Macular vessel density is associated with cognitive function in preclinical p.R544C NOTCH3 mutation carriers: Erratum
Bibliographic record
Abstract
In the article “Macular vessel density is associated with cognitive function in preclinical p.R544C NOTCH3 mutation carriers: Journal of the Chinese Medical Association. 88(8):601-608, April 2025.”, portions of the abstract and lay summary are disorganized due to certain text being placed under incorrect subtitles, which may affect clarity and readability. To ensure accuracy and proper interpretation, the corrected abstract and lay summary are provided below. Abstract Background: Identifying early and covert manifestations of cerebral small vessel disease remains a major challenge. In Taiwan, the p.R544C NOTCH3 mutation, which causes cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is common, yet its preclinical microvascular correlates are not well understood. We investigated whether macular microvascular changes, quantified by optical coherence tomography angiography (OCTA), are associated with cognitive performance and white matter hyperintensities (WMH) in asymptomatic NOTCH3 mutation carriers. Methods: This cross-sectional study included genetically confirmed p.R544C NOTCH3 carriers without stroke history or cognitive complaints. All participants underwent OCTA to measure parafoveal vessel density in the superficial retinal plexus (SRP), brain MRI to quantify whole-brain WMH, and neuropsychological testing. Multivariable regression models adjusted for age, sex, and vascular risk factors were used to assess associations. Results: Forty-nine asymptomatic carriers (median age 61 years; 44.9% men) were analyzed. Higher parafoveal SRP vessel density was independently associated with lower Montreal Cognitive Assessment (MoCA) scores (β = −0.31; 95% CI: −0.15 to 0.00; p = 0.044) and slower Color Trails Test performance (CTT-1: β = −0.60; p < 0.001; CTT-2: β = −0.45; p = 0.008). WMH volume correlated with MoCA scores but not with SRP vessel density, suggesting distinct microvascular processes in retina and brain. Conclusion: Even in the absence of clinical symptoms, increased macular SRP vessel density in NOTCH3 mutation carriers is linked to subtle cognitive decline and may reflect an autoregulatory response to early capillary plexus dysfunction. OCTA offers a rapid, noninvasive biomarker for identifying individuals at risk for CADASIL-related cognitive impairment before overt neurological manifestations appear.1 Lay Summary: The p.R544C NOTCH3 gene mutation increases the risk of CADASIL, a rare small vessel disease that can cause stroke and dementia after years of silent damage. We studied 49 symptom-free carriers using optical coherence tomography angiography (OCTA), a quick eye scan, along with brain MRI and cognitive tests. Higher macular vessel density was associated with poorer memory and attention, even when MRI changes were mild, which may reflect early compensatory blood flow. These findings suggest that a simple OCTA scan may detect early brain changes in CADASIL before symptoms appear. The authors apologize for the mistake and for any confusion this may have caused.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.003 | 0.027 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.002 | 0.004 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; both teacher heads agree on what is shown here.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".