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Updated: Jun 13, 2026
08:17
Optical Coherence Tomography: Imaging Mouse Retinal Ganglion Cells In Vivo
Published on: September 22, 2017
[Paraneoplastic retinopathy and optic neuropathy].
Masamichi Ikawa1, Masaru Kuriyama
1Second Department of Internal Medicine (Neurology), Faculty of Medical Sciences, University of Fukui, 23-3 Shimoaiduki, Matsuoka, Eiheiji-cho, Yoshida-gun, Fukui 910-1193, Japan.
Brain and Nerve = Shinkei Kenkyu No Shinpo
|April 28, 2010
Summary
Paraneoplastic retinopathy and optic neuropathy are rare visual disorders linked to cancer. Autoantibodies against retinal proteins cause these conditions, impacting vision and sometimes responding to treatment.
Area of Science:
- Ophthalmology
- Neuro-oncology
- Immunology
Background:
- Paraneoplastic retinopathy (cancer-associated retinopathy [CAR], melanoma-associated retinopathy [MAR]) and paraneoplastic optic neuropathy (PON) are rare visual disorders associated with systemic cancer.
- CAR involves photoreceptor dysfunction, presenting with vision loss and photopsia, often linked to anti-recoverin antibodies and small-cell lung cancer (SCLC).
- MAR, associated with cutaneous melanoma, features photopsias and distinct electroretinogram patterns, with antibodies targeting retinal bipolar cells.
- PON presents as progressive visual loss and optic disc edema, frequently associated with SCLC and antibodies against collapsin-responsive mediator protein-5 (CRMP-5).
Purpose of the Study:
- To review the clinical manifestations, underlying mechanisms, and treatment approaches for paraneoplastic visual syndromes.
- To highlight the association between specific autoantibodies and distinct paraneoplastic visual disorders.
- To emphasize the importance of early diagnosis and management of the underlying malignancy.
Main Methods:
- Review of clinical presentations, diagnostic criteria, and immunological findings in paraneoplastic retinopathy and optic neuropathy.
- Analysis of the association between specific cancers (SCLC, melanoma) and the identified autoantibodies.
- Evaluation of treatment outcomes with immunotherapy and cancer-directed therapies.
Main Results:
- CAR is characterized by photoreceptor dysfunction, photosensitivity, ring scotoma, and anti-recoverin antibodies, commonly with SCLC.
- MAR presents with photopsias and specific ERG abnormalities, linked to melanoma and anti-bipolar cell antibodies.
- PON involves optic neuropathy, often with other neurological syndromes, and is associated with anti-CRMP-5 antibodies, frequently with SCLC.
- Treatment with corticosteroids, plasma exchange, IVIg, and tumor management can improve visual function in some cases.
Conclusions:
- Paraneoplastic visual syndromes are immune-mediated disorders triggered by cancer, with distinct clinical and serological profiles.
- Early diagnosis and prompt treatment of both the visual disorder and the underlying malignancy are crucial for visual recovery and improved prognosis.
- Understanding the specific autoantibodies involved aids in diagnosing and managing these complex conditions.
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