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The authors aimed to investigate cognitive function in the presurgical phase of pediatric patients with MMD with no apparent brain lesions.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>In this prospective, observational, single-center study, 21 children (mean age 10 \u00b1 3.0 years, range 5\u201314 years) diagnosed with MMD at Hokkaido University Hospital between 2012 and 2018 were enrolled. A cross-sectional evaluation of intellectual ability was performed using the Wechsler Intelligence Scale for Children\u2014Fourth Edition at the initial diagnosis. rCBF was measured using [<jats:sup>123<\/jats:sup>I] <jats:italic>N<\/jats:italic>-isopropyl <jats:italic>p<\/jats:italic>-iodoamphetamine\/SPECT. The associations among clinical factors, disease severity, regional cerebral blood flow (rCBF), and intelligence test scores were also examined.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>The mean full-scale intelligence quotient (FIQ) was 101.8 \u00b1 12.5 (range 76\u2013125) in children with no apparent brain lesions. A significant difference in the intelligence scale index score was observed, most frequently (42.9%) between working memory index (WMI) and verbal comprehension index (VCI; VCI \u2212 WMI &gt; 11 points). Regional CBF was significantly reduced both in the left and right medial frontal cortices (left: 61.3 \u00b1 5.3 ml\/100 g\/min, right 65.3 \u00b1 5.3 ml\/100 g\/min; p &lt; 0.001) compared to the cerebellum (77.8 \u00b1 6.8 ml\/100 g\/min). There was a significant association of rCBF in the left dorsolateral prefrontal cortex (DLPFC) with FIQ (r = 0.46, p = 0.034), perceptual reasoning index (PRI; r = 0.44, p = 0.045), and processing speed index (PSI; r = 0.44, p = 0.045). There was an association between rCBF of the left medial frontal cortex and PSI (r = 0.49, p = 0.026). Age of onset, family history, ischemic symptoms, and angiographic severity were not associated with poor cognitive performance.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSIONS<\/jats:title><jats:p>Although average intellectual ability was not reduced in children with MMD, the association of reduced rCBF in the left DLPFC and medial frontal cortex with FIQ, PRI, and PSI suggests mild cognitive dysfunction due to cerebral hypoperfusion.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2019.7.peds19312","type":"journal-article","created":{"date-parts":[[2019,10,19]],"date-time":"2019-10-19T07:42:23Z","timestamp":1571470943000},"page":"62-68","source":"Crossref","is-referenced-by-count":25,"title":["Association of cognitive function with cerebral blood flow in children with moyamoya disease"],"prefix":"10.3171","volume":"25","author":[{"given":"Ken","family":"Kazumata","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kikutaro","family":"Tokairin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taku","family":"Sugiyama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaki","family":"Ito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruto","family":"Uchino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiya","family":"Osanai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahito","family":"Kawabori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Nakayama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kiyohiro","family":"Houkin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Hum Brain Mapp","doi-asserted-by":"publisher","first-page":"828","DOI":"10.1002\/hbm.20581","article-title":"Modulation of temporally coherent brain networks estimated using ICA at rest and during cognitive tasks","volume":"29","author":"Calhoun VD","year":"2008","unstructured":"Calhoun VD, Kiehl KA, Pearlson GD: Modulation of temporally coherent brain networks estimated using ICA at rest and during cognitive tasks. 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