Angina

Peripheral failure. Autonomic failure peripheral Autonomic failure syndrome

Peripheral autonomic failure

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

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

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

Diagnosis and differential diagnosis. The diagnosis is made clinically, taking into account the above complaints. The diagnostic search is aimed at excluding primary pathological processes that can lead to the development of peripheral autonomic failure.

Treatment is symptomatic. In case of acute adrenal insufficiency, corticosteroids are used. Sufficient administration of liquid and table salt can have a positive effect. In the absence of contraindications, it is possible to prescribe vasoconstrictor drugs (amisinium, ergotamine). Bandage the lower extremities with an elastic bandage, compression stockings or tights.

Raynaud's syndrome

Raynaud's syndrome is a vasospastic disease characterized by paroxysmal disorder of 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 collagenosis, rheumatoid arthritis, vasculitis, endocrine, neurological pathology, blood diseases, and occupational diseases. Clinically, Raynaud's syndrome is manifested by attacks, including sequential pallor, cyanosis and hyperemia of the fingers or toes, chin, and tip of the nose. Raynaud's syndrome leads to gradual trophic changes in tissue. 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 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 given to 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, caused by blood retention in the venules and arteriovenular anastomoses, skin pallor 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 assumed. The role of heredity, endocrine dysfunction, mental trauma, chronic nicotine and alcohol intoxication has been established in the occurrence of Raynaud's disease. Raynaud's disease is more common among women 20-40 years old who suffer from migraines.

Symptoms of Raynaud's syndrome

The 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, and sometimes the chin, ears and nose. Attacks of ischemia are initially short-lived and rare; occur 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, and a feeling of fullness. The attack ends with a sharp hyperemia of the skin and a feeling of heat.

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

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

Diagnosis of Raynaud's syndrome

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

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

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

Laser Doppler flowmetry, used to assess peripheral microcirculation, reveals defects in the 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.

Treatment of Raynaud's syndrome

The first principle of treatment for Raynaud's syndrome is the exclusion of provoking factors - smoking, cooling, vibration and other household and work factors. The primary disease that caused the development of Raynaud's syndrome is identified and treated.

Among the vasodilators for Raynaud's syndrome, the prescription of calcium antagonists - nifedipine (Cordafen, Corinfar, etc.), vasaprostan, selective calcium channel blockers - verapamil, nicardipine diltiazem - is effective. If necessary, ACE inhibitors (captopril) and selective HS2-serotonin receptor blockers (ketanserin) are used.

For Raynaud's syndrome, antiplatelet drugs are prescribed - dipyridamole, pentoxifylline (agapurine, trental), low molecular weight dextrans (reopolyglucin).

Progression and resistance of Raynaud's syndrome to drug therapy is an indication for surgical sympathectomy or ganglionectomy.

In the event of an ischemic attack, immediate measures include warming the limb in warm water, massaging with woolen cloth, and offering the patient a hot drink. In case of a prolonged attack, injectable forms of antispasmodics (no-shpa, platyphylline), seduxen and other drugs are prescribed.

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

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

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; ischemic attacks can spontaneously stop after a change in habits, climate, profession, sanatorium treatment, etc.

The absence of primary preventive measures allows us to speak only about secondary prevention of Raynaud's syndrome, i.e., about the exclusion of trigger factors leading to vasospasm - hypothermia, vibration, smoking, psycho-emotional stress.

Damage to the peripheral structures of the autonomic nervous system, leading to a disorder of the innervation of the somatic systems of the body. It is distinguished by polymorphism, polysystemism, and the dysfunctional nature of symptoms. The leading clinical manifestation is orthostatic syndrome. The diagnosis is based on the results of cardiovascular testing, examination of major organs and systems, and biochemical blood tests. Treatment is symptomatic and consists of a combination of non-drug methods and drug therapy.

General information

Peripheral autonomic failure (PVF) is a complex concept in neurology, which includes lesions of the peripheral part of the autonomic nervous system (ANS) of various etiologies. The latter is also called autonomous. Ensures the functioning of somatic organs and systems through their sympathetic and parasympathetic innervation. The autonomic nervous system regulates vascular tone, respiratory rhythm, intestinal peristalsis, bladder tone, and the functioning of the lacrimal, sweat, and sex glands. In domestic medicine, A. M. Vein was deeply involved in research into the ANS. In 1991, he and his co-authors proposed an etiological classification of PVN, which is widely used today.

Causes of PVN

Primary (idiopathic) autonomic failure is caused by genetically determined degenerative changes in the fibers of the peripheral ANS. It is often combined with degeneration of other structures of the nervous system: Parkinson’s disease, hereditary polyneuropathies, multiple system atrophy. More common is secondary PVN, which occurs against the background of the underlying disease. The peripheral ANS is affected by infectious diseases (herpes infection, syphilis, AIDS, leprosy), but more often the etiofactor is variable dysmetabolic disorders, which lead to:

Endocrine disorders: adrenal insufficiency, diabetes, hypothyroidism. Pathology of the endocrine glands provokes metabolic disorders, trophic disorders of nerve fibers, leading to loss of their function.
Systemic diseases: Sjögren's disease, amyloidosis, systemic scleroderma. Cause autoimmune damage to vegetative fibers.
Chronic intoxication for alcoholism, uremic syndrome, liver failure. Violation of the excretory function of the kidneys and detoxification processes in the liver cause the accumulation of toxic compounds in the body, the adverse effects of which are exposed to the peripheral ANS.
Toxic lesions lead, organophosphorus compounds, manganese, drugs. The latter include adrenergic blockers, dopa-containing medications, and anticholinesterase drugs.
Oncological diseases accompanied by dysmetabolic processes. In such cases, autonomic failure is part of the structure of paraneoplastic syndrome.
Respiratory failure, leading to hypoxia of nerve fibers. Develops in chronic pathology of the respiratory system.

Pathogenesis

Coordinated regulation of the work of internal organs (gastrointestinal tract, cardiovascular, respiratory system, urinary tract), excretory activity of the glands is ensured by sympathetic and parasympathetic innervation. Dysfunction of a certain part of the ANS that occurs under the influence of etiological factors causes functional disorders in the structures innervated by it. The most noticeable are vascular disorders, manifested by a drop in blood pressure when the body position changes from horizontal to vertical. Due to the lack of compensatory narrowing of peripheral vessels, blood is deposited in the lower parts of the body. Blood supply to the brain sharply decreases, which is clinically characterized by presyncope or syncope.

Orthostatic hypotension has several mechanisms of development. A disorder of the autonomic innervation of the veins of the lower extremities leads to the retention of a large volume of blood in them. Sympathetic denervation of the arteries leads to loss of their tonic response. A malfunction in the functioning of the afferent autonomic nerves that provide the baroreceptor reflex causes blocking of impulses carrying information about changes in vascular pressure.

Classification

Based on etiology, peripheral autonomic failure is divided into primary and secondary. Depending on the course, acute and chronic disorders are distinguished. Below is the classification proposed in 1996 by Low, supplemented in 2000 by Mathias.

Primary PVN includes cases with unknown or genetically determined etiology. Some forms are hereditary.

Isolated autonomic failure is a lesion of preganglionic autonomic neurons of the spinal cord. The main manifestation is orthostatic hypotension.
Idiopathic autonomic neuropathy. The autoimmune nature of the disease is assumed to be related to the Epstein-Barr virus. Orthostatic syndrome is combined with dry mucous membranes and sensory disorders.
Idiopathic anhidrosis. The autonomic regulation of the sweat glands is disrupted. Manifested by dry skin.
Postural orthostatic tachycardia. It is observed in young people; women get sick 5 times more often. A typical clinical symptom is tachycardia when moving to a vertical position.
Autonomic failure in the elderly. The typical age of the patients is over 60 years.
Dysimmune autonomic neuropathies. They have an acute course. They are divided into adrenergic, cholinergic and mixed (pandysautonomia).
Hereditary autonomic neuropathies. Includes familial amyloid form, Fabry disease, Riley-Day syndrome, etc.

Secondary military equipment develops as a result of lesions of vegetative structures of various origins. It appears against the background of the clinical picture of the underlying pathology.

PVN of dysmetabolic conditions. There are diabetic, amyloid, porphyritic, uremic, and nutritional variants.
PVN of toxic lesions. This includes alcohol, medicinal forms, and peripheral insufficiency in case of poisoning.
PVN for inflammatory polyneuropathies. May occur with Guillain-Barré syndrome, CIDP.
Infectious PVN. Accompanies diphtheria, botulism, HIV, borreliosis, Hagas disease.
PVN of oncological diseases. May manifest as paraneoplastic dysautonomia, intestinal dysfunction (pseudoileus), myasthenic symptom complex, sensory polyneuropathy.
PVN of systemic pathology. Characteristic of collagenosis, systemic intestinal pathology (UC, Crohn's disease).
PVN for other diseases. Includes pathology of the ANS in chronic damage to the respiratory system, multiple lipomatosis.

Symptoms of PVN

Clinical manifestations are multisystem in nature and are not very specific. Failure in the functioning of the sympathetic department of the ANS is manifested by hypohidrosis, diarrhea, ptosis, urinary incontinence, and ejaculation disorders. Weakening of parasympathetic innervation causes constipation, hyperhidrosis, urinary retention, and impotence. Primary peripheral failure occurs with a predominance of orthostatic hypotension, secondary - depends on the underlying disease.

Cardiovascular disorders characterized by orthostatic tachycardia and hypotension, arterial hypertension in a horizontal position. Postural hypotension when standing up is felt by the patient as an attack of lightheadedness, weakness, or a fainting episode. The patient may experience a feeling of “failing,” darkening before the eyes, and noise in the head. In severe cases, the attack ends in fainting lasting about 10 seconds. Orthostatic tachycardia is accompanied by an increase in heart rate by 30 beats/min. with a slight decrease in blood pressure. Patients complain of palpitations, anxiety, sweating, and pain in the cardiac region. In some cases, resting tachycardia is observed (heart rate is fixed at 95-100 beats/min), independent of physical activity.

Respiratory system dysfunction manifested by short-term episodes of respiratory arrest (including sleep apnea), attacks of suffocation. Severe respiratory distress with blocked cardiovascular reflexes can cause sudden death.

Gastrointestinal disorders include dyskinesias and atony of the stomach, esophagus, intestines, biliary tract, and gall bladder. Intestinal dysfunction is expressed by periodic constipation or diarrhea. Gastric atony is manifested by a feeling of fullness, anorexia, vomiting, and nausea.

Genitourinary disorders may be accompanied by polyuria at night, increased frequency of urges, a feeling of incomplete emptying of the bladder, incontinence, difficulty urinating, erectile dysfunction in men, decreased sensitivity of the clitoris in women. Up to 90% of men with primary forms of PVN suffer from impotence.

Visual disorders. There is a constriction of the pupils, a disorder of their adaptation in the dark. As a result, twilight vision deteriorates.

Dysfunction of the excretory glands. The functioning of the sweat glands can be disrupted locally, symmetrically, or diffusely. Hypohidrosis is more common. Hyperhidrosis can be nocturnal. A decrease in tear production causes the development of dry eye syndrome, and a disorder of salivation causes dry mouth.

Complications

Severe disorders of autonomic innervation negatively affect the daily life of patients. Patients with severe orthostatic hypotension without adequate therapy often become bed sick. Disturbances in afferent cardiac innervation lead to a painless course of myocardial infarction, which makes it difficult to identify and stop. Gastrointestinal disorders lead to decreased nutrition, poor absorption of nutrients, and hypovitaminosis. Urogenital dysfunction is complicated by the addition of infection with the development of cystitis, urethritis, pyelonephritis; leads to infertility. Dry eye mucosa threatens the occurrence of conjunctivitis, keratitis, and uveitis.

Diagnostics

Peripheral autonomic failure is characterized by a large polymorphism of symptoms. The nonspecificity of clinical symptoms makes diagnosis difficult and requires a comprehensive examination of the patient. The presence of syncope with hypohidrosis argues in favor of PVN. The diagnosis is more likely when these manifestations are combined with gastrointestinal and urinary disorders. Instrumental studies are necessary to exclude organic pathology, if primary peripheral failure is suspected, to identify the underlying pathology in secondary forms. The diagnostic complex includes:

Cardiovascular tests. An orthostatic test diagnoses a drop in systolic pressure >20 mmHg. Art., diastolic - >10 mm Hg. Art. There is an insufficient increase in heart rate during isometric exercise (squeezing the dynamometer), standing up, deep breathing, and the Valsalva maneuver.
Neurological examination. An examination by a neurologist can reveal miosis, signs of polyneuropathy, parkinsonism, multiple sclerosis, and organic damage. In some cases, the neurological status is normal. Electroneurography helps to assess the condition of the nerve trunks and exclude muscle pathology. MRI of the brain in primary forms can diagnose degenerative processes, atrophic changes, in secondary forms - the underlying disease (..., aimed at comprehensive relief of emerging symptoms. In accordance with the indications, the following is carried out:
- Relief of orthostatic hypotension. It has 2 directions: increasing the volume of circulating blood, reducing the space it occupies. Non-medicinal methods are used: increased fluid and salt intake, fractional meals, situational intake of coffee, tea, bandaging of the lower extremities (wearing compression tights), avoidance of sudden changes in body position. The lack of effectiveness of these methods is an indication for pharmacotherapy. Caffeine preparations, sympathomimetics, in some cases - non-steroidal anti-inflammatory drugs, and for orthostatic tachycardia - beta blockers (propranolol) are prescribed. Long-term therapy for hypotension is carried out with amesinium methyl sulfate.
- Treatment of urinary disorders. Antidiuretic hormone, exercises with tension in the pelvic floor muscles, and electrical stimulation of the bladder are effective. For frequent urges and incontinence, antispasmodics are indicated, for difficulty in emptying - cholinergic drugs, for sphincter spasms - adrenoblockers.
- Treatment of atonic gastrointestinal disorders. It is carried out with antiemetic, anticholinesterase, prokinetic medications. For constipation, herbal laxatives and cellulose preparations are prescribed.
- Stimulation of glandular excretion. It is carried out with pilocarpine. Nicotinic acid and vitamin A help reduce dry mouth. Moisturizing the skin is achieved with creams and oil compresses. Severe xerophthalmia, while maintaining the function of other glands, is an indication for surgical treatment - moving the parotid duct into the conjunctival sac.
Prognosis and prevention
The course and outcome depend on the form of the disease. Isolated peripheral failure may occur with periods of exacerbation, and gradual regression of symptoms is possible. Idiopathic neuropathy has a progressive course. Postural tachycardia often spontaneously regresses until complete recovery. The situation becomes more complicated when the peripheral autonomic nervous system is affected in combination with degenerative changes in the central nervous system. The prognosis of secondary forms is related to the underlying disease. Prevention of PVN has not been developed.

Classification: idiopathic and for hereditary neuropathies.

Clinical symptoms of PVP

Fainting 95%

Impotence 92%

General weakness 92%

Hypo, anhidrosis 70%

Arterial hypotension in the supine position 50%

Urinary incontinence 43%

Constipation, gastroparesis, diarrhea 41%

Clinical manifestations of IOH(idiopathic orthostatic hypotension)

A drop in systolic blood pressure by more than 30 mm Hg when standing up. (normal is 10)

Lipotymic states and fainting. The only fainting spells from which the patient can die. Usually, during fainting, the skin is moist, there is tachycardia, and after fainting, blood pressure is restored. Here the skin is dry, there is no tachycardia, blood pressure is very low. Blood pressure does not increase after fainting.

Hypo- and anhidrosis

"Fixed" pulse

Tachycardia at rest

Reduction of clinical symptoms in a horizontal position

Patients feel more or less satisfactory in a horizontal position and in a pool. Any load, food intake, etc. causes the condition to worsen.

The disease is based on damage to the PVS (baroreceptors of the aortic arch, sympathetic vasoconstrictors of muscles, arteries and internal organs).

Treatment of postural hypotension

Non-pharmacological

Avoid
1. Sudden rise of the head, especially in the morning
  Stress in the toilet
  Sudden changes in temperature
  Heavy loads
  Alcohol
  Overeating, especially carbohydrates and fats

Use

Elevated head position
Frequent small meals
Increasing salt in the diet
Elastic stockings, bandages, bandages (in a horizontal position, stand up after the bandage)
Anti-gravity suit (astronaut suit)

Pharmacological

Mineralocorticoids
Vasoconstrictors - sympathomimetics (midodrine, dihydroergotamine, ephedrine, MAO-A inhibitors, tyramine)
Preventing vasodilation (indomethacin, cerucal, propranolol)
Preventing hypotension after meals (caffeine, somatostatin)
Increase cardiac output (pindolol)
Increasing the number of red blood cells and oxygen (erythropoietin)
Reducing nocturnal polyuria (desmopressin)

Principles of treatment

Increase the amount of circulating blood (erythropoietin, salt)

Reduce the volume where blood circulates (vasoconstrictors, anti-gravity suit, swimming)

Horner's syndrome (enophthalmos, miosis, ptosis, heterochromia of the iris and anhidrosis of the upper part of the face of the same name) - the sympathetic innervation of the eye suffers.

Argay-Robertson syndrome (narrow pupils, deformed pupils, lack of direct and friendly reaction to light, but the reaction of the pupils to convergence is preserved) is pathognomonic for all forms of neurosyphilis. RW should be investigated, but the syndrome can also be positive in patients with repeated traumatic brain injury, meningoencephalitis, orbital trauma, subarachnoid hemorrhage, one or more general anesthesia, abuse of luminal, benzodiazepines and narcotics.

AD syndrome (pilotonic reaction of the pupil to light, the pupil is dilated, less often constricted, usually one pupil is affected at the beginning of the development of the syndrome) - observed in women 20-40 years old. Lack of knee reflexes. It may end on one eye and move on to the other. During bright seasons, it is recommended to wear safety glasses. It is advised not to instill miotics, but to instill miotics into the other eye before an important task. Sleep apnea. All diseases with a violation of the autonomic innervation of the digestive tract organs are accompanied by the same symptoms as diseases of these organs.

Organic impotence does not respond to emotions. The parasympathetic innervation of the corpora cavernosa suffers. Erection and sexual intercourse are impossible. Introduction of noshpa and papaverine into the corpora cavernosa.

During treatment, preopism may develop - prolonged painful erection, gangrene, painful shock, death.

Diagnosis of PVN

The difference between systolic blood pressure while lying down and when standing up is 30 mmHg (normally 10)

Norepinephrine in plasma

Lying down reduced (normally normal)
Decreased when standing up (normally decreased)

Test with norepinephrine – a significant increase in blood pressure or a decrease (normally an increase)

Test with anaprilin - does not work (normally reduces heart rate)

Test with atropine - no effect (normally increases heart rate)

Heart rate remains virtually unchanged during deep breathing (less than 1.2 times)

Sample 30:15 – 1.0

Valsalva ratio – practically does not change heart rate

It is more convenient to talk with patients about urination, because... When urination is impaired, the functioning of the rectum is also usually impaired. Neurogenic urinary disorders:

Incontinence and urgency

Reducing bubble contraction
1. Platyfillin
2. Amitriptyline
  Baclofen
  Indomethacin
  Parlodel

Decrease

b-blockers
External urine collection
Intermittent catheterization
Detrusitol

Delay

To increase intravesical pressure:
1. Cholinomimetics
  Anticholinesterase
  Trigger zone stimulation
  Electrical stimulation of the bladder

Relaxation of the external sphincter

Prazosin
Transurethral resection of the bladder neck
Dissection of the pudendal nerve
Intermittent catheterization

Sphincter-detrusor dyssynergia.

Urination centers: in the spinal cord, pons and postcentral gyrus.

Autonomic failure is a syndrome associated with diffuse disruption of the innervation of internal organs, blood vessels, and 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 simultaneously, but sometimes dysfunction of one of these parts predominates.

Etiology. Autonomic failure can be primary or secondary. Primary autonomic failure is caused by hereditary or degenerative diseases involving autonomic neurons of the central nervous system or neurons of the autonomic ganglia (eg, hereditary polyneuropathies, primary autonomic neuropathy, or multiple system atrophy). Secondary autonomic failure most 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, with a stroke, traumatic brain injury or tumor).

The clinical picture of autonomic failure consists of symptoms of damage to various organs and systems. Dysfunction of the cardiovascular system is primarily manifested by orthostatic hypotension, characterized by impaired maintenance of 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 moving to a vertical position, they do not narrow and blood is deposited in these vessels. Renal denervation 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 to a vertical position or during prolonged standing (especially in a stationary position) by dizziness, blurred vision, headache or heaviness in the back of the head, and a sudden feeling of weakness. With a sharp drop in blood pressure, fainting is possible. With severe orthostatic hypotension, patients become bedridden. In the supine position, blood pressure can, on the contrary, increase 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 eating food that 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, and 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.

A disorder of the innervation of the sweat glands usually leads to decreased sweating (hypohidrosis, anhidrosis) and dry skin, but some patients experience regional hyperhidrosis (for example, in the face or hands) or night sweats.

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

Diagnosis of autonomic failure is facilitated by identifying 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 (eg, antihypertensive drugs), blood and cardiovascular diseases, endocrine disorders (eg, adrenal insufficiency).

Treatment primarily involves addressing the underlying disease. Symptomatic treatment is determined by the leading syndrome. For orthostatic hypotension, a set of non-drug measures is recommended.

It is necessary to avoid provoking factors: straining (if you are prone to constipation, foods high in dietary fiber and sometimes laxatives are recommended), sudden changes in body position, thermal procedures, overheating, drinking alcohol, prolonged bed rest, intense physical exercise, especially isometric exercise.

Moderate physical activity in an isotonic mode is recommended, especially in an aquatic environment.

If you need to stand for a long time, you should often change your position, shifting and lifting one or the other leg, crossing your legs, squatting, crossing or tucking your legs in a sitting position, crossing one leg over the other, changing their places from time to time. All these techniques prevent blood from pooling in the legs.

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

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

Sometimes wearing elastic stockings, which should be pulled on in the morning, before getting up in bed, helps.

If the above measures are not effective enough. then they resort to medications that increase circulating blood volume and vascular tone. The most effective of them is the fluorine-containing synthetic corticosteroid fludrocortisone (Cortinef). Sometimes it is combined with other drugs that increase the tone of the sympathetic system (for example, midodrine). All drugs that reduce orthostatic hypotension increase hypertension in the supine position, so during treatment it is necessary to monitor blood pressure not only before taking the drug and 1 hour after taking it, but also in the morning after waking up.

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

Etiology.

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

Dysfunction of the gastrointestinal tract leads to impaired motility of the stomach, intestines, and 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 to strain at the beginning of urination, and a feeling of incomplete emptying of the bladder. A disorder of the innervation of the sweat glands usually leads to decreased sweating (hypohidrosis, anhidrosis) and dry skin, but some patients experience regional hyperhidrosis (for example, in the face or hands) or night sweats.

Diagnosis.

To identify 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 indicated by a drop in systolic pressure in a vertical position of at least 20 mmHg. Art., and diastolic - by at least 10 mm Hg. Art. To determine whether the pulse is fixed, its fluctuations are assessed during deep breathing, straining, changing body position, and physical activity. Motility of the gastrointestinal tract is examined using fluoroscopy or endoscopic techniques. To clarify the nature of the urination disorder, cystometry and ultrasound examination are performed.

Treatment.

Treatment primarily involves addressing the underlying disease. Symptomatic treatment is determined by the leading syndrome. For orthostatic hypotension, a set of non-drug measures is recommended.

Moderate physical activity in an isotonic mode is recommended, especially in an aquatic environment.
If you need to stand for a long time, you should often change your position, shifting and lifting one or the other leg, crossing your legs, squatting, crossing or tucking your legs in a sitting position, crossing one leg over the other, changing their places from time to time. All these techniques prevent blood from pooling in the legs.

Central cholinomimetics: rivastigmine (Exelon), donepezil (Aricept), amiridin, gliatilin, etc.;

Nonsteroidal anti-inflammatory drugs (eg, ibuprofen);

If possible, stop taking or reduce the dose of vasodilators.
In the absence of heart failure, it is recommended to increase salt intake (up to 4-10 g/day) and fluid (up to 3 l/day), but limit fluid intake at night, eat more often, but in small portions, avoiding high-carbohydrate foods.
You need to sleep with your head held high (the head should be raised 15-20 cm); this reduces not only orthostatic hypotension, but also nocturnal polyuria and hypertension in the supine position.
Sometimes wearing elastic stockings, which should be pulled on in the morning, before getting up in bed, helps.

If the above measures are not effective enough, then they resort to medications that increase the volume of circulating blood and vascular tone. The most effective of them is the fluorine-containing synthetic corticosteroid fludrocortisone (Cortinef). Sometimes it is combined with other drugs that increase the tone of the sympathetic system (for example, midodrine). All drugs that reduce orthostatic hypotension increase hypertension in the supine position, so during treatment it is necessary to monitor blood pressure not only before taking the drug and 1 hour after taking it, but also in the morning after waking up.

Peripheral autonomic failure is a lesion of the peripheral structures of the autonomic nervous system, leading to a disorder of the innervation of the somatic systems of the body. It is distinguished by polymorphism, polysystemism, and the dysfunctional nature of symptoms. The leading clinical manifestation is orthostatic syndrome. The diagnosis is based on the results of cardiovascular testing, examination of major organs and systems, and biochemical blood tests. Treatment is symptomatic and consists of a combination of non-drug methods and drug therapy.

Peripheral autonomic failure

Causes of PVN

Endocrine disorders: adrenal insufficiency, diabetes, hypothyroidism. Pathology of the endocrine glands provokes metabolic disorders, trophic disorders of nerve fibers, leading to loss of their function.
Systemic diseases: Sjogren's disease, amyloidosis, systemic scleroderma. Cause autoimmune damage to vegetative fibers.
Chronic intoxication due to alcoholism, uremic syndrome, liver failure. Violation of the excretory function of the kidneys and detoxification processes in the liver cause the accumulation of toxic compounds in the body, the adverse effects of which are exposed to the peripheral ANS.
Toxic damage from lead, organophosphorus compounds, manganese, drugs. The latter include adrenergic blockers, dopa-containing medications, and anticholinesterase drugs.
Oncological diseases accompanied by dysmetabolic processes. In such cases, autonomic failure is part of the structure of paraneoplastic syndrome.
Respiratory failure leading to hypoxia of nerve fibers. Develops in chronic pathology of the respiratory system.

Pathogenesis

Classification

Primary PPV includes cases with an unknown or genetically determined etiology. Some forms are hereditary.

Isolated autonomic failure is a lesion of preganglionic autonomic neurons of the spinal cord. The main manifestation is orthostatic hypotension.
Idiopathic autonomic neuropathy. The autoimmune nature of the disease is assumed to be related to the Epstein-Barr virus. Orthostatic syndrome is combined with dry mucous membranes and sensory disorders.
Idiopathic anhidrosis. The autonomic regulation of the sweat glands is disrupted. Manifested by dry skin.
Postural orthostatic tachycardia. It is observed in young people; women get sick 5 times more often. A typical clinical symptom is tachycardia when moving to a vertical position.
Autonomic failure in the elderly. The typical age of the patients is over 60 years.
Dysimmune autonomic neuropathies. They have an acute course. They are divided into adrenergic, cholinergic and mixed (pandysautonomia).
Hereditary autonomic neuropathies. Includes familial amyloid form, Fabry disease, Riley-Day syndrome, etc.

Secondary PVN develops as a result of lesions of vegetative structures of various origins. It appears against the background of the clinical picture of the underlying pathology.

PVN of dysmetabolic conditions. There are diabetic, amyloid, porphyritic, uremic, and nutritional variants.
PVN of toxic lesions. This includes alcohol, medicinal forms, and peripheral insufficiency in case of poisoning.
PVN for inflammatory polyneuropathies. May occur with Guillain-Barre syndrome, CIDP.
Infectious PVN. Accompanies diphtheria, botulism, HIV, borreliosis, Hagas disease.
PVN of oncological diseases. May manifest as paraneoplastic dysautonomia, intestinal dysfunction (pseudoileus), myasthenic symptom complex, sensory polyneuropathy.
PVN of systemic pathology. Characteristic of collagenosis, systemic intestinal pathology (UC, Crohn's disease).
PVN for other diseases. Includes pathology of the ANS in chronic damage to the respiratory system, multiple lipomatosis.

Symptoms of PVN

Cardiovascular disorders are characterized by orthostatic tachycardia and hypotension, arterial hypertension in a horizontal position. Postural hypotension when standing up is felt by the patient as an attack of lightheadedness, weakness, or a fainting episode. The patient may experience a feeling of “failing,” darkening before the eyes, and noise in the head. In severe cases, the attack ends in fainting lasting about 10 seconds. Orthostatic tachycardia is accompanied by an increase in heart rate by 30 beats/min. with a slight decrease in blood pressure. Patients complain of palpitations, anxiety, sweating, and pain in the cardiac region. In some cases, resting tachycardia is observed (heart rate is fixed at the level of beats/min.), independent of physical activity.

Dysfunction of the respiratory system is manifested by short-term episodes of respiratory arrest (including sleep apnea), attacks of suffocation. Severe respiratory distress with blocked cardiovascular reflexes can cause sudden death.

Gastrointestinal disorders include dyskinesia and atony of the stomach, esophagus, intestines, biliary tract, and gall bladder. Intestinal dysfunction is expressed by periodic constipation or diarrhea. Gastric atony is manifested by a feeling of fullness, anorexia, vomiting, and nausea.

Genitourinary disorders may be accompanied by polyuria at night, increased urge, a feeling of incomplete emptying of the bladder, incontinence, difficulty urinating, erectile dysfunction in men, and decreased sensitivity of the clitoris in women. Up to 90% of men with primary forms of PVN suffer from impotence.

Visual disorders. There is a constriction of the pupils, a disorder of their adaptation in the dark. As a result, twilight vision deteriorates.

Dysfunction of the excretory glands. The functioning of the sweat glands can be disrupted locally, symmetrically, or diffusely. Hypohidrosis is more common. Hyperhidrosis can be nocturnal. A decrease in tear production causes the development of dry eye syndrome, and a disorder of salivation causes dry mouth.

Complications

Severe disorders of autonomic innervation negatively affect the daily life of patients. Patients with severe orthostatic hypotension without adequate therapy often become bed sick. Disorders of afferent cardiac innervation lead to a painless course of myocardial infarction, which makes it difficult to identify and stop it. Gastrointestinal disorders lead to decreased nutrition, poor absorption of nutrients, and hypovitaminosis. Genitourinary dysfunction is complicated by infection with the development of cystitis, urethritis, pyelonephritis; leads to infertility. Dry eye mucosa threatens the occurrence of conjunctivitis, keratitis, and uveitis.

Diagnostics

Cardiovascular tests. An orthostatic test diagnoses a drop in systolic pressure >20 mmHg. Art., diastolic - >10 mm Hg. Art. There is an insufficient increase in heart rate during isometric exercise (squeezing the dynamometer), standing up, deep breathing, and the Valsalva maneuver.
Neurological examination. An examination by a neurologist can reveal miosis, signs of polyneuropathy, parkinsonism, multiple sclerosis, and organic damage. In some cases, the neurological status is normal. Electroneurography helps to assess the condition of the nerve trunks and exclude muscle pathology. MRI of the brain in primary forms can diagnose degenerative processes, atrophic changes, in secondary forms - the underlying disease (brain tumor).
Cardiac examination. Necessary to exclude/detect cardiac pathology. A consultation with a cardiologist, ECG, rhythmocardiography, and ultrasound of the heart are carried out.
Gastrointestinal tract diagnostics. Performed by a gastroenterologist and endoscopist. Contrast radiography of the stomach and intestines, endoscopy, and, if indicated, MSCT of the abdominal cavity are prescribed.
Examination of the urinary tract. Conducted by a urologist. The examination includes the Rehberg test, urodynamic tests, cystoscopy, excretory urography.
Biochemical blood test. Helps to identify the toxic etiology of the disease, assess kidney function, liver function, and the degree of dysmetabolic disorders.

Peripheral ANS insufficiency requires differential diagnosis with absence epilepsy, fainting of other etiologies, Guillain-Barré syndrome, cardiovascular pathology (cardiomyopathy, mitral valve prolapse), inflammatory polyneuropathies, endocrine diseases (pheochromocytoma, Addison's disease). It is necessary to exclude electrolyte disturbances and side effects of certain pharmaceuticals.

Treatment of PVN

Specific therapy has not yet been developed. Treatment is carried out by a neurologist-vegetologist and is aimed at comprehensive relief of emerging symptoms. In accordance with the indications, the following is carried out:

Relief of orthostatic hypotension. It has 2 directions: increasing the volume of circulating blood, reducing the space it occupies. Non-medicinal methods are used: increased fluid and salt intake, fractional meals, situational intake of coffee, tea, bandaging of the lower extremities (wearing compression tights), avoidance of sudden changes in body position. The lack of effectiveness of these methods is an indication for pharmacotherapy. Caffeine preparations, sympathomimetics, in some cases - non-steroidal anti-inflammatory drugs, and for orthostatic tachycardia - beta blockers (propranolol) are prescribed. Long-term therapy for hypotension is carried out with amesinium methyl sulfate.
Treatment of urinary disorders. Antidiuretic hormone, exercises with tension in the pelvic floor muscles, and electrical stimulation of the bladder are effective. For frequent urges and incontinence, antispasmodics are indicated, for difficulty in emptying - cholinergic drugs, for sphincter spasms - adrenoblockers.
Treatment of atonic gastrointestinal disorders. It is carried out with antiemetic, anticholinesterase, prokinetic medications. For constipation, herbal laxatives and cellulose preparations are prescribed.
Stimulation of gland excretion. It is carried out with pilocarpine. Nicotinic acid and vitamin A help reduce dry mouth. Moisturizing the skin is achieved with creams and oil compresses. Severe xerophthalmia, while maintaining the function of other glands, is an indication for surgical treatment - moving the parotid duct into the conjunctival sac.

Prognosis and prevention

The course and outcome depend on the form of the disease. Isolated peripheral failure may occur with periods of exacerbation, and gradual regression of symptoms is possible. Idiopathic neuropathy has a progressive course. Postural tachycardia often spontaneously regresses until complete recovery. The situation becomes more complicated when the peripheral autonomic nervous system is affected in combination with degenerative changes in the central nervous system. The prognosis of secondary forms is related to the underlying disease. Prevention of PVN has not been developed.

Peripheral autonomic failure - treatment in Moscow

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Peripheral autonomic failure - Symptoms

Symptoms of peripheral autonomic failure are present in all physiological systems of the body and can occur under the guise of many somatic diseases. Typical clinical syndromes are the following:

Orthostatic hypotension.
Tachycardia at rest.
Hypertension in the supine position.
Hypohidrosis.
Impotence.
Gastroparesis.
Constipation.
Diarrhea.
Urinary incontinence.
Decreased vision at dusk.
Sleep apnea.

These syndromes are presented in the sequence that corresponds to the prevailing frequency of occurrence. However, in each specific case of peripheral autonomic failure, the “set” of symptoms may be different and not always complete (11 signs). Thus, for primary forms of peripheral autonomic failure, such manifestations as orthostatic hypotension, tachycardia at rest, hypohidrosis, and impotence are more typical. With secondary syndromes of peripheral autonomic failure, in some cases sweating disorders dominate (with alcoholism, polyneuropathy), in others - tachycardia at rest (with diabetes) or gastrointestinal disorders (amyloidosis, porphyria), etc. It is not surprising that patients with signs of autonomic failure are addressed to specialists of various profiles - cardiologists, neurologists, gynecologists, sex therapists, geriatricians, etc.

The most dramatic manifestation of peripheral autonomic failure in the cardiovascular system is orthostatic hypotension, leading to frequent fainting when moving to an upright position or during prolonged standing. Orthostatic hypotension is a condition that occurs in a variety of diseases (neurogenic syncope, anemia, varicose veins, heart disease, etc.). It should, however, be noted that in case of peripheral autonomic failure, orthostatic hypotension is caused by damage to the lateral horns of the spinal cord and/or efferent sympathetic vasomotor conductors that exert vasoconstrictive effects on peripheral and visceral vessels. Therefore, during orthostatic loads, peripheral vasoconstriction does not occur, which leads to a drop in systemic blood pressure, and then, accordingly, to acute anoxia of the brain and the development of fainting.

Patients may experience varying severity of clinical manifestations. In mild cases, soon after assuming a vertical position (standing up), the patient begins to notice signs of a pre-fainting state (lipothymia), manifested by a feeling of lightheadedness, dizziness, and a premonition of loss of consciousness. The patient, as a rule, complains of general weakness, darkening of the eyes, noise in the ears and head, unpleasant sensations in the epigastric region, sometimes a feeling of “sinking,” “floating away from under the feet,” etc. Pallor of the skin is noted. integument, short-term postural instability. The duration of lipothymia is 3-4 s. In more severe cases, following lipothymia, fainting may develop. The duration of fainting with peripheral autonomic failure is 8-10 seconds, sometimes (with Shy-Drager syndrome) - several tens of seconds. During fainting, diffuse muscle hypotension, dilated pupils, upward abduction of the eyeballs, threadlike pulse, and low blood pressure (60-50/40-30 mm Hg and below) are noted. If fainting lasts more than 10 seconds, convulsions, hypersalivation, loss of urine may occur, and in extremely rare cases, tongue biting may occur. Severe orthostatic circulatory disorders can lead to death. Fainting states with peripheral autonomic failure differ from other fainting states by the presence of hypo- and anhidrosis and the absence of a vagal reaction to slow PC. To assess the severity of orthostatic disorders, in addition to taking into account clinical manifestations, it is convenient to use the indicator of the rate of onset of fainting after assuming a vertical body position. The time interval from the moment the patient transitions from a horizontal to a vertical position until the development of syncope is reduced to several minutes or even to 1 minute or less. This indicator is always adequately indicated by the patient and quite accurately characterizes the severity of orthostatic circulatory disorders. In dynamics, it also reflects the rate of progression of the disease. In severe cases, fainting can occur even while sitting.

Orthostatic hypotension is the main symptom of primary peripheral autonomic failure. Secondarily, it can be observed in diabetes mellitus, alcoholism, Guillain-Barre syndrome, chronic renal failure, amyloidosis, porphyria, bronchial carcinoma, leprosy and other diseases.

Along with orthostatic hypotension, with peripheral autonomic failure, a phenomenon such as arterial hypertension in the supine position often develops. As a rule, in these cases, when lying down for a long time during the day or during night sleep, blood pressure rises to high numbers (/mm Hg). These changes in blood pressure are caused by the so-called post-denervation hypersensitivity of adrenergic receptors of vascular smooth muscles, which inevitably forms during chronic denervation processes (Canon's law of post-denervation hypersensitivity). Taking into account the possibility of arterial hypertension in patients with peripheral autonomic failure suffering from orthostatic hypotension is extremely important when prescribing drugs that increase blood pressure. As a rule, drugs with a powerful direct vasoconstrictor effect (norepinephrine) are not prescribed.

Another clear sign of peripheral autonomic failure is resting tachycardia (bpm). Due to the reduced heart rate variability, this phenomenon is called “fixed pulse”. In a patient with peripheral autonomic failure, various loads (standing up, walking, etc.) are not accompanied by an adequate change in heart rate, with a clear tendency to tachycardia at rest. It has been proven that tachycardia and reduced variability in this case are due to parasympathetic insufficiency due to damage to the efferent vagal cardiac branches. Damage to the afferent visceral nerves coming from the heart muscle leads to the fact that myocardial infarction can occur without pain. For example, in patients with diabetes, every third myocardial infarction occurs without pain. Silent myocardial infarction is one of the causes of sudden death in diabetes mellitus.

One of the characteristic manifestations of peripheral autonomic failure is hypo- or anhidrosis. Reduced sweating on the limbs and trunk with peripheral autonomic failure is the result of damage to the efferent sudomotor sympathetic apparatus (lateral horns of the spinal cord, autonomic ganglia of the sympathetic chain, pre- and posttanglionic sympathetic fibers). The prevalence of sweating disorders (diffuse, distal, asymmetric, etc.) is determined by the mechanisms of the underlying disease. As a rule, patients do not pay attention to reduced sweating, so the doctor must clarify and evaluate the state of the sweating function himself. Detection of hypohidrosis along with orthostatic hypotension, resting tachycardia, gastrointestinal disorders, and urinary disorders makes the diagnosis of peripheral autonomic failure more likely.

Peripheral autonomic failure in the gastrointestinal system is caused by damage to both sympathetic and parasympathetic fibers, manifested by impaired motility of the gastrointestinal tract and the secretion of gastrointestinal hormones. Gastrointestinal symptoms are often nonspecific and variable. The symptom complex of gastroparesis includes nausea, vomiting, a feeling of a “full” stomach after eating, anorexia and is caused by damage to the gastric motor branches of the vagus nerve. It should be emphasized that constipation and diarrhea in peripheral autonomic failure are not associated with nutritional factors, and their severity depends on the degree of disturbance of the parasympathetic and sympathetic innervation of the intestine, respectively. These disorders can occur in attacks ranging from several hours to several days. Between attacks, bowel function is normal. Correct diagnosis requires exclusion of all other causes of gastroparesis, constipation and diarrhea.

Impaired bladder function in peripheral autonomic failure is caused by the involvement in the pathological process of the parasympathetic innervation of the detrusor and sympathetic fibers going to the internal sphincter. Most often, these disorders are manifested by a picture of bladder atony: straining when urinating, long breaks between acts of urination, urine leakage from a full bladder, a feeling of incomplete emptying, and the addition of a secondary urinary infection. The differential diagnosis of doleken includes adenoma and hypertrophy of the prostate gland, and other obstructive processes in the genitourinary area.

One of the symptoms of peripheral autonomic failure is impotence, caused in such cases by damage to the parasympathetic nerves of the cavernous and spongy bodies. In primary forms, impotence occurs in up to 90% of cases, in diabetes mellitus - in 50% of patients. The most pressing task is to distinguish between psychogenic impotence and impotence due to peripheral autonomic failure. It is important to pay attention to the features of the onset of impotence (psychogenic forms occur suddenly, organic forms (peripheral autonomic failure) - gradually) and the presence of erections during night sleep. The preservation of the latter confirms the psychogenic nature of the disorder.

Peripheral autonomic failure can manifest itself as disturbances in the respiratory system. These include, for example, short-term stops in breathing and cardiac activity in diabetes mellitus (the so-called “cardiorespiratory arrests”). They usually occur during general anesthesia and in severe bronchopneumonia. Another common clinical phenomenon in patients with peripheral autonomic failure (Shy-Drager syndrome, diabetes mellitus) are episodes of sleep apnea, which can sometimes become dramatic; Involuntary attacks of suffocation (stridor, “cluster” breathing) are less often described. These ventilation disorders become dangerous when cardiovascular reflexes are impaired, and are suggested to be a cause of sudden unexplained death, particularly in diabetes mellitus.

Visual impairment at twilight with peripheral autonomic failure is associated with impaired innervation of the pupil, which leads to its insufficient dilation in low light conditions and, accordingly, disrupts visual perception. Such a disorder should be distinguished from the condition that occurs with vitamin A deficiency. Other symptoms of peripheral autonomic failure or manifestations of hypovitaminosis A may be auxiliary. Usually, pupillary disorders with peripheral autonomic failure do not reach a pronounced degree and are not noticed by patients for a long time.

Thus, it should be emphasized that the clinical manifestations of peripheral autonomic failure are multisystem and often nonspecific. It is some of the clinical nuances described above that suggest the presence of peripheral autonomic failure in the patient. To clarify the diagnosis, it is necessary to exclude all other possible causes of the existing clinical symptoms, for which additional research methods can be used.

Medical Expert Editor

Portnov Alexey Alexandrovich

Education: Kyiv National Medical University named after. A.A. Bogomolets, specialty - “General Medicine”

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Autonomic failure

Isolated autonomic failure appears as a result of the death of neurons in the autonomic ganglia and causes orthostatic hypotension and other autonomic symptoms.

This autonomic failure, which was previously called idiopathic orthostatic hypotension (otherwise known as Bradbury-Eggleston syndrome), is a generalized autonomic failure, without involving the central nervous system. A disease of this type differs from the multiple atrophy system in that the central link is not involved in the pathological process. In most cases, deficiency occurs in women of one year of age and continues to progress further.

Pain is the most common reason for visiting doctors. As long as a person does not experience pain, he does not consider it necessary to use the services of doctors. Pain has emotional and sensory components and can be chronic or acute. In a situation with acute pain, hyperactivity of the nervous system begins, along with it an emotional reaction occurs - anxiety sets in. With chronic pain there is no activity in the nervous system, but there is an association with autonomic symptoms. There is a separate reaction from emotions here – depression. Each person experiences pain differently.

Reasons

Severe pain as a reaction to tissue damage is formed as a result of excitation of pain receptors and specific sensory fibers. In chronic pain, these fibers appear to be in a constant state of excitation. Chronic pain can be neuropathic. It occurs from damage to the central or peripheral nervous system, but not from stimulation of pain receptors.

Pain, called nociceptive, mainly occurs as a result of disease or injury. It is divided, in turn, into somatic and visceral. In case of somatic pain, pain receptors are located in the skin, subcutaneous tissue, fascia and other types of connective tissue. They are also found in the periosteum, articular capsules and endosteum. Activation of these receptors leads to dull or sharp pain sensations. Visceral receptors are located in the internal organs and connective tissue that is located around. Visceral pain appears as a result of damage to the genital organ, in most cases it is aching, deep, or occurs in the form of contractions. If such pain occurs as a result of damage to the connective tissue or capsule of an organ, then it will be acute or more limited. We hope that the causes of pain during autonomic failure are clear to you.

Psychological human factors significantly influence the development of chronic pain. In some patients they may predominate.

The standard level of adrenaline in most cases is less than 100 pg/ml, with the body lying down. And when the body acquires a vertical position, the indicator of this degree does not increase.

The method of treatment for painful sensations of autonomic failure is symptomatic: in the presence of orthostatic hypotension, elastic stockings and vasopressors are used; if constipation is present, use stool softeners and a fiber-based diet; if the activity of the bladder is impaired, then take antispasmodics; Those suffering from excessive sweating need to protect themselves from overheating.

The causes of pain due to autonomic failure are still unknown; in some cases, the appearance of diseases is directly related to the presence of autonomic autoimmune neuropathy.

The main symptom is orthostatic hypotension, although it is quite possible that other symptoms may also appear. The diagnosis is established by exclusion - it is called “psychogenic pain”. The term “psychophysiological pain” is best suited here, since it appears due to a combination of psychological and physiological phenomena. This type of pain is classified under the terms used to define somatoform disorders in the Statistical and Diagnostic Bureau of Mental Disorders, fourth edition.

Most pain syndromes are multifactorial. For example, chronic pain in the lumbar region and most syndromes in the presence of cancer have a pronounced nociceptive component. But they can also be associated with neuropathic pain, due to the fact that the nerve is damaged.

Painful sensations

Pain fibers pass through the dorsal root ganglia, invade the spinal cord, pass in the lateral cords to the thalamus and then enter the cerebral cortex. When the signal moves along a certain path, the pain signal is regulated by inhibitory and excitatory impulses and various neurochemical transmitters. These modulators have not been fully studied to date, but one thing is clear: when they interact, there is a decrease or increase in the response to pain and its awareness.

Secondary stimulation increases the sensitivity of neurons in the spinal cord so that a peripheral stimulus of reduced intensity triggers pain. Increased sensitivity of peripheral nerves and structures at other levels of the central nervous system, causing tangible changes in cortical sensory areas, will result in increased perception of pain.

The substances and components of the inflammatory cascade released when tissues are damaged also increase the sensitivity of the so-called peripheral nociceptors. This includes vasoactive peptides and other mediators.

Psychological nuances are significant pain modulators. In addition to recording the patient's verbal assessment of pain, they also generate signals that modulate the neurotransmission of pain along the conductor. The psychological response to ongoing pain, combined with other underlying factors, results in long-lasting changes in pain awareness.

During the treatment of chronic pain, neuromodulators involved in the modulation of pain sensations are intermediaries in the action of pain relief when taking appropriate medications. These drugs are not analgesics, unlike antidepressants, anticonvulsants, and membrane stabilizers.

Methods for examining a patient with pain syndrome

The doctor is obliged to find out the causes, nature and depth of pain. It is also necessary to establish the level of influence of this sensation on the patient’s daily activities and his psychological state. Assessing the causes of severe pain is found in other sections of the manual; this section specifically analyzes chronic pain.

The anamnesis must contain information characterizing pain, localization and depth of pain, its presence and type, duration, changes in the disease process, frequency and duration of remissions, fluctuations in the severity of pain, and factors that can intensify and reduce pain. You should find out what the impact of pain is on the patient’s life, observing, at the same time, his every day. It is also necessary to examine how the patient copes with work, does hobbies, and builds relationships in the family. All these nuances need to be carefully assessed.

It is necessary to clarify what the expression “pain” means for the patient, while psychological nuances, anxiety, stress, and despondency should be separated. This information is very necessary in order to select the appropriate therapy. It is necessary to distinguish between pain and suffering, especially in patients with cancer. After all, their suffering is determined more by the loss of any functions and the fear of death than by their own pain.

It is necessary to clarify the importance of secondary benefits in the occurrence of pain or the tendency to disability. Understanding pain may be more important than the actual damage caused by the disease itself.

You should ask the patient what medications he used before, how effective they were, and what their side effects were. It is also worth learning about other ways to carry out treatment earlier. It is imperative to make inquiries about the patient’s past lifestyle. For example, was he an alcoholic, a drug addict; whether he is a participant in any legal proceedings, as a result of which monetary compensation is due for damage to his health. A family and individual history of chronic pain can reveal the nature of the true disease, since it is quite possible that family members directly influence the presence of pain in the patient. So, the surrounding circumstances have a certain impact on the patient’s condition.

Intensity of pain. Since external signs and the nature of complaints about the presence of pain depend on the type of personality and the degree of its culture, the strength of pain must be measured and clarified. If you determine the initial parameters of the pain syndrome, then you can easily evaluate the effectiveness of treatment.

The following methods are used to accurately assess pain: a verbal scale of pain categories, a visual analogue scale and a numerical scale. The latter makes it possible to set the severity of pain from 0 to 10 points. The visual analogue scale establishes the severity of pain by marking on an unmarked 10cm line the reading “no pain” on the left, and “unbearable pain” on the right. The strength of the pain is recognized by the segment that remains to the end of the entire line. To determine pain in mentally handicapped and illiterate children, they use drawings of various emotional tones or pictures of fruits of various sizes.

Pain therapy in the presence of autonomic failure

Pain therapy for autonomic failure

Narcotic drugs and non-narcotic analgesics are the main drugs used in the treatment of pain. Anticonvulsants, antidepressants and other drugs with an analgesic side effect are used to treat neuropathic or chronic pain. Additional methods for treating pain include neurostimulation, intraspinal infusion, injection therapy, and peripheral and root nerve blocking. Behavioral therapy and psychotherapy can help patients and change their response to pain.

Non-opioid analgesics

Non-steroidal anti-inflammatory drugs and acetaminophen are used to relieve moderate and mild pain. Non-narcotic analgesics do not contribute to the formation of dependence. Acetaminophen does not have an anti-inflammatory effect and does not irritate the stomach at all. Aspirin has an antithrombotic effect.

According to some experiments, the use of coxibs increases the risk of heart attack, stroke and claudication. This directly depends on the dose and duration of treatment for autonomic failure.

Some clinicians use coxibs first, others prescribe them only to patients who are prone to stimulation of the gastric mucosa. If treatment prevention continues for some time, then physical dependence is quite possible. But the formation of mental dependence is extremely rare. Before starting treatment with opioids, the doctor must carefully examine the patient’s risk of developing a certain addiction. And even if this risk is present, it is still necessary to treat with the help of opioids, but at the same time, clearly control the process.

Methods of administration. All sorts of administration methods are used. If the treatment is long-term, then you need to choose oral administration and transdermal administration. Both options are quite effective and the level of the drug administered into the blood is stabilized. Transdermal and long-acting oral forms improve the order of administration. This fact is especially important for the painkiller to have a beneficial effect at night. Oral fentanyl is absorbed into the oral mucosa and is therefore used for sedation and pain relief in children.

The intravenous method of administration allows for rapid action of the drug and provides convenience in dosage titration. The only drawback of the method is that the duration of the analgesic effect is very limited. The patient may then experience severe pain. Constant intravenous infusion can prevent this result, but then extremely expensive equipment is needed. This method, in most cases, causes gastrointestinal disorders, especially after pre-treatment with other NSAIDs. If a treatment option with any of the NSAIDs, such as coxibs, is used, the patient with heart disease needs to be especially careful. You should also be careful when prescribing all types of NSAIDs to people suffering from renal failure.

By the way, coxibs can also have undesirable effects on the kidneys.

If the initial dose does not give the desired analgesic effect, then you can increase it, this is acceptable. But if you still do not get the expected result, then you must immediately stop taking the drug. When pain is not particularly noticeable, it is worth trying another non-steroidal anti-inflammatory drug. A long course of treatment with NSAIDs will require constant monitoring of hemogram parameters, blood balance, kidney and liver performance. It is also necessary to perform a stool test for occult blood.

Opioid analgesics

"Opioids" is a general name for synthetic and natural components. They are also called drugs. Some of these drugs used for pain relief can act as both agonists and antagonists. The latter are more dangerous - they can be abused, but agonists can also have a negative effect on patients who are physically dependent on opioids. When acute pain is observed, it is better to use short-acting drugs. If the pain is chronic, then you need to take medications that act for a long time.

Opioid analgesics are used to treat chronic or acute pain. Limited use of opioids results in pain and suffering—doctors often lower the dose needed to relieve pain after surgery.

The intramuscular method provides a long-term analgesic effect. But, due to the fact that the medicine is absorbed unevenly and severe pain occurs, this method is often abandoned.

Administration of opioids intrathecally provides long-term pain relief (about 24 hours). The method is used after operations have been completed.

Titration and dosage procedure. The initial dosage of analgesics is prescribed depending on the patient's response. If necessary, the dosage is gradually increased. It must be in balance between the level of sensitivity and the risk of side effects. If there is no balance, then the dosage should be reduced.

Newborns and premature babies are particularly susceptible to opioids.

For transient moderate pain, opioids are administered as needed. If the pain lasts for some time and is severe, then such medications should be administered continuously. And there is no need to wait for the pain to increase. If there is an urgent need, it is worth starting dosages to maintain a more or less stable state. In these kinds of circumstances, mistakes are very often made. For example, short-acting medications are prescribed for a long period of time. As a result, outbreaks of severe pain occur, since the medicine does not dissolve well in the blood.

To provide controlled analgesia, the patient, by pressing a button, administers the dosage needed to reduce pain. Initially, a dose of morphine of 0.5-1 mg/hour is acceptable, but this is not always necessary. The doctor monitors the number of doses administered, as well as the duration of the break between them. Patients previously treated with narcotic analgesics, or with chronic pain, should be started on a continuous infusion. Then, you can switch to painful administration of the drug, but significantly increase the dosage. During the treatment process, administer this dosage at your discretion.

Treatment

As a result of treatment of autonomic failure with opioids, addiction to the analgesic effect may occur. Along with this, further side effects may appear. More patients choose a dosage that provides acceptable pain relief and take it for a long period of time. If suddenly the dosage needs to be urgently increased, this indicates that the disease has intensified. From this we can conclude that addiction to drugs is not so scary. There is no need to be afraid and prescribe opioids quickly and generously.

Opioid analgesics are often combined with non-narcotic drugs. This is a very convenient form, but there is one caveat. The peculiarities of a non-narcotic analgesic do not make it possible to increase, if necessary, the dosage of a narcotic analgesic.

Side effects. The most significant side effects are disturbances in the respiratory processes, the presence of constipation, nausea, and vomiting. Because the plasma concentration of a drug does not reach equilibrium until half-life 4 or 5, treatment with drugs with a long half-life carries the risk of toxicity as plasma solubility increases. If you are dealing with modified release opioids, then a number of days of treatment are required to achieve balance concentrations.

The most common finding of adverse events is in older patients treated with opioids. These drugs cause urinary retention in older men who suffer from benign prostatic hyperplasia.

Opioids should also be used very carefully in patients with renal failure, lung disease, liver disease, dementia, and encephalopathy.

Usually, if you use opioids for a long time, constipation occurs. To carry out appropriate prevention, it is necessary to increase the amount of fluid and fiber in the diet. You should also periodically take laxative medications.

Severe constipation is treated as follows: every 2-3 days, 90 ml of magnesium citrate is taken orally, lactulose (15 ml each) or propylethylene glycol powder is taken 2 times a day.

Depending on the situation, it is possible to prescribe the prevention of excessive sedation with drugs such as modafinil, dextroamphetamine or methylphenidate. It is better to take them in the morning and, if necessary, during the day. The largest portion of methylphenidate, in very rare cases, is more than 60 mg/day. Some patients can simply drink drinks containing caffeine - this will be enough for them. In addition, these stimulating drinks can produce an analgesic effect.

Nausea is treated with the use of hydroxyzine: 4 times a day, 25-50 mg, also metoclopramide - 4 times a day, 10-20 mg.

Respiratory depression is an extremely rare side effect even with long-term use of the drug. If such a disease progresses, then artificial ventilation should be used.

Opioid antagonists. These structural patterns are associated with opioid receptors, but may or may not have narcotic activity. They are used mainly to relieve signs of opioid overdose, and primarily to eliminate respiratory depression.

Naloxone begins to act approximately 1 minute after intravenous administration, and later when administered intramuscularly. But the duration of its antagonistic effect is much shorter than the duration of respiratory depression by opioids. In such cases, naloxone should be administered again and detailed monitoring should be performed. The dose in the presence of an acute overdose of opioids should be 0.4 mg (intravenous), if necessary, administer again after 2-3 minutes. For patients taking opioids for a long time, naloxone should only be used to relieve respiratory depression. But it must be administered extremely carefully so as not to cause rapid withdrawal and pain. The order of application should be as follows: the diluted composition is administered intravenously in an amount of 1 ml, after 1 or 2 minutes in order for breathing to normalize.

A drug called naltrexone is an opioid antagonist taken orally. It is used as an additional drug in the treatment of alcohol and even opioid addiction. It is easily portable and long lasting.

Additional drugs for neuropathy

As additional analgesics, anticonvulsants, antidepressants, glucocorticoids and local anesthetics are very effective. These remedies are quite common, but primarily for treating pain with a neuropathic component. It is often advised to take gabapentin in the highest possible doses. For example, about 1200 mg of the drug is acceptable 3 times a day. Even higher doses are possible.

They also use topical products, for example, capsaicin cream, combination creams, lidocaine patches and many others. They practically do not cause side effects, so they can treat many types of pain.

Neural block

Suspending the transmission of nerve impulses through central or peripheral conductors with the help of drugs or physical methods brings some improvements. These can be short-term improvements, or longer ones. Such a stop is used in rare cases. Local anesthetic drugs are administered intravenously, intrapleurally, intrathecally, transdermally, epidurally or subcutaneously. Epidural analgesia involves the use of local drugs for anesthesia, and after surgery. The administration of this drug has long been used in relation to patients with severe pain and an expected short life expectancy. In order to administer the medicine intravertebrally for a long time, it is recommended to administer it intrathecally, using an implanted infusion pump.

Neuroablation

Neuroablation is the process of stopping the pathway using surgery or radiofrequency destruction. This process is used to relieve pain in cancer patients. The method is more effective in the treatment of somatic pain than in the treatment of visceral pain. In ordinary cases, neuroablation of the spinothalamic tract is performed - this blocks the pain for a certain number of years. But there will be side effects - dysesthesia and anesthesia. Neuroablation of the dorsal roots is performed if a clear topical study of the extent of the lesion is possible.

Neurostimulation

Neurostimulation can reduce chronic pain. This occurs by activating endogenous pain modulation. The most common method is electrical neurostimulation, performed through the skin. This happens as follows: a small electrical voltage acts on the skin. In addition, electrodes can be implanted in peripheral nerves or moved along the dorsal cord in the epidural space. The experience of using thorough stimulation of brain activity and also stimulation of motor areas in the cerebral cortex has not yet brought the expected positive results.

All treatment methods have contraindications; consultation with an appropriate doctor is required.