Guillain-Barre syndrome: Difference between revisions

No edit summary
No edit summary
(21 intermediate revisions by 7 users not shown)
Line 1: Line 1:
==Background==
==Background==
*Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction
*Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction
*Associated with viral or febrile illness, campylobacter infection, or vaccination
*Although there is often a motor component, patients can also present with sensory deficits. 
*Associated with [[viral syndrome|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  
*Symptoms at worst 2-4wk after onset, then plateau for 2-4wk, then remit from wks-months  
*Associated with [[Campylobacter jejuni]], [[cytomegalovirus]], [[Epstein-Barr virus]], and [[Mycoplasma pneumoniae]]
*Associated with [[Campylobacter jejuni]], [[cytomegalovirus]], [[Epstein-Barr virus]], and [[Mycoplasma pneumoniae]]


==Clinical Features==
==Clinical Features==
*Viral illness → ascending, symmetric weakness or paralysis and loss of DTRs
*[[Viral syndrome|Viral illness]] ASCENDING, symmetric [[weakness]] or paralysis and loss of DTRs
*Little or no sensory involvement  
*Little or no sensory involvement  
*May progress to diaphragm resulting in need for mechanical ventilation (33% of pts)
*May progress to diaphragm resulting in need for mechanical ventilation (33% of patients)
*Autonomic dysfunction occurs in 50% of pts
*Autonomic dysfunction occurs in 50% of patients


===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 but DESCENDING; disease course milder than classic GBS
==Differential Diagnosis==
{{Weakness DDX}}
==Diagnosis==
===Required===
===Required===
*Progressive [[weakness]] of more than one limb
*Progressive [[weakness]] of more than one limb
Line 37: Line 28:
**Facial flushing
**Facial flushing
*Absence of [[fever]] at onset
*Absence of [[fever]] at onset
*Albumin-cytological dissociation of [[CSF]] (high protein (>45) and low WBC count (<10))<ref name="Rosen">Bunney EB, Gallagher EJ: Peripheral Nerve Disorders, in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 7. St. Louis, Mosby, Inc., 2010, (Ch) 105:p 1400-1401.</ref>
 
===Variants and Subtypes===
*Multiple variants<ref>Ho TW et al.
Guillain-Barrésyndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995;118 ( Pt 3):597. </ref><ref>Sumner AJ et al. The physiological basis for symptoms in Guillain-Barrésyndrome. Ann Neurol. 1981;9 Suppl:28. </ref>
*Acute inflammatory demyelinating polyneuropathy
**most common type
**progressive symmetric muscle [[weakness]]
**often with decreased deep tendon reflexes due to peripheral nerve involvement
*Acute motor axonal neuropathy
**often associated with [[campylobacter]] infections
**only motor involvement
*Acute motor and sensory axonal neuropathy
**presence both motor and sensory involement
*Miller-Fisher Syndrome
**Associated with [[campylobacter]] infection
**More likely to be preceded by [[diarrhea]] than viral prodrome
**Presence of ''ophthalmoplegia'' with [[ataxia]] and areflexia
**Weakness is less severe but DESCENDING; disease course milder than classic GBS
**May present similarly to [[botulism]], which is also descending paralysis
 
==Differential Diagnosis==
{{Weakness DDX}}
 
==Evaluation==
*[[LP]]
**'''Albumin-cytological dissociation of [[CSF]] (high protein (>45) and low WBC count (<10)) is most common
**Patients with HIV can have a pleocytosis<ref name="Rosen">Bunney EB, Gallagher EJ: Peripheral Nerve Disorders, in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 7. St. Louis, Mosby, Inc., 2010, (Ch) 105:p 1400-1401.</ref><ref>Brannagan TH et al. HIV-associated Guillain-Barré syndrome. J Neurol Sci. 2003;208(1-2):39. </ref>'''
*Typical findings on electromyogram and nerve conduction studies
*Typical findings on electromyogram and nerve conduction studies
*MRI: Selective enhancement of the anterior spinal nerve roots is suggestive<ref name="Rosen" />
*MRI: Selective enhancement of the anterior spinal nerve roots is suggestive<ref name="Rosen" />


==Treatment==
==Management==
IVIG
===[[IVIG]]===
* Treat nonambulatory pts within 2 weeks of symptom onset
*Treat non-ambulatory patients within 2 weeks of symptom onset


IVIG vs plasmapharesis
===[[IVIG]] vs [[Plasmapheresis]]===
*IVIG a/w thromboembolism and asceptic meningitis
*IVIG associated with [[thromboembolism]] and aseptic [[meningitis]]
*Plasmapheresis a/w greater hemodynamic instability, lower rate of relapse
*Plasmapheresis associated with greater hemodynamic instability, lower rate of relapse
*Combined IVIG and plasmapharesis no better than single therapy (IVIG or plasmapharesis)
*Combined IVIG and plasmapheresis no better than single therapy (IVIG or plasmapheresis)
*IVIG preferred due to convenience and availability
*IVIG preferred due to convenience and availability


Intubation indications
===Intubation indications===
*Vital capacity <15mL/kg
*Vital capacity <15mL/kg
*Negative Inspiratory Force < 30 cm H2O
*Negative Inspiratory Force < 30 cm H2O
Line 57: Line 74:
*Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
*Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
*Aspiration
*Aspiration
*''Avoid'' [[succinylcholine]] during intubation, as this may cause severe hyperkalemia


==Disposition==
==Disposition==
Line 75: Line 93:
<references/>
<references/>


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

Revision as of 15:51, 14 September 2019

Background

Clinical Features

  • Viral illness → ASCENDING, symmetric weakness or paralysis and loss of DTRs
  • Little or no sensory involvement
  • May progress to diaphragm resulting in need for mechanical ventilation (33% of patients)
  • Autonomic dysfunction occurs in 50% of patients

Required

  • Progressive weakness of more than one limb
  • Areflexia

Suggestive

Variants and Subtypes

  • Multiple variants[1][2]
  • Acute inflammatory demyelinating polyneuropathy
    • most common type
    • progressive symmetric muscle weakness
    • often with decreased deep tendon reflexes due to peripheral nerve involvement
  • Acute motor axonal neuropathy
    • often associated with campylobacter infections
    • only motor involvement
  • Acute motor and sensory axonal neuropathy
    • presence both motor and sensory involement
  • Miller-Fisher Syndrome
    • Associated with campylobacter infection
    • More likely to be preceded by diarrhea than viral prodrome
    • Presence of ophthalmoplegia with ataxia and areflexia
    • Weakness is less severe but DESCENDING; disease course milder than classic GBS
    • May present similarly to botulism, which is also descending paralysis

Differential Diagnosis

Weakness

Evaluation

  • LP
    • Albumin-cytological dissociation of CSF (high protein (>45) and low WBC count (<10)) is most common
    • Patients with HIV can have a pleocytosis[3][4]
  • Typical findings on electromyogram and nerve conduction studies
  • MRI: Selective enhancement of the anterior spinal nerve roots is suggestive[3]

Management

IVIG

  • Treat non-ambulatory patients within 2 weeks of symptom onset

IVIG vs Plasmapheresis

  • IVIG associated with thromboembolism and aseptic meningitis
  • Plasmapheresis associated with greater hemodynamic instability, lower rate of relapse
  • Combined IVIG and plasmapheresis no better than single therapy (IVIG or plasmapheresis)
  • IVIG preferred due to convenience and availability

Intubation indications

  • Vital capacity <15mL/kg
  • Negative Inspiratory Force < 30 cm H2O
  • PaO2 <70 mm Hg on room air
  • Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
  • Aspiration
  • Avoid succinylcholine during intubation, as this may cause severe hyperkalemia

Disposition

Indications for admission to ICU

  • Autonomic dysfunction
  • Bulbar dysfunction
  • Initial vital capacity <20 mL/kg
  • Initial negative inspiratory force <–30 cm of water
  • Decrease of >30% of vital capacity or negative inspiratory force
  • Inability to ambulate
  • Treatment with plasmapheresis
  • Anticipated clinical course requiring mechanical ventilation

See Also

References

  1. Ho TW et al. Guillain-Barrésyndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain. 1995;118 ( Pt 3):597.
  2. Sumner AJ et al. The physiological basis for symptoms in Guillain-Barrésyndrome. Ann Neurol. 1981;9 Suppl:28.
  3. 3.0 3.1 Bunney EB, Gallagher EJ: Peripheral Nerve Disorders, in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 7. St. Louis, Mosby, Inc., 2010, (Ch) 105:p 1400-1401.
  4. Brannagan TH et al. HIV-associated Guillain-Barré syndrome. J Neurol Sci. 2003;208(1-2):39.