title: Abnormal dark adaptation and rhodopsin kinetics in Sorsby's fundus dystrophy.
abstract: Scotopic visual thresholds and time courses for dark adaptation were determined in eight patients with Sorsby's fundus dystrophy. Rhodopsin regeneration also was recorded in two. All patients had poor night vision and a visible yellow deposit at the level of Bruch's membrane that was confluent in the posterior pole. In retinal regions with the yellow deposit, scotopic thresholds were elevated, the rod-cone break was delayed or indistinct, the time courses for the rod portion of the dark adaptation curve was prolonged, and rhodopsin regeneration was slow in the one patient in whom measurements were made. In regions of ophthalmoscopically normal retina, dark adaptation was affected minimally, and in one patient, rhodopsin was regenerated at a normal rate. It was hypothesized that the abnormal dark adaptation and rhodopsin kinetics might be caused by reduced metabolic exchange across a thickened Bruch's membrane.
authors.affiliation: Department of Clinical Ophthalmology, Institute of Ophthalmology, London, England.
authors.forename: R L
authors.initials: RL
authors.lastname: Steinmetz
authors.forename: P C
authors.initials: PC
authors.lastname: Polkinghorne
authors.forename: F W
authors.initials: FW
authors.lastname: Fitzke
authors.forename: C M
authors.initials: CM
authors.lastname: Kemp
authors.forename: A C
authors.initials: AC
authors.lastname: Bird
mesh_terms: D000328:Adult; D016570:Bruch Membrane / Q000378:metabolism / Q000473:pathology; D003623:Dark Adaptation; D005654:Fundus Oculi; D006801:Humans; D007700:Kinetics; D008875:Middle Aged; D009755:Night Blindness / Q000378:metabolism / Q000473:pathology; D012162:Retinal Degeneration / Q000378:metabolism* / Q000473:pathology; D012243:Rhodopsin / Q000378:metabolism*; D012684:Sensory Thresholds; D014786:Vision Disorders / Q000378:metabolism / Q000503:physiopathology
chemical_list: D012243:Rhodopsin