Visual Dysfunction in Huntington's Disease: A Systematic Review

• Published in: Journal of Huntington's disease Vol. 8; no. 2; pp. 233 - 242
• Main Authors: Dhalla, Amit, Pallikadavath, Susil, Hutchinson, Claire V
• Format: Journal Article
• Language: English
• Published: Netherlands IOS Press BV 01.01.2019
• Subjects: Cognition; Cortex (somatosensory); Humans; Huntington Disease - complications; Huntington Disease - pathology; Huntington's disease; Information processing; Retina; Sensory integration; Structure-function relationships; Systematic review; Thinning; Vision Disorders - etiology; Vision Disorders - pathology; Visual cortex; Visual Cortex - pathology; Visual evoked potentials; Visual pathways; Visual Pathways - pathology; Visual perception; Visual system; vision; primary visual cortex; visual pathways; motion perception; retina; Huntington’s disease; contrast sensitivity; colour vision
• ISSN: 1879-6397; 1879-6400; 1879-6397
• DOI: 10.3233/JHD-180340

It is well-documented that patients with Huntington's disease (HD) exhibit specific deficits in visual cognition. A less well-documented literature also exists that suggests people with HD experience a number of disease-related changes to more rudimentary sensory visual processing. Here, we review evidence for the effects of HD on the integrity of the early visual pathways in humans along with changes to low-level visual sensitivity. We find evidence for reduced structural and functional integrity of the visual pathways, marked by retinal thinning, reduced VEP amplitude, and cell loss and thinning in visual cortex. We also find evidence of visual perceptual deficits, particularly for colour and motion. We suggest that future studies with well-defined HD and HD-related groups in appropriate numbers that systematically examine the relationship between structural changes to the visual system, basic visual perceptual deficits and disease stage/severity are therefore likely to yield promising results.