Guillain-Barré syndrome symptoms, causes and consequences

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Egbert Haynes
Guillain-Barré syndrome symptoms, causes and consequences

The Guillain Barre syndrome (GBS) is an autoimmune process in which the body creates antibodies that attack the components of the peripheral nerves (Peña et al., 2014). It is one of the most common acquired polyneuropathies (KopyKo & Kowalski, 2014). Different studies show that it is the first cause of extensive acute paralysis in developed countries since the eradication of poliomyelitis (Ritzenthaler et al., 2014).

It seems that this pathology is the result of a process mediated by the immune system that, on many occasions, appears after an episode of an infectious type caused by viruses, and that essentially affects the motor neurons (Janeiro et al., 2010).

Guillain-Barré syndrome virus

This type of syndrome is characterized by an ascending paralysis or flaccid weakness that begins in the lower limbs and is symmetrical and areflexic; it can also be associated with sensory symptoms and autonomic alterations (Vázquez-López et al., 2012).

Due to the fact that it is a pathology of an evolutionary or progressive type that can leave sequelae, a thorough and repeated examination is essential to confirm the diagnosis and control the possible complications derived from the development of acute respiratory insufficiency (Ritzenthaler et al.).

Article index

  • 1 Prevalence
  • 2 Symptoms
    • 2.1 Extension phase
    • 2.2 Plateau phase
    • 2.3 Recovery phase
  • 3 Causes and pathophysiology
  • 4 Diagnosis
  • 5 Consequences and possible complications
  • 6 Treatment
    • 6.1 Plasmapheresis
    • 6.2 Immunoglobulin therapy
    • 6.3 Steroid hormones
    • 6.4 Assisted breathing
    • 6.5 Physical intervention
    • 6.6 Early rehabilitation
    • 6.7 Physiotherapeutic intervention
  • 7 Conclusions
  • 8 References

Prevalence

Guillain-Barré syndrome (GBS) is considered a rare or rare disease. Despite intensive treatments, its mortality ranges from 4% to 15% (KopyKo & Kowalski, 2014).

In western countries its incidence is estimated at approximately between 0, 81 and 1.89 cases per 100,000 inhabitants per year (Ritzenthaler et al., 2014)

Statistical data show that this disease can appear at any stage of life and affects men and women proportionally (KopyKo & Kowalski, 20014).

However, there is evidence about the highest proportion of the disease in men, these being 1.5 times more prone to its condition (Peña et al., 2014). In addition, it seems that the risk of suffering from Guillain-Barré syndrome increases with age, increasing its incidence after 50 years to 1.7-3.3 cases per 100,000 inhabitants per year (Peña et al., 2014).

On the other hand, in the case of children, its incidence has been estimated at 0.6-2.4 per 100,000 cases..

Symptoms

It is a progressive disease that affects the peripheral nervous system that usually presents three phases or stages: an extension phase, a plateau phase and a recovery phase (Ritzenthaler et al., 2014)

Extension phase

The first symptoms or signs of this pathology are manifested with the presence of different degrees of weakness or paralysis, or of tingling sensations in the lower extremities that will progressively expand towards the arms and torso (National Institute of Neurological Disorders and Stroke, 2014).

Symptoms are likely to increase in severity until limbs and muscles become nonfunctional and severe paralysis occurs. This paralysis can cause significant problems in maintaining breathing, blood pressure and heart rhythm, even requiring assisted respiration (National Institute of Neurological Disorders and Stroke, 2014).

Plateau phase

Normally, in the first two weeks of appearance of the first symptoms, a significant weakness is usually reached. In the third week, approximately 90% of patients are in the phase of greatest weakness (National Institute of Neurological Disorders and Stroke, 2014).

Thus, in 80% paresthesias and painful processes or areflexia are already present, also in 80% areflexia is generalized, giving a loss of gait in 75% of patients. In addition, 30% of cases progress to heart failure (Ritzenthaler et al., 2014)

Recovery phase

This increase in symptoms is usually followed by a remission phase lasting 6 to 14 months (KopyKo & Kowalski, 20014).

In the case of motor reacquisition, most individuals do not recover from paralysis processes until approximately 6 months later. In addition, approximately 10% may have residual symptoms up to 3 years after resolution of the episode (Ritzenthaler et al., 2014)

On the other hand, relapses do not usually occur frequently, appearing in 2-5% of cases. Although fluctuations may appear after the start of treatment (Ritzenthaler et al., 2014).

Most patients recover, including the most severe cases of Guillain-Barré syndrome, although some continue to have a certain degree of weakness (National Institute of Neurological Disorders and Stroke, 2014).

Causes and pathophysiology

The exact causes of the factors that trigger Guillain-Barré syndrome are not known. However, various lines of research propose that different infectious or viral agents can trigger an abnormal immune response (Janeiro et al., 2010).

In many cases it is considered a postinfectious syndrome. A history of digestive and respiratory infections or gripla syndromes is usually described in the patient's medical history. The main triggering agents are bacterial (Campylobacter jejuni, Mycoplasma pneumoniae, Heamophilus influenzae), viral (cytomegalovirus, Epstein-Barr virus) or human immunodeficiency virus (Ritzenthaler et al., 2014)

However, it is known from the pathophysiological mechanisms that the body's immune system begins to destroy the axonal myelin sheaths of the peripheral nerves..

The involvement of the nerves will impede the transmission of the signal, so the muscles begin to lose their ability to respond and also, fewer sensory signals will be received, making it difficult in many cases to perceive textures, heat, pain, etc. (National Institute of Neurological Disorders and Stroke, 2014).

Diagnosis

The signs and symptoms of the syndrome can be quite varied, so doctors may find it difficult to diagnose Guillain-Barré syndrome in its early stages (National Institute of Neurological Disorders and Stroke, 2014).

For example, doctors will look at whether symptoms appear on both sides of the body (most common in Guillain-Barré syndrome) and the speed with which symptoms appear (in other disorders, muscle weakness can progress over months. instead of days or weeks) (National Institute of Neurological Disorders and Stroke, 2014).

Therefore, the diagnosis is mainly clinical and complementary tests are performed for differential diagnosis (Ritzenthaler et al., 2014). The following tests are commonly used:

  • Electromyograms: used to study nerve conduction velocity since demyelination slows down these signals.
  • Lumbar puncture: it is used to analyze the cerebrospinal fluid since in patients with Guillain-Barré syndrome it contains more proteins than normal.

Consequences and possible complications

Most complications will derive from the presence of muscle paralysis and nerve conduction deficiency. They may appear (Ritzenthaler et al., 2014):

  • Severe respiratory insufficiency: it is one of the main causes of mortality. Its appearance requires the use of mechanical ventilation. Normally the first signs that appear are of the type orthopnea, tachypnea, polypnea, sensation of chest pressure or difficulty speaking. Control of respiratory function is vital for patient survival.
  • Bulbar involvement: the main complications that occur are of the type of aspiration, risk of lung disease, respiratory failure and ateclectasis.
  • Dysautomy: the affectation of the autonomic nervous system will cause heart rhythm disorders, tension lability, urinary retention, etc..
  • Pains: They occur in most patients and are usually derived from paresthesias and dysesthesias in the extremities. Generally, pain is usually correlated with the degree of motor impairment.
  • Venous thromboembolic disease: prolonged paralysis of the individual will increase the risk of suffering from venous thrombosis or pulmonary embolisms.

In addition to these eminently medical complications, we will have to consider the possible neuropsychological sequelae.

It is a progressive disease that fundamentally affects the mobility of the individual, so suffering a process of progressive paralysis will have important repercussions on the quality of life of the patient.

The limitation of gait, movements and even dependence on assisted ventilation will drastically limit the work, daily and even personal activities of the patient. Generally, there is also a decrease in social interactions due to functional limitations.

The impact of all the symptoms can also interfere with normal cognitive functioning, causing difficulties in concentration, attention, decision-making or slight alterations in memory processes..

Treatment

The National Institute of Neurological Disorders and Stroke (2014), highlights that a specific cure for Guillain-Barré syndrome has not currently been identified. However, there are different therapeutic interventions aimed at reducing the severity of the symptoms that occur and promoting the speed of recovery in these patients..

The specific treatment of Guillain-Barré syndrome is based on plasmapheresis or polyvalent immunoglobulins. However, treatment should be based primarily on prevention and symptomatic treatment of complications (Ritzenthaler et al., 2014)

Therefore, there are different approaches in the treatment of the different complications derived from the suffering of Guillain-Barré syndrome (National Institute of Neurological Disorders and Stroke, 2014):

Plasmapheresis

It is a method in which all the blood reserves in the body are extracted and processed by separating the white and red blood cells from the blood plasma. Once the plasma has been removed, the blood cells are reintroduced into the patient.

Although the exact mechanisms are not known, these types of techniques reduce the severity and duration of the Guillain-Barré syndrome episode..

Immunoglobulin therapy

In this type of therapy, specialists administer intravenous injections of immunoglobulins; in small doses the body uses this protein to attack invading organisms. 

Steroid hormones

The use of these hormones has also been tested to reduce the severity of episodes, however detrimental effects on the disease have been identified.

Assisted breathing

In many cases, the presence of respiratory failure may require the use of a respirator, heart rate monitors and other elements to control and monitor body functions..

Physical intervention

Even before recovery begins, caregivers are instructed to manually move their limbs to help keep muscles flexible and strong..

Early rehabilitation

Early and intensive rehabilitation appears to be effective for motor recovery and residual fatigue. Respiratory physiotherapy, with secretion elimination techniques, is of special interest in preventing the accumulation of bronchial secretions and pulmonary superinfections (Ritzenthaler et al., 2014).

Physiotherapeutic intervention

As the patient begins to regain control of the extremities, physical therapy with specialists begins with the aim of recovering motor functions and alleviating the symptoms derived from paresthesia and paralysis..

Conclusions.

Guillain-Barré syndrome is a rare disease that usually has a good prognosis with intensive treatment, with an estimated mortality of 10%.

On the other hand, the prognosis for motor recovery is also favorable. However, within a period of 5 years, patients may maintain different sequelae such as pain, bulbar symptoms or enphincteric disorders..

Due to the risk of suffering from heart failure, it is a medical emergency that must be carefully controlled to reach the recovery phase in the shortest period of time possible..

References

  1. Janeiro, P., Gomez, S., Silva, R., Brito, M., & Calado, E. (2010). Guillain-Barré syndrome after chickenpox. Rev Neurol, 764-5.
  2. Kopytko, D., & Kowalski, P. M. (2014). Guillain-Barré syndrome- Literature overview. Annals of Medicine, 158-161.
  3. Peña, L., Moreno, C., & Gutierrez-Alvarez, A. (2015). Pain management in Guillain-Barré Syndrome. Systematic review. Rev Neurol, 30
    (7), 433-438.
  4. Ritzenthaler, T., Sharshar, T., & Orlijowski, T. (2014). Guillain Barre syndrome. EMC-Anesthesia-Resuscitation, 40(4), 1-8.

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