Cytomegalovirus retinitis associated with Good's syndrome.

PURPOSE: To report a case of cytomegalovirus (CMV) retinitis associated with Good's syndrome. METHODS: A 57-year-old man presented to our ophthalmology clinic with complaints of visual loss in the left eye for 2 weeks. His medical anamnesis revealed myasthenia gravis, thymoma resection, multiple chest infections, and Campylobacter septicemia. Left eye examination revealed mild anterior uveitis, moderate vitritis, and superotemporal active retinitis. RESULTS: Polymerase chain reaction of both aqueous humor and vitreous tap were positive for CMV DNA, which suggested CMV retinitis. The patient was treated with systemic treatment of acyclovir and ganciclovir combined with weekly intravitreal injections of ganciclovir and foscarnet. Retinitis resolved within 3 weeks and visual acuity improved. CONCLUSIONS: CMV retinitis can be associated with Good's syndrome.

Constitutive overexpression of human erythropoietin protects the mouse retina against induced but not inherited retinal degeneration.

Elevation of erythropoietin (Epo) concentrations by hypoxic preconditioning or application of recombinant human Epo (huEpo) protects the mouse retina against light-induced degeneration by inhibiting photoreceptor cell apoptosis. Because photoreceptor apoptosis is also the common path to cell loss in retinal dystrophies such as retinitis pigmentosa (RP), we tested whether high levels of huEpo would reduce apoptotic cell death in two mouse models of human RP. We combined the two respective mutant mouse lines with a transgenic line (tg6) that constitutively overexpresses huEpo mainly in neural tissues. Transgenic expression of huEpo caused constitutively high levels of Epo in the retina and protected photoreceptors against light-induced degeneration; however, the presence of high levels of huEpo did not affect the course or the extent of retinal degeneration in a light-independent (rd1) and a light-accelerated (VPP) mouse model of RP. Similarly, repetitive intraperitoneal injections of recombinant huEpo did not protect the retina in the rd1 and the VPP mouse. Lack of neuroprotection by Epo in the two models of inherited retinal degeneration was not caused by adaptational downregulation of Epo receptor. Our results suggest that apoptotic mechanisms during acute, light-induced photoreceptor cell death differ from those in genetically based retinal degeneration. Therapeutic intervention with cell death in inherited retinal degeneration may therefore require different drugs and treatments.

Photodynamic therapy for choroidal neovascularization secondary to choroidal nevus.

PURPOSE: To describe a patient treated with photodynamic therapy for subfoveal choroidal neovascularization secondary to choroidal nevus. DESIGN: Interventional case report. METHODS: A 61-year-old woman presented with subfoveal choroidal neovascularization secondary to choroidal nevus and best-corrected visual acuity of 20/50. The choroidal neovascularization was treated with two verteporfin photodynamic therapy sessions, separated by 3 months. RESULTS: The choroidal neovascularization was occluded after two sessions. Best-corrected visual acuity improved to 20/25 and remained stable throughout an 18-month follow-up. CONCLUSION: Photodynamic therapy seems to be an effective treatment for subfoveal choroidal neovascularization secondary to choroidal nevus.

Clinical characteristics of acute HSV-2 retinal necrosis.

PURPOSE: To report the clinical features and evaluate the visual outcome of eleven cases of herpes simplex virus-2 (HSV-2) related acute retinal necrosis syndrome (ARN). DESIGN: Retrospective interventional case series. METHODS: Twelve eyes of eleven patients from two European centers, diagnosed with HSV-2 related acute retinal necrosis syndrome were retrospectively reviewed. Herpes simplex virus-2 DNA was detected by polymerase chain reaction in intraocular fluids (aqueous and/or vitreous). Findings at initial examination, clinical evolution with antiviral therapy, complications and final visual acuity were evaluated. RESULTS: Herpes simplex virus-2 DNA was detected in all cases. No sample was positive for more than one virus. The mean age of disease in the first eye was 36 years (ranged from 10 to 57 years). Five patients were women and six were men. All patients were immunocompetent. Previous medical history included neonatal herpes (n = 1), previous ARN (n = 3), trauma (n = 1) and systemic corticosteroid administration before occurrence of ARN (n = 3). Preexisting pigmented chorioretinal scars were found in three cases. Patients were treated with high dose intravenous acyclovir or foscarnet +/- intravitreal ganciclovir +/- interferon. The mean follow-up was 14.5 months (from 5 to 22 months). At the end of the follow-up period, five eyes (41.7%) showed improvement of visual acuity of two or more lines. Final visual acuity was 20/60 or better in four eyes (33.3%), 20/400 or better in four eyes (33.3%) and less than 20/400 in four eyes. CONCLUSION: History of neonatal herpes, triggering events such as neurosurgery, periocular trauma, high-dose corticosteroids, and chorioretinal scars suggest that HSV-2 retinitis reflects reactivation of HSV-2 infection.

Posterior ischemic optic neuropathy after hemodialysis.

PURPOSE: To report a case of visual loss from posterior ischemic optic neuropathy (PION) after hemodialysis. DESIGN: Observational case report. METHODS: Neuro-ophthalmic examination, neuro-imaging including computed tomography (CT) scan, magnetic resonance imaging (MRI) of the head and orbits, and magnetic resonance angiography (MRA) of the neck and cerebral vasculature, as well as electrophysiologic testing including electroretinogram (ERG) and visually evoked response (VER) were performed. RESULTS: Acute onset of painless bilateral no light perception vision with absent pupillary response to light and normal funduscopic examination occurred shortly after completion of hemodialysis. Computed tomography scan and MRA results were normal. Magnetic resonance imaging scan showed small vessel ischemic white matter changes. Electroretinogram results were normal and the VER was unrecordable. CONCLUSIONS: Visual loss after hemodialysis is a rare complication and is associated with anemia and hypotensive events. The visual loss is usually a result of anterior ischemic optic neuropathy. We were unable to find another instance in the literature of visual loss after hemodialysis resulting from PION.

Pseudotumor cerebri associated with Sjögren's syndrome.

PURPOSE: To report a case of papilledema and pseudotumor cerebri developed in association with Sjögren's syndrome. METHODS: Case-report of a 38-year-old woman with history, imaging and histology confirming the diagnosis of both pseudotumor cerebri and Sjögren's syndrome who presented with bilateral decrease of vision. RESULTS: Papilledema associated with pseudotumor cerebri was observed in both eyes. The patient's visual acuity improved transiently with the administration of intravenous steroids and cyclophosphamide; subsequently she needed a ventriculoperitoneal shunt. CONCLUSION: Sjögren's syndrome should be considered in the different etiologies of pseudotumor cerebri. The major improvement with corticosteroids and ventriculoperitoneal shunt makes prompt diagnosis essential.