Elsevier

Vision Research

Volume 42, Issue 11, May 2002, Pages 1475-1483
Vision Research

Abnormalities of the long flash ERG in congenital stationary night blindness of the Schubert–Bornschein type

https://doi.org/10.1016/S0042-6989(02)00068-8Get rights and content
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Abstract

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.

Keywords

X-chromosomal nightblindness
ON–OFF ERG
Oscillatory potentials

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Supported by the grants SFB 430/C2 of the Deutsche Forschungsgemeinschaft (DFG) and fortüne Nr 514 and Nr 708 of the Eberhart-Karls Universität, Tübingen, Germany.