Progressive paralysis — the late form of syphilis proceeding in the form of the progressing inflammatory and dystrophic total defeat of cerebral structures. Differs in staging of development, the pseudo-neurasthenia symptomatology, then mental deviations against the background of intellectual decrease, at the final stage – heavy dementia is observed in the beginning. Diagnostics is carried out on the basis of the conclusions of the neurologist, psychiatrist, results of blood tests and a likvor on syphilis given to cerebral MRT. Treatment is performed by in a complex pyrogene, antitreponemny, psychocorrective, neurometabolic medicines.
Progressive paralysis is a separate nosological form of late neurosyphilis. Represents a syphilitic encephalomeningitis — inflammatory defeat of covers and a parenchyma of a brain. The disease was in detail described in 1822 by the French psychiatrist Antawn Beyl in honor of whom in neurology the name got Beyl's disease. In 1913 the Japanese pathologist of X. Noguchi proved a syphilitic etiology of a disease, having found pale treponema in brain tissue of patients. Progressive paralysis occurs at 1-5% of patients with syphilis. The time span of development of symptomatology lies ranging from 3 up to 30 years from the moment of infection, makes 10-15 years more often. The peak of incidence is the share of an age interval of 35-50 years. In modern neurologic practice Beyl's disease is rather rare pathology that is caused by timely diagnosis and treatment of syphilis.
Reasons of progressive paralysis
As Etiofaktor of a disease the causative agent of syphilis — a pale treponema acts. The sick person, the main way of infection — sexual and injection becomes a source of infection. Transfer of the activator through saliva is possible. The drift of the infectious agent in cerebral fabrics occurs in the hematogenic and limfogenny way. The reason of a long persistention of the activator with which the progressing paralysis is connected is authentically unknown. The probable contributing factors are absence or insufficiency of correct treatment of early forms of syphilis, hereditary predisposition, alcoholism. The craniocereberal trauma, easing of immunity against the background of sharp and chronic somatic diseases are considered as the triggers provoking activation of an infection.
The mechanism of activation of an infection is not clear. Assume the constraining role of the immune system blocking reproduction of the activator. Violations of reactivity of an organism, immunosupressivny states lead to distribution of a pale treponema in cerebral fabrics. In an initial stage progressive paralysis is characterized by inflammatory process, covering all brain structures — pan-encephalitis, then inflammatory changes are succeeded by atrophic.
Turbidity of a soft cerebral cover, its union with the subject brain parenchyma is macroscopically noted. It is typical ependimit — an inflammation of a vystilka of ventricles of a brain. Hydrocephaly, a diffusion atrophy of bark with expansion of furrows between crinkles is observed. The microscopic picture is presented vaskulity small cerebral vessels with plazmotsitarny perivaskulyarny infiltration, wrinkling and death of neurons, demiyelinizatsiy and atrophic changes of nervous fibers, growth of a glia, emergence of amyobevidny cages.
The clinical symptomatology is variable, poorly shown in a disease debut, changes and aggravated in process of progressing. The understanding of a stage of a course of process is necessary for selection of the most effective therapy. In this regard in neurologic practice progressive paralysis is subdivided into three clinical periods:
- Initial. Corresponds to the beginning of clinical symptomatology. Symptoms of a neurasthenia, a sleep disorder, decrease in working capacity are characteristic. The initial stage proceeds from several weeks to 2-3 months. By the end of the period changes of the personality with a tendency to loss of ethical standards become obvious.
- Development of a disease. The accruing personal changes, disorders of behavior, violation of the speech, gait and handwriting, intellectual decrease prevail. Trophic violations, dystrophic changes of somatic bodies are typical. Without performing treatment the stage lasts 2-3 years.
- Dementia. The progressing disintegration of the cognitive sphere is followed by apathy, full loss of interest in surrounding. Epileptic seizures, strokes are often observed. Trophic frustration are aggravated, fragility of bones increases. The patient perishes from a stroke, interkurrentny infections.
Sindromologichesky approach is the basis for the following classification. Taking into account the prevailing simptomokompleks the whole list of options of a disease is selected. The following clinical forms act as the main of them:
- Dementny (simple) — the accruing weak-mindedness with prevalence of euphoria. It is most widespread. Is not followed by the expressed mental disorders.
- Expansive — the expressed excitement with prevalence of crazy thoughts of own greatness, wealth. Euphoric - the complacent mood sharply is replaced by irascibility. Typically mnogorechy, the criticism is completely lost.
- Euphoric — the progressing dementia is followed by the fragmentary ideas of greatness. Unlike an expansive form proceeds without the expressed psychological excitement.
- Depressive — weak-mindedness progresses against the background of ridiculous ipokhondrichesky complaints. It seems to patients that at there are no interiors that they already died. Elements of depersonalization, a derealization up to a Cotard delusion are characteristic.
- Agitated — excitement is sharply expressed, tendency to aggression is noted. Visual, acoustical hallucinations are observed. The consciousness confused occurs bystry disintegration of the personality.
- Circulator — is similar to displays of bipolar disorder. The period of maniacal excitement is replaced by a heavy depression. There is an opinion that this form is implemented against the background of genetic predisposition to mental diseases.
- Hallucinatory and paranoid — psychosis with prevalence of a hallucinatory syndrome, nonsense of prosecution, a catatonia. It is suggested about emergence of this option of a disease at patients with a schizoid psychopathia.
Beyl onset of the illness in the middle age is the most typical. The progressive paralysis demonstrating at children's and advanced age differs in a number of features. Respectively allocate 2 age forms of a disease:
- Juvenile. Debuts at the age of 6-16 years after the postponed congenital syphilis which resulted from transplacentary infection. Proceeds in the form of a dementny form with Getchinson's triad more often (keratity, relative deafness, defeat of the top cutters). The delay of mental development can precede a demonstration of symptomatology.
- Senile. The debut falls on the period after 60 years. Typically long asymptomatic current. Development of a korsakovsky syndrome is possible. It is extremely difficult to differentiate a senile form from senile dementia.
Symptoms of progressive paralysis
The disease begins the nonspecific, slowly accruing symptomatology similar to neurotic manifestations. Patients complain of the lowered working capacity, unusual fatigue, irritability, headaches, a memory impairment, sleep disorders. Over time relatives of the patient notice changes in his personality: indifference to problems of people around, loss of feeling of a step, a behavior exit for a framework of ethical standards. Patients begin to make gross blunders in habitual professional activity, when progressing symptoms lose ability to realize them. Frustration of a dream are aggravated: in the afternoon the gipersomniya, at night — sleeplessness is noted. There are violations of food behavior (anorexia, bulimia). The speech accelerates excessively or slowed down. The criticism to the behavior decreases.
At a stage of development of a disease progressive paralysis is followed by aggravation of personal and behavioural frustration. The inadequate behavior, ridiculous acts, flat jokes comes to light. Mental violations differ in polymorphism, euphoria, a depression, the crazy ideas, hallucinations, depersonalization are found. In the speech there are difficulties of a pronunciation of compound words, then the dizartriya is formed — the speech becomes muffled, greased with the admission of separate sounds.
Gait is uncertain, stirred up, handwriting uneven, the dysgraphia with the admission of letters is noted. Faints, epileptic attacks are observed (is more often as dzheksonovsky epilepsy). Violations of appetite become the reason of weight loss or a set of body weight. There are trophic frustration: decrease in turgor of skin, fragility of nails, easy infection with development of furuncles, abscesses. The increased fragility of bones, dystrophic changes of heart (cardiomyopathy) and a liver is characteristic ().
In the period of dementia there is an increase of weak-mindedness up to marasmus. The patient ceases to serve himself, to interact with people around, to randomly control function of pelvic bodies. The refusal of meal leads to the expressed weight loss. The dysphagy — disorder of swallowing is sometimes noted. Numerous trophic ulcers are formed, the increasing fragility of bones becomes the reason of changes.
Cerebral accompanying Beyl's disease vaskulit is the reason of frequent ONMK. As a result of the had stroke resistant neurologic deficiency is formed: paresis, aphasia, apraxia. Trophic changes lead to decrease in barrier function of skin with development of a piodermiya, abscesses, at later stages — decubituses. Pneumonia, an inflammation of urinary tract (cystitis, pyelonephritis) is possible. Penetration of infectious agents into blood causes developing of sepsis. Strokes and infectious complications are the most frequent reason of a lethal outcome.
It is the most difficult to diagnose a disease in the initial period. Certain patients have no anamnestichesky data on existence of syphilis in the past that also complicates definition of a nosology. The diagnosis is established at laboratory confirmation of a syphilitic etiology. Enter the list of necessary inspections:
- Neurologic survey. In a stage of a debut pay attention to an easy anizokoriya, paresis of glazodvigatelny muscles, asymmetry of reflexes, instability, unsteadiness of gait, monotony of the speech. In the symptomatology heat the neurologist reveals decrease in photoreaction of pupils, a dizartriya, the expressed cognitive dysfunction. The paresis, aphasia and other focal symptoms caused transferred ONMK are possible.
- Neuropsychological testing. It is carried out by the psychiatrist, the neuropsychologist. Includes assessment of cognitive abilities and the mental status. Dementia, lack of criticism, psychomotor excitement, depressive , hallucinatory, crazy syndromes is characteristic various expressiveness. Perhaps twilight consciousness.
- Laboratory diagnostics. Treponemny blood tests (Wasserman's reaction, the RPR test) are positive practically at 100% of patients. In doubtful cases repeated analyses in dynamics, RIF, RIT are in addition carried out.
- Research of tserebrospinalny liquid. Likvor receive by a lyumbalny puncture. The analysis reveals the increased amount of protein, positive globulinovy reactions, at the expense of lymphocytes. Lange's reaction gives a specific form of a curve in the form of a tooth. At most of patients reactions the REEF and RIBT with a likvor are positive.
- Brain MRT. Depending on a phase of a disease reveals the diffusion inflammatory or atrophic changes affecting covers, substance of a brain and an ependima of ventricles. Scanning allows to exclude existence of volume education (an intracerebral tumor, brain abscess), to carry out a difdiagnostika with the progressing degenerate diseases.
The initial stage of a disease of Beyl should be differentiated from a neurasthenia. The features of the neurologic status, existence of epileptic episodes noticed by relatives early changes of the identity of the patient can help. Further the difdiagnostika with mental disorders, degenerate cerebral processes (vascular dementia, Alzheimer's disease, a multisystem atrophy, the Peak disease) is carried out. Positive treponemny reactions of blood and tserebrospinalny liquid act as the most reliable criterion confirming progressive paralysis.
Treatment of progressive paralysis
Etiotropny and psikhokorregiruyushchy therapy is necessary for patients. The medical effect of high temperature of a body was experimentally found. Modern treatment includes four the main components:
- Pyrotherapy. Earlier it was carried out by introduction of causative agents of malaria with the subsequent antimalarial therapy. Now pyrogene medicines are used.
- Antibacterial therapy. Protivotreponemny effect antibiotics of a penicillinic row, , quinine, bismuth nitrate have. Etiotropny therapy is begun with high doses, continued 2-3 weeks. In parallel appoint a probiotics, polyvitaminic medicines, if necessary — gepatoprotektor.
- Psychotropic treatment. It is shown in the second phase of a disease of Beyl. Individual selection of pharmaceuticals (sedative, neuroleptics, anti-psychotics, antidepressants) is carried out according to symptomatology.
- Neyrotropny therapy. It is directed to improvement of metabolic processes in a brain and restoration of its functions. Includes pharmaceuticals of vascular, nootropic, neurometabolic action. In dementia stage neyrotropny treatment does not render essential effect.
Forecast and prevention
Results of treatment depend on timeliness of its beginning. In 20% of cases correct therapy allows to achieve almost complete recovery of health. At 30-40% of patients it is possible to reach permanent remission with the moderate deviations of mentality allowing to adapt in society. In 40% of cases persistent organic dementia remains. Process of sanitation of a likvor takes up to 3-4 years half a year. Progressive paralysis allows to prevent respect for personal hygiene, refusal of casual sexual communications, the unprotected sexual intercourses. At for the first time the established syphilis correct treatment before full sanitation of biological environments is necessary. In the subsequent observation and periodic inspection for early identification of late forms of syphilis is recommended to the patient.