Prevalence, and Predictors, of Vascular Cognitive Impairment in CADASIL
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Publication Date
2022-05-23Journal Title
Neurology
ISSN
0028-3878
Publisher
Lippincott, Williams & Wilkins
Type
Article
This Version
VoR
Metadata
Show full item recordCitation
Jolly, A., Nannoni, S., Edwards, H., Morris, R., & Markus, H. (2022). Prevalence, and Predictors, of Vascular Cognitive Impairment in CADASIL. Neurology https://doi.org/10.1212/WNL.0000000000200607
Abstract
Abstract
Background and Objective
CADASIL is the most common monogenic form of stroke and early onset dementia. We determined the prevalence of vascular cognitive impairment (VCI) in a cohort of CADASIL patients, and investigated which factors were associated with VCI risk, including clinical, genetic and MRI parameters.
Methods
Cognition was assessed in genetically confirmed CADASIL patients (n = 176) and healthy controls (n= 265) (mean(SD) age 50.95(11.35) v 52.37(7.93) years), using the Brief Memory and Executive Test (BMET) and the Montreal Cognitive Assessment (MoCA). VCI was defined according to previously validated cut-offs. We determined the prevalence of VCI and its associations with clinical risk factors, mutation location (EGFr 1-6 versus EGFr 7-34), and MRI markers of small vessel disease.
Results
VCI was more common in CADASIL than controls; 39.8 v 10.2% on BMET 47.7% v 19.6% of MOCA. CADASIL patients had worse performance across all cognitive domains. History of stroke was associated with VCI on the BMET (OR 2.12, 95% CI [1.05, 4.27] p = 0.04) and on the MoCA
(OR 2.55 [1.21, 5.41] p = 0.01), after controlling for age and sex. There was no association of VCI with mutation site. Lacune count was the only MRI parameter independently associated with VCI on the BMET (OR: 1.63, 95% CI [1.10, 2.41], p = 0.014), after controlling for other MRI parameters.
These associations persisted after controlling for education in the sensitivity analyses.
Conclusions
VCI is present in almost half of CADASIL patients with a mean age of 50. Stroke and lacune count on MRI were both independent predictors of VCI on the BMET.
Sponsorship
The UK Familial Cerebral Small Vessel Disease is funded by the British Heart Foundation programme grant (RG/4/32218). AJs studentship is supported by the Stroke Association R4VaD grant (RRZA/199). SNs salary is funded by an MRC experimental medicine grant (MR/N026896/1). HSM is supported by an NIHR Senior Investigator award. Recruitment was supported by the NIHR Clinical Research Network. This research was
supported by the NIHR Cambridge Biomedical Research Centre (BRC-1215-20014) and the Cambridge BHF Centre of Research Excellence [RE/18/1/34212].
Identifiers
External DOI: https://doi.org/10.1212/WNL.0000000000200607
This record's URL: https://www.repository.cam.ac.uk/handle/1810/335076
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