title: Of grave concern.
abstract: 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.
authors.affiliation: Department of Ophthalmology, Macquarie University Hospital, Sydney, Australia; Save Sight Institute, Department of Ophthalmology, Faculty of Medicine, University of Sydney, Australia. Electronic address: manuscript@clarefraser.com.
authors.forename: Clare L
authors.initials: CL
authors.lastname: Fraser
authors.affiliation: Department of Ophthalmology, Macquarie University Hospital, Sydney, Australia; Save Sight Institute, Department of Ophthalmology, Faculty of Medicine, University of Sydney, Australia.
authors.forename: Simon
authors.initials: S
authors.lastname: Taylor
authors.affiliation: Department of Ophthalmology, Canberra Hospital, Canberra, Australia.
authors.forename: Kate
authors.initials: K
authors.lastname: Reid
authors.affiliation: Department of Neurology, Macquarie University Hospital, Sydney, Australia.
authors.forename: Omar
authors.initials: O
authors.lastname: Ahmad
authors.affiliation: Wills Eye Hospital, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
authors.forename: Mark L
authors.initials: ML
authors.lastname: Moster
mesh_terms: D000368:Aged; D019299:Decompression, Surgical / Q000379:methods*; D003937:Diagnosis, Differential; D004569:Electroencephalography; D005094:Exophthalmos / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D049970:Graves Ophthalmopathy / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D009778:Occipital Lobe / Q000000981:diagnostic imaging*; D009900:Optic Nerve / Q000000981:diagnostic imaging*; D009915:Orbit / Q000000981:diagnostic imaging*
keywords: Creutzfeldt-Jacob disease; Heidenhain variant; compressive optic neuropathy; higher visual processing; proptosis; spongiform encephalopathy; thyroid eye disease; visual fields