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It is characterized by steno\u2010occlusive changes at the terminal portion of the internal carotid artery (ICA), which is accompanied by variable development of the basal collaterals called moyamoya vessels. In this study, we investigate the potential for structural T1 magnetic resonance imaging (MRI) to help characterize MMD clinically, with the help of regionally distributed relative signal intensities (RRSIs) and volumes (RRVs). These RRSIs and RRVs provide the ability to characterize aspects of regional brain development and represent an extension to existing automated biomarker extraction technologies. This study included 269 MRI examinations from MMD patients and 993 MRI examinations from neurotypical controls, with regional biomarkers compared between groups with the area under the receiver operating characteristic curve (AUC). Results demonstrate abnormal presentation of RRSIs and RRVs in the insula (15\u2010 to 20\u2010year old cohort, left AUC: 0.74, right AUC: 0.71) and the lateral orbitofrontal region (5\u2010 to 10\u2010year old cohort, left AUC: 0.67; 15\u201320\u2009year cohort, left AUC: 0.62, right AUC: 0.65). Results indicate that RRSIs and RRVs may help in characterizing brain development, assist in the assessment of the presentation of the brains of children with MMD and help overcome standardization challenges in multiprotocol clinical MRI. Further investigation of the potential for RRSIs and RRVs in clinical imaging is warranted and supported through the release of open\u2010source software.<\/jats:p>","DOI":"10.1002\/jdn.10167","type":"journal-article","created":{"date-parts":[[2021,12,30]],"date-time":"2021-12-30T22:32:58Z","timestamp":1640903578000},"page":"146-158","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Structural abnormalities in paediatric moyamoya disease revealed by clinical magnetic resonance imaging, regionally distributed relative signal intensities and volumes"],"prefix":"10.1002","volume":"82","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5447-9174","authenticated-orcid":false,"given":"Prahar","family":"Ijner","sequence":"first","affiliation":[{"name":"Department of Computer Science St. Francis Xavier University  Antigonish Nova Scotia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4091-9970","authenticated-orcid":false,"given":"Grace","family":"Tompkins","sequence":"additional","affiliation":[{"name":"Department of Mathematics and Statistics St. Francis Xavier University  Antigonish Nova Scotia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0076-5882","authenticated-orcid":false,"given":"Tadashi","family":"Shiohama","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emi","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Division of Newborn Medicine, Department of Medicine Boston Children's Hospital  Boston Massachusetts USA"},{"name":"Athinoula A. Martinos Center for Biomedical Imaging Massachusetts General Hospital  Charlestown Massachusetts USA"},{"name":"Department of Radiology Harvard Medical School  Boston Massachusetts USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9604-3157","authenticated-orcid":false,"given":"Jacob","family":"Levman","sequence":"additional","affiliation":[{"name":"Department of Computer Science St. Francis Xavier University  Antigonish Nova Scotia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,1,13]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1161\/STROKEAHA.113.004273"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2007.130666"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-014-2582-5"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.307.6908.852"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/1352458515579439"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1161\/STROKEAHA.111.621300"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2012.01.021"},{"issue":"1","key":"e_1_2_10_9_1","first-page":"93","article-title":"Moyamoya","volume":"8","author":"Ganesan V.","year":"2010","journal-title":"Journal of Pediatric Neurology"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00234-016-1737-3"},{"key":"e_1_2_10_11_1","unstructured":"Ijner P. 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