Four clinical stages:
Prodromal stage of neurologic and auditory disturbances
Bilateral posterior uveitis + diffuse granulomatous choroiditis
Optic disc oedema
Exudative RD – multifocal and shallow RD
Retinal +/- choroidal folds
Patchy hypofluorescence (delayed choroidal filling)
Early frames – Multiple punctate hyperfluorescent dots at RPE.
Late frames – hyperfluorescent dots enlarge and stains surrounding SRF (extent of exudative RD) + optic disc hyperfluorescence.
Alternate hypofluorescent / hyperfluorescent bands – indicate folds.
Variable degree of choroidal depigmentation
Sunset glow fundus (loss of choroidal melanocytes)
Focal RPE atrophy – nummular depigmented round well-defined chorioretinal scars at mid-peripheral.
Hyperpigmented linear focal lesions – RPE migration/clumping.
Cutaneous changes – vitiligo, alopeica or poliosis of eyelashes/brows.
OCT to monitor response to treatment – SRF / RPE undulations
Treat complications – cataract, glaucoma, CNV or subretinal fibrosis accordingly.