Of grave concern

Clare L. Fraser*, Simon Taylor, Kate Reid, Omar Ahmad, Mark L. Moster

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)96-102
Number of pages7
JournalSurvey of Ophthalmology
Issue number1
Publication statusPublished - 1 Jan 2017


  • compressive optic neuropathy
  • Creutzfeldt-Jacob disease
  • Heidenhain variant
  • higher visual processing
  • proptosis
  • spongiform encephalopathy
  • thyroid eye disease
  • visual fields


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