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.
Original language | English |
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Pages (from-to) | 96-102 |
Number of pages | 7 |
Journal | Survey of Ophthalmology |
Volume | 62 |
Issue number | 1 |
DOIs | |
Publication status | Published - 1 Jan 2017 |
Keywords
- compressive optic neuropathy
- Creutzfeldt-Jacob disease
- Heidenhain variant
- higher visual processing
- proptosis
- spongiform encephalopathy
- thyroid eye disease
- visual fields