No known gender predilection exists. The condition often is not detected until much later in the disease because visual acuity may remain good for many years. The atrophic stage usually occurs after age 40. Many individuals initially are asymptomatic, with fundus lesions noted on examination. These symptoms may worsen if the disease progresses to the atrophic stage.
Some carriers have a normal examination and remain asymptomatic.
Findings usually are bilateral and can be asymmetric. Not all individuals progress beyond the early stages. Other individuals can skip from the earliest stages to an atrophic-appearing macula. Unilateral findings and multifocal lesions have been described. Yellow material can break through the RPE and accumulate in the subretinal space in a cyst with a fluid level formed.