| Title | Colour Vision in Stargardt Disease |
| Purpose | 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. |
| Methods | 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. |
| Results | 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. |
| Last name | VANDENBROUCKE |
| Initials | T |
| Department | Dept Ophthalmol, Ghent University Hospital |
| City | Ghent |
| Last name | Buyl |
| Initials | R |
| Department | Dept Biostatistics, Vrije Universiteit Brussel |
| City | Brussels |
| Last name | De Zaeytijd |
| Initials | J |
| Department | Dept Ophthalmol, Ghent University Hospital |
| City | Ghent |
| Last name | Uvijls |
| Initials | A |
| Department | Dept Ophthalmol, Ghent University Hospital |
| City | Ghent |
| Last name | De Baere |
| Initials | E |
| Department | Ctr Medical Genetics, Ghent University Hospital |
| City | Ghent |
| Last name | Leroy |
| Initials | BP |
| Department | Dept Ophthalmol, Ghent University Hospital |
| City | Ghent |