Key points
Guillain-Barré syndrome is a complex and generalized disorder of the peripheral nervous system, which can also affect the CNS and respiratory muscles
Guillain-Barré syndrome: causes
Guillain-Barré syndrome is the expression of an "abnormal autoimmune response, probably triggered by bacterial infections (H. influenzae, C. jejuni, Mycoplasma) or viral (EBV, Cytomegalovirus, HIV I and II, Hepatitis A, B and C virus)
Guillain-Barré syndrome: symptoms
Guillain-Barré syndrome: diagnosis
A suspected Guillain-Barré syndrome is ascertained by spinal tap, antibody screening, spirometry and ECG
Guillain-Barré syndrome: therapy
Plasma exchange and intravenous IgG administration (possibly associated with steroid drugs) are the two first-line therapies for the treatment of Guillain-Barré syndrome.
Guillain-Barré syndrome
Guillain-Barré syndrome is a complex generalized affection of the peripheral nervous system, expression of an autoimmune disorder.
Guillain-Barré syndrome - less commonly called acute inflammatory poly-neuropathy - causes the progressive degeneration of neuronal axons, and is often accompanied by weakness, paraesthesia, progressive paralysis of the limbs and hyporeflexia (reduction of the ability to react to nerve stimuli caused by mechanical stress). Guillain-Barré syndrome can cause devastating damage to the body, especially when it also affects the autonomic nervous system and respiratory muscles.
Although the precise triggering cause has not yet been identified with certainty, it seems that Guillain-Barré syndrome is triggered by a "bacterial or viral infection. According to recent medical statistics, it seems that 75% of affected patients have a previous history of "infection, particularly of the gastrointestinal tract and respiratory tract.
The therapies of choice for Guillain-Barré syndrome are plasmapheresis and the administration of immunoglobulins: these strategies have proved extremely effective for the complete remission of the disease.
Incidence
From the data reported in the Medical Journal The Lancet, it appears that the Guillain-Barré syndrome occurs in Europe in 1.2-1.9 cases per 100,000 inhabitants. The incidence rate is higher in children between the ages of 15 and 35 and in adults between the ages of 50 and 75.
Causes
Guillain-Barré syndrome is the result of an autoimmune disorder: it occurs when the immune army mistakenly attacks a part of the nervous system, recognizing it as foreign and dangerous. As mentioned, the triggering factor has not been identified with absolute certainty. ; however, it appears that some infections may trigger a similar, abnormal and exaggerated autoimmune response.
The pathogenic microorganisms most involved in the syndrome appear to be bacteria or viruses:
- Campylobacter jejuni (infection most often diagnosed in patients with Guillain-Barré syndrome)
- Epstein Barr virus (EBV): is a virus involved in the onset of infectious mononucleosis and - at least so it seems - in the genesis of Burkitt's lymphoma, Hodgkin's lymphoma, multiple sclerosis and other epithelial tumors
- Cytomegalovirus: Viruses involved in common diseases, such as chicken pox, cold sores, St. Anthony's fire, genital herpes and mononucleosis
- Mycoplasma: etiological agents of primary atypical pneumonia, urethritis, neonatal meningitis
- Human Immunodeficiency Virus (HIV I and HIV II)
- Hepatitis A, B and C virus
- Haemophilus influenzae
- Influenza virus: In some cases, the subject exhibits all the signs and symptoms of Guillain-Barré syndrome AFTER a "flu virus infection" or following a flu shot. The incidence of Guillain-Barré syndrome post-influenza vaccination has grown enormously after the 1976-1977 swine flu pandemic. However, the risk current of developing the syndrome after immunization is extremely low (1 case per million vaccinations).
Probably, sarcoidosis, systemic lupus erythematosus and salmonellosis can also predispose the person to Guillain-Barré syndrome.
To understand...
How can infectious agents trigger the autoimmune response that characterizes Guillain-Barré syndrome?
It is hypothesized that the pathogens can become coated with some myelinated components, consequently activating a process of self-immunization against the myelin itself. The defense system, by mistakenly recognizing myelin antigens as foreign and potentially dangerous agents, triggers an exaggerated attack against both the infectious agent and the myelin of the peripheral nerves.
Symptoms
Guillain-Barré syndrome is responsible for one of the so-called "acute flaccid paralysis syndromes": the "uncontrolled production of antibodies in the peripheral nervous system causes progressive paralysis of the lower and upper limbs.
The Guillain-Barré syndrome has an acute course: the progression of the neurological deficit occurs over a few days, a maximum of several weeks. Subsequently, a "plateau" phase and a recovery stage follow.
The distinctive symptoms of Guillain-Barré syndrome are:
- Progressive weakness of the limbs (omnipresent symptom), up to the involvement of the muscles of the face (facial paralysis)
- Respiratory difficulties
- Dysarthria (speech disorders)
- Dysphagia (difficulty swallowing solid or liquid food)
- Autonomic (autonomic) dysfunction: altered heart rate, arrhythmias, altered blood pressure (orthostatic hypotension) and basal temperature
- Bladder dysfunction (uncommon)
- Neuropathic pain, especially in the limbs
- Respiratory failure due to paralysis of the diaphragm
- Hypoesthesia: reduced sensitivity of response to a stimulus
- Hypotonia: reduction in the nervous activity of an organ or tissue (muscle hypotonia)
- Abnormal eye movements (uncommon)
- Progressive paralysis of the limbs: a distinctive symptom of Guillain-Barré syndrome
- Paralysis of the respiratory muscles
- Paresthesia (change in sensation in the limbs or other parts of the body)
Complications
When the therapy is postponed, the symptoms of Guillain-Barré syndrome can be accentuated, and the patient's clinical picture can precipitate. The most common complications are:
- cardiac arrhythmia
- psychiatric disorders: anxiety, depression
- ileus (intestinal obstruction)
- respiratory failure (requires mechanical ventilation)
- hypotension / severe hypertension
- permanent paralysis: in hyperacute forms of Guillain-Barré syndrome, total paralysis occurs within 24 hours
- Severe urinary retention
- Thromboembolism
- Deep vein thrombosis
With modern therapeutic strategies (intensive supportive care) the prognosis is excellent in most patients. After therapy, it is estimated that 20% of Guillain-Barré syndrome patients retain a partial disability (constant weakness, impaired sensitivity), while 10% die from respiratory failure or pulmonary embolism. Death can also occur after a severe CNS alteration, or it can be the result of complications from prolonged intubation.
Rapid progression of symptoms, advanced age and long-term assisted ventilation greatly increase the risk of poor prognosis in patients with Guillain-Barré syndrome.
Guillain-Barré Syndrome: Diagnosis and Therapies "
