Miller Fisher syndrome: axonal, demyelinating or both?

Electromyogr Clin Neurophysiol. 2000 Dec;40(8):497-502.


Controversy exists concerning whether Miller Fisher syndrome (MFS) is the result of a predominantly axonal or demyelinating polyneuropathy and whether the Guillain-Barré syndrome variant of acute ataxia and areflexia without ophthalmoplegia, ataxic Guillain-Barré syndrome (atxGBS), has a distinct pathophysiology. We explored these issues by reviewing the electrophysiologic features of 6 patients with MFS and 2 patients with atxGBS. EMG laboratory records were reviewed and electrophysiologic findings were categorized as axonal or demyelinating neuropathy using previously defined criteria. Of the 6 patients with MFS, 5 had electrophysiologic evidence suggestive of an axonal, predominantly sensory polyneuropathy; only 1 patient met criteria for demyelinating polyneuropathy. Both patients with atxGBS had demyelinating sensorimotor polyneuropathy. Electrophysiologic abnormalities in MFS typically suggest a predominantly axonal, sensory polyneuropathy, though demyelinating forms occur and may be under-diagnosed using current criteria. AtxGBS, in our experience, is a predominantly demyelinating polyneuropathy.

MeSH terms

  • Adult
  • Aged
  • Ataxia / complications
  • Axons / physiology*
  • Child
  • Demyelinating Diseases / complications*
  • Electromyography
  • Female
  • Guillain-Barre Syndrome / complications
  • Guillain-Barre Syndrome / physiopathology
  • Humans
  • Male
  • Middle Aged
  • Miller Fisher Syndrome / etiology*
  • Miller Fisher Syndrome / physiopathology*
  • Reference Values
  • Reflex, Abnormal