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D. M. Wu, Y. Lu, L. Jai, H. Khanna, M. Leys, J. R. Heckenlively; Atypical Melanoma Associated Retinopathy. Invest. Ophthalmol. Vis. Sci. 2008;49(13):807.
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© ARVO (1962-2015); The Authors (2016-present)
To report multidimensional features in six cases of melanoma associated retinopathy.
In this retrospective case series, the clinical records of 6 patients with melanotic lesions (2 with extraocular melanoma, 2 with retinal melanoma, one with ciliary body tumor, and one with an elevated choroidal nevus) were reviewed. Evaluations consisted of ophthalmological examinations, standardized electroretinograms (ERGs), and Goldmann visual fields. A subset of patients received FA and OCT. Serum was collected from all patients and western blots against retinal extracts (human, bovine, and mouse) and purified retinal proteins were performed.
All six patients had classical symptoms of MAR including photopsias and visual field changes, with a subset also reporting nyctalopia. Visual fields showed central or paracentral scotomas. All patients showed blunting of the foveal reflex with a subset of patients showing pigmentary changes. ERGs revealed negative waveforms in all patients. Venous staining on FA, a previously reported finding with autoimmune retinopathies, was seen in the FA of one patient. Except for one patient who showed cystic changes, OCTs were unremarkable. On western blots, all patients demonstrated the presence of anti-retinal antibodies against human, bovine, and mouse retinal extracts. Patient antisera showed corresponding reactivity against purified arrestin and enolase in two separate patients. One patient's serum stained both cones and bipolar cells on indirect immunofluorescence staining. Interestingly, the serum of the remaining patients did not result in any retinal staining.
We present a series of six patients with melanoma-associated retinopathy as well as an additional patient with an elevated choroidal nevus. All show a stereotyped clinical presentation of MAR in the presence of a melanotic lesion. Only one patient demonstrated antisera reactivity on indirect immunofluoresence to human retinal tissue (to cone, rather than bipolar cells). However, all demonstrate the presence of anti-retinal antibodies as well as the expected central/paracentral scotoma and negative waveform on ERG. Further work is underway to characterize the identity of the antibodies that may be responsible for these visual changes.
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