MR evidence of long T2 water in pathological white matter.

PURPOSE: To describe what, if any, specific long T(2)-related abnormalities occur in the white matter of subjects with either phenylketonuria (PKU) or multiple sclerosis (MS). MATERIALS AND METHODS: The 48-echo T(2) relaxation data (maximum TE = 1.12 sec) were acquired from 15 PKU subjects, 20 MS subjects, and 15 healthy volunteers. Regions of interest were drawn in diffuse white matter hyperintensities (DiffWM), lesions, normal-appearing white matter (NAWM), and normal white matter. Long T(2) maps (200 msec < T(2) < 800 msec) were created for each subject. RESULTS: A new water reservoir with a markedly prolonged T(2) peak was identified in DiffWM and NAWM in 12 out of 15 subjects with PKU and a long T(2) signal was also seen in 23/97 lesions in 50% of subjects with MS. Additionally, a long T(2) component was observed in the corticospinal tracts of 10 healthy volunteers. The characteristics of the long T(2) signal were unique for each subject group. Potential sources of this signal include vacuolation and increases in extracellular water. CONCLUSION: This study supports the usefulness of increasing the data acquisition window of the multiecho T(2) relaxation sequence to better characterize the T(2) decay from pathological brain.

Normal-appearing white matter in patients with phenylketonuria: water content, myelin water fraction, and metabolite concentrations.

PURPOSE: To prospectively assess relative water content (RWC), myelin water fraction (MWF), and hydrogen 1 magnetic resonance (MR) spectroscopy findings in the white matter (WM) of patients with phenylketonuria (PKU). MATERIALS AND METHODS: This study was approved by the institution's investigational review board, and informed consent was obtained. T2 water relaxation data were acquired by using a 48-echo measurement in a transverse plane through the genu and splenium of the corpus callosum in 16 patients (six men, 10 women; age range, 18-40 years) with PKU and 16 age- and sex-matched control subjects. MR spectroscopy was performed in a voxel (94x70x15 mm) above the ventricles. WM in control subjects (defined as normal WM) was compared with normal-appearing WM (NAWM) and diffuse WM lesions in patients with PKU by using a Student t test. RESULTS: Patients with PKU had two forms of NAWM: (a) areas that looked normal on intermediate-weighted (IW) and T2-weighted MR images and long T2 maps and (b) areas that looked normal on IW and T2-weighted MR images but were hyperintense on long T2 maps. Both forms of NAWM showed increased RWC (up to 2.5%, P<.001) and reduced MWF (up to 56%, P<.001) relative to normal WM; these changes paralleled those seen in diffuse WM lesions. Approximately 9% of the water in diffuse WM lesions was in a reservoir with a long T2 time of 200-800 msec. Myoinositol concentrations were reduced by 14% (P=.003) in patients with PKU. CONCLUSION: In patients with PKU, NAWM and diffuse WM lesions have altered RWC and MWF relative to normal WM, and diffuse WM lesions show a redistribution of water into an extracellular reservoir with a long T2 time.