronounced depressive state occurs with possible suicidal trends. Perhaps the occurrence of delusional ideas, especially the delight of greatness and one of his varieties - Brad of wealth.

Fast rise in dementia imposes an obvious imprint and on the delusional creativity of patients. Paralytic nonsense is usually distinguished by absurdity and ambitious dimensions. Less often appear the ideas of persecution, hypochondriam, etc., usually also ridiculous content. Occasionally there are hallucinations, mainly auditory.

Over time, a decrease in intelligence becomes increasingly noticeable. The memory is sharply violated, the weakness of judgment and loss of criticism are increasingly discovered. Weakness with progressive paralysis, in contrast to the syphilis of the brain, is diffuse, general.

Maratism Stage

For the third (marasmatic) stage of the disease (to which, as already mentioned, the painful process is now usually not reaching) the most characteristic is deep dementia, full mental insanity (along with physical insanity).

Somathorological disorders

Quite often, the combination of progressive paralysis with syphilitic meal is noted. Specific lesions of the liver, lungs, sometimes skin and mucous membranes may also be marked. Very characteristic general exchange disorders. Possible trophic skin impairment up to the formation of ulcers, increased nail fragility, hair loss, the occurrence of edema. With a good and even increased appetite, there may be sharply progressive exhaustion. The resistance of the body is reduced, in patients with progressive paralysis, intercurrent (associated) diseases are easily arising, in particular purulent processes.

Clinical picture

Characteristic and one of the first neurological signs is argail-robertson syndrome - the absence of a pupil response to light while maintaining it to convergence and accommodation. Other pupil disorders are characterized: a sharp narrowing of the magnitude of the pupils (MIOS), sometimes to the size of the pin head, an anisocorium or deformation of pupils is possible. Often, under progressive paralysis, the asymmetry of nasolabial folds, ptosis, a mask face, deviation of the tongue aside, separate fibrillar twitching muscles of the muscles and circular muscles of the mouth (the so-called Zarnitsa) are also observed. Very typical rather early appearing dysarthria. In addition to the inadvertence and fuzziness of speech, patients can skip individual words or, on the contrary, stuck on a syllable, in many respects repeating it (logoclonium). Often, it becomes a gnusava (Rinolalia).

Early manifestations also include characteristic changes of the handwriting (it becomes uneven, trembling) and violations of coordination of subtle movements. With the course of the disease in the letter of patients, more coarse errors appear in the form of skips or rearrange the syllables, replacing some letters to others, the repetition of the same syllables, etc.

All coordination disorders can become more and more rude. Quite often, changes in tendon reflexes, as well as a more or less pronounced reduction in sensitivity. The appearance of pathological reflexes is possible.

It is not unimaging the innervation of pelvic organs. The most rude neurological symptoms is characteristic of the last, third stage of the disease. Preferably in this stage, apoplexiform seizures are observed, leaving initially smoothing, and then increasingly resistant focal disorders in the form of paresses and paralysis of the limbs, phenomena of aphasia, aproxia, etc.

Non-standard pathology in progressive paralysis are epileptiform seizures, in the third stage of the disease, usually emerging series or in the form of statuses with possible fatal outcome.

Forms of progressive paralysis

Expansive (classical, maniacal) form was described by one of the first not only due to its bright clinical picture, but also to great distribution. Now this form, especially in its pure form, is quite rare. It is characterized by the emergence of a manic state and ridiculous ideas of greatness. Against the background of complacent and euphoric moods, the flashes of anger may sometimes have suddenly, but irritation is usually short-term, and the patient soon becomes euphorical and complacent.

Currently, the dementary form is most common (up to 70% of all cases). The first plan is a bright pattern of general (total) dementia. In the mood, either grateful, or lethargy, indifference to the whole surrounding.

In addition, depressive, agitated, enclosure, circular, paranoid and atypical forms of progressive paralysis are isolated.

In most cases, the Vasserman reaction in the blood and the liquor is positive already during dilution of 0.2. The reactions of immobilization of pale treponia (RIBT and RIT) are used, as well as the immunofluorescence reaction (reef). The reaction of Lange is characteristic.

Untreated paralysis after 2-5 years leads to a complete marasmus and a fatal outcome. Especially rapidly the phenomena of the general maratism increases with the so-called galopulating paralysis characterized by the catastrophic flow of the disease. Most often it happens with an accounted form. Plotted form is very unpleasant.

The forecast (both in relation to life and the further flow of illness and recovery) is determined, as a rule, the results of the treatment that must be started as early as possible.

Treatment of progressive paralysis is to apply combined specific therapy: antibiotics and bismuth and iodine preparations are prescribed by repeated courses according to the schemes.

Progressive paralysis, or bale's disease - a psycho-organic disease of the luethic genesis, characterized by a progressive impairment of mental activities with the formation of a persistent defect, up to dementia in combination with neurological and somatic disorders. For the first time was described by the French psychiatrist Antoine Laurent Beil in 1822. The term "progressive paralysis" was proposed by Delane. For the first time the possibility of syphilitic lesions of the brain shells and the brain substance was identified by Lalleman in 1834 at the opening of the patient deceased from syphilis. The proof of the syphilitic nature of the disease was the discovery of pale treponam in the cerebral core of patients with progressive paralysis Pyatnitsky in 1911 and Japanese scientists in 1913. Progressive paralysis arises in adulthood (30-50 years), ≈ twice as often in men, and more malignant than the syphilis of the brain, leading to severe little consequences.

During the progressive paralysis, three stages are distinguished: initial, the stage of development of the disease and the stage of dementia.

Initial stage, like during the syphilis of the brain, is called neurasthenic Stages, or stages of harbing, and is characterized by the appearance of fast fatigue and fatigue, in general muscle weakness, increased irritability imposing apathy. Over time, the personality changes with the loss of the patient's behavior are more distinctly presented, the tact is lost, the criticism of their behavior is reduced. To this period include the increase in drowsiness during the daytime and the appearance of insomnia at night; Patients lose their appetite or they appear excessive voraciousness. Against the background of signs of neurasthenia, the most typical for the progressive paralysis of the symptoms is found - the loss of care about the family, the disappearance of sensitivity to loved ones, wastefulness, sludge, loss of smallestness, involuntary, inappropriate use of obscene, cynical, obscene expressions with complete loss of criticism. Subsequently, lethargy is quickly joined and increasing, insurgent to surrounding with weak, sentimentality, passivity (there was a point of view that all these disorders are associated with affective violations). In the future, the dischargeability fall increases: patients in their usual work begin to make gross mistakes, which over time cease to notice due to loss of attention and criticality.

In Neurostatus at this stage, the transient non-uniformity of pupils, the paresis of the eye muscles, tremor, the unevenness of tendon reflexes, the discoordination of movements, the insecurity of the gait is found. Speech becomes monotonous, inhibited or, on the contrary, unreasonably hurried.

In the blood, the Vasserman reaction is more often weakly alone or negative. In the study of the WASSERMAN reaction, the non-older reaction, the Pandy is converted, cytosis (20 - 30 cells) are detected, an increase in protein content. Rit and reef in all dilutions are sharply positive.

IN the period of heyday- In the second stage of the disease - along with increasing dementia, decrease in memory and weakness of the thought process, delusional ideas arise, improving the mood with gross sexual lesibility. There is no criticism for the state of such patients. Sometimes depression is developing with suicidal tendencies, nihilistic nonsense. According to a predominance in the clinic of the second stage of one or another psychopathological symptoms allocated

Forms of progressive paralysis .

Each of them is distinguished by the predominance in the clinical picture of certain disorders from those described above. Many forms of progressive paralysis are development phases Diseases, reflecting along with the depth of the defeat of mental activity at the moment of the painful process and denoting the pace of its development.

Simple, or dementant, The form is up to 70% of cases of progressive paralysis at present. It is characterized by the gradual increase in paralytic dementia with the carelessness, the loss of the tact and the highest ethical norms of behavior, critics, indifference and indifference to the surrounding, reduced memory, show signs of mental failure. Acquisition of new skills becomes impossible. These disorders appear against the background of complete indifference or complacent attitude towards the surrounding and ridiculous acts. The patient is bought at once four umbrellas, not focusing in their city. The patient brings two kilograms in his pockets. Another patient, a cook, pours Kerosene to Salad, adds sugar to soup. Sometimes easy excitement appears. Patients get up at night, stood ridiculous walks, allow hedonic excesses, not paying attention to traditions and customs. However, professional skills in this form persist relatively long. Later joined dizziness, fainting, arise apoplexiform and epileptiform epiphyroids. Apoplemic seizures are accompanied by the development of mono- and hemipreps, speech disorders; Epileptiform differ in ability and often resemble Jackson. Over time, the patient loses the ability to comprehend the easiest things, forgets the current events.

Stationary paralysis It is characterized by a slow course with remissions, which usually occur with the initial therapy.

Expansive (manic) The form is considered classic. It is characterized by a sharp increase in mood with the occurrence against the background of the total demention of ridiculous delusional ideas of greatness. In particular, patients assure that they are the Lords of the Galaxy, the Lords of the Stratosphere, which have millions of wealth, thousands of residential rooms and cars, which does not prevent them from "shooting" cigarettes from the chamber's neighbors. Against the background of euphoric mood, they can occur episodically, short-term flashes of anger, which change the "royal" complacency. Inadequate situation becomes behavior when patients make frivolous, often ridiculous acts; Show a tendency to ambiguous flat jokes. Not only doctors, but also the patient surrounding the grace, euphoria, carelessness, unjustified joy, as a rule, accompanied by ridiculous delusions of grandeur and wealth (he may be unstable). The clinic of expansive paralysis is characteristic so that in society it was customary to correlate nonsense with global buildings - Megalomania - specifically with progressive paralysis. "Listen, Garin, and you did not have syphilis?" - asks Shelga Engineer Garina, describing his grand ideas in the novel by A. Tolstoy. Sick Guy de Maupassan, already staying on the verge of poverty, claimed that he received an inheritance from Rothschilds, that he was the richest man in the world, at the same time not shy to ask for food. As E. Blair writes, "... the patient is not just God, he is the Supreme God, ships, the size of each with the Lake Geneva, is carrying thousands of millions of millions of diamonds from India ... He invents a bike on which it is possible to drive around the globe around three minutes."

For depressive form There is a decrease in the background of the mood, tears, hypochondriad statements, ridiculous delusional self-evidence ideas are also megamastea. Patients accuse themselves in misconduct, leading to the death of the earth, the whole universe. Sometimes a depressive-ipochondria form with a predominance of a hypochondriatic nonsense with coar syndrome on the background of low mood with tearfulness is distinguished. Depression, longing can lead patient to suicide.

Circular shape Combines two previous and manifests change of manic and depressive states. In mania, paying attention to the euphoric tint and inactive, in depressions - a sullen-gloomy mood, replacing the dysphoria. It is considered characteristic of cyclotimics.

Mounted form It is characterized by sharply pronounced motor excitations with a tendency to destructive trends, aggression, visual and auditory hallucinations and illusions. The speech is incoherent, consciousness is praised by type of twilight. Patients scream, break things, waving their hands, jump, undress, pronounce cynical phrases. Later join the convulsive movements, grinding with teeth, and, later, the death relief. With this form, the exhaustion occurs within a few weeks, with the next seizure there comes death. In less severe cases, the disease in this form stretches for months.

Galoping form It has an extremely unfavorable rapid flow and is accompanied by severely pronounced motor excitations, incoherent nonsense, epileptiform or, less often, apopleptiform seizures, vegetative and trophic disorders, increasing exhaustion and fatal outcome. With the predominance of seizures sometimes allocate epileptiform Palsy, as a rule, flowing unfavorable. "A solid baker is somewhat frivolous about 52 years, lost a decent state on speculation and inventions. One day, suddenly became restless, began to run out of the house, although each time I found some reason to this - I didn't want to tram my oven and so on. A few months later, a hallucinatory attack was suddenly occurring; The voices were ordered to undress donaga, eat only three throats, cut off three fingers. The clinic does the opposite on the contrary, pours soup from the plate in the pelvis and from the pelvic to the plate until everything falls to the floor; It knocks on the door day and night, tears things, jumps, trying to beat the staff, does not recognize the surroundings. Fixing his attention fails. It's completely confused. In addition, the patient has neurological symptoms of progressive paralysis. After 8 days the patient is completely depleted and it is possible to keep it in bed (first with sleeping pills), although he is still fussy. Then moves are made powerless and disorderly; After 8 days, he dies from exhaustion. " (by E. Bleiler) These forms are more characteristic, mainly for persons of an excitable circle.

Hallucinatorium-paranoid The form consists of true and (more) pseudogalucinations, low-powerful ridiculous perception ideas - persecution, impact; catatonic disorders. Arrives people with schizoid features.

Katatonic The form is characterized by the development of a catatonic stupor or excitation. Prognostically unfavorable.

Senile progressive paralysis It develops in persons over 60 years and is distinguished by a long (up to 40 years) latent period. The painting of the disease in these cases is quite difficult to distinguish from the senile dementia. The development of Corsakovsky syndrome with increasing memory disorders, correlations and delirium magnitude is possible.

In cases where focal symptoms are prevailing, characteristic of the preferential localization of the process in the rear sections of the brain - apraxia, agnosia and other similar symptoms with slow progression of dementia - allocate paralysis of Lisouera.

For congenital Syphilis can develop children's and juvenile paralysis. It is often preceded by the phenomena of mental delay, the expansive nonsense detention has been joined later. The presence of pathognomonic for Lues Congenita somatic signs - saber-shaped legs, luethic aortitis, the hetchinson triad, including parenchymal kerats, labyrinthitis with deafness and getchinson teeth are central upper incisors, having a barrel shape, narrowed to the cutting edge, with a characteristic concreteness of it and slightly deployed inside .

Taboparalich It is a combination of progressive paralysis with a spinal dry shot. In this case, in addition to the symptoms inherent in progressive paralysis, the symptoms of the Tabes are developing - the complete disappearance of the knee and achyllated reflexes, the disruption of sensitivity, etc. is less likely to be depressed with nihilistic nonsense achieving the degree of coat syndrome. Attention is drawn to a violation of a patient's gait: at first it becomes awkward, in the future sobbed and unstable. Tabetic vegetative-somatic violations are developing, peripheral trophic disorders: some patients lose weight, others will be fulfilled, the trick of the face appears, the turgor changes; Against this background, various intercurrent diseases are easily developed. Complications arise: abscesses, furuncula, increased bone fragility, etc. With a hardware examination, dystrophic changes of all internal organs with polyorgan deficiency are detected.

CSR blood in these cases is always positive. In the cerebrospinal fluid, the reaction of Wasserman and other serological reactions are sharply positive, the reaction of Lange paralytic type (665543210).

Mental disorders under progressive paralysis are accompanied by multiple neurological symptoms. One of the early signs are pupil symptoms: a persistent anisocorium with myiosis and strain deformation in combination with the symptom of Argya Robertson - with a weakening or lack of photoreaction while maintaining the reaction to accommodation and convergence, pathognomonic for the Loethic Deviation of the CNS. An important feature is dysarthria: the patient is not able to purely articulate individual phrases; During the pronunciation of the words, he whatever stupid on the syllables. In addition to the dysarthritia there is a logoclonium. It is characterized by repeated repetition of the last syllable in a word, for example "Monet-Ta-Ta-Ta". Often there is a bilateral paresis of facial nerve. In this case, the face of the patient is a mask with a sluggish and meaningless expression, subtle mimic movements disappear at all. Speech vile due to the paralysis of the soft sky. Language is shown insecure, it is noticeable to twitch individual muscle beams. Gradually, dysarthria increases, it becomes more and more vague, lubricated, and then low. The handwriting is frustrated: the lines when writing becomes uneven (then take off up, then falling down), there are letters of letters. The letters of unequal, with corners instead of roundings, they are skipped or by way repeated.

Troops of patients with progressive paralysis are also violated. They have increased bone fragility, hair loss, swelling, the formation of trophic ulcers. Despite excessive appetite, there is a sharp progressive depletion of patients.

Stage Dementia It is characterized by sharply pronounced dementia with a decrease in criticism, the weakening of the judgments and the presence of ridiculous conclusions in combination with the euphoria, by the time of replacing apathy, and then constant aspotion. Patients are completely losing interest in the surrounding, cannot serve themselves, do not answer questions. In rare (at present) cases, insanity is developing with disorders of swallowing act, involuntary urination and defecation.

With a neurological examination, the same disorders are found as in the heyday of the disease, however, they proceed noticeably harder, the seizures occur significantly more often, strokes are developing with subsequent steady paresis, paralegia, aphasia and apraxia, often leading to death. In somatic status, a sharp weight loss, numerous trophic ulcers, bone fragility, urinary bubble paralysis, breakdown, are drawn.

The causes of death are intercurrent diseases (pneumonia, sepsis) or a luethic lesion of vital organs (aortic aneurysm break).

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 spread at the time. The fact is that a large number of cases of polymorphic postientephalitic parkinsonism masked rare pathologies, which were considered atypical forms.

Progressive supervising paralysis as independent neuropathology was allocated in 1963-1964. Group of Canadian doctors: neurologists J. Steel and J. Richardson and the J. Olszewski pathorphologist. They described and analyzed 7 cases of neurodegeneration with a characteristic clinical picture. In the USSR, progressive supervised paralysis was first mentioned in 1980 by the doctors of the clinic of nervous diseases of the Moscow Medical Academy. THEM. Sechenov, who observed two patients.

Subsequently, the disease continued to be studied, was allocated in domestic and global classifications as a separate nosological unit. In the ICD-10, progressive supervising paralysis refers to diseases of the nervous system (section of extrapyramidal and other motor disorders, subsection of other degenerative diseases of basal ganglia), is encoded by G23.1.

General

Progressive paralysis is a separate nosological form of late neurosimifilis. It is syphilitic meningoencephalitis - inflammatory lesions of the shells and cerebral parenchyma. The disease was described in detail in 1822 by the French psychiatrist Antoine Beil, in honor of which in neurology was called Beil's disease.

In 1913, the Japanese Patomorphologist X. The legs proved the syphilitic etiology of the disease, discovering pale treponams in the cerebral tissue of patients. Progressive paralysis occurs in 1-5% of patients with syphilis. The temporary range of symptomatic development lies between 3 to 30 years since infection, more often is 10-15 years.

Progressive supervising paralysis (PNP) is a degenerative defeat of the brain of unclear etiology. Along with Alzheimer's disease, multisystem atrophy, corticaase degeneration, peak disease, PNP refers to tapaks characterized by the formation of inclusions of Tau-protein in neurons and glial cells.

The progressive supervising paralysis was first described in detail in 1963-64 by Canadian neurologists with stylus and Richardson in co-authorship with the Olshevsky pathologist, in honor of which the name of Styla-Richardson-Olshevsky was called. The prevalence of the disease according to various information sources varies within 1,4-6.4 cases per 100 thousand population.

Prevalence

At the heart of the disease - the progressive degeneration of neurons arising from the accumulation of pathological protein in them.

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

The disease is revealed mainly in the people of the older age group, even the hereditary-conditioned forms are usually manifested between the ages of 50.

Causes

An etio-factor of the disease is the causative agent of Syphilis - Pale Treplema. The source of infection becomes a sick person, the main way of infection - sexual and injection. Possible transmission of the pathogen through saliva. The skid of the infectious agent in cerebral tissues is taken by hematogenic and lymphogenic way.

The reason for the long-term persistence of the pathogen, with which the progressive paralysis is associated is reliably unknown. Probable predisposing factors are the absence or insufficiency of the correct treatment of early forms of syphilis, hereditary predisposition, alcoholism. The triggers provoking the activation of infection are considered to be ancient brain injury, the weakening of immunity against the background of acute and chronic somatic diseases.

Et phase, launching degenerative processes of a certain cerebral localization, remain unknown. Most cases of disease are sporadic. Separate family options with alleged autosomal dominant inheritance were identified after 1995. Molecular genetic studies have shown that some forms of the PNP are determined by the defects of the gene encoder-protein gene localized in Locus 17Q21.31. The most likely the multifactor mechanism for the occurrence of pathology, implemented against the background of genetic susceptibility.

Etiology: why this disease develops

In 1913, X. The legs proved the syphilitic etiology of P. p., Finding a pale trepony in brain tissues.

Pathogenesis is not studied enough. K. Levaditi on the basis of observations when P. P. There were both spouses or several persons infected with syphilis from the same source, put forward the assumption of the existence of a special neurotropic treponem. What, however, has not been proven. Perhaps, in the pathogenesis, the role of violation of the body's reactivity is played (see) with the sensitization of the brain tissue, as a result of which, in some cases, the brain is penetrated in some cases.

The reasons for the development of progressive supervising paralysis are not reliably known. This disease is not associated with any infections transferred to injuries or exposure to external adverse factors.

Progressive supervised paralysis is considered sporadic pathology (manifested in a population of unsystematic). At the same time, since 1995, information on the presence of rare family cases of the disease with an autosomal dominant type of inheritance are published. This variant of the disease is associated with the heterozygous mutation of the gene encoding Tau-protein and located at 17Q21.31.

Classification

Clinical symptoms of variable, weakly manifested in the debut of the disease, changes and aggravated as progression. Understanding the process of the process is necessary for the selection of the most effective therapy. In this regard, in neurological practice, progressive paralysis is divided into three clinical periods:

• Initial. Corresponds to the beginning of clinical symptoms. Characterized symptoms of neurasthenia, sleep disorders, reduced performance. The initial stage continues from a few weeks to 2-3 months. By the end of the period, the identity changes are becoming obvious with the trend towards the loss of ethical norms.
• Disease development.The growing personal changes, disorders of behavior, speech disorders, gait and handwriting, intellectual decline. Typical trophic violations, dystrophic changes in somatic organs. Without treatment, the stage lasts 2-3 years.
• Dementia. The progressive decay of the cognitive sphere is accompanied by apatine, complete loss of interest in the surrounding. Epileptic seizures are often observed, strokes. Trophy disorders are aggravated, bone fragility increases. The patient dies from stroke, intercurrent infections.

The following classification is based on a syndromological approach. Taking into account the prevailing symptom complex allocated a whole list of diseases. The main ones are the following clinical forms:

• Dementant (simple) - increasing dementia with the predominance of Euphoria. Most common. Not accompanied by severe mental disorders.
• Expansive - pronounced arousal with the predominance of delusional thoughts about its own greatness, wealth. Euphorically complacent mood is sharply replaced by angiveness. Typically multiple, criticism is completely lost.
• Euphoric - progressive dementia is accompanied by fragmented ideas of greatness. In contrast to the expansive form proceeds without pronounced neuropsychiatric arousal.
• Depressive - dementia progresses against the background of ridiculous hypochondria complaints. Patients seem to have no insides that they have already died. Characterized elements of depersonalization, delaying up to catar syndrome.
• Assembly - excitement sharply pronounced, there is a tendency to aggression. Observed visual, auditory hallucinations. Consciousness matured, a quick decay of the personality occurs.
• Circulatory - like manifestations bipolar disorder. The period of maniacal excitement is replaced by a heavy depression. It is believed that this form is implemented against the background of genetic predisposition to mental illness.
• Hallucinatorium-paranoid - psychosis with the predominance of hallucinatory syndrome, brad Pursuit, catatonia. It was suggested about the emergence of this disease in patients with schizoid psychopathy.

The most typically start of the Bale's disease at middle age. Progressive paralysis, manifesting in kindergarten and old age, is characterized by a number of features. Accordingly, 2 age-related forms of the disease are distinguished:

• Juvenile. Debuts aged 6-16 years after the suffered congenital syphilisaresulting from a transplacentating infection. More often proceeds in the form of a dementary form with triad heetchinson (keratitis, hearing loss, lesion of the upper cutters). Symptomatic manifestations can precede impaired mental function.
• Street. The debut accounts for the period after 60 years. Typically long asymptomatic flow. Perhaps development korsakovsky syndrome. The elder form is extremely difficult to differentiate from sedenie dementia.

Pathogenesis: What is happening in the brain?

The development of symptoms of progressive oversale paralysis is associated with irreversible and steadily increasing degeneration of neurons in certain areas of the brain. The basis of such a destructive process is the excess intracellular accumulation of neurofibrillary glomers and lost the structurality of neuropyl threads. They violate the functioning of neurons, contribute to 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 hyperphospory. Normally, it is attached to the microtubules of tubulin, is responsible for their polymerization and stabilization of microtubules and fixing some intracellular enzymes.

The main functions of the normal Tau-Protein include:

1. participation in the processes of maintaining the neuronal cytoskeleton (carcass of the nervous cell);
2. education and elongation of axonal processes;
3. restoration of neurons after damage;
4. regulation of intracellular vesicle transport (cytoplasmic bubbles) with synthesized neuropeptides.

Hyper phosphorylated Tau-protein is no longer able to hold the structure of microtubules. They are disintegrating, and the anomalous protein has formed a filament (tube) of the wrong shape, which are assembled in the cytoplasm in neurofibrillar balls. At the affected cell, biochemical contact with other neurons is disturbed, the ability to form and retain axonal bonds, the cytoskeleton becomes unstable, life is significantly reduced. Such neurodegeneration is irreversible and progressive, gradually spreading from the characteristic primary zones on the entire brain.

Neurofibrillary tangles in neurons are formed not only with progressive supervising paralysis. Similar degenerative changes of the brain are also found in Alzheimer's disease, corticational degeneration, front and temporal dementia and other other other, more rare diseases.

The study of various Tupathas, the development of their differential diagnosis and the clarification of nosological criteria is made by a specially established Risenburg Working Group (ReiseThies with Parkinsonism).

Symptoms of PNP

Progressive supervising paralysis is characterized by nonspecific clinical debut. The symptoms of this period is represented by unusual fatigue, reduced efficiency, cephalgia, dizziness, reduced mood, narrowing the circle of interest, sleep disorders, including insomnia at night and hypersigny. Subsequently, the symptoms of Akinetic-Rigid Parkinsonism are joined. Postural tremor in most patients are missing. Muscle rigidity is expressed mainly in axial muscles - muscles walking along the cervical spine connecting it with the skull. Patients complain of stiffness in the neck, back. The increase in tone in the back muscles of the neck leads to a typical "proud" position of the patient's head. Parkinsonic ataxia is characterized, due to disorder coordination to the position of the body and lower limbs relative to the center of gravity. Difficulties in maintaining equilibrium in the process of walking lead to frequent drops back.

A distinctive feature of the PNP is ophthalmoplegia, which arises on average after 2-3 years from the debut of the disease. Against the background of the slow motion of the eyeballs, paralysis of the gaze in the vertical plane, the patient cannot lower the eyes down. Because of the rare blinking, the patient feels discomfort, burning in the eyes. Blurry of vision, convergence disorder, blefarospasm is possible. Progressive supervised ophthalmoparez is accompanied by a restriction of the look down and up, with time it can lead to glazation disorders in the horizontal plane. With the development of complete ophthalmoplegia, the retraction of the upper eyelids is formed, which gives the face a surprised expression.

In the clinical picture of the PNP, pseudobulbar manifestations occur relatively early: dysarthria, dysphagia, violent crying or laughter. Changes are occurring a personality-emotional sphere, patients become closed, apathetic, demotivated, indifferent. Cognitive violations in most cases are joined in the midst of the disease, in 10-30% of cases - at the debut stage. It is characteristic of an intellectual decline, disorder of abstract thinking and memory, visual-spatial apraxia, elements of agnosia. Dementia is observed in 60% of patients with 3 years of experience.

The disease begins nonspecific, slowly increasing symptoms, similar to neurasthenic manifestations. Sick complains of reduced performance, unusual fatigue, irritability, headaches, worsening memory, sleep disorders. Over time, close patients notice the changes in his personality: indifference to the problems of others, the loss of the sense of tact, the exit of behavior beyond the framework of ethical norms. Patients begin to make gross mistakes in familiar professional activities, when progressing symptoms lose their ability to realize them. The sleep disorders are aggravated: the day is celebrated by hypersmia, at night - insomnia. There are violations of food behavior (anorexia, bulimia). The speech is overly accelerated or slowed down. Criticism is reduced to their behavior.

At the stage of development of the disease, progressive paralysis is accompanied by aggravation of personal and behavioral disorders. Inadequate behavior, ridiculous actions, flat jokes are revealed. Mental disorders differ in polymorphism, detects euphoria, depression, delusional ideas, hallucinations, depersonalization. In the speech there are difficulty pronunciation of complex words, then distemper is formed - it becomes a challenged, lubricated with the passage of individual sounds.

The gait is unsure, breaking, the handwriting is uneven, the disgrave is noted with letters of letters. Place the fainting, epileptic attacks (more often by type jackson epilepsy). Disorders of appetite are the cause of weight loss or body weight. Trophic disorders appear: reducing the leather turgora, nail fragility, light infection with the development of furunculus, abscesses. It is characterized by increased bone fragility, dystrophic changes in the heart (cardiomyopathy) and liver (hepatosis).

During the period of dementia there is a rise in dementia to the maratism. The patient ceases to serve itself, interact with others, arbitrarily controlling the function of pelvic organs. Failure to receive food leads to pronounced weight loss. Sometimes dysphagia is marked - swallowing. Numerous are formed trophic ulcers, increasing bone fragility becomes the cause of fractures.

Patients need etiotropic and psychocorrenetrating therapy. The healing effect of high body temperature was discovered experimentally. Modern treatment includes four main components:

• Pyroterapy. Earlier, it was carried out by the introduction of malaria causative agents with subsequent antimalarial therapy. Pyrogenic preparations are currently used.
• Antibacterial therapy. Antibiotics of penicillin row, ceftriaxone, uodvismutat quinine, bismuth nitrate have antibiotics. Ethiotropic therapy begins with large doses, continue 2-3 weeks. Parallel prescribe probiotics, polyvitamin preparations, if necessary, hepatoprotectors.
• Psychotropic treatment. Showing in the second phase of Bale's disease. Individual selection of pharmaceuticals (sedative, neuroleptics, antipsychotics, antidepressants) is carried out in accordance with symptomatics.
• Neurotropic therapy.Aims to improve metabolic processes in the brain and restoring its functions. Includes chorical, nootropic, neurometabolic action pharmaceutical. In the stage of dementia, neurotropic treatment does not have a significant effect.

What is affected by progressive supervised paralysis

With progressive supervising paralysis, neurodegeneration in most cases begins in subcortical and stem formations. The bark of large hemispheres is initially captured to a lesser extent, but as the disease progressing the process, the process is steadily applied to it. At the same time, the front departments of large hemispheres are most affected.

Localization of the main changes:

• black substance;
• subtamelamic and peduncular kernel;
• pale ball;
• talamus;
• middle brain tire;
• stem portion of reticular formation;
• the temporal and prefrontal zones of large hemispheres.

The primary defeat of these areas explains the characteristic order of the appearance of symptoms and the typical Parkinson-like debut of the disease. And the mandatory presence of glazation disorders is associated with the degeneration of the dorsal part of the middle brain, which leads to disunity between the ties between the centers in the core and the trunk of the brain. The core cereal cores themselves responsible for the work of the muscles of the eyeballs remain intact. Therefore, paralysis is called the oven.

Are a mandatory manifestation of the disease and are characterized by a characteristic combination of symptoms:

• Loss of ability to arbitrary movement of eyeballs: usually first in the horizontal and then in the vertical plane. The outcome is complete offpalmoplegia with the inability to purposefully translate the view.
• Reducing the convergence, which already in the early stages of the disease is accompanied by complaints about the blurredness of vision and the cooler in the eyes when translating a view to the developed items.
• Preservation of reflector friendly movements of eyeballs.
• The appearance of phenomenon puppet eyes when the eyeballs when the head moves continue to involuntinate the object. This is due to the absence of suppression of vestibularlyocular syndrome. In earlier stages, there is an intermittentness and "lag" tracking the movement of a moving object (with an inspection of a neurological hammer), which leads to the appearance of hopping "catchy" movements of eyeballs.
• Gradual fading of amplitude and speed of arbitrary saccadic movements of eyeballs. During a neurological inspection, this is detected when checking the tracking movements, each re-transfer of the view in extreme-lateral leads is accompanied by an increasing restriction of eye mobility (hypothrity).
• The absence of spontaneous nystagma.

The method of provocation of nutritional nystagma can be used, with the rotation of the striped drum in the patient's face. With progressive supervising paralysis, the rapid phase of the nastagma in the vertical plane is initially slowed down, and at later the stages of the disease it is not at all caused.

The first signs of ophthalmopathy appear usually in the early stages of the disease. Moreover, the decrease in the convergence, changes in the optoxic nastagma and the decrease in arbitrary vertical saccad are often detected and in the absence of a patient complaints about violations. To form an alleged diagnosis, it is necessary to have at least limitations of the look down, in combination with other signs of the neurodegenerative process.

Complications

The accompanying disease of Beil cerebral vasculitis is the cause of frequent ONMK. As a result of the transferred stroke, a resistant neurological deficit is formed: paresis, aphasia, apraxia. Trophic changes lead to a decrease in the barrier function of the skin with the development of pyodermia, abscesses, at later stages - flaps.

In the initial period of falling the patient without the possibility of coordinating their movements lead to the bruises and fractures. A few years later, progressive oligotradichetic syndrome is caught by patients to bed. In the absence of proper care, immobility is hazardous by the development of contractures of joints, laying out, stagnant pneumonia.

Progressive pseudobulberry paralysis determines the sticking of food with the risk of asphyxia, aspiration pneumonia. Night apnea can cause sudden death in a dream. A serious complication is the accession of intercurrent infections (pneumonia, cystitis, pyelonephritis), since against the background of reduced immunity there is a high risk of sepsis.

Clinical picture

P. p. - Heavy illness of the whole organism, and the most vivid manifestations - violations of mental activity. The main syndrome is the progrement of total dementia (see): the intellect is rudely suffering, the disorder disorders, criticism and especially self-criticism disappears. There is no mind of the disease, the memory sharply decreases, feedback arise (see confable meter).

The manifestations of dementia are amplified due to the frequently observed euphoria (see Psycho-Organic Syndrome). Neurol. The symptoms lies in speech disorders, primarily in violation of articulation - dysarthria (see). It becomes unclear, lubricated, especially when pronuncated by long words, the patient misses or overstoves the syllables, does not pronounce the endings of words.

The handwriting is done uneven, individual letters and syllables fall out of words. Voice timbre changes, it becomes deaf. The patient's face is inexpressively, the masculine, since the innervation of the muscles of the face is disturbed, blefaroptosis arises (see Ptoz). Temor reflexes are often increased and uneven, except for the cases of papoparalacy (see).

Based on psychopathol. The manifestations are distinguished four stages of the disease: latent (from syphilis contamination before the manifestations of P. p.), the stage of initial manifestations, the stage of the full development of the disease and the stage of the marasma (see). In the latent stage, headaches, dizziness, fainting states may be occurring, in some cases there are characteristic changes in the cerebrospinal fluid (see).

The initial manifestation stage is characterized by increased fatigue, irritability, weak. Patients complain of the decline of strength and reduced performance, although they can still do the usual work to some extent. Previously, such conditions due to external similarity with neurotic symptoms were incorrectly called preparalitic neurality.

In some cases, in the stage of initial manifestations, depressive and delusional disorders are observed - anxiety depression with hypochondriam, anxious-armed depression, delusional ideas of jealousy, persecution, poisoning; As the symptoms are increasing, these endoform disorders disappear.

Improving memory is very early. Separate actions indicate a violation of criticism. The sphere of deposits is upset, patients become voracious, erotic. The increase in these disorders speaks of the transition of the disease in the stage of complete development, K-paradis is manifested in various wedges. Forms.

Expansive, or classical, form (previously widespread) is more often found in men. It is characterized by the presence of maniacal excitation with the manifestation of amphibiousness, grand deliberation of greatness (see nonsense). The dementary form is characterized by increasing dementia against the background of a lackless euphoria. In depressive form, the depressed mood is developing, often with anxiety and desire for suicide (see

Depressive syndromes) are often observed with a ridiculous hypochondriatic nonginistic content. The circular shape, first described by S. S. Korsakov, proceeds with the change of states of arousal and depression. The hallucinatory-paranoid form is characterized by the development of paranoid syndrome (see) with mainly auditory hallucinations and nonsense prosecution.

With a catatonic form, a stuporous state occurs (see) with phenomena of mutism and negativism (see Cattonic syndrome). In the stage of Mazesma, conscious activities are stopped, the speech disappears, patients publish inseparal sounds, cannot stand and move. In this stage, they die from intercurrent disease.

By the flow, a particularly malignant molded shape (galloping paralysis) was isolated with a sharp motor excitation and a disturbance of the aimative type and so called. Stationary paralysis, with K-ROM, there is a slow flow with a gradual decrease in intelligence and lethargy.

Atypical forms of P. p. Are the youthful and senile P. p., As well as paralysis of Lisouere and Taboparalich (see). Junior 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 arises in children who were considered healthy.

Most often flows in a dementary form; It is often observed local symptoms, for example, atrophy of the visual nerves. The senile P. P. Arises over the age of 60 years and is characterized primarily by a long latent stage (up to 40 years). Wedge, the picture resembles senile dementia (see) with pronounced memory disorders; Sometimes the disease occurs according to the type of Corsakovsky syndrome (see).

Paralysis of Lisouera and Taboparalini are characterized by relatively slow increase in dementia. At Lisoueer paralysis, there is a tendency to the local affix of the brain, mainly parietal fractions, while developing aphasia (see), agnosia (see), apraxia (see), apochkitiform and epileptiform seizures.

Tremor of rest for the progressive supervising paralysis is not characteristic.

All symptoms of progressive supervising paralysis are combined into several groups:

• Overall disorders in the form of a paralysis of the gaze and a number of other symptoms, accompanied by retraction (lifting) of the upper eyelids with the formation of the characteristic "surprised" facial expression.
• Parkinsonianism (akinatic-rigid form). Moreover, extrapyramidal disorders in the classical course of the progressive outdantiary paralysis have a number of features that allow us to carry out the correct differential diagnosis. The prevalence of the rigidity of the muscles of the neck and the shoulder belt with the formation of the characteristic "proud" posture, bradykinosis (slowdiness of movements), the symmetry of violations even at the initial stages, early appearance of postural instability. This extrapyramidal symptomatics is not corrected by anti-marketsonic drugs. Not characteristic Tremor rest, fall, obvious vegetative and pelvic disorders.
• Walking disorders, usually by type of subcortical Astasia with a pronounced influence of postural instability. At the same time, the length of the step, the area of \u200b\u200bthe support and the initiation of movements are not changed, the friendly movements of the hands and legs are preserved. People with progressive supervising paralysis already in the early stages of the disease are easily losing stability when turning, changing the speed of movement, jogging, walking along the inclined surface. During the first year of the disease, there are falls backwards, and without attempts to keep equilibrium.
• Cognitive violations, with a fairly rapid development of the dementia of the frontal-subcortical type. It is impossible to decrease, the ability to abstraction and generalization is lost, the apathetic, field behavior, low speech activity, echopracts are characterized.
• Pseudobulberry syndrome due to the defeat of the frontal bark and speaking control paths from it. Early develop dysarthria (fuzziness soundlessness), dysphagia (dysfunction of swallowing, with a preserved and even increased pharyngeal reflex), symptoms of oral automatism, violent laughter and crying.

For progressive surgery, paralysis is not characteristic of illusion, hallucinatory-delusional syndrome, high-quality and quantitative disorders of consciousness, bright affective disorders.

Atiypical clinical forms of progressive supervising paralysis are possible: with the predominance of Parkinsonism and the appearance of asymmetric dystonia limbs, with the debut in the form of rapidly increasing cognitive disorders, with the predominance of primary progressive aphasia.

Prediction and prevention

With supervised paralysis, non-stop progression of symptomatics is observed. Conducted therapy does not have a significant effect on the course of the disease. The life expectancy of patients varies within 5-15 years. The fatal outcome is due to intercurrent infections, a protracted sleep apnea, aspiration pneumonia.

The results of treatment depend on the timeliness of its start. In 20% of cases, correct therapy allows you to achieve practically complete health restoration. In 30-40% of patients, it is possible to achieve a resistant remission with moderate disabilities of psyche, allowing adapting in society. In 40% of cases, persistent organic dementia remains.

The process of sanitation of the liquor takes from half a year to 3-4 years. Prevent progressive paralysis allows personal hygiene compliance, rejection of random sexual relations, unprotected sexual intercourse. With the first established syphilis, it is necessary to prior to complete sanitation of biological environments. Subsequently, the patient recommended observation and periodic examination for early detection of late forms of syphilis.

With the defeat of both shells, vessels and brain substances (parenchyma).

Clinically progressive paralysis is characterized by the growing decay of the psyche (dementia) and the extreme somatic depletion of the patient (insanity). The main clinical symptom of progressive paralysis, therefore, is dementia. However, the first signs of the disease can be detected by very imperceptible, at first glance, the usual symptoms of the depletion of the nervous system. In this cunning of this disease. From the moment of infection with Syphilis before the detection of progressive paralysis takes place on average from 8 to 15 years. Faced progressive paralysis not all ill syphilis, but only 3 to 5%; A bowl of men aged is usually from 35 to 50 years. The development of neurosimifilis contributes to the weakness of the nervous system and the presence of additional factors causing this weakness (injury, alcoholism). The number of cases of diseases by progressive paralysis in our country decreased dramatically due to a significant reduction in syphilis disease.

The course of progressive paralysis can be divided into three periods:

First period The initial symptoms of the disease are expressed in common neurasthenic complaints (period of preparalitic neurasthenia). There is an increased irritability, scattered, forgetfulness, insomnia, a decrease in working capacity, tearfulness. Unlike conventional neurotic (as the form of neurosis of a breakdown of the nervous system), the symptoms of which after a minor rest and the appropriate treatment are carried out without a trace, the symptoms of preparaltic neurasthenia are growing. Already at the very beginning, symptoms are observed, indicating organic lesions of the brain, which does not happen in conventional neurasthenia (see the section of neurosis). When studying the nervous system, an anisocorium is found, the symptom of Ardzhill Robertson, the smoothness of the nasolabial fold, an increase, lowering or uneven tendon reflexes. An important and early neurological symptom of progressive paralysis is a dysarthria, stubbing on some complex phrases for pronunciation. So, for example, the patient can not be clearly, a meselly pronounces the patter: "Serum from the prokubvashi", "She sews the cap, but not in Czpakovski, it is necessary to smash it", "in the courtyard grass, on the grass of firewood." The same early symptom is the letter disorder: the handwriting becomes an uneven, the sick letters, a syllable or doubt them; So, for example, instead of the word "distribute" writes "Crane" or "Raprell". The presence of described neurological symptoms is the main differential feature and facilitates recognition of early forms of progressive paralysis. The combination of neurasthenic complaints with the specified symptoms from the nervous system gives a complete basis for the clinical diagnosis of progressive paralysis and the immediate direction of the patient in a psychiatric hospital for treatment. The duration of the first period is small from about a few weeks to 2 3 months.

Second period, Period of complete development of symptoms of the disease. In this period, all the symptoms of already developing dementia are all symptoms: the patient loses the ability to critically assess its condition, considers herself healthy. The mood is predominantly complacent. The patient euphoric, revived, makes actions, whose absurdity often draws attention to others. Patients become erotic, rafting frivolous ties, become wasteful, spend large amounts of money for the purchase of unnecessary things; So, one of our patient in May for all crescent payments 700 rubles bought a carcass in the car and on a wheelbarrow, to the amazement of loved ones, delivered home. In this period, symptoms of physical exhaustion are gradually enhanced.

Third period Demolition period (ins). The dementia reaches extreme degrees: the patient utters some inseparable sounds, remains fixed in bed due to the full paralysis of the limbs. Involuntarily allocates urine and feces as a result of paralysis of sphincter. Physical exhaustion is very sharp. Leather with trophic disorders, with abundant variages that are infected with feces. The subcutaneous fiber almost disappeared. The skin takes the bones that become very brittle, as a result of which fractures arise.

The described picture of general maratism requires patient, very careful attitude towards the patient and especially careful care. Since in our country the number of cases of syphilis significantly decreased, then cases of progressive paralysis (especially the launched form of progressive paralysis) in a psychiatric hospital became rare due to well-organized prevention and treatment. In the original, third, periods of patients die most often from Sepsis. Very often in this period of the disease, epileptiform seizures are observed, which are also caused by death. In some cases, epileptiform seizures appear at the very beginning of the progressive paralysis and facilitate the recognition of the disease, when other symptoms are still expressed. During the progressive paralysis, brain strokes with subsequent hemiplegia may occur, for which, in contrast to paralysis, arteriosclerosis is characterized by short-term, since the lesion foci is insignificant here. Movements in paralyzed limbs are pretty quickly (in a few days, weeks) can recover.

The complete development of the clinical picture of the progressive paralysis is usually at the second period of the disease (the period of complete development of the disease).

The following main clinical forms of progressive paralysis are distinguished:

Manic, or expansive, form It is characterized by an increased, complacent mood (euphoria), against the background of which the patient expresses the ridiculous unstable nonsense of greatness (the patient says that he is a billionaire, he has all the reserves of world gold, he himself all out of gold, etc.). In behavior he is ridiculous, "distributes" non-existent wealth, often quinic, erotic. He considers himself healthy and satisfied with the stay in a psychiatric hospital.

Depressive form It is distinguished by the depressed mood, against the background of which the patient expresses ridiculous hypochondriage nonsense (he does not have a stomach, food falls directly into the bladder, etc.).

Dementian form It is characterized by increasing dementia with a decrease in the ability to critically evaluate the surrounding, express the right judgments, to understand its painful state. The mood, despite the severity of the state, continues to be euphorical, complacent.

Tabetical form It is characterized by a combination of symptoms of progressive paralysis with a spinal dry shot. This form is characterized by a gradual, slow flow.

Mounted form It has a fast, galloping malignant current, with a sharp motor excitation, with a confused and rapid decay of the psyche. This relatively rare form of progressive paralysis usually ends with death.

From the forms of progressive paralysis described above, as a result of the acquired syphilis, a relatively rare form of juvenile progressive paralysis, developing in the soil of congenital syphilis, is distinguished. It usually takes the type of dementary form. Pathologic changes in the brain in progressive paralysis are reduced to turbidity, sticking the brain shells, to the atrophy of the brainstorm with the death of nerve cells (mainly in the frontal fractions), to the brain water.

One of the first cases of undoubted progressive paralysis described J. Eskiller. In 1822, A. Beil allocated a progressive paralysis as an independent disease, emphasizing that it appears both mental disorders and neurological disorder associated with chronic inflammation of the cerebral cerebral shell. The approval of Beil met objections from some researchers. And only in the 50s of the 19th century, progressive paralysis was recognized as an independent disease.

Progressive paralysis suffers about 5% of all patients with syphilis (see the full arch of knowledge), and women are so much less often than men. The disease usually occurs after 10-15 or more years after infection. Thus, if we consider that the period of the greatest sexual activity falls for 20-55 years, the age of sick is most often 35-50 years. When infected with syphilis at a late age (45-60 years), the specified gap is usually shortened.

Clinical picture. Progressive paralysis - severe illness of the whole organism, and the most vivid manifestations - violations of mental activity. The main syndrome is the progressive total dementia (see the full arch of knowledge): the intellect is rude, the disorders of judgments are rather indicated, criticism disappears and especially self-criticism. There is no mind the disease, the memory sharply decreases, confabedions occur (see the full arch of knowledge: confability). The manifestations of dementia are amplified due to the frequently observed euphoria (see the full arch of knowledge: psychoorganic syndrome). Neurological symptoms lies in speech disorders, primarily in violation of articulation - dysarthritia (see the full arch of knowledge). It becomes unclear, lubricated, especially when pronuncated by long words, the patient misses or overstoves the syllables, does not pronounce the endings of words. The handwriting is done uneven, individual letters and syllables fall out of words. Voice timbre changes, it becomes deaf. The patient's face is inexpressive, the masculine, as the innervation of the muscles of the face is disturbed, blefaroptosis arises (see the full arch of knowledge: Ptosis). Tender reflexes are often improved and uneven, except for the cases of Taboparalych (see the full arch of knowledge). In connection with the sphincter disorders, patients do not hold urine and feces. As a result of vasomotor and trophic violations, edema appear, bone fragility, muscle atrophy, lying around.

Based on psychopathological manifestations, four stages of the disease are distinguished: latent (from syphilis infection before progressive paralysis), the stage of initial manifestations, the stage of complete development of the disease and the stage of Maja (see the full arch of knowledge). In the latent stage, headaches, dizziness, fainting states may occur, in some cases there are characteristic changes in the cerebrospinal fluid (see the full arch of knowledge). The initial manifestation stage is characterized by increased fatigue, irritability, weak. Patients complain of the decline of strength and reduced performance, although they can somehow carry out the usual work. Previously, such conditions due to external similarity with neurotic symptoms were incorrectly called preparalitic neurality. In some cases, in the stage of initial manifestations, depressive and delusional disorders are observed - anxiety depression with hypochondriam, anxious-armed depression, delusional ideas of jealousy, persecution, poisoning; As the symptoms are increasing, these endoform disorders disappear. Improving memory is very early. Separate actions indicate a violation of criticism. The sphere of deposits is upset, patients become voracious, erotic. The increase in these disorders is talking about the transition of the disease in the stage of complete development, which manifests itself in various clinical, forms. Expansive, or classical, form (previously widespread) is more common in men. It is characterized by the presence of a manifold excitation with the manifestation of amphibiousness, grand deliberation of greatness (see the full set of knowledge: nonsense). The dementary form is characterized by increasing dementia against the background of a lackless euphoria.

With depressive form, the depressed mood is developing, often with anxiety and desire for suicide (see the full assertion of knowledge: depressive syndromes), often observed is a ridiculous hypochondriatic nonginistic content. The circular shape, first described by S. S. Korsakov, proceeds with the change of states of arousal and depression. The hallucinatorium-paranoid form is characterized by the development of paranoid syndrome (see the full arch of knowledge) with mainly auditory hallucinations and prosecution. With a catatonic form, a stuporous state arises (see the full arch of knowledge) with phenomena of mutism and negativism (see the full arch of knowledge: catatonic syndrome). In the stage of Mazesma, conscious activities are stopped, the speech disappears, patients publish inseparal sounds, cannot stand and move. In this stage, they die from intercurrent disease.

By the flow, a particularly malignant-in-quality settled form (galloping paralysis) was isolated with a sharp motor excitation and a disturbance for the aimative type and the so-called stationary paralysis, in which the slow flow with a gradual decrease in intelligence and lethargy is noted.

Atypical forms, progressive paralysis are youthful and senile progressive paralysis, as well as paralysis of Lisouere and Taboparalich (see the full arch of knowledge). Youth 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 innate syphilis, in other cases it arises in children who were considered to be healthy. Most often flows in a dementary form; We often observe local symptoms, for example, atrophy of the visual nerves. Older progressive paralysis arises over the age of 60 years and is characterized primarily by a long latent stage (up to 40 years). Clinical, the picture resembles senile dementia (see the full arch of knowledge) with pronounced memory disorders; Sometimes the disease flows along the type of Korsakovsky syndrome (see the full arch of knowledge).

Paralysis of Lisouera and Taboparalini are characterized by relatively slow increase in dementia. With paralysis of Lisoueer, there is a tendency to the local striking brain, predominantly dark shares, and aphasia develop (see the full knowledge of knowledge), agnosia (see the full arch of knowledge), apraxia (see the full vault of knowledge), apochlectiform and epileptiform seizures. Taboparalic is a combination of symptoms progressive paralysis and spinal dry house (see the full vault of knowledge), and the spinal disorders precede the development of symptoms progressive paralysis, usually its dementary form.

Etiology and pathogenesis. In 1913, X. The legs proved syphilitic etiology progressive paralysis, discovering a pale trepony in brain tissues.

Pathogenesis is not studied enough. K. Levaditi on the basis of observations when the progressive paralysis was ill with both spouses or several persons infected with syphilis from the same source, put forward an assumption about the existence of a special neurotropic treponem, which, however, was not proven. Perhaps the pathogenesis plays the role of violation of the body's reactivity (see the full arch of knowledge) with the sensitization of the tissue of the brain, as a result of which, in some cases, the brain penetrates penetrate.

Pathological anatomy. Macroscopically, with progressive paralysis, atrophy of the intrigues of the brain and the expansion of the furrow is noted. The soft sheath of the brain is cloudy, fucked with a brain substance; Edendatitis is observed (see the full set of knowledge: chorioependatimitis). The microscopically reveals the inflammatory process in the soft shell of the brain and in vessels, especially small. Near the vessels is characterized by plasma cell infiltration. In the brain tissue there is a launch of the crust due to the destruction of nerve cells and fibers. In neuroglia, gliose fibers grow, laughter and amosoid cells appear. Pale treponams are found near the nervous cells.

Diagnosis in the stage of complete development of the disease usually does not cause difficulties. It is established on the basis of psychopathological, neurological manifestations and data laboratory research. The rise of the phenomena of total dementia with the absence of criticism, dysarthria, violations of pupil reactions (see the full arch of knowledge: Argailla Robertson Syndrome), consistency of serological indicators - all this in the aggregate makes the diagnosis of progressive paralysis reliable. The reaction of the vasserman in the blood is in most cases positive (see the full set of knowledge: Vasserman reaction); In a cerebrospinal fluid, it, like protein reactions (Nonn - Apert, Panda, Weikhbrodt), is sharply positive (see the full arch of knowledge: coagulative samples, cerebrospinal fluid). The number of cell elements in the cerebrospinal fluid is increased, sometimes significantly. The total protein content is increased. When the Lange reaction is set (see the full arch of knowledge: the cerebrospinal fluid) marks the discoloration of the fluid in the first 4-6 tubes and an increase in the intensity of the color in the following (the so-called paralytic curve). The reaction of immobilization by Treplem (see the full arch of knowledge: nonsense - Meyer reaction) and the immunofluorescence reaction (see the full vault of knowledge) are sharply positive in the blood and in the cerebrospinal fluid.

Differential diagnosis is carried out with the syphilis of the brain (see the full set of knowledge: syphilis), in which dementia is lacuniary, with a greater or less conservation of criticism, halucinations are more often observed; Lange reaction curve has a so-called syphilitic prong. Progressive paralysis

differentiate with alcoholic pseudo-pump (see the full set of knowledge: alcoholic encephalopathy) and senile dementia (see the full arch of knowledge) on the basis of serological indicators; With brain injuries (see a full arch of knowledge), especially the frontal share, in neurological symptoms and serological indicators. The pseudo-paralytic syndrome in brain tumors is accompanied by increased intracranial pressure (see the full arch of knowledge: hypertensive syndrome). Psychoses of vascular genesis differ from progressive paralysis by the development of lacunar dementia (see the full set of knowledge: atherosclerosis), which is not characterized by progressive paralysis, the presence of paralytic dementia allows to deliver the circular form of progressive paralysis from manic-depressive psychosis (see the full arch of knowledge), and the hallucinator-paranoid shape - From schizophrenia (see the full arch of knowledge).

Treatment. Pyroterapy is shown (see the full arch of knowledge) - infectious therapy and pyrogenic substances (see the full arch of knowledge) in combination, as a rule, with antibiotic therapy. Back in 1845, V. F. Sabler noted the beneficial effect of a number of feverish diseases during psychosis. The priority of psychosis infectious therapies belongs to A. S. Rosenblum, who treated mentally ill by vaccination of return title. In 1917, the Vienna Psychiatrist Y. Wagner Yauregg proposed treatment of patients with progressive paralysis by vaccinations of malaria. The method got widespread; It lies in the introduction of a progressive progressive paralysis of blood, taken from a patient with three-day malaria. The incubation period lasts 4-20 days, the first attacks proceed by the type of three-day malaria, in the future, as a rule, are observed daily. After 10-12 attacks, they are fed by the reception of quinine hydrochloride. Inf. Therapy is also carried out, infecting patients with progressive paralysis by European and African returning typhoid. The results of this therapy are less pronounced, but it is convenient, as the vaccination material, previously obtained as a result of the contamination of the mouse, can be transported over long distances. A somatic weakened patients vaccinate the Japanese rat tit. In cases where the infection is not possible, as well as during somatic contraindications, pyrogenic substances are prescribed (pyrogenal and others).

Since 40 years of the 20th century, comprehensive treatment is carried out - pyroterapy in combination with the introduction of antibiotics. At the same time, 40,000,000 units of Penicillin are prescribed on the course of treatment; Penicillinotherapy repeated courses are carried out at intervals 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 (instigation of malaria) is recommended, which contributes to the penetration of antibiotics into the brain tissue, and besides, to increase the protective forces of the body. Inf. Therapy is contraindicated in high age, with marason, heart failure, aortic aneurysm, kidney diseases, diabetes mellitus. In addition, treatment with one penicillin has been developed. Several pecticillin treatment schemes were proposed, but some researchers consider the use of one penicillin insufficient.

Forecast. The duration of the flow of the untreated progressive paralysis from its initial manifestations to the fatal outcome is on average about 2½ years. Youth progressive paralysis flows slower (5-6 years), stationary paralysis - up to 20 or more years. The mounted form ends with the death of the patient in a few months. With expansive form, long-term remissions are observed. Remissions after treatment have a different duration. Remissions are known over 20 years. According to a number of researchers, remission is more resistant.

Prevention consists in timely treatment of syphilis, which is carried out by specific and non-specific means on certain schemes (see the full set of knowledge: syphilis).

Social and forensicity. Only in isolated cases (persistent remission after treatment) can be resolved the patient to return to the previous professional activities. With a forensic psychiatric examination (see the full arch of knowledge) of the patient almost in all cases establish insaneness (see the full arch of knowledge). Only with the treated progressive paralysis with a steady remission, a duration of at least 3 years of patient can be declared.

Are you categorically not satisfied with the prospect of irretrievably disappear from this world? You do not want to finish your life path in the form of a disgusting rotting organic mass devoured by the grave worms in it? Would you like to return to youth to live another life? Start all over again? Fix mistakes? Do unstable dreams? Follow this link: