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Case Reports
. 2000 Sep;21(8):1478-82.

MR imaging and proton MR spectroscopy in adult Krabbe disease

L Farina  1 , A Bizzi, G Finocchiaro, D Pareyson, A Sghirlanzoni, B Bertagnolio, M Savoiardo, S Naidu, B S Singhal, D A Wenger
Affiliations
Case Reports

MR imaging and proton MR spectroscopy in adult Krabbe disease

L Farina et al. AJNR Am J Neuroradiol. 2000 Sep.

Abstract

We present the MR imaging findings in four patients (two pairs of siblings from two unrelated families) with adult Krabbe disease. In the first family, clinical presentation mimicked familial spastic paraplegia. Their MR images showed selective, increased signal intensity on T2-weighted sequences along the corticospinal tracts, most prominently in the proband and barely detectable in her brother. Proton MR spectroscopy showed increased choline and myo-inositol in the affected white matter. In the second family, the clinical presentation differed in that the signs of pyramidal tract involvement were asymmetrical, with concomitant asymmetry on MR images in one. In adults, Krabbe disease may present on MR imaging with selective pyramidal fiber involvement.

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Figures

<sc>fig</sc> 1.
fig 1.
Case 1. A, Axial FLAIR image (9264/150/2; TI = 2200) shows increased signal intensity of the white matter of the precentral gyri, which are atrophic (arrowhead). B, Coronal FLAIR image (9264/150/2; TI = 2200) shows the hyperintensity limited to the upper part of the motor cortex (arrows) and extending across the corpus callosum (arrowheads). C, Midline sagittal turbo spin-echo T2-weighted image (5422/110/2) shows thin, slightly hyperintense isthmus of the corpus callosum (arrow).
<sc>fig</sc> 2.
fig 2.
Case 2. Selected proton MR spectra (PRESS, 1500/136/1; nominal voxel size, 2 cm3) from the voxels indicated on the scout spin-echo T2-weighted MR image (2000/90/1; thickness, 10 mm) show moderate choline elevation (3.2 ppm), mild NAA loss (2.02 ppm), normal creatine peak (3.0 ppm), and an abnormally elevated peak at 3.6 ppm (myo-inositol) in the centrum semiovale. Spectra from the parasagittal cortical gray matter show only mild NAA loss. Similar proton MR spectroscopic findings in the centrum semiovale were found in case 1
<sc>fig</sc> 3.
fig 3.
Case 4. A and B, Coronal T2-weighted images (3648/99/2) of adjacent sections, A posterior to B, show nearly symmetrical involvement of the corticospinal tract from the cortex (A) to the brain stem (B) (asterisks). C, Axial T2-weighted image (2100/90/2) at pontine level shows symmetrical pyramidal involvement (arrowhead).
See this image and copyright information in PMC

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