This 6 year old girl has had poor acuity her entire life. At a few months of age, her parents noticed her nystagmus and sought medical attention.

The clinical features supporting the diagnosis of severe early childhood onset retinal dystrophy (SECORD) in this patient include: nystagmus noted shortly after birth; reduced acuity for her entire life, a non-recordable ERG, legal blindness before age 10 (visual field criteria), a relatively normal fundus appearance with pink optic disks; hyperopia; and, normally sighted parents.