title: Abnormalities of visual-evoked potentials and pupillary light reflexes in a family with autosomal dominant occult macular dystrophy.
abstract: 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.
authors.forename: Takashi
authors.initials: T
authors.lastname: Okuno
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Oku
authors.forename: Mineo
authors.initials: M
authors.lastname: Kondo
authors.forename: Yozo
authors.initials: Y
authors.lastname: Miyake
authors.forename: Jun
authors.initials: J
authors.lastname: Sugasawa
authors.forename: Takashi
authors.initials: T
authors.lastname: Utsumi
authors.forename: Tsunehiko
authors.initials: T
authors.lastname: Ikeda
mesh_terms: D000328:Adult; D004596:Electroretinography; D005074:Evoked Potentials, Visual / Q000235:genetics*; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008268:Macular Degeneration / Q000175:diagnosis / Q000235:genetics*; D008875:Middle Aged; D009901:Optic Nerve Diseases / Q000175:diagnosis / Q000235:genetics; D010375:Pedigree; D012025:Reflex, Pupillary / Q000235:genetics*