Assessment of night vision problems in patients with congenital stationary night blindness.

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 ni...

Full description

Bibliographic Details
Main Authors: Mieke M C Bijveld, Maria M van Genderen, Frank P Hoeben, Amir A Katzin, Ruth M A van Nispen, Frans C C Riemslag, Astrid M L Kappers
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2013-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3643903?pdf=render
_version_ 1818846917753307136
author Mieke M C Bijveld
Maria M van Genderen
Frank P Hoeben
Amir A Katzin
Ruth M A van Nispen
Frans C C Riemslag
Astrid M L Kappers
author_facet Mieke M C Bijveld
Maria M van Genderen
Frank P Hoeben
Amir A Katzin
Ruth M A van Nispen
Frans C C Riemslag
Astrid M L Kappers
author_sort Mieke M C Bijveld
collection DOAJ
description 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.
first_indexed 2024-12-19T05:53:10Z
format Article
id doaj.art-2fbacc0c497a4118bdcbb3bb5e6b7c8a
institution Directory Open Access Journal
issn 1932-6203
language English
last_indexed 2024-12-19T05:53:10Z
publishDate 2013-01-01
publisher Public Library of Science (PLoS)
record_format Article
series PLoS ONE
spelling doaj.art-2fbacc0c497a4118bdcbb3bb5e6b7c8a2022-12-21T20:33:32ZengPublic Library of Science (PLoS)PLoS ONE1932-62032013-01-0185e6292710.1371/journal.pone.0062927Assessment of night vision problems in patients with congenital stationary night blindness.Mieke M C BijveldMaria M van GenderenFrank P HoebenAmir A KatzinRuth M A van NispenFrans C C RiemslagAstrid M L KappersCongenital 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.http://europepmc.org/articles/PMC3643903?pdf=render
spellingShingle Mieke M C Bijveld
Maria M van Genderen
Frank P Hoeben
Amir A Katzin
Ruth M A van Nispen
Frans C C Riemslag
Astrid M L Kappers
Assessment of night vision problems in patients with congenital stationary night blindness.
PLoS ONE
title Assessment of night vision problems in patients with congenital stationary night blindness.
title_full Assessment of night vision problems in patients with congenital stationary night blindness.
title_fullStr Assessment of night vision problems in patients with congenital stationary night blindness.
title_full_unstemmed Assessment of night vision problems in patients with congenital stationary night blindness.
title_short Assessment of night vision problems in patients with congenital stationary night blindness.
title_sort assessment of night vision problems in patients with congenital stationary night blindness
url http://europepmc.org/articles/PMC3643903?pdf=render
work_keys_str_mv AT miekemcbijveld assessmentofnightvisionproblemsinpatientswithcongenitalstationarynightblindness
AT mariamvangenderen assessmentofnightvisionproblemsinpatientswithcongenitalstationarynightblindness
AT frankphoeben assessmentofnightvisionproblemsinpatientswithcongenitalstationarynightblindness
AT amirakatzin assessmentofnightvisionproblemsinpatientswithcongenitalstationarynightblindness
AT ruthmavannispen assessmentofnightvisionproblemsinpatientswithcongenitalstationarynightblindness
AT fransccriemslag assessmentofnightvisionproblemsinpatientswithcongenitalstationarynightblindness
AT astridmlkappers assessmentofnightvisionproblemsinpatientswithcongenitalstationarynightblindness