Senile Reticular Pigmentary Degeneration

Humphrey, William T.; Carlson, R.V.; Valone, James A.

doi:10.1016/0002-9394(84)90686-x

Cited by 15 publications

(4 citation statements)

References 6 publications

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“…There were also no cases of PRPD presenting exclusively in the temporal periphery. These results are corroborated by previous reports showing a predilection for the nasal and superior quadrant of the fundus [2,9]. Feke and associates reported that blood flow to the temporal side of the retina was approximately three times larger than to the nasal side [17].…”

Section: Discussionsupporting

confidence: 90%

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Peripheral Reticular Pigmentary Degeneration and Choroidal Vascular Insufficiency, Studied by Ultra Wide-Field Fluorescein Angiography

et al. 2017

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PurposeTo explore the pathogenesis of peripheral reticular pigmentary degeneration (PRPD) and its clinical significance.MethodsThis cross-sectional, observational study (conducted between January 2010 and May 2015) enrolled 441 eyes of 229 subjects, including 35 eyes with PRPD and 406 eyes without PRPD, which was identified by ultra-wide-field fluorescein angiography (UWFA). The distribution and angiographic circulation time of PRPD were assessed by UWFA. The frequencies of systemic and ophthalmologic comorbidities were compared between groups. Univariate and multivariate generalized estimation equation methods were used to determine the risk factors for PRPD.ResultsThe patients with PRPD had a mean age of 75.7 ± 8.5 years (range, 59–93 years), whereas the patients without PRPD had a mean age of 60.1 ± 14.9 years (range, 9–92 years). All eyes with PRPD manifested the lesion in the superior nasal periphery with or without circumferential extension. Among those, only 16 eyes (45.7%) in the PRPD group showed distinctive features in the same location on fundus photographs. There was significant choroidal filling delay in the PRPD group when compared with the control group (1.42±1.22 vs. -0.02±1.05 seconds, P < 0.001). Multivariate regression analysis revealed that older age (P < 0.001), stroke (P = 0.018), ischemic optic neuropathy (P < 0.001), and age-related macular degeneration (P = 0.022) were significantly associated with PRPD.ConclusionsUWFA may enhance the diagnostic sensitivity of PRPD. Choroidal vascular insufficiency with compromised systemic circulation in the elderly was related to the manifestation of PRPD. These results help to better understand the pathophysiology of PRPD. Co-existence of systemic and ophthalmic circulatory disorders should be considered in patients with PRPD.

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Section: Discussionsupporting

confidence: 90%

“…Previous reports indicated that about a third of patients with age-related macular degeneration manifested PRPD [27], and two thirds of patients with PRPD have accompanying age-related macular degeneration [2]. The current study also confirmed a significant association between AMD and PRPD.…”

Section: Discussionsupporting

confidence: 86%

Peripheral Reticular Pigmentary Degeneration and Choroidal Vascular Insufficiency, Studied by Ultra Wide-Field Fluorescein Angiography

et al. 2017

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“…32,33 Peripheral reticular degeneration is highly associated with advancing age, 32 as well as age-related macular degeneration. 31,32,34 In one report of 104 patients with peripheral reticular degeneration, only one was younger than 50 years, and 82% were older than 60 years. 34 In postmortem histology, none of the 129 subjects younger than 50 years were found to have peripheral reticular degeneration.…”

Section: Discussionmentioning

confidence: 99%

“…31,32,34 In one report of 104 patients with peripheral reticular degeneration, only one was younger than 50 years, and 82% were older than 60 years. 34 In postmortem histology, none of the 129 subjects younger than 50 years were found to have peripheral reticular degeneration. 32 In this context, our finding of 2 patients younger than 50 years out of a total of 6 patients with peripheral reticular degeneration suggests that the finding of deep peripheral pigment in our cohort could, at least in part, be associated with Stargardt disease, and not an incidental finding due to the relatively advanced age of the cohort.…”

Section: Discussionmentioning

confidence: 99%

CLINICAL CHARACTERIZATION OF STARGARDT DISEASE PATIENTS WITH THE p.N1868I ABCA4 MUTATION

Collison¹,

Lee

Fishman

et al. 2019

Retina

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Purpose: To investigate the Stargardt disease phenotype associated with an unusually common and “extremely hypomorphic” ABCA4 variant, p.N1868I. Methods: The charts of 27 patients with p.N1868I on one allele and a severe/deleterious mutation on the other allele were reviewed. Subjective age of onset, best-corrected visual acuity, and stage of disease were recorded for all 27 patients, 18 of whom had multiple visits. When available, fundus photography, spectral domain optical coherence tomography, fundus autofluorescence, full-field electroretinograms, Goldmann visual fields, and fluorescein angiography were included. Five families with multiple affected members were analyzed. Results: The median age at symptom onset was 41.5 years, and 3 p.N1868I patients had not developed visual symptoms as of the most recent eye examination. Median best-corrected visual acuity in the better-seeing eye at baseline was 20/25−2, and the median duration from symptom onset to legal blindness was 25 years. The five families described in this study demonstrated clinically significant intrafamilial variability, and affected family members who did not share the p.N1868I variant had relatively more severe phenotypes. Conclusion: This study demonstrates the consistency of foveal sparing, the variation in age at onset, the intrafamilial variability, and the prognosis with regard to visual acuity in p.N1868I-associated Stargardt disease.

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