Macular sensitivity in patients with congenital stationary night-blindness

Br J Ophthalmol. 2019 Oct;103(10):1507-1510. doi: 10.1136/bjophthalmol-2018-313072. Epub 2018 Dec 20.

Abstract

Aim: To evaluate and correlate mean light sensitivity thresholds (MLST) in patients with congenital stationary night-blindness (CSNB) in comparison with healthy subjects using microperimetry (MP1).

Methods: Eleven patients with CSNB and 13 healthy subjects were compared. In all subjects, static threshold perimetry was performed using MP1 evaluating the central 6 mm of the retina. This central retinal area was divided into three rings through using the ETDRS grid algorithm with an innermost (1 mm), inner (3 mm) and outer ring (6 mm). The MLSTs were acquired in nine sectors of the ETDRS grid. A comparison of MLST was performed between both groups using a t-test (significance level p<0.005).

Results: A significant reduction of MLST in the fovea (innermost ring, 1 mm) was observed for patients with CSNB (7.2±3.90 dB) in comparison to healthy subjects (19.7±0.75; p<0.0001). Similarly, comparison of MLST in all other sectors (superior/inferior/temporal and nasal) within the inner and outer ring revealed a statistically significant reduction in patients with CSNB compared with healthy subjects (p<0.001).

Conclusions: Examination of macular retinal sensitivity intensity using MP1 revealed for the first time a significant reduction of MLST within the central 6 mm of the retina in patients with CSNB compared with healthy subjects. This finding supports MP1 as an additional diagnostic tool when examining patients with retinal dysfunctions such as CSNB.

Keywords: degeneration; field of vision; macula; retina; vision.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Calcium Channels, L-Type / genetics
  • Electroretinography
  • Eye Diseases, Hereditary / diagnosis
  • Eye Diseases, Hereditary / genetics
  • Eye Diseases, Hereditary / physiopathology*
  • Female
  • Genetic Diseases, X-Linked / diagnosis
  • Genetic Diseases, X-Linked / genetics
  • Genetic Diseases, X-Linked / physiopathology*
  • Humans
  • Light*
  • Macula Lutea / physiopathology*
  • Male
  • Myopia / diagnosis
  • Myopia / genetics
  • Myopia / physiopathology*
  • Night Blindness / diagnosis
  • Night Blindness / genetics
  • Night Blindness / physiopathology*
  • Proteoglycans / genetics
  • Sensory Thresholds / physiology*
  • Visual Acuity / physiology
  • Visual Field Tests
  • Visual Fields / physiology

Substances

  • CACNA1F protein, human
  • Calcium Channels, L-Type
  • NYX protein, human
  • Proteoglycans

Supplementary concepts

  • Night blindness, congenital stationary