Corpus ID: 22905609

Congenital stationary night blindness: an animal model.

  title={Congenital stationary night blindness: an animal model.},
  author={Donald A. Witzel and E. Smith and R. D. Wilson and Gustavo D Aguirre},
  journal={Investigative ophthalmology \& visual science},
  volume={17 8},
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… Expand
Cone function in congenital nyctalopia
A patient with congenital stationary night blindness transmitted as an autosomal recessive trait was studied with several tests of electrical function as well as a variety of psychophysical procedures, and the focal ERG of the CSNB patient showed normal amplitude and normal phase delays, supporting the idea that the focalERG samples primarily cone photoreceptor activity. Expand
Congenital stationary night blindness with myopia: a clinicopathologic study
It is proposed that the cause of night blindness in congenital stationary cases with Schubert-Bornschein type ERG may be related to the mechanisms inhibitory to cells of the bipolar layer. Expand
Congenital stationary night blindness in a Thoroughbred and a Paso Fino.
In the Paso Fino, severe vision impairment from birth to approximately 1 year of age in both dim and bright light situations led to humane euthanasia and histopathologic confirmation of the disorder. Expand
Spontaneous occurrence of a potentially night blinding disorder in guinea pigs
A pedigree of guinea pigs is presented where, as a result of a consanguineous mating and subsequent selective breeding, a new and naturally occurring animal model of a rod disorder is developed and it is believed that this condition is inherited as an autosomal recessive trait in the guinea pig population. Expand
The Briard dog: a new animal model of congenital stationary night blindness.
It appears that the Briard dog may become a valuable model of human CSNB, where rhodopsin concentration and regeneration were found to be normal, suggesting a disturbed transduction mechanism. Expand
Clinical and electroretinographic characteristics of congenital stationary night blindness in the Appaloosa and the association with the leopard complex.
ERG abnormalities support the hypothesis that CSNB is caused by a defect in neural transmission through the rod pathway involving the inner nuclear layer in Appaloosa horses. Expand
Congenital stationary night blindness
  • M. Haim
  • Medicine
  • Acta ophthalmologica
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The provisional findings seem to indicate that 3 genetic variants are present in the Danish population, and the real prevalence is estimated considerably higher than 17 out of 5 million. Expand
A Naturally Occurring Canine Model of Autosomal Recessive Congenital Stationary Night Blindness
The results indicate involvement of a yet unknown gene in this canine model of complete CSNB, which is similar to the Schubert-Bornschein form of completeCSNB in humans. Expand
ERGs in female carriers of incomplete Congenital Stationary Night Blindness (I-CSNB) A family report
These last two ERG results suggest a possible modifications of synaptic transmission at a post-receptoral site (outer plexiform layer or involvement of the bipolar pathways) in these two carriers. Expand
Presentation of TRPM1-Associated Congenital Stationary Night Blindness in Children
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Photopic abnormalities in congenital stationary nightblindness.
Fourteen patients with congenital nightblindness, representing all three hereditary types, were studied, and all were found to have some type of photopic abnormality. The photopic abnormalitiesExpand
Visual functions in congenital night blindness.
Measurements of spectral sensitivity measurements in the dark-adapted peripheral retina indicate that rod signals, although greatly attenuated, may reach cortical centers and measurements of visual threshold as a function of stimulus area showed that the integrative properties of the retina are normal. Expand
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  • Chemistry, Medicine
  • UCLA forum in medical sciences
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Three major neural components of the mammalian ERG have been demonstrated, including the late RP, b -wave, and d.c. component, which give an adequate explanation of all main features of the cone ERG, plus the c -wave from the pigment epithelium. Expand
Synaptic organization of the frog retina: an electron microscopic analysis comparing the retinas of frogs and primates
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  • Biology, Medicine
  • Proceedings of the Royal Society of London. Series B. Biological Sciences
  • 1968
These findings suggest that in the inner plexiform layer of the frog: bipolar terminals synapse primarily with amacrine processes; (2) amacine processes synapse extensively with the processes of other isacrine cells; and (3) ganglion cells are driven primarily by the amACrine cells. Expand
Electroretinography of congenital night blindness in an Appaloosa filly
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