Data from: Is retina affected in Huntington's disease? Is optical coherence tomography a good biomarker?
Abstract
Prospective cross-sectional measurement of peripapillary retinal nerve fiber layer thickness (RNFL) and total macular volume (TMV) measured by spectral domain optical coherence tomography (OCT) of retina, Pelli-Robson Contrast Sensitivity test, Farnsworth 15 Hue Color discrimination test, and Unified Huntington’s disease Rating Scale (UHDRS).
https://doi.org/10.5061/dryad.ncjsxksxr
Description of the data and file structure
OCT measurement of patients with Huntington's disease.
Files and variables
File: data.csv
Description: OCT Measurements in Huntington's Disease and Healthy Controls
Variables:
- record_id: unique id of a subject
- group: whether the subject is in Huntington's Disease patient group or healthy controls group
- gender: gender of the subject
- age: age range group of the subject at the time of OCT measurement in years
- visit: number of visit (first or second measurement)
- disease_length: disease length range group at the time of OCT measurement in years. 'NA' means 'not applicable', it is in healthy conrols that do not have the disease.
- triplet_longer: CAG repeat number. 'NA' means 'not applicable', healthy controls do not have CAG expansion.
- pelli_robson_both_eyes: Pelli Robson Contrast Sensitivity using both eyes. 'NA' means 'not available'. It was not measured in healthy controls. For the patients, some of them where not able to undergo this examination due to the severity of the disease.
- pelli_robson: Pelli Robson Contrast Sensitivity for the respective eye. 'NA' means 'not available'. It was not measured in healthy controls. For the patients, some of them where not able to undergo this examination due to the severity of the disease.
- eye: whether OCT measurements are for the right or the left eye of the subject
- uhdrs_tfc: Unified Huntington's Disease Rating Scale Total Functional Capacity. 'NA' means 'not applicable', it was not measured in healthy controls.
- uhdrs_tms: Unified Huntington's Disease Rating Scale Total Motor Score. 'NA' means 'not applicable', it was not measured in healthy controls.
- hue15: Farnsworth D-15 Color test. 'NA' means 'not applicable', it was not measured in healthy controls.
- oct_rnfl_g: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer global. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_rnfl_pmb: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer Peripapilary Segment. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_rnfl_nt_ratio: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer Nasal to Temporal Ratio. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_rnfl_ns: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer Nasal Superior Segment. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_rnfl_n: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer Nasal Segment. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_rnfl_ni: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer Nasal Inferior Segment. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_rnfl_ti: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer Temporal Inferior Segment. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_rnfl_t: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer Temporal Segment. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_rnfl_ts: Optical Cohorence Tomography measurement of thickness of Retinal Nerve Fiber Layer Temporal Superior Segment. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
- oct_macular_vol: Optical Cohorence Tomography measurement of total macular volume. 'NA' means 'not available'; some subjects did not finish the measurement due to fatigue.
Human subjects data
The patients consented with de-identified data publication. For identifiable fields, only ranges (e.g. for age and disease length) are published.
Patients were assessed by Unified Huntington's disease Rating Scale (UHDRS). Pelli-Robson Contrast Sensitivity test Chart 4K, which uses a single large letter size (20/60 optotype) with contrast varying across groups of letters, was used to evaluate Contrast Sensivity. A Pelli-Robson score of 1.5 to 2.25 indicates normal Contrast Sensitivity, a score of 0.9 to 1.35 indicates moderate contrast loss, a score of 0.3 to 0.75 severe contrast loss and a score of 0.15 and less indicates profound contrast loss. Farnsworth D-15 Color test was performed to evaluate Color Discrimination. The results of the Farnworth D-15 test determine color perception or defects in deutan, protan or tritan discrimination.
Using a spectral-domain OCT machine (Heidelberg Spectralis), without pupil dilation, we obtained a circular scan manually centered on the optic nerve head (diameter 3.4mm, Automated Real-Time - ART 100) and a macular volume scan centered on the fovea (63 lines, ART 20). The thickness of the peripapillary retinal nerve fiber layer (RNFL) in µm and total macular volume (TMV) in mm3 was automatically determined by the instrument (Heyex version 5.8). All retinal images were checked for scan quality. We analyzed the mean peripapillary RNFL thickness (RNFL-G) as the main measure of axonal health in the retina and the thickness of RNFL in the temporal peripapillary segment (RNFL-T) that represents the most vulnerable retinal fibers. We also used total macular volume (TMV) measured in the area of a 6 mm wide circular mask to represent global retinal changes.