Ascending paralysis

Neurological syndrome - ascending paralysis - is an acute form of post-infection multiple damage to the peripheral nerves. Other names of this pathology are ascending Landry paralysis or Landry syndrome, ascending paralysis of Guillain-Barre (Guillain-Barre-Strohl syndrome, GBS). There is also the name of the Landry-Guillain-Barre syndrome.

In general, all these terms are used to describe a fairly clinically heterogeneous pathology - inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor neuropathy of axons, acute motor-sensory neurosonic axons and Miller-Fisher syndrome.

[1], [2], [3], [4],

Epidemiology

The total annual statistics for ascending paralysis is one case for 55-91 thousand people. In Western countries, the number of new episodes per year is from 0.89 to 1.89 cases per 100,000 people. The risk of developing an ascending paralysis is increased by 20% for each decade of life (data from the European journal of physical and rehabilitation medicine).

[5], [6], [7], [8],

Causes of the ascending paralysis

The ascending paralysis of Guillain-Barre (or Landry) develops as a result of damage to myelin sheaths of axons of nerve fibers.

Recently, it is believed that the causes of ascending paralysis are of an autoimmune nature: the body's defense system mistakenly attacks the nerve cells of the peripheral nervous system and their supporting structures. Axons (processes) of nerve cells that transmit nerve impulses to neuromuscular junctions are covered with a sheath of Schwann cells that contain myelin.

[9], [10], [11], [12]

Pathogenesis

Today, the pathogenesis of ascending paralysis, which is caused by the violation of the myelin sheath of nerve fibers and the reduction or complete cessation of nerve signals, is associated with the activation of cellular immunity (T-lymphocytes and macrophages) and the development of a state that, by its mechanism, is close to a slowed down allergic reaction. This is expressed in the production of antibodies IgG, IgM and IgA against the envelope cells (gangliosides GM1, GD1a, GT1a and GQ1b).

Most often, the synthesis of autoantibodies in the body is provoked by a previous infection. Infectious causes of ascending paralysis are extremely diverse. As one of the symptoms, ascending paralysis of Guillain-Barre (ascending paralysis of Landry) can be observed in patients with:

• influenza, diphtheria, chickenpox, rubella and measles;
• rabies and brucellosis;
• Herpes viral infection, cytomegalovirus, Epstein-Barr virus and hepatitis E;
• primary infectious and secondary (postvaccinal) encephalitis;
• iksodovy tick-borne borreliosis;
• respiratory form of mycoplasmosis and chlamydia, that is caused by Mycoplasma pneumoniae and Chlamydophila pneumoniae atypical pneumonia;
• lymphocytic choriomeningitis (which is a viral infection carried by mice);
• acute disseminated encephalomyelitis;
• systemic lupus erythematosus.

Closely associated with acute infectious disease from the category of zoonoses, campylobacteriosis and ascending paralysis. Campylobacteriosis is caused by the bacterium Campylobacter jejuni, which, penetrating the digestive tract, begins to multiply with the release of toxins. As a result, inflammation, swelling and even ulceration of the mucous membranes of the gastrointestinal tract develops, as well as general intoxication of the body (through blood and lymph). In this case, the body produces lipo-oligosaccharides of campylobacterial membranes of IgA and IgG antibodies, which cause inflammation and degeneration of the human myelin sheaths and nerve cells.

According to the US National Institute of Neurological Disorders and Stroke (NINDS), about a third of cases of ascending paralysis are caused by campylobacteriosis.

Among the vaccines that caused the upward paralysis, the scandal with vaccination against swine flu in 1976-1977 is known. And in the instructions for the Priori vaccine (from measles, rubella and epidparotitis viruses) as one of the dozens of possible side effects, the ascending paralysis of Guillain- Barre.

[13], [14], [15], [16], [17], [18]

Symptoms of the ascending paralysis

Physicians note that symptoms of ascending paralysis appear in patients after obvious signs of infection in the form of sore throat, rhinitis, or diarrhea for one to six weeks. And only after this, the first signs of GBS are manifested: weakness in the legs and hands. Often, weakness is accompanied by paresthesia of the fingers of the feet and hands and muscle pain, which rises from the distal extremities to the proximal ones.

The process can affect both sides equally (para- or tetraplegia), but it can also be one-sided (hemiplegia). Over time, the condition is exacerbated. Muscular weakness and impaired movement in the form of mild paralysis increases in all different ways: abruptly (for 7-12 hours) or more measuredly (up to two weeks and longer). At every fifth patient, muscle weakness continues to progress for a month.

After weakness ceases to progress, a stabilization phase occurs, which can last from two to seven days to six months. The main symptoms of ascending paralysis at this stage include painful paresthesia; pain in the muscles in the head, neck and back; reduction or absence of tendon reflexes (hypo- or areflexia).

In almost half of the cases of the ascending paralysis of Guillain-Barre, the muscles of the neck and skull may be affected, causing weakness in the facial muscles, difficulty with swallowing and chewing, and sometimes the weakness of the eye muscles-ophthalmoplegia (Miller-Fisher syndrome).

In 8% of cases paralysis affects only the lower extremities (paraplegia or paraparesis), and about a fifth of patients are unable to walk unaided after six months of illness. However, about a third of patients are able to move independently (with some deviations in coordination of movements).

[19], [20],

Complications and consequences

Complications of chronic paralysis are accompanied by muscle tissue atrophy and complete disability. Vegetative disorders - sudden fluctuations in blood pressure, cardiac arrhythmias, swelling, increased sweating - are noted in at least 40% of patients with ascending paralysis. Often, cardiac complications reach the urgent need for stimulation of myocardial contraction or the installation of a pacemaker driver.

Consequences in severe cases (up to 25%) - weakening of the diaphragm and paralysis of the respiratory muscles with a lethal outcome.

[21], [22], [23], [24], [25], [26], [27]

Diagnostics of the ascending paralysis

Clinical diagnosis of ascending paralysis is performed using lumbar puncture in the lumbar region of the spinal column and examination of the obtained cerebrospinal fluid (for the presence of protein and the level of cellular elements). The diagnosis is confirmed by the presence of albuminocytological dissociation in the cerebrospinal fluid.

Also blood tests are taken - general, biochemical and immunoenzymatic (for antibodies to bacterial agents). Serological and cytological examination of smears from the throat, feces analysis can be prescribed.

Instrumental diagnostics include:

• electromyography (EMG), which allows to investigate the conductivity of peripheral nerves;
• magnetic resonance imaging (MRI) of the spinal cord.

[28], [29], [30], [31]

Differential diagnosis

Differential diagnosis of the ascending paralysis of Guillain-Barre is necessary to exclude the compression of the spinal cord, spinal muscular atrophy, leukemia, poliomyelitis, epiduritis, hematomyelia, lymphoma, multiple sclerosis, neurosyphilis (spinal cord), syringomyelia, cerebral palsy due to brain injury . In addition, Guillain-Barre's ascending paralysis from acute myelopathies (with chronic back pain), porphyria (with abdominal pain, cramps and mental disorders), polyradiculitis in HIV infected patients and patients with Lyme disease, as well as symptoms of organophosphorous poisoning compounds, thallium, arsenic and hemlock.

Treatment of the ascending paralysis

Treatment of ascending paralysis is carried out in a neurological hospital.

If the ascending paralysis of Landry progresses rapidly, emergency medical care is required in the intensive care unit, where there are conditions for using (if necessary) an artificial lung ventilation device.

Key methods of treatment of Guillain-Barre syndrome are plasmapheresis or intravenous immunoglobulin (Immunoglobulin human), that is, immunotherapy is aimed at reducing the symptoms and complications of ascending paralysis.

Therapeutic plasmapheresis (blood filtration) is performed to remove the attacking nerve cells of antibodies from the bloodstream (five procedures for two weeks). Similarly, neutralizes harmful antibodies and inflammation by the introduction of IgG-Gabriglobin, Hamuneks, Gamimun, Octagam, Flebogamma, Gammagard, etc. Into the blood. They are administered by infusion, the daily dose is calculated at 0.4 g per kilogram of body weight. The standard amount of infusion is one for 5 days. Among possible side effects of immunoglobulins are temperature increase, headache, nausea, vomiting, allergic reactions and inflammation of the liver. It should be borne in mind that with violations of the blood supply to the brain or heart, with excessive body weight and with a decrease in the volume of circulating blood (hypovolemia), the introduction of immunoglobulin can lead to the formation of thrombi. Therefore, concurrently prescribed drugs for thrombosis (anticoagulants).

According to the Journal of Clinical Immunology, both treatments are equally effective. Plasmapheresis accelerates recovery when used within four weeks of the onset of symptoms. And treatment of an ascending paralysis by a combination of immunoglobulins with plasmapheresis is carried out within two weeks after the onset of symptoms and has fewer complications. Western doctors came to the conclusion that the use of glucocorticoids in the therapy of this syndrome does not help accelerate the recovery and potentially can even postpone it. However, in domestic clinical practice in some places continue to use corticosteroid hormones (for example, intravenously administered prednisolone).

Also, drugs - Suprastin or Tavegil (on a tablet three times a day) are used, however, among the side effects of antihistamines, there are general weakness (in addition to increased drowsiness) and a decrease in blood pressure.

Used inhibitory cholinesterase and pulse-boosting drug Ipidacrin (Neuromidine), which is administered subcutaneously (0.2 g per day). The use of this drug can cause dyspepsia, diarrhea, dizziness, skin reactions, as well as a decrease in heart rate and spasm of the bronchi and musculature of the uterus. It is not used in patients with angina, bronchial asthma and vestibular apparatus disorders.

In addition, in the treatment of ascending paralysis, it is necessary to take B vitamins.

In the recovery period, physiotherapeutic treatment is shown: hydrotherapy, electrophoresis, iontophoresis, UV irradiation, acupuncture, therapeutic massage.

Forecast

The rate and degree of recovery after ascending paralysis are different. And the forecast of Guillain-Barre syndrome is determined taking into account age: in patients older than 40 years, the results of treatment may be lower than in younger patients.

Almost 85% of patients recover within a year after illness; 5-10% after treatment have problems with movement. Because of the complications and the initial severity of the symptoms, about 5% of cases end up lethal.

Ascending paralysis of Guillain-Barre can give relapses (2-3% of cases), especially if the transferred infections gave serious systemic complications.

[32], [33], [34], [35], [36]