Rising paralysis

Neurological syndrome – ascending paralysis – is an acute form of post-infectious multiple damage to peripheral nerves. Other names for this pathology: ascending Landry paralysis or Landry syndrome, ascending Guillain-Barré paralysis (Guillain-Barré-Strall syndrome, GBS). There is also the name Landry-Guillain-Barré syndrome.

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

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Epidemiology

The overall annual statistics for ascending paralysis is one case per 55-91 thousand people. In Western countries, the number of new episodes per year is from 0.89 to 1.89 cases per 100 thousand people. The risk of developing ascending paralysis increases for each decade of life by 20% (data from the European Journal of Physical and Rehabilitation Medicine).

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Causes of ascending paralysis

Ascending Guillain-Barré (or Landry's) palsy develops as a result of damage to the myelin sheaths of the axons of nerve fibers.

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

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Pathogenesis

Today, neurologists associate the pathogenesis of ascending paralysis, which results from damage to the myelin sheath of nerve fibers and a decrease or complete cessation of nerve signal transmission, with the activation of cellular immunity (T-lymphocytes and macrophages) and the development of a condition that is similar in its mechanism to a delayed allergic reaction. This is expressed in the production of IgG, IgM and IgA antibodies against the cells of the sheath (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 Guillain-Barré paralysis (ascending Landry paralysis) can be observed in patients with:

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

Closely related are the acute infectious disease from the zoonotic category campylobacteriosis and ascending paralysis. Campylobacteriosis is caused by the bacterium Campylobacter jejuni, which, penetrating the gastrointestinal tract, begins to multiply and release toxins. As a result, inflammation, swelling and even ulceration of the mucous membranes of the gastrointestinal tract develop, as well as general intoxication of the body (through the blood and lymph). At the same time, the body produces IgA and IgG antibodies to the lipo-oligosaccharides of the cell membranes of campylobacter, which cause inflammation and degeneration of the person's own 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 have caused ascending paralysis, the scandal with swine flu vaccinations in 1976-1977 is well known. And in the instructions for the Priorix vaccine (against measles, rubella and mumps viruses), ascending Guillain-Barré paralysis is listed as one of three dozen possible side effects.

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Symptoms of ascending paralysis

Doctors note that the symptoms of ascending paralysis appear in patients after obvious signs of infection in the form of a sore throat, rhinitis or diarrhea for one to six weeks. And only after this, the first signs of GBS appear: weakness in the legs and arms. Weakness is often accompanied by paresthesia of the toes and hands and muscle pain ascending from the distal to the proximal parts of the limbs.

The process may affect both sides equally (para- or tetraplegia), but may also be unilateral (hemiplegia). Over time, the condition worsens. Muscle weakness and movement disorders in the form of mild paralysis increase differently in everyone: sharply (in 7-12 hours) or more gradually (up to two weeks or longer). In every fifth patient, muscle weakness continues to progress over the course of a month.

After the weakness stops progressing, 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; muscle pain in the head, neck, and back; decreased or absent tendon reflexes (hypo- or areflexia).

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

In 8% of cases, paralysis affects only the lower limbs (paraplegia or paraparesis), and about a fifth of patients are unable to walk without assistance after six months of illness. However, about a third of patients are able to move independently (with some deviations in motor coordination).

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Complications and consequences

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

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

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Diagnostics of ascending paralysis

Clinical diagnosis of ascending paralysis is made by 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.

Blood tests are also taken - general, biochemical and enzyme immunoassay (for antibodies to bacterial agents). Serological and cytological studies of throat smears and stool analysis may be prescribed.

Instrumental diagnostics includes:

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

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Differential diagnosis

Differential diagnosis of ascending Guillain-Barré palsy is necessary to exclude spinal cord compression, spinal muscular atrophy, leukomyelitis, poliomyelitis, epiduritis, hematomyelia, lymphoma, multiple sclerosis, neurosyphilis (tabes dorsalis), syringomyelia, and cerebral palsy due to brain injury. In addition, ascending Guillain-Barré palsy should be distinguished from acute myelopathies (with chronic back pain), porphyria (with abdominal pain, seizures, and mental disorders), polyradiculitis in HIV-infected patients and patients with Lyme disease, as well as from symptoms of poisoning with organophosphorus compounds, thallium, arsenic, and hemlock poisoning.

Treatment of ascending paralysis

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

If Landry's ascending paralysis progresses rapidly, emergency medical care is required in an intensive care unit where there are facilities for the use (if necessary) of a ventilator.

The key treatment methods for Guillain-Barré syndrome are plasmapheresis or intravenous administration of immunoglobulins (human immunoglobulin), that is, immunotherapy is aimed at reducing the symptoms and complications of ascending paralysis.

Therapeutic plasmapheresis (blood filtration) is performed to remove antibodies attacking nerve cells from the bloodstream (five procedures over two weeks). Similarly, the introduction of immunoglobulins IgG into the blood neutralizes harmful antibodies and inflammation - Gabriglobin, Gamunex, Gamimune, Octagam, Flebogamma, Gammagard, etc. They are administered by infusion, the daily dose is calculated at 0.4 g per kilogram of body weight. The standard number of infusions is one for 5 days. Possible side effects of immunoglobulins include fever, headache, nausea, vomiting, allergic reactions and liver inflammation. It should be borne in mind that in case of impaired blood supply to the brain or heart, excess body weight and a decrease in the volume of circulating blood (hypovolemia), the introduction of immunoglobulin can lead to the formation of blood clots. Therefore, antithrombotic drugs (anticoagulants) are prescribed at the same time.

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

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

The drug Ipidacrine (Neuromidin), which inhibits cholinesterase and improves impulse transmission, 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 uterine muscles. It is not used in patients with angina pectoris, bronchial asthma and vestibular disorders.

In addition, when treating ascending paralysis, it is necessary to take B vitamins.

During the recovery period, physiotherapy treatment is indicated: hydrotherapy, electrophoresis, iontophoresis, UV irradiation, acupuncture, therapeutic massage.

Forecast

The speed and degree of recovery from ascending paralysis vary. And the prognosis for Guillain-Barré syndrome is determined by age: in patients over 40 years of age, treatment results may be lower than in younger patients.

Almost 85% of patients recover from the disease within a year; 5-10% have problems with movement after treatment. Due to complications and the initial severity of symptoms, about 5% of cases end in death.

Ascending Guillain-Barré paralysis may relapse (2-3% of cases), especially if previous infections have resulted in serious systemic complications.

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