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Normal-appearing White Matter in Patients with Phenylketonuria: Water Content, Myelin Water Fraction, and Metabolite Concentrations¹

¹ From the Departments of Medicine (S.M.S.), Radiology (C.L., A.L.M.), Physics and Astronomy (B.M., S.A.T., A.L.M.), and Psychology (C.B.), University of British Columbia, Vancouver, British Columbia, Canada; and Department of Physics, Simon Fraser University, Burnaby, British Columbia, Canada (E.E.B.). Received October 28, 2005; revision requested December 1; revision received January 20, 2006; accepted February 6; final version accepted March 8. Supported by a Vancouver Hospital and Health Sciences Centre Interdisciplinary Grant. Address correspondence to S.M.S., Level 4, Diamond Health Care Center, 2775 Laurel St, Vancouver, BC, Canada V5Z 1M9 (e-mail: sandra.sirrs{at}vch.ca).

View larger version (134K): [in this window] [in a new window] [Download PPT slide]   Figure 1a: (a) Sagittal MR image (300/14) shows T2 measurement section (line) and spectroscopy voxel (box). Transverse image (inset; 2200/28) shows anteroposterior and right-to-left extent of voxel. (b) Regions of interest of WM structures on IW MR image (2200/28) for analysis of MWF and RWC. Regions of interest were analyzed on both right and left sides.

View larger version (107K): [in this window] [in a new window] [Download PPT slide]   Figure 1b: (a) Sagittal MR image (300/14) shows T2 measurement section (line) and spectroscopy voxel (box). Transverse image (inset; 2200/28) shows anteroposterior and right-to-left extent of voxel. (b) Regions of interest of WM structures on IW MR image (2200/28) for analysis of MWF and RWC. Regions of interest were analyzed on both right and left sides.

View larger version (19K): [in this window] [in a new window] [Download PPT slide]   Figure 2: RWC values and standard errors according to structure for normal WM (NWM) in 15 control subjects and NAWM and diffuse WM (DiffWM) lesions in 15 patients with PKU. Note the increased RWC exhibited in all IW-T2–long T2 NAWM and IW-T2 NAWM structures. IC = internal capsules, Major = major forceps, Minor = minor forceps.

View larger version (18K): [in this window] [in a new window] [Download PPT slide]   Figure 3: MWF values and standard errors according to structure in normal WM (NWM) in 15 control subjects and NAWM and diffuse WM (DiffWM) lesions in 15 patients with PKU. Note the decreased MWF in diffuse WM lesions and, on average, all WM. IC = internal capsules, Major = major forceps, Minor = minor forceps.

View larger version (39K): [in this window] [in a new window] [Download PPT slide]   Figure 4: A, MR image (3000/90) shows region of interest in patient with PKU for IW-T2–long T2 NAWM and diffuse WM (DiffWM) lesions. Panels B and C show NAWM T2 decay curve and corresponding T2 distribution, respectively. D and E show diffuse WM lesion T2 decay curve and corresponding T2 distribution, respectively. The residuals of the decay curve fits are shown above the curves in B and D. Note the prolonged T2 peak in E.