A 26-year-old woman was referred with a history of excruciating headache and ocular pain in both eyes for 2 weeks. She noted mild decreased vision and perception of "dark spots" centrally in her right eye. She denied any symptoms in her left eye. There was a history of long-term medication use related to a poorly defined psychological condition. There was no family history of eye disease or parental consanguinity, fetal loss, or oral contraceptives. She had a history of childhood vaccination with BCG (bacille Calmette-Guérin [immunization against tuberculosis]).At examination, visual acuity was 20/30 in the right eye and 20/20 in the left eye. No afferent pupillary defect was present. The anterior segment was unremarkable. Dilated fundus examination showed no vitreous inflammatory activity. Posterior examination revealed vascular sheathing in the superotemporal arcade. There were also small blot intraretinal hemorrhages with extensive capillary nonperfusion (Fig. 1). There was no sign of neovascularization or anomalous disk hyperfluorescence. Results of left eye examination were completely normal.Standard hematologic blood cell count, serum chemistry levels, hepatic enzyme levels, urinalysis findings, and complete lipid profile were normal. Results of chest radiography and cerebrospinal fluid analysis were unremarkable. Results of serologic tests for Toxoplasma (IgM and IgG antibodies), hepatitis C virus, and cytomegalovirus were negative. FTA-ABS and VDRL tests were negative. Testing for antibody to human immunodeficiency virus types 1 and 2 was negative. The tuberculin test (PPD) was positive (1.8 cm [strong reaction, Ͼ1.0 cm]). Hemoglobin electrophoresis findings were normal. Angiotensin-converting enzyme was negative. Antinuclear antibodies and rheumatoid factor were unremarkable. Testing for anticardiolipin antibodies revealed a weakly pos-itive titer of IgM and a highly positive titer of IgG. Lupus anticoagulant was negative. Other findings included the following values: protein S, 56% (slightly low); protein C, 200% (moderately high); complement C3, 126 (normal); complement C4, 17 (normal); ANCA P and C, negative; and C-reactive protein, Ͻ0.03 mg/dL (normal). This case is presented for comment concerning diagnosis, workup, and management. We asked two experts for their opinion.
Dr. Craig M. Greven (Winston-Salem, NC):Drs. Oliveira and Ferreira describe a 26-year-old woman with acute onset of "excruciating headache" and eye pain for 2 weeks. Only mild blurred vision (20/30) was found in the right eye. The fundus photograph and fluorescein angiogram of the superotemporal retinal midzone in the right eye show venous beading and mild sheathing of the superotemporal arcade vein with marked sheathing and vasculitis of the more distal veins. The arterioles do not appear to be significantly involved. There are numerous dotand-blot hemorrhages in the adjacent retina. Interestingly, the investigators state that there are no vitreous cells overlying the area of marked vascular sheathing and that there is no intraocul...