Abnormalities of the long flash ERG in congenital stationary night blindness of the Schubert-Bornschein type.
We investigated abnormalities of the retinal cone ON- and OFF-pathways in 24 males with Schubert-Bornschein congenital stationary night blindness (CSNB). Substantial differences were found between both CSNB types. In incomplete type, a-, b- and d-waves were reduced and delayed, whereas in complete type only the b-wave showed significant changes. Oscillatory potentials (OPs) were not discernible from noise in incomplete CSNB and showed significant peak alterations of the ON-OPs only in complete CSNB. In the complete CSNB type, the ON pathway appeared to be mainly affected. In the incomplete CSNB form marked involvement of both the ON and the OFF pathways was noted.
Langrová
Hana
H
Department of Pathophysiology of Vision and Neuroophthalmology, University Eye Hospital, 72075 Tübingen, Germany. langrovah@lfhk.cuni.cz
Gamer
Daphne
D
Friedburg
Christoph
C
Besch
Dorothea
D
Zrenner
Eberhart
E
Apfelstedt-Sylla
Eckart
E
D000293:Adolescent; D000328:Adult; D004596:Electroretinography; D008040:Genetic Linkage; D006801:Humans; D008297:Male; D008875:Middle Aged; D009755:Night Blindness / Q000151:congenital* / Q000235:genetics / Q000503:physiopathology; D010775:Photic Stimulation / Q000379:methods; D017949:Retinal Cone Photoreceptor Cells / Q000503:physiopathology; D014960:X Chromosome