Guillain-Barré syndrome: symptoms, causes and treatment

Guillain-Barré syndrome is a rare disease that destroys the myelin of peripheral nerves. of the organism and causes muscular and sensory alterations, generating a great functional disability in the person who suffers from it. It is a serious disorder that must be addressed urgently, since it can lead to respiratory complications that can put the patient's life at risk.

In this article we explain what this neurological disease consists of, what are its causes, signs and symptoms, how it is diagnosed and what is its treatment.

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Guillain-Barré syndrome: what it is and how it occurs

Guillain-Barré syndrome, or acute polyradiculoneuritis, is a rare neurological disease of autoimmune origin that It is characterized by causing rapid muscle weakening (starting distally and progressing proximally), accompanied by changes in sensation, such as pain or tingling sensations and loss of osteotendinous reflexes, which may also affect the respiratory bulbar musculature.

This disorder primarily affects the peripheral nervous system and It is the most common cause of acute generalized paralysis.. The damage occurs in the myelin sheaths of the nerves (which increase the speed of transmission of nerve impulses), and it is the patient's own immune system that causes it.

Guillain-Barré syndrome affects all races, sexes, and ages equally. Its incidence is 1 or 2 cases per 100,000 people. The course of the disease can be fulminant, with a rapid evolution that usually requires ventilatory assistance after a few days.

• You may be interested in: "Demyelinating polyneuropathies: what they are, types, symptoms and treatment"

Possible causes

Although the causes are still unknown, the most plausible hypotheses point to a viral or bacterial infectious origin, which could be generating an autoimmune response that triggers a reaction against the basic proteins of the nerves, giving rise to the process of demyelination.

Diagnosis

Guillain-Barré syndrome cannot be diagnosed with the administration of a single test. Its existence is usually suspected when the patient presents the Asbury and Cornblath diagnostic criteria: progressive weakness in more than one limb and universal osteotendinous areflexia.

On the other hand, there are another series of clinical features that support the diagnosis; the progression of the weakness, that the affectation is relatively symmetrical; mild sensory signs and symptoms present; that the patient presents an autonomic dysfunction (tachycardia, arterial hypertension or vasomotor signs); that there is involvement of the cranial nerves (with facial weakness in half of the cases); and the absence of fever.

Although the clinical picture can vary, Guillain-Barré syndrome is the most common current cause of symmetric weakness that develops within just a few hours. Progressive paralysis, respiratory insufficiency and cardiovascular complications will also determine the diagnosis.

Other clinical manifestations may vary from one patient to another, such as: having a fever at the beginning; severe sensory loss and pain; that disease progression ceases without recovery or with significant permanent sequelae; that the sphincters are affected; and there are lesions in the central nervous system.

Differential diagnosis should take into account the following disorders: motor neuron diseases (such as acute viral poliomyelitis, amyotrophic lateral sclerosis, etc.); polyneuropathies (for example, porphyria, other forms of Guillain-Barré syndrome, Lyme disease, etc.); disorders of neuromuscular transmission (such as autoimmune myasthenia gravis or botulism); and other muscular and metabolic disorders.

Clinical symptoms and signs

Initial symptoms in Guillain-Barré syndrome may involve abnormal sensations (paresthesias) that manifest themselves in various ways, first in one of the extremities and later in both, such as example: tingling, numbness, numbness, or a feeling of something walking under the skin (formication).

Muscle weakness is also present and usually starts in the lower limbs, later affecting other areas of the body. This weakness is sometimes progressive and affects the arms, legs, respiratory muscles, etc., configuring the typical clinical picture of Guillain-Barré syndrome. The cranial nerves are also affected in 25% of patients, with bilateral facial palsy being the most characteristic sign.

The disease follows a course that lasts between 3 and 6 months, evolving in several phases.: the phase of progression, stabilization and recovery or regression.

1. progression phase

In the progression stage, the person experiences the first signs and symptoms such as tingling and paresthesia in the feet and hands, followed by muscle weakness that can end in paralysis. Generally, it usually begins in the feet or legs and then gradually spreads to the rest of the body, causing facial or respiratory paralysis.

This first phase can last from a few hours to three or four weeks and, depending on the severity of the symptoms, may require urgent medical intervention, due to possible blockage of airways respiratory.

2. stabilization phase

This second stage, known as the stabilization phase, encompasses the end of disease progression and the onset of clinical recovery. In this phase, the signs and symptoms of Guillain-Barré syndrome usually stabilize; however, problems such as hypertension or hypotension, tachycardia and some complications such as pressure ulcers, blood clots or urinary infections may appear.

The duration of the stabilization phase is variable, and can be from a few days to several weeks, or even months. However, it should be noted that this stage may be absent during the course of the disease.

3. Regression or recovery phase

This last stage is between the beginning of recovery and the end of the disease. During the same, the symptoms gradually diminish. From this last phase, if neurological damage persists in the patient, it can already be considered as permanent sequelae..

This phase usually lasts approximately 4 weeks, although this time varies from one subject to another depending on the severity and extent of the neurological lesions, and can last for months.

Treatment

Guillain-Barré syndrome is quite likely to progress with rapid deteriorationTherefore, all patients suspected of having the disease must be hospitalized, and their respiratory function must be monitored. Similarly, if the patient has swallowing difficulties, they should be fed through a stomach tube.

In case the person presents respiratory paralysis, assistance through mechanical ventilation devices will be necessary. Management of respiratory function includes airway patency, the person's ability to cough and expectorate, the ability to swallow and the appearance of symptoms of hypoxemia (decreased oxygen in the blood) or hypercapnia (increased carbon dioxide in the blood).

The indicated treatment for this disorder includes, on the one hand, plasmapheresis, a procedure that consists of purifying the blood, this is, to extract a certain volume of blood plasma to eliminate particles and pathogens that intervene in the immune response pathological; and on the other hand, the intravenous administration of immunoglobulins, a treatment to replace a person's defenses when suffering from an infectious or autoimmune disease.

Bibliographic references:

• Hughes, R. A., & Cornblath, D. R. (2005). Guillain-barre syndrome. The Lancet, 366(9497), 1653-1666.
• Tellería-Díaz, A., & Calzada-Sierra, D. J. (2002). Guillain Barre syndrome. Rev Neurol, 34(10), 966-976.