scholarly journals Perifoveal Cone- and Rod-Mediated Temporal Contrast Sensitivities in Stargardt Disease/Fundus Flavimaculatus

2021 ◽  
Vol 62 (14) ◽  
pp. 24
Author(s):  
Julien Fars ◽  
Francesca Pasutto ◽  
Jan Kremers ◽  
Cord Huchzermeyer

Retina ◽  
2004 ◽  
Vol 24 (6) ◽  
pp. 920-928 ◽  
Author(s):  
KEAN T. OH ◽  
RICHARD G. WELEBER ◽  
EDWIN M. STONE ◽  
DAWN M. OH ◽  
JUSTIN ROSENOW ◽  
...  




2008 ◽  
Vol 18 (2) ◽  
pp. 239-247 ◽  
Author(s):  
G. Querques ◽  
R. Prato ◽  
C. Iaculli ◽  
M. Voigt ◽  
N. Delle Noci ◽  
...  

Purpose To investigate the relationship between morphologic lesions of the retina and functional abnormalities in patients with Stargardt disease (STGD) and fundus flavimaculatus (FFM). DESIGN. Case-controlled, prospective, comparative observational study. Methods A complete ophthalmologic examination, including best-corrected visual acuity (BCVA) and optical coherence tomography (OCT), was performed in 61 eyes of 32 consecutive patients with STGD/FFM and in 60 eyes of 30 matched healthy control subjects. Furthermore, fundus-related perimetry was performed in 12 of the affected eyes. Results The age ranged from 21 to 71 years in STGD/FFM patients and from 21 to 72 years in controls. BCVA ranged from 20/20 to 20/400 and from 20/20 to 20/32, respectively, in STGD/FFM patients and in controls. A foveal thinning was found by OCT Stratus in almost all cases (average 160 μm) compared with controls (average 210 μm) (pp<0.001). BCVA impairment significantly correlated to the degree of foveal thinning (r2=0.16; p=0.0014). Moreover, in STGD/FFM patients the authors observed two types of hyperreflective deposits which were not correlated with BCVA impairment or foveal thinning. In addition, fundus-related perimetry revealed a stable fixation in 8/12 eyes, that was predominantly central in only 4 of these eyes. A smaller degree of foveal thinning correlated to a more stable fixation (p=0.0108), even if not predominantly central (p=0.0218). Conclusions In this series, lower visual acuity and unstable fixation correlated with a greater transverse foveal thinning. OCT and fundus-related perimetry may be useful tools in STGD/FFM patients.



Stargardt macular dystrophy is a hereditary retinal degeneration that lacks effective treatment options. The pathophysiology of the disease is still not fully understood. While there are currently no available treatments for Stargardt disease, there are many categories of therapeutics under investigation to fulfill this unmet need for treatment. These include investigational visual cycle modulators, complement inhibitors, ABCA4 gene therapy, and subretinal transplantation of stem cell-derived retina pigment epithelial cells. Further trials are warranted to assess efficacy and safety in humans. In this review, the treatments investigated for the Stargardt disease are explained.



2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Hans Rutger Bosker ◽  
Matthias J. Sjerps ◽  
Eva Reinisch


1998 ◽  
Vol 38 (1) ◽  
pp. 55-59 ◽  
Author(s):  
Joseph Bilotta ◽  
Francesca M. Lynd ◽  
Maureen K. Powers


2021 ◽  
Vol 1 (1) ◽  
pp. 100005
Author(s):  
Rachael C. Heath Jeffery ◽  
Jennifer A. Thompson ◽  
Johnny Lo ◽  
Tina M. Lamey ◽  
Terri L. McLaren ◽  
...  


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