Neurology-Online

Guillain Barré Syndrome (GBS)

Illustration of a nerve cell

Synonyms in a broader sense

Acute idiopathic polyradiculoneuritis
Polyneuritis
Landry-Guillain-Barré-Strohl Syndrome
Polyradiculitis
idiopathic polyradiculo- neuropathy
Kussmaul-Landry syndrome
GBS

English: Guillain-Barré syndrome, acute inflammatory demyelinating polyradiculoneuropathy (AIDP)

definition

The Guillain-Barré syndrome is a neurological disorder that is based on the demyelination of nerve fibers.
There are two incidence peaks around the age of 25 and 60. Men are affected more frequently than women.
The frequency of Guillain-Barré syndrome is 1-2 / 100,000 / year.

history

The rapidly progressing form of the disease with the most severe paralysis within a day or two legs, poor, Neck and Respiratory muscles was already described in 1859 by Jean-Baptiste-Octave Landry de Thézillat (1826 - 1865).
He wrote a report on ten patients with acute ascending paralysis. For this reason, when the severe courses of the Guillain-Barré syndrome develop particularly rapidly, the Landry paralysis spoken.

Ernst von Leyden (1832 - 1910), as early as 1880 differentiated between "acute and subacute multiple neuritides" as primary inflammatory diseases of the nerve processes and primary diseases of the spinal cord, especially the poliomyelitis (Poliomyelitis).

The Guillain-Barré Syndrome should actually Guillain-Barré-Strohl Syndrome be called. In 1916, Georges Guillain, Jean Alexandre Barré and André Strohl were the first to describe the typical for Guillain-Barré syndrome increased protein levels with normal cell count (cytoalbuminal dissociation) im Nerve water (Liquor) of a patient suffering from acute radiculoneuritis (inflammation of the roots of the nerves).
The collection of nerve water (liquor puncture) for the so-called CSF diagnostics was invented in 1891 by the German internist Heinrich Irenaeus Quincke.

The first larger account of the anatomic-pathological changes in Guillain-Barré syndrome / GBS was published by W. Haymaker and J.W. Kernohan put together.
In the dispute about the cause, the term “infectious” or “rheumatic” origin was spoken of early on. Alfred Bannwarth (1903-1970) and Heinrich Pette (1887-1964) spoke out in favor of an allergic-hyperergic cause in the early 1940s. So you already suspected an essential one Involvement of the immune system.

In 1956, the Canadian Miller Fisher described another form of the disease. He reported the course of the disease in three patients, the acute ones Paralysis of the eye muscles, a disorder of the target movements (ataxia) and a lack of muscle reflexes in the arms and legs.
One patient also had paralysis Facial muscles. Recovery was spontaneous in all three patients.

Two years later, J. H. Austin described one chronic form the disease now known as chronic inflammatory demyelinating polyneuritis (CIDP).

Summary

Guillain-Barré Syndrome (GBS) is a neurological disorder that is due to demyelinating Nerve fibers is based. Here they lose Nerve cell their insulating layer, comparable to a power cable, whereby the nerve cell loses its function of transmitting information.
The cause is not fully understood. An autoimmune reaction and neuroallergic reactions to previous infections are discussed.
In principle, Guillain-Barré syndrome can occur at any age, but especially around the age of 25 and 60.
Typically the disease begins 2-4 weeks after an infection of the upper respiratory tract or the gastrointestinal tract.
The first symptoms are Back pain, Numbness and abnormal sensations in the area of the Feet. This is followed by a mostly symmetrical motor weakness of the legs, Muscle aches, Walking and standing difficulties.
The paralysis rising from below can lead to respiratory paralysis and cardiac arrest, so ventilation and a Pacemaker may become necessary.
The diagnosis is made through an intensive questioning, a physical-neurological examination and a puncture of nerve water (CSF diagnostics) posed.
The therapy is symptomatic under intensive medical supervision. Favorable gradients predominate.
Older patients and patients with long ventilation periods (longer than 1 month) have a poorer prognosis. Up to 4% of patients suffer a relapse after months to years and fall ill again.

root cause

The cause of Guillain-Barré syndrome has not been definitively established. An autoimmune reaction seems to take place, that is, the own immune system attacks the body's own tissue.
In Guillain-Barré syndrome (GBS), microscopic examination of tissue samples reveals inflammation and infiltration with certain immune cells (including macrophages) in the medullary sheaths of the nerves.

Illustration of a nerve cell

Dendrites
Cell body
Axon with medullary sheath, which are attacked in GBS
Cell nucleus

More information on the structure of a nerve cell under our topic: nerve cell

This results in a medullary sheath disintegration from the anterior and posterior roots of the Spinal cord (Polyradiculitis) and the associated peripheral nerves (polyneuritis), which are responsible for the muscles and sensitivity.
You can find further information under our topic: Spinal cord
Another possible explanation for Guillain-Barré syndrome (GBS) is a neuroallergic reaction to previous viral (e.g. Epstein-Barr Virus, Varicella zoster virus) or bacterial (e.g. Campylobacter jejuni, mycoplasma, leptospira, rickettsial) infections.

Symptoms

Often, 2-4 weeks after an infection of the upper airways or gastrointestinal tract begin

uncharacteristic back pain
above all paresthesia removed from the center of the body (distal paresthesia)
Numbness of the feet
then motor symmetric weakness of the legs (walking is difficult or impossible)
Muscle pain (myalgia)
Pain emanating from the nerve roots and impaired coordination when standing and walking due to a lack of deep sensitivity (ataxia).

The paralysis increases from bottom to top up to high paraplegia (tetraplegia), in which neither arms nor legs can be moved. If the diaphragm is involved, breathing will also fail and the patient must be ventilated.
Respiratory paralysis occurs in around 20% of patients. The cranial nerves (cranial neuritis) can also be involved and lead to swallowing disorders and facial paralysis (facial paralysis). The facial paralysis results in difficulty speaking and chewing, as well as decreased tear flow and decreased saliva secretion. Eye movement disorders are also rare.

Read more on this topic at: Facial palsy

The involuntary (autonomic, vegetative) nervous system is also affected. Vegetative symptoms are sweat secretion disorders, cardiac arrhythmias, blood pressure and temperature fluctuations, pupil movement disorders (pupillomotor system), excessively high blood sugar levels (hyperglycaemia) and bladder and bowel emptying disorders.
The peak of the disease is in 90% of patients within 3-4 weeks.

diagnosis

History (anamnesis): In particular, previous, unspecific infections of the respiratory tract or the gastrointestinal tract play a role here.

Investigation from Nervous system : muscle failures (paralysis, paresis), lack of reflexes (areflexia) and sensory disorders are noticeable.

Collection and examination of nerve water (CSF puncture for CSF diagnostics): clear nerve water, normal to slightly increased cell count, normal sugar, increased protein to> 100mg / dl with signs of a barrier disorder (so-called cytoalbuminal dissociation is typical).

The nerve conduction velocity (NLG) is partially slowed down to the point of conduction block.

The Electromyography (EMG), with which the activity of the muscle can be recorded, shows an inadequate or missing supply of the muscles with nerve signals (denervation signs).

With the help of tissue removal from a nerve (nerve biopsy), demyelination of the nerves can be detected under the microscope (histological / pathological).

A pathogen detection (Campylobacter jejuni, Epstein-Barr virus, Varicella zoster virus, Mycoplasma, leptospira, rickettsia) in the blood succeed in some cases.

therapy

Treatment is symptomatic under intensive medical supervision. That is, mainly the function of heart and Lungs are continuously monitored.
If necessary, a Pacemaker and a Ventilation necessary. The nutrition may have to be ensured by giving nutrients and fluids via a vein (infusions).
Furthermore, physiotherapy, psychological care and prophylactic measures to prevent pressure sores, Blood clot formation (Thrombosis), Joint stiffeners (Contractures) and lung infection (Pneumonia) important therapy components.
The medical therapy consists of the high-dose administration of antibodies (7-S immunoglobulin G 0.4g / kg body weight / day) via a vein for 5 days.
Cortisone preparations are only used in the chronic form of Guillain-Barré syndrome.
If the disease progresses and the ability to walk is severely restricted (walking distance less than 5 m without assistance), plasmapheresis is performed 5 times every 2 days.
In the Plasmapheresis the blood fluid (plasma) is separated from the blood cells. Then the plasma can be cleaned and returned to the patient together with the cells.
Alternatively, the cells can be returned together with foreign plasma, plasma substitutes, etc. The plasmapheresis leads to a faster resolution of the symptoms and to a shorter ventilation time.

forecast

The recovery lasts Weeks to months.
The neurological deficits regress in the reverse order. Lethality (lethality) depends on the care and is today less than 5%.
The prognosis is unfavorable if you need ventilation for more than a month. In about 70% of the cases, the Guillain-Barré syndrome heals with motor weaknesses and reflex deficits, but without hindrance to daily life. 5-15% retain impairing disabilities.
In around 4%, the disease breaks out again (relapse) after months or years.

Complications

Of the Cardiac arrest (Asystole) is a complication of Guillain-Barré syndrome, which is caused by a conduction disorder of the nerve cells in the heart.
Due to the pathological changes in special nerve fibers (autonomic neuropathy), which are responsible for a heart rate appropriate to the situation, the heart gets out of rhythm. The consequences are Cardiac arrhythmiaswhere the heart beats too fast (tachycardia), too slowly (bradycardia) or stops beating at all (asystole). In this situation, a pacemaker or resuscitation (resuscitation) to be necessary.

Since the pathological change in special nerve fibers (autonomic neuropathy) affects not only those fibers that pull to the heart, but also e.g. those who go to lung pull, respiratory paralysis can also occur. If this is the case, the patient must be ventilated to ensure an adequate supply of oxygen.

The paralysis (paresis) that occurs in Guillain-Barré syndrome can lead to Blood clot formation / thrombosis and Pulmonary embolism come because the lack of movement tends to make the blood coagulate.
The lack of exercise also promotes the development of pressure sores (bedsores, bedsores), joint stiffening (contractures) and pneumonia.