G Alvarez1, A Rey1, F B Sanchez-Dalmau1, E Muñoz2, J Ríos3, A Adán1. 1. Department of Ophthalmology, Hospital Clinic de Barcelona, Calle Sabino de Arana, Barcelona, Spain. 2. Department of Neurology, Hospital Clínic i Provincial de Barcelona, Calle Villarroel, Barcelona, Spain. 3. Laboratory of Biostatistics and Epidemiology, Hospital Clínic de Barcelona, calle Villarroel, Barcelona, Spain.
Abstract
PURPOSE: To report optical coherence tomography (OCT) findings in order to detect subclinical alterations of the afferent visual pathways in spinocerebellar ataxia 3 (SCA-3). PATIENTS AND METHODS: Nine genetically confirmed patients (18 eyes) were evaluated with a complete ophthalmologic examination including visual acuity, colour vision, visual field test, and retinal nerve fibre layer (RNFL) and macular thickness with OCT Cirrus HD. A neurological examination was performed and the Scale for the Assessment and Rating of Ataxia (SARA score) was determined in all patients. RESULTS: The mean RNFL thickness was 77.39 microns, standard deviation (SD) was ± 5.93. In 15 eyes (83.33%), the mean RNFL thickness was lower than the population average considering age and sex. In 10 cases, there was a reduction of the RNFL thickness in the superior sector, eight in the inferior and four in the nasal. Temporal sector RNFL thickness was preserved in all eyes. RNFL thickness was inversely correlated to SARA score (r=-0.64, P=0.012). The mean macular thickness was 252.61 microns, SD ± 22.80, being inferior respecting average population in only two eyes (11.11%). In four patients, (eight eyes) OCT studies were performed during a mean follow-up of 14.25 months, and in five eyes (62.50%) there was a mild trend to a RNFL thickness decrease in this period. CONCLUSION: A mild and progressive decrease in RNFL thickness can be observed in SCA-3 patients. A negative correlation exists between an anatomic marker (RNFL thickness) and a clinical severity scale (SARA score); thus, RNFL thickness could be considered as a promising biomarker of the disease.
PURPOSE: To report optical coherence tomography (OCT) findings in order to detect subclinical alterations of the afferent visual pathways in spinocerebellar ataxia 3 (SCA-3). PATIENTS AND METHODS: Nine genetically confirmed patients (18 eyes) were evaluated with a complete ophthalmologic examination including visual acuity, colour vision, visual field test, and retinal nerve fibre layer (RNFL) and macular thickness with OCT Cirrus HD. A neurological examination was performed and the Scale for the Assessment and Rating of Ataxia (SARA score) was determined in all patients. RESULTS: The mean RNFL thickness was 77.39 microns, standard deviation (SD) was ± 5.93. In 15 eyes (83.33%), the mean RNFL thickness was lower than the population average considering age and sex. In 10 cases, there was a reduction of the RNFL thickness in the superior sector, eight in the inferior and four in the nasal. Temporal sector RNFL thickness was preserved in all eyes. RNFL thickness was inversely correlated to SARA score (r=-0.64, P=0.012). The mean macular thickness was 252.61 microns, SD ± 22.80, being inferior respecting average population in only two eyes (11.11%). In four patients, (eight eyes) OCT studies were performed during a mean follow-up of 14.25 months, and in five eyes (62.50%) there was a mild trend to a RNFL thickness decrease in this period. CONCLUSION: A mild and progressive decrease in RNFL thickness can be observed in SCA-3patients. A negative correlation exists between an anatomic marker (RNFL thickness) and a clinical severity scale (SARA score); thus, RNFL thickness could be considered as a promising biomarker of the disease.
Authors: Stephanie B Syc; Christina V Warner; Girish S Hiremath; Sheena K Farrell; John N Ratchford; Amy Conger; Teresa Frohman; Gary Cutter; Laura J Balcer; Elliot M Frohman; Peter A Calabresi Journal: Mult Scler Date: 2010-06-08 Impact factor: 6.312
Authors: Sarah Stricker; Timm Oberwahrenbrock; Hanna Zimmermann; Jan Schroeter; Matthias Endres; Alexander U Brandt; Friedemann Paul Journal: PLoS One Date: 2011-07-29 Impact factor: 3.240
Authors: Xiaoxuan Liu; Aditya Uday Kale; Nicholas Capewell; Nicholas Talbot; Sumiya Ahmed; Pearse A Keane; Susan Mollan; Antonio Belli; Richard J Blanch; Tonny Veenith; Alastair K Denniston Journal: BMJ Open Date: 2019-11-07 Impact factor: 2.692
Authors: Thomas Ihl; Ella M Kadas; Timm Oberwahrenbrock; Matthias Endres; Thomas Klockgether; Jan Schroeter; Alexander U Brandt; Friedemann Paul; Martina Minnerop; Sarah Doss; Tanja Schmitz-Hübsch; Hanna G Zimmermann Journal: Cerebellum Date: 2020-08 Impact factor: 3.847
Authors: Petya Bogdanova-Mihaylova; Helena Maria Plapp; Hongying Chen; Anne Early; Lorraine Cassidy; Richard A Walsh; Sinéad M Murphy Journal: Tomography Date: 2021-12-08
Authors: Vasileios Toulis; Sílvia García-Monclús; Carlos de la Peña-Ramírez; Rodrigo Arenas-Galnares; Josep F Abril; Sokol V Todi; Naheed Khan; Alejandro Garanto; Maria do Carmo Costa; Gemma Marfany Journal: Cell Rep Date: 2020-11-10 Impact factor: 9.423