Electric Fields Direct Full-Length Optic Nerve Regeneration and Partial Restoration of Visual Function

Restoration of vision in patients blinded by advanced optic neuropathies requires therapies that promote retinal ganglion cell (RGC) survival and direct RGC axon regeneration. Previously, we showed that electric fields (EFs) direct RGC axon growth towards the cathode (negative electrode), in vitro. Here, we hypothesized that exogenous application of EFs can be exploited to direct optic nerve regeneration in vivo

Electric Fields Direct Full-Length Optic Nerve Regeneration and Partial Restoration of Visual Function

Restoration of vision in patients blinded by advanced optic neuropathies requires therapies that promote retinal ganglion cell (RGC) survival and direct RGC axon regeneration. Previously, we showed that electric fields (EFs) direct RGC axon growth towards the cathode (negative electrode), in vitro. Here, we hypothesized that exogenous application of EFs can be exploited to direct optic nerve regeneration in vivo

Bilateral Optic Atrophy and Sensorineural Hearing Loss in Two Siblings With Brown-Vialetto-Van Laere Syndrome

The differential diagnosis of optic atrophy and hearing loss includes genetic disorders such as Wolfram syndrome and dominant optic atrophy. We report two siblings with optic atrophy, sensorineural hearing loss, and sensory neuropathy with ataxia that were found to have heterozygous pathogenic mutations in SLC52A2, a riboflavin transporter gene that is associated with Brown-Vialetto-Van Laere (BVVL) syndrome

Calciphylaxis Associated Bilateral Ischemic Optic Neuropathy: A Case Report and Literature Review

Calciphylaxis is a rare disease that occurs predominantly in patients with end-stage renal disease (ESRD). Cutaneous manifestations of calciphylaxis are common, however ophthalmologic/neuro-ophthalmologic manifestations are rare, with only 20 other reported cases of calciphylaxis associated ischemic optic neuropathy

Optic Nerve Regeneration: How Will We Get There?

Background: Restoration of vision in patients blinded by advanced optic neuropathies requires technologies that can either 1) salvage damaged and prevent further degeneration of retinal ganglion cells (RGCs), or 2) replace lost RGCs. Evidence acquisition: Review of scientific literature. Results: In this article, we discuss the different barriers to cell-replacement based strategies for optic nerve regeneration and provide an update regarding what progress that has been made to overcome them. We also provide an update on current stem cell-based therapies for optic nerve regeneration. Conclusions: As neuro-regenerative and cell-transplantation based strategies for optic nerve regeneration continue to be refined, researchers and clinicians will need to work together to determine who will be a good candidate for such therapies.ICopticnerveregenerationchallenge

Confirmatory Factor Analysis of the SLEEP-50 Questionnaire in Trichotillomania (Hair-Pulling Disorder) and Excoriation (Skin-Picking) Disorder

The study objective was to perform a confirmatory factor analysis of the SLEEP-50 Questionnaire (SLEEP-50) in Trichotillomania (Hair-Pulling Disorder) and Excoriation (Skin-Picking) Disorder and compare sleep complaints in adults with Trichotillomania, Excoriation Disorder and non-affected controls. Participants were 234 adults with Trichotillomania, 170 with Excoriation Disorder, and 146 non-affected controls. Participants rated sleep using the SLEEP-50 and Pittsburgh Sleep Quality Index (PSQI). Confirmatory factor analysis was used to assess fit of the originally-proposed SLEEP-50 factors within Trichotillomania and Excoriation Disorder. Findings revealed acceptable to good fit of the original factors. Internal consistency was excellent in Trichotillomania and good in Excoriation Disorder for the total score and poor to good for subscales. Convergent validity was strong for the total and weak to strong for subscales in both groups. Findings suggest greater sleep complaints in Trichotillomania and Excoriation Disorder than in the general population. Trichotillomania and Excoriation Disorder groups reported greater rates of sleep apnea, narcolepsy, restless leg syndrome/periodic limb movement disorder, circadian rhythms sleep disorder, and sleep-related affective disorder relative to controls. There were no significant differences for insomnia, sleep state misperception, sleepwalking, nightmares, or hypersomnia. Results underscore the importance of clinical assessment of sleep disorders in Trichotillomania and Excoriation Disorder

In Vivo Application of Electric Fields Collaborate With rRHEB to Direct Optic Nerve Regeneration

Regenerating the optic nerve requires signals that both "fuel" and "steer" retinal ganglion cell (RGC) axon growth to appropriate targets. Although strategies to increase mammalian target of rapamycin (mTOR) activity have been employed to "fuel" RGC axon regeneration, they are not as effective at "steering" regeneration. Based on compelling evidence that electric field (EF) application does both, we investigated whether EFs-in conjunction with mTOR activation-direct more target-specific RGC axon regeneration than either treatment alone

Retinal ganglion cell repopulation for vision restoration in optic neuropathy: a roadmap from the RReSTORe Consortium

Abstract Retinal ganglion cell (RGC) death in glaucoma and other optic neuropathies results in irreversible vision loss due to the mammalian central nervous system’s limited regenerative capacity. RGC repopulation is a promising therapeutic approach to reverse vision loss from optic neuropathies if the newly introduced neurons can reestablish functional retinal and thalamic circuits. In theory, RGCs might be repopulated through the transplantation of stem cell-derived neurons or via the induction of endogenous transdifferentiation. The RGC Repopulation, Stem Cell Transplantation, and Optic Nerve Regeneration (RReSTORe) Consortium was established to address the challenges associated with the therapeutic repair of the visual pathway in optic neuropathy. In 2022, the RReSTORe Consortium initiated ongoing international collaborative discussions to advance the RGC repopulation field and has identified five critical areas of focus: (1) RGC development and differentiation, (2) Transplantation methods and models, (3) RGC survival, maturation, and host interactions, (4) Inner retinal wiring, and (5) Eye-to-brain connectivity. Here, we discuss the most pertinent questions and challenges that exist on the path to clinical translation and suggest experimental directions to propel this work going forward. Using these five subtopic discussion groups (SDGs) as a framework, we suggest multidisciplinary approaches to restore the diseased visual pathway by leveraging groundbreaking insights from developmental neuroscience, stem cell biology, molecular biology, optical imaging, animal models of optic neuropathy, immunology & immunotolerance, neuropathology & neuroprotection, materials science & biomedical engineering, and regenerative neuroscience. While significant hurdles remain, the RReSTORe Consortium’s efforts provide a comprehensive roadmap for advancing the RGC repopulation field and hold potential for transformative progress in restoring vision in patients suffering from optic neuropathies