Cross-sectional study of visual acuity and electroretinogram in two types of dominant drusen

Détails

ID Serval
serval:BIB_F79957725002
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Cross-sectional study of visual acuity and electroretinogram in two types of dominant drusen
Périodique
Investigative Ophthalmology and Visual Science
Auteur(s)
Gerber  D. M., Munier  F. L., Niemeyer  G.
ISSN
0146-0404 (Print)
Statut éditorial
Publié
Date de publication
02/2003
Volume
44
Numéro
2
Pages
493-6
Notes
Comparative Study
Journal Article
Research Support, Non-U.S. Gov't --- Old month value: Feb
Résumé
PURPOSE: To compare the changes with increasing age of ERG parameters in relation to clinical data in two distinct phenotypes of genetically determined, dominantly inherited macular drusen: malattia leventinese (ML) and Zermatt macular dystrophy (ZMD). METHODS: Ganzfeld rod- and cone-electroretinograms (ERGs) from 15 patients affected with ML and 14 patients with ZMD and clinical data were analyzed retrospectively. The patients' ages ranged from 20 to 77 years in the ML group and from 9 to 74 years in the ZMD group. RESULTS: Both inherited macular degenerations caused a marked decrease in visual acuity, the latest after age 65. Most patients with ML retained good visual function (0.8-1.0) until the fifth decade, followed by a rapid decrease in the fifth or sixth decade. ZMD is characterized by a relatively continuous decrease in visual acuity with increasing age. Morphologically, in the juvenile stages in both entities, drusen were observed at the posterior pole. Rod-driven and cone-driven ERG b-wave amplitudes decreased nearly linearly in ML and ZMD in accord with the normal loss of amplitude with increasing age. Implicit times of cone b-waves for ML increased markedly with age, whereas in ZMD the values were always prolonged beyond the normal range with a slight increase with age. CONCLUSIONS: In terms of visual acuity, the progression of both dominantly inherited macular dystrophies is quite different. This is not reflected in the amplitudes of the b-waves in the Ganzfeld ERGs, which decrease normally for both entities. Implicit times of the cone-b waves were more markedly prolonged in ML compared with ZMD. In-depth longitudinal documentation of the natural course of those dominantly inherited macular diseases should facilitate patient counseling.
Mots-clé
Adolescent Adult Aged Aging/physiology Child Cross-Sectional Studies Dark Adaptation Disease Progression Electroretinography Humans Middle Aged Retina/*physiology Retinal Drusen/classification/*genetics/*physiopathology Retrospective Studies Visual Acuity/*physiology
Pubmed
Web of science
Création de la notice
28/01/2008 13:53
Dernière modification de la notice
03/03/2018 22:50
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