Peripheral insufficiency. Vegetative failure

Autonomic insufficiency is a syndrome associated with a diffuse violation of the innervation of internal organs, blood vessels, secretory glands. In most cases, autonomic failure is caused by damage to the peripheral autonomic system (peripheral autonomic failure).

Most often, the function of both the sympathetic and parasympathetic parts of the autonomic nervous system suffers at the same time, but sometimes dysfunction of one of these parts predominates.

Etiology. Vegetative failure is primary and secondary. Primary autonomic failure is caused by hereditary or degenerative diseases involving autonomic neurons in the central nervous system or neurons in the autonomic ganglia (eg, hereditary polyneuropathies, primary autonomic neuropathy, or multisystem atrophy). Secondary autonomic failure often occurs with metabolic polyneuropathies (diabetic, amyloid, alcoholic, etc.), diffusely damaging autonomic fibers, sometimes with damage to the brain stem or spinal cord (for example, in stroke, traumatic brain injury or tumor).

The clinical picture of autonomic insufficiency consists of symptoms of damage to various organs and systems. Dysfunction of the cardiac vascular system is primarily manifested by orthostatic hypotension, characterized by a violation of maintaining blood pressure in an upright position. Orthostatic hypotension is mainly caused by sympathetic denervation of the veins of the lower extremities and abdominal cavity, as a result of which, when they move to an upright position, they do not narrow and blood is deposited in these vessels. Denervation of the kidneys also contributes to the development of orthostatic hypotension, which causes nocturnal polyuria, leading to a decrease in circulating blood volume in the early morning hours. Orthostatic hypotension can manifest itself when moving from a horizontal position to a vertical one or during prolonged standing (especially in a stationary position), dizziness, the appearance of a veil before the eyes, a headache or heaviness in the back of the head, a sudden feeling of weakness. With a sharp drop in blood pressure, fainting is possible. With severe orthostatic hypotension, patients are bedridden. In the supine position, blood pressure can, on the contrary, rise sharply, which makes the treatment of orthostatic hypotension extremely difficult.

A decrease in blood pressure with the development of dizziness or weakness can also be triggered by food intake, which causes a rush of blood to the abdominal organs, as well as overheating, straining, and physical activity.

As a result of a violation of the innervation of the heart, the heart rate is fixed at the same level and does not change depending on the respiratory cycle, physical activity, body position (fixed pulse). Most often, a fixed pulse is associated with parasympathetic denervation of the heart, so it is usually "fixed" at the level of tachycardia.

Dysfunction of the gastrointestinal tract leads to impaired motility of the stomach, intestines, gallbladder, which is clinically manifested by a feeling of heaviness in the epigastrium after eating, a tendency to constipation or diarrhea. Dysfunction of the genitourinary system is manifested by impotence, frequent urination, especially at night, the need for straining at the beginning of urination, a feeling of incomplete emptying of the bladder.

Disorder of the innervation of the sweat glands usually leads to a decrease in sweating (hypohidrosis, anhidrosis) and dry skin, but some patients have regional hyperhidrosis (for example, in the face or hands) or night sweats.

Diagnosis. To detect orthostatic hypotension, blood pressure is measured in the supine position (before this, the patient must lie for at least 10 minutes), and then after standing up (no earlier than 2 minutes later). The presence of orthostatic hypotension is evidenced by a drop in the upright position of systolic pressure by at least 20 mm Hg. Art., and diastolic - at least 10 mm Hg. Art. To identify a fixed pulse rate, its fluctuations are assessed during deep breathing, straining, changing body position, and physical activity. The motility of the gastrointestinal tract is examined using fluoroscopy or endoscopic techniques. To clarify the nature of urination disorders, cystometry and ultrasound examination are performed.

The diagnosis of autonomic failure is facilitated by the identification of concomitant neurological syndromes, indicating damage to the central or peripheral nervous system. It is important to rule out other conditions that cause similar symptoms, such as drug overdose (for example, antihypertensive drugs), diseases of the blood and cardiovascular system, endocrine disorders (for example, adrenal insufficiency).

Treatment primarily involves the impact on the underlying disease. Symptomatic treatment is determined by the leading syndrome. With orthostatic hypotension, a complex of non-drug measures is recommended.

It is necessary to avoid provoking factors: straining (with a tendency to constipation, foods with a high content of dietary fiber are recommended, sometimes laxatives), sudden changes in body position, thermal procedures, overheating, alcohol intake, prolonged bed rest, intense physical exercise, especially in isometric mode.

Shown are moderate physical activity in isotonic mode, especially in the aquatic environment.

If you need to stand for a long time, you should often change your position, shifting and raising one or the other leg, cross your legs, squat down, cross or curl your legs in a sitting position, throw one leg over the other, changing their places from time to time. All of these techniques prevent the deposit of blood in the legs.

If possible, stop taking or reduce the dose of vasodilators.

You need to sleep with your head held high (your head should be raised 15-20 cm); this reduces not only orthostatic hypotension, but also nocturnal polyuria and arterial hypertension in the supine position.

Sometimes it helps to wear elastic stockings, which should be pulled in the morning, before getting up in bed.

If the listed measures were not effective enough. then they resort to medications that increase the volume of circulating blood and vascular tone. The most effective of these is the fluoride synthetic corticosteroid fludrocortisone (cortinef). Sometimes it is combined with other drugs that increase the tone of the sympathetic system (for example, midodrin). All drugs that reduce orthostatic hypotension increase hypertension in the supine position, therefore, during treatment, it is necessary to control blood pressure not only before taking the drug and 1 hour after taking it, but also in the morning after waking up.

Vegetative dysfunction is a complex of functional disorders caused by dysregulation of vascular tone and leading to the development of neuroses and a deterioration in the quality of life. This condition is characterized by the loss of the normal reaction of the vessels to various stimuli: they either strongly narrow or expand. Such processes disrupt the general well-being of a person.

Autonomic dysfunction is quite common, affecting 15% of children, 80% of adults, and 100% of adolescents. The first manifestations of dystonia are noted in childhood and adolescence, the peak incidence falls in the age range of 20-40 years. Women suffer from vegetative dystonia several times more often than men.

The autonomic nervous system regulates the functions of organs and systems in accordance with exogenous and endogenous irritating factors. It functions unconsciously, helps maintain homeostasis and adapts the body to changing environmental conditions. The autonomic nervous system is divided into two subsystems - sympathetic and parasympathetic, which work in the opposite direction.

• Sympathetic nervous system weakens intestinal motility, increases sweating, increases heart rate and enhances heart function, dilates pupils, narrows blood vessels, increases blood pressure.
• Parasympathetic division reduces muscles and enhances gastrointestinal motility, stimulates the body's glands, dilates blood vessels, slows down the heart, lowers blood pressure, narrows the pupil.

Both of these departments are in a state of equilibrium and are activated only as needed. If one of the systems begins to dominate, the work of the internal organs and the body as a whole is disrupted. This is manifested by the corresponding clinical signs, as well as the development, psychovegetative syndrome, vegetopathy.

Somatoform dysfunction of the autonomic nervous system is a psychogenic condition accompanied by symptoms of somatic diseases in the absence of organic lesions. Symptoms in these patients are very varied and variable. They visit different doctors and present vague complaints that are not confirmed by examination. Many experts believe that these symptoms are invented, but in fact they cause a lot of suffering and are of an exclusively psychogenic nature.

Etiology

Violation of nervous regulation is the basic cause of autonomic dystonia and leads to disorders in the activity of various organs and systems.

Factors contributing to the development of autonomic disorders:

1. Endocrine diseases - obesity, hypothyroidism, adrenal dysfunction,
2. Hormonal changes - menopause, pregnancy, puberty,
3. Heredity,
4. Increased suspiciousness and anxiety of the patient,
5. Bad habits,
6. Improper nutrition
7. The foci of chronic infection in the body - caries, sinusitis, rhinitis, tonsillitis,
8. Allergy,
9. Traumatic brain injury
10. Intoxication,
11. Occupational hazards - radiation, vibration.

The causes of pathology in children are during pregnancy, birth trauma, diseases during the neonatal period, an unfavorable climate in the family, overwork at school, stressful situations.

Symptoms

Autonomic dysfunction manifests itself in a wide variety of symptoms and signs: asthenization of the body, insomnia, anxiety, shortness of breath, obsessive phobias, a sharp change in fever and chills, numbness of the extremities, tremor of the hands, myalgia and arthralgia, heart pain, low-grade fever, dysuria, biliary dyskinesia, fainting, hyperhidrosis and hypersalivation, dyspepsia , pressure fluctuations.

The initial stage of pathology is characterized by autonomic neurosis. This conditional term is synonymous with autonomic dysfunction, but at the same time it spreads beyond it and provokes further development of the disease. Vegetative neurosis is characterized by vasomotor changes, impaired skin sensitivity and muscle trophism, visceral disorders and allergic manifestations. At the beginning of the disease, signs of neurasthenia come to the fore, and then the rest of the symptoms join.

The main syndromes of autonomic dysfunction:

• Mental Disorder Syndrome manifests itself as low mood, impressionability, sentimentality, tearfulness, lethargy, melancholy, a tendency to self-blame, indecision, hypochondria, decreased motor activity. Patients develop uncontrollable anxiety regardless of a specific life event.
• Cardiac syndrome manifests itself of a different nature: aching, paroxysmal, burning, short-term, constant. It occurs during or after physical exertion, stress, emotional distress.
• Astheno-vegetative syndrome characterized by increased fatigue, decreased performance, exhaustion of the body, intolerance to loud sounds, meteosensitivity. Adjustment disorder is manifested by an excessive painful reaction to any event.
• Respiratory Syndrome occurs with somatoform autonomic dysfunction of the respiratory system. It is based on the following clinical signs: the appearance of shortness of breath at the time of stress, a subjective feeling of lack of air, chest compression, difficulty breathing, choking. The acute course of this syndrome is accompanied by severe shortness of breath and may result in suffocation.
• Neurogastric syndrome manifested by aerophagia, esophageal spasm, duodenostasis, heartburn, frequent belching, the appearance of hiccups in public places, flatulence, constipation. Immediately after stress, the swallowing process is disturbed in patients, pain in the chest arises. Solid food becomes much easier to swallow than liquid food. Stomach pain is usually not associated with food intake.
• Symptoms of the cardiovascular syndrome are heart pains that occur after stress and are not stopped by the intake of coronalytics. The pulse becomes labile, fluctuates, the heart rate increases.
• Cerebrovascular syndrome manifests itself as impaired intelligence, increased irritability, in severe cases - and development.
• Syndrome of peripheral vascular disorders characterized by the appearance of edema and hyperemia of the extremities, myalgia,. These signs are due to impaired vascular tone and vascular wall permeability.

Vegetative dysfunction begins to manifest itself in childhood. Children with such problems often get sick, complain of headaches and general malaise when the weather changes abruptly. As they get older, autonomic dysfunctions often go away on their own. But this is not always the case. Some children become emotionally labile at puberty, often cry, retire or, on the contrary, become irritable and hot-tempered. If autonomic disorders are disrupting a child's life, see a doctor.

There are 3 clinical forms of pathology:

1. Excessive activity of the sympathetic nervous system leads to the development of autonomic dysfunction ... It is manifested by an increased heart rate, attacks of fear, anxiety and fear of death. In patients, blood pressure rises, intestinal motility is weakened, the face becomes pale, pink dermographism appears, a tendency to increase body temperature, agitation and motor restlessness.
2. Autonomic dysfunction may occur type with excessive activity of the parasympathetic division of the nervous system. In patients, blood pressure drops sharply, the skin turns red, cyanosis of the extremities, greasiness of the skin and acne appear. usually accompanied by severe weakness, bradycardia, difficulty breathing, shortness of breath, dyspepsia, fainting, and in severe cases - involuntary urination and defecation, abdominal discomfort. There is a tendency to allergies.
3. Mixed form autonomic dysfunction is manifested by a combination or alternation of symptoms of the first two forms: the activation of the parasympathetic nervous system often ends. Patients develop red dermographism, hyperemia of the chest and head, hyperhidrosis and acrocyanosis, tremor of the hands, subfebrile condition.

Diagnostic measures for autonomic dysfunction include the study of the patient's complaints, his comprehensive examination and a number of diagnostic tests: electroencephalography, electrocardiography, magnetic resonance imaging, ultrasound, FGDS, blood and urine tests.

Treatment

Drug-free treatment

Eliminate sources of stress: to normalize family and household relations, to prevent conflicts at work, in children's and educational groups. Patients should not be nervous, they should avoid stressful situations. Positive emotions are simply necessary for patients with autonomic dystonia. It is useful to listen to pleasant music, watch only good films, and receive positive information.

Nutrition should be balanced, fractional and frequent. Patients are advised to limit the use of salty and spicy foods, and in case of sympathicotonia, to completely exclude strong tea and coffee.

Inadequate and inadequate sleep disrupts the nervous system. Sleep at least 8 hours a day in a warm, well-ventilated area on a comfortable bed. The nervous system has been loosened over the years. To restore it requires persistent and long-term treatment.

Medicines

TO individually selected drug therapy is transferred only if the general strengthening and physiotherapeutic measures are insufficient:

Physiotherapy and balneotherapy give a good therapeutic effect. Patients are recommended to undergo a course of general and acupressure, acupuncture, visit the pool, exercise exercise therapy and breathing exercises.

Among physiotherapeutic procedures, the most effective in the fight against autonomic dysfunction are electrosleep, galvanization, electrophoresis with antidepressants and tranquilizers, water procedures - therapeutic baths, Charcot's douche.

Phytotherapy

In addition to basic drugs for the treatment of autonomic dysfunction, herbal medicines are used:

Prophylaxis

To avoid the development of autonomic dysfunction in children and adults, it is necessary to carry out the following activities:

Video: a neurologist on autonomic dysfunction

Peripheral autonomic failure

It is a syndrome of diffuse lesions of the peripheral autonomic nervous system. It is a consequence of endocrine (hypothyroidism, diabetes mellitus, acute adrenal insufficiency), autoimmune (myasthenia gravis, Guillain-Barré syndrome), systemic (scleroderma, systemic lupus erythematosus), oncological (carcinomatosis) diseases, intoxication (in particular, adrenergic blockers). It can be combined with other syndromes of damage to the nervous system (parkinsonism, multisystem degeneration). Less commonly, it occurs in the form of an isolated syndrome within the framework of hereditary forms of polyneuropathies.

Clinical manifestations. The main symptoms are manifestations of systemic insufficiency of the peripheral part of the autonomic nervous system: orthostatic hypotension in combination with arterial hypertension in a horizontal position, tachycardia or a fixed pulse during physical exertion, hypo or anhidrosis, intestinal paresis, tendency to constipation or diarrhea, urinary incontinence, nocturnal apnea, visual impairment in the dark.

Orthostatic fainting is noted, with severe forms of the disease developing even in a sitting position. At the same time, tachycardia and arterial hypertension at rest, lying down, are possible.

Diagnostics and differential diagnostics. The diagnosis is established clinically, taking into account the complaints listed above. Diagnostic search is aimed at excluding primary pathological processes that can lead to the development of peripheral autonomic failure.

Treatment is symptomatic. In the case of acute adrenal insufficiency, corticosteroids are used. A sufficient intake of liquid and table salt can give a positive effect. In the absence of contraindications, it is possible to prescribe drugs with vasoconstrictor action (amizinia, ergotamine). Bandaging of the lower extremities with an elastic bandage, compression stockings or tights is used.

Raynaud's syndrome

Raynaud's syndrome is a vasospastic disease characterized by a paroxysmal disorder of the arterial circulation in the vessels of the extremities (feet and hands) under the influence of cold or emotional excitement. Raynaud's syndrome develops against the background of collagen diseases, rheumatoid arthritis, vasculitis, endocrine, neurological pathology, blood diseases, and occupational diseases. Clinically, Raynaud's syndrome is manifested by seizures including sequential blanching, cyanosis, and flushing of the fingers or toes, chin, and tip of the nose. Raynaud's syndrome leads to gradual trophic tissue changes. Conservative measures include taking vasodilators, surgical treatment consists of sympathectomy.

Raynaud's syndrome is a secondary condition that develops against the background of a number of diseases: diffuse connective tissue pathology (scleroderma, systemic lupus erythematosus), systemic vasculitis, rheumatoid arthritis, diseases of the sympathetic ganglia, endocrine and hematological disorders, diencephalic disorders, compression of the neurovascular bundles. In addition, Raynaud's syndrome can be triggered by exposure to occupational hazards (cooling, vibration).

In the pathogenesis of Raynaud's syndrome, the leading role is played by endogenous vasoconstrictors - catecholamines, endothelin, thromboxane A2. In the development of Raynaud's syndrome, three successive phases are distinguished: ischemic, cyanotic and hyperemic. The ischemic phase develops due to spasm of peripheral arterioles and complete emptying of capillaries; manifested by local blanching of the skin. In the second phase, due to blood retention in the venules and arteriovenular anastomoses, the blanching of the skin is replaced by cyanosis (cyanosis). In the last phase - reactive-hyperemic, redness of the skin is noted.

In the absence of etiofactors characteristic of Raynaud's syndrome, the presence of Raynaud's disease is suggested. In the onset of Raynaud's disease, the role of heredity, endocrine dysfunctions, mental trauma, chronic nicotine and alcohol intoxication has been established. Raynaud's disease is more common among women in their 20s and 40s with migraines.

Raynaud's syndrome symptoms

Symptoms of Raynaud's syndrome are caused by paroxysmal vasospasm and resulting tissue damage.

In typical cases, Raynaud's syndrome affects the fourth and second fingers of the feet and hands, sometimes the chin, ears and nose. Attacks of ischemia are short-term at first, rare; arise under the influence of cold agents, as a result of excitement, smoking, etc. Suddenly paresthesia develops, cold fingers, the skin becomes alabaster-white. Numbness is replaced by a burning sensation, aching pain, a feeling of fullness. The attack ends with a sharp flushing of the skin and a feeling of heat.

The progression of Raynaud's syndrome leads to a lengthening of the time of attacks up to 1 hour, their frequency, spontaneous occurrence without visible provocations. After the height of the paroxysm, the cyanotic phase begins, there is a slight swelling of the tissues. In the intervals between attacks, the feet and hands remain cold, cyanotic, moist. For paroxysms of ischemia in Raynaud's syndrome, symmetrical and sequential development of manifestations is characteristic: first on the fingers of the hands, then on the feet.

The consequences of tissue ischemia in the case of a prolonged and severe course of Raynaud's syndrome can be trophic changes in the form of poorly healing trophic ulcers, areas of necrosis, degenerative lesions of the nail plates, osteolysis and deformation of the phalanges, gangrene.

Diagnostics of the Raynaud's syndrome

A patient with Raynaud's syndrome is referred for consultation with a rheumatologist and vascular surgeon.

In Raynaud's syndrome, changes in the distal arteries can be detected by angiography of the peripheral vascular bed, in which areas of uneven stenosis and total vascular obstruction, the absence of capillary networks and collaterals are determined.

Capillaroscopy of the nail bed and the anterior surface of the eye reveals morphological changes in the microvascular pattern, indicating a violation of perfusion.

Laser Doppler flowmetry, used to assess peripheral microcirculation, reveals defects in metabolic and myogenic regulation of blood circulation, a decrease in veno-arterial reactions and sympathetic activity.

In the period between attacks with Raynaud's syndrome, a cold test can be used to provoke vasospasm and assess the state of blood flow.

Raynaud's syndrome treatment

The first principle of therapy for Raynaud's syndrome is the elimination of provoking moments - smoking, cooling, vibration and other domestic and industrial factors. The primary disease that caused the development of Raynaud's syndrome is being identified and treated.

Among vasodilators in Raynaud's syndrome, the prescription of calcium antagonists - nifedipine (cordafen, corinfar, etc.), vazaprostan, selective calcium channel blockers - verapamil, nicardipine diltiazem is effective. If necessary, ACE inhibitors (captopril), selective blockers of HS2-serotonin receptors (ketanserin) are used.

In Raynaud's syndrome, antiplatelet drugs are prescribed - dipyridamole, pentoxifylline (agapurin, trental), low molecular weight dextrans (rheopolyglucin).

The progression and resistance of Raynaud's syndrome to drug therapy is an indication for surgical sympathectomy or gangliectomy.

When an ischemic attack develops, urgent measures are to warm the limb in warm water, massage with a woolen cloth, and offer the patient a hot drink. With a prolonged attack, injectable forms of antispasmodics (no-shpy, platifillin), seduxen and other drugs are prescribed.

With Raynaud's syndrome, non-drug methods are used - psychotherapy, reflexology, physiotherapy, hyperbaric oxygenation. In Raynaud's syndrome caused by systemic collagenoses, sessions of extracorporeal hemocorrection are indicated.

A breakthrough in the treatment of Raynaud's syndrome is stem cell therapy aimed at normalizing peripheral blood flow. Stem cells promote the opening of new collaterals in the vascular bed, stimulate the regeneration of damaged nerve cells, which ultimately leads to the cessation of vasoconstriction paroxysms.

Forecast and prevention of Raynaud's syndrome

The prognosis of Raynaud's syndrome depends on the progression of the underlying pathology. The course of the syndrome is relatively favorable, attacks of ischemia can spontaneously stop after a change in habits, climate, profession, sanatorium treatment, etc.

The lack of primary preventive measures allows us to speak only of secondary prevention of Raynaud's syndrome, i.e., the elimination of triggering factors leading to vasospasm - hypothermia, vibration, smoking, psychoemotional stress.

Peripheral autonomic failure (PVI)- a syndrome represented by a complex of pathological vegetative manifestations that develop with damage (as a rule, organic) of the peripheral (segmental) part of the autonomic nervous system, which causes a violation of the innervation of internal organs, vessels, endocrine glands. The causes of damage to the peripheral autonomic nervous system are primarily systemic, metabolic and endocrine diseases.

• Typical clinical manifestations of PVI are:
  • Orthostatic hypotension, which is manifested by pre-syncope and fainting.
  • Tachycardia at rest, fixed (rigid) pulse, arterial hypertension in the supine position.
  • Dyskinesia or paresis of the stomach, intestines, constipation, diarrhea.
  • Atony of the bladder, urinary incontinence, increased urgency of urination.
  • Impotence.
  • Hypohidrosis.
  • Dry eyes.
  • Dry mouth.
  • Decreased vision at dusk.
  • Sleep apnea.

Allocate primary (idiopathic, hereditary) PVI, caused by chronic slowly-progressive degenerative diseases with an unrecognized etiology, and secondary peripheral autonomic failure associated with a primary neurological or somatic disease. The main symptom of primary PVI is orthostatic hypotension, i.e. a transient clinically significant drop in systemic arterial pressure during the transition to an upright position or during prolonged standing.

Diagnostics is carried out on the basis of clinical data and identification of the underlying disease.

Treatment for peripheral autonomic insufficiency is symptomatic, in addition to therapy for the underlying disease.

Diseases in which peripheral autonomic insufficiency is observed are quite fully reflected in the etiological classification of peripheral autonomic insufficiency. Below are the most common causes of PVI that are not reflected in the classification.

• Primary forms of PVI, as a rule, are caused by diseases with an unknown etiology, such as:
  • Chronic slowly progressive diseases, which are based on the defeat of peripheral autonomic structures in its pure form ("pure" PVN), for example, Bradbury-Egglestone syndrome, idiopathic orthostatic hypotension, chronic idiopathic anhidrosis, postural orthostatic tachycardia syndrome.
  • Or diseases with parallel degeneration in other structures of the nervous system (for example, with parkinsonism or multisystem atrophy).
  • Or they are observed in the framework of hereditary polyneuropathies, when, along with autonomic fibers, motor and sensory ones suffer.

In the first two cases, the term "progressive autonomic failure" is sometimes used.

• Diseases in which primary PVI is observed:
  • Idiopathic form of primary PVI (Bradbury – Egglestone syndrome).
  • Idiopathic orthostatic hypotension (primary autonomic neuropathy).
  • Degenerative diseases of the nervous system (multisystem atrophy, Parkinson's disease).
  • Acute (subacute) pandizautonomy (autoimmune autonomic neuropathy (gangliopathy)).
  • Hereditary sensory autonomic neuropathies (in particular, Riley-Day syndrome).
  • Hereditary motor-sensory neuropathies (Charcot-Marie-Tooth disease).

• Secondary PVI is observed against the background of a somatic or neurological disease, such as:
  • Endocrine disorders (diabetes mellitus, hypothyroidism).
  • Immune disorders (amyloidosis, systemic connective tissue diseases, inflammatory demyelinating polyneuropathies (Guillain-Barré syndrome)).
  • Metabolic disorders, intoxication and drug disorders (alcoholism, porphyria, uremia, vitamin B deficiency, adrenergic blockers and adrenergic drugs, intoxication with arsenic, lead, vincristine, organophosphates, organic solvents, acrylamide).
  • Infectious diseases (herpes infections, AIDS, leprosy, syphilis).
  • Diseases of the central nervous system (some lesions of the spinal cord, tumors of the posterior cranial fossa, multiple sclerosis, syringomyelia, Wernicke's encephalopathy, hydrocephalus).

In the clinical picture of peripheral autonomic insufficiency, there are signs of impairment (decrease) in the function of the autonomic nervous system, which is manifested by cardiovascular, respiratory, genitourinary, gastrointestinal and some other disorders that can be observed in various combinations of pathological signs and can be of varying severity. The clinical manifestations of PVI are polysystemic and often nonspecific.

With a decrease in the function of the sympathetic system, there may be signs such as: orthostatic hypotension, hypo- or anhidrosis, ejaculatory dysfunction, ptosis (drooping of the upper eyelid caused by Horner's syndrome). With a weakening of parasympathetic influences, constipation, nausea, urinary retention, erectile dysfunction can be observed.

In secondary forms of PVI, in some cases, perspiration disorders prevail, in others tachycardia at rest (with diabetes mellitus) or gastrointestinal disorders (with amyloidosis, porphyria).

• • The forms of primary PVI include such syndromes as:
    • Idiopathic ("pure") autonomic failure.
    • Idiopathic orthostatic hypotension.
    • Autoimmune autonomic neuropathy (acute pandizautonomy).
    • Postural orthostatic tachycardia syndrome.
    • Family Disautonomy (Riley - Dey).
    • Shai-Dredger syndrome (multiple systemic atrophy (MSA) with a predominance of manifestations of peripheral autonomic failure).
    • Some other pathological conditions.

  Clinically, these syndromes are sometimes difficult to differentiate between themselves, especially at an early stage of the disease, which leads to some confusion in the nomenclature of primary syndromes.

  The term "pure" autonomic failure "includes disorders of autonomic functions not associated with the central nervous system. Idiopathic orthostatic hypotension (sometimes referred to as Bradbury-Egglestone syndrome) falls into the category of "pure" autonomic failure syndromes.

  Although patients with primary autonomic syndromes are characterized by a generality primarily of autonomic disorders, such as orthostatic hypotension, today it is obvious that different diseases underlie the syndromes. Patients with a clinical picture of "pure" autonomic failure may have autoimmune autonomic neuropathy, while others may have parkinsonism or multiple systemic atrophies.

  The morphological substrate of primary forms of PVI is degenerative changes in brain structures related to segmental and brainstem autonomic (adrenergic) and motor systems (substantia nigra, pallidus, lateral horns of the spinal cord, autonomic ganglia, etc.). Depending on the prevalence of the pathological process in the brain, concomitant neurological syndromes (parkinsonism, less often cerebellar syndrome, amyotrophy, myoclonus and other symptoms) may develop.

• • There are no diagnostic procedures and studies specific to peripheral autonomic failure.
  • Peripheral autonomic failure is a syndrome with a variety of causes. To clarify the diagnosis, it may be necessary to exclude all other possible causes of the existing clinical symptoms, for which additional research methods can be used.
  • When identifying one or more characteristics characteristic of a DPS, some of their features, characteristic of DPS, can be useful in diagnostics:
    • In the presence of fainting in the patient, the characteristic of syncope with PVI is the presence of hypo- and anhidrosis and the absence of a vagal reaction of slowing down the heart rate during an attack.
    • The detection of orthostatic hypotension along with hypohidrosis, tachycardia at rest, gastrointestinal disorders, and urinary disorders makes the diagnosis of PVI more likely.
    • Constipation and diarrhea can occur in seizures from several hours to several days, which is typical for PVI. Bowel function is normal between attacks.
    • Identification of painless myocardial infarction in the patient's anamnesis should lead one to think about PVI.
  • For the differential diagnosis of peripheral and central lesions of the autonomic nervous system, the determination of the level of norepinephrine (NA) in blood plasma is used. In a healthy person, in the supine position, the plasma NA indicator is kept at a constant level (110 - 410 pg / ml x 5.91 or 650 - 2423 pmol / l) and sharply increases upon transition to an upright position (123 - 700 pg / ml x 5, 91 or 739 - 4137 pmol / l). With central lesions of the autonomic nervous system, there is a certain level of NA in the plasma (normal or increased), which does not change when moving to an upright position. In peripheral lesions (postganglionic sympathetic neuron), the level of NA in the supine position is sharply reduced and does not increase with the orthostatic test. Plasma HA concentration can be used as an index of sympathetic nervous activity.
  • The diagnosis of PVI is largely a diagnosis of exclusion. If there is a suspicion of PVI, it is necessary to differentiate the primary forms from the secondary ones.
    • For primary forms of PVI, such manifestations as orthostatic hypotension, tachycardia at rest, hypohidrosis, impotence are more characteristic.
    • In secondary forms of PVI, in some cases, perspiration disorders prevail, in others tachycardia at rest (with diabetes mellitus) or gastrointestinal disorders (with amyloidosis, porphyria). ...
  • Diagnosis of secondary forms of autonomic failure involves the identification of the underlying disease.
  • The nature of the onset of PVI can provide additional clues when diagnosing:
    • Acute development of PVI symptoms in the absence of other neurological disorders or in the presence of possible complaints of weakness or numbness in the extremities requires the exclusion of acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome).
    • Subacute onset in the absence of other neurological or systemic disorders requires exclusion of autoimmune autonomic neuropathy. For this purpose, if possible, it is necessary to determine the presence of antibodies to the acetylcholine receptors of the autonomic ganglia (AChR) in the blood.
    • In the case of chronic onset of PVI, it is necessary to try to identify other neurological symptoms, in particular, to exclude parkinsonism and multisystem atrophy (MSA). There are no specific studies that can confirm these two diagnoses.
  • A positive family history of autonomic disorders with onset in the first decades of life may suggest congenital sensory or autonomic neuropathy.
  • It should be borne in mind that the use of drugs or toxic substances can cause generalized or organ-specific autonomic dysfunction. Chemical agents can have the following effects on vegetative functions:
    • An increase in sympathetic effects can be a consequence of the use of amphetamines, cocaine, tricyclic antidepressants, MAO inhibitors and beta-adrenergic agonists.
    • Weakening of sympathetic activity can be observed with the use of clonidine, methyldopa, reserpine, barbiturates, alpha and beta-blockers.
    • An increase in parasympathetic activity can be observed with the use of cholinomimetics (such as pilocarpine, bethanechol), or cholinesterase inhibitors (pyridostigmine), or organophosphate pesticides.
    • Weakening of parasympathetic activity can be observed with the use of antidepressants, phenothiazines, anticholinergic drugs, botulinum toxin.
  • In case of suspicion of secondary pandizautonomy (autoimmune autonomic neuropathy), the following laboratory tests are performed:
    • If diabetes mellitus is suspected, blood sugar and daily urine, glucose tolerance, C-peptide are determined.
    • Patients in whom autoimmune autonomic neuropathy is combined with cognitive disorders and sensory neuropathy should be examined for the presence of type 1 antineuronal antibodies (ANNA-1) in the blood serum to exclude paraneoplastic neuropathy.
    • In some cases, Eaton-Lambert syndrome (myasthenic syndrome with impaired presynaptic transmission) is associated with acute or subacute peripheral autonomic failure, and tumors are detected in half of these cases (small cell lung cancer in 80%). If Eaton-Lambert syndrome is suspected, antibodies to voltage-gated calcium channels are determined.
    • In some cases, botulism can be accompanied by severe symptoms of PVI. To diagnose botulism, the presence of botulinum toxin in the blood, vomit, gastric lavage, and feces is determined.
    • If familial amyloid neuropathy or polyneuropathy is suspected in primary systemic amyloidosis, Bens-Jones protein in urine is determined, as well as immunoelectrophoresis of serum and urine proteins, in which 85% of patients with primary and secondary amyloidosis are identified with monoclonal immunoglobulins.
    • If there is a suspicion of PVI associated with syphilis or AIDS, tests are performed for antibodies to Treponema pallidum (IgM and IgG) or human immunodeficiency virus 1, 2, respectively.
    • To diagnose porphyric polyneuropathy (observed in hepatic porphyria), the content of uroporphyrins is determined, including in daily urine (in particular, the Watson-Schwarz or Hosh tests), and the activity of porphobilinogen deaminase in erythrocytes is determined.
    • To diagnose PVI within the framework of polyneuropathy in diffuse connective tissue diseases (with rheumatoid arthritis, SLE, Sjogren's syndrome, systemic scleroderma), ESR is determined, C - reactive protein, rheumatoid factor, antinuclear antibodies (ANA), and other studies are carried out depending on the clinical situations.