{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:48:02Z","timestamp":1786978082960,"version":"build-2736575974"},"reference-count":37,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,5,27]],"date-time":"2015-05-27T00:00:00Z","timestamp":1432684800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NIH","award":["T32 GM007454"],"award-info":[{"award-number":["T32 GM007454"]}]},{"name":"NIH","award":["K08 MH100609"],"award-info":[{"award-number":["K08 MH100609"]}]},{"name":"The Genzyme Corporation, a Sanofi Company."}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Magnetic Resonance Imaging"],"published-print":{"date-parts":[[2015,12]]},"abstract":"\n Background<\/jats:title>\n To identify quantitative MRI parameters associated with diffusion tensor imaging (DTI) and fast bound\u2010pool fraction imaging (FBFI) that may detect alterations in gray matter and\/or white matter in adults with Fabry disease, a lysosomal storage disorder.<\/jats:p>\n <\/jats:sec>\n \n Materials and Methods<\/jats:title>\n \n Twelve healthy controls (mean age \u00b1 standard deviation: 48.0 \u00b1 12.4 years) and 10 participants with Fabry disease (46.7 \u00b1 12.9 years) were imaged at 3.0 Tesla. Whole\u2010brain parametric maps of diffusion tensor metrics (apparent diffusion coefficient [ADC] and fractional anisotropy [FA]) and the bound\u2010pool fraction (\n f<\/jats:italic>\n ) were acquired. Mean voxel values of parametric maps from regions\u2010of\u2010interest within gray and white matter structures were compared between cases and controls using the independent t\u2010test. Spearman's rho was used to identify associations between parametric maps and age.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Compared with controls, the left thalamus of Fabry participants had an increase in FA (0.29 \u00b1 0.02 versus 0.33 \u00b1 0.05, respectively;\n P<\/jats:italic>\n = 0.030) and a trend toward an increase in ADC (0.73 \u00b1 00.02 versus 0.76 \u00b1 0.03 \u03bcm\n 2<\/jats:sup>\n \/s, respectively;\n P<\/jats:italic>\n = 0.082). The left posterior white matter demonstrated a reduction in\n f<\/jats:italic>\n (10.45 \u00b1 0.37 versus 9.00 \u00b1 1.84%, respectively;\n P<\/jats:italic>\n = 0.035), an increase in ADC (0.78 \u00b1 0.04 versus 0.94 \u00b1 0.19 \u03bcm\n 2<\/jats:sup>\n \/s, respectively;\n P<\/jats:italic>\n = 0.024), and a trend toward a reduction in FA (0.42 \u00b1 0.07 versus 0.36 \u00b1 0.08, respectively;\n P<\/jats:italic>\n = 0.052). Among all parameters, only\n f<\/jats:italic>\n measured in the left posterior white matter was significantly associated with age in Fabry participants (rho = \u20130.71;\n P<\/jats:italic>\n = 0.022).\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n Parameters derived from DTI and FBFI detect Fabry\u2010related changes in the adult human brain, particularly in the posterior white matter where reductions in myelin density as measured by FBFI appear age related. J. MAGN. RESON. IMAGING 2015;42:1611\u20131622.<\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/jmri.24952","type":"journal-article","created":{"date-parts":[[2015,5,27]],"date-time":"2015-05-27T13:55:47Z","timestamp":1432734947000},"page":"1611-1622","source":"Crossref","is-referenced-by-count":12,"title":["Detecting the effects of Fabry disease in the adult human brain with diffusion tensor imaging and fast bound\u2010pool fraction imaging"],"prefix":"10.1002","volume":"42","author":[{"given":"Hunter R.","family":"Underhill","sequence":"first","affiliation":[{"name":"Departments of Neurological Surgery and Medicine, Division of Medical Genetics University of Washington Seattle Washington USA"},{"name":"Department of Pediatrics, Division of Medical Genetics University of Utah Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie","family":"Golden\u2010Grant","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Washington Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren T.","family":"Garrett","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Washington Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefanie","family":"Uhrich","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Washington Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brandon A.","family":"Zielinski","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Neurology University of Utah Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. 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