Severe early-childhood-onset retinal dystrophy
Severe early-childhood-onset retinal dystrophy
Definition
Severe early childhood onset retinal dystrophy (SECORD) is an inherited retinal dystrophy, characterized by a severe congenital night blindness, progressive retinal dystrophy and nystagmus. Best corrected visual acuity can reach 0.3 in the first decade of life and can pertain well into the second decade of life. Blindness is often complete by the age of 30 years. An overlap with Leber congenital amaurosis (LCA) occurs when patients are characterized by their visual acuity and panretinal dystrophy.
Also known as early-onset severe retinal dystrophy, EOSRD, SECORD, Stargardt disease type 1 — per MONDO