Morphological and functional involvement of the inner retina in retinitis pigmentosa

Arrigo, Alessandro; Aragona, Emanuela; Perra, Cristian; Saladino, Andrea; Amato, Alessia; Bianco, Lorenzo; Pina, Adelaide; Basile, Giulia; Bandello, Francesco; Parodi, Maurizio Battaglia

doi:10.1038/s41433-022-02139-7

Cited by 8 publications

(8 citation statements)

References 36 publications

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“…3,5 On OCT, only changes happening in the later stages of the disease were observed, such as a thinning of the inner retinal layers, with direct consequences for BCVA and retinal sensitivity. 6,11,20 In this study, we describe the HGB as a new feature of the GCL, found by OCT in approximately a quarter of the patients affected by RP. We define it as a continuous hyperreflective band that is fully enclosed in the GCL, bilaterally in almost all cases, becoming more frequent in females.…”

Section: Discussionmentioning

confidence: 89%

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Hyperreflective band in the ganglion cell layer in retinitis pigmentosa

Antropoli

Arrigo

Bianco

et al. 2023

Retina

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Purpose: To describe a sign that takes the form of a continuous hyperreflective band within the thickness of the ganglion cell layer (GCL), thus dubbed the “hyperreflective ganglion cell layer band” (HGB), which the authors detected in a fraction of patients affected by retinitis pigmentosa (RP). Methods: Retrospective, cross-sectional, observational study. Optical coherence tomography (OCT) images of patients with RP examined between May 2015 and June 2021 were retrospectively reviewed for the presence of HGB, epiretinal membrane (ERM), macular hole, and cystoid macular edema (CME). The ellipsoid zone (EZ) width was also measured. A subgroup of patients underwent microperimetry in the central 2°, 4°, and 10°. Results: One hundred and fifty-four eyes from 77 subjects were included in the study. The HGB was present in 39 (25.3%) eyes with RP. Mean best-corrected visual acuity (BCVA) was 0.39 ± 0.05 logMAR (approximately 20/50 Snellen equivalent) and 0.18 ± 0.03 logMAR (approximately 20/32 Snellen equivalent) in eyes with and without HGB, respectively (P < 0.001). The two groups did not differ regarding EZ width; mean 2°, 4°, and 10° retinal sensitivity; and prevalence of CME, ERM, and macular hole. The multivariable analysis showed the presence of HGB to be a predictor of poorer BCVA (P < 0.001). Conclusion: HGB is an OCT finding detectable in approximately a quarter of eyes with RP and is associated with a poorer visual function. In the discussion, the authors speculate about possible morphogenetic scenarios to explain this observation.

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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Hyperreflective band in the ganglion cell layer in retinitis pigmentosa

Antropoli

Arrigo

Bianco

et al. 2023

Retina

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“…12 On the other hand, other studies utilizing SD-OCT have revealed that patients with RP exhibit significant impairments and thinning in their inner retinal structures, which negatively impacts retinal function. [33][34][35][36] The underlying cause of these alterations in the inner retina is currently unknown, however, possible explanations include remodeling of the retina due to the loss of photoreceptors or progressive spread of retinal pigment, leading to increased damage to the retina. 36,37 In an earlier study conducted by our research group, we assessed the condition of the inner retina in patients with RP.…”

Section: Inner Retinamentioning

confidence: 99%

“…[33][34][35][36] The underlying cause of these alterations in the inner retina is currently unknown, however, possible explanations include remodeling of the retina due to the loss of photoreceptors or progressive spread of retinal pigment, leading to increased damage to the retina. 36,37 In an earlier study conducted by our research group, we assessed the condition of the inner retina in patients with RP. 36 Our findings revealed a substantial reduction in thickness across all inner retinal layers, with the exception of the INL and OPL which were found to be significantly thicker.…”

Section: Inner Retinamentioning

confidence: 99%

“…36,37 In an earlier study conducted by our research group, we assessed the condition of the inner retina in patients with RP. 36 Our findings revealed a substantial reduction in thickness across all inner retinal layers, with the exception of the INL and OPL which were found to be significantly thicker. Additionally, we demonstrated that the thinning of these inner retinal layers is correlated with reduced retinal sensitivity as measured by microperimetry.…”

Section: Inner Retinamentioning

confidence: 99%

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New insights in the multimodal imaging of retinitis pigmentosa

Berni

Arrigo

Bianco

et al. 2023

European Journal of Ophthalmology

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Retinitis pigmentosa (RP) is a group of inherited rod-cone dystrophies, noted for a high genotypical and phenotypical heterogeneity. Traditionally, VA, visual field, and electroretinography have been used to assess RP progression. However, visual acuity and visual field tests are essentially subjective and, especially in the late stages of the disease, are unable to confidently reveal minor progression. Therefore, there is a need for novel examination modalities that rely on quantitative, structural measurements. In this regard, several non-invasive imaging techniques have been studied, including spectral-domain optical coherence tomography, optical coherence tomography angiography, and fundus autofluorescence. By correlating surrogate biomarkers with functional measurements of the disease, these techniques may be able to develop reliable outcome meters that can be used to gain a deeper understanding of the underlying causes of the disease and to assess the effectiveness of therapy even before an actual loss of vision occurs. In this review, we will summarize the recent imaging findings and biomarkers that have been identified in RP patients. Our goal is to provide information that can promptly aid in selecting patients for clinical trials and new gene therapies, monitoring the disease progression, and evaluating treatment outcomes.

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