Abnormalities of visual-evoked potentials and pupillary light reflexes in a family with autosomal dominant occult macular dystrophy.

Occult macular dystrophy (OMD) is an unusual inherited or sporadic macular dystrophy characterized by an essentially normal appearing ocular fundus and normal ocular circulation determined by fluorescein angiography but with a progressive decrease of visual acuity. We present a family with OMD who have a delay in the implicit times of both the visual-evoked potentials and the pupillary light reflexes. Because these delays are usually indicative of optic nerve disorders, deciding on a diagnosis between OMD and optic nerve disorder should be done carefully. We are not aware of any studies that have reported these findings in patients with OMD.