Inverse pattern of photoreceptor abnormalities in retinitis pigmentosa and cone–rod dystrophy

• Published in: Documenta ophthalmologica Vol. 125; no. 3; pp. 211 - 218
• Main Authors: Yokochi, Midori, Li, Danjie, Horiguchi, Masayuki, Kishi, Shoji
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer-Verlag 01.12.2012; Springer Nature B.V
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Electroretinography; Female; Humans; Male; Medicine; Medicine & Public Health; Middle Aged; Ophthalmology; Original; Original Research Article; Retinal Cone Photoreceptor Cells - pathology; Retinal Cone Photoreceptor Cells - physiology; Retinal Photoreceptor Cell Inner Segment - pathology; Retinal Photoreceptor Cell Outer Segment - pathology; Retinal Rod Photoreceptor Cells - pathology; Retinal Rod Photoreceptor Cells - physiology; Retinitis Pigmentosa - physiopathology; Tomography, Optical Coherence - methods; Visual Acuity - physiology; Visual Field Tests; Visual Fields - physiology; Cone–rod dystrophy; Goldmann perimetry; Optical coherence tomography; Photoreceptor inner and outer segment (IS/OS); Retinitis pigmentosa; Full-field electroretinogram
• ISSN: 0012-4486; 1573-2622; 1573-2622
• DOI: 10.1007/s10633-012-9348-8

Purpose To determine the characteristics of the photoreceptor abnormalities in retinitis pigmentosa (RP) and cone–rod dystrophy (CRD). Methods We evaluated the photoreceptor abnormalities using spectral-domain optical coherence tomography (SD-OCT) in 28 patients with RP and 17 patients with CRD. The OCT images and full-field electroretinograms were obtained from 21 eyes in normal subjects who were age-matched to patients with RP and CRD and served as controls. Results Eyes with RP and CRD had markedly decreased rod responses (6.5 and 57.5 % of normal value), maximal responses (9.6 and 51.6 %), cone (16.5 and 25.8 %), and 30-Hz flicker responses (17.8 and 30.1 % of normal value), and their P values were smaller than 0.0003. On comparison of ERG data between RP and CRD, they had statistically significant differences in rod responses ( P  < 0.0003) and maximal responses ( P  < 0.0003). However, there were no statistical differences in cone response and a weak difference in 30-Hz flicker responses ( P  < 0.017). The best-corrected visual acuity was −0.03 ± 0.09 (logMAR, mean ± standard deviation [SD]) in eyes with RP, but 0.57 ± 0.54 in eyes with CRD. SD-OCT showed that eyes with RP had an intact reflective line at the junction between the photoreceptor inner and outer segment (IS/OS) at the fovea, while eyes with CRD had no IS/OS. The extent of the central visual field was correlated with the IS/OS length at the macula in eyes with RP. Conclusion The distribution patterns of the IS/OS line help to differentiate between RP and CRD.