Of grave concern

Surv Ophthalmol. 2017 Jan-Feb;62(1):96-102. doi: 10.1016/j.survophthal.2015.12.009. Epub 2015 Dec 29.


A 73-year-old woman presented with vision loss and recurrent proptosis with conjunctival chemosis 4 weeks after an uncomplicated fat-only orbital decompression for thyroid eye disease. Her Graves disease was biochemically "burnt out," and she had diabetes mellitus. Initial neuro-imaging showed a straightened optic nerve and orbital apex fat streaking so a bilateral bony decompression of the orbital apex was performed for presumed compressive optic neuropathy. Vision failed to improve, and she experienced cognitive decline. She described metamorphopsia and insomnia. Examination showed a hemifield loss of red sensitivity, difficulties with higher visual processing, and memory problems. Occipital cortical ribboning was seen on diffusion weighted magnetic resonance imaging, cerebrospinal fluid was positive for 14-3-3, and her electroencephalogram showed periodic complexes. A diagnosis of the Heidenhain variant of Creutzfeldt-Jakob disease was made. She became akinetic and mute, dying a few months later.

Keywords: Creutzfeldt-Jacob disease; Heidenhain variant; compressive optic neuropathy; higher visual processing; proptosis; spongiform encephalopathy; thyroid eye disease; visual fields.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Decompression, Surgical / methods*
  • Diagnosis, Differential
  • Electroencephalography
  • Exophthalmos / diagnosis
  • Exophthalmos / etiology*
  • Female
  • Graves Ophthalmopathy / complications*
  • Graves Ophthalmopathy / diagnosis
  • Graves Ophthalmopathy / surgery
  • Humans
  • Magnetic Resonance Imaging
  • Occipital Lobe / diagnostic imaging*
  • Optic Nerve / diagnostic imaging*
  • Orbit / diagnostic imaging*