Growing Suspicion

Abstract

loss in the right eye for 3 months. On presentation, vision was 4/200 in the right and 20/20 in the left with an afferent pupillary defect on the right. His visual field was full to confrontation but automated perimetry revealed a central scotoma. The remainder of the exam was normal with the exception of slight elevation of the optic nerve head along with mild perivascular sheathing. He received IV solumedrol for 3 days followed by an oral steroid taper. Fat saturated MRI did not show enhancement of the optic nerve nor any brain abnormalities or mass lesions. He had a normal lab workup including CBC, BMP, quantiferon gold, B12, RPR, and ACE and a normal CXR. On follow up one month later, he experienced no vision improvement. At this point, testing for Leber's hereditary optic neuropathy (LHON) was performed and was negative. He returned 6 months later with subjective worsening of vision in the right eye, however acuity was stable at 4/200. He was started on IVIg therapy and autoimmune and NMO antibodies were drawn. Antibody testing was negative and on follow up one month later, his acuity remained unchanged and his scotoma was larger and denser. He was again lost to follow up for 3 years until he began losing vision in the left eye. Exam revealed counting fingers vision in the right eye and 20/40 with a temporal visual field defect in the left eye. MRI showed an enhancing mass extending from the planum tuberculum and suprasellar area to the right temporal lobe and into both orbits. A procedure was performed