Summary:
The purpose of this study is to refer three case reports of adult Best’s macular
dystrophy. The group consists of three patients, one woman and two men, aged 49
– 67 years. In all patients of the group only one lesion localized in the macula of
the affected eye was noticed. In two patients, both eyes were affected; in the last
one the finding was unilateral. All lesions had typical clinical finding, which
corresponded to the vitelliform stage of the Best’s disease.
All patients underwent complex ophthalmologic examination including medical
history taking, visual acuity for far and for near, intraocular pressure measurement,
examination of the visual fields, retinal examination with dilated pupils,
optical coherent tomography, and electroretinogram, and electro-oculogram as
well. In all three patients, also the fundus fluorescein angiography was performed.
The decrease of the visual acuity closely correlated to the seriousness and the
duration of the retinal finding. The vitelliform lesion was located always in the
macula, in sub- or juxtafoveolar localization. In none of those patients the authors
could find the genetic linkage or the transmission of the disease to the relatives.
Key words:
Best’s vitelliform macular dystrophy, adult form, autosomal dominant
inheritance, pathologic electro-oculogram
|