Partial visual deprivation from early monocular enucleation (the surgical removal of one eye within the first few years of life) results in a number of long-term morphological adaptations in adult cortical and subcortical visual, auditory, and multisensory brain regions. In this study, we investigated whether early monocular enucleation also results in the altered development of white matter structure. Diffusion tensor imaging and probabilistic tractography were performed to assess potential differences in visual system white matter in adult participants who had undergone early monocular enucleation compared to binocularly intact controls. To examine the microstructural properties of these tracts, mean diffusion parameters including fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) were extracted bilaterally. Asymmetries opposite to those observed in controls were found for FA, MD, and RD in the optic radiations, the projections from primary visual cortex (V1) to the lateral geniculate nucleus (LGN), and the interhemispheric V1 projections of early monocular enucleation participants. Early monocular enucleation was also associated with significantly lower FA bidirectionally in the interhemispheric V1 projections. These differences were consistently greater for the tracts contralateral to the enucleated eye, and are consistent with the asymmetric LGN volumes and optic tract diameters previously demonstrated in this group of participants. Overall, these results indicate that early monocular enucleation has long-term effects on white matter structure in the visual pathway that results in reduced fiber organization in tracts contralateral to the enucleated eye. Hum Brain Mapp 39:133-144, 2018.V C 2017 Wiley Periodicals, Inc.

No abstract

Purpose: Similar to early blindness, monocular enucleation (the removal of one eye) early in life results in crossmodal behavioral and morphological adaptations. Previously it has been shown that partial visual deprivation from early monocular enucleation results in structural white matter changes throughout the visual system (Wong et al., 2018). The current study investigated structural white matter of the auditory system in adults who have undergone early monocular enucleation compared to binocular control participants. Methods: We reconstructed four auditory and audiovisual tracts of interest using probabilistic tractography and compared microstructural properties of these tracts to binocularly intact controls using standard diffusion indices. Results: Although both groups demonstrated asymmetries in indices in intrahemispheric tracts, monocular enucleation participants showed asymmetries opposite to control participants in the auditory and A1-V1 tracts. Monocular enucleation participants also demonstrated significantly lower fractional anisotropy in the audiovisual projections contralateral to the enucleated eye relative to control participants. Conclusions: Partial vision loss from early monocular enucleation results in altered structural connectivity that extends into the auditory system, beyond tracts primarily dedicated to vision.

No abstract

No abstract

Altered retinal dopamine and ON-pathway activity may underlie myopia development. It has been shown that the stimulation of the blind spot with short-wavelength light increases the electroretinogram (ERG) b-wave amplitude of myopic eyes and may engage the retinal dopaminergic system. This study evaluated the impact of various durations of blind spot stimulation on the electrophysiological response of the myopic retina and their relationship to axial length. Six myopic individuals underwent three short-wavelength blue light blind spot stimulation protocols (10 s, 1 min, 10 min) using a virtual reality headset. As a control condition, no stimulation was shown for 1 min. The b-wave amplitude of the photopic full-field ERG was measured at baseline and 10, 20, 30, 40, 50, and 60 min after each condition. A significant increase in b-wave amplitude was observed for all stimulation protocols compared to the control. The peak b-wave amplitude was observed 20 min after the 1-min stimulation protocol and 60 min after the 10-min stimulation protocol. A significant positive correlation was found between axial length of the eye and percent change in b-wave amplitude for the 10-min stimulation protocol. A rapid and a delayed b-wave time course responses were observed following 1 min and 10 min of blind spot stimulation, respectively. Overall, these results indicate that light stimulation of the blind spot for various durations elevates ON-bipolar cell activity in the retina and as such is assumed to reduce the myopic response. These findings could have implications for future myopia treatment.

Hailey-Hailey disease (HHD) is an autosomal dominant blistering dermatosis with incomplete penetrance caused by an ATP2C1 gene mutation. Currently, there is no cure for HHD; however therapeutic options aim to minimize the exacerbating factors and manage patients’ symptoms. A 58-year-old male presented with a 10-year history of biopsy-proven HHD. He was seen consistently over the course of nine years with multiple flares a year consisting of pruritic and painful intertriginous plaques with involvement of the chest, upper arms, and back. His disease was recalcitrant to topicals, oral and topical antibiotics, phototherapy, and systemic corticosteroids. He was started on dupilumab and noticed significant improvement. We present a case of HHD recalcitrant to various modalities of treatment. Our description of rapid improvement with dupilumab suggests a role for Th2 signaling in the pathophysiology of HHD. We propose that dupilumab works for HHD due to the significant skin barrier dysfunction, similar to atopic dermatitis, and consider whether inflammation plays an earlier role in the disease. Although used for an off-label purpose, in this case, further studies should assess the clinical response and safety of patients with recalcitrant HHD treated with dupilumab.

No abstract