In general, the age at manifestation of the
disease is between 6 and 20 years with decreased central vision being the
first symptom, although some patients do not suffer from any symptoms (loss
of color vision, loss of peripheral vision, photophobia, paracentral
scotoma, night blindness) until the age of 40 years. Fundus flavimaculatus
is the descriptive term for this macular degeneration surrounded by
white-yellowish flecks and spots distributed all over the fundus, although
some researchers consider fundus flavimaculatus to be an allelic variant of
Stargardt disease with differences in age at onset (between 17 and 60
years), severity, and clinical course (more progressive). The rapidly
progressive disease occurs usually bilaterally and symmetrically and results
in legal blindness in 50% of patients by the age of 50 years. No
effective treatment is available.