- More prevalent in Asians, i.e. Japanese and Chinese or Italians.
- Symptoms: mild to moderate decrease in vision, nyctalopia, paracentral scotoma.
- Slowly progressive disease
- Intraretinal glittering yellow-white crystalline deposits, which is most numerous in the posterior pole
- Multiple areas of geographic atrophy of the RPE and sclerosis or loss of choriocapillaris
- Paralimbal conjunctival or corneal crystalline depositions may be found
- Fluorescein angiography demonstrates patchy areas of blocked fluorescence by the crystals, multiple areas of RPE depigmentation and choriocapillaris nonperfusion.
- Differential diagnoses include: oxalosis, cystinosis, Sjögren-Larsson Syndrome, calcified drusen, tamoxifen intoxication, autosomal dominant crystalline dystrophy.