Historical Summary
- A 56-year-old white female presents for yet another opinion concerning her visual disorder that began in 1986. She was first evaluated more than 2 decades earlier because of a blind spot and flashes in the right eye.
- Best corrected VA at that time was 20/25 OD and 20/20 OS.
- The fundus exam was essentially normal but several fine pigmentary changes were noted by one retinal specialist in the far periphery OD.
- Visual fields back in 1988 revealed a large and dense superior arcuate scotoma OD with substantial nasal loss that did not respect the horizontal meridian. MD was -18 dB OD. Blind spot enlargement was noted OS with MD -6 dB.
- The fundus was completely normal ophthalmoscopically in the areas corresponding to the field loss and hence the term “occult” was appropriate.
- Color vision was normal in 1988 and ERGs were reduced in amplitude right greater than left with borderline implicit times.
- Several retinal specialists concluded that all the findings supported a mild inflammatory process but not a degenerative disorder. Several years later, she was evaluated by Dr. Donald Gass at Bascom Palmer in Miami.
- Her acute onset, a non-contributory battery of tests for systemic disease, blind spots, reduced vision with photopsia in the right eye and a reduced ERG convinced Dr. Gass that she had a disorder similar to a dozen other patients.
- In 1993, Gass first reported these 13 patients as the first in the world’s literature with AZOOR (Acute Zonal Occult Outer Retinopathy).1
- Our patient was case number 12 in the original report by Dr. Gass and was also presented by Gass in the Donders Lecture in Holland in 1992.1
- Follow-up by Gass, his colleagues and many others over the past 2 decades all “confirmed” AZOOR as the diagnosis.