Guillain-Barre syndrome

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Background

  • Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction. Although there is often a motor component, patients can also present with sensory deficits.
  • 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
  • Associated with Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, and Mycoplasma pneumoniae

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

Variants and Subtypes

There are multiple Guillain Barre variants with differing presentaions[1][2]

  1. Acute inflammatory demyelinating polyneuropathy - most common type with progressive symmetric muscle weakness often with decreased deep tendon reflexes due to peripheral nerve involvment
  2. Acute motor axonal neuropathy - often associated with campylobacter infections with only motor involvement
  3. Acute motor and sensory axonal neuropathy - presence both motor and sensory involement
  4. Miller-Fisher Syndrome - presence of ophthalmoplegia with ataxia and areflexia

Miller-Fisher Syndrome

  • Associated with 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
  • May present similarly to botulism, which is also descending paralysis

Differential Diagnosis

Weakness

Evaluation

Required

  • Progressive weakness of more than one limb
  • Areflexia

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
  • Absence of fever at onset
  • Albumin-cytological dissociation of CSF (high protein (>45) and low WBC count (<10)) is most common. However 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 nonambulatory patients within 2 weeks of symptom onset

IVIG vs plasmapharesis

  • IVIG associated with thromboembolism and aseptic meningitis
  • Plasmapheresis associated with greater hemodynamic instability, lower rate of relapse
  • Combined IVIG and plasmapharesis no better than single therapy (IVIG or plasmapharesis)
  • 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

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.