Quantitative MRI in CADASIL: Correlation with disability and cognitive performance

Dichgans, M.; Filippi, M.; Brüning, R.; Iannucci, G.; Berchtenbreiter, C.; Minicucci, L.; Uttner, I.; Crispin, A.; Ludwig, H.; Gasser, T.; Yousry, T.A.

doi:10.1212/wnl.52.7.1361

Cited by 120Cerebrovascular and genetic disordersNeurological Disease Mechanisms and TreatmentsGenetic Neurodegenerative Diseases

Quantitative MRI in CADASIL: Correlation with disability and cognitive performance

Dichgans, M., Filippi, M., Brüning, R., Iannucci, G., Berchtenbreiter, C., Minicucci, L., Uttner, I., Crispin, A. +3 more·1999·Neurology

DOI 10.1212/wnl.52.7.1361

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Abstract

OBJECTIVE: To study correlations between total lesion load on brain MRI and clinical features, and to evaluate the influence of demographic variables on quantitative MRI variables in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). BACKGROUND: CADASIL is a hereditary form of small-vessel disease caused by mutations within the Notch3 gene. MRI abnormalities have been found both in asymptomatic and symptomatic CADASIL individuals. METHODS: Quantitative measurements on cerebral MRI were performed in 64 CADASIL individuals. MRI lesions were quantified using a semi-automated segmentation technique based on local thresholds. RESULTS: MRI total lesion volume correlated significantly with disability (Rankin Scale) on both T1- and proton density (PD)-weighted images. There was a significant inverse correlation between total lesion volume and overall cognitive performance as determined by the Mini-Mental State Examination. Age but not sex was correlated with lesion load both on T1- and PD-weighted images. There was no detectable influence of the Notch3 genotype on quantitative MRI variables. CONCLUSIONS: This study demonstrates correlations between MRI lesion volume and clinical characteristics in CADASIL. Longitudinal studies are now warranted to investigate whether quantitative MRI could be used as an adjunct outcome measure in future therapeutic trials in CADASIL.

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Overall lesion load on T1-weighted MRI is associated with global disability, often measured by the modified Rankin Scale, and with performance on different cognitive domains in CADASIL.
“The overall lesion load observed on T1-weighted MR images has been associated with the degree of global disability, often quantified using the modified Rankin Scale [4], and with performance on different cognitive domains [18, 76].”
From Clinical and research applications of magnetic resonance imaging in the study of CADASIL (2019) · cites this paper as [18]

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“White matter lesions, detected on T2 weighted brain MRI images, are present prior to the onset of clinical symptoms and correlate with disease severity [15], but are not a reliable predictor of disease progression [16].”
Background
Hereditary Vascular Dementia Linked to Notch 3 Mutations: CADASIL in British Families
Thomas, N., Morris, C. M., Scaravilli, F., JOHANSSON, J., Rossor, M. N., LANGE, R. D., Clair, D. S., Nicoll, J. A. R., Blank, C., Coulthard, A., et al. (2000) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.2000.tb06379.x · Cited by 34 · see citing works
Effects of Cerebral Blood Flow and White Matter Integrity on Cognition in CADASIL Patients
Yin, X., Zhou, Y., Yan, S., & Lou, M. (2019) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2018.00741 · Cited by 33 · see citing works
Structural and metabolic brain abnormalities in preclinical cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy
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Description of a simple test for CADASIL disease and determination of mutation frequencies in sporadic ischaemic stroke and dementia patients
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Influence of vascular risk factors and neuropsychological profile on functional performances in CADASIL: results from the MIcrovascular LEukoencephalopathy Study (MILES)
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A magnetic resonance imaging study of the cervical cord of patients with CADASIL
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Self-citation
Altered Brain Glucose Metabolism Assessed by 18F-FDG PET Imaging Is Associated with the Cognitive Impairment of CADASIL
Su, J., Huang, Q., Ren, S., Xie, F., Zhai, Y., Guan, Y., Liu, J., & Hua, F. (2019) · Neuroscience · doi:10.1016/j.neuroscience.2019.07.048 · Cited by 27 · see citing works
Nocturnal blood pressure dip in CADASIL
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Whole brain diffusion tensor imaging histogram analysis in vascular cognitive impairment
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“As reported for other small-vessel diseases, the extent of these MRI signal abnormalities cannot predict the clinical severity [9,10].”
Background
Genetic Animal Models of Cerebral Vasculopathies
Lee, J. H., Bacskai, B. J., & Ayata, C. (2011) · Progress in molecular biology and translational science · doi:10.1016/b978-0-12-394596-9.00002-0 · Cited by 21 · see citing works
“It has been difficult to characterize genotype–phenotype correlations, because marked phenotypic heterogeneity exists among CADASIL families and even among the affected members of a single family.(177,178) It is possible that the presence of common vascular risk factors or polymorphisms in other genes that modulate NO…”
Background
Formes pauci symptomatiques de CADASIL après 60 ans
Mourad, A., Levasseur, M., Bousser, M., & Chabriat, H. (2006) · Revue Neurologique · doi:10.1016/s0035-3787(06)75085-8 · Cited by 20 · see citing works
CADASIL as a useful medical model and genetic form of vascular depression
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WMH Contributions to Cognitive Impairment: Rationale and Design of the Diverse VCID Study
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“Similarly, phenotypic variation was shown both within families and between families of Finnish origin for R133C mutation and the lack of genotype–phenotype relationships in CADASIL was acknowledged by previous studies [15,23–25].”
Result
CADASIL and genetics of cerebral ischaemia
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Correlation of cognitive status, MRI‐ and SPECT‐imaging in CADASIL patients
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Self-citation
Enlarged perivascular spaces are associated with white matter injury, cognition and inflammation in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
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NOTCH3 gene mutations in subjects clinically suspected of CADASIL
Mosca, L., Marazzi, R., Ciccone, A., Santilli, I. M., Bersano, A., Sansone, V., Grosso, E., Mandrile, G., Giachino, D. F., Adobbati, L., et al. (2011) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2011.04.019 · Cited by 14 · see citing works
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Report of two Chinese families and a review of Mainland Chinese CADASIL patients
Yin, X., Ding, M., Zhang, B., Liu, J., Zhang, L., Wang, P., Zhou, F., & Zhao, G. (2009) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2008.12.011 · Cited by 11 · see citing works
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Hallazgos clínicos y de neuroimagen en una familia con CADASIL asociada a la mutación C475T
Sempere, Á. P., Pérez‐Tur, J., Barragán, N. G., Martínez, J. S., Martínez, V. M., & de Rodas, S. M. C. (2004) · Revista de Neurología · doi:10.33588/rn.3801.2003328 · Cited by 6 · see citing works
A novel mutation (C271F) in the Notch3 gene in a Chinese man with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
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Monogenic Causes of Stroke
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Self-citation
Vascular Dementia
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A Japanese Case of CADASIL with a Rare Mutation in Exon 24 of the NOTCH3 Gene
Ebihara, Y., Mochizuki, H., Ishii, N., Mizuta, I., Shiomi, K., Mizuno, T., & Nakazato, M. (2018) · Internal Medicine · doi:10.2169/internalmedicine.0723-17 · Cited by 4 · see citing works
“The relationship between the genotype and phenotype of NOTCH3 mutations has been thought to be weak (7, 11).”
Background
Brain MRI in Monogenic Cerebral Small Vessel Diseases: A Practical Handbook
Ulivi, L., Cosottini, M., Migaleddu, G., Orlandi, G., Giannini, N., Siciliano, G., & Mancuso, M. (2021) · Current Molecular Medicine · doi:10.2174/1566524021666210510164003 · Cited by 3 · see citing works
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Neuroradiologische Aspekte bei Encephalitis disseminata
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Enlarged Perivascular Spaces are Associated with White Matter Injury, Brain Atrophy, Cognitive Decline and Markers of Inflammation in an Autosomal Dominant Vascular Neurodegenerative Disease (CADASIL)Preprint
Karvelas, N., Oh, B. C., Wang, E., Cobigo, Y., Tsuei, T., Fitzsimons, S., Ehrenberg, A. J., Geschwind, M. D., Schwartz, D., Kramer, J. H., et al. (2023) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2023.08.17.553732 · Cited by 3 · see citing works
Dementia and management of vascular cognitive impairment
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Zidverc-Trajkovic, J. J., Pavlovic, A. M., Tomić, G., & Mandić, G. (2008) · Cited by 2 · see citing works
The Neurovascular Neuropsychology of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) and Mitochondrial Encephalomyopathy Lactic Acidosis and Stroke-Like Episodes (MELAS)
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Clinicogenetic and Pathologic Characteristics of CADASIL
Roberts, K. A., O’Rourke, K., & Ross, O. A. (2012) · Springer eBooks · doi:10.1007/978-0-85729-209-4_6 · Cited by 1 · see citing works
A Chinese CADASIL family with p.R578C mutation at exon 11 of the NOTCH3 gene
Wu, X., Zhang, A., Li, Y., Lei, X., Guo, S., Tian, T., Gong, H., & He, D. (2021) · Clinical Neurology and Neurosurgery · doi:10.1016/j.clineuro.2021.106833 · Cited by 1 · see citing works
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Some Translations in Vascular Neurology
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Microvascular Pathology in Familial Multiinfarct Dementia or CADASIL: Implications for Cerebral Blood Flow
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CADASIL: a genetic model of arteriolar degeneration, white matter injury, and dementia in later life
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Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) and Mitochondrial Encephalomyopathy Lactic Acidosis and Stroke-like Episodes (MELAS)
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Magnetic resonance imaging characteristics of CADASIL
van den Boom, R. (2006)

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Characteristic MR Lesion Pattern and Correlation of T1 and T2 Lesion Volume with Neurologic and Neuropsychological Findings in Cerebral Autosomal Dominant Arteriopathy with
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Heritability of MRI Lesion Volume in CADASIL
ChristianOpherk, NilsPeters, MarkusHoltmannspötter, AndreasGschwendtner, BertramMüller-Myhsok, & MartinDichgans (2006) · Stroke
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Lacunar lesions are independently associated with disability and cognitive impairment in CADASIL
Viswanathan, A., Gschwendtner, A., Guichard, J. P., Buffon, F., Cumurciuc, R., O'Sullivan, M., Holtmannspötter, M., Pachai, C., Bousser, M. G., Dichgans, M., et al. (2007) · Neurology · doi:10.1212/01.wnl.0000265221.05610.70 · Cited by 155 · see citing works
Self-citation

Authors: Dichgans, M., Filippi, M., Brüning, R., Iannucci, G., Berchtenbreiter, C., Minicucci, L., Uttner, I., Crispin, A., Ludwig, H., Gasser, T., & Yousry, T. A.
Affiliations: University of Milan (IT)
From the Departments of Neurology (Drs. DichgansUttner, and Gasser, and H. Ludwig), Medical Informatics, Biometry, and Epidemiology (A. Crispin), and Neuroradiology (Dr. Yousry), Klinikum Grosshadern, Ludwig-Maximilians-University, Munich, Germany; the Neuroimaging Research Unit (Drs. Filippi, Iannucci, and Minicucci), Department of Neuroscience, Scientific Institute Ospedale San Raffaele, University of Milan, Italy; and the Institute for Diagnostic Radiology (Drs. Brüning and Berchtenbreiter),...
ORCID: Dichgans, M.
Filippi, M.
Uttner, I.
Crispin, A.
Gasser, T.
Yousry, T.A.
Venue: Neurology
Publisher: Ovid Technologies (Wolters Kluwer Health)
Published: 1999-04
Volume: 52
Issue: 7
Pages: 1361-1361
ISSN: 0028-3878 (ISSN-L)
1526-632X
DOI: 10.1212/wnl.52.7.1361
OpenAlex: W2150080259
Cited by: 120
References: 31
Citations by year: 2026: 2 · 2025: 1 · 2024: 3 · 2023: 3 · 2021: 3 · 2020: 2 · 2019: 3 · 2018: 2 · 2017: 4 · 2016: 3 · 2015: 1 · 2014: 4 · 2013: 6 · 2012: 3
FWCI: 5.76
Type: journal article
Language: en
Open access: closed
Locations: Neurology (publishedVersion)
PubMed (publishedVersion)
UCL Discovery (University College London) (submittedVersion)
Topics: Health Sciences › Medicine › Neurology › Cerebrovascular and genetic disorders
Life Sciences › Neuroscience › Neurology › Neurological Disease Mechanisms and Treatments
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
Keywords: CADASIL, NOTCH3 gene, cerebral small vessel disease, quantitative MRI, lesion volume, T1-weighted imaging, proton density-weighted imaging, disability, cognitive performance, outcome measures
MeSH terms: Adult, Aged, Brain, Cerebral Arterial Diseases, Cerebral Infarction, Cognition, Disability Evaluation, Female, Humans, Leukoencephalopathy, Progressive Multifocal, Magnetic Resonance Imaging, Male, Middle Aged

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Quantitative MRI in CADASIL: Correlation with disability and cognitive performance

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