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Paraneoplastic Visual Syndromes

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Book cover Ophthalmic Oncology

Part of the book series: M.D. Anderson Solid Tumor Oncology Series ((MDA,volume 6))

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Abstract

A paraneoplastic visual syndrome is a syndrome of cancer-related ocular dysfunction that is not due to either direct effects of primary or metastatic tumor or treatment or treatment-related complications. Paraneoplastic visual syndromes are thought to be principally autoimmune conditions in which the tumor expresses a neuronal antigen that triggers an immune response against cross-reacting ocular antigens. Carcinoma-associated retinopathy (CAR), the most common paraneoplastic visual disorder, results in visual loss that is usually bilateral, rapid, and painless, with both cone and rod dysfunction. The carcinoma-associated cone dysfunction syndrome, part of the CAR spectrum, is very rare; patients with this syndrome have antibodies primarily directed against cones. Melanoma-associated retinopathy is distinguished by symptoms such as sudden shimmering and night blindness; usually, there is near normal color vision, visual acuity, and central vision. Paraneoplastic optic neuropathy is a syndrome of visual loss that is usually characterized by bilateral optic disc swelling, nerve fiber layer hemorrhages, and vitritis; sometimes there are abnormal findings on electroretinography. Treatments that have been tried in patients with paraneoplastic visual syndromes include immunotherapies and monoclonal antibodies. Though temporary improvement in vision has been reported, a progressive decline to severe visual loss is the usual course, even with treatment.

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Correspondence to Anitha Raghunath .

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Raghunath, A., Schiffman, J.S. (2010). Paraneoplastic Visual Syndromes. In: Esmaeli, B. (eds) Ophthalmic Oncology. M.D. Anderson Solid Tumor Oncology Series, vol 6. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-0374-7_33

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  • DOI: https://doi.org/10.1007/978-1-4419-0374-7_33

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