Guillain–Barré syndrome (GBS) - neuro

Question 1

Guillain–Barré syndrome (GBS)

Answer 1

-is a neurologic emergency
-Also known as acute inflammatory
polyradiculopathy
-Occurrence rate 1.5 per 100,000 people
-Etiology: thought to be immunologic
-Mortality: 3-5%

Question 2

who/what

Answer 2

Characterized by ascending, progressive (often

profound) weakness; absent deep tendon reflexes; high CSF protein; possible cranial nerve and respiratory compromise; substantial or complete spontaneous recovery
- Young adults and early middle age mostly
- 50% preceded by infectious illness that clears before neurologic dysfunction begins

Question 3

Presentation

Answer 3

Typical: symmetric, ascending, flaccid paralysis
• Usually begins in lower extremities (10% upper)
• Progresses upward
• Should not progress more than 4 weeks
• Weakness
• Greatest in proximal muscles
• Facial weakness in about 50%
-Previous immunization, surgery, hematologic
malignancy, hepatitis B or mycoplasma infection
sometimes
-Areflexia may precede weakness
-Sensory: Many complain of distal paresthesias, Formal testing rarely demonstrates significant sensory loss

Question 4

tests

Answer 4

CSF: normal in the first week, elevated protein and < 10 mononuclear cells by the 2nd weeks

• nerve conduction studies: may be normal early
• Protein elevation is hallmark

Question 5

Treatment

Answer 5

-Respiratory failure and cardiovascular collapse may occur within 24 hours of onset
-ICU/Intermediate care admit until plateau of
progression
-Check vital capacity every 4-6 hours - if < 60%
predicted, consider intubation (23% require
ventilation)
-Autonomic instability may be severe - marked
fluctuations in BP, tachycardia, and malignant
arrhythmias
-Continuous cardiac monitoring
-Hypotension usually mild, managed with fluids
-Caution in treating hypertension - short acting,
easily titrated drugs (nitroprusside)
-Tachycardia - small doses beta blockers
-IVIG cornerstone of treatment - unknown mechanism
-Plasmapheresis - within 2 weeks of onset
-Shortens time to recovery, does not decrease
incidence of respiratory failure
-Contraindicated in severe autonomic instability

Question 6

Prognosis

Answer 6

• Self limited disease - full recovery expected
• Some have residual disabilities, including loss of independent ambulation
• Predictors of poor outcome: Advanced age, Preceding Campylobacter jejuni GI illness or CMV, Ventilation requirement, Axonal degeneration (usually just demyelinating)