He 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 frequent acquired polyneuropathies (KopyKo & Kowalski, 2014).
Different studies show that this is the first cause of extensive acute paralysis in the cases developed since the eradication of poliomyelitis (Ritzenthaler et al., 2014).
It appears that this pathology is the result of a process mediated by the immune system which, in many cases, appears after an episode of virus infectious type, and which essentially affects the motoneurons (January et al., 2010).
This type of syndrome is characterized by ascending flaccid paralysis or weakness that begins in the lower limbs and is symmetrical and And may also be associated with sensory symptoms and autonomic alterations (Vázquez-López et al., 2012).
Because it is an evolutionary or progressive condition that can have sequelae, a thorough and repeated examination is essential to confirm the diagnosis and To control the possible complications resulting from the development of acute respiratory insufficiencies (Ritzenthaler et al.).
Prevalence of Guillain-Barré syndrome
Guillain-Barré disease (GBS) is considered a rare or rare disease. In spite of intensive treatments, GBS mortality oscillates Between 4% and 15% KopyKo & Kowalski, 2014).
In Western countries, their incidence is estimated at approximately 0.81 to 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 in a proportional way (Kopyko & Kowalski, 20014).
However, there is evidence of the highest proportion of the disease in men, being 1, 5 times more likely to suffer from it (Peña et al. Al., 2014). In addition, it appears that the risk of GBS 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. Of these, between 70% and 80% of those affected Suffered infectious processes before the onset of the syndrome (January et al., 2010).
Definition and symptoms of Guillain-Barré syndrome
It is a progressive disease that affects the peripheral nervous system that usually has three phases or stages: an extension phase, a phase of Plateau and a recovery phase (Ritzenthaler et al., 2014)
Extension Phase
The First symptoms or signs Of this pathology are manifested by the presence of different degrees of weakness or paralysis, or Tingling sensations in the lower extremities that will progressively expand into the arms and torso (National Institute of Neurological Disorders and Stroke, 2014).
Symptoms are likely to increase in severity until limbs and muscles are not functional and there is a Severe paralysis . This paralysis can cause major problems in maintaining breathing, blood pressure and Heart rate, requiring assisted breathing (National Institute of Neurological Disorders and Stroke, 2014).
Plateau phase
Usually, in the first two weeks of the appearance of the first symptoms, a significant weakness is usually already 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, 80% of the patients are already present with paresthesias and processes of pain or orthodontics. In 80% of cases, the reflex is usually with a loss of Gait in 75% of patients. In addition, 30% of cases progress to cardiac failure (Ritzenthaler et al., 2014)
Recovery Phase
This increase in symptoms is usually followed by a remission phase lasting from 6 to 14 months (KopyKo & Kowalski, 20014).
In the case of motor reacquisition, most individuals do not recover from the paralysis processes until about 6 months later. In addition, approximately 10% may present 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 occur after Initiation of treatment (Ritzenthaler et al., 2014).
Most patients recover, including the more 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 GBS are unknown. However, several lines of research propose that different agents Infectious or viral infections can trigger an abnormal immune response (January et al., 2010).
In many cases a post-infectious syndrome is considered. The patient's clinical history usually describes a history of digestive infections, Respiratory or gripla syndromes. The major deoxygenating agents are bacterial (Campylobacter jejuni, Mycoplasma pneumoniae, Heamophilus Influenzae), viral (cytomegalovirus, Epstein-Barr virus) or human immunodeficiency virus (Ritzenthaler et al., 2014)
However, from the pathophysiological mechanisms it is known that the body's immune system begins to destroy the axonal covers of myelin Of the peripheral nerves.
The involvement of the nerves will prevent signal transmission so that the muscles begin to lose their ability to Response, and in addition, less sensory signals will be received, which in many cases diffuse the perception of 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 the syndrome. Guillain-Barré in its early stages (National Institute of Neurological Disorders and Stroke, 2014).
For example, doctors will look for symptoms on both sides of the body (most common in Guillain-Barré syndrome) and speed with The symptoms appear (in other disorders, muscle weakness can progress through months instead of days or weeks) National Institute of Neurological Disorders and Stroke, 2014).
Therefore, the diagnosis is mainly clinical and the complementary tests are performed for the differential diagnosis (Ritzenthaler et al. Al., 2014). The following tests are usually used:
- Electromyograms : Are used for the study of nerve conduction velocity since demyelination slows down these signals.
- Lumbar puncture : It is used to analyze the cerebrospinal fluid since in patients with GBS it contains more proteins than normal.
Consequences and possible complications
The majority of complications are due to the presence of musculoskeletal paralysis and nerve conduction deficiency. They may appear (Ritzenthaler Et al., 2014):
- Severe respiratory insufficiency : Is one of the main causes of mortality. Its appearance requires the use of mechanical ventilation. Usually the first signs that appear are of the type orthopnea, tachypnea, polypnea, sensation of chest pressure or difficulty to speak. He Control of respiratory function is vital for patient survival.
- Bulbar affectation : The main complications that appear are of the type of brocoaspiración, risk of pneumopathy, insufficiency Respiratory and atelectasis.
- Dysautonomia : The effect of autonomic nervous system Will cause heart rhythm disorders, tension lability, urinary retention, etc.
- Dolores : They occur in the majority of the patients and are usually derived from the Paraesthesia And distended limbs. Generally, the Pain is often correlated with the degree of motor involvement.
- Venous thromboembolic disease : Prolonged paralysis of the individual will increase the risk of thrombosis Venous or pulmonary embolism.
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 that undergoing a process of progressive paralysis will Have important repercussions on the patient's quality of life.
Limitation of walking, movements and even dependence on assisted breathing will drastically limit daily work activities And even patient's personal. Generally, there is also a decrease in social interactions due to the limitations Functional.
The impact of all symptoms can also interfere with normal cognitive functioning, producing difficulties of concentration, attention, Of decisions or slight alterations of memory processes.
Treatment
He National Institute of Neurological Disorders and Stroke (2014), emphasizes that there is currently no specific cure for Guillain-Barré. However, there are different therapeutic interventions aimed at reducing the severity of the symptoms presented and promoting the Speed of recovery of these patients.
The specific treatment of GBS is based on plasmapheresis or polyvalent immunoglobulins. However, treatment should be based primarily on In the prevention and symptomatic treatment of complications (Ritzenthaler et al., 2014)
Therefore, different approaches exist in the treatment of the different complications derived from the disease of the GBS (National Institute of Neurological Disorders and Stroke, 2014):
- Plasmapheresis: It is a method in which all the blood reserves of the organism are extracted and processed by separating the White blood cells Y Red Of the blood plasma. Once the plasma (Which may contain elements of the immune system that damage myelin), become Introduce the blood cells into the patient. Although exact mechanisms are not known, these techniques reduce the severity and duration of Episode of Guillain-Barré Syndrome.
- Immunoglobulin therapy : In this type of therapy, specialists administer intravenous injections of Immunoglobulins (In small Dose the body uses this protein to attack the invading organisms) It has been proven that the administration of high doses, can reduce the Destruction that causes the immune system.
- Steroid hormones : The use of these lasts has also been tried to reduce the severity of the episodes, however they have been identified Detrimental effects on the disease.
- Breathing assisted : In many cases the presence of respiratory insufficiency may require the use of a respirator, rhythm monitors Cardiac and other elements for the control and monitoring of bodily functions.
- Physical intervention : Even before recovery begins, caregivers are instructed to Manually move the limbs of patients to help keep muscles flexible and strong.
- Early rehabilitation : Early and intensive rehabilitation seems to be effective for motor recovery and residual fatigue. Physiotherapy Respiratory secretion techniques, is of particular 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 limbs, physical therapy begins with Specialists with the aim of recovering the motor functions and alleviating the symptoms derived from paraesthesia and paralysis.
CONCLUSIONS
Guillain-Barré syndrome is a rare disease that usually has a good prognosis with intensive treatment, being estimated the Mortality by 10%.
On the other hand, the prognosis of motor recovery is also favorable. However, within 5 years, patients can keep different Sequelae such as pain, bulbaric symptoms or enfinterian disorders.
Due to the risk of suffering from heart failure, it is a medical emergency that must be carefully controlled in order to achieve the Recovery phase in the shortest time possible.
References
- January, P., Gomez, S., Silva, R., Brito, M., & Calado, E. (2010). Guillain-Barré syndrome after chickenpox. Rev Neurol , 764-5.
- Kopytko, D., & Kowalski, P. M. (2014). Guillain-Barré syndrome- Literature overview. Annals of Medicine , 158-161.
- Peña, L., Moreno, C., & Gutierrez-Alvarez, A. (2015). Management of the dolphin in Guillain-Barré Syndrome. Systematic review. Rev Neurol, 30
(7), 433-438. - Ritzenthaler, T., Sharshar, T., & Orlijowski, T. (2014). Guillain Barre syndrome. EMC-Anesthesia-Reanimation, 40 (4), 1-8.