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Relaxometry yields information on myelin loss or malformation and may substantiate results from DTI attributed to myelin changes. Relaxometry was used to determine four brain compartments in normal\u2010appearing brain tissue (NABT) and in lesions: water in myelin bilayers (myelin water, MW), water in gray matter (GM), water in WM, and water with long relaxation times (cerebrospinal fluid [CSF]\u2010like signals). DTI yielded apparent diffusion coefficients (ADCs) and fractional anisotropies. MW and WM content were reduced in NABT and in lesions of PKU patients, while CSF\u2010like signals were significantly increased. ADC values were reduced in PKU lesions, but also in the corpus callosum. Diffusion anisotropy was reduced in lesions because of a stronger decrease in the longitudinal than in the transverse diffusion. WM content and CSF\u2010like components in lesions correlated with anisotropy and ADC. ADC values in lesions and in the corpus callosum correlated negatively with blood and brain phenylalanine (Phe) concentrations. Intramyelinic edema combined with vacuolization is a likely cause of the WM alterations. Correlations between diffusivity and Phe concentrations confirm vulnerability of WM to high Phe concentrations. Magn Reson Med 58:1145\u20131156, 2007. \u00a9 2007 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/mrm.21422","type":"journal-article","created":{"date-parts":[[2007,11,28]],"date-time":"2007-11-28T16:46:32Z","timestamp":1196268392000},"page":"1145-1156","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Characterization of white matter alterations in phenylketonuria by magnetic resonance relaxometry and diffusion tensor imaging"],"prefix":"10.1002","volume":"58","author":[{"given":"Peter","family":"Vermathen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laure","family":"Robert\u2010Tissot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joachim","family":"Pietz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Lutz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"Boesch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roland","family":"Kreis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,11,28]]},"reference":[{"key":"e_1_2_6_2_2","first-page":"1667","volume-title":"The metabolic and molecular bases of inherited disease","author":"Scriver CR","year":"2001"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1097\/00019052-199812000-00012"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01957228"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(94)91281-5"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005621727560"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1007\/3-540-27660-2_36"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0455.2001.420505.x"},{"key":"e_1_2_6_9_2","first-page":"1583","article-title":"Diffusion\u2010weighted imaging of white matter abnormalities in patients with phenylketonuria","volume":"22","author":"Phillips MD","year":"2001","journal-title":"AJNR Am J Neuroradiol"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2362040611"},{"key":"e_1_2_6_11_2","unstructured":"TosettiM LeuzziV D'AlesioV MontanaroD ScarabinoT.Brain abnormalities in phenylketonuria: evaluation with a 3 T MR system. 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