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Updated: Feb 22 2022

Guillain-Barre Syndrome

  • Snapshot
    • A 35-year-old man presents to the emergency department for difficulty with walking. His symptoms began approximately 1 week ago and has progressively worsened. He has noticed some lightheadedness with standing up from a seated position and some numbness in the bilateral lower extremities. Physical examination is notable for 4/5 power and decreased sensation to light touch and pinprick in the bilateral lower extremity and absent patellar and ankle reflexes. A lumbar puncture is performed, which demonstrates a cell count of 1/uL, protein is 135 mg/dL, and glucose is 65 mg/dL. He is admitted to the neurology floor for frequent negative inspiratory pressure and vital capacity measures and intravenous immunoglobulin.
  • Introduction
    • Definition
      • an immune-mediated polyneuropathy that has many variant forms
        • acute inflammatory demyelinating polyneuropathy
        • chronic inflammatory demyelinating polyneuropathy
        • Miller Fisher syndrome
          • ophthalmoplegia
          • ataxia
          • areflexia
  • Epidemiology
    • Risk factors
      • recent infection
        • Campylobacter jejuni
          • most commonly identified
        • Mycoplasma pneumoniae
        • HIV
        • Ebstein-Barr virus
        • Zika virus
  • Pathogenesis
    • A previous infection triggers an immune response that cross-reacts with parts of the peripheral nerve via molecular mimicry
      • damages the Schwann cells
  • Presentation
    • Symptoms/physical exam
      • classic presentation
        • typically ascending (and fairly symmetric) muscle weakness along depressed or absent reflexes
      • other findings may include
        • autonomic dysfunction
        • peripheral cranial nerve palsies
          • cranial nerve III-XII
      • Miller Fisher variant
        • ophthalmoplegia
        • ataxia
        • areflexia
  • Imaging
    • MRI
      • indication
        • typically used to rule out other causes of weakness
      • findings
        • in acute cases, may demonstrate enhancement and thickening of the
          • intrathecal spinal nerve roots
          • cauda equina
  • Studies
    • Spirometry
      • to ensure adequate respiratory function
      • intubate early if necessary
    • Lumbar puncture
      • albuminocytologic dissociation
        • elevated protein
        • normal white blood cell count
    • Nerve conduction studies
      • slowed conduction velocity
      • reduced amplitude in compound muscle action potentials
  • Differential
    • Acute myelopathy
      • differentiating factors
        • spinal cord lesion on MRI
  • Treatment
    • Conservative
      • acute supportive care
        • indication
          • to acutely manage complications of Guillain-Barre syndrome, such as
            • respiratory failure
              • managed with intubation
            • hypotension
              • managed with fluids or phenylephrine
            • atrioventricular blocks and asystole
              • managed with atropine or cardiac pacing
    • Medical
      • plasmapheresis
        • indication
          • hastens neurological recovery
      • intravenous immunoglobulin (IVIG)
        • indication
          • hastens neurological recovery
      • note that plasmapheresis and IVIG are equally effective
  • Complications
    • Respiratory failure
    • Autonomic instability
    • Complications from IVIG and plasmapheresis
  • Prognosis
    • Improved outcomes with either plasmapheresis or intravenous immunoglobulin
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