Retinitis Pigmentosa: From Pathomolecular Mechanisms to Therapeutic Strategies.

Retinitis pigmentosa is an inherited disease, in which mutations in different types of genes lead to the death of photoreceptors and the loss of visual function. Although retinitis pigmentosa is the most common type of inherited retinal dystrophy, a clear line of therapy has not yet been defined. In this review, we will focus on the therapeutic aspect and attempt to define the advantages and disadvantages of the protocols of different therapies. The role of some therapies, such as antioxidant agents or gene therapy, has been established for years now. Many clinical trials on different genes and mutations causing RP have been conducted, and the approval of voretigene nepavorec by the FDA has been an important step forward. Nonetheless, even if gene therapy is the most promising type of treatment for these patients, other innovative strategies, such as stem cell transplantation or hyperbaric oxygen therapy, have been shown to be safe and improve visual quality during clinical trials. The treatment of this disease remains a challenge, to which we hope to find a solution as soon as possible.

Visuo-Acoustic Stimulation's Role in Synaptic Plasticity: A Review of the Literature.

Brain plasticity is the capacity of cerebral neurons to change, structurally and functionally, in response to experiences. This is an essential property underlying the maturation of sensory functions, learning and memory processes, and brain repair in response to the occurrence of diseases and trauma. In this field, the visual system emerges as a paradigmatic research model, both for basic research studies and for translational investigations. The auditory system remains capable of reorganizing itself in response to different auditory stimulations or sensory organ modification. Acoustic biofeedback training can be an effective way to train patients with the central scotoma, who have poor fixation stability and poor visual acuity, in order to bring fixation on an eccentrical and healthy area of the retina: a pseudofovea. This review article is focused on the cellular and molecular mechanisms underlying retinal sensitivity changes and visual and auditory system plasticity.

Microperimetric biofeedback training: fundamentals, strategies and perspectives.

Microperimetric biofeedback training (MBFT) is a visual rehabilitative strategy based on fixation stability improvement reinforcing or creating a new preferential fixation locus. The rationale consists in reeducating visual system to a new visual condition, promoting retina-brain transmission, and thus cortical plasticity. The use of MBFT found is major application in visual diseases involving central vision, but later it revealed promising functional outcomes even in myopia, inherited retinal degenerations and nystagmus. However, the use of microperimetric biofeedback is still limited due to poor knowledge of the procedure and inconsistent standards of practice, and thus an incipient skepticism on its efficacy. This review provides an overview of the rationale, current implications, procedures and future perspectives of microperimetric biofeedback training.

Circulating levels of reactive oxygen species in patients with nonproliferative diabetic retinopathy and the influence of antioxidant supplementation: 6-month follow-up.

AIMS: The aim was to evaluate circulating levels of reactive oxygen species (ROS) and changes in central macular thickness (CMT) in patients with nonproliferative diabetic retinopathy (NPDR) after antioxidant supplementation. MATERIALS AND METHODS: A total of 68 patients (68 eyes) with NPDR were enrolled. Patients were randomly divided into two groups: Treated with antioxidant supplement (Group A) and untreated control group (Group B). Each tablet, for oral administration, containing pycnogenol 50 mg, Vitamin E 30 mg and coenzyme Q10 20 mg. CMT and free oxygen radical test (FORT) were analyzed at baseline (T0), 3 (T1) and 6 (T2) months in both groups. RESULTS: In Group A, FORT levels and CMT were significantly reduced over time (P < 0.001 for both). In Group B, FORT levels were increased (P < 0.001) and CMT did not vary significantly (P = 0.81) over 3 time points. CONCLUSIONS: This is the first study showing the reduction of ROS levels in patients with NPDR thanks to antioxidant therapy. Moreover, our findings have suggested also an influence on retinal thickness.

Visual rehabilitation in patients with myopic maculopathy: our experience.

OBJECTIVE: To evaluate the efficacy of the MP-1 microperimeter (Nidek Technologies Srl, Padova, Italy) and Visual Pathfinder (LACE Inc) in improving visual function of patients with myopic maculopathy. DESIGN: Prospective, nonrandomized, interventional case series. PARTICIPANTS: Seventeen patients (34 eyes) between 36 and 58 years of age with myopic maculopathy and central retinal scotomas. METHODS: After a complete eye examination, all patients underwent 10 training sessions with MP-1 biofeedback (7 minutes) and Visual Pathfinder (3 minutes) for each eye once a week. Statistical analysis was performed with Student t test. The p values less than 0.05 were considered statistically significant. RESULTS: The mean best corrected visual acuity increased from 0.64 ± 0.22 to 0.38 ± 0.20 logMAR at the end of follow-up (p = 0.03); visual-evoked potential P100 amplitude increased from 3.54 ± 1.90 to 6.64 ± 2.91 µV at the end of follow-up (p = 0.04); average retinal sensitivity, calculated in the 12 degrees of the central retina, increased from 6.6 ± 2.6 to 14.6 ± 3.6 dB (p = 0.03). Fixation behaviour in the 2 degrees of the central retina increased from 45% ± 17% to 75% ± 23% (p = 0.04). The bivariate contour ellipse area (95%) increased from 10.34 ± 3.11 to 7.64 ± 2.71 square degrees (p = 0.04). CONCLUSIONS: The combination of acoustic biofeedback training with MP-1 and Visual Pathfinder offers a reasonable improvement of visual function in patients with myopic maculopathy. This method might be considered as a rehabilitative strategy as a "therapeutic option" in these patients for whom most treatments usually do not work.

New trends in visual rehabilitation with MP-1 microperimeter biofeedback: optic neural dysfunction.

The aim of this study was to evaluate the efficacy of visual rehabilitation with MP-1 microperimeter biofeedback in advanced optic neural dysfunction due to glaucoma, and to precisely characterize fixation stability and location in affected patients. Ten patients (18 eyes) with advanced glaucoma were submitted to a rehabilitation protocol that consisted of: a 25-item questionnaire (National Eye Institute Visual Functioning Que stionnaire); measurement of visual acuity; a reading speed test; microperimetry with fixation study, retinal sensitivity and the bivariate contour ellipse area (BCEA). The rehabilitation program consisted of 10 training sessions of 10 minutes per eye performed over a period of one week and was repeated at four months, eight months, and one year. Statistical analysis was performed using the Student's t-test and Spearman correlation; p values less than 0.05 were considered statistically significant. In 13 eyes fixation changed from unstable to relatively unstable while its location changed from predominantly eccentric to predominantly central. In five eyes, fixation changed from relatively unstable to stable with a change of location from poor central fixation to predominantly New trends in visual rehabilitation with MP-1 microperimeter biofeedback: optic neural dysfunction central fixation. Mean retinal sensitivity changed from 7.43±8.28 dB to 8.33±9.04 dB (p<0.05); the mean best corrected visual acuity was 0.98±0.66 logMAR at the baseline assessment, and 0.75±0.6 logMAR at the end of rehabilitation (p>0.05); reading speed improved from a mean value of 31.4±4.3 words/minute to 55.6±3.2 words/minute at the end of the training (p<0.05). The BCEA changed from 0.94±0.39 deg2 to 0.86±0.46 deg2 (p=0.76). Rehabilitation with MP-1 biofeedback in patients with advanced glaucoma is a useful means of improving these patients' fixation stability, reading speed and quality of life.

Slowing the degenerative process, long lasting effect of hyperbaric oxygen therapy in retinitis pigmentosa.

BACKGROUND: Retinitis pigmentosa (RP) therapy is still an unsolved challenge. Recent reports have underlined that hyperbaric oxygen (HBO) therapy could play a role in slowing the retinal degenerative process. The aim of this study was to assess the efficacy of HBO therapy on visual function in RP patients. METHODS: We performed a single-center, comparative, longitudinal case-controlled randomized clinical trial, which lasted 10 years. We randomly divided RP patients into two groups. Group 1, the control group, consisted of 44 RP patients (21 males and 23 females; mean age 35.5) who took Vitamin A. Group 2, with 44 RP patients (21 males and 23 females; mean age 35,02), underwent HBO therapy. No statistically significant difference was found at the beginning of the study between the two groups. We compared the results concerning visual acuity, Goldmann perimetry, static perimetry Humphrey field analyzer (HFA), and electroretinogram (ERG) obtained in the two groups at 5 and 10 years follow-up. Statistical analysis was performed with Kaplan-Meier life-table with the evaluation of log-rank coefficient. RESULTS: At 5 year follow-up, 87.5% of group 2 patients preserved 80% of the initial visual acuity, while the same result was achieved in only 70.4% of group 1 patients (X(2) = 8.2; p < 0.01); at 10 year follow-up, 63.33% of group 2 patients preserved 80% of the initial visual acuity, while the same percentage of residual visual acuity was maintained in 40% of group 1 patients (X(2) = 3.22; p = 0.05). At 10 year follow-up, Goldmann perimetry (target I4e) did not change in 31.6% of group 2 and in 10.5% of group 1; evaluation of mean defect (MD) with static perimetry HFA showed that 53% of HBO patients had 80% of residual mean sensitivity compared to 23.5% of the control group patients (X(2) = 4.72; p = 0.035). ERG b-wave mean values at the end of the protocol were significantly higher in the HBO treated group (X(2) = 4.53; p = 0.013). CONCLUSION: Our study underlines that HBO therapy can be a safe alternative approach to RP patients, contributing to the stabilization of their visual function concerning visual acuity, visual field, and ERG responses while waiting for a definite cure.