Ambulatory Report -2.21.17 – GBS!

Thanks to Nick Iverson for presenting a great case of a 61 yo M with a history of Graves presenting with acute onset of weakness ultimately thought to have Guillain Barre Syndrome.
  • GBS Pearls
    • rapid onset of “rising weakness” (LMN pattern, legs weak before arms)
    • CSF with albuminocytologic dissociation (elevated CSF protein with a normal CSF white blood cell count)
    • absent reflexes
    • normal sensory exam
    • autonomic dysfunction
    • always check a MIF and FVC to evaluate for respiratory compromise
    • treatment: IVIG, plasma exchange
  • Presentation
    • Weakness starting in the legs (but starts in arms/facial muscles in 10% of pts)
    • Paresthesias in the hands/feet (in 80%)
    • Mild sensory abnormalities
    • Pain in back & extremities (in 66% of presenting patients)
    • Dysautonomia in 70% – tachycardia, urinary retention, HTN/hypotension, orthostatic hypotension, bradycardia, arrhythmias, ileus, loss of sweating
    • Severe respiratory muscle weakness (needing ventilatory support in 10-30%)
    • Facial weakness occurs in 50% or oropharyngeal weakness occurs in 50%,
    • Oculomotor weakness in 15%
  • Work Up
    • LP & CSF – protein, cell count & diff
    • Nerve Conduction Study
    • EMG
    • Antibodies – numerous serologies (look on UpToDate)
  • Variants
    • So first for some nomenclature:
      • Acute immune-mediated polyneuropathies are classified under the larger umbrella = Guillain-Barre Syndrome (GBS)
    • Within GBS, there are several Variants:
      • AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy)
        • Progressive, fairly symmetric muscle weakness w/ absent or depressed deep tendon reflexes (DTRs)
      • Miller Fisher syndrome
        • TRIAD = ophthalmoplegia with ataxia and areflexia
        • Starts with CN sx —> then progresses
      • AMAN (Acute Motor Axonal Neuropathy)
        • DTRs occasionally preserved, Sensory nerves NOT affected
        • Different from AIDP = selective involvement of motor nerves & only involving axons on EMG
      • AMSAN (Acute Sensorimotor Axonal Neuropathy)
        • sensory & motor fibers affect
        • often have delayed or incomplete recovery
      • Look to UpToDate for the many other variants!

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