Paraneoplastic retinopathies
Abstract
Background: Two types of retinopathy are known to be associated with patients with malignancies: cancer associated retinopathy-CAR and cutaneus melanoma associated retinopathy-MAR. Autoantibodies against recoverin or against α -enolase have been described in CAR, and autoatibodies against ON-bipolar cells are formed in MAR. An outline of the pathogenetic mechanisms and an example of each cases disorder are presented.
Case reports: In both patients, the following were performed: complete clinical examination, visual acuity, visual fields, fluorescein angiography and electroretinography. The first patient was a 78 year-old man with epidermoid carcinoma of the the lung and metastases of liver and kidney suffered rapidly progressive bilateral visual loss. He suffered complete loss of vision (amaurosis with Anton’s syndrome). Retinal examination showed severe arterial narrowing with lipid plaques and age-related pigmentary changes. The ERG was nondetectable, and the diagnosis was CAR. The second patient was a 62 years old man who presented with acute onset of bilateral night blindness, photopsia and shimmering four years after excision of a cutaneus malignant melanoma. Visual acuity was 0.5 in the right eye and 0.8 in the left eye. The visual fields showed constriction to 15° in both eyes on Goldmann perimetry. The retinal examination was normal. ERG maximal response was electronegative, with normal a-wave amplitude and reduced b-wave. ON-OFF ERG showed loss of ON-response b-wave and normal OFF-response d-wave, typical for MAR.
Conclusions: CAR and MAR are rare paraneoplastic sindromes, that are characterised by rapid onset of acquired bilateral visual loss and night blindness. There are usually shimmering photopsias in MAR and fundus examination is normal. The fundus is initially normal in CAR, but becomes abnormal as the disorder progresses. Typical changes in ERG may precede the presentation of malignancy and therefore awareness of this complication is important.
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References
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