Paraneoplastic Syndromes in Neuro-ophthalmology

Lynn Gordon, MD, PhD; Marc Dinkin, MD Neuro-ophthalmology p. 1401-1421 October 2019, Vol.25, No.5 doi: 10.1212/CON.0000000000000788
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PURPOSE OF REVIEW: This article discusses the varied types of paraneoplastic syndromes that commonly have neuro-ophthalmologic manifestations. Diagnostic considerations and therapeutic options for individual diseases are also discussed.

RECENT FINDINGS: Paraneoplastic syndromes can affect the afferent and efferent visual systems. Paraneoplastic syndromes may result in reduced visual acuity from retinal degeneration, alterations in melanocyte proliferation and uveal thickening, or acquired nystagmus. Ocular motor abnormalities related to paraneoplastic syndromes may present with symptoms from opsoclonus or from neuromuscular junction disease. Diagnosis remains challenging, but serologic identification of some specific antibodies may be helpful or confirmatory. Treatment, in addition to directed therapies against the underlying cancer, often requires systemic corticosteroids, plasma exchange, or immunosuppression, but some specific syndromes improve with use of targeted pharmacologic therapy.

SUMMARY: Diagnosis and therapy of paraneoplastic syndromes presenting with neuro-ophthalmic symptoms remain a challenge, but strategies are evolving and new approaches are on the horizon.

Address correspondence to Dr Lynn Gordon, Jules Stein Eye Institute, David Geffen School of Medicine, University of California Los Angeles, 885 Tiverton Dr, Los Angeles, CA 90095, lgordon@mednet.ucla.edu.

RELATIONSHIP DISCLOSURE: Dr Gordon serves on the board of trustees of the American Academy of Ophthalmology and on the editorial boards of Ophthalmology Retina, Ocular Immunology and Inflammation, and the Journal of Neuro-Ophthalmology. Dr Gordon has received research/grant support from the National Institutes of Health and licensing fees from the University of California Los Angeles for epithelial membrane protein 2. Dr Dinkin serves as an associate editor for the Journal of Neuro-Ophthalmology and as an editor for Practical Neurology and has received compensation for travel for speaking engagements from The American Austrian Foundation and research/grant support from the Helen and Robert Apel Foundation. Dr Dinkin has provided depositions and expert testimony on medicolegal cases involving idiopathic intracranial hypertension, ischemic optic neuropathy, and head trauma.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Drs Gordon and Dinkin discuss the unlabeled/investigational use of azathioprine for cancer-associated retinopathy; corticosteroids for bilateral diffuse uveal melanocytic proliferation, cancer-associated retinopathy, melanoma-associated retinopathy, opsoclonus-myoclonus syndrome, and paraneoplastic optic neuropathy; IV immunoglobulin (IVIg) for cancer-associated retinopathy, melanoma-associated retinopathy, and opsoclonus-myoclonus syndrome; lenalidomide for POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes); mycophenolate mofetil for cancer-associated retinopathy and paraneoplastic optic neuropathy; and rituximab for cancer-associated retinopathy.

© 2019 American Academy of Neurology.