ArticleJournal of stroke2022
Elderly CADASIL patients with intact neurological status.
Article in Journal of stroke, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
What it found
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Who cites it
14 citing papers in PubMed, 16 citations in OpenAlex.
- Prognostic Significance ofNeurology. Genetics · 2026Article
- Clinical Impact of NOTCH3 Variant Location After First Stroke in CADASIL.Annals of clinical and translational neurology · 2026Article
- Association Between Vascular NOTCH3 Aggregation and Disease Severity in a CADASIL Cohort - Implications for NOTCH3 Variant-Specific Disease Prediction.Annals of neurology · 2025Article
- Is migraine a common manifestation of CADASIL? Arguments Pros.The journal of headache and pain · 2025Article
- NOTCH3 Variant Position Affects the Phenotype at the Pluripotent Stem Cell Level in CADASIL.Neuromolecular medicine · 2025Article
- Disease Severity Staging System for NOTCH3-Associated Small Vessel Disease, Including CADASIL.JAMA neurology · 2025Article
- Genetic diagnosis of individuals at risk of CADASIL: prospect for future therapeutic development.Journal of neurology · 2024Article
- Early remodeling and loss of light-induced dilation of retinal small arteries in CADASIL.Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism · 2024Article
- Association ofNeurology · 2024Article
- The protocol for an observational Australian cohort study of CADASIL: The AusCADASIL study.Cerebral circulation - cognition and behavior · 2024Article
- The CADA-MRIT: An MRI Inventory Tool for Evaluating Cerebral Lesions in CADASIL Across Cohorts.Neurology · 2023Article
- Dental procedure induced cerebellar haemorrhage with visual tilt and unsuspected CADASIL.BMJ case reports · 2023Article
- Bibliometric Analysis of Research on Migraine-Stroke Association from 2013 to 2023.Journal of pain research · 2023Article
- Novel grading system for CADASIL severity: A multicenter cross-sectional study.Cerebral circulation - cognition and behavior · 2023Article
Corrections and comments
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Authors and funding
10 authors at 3 institutions in 2 countries.
Funding
Abstract
background and purposeCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is one of the most devastating cerebral small vessel diseases. However, despite its progression with aging, some patients remain neurologically intact (Nint) even when they get older. Their main characteristics are poorly known. We aimed to delineate their clinical, imaging, and molecular features.
methodsIndividuals aged over 65 years were selected from a cohort of 472 CADASIL patients. Subjects who had no focal deficit, cognitive impairment, or disability were considered Nint. Their demographic, genetic, clinical, and imaging features were compared to those with permanent neurological symptoms (Nps).
resultsAmong 129 patients, 23 (17.8%) individuals were considered Nint. The frequency of vascular risk factors and NOTCH3 cysteine mutations in epidermal growth factor-like repeat (EGFr) domains 7-34 did not differ between Nint and Nps patients but Nint patients had less stroke events and were more likely to have migraine with aura. The number of lacunes and microbleeds and degree of brain atrophy were lower in the Nint group, but the volume of white matter hyperintensities did not differ between the two groups.
conclusionsNearly one in five CADASIL patients can remain Nint after the age of 65 years. Their clinical and imaging profile differed from that of other age-matched CADASIL patients. The location of NOTCH3 mutation inside or outside EGFr domains 1-6 cannot fully explain this discrepancy. The factors involved in their relative preservation of brain tissue from severe damage despite aging remain to be determined.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.