Purpose of review: The aim of this review is to discuss and highlight the recent advances in our understanding of paraneoplastic syndromes in neuro-ophthalmology and their significance.
Recent findings: The pathophysiologic mechanism in neuro-ophthalmic paraneoplastic syndromes involves an immune response triggered by aberrant expression of onconeuronal antigens that cross-react with antigens in the visual system. Recently, 18-fluoro-deoxy-glucose/positron emission tomography with computed tomography scanning has emerged as a useful modality in diagnosing occult tumors responsible for paraneoplastic syndromes. Paraneoplastic optic neuropathy has been recently associated with the anti-CV2/CRMP-5 antibody. The use of serologic analysis of recombinantly expressed clones (SEREX) has led to the identification of new antigens associated with melanoma-associated retinopathy, such as visual arrestin, rhodopsin, titin, and mitofilin. Calcium-channel blockers and alemtuzumab have been found to improve visual function in cancer-associated retinopathy. Rituximab has been found to be effective in childhood opsoclonus-myoclonus syndrome.
Summary: A high index of suspicion is needed to diagnose neuro-ophthalmic paraneoplastic syndromes. There have been recent advances in our understanding of the pathophysiology and treatment of these disorders. This will facilitate early treatment of causative occult tumors and improves the prognosis.