Concern of photosensitive seizures evoked by 3D video displays or virtual reality headsets in children: Current perspective

This review assesses the risk of a photic-induced seizure in a child during viewing of 3D (binocular 3 dimensional, stereoscopic) movies or games, either on standard video displays or when wearing a virtual reality (VR) headset. Studies published by pediatric epilepsy experts emphasize the low risk of 3D viewing even for children with known photosensitive epilepsy (PSE). The low incidence of PSE is noteworthy because the number of hours devoted to 2D or 3D screen viewing and/or VR headset use by children worldwide has increased markedly over the last decade. The medical literature does not support the notion that VR headset use poses a risk for PSE

Refractive surgery to correct visual impairments in 267 children with autism spectrum and related neuro-developmental disorders: improvements in vision and behavior

Background: Children with autism spectrum disorder (ASD) may have impaired vision owing to high refractive errors and aversion to spectacles or contact lenses. Visual blurring is caused by near-sighted myopia, far-sighted hyperopia, or astigmatism in one or both eyes. Refractive surgery can restore sharp vision and eliminate the need for spectacles and contact lenses. Restoration of sharp vision may improve ASD behavior. We aimed to determine the refractive outcomes in this cohort using ophthalmic measures and behavioral and school performance alterations after refractive surgery by employing parent–proxy reports. Methods: This interventional, retrospective case series included data from 267 children with refractive errors and neurodevelopmental disorders (NDDs) diagnosed as ASD alone or NDD with ASD-like behaviors over a 15-year period. One of three refractive surgery methods was employed, with the choice of method uniquely tailored to the child’s eye anatomy. Laser photorefractive keratectomy (PRK) was performed in 131 children, implantation of a phakic intraocular lens (pIOL) in 115 children, and removal of the crystalline lens and implantation of an intraocular lens (refractive lens exchange, RLE) in 21 children. All procedures were performed under brief general anesthesia, with the child returning home on the same day. Results: The median age at surgery was 10.9 years and the median follow-up period was 3.1 years. Pre-operative refractive errors ranged from a mean (standard deviation) +7.5 (0.09) D to -14.3 (4.8) D. Surgery corrected 87% of the children to normal focal length (± 1 D). Visual acuity improved an average of 0.6 logarithm of the minimum angle of resolution, the equivalent of 6 lines on a standard eye chart. Change in visual acuity was significant (all P < 0.01) between baseline and the most recent follow-up examination in each of subgroups. Change in spherical equivalent refractive error at 3, 12, 24, 36, 60, and > 60 months post-operatively were significant (all P < 0.01) between baseline and each follow-up visit in each of subgroups. Social interactions and ASD behaviors improved in 72% (192) of the treated children (P < 0.01). The incidence of sight-threatening complications was low. Conclusions: Refractive surgery improves both visual function and behavior in most children with ASD and major myopia, hyperopia, or astigmatism. The PRK, pIOL, and RLE procedures appear to be effective and reasonably safe methods for improving refractive error, visual acuity, and behavior in many ametropic children with ASD and ASD-like NDDs

Visual pathway function and structure in Wolfram syndrome: Patient age, variation and progression

Background/aimsTo report alterations in visual acuity and visual pathway structure over an interval of 1–3 years in a cohort of children, adolescents and young adults who have Wolfram syndrome (WFS) and to describe the range of disease severity evident in patients with WFS whose ages differed by as much as 20 years at first examination.MethodsAnnual, prospective ophthalmological examinations were performed in conjunction with retinal nerve fibre layer (RNFL) analysis. Diffusion tensor MRI-derived fractional anisotropy was used to assess the microstructural integrity of the optic radiations (OR FA).ResultsMean age of the 23 patients with WFS in the study was 13.8 years (range 5–25 years). Mean log minimum angle resolution visual acuity was 0.66 (20/91). RNFL thickness was subnormal in even the youngest patients with WFS. Average RNFL thickness in patients with WFS was 57±8 µ or ~40% thinner than that measured in normal (94±10 µ) children and adolescents (P&lt;0.01). Lower OR FA correlated with worse visual acuity (P=0.006). Subsequent examinations showed declines (P&lt;0.05) in visual acuity, RNFL thickness and OR FA at follow-up intervals of 12–36 months. However, a wide range of disease severity was evident across ages: some of the youngest patients at their first examination had deficits more severe than the oldest patients.ConclusionThe genetic mutation of WFS causes damage to both pregeniculate and postgeniculate regions of the visual pathway. The damage is progressive. The decline in visual pathway structure is accompanied by declines of visual function. Disease severity differs widely in individual patients and cannot be predicted from their age.</jats:sec

Human deprivation amblyopia: treatment insights from animal models

Amblyopia is a common visual impairment that develops during the early years of postnatal life. It emerges as a sequela to eye misalignment, an imbalanced refractive state, or obstruction to form vision. All of these conditions prevent normal vision and derail the typical development of neural connections within the visual system. Among the subtypes of amblyopia, the most debilitating and recalcitrant to treatment is deprivation amblyopia. Nevertheless, human studies focused on advancing the standard of care for amblyopia have largely avoided recruitment of patients with this rare but severe impairment subtype. In this review, we delineate characteristics of deprivation amblyopia and underscore the critical need for new and more effective therapy. Animal models offer a unique opportunity to address this unmet need by enabling the development of unconventional and potent amblyopia therapies that cannot be pioneered in humans. Insights derived from studies using animal models are discussed as potential therapeutic innovations for the remediation of deprivation amblyopia. Retinal inactivation is highlighted as an emerging therapy that exhibits efficacy against the effects of monocular deprivation at ages when conventional therapy is ineffective, and recovery occurs without apparent detriment to the treated eye

A phase Ib/IIa clinical trial of dantrolene sodium in patients with Wolfram syndrome

BACKGROUNDWolfram syndrome is a rare ER disorder characterized by insulin-dependent diabetes mellitus, optic nerve atrophy, and progressive neurodegeneration. Although there is no treatment for Wolfram syndrome, preclinical studies in cell and rodent models suggest that therapeutic strategies targeting ER calcium homeostasis, including dantrolene sodium, may be beneficial.METHODSBased on results from preclinical studies on dantrolene sodium and ongoing longitudinal studies, we assembled what we believe is the first-ever clinical trial in pediatric and adult Wolfram syndrome patients with an open-label phase Ib/IIa trial design. The primary objective was to assess the safety and tolerability of dantrolene sodium in adult and pediatric Wolfram syndrome patients. Secondary objectives were to evaluate the efficacy of dantrolene sodium on residual pancreatic β cell functions, visual acuity, quality-of-life measures related to vision, and neurological functions.RESULTSDantrolene sodium was well tolerated by Wolfram syndrome patients. Overall, β cell functions were not significantly improved, but there was a significant correlation between baseline β cell functions and change in β cell responsiveness (R2, P = 0.004) after 6-month dantrolene therapy. Visual acuity and neurological functions were not improved by 6-month dantrolene sodium. Markers of inflammatory cytokines and oxidative stress, such as IFN-γ, IL-1β, TNF-α, and isoprostane, were elevated in subjects.CONCLUSIONThis study justifies further investigation into using dantrolene sodium and other small molecules targeting the ER for treatment of Wolfram syndrome.TRIAL REGISTRATIONClinicalTrials.gov identifier NCT02829268FUNDINGNIH/National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) (DK112921, DK113487, DK020579), NIH/National Center for Advancing Translational Sciences (NCATS) (TR002065, TR000448), NIH training grant (F30DK111070), Silberman Fund, Ellie White Foundation, Snow Foundation, Unravel Wolfram Syndrome Fund, Stowe Fund, Eye Hope Foundation, Feiock Fund, Washington University Institute of Clinical and Translational Sciences grant UL1TR002345 from NIH/NCATS, Bursky Center for Human Immunology & Immunotherapy Programs

CAUSING AND CURING INFANTILE ESOTROPIA IN PRIMATES: THE ROLE OF DECORRELATED BINOCULAR INPUT (AN AMERICAN OPHTHALMOLOGICAL SOCIETY THESIS)

Purpose: Human infants at greatest risk for esotropia are those who suffer cerebral insults that could decorrelate signals from the 2 eyes during an early critical period of binocular, visuomotor development. The author reared normal infant monkeys, under conditions of binocular decorrelation, to determine if this alone was sufficient to cause esotropia and associated behavioral as well as neuroanatomic deficits

The Neural Mechanism for Latent Nystagmus

To elucidate the neural mechanism for unstable gaze holding in the form of latent fixation nystagmus. Latent nystagmus (LN) is the most common subtype of pathologic nystagmus observed in human and non-human primates. It is linked strongly to binocular maldevelopment in infancy, either from strabismus or deprivation of monocular spatial vision

The Impact of Gaze Deficits in Children with Cerebral Palsy

Cerebral palsy (CP) is a permanent, nonprogressive disorder of movement and posture due to a lesion of the fetal or infant brain. The goal was to determine whether children with different severities of CP, as defined using the Gross Motor Function Classification System (GMFCS), had different degrees or types of visual dysfunction.SKcerebralpals

İnfantil ezotropyada alt oblik hiperfonksiyonu ve disosiye vertikal deviasyon tedavisinde alt oblik öne transpozisyonu

AMAÇ: İnfantil ezotropya olgularında alt oblik öne transpozisyonunun (AOÖT) alt oblik hiperfonksiyonu (AOHF) ve disosiye vertikal deviasyon (DVD) üzerindeki tedavi edici ve DVD gelişiminden koruyucu rolü değerlendirildi. YÖNTEM: AOHF için AOÖT uygulanmış ve cerrahi sonrası en az 6 aylık izlem süresi bulunan 74 İnfantil ezotropyalı olgunun (143 göz) kayıtları retrospektif olarak incelendi. Cerrahi öncesi ve sonrası DVD derecesi ve miktarı ile AOHF derecesi tespit edildi. AO fonksiyonu -4 hipofonksiyon ile +4 hiperfonksiyon değerleri arasında; sıfır normal fonksiyonu temsil etmek üzere derecelendirildi. BULGULAR: AOÖT 69 olguda bilateral ve 5 olguda da unilateral olarak uygulanmıştı. AOÖT sonrası 99 gözde (%69) normal AO fonksiyonu sağlanırken, 17 gözde (%12) +1 ve 11 gözde (%8) belirgin (+2 veya üzeri) olmak üzere toplam 28 gözde (%20) rezidüel AOHF saptandı. Onaltı gözde ise (%11) -2 veya daha az miktarlarda AO hipofonksiyonu gelişti. AOÖT öncesi 40 olguda (75 göz) uni- veya bilateral DVD mevcuttu. AOÖT sonrası DVD miktarı 50 gözde (%67) tamamen düzelmiş, 14 gözde (%19) daha küçük miktarlara gerilemiş, 10 gözde (%13) değişmemiş, 1 gözde ise (%1) artış göstermişti. AOÖT öncesi DVD bulunmayan 34 İnfantil ezotropyalı olgunun 3'iinde (%9) en son muayene oldukları dönemde DVD gelişimi saptandı. AOÖT sonrası yalnız 1 olguda hipotropya ve abduksiyonda belirgin elevasyon kısıtlılığı gözlendi. SONUÇ: AOÖT, İnfantil ezotropya olgularında AOHF mevcudiyetinde eğer DVD eşlik ediyor veya ilerde DVD gelişim riski varsa tercih edilebilecek bir cerrahi yöntemdir