Guillain-Barré syndrome is an acute, usually very rapidly progressive, but self-limiting inflammatory polyneuropathy and is characterized by muscle weakness and mild distal sensory loss. The cause is thought to be an autoimmune process. The diagnosis is made clinically. Treatment includes i.v. immunoglobulins, plasma exchange and, in severe cases, mechanical ventilation.
Guillain-barré syndromeG61.0
DefinitionThis section has been translated automatically.
EtiopathogenesisThis section has been translated automatically.
Probably autoimmunological genesis. Symptomatology 5 days to 3 weeks after a banal infection, also after vaccinations. Infection is the trigger in > 50% of patients; common pathogens include.
- Campylobacter jejuni
- Enteric viruses
- Herpes viruses (including cytomegalovirus and Epstein-Barr virus)
- Mycoplasma species
A cluster of cases followed the swine flu vaccination program in 1976, but a link was later denied.
There are reports of cases in which Guillain-Barré syndrome occurred after vaccination against coronavirus. Patients who had previously received the vector vaccine from AstraZeneca or from Johnson & Johnson were affected. In most cases, they developed the first signs of paralysis two to three weeks after vaccination - and thus at a time when the maximum immune response to vaccination typically occurs.
Specifically, the U.S. Vaccine Surveillance System (VAERS ) reports 100 such GSBS cases following Johnson & Johnson vaccination in the U.S. Ninety-five of those affected required hospitalization. One pat. died. The European Medicines Agency (EMA) counted a total of 156 cases of GBS in connection with the administration of AstraZeneca's vaccine up to and including the end of May 2021 - with around 40 million doses vaccinated to date.
A syndrome similar to Guillain-Barré syndrome is one of the adverse effects of immune checkpoint inhibitors.
Clinical featuresThis section has been translated automatically.
Clinically superficial and striking is a flaccid paralysis. It begins with symmetrical weakness with paresthesias, usually in the legs. Spread to the arms. GBS may occasionally begin in the arms or head. Maximum symptomatology in the 3rd to 4th week of illness. Muscle reflexes fail. The sphincters usually remain absent. The weakness persists for several weeks. Afterwards regression.
The function of the facial and oropharyngeal muscles is disturbed in > 50% of patients. The consequences are: dehydration and hyperosmolarity. Complication is in 5-10% of the cases a respiratory paralysis with endotracheal intubation and mechanical ventilation.
Furthermore, significant, life-threatening autonomic dysfunction can be observed:
- blood pressure fluctuations
- cardiac arrhythmias
- gastrointestinal stasis
- urinary retention .
In Fisher's variant (also called Miller-Fisher syndrome), a combination of only ophthalmoparesis, ataxia and areflexia may occur.
DiagnosticsThis section has been translated automatically.
The diagnosis of Guillain-Barré syndrome is primarily made clinically:
- Clinical status
- ECG
- CSF analysis
Differential diagnosisThis section has been translated automatically.
Similar acute weakness can be caused by myasthenia gravis, botulism, poliomyelitis (especially outside the United States), tick paralysis, West Nile virus infection, and metabolic neuropathies, but these disorders can usually be distinguished from Guillain-Barré syndrome as follows:
Myasthenia gravis: intermittent course; aggravated by physical exertion.
Botulism (rare): rigid dilated pupils (in 50% of cases) and other prominent cranial nerve disorders with normal sensory function.
Poliomyelitis (rare): epidemic occurrence with febrile symptomatology.
Tick paralysis (not observed in Europe so far - North and South America, Africa and Australia): causes ascending paralysis, but does not affect the sensory system (More than 40 different tick species have been described as triggers of tick paralysis , belonging to the genera Amblyomma, Dermacentor, Haemaphysalis, Hyalomma, Ixodes and Rhipicephalus. The nerve toxin responsible for the clinical picture is only produced by the tick after about five to seven days of sucking activity, which is why tick paralysis is a very rare clinical picture. Children in particular are affected.
West Nile virus infection: Infectious symptoms with headache, fever and asymmetric flaccid paralysis. Sensory system not affected.
Metabolic neuropathies occur in the context of chronic metabolic disorders.
Complication(s)This section has been translated automatically.
After initial improvement, 2-5% of patients develop chronic inflammatory demyelinating polyneuropathy (CIDP).
TherapyThis section has been translated automatically.
IVIG: Early administration of IVIG 2 g/kg for 1 to 2 days or, more slowly, 400 mg/kg i.v. once daily for 5 consecutive days is the therapy of choice; it has some benefit up to 1 month after disease onset.
Plasmapheresis: Plasma exchange helps if done early; it is used when IVIG is ineffective. Plasma exchange is relatively safe; it shortens the course of the disease and length of hospital stay, lowers the risk of mortality, and reduces the incidence of permanent paralysis. Plasma exchange removes all previously administered IVIG, negating its benefit, and thus should never be performed during or soon after IVIG use. It is recommended to wait until at least 2-3 days after discontinuation of IVIG.
Low molecular weight heparin (LMWH) helps prevent deep vein thrombosis in bedridden patients.
Corticosteroids are contraindicated in Guillain-Barré syndrome because they may worsen the outcome.
General therapyThis section has been translated automatically.
Guillain-Barré syndrome is a medical emergency.
The syndrome requires constant monitoring and support of vital signs, typically in an intensive care unit. Forced vital capacity should be measured frequently so that respiratory support can be provided if necessary. If the vital capacity is < 15 ml/kg, endotracheal intubation is indicated. Inability to lift the head off the pillow by neck flexion is another danger sign; it often develops concomitantly with weakness of the phrenic nerve (diaphragmatic paralysis).
If there is difficulty with oral fluid intake, i.v. fluids are given as needed to maintain a urine volume of at least 1-1.5 L/day. Extremities should be protected from injury and positional damage when bedridden.
Heat treatment helps relieve pain and makes early physical therapy possible.
Cave: Immobilization. Immobilization leads to ankylosis and contractures. Passive range of motion of all joints throughout the range of motion should be started immediately, and active exercise treatment should follow as soon as possible when acute symptoms resolve.
Progression/forecastThis section has been translated automatically.
Approximately 70% of patients make a full recovery. In
< 2% of patients, Guillain-Barré syndrome is fatal.
Most patients show significant improvement over a period of months
However, 2-5% of patients develop chronic inflammatory demyelinating polyneuropathy.
30% of patients still show residual paralysis after 3 years.