Orphanet: Guillain Barré syndrome
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Guillain-Barré syndrome

Disease definition

A clinically heterogeneous spectrum of rare post-infectious neuropathies that usually occur in otherwise healthy patients and encompasses acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN), Miller-Fisher syndrome (MFS) and some other regional variants.

ORPHA:2103

Classification level: Group of disorders
  • Synonym(s):
    • GBS
    • Guillain-Barré-Strohl syndrome
  • Prevalence: 1-9 / 100 000
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: All ages
  • ICD-10: G61.0
  • ICD-11: 8C01.0
  • OMIM: -
  • UMLS: C0018378
  • MeSH: D020275
  • GARD: 6554
  • MedDRA: 10018767

Summary

Epidemiology

The overall annual incidence of GBS varies between 1/91,000 and 1/55,000. In Europe and North America, AIDP is the most frequent form of GBS (accounting for around 90% of cases) and thus the term GBS in general is synonymous with AIDP in Western countries. The axonal forms account for only 3-5% of cases in Western countries but are much more frequent (30%-50% of GBS cases) in Asia and Latin America.

Etiology

In the majority of cases, an infectious disease precedes the onset of limb weakness with Campylobacter jejuni infection being the most frequently identified initiating event. GBS has also been reported to occur after vaccination or following a surgical intervention.

Management and treatment

Treatment consists of rapid administration of intravenous immunoglobulin (IVIg) or plasma exchange (PE). Physiotherapy and rehabilitation are also important.

Prognosis

The prognosis is variable depending on the form of GBS and ranges from patients with complete recovery, to those who are unable to walk 6 months after disease onset, and to patients in whom the disease has a fatal outcome.

Expert reviewer(s):  Pr P.A. [Pieter] VAN DOORN - Last update: December 2009

  A summary on this disease is available in Deutsch (2009) Español (2009) Français (2009) Italiano (2009) Nederlands (2009) Português (2009) Slovak (2009, pdf) Greek (2009, pdf)

Detailed information

General public

Guidelines

Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.