Guillain–barré Syndrome
Basics
Description
Description
- Group of peripheral nerve disorders characterized by autoimmune demyelination and axonal degeneration of peripheral nerves usually leading to acute ascending paralysis
- Humoral and cellular immune mediated
- Leading cause of acute flaccid paralysis worldwide (since polio vaccination)
- Triggered by antecedent bacterial/viral infection
- Increasing incidence with advancing age, male gender
- Average 1.1 per 100,000 per yr
- Weakness reaches nadir at 2–4 wk
- Spontaneous resolution occurs over weeks to months:
- 80% full recovery at 1 yr
- 20% unable to walk at 6 mo
- 5% die of complications:
- PE
- Infection
- Cardiac
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP):
- Most common form of Guillain–Barré syndrome (GBS; 90% of all GBS cases)
- Demyelination sometimes accompanied by axonal loss
- Other forms of GBS:
- Acute motor axonal neuropathy (AMAN):
- Pure motor axonal involvement
- 67% seropositive for Campylobacter jejuni
- Recovery often rapid
- Often pediatric patients
- Acute motor sensory axonal neuropathy (AMSAN):
- Degeneration of myelinated motor and sensory nerves without significant inflammation or demyelination
- Similar to AMAN, but also involves sensory nerves
- Acute panautonomic neuropathy:
- Very rare
- Involves sympathetic and parasympathetic nerves
- Postural hypotension, dysrhythmias, tachycardia, hypertension
- Blurry vision, dry eyes, anhidrosis
- Recovery gradual, often incomplete
- Miller Fisher syndrome:
- Rare
- Rapidly evolving ataxia, areflexia, and ophthalmoplegia without weakness
- Demyelination and inflammation of cranial nerves II and VI, spinal ganglia, and peripheral nerves
- Resolves in 1–3 mo
- Acute motor axonal neuropathy (AMAN):
Etiology
Etiology
- Postinfectious:
- 2/3 with antecedent illness, usually respiratory or GI
- Different autoantibodies associated with different subtypes
- 1–3 wk between prodromal illness and neurologic symptoms
- C. jejuni most common antecedent bacterial infection
- Cytomegalovirus most common antecedent viral infection
- Epstein–Barr virus, VZV, HIV, mycoplasma also associated
- Emerging association with Zika virus, unclear if other viruses also necessary to precipitate GBS after Zika
- Also emerging association with immune modulators (e.g., adalimumab)
- Relationship to vaccines is questionable
- Slightly increased risk ascribed to influenza vaccine
- Incidence ∼2 cases per million vaccines given
- Lower than risks associated with developing influenza
- Case reports of GBS after meningococcal vaccine in adolescent
- No definite association in larger studies
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Citation
Schaider, Jeffrey J., et al., editors. "Guillain–barré Syndrome." 5-Minute Emergency Consult, 6th ed., Lippincott Williams & Wilkins, 2020. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307638/all/Guillain_barré_Syndrome.
Guillain–barré Syndrome. In: Schaider JJJ, Barkin RMR, Hayden SRS, et al, eds. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307638/all/Guillain_barré_Syndrome. Accessed December 11, 2024.
Guillain–barré Syndrome. (2020). In Schaider, J. J., Barkin, R. M., Hayden, S. R., Wolfe, R. E., Barkin, A. Z., Shayne, P., & Rosen, P. (Eds.), 5-Minute Emergency Consult (6th ed.). Lippincott Williams & Wilkins. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307638/all/Guillain_barré_Syndrome
Guillain–barré Syndrome [Internet]. In: Schaider JJJ, Barkin RMR, Hayden SRS, Wolfe RER, Barkin AZA, Shayne PP, Rosen PP, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. [cited 2024 December 11]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307638/all/Guillain_barré_Syndrome.
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