10.6084/m9.figshare.5128570.v1
Vandenbroucke T.
Vandenbroucke
T.
Buyl R.
Buyl
R.
De Zaeytijd J.
De Zaeytijd J.
Bauwens M.
Bauwens
M.
Uvijls A.
Uvijls
A.
De Baere E.
De Baere E.
Leroy B.P.
Leroy
B.P.
Supplementary Material for: Colour Vision in Stargardt Disease
Karger Publishers
2015
Colour vision and electrophysiological tests
Stargardt macular dystrophy
Acquired colour vision defect
Stargardt disease
Fundus flavimaculatus
Genetics
2015-10-23 00:00:00
Dataset
https://karger.figshare.com/articles/dataset/Supplementary_Material_for_Colour_Vision_in_Stargardt_Disease/5128570
<b><i>Purpose:</i></b> To investigate the type and severity of acquired colour vision deficiencies (CVDs) in molecularly proven Stargardt disease (STD) and to establish whether a relationship exists between best-corrected visual acuity (BCVA) and full-field electroretinography (ffERG), and the degree of CVD. <b><i>Methods:</i></b> A retrospective, cross-sectional study of 73 patients with a molecularly proven diagnosis of STD, who underwent extensive colour vision evaluation, using pseudo-isochromatic and arrangement tests. Thirteen patients underwent Nagel anomaloscopy. <b><i>Results:</i></b> Normal colour vision was found in almost 20% of patients. Red/green (R/G) CVDs increased as BCVA declined. About 45% of all R/G CVDs were of the deutan type, although protan type CVDs became progressively apparent when moving from the high to the low BCVA group. An additional blue/yellow CVD was noted in about 25% of patients. In 10/13 patients, a pseudoprotanomaly was noted on anomaloscopy. Severe CVDs leading to scotopization were noted in patients with low BCVA and/or long-standing disease. No statistically significant differences in ERG results were found between groups with or without a CVD. <b><i>Conclusions:</i></b> The degree and type of colour vision deficiency in STD patients correlate better with BCVA than with ffERG results. The presence of specific CVDs may help to establish a diagnosis of STD. A battery of colour vision tests is required to properly evaluate CVDs in STD.