[A case of cancer-associated retinopathy rapidly leading to blindness with a unique pupillary light reflex]

We herein report a case of cancer-associated retinopathy (CAR) associated with malignant lymphoma, beginning with uveitis and rapidly leading to blindness.

A 54-year-old woman presented with a seven-day history of bilateral progressive vision loss. She had no light perception OU. She had keratic precipitates, anterior chamber cells and fine vitreous opacities OU. Fundus examination showed no signs of inflammation, but both a narrowing of the retinal arterioles and non-active diabetic retinopathy were observed. The electroretinogram was extinguished, and optical coherence tomography showed thinning of the outer retinal layer. Systemic examination revealed malignant lymphoma. Anti-recoverin antibody was detected in the patient's serum. Based on these observations, the patient was diagnosed as having CAR. She was treated with steroid pulse therapy, but her visual acuity did not improve. Although she had no light perception, her pupils showed slow and persistent constriction in reaction to strong and long light stimulation during the follow-up period.

In certain circumstances CAR can progress rapidly and lead to blindness within a few days. We observed a delayed, slow, and persistent pupillary light reflex in a CAR patient with no light perception, presumably due to the intrinsic photoactivation of the melanopsin-containing retinal ganglion cells in the uninvolved inner retina.