AJDRAJNR - American Journal of Neuroradiology

Published ahead of print on May 15, 2008
doi: 10.3174/ajnr.A1106

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Classification of Childhood White Matter Disorders Using Proton MR Spectroscopic Imaging

A. Bizzia, G. Castellia, M. Bugianib, P.B. Barkerd, E.H. Herskovitsc, U. Danesia, A. Erbettaa, I. Moronib, L. Farinaa and G. Uzielb

a Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico "Carlo Besta," Milan, Italy
b Child Neurology, Fondazione IRCCS Istituto Neurologico "Carlo Besta," Milan, Italy
c Department of Radiology, University of Pennsylvania, Philadelphia, Pa
d Russell H Morgan Department of Radiology and Radiological Science, Johns Hopkins University, Baltimore, Md


Figure 1
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Fig 1. T2-weighted MR, selected spectra, and metabolic images of Cho, Cr, NAA, and lactate (Lac) in a typical case of hypomyelination (Pelizaeus-Merzbacher disease). Spectral patterns from both white and gray matter show near-normal profiles, with a large NAA signal intensity and near-equal Cho and Cr. Bright signal at the corners of the metabolic images arises from residual lipid signals from the scalp. PPM indicates parts per million.


Figure 2
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Fig 2. T2-weighted MR, selected spectra, and metabolic images of Cho, Cr, NAA, and lactate (Lac) in a case of rarefaction of white matter (LVWM). Metabolic images clearly show decreased Cr and NAA in T2-hyperintense white matter regions, whereas Cho is largely unchanged. In white matter voxels, the NAA/Cho ratio is approximately 1:1. This patient was diagnosed with LVWM at a very early stage and is 1 of the 2 patients with LVWM misclassified in group 1. PPM indicates parts per million.


Figure 3
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Fig 3. T2-weighted MR, selected spectra, and metabolic images of Cho, Cr, NAA, and lactate (Lac) in a typical demyelination case (metachromatic leukodystrophy). T2-hyperintense white matter regions are characterized by increased Cho and decreased NAA. The gray matter spectrum (voxel 4) also shows a somewhat decreased NAA/Cho ratio. PPM indicates parts per million.


Figure 4
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Fig 4. T2-weighted MR, selected spectra, and metabolic images of Cho, Cr, NAA, and lactate (Lac) in a typical patient with Canavan disease. NAA is higher than normal in both white and gray matter regions. Note lower (ie, more normal) NAA levels, as seen in voxel 2 and in the NAA metabolic image, in the deeper white matter where there is less signal-intensity abnormality on the T2-weighted MR image. PPM indicates parts per million.