[Multimodal diagnostic of CSNB1 with NYX gene mutation]

Ophthalmologe. 2019 Dec;116(12):1207-1211. doi: 10.1007/s00347-019-0886-2.
[Article in German]

Abstract

This article presents the case of a young male patient with complete congenital stationary night blindness (CSNB1). The informative value of the general medical history and clinical findings for the diagnosis was impaired due to language barriers and low compliance. Full-field electroretinography and optical coherence tomography help to define particular hereditary retinal dystrophies. Molecular genetic analysis by next generation sequencing as a part of multimodal diagnostics finally uncovered a rare, causal missense mutation in the nyctalopin (NYX) gene.

Keywords: CSNB1; Full-field electroretinography; Multimodal diagnostic; NYX gene mutation; OCT.

Publication types

  • Case Reports

MeSH terms

  • Electroretinography
  • Eye Diseases, Hereditary* / diagnostic imaging
  • Eye Diseases, Hereditary* / genetics
  • Genetic Diseases, X-Linked* / diagnostic imaging
  • Genetic Diseases, X-Linked* / genetics
  • Humans
  • Male
  • Mutation
  • Myopia* / diagnostic imaging
  • Myopia* / genetics
  • Night Blindness* / diagnostic imaging
  • Night Blindness* / genetics
  • Proteoglycans* / genetics

Substances

  • NYX protein, human
  • Proteoglycans

Supplementary concepts

  • Night blindness, congenital stationary