Guillain-Barré Syndrome

from Wikipedia, the free encyclopedia
Classification according to ICD-10
G61.0 Guillain-Barré Syndrome
ICD-10 online (WHO version 2019)
Construction of a nerve cell with a myelin sheath

The Guillain-Barré syndrome ( GBS ; pronunciation: ɡilɛ̃ baˈʁeː zʏnˈdʀoːm ; often, but incorrectly, also: ɡiˈjɛ̃ baˈʁeː zʏnˈdʀoːm ), also Landry-Guillain-Barré-Strohl syndrome , is an acute neurological disease that can be inflamed (inflammatory) changes in the peripheral nervous system. The nerve roots emerging from the spinal cord ( polyradiculitis ) and the associated anterior or proximal nerve segments are particularly affected . The exact cause is not known. In some cases, previous infections and other suspected triggers are blamed. Various courses of varying length can occur, GBS can develop from hours or days to months.

Immunoglobulins and plasmapheresis are used for drug therapy . The prognosis of the Guillain-Barré syndrome: About a fifth of all patients retain functional failures, the mortality rate is about 5%.

frequency

Idiopathic immune neuropathies
Acute
  • Acute inflammatory demyelinating polyneuropathy (AIDP)
  • Acute motor axonal neuropathy (AMAN)
  • Acute Motor and Sensitive Axonal Neuropathy (AMSAN)
  • Regional variants of the GBS
    • Miller-Fisher Syndrome (MFS)
    • Ophthalmoplegia with GQ 1 B autoantibodies
    • MFS-GBS mixed syndrome (overlapping syndrome)
    • Bilateral facial palsy or abdomen palsy with distal paresthesia
    • Pharyngeal-brachial-cervical variant (PCB variant) of GBS
    • Oculopharyngeal weakness
    • Purely paraparetic variant
  • Functional variants of the GBS
    • Acute pandys autonomy
    • Purely sensory GBS
    • Purely motor GBS
    • Purely atactic GBS
Subacute
  • Subacute inflammatory demyelinating polyradiculoneuropathy (SIDP)
Chronic
  • Chronic inflammatory demyelinating polyneuropathy (CIDP)
  • Multifocal motor neuropathy
  • Chronic sensory atactic neuropathy
  • Chronically relapsing axonal neuropathy

Every year around 1.2 to 2.3 in 100,000 people develop Guillain-Barré syndrome. Men are affected 1.5 times more often than women. The disease can occur at any age.

variants

Several variants of Guillain-Barre syndrome are known. The most common ("classic") form of Guillain-Barré syndrome is also known as acute inflammatory demyelinating polyneuropathy . The nerve fibers ( axons ) are retained in their classic shape. Typically, there are ascending, varying degrees of paralysis , beginning in the leg and later also the arm muscles, as well as mostly mild sensitivity disorders . It can paresis in the cranial nerves served regions and disorders of the autonomic nervous system come. The latter can lead to cardiac arrhythmias , for example .

In addition to the classic form, there are variants with preferential damage to the nerve fibers (axonal damage). The secondary axonal damage that occurs in classic AIDP as a result of demyelination must be differentiated from the axonal variants with primary axonal damage. Among the axonal forms that belong A kute M otorische and S ensible A xonale N europathie (AMSAN) and in China and Japan more common A kute M otorische A xonale N europathie (AMAN). The two forms differ, as can be seen from the name, by the nerve fibers that are primarily affected (motor and / or sensory). Because of the axonal damage, both forms are characterized by a comparatively severe course of the disease with a poor prognosis. Axonal forms make up 5 to 10% of diagnosed Guillain-Barré syndromes in North America.

A variant which, by definition, not part of the entity of the Guillain-Barre syndrome, however, differs mainly by a slower progression within four to eight weeks from GBS, which is S ubakute I nflammatorische (inflammatory) D emyelinisierende P olyradikuloneuropathie (SIDP).

Disease emergence

Guillain-Barre syndrome it comes to inflammation (inflammatory) of the myelin sheath (myelin sheath) of a plurality of the spinal cord produces continuous nerve roots ( polyradiculitis ) and the front corresponding or proximal nerve sections. The inflammation leads to demyelination in the sections mentioned. The myelin layer surrounding the nerve fibers is destroyed by an autoimmune reaction ( neuropathy ). This damages the nerve fibers (axons). The latter occurs especially in the special form of Miller-Fisher syndrome . By destroying the myelin layer, the nerve impulses are transmitted only weakly or not at all. As a result, the muscles do not receive any nerve impulses. This explains the paralysis (disruption of the motor nerve tracts). Sensory restrictions (sense of touch, double vision of the eyes, hearing disorders) are the result of the demyelination of sensory nerve tracts.

The disease is caused by an autoimmune pathological mechanism in which autoantibodies ( IgG or IgM ) against gangliosides or myelin or the cell membranes of the axons of the peripheral nervous system are formed in the body.
Due to structural similarities, the antibodies not only recognize the bacteria, but also molecules in the nerve cell envelope. With Mycoplasma pneumoniae it was shown that the antibodies bind to a bacterial glycolipid , a sugar-fat molecule on the cell membrane of the pathogen, and at the same time to galactocerebroside (GalC), one of the most common building blocks in human myelin. This fatty substance is used to electrically isolate the nerve fibers. Anti-GalC antibodies were also found in patients without Guillain-Barré syndrome who had recently been infected with mycoplasma. However, these were only of the antibody isotype M (IgM), the earliest type formed in an immune word. Anti-GalC antibodies in patients with Guillain-Barré syndrome, on the other hand, were of the IgG type. The change in antibody type is partly responsible for the development of the disease.

Much more often, however, it is preceded by infections with other pathogens, primarily Campylobacter jejuni , but viruses such as Epstein-Barr virus , cytomegalovirus , varicella-zoster virus or SARS-CoV-2 have also been described. In rare cases, Guillain-Barré syndrome has been observed in association with vaccinations. The first causal connection in the context of the vaccination against the influenza virus variant A / New Jersey / 1/1976 ( The "swine flu" of 1976 ) was shown in US soldiers in the 1970s (1 case per 100,000 vaccinations) Vaccination program has been discontinued. A causal relationship has also been found in vaccinations against influenza . However, the risk of developing GBS after vaccination is much lower than that of a natural influenza disease itself. A very large pharmacoepidemiological study indicates that out of around 800,000 HPV-vaccinated girls, a single occurrence of GBS can be unequivocally assigned to the vaccination leaves. A case-control study from China, on the other hand, could not identify any causal relationship between an influenza vaccination (or other vaccinations) and GBS.

An occurrence after insect and tick bites, pregnancy and operations has also been observed, but causalities have not been proven beyond doubt.

Symptoms

Guillain-Barré syndrome is characterized by the development of weakness.

Paralysis typically develops in the legs first and spreads down the trunk and arms towards the head. The muscles affected first are usually more severely affected than those affected later. Usually the muscles are symmetrically weakened or paralyzed. Paralysis of the breathing and swallowing muscles are life-threatening and require intensive medical therapy. The degree of paralysis is very variable; H. the spectrum ranges from barely noticeable restrictions on movement to severe paralysis of large parts of the body. Up to 25% of patients suffer from respiratory paralysis and have to be ventilated to sustain life . Many of these patients then suffer from some form of nightmares ( oneiroid syndrome ).

In addition to motor problems, sensitive irritation symptoms also occur regularly. They are usually much lighter. Pain in the muscles is also often reported.

The involvement of the autonomic nervous system with over or underactivity of the sympathetic and parasympathetic nervous system is also essential . Symptoms you may experience are:

  • rapid rise or fall in blood pressure
  • Increase ( tachy ) or decrease ( bradycardia ) in heart rate
  • increased sweating
  • Bladder and bowel disorders

Course and prognosis

The disease usually develops over days and lasts for weeks to months, with a long convalescence phase . In one fifth of the sick, failures persist, the mortality rate is approx. 5%. Relapses ( recurrences ) are observed only very rarely.

One prognostic factor is the ventilation dependency during the acute phase. In patients who have to be ventilated in the acute phase, the mortality rate is 5.5% in the acute phase and 13.6% within a period of 52 months.

There are several forms of Guillain-Barré syndrome: The most common (classic) form of Guillain-Barré syndrome is also known as acute inflammatory demyelinating polyneuropathy . Typically, within four weeks, paralyzes of varying degrees of severity, first of the leg muscles and later also of the arm muscles, and mostly minor sensory disorders, with which the disease often begins. It can also lead to paralysis of the body regions supplied by cranial nerves and to disorders of the autonomic nervous system . The latter can lead to cardiac arrhythmias , for example .

By definition, the symptoms of the disease do not worsen for more than four weeks ( lit .: guideline). Two to four weeks after the peak symptoms begin to regress, which can take months or years.

The more pronounced the paralysis and the longer the course, the worse the prognosis. The Guillain-Barré syndrome can progress to its maximum extent, in which the affected people remain fully conscious but are completely paralyzed. They can only be kept alive through intensive medical treatment.

The prognosis of the axonal course is less favorable, here paralyzes of various forms often remain.

Landry paralysis

A rapidly developing polyradiculitis with ascending flaccid paralysis is called Landry paralysis or Landry-Kussmaul syndrome . Landry's paralysis is a very rapidly progressing form in which artificial respiration is necessary within a few hours.

The name goes back to the French doctor Jean-Baptiste-Octave Landry de Thézillat (1826-1865), who in 1859, at the same time as the German internist Adolf Kussmaul (1822-1902), developed a rapidly developing form of polyradiculitis with the most severe within a few days Described paralysis.

Miller-Fisher Syndrome

The Miller-Fisher syndrome is a rare variant of GBS and is characterized by ocular palsies , areflexia and heavier incoordination ( ataxia ).

Acute motor axonal neuropathy

In acute motor axonal neuropathy (AMAN), not only the nerve sheath (myelin sheath) but also the inner nerve part (axon) are affected.

The prognosis of the rare variants is less favorable in terms of complete healing.

Chronic inflammatory demyelinating polyradiculoneuropathy

The chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is indistinguishable in the early stages of GBS, but lasts longer than four weeks and responds to therapy with glucocorticoids.

Diagnosis

After the first week, an increase in protein with a normal number of cells can be found in the cerebrospinal fluid (“nerve water”) ( cytoalbuminous dissociation ). The nerve conduction speed of the peripheral nerves is significantly slowed. Other parameters can be determined using transcranial magnetic stimulation , electromyography and somatosensitive evoked potentials .

A Chinese study in October 2012 showed that the concentration of the interleukins IL-17 and IL-22 in particular is significantly increased in the CSF, with initial results indicating a correlation between the interleukin concentration and the severity in the mEGOS score.

Antibody detection against GM1 is also suitable for laboratory diagnostics. Antibodies against the ganglioside GQ1b are detectable in nine out of ten people with Miller-Fisher syndrome .

therapy

The syndrome can be completely cured if the diagnosis is made on time. Prevention of infections and thromboses as well as physiotherapy for the prevention of contractures come into question as basic therapy for milder forms . In acute and severe cases, immunotherapy is indicated. Either immunoglobulins can be administered or plasmapheresis can be used. The therapy with immunoglobulins is more expensive, but much gentler and is accompanied by fewer side effects. Plasmapheresis has proven to be particularly effective in the case of rapidly progressing and long-lasting disease processes.

history

The syndrome is named after the French doctors Georges Charles Guillain (1876–1961) and Jean-Alexandre Barré (1880–1967) who, together with André Strohl, described the symptoms of two soldiers in the First World War in 1916 . The two soldiers had developed acute paresis with an areflexia that resolved spontaneously. The symptoms were also accompanied by an increase in protein with a normal number of cells in the nerve water. Similar cases were described in 1859 by the French doctor Jean Landry . Strohl and Landry are often not mentioned in the name of the syndrome.

literature

Guidelines

Review article

  • PA van Doorn et al .: Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. In: The Lancet Neurology 2008 Oct; 7 (10), pp. 939-950. PMID 18848313
  • TM Burns: Guillain-Barré Syndrome. In: Semin Neurol. 2008 Apr; 28 (2), pp. 152-167. PMID 18351518
  • MPT Lunn, HJ Willison: Diagnosis and treatment in inflammatory neuropathies. In: J Neurol Neurosurg Psychiatry. 2009 Mar; 80 (3), pp. 249-258. PMID 19228670
  • JP Malin, E. Sindern: The acute Guillain-Barré syndrome. In: Dtsch Arztebl. 1996; 93 (28-29), pp. A-1895.

pronunciation

Books

  • W. Hacke: Neurology. 13th edition. Springer-Verlag, 2010, ISBN 978-3-642-12381-8 , pp. 705 ff.
  • Wolfgang Trabert: The Guillain Barre Syndrome; Clinical, nosological and prognostic studies based on 39 cases from 1972–1981. RG Fischer, 1983, ISBN 3-88323-405-2 .

Individual evidence

  1. ↑ MPT Lunn, HJ Willison: Diagnosis and treatment in inflammatory neuropathies. In: J Neurol Neurosurg Psychiatry. 2009 Mar; 80 (3), p. 250. PMID 19228670
  2. ↑ A. Ropper, M. Samuels: Adams and Victor's Principles of Neurology. 9th edition. McGraw-Hill Professional, 2009, ISBN 978-0-07-149992-7 , p. 1264.
  3. ^ CW Wallesch: Neurology: Diagnosis and therapy in clinic and practice. 1st edition. Elsevier, 2005, ISBN 3-437-23390-4 , p. 758.
  4. ↑ a b P. A. van Doorn et al .: Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. In: Lancet Neurol. 2008 Oct; 7 (10), p. 939. PMID 18848313
  5. ^ W. Hacke: Neurology. 13th edition. Springer-Verlag, 2010, ISBN 978-3-642-12381-8 , p. 705.
  6. ↑ a b T. M. Burns: Guillain-Barré Syndrome. In: Semin Neurol. 2008; Apr; 28 (2), p. 154. PMID 18351518 .
  7. ↑ Patrick M. Meyer Sauteur et al .: Mycoplasma pneumoniae triggering the Guillain-Barré syndrome: a case-control study. In: Annals of Neurology . October 20, 2016 , doi: 10.1002 / ana.24755 .
  8. ↑ AE Zautner et al .: seroprevalence of campylobacteriosis and relevant post-infectious sequelae. In: Eur J Clin Microbiol Infect Dis. 2014 Jun; 33 (6), pp. 1019-1027. PMID 24413899
  9. ↑ Werner Hacke (Ed.), Neurologie , 14th edition, Springer 2016, p. 801.
  10. ^ H. Renz-Polster, J. Braun: Basic textbook internal medicine. 1st edition. Urban & Fischer, 2000, p. 1092.
  11. ↑ SARS-CoV-2 can trigger the dreaded Guillain-Barré syndrome - press release of the German Society for Neurology from April 23, 2020 (references in the text)
  12. ↑ a b Julia Stowe et al .: Do Vaccines Trigger Neurological Diseases? Epidemiological Evaluation of Vaccination and Neurological Diseases Using Examples of Multiple Sclerosis, Guillain-Barré Syndrome and Narcolepsy . In: CNS drugs . October 1, 2019, doi : 10.1007 / s40263-019-00670-y , PMID 31576507 .
  13. ↑ J. -P. Goullé and L. Grangeot-Keros: Aluminum and vaccines: Current state of knowledge . In: Médecine et Maladies Infectieuses . October 11, 2019, doi : 10.1016 / j.medmal.2019.09.012 .
  14. ^ Yong Chen et al .: Vaccines and the risk of Guillain-Barré syndrome . In: European Journal of Epidemiology . December 19, 2019, p. 1-8 , doi : 10.1007 / s10654-019-00596-1 , PMID 31858323 .
  15. ^ J. Witsch et al .: Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation. doi: 10.1007 / s00415-012-6806-x
  16. ↑ Landry de Thézillat: Traité complet des paralysies. Masson, TI Paris 1859.
  17. ↑ Kußmaul: Two cases of paraplegia with fatal outcome without an anatomically demonstrable or toxic cause. Erlangen 1859.
  18. Jump up ↑ S. Li et al .: IL-17 and IL-22 in Cerebrospinal Fluid and Plasma Are Elevated in Guillain-Barré Syndrome. In: Mediators Inflamm. 2012, p. 260473 ff .; doi: 10.1155 / 2012/260473 ; PMID 23091305 .
  19. ↑ Gerd Herold: Internal Medicine. 2010, p. 836.
  20. ↑ Guillain-Barré-Strohl syndrome. In: whonamedit.com. Retrieved February 6, 2011 .
  21. ↑ G. Guillain, J. Barré, A. Strohl: Sur un syndrome de radiculo-nevrite avec hyperalbuminose du liquide cephalorachidien sans reaction cellulaire. Remarques on les caracteres cliniques et graphiques des reflexes tendineux. In: Bull Soc Med Hop Paris. 1916; 28, pp. 1462-1470.
  22. ^ O. Landry: Note sur la paralysie ascendante aigue. In: Gazette Hebdomadaire Méd Chir. 1859; 6, pp. 472-474, 486-488.

Web links