Fisher syndrome with optic neuropathy has been rarely reported. We report a 78-year-old Frenchman with Fisher syndrome. The patient complained of dizziness and bilateral blurred vision. His corrected visual acuity was 0.03 in the right eye, and 0.02 in the left eye. Deep tendon reflexes were absent. A few days later, bilateral complete external ophthalmoplegia appeared. Both pupils were dilated and pupillary reflexes were absent. Serum anti-GQlb antibodies and anti-GT1a antibodies were detected. After intravenous immunoglobulin treatments, all neurological symptoms including optic neuropathy and external ophthalmoplegia disappered except for pupillary dilatation associated with light-near dissociation. Tonic pupil indicated disorder of the peripheral nervous system.
Conclusion: Fisher syndrome may complicate optic neuropathy.