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Enlarged and Enhancing Optic Nerves in Advanced Glial Fibrillary Acidic Protein Meningoencephalomyelitis

White, Daniel MBBS, MRCP; Mollan, Susan P. FRCOphth; Ramalingam, Satheesh MRCP, FRCR; Nagaraju, Santhoosh FRCPath; Hayton, Tom MRCP, PhD; Jacob, Saiju FRCP, DPhil, MD

Section Editor(s): Gold, Daniel R. DO; Levin, Marc MD, PhD

doi: 10.1097/WNO.0000000000000842
Clinical-Pathological Case Study
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Abstract: A 36-year-old woman presented with intermittent fever, nausea and vomiting, generalized polyarthralgias, and bilateral optic disc swelling. She had a history of difficult-to-control myasthenia gravis since the age of 18 years. Lumbar puncture demonstrated a normal opening pressure; cerebrospinal fluid (CSF) was remarkable for high protein, low glucose, and a mononuclear pleocytosis. Although initial MRI of the brain was normal, a repeat study 8 weeks later revealed enlarged and enhancing bilateral intraorbital and intracranial optic nerves. After a nondiagnostic brain biopsy, a CSF sample tested positive for antibodies to glial fibrillary acidic protein (GFAP). Findings in this case indicate that optic nerve swelling encountered in GFAP meningoencephalomyelitis is more likely due to optic nerve inflammation rather than elevated intracranial pressure.

Department of Neurology (DW, TH, SJ), University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom; Birmingham Neuro-Ophthalmology Unit (SPM), Ophthalmology Department, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom; Department of Neuroradiology (SR), University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom; and Department of Neuropathology (SN), University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom.

Address correspondence to Susan P. Mollan, FRCOphth, Birmingham Neuro-Ophthalmology Unit, Ophthalmology Department, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, United Kingdom; E-mail: eye@mollan.net

The authors report no conflicts of interest.

© 2019 by North American Neuro-Ophthalmology Society