This report describes general characteristics of the group of diseases classified as congenital stationary night blindness (CSNB). Congenital stationary night blindness is a genetically heterogeneous disorder, with multiple genes and mapped loci. A listing of CSNB subtypes, as defined by OMIM, may be found in the table below, with links to more detailed reports for subtypes that have been investigated using fly models.
[updated Feb. 2024 by FlyBase; FBrf0222196]
Congenital stationary night blindness (CSNB) is a clinically and genetically heterogeneous group of nonprogressive retinal disorders that can be characterized by impaired night vision, decreased visual acuity, nystagmus, myopia, and strabismus. CSNB can be classified into 2 groups based on electroretinography (ERG) findings: the Schubert-Bornschein type is characterized by an ERG in which the b-wave is smaller than the a-wave, whereas the Riggs type is defined by proportionally reduced a- and b-waves. In addition, Schubert-Bornschein CSNB is associated with decreased visual acuity, myopia, and nystagmus, whereas in Riggs CSNB patients have visual acuity within the normal range and no symptoms of myopia and/or nystagmus (summary by Riazuddin et al., 2010, pubmed:20850105). Additionally, Schubert-Bornschein CSNB can be subdivided into 'complete' and 'incomplete' forms (summary by Riazuddin et al., 2010), pubmed:20850105) [from MIM:310500; 2024.02.21]
CSNB is a genetically heterogeneous disorder, with several causative genes and mapped loci. [from MIM:310500; 2024.02.21]