Guillain-Barré syndrome is a rare disease that destroys myelin in peripheral nerves of the organism and causes muscular and sensitive alterations, generating in the person who suffers from it a great functional handicap. This is a serious disorder that must be treated urgently, as it can lead to respiratory complications that could endanger the patient’s life.
In this article, we explain what this neurological disease is, what are its causes, signs and symptoms, how it is diagnosed and what is its treatment.
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 is characterized by rapid muscle weakening (distal onset and proximal advance), accompanied by alterations in sensitivity, Such as pain or tingling sensations and loss of tendon reflexes, and may also affect the respiratory bulbar muscles.
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 rate of transmission of nerve impulses), and it is the patient’s immune system that causes it.
Guillain-Barré syndrome affects all races, genders and ages alike. Its incidence is 1 or 2 cases per 100,000 people. The course of the disease can be fulminant, with a rapid course that usually requires ventilatory support after a few days.
Although the causes are still unknown, the most plausible hypotheses point to an infectious origin of the viral or bacterial type, Which could be generators of an autoimmune response that triggers a reaction against basic nerve proteins, leading to the process of demyelination.
Guillain-Barré syndrome cannot be diagnosed with just one test. Its existence is often suspected when the patient presents the diagnostic criteria of Asbury and Cornblath: progressive weakness of more than one limb and universal tendon reflex.
On the other hand, there is another set of clinical features that support the diagnosis; progression of weakness, that the assignment is relatively symmetrical; that slight sensory signs and symptoms appear; that the patient has autonomic dysfunction (tachycardia, hypertension or vasomotor signs); that there is damage to 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 it is the most common current cause of symmetrical weakness that develops in just a few hours. Progressive paralysis, respiratory failure and cardiovascular complications also tilted the diagnosis.
Other clinical manifestations may vary from patient to patient, such as: having a fever initially; that it is a severe and painful sensory loss; that the progression of the disease ceases without cure or with significant permanent sequelae; that the sphincters are affected; I that there is damage to 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.); polyneuropathy (eg, porphyria, other forms of Guillain-Barré syndrome, Lyme disease, etc.); neuromuscular transmission disorders (such as autoimmune myasthenia gravis or botulism); and other muscle and metabolic disorders.
Symptoms and clinical signs
The first symptoms of 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: tingling, numbness, numbness, or feeling like something is walking under the skin (tingling).
Muscle weakness is also present and usually begins in the lower extremities, then affecting other areas of the body. This weakness is sometimes progressive and affects the arms, legs, respiratory muscles, etc., shaping the typical clinical picture of Guillain-Barré syndrome. The cranial pairs are also affected in 25% of patients, bilateral facial paresis being the most characteristic sign.
The disease follows a course that lasts between 3 and 6 months, progressing in several phases: The phase of progression, stabilization and recovery or regression.
1. Progression phase
In the progression phase, the person experiences the first signs and symptoms such as tingling and paresthesia in the feet and hands, Followed by muscle weakness which can lead to paralysis. It usually starts on 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 the possible blockage of the airways.
2. Stabilization phase
This second step, known as the stabilization phase, it includes the end of disease progression and the start of clinical recovery. At this point, the signs and symptoms of Guillain-Barré syndrome usually stabilize; however, problems such as hypertension or hypotension, tachycardia and certain complications such as pressure sores, blood clots or urinary tract infections can occur.
The duration of the stabilization phase is variable and can range 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 onset of healing and the end of the disease. During this time, the symptoms gradually diminish. From this last phase, if neurological damage persists in the patient, they can already be considered as permanent sequelae..
This phase usually lasts about 4 weeks, although this time varies from subject to subject depending on the severity and extent of neurological damage, and can last for months.
Guillain-Barré syndrome is quite likely to deteriorate quickly, So that all patients suspected of having the disease are hospitalized and their respiratory function monitored. Likewise, if the patient has difficulty swallowing, they should be fed through a gastric tube.
In case the person has respiratory paralysis, Assistance with mechanical ventilation devices will be necessary. Management of respiratory function includes patency of the airways, the person’s ability to cough and expectorate, the ability to swallow, and the onset of symptoms of hypoxemia (decreased oxygen in the blood) or hypercapnia (increased carbon dioxide in the blood).
The treatment indicated for this disorder includes, on the one hand, plasmapheresis, a procedure which consists in purifying the blood, that is to say, in extracting a certain volume of blood plasma to eliminate the particles and pathogens that participate in the pathological immune response; and on the other hand, the intravenous administration of immunoglobulins, treatment intended to replace the defenses of a person who suffers from an infectious or autoimmune disease.
- Hughes, RA and Cornblath, DR (2005). Guillain Barre syndrome. The Lancet, 366 (9497), 1653-1666.
- Tellería-Díaz, A., and Calçada-Serra, DJ (2002). Guillain Barre syndrome. Rev Neurol, 34 (10), 966-976.