THE CASE
- 42 yo M with no PMH BIBEMS for SOB and generalized weakness
- Nausea, vomiting, diarrhea x 3 days
- Became acutely dyspneic on day of presentation
- Unable to ambulate
- Slurred speech
Allergies: NKMA
Neuro: No spontaneous movement or response to noxious stim in any extremity, DTRs 0 bilaterally
LP: Normal
NCHCT: No acute pathology
- Admitted to MICU
- EMG: Severe demyelinating polyneuropathy
- Repeat LP with ↑, nl WBC
- IVIG started
- Became bradycardic to 30s → Transvenous pacer placed
- Acute polyneuropathy 2/2 immune-mediated peripheral nerve myelin sheath destruction
- Cause unknown
- Suspected: viral/febrile illness (C. jejuni), vaccination
- S/sx worsen over 2-4w; recovery = weeks to years
- 70% of pts develop dysautonomia
- Ascending weakness/paralysis
- May affect diaphragm
- 1/3 of patients require ETT
- May affect diaphragm
- Loss of DTRs
- Diagnostic criteria:
- Required
- Progressive weakness of >1 limb
- Areflexia
- Suggestive
- Progression over days
- Recovery beginning 2-4 wk after cessation of progression
- Relative symmetry of symptoms
- Mild sensory s/sx
- CN involvement (Bell’s palsy, dysphagia, dysarthria, ophthalmoplegia)
- Autonomic dysfunction (tachycardia, bradycardia, dysrhythmias, wide variations in BP, postural HoTN, urinary retention, constipation, facial flushing, anhydrosis, hypersalivation)
- Absence of fever at onset
- Cytoalbuminologic dissociation of CSF (↑ protein, nl WBC)
- In 50-66% of pts at onset
- Protein >45 mg/dL
- WBCs <10 cells/mm3
- Consider HIV if WBC >10 cells/mm3
- Required
- Miller-Fisher variant
- A/w C. jejuni infxn
- Preceded by diarrhea (rather than viral prodrome)
- Signs: Ophthalmoplegia, ataxia, decreased/absent DTRs
- Weakness less severe than GBS
- Antibody testing for C. jejuni for Dx
- Assess respiratory function
- Vital capacity best parameter
- Trend in ED: Have pt count from 1 to 25 in single breath
- Indications for intubation
- VC <15 mL/kg
- PaO2 <70 mmHg on RA
- Bulbar dysfunction (difficulty breathing, swallowing, or speech)
- Vital capacity best parameter
- Indications for ICU admission
- Autonomic dysfunction
- Bulbar dysfunction
- Inability to ambulate
- Treatment with plasmapheresis
- Who to treat:
- IVIG
- Nonambulatory pts within 2 weeks of symptom onset
- IVIG
- IVIG vs plasmapharesis
- IVIG a/w thromboembolism and asceptic meningitis
- Plasmapheresis a/w 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
- Indications for pacemaker placement
- Severe bradycardia
Tintinalli, Judith E., and J. Stephan. Stapczynski. Tintinalli’s Emergency Medicine: A Comprehensive Study Guide. 7th ed. New York: McGraw-Hill, 2011.
Vriesendorp, Francine. “Clinical features and diagnosis of Guillain-Barré syndrome in adults.” Up To Date. http://www.uptodate.com, 17 Nov. 2015. Web. 15 Jan. 2015. http://www.uptodate.com/contents/clinical-features-and-diagnosis-of-guillain-barre-syndrome-in-adults
Vriesendorp, Francine. “Treatment and prognosis of Guillain-Barré syndrome in adults.” Up To Date. http://www.uptodate.com, 3 Jun. 2015. Web. 15 Jan. 2015. http://www.uptodate.com/contents/treatment-and-prognosis-of-guillain-barre-syndrome-in-adults
Additional FOAM Resources: