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Congenital stationary night blindness: an animal model.

Abstract: Electroretinographic studies of myctalopic Appaloosa horses demonstrated photopic and scotopic abnormalities similar to those in humans with congenital stationary night blindness (CSNB) of the Schubert-Bornschein type. The phototopic abnormalities consisted of reduced b-wave amplitudes and slower than normal b-wave implict time. The dark-adapted ERG's consisted of a simple negative potential; the scotopic b-wave was nonrecordable. However, a normal c-wave was present in the dark-adapted response. Histologic studies demonstrated no structural abnormalities that could explain the functional defect.
Publication Date: 1978-08-01 PubMed ID: 308060
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  • Journal Article
  • Research Support
  • U.S. Gov't
  • P.H.S.

Summary

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This study investigates night blindness in horses, using Appaloosa horses with myctalopia as a model. It was found that their photopic and scotopic abnormalities were similar to those seen in humans with congenital stationary night blindness (CSNB) of the Schubert-Bornschein type, yet no structural abnormalities were found that could explain the functional defect.

Understanding Night Blindness in Horses

  • The researchers focused on Electroretinographic (ERG) studies of Appaloosa horses afflicted with myctalopia, a condition that impairs night vision, popularly known as night blindness.
  • Through ERG, the researchers studied the horse’s response to light, investigating both photopic (light adapted) and scotopic (dark adapted) conditions.
  • The study focused on two types of waves in the Electroretinography: the b-wave, which evaluates the functionality of the inner retina, and the c-wave, which assesses the integrity of the outer retina.

Comparison to Human Night Blindness

  • Interestingly, the studies revealed photopic and scotopic abnormalities in these horses similar to those found in humans suffering from a specific type of congenital stationary night blindness (CSNB) – the Schubert-Bornschein type.
  • In both humans and these horses, the phototopic abnormalities manifest as reduced b-wave amplitudes and slower than normal b-wave implicit time, implicating the diminished functionality of the inner retina.
  • Similarly, in dark-adapted conditions, the scotopic b-wave was nonrecordable, further showcasing the disrupted retinal function in the presence of night blindness.

Notable Findings and Limitations

  • However, a study found that a normal c-wave was present in the dark-adapted response, suggesting that the outer retina’s integrity is maintained in the face of night blindness.
  • This was surprising as the c-wave generally declines in retinal degenerative conditions, but in this case it was preserved despite the vision defect.
  • Moreover, histologic studies, which involve the study of the microscopic structure of tissues, found no structural abnormalities that could explain the functional defect, suggesting that the disturbances are likely arising at a functional or physiological level rather than a gross structural one.

Cite This Article

APA
Witzel DA, Smith EL, Wilson RD, Aguirre GD. (1978). Congenital stationary night blindness: an animal model. Invest Ophthalmol Vis Sci, 17(8), 788-795.

Publication

ISSN: 0146-0404
NlmUniqueID: 7703701
Country: United States
Language: English
Volume: 17
Issue: 8
Pages: 788-795

Researcher Affiliations

Witzel, D A
    Smith, E L
      Wilson, R D
        Aguirre, G D

          MeSH Terms

          • Adaptation, Physiological
          • Animals
          • Dark Adaptation
          • Disease Models, Animal
          • Electroretinography
          • Female
          • Horse Diseases / congenital
          • Horses
          • Light
          • Male
          • Night Blindness / congenital
          • Night Blindness / veterinary
          • Retina / pathology
          • Retina / physiopathology
          • Retina / ultrastructure

          Citations

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