MILLER-FISHER SYNDROME
 
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MILLER-FISHER SYNDROME

 

DEFINITION:

A benign variant of Guillain-Barre syndrome characterized by ataxia, areflexia, and ophthalmoplegia.

EPIDEMIOLOGY:

  • incidence: rare
  • age of onset:
    • young children
  • risk factors:
    • viral infections
      • viral prodrome 5-10 days prior to onset of acute ataxia in 50% of cases

CLINICAL FEATURES:*

1. Neurological Manifestations

  • truncal ataxia (limbs may also be affected)
  • areflexia
  • motor weakness
    • limbs
    • unilateral or bilateral facial weakness

2. Ocular Manifestations

  • 1. Ophthalmoplegia
    • paralysis of vertical gaze (upward > downward)
    • horizontal gaze is unaffected
    • dissociated nystagmus - most marked in abducting eye
    • ptosis

    •  

  • *Note: While Guillain-Barre syndrome affects only the peripheral nervous system (PNS), MILLER-FISHER SYNDROME is thought to affect both the PNS and CNS.

    • INVESTIGATIONS:

    1. Cerebrospinal Fluid

    • similar to Guillain-Barre
      • slight pleocytosis
      • elevated protein

    MANAGEMENT:

    1. Supportive

    • no treatment for syndrome
    • condition is self-resolving - recovery begins 2-4 weeks after onset of ataxia and complete resolution by 6 months

     

     

    Pediatric Database - MILLER-FISHER SYNDROME

     

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