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Adam M Dubis, Rola Ba-Abbad, Jonathan Aboshiha, Alfredo Dubra, Andrew Webster, Joseph Carroll, Michel Michaelides; Multimodal Imaging of the Tapetal-like Reflex in Carriers of RPGR-associated Retinopathy. Journal of Vision 2016;16(4):15-16. doi: 10.1167/16.4.6.
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© 2017 Association for Research in Vision and Ophthalmology.
Purpose: Retinal structure and function deficits in carriers of X-linked retinitis pigmentosa (RP) caused by mutations in RPGR have been shown to be heterogeneous. One particularly interesting feature is the retinal tapetal-like reflex (TLR).[1–3] In this study we assessed the photoreceptor mosaic changes in a group of unrelated obligate carriers of X-linked RP and examined the TLR patterns using multimodal retinal imaging.
Methods: Female carriers of X-linked RP and unaffected females underwent multimodal ophthalmic imaging including spectral domain optical coherence tomography (SDOCT) and fundus autofluorescence imaging. Adaptive optics scanning light ophthalmoscope (AOSLO) images of the photoreceptor mosaic were obtained for the fovea and temporal parafoveal region.
Results: Nine female carriers (age 28–61) and three non-carrier females (ages 23–35) were examined. A TLR was clinically detectable in all but one carrier. One subject had reduced acuity due to RPGR associated degeneration. On SDOCT, inner segment ellipsoid (ISe) and retinal pigment epithelium (RPE) local contrast was reduced in areas of TLR. AOSLO images showed zones of decreased cone density with mosaic irregularity and increased photoreceptor reflectivity. Increased reflectivity on AOSLO was co-localized to areas of TLR. No localized areas of decreased density or increased reflectivity were seen in non-carrier females.
Conclusion: AOSLO reflectance patterns in X-linked carriers of RP appear to identify the cellular origin that underlie the TLR. Similar cellular changes have been observed with AOSLO in affected males , which, supports the notion that such reflexes are related to random X-inactivation in females.
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