A 53-year-old man presented with a 5-day history of progressive painless loss of vision in his right eye and a 1-day history of a similar deterioration in the left eye. He denied headaches, diplopia, transient visual obscurations, or other neurological symptoms. He stated that he was in good health and denied any systemic diseases. He was a vegetarian who exercised regularly.Ophthalmological examination revealed that the patient's best-corrected visual acuity was 20/200 in the right eye and 20/40 in the left eye. His pupils were equal and reactive to light; there was a questionable right relative afferent pupillary defect. Confrontation visual field testing showed that he could count fingers in all quadrants of both eyes. Kinetic perimetry of the right eye performed using the I-4e and I-2e test targets showed a moderately dense central scotoma, an enlarged blind spot, and slightly constricted peripheral isopters. The central scotoma in the right eye was confirmed using an Amsler grid. In the left eye, kinetic perimetry showed minimal constriction of the isopters superotemporally. With an Amsler grid, the patient noted distortion and waviness of lines superotemporally. The patient was able to identify 12 of 15 Ishihara plates with the right eye by fixating eccentrically with his nasal field. He identified all 15 color plates with his left eye. Extraocular movements were full, and the slit-lamp examination revealed no abnormalities. Intraocular pressures were within normal limits. Both optic discs were swollen and surrounded by multiple flame-shaped hemorrhages that radiated from the optic discs and obliterated the disc margins (Fig. 1). Small exudates and scattered dot and blot hemorrhages surrounded both optic discs; there was no substantial hemorrhage noted along the retinal vascular arcades outside the immediate peripapillary area. In both eyes, the retinal veins appeared distended and engorged, with boxcarring of the blood column. There were serous macular detachments in both eyes. There were no abnormalities in the retinal periphery of either eye.Patient evaluation showed mild pancytopenia (white blood cell count, 3.1 K/mL; hemoglobin B, 9.7 g/dL; platelets, 135 K/mL), high serum protein, low serum albumin, and high lactate dehydrogenase. Serum vitamin B12, folate, and iron levels were normal. CT and MRI of the brain, MRA, and venography were performed.
Dr. Luetmer:CT of the head without contrast shows no abnormalities; however, magnetic resonance studies reveal a poorly defined heterogeneous infiltrate involving the clivus consistent with a marrow replacement process (Fig. 2).
Dr. Skarf:Given the magnetic resonance findings, a whole-body bone scan and a metastatic bone survey were performed.
Dr. Luetmer:The bone scan shows multiple areas of uptake involving the ribs on both sides, the right scapula, and the left femur (Fig. 3). The metastatic bone survey reveals vague lucencies throughout the skull.
Dr. Skarf:Serum electrophoresis with immunofixation revealed a large IgA lambda monoclonal protein in the beta...