Acute Idiopathic Blind Spot Enlargement and Acute Zonal Occult Outer Retinopathy: Potential Mimics of Neuro-Ophthalmic Disease.

In 1988, William Hoyt, MD, et al described "acute idiopathic blind spot enlargement" (AIBSE) in 7 symptomatic patients who had no apparent abnormalities of the optic disc or surrounding retina. With the use of multifocal electroretinography, they showed that the scotoma was caused by occult retinal dysfunction. In 1992, J. Donald Gass, MD, described "acute zonal occult outer retinopathy" (AZOOR) in 13 patients who had sudden loss of often large zones of visual field without fundus abnormalities. Most patients developed zonal atrophy of retinal pigment epithelium and had no improvement in vision. Gass believed that AZOOR, multiple evanescent white dot syndrome, multifocal choroiditis, and AIBSE were all variants of the same disorder. Despite over 3 decades of numerous reports, the classification of these entities, their pathogenesis, and treatment remain controversial. AIBSE and AZOOR may be mistaken for an acute optic neuropathy, so it behooves the neuro-ophthalmologist to be familiar with these disorders. This review describes the initial recognition of AIBSE and its relationship to AZOOR.

Cytomegalovirus Retinal Necrosis With Occlusive Vasculopathy Secondary to Steroid Immunosuppression for Giant Cell Arteritis.

Purpose: This case report discusses an atypical case of cytomegalovirus (CMV) retinal necrosis with panretinal occlusive vasculopathy in a 77-year-old man who was immunosuppressed following treatment for giant cell arteritis (GCA). Methods: A case report is presented. Results: Clinical examination demonstrated a central retinal artery occlusion and pale disc suspicious for arteritic ischemic optic neuropathy in the right eye. Biopsy-proven GCA prompted treatment with oral prednisone. While on glucocorticoid immunosuppression, the patient suffered vision loss in the left eye from CMV-necrotizing retinitis with occlusive vasculopathy. Treatment controlled the CMV infection but tapering of his steroids resulted in worsening GCA, requiring a steroid-sparing treatment, tocilizumab. Conclusions: Corticosteroid immunosuppression for GCA may lead to immune dysfunction allowing for an atypical occlusive vasculitis with retinal necrosis from CMV. Early identification and treatment are essential to adjust the level of immunosuppression and consider alternate therapies to control the GCA and prevent worsening of this opportunistic infection.

SUSAC SYNDROME.

PURPOSE: To describe an atypical presentation of Susac syndrome. METHODS: Observational case report. RESULTS: A 44-year-old man with no significant medical history presented with inferonasal visual field loss in his left eye of several months of duration. He was found to have bilateral migratory arteritis with focal areas of arteriolar occlusion in both eyes and peripheral ischemia superotemporally in his left eye. An extensive hematologic workup was negative for autoimmune disease or coagulopathy. Magnetic resonance imaging with contrast of his brain revealed a hyperintense lesion in the splenium of the corpus callosum. Auditory testing was significant for nonspecific high-frequency hearing loss in the right ear. Given the full clinical picture, a diagnosis of Susac syndrome was made. CONCLUSION: Susac syndrome is a multisystemic, immune-mediated occlusive endotheliopathy characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. However, patients may present with varying degrees of this triad; thus, there should be a high index of suspicion in patients presenting with multiple artery occlusions or multifocal arteritis.

Papiledema unilateral na síndrome do pseudotumor cerebral / Unilateral papilledema in pseudotumor cerebri

Säo apresentadas as fotografias de fundo de olho de 6 mulheres com papiledema inilateral e a síndrome do pseudotumor cerebral. Achados associados foram: obesidade em 5 e a síndrome de sela vazia parcial em 2. Säo discutidas as possíveis explicaçöes para o desenvolvimento de papiledema unilateral nesses pacientes e que favorecem um mecanismo presente na porçäo distal dos nervos ópticos

Papilopatia por irradiaçäo: o seu aspecto oftalmoscópico nas fases aguda e crônica / Papilopathy induced by radiation: its ophthalmoscopic aspect in acute and chronic phases

Fotografias fundoscópicas de sete olhos com papilopatia causada por irradiaçäo revelaram um amplo espectro de alteraçöes no disco óptico. Na fase aguda havia edema pálido de papila, exsudatos algodonosos confluentes na superfície do disco, hemorragias pré-papilares esparsas, estreitamento focal de vasos retinianos de grosso calibre, e exsudatos "duros" intraretinianos de disposiçäo circumpapilar. Nas fases crônicas havia acentuada palidez óptica, excrescências semelhantes à drusas no disco, capilares telangiectásicos, estreitamento e embainhamento de troncos vasculares retinianos e resoluçäo parcial dos exsudatos peripapilares