severe early-childhood-onset retinal dystrophy
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.
MONDO:0019353
Stargardt disease
is_a
MONDO:0800406
ABCA4-related retinopathy
is_a
EOSRD
exact
SECORD
exact
STGD1
related
Stargardt disease 1
related
Stargardt disease type 1
exact
early-onset severe retinal dystrophy
exact