Impairment of retinal increment thresholds in Huntington's disease

Abstract

We have investigated detection thresholds for a foveal blue test light using a Maxwellian view system in 61 normal subjects, 19 patients with Huntington's chorea, 14 patients with Tourette's syndrome, and 20 patients with schizophrenia. Ten measurements were made: The blue test light (1 degree diameter, 500 msec duration) was presented either superimposed on a yellow adaptation field (5 degree diameter) or 500 msec after switching off this field (transient tritanopia effect). In both cases five different background intensities were presented. The only abnormality found was in patients with Huntington's chorea. During adaptation these patients' thresholds are significantly higher than normal (p < 0.005). No change was found in the transient tritanopia effect. Huntington's disease causes degeneration of several different transmitter systems in the brain. Increment threshold testing allows for noninvasive investigation of patients and confirms the involvement of the retina in the degenerative process in Huntington's chorea.

Fine‐structural analysis and connexin expression in the retina of a transgenic model of Huntington's disease

“…Involvement of the visual system in HD has been documented previously on the basis of visual tests in HD patients (Paulus et al, 1993). The authors investigated detection thresholds for a foveal blue test light using a Maxwellian view system.…”

Optical coherence tomography findings in Huntington’s disease: a potential biomarker of disease progression

“…To date, the retina has been thought to be spared in HD although it has not been studied extensively. One group of investigators identified electrophysiological changes in 19 patients that suggest abnormal cone function [40]. A post-mortem examination of one HD patient with advanced disease (28 year history) did not reveal any histological abnormalities, when compared with a control eye [41].…”

Cognitive control predicted by color vision, and vice versa

“…Even though the correlative nature of our findings does not directly speak to the underlying causal relations, the observed pattern fits with previous demonstrations that populations suffering from dopaminergic imbalance have more trouble in processing 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 11 conflicting information and additionally display blue-yellow CVI, such as in Parkinson's disease (Wylie et al, 2010;Pieri et al, 2000), Huntington's disease and Tourette syndrome (Georgiou et al, 1995;Paulus et al, 1993;Melun et al, 2001), ADHD (Spinelli et al, 2011;Tannock et al, 2006) and cocaine use (Sellaro et al, 2014;Hulka et al, 2013). Thus, given the relation between DA and response conflict (Botvinick 2007), our data provide additional clues in favor of the hypothesis that blue-yellow CVI is likely to indicate a central hypodopaminergic state (Roy, 2003;Lagerlöf (1982).…”

“…Interestingly for our purposes, however, DA can be found in high concentration in the amacrine and interplexiform cells of the retina (Bodis-Wollner&Tzelepi, 1998;Witkovsky, 2004). Abnormal color discrimination has been described for several neuropsychiatric conditions underlying altered dopaminergic functions, such as Parkinson's and Huntington's disease, Tourette syndrome, ADHD, and cocaine use (Melun et al, 2001;Paulus et al, 1993;Pieri et al, 2000;Tannock et al, 2006;Hulka et al, 2013). Moreover, Lagerlöf (1982) found evidence that the intake of DAD2 receptorantagonists, such as haloperidol,induces moderate blue-yellow deficits.…”