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|ICD-10||A 52.1 52.1|
|ICD-9||090.40 090.40 094.1 094.1|
Progressive paralysis, paralysis dementia, paralytic dementia or pain of belle - psychoorganic disease of syphilitic origin, characterized by progressive mental disorder with the formation of a persistent defect up to dementia in combination with neurological and somatic disorders. It was first described by the French psychiatrist A. L. Beyle in 1822: 26. The term "progressive paralysis" is suggested by Delane. For the first time, the possibility of syphilitic damage of the meninges and brain substance was discovered by Lalleman in 1834 when the patient who died of syphilis was dissected. The evidence of the syphilitic nature of the disease was the discovery of pale treponema in the cerebral cortex of patients with progressive paralysis Pyatnitsky in 1911 and the American scientist of Japanese origin H. Noguchi in 1913. Progressive paralysis occurs with a peak in the incidence of 10–12 years after infection in 1–5% of people with syphilis: 398, therefore at a mature age (30–50 years), it is twice as common in men and proceeds more malignantly than brain syphilis, leading to severe low reversal.
In neurosyphilis, progressive paralysis (in case of primary brain damage) and brain syphilis (in case of primary damage to blood vessels and shells of the brain) are distinguished as separate nosoological forms: 394. Progressive paralysis and syphilis of the brain differ in the clinical picture, the time of onset of the disease, as well as the nature and localization of the pathological process: 394.
Before using penicillin, patients with progressive paralysis accounted for 5 to 10% of all patients in psychiatric hospitals, and malaria therapy was the only therapeutically effective treatment. For the development of this therapy, Wagner-Jauregg in 1927 received the Nobel Prize in Physiology and Medicine.
What are the signs of paralysis?
The first signs of central brain paralysis are elevated tendon reflexes, muscle hypertension and painful reflexes. There is another name for central paralysis - spastic, named for hypertension or muscle spasticity. It feels the muscles are always tight, tense, and with inert movements, there is a clear reaction, which is sometimes hard to beat.
This state represents the consequences of raising the reflex tone and is divided, as a rule, unevenly. The result is typical contractures. The general picture of brain paralysis is typically the same: the fingers, the hand, and the arm itself are bent. The leg is unbent at the knee and pelvic area, the foot is tilted inward. Hyperreflexia is also considered a manifestation of intense, self-acting work of the spinal cord. In contrast to tendon reflexes with central paralysis, the sensitivity of the skin does not increase, but disappears or decreases.
Concomitant movements appear in the paralyzed limbs involuntarily, mainly when healthy muscles are tense. There are several causes of synkinesis that explain muscle work during brain paralysis. For example, if you use a healthy hand for a handshake, the paralyzed hand performs the accompanying reflex flexion. The same flexion occurs when sneezing or coughing.
With the above factors in the paralyzed leg involuntary unwinding is noticed. If in the supine position the patient puts aside a healthy leg, then the paralyzed leg involuntarily is also retracted. Painful reflexes in the facial part are inherent to central paralysis, they are the result of a bilateral lesion of the cortical, subcortical or stem sections. Already with an external examination of the patient can detect defects in the muscles and motility.
In a conversation with the patient, one can detect facial paralysis and speech disorder. Inherent tremor, convulsive twitching and other unpleasant conditions. As a rule, doctors will certainly check the patient's walk, which is likely to be upset. The front part of the patient is examined, starting with the forehead (wrinkling up), the connection of the eyelids, the movement of the eyes, the opening of the mouth, abduction of the corners of the mouth to the outside, extension of the tongue.
Then the boundaries of turning the head to the sides, shrugging the shoulders, raising the arms horizontally and vertically, their extension and flexion, the work of fingers and hands are determined. After that, the slopes of the body, walking on the toes and heels.
Movement control may be impaired due to lesion of the cerebellum and with loss of coordination of movements (loss of sensation of location and movement). Abruptly developing brain paralysis can result from encephalitis, hemorrhage, stroke, head injuries, infections transmitted by parasites, inflammation of the brain. Also, this disease may be a consequence of epilepsy. Detection of a specific degree of susceptibility pathology in the body makes it possible to more specifically determine the affected area of the spinal cord.
When forming the lower paralysis, it is necessary to immediately eliminate the compression of the spinal cord. Other causes of spinal cord injury are sarcoidosis, vascular disease, ischemia of the anterior cerebral artery, neurosyphilis, thrombosis of the upper sagittal sinus, tumor, hydrocephalus. The consequence of brain damage are also depression, drowsiness, confusion, cognitive impairment.
Expert Editor: Pavel Alexandrovich Mochalov | D.M.N. general practitioner
Education: Moscow Medical Institute. I. M. Sechenov, specialty - “Medicine” in 1991, in 1993 “Occupational diseases”, in 1996 “Therapy”.
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Beyle disease is diagnosed in 5% of patients with syphilis. Epidemiology indicates that women suffer two times less often than men. The only source of infection is man. The frequency of infection depends on the duration of the disease. The pattern of spread of the disease is associated with low awareness of infection methods and risk factors for pathology.
The most infectious are patients with early forms of syphilis from 3 to 5 years. Late forms, the term of which is more than 5 years low-contagious. The main danger of the development of neurosyphilis is that in the initial pores the disorder is asymptomatic. In most cases, this is due to the high resistance to pale treponema, which leads to the appearance of the encystified and L-forms of the pathogen.
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Causes of Progressive Paralysis
The main causes of progressive paralysis are associated with positive reactions to syphilis. Pale treponema causes damage to the lining of the brain. The disorder develops in 1 of 5% of patients with syphilis, men are more susceptible to the disease. The average age of patients from 30 to 50 years. This confirms the fact that the pathology develops 10-15 years after infection.
The causes of progressive paralysis may be non-specific. Traumatic brain injury, emotional distress, stress, reduced protective properties of the immune system, and late treatment of infectious pathologies can also trigger a psycho-organic disorder. Very often, the true nature of the deviation is established in a psychiatric hospital. That is, the patient may not suspect the presence of syphilis before the appearance of neurological symptoms.
After establishing the cause of paralysis, the patient is referred to a number of specialists, including the venereologist, who conducts additional diagnostic tests. They are necessary to determine the stage of the disorder and plan a further treatment.
There are such risk factors for developing the disease:
- Sexual - the most common variant of infection. The bacterium penetrates through the lesions on the skin and mucous membranes. Infection is possible through contact with syphilitic gumma or a solid hinge, that is, in contact with an open source of infection.
- Blood transfusion - infection during blood transfusion from an infected donor, during surgery or dental procedures (this risk factor is minimal, since all donors and blood products are tested for syphilis, and all surgical instruments are sterile).
- Transplacental - the disease can be transmitted from a pregnant woman to a child during gestation or during the birth process. The risk of infection is associated with possible injuries and breaks in the skin and mucous membranes, which act as entry gates for infection.
- Contact and household - the use of common household items with the infected (towels, dishes, linens, clothing, hygiene items).
- Professional - this risk factor is present in health care workers who come into contact with body fluids (saliva, blood, semen). Infection is possible at necropsy or childbirth.
Regardless of the factor that provoked the disease, the causative agent of neurosyphilis disappears into the lymph nodes and spreads throughout the body through the bloodstream.
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The main causative agent of progressive paralysis is the bacterium Pale Treponema. Pathogenesis, that is, the mechanism of disease development is most often associated with sexual factor.
There are two ways of penetration of bacteria into the substance of the brain:
- Lymphogenous - pathogenic microorganism penetrates the human body through mucous membranes or damaged skin. With the bloodstream, the infection spreads through the body, responding to it with the production of specific antibodies.
- Hematogenous - due to the weakening of the blood-brain barrier, the infection penetrates into the spinal substrate. First, the vessels and membranes of the spinal cord and brain are affected. This provokes the beginning of the inflammatory process with the release of exudate. Scar changes are formed in the brain tissue.
Over time, the body weakens and ceases to fight the harmful microorganism, opening access to the parenchyma of the central nervous system. As a rule, this occurs 5-7 years after infection with syphilis or at later stages. The bacterium enters the nerve cells and fibers, causing their pathological changes and the symptoms of neurosyphilis.
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Symptoms of progressive paralysis
Psychoorganic pathology has a wide variety of clinical manifestations, pronounced neurological and mental disorders. The symptoms of progressive paralysis depend on the stage of the disease, and it is not uncommon for there to be no clear boundaries between the various forms.
Beyle disease is characterized by impaired pupil shape and reactivity. The patient suffers from imbalances with closed eyes in a standing position, loss of sensitivity, progressive dementia.
The disorder most often occurs between the ages of 35 and 50 and has unspecified neurotic complaints. The developed stage is characterized by disorders of memory and attention. Also observed pathology in the emotional sphere, frequent mood swings, irritability, temper, depression. There may be muscle spasms in the limbs and other parts of the body, delusions and hallucinations.
After hitting the pale treponema in the spinal cord or brain, it begins its active reproduction. The first signs develop gradually and in a staged manner. The symptomatology of the disease is very similar to the signs of acute inflammation of the lining of the brain, that is, meningitis:
- Severe headaches and dizziness
- Increased intracranial pressure
- Noise in ears
- Digestive disorders
As soon as the infection penetrates the cerebral vessels, the following symptoms appear:
- Insomnia and Migraine Attacks
- Decreased tactile sensitivity
At the next stage, the spinal cord is damaged, which is manifested by the following symptoms:
- Disorders of the pelvic organs
- Weakening of the muscles of the lower limbs
- Reduced muscle and articular sensitivity
- Long healing skin wounds
- Constriction of the eye pupils and lack of response to light
- Movement Coordination
- Pain in the lower back
Due to the long-term presence of bacteria in brain tissue, their gradual destruction occurs. This is manifested by the following features:
- Progressive personality disorders
- Memory loss and thinking disorder
- Manic and depressive states
- Hallucinations, crazy ideas
In some cases, the disease provokes epileptic seizures and paralysis. Special attention during the diagnosis is given to the absence of pupillary reaction to light. Nasolabial folds acquire asymmetry, the handwriting changes, tendon reflexes become uneven.
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Juvenile progressive paralysis
A rare form of congenital syphilis that occurs during transplacental infection is juvenile progressive paralysis. The morphological basis of the pathology is the cellular abnormalities in the location of the nerve cells, oblageriruyuschy endarteritis of the cerebral vessels.
Pathological condition can manifest itself both in early childhood and in later age. In early childhood, defects in the structure of the bone apparatus (curvature of the limbs, saddle nose), poorly developed muscles, and damage to the cranial nerves can be detected. From 7–9, and in some cases from 13–15 years, neurosyphilis progresses rapidly, acquiring a demental form. There is a loss of acquired skills and emotional disorder. At 15–20 years, the disease is characterized by increasing dementia and epileptiform seizures.
There are several stages of pathology:
- Latent - asymptomatic stage with pathological changes in cerebrospinal fluid. Develops within 1 year after infection. Neurological disorders do not appear.
- Acute - manifested in the first 1-2 years after infection. Symptoms such as headaches, bouts of nausea and vomiting, and maculopapular rash are typical for this stage. Pathologies can also be observed from the side of sight, hearing and facial nerves. In rare cases, hydrocephalus and stagnation of the optic nerve discs develop.
- Meningovascular - can develop in 1-5 months after infection, but is most often diagnosed in 5-7 year of the disease. Manifested with symptoms of ischemic or hemorrhagic stroke. Concentric narrowing of large arteries and local narrowing / dilation of small arteries are observed. Before the ischemic crisis, the patient complains of frequent headaches, emotional lability, personality disorders.
- Meningomyelitis - at this stage, the lower spastic paraparesis is progressing with impaired pelvic organ function and deep sensitivity. Pathological symptoms may develop asymmetrically with signs of Brown-Sekara syndrome.
- Spinal troughs - the incubation period of this stage takes from 5 to 50 years, but on average appears 20 years after infection. At this stage, inflammatory infiltration and degeneration of the posterior roots and spinal cord canals is observed. The patient is faced with sharp shooting pains, a violation of deep sensitivity, and neurogenic disorders.Without treatment, these symptoms begin to progress.
- Paralytic dementia is a stage of progressive paralysis that develops 10-20 years after being infected with syphilis. The patient suffers from a depressive state, various severity of neurological disorders, reduced muscle tone, epileptic seizures, hallucinations. It is characterized by persistent progression, which is not rarely fatal.
From the moment of infection to the stage of progressive paralysis passes at least 8-15 years. The first signs of the disease do not cause concern, since they are similar to the usual depletion of the nervous system. Pathological factors contribute to the development of pathology, for example, chronic diseases, alcoholism, drug addiction.
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There are several clinical forms of neurosyphilis:
- Expansive - manic form is characterized by a state of euphoria and delusional, meaningless ideas. The patient thinks that he is successful and ascribes to himself non-existent wealth. The patient considers himself completely healthy and satisfied with being in a psychiatric institution.
- Depressive - depressed mood combined with absurd hypochondriacal delusions.
- Dementia - increasing dementia, a decrease in the ability to critically evaluate the environment, to express the right judgment and to perceive one’s condition. The mood is euphoric, complacent.
- Tabetic - at this stage with a slow and protracted course, a combination of symptoms of paralytic dementia and dorsal marrow is observed.
- Agitated - has a malignant course with pronounced bouts of motor excitation. There is confusion, rapid disintegration of the psyche.
All the above stages and forms can be accompanied by epileptiform seizures, cerebral strokes with hemiplegia and paresis, arteriosclerosis. Without proper treatment, each of the forms can be fatal.
Psychoorganic pathology has several stages of development, each of which is distinguished by its symptoms. Such stages of progressive paralysis are distinguished:
- Initial is the stage of preparative asthenia. It is characterized by cerebrastia, severe headaches, speech and articulation disorders, increased irritability.
- Deployed - paralytic dementia with a progressive decline in intellectual-mnestic qualities and various personality disorders.
- Terminal - total disintegration of mental activity, insanity.
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Complications and consequences
Neurosyphilis causes severe consequences and complications. Patients face problems such as:
- Communication Disorder
- Injuries from falling due to cramps
- Paresis of the limbs
- Atrophy of the optic nerve
Complications of this pathology can lead to disability. Launched forms are practically not amenable to treatment and threaten to be fatal.
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Diagnosis of progressive paralysis
Detection of Beyle's disease occurs on the basis of existing neurological disorders and mental disorders. Diagnosis of progressive paralysis consists of a complex of various clinical studies:
- History taking and analysis of patient complaints
- Lab tests
- Instrumental methods
- Differential diagnostics
During diagnosis, the patient may be sent to a mental hospital for observation. According to the results of all studies, the doctor makes the final diagnosis, determines the stage of the disease, the methods of its treatment. If progressive paralysis is diagnosed in the early stages, then this significantly improves the prognosis for recovery.
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If you suspect diffuse syphilitic meningoencephalitis with damage to the membranes, blood vessels and brain substances, laboratory diagnostics are shown. Analyzes are necessary to identify the causative agent of the disease - pale treponema or other abnormalities. The patient is prescribed the following studies:
- Wasserman reaction
- Blood and liquor tests
- Treponemal and non-treponemal tests with serum and liquor
- Precipitation microreaction with cardiolipin antigen
- Immunofluorescence Reaction (RIF)
- The reaction of immobilization of treponem (RHS)
The diagnosis of neurosyphilis is confirmed by positive test results for treponemal reactions in the study of blood serum and in the reaction of immunofluorescence. As well as changes in cerebrospinal fluid (Wasserman reaction), inflammatory changes in the cerebrospinal fluid with cytosis of more than 20 μl and protein above 0.6 g / l.
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Symptoms characteristic of Baile’s disease require extensive investigation. Instrumental diagnostics is necessary to confirm psychoorganic pathology. The patient undergo computed and magnetic resonance imaging.
These methods reveal nonspecific changes: heart attacks, gums, atrophy, increased contrasting of the meninges, multifocal lesions of the white matter and more. The results of these studies are used in the differentiation of the diagnosis. Also, the patient is sent to the oculist to examine the fundus, because the disease is characterized by visual impairment of varying severity.
The early stages of progressive paralysis are similar in their symptoms to many other neuropsychiatric disorders. Differential diagnosis helps to separate the true disease from other disorders.
Neurosyphilis differentiate with such pathologies:
- Tumor lesions of the frontal lobes of the brain
- Pick's disease
- Vascular diseases of the brain
- Serous meningitis
- Cerebral atherosclerosis
- Malignant forms of hypertension
Despite the pronounced symptomatology of the disease, the differential diagnosis is complicated by the fact that various brain lesions can give a picture of dementia, which is present in paralytic dementia. A crucial role in the diagnosis is played by anamnesis, somatic and neurological examinations, the results of serological reactions.
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Treatment of progressive paralysis
Symptoms of neurosyphilis require careful diagnosis and properly formulated therapy. Treatment of progressive paralysis takes place in the hospital. The treatment is engaged in a neurologist, venereologist, psychiatrist. The patient is prescribed a combination specific therapy, which consists of drugs (antibiotics, iodine and bismuth preparations) and physiotherapy.
For the treatment of specific infections of the nervous system, consisting in the penetration of the causative agent of syphilis in the nervous and brain tissue, can use the method of pyrotherapy. By pyrotherapy is meant the use of antimalarial drugs. The drugs are administered intramuscularly and intravenously. The incubation period is 4 to 20 days. Improvement of the patient’s condition is observed in a month.
The effectiveness of therapy depends entirely on the stage of the disease, the best results are shown by treatment at the first symptoms of the pathology. The results are assessed by the regression of signs of progressive paralysis and improvement of cerebrospinal fluid performance. The control of cure is carried out for two years every six months. With the appearance of new neurological symptoms, the growth of old or with cytosis in the cerebrospinal fluid, repeated therapy is indicated.
The main goal of the treatment of progressive paralysis is the destruction of the bacterium that caused syphilis. Medicines are prescribed to minimize the pathological neurological symptoms and reduce the epidemiological risk of the patient in relation to healthy ones. During treatment using techniques aimed at the destruction of pale treponema, as well as ancillary drugs and vitamins. The most complicated are the advanced forms of the disease, since they require long-term therapy and careful selection of drugs.
Treatment of psychoorganic pathology:
- Antibiotic drugs. Patients are prescribed drugs from the group of penicillins, because they have a wide spectrum of action. They suppress many harmful microorganisms by acting on the walls of their cells.
Antimicrobial agent from the class of β-lactam antibiotics. The main active ingredient is 6-aminopenicillanic acid. The drug is administered intravenously (intramuscular administration is ineffective), starting with high doses of 2-4 million ED 6 times a day for 10-14 days. In the first hours after the introduction, symptoms of acute fever, tachycardia, severe headaches and myalgia, aggravation of neurological pathologies may appear. As a rule, adverse symptoms regress within 24 hours. In severe cases, corticosteroids or NSAIDs are used.
Appointed with contraindications to penicillin drugs. Refers to bacteriostatic, representative of macrolides. By its antimicrobial action similar to penicillins. It is active against many harmful microorganisms, including the causative agent of Beyle’s disease. It has several forms of release: tablets, capsules, ointment, powder for intravenous and intramuscular injections. Dosage, as well as the form of release, depends on the stage of the disease and the characteristics of the patient. Side effects are rare and manifest as nausea, vomiting and gastrointestinal disorders. With prolonged use may develop resistance and allergic reactions. The drug is contraindicated in its intolerance and severe liver disorders.
Third generation cephalosporin antibiotic with bactericidal properties. Its activity is associated with inhibition of the synthesis of the cell wall of bacteria. It has a broad spectrum of antimicrobial action. It is used intramuscularly and intravenously in dilution with sterile water for injection. Side effects are manifested by the digestive system, allergic reactions are also possible. The drug is contraindicated in case of hypersensitivity to cephalosporins and penicillins, in the first trimester of pregnancy and in renal and hepatic failure. In case of overdose, a change in the blood picture is possible Symptomatic therapy is indicated for treatment.
- Antisyphilitic drugs. In the early stages of the disease are used as monotherapy, but most often they are prescribed in combination with other drugs.
It is used in all forms of syphilitic infection. Available in vials for intramuscular administration. The drug is administered in 1.5 ml 2 times a week, the course of treatment is 16-20 ml. Side effects are manifested in the form of increased salivation, inflammation of the mucous membrane of the gums, stomatitis. It is also possible neuritis of the trigeminal nerve and bismuth nephropathy. The drug is contraindicated in diseases of the kidneys and liver, diabetes, pathologies of the cardiovascular system.
In the treatment of neurosyphilis is used in combination with penicillin preparations. Effective with non-specific lesions of the central nervous system. Introduced intramuscularly, in a two-component way. Dosage 3 ml every 4 days, course dose 30-40 ml. Adverse reactions occur as dermatitis, kidney damage and stomatitis. It is contraindicated to use in case of kidney and liver disease, increased bleeding. Not prescribed to patients with severe tuberculosis and with hypersensitivity to quinine.
- Preparations for the elimination of neurological symptoms, improvement of metabolic processes and blood circulation in the brain.
The synthesis of dopamine in the brain increases, the amount of acetylcholine and the density of cholinergic receptors increase. It has a positive effect on metabolic processes and blood circulation in the brain, stimulates redox reactions, and improves regional blood flow. It is used for cerebrovascular insufficiency, changes in cerebral circulation, with injuries and brain intoxication, atherosclerosis and other neurological disorders.
The tool has several forms of release, therefore, is suitable for both oral and intravenous administration. Dosage and treatment depend on the severity of the pathological process. Side effects are manifested in the form of mental excitement, irritability, sleep disorders. Possible disorders of the gastrointestinal tract, convulsions, tremor of the extremities. Contraindications associated with individual intolerance to the components of the tool. Not used for diabetes and acute renal failure.
Drug from the class of nootropics. Improves cognitive processes in the brain, affects the central nervous system (improves the metabolism of neurons, changes the speed of the excitation process, improves microcirculation by stabilizing the rheological properties of blood). It does not have a vasodilating effect, but improves the characteristics of cerebral circulation.
It is used for symptomatic treatment of psychoorganic syndrome. Daily dosage is 160 mcg / kg body weight (divided into 2-4 doses). Side effects are manifested in the form of increased nervousness, depression, drowsiness, asthenia. Headaches, insomnia, hallucinations, gastrointestinal disorders, increased libido and allergic reactions are possible. Contraindicated in renal failure, hemorrhagic stroke and intolerance to pyrrolidone, piracetam and other components of Nootropil. In the case of overdose, there is an increase in adverse reactions, symptomatic treatment.
Expands blood vessels of the brain, improves blood flow and oxygen supply to the brain. Lowers systemic blood pressure. It is used for neurological and mental disorders caused by cerebral blood circulation disorders of different etiologies. It has several forms of release (tablets, solution for injection), which allows you to choose the most suitable. As a rule, the drug is well tolerated. Adverse reactions are possible with intravenous administration - hypotension, tachycardia. Not used for violations of heart rhythm, unstable blood pressure and low vascular tone. Subcutaneous administration and use with heparin is contraindicated.
The effectiveness of the treatment of progressive paralysis is assessed by regression or the absence of neurological symptoms, normalization of cerebrospinal fluid. Therapy is carried out under strict medical supervision with periodic testing to identify the causative agent in the cerebrospinal fluid. If the amount of the harmful microorganism is not reduced, then higher doses of drugs or selection of other drugs are indicated.
Syphilis of the brain requires complex treatment. Vitamins are part of the therapy used in all forms and stages of the disease. Consider what useful macro and microelements are necessary for neurosyphilis:
- B - vitamins of this group are involved in the processes of cellular metabolism, normalize the work of the whole organism. Useful for the nervous system, regulate energy metabolism, improve visual function and synthesize hemoglobin. Vitamins B6 and B12 regulate the nervous system, synthesize and regenerate red blood cells, create antibodies.
- A - represented by three bioorganic compounds from the group of retinoids (retinol, dehydroretinol, retinal). It has a powerful antioxidant effect, improves the growth and development of all cells, organs and tissues. Participates in the functioning of the mucous membranes and skin epithelium. Retinol is part of the visual pigment, which is located in the cells of the retina. A sufficient amount of it supports good vision. Improves the condition of hair, gums and teeth.It acts as a prophylactic agent of malignant lesions of the body, regulates the immune system, enhances the synthesis of immunoglobulins (antibodies) of all classes.
- E - fat-soluble compound with antioxidant properties. It prevents the formation of blood clots, improves microcirculation and prevents blood stasis. Improves blood properties and the functioning of the immune system. Reduces pressure, expands and strengthens the walls of blood vessels, prevents anemia and cataracts. Maintains the normal functioning of the nervous system.
- Tonic iron preparations - this category includes drugs with 30 mg or more of elemental iron. They are used as tonic and auxiliary agents for anemia, pathologies of the central nervous system, various inflammatory processes, lesions of the gastrointestinal tract, and abundant bleeding.
- Glycine is an amino acid with antioxidant, neurometabolic and neuroprotective properties. It regulates the metabolism, stimulates the processes of protective inhibition of the central nervous system, prevents and stops psycho-emotional stress. It is used for various disorders of the nervous system, sleep disorders, excessive excitability and emotionality, disorders of the autonomic system. Assigned to patients with ischemic stroke, as well as with encephalopathy of various origins.
- Nicotinic acid - has anti-pelagic properties, has a vasodilating effect, improves the functioning of the digestive tract. Reduces the level of lipoproteins in the blood. It is used for diseases of the vessels, brain, neuritis of the facial nerve, nonhealing ulcers and wounds, infectious and other lesions of the body.
All of the above vitamins are used only as directed by the physician, who determines the required dosage and frequency of their use.
In the late stages of progressive paralysis, that is, with a dorsal worm, physiotherapy is indicated. The patient is prescribed a limb massage, electrophoresis and Frenkel gymnastics.
Consider the main therapeutic physiotherapy:
- Massage - conduct several courses with short and intense sessions. This is necessary to restore the functions of the limbs, as well as to prevent complications of psychoorganic pathology.
- Electrophoresis is a method of delivering drug components to the body using an electric current. Medicinal substances penetrate the tissues through the ducts of the sweat and sebaceous glands. Electric current increases the synthesis of biologically active substances and increases ionic activity in conducting tissues. When neurosyphilis use the drug Lidaza.
- Frenkel gymnastics is a system of exercises performed on a slow repetition with a gradually increasing complexity. Exercises are designed to treat and prevent movement regulation disorders. This technique is based on the activation of an intact sensory mechanism (sight, touch, hearing), compensating for the kinetic losses.
Physiotherapy treatment is carried out under medical supervision in sanatorium-medical institutions.
In diffuse syphilitic meningoencephalitis with damage to the membranes, vessels, and brain parenchyma, timely diagnosis and properly selected therapy are necessary. Folk treatment of progressive paralysis is not recommended, as it is ineffective in the fight against treponema pallidum. But despite this, there are a number of popular recipes recommended for neurosyphilis, consider them:
- Chop 500 g of aloe with a meat grinder and mix with 250 ml of liquid honey. Therapeutic agent should infuse in a dark cool place for 2-3 days. After that, mix it with ½ cup of red wine and again put in a cool place for 1-2 days. The tool can be stored in the refrigerator and take 1 spoon 2-3 times a day.
- Crush 2-3 apples, add to them a glass of hawthorn and crushed rosehips. To the mixture, add 5-8 cloves of chopped garlic and pour all 2 liters of boiling water. The tool should be infused for 2-3 hours, then it should be drained and taken 3 times a day, half an hour after meals.
- Crush the onion and squeeze the juice. Mix onion juice with honey in a 1: 2 ratio. The tool must be taken 3-4 times a day 30 minutes before meals.
- 100 g of dry and chopped straw of oats pour 500 ml of boiling water and let it brew until it cools. As soon as the infusion has cooled, strain it and take ½ cup throughout the day. This medicine has a tonic effect and tones the body.
Before using non-traditional methods of treatment, you should consult with your doctor. This is due to the fact that some recipes can cause adverse reactions that worsen the patient’s condition and the course of the underlying disease.
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Herbal ingredients with healing properties are used in many diseases. Herbal treatment of progressive paralysis is possible in its early stages and only in combination with drug therapy. Consider effective herbal remedies:
- 20 g of dry crushed roots of sedge pour 500-750 ml of boiling water and boil on low heat until the liquid boils down twice. Broth must insist 2 hours and strain. Take ¼ cup 3-4 times a day.
- A couple of tablespoons of dry grass field field, pour 250 ml of boiling water and let it brew for 4 hours in a sealed container. After cooling, the infusion should be filtered and take 1 teaspoon 4-5 times a day.
- A tablespoon of burdock root pour 250 ml of hot water and boil in a water bath for 20 minutes. Once the liquid has cooled, it must be drained and take 1 spoon 3-4 times a day.
The duration of the application settings and decoctions should be monitored by the attending physician. Independent use of unconventional methods, with Beyle's disease is not recommended.
Treatment of diseases by affecting the body of such pathological factors is homeopathy. For progressive paralysis, drugs diluted in water in a ratio of 1:10 or 1: 100 are used. Alternative therapy is aimed at activating the hidden resources of the body to combat the disease. But to achieve a positive result is possible only with the use of substances that cause symptoms characteristic of syphilis.
Algorithm of homeopathic therapy:
- At the initial stages of the disease of Beyl, Mercury sublimate of Corosivus is used, 5-6 drops 3-4 times a day for a week.
- A week later, the therapy is supplemented with Nitri acidum in a dosage of 4 drops 3-4 times a day.
- After this, it is necessary to start monotherapy with the mercury preparation Mercurius biiodatus, taking Nitri acid every other day. Such treatment should continue until the disappearance of pathological symptoms.
- At advanced stages of neurosyphilis, iodine preparations are recommended. Patients are prescribed Kali iodate 11 to 10 drops at the beginning of treatment and up to 20 drops by the end of therapy. This remedy reduces, and in some cases completely eliminates the symptoms of the disease.
- In case of plaque typical for syphilitic infection on mucous membranes and skin, rinsing is carried out with Fitolacca decandra. If there is a runny nose of a syphilitic nature, then Kali bichromicum is recommended.
- With pathological lesions of the nerve centers - Kali iodatum with Aurum muriathicum natronatum and Aurum iodatum.
- In some cases, herbal remedies are used to treat brain syphilis: Corydalis formosis and Sarsaparilla.
All homeopathic preparations, their dosage and duration of use, are determined by the attending physician. Independent use of the above drugs is contraindicated, as they can significantly aggravate the course of the disease.
Progressive paralysis therapy involves the use of conservative techniques. Surgical treatment of neurosyphilis is not carried out. Surgical intervention is possible with pathological complications of the disease, requiring surgery.
In other cases, patients are prescribed a complex of antibiotics sensitive to treponema, pyrotherapy and physiotherapy (treatment with fever). To assess the cure is carried out rehabilitation of the cerebrospinal fluid and the involution of clinical symptoms of pathology.
Until the middle of the XX century, this disease was not considered as an independent nosology, it was mistakenly interpreted as the consequences of the epidemic encephalitis that was widespread at that time. The fact is that a large number of cases of polymorphic postencephalitic parkinsonism masked more rare pathologies that were considered atypical forms. And in the absence of any data for neuroinfection in the history, it was assumed that the patients had transferred encephalitis in the erased (subclinical) and non-diagnosed form.
Progressive supranuclear palsy as an independent neuropathology was identified in 1963-1964. a group of Canadian doctors: the neurologists J. Steel and J. Richardson and the pathologist J. Olszewski. They described and analyzed 7 cases of neurodegeneration with a characteristic clinical picture. In the USSR, progressive supranuclear palsy was first mentioned in 1980 by doctors of the Nervous Disease Clinic of the Moscow Medical Academy. THEM. Sechenov, who observed two patients.
Subsequently, the disease continued to be studied, it was allocated in the domestic and world classifications as a separate nosological unit. In ICD-10, progressive supranuclear palsy refers to diseases of the nervous system (section extrapyramidal and other motor disorders, a subsection of other degenerative diseases of the basal ganglia), encoded by G23.1.
According to modern medical statistics, progressive supranuclear palsy is the cause of 4-7% of cases of diagnosed parkinsonism. But even now some of the patients with this disease have erroneous diagnoses, especially in the early stages. The overall prevalence of progressive supranuclear palsy in the population is on average 5 cases per 100 thousand of the population, from 1.4 to 6.4 cases in different countries.
The disease is detected mainly in people of the older age group, even hereditary forms usually occur at the age of 50 years.
Etiology: why does this disease develop
The reasons for the development of progressive supranuclear paralysis are not reliably known. This disease is not associated with any infections, injuries or exposure to external adverse factors.
Progressive supranuclear palsy is considered sporadic pathology (manifested unsystematically in the population). At the same time, since 1995, information has been published on the presence of rare familial cases of the disease with an autosomal dominant mode of inheritance. This variant of the disease is associated with a heterozygous mutation of the gene encoding the tau protein and located at 17q21.31.
Pathogenesis: what happens in the brain?
The development of symptoms of progressive supranuclear palsy is associated with irreversible and steadily increasing degeneration of neurons in certain areas of the brain. At the heart of such a destructive process is excessive intracellular accumulation of neurofibrillary glomeruli and neurofile filaments that have lost their structure. They disrupt 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 hyperphosphorylated state. Normally, it is attached to tubulin microtubules, which is responsible for their polymerization and stabilization of microtubules and the fixation of certain intracellular enzymes.
The main functions of normal tau protein include:
- participation in the process of maintaining the neuronal cytoskeleton (the skeleton of the nerve cell),
- formation and lengthening of axonal processes,
- recovery of neurons after damage,
- regulation of intracellular transport of vesicles (cytoplasmic vesicles) with synthesized neuropeptides.
Hyperphosphorylated tau protein is no longer able to retain the structure of microtubules. They disintegrate, and the abnormal protein forms filaments (tubules) of irregular shape, which are collected in the cytoplasm in neurofibrillary tangles. In the affected cell, biochemical contact with other neurons is disturbed, the ability to form and retain axonal connections is lost, the cytoskeleton becomes unstable, and the lifespan is significantly reduced. Such neurodegeneration is irreversible and progressive, gradually spreading from the characteristic primary zones to the entire brain.
Neurofibrillary tangles in neurons are formed not only with progressive supranuclear paralysis. Similar degenerative changes in the brain are also found in Alzheimer's disease, corticobasal degeneration, frontotemporal dementia, and in other certain other, more rare diseases. They have a number of common symptoms and come together in a group of taupathies with parkinsonism. They are also called Parkinson's disease plus, implying the mandatory presence of extrapyramidal disorders in the clinic in combination with other symptoms.
The study of various taupathies, the development of questions of their differential diagnosis and the refinement of nosological criteria are dealt with by a specially created Reisenburg Working Group (Reisensburg Working Group for Tauopathies With Parkinsonism).
What is affected with progressive supranuclear palsy
With progressive supranuclear palsy, neurodegeneration in most cases begins in the subcortical and stem formations. The cerebral cortex is initially captured to a lesser extent, but as the disease progresses, the process continues to spread to it. In this case, mainly affected are the anterior sections of the large hemispheres.
Localization of major changes:
- black substance
- subtalamic and peduncular nuclei,
- pale ball
- midbrain lid,
- stem part of the reticular formation,
- temporal and prefrontal areas of the cerebral cortex.
The primary lesion of these areas explains the characteristic sequence of onset of symptoms and the typical parkinson-like disease debut. And the obligatory presence of oculomotor disturbances is associated with degeneration of the dorsal part of the midbrain, which leads to a separation of the connections between the centers of gaze in the cortex and the brainstem. The nuclei of the cranial nerves responsible for the work of the muscles of the eyeballs themselves remain intact. Therefore, paralysis is called supranuclear.
All symptoms of progressive supranuclear palsy are combined into several groups:
- The oculomotor disturbances in the form of 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.
- Parkinsonism (akinetic-rigid form). Moreover, extrapyramidal disorders in the classical course of progressive supranuclear paralysis have a number of features that allow for the correct differential diagnosis. Characteristic is the predominance of rigidity of the muscles of the neck and shoulder girdle with the formation of a characteristic “proud” posture, bradykinesia (slowness of movements), symmetry of disturbances even in the initial stages, the early appearance of postural instability.This extrapyramidal symptomatology is not corrected by anti-parsin drugs. Not characteristic resting tremor, falls, pronounced vegetative and pelvic disorders.
- Violations of the walk, usually the type of subcortical astasia with a pronounced effect of postural instability. At the same time, the step length, the area of the support and the initiation of movements initially do not change, friendly movements of the arms and legs remain. People with progressive supranuclear palsy already in the early stages of the disease easily lose their stability when turning, changing the speed of movement, pushing, walking on an inclined surface. During the first year of the disease there are drops back, and without trying to maintain balance.
- Cognitive impairment, with a fairly rapid development of dementia of the fronto-subcortical type. Speech is impoverished, the ability to abstract and generalize is lost, apathy, field behavior, low speech activity, ecopraxia are characteristic.
- Pseudobulbar syndrome due to lesion of the frontal cortex and regulatory pathways leading from it. Early develop dysarthria (indistinctness of sound pronunciation), dysphagia (disorders of swallowing, and even with an intact and even increased pharyngeal reflex), symptoms of oral automatism, violent laughter and crying.
For progressive supranuclear palsy, illusions, hallucinatory-delusional syndrome, qualitative and quantitative disturbances of consciousness, and bright affective disorders are not characteristic.
Atypical clinical forms of progressive supranuclear palsy are also possible: with a predominance of parkinsonism and the appearance of asymmetric dystonia of the extremities, with a debut in the form of rapidly increasing cognitive impairment, with a predominance of primary progressive aphasia.
Oculomotor disturbances in patients with progressive supranuclear palsy
They are an obligatory manifestation of the disease and are characterized by a characteristic combination of symptoms:
- Loss of ability to arbitrary movement of the eyeballs: usually first in the horizontal and then in the vertical plane. The outcome is a complete ophthalmoplegia with the inability to purposefully translate the view.
- The decline in convergence, which is already in the early stages of the disease, is accompanied by complaints of blurred vision and double vision in the translation of the gaze on distant objects.
- Preservation of the reflex friendly movements of the eyeballs.
- The appearance of the puppet eyes phenomenon, when the eyeballs continue to involuntarily fix the object during head movements. This is due to the lack of suppression of vestibuloocular syndrome. At earlier stages, discontinuity and “lagging” of tracking the eye of a moving object are noted (when viewed from the neurological hammer), which leads to the appearance of abrupt “catching up” movements of the eyeballs.
- Gradual extinction of the amplitude and speed of arbitrary saccadic movements of the eyeballs. During a neurological examination, this is detected when checking follow-up movements, each re-translation of the gaze into extremely lateral leads is accompanied by an increasing limitation of eye mobility (hypometry).
- Absence of spontaneous nystagmus.
The method of provoking optokinetic nystagmus can be used, with rotation of the striped drum in the face of the patient. With progressive supranuclear palsy, the fast nystagmus phase in the vertical plane initially slows down, and in the later stages of the disease it is not caused at all.
The first signs of ophthalmopathy usually appear already in the early stages of the disease. Moreover, a decrease in convergence, changes in optokinetic nystagmus and a decrease in arbitrary vertical saccades are often detected even if the patient has no complaints of visual impairment. To make a presumptive diagnosis, it is necessary to have at least a downward gaze in combination with other signs of a neurodegenerative process.
Forms of progressive paralysis
During progressive paralysis, there are three stages: initial, stage of disease development and stage of dementia.
Initial stageas with syphilis of the brain, is called neurasthenic stages, or precursor stages, and is characterized by the appearance of fatigue and exhaustion, general muscle weakness, increased irritability, alternating apathy. Over time, changes in the personality with the loss of ethical norms of behavior peculiar to the patient seem more distinct, tact and sense of shyness are lost, and criticism of their behavior is reduced. This period includes an increase in sleepiness during the daytime and the appearance of insomnia at night, patients lose their appetite, or they become excessively voracious. Against the background of signs of neurasthenia, the most typical symptoms of progressive paralysis are found - loss of family care, loss of sensitivity to loved ones, wastefulness, sloppiness, loss of modesty, involuntary, inappropriate use of obscene, cynical, obscene expressions with a complete loss of criticism. Subsequently, lethargy, indifference to the environment with faintheartedness, sentimentality, and passivity quickly join in (there was a point of view that all these disorders are associated with affective disorders). In the future, the decline in working capacity increases: patients in their usual work begin to make blunders, which eventually cease to notice due to loss of attention and criticality.
At this stage, the neurostatus shows transient irregular pupils, paresis of the eye muscles, tremor, uneven tendon reflexes, discoordination of movements, and uncertainty of the gait. Speech becomes monotonous, inhibited, or, conversely, unreasonably hasty.
In the blood, Wasserman reaction is often weakly positive or negative. In the study of CSF, Wasserman, Nonne-Apelt, Pandi reactions are sharply positive, cytosis (20-30 cells), an increase in protein content are detected. RIT and RIF in all dilutions sharply positive.
AT heyday - in the second stage of the disease - along with the increasing dementia, loss of memory and the weakness of the thinking process, crazy ideas arise, an increase in mood with gross sexual promiscuity. Criticism to the state of these patients is absent and anosognosia is observed. Sometimes depression develops with suicidal tendencies, nihilistic delusions.
At the final (third) stage, also called maratic stage, there is a total disintegration of mental activity, complete helplessness and physical insanity: 398.