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).
- ) 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)
- ) Check out this great reference based out of WashU that includes lots of information comparing and contrasting various neuromuscular conditions
- ) 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.
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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)
- Up to date has a list of other variants
- 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