Titre | Colour Vision in Stargardt Disease |
But | To investigate the type and severity of colour vision deficiencies (CVDs) in Stargardt disease (STD). And, to establish how the degree of CVD relates to best-corrected visual acuity (BCVA), full field ERG (ffERG) and duration of disease. |
Méthodes | A retrospective, cross-sectional study of 97 patients with a clinical diagnosis of STD included a comprehensive medical history and a full clinical work-up, with extensive colour vision testing. Eight patients underwent anomaloscopy. ABCA4 was screened in 92 patients. |
Résultats | Patients were allocated to 5 BCVA groups and to 3 ffERG groups. Normal colour vision was found in almost 30% of patients. R/G CVDs increased as BCVA declined. More than 50% had a deutan type R/G CVD, although protan R/G CVDs became progressively apparent as BCVA decreased. A predominance of pseudoprotanomaly was evident only on anomaloscopy. Additional Blue/Yellow (B/Y) CVDs were noted in 25% of patients. B/Y CVDs and BCVA higher than 0.75 were seen in adult-onset STD. CVDs evolve to scotopization in patients with low BCVA and/or longstanding disease. Duration of disease did not correlate well with CVDs. Also, no statistically significant differences in ERG results were found between groups with or without a CVD. |
Conclusion | Since colour vision function is better correlated to BCVA than either disease duration or ffERG, it is a rather reliable indicator of disease severity. The presence of CVDs may help to establish an early diagnosis of STD. |
Nom | VANDENBROUCKE |
Initiales | T |
Institut | Dept Ophthalmol, Ghent University Hospital |
Ville | Ghent |
Nom | Buyl |
Initiales | R |
Institut | Dept Biostatistics, Vrije Universiteit Brussel |
Ville | Brussels |
Nom | De Zaeytijd |
Initiales | J |
Institut | Dept Ophthalmol, Ghent University Hospital |
Ville | Ghent |
Nom | Uvijls |
Initiales | A |
Institut | Dept Ophthalmol, Ghent University Hospital |
Ville | Ghent |
Nom | De Baere |
Initiales | E |
Institut | Ctr Medical Genetics, Ghent University Hospital |
Ville | Ghent |
Nom | Leroy |
Initiales | BP |
Institut | Dept Ophthalmol, Ghent University Hospital |
Ville | Ghent |