Titel | Colour Vision in Stargardt Disease |
Doel | 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. |
Methodes | 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. |
Resultaten | 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. |
Conclusie | 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. |
Naam | VANDENBROUCKE |
Initialen | T |
Instituut | Dept Ophthalmol, Ghent University Hospital |
Stad | Ghent |
Naam | Buyl |
Initialen | R |
Instituut | Dept Biostatistics, Vrije Universiteit Brussel |
Stad | Brussels |
Naam | De Zaeytijd |
Initialen | J |
Instituut | Dept Ophthalmol, Ghent University Hospital |
Stad | Ghent |
Naam | Uvijls |
Initialen | A |
Instituut | Dept Ophthalmol, Ghent University Hospital |
Stad | Ghent |
Naam | De Baere |
Initialen | E |
Instituut | Ctr Medical Genetics, Ghent University Hospital |
Stad | Ghent |
Naam | Leroy |
Initialen | BP |
Instituut | Dept Ophthalmol, Ghent University Hospital |
Stad | Ghent |