Vancouver IM LMR

In today’s noon report we review a case of a 60 year old man who presented with acute onset dysphagia, dysarthria, ophthalmoplegia, ataxia, and symmetrical distal peripheral neuropathy (sensory predominant). He was diagnosed with – you guessed it – Guillain-Barré Syndrome of the Miller Fisher variant!

See the didactic slide deck here.

Key takeaway points:

• Differential for polyneuropathy:
  • Systemic diseases (DM, renal disease, amyloidosis, B1 or B12 deficiency, HIV, lyme, connective tissue diseases/ vasculitis, paraneoplastic, critical illness)
  • Toxic (alcohol, heavy metals, lots of prescription medications e.g. chemotherapies, fluoroquinolones, metronidazole, nitrofurantoin, isoniazid and pyridoxine, etc)
  • Autoimmune
    • Guillain-Barré Syndrome (acute)
    • Chronic immune-mediated demyelinating polyneuropathies
  • Others
    • Hereditary (Charcot-Marie-Tooth)
    • Environmental
    • Idiopathic
• Guillain-Barré Syndrome
  • Often preceding infectious illness triggers an immune response against Schwann cells/ myelin → demyelinating disease
  • Variants:
    • Acute immune-mediated demyelinating polyneuropathy (AIDP): classic findings of progressive and symmetric muscle weakness + decreased/ absent deep tendon reflexes, +/-sensory changes, bulbar symptoms, dysautonomia
    • Acute motor axonal neuropathy (AMAN): axonal, not demyelinating; only motor involvement, and can have normal or even increased deep tendon reflexes
    • Miller Fisher Syndrome (MFS): characterized by ophthalmoplegia, ataxia, areflexia, bulbar symptoms, sensory symptoms, and may or may not have weakness
  • Treatment:
    • Supportive care: monitor neuro exam, respiratory status (especially vital capacity!) and hemodynamics
    • These patients can deteriorate quickly – know the indications for HAU/ ICU level care
    • Disease modifying treatment is with IVIG or PLEX (steroids have no role)