Guillain-Barre Syndrome

Question 1

Common features of GBS

Answer 1

• History of diarrhea (C. jejuni)
• Ascending paralysis over hours/days that starts with proximal LE muscles
• Areflexia on exam

Question 2

Autonomic involvement in GBS

Answer 2

In addition to motor phenomena, GBS patients often have autonomic involvement causing bradycardia and hypotension

Question 3

Longer list of organisms that may be associated with GBS

Answer 3

• Campylobacter jejuni
• Haemophilus influenzae
• Mycoplasma pneumoniae
• Borrelia burgdorferi
• HIV
• CMV
• EBV

Question 4

Non-infection-associated causes of GBS

Answer 4

• Vaccination (rarely, particularly influenza)
• SLE
• Sarcoidosis
• Lymphoma
• Post-partum
• Certain meds

Question 5

Miller-Fisher variant GBS

Answer 5

• Tetrad:
  
  • Areflexia
  • Ataxia
  • Ophthalmoplegia
  • Cranial nerve weakness (but not extremity weakness)

Question 6

Antibody associated with Miller-Fisher variant GBS

Answer 6

Anti-GQ1b (a ganglioside)

Question 7

Acute motor-axonal neuropathy variant of GBS (AMAN)

Answer 7

Purely motor form of GBS

Affects mostly children, with 70% having evidence of Campylobacter infection

Carries a good prognosis for recovery

Question 8

Acute motor-sensory axonal neuropathy (AMSAN)

Answer 8

Motor and sensory form of GBS

Occurs mostly in adults

Causes significant muscle atrophy and therefore carries a poor prognosis for recovery.

Question 9

Acute panautonomic neuropathy

Answer 9

Rarest subtype of GBS

Associated with high morality rate from cardiovascular involvement and dysrhythmias

Question 10

Three acute paralysis disorders that are often tested against one another

Answer 10

GBS (ascending)

Botulism (descending)

Tick paralysis (asecending)

Question 11

Tick paralysis

Answer 11

Rapidly ascending paralysis with areflexia, ataxia, and respiratory insufficiency

Looks a lot like GBS

Removal of the discovered female tick is curative

Question 12

Mean interval from GBS onset to most severe impairment

Answer 12

12 days

Question 13

Two major causes of mortality in GBS

Answer 13

1. Respiratory insufficiency
2. Arrhythmia

Question 14

LP in GBS

Answer 14

Shows rising protein level up to 400 mg/dL with no associated increase in cell count

“Cytoalbuminologic dissociation”

Classical finding of GBS, but can also occur in some other disorders

Of note, this may not be seen until 1-2 weeks after onset, and remains normal in 10% of cases.

Question 15

Diagnostic workup for GBS

Answer 15

1. LP (looking for albuminocytologic dissociation)
2. C. jejuni antibody and stool culture
3. MRI spine and brain (to rule out secondary etiologies)

• Sometimes:
  
  • ESR
  • Antiganglioside antibodies
  • Anti-GQ1b (if Miller-Fisher presentation)
  • Anti-GM1
  • FVC if respiratory insufficiency is suspected
  • ECG to look for arrhythmias
  • Nerve conduction studies

Question 16

Treatment for GBS

Answer 16

• General measures:
  
  • Intubation and mechanical ventilation should at least be considered in all patients
    
    • If FVC < 15 mL/kg or NIF worse than -30 cm H₂O, intubate
  • Intensive care monitoring for the presence of arrhythmias and blood pressure instability
• Treating GBS:
  
  • IVIg
  • Plasma exchange
    
    • Note: There is no data to suggest that steroids are helpful

Question 17

“Acute inflammatory demyelinating polyneuropathy”

Answer 17

in other words, GBS