Acute onset of a bilateral areflexical mydriasis in Miller-Fisher syndrome: a rare neuro-ophthalmologic disease

J Neuroophthalmol. 2000 Mar;20(1):61-2. doi: 10.1097/00041327-200020010-00018.


Miller-Fisher syndrome (MFS) is characterized by variable ophthalmoplegia, ataxia, and tendon areflexia. It seems to be a variant of Guillain-Barré syndrome (GBS), but unlike in GBS, there is a primitive involvement of the ocular motor nerves, and in some cases there is brainstem or cerebellum direct damage. The unusual case of MFS in the current study started with a bilateral areflexical mydriasis and a slight failure of accommodative-convergence. Ocular-movement abnormalities developed progressively with a palsy of the upward gaze and a bilateral internuclear ophthalmoplegia to a complete ophthalmoplegia. In the serum of this patient, high titers of an IgG anti-GQ1b ganglioside and IgG anti-cerebellum. anti-Purkinje cells in particular, were found. The former autoantibody has been connected to cases of MFS, of GBS with associated ophthalmoplegia, and with other acute ocular nerve palsies. The anti-cerebellum autoantibody could explain central nervous system involvement in MFS. The role of these findings and clinical implications in MFS and in other neuro-ophthalmologic diseases are discussed.

Publication types

  • Case Reports

MeSH terms

  • Accommodation, Ocular
  • Acute Disease
  • Autoantibodies / analysis
  • Cerebellum / immunology
  • Child
  • Convergence, Ocular
  • Dexamethasone / therapeutic use
  • Gangliosides / immunology
  • Humans
  • Immunoglobulin G / analysis
  • Immunoglobulins, Intravenous / therapeutic use
  • Male
  • Miller Fisher Syndrome / complications*
  • Miller Fisher Syndrome / immunology
  • Miller Fisher Syndrome / therapy
  • Mydriasis / etiology*
  • Mydriasis / immunology
  • Mydriasis / therapy
  • Ophthalmoplegia / etiology*
  • Ophthalmoplegia / immunology
  • Ophthalmoplegia / therapy
  • Plasmapheresis
  • Purkinje Cells / immunology


  • Autoantibodies
  • Gangliosides
  • Immunoglobulin G
  • Immunoglobulins, Intravenous
  • GQ1b ganglioside
  • Dexamethasone