Guillain-Barré syndrome

Last revised by Mostafa Elfeky on 11 Jun 2024

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.

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) 1,3. 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) 2. The relationship between the administration of COVID-19 vaccines and the onset of Guillain-Barré syndrome is unclear 9.

The classical presentation of Guillain-Barré syndrome is that of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), which includes symmetrical ascending weakness, areflexia/hyporeflexia, and variable sensory or autonomic involvement. Thus, the term Guillain-Barré syndrome is often used synonymously with acute inflammatory demyelinating polyradiculoneuropathy (AIDP).

However, Guillain-Barré syndrome includes a spectrum of disease and several phenotypes have been described, including:

Cerebrospinal fluid (CSF) abnormalities are characterized by increased protein without pleocytosis (i.e. albuminocytologic dissociation), which is a non-specific finding in isolation, seen in many of the conditions which mimic Guillain-Barré syndrome clinically and on imaging 1,2.

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.

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

Typical findings in Guillain-Barré syndrome are thickening and contrast enhancement of the spinal nerve roots, especially in the region of the cauda and conus medullaris 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 cranial nerve 1.

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

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.

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

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