Multimodal imaging analyses in patients with genetic and sporadic forms of small vessel disease View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2019-12

AUTHORS

Ko Woon Kim, Hunki Kwon, Young-Eun Kim, Cindy W. Yoon, Yeo Jin Kim, Yong Bum Kim, Jong Min Lee, Won Tae Yoon, Hee Jin Kim, Jin San Lee, Young Kyoung Jang, Yeshin Kim, Hyemin Jang, Chang-Seok Ki, Young Chul Youn, Byoung-Soo Shin, Oh Young Bang, Gyeong-Moon Kim, Chin-Sang Chung, Seung Joo Kim, Duk L. Na, Marco Duering, Hanna Cho, Sang Won Seo

ABSTRACT

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is thought to be a pure genetic form of subcortical vascular cognitive impairment (SVCI). The aim of this study was to compare white matter integrity and cortical thickness between typical CADASIL, a genetic form, and two sporadic forms of SVCI (with NOTCH3 and without NOTCH3 variants). We enrolled typical CADASIL patients (N = 11) and SVCI patients [with NOTCH3 variants (N = 15), without NOTCH3 variants (N = 101)]. To adjust the age difference, which reflects the known difference in clinical and radiologic courses between typical CADASIL patients and SVCI patients, we constructed a W-score of measurement for diffusion tensor image and cortical thickness. Typical CADASIL patients showed more frequent white matter hyperintensities in the bilateral posterior temporal region compared to SVCI patients (p < 0.001, uncorrected). We found that SVCI patients, regardless of the presence of NOTCH3 variants, showed significantly greater microstructural alterations (W-score, p < 0.05, FWE-corrected) and cortical thinning (W-score, p < 0.05, FDR-corrected) than typical CADASIL patients. In this study, typical CADASIL and SVCI showed distinct anatomic vulnerabilities in the cortical and subcortical structures. However, there was no difference between SVCI with NOTCH3 variants and SVCI without NOTCH3 variants. More... »

PAGES

787

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/s41598-018-36580-0

DOI

http://dx.doi.org/10.1038/s41598-018-36580-0

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1111672814

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/30692550


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433 https://www.grid.ac/institutes/grid.254224.7 schema:alternateName Chung-Ang University
434 schema:name Department of Neurology, Chung-Ang University College of Medicine, Seoul, Korea
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436 https://www.grid.ac/institutes/grid.256753.0 schema:alternateName Hallym University
437 schema:name Department of Neurology, Chuncheon Sacred Heart Hospital, Hallym University College of Medicine, Chuncheon, Korea
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439 https://www.grid.ac/institutes/grid.264381.a schema:alternateName Sungkyunkwan University
440 schema:name Department of Clinical Research Design & Evaluation, SAIHST, Sungkyunkwan University, Seoul, Korea
441 Department of Neurology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
442 Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
443 Neuroscience Center, Samsung Medical Center, Seoul, Korea
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446 schema:name Department of Neurology, Kyung Hee University Hospital, Seoul, Korea
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448 https://www.grid.ac/institutes/grid.411545.0 schema:alternateName Chonbuk National University
449 schema:name Department of Neurology, Chonbuk National University Medical School & Hospital, Jeonju, Korea
450 Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
451 rdf:type schema:Organization
452 https://www.grid.ac/institutes/grid.412010.6 schema:alternateName Kangwon National University
453 schema:name Department of Neurology, Kangwon National University Hospital, Kangwon National University College of Medicine, Chuncheon, Korea
454 rdf:type schema:Organization
455 https://www.grid.ac/institutes/grid.47100.32 schema:alternateName Yale University
456 schema:name Department of Biomedical Engineering, Hanyang University, Seoul, Korea
457 Department of Neurology, Yale University School of Medicine, New Haven, Connecticut, USA
458 rdf:type schema:Organization
459 https://www.grid.ac/institutes/grid.49606.3d schema:alternateName Hanyang University
460 schema:name Department of Biomedical Engineering, Hanyang University, Seoul, Korea
461 rdf:type schema:Organization
 




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