Dilated perivascular spaces in small-vessel disease: a study in CADASIL
Abstract
Background and Aim: Dilated perivascular spaces (dPVS) have previously been associated with aging and hypertension-related cerebral microangiopathy. However, their risk factors, radiological features and clinical relevance have been poorly evaluated in CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy), a unique model to investigate the pathophysiology of ischemic small-vessel disease. The purpose of this study was to investigate these different aspects in a large cohort of patients with this disorder. Methods: Demographic and MRI data of 344 patients from a prospective cohort study were analyzed. The severity of dPVS was evaluated separately in the anterior temporal lobes, subinsular areas, basal ganglia and white matter, using validated semiquantitative scales. Logistic and multiple linear regression models were used to determine the risk factors associated with the severity of dPVS in these different regions and their relationships with cognition, disability and the MRI markers of the disease (white matter hyperintensities (WMH) lacunar infarcts, microbleeds and brain parenchymal fraction (BPF)). Results: The severity of dPVS was found to increase with age regardless of cerebral area (p < 0.001). In contrast with dPVS in other locations, the severity of dPVS in the temporal lobes or subinsular areas was also found strongly and specifically related to the extent of WMH (p < 0.001). Conversely, no significant association was detected with lacunar volume, number of microbleeds or BPF. A high degree of dPVS in the white matter was associated with lower cognitive performances independently of age and other MRI markers of the disease including BPF (p ≤ 0.04). Conclusions: In CADASIL, the progression of the hereditary microangiopathy with aging may promote the dilation of perivascular spaces throughout the whole brain but with variable extent according to cerebral location. In temporal lobes and subinsular areas, dPVS are common MRI features and may share a similar pathogenesis with the extension of WMH during the course of the disease. dPVS may also participate in the development of cognitive decline in this model of small-vessel disease, and their large number in white matter may alert clinicians to a higher risk of cognitive decline in CADASIL.
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Yao et al. (2014) demonstrated that the severity of perivascular space dilatation was worsened in demented CADASIL patients and was associated with global cognitive functioning regardless of age.
“For example, Yao et al. (2014) demonstrated that the severity of PVS dilatation was worsened in demented CADASIL patients and was associated with global cognitive functioning, regardless of the age [75].”
From Clinical and research applications of magnetic resonance imaging in the study of CADASIL (2019) · cites this paper as [75]
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- Chabriat H, Joutel A, Dichgans M, Tournier- Lasserve E, Bousser MG: CADASIL. Lancet Neurol 2009; 8: 643-653.
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Experiencias educativas en el Parque Nacional de Doñana
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Chabriat, H., & Bousser, M. (2017) · EMC - Neurologia · doi:10.1016/s1634-7072(17)85562-8
Self-citationDietemann, J.-L., Draghici, S., Kremer, S., Bogorin, A., Eid, M. A., & Sanda, R. (2018) · Elsevier eBooks · doi:10.1016/b978-2-294-75394-7.00002-3
Rutten, J. W., Cerfontaine, M. N., Dijkstra, K. L., Mulder, A. A., Kruit, M. C., Koning, R. I., de Bot, S. T., van Nieuwenhuizen, K. M., Baelde, H. J., Berendse, H. W., et al. (2023) · medRxiv · doi:10.1101/2023.05.16.23289676
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Chabriat, H. (2024) · Springer eBooks · doi:10.1007/978-3-031-41777-1_6
Self-citationTroubles neurocognitifs vasculaires et post-AVC
Chabriat, H., Jabouley, A., Reyes, S., & Jouvent, É. (2017) · De Boeck Supérieur eBooks · doi:10.3917/dbu.rouss.2017.01.0037
Self-citationGuey, S., & Chabriat, H. (2026) · Encyclopédie médico-chirurgicale. Neurologie · doi:10.1016/s0246-0378(26)49795-x
Self-citationGuey, S., & Chabriat, H. (2026) · EMC - Neurologia · doi:10.1016/s1634-7072(26)52356-0
Self-citationKarkoska, K. A., Gollamudi, J., Williamson, B. J., Sawyer, R. P., Woo, D., Vagal, A. S., & Hyacinth, H. I. (2026) · British Journal of Haematology · doi:10.1111/bjh.70894
- Authors
- Yao, M., Hervé, D., Jouvent, E., Duering, M., Reyes, S., Godin, O., Guichard, J. P., Dichgans, M., & Chabriat, H.
- Affiliations
- Peking Union Medical College Hospital (CN)
- Sorbonne Paris Cité (FR)
- Munich Cluster for Systems Neurology (DE)
- Assistance Publique – Hôpitaux de Paris (FR)
- German Center for Neurodegenerative Diseases (DE)
- Inserm (FR)
- ORCID
- Venue
- Cerebrovascular Diseases
- Publisher
- S. Karger AG
- Published
- 2014
- Volume
- 37
- Issue
- 3
- Pages
- 155-163
- ISSN
- 1015-9770 (ISSN-L)
- 1421-9786
- OpenAlex
- W2075655093
- Cited by
- 71
- References
- 23
- Citations by year
- 2026: 4 · 2025: 4 · 2024: 10 · 2023: 4 · 2022: 4 · 2021: 6 · 2020: 4 · 2019: 7 · 2018: 8 · 2017: 7 · 2016: 1 · 2015: 7 · 2014: 4
- FWCI
- 4.13
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Cerebrovascular Diseases (publishedVersion)
- PubMed (publishedVersion)
- Open access LMU (Ludwid Maxmilian's Universitat Munchen) (submittedVersion)
- DZNE Pub (submittedVersion)
- Funders
- China Scholarship Council
- Deutsches Zentrum für Neurodegenerative Erkrankungen
- Assistance publique-Hôpitaux de Paris
- Stiftung zur Erforschung der Vaskulären Demenz
- Topics
- Health Sciences › Medicine › Neurology › Cerebrovascular and genetic disorders
- Health Sciences › Medicine › Neurology › Intracerebral and Subarachnoid Hemorrhage Research
- Life Sciences › Neuroscience › Neurology › Neurological Disease Mechanisms and Treatments
- Keywords
- dilated perivascular spaces, CADASIL, cerebral small vessel disease, white matter hyperintensities, cognitive decline, aging, temporal lobe, white matter, brain parenchymal fraction, microbleeds, lacunar infarction, cerebral microangiopathy
- MeSH terms
- Adult, Aged, Aged, 80 and over, Basal Ganglia, Brain, Cerebral Hemorrhage, Cognition Disorders, Dilatation, Pathologic, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Organ Size, Prospective Studies, Risk Factors, Severity of Illness Index, Temporal Lobe, Imaging, Three-Dimensional, CADASIL, Young Adult, Stroke, Lacunar
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