Guillain-Barre syndrome: Difference between revisions

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==Background==
==Background==
# acute idiopathic inflammatory demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia usually associated with sponatneous remission
*Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction
# Post-infectious autoimmune destruction of peripheral nerves
*Associated with viral or febrile illness, campylobacter infection, or vaccination
## may be associated with hx of antecedent respiratory tract or GI infection (camphylobacter)
*Symptoms at worst 2-4wk after onset, then plateau for 2-4wk, then remit from wks-months  
# Characterized by:
 
## Ascending paralysis; symmetric in legs. rapid onset.
==Clinical Features==
## Autonomic dysfunction: 50%
*Viral illness -> ascending, symmetric weakness or paralysis and loss of DTRs
## arrhythmias, brady/tachy, hypotension, sweating, urinary retention, respiratory failure
*May progress to diaphragm resulting in need for mechanical ventilation (33% of pts)
## symptoms progress and peak ~2-4 weeks after onset, plateau for 2-4 weeks then remit from weeks to months  
*Autonomic dysfunction occurs in 50% of pts
# Intubation in 25% of pts.
 
# 90% full recovery in months with 5% Mortality
*Miller-Fisher Syndrome
# Forms:
**Associated w/ campylobacter infection
## Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
**More likely to be preceded by diarrhea than viral prodrome
### demyelination is immunologically mediated
**Consists of ophthalmoplegia and ataxia
## Acute Motor Axonal Neuropathy (AMAN)
**Weakness is less severe; disease course milder than classic GBS
### pure motor form
### seasonal incidence, associated with Camphylobacter infection
### pts with this form tend to require ventilatory assistance
## Acute Motor Sensory Axonal Neuropathy (AMSAN)
### motor and sensory symptoms
### Miller-Fisher (4%) variant is descending: ophthalmoplegia, ataxia, mainly affects CN's


==Diagnosis==
==Diagnosis==
Physical Exam:
#Required
# symmetric weakness with diminished/absent reflexes
##Progressive weakness of more than one limb
# minimal loss of sensation
##Areflexia
# signs of autonomic dysfunction: dysarrythmias, orthostatic hypotension, transient/persistent hypertension, paralytic ileus, bladder dysfunction, abnormal sweating
#Suggestive
##Progression over days to weeks
##Recovery beginning 2–4 wk after cessation of progression
##Relative symmetry of symptoms
##Mild sensory signs and symptoms
##CN involvement (Bell's palsy, dysphagia, dysarthria, ophthalmoplegia)
##Autonomic dysfunction
###Tachycardia, bradycardia, dysrhythmias, wide variations in BP, postural hypotension
###Urinary retention
###Constipation
###Facial flushing
##Absence of fever at onset
##Cytoalbuminologic dissociation of CSF (high protein (>45) and low WBC count (<10))
##Typical findings on electromyogram and nerve conduction studies


==Work-up==
==Treatment==
# nerve conduction study: slowed nerve conduction velocities, partial motor conduction block, abnormal temporal dispersion, prolonged distal latencies
#Intubation indications:
# LP: normal pressure, few cells (mononuclear), elevated protein (>50mg/dL)
##Vital capacity <15mL/kg
##PaO2 <70 mm Hg on room air
##Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
##Aspiration
#IVIG OR plasmapheresis (provide equivalent but not additive effects)


==Treatment==
==Disposition==
# Admit to neuro / ICU
#Indications for admission to ICU:
# Intubation (if indicated)
##Autonomic dysfunction
## 20/30/40 rule: patient with vital capacity <20mL/kg, max inspiratory pressure <30 cmH20, or max expiratory <40 cm H20 will generally progress to require mechanical ventilation
##Bulbar dysfunction
# Supportive care, ABC's, close monitoring
##Initial vital capacity <20 mL/kg
# Steroids iv (no proven benefit)
##Initial negative inspiratory force <–30 cm of water
# plasmaphoresis vs IVIG
##Decrease of >30% of vital capacity or negative inspiratory force
# DVT Prophylaxis (subQ heparin and SCDs)
##Inability to ambulate
##Treatment with plasmapheresis
##Anticipated clinical course requiring mechanical ventilation
 
==Source==
Tintinalli


[[Category:Neuro]]
[[Category:Neuro]]

Revision as of 04:09, 6 October 2011

Background

  • Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction
  • Associated with viral or febrile illness, campylobacter infection, or vaccination
  • Symptoms at worst 2-4wk after onset, then plateau for 2-4wk, then remit from wks-months

Clinical Features

  • Viral illness -> ascending, symmetric weakness or paralysis and loss of DTRs
  • May progress to diaphragm resulting in need for mechanical ventilation (33% of pts)
  • Autonomic dysfunction occurs in 50% of pts
  • Miller-Fisher Syndrome
    • Associated w/ campylobacter infection
    • More likely to be preceded by diarrhea than viral prodrome
    • Consists of ophthalmoplegia and ataxia
    • Weakness is less severe; disease course milder than classic GBS

Diagnosis

  1. Required
    1. Progressive weakness of more than one limb
    2. Areflexia
  2. Suggestive
    1. Progression over days to weeks
    2. Recovery beginning 2–4 wk after cessation of progression
    3. Relative symmetry of symptoms
    4. Mild sensory signs and symptoms
    5. CN involvement (Bell's palsy, dysphagia, dysarthria, ophthalmoplegia)
    6. Autonomic dysfunction
      1. Tachycardia, bradycardia, dysrhythmias, wide variations in BP, postural hypotension
      2. Urinary retention
      3. Constipation
      4. Facial flushing
    7. Absence of fever at onset
    8. Cytoalbuminologic dissociation of CSF (high protein (>45) and low WBC count (<10))
    9. Typical findings on electromyogram and nerve conduction studies

Treatment

  1. Intubation indications:
    1. Vital capacity <15mL/kg
    2. PaO2 <70 mm Hg on room air
    3. Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
    4. Aspiration
  2. IVIG OR plasmapheresis (provide equivalent but not additive effects)

Disposition

  1. Indications for admission to ICU:
    1. Autonomic dysfunction
    2. Bulbar dysfunction
    3. Initial vital capacity <20 mL/kg
    4. Initial negative inspiratory force <–30 cm of water
    5. Decrease of >30% of vital capacity or negative inspiratory force
    6. Inability to ambulate
    7. Treatment with plasmapheresis
    8. Anticipated clinical course requiring mechanical ventilation

Source

Tintinalli

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