Shai syndrome. Central failure syndrome of the autonomic nervous system (idiopathic orthostatic hypotension, Shy-Drager syndrome). Treatment of Shy-Drager syndrome

is a progressive group of diseases that belong to the neurodegenerative type.

There are three diseases in this group, but the most commonly diagnosed is Shy-Drager syndrome. It is most often diagnosed in men, and very rarely in women. It is believed that this pathology affects 1% of all people on the planet over 50 years of age.

Causes

Shy-Drager syndrome develops for unknown reasons. During the entire period of pathological research, its causes were never established. Nephron degeneration occurs in a variety of parts of the brain, and the degree of damage can vary greatly. This is what determines the clinical symptoms of the disease.

How it manifests itself

The first clinical manifestations can be very different. But the appearance of parkinsonism, which is not controlled by levodopa drugs, as well as cerebellar disorders and autonomic failure, suggest this diagnosis.

The first thing you should pay attention to is parkinsonism. The patient has bradykinesia and jerky tremor. There is trembling of the tongue and lips when pronouncing words.

In this case, all symptoms, as a rule, completely disappear with rest and are not amenable to treatment with levodopa. This is what makes it possible to distinguish the symptoms of Shy-Drager syndrome from.

Cerebellar abnormalities include ataxia, dysmetria, and difficulty the patient may experience with rapidly alternating movements. There is also a lack of coordination of the eyeballs.

Autonomic failure is another symptom of multisystem brain atrophy. These symptoms develop in the later stages of the disease. May include decreased sweating, difficulty breathing and swallowing, urinary and fecal incontinence, and decreased salivation.

Sleep disturbances and difficulty breathing are common. Nocturnal polyuria is present. Many patients do not notice that something is wrong with them and continue to live their normal lives, considering all such manifestations to be age-related changes.

Diagnostics

The basis for diagnosing multisystem cerebral atrophy is MRI. However, precise diagnostic signs may not be seen here either. Most often, the diagnosis is made after death, but if there is a combination of all the described symptoms and Parkinson’s disease is completely excluded, then this diagnosis is made.

Diagnosis is also complicated by the fact that the disease is considered relatively rare, and some doctors cannot make the correct diagnosis for a long time, since they have not encountered a single similar case before.

How to get rid

There is no treatment for multiple system brain atrophy. Only maintenance therapy is provided, which helps correct most symptoms. To get rid of orthostatic hypotension, it is recommended to increase the amount of water you drink, wear compression garments, and regularly take midodrine. It is recommended to sleep with the head of the bed raised.

In order to get rid of the symptoms of parkinsonism, the use of levodopa is not enough, and this medicine has practically no effect on the symptoms in this case. And if there are any improvements, they are insignificant.

For urinary incontinence, oxybutynin chloride or tolterodine have a good effect. If urinary retention occurs, catheterization may be required. Doctors advise patients with this diagnosis to master the catheterization technique on their own.

To prevent constipation, a special diet is required. If constipation lasts for a long time, an enema may be necessary.

Multiple system atrophy has a very poor prognosis because there is no medicine that can prevent further destruction of brain cells. Some patients at a very early stage tolerate L-DOPA preparations well, which significantly improve their general condition. Amantadine can also be prescribed either orally or in the form of intravenous systems. However, in most cases, it is not drug therapy that is important, but proper and careful care.

The patient must adequately assess his condition, and only a consultation with a psychotherapist can help with this. Classes with a speech therapist are also very important to help preserve speech and swallowing ability.

Physical therapy is required to help maintain balance and muscle strength. You need to study every day, and classes can be both group and individual.

If it is impossible to eat during feeding, a gastric tube is used. If urinary incontinence is very severe, a permanent catheter is placed.

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Shai-Drager syndrome(G.M. Shy, American neurologist, 1919-1967; G.A. Drager, American neurologist, born in 1917) - degenerative damage to the central nervous system of unknown etiology, manifested by orthostatic arterial hypotension in combination at different stages of the disease with symptoms of parkinsonism , impaired sweating (anhidrosis), dysfunction of the pelvic organs and other neurological disorders. The disease is rare.

Before the description of Shy-Drager syndrome, the so-called idiopathic orthostatic hypotension was already known, the leading manifestation of which is a postural drop in blood pressure of unknown origin with severe orthostatic circulatory disorders. In 1960, Shay and Drager described two cases of such hypotension, drawing attention to its combination with symptoms of widespread organic damage to the central nervous system. - rigidity and trembling of the limbs, loss of coordination of movements, atony of the sphincters of the pelvic organs, sweating disorders.
Since these disorders preceded severe orthostatic hypotension, Shai and Drager suggested that organic changes in the central nervous system. in this disease are primary and not caused by orthostatic disorders of the blood supply to the brain. At the same time, intermittent brain hypoxia may acquire additional pathogenetic significance as the disease progresses. Currently, idiopathic orthostatic hypotension and Shy-Drager syndrome are considered as variants of the manifestation of a single pathology, and both terms are often used as synonyms.

Etiology not known. The toxic nature of the degeneration of nerve structures is unlikely, although the changes observed in this disease are similar to the changes that occur during chronic poisoning of certain metals. There is an observation of a familial form of Shy-Drager syndrome with a dominant type of inheritance. Shy-Drager syndrome has been described in tonsil cancer as a (presumably) paraneoplastic process.

Pathogenesis insufficiently studied.
The morphological substrate of parkinsonism in Shy-Drager syndrome is degenerative changes in the cells of the substantia nigra. However, the pathogenesis of parkinsonism remains unclear and differs from that in Parkinson's disease (anticholinergics do not reduce the manifestations of parkinsonism in Shy-Drager syndrome, and levodopa in some cases even enhances the manifestations of neurological disorders).

The genesis of orthostatic hypotension is relatively clear, the connection of which with the loss of sympathetic regulation of hemodynamics is proven not only by pathological data, but also by the fact that in patients, unlike healthy individuals, when moving from a horizontal to a vertical position, the concentration of norepinephrine in the blood does not increase (it even decreases ), and in some patients a decrease in the concentration of norepinephrine in the blood and cerebrospinal fluid is found even in a horizontal position of the body. The hemodynamics and tone of peripheral vessels in a horizontal position in patients do not differ significantly from the norm, but in a vertical position there is no increase in heart rate observed in healthy people (the so-called fixed rate) and orthostatic vascular reactions dependent on sympathetic regulation are sharply reduced or absent.
This leads to a sharp decrease in cardiac output, an uncompensated increase in peripheral vascular resistance, as a result of which in orthostatic patients with Shy-Drager syndrome both systolic and diastolic blood pressure drop sharply, blood flow to the brain decreases, and syncope develops due to its diffuse ischemia .

A decrease in the tone of the sympathetic nerves is manifested by hypofunction of the sweat glands, weakening of sweating, as well as a significant increase in the sensitivity of a-adrenergic receptors of blood vessels to norepinephrine. The latter manifests itself in severe hypertensive reactions even with very slow intravenous administration of norepinephrine to patients (at a rate of only 0.5 or even 0.1 mcg/kg per minute). Since levodopa in patients with Shy-Drager syndrome does not have a central hypotensive effect, often increasing blood pressure (peripheral effect), we can conclude that sympathetic connections between the brain and the periphery are disconnected.

Information about the pathogenesis of other neurological disorders is limited to an indication of their connection with disorders of somatic and autonomic innervation, cell degeneration in various structures of the central nervous system, including the dorsal nucleus of the vagus nerve, as well as in the sympathetic ganglia.

Pathological anatomy.
During pathological examination, changes specific to Shy-Drager syndrome are not macroscopically detected. In some cases, adrenal hypoplasia with focal cortical hyperplasia in the form of small adenomas is noted. The main changes are found in the central nervous system. Histologically, degeneration of neurons in the putamen, substantia nigra, inferior olives, caudate nucleus, degenerative changes in the cerebellum, pons, strionigral, striatopallidal and olivopontocerebellar tracts, interstitial brain, a significant decrease in the number of Purkinje cells, pathological changes in the cells of the ventral column, intermediolateral substance of the spinal cord and cells of Clark's columns, changes in neurons of the autonomic ganglia.

CLINICAL PICTURE
Shy-Drager syndrome occurs in people of both sexes, most often between the ages of 50-70 years. The first visits to a doctor are usually associated with the appearance of orthostatic hemodynamic disorders. However, according to the anamnesis, they are often preceded (several months or 1-3 years before) by other symptoms of the disease - most often decreased libido in women, impotence in men, vegetative disorders (dysuria, sweating disorders, bowel function).

Initially, patients complain of weakness, fatigue, and unsteady gait. Subsequently, prolonged standing becomes impossible due to “dizziness,” darkening of the eyes, and a premonition of loss of consciousness. Then the severity of orthostatic disorders quickly progresses, orthostatic fainting often occurs, which becomes dominant and determines the severity of the patient’s condition, regardless of the degree of concomitant neurological disorders. Over the course of several months, the time interval from the moment the patient moves from a horizontal to a vertical position until the onset of fainting is reduced to several minutes, and subsequently to 1 minute or less. In severe cases, fainting can occur even while sitting in bed; passive transfer of the patient to an upright position threatens the development of deep fainting with severe cerebral ischemia. In most cases, a few months after the onset of orthostatic hemodynamic disorders or simultaneously with their appearance (less often earlier), rigidity and trembling of the muscles of the limbs, and coordination disorders are detected. Full-blown manifestations of parkinsonism are observed in approximately 60% of patients and do not always correspond to the akinetic form, which was considered characteristic of Shy-Drager syndrome. Of the extrapyramidal signs, more than half of the patients have amymia, rigidity of the muscles of the limbs, and in about 1/3 of the patients - a monotonous voice, trembling of the limbs. Corticobulbar disorders (sucking reflex, less commonly swallowing disorders) appear in approximately 1/5 of patients. Relatively often (in about 40% of cases) symptoms of cerebellar damage are found in the form of intention tremor, ataxic gait, and dysarthria. Bernard-Horner syndrome, anisocoria, and sensory disturbances (hypesthesia) are occasionally observed. Autonomic disorders are most often manifested by various urinary disorders (in 65% of patients), including enuresis, as well as bowel function, incl. atony of the rectal sphincter.

In the absence of cerebellar ataxia, patients often try to adapt gait and body posture to orthostatic changes in hemodynamics. They move with wide, pressed slightly to the side, quick steps on their legs slightly bent at the knees, their torso bent low forward and their head lowered (skater’s pose). To prolong the time spent in an upright position, patients take tense, sometimes bizarre poses - often crossing their legs, squeezing the veins of the legs and thighs with strong muscle tension, which increases the venous return of blood to the heart.

When examining patients in a horizontal position, no significant changes in the activity of internal organs (except for those associated with concomitant diseases, complications or caused by disorders of nervous regulation) are noted: blood pressure is normal, sometimes increased or moderately decreased; characterized by its instability during hourly and daily measurements. When performing orthostatic tests (see Orthostatic circulatory disorders), the reaction of blood pressure and pulse corresponds to the asympathicotonic type of orthostatic regulation disorders - both systolic and diastolic blood pressure quickly drops in the absence of changes in pulse rate or its slight increase, “alabaster” pallor of the skin occurs and quickly progresses head, face, neck, upper half of the body, the patient’s gaze becomes unfixed, fainting occurs and, in the absence of outside help, the patient falls. With the patient lying down, consciousness gradually returns, and for several hours after this the patient notices severe weakness, fatigue, and drowsiness.

Complications observed with Shy-Drager syndrome are most often associated with orthostatic hemodynamic disorders: injuries due to a fall due to fainting are possible, mental and intellectual disorders as a result of intermittent ischemia of the cerebral cortex, and ischemic strokes sometimes develop in elderly people. Disorders of the urinary system organs are often complicated by cystitis and pyelitis.

The diagnosis of early development of orthostatic hemodynamic disorders is not difficult if the doctor is informed about the essence of the syndrome. A presumptive diagnosis is substantiated by the identification of signs of a pronounced deficiency of sympathetic activity in orthostatic tests and the detection of neurological disorders. In all cases, the patient is hospitalized, because The final diagnosis is established only in the hospital after an in-depth examination of the patient and exclusion of diseases with similar manifestations. Sometimes it becomes necessary to exclude organic occlusion of the internal carotid artery and carotid sinus syndrome.

Depending on the syndrome of neurological disorders, differential diagnosis is made with Parkinson's disease, consequences of encephalitis, secondary degenerative changes in the central nervous system. for various diseases and intoxications. The autonomic disorders that develop with parkinsonism (anhidrosis, enuresis, atony of the rectal sphincter) occur later than with Shy-Drager syndrome and are much less pronounced. Postencephalitic parkinsonism is characterized by hypersalivation, hyperhidrosis, blepharospasm, slowdown and inertia of mental processes. Differential diagnosis with degenerative changes in the central nervous system. toxic genesis is based on a thorough toxicological history and identification of signs of damage to peripheral nerves, which is not characteristic of Shy-Drager syndrome.

Treatment consists of correcting the most severe manifestations of the disease - orthostatic hemodynamic disorders and parkinsonism. To limit the rate of development of orthostatic arterial hypotension, tight bandaging of the limbs, pelvic girdle and abdomen is used. Of the pharmacological agents, synthetic fluorinated corticosteroids (dexamethasone, triamcinolone acetonide, etc.) and dihydrogenated ergot alkaloids (dihydroergotamine or dihydroergotoxin orally or in the form of intramuscular injections) are most often effective. The use of α-adrenergic agonists is less effective and is associated with the occurrence of arterial hypertension in the horizontal position of the patient and the risk of complications. In some cases, tolerance to a vertical position is somewhat improved by MAO inhibitors, tyramine, and a salt-enriched diet; a positive result from the use of indomethacin has been described. There are reports of significant effectiveness of implantation of a pacemaker that reduces the atrial contraction rate to 100 per minute.

Parkinsonism in Shy-Drager syndrome is resistant to the use of levodopa and is difficult to correct. There is evidence of the effectiveness of the combined use of cyclodol and dibenzipine.

FORECAST
The disease usually progresses quickly. In rare cases, there is a relative stabilization of the manifestations of Shy-Drager syndrome for 3-5 years. Death in most patients can occur 7-8 years after the first symptoms appear.

It just so happens that in neurology almost all diseases are similar to Parkinson's disease. This pathology is no exception. It is also somewhat similar to Parkinson's disease. Sometimes Shai Dreijar disease called idiopathic orthostatic hypotension.

This pathology is caused by insufficient nutrition of the autonomic nervous system.

And this leads to problems in all systems for which it is responsible. And she is responsible for many things, including: heart rate; blood pressure; the work of the glands; fixation of gaze.

When this disease appears, many body systems begin to work with errors and lose their ability to recover.

Causes of pathology

The exact reasons are still not known; the influence of genetics is assumed. Men aged 39 to 61 years are affected.

It happens that Shai Drager's disease occurs while taking certain medications.

These include:

1. Diuretics, which, due to the removal of fluid, lead to a decrease in blood pressure.
2. Drugs that dilate blood vessels.
3. Antihypertensive drugs.

At the moment, it is known about this disease that it often causes loss of consciousness, which leads to:

1. Cardiac arrhythmia.
2. Insufficient blood supply to the heart.
3. Impaired cardiac functions due to lack of nutrition.
4. Bradycardia.

Clinical manifestations of the syndrome

They are as follows:

Lack of attraction to the opposite sex or inability to ejaculate.
Problems with urination. It happens to both men and women. This is said to be due to a lack of normal bladder tone.
Patients have impaired production of all body media: tears; saliva; sweat.
There is a sharp drop in pressure when changing position.
Frequent constipation.
Coordination impairment similar to Parkinson's disease.
Fast fatiguability.
Dizziness when standing for a long time.
The feeling of fainting is always with a person.

As the disease progresses, the condition worsens further.

If the severity of the disease is severe, then fainting occurs even when sitting in bed.
After fainting, the body trembles like an aspen leaf. Parkinsonism-type manifestations are observed in 61% of patients.

This condition can be suspected if, in addition to the above symptoms, the following appear:

Feeling hot or cold at standard body temperature.
Snoring too much.
Raising your legs high when walking.
Throat paralysis.
Flashing of flies before the eyes or double vision.

Combinations of the listed manifestations of the disease may occur; not all of them will necessarily be present.

Diagnostics

If the doctor is sufficiently qualified, he can easily identify the disease in its early stages.

Hospitalization is required to exclude hydrocephalic syndrome.

Autonomic disorders such as: lack of rectal tone; urinary incontinence and a small amount of sweat appear much earlier than in Parkinson's disease, and are more pronounced.

Also required for diagnosis:

Toxicological history.
Collecting information about lifestyle.
Conversation with the patient's relatives.
Rotary table examination. On it, the person is first positioned horizontally, and then the table abruptly turns the patient into a vertical position. Next, the pressure is checked.

Treatment of patients

Only comprehensive, under blood pressure control.

The goal of treatment: to slightly alleviate the patient’s condition and increase blood pressure to normal.

Treatment methods:

Reducing the volume of space filled with blood. To organize this, apply a tight bandage to the limbs and pelvic regions. It is possible to use rubber suits for these purposes.
Swimming helps a lot.
High blood pressure due to increased blood volume.
Combination therapy helps.
Proper diet, with increased salt and water, as well as fiber. Reduced carbohydrate intake.
Preparations for salt retention in the body.
Implantation of a pacemaker for contraction rates up to 100 beats/min.
To give up smoking.
When sleeping, a person should place his head on a raised surface.
24-hour supervision is required.

Complications and prognosis

With a sharp decrease in blood pressure after normal pressure, a complication is possible - cerebral ischemia.

The prognosis is usually not very good, since the disease progresses quickly, but sometimes there is still a pause in the development of the syndrome for 4-6 years.

Basically, after the first symptoms of the disease, death occurs 6-9 years later.

The percentage of diseases is 2% per 20 thousand cases.

As a rule, men are sick, less often women. When the first signs appear, immediately consult a doctor, only he will be able to confirm or dispel your assumptions about the disease and, if necessary, prescribe treatment.

Shy Drager syndrome is a systemic disease, the etymology of which has not yet been fully understood. It is characterized by damage to the autonomic nervous system, which regulates the functioning of all internal systems of the body, from the glands to the circulatory system. It occurs quite rarely, in approximately 5 people per 100,000. There is a disorder in the functioning of the cerebellum, and possible manifestations of parkinsonism syndrome and endocrine system disorders. Symptoms can appear one at a time in any order. Some may remain latent for a long time or go unnoticed against the background of other manifestations. Shy syndrome primarily affects men over 50 years of age.

Risk factors, What are they?

There are several types of atrophy: physiological and pathological.

Physiological is a natural process that occurs with every person throughout life. An example is the teenage thymus gland, which disappears after puberty. This type of atrophy is also part of the aging process: atrophy of bone and cartilage tissue in old age, dryness and decreased elasticity of the skin. In the case of manifestation of Shy's syndrome, we are talking about pathological atrophy.

Pathological atrophy is usually divided into general and local. Shy syndrome is a multisystem or general atrophy. This is a neurodegenerative, slowly progressive pathology with damage to the nerve cells of the subcortical connections of the white matter of one or both hemispheres, the brain stem and the cerebellum. It can also affect the spinal cord.

In addition to Shy-Drager syndrome, the type of multisystem atrophy, depending on the clinical manifestations, includes: striatonigral and olivopontocerebellar atrophy.

Although the causes of this pathology have not been studied, the main one is considered to be a genetic predisposition to multisystem atrophy. Failures in the autonomic system begin due to degeneration and death of nerve cells in the central and spinal cord. For this reason, unconditioned reflexes deteriorate, and this entails serious consequences.

Most often, men between 40 and 60 years old are susceptible to this pathology. Those who systematically take are also at risk:

laxatives that remove fluid from the body;
alcohol and drugs.
substances that lower blood pressure.

Causes

Atrophy of this type is never congenital, but only acquired during life and manifests itself in old age. This is expressed in the fact that previously healthy and properly functioning organs lose their normal volume due to drying out and stop developing.

Multisystem atrophy is provoked by factors such as:

Insufficient supply of nutrients;
Oncological diseases;
(hypothalamus);
Endocrine system disorders;
Long-term illness with infectious diseases;
Long-term uncontrolled use of hormonal drugs;
Genetic predisposition.

Our boarding houses:

Symptoms

The multisystem type of atrophy is detected during inpatient observation of signs of several types of disorders at once. Signs indicating Shy-Drager syndrome. Symptoms can appear individually or in combination:

speech dysfunction;
defecation disorders, alternating diarrhea and constipation;
disturbances in sweating;
a sharp decrease in pressure in the circulatory system when taking a vertical position;
decreased libido and possible impotence;
uncontrolled urination;
impaired salivation, dryness and viscosity in the mouth;
Violation of eye accommodation, as a result of deterioration of vision;
Decreased tone of the motor muscles of the eyeball;
Sleep apnea;
Parkinsonism syndrome;
Chills.

To prescribe effective treatment, it is necessary to accurately separate Shai's symptoms from other diseases with a similar clinical picture, for example, parkinsonism syndrome. Therefore, the sequence of manifestations is important. An early symptom of the disease is damage to the cerebellum, the part of the brain that is responsible for coordinating movements. If the disease affects him, the person has poor balance. If it is not affected, patients may assume unusual positions that encourage venous flow to the heart. When walking, the posture becomes more stooped, the head is pushed forward, the steps become wider and softer due to half-bent knees. This helps the body adapt to orthostatic hypotension.

A patient most often seeks medical help when problems with blood pressure and the circulatory system become noticeable. Increased fatigue is observed, even if the patient has slept normally, he may be lethargic all day. They appear periodically, especially with a sharp change in the position of the body in space; the vision becomes dark. In this case, in a supine position, the measured pressure will be normal, whereas when raised to a vertical position, it drops sharply until it is completely absent.

After this, other symptoms usually begin to appear, indicating a disorder in the autonomic nervous system: decreased sexual activity, urinary incontinence, and systematic bowel movements.

Over time, these symptoms worsen and if at first the disease, loss of consciousness is quite rare, then in the end they become so frequent that the patient is practically unable to stand on his feet. Each time, the time until he can walk normally decreases down to one to two minutes.

In addition, strokes are a fairly common complication of Shy-Drager syndrome. This occurs due to constant pressure surges that the brain vessels simply cannot withstand. Depending on the area in which the hemorrhage occurred, the severity of the consequences depends. For example, if this happened in the hemisphere, then the stroke may go unnoticed, but if it affects important functional areas, the consequences can affect speech, motor ability, etc. Also possible: ischemia of the cortex and occipital lobes of the brain, as well as physical injuries of varying severity caused by loss of consciousness.

Diagnostics

Every person feels when changes begin to occur in his body, but all of them are dangerous. When should you see a doctor:

There is a constant feeling of weakness in the body, increased fatigue is noticeable;
Reduced speed of movement (mainly when walking);
Manifestation of tremor in the hands - trembling is especially noticeable while weighing, when a person tries to maintain one position;
In the presence of vegetative symptoms, such as dizziness and subsequent loss of consciousness, disturbances in defecation and urination, sensation;
With decreased libido;
Visual impairment: darkening or double vision.

In case of loss of consciousness, it is better not to lift the patient, as this is a natural reaction of the body, which is trying to normalize the pressure. You can move him to the sofa and call an ambulance.

Subsequent diagnosis is based on the clinical manifestations of the disease. Orthostatic tests are performed. In this case, it is necessary to carry out diagnostics on a hospital basis to exclude diseases with similar symptoms.

At the first appointment, the doctor conducts a survey and thoroughly records the medical history. It is noted under what circumstances symptoms occur.

In order to identify pathology, doctors offer to undergo cognitive tests, which make it possible to establish speed and thinking abilities and presumably determine the presence and degree of development of pathology. To exclude the vascular origin of atrophy, Doppler ultrasound of the vessels of the head and neck is performed. This way you can find the exact location of atherosclerotic changes. For a more detailed examination, an X-ray machine is used. This reveals structural changes, such as tumors or hemorrhages.

Treatment at different stages

Treatment for Shy syndrome is only symptomatic. It is impossible to completely cure the disease. Sometimes temporary remission is possible for up to 5 years.

Treatment mainly involves the use of a range of different specialized medications. The most significant effect on the course of Shy-Drager disease is the implantation of a pacemaker, which controls the heart rate or contractions of the atria.

Forecast

The disease progresses quite quickly, and death occurs in most cases within about 8 years after onset. Sometimes there is a temporary remission lasting from 3 to 5 years. The worst case scenario is observed when the Autonomic Nervous System is damaged.

Disease prevention

Prevention of Shay's syndrome consists of preventing the causes that provoke the development of the systemic atrophic process. To prevent the development of functional disorders of the brain, it is very important that the patient leads a healthy lifestyle and exhibits physical activity. A positive attitude and the prevention of unnecessary worries are also important.

To date, medications have not been able to cure a person from multiple system brain atrophy. Shy syndrome develops slowly, but can ultimately lead to dementia and loss of self-sufficiency. Therefore, it is important not only to try to prevent this disease, but also to undergo timely diagnosis and treatment from specialists. By procrastinating or neglecting symptoms, the patient can deprive himself of valuable time and lose health.

In 1960, Shy and Drager published the results of a clinical and morphological examination of two patients, whose clinical picture was dominated by symptoms of parkinsonism and orthostatic hypotension. The disease is rare, although in recent years it has been repeatedly described in a number of works; we also had the opportunity to observe several such patients.

It is likely that Shy-Drager syndrome occurs much more often than is recognized. Typically, these patients appear in medical records as having parkinsonism.

Expanded clinical picture of Shy-Drager syndrome consists mainly of moderately expressed akinetic-rigid syndrome with manifestations of hypokinesia, acheirokinesis, plastic rigidity and tremor, as well as severe orthostatic hypotension. Characteristic signs also include anhidrosis, bladder atony, sometimes with signs of urinary incontinence, fixed heart rate and (sometimes) decreased rectal sphincter tone.

In neurological status Clinical (and electromyographic) indications of the involvement of cells of the anterior horns of the spinal cord in the pathological process are often found. Atrophy of the distal limbs and fasciculations are possible. Cerebellar symptoms are less commonly identified in the form of more or less pronounced intention tremor, adiadochokinesis, etc. Paresis of the external eye muscles and atrophy of the iris may occur.

Men are more susceptible to this disease. The disease debuts at the age of 50-60 years and progresses rapidly. The disease often begins with complaints of fatigue, weakness in the limbs, dizziness and heat intolerance (anhidrosis). Then repeated syncope attacks appear, which sometimes begins the disease. The most painful thing for patients is the tendency to faint, which in severe cases can be observed even when the patient is sitting.
Dizziness and fainting do not respond well to conventional symptomatic therapy.

When changing body position there are pronounced fluctuations in blood pressure; Shai and Drager present their own observation in which the patient’s systolic pressure decreased from 170 in the supine position to 40 mm Hg. Art. in a standing position. Characteristic is a slight change in heart rate when changing body position.

Bladder atony may be accompanied by frequent urination and insufficient voluntary control of the sphincter. Some authors describe other symptoms of this disease that are optionally present in individual observations (tendon hyperreflexia, Babinski's symptom, decreased lacrimation and salivation, impotence, etc.).

Pathological descriptions indicate the death of neurons in the structures of the autonomic nervous system, pathological changes in the corticonuclear and corticospinal tracts, basal ganglia, and cerebellar connections. Morphological findings indicate the nosological independence of this disease, the essence of which is progressive encephalomyelopathy of unknown etiology. The disease is considered degenerative.