BUTTERFLY-SHAPED MACULAR DYSTROPHY - LONGITUDINAL CASE-STUDY OF 2 SIBLINGS

Authors
PETRACCI M PANNINI S FREZZOTTI R
Citation
M. Petracci et al., BUTTERFLY-SHAPED MACULAR DYSTROPHY - LONGITUDINAL CASE-STUDY OF 2 SIBLINGS, Ophthalmic paediatrics and genetics, 14(4), 1993, pp. 165-175
Citations number
NO
Categorie Soggetti
Pediatrics,Ophthalmology
Journal title
Ophthalmic paediatrics and genetics → ACNP
ISSN journal
01676784
Volume
14
Issue
4
Year of publication
1993
Pages
165 - 175
Database
ISI
SICI code
0167-6784(1993)14:4<165:BMD-LC>2.0.ZU;2-G
Abstract
Two brothers with butterfly-shaped macular dystrophy (BSMD) are report ed. The anatomical and functional data were analyzed in 11 family memb ers. Progressive photoreceptor dysfunction, supported by ERG abnormali ties, was documented in both patients. The progression of the disease was observed over a period of seven years. The present observations em phasize that BSMD, at least in some cases, can be a chronic progressiv e disorder with secondary involvement of the photoreceptors.