- Clinical science
Guillain-Barré syndrome (GBS) is an acute postinfectious polyneuropathy characterized by symmetric and ascending flaccid paralysis. In affected patients, cross‑reactive autoantibodies attack the host's own axonal antigens, resulting in inflammatory and demyelinating polyneuropathy. Albuminocytologic dissociation, characterized by elevated protein levels and normal cell counts in cerebrospinal fluid (CSF), is a hallmark finding of GBS. Additionally, muscle and nerve electrophysiology are used to diagnose demyelinating processes. Symptomatic and supportive treatment results in disease remission in about 70% of cases. In severe cases or patients who do not respond to treatment, intravenous immunoglobulin (IVIG) administration and/or plasmapheresis may be used. Potentially acute life-threatening complications such as respiratory insufficiency, pulmonary embolism, and/or cardiac arrest increase mortality. Although GBS is associated with a good prognosis overall, up to 20% of patients remain severely disabled and approximately 5% of cases are fatal, despite immunotherapy.
- Incidence: 1–4 cases per 100,000
- Sex: ♂ > ♀ (1.5:1)
- Adults are affected more frequently than children.
Epidemiological data refers to the US, unless otherwise specified.
- About ⅔ of GBS patients experience symptoms of an upper respiratory or gastrointestinal tract infection 1–4 weeks prior to onset of GBS.
- Pathogens associated with GBS: Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, HIV, influenza, and Mycoplasma pneumoniae
- Postinfectious autoimmune reaction that generates cross-reactive antibodies (molecular mimicry)
- Infection triggers humoral response → autoantibodies against gangliosides or other unknown antigens of peripheral Schwann cells → immune-mediated segmental demyelination → axonal degeneration
- Initial symptoms: back and limb pain, esp. paresthesias affecting distal extremities
- Ascending paralysis: Bilateral flaccid paralysis spreads from the lower to the upper limbs in a “stocking‑glove” distribution.
- Reduced or absent muscle reflexes
- Peripheral, symmetric paresthesias in the hands and feet
- Neuropathic pain develops in about ⅔ of patients
- Cardiovascular autonomic dysfunction (arrhythmia), voiding dysfunction, and/or intestinal dysfunction
GBS paralysis affects the muscles of respiration, possibly leading to death due to respiratory failure!
|Acute inflammatory demyelinating polyneuropathy|| || |
|Chronic inflammatory demyelinating polyneuropathy (CIDP)|| || |
|Miller-Fisher syndrome|| || || || |
|Multifocal motor neuropathy (MMN)|| |
|Acute pandysautonomia|| || |
- Albuminocytologic dissociation: elevated protein levels and normal cell counts in cerebrospinal fluid (CSF); CSF cell counts higher than 50 cells per μl CSF indicate that GBS is unlikely!
- Serological screening to identify potential pathogens (e.g., Campylobacter jejuni)
- Detection of antibodies directed against gangliosides (e.g., anti‑GM1 antibodies)
- Slightly elevated myoglobin and total CK blood levels
- Electroneurography: reduced nerve conduction velocity (NCV) due to demyelination : increased
- Electromyography: signs of denervation and pathologic spontaneous activity (unfavorable prognostic sign)
- ECG: Autonomic cardiac dysregulation → impaired heart frequency variation.
- Supportive management
- High dose of intravenous immunoglobulins
- In adults: equivalent outcome as IV immunoglobulins
- In children: only recommended in children with rapidly progressing or severe disease
Although GBS is considered an autoimmune disease, glucocorticoids are not recommended for treatment. They have not shown to hasten recovery or affect the long-term outcome.
- Up to 70% of patients with GBS have a good prognosis: Disease progression peaks 2–4 weeks after the onset of symptoms. Symptoms then recede in reverse order of their development, i.e., the last symptoms to appear resolve first, as Schwann cells remyelinate peripheral nerve axons.
- 3–7% of patients with GBS die due to acute complications such as respiratory paralysis (apnea), pulmonary infection, pulmonary embolism, or cardiac dysfunction.
Death can occur as many as > 30 days after onset of symptoms, during the recovery phase!