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Optic Disc Edema in Glial Fibrillary Acidic Protein Autoantibody–Positive Meningoencephalitis

Chen, John, J., MD, PhD; Aksamit, Allen, J., MD; McKeon, Andrew, MB, BCh; Pittock, Sean, J., MD; Weinshenker, Brian, G., MD; Leavitt, Jacqueline, A., MD; Morris, Padraig, P., MB, BCh; Flanagan, Eoin, P., MB, BCh

doi: 10.1097/WNO.0000000000000593
Original Contribution: PDF Only

Background: Glial fibrillary acidic protein (GFAP) autoantibody–positive meningoencephalitis is a newly described entity characterized by a corticosteroid-responsive meningoencephalomyelitis. Some patients with GFAP autoantibody–positive meningoencephalitis have been found to have optic disc edema, which has previously not been well characterized.

Methods: We performed a retrospective, observational case series of Mayo Clinic patients found to have GFAP-IgG and optic disc edema from January 1, 2000, to December 31, 2016. We identified 40 patients with GFAP-IgG seropositivity by tissue-based immunofluorescence and cell-based assay. Patients were screened for the following inclusion criteria: 1) serum, cerebrospinal fluid, or both that yielded a characteristic astrocytic pattern of mouse tissue immunostaining with confirmation of IgG reactive with specific GFAPα isoform by cell-based assay; 2) meningoencephalitis or encephalitis; and 3) optic disc edema. We excluded those with coexisting aquaporin-4-IgG or insufficient clinical information.

Results: Ten patients had optic disc edema and met inclusion criteria. The median age was 39.5 years and 60% were men. Visual acuity was unaffected and disc edema was bilateral in all cases. Mild vitreous cell was noted in 3 patients. The optic disc edema resolved with corticosteroid treatment but resulted in mild optic atrophy in 2 patients. The median lumbar puncture opening pressure was 144 mm H2O (range, 84–298 mm H2O). Brain MRI revealed radial perivascular enhancement in all except 1 patient. Fluorescein angiography was available for 1 patient with optic disc edema, which showed leakage from the venules.

Conclusions: Patients with GFAP autoantibody–positive meningoencephalitis can have optic disc edema that can mimic papilledema. The cause of the optic disc edema remains uncertain, but most patients did not have raised intracranial pressure.

Departments of Ophthalmology (JJC, JAL), Neurology (AJA, AM, SJP, BGW, EPF), Laboratory Medicine and Pathology (AM, SJP, EPF), and Neuroradiology (PPM), Mayo Clinic, Rochester, Minnesota.

Address correspondence to John J. Chen, MD, PhD, Departments of Ophthalmology and Neurology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905; E-mail: chen.john@mayo.edu

Supported in part by an unrestricted grant to the Department of Ophthalmology by Research to Prevent Blindness, Inc, New York, NY; the Mayo Clinic Center for Individualized Medicine; Department of Laboratory Medicine and Pathology; and the Center of MS and Autoimmune Neurology.

Dr. A. McKeon is a named inventor on a patent application filed by the Mayo Foundation relating to the GFAP antibody. The remaining authors report no conflicts of interest.

© 2018 by North American Neuro-Ophthalmology Society