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Review Inherited multifocal RPE-diseases: mechanisms for local dysfunction in global retinoid cycle gene defects. 2003
Besch D, Jägle H, Scholl HP, Seeliger MW, Zrenner E. · University Eye Hospital, Schleichstr. 12-16, D-72076 Tübingen, Germany. · Vision Res. · Pubmed #14611947 No free full text.
Abstract: Alterations of retinoid cycle genes are known to cause retinal diseases characterized by focal white dot fundus lesions. Fundus appearances reveal circumscribed RPE-changes, although generalized metabolic defects and global functional abnormalities are present. As a possible explanation, topographic inhomogeneities of the human photoreceptor mosaic and the role of a cone specific visual cycle will be discussed. Due to particular characteristics of photoreceptor subtypes as well as different pathways for photopigment regeneration the metabolic demand of individual RPE cells might differ. In "flecked retina diseases" heterogeneity of metabolic demand in individual RPE cells could therefore be responsible for their multifocal appearance.
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Article Full macular translocation (FMT) versus photodynamic therapy (PDT) with verteporfin in the treatment of neovascular age-related macular degeneration: 2-year results of a prospective, controlled, randomised pilot trial (FMT-PDT). 2009
Lüke M, Ziemssen F, Völker M, Altpeter E, Beutel J, Besch D, Bartz-Schmidt KU, Gelisken F. · University Eye Hospital, University of Lübeck, Lübeck, Germany. · Graefes Arch Clin Exp Ophthalmol. · Pubmed #19214552 No free full text.
Abstract: BACKGROUND: To report the outcome of best-corrected visual acuity (BCVA), near visual acuity (NVA), contrast sensitivity (CS) and vision-related quality of life (VRQOL) in patients 2 years after undergoing photodynamic therapy (PDT) or full macular translocation (FMT) for the treatment of neovascular age-related macular degeneration (AMD). METHODS: Fifty patients with predominantly classic subfoveal choroidal neovascularisation (CNV) secondary to AMD were randomized to PDT or FMT. BCVA was determined according a standardized protocol with ETDRS charts. NVA were calculated after testing with SNAB (Swiss National Association of and for the Blind) visual acuity cards. CS was measured with Pelli-Robson charts. The 39-item National Eye Institute Visual Function Questionnaire (NEI-VFQ-25 plus supplement) was performed. Primary end points were the changes of BCVA, NVA, CS and VRQOL at 24-month examination. RESULTS: A stabilisation of BCVA (+0.3 letters) was found in the FMT group, whereas a decrease of more than 12 letters (-12.6 letters) was found in the PDT group (p = 0.052). Mean NVA improved by 7.0 letters in the FMT group and was superior to the PDT group (-9.6 letters, p = 0.036), while mean CS showed a time-dependent decrease in both treatment groups (FMT: -3.3 letters, PDT: -3.8 letters, p = 0.726). Considering the results of the VRQOL scores, the improvement of the subscales scores for general vision (p = 0.015), mental health (p = 0.028) and near activity (p = 0.020) were significantly higher in the FMT group. CONCLUSIONS: FMT can stabilise BCVA and improve NVA over a period of 2 years in patients with subfoveal classic CNV secondary to neovascular AMD, whereas a decrease of BCVA and NVA was found in the PDT group. CS did not differ between FMT and PDT. A significant increase of VRQOL scores was only found in the FMT group and not in the PDT group. FMT seems to be a therapeutic approach that can increase visual function resulting in an improvement of patient's VRQOL, but exhibits a higher number of severe complications compared to PDT.
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Article Reading performance with low-vision aids and vision-related quality of life after macular translocation surgery in patients with age-related macular degeneration. 2007
Nguyen NX, Besch D, Bartz-Schmidt K, Gelisken F, Trauzettel-Klosinski S. · Department of Ophthalmology II, University Eye Hospital, Tübingen, Germany. · Acta Ophthalmol Scand. · Pubmed #17651462 No free full text.
Abstract: PURPOSE: The aim of the present study was to evaluate the power of magnification required, reading performance with low-vision aids and vision-related quality of life with reference to reading ability and ability to carry out day-to-day activities in patients after macular translocation. METHODS: This study included 15 patients who had undergone macular translocation with 360-degree peripheral retinectomy. The mean length of follow-up was 19.2 +/- 10.8 months (median 11 months). At the final examination, the impact of visual impairment on reading ability and quality of life was assessed according to a modified 9-item questionnaire in conjunction with a comprehensive clinical examination, which included assessment of best corrected visual acuity (BCVA), the magnification power required for reading, use of low-vision aids and reading speed. Patients rated the extent to which low vision restricted their ability to read and participate in other activities that affect quality of life. Responses were scored on a scale of 1.0 (optimum self-evaluation) to 5.0 (very poor). RESULTS: In the operated eye, overall mean postoperative BCVA (distance) was not significantly better than mean preoperative BCVA (0.11 +/- 0.06 and 0.15 +/- 0.08, respectively; p = 0.53). However, 53% of patients reported a subjective increase in visual function after treatment. At the final visit, the mean magnification required was x 7.7 +/- 6.7. A total of 60% of patients needed optical magnifiers for reading and in 40% of patients closed-circuit TV systems were necessary. All patients were able to read newspaper print using adapted low-vision aids at a mean reading speed of 71 +/- 40 words per minute. Mean self-reported scores were 3.2 +/- 1.1 for reading, 2.5 +/- 0.7 for day-to-day activities and 2.7 +/- 3.0 for outdoor walking and using steps or stairs. Patients' levels of dependency were significantly correlated with scores for reading (p = 0.01), day-to-day activities (p < 0.001) and outdoor walking and using steps (p = 0.001). CONCLUSIONS: The evaluation of self-reported visual function and vision-related quality of life in patients after macular translocation is necessary to obtain detailed information on treatment effects. Our results indicated improvement in patients' subjective evaluations of visual function, without significant improvement in visual acuity. The postoperative clinical benefits of treatment coincide with subjective benefits in terms of reading ability, quality of life and patient satisfaction. Our study confirms the importance and efficiency of visual rehabilitation with aids for low vision after surgery.
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Article Full macular translocation versus photodynamic therapy with verteporfin in the treatment of neovascular age-related macular degeneration: 1-year results of a prospective, controlled, randomised pilot trial (FMT-PDT). 2007
Gelisken F, Voelker M, Schwabe R, Besch D, Aisenbrey S, Szurman P, Grisanti S, Herzau V, Bartz-Schmidt KU. · Centre for Ophthalmology, University of Tuebingen, Schleichstr. 12, 72076 Tuebingen, Germany. · Graefes Arch Clin Exp Ophthalmol. · Pubmed #17219106 No free full text.
Abstract: BACKGROUND: The purpose of this study was to compare full macular translocation (FMT) with photodynamic therapy (PDT) in the treatment of neovascular age-related macular degeneration (AMD). METHODS: In a prospective, randomised, non-masked, mono-center, pilot-trial, 50 eyes of 50 patients were assigned to either FMT or PDT. Baseline and control examinations in 3-monthly intervals over a 12-month period included standardized protocol refraction, visual acuity testing and fluorescein angiography. Primary outcome measurements were made to establish the change in distant visual acuity from the baseline to the 12-month examination. The statistical analyses were carried out on the intent-to-treat principle. RESULTS: The improvement of one or more ETDRS lines was 56% (14/25) of the eyes in the FMT and 16% (4/25) of the eyes in the PDT arm (P = 0.007). Twenty eyes (80%) in the FMT and 16 eyes (64%) in the PDT group had less than three ETDRS lines of vision loss (P = 0.35). Retinal detachment (six eyes) and diplopia (five patients) were recorded in the FMT group. None of the eyes treated in the FMT group had phtysis. CONCLUSION: This pilot study showed that no statistically significant difference existed between the FMT and PDT in terms of the vision loss of less than three ETDRS lines in eyes with neovascular AMD. The chance of vision improvement was significantly higher for the patients in the FMT group. However, in the era of promising therapy with anti-vascular endothelial growth factor for neovascular AMD, FMT should not be offered as a standard primary procedure for neovascular AMD.
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Article Neuroprotective effects of cardiotrophin-like cytokine on retinal ganglion cells. 2005
Schuettauf F, Zurakowski D, Quinto K, Varde MA, Besch D, Laties A, Anderson R, Wen R. · Department of Ophthalmology, Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania, USA. · Graefes Arch Clin Exp Ophthalmol. · Pubmed #15838664 No free full text.
Abstract: BACKGROUND: Premature neuronal cell death is a feature of numerous central nervous system and eye diseases, including glaucoma. Neurons (including retinal ganglion cells, RGCs) are protected by several neurotrophic factors, among those the IL-6 family of cytokines. Lately, a novel member of the IL-6 family of cytokines has been identified and cloned. This cytokine is known as novel neurotrophin-1/B-cell-stimulating factor-3 (NNT-1/BSF-3) or cardiotrophin-like cytokine (CLC). It shows neurotrophic as well as B-cell stimulatory effects. METHODS: In this study, the neuroprotective properties of CLC on RGC loss in vivo were investigated. RESULTS: CLC significantly protected RGCs from degeneration in both chosen models of retinal neuronal damage: optic nerve crush (P<0.01) and N-methyl-D-aspartate (NMDA) injection (P<0.001). CONCLUSIONS: CLC shows neuroprotective effects on RGCs in vivo and might be a treatment option for chronic neurodegenerative eye diseases such as glaucoma. Clinical feasibility for the substance requires further investigation since the immunomodulatory and possible adverse effects have not yet been thoroughly characterized.
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Article Alterations of slow and fast rod ERG signals in patients with molecularly confirmed Stargardt disease type 1. free! 2002
Scholl HP, Besch D, Vonthein R, Weber BH, Apfelstedt-Sylla E. · Department of Pathophysiology of Vision and Neuroophthalmology, University Eye Hospital Tübingen, Tübingen, Germany. · Invest Ophthalmol Vis Sci. · Pubmed #11923272 links to free full text
Abstract: PURPOSE: To investigate the slow and fast rod signals of the scotopic 15-Hz flicker ERG in patients with molecularly confirmed Stargardt disease type I (STGD1). There is evidence that these slow and the fast rod ERG signals can be attributed to the rod bipolar-AII cell pathway and the rod-cone coupling pathway, respectively. METHODS: Twenty-seven patients with STGD1 with mutations in both alleles of the ABCA4 gene were included. Scotopic ERG response amplitudes and phases to flicker intensities ranging from -3.37 to -0.57 log scotopic troland x sec (log scot td x sec) were measured at a flicker frequency of 15 Hz. In addition, scotopic standard ERGs were obtained. Twenty-two normal subjects served as controls. RESULTS: The amplitudes of both the slow and fast rod ERG signals were significantly reduced in the STGD1 group. The phases of the slow rod signals lagged significantly, whereas those of the fast rod signals did not. The standard scotopic ERG did not reveal significant alterations. CONCLUSIONS: The results provide evidence that a defective ABCA4 transporter can functionally affect both the rod bipolar-AII cell pathway and the rod-cone coupling pathway. In STGD1, the scotopic 15-Hz flicker ERG may reveal subtle abnormalities at different sites within the rod system that remain undetected by standard ERG techniques.
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