- More prevalent in Asians, i.e. Japanese and Chinese or Italians.
- Clinical features:
- Symptoms: mild to moderate decrease in vision,
nyctalopia, paracentral scotoma.
- Signs:
- 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.
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