Of grave concern

详细信息    查看全文

• 关键词：thyroid eye disease ; proptosis ; compressive optic neuropathy ; visual fields ; higher visual processing ; Creutzfeldt-Jacob disease ; Heidenhain variant ; spongiform encephalopathy
• 刊名：Survey of Ophthalmology
• 出版年：2017
• 出版时间：January-February 2017
• 年：2017
• 卷：62
• 期：1
• 页码：96-102
• 全文大小：2041 K
• 卷排序：62

文摘

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.