Paraneoplastic vision loss.

ABSTRACT

Paraneoplastic vision loss, which represents a small percentage of paraneoplastic neurologic syndromes, can be a blinding disease. Presenting visual symptoms are variable, making diagnosis challenging. History of the presenting illness, ocular examination, and utilization of various modalities, such as automated perimetry, ocular coherence tomography, and electroretinogram allow for localization of vision loss to the optic nerves or retina, guiding in diagnosis and management. Paraneoplastic vision loss is often painless, bilateral, and subacute, and accompanies other neurologic symptoms but can be the first presenting symptom. Paraneoplastic optic neuropathy has been described in association with several antibodies, but most commonly anti-CRMP5. Cancer-associated retinopathy is the most common paraneoplastic autoimmune retinopathy; however, melanoma-associated retinopathy and bilateral diffuse uveal melanocytic proliferation have also been described to be associated with a paraneoplastic process affecting the retina. Paraneoplastic visual loss is an expanding field and advances in research have improved phenotypic characterization; however, further work is needed to identify more reliable biomarkers of disease and to better understand the underlying mechanisms and management.