Guillain-Barré syndrome

Thanks Jay for a great case! This is a patient with a recent hospital stay for likely embolic strokes with acute paresthesias, myagias and rapidly progressing weakness with respiratory compromise found to have Guillan-Barré syndrome (GBS).

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Main points:

  1. ) Diagnoses to consider in a patient with acute onset peripheral neuropathy include: AIDP/ GBS, vasculitis, heavy metal toxicity, acute intermittent porphyria, metabolic/ electrolyte disorders or paraneoplastic (see N Engl J Med 2014;371:2218-23)
  2. ) Check out this great reference based out of WashU that includes lots of information comparing and contrasting various neuromuscular conditions
  3. ) Guillan Barré syndrome can present atypically in case reports/ series: pain is a common symptom (especially back and proximal extremities) and in 1/3 of cases can precede weakness. Additionally greater than half can have paresthesias as well. Although patients classically present with areflexia, a small percent can have normal reflexes or even hyperreflexia.

Want to learn more?

Acute Inflammatory Demyelinating Polyneuropathy

  • Or AIDP, of which GBS is one form
  • Thought to be an autoimmune antibody mediated disease which leads to demyelination and/or axonal damage
  • Other pathophysiologic “immune stimulus” associations include: upper respiratory/ GI/ viral prodrome (classically C jejuni, URIs, now emerging association with Zika virus), acute eosinophilc pneumonia, side effect of checkpoint inhibitors
  • Many don’t have the textbook presentation of acute symmetric ascending (length-dependent) motor deficits without sensory deficits
  • Patients commonly will have paresthesias, dysautonomia, and extremity/ back pain
  • LP shows albuminocytologic dissociation – increased CSF protein with normal WBCs
  • A small % of patients develop respiratory compromise so NIFs and FVCs have to be monitored closely
  • There are several variants of AIDP, the most well known being Miller-Fisher (triad of opthalmoplegia, ataxia and areflexia; also with cranial nerve deficits)
  • Treatment includes IVIG, plasma exchange
  • Check out this review article from Lancet in 2016 which gives a good overview of pathophys, varied presentation and prognosis
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