MR Imaging and Proton MR Spectroscopy in Adult Krabbe Disease
Laura Farina
,a,
Alberto Bizzia,
Gaetano Finocchiaroa,
Davide Pareysona,
Angelo Sghirlanzonia,
Barbara Bertagnolioa,
Mario and Savoiardoa,
and SakkuBai Naidua,
Bhim S. Singhala and
David A. Wengera
a From the Departments of Neuroradiology (L.F., A.B., M.S.), Neuro-Oncology and Gene Therapy (G.F.), Neurology (D.P., A.S.), and Biochemistry and Genetics (B.B.), Istituto Nazionale Neurologico, Milan, Italy; the Department of Pediatrics and Neurology, Johns Hopkins Medical Institutions and the Neurogenetics Unit, Kennedy Krieger Institute, Baltimore, MD (S.N.); the Department of Neurology, Bombay Hospital, Medical Research Centre, Bombay, India (B.S.S.); and the Department of Neurology, Jefferson Medical College, Philadelphia PA (D.A.W.).
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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).
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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
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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).
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