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Non-Organic Vision Loss
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Ashwini Kini, Mangayarkarasi Thandampallayam Ajjeya, Padmaja Sudhakar
Paraneoplastic syndromes: Paraneoplastic syndromes include patients with cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR) or paraneoplastic optic neuropathy (PON). They often present as bilateral progressive painless vision loss, decreased nighttime vision, worsening color vision, positive visual phenomena and visual field loss. Patients have a normal fundus exam and have normal CT/MRI head. In clinically suspected cases, one should promptly further evaluate by obtaining retinal and optic nerve autoantibodies and ERG.
Impairment of visual functions
Published in Ramar Sabapathi Vinayagam, Integrated Evaluation of Disability, 2019
Retinitis pigmentosa, vitamin A deficiency, glaucoma, X-linked congenital stationary night blindness, gyrate atrophy, Laurence-Moon syndrome, Oguchi disease, optic atrophy, peripheral chorioretinitis, siderosis retinae, cancer-associated retinopathy or hypoxia, and uncorrected myopia causes night blindness (nyctalopia). If night blindness is irreversible even after maximum medical management, Integrated Evaluation of Disability assigns an impairment of 25%.
Posterior uveitis
Published in Gwyn Samuel Williams, Mark Westcott, Carlos Pavesio, Bushra Thajudeen, Practical Uveitis, 2017
Gwyn Samuel Williams, Mark Westcott
Although this paraneoplastic syndrome is extremely rare, patients present with similar symptoms, hence the mention here. Patients complain of significant photopsia, photophobia and acute painless loss of vision. Symptoms are bilateral but can be asymmetric and suggestive of both cone involvement (visual acuity loss, colour disturbance and central scotoma) and rod involvement (peripheral field loss and impaired dark adaptation). In keeping with the theme of these diseases in the initial stages the examination will be normal. In late disease a retinal appearance similar to AZOOR can develop. It is thought to be due to a tumour-related immune response in which antibodies are created which cross-react with retinal proteins. Continuing the similarity with AZOOR, abnormal visual fields, fundus autofluorescence and outer segment loss on OCT will be present. Electrodiagnostic testing is key for the diagnosis and will always demonstrate abnormalities, usually of both rod and cone dysfunction. Cancer-associated retinopathy (CAR) is one of the rare causes of an ‘electronegative’ ERG but this phenomenon is far more common with melanoma-associated retinopathy (MAR – see below).
Proceedings of the 43nd Annual Upper Midwest Neuro-Ophthalmology Group Meeting, July 23, 2021 and Second Virtual Upper Midwest Neuro-Ophthalmology Group Meeting
Published in Neuro-Ophthalmology, 2021
Deena Tajfirouz, Casey Judge, John J. Chen, Collin McClelland
Judy Chen MD, University of Wisconsin, presented three interesting cases of painless visual loss occurring across three generations of women in one African-American family attributable to Leber’s hereditary optic neuropathy (LHON). Each case had a unique presentation. A 10-year-old girl presented with severe acute painless bilateral vision loss and was found to harbour anti-retinal antibodies concerning for cancer-associated retinopathy/optic neuropathy. She was treated with IVIG therapy before the diagnosis of LHON was made. The 10-year-old’s mother presented at age 42 with chronic progressive vision loss over a few months. Six months later, the 10-year-old’s 60-year-old grandmother presented with progressive painless bilateral vision loss. Discussion was held regarding the interesting disparity in ages of presentation of vision loss and the unusual occurrence among females in this case. The non-specific nature of anti-retinal antibodies was also discussed.
Cancer-associated Retinopathy Developing After 10 Years of Complete Breast Cancer Remission
Published in Neuro-Ophthalmology, 2019
Nozomi Igarashi, Hiromasa Sawamura, Toshikatsu Kaburaki, Makoto Aihara
Cancer-associated retinopathy (CAR) is a well-known autoimmune paraneoplastic retinopathy. It is thought to be a form of autoimmune retinopathy, which is characterized by pan-retinal degeneration, fast deterioration of visual functions, severe electroretinographic changes, anti-retinal antibodies, and a history of carcinoma.1,2 Typical visual symptoms of CAR include bilateral and progressive, painless, severe, and permanent visual loss.1,3 Immunity against the cancer and cross-reactivity with retinal antigens are thought to be the cause of the disease.4–7 Frequent primary malignancies are small-cell lung carcinoma, gynaecological malignancies, and breast cancer.4 Based on a cohort study of 209 patients, the interval between the diagnosis of cancer and the onset of CAR varied from weeks to years, depending on the cancer.8 According to a study of CAR in breast cancer, the interval was an average of 4.6 years, but its peak was 2–3 years after the clinical diagnosis in autoantibody-positive patients.5 Cases with long intervals between the clinical diagnosis of malignancy and visual loss, especially >10 years, have not been well-documented. We describe an anti-recoverin, antibody-positive CAR patient who showed rapid deterioration of the visual field and progressive loss of ellipsoid zones as revealed by spectral domain-optical coherence tomography (SD-OCT) after 10 years of complete remission from breast cancer.
Cancer-Associated Retinopathy and Treatment with Intravenous Immunoglobulin Therapy. A Seldom Used Approach?
Published in Ocular Immunology and Inflammation, 2021
Luis Ramos-Ruperto, Carmen Busca-Arenzana, Ana Boto-de los Bueis, Arnelle Schlincker, Francisco Arnalich-Fernández, Ángel Robles-Marhuenda
Therefore, using the search terms ‘cancer associated retinopathy’ we collected in MEDLINE data base cases from 1999 to 2018 in which the use of IVIG had been reported (Table 1). We didn´t include cases associating optic neuropathy. We analyzed the time elapsed from the onset of symptoms to the treatment with IVIG, the treatment received in chronological order and the clinical response to IVIG therapy in 12 cases. We observed that 9 out of 12 patients showed stabilization of visual symptoms after the treatment. However, only 4 out of 12 patients showed improvement. In all the cases of improvement a month or less had passed from the onset of visual symptoms to the start of IVIG therapy. We observed that the shorter the time elapsed is from the onset of visual symptoms to the start of the treatment with IVIG, the better the clinical response results. Nonetheless, in most cases, steroid therapy and other treatments which have proved to provide certain benefits, such as rituximab or plasmapheresis11,12, were applied together with IVIG therapy. The isolated effect of IVIG could only be documented in a single case, in which IVIG infusion was early started from the onset of visual symptoms, to respond to a potential case of CAR.6 The observed data seem consistent with what has been previously describe in literature: paraneoplastic syndromes affecting the CNS benefit from IVIG therapy when it is early applied, and it is more effective during the first 4 weeks after the onset of symptoms.4 However, the final clinical benefit varies and may only come to and stabilization.4 A limitation of the review is that the determination of the visual field, which is more objective than visual acuity is not available after treatment in all cases.