Search

Background Posterior cortical atrophy (PCA), the visual variant of Alzheimer's disease, leads to high-level visual deficits such as alexia or agnosia. Visual field deficits have also been identified, but often inconsistently reported. Little is known about the pattern of visual field deficits or the underlying cortical changes leading to this visual loss. Methods Multi-modal magnetic resonance imaging was used to investigate differences in gray matter volume, cortical thickness, white matter microstructure and functional activity in patients with PCA compared to age-matched controls. Additional analyses investigated hemispheric asymmetries in these metrics according to the visual field most affected by the disease. Results Analysis of structural data indicated considerable loss of gray matter in the occipital and parietal cortices, lateralized to the hemisphere contralateral to the visual loss. This lateralized pattern of gray matter loss was also evident in the hippocampus and parahippocampal gyrus. Diffusion-weighted imaging showed considerable effects of PCA on white matter microstructure in the occipital cortex, and in the corpus callosum. The change in white matter was only lateralized in the occipital lobe, however, with greatest change in the optic radiation contralateral to the visual field deficit. Indeed, there was a significant correlation between the laterality of the optic radiation microstructure and visual field loss. Conclusions Detailed brain imaging shows that the asymmetric visual field deficits in patients with PCA reflect the pattern of degeneration of both white and gray matter in the occipital lobe. Understanding the nature of both visual field deficits and the neurodegenerative brain changes in PCA may improve diagnosis and understanding of this disease., Highlights • Patients with posterior cortical atrophy show asymmetric visual field deficits manifesting as hemianopia. • Both gray and white matter show lateralized degeneration corresponding to the most affected visual field. • Laterality of microstructure in the optic radiation correlates with visual field loss.

While coeliac disease is primarily a disease of the digestive system, there have been several reports of neurological effects, both motor and cognitive. Here, we present the case of a woman with coeliac disease, under dietary control, in whom there is profound long-standing visual disturbance including reduction of visual fields, loss of rapid flicker and colour sensitivity and severe deficits in acuity. Structural magnetic resonance imaging (MRI) indicates large regions of calcification and abnormal tissue that is restricted to the occipital cortex, particularly the posterior region. Functional MRI indicates an absence of normal visual activation in the primary visual cortex, but at least in one hemisphere, there is neural activity to moving stimuli in visual motion area hMT+. White matter microstructure in the pathway between the lateral geniculate nucleus and hMT+ is normal compared to healthy control subjects, but is severely abnormal between the lateral geniculate nucleus and primary visual cortex. This case study illustrates the very specific nature of cortical deficit that can arise in association with coeliac disease, and highlights the importance of early dietary control for the disease.

PURPOSE OF REVIEW: The arrival of large datasets and the on-going refinement of neuroimaging technology have led to a number of recent advances in our understanding of visual pathway disorders. This work can broadly be classified into two areas, both of which are important when considering the optimal management strategies. The first looks at the delineation of damage, teasing out subtle changes to (specific components of) the visual pathway, which may help evaluate the severity and extent of disease. The second uses neuroimaging to investigate neuroplasticity, via changes in connectivity, cortical thickness, and retinotopic maps within the visual cortex. RECENT FINDINGS: Here, we give consideration to both acquired and congenital patients with damage to the visual pathway, and how they differ. Congenital disorders of the peripheral visual system can provide insight into the large-scale reorganization of the visual cortex: these are investigated with reference to disorders of the optic chiasm and anophthalmia (absence of the eyes). In acquired conditions, we consider the recent work describing patterns of degeneration, both following single insult and in neurodegenerative conditions. We also discuss the developments in functional neuroimaging, with particular reference to work on hemianopia and the controversial suggestion of cortical reorganization following acquired retinal injury. SUMMARY: Techniques for comparing neuro-ophthalmological conditions with healthy visual systems provide sensitive metrics to uncover subtle differences in grey and white matter structure of the brain. It is now possible to compare the massive reorganization present in congenital conditions with the apparent lack of plasticity following acquired damage. © 2014 Wolters Kluwer Health Lippincott Williams and Wilkins.

Background The existence of transsynaptic retrograde degeneration (TRD) in the human visual system has been established, however the dependence of TRD on different factors such as lesion location, size and manner of lesion acquisition has yet to be quantified. Methods We obtained T1-weighted structural and diffusion-weighted images for 26 patients with adult-acquired or congenital hemianopia and 12 age-matched controls. The optic tract (OT) was defined and measured in the structural and diffusion-weighted images, and degeneration assessed by comparing the integrity of tracts in the lesioned and in the undamaged hemisphere. Results OT degeneration was found in all patients with established lesions, regardless of lesion location. In patients with acquired lesions, the larger the initial lesion, the greater is the resulting TRD. However, this was not the case for congenital patients, who generally showed greater degeneration than would be predicted by lesion size. A better predictor of TRD was the size of the visual field deficit, which was correlated with degeneration across all patients. Interestingly, although diffusion-weighted imaging (DWI) is more frequently used to examine white matter tracts, in this study the T1-weighted scans gave a better indication of the extent of tract degeneration. Conclusions We conclude that TRD of the OT occurs in acquired and congenital hemianopia, is correlated with visual field loss, and is most severe in congenital cases. Understanding the pattern of TRD may help to predict effects of any visual rehabilitation training.