Guillain-Barré-Syndrom

School ager
with paralysis after sepsis. Sagittal (left and middle) and axial (right) T1 MRI with contrast of the lumbar spine shows diffuse enhancement of the anterior and posterior nerve roots of the cauda equina. The diagnosis was Guillain-Barre syndrome.
pediatricimaging.org • CC-by-nc-sa 4.0
Radiological
approach to non-compressive myelopathies. Guillain–Barre’s syndrome in a 16-year-old male who presented with acute ascending flaccid paralysis. Axial post-contrast image (A) at the level of conus medullaris demonstrates abnormal enhancement of the ventral nerve roots (arrow). Sagittal post-contrast image through the lower thoracic and lumbar spine (B) shows abnormal enhancement of nerve roots (white arrow) and ventral horns of the spinal cord (black arrow)
ejrnm.springeropen.com • CC-by-4.0
Location,
length, and enhancement: systematic approach to differentiating intramedullary spinal cord lesions. Guillian-Barré syndrome. A 2-year-old patient status post-viral prodrome with progressive ascending weakness/paralysis. a Sagittal T2 image of the thoracolumbar spine demonstrates subtle hyperintensity at the level of the conus medullaris (arrow). b Axial T1 post-contrast image of the lumbar spine demonstrates enhancement of multiple nerve roots (arrows)
insightsimaging.springeropen.com • Open Access
Role of MRI
evaluation in acute secondary inability to walk in children. MRI demonstrates a, c sagittal and axial T1W show mildly thickened cauda equina nerve roots. b, d Sagittal and axial post-contrast T1W show anterior and posterior nerve roots of the cauda equina which is predominant anteriorly, pattern 2 of contrast enhancement
ejrnm.springeropen.com • CC-by-4.0
Role of MRI
evaluation in acute secondary inability to walk in children. MRI demonstrates a, c sagittal and axial T1W show mildly thickened cauda equina nerve roots. b, d Sagittal and axial post-contrast T1W show anterior and posterior nerve roots of the cauda equina, pattern 3 of contrast enhancement
ejrnm.springeropen.com • CC-by-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Guillain-Barré syndrome (GBS) is a heterogeneous group of autoimmune polyradiculopathies, involving sensory, motor, and autonomic nerves. It is the most common cause of rapidly progressive flaccid paralysis. It is believed to be one of a number of related conditions, sharing a similar underlying autoimmune abnormality, collectively known as anti-GQ1b IgG antibody syndrome.

Epidemiology

Most cases are preceded by upper respiratory tract infections or diarrhea one to three weeks before their onset, most commonly caused by Campylobacter jejuni (25-40% of patients are seropositive) . Molecular mimicry with the bacterial agents is thought to cause the autoimmunity with the development of anti-GQ1b IgG antibodies.

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

Clinical presentation

The classical presentation of Guillain-Barré syndrome includes symmetrical ascending muscle paresis/paralysis, areflexia/hyporeflexia, and variable sensory or autonomic involvement.

Several subtypes have been described including:

  • acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
    • most common form (60-90%)
  • axonal subtypes
  • regional GBS syndromes

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

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

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

Chronic inflammatory demyelinating polyneuropathy (CIDP) is considered the chronic counterpart to Guillain-Barré syndrome.

Radiographic features

Radiological studies are requested to exclude other causes and in cases where nerve conduction studies and CSF examination are equivocal. MRI of the spine is most useful, helping to exclude other etiologies, such as transverse myelitis and compressive causes of polyradiculopathy.

MRI

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

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

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 .

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

Treatment and prognosis

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

History and etymology

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

Differential diagnosis

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

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Guillain-Barré-Syndrom:
Neurosarkoidose
 
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chronisch
adhäsive Arachnopathie
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