Is the Acute Generalized Paralysis Associated with Guillain-Barré Syndrome Related to COVID-19 or Vaccines? Two cases suggest this.
After the almost complete disappearance of Poliomyelitis is this Guillain-Barré-Syndrome (GBS) is the most common cause of acute generalized paralysis in our latitudes. With an incidence of 1-2 cases per 100,000 per year worldwide, the disease is very rare.
The clinical picture has been known since the middle of the last century. The name comes from the French neurologists G. Guillain and JA Barré, who described this disease in 1916 with its typical clinical and liquor biochemical findings. Incidence increases by 20% for every 10-year increase in age, and men are more at risk than women.
Bacterial infection as a trigger
GBS is a rapidly progressive peripheral neuropathy and Main cause for flaccid paralysis after an acute infection. Two-thirds of patients have symptoms of a previous infection and the most commonly identified cause is Campylobacter jejuni. These bacteria are caused by contaminated food, particularly undercooked poultry and unpasteurized cow’s milk. That too Zika-Virus can cause many patients neurological symptoms such as GBS develop.
The exact pathomechanisms are unclear. GBS is probably looking for a C. jejuni-Infection an autoantibody-mediated disease. Very few patients with a microbial infection develop GBS, suggesting the involvement of host factors that trigger this autoimmunity. Genetic susceptibility may be a predisposing factor for GBS, although the genetic factors affecting microbial-host interactions are poorly understood. The host complement system plays a crucial role in the pathogenetic mechanism of GBS.
In the past ten years, mannose-binding lectin has been im center of interestas it has the ability to turn on the complement pathway through enzymatic regulation, thus affecting disease susceptibility and severity.
Variable appearance
The clinical presentation of GBS varies, with weakness usually beginning in the legs, while earlier involvement of the arms or facial muscles is reported in about 10% of patients. In 10-30% of patients, respiratory muscle weakness may require ventilator support. Other features include facial and ocular nerve involvement, areflexia or decreased reflexes with paresthesia and pain during the acute phase of the disease.
The autonomic dysfunction in GBS occurs predominantly in the acute phase of the disease, but can also manifest in the convalescent phase. The exact mechanism is still unknown but likely involves dysfunction of the sympathetic and parasympathetic systems. Such autonomic dysfunction is observed in 70% of cases. It includes features like Tachycardia, Bradycardiafacial flushing, hypertension alternating with hypotensionorthostatic hypotension, Anhydrosis or diaphoresis and urinary retention. Gastrointestinal autonomic manifestation includes diarrhea or constipation. Severe autonomic dysfunction is an important factor to recognize and treat appropriately, as it is associated with a 5-7% sudden death rate.
The prognosis is determined by the form and severity of GBS and ranges from patients with complete recovery to patients who are no longer able to walk 6 months after the onset of the disease to courses with a fatal outcome.
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Characteristics |
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disease name |
Guillain-Barré-Syndrome |
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Other names |
Idiopathic polyradiculoneuritis Landry-Guillain-Barré-Strohl-Syndrom Acute inflammatory demyelinating polyneuropathy Chronic inflammatory demyelinating polyneuropathy |
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frequency |
1–2/100.000 |
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disturbed function |
Neurological autoimmune reaction to pathogens (C. jejuni, Zika, EBV, herpes) or vaccinations (including COVID-19) |
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therapy |
Corticosteroids immunoglobulins Plasmapharese |
No curative therapies available
Standard therapy is the high-dose administration of iv immunoglobulins (IVIG), plasmapheresis or immune adsorption. However, therapy with a standard dose (2 g/kg) of intravenous immunoglobulins in a proportion of patients with severe Guillain-Barré syndrome insufficiently effective.
immunoglobulins are endogenous proteins that can dock onto foreign bodies with one end. Elements of the innate immune system are modulated with the other end of the molecule. The immunoglobulins used for therapy are obtained from the blood plasma of 5,000 to 10,000 healthy donors. One and the same preparation is therefore suitable for the treatment of different autoimmune diseases.
A randomized Study with 93 subjects in the Netherlands in patients with Guillain-Barré syndrome and poor prognosis showed no therapeutic benefit of a second intravenous administration of high-dose immunoglobulins. Therefore, a second course of treatment with immunoglobulins is not recommended.
Guidelines speak plain language
The guideline of the German Society for Neurology includes the following core statements:
- A Cochrane-Review confirmed that Glucocorticoid in monotherapy have no influence on the regression of Guillain-Barré syndrome or its long-term course. They can even be a hindrance to recovery.
- One big study with 172 participants for subcutaneous immunoglobulin administration (ScIG), which allows the patient more self-determination, showed a positive effect of two doses of ScIG (0.2 and 0.4 g/kg) of a 20% IG solution compared to placebo in the Maintenance therapy of GBS
- A Cochrane-Review from 2017 confirmed the lack of evidence of efficacy for immunosuppressants (other than glucocorticoids) in GBS. Evidence of the lack of effect is available for methotrexate and Interferon-beta-1a before.
- IVIG and Plasmapherese are equivalent in the treatment of acute GBS and better than placebo. One of the procedures is intended to be used for moderate to severe GBS.
GBS bei COVID-19
The realization that vaccination can lead to neurological or immunological side effects is not new. However, almost all rare diseases are genetic. The occurrence of GBS after a COVID-19 vaccination is therefore a rarity in several respects.
But the disease could also SARS-CoV-2 self-triggered, as an international cohort study shows. Since the beginning of Pandemic identified over 90 GBS patients possibly related to COVID-19 reported. Patients with confirmed or probable SARS-CoV-2 infection commonly had sensorimotor variant (8/11, 73%) and facial nerve palsy (7/11, 64%). The median time from onset of infection to neurological symptoms was 16 days. Patients with corona infection presented consistent neurological features similar to those previously described in other postviral GBS patients.
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The immune system forms antibodies against components of bacteria or viruses, which also recognize and attack the myelin sheaths of peripheral nerve fibers. The neurological symptoms are then the result of demyelization of the axons, which, however, is reversible. “Although rare, this case report suggests that the physician should remain vigilant after COVID-19 vaccination for GBS,” Anjum et al. in a casuistic. In another casuistic by Lanman et. the symptoms appeared three days after a single vaccination.
Image source: Steven HWG, unsplash