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AJNR - American Journal of Neuroradiology

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Emery-Dreiffus Muscular Dystrophy: MR Imaging and Spectroscopy in the Brain and Skeletal Muscle

Robert Semnic^a, Goran Vucurevic^b, Dusko Kozic^a, Katarina Koprivsek^a, Jelena Ostojic^a and Rifat Nuri Sener^c

^a Diagnostic Imaging Department, Institute of Oncology, Sremska Kamenica, Serbia and Montenegro, Maintz, Germany
^b Department of Neuroradiology, University of Maintz, Maintz, Germany
^c Department of Radiology, Ege University Hospital, Bornova, Izmir, Turkey

View larger version (127K): [in a new window]   FIG 1. T2-weighted image obtained in a 15-year-old boy reveals bilateral high-signal intensity changes in the periatrial white matter, likely representing hypomyelination. A region of encephalomalacia is noted in the right internal capsule.

View larger version (149K): [in a new window]   FIG 2. T2-weighted image obtained in an 11-year-old boy reveals bilateral high-signal intensity changes in the periatrial white matter similar to the older brother.

View larger version (26K): [in a new window]   FIG 3. Proton MR spectrum (STEAM; TE = 20 ms, TR = 2000 ms) obtained in the 15-year-old boy reveals normal peaks of NAA, Cr, Cho, and myoinositol. A prominent peak is evident, centered at 1.5 ppm, likely belonging to proteolipids. Spectroscopy of the younger brother revealed identical changes.