Guillain-Barré Syndrome (GBS)
Acute autoimmune demyelinating polyradiculoneuropathy that presents with rapidly progressive flaccid weakness
Epidemiology:
• Incidence: 1 to 2 cases per 100,000 per year, slightly greater in males than in females.
Pathogenesis:
• Immune response to a preceding infection that cross-reacts with peripheral nerve components because of molecular mimicry.
• Immune response can be directed towards the myelin or the axon of peripheral nerve
• Resulting in demyelinating and axonal forms of GBS.
Common organisms:
• Most common infection is Campylobacter jejuni
• Other infections: Cytomegalovirus, Epstein-Barr virus, human immunodeficiency virus (HIV), and Zika virus
• Other triggering events : vaccination (Influenza, Meningococcal), surgery, trauma, and bone-marrow transplantation
CLINICAL MANIFESTATIONS:
• The cardinal features of GBS include progressive, fairly symmetric muscle weakness, absent or depressed deep tendon reflexes.
• Onset: few days to a week
• Weakness can vary from mild difficulty with walking to nearly complete paralysis of all extremity, facial, respiratory, and bulbar muscles
DIAGNOSIS:
• Lumber Puncture:
- Elevated CSF protein with a normal CSF white blood cell count = albuminocytologic dissociation
- Of note, normal CSF protein is found in 1/3 to 1/2 of patients when tested earlier than one after symptom onset
• Electrodiaqnostic studies - Electrodiagnostic studies (Ex EMG) are the most specific and sensitive tests for diagnosis of GBS, and establish the underlying pathophysiology as either demyelinating or axonal
• MRI - Spinal MRI may reveal thickening and enhancement of the intrathecal spinal nerve roots and cauda equina
TREATMENT
• Plasmapheresis + Intravenous Immune globulin (IVIG)
• Even before initiating specific therapy, the clinician must decide when and whether to admit the patient to the intensive care unit (ICU) and whether mechanical ventilation is required
• Glucocorticoids are contraindicated in GBS and may worsen outcome.
Sinai Hospital of Baltimore IM Residency @SinaiBmoreIMRes
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