Carlos Andrade1,2, João Beato3, Ana Monteiro1,2, Andreia Costa1,2, Susana Penas3,4, Joana Guimarães1,2,5, Fernando Falcão Reis3,4, Carolina Garrett1,2,5. 1. Department of Neurology, Centro Hospitalar São João, Porto, Portugal. 2. Neurology and Neurosurgery Unit of Clinical Neurosciences and Mental Health Department, Porto, Portugal. 3. Department of Ophthalmology, Centro Hospitalar São João, Porto, Portugal. 4. Ophthalmology Unit of Sense Organs Department, Faculty of Medicine University of Porto, Porto, Portugal. 5. Institute for Molecular and Cell Biology, University of Porto, Portugal.
Abstract
BACKGROUND: Spectral-domain optical coherence tomography has been used in several neurological conditions, and peripapillary and macular measurements have been proposed as potential biomarkers in these disorders. The aim of this study was to investigate retinal and choroidal changes in Huntington's disease and to evaluate any potential correlation with the stage of the disease. METHODS: A cross-sectional observational study compared patients with Huntington's disease and controls. Patients were evaluated using the Unified Huntington's Disease Rating Scale. Spectral-domain optical coherence tomography with enhanced depth imaging was used, and peripapillary choroidal and retinal nerve fiber layer thickness and macular retinal and choroidal thickness were evaluated. RESULTS: Fifteen eyes of 8 patients and 16 eyes of 8 sex-, age-, and mean refractive error-matched healthy controls were included. Average (231.3 ± 52.8 vs 296.2 ± 57.1, P = 0.033), central (341.8 ± 70.5 vs 252.0 ± 57.9, P = 0.015), and inferior (225.3 ± 57.9 vs 313.8 ± 55.2, P = 0.007) macular choroidal thickness were significantly reduced in patients, in comparison with controls. No differences were observed in macular retina or peripapillary retinal and choroidal measurements. However, there was a negative correlation between Total Motor Score of the Unified Huntington's Disease Rating Scale and average (r(2) = 0.585, P = 0.027), superior (r(2) = 0.653, P = 0.015), nasal (r(2) = 0.642, P = 0.017), and inferior (r(2) = 0.574, P = 0.029) macular retinal thickness. CONCLUSIONS: Our results suggest that both the choroidal and retinal macula are altered in Huntington's disease and may become useful biomarkers for monitoring neurodegeneration in this disease. The involvement of the choroid may also support the recent findings of vascular involvement in Huntington's disease.
BACKGROUND: Spectral-domain optical coherence tomography has been used in several neurological conditions, and peripapillary and macular measurements have been proposed as potential biomarkers in these disorders. The aim of this study was to investigate retinal and choroidal changes in Huntington's disease and to evaluate any potential correlation with the stage of the disease. METHODS: A cross-sectional observational study compared patients with Huntington's disease and controls. Patients were evaluated using the Unified Huntington's Disease Rating Scale. Spectral-domain optical coherence tomography with enhanced depth imaging was used, and peripapillary choroidal and retinal nerve fiber layer thickness and macular retinal and choroidal thickness were evaluated. RESULTS: Fifteen eyes of 8 patients and 16 eyes of 8 sex-, age-, and mean refractive error-matched healthy controls were included. Average (231.3 ± 52.8 vs 296.2 ± 57.1, P = 0.033), central (341.8 ± 70.5 vs 252.0 ± 57.9, P = 0.015), and inferior (225.3 ± 57.9 vs 313.8 ± 55.2, P = 0.007) macular choroidal thickness were significantly reduced in patients, in comparison with controls. No differences were observed in macular retina or peripapillary retinal and choroidal measurements. However, there was a negative correlation between Total Motor Score of the Unified Huntington's Disease Rating Scale and average (r(2) = 0.585, P = 0.027), superior (r(2) = 0.653, P = 0.015), nasal (r(2) = 0.642, P = 0.017), and inferior (r(2) = 0.574, P = 0.029) macular retinal thickness. CONCLUSIONS: Our results suggest that both the choroidal and retinal macula are altered in Huntington's disease and may become useful biomarkers for monitoring neurodegeneration in this disease. The involvement of the choroid may also support the recent findings of vascular involvement in Huntington's disease.
Authors: Christopher Kai Shun Leung; Alexander Ka Ngai Lam; Robert Neal Weinreb; David F Garway-Heath; Marco Yu; Philip Yawen Guo; Vivian Sheung Man Chiu; Kelvin Ho Nam Wan; Mandy Wong; Ken Zhongheng Wu; Carol Yim Lui Cheung; Chen Lin; Carmen Kar Mun Chan; Noel Ching Yan Chan; Ka Wai Kam; Gilda Wing Ki Lai Journal: Nat Biomed Eng Date: 2022-01-06 Impact factor: 25.671
Authors: Amir H Kashani; Samuel Asanad; Jane W Chan; Maxwell B Singer; Jiong Zhang; Mona Sharifi; Maziyar M Khansari; Farzan Abdolahi; Yonggang Shi; Alessandro Biffi; Helena Chui; John M Ringman Journal: Prog Retin Eye Res Date: 2021-01-15 Impact factor: 19.704