Neuro-ophthalmic manifestations of the syndrome of ophthalmoplegia, ataxia and areflexia. Observations on 20 patients

Acta Neurol Scand. 1994 Feb;89(2):87-94. doi: 10.1111/j.1600-0404.1994.tb01641.x.


The neuro-ophthalmological manifestations of 20 patients with the syndrome of ophthalmoplegia, ataxia and areflexia are described. The symmetrical nature of the ophthalmoplegia and the associated cerebellar ataxia point to centrally placed lesions. Several supranuclear, nuclear and internuclear ophthalmological signs are identified. Some of these, like partial sparing of the levator palpebrae and normal downgaze in the presence of severe ophthalmoplegia are noted too frequently to be just unusual signs of peripheral oculomotor dysfunction. Other identified features included upper lid retraction on attempted upgaze and preserved Bell's phenomenon in the presence of paralysis of the latter, as well as several other central ophthalmological signs. These findings contrast with those seen in the Guillain-Barré syndrome and, thus, the syndrome of ophthalmoplegia, ataxia and areflexia is not a mere variant of it.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Blepharoptosis / complications
  • Blepharoptosis / diagnosis
  • Blepharoptosis / physiopathology
  • Brain / physiopathology
  • Cerebellar Ataxia / complications*
  • Cerebellar Ataxia / diagnosis
  • Cerebellar Ataxia / physiopathology
  • Child
  • Diagnosis, Differential
  • Female
  • Humans
  • Male
  • Middle Aged
  • Ophthalmoplegia / complications*
  • Ophthalmoplegia / diagnosis
  • Ophthalmoplegia / physiopathology
  • Polyradiculoneuropathy / diagnosis
  • Syndrome