Retina On Fire: A Challenging Case of Recurrent Neuroretinitis in a 52-Year-Old Female
Anika Zahoor1, Eric Seachrist1, Subtain Ali2, Abdul Tarabishy2
1Department of Neurology, 2Department of Neuroradiology, West Virginia University
Objective:
To raise awareness among neurologists about the diagnosis, management, and treatment of Chronic Recurrent Idiopathic Neuroretinitis (RINR), a rare condition causing attacks of severe vision loss.
Background:
RINR can mimic severe vision loss events, such as optic neuritis seen in NMO or MOG disorders. While acute neuroretinitis is typically treated with antimicrobials, the treatment for RINR is not well established.
Design/Methods:
A case study.
Results:

A 52-year-old white female with a history of neuroretinitis, diagnosed in 2022, was admitted due to progressive vision changes in her left eye. Symptoms included white spots, a dark curtain effect, and reduced visual acuity. Visual acuity was diminished in both eyes, with chronic impairment in the right and acute impairment in the left. An afferent pupillary defect was also present. These symptoms developed over several days and did not improve with steroids or doxycycline.

Ophthalmologic examination revealed blurry optic nerve margins, elevation, a single peripapillary hemorrhage, and subretinal fluid under the macula, but no macular exudates. MRI showed nonspecific white matter lesions in the right parietal lobe and optic nerve atrophy, more pronounced on the right. CSF analysis was unremarkable.

A comprehensive workup for infections and autoimmune conditions (Lyme disease, syphilis, HIV, HSV, Bartonella, and MOG antibodies) returned negative results. Given the new left eye symptoms, RINR was suspected. An outpatient follow-up examination revealed a macular star in the left eye, confirming the suspicion of RINR.

A trial of high-dose steroids, tapered over time, along with doxycycline, was initiated, and immunosuppressants were planned.

Conclusions:
RINR can mimic conditions commonly encountered, such as severe optic neuritis, making it essential for neurologists to be aware of it. Additional research is needed to determine the best long-term management of this rare disease.
10.1212/WNL.0000000000212539
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