Inflammatory polyneuropathy (inflammatory polyneuropathy)

According to the speed of development, they are distinguished:

• acute polyneuropathies develop in less than four weeks: the most common is Guillain-Barré syndrome;
• subacute polyneuropathy is called if the disease develops over 4-8 weeks;
• chronic course, when the disease develops for more than two months (8 weeks). The chronic course is more typical with alcoholism, uncompensated diabetes mellitus, vegetarianism, or simply poor nutrition leading to a deficiency of B vitamins, hereditary causes, inflammatory processes involving autoimmune mechanisms that primarily affect the myelin sheath of the nerves, etc.

In addition to the above, the cause of this disease can be: the use of neurotoxic drugs, chemotherapy, intoxication with salts of heavy metals, some chronic somatic diseases with symptoms of liver and kidney failure, diseases of the endocrine organs, for example, the thyroid gland.

Polyneuropathy also occurs in autoimmune diseases, both affecting predominantly peripheral nerves (Guillain-Barré syndrome, multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy, paraproteinemic polyneuropathy) and systemic connective tissue diseases (rheumatoid arthritis, Sjögren's disease, systemic lupus erythematosus).

In the development of polyneuropathy, infectious factors of both a bacterial nature (borreliosis, botulism, diphtheria, syphilis, HIV infection) and viral (influenza, cytomegalovirus, herpes, etc.) often play an etiological role. A special place in the group of polyneuropathies is occupied by hereditary neurodegenerative diseases, such as transthyretin familial amyloid polyneuropathy, polyneuropathy with porphyria, hereditary neuropathy with a tendency to paralysis from compression.

Polyneuropathy (PNP)

Polyneuropathy (PN) (patient information) Polyneuropathy is one of the most common diseases of the peripheral nervous system.

What is polyneuropathy? Polyneuropathies are a group of diseases characterized by multiple and diffuse damage to roots, plexuses and peripheral nerves.

What symptoms bother a patient with polyneuropathy? Depending on which nerve fibers are more involved in the pathological process, the following complaints arise:

• sensitive symptoms: the patient is bothered by numbness, tingling, burning, “crawling” in the hands and/or feet; unsteadiness when walking, aggravated in the dark and when closing the eyes;
• motor symptoms: clumsiness, weakness in the hands and/or feet develops; weight loss in the muscles of the arms and legs; impaired fine motor skills (difficulty fastening buttons and zippers, tying shoelaces, etc.); difficulty walking (“spanking” feet, difficulty getting up and down stairs, etc.);
• vegetative symptoms: fluctuations in blood pressure, “interruptions” in the heart, constipation or diarrhea, dry skin or increased sweating, decreased libido, erectile dysfunction.

With polyneuropathy, either an isolated lesion of a certain type of nerve fiber or a combination of them can occur, which leads to the appearance of a varied clinical picture.

How does polyneuropathy develop? According to the speed of development, they are distinguished:

• acute polyneuropathies (development < 4 weeks): the most common cause is Guillain-Barré syndrome;
• subacute polyneuropathy (development within 4-8 weeks);
• chronic polyneuropathies (development over > 8 weeks): common causes are diabetes mellitus, alcohol abuse, lack of B vitamins, chronic inflammatory demyelinating polyneuropathy, hereditary causes, etc.

What are the causes of the development of polyneuropathy? The most common causes of polyneuropathy in developed countries are diabetes and alcohol abuse. Other possible reasons may be:

• dieting, poor nutrition, vegetarianism, leading to the development of vitamin B12 deficiency;
• chemotherapy, the use of neurotoxic drugs, less often - intoxication with heavy metals, etc.;
• autoimmune damage to peripheral nerves with the development of dysimmune neuropathies (Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, paraproteinemic polyneuropathy, multifocal motor neuropathy, etc.);
• chronic somatic diseases: systemic autoimmune connective tissue diseases (systemic lupus erythematosus, rheumatoid arthritis, Sjogren's disease, etc.), chronic renal or liver failure, thyroid pathology, etc.;
• infectious diseases (Lyme borreliosis, HIV infection, neurosyphilis);
• oncological diseases with the development of paraneoplastic process;
• hereditary neuropathies (hereditary motor-sensory neuropathies, hereditary neuropathy with a tendency to paralysis from compression, transthyretin familial amyloid polyneuropathy, porphyritic polyneuropathy, etc.).

It is important to clarify the cause of polyneuropathy as early as possible, because The prognosis and treatment tactics depend on this!!!

What diagnostic methods can be used to detect polyneuropathy? An examination of a patient with polyneuropathy begins with a consultation with a neurologist, during which the doctor analyzes complaints and anamnestic information and assesses the neurological status. If there are subjective and objective symptoms indicating the presence of polyneuropathy, the doctor prescribes an examination:

• Electroneuromyography (ENMG) is the main instrumental method for diagnosing diseases of peripheral nerves, which not only confirms the fact of nerve damage, but also determines the nature of their damage (demyelinating, axonal, with conduction blocks).

In complex diagnostic cases, the doctor may recommend ultrasound of peripheral nerves, MRI of the plexuses with contrast, diagnostic lumbar puncture, morphological examination of the nerve (biopsy).

What diagnostic methods can be used to clarify the cause of polyneuropathy? To clarify the reasons that caused widespread damage to peripheral nerves, a thorough examination is necessary:

• comprehensive laboratory blood test: minimum volume - detailed general and biochemical blood test, RW, anti-HIV, HBsAg and anti-HCV, level of vitamins B1, B6, B9, B12, homocysteine ​​(if necessary, in each case individually, spectrum of laboratory examination is expanding)
• if hereditary neuropathy is suspected - molecular genetic analysis (search for mutations in the PMP22 gene, neuromuscular disease panel or full genomic sequencing), etc.;
• electrophoresis of serum and urine proteins with immunofixation + freelite;
• lumbar puncture with general analysis of cerebrospinal fluid;
• instrumental general somatic examination, including cancer screening (CT of the lungs, ultrasound of the mammary glands, mammography, ultrasound of the prostate and scrotum, endoscopy, colonoscopy, ultrasound of the abdominal and pelvic organs, PET-CT, etc.).

The examination plan is drawn up individually! It is important to remember that in every third case, after a thorough examination, it is not possible to clarify the cause of polyneuropathy - in this case, idiopathic polyneuropathy is diagnosed!!!

What treatment methods have been developed for polyneuropathy? From the standpoint of evidence-based medicine, pathogenetic treatment has not been developed for all polyneuropathies. Thus, with diabetic polyneuropathy, first of all, careful monitoring of blood glucose levels and compliance with all recommendations of the treating endocrinologist is necessary. For toxic polyneuropathy, such as alcoholic polyneuropathy, treatment begins with complete cessation of the bad habit. If deficiency polyneuropathy is detected, vitamin therapy is carried out, and the causes causing the development of the disease are eliminated.

Pathogenetic therapy (i.e. therapy aimed at correcting the mechanisms of disease development) is the main one in the treatment of dysimmune polyneuropathy (Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, paraproteinemic polyneuropathy, multifocal motor neuropathy, etc.). Depending on the type of dysimmune neuropathy, the optimal treatment method or their combination is selected individually, taking into account all the nuances of the disease. The following methods of pathogenetic therapy for dysimmune neuropathies have been developed: hormone therapy (use of high doses of glucocorticosteroid drugs), high-volume plasmapheresis, high-dose intravenous immunotherapy, as well as the use of cytostatics and monoclonal antibody preparations.

Currently, pathogenetic therapy has been developed for some hereditary polyneuropathies:

• Transthyretin familial amyloid polyneuropathy (TTR-FAP) – liver transplantation, stabilization of the transthyretin molecule;
• Fabry disease – enzyme replacement therapy;
• Porphyritic polyneuropathy - heme arginate.
• Refsum's disease - diet therapy, plasmapheresis.

Symptomatic therapy is used to eliminate unpleasant sensory sensations (numbness, burning, burning pain) in polyneuropathy. Local anesthetics, anticonvulsants, antidepressants, opioid analgesics, and psychotherapy are recommended.

Restorative and rehabilitation methods of treatment (transcutaneous electrical neurostimulation, acupuncture, biofeedback, interventional methods, massage, physiotherapy, balance therapy, robotic therapy) occupy an important place in the correction of neurological disorders in polyneuropathy.

If you have symptoms of polyneuropathy or have been diagnosed with polyneuropathy, you can undergo a comprehensive examination at the Center for Diseases of the Peripheral Nervous System of the Federal State Budgetary Institution National Center of Neurology, where they will help you clarify the diagnosis, identify the causes of damage to the peripheral nerves and prescribe therapy based on evidence-based medicine.

This area has long been one of the priorities for the Federal State Budgetary Institution Scientific Center for Science and Technology. Here, the country's first neuroreanimation department was created, which was famous for its unique methodology of caring for patients with severe forms of polyneuropathy requiring long-term artificial ventilation. Several decades ago, unique work was carried out on the diagnosis and treatment of certain forms of neuropathies: hereditary, diphtheria, dysimmune, etc. Here, for the first time in the USSR, plasmapheresis was introduced as a method of treating autoimmune diseases of the nervous system. We have accumulated unique domestic experience in the treatment and rehabilitation of patients with Guillain-Barre syndrome, severe forms of CIDP and neuropathies of other origins. The center for diseases of the peripheral nervous system includes 13 specialists, including 10 neurologists and 3 endocrinologists. All neurologists are proficient in ENMG research techniques.

Employees of the Center for Peripheral Nervous System Diseases provide consultations to patients on an outpatient basis within the framework of compulsory medical insurance and on a commercial basis.

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Important

In order to determine treatment tactics and correctly make a prognosis, it is fundamentally important to correctly establish the cause of the disease.
Naturally, this should begin with a thorough collection of the patient’s medical history and a detailed neurological examination. The neurologist analyzes the information received during the consultation and, based on his experience and knowledge, having discovered the symptoms of polyneuropathy, prescribes an examination.

For examination for certain indications, instrumental methods are used:

• ENMG (electroneuromyography) method is the most important not only for determining damage to a peripheral nerve, but also for clarifying the nature of the damage (damage to the myelin sheath, impulse conduction block, or axonal damage).
• Ultrasound of peripheral nerves.
• MRI of the nerve plexuses with contrast.
• Carrying out a biopsy (study of the nerve using morphological methods)
• Performing a lumbar puncture.

Symptoms

Symptoms of polyneuropathy may include:

• Gradual onset of numbness and tingling in the legs or arms that may spread up the arms and legs
• Sharp stabbing or burning pain
• Increased sensitivity to touch
• Lack of coordination and falling
• Muscle weakness or paralysis if motor nerves are damaged

If autonomic nerves are involved, symptoms may include:

• Heat intolerance, as well as changes in sweating
• Digestive, bladder, or bowel problems
• Changes in blood pressure, which may cause dizziness

To diagnose and clarify the causes, laboratory tests are carried out:

• clinical and biochemical blood test;
• the level of B vitamins and blood homocysteine ​​are determined: the presence of antibodies to the most common infections is determined - HIV, syphilis, hepatitis B and C, etc. according to indications);
• if there is reason to suspect a hereditary cause of the disease, a complex of genetic studies is carried out;
• the protein composition of blood serum is determined using freelite immunofixation;
• A cerebrospinal fluid analysis is performed.

2. Causes of the disease

There are many causes of polyneuropathy. The most common are type 1 and type 2 diabetes. Other causes of polyneuropathy include age, alcohol or drug abuse, AIDS, pollution and heredity. Be that as it may, in about a third of all cases the causes of polyneuropathy remain unknown.

If the nerves are indeed damaged, the reasons are as follows:

• Insufficient blood supply to the nerves;
• Abnormal behavior of nerves.

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To exclude paraneoplastic syndromes, oncological screening is performed:

• carefully examine the mammary glands (ultrasound and mammography);
• lungs (CT);
• gastrointestinal tract (endoscopic examinations are performed); Ultrasound of the abdominal cavity, pelvis, genital organs;
• if necessary, PET-CT.

It is necessary to understand that drawing up an examination plan is individual for each patient. The polyetiology and different mechanisms of development of the disease, the diversity of the clinical picture require a detailed and comprehensive examination, but despite this, it is still not always possible to identify the cause of the disease. In this case, the diagnosis of idiopathic polyneuropathy is established.

Treatment

Treatment of polyneuropathy includes the maximum possible elimination of the cause that led to its development.

• So, if there is diabetic polyneuropathy, then it is necessary to strictly and constantly monitor blood sugar and be under the supervision of an endocrinologist in order to prevent decompensation of the underlying disease.
• If polyneuropathy has developed due to alcoholism, you must completely stop drinking alcohol. It is necessary to adjust your diet and treat with vitamins if a deficiency has developed.
• Treatment of polyneuropathies, which are based on autoimmune processes, is called pathogenetic therapy. At the same time, treatment is also individualized and selected depending on the type of disease, taking into account all the nuances of the disease mechanism and the individual characteristics of the patient. This includes treatment with high doses of glucocorticoid hormones, the use of cytostatics and monoclonal antibody drugs in the treatment, high-dose intravenous immunotherapy with human class G immunoglobulin drugs and high-volume programmed plasmapheresis.