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Show PHOTO ESSAY Retinal Arteriolar Spasm During Transient Monocular Visual Loss in Eosinophilic Vasculitis Kalliopi Stasi, MD, PhD, Rajeev S. Ramchandran, MD, Narsing A. Rao, MD, Steven E. Feldon, MD, MBA, and David A. DiLoreto, Jr., MD, PhD FIG. 1. Fundus photography performed 20 minutes after vision had returned to baseline after an amaurotic episode. The right eye shows spaces in the blood columns of the veins (arrows) along with attenuated arteries (arrowheads). The left eye shows no abnormalities. Abstract: A patient with eosinophilic vasculitis and acquired immunodeficiency syndrome (AIDS) de-veloped episodic transient monocular visual loss. During or immediately after two visual loss episodes, we demonstrated narrowed retinal arterioles, delayed University of Rochester Eye Institute (JS, RSR, SEF, DAD), University of Rochester Medical Center, Rochester, New York; and A. Ray Irvine Jr., MD, Ocular Pathology Laboratory (NAR), Doheny Eye Institute, University of Southern California Keck School of Medicine, Los Angeles, California. Address correspondence to David A. DiLoreto, Jr., MD, PhD, University of Rochester Eye Institute, 601 Elmwood Ave., Box 659, Rochester, NY 14642; E-mail: david_diloreto@urmc.rochester.edu This work was supported in part by National Institutes of Health Grant K08 EY016742-01 (DD) and by an unrestricted grant to the University of Rochester Eye Institute from Research to Prevent Blindness, New York, NY. arterial filling time, and segmented retinal venous flow in the affected eye on fundus photography and fluorescein angiography (FA). Such findings have only rarely been reported in patients with transient monocular visual loss in other conditions, probably because the episodes have ended before fundus photography and FA could be performed. This is the first report to capture retinal vascular changes associated with transient monocular visual loss in a patient with eosinophilic vasculitis. (J Neuro-Ophthalmol 2009;29:58-61) A57-year-old African-American man was referred to our service because of an 8-month history of episodic bilateral transient visual loss. During our examination, he experienced sudden, painless visual loss to light perception 58 J Neuro-Ophthalmol, Vol. 29, No. 1, 2009 Retinal Arteriolar Spasm J Neuro-Ophthalmol, Vol. 29, No. 1, 2009 FIG. 2. Fundus photography performed 60 minutes after vision had returned to baseline after the amaurotic episode described in Figure 1. There are no abnormalities in either eye. in the right eye lasting 2 minutes. Twenty minutes after the conclusion of this episode, vision returned to his baseline of 20/25, and fundus photographs were obtained. They showed segmentation in the blood columns of the retinal veins (‘‘box-carring'') and attenuated retinal arteries in the right eye (Fig. 1A) and no abnormalities in the left eye (Fig. 1B). Sixty minutes after the end of the amaurotic episode, blood flow in the right eye had returned to normal (Fig. 2). A fluorescein angiogram (FA) was performed 75 minutes after the amaurotic episode had concluded. Initial frames of the FA appeared normal in both eyes. During the angiogram, the patient suffered a second amaurotic episode. Within 20 seconds after he reported right eye visual loss to the light perception level, decreased perfusion was apparent in the right eye on the FA (Fig. 3). Perfusion and vision returned to normal after 5 minutes. The perfusion of the left eye remained normal throughout the FA. Optic nerve head hyperfluorescence was evident in the late phase of the angiogram in both eyes (Fig. 3). The patient was admitted to the hospital and treated with heparin (15 U/kg/h). He did not report any further episodes of visual loss after 24 hours of heparin therapy. Results of a FA performed after 48 hours of heparin therapy were normal. Carotid ultrasound examination showed low internal carotid artery velocities without stenosis. Findings from aortic arch, neck, and brain MRI and MRA were significant for an old left occipital lobe infarct. Results of a transesophageal echocardiogram were normal. The patient had the diagnosis of acquired immune deficiency syndrome (AIDS) 20 years earlier. His current CD4 count and viral load were 59 cells/mm3 and 31,000 copies/ml, respectively. Because prominent tender, pulsating temporal arter-ies were noted bilaterally and the erythrocyte sedimentation rate was 46 and C-reactive protein was 15 (normal 0-10), a temporal artery biopsy was performed. Histopathologic analysis of the biopsy specimen revealed eosinophilic leukocytic infiltration of the intima and media consistent with a diagnosis of eosinophilic vasculitis (Fig. 4). A review of the patient's blood studies demonstrated persistent eosinophilia, ranging from 900 to 6,600 eosinophils/mm3 over the previous 3 months. Therapy with 80 mg/day oral prednisone was started, and the eosinophil level returned to normal (<500) within 1 week. During the next 18 months, the patient underwent a very slow taper of prednisone and was then maintained on 1 mg/day prednisone, together with warfarin and highly active antiretroviral treatment. He was free of systemic and visual symptoms during this period and returned to work and other regular activities, which included running a few miles per day. The vascular changes associated with transient mon-ocular vision loss have rarely been photographed (1-4). Others have captured vasospasm associated with concurrent transient monocular vision loss photographically during exercise (1) and angiographically in retinal migraine (2) and impending central retinal vein occlusion (3). However, the 59 J Neuro-Ophthalmol, Vol. 29, No. 1, 2009 Stasi et al FIG. 3. Fluorescein angiography performed 70 minutes after vision had returned to baseline after the amaurotic episode described in Figure 1. A. Right eye, 1:01 minutes after dye injection, is normal. B. Left eye, 1:37 minutes, is normal. C. Right eye, 5:52 minutes. The patient has reported a second amaurotic episode of the right eye. Speckled hyper- and hypofluorescence are evident within the retinal vessels, consistent with interrupted blood flow. D. Right eye, 6:04 minutes. Same findings as in C. E. Left eye, 6:26 minutes, is normal. F. Right eye, 10:53 minutes. The patient has reported that vision in the right eye is returning to baseline. Speckled hyperfluorescence, representing decreased perfusion and concentration of fluorescein, is seen in some vessels (arrowheads). Homogeneous low level fluorescence, representing normal flow in the recirculation phase, is seen in other vessels (arrows). G. Right eye, 11:10 minutes. The patient has reported that vision in the right eye has recovered fully. Vessels appear normal. H. Left eye, 11:41 minutes, is normal. present report is the first to demonstrate such a phenomenon in an FA for a patient with eosinophilic vasculitis. Eosinophilic vasculitis is a condition in which eosin-ophil levels in the blood are abnormally high. Eosinophils infiltrate tissues, including the walls of blood vessels, and cause local inflammatory damage. This condition may manifest in isolation or in association with systemic vasculitides such as Churg-Strauss syndrome, connective tissue diseases, malignancies, drug hypersensitivities, and infections, including AIDS, as in our patient (5-10). Schwartz et al (11) reported a case similar to the present one in which a patient with eosinophilic vasculitis related to AIDS presented with transient monocular visual loss. In eosinophilic vasculitis, infiltration of the vessel wall by eosinophils may lead to inflammation of the temporal artery that can precipitate an occlusive thrombus as well as FIG. 4. Histology of the temporal artery biopsy. Low-power view (A) shows inflammatory cell infiltration with focal disruption of the elastic lamina (arrow). L, lumen. High-power view (B) shows that the infiltrate is made up of chronic inflammatory cells mixed with a few giant cells as well as several eosinophilic leukocytes (arrows) located between the elastic lamina and the lumen of the vessel shown in A. 60 q 2009 Lippincott Williams & Wilkins Retinal Arteriolar Spasm J Neuro-Ophthalmol, Vol. 29, No. 1, 2009 localized vasospasm (11-15). 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