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Author (up) Bijveld, M.M.C.; van Genderen, M.M.; Hoeben, F.P.; Katzin, A.A.; van Nispen, R.M.A.; Riemslag, F.C.C.; Kappers, A.M.L. url  doi
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  Title Assessment of night vision problems in patients with congenital stationary night blindness Type Journal Article
  Year 2013 Publication PloS one Abbreviated Journal PLoS One  
  Volume 8 Issue 5 Pages e62927  
  Keywords Vision; Adolescent; Adult; Case-Control Studies; Child; *Dark Adaptation; Electroretinography; Eye Diseases, Hereditary/*physiopathology; Female; Genetic Diseases, X-Linked/*physiopathology; Humans; Light; Male; Middle Aged; Myopia/*physiopathology; Night Blindness/*physiopathology; *Night Vision; *Pattern Recognition, Visual; Surveys and Questionnaires; *Visual Acuity; Visual Fields  
  Abstract Congenital Stationary Night Blindness (CSNB) is a retinal disorder caused by a signal transmission defect between photoreceptors and bipolar cells. CSNB can be subdivided in CSNB2 (rod signal transmission reduced) and CSNB1 (rod signal transmission absent). The present study is the first in which night vision problems are assessed in CSNB patients in a systematic way, with the purpose of improving rehabilitation for these patients. We assessed the night vision problems of 13 CSNB2 patients and 9 CSNB1 patients by means of a questionnaire on low luminance situations. We furthermore investigated their dark adapted visual functions by the Goldmann Weekers dark adaptation curve, a dark adapted static visual field, and a two-dimensional version of the “Light Lab”. In the latter test, a digital image of a living room with objects was projected on a screen. While increasing the luminance of the image, we asked the patients to report on detection and recognition of objects. The questionnaire showed that the CSNB2 patients hardly experienced any night vision problems, while all CSNB1 patients experienced some problems although they generally did not describe them as severe. The three scotopic tests showed minimally to moderately decreased dark adapted visual functions in the CSNB2 patients, with differences between patients. In contrast, the dark adapted visual functions of the CSNB1 patients were more severely affected, but showed almost no differences between patients. The results from the “2D Light Lab” showed that all CSNB1 patients were blind at low intensities (equal to starlight), but quickly regained vision at higher intensities (full moonlight). Just above their dark adapted thresholds both CSNB1 and CSNB2 patients had normal visual fields. From the results we conclude that night vision problems in CSNB, in contrast to what the name suggests, are not conspicuous and generally not disabling.  
  Address Bartimeus Institute for the Visually Impaired, Zeist, The Netherlands. mbijveld@bartimeus.nl  
  Corporate Author Thesis  
  Publisher Place of Publication Editor  
  Language English Summary Language Original Title  
  Series Editor Series Title Abbreviated Series Title  
  Series Volume Series Issue Edition  
  ISSN 1932-6203 ISBN Medium  
  Area Expedition Conference  
  Notes PMID:23658786; PMCID:PMC3643903 Approved no  
  Call Number GFZ @ kyba @ Serial 3051  
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