Guillain-Barré syndrome is a rare disease that destroys the myelin of the peripheral nerves of the body and causes muscular and sensory alterations, generating in the person who suffers it a great functional disability. 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 its causes are, the signs and symptoms, how it is diagnosed and what its treatment is.

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

Guillain-Barré syndrome, or acute polyradiculoneuritis, is a rare neurological disease of autoimmune origin, which is characterized by rapid muscle weakening (distal onset and proximal advancement), accompanied by alterations in sensation , such as pain or tingling sensations and loss of osteotendinous reflexes, and may also affect the respiratory bulbar muscles.

This disorder mainly affects the peripheral nervous system and is the most common cause of generalized acute paralysis . The damage occurs to the myelin sheaths of the nerves (which increase the speed of transmission of nerve impulses), and is caused by the patient’s own immune system.

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 rapid progression often requiring ventilatory support within a few days.

Possible causes

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

Diagnosis

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

On the other hand, there is another series of clinical features that support the diagnosis: the progression of the weakness, that the involvement is relatively symmetrical; that there are mild sensory signs and symptoms; that the patient presents an autonomic dysfunction (tachycardia, high blood pressure 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 may vary, Guillain-Barré syndrome is the most common current cause of symmetrical weakness that develops within a few hours . Progressive paralysis, respiratory failure and cardiovascular complications will also influence the diagnosis.

Other clinical manifestations may vary from patient to patient, such as: having a fever at the beginning; severe sensory loss and pain; cessation of disease progression without recovery or with significant permanent sequelae; sphincter involvement; and lesions in the central nervous system .

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

Clinical signs and symptoms

Initial symptoms in Guillain-Barré syndrome may involve abnormal sensations (paresthesias) that manifest themselves in various ways, first in one limb and later in both, for example: tingling, numbness, numbness or the sensation of something walking under the skin (formation) .

Muscle weakness is also present and usually begins in the lower limbs, then affects other areas of the body. This weakness is sometimes progressive and affects the arms, legs, respiratory muscles, etc., forming the typical clinical picture of Guillain-Barré syndrome. Cranial pairs are also affected in 25% of patients, with bilateral facial paresis 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 may end in paralysis. It usually starts 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 airway blockage.

2. Stabilisation phase

This second stage, known as the stabilization phase, comprises the end of the disease progression and the beginning 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 occur.

The duration of the stabilization phase is variable, ranging 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 this stage, the symptoms gradually decrease. From this last phase onwards, if neurological damage persists in the patient, it can be considered as a permanent consequence .

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 injuries, and may last for months.

Treatment

It is quite likely that Guillain-Barré syndrome will be cured by rapid deterioration , so all patients suspected of having the disease should be hospitalized, and their respiratory function monitored. Similarly, if the patient has swallowing difficulties, he or she should be fed through a stomach tube.

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

The indicated treatment for this disorder includes, on the one hand, plasmapheresis, a procedure that consists of purifying the blood, that is, removing a determined volume of blood plasma to eliminate particles and pathogens that intervene in the pathological immune response; and on the other hand, the intravenous administration of immunoglobulins, a treatment to replace a person’s defenses when he or she suffers 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-Barré syndrome. Rev Neurol, 34(10), 966 – 976.