neuropathy of the axonal type. Tibial neuropathy

30.12.202027.05.2017

Axonal polyneuropathy is a disease associated with damage to the motor, sensory or autonomic nerves. This pathology leads to impaired sensitivity, paralysis, vegetative disorders. The disease is caused by intoxication, endocrine disorders, lack of vitamins, malfunction of the immune system, circulatory disorders.

There are acute, subacute and chronic course of axonal demyelinating polyneuropathy. Pathology in some cases is cured, but sometimes the disease remains forever. There are primary axonal and demyelinating polyneuropathy. In the course of the development of the disease, demyelination is secondary to the axonal component, and the axonal component is secondary to the demyelinating component.

Symptoms of axonal polyneuropathy

The main manifestations of axonal polyneuropathy:

Flaccid or spastic paralysis of the limbs, muscle twitching.
Circulatory disorders: swelling of the arms and legs,.
Change in sensitivity: tingling, goosebumps, burning, weakening or strengthening of tactile, temperature and pain sensations.
Violation of gait, speech.
Vegetative symptoms: tachycardia, bradycardia, excessive sweating (hyperhidrosis) or dryness, blanching or redness of the skin.
Sexual disorders associated with erection or ejaculation.
Violation of the motor function of the intestine, Bladder.
Dry mouth or increased salivation, eye accommodation disorder.

Axonal polyneuropathy is manifested by impaired function of damaged nerves. Peripheral nerves are responsible for sensitivity, muscle movement, autonomic influence (regulation of vascular tone). If the conduction of the nerves is disturbed in this disease, sensory disorders occur:

goosebumps (paresthesia);
increased (hyperesthesia) sensitivity;
decreased sensitivity (hypesthesia);
loss of sensory function like seals or socks (the patient does not feel his hands or feet).

When vegetative fibers are damaged, the regulation of vascular tone goes out of control. After all, nerves can constrict and dilate blood vessels. In the case of axonal demyelinating polyneuropathy, capillary collapse occurs, resulting in tissue edema. The upper or lower limbs increase in size due to the accumulation of water in them.

Since in this case all the blood accumulates in the affected parts of the body, especially with polyneuropathy of the lower extremities, dizziness is possible when standing up. Redness or blanching of the skin of the affected areas is possible due to loss of function of the sympathetic or parasympathetic nerves. Trophic regulation disappears, resulting in erosive and ulcerative lesions.

Characteristic features! Movement disorders are also characteristic of axonal polyneuropathy of the lower extremities and hands. Damage to the motor fibers responsible for the movement of the legs and arms leads to paralysis of their muscles. Immobilization can be manifested as stiffness of the muscles - with spastic paralysis, and their relaxation - flaccid paresis. A moderate degree of damage is also possible, in which case the muscle tone will be weakened. Tendon and periosteal reflexes can be either enhanced or weakened, sometimes they are not observed by a neurologist during examination.

Cranial nerve injury (CN) also occurs. This can be manifested by deafness (with the pathology of the 8th pair - the vestibulocochlear nerve), paralysis of the hyoid muscles and the muscles of the tongue (12th pair of CN suffers), difficulty in swallowing (9th pair of CN). The oculomotor and trigeminal, facial nerves can also suffer, this is manifested by a change in sensitivity and paralysis, facial asymmetry, muscle twitching.

With axonal-demyelinating polyneuropathy of the lower extremities, the lesions of the hands can be asymmetrical. This happens with multiple mononeuropathies, when carpo-radial, knee, Achilles reflexes are asymmetrical.

Causes

The origin of polyneuropathy can be different. Its main reasons are:

Exhaustion, lack of vitamin B1, B12, diseases leading to dystrophy.
Intoxication with lead, mercury, cadmium, carbon monoxide, alcohol, organophosphorus compounds, methyl alcohol, drugs.
Diseases of the circulatory and lymphatic systems (lymphoma, multiple myeloma).
Endocrine diseases: diabetes mellitus.
Endogenous intoxication in renal failure.
autoimmune processes.
Occupational hazards (vibration).
Amyloidosis.
hereditary polyneuropathy.

Deficiency of B vitamins, especially pyridoxine and cyanocobalamin, can negatively affect the conduction of nerve fibers and cause neuropathy. This can occur with chronic alcohol intoxication, intestinal diseases with malabsorption, helminthic invasions, exhaustion.

Violate the conductivity of nerve fibers such neurotoxic substances as mercury, lead, cadmium, carbon monoxide, organic phosphorus compounds, arsenic. Methyl alcohol in small doses can cause neuropathy. Drug-induced polyneuropathy caused by neurotoxic drugs (aminoglycosides, gold salts, bismuth) also occupies a significant share in the structure of axonal neuropathies.

In diabetes mellitus, nerve dysfunction occurs due to the neurotoxicity of fatty acid metabolites - ketone bodies. This is due to the impossibility of using glucose as the main source of energy; fats are oxidized instead. Uremia in kidney failure also impairs nerve function.

Autoimmune processes, in which the immune system attacks its own nerve fibers, may also be involved in the pathogenesis of axonal polyneuropathy. This may occur due to the provocation of immunity with the careless use of immunostimulating methods and medications. Triggering factors in people prone to autoimmune diseases can be immunostimulants, vaccination, autohemotherapy.

Amyloidosis is a disease in which the protein amyloid accumulates in the body, which disrupts the function of nerve fibers. It can occur with multiple myeloma, lymphoma, bronchial cancer, chronic inflammation in the body. The disease may be hereditary.

Diagnostics

The therapist must examine and question the patient. A neuropathologist who deals with disorders of nerve functions checks tendon and periosteal reflexes, their symmetry. It is necessary to conduct a differential diagnosis with multiple sclerosis, traumatic nerve damage.

Laboratory tests for the diagnosis of uremic neuropathy - the level of creatinine, urea, uric acid. If diabetes is suspected, blood is taken from a finger for sugar, as well as for glycated hemoglobin from a vein. If intoxication is suspected, then an analysis for toxic compounds is prescribed, the patient and his relatives are interviewed in detail.

Treatment of axonal polyneuropathy

If axonal polyneuropathy is diagnosed, treatment should be comprehensive, addressing the cause and symptoms. Assign therapy with B vitamins, especially in chronic alcoholism and dystrophy. With flaccid paralysis, cholinesterase inhibitors (Neostigmine, Kalimin, Neuromidin) are used. Spastic paralysis is treated with muscle relaxants and anticonvulsants.

If polyneuropathy is caused by intoxication, specific antidotes, gastric lavage, forced diuresis during infusion therapy, and peritoneal dialysis are used. In case of heavy metal poisoning, tetacin-calcium, sodium thiosulfate, D-penicillamine are used. If intoxication with organophosphorus compounds occurs, then atropine-like agents are used.

Glucocorticoid hormones are used to treat autoimmune neuropathies. Diabetic neuropathy requires treatment with hypoglycemic drugs (Metformin, Glibenclamide), antihypoxants (

Medical and social expertise and disability in polyneuropathies

Definition
Polyneuropathy (polyradiculoneuropathy) is a large heterogeneous group of diseases caused by the influence of exogenous and endogenous factors, characterized by multiple, mainly distal, symmetrical lesions of peripheral nerves, manifested by sensory, motor, trophic and vegetative-vascular disorders.

Epidemiology
Summarized data on the epidemiology of polyneuropathy due to a number of reasons (imperfection of registration forms, syndromic nature of the lesion in many somatic diseases, etc.) are far from complete. The primary incidence of polyneuropathies is about 40 per 100,000 population per year. Among diseases of the peripheral nervous system, polyneuropathies occupy the second place after vertebrogenic lesions, and undoubtedly are a common cause of temporary disability and disability. For example, 32% of patients who have undergone AIDP become disabled, of which about 5% are bedridden or chair-bound. Disability due to polyneuropathy is about 15% of patients with diabetes mellitus. Chronically occurring neuropathies of toxic, autoimmune, diabetic etiology limit the vital activity and lead to social insufficiency of patients most significantly and for a long time.

Classification
(WHO, 1982; modified)
I. Depending on the morphological features of the lesion:
1) axonopathy: axonal degeneration of the predominantly distal part of the axon with simultaneous destruction of the myelin sheath, muscle atrophy. Functional recovery is usually slow and incomplete or does not occur. With ENMG, the speed of impulse conduction along motor fibers decreases slightly, but the number of functioning motor units decreases;
2) myelinopathy: segmental demyelination with primary damage to myelin and Schwann cells with preservation of axons and blockade of conduction along nerve fibers.
Full or partial remyelination is possible with restoration of functions, moderate or slight residual defect. The speed of conduction along the motor fibers according to ENMG data is reduced to 20-60% of the norm or less. The number of functioning motor units is reduced.
Pathological differences between axonopathies and myelinopathies are not always clear, possibly combined damage to axons and myelin sheaths, which determines the doubtfulness of the clinical prognosis.

II. According to the prevailing clinical signs:
1) motor polyneuropathy;
2) sensitive polyneuropathy;
3) autonomic polyneuropathy;
4) mixed polyneuropathy (sensomotor and vegetative);
5) combined: simultaneous or sequential damage to the peripheral nerves, roots (polyradiculoneuropathy, multiple mono-, polyneuropathy) or the central nervous system (encephalomyelopolyradiculoneuropathy, etc.).

III. By the nature of the flow:
1) acute (sudden onset, rapid development);
2) subacute;
3) chronic (gradual onset and development);
4) recurrent (acute or chronic with periods of partial or complete recovery of functions).

IV. Classification according to the etiological (pathogenetic) principle:
1) infectious and autoimmune;
2) hereditary;
3) somatogenic;
4) with diffuse diseases of the connective tissue;
5) toxic (including drugs);
6) due to the influence of physical factors (with vibration disease, cold, etc.).

Risk factors for occurrence, progression
1. General: a) unbalanced diet (avitaminosis B); b) elderly age; c) diabetes mellitus; d) oncological disease; e) hypothermia; f) insufficient or inadequate therapy of somatic and endocrine diseases.

2. Caused by the etiology of polyneuropathies: a) professional and domestic intoxications; b) the impact of physical factors in the labor process; c) overdose and uncontrolled intake of certain medicines; G) infectious diseases: diphtheria, influenza, brucellosis, HIV infection; leprosy, etc.; e) vaccination; f) hereditary neuropathies in history.

Clinic and diagnostic criteria
I. General clinical criteria:
1. Anamnesis: risk factors for polyneuropathies, including occupational ones; typical onset and development of the disease (paresthesia, pain, less often - muscle weakness in the distal lower extremities).

2. Symmetry of sensory, motor, vegetative disorders, their combination (with different severity depending on the etiology of the disease) and ascending distribution. Rare isolated motor, sensory, or autonomic polyneuropathy.
3. Variety of sensory disorders, mostly subjective. Sympathetic (hyperpathic) nature of pain (burning, tingling), usually intense, difficult to tolerate by patients. Distal hypalgesia, as well as a violation of deep (vibration, muscle-articular) sensitivity.
4. Common autonomic disorders, often manifested as symptoms of progressive autonomic failure, and distinct trophic disorders.

II. Features of the clinical picture due to the etiology of polyneuropathy (the forms that are most significant in neurological, including expert, practice are presented):
1. Infectious and autoimmune. An extensive group of polyneuropathy, mainly secondary (parainfectious, post-vaccination). They can be caused by direct action of an infectious agent on peripheral nerves (with rabies, brucellosis, leptospirosis, leprosy, herpes and HIV infection) and indirect (toxic, due to an autoimmune process): primary inflammatory, with diphtheria, botulism, typhoid fever, etc.
1.1. Acute inflammatory demyelinating polyneuropathy of Guillain-Barré (AIDP).
There is reason to distinguish acute primary polyradiculopathy as an independent disease of an autoimmune nature with a triggering factor most often in the form of a viral infection and Guillain-Barré syndrome in various well-defined diseases (diphtheria, primary amyloidosis, intermittent porphyria, lupus erythematosus, multiple myeloma, etc.). ). True Guillain-Barré polyradiculopathy is a common disease (1.2-1.7 per 100,000 population). It is more common at the age of 20-50 years, in men and manual workers. Previous events - acute respiratory diseases, tonsillitis, hypothermia, overwork. Often subfibrillation, sometimes fever up to 38-39 °. Often acceleration of ESR, moderate leukocytosis. The development is acute, subacute, usually begins with sensory disorders (paresthesia, pain in the legs), less often with motor disorders. The increase in symptoms on average within 20 days. Movement disorders (flaccid, sometimes mixed paresis and paralysis) are initially manifested by lower paraparesis of various distributions (often distal, diffuse, less often proximal). In dynamics, tetraparesis develops. Reflexes symmetrically decrease or drop out. With mixed paresis, pathological foot signs are possible. In 30% of patients, one can speak of a predominantly motor variant of the disease. In almost 30% of patients, the motor defect clearly predominates. Sensitivity disorders of the radicular, radicular-polyneuritic or polyneuritic type (in the form of "socks" and "gloves"). Radicular and distal pains with a hyperpathic component, Lasegue's symptom in half of the patients are observed at the onset of the disease. In some cases, deep sensitivity suffers, manifested by sensitive ataxia. Cranial nerves are affected in 25-50% of patients (often facial). The nerves of the bulbar group are usually involved in the process (along with the diaphragmatic and intercostal ones) in the ascending course of the disease, similar to Landry's palsy. Respiratory disturbances are observed, which requires respiratory assistance. Restoration of spontaneous breathing often occurs after 2-3 weeks, although a fatal outcome is also possible. Vegetative and trophic disorders in the extremities are typical (cyanosis and pastosity of the feet, hands, hyperhidrosis or dry skin, bedsores). In severe cases of AIDP, a typical syndrome of progressive autonomic failure often develops: orthostatic hypotension, tachycardia, paroxysmal arrhythmia with ECG changes, pelvic disorders, and other characteristic symptoms. Acute cardiovascular insufficiency with autonomic dysfunction can cause the death of the patient. From the second week of the disease, protein-cell dissociation in the cerebrospinal fluid is constantly detected (the amount of protein is from 0.45 to 5.0 g/l).
Criteria for diagnosing AIDP: 1) symmetrical weakness in all limbs; 2) paresthesia in the hands and feet; 3) decrease or absence of reflexes starting from the first week of the disease; 4) progression of the listed symptoms from several days to 1 month; 5) an increase in the protein content in the cerebrospinal fluid (more than 0.45 g/l) during the first three weeks from the onset of the disease; 6) a decrease in the rate of propagation of excitation along the motor and (or) sensory fibers of the nerve and the absence, especially at an early stage of the disease, of damage to the axial cylinder (according to ENMG).
In connection with the peculiarities of the clinical picture, it is advisable to distinguish between polyradiculoneuropathy and myelopolyradiculoneuropathy (Margulis variant), which occurs in 5% of patients. Perhaps a widespread CNS lesion (encephalomyelopolyradiculopathy). A predominantly axonal variant of ARDP is also described - motor or motor-sensory axonal neuropathy with the most acute development of tetraplegia, bulbar and respiratory disorders.

Depending on the severity of symptoms in the acute period, 3 degrees of severity of the disease are distinguished: a) mild (in 27% of patients): moderate severity of paresis, sensory disturbances, pain syndrome; b) moderate (45%): para- and tetraparesis, severe pain and other sensory disturbances; c) severe (19%): paralysis and severe paresis of the limbs, significant sensory, trophic and autonomic disorders, often ascending course of the Landry type with rapidly developing damage to the respiratory muscles and bulbar nerves, which requires respiratory assistance, sometimes for 2-4 weeks.

The course and prognosis are usually favorable. Lethal outcome in 5% of cases. About 70% of patients recover completely (more often with an acute onset of the disease), the rest have consequences in the form of motor, less often sensory disorders. Recovery of functions within 2-3 months or more (up to two years). In 25% of patients, relapses are observed, sometimes repeated, with a more pronounced neurological deficit. In 10% of cases, a chronic course occurs with an increase in motor disorders and spontaneous improvement.

Differential diagnosis is carried out with poly-, radiculoneuropathy clinically manifested by Guillain-Barré syndrome, in particular diphtheria, porphyria neuropathy, polyradiculoneuropathy with herpes zoster, tick-borne borreliosis, sarcoidosis, connective tissue diseases, systemic necrotizing angiitis (Degos syndrome) and other vasculitis.

1.2. Fisher's syndrome. The clinical form is close to AIDP, and possibly an independent disease. Clinical and diagnostic criteria: a) subacute onset and monophasic course; b) ophthalmoplegia, cerebellar ataxia, decrease or loss of tendon reflexes with preserved or slightly reduced muscle strength; c) protein-cell dissociation in the cerebrospinal fluid. Perhaps concomitant damage to the facial and less often other cranial nerves. ENMG, in contrast to the classical OVDP. reveals changes characteristic of axonal neuropathy. The undoubted involvement of the central nervous system does not contradict the features of the autoimmune process. Differentiate Fisher's syndrome with a tumor of the brain stem, the so-called stem encephalitis, myasthenia gravis. The prognosis is favorable, usually with full recovery of functions.

1.3 Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). It has common pathogenetic and clinical signs with AIDP. However, significant clinical features allow us to consider CIDP as a special nosological form. Diagnostic criteria, according to the standards of the international research group on neuromuscular diseases:
1) bilateral, as a rule, symmetrical weakness in the limbs;
2) paresthesia in the feet and hands;
3) progression of the process for more than 6 weeks, accompanied by periods of increase and decrease in weakness in the limbs for at least 3 months or slow progression from 6 weeks to several months;
4) decreased reflexes in paretic limbs, absence of Achilles reflexes;
5) an increase in the protein content in the cerebrospinal fluid over 1 g/l during the period of clinical deterioration.

Differences from OVDP Guillain-Barre:
1) slow (rarely subacute) onset, gradually, without previous infection, followed by progression (often with relapses) over months, sometimes many years;
2) more common after the age of 40 years;
3) in a quarter of patients, a tremor in the hands is observed, resembling an essential one, disappearing during remission and reappearing during relapse;
4) the originality of the results of the ENMG study, in particular, the presence of local areas of blockade of the conduction of excitation in various nerves and a heterogeneous block at different levels of one nerve; 5) worse prognosis and the need for special treatment tactics. With the help of CT and MRI, foci of demyelination in the brain are detected in some patients, which indicates a possible combination of demyelination in the central and peripheral nervous system.
The course is long, the prognosis is doubtful. About 30% of patients recover, the rest have sensorimotor disorders of varying severity (about half of them are II or I group invalids). A fatal outcome is also possible.
Differential diagnosis with AIDP, at an early stage of the disease - with polymyositis, myasthenia gravis.

1.4. Diphtheria polyneuropathy.
It belongs to the group of infectious and autoimmune polyneuropathies, although exposure to neurotoxin plays a major role in the pathogenesis of early neurological complications of diphtheria. They manifest themselves in the first days of the disease as bulbar and oculomotor disorders. Severe forms of bulbar paralysis, often combined with distal tetraparesis, currently occur in 55% of cases and can lead to the death of patients.
Late polyneuropathies complicate diphtheria in 8-40% of cases. IN last years more often develop in adult patients not only with toxic, but also with localized forms of the disease. They are caused by an autoimmune process, they are detected on the 3rd-10th week of the disease (“fiftieth day syndrome” of Glatzman-Zeland), usually after the patient is discharged from the infectious diseases hospital. They are manifested mainly by paresis and paralysis, more pronounced in the legs. Pain syndrome, as well as distal hypoesthesia, mild or moderate. The muscular-articular feeling is noticeably disturbed, which leads to sensitive ataxia when walking. In 3% of patients, PVN syndrome is observed against the background of peripheral autonomic disorders. ENMG confirms the myelinopathic nature of polyneuropathy.
The prognosis is favorable in most cases, but there are severe forms with widespread damage to the muscles of the trunk, neck and diaphragm, respiratory failure, when respiratory assistance is required and death is possible. Restoration of functions is delayed up to 3-6 months, sometimes for 1-2 years. A year later, in 85% of those who have been ill, the motor function of the limbs is restored completely, in the rest - residual manifestations (distal paresis, hypoesthesia, vegetative-vascular disorders).
The diagnosis is based on the data of the anamnesis, the characteristic clinical picture of diphtheria. When judging late polyneuropathy, data on the time of occurrence of paresis after the acute period of the disease are important.

1.5. Herpetic neuropathies. They are the most common manifestations of herpes infection, primarily herpes zoster. Immunodeficiency contributes to the disease, in particular as a result of HIV infection. An obligatory component is a viral ganglionitis with a lesion of 3-4 or more ganglia. Typical are multiple monoganglioneuritis of the thoracic, cranial, less often lumbosacral and cervical localization. Spinal polyganglioneuritis is observed in 53% of cases. In the clinical picture, a typical sympathetic pain syndrome (usually against the background of herpetic eruptions), sensory and autonomic disorders, later mild or moderate paresis of the limbs, abdominal wall muscles. The zone of movement disorders may be wider than the localization of the rash, the proximal extremities are predominantly affected. The diagnosis is difficult when the virus reactivates with damage to the peripheral nervous system, not accompanied by skin rashes. The outcome is generally favorable, but the recovery of motor functions can be delayed up to 3 months.
The course of the disease is complicated by postherpetic neuralgia (in 30% of cases, usually in elderly patients), which occurs with exacerbations, sometimes for many months. It is diagnosed if the pain syndrome is observed for more than 4-6 weeks after the disappearance of the rash.
Regardless of the primary localization of ganglioneuritis, in the first 2 weeks from the onset of the disease, polyradiculoneuropathy (Guillain-Barré syndrome) may develop with segmental demyelination, a typical clinical picture, but more severe (death was recorded in 30% of patients).

2. Hereditary motor-sensory and autonomic neuropathies.
2.1 Neural amyotrophy of Charcot-Marie-Tooth is the most well-known, most common form. The disease is inherited in an autosomal dominant, rarely recessive manner. It is customary to distinguish two variants: 1) hypertrophic with thickening of the nerves, segmental demyelination, reduced speed of nerve conduction, and 2) with axonal degeneration without a significant change in the speed of conduction along the motor nerve.
clinical picture. In the first (classic) variant, the disease begins in the first (more often) or second decade of life, debuts with difficulty walking or running, and deformity of the feet is detected early. Amyotrophies are distal, symmetrical; they rarely and late spread to the proximal lower extremities. Moderate hypotrophy of the muscles of the upper limbs (hands) is usually detected several years after the onset of the disease. Tendon and periosteal reflexes fall out early, primarily Achilles. Often there are limited muscle fasciculations. In 70% of patients, loss of sensitivity is observed, more often vibration, then pain and temperature. Loss of musculoskeletal feeling, manifested by sensitive ataxia, along with areflexia and distal muscular hypotrophy, is considered within the Roussy-Levi syndrome. Peripheral nerves, especially the peroneal, often thicken, which is determined by palpation or visually.
The severity of neurological symptoms, primarily motor disorders, varies significantly. Often there are patients with rudimentary manifestations of hereditary neuropathy ("bird's legs", "horse foot"), who have never consulted a doctor.
In the case of the second variant (axonal type of lesion), the disease usually develops at a later date, often at 40-60 years of age. Clinical manifestations are similar to the first variant, however, only half of the patients suffer from upper limbs and there are signs of impaired sensitivity. How component neurological picture, a syndrome of congenital insensitivity to pain is possible.
The course is slowly progressive, sometimes stabilization of the process occurs. The ability to move independently is rarely lost, usually after the age of 50, although the ability to work may decrease at a young age. In women, the disease is usually less severe than in men.
Diagnostics. Family history, typical time of onset of the disease, clinical pattern, in particular, slowly progressive type of course, absence of pain syndrome, ENMG data are taken into account. In the differential diagnosis with acquired polyneuropathies, one should also keep in mind the deformity of the feet, often occurring scoliosis, and hypertrophy of the nerve trunks. The diagnosis is helped by a clinical examination, including ENMG of the patient's relatives, since the disease can occur in them asymptomatically or in a rudimentary form.

2.2. Porphyrian polyneuropathy is observed more often in acute intermittent porphyria. A genetically determined disease belonging to a large group of porphyrias and associated with the accumulation of porphyrins, inherited in an autosomal dominant manner, manifests itself mainly in women.
The pathogenesis of polyneuropathy seems to be heterogeneous: primary axonal degeneration of metabolic origin and segmental demyelination, possibly of ischemic origin. Polyneuropathy syndrome develops against the background of an acute attack of the disease, in 70% of cases provoked by the intake of barbiturates, sulfonamides, antipsychotics, hormonal drugs, alcohol and manifested by severe pain in the abdomen, lower back, nausea, vomiting, stool retention, tachycardia. There is general weakness, sometimes psychomotor agitation, convulsive seizures. Urine wine-red color (stained only after a few hours). Neuropathy is predominantly motor, often begins with paresis in the hands. The onset of weakness may be preceded by pain in the extremities, distal sensory disturbances. The paradoxical preservation of Achilles reflexes is characteristic. Typical general autonomic failure (orthostatic hypotension, fixed tachycardia and other symptoms of PVN). The attack lasts 4-6 weeks or more, but the recovery of motor functions can be delayed for many months, sometimes for 1-2 years. In untreated patients, bulbar disorders and respiratory disorders may occur due to damage to the cranial and intercostal nerves, sometimes with a fatal outcome. However, the prevention and early treatment of attacks contributes to the preservation of a sufficiently high quality of life of the patient.
Diagnosis is determined by the characteristic combination of abdominal pain, convulsions, psychomotor agitation with polyneuropathy. The red color of the urine or the appearance of Pink colour in a test with Erlich's reagent (detection of porphobilinogen). It is necessary to take into account the data of the anamnesis about the recurrence of seizures provoked by certain medicines, infection, stress, remission from several months to several years, hospitalizations for an acute abdomen.
Differential diagnosis with polyneuropathies of other etiologies; in particular lead, OVDP Guillain-Barre.

3. Somatogenic polyneuropathies. They refer to neuropathies that develop in the pathology of internal organs, the endocrine system, blood diseases, malignant neoplasms and other diseases. Their diagnosis is often difficult, and clinical manifestations are not always taken into account in the complex of functional disorders in internal diseases, which determine the state of life and work capacity of patients. Neuropathy in systemic diseases is ambiguous in terms of etiology, morphological features, onset and course, predominant clinical signs, which is reflected in the table, which lists the main ones.

3.1 Diabetic neuropathy. It is diagnosed in 8% of patients during the initial diagnosis of diabetes and in 40-80% after 20 years from the onset of the disease (Prikhozhan V. M., 1981). The rate of development of neuropathy is different, sometimes it is asymptomatic for a number of years, it is detected earlier in insulin-dependent, poorly controlled diabetes. Clinical forms:
1) distal symmetric polyneuropathy; 2) symmetrical proximal motor neuropathy; 3) local and multiple mononeuropathy. These syndromes can occur alone or in combination.

Distal symmetric polyneuropathy belongs to the axonal type and accounts for about 70% of all diabetic neuropathies. A sensory-motor-vegetative type of lesion is characteristic, but sensory-vegetative and motor variants are possible. The latter is much less common, especially pronounced paresis. In the initial stage of the disease, nocturnal paresthesias, burning pains in the distal extremities, especially in the legs, are observed. Early violations of vibrational sensitivity are detected, then superficial in the form of "socks" and "gloves". Tendon and periosteal reflexes decrease, and then fall out (earlier than Achilles). Vegetative and trophic disorders occur in a third of patients (thinning of the skin, anhidrosis, hypotrichosis, swelling of the feet). Peripheral autonomic dysfunction (vegetative neuropathy), which usually develops in young patients with insulin-dependent diabetes, in severe cases fits into the clinical picture of the syndrome of progressive autonomic failure: tachycardia at rest, orthostatic hypotension, incomplete emptying of the bladder, diarrhea, impaired pupillary innervation, impotence, etc. In the late stage of the disease, there are flaccid paresis of the feet, pronounced distal trophic disorders: ulcers, arthropathy, gangrene (diabetic foot).
The course is in most cases slowly progressive over many years. The rapid progression of polyneuropathy is facilitated by repeated hypo- or hyperglycemic coma. However, a stationary defect and partial restoration of functions during treatment are possible. Often the pain syndrome is easier to stop.
Diagnosis is difficult if symptoms of polyneuropathy appear in patients with previously undiagnosed diabetes. It is based on a typical clinical picture, the relationship of the development of polyneuropathy with prescription and the course of diabetes. A combination with encephalo- and myelopathy of diabetic etiology is possible (Prikhozhan V. M., 1981). EMG reveals a decrease in the amplitude of biopotentials during voluntary muscle contraction, even in the absence of obvious paresis. There is also a slight decrease in the speed of conduction of excitation along the nerve in the distal parts of the lower extremities.
Differential diagnosis is carried out with polyneuropathies of a different etiology, mainly toxic (alcoholic). This takes into account the possible combination of etiological factors.
Symmetrical proximal motor neuropathy (Garland's amyotrophy) is rare, sometimes in association with typical polyneuropathy. It is manifested by weakness of the muscles of the pelvic girdle, mainly the hips, aching pains. Paresis develops acutely or subacutely over several weeks. According to ENMG and EMG - neurogenic nature of the process and damage to the cells of the anterior horns of the spinal cord. The prognosis is relatively favorable: restoration of motor functions in a few weeks or months, subject to insulin therapy and compensation for diabetes.
Local and multiple neuropathy. Acute, ischemic nature, multiple lesions of the femoral, obturator, sciatic, rarely ulnar and median nerves, sometimes occurs in elderly patients. Progression within a few hours or days, there are severe pain, muscle atrophy.
Relatively common are cranial neuropathies: of the oculomotor nerve (unilateral painful ophthalmoplegia, with preserved pupillary reactions, sometimes recurrent); mononeuropathies of intercostal and other nerves, in particular tunnel ones.
Differential diagnosis in the case of painful ophthalmoplegia should be carried out with Tolosa-Hunt syndrome, an aneurysm of the internal carotid artery. The prognosis is favorable, paralysis and pain syndrome usually regress within 6-12 months.
Distal symmetric motor neuropathy occurs in patients with repeated hypoglycemic conditions due to insulinoma. Possible coma with loss of consciousness, convulsions. Typically decreased intelligence. Differential diagnosis with cerebral tumor, epilepsy. Treatment is operative.

3.2. Polyneuropathies in paraneoplastic syndrome develop against the background of a malignant tumor of various localization (small cell lung cancer, cancer of the breast, stomach, colon, lymphoma, chronic lymphocytic leukemia, myeloma). More common in older patients. Along with other etiological factors (decrease in the level of metabolism and regeneration against the background of beriberi, somatic diseases), they are included in the group of so-called polyneuropathies of senile age. May be the first clinical manifestations of a malignant tumor, sometimes ahead of the appearance of other symptoms of the tumor by 5 years or more. The causes of early paraneoplastic neuropathy remain unclear. Often combined with other paracarcinomatous syndromes (Lambert-Eaton, myopathy, subacute cerebellar degeneration, etc.). The main type of lesion is axonal degeneration, although myelinopathy is also possible with a recurrent course. Sensory and sensorimotor polyneuropathies predominate. Pain syndrome (burning pains, paresthesias) is moderately expressed, although it may debut in the clinical picture of the disease. Movement disorders (steppage), muscle hypotrophy is more pronounced in the lower extremities. Objectively detectable sensory disorders relate to all types of sensitivity.
In chronic lymphocytic leukemia, multiple myeloma, macroglobulinemia, acute or subacute polyradiculoneuropathy of the Guillain-Barré syndrome type with characteristic protein-cell dissociation can develop. It should be noted that in patients with lymphogranulomatosis and multiple myeloma, toxic polyneuropathy also occurs (during the treatment with vincristine). Clinically, this is a typical sensory-motor symptom complex with an axonal type of lesion. Neurological deficits may worsen with repeated courses of chemotherapy. The course of paraneoplastic polyneuropathies is often progressive, although remissions are possible, in particular, after surgical removal of the tumor and corticosteroid therapy.
Differential diagnosis - with alimentary (avitaminous) and toxic-alimentary neuropathies. It is difficult in elderly patients, with the early appearance of polyneuropathy, cancer of unclear localization. It is necessary to take into account the possibility of toxic damage to the nerves during chemotherapy.

4. Neuropathy in diffuse connective tissue diseases. May occur in the form of multiple mononeuropathy, tunnel neuropathy. They are caused by damage to the peripheral nerves due to concomitant (secondary) vasculitis, but neuropathies are possible with the so-called systemic vasculitis (nodular periarteritis, Wegener's granulomatosis). The syndrome of polyradiculopathy was also described in systemic necrotizing angiitis of Degos (Makarov A. Yu. et al., 1993). Neuropathy in collagenosis and primary vasculitis, in addition to the pathology of internal organs, is often manifested by damage to the brain and spinal cord, which occurs with appropriate neurological symptoms. They are more often referred to as axonopathies, however, demyelinating variants of the lesion, Wallerian degeneration are possible.

4.1. Neuropathy in systemic lupus erythematosus develops in 10% of patients, usually against the background of the activity of the process, but may also be the first clinical symptom. In the case of polyneuropathy, paresthesias appear in the distal extremities, pain and temperature sensitivity is disturbed. Increased fatigue of the legs when walking is possible. With mononeuropathy of the lower extremities, weakness of the foot appears, deep sensitivity is lost. There are bulbar symptoms due to damage to the cranial nerves of the caudal group. In the cerebrospinal fluid, protein-cell dissociation is possible (with a clinical picture similar to the atypical Guillain-Barré syndrome). The course is rapidly progressive, the motor defect is pronounced and persistent.

4.2 Neuropathy in rheumatoid arthritis occurs in approximately 10% of patients with a long and severe course of the disease. Distal symmetric polyneuropathy usually presents with paresthesia and decreased sensation in the upper and lower extremities. There are no movement disorders. The course is slowly progressive, sometimes stabilization of the process or a distinct progression. In the latter case, severe distal sensory-motor neuropathy may develop against the background of generalized vasculitis with a poor prognosis. There are mononeuropathies, also with a tendency to progression. The appearance of paresis is preceded by pain syndrome. Rheumatoid arthritis in such patients occurs with destructive changes in the joints, pronounced trophic disorders of the skin. Tunnel neuropathies (carpal, tarsal canal, etc.) are also widely represented.

4.3. Neuropathy with periarteritis nodosa occurs in 27% of patients. Occur against the background of a typical clinical picture (temperature, increased ESR, damage to the kidneys, gastrointestinal tract, high blood pressure, etc.). In fact, they are multiple mononeuropathies with a predominant lesion of the sciatic, tibial, median, ulnar nerves, sometimes asymmetrical. First, shooting burning pains appear, mainly in the muscles, then reflexes fall out, sensitivity is disturbed, paresis and paralysis develop. Possible damage to the spinal and cranial nerves. The course is chronic for several years, improvement often occurs on the background of hormone therapy.

5. Toxic polyneuropathy of various etiologies. They represent a large group of diseases caused by single or chronic exposure to substances of three groups - heavy metals, toxic organic compounds and drugs. The rate of development of polyneuropathies, concomitant damage to various parts of the central nervous system, internal organs, the severity of motor, sensory, vegetative manifestations, the prognosis depend on the characteristics of the toxic agent. Currently, only some types of toxic polyneuropathies are relevant. Frequent in the past lead mononeuropathies, as well as mercury polyneuropathies, are rare.

5.1. Alcoholic polyneuropathy accounts for about 30% of all polyneuropathies. It develops in 20-70% of patients with chronic alcoholism, usually with a significantly pronounced pathology of the digestive system. The pathogenesis is alimentary-toxic. Vitamin B12 deficiency plays a major role. It refers to axonopathy, however, segmental demyelination and a mixed variant of the lesion may occur.
clinical picture. Initial manifestations in the form of paresthesias in the distal extremities, pain in the calves are usually not fixed by patients. Gradually, sometimes within a few days, paresis of the muscles of the distal legs and arms is revealed, Achilles reflexes disappear. The most distinct weakness of the extensors of the feet (steppage when walking). Pain, hyperalgesia with hyperpathic phenomena are typical, especially in the feet. In the future, within a few weeks, months, muscle hypotrophy, sensitive ataxia appear, paresis, vegetative-trophic disorders in the extremities are aggravated. The latter are not uncommon at the subclinical stage of the disease. Perhaps the acute development of distal paresis and hypesthesia against the background of poisoning with alcoholic surrogates.
The flow is different. Progression is possible, when alcohol is stopped, the process stops, but paresis and ataxia remain. A relapsing course has been described. In diagnostic terms, the combination with other alcoholic CNS lesions (Korsakov's psychosis, encephalomyelopathy, cerebellar degeneration) is unfavorable.
Differential diagnosis is carried out with alcoholic myopathy, other toxic and endogenous neuropathies, and with severe sensitive ataxia - with dorsal tabes.

5.2 Arsenic polyneuropathy develops in acute and chronic poisoning, but the clinical picture is most pronounced in case of acute intoxication. A distal pain syndrome is characteristic, after I-2 weeks sensitive prolapses appear, including deep sensitivity, which leads to ataxia. Movement disorders begin with the feet, in severe cases tetraparesis develops. Atrophy of the muscles of the distal extremities is pronounced. In chronic poisoning, abortive manifestations of polyneuropathy can be observed. Recovery of functions is slow (from 1 year to several years). In severe cases, paresis, amyotrophy, contractures may remain.

5.3. Polyneuropathy from exposure to organophosphorus compounds. Poisoning by insecticides (thiophos, karbofos, chlorophos, etc.) is almost exclusively found. The toxic action of FOS is based on the inactivation of cholinesterase. 1-3 weeks after acute poisoning, mild distal paresthesias occur, muscle weakness that progresses over several days, Achilles reflexes fall out. Typically, the involvement of the central motor neuron in the process, and therefore the nature of the paresis is mixed. Treatment is ineffective. Spastic paraparesis often remains as a consequence.

5.4. Medicinal polyneuropathies can develop in the treatment of massive doses of isoniazid (tubazid), sulfonamides, antitumor and cytostatic agents (azothioprine), vinca alkaloids (vinblastine, vincristine), amiodarone (cordarone), disulfiram (teturam), phenobarbital, diphenin, tricyclic antidepressants and etc. They are mostly sensory, or sensorimotor. The latter, in particular, are not uncommon in long-term treatment of tuberculosis patients with isoniazid at a dose of 5-20 mg / kg per day (due to vitamin B12 deficiency) and the constant intake of cordarone (400 mg per day for a year). Characterized by distal paresthesia, sensory disturbances with moderate paresis, autonomic dysfunction. Drug-induced polyneuropathy usually regresses after discontinuation of the drug.
Diagnosis of toxic polyneuropathies is based on the identification of the fact of intoxication, determination of the nature of the toxic agent (using methods of biochemical analysis). The clinical picture of damage to other organs and systems of the body is taken into account. Often, consultation with an occupational pathologist or hospitalization in an appropriate hospital is necessary. In order to objectify motor, vegetative disorders, judgments about the prognosis of the restoration of functions, EMG and ENMG, RVG, thermal imaging are used.

6. Polyneuropathies caused by the influence of physical factors. They belong (along with some toxic ones) to professional neuropathies. They include predominantly vegetative forms that are included in the clinical picture of vibrational disease due to local or general vibration. With a combined effect, a polyneuropathic syndrome is possible with damage not only to the upper, but also to the lower extremities. They are found in occupational “overstrain diseases” (in workers in the textile and shoe industries, poultry farms, seamstresses, typists, etc.). A common form (mainly in workers of meat processing plants, fishermen) is cold polyneuropathy, which is clinically manifested by vegetative-vascular, sensory and trophic disorders mainly in the distal parts of the upper limbs (Palchik A. B., 1988). Such polyneuropathies can be attributed to the group of angiotrophopathies due to the main pathogenetic significance of angiodystonic disorders. The type of lesion is predominantly axonal. The disease is characterized by a progressive course. In severe cases, the lower limbs are involved in the process.

III. Additional research.
1. Identification of possible causes of the disease: a) neurotoxic agents and physical factors that can cause disease in everyday life or at work; b) medicines; c) hereditary conditionality of neuropathy and type of inheritance; d) features of damage to internal organs, skin, other formations of the peripheral and central nervous system, shedding light on the etiology of polyneuropathy.
2. EMG, ENMG: judgment about the type (axonopathy, myelinopathy) and the extent of the lesion in dynamics; help in differentiation with myasthenia gravis, myopathic syndrome. It should be taken into account that the regression of movement disorders is not necessarily accompanied by the normalization of the nerve conduction function according to ENMG;
3. Research of cerebrospinal fluid: detection of protein-cell dissociation in order to clarify the nature of polyneuropathy (autoimmune, Guillain-Barré syndrome).
4.Sural nerve biopsy is an invasive procedure, the use of which is limited by strict indications for obtaining diagnostic information.
5.Biochemical studies of blood and urine. Conducted for diagnostic and differential diagnostic purposes. The range of substances to be determined or their metabolites depends on the proposed etiology of polyneuropathy (diabetes, porphyria, hypoglycemia, uremia, etc.).
6. Somatic, X-ray, ophthalmological and other examinations, taking into account the alleged etiology.
7. Bacteriological, virological, immunological examination, depending on the possible etiological factor.
8. Identification of peripheral vegetative-vascular disorders using additional methods: RVG, thermal imaging, etc.
9. Diagnosis of the syndrome of progressive autonomic failure, most often observed in diabetic, porphyria, alcoholic, acute inflammatory demyelinating polyneuropathy.

Differential Diagnosis
1. Between polyneuropathies of various etiologies.
2. With myopathies, damage to peripheral nerves in other diseases (noted above in the description of individual clinical forms of polyneuropathies).

Course and forecast
Four types of polyneuropathy can be distinguished: acute (symptoms develop over several days); subacute (no more than a month); chronic (more than a month); recurrent (repeated exacerbations occur over many months or years). The prognosis, like the course, clearly depends on the etiology of the disease.

Principles of treatment
1. Hospitalization in the neurological department is mandatory for AIDP, CIDP, diphtheria, porphyria polyneuropathy (due to the possibility of respiratory and bulbar disorders), it is desirable for the purpose of diagnosis and treatment in case of suspected neuropathy of any etiology.
2. Staged and complex therapy, an adequate combination of pharmacological drugs (for pain syndrome - analgesics), physical and other methods (hyperbaric oxygenation, magnetic stimulation, laser blood irradiation, massage, physiotherapy, mechanotherapy, etc.), patient care taking into account the period and course of the disease.

3. Features of therapy, taking into account the etiological factor of polyneuropathy.
3.1. Infectious and autoimmune polyneuropathies. Treatment should be stationary:
- Corticosteroids are not prescribed for mild and moderate forms of AIDP.
In a severe form, an ascending course of the process, especially in case of respiratory failure, plasmapheresis (2-3 sessions), large doses of glucocorticoids (prednisolone, metipred - 1000 mg intravenously daily for 3 days) are used, if necessary - against the background of mechanical ventilation, which reduces the time respiratory assistance. There is evidence of the effectiveness of intravenous administration of immunoglobulin.
In the recovery period, drugs are used that improve microcirculation and tissue trophism (trental, sermion, phosphaden, cerebrolysin, B vitamins, etc.), physiotherapy, massage, exercise therapy (early, but carefully). Careful care is required;
- in CIDP, prednisolone or metipred is used at a dose of 1-1.5 mg/kg per day daily. A maintenance dose of prednisolone (10-20 mg every other day) is prescribed for a long time (up to 6-8 months) and is canceled after the restoration of motor functions. With an increase in sensorimotor disorders, pulse therapy is performed (as in severe manifestations of AIDP). In particularly severe cases, immunosuppressive drugs (azathioprine) may be effective. Other methods of treatment are similar to those used in patients with AIDP;
- in the treatment of diphtheria polyneuropathy, it is advisable to take into account the time of occurrence of neurological symptoms. In the case of early pharyngeal neuropathy, diphtheria toxoid is used, the best effect is achieved as a result of plasmapheresis, and in case of late demyelination, vasoactive drugs (trental, actovegin) and plasmapheresis;
- with herpetic neuropathy - etiotropic drugs: acyclovir (Zavirax) orally for 5-7 days, bonafton orally and topically in the form of an ointment, antihistamines, analgesics, B vitamins, UHF, ultrasound. With postherpetic neuralgia - famciclovir, tricyclic antidepressants, adrenergic blockers.

3.2. Hereditary neuropathies. Therapy for neural amyotrophy is ineffective. Supportive drug treatment is carried out in order to improve microcirculation and tissue trophism, massage, physiotherapy exercises. Of great importance is the care of the skin of the legs, orthopedic correction of deformities of the feet, with hanging feet - special shoes.
In acute intermittent porphyria, inpatient treatment: large doses of carbohydrates (glucose or levulose) intravenously, hematin, cytochrome C for 5-7 days, analgesics, anaprilin, chlorpromazine. With respiratory failure - controlled breathing, other resuscitation measures. Plasmapheresis is indicated.

3.3. Somatogenic polyneuropathies:
- in case of diabetic polyneuropathy, hospitalization in the endocrinological or neurological department is advisable in order to correct etiotropic therapy. Outpatient treatment is carried out in conjunction with an endocrinologist. It is necessary to normalize blood glucose levels and compensate for other manifestations of diabetes by rational dosing of long-acting insulin. Antiplatelet agents, nicotinic acid preparations, solcoseryl, trental (pentyline) are used. In non-insulin dependent diabetes, alpha-lipoic acid is effective. Anabolic hormones are prescribed in case of proximal motor neuropathy. With intractable pain syndrome, analgesics (paracetamol), finlepsin and amitriptyline in small doses. Physiotherapeutic methods (novocaine electrophoresis, four-chamber baths, etc.), oxygen therapy can be recommended;
- in case of paraneoplastic polyneuropathies, the treatment is symptomatic, regression of symptoms is possible after removal of the tumor and corticosteroid therapy.

3.4. Neuropathy in diffuse connective tissue diseases. Treatment in conjunction with a therapist, usually in a hospital setting. Glucocorticoids (prednisolone) are needed, in severe cases, plasmapheresis. Prescribe analgesics, B vitamins, trental. Therapy for tunnel neuropathies is common.
3.5. Toxic polyneuropathies. The general principle is the exclusion of the influence of the etiotropic factor. Treatment in the acute period of poisoning in an occupational pathology or neurological hospital, in the period of restoration of motor functions in the rehabilitation department, on an outpatient basis. The nature of therapy depends on the specific toxic agent. Therapy of polyneuropathies is carried out in a complex manner according to the usual rules. Identification of drug polyneuropathy requires discontinuation of the drug. Treatment of tuberculosis with isoniazid (tubazid) should be accompanied by the use of pyridoxine (vitamin B6). With diagnosed polyneuropathy, pyridoxine is administered parenterally.
- alcoholic polyneuropathy. Inpatient treatment is recommended in case of progression of neurological symptoms (in the neurological department, psychiatric hospital). You need a complete rejection of alcohol, a balanced diet rich in vitamins. Vitamins B1, B6, B12 parenterally, analgesics, antidepressants, clonazepam, finlepsin (in case of persistent pain syndrome), physiotherapy, massage, corrective gymnastics.

3.6. Polyneuropathies caused by the influence of physical factors. A change in working conditions is required (temporary or permanent exclusion of the etiological factor). Treatment of neuropathy according to general principles, taking into account the predominance of peripheral vegetative-vascular disorders: ascorbic acid, indomethacin, trental, Ca blockers (nifedipine) and other drugs that improve microcirculation.

Medical and social examination Criteria of VUT
1. In infectious and autoimmune polyneuropathies:
- AIDP, Fisher's syndrome and diphtheria polyneuropathy. The timing of VN depends on the rate of recovery of motor functions. In the case of early regression of symptoms, they do not exceed 3-4 months, with a delayed one, it is advisable to continue treatment on a sick leave according to the decision of the VC, sometimes up to 6-8 months (if it is assumed that the patient will be able to return to work or it will be possible to determine a less severe disability group ). The terms of treatment in a hospital (including the department of rehabilitation therapy) range from 1-2 to 3-4 months, depending on the severity of the disease. More than half of patients are discharged from the hospital after full recovery of functions. They need short-term outpatient treatment due to asthenic syndrome. Persons of physical labor need temporary relief of working conditions on the recommendation of the VC. The pronounced consequences of the disease (in severe cases) give rise to referral to the BMSE until the end of the recovery period, no later than 4 months of being on sick leave. In a similar way, the issue is resolved in patients with relapses and a progressive course;
- HIDP. Usually long-term VN (up to 4 months). In case of recovery - return to work, often with restrictions depending on the profession. With the ineffectiveness of therapy, relapses - referral to BMSE. As a rule, there are no grounds for extending treatment on sick leave;
- herpetic neuropathy. Treatment in a hospital - an average of 20 days. VN is most often limited to 1-2 months, however, with postherpetic neuralgia, the terms are long; in addition, patients are temporarily disabled during the period of exacerbation. This also applies to patients with Guillain-Barré syndrome.
2. Hereditary neuropathies:
- with neural amyotrophy of Charcot-Marie-Thus, the basis for treatment on a sick leave may be decompensation of the disease, more often due to unfavorable working conditions, the need for examination, treatment (duration of VN - 1 - 2 months);
- porphyric polyneuropathy. Patients are temporarily unable to work during an attack (1.5-2 months), when inpatient treatment is necessary. With prolonged restoration of functions - up to 3-4 months, sometimes with an extension for another 2-3 months or referral to the BMSE (in case of a pronounced motor defect).

3.Somatogenic polyneuropathies:
- diabetic. VN is determined taking into account the course of diabetes (decompensation). Progressive polyneuropathy, especially manifested by vegetative and trophic disorders, lengthens the duration of treatment on sick leave. In the case of proximal motor neuropathy, local and multiple neuropathies, the duration of VN mainly depends on the rate of recovery of motor functions (usually 2-3 months).
- paraneoplastic polyneuropathies are the basis for VL during the initial diagnosis of cancer. In the future, the need for VN and the timing depend on the results of surgical or other treatment of the neoplasm.

4. Neuropathy in diffuse connective tissue diseases. The need for VN mainly depends on the clinical manifestations of the underlying disease. However, neuropathies, including tunnel ones, Guillain-Barré syndrome can also be the main reason for sick leave treatment. The terms of VN are determined by their curability, the nature of the course.

5. Toxic polyneuropathies. Insufficient effectiveness of therapy, the duration of recovery of functions in most forms cause a long-term VL, the need to continue the sick leave according to the decision of the VC. In patients with alcoholic polyneuropathy, combined damage to the central nervous system and the possibility of relapses are taken into account. Prolongation of VN beyond 4 months is usually not advisable. With arsenic, organophosphorus polyneuropathy due to the ineffectiveness of therapy, long-term (more than a year) restoration of VL functions should not be more than 4 months. Drug-induced polyneuropathy, which usually regresses well after discontinuation of the drug, by itself can lead to LN within 2-3 months.

6. Polyneuropathy due to exposure to physical and toxic factors, as a rule, professional. Therefore, in the initial phase of the disease, it is worth limiting the temporary transfer of the patient to easier work according to the professional bulletin (for 1.5-2 months). With persistent pronounced trophic and motor disorders, patients are temporarily disabled for the period of outpatient or inpatient treatment (1-2 months).
The main causes of disability
1. Motor defect due to peripheral, rarely mixed para-, tetraparesis of the extremities. Due to the more pronounced and earlier emerging lower paraparesis in the residual period of polyneuropathy or in the progressive course of the disease, the ability to move and overcome obstacles is impaired to varying degrees. In the case of severe lower paraparesis, movement is possible only with the help of assistive devices, with paraplegia, patients depend on the help of other people. Upper paraparesis, due to the predominant dysfunction of the hands, to a greater extent limits the labor opportunities of patients, depending on the profession. Significant severity of paraparesis reduces the ability or makes it impossible to perform applied activities in everyday life (personal care and other tasks that require sufficient manual activity). Severe tetraparesis, tetraplegia lead to the need for constant outside care and assistance.

2. Violations of sensitivity. Pain syndrome affects the state of life of a relatively small number of patients (especially with occupational polyneuropathy, postherpetic neuralgia). Distal hypesthesia, and especially sensitive ataxia, exacerbate the degree of disability and labor opportunities in patients with diabetic, alcoholic and some other polyneuropathies.

3. Vegetative-vascular and trophic disorders can significantly affect the motor functions of the limbs, reduce the ability to walk for a long time, stand, reduce the possibility of manual operations, which leads to limited life and disability (more often with polyneuropathies caused by physical factors).
1. General: adverse weather conditions, low temperature, high humidity, significant physical exertion, contact with neurotoxic substances.
2. Individual (depending on the profession, working conditions): exposure to a specific toxic substance, functional overstrain of the upper limbs, prolonged walking, standing, work at height, near moving mechanisms (with ataxia), associated with local and general vibration.

Able-bodied patients
1. Those who have had acute infectious, autoimmune polypolyneuropathy with a good (complete) recovery of functions or when mild and moderate motor, sensory, trophic disorders do not prevent the continuation of work in the specialty.
2. Patients with diabetic polyneuropathy (in the initial stage of the disease, with compensated diabetes), mild manifestations of Charcot-Marie-Tooth neural amyotrophy, alcoholic, some other toxic (drug), somatogenic polyneuropathies with a regressive or stationary course of the disease (if the clinical manifestations of the underlying disease are not limit the ability of patients to work).
3. Patients with occupational polyneuropathy with moderate functional impairment, without motor disorders, with a low-progressive course of the disease, capable of performing work in their main profession or having low qualifications, if it is necessary to change working conditions, entailing some reduction in earnings, or if professional work requires more stress, than before (when there are no grounds for determining the III group of disability). A loss of 10 to 30% of professional ability to work is established.

Indications for referral to BMSE
1. Paresis and paralysis of the limbs, persistent pain syndrome, sensitive ataxia, pronounced autonomic and trophic disorders, manifestations of progressive autonomic failure, significantly limiting the patient's life.
2. Long-term temporary disability with a poor or doubtful prognosis regarding the restoration of motor and other functions.
3. Progressive course and relapses of the disease, taking into account the etiology of polyneuropathy, postherpetic neuralgia.
4. Inability to return to work in the specialty due to a motor defect and (or) contraindicated working conditions that cannot be eliminated by the conclusion of the VC.

Required minimum examination when referring to BMSE
1. General blood and urine tests.
2. Data from the study of cerebrospinal fluid (in patients with infectious and autoimmune polyneuropathies).
3.EMG, ENMG (preferably in dynamics).
4. Data of somatic, ophthalmological examination (taking into account the etiology of polyneuropathy).
5. Results of bacteriological, virological research (depending on the etiology of polyneuropathy).
6.RVG, thermal imaging.
7. Biochemical studies of blood and urine (for toxic, porphyria, somatic polyneuropathies).

Disability Criteria
Group III: moderate motor and (or) atactic, moderate or pronounced vegetative-vascular, trophic, sensory disorders in the stationary or slowly progressing course of the disease in case of: a) loss of profession (the need for professional retraining for the period of its implementation); b) the need to transfer to another job, which is associated with a significant reduction in the volume of production activities (according to the criteria for limiting the ability to work, independent movement of the first degree).

Group II: severe disability due to severe motor and (or) atactic, vegetative, trophic disorders, severe and persistent pain syndrome in the progressive, recurrent or stationary course of the disease (according to the criteria for limiting the ability to work of the second degree, to move and self-service of the second degrees). The uneven involvement of the upper and lower extremities in the pathological process, the frequent preservation of the functions of the hands, even with severe lower paraparesis, makes it possible to recommend such patients to work at home or in specially created conditions at enterprises, institutions or organizations.

Group I: a pronounced limitation of life in patients with distal tetraparesis, lower paraplegia, often in combination with sensitive ataxia, which necessitates constant outside care (according to criteria for limiting the ability to move and self-care of the third degree).

In case of persistent pronounced impairment of motor functions, after 4 years of observation, disability is determined indefinitely.

Causes of disability: 1) general illness; 2) occupational disease: a) in patients with polyneuropathies that have developed as a result of exposure to toxic substances in production conditions; b) due to the influence of physical factors; vibration disease, cold polyneuropathy; autonomic polyneuropathy due to overexertion of the upper limbs. At the same time, the degree of loss of professional ability to work is determined in percent; 3) disability due to an illness acquired during the period military service(or occurred within 3 months from the date of dismissal from the army); 4) disability since childhood.

Exam questions:

2.7. Osteochondrosis of the cervicothoracic spine: main syndromes of lesions, clinic, diagnosis, treatment.

2.8. Osteochondrosis of the lumbosacral spine: main lesion syndromes, clinic, diagnosis, treatment.

2.9. Polyradiculoneuropathy: etiology, pathogenesis, classification, clinic, diagnosis, treatment, disability examination, prevention.

Anatomical and physiological features of the peripheral nervous system

Peripheral nervous system is part of the nervous system that connects the central nervous system with sensory organs and with voluntary muscles, two different groups of nerves are distinguished in it: cranial and spinal:

- Rootsspinal cord and brain fundamentally have a similar functional structure and include motor, sensory and autonomic fibers However, due to the peculiarities of the phylo- and ontogenesis of the head end of the body, the cranial nerves differ anatomically from the spinal ones.

- Peripheral nervous system of the head and neck (cranial nerves) includes 10 (11) cranial nerves (with the exception of I and II), discussed in the sections on the brain stem and divided into systems:

1) Analyzers: vestibular and auditory (VIII),

2) Oculomotor nerves (III, IV, VI) - ensuring the movement of the eyeball,

3) System general sensitivity faces (V) - analogue of the posterior horns of the spinal cord,

4) System facial nerve(VII) - providing facial expressions,

5) System ensure digestion- chewing (V, XII), taste and salivation (VII, IX, X), swallowing and digestion (IX, X) - and functions of internal organs- heart, lungs, etc. (X)

6) Accessory nerve(XI) - ensuring the movement of a part of the muscles of the upper shoulder girdle.

- Peripheral nervous system of the trunk and limbs includes:

1) at the cervical level - spinal nerve roots from C1 to Th1, as well as the cervical and brachial plexus,

2) at the chest level - spinal nerve roots from Th2 to Th12, do not form plexuses,

3) at the lumbosacral level - spinal nerve roots from Th12 to Co2, as well as the lumbar, sacral and coccygeal plexus.

PNS diseases: general questions

Diseases of the peripheral nervous system(PNS) make up about half in the structure of neurological morbidity in the adult population. They are the most common cause of temporary disability (76% of cases in outpatient clinics and 55.5% in neurological hospitals). Among all the causes of temporary disability, PNS diseases occupy the 4th place (Antonov I.P., Gitkina L.S., 1987). In this case, the main etiological factor is osteochondrosis of the spine (according to various sources, from 60 to 90%). Tunnel compression-ischemic lesions of nerves account for 20-40%. However, epidemiological data are fragmentary and incomplete due to the dispersion of PNS diseases in various sections of the ICD-X. In addition to class VI, they are included in common group diseases of the musculoskeletal system and connective tissue (class XIII), there are also other classes.

Classification of diseases of the peripheral nervous system

- I. Vertebrogenic lesions.

- II. Damage to the nerve roots, nodes, plexuses:

1. Meningoradiculitis, radiculitis (non-vertebrogenic);

2. Radiculoanglionitis, ganglionitis, truncites;

3. Plexites;

4. Plexus injuries

- III. Multiple lesions of roots, nerves:

1. Infectious-allergic polyradiculoneuritis;

2. Infectious polyneuritis;

3. Polyneuropathies: 3.1. Toxic; 3.2. allergic; 3.3. Dysmetabolic; 3.4. Discirculatory; 3.5. Idiopathic and hereditary.

- IV. Damage to individual spinal nerves:

1. Traumatic

2. Compression-ischemic (mononeuropathies)

3. Inflammatory (mononeuritis).

- V. Damage to the cranial nerves:

1. Trigeminal neuralgia and other cranial nerves;

2. Neuritis, neuropathy of the facial nerve;

3. Neuritis of other cranial nerves;

4. Prosopalgia:

5. Dentistry, glossalgia.

Anatomical and physiological features of lesions of the spinal nerves. Root Syndrome.

The roots of the spinal cord have a strictly segmental structure. and consists of several elements:

- back spine(dendrites and axon of afferent neuron) with spinal ganglion(the body of the first neuron of any afferent pathway - the pathways of superficial and deep sensitivity, cerebellar and autonomic pathways), with damage to which occurs:

1) girdle pain in the zone of innervation of the segment,

2) violation of all types of sensitivity according to the segmental type,

3) decrease in reflexes (interruption of the afferent part of the reflex),

4) soreness at the exit points of the roots.

- front spine(axon II of the [peripheral] motor neuron, axon II of the autonomic neuron), with damage to which occurs:

1) peripheral paralysis in the zone of innervation of the segment with a decrease in the corresponding reflex

- mixed spinal nerve, formed fusion of the anterior and posterior roots the spinal cord, which, leaving the intervertebral foramen of the spinal canal, is divided into four parts:

1) front branch - forms nerve plexuses and innervates the skin and muscles of the limbs and the anterior surface of the body,

2) back- innervates the skin and muscles of the posterior surface of the body,

3) shell part- innervates the membranes of the spinal cord

4) connecting part- innervates sympathetic ganglions.

radicular syndrome- a set of symptoms that occur when compression (or other impact) of the spinal root, consists of the following symptoms:

- pain shooting character along the affected root,

- sensory disturbances- more often hypoesthesia in the zone of innervation,

- movement disorders- Peripheral paresis of a group of muscles.

Separate spinal nerves and symptoms of their defeat:

- SpineC1:

Cranio-vertebral SMS, passes between the occipital bone and the 1st cervical vertebra,

2) front branch

3) back branch- suboccipital nerve, n. suboccipitalis (CI) - under the vertebral artery, in the groove of the vertebral artery of the atlas, then passes into the triangular space formed by the posterior rectus capitis major muscle, inferior and superior oblique muscles of the head, innervates muscles- m.rectus capitis posterir major et minor, obliquus capitis superior et inferior - and then skin- parietal region of the head

4) research methods(according to the ISCSCI system): muscle group- No, sensitivity zone- No

5) symptoms of damage: pain- parietal region, hypoesthesia- parietal region, paresis- is compensated by C2 muscles at the expense of the connecting branch.

- SpineC2:

1) place of exit from the spine- diskless PDS C I -C II,

2) front branch- as part of the cervical plexus,

3) back branch- goes around the lower edge of the inferior oblique muscle of the head and is divided into a number of short branches to muscles- m.semispinalis capitis - and n. occipitalis major, which, accompanying the occipital artery, pierces the semispinalis muscle of the head and the tendon of the trapezius muscle, innervates skin- parieto-occipital region.

4) research methods(according to the ISCSCI system): muscle group- No, sensitivity zone- occipital protuberance.

5) symptoms of damage: pain hypoesthesia- parieto-occipital region, paresis- compensated by C1 muscles at the expense of the connecting branch.

- Spine C3:

1) place of exit from the spine- PDS C II -C III,

2) front branch- as part of the cervical plexus,

3) back branch- third occipital nerve, n. occipitalis tertius - located medially from the large occipital nerve, muscles- No, leather- occipital region.

4) research methods(according to the ISCSCI system): muscle group- No, sensitivity zone- supraclavicular fossa

5) symptoms of damage: pain- neck, sensation of swelling of the tongue (connecting branch from XII c.n.), hypoesthesia- neck, paresis- No.

- Spine C4:

1) place of exit from the spine- PDS C III -C IV,

2) front branch- as part of the cervical plexus,

3) back branch- to deep muscles neck - m.semispinales cervicis et capitis, splenius capitis - further perforates the fascia, innervating skin in the paravertebral region

4) research methods(according to the ISCSCI system): muscle group- No, sensitivity zone- acromioclavicular joint

5) symptoms of damage: pain- shoulder girdle, collarbone, in the region of the heart and liver, hiccups (participates in the formation of n.phrenicus), hypoesthesia- shoulder girdle, paresis- Difficulty in neck extension, respiratory disorders.

- SpineC5:

1) place of exit from the spine- PDSS IV -С V,

2) front branch

3) back branch - to deep muscles skin in the paravertebral region

4) research methods(according to the ISCSCI system): muscle group- flexion at the elbow and raising your hand to the horizontal, sensitivity zone- lateral side of cubital fossa

5) symptoms of damage:pain- the outer surface of the shoulder, the medial part of the scapula, hypoesthesia- the upper part of the outer surface of the shoulder (above the deltoid muscle), paresis- abduction and external rotation of the shoulder, partially - flexion of the forearm, weakness and hypotrophy of the deltoid muscle, areflexia- bicipital.

- SpineC6:

1) place of exit from the spine- PDSS V -C VI,

2) front branch- in the brachial plexus

3) back branch - to deep muscles neck - m.semispinales cervicis, splenius cervicis - further perforates the fascia, innervating skin in the paravertebral region

4) research methods(according to the ISCSCI system): muscle group- dorsiflexion of the hand , sensitivity zone- thumb,

5) symptoms of damage:pain- lateral surface of the forearm and hand, I-II fingers, hypoesthesia-lateral surface of the forearm and hand, I-II fingers, paresis- flexion and internal rotation of the forearm, partially - extension of the hand, areflexia- bicipital.

- SpineC7:

1) place of exit from the spine- PDSS VI -C VII,

2) front branch- in the brachial plexus

3) back branch - to deep muscles neck and skin in the paravertebral region

4) research methods(according to the ISCSCI system): muscle group- extension in the elbow joint , sensitivity zone- middle finger,

5) symptoms of damage: pain- neck, shoulder blade, shoulder girdle, back surface of the shoulder and forearm up to II-III fingers, hypoesthesia - II-III fingers, back surface of the hand and forearm, paresis - extension of the shoulder, extension of the hand and fingers, partially - flexion of the hand, areflexia- tricipal.

- SpineC8:

1) place of exit from the spine- PDSS VII -Th I,

2) front branch- in the brachial plexus

3) back branch - to deep muscles back, then pierces the fascia, innervating skin in the paravertebral region

4) research methods(according to the ISCSCI system): muscle group- flexion of the distal phalanx III fingers , sensitivity zone- little finger,

5) symptoms: pain- neck, inner surface of the forearm, hands up to IV-V fingers, hypoesthesia- IV-V fingers, the inner surface of the hand and forearm, paresis- flexion and spreading of the fingers, atrophy of the muscles of the elevation of the little finger, areflexia- tricipal.

- SpineTh1:

1) place of exit from the spine- PDSTh I -Th II,

2) front branch- in the brachial plexus

3) back branch - to deep muscles back, then pierces the fascia, innervating skin in the paravertebral region

4) research methods(according to the ISCSCI system): muscle group- abduction I finger , sensitivity zone- medial side of the cubital fossa,

5) symptoms: pain- the inner surface of the shoulder and axillary region, hypoesthesia- the inner surface of the shoulder and upper forearm, armpit, paresis- spreading fingers areflexia- No.

- SpineTh2-12:

1) place of exit from the spine- PDSTh I -Th II,

2) front branch- nn. intercostales (Th1-6); nn. thoracicoabdominal (Th7-11), n. subcostalis (Th12) - between the ribs to the intercostal muscles, the skin of the corresponding segment and the pleura, the final parts - to the abdominal muscles:

Outer group of costal muscles - mm. intercostales intimi, interni et externi (Th1-Th11), subcostale (Th12)

The inner group of costal muscles - m. transversus thoracis (Th1-Th11),

Paravertebral costal muscles - m.seratus posterior superior (Th2-5), m.serratus posterior inferior (Th9-12)

Abdominal muscles - m.obliquus abdominis extemus (Th5-12), m.obliquus abdominis internus (Th8-12), m.transversus abdominis (Th7-12), m. rectus abdominis (Th7-12), m. pyramidalis (Th12), m. quadratus lumborum (Th12);

3) back branch - to deep muscles back and mm.levatores costarum, then perforates the fascia, innervating skin in the paravertebral and scapular region,

4) research methods(according to the ISCSCI system): muscle group- No, sensitivity zone- For 2 - apex of the armpit 3 - 3rd intercostal space, 4 - level of nipples, 5,6,7,8,9 - 5,6,7,8,9 intercostal space, 10 - navel level 11 - 11 intercostal space, 12 - inguinal fold.

5) symptoms: pain and hypoesthesia- along the corresponding segment of the body, paresis- No, areflexia- upper abdominal (Th7-8), middle abdominal (Th9-10) and lower abdominal (Th11-12).

- SpineL1:

1) place of exit from the spine- PDSL I -L II,

2) front branch