Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is defined as heterogeneous group of autoimmune polyradiculopathies, involving sensory, motor and autonomic nerves. It is the most common cause of rapidly progressive flaccid paralysis 2.

Epidemiology

Most cases preceded by upper respiratory tract infections or diarrhoea 1-3 weeks before its onset, most commonly caused by Campylobacter jejuni (25-40% of patients are seropositive) 1,3. Molecular mimicry with the bacterial agents is thought to cause the autoimmunity.

Other predisposing factors include recent surgery, lymphoma and systemic lupus erythematosus (SLE) 2.

Clinical presentation

Classical presentation of GBS includes symmetrical ascending muscle paresis or palsy, areflexia or hyporeflexia along with variable degree of sensory or autonomic involvement.

Several subtypes have been described including:

Guillain–Barré syndrome is diagnosed by combination of clinical presentation, CSF study and electrophysiological criteria. 

CSF abnormalities are characterized by increase protein without pleocytosis, which is a non-specific finding, seen in many of the conditions which mimic GBS on imaging and clinically 1-2.

Nerve conduction abnormalities include slow or blocked nerve conduction, prolongation of distal latency and f-waves.

Chronic inflammatory demyelinating polyneuropathy (CIPD) is considered the chronic counterpart to GBS.

Pathology

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Radiographic features

Radiological studies are ordered to exclude other causes and in cases where nerve conduction studies and CSF examination are equivocal. MRI of the spine is most useful helps excluding other aetiologies such as transverse myelitis and compressive causes of polyradiculopathy.

MRI

It is essential that contrast be administered if the diagnosis is suspected as non-contrast sequences are essentially normal 2.

Typical findings in Guillain–Barré syndrome are surface thickening and contrast enhancement on the conus medullaris and the nerve roots of the cauda equina 2.

The most common site of enhancement in Guillain–Barré syndrome is considered to be anterior nerve roots, although enhancement of the posterior nerve roots is also seen 2.

In the brain the facial nerve (CN VII) is the most commonly affected 1.

Treatment and prognosis

Guillain–Barré syndrome is primarily managed with IV immunoglobulins or plasmapheresis along with supportive measures, which can speed up recovery 1. Typically improvement occurs after a number of weeks to months 1 although there is significant mortality (3-10%) 5.

History and etymology

The syndrome was named after Georges Guillain (1876-1961) and Jean Alexandre Barré (1880-1967), French neurologists. André Strohl (1887-1977), a French physiologist, worked together with the both neurologists and is third author in the description done in 1916, and for this reason the syndrome is also referred as Guillain-Barré-Strohl syndrome.

Differential diagnosis

The differential is essentially that of nerve root / cauda equina enhancement: 


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Article Information

rID: 7533
Section: Syndromes
Synonyms or Alternate Spellings:
  • Guillain Barre syndrome
  • Guillain–Barré syndrome (GBS)
  • GBS
  • GB syndrome
  • Guillain-Barré syndrome (GBS)
  • Guillain-Barré syndrome
  • Guillain–Barré–Strohl syndrome
  • Landry's paralysis
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