1. Diagnostic value of proton MR spectroscopy and diffusion-weighted MR imaging in childhood inherited neurometabolic brain diseases and review of the literature 
Handan Cakmakci, Yeliz Pekcevik, Uluc Yis, Aycan Unalp, Semra Kurul 
European Journal of Radiology 
Volume 74, Issue 3, Pages e161-e171 (June 2010)
DOI: /j.ejrad
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2. Fig. 1
Pelizaeus-Merzbacher disease, T2-weighted MR images (a and b) show increased signal in pons (arrows on b), internal capsula (arrows on a), cerebral white matter. ADC map (c) shows increased diffusion in white matter (0.81±0.06×10−5cm2/s). Single voxel MRS (d) reveals decreased Cho/Cr ratio (0.73).
European Journal of Radiology  , e161-e171DOI: ( /j.ejrad )
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3. Fig. 2
Van der Knaap disease, T2-weighted axial (a) and T1-weighted coronal (b) images show extensive altered signal within the white matter and subcortical cysts (arrows) in the frontal and temporal lobes. ADC map shows increased diffusion in the lesions (1.96±0.08×10−5cm2/s) (c). Single voxel MRS (d) demonstrates normal NAA/Cr, increased mI/Cr and Glx/Cr ratios (arrows).
European Journal of Radiology  , e161-e171DOI: ( /j.ejrad )
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4. Fig. 3
Galactosemia T2-weighted image (a) shows increased signal in the cerebral white matter. ADC map (b) demonstrates increased diffusion (2.53±0.01×10−5cm2/s). There is a peak at 3.7ppm, inconsistent with galactitol (arrow) on single voxel short TE MRS spectra. NAA/Cr, Cho/Cr, mI/Cr ratios were also decreased.
European Journal of Radiology  , e161-e171DOI: ( /j.ejrad )
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5. Fig. 4
Canavan disease, T2-weighted axial image (a) demonstrates diffuse increased signal within the white matter, bilateral globus pallidum, thalamic nuclei (arrows). ADC map (b) showed slightly increased diffusion on white matter, globus pallidum, thalamic nucleus and MRS (c) revealed prominent increased NAA/Cr ratio (arrow).
European Journal of Radiology  , e161-e171DOI: ( /j.ejrad )
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6. Fig. 5
X-linked adrenoleukodystrophy, splenium of corpus callosum (arrow), temporooccipital white matter have increased signal on T2-weighted image (a). After contrast material administration peripheral increased signal is shown on axial T1-weighted image (b). ADC values have prominent increase from peripheral to central areas on ADC map (c). MRS (d) shows decreased NAA/Cr, significantly increased Cho/Cr levels and lactate peak (arrow) which reflects active demyelination.
European Journal of Radiology  , e161-e171DOI: ( /j.ejrad )
Copyright © 2009 Elsevier Ireland Ltd Terms and Conditions