Optic Disc Edema in Glial Fibrillary Acidic Protein Autoantibody-Positive Meningoencephalitis.

Abstract

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.

• Authors: John J Chen, Allen J Aksamit, Andrew McKeon, Sean J Pittock, Brian G Weinshenker, Jacqueline A Leavitt, Padraig P Morris, Eoin P Flanagan
• Journal: Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society (J Neuroophthalmol) Vol. 38 Issue 3 Pg. 276-281 (09 2018) ISSN: 1536-5166 [Electronic] United States
• PMID: 29210929 (Publication Type: Journal Article, Observational Study, Research Support, Non-U.S. Gov't)

Chemical References

• Autoantibodies
• Biomarkers
• Glial Fibrillary Acidic Protein

Topics

• Adult
• Aged
• Autoantibodies (blood)
• Biomarkers (metabolism)
• Female
• Fluorescein Angiography
• Fundus Oculi
• Glial Fibrillary Acidic Protein (immunology)
• Humans
• Magnetic Resonance Imaging
• Male
• Meningoencephalitis (complications, diagnosis, immunology)
• Middle Aged
• Optic Disk (pathology)
• Papilledema (diagnosis, etiology, metabolism)
• Retrospective Studies
• Young Adult