Stargardt's disease/fundus flavimaculatus: psychophysical and electrophysiologic results. 1993

R Itabashi, and O Katsumi, and M C Mehta, and R Wajima, and M Tamai, and T Hirose
Schepens Eye Research Institute, Boston, MA 02114.

Retinal functions were analyzed psychophysically and electrophysiologically in 73 patients (146 eyes) with Stargardt's disease/fundus flavimaculatus. Patients were classified into types 1, 2, 3, and 4; patients with type 3 were subdivided into 3E and 3L (early and late onset of initial symptoms). Most had visual acuity (VA) of 20/200 or greater at initial testing. VA declined 0.25 octave/year during follow-up (mean, 6.1 years). Four of 16 patients (25%) older than 40 years had VA of 20/200 or less in the stronger eye. Psychophysical tests (flicker profile, central scotoma, dark adaptation) showed variable degrees of abnormalities. Electrophysiological tests showed significant photopic b-wave amplitude decreases, particularly in type 3E (42.2% of normal). The electro-oculogram light peak/dark trough ratio was abnormal in 60 of 132 eyes (45.5%), especially in type 3E (25 of 34 eyes, 73.5%). Visual prognosis and overall visual function varied depending upon disease type, location of retinal lesions, and age of onset. In Type 3E, overall retinal function was poorest and accompanied by the most severe decline of central vision and function in the surrounding macula.

UI MeSH Term Description Entries
D008268 Macular Degeneration Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms. Maculopathy,Maculopathy, Age-Related,Age-Related Macular Degeneration,Age-Related Maculopathies,Age-Related Maculopathy,Macular Degeneration, Age-Related,Macular Dystrophy,Maculopathies, Age-Related,Age Related Macular Degeneration,Age Related Maculopathies,Age Related Maculopathy,Age-Related Macular Degenerations,Degeneration, Macular,Dystrophy, Macular,Macular Degeneration, Age Related,Macular Degenerations,Macular Dystrophies,Maculopathies,Maculopathy, Age Related
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D011601 Psychophysics The science dealing with the correlation of the physical characteristics of a stimulus, e.g., frequency or intensity, with the response to the stimulus, in order to assess the psychologic factors involved in the relationship. Psychophysic
D012160 Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the OPTIC NERVE and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the CHOROID and the inner surface with the VITREOUS BODY. The outer-most layer is pigmented, whereas the inner nine layers are transparent. Ora Serrata
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D004596 Electroretinography Recording of electric potentials in the retina after stimulation by light. Electroretinographies
D005260 Female Females
D005451 Fluorescein Angiography Visualization of a vascular system after intravenous injection of a fluorescein solution. The images may be photographed or televised. It is used especially in studying the retinal and uveal vasculature. Fluorescence Angiography,Fundus Fluorescence Photography,Angiography, Fluorescein,Angiography, Fluorescence,Fluorescence Photography, Fundus,Photography, Fundus Fluorescence

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