Guillain-Barre Syndrome – Causes, Symptoms,Diagnosis, Treatment And Prognosis
Guillain-Barre syndrome is a unique pathological condition that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes, being the most important cause of acute flaccid paralysis. Also known as polyradiculonevritis, the disease can rapidly and severely damage, within a few days, a previously healthy patient and can evolve either to death or to complete remission.
The annual incidence of the disease is 2 cases per 100,000 inhabitants. Affects all age groups, with an average onset at the age of 40 years and a slight preponderance in males. Although seasonal variations are not evident, however, some studies shown a high frequency of Guillian-Barre syndrom in fall and winter.
Guillain-Barre Syndrome Causes
In 50% -60% of cases, Guillain-Barre syndrome debuted after respiratory or gastrointestinal infections with viruses and bacteria or vaccinations to prevent them.
Among systemic infections that are associated with Guillain-Barre syndrome, have been reported infections with adenoviruses, hepatitis B virus, hepers simplex virus, varicella-zoster virus and cytomegal virus. The association between acquired immunodeficiency syndrome (AIDS) and Guillain-Barre syndrome is well known.
Guillain-Barre syndrome is most commonly associated with bacterial infection with Campylobacter jejuni. Syndrome occurs in 10% -50% cases of infections caused by this bacterium. Polyradiculonevritis seems to be caused by an immune cross-response against Campylobacter jejuni, because it was observed that the disease occurs at 1-3 weeks after initial infection.
The disease is considered an acquired immunological disorder, in which in the perivascular infiltrates with monocytes and macrophages are associated with neuronal segmental demyelination. Immunological studies have shown that in the disease development are involved both cellular and humoral immunity.
After an infectious process, most commonly with Campylobacter jejuni, is induced the production of antibodies that cross react with gangliosides and glycolipide such as GM1 and GD1b, which are components of the myelin.
Guillain-Barre Syndrome Symptoms
Paralysis represent the predominant symptom of the disease. At the onset, the patient is not feverish, but is complaining by paresthesias and spontaneous muscle weakness in the limbs affected by the disease.
Paralysis are relatively symmetrical and rapidly progressive, affect manly proximal muscles of the limb muscles and can reach a maximum intensity within a few days to 4 weeks. Onset is usually in the legs and may take an ascending evolution, to the upper limbs, patient presenting a peripheral quadriplegia with muscle weakness and diminished reflexes. If paralysis are persistent, then muscular atrophies will develop. Trunk and neck muscles, intercostal nerves and cranial nerves are affected later in the evolution of the disease.
Respiratory crisis represents a major life threatening complication and is caused by respiratory muscles paralysis. Respiratory crisis is an emergency and require assisted mechanical ventilation. As disease progression is faster, within hours or days, the faster respiratory crisis can install. Respiratory impairment is present in 50% of patients with Guillain-Barre syndrome, and 25% of them developed respiratory failure.
Were reported cases with onset in the upper limbs, in the neck muscles, with or without extension to the legs, cases in which respiratory muscles were constantly affected.
Autonomic nervous system impairment is present in 20% of patients and represents an important cause of death. Autonomic nervous system impairment may be mild, causing variations in blood pressure and in heart rate or may be severe, causing hypotension, supraventricular tachycardia, cardiac arrest or sudden cardiac death. Urinary retention is present in 15% of patients with Guillain-Barre syndrome. Hyponatremia and transient diabetes insipidus can occur through inappropriate secretion of antidiuretic hormone. Other autonomic nervous system impairments are facial redness, sudoration abnormalities (anhidrosis or diaphoresis).
Cranial nerves are commonly affected in this syndrome. Typically occurs facial diplegia (bilateral facial nerve paralysis), which is less common other neuropathies. Bilateral facial paralysis occurs in more than 50% of patients and has a transient character. Sometimes occur bulbar paralysis with glosopharyngeal and vagus nerve impairment.
Oculomotor nerves are affected in less than 10% of cases, oftalmo-paresis being the most common ocular sign seen in Guillain-Barre syndrome. It may be complete or incomplete and it may be accompanied by pupillary changes. Cranial nerve VI is most commonly affected, alone or associated cranial nerves IV and III impairment. It is assumed that oculomotor disturbances occur due to a type of IgG antibody directed against GQ1b gangliosides. Unilateral or bilateral ptosis may be present. The main pupillary disturbances are the reduction of pupillary reaction to light and accommodation disorders.
Paiplar edema was reported in a small percentage of cases and may be correlate with increased protein concentration in the cerebrospinal fluid.
Optic neuritis was also detected and is most often bilateral. Vision loss may be mild or severe, and in cases with favorable evolution, vision recovery may be partial or complete.
Guillain-Barre Syndrome Diagnosis
Guillain-Barre syndrome is diagnosed based on clinical and laboratory examinations, the most important investigations are represented by cerbrospinal fluid examination and electrophysiological study.
Lumbar puncture reveals a cerebrospinal fluid with normal pressure and acellular in 90% of cases. The most important anomaly is represented by an increased protein concentration over 400 mg / dl, but without a high cellularity. This anomaly is characteristic for the disease and is called albuminocytological dissociation, being present in over 90% of patients.
Electrophysiological tests of peripheral nerves and muscles are often normal, demonstrating the proximal nature of the disease. The most frequent modification is represented by decreased nerve conduction velocity in proximal nerve segments, with over 60% of normal nerve conduction velocity.
Serum autoantibodies are not routinely used in the diagnosis of Guillain-Barre syndrome, but may be helpful in patients with a questionable diagnosis. Glycolipids antibodies are present in 60-70% of patients with Guillain-Barre syndrome during the acute phase.
Guillain-Barre Syndrome Treatment
ECG and blood pressure monitoring are required and sometimes a pacemaker implantation is required to prevent the occurrence of fatal arrhythmias. Respiratory dysfunction can lead to respiratory failure and respiratory infections and often require intubation, assisted ventilation and sustained antibiotic treatment of respiratory infections. Urinary tract infections require also supported antibiotic treatment.
Periodic repositioning of the patient is recommended in order to prevent pressure sores and nerve paresis by compression. Passive movements of the limbs and joints should be made, in order to decrease the risk of thrombophlebitis. Low-doses of heparin are recommended to prevent deep vein thrombosis and pulmonary embolism.
Physiokinetotherapy should be started early in the recovery period in order to reduce functional deficits, impairments and disabilities resulting from Guillain-Barre syndrome.
Plasmapheresis has beneficial effects, especially if is performed in the first 14 days of onset. Are performed 4-6 procedures. Potential complications are the emergence of bleeding (decreased fibrinogen), cardiac arrhythmias and hypotension.
Corticosteroid therapy has not proved its effectiveness in the treatment of Guillain-Barre syndrome.
Guillain-Barre Syndrome Prognosis
Despite sustained therapy, between 3% -8% of patients die in the acute phase of disease by various complications such as respiratory failure or pulmonary embolism. Between 5% -10% of patients remain with motor disabilities, paresis or sensitive disorders. Between 10% -15% of patients present a complete recovery. Disease recurrence was detected in 5% -33% of patients.