scholarly journals Longitudinal Assessment Using Optical Coherence Tomography in Patients with Friedreich’s Ataxia

Tomography ◽  
2021 ◽  
Vol 7 (4) ◽  
pp. 915-931
Author(s):  
Petya Bogdanova-Mihaylova ◽  
Helena Maria Plapp ◽  
Hongying Chen ◽  
Anne Early ◽  
Lorraine Cassidy ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Disease Severity ◽  
Disease Duration ◽  
Age At Onset ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Optical Coherence ◽  
Longitudinal Assessment ◽  
Ocular Abnormalities ◽  
Rnfl Thickness

Ocular abnormalities occur frequently in Friedreich’s ataxia (FRDA), although visual symptoms are not always reported. We evaluated a cohort of patients with FRDA to characterise the clinical phenotype and optic nerve findings as detected with optical coherence tomography (OCT). A total of 48 patients from 42 unrelated families were recruited. Mean age at onset was 13.8 years (range 4–40), mean disease duration 19.5 years (range 5–43), mean disease severity as quantified with the Scale for the Assessment and Rating of Ataxia 22/40 (range 4.5–38). All patients displayed variable ataxia and two-thirds had ocular abnormalities. Statistically significant thinning of average retinal nerve fibre layer (RNFL) and thinning in all but the temporal quadrant compared to controls was demonstrated on OCT. Significant RNFL and macular thinning was documented over time in 20 individuals. Disease severity and visual acuity were correlated with RNFL and macular thickness, but no association was found with disease duration. Our results highlight that FDRA is associated with subclinical optic neuropathy. This is the largest longitudinal study of OCT findings in FRDA to date, demonstrating progressive RNFL thickness decline, suggesting that RNFL thickness as measured by OCT has the potential to become a quantifiable biomarker for the evaluation of disease progression in FRDA.

scholarly journals Structural abnormalities associated with glaucoma using swept-source optical coherence tomography in patients with systemic sclerosis

2021 ◽  
Author(s):  
Cecilia Tito ◽  
Juliana Silvatti ◽  
Izabela N. F. Almeida ◽  
Elise V. Taniguchi ◽  
Tiago Prata ◽  
...  
Keyword(s):  
Blood Flow ◽  
Optical Coherence Tomography ◽  
Systemic Sclerosis ◽  
Ganglion Cell ◽  
Disease Duration ◽  
Optical Coherence ◽  
Swept Source ◽  
Rnfl Thickness ◽  
Structural Abnormalities ◽  
Digital Blood Flow

Abstract PurposeVasospasm represents an early event in systemic sclerosis (SSc). Ocular vasospasm may induce optic nerve head (ONH) damage and has been involved in the pathogenesis of glaucoma, especially normal-tension glaucoma (NTG). We aimed to investigate the presence of structural abnormalities associated with NTG using swept-source optical coherence tomography (SS-OCT) and to correlate the OCT parameters with clinical, capillaroscopy and digital blood flow measures in patients with SSc. MethodsIn this cross-sectional study, 40 patients with SSc and 23 age-matched controls were included. The following parameters were measured using SS-OCT: mean and sectoral retinal nerve fiber layer (RNFL) thickness, macular ganglion cell layer complex (GCC) thickness and ONH morphology. Nailfold capillaroscopy (NFC) and digital blood flow measurements using laser Doppler imaging (LDI) were performed in all subjects. ResultsPatients with SSc showed a thinner temporal RNFL than the controls (69.23 ± 11.74 versus 83.35 ± 20.19 µm, p=0.001). The other parameters were similar between the two groups. In SSc patients, there was an inverse correlation between the disease duration and the average, superior and inferior RNFL thickness and the GCC thickness and between Raynaud’s phenomenon duration and the average RNFL and GCC thickness (p<0.05). NFC and LDI measurements did not show correlations with OCT parameters.ConclusionA thinner temporal RNFL and the correlation between Raynaud’s phenomenon and disease duration and structural abnormalities on OCT suggest the presence of early ganglion cell damage in patients with SSc. Although mild, these findings indicate the need to monitor ocular abnormalities in SSc.

scholarly journals Early retinal degeneration in Huntington's disease based on optical coherence tomography: a case-control study

2020 ◽  
Vol 1 (1) ◽  
pp. 18-24
Author(s):  
Sergey Svetozarskiy ◽  
Svetlana Kopishinskaya ◽  
Igor Smetankin
Keyword(s):  
Optical Coherence Tomography ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Duration ◽  
Case Control Study ◽  
Case Control ◽  
Optical Coherence ◽  
Rnfl Thickness ◽  
The Mean ◽  
Control Study

Background: The purpose of this study was to analyze optical coherence tomography (OCT) parameters of the choroid and retina in subjects with pre-manifest and manifest Huntington's disease (HD). Methods: In this case-control study, the retinal parameters of patients with genetically confirmed HD and healthy controls were evaluated using spectral-domain optical coherence tomography (SD-OCT). Genetic and neurological assessments were performed besides a thorough ophthalmological examination. Contrast Sensitivity (CS) logarithm was evaluated using the Freiburg Vision Test. The association between OCT parameters and clinical and genetic characteristics was studied. Results: A total of 91 subjects, including 60 HD subjects (60 eyes) and 31 control subjects (31 eyes) were eligible according to the inclusion and exclusion criteria. The range of the CAG (cytosine-adenine-guanine) repeat expansion size was 38–56 repeats, the mean ± standard deviation (SD) of the Unified HD Rating Scale (UHDRS) motor scores was 36.3±29.7, and disease duration was 13.7±7.2 years in HD subjects. Compared to the control group, significant decreases in the mean ganglion cell complex thickness and mean, temporal, superior, inferior, and nasal retinal nerve fiber layer (RNFL) thickness in HD subjects was revealed in OCT examination (P-values < 0.001, < 0.001, < 0.001, 0.023, 0.007 and 0.014, respectively). An inverse correlation between the disease duration and the mean RNFL thickness (r =- 0.470, P = 0.002) was found. Conclusions: Localization of retinal thickness loss shows a specific pattern of retinal neurodegeneration in HD, similar to Parkinson’s disease and mitochondrial diseases. The association with the disease duration confirms the progressive nature of these changes.

scholarly journals Comparison of Optical Coherence Tomography Angiography Metrics in Primary Angle-Closure Glaucoma and Normal-Tension Glaucoma

2020 ◽  
Author(s):  
Ruyue Shen ◽  
Yu Wang ◽  
Carol Cheung ◽  
Clement Tham ◽  
Poemen Chan
Keyword(s):  
Optical Coherence Tomography ◽  
Disease Severity ◽  
Optical Coherence Tomography Angiography ◽  
Normal Tension Glaucoma ◽  
Angle Closure ◽  
Optical Coherence ◽  
Fiber Layer ◽  
Primary Angle Closure Glaucoma ◽  
Rnfl Thickness ◽  
Similar Disease

Abstract Purpose: To investigate the peripapillary vascular metrics in early normal tension glaucoma (NTG) and early primary angle closure glaucoma (PACG) eyes using optical coherence tomography angiography (OCT-A).Methods: One or both eyes of each subjects were imaged for a 3x3mm peripapillary region by swept-source OCT-A (DRI-OCT Triton, Topcon, Japan) and assessed by an automated MATLAB program. OCT-A metrics including circumpapillary vessel density (cpVD) and fractal dimension (cpFD) were compared. Their association with visual field (VF) parameters and retinal nerve fiber layer (RNFL) thickness were determined.Results: Sixty-eight eyes of 51 PACG, 68 eyes of 48 NTG, and 68 eyes of 49 control subjects were cross-sectionally analyzed. NTG eyes had significantly lower global cpVD (52.369±0.781%) compared with PACG eyes (55.389±0.721%, P=0.004) that had comparable disease severity and average RNFL thickness. Multivariable analysis reviewed that, for PACG and NTG eyes, decreased cpVD ([PACG] β=-4.242; CI:-8.120, -0.363 vs [NTG] β=-5.531; CI:-9.472, -1.590) and cpFD ([PACG] β=-8.894;CI:-11.925, -5.864 vs [NTG] β=-12.064; CI:-17.095, -6.932) were associated with decreased RNFL thickness (all P≤0.032); with a stronger association between decrease cpFD and decreased RNFL thickness in NTG eyes (Fisher’s Z-test, P=0.045). Decreased cpVD was associated with decrease mean deviation (MD) in NTG eyes (β=-0.707; CI:-1.090, -0.324; P≤0.001) and not associated with the visual field parameters in PACG eyes. Conclusions: Early NTG had lower global cpVD compared with early PACG, despite similar disease severity and average RNFL thickness. Précis:Optical coherence tomography angiography observed a lower peripapillary microvascular perfusion in early NTG eyes compared with early PACG eyes, despite similar disease severity and retinal nerve fiber layer thickness.

Optical coherence tomography as a biomarker for disease severity in ALS

2020 ◽  
Author(s):  
Carmen Balian ◽  
Christopher Hudson ◽  
Saba Samet ◽  
Hyejung Jung ◽  
Faryan Tayyari ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Disease Severity ◽  
Optical Coherence

scholarly journals Functional Monitoring after Trabeculectomy or XEN Microstent Implantation Using Spectral Domain Optical Coherence Tomography and Visual Field Indices—A Retrospective Comparative Cohort Study

Biology ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 273
Author(s):  
Marc Schargus ◽  
Catharina Busch ◽  
Matus Rehak ◽  
Jie Meng ◽  
Manuela Schmidt ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Cohort Study ◽  
Visual Field ◽  
Spectral Domain ◽  
Optical Coherence ◽  
Treatment Groups ◽  
Rnfl Thickness ◽  
Comparative Cohort Study ◽  
The Mean ◽  
All Treatment

The aim of this study was to compare the efficacy of trabeculectomy (TE), single XEN microstent implantation (solo XEN) or combined XEN implantation and cataract surgery (combined XEN) in primary open-angle glaucoma cases, naïve to prior surgical treatment, using a monocentric retrospective comparative cohort study. Intraocular pressure (IOP) and the number of IOP-lowering drugs (Meds) were monitored during the first 24 months after surgery. Further disease progression was monitored using peripapillary retinal nerve fiber layer (RNFL) thickness examinations using spectral domain optical coherence tomography (OCT) as well as visual acuity (VA) and visual field (VF) tests. In the TE group (52 eyes), the mean IOP decreased from 24.9 ± 5.9 to 13.9 ± 4.2 mmHg (p < 0.001) and Meds decreased from 3.2 ± 1.2 to 0.5 ± 1.1 (p < 0.001). In the solo XEN (38 eyes) and the combined XEN groups, the mean IOP decreased from 24.1 ± 4.7 to 15.7 ± 3.0 mmHg (p < 0.001) and 25.4 ± 5.6 to 14.7 ± 3.2 mmHg (p < 0.001), while Meds decreased from 3.3 ± 0.8 to 0.8 ± 1.2 (p < 0.001) and 2.7 ± 1.2 to 0.4 ± 1.0 (p < 0.001), respectively. The VF and VA indices showed no sign of further deterioration, the RNFL thickness further decreased in all treatment groups after surgery. TE and XEN led to comparable reductions in IOP and Meds. Although the VA and VF indices remained unaltered, the RNFL thickness continuously decreased in all treatment groups during the 24-month follow-up.

scholarly journals Longitudinal assessment of optic nerve head changes using optical coherence tomography in a primate microbead model of ocular hypertension

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Anita S. Y. Chan ◽  
Tin Aung Tun ◽  
John C. Allen ◽  
Myoe Naing Lynn ◽  
Sai Bo Bo Tun ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Optic Nerve ◽  
Ocular Hypertension ◽  
Optic Nerve Head ◽  
Structural Damage ◽  
Structural Changes ◽  
Lamina Cribrosa ◽  
Optical Coherence ◽  
Longitudinal Assessment ◽  
The Mean

Abstract In humans, the longitudinal characterisation of early optic nerve head (ONH) damage in ocular hypertension (OHT) is difficult as patients with glaucoma usually have structural ONH damage at the time of diagnosis. Previous studies assessed glaucomatous ONH cupping by measuring the anterior lamina cribrosa depth (LCD) and minimal rim width (MRW) using optical coherence tomography (OCT). In this study, we induced OHT by repeated intracameral microbead injections in 16 cynomolgus primates (10 unilateral; 6 bilateral) and assessed the structural changes of the ONH longitudinally to observe early changes. Elevated intraocular pressure (IOP) in OHT eyes was maintained for 7 months and serial OCT measurements were performed during this period. The mean IOP was significantly elevated in OHT eyes when compared to baseline and compared to the control eyes. Thinner MRW and deeper LCD values from baseline were observed in OHT eyes with the greatest changes seen between month 1 and month 2 of OHT. Both the mean and maximum IOP values were significant predictors of MRW and LCD changes, although the maximum IOP was a slightly better predictor. We believe that this model could be useful to study IOP-induced early ONH structural damage which is important for understanding glaucoma pathogenesis.

Sign in / Sign up

Export Citation Format

Share Document